Sample records for galen arteriovenous malformation

  1. Loss of function mutations in EPHB4 are responsible for vein of Galen aneurysmal malformation.

    PubMed

    Vivanti, Alexandre; Ozanne, Augustin; Grondin, Cynthia; Saliou, Guillaume; Quevarec, Loic; Maurey, Helène; Aubourg, Patrick; Benachi, Alexandra; Gut, Marta; Gut, Ivo; Martinovic, Jelena; Sénat, Marie Victoire; Tawk, Marcel; Melki, Judith

    2018-04-01

    See Meschia (doi:10.1093/brain/awy066) for a scientific commentary on this article.Vein of Galen aneurysmal malformation is a congenital anomaly of the cerebral vasculature representing 30% of all paediatric vascular malformations. We conducted whole exome sequencing in 19 unrelated patients presenting this malformation and subsequently screened candidate genes in a cohort of 32 additional patients using either targeted exome or Sanger sequencing. In a cohort of 51 patients, we found five affected individuals with heterozygous mutations in EPHB4 including de novo frameshift (p.His191Alafs*32) or inherited deleterious splice or missense mutations predicted to be pathogenic by in silico tools. Knockdown of ephb4 in zebrafish embryos leads to specific anomalies of dorsal cranial vessels including the dorsal longitudinal vein, which is the orthologue of the median prosencephalic vein and the embryonic precursor of the vein of Galen. This model allowed us to investigate EPHB4 loss-of-function mutations in this disease by the ability to rescue the brain vascular defect in knockdown zebrafish co-injected with wild-type, but not truncated EPHB4, mimicking the p.His191Alafs mutation. Our data showed that in both species, loss of function mutations of EPHB4 result in specific and similar brain vascular development anomalies. Recently, EPHB4 germline mutations have been reported in non-immune hydrops fetalis and in cutaneous capillary malformation-arteriovenous malformation. Here, we show that EPHB4 mutations are also responsible for vein of Galen aneurysmal malformation, indicating that heterozygous germline mutations of EPHB4 result in a large clinical spectrum. The identification of EPHB4 pathogenic mutations in patients presenting capillary malformation or vein of Galen aneurysmal malformation should lead to careful follow-up of pregnancy of carriers for early detection of anomaly of the cerebral vasculature in order to propose optimal neonatal care. Endovascular

  2. Human genetics and molecular mechanisms of vein of Galen malformation.

    PubMed

    Duran, Daniel; Karschnia, Philipp; Gaillard, Jonathan R; Karimy, Jason K; Youngblood, Mark W; DiLuna, Michael L; Matouk, Charles C; Aagaard-Kienitz, Beverly; Smith, Edward R; Orbach, Darren B; Rodesch, Georges; Berenstein, Alejandro; Gunel, Murat; Kahle, Kristopher T

    2018-04-01

    Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation-arteriovenous malformation syndrome ( RASA1) and hereditary hemorrhagic telangiectasia ( ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients' families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances

  3. Pediatric neuroanesthesia. Arteriovenous malformations.

    PubMed

    Newfield, P; Hamid, R K

    2001-06-01

    Intracranial arteriovenous malformations can occur singly, multiply, and in conjunction with aneurysms and denovo, family, or in conjunction with connective-tissue disorders. Intracranial hemorrhage is the most common presentation, occurring in 20% to 50% of cases. In children, seizures are the second most common presentation occurring in 15% to 20% of cases. The modalities available treatment of arteriovenous malformations are microsurgery, embolization, and stereotactic radiosurgery with heavy particles, alpha knife, or linear accelerator. Induction, maintenance, and emergence from anesthesia are designed to prevent rupture of arteriovenous malformation and aneurysm and to improve intracranial compliance in the presence of an intracranial hematoma, during both diagnostic (CT, MR scanning) and therapeutic procedures.

  4. Acquired Uterine Arteriovenous Malformation and Retained Placenta Increta.

    PubMed

    Roach, Michelle K; Thomassee, May S

    2015-09-01

    Uterine arteriovenous malformations are rare and have been reported to occur after uterine trauma (eg, surgery, gestational trophoblastic disease, malignancy). A 33-year-old woman, gravida 3 para 3, presented 4 weeks post-cesarean delivery with episodic profuse vaginal bleeding. Pelvic ultrasonography and magnetic resonance imaging revealed a left uterine arteriovenous malformation. After consideration of all treatment options, total laparoscopic hysterectomy was performed. Acquired uterine arteriovenous malformations and placental ingrowth into the myometrium are increasingly reported after surgical uterine procedures. This case of a postpartum patient with both uterine arteriovenous malformation and retained placenta increta suggests a correlation between the two complications.

  5. Arteriovenous Malformations

    MedlinePlus

    Arteriovenous malformations (AVMs) are defects in your vascular system. The vascular system includes arteries, veins, and capillaries. Arteries carry blood away from the heart to other organs; veins carry blood back to the heart. Capillaries connect the arteries and veins. An ...

  6. Ultrasound-guided high-intensity focused ultrasound ablation for treating uterine arteriovenous malformation.

    PubMed

    Yan, X; Zhao, C; Tian, C; Wen, S; He, X; Zhou, Y

    2017-08-01

    To explore HIFU treatment for uterine arteriovenous malformation. A case report. Gynaecological department in a university teaching hospital of China. A patient with uterine arteriovenous malformation. The diagnosis of uterine arteriovenous malformation was made through MRI. Ultrasound-guided high-intensity focused ultrasound (USgHIFU) ablation was performed. HIFU is effective in treating uterine arteriovenous malformation. The patient had reduction of the lesion volume and obvious symptom relief, without significant adverse effects. HIFU can be used as a new treatment option for uterine arteriovenous malformation. Ultrasound-guided high-intensity focused ultrasound ablation is effective in treating uterine arteriovenous malformation. © 2017 Royal College of Obstetricians and Gynaecologists.

  7. Notch-1 Signalling Is Activated in Brain Arteriovenous Malformations in Humans

    ERIC Educational Resources Information Center

    ZhuGe, Qichuan; Zhong, Ming; Zheng, WeiMing; Yang, Guo-Yuan; Mao, XiaoOu; Xie, Lin; Chen, Gourong; Chen, Yongmei; Lawton, Michael T.; Young, William L.; Greenberg, David A.; Jin, Kunlin

    2009-01-01

    A role for the Notch signalling pathway in the formation of arteriovenous malformations during development has been suggested. However, whether Notch signalling is involved in brain arteriovenous malformations in humans remains unclear. Here, we performed immunohistochemistry on surgically resected brain arteriovenous malformations and found that,…

  8. Anaesthetic management of a child with massive extracranial arteriovenous malformation

    PubMed Central

    Shamim, Faisal; Ullah, Hameed; Rehman, Azhar

    2012-01-01

    Vascular tumors affect the head and neck commonly but arteriovenous malformations are rare. Vascular malformations are often present at birth and grow with the patient, usually only becoming significant later in childhood. Embolization has been the mainstay of treatment in massive and complex arteriovenous malformations. We present a case of massive extracranial arteriovenous malformation in a 7-year-old boy causing significant workload on right heart and respiratory distress. The management of angioembolization under general anaesthesia and anaesthetic concerns are presented. PMID:22345959

  9. Life-threatening haemothorax: a rare presentation of pulmonary arteriovenous malformation.

    PubMed

    Kundu, Somenath; Mitra, Subhra; Mukherjee, Shubhasis; Chakravorty, Anushree

    2010-11-01

    Arteriovenous malformations of the lung are rare pulmonary vascular disorders which can suddenly lead to life threatening complications. Haemothorax due to rupture of a pulmonary arteriovenous malformation (PAVM) is very rare. We report here a case of a 39 year-old lady who presented with an acute onset of shortness of breath due to right-sided massive haemothorax and was subsequently detected to have pulmonary as well as cerebral arteriovenous malformation (CAVM).

  10. Numerical modeling process of embolization arteriovenous malformation

    NASA Astrophysics Data System (ADS)

    Cherevko, A. A.; Gologush, T. S.; Petrenko, I. A.; Ostapenko, V. V.

    2017-10-01

    Cerebral arteriovenous malformation is a difficult, dangerous, and most frequently encountered vascular failure of development. It consists of vessels of very small diameter, which perform a discharge of blood from the artery to the vein. In this regard it can be adequately modeled using porous medium. Endovascular embolization of arteriovenous malformation is effective treatment of such pathologies. However, the danger of intraoperative rupture during embolization still exists. The purpose is to model this process and build an optimization algorithm for arteriovenous malformation embolization. To study the different embolization variants, the initial-boundary value problems, describing the process of embolization, were solved numerically by using a new modification of CABARET scheme. The essential moments of embolization process were modeled in our numerical experiments. This approach well reproduces the essential features of discontinuous two-phase flows, arising in the embolization problems. It can be used for further study on the process of embolization.

  11. Unusual Association: Cerebral Arteriovenous Malformation and Chiari Type I Malformation.

    PubMed

    Ogul, Hayri; Kantarci, Mecit

    2017-06-01

    Cerebral arteriovenous malformation (AVM) is a common type of cerebral vascular malformation. The imaging findings are enlarged vessels, thrombosed sinuses, and hemorrhage or gliosis on adjacent brain parenchyma. Magnetic resonance (MR) imaging can be used safely for diagnosis. Chiari type I malformation is characterized by a caudal descent of the cerebellar tonsil. Coincidence of cerebral AVM and Chiari type I malformation is very rare. In this paper, the authors report MR imaging findings of a patient with coincidence of cerebral AVM and Chiari type I malformation.

  12. RASA1 analysis guides management in a family with capillary malformation-arteriovenous malformation

    PubMed Central

    Flore, Leigh Anne; Leon, Eyby; Maher, Tom A.; Milunsky, Jeff M.

    2012-01-01

    Capillary malformation-arteriovenous malformation (CM-AVM; MIM 60354) is an autosomal dominant disorder characterized by multifocal cutaneous capillary malformations, often in association with fast-flow vascular lesions, which may be cutaneous, subcutaneous, intramuscular, intraosseus, or cerebral arteriovenous malformations or arteriovenous fistulas. CM-AVM results from heterozygous mutations in the RASA1 gene. Capillary malformations of the skin are common, and clinical examination alone may not be able to definitively diagnose-or exclude- CM-AVM. We report a family in which the proband was initially referred for a genetic evaluation in the neonatal period because of the presence of a cardiac murmur and minor dysmorphic features. Both he and his mother were noted to have multiple capillary malformations on the face, head, and extremities. Echocardiography revealed dilated head and neck vessels and magnetic resonance imaging and angiography of the brain revealed a large infratentorial arteriovenous fistula, for which he has had two embolization procedures. RASA1 sequence analysis revealed a heterozygous mutation, confirming his diagnosis of CM-AVM. We established targeted mutation analysis for the proband's mother and sister, the latter of whom is a healthy 3-year-old whose only cutaneous finding is a facial capillary malformation. This revealed that the proband's mother is also heterozygous for the RASA1 mutation, but his sister is negative. Consequently, his mother will undergo magnetic resonance imaging and angiography screening for intracranial and spinal fast-flow lesions, while his sister will require no imaging or serial evaluations. Targeted mutation analysis has been offered to additional maternal family members. This case illustrates the benefit of molecular testing in diagnosis and making screening recommendations for families with CM-AVM. PMID:27625812

  13. RASA1 analysis guides management in a family with capillary malformation-arteriovenous malformation.

    PubMed

    Flore, Leigh Anne; Leon, Eyby; Maher, Tom A; Milunsky, Jeff M

    2012-06-01

    Capillary malformation-arteriovenous malformation (CM-AVM; MIM 60354) is an autosomal dominant disorder characterized by multifocal cutaneous capillary malformations, often in association with fast-flow vascular lesions, which may be cutaneous, subcutaneous, intramuscular, intraosseus, or cerebral arteriovenous malformations or arteriovenous fistulas. CM-AVM results from heterozygous mutations in the RASA1 gene. Capillary malformations of the skin are common, and clinical examination alone may not be able to definitively diagnose-or exclude- CM-AVM. We report a family in which the proband was initially referred for a genetic evaluation in the neonatal period because of the presence of a cardiac murmur and minor dysmorphic features. Both he and his mother were noted to have multiple capillary malformations on the face, head, and extremities. Echocardiography revealed dilated head and neck vessels and magnetic resonance imaging and angiography of the brain revealed a large infratentorial arteriovenous fistula, for which he has had two embolization procedures. RASA1 sequence analysis revealed a heterozygous mutation, confirming his diagnosis of CM-AVM. We established targeted mutation analysis for the proband's mother and sister, the latter of whom is a healthy 3-year-old whose only cutaneous finding is a facial capillary malformation. This revealed that the proband's mother is also heterozygous for the RASA1 mutation, but his sister is negative. Consequently, his mother will undergo magnetic resonance imaging and angiography screening for intracranial and spinal fast-flow lesions, while his sister will require no imaging or serial evaluations. Targeted mutation analysis has been offered to additional maternal family members. This case illustrates the benefit of molecular testing in diagnosis and making screening recommendations for families with CM-AVM.

  14. Pathophysiology of increased cerebrospinal fluid pressure associated to brain arteriovenous malformations: The hydraulic hypothesis.

    PubMed

    Rossitti, Sandro

    2013-01-01

    Brain arteriovenous malformations (AVMs) produce circulatory and functional disturbances in adjacent as well as in remote areas of the brain, but their physiological effect on the cerebrospinal fluid (CSF) pressure is not well known. The hypothesis of an intrinsic disease mechanism leading to increased CSF pressure in all patients with brain AVM is outlined, based on a theory of hemodynamic control of intracranial pressure that asserts that CSF pressure is a fraction of the systemic arterial pressure as predicted by a two-resistor series circuit hydraulic model. The resistors are the arteriolar resistance (that is regulated by vasomotor tonus), and the venous resistance (which is mechanically passive as a Starling resistor). This theory is discussed and compared with the knowledge accumulated by now on intravasal pressures and CSF pressure measured in patients with brain AVM. The theory provides a basis for understanding the occurrence of pseudotumor cerebri syndrome in patients with nonhemorrhagic brain AVMs, for the occurrence of local mass effect and brain edema bordering unruptured AVMs, and for the development of hydrocephalus in patients with unruptured AVMs. The theory also contributes to a better appreciation of the pathophysiology of dural arteriovenous fistulas, of vein of Galen aneurismal malformation, and of autoregulation-related disorders in AVM patients. The hydraulic hypothesis provides a comprehensive frame to understand brain AVM hemodynamics and its effect on the CSF dynamics.

  15. Recurrent High-Flow Arterio-Venous Malformation of the Thyroid Gland.

    PubMed

    Borchert, D H; Massmann, A; Kim, Y J; Bader, C A; Wolf, G; Eisele, R; Minko, P; Bücker, A; Glanemann, M

    2015-09-01

    Vascular malformations and hemangiomas of the thyroid gland are rare disorders. The first case of a patient with recurrent high-flow arterio-venous malformation of the right thyroid gland involving the right endolarynx is presented. In June 2013, a 42-year-old female patient presented to the surgical department with recurrent hoarseness and a soft, vibrating mass on the right side of her neck. In 1993, she underwent right subtotal hemithyroidectomy with embolization on the day before surgery for a high-flow arterio-venous malformation of the thyroid gland. Diagnostic work-up in 2013 demonstrated a complex recurrent high-flow arterio-venous malformation on the right side of her neck involving the endolarynx. Full function of the right vocal fold could not be ascertained. The lesion was embolized again and excised the following day. Intraoperative gross bleeding and scar tissue prevented visualization and monitoring of the recurrent laryngeal nerve. Gross bleeding was also noted on hemithyroidectomy after embolization in 1993. No therapy was needed for the endolaryngeal part of the lesion. Histology showed large arterio-venous malformations with thyroid tissue. She remains well without signs of recurrence 18 month later but with a definitive voice handicap. This is the first report of a recurrent high-flow arterio-venous malformation originally developing from the right thyroid gland involving the right endolarynx. Counseling, diagnostic, and therapeutic work-up of the patient was possible only with an interdisciplinary team. The endolaryngeal part of the hemangioma dried out after embolization and completion hemithyroidectomy. Her hoarseness has greatly improved but a definitive voice handicap remains. High-flow arterio-venous malformations of the thyroid gland are a rare disease, and recurrent lesions have not been reported. Interdisciplinary management of these patients is mandatory due to the complex nature of the underlying pathology. Recurrence might develop

  16. Linking Brain Arteriovenous Malformations With Anorectal Hemorrhoids: A Clinical and Anatomical Review

    PubMed Central

    Cuoco, Joshua A.; Hoehmann, Christopher L.; Hitscherich, Kyle; Zakhary, Sherry M.; Leheste, Joerg R.

    2017-01-01

    ABSTRACT Patients who harbor brain arteriovenous malformations are at risk for intracranial hemorrhage. These malformations are often seen in inherited vascular diseases such as hereditary hemorrhagic telangiectasia. However, malformations within the brain also sporadically occur without a hereditary‐coding component. Here, we review recent insights into the pathophysiology of arteriovenous malformations, in particular, certain signaling pathways that might underlie endothelial cell pathology. To better interpret the origins, determinants and consequences of brain arteriovenous malformations, we present a clinical case to illustrate the phenotypic landscape of the disease. We also propose that brain arteriovenous malformations might share certain signaling dimensions with those of anorectal hemorrhoids. This working hypothesis provides casual anchors from which to understand vascular diseases characterized by arteriovenous lesions with a hemorrhagic‐ or bleeding‐risk component. Anat Rec, 2017. © The Authors. The Anatomical Record published by Wiley Periodicals, Inc. on behalf of American Association of Anatomists. Anat Rec, 300:1973–1980, 2017. © 2017 The Authors. The Anatomical Record published by Wiley Periodicals, Inc. on behalf of American Association of Anatomists. PMID:28696502

  17. Diagnosis of Vein of Galen aneurysmal malformation using fetal MRI.

    PubMed

    Zhou, Li-Xia; Dong, Su-Zhen; Zhang, Ming-Feng

    2017-11-01

    To present three fetal vein of Galen aneurysmal malformations (VGAMs), which were diagnosed through magnetic resonance imaging (MRI), and highlight these cardiovascular findings. We retrospectively reviewed three fetuses with VGAM at 31, 32, and 33 weeks of gestation. Feeding arteries and draining veins were observed by MRI. Secondary changes in the brain and high-output heart failure caused by high blood flow in the lesion were evaluated. Two fetuses were born, and neonatal MRI was performed. One fetus was terminated. A characteristic dilated structure in the midline of the brain presented in each fetus. The arteriovenous fistula led to anatomical brain changes such as in the hydrocephalus, dilated feeding vessels (one or more), jugular vein, and/or superior vena cava. Substantial brachiocephalic vessel dilation was observed in two fetuses. Following parturition, one baby had neonatal asphyxia and sinus thrombosis, and MRI revealed hypoxic-ischemic encephalopathy. Cardiomegaly was detected in all three cases. With a large field of view, fetal MRI can observe brain VGAM, as well as the heart and affected large vessels. It can determine hydrocephalus, ischemia, intracranial hemorrhage, and sinus thrombosis. Providing such information on the infant's entire body can aid clinicians in determining the most appropriate treatment. 4 J. Magn. Reson. Imaging 2017;46:1535-1539. © 2016 International Society for Magnetic Resonance in Medicine.

  18. Pathophysiology of increased cerebrospinal fluid pressure associated to brain arteriovenous malformations: The hydraulic hypothesis

    PubMed Central

    Rossitti, Sandro

    2013-01-01

    Background: Brain arteriovenous malformations (AVMs) produce circulatory and functional disturbances in adjacent as well as in remote areas of the brain, but their physiological effect on the cerebrospinal fluid (CSF) pressure is not well known. Methods: The hypothesis of an intrinsic disease mechanism leading to increased CSF pressure in all patients with brain AVM is outlined, based on a theory of hemodynamic control of intracranial pressure that asserts that CSF pressure is a fraction of the systemic arterial pressure as predicted by a two-resistor series circuit hydraulic model. The resistors are the arteriolar resistance (that is regulated by vasomotor tonus), and the venous resistance (which is mechanically passive as a Starling resistor). This theory is discussed and compared with the knowledge accumulated by now on intravasal pressures and CSF pressure measured in patients with brain AVM. Results: The theory provides a basis for understanding the occurrence of pseudotumor cerebri syndrome in patients with nonhemorrhagic brain AVMs, for the occurrence of local mass effect and brain edema bordering unruptured AVMs, and for the development of hydrocephalus in patients with unruptured AVMs. The theory also contributes to a better appreciation of the pathophysiology of dural arteriovenous fistulas, of vein of Galen aneurismal malformation, and of autoregulation-related disorders in AVM patients. Conclusions: The hydraulic hypothesis provides a comprehensive frame to understand brain AVM hemodynamics and its effect on the CSF dynamics. PMID:23607064

  19. Stomach arteriovenous malformation resected by laparoscopy-assisted surgery: A case report.

    PubMed

    Hotta, Masahiro; Yamamoto, Kazuhito; Cho, Kazumitsu; Takao, Yoshimune; Fukuoka, Takeshi; Uchida, Eiji

    2016-05-01

    Arteriovenous malformations of the stomach are an uncommon cause of upper GI bleeding. We report a case of stomach arteriovenous malformation in an 85-year-old Asian man who presented with massive hematemesis. Initial esophagogastroduodenoscopy did not detect this lesion, but contrast multi-detector CT confirmed GI bleeding. Multi-detector CT revealed a mass of blood vessels underlying the submucosa that arose from the right gastroepiploic artery. Repeat esophagogastroduodenoscopy showed that the lesion was a submucosal tumor with erosion and without active bleeding in the lower body of the stomach on the greater curvature. We performed partial gastrectomy via laparoscopy-assisted surgery. The histopathological diagnosis was arteriovenous malformation. © 2016 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd.

  20. Balloon-Occluded Retrograde Transvenous Embolization of a Pelvic Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Mitsuzaki, Katsuhiko; Yamashita, Yasuyuki; Utsunomiya, Daisuke

    1999-11-15

    We successfully performed embolization therapy for a pelvic arteriovenous malformation by the retrograde transvenous approach using a liquid embolic material. This malformation was unique in that it had a single draining vein, which allowed this technique employing an occlusion balloon.

  1. Arteriovenous malformation of the external ear: a clinical assessment with a scoping review of the literature.

    PubMed

    Kim, Shin Hye; Han, Seung Hoon; Song, Yoonjae; Park, Chang Sik; Song, Jae-Jin

    Auricular Arteriovenous Malformation of the external ear is a rarely encountered disease; in particular, arteriovenous malformation arising from the auricle, with spontaneous bleeding, has seldom been reported. In the current study, we report an unusual case of late-onset auricular arteriovenous malformation originating from the posterior auricular artery that was confirmed by computed tomographic angiography. The case was successfully managed by pre-surgical intravascular embolization followed by total lesion excision. Prompted by this case, we also present a scoping review of the literature. A case of a 60 year-old man with right auricular arteriovenous malformation treated in our tertiary care center, and 52 patients with auricular arteriovenous malformation described in 10 case reports and a retrospective review are presented. Auricular arteriovenous malformation can manifest as swelling of the ear, pulsatile tinnitus, pain, and/or bleeding. On physical examination, a pulsatile swelling and/or a tender mass is evident. When arteriovenous malformation is suspected, the lesions should be visualized using imaging modalities that optimally detect vascular lesions, and managed via embolization, mass excision, or auricular resection. Effectiveness of the various diagnostic methods used and the treatment outcomes were analyzed. Various imaging modalities including Doppler sonography, computed tomographic angiography, magnetic resonance angiography, and/or transfemoral cerebral angiography were used to diagnose 38 cases reported in the literature. In another 15 cases, no imaging was performed; treatment was determined solely by physical examination and auscultation. Of the total of 53 cases, 12 were not treated (their symptoms were merely observed) whereas 20 underwent therapeutic embolization. In total, 32 patients, including 1 patient who was not treated and 10 with persistent or aggravated arteriovenous malformation after previous embolization, underwent mass

  2. Unexpected Silent Infarctions after Embolization of Cerebral Arteriovenous Malformations and Fistulas

    PubMed Central

    Suazo, L.; Putman, J.O.; Vilchez, C.; Stoeter, P.

    2013-01-01

    Summary We investigated the number and possible causes of clinically silent lesions seen in diffusion-weighted magnetic resonance imaging after embolization of arteriovenous malformations (AVMs) and fistulas using acrylate only or in combination with coils. Included were 19 patients with 18 AVMs and one case of a vein of Galen aneurysm in which 25 interventions were carried out. Results of diffusion-weighted imaging, the appearance of perinidal and distant lesions, were correlated to Spetzler grade, nidus size, flow, number of feeders occluded, rate of nidus occlusion and duration of the intervention. We found seven distant lesions corresponding to non-symptomatic infarcts in the given clinical setting. The only significant correlation between lesion size and parameters analyzed was the degree of nidus occlusion achieved during the intervention. Because most of the lesions presented in cases with a high occlusion rate, they appear to be related to the intention to reach a complete occlusion of the nidus. These results emphasize that the risk involved in eliminating the nidus completely must be reconsidered with special care, particularly in a situation where most high-flow feeders have been occluded. PMID:23693045

  3. Embolization with Gamma Knife Radiosurgery of Giant Intracranial Arteriovenous Malformations.

    PubMed

    Chun, Dong Hyun; Kim, Moo Seong; Kim, Sung Tae; Paeng, Sung Hwa; Jeong, Hae Woong; Lee, Won Hee

    2016-01-01

    Giant arteriovenous malformations (i.e., those greater than 6 cm maximum diameter or volume > 33 cc) are difficult to treat and often carry higher treatment morbidity and mortality rates. In our study, we reviewed the angiographic results and clinical outcomes for 11 patients with giant arteriovenous malformations who were treated between 1994 and 2012. The patients selected included 9 males (82%) and 2 females (18%). Their presenting symptoms were hemorrhage (n=2; 18%), seizure (n=7; 64%), and headache (n=2; 12%). Nine patients were Spetzler-Martin Grade III, 2 were Spetzler-Martin Grade IV. The mean arteriovenous malformation volume was 41 cc (33-52 cc). The mean age of the patients was 45.1 years (24-57 years) and the mean radiation dose delivered to the margin of the nidus was 14.2 Gy. Ten patients received pre-Gamma Knife radiosurgery embolization and Gamma Knife radiosurgery, 1 patient received pre-Gamma Knife radiosurgery embolization and Gamma Knife radiosurgery twice and the interval between Gamma Knife radiosurgeries was 3 months. The complete obliteration rate following Gamma Knife radiosurgery was 36%, subtotal obliteration ( > 70% decreased size of nidus) was 36%, and partial obliteration was 28%. One patient experienced a small hemorrhage after embolization. Combined embolization and Gamma Knife radiosurgery showed successful obliteration of the arteriovenous malformation nidus. The use of embolization to initially reduce nidus size followed by Gamma Knife radiosurgery improves the treatment results. Repeated Gamma Knife radiosurgery should be a treatment option when there is a small nidus remnant.

  4. Embolization as the treatment for a life-threatening mandibular arteriovenous malformation.

    PubMed

    Loureiro, Caio Cesar de Souza; Falchet, Paula Cristina Felix; Gavranich, João; Lobo Leandro, Luiz Fernando

    2010-03-01

    Mandibular arteriovenous malformation is a severe vascular pathological condition that may lead to life-threatening hemorrhages. In child and teenaged patients, a conservative treatment is preferred to avoid profuse blood loss and/or mutilating alterations in the still-growing patients' face. The aim of this article was to present a case of an 11-year-old boy with an arteriovenous malformation involving the left mandible and treated by means of endovascular embolization with direct lesional delivery of N-butyl-cyanoacrylate glue through transfemoral catheterization.

  5. Rare malformation of glans penis: arteriovenous malformation.

    PubMed

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

    Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children.

  6. Ten-year detection rate of brain arteriovenous malformations in a large, multiethnic, defined population.

    PubMed

    Gabriel, Rodney A; Kim, Helen; Sidney, Stephen; McCulloch, Charles E; Singh, Vineeta; Johnston, S Claiborne; Ko, Nerissa U; Achrol, Achal S; Zaroff, Jonathan G; Young, William L

    2010-01-01

    To evaluate whether increased neuroimaging use is associated with increased brain arteriovenous malformation (BAVM) detection, we examined detection rates in the Kaiser Permanente Medical Care Program of northern California between 1995 and 2004. We reviewed medical records, radiology reports, and administrative databases to identify BAVMs, intracranial aneurysms (IAs: subarachnoid hemorrhage [SAH] and unruptured aneurysms), and other vascular malformations (OVMs: dural fistulas, cavernous malformations, Vein of Galen malformations, and venous malformations). Poisson regression (with robust standard errors) was used to test for trend. Random-effects meta-analysis generated a pooled measure of BAVM detection rate from 6 studies. We identified 401 BAVMs (197 ruptured, 204 unruptured), 570 OVMs, and 2892 IAs (2079 SAHs and 813 unruptured IAs). Detection rates per 100 000 person-years were 1.4 (95% CI, 1.3 to 1.6) for BAVMs, 2.0 (95% CI, 1.8 to 2.3) for OVMs, and 10.3 (95% CI, 9.9 to 10.7) for IAs. Neuroimaging utilization increased 12% per year during the time period (P<0.001). Overall, rates increased for IAs (P<0.001), remained stable for OVMs (P=0.858), and decreased for BAVMs (P=0.001). Detection rates increased 15% per year for unruptured IAs (P<0.001), with no change in SAHs (P=0.903). However, rates decreased 7% per year for unruptured BAVMs (P=0.016) and 3% per year for ruptured BAVMs (P=0.005). Meta-analysis yielded a pooled BAVM detection rate of 1.3 (95% CI, 1.2 to 1.4) per 100 000 person-years, without heterogeneity between studies (P=0.25). Rates for BAVMs, OVMs, and IAs in this large, multiethnic population were similar to those in other series. During 1995 to 2004, a period of increasing neuroimaging utilization, we did not observe an increased rate of detection of unruptured BAVMs, despite increased detection of unruptured IAs.

  7. Occlusion of an Intraosseous Arteriovenous Malformation With Percutaneous Injection of Polymethylmethacrylate

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Ierardi, Anna Maria, E-mail: amierardi@yahoo.it; Mangini, Monica, E-mail: monica.mangini@tin.it; Vaghi, Massimo, E-mail: vaghim@yahoo.it

    Primary intraosseous arteriovenous malformations are rare. Many minimally invasive procedures can be considered preoperative steps and/or definitive treatment. The case reported regards a young woman with a voluminous arteriovenous extratroncular infiltrating malformation of the humerus. She underwent several treatments, but none of them was completely occlusive. The last treatment consisted of direct percutaneous puncture of the intraosseous alteration and injection of polymethylmethacrylate (PMMA), which is normally used in percutaneous vertebroplasty. We obtained complete occlusion of the humerus lytic lesion. To the best of our knowledge, this represents the first case of intraosseous AVM treated by percutaneous injection of PMMA.

  8. Coiling of a vulvar arterio-venous malformation.

    PubMed

    Van der Woude, Daisy Adriana Annejan; Stegeman, Marjan; Seelen, Jan L

    2011-12-01

    The authors report the case of a 13-year-old girl with a painful vulvar swelling and abnormal vaginal bleeding, increasing in size after trauma. With MRI (GE Signa HDx 1.5 Tesla), it is diagnosed as an arterio-venous malformation arising from the left superior femoral artery. It is treated by embolisation using a coil.

  9. Management of small arteriovenous malformations of the brain.

    PubMed

    Morgan, M K; Davidson, J; Sorby, W; Grinnell, V

    1995-10-01

    A consecutive series of arteriovenous malformations of the brain, no greater than 3 cm in diameter, referred to the author between January 1989 and October 1994 is reviewed. Of these 42 cases 39 underwent surgical excision of the lesion. The surgical group had mortality, 2.5% morbidity and 100% angiographic cure. Surgery in non-eloquent brain (n=21) resulted in no neurological morbidity and surgery in eloquent brain (n=18) resulted in a 5.5% neurological morbidity. The reason for not operating in three cases was elderly age in one, poor neurological condition in one, and involvement of the posterior limb of the internal capsule in one. Arteriovenous malformations with diameters no greater than 3 cm are considered by some to be suitable for treatment by focussed irradiation. However, surgery is likely to remain the more effective and safer mode of treatment for the majority of cases when factoring in the expected complications of focussed irradiation during the latency period. These risks are discussed.

  10. Ultrasound diagnosis and management of acquired uterine enhanced myometrial vascularity/arteriovenous malformations.

    PubMed

    Timor-Tritsch, Ilan E; Haynes, Meagan Campol; Monteagudo, Ana; Khatib, Nizar; Kovács, Sándor

    2016-06-01

    Arteriovenous malformation is a short circuit between an organ's arterial and venous circulation. Arteriovenous malformations are classified as congenital and acquired. In the uterus, they may appear after curettage, cesarean delivery, and myomectomy among others. Their clinical feature is usually vaginal bleeding, which may be severe, if curettage is performed in unrecognized cases. Sonographically on 2-dimensional grayscale ultrasound scanning, the pathologic evidence appears as irregular, anechoic, tortuous, tubular structures that show evidence of increased vascularity when color Doppler is applied. Most of the time they resolve spontaneously; however, if left untreated, they may require involved treatments such as uterine artery embolization or hysterectomy. In the past, uterine artery angiography was the gold standard for the diagnosis; however, ultrasound scanning has diagnosed successfully and helped in the clinical management. Recently, arteriovenous malformations have been referred to as enhanced myometrial vascularities. The purpose of this study was to evaluate the role of transvaginal ultrasound scanning in the diagnosis and treatment of acquired enhanced myometrial vascularity/arteriovenous malformations to outline the natural history of conservatively followed vs treated lesions. This was a retrospective study to assess the presentation, treatment, and clinical pictures of patients with uterine Enhanced myometrial vascularity/arteriovenous malformations that were diagnosed with transvaginal ultrasound scanning. We reviewed both (1) ultrasound data (images, measured dimensions, and Doppler blood flow that were defined by its peak systolic velocity and (2) clinical data (age, reproductive status, clinical presentation, inciting event or procedure, surgical history, clinical course, time intervals that included detection to resolution or detection to treatment, and treatment rendered). The diagnostic criteria were "subjective" with a rich vascular

  11. Histologic change of arteriovenous malformations of the face and scalp after free flap transfer.

    PubMed

    Tark, K C; Chung, S

    2000-07-01

    In three patients with long-standing vascular malformations of the face and scalp, radial forearm free flaps were transferred after a near-total excision of the lesion. All patients had typical high-flow malformations with thrill and bruit. The onset and progression of the malformations were analyzed through clinical and histologic studies. After free flap transfer, the vascular malformations were followed up grossly and histologically for between 4 and 9 years. There was no recurrence of arteriovenous malformation after free flap transfer. The portion of the residual lesion adjacent to the transferred free flap disappeared, and the remaining discoloration also vanished grossly. Histologic comparison of immediate postoperative and 4-month postoperative specimens from the margin and residual lesion using Victoria blue staining showed that the typical preoperative findings for arteriovenous malformation-an intermingling of thick-walled vessels with abundant elastic fibers and thin-walled vessels without elastic fibers-had undergone change, resulting in the disappearance of the thick-walled vessels and leaving only homogeneous, thin-walled vasculature. The highly vascularized free flap, which does not contain abnormal fistulas, impacted the histologic change of the arteriovenous malformation by blocking the vicious cycle of ischemia and anatomic replacement of disfigured skin and subcutaneous tissues.

  12. [Venous and arteriovenous malformations in the head and neck region. Therapeutic options and challenges].

    PubMed

    Eivazi, B; Werner, J A

    2014-01-01

    Venous malformations are the prototype low-flow malformations in the head and neck region. Arteriovenous malformations (AVM) represent the main high-flow malformations. In recent years it has been possible to significantly optimize the therapeutic options for venous malformations. In addition to conventional surgery, laser treatment and sclerotherapy have become established techniques and the importance of embolization with new alcohol-based materials is increasing. AVM are progressive and destructive diseases. Therapy of choice is usually a combined treatment comprising embolization and surgical removal of the arteriovenous nidus. This curative approach is usually possible if diagnosis is made at an early stage. Incomplete embolization or sole ligation of the arterial supply causes progression. There is a clear need for improved therapeutic methods and pharmacotherapeutic approaches.

  13. Pros, cons, and current indications of open craniotomy versus gamma knife in the treatment of arteriovenous malformations and the role of endovascular embolization.

    PubMed

    Surdell, Daniel L; Bhattacharjee, Sumon; Loftus, Christopher M

    2002-06-01

    The successful treatment of an intracranial arteriovenous malformation poses both technical and conceptual problems to the neurosurgeon. Treatment decisions are made in light of current understanding of the natural history of these lesions. It is important to understand the pros, cons and current indication of open craniotomy vs. gamma knife in the treatment of arteriovenous malformations and the role of endovascular embolization. Surgical removal of an arteriovenous malformation is indicated when the operative risk is less than the morbidity and mortality associated with its natural history. The treatment goal of complete angiographic obliteration of arteriovenous malformations is achieved most effectively by microneurosurgery in low-grade lesions. Large lesions frequently require a combination of embolization and microsurgery. Although recent advances in technology and medical management have allowed previously inoperable arteriovenous malformations to be surgically excised, there is still a small group of arteriovenous malformations that cannot be excised safely due to their size and location. Stereotactic radiosurgery is clearly an important adjunct in the multimodality treatment approach for large arteriovenous malformations. Endovascular embolization can potentially increase safety and efficacy in the treatment of arteriovenous malformations when applied to selective cases with well-defined treatment goals.

  14. Diagnosis and Nonsurgical Management of Uterine Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Rangarajan, R. D.; Moloney, J. C.; Anderson, H. J.

    Uterine arteriovenous malformation (AVM) is an uncommon problem and traditional treatment by hysterectomy excludes the possibility of future pregnancy. Developments in interventional techniques make transcatheter embolization of the feeding vessel(s) a therapeutic alternative, potentially preserving the patient's fertility. We present a case of successful endovascular treatment of uterine AVM.

  15. Linear accelerator radiosurgery for arteriovenous malformations: Updated literature review.

    PubMed

    Yahya, S; Heyes, G; Nightingale, P; Lamin, S; Chavda, S; Geh, I; Spooner, D; Cruickshank, G; Sanghera, P

    2017-04-01

    Arteriovenous malformations (AVMs) are the leading causing of intra-cerebral haemorrhage. Stereotactic radiosurgery (SRS) is an established treatment for arteriovenous malformations (AVM) and commonly delivered using Gamma Knife within dedicated radiosurgery units. Linear accelerator (LINAC) SRS is increasingly available however debate remains over whether it offers an equivalent outcome. The aim of this project is to evaluate the outcomes using LINAC SRS for AVMs used within a UK neurosciences unit and review the literature to aid decision making across various SRS platforms. Results have shown comparability across platforms and strongly supports that an adapted LINAC based SRS facility within a dynamic regional neuro-oncology department delivers similar outcomes (in terms of obliteration and toxicity) to any other dedicated radio-surgical platform. Locally available facilities can facilitate discussion between options however throughput will inevitably be lower than centrally based dedicated national radiosurgery units. Copyright © 2016. Published by Elsevier Ltd.

  16. Correlation of pressure measurements with angiographic characteristics predisposing to hemorrhage and steal in cerebral arteriovenous malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Norbash, A.M.; Marks, M.P.; Lane, B.

    1994-05-01

    To determine whether there is a physiologic explanation for the predisposition of patients with certain angiographic characteristics to symptoms of hemorrhage and steal. Superselective transcatheter feeding arterial pressure and mean arterial pressure measurements were obtained before embolotherapy in 32 patients with cerebral arteriovenous malformations. Pressures were correlated with previously described angioarchitectural characteristics predisposing to hemorrhage and steal. These included size of the arteriovenous malformation, feeding artery length, venous drainage pattern, and angiomatous change. The feeding arterial pressure and feeding arterial pressure/mean arterial pressure ratios were significantly decreased in patients with angiomatous change. Feeding arterial pressure and feeding arterial pressure/mean arterialmore » pressure ratios progressively decreased as lesions went from peripheral, to mixed, to central venous drainage. A trend for lower feeding arterial pressure was also demonstrated with greater feeding pedicle length. A statistically significant correlation could not be demonstrated between feeding arterial pressure or feeding arterial pressure/mean arterial pressure ratios and size of the arteriovenous malformation, hemorrhage, or symptoms of steal. Feeding arterial pressure measurements help provide a physiologic basis for the relationship between certain angiographic characteristics and hemorrhage and steal symptoms in patients with arteriovenous malformation. 27 refs., 1 fig.« less

  17. Congenital intrahepatic arterioportal and portosystemic venous fistulae with jejunal arteriovenous malformation depicted on multislice spiral CT.

    PubMed

    Chae, Eun Jin; Goo, Hyun Woo; Kim, Seong-Chul; Yoon, Chong Hyun

    2004-05-01

    We report a symptomatic infant with very rare congenital arterioportal and portosystemic venous fistulae in the liver. Multislice CT after partial transcatheter embolisation revealed not only the complicated vascular architecture of the lesion, but also an incidental jejunal arteriovenous malformation which explained the patient's melena. The patient underwent ligation of the hepatic artery and resection of the jejunal arteriovenous malformation. Postoperative multislice CT clearly demonstrated the success of the treatment.

  18. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon.

    PubMed

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2013-03-22

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM.

  19. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon.

    PubMed

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2014-04-01

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM.

  20. Measurement of Blood Flow in Arteriovenous Malformations before and after Embolization Using Arterial Spin Labeling

    PubMed Central

    Suazo, L.; Foerster, B.; Fermin, R.; Speckter, H.; Vilchez, C.; Oviedo, J.; Stoeter, P.

    2012-01-01

    Summary The assessment of shunt reduction after an embolization of an arteriovenous malformation (AVM) or fistula (AVF) from conventional angiography is often difficult and may be subjective. Here we present a completely non-invasive method using magnetic resonance imaging (MRI) to measure shunt reduction. Using pulsed arterial spin labeling (PASL), we determined the relative amount of signal attributed to the shunt over 1.75 s and 6 different slices covering the lesion. This amount of signal from the shunt was related to the total signal from all slices and measured before and after embolization. The method showed a fair agreement between the PASL results and the judgement from conventional angiography. In the case of a total or subtotal shunt occlusion, PASL showed a shunt reduction between 69% and 92%, whereas in minimal shunt reduction as judged by conventional angiography, the ASL result was –6% (indicating slightly increased flow) to 35% in a partially occluded vein of Galen aneurysm. The PASL method proved to be fairly reproducible (up to 2% deviation between three measurements without interventions). On conclusion, PASL is able to reliably measure the amount of shunt reduction achieved by embolization of AVMs and AVFs PMID:22440600

  1. Paraspinal arteriovenous malformation Onyx embolization via an Ascent balloon

    PubMed Central

    Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F

    2013-01-01

    Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM. PMID:23524491

  2. Unruptured Brain Arteriovenous Malformations: Primary ONYX Embolization in ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations)-Eligible Patients.

    PubMed

    Singfer, Uri; Hemelsoet, Dimitri; Vanlangenhove, Peter; Martens, Frederic; Verbeke, Luc; Van Roost, Dirk; Defreyne, Luc

    2017-12-01

    In light of evidence from ARUBA (A Randomized Trial of Unruptured Brain Arteriovenous Malformations), neurovascular specialists had to reconsider deliberate treatment of unruptured brain arteriovenous malformations (uBAVMs). Our objective was to determine the outcomes of uBAVM treated with primary embolization using ethylene vinyl alcohol (ONYX). Patients with uBAVM who met the inclusion criteria of ARUBA and were treated with primary Onyx embolization were assigned to this retrospective study. The primary outcome was the modified Rankin Scale score. Secondary outcomes were stroke or death because of uBAVM or intervention and uBAVM obliteration. Sixty-one patients (mean age, 38 years) were included. The median observation period was 60 months. Patients were treated by embolization alone (41.0%), embolization and radiosurgery (57.4%), or embolization and excision (1.6%). Occlusion was achieved in 44 of 57 patients with completed treatment (77.2%). Forty-seven patients (77.1%) had no clinical impairment at the end of observation (modified Rankin Scale score of <2). Twelve patients (19.7%) reached the outcome of stroke or death because of uBAVM or intervention. Treatment-related mortality was 6.6% (4 patients). In uBAVM, Onyx embolization alone or combined with stereotactic radiosurgery achieves a high occlusion rate. Morbidity remains a challenge, even if it seems lower than in the ARUBA trial. © 2017 American Heart Association, Inc.

  3. Long-Term Follow-up After Embolization of Pulmonary Arteriovenous Malformations with Detachable Silicone Balloons

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Andersen, Poul Erik; Kjeldsen, Anette D.

    2008-05-15

    Long-term follow-up results after embolization of 13 pulmonary arteriovenous malformations in 10 patients by use of 14 detachable silicone balloons are given. Patients were followed for a mean of 99 months (range, 63-123 months) with chest x-rays and for a mean of 62 months (range, 3-101 months) with pulmonary angiography. Fifty-four percent of the balloons were deflated at latest radiographic chest film follow-up, but at pulmonary angiographic follow-up all embolized malformations were without flow irrespective of whether or not the balloons were visible. Detachable silicone balloons are not available anymore, but use of these balloons for embolization of pulmonary arteriovenousmore » malformations has been shown to be a safe and precise method, with immediate occlusion of the feeding artery and with long-lasting occlusion, even though many balloons deflate with time, leaving a fibrotic scar replacing the pulmonary arteriovenous malformation. No case of recanalization has been discovered, and these results seem to justify a reduced number of controls of these balloon-embolized malformations.« less

  4. Imaging Characteristics of Dural Arteriovenous Fistulas Involving the Vein of Galen: A Comprehensive Review.

    PubMed

    Kassem, Mohammad W; Choi, Paul J; Iwanaga, Joe; Mortazavi, Martin M; Tubbs, R Shane

    2018-02-11

    Vein of Galen aneurysmal malformation (VGAM) is a rare angiopathy, which most commonly presents in infancy. Although very rare, it is associated with high morbidity and mortality rates. In order to minimize such morbid rates, a prompt diagnosis followed by a timely initiation of management is crucial. Multiple antenatal and postnatal imaging techniques for the diagnosis have been described and discussed in the literature. However, to our knowledge, a comprehensive review exploring such a list of imaging options for VGAM has never been established. We aim to review the diagnostic tools to aid in better understanding of the investigative modalities physicians may choose from when treating patients with a VGAM.

  5. Effect of Embolization Material in the Calculation of Dose Deposition in Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    De la Cruz, O. O. Galvan; Moreno-Jimenez, S.; Larraga-Gutierrez, J. M.

    2010-12-07

    In this work it is studied the impact of the incorporation of high Z materials (embolization material) in the dose calculation for stereotactic radiosurgery treatment for arteriovenous malformations. A statistical analysis is done to establish the variables that may impact in the dose calculation. To perform the comparison pencil beam (PB) and Monte Carlo (MC) calculation algorithms were used. The comparison between both dose calculations shows that PB overestimates the dose deposited. The statistical analysis, for the quantity of patients of the study (20), shows that the variable that may impact in the dose calculation is the volume of themore » high Z material in the arteriovenous malformation. Further studies have to be done to establish the clinical impact with the radiosurgery result.« less

  6. Progressive regression of intracranial arteriovenous malformations after Onyx embolization.

    PubMed

    Kocur, Damian; Przybyłko, Nikodem; Hofman, Mariusz; Jamróz, Tomasz; Doleżych, Hanna; Baron, Jan; Kwiek, Stanisław

    Progressive regression of cerebral arteriovenous malformations (AVMs) is a rare phenomenon that may occur spontaneously or after previous surgical or endovascular incomplete obliteration. We present two cases of AVMs occluded partially with Onyx followed by the unexpected cure of the lesions with the angiographic evidences as well as multiannual follow-up. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  7. Clinical and angioarchitectural factors influencing the endovascular approach to galenic dural arteriovenous fistulas in adults: case series and review of the literature.

    PubMed

    Cohen, José E; Gomori, John Moshe; Rajz, Gustavo; Paldor, Iddo; Moscovici, Samuel; Itshayek, Eyal

    2017-05-01

    Galenic dural arteriovenous fistulas (DAVF) are rare; however, they are the most frequent type of DAVF to manifest aggressive clinical behavior and usually represent a diagnostic and therapeutic challenge for clinicians. We retrospectively reviewed clinical and imaging data of patients managed with neuroendovascular techniques for the treatment of galenic DAVFs from 2000 to 2016. We searched the 2000-2016 English-language literature for papers discussing neuroendovascular management of galenic DAVFs, with or without companion surgical procedures. Five patients were treated for galenic DAVFs during the study period (four males; mean age, 61 years). Three presented with progressive neurological deterioration due to venous congestion, two with acute intracranial hemorrhage. Three were treated by staged transarterial embolization procedures (three procedures in two, four procedures in one); two underwent a single transvenous embolization procedure. Four out of five fistulas were completely occluded. All patients improved clinically; the patient whose fistula was partially occluded remains angiographically stable at 2-year follow-up. Six reports describing 17 patients are reviewed. Embolization was performed via transvenous approach in 1/17 and transarterial approach in 16/17 with additional open surgery in 9/16. The trend toward the use of transarterial approaches is based primarily on advances on embolization techniques that allow better and more controllable penetration of the embolizing agents with improved clinical and angiographic results, as well as the technical complexity of the transvenous approach. Although transarterial embolization is the preferred endovascular route for the management of most galenic DAVFs, selected cases can be successfully treated by transvenous approach.

  8. Imaging Characteristics of Dural Arteriovenous Fistulas Involving the Vein of Galen: A Comprehensive Review

    PubMed Central

    Kassem, Mohammad W; Iwanaga, Joe; Mortazavi, Martin M; Tubbs, R. Shane

    2018-01-01

    Vein of Galen aneurysmal malformation (VGAM) is a rare angiopathy, which most commonly presents in infancy. Although very rare, it is associated with high morbidity and mortality rates. In order to minimize such morbid rates, a prompt diagnosis followed by a timely initiation of management is crucial. Multiple antenatal and postnatal imaging techniques for the diagnosis have been described and discussed in the literature. However, to our knowledge, a comprehensive review exploring such a list of imaging options for VGAM has never been established. We aim to review the diagnostic tools to aid in better understanding of the investigative modalities physicians may choose from when treating patients with a VGAM. PMID:29657906

  9. Small Arteriovenous Malformation of the Common Bile Duct Causing Hemobilia in a Patient with Hereditary Hemorrhagic Telangiectasia

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hayashi, Sadao, E-mail: hayashi@m.kufm.kagoshima-u.ac.jp; Baba, Yasutaka; Ueno, Kazuto

    We report a 54-year-old male patient with arteriovenous malformation located at the common bile duct and hereditary hemorrhagic telangiectasia. The patient was treated as gallstone pancreatitis at first. Three days after endoscopic nasobiliary drainage (ENBD) for biliary drainage to subside gallstone pancreatitis, hemobilia was drained from the ENBD tube and the serum hemoglobin level gradually decreased. Cholangioscopy and angiography revealed that hemobilia was due to a small arteriovenous malformation located at the common bile duct. Subsequently, the patient was successfully treated by endovascular intervention.

  10. Radiosurgery for cerebral arteriovenous malformations in hereditary hemorrhagic telangiectasia.

    PubMed

    Maarouf, M; Runge, M; Kocher, M; Zähringer, M; Treuer, H; Sturm, V

    2004-07-27

    The authors evaluated the efficacy of radiosurgery (RS) for cerebral arteriovenous malformations in hereditary hemorrhagic telangiectasia (HHT AVMs). Two patients with seven HHT AVMs were treated by linear accelerator-RS. Complete obliteration was achieved 18 to 24 months post-treatment without side effects. Because HHT AVMs are small and multiple, RS is superior to microsurgery because it is noninvasive and all AVMs can be treated in one session regardless of their location.

  11. RBPJ and EphrinB2 as Molecular Targets to Treat Brain Arteriovenous Malformation in Notch4 Induced Mouse Model

    DTIC Science & Technology

    2017-10-01

    mouse genetic breeding, provided genotyping, immunostaining, histological analysis, and molecular expertise. Funding Support NIH/NHLBI Name: Bert...AWARD NUMBER: W81XWH-16-1-0665 TITLE: RBPJ and EphrinB2 as Molecular Targets to Treat Brain Arteriovenous Malformation in Notch4-Induced Mouse...2016 - 29 Sep 2017 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER RBPJ and EphrinB2 as Molecular Targets to Treat Brain Arteriovenous Malformation in

  12. Challenges in the management of massive intraorbital and hemifacial arteriovenous malformation as causing life-threatening epistaxis.

    PubMed

    Manuel, Anura Michelle; Kalimuthu, Santhi; Pathmanathan, Sitra Siri; Narayanan, Prepageran; Zainal Abidin, Zurina; Azmi, Khairul; Khalil, Alizan

    2017-04-01

    Arteriovenous malformations are congenital lesions that may evolve with time and manifest in a plethora of presentations. They can occur as torrential epistaxis when it extensively involves the facial region. Multi-imaging modalities are available to assist in characterizing the structure of the lesion as well as its location and extent. This complex disease requires a multidisciplinary team approach with preoperative embolization and surgery. We present a rare cause of life-threatening epistaxis in a gentleman with a longstanding orbital and hemifacial arteriovenous malformation and discuss the complexities involved in its management. Copyright © 2017. Published by Elsevier Taiwan.

  13. Hereditary hemorrhagic telangiectasia patient presenting with brain abscess due to silent pulmonary arteriovenous malformation.

    PubMed

    Themistocleous, Marios; Giakoumettis, Dimitrios; Mitsios, Andreas; Anagnostopoulos, Christos; Kalyvas, Aristoteles; Koutsarnakis, Christos

    2016-01-01

    Hereditary hemorrhagic telangiectasia is a rare autosomal dominant inherited disease that is usually complicated by visceral vascular malformations. Patients harboring such malformations are at increased risk of brain abscess formation, which despite advances in diagnostic and surgical methods remains a life threatening medical emergency with high mortality and morbidity rates. In the present report we describe a case of cerebral abscess due to silent pulmonary arteriovenous malformation (AVM) in a young patient previously undiagnosed for hereditary hemorrhagic telangiectasia syndrome (HHT).

  14. Novel Image-Guided Management of a Uterine Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Przybojewski, Stefan J., E-mail: drstefanp@hotmail.com; Sadler, David J.

    The investigators present a novel image-guided embolization, not previously described, of a uterine arteriovenous malformation (AVM) resistant to endovascular management. The uterus was exposed surgically, and Histoacryl (Braun, Fulda, Germany) was injected directly into the nidus using ultrasound guidance and fluoroscopy. The patient had a successful full-term pregnancy after this procedure. This technique may be a useful alternative management strategy in patients with uterine AVM who fail traditional endovascular embolization and who still desire fertility.

  15. Role of stereotactic radiosurgery with a linear accelerator in treatment of intracranial arteriovenous malformations and tumors in children.

    PubMed

    Loeffler, J S; Rossitch, E; Siddon, R; Moore, M R; Rockoff, M A; Alexander, E

    1990-05-01

    Between 1986 and 1988, 16 children were treated for 10 arteriovenous malformations and 6 recurrent intracranial tumors with stereotactic radiation therapy using a modified Clinac 6/100 linear accelerator. The median age of our patients was 10.5 years. For the group with arteriovenous malformation, follow-up ranged from 6 months to 37 months (median was 20 months). No patient bled during the follow-up period. Five of eight patients with follow-up longer than 12 months have achieved complete obliteration of their arteriovenous malformation by angiogram. The four remaining patients who have not achieved a complete obliteration are awaiting their 2-year posttreatment angiogram. The other patient has been treated within the year and have not yet been studied. Five of the six recurrent tumor patients are alive with a median follow-up of 8 months. The remaining patient was controlled locally, but he died of recurrent disease outside the area treated with radiosurgery. The radiographic responses of these patients have included three complete responses, two substantial reductions in tumor volume (greater than 50%) and one stabilization. Despite previous radiotherapy, there have been no significant complications in these patients. We conclude that stereotactic radiation therapy using a standard linear accelerator is an effective and safe technique in the treatment of selected intracranial arteriovenous malformations and tumors in children. In addition, stereotactic radiosurgery may have unique applications in the treatment of localized primary and recurrent pediatric brain tumors.

  16. Stereotactic radiosurgery for deep intracranial arteriovenous malformations, part 1: Brainstem arteriovenous malformations.

    PubMed

    Cohen-Inbar, Or; Ding, Dale; Chen, Ching-Jen; Sheehan, Jason P

    2016-02-01

    The management of brainstem arteriovenous malformations (AVM) are one of the greatest challenges encountered by neurosurgeons. Brainstem AVM have a higher risk of hemorrhage compared to AVM in other locations, and rupture of these lesions commonly results in devastating neurological morbidity and mortality. The potential morbidity associated with currently available treatment modalities further compounds the complexity of decision making for affected patients. Stereotactic radiosurgery (SRS) has an important role in the management of brainstem AVM. SRS offers acceptable obliteration rates with lower risks of hemorrhage occurring during the latency period. Complex nidal architecture requires a multi-disciplinary treatment approach. Nidi partly involving subpial/epipial regions of the dorsal midbrain or cerebellopontine angle should be considered for a combination of endovascular embolization, micro-surgical resection and SRS. Considering the fact that incompletely obliterated lesions (even when reduced in size) could still cause lethal hemorrhages, additional treatment, including repeat SRS and surgical resection should be considered when complete obliteration is not achieved by first SRS. Patients with brainstem AVM require continued clinical and radiological observation and follow-up after SRS, well after angiographic obliteration has been confirmed. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Computational modelling for the embolization of brain arteriovenous malformations.

    PubMed

    Orlowski, Piotr; Summers, Paul; Noble, J Alison; Byrne, James; Ventikos, Yiannis

    2012-09-01

    Treatment of arteriovenous malformations (AVMs) of the brain often requires the injection of a liquid embolic material to reduce blood flow through the malformation. The type of the liquid and the location of injection have to be carefully planned in a pre-operative manner. We introduce a new model of the interaction of liquid embolic materials with blood for the simulation of their propagation and solidification in the AVM. Solidification is mimicked by an increase of the material's viscosity. Propagation is modelled by using the concept of two-fluids modelling and that of scalar transport. The method is tested on digital phantoms and on one anatomically derived patient AVM case. Simulations showed that intuitive behaviour of the two-fluid system can be confirmed and that two types of glue propagation through the malformation can be reproduced. Distinction between the two types of propagation could be used to identify fistulous and plexiform compartments composing the AVM and to characterize the solidification of the embolic material in them. Copyright © 2011 IPEM. Published by Elsevier Ltd. All rights reserved.

  18. Embolization of Uterine Arteriovenous Malformations Associated with Cyanotic Congenital Heart Disease

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Wijesekera, N. T., E-mail: n.wijesekera@doctors.net.uk; Padley, S. P.; Kazmi, F.

    2009-09-15

    Uterine arteriovenous malformation (AVM) is a rare cause of vaginal bleeding and miscarriage. We report two cases of uterine AVMs in patients with a history of complex congenital heart disease, an association that has not been previously described. Both patients were treated by selective uterine artery embolization, a minimally invasive therapy that has revolutionized the management of uterine AVMs, thus offering an alternative to conventional hysterectomy.

  19. Congenital supratentorial meningeal arteriovenous malformation with hemangioma and massive arachnoid cell hyperplasia.

    PubMed

    Nabeel, Alnaghmoosh; Lach, Boleslaw; Al-Shail, Essam; Patay, Zoltan

    2005-11-01

    We describe the clinical, radiological and pathological findings of concurrent, congenital leptomeningeal arteriovenous malformation with hemangioma diagnosed in a newborn by prenatal and immediately postnatal magnetic resonance imaging. Vascular abnormalities were accompanied by massive arachnoidal cell hyperplasia reminiscent of meningioma. To the best of our knowledge, this is the first case of such a lesion reported in the literature.

  20. A Case Report of Onyx Pulmonary Arterial Embolism Contributing to Hypoxemia During Awake Craniotomy for Arteriovenous Malformation Resection.

    PubMed

    Tolly, Brian T; Kosky, Jenna L; Koht, Antoun; Hemmer, Laura B

    2017-02-15

    A healthy 26-year-old man with cerebral arteriovenous malformation underwent staged endovascular embolization with Onyx followed by awake craniotomy for resection. The perioperative course was complicated by tachycardia and severe intraoperative hypoxemia requiring significant oxygen supplementation. Postoperative chest computed tomography (CT) revealed hyperattenuating Onyx embolization material within the pulmonary vasculature, and an electrocardiogram indicated possible right heart strain, supporting clinically significant embolism. With awake arteriovenous malformation resection following adjunctive Onyx embolization becoming increasingly employed for lesions involving the eloquent cortex, anesthesiologists need to be aware of pulmonary migration of Onyx material as a potential contributor to significant perioperative hypoxemia.

  1. Renal arteriovenous malformation: an unusual cause of recurrent haematuria

    PubMed Central

    Ali, Mazhar; Aziz, Wajahat; Abbas, Farhat

    2013-01-01

    A 54-year-old woman presented with gross painless haematuria. Initial workup showed no abnormality except mild hydronephrosis on CT scan. Cystoscopy and retrograde pyelography did not find any gross lesion and her urine cytology was also negative. She had recurrent haematuria so her CT was reviewed with the radiologist with clinical suspicion of arteriovenous malformation (AVM) which was suggested by relatively increased contrast density in the hemiazygous vein and renal vein in the arterial phase. She underwent angioembiolisation of left renal AVM after which her haematuria settled. PMID:23867881

  2. Untreated brain arteriovenous malformation

    PubMed Central

    Al-Shahi Salman, Rustam; McCulloch, Charles E.; Stapf, Christian; Young, William L.

    2014-01-01

    Objective: To identify risk factors for intracranial hemorrhage in the natural history course of brain arteriovenous malformations (AVMs) using individual patient data meta-analysis of 4 existing cohorts. Methods: We harmonized data from Kaiser Permanente of Northern California (n = 856), University of California San Francisco (n = 787), Columbia University (n = 672), and the Scottish Intracranial Vascular Malformation Study (n = 210). We censored patients at first treatment, death, last visit, or 10-year follow-up, and performed stratified Cox regression analysis of time-to-hemorrhage after evaluating hemorrhagic presentation, sex, age at diagnosis, deep venous drainage, and AVM size as predictors. Multiple imputation was performed to assess impact of missing data. Results: A total of 141 hemorrhage events occurred during 6,074 patient-years of follow-up (annual rate of 2.3%, 95% confidence interval [CI] 2.0%–2.7%), higher for ruptured (4.8%, 3.9%–5.9%) than unruptured (1.3%, 1.0%–1.7%) AVMs at presentation. Hemorrhagic presentation (hazard ratio 3.86, 95% CI 2.42–6.14) and increasing age (1.34 per decade, 1.17–1.53) independently predicted hemorrhage and remained significant predictors in the imputed dataset. Female sex (1.49, 95% CI 0.96–2.30) and exclusively deep venous drainage (1.60, 0.95–2.68, p = 0.02 in imputed dataset) may be additional predictors. AVM size was not associated with intracerebral hemorrhage in multivariable models (p > 0.5). Conclusion: This large, individual patient data meta-analysis identified hemorrhagic presentation and increasing age as independent predictors of hemorrhage during follow-up. Additional AVM cohort data may further improve precision of estimates, identify new risk factors, and allow validation of prediction models. PMID:25015366

  3. High-Flow Arteriovenous Malformation of the Lower Extremity: Ethanolamine Oleate Sclerotherapy

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hyodoh, Hideki; Fujita, Akifumi; Hyodoh, Kazusa

    2001-09-15

    We report the case of a young man presenting with high-flow arteriovenous malformation (AVM), in whom percutaneous direct nidus puncture ethanolamine oleate (EO) sclerotherapy was useful in the management of the AVM. To our knowledge, this is the first report of percutaneous trans-nidus EO sclerotherapy for AVM in the extremities. Percutaneous trans-nidus sclerotherapy should be considered as an alternative choice for the management of symptomatic AVM.

  4. Transcatheter embolization of pancreatic arteriovenous malformation associated with recurrent acute pancreatitis

    PubMed Central

    Rajesh, S; Mukund, Amar; Bhatia, Vikram; Arora, Ankur

    2016-01-01

    Pancreatic arteriovenous malformation (PAVM) is extremely rare; even rarer is its association with pancreatitis. The authors report a case of PAVM causing recurrent episodes of acute pancreatitis in a 46-year-old male. Patient refused surgery and was treated with transcatheter arterial embolization using liquid embolic agent (mixture of n-butyl-2-cyanoacrylate glue and lipiodol), which resulted in a significant decrease in the size of the PAVM. PMID:27081231

  5. Is a Swine Model of Arteriovenous Malformation Suitable for Human Extracranial Arteriovenous Malformation? A Preliminary Study

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lv, Ming-ming, E-mail: lvmingming001@163.com; Fan, Xin-dong, E-mail: fanxindong@yahoo.com.cn; Su, Li-xin, E-mail: sulixin1975@126.com

    Objective: A chronic arteriovenous malformation (AVM) model using the swine retia mirabilia (RMB) was developed and compared with the human extracranial AVM (EAVM) both in hemodynamics and pathology, to see if this brain AVM model can be used as an EAVM model. Methods: We created an arteriovenous fistula between the common carotid artery and the external jugular vein in eight animals by using end-to-end anastomosis. All animals were sacrificed 1 month after surgery, and the bilateral retia were obtained at autopsy and performed hematoxylin and eosin staining and immunohistochemistry. Pre- and postsurgical hemodynamic evaluations also were conducted. Then, the bloodmore » flow and histological changes of the animal model were compared with human EAVM. Results: The angiography after operation showed that the blood flow, like human EAVM, flowed from the feeding artery, via the nidus, drained to the draining vein. Microscopic examination showed dilated lumina and disrupted internal elastic lamina in both RMB of model and nidus of human EAVM, but the thickness of vessel wall had significant difference. Immunohistochemical reactivity for smooth muscle actin, angiopoietin 1, and angiopoietin 2 were similar in chronic model nidus microvessels and human EAVM, whereas vascular endothelial growth factor was significant difference between human EAVM and RMB of model. Conclusions: The AVM model described here is similar to human EAVM in hemodynamics and immunohistochemical features, but there are still some differences in anatomy and pathogenetic mechanism. Further study is needed to evaluate the applicability and efficacy of this model.« less

  6. Congenital Arteriovenous Malformation of the Scalp Involving the Orbit.

    PubMed

    Feletti, Alberto; Dimitriadis, Stavros; Vallone, Stefano; Pavesi, Giacomo

    2018-06-15

    Arteriovenous malformations (AVMs) of the scalp are rare and infrequently encountered by the neurosurgeon.  We report a unique case of a 42-year-old patient who presented with a progressive worsening of visual acuity in the right eye (lower quadrantanopia) and palpebral ptosis. Physical examination revealed a right exophthalmos and a right frontoparietal scalp soft swelling when the patient was in the supine position. Neurologic work-up showed a scalp AVM extending into the orbit and connected to an intraorbital cavernous angioma. The patient was treated with a frontotemporal craniotomy and decompression of the orbit.  In the rare case of intraorbital extension of a scalp AVM, neurologic symptoms may appear when the size of the vascular malformation increases with age. The aims of surgery should be decompression of the orbit and aesthetic preservation, rather than complete excision. A review of the literature is also provided. Georg Thieme Verlag KG Stuttgart · New York.

  7. Ischaemic Strokes in Patients with Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia: Associations with Iron Deficiency and Platelets

    PubMed Central

    Shovlin, Claire L.; Chamali, Basel; Santhirapala, Vatshalan; Livesey, John A.; Angus, Gillian; Manning, Richard; Laffan, Michael A.; Meek, John; Tighe, Hannah C.; Jackson, James E.

    2014-01-01

    Background Pulmonary first pass filtration of particles marginally exceeding ∼7 µm (the size of a red blood cell) is used routinely in diagnostics, and allows cellular aggregates forming or entering the circulation in the preceding cardiac cycle to lodge safely in pulmonary capillaries/arterioles. Pulmonary arteriovenous malformations compromise capillary bed filtration, and are commonly associated with ischaemic stroke. Cohorts with CT-scan evident malformations associated with the highest contrast echocardiographic shunt grades are known to be at higher stroke risk. Our goal was to identify within this broad grouping, which patients were at higher risk of stroke. Methodology 497 consecutive patients with CT-proven pulmonary arteriovenous malformations due to hereditary haemorrhagic telangiectasia were studied. Relationships with radiologically-confirmed clinical ischaemic stroke were examined using logistic regression, receiver operating characteristic analyses, and platelet studies. Principal Findings Sixty-one individuals (12.3%) had acute, non-iatrogenic ischaemic clinical strokes at a median age of 52 (IQR 41–63) years. In crude and age-adjusted logistic regression, stroke risk was associated not with venous thromboemboli or conventional neurovascular risk factors, but with low serum iron (adjusted odds ratio 0.96 [95% confidence intervals 0.92, 1.00]), and more weakly with low oxygen saturations reflecting a larger right-to-left shunt (adjusted OR 0.96 [0.92, 1.01]). For the same pulmonary arteriovenous malformations, the stroke risk would approximately double with serum iron 6 µmol/L compared to mid-normal range (7–27 µmol/L). Platelet studies confirmed overlooked data that iron deficiency is associated with exuberant platelet aggregation to serotonin (5HT), correcting following iron treatment. By MANOVA, adjusting for participant and 5HT, iron or ferritin explained 14% of the variance in log-transformed aggregation-rate (p = 0.039/p = 0

  8. Dynamic contrast-enhanced MRA at 1.5 T for detection of arteriovenous shunting before and after Onyx embolization of cerebral arteriovenous malformations.

    PubMed

    Nogueira, Raul G; Bayrlee, Ahmad; Hirsch, Joshua A; Yoo, Albert J; Copen, William A

    2013-10-01

    Conventional non-invasive angiographic techniques for evaluating cerebral Arteriovenous Malformations (cAVMs) after embolization treatment are limited by their inability to acquire time-resolved images. We describe the use of dynamic contrast-enhanced magnetic resonance angiography (MRA) in the evaluation of residual arteriovenous shunting in cAVMs following Onyx embolization. Six subjects who underwent multimodal MR imaging including dynamic MRA after different stages of endovascular treatment with Onyx were included. Each MRA was assessed for the presence of residual arteriovenous shunting. The results were compared with digital subtraction angiography (DSA). Mean age was 41 years (range, 25-63) and the mean maximum AVM diameter was 5.3 cm (range, 4.7-6.0). Fourteen dynamic MRA were performed using a 1.5 T scanner. Arteriovenous shunting was detected in thirteen of fourteen patients by both dynamic MRA and DSA, with complete agreement between the two techniques. The only MRA without detectable residual arteriovenous shunting was for a subject who had complete treatment with no residual cAVM as confirmed by the DSA images. Dynamic contrast-enhanced MRA is a promising non-invasive modality in identifying residual arteriovenous shunting after different stages of AVM embolization, achieving 100% agreement in this small study. Embolization with Onyx caused no significant image artifact. Copyright © 2013 by the American Society of Neuroimaging.

  9. Stereotactic radiosurgery with the linear accelerator: treatment of arteriovenous malformations.

    PubMed

    Betti, O O; Munari, C; Rosler, R

    1989-03-01

    An original stereotactic radiosurgical approach coupling a) Talairach's stereotactic methodology, b) a specially devised mechanical system, and c) a linear accelerator is detailed. The authors present their preliminary results on 66 patients with nonsurgical intracranial arteriovenous malformations. The doses delivered for treatment varied from 20 to 70 Gy. Doses of no more than 40 Gy were used in 80% of patients. An angiographic study was performed when the computed tomographic scan controls showed relevant modifications of the lesion volume. Total obliteration was obtained in 27 of the 41 patients (65.8%) who were followed up for at least 24 months. The percentage of the cured patients is significantly higher when a) the entire malformation is included in the 75% isodose (96%) and b) the maximum diameter of the lesion is less than 12 mm (81%). Two patients died of rebleeding at 18 and 29 months after treatment.

  10. Brain Arteriovenous Malformation Pathogenesis: A Response-to-Injury Paradigm

    PubMed Central

    Kim, Helen; Su, Hua; Weinsheimer, Shantel; Pawlikowska, Ludmila; Young, William L.

    2011-01-01

    Brain arteriovenous malformations (AVMs) are a rare but important cause of intracranial hemorrhage (ICH) in young adults. In this paper, we review both human and animal studies of brain AVM, focusing on the: (1) natural history of AVM hemorrhage; (2) genetic and expression studies of AVM susceptibility and hemorrhage; and (3) strategies for development of a brain AVM model in adult mice. These data target various mechanisms which must act in concert to regulate normal angiogenic response to injury. Based on the various lines of evidence reviewed in this paper, we propose a “response-to-injury” model of brain AVM pathogenesis. PMID:21725736

  11. [Emergency cesarean section and craniectomy in a patient with rupture of a cerebral arteriovenous malformation].

    PubMed

    Monsalve-Mejía, G; Palacio, W; Rodríguez, C

    2014-04-01

    The intracerebral hemorrhage in pregnancy is a rare event, but can have catastrophic consequences for both mother and fetus. The management of non-ruptured arteriovenous malformations in pregnancy is not free of controversy in the current literature, as there is the possibility of spontaneous bleeding and becoming a true emergency. We report the case of a pregnant patient of 35 weeks with a diagnosis of a cerebral arteriovenous malformation, who developed a sudden onset of headache, generalized tonic-clonic seizures, loss of consciousness, and hemiparesis with radiological images of an intracranial hematoma with a mass effect, and signs of herniation. The multidisciplinary management is discussed, emphasizing perioperative cesarean approach plus craniotomy and drainage of the hematoma, and subsequent management in intensive care, and definitive management by neuroradiology, with a successful outcome. Copyright © 2012 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  12. Transseptal Guidewire Stabilization for Device Closure of a Large Pulmonary Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Joseph, George, E-mail: joseph59@gmail.com; Kunwar, Brajesh Kumar, E-mail: kunwar_brajesh@yahoo.com

    A 46-year-old man presenting with massive hemoptysis was found to have a large pulmonary arteriovenous malformation (PAVM) in the right lung. Closure of the PAVM with an Amplatzer-type duct occluder was hampered by inability to advance the device delivery sheath into the PAVM due to vessel tortuosity and inadequate guidewire support. Atrial septal puncture was performed and a femorofemoral arteriovenous guidewire loop through the right pulmonary artery, PAVM, and left atrium was created. Traction on both ends of the guidewire loop allowed advancement of the device delivery sheath into the PAVM and successful completion of the procedure. Transseptal guidewire stabilizationmore » can be a valuable option during device closure of large PAVMs when advancement, stability, or kinking of the device delivery sheath is an issue.« less

  13. Ruptured renal arteriovenous malformation successfully treated by catheter embolization: a case report.

    PubMed

    Takeuchi, Nobuhiro; Nomura, Yusuke

    2014-01-09

    Renal arteriovenous fistula (RAVF) is a comparatively rare malformation. Here, we report a case of ruptured RAVF that was successfully treated by catheter embolization. An 89-year-old female was transferred to our institution with massive gross hematuria in March 2011. Plain abdominal computed tomography (CT) revealed dilated left renal pelvis with high-density contents. Hematoma was suspected. Subsequent plain abdominal magnetic resonance imaging revealed left hydronephrosis and blood retention in the dilated left renal pelvis. No renal or ureteral cancer was evident. Hematuria was conservatively treated using hemostatic agents but hematuria persisted. Repeated urinary cytology revealed no malignant cells. On day 9, the patient went into septic and/or hemorrhagic shock. Fluid and catecholamine infusion, blood transfusion, and antibacterial drugs were rapidly initiated, and the patient's general condition gradually improved. Contrast-enhanced abdominal CT revealed marked expansion of the hematoma in the renal pelvis and microaneurysms in the segmental arteries of the left kidney. Inflammation improved, and a left double-J stent was inserted. Selective renal angiography revealed RAVF with microaneurysms in the left segmental arteries; therefore, catheter embolization using metallic coils was performed, which resolved hematuria. We report a case of ruptured renal arteriovenous malformation, which was successfully treated by catheter embolization.

  14. Ruptured Aneurysm of Intercostal Arteriovenous Malformation Associated With Neurofibromatosis Type 1: A Case Report

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kim, Hyung Jun; Seon, Hyun Ju, E-mail: sunaura@hanmail.net; Choi, Song

    Intercostal arteriovenous malformations (AVM) are rare, with most being secondary to trauma or iatrogenic therapeutic procedures. Only one case of presumably congenital AVM has been reported. Here we report the first case of a ruptured aneurysm of intercostal AVM associated with neurofibromatosis type 1 in a 32-year-old woman who experienced hypovolemic shock caused by massive hemothorax.

  15. Transcatheter Embolization of a Large Symptomatic Pelvic Arteriovenous Malformation with Glubran 2 Acrylic Glue

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Gandini, R.; Angelopoulos, G., E-mail: giorginos78@msn.com; Konda, D.

    A young patient affected by a pelvic arteriovenous malformation (pAVM) with recurrent episodes of hematuria following exercise, underwent transcatheter embolization using Glubran 2 acrylic glue (GEM, Viareggio, Italy). All branches of the pAVM were successfully occluded. The patient showed prompt resolution of symptoms and persistent occlusion of the pAVM at the 6 month follow-up.

  16. Regression of pulmonary artery hypertension due to development of a pulmonary arteriovenous malformation

    PubMed Central

    Hasan, Ashfaq; Sastry, B.K.S.; Aleem, M.A.; Reddy, Gokul; Mahmood, Syed

    2014-01-01

    Idiopathic Pulmonary Hypertension (IPAH) is characterized by elevated pulmonary arterial pressure in the absence of an identifiable underlying cause. The condition is usually relentlessly progressive with a short survival in the absence of treatment.1 We describe a patient of IPAH in whom the pulmonary artery pressures significantly abated with complete disappearance of symptoms, following spontaneous development of a pulmonary arterio-venous malformation (PAVM). PMID:25443608

  17. Genome-wide association study of sporadic brain arteriovenous malformations.

    PubMed

    Weinsheimer, Shantel; Bendjilali, Nasrine; Nelson, Jeffrey; Guo, Diana E; Zaroff, Jonathan G; Sidney, Stephen; McCulloch, Charles E; Al-Shahi Salman, Rustam; Berg, Jonathan N; Koeleman, Bobby P C; Simon, Matthias; Bostroem, Azize; Fontanella, Marco; Sturiale, Carmelo L; Pola, Roberto; Puca, Alfredo; Lawton, Michael T; Young, William L; Pawlikowska, Ludmila; Klijn, Catharina J M; Kim, Helen

    2016-09-01

    The pathogenesis of sporadic brain arteriovenous malformations (BAVMs) remains unknown, but studies suggest a genetic component. We estimated the heritability of sporadic BAVM and performed a genome-wide association study (GWAS) to investigate association of common single nucleotide polymorphisms (SNPs) with risk of sporadic BAVM in the international, multicentre Genetics of Arteriovenous Malformation (GEN-AVM) consortium. The Caucasian discovery cohort included 515 BAVM cases and 1191 controls genotyped using Affymetrix genome-wide SNP arrays. Genotype data were imputed to 1000 Genomes Project data, and well-imputed SNPs (>0.01 minor allele frequency) were analysed for association with BAVM. 57 top BAVM-associated SNPs (51 SNPs with p<10(-05) or p<10(-04) in candidate pathway genes, and 6 candidate BAVM SNPs) were tested in a replication cohort including 608 BAVM cases and 744 controls. The estimated heritability of BAVM was 17.6% (SE 8.9%, age and sex-adjusted p=0.015). None of the SNPs were significantly associated with BAVM in the replication cohort after correction for multiple testing. 6 SNPs had a nominal p<0.1 in the replication cohort and map to introns in EGFEM1P, SP4 and CDKAL1 or near JAG1 and BNC2. Of the 6 candidate SNPs, 2 in ACVRL1 and MMP3 had a nominal p<0.05 in the replication cohort. We performed the first GWAS of sporadic BAVM in the largest BAVM cohort assembled to date. No GWAS SNPs were replicated, suggesting that common SNPs do not contribute strongly to BAVM susceptibility. However, heritability estimates suggest a modest but significant genetic contribution. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  18. [Melorheostosis associated with arteriovenous malformation of the ear].

    PubMed

    Ingen-Housz-Oro, S; Chigot, V; Hamel-Teillac, D; Brunelle, F; De Prost, Y

    2001-09-01

    Melorheostosis is a rare bone dystrophy that may be associated with various vascular malformations. We report a case of arteriovenous fistulae of the ear associated with melorheostosis limited to the same side of the body. A 13 year-old boy presented a congenital port-wine nevus of the right side of the head complicated by an arteriovenous fistulae and angiomatous nodules of the ear. He was treated by laser, surgery of the nodules, arterial embolisations and sclerotherapy. In 1999, he had a benign trauma of the right hand. The X-ray showed hyperostosis resembling wax flowing down a candle reaching the carpus and some of the metacarpals and the phalanges of the right hand, typical of melorheostosis. The complete radiographic check-up showed the same characteristic appearance on the right side of the skull and the long bones of the right upper limb. Except a deformation of the right fingers, there were no others symptoms. Melorheostosis is a rare, sporadic and benign bone dysplasia that may be localized to a single limb or disseminated. The diagnosis is usually made in late childhood. Pain, stiffness, deformation of a limb are the main clinical manifestations. The skin may be erythematous and sclerotic. The radiographic appearance is characteristic with hyperostosis on one side of the bone resembling wax flowing down a candle. A vascular abnormality is present in 17 p. 100 of cases (hemangiomas, aneurysms, renal artery stenosis.). In these cases, melorheostosis is usually limited to the same side of the vascular lesion. We report the first case of arteriovenous fistulae of the ear associated with melorheostosis, on the same side of the body. The physiopathology of melorheostosis is still unknown but the association with a homolateral vascular abnormality suggests a localized defect in embryogenesis of the vascular and skeletal systems.

  19. Arteriovenous malformation of the vestibulocochlear nerve

    PubMed Central

    Tucker, Adam; Tsuji, Masao; Yamada, Yoshitaka; Hanabusa, Kenichiro; Ukita, Tohru; Miyake, Hiroji; Ohmura, Takehisa

    2015-01-01

    We describe a rare case of an arteriovenous malformation (AVM) embedded in the vestibulocochlear nerve presenting with subarachnoid hemorrhage (SAH) treated by microsurgical elimination of the main feeding artery and partial nidus volume reduction with no permanent deficits. This 70-year-old woman was incidentally diagnosed 4 years previously with two small unruptured tandem aneurysms (ANs) on the right anterior inferior cerebral artery feeding a small right cerebellopontine angle AVM. The patient was followed conservatively until she developed sudden headache, nausea and vomiting and presented to our outpatient clinic after several days. Magnetic resonance imaging demonstrated findings suggestive of early subacute SAH in the quadrigeminal cistern. A microsurgical flow reduction technique via clipping between the two ANs and partial electrocoagulation of the nidus buried within the eighth cranial nerve provided radiographical devascularization of the ANs with residual AVM shunt flow and no major deficits during the 2.5 year follow-up. This is only the second report of an auditory nerve AVM. In the event of recurrence, reoperation or application of alternative therapies may be considered. PMID:26244159

  20. Brainstem arteriovenous malformation presenting with dyspraxic handwriting in a young girl.

    PubMed

    Coppola, Giangennaro; D'Amico, Alessandra; Pironti, Erica; Martino, Federica; Santoro, Elena; Di Paolo, Nilde; Isone, Claudia; Catone, Gennaro

    2014-06-01

    We report the case of a 11-year-old girl who developed an isolated hand-writing disorder with dysgraphia at the beginning of the school year in the sixth grade. A brain magnetic resonance angiography showed a round arteriovenous malformation sited in the left side of the midbrain extending to the ipsilateral medio-basal thalamus. Child neurologists should never neglect a thorough neurological evaluation in case of isolated worsening of handwriting, to rule out possible underlying organic causes. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  1. Cerebral arteriovenous malformation

    MedlinePlus

    AVM - cerebral; Arteriovenous hemangioma; Stroke - AVM; Hemorrhagic stroke - AVM ... The cause of cerebral AVM is unknown. An AVM occurs when arteries in the brain connect directly to nearby veins without having the ...

  2. Acquired uterine arteriovenous malformation developing in retained products of conception: a diagnostic dilemma.

    PubMed

    Goyal, Surbhi; Goyal, Ankur; Mahajan, Surbhi; Sharma, Shikha; Dev, Geeta

    2014-01-01

    Abnormal uterine bleeding in the postabortal period requires meticulous diagnostic work-up to decide proper management. Imaging modalities including Doppler sonography and magnetic resonance imaging in concert with clinical and laboratory findings are useful to narrow the differential diagnoses but are not definitive. Presence of increased uterine vascularity and arteriovenous shunting is non-specific and can be detected in a variety of conditions including retained trophoblastic tissue, gestational trophoblastic disease, arteriovenous malformation (AVM), placental polyp and vascular neoplasm. We present here a case of a multiparous woman with unexplained postabortal bleeding posing a diagnostic challenge. Excluding the possibility of AVM before attempting dilatation and curettage in such a clinical scenario is crucial to prevent catastrophic bleeding. © 2013 The Authors. Journal of Obstetrics and Gynaecology Research © 2013 Japan Society of Obstetrics and Gynecology.

  3. Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single-institution case series.

    PubMed

    Gopalan, Vignesh; Rennie, Adam; Robertson, Fergus; Kanagarajah, Lakshmi; Toolis, Claire; Bhate, Sanjay; Ganesan, Vijeya

    2018-04-01

    To describe presentation, clinical course, and outcome in postneonatal presentations of vein of Galen malformation (VGM). Children older than 28 days presenting with VGM (from 2006-2016) were included. Notes/scans were reviewed. Outcome was dichotomized into 'good' or 'poor' using the Recovery and Recurrence Questionnaire. Logistic regression was performed to explore relationships between clinico-radiological features and outcome. Thirty-one children (18 males, 13 females) were included, presenting at a median age of 9.6 months (range 1.2mo-11y 7mo), most commonly with macrocrania (n=24) and prominent facial veins (n=9). Seven had evidence of cardiac failure. VGM morphology was choroidal in 19. Hydrocephalus (n=24) and loss of white matter volume (n=15) were the most common imaging abnormalities. Twenty-nine patients underwent glue embolization (median two per child). Angiographic shunt closure was achieved in 21 out of 28 survivors. Three children died of intracranial haemorrhage (1y, 6y, and 30d after embolization). Ten patients underwent neurosurgical procedures; to treat haemorrhage in four, and hydrocephalus in the rest. Outcome was categorized as good in 20 out of 28 survivors, but this was not predictable on the basis of the variables listed above. Postneonatally presenting VGM has distinctive clinico-radiological features, attributable to venous hypertension. Endovascular treatment is associated with good outcomes, but more specific prognostic prediction was not possible within this cohort. Clinical and radiological features in older children with vein of Galen malformation relate to venous hypertension. Outcome is good in most cases with endovascular therapy. Mortality is low but is related to intracranial haemorrhage. © 2018 Mac Keith Press.

  4. Endothelial Depletion of Acvrl1 in Mice Leads to Arteriovenous Malformations Associated with Reduced Endoglin Expression

    PubMed Central

    Allinson, Kathleen R.; Redgrave, Rachael E.; Zhai, Zhenhua; Oh, S. Paul; Fruttiger, Marcus; Arthur, Helen M.

    2014-01-01

    Rare inherited cardiovascular diseases are frequently caused by mutations in genes that are essential for the formation and/or function of the cardiovasculature. Hereditary Haemorrhagic Telangiectasia is a familial disease of this type. The majority of patients carry mutations in either Endoglin (ENG) or ACVRL1 (also known as ALK1) genes, and the disease is characterized by arteriovenous malformations and persistent haemorrhage. ENG and ACVRL1 encode receptors for the TGFβ superfamily of ligands, that are essential for angiogenesis in early development but their roles are not fully understood. Our goal was to examine the role of Acvrl1 in vascular endothelial cells during vascular development and to determine whether loss of endothelial Acvrl1 leads to arteriovenous malformations. Acvrl1 was depleted in endothelial cells either in early postnatal life or in adult mice. Using the neonatal retinal plexus to examine angiogenesis, we observed that loss of endothelial Acvrl1 led to venous enlargement, vascular hyperbranching and arteriovenous malformations. These phenotypes were associated with loss of arterial Jag1 expression, decreased pSmad1/5/8 activity and increased endothelial cell proliferation. We found that Endoglin was markedly down-regulated in Acvrl1-depleted ECs showing endoglin expression to be downstream of Acvrl1 signalling in vivo. Endothelial-specific depletion of Acvrl1 in pups also led to pulmonary haemorrhage, but in adult mice resulted in caecal haemorrhage and fatal anaemia. We conclude that during development, endothelial Acvrl1 plays an essential role to regulate endothelial cell proliferation and arterial identity during angiogenesis, whilst in adult life endothelial Acvrl1 is required to maintain vascular integrity. PMID:24896812

  5. Arteriovenous malformation of the vulva: a case report.

    PubMed

    Pereira, Nigel; Dormosh, Mayes; Mirmanesh, Michael; Guilfoil, Daniel S

    2014-01-01

    To report the case of a patient with a large and symptomatic vulvar lesion, necessitating surgical excision. We report the case of a 57-year-old postmenopausal woman with a 6-month history of an enlarging vulvar lesion associated with vulvar pruritus. On examination, a pedunculated 7 × 5 × 4-cm soft tissue mass attached to the left labium majus was noted. Surgical excision was performed and histopathologic evaluation revealed variably dilated, submucosal vessels with thick muscular walls and intimal thickening, but without endothelial atypia or multilayering. These findings were consistent with a final diagnosis of arteriovenous malformation of the vulva. Given the complex anatomy of the vulva, the differential diagnosis for vulvar vascular lesions can be challenging. Hence, surgical excision and histopathologic evaluation become imperative to distinguish them from other dermatologic and neoplastic conditions of the vulva.

  6. Asystole During Onyx Embolization of a Pediatric Arteriovenous Malformation: A Severe Case of the Trigeminocardiac Reflex.

    PubMed

    Khatibi, Kasra; Choudhri, Omar; Connolly, Ian D; McTaggart, Ryan A; Do, Huy M

    2017-02-01

    Trigeminal-cardiac reflex (TCR) from the stimulation of sensory branches of trigeminal nerve can lead to hemodynamic instability. This phenomenon has been described during ophthalmologic, craniofacial, and skull base surgeries. TCR has been reported rarely with endovascular onyx embolization of dural arteriovenous fistulas. We report a case of TCR during endovascular Onyx embolization of an arteriovenous malformation (AVM). A 16-year-old boy presented with a large cerebellar AVM with arterial feeders from the external carotid artery and posterior cerebral artery branches. The middle meningeal artery was catheterized, through which dimethyl sulfoxide was injected, followed by Onyx, into the nidus and the feeders. Near the completion of embolization, patient became bradycardic and proceeded to asystole; he was resuscitated with chest compression, atropine, and vasopressors. We used PubMed to identify the reported cases of Onyx and other endovascular embolizations complicated by hemodynamic instability. We found 16 cases of endovascular onyx embolization complicated by clinically significant hemodynamic changes in the treatment of dural arteriovenous fistula, cavernous carotid fistula, and juvenile nasopharygeal angiofibroma but not with AVMs. In these cases, arterial supply to the nidus involved the sensory receptive field of trigeminal nerve. Hemodynamic changes have been reported during the injection of dimethyl sulfoxide before the introduction of Onyx, as well as Onyx injection and cast formation. TCR can lead to significant hemodynamic changes during endovascular Onyx embolization of vascular malformations (both pial AVM and dural arteriovenous fistulas) involving receptive field of trigeminal nerve. Therefore, the anesthesiologist should be made aware of treatment approach before intervention and appropriate precautions taken. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Automatic detection and quantification of pulmonary arterio-venous malformations in hereditary hemorrhagic telangiectasia

    NASA Astrophysics Data System (ADS)

    Fetita, Catalin; Fortemps de Loneux, Thierry; Kouvahe, Amélé Florence; El Hajjam, Mostafa

    2017-03-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomic dominant disorder, which is characterized by the development of multiple arterio-venous malformations in the skin, mucous membranes, and/or visceral organs. Pulmonary Arterio-Venous Malformation (PAVM) is an abnormal connection where feeding arteries shunt directly into draining veins with no intervening capillary bed. This condition may lead to paradoxical embolism and hemorrhagic complications. PAVMs patients should systematically be screened as the spontaneous complication rate is high, reaching almost 50%. Chest enhanced contrast CT scanner is the reference screening and follow-up examination. When performed by experienced operators as the prime treatment, percutaneous embolization of PAVMs is a safe, efficient and sustained therapy. The accuracy of PAVM detection and quantification of its progression over time is the key of embolotherapy success. In this paper, we propose an automatic method for PAVM detection and quantification relying on a modeling of vessel deformation, i.e. local caliber increase, based on mathematical morphology. The pulmonary field and vessels are first segmented using geodesic operators. The vessel caliber is estimated by means of a granulometric measure and the local caliber increase is detected by using a geodesic operator, the h-maxdomes. The detection sensitivity can be tuned up according to the choice of the h value which models the irregularity of the vessel caliber along its axis and the PAVM selection is performed according to a clinical criterion of >3 mm diameter of the feeding artery of the PAVM. The developed method was tested on a 20 patient dataset. A sensitivity study allowed choosing the irregularity parameter to maximize the true positive ratio reaching 85.4% in average. A specific false positive reduction procedure targeting the vessel trunks of the arterio-venous tree near mediastinum allows a precision increase from 13% to 67% with an average number of 1

  8. Extensive uterine arteriovenous malformation with hemodynamic instability: Embolization for whole myometrium affection.

    PubMed

    El Agwany, Ahmed Samy; Elshafei, Mohamed

    2018-03-01

    Uterine arteriovenous malformation is abnormal and nonfunctional connections between the uterine arteries and veins. Patients typically present with vaginal bleeding which may be life-threatening. Treatment depends on the symptoms, age, desire for future fertility, localization and size of the lesion. Embolization of the uterine artery is the first choice in symptomatic AVM in patients in the reproductive age. We report a case of acquired AVM with an extensive lesion on ultrasound and MRI, which was successfully treated with uterine artery embolization for severe bleeding (UAE). Copyright © 2018 Elsevier B.V. All rights reserved.

  9. Seizures after Onyx embolization for the treatment of cerebral arteriovenous malformation.

    PubMed

    de Los Reyes, K; Patel, A; Doshi, A; Egorova, N; Panov, F; Bederson, J B; Frontera, J A

    2011-09-01

    Onyx embolization of cerebral arteriovenous malformations (AVM) has become increasingly common. We explored the risk of seizures after Onyx use.A retrospective review was conducted of 20 patients with supratentorial brain arteriovenous malformation (AVM) who received Onyx embolization between 2006 and 2009. Baseline demographics, clinical history, seizure history, AVM characteristics and treatment were compared between those who developed post-onyx seizure and those who did not. MRIs were reviewed for edema following Onyx treatment.Of 20 patients who underwent Onyx embolization, the initial AVM presentation was hemorrhage in 40% (N=8). The median number of embolizations was two (range 1-4) and the median final obliteration amount was 90% (range 50-100%). A history of seizure was present in 50% (N=10) of patients pre-embolization and 12 (60%) patients received seizure medications (treatment or prophylaxis) prior to embolization. Seizur post-Onyx embolization occurred in 45% (N=9). The median time to seizur post-Onyx was seven days (range 0.3-210). Four patients (20%) with seizures post-Onyx had no seizure history. Two of these patients (10%) had no other identifiable cause for seizure other than recent Onyx embolization. Seizures in these two patients occurred within 24 hours of Onyx administration. Among patients with post-Onyx seizures, there was a trend toward larger AVM size (P=0.091) and lower percent obliteration (P=0.062). Peri-AVM edema was present in 75% of MRIs performed within one month of Onyx treatment and may represent a possible etiology for seizures.New onset seizures post-Onyx embolization are not uncommon. Further study of seizure prevention is warranted.

  10. Seizures after Onyx Embolization for the Treatment of Cerebral Arteriovenous Malformation

    PubMed Central

    de los Reyes, K.; Patel, A.; Doshi, A.; Egorova, N.; Panov, F.; Bederson, J.B.; Frontera, J.A.

    2011-01-01

    Summary Onyx embolization of cerebral arteriovenous malformations (AVM) has become increasingly common. We explored the risk of seizures after Onyx use. A retrospective review was conducted of 20 patients with supratentorial brain arteriovenous malformation (AVM) who received Onyx embolization between 2006 and 2009. Baseline demographics, clinical history, seizure history, AVM characteristics and treatment were compared between those who developed post-onyx seizure and those who did not. MRIs were reviewed for edema following Onyx treatment. Of 20 patients who underwent Onyx embolization, the initial AVM presentation was hemorrhage in 40% (N=8). The median number of embolizations was two (range 1-4) and the median final obliteration amount was 90% (range 50-100%). A history of seizure was present in 50% (N=10) of patients pre-embolization and 12 (60%) patients received seizure medications (treatment or prophylaxis) prior to embolization. Seizur post-Onyx embolization occurred in 45% (N=9). The median time to seizur post-Onyx was seven days (range 0.3-210). Four patients (20%) with seizures post-Onyx had no seizure history. Two of these patients (10%) had no other identifiable cause for seizure other than recent Onyx embolization. Seizures in these two patients occurred within 24 hours of Onyx administration. Among patients with post-Onyx seizures, there was a trend toward larger AVM size (P=0.091) and lower percent obliteration (P=0.062). Peri-AVM edema was present in 75% of MRIs performed within one month of Onyx treatment and may represent a possible etiology for seizures. New onset seizures post-Onyx embolization are not uncommon. Further study of seizure prevention is warranted. PMID:22005695

  11. Oral Surgical Procedures Performed Safely in Patients With Head and Neck Arteriovenous Malformations: A Retrospective Case Series of 12 Patients.

    PubMed

    Karim, Abdul Basit; Lindsey, Sean; Bovino, Brian; Berenstein, Alejandro

    2016-02-01

    This case series describes patients with head and neck arteriovenous malformations who underwent oral and maxillofacial surgical procedures combined with interventional radiology techniques to minimize blood loss. Twelve patients underwent femoral cerebral angiography to visualize the extent of vascular malformation. Before the surgical procedures, surgical sites were devascularized by direct injection of hemostatic or embolic agents. Direct puncture sclerotherapy at the base of surgical sites was performed using Surgiflo or n-butylcyanoacrylate glue. Surgical procedures were carried out in routine fashion. A hemostatic packing of FloSeal, Gelfoam, and Avitene was adapted to the surgical sites. Direct puncture sclerotherapy with Surgiflo or n-butylcyanoacrylate glue resulted in minimal blood loss intraoperatively. Local application of the FloSeal, Gelfoam, and Avitene packing sustained hemostasis and produced excellent healing postoperatively. Patients with arteriovenous malformations can safely undergo routine oral and maxillofacial surgical procedures with minimal blood loss when appropriate endovascular techniques and local hemostatic measures are used by the interventional radiologist and oral and maxillofacial surgeon. Copyright © 2016 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  12. A large pulmonary arteriovenous malformation causing cerebrovascular accidents.

    PubMed

    Sladden, David; Casha, Aaron; Azzopardi, Conrad; Manche', Alexander

    2015-04-16

    The incidence of pulmonary arteriovenous malformations (PAVMs) is 2.5 in 100,000. 80% are associated with Osler-Weber-Rendu syndrome or hereditary haemorrhagic telangiectasia. We report the case of a 70-year-old man with a 6 cm spherical mass incidentally found on chest X-ray. There was a localised systolic bruit over the right lower zone posteriorly; however, he was asymptomatic. He had suffered a stroke, affecting his right hand and his speech, from which he recovered. He experienced regular transient ischaemic attacks, on an average of every 2 months. He underwent a right lower lobectomy and on ligating the right lower lobe pulmonary artery the saturations of oxygen rose from 92% to 97%, demonstrating a significant right to left extracardiac shunt. Postoperative recovery was excellent and 1 year later the patient reports no further neurological symptoms. 40% of such lesions exhibit symptoms, however, only one-third are neurological. Treatment should be by percutaneous embolisation. 2015 BMJ Publishing Group Ltd.

  13. Use of the TriSpan Coil to Facilitate the Transcatheter Occlusion of Pulmonary Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Cil, Barbaros E., E-mail: barbaroscil@hotmail.com, E-mail: barbaros@hacettepe.edu.tr; Erdogan, Cueneyt; Akmangit, Ilkay

    Pulmonary arteriovenous malformation (PAVM) is a rare vascular malformation of the lung which may occur as an isolated entity or in association with hereditary hemorrhagic telangiectasia (HHT). Because of considerable risk of serious complications such as cerebral embolism, brain abscess and pulmonary hemorrhage, definitive treatment should be considered in most patients. Embolization with coils or detachable balloons is currently the preferred treatment. Paradoxical embolization of coils and balloons may happen, especially in patients with PAVMs with large feeding arteries. In this report we present our initial experience with the use of the TriSpan coil to lower the risk of coilmore » migration during the transcatheter occlusion of PAVMs.« less

  14. Transcatheter Closure of Bilateral Multiple Huge Pulmonary Arteriovenous Malformations with Homemade Double-Umbrella Occluders

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Zhong Hongshan, E-mail: zhonghongshan@hotmail.com; Xu Ke; Shao Haibo

    2008-07-15

    A 28-year-old man underwent successful transcatheter occlusion of three huge pulmonary arteriovenous malformations (PAVMs) using homemade double-umbrella occluders and stainless steel coils. Thoracic CT with three-dimensional reconstruction and pulmonary angiography were used for treatment planning and follow-up. The diameters of the feeding vessels were 11 mm, 13 mm, and 14 mm, respectively. This report demonstrates the novel design and utility of the double-umbrella occluder, an alternative tool for treatment of large PAVMs.

  15. Utility of indocyanine green videoangiography in subcortical arteriovenous malformation resection.

    PubMed

    Rustemi, Oriela; Scienza, Renato; Della Puppa, Alessandro

    2017-07-01

    Subcortical arteriovenous malformations (AVMs) are surgically challenging. Localization is crucial for eloquent areas, and complete resection evaluation is uncertain. Indocyanine green videoangiography (ICG-VA) can assist this surgery. An illustrative video of a subcortical frontoparietal bleeding AVM resection assisted by ICG-VA is presented. A bleeding arterial feeder aneurysm was embolized in the acute phase to protect against rebleeding. ICG-VA helped to detect the AVM's superficial arterialized draining vein, distinguishing it from normal cortical veins. This enabled a customized sulcus approach. ICG-VA showed normalized flow through the previously arterialized vein, confirming the AVM's complete resection. This applies when there is a single drainage remaining. The video can be found here: https://youtu.be/L7yJEE66kV0 .

  16. De novo development of a cerebral arteriovenous malformation following radiation therapy: Case report and an update to classical arteriovenous malformation nomenclature.

    PubMed

    Koch, Matthew J; Agarwalla, Pankaj K; Stapleton, Christopher J; Ogilvy, Christopher S; Loeffler, Jay S

    2016-06-01

    Cerebral arteriovenous malformations (AVM) are traditionally considered primary congenital lesions that result from embryological aberrations in vasculogenesis. Recent insights, however, suggest that these lesions may be secondary to a vascular insult such as ischemia or trauma. Herein, the authors present a rare case of a secondary cerebral AVM, occurring in a young girl who received prior cranial radiation therapy. At age 3years, she underwent surgical resection, chemotherapy, and photon radiation therapy for treatment of a fourth ventricular ependymoma. At age 19years, she developed new onset seizures and was found to have a left medial temporal lobe AVM. Her seizures were managed successfully with anti-epileptic medications and the AVM was treated with proton radiation therapy. This case highlights a rare but possible vascular sequela of radiation therapy and adds to the growing body of evidence that cerebral AVM may arise as secondary lesions. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. Spontaneous thrombosis of the main draining vein revealing an unruptured brain arteriovenous malformation

    PubMed Central

    Cao, Catherine; Sourour, Nader; Reina, Vincent; Nouet, Aurélien; Di Maria, Federico; Chiras, Jacques; Cornu, Philippe

    2015-01-01

    Haemorrhage is the most frequent revealing condition of brain arteriovenous malformations (bAVMs). We report a rare case of unruptured parietal bAVM revealed by spontaneous thrombosis of the main draining vein, responsible for a focal neurological deficit. The bAVM was embolized in emergency conditions; complete regression of the neurological symptoms was observed within five days after the embolization. Potential mechanisms of such spontaneous thrombosis of the bAVM’s main drainage pathway as well as an exhaustive review of the literature concerning this rare revealing condition are presented and discussed. PMID:25964440

  18. Onyx(®) in endovascular treatment of cerebral arteriovenous malformations - a review.

    PubMed

    Szajner, Maciej; Roman, Tomasz; Markowicz, Justyna; Szczerbo-Trojanowska, Małgorzata

    2013-07-01

    Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. In most cases, the disorder may be asymptomatic. The objective of endovascular AVM treatment is set individually for each case upon consultations with a neurosurgeon and a neurologist. The endpoint of the treatment should consist in prevention of AVM bleeding in a management procedure characterized by a significantly lower risk of complications as compared to the natural history of AVM. Endovascular interventions within AVM may include curative exclusion of AVM from circulation, embolization adjuvant to resection or radiation therapy, targeted closure of a previously identified bleeding site as well as palliative embolization. Onyx was first described in the 1990s. It is a non-adhesive and radiolucent compound. Onyx-based closure of the lumen of the targeted vessel is obtained by means of precipitation. The process is enhanced peripherally to the main flux of the injected mixture. This facilitates angiographic monitoring of embolization at any stage. The degree of lumen closure is associated with the location of the vessel. Supratentorial and cortical locations are most advantageous. Dense and plexiform structure of AVM nidus as well as a low number of supplying vessels and a single superficial drainage vein are usually advantageous for Onyx administration. Unfavorable factors include nidus drainage into multiple compartments as well as multiarterial supply of the AVM, particularly from meningeal arteries, en-passant arteries or perforating feeders. Onyx appears to be a safe and efficient material for embolization of cerebral AVMs, also in cases of intracranial bleeding associated with AVM. Curative embolization of small cerebral AVMs is an efficient and safe alternative to neurosurgical and radiosurgical methods. Careful angiographic assessment of individual arteriovenous malformations should be performed before each Onyx administration.

  19. Double Coaxial Microcatheter Technique for Glue Embolization of Renal Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Uchikawa, Yoko, E-mail: jauchikawa@gmail.com; Mori, Kensaku, E-mail: moriken@md.tsukuba.ac.jp; Shiigai, Masanari, E-mail: m-41gai@yahoo.co.jp

    PurposeTo demonstrate the technical benefit of the double coaxial microcatheter technique for embolization of renal arteriovenous malformations (AVMs) with n-butyl cyanoacrylate and iodized oil (glue).Materials and MethodsSix consecutive patients (1 man and 5 women; mean age 61 years; range 44–77 years) with renal AVMs were included. Five patients had hematuria, and one had a risk of heart failure due to a large intrarenal arteriovenous shunt. All patients underwent transarterial embolization using glue and the double coaxial microcatheter technique with outer 2.6F and inner 1.9F microcatheters. After glue injection, the inner microcatheter was retracted, while the outer microcatheter was retained. We assessed themore » complications and clinical outcomes of this technique.ResultsTechnical success was achieved in all patients. In 9 sessions, 34 feeding arteries were embolized with glue using the double coaxial microcatheter technique, 1 was embolized with glue using a single microcatheter, and 2 were embolized with coils. The double coaxial microcatheter technique was useful for selecting small tortuous feeding arteries, preventing glue reflux to the proximal arteries, and approaching multiple feeding arteries without complete retraction of the microcatheters. As a minor complication, glue migrated into the venous system in four patients without any sequelae. In all patients, favorable clinical outcomes, including hematuria cessation in five patients and improvement of the large intrarenal arteriovenous shunt in one patient, were obtained without deterioration of renal function.ConclusionGlue embolization with the double coaxial microcatheter technique was useful for treating renal AVMs with multiple tortuous feeding arteries.« less

  20. An endovascular option is the final treatment for a giant arteriovenous malformation.

    PubMed

    Benedetto, Filippo; Pipitò, Narayana; Barillà, David; Spinelli, Domenico; Stilo, Francesco; Spinelli, Francesco

    2014-11-01

    We report the case of a 58-year-old man presented with bleeding ulcer of the left arm caused by a high-flow type-C arteriovenous malformation (AVM), feed by branches from both the subclavian arteries. He had been previously treated with AVM sclerotherapy, embolization, humeral artery endografting, and open surgery. We urgently performed coil embolization of the left vertebral artery, and we covered the huge subclavian artery by a thoracic endograft. Then, we embolized the right tyrocervical trunk. The result was an immediate interruption of bleeding. At 12 months, the patient had no neurologic complications, and the upper limb continued to decompress. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Nonimmune hydrops fetalis, hydramnios, microcephaly, and intracranial meningeal hemangioendothelioma.

    PubMed

    Drut, R; Sapia, S; Gril, D; Velasco, J C; Drut, R M

    1993-01-01

    Necropsy findings in a male stillborn at 31 weeks gestational age included nonimmune hydrops, hydramnios, and microcephaly secondary to a hemangioendotheliomatous malformation at the tentorium. The vascular lesion was composed by large and small tortuous endothelium-lined vessels and leiomuscular septa. The lesion is thought to be related to the more frequent arteriovenous malformation of the vein of Galen.

  2. Pancreatic Arteriovenous Malformation Involving the Duodenum Embolized with Ethylene-Vinyl Alcohol Copolymer (Onyx)

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Grasso, Rosario Francesco, E-mail: r.grasso@unicampus.it; Cazzato, Roberto Luigi; Luppi, Giacomo

    Arteriovenous malformation (AVM) of the pancreas is a rare condition. Most patients are asymptomatic or alternatively may present with a wide spectrum of symptoms. Traditionally, surgery has been considered the treatment of choice; however, alternative approaches, such as transcatheter embolization (TAE), may be proposed. We report a case of a 48-year-old man with a pancreatic head AVM, presenting with upper abdominal pain and slight anemia. The patient refused surgery and underwent TAE by means of ethylene-vinyl alcohol copolymer (EVOH). At 3 months follow-up, the patient was able to eat regularly, with no residual pain and no signs of anemia.

  3. Pathogenesis of arteriovenous malformations in the absence of endoglin.

    PubMed

    Mahmoud, Marwa; Allinson, Kathleen R; Zhai, Zhenhua; Oakenfull, Rachael; Ghandi, Pranita; Adams, Ralf H; Fruttiger, Marcus; Arthur, Helen M

    2010-04-30

    Arteriovenous malformations (AVMs) result in anomalous direct blood flow between arteries and veins, bypassing the normal capillary bed. Depending on size and location, AVMs may lead to severe clinical effects including systemic cyanosis (pulmonary AVMs), hemorrhagic stroke (cerebral AVMs) and high output cardiac failure (hepatic AVMs). The factors leading to AVM formation are poorly understood, but patients with the familial disease hereditary hemorrhagic telangiectasia (HHT) develop AVMs at high frequency. As most HHT patients have mutations in ENG (endoglin) or ACVRL1 (activin receptor-like kinase 1), a better understanding of the role of these genes in vascular development is likely to reveal the etiology of AVM formation. Using a mouse with a conditional mutation in the Eng gene, we investigated the sequence of abnormal cellular events occurring during development of an AVM. In the absence of endoglin, subcutaneous Matrigel implants in adult mice were populated by reduced numbers of new blood vessels compared with controls, and resulted in local venous enlargement (venomegaly). To investigate abnormal vascular responses in more detail, we turned to the more readily accessible vasculature of the neonatal retina. Endoglin-deficient retinas exhibited delayed remodeling of the capillary plexus, increased proliferation of endothelial cells and localized AVMs. Muscularization of the resulting arteriovenous shunts appeared to be a secondary response to increased blood flow. AVMs develop when an angiogenic stimulus is combined with endoglin depletion. Moreover, AVM formation appears to result from the combination of delayed vascular remodeling and an inappropriate endothelial cell proliferation response in the absence of endoglin.

  4. Conventional digital subtractional vs non-invasive MR angiography in the assessment of brain arteriovenous malformation.

    PubMed

    Cuong, Nguyen Ngoc; Luu, Vu Dang; Tuan, Tran Anh; Linh, Le Tuan; Hung, Kieu Dinh; Ngoc, Vo Truong Nhu; Sharma, Kulbhushan; Pham, Van Huy; Chu, Dinh-Toi

    2018-06-01

    Digital subtractional angiography (DSA) is the standard method for diagnosis, assessment and management of arteriovenous malformation in the brain. Conventional DSA (cDSA) is an invasive imaging modality that is often indicated before interventional treatments (embolization, open surgery, gamma knife). Here, we aimed to compare this technique with a non-invasive MR angiography (MRI DSA) for brain arteriovenous malformation (bAVM). Fourteen patients with ruptured brain AVM underwent embolization treatment pre-operation. Imaging was performed for all patients using MRI (1.5 T). After injecting contrast Gadolinium, dynamic MRI was performed with 40 phases, each phase of a duration of 1.2 s and having 70 images. The MRI results were independently assessed by experienced radiologist blinded to the cDSA. The AVM nidus was depicted in all patients using cDSA and MRI DSA; there was an excellent correlation between these techniques in terms of the maximum diameter and Spetzler Martin grading. Of the fourteen patients, the drainage vein was depicted in 13 by both cDSA and MRI DSA showing excellent correlation between the techniques used. MRI DSA is a non-invasive imaging modality that can give the images in dynamic view. It can be considered as an adjunctive method with cDSA to plan the strategy treatment for bAVM. Copyright © 2018 Elsevier B.V. All rights reserved.

  5. Introduction to the supratentorial cerebral arteriovenous malformation video supplement.

    PubMed

    Starke, Robert M; Albuquerque, Felipe C; Lawton, Michael T

    2017-07-01

    It is with great pleasure that we present this Neurosurgical Focus video supplement on supratentorial cerebral arteriovenous malformations (AVMs). We were privileged to view a remarkable number of outstanding videos demonstrating current state-of-the-art management of brain AVMs using endovascular and microsurgical modalities. Careful and critical review was required to narrow down the submitted videos to a workable volume for this supplement, which reflects the excellent work being done at multiple centers with these lesions. This issue consists of videos that represent modern microsurgical and neuroendovascular techniques for the treatment of supratentorial cerebral AVMs. The videos demonstrate cutting-edge therapies as well as standard ones, which will be valuable to both novice and expert neurointerventionists and neurosurgeons. We are honored to be involved with this project and proud of its content and expert authors. We believe you will enjoy the video content of this supplement and hope that it will raise the collective expertise of our community of AVM surgeons.

  6. Onyx® in endovascular treatment of cerebral arteriovenous malformations – a review

    PubMed Central

    Szajner, Maciej; Roman, Tomasz; Markowicz, Justyna; Szczerbo-Trojanowska, Małgorzata

    2013-01-01

    Summary Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. In most cases, the disorder may be asymptomatic. The objective of endovascular AVM treatment is set individually for each case upon consultations with a neurosurgeon and a neurologist. The endpoint of the treatment should consist in prevention of AVM bleeding in a management procedure characterized by a significantly lower risk of complications as compared to the natural history of AVM. Endovascular interventions within AVM may include curative exclusion of AVM from circulation, embolization adjuvant to resection or radiation therapy, targeted closure of a previously identified bleeding site as well as palliative embolization. Onyx was first described in the 1990s. It is a non-adhesive and radiolucent compound. Onyx-based closure of the lumen of the targeted vessel is obtained by means of precipitation. The process is enhanced peripherally to the main flux of the injected mixture. This facilitates angiographic monitoring of embolization at any stage. The degree of lumen closure is associated with the location of the vessel. Supratentorial and cortical locations are most advantageous. Dense and plexiform structure of AVM nidus as well as a low number of supplying vessels and a single superficial drainage vein are usually advantageous for Onyx administration. Unfavorable factors include nidus drainage into multiple compartments as well as multiarterial supply of the AVM, particularly from meningeal arteries, en-passant arteries or perforating feeders. Onyx appears to be a safe and efficient material for embolization of cerebral AVMs, also in cases of intracranial bleeding associated with AVM. Curative embolization of small cerebral AVMs is an efficient and safe alternative to neurosurgical and radiosurgical methods. Careful angiographic assessment of individual arteriovenous malformations should be performed before each Onyx administration

  7. Successful Pregnancy with a Full-Term Vaginal Delivery One Year After n-Butyl Cyanoacrylate Embolization of a Uterine Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    McCormick, Colleen C.; Kim, Hyun S.

    Uterine arteriovenous malformation (AVM) causes significant morbidity with vaginal bleeding. Traditional therapy is a hysterectomy with no potential for future pregnancy. We present a case of successful superselective embolization of uterine AVM using n-butyl cyanoacrylate with subsequent normal term pregnancy and uncomplicated vaginal delivery in 1 year.

  8. Pretreatment Predictors of Adverse Radiation Effects After Radiosurgery for Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hayhurst, Caroline; Monsalves, Eric; Prooijen, Monique van

    Purpose: To identify vascular and dosimetric predictors of symptomatic T2 signal change and adverse radiation effects after radiosurgery for arteriovenous malformation, in order to define and validate preexisting risk models. Methods and Materials: A total of 125 patients with arteriovenous malformations (AVM) were treated at our institution between 2005 and 2009. Eighty-five patients have at least 12 months of clinical and radiological follow-up. Any new-onset headaches, new or worsening seizures, or neurological deficit were considered adverse events. Follow-up magnetic resonance images were assessed for new onset T2 signal change and the volume calculated. Pretreatment characteristics and dosimetric variables were analyzedmore » to identify predictors of adverse radiation effects. Results: There were 19 children and 66 adults in the study cohort, with a mean age of 34 (range 6-74). Twenty-three (27%) patients suffered adverse radiation effects (ARE), 9 patients with permanent neurological deficit (10.6%). Of these, 5 developed fixed visual field deficits. Target volume and 12 Gy volume were the most significant predictors of adverse radiation effects on univariate analysis (p < 0.001). Location and cortical eloquence were not significantly associated with the development of adverse events (p = 0.12). No additional vascular parameters were identified as predictive of ARE. There was a significant target volume threshold of 4 cm{sup 3}, above which the rate of ARE increased dramatically. Multivariate analysis target volume and the absence of prior hemorrhage are the only significant predictors of ARE. The volume of T2 signal change correlates to ARE, but only target volume is predictive of a higher volume of T2 signal change. Conclusions: Target volume and the absence of prior hemorrhage is the most accurate predictor of adverse radiation effects and complications after radiosurgery for AVMs. A high percentage of permanent visual field defects in this series

  9. Arterio-venous malformations in childhood: clinical presentation, results after operative treatment and long-term follow-up.

    PubMed

    Kahl, W; Kessel, G; Schwarz, M; Voth, D

    1989-01-01

    In a series of 182 arterio-venous malformations (AVM) recently published we found 33 children aged 1 to 16 years. In 28 cases, the admitting condition was hemorrhage. For 31 AVMs total excision was possible. We observed one postoperative death accounting for a mortality of 3%. All children but two were followed-up by personal examination. Two were not able to work due to severe neurological deficits and seven had not been able to get into their intended occupation.

  10. [The clinico-neuropsychological aspects of arteriovenous malformations of the hippocampus].

    PubMed

    Buklina, S B; Filatov, Iu M; Eliava, Sh Sh

    1998-01-01

    A comprehensive examination was made in 39 patients with arteriovenous malformations (AVM) of hippocampus. Prior to and following surgery, all the patients underwent neuropsychological study after A. R. Luriia (1962). Mnestic disorder was found to be the most common abnormality in patients with AVM at this site. Before surgery, they were detected in 34 of 39 patients, 11 of them having severe memory disorders with the traits of the Korsakoff's syndrome. These patients were found to have mixed posthemorrhagic lesion of the hippocampus, other portions of the temporal lobe and periventricular structures. Twenty nine patients were operated on, 14 of them had progressive mnestic disorder of the modally nonspecific type irrespective the side operated on. There were no postoperative Korsakoff's syndromes. There was no progression in memory defects in patients after surgery on the brain drastically changed after hemorrhage or removal of minor malformations. Before hemorrhage, epileptic paroxysms were observed in 2 of the 39 patients only in the presence of massive AVM obligatorily involving the temporal cortex. Following surgery, there were no new epileptic paroxysms and changes in the emotional status and motivations in the patients. Thus, the hippocampal formation is involved in the primary mechanisms of fixation, retention, reproduction of a memory trace. The participation of many structures of the brain is required to form an emotional status, motivation, and clinical manifestations of epileptic activity.

  11. Molecular and cellular biology of cerebral arteriovenous malformations: a review of current concepts and future trends in treatment.

    PubMed

    Rangel-Castilla, Leonardo; Russin, Jonathan J; Martinez-Del-Campo, Eduardo; Soriano-Baron, Hector; Spetzler, Robert F; Nakaji, Peter

    2014-09-01

    Arteriovenous malformations (AVMs) are classically described as congenital static lesions. However, in addition to rupturing, AVMs can undergo growth, remodeling, and regression. These phenomena are directly related to cellular, molecular, and physiological processes. Understanding these relationships is essential to direct future diagnostic and therapeutic strategies. The authors performed a search of the contemporary literature to review current information regarding the molecular and cellular biology of AVMs and how this biology will impact their potential future management. A PubMed search was performed using the key words "genetic," "molecular," "brain," "cerebral," "arteriovenous," "malformation," "rupture," "management," "embolization," and "radiosurgery." Only English-language papers were considered. The reference lists of all papers selected for full-text assessment were reviewed. Current concepts in genetic polymorphisms, growth factors, angiopoietins, apoptosis, endothelial cells, pathophysiology, clinical syndromes, medical treatment (including tetracycline and microRNA-18a), radiation therapy, endovascular embolization, and surgical treatment as they apply to AVMs are discussed. Understanding the complex cellular biology, physiology, hemodynamics, and flow-related phenomena of AVMs is critical for defining and predicting their behavior, developing novel drug treatments, and improving endovascular and surgical therapies.

  12. Spontaneous thrombosis of the main draining vein revealing an unruptured brain arteriovenous malformation.

    PubMed

    Cao, Catherine; Sourour, Nader; Reina, Vincent; Nouet, Aurélien; Di Maria, Federico; Chiras, Jacques; Cornu, Philippe; Clarençon, Frédéric

    2015-04-01

    Haemorrhage is the most frequent revealing condition of brain arteriovenous malformations (bAVMs). We report a rare case of unruptured parietal bAVM revealed by spontaneous thrombosis of the main draining vein, responsible for a focal neurological deficit. The bAVM was embolized in emergency conditions; complete regression of the neurological symptoms was observed within five days after the embolization. Potential mechanisms of such spontaneous thrombosis of the bAVM's main drainage pathway as well as an exhaustive review of the literature concerning this rare revealing condition are presented and discussed. © The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  13. Factors Associated With Proximal Intracranial Aneurysms to Brain Arteriovenous Malformations: A Prospective Cohort Study.

    PubMed

    Morgan, Michael Kerin; Alsahli, Khalid; Wiedmann, Markus; Assaad, Nazih N; Heller, Gillian Z

    2016-06-01

    The risk of hemorrhage from a brain arteriovenous malformation (bAVM) is increased when an associated proximal intracranial aneurysm (APIA) is present. Identifying factors that are associated with APIA may influence the prediction of hemorrhage in patients with bAVM. To identify patient- and bAVM-specific factors associated with APIA. We analyzed a prospective database of bAVMs for factors associated with the presence of APIA. Factors analyzed included age, sex, bAVM size, aneurysm size, circulation contributing to the bAVM, location of the aneurysm, deep venous drainage, and Spetzler-Ponce categories. Multiple logistic regression was performed to identify an association with APIA. Of 753 cases of bAVM with complete angiographic surveillance, 67 (9%) were found to have APIA. Older age (continuous variable; odds ratio, 1.04; 95% confidence interval, 1.02-1.05) and posterior circulation supply to the bAVM (odds ratio, 2.29; 95% confidence interval, 1.32-3.99) were factors associated with increased detection of APIA. The association of posterior circulation-supplied bAVM was not due to infratentorial bAVM location because 72% of posterior circulation APIAs were supplying supratentorial bAVM. APIAs appear to develop with time, as evident from the increased age for those with APIAs. Furthermore, they were more likely present in bAVMs supplied by the posterior circulation. This may be due to a difference in hemodynamic stress. APIA, associated proximal intracranial aneurysmbAVM, brain arteriovenous malformationDSA, digital subtraction angiographySMG, Spetzler-Martin gradeSPC, Spetzler-Ponce category.

  14. Modern radiosurgical and endovascular classification schemes for brain arteriovenous malformations.

    PubMed

    Tayebi Meybodi, Ali; Lawton, Michael T

    2018-05-04

    Stereotactic radiosurgery (SRS) and endovascular techniques are commonly used for treating brain arteriovenous malformations (bAVMs). They are usually used as ancillary techniques to microsurgery but may also be used as solitary treatment options. Careful patient selection requires a clear estimate of the treatment efficacy and complication rates for the individual patient. As such, classification schemes are an essential part of patient selection paradigm for each treatment modality. While the Spetzler-Martin grading system and its subsequent modifications are commonly used for microsurgical outcome prediction for bAVMs, the same system(s) may not be easily applicable to SRS and endovascular therapy. Several radiosurgical- and endovascular-based grading scales have been proposed for bAVMs. However, a comprehensive review of these systems including a discussion on their relative advantages and disadvantages is missing. This paper is dedicated to modern classification schemes designed for SRS and endovascular techniques.

  15. Modern classification and outcome predictors of surgery in patients with brain arteriovenous malformations.

    PubMed

    Tayebi Meybodi, Ali; Lawton, Michael T

    2018-02-23

    Brain arteriovenous malformations (bAVM) are challenging lesions. Part of this challenge stems from the infinite diversity of these lesions regarding shape, location, anatomy, and physiology. This diversity has called on a variety of treatment modalities for these lesions, of which microsurgical resection prevails as the mainstay of treatment. As such, outcome prediction and managing strategy mainly rely on unraveling the nature of these complex tangles and ways each lesion responds to various therapeutic modalities. This strategy needs the ability to decipher each lesion through accurate and efficient categorization. Therefore, classification schemes are essential parts of treatment planning and outcome prediction. This article summarizes different surgical classification schemes and outcome predictors proposed for bAVMs.

  16. Modeling process of embolization arteriovenous malformation on the basis of two-phase filtration model

    NASA Astrophysics Data System (ADS)

    Cherevko, A. A.; Gologush, T. S.; Ostapenko, V. V.; Petrenko, I. A.; Chupakhin, A. P.

    2016-06-01

    Arteriovenous malformation is a chaotic disordered interlacement of very small diameter vessels, performing reset of blood from the artery into the vein. In this regard it can be adequately modeled using porous medium. In this model process of embolization described as penetration of non-adhesive substance ONYX into the porous medium, filled with blood, both of these fluids are not mixed with each other. In one-dimensional approximation such processes are well described by Buckley-Leverett equation. In this paper Buckley-Leverett equation is solved numerically by using a new modification of Cabaret scheme. The results of numerical modeling process of embolization of AVM are shown.

  17. [Radiosurgical treatment of minor intracranial arteriovenous malformations by using a linear accelerator].

    PubMed

    Betti, O O; Munari, C

    1992-01-01

    This study deals with 43 patients with cerebral arteriovenous malformations (AVMs) of a maximum of 20 mm in diameter. All of them were radiosurgically treated with a linear accelerator in stereotatic conditions (UMIC). The delivered doses vary from 20 gys to 50 gys. Thirty-seven were controlled angiographically and 35 of them showed the disappearence of the AVM. Different parameters can modify the results: delivered dose, the size and shape of the lesion, target-volume, peripheral lesion isodosis (75%), location, underestimation of the size or dose. These results show that small lesions are best to treat than larger ones, particularly because their volume enables us to encompass them more easily. The uniformity of this series is related to the homogenous size of the treated AVMs, thus avoiding the discussion of global, unclear, results.

  18. Central nervous system infection after Onyx embolisation of arterio-venous malformations in two paediatric patients.

    PubMed

    Pulhorn, H; Hartley, J C; Shanmuganathan, M; Lee, C H; Harkness, W; Thompson, D N P

    2014-09-01

    Increasingly, Onyx is used for endovascular embolization of aneurysms and arterio-venous malformations. Although reports in the literature on the use of Onyx are favourable, there have been so far no reports on the central nervous system (CNS) infection rate after embolisation with Onyx and no recommendations as to the management of these infections. We present two cases of paediatric patients who acquired CNS infection with Pseudomonas aeruginosa after Onyx embolisation of AVMs and describe their subsequent management. Presence of established infection after Onyx embolisation should be dealt with by removal of infected material, administration of appropriate antibiotic therapy and supportive treatment.

  19. Multiple cerebral arteriovenous malformations associated to rostral hypoplasia of the superior sagittal sinus: case report.

    PubMed

    TORNè, Ramon; Molina Jaque, Felipe A; Rodriguez-Hernandez, Ana; Arikan, Fuat; Lopez-Bermeo, Diego; Tomasello, Alejandro

    2016-06-07

    Multiple cerebral arteriovenous malformations (AVMs) are a rare occurrence usually associated with defined genetic disorders or a family history of cerebrovascular disease. The remaining cases cannot be associated to a genetic pathogenesis and are considered idiopathic. We report an extremely unusual case nor genetic neither idiopathic, but linked to an anatomical intracranial venous variation. The patient presented two independent frontal AVMs associated with rostral hypoplasia of the superior sagittal sinus. This anatomical variation may have induced frontal venous hypertension (VHT) triggering the development of the two AVMs. Throughout this intriguing case, we discuss the role of VHT in AVM development.

  20. Gadofosveset-enhanced magnetic resonance angiography as a means of evaluating pulmonary arteriovenous malformation: a case report.

    PubMed

    Pressacco, Josephine; Papas, Konstantin

    2012-07-01

    This case report is a unique presentation of a new potential indication for Gadofosvest (Ablavar), a blood pool contrast agent for magnetic resonance angiography (MRA). Ablavar is an excellent MRA contrast agent because it provides optimal contrast opacification of both the arterial and venous system, unlike the conventional extracellular agents that are used for arterial imaging only. The present case report demonstrates the ability of Ablavar to demonstrate pulmonary arteriovenous malformation (AVM), showing both its arterial feeders as well as its venous drainage tract. Crown Copyright © 2012. Published by Elsevier Inc. All rights reserved.

  1. Radiation arteriopathy in the transgenic arteriovenous fistula model.

    PubMed

    Lawton, Michael T; Arnold, Christine M; Kim, Yung J; Bogarin, Ernesto A; Stewart, Campbell L; Wulfstat, Amanda A; Derugin, Nikita; Deen, Dennis; Young, William L

    2008-05-01

    The transgenic arteriovenous fistula model, surgically constructed with transgenic mouse aorta interposed in common carotid artery-to-external jugular vein fistulae in nude rats, has a 4-month experimental window because patency and transgenic phenotype are lost over time. We adapted this model to investigate occlusive arteriopathy in brain arteriovenous malformations after radiosurgery by radiating grafted aorta before insertion in the fistula. We hypothesized that high-dose radiation would reproduce the arteriopathy observed clinically within the experimental time window and that deletions of endoglin (ENG) and endothelial nitric oxide synthase (eNOS) genes would modify the radiation response. Radiation arteriopathy in the common carotid arteries of 171 wild-type mice was examined with doses of 25, 80, 120, or 200 Gy (Experiment 1). Radiation arteriopathy in 68 wild-type arteriovenous fistulae was examined histologically and morphometrically with preoperative radiation doses of 0, 25, or 200 Gy (Experiment 2). Radiation arteriopathy in 51 transgenic arteriovenous fistulae (36 ENG and 15 eNOS knock-out fistulae) was examined using preoperative radiation doses of 0, 25, or 200 Gy (Experiment 3). High-dose radiation (200 Gy) of mouse common carotid arteries induced only mild arteriopathy (mean score, 0.66) without intimal hyperplasia and with high mortality (68%). Radiation arteriopathy in wild-type arteriovenous fistulae was severe (mean score, 3.5 at 200 Gy), with intimal hyperplasia and medial disruption at 3 months, decreasing luminal areas with increasing dose, and no mortality. Arteriopathy was robust in transgenic arteriovenous fistulae with ENG +/- and with eNOS +/-, with thick intimal hyperplasia in the former and distinct smooth muscle cell proliferation in the latter. The transgenic arteriovenous fistula model can be adapted to rapidly reproduce radiation arteriopathy observed in resected brain arteriovenous malformations after radiosurgery. High

  2. Onyx in Brain Arteriovenous Malformation Embolisation.

    PubMed

    Hashim, Hilwati; Muda, A Sobri; Abdul Aziz, Aida; Abdul Hamid, Zuhanis

    2016-07-01

    Embolisation has long been used as an adjunct to surgical resection in the treatment of brain arteriovenous malformation (bAVM). The most commonly used embolic material, n-butylcyanoacrylate glue, requires experience and skill to handle its quick and unpredictable flow and polymerisation. A new liquid embolic agent, ethylene vinyl alcohol copolymer (Onyx), is less adhesive and polymerises slowly, which provides better control for radiologists performing embolisation. To report our experience in embolisation using Onyx alone or in combination with histoacryl for bAVM embolisation in our tertiary referral centre. We retrospectively reviewed the anatomy, technical conditions, complications and clinical outcome of all bAVM patients embolised at our centre using Onyx alone or in combination with n-butylcyanoacrylate glue. Between 2010 and 2013, 13 patients [6 (46.2%) male; 7 (53.8%) female; aged, 14-57 years] were included, and a total of 31 embolisations were performed. Clinical presentation included hemorrhage [9 (69.2%)], seizures [2 (15.4%)], and headache [2 (15.4%)]. Most AVMs were located in the brain hemispheres [12 (92.3%)] and measured <3 cm [7 (53.8%]. Complete occlusion of the AVM was obtained in 2 (15.4%) patients; 11 (84.6%) patients had partial occlusion [6 (54.5%) had <50% nidus occlusion]. Complications occurred in four procedures involving 3 patients (morbidity, 23.1%). This resulted in the death of 1 patient (mortality, 7.7%) and complete recovery with no disability in 2 patients. The total nidal occlusion achieved herein is comparable to other similar studies. Our morbidity and mortality were higher compared to other studies which may be attributed to the small number of patients. More data is being collected which may better reflect on our experience.

  3. Generalized pixel profiling and comparative segmentation with application to arteriovenous malformation segmentation.

    PubMed

    Babin, D; Pižurica, A; Bellens, R; De Bock, J; Shang, Y; Goossens, B; Vansteenkiste, E; Philips, W

    2012-07-01

    Extraction of structural and geometric information from 3-D images of blood vessels is a well known and widely addressed segmentation problem. The segmentation of cerebral blood vessels is of great importance in diagnostic and clinical applications, with a special application in diagnostics and surgery on arteriovenous malformations (AVM). However, the techniques addressing the problem of the AVM inner structure segmentation are rare. In this work we present a novel method of pixel profiling with the application to segmentation of the 3-D angiography AVM images. Our algorithm stands out in situations with low resolution images and high variability of pixel intensity. Another advantage of our method is that the parameters are set automatically, which yields little manual user intervention. The results on phantoms and real data demonstrate its effectiveness and potentials for fine delineation of AVM structure. Copyright © 2012 Elsevier B.V. All rights reserved.

  4. Trans-venous embolization of a basal ganglia ruptured arteriovenous malformation with open surgical arterial control: A hybrid technique.

    PubMed

    Kulcsár, Zsolt; Machi, Paolo; Schaller, Karl; Lovblad, Karl Olof; Bijlenga, Philippe

    2018-05-01

    Treatment of ruptured deep-seated arteriovenous malformations is challenging and associated with elevated risks. This is due to the proximity or involvement of critical brain structures and the specifically fine and delicate angioarchitecture of these lesions, making both endovascular and surgical access technically complicated. We present the advantages of a true combined, open surgical and endovascular transvenous approach in a hybrid operating room. The technique may overcome in part the difficulties and may improve safety and risk related concerns. Copyright © 2018. Published by Elsevier Masson SAS.

  5. Abnormal vessel of the ear associated with congenital arteriovenous malformation in the postauricular region.

    PubMed

    Dixit, Shilpi Gupta; Dixit, Rakesh

    2013-08-01

    Variations in the branching pattern of the common, external, and internal carotid arteries can present as arteriovenous malformations, and their basis can be explained embryologically. Our case was a rare variation presenting as a congenital, very gradually increasing bluish painless swelling at the region of the left lobule of the ear arising from an abnormal vessel (from the postauricular artery) which was explored under general anesthesia through a postauricular curved incision. The abnormal vessel and other feeding vessels were ligated and a sclerosing agent injected. Anomalies of pharyngeal arch arteries like our case can be found resulting from the persistence of channels that normally disappear, and prior knowledge of these anomalies is essential before surgeries like mastoidectomy to prevent alarming hemorrhage.

  6. Onyx extravasation during embolization of a brain arteriovenous malformation.

    PubMed

    Ikeda, Hiroyuki; Imamura, Hirotoshi; Agawa, Yuji; Imai, Yukihiro; Tani, Shoichi; Adachi, Hidemitsu; Ishikawa, Tatsuya; Mineharu, Yohei; Sakai, Nobuyuki

    2017-04-01

    During Onyx embolization to treat brain arteriovenous malformation (AVM), carefully observing the penetration of Onyx to the nidus is important in order to avoid complications such as hemorrhage, ischemia, and difficulty with microcatheter removal. We encountered a case of Onyx extravasation during embolization of a cerebellar AVM confirmed by surgical resection and pathological analysis. The patient was a 44-year-old man with Spetzler-Martin grade I cerebellar AVM who underwent Onyx embolization prior to resection of the brain AVM. While injecting Onyx into the nidus using the "plug-and-push" technique, Onyx extravasation was observed. Onyx injection was paused and subsequently restarted, thereby allowing continuation of embolization. An oblate Onyx cast that was entirely covered in cerebellar tissue was removed during total resection of the AVM, performed the same day. The surgically removed oblate Onyx cast did not contain brain tissue or vessel wall, and immunohistochemical staining against glial fibrillary acidic protein (GFAP) showed Onyx penetration into GFAP-positive cerebellar tissue. Onyx extravasation was confirmed based on intraoperative findings during resection as well as pathological findings. The patient has been followed for four years postoperatively, and adverse events caused by Onyx extravasation have not been observed. Unexpected cast of Onyx, remote from the vascular architecture of the AVM, may represent an intra-parenchymal extravasation.

  7. Transarterial Coil-Augmented Onyx Embolization for Brain Arteriovenous Malformation

    PubMed Central

    Gao, Xu; Liang, Guobiao; Li, Zhiqing; Wang, Xiaogang; Yu, Chunyong; Cao, Peng; Chen, Jun; Li, Jingyuan

    2014-01-01

    Summary Onyx has been widely adopted for the treatment of arteriovenous malformations (AVMs). However, its control demands operators accumulate a considerable learning curve. We describe our initial experience using a novel injection method for the embolization of AVMs. We retrospectively reviewed the data of all 22 patients with brain AVMs (12 men, 10 women; age range, 12-68 years; mean age, 43.2 years) treated by the transarterial coil-augmented Onyx injection technique. The size of the AVMs ranged from 25 mm to 70 mm (average 35.6 mm). The technical feasibility of the procedure, procedure-related complications, angiographic results, and clinical outcome were evaluated. In every case, endovascular treatment (EVT) was completed. A total of 31 sessions were performed, with a mean injection volume of 6.1 mL (range, 1.5-16.0 mL). An average of 96.7% (range 85%-100%) estimated size reduction was achieved, and 18 AVMs could be completely excluded by EVT alone. The results remained stable on follow-up angiograms. A procedural complication occurred in one patient, with permanent mild neurologic deficit. Our preliminary series demonstrated that the coil-augmented Onyx injection technique is a valuable adjunct achieving excellent nidal penetration and improving the safety of the procedure. PMID:24556304

  8. Galen and astrology: a Mésalliance?

    PubMed

    Cooper, Glen M

    2011-01-01

    The author examines the question of Galen's affinity with astrology, in view of Galen's extended astrological discussion in the De diebus decretoriis (Critical Days). The critical passages from Galen are examined, and shown to be superficial in understanding. The author performs a lexical sounding of Galen's corpus, using key terms with astrological valences drawn from the Critical Days, and assesses their absence in Galen's other works. He compares Galen's astrology with the astrology of Ptolemy's Tetrabiblos, and evaluates their respective strategies of scientific reasoning. Three types of inference are introduced and applied to Galen's astrology. Finally, he concludes that the empirical side of Galen's science does not depend upon astrological methods or concepts, but that these were introduced for their rhetorical effect in presenting his new medical methodology. It is suggested that continued attention to Galen's astrology has obscured the truly important empirical scientific method that Galen developed.

  9. Surgery or gamma -knife for the treatment of arteriovenous malformations?

    PubMed

    Shigeno, T; Atsuchi, M; Tanaka, J; Goto, K; Ogata, N

    2000-09-01

    Decision making for either surgery or gamma-knife for the treatment of arteriovenous malformations (AVMs) cannot be uniform. The skill of the neurosurgeon in operating on AVMs is now being compared with that of the gamma-knife. The decision varies from case to case and is to be taken by the neurosurgeon. This report presents three cases in which such decision making was not easy. Case 1 was a non-ruptured cingulate AVM of 2.5 cm diameter in the cingulate cortex. The operative field was anticipated to be very narrow between the parietal bridging veins. Case 2 was a tiny ruptured AVM in the speech-motor area which was buried underneath the cortex. Case 3 was a large ruptured thalamo-stiriate-capsular AVM with feeders from the anterior and posterior choroidal arteries. All cases were operated without serious morbidity. A combination of pre-operative intravascular surgery (cases 1 and 3) or postoperative gamma-knife (case 3) was adopted. In conclusion, there is no unitary rule to decide on surgery or gamma-knife for the treatment of AVMs. It depends on what good or harm the responsible surgeon or the gamma-knife does.

  10. The Hemodynamic Changes in Patients with Cerebral Arteriovenous Malformations before and after Interventional Embolization Therapy with Glubran 2 Acrylic Glue.

    PubMed

    Chen, Qi-Yu; Zhu, Xiao-Rui; Zhang, Yu

    2017-01-01

    The study explored hemodynamic changes in patients with cerebral arteriovenous malformations (CAVM) before and -after interventional embolization therapy with Glubran 2 acrylic glue and analyzed the related factors. CAVM patients received endovascular embolization therapy with Glubran 2. Patients' systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), blood flow velocity (BFV), and pulsatility index (PI) were measured. The location of malformed vessels, Spetzler-Martin (SM) grade, CAVM size, and type of feeding artery and venous drainage were analyzed. CAVM patients showed increased DBP, SBP, MAP, and PI and decreased average BFV compared to before therapy. CAVM patients with big CAVM size, SM grade IV/V, deep location malformed vessels, deep, and mixed venous drainage, and cortical branch and mixed artery blood-supply exhibited lower DBP, SBP, MAP, and PI but higher average BFV. Hypertensive CAVM patients showed lower DBP, SBP, MAP, average BFV, and PI before or after embolization. Hypertension, SM grade, CAVM size, malformed vessels location, venous drainage, and artery blood-supply were correlated to the hemodynamic changes of CAVM patients. Embolization with Glubran 2 acrylic glue could enhance hemodynamics in CAVM patients, and the hemodynamic changes were in correlation with the SM grade, CAVM size, and malformed vessels location. © 2017 S. Karger AG, Basel.

  11. Multistage stereotactic radiosurgery for large cerebral arteriovenous malformations using the Gamma Knife platform.

    PubMed

    Ding, Chuxiong; Hrycushko, Brian; Whitworth, Louis; Li, Xiang; Nedzi, Lucien; Weprin, Bradley; Abdulrahman, Ramzi; Welch, Babu; Jiang, Steve B; Wardak, Zabi; Timmerman, Robert D

    2017-10-01

    Radiosurgery is an established technique to treat cerebral arteriovenous malformations (AVMs). Obliteration of larger AVMs (> 10-15 cm 3 or diameter > 3 cm) in a single session is challenging with current radiosurgery platforms due to toxicity. We present a novel technique of multistage stereotactic radiosurgery (SRS) for large intracranial arteriovenous malformations (AVM) using the Gamma Knife system. Eighteen patients with large (> 10-15 cm 3 or diameter > 3 cm) AVMs, which were previously treated using a staged SRS technique on the Cyberknife platform, were retrospectively selected for this study. The AVMs were contoured and divided into 3-8 subtargets to be treated sequentially in a staged approach at half to 4 week intervals. The prescription dose ranged from 15 Gy to 20 Gy, depending on the subtarget number, volume, and location. Gamma Knife plans using multiple collimator settings were generated and optimized. The coordinates of each shot from the initial plan covering the total AVM target were extracted based on their relative positions within the frame system. The shots were regrouped based on their location with respect to the subtarget contours to generate subplans for each stage. The delivery time of each shot for a subtarget was decay corrected with 60 Co for staging the treatment course to generate the same dose distribution as that planned for the total AVM target. Conformality indices and dose-volume analysis were performed to evaluate treatment plans. With the shot redistribution technique, the composite dose for the multistaged treatment of multiple subtargets is equivalent to the initial plan for total AVM target. Gamma Knife plans resulted in an average PTV coverage of 96.3 ± 0.9% and a PITV of 1.23 ± 0.1. The resulting Conformality indices, V 12Gy and R 50 dose spillage values were 0.76 ± 0.05, 3.4 ± 1.8, and 3.1 ± 0.5 respectively. The Gamma Knife system can deliver a multistaged conformal dose to treat large AVMs when correcting for

  12. Head and neck vascular malformations: time-resolved MR projection angiography.

    PubMed

    Ziyeh, S; Schumacher, M; Strecker, R; Rössler, J; Hochmuth, A; Klisch, J

    2003-10-01

    Extracranial vascular anomalies can be divided into haemangiomas and vascular malformations. The latter can be subdivided on the basis of the predominant type of vascular channels. Separation of high- and low-flow vascular malformations is of clinical importance. We report preliminary observations on time-resolved magnetic resonance projection angiography (MRPA) of vascular malformations of the head and neck. We examined eight patients with vascular anomalies of the head and neck. On MRPA the time between the early arterial phase and enhancement of the malformation could be used to distinguish high- and low-flow lesions. High-flow arteriovenous malformations showed early, intense enhancement. Venous malformations were either not visible on MRPA or showed late enhancement of veins. One patient was examined after embolisation of an arteriovenous fistula of the mandible. Normal MRPA was taken to indicate absence of a residual lesion.

  13. Compliance of systematic reviews articles in brain arteriovenous malformation with PRISMA statement guidelines: Review of literature.

    PubMed

    Akhigbe, T; Zolnourian, A; Bulters, D

    2017-05-01

    The knowledge of reporting compliance of systematic reviews with PRISMA guidelines may assist in improving the quality of secondary research in brain AVM management and subsequently application to patient population and clinical practice. This may allow researchers and clinicians to be equipped to appraise existing literatures based on known deficit to look for or expect. The objective of this study was to assess the compliance of systematic reviews and meta-analysis in the management of brain AVM. Systematic reviews and meta-analyses articles published in medical journals between 1st of May 2011 and 30th April 2016 (five-year period) were examined. Exclusion criteria were articles that were not systematic reviews and not meta-analyses, narrative literature reviews, historical literature reviews, animal studies, unpublished articles, commentaries and letter to the editor. Electronic database search performed through Medline PubMed on 20th September 2016. This systematic review examined seven systematic review articles on intracranial arteriovenous malformation compliance with PRISMA statement guidelines. The mean percentage of applicable PRISMA items across all studies was 74% (range 67-93%). Protocol registration and declaration, risk of bias and funding sources were the most poorly reported of the PRISMA items (14% each). A significant variance in the total percentages was evident between studies (67-93%). Systematic review reporting in medical literature is excessively variable and overall poor. As these papers are being published with increasing frequency, need to fully adhere to PRISMA statement guide for systematic review to ensure high-quality publications. Complete reporting of PRISMA items within systematic reviews in cerebral arteriovenous malformation enhance quality assessment, robust critical appraisal, better judgement and ultimately sound application to practice thereby improving research standards and patients care. Crown Copyright © 2017

  14. Biology of vascular malformations of the brain.

    PubMed

    Leblanc, Gabrielle G; Golanov, Eugene; Awad, Issam A; Young, William L

    2009-12-01

    This review discusses recent research on the genetic, molecular, cellular, and developmental mechanisms underlying the etiology of vascular malformations of the brain (VMBs), including cerebral cavernous malformation, sporadic brain arteriovenous malformation, and the arteriovenous malformations of hereditary hemorrhagic telangiectasia. Summary of Review- The identification of gene mutations and genetic risk factors associated with cerebral cavernous malformation, hereditary hemorrhagic telangiectasia, and sporadic arteriovenous malformation has enabled the development of animal models for these diseases and provided new insights into their etiology. All of the genes associated with VMBs to date have known or plausible roles in angiogenesis and vascular remodeling. Recent work suggests that the angiogenic process most severely disrupted by VMB gene mutation is that of vascular stabilization, the process whereby vascular endothelial cells form capillary tubes, strengthen their intercellular junctions, and recruit smooth muscle cells to the vessel wall. In addition, there is now good evidence that in some cases, cerebral cavernous malformation lesion formation involves a genetic 2-hit mechanism in which a germline mutation in one copy of a cerebral cavernous malformation gene is followed by a somatic mutation in the other copy. There is also increasing evidence that environmental second hits can produce lesions when there is a mutation to a single allele of a VMB gene. Recent findings begin to explain how mutations in VMB genes render vessels vulnerable to rupture when challenged with other inauspicious genetic or environmental factors and have suggested candidate therapeutics. Understanding of the cellular mechanisms of VMB formation and progression in humans has lagged behind that in animal models. New knowledge of lesion biology will spur new translational work. Several well-established clinical and genetic database efforts are already in place, and further

  15. Bleeding complications after endovascular therapy of cerebral arteriovenous malformations.

    PubMed

    Heidenreich, J O; Hartlieb, S; Stendel, R; Pietilä, T A; Schlattmann, P; Wolf, K-J; Schilling, A M

    2006-02-01

    Intracerebral hemorrhages after embolization of arteriovenous malformations (AVMs) are the most dreaded complications of this well-established therapy. Apart from the known risk factors, our center noticed a high incidence of complications during postinterventional monitoring in medical intensive care units (ICUs) and stroke units. We report 125 consecutive interventions performed on 66 patients by using flow-dependent microcatheters and n-butyl cyanoacrylate as the embolic agent. Postinterventional intensive care monitoring was performed in an interdisciplinary operative ICU, a stroke unit, or a medical ICU. Patients were compared with regard to bleeding complications, AVM morphology, embolization result, postinterventional monitoring, and demographic factors. Intracerebral hemorrhages occurred in 7 patients. Significant differences in outcome were found between 66 patients monitored in the interdisciplinary operative ICU from medical ICU or stroke unit. This was also true when adjusted for age and extent of AVM reduction by using exact logistic regression. A partial AVM reduction of >60% was a considerable risk factor for hemorrhage (odds ratio [OR] = 18.8; 95% confidence interval [CI] [1.341, not available]. Age was also an essential risk factor. An age difference of 10 years leads to an OR of 2.545 (95% CI [1.56, 7.35]). A considerable AVM reduction in one session appears to increase the risk of hemorrhage technically. This suggests a distribution of the interventions in many partial steps.

  16. Onyx extravasation during embolization of a brain arteriovenous malformation

    PubMed Central

    Imamura, Hirotoshi; Agawa, Yuji; Imai, Yukihiro; Tani, Shoichi; Adachi, Hidemitsu; Ishikawa, Tatsuya; Mineharu, Yohei; Sakai, Nobuyuki

    2016-01-01

    During Onyx embolization to treat brain arteriovenous malformation (AVM), carefully observing the penetration of Onyx to the nidus is important in order to avoid complications such as hemorrhage, ischemia, and difficulty with microcatheter removal. We encountered a case of Onyx extravasation during embolization of a cerebellar AVM confirmed by surgical resection and pathological analysis. The patient was a 44-year-old man with Spetzler-Martin grade I cerebellar AVM who underwent Onyx embolization prior to resection of the brain AVM. While injecting Onyx into the nidus using the “plug-and-push” technique, Onyx extravasation was observed. Onyx injection was paused and subsequently restarted, thereby allowing continuation of embolization. An oblate Onyx cast that was entirely covered in cerebellar tissue was removed during total resection of the AVM, performed the same day. The surgically removed oblate Onyx cast did not contain brain tissue or vessel wall, and immunohistochemical staining against glial fibrillary acidic protein (GFAP) showed Onyx penetration into GFAP-positive cerebellar tissue. Onyx extravasation was confirmed based on intraoperative findings during resection as well as pathological findings. The patient has been followed for four years postoperatively, and adverse events caused by Onyx extravasation have not been observed. Unexpected cast of Onyx, remote from the vascular architecture of the AVM, may represent an intra-parenchymal extravasation. PMID:27903925

  17. Use of n-butyl cyanoacrylate to reduce left to right shunting of an abdominal arteriovenous malformation in a dog.

    PubMed

    Eason, B D; Hogan, D F; Lim, C; Hogan, M J

    2017-08-01

    A 9-month old castrated male Labradoodle presented to the cardiology service at Purdue University for evaluation of a low-grade murmur. Physical examination, thoracic radiography, and echocardiography were strongly supportive of an extracardiac left-to-right shunt. Subsequent evaluation with nuclear scintigraphy and computed tomography angiography revealed a large, complex arteriovenous malformation within the cranial abdomen. Staged interventional attenuation of the shunt was performed using n-butyl cyanoacrylate that resulted in a reduction in echocardiographic and nuclear scintigraphy derived shunt estimation. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Recurrence of a paediatric arteriovenous malformation 9 years postcomplete excision: case report and review of literature

    PubMed Central

    McCarthy, Claire; Kaliaperumal, Chandrasekaran; O'Sullivan, Michael

    2012-01-01

    Cerebral arteriovenous malformations (AVMs) are a common congenital vascular anomaly, which often present in both children and adults. Surgery is considered curative once postoperative angiography confirms the absence of vessels. We describe a 6-year-old girl, who had a Spetzler-Martin Grade II AVM resected successfully, in which a recurrent AVM was detected on routine follow-up over 9 years post excision. The aetiopathogenesis of this rare occurrence with a review of literature is discussed. Long-term postoperative follow-up in the form of MRI/MR angiogram is recommended for all fully resected AVMs in the paediatric age group, anticipating the possibility of future recurrence. PMID:23010462

  19. Gamma knife surgery for brainstem arteriovenous malformations.

    PubMed

    Yen, Chun-Po; Steiner, Ladislau

    2011-01-01

    To evaluate the long-term imaging and clinical outcomes of patients with brainstem arteriovenous malformations (AVMs) treated with Gamma Knife surgery (GKS). The study included 85 patients with brainstem AVMs undergoing GKS during the period 1989-2007. The locations of the nidi were the midbrain in 42 patients, pons in 31 patients, and medulla oblongata in 12 patients. The volume of the nidi ranged from 0.1-8.9 mL (median 1.4 mL, mean 1.9 mL), and the prescription dose ranged from 5-32 Gy (median 20 Gy, mean 19.9 Gy). After the initial Gamma procedure, 18 patients had repeat GKS for AVM residuals that were still patent. Two patients had a third GKS 7 years and 16 years after a failed repeat GKS. Clinical follow-up ranged from 24-252 months with a mean of 100 months (median 102 months) after the initial GKS. GKS yielded a total angiographic obliteration in 50 (58.8%) patients and subtotal obliteration in 4 (4.7%) patients. In 22 (25.9%) patients, the AVMs remained patent. In 9 patients (10.6%), no flow voids were observed on magnetic resonance imaging (MRI), but angiographic confirmation was unavailable. A small nidus volume and a high prescription dose were significantly associated with increased AVM obliteration rate. Radiation-induced changes developed in 34 patients (40%); 24 were asymptomatic, 1 patient had only headache, and 9 patients developed neurologic deficits. One patient developed a large cyst 6 years after GKS. Given the poor surgical outcome of brainstem AVMs, the results of 59% nidus obliteration and 6% permanent neurologic deficits make GKS a reasonable management of these difficult lesions. Copyright © 2011 Elsevier Inc. All rights reserved.

  20. Acute Respiratory Distress Syndrome after Onyx Embolization of Arteriovenous Malformation

    PubMed Central

    Tawil, Isaac; Carlson, Andrew P.; Taylor, Christopher L.

    2011-01-01

    Purpose. We report a case of a 60-year-old male who underwent sequential Onyx embolizations of a cerebral arteriovenous malformation (AVM) which we implicate as the most likely etiology of subsequent acute respiratory distress syndrome (ARDS). Methods. Case report and literature review. Results. Shortly after the second Onyx embolization procedure, the patient declined from respiratory failure secondary to pulmonary edema. Clinical entities typically responsible for pulmonary edema including cardiac failure, renal failure, iatrogenic volume overload, negative-pressure pulmonary edema, and infectious etiologies were evaluated and excluded. The patient required mechanical ventilatory support for several days, delaying operative resection. The patient met clinical and radiographic criteria for ARDS. After excluding other etiologies of ARDS, we postulate that ARDS developed as a result of Onyx administration. The Onyx copolymer is dissolved in dimethyl sulfoxide (DMSO), a solvent excreted through the lungs and has been implicated in transient pulmonary side effects. Additionally, a direct toxic effect of the Onyx copolymer is postulated. Conclusion. Onyx embolization and DMSO toxicity are implicated as the etiology of ARDS given the lack of other inciting factors and the close temporal relationship. A strong physiologic rationale provides further support. Clinicians should consider this uncommon but important complication. PMID:21687580

  1. Application of Onyx for Renal Arteriovenous Malformation With First Case Report of a Renal Hyperdense Striation Sign

    PubMed Central

    Juan, Yu-Hsiang; Lin, Yu-Ching; Sheng, Ting-Wen; Cheung, Yun-Chung; Ng, Shu-Hang; Yu, Chin-Wei; Wong, Ho-Fai

    2015-01-01

    Abstract Onyx is an emerging treatment modality for visceral vascular malformations, especially in cases in which delicate nidal penetration of the arteriovenous malformation (AVM) is desired. A computed tomography (CT) image presentation of hyperdense striations along the renal medulla secondary to the tantalum powder has not been previously reported. A 65-year-old woman presented to our institution with intermittent gross hematuria and left flank pain for 10 days. Both CT and conventional angiographies confirmed cirsoid-type renal AVM, which was successfully treated with Onyx. Follow-up CT after treatment revealed presence of hyperdense striations along the renal medulla, which resolved during later image follow-up. Despite its frequent usage in neural intervention, the application of Onyx in visceral AVM is gradually gaining interest, especially in cases in which delicate nidal penetration of the AVM is desired. Renal hyperdense striation sign should be recognized to avoid confusion with embolizer migration, and further studies in patients with renal function impairment may be helpful in understanding its influence of renal function. PMID:26426661

  2. Use of intracranial and ocular thermography before and after arteriovenous malformation excision

    NASA Astrophysics Data System (ADS)

    Hwang, Peter Y. K.; Lewis, Philip M.; Maller, Jerome J.

    2014-11-01

    Excision of arteriovenous malformations (AVMs) is known to carry a risk of postoperative hemorrhage, postulated to be the result of normal perfusion pressure breakthrough. It is also possible that AVMs may cause a steal effect, reducing perfusion in nearby vessels. There is currently no simple method of visualizing the presence or absence of steal effect intraoperatively. We hypothesized that the infrared thermographic (heat sensitive) imaging of perilesional brain may be useful for detecting reduced perfusion due to steal. Moreover, we hypothesized that if steal effect was present, it could impact on ocular perfusion and thereby temperature. Our objective was, therefore, to investigate whether perilesional cortical and ocular temperature (OT) may be a marker of steal effect. We intraoperatively acquired conventional and thermal images of the surgical field and eyes bilaterally, pre- and post-excisions of a large left hemisphere AVM. We found OT asymmetry preoperatively, which was absent after the AVM was excised. Intraoperative thermal images showed an increase of perilesional temperature, although this could be confounded by generalized changes in cortical perfusion due to anesthetics or surgery.

  3. Alternatively Activated Macrophages Play an Important Role in Vascular Remodeling and Hemorrhaging in Patients with Brain Arteriovenous Malformation.

    PubMed

    Nakamura, Yukihiko; Sugita, Yasuo; Nakashima, Shinji; Okada, Yousuke; Yoshitomi, Munetake; Kimura, Yoshizou; Miyoshi, Hiroaki; Morioka, Motohiro; Ohshima, Koichi

    2016-03-01

    Angiogenic and immunoactive lesions in brain arteriovenous malformation (BAVM) contribute to hemorrhagic events and the growth of BAVMs. However, the detailed mechanism is unclear. Our objective is to clarify the relationship between hemorrhagic events of BAVM and alternatively activated macrophages in the perinidal dilated capillary network (PDCN). We examined microsurgical specimens of BVMs (n = 29) and focused on the PDCN area. Ten autopsied brains without intracranial disease were the controls. We performed immunostaining of the inflammatory and endothelial cell markers, macrophage markers (CD163 and CD68), and vascular endothelial growth factor A (VEGF-A). We evaluated each cell's density and the vessel density in the PDCN and analyzed the relationship to hemorrhagic events of BAVM. The PDCN was involved in all the resected arteriovenous malformations, and these vessels showed a high rate of CD105 expression (72.0 ± 10.64%), indicating newly proliferating vessels. Alternatively activated macrophages were found, with a high rate (85.6%) for all macrophages (controls, 56.6%). In the hemorrhagic cases, the cell density was significantly higher than that in the nonhemorrhagic cases and controls (hemorrhagic group, 290 ± 44 cells/mm(2); nonhemorrhagic group, 180 ± 59 cells/mm(2); and control, 19 ± 8 cells/mm(2)). The cell density of alternatively activated macrophages showed a positive correlation with the vessel density of the PDCN. Double immunostaining showed that VEGF-A was secreted by alternatively activated macrophages. Our data suggest that alternatively activated macrophages may have some relationships with angiogenesis of PDCN and hemorrhagic event of BAVM. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  4. Prediction of obliteration after gamma knife surgery for cerebral arteriovenous malformations.

    PubMed

    Karlsson, B; Lindquist, C; Steiner, L

    1997-03-01

    To define the factors of importance for the obliteration of cerebral arteriovenous malformations (AVMs), thus making a prediction of the probability for obliteration possible. In 945 AVMs of a series of 1319 patients treated with the gamma knife during 1970 to 1990, the relationship between patient, AVMs, and treatment parameters on the one hand and the obliteration of the nidus on the other was analyzed. The obliteration rate increased both with increased minimum (lowest periphery) and average dose and decreased with increased AVM volume. The minimum dose to the AVMs was the decisive dose factor for the treatment result. The higher the minimum dose, the higher the chance for total obliteration. The curve illustrating this relation increased logarithmically to a value of 87%. A higher average dose shortened the latency to AVM obliteration. For the obliterated cases, the larger the malformation, the lower the minimum dose used. This prompted us to relate the obliteration rate to the product minimum dose (AVM volume)1/3 (K index). The obliteration rate increased linearly with the K index up to a value of approximately 27, and for higher K values, the obliteration rate had a constant value of approximately 80%. For the group of 273 cases treated with a minimum dose of at least 25 Gy, the obliteration rate at the study end point (defined as 2-yr latency) was 80% (95% confidence interval = 75-85%). If obliterations that occurred beyond the end point are included, the obliteration rate increased to 85% (81-89%). The probability of obliteration of AVMs after gamma knife surgery is related both to the lowest dose to the AVMs and the AVM volume, and it can be predicted using the K index.

  5. [The scintigraphic 99mTc-MAA imaging quantification of the right-to-left shunt in a patients with multiple pulmonary arteriovenous malformation and familial teleangiectasis].

    PubMed

    Dolezal, J

    2008-02-01

    To present a case report about 57-years-old woman with hypoxemia, multiple pulmonary arteriovenous (AV) malformations and lips teleangiectasis where the right-to-left shunt quantification was assessed by means of whole body scintigraphy with 9mTc-labelled human macro-aggregated albumin (MAA). A 57-years-old woman underwent X-ray and bolus enhanced lung CT for dyspnoea, hypoxemia and cyanosis. A multiple intrapulmonary arteriovenous malformations were detected. The whole-body 99mTc-MAA scintigraphy for the right-to-left shunt quantification was performed. The whole-body scintigraphy in anterior and posterior view was started after intravenous application of 185 MBq 99mTc-MAA. The double-head gamma camera Infinia (General Electric Medical Systems--GE MS) with infrared body countouring and the large field of view was used. The Gamma camera was fitted with low-energy, high resolution, parallel-hole collimator. Images were evaluated by processing system Xeleris (GE MS). The whole-body 99mTc-MAA scintigraphy revealed significant R-L shunt and uptake of radiotracer in extrapulmonary organs (brain, kidney, spleen). The right-to-left shunt ratio was 36%. The woman underwent successful percutaneous transcatheter microembolization treatment. After treatment the woman underwent the next 99mTc-MAA whole-body scintigraphy and the R-L shunt ratio decreased to 17%. The 99mTc-MAA whole-body scintigraphy assessed the right-to-left shunt ratio and improved the management of patients with multiple intrapulmonary A-V malformations. The next 99mTc-MAA scintigraphy after the percutaneous transcatheter microembolization of multiple intrapulmonary A-V malformations confirmed success of treatment.

  6. Embolization of peripheral high-flow arteriovenous malformations with Onyx.

    PubMed

    Saeed Kilani, M; Lepennec, V; Petit, P; Magalon, G; Casanova, D; Bartoli, J-M; Vidal, V

    2017-03-01

    The aim of this study was to report our experience in embolization of high flow peripheral arteriovenous malformations (AVMs) with Onyx. Nineteen patients (10 men, 9 women) with peripheral high-flow AVMs who were treated with arteruial embolization using Onyx were retrospectively included. AVMs were located in the head and neck (6), extremities (5), chest (2), kidney (2), uterus (2), pelvis (1) and parietal (1). In 13 patients, embolization was done using Onyx only. One patient underwent embolization by direct puncture, the others by transarterial approach. Embolization was performed in one or multiple sessions (up to 5). A total of 28 sessions were performed. Follow-up was performed with a delay between 10 and 34 months. Technical success was achieved in all patients. Complete devascularization was obtained in 12 patients. Surgical excision was performed in 9 patients. Non-target Onyx embolization was not observed. One patient developed stroke. In 1 patient microcatheter fracture occured. One patient presented severe pain and bradycardia during the procedure that disappeared shortly after. One patient had persistent but less frequent epistaxis after embolization. Another patient had persistent pain without improvement. One patient was lost to follow-up. Other patients were free of symptoms on follow-up. Embolization with Onyx ® is an interesting option for management of peripheral high-flow AVMs either preoperatively or as a single treatment. Copyright © 2016 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

  7. An unusual combination of extra-adrenal pheochromocytoma and arteriovenous malformation of the ureter in a young adult.

    PubMed

    Khawaja, Ali; Aziz, Wajahat; Nazim, Syed Muhammad; Abbas, Farhat

    2013-04-23

    We present a case of a 24-year-old gentleman who presented with painless pan haematuria for 2 weeks. During the workup, he was diagnosed to have a retrocaval mass after a CT scan while cystoscopy revealed a polypoidal pulsating lesion in the left ureter. After surgical manipulation of the retrocaval mass, the blood pressure of the patient raised to 260/130 mm Hg. It was completely resected and diagnosed as extra-adrenal pheochromocytoma (paraganglioma) after histopathology. The lesion in the ureter was completely excised and fulgurated and diagnosed as an arteriovenous malformation. To the best of our knowledge, this is the first patient to be presented in the literature with this unusual combination.

  8. Onyx, a New Liquid Embolic Material for Peripheral Interventions: Preliminary Experience in Aneurysm, Pseudoaneurysm, and Pulmonary Arteriovenous Malformation Embolization

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Vanninen, Ritva L., E-mail: ritva.vanninen@kuh.fi; Manninen, I.

    Purpose. To describe our preliminary experience with a new liquid embolization agent, Onyx, in peripheral interventions. Methods and results. We successfully treated two peripheral aneurysms (one in an internal iliac artery, one in a thoracic collateral artery of an aortic coarctation), two peripheral pseudoaneurysms (one in a lumbar artery, one in a renal artery), and one pulmonary arteriovenous malformation. Conclusion. Onyx is a promising alternative embolic material for peripheral interventions. It can be combined with coils in selected cases, and balloon catheters can be effectively used during slow injection of embolic material to control flow and protect the aneurysm neck.

  9. Contralateral decubitus positioning enhances computed tomographic angiographic evaluation of pulmonary vasculature in a patient with a pulmonary arteriovenous malformation.

    PubMed

    Tafti, Bashir Akhavan; Berenji, Gholam R; Santiago, Silverio; Barack, Bruce M

    2012-11-01

    Computed tomographic pulmonary angiography has become the diagnostic procedure of choice in patients suspected of having a pulmonary embolus. However, intrapulmonary shunting of blood in a variety of pathologic conditions can cause suboptimal opacification of the pulmonary arterial circulation and result in a suboptimal or even nondiagnostic study. Radiologists should be aware of these conditions and be familiar with positioning techniques to minimize such shunting. We report a patient suspected of having pulmonary embolism, in whom a preexisting unilateral arteriovenous malformation prevented adequate evaluation of the pulmonary circulation. Positioning the patient in the contralateral decubitus position significantly enhanced image quality.

  10. Endovascular treatment of head and neck arteriovenous malformations.

    PubMed

    Dmytriw, A A; Ter Brugge, K G; Krings, T; Agid, R

    2014-03-01

    Head and neck arteriovenous malformations (H&N AVM) are associated with considerable clinical and psychosocial burden and present a significant treatment challenge. We evaluated the presentation, response to treatment, and outcome of patients with H&N AVMs treated by endovascular means at our institution. Patients with H&N AVMs treated by endovascular means from 1984 to 2012 were evaluated retrospectively. These included AVMs involving the scalp, orbit, maxillofacial, and upper neck localizations. Patient's clinical files, radiological images, catheter angiograms, and surgical reports were reviewed. Eighty-nine patients with H&N AVMs (46 females, 43 males; 48 small, 41 large) received endovascular therapy. The goals of treatment were curative (n = 30), palliative (n = 34), or presurgical (n = 25). The total number of endovascular treatment sessions was 244 (average of 1.5 per patient). The goal of treatment was met in 92.1 % of cases. Eventual cure was achieved in 42 patients accounting for 58.4 % (52/89) of all patients who underwent treatment for any goal. Twenty-eight of these patients were cured by embolization alone (28/89, 31.4 %) of which 18 were single-hole AVFs. Twenty-four were cured by planned surgical excision after presurgical embolization (24/89, 27 %). Seven patients (7/89, 7.2 %) suffered transient and two (2/89, 2.2 %) permanent endovascular treatment complications. Endovascular treatment is effective for H&N AVMs and relatively safe. It is particularly effective for symptom palliation and presurgical aid. Embolization is curative mostly in small lesions and single-hole fistulas. In patients with large non-curable H&N AVMs, endovascular therapy is often the only palliative option.

  11. Appraising the plasticity of the circle of Willis: a model of hemodynamic modulation in cerebral arteriovenous malformations.

    PubMed

    Chuang, Yu-Ming; Guo, Wanyuo; Lin, Ching-Po

    2010-01-01

    Cerebral arteriovenous malformations (AVMs) harbor a network of abnormal vasculatures, namely the nidus between arterial and venous components. The pressure gradient between these two components results in abnormal high-velocity arteriovenous shunts flowing through the nidus and alternate intracranial hemodynamics. This study hypothesizes that the flow patterns of the circle of Willis (CoW) are modulated by the alternation of intracranial hemodynamics occurring in cerebral AVMs. The flow patterns of the CoW before and after AVMs had been corrected and the arteriovenous shunts closed by radiosurgery were assessed to validate the hypothesis. Fifty patients (32 men and 18 women; mean age 35.8 +/- 4.2, range 23-52 years) with cerebral AVMs previously treated by radiosurgery were retrospectively investigated. This investigation used magnetic resonance angiography, performed prior to and after AVM surgery, to assess the CoW flow patterns. The CoW flow patterns in nearly half of the subjects (20/50, 40%) altered after the AVMs had been corrected. The alterations included: (1) decreased size or ceased flow patterns in the CoW vascular segment: ipsilateral A1 (n = 1) of the anterior cerebral artery (ACA), ipsilateral posterior communicating artery (PCoA) segment (n = 7), contralateral PCoA collateral (n = 4), bilateral PCoA (n = 2); (2) increased size or opening of the previous 'hypoplastic' segment of CoW: ipsilateral A1 of ACA (n = 1), contralateral PCoA (n = 2), bilateral PCoA (n = 1), and (3) biphasic alteration of the CoW: ceased ipsilateral PCoA segment and opening ipsilateral A1 of the ACA (n = 1), ceased ipsilateral PCoA and opening contralateral P1 of the posterior cerebral artery (n = 1). The plasticity of the flow patterns in the CoW are modulated by intracranial hemodynamics as shown by the AVM model. The calibers of CoW arterial segments are not a static feature. Willisian collateralization with recruitment of the CoW segment may cease, or hypoplastic segments

  12. Gamma Knife Radiosurgery for Pediatric Arteriovenous Malformations: A Canadian Experience.

    PubMed

    Zeiler, Fred A; Janik, Maciej K; McDonald, Patrick J; Kaufmann, Anthony M; Fewer, Derek; Butler, Jim; Schroeder, Garry; West, Michael

    2016-01-01

    Gamma Knife (GK) radiosurgery for pediatric arteriovenous malformations (AVM) of the brain presents a non-invasive treatment option. We report our institutional experience with GK for pediatric AVMs. We performed a retrospective review of all pediatric patients treated with GK for cerebral AVMs at our institution from November 2003 up to and including September 2014. Patient demographics, AVM characteristics, treatment parameters and AVM responses were recorded. Nineteen patients were treated, with 4 lost to follow-up. The mean age was 14.2 years (range. 7-18 years), with 10 being males (52.6%). The mean AVM diameter and volume were 2.68 cm and 3.10 cm3 respectively. The mean Spetzler-Martin (SM) and Pollock grades of the treated AVMs were 2.4 and 0.99 respectively. The mean follow-up was 62 months. All AVMs treated demonstrated a response on follow-up imaging. Nine of 15 (60.0%) patients displayed obliteration of their AVMs. Nine of 11 patients with a minimum of 3 years follow-up (81.8%) displayed obliteration, with SM and Pollock grades correlating to the chance of obliteration in this group. Two patients developed post-GK edema requiring short course dexamethasone therapy. No other major complications occurred. No permanent complications occurred. GK radiosurgery for pediatric AVMs offers a safe and effective treatment option, with low permanent complication rates during early follow-up.

  13. Overall outcomes following early interventions for intracranial arteriovenous malformations with hematomas.

    PubMed

    Bir, Shyamal C; Maiti, Tanmoy Kumar; Konar, Subhas; Nanda, Anil

    2016-01-01

    We evaluated the timing and predictors of surgical intervention for intracranial arteriovenous malformations (AVM) with hematoma. A ruptured intracranial AVM with hematoma is an emergency condition, and the optimal timing for surgical intervention is not well understood. In addition, the outcome predictors of surgical intervention have rarely been reported. We identified and analyzed 78 patients treated with microsurgical resection for pathologically proven AVM at Louisiana State University Health in Shreveport from February 1992 to December 2004. All 78 patients were diagnosed with ruptured AVM before surgery. The independent variables, including patient demographics, timing of surgery, location of the AVM and comorbidities were analyzed to assess outcome. The results of this series revealed that surgical intervention after 48hours resulted in poor outcomes for patients with hematoma, following a ruptured AVM. Several other prognostic factors, including younger age (11-40years), Spetzler-Martin Grade I and II, and AVM in a supratentorial location, had significant positive effects on outcomes. However, hypertension, smoking, and a prior embolization showed significant negative effects on outcomes after surgery. The multiple logistic regression analyses also revealed that the timing of surgical intervention had a significant effect on outcomes in patients with hematoma following ruptured AVM. Early intervention is the key to success in these patients. Published by Elsevier Ltd.

  14. Tool-Tissue Interaction Forces in Brain Arteriovenous Malformation Surgery.

    PubMed

    Sugiyama, Taku; Gan, Liu Shi; Zareinia, Kourosh; Lama, Sanju; Sutherland, Garnette R

    2017-06-01

    Surgical resection of a brain arteriovenous malformation (AVM) poses a technical challenge because of the fragility and number of small feeding and draining vessels around the nidus. Acquiring knowledge of the optimal force applied to such tissue is important in surgical performance and education. A force-sensing bipolar forceps was developed through installation of strain gauge sensors, and force profiles were obtained from 2 AVM surgeries. The force data associated with vessel injury, unsuccessful trial, was compared with that from successful trials. Receiver operating curve analysis was used for determining optimal force threshold and evaluating the discriminative accuracy of measurement. Force data from 519 trials was collected, of which 16 (3.1%) were unsuccessful. The mean and maximum forces in successful trials were 0.23 ± 0.06 N and 0.35 ± 0.11 N compared with unsuccessful trials of 0.33 ± 0.05 N and 0.53 ± 0.11 N, respectively (P < 0.001). There was a strong association of mean and maximum force peaks with unsuccessful trials as reflected by the area under the curve of 0.91 and 0.87, respectively. Threshold analysis showed that the rate of unsuccessful trials and error forces tended to increase with surgical time. Excessive force at the tool tip may result in injury to fragile vessels during AVM surgery. A quantifiable metric through force sensing instruments can detect and predict the occurrence of such injury. Such an instrument may be ideal for resident training and evaluation. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Spatiotemporal Fractionation Schemes for Irradiating Large Cerebral Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Unkelbach, Jan, E-mail: junkelbach@mgh.harvard.edu; Bussière, Marc R.; Chapman, Paul H.

    2016-07-01

    Purpose: To optimally exploit fractionation effects in the context of radiosurgery treatments of large cerebral arteriovenous malformations (AVMs). In current practice, fractionated treatments divide the dose evenly into several fractions, which generally leads to low obliteration rates. In this work, we investigate the potential benefit of delivering distinct dose distributions in different fractions. Methods and Materials: Five patients with large cerebral AVMs were reviewed and replanned for intensity modulated arc therapy delivered with conventional photon beams. Treatment plans allowing for different dose distributions in all fractions were obtained by performing treatment plan optimization based on the cumulative biologically effective dosemore » delivered at the end of treatment. Results: We show that distinct treatment plans can be designed for different fractions, such that high single-fraction doses are delivered to complementary parts of the AVM. All plans create a similar dose bath in the surrounding normal brain and thereby exploit the fractionation effect. This partial hypofractionation in the AVM along with fractionation in normal brain achieves a net improvement of the therapeutic ratio. We show that a biological dose reduction of approximately 10% in the healthy brain can be achieved compared with reference treatment schedules that deliver the same dose distribution in all fractions. Conclusions: Boosting complementary parts of the target volume in different fractions may provide a therapeutic advantage in fractionated radiosurgery treatments of large cerebral AVMs. The strategy allows for a mean dose reduction in normal brain that may be valuable for a patient population with an otherwise normal life expectancy.« less

  16. [Ultrasound diagnosis of aneurysm of the vein of Galen in children].

    PubMed

    Gazikalović, S; Kosutić, J; Komar, P; Vukomanović, V; Mogić, M

    2001-01-01

    Aneurysm of the vein of Galen is rare and complex vascular disorder that develops during embriogenesis and provokes significant haemodynamic changes. Boys are more frequently involved. During the foetal period Ballantyne syndrome may develop, and postnatal clinical presentation vary with ages. Serious haemodynamic changes are followed by congestive heart failure and, if not treated, with lethal exitus. Fast and correct diagnosis is very important. Ultrasound examination of central nervous system supported with Duplex-Doppler and Colour-Doppler examination of the head and heart enables the diagnosis. This text comments ultrasound presentation of the malformation and ultrasound diagnostic possibilities.

  17. Thalidomide Reduces Hemorrhage of Brain Arteriovenous Malformations in a Mouse Model.

    PubMed

    Zhu, Wan; Chen, Wanqiu; Zou, Dingquan; Wang, Liang; Bao, Chen; Zhan, Lei; Saw, Daniel; Wang, Sen; Winkler, Ethan; Li, Zhengxi; Zhang, Meng; Shen, Fanxia; Shaligram, Sonali; Lawton, Michael; Su, Hua

    2018-05-01

    Brain arteriovenous malformation (bAVM) is an important risk factor for intracranial hemorrhage. Current treatments for bAVM are all associated with considerable risks. There is no safe method to prevent bAVM hemorrhage. Thalidomide reduces nose bleeding in patients with hereditary hemorrhagic telangiectasia, an inherited disorder characterized by vascular malformations. In this study, we tested whether thalidomide and its less toxic analog, lenalidomide, reduce bAVM hemorrhage using a mouse model. bAVMs were induced through induction of brain focal activin-like kinase 1 ( Alk1 , an AVM causative gene) gene deletion and angiogenesis in adult Alk1 -floxed mice. Thalidomide was injected intraperitoneally twice per week for 6 weeks, starting either 2 or 8 weeks after AVM induction. Lenalidomide was injected intraperitoneally daily starting 8 weeks after AVM induction for 6 weeks. Brain samples were collected at the end of the treatments for morphology, mRNA, and protein analyses. The influence of Alk1 downregulation on PDGFB (platelet-derived growth factor B) expression was also studied on cultured human brain microvascular endothelial cells. The effect of PDGFB in mural cell recruitment in bAVM was explored by injection of a PDGFB overexpressing lentiviral vector to the mouse brain. Thalidomide or lenalidomide treatment reduced the number of dysplastic vessels and hemorrhage and increased mural cell (vascular smooth muscle cells and pericytes) coverage in the bAVM lesion. Thalidomide reduced the burden of CD68 + cells and the expression of inflammatory cytokines in the bAVM lesions. PDGFB expression was reduced in ALK1-knockdown human brain microvascular endothelial cells and in mouse bAVM lesion. Thalidomide increased Pdgfb expression in bAVM lesion. Overexpression of PDGFB mimicked the effect of thalidomide. Thalidomide and lenalidomide improve mural cell coverage of bAVM vessels and reduce bAVM hemorrhage, which is likely through upregulation of Pdgfb expression

  18. Absolute Ethanol Embolization of Arteriovenous Malformations in the Periorbital Region

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Su, Li-xin, E-mail: sulixin1975@126.com; Jia, Ren-Bing, E-mail: jrb19760517@hotmail.com; Wang, De-Ming, E-mail: wdmdeming@hotmail.com

    2015-06-15

    ObjectiveArteriovenous malformations (AVMs) involving the periorbital region are technically challenging clinical entities to manage. The purpose of the present study was to present our initial experience of ethanol embolization in a series of 16 patients with auricular AVMs and assess the outcomes of this treatment.MethodsTranscatheter arterial embolization and/or direct percutaneous puncture embolization were performed in the 16 patients. Pure or diluted ethanol was manually injected. The follow-up evaluations included physical examination and angiography at 1- to 6-month intervals.ResultsDuring the 28 ethanol embolization sessions, the amount of ethanol used ranged from 2 to 65 mL. The obliteration of ulceration, hemorrhage, pain, infection,more » pulsation, and bruit in most of the patients was obtained. The reduction of redness, swelling, and warmth was achieved in all the 16 patients, with down-staging of the Schobinger status for each patient. AVMs were devascularized 100 % in 3 patients, 76–99 % in 7 patients, and 50–75 % in 6 patients, according to the angiographic findings. The most common complications were necrosis and reversible blister. No permanent visual abnormality was found in any of the cases.ConclusionEthanol embolization is efficacious and safe in the treatment of AVMs in the periorbital region and has the potential to be accepted as the primary mode of therapy in the management of these lesions.« less

  19. [A vertebral arteriovenous fistula diagnosed by auscultation].

    PubMed

    Iglesias Escalera, G; Diaz-Delgado Peñas, R; Carrasco Marina, M Ll; Maraña Perez, A; Ialeggio, D

    2015-01-01

    Cervical artery fistulas are rare arteriovenous malformations. The etiology of the vertebral arteriovenous fistulas (AVF) can be traumatic or spontaneous. They tend to be asymptomatic or palpation or continuous vibration in the cervical region. An arteriography is necessary for a definitive diagnosis. The treatment is complete embolization of the fistula. We present the case of a two year-old male, where the mother described it «like a washing machine in his head». On palpation during the physical examination, there was a continuous vibration, and a continuous murmur in left cervical region. A vascular malformation in vertebral region was clinically suspected, and confirmed with angio-MRI and arteriography. AVF are rare in childhood. They should be suspected in the presence of noises, palpation or continuous vibration in the cervical region. Early diagnosis can prevent severe complications in asymptomatic children. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  20. A Retrograde Transvenous Embolization Technique with Balloon-Assisted Arterial Aspiration for a Peripheral Arteriovenous Malformation with a Venous Pouch

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kuhara, Asako, E-mail: kuhara-asako@med.kurume-u.ac.jp; Tanaka, Norimitsu; Koganemaru, Masamichi

    Management of arteriovenous malformations (AVMs) is challenging, and there is no consensus regarding either the ideal approach or the treatment timing. Percutaneous embolization is the most frequent approach currently used and is considered the first-line technique for AVMs. There is an ongoing discussion about the best technical approach to embolize AVMs. AVMs associated with a dominant outflow vein (DOV) are rare. Embolization of both the DOV and the nidus is considered more effective. Herein, we report a novel technique of transvenous embolization of a DOV under negative pressure from an arterial balloon catheter in a case of a peripheral AVM.more » This technique allows the embolization of the DOV and the nidus retrogradely.« less

  1. Radiosurgery for Arteriovenous Malformations and the Impact on Headaches.

    PubMed

    Bowden, Greg; Cavaleri, Jonathon; Kano, Hideyuki; Monaco, Edward; Niranjan, Ajay; Flickinger, John; Dade Lunsford, L

    2017-05-01

    Arteriovenous malformations (AVMs) can underlie many diverse neurological signs and symptoms. Headaches are a common presentation that can have a significant impact on quality of life. The authors investigated Gamma Knife ® stereotactic radiosurgery (SRS) outcomes in patients with AVMs and associated headaches. This retrospective study analyzed 102 patients with AVMs who underwent SRS between 1995 and 2013. The patient's headache symptoms led to their AVM diagnosis or developed post hemorrhage of their AVM. Information regarding headache characteristics was obtained from the patient's medical records and at follow-up using a scripted clinical interview. The median imaging follow-up was 61.7 months and clinical follow-up was 89.7 months. The median treatment volume at SRS was 4.1 cm 3 and the median marginal dose was 20 Gy. The actuarial AVM obliteration rate was 60% at 5 years and 78% at 10 years. Patients reported that their overall headache severity decreased by -43.6% and their headache frequency was reduced by -53.4%. Headache reduction was reported in 49.1% of patients at 1 year and 69.5% at 5 years. The median time until improvement was 6.5 months. After SRS, headache medication usage decreased in 29% of patients. Permanent adverse radiation effects after SRS occurred in 3% of patients. Until obliteration was complete, the annual risk of a hemorrhage after SRS was 0.4% per year. Although recall bias related to a retrospective analysis can impact outcomes, headache symptoms associated with AVMs may potentially be decreased or eliminated in a subset of patients treated with Gamma Knife radiosurgery. © 2017 American Headache Society.

  2. MR angiography fusion technique for treatment planning of intracranial arteriovenous malformations.

    PubMed

    McGee, Kiaran P; Ivanovic, Vladimir; Felmlee, Joel P; Meyer, Fredrick B; Pollock, Bruce E; Huston, John

    2006-03-01

    To develop an image fusion technique using elliptical centric contrast-enhanced (CE) MR angiography (MRA) and three-dimensional (3D) time-of-flight (TOF) acquisitions for radiosurgery treatment planning of arteriovenous malformations (AVMs). CE and 3D-TOF MR angiograms with disparate in-plane fields of view (FOVs) were acquired, followed by k-space reformatting to provide equal voxel dimensions. Spatial domain addition was performed to provide a third, fused data volume. Spatial distortion was evaluated on an MRA phantom and provided slice-dependent and global distortion along the three physical dimensions of the MR scanner. In vivo validation was performed on 10 patients with intracranial AVMs prior to their conventional angiogram on the day of gamma knife radiosurgery. Spatial distortion in the phantom within a volume of 14 x 14 x 3.2 cm(3) was less than +/-1 mm (+/-1 standard deviation (SD)) for CE and 3D-TOF data sets. Fused data volumes were successfully generated for all 10 patients. Image fusion can be used to obtain high-resolution CE-MRA images of intracranial AVMs while keeping the fiducial markers needed for gamma knife radiosurgery planning. The spatial fidelity of these data is within the tolerance acceptable for daily quality control (QC) purposes and gamma knife treatment planning. (c) 2006 Wiley-Liss, Inc.

  3. Pulmonary Arteriovenous Malformations After the Superior Cavopulmonary Shunt: Mechanisms and Clinical Implications

    PubMed Central

    Kavarana, Minoo N.; Jones, Jeffrey A.; Stroud, Robert E.; Bradley, Scott M.; Ikonomidis, John S.; Mukherjee, Rupak

    2015-01-01

    Children with functional single ventricle heart disease are commonly palliated down a staged clinical pathway toward a Fontan completion procedure (total cavopulmonary connection). The Fontan physiology is fraught with long term complications associated with lower body systemic venous hypertension, eventually resulting in significant morbidity and mortality. The bidirectional Glenn shunt or superior cavopulmonary connection (SCPC) is commonly the transitional stage in single ventricle surgical management and provides excellent palliation. Some studies have demonstrated lower morbidity and mortality with the SCPC when compared with the Fontan. Unfortunately the durability of the SCPC is significantly limited by the development of pulmonary arteriovenous malformations (PAVMs) which have been commonly attributed to the absence of hepatic venous blood flow and the lack of pulsatile flow to the affected lungs. Abnormal angiogenesis has been suggested as a final common pathway to PAVM development. Understanding these fundamental mechanisms through the investigation of angiogenic pathways associated with the pathogenesis of PAVMs would help to develop medical therapies that could prevent or reverse this complication following SCPC. Such therapies could improve the longevity of the SCPC, potentially eliminate or significantly postpone the Fontan completion with its associated complications, and improve long-term survival in children with single ventricle disease. PMID:24758411

  4. Galen and his anatomic eponym: vein of Galen.

    PubMed

    Ustun, Cagatay

    2004-09-01

    Galen or Galenus was born at Pergamum (now Bergama in Turkey) in 129 A.D., and died in the year 200 A.D. He was a 2nd century Greek philosopher-physician who switched to the medical profession after his father dreamt of this calling for his son. Galen's training and experiences brought him to Alexandria and Rome and he rose quickly to fame with public demonstrations of anatomical and surgical skills. He became physician to emperor Marcus Aurelius and the emperor's ambitious son, Commodus. He wrote prodigiously and was able to preserve his medical research in 22 volumes of printed text, representing half of all Greek medical literature that is available to us today. The structures, the great cerebral vein and the communicating branch of the internal laryngeal nerve, bear his eponym.

  5. Detachable-Tip Microcatheters for Liquid Embolization of Brain Arteriovenous Malformations and Fistulas: A United States Single-Center Experience.

    PubMed

    Herial, Nabeel A; Khan, Asif A; Sherr, Gregory T; Qureshi, Mushtaq H; Suri, M Fareed K; Qureshi, Adnan I

    2015-09-01

    The US Food and Drug Administration recently approved a detachable-tip microcatheter, the Apollo microcatheter (eV3, Inc, Irvine, California), to prevent catheter entrapment during embolization of brain arteriovenous malformations (AVMs) using liquid embolic systems. To report technical aspects and clinical results of cerebral embolizations with the Apollo microcatheter in 7 embolizations in 3 adult patients. A 62-year-old man presented with an AVM in the parieto-occipital region measuring 3.6 × 1.6 cm with major cortical feeders from the right middle cerebral artery (MCA) and minor contribution from the distal right anterior cerebral artery. Two pedicles originating from the MCA were embolized. A 48-year-old woman presented with a left frontal AVM measuring 3.3 × 1.8 cm with arterial feeders from the left MCA, left middle meningeal artery, and contralateral anterior cerebral artery. Three pedicles originating from the left MCA were embolized. A 76-year-old man presented with an arteriovenous fistula with multiple fistulous connections and feeders from both vertebral and occipital arteries and the left posterior cerebral artery draining into the left transverse, torcula, and left sigmoid sinus. Two major occipital artery feeders were embolized. Seven Apollo microcatheters were used with the Onyx 18 liquid embolic system. The length of the detachable tip was 15 mm in 2 and 30 mm in 5 embolizations. The mean microcatheter in-position time within the pedicle was 20 minutes. Detachment of tip occurred in 3 instances. No limitations in accessing target arterial feeders and safe tip disengagement were noted despite prolonged injection times. Our initial experience supports the feasibility, safety, and effectiveness of detachable-tip microcatheters in treating brain AVMs and arteriovenous fistulas.

  6. Pelvic arteriovenous malformation treated by transarterial glue embolisation combining proximal balloon occlusion and devascularisation of multiple feeding arteries.

    PubMed

    Murakami, Kenji; Yamada, Takayuki; Kumano, Reiko; Nakajima, Yasuo

    2014-06-06

    We present a case of a 70-year-old man with abdominal aortic aneurysm and coincident pelvic arteriovenous malformation (AVM). Before the operation for the aneurysm, we embolised the pelvic AVM that had multiple feeding arteries and an aneurysmal-dilated draining vein. After decreasing the number of the feeding arteries by coil embolisation, an n-butyl-2-cyanoacrylate/lipiodol mixture (1:1) was injected into the prominent feeding artery and nidus with proximal balloon occlusion of the right internal iliac artery to decrease the flow to the nidus. The mixture (1:4-8) was also added for the finer feeding arteries that became apparent after the initial procedure to embolise the rest of the nidus. A follow-up study showed no contrast enhancement of the nidus and aneurysmal draining vein. 2014 BMJ Publishing Group Ltd.

  7. Combined deficiency of Notch1 and Notch3 causes pericyte dysfunction, models CADASIL, and results in arteriovenous malformations

    PubMed Central

    Kofler, Natalie M.; Cuervo, Henar; Uh, Minji K.; Murtomäki, Aino; Kitajewski, Jan

    2015-01-01

    Pericytes regulate vessel stability and pericyte dysfunction contributes to retinopathies, stroke, and cancer. Here we define Notch as a key regulator of pericyte function during angiogenesis. In Notch1+/−; Notch3−/− mice, combined deficiency of Notch1 and Notch3 altered pericyte interaction with the endothelium and reduced pericyte coverage of the retinal vasculature. Notch1 and Notch3 were shown to cooperate to promote proper vascular basement membrane formation and contribute to endothelial cell quiescence. Accordingly, loss of pericyte function due to Notch deficiency exacerbates endothelial cell activation caused by Notch1 haploinsufficiency. Mice mutant for Notch1 and Notch3 develop arteriovenous malformations and display hallmarks of the ischemic stroke disease CADASIL. Thus, Notch deficiency compromises pericyte function and contributes to vascular pathologies. PMID:26563570

  8. Embolization biomaterial reinforced with nanotechnology for an in-situ release of anti-angiogenic agent in the treatment of hyper-vascularized tumors and arteriovenous malformations.

    PubMed

    Jubeli, E; Yagoubi, N; Pascale, F; Bédouet, L; Slimani, K; Labarre, D; Saint-Maurice, J P; Laurent, A; Moine, L

    2015-10-01

    A polymer based material was developed to act as an embolic agent and drug reservoir for the treatment of arteriovenous malformations (AVM) and hyper vascularized solid tumors. The aim was to combine the blocking of blood supply to the target region and the inhibition of the embolization-stimulated angiogenesis. The material is composed of an ethanolic solution of a linear acrylate based copolymer and acrylate calibrated microparticles containing nanospheres loaded with sunitinib, an anti-angiogenic agent. The precipitation of the linear copolymer in aqueous environment after injection through microcatheter results in the formation of an in-situ embolization gel whereas the microparticles serve to increase the cohesive properties of the embolization agent and to form a reservoir from which the sunitinib-loaded nanospheres are released post-embolization. The swollen state of the microparticles in contact with aqueous medium results in the release of the nanospheres out of microparticles macromolecular structure. After the synthesis, the formulation and the characterization of the different components of the material, anti-angiogenic activity was evaluated in vitro using endothelial cells and in vivo using corneal neovascularization model in rabbit. The efficiency of the arterial embolization was tested in vivo in a sheep model. Results proved the feasibility of this new system for vascular embolization in association with an in situ delivery of anti-angiogenic drug. This combination is a promising strategy for the management of arteriovenous malformations and solid tumors. Copyright © 2015 Elsevier B.V. All rights reserved.

  9. Vascular malformations: an update.

    PubMed

    Gloviczki, Peter; Duncan, Audra; Kalra, Manju; Oderich, Gustavo; Ricotta, Joseph; Bower, Thomas; McKusick, Michael; Bjarnason, Haraldur; Driscoll, David

    2009-06-01

    Vascular malformations occur as a result of an arrest in the development of the vascular system. The modified Hamburg classification distinguishes arterial, venous, arteriovenous, capillary, lymphatic, and mixed vascular malformations. Each malformation is further subdivided based on anatomy and on the time when arrest in development of the embryogenesis occurred; malformations can be truncular or extratruncular. Progress in the last decade in management has been significant because of improvements in open surgical procedures and perfection of percutaneous and hybrid endovascular interventions and devices, such as balloons, stents, and stent-grafts. There has been increasing use of embolization for the treatment of malformations with coils, other particles, glue, or with endovascular placement of occlusive plugs. Absolute alcohol, detergent liquids, or foam have been used for sclerotherapy with improved efficacy. The agents are delivered percutaneously or through a catheter placed either into the feeding arteries or the draining veins. This review aims to aid vascular and endovascular specialists in staying familiar with vascular malformations. These specialists need to be able to evaluate the patients, perform treatment if appropriate, or refer complex cases to multidisciplinary vascular malformation clinics and vascular centers.

  10. Awake Craniotomy in Arteriovenous Malformation Surgery: The Usefulness of Cortical and Subcortical Mapping of Language Function in Selected Patients.

    PubMed

    Gamble, Alexander J; Schaffer, Sarah G; Nardi, Dominic J; Chalif, David J; Katz, Jeffery; Dehdashti, Amir R

    2015-11-01

    Awake craniotomy for removal of intra-axial lesions is a well-established procedure. Few studies, however, have investigated the usefulness of this approach for resection of arteriovenous malformations adjacent to eloquent language areas. We demonstrate our experience by using cortical stimulation mapping and report for the first time on the usefulness of subcortical stimulation with interrogation of language function during resection of arteriovenous malformations (AVMs) located near language zones. Patients undergoing awake craniotomy for AVMs located in language zones and at least 5 mm away from the closest functional magnetic resonance imaging activation were analyzed. During surgery, cortical bipolar stimulation at 50 Hz, with an intensity of 2 mA, increased to a maximum of 10 mA was performed in the region around the AVM before claiming it negative for language function. In positive language site, the area was restimulated 3 times to confirm the functional deficit. The AVM resection was started based on cortical mapping findings. Further subcortical stimulation performed in concert with speech interrogation by the neuropsychologist continued at key points throughout the resection as feasible. The usefulness of cortical and subcortical stimulation in addition to patient outcomes was analyzed. Between March 2009 and September 2014, 42 brain AVM resections were performed. Four patients with left-sided language zone AVMs underwent awake craniotomy. The AVM locations were fronto-opercular in 2 patients and posterior temporal in 2. The AVM Spetzler-Martin grades were II (2 patients) and III (2 patients). In 1 patient, complete speech arrest was noticed during mapping of the peri-malformation zone, which was not breached during resection. In a second patient who initially demonstrated negative cortical mapping, a speech deficit was noticed during resection and subcortical stimulation. This guided the approach to protect and avoid the sensitive zone. This patient

  11. Complications following linear accelerator based stereotactic radiation for cerebral arteriovenous malformations.

    PubMed

    Skjøth-Rasmussen, Jane; Roed, Henrik; Ohlhues, Lars; Jespersen, Bo; Juhler, Marianne

    2010-06-01

    Primarily, gamma knife centers are predominant in publishing results on arteriovenous malformations (AVM) treatments including reports on risk profile. However, many patients are treated using a linear accelerator-most of these at smaller centers. Because this setting is different from a large gamma knife center, the risk profile at Linac departments could be different from the reported experience. Prescribed radiation doses are dependent on AVM volume. This study details results from a medium sized Linac department center focusing on risk profiles. A database was searched for all patients with AVMs. We included 50 consecutive patients with a minimum of 24 months follow-up (24-51 months). AVM occlusion was verified in 78% of patients (39/50). AVM occlusion without new deficits (excellent outcome) was obtained in 44%. Good or fair outcome (AVM occlusion with mild or moderate new deficits) was seen in 30%. Severe complications after AVM occlusion occurred in 4% with a median interval of 15 months after treatment (range, 1-26 months). We applied an AVM grading score developed at the Mayo Clinic to predict probable outcome after radiosurgery in a large patient population treated with Gamma knife. A cutoff above and below a score of 1.5 could not discriminate between the likelihood of having an excellent outcome (approximately 45%). The chance of having an excellent or good outcome was slightly higher in patients with an AVM score below 1.5 (64% vs. 57%). Copyright 2010 Elsevier Inc. All rights reserved.

  12. Computer simulation of Cerebral Arteriovenous Malformation-validation analysis of hemodynamics parameters.

    PubMed

    Kumar, Y Kiran; Mehta, Shashi Bhushan; Ramachandra, Manjunath

    2017-01-01

    The purpose of this work is to provide some validation methods for evaluating the hemodynamic assessment of Cerebral Arteriovenous Malformation (CAVM). This article emphasizes the importance of validating noninvasive measurements for CAVM patients, which are designed using lumped models for complex vessel structure. The validation of the hemodynamics assessment is based on invasive clinical measurements and cross-validation techniques with the Philips proprietary validated software's Qflow and 2D Perfursion. The modeling results are validated for 30 CAVM patients for 150 vessel locations. Mean flow, diameter, and pressure were compared between modeling results and with clinical/cross validation measurements, using an independent two-tailed Student t test. Exponential regression analysis was used to assess the relationship between blood flow, vessel diameter, and pressure between them. Univariate analysis is used to assess the relationship between vessel diameter, vessel cross-sectional area, AVM volume, AVM pressure, and AVM flow results were performed with linear or exponential regression. Modeling results were compared with clinical measurements from vessel locations of cerebral regions. Also, the model is cross validated with Philips proprietary validated software's Qflow and 2D Perfursion. Our results shows that modeling results and clinical results are nearly matching with a small deviation. In this article, we have validated our modeling results with clinical measurements. The new approach for cross-validation is proposed by demonstrating the accuracy of our results with a validated product in a clinical environment.

  13. Time-of-flight magnetic resonance angiography imaging of a residual arteriovenous malformation nidus after Onyx embolization for stereotactic radiosurgery planning. Technical note.

    PubMed

    Loy, David N; Rich, Keith M; Simpson, Joseph; Dorward, Ian; Santanam, Lakshmi; Derdeyn, Colin P

    2009-05-01

    This report demonstrates that time-of-flight (TOF) MR angiography is a useful adjunct for planning stereotactic radiosurgery (SRS) of large arteriovenous malformations (AVMs) after staged embolization with Onyx. Onyx (ethylene vinyl copolymer), a recently approved liquid embolic agent, has been increasingly used to exclude portions of large AVMs from the parent circulation prior to SRS. Limiting SRS to regions of persistent arteriovenous shunting and excluding regions eliminated by embolization may reduce unnecessary radiation doses to eloquent brain structures. However, SRS dosimetry planning presents unique challenges after Onyx embolization because it creates extensive artifacts on CT scans, and it cannot be delineated from untreated nidus on standard MR sequences. During the radiosurgery procedure, MR images were obtained using a GE Signa 1.5-T unit. Standard axial T2 fast spin echo high-resolution images (TR 3000 msec, TE 108 msec, slice thickness 2.5 mm) were generated for optimal visualization of brain tissue and AVM flow voids. The 3D TOF MR angiography images of the circle of Willis and vertebral arteries were subsequently obtained to visualize AVM regions embolized with Onyx (TR 37 msec, TE 6.9 msec, flip angle 20 degrees). Adjunct TOF MR angiography images demonstrated excellent contrast between nidus embolized with Onyx and regions of persistent arteriovenous shunting within a large AVM prior to SRS. Additional information derived from these sequences resulted in substantial adjustments to the treatment plan and an overall reduction in the treated tissue volume.

  14. [Clinical-neuropsychological study of patients with hematomas, cavernomas and arteriovenous malformations of the brain stem].

    PubMed

    Buklina, S B; Gavriushin, A V; Okishev, D N

    2009-01-01

    A clinical-neuropsychological study of 25 patients with hematomas, cavernomas and arteriovenous malformations of different brain stem regions has been performed. Patients with hydrocephalic-hypertensive and dislocation syndromes as well as a history of neurological diseases were excluded from the study. All patients had hemorrhages in different brain stem regions, most of which had occurred several weeks ago. Hematomas were located in different regions of the pons (14 patients) and midbrain (7 patients) and spread to both regions in 4 patients. All patients underwent MRI study of the brain and complex neuropsychological investigation using the A.R. Luria's method. Neuropsychological symptoms before the surgery were found in 20 patients. Cognitive disturbances similar by the lesion of frontal lobes, in particular the promoter zone, that manifested themselves in disturbances of dynamic praxis, writing, verbal memory, were observed most often. Cognitive disturbances similar by the lesion of occipital hemisphere regions, i.e. disturbances of visual gnosis and spatial defects, were found less often. The most severe symptoms were observed in the lesion of the midbrain and upper regions of the pons.

  15. Concomitant intramedullary arteriovenous malformation and a vertebral hemangioma of cervical spine discovered by a pathologic fracture during bicycle accident.

    PubMed

    Ayhan, Selim; Palaoglu, Selcuk; Geyik, Serdar; Saatci, Isil; Onal, Mehmet Bulent

    2015-01-01

    Spinal intramedullary arteriovenous malformations are uncommon and a challenging type of neurosurgical entities. They are rarely located to cervical segment. On the other hand, although hemangiomas are relatively common bone tumors, cervical involvement is again rare and clinically significant ones are infrequent. A 14 year-old-male patient referred to an academic tertiary care unit and presented with neck pain and left hand weakness. Neurological examination revealed motor strength deficit at intrinsic muscles and hyperesthesia at the left hand. Furthermore the pathological reflexes were positive on the left hand side. Imaging studies showed compression fracture, lytic changes resembling a hemangioma at C7 vertebra, and also an intramedullary vascular pathology at C5-6 level which was shown to be an intradural-intramedullary arteriovenous malformation (AVM) on digital subtraction angiography. Based on neurological and radiological findings, the decision was to treat the patient. After embolization of the AVM, the neurological condition of the patient deteriorated and immediate MRI scan of the cervical spine revealed edema of the spinal cord at the C5-6 level. Thus an emergent surgery was performed and C5-6-7 laminectomies with C5-T2 posterior fixation and arthrodesis were implemented. A second stage operation was carried out as C7 corpectomy with a distractable titanium cage 2 weeks after initial surgery. A follow-up evaluation at five years revealed 4/5 motor strength on his left intrinsic hand muscles and mild hyperactive deep tendon reflexes. Imaging studies at the postoperative period showed stable placement of the construct and no evidence of contrast enhancement at the C5-6 level inside the spinal cord. A rare case of multiple pathologies affecting the cervical spine, coincidentally diagnosed after a pathological fracture during a bicycle accident as vertebral hemangioma and intradural-intramedullary AVM that was successfully treated with early detection

  16. Intraoperative monitoring of brain tissue oxygenation during arteriovenous malformation resection.

    PubMed

    Arikan, Fuat; Vilalta, Jordi; Noguer, Montserrat; Olive, Montserrat; Vidal-Jorge, Marian; Sahuquillo, Juan

    2014-10-01

    In normal perfusion pressure breakthrough (NPPB) it is assumed that following arteriovenous malformation (AVM) resection, vasoparalysis persists in the margins of the lesion and that a sudden increase in cerebral blood flow (CBF) after AVM exclusion leads to brain swelling and postsurgical complications. However, the pathophysiology NPPB remains controversial.The aim of our study was to investigate the oxygenation status in tissue surrounding AVMs and in the distant brain using intraoperative monitoring of cerebral partial pressure of oxygen (PtiO(2)) to achieve a better understanding of NPPB pathophysiology. Patients with supratentorial AVMs were monitored intraoperatively using 2 polarographic Clark-type electrodes. To establish reference values, we also studied PtiO(2) in a group of patients who underwent surgery to treat incidental aneurysms. Twenty-two patients with supratentorial AVMs and 16 patients with incidentally found aneurysms were included. Hypoxic pattern was defined as PtiO(2)≤15 mm Hg and/or PtiO(2)/PaO(2) ratio ≤0.10. Tissue hypoxia was detected in 63.6% of the catheters placed in the perinidal area and in 43.8% of catheters placed in a distant area. AVM excision significantly improved oxygenation both around the AVM and in the distant area. The PtiO(2)/PaO(2) ratio is a better indicator than absolute PtiO(2) in detecting tissue hypoxia in mechanically ventilated patients. Intraoperative monitoring showed tissue hypoxia in the margins of AVMs and in the distant ipsilateral brain as the most common finding. Surgical removal of AVMs induces a significant improvement in the oxygenation status in both areas.

  17. A Systematic Review of Acquired Uterine Arteriovenous Malformations: Pathophysiology, Diagnosis, and Transcatheter Treatment

    PubMed Central

    Yoon, Daniel J.; Jones, Megan; Taani, Jamal Al; Buhimschi, Catalin; Dowell, Joshua D.

    2015-01-01

    Objective An acquired uterine arteriovenous malformation (AVM) is a rare cause of vaginal bleeding and, although hysterectomy is the definitive therapy, transcatheter embolization (TCE) provides an alternative treatment option. This systematic review presents the indications, technique, and outcomes for transcatheter treatment of the acquired uterine AVMs. Study Design Literature databases were searched from 2003 to 2013 for eligible clinical studies, including the patient characteristics, procedural indication, results, complications, as well as descriptions on laterality and embolic agents utilized. Results A total of 40 studies were included comprising of 54 patients (average age of 33.4 years). TCE had a primary success rate with symptomatic control of 61% (31 patients) and secondary success rate of 91% after repeated embolization. When combined with medical therapy, symptom resolution was noted in 48 (85%) patients without more invasive surgical procedures. Conclusion Low-level evidence supports the role of TCE, including in the event of persistent bleeding following initial embolization, for the treatment of acquired uterine AVMs. The variety of embolic agents and laterality of approach delineate the importance of refining procedural protocols in the treatment of the acquired uterine AVM. Condensation A review on the management of patients with acquired uterine AVMs. PMID:26929872

  18. Treatment of arteriovenous malformations involving the hand.

    PubMed

    Park, Ui-Jun; Do, Young-Soo; Park, Kwang-Bo; Park, Hong-Suk; Kim, Young-Wook; Lee, Byung-Boong; Kim, Dong-Ik

    2012-07-01

    Hand arteriovenous malformations (AVMs) are difficult to treat because of the necessity to maintain function and the high complication rate of treatment. The purpose of this study was to review the treatment of hand AVMs with embolo/sclerotherapy and the surgical procedures at a single institute. We retrospectively reviewed the medical records and identified the patients who were referred to the vascular division owing to hand AVMs between 1995 and 2009. The lesions were classified according to their affected areas. The treatments used at the clinic included conservative treatment, amputation, and embolo/sclerotherapy. We investigated the clinical data and assessed the treatment results. Sixty-four patients were involved in this study. The median follow-up duration was 26.9 months (range: 3.5-141.8 months). The median age of the patients was 31.5 years (range: 0.3-75.0 years). All of the lesions were of the extratruncal (ET) form, and 37 cases (57.8%) were of the infiltrating type. Sixteen patients were treated conservatively. Primary amputation was performed in seven cases with previous complications such as ulcer, bleeding, or functional limitations. Embolo/sclerotherapy with ethanol was performed in 41 patients. Sixteen (39.0%) of them showed clinical improvement. The treatment of 20 (48.8%) of the 41 patients was interrupted owing to a variety of complications, and 2 (4.9%) of these patients failed with embolo/sclerotherapy. Skin necrosis was the major complication, and this occurred in 17 patients treated with embolo/sclerotherapy--14 of these cases were small and the skin necrosis healed with conservative treatment; 1 patient had autoamputation owing to necrosis; and 2 patients underwent amputation surgery owing to gangrene. The risk for skin necrosis was higher for the AVMs that involved the subcutaneous layer and the AVMs that extended diffusely (P = 0.021, P = 0.011). Seven neuropathic complications developed after embolo/sclerotherapy, and all of them

  19. Use of the Apollo detachable-tip microcatheter for endovascular embolization of arteriovenous malformations and arteriovenous fistulas.

    PubMed

    Flores, Bruno C; See, Alfred P; Weiner, Gregory M; Jankowitz, Brian T; Ducruet, Andrew F; Albuquerque, Felipe C

    2018-03-23

    OBJECTIVE Liquid embolic agents have revolutionized endovascular management of arteriovenous malformations (AVMs) and arteriovenous fistulas (AVFs). Nonetheless, since 2005, the US FDA has received more than 100 reports of microcatheter breakage or entrapment related to Onyx embolization, including 9 deaths. In 2014, the Apollo detachable-tip microcatheter became the first of its kind available in the US. Since then, few reports on its safety have been published. METHODS The authors conducted a retrospective review of endovascular cases by searching the patient databases at 2 tertiary cerebrovascular centers (Barrow Neurological Institute and University of Pittsburgh Medical Center). Patients who underwent endovascular embolization of an AVM or AVF using the Apollo microcatheter were identified. Patient demographics and lesion characteristics were collected. The authors analyzed Apollo-specific endovascular variables, such as number of microcatheterizations, sessions, and pedicles embolized; microcatheter tip detachment status; obliteration rate; and endovascular- and microcatheter-related morbidity and mortality. RESULTS From July 2014 to October 2016, a total of 177 embolizations using the Apollo microcatheter were performed in 61 patients (mean age 40.3 years). The most frequent presentation was hemorrhage (22/61, 36.1%). Most lesions were AVMs (51/61, 83.6%; mean diameter 30.6 mm). The mean Spetzler-Martin grade was 2.4. Thirty-nine (76.5%) of 51 patients with AVMs underwent resection. Microcatheterization was successful in 172 pedicles. Most patients (50/61, 82%) underwent a single embolization session. The mean number of pedicles per session was 2.5 (range 1-7). Onyx-18 was used in 103 (59.9%), N-butyl cyanoacrylate (NBCA) in 44 (25.6%), and Onyx-34 in 25 (14.5%) of the 172 embolizations. In 45.9% (28/61) of the patients, lesion obliteration of 75% or greater was achieved. Tip detachment occurred in 19.2% (33/172) of microcatheters. Fifty-three (86.9%) of the

  20. Cranial Nerve Palsy after Onyx Embolization as a Treatment for Cerebral Vascular Malformation

    PubMed Central

    Lee, Jong Min; Whang, Kum; Cho, Sung Min; Kim, Jong Yeon; Oh, Ji Woong; Koo, Youn Moo; Hu, Chul; Pyen, Jinsoo

    2017-01-01

    The Onyx liquid embolic system is a relatively safe and commonly used treatment for vascular malformations, such as arteriovenous fistulas and arteriovenous malformations. However, studies on possible complications after Onyx embolization in patients with vascular malformations are limited, and the occurrence of cranial nerve palsy is occasionally reported. Here we report the progress of two different types of cranial nerve palsy that can occur after embolization. In both cases, Onyx embolization was performed to treat vascular malformations and ipsilateral oculomotor and facial nerve palsies were observed. Both patients were treated with steroids and exhibited symptom improvement after several months. The most common types of neuropathy that can occur after Onyx embolization are facial nerve palsy and trigeminal neuralgia. Although the mechanisms underlying these neuropathies are not clear, they may involve traction injuries sustained while extracting the microcatheter, mass effects resulting from thrombi and edema, or Onyx reflux into the vasa nervorum. In most cases, the neuropathy spontaneously resolves several months following the procedure. PMID:29159152

  1. Cranial Nerve Palsy after Onyx Embolization as a Treatment for Cerebral Vascular Malformation.

    PubMed

    Lee, Jong Min; Whang, Kum; Cho, Sung Min; Kim, Jong Yeon; Oh, Ji Woong; Koo, Youn Moo; Hu, Chul; Pyen, Jinsoo; Choi, Jong Wook

    2017-09-01

    The Onyx liquid embolic system is a relatively safe and commonly used treatment for vascular malformations, such as arteriovenous fistulas and arteriovenous malformations. However, studies on possible complications after Onyx embolization in patients with vascular malformations are limited, and the occurrence of cranial nerve palsy is occasionally reported. Here we report the progress of two different types of cranial nerve palsy that can occur after embolization. In both cases, Onyx embolization was performed to treat vascular malformations and ipsilateral oculomotor and facial nerve palsies were observed. Both patients were treated with steroids and exhibited symptom improvement after several months. The most common types of neuropathy that can occur after Onyx embolization are facial nerve palsy and trigeminal neuralgia. Although the mechanisms underlying these neuropathies are not clear, they may involve traction injuries sustained while extracting the microcatheter, mass effects resulting from thrombi and edema, or Onyx reflux into the vasa nervorum. In most cases, the neuropathy spontaneously resolves several months following the procedure.

  2. A case of pancreatic AV malformation in an elderly man.

    PubMed

    Gupta, Vipin; Kedia, Saurabh; Sonika, Ujjwal; Madhusudhan, Kumble Seetharama; Pal, Sujoy; Garg, Pramod

    2018-06-01

    A 60-year-old man presented with recurrent abdominal pain and weight loss for 6 months. Abdominal imaging showed a large vascular lesion in the head and neck of pancreas suggestive of arteriovenous malformation (AV malformation). Endoscopic ultrasound was done which showed features of AV malformation with no evidence of pancreatic malignancy. Surgery was planned for definitive treatment of malformation. Digital subtraction angiography with angioembolization was done prior to surgery to reduce vascularity of the lesion. He recovered after a pylorus preserving pancreaticoduodenectomy. Histopathology of the resected specimen confirmed the pancreatic AV malformation. There has been no recurrence at 2 years of follow-up.

  3. Galen, satire and the compulsion to instruct.

    PubMed

    Rosen, Ralph M

    2010-01-01

    This chapter explores Galen's attitude toward instruction and teaching, and in particular the ways in which he conceptualized and articulated the didactic function of his writings. Galen's own rhetoric about why he wrote was often strident - his disparagement of contemporaries is famous, and his fondness for polemic is often regarded as a function of an eristic and arrogant personality. I suggest, however, that Galen's self-avowed role as a kind of public censor may derive as much from an amalgamation of rhetorical postures found in various literary and philosophical genres as it does from an inherently intemperate character. By examining various passages in Galen's protreptic and psychological works, I argue that his frequent stances of vituperative indignation and self-righteousness often resemble those found in satirical writings, from Cynic diatribe through Greek and Roman satirical poetry. Galen no doubt felt himself to be working in a serious tradition of Platonic and Stoic moralizing, but his particular form of didacticism was informed by various strategies assimilated from Greco-Roman serio-comic traditions.

  4. Non-Enhanced MR Imaging of Cerebral Arteriovenous Malformations at 7 Tesla.

    PubMed

    Wrede, Karsten H; Dammann, Philipp; Johst, Sören; Mönninghoff, Christoph; Schlamann, Marc; Maderwald, Stefan; Sandalcioglu, I Erol; Ladd, Mark E; Forsting, Michael; Sure, Ulrich; Umutlu, Lale

    2016-03-01

    To evaluate prospectively 7 Tesla time-of-flight (TOF) magnetic resonance angiography (MRA) and 7 Tesla non-contrast-enhanced magnetization-prepared rapid acquisition gradient-echo (MPRAGE) for delineation of intracerebral arteriovenous malformations (AVMs) in comparison to 1.5 Tesla TOF MRA and digital subtraction angiography (DSA). Twenty patients with single or multifocal AVMs were enrolled in this trial. The study protocol comprised 1.5 and 7 Tesla TOF MRA and 7 Tesla non-contrast-enhanced MPRAGE sequences. All patients underwent an additional four-vessel 3D DSA. Image analysis of the following five AVM features was performed individually by two radiologists on a five-point scale: nidus, feeder(s), draining vein(s), relationship to adjacent vessels, and overall image quality and presence of artefacts. A total of 21 intracerebral AVMs were detected. Both sequences at 7 Tesla were rated superior over 1.5 Tesla TOF MRA in the assessment of all considered AVM features. Image quality at 7 Tesla was comparable with DSA considering both sequences. Inter-observer accordance was good to excellent for the majority of ratings. This study demonstrates excellent image quality for depiction of intracerebral AVMs using non-contrast-enhanced 7 Tesla MRA, comparable with DSA. Assessment of untreated AVMs is a promising clinical application of ultra-high-field MRA. • Non-contrast-enhanced 7 Tesla MRA demonstrates excellent image quality for intracerebral AVM depiction. • Image quality at 7 Tesla was comparable with DSA considering both sequences. • Assessment of intracerebral AVMs is a promising clinical application of ultra-high-field MRA.

  5. Complications Following Linear Accelerator Based Stereotactic Radiation for Cerebral Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Skjoth-Rasmussen, Jane, E-mail: jane@skjoeth-rasmussen.d; Roed, Henrik; Ohlhues, Lars

    2010-06-01

    Purpose: Primarily, gamma knife centers are predominant in publishing results on arteriovenous malformations (AVM) treatments including reports on risk profile. However, many patients are treated using a linear accelerator-most of these at smaller centers. Because this setting is different from a large gamma knife center, the risk profile at Linac departments could be different from the reported experience. Prescribed radiation doses are dependent on AVM volume. This study details results from a medium sized Linac department center focusing on risk profiles. Method and Materials: A database was searched for all patients with AVMs. We included 50 consecutive patients with amore » minimum of 24 months follow-up (24-51 months). Results: AVM occlusion was verified in 78% of patients (39/50). AVM occlusion without new deficits (excellent outcome) was obtained in 44%. Good or fair outcome (AVM occlusion with mild or moderate new deficits) was seen in 30%. Severe complications after AVM occlusion occurred in 4% with a median interval of 15 months after treatment (range, 1-26 months). Conclusions: We applied an AVM grading score developed at the Mayo Clinic to predict probable outcome after radiosurgery in a large patient population treated with Gamma knife. A cutoff above and below a score of 1.5 could not discriminate between the likelihood of having an excellent outcome (approximately 45%). The chance of having an excellent or good outcome was slightly higher in patients with an AVM score below 1.5 (64% vs. 57%).« less

  6. Early outcomes and periprocedural complications of transarterial embolization of brain arteriovenous malformations with Onyx®.

    PubMed

    Poncyljusz, Wojciech; Sawicki, Marcin; Lubkowska, Katarzyna; Rać, Monika

    Brain arteriovenous malformation (BAVM) is a rare pathology diagnosed mostly in young adults. However, due to its hemorrhagic complications, it constitutes an important clinical problem. Treatment modalities available include endovascular, surgery and radiosurgery. The aim of the study was to assess the efficacy and safety of endovascular treatment of BAVM with Onyx ® by reporting one-center experience. Between 2006 and 2013, 54 patients with BAVM were embolized with Onyx. The group consisted of 24 males and 30 females, aged 10 to 65 years (mean 42.6±15.4). Clinical manifestations of BAVMs were: hemorrhage in 27 (50.0%), headaches in 12 (22.2%), seizures in 7 (13.0%) and focal neurologic deficits in 2 (3.7%) patients. Six (11.1%) patients were asymptomatic. A majority of BAVMs were of II and III grade in Spetzler-Martin scale (19 and 22 cases respectively). A total number of 108 endovascular procedures were performed (mean 2.00±0.98 sessions/patient). Complete obliteration of malformation was achieved in 25 (46.3%) patients, mostly with grade II and III BAVMs. In 29 (53.7%) patients, embolization led to a decrease in size of BAVM that made it feasible for other treatment modality. Morbidity and mortality rates were 5.6% and 1.8% respectively. The rate of hemorrhagic complications was 9.3%. Embolization of BAVM with Onyx ® is an effective and safe method of treatment. However, regarding type and consequences of complications, the technique needs further improvement. Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.

  7. Developmental deficits in adult patients with arteriovenous malformations.

    PubMed

    Lazar, R M; Connaire, K; Marshall, R S; Pile-Spellman, J; Hacein-Bey, L; Solomon, R A; Sisti, M B; Young, W L; Mohr, J P

    1999-01-01

    Cerebral arteriovenous malformations (AVMs) are congenital masses of arteries and veins that appear to undergo an unclear "maturation" for many years. Using structured interviews, we compared developmental history of adult patients with AVM with a comparison group of patients with cerebral tumor or aneurysm. To determine whether a remote history of developmental abnormality in adult patients with AVM might be an early marker of cerebral status. Adult patients with AVM and a comparison group of patients with cerebral aneurysm or low-grade tumor participated in a survey. Urban medical school-based tertiary care center. Forty-four randomly selected patients with AVM from the Columbia-Presbyterian AVM Database. There were 32 comparison patients:15 randomly chosen patients from the institution's Cerebral Aneurysm Database and all 17 patients who underwent a biopsy from 1990 to 1995 with a diagnosis of low-grade tumor and who could be contacted. A brief, structured interview adapted from the Centers for Disease Control and Prevention for its 1994 study of the prevalence of learning disabilities in American children. We defined the positive occurrence of a condition as an affirmative answer to the question, " Did have (condition) during his/her school-age years?" Each patient was also asked if there had been any problems in the following skill areas: reading, writing, listening, speaking, attention, impulsivity, organization, mathematics, or drawing. The AVM size was calculated on the angiographic film by measuring its longest diameter in any dimension. Patients with AVM were significantly more likely to report a positive occurrence to any survey question (P<.05). Two thirds of all patients with AVM (66%) reported at least 1 skill difficulty during their school years, significantly more than the comparison group (P<.001). Neither the maximum AVM diameter nor the occurrence of hemorrhage as an adult differed between patients with AVM with and without early skill difficulty

  8. Interobserver Agreement on Arteriovenous Malformation Diffuseness Using Digital Subtraction Angiography.

    PubMed

    Braileanu, Maria; Yang, Wuyang; Caplan, Justin M; Lin, Li-Mei; Radvany, Martin G; Tamargo, Rafael J; Huang, Judy

    2016-11-01

    Arteriovenous malformation (AVM) diffuseness has been shown to be prognostic of treatment outcomes. We assessed interobserver agreement of AVM diffuseness among physicians of different specialty and training backgrounds using digital subtraction angiography (DSA). All research protocols were approved by the institutional review board for this retrospective chart review. In a single-blinded setting, 2 attending neurosurgeons, 1 attending interventional neuroradiologist, and 1 senior neurosurgical resident rated 80 DSA views of 36 AVMs as either compact or diffuse. Individual interobserver agreement and subgroup agreement were analyzed using κ agreement and intraclass correlation coefficient. Disagreement regarding AVM diffuseness occurred in 43.8% of all DSA views (n = 80). Interobserver κ agreement on AVM diffuseness using DSA views among 4 physicians ranged from fair (κ = 0.40 [95% confidence interval (CI) = 0.22-0.58]) to substantial (κ = 0.65 [95% CI = 0.48-0.81]), whereas total intraclass correlation coefficient was 0.81 (95% CI = 0.73-0.87). For the 36 AVMs, κ agreement ranged from fair (κ = 0.36 [95% CI = 0.13-0.60]) to moderate (κ = 0.57 [95% CI = 0.35-0.79]), whereas intraclass correlation coefficient among all 4 physicians was 0.68 (95% CI = 0.47-0.82). Moderate agreement on AVM diffuseness (n = 80) was found between attending and resident assessments (κ = 0.57 [95% CI = 0.39-0.75]) and between neurosurgeon and interventional neuroradiologist assessments (κ = 0.55 [95% CI = 0.37-0.73]). Agreement of individual physicians on AVM diffuseness varies from fair to substantial. Objective and three-dimensional measures of AVM diffuseness should be developed for consistent clinical application. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. [Multiple coronary arteriovenous fistulae. Hazard or predetermination?].

    PubMed

    Rangel, Alberto; Muñoz-Castellanos, Luis; Solorio, Sergio

    2003-01-01

    The authors present the clinical cases of three adult patients (49, 53 and 61 year-old), with rheumatic cardiac valvulopathy, and bilateral coronary arteriovenous fistulae draining in the main pulmonary artery. Based on documental investigation, the authors speculate about the predeterminate origin of coronary arteriovenous fistulae. At first glance, it seems obvious that congenital cardiopathies occur at random, i.e., embryonic development deviate or stops due to unknown reasons, originating the persistence of lacunar blood spaces prior to the development of coronary arteries cords. There are two factors involved in the genesis of congenital malformations: a genomic preexisting factor and the presence of an environmental precipitating factor, i.e., isolated pulmonary valve atresia or left ventricular hypoplastic syndrome, with mitral and aortic valve stenosis, can predispose development of coronary arteriovenous fistulae. Recently, the question has been raised whether there is a relation of coronary arteries fistulae with: ethnic groups, hereditary gigantism, autoimmune diseases, such as polymyositis, hereditary hemorrhagic telangiectasia, and apical hypertrophic myocardiopathy. Coronary arteriovenous fistulae, as well as some congenital cardiopathies, could be due to chromosome alterations or might be related to hereditary diseases, such as hemorrhagic telangiectasia, induced by a disturbed genetic program. Although, there is no concrete evidence that a genetic factor is related to the development of coronary arteriovenous fistulae, there are signs that suggest that such a possibility could be investigated.

  10. Onyx embolization of intracranial arteriovenous malformations in pediatric patients.

    PubMed

    Soltanolkotabi, Maryam; Schoeneman, Samantha E; Alden, Tord D; Hurley, Michael C; Ansari, Sameer A; DiPatri, Arthur J; Tomita, Tadanori; Shaibani, Ali

    2013-04-01

    The authors undertook this study to assess the safety and efficacy of Onyx embolization in the treatment of intracranial arteriovenous malformations (AVMs) in pediatric patients. All pediatric Onyx embolization of intracranial AVM cases performed consecutively at a single children's hospital over a 5-year period were collected and evaluated. Twenty-five patients (mean age 10.5 years) underwent a total of 38 procedures. An aggregate of 56 pedicles were embolized (mean 1.47 per session). The Spetzler-Martin grade was determined in all cases. Onyx embolization resulted in complete obliteration of the AVM in 3 cases (12%) and partial obliteration in 22 cases (88%). A total of 23 patients underwent surgical treatment. The mean preoperative AVM devascularization in these cases was 72%. One patient was treated with radiosurgery following Onyx embolization. Overall, 10 complications occurred in a total of 38 procedures (26.3%). None of the complications resulted in permanent neurological morbidity. The rate of transient neurological complications was 10.5% (4 of 38 procedures) and the rate of transient nonneurological complications was 5.3% (2 of 38 procedures). The remaining 4 complications were clinically silent (rate of 10.5%). There were no procedure-related deaths in this study population. There was no significant difference in patients with and without complications in terms of demographic characteristics, AVM grade, or embolization features (p ≥ 0.2). Deep venous drainage was associated with higher complication rates (p = 0.03). Onyx utilization is feasible for preoperative or primary embolization in the treatment of pediatric intracranial AVMs; however, the spectrum of complications encountered is broad, and attention must be paid to the technical nuances of and indications for its use to avoid many potential dangerous effects. Although the overall complication rates were higher than expected, all were either clinically silent or had only transient clinical

  11. Features predictive of brain arteriovenous malformation hemorrhage: extrapolation to a physiologic model.

    PubMed

    Sahlein, Daniel H; Mora, Paloma; Becske, Tibor; Huang, Paul; Jafar, Jafar J; Connolly, E Sander; Nelson, Peter K

    2014-07-01

    Although there is generally thought to be a 2% to 4% per annum rupture risk for brain arteriovenous malformations (bAVMs), there is no way to estimate risk for an individual patient. In this retrospective study, patients were eligible who had nidiform bAVMs and underwent detailed pretreatment diagnostic cerebral angiography at our medical center from 1996 to 2006. All patients had superselective microcatheter angiography, and films were reviewed for the purpose of this project. Patient demographics, clinical presentation, and angioarchitectural characteristics were analyzed. A univariate analysis was performed, and angioarchitectural features with potential physiological significance that showed at least a trend toward significance were added to a multivariate logistic regression model. One hundred twenty-two bAVMs met criteria for study entry. bAVMs with single venous drainage anatomy were more likely to present with hemorrhage. In addition, patients with multiple draining veins and a venous stenosis reverted to a risk similar to those with 1 draining vein, whereas those with multiple draining veins and without stenosis had diminished association with hemorrhage presentation. Those bAVMs with associated aneurysms were more likely to present with hemorrhage. These findings were robust in both univariate and multivariate models. The results of this article lead to the first physiological, internally consistent model of individual bAVM hemorrhage risk, where 1 draining vein, venous stenosis, and associated aneurysms increase risk. © 2014 American Heart Association, Inc.

  12. Microsurgical management of a high-grade brain arteriovenous malformation in the central lobe after unsuccessful radiosurgery.

    PubMed

    de Oliveira, Jean G; Massella, Carlos R; de Holanda, Carlos Vanderlei M; Giudicissi-Filho, Miguel; Borba, Luis A B

    2017-07-01

    The recommendation for the great majority of high-grade brain arteriovenous malformations (bAVMs) is observation, except for those patients with recurrent hemorrhages, progressive neurological deficits, steal-related symptoms, or AVM-related aneurysms, for whom intervention should be considered. These are general recommendations, and the decision should be made on an individual basis. Surgical resection of an AVM in the central lobe may cause postoperative sensorimotor deficits since this anatomical region includes the pre- and postcentral gyri on the lateral surface and the paracentral lobule on the medial surface. The authors present a patient with a ruptured high-grade bAVM in the central lobe who underwent previous surgery for hematoma evacuation and previous radiosurgery, and whose indication for reoperation was proposed based on progressive hemiparesis. Microsurgical resection was possible after a wide frontoparietal craniotomy, which made all the nidus borders accessible. This case illustrates the anatomy and surgical technique for large nidus AVMs in eloquent areas, showing that complete microsurgical resection is possible with good clinical outcome. The video can be found here: https://youtu.be/Cpd1PK6BLIM .

  13. Supplementary motor area activation in patients with frontal lobe tumors and arteriovenous malformations.

    PubMed

    Sailor, Janet; Meyerand, M Elizabeth; Moritz, Chad H; Fine, Jason; Nelson, Lindsey; Badie, Behnam; Haughton, Victor M

    2003-10-01

    Some patients who undergo surgical resection of portions of the supplementary motor area (SMA) have severe postoperative motor and language deficits, whereas others have no deficits. We tested the hypothesis that in some patients with lesions affecting the SMA, the contralateral SMA exhibits some of the activation normally associated with the ipsilateral SMA. Functional MR imaging studies in seven healthy volunteers and 19 patients with frontal lobe tumors or arteriovenous malformations were reviewed retrospectively. The hemisphere in which the SMA activation predominated was tabulated for right and left motor tasks. The relative hemispheric dominance in the SMA for the right and left motor tasks was compared in the healthy and patient groups and with the location of the lesion in the patient group. None of the control subjects performing a right hand motor task activated predominantly the right SMA. Fifty percent of the patients with lesions overlapping the left SMA performing the right motor task activated predominantly the right SMA. Fifty-seven percent of control subjects performing the left hand motor task activated the left SMA predominantly. One hundred percent of patients with lesions overlapping the right frontal SMA performing the left motor task activated the left SMA predominantly. Differences between patients and controls were statistically significant. A lesion that contacts or overlaps the SMA is associated with an increased functional MR imaging response within the contralateral SMA.

  14. Augmented reality in the surgery of cerebral arteriovenous malformations: technique assessment and considerations.

    PubMed

    Cabrilo, Ivan; Bijlenga, Philippe; Schaller, Karl

    2014-09-01

    Augmented reality technology has been used for intraoperative image guidance through the overlay of virtual images, from preoperative imaging studies, onto the real-world surgical field. Although setups based on augmented reality have been used for various neurosurgical pathologies, very few cases have been reported for the surgery of arteriovenous malformations (AVM). We present our experience with AVM surgery using a system designed for image injection of virtual images into the operating microscope's eyepiece, and discuss why augmented reality may be less appealing in this form of surgery. N = 5 patients underwent AVM resection assisted by augmented reality. Virtual three-dimensional models of patients' heads, skulls, AVM nidi, and feeder and drainage vessels were selectively segmented and injected into the microscope's eyepiece for intraoperative image guidance, and their usefulness was assessed in each case. Although the setup helped in performing tailored craniotomies, in guiding dissection and in localizing drainage veins, it did not provide the surgeon with useful information concerning feeder arteries, due to the complexity of AVM angioarchitecture. The difficulty in intraoperatively conveying useful information on feeder vessels may make augmented reality a less engaging tool in this form of surgery, and might explain its underrepresentation in the literature. Integrating an AVM's hemodynamic characteristics into the augmented rendering could make it more suited to AVM surgery.

  15. Vascular deficiency of Smad4 causes arteriovenous malformations: a mouse model of Hereditary Hemorrhagic Telangiectasia.

    PubMed

    Crist, Angela M; Lee, Amanda R; Patel, Nehal R; Westhoff, Dawn E; Meadows, Stryder M

    2018-05-01

    Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder that leads to abnormal connections between arteries and veins termed arteriovenous malformations (AVM). Mutations in TGFβ pathway members ALK1, ENG and SMAD4 lead to HHT. However, a Smad4 mouse model of HHT does not currently exist. We aimed to create and characterize a Smad4 endothelial cell (EC)-specific, inducible knockout mouse (Smad4 f/f ;Cdh5-Cre ERT2 ) that could be used to study AVM development in HHT. We found that postnatal ablation of Smad4 caused various vascular defects, including the formation of distinct AVMs in the neonate retina. Our analyses demonstrated that increased EC proliferation and size, altered mural cell coverage and distorted artery-vein gene expression are associated with Smad4 deficiency in the vasculature. Furthermore, we show that depletion of Smad4 leads to decreased Vegfr2 expression, and concurrent loss of endothelial Smad4 and Vegfr2 in vivo leads to AVM enlargement. Our work provides a new model in which to study HHT-associated phenotypes and links the TGFβ and VEGF signaling pathways in AVM pathogenesis.

  16. Absolute Ethanol Embolisation of Mandibular Arteriovenous Malformations Following Direct Percutaneous Puncture and Release of Coils via a Microcatheter.

    PubMed

    Wang, D; Su, L; Han, Y; Wang, Z; Zheng, L; Fan, X

    2017-06-01

    To evaluate the safety, efficacy, and medium-term outcome of a modified technique of ethanol embolisation of mandibular arteriovenous malformations (AVMs) following a direct percutaneous transvenous approach to the release of coils via a microcatheter. From January 2012 to July 2014, 18 consecutive patients (mean age 20.9 years [range 10-35 years]) with symptomatic AVMs of the mandible were enrolled. A microcatheter was inserted into the lesion via a direct percutaneous puncture needle. Electrolytically detachable coils and 0.018 mm coils were super-selectively placed to decrease the flow and volume of the arteriovenous fistulas via a microcatheter. Absolute ethanol was injected to obliterate the fistulas. Clinical follow-up was performed in all patients. Therapeutic outcomes were determined by evaluating the degree of devascularisation at follow-up angiography and symptoms and signs. Transvenous release of coils combined with absolute ethanol embolisation were used in all cases. The amount of ethanol used ranged from 5 to 50 mL (mean 25.7 mL) in a single session. Sixteen of 18 patients were cured, and two had partial remission. Follow-up times ranged from 8 to 26 months (medium 15.7 months), and there was no angiographic recurrence of the lesions. Minor complication occurred in five of the 18 patients. There were no major complications. Absolute ethanol embolisation following a direct percutaneous transvenous approach to release coils via a microcatheter is a feasible, safe, and highly effective method for the management of mandibular AVMs. Copyright © 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

  17. Incorporate Imaging Characteristics Into an Arteriovenous Malformation Radiosurgery Plan Evaluation Model

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Zhang Pengpeng; Department of Medical Physics, Memorial Sloan-Kettering Cancer Center, New York, NY; Wu, Leester

    Purpose: To integrate imaging performance characteristics, specifically sensitivity and specificity, of magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) into arteriovenous malformation (AVM) radiosurgery planning and evaluation. Methods and Materials: Images of 10 patients with AVMs located in critical brain areas were analyzed in this retrospective planning study. The image findings were first used to estimate the sensitivity and specificity of MRA and DSA. Instead of accepting the imaging observation as a binary (yes or no) mapping of AVM location, our alternative is to translate the image into an AVM probability distribution map by incorporating imagers' sensitivity and specificity,more » and to use this map as a basis for planning and evaluation. Three sets of radiosurgery plans, targeting the MRA and DSA positive overlap, MRA positive, and DSA positive were optimized for best conformality. The AVM obliteration rate (ORAVM) and brain complication rate served as endpoints for plan comparison. Results: In our 10-patient study, the specificities and sensitivities of MRA and DSA were estimated to be (0.95, 0.74) and (0.71, 0.95), respectively. The positive overlap of MRA and DSA accounted for 67.8% {+-} 4.9% of the estimated true AVM volume. Compared with plans targeting MRA and DSA-positive overlap, plans targeting MRA-positive or DSA-positive improved ORAVM by 4.1% {+-} 1.9% and 15.7% {+-} 8.3%, while also increasing the complication rate by 1.0% {+-} 0.8% and 4.4% {+-} 2.3%, respectively. Conclusions: The impact of imagers' quality should be quantified and incorporated in AVM radiosurgery planning and evaluation to facilitate clinical decision making.« less

  18. Transarterial Balloon-assisted Onyx Embolization of Intracranial Arteriovenous Malformations Using a Dual-lumen Balloon Microcatheter: Two Case Reports.

    PubMed

    Kim, Sang Heum; Kim, Tae Gon; Kong, Min Ho

    2017-09-01

    The Onyx system has been well established in recent years as a very important material in the treatment of arteriovenous malformations (AVMs). When using the Onyx, it is essential to wait for the creation of a plug around the tip of the catheter, which enables the effective forward penetration of Onyx. Recent reports have shown that the introduction of a dimethyl sulfoxide compatible dual-lumen balloon microcatheter improves the efficiency of AVM embolization. We report our recent experience of two cases of intracranial AVM embolization using Onyx and the transarterial balloon-assisted technique. In both cases, the procedures were successfully performed and the nidus of the AVM was totally occluded in a relatively short time. This technique may enable immediate forward flow and penetration of Onyx without concern about reflux. It may also reduce the procedure time and increase the angiographic occlusion rate. Navigation of the dual-lumen balloon microcatheter nevertheless remains a challenge.

  19. Transarterial Balloon-assisted Onyx Embolization of Intracranial Arteriovenous Malformations Using a Dual-lumen Balloon Microcatheter: Two Case Reports

    PubMed Central

    Kim, Sang Heum; Kong, Min Ho

    2017-01-01

    The Onyx system has been well established in recent years as a very important material in the treatment of arteriovenous malformations (AVMs). When using the Onyx, it is essential to wait for the creation of a plug around the tip of the catheter, which enables the effective forward penetration of Onyx. Recent reports have shown that the introduction of a dimethyl sulfoxide compatible dual-lumen balloon microcatheter improves the efficiency of AVM embolization. We report our recent experience of two cases of intracranial AVM embolization using Onyx and the transarterial balloon-assisted technique. In both cases, the procedures were successfully performed and the nidus of the AVM was totally occluded in a relatively short time. This technique may enable immediate forward flow and penetration of Onyx without concern about reflux. It may also reduce the procedure time and increase the angiographic occlusion rate. Navigation of the dual-lumen balloon microcatheter nevertheless remains a challenge. PMID:29159158

  20. Penfield's ceiling: Seeing brain injury through Galen's eyes.

    PubMed

    Adams, Zoe M; Fins, Joseph J

    2017-08-22

    The cathedral ceiling located in the entrance hall of the Montreal Neurological Institute, planned by its founder Wilder Penfield, has intrigued visitors since it was erected in 1934. Central to its charm is a cryptic comment by the ancient physician Galen of Pergamum, which refutes a dire Hippocratic aphorism about prognosis in brain injury. Galen's optimism, shared by Penfield, is curious from a fellow ancient. In this article, we use primary sources in Ancient Greek as well as secondary sources to not only examine the origins of Galen's epistemology but also, using a methodology in classics scholarship known as reception studies , illustrate how an awareness of this ancient debate can illuminate contemporary clinical contexts. While Galen based his prognostications on direct clinical observations like the Hippocratics, he also engaged in experimental and anatomic work in both animals and humans, which informed his views on neurologic states and outcomes. Penfield's memorialization of Galen is representative of the evolution of the neurosciences and the ongoing importance of evidence-based prognostication in severe brain injury. © 2017 American Academy of Neurology.

  1. Impact on cognitive functions following gamma knife radiosurgery for cerebral arteriovenous malformations

    PubMed Central

    Raghunath, A.; Bennett, Niranjana; Arimappamagan, Arivazhagan; Bhat, Dhananjaya I.; Srinivas, Dwarakanath; Thennarasu, K.; Jamuna, R.; Somanna, Sampath

    2016-01-01

    Background: Radiosurgery is an alternative to surgical resection of arteriovenous malformation (AVM). Very few studies have addressed the concern of radiation injury to the brain and its attendant adverse effects on cognitive function. Materials and Methods: This prospective study included all patients who underwent gamma knife radiosurgery (GKRS) at our institute for cerebral AVM between 2006 and December 2008 (n = 34). All patients underwent neuropsychological evaluation before the procedure. Neuropsychological evaluation was repeated in eighteen patients 2 years following GKRS. Clinical outcome, AVM obliteration, and factors influencing outcome were analyzed in these eighteen patients. Results: Before GKRS, more than 50% had significant impairment of neuropsychological functions compared to normal population norms. 66.6% achieved the excellent radiosurgical outcome. At 2 years follow-up, patients showed varied improvement in neuropsychological function in various categories. Pretherapeutic median value for percentage perseverative responses was 26.5 and at follow-up, it reduced to 18.2 (P = 0.039). Set shifting improved in 11 patients (61.1%), remained same in 5 patients (27.7%), and deteriorated in two patients (11.1%). Patients with a higher Spetzler-Martin grade AVM demonstrated a significantly more favorable shift in follow-up test values for set shifting function (P = 0.021). Patients with postradiation imaging changes had lesser tendency to improve in neuropsychological performance at follow-up. Conclusions: GKRS has no clinically harmful effect on cognitive and neuropsychological functioning in patients with brain AVM. On the contrary, there is an improvement in majority of patients at 2 years following radiosurgery when nidus is obliterated. PMID:26933340

  2. Impact on cognitive functions following gamma knife radiosurgery for cerebral arteriovenous malformations.

    PubMed

    Raghunath, A; Bennett, Niranjana; Arimappamagan, Arivazhagan; Bhat, Dhananjaya I; Srinivas, Dwarakanath; Thennarasu, K; Jamuna, R; Somanna, Sampath

    2016-01-01

    Radiosurgery is an alternative to surgical resection of arteriovenous malformation (AVM). Very few studies have addressed the concern of radiation injury to the brain and its attendant adverse effects on cognitive function. This prospective study included all patients who underwent gamma knife radiosurgery (GKRS) at our institute for cerebral AVM between 2006 and December 2008 (n = 34). All patients underwent neuropsychological evaluation before the procedure. Neuropsychological evaluation was repeated in eighteen patients 2 years following GKRS. Clinical outcome, AVM obliteration, and factors influencing outcome were analyzed in these eighteen patients. Before GKRS, more than 50% had significant impairment of neuropsychological functions compared to normal population norms. 66.6% achieved the excellent radiosurgical outcome. At 2 years follow-up, patients showed varied improvement in neuropsychological function in various categories. Pretherapeutic median value for percentage perseverative responses was 26.5 and at follow-up, it reduced to 18.2 (P = 0.039). Set shifting improved in 11 patients (61.1%), remained same in 5 patients (27.7%), and deteriorated in two patients (11.1%). Patients with a higher Spetzler-Martin grade AVM demonstrated a significantly more favorable shift in follow-up test values for set shifting function (P = 0.021). Patients with postradiation imaging changes had lesser tendency to improve in neuropsychological performance at follow-up. GKRS has no clinically harmful effect on cognitive and neuropsychological functioning in patients with brain AVM. On the contrary, there is an improvement in majority of patients at 2 years following radiosurgery when nidus is obliterated.

  3. Microsurgery Versus Stereotactic Radiosurgery for Brain Arteriovenous Malformations: A Matched Cohort Study.

    PubMed

    Chen, Ching-Jen; Ding, Dale; Wang, Tony R; Buell, Thomas J; Ilyas, Adeel; Ironside, Natasha; Lee, Cheng-Chia; Kalani, M Yashar; Park, Min S; Liu, Kenneth C; Sheehan, Jason P

    2018-05-12

    Microsurgery (MS) and stereotactic radiosurgery (SRS) remain the preferred interventions for the curative treatment of brain arteriovenous malformations (AVM), but their relative efficacy remains incompletely defined. To compare the outcomes of MS to SRS for AVMs through a retrospective, matched cohort study. We evaluated institutional databases of AVM patients who underwent MS and SRS. MS-treated patients were matched, in a 1:1 ratio based on patient and AVM characteristics, to SRS-treated patients. Statistical analyses were performed to compare outcomes data between the 2 cohorts. The primary outcome was defined as AVM obliteration without a new permanent neurological deficit. The matched MS and SRS cohorts were each comprised of 59 patients. Both radiological (85 vs 11 mo; P < .001) and clinical (92 vs 12 mo; P < .001) follow-up were significantly longer for the SRS cohort. The primary outcome was achieved in 69% of each cohort. The MS cohort had a significantly higher obliteration rate (98% vs 72%; P = .001), but also had a significantly higher rate of new permanent deficit (31% vs 10%; P = .011). The posttreatment hemorrhage rate was significantly higher for the SRS cohort (10% for SRS vs 0% for MS; P = .027). In subgroup analyses of ruptured and unruptured AVMs, no significant differences between the primary outcomes were observed. For patients with comparable AVMs, MS and SRS afford similar rates of deficit-free obliteration. Nidal obliteration is more frequently achieved with MS, but this intervention also incurs a greater risk of new permanent neurological deficit.

  4. Management of intracranial aneurysms associated with arteriovenous malformations.

    PubMed

    Flores, Bruno C; Klinger, Daniel R; Rickert, Kim L; Barnett, Samuel L; Welch, Babu G; White, Jonathan A; Batjer, H Hunt; Samson, Duke S

    2014-09-01

    Intracranial or brain arteriovenous malformations (BAVMs) are some of the most interesting and challenging lesions treated by the cerebrovascular neurosurgeon. It is generally believed that the combination of BAVMs and intracranial aneurysms (IAs) is associated with higher hemorrhage rates at presentation and higher rehemorrhage rates and thus with a more aggressive course and natural history. There is wide variation in the literature on the prevalence of BAVM-associated aneurysms (range 2.7%-58%), with 10%-20% being most often cited in the largest case series. The risk of intracranial hemorrhage in patients with unruptured BAVMs and coexisting IAs has been reported to be 7% annually, compared with 2%-4% annually for those with BAVM alone. Several different classification systems have been applied in an attempt to better understand the natural history of this combination of lesions and implications for treatment. Independent of the classification used, it is clear that a few subtypes of aneurysms have a direct hemodynamic correlation with the BAVM itself. This is exemplified by the fact that the presence of a distal flow-related or an intranidal aneurysm appears to be associated with an increased hemorrhage risk, when compared with an aneurysm located on a vessel with no direct supply to the BAVM nidus. Debate still exists regarding the etiology of the association between those two vascular lesions, the subsequent implications for patients' risk of hemorrhagic stroke, and finally the determination of which patients warrant treatment and when. The ultimate goals of the treatment of a BAVM associated with an IA are to prevent hemorrhage, avoid stepwise neurological deterioration, and eliminate the mortality risk associated with recurrent hemorrhagic events. The treatment is only justifiable if the risks associated with an intervention are lower than or equivalent to the long-term risks of disability or mortality caused by the lesion itself. When faced with this

  5. The Roman Empire legacy of Galen (129-200 AD).

    PubMed

    Shoja, Mohammadali M; Tubbs, R Shane; Ghabili, Kamyar; Griessenauer, Christoph J; Balch, Margaret Wood; Cuceu, Mariana

    2015-01-01

    Galen of Pergamum was the physician of Roman Emperors and contributed to our early understanding of medicine and anatomy. Herein, we present a short biography of Galen and review his multiple contributions to medicine and anatomy. Although it has been almost 2,000 years since Galen walked the streets of the Roman Empire, his legacy continues via multiple eponyms that bare his name.

  6. [Paradoxical brain embolism mediated through a pulmonary arteriovenous malformation with hereditary hemorrhagic telangiectasia in a Japanese patient].

    PubMed

    Takeda, June; Todo, Kenichi; Yamamoto, Shiro; Yamagami, Hiroshi; Kawamoto, Michi; Kohara, Nobuo

    2012-01-01

    We report a case of paradoxical brain embolism mediated through a pulmonary arteriovenous malformation (PAVM) with hereditary hemorrhagic telangiectasia (HHT). A 25-year-old right handed man was admitted to our hospital after sudden headache and visual field abnormality. In neurologic examinations, he had left superior-quadrantanopsia. Laboratory findings showed iron deficiency anemia. Diffusion weighted images disclosed a high-signal-intensity area in the right occipito-temporal lobe, and intraarterial digital subtraction cerebral angiography revealed occlusion of the right posterior cerebral artery. Transesophageal echocardiography revealed continuous right-to-left shunt. We confirmed his history of spontaneous recurrent epistaxis and the first-degree relatives with epistaxis or PAVM. A contrast enhanced CT scan of the chest revealed a PAVM. The diagnosis of paradoxical brain embolism mediated through the PAVM with HHT was, thus, established. The PAVM was occluded by using embolization coils successfully. In Asian countries, the prevalence of PAVM with HHT is thought to be lower than in European countries. We should carefully take medical and family histories, especially epistaxis, in a young stroke patient.

  7. Hippocrates as Galen's teacher.

    PubMed

    Jouanna, Jacques

    2010-01-01

    Starting from the frescoes of the cathedral of Anagni which present an obvious relationship between Hippocrates as Galen's teacher and the medieval image of man's place in the universe dominated by the number four, this paper returns to the origins of this quaternary theory in Hippocratic medicine with the four humors (Nature of Man), then follows its evolution in Galen and finally into late Greek and Byzantine medicine where the quaternary division will have an unprecedented extension, with the four temperaments. In particular, a new piece of evidence from this late period attributed to Hippocrates (the small treatise of Greek Medicine The Pulse and the Human Temperament) appears as the veritable source of the Latin Letter attributed to Vindicianus. Therefore, contrary to what was believed until now, the doctrine of the four temperaments was not elaborated first in a Latin form. Throughout its history, the quaternary theory will remain connected to Hippocrates, but the image and teaching of the Father of Medicine will change as the theory evolves. A second rediscovered treatise of the late period (The Formation of Man) starts with this phrase: 'Words of Hippocrates to Galen his own pupil'. This seems a felicitous commentary to the medical scene in the cathedral of Anagni.

  8. Ligation of Macroscopically Detectable Arteriovenous Fistulas in Stewart-Bluefarb Syndrome

    PubMed Central

    Sung, Shih-Ying; Lin, Yi-Chang; Tsai, Yi-Ting; Lin, Chih-Yuan; Lee, Chung-Yi; Tsai, Chien-Sung

    2014-01-01

    We herein describe the case of a 21-year-old woman with Stewart-Bluefarb syndrome presenting with recurrent ulcers on the right foot and multiple congenital arteriovenous malformations. The painful recurrent ulcers and brownish macules at the dorsum of the right foot had appeared at 13 years of age, and the size of the right foot gradually became larger than the left. She underwent conservative treatment and polyvinyl alcohol embolization but the ulcer was recurrent. Two macroscopic detectable feeding arteries to arteriovenous fistulas were ligated under Doppler sonography. At her 6 month follow-up, the chronic ulcer had begun to heal and pain had been alleviated. PMID:27122807

  9. Endovascular balloon-assisted embolization of intracranial and cervical arteriovenous malformations using dual-lumen coaxial balloon microcatheters and Onyx: initial experience.

    PubMed

    Jagadeesan, Bharathi D; Grigoryan, Mikayel; Hassan, Ameer E; Grande, Andrew W; Tummala, Ramachandra P

    2013-12-01

    Ethylene vinyl alcohol copolymer (Onyx) is widely used for the embolization of arteriovenous malformations (AVMs) of the brain, head, and neck. Balloon-assisted Onyx embolization may provide additional unique advantages in the treatment of AVMs in comparison with traditional catheter-based techniques. To report our initial experience in performing balloon-assisted AVM embolization for brain and neck AVMs with the use of the new Scepter-C and Scepter-XC coaxial dual-lumen balloon microcatheters. Balloon-assisted transarterial embolization was performed in a series of 7 patients with AVMs (4 with brain AVMs, 1 with a dural arteriovenous fistula, and 2 with neck AVMs) by using Onyx delivered through the lumen of Scepter-C or Scepter XC coaxial balloon microcatheters. Following the initial balloon-catheter navigation into a feeding artery and the subsequent inflation of the balloon, the embolization was performed by using Onyx 18, Onyx 34, or both. A total of 12 embolization sessions were performed via 17 arterial feeders in these 7 patients. In 1 patient, there was an arterial perforation from the inflation of the balloon; in all others, the embolization goals were successfully achieved with no adverse events. The balloon microcatheters showed excellent navigability, and there were no problems with retrieval or with the repeated inflation and deflation of the balloons. A proximal Onyx plug, which is crucial in many AVM embolizations, was not necessary with this technique. Additionally, fluoroscopy and procedural times seemed lower with this technique compared with conventional embolization methods.

  10. [N. Leonicenus interpretes of Galen on causa coniuncta].

    PubMed

    Mugnai, Daniela

    2010-01-01

    The philosopher and physician Nicolò Leoniceno, one of the most important members of the Medical Humanism, in the N. Leoniceni in libros Galeni e Graeca in linguam Latinam a se translatos Praefatio communis (1508) discusses his emendation to Galens's Ars Medicinalis (28, 4 Boudon = I 381 Kühn, [see text]). In spite of the debatable conjecture, it is a significant effort to solve a serious contradiction in Galen's text. Leoniceno rejects the solutions proposed by the Arabic and Medieval Latin commentators and offers the right interpretation of causa coniuncta in Galen's concept of disease.

  11. Elaboration of a semi-automated algorithm for brain arteriovenous malformation segmentation: initial results.

    PubMed

    Clarençon, Frédéric; Maizeroi-Eugène, Franck; Bresson, Damien; Maingreaud, Flavien; Sourour, Nader; Couquet, Claude; Ayoub, David; Chiras, Jacques; Yardin, Catherine; Mounayer, Charbel

    2015-02-01

    The purpose of our study was to distinguish the different components of a brain arteriovenous malformation (bAVM) on 3D rotational angiography (3D-RA) using a semi-automated segmentation algorithm. Data from 3D-RA of 15 patients (8 males, 7 females; 14 supratentorial bAVMs, 1 infratentorial) were used to test the algorithm. Segmentation was performed in two steps: (1) nidus segmentation from propagation (vertical then horizontal) of tagging on the reference slice (i.e., the slice on which the nidus had the biggest surface); (2) contiguity propagation (based on density and variance) from tagging of arteries and veins distant from the nidus. Segmentation quality was evaluated by comparison with six frame/s DSA by two independent reviewers. Analysis of supraselective microcatheterisation was performed to dispel discrepancy. Mean duration for bAVM segmentation was 64 ± 26 min. Quality of segmentation was evaluated as good or fair in 93% of cases. Segmentation had better results than six frame/s DSA for the depiction of a focal ectasia on the main draining vein and for the evaluation of the venous drainage pattern. This segmentation algorithm is a promising tool that may help improve the understanding of bAVM angio-architecture, especially the venous drainage. • The segmentation algorithm allows for the distinction of the AVM's components • This algorithm helps to see the venous drainage of bAVMs more precisely • This algorithm may help to reduce the treatment-related complication rate.

  12. Radiosurgery for cerebral arteriovenous malformation during pregnancy: A case report focusing on fetal exposure to radiation

    PubMed Central

    Nagayama, Kazuki; Kurita, Hiroki; Tonari, Ayako; Takayama, Makoto; Shiokawa, Yoshiaki

    2010-01-01

    Introduction: We present the case of a pregnant woman who underwent linear accelerator (LINAC)-based stereotactic radiosurgery (SRS) and we discuss the fetal exposure to radiation. Clinical Presentation: A 20-year-old woman at 18 weeks of gestation presented with right cerebral hemorrhage and underwent urgent evacuation of the hematoma. She recovered well after surgery, but cerebral angiography after the surgery revealed a small deeply seated arteriovenous malformation (AVM) in the right frontal lobe extending to the right basal ganglia. Methods and Results: We examined the diffuse AVM and treated it with LINAC-based SRS at 24 weeks of gestation. Before SRS, the fetus was exposed to a radiation dose of 8.26 mGy, which was estimated by conducting an experiment using an adult RANDO phantom, and a radiophotoluminescent (RPL) glass rod dosimeter (GRD) system. The patient underwent Caesarean delivery at 36 weeks of gestation and gave birth to a healthy baby. Conclusion: The exposure of fetus to radiation during SRS was exceedingly low. SRS can be used as an alternative treatment to microsurgery for resolving small deeply seated AVMs even in pregnant patients. PMID:22028762

  13. Effect of the embolization material in the dose calculation for stereotactic radiosurgery of arteriovenous malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Galván de la Cruz, Olga Olinca; Lárraga-Gutiérrez, José Manuel, E-mail: jlarraga@innn.edu.mx; Laboratorio de Física Médica, Instituto Nacional de Neurología y Neurocirugía

    2013-07-01

    It is reported in the literature that the material used in an embolization of an arteriovenous malformation (AVM) can attenuate the radiation beams used in stereotactic radiosurgery (SRS) up to 10% to 15%. The purpose of this work is to assess the dosimetric impact of this attenuating material in the SRS treatment of embolized AVMs, using Monte Carlo simulations assuming clinical conditions. A commercial Monte Carlo dose calculation engine was used to recalculate the dose distribution of 20 AVMs previously planned with a pencil beam dose calculation algorithm. Dose distributions were compared using the following metrics: average, minimal and maximummore » dose of AVM, and 2D gamma index. The effect in the obliteration rate was investigated using radiobiological models. It was found that the dosimetric impact of the embolization material is less than 1.0 Gy in the prescription dose to the AVM for the 20 cases studied. The impact in the obliteration rate is less than 4.0%. There is reported evidence in the literature that embolized AVMs treated with SRS have low obliteration rates. This work shows that there are dosimetric implications that should be considered in the final treatment decisions for embolized AVMs.« less

  14. Development of three-dimensional brain arteriovenous malformation model for patient communication and young neurosurgeon education.

    PubMed

    Dong, Mengqi; Chen, Guangzhong; Qin, Kun; Ding, Xiaowen; Zhou, Dong; Peng, Chao; Zeng, Shaojian; Deng, Xianming

    2018-01-15

    Rapid prototyping technology is used to fabricate three-dimensional (3D) brain arteriovenous malformation (AVM) models and facilitate presurgical patient communication and medical education for young surgeons. Two intracranial AVM cases were selected for this study. Using 3D CT angiography or 3D rotational angiography images, the brain AVM models were reconstructed on personal computer and the rapid prototyping process was completed using a 3D printer. The size and morphology of the models were compared to brain digital subtraction arteriography of the same patients. 3D brain AVM models were used for preoperative patient communication and young neurosurgeon education. Two brain AVM models were successfully produced. By neurosurgeons' evaluation, the printed models have high fidelity with the actual brain AVM structures of the patients. The patient responded positively toward the brain AVM model specific to himself. Twenty surgical residents from residency programs tested the brain AVM models and provided positive feedback on their usefulness as educational tool and resemblance to real brain AVM structures. Patient-specific 3D printed models of brain AVM can be constructed with high fidelity. 3D printed brain AVM models are proved to be helpful in preoperative patient consultation, surgical planning and resident training.

  15. Retrieving microcatheters from Onyx casts in a series of brain arteriovenous malformations: a technical report.

    PubMed

    Alamri, A; Hyodo, A; Suzuki, K; Tanaka, Y; Uchida, T; Takano, I; Kowata, K; Iwatate, K; Suzuki, R

    2012-11-01

    To date, the "monorail snare technique" for the retrieval of entombed microcatheter tips during Onyx(TM) (ev3, Irvine, CA) embolisation of brain arteriovenous malformations (BAVM) has not been described. We report our experiences and some technical aspects in using this technique for the retrieval of entombed Marathon(TM) microcatheter (ev3, Plymouth, MN) tips during Onyx embolisation of BAVM treatment. Onyx was used in the embolisation of 11 patients using 25 feeders over 14 sessions. The 'monorail snare technique' was employed for 14 feeders. Each time, an Amplatz 4 mm Gooseneck Microsnare(TM) (ev3, Plymouth, MN) was loaded into an Excelsior 1018(TM) microcatheter (Boston Scientific, Natick, MA). The Marathon microcatheter was cut just distal to the hub, and the Amplatz/Excelsior combination was introduced along the length of the Marathon microcatheter towards its distal end, as far as possible. The embedded catheter was ensnared and both catheters were pulled free. Microcatheter tip removal was successful in all cases, except for one microcatheter tip becoming detached and needing no further intervention. There were no complications as a direct result of the snare technique. The monorail snare technique is a safe and easy technique for retrieving Onyx-encased microcatheter tips in the treatment of BAVM.

  16. A 3-tier classification of cerebral arteriovenous malformations. Clinical article.

    PubMed

    Spetzler, Robert F; Ponce, Francisco A

    2011-03-01

    The authors propose a 3-tier classification for cerebral arteriovenous malformations (AVMs). The classification is based on the original 5-tier Spetzler-Martin grading system, and reflects the treatment paradigm for these lesions. The implications of this modification in the literature are explored. Class A combines Grades I and II AVMs, Class B are Grade III AVMs, and Class C combines Grades IV and V AVMs. Recommended management is surgery for Class A AVMs, multimodality treatment for Class B, and observation for Class C, with exceptions to the latter including recurrent hemorrhages and progressive neurological deficits. To evaluate whether combining grades is warranted from the perspective of surgical outcomes, the 3-tier system was applied to 1476 patients from 7 surgical series in which results were stratified according to Spetzler-Martin grades. Pairwise comparisons of individual Spetzler-Martin grades in the series analyzed showed the fewest significant differences (p < 0.05) in outcomes between Grades I and II AVMs and between Grades IV and V AVMs. In the pooled data analysis, significant differences in outcomes were found between all grades except IV and V (p = 0.38), and the lowest relative risks were found between Grades I and II (1.066) and between Grades IV and V (1.095). Using the pooled data, the predictive accuracies for surgical outcomes of the 5-tier and 3-tier systems were equivalent (receiver operating characteristic curve area 0.711 and 0.713, respectively). Combining Grades I and II AVMs and combining Grades IV and V AVMs is justified in part because the differences in surgical results between these respective pairs are small. The proposed 3-tier classification of AVMs offers simplification of the Spetzler-Martin system, provides a guide to treatment, and is predictive of outcome. The revised classification not only simplifies treatment recommendations; by placing patients into 3 as opposed to 5 groups, statistical power is markedly increased

  17. Management of Arteriovenous Malformations Associated with Developmental Venous Anomalies: A Literature Review and Report of 2 Cases.

    PubMed

    Zhang, Michael; Connolly, Ian D; Teo, Mario K; Yang, George; Dodd, Robert; Marks, Michael; Zuccarello, Mario; Steinberg, Gary K

    2017-10-01

    Classification of cerebrovascular malformations has revealed intermediary lesions that warrant further review owing to their unusual presentation and management. We present 2 cases of arteriovenous malformation (AVM) associated with a developmental venous anomaly (DVA), and discuss the efficacy of previously published management strategies. Two cases of AVMs associated with DVA were identified, and a literature search for published cases between 1980 and 2016 was conducted. Patient demographic data and clinical features were documented. In case 1, a 29-year-old female presenting with parenchymal hemorrhage and left homonymous hemianopia was found to have a right parieto-occipital AVM fed from the anterior cerebral, middle cerebral, and posterior cerebral arteries, with major venous drainage to the superior sagittal sinus. In case 2, imaging in a 34-year-old female evaluated for night tremors and incontinence revealed a left parietal AVM with venous drainage to the superior sagittal sinus. Including our 2 cases, 22 cases of coexisting AVMs and DVAs have been reported in the literature. At presentation, 68% had radiographic evidence of hemorrhage. Stereotactic radiosurgery was performed in 7 cases, embolization in 6 cases, surgical resection in 4 cases, and multimodal therapy in 5 cases. Radiography at follow-up demonstrated successful AVM obliteration in 67% of cases (12 of 18). Patients with coexisting AVMs and DVAs tend to have a hemorrhagic presentation. Contrary to traditional AVM management, in these cases it is important to preserve the draining vein via the DVA to ensure a safe, sustained circulatory outflow of the associated brain parenchyma while achieving safe AVM obliteration. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. Three-dimensional brain arteriovenous malformation models for clinical use and resident training.

    PubMed

    Dong, Mengqi; Chen, Guangzhong; Li, Jianyi; Qin, Kun; Ding, Xiaowen; Peng, Chao; Zhou, Dong; Lin, Xiaofeng

    2018-01-01

    To fabricate three-dimensional (3D) models of brain arteriovenous malformation (bAVM) and report our experience with customized 3D printed models of patients with bAVM as an educational and clinical tool for patients, doctors, and surgical residents. Using computerized tomography angiography (CTA) or digital subtraction angiography (DSA) images, the rapid prototyping process was completed with specialized software and "in-house" 3D printing service. Intraoperative validation of model fidelity was performed by comparing to DSA images of the same patient during the endovascular treatment process. 3D bAVM models were used for preoperative patient education and consultation, surgical planning, and resident training. 3D printed bAVM models were successful made. By neurosurgeons' evaluation, the printed models precisely replicated the actual bAVM structure of the same patients (n = 7, 97% concordance, range 95%-99% with average of < 2 mm variation). The use of 3D models was associated shorter time for preoperative patient education and consultation, higher acceptable of the procedure for patients and relatives, shorter time between obtaining intraoperative DSA data and the start of endovascular treatment. Thirty surgical residents from residency programs tested the bAVM models and provided feedback on their resemblance to real bAVM structures and the usefulness of printed solid model as an educational tool. Patient-specific 3D printed models of bAVM can be constructed with high fidelity. 3D printed bAVM models were proven to be helpful in preoperative patient consultation, surgical planning, and resident training. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

  19. Embolization as one modality in a combined strategy for the management of cerebral arteriovenous malformations.

    PubMed

    Raymond, J; Iancu, D; Weill, A; Guilbert, F; Bahary, J P; Bojanowski, M; Roy, D

    2005-10-05

    We attempted to assess clinical results of management of cerebral arteriovenous malformation using a combination of endovascular, surgical and radiotherapeutic approaches. We retrospectively reviewed the angiographic and clinical data on prospectively collected consecutive patients treated by embolization from 1994 to 2004. The general philosophy was to attempt treatment by a combination of approaches only when an angiographic cure was likely or at least possible. The clinical outcome was assessed according to the modified Rankin scale. Although 404 patients were collected, complete files and follow-ups are available for 227 or 56% only. Most patients presented with hemorrhages (53%) or seizures (23%). The final management consisted in embolization alone in 34%, embolization followed by surgery in 47%, embolization and radiotherapy in 16%, and embolization, surgery and radiotherapy in 3% of patients. The embolization procedure itself could lead to an angiographic cure in only 16% of patients. When the management strategy could be completed, the cure rate increased to 66%. Complications of embolization occurred in 22.6% of patients. Overall clinical outcome was excellent (Rankin 0) in 43%, good (Rankin 1) in 38%, fair (Rankin 2) in 10%, poor (Rankin 3-5) in 2%, and the death rate was 7%. A combined strategy initially designed to provide angiographic cures cannot be completed in a significant number of patients; the total morbidity of treatment remains significant. There is no scientific evidence that cerebral arteriovenous malformations should be treated, and no clinical trial to prove that one approach is better than the other. Various treatment protocols have been proposed on empirical grounds. Small lesions can often be eradicated, with surgery when lesions are superficial, or with radiation therapy for deeper ones. There has been little controversy regarding therapeutic indications in these patients (1). The management of larger AVMs, sometimes in more eloquent

  20. Galen and the beginnings of Western physiology.

    PubMed

    West, John B

    2014-07-15

    Galen (129-c. 216 AD) was a key figure in the early development of Western physiology. His teachings incorporated much of the ancient Greek traditions including the work of Hippocrates and Aristotle. Galen himself was a well-educated Greco-Roman physician and physiologist who at one time was a physician to the gladiators in Pergamon. Later he moved to Rome, where he was associated with the Roman emperors Marcus Aurelius and Lucius Verus. The Galenical school was responsible for voluminous writings, many of which are still extant. One emphasis was on the humors of the body, which were believed to be important in disease. Another was the cardiopulmonary system, including the belief that part of the blood from the right ventricle could enter the left through the interventricular septum. An extraordinary feature of these teachings is that they dominated thinking for some 1,300 years and became accepted as dogma by both the State and Church. One of the first anatomists to challenge the Galenical teachings was Andreas Vesalius, who produced a magnificent atlas of human anatomy in 1543. At about the same time Michael Servetus described the pulmonary transit of blood, but he was burned at the stake for heresy. Finally, with William Harvey and others in the first part of the 17th century, the beginnings of modern physiology emerged with an emphasis on hypotheses and experimental data. Nevertheless, vestiges of Galen's teaching survived into the 19th century. Copyright © 2014 the American Physiological Society.

  1. Microcatheter entrapment retrieval from Onyx embolization in brain arteriovenous malformations: A technical note.

    PubMed

    Vu, Phat D; Grigorian, Arthur A

    2015-10-01

    Many techniques have been use for retrieval of an entrapped microcatheter during Onyx (eV3 Neurovascular) embolization of brain arteriovenous malformations (BAVMs). We report our technique that we term "pull-push-pull" that can be utilized as first management in retrieving the microcatheter. We analyzed a total of 37 patients that underwent BAVM embolization with either Onyx 18 or 34 at our institution. Standard embolization techniques were utilized with the use of Marathon (eV3 Neurovascular) microcatheter. When difficulty in retrieving the microcatheter arose, we used the "pull-push-pull" technique. The technique comprises the eV3 protocol of retraction. In addition, the microcatheter is stretched causing the Onyx cast to stretch in its inner core, creating a more thorough cohesive property amongst the Onyx mixture. Then the microcatheter is pushed back and to its point of embolization origin. Afterwards, retraction of the microcatheter is enabled as it can be easily dislodged from the cast. Multiple attempts can be repeated as needed. We had three patients that had difficulty with removal of microcatheter (8.1%). Utilization of the "pull-push-pull" technique was used on two of those patients. No neurological complication was observed with our technique. We believe the cohesive property of Onyx solution helps in the retrieval of the catheter by our method and technique. We believe the "pull-push-pull" can be utilized and be an additional technique before attempting other catheter retrieval techniques in Onyx BAVM embolization. © The Author(s) 2015.

  2. Intracranial arteriovenous malformation: relationships between clinical and radiographic factors and ipsilateral steal severity

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Batjer, H.H.; Devous, M.D. Sr.; Seibert, G.B.

    1988-09-01

    Intracranial arteriovenous malformations (AVMs) are high flow shunts that may jeopardize the perfusion of adjacent tissue. Clinical and radiographic data from 62 patients were analyzed to determine their relationship to the severity of steal measured by single photon emission computed tomography (SPECT). The ipsilateral steal index (ISteal(i)) was determined by dividing regional cerebral blood flow (rCBF) values within hand-drawn regions of hypoperfusion in the ipsilateral hemisphere by total brain flow, which was calculated as the average rCBF of each hemisphere. Of the patients, 40% were less than 30 years of age, 45% were 30 to 50 years old, and 15%more » were over 50. Forty-eight per cent presented with hemorrhage and 34% presented with progressive deficits. There was angiographic steal in 37%, and postoperative hyperemic complications developed in 21%. All patients had ipsilateral regions of hypoperfusion. The ISteal(i) was less than 0.7 in 23 (37%), 0.7 to 0.8 in 20 (32%), and greater than 0.8 in 19 (31%). The ISteal(i) was significantly less severe in the patients over 50; 78% of these patients had an ISteal(i) of greater than 0.8 (P less than 0.01). A history of hemorrhage was associated with less severe steal than that in patients who had not bled (P = 0.088). Patients presenting with a history of progressive deficits had increased severity of steal compared with those without progressive deficits (P less than 0.05). A trend toward decreased severity of steal was noted in patients with unfavorable outcomes.« less

  3. Microcatheter entrapment retrieval from Onyx embolization in brain arteriovenous malformations: A technical note

    PubMed Central

    Grigorian, Arthur A

    2015-01-01

    Objective Many techniques have been use for retrieval of an entrapped microcatheter during Onyx (eV3 Neurovascular) embolization of brain arteriovenous malformations (BAVMs). We report our technique that we term “pull-push-pull” that can be utilized as first management in retrieving the microcatheter. Method We analyzed a total of 37 patients that underwent BAVM embolization with either Onyx 18 or 34 at our institution. Standard embolization techniques were utilized with the use of Marathon (eV3 Neurovascular) microcatheter. When difficulty in retrieving the microcatheter arose, we used the “pull-push-pull” technique. The technique comprises the eV3 protocol of retraction. In addition, the microcatheter is stretched causing the Onyx cast to stretch in its inner core, creating a more thorough cohesive property amongst the Onyx mixture. Then the microcatheter is pushed back and to its point of embolization origin. Afterwards, retraction of the microcatheter is enabled as it can be easily dislodged from the cast. Multiple attempts can be repeated as needed. Result and discussion We had three patients that had difficulty with removal of microcatheter (8.1%). Utilization of the “pull-push-pull” technique was used on two of those patients. No neurological complication was observed with our technique. We believe the cohesive property of Onyx solution helps in the retrieval of the catheter by our method and technique. Conclusion We believe the “pull-push-pull” can be utilized and be an additional technique before attempting other catheter retrieval techniques in Onyx BAVM embolization. PMID:26232252

  4. Iron deficiency, ischaemic strokes, and right-to-left shunts: From pulmonary arteriovenous malformations to patent foramen ovale?

    PubMed Central

    Shovlin, Claire L.

    2014-01-01

    Summary Has the recent identification of iron deficiency as a risk factor for ischaemic stroke in patients with pulmonary arteriovenous malformations (AVMs) unmasked a new paradigm for stroke/infarct pathogenesis? This commentary reviews evidence that spans associations between iron deficiency and ischaemic strokes, iron deficiency enhancement of platelet aggregation in response to serotonin/5HT, settings in which plasma 5HT is elevated, and clinical trial confirmation that 5HT receptor antagonists prevent ischaemic stroke. The critical leap which directs attention away from atherothrombotic events at the neurovascular wall is that ischaemic strokes due to pulmonary AVMs are attributable to compromised pulmonary capillary bed filtration of venous blood. Right-to-left shunting is continuous through pulmonary AVMs, but also occurs intermittently in approximately 30% of the general population with intracardiac shunts such as patent foramen ovale (PFO). The testable hypothesis presented is that paradoxical embolism of venous platelet-based aggregates may constitute part of the causal chain between iron deficiency and ischaemic stroke, not only in the rare disease state of pulmonary AVMs, but also in major subgroups of the general population. PMID:25343129

  5. Surgical management of dural arteriovenous fistulas with transosseous arterial feeders involving the jugular bulb.

    PubMed

    Tirakotai, W; Benes, L; Kappus, C; Sure, U; Farhoud, A; Bien, S; Bertalanffy, H

    2007-01-01

    Dural arteriovenous fistulas located in the vicinity of the jugular foramen are complex vascular malformations and belong to the most challenging skull base lesions to treat. The authors comprehensively analyze multiple features in a series of dural arteriovenous fistulas with transosseous arterial feeders involving the jugular bulb. Four patients who underwent surgery via the transcondylar approach to treat dural arteriovenous fistulas around the jugular foramen were retrospectively reviewed. Previously, endovascular treatment was attempted in all patients. The success of the surgical treatment was examined with postoperative angiography. Complete obliteration of the dural arteriovenous fistulas (DAVFs) was achieved in three patients, and significant flow reduction in one individual. All patients had a good postoperative outcome, and only one experienced mild hypoglossal nerve palsy. Despite extensive bone drilling, an occipitocervical fusion was necessary in only one patient with bilateral lesions. The use of an individually tailored transcondylar approach to treat dural arteriovenous fistulas at the region of the jugular foramen is most effective. This approach allows for complete obliteration of the connecting arterial feeders, and removal of bony structures containing pathological vessels.

  6. Arteriovenous malformations of the uterus.

    PubMed

    Cura, M; Martinez, N; Cura, A; Dalsaso, T J; Elmerhi, F

    2009-09-01

    Arterial venous malformations (AVM) of the uterus are uncommon entities and should be considered in patients who present with profuse genital bleeding. There are two types of uterine AVM: acquired and congenital. Acquired uterine AVMs are conformed by communications between the uterine arteries and the myometrial veins, and are caused by an iatrogenic event or a pathological condition. Congenital AVMs are the result of abnormal development of primitive vessels that result in connections between pelvic arteries and veins in the uterus without an interconnecting capillary bed. Ultrasonography is a noninvasive diagnostic method able to demonstrate and characterize AVMs of the uterus. AVM in the pelvis may be noted incidentally by computed tomography (CT) of the pelvis, and magnetic resonance imaging (MRI) is frequently used to confirm and further characterize the sonographic findings of uterine AVM. Catheter angiography and embolization are very effective in defining the vascular anatomy and treating uterine vascular abnormalities.

  7. Treatment of Brain Arteriovenous Malformations with Hemodynamic Aneurysms: A Series of 131 Consecutive Cases.

    PubMed

    Eliava, Shalva; Dmitriev, Alexey; Shekhtman, Oleg; Yakovlev, Sergey; Kheireddin, Ali; Pilipenko, Yuriy

    2018-02-01

    The natural history of hemodynamic aneurysms (HAs) associated with brain arteriovenous malformations (AVMs) remains controversial, with no single approach to treatment. The purpose of this study was to justify preventive treatment tactics for HAs that pose an increased risk of rupture based on hemodynamic studies demonstrating hypertension in the afferent bed after AVM exclusion. This retrospective analysis included 131 of 1740 patients (8%) with brain AVMs and at least 1 aneurysm treated at Burdenko Neurosurgical Institute between 2000 and 2016. Treatment consisted of microsurgery, endovascular interventions, or a combination of modalities. Patients were evaluated with the modified Rankin Scale before and after treatment. A total of 205 aneurysms were discovered. Multiple HAs were found in 46 patients (35%), and were significantly more often associated with posterior fossa AVMs; in addition, most were distally located. There was no difference in the incidence of hemorrhage between proximal and distal HAs. Microsurgical treatment was marked by high radicalism; 85% of HAs and 94% of AVMs were totally excluded based on control studies. In 10 cases, aneurysms were found after AVM removal, including 4 de novo aneurysms. In 1 case, the aneurysm regressed after AVM treatment. The mortality rate was 2.3%. Preoperative imaging should be carefully examined for associated aneurysms before and after surgical treatment. Our data suggests that HA exclusion, either as the first step or simultaneously with AVM treatment, is most beneficial to patients. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Procedure for the Isolation of Endothelial Cells from Human Cerebral Arteriovenous Malformation (cAVM) Tissues.

    PubMed

    Hao, Qiang; Chen, Xiao-Lin; Ma, Li; Wang, Tong-Tong; Hu, Yue; Zhao, Yuan-Li

    2018-01-01

    In this study, we successfully established a stable method for the isolation of endothelial cells (ECs) from human cerebral arteriovenous malformation (cAVM) tissues. Despite human cAVM tissues having a minor population of ECs, they play an important role in the manifestation and development of cAVM as well as in hemorrhagic stroke and thrombogenesis. To characterize and understand the biology of ECs in human cAVM (cAVM-ECs), methods for the isolation and purification of these cells are necessary. We have developed this method to reliably obtain pure populations of ECs from cAVMs. To obtain pure cell populations, cAVM tissues were mechanically and enzymatically digested and the resulting single cAVM-ECs suspensions were then labeled with antibodies of specific cell antigens and selected by flow cytometry. Purified ECs were detected using specific makers of ECs by immunostaining and used to study different cellular mechanisms. Compared to the different methods of isolating ECs from tissues, we could isolate ECs from cAVMs confidently, and the numbers of cAVM-ECs harvested were almost similar to the amounts present in vessel components. In addition to optimizing the protocol for isolation of ECs from human cAVM tissues, the protocol could also be applied to isolate ECs from other human neurovascular-diseased tissues. Depending on the tissues, the whole procedure could be completed in about 20 days.

  9. An approach to the symbolic representation of brain arteriovenous malformations for management and treatment planning.

    PubMed

    Orlowski, Piotr; Mahmud, Imran; Kamran, Mudassar; Summers, Paul; Noble, Alison; Ventikos, Yiannis; Byrne, James V

    2014-03-01

    There is currently no standardised approach to arteriovenous malformation (AVM) reporting. Existing AVM classification systems focuses on angioarchitectural features and omit haemodynamic, anatomical and topological parameters intuitively used by therapists. We introduce a symbolic vocabulary to represent the state of an AVM of the brain at different stages of treatment. The vocabulary encompasses the main anatomic and haemodynamic features of interest in treatment planning and provides shorthand symbols to represent the interventions themselves in a schematic representation. The method was presented to 50 neuroradiologists from 14 countries during a workshop and graded 7.34 ± 1.92 out of ten for its usefulness as means of standardising and facilitating communication between clinicians and allowing comparisons between AVM cases. Feedback from the survey was used to revise the method and improve its completeness. For an AVM test case, participants were asked to produce a conventional written report and subsequently a diagrammatic report. The two required, on average, 6.19 ± 2.05 and 5.09 ± 3.01 min, respectively. Eighteen participants said that producing the diagram changed the way they thought about the AVM test case. Introduced into routine practice, the diagrams would represent a step towards a standardised approach to AVM reporting with consequent benefits for comparative analysis and communication as well as for identifying best treatment strategies.

  10. Intraosseous embolotherapy of central arteriovenous malformations in the jaw: long-term experience with 8 cases.

    PubMed

    Liu, Denggao; Ma, Xuchen; Zhao, Fuyun; Zhang, Jianguo

    2009-11-01

    To investigate the long-term effects of direct intraosseous histoacryl embolotherapy on central arteriovenous malformations (AVMs) of the jaw. Eight patients with central AVMs of the jaw (3 in the maxilla and 5 in the mandible) were treated with direct intraosseous histoacryl injection. These AVMs exhibited cystic radiolucency, with (n = 5) or without (n = 3) honeycombed component. On angiography, all the AVMs exhibited a large intraosseous nidus with multiple suppliers and drainages. The intraosseous lesions were percutaneously punctured with an 18- to 20-gauge needle, and NBCA diluted 30% to 40% with iodized oil was injected during venous compression. Postembolic arteriograms showed that all the AVMs were completely or nearly completely devascularized after single or multiple injections at the initial treatment. The follow-up period ranged from 3 to 8 years. Two AVMs experienced postembolic curettage and were anatomically cured. Three AVMs were anatomically cured after 1 to 3 sessions of embolotherapy. The other 3 AVMs were clinically cured after 1 (n = 2) to 4 (n = 1) sessions of embolotherapy. There were no procedure-related severe complications. Direct intraosseous glue embolotherapy can be a simple and safe technique for endovascular management of central AVMs in the jaw and is particularly effective in an emergency. Complete reossification of the intraosseous nidus can be anticipated if a complete occlusion is obtained.

  11. Stereotactic radiosurgery for arteriovenous malformations after Onyx embolization: a case-control study.

    PubMed

    Lee, Cheng-Chia; Chen, Ching-Jen; Ball, Benjamin; Schlesinger, David; Xu, Zhiyuan; Yen, Chun-Po; Sheehan, Jason

    2015-07-01

    Onyx, an ethylene-vinyl alcohol copolymer mixed in a dimethyl sulfoxide solvent, is currently one of the most widely used liquid materials for embolization of intracranial arteriovenous malformations (AVMs). The goal of this study was to define the risks and benefits of stereotactic radiosurgery (SRS) for patients who have previously undergone partial AVM embolization with Onyx. Among a consecutive series of 199 patients who underwent SRS between January 2007 and December 2012 at the University of Virginia, 25 patients had Onyx embolization prior to SRS (the embolization group). To analyze the obliteration rates and complications, 50 patients who underwent SRS without prior embolization (the no-embolization group) were matched by propensity score method. The matched variables included age, sex, nidus volume before SRS, margin dose, Spetzler-Martin grade, Virginia Radiosurgery AVM Scale score, and median imaging follow-up period. After Onyx embolization, 18 AVMs were reduced in size. Total obliteration was achieved in 6 cases (24%) at a median of 27.5 months after SRS. In the no-embolization group, total obliteration was achieved in 20 patients (40%) at a median of 22.4 months after SRS. Kaplan-Meier analysis demonstrated obliteration rates of 17.7% and 34.1% in the embolization group at 2 and 4 years, respectively. In the no-embolization group, the corresponding obliteration rates were 27.0% and 55.9%. The between-groups difference in obliteration rates after SRS did not achieve statistical significance. The difference in complications, including adverse radiation effects, hemorrhage episodes, seizure control, and patient mortality also did not reach statistical significance. Onyx embolization can effectively reduce the size of many AVMs. This case-control study did not show any statistically significant difference in the rates of embolization or complications after SRS in patients who had previously undergone Onyx embolization and those who had not.

  12. Embolization techniques for high-flow arteriovenous malformations with a dominant outflow vein.

    PubMed

    Conway, Allan M; Qato, Khalil; Drury, Jennifer; Rosen, Robert J

    2015-04-01

    The aim of this study was to assess the management and outcomes of arteriovenous malformations (AVMs) with a dominant outflow vein (DOV) treated with retrograde venous embolization. A retrospective review was performed from November 2010 to May 2014 on all patients with a high-flow AVM and associated DOV who underwent transvenous embolization of the DOV. Indications, techniques, complications, and outcomes were reviewed. Fourteen patients (five male; 36%) underwent transvenous embolization of high-flow AVMs with a DOV. Median age was 41.6 years (15.7-65.8 years). The AVM was located on an extremity in eight patients (57%) and in the pelvis in six patients (43%). The indication for the procedure was pain in 11 patients (79%), swelling in 3 patients (21%), a nonhealing wound in 1 patient (7%), and impotence in 1 patient (7%). The median number of prior procedures to treat the AVM was 2.5 (0-13). Transvenous embolization with coils was performed in 13 patients (93%). The Amplatzer vascular plug and Amplatzer septal occluder (St. Jude Medical, St. Paul, Minn) were used in four patients (29%). Concurrent percutaneous puncture embolization of the AVM nidus was used in seven patients (50%) and transcatheter arterial embolization in eight patients (57%). Technical angiographic success was seen in all patients. Five patients (36%) experienced a complete response to treatment, whereas eight (57%) experienced a partial response. Seven patients (50%) required further procedures for residual symptoms. AVMs with a DOV can be successfully treated by a transvenous approach. Percutaneous puncture embolization of the nidus or draining vein and transcatheter arterial embolization may assist in reducing flow. Copyright © 2015 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

  13. Dialectic and science: Galen, Herophilus and Aristotle on phenomena.

    PubMed

    Tieleman, T

    1995-01-01

    This paper examines the nature of Galen's argument in the De placitis Hippocratis et Platonis, books 2-3, concerned with the location of the psychic functions within the body. To this question Galen applies a coherent set of methodological principles, integrating Aristotelian dialectic and scientific demonstration based on anatomical experiments. Galen disagrees with Aristotle in that he relegates the endoxa from the realm of dialectic to that of rhetoric. His attitude is marked by a distinctive emphasis on perceptible phenomena as the starting point for scientific inquiry. This and other features can be traced back to the Hellenistic scientist Herophilus.

  14. Soluble FLT1 Gene Therapy Alleviates Brain Arteriovenous Malformation Severity

    PubMed Central

    Zhu, Wan; Shen, Fanxia; Mao, Lei; Zhan, Lei; Kang, Shuai; Sun, Zhengda; Nelson, Jeffrey; Zhang, Rui; Zou, Dingquan; McDougall, Cameron M.; Lawton, Michael T.; Vu, Thiennu H.; Wu, Zhijian; Scaria, Abraham; Colosi, Peter; Forsayeth, John; Su, Hua

    2017-01-01

    Background and Purpose Brain arteriovenous malformation (bAVM) is an important risk factor for intracranial hemorrhage. Current therapies are associated with high morbidities. Excessive vascular endothelial growth factor (VEGF) has been implicated in bAVM pathophysiology. Because soluble FLT1 binds to VEGF with high affinity, we tested intravenous (IV) delivery of an adeno-associated viral vector serotype 9 expressing soluble FLT1 (AAV9-sFLT1) to alleviate the bAVM phenotype. Methods Two mouse models were used. Model 1: bAVM was induced in R26CreER;Eng2f/2f mice through global Eng gene deletion and brain focal angiogenic stimulation; AAV2-sFLT02 (an AAV expressing a shorter form of sFLT1) was injected into the brain at the time of model induction, and AAV9-sFLT1, IV-injected eight weeks after. Model 2: SM22αCre;Eng2f/2f mice had a 90% occurrence of spontaneous bAVM at 5 weeks of age and 50% mortality at 6 weeks; AAV9-sFLT1 was IV-delivered into 4–5-week-old mice. Tissue samples were collected four weeks after AAV9-sFLT1 delivery. Results AAV2-sFLT02 inhibited bAVM formation and AAV9-sFLT1 reduced abnormal vessels in Model 1 (GFP vs sFLT1: 3.66 ± 1.58/200 vessels vs 1.98 ± 1.29, p<0.05). AAV9-sFLT1 reduced the occurrence of bAVM (GFP vs sFLT1: 100% vs 36%) and mortality [GFP vs sFLT1: 57% (12/22 mice) vs 24% (4/19 mice), p<0.05] in Model 2. Kidney and liver function did not change significantly. Minor liver inflammation was found in 56% of AAV9-sFLT1-treated Model 1 mice. Conclusion By applying a regulated mechanism to restrict sFLT1 expression to bAVM, AAV9-sFLT1 can potentially be developed into a safer therapy to reduce the bAVM severity. PMID:28325846

  15. Unusual Systemic Artery to Pulmonary Artery Malformation Without Evidence of Systemic Disease, Trauma or Surgery

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Geyik, Serdar; Yavuz, Kivilcim; Keller, Frederick S.

    2006-10-15

    Connections between the systemic and pulmonary arterial systems are rare conditions that can be due to either congenital or acquired diseases such as anomalous systemic arterial supply to normal lung, pulmonary sequestration, and systemic supply to pulmonary arteriovenous malformations. Herein, a unique case of systemic artery to pulmonary arterial malformation and its endovascular treatment in a patient with no history of the usual etiologies is reported.

  16. Outcome after conservative management or intervention for unruptured brain arteriovenous malformations.

    PubMed

    Al-Shahi Salman, Rustam; White, Philip M; Counsell, Carl E; du Plessis, Johann; van Beijnum, Janneke; Josephson, Colin B; Wilkinson, Tim; Wedderburn, Catherine J; Chandy, Zoe; St George, E Jerome; Sellar, Robin J; Warlow, Charles P

    Whether conservative management is superior to interventional treatment for unruptured brain arteriovenous malformations (bAVMs) is uncertain because of the shortage of long-term comparative data. To compare the long-term outcomes of conservative management vs intervention for unruptured bAVM. Population-based inception cohort study of 204 residents of Scotland aged 16 years or older who were first diagnosed as having an unruptured bAVM during 1999-2003 or 2006-2010 and followed up prospectively for 12 years. Conservative management (no intervention) vs intervention (any endovascular embolization, neurosurgical excision, or stereotactic radiosurgery alone or in combination). Cox regression analyses, with multivariable adjustment for prognostic factors and baseline imbalances if hazards were proportional, to compare rates of the primary outcome (death or sustained morbidity of any cause by Oxford Handicap Scale [OHS] score ≥2 for ≥2 successive years [0 = no symptoms and 6 = death]) and the secondary outcome (nonfatal symptomatic stroke or death due to bAVM, associated arterial aneurysm, or intervention). Of 204 patients, 103 underwent intervention. Those who underwent intervention were younger, more likely to have presented with seizure, and less likely to have large bAVMs than patients managed conservatively. During a median follow-up of 6.9 years (94% completeness), the rate of progression to the primary outcome was lower with conservative management during the first 4 years of follow-up (36 vs 39 events; 9.5 vs 9.8 per 100 person-years; adjusted hazard ratio, 0.59; 95% CI, 0.35-0.99), but rates were similar thereafter. The rate of the secondary outcome was lower with conservative management during 12 years of follow-up (14 vs 38 events; 1.6 vs 3.3 per 100 person-years; adjusted hazard ratio, 0.37; 95% CI, 0.19-0.72). Among patients aged 16 years or older diagnosed as having unruptured bAVM, use of conservative management compared with intervention was

  17. Transarterial Embolization of a Spontaneous Intraorbital Arteriovenous Fistula With n-BCA Glue.

    PubMed

    Konstas, Angelos A; Rootman, Daniel B; Quiros, Peter A; Ross, Ian B

    Arteriovenous fistulae of the orbit are exceedingly rare. They are high-flow vascular malformations involving a fistula from the ophthalmic artery to one of the draining ophthalmic veins. Presenting symptoms can mimic those of carotid cavernous fistulae or of ophthalmic venous varices, and include diplopia, proptosis, chemosis, decreased visual acuity, and retro-orbital pain. Very few case reports are published on the treatment of this aggressive vascular malformation, and they uniformly describe techniques involving a transvenous access for the embolization of the fistula. To the best of authors' knowledge, they report the first case of transarterial embolization of an intraorbital AVF.

  18. A case report of spinal dural arteriovenous fistula: origins, determinants, and consequences of abnormal vascular malformations.

    PubMed

    Zakhary, Sherry M; Hoehmann, Christopher L; Cuoco, Joshua A; Hitscherich, Kyle; Alam, Hamid; Torres, German

    2017-06-01

    A spinal dural arteriovenous fistula is an abnormally layered connection between radicular arteries and venous plexus of the spinal cord. This vascular condition is relatively rare with an incidence of 5-10 cases per million in the general population. Diagnosis of spinal dural arteriovenous fistula is differentiated by contrast-enhanced magnetic resonance angiography or structural magnetic resonance imaging, but a definitive diagnosis requires spinal angiography methods. Here, we report a case of a 67-year-old female with a spinal dural arteriovenous fistula, provide a pertinent clinical history to the case nosology, and discuss the biology of adhesive proteins, chemotactic molecules, and transcription factors that modify the behavior of the vasculature to possibly cause sensorimotor deficits.

  19. Time-resolved magnetic resonance angiography (MRA) at 3.0 Tesla for evaluation of hemodynamic characteristics of vascular malformations: description of distinct subgroups.

    PubMed

    Hammer, Simone; Uller, Wibke; Manger, Florentine; Fellner, Claudia; Zeman, Florian; Wohlgemuth, Walter A

    2017-01-01

    Quantitative evaluation of hemodynamic characteristics of arteriovenous and venous malformations using time-resolved magnetic resonance angiography (MRA) at 3.0 Tesla. Time-resolved MRA with interleaved stochastic trajectories (TWIST) at 3.0 Tesla was studied in 83 consecutive patients with venous malformations (VM) and arteriovenous malformations (AVM). Enhancement characteristics were calculated as percentage increase of signal intensity above baseline over time. Maximum percentage signal intensity increase (signal max ), time intervals between onset of arterial enhancement and lesion enhancement (t onset ), and time intervals between beginning of lesion enhancement and maximum percentage of lesion enhancement (t max ) were analyzed. All AVMs showed a high-flow hemodynamic pattern. Two significantly different (p < 0.001) types of venous malformations emerged: VMs with arteriovenous fistulas (AVF) (median signal max 737 %, IQR [interquartile range] = 511 - 1182 %; median t onset 5 s, IQR = 5 - 10 s; median t max 35 s, IQR = 26 - 40 s) and without AVFs (median signal max 284 %, IQR = 177-432 %; median t onset 23 s, IQR = 15 - 30 s; median t max 60 s, IQR = 55 - 75 s). Quantitative evaluation of time-resolved MRA at 3.0 Tesla provides hemodynamic characterization of vascular malformations. VMs can be subclassified into two hemodynamic subgroups due to presence or absence of AVFs. • Time-resolved MRA at 3.0 Tesla provides quantitative hemodynamic characterization of vascular malformations. • Malformations significantly differ in time courses of enhancement and signal intensity increase. • AVMs show a distinctive high-flow hemodynamic pattern. • Two significantly different types of VMs emerged: VMs with and without AVFs.

  20. Extravasation of contrast (Spot Sign) predicts in-hospital mortality in ruptured arteriovenous malformation.

    PubMed

    Ye, Zengpanpan; Ai, Xiaolin; Zheng, Jun; Hu, Xin; You, Chao; Andrew M, Faramand; Fang, Fang

    2017-10-09

    The spot sign is a highly specific and sensitive predictor of hematoma expansion in following primary intracerebral hemorrhage (ICH). Rare cases of the spot sign have been documented in patients with intracranial hemorrhage secondary to arteriovenous malformation (AVM). The purpose of this retrospective study is to assess the accuracy of spot sign in predicting clinical outcomes in patients with ruptured AVM. A retrospective analysis of a prospectively maintained database was performed for patients who presented to West China Hospital with ICH secondary to AVM in the period between January 2009 and September 2016. Two radiologists blinded to the clinical data independently assessed the imaging data, including the presence of spot sign. Statistical analysis using univariate testing, multivariate logistic regression testing, and receiver operating characteristic curve (AUC) analysis was performed. A total of 116 patients were included. Overall, 18.9% (22/116) of subjects had at least 1 spot sign detected by CT angiography, 7% (8/116) died in hospital, and 27% (31/116) of the patients had a poor outcome after 90 days. The spot sign had a sensitivity of 62.5% and specificity of 84.3% for predicting in-hospital mortality (p = .02, AUC 0.734). No correlation detected between the spot sign and 90-day outcomes under multiple logistic regression (p = .19). The spot sign is an independent predictor for in-hospital mortality. The presence of spot sign did not correlate with the 90 day outcomes in this patient cohort. The results of this report suggest that patients with ruptured AVM with demonstrated the spot sign on imaging must receive aggressive treatment early on due to the high risk of mortality.

  1. Magnetic Resonance Angiography in the Diagnosis of Cerebral Arteriovenous Malformation and Dural Arteriovenous Fistulas: Comparison of Time-Resolved Magnetic Resonance Angiography and Three Dimensional Time-of-Flight Magnetic Resonance Angiography

    PubMed Central

    Cheng, Yu-Ching; Chen, Hung-Chieh; Wu, Chen-Hao; Wu, Yi-Ying; Sun, Ming-His; Chen, Wen-Hsien; Chai, Jyh-Wen; Chi-Chang Chen, Clayton

    2016-01-01

    Background Traditional digital subtraction angiography (DSA) is currently the gold standard diagnostic method for the diagnosis and evaluation of cerebral arteriovenous malformation (AVM) and dural arteriovenous fistulas (dAVF). Objectives The aim of this study was to analyze different less invasive magnetic resonance angiography (MRA) images, time-resolved MRA (TR-MRA) and three-dimensional time-of-flight MRA (3D TOF MRA) to identify their diagnostic accuracy and to determine which approach is most similar to DSA. Patients and Methods A total of 41 patients with AVM and dAVF at their initial evaluation or follow-up after treatment were recruited in this study. We applied time-resolved angiography using keyhole (4D-TRAK) MRA to perform TR-MRA and 3D TOF MRA examinations simultaneously followed by DSA, which was considered as a standard reference. Two experienced neuroradiologists reviewed the images to compare the diagnostic accuracy, arterial feeder and venous drainage between these two MRA images. Inter-observer agreement for different MRA images was assessed by Kappa coefficient and the differences of diagnostic accuracy between MRA images were evaluated by the Wilcoxon rank sum test. Results Almost all vascular lesions (92.68%) were correctly diagnosed using 4D-TRAK MRA. However, 3D TOF MRA only diagnosed 26 patients (63.41%) accurately. There were statistically significant differences regarding lesion diagnostic accuracy (P = 0.008) and venous drainage identification (P < 0.0001) between 4D-TRAK MRA and 3D TOF MRA. The results indicate that 4D-TRAK MRA is superior to 3D TOF MRA in the assessment of lesions. Conclusion Compared with 3D TOF MRA, 4D-TRAK MRA proved to be a more reliable screening modality and follow-up method for the diagnosis of cerebral AVM and dAVF. PMID:27679690

  2. Color-coded perfusion analysis of CEUS for pre-interventional diagnosis of microvascularisation in cases of vascular malformations.

    PubMed

    Teusch, V I; Wohlgemuth, W A; Piehler, A P; Jung, E M

    2014-01-01

    Aim of our pilot study was the application of a contrast-enhanced color-coded ultrasound perfusion analysis in patients with vascular malformations to quantify microcirculatory alterations. 28 patients (16 female, 12 male, mean age 24.9 years) with high flow (n = 6) or slow-flow (n = 22) malformations were analyzed before intervention. An experienced examiner performed a color-coded Doppler sonography (CCDS) and a Power Doppler as well as a contrast-enhanced ultrasound after intravenous bolus injection of 1 - 2.4 ml of a second-generation ultrasound contrast medium (SonoVue®, Bracco, Milan). The contrast-enhanced examination was documented as a cine sequence over 60 s. The quantitative analysis based on color-coded contrast-enhanced ultrasound (CEUS) images included percentage peak enhancement (%peak), time to peak (TTP), area under the curve (AUC), and mean transit time (MTT). No side effects occurred after intravenous contrast injection. The mean %peak in arteriovenous malformations was almost twice as high as in slow-flow-malformations. The area under the curve was 4 times higher in arteriovenous malformations compared to the mean value of other malformations. The mean transit time was 1.4 times higher in high-flow-malformations compared to slow-flow-malformations. There was no difference regarding the time to peak between the different malformation types. The comparison between all vascular malformation and surrounding tissue showed statistically significant differences for all analyzed data (%peak, TTP, AUC, MTT; p < 0.01). High-flow and slow-flow vascular malformations had statistically significant differences in %peak (p < 0.01), AUC analysis (p < 0.01), and MTT (p < 0.05). Color-coded perfusion analysis of CEUS seems to be a promising technique for the dynamic assessment of microvasculature in vascular malformations.

  3. Galen's Contribution to Head and Neck Surgery.

    PubMed

    Stathopoulos, Panagiotis

    2017-06-01

    The historical relevance of Galen has always been very high and his works have exerted a profound influence on medical thought for more than 1,300 years. This could be the main reason why his surgical expertise is often neglected. This brief study focuses on the contribution of this distinguished physician to head and neck surgery and it shows that Galen's surgical experience is well documented in his work. Copyright © 2017 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  4. Awake craniotomy for excision of arteriovenous malformations? A qualitative comparison study with stereotactic radiosurgery.

    PubMed

    Chan, David Yuen Chung; Chan, Danny Tat Ming; Zhu, Cannon Xian Lun; Kan, Patricia Kwok Yee; Ng, Amelia Yikjin; Hsieh, Yi-Pin Sonia; Abrigo, Jill; Poon, Wai Sang; Wong, George Kwok Chu

    2018-05-01

    Treatment of arteriovenous malformations (AVM) located at the eloquent area has been a challenge. Awake brain mapping allows identification of a non-eloquent gyrus for intervention and can potentially facilitate resection with preservation of functions. An alternative treatment option is stereotactic radiosurgery (SRS). The objective of this study was to perform a qualitative comparison of the treatment outcome of awake AVM excision versus SRS. We conducted a 13-year retrospective review of AVM excision under awake craniotomy performed at Prince of Wales Hospital, Hong Kong, from 2003 to 2016. Patients' presentation, Spetzler-Martin (SM) grading, rate of obliteration and complication were reviewed and analyzed with the modified radiosurgery-based AVM score (RS score). Six patients had excision of AVM under awake mapping during this period of time. Two were SM Grade II and four were SM Grade III. Five located at the peri-rolandic region while one at the temporal language area. None had failed mapping. Five out of six achieved complete obliteration (83.3%). Qualitative comparative analysis had revealed better treatment outcome with awake AVM excision as compared to SRS with the obliteration rate of 100% versus 96% for RS score ≤1.00, 100% versus 78% for RS score 1.01-1.50, and 66% versus 50% for RS score >2.00 respectively. In conclusion, awake mapping and excision of AVMs at the eloquent area is feasible. Qualitative comparative analysis had revealed higher obliteration rate with awake AVM excision as compared to SRS. Copyright © 2018 Elsevier Ltd. All rights reserved.

  5. Surgical planning for microsurgical excision of cerebral arterio-venous malformations using virtual reality technology.

    PubMed

    Ng, Ivan; Hwang, Peter Y K; Kumar, Dinesh; Lee, Cheng Kiang; Kockro, Ralf A; Sitoh, Y Y

    2009-05-01

    To evaluate the feasibility of surgical planning using a virtual reality platform workstation in the treatment of cerebral arterio-venous malformations (AVMs) Patient-specific data of multiple imaging modalities were co-registered, fused and displayed as a 3D stereoscopic object on the Dextroscope, a virtual reality surgical planning platform. This system allows for manipulation of 3D data and for the user to evaluate and appreciate the angio-architecture of the nidus with regards to position and spatial relationships of critical feeders and draining veins. We evaluated the ability of the Dextroscope to influence surgical planning by providing a better understanding of the angio-architecture as well as its impact on the surgeon's pre- and intra-operative confidence and ability to tackle these lesions. Twenty four patients were studied. The mean age was 29.65 years. Following pre-surgical planning on the Dextroscope, 23 patients underwent microsurgical resection after pre-surgical virtual reality planning, during which all had documented complete resection of the AVM. Planning on the virtual reality platform allowed for identification of critical feeders and draining vessels in all patients. The appreciation of the complex patient specific angio-architecture to establish a surgical plan was found to be invaluable in the conduct of the procedure and was found to enhance the surgeon's confidence significantly. Surgical planning of resection of an AVM with a virtual reality system allowed detailed and comprehensive analysis of 3D multi-modality imaging data and, in our experience, proved very helpful in establishing a good surgical strategy, enhancing intra-operative spatial orientation and increasing surgeon's confidence.

  6. Evaluation of Angioarchitectural Features of Unruptured Brain Arteriovenous Malformation by Susceptibility Weighted Image (SWI).

    PubMed

    Wu, Chun-Xue; Ma, Li; Chen, Xu-Zhu; Chen, Xiao-Lin; Chen, Yu; Zhao, Yuan-Li; Hess, Christopher; Kim, Helen; Jin, Heng-Wei; Ma, Jun

    2018-05-30

    A precise assessment of angioarchitectural characteristics using non-invasive imaging is helpful for serial follow-up and weighting risk of natural history in uruptured brain arteriovenous malformation (bAVM). This study aimed to test the hypothesis that susceptibility weighted image (SWI) would provide an accurate evaluation of angioarchitectural features of unruptured bAVM.. A total of 81 consecutive patients with unruptured bAVM were examined. Image quality of SWI for the assessment of bAVM angioarchitectural features were determined by a five-point scale. The accuracy of SWI for detection of angioarchitectural features was evaluated using DSA as a standard reference. And further compared among unruptured bAVMs with or without silent intralesional microhemorrhage on SWI to examine the potential confounding effect of microhemorrhage on image analysis. All lesions were identified on SWI. Image quality of SWI was judged to be at least adequate for diagnosis (range, 3-5) in all patients by both readers. Using DSA as reference standard, the area under receiver operating curve (AUC) of detection of deep or posterior fossa location, exclusively deep venous drainage, venous ectasia, venous varices and the presence of associated aneurysm on SWI was 1, 0.93, 0.94, 0.95, and 0.83, respectively. Silent intralesional microhemorrhage were detected in 39 patients (48.15%) on SWI and no significant difference (P > 0.05) was found in angioarchitectural features between cases with and without silent microhemorrhage. SWI might be a non-invasive alternative technique for angiogram in the angioarchitectural assessment of unruptured bAVM. Copyright © 2018. Published by Elsevier Inc.

  7. Introduction to tractography-guided navigation: using 3-tesla magnetic resonance tractography in surgery for cerebral arteriovenous malformations.

    PubMed

    Kikuta, K; Takagi, Y; Nozaki, K; Hashimoto, N

    2008-01-01

    To examine the effectiveness of magnetic resonance (MR) tractography in surgery for cerebral arteriovenous malformations (AVMs). A preoperative evaluation of major neural tracts around the nidus was carried out with 3-tesla (3 T) MR tractography in 25 consecutive patients with cerebral AVMs. The patients were 12 men and 13 women ranging in age from 4 to 60 years of age (mean age: 31.2 +/- 14.1 years). Twelve presented with hemorrhage. Images were obtained with T2-weighted turbo spin echo sequences, axial T1-weighted three-dimensional magnetization-prepared rapid acquisition gradient-echo (MPRAGE) sequences, three-dimensional time-of-flight MR angiography (3D TOF MRA), and thin-section diffusion-tensor imaging (DTI). The AVMs were obliterated in 22 of the 25 patients. A postoperative study of the MR tractography was carried out in 24 patients. In 21 patients, tracts were preserved and no postoperative neurological worsening was observed. Disruption of the tracts was found in 3 patients, and postoperative worsening was observed in 2 patients. However, no deterioration occurred in 1 patient with cerebellar AVM. Notwithstanding the limitations of this method, MR tractography can be considered useful for confirming the integrity of deviated tracts, for localizing deviated tracts, and for evaluating surgical risk, especially in cases of non-hemorrhagic AVM.

  8. Early Rebleeding after Brain Arteriovenous Malformation Rupture, Clinical Impact and Predictive Factors: A Monocentric Retrospective Cohort Study.

    PubMed

    Shotar, Eimad; Pistocchi, Silvia; Haffaf, Idriss; Bartolini, Bruno; Jacquens, Alice; Nouet, Aurélien; Chiras, Jacques; Degos, Vincent; Sourour, Nader-Antoine; Clarençon, Frédéric

    2017-01-01

    Brain arteriovenous malformations (BAVMs) are a leading cause of intracranial hemorrhage in young adults. This study aimed to identify individual predictive factors of early rebleeding after BAVM rupture and determine its impact on prognosis. Early rebleeding was defined as a spontaneous intracranial hemorrhage within 30 days of BAVM rupture in patients with nonobliterated BAVMs. One hundred fifty one patients with 158 BAVM hemorrhagic events admitted to a tertiary care center during 14 years were included. Univariate followed by multivariate logistic regression was performed to assess the impact of early rebleeding on in-hospital mortality and modified Rankin Scale (mRS) score beyond 3 months and to identify independent predictors of early rebleeding. Eight early rebleeding events were observed, 6 of which occurred during the first 7 days. Early rebleeding was independently and significantly associated with poor outcome (mRS ≥3 beyond 3 months, p = 0.004) but not with in-hospital mortality (p = 0.9). Distal flow-related aneurysms (p = 0.009) and altered consciousness with a Glasgow coma scale score of 3 (p = 0.01) were independently associated with early rebleeding. Early rebleeding is a severe complication that can occur after BAVM-related hemorrhage. Distal flow-related aneurysms and initial altered consciousness are associated with early rebleeding. © 2017 S. Karger AG, Basel.

  9. Clinical presentation and treatment paradigms of brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia.

    PubMed

    Gamboa, Nicholas T; Joyce, Evan J; Eli, Ilyas; Park, Min S; Taussky, Philipp; Schmidt, Richard H; McDonald, Jamie; Whitehead, Kevin J; Kalani, M Yashar S

    2018-05-01

    Hereditary hemorrhagic telangiectasia (HHT) is characterized by recurrent spontaneous epistaxis, mucocutaneous telangiectases, and multisystem arteriovenous malformations (AVMs). Brain AVMs typically present at birth and are identified in approximately 10-20% of patients with HHT. A retrospective review was undertaken of all HHT patients with known single or multiple brain AVMs treated at our institution. Thirty-nine patients with brain AVM(s) were diagnosed with HHT. Most patients presented with at least one Curaçao criterion. A total of 78 brain AVMs were identified in 39 patients. Two-thirds of patients had solitary brain AVMs, whereas 33% of patients harbored at least two lesions (range: 2-16). Brain AVMs of the supratentorial cerebral hemispheres comprised 83% of all lesions, whereas infratentorial lesions accounted for only 17%. Of the 55 brain AVMs assigned Spetzler-Martin grading, the majority of patients were Grade 1 (73%), and 23% and 4% were Grades 2 and 3, respectively. Patients were treated with surgery alone (51%), embolization alone (6%), embolization followed by surgery (9%), stereotactic radiosurgery (11%), stereotactic radiosurgery followed by surgery (3%), or observation (20%). Of patients who underwent genetic analysis, 62% possessed mutations in ENG (HHT type 1), whereas 38% had mutations in ACVRL1 (HHT type 2). This robust patient cohort of brain AVMs in 39 patients with HHT advances the collective understanding of this disease's varied presentation, diagnostic workup, genetic underpinnings, and available treatment options. Copyright © 2018. Published by Elsevier Ltd.

  10. Replication Study of Polymorphisms Associated With Brain Arteriovenous Malformation in a Population From South of Brazil

    PubMed Central

    Ludwig, Fernanda S; Matte, Ursula S; Mota, Simone; Stefani, Marco A

    2016-01-01

    Introduction The aim of this study was to reproducibly determine if any of the polymorphisms were associated with the susceptibility to brain arteriovenous malformations (BAVM) or the risk of intracranial hemorrhage (ICH) presentation. Methods We recruited 63 BAVM patients and 96 controls. The polymorphisms selected for evaluation were apolipoprotein E (APOE), tumor necrosis factor alpha (TNF 238G>A - rs361525), interleukin 1 beta (IL1B 511C>T - rs16944 and IL1B -31T>C - rs1143627), activin-like kinase 1 (ACVRL1 IVS3-35A>G - rs2071219), endoglin (ENG 207G>A - rs11545664), and interleukin 6 (IL6 174G>C - rs1800795). Results In the single analysis, we observed statistically significant differences in the allele distributions for IL1B -31T>C (rs1143627) between the BAVM patients and control subjects (P = 0.02). There was a trend toward significance for the association between the IL1B 511C>T (rs16944) allele and BAVM risk (P = 0.07). In further logistic regression analysis, no polymorphism was significantly associated with the risk of BAVM. No polymorphisms were associated with hemorrhage presentation according to both single and multivariable analyses. Conclusions In our sample from a south Brazil population, we found no association between the risks of BAVM and ICH presentation with any of the selected polymorphisms. PMID:27026833

  11. Replication Study of Polymorphisms Associated With Brain Arteriovenous Malformation in a Population From South of Brazil.

    PubMed

    Franciscatto, André Cerutti; Ludwig, Fernanda S; Matte, Ursula S; Mota, Simone; Stefani, Marco A

    2016-02-24

    The aim of this study was to reproducibly determine if any of the polymorphisms were associated with the susceptibility to brain arteriovenous malformations (BAVM) or the risk of intracranial hemorrhage (ICH) presentation. We recruited 63 BAVM patients and 96 controls. The polymorphisms selected for evaluation were apolipoprotein E (APOE), tumor necrosis factor alpha (TNF 238G>A - rs361525), interleukin 1 beta (IL1B 511C>T - rs16944 and IL1B -31T>C - rs1143627), activin-like kinase 1 (ACVRL1 IVS3-35A>G - rs2071219), endoglin (ENG 207G>A - rs11545664), and interleukin 6 (IL6 174G>C - rs1800795). In the single analysis, we observed statistically significant differences in the allele distributions for IL1B -31T>C (rs1143627) between the BAVM patients and control subjects (P = 0.02). There was a trend toward significance for the association between the IL1B 511C>T (rs16944) allele and BAVM risk (P = 0.07). In further logistic regression analysis, no polymorphism was significantly associated with the risk of BAVM. No polymorphisms were associated with hemorrhage presentation according to both single and multivariable analyses. In our sample from a south Brazil population, we found no association between the risks of BAVM and ICH presentation with any of the selected polymorphisms.

  12. Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia

    PubMed Central

    Park, Sung Ok; Wankhede, Mamta; Lee, Young Jae; Choi, Eun-Jung; Fliess, Naime; Choe, Se-Woon; Oh, Seh-Hoon; Walter, Glenn; Raizada, Mohan K.; Sorg, Brian S.; Oh, S. Paul

    2009-01-01

    Arteriovenous malformations (AVMs) are vascular anomalies where arteries and veins are directly connected through a complex, tangled web of abnormal arteries and veins instead of a normal capillary network. AVMs in the brain, lung, and visceral organs, including the liver and gastrointestinal tract, result in considerable morbidity and mortality. AVMs are the underlying cause of three major clinical symptoms of a genetic vascular dysplasia termed hereditary hemorrhagic telangiectasia (HHT), which is characterized by recurrent nosebleeds, mucocutaneous telangiectases, and visceral AVMs and caused by mutations in one of several genes, including activin receptor–like kinase 1 (ALK1). It remains unknown why and how selective blood vessels form AVMs, and there have been technical limitations to observing the initial stages of AVM formation. Here we present in vivo evidence that physiological or environmental factors such as wounds in addition to the genetic ablation are required for Alk1-deficient vessels to develop to AVMs in adult mice. Using the dorsal skinfold window chamber system, we have demonstrated for what we believe to be the first time the entire course of AVM formation in subdermal blood vessels by using intravital bright-field images, hyperspectral imaging, fluorescence recordings of direct arterial flow through the AV shunts, and vascular casting techniques. We believe our data provide novel insights into the pathogenetic mechanisms of HHT and potential therapeutic approaches. PMID:19805914

  13. A Modified Radiosurgery-Based Arteriovenous Malformation Grading Scale and Its Correlation With Outcomes

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Wegner, Rodney E.; Oysul, Kaan; Pollock, Bruce E.

    Purpose: The Pittsburgh radiosurgery-based arteriovenous malformation (AVM) grading scale was developed to predict patient outcomes after radiosurgery and was later modified with location as a two-tiered variable (deep vs. other). The purpose of this study was to test the modified radiosurgery-based AVM score in a separate set of AVM patients managed with radiosurgery. Methods and Materials: The AVM score is calculated as follows: AVM score = (0.1)(volume, cc) + (0.02)(age, years) + (0.5)(location; frontal/temporal/parietal/occipital/intraventricular/corpus callosum/cerebellar = 0, basal ganglia/thalamus/brainstem = 1). Testing of the modified system was performed on 293 patients having AVM radiosurgery from 1992 to 2004 at themore » University of Pittsburgh with dose planning based on a combination of stereotactic angiography and MRI. The median patient age was 38 years, the median AVM volume was 3.3 cc, and 57 patients (19%) had deep AVMs. The median modified AVM score was 1.25. The median patient follow-up was 39 months. Results: The modified AVM scale correlated with the percentage of patients with AVM obliteration without new deficits ({<=}1.00, 62%; 1.01-1.50, 51%; 1.51-2.00, 53%; and >2.00, 32%; F = 11.002, R{sup 2} = 0.8117, p = 0.001). Linear regression also showed a statistically significant correlation between outcome and dose prescribed to the margin (F = 25.815, p <0.001). Conclusions: The modified radiosurgery-based AVM grading scale using location as a two-tiered variable correlated with outcomes when tested on a cohort of patients who underwent both angiography and MRI for dose planning. This system can be used to guide choices among observation, endovascular, surgical, and radiosurgical management strategies for individual AVM patients.« less

  14. [Hemangiomas and vascular malformations of the head and neck].

    PubMed

    Hassmann-Poznańska, Elibieta; Kurzyna, Agnieszka

    2006-01-01

    This paper presents the review of current knowledge regarding vascular lesions of the head and neck. For many years the term hemangioma was used to describe all vascular lesions. Mulliken and Glowacki classified congenital vascular lesions and recognized two distinct entities, hemangiomas-vascular tumors and vascular malformations. Hemangiomas are usually not present at birth, proliferate during first year of life and then involute. They are composed of proliferating endothelial cells. Vascular malformations are always present at birth although not always apparent, increase slowly in size throughout whole life and never involute. They enlarge by hypertrophy of malformed vessels. Vascular malformations can be further subdivided according to the type of involved vessels as arterial, arteriovenous, venous, capillary or lymphatic. Accurate diagnosis of hemangiomas and vascular malformations remains a challenge for physicians. Although majority of hemangiomas are self limiting lesions some of them may develop complications such as; ulceration, airway obstruction, ophthalmic complications, psychosocial consequences. Segmental hemangiomas are associated with the risk of structural anomalies such as those that occur in PHACE syndrome. Clinical presentation and forms of treatment of various forms of vascular malformations are presented. Vascular malformations have to be treated according to their histopathology and location, as well as their hemodynamic features shown by radiological examinations.

  15. Single-Fraction Proton Beam Stereotactic Radiosurgery for Cerebral Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hattangadi-Gluth, Jona A.; Chapman, Paul H.; Kim, Daniel

    2014-06-01

    Purpose/Objective(s): To evaluate the obliteration rate and potential adverse effects of single-fraction proton beam stereotactic radiosurgery (PSRS) in patients with cerebral arteriovenous malformations (AVMs). Methods and Materials: From 1991 to 2010, 248 consecutive patients with 254 cerebral AVMs received single-fraction PSRS at our institution. The median AVM nidus volume was 3.5 cc (range, 0.1-28.1 cc), 23% of AVMs were in critical/deep locations (basal ganglia, thalamus, or brainstem), and the most common prescription dose was 15 Gy(relative biological effectiveness [RBE]). Univariable and multivariable analyses were performed to assess factors associated with obliteration and hemorrhage. Results: At a median follow-up time of 35 months (range, 6-198 months),more » 64.6% of AVMs were obliterated. The median time to total obliteration was 31 months (range, 6-127 months), and the 5-year and 10-year cumulative incidence of total obliteration was 70% and 91%, respectively. On univariable analysis, smaller target volume (hazard ratio [HR] 0.78, 95% confidence interval [CI] 0.86-0.93, P<.0001), smaller treatment volume (HR 0.93, 95% CI 0.90-0.96, P<.0001), higher prescription dose (HR 1.16, 95% CI 1.07-1.26, P=.001), and higher maximum dose (HR 1.14, 95% CI 1.05-1.23, P=.002) were associated with total obliteration. Deep/critical location was also associated with decreased likelihood of obliteration (HR 0.68, 95% CI 0.47-0.98, P=.04). On multivariable analysis, critical location (adjusted HR [AHR] 0.42, 95% CI 0.27-0.65, P<.001) and smaller target volume (AHR 0.81, 95% CI 0.68-0.97, P=.02) remained associated with total obliteration. Posttreatment hemorrhage occurred in 13 cases (5-year cumulative incidence of 7%), all among patients with less than total obliteration, and 3 of these events were fatal. The most common complication was seizure, controlled with medications, both acutely (8%) and in the long term (9.1%). Conclusions: The current series is the

  16. [Galen's "On bones for beginners" translation from the Greek text and discussion].

    PubMed

    Sakai, Tatsuo; Ikeda, Reitaro; Sawai, Tadashi

    2007-09-01

    Galen's article "On bones for beginners" was translated literally from the Greek text (Kühn's edition, vol. 2, pp. 732-778) into Japanese, applying the knowledge of modern anatomy. The previous Latin and English translations were utilized as references for the present translation. The present study has revealed that many of the current basic vocabularies for the bones and junctions were established already in Galen's treatises, but have changed their meanings and usages considerably. It has become also apparent that, for the skull, Galen did not observe individual bones but distinguished them by precise observations on the sutures of the skull in monkeys. The precise understanding of Galenic anatomy provides essential information to understand the origin of current anatomy.

  17. Popliteal cyst before William Baker: first report in the Galenic Corpus.

    PubMed

    Papadakis, Marios; Manios, Andreas; Trompoukis, Constantinos

    2017-04-01

    A popliteal cyst, also known as Baker cyst, is a benign fluctuant swelling of the gastrocnemius-semimembranosus bursa in the popliteal fossa at the back of the knee. The cyst is named after William Morrant Baker, who is considered to have first described this fluid collection with new sac formation outside of the knee-joint in 1877. We herein present a short description contained in the Galenic Corpus that appears to match to what we now call the Baker cyst. Α thorough survey of Kühn's Galenic Corpus was performed. The 14th book of Kühn's edition contains a brief review of all the anatomical structures and pathologies. In the 17th chapter of this treatise, devoted to skin lesions, the author states that steatomas appear in the popliteal fossa. However, it is no more believed to reflect the Galenic teaching and is ascribed to Pseudo-Galen. With regard to the descriptions survived and the anatomy knowledge in the post-Galenic era time, the brief report of the ancient text of unknown origin, appears to match what we now describe as popliteal cyst.

  18. New predictive model for microsurgical outcome of intracranial arteriovenous malformations: study protocol.

    PubMed

    Tong, Xianzeng; Wu, Jun; Cao, Yong; Zhao, Yuanli; Wang, Shuo

    2017-01-27

    Although microsurgical resection is currently the first-line treatment modality for arteriovenous malformations (AVMs), microsurgery of these lesions is complicated due to the fact that they are very heterogeneous vascular anomalies. The Spetzler-Martin grading system and the supplementary grading system have demonstrated excellent performances in predicting the risk of AVM surgery. However, there are currently no predictive models based on multimodal MRI techniques. The purpose of this study is to propose a predictive model based on multimodal MRI techniques to assess the microsurgical risk of intracranial AVMs. The study consists of 2 parts: the first part is to conduct a single-centre retrospective analysis of 201 eligible patients to create a predictive model of AVM surgery based on multimodal functional MRIs (fMRIs); the second part is to validate the efficacy of the predictive model in a prospective multicentre cohort study of 400 eligible patients. Patient characteristics, AVM features and multimodal fMRI data will be collected. The functional status at pretreatment and 6 months after surgery will be analysed using the modified Rankin Scale (mRS) score. The patients in each part of this study will be dichotomised into 2 groups: those with improved or unchanged functional status (a decreased or unchanged mRS 6 months after surgery) and those with worsened functional status (an increased mRS). The first part will determine the risk factors of worsened functional status after surgery and create a predictive model. The second part will validate the predictive model and then a new AVM grading system will be proposed. The study protocol and informed consent form have been reviewed and approved by the Institutional Review Board of Beijing Tiantan Hospital Affiliated to Capital Medical University (KY2016-031-01). The results of this study will be disseminated through printed media. NCT02868008. Published by the BMJ Publishing Group Limited. For permission to use

  19. Geometric parameter analysis to predetermine optimal radiosurgery technique for the treatment of arteriovenous malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Mestrovic, Ante; Clark, Brenda G.; Department of Medical Physics, British Columbia Cancer Agency, Vancouver, British Columbia

    2005-11-01

    Purpose: To develop a method of predicting the values of dose distribution parameters of different radiosurgery techniques for treatment of arteriovenous malformation (AVM) based on internal geometric parameters. Methods and Materials: For each of 18 previously treated AVM patients, four treatment plans were created: circular collimator arcs, dynamic conformal arcs, fixed conformal fields, and intensity-modulated radiosurgery. An algorithm was developed to characterize the target and critical structure shape complexity and the position of the critical structures with respect to the target. Multiple regression was employed to establish the correlation between the internal geometric parameters and the dose distribution for differentmore » treatment techniques. The results from the model were applied to predict the dosimetric outcomes of different radiosurgery techniques and select the optimal radiosurgery technique for a number of AVM patients. Results: Several internal geometric parameters showing statistically significant correlation (p < 0.05) with the treatment planning results for each technique were identified. The target volume and the average minimum distance between the target and the critical structures were the most effective predictors for normal tissue dose distribution. The structure overlap volume with the target and the mean distance between the target and the critical structure were the most effective predictors for critical structure dose distribution. The predicted values of dose distribution parameters of different radiosurgery techniques were in close agreement with the original data. Conclusions: A statistical model has been described that successfully predicts the values of dose distribution parameters of different radiosurgery techniques and may be used to predetermine the optimal technique on a patient-to-patient basis.« less

  20. Impact of Onyx Embolization on Radiosurgical Management of Cerebral Arteriovenous Malformations: Treatment and Outcome.

    PubMed

    Strauss, Ido; Haim, Oz; Umansky, Daniel; Corn, Benjamin W; Frolov, Vladimir; Shtraus, Natan; Maimon, Shimon; Kanner, Andrew A

    2017-12-01

    Stereotactic radiosurgery (SRS) is a well-established treatment modality for cerebral arteriovenous malformations (AVMs). The main limiting factor in the radiosurgical treatment of AVMs is the volume of the nidus, with high-grade lesions often requiring combined treatment to reduce the SRS target volume. To overcome this limitation, we have been using a combined treatment approach consisting of endovascular embolization with Onyx followed by SRS. To evaluate our clinical experience for safety and feasibility of this multimodality treatment approach. This is a retrospective review of all adult patients with cerebral AVMs who received SRS treatment to their AVM after endovascular embolization with Onyx between June 2007 and June 2014. Thirty-five consecutive patients were identified. The mean follow-up period was 52.4 ± 22.6 months (range 18-97 months). We confirmed 18 (51.4%) complete nidus closures at a median time of 49.5 months (range 6.5-81 months) from SRS. High-resolution Magnetic resonance imaging/magnetic resonance angiography was performed routinely in all patients until closure of the nidus. Digital subtraction angiography was performed to confirm complete obliteration in 5 of the patients (28%); 13 patients are either planned for digital subtraction angiography or have refused it. In 6 patients (17%) a significant flow reduction was noted after a mean of 32 ± 16 months. No significant improvement was observed in 9 patients (26%) during the follow-up period. Two patients were lost to follow-up. The multimodality treatment of cerebral AVMs using embolization with Onyx followed by SRS is feasible and safe. The use of Onyx significantly reduced the SRS treatment target volume. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Curative transvenous embolization for ruptured brain arteriovenous malformations: a single center experience from China.

    PubMed

    He, Yingkun; Bai, Weixing; Li, Tianxiao; Hui, Ferdinand K; He, Yanyan; Xu, Bin

    2018-05-09

    To evaluate the feasibility and safety of transvenous embolization treatment for brain arteriovenous malformations (bAVM). Between November 2016 and August 2017, a transvenous endovascular embolization protocol was implemented at the Henan Provincial People's Hospital for consecutive patients with ruptured bAVM. Therapeutic decision making was based on Spetzler-Martin grades, bAVM location, pattern of venous drainage, and angioarchitecture. And the transvenous embolization procedure was combined with transarterial support. Complete angiographic obliteration of the nidus was the objective of treatment. Among the 10 patients with ruptured bAVM, 8 were males. The Spetzler-Martin grades before the transvenous embolization session were IV for 3 of the 10 patients, III for 5 patients, II for 1 patient, and I for 1 patient. The preprocedural modified Rankin scale score (mRS) was 0-2 for 6 of the 10 patients, and 5 of 10 patients also had deep venous drainage. Arterial blood pressure control management and venous pressure cooker technique were used in all 10 patients; 9 patients had immediate angiographic occlusion, and 2 patients were found with a ventricular hemorrhage, which did not cause any disability after medical treatment. Seven patients underwent angiography 3 to 5 months after operation and complete obliteration of the nidus conformed. The median clinical follow-up for all 10 patients was 8 months (range 3-12), epilepsy occurred in one patient 3 months after the procedure, and the mRS scores of all patients were ≤1. Transvenous embolization of bAVM is feasible and may improve the cure rate. However, the safety and long-term effects need further validation. Copyright © 2018 Elsevier Inc. All rights reserved.

  2. Indocyanine green videoangiography "in negative": definition and usefulness in intracranial dural arteriovenous fistulae.

    PubMed

    Simal Julián, Juan Antonio; Miranda Lloret, Pablo; Aparici Robles, Fernando; Beltrán Giner, Andrés; Botella Asunción, Carlos

    2013-09-01

    Indocyanine green videoangiography (IGV) raises important limitations when we use it in vascular pathology, especially in cases with arterialization of the venous system such as arteriovenous malformations and fistulae. Our objective was to provide a simple procedure that overcomes the limitations of conventional IGV. We define IGV in negative (IGV-IN), so-called because, in its first phase, the vessel to analyze is clipped, and we report 3 cases of intracranial dural arteriovenous fistulae treated with this procedure. In 2011, we applied IGV-IN to 3 patients at our center with Borden type III intracranial arteriovenous fistulae. In all 3 cases, IGV-IN enabled both diagnosis and post-dural arteriovenous fistula exclusion control in 1 integrated procedure no longer than 1 minute, requiring only 1 visualization. IGV-IN is an improvement over the conventional IGV method and is able to provide more information in a shorter period of time. It is an intuitive and highly visual procedure, and, more importantly, it is reversible. Studies with larger samples are necessary to determine whether IGV-IN can further reduce the need for postoperative digital subtraction angiography.

  3. Assessment of craniospinal arteriovenous malformations at 3T with highly temporally and highly spatially resolved contrast-enhanced MR angiography.

    PubMed

    Saleh, R S; Lohan, D G; Villablanca, J P; Duckwiler, G; Kee, S T; Finn, J P

    2008-05-01

    Patients with arteriovenous malformation (AVM) are known to have an elevated risk of complications with conventional catheter angiography (CCA) but nonetheless require monitoring of hemodynamics. Thus, we aimed to evaluate both anatomy and hemodynamics in patients with AVM noninvasively by using contrast-enhanced MR angiography (CE-MRA) at 3T and to compare the results with CCA. Institutional review board approval and informed consent were obtained for this Health Insurance Portability and Accountability Act-compliant study. Twenty control subjects without vascular malformation (6 men, 18-70 years of age) and 10 patients with AVMs (6 men, 20-74 years of age) underwent supra-aortic time-resolved and high-spatial-resolution CE-MRA at 3T. Large-field-of-view coronal acquisitions extending from the root of the aorta to the cranial vertex were obtained for both MRA techniques. Image quality was assessed by 2 specialized radiologists by using a 4-point scale. AVM characteristics and nidus size were evaluated by using both CE-MRA and CCA in all patients. In patients, 96.6% (319/330) of arterial segments on high-spatial-resolution MRA and 87.7% (272/310) of arterial segments on time-resolved MRA were graded excellent/good. MRA showed 100% specificity for detecting feeding arteries and venous drainage (n = 8) and complete obliteration of the AVM in 2 cases (concordance with CCA). Nidus diameters measured by both MRA and CCA resulted in a very strong correlation (r = 0.99) with a mild overestimation by MRA (0.10 cm by using the Bland-Altman plot). By combining highly temporally resolved and highly spatially resolved MRA at 3T as complementary studies, one can assess vascular anatomy and hemodynamics noninvasively in patients with AVM.

  4. Post procedure headache in patients treated for neurovascular arteriovenous malformations and aneurysms using endovascular therapy.

    PubMed

    Khan, Sabrina; Amin, Faisal Mohammad; Hauerberg, John; Holtmannspötter, Markus; Petersen, Julie Falkenberg; Fakhril-Din, Zainab; Gaist, David; Ashina, Messoud

    2016-12-01

    Though endovascular therapy (EVT) is increasingly applied in the treatment of intracranial vascular lesions, little is known about the effect of EVT on post-procedure headache. We aimed to investigate the prevalence of headache in patients who have undergone EVT for cerebral arteriovenous malformations (AVMs) and aneurysms. A total of 324 patients underwent EVT treatment for aneurysms and AVMs at the Danish National Hospital from January 2012 to December 2014. We applied strict exclusion criteria in order to minimize the effect of other factors on headache occurrence, e.g., craniotomy. Eligible subjects were phone-interviewed using a purpose-developed semi-structured questionnaire. Headaches were classified according to ICHD-III beta criteria. The 59 patients underwent treatment of aneurysms (n = 43), cranial dural fistulas (n = 11), and AVMs (n = 5). There was a significant increase in overall headache (p = 0.017) and tension-type headache (TTH) (p = 0.012) within the first 3 months after EVT compared to 1 month before EVT. However, at interview time (median 2.5 years post-EVT), the increase in overall headache, migraine, and tension-type headache was not statistically significant. A minority of patients experienced headaches for the first time within 3 months of their EVT (migraine 4, TTH 10). At interview time, 50 % of these new headaches still persisted. Our results suggest a temporary increase in headache in the first 3 months after EVT, which normalizes over time. Clinicians may use this knowledge to better inform their patients of functional outcomes after their EVT procedure.

  5. Safety and efficacy of endovascular therapy and gamma knife surgery for brain arteriovenous malformations in China: Study protocol for an observational clinical trial.

    PubMed

    Jin, Hengwei; Huo, Xiaochuan; Jiang, Yuhua; Li, Xiaolong; Li, Youxiang

    2017-09-01

    Brain arteriovenous malformations (BAVMs) are associated with high morbidity and mortality. The treatment of BAVM remains controversial. Microinvasive treatment, including endovascular therapy and gamma knife surgery, has been the first choice in many conditions. However, the overall clinical outcome of microinvasive treatment remains unknown and a prospective trial is needed. This is a prospective, non-randomized, and multicenter observational registry clinical trial to evaluate the safety and efficacy of microinvasive treatment for BAVMs. The study will require up to 400 patients in approximately 12 or more centers in China, followed for 2 years. Main subjects of this study are BAVM patients underwent endovascular therapy and/or gamma knife surgery. The trial will not affect the choice of treatment modality. The primary outcomes are perioperative complications (safety), and postoperative hemorrhage incidence rate and complete occlusion rate (efficacy). Secondary outcomes are elimination of hemorrhage risk factors (coexisting aneurysms and arteriovenous fistula), volume reduction and remission of symptoms. Safety and efficacy of endovascular therapy, gamma knife surgery, and various combination modes of the two modalities will be compared. Operative complications and outcomes at pretreatment, post-treatment, at discharge and at 3 months, 6 months and 2 years follow-up intervals will be analyzed using the modified Rankin Scale (mRS). The most confusion on BAVM treatment is whether to choose interventional therapy or medical therapy, and the choice of interventional therapy modes. This study will provide evidence for evaluating the safety and efficacy of microinvasive treatment in China, to characterize the microinvasive treatment strategy for BAVMs.

  6. A challenging entity of endovascular embolization with ONYX for brainstem arteriovenous malformation: Experience from 13 cases.

    PubMed

    Jin, Hengwei; Liu, Zhan; Chang, Qing; Chen, Chang; Ge, Huijian; Lv, Xianli; Li, Youxiang

    2017-10-01

    Objective Brainstem arteriovenous malformations (AVMs) are rare lesions with a high risk of intracranial hemorrhage and are challenging to treat. We present our experience of endovascular embolization with Onyx in these aggressive lesions. Materials and methods Between 2007 and 2016, 13 patients with brainstem AVMs were embolized with Onyx at our center. Twelve patients presented with intracranial hemorrhage and one with headache. Retrospective examinations of patient demographics, clinical presentation, angiographic features, treatment modalities, postoperative complications and outcomes were carried out. Results The AVMs were in the midbrain in 10 patients (one anterior and nine posterior or dorsal), in the posterior pons in two and pontomedullary in one. Complete occlusion was achieved in three patients. Gamma knife radiosurgery was performed in six patients who were near-completely or partially embolized. Postoperative complications, including five cases of ischemia and one case of hemorrhage, resulted in four cases of neurological deterioration and two deaths. Clinical follow-up was obtained in 10 patients at a mean period of 45.2 months (range 3 to 93 months). During the follow-up, good clinical outcomes were observed in seven patients with posterior or dorsal midbrain AVMs, and one patient with a posterior pons AVM that was partially occluded died of intracranial hemorrhage. Conclusion Endovascular embolization for brainstem AVM with Onyx is a technical challenge and the reflux of Onyx may cause severe complications. Individualized treatment is needed based on the specific subtype of brainstem AVM.

  7. Interlocking Detachable Coil Embolization by Technique of Dumbbell-Shaped Framing and Filling and Bridge Formation Under Balloon Dilatation for Huge Conglomerate Pulmonary Arteriovenous Malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kawai, Nobuyuki, E-mail: nobkawai@wakayama-med.ac.jp; Sato, Morio, E-mail: morisato@mail.wakayama-med.ac.jp; Minamiguchi, Hiroyuki, E-mail: hiromina4@hotmail.com

    A 60-year-old woman presented with a conglomerate pulmonary arteriovenous malformation (PAVM) size 8.2 Multiplication-Sign 7.2 cm on chest X-ray. Feeding arteries were A{sub a}{sup 7} and A{sub b}{sup 7}, A{sup 8}, and A{sup 10}. The diameter and length of the A{sub b}{sup 7} neck were 15.5 and 16 mm, respectively. After percutaneous transcatheter embolization of A{sup 8} and A{sup 10}, PTE of A{sub a}{sup 7} and A{sub b}{sup 7} was conducted under balloon occlusion with interlocking detachable coils using a technique of dumbbell-shaped framing and filling, making a bridge from A{sub b}{sup 7} to the trunk of A{sup 9} andmore » A{sup 10}across A{sup 7}. Follow-up computed tomography 10 months after treatment showed marked shrinkage of the PAVM.« less

  8. Angioarchitectural features of brain arteriovenous malformations associated with seizures: a single center retrospective series.

    PubMed

    Sturiale, C L; Rigante, L; Puca, A; Di Lella, G; Albanese, A; Marchese, E; Di Rocco, C; Maira, G; Colicchio, G

    2013-05-01

    Epileptic seizures account for 24-40% of all clinical onsets in patients with brain arteriovenous malformations (AVMs). We retrospectively reviewed the angioarchitectural features of AVMs associated with seizures in 168 patients admitted to our Department from 1997 to 2012. Patients were dichotomized according to demographic characteristics, type of treatment, bleeding occurrence, and morphological and topographic features. Clinical status at admission and discharge was also recorded. The association of each one of these variables with seizures occurrence was statistically tested. Continuous variables and outcome were compared with Student's t-test, whereas categorical ones were compared using Fisher's exact test. The independent contribution of some seizures predictors was assessed with a logistic regression model. Associations were considered significant for P < 0.05. About 29% patients showed seizures and 47% bleeding. No significant difference in age and sex was observed between patients with and without seizures. AVMs > 4 cm (P = 0.001) and those fed by dilated arterial feeders (P = 0.02) were associated with increased risk of seizures. A higher risk of seizures occurrence was also observed in cortical AVMs compared with deeper ones (75.5% vs. 55.4%; P = 0.01), and in AVMs fed by middle and posterior cerebral arteries branches compared with the other vessels (81.6% vs. 45.3%; P < 0.001 and 48.9% vs. 23.5%; P = 0.002, respectively). No lobar predisposition was observed. A nidus > 4 cm also appeared as an independent risk factor of seizures occurrence (OR 2.82; 95% CI, 1.26-6.31; P = 0.009) at logistic regression analysis. AVM morphology, especially nidus dimension, appeared to more significantly influence seizures occurrence than their topography. © 2013 The Author(s) European Journal of Neurology © 2013 EFNS.

  9. Linear Accelerator-Based Radiosurgery Alone for Arteriovenous Malformation: More Than 12 Years of Observation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Matsuo, Takayuki, E-mail: takayuki@nagasaki-u.ac.jp; Kamada, Kensaku; Izumo, Tsuyoshi

    Purpose: Although radiosurgery is an accepted treatment method for intracranial arteriovenous malformations (AVMs), its long-term therapeutic effects have not been sufficiently evaluated, and many reports of long-term observations are from gamma-knife facilities. Furthermore, there are few reported results of treatment using only linear accelerator (LINAC)-based radiosurgery (LBRS). Methods and Materials: Over a period of more than 12 years, we followed the long-term results of LBRS treatment performed in 51 AVM patients. Results: The actuarial obliteration rates, after a single radiosurgery session, at 3, 5, 10, and 15 years were 46.9%, 54.0%, 64.4%, and 68.0%, respectively; when subsequent radiosurgeries were included, themore » rates were 46.9%, 61.3%, 74.2%, and 90.3%, respectively. Obliteration rates were significantly related to target volumes ≥4 cm{sup 3}, marginal doses ≥12 Gy, Spetzler-Martin grades (1 vs other), and AVM scores ≥1.5; multivariate analyses revealed a significant difference for target volumes ≥4 cm{sup 3}. The postprocedural actuarial symptomatic radiation injury rates, after a single radiation surgery session, at 5, 10, and 15 years were 12.3%, 16.8%, and 19.1%, respectively. Volumes ≥4 cm{sup 3}, location (lobular or other), AVM scores ≥1.5, and the number of radiosurgery were related to radiation injury incidence; multivariate analyses revealed significant differences associated with volumes ≥4 cm{sup 3} and location (lobular or other). Conclusions: Positive results can be obtained with LBRS when performed with a target volume ≤4 cm{sup 3}, an AVM score ≤1.5, and ≥12 Gy radiation. Bleeding and radiation injuries may appear even 10 years after treatment, necessitating long-term observation.« less

  10. Proposal for a New Prognostic Score for Linac-Based Radiosurgery in Cerebral Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Milker-Zabel, Stefanie, E-mail: Stefanie_Milker-Zabel@med.uni-heidelberg.de; Kopp-Schneider, Annette; Wiesbauer, Hannah

    2012-06-01

    Purpose: We evaluate patient-, angioma-, and treatment-specific factors for successful obliteration of cerebral arteriovenous malformations (AVM) to develop a new appropriate score to predict patient outcome after linac-based radiosurgery (RS). Methods and Materials: This analysis in based on 293 patients with cerebral AVM. Mean age at treatment was 38.8 years (4-73 years). AVM classification according Spetzler-Martin was 55 patients Grade I (20.5%), 114 Grade II (42.5%), 79 Grade III (29.5%), 19 Grade IV (7.1%), and 1 Grade V (0.4%). Median maximum AVM diameter was 3.0 cm (range, 0.3-10 cm). Median dose prescribed to the 80% isodose was 18 Gy (range,more » 12-22 Gy). Eighty-five patients (29.1%) had prior partial embolization; 141 patients (51.9%) experienced intracranial hemorrhage before RS. Median follow-up was 4.2 years. Results: Age at treatment, maximum diameter, nidus volume, and applied dose were significant factors for successful obliteration. Under presumption of proportional hazard in the dose range between 12 and 22 Gy/80% isodose, an increase of obliteration rate of approximately 25% per Gy was seen. On the basis of multivariate analysis, a prediction score was calculated including AVM maximum diameter and age at treatment. The prediction error up to the time point 8 years was 0.173 for the Heidelberg score compared with the Kaplan-Meier value of 0.192. An increase of the score of 1 point results in a decrease of obliteration chance by a factor of 0.447. Conclusion: The proposed score is linac-based radiosurgery-specific and easy to handle to predict patient outcome. Further validation on an independent patient cohort is necessary.« less

  11. Fiber 1.56-1.9 μm lasers in treatment of vascular malformations in children and adults

    NASA Astrophysics Data System (ADS)

    Abushkin, Ivan A.; Privalov, Valeriy A.; Lappa, Alexander V.; Minaev, Vladimir P.

    2013-03-01

    A few new low invasive fiber laser technologies for treatment of 1) capillary malformations (port-wine stains), 2) venous, arterial, and arteriovenous malformations, 3) lymphatic malformations of 3 types: micro, small and large-cystic lymphangiomas are presented in this work. There were applied 1.56 μm laser distant photocoagulation, 1.56 μm laser endovascular thermotherapy, 1.9 μm laser instant ablation, 1.9 μm laser interstitial thermotherapy, and 1.9 μm laser excision. The technologies were applied to about 300 patients. Good clinical and esthetic results have been achieved in great majority cases.

  12. A novel non-contrast-enhanced MRA using silent scan for evaluation of brain arteriovenous malformation: A case report and review of literature.

    PubMed

    Moon, Jin Il; Baek, Hye Jin; Ryu, Kyeong Hwa; Park, Hyun

    2017-11-01

    Brain arteriovenous malformations (AVMs) are congenital vascular abnormalities involving abnormal connections between arteries and veins. In clinical practice, imaging studies help evaluate feeding arteries, niduses, draining venous systems, and coexisting complications in patients with brain AVM. They also have an impact on decision-making regarding clinical management. We applied a novel non-contrast-enhanced MR angiography (MRA) technique, termed "silent MRA," for evaluating an incidental brain AVM. Here, we describe the clinical case with radiological review and highlight the technical background and clinical usefulness of silent MRA. A 60-year-old woman underwent neuroimaging study including MRA to evaluate intracranial cause of headache. The brain AVM, including its nidus and draining veins, was conspicuously delineated on silent MRA images; these findings correlated well with conventional angiographic findings. The patient did not receive interventional or surgical treatment. The patient is being followed up regularly at the outpatient department. The silent MRA can be a suitable imaging modality for repeated follow-up evaluation for not only brain AVMs but also various intracranial vascular diseases without the use of contrast materials.

  13. Persistence of pulmonary arteriovenous malformations after successful embolotherapy with Amplatzer vascular plug: long-term results

    PubMed Central

    Abdel-Aal, Ahmed Kamel; Ibrahim, Rafik Mohamed; Moustafa, Amr Soliman; Hamed, Maysoon Farouk; Saddekni, Souheil

    2016-01-01

    PURPOSE We aimed to evaluate the frequency of persistence and complication rates of pulmonary arteriovenous malformations (PAVMs) treated with Amplatzer vascular plug (AVP) or Amplatzer vascular plug type 2 (AVP2). METHODS We retrospectively reviewed a total of 22 patients with 54 PAVMs between June 2004 and June 2014. We included 12 patients with 35 PAVMs who received percutaneous embolization using AVP or AVP2 only without the use of any other embolic devices. The mean follow-up was 54±24.3 months (range, 31–97 months). The primary end-points of the study were the efficacy of embolotherapy, the increase in oxygen saturation, and the persistence of PAVM on follow-up. Secondary end point was the incidence of complications. RESULTS The study included 10 female and two male patients with a mean age of 50.2±13.7 years (range, 21–66 years). All PAVMs had a simple angioarchitecture. The technical success of the procedure for PAVM occlusion was 100%. There was a significant increase in the oxygen saturation following embolotherapy (P < 0.0001). Follow-up computed tomography angiography revealed successful treatment in 34 PAVMs (97%) and failed treatment in one PAVM (3%). Twenty-three aneurysmal sacs (67%) showed complete disappearance. The failed treatment was due to persistence of PAVM caused by subsequent development of systemic reperfusion, which did not require further intervention. There were two minor complications but no major complications were encountered. CONCLUSION Embolotherapy of PAVMs using AVP or AVP2 devices is safe and effective, with high technical success rate, low persistence and complication rates, and with excellent long-term results. PMID:27244759

  14. Treatment of Early-stage Extracranial Arteriovenous Malformations with Intralesional Interstitial Bleomycin Injection: A Pilot Study.

    PubMed

    Jin, Yunbo; Zou, Yun; Hua, Chen; Chen, Hui; Yang, Xi; Ma, Gang; Chang, Lei; Qiu, Yajing; Lyu, Dongze; Wang, Tianyou; Chang, Shih-Jen; Qiao, Congzhen; Luo, Chunfen; Tremp, Mathias; Lin, Xiaoxi

    2018-04-01

    Purpose To assess the efficacy and safety of intralesional interstitial bleomycin injection in the treatment of early-stage (Schobinger stage I or II) extracranial arteriovenous malformations (AVMs). Materials and Methods This prospective study involved 34 patients with early-stage AVMs, as defined by the Schobinger staging system. The patients received intralesional interstitial bleomycin injected at a maximum dose of 15 000 IU or 1000 IU per kilogram of body weight for children who weighed less than 15 kg per procedure for a total of 6 months (once every month). Therapeutic outcome was evaluated by the degree of devascularization at angiography and the clinical outcome 3 months after the last treatment. Further follow-up was evaluated based on further clinical outcome. Adverse events were recorded according to the Society of Interventional Radiology classification. Results Of the 34 patients with early-stage AVM, 32 (mean age, 20.5 years; 24 female [75%]) completed the study. The results showed that 27 (84.4%, 95% confidence interval [CI]: 71.1, 97.7) patients were responsive to bleomycin injection, including nine (28.1%) with a complete response. Four (12.5%) patients showed no response, and one (3.1%) patient experienced worsening 3 months after the last treatment. During further follow-up (mean follow-up time, 20.7 months; range, 5-28 months), the outcome remained stable in 31 (96.9%) of the 32 patients. A major complication, anaphylactic shock, was observed in one (3.1%, 95% CI: 0, 9.5) patient. Common minor complications included hyperpigmentation, nausea, pruritus, and bullae. Conclusion Intralesional interstitial bleomycin injection is a feasible approach for early-stage AVMs and yields safe and effective outcomes. © RSNA, 2017.

  15. Identification of a rare BMP pathway mutation in a non-syndromic human brain arteriovenous malformation via exome sequencing.

    PubMed

    Walcott, Brian P; Winkler, Ethan A; Zhou, Sirui; Birk, Harjus; Guo, Diana; Koch, Matthew J; Stapleton, Christopher J; Spiegelman, Dan; Dionne-Laporte, Alexandre; Dion, Patrick A; Kahle, Kristopher T; Rouleau, Guy A; Lawton, Michael T

    2018-01-01

    Brain arteriovenous malformations (AVMs) are abnormal connections between arteries and veins that can result in hemorrhagic stroke. A genetic basis for AVMs is suspected, and we investigated potential mutations in a 14-year-old girl who developed a recurrent brain AVM. Whole-exome sequencing (WES) of AVM lesion tissue and blood was performed accompanied by in silico modeling, protein expression observation in lesion tissue and zebrafish modeling. A stop-gain mutation (c.C739T:p.R247X) in the gene SMAD family member 9 ( SMAD9 ) was discovered. In the human brain tissue, immunofluorescent staining demonstrated a vascular predominance of SMAD9 at the protein level. Vascular SMAD9 was markedly reduced in AVM peri-nidal blood vessels, which was accompanied by a decrease in phosphorylated SMAD4, a downstream effector protein of the bone morphogenic protein signaling pathway. Zebrafish modeling ( Tg kdrl:eGFP ) of the morpholino splice site and translation-blocking knockdown of SMAD9 resulted in abnormal cerebral artery-to-vein connections with morphologic similarities to human AVMs. Orthogonal trajectories of evidence established a relationship between the candidate mutation discovered in SMAD9 via WES and the clinical phenotype. Replication in similar rare cases of recurrent AVM, or even more broadly sporadic AVM, may be informative in building a more comprehensive understanding of AVM pathogenesis.

  16. Incidental occlusion of anterior spinal artery due to Onyx reflux in embolization of spinal type II arteriovenous malformation.

    PubMed

    Kim, Joohyun; Lee, Jang-Bo; Cho, Tai-Hyoung; Hur, Junseok W

    2017-05-01

    Onyx embolization is one of the standard treatments for brain arteriovenous malformations (AVMs) and is a promising method for spinal AVMs as well. Its advantages have been emphasized, and few complications have been reported with Onyx embolization in spinal AVMs. Here, we report an incidental anterior spinal artery (ASA) occlusion due to Onyx reflux during embolization of a spinal type II AVM. A 15-year-old boy presented with weakness in both upper and lower extremities. Magnetic resonance imaging and spinal angiogram revealed a spinal type II AVM with two feeders including the right vertebral artery (VA) and the right deep cervical artery. Onyx embolization was performed gradually from the VA to the deep cervical artery and an unexpected Onyx reflux to the ASA was observed during the latter stage deep cervical artery embolization. Post-operative quadriplegia and low cranial nerves (CN) dysfunction were observed. Rehabilitation treatment was performed and the patient showed marked improvement of neurologic deterioration at 1-year follow-up. Onyx is an effective treatment choice for spinal AVMs. However, due to the small vasculature of the spine compared to the brain, the nidus is rapidly packed with a small amount of Onyx, which allows Onyx reflux to unexpected vessels. Extreme caution is required and dual-lumen balloon catheter could be considered for Onyx embolization in spinal AVMs treatment.

  17. Fatal rupture of a brain arteriovenous malformation flow-related aneurysm during microcatheter removal: a rare complication.

    PubMed

    Gabrieli, Joseph; Clarençon, Frédéric; Di Maria, Federico; Fahed, Robert; Boch, Anne-Laure; Degos, Vincent; Chiras, Jacques; Sourour, Nader-Antoine

    2015-04-01

    Intracranial aneurysms are relatively frequently encountered in patients with brain arteriovenous malformations (BAVMs). They may be located on the circle of Willis, on arterial feeders, or even inside the nidus. Because BAVM-associated aneurysms represent a risk factor of bleeding, the question of the timing and modality of their management remains a matter of debate in unruptured BAVMs. The authors present a case of fatal periprocedural rupture of a flow-related aneurysm (FRA) during the removal of the microcatheter after injection of a liquid embolic agent. A 40-year-old man was treated at the authors' institution for the management of a Spetzler-Martin Grade III left unruptured frontal BAVM, revealed by seizures and a focal neurological deficit attributed to flow steal phenomenon. After a multidisciplinary meeting, endovascular treatment was considered to reduce the flow of the BAVM. A proximal FRA located on the feeding internal carotid artery (ICA) was purposely left untreated because it did not meet the criteria of the authors' institution for preventative treatment (i.e., small size [2.5 mm]). During embolization, at the time of microcatheter retrieval, and after glue injection, the aneurysm unexpectedly ruptured. The aneurysm's rupture was attributed to the stress (torsion/flexion) on the ICA caused by the microcatheter removal. Despite the attempts to manage the bleeding, the patient eventually died of the acute increase of intracranial pressure related to the massive subarachnoid hemorrhage. This case highlights a previously unreported mechanism of FRA rupture during BAVM embolization: the stress transmitted to the parent artery during the removal of the microcatheter.

  18. Cerebral dominance for speech and handwriting of patients with cortical vascular malformations.

    PubMed

    Sass, K J; Buchanan, C P; Westerveld, M; Spencer, D D

    1994-10-01

    Lateralization of speech dominance was established using amobarbital for 22 patients with vascular malformations lateralized to the left cerebral hemisphere. Patients' histories were negative for clinically evident neurological events (e.g., seizures or hemorrhage) prior to adulthood. The vascular lesions were categorized as high flow arteriovenous malformations (AVMs) (n = 4), low flow AVMs (n = 6), cavernous hemangiomas (n = 10), or venous angiomas (n = 2) by reviewing angiographic findings and surgical pathology for those patients whose lesions were excised. Three of the malformations encroached upon primary language areas. The frequency of right hemisphere speech dominance was not significantly elevated in comparison with the normal population, even though the incidence of nonright-handedness was. Ninety-five percent of the patients were left hemisphere dominant for speech: only one patient, with a parietal lobe cavernous hemangioma, was found to be right hemisphere dominant for speech. This malformation did not involve the primary language areas. These findings suggest that vascular malformations do not affect speech dominance as readily as other neurological diseases, but frequently affect manual dominance.

  19. Progesterone receptors identified in vascular malformations of the head and neck.

    PubMed

    Duyka, Landon J; Fan, Chun Y; Coviello-Malle, Jean M; Buckmiller, Lisa; Suen, James Y

    2009-10-01

    To identify hormone receptors within vascular malformations (arteriovenous malformations [AVMs], venous malformations [VMs], and lymphatic malformations [LMs]) of the head and neck. Immunohistochemical staining for estrogen receptor (ER) and progesterone receptor (PR) was performed on archival vascular malformation tissue collected from both pediatric and adult patients. Tertiary referral center from 2006 to 2008. Twelve AVM, 10 VM, and eight LM specimens were stained for both ER and PR. Ten breast carcinoma specimens were used as controls, with the carcinoma cells serving as positive controls, and the endothelium and smooth muscle cells of the blood vessels serving as negative controls. Five normal supraglottic mucosal samples served as head and neck controls. The Fisher exact test was used for statistical analysis. Ten of the 12 (83%) AVM specimens stained diffusely positive for PR within the nuclei of the endothelium and smooth muscle of the malformed vessels (P < 0.0001). Five of the 10 (50%) VM specimens stained positive for PR (2 [20%] focal, 3 [30%] diffuse) within the nuclei of the endothelium and smooth muscle of the malformed vessels (P = 0.0325). Four of the eight (50%) LM specimens stained focally positive for PR within the nuclei of the endothelium of the malformed vessels (P = 0.0229). None of the vascular malformation specimens stained positive for ER. Our data suggest that PR, but not ER, is expressed in AVMs, VMs, and LMs of the head and neck.

  20. [Galenic forms for oral medication].

    PubMed

    El Semman, Ousseid; Certain, Agnès; Bouziane, Faouzia; Arnaud, Philippe

    2012-10-01

    Galenic science is interested in the art and the way of formulating an active principle with an excipient in order for it to be administered to the patient. The pharmaceutical forms envisage different administration routes, including by mouth. Nurses need to handle and sometimes modify the pharmaceutical form of a drug to make it easier for the patient to take. This requires vigilance.

  1. Three-dimensional magnetic resonance imaging based on time-of-flight magnetic resonance angiography for superficial cerebral arteriovenous malformation--technical note.

    PubMed

    Murata, Takahiro; Horiuchi, Tetsuyoshi; Rahmah, Nunung Nur; Sakai, Keiichi; Hongo, Kazuhiro

    2011-01-01

    Direct surgery remains important for the treatment of superficial cerebral arteriovenous malformation (AVM). Surgical planning on the basis of careful analysis from various neuroimaging modalities can aid in resection of superficial AVM with favorable outcome. Three-dimensional (3D) magnetic resonance (MR) imaging reconstructed from time-of-flight (TOF) MR angiography was developed as an adjunctive tool for surgical planning of superficial AVM. 3-T TOF MR imaging without contrast medium was performed preoperatively in patients with superficial AVM. The images were imported into OsiriX imaging software and the 3D reconstructed MR image was produced using the volume rendering method. This 3D MR image could clearly visualize the surface angioarchitecture of the AVM with the surrounding brain on a single image, and clarified feeding arteries including draining veins and the relationship with sulci or fissures surrounding the nidus. 3D MR image of the whole AVM angioarchitecture was also displayed by skeletonization of the surrounding brain. Preoperative 3D MR image corresponded to the intraoperative view. Feeders on the brain surface were easily confirmed and obliterated during surgery, with the aid of the 3D MR images. 3D MR imaging for surgical planning of superficial AVM is simple and noninvasive to perform, enhances intraoperative orientation, and is helpful for successful resection.

  2. Acute fatal hemorrhage from previously undiagnosed cerebral arteriovenous malformations in children: a single-center experience.

    PubMed

    Riordan, Coleman P; Orbach, Darren B; Smith, Edward R; Scott, R Michael

    2018-06-01

    OBJECTIVE The most significant adverse outcome of intracranial hemorrhage from an arteriovenous malformation (AVM) is death. This study reviews a single-center experience with pediatric AVMs to quantify the incidence and characterize clinical and radiographic factors associated with sudden death from the hemorrhage of previously undiagnosed AVMs in children. METHODS A single-center database review of the period from 2006 to 2017 identified all patients with a first-time intracranial hemorrhage from a previously undiagnosed AVM. Clinical and radiographic data were collected and compared between patients who survived to hospital discharge and those who died at presentation. RESULTS A total of 57 patients (average age 10.8 years, range 0.1-19 years) presented with first-time intracranial hemorrhage from a previously undiagnosed AVM during the study period. Of this group, 7/57 (12%) patients (average age 11.5 years, range 6-16 years) suffered hemorrhages that led directly to their deaths. Compared to the cohort of patients who survived their hemorrhage, patients who died were 4 times more likely to have an AVM in the posterior fossa. No clear pattern of antecedent triggering activity (sports, trauma, etc.) was identified, and 3/7 (43%) experienced cardiac arrest in the prehospital setting. Surviving patients were ultimately treated with resection of the AVM in 42/50 (84%) of cases. CONCLUSIONS Children who present with hemorrhage from a previously undiagnosed intracranial AVM had a 12% chance of sudden death in our single-institution series of pediatric cerebrovascular cases. Clinical triggers of hemorrhage are unpredictable, but subsequent radiographic evidence of a posterior fossa AVM was present in 57% of fatal cases, and all fatal cases were in locations with high risk of potential herniation. These data support a proactive, aggressive approach toward definitive treatment of AVMs in children.

  3. Transcatheter arterial embolization with ethylene vinyl alcohol copolymer (Onyx) for the treatment of hemorrhage due to uterine arteriovenous malformations.

    PubMed

    Barral, P-A; Saeed-Kilani, M; Tradi, F; Dabadie, A; Izaaryene, J; Soussan, J; Bartoli, J-M; Vidal, V

    2017-05-01

    The purpose of this study was to evaluate the effectiveness of ethylene vinyl alcohol copolymer (Onyx) as a single embolic agent for percutaneous arterial treatment of hemorrhage due to uterine arteriovenous malformations (AVMs). Twelve women (mean age, 33 years) with metrorrhagia due to uterine AVMs who were treated by percutaneous arterial embolization using Onyx as a single embolic agent were retrospectively included. The diagnosis of uterine AVM was suggested by pelvic ultrasound and/or magnetic resonance imaging findings and further confirmed by angiography. Clinical files and angiographic examinations were reviewed for angiographic findings, technical and clinical success, procedure complication and further pregnancies. Clinical success was defined by absence of metrorrhagia at 1 month following embolization. Sixteen arterial embolization procedures were performed. Angiographically, 6 women had high flow AVM and 6 had low flow AVM. The rate of technical and clinical success was 92% (11/12 patients). One woman with early repeat hemorrhage underwent two embolization procedures and further hysterectomy. No severe complications were observed after embolization. Three women (3/12; 25%) became pregnant following embolization including one full term pregnancy. In women with metrorrhagia due to AVM, arterial embolization with Onyx is effective and safe. Additional research is needed to confirm the possibility of future pregnancy after Onyx embolization. Copyright © 2016 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

  4. Outcomes and Complications After Endovascular Treatment of Brain Arteriovenous Malformations: A Prognostication Attempt Using Artificial Intelligence.

    PubMed

    Asadi, Hamed; Kok, Hong Kuan; Looby, Seamus; Brennan, Paul; O'Hare, Alan; Thornton, John

    2016-12-01

    To identify factors influencing outcome in brain arteriovenous malformations (BAVM) treated with endovascular embolization. We also assessed the feasibility of using machine learning techniques to prognosticate and predict outcome and compared this to conventional statistical analyses. A retrospective study of patients undergoing endovascular treatment of BAVM during a 22-year period in a national neuroscience center was performed. Clinical presentation, imaging, procedural details, complications, and outcome were recorded. The data was analyzed with artificial intelligence techniques to identify predictors of outcome and assess accuracy in predicting clinical outcome at final follow-up. One-hundred ninety-nine patients underwent treatment for BAVM with a mean follow-up duration of 63 months. The commonest clinical presentation was intracranial hemorrhage (56%). During the follow-up period, there were 51 further hemorrhagic events, comprising spontaneous hemorrhage (n = 27) and procedural related hemorrhage (n = 24). All spontaneous events occurred in previously embolized BAVMs remote from the procedure. Complications included ischemic stroke in 10%, symptomatic hemorrhage in 9.8%, and mortality rate of 4.7%. Standard regression analysis model had an accuracy of 43% in predicting final outcome (mortality), with the type of treatment complication identified as the most important predictor. The machine learning model showed superior accuracy of 97.5% in predicting outcome and identified the presence or absence of nidal fistulae as the most important factor. BAVMs can be treated successfully by endovascular techniques or combined with surgery and radiosurgery with an acceptable risk profile. Machine learning techniques can predict final outcome with greater accuracy and may help individualize treatment based on key predicting factors. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Neurorehabilitation of saccadic ocular movement in a patient with a homonymous hemianopia postgeniculate caused by an arteriovenous malformation

    PubMed Central

    Pineda-Ortíz, Mirna; Pacheco-López, Gustavo; Rubio-Osornio, Moisés; Rubio, Carmen; Valadez-Rodríguez, Juan

    2018-01-01

    Abstract Rationale: Visual therapy, which includes a restorative and compensatory approach, seems to be a viable treatment option for homonymous defects of the visual field in patients with postgeniculate injury of the visual pathway, due to occipital arteriovenous malformation (AVM). Until now, the Mexican population suffering from homonymous hemianopia did not have health services that provided any type of visual therapy for their condition. Patient concerns: A 31-year-old patient, who underwent a surgical procedure for resection of the AVM, was referred with posterior low vision on the left side. Diagnoses: The patient was diagnosed with left homonymous hemianopia. Interventions: Visual neurorehabilitation therapy (NRT), which integrated restorative and compensatory approaches, was administered for 3 hours each week. NRT included fixation, follow-up, search, peripheral vision, and reading. Outcomes: The NRT did not change visual field defects and, retinotopocally, the same campimetric defects remained. However, after training the tracking ocular movements improved to standard values on the ENG, further, the visual search became more organized. The reading reached a level without mistakes, with rhythm and goog intonation. The Beck test demostrated an improvement in depression symptoms. Regarding the daily life activities, the patient reported significant improvements. Lessons: Visual NRT can significantly improve eye movements, as well as the quality of life and independence of the patient. This integral approach could be an effective therapeutic option for homonymous defects of the visual field. PMID:29538218

  6. In vitro characteristics of endothelial cells prepared from human cerebral arteriovenous malformation lesions using a novel method.

    PubMed

    Hao, Q; Chen, X L; Ma, L; Ye, X; Wang, H; Wang, T T; Hu, Y; Zhao, Y L

    2018-03-01

    The cerebral arteriovenous malformation (cAVM) is a usual and continually unaware reason of heamorrhage and seizure. It contains of feeder arteries, drain veins and abnormal vessel nets. However, pathologic mechanisms of the development of cAVM are unknown. The purpose of this study was to explore a novel protocol to isolate, culture and passage endothelial cells (ECs) from human cAVM lesions. We developed a protocol for isolating and growing ECs from eight patients with cAVM. The tissues were microsurgically removed from cAVM lesion and were digested by 0.25% Trypsin-EDTA, and cultured in ECM medium. ECs were selected by FACS and confirmed their EC origin by immunocytochemistry of the basic expression patterns of CD31 and CD34. LDL-uptake and capillary tube formation were used to determine their functional features. The isolated cAVM-ECs exhibited contact inhibition of growth and appearance of rounded cobblestone. cAVM-ECs were CD31- and CD34-positive. In functional assays, cAVM-ECs were able to uptake LDL and form capillary tubes. cAVM-ECs from younger patients proliferated faster than that from elders, and cAVM-ECs were less stable than normal artery ECs. In addition, cAVM-ECs appeared to more easily transform into mesenchymal cells than normal artery ECs. Using the protocol, isolated cAVM-ECs are stably established, and retain their endothelial phenotypes. These cAVM-ECs may provide a biological tool to examine molecular phenotypes and mechanisms responsible for human cAVM. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Results of Surgery for Low-Grade Brain Arteriovenous Malformation Resection by Early Career Neurosurgeons: An Observational Study.

    PubMed

    Patel, Nirav J; Bervini, David; Eftekhar, Behzad; Davidson, Andrew Stewart; Walsh, Daniel C; Assaad, Nazih N; Morgan, Michael Kerin

    2018-03-28

    For sustainability of arteriovenous malformation (AVM) surgery, results from early career cerebrovascular neurosurgeons (ECCNs) must be acceptably safe. To determine whether ECCNs performance of Spetzler-Ponce Class A AVM (SPC A) resection can be acceptably safe. ECCNs completing a cerebrovascular fellowship (2004-2015) with the last author were included. Inclusion of the ECCN cases occurred if they: had a prospective database of all AVM cases since commencing independent practice; were the primary surgeon on SPC A; and had made the significant management decisions. All SPC A surgical cases from the beginning of the ECCN's independent surgical practice to a maximum of 8 yr were included. An adverse outcome was considered a complication of surgery leading to a new permanent neurological deficit with a last modified Rankin Scale score >1. A cumulative summation (Cusum) plot examined the performance of each surgery. The highest acceptable level of adverse outcomes for the Cusum was 3.3%, derived from the upper 95% confidence interval of the last author's reported series. Six ECCNs contributed 110 cases for analysis. The median number of SPC A cases operated by each ECCN was 16.5 (range 4-40). Preoperative embolization was performed in 5 (4.5%). The incidence of adverse outcomes was 1.8% (95% confidence interval: <0.01%-6.8%). At no point during the accumulated series did the combined cohort become unacceptable by the Cusum plot. ECCNs with appropriate training appointed to large-volume cerebrovascular centers can achieve results for surgery for SPC A that are not appreciably worse than those published from high-volume neurosurgeons.

  8. Interdisciplinary Clinical Management of High Grade Arteriovenous Malformations and Ruptured Flow-Related Aneurysms in the Posterior Fossa

    PubMed Central

    Mpotsaris, A.; Loehr, C.; Harati, A.; Lohmann, F.; Puchner, M.; Weber, W.

    2010-01-01

    Summary Posterior fossa arteriovenous malformations are rare entities and treatment modalities technically challenging. In recent years new therapeutic options have emerged through microsurgical and endovascular means. Based on a series of six cases we describe combined interdisciplinary treatment strategies and report the outcome in a midterm follow-up interval of 12 months. Clinical case data were collected during acute phase and follow-up including standardized angiographic control intervals during follow-up and assessment of the outcome. Treatment options included endovascular techniques as well as microsurgical techniques. All reported cases had SAH based on ruptured flow-related aneurysms in posterior fossa AVM; three out of six had multiple aneurysms. In one case we observed a de novo formation of two flow-associated distal aneurysms in an interval of ten years. Two patients were treated only endovascularly, one patient only surgically and three patients with combined methods. Five out of six patients had a good outcome (GOS 4 or 5). One died in the acute phase. Infratentorial AVMs are rare but characterized by a high risk of rupture and SAH, especially in conjunction with flow related aneurysms, which are predictors of poor outcome. The anatomic conditions of the posterior fossa may lead quickly to life-threatening complications due to mass effects. The present study indicates that treatment strategies in the acute phase should focus on flow-related aneurysms, followed by an elective AVM embolization and ectomy whenever possible. An experienced interdisciplinary team and the combination of techniques contribute to a reduction of complications and to a better outcome. PMID:21162770

  9. Clinical results of stereotactic heavy-charged-particle radiosurgery for intracranial angiographically occult vascular malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Levy, R.P.; Fabrikant, J.I.; Phillips, M.H.

    1989-12-01

    Angiographically occult vascular malformations (AOVMs) of the brain have been recognized for many years to cause neurologic morbidity and mortality. They generally become symptomatic due to intracranial hemorrhage, focal mass effect, seizures or headaches. The true incidence of AOVMs is unknown, but autopsy studies suggest that they are more common than high-flow angiographically demonstrable arteriovenous malformations (AVMs). We have developed stereotactic heavy-charged-particle Bragg peak radiosurgery for the treatment of inoperable intracranial vascular malformations, using the helium ion beams at the Lawrence Berkeley Laboratory 184-inch Synchrocyclotron and Bevatron. This report describes the protocol for patient selection, radiosurgical treatment planning method, clinicalmore » and neuroradiologic results and complications encountered, and discusses the strengths and limitations of the method. 10 refs., 1 fig.« less

  10. Planned Two-Fraction Proton Beam Stereotactic Radiosurgery for High-Risk Inoperable Cerebral Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hattangadi, Jona A.; Chapman, Paul H.; Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA

    2012-06-01

    Purpose: To evaluate patients with high-risk cerebral arteriovenous malformations (AVMs), based on eloquent brain location or large size, who underwent planned two-fraction proton stereotactic radiosurgery (PSRS). Methods and Materials: From 1991 to 2009, 59 patients with high-risk cerebral AVMs received two-fraction PSRS. Median nidus volume was 23 cc (range, 1.4-58.1 cc), 70% of cases had nidus volume {>=}14 cc, and 34% were in critical locations (brainstem, basal ganglia). Median AVM score based on age, AVM size, and location was 3.19 (range, 0.9-6.9). Many patients had prior surgery or embolization (40%) or prior PSRS (12%). The most common prescription was 16more » Gy radiobiologic equivalent (RBE) in two fractions, prescribed to the 90% isodose. Results: At a median follow-up of 56.1 months, 9 patients (15%) had total and 20 patients (34%) had partial obliteration. Patients with total obliteration received higher total dose than those with partial or no obliteration (mean dose, 17.6 vs. 15.5 Gy (RBE), p = 0.01). Median time to total obliteration was 62 months (range, 23-109 months), and 5-year actuarial rate of partial or total obliteration was 33%. Five-year actuarial rate of hemorrhage was 22% (95% confidence interval, 12.5%-36.8%) and 14% (n = 8) suffered fatal hemorrhage. Lesions with higher AVM scores were more likely to hemorrhage (p = 0.024) and less responsive to radiation (p = 0.026). The most common complication was Grade 1 headache acutely (14%) and long term (12%). One patient developed a Grade 2 generalized seizure disorder, and two had mild neurologic deficits. Conclusions: High-risk AVMs can be safely treated with two-fraction PSRS, although total obliteration rate is low and patients remain at risk for future hemorrhage. Future studies should include higher doses or a multistaged PSRS approach for lesions more resistant to obliteration with radiation.« less

  11. Hippocrates in the pseudo-Galenic Introduction: or how was medicine taught in Roman times?

    PubMed

    Petit, Caroline

    2010-01-01

    The Pseudo-Galenic Introduction (Introductio Sive medicus, 14.674-797 K.), a medical handbook of the Roman period, witnesses the importance of Hippocrates in medical teaching at the time. Numerous quotations, allusions and reminiscences from the Hippocratic Corpus illustrate Hippocrates' authority for Pseudo-Galen. In the light of the first critical edition of the text (C. Petit, Les Belles Lettres, Paris, 2009), this article discusses the function of Hippocrates, and the various reminiscences of the Hippocratic Corpus, in order to assess Pseudo-Galen's quotation technique and, ultimately, his reliability as a source for the history of medicine.

  12. Pulmonary Arteriovenous Malformations Embolized Using a Micro Vascular Plug System: Technical Note on a Preliminary Experience

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Boatta, Emanuele, E-mail: emanuele.boatta@yahoo.it; Jahn, Christine, E-mail: christine.jahn@chru-strasbourg.fr; Canuet, Matthieu, E-mail: matthieu.canuet@chru-strasbourg.fr

    AIMTo report our preliminary experience using a Micro Vascular Plug (MVP) deployed through a 2.8Fr micro-catheter for the treatment of pulmonary arteriovenous malformations (PAVMs) in a cohort of patients affected by Hereditary Haemorrhagic Telangiectasia (HHT).Materials and MethodsFour consecutive female patients (mean age 38.0 years; range 25–55 years) with PAVMs diagnosed on echocardiogram/bubble test and contrast-enhanced CT (CECT) underwent MVP embolization. One patient was symptomatic with recent transient ischaemic attack. Follow-up was undertaken at 1-month post-procedure with CECT to assess PAVMs permeability and MVP positioning and at 1-, 6-, and 12-month post-procedure, with echocardiography/bubble test and standard neurological history, to confirm absence ofmore » right-to-left shunts and recurrent symptoms.ResultsEight PAVMs were treated in 4 patients over 5 interventional sessions (mean 1.6 PAVMs per session). All PAVMs were simple, with mean feeding artery diameter of 4.25 mm. Eight 6.5 mm MVPs were deployed in total (one per lesion). Technical success was 100%. Mean procedural time and patient dose per session were 70 min (range 40–70 min) and 53418 mGy.cm{sup 2} (range 6113–101628 mGy.cm{sup 2}), respectively. No signs of reperfusion neither of MPV migration were noted at 1-month CECT follow-up. At early follow-up (mean 3.75 months; range 1–12 months), clinical success was 100% with no evidence of recurrent right-to-left shunt, and no neurological symptoms. No immediate or late complications were observed.ConclusionsMVP embolization of PAVMs appears technically feasible, safe, and effective at early follow-up. Further prospective studies are required to confirm long-term safety and efficacy of this promising technique.« less

  13. Benefit of cone-beam computed tomography angiography in acute management of angiographically undetectable ruptured arteriovenous malformations.

    PubMed

    Rahal, Jason P; Malek, Adel M

    2013-10-01

    Ruptured arteriovenous malformations (AVMs) are a frequent cause of intracerebral hemorrhage (ICH). In some cases, compression from the associated hematoma in the acute setting can partially or completely occlude an AVM, making it invisible on conventional angiography techniques. The authors report on the successful use of cone-beam CT angiography (CBCT-A) to precisely identify the underlying angioarchitecture of ruptured AVMs that are not visible on conventional angiography. Three patients presented with ICH for which they underwent examination with CBCT-A in addition to digital subtraction angiography and other imaging modalities, including MR angiography and CT angiography. All patients underwent surgical evacuation due to mass effect from the hematoma. Clinical history, imaging studies, and surgical records were reviewed. Hematoma volumes were calculated. In all 3 cases, CBCT-A demonstrated detailed anatomy of an AVM where no lesion or just a suggestion of a draining vein had been seen with other imaging modalities. Magnetic resonance imaging demonstrated enhancement in 1 patient; CT angiography demonstrated a draining vein in 1 patient; 2D digital subtraction angiography and 3D rotational angiography demonstrated a suggestion of a draining vein in 2 cases and no finding in the third. In the 2 patients in whom CBCT-A was performed prior to surgery, the demonstrated AVM was successfully resected without evidence of a residual lesion. In the third patient, CBCT-A allowed precise targeting of the AVM nidus using Gamma Knife radiosurgery. Cone-beam CT angiography should be considered in the evaluation and subsequent treatment of ICH due to ruptured AVMs. In cases in which the associated hematoma compresses the AVM nidus, CBCT-A can have higher sensitivity and anatomical accuracy than traditional angiographic modalities, including digital subtraction angiography.

  14. Balloon-augmented Onyx embolization of cerebral arteriovenous malformations using a dual-lumen balloon: a multicenter experience.

    PubMed

    Spiotta, Alejandro M; James, Robert F; Lowe, Stephen R; Vargas, Jan; Turk, Aquilla S; Chaudry, M Imran; Bhalla, Tarun; Janjua, Rashid M; Delaney, John J; Quintero-Wolfe, Stacey; Turner, Raymond D

    2015-10-01

    Conventional Onyx embolization of cerebral arteriovenous malformations (AVMs) requires lengthy procedure and fluoroscopy times to form an adequate 'proximal plug' which allows forward nidal penetration while preventing reflux and non-targeted embolization. We review our experience with balloon-augmented Onyx embolization of cerebral AVMs using a dual-lumen balloon catheter technique designed to minimize these challenges. Retrospectively acquired data for all balloon-augmented cerebral AVM embolizations performed between 2011 and 2014 were obtained from four tertiary care centers. For each procedure, at least one Scepter C balloon catheter was advanced into the AVM arterial pedicle of interest and Onyx embolization was performed through the inner lumen after balloon inflation via the outer lumen. Twenty patients underwent embolization with the balloon-augmented technique over 24 discreet treatment episodes. There were 37 total arterial pedicles embolized with the balloon-augmented technique, a mean of 1.9 per patient (range 1-5). The treated AVMs were heterogeneous in their location and size (mean 3.3±1.6 cm). Mean fluoroscopy time for each procedure was 48±26 min (28 min per embolized pedicle). Two Scepter C balloon catheter-related complications (8.3% of embolization sessions, 5.4% of pedicles embolized) were observed: an intraprocedural rupture of a feeding pedicle and fracture and retention of a catheter fragment. This multicenter experience represents the largest reported series of balloon-augmented Onyx embolization of cerebral AVMs. The technique appears safe and effective in the treatment of AVMs, allowing more efficient and controlled injection of Onyx with a decreased risk of reflux and decreased fluoroscopy times. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  15. Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia.

    PubMed

    Tau, Noam; Atar, Eliyahu; Mei-Zahav, Meir; Bachar, Gil N; Dagan, Tamir; Birk, Einat; Bruckheimer, Elchanan

    2016-08-01

    Coil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia. The study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004-2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both), and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher's exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p < 0.05. 16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4-18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413). The use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.

  16. Does the type and size of Amplatzer vascular plug affect the occlusion time of pulmonary arteriovenous malformations?

    PubMed Central

    Abdel-Aal, Ahmed Kamel; Massoud, Moustafa Omar; Elantably, Dina Mahmoud

    2017-01-01

    PURPOSE Occlusion time (OT) is an important factor in the treatment of pulmonary arteriovenous malformations (PAVMs) since it can lead to serious complications. The purpose of our study is to calculate the OT of Amplatzer vascular plug (AVP, St Jude Medical), and correlate it to the type of the device used (AVP or AVP 2) and the percent of device oversizing. Technical success rates and complications were also recorded. METHODS We retrospectively studied a total of 19 patients with 47 PAVMs who received percutaneous transcatheter embolization therapy using either AVP or AVP 2. We recorded the location, type, feeding artery diameter, AVP device used, and OT of each PAVM. We correlated the percent of device oversizing and the type of AVP with the OT. We also studied the rate of persistence of PAVM for both devices. RESULTS Forty-six (98%) of the PAVMs were simple. Device diameters ranged from 4.0–16.0 mm with device oversizing ranging between 14% and 120%. There was a statistically significant difference in the OT of AVP and AVP 2 (3 min 54 s vs. 5 min 30 s, P = 0.030). There was a weak positive correlation between OT and device oversizing for AVP (r=0.246, P = 0.324) and AVP 2 (r=0.261, P = 0.240). No major complications were identified. Immediate technical success rate was 100%. CONCLUSION The use of AVP 2, and increase in device oversizing were not associated with reduction in the OT of PAVMs. There was no reported difference in safety between the two devices, and no major complications were noted. PMID:27856403

  17. The Retrograde Transvenous Push-Through Method: A Novel Treatment of Peripheral Arteriovenous Malformations with Dominant Venous Outflow

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Wohlgemuth, Walter A., E-mail: walter.wohlgemuth@ukr.de; Müller-Wille, René, E-mail: Rene.Mueller-Wille@ukr.de; Teusch, Veronika I., E-mail: Veronika.Teusch@gmx.de

    2015-06-15

    PurposeTo evaluate the efficacy and safety of a novel retrograde transvenous embolization technique of peripheral arteriovenous malformations (AVMs) using Onyx.Materials and MethodsWe conducted a retrospective analysis of all patients who underwent transvenous retrograde Onyx embolization of peripheral AVMs with dominant venous outflow over a 29-month period. The embolization is aimed at retrograde filling of the nidus after building a solid plug in the dominant venous outflow (push-through). Classification, clinical signs, technical aspects, clinical and technical success rates, and complications were recorded. Short-term outcome was assessed.Results11 Symptomatic patients (8 female; mean age 31.4 years) were treated at our Vascular Anomalies Center withmore » this method between January 2012 and May 2014. The AVMs were located on the upper extremity (n = 3), pelvis (n = 2), buttock (n = 2), and lower extremity (n = 4). Retrograde embolization was successfully carried out after preparatory transarterial-flow reduction in eight cases (73 %) and venous-flow reduction with Amplatzer Vascular Plugs in four cases (36 %). Complete devascularization (n = 10; 91 %) or 95 % devascularization (n = 1; 9 %) led to complete resolution (n = 8; 73 %) or improvement of clinical symptoms (n = 3; 27 %). One minor complication occurred (pain and swelling). During a mean follow-up time of 8 months, one clinically asymptomatic recurrence of AVM was detected.ConclusionInitial results suggest that retrograde transvenous Onyx embolization of peripheral AVMs with dominant venous outflow is a safe and effective novel technique with a low complication rate.« less

  18. Curative embolization of pediatric intracranial arteriovenous malformations using Onyx: the role of new embolization techniques on patient outcomes.

    PubMed

    de Castro-Afonso, L H; Nakiri, G S; Oliveira, R S; Santos, M V; Santos, A C Dos; Machado, H R; Abud, D G

    2016-06-01

    Intracranial arteriovenous malformations (AVMs) are the most frequent cause of hemorrhagic strokes in the pediatric population. The study aim was to retrospectively assess the safety and efficacy of Onyx embolization with the intention to cure AVMs in a pediatric population. A retrospective analysis of all patients (<18 years) who underwent endovascular embolization using Onyx at our institution was conducted. The primary endpoint was the composite complete angiographic occlusion of AVM immediately after the last embolization session that had no procedure-related complication requiring emergency surgery. Secondary endpoints were angiographic occlusion rates, procedure-related complications, and clinical outcomes after treatment and at the 6-month follow-up Twenty-three patients (mean age, 11.7 years) underwent a total of 45 embolization sessions. The median Spetzler-Martin grade was 3 (range 1 to 4). The primary endpoint was achieved in 19 patients (82.6 %). Complete angiographic occlusion of the AVM was obtained in 21 patients (91.3 %) immediately after embolization and at the 6-month follow-up. Embolization-related complications were observed in three patients (13 %). None of the complications resulted in permanent functional disability or death. In two patients (8.7 %), the AVM could not be completely occluded by embolization alone and the patients were referred to radiosurgery and microsurgery, respectively. Onyx embolization of AVM in pediatric patients with the intention to cure resulted in high occlusion rates without increasing neurological disability or death. The development of new embolization techniques and devices seems to improve the safety of Onyx embolization.

  19. Hydrocephalus in a patient with an unruptured pial arteriovenous fistula: hydrodynamic considerations, endovascular treatment, and clinical course.

    PubMed

    Morales-Gómez, Jesús A; Garza-Oyervides, Vicente V; Arenas-Ruiz, José A; Mercado-Flores, Mariana; Elizondo-Riojas, C Guillermo; Boop, Frederick A; de León, Ángel Martínez-Ponce

    2017-03-01

    Intracranial pial arteriovenous fistulas, also known as nongalenic fistulas, are rare vascular malformations affecting predominantly the pediatric population. Hydrocephalus is an unusual presentation in which the exact pathophysiology is not fully understood. The aim of treatment in these cases is occlusion of the fistula prior to considering ventricular shunting. Here, the authors describe the hydrodynamic considerations of the paravascular pathway and the resolution of hydrocephalus with endovascular treatment of the fistula.

  20. The pancreas from Aristotle to Galen.

    PubMed

    Tsuchiya, Ryoichi; Kuroki, Tamotsu; Eguchi, Susumu

    2015-01-01

    The first description of the pancreas in literature is found in Aristotle's Historia Animalium, but it is modified by "so-called". Therefore, the origin is pursued more extensively. The Greek-English Lexicon recommends three treatises as a possible original source. These three and Galen's other papers are investigated. In 2005, Sachs et al. suggested an origin of the pancreas might have derived from the intestinal divination using the avian pancreas. This report is evaluated. The avian pancreas which is the intraperitoneal organ, might have been well known by the intestinal divination, and people have called the organ pankreas or kallikreas. Anatomical dissection on human body was not accepted before the Aristotle's time. "So-called pancreas" in Historia must have been interpolated by Theophrastus. He was the most faithful and reliable disciple of Aristotle and succeeded the Aristotle's school. He and Macedonian ruler of Egypt Ptolemy I had known each other and there had been a strong link between them. The contemporary Herophilus performed many public dissections on both human and animal bodies in Alexandria. He named the various parts of the human body and designated the beginning intestine as duodenum. Yet in his extant works, the pancreas is not found. It is surmised that Herophilus may be the first to recognize the human pancreas, which is fixed with retroperitoneal tissue, and he named it "so-called pancreas". Theophrastus might have interpolated Herophilus' designation in Historia Animalium. Galen also uses "so-called pancreas" to designate the human pancreas. Galen's descriptions, that is, "Nature created 'so-called pancreas 'and spread it beneath all vessels" are not generally acceptable but propose the very rare portal vein anomalies. Since the early years of the 20th century, cases with a preduodenal portal vein or a prepancreatic portal vein have been reported. Although the incidence is very rare, its surgical importance is emphasized. Copyright © 2014

  1. The "focus on aneurysm" principle: Classification and surgical principles of management of concurrent arterial aneurysm with arteriovenous malformation causing intracranial hemorrhage.

    PubMed

    Jha, Vikas; Behari, Sanjay; Jaiswal, Awadhesh K; Bhaisora, Kamlesh Singh; Shende, Yogesh P; Phadke, Rajendra V

    2016-01-01

    Concurrent arterial aneurysms (AAs) occurring in 2.7-16.7% patients harboring an arteriovenous malformation (AVM) aggravate the risk of intracranial hemorrhage. We evaluate the variations of aneurysms simultaneously coexisting with AVMs. A classification-based management strategy and an abbreviated nomenclature that describes their radiological features is also proposed. Tertiary care academic institute. Test of significance applied to determine the factors causing rebleeding in the groups of patients with concurrent AVM and aneurysm and those with only AVMs. Sixteen patients (5 with subarachnoid hemorrhage and 11 with intracerebral/intraventricular hemorrhage; 10 with low flow [LF] and 6 with high flow [HF] AVMs) underwent radiological assessment of Spetzler Martin (SM) grading and flow status of AA + AVM. Their modified Rankin's score (mRS) at admission was compared with their follow-up (F/U) score. Pre-operative mRS was 0 in 5, 2 in 6, 3 in 1, 4 in 3 and 5 in 1; and, SM grade I in 5, II in 3, III in 3, IV in 4 and V in 1 patients, respectively. AA associated AVMs were classified as: (I) Flow-related proximal (n = 2); (II) flow-related distal (n = 3); (III) intranidal (n = 5); (IV) extra-intranidal (n = 2); (V) remote major ipsilateral (n = 1); (VI) remote major contralateral (n = 1); (VII) deep perforator related (n = 1); (VIII) superficial (n = 1); and (IX) distal (n = 0). Their treatment strategy included: Flow related AA, SM I-III LF AVM: aneurysm clipping with AVM excision; nidal-extranidal AA, SM I-III LF AVM: Excision or embolization of both AA + AVM; nidal-extranidal and perforator-related AA, SM IV-V HF AVM: Only endovascular embolization or radiosurgery. Surgical decision-making for remote AA took into account their ipsilateral/contralateral filling status and vessel dominance; and, for AA associated with SM III HF AVM, it varied in each patient based on diffuseness of AVM nidus, flow across arteriovenous fistula and eloquence of cortex. Follow up (F

  2. Radiosurgery reduces plasma levels of angiogenic factors in brain arteriovenous malformation patients.

    PubMed

    Xu, Ming; Liu, Xiaoxia; Mei, Guanghai; Zhang, Junjie; Wang, Weixing; Xu, Hongzhi

    2018-05-09

    Aberrant expression of angiogenic factors has been anecdotally documented in brain arteriovenous malformation (AVM) nidus vessels; however, no data is available on the effect of radiosurgery on the levels of angiogenic factors in AVM patients. We sought to determine the plasma contents of VEGF, TGF-β, Ang-2 and bFGF in 28 brain AVM patients at baseline and post radiosurgery and further analyzed the relationship between plasma contents of these angiogenic factors with clinicopathologic variables of these patients. We enrolled brain AVM patients who underwent Cyberknife radiosurgery at our hospital between January 2014 and December 2015. Brain AVM was confirmed by cerebral angiography and radiosurgery was performed with Cyberknife irradiation. Plasma contents of VEGF, TGF-β, Ang-2 and bFGF were analyzed using commercially available enzyme-linked immunoassay (ELISA) kits. The baseline plasma VEGF content was 222.63 pg/mL (range 43.25-431.25 pg/mL). At three months post surgery, there was a significant -34.29% decline in plasma VEGF content versus baseline (P = 0.000). Furthermore, the median baseline plasma VEGF levels were higher in brain AVM with a nidus volume ≥ 10 cm 3 ) than those with a nidus volume < 10 cm 3 [median(IQR) 293.5 (186.5,359.25) vs. 202 (59.75, 270.75) pg/mL, P = 0.057]. The baseline plasma TGF-β content was 556.17 pg/mL (range 44.44-1486.11 pg/mL) and there was a significant -27.47% decline in plasma TGF-β content at 3 months post radiosurgery versus baseline (P = 0.015). Moreover, the baseline plasma ANG-2 content was 214.27 pg/mL (range 77.14-453.76 pg/mL). There was an immediate and significant -12.47% decline in plasma ANG-2 content post surgery versus baseline (P = 0.002). At three months post surgery, the plasma ANG-2 content still remained significantly depressed versus baseline (P = 0.002). In addition, the baseline plasma bFGF content was 9.17 pg/mL (range 3.67-36.78 pg/mL). No

  3. Retrospective study of long-term outcome after brain arteriovenous malformation rupture: the RAP score.

    PubMed

    Shotar, Eimad; Debarre, Matthieu; Sourour, Nader-Antoine; Di Maria, Federico; Gabrieli, Joseph; Nouet, Aurélien; Chiras, Jacques; Degos, Vincent; Clarençon, Frédéric

    2018-01-01

    OBJECTIVE The authors aimed to design a score for stratifying patients with brain arteriovenous malformation (BAVM) rupture, based on the likelihood of a poor long-term neurological outcome. METHODS The records of consecutive patients with BAVM hemorrhagic events who had been admitted over a period of 11 years were retrospectively reviewed. Independent predictors of a poor long-term outcome (modified Rankin Scale score ≥ 3) beyond 1 year after admission were identified. A risk stratification scale was developed and compared with the intracranial hemorrhage (ICH) score to predict poor outcome and inpatient mortality. RESULTS One hundred thirty-five patients with 139 independent hemorrhagic events related to BAVM rupture were included in this analysis. Multivariate logistic regression followed by stepwise analysis showed that consciousness level according to the Glasgow Coma Scale (OR 6.5, 95% CI 3.1-13.7, p < 10 -3 ), hematoma volume (OR 1.8, 95% CI 1.2-2.8, p = 0.005), and intraventricular hemorrhage (OR 7.5, 95% CI 2.66-21, p < 10 -3 ) were independently associated with a poor outcome. A 12-point scale for ruptured BAVM prognostication was constructed combining these 3 factors. The score obtained using this new scale, the ruptured AVM prognostic (RAP) score, was a stronger predictor of a poor long-term outcome (area under the receiver operating characteristic curve [AUC] 0.87, 95% CI 0.8-0.92, p = 0.009) and inpatient mortality (AUC 0.91, 95% CI 0.85-0.95, p = 0.006) than the ICH score. For a RAP score ≥ 6, sensitivity and specificity for predicting poor outcome were 76.8% (95% CI 63.6-87) and 90.8% (95% CI 81.9-96.2), respectively. CONCLUSIONS The authors propose a new admission score, the RAP score, dedicated to stratifying the risk of poor long-term outcome after BAVM rupture. This easy-to-use scoring system may help to improve communication between health care providers and consistency in clinical research. Only external prospective cohorts and population

  4. The correlation between hematoma volume and outcome in ruptured posterior fossa arteriovenous malformations indicates the importance of surgical evacuation of hematomas.

    PubMed

    Yilmaz, Adem; Musluman, Ahmet Murat; Kanat, Ayhan; Cavusoglu, Halit; Terzi, Yuksel; Aydin, Yunus

    2011-01-01

    The correlation between hematoma volume and outcome in ruptured arteriovenous malformations (AVM) with accompanying posterior fossa hematoma was retrospectively evaluated. Microsurgery operations were performed on 127 patients with intracranial AVM between January 1998 and January 2009 at our clinic. Fifteen (11.8%) patients were identified as suffering from posterior fossa AVM, and twelve of these patients presented with a cerebellar hematoma. All patients were clinically evaluated according to the following criteria: modified Rankin Scale (mRS) prior to surgery, Spetzler-Martin grade (SMG) of the AVMs, hematoma volume prior to surgery, and mRS following surgery. Postoperative mRS scores were significantly lower than preoperative scores (p=0.0001). Postoperative outcomes were concordant with the SMG of the AVMs (r=0.67, p=0.033), hematoma volume (r=0.537, p=0.072) and preoperative mRS scores (r=0.764, p=0.004). These analyses show that the postoperative mRS score is strongly correlated with a preoperative mRS score, hematoma volume and SMG. Posterior fossa AVMs present an increased risk for hemorrhage and for increased morbidity and mortality. Cases with hematoma should be operated on an urgent basis. We conclude that hematoma volume is a factor that impacts postoperative results and prognosis. SMG and preoperative mRS scores were also correlated with outcome.

  5. Rescue N-butyl-2 cyanoacrylate embolectomy using a Solitaire FR device after venous glue migration during arteriovenous malformation embolization: technical note.

    PubMed

    Fahed, Robert; Clarençon, Frédéric; Sourour, Nader-Antoine; Chauvet, Dorian; Le Jean, Lise; Chiras, Jacques; Di Maria, Federico

    2016-07-01

    One of the procedural risks in arteriovenous malformation (AVM) embolization is possible migration of the embolic agent into the venous drainage with an incomplete nidus occlusion, which may lead to severe hemorrhagic complications. This report presents the case of a 29-year-old man who presented with a deep intraparenchymal hematoma on the left side secondary to the spontaneous rupture of a claustral AVM. Upon resorption of the hematoma, the patient underwent an initial therapeutic session of N-butyl-2 cyanoacrylate endovascular embolization, with the purpose of reducing the AVM volume and flow before performing Gamma Knife radiosurgery. After glue injection into one of the arterial feeders, the control angiography showed a partial migration of the glue cast into the straight sinus, with most of the nidus still visible. Because of the bleeding risk due to possible venous hypertension, it was decided to try to retrieve the glue from the vein by using a stent retriever via jugular access. This maneuver allowed a nearly complete removal of the glue cast, thereby restoring normal venous flow drainage. The patient showed no clinical worsening after the procedure. To the authors' knowledge, this is the first report of the use of the Solitaire FR device as a rescue glue retriever. This method should be considered by physicians in cases of unintended glue migration into the venous circulation during AVM embolization.

  6. Amplatzer Vascular Plugs Versus Coils for Embolization of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Tau, Noam, E-mail: taunoam@gmail.com; Atar, Eliyahu; Mei-Zahav, Meir

    PurposeCoil embolization of pulmonary arteriovenous malformations (PAVMs) has a high re-canalization/re-perfusion rate. Embolization with Amplatzer plugs has been previously described, but the long-term efficacy is not established. This study reports the experience of a referral medical center with the use of coils and Amplatzer plugs for treating PAVMs in patients with hereditary hemorrhagic telangiectasia.MethodsThe study was approved by the Institutional Review Board with waiver of informed consent. The cohort included all patients who underwent PAVM embolization in 2004–2014 for whom follow-up imaging scans were available. The medical files were retrospectively reviewed for background data, embolization method (coils, Amplatzer plugs, both),more » and complications. Re-canalization of treated PAVMs was assessed from intrapulmonary angiograms (following percutaneous procedures) or computed tomography angiograms. Fisher’s exact test and Pearson Chi-squared test or t test were used for statistical analysis, with significance at p < 0.05.Results16 patients met the study criteria. Imaging scans were available for 63 of the total 110 PAVMs treated in 41 procedures. Coils were used for embolization in 37 PAVMs, Amplatzer plugs in 21, and both in five. Median follow-up time was 7.7 years (range 1.4–18.9). Re-canalization was detected in seven vessels, all treated with coils; there were no cases of re-canalization in plug-occluded vessels (p = 0.0413).ConclusionThe use of Amplatzer plugs for the embolization of PAVMs in patients with hemorrhagic telangiectasia is associated with a significantly lower rate of re-canalization of feeding vessels than coils. Long-term prospective studies are required to confirm these findings.« less

  7. Targeted Embolization of Aneurysms Associated With Brain Arteriovenous Malformations at High Risk for Surgical Resection: A Case-Control Study.

    PubMed

    Alexander, Matthew D; Hippe, Daniel S; Cooke, Daniel L; Hallam, Danial K; Hetts, Steven W; Kim, Helen; Lawton, Michael T; Sekhar, Laligam N; Kim, Louis J; Ghodke, Basavaraj V

    2018-03-01

    High-risk components of brain arteriovenous malformations (BAVMs) can be targeted to reduce the risk of lesion rupture. To evaluate targeted embolization of aneurysms against other means of treatment with a case-control analysis; we previously investigated this approach associated with BAVMs. Retrospective analysis of patients with BAVMs was performed, identifying patients treated with intention to occlude only an aneurysm associated with a BAVM. For each targeted aneurysm embolization (TAE) patient identified, 4 control patients were randomly selected, controlling for rupture status, age, and Spetzler-Martin plus Lawton-Young supplemental score. Analysis was performed to compare rates of adverse events (hemorrhage, new seizure, and death) between the 2 groups. Thirty-two patients met inclusion criteria, and 128 control patients were identified, out of 1103 patients treated during the study period. Thirty-four adverse events occurred (15 ruptures, 15 new seizures, and 11 deaths) during the follow-up period (mean 1157 d for the TAE cohort and 1036 d for the non-TAE cohort). Statistically lower associations were noted for the TAE group for any adverse event (hazard ratio 0.28, P = .037) and the composite outcome of hemorrhage or new seizure (hazard ratio 0.20, P = .029). For BAVMs at high risk for surgical resection, TAE can be performed safely and effectively. Patients treated with TAE had better outcomes than matched patients undergoing other combinations of treatment. TAE can be considered for BAVMs with high operative risk prior to radiosurgery or when no other treatment options are available. Copyright © 2017 by the Congress of Neurological Surgeons

  8. In vivo imaging of endothelial cell adhesion molecule expression after radiosurgery in an animal model of arteriovenous malformation.

    PubMed

    Raoufi-Rad, Newsha; McRobb, Lucinda S; Lee, Vivienne S; Bervini, David; Grace, Michael; Ukath, Jaysree; Mchattan, Joshua; Sreenivasan, Varun K A; Duong, T T Hong; Zhao, Zhenjun; Stoodley, Marcus A

    2017-01-01

    Focussed radiosurgery may provide a means of inducing molecular changes on the luminal surface of diseased endothelium to allow targeted delivery of novel therapeutic compounds. We investigated the potential of ionizing radiation to induce surface expression of intercellular adhesion molecule 1 (ICAM-1) and vascular cell adhesion molecule 1 (VCAM-1) on endothelial cells (EC) in vitro and in vivo, to assess their suitability as vascular targets in irradiated arteriovenous malformations (AVMs). Cultured brain microvascular EC were irradiated by linear accelerator at single doses of 0, 5, 15 or 25 Gy and expression of ICAM-1 and VCAM-1 measured by qRT-PCR, Western, ELISA and immunocytochemistry. In vivo, near-infrared (NIR) fluorescence optical imaging using Xenolight 750-conjugated ICAM-1 or VCAM-1 antibodies examined luminal biodistribution over 84 days in a rat AVM model after Gamma Knife surgery at a single 15 Gy dose. ICAM-1 and VCAM-1 were minimally expressed on untreated EC in vitro. Doses of 15 and 25 Gy stimulated expression equally; 5 Gy was not different from the unirradiated. In vivo, normal vessels did not bind or retain the fluorescent probes, however binding was significant in AVM vessels. No additive increases in probe binding were found in response to radiosurgery at a dose of 15 Gy. In summary, radiation induces adhesion molecule expression in vitro but elevated baseline levels in AVM vessels precludes further induction in vivo. These molecules may be suitable targets in irradiated vessels without hemodynamic derangement, but not AVMs. These findings demonstrate the importance of using flow-modulated, pre-clinical animal models for validating candidate proteins for vascular targeting in irradiated AVMs.

  9. [The doctor and the consul. Relations between Galen and Flavius Boethos under Marcus Aurelius].

    PubMed

    Gourevitch, Danielle

    2012-01-01

    The author describes the rich relationship between young Galen, just arrived in Rome from his native Minor Asia, and Flavius Boethos a consul suffectus who was to become the governor of Syria, and asked him to cure his wife and then their son, Cyrillos. Although very famous, Galen who never became a Roman citizen, needed such a friend and social mentor.

  10. Endovascular treatment of brain arteriovenous malformations with prolonged intranidal Onyx injection technique: long-term results in 350 consecutive patients with completed endovascular treatment course.

    PubMed

    Saatci, Isil; Geyik, Serdar; Yavuz, Kivilcim; Cekirge, H Saruhan

    2011-07-01

    The purpose of this study was to present the authors' clinical experience and long-term angiographic and clinical follow-up results in 350 patients with brain arteriovenous malformations (AVMs) treated using prolonged intranidal Onyx injection with a very slow "staged" reflux technique described by the authors. Three hundred and fifty consecutive patients with brain AVMs treated using Onyx between 1999 and 2008 and in whom definitive status for endovascular treatment was reached are presented. There were 206 (59%) male and 144 (41%) female patients, with a mean age of 34 years. There were 607 endovascular sessions performed. Onyx was the only agent used for intranidal injections in all patients, but in 42 patients high-concentration N-butyl cyanoacrylate glue was used adjunctively to close high-flow direct arteriovenous intra- or perinidal fistulas, or when a feeding vessel or nidus perforation and/or dissection occurred. Angiographically confirmed obliteration was achieved in 179 patients (51%) with only endovascular treatment; 1 patient died due to intracranial hemorrhage after the treatment. Twenty-two patients underwent resection, and 136 patients were sent to radiosurgery after endovascular treatment. In 4 patients embolization therapy was discontinued, and 5 additional patients refused the suggested complementary surgery. In all 178 surviving patients who had angiographically confirmed AVM obliteration by embolization alone, 1-8 years of control angiography (mean 47 months) confirmed stable obliteration, except for 2 patients in whom a very small recruitment was noted in the 1st year on control angiography studies, despite initial apparent total obliteration (recanalization rate 1.1%). In the entire series, 5 patients died; the mortality rate was 1.4%. The permanent morbidity rate was 7.1%. With the prolonged intranidal injection technique described herein, Onyx allows the practitioner to achieve higher rates of anatomical cures compared with the cure rates

  11. The galenic plague: a breakdown of the imperial pathocoenosis. Pathocoenosis and longue durée.

    PubMed

    Gourevitch, Danielle

    2005-01-01

    Is 'pathocoenosis', a notion conceived and a word coined by Mirko Grmek (1969), useful as far as ancient history is concerned? The author is interested in Galenic pathocoenosis, that of doctor Galen and his Emperor Marcus Aurelius (IInd cent. A.D.), when a new 'pestilence' or 'plague' (smallpox?) devastated the whole empire, from Mesopotamia to the Danube at least.

  12. Dosimetric effects of Onyx embolization on Gamma Knife arteriovenous malformation dose distributions.

    PubMed

    Schlesinger, David J; Nordström, Håkan; Lundin, Anders; Xu, Zhiyuan; Sheehan, Jason P

    2016-12-01

    OBJECTIVE Patients with arteriovenous malformations (AVMs) treated with Gamma Knife radiosurgery (GKRS) subsequent to embolization suffer from elevated local failure rates and differences in adverse radiation effects. Onyx is a common embolic material for AVMs. Onyx is formulated with tantalum, a high atomic number (Z = 73) element that has been investigated as a source of dosimetric uncertainty contributing to the less favorable clinical results. However, prior studies have not modeled the complicated anatomical and beam geometries characteristic of GKRS. This study investigated the magnitude of dose perturbation that can occur due to Onyx embolization using clinically realistic anatomical and Gamma Knife beam models. METHODS Leksell GammaPlan (LGP) was used to segment the AVM nidus and areas of Onyx from postcontrast stereotactic MRI for 7 patients treated with GKRS postembolization. The resulting contours, skull surface, and clinically selected dose distributions were exported from LGP in DICOM-RT (Digital Imaging and Communications in Medicine-radiotherapy) format. Isocenter locations and dwell times were recorded from the LGP database. Contours were converted into 3D mesh representations using commercial and in-house mesh-editing software. The resulting data were imported into a Monte Carlo (MC) dose calculation engine (Pegasos, Elekta Instruments AB) with a beam geometry for the Gamma Knife Perfexion. The MC-predicted dose distributions were calculated with Onyx assigned manufacturer-reported physical constants (MC-Onyx), and then compared with corresponding distributions in which Onyx was reassigned constants for water (MC-water). Differences in dose metrics were determined, including minimum, maximum, and mean dose to the AVM nidus; selectivity index; and target coverage. Combined differences in dose magnitude and distance to agreement were calculated as 3D Gamma analysis passing rates using tolerance criteria of 0.5%/0.5 mm, 1.0%/1.0 mm, and 3.0%/3.0 mm

  13. Dosimetric measurements of an n-butyl cyanoacrylate embolization material for arteriovenous malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Labby, Zacariah E., E-mail: zelabby@humonc.wisc.edu; Chaudhary, Neeraj; Gemmete, Joseph J.

    2015-04-15

    Purpose: The therapeutic regimen for cranial arteriovenous malformations often involves both stereotactic radiosurgery and endovascular embolization. Embolization agents may contain tantalum or other contrast agents to assist the neurointerventionalists, leading to concerns regarding the dosimetric effects of these agents. This study investigated dosimetric properties of n-butyl cyanoacrylate (n-BCA) plus lipiodol with and without tantalum powder. Methods: The embolization agents were provided cured from the manufacturer with and without added tantalum. Attenuation measurements were made for the samples and compared to the attenuation of a solid water substitute using a 6 MV photon beam. Effective linear attenuation coefficients (ELAC) were derivedmore » from attenuation measurements made using a portal imager and derived sample thickness maps projected in an identical geometry. Probable dosimetric errors for calculations in which the embolized regions are overridden with the properties of water were calculated using the ELAC values. Interface effects were investigated using a parallel plate ion chamber placed at set distances below fixed samples. Finally, Hounsfield units (HU) were measured using a stereotactic radiosurgery CT protocol, and more appropriate HU values were derived from the ELAC results and the CT scanner’s HU calibration curve. Results: The ELAC was 0.0516 ± 0.0063 cm{sup −1} and 0.0580 ± 0.0091 cm{sup −1} for n-BCA without and with tantalum, respectively, compared to 0.0487 ± 0.0009 cm{sup −1} for the water substitute. Dose calculations with the embolized region set to be water equivalent in the treatment planning system would result in errors of −0.29% and −0.93% per cm thickness of n-BCA without and with tantalum, respectively. Interface effects compared to water were small in magnitude and limited in distance for both embolization materials. CT values at 120 kVp were 2082 and 2358 HU for n-BCA without and with tantalum

  14. Testing the radiosurgery-based arteriovenous malformation score and the modified Spetzler-Martin grading system to predict radiosurgical outcome.

    PubMed

    Andrade-Souza, Yuri M; Zadeh, Gelareh; Ramani, Meera; Scora, Daryl; Tsao, May N; Schwartz, Michael L

    2005-10-01

    The aim of this study was to validate the radiosurgery-based arteriovenous malformation (AVM) score and the modified Spetzler-Martin grading system to predict radiosurgical outcome. One hundred thirty-six patients with brain AVMs were randomly selected. These patients had undergone a linear accelerator radiosurgical procedure at a single center between 1989 and 2000. Patients were divided into four groups according to an AVM score, which was calculated from the lesion volume, lesion location, and patient age (Group 1, AVM score <1; Group 2, AVM score 1-1.49; Group 3, AVM score 1.5-2; and Group 4, AVM score >2). Patients with a Spetzler-Martin Grade III AVM were divided into Grades IIIA (lesion >3 cm) and IIIB (lesion <3 cm). Sixty-two female (45.6%) and 74 male (54.4%) patients with a median age of 37.5 years (mean 37.5 years, range 5-77 years) were followed up for a median of 40 months. The median tumor margin dose was 15 Gy (mean 17.23 Gy, range 15-25 Gy). The proportions of excellent outcomes according to the AVM score were as follows: 91.7% for Group 1, 74.1% for Group 2, 60% for Group 3, and 33.3% for Group 4 (chi-square test, degrees of freedom (df) = 3, p < 0.001). Based on the modified Spetzler-Martin system, Grade I lesions had 88.9% excellent results; Grade II, 69.6%; Grade IIIB, 61.5%; and Grades IIIA and IV, 44.8% (chi-square test, df = 3, p = 0.047). The radiosurgery-based AVM score can be used accurately to predict excellent results following a single radiosurgical treatment for AVM. The modified Spetzler-Martin system can also predict radiosurgical results for AVMs, thus making it possible to use this system while deciding between surgery and radiosurgery.

  15. Role of time-resolved-CTA in intracranial arteriovenous malformation evaluation at 128-slice CT in comparison with digital subtraction angiography.

    PubMed

    Singh, Rupinder; Gupta, Vivek; Ahuja, Chirag; Kumar, Ajay; Mukherjee, Kanchan K; Khandelwal, Niranjan

    2018-06-01

    Introduction The present study aimed to evaluate the accuracy of time-resolved-computed tomographic angiography (TR-CTA) on a 128-slice CT scanner vis-à-vis cerebral digital subtraction angiography (DSA) in defining the morphological and haemodynamic characteristics of cerebral arteriovenous malformation (AVM). Methods Twenty-one patients (age range 10-46, mean 24.8 years) with clinical suspicion of AVM and three patients (age range 23-35, mean 24.3 years) with diagnosed AVM who were on follow-up underwent DSA and TR-CTA, on average 1.5 days apart. Three independent neuroradiologists analysed both studies in a blinded fashion based on the following parameters: AVM location, arterial feeder territories, venous drainage pattern, nidus flow characteristics, venous outflow obstruction, arterial feeder enlargement, external carotid artery feeder, location of aneurysm if any, leptomeningeal and transdural recruitment, neoangiogenesis, and pseudophlebitic pattern. Results The TR-CTA correctly demonstrated AVM in all 21 positive cases. It concordantly detected location (21/21), venous drainage pattern (21/21), nidus flow characteristics (21/21), and the venous outflow obstruction (9/9). However, discordance was seen in the demonstration of the arterial feeder (2/45) ( p = 0.49), arterial enlargement (13/17) ( p = 0.103), external carotid artery feeder (0/1), aneurysmal location (3/5) ( p = 0.40), leptomeningeal recruitment (1/3) ( p = 0.40), neoangiogenesis (0/4) ( p = 0.028) and in the pseudophlebitic pattern (2/5) ( p = 0.167) demonstration. Conclusions The results suggest that TR-CTA can provide the important features of cerebral AVM which are required in patient management.

  16. Onyx Embolization Before the Surgical Treatment of Grade III Spetzler-Martin Brain Arteriovenous Malformations: Single-Center Experience and Technical Nuances.

    PubMed

    Luzzi, Sabino; Del Maestro, Mattia; Bongetta, Daniele; Zoia, Cesare; Giordano, Aldo V; Trovarelli, Donatella; Raysi Dehcordi, Sohelia; Galzio, Renato J

    2018-05-09

    Grade III Spetzler-Martin brain arteriovenous malformations (AVMs) are a specific set of AVMs with high variability in terms of site, size, angioarchitecture, flow dynamics, and involvement of eloquent areas. Surgery preceded by preoperative embolization has been reported as a useful treatment option for these lesions. The aim of this study is to report outcomes and personal experience of combined preoperative Onyx embolization and surgical resection on a consecutive series of grade III brain AVMs. Between 2005 and 2017, 27 grade III AVMs were treated by means of a staged Onyx embolization and subsequent surgical treatment. Site and size of the AVMs, embolization, and surgical specifics as well as complications and outcomes were retrospectively reviewed. All AVMs were supratentorial, 13 of which were hemorrhagic. Mean nidal volume was 19.5 mL. Average embolization sessions were 1.6. Mean embolization-related obliteration rate and morbidity were 28.8% and 3.7%, respectively. Surgery was performed within 3.7 days on average. In our experience, Onyx embolization made the nidus excision easier, facilitated the hemostasis, and contributed to the early identification of the lesion in cases of small or racemose nidus. The surgical obliteration rate was 92.6%. A good overall outcome (modified Rankin Scale score 0-2) was achieved in 70.4% of patients. In our experience, preoperative Onyx embolization helped the surgical management of grade III Spetzler-Martin brain AVMs. Careful evaluation of the angioarchitecture, a tailored strategy in the embolization process, and full cooperation within the neurosurgical-neuroendovascular team are mandatory. Copyright © 2018 Elsevier Inc. All rights reserved.

  17. Retrospective analysis of imaging techniques for treatment planning and monitoring of obliteration for gamma knife treatment of cerebral arteriovenous malformation.

    PubMed

    Amponsah, Kwame; Ellis, Thomas L; Chan, Michael D; Lovato, James F; Bourland, J Daniel; deGuzman, Allan F; Ekstrand, Kenneth E; Munley, Michael T; McMullen, Kevin P; Shaw, Edward G; Tatter, Stephen B

    2012-10-01

    It has been well established that Gamma Knife radiosurgery (GKS) is an effective treatment for brain arteriovenous malformations (AVMs). To evaluate complete obliteration rates for magnetic resonance imaging (MRI)-based GKS treatment planning performed with and without angiography and to conduct a preliminary assessment of the utility of using pulsed arterial spin labeling (PASL) magnetic resonance (MR) perfusion imaging to confirm complete obliteration. Forty-six patients were identified who had undergone GKS without embolization with a minimum follow-up of 2 years. One group was planned with integrated stereotactic angiography and MR (spoiled gradient recalled) images obtained on the day of GKS. A second technique avoided the risk of arteriography by using only axial MR images. Beginning in 2007, PASL MR perfusion imaging was routinely performed as a portion of the follow-up MRI to assess the restoration of normal blood flow of the nidus and surrounding area. The overall obliteration rate for the angiography/MRI group was 88.0% (29 of 33). Patients in the MRI-only group had an obliteration rate of 61.5% (8 of 13), with P=.092 with the Fisher exact test, which is not statistically significant. A Kaplan-Meier analysis was also not statistically significant (log rank test, P=.474). Four of 9 patients with incomplete obliteration on angiography also had shown residual abnormal blood flow on PASL imaging. This retrospective analysis shows that treatment planning technique used in GKS does not play a role in the eventual obliteration of treated AVMs. PASL may have potential in the evaluation of AVM obliteration.

  18. Interest of HYPR flow dynamic MRA for characterization of cerebral arteriovenous malformations: comparison with TRICKS MRA and catheter DSA.

    PubMed

    Dautry, Raphaël; Edjlali, Myriam; Roca, Pauline; Rabrait, Cécile; Wu, Yijing; Johnson, Kevin; Wieben, Olivier; Trystram, Denis; Rodriguez-Régent, Christine; Alshareef, Fawaz; Turski, Patrick; Meder, Jean-François; Naggara, Olivier; Oppenheim, Catherine

    2015-11-01

    HYPR flow is a 3D dynamic contrast-enhanced MRA technique providing isotropic sub-millimetre resolution with half-second temporal resolution. We compared HYPR flow and time-resolved imaging of contrast kinetics (TRICKS) MRA for the characterization of cerebral arteriovenous malformations (cAVMs), using catheter DSA as reference. Twenty-two patients underwent HYPR flow and TRICKS MRA within 15 days of DSA. HYPR flow and TRICKS datasets were reviewed separately by two readers for image quality, Spetzler-Martin grade, venous ectasia, and deep venous drainage. Image quality was better for HYPR flow than for TRICKS (narrower full width at half maximum; larger arterial diagnostic window; greater number of arterial frames, P ≤ 0.05). Using HYPR flow, inter-reader agreement was excellent for all cAVM characteristics. The agreement with DSA for the overall Spetzler-Martin grade was excellent for HYPR flow (ICC = 0.96 and 0.98, depending on the reader) and TRICKS (ICC = 0.82 and 0.95). In comparison to TRICKS, HYPR flow showed higher concordance with DSA for the identification of venous ectasia and deep venous drainage. Owing to an excellent agreement with DSA with respect to depiction of the vascular architecture of cAVMs, HYPR flow could be useful for the non-invasive characterization of cAVMs. • Dynamic MRA is used for cerebral AVM depiction and follow-up • HYPR flow is a new, highly-resolved dynamic MRA sequence • HYPR flow provides whole brain coverage • HYPR flow provides excellent agreement with the Spetzler-Martin grade • Compared to TRICKS MRA, HYPR flow improves cerebral AVM characterization.

  19. The Treatment of Brain Arteriovenous Malformation Study (TOBAS): A preliminary inter- and intra-rater agreement study on patient management.

    PubMed

    Fahed, Robert; Batista, André L; Darsaut, Tim E; Gentric, Jean-Christophe; Ducroux, Célina; Chaalala, Chiraz; Roberge, David; Bojanowski, Michel W; Weill, Alain; Roy, Daniel; Magro, Elsa; Raymond, Jean

    2017-07-01

    The best management of brain arteriovenous malformation (bAVM) patients remains unknown. Randomized allocation may be more readily accepted when there is uncertainty and disagreement regarding the management of potential participants. In planning for a trial, we aimed to assess variability and agreement among physicians managing bAVM patients. A portfolio composed of 35 patients was sent to 47 clinicians of various specialties managing bAVM patients. For each patient, physicians were asked their best management decision (surgery/embolization/radiosurgery/conservative), their confidence level, and whether they would include the patient in a randomized trial comparing conservative and curative management. Seven physicians, who had access to all images of each patient, independently responded twice, to assess inter and intra-rater agreement using kappa statistics. The inter-rater agreement (30 raters, including 16 neuroradiologists) for best management decision was only "fair" (κ [95%CI]=0.210[0.157; 0.295]). Agreement remained below 'substantial' (κ<.6) between physicians of the same specialty, and when no distinctions were made between various treatments (when responses were dichotomized as conservative versus curative). With access to all images the inter-rater agreement remained fair. The intra-rater agreement reached "substantial" only for the dichotomized decisions. Responding clinicians were willing to include 54.4% of patients (mainly unruptured bAVMs) in a randomized trial. There is a lack of agreement among clinicians involved in the management of bAVM patients. In this study a substantial proportion of clinicians were willing to offer randomized allocation of management options to a substantial number of patients. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  20. Outcome after interventional or conservative management of unruptured brain arteriovenous malformations: a prospective, population-based cohort study.

    PubMed

    Wedderburn, Catherine J; van Beijnum, Janneke; Bhattacharya, Jo J; Counsell, Carl E; Papanastassiou, Vakis; Ritchie, Vaughn; Roberts, Richard C; Sellar, Robin J; Warlow, Charles P; Al-Shahi Salman, Rustam

    2008-03-01

    The decision about whether to treat an unruptured brain arteriovenous malformation (AVM) depends on a comparison of the estimated lifetime risk of intracranial haemorrhage with the risks of interventional treatment. We aimed to test whether outcome differs between adults who had interventional AVM treatment and those who did not. All adults in Scotland who were first diagnosed with an unruptured AVM during 1999-2003 (n=114) entered our prospective, population-based study. We compared the baseline characteristics and 3-year outcome of adults who received interventional treatment for their AVM (n=63) with those who did not (n=51). At presentation, adults who were treated were younger (mean 40 vs 55 years of age, 95% CI for difference 9-20; p<0.0001), more likely to present with a seizure (odds ratio 2.4, 95% CI 1.1-5.0), and had fewer comorbidities (median 3 vs 4, p=0.03) than those who were not treated. Despite these baseline imbalances, treated and untreated groups did not differ in progression to Oxford Handicap Scale (OHS) scores of 2-6 (log-rank p=0.12) or 3-6 (log-rank p=0.98) in survival analyses. In a multivariable Cox proportional hazards analysis, the risk of poor outcome (OHS 2-6) was greater in patients who had interventional treatment than in those who did not (hazard ratio 2.5, 95% CI 1.1-6.0) and was greater in patients with a larger AVM nidus (hazard ratio 1.3, 95% CI 1.1-1.7). The treated and untreated groups did not differ in time to an OHS score of 2 or more that was sustained until the end of the third year of follow-up, or in the spectrum of dependence as measured by the OHS at 1, 2, and 3 years of follow-up. Greater AVM size and interventional treatment were associated with worse short-term functional outcome for unruptured AVMs, but the longer-term effects of intervention are unclear.

  1. Assistance of intraoperative microvascular Doppler in the surgical obliteration of spinal dural arteriovenous fistula: cases description and technical considerations.

    PubMed

    Iacopino, D G; Conti, A; Giusa, M; Cardali, S; Tomasello, F

    2003-02-01

    Intraoperative microvascular Doppler may be valuable in assisting in the surgical obliteration of dural arteriovenous fistula of the spinal cord. It enables identification, through flow spectrum analysis, of the anatomic components and haemodynamic features of this type of vascular malformation. In two cases, intraoperative microvascular Doppler was used to assist in the surgical obliteration of dural arteriovenous fistula of the spinal cord. The fistulas were identified prior to the dura opening, and for this only minimally invasive surgery was required. Direct recordings of the arterialised draining vein and the nidus of the fistula demonstrated a pathological spectrum caused by the arterial supply and the disturbed venous outflow in which a high-resistance flow pattern and low diastolic flow resembling an arterial-like flow velocity were observed. The fistulas were obliterated by interruption of the draining vein, and Doppler measurements provided information on flow velocity changes in the medullary veins from an arterial to a venous pattern. The absence of any residual flow in the draining vein confirmed successful haemodynamic treatment. Intraoperative microvascular Doppler recording is valuable assistance in surgical closure of spinal arteriovenous fistula.

  2. Utilization of Cone-Beam Computed Tomographic Angiography in Planning for Gamma Knife Radiosurgery of Arteriovenous Malformations: A Case Series and Early Report

    PubMed Central

    Safain, Mina G.; Rahal, Jason P.; Raval, Ami; Rivard, Mark J.; Mignano, John; Wu, Julian; Malek, Adel M.

    2014-01-01

    Background The effectiveness of Gamma Knife radiosurgery (GKR) for cerebral arteriovenous malformations (AVM) is predicated on inclusion of the entire nidus while excluding normal tissue. As such, GKR may be limited by the resolution and accuracy of the imaging modality used in targeting. Objective We present the first case series to demonstrate the feasibility of utilizing ultra-high-resolution C-arm cone beam computed tomography angiography (CBCT-A) in AVM targeting. Methods From June 2009 to June 2013, CBCT-A was utilized for targeting of all patients with AVMs treated with GKR at our institution. Patients underwent Leksell stereotactic head frame placement followed by catheter-based biplane 2-D digital subtraction angiography (DSA), 3-D rotational angiography (3DRA), as well as CBCT-A. The CBCT-A dataset was used for stereotactic planning for GKR. Patients were followed up at 1, 3, 6, and 12 months, and then annually thereafter. Results CBCT-A-based targeting was used in twenty-two consecutive patients. CBCT-A provided detailed spatial resolution and sensitivity of nidal angioarchitecture enabling treatment. The average radiation dose to the margin of the AVM nidus corresponding to the 50% percent isodose line was 15.6 Gy. No patient had treatment-associated hemorrhage. At early follow-up (mean=16 months), 84% of patients had a decreasing or obliterated AVM nidus. Conclusion CBCT-A-guided radiosurgery is feasible and useful because it provides sufficient detailed resolution and sensitivity for imaging brain AVMs. PMID:24584136

  3. [Therapeutic indications for percutaneous laser in patients with vascular malformations and tumors].

    PubMed

    Labau, D; Cadic, P; Ouroussoff, G; Ligeron, C; Laroche, J-P; Guillot, B; Dereure, O; Quéré, I; Galanaud, J-P

    2014-12-01

    Lasers are increasingly used to treat vascular abnormalities. Indeed, this technique is non-invasive and allows a specific treatment. The aim of this review is to present some biophysical principles of the lasers, to describe the different sorts of lasers available for treatment in vascular medicine indications. Three principal lasers exist in vascular medicine: the pulsed-dye laser, for the treatment of superficial pink lesions, the NdYAG-KTP laser for purple and bigger lesions, and the NdYAG long pulse laser for even deeper and bigger vascular lesions. In vascular malformations, port wine stains can also be treated by pulsed-dye laser, KTP or NdYAG when they are old and thick. Telangiectasias are good indications for the three sorts of lasers, depending on their depth, color and size. Microcystic lymphatic malformations can be improved by laser treatment. Arterio-venous malformations constitute a contraindication of laser treatment. In vascular tumors, involuted infantile hemangiomas constitute an excellent indication of pulsed-dye laser treatment. Controlled studies are necessary to evaluate and to compare the efficacy of each laser, in order to determine their optimal indications and optimal parameters for each machine. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  4. The “focus on aneurysm” principle: Classification and surgical principles of management of concurrent arterial aneurysm with arteriovenous malformation causing intracranial hemorrhage

    PubMed Central

    Jha, Vikas; Behari, Sanjay; Jaiswal, Awadhesh K.; Bhaisora, Kamlesh Singh; Shende, Yogesh P.; Phadke, Rajendra V.

    2016-01-01

    Context: Concurrent arterial aneurysms (AAs) occurring in 2.7-16.7% patients harboring an arteriovenous malformation (AVM) aggravate the risk of intracranial hemorrhage. Aim: We evaluate the variations of aneurysms simultaneously coexisting with AVMs. A classification-based management strategy and an abbreviated nomenclature that describes their radiological features is also proposed. Setting: Tertiary care academic institute. Statistics: Test of significance applied to determine the factors causing rebleeding in the groups of patients with concurrent AVM and aneurysm and those with only AVMs. Subjects and Methods: Sixteen patients (5 with subarachnoid hemorrhage and 11 with intracerebral/intraventricular hemorrhage; 10 with low flow [LF] and 6 with high flow [HF] AVMs) underwent radiological assessment of Spetzler Martin (SM) grading and flow status of AA + AVM. Their modified Rankin's score (mRS) at admission was compared with their follow-up (F/U) score. Results: Pre-operative mRS was 0 in 5, 2 in 6, 3 in 1, 4 in 3 and 5 in 1; and, SM grade I in 5, II in 3, III in 3, IV in 4 and V in 1 patients, respectively. AA associated AVMs were classified as: (I) Flow-related proximal (n = 2); (II) flow-related distal (n = 3); (III) intranidal (n = 5); (IV) extra-intranidal (n = 2); (V) remote major ipsilateral (n = 1); (VI) remote major contralateral (n = 1); (VII) deep perforator related (n = 1); (VIII) superficial (n = 1); and (IX) distal (n = 0). Their treatment strategy included: Flow related AA, SM I-III LF AVM: aneurysm clipping with AVM excision; nidal-extranidal AA, SM I-III LF AVM: Excision or embolization of both AA + AVM; nidal-extranidal and perforator-related AA, SM IV-V HF AVM: Only endovascular embolization or radiosurgery. Surgical decision-making for remote AA took into account their ipsilateral/contralateral filling status and vessel dominance; and, for AA associated with SM III HF AVM, it varied in each patient based on diffuseness of AVM nidus, flow

  5. Feasibility of Non-contrast-enhanced MR Angiography Using the Time-SLIP Technique for the Assessment of Pulmonary Arteriovenous Malformation

    PubMed Central

    HAMAMOTO, Kohei; MATSUURA, Katsuhiko; CHIBA, Emiko; OKOCHI, Tomohisa; TANNO, Keisuke; TANAKA, Osamu

    2016-01-01

    Purpose: The purpose of this study was to evaluate the diagnostic performance of non-contrast-enhanced magnetic resonance angiography with time-spatial labeling inversion pulse (time-SLIP MRA) in the assessment of pulmonary arteriovenous malformation (PAVM). Methods: Eleven consecutive patients with 38 documented PAVMs underwent time-SLIP MRA with a 3-tesla unit. Eight patients with 25 lesions were examined twice, once before and once after embolotherapy. The lesions were divided into two groups—initial diagnosis (n = 35) and follow-up (n = 28)—corresponding to untreated and treated lesions, respectively, and were evaluated separately. To evaluate the initial diagnosis group, two reviewers assessed image quality for visualization of PAVMs by using a qualitative 4-point scale (1 = not assessable to 4 = excellent). The location and classification of PAVMs were also evaluated. The results were compared with those from digital subtraction angiography. For evaluation of the follow-up group, the reviewers assessed the status of treated PAVMs. Reperfusion and occlusion were defined respectively as visualization or disappearance of the aneurysmal sac. The diagnostic accuracy of time-SLIP MRA was assessed and compared with standard reference images. Interobserver agreement was evaluated with the κ statistic. Results: In the initial diagnosis group, time-SLIP MRA correctly determined the PAVMs in all but one patient with one lesion who had image degradation due to irregular breath. Image quality was considered excellent (median = 4) and the κ coefficient was 0.85. Additionally, both readers could correctly localize and classify the PAVMs on time-SLIP MRA images with both κ coefficient of 1.00. In the follow-up group, the sensitivity and specificity of time-SLIP MRA for reperfusion of PAVMs were both 100%, and the κ coefficient was 1.00. Conclusion: Time-SLIP MRA is technically and clinically feasible and represents a promising technique for noninvasive pre- and post

  6. The Application of the Novel Grading Scale (Lawton-Young Grading System) to Predict the Outcome of Brain Arteriovenous Malformation.

    PubMed

    Hafez, Ahmad; Koroknay-Pál, Päivi; Oulasvirta, Elias; Elseoud, Ahmed Abou; Lawton, Michael T; Niemelä, Mika; Laakso, Aki

    2018-05-04

    A supplementary grading scale (Supplemented Spetzler-Martin grade, Supp-SM) was introduced in 2010 as a refinement of the SM system to improve preoperative risk prediction of brain arteriovenous malformations (AVMs). To determine the ability to predict surgical outcomes using the Supp-SM grading scale. This retrospective study was conducted on 200 patients admitted to the Helsinki University Hospital between 2000 and 2014. The validity of the Supp-SM and SM grading systems was compared using the area under the receiver operating characteristic (AUROC) curves, with respect to the change between preoperative and early (3-4 mo) as well as final postoperative modified Rankin Scale (mRS) scores. The performance of the Supp-SM was superior to that of the SM grading scale in the early follow-up (3-4 mo): AUROC = 0.57 (95% confidence interval [CI]: 0.49-0.65) for SM and AUROC = 0.67 (95% CI: 0.60-0.75) for Supp-SM. The Supp-SM performance continued improving over SM at the late follow-up: AUROC = 0.63 (95% CI: 0.55-0.71) for SM and AUROC = 0.70 (95% CI: 0.62-0.77) for Supp-SM. The perforating artery supply, which is not part of either grading system, plays an important role in the early follow-up outcome (P = .008; odds ratio: 2.95; 95% CI: 1.32-6.55) and in the late follow-up outcome (P < .001; odds ratio: 5.89; 95% CI: 2.49-13.91). The Supp-SM grading system improves the outcome prediction accuracy and is a feasible alternative to the SMS, even for series with higher proportion of high-grade AVMs. However, perforators play important role on the outcome.

  7. EPH Receptor B4 (EPHB4) Gene Polymorphisms and Risk of Intracranial Hemorrhage in Patients with Brain Arteriovenous Malformations

    PubMed Central

    Weinsheimer, Shantel; Kim, Helen; Pawlikowska, Ludmila; Chen, Yongmei; Lawton, Michael T.; Sidney, Stephen; Kwok, Pui-Yan; McCulloch, Charles E.; Young, William L.

    2009-01-01

    Background Brain arteriovenous malformations (BAVM) are a tangle of abnormal vessels directly shunting blood from the arterial to venous circulation and an important cause of intracranial hemorrhage (ICH). EphB4 is involved in arterial-venous determination during embryogenesis; altered signaling could lead to vascular instability resulting in ICH. We investigated the association of single-nucleotide polymorphisms (SNPs) and haplotypes in EPHB4 with risk of ICH at clinical presentation in BAVM patients. Methods and Results Eight haplotype-tagging SNPs spanning ∼29 kb were tested for association with ICH presentation in 146 Caucasian BAVM patients (phase I: 56 ICH, 90 non-ICH) using allelic, haplotypic, and principal components analysis. Associated SNPs were then genotyped in 102 additional cases (phase II: 37 ICH, 65 non-ICH) and data combined for multivariable logistic regression. Minor alleles of 2 SNPs were associated with reduced risk of ICH presentation (rs314313 C, P=0.005; rs314308 T, P=0.0004). Overall, haplotypes were also significantly associated with ICH presentation (χ2=17.24, 6 df, P=0.008); 2 haplotypes containing the rs314308 T allele (GCCTGGGT, P=0.003; GTCTGGGC, P=0.036) were associated with reduced risk. In principal components analysis, 2 components explained 91% of the variance, and complemented haplotype results by implicating 4 SNPs at the 5′ end, including rs314308 and rs314313. These 2 SNPs were replicated in the phase II cohort, and combined data resulted in greater significance (rs314313, P=0.0007; rs314308, P=0.00008). SNP association with ICH presentation persisted after adjusting for age, sex, BAVM size, and deep venous drainage. Conclusions EPHB4 polymorphisms are associated with risk of ICH presentation in BAVM patients, warranting further study. PMID:20031623

  8. Use of ethylene-vinyl alcohol copolymer as a liquid embolic agent to treat a peripheral arteriovenous malformation in a dog

    PubMed Central

    Culp, William T. N.; Glaiberman, Craig B.; Pollard, Rachel E.; Wisner, Erik R.

    2015-01-01

    Case Description An 11-year-old castrated male Tibetan Mastiff was evaluated because of a visibly enlarged blood vessel and progressively worsening swelling of the right hind limb. Clinical Findings On physical examination, the right hind limb was markedly larger than the left hind limb and the dog was minimally weight bearing on the affected limb. A bruit was auscultated over the affected region. Ultrasonography of the tarsal region of the right hind limb revealed an artery with turbulent flow that communicated with venous drainage. A CT scan confirmed the presence of an arteriovenous malformation (AVM). Treatment and Outcome Embolization of the AVM with a liquid embolic agent (ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide) was elected. An arteriogram was performed prior to treatment and delineated the vessels that were targeted for embolization. The embolic agent was infused into the AVM, and a postinjection arteriogram confirmed complete occlusion of the AVM nidus and normal arterial flow to the paw with subsequent normal venous drainage. The circumference of the abnormal paw was 51 cm before the procedure and 22.9 cm at 4 weeks after the procedure. Additionally, the gait of the dog dramatically improved. No complications associated with the procedure developed. Clinical Relevance Peripheral AVMs in dogs are uncommon, and described treatment options are limited and generally associated with serious morbidity. A liquid embolic agent, ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide, was successfully administered in this case, and no morbidity was observed secondary to the procedure. Clinical success was characterized by substantial improvement in limb swelling and marked improvement in the gait of the dog. PMID:24984133

  9. The spinal cord and its roots according to Galen.

    PubMed

    Viale, Giuseppe L

    2004-06-01

    Galen's methodological approach to medicine anticipated modern rules. His experiments on the spinal cord contributed greatly to our knowledge of this structure by reporting the variegated pattern of neurological impairment after sectioning at different levels. His approach to injuries of the spinal roots and peripheral nerves documents both diagnostic skill and intellectual honesty.

  10. Obliteration dynamics in cerebral arteriovenous malformations after cyberknife radiosurgery: quantification with sequential nidus volumetry and 3-tesla 3-dimensional time-of-flight magnetic resonance angiography.

    PubMed

    Wowra, Berndt; Muacevic, Alexander; Tonn, Jörg-Christian; Schoenberg, Stefan O; Reiser, Maximilian; Herrmann, Karin A

    2009-02-01

    To investigate the time-dependent obliteration of cerebral arteriovenous malformations (cAVM) after CyberKnife radiosurgery (CKRS) (Accuray, Inc., Sunnyvale, CA) by means of sequential 3-T, 3-dimensional (3D), time-of-flight (TOF) magnetic resonance angiography (MRA), and volumetry of the arteriovenous malformation (AVM) nidus. In this prospective study, 3D TOF MRA was performed on 20 patients with cAVMs treated by single-fraction CKRS. Three-dimensional TOF MRA was performed on a 3-T, 32-channel magnetic resonance scanner (Magnetom TIM Trio; Siemens Medical Solutions, Erlangen, Germany) with isotropic voxel size at a spatial resolution of 0.6 x 0.6 x 0.6 mm3. The time-dependent relative decay of the transnidal blood flow evidenced by 3D TOF MRA was referred to as "obliteration dynamics." Volumetry of the nidus size was performed with OsiriX imaging software (OsiriX Foundation, Geneva, Switzerland). All patients had 3 to 4 follow-up examinations at 3- to 6-month intervals over a minimum follow-up period of 9 months. Subtotal obliteration was determined if the residual nidus volume was 5% or less of the initial nidus volume. Stata/IC software (Version 10.0; Stata Corp., College Station, TX) was used for statistical analysis and to identify potential factors of AVM obliteration. Regarding their clinical status, case history, and pretreatments, the participants of this study represent difficult-to-treat cAVM patients. The median nidus volume was 1.8 mL (range, 0.4-12.5 mL); the median minimum dose prescribed to the nidus was 22 Gy (range, 16-24 Gy) delivered to the 67% isodose line (range, 55-80%). CKRS was well tolerated, with complications in 2 patients. No further hemorrhages occurred after RS, except 1 small and clinically inapparent incident. The median follow-up period after RS was 25.0 months (range, 11.7-36.8 months). After RS, a statistically significant obliteration was observed in all patients. However, the obliteration dynamics of the cAVMs showed a

  11. [Marcus Aurelius Antonius (121-180AD), philosopher and Roman emperor, and Galen's plague].

    PubMed

    Muñoz-Sanz, Agustín

    2012-11-01

    The study of the aetiologies of diseases in Ancient Times is usually a speculative intellectual exercise. When some authors attribute a specific aetiology to an old disease, there is a great risk of committing a methodological error, known as presentism by the modern historiography. The authority of the investigator, more than the weight of the scientific truth, is usually the reason why the diagnosis has remained over the years. The great epidemic of the years 164-165AD and afterwards, could have been smallpox (haemorrhagic form). Claude Galen, the famous doctor, described the symptoms in several books of his great Opera Omnia. For this reason, it is currently known among the scholars as Galen's plague. The epidemic was described for the first time in Seleucia (Mesopotamia). Until now, the actual geographic origin is unknown. We propose here that the beginning might be the kingdom of the old Han dynasty (now the Chinese Popular Republic). The epidemic swept the Roman Empire, from the east to the west, and from the southern to the northern borders. An immediate consequence of the infection was a high morbidity and mortality. In this sense, Galen's epidemic was one of the many factors that caused the fall and destruction of the Roman Empire. On the other hand, there is a general agreement among historians, biographers and researchers that the philosopher emperor Marcus Aurelius Antoninus (121-180AD was affected by the infection in the epidemic wave of 164-165AD. The death of Marcus Aurelius occurred on March 17 in the year 180AD, in Vindobonne, or perhaps Sirminium (near to Vienna). Many authors propose that the cause of the emperor's death was the same epidemic. We consider that it is not possible to demonstrate any of those speculative diagnoses. Finally, the epidemic of 189-190AD, that we have named of Commodus, was probably a different disease to the Galen's plague. There were several kinds of animals affected (anthropozoonoses). In this sense, this infection

  12. Autogenous forearm loop arteriovenous fistula creation.

    PubMed

    Tang, Weng Jun; Mat Saad, Arman Zaharil

    2018-03-01

    Arteriovenous fistula is a lifeline for end-stage kidney disease patients on dialysis. The quality of the vein and artery to be used plays a crucial role in attaining a functioning, reliable and long-lasting arteriovenous fistula. The aim of the study is to present an alternative for haemodialysis access to preserve the upper arm vasculature for future use - the forearm loop arteriovenous fistula. From October 2015 to September 2016, 202 patients with chronic kidney disease (CKD), stages 4 and 5, underwent arteriovenous fistula creation at the Universiti Sains Malaysia Hospital, Malaysia. Nine patients, with severe atherosclerosis of the distal artery, but with satisfactory veins, underwent forearm loop arteriovenous fistula creation. Maturation of the fistula was based on the classification by the National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF KDOQI). All nine patients who underwent forearm loop arteriovenous fistula have had diabetes mellitus for more than 10 years. Only one fistula failed to mature within 6 weeks. Two arteriovenous fistulas thrombosed at 3 and 5 months, respectively, after the commencement of haemodialysis. However, the other six matured fistulas are still functioning well after a year of regular usage. Distal forearm arteries in diabetics may be severely atherosclerotic. Forearm loop arteriovenous fistula can be considered as the primary access for cases decided as inconvenient for fistula creation due to severe occlusive atherosclerotic disease of the forearm arteries; in order to preserve upper arm veins for future access procedures.

  13. Diffusion of counterfeit drugs in developing countries and stability of galenics stored for months under different conditions of temperature and relative humidity.

    PubMed

    Baratta, Francesca; Germano, Antonio; Brusa, Paola

    2012-04-01

    To investigate the diffusion of counterfeit medicines in developing countries and to verify the stability of galenic dosage forms to determine the stability of galenics prepared and stored in developing countries. We purchased 221 pharmaceutical samples belonging to different therapeutic classes both in authorized and illegal pharmacies and subjected them to European Pharmacopoeia, 7th ed. quality tests. An UV-visible spectrophotometric assay was used to determine the galenics stability under different conditions of temperature (T) and relative humidity (RH). A substantial percentage of samples was substandard (52%) and thus had to be considered as counterfeit. Stability tests for galenics showed that the tested dosage forms were stable for 24 months under "standard" (t=25±2°C, RH=50±5%) conditions. Under "accelerated" (t=40±2°C, RH=50±5%) conditions, samples were stable for 3 months provided that they were stored in glass containers. Stability results of samples stored in "accelerated" conditions were similar to those obtained by on site in tropical countries and could so supply precious information on the expected stability of galenics in tropical countries. This study gives useful information about the presence of counterfeit medicinal products in the pharmacies of many developing countries. This should serve as an alarm bell and an input for the production of galenics. We recommend setting up of galenic laboratories in developing countries around the globe.

  14. Is Religion Not Prosocial at All? Comment on Galen (2012)

    ERIC Educational Resources Information Center

    Saroglou, Vassilis

    2012-01-01

    Galen (2012), critically reviewing recent research on religion and prosociality, concludes that the religious prosociality hypothesis is a (congruence) fallacy. The observed effects are not real: They only reflect stereotypes and ingroup favoritism, are due to secular psychological effects, are inconsistent, and confound (e.g., by ignoring…

  15. Difference of language cortex reorganization between cerebral arteriovenous malformations, cavernous malformations, and gliomas: a functional MRI study.

    PubMed

    Deng, Xiaofeng; Xu, Long; Zhang, Yan; Wang, Bo; Wang, Shuo; Zhao, Yuanli; Cao, Yong; Zhang, Dong; Wang, Rong; Ye, Xun; Wu, Jun; Zhao, Jizong

    2016-04-01

    The authors attempted to demonstrate the difference in language cortex reorganization between cerebral malformations (AVMs), cavernous malformations (CMs), and gliomas by blood oxygen level-dependent (BOLD) functional magnetic resonance imaging. Clinical and imaging data of 27 AVM patients (AVM-L group), 29 CM patients (CM-L group), and 20 glioma patients (Glioma-L group) were retrospectively reviewed, with lesions overlying the left inferior frontal gyrus (Broca area). As a control, patients with lesions involving the right inferior frontal gyrus were also enrolled, including 14 AVM patients (AVM-R group), 20 CM patients (CM-R group), and 14 glioma patients (Glioma-R group). All patients were right-handed. Lateralization indices (LI) of BOLD signal activations were calculated separately for Broca and Wernicke areas. In AVM-L group, right-sided lateralization of BOLD signals was observed in 10 patients (37.0%), including 6 in the Broca area alone, 1 in the Wernicke area alone, and 3 in both areas. Three patients (10.3%) of CM-L group showed right-sided lateralization in both Broca and Wernicke areas, and 1 patient (5.0%) of Glioma-L group had right-sided lateralization in the Wernicke area alone. A significant difference of right-sided lateralization was observed between the AVM-L group and CM-L group (P = 0.018) and also between the AVM-L group and Glioma-L group (P = 0.027). No patient in AVM-R, CM-R, or Glioma-R groups showed right-sided lateralization. Language cortex reorganization may occur in AVM, CM, and glioma patients when the traditional language cortex was involved by lesions, but the potential of reorganization for CM and glioma patients seems to be insufficient compared with AVM patients.

  16. Estimating the Global Incidence of Aneurysmal Subarachnoid Hemorrhage: A Systematic Review for Central Nervous System Vascular Lesions and Meta-Analysis of Ruptured Aneurysms.

    PubMed

    Hughes, Joshua D; Bond, Kamila M; Mekary, Rania A; Dewan, Michael C; Rattani, Abbas; Baticulon, Ronnie; Kato, Yoko; Azevedo-Filho, Hildo; Morcos, Jacques J; Park, Kee B

    2018-04-09

    There is increasing acknowledgement that surgical care is important in global health initiatives. In particular, neurosurgical care is as limited as 1 per 10 million people in parts of the world. We performed a systematic literature review to examine the worldwide incidence of central nervous system vascular lesions and a meta-analysis of aneurysmal subarachnoid hemorrhage (aSAH) to define the disease burden and inform neurosurgical global health efforts. A systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines to estimate the global epidemiology of central nervous system vascular lesions, including unruptured and ruptured aneurysms, arteriovenous malformations, cavernous malformations, dural arteriovenous fistulas, developmental venous anomalies, and vein of Galen malformations. Results were organized by World Health Organization regions. After literature review, because of a lack of data from particular World Health Organization regions, we determined we could only provide an estimate of aSAH. Using data from studies with aSAH and 12 high-quality stroke studies from regions lacking data, we meta-analyzed the yearly crude incidence of aSAH per 100,000 persons. Estimates were generated via random-effects models. From an initial yield of 1492 studies, 46 manuscripts on aSAH incidence were included. The final meta-analysis included 58 studies from 31 different countries. We estimated the global crude incidence for aSAH to be 6.67 per 100,000 persons with a wide variation across WHO regions from 0.71 to 12.38 per 100,000 persons. Worldwide, almost 500,000 individuals will suffer from aSAH each year, with almost two-thirds in low- and middle-income countries. Copyright © 2018 Elsevier Inc. All rights reserved.

  17. Superficial middle cerebral vein connection to the cavernous sinus is not infrequent in brain arteriovenous malformations: an argument against their congenital origin?

    PubMed

    Shotar, Eimad; Guédon, Alexis; Sourour, Nader; Di Maria, Federico; Gabrieli, Joseph; Nouet, Aurélien; Chiras, Jacques; Clarençon, Frédéric

    2016-07-01

    The aim of this study was to test the hypothesis that the superficial middle cerebral vein (SMCV) is frequently absent or fails to connect with the cavernous sinus (CS) in the presence of brain arteriovenous malformations (AVMs), a frequently reported argument for the congenital origin of brain AVMs. The SMCV was retrospectively compared between patients with a brain AVM and a control group. The presence or absence of the SMCV, its direct or indirect connection to the CS and its termination in a laterocavernous sinus (LCS), paracavernous sinus (PCS), or directly in the CS was studied on digital subtraction angiography. One hundred twenty-five left or right side carotid angiograms from 70 patients with a brain AVM were compared to 125 angiograms from 74 controls. The SMCV was present in 88 (70.4 %) cases in the brain AVM group and 96 (76.8 %) cases in controls (p = 0.25). The SMCV was connected directly or indirectly to the CS in 65 (52 %) cases in the brain AVM group and 65 (52 %) cases in controls (p = 1). When comparing the subgroup of carotid angiograms ipsilateral to a supratentorial AVM, no statistically significant difference was found with controls. In three of six cases in which a SMCV drained an AVM, the vein terminated directly or indirectly in the CS. No difference of SMCV presence and direct or indirect connection to the CS was found between patients with AVM and a control group. SMCV anatomy does not support the congenital origin of brain AVMs.

  18. A prospective, multicenter, randomized trial of the Onyx liquid embolic system and N-butyl cyanoacrylate embolization of cerebral arteriovenous malformations. Clinical article.

    PubMed

    Loh, Yince; Duckwiler, Gary R

    2010-10-01

    The Onyx liquid embolic system (Onyx) was approved in the European Union in 1999 for embolization of lesions in the intracranial and peripheral vasculature, including brain arteriovenous malformations (AVMs) and hypervascular tumors. In 2001 a prospective, equivalence, multicenter, randomized controlled trial was initiated to support a submission for FDA approval. The objective of this study was to verify the safety and efficacy of Onyx compared with N-butyl cyanoacrylate (NBCA) for the presurgical treatment of brain AVMs. One hundred seventeen patients with brain AVMs were treated with either Onyx (54 patients) or NBCA (63 patients) for presurgical endovascular embolization between May 2001 and April 2003. The primary end point was technical success in achieving ≥ 50% reduction in AVM volume. Secondary end points were operative blood loss and resection time. All adverse events (AEs) were reported and assigned a relationship to the Onyx or NBCA system, treatment, disease, surgery, or other/unknown. The Data Safety Monitoring Board adjudicated AEs, and a blinded, independent core lab assessed volume measurements. Patients were monitored through discharge after the final surgery or through a 3- and/or 12-month follow-up if resection had not been performed or was incomplete. The use of Onyx led to ≥ 50% AVM volume reduction in 96% of cases versus 85% for NBCA (p = not significant). The secondary end points of resection time and blood loss were similar. Serious AEs were also similar between the 2 treatment groups. Onyx is equivalent to NBCA in safety and efficacy as a preoperative embolic agent in reducing brain AVM volume by at least 50%.

  19. Diffusion of counterfeit drugs in developing countries and stability of galenics stored for months under different conditions of temperature and relative humidity

    PubMed Central

    Baratta, Francesca; Germano, Antonio; Brusa, Paola

    2012-01-01

    Aim To investigate the diffusion of counterfeit medicines in developing countries and to verify the stability of galenic dosage forms to determine the stability of galenics prepared and stored in developing countries. Methods We purchased 221 pharmaceutical samples belonging to different therapeutic classes both in authorized and illegal pharmacies and subjected them to European Pharmacopoeia, 7th ed. quality tests. An UV-visible spectrophotometric assay was used to determine the galenics stability under different conditions of temperature (T) and relative humidity (RH). Results A substantial percentage of samples was substandard (52%) and thus had to be considered as counterfeit. Stability tests for galenics showed that the tested dosage forms were stable for 24 months under “standard” (t = 25 ± 2°C, RH = 50 ± 5%) conditions. Under “accelerated” (t = 40 ± 2°C, RH = 50 ± 5%) conditions, samples were stable for 3 months provided that they were stored in glass containers. Stability results of samples stored in “accelerated” conditions were similar to those obtained by on site in tropical countries and could so supply precious information on the expected stability of galenics in tropical countries. Conclusion This study gives useful information about the presence of counterfeit medicinal products in the pharmacies of many developing countries. This should serve as an alarm bell and an input for the production of galenics. We recommend setting up of galenic laboratories in developing countries around the globe. PMID:22522996

  20. Surgical and interventional radiographic treatment of dogs with hepatic arteriovenous fistulae.

    PubMed

    Chanoit, Guillaume; Kyles, Andrew E; Weisse, Chick; Hardie, Elizabeth M

    2007-04-01

    To report outcome after surgical and interventional radiographic treatment of hepatic arteriovenous fistulae (HAVF) in dogs. Retrospective study. Dogs (n=20) with HAVF. Medical records of dogs with HAVF were reviewed. Referring veterinarians and owners were contacted by telephone. History, clinical signs, biochemical and hematologic variables, ultrasonographic and angiographic findings, surgical findings, techniques used to correct the HAVF, survival time, and clinical follow-up were recorded. Canine HAVF often appeared to be an arteriovenous malformation rather than a single fistula. Multiple extrahepatic portosystemic shunts were identified in 19 dogs. Surgery (lobectomy or ligation of the nutrient artery) and/or interventional radiology (glue embolization of the abnormal arterial vessels) was performed in 17 dogs. Thirteen dogs were treated by surgery alone, 4 dogs by glue embolization alone, and 1 dog by glue embolization and surgery. Three dogs treated by surgery alone died <1 month later, and 3 dogs were subsequently euthanatized or died because of persistent clinical signs. None of the dogs treated by glue embolization died <1month after the procedure and all were alive, without clinical signs, at follow-up (9-17 months). Overall, 9 of 12 (75%) dogs with long-term follow-up required dietary or medical management of clinical signs. HAVF-related death occurred less frequently after glue embolization than after surgery. Glue embolization may be a good alternative to surgery for treatment of certain canine HAVF.

  1. Gamma Knife radiosurgery for cerebral arteriovenous malformations in children/adolescents and adults. Part II: Differences in obliteration rates, treatment-obliteration intervals, and prognostic factors

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Nicolato, Antonio; Lupidi, Francesco; Section of Sterotactic and Functional Neurosurgery, University of Verona and University Hospital, Verona

    2006-03-01

    Purpose: To evaluate and compare obliteration rates (OBRs) and treatment-obliteration intervals (TOIs) for cerebral arteriovenous malformations (cAVMs) treated with Gamma Knife radiosurgery in children/adolescents and adults; and to determine factors predicting the OBR and TOI in these two populations. Methods and Materials: This study concerned 62 children/adolescents and 193 adults observed for {>=}3 years. Fisher exact two-tailed and Wilcoxon rank-sum tests, multiple logistics, and Cox proportional hazard models were used for statistical analysis. Results: The overall OBR was 85.5% in children/adolescents and 87.6% in adults (p 0.671), but children/adolescents showed higher 36-month actuarial OBRs (69.35%) and shorter median TOIs (25.7more » months) than adults (66.84% and 28.2 months; p 0.006 and p = 0.017, respectively). In children/adolescents, lower Spetzler-Martin grades (p = 0.043) and younger age (p = 0.019) correlated significantly with OBRs, and lower Spetzler-Martin grades (p 0.024) and noneloquent cAVM locations (p = 0.046) with TOIs. In adults, low flow through the cAVM and <6.2-cm{sup 3} volume were associated with both OBR and TOI (p 0.012 and p = 0.002, respectively). Conclusions: The differences in OBRs within 3 years and TOIs, although slight, seem to show that pediatric cAVMs behave differently from those in adults after Gamma Knife radiosurgery.« less

  2. Time resolved imaging of contrast kinetics (TRICKS) MR angiography of arteriovenous malformations of head and neck.

    PubMed

    Razek, Ahmed Abdel Khalek Abdel; Gaballa, Gada; Megahed, Abdel Salam; Elmogy, Ebrahiem

    2013-11-01

    To evaluate vasculature of arteriovenous malformations (AVMs) of head and neck with time resolved imaging of contrast kinetics (TRICKS) MR angiography (MRA). Prospective study was conducted upon 19 patients (age range, 12-29 years; mean age 18 years; 10 males and 9 females) with AVM of head and neck. TRICKS-MRA of head and neck was performed during injection of contrast medium. Post processing with reconstruction of the images was done. Two independent readers assessed the overall TRICKS-MRA image quality score using a 5-point scale and depiction of the main arterial feeders, nidus, and venous drainage using 3 points scale. The Kappa test for interobserver agreement was done. The AVMs were evaluated morphologically in terms of number and origin of the main arterial feeders, the location and size of nidus either small (>2 cm) or large (>2 cm) and the draining veins into the superficial or deep venous drainage. The average TRICKS-MRA image quality score as judged by reader 1 was 3.89 ± 1.15 and that as judged by reader 2 was 3.89 ± 0.10, which yielded excellent interobserver agreement (k=0.77, 95% CI=0.53-0.98, r=0.78, P=0.001). The interobserver agreement of both readers was excellent for the arterial feeders (k=0.81, 95% CI=0.57-1.00, r=0.83, P=0.001), excellent for the nidus (k=0.91, 95% CI=0.75-1.00, r=0.92, P=0.001), and good for the venous drainage (k=0.77, 95% CI=0.53-0.98, r=0.78, P=0.001). The arterial feeders were single (n=14) or multiple (n=5), the nidus was large (n=16) or small (n=3) and the venous drainage was into the internal jugular (n=17) or the external jugular (n=2) veins. Three patients with small nidus and single arterial feeder were treated with sclerotherapy. Eleven patients with large nidus and single arterial feeder were referred for embolization. Combined embolization and surgery were done for five patients with large nidus and multiple arterial feeders. We concluded that TRICKS-MRA is a reliable non invasive tool for evaluation of the

  3. Gamma Knife surgery for arteriovenous malformations in the brain: integration of time-resolved contrast-enhanced magnetic resonance angiography into dosimetry planning. Technical note.

    PubMed

    Taschner, Christian A; Le Thuc, Vianney; Reyns, Nicolas; Gieseke, Juergen; Gauvrit, Jean-Yves; Pruvo, Jean-Pierre; Leclerc, Xavier

    2007-10-01

    The aim of this study was to develop an algorithm for the integration of time-resolved contrast-enhanced magnetic resonance (MR) angiography into dosimetry planning for Gamma Knife surgery (GKS) of arteriovenous malformations (AVMs) in the brain. Twelve patients harboring brain AVMs referred for GKS underwent intraarterial digital subtraction (DS) angiography and time-resolved MR angiography while wearing an externally applied cranial stereotactic frame. Time-resolved MR angiography was performed on a 1.5-tesla MR unit (Achieva, Philips Medical Systems) using contrast-enhanced 3D fast field echo sequencing with stochastic central k-space ordering. Postprocessing with interactive data language (Research Systems, Inc.) produced hybrid data sets containing dynamic angiographic information and the MR markers necessary for stereotactic transformation. Image files were sent to the Leksell GammaPlan system (Elekta) for dosimetry planning. Stereotactic transformation of the hybrid data sets containing the time-resolved MR angiography information with automatic detection of the MR markers was possible in all 12 cases. The stereotactic coordinates of vascular structures predefined from time-resolved MR angiography matched with DS angiography data in all cases. In 10 patients dosimetry planning could be performed based on time-resolved MR angiography data. In two patients, time-resolved MR angiography data alone were considered insufficient. The target volumes showed a notable shift of centers between modalities. Integration of time-resolved MR angiography data into the Leksell GammaPlan system for patients with brain AVMs is feasible. The proposed algorithm seems concise and sufficiently robust for clinical application. The quality of the time-resolved MR angiography sequencing needs further improvement.

  4. Arteriovenous Malformation

    MedlinePlus

    ... blood clot to close the lesion or focused irradiation treatment that is designed to damage the blood ... blood clot to close the lesion or focused irradiation treatment that is designed to damage the blood ...

  5. Spinal dural arteriovenous fistulas: the most frequent vascular malformations of the spinal cord.

    PubMed

    Iglesias Gordo, J; Martínez García, R

    Spinal dural arteriovenous fistulas are produced by direct communication between the arterial and venous systems of the spinal cord, causing hypertension in the latter with spinal cord dysfunction. It is a rare pathology with unknown etiology and non-specific clinical symptoms that usually results in a delayed diagnosis. Often radiologists are the first to guide the disease towards an adequate diagnosis. Characteristic findings can be seen through MR or MR angiography, and may even locate the fistula in a high percentage of cases, although the pathology must be confirmed by spinal angiography. There are two treatment modalities: endovascular and surgical therapy. Endovascular treatment has improved in recent years with the advantages of a less invasive approach and is therefore usually chosen as primary therapy. In this article we review the main clinical manifestations, imaging findings and treatment of this pathology. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  6. Cerebral arteriovenous malformations and seizures: differential impact on the time to seizure-free state according to the treatment modalities.

    PubMed

    Hyun, Seung-Jae; Kong, Doo-Sik; Lee, Jung-Il; Kim, Jong-Soo; Hong, Seung-Chyul

    2012-06-01

    To determine the prognostic factors for the incidence and the outcome of seizure in patients with cerebral arteriovenous malformation (AVM) and to identify the time to seizure-free state according to the treatment modalities. Between 1995 and 2008, the multidisciplinary team at our institution treated 399 patients with cerebral AVMs. Treatment consisted of surgical resection, radiosurgery, and embolization, either alone or in combination. The median follow-up period was 6.0 years (range, 3.0-16.2 years). Eighty-six patients (21.5 %) experienced seizures before treatment. We investigated the variables associated with seizure incidence and seizure outcome and analyzed the outcomes of seizure among each treatment modality. After treatment, 60 (70 %) patients were seizure-free. Compared with 313 patients who did not experience seizures, we found that younger age (≤ 35 years), size ≥ 3 cm, and location of temporal lobe were associated with seizures (p < 0.05). Short seizure history, accompanying intracerebral hemorrhage, generalized tonic-clonic type seizure, deep-seated or infratentorial AVM, complete obliteration of AVM, and a favorable neurological outcome at 12 months were closely associated with Engel Class I outcomes (p < 0.05). Seizure-free outcomes after microsurgery, radiosurgery, or embolization were 78 %, 66 %, and 50 %, respectively. The overall annual bleeding rate was 1.0 % and 2.2 % in microsurgery-treated and radiosurgery-treated AVMs, respectively. In the surgery group, the median time to seizure-free status was 1.1 months (95 % CI, 0.7-1.2 months), whereas the radiosurgery group and embolization-alone group showed 20.5 months (95 % CI, 18.3-23.8 months), and 8.1 months (95 % CI, 6.0-13.5 months), respectively. A multidisciplinary team approach for cerebral AVMs achieved satisfactory seizure control results. Microsurgery led to the highest percentage of seizure-free outcomes and had the lowest annual bleeding rate, whereas radiosurgery had a higher

  7. Balloon-Occluded Retrograde Transvenous Obliteration of a Gastric Vascular Malformation: An Innovative Approach to Treatment of a Rare Condition

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hansing, Catherine E., E-mail: catherine.e.hansing.mil@mail.mil; Marquardt, Joseph P.; Sutton, Daniel M.

    Arteriovenous malformations (AVMs) are a high-flow form of a vascular malformation, which can be found anywhere in the body. While historically treated surgically, a multidisciplinary approach utilizing multiple specialties and treatment modalities is now commonly employed. In order to effectively treat an AVM, the nidus must be targeted and eradicated, which can be done via multiple approaches. We present the case of a 43-year-old male with a gastric wall AVM, which was initially incompletely treated using a percutaneous transarterial approach. The gastric AVM was noted to have dominant drainage through a gastrorenal shunt; therefore, Balloon-occluded Retrograde Transvenous Obliteration (BRTO) wasmore » utilized to eradicate the AVM nidus. This case illustrates the utility of Interventional Radiology, specifically BRTO, as another treatment option for challenging AVMs.« less

  8. OUT-OF-FIELD DOSES IN CHILDREN TREATED FOR LARGE ARTERIOVENOUS MALFORMATIONS USING HYPOFRACTIONATED GAMMA KNIFE RADIOSURGERY AND INTENSITY-MODULATED RADIATION THERAPY.

    PubMed

    De Saint-Hubert, Marijke; Majer, Marija; Hršak, Hrvoje; Heinrich, Zdravko; Kneževic, Željka; Miljanic, Saveta; Porwol, Paulina; Stolarczyk, Liliana; Vanhavere, Filip; Harrison, Roger M

    2018-01-17

    The purpose of this study was to measure out-of-field organ doses in two anthropomorphic child phantoms for the treatment of large brain arteriovenous malformations (AVMs) using hypofractionated gamma knife (GK) radiosurgery and to compare these with an alternative treatment using intensity-modulated radiation therapy (IMRT). Target volume was identical in size and shape in all cases. Radiophotoluminescent (RPL), thermoluminescent (TL) and optically stimulated luminescent (OSL) dosimeters were used for out-of-field dosimetry during GK treatment and a good agreement within 1-2% between results was shown. In addition, the use of multiple dosimetry systems strengthens the reliability of the findings. The number of GK isocentres was confirmed to be important for the magnitude of out-of-field doses. Measured GK doses for the same distance from the target, when expressed per target dose and isocentre, were comparable in both phantoms. GK out-of-field doses averaged for both phantoms were evaluated to be 120 mGy/Gy for eyes then sharply reduced to 20 mGy/Gy for mandible and slowly reduced up to 0.8 mGy/Gy for testes. Taking into account the fractionation regimen used to treat AVM patients, the total treatment organ doses to the out-of-field organs were calculated and compared with IMRT. The eyes were better spared with GK whilst for more distant organs doses were up to a factor of 2.8 and 4 times larger for GK compared to IMRT in 5-year and 10-year old phantoms, respectively. Presented out-of-field dose values are specific for the investigated AVM case, phantoms and treatment plans used for GK and IMRT, but provide useful information about out-of-field dose levels and emphasise their importance. © The Author(s) 2018. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  9. Melorheostosis with renal arterio-venous malformation: A case report with review of literature

    PubMed Central

    Lone, Abdul Rashid; Ahmad, Mushtaq; Aziz, Sheikh Aejaz; Bhat, Gul Mohammad; Bhat, Javid Rasool; Jahan, Rifat; Khan, Shoukat H

    2009-01-01

    Melorheostosis, also known as Leri′s disease and flowing periosteal hyperostosis, is a rare cause of pain and stiffness in a limb. The appearance is of "candle greasing" down one side of one or several bones of the body. We describe a case referred to tertiary care center with suspicion of renal cell carcinoma with diffuse bone metastasis. After reassessment, the patient was diagnosed melorheostosis with renal AV malformation. He was reassured about the benign nature of the disease and is asymptomatic. PMID:20668607

  10. Cannabinoids concentration variability in cannabis olive oil galenic preparations.

    PubMed

    Carcieri, Chiara; Tomasello, Cristina; Simiele, Marco; De Nicolò, Amedeo; Avataneo, Valeria; Canzoneri, Luca; Cusato, Jessica; Di Perri, Giovanni; D'Avolio, Antonio

    2018-01-01

    Knowledge of the exact concentration of active compounds in galenic preparations is crucial to be able to ensure their quality and to properly administer the prescribed dose. Currently, the need for titration of extracts is still debated. Considering this, together with the absence of a standard preparation method, the aim of this study was to evaluate cannabinoids concentrations variability in galenic olive oil extracts, to evaluate the interlot and interlaboratory variability in the extraction yield and in the preparation composition. Two hundred and one extracts (123 (61.2%) from Bedrocan ® , 54 (26.9%) from Bediol ® , 11 (5.5%) from Bedrolite ® , and 13 (6.5%) from mixed preparations) were analysed by liquid chromatography coupled with tandem mass spectrometry, quantifying cannabinoids (THC, CBD, THCA, CBDA and CBN) concentrations. The RSD% of THC and CBD concentrations resulted higher than 50%. Specifically for Bedrocan ® , Bediol ® , Bedrolite ® (5 g/50 ml), these were THC 82%, THC 53% and CBD 91%, THC 58% and CBD 59%, respectively. The median extraction yields were greater than 75% for all preparations. Our results highlighted a wide variability in THC and CBD concentrations that justify the need for titration and opens further questions about other pharmaceutical preparations without regulatory indication for this procedure. © 2017 Royal Pharmaceutical Society.

  11. Neuroanatomical study of Galen's anastomosis (nervus laryngeus) in the dog.

    PubMed

    Henry, C; Cazals, Y; Gioux, M; Didier, A; Aran, J M; Traissac, L

    1988-01-01

    To further knowledge of the laryngeal nerves, the nerve fibers of Galen's anastomosis were studied using two neuroanatomical methods, namely nerve degeneration and horseradish peroxidase labeling. It is demonstrated that the superior laryngeal nerve forms part of the tracheal and esophageal nervous system. The value of the results in relation to physiological laryngeal studies and to human laryngeal diseases is discussed.

  12. Loss of unc45a precipitates arteriovenous shunting in the aortic arches

    PubMed Central

    Anderson, Matthew J.; Pham, Van N.; Vogel, Andreas M.; Weinstein, Brant M.; Roman, Beth L.

    2008-01-01

    Aortic arch malformations are common congenital disorders that are frequently of unknown etiology. To gain insight into the factors that guide branchial aortic arch development, we examined the process by which these vessels assemble in wild type zebrafish embryos and in kurzschlusstr12 (kus tr12) mutants. In wild type embryos, each branchial aortic arch first appears as an island of angioblasts in the lateral pharyngeal mesoderm, then elaborates by angiogenesis to connect to the lateral dorsal aorta and ventral aorta. In kustr12 mutants, angioblast formation and initial sprouting are normal, but aortic arches 5 and 6 fail to form a lumenized connection to the lateral dorsal aorta. Blood enters these blind-ending vessels from the ventral aorta, distending the arteries and precipitating fusion with an adjacent vein. This arteriovenous malformation (AVM), which shunts nearly all blood directly back to the heart, is not genetically programmed, as its formation correlates with blood flow and aortic arch enlargement. By positional cloning, we have identified a nonsense mutation in unc45a in kustr12 mutants. Our results are the first to ascribe a role for Unc45a, a putative myosin chaperone, in vertebrate development, and identify a novel mechanism by which an AVM can form. PMID:18462713

  13. Radiological aspects of gamma knife radiosurgery for arteriovenous malformations and other non-tumoural disorders of the brain.

    PubMed

    Guo, W Y

    1993-01-01

    The aims of the thesis were to investigate stereotaxic procedures in radiosurgery for cerebral arteriovenous malformations (AVMs) and radiation effects of single session high-dose irradiation delivered by gamma knife on the human brain. Investigation of gamma knife radiosurgery in 1,464 patients constitutes the data base of this thesis. High quality stereotaxic angiography is the gold standard targeting imaging in radiosurgery for cerebral AVMs, particularly for small AVMs or residual AVMs after other treatments. For medium and large size AVMs, stereotaxic MR techniques can improve targeting precision and decrease irradiation volume as compared to stereotaxic angiography in selected cases provided that proper pulse sequences are used. Combined treatments, where embolization precedes radiosurgery, can improve amenability of the treatment for large AVMs. This is on condition that the partially embolized nidi are well delineated and the volume of the residual nidi has been decreased to a level where an optimum irradiation can be safely prescribed. Radiologically, adverse radiation effects (ARE) of gamma knife radiosurgery for cerebral AVMs are observed in 16% (131/816) of the patients. The ARE are observed as a focal low attenuation on CT or as a focal high signal on MR image without enhancement in 47% (61/131), and as a peripheral or homogeneous enhancing lesion in 48% (63/131). MR imaging is more sensitive than CT in detecting the ARE. 91% of the ARE are observed within 18 months after radiosurgery and 89% are seen to regress within 18 months. Clinically, symptomatic ARE are only observed in 6% (51/816) and only in half of them, i.e. 3%, are the symptoms permanent. The risk of ARE in radiosurgery for venous angiomas is higher as compared to AVMs. Other mechanisms have probably been employed. In gamma capsulotomy, the necrotic lesions and reaction volumes created by using multiple isocentres of 4 mm collimators are less predictable as compared to that by single

  14. Planning Evaluation of C-Arm Cone Beam CT Angiography for Target Delineation in Stereotactic Radiation Surgery of Brain Arteriovenous Malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kang, Jun; Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland; Huang, Judy

    Purpose: Stereotactic radiation surgery (SRS) is one of the therapeutic modalities currently available to treat cerebral arteriovenous malformations (AVM). Conventionally, magnetic resonance imaging (MRI) and MR angiography (MRA) and digital subtraction angiography (DSA) are used in combination to identify the target volume for SRS treatment. The purpose of this study was to evaluate the use of C-arm cone beam computed tomography (CBCT) in the treatment planning of SRS for cerebral AVMs. Methods and Materials: Sixteen consecutive patients treated for brain AVMs at our institution were included in this retrospective study. Prior to treatment, all patients underwent MRA, DSA, and C-arm CBCT.more » All images were coregistered using the GammaPlan planning system. AVM regions were delineated independently by 2 physicians using either C-arm CBCT or MRA, resulting in 2 volumes: a CBCT volume (VCBCT) and an MRA volume (V{sub MRA}). SRS plans were generated based on the delineated regions. Results: The average volume of treatment targets delineated using C-arm CBCT and MRA were similar, 6.40 cm{sup 3} and 6.98 cm{sup 3}, respectively (P=.82). However, significant regions of nonoverlap existed. On average, the overlap of the MRA with the C-arm CBCT was only 52.8% of the total volume. In most cases, radiation plans based on V{sub MRA} did not provide adequate dose to the region identified on C-arm CBCT; the mean minimum dose to V{sub CBCT} was 29.5%, whereas the intended goal was 45% (P<.001). The mean volume of normal brain receiving 12 Gy or more in C-arm CBCT-based plans was not greater than in the MRA-based plans. Conclusions: Use of C-arm CBCT images significantly alters the delineated regions of AVMs for SRS planning, compared to that of MRA/MRI images. CT-based planning can be accomplished without increasing the dose to normal brain and may represent a more accurate definition of the nidus, increasing the chances for successful obliteration.« less

  15. Tentorial dural arteriovenous fistula presenting as myelopathy: Case series and review of literature

    PubMed Central

    Gross, Robert; Ali, Rushna; Kole, Max; Dorbeistein, Curtis; Jayaraman, Mahesh V; Khan, Muhib

    2014-01-01

    Dural arteriovenous fistula (DAVF) is a rare type of cerebral arteriovenous malformation. Common presenting symptoms are related to hemorrhage. However, rarely these patients may present with myelopathy. We present two cases of DAVF presenting as rapidly progressive myelopathy. Two treatment options are available: microsurgical interruption of the fistula and endovascular embolization. These treatment options of DAVFs have improved significantly in the last decade. The optimal treatment of DAVFs remains controversial, and there is an ongoing debate as to whether primary endovascular or primary microsurgical treatment is the optimal management for these lesions. However, despite treatment a high percentage of patients are still left with severe disability. The potential for functional ambulation in patients with DAVF is related to the time of intervention. This emphasizes the important of early diagnosis and early intervention in DAVF. The eventual outcome may depend on several factors, such as the duration of symptoms, the degree of disability before treatment, and the success of the initial procedure to close the fistula. The usage of magnetic resonance imaging and selective angiography has significantly improved the ability to characterize DAVFs, however, these lesions remain inefficiently diagnosed. If intervention is delayed even prolonged time in rehabilitation does not change the grave prognosis. This review outlines the presentation, classication and management of DAVF as well as discussing patient outcomes. PMID:25516869

  16. Cerebral Abscess Associated With Odontogenic Bacteremias, Hypoxemia, and Iron Loading in Immunocompetent Patients With Right-to-Left Shunting Through Pulmonary Arteriovenous Malformations.

    PubMed

    Boother, Emily J; Brownlow, Sheila; Tighe, Hannah C; Bamford, Kathleen B; Jackson, James E; Shovlin, Claire L

    2017-08-15

    Cerebral abscess is a recognized complication of pulmonary arteriovenous malformations (PAVMs) that allow systemic venous blood to bypass the pulmonary capillary bed through anatomic right-to-left shunts. Broader implications and mechanisms remain poorly explored. Between June 2005 and December 2016, at a single institution, 445 consecutive adult patients with computed tomography-confirmed PAVMs (including 403 [90.5%] with hereditary hemorrhagic telangiectasia) were recruited to a prospective series. Multivariate logistic regression was performed and detailed periabscess histories were evaluated to identify potential associations with cerebral abscess. Rates were compared to an earlier nonoverlapping series. Thirty-seven of the 445 (8.3%) patients experienced a cerebral abscess at a median age of 50 years (range, 19-76 years). The rate adjusted for ascertainment bias was 27 of 435 (6.2%). Twenty-nine of 37 (78.4%) patients with abscess had no PAVM diagnosis prior to their abscess, a rate unchanged from earlier UK series. Twenty-one of 37 (56.7%) suffered residual neurological deficits (most commonly memory/cognition impairment), hemiparesis, and visual defects. Isolation of periodontal microbes, and precipitating dental and other interventional events, emphasized potential sources of endovascular inoculations. In multivariate logistic regression, cerebral abscess was associated with low oxygen saturation (indicating greater right-to-left shunting); higher transferrin iron saturation index; intravenous iron use for anemia (adjusted odds ratio, 5.4 [95% confidence interval, 1.4-21.1]); male sex; and venous thromboemboli. There were no relationships with anatomic attributes of PAVMs, or red cell indices often increased due to secondary polycythemia. Greater appreciation of the risk of cerebral abscess in undiagnosed PAVMs is required. Lower oxygen saturation and intravenous iron may be modifiable risk factors. © The Author 2017. Published by Oxford University Press

  17. Sci-Fri PM: Radiation Therapy, Planning, Imaging, and Special Techniques - 02: Feasibility of using multileaf collimation for stereotactic radiosurgery of arteriovenous malformation

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Lee, Young; Ruschin, Mark

    SRS using linac and cones offers steep dose fall-off but a tradeoff exists between conformality and treatment time, which depends on the number of isocentres. Purpose of this study is to quantify planning metrics between cones- and MLC-based SRS for arteriovenous malformation(AVM). Seven AVM cases treated with cones were re-planned with MLC on Pinnacle treatment planning system. Planning target volume(PTV) was created with 1mm uniform margin to the AVM to account for MLC positional variation. Clinically-planned prescription dose(15–25Gy) was used. Four plans were generated per case:non-coplanar VMAT(ncV), single-arc VMAT(saV), non-coplanar IMRT(ncI), non-coplanar conformal(ncC). Plans were compared for conformity(CI), heterogeneity(HI) andmore » gradient(GI) indices and brain doses. Estimated treatment times and monitor units(MU) were compared. Cone-based plans required 2–6 isocentres. Though CI-RTOG was similar for plans(median=0.98), CI-Paddick was most favourable for ncV(median=0.86) and worst for cones(0.54). HI for MLC plans(median=1.19–1.27) were lower than cone-based plans(1.43). GI was similar for all plans. For 2/7 ncC had brainstem maximum dose>16.7Gy and therefore were clinically unacceptable. Brain V12Gy,V10Gy,V2Gy were lowest in the cones plan. ncV brain V12Gy,V10Gy,V2Gy were lowest of all MLC-based plans studied. Treatment MUs were similar for MLC-based plans and up to 70% lower than clinically delivered plans. ncV showed best conformality in this study. Of the MLC-based plans, ncV also showed lowest normal tissue dose with reasonable treatment time.« less

  18. Cerebral Abscess Associated With Odontogenic Bacteremias, Hypoxemia, and Iron Loading in Immunocompetent Patients With Right-to-Left Shunting Through Pulmonary Arteriovenous Malformations

    PubMed Central

    Boother, Emily J.; Brownlow, Sheila; Tighe, Hannah C.; Bamford, Kathleen B.; Jackson, James E.

    2017-01-01

    Abstract Background Cerebral abscess is a recognized complication of pulmonary arteriovenous malformations (PAVMs) that allow systemic venous blood to bypass the pulmonary capillary bed through anatomic right-to-left shunts. Broader implications and mechanisms remain poorly explored. Methods Between June 2005 and December 2016, at a single institution, 445 consecutive adult patients with computed tomography–confirmed PAVMs (including 403 [90.5%] with hereditary hemorrhagic telangiectasia) were recruited to a prospective series. Multivariate logistic regression was performed and detailed periabscess histories were evaluated to identify potential associations with cerebral abscess. Rates were compared to an earlier nonoverlapping series. Results Thirty-seven of the 445 (8.3%) patients experienced a cerebral abscess at a median age of 50 years (range, 19–76 years). The rate adjusted for ascertainment bias was 27 of 435 (6.2%). Twenty-nine of 37 (78.4%) patients with abscess had no PAVM diagnosis prior to their abscess, a rate unchanged from earlier UK series. Twenty-one of 37 (56.7%) suffered residual neurological deficits (most commonly memory/cognition impairment), hemiparesis, and visual defects. Isolation of periodontal microbes, and precipitating dental and other interventional events, emphasized potential sources of endovascular inoculations. In multivariate logistic regression, cerebral abscess was associated with low oxygen saturation (indicating greater right-to-left shunting); higher transferrin iron saturation index; intravenous iron use for anemia (adjusted odds ratio, 5.4 [95% confidence interval, 1.4–21.1]); male sex; and venous thromboemboli. There were no relationships with anatomic attributes of PAVMs, or red cell indices often increased due to secondary polycythemia. Conclusions Greater appreciation of the risk of cerebral abscess in undiagnosed PAVMs is required. Lower oxygen saturation and intravenous iron may be modifiable risk factors. PMID

  19. Factors predicting language lateralization in patients with perisylvian vascular malformations

    PubMed Central

    Lee, Darrin J.; Pouratian, Nader; Bookheimer, Susan Y.; Martin, Neil A.

    2017-01-01

    Object The authors conducted a study to determine the factors associated with right-sided language dominance in patients with cerebrovascular malformations. Methods Twenty-two patients with either arteriovenous malformations (AVMs [15 cases]) or cavernous malformations (7 cases) underwent functional MR (fMR) imaging studies of language function; a 3.0-T head-only unit was used. Lateralization indices were calculated separately for Broca and Wernicke areas. Lesion size, Spetzler-Martin grade, and the distance between the lesion and anatomically defined language cortex were calculated for each patient. Results Right-sided language dominance occurred in 5 patients, all of whom had AVMs within 10 mm of canonical language areas. Three patients had right-sided language dominance in the Wernicke area alone whereas 2 had right-sided language dominance in both Broca and Wernicke areas. Wada testing and intraoperative electrocortical stimulation were performed as clinically indicated to corroborate fMR imaging findings. Conclusions The primary factor associated with right-sided language dominance was the AVM being within 10 mm of anatomically defined language areas. The lesion size and the Spetzler-Martin grade were not significant factors. Anomalous fMR imaging laterality was typically confined to the language area proximate to the lesion, with the distal language area remaining in the left hemisphere dominant. This study emphasizes the need to map each case individually in patients with left perisylvian AVMs. Assumptions about eloquent cortex based on anatomical landmarks (a key component of Spetzler-Martin grading) may have to be reconsidered. PMID:20302390

  20. Pregnancy causes diminished myogenic tone and outward hypotrophic remodeling of the cerebral vein of Galen.

    PubMed

    van der Wijk, Anne-Eva; Schreurs, Malou P H; Cipolla, Marilyn J

    2013-04-01

    Pregnancy increases the risk of several complications associated with the cerebral veins, including thrombosis and hemorrhage. In contrast to the cerebral arteries and arterioles, few studies have focused on the effect of pregnancy on the cerebral venous side. Here, we investigated for the first time the effect of pregnancy on the function and structure of the cerebral vein of Galen in rats. Our major finding was that cerebral veins from late-pregnant (LP, n=11) rats had larger lumen diameters and thinner walls than veins from nonpregnant (NP, n=13) rats, indicating that pregnancy caused outward hypotrophic remodeling of the vein of Galen. Moreover, veins from NP animals had a small amount of myogenic tone at 10 mm Hg (3.9±1.0%) that was diminished in veins during pregnancy (0.8±0.3%; P<0.01). However, endothelium-dependent and -independent vasodilation of the veins was unchanged during pregnancy. Using immunohistochemistry, we show that the vein of Galen receives perivascular innervation, and that serotonergic innervation of cerebral veins is significantly higher in veins from LP animals. Outward hypotrophic remodeling and diminished tone of cerebral veins during pregnancy may contribute to the development of venous pathology through elevated wall tension and wall stress, and possibly by promoting venous blood stasis.

  1. Rationalism, Empiricism, and Evidence-Based Medicine: A Call for a New Galenic Synthesis.

    PubMed

    Webb, William M

    2018-04-25

    Thirty years after the rise of the evidence-based medicine (EBM) movement, formal training in philosophy remains poorly represented among medical students and their educators. In this paper, I argue that EBM’s reception in this context has resulted in a privileging of empiricism over rationalism in clinical reasoning with unintended consequences for medical practice. After a limited review of the history of medical epistemology, I argue that a solution to this problem can be found in the method of the 2nd-century Roman physician Galen, who brought empiricism and rationalism together in a synthesis anticipating the scientific method. Next, I review several of the problems that have been identified as resulting from a staunch commitment to empiricism in medical practice. Finally, I conclude that greater epistemological awareness in the medical community would precipitate a Galenic shift toward a more epistemically balanced, scientific approach to clinical research.

  2. Reproducibility of oxygen saturation monitoring during six-minute walk test and exercise stress test in patients with pulmonary arteriovenous malformations associated with hereditary hemorrhagic telangiectasia.

    PubMed

    Li, Walter; Niu, Bolin; Henderson, Katherine; Northrup, Veronika; Pollak, Jeffery S; Trow, Terence; Fahey, John; White, Robert I

    2011-06-01

    Patients with pulmonary arteriovenous malformations (PAVMs) are at risk for multiple complications and require close follow-up. We investigated the reproducibility of the 6-minute walk test (6MWT) and exercise stress test (EST) for the evaluation of low oxygen saturation in patients with PAVMs. Twenty-two patients with PAVMs, most of whom had hereditary hemorrhagic telangiectasia (HHT), participated in a Human Investigations Committee-approved protocol. Patients ranged from 9 to 74 years of age (mean 28) and had a broad spectrum of anatomic subtypes of PAVMs, including focal and diffuse. Standard 6MWT and cycle ergometry EST were both performed twice with adequate rest between tests. Heart rate (HR) and oxygen saturation were measured at the beginning and end of each test. Distance walked and maximum resistance was also recorded. The intraclass correlation coefficients (r(i)) at the end of 6MWT were as follows: HR (r(i) = 0.940; 95% confidence interval [CI] 0.863-0.975), oxygen saturation (r(i) = 0.973; 95% CI 0.933-0.989), and distance (r(i) = 0.942; 95% CI 0.867-0.975). The r(i)s at the end of EST were as follows: HR (r(i) = 0.941; 95% CI 0.865-0.975), oxygen saturation (r(i) = 0.993; 95% CI 0.982-0.997), and maximum resistance (r(i) = 0.941; 95% CI 0.864-0.975). 6MWT and EST were reproducible measures of exercise capacity and oxygen saturation and are potential adjunct tests in the follow-up assessment for patients with PAVMs.

  3. Assessment of blood supply to intracranial pathologies in children using MR digital subtraction angiography.

    PubMed

    Chooi, Weng Kong; Connolly, Dan J A; Coley, Stuart C; Griffiths, Paul D

    2006-10-01

    MR digital subtraction angiography (MR-DSA) is a contrast-enhanced MR angiographic sequence that enables time-resolved evaluation of the cerebral circulation. We describe the feasibility and technical success of our attempts at MR-DSA for the assessment of intracranial pathology in children. We performed MR-DSA in 15 children (age range 5 days to 16 years) referred for MR imaging because of known or suspected intracranial pathology that required a dynamic assessment of the cerebral vasculature. MR-DSA consisted of a thick (6-10 mm) slice-selective RF-spoiled fast gradient-echo sequence (RF-FAST) acquired before and during passage of an intravenously administered bolus of Gd-DTPA. The images were subtracted and viewed as a cine loop. MR-DSA was performed successfully in all patients. High-flow lesions were shown in four patients; these included vein of Galen aneurysmal malformation, dural fistula, and two partially treated arteriovenous malformations (AVMs). Low-flow lesions were seen in three patients, all of which were tumours. Normal flow was confirmed in eight patients including two with successfully treated AVMs, and in three patients with cavernomas. Our early experience suggests that MR-DSA is a realistic, non-invasive alternative to catheter angiography in certain clinical settings.

  4. Long-term Outcomes With Planned Multistage Reduced Dose Repeat Stereotactic Radiosurgery for Treatment of Inoperable High-Grade Arteriovenous Malformations: An Observational Retrospective Cohort Study.

    PubMed

    Marciscano, Ariel E; Huang, Judy; Tamargo, Rafael J; Hu, Chen; Khattab, Mohamed H; Aggarwal, Sameer; Lim, Michael; Redmond, Kristin J; Rigamonti, Daniele; Kleinberg, Lawrence R

    2017-07-01

    There is no consensus regarding the optimal management of inoperable high-grade arteriovenous malformations (AVMs). This long-term study of 42 patients with high-grade AVMs reports obliteration and adverse event (AE) rates using planned multistage repeat stereotactic radiosurgery (SRS). To evaluate the efficacy and safety of multistage SRS with treatment of the entire AVM nidus at each treatment session to achieve complete obliteration of high-grade AVMs. Patients with high-grade Spetzler-Martin (S-M) III-V AVMs treated with at least 2 multistage SRS treatments from 1989 to 2013. Clinical outcomes of obliteration rate, minor/major AEs, and treatment characteristics were collected. Forty-two patients met inclusion criteria (n = 26, S-M III; n = 13, S-M IV; n = 3, S-M V) with a median follow-up was 9.5 yr after first SRS. Median number of SRS treatment stages was 2, and median interval between stages was 3.5 yr. Twenty-two patients underwent pre-SRS embolization. Complete AVM obliteration rate was 38%, and the median time to obliteration was 9.7 yr. On multivariate analysis, higher S-M grade was significantly associated ( P = .04) failure to achieve obliteration. Twenty-seven post-SRS AEs were observed, and the post-SRS intracranial hemorrhage rate was 0.027 events per patient year. Treatment of high-grade AVMs with multistage SRS achieves AVM obliteration in a meaningful proportion of patients with acceptable AE rates. Lower obliteration rates were associated with higher S-M grade and pre-SRS embolization. This approach should be considered with caution, as partial obliteration does not protect from hemorrhage. Copyright © 2017 by the Congress of Neurological Surgeons

  5. A Review of Vascular Abnormalities of the Spine.

    PubMed

    Singh, Rahul; Lucke-Wold, Brandon; Gyure, Kymberly; Boo, Sohyun

    2016-01-01

    Patients with spinal vascular lesions present with unique symptoms and have important anatomical and physiologic changes that must be considered prior to treatment. In this mini-review, we provide an overview of normal spinal vascular anatomy and discuss several key spinal vascular lesions. We provide an overview of cavernous malformations, intradural arteriovenous malformations, perimedullary arteriovenous fistulas, and dural arteriovenous fistulas. Important considerations are addressed in terms of pathologic characterization, specific imaging findings, and treatment approaches.

  6. Stereotactic radiosurgery of angiographically occult vascular malformations: Indications and preliminary experience

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Kondziolka, D.; Lunsford, L.D.; Coffey, R.J.

    1990-12-01

    Stereotactic radiosurgery has been shown to treat successfully angiographically demonstrated arteriovenous malformations of the brain. Angiographic obliteration has represented cure and eliminated the risk of future hemorrhage. The role of radiosurgery in the treatment of angiographically occult vascular malformations (AOVMs) has been less well defined. In the initial 32 months of operation of the 201-source cobalt-60 gamma knife at the University of Pittsburgh, 24 patients meeting strict criteria for high-risk AOVMs were treated. Radiosurgery was used conservatively; each patient had sustained two or more hemorrhages and had a magnetic resonance imaging-defined AOVM located in a region of the brain wheremore » microsurgical removal was judged to pose an excessive risk. Venous angiomas were excluded by performance of high-resolution subtraction angiography in each patient. Fifteen malformations were in the medulla, pons, and/or mesencephalon, and 5 were located in the thalamus or basal ganglia. Follow-up ranged from 4 to 24 months. Nineteen patients either improved or remained clinically stable and did not hemorrhage again during the follow-up interval. One patient suffered another hemorrhage 7 months after radiosurgery. Five patients experienced temporary worsening of pre-existing neurological deficits that suggested delayed radiation injury. Magnetic resonance imaging demonstrated signal changes and edema surrounding the radiosurgical target. Dose-volume guidelines for avoiding complications were constructed. Our initial experience indicates that stereotactic radiosurgery can be performed safely in patients with small, well-circumscribed AOVMs located in deep, critical, or relatively inaccessible cerebral locations.« less

  7. Increase in fluoroscopic radiation dose in successive sessions of multistage Onyx embolization of brain arteriovenous malformations compared with the first session.

    PubMed

    Sheen, Jae Jon; Jiang, Yuan Yuan; Kim, Young Eun; Maeng, Jun Young; Kim, Tae-Il; Lee, Deok Hee

    2018-03-23

    Onyx embolization is a treatment for brain arteriovenous malformations (AVMs). However, multistage embolization usually involves the presence of radiodense Onyx cast from the previous sessions, which may influence the fluoroscopic radiation dose. We compared the fluoroscopic dose between the initial and final embolization sessions. From January 2014 to September 2016, 18 patients underwent multistage Onyx embolization (more than twice) for brain AVMs. The total fluoroscopic duration (minutes), dose-area product (DAP, Gy×cm 2 ), and cumulative air kerma (CAK, mGy) of both the frontal and lateral planes were obtained. We compared the frontal and lateral fluoroscopic dose rates (dose/time) of the final embolization session with those of the initial session. The relationship between the injected Onyx volume and radiation dose was tested. The initial and final procedures on the frontal plane showed significantly different fluoroscopic dose rates (DAP: initial 0.668 Gy×cm 2 /min, final 0.848 Gy×cm 2 /min, P=0.02; CAK: initial 12.7 mGy/min, final 23.1 mGy/min, P=0.007). Those on the lateral plane also showed a similar pattern (DAP: initial 0.365 Gy×cm 2 /min, final 0.519 Gy×cm 2 /min, P=0.03; CAK: initial 6.2 mGy/min, final 12.9 mGy/min, P=0.01). The correlation between the cumulative Onyx volume (vials) and radiation dose ratio of both planes showed an increasing trend (rho 0.4325-0.7053; P=0.0011-0.0730). Owing to the automatic exposure control function during fluoroscopy, successive Onyx embolization procedures increase the fluoroscopic radiation dose in multistage brain AVM embolization because of the presence of radiodense Onyx mass. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  8. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 2: Outcomes after stereotactic radiosurgery.

    PubMed

    Starke, Robert M; Ding, Dale; Kano, Hideyuki; Mathieu, David; Huang, Paul P; Feliciano, Caleb; Rodriguez-Mercado, Rafael; Almodovar, Luis; Grills, Inga S; Silva, Danilo; Abbassy, Mahmoud; Missios, Symeon; Kondziolka, Douglas; Barnett, Gene H; Dade Lunsford, L; Sheehan, Jason P

    2017-02-01

    OBJECTIVE Pediatric patients (age < 18 years) harboring brain arteriovenous malformations (AVMs) are burdened with a considerably higher cumulative lifetime risk of hemorrhage than adults. Additionally, the pediatric population was excluded from recent prospective comparisons of intervention versus conservative management for unruptured AVMs. The aims of this multicenter, retrospective cohort study are to analyze the outcomes after stereotactic radiosurgery for unruptured and ruptured pediatric AVMs. METHODS We analyzed and pooled AVM radiosurgery data from 7 participating in the International Gamma Knife Research Foundation. Patients younger than 18 years of age who had at least 12 months of follow-up were included in the study cohort. Favorable outcome was defined as AVM obliteration, no post-radiosurgical hemorrhage, and no permanently symptomatic radiation-induced changes (RIC). The post-radiosurgery outcomes of unruptured versus ruptured pediatric AVMs were compared, and statistical analyses were performed to identify predictive factors. RESULTS The overall pediatric AVM cohort comprised 357 patients with a mean age of 12.6 years (range 2.8-17.9 years). AVMs were previously treated with embolization, resection, and fractionated external beam radiation therapy in 22%, 6%, and 13% of patients, respectively. The mean nidus volume was 3.5 cm 3 , 77% of AVMs were located in eloquent brain areas, and the Spetzler-Martin grade was III or higher in 59%. The mean radiosurgical margin dose was 21 Gy (range 5-35 Gy), and the mean follow-up was 92 months (range 12-266 months). AVM obliteration was achieved in 63%. During a cumulative latency period of 2748 years, the annual post-radiosurgery hemorrhage rate was 1.4%. Symptomatic and permanent radiation-induced changes occurred in 8% and 3%, respectively. Favorable outcome was achieved in 59%. In the multivariate logistic regression analysis, the absence of prior AVM embolization (p = 0.001) and higher margin dose (p < 0

  9. Greek and Roman patients under Galen's gaze: a doctor at the crossroads of two cultures.

    PubMed

    Boudon-Millot, Véronique

    2014-01-01

    Born in Pergamum in 129 A.D., Galen received his first medical training in his native city and then continued his studies in Smyrna, Corinth, and Alexandria. He began his medical career in Asia Minor, treating peasants and performing surgery on the gladiatorial troupe that worked as slaves under the high priest upon his return to Pergamum in 157. Subsequently, he settled in Rome, where he lived most of his life and treated many prominent patients. The aim of this paper is to explore how Galen viewed his Asian and Roman patients and how he adapted his practice and medical procedures based not only on each patient's social and economic status, but on his or her intellectual acumen and customs as well, through proposing an intelligent and original synthesis of Asian and Roman lifestyles.

  10. Onyx is associated with poor venous penetration in the treatment of spinal dural arteriovenous fistulas.

    PubMed

    Blackburn, Spiros L; Kadkhodayan, Yasha; Ray, Wilson Z; Zipfel, Gregory J; Cross, DeWitte T; Moran, Christopher J; Derdeyn, Colin P

    2014-09-01

    The use of Onyx has become the mainstream for the treatment of cranial dural arteriovenous fistulas (AVFs) and arteriovenous malformations, but the reported success for type I spinal dural arteriovenous fistulas (sDAVFs) remains limited. We review our experience with Onyx and report its limitations in the treatment of spinal AVFs. We retrospectively reviewed the Interventional Neuroradiology Procedure database at Washington University for cases of sDAVF embolization. Radiology reports were reviewed for fistula classification, treatment technique, and initial and follow-up results. Angiographic images were reviewed to confirm diagnosis, treatment, and penetration of embolisate into the draining vein. With the use of Onyx, sDAVFs were obliterated in six of seven patients at the time of treatment. Follow-up angiography confirmed sDAVF obliteration in two patients, and recurrence in two cases. Two patients had no follow-up. One patient not cured at the time of treatment was treated surgically. Of the nine total treatments, Onyx successfully crossed the nidus into the draining vein in only four cases. Successful venous embolization was facilitated with positioning of the microcatheter to less than 5 mm from the nidus in three of the four cases. The use of n-butyl cyanoacrylate (NBCA) resulted in venous penetration in eight of 10 cases, and short term follow-up cure in seven of 10 patients. Our experience with Onyx for type I sDAVF embolization has been tempered by difficulty in achieving venous penetration and, consequently, a high rate of recurrence. For management of these fistulas, we favor NBCA or surgical treatment. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  11. A Review of Vascular Abnormalities of the Spine

    PubMed Central

    Singh, Rahul; Lucke-Wold, Brandon; Gyure, Kymberly; Boo, Sohyun

    2017-01-01

    Patients with spinal vascular lesions present with unique symptoms and have important anatomical and physiologic changes that must be considered prior to treatment. In this mini-review, we provide an overview of normal spinal vascular anatomy and discuss several key spinal vascular lesions. We provide an overview of cavernous malformations, intradural arteriovenous malformations, perimedullary arteriovenous fistulas, and dural arteriovenous fistulas. Important considerations are addressed in terms of pathologic characterization, specific imaging findings, and treatment approaches. PMID:28191502

  12. Combining stereotactic angiography and 3D time-of-flight magnetic resonance angiography in treatment planning for arteriovenous malformation radiosurgery.

    PubMed

    Bednarz, G; Downes, B; Werner-Wasik, M; Rosenwasser, R H

    2000-03-15

    This study was initiated to evaluate the advantages of using three-dimensional time-of-flight magnetic resonance angiography (3D TOF MRA), as an adjuvant to conventional stereotactic angiography, in obtaining three-dimensional information about an arteriovenous malformation (AVM) nidus and in optimizing radiosurgical treatment plans. Following angiography, contrast-enhanced MRI and MRA studies were obtained in 22 consecutive patients undergoing Gamma Knife radiosurgery for AVM. A treatment plan was designed, based on the angiograms and modified as necessary, using the information provided by MRA. The quantitative analysis involved calculation of the ratio of the treated volume to the MRA nidus volume (the tissue volume ratio [TVR]) for the initial and final treatment plans. In 12 cases (55%), the initial treatment plans were modified after including the MRA information in the treatment planning process. The mean TVR for the angiogram-based plans was 1.63 (range 1.17-2.17). The mean coverage of the MRA nidus by the angiogram-based plans was 93% (range 73-99%). The mean MRA nidus volume was 2.4 cc (range 0. 6-5.3 cc). The MRA-based modifications resulted in increased conformity with the mean TVR of 1.46 (range 1.20-1.74). These modifications were caused by MRA revealing irregular nidi and/or vascular components superimposed on the angiographic projections of the nidi. In a number of cases, the information from MRA was essential in defining the nidus when the projections of the angiographic outlines showed different superior and/or inferior extent of the nidus. In two cases, MRA revealed irregular nidi, correlating well with the angiograms and showed that the angiographically acceptable plans undertreated 27% of the MRA nidus in one case and 18% of the nidus in the other case. In the remaining 10 cases (45%), both MRI and MRA failed to detect the nidus due to surgical clip artifacts and the presence of embolizing glue. The 3D TOF MRA provided information on irregular

  13. Gamma Knife radiosurgery for cerebral arteriovenous malformations in children/adolescents and adults. Part I: Differences in epidemiologic, morphologic, and clinical characteristics, permanent complications, and bleeding in the latency period

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Nicolato, Antonio; Lupidi, Francesco; Section of Stereotactic and Functional Neurosurgery, University of Verona and University Hospital, Verona

    Purpose: To compare the epidemiologic, morphologic, and clinical characteristics of 92 children/adolescents (Group A) and 362 adults (Group B) with cerebral arteriovenous malformations (cAVMs) considered suitable for radiosurgery; to correlate radiosurgery-related permanent complication and post-radiosurgery bleeding rates in the 75 children/adolescents and 297 adults available for follow-up. Methods and Materials: Radiosurgery was performed with a model C 201-source Co{sup 6} Leksell Gamma Unit (Elekta Instruments, Stockholm, Sweden). Fisher exact two-tailed, Wilcoxon rank-sum, and two-sample binomial exact tests were used for statistical analysis. Results: There were significant differences between the two populations in sex (p = 0.015), clinical presentation (p =more » 0.001), and location (p = 0.008). The permanent complication rate was lower in younger (1.3%) than in older patients (5.4%), although the difference was not significant (p = 0.213). The postradiosurgery bleeding rate was lower in Group A (1.3%) than in Group B (2.7%) (p = 0.694), with global actuarial bleeding rates of 0.56% per year and 1.15% per year, respectively. Conclusions: The different characteristics of child/adolescent and adult cAVMs suggest that they should be considered two distinct vascular disorders. The similar rates of radiosurgery-related complications and latency period bleeding in the two populations show that gamma knife radiosurgery does not expose young patients to a higher risk of sequelae than that for older patients.« less

  14. Chiari Malformation

    MedlinePlus

    ... Staying Safe Videos for Educators Search English Español Chiari Malformation KidsHealth / For Parents / Chiari Malformation What's in this ... balance problems, and other symptoms. What Is a Chiari Malformation? Normally, the cerebellum sits at the back of ...

  15. Risk factors for worsened muscle strength after the surgical treatment of arteriovenous malformations of the eloquent motor area.

    PubMed

    Lin, Fuxin; Zhao, Bing; Wu, Jun; Wang, Lijun; Jin, Zhen; Cao, Yong; Wang, Shuo

    2016-08-01

    OBJECT Case selection for the surgical treatment of arteriovenous malformations (AVMs) of the eloquent motor area remains challenging. The aim of this study was to determine the risk factors for worsened muscle strength after surgery in patients with this disorder. METHODS At their hospital the authors retrospectively studied 48 consecutive patients with AVMs involving motor cortex and/or the descending pathway. All patients had undergone preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI), followed by resection. Both functional and angioarchitectural factors were analyzed with respect to the change in muscle strength. Functional factors included lesion-to-corticospinal tract distance (LCD) on DTI and lesion-to-activation area distance (LAD) and cortical reorganization on fMRI. Based on preoperative muscle strength, the changes in muscle strength at 1 week and 6 months after surgery were defined as short-term and long-term surgical outcomes, respectively. Statistical analysis was performed using the statistical package SPSS (version 20.0.0, IBM Corp.). RESULTS Twenty-one patients (43.8%) had worsened muscle strength 1 week after surgery. However, only 10 patients (20.8%) suffered from muscle strength worsening 6 months after surgery. The LCD was significantly correlated with short-term (p < 0.001) and long-term (p < 0.001) surgical outcomes. For long-term outcomes, patients in the 5 mm ≥ LCD > 0 mm (p = 0.009) and LCD > 5 mm (p < 0.001) categories were significantly associated with a lower risk of permanent motor worsening in comparison with patients in the LCD = 0 mm group. No significant difference was found between patients in the 5 mm ≥ LCD > 0 mm group and LCD > 5 mm group (p = 0.116). Nidus size was the other significant predictor of short-term (p = 0.021) and long-term (p = 0.016) outcomes. For long-term outcomes, the area under the ROC curve (AUC) was 0.728, and the cutoff point was 3.6 cm. Spetzler-Martin grade was not associated with

  16. Deletion of Rbpj from postnatal endothelium leads to abnormal arteriovenous shunting in mice

    PubMed Central

    Nielsen, Corinne M.; Cuervo, Henar; Ding, Vivianne W.; Kong, Yupeng; Huang, Eric J.; Wang, Rong A.

    2014-01-01

    Arteriovenous malformations (AVMs) are tortuous vessels characterized by arteriovenous (AV) shunts, which displace capillaries and shunt blood directly from artery to vein. Notch signaling regulates embryonic AV specification by promoting arterial, as opposed to venous, endothelial cell (EC) fate. To understand the essential role of endothelial Notch signaling in postnatal AV organization, we used inducible Cre-loxP recombination to delete Rbpj, a mediator of canonical Notch signaling, from postnatal ECs in mice. Deletion of endothelial Rbpj from birth resulted in features of AVMs by P14, including abnormal AV shunting and tortuous vessels in the brain, intestine and heart. We further analyzed brain AVMs, as they pose particular health risks. Consistent with AVM pathology, we found cerebral hemorrhage, hypoxia and necrosis, and neurological deficits. AV shunts originated from capillaries (and possibly venules), with the earliest detectable morphological abnormalities in AV connections by P8. Prior to AV shunt formation, alterations in EC gene expression were detected, including decreased Efnb2 and increased Pai1, which encodes a downstream effector of TGFβ signaling. After AV shunts had formed, whole-mount immunostaining showed decreased Efnb2 and increased Ephb4 expression within AV shunts, suggesting that ECs were reprogrammed from arterial to venous identity. Deletion of Rbpj from adult ECs led to tortuosities in gastrointestinal, uterine and skin vascular beds, but had mild effects in the brain. Our results demonstrate a temporal requirement for Rbpj in postnatal ECs to maintain proper artery, capillary and vein organization and to prevent abnormal AV shunting and AVM pathogenesis. PMID:25209249

  17. Quality of life and disability 12 months after surgery vs. conservative management for unruptured brain arteriovenous malformations: Scottish population-based and Australian hospital-based studies.

    PubMed

    O'Donnell, Joan Margaret; Al-Shahi Salman, Rustam; Manuguerra, Maurizio; Assaad, Nazih; Morgan, Michael Kerin

    2018-03-01

    Few data are available on disability and quality of life (QOL) after surgery versus conservative management for unruptured brain arteriovenous malformations (uAVMs). The aim of this study was to test the hypothesis that QOL and disability are worse after surgery ± preoperative embolisation for uAVM compared with conservative management. We included consecutive patients diagnosed with uAVM from a prospective population-based study in Scotland (1999-2003; 2006-2010) and a prospective hospital-based series in Australia (2011-2015). We assessed outcomes on the modified Rankin Scale (mRS) and the Short Form (SF)-36 at ~ 12 months after surgery or conservative treatment and compared these groups using continuous ordinal regression in the two cohorts separately. Surgery was performed for 29% of all uAVM cases diagnosed in Scotland and 84% of all uAVM referred in Australia. There was no statistically significant difference between surgery and conservative management at 12 months among 79 patients in Scotland (mean SF-36 Physical Component Score (PCS) 39 [SD 14] vs. 39 [SD 13]; mean SF-36 Mental Component Score (MCS) 38 [SD 14] vs. 39 [SD 14]; mRS > 1, 24 vs. 9%), nor among 37 patients in Australia (PCS 51 [SD 10] vs. 49 [SD 6]; MCS 48 [SD 12] vs. 49 [SD 10]; mRS > 1, 19 vs. 30%). In the Australian series, there was no statistically significant change in the MCS and PCS between baseline before surgery or conservative management and 12 months. We did not find a statistically significant difference between surgery ± preoperative embolisation and conservative management in disability or QOL at 12 months.

  18. Hot heads and cold brains. Aristotle, Galen and the "radiator theory".

    PubMed

    Longo, O

    1996-01-01

    The Author examines two similar theories about the functioning of human brain as a refrigerator: Falk's and Fialkowski's (1990) and Aristotle's (IVth century b.C.). There are surprising, although fortuitous, convergences between the two, with the remarkable difference, however, that Artistotle's doctrine (later severely criticized by Galen) thinks of the brain merely as an organ for the cooling of the body's (the heart's) heat, while according to the modern radiator theory the human brain developed starting as a refrigerator of itself.

  19. Volumetric Modulated Arc-Based Hypofractionated Stereotactic Radiotherapy for the Treatment of Selected Intracranial Arteriovenous Malformations: Dosimetric Report and Early Clinical Experience

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Subramanian, Sai; Srinivas, Chilukuri; Ramalingam, K.

    2012-03-01

    Purpose: To evaluate, with a dosimetric and clinical feasibility study, RapidArc (a volumetric modulated arc technique) for hypofractionated stereotactic radiotherapy treatment of large arteriovenous malformations (AVMs). Methods and Materials: Nine patients were subject to multimodality imaging (magnetic resonance, computed tomography, and digital subtraction angiography) to determine nidus and target volumes, as well as involved organs at risk (optical structures, inner ear, brain stem). Plans for multiple intensity-modulated arcs with a single isocenter were optimized for a fractionation of 25 Gy in 5 fractions. All plans were optimized for 6-MV photon beams. Dose-volume histograms were analyzed to assess plan quality. Deliverymore » parameters were reported to appraise technical features of RapidArc, and pretreatment quality assurance measurements were carried out to report on quality of delivery. Results: Average size of AVM nidus was 26.2 cm{sup 3}, and RapidArc plans provided complete target coverage with minimal overdosage (V{sub 100%} = 100% and V{sub 110%} < 1%) and excellent homogeneity (<6%). Organs at risk were highly spared. The D{sub 1%} to chiasm, eyes, lenses, optic nerves, and brainstem (mean {+-} SD) was 6.4 {+-} 8.3, 1.9 {+-} 3.8, 2.3 {+-} 2.2, 0.7 {+-} 0.9, 4.4 {+-} 7.2, 12.2 {+-} 9.6 Gy, respectively. Conformity index (CI{sub 95%}) was 2.2 {+-} 0.1. The number of monitor units per gray was 277 {+-} 45, total beam-on time was 2.5 {+-} 0.3 min. Planning vs. delivery {gamma} pass rate was 98.3% {+-} 0.9%. None of the patients developed acute toxicity. With a median follow-up of 9 months, 3 patients presented with deterioration of symptoms and were found to have postradiation changes but responded symptomatically to steroids. These patients continue to do well on follow-up. One patient developed headache and seizures, which was attributed to intracranial bleed, confirmed on imaging. Conclusion: Hypofractionated stereotactic radiotherapy can be

  20. Amphibian malformations

    USGS Publications Warehouse

    ,

    1998-01-01

    Frog malformations have been reported from 42 states. The broad geographic distribution of these malformations warrants national attention. Scientists at the USGS National Wildlife Health Center in Madison, Wisconsin are studying this problem in an effort to document its scope and to determine the causes of the observed malformations.

  1. Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation.

    PubMed

    Moore, Hannah E; Moore, Kevin R

    2014-11-01

    Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords "Chiari" and "Chiari 1" to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in those with Chiari 1 malformation

  2. Effectiveness of Preradiosurgical Embolization with NBCA for Arteriovenous Malformations - Retrospective Outcome Analysis in a Japanese Registry of 73 Patients (J-REAL study)

    PubMed Central

    Izumi, Takashi; Satow, Tetsu; Srivatanakul, Kittipong; Matsumoto, Yasushi; Terada, Tomoaki; Matsumaru, Yuji; Kiyosue, Hiro

    2017-01-01

    Purpose Recent reports have posed doubts about the effect of preradiosurgical embolization in brain arteriovenous malformation (AVM) because it makes the planning of stereotactic radiosurgery (SRS) difficult and has the risk of recanalization out of the target. We investigated whether the performance and quality of embolization may influence the success of SRS based on a retrospective case cohort study. Materials and Methods Seventy-three patients who underwent embolization followed by SRS between 2003 and 2012 in eight institutes with neurointerventionists were considered. They were divided into the following two groups at 3 years of follow up after the final SRS: “successful occlusion group” (S group), with radiologically complete occlusion of AVM; and “non-successful occlusion group” (N group) with persistent remnant nidus or abnormal vascular networks. Patient background, AVM profile, embolization performance grade and complications were compared in each group. The quality of embolization was evaluated with the new grading system: embolization performance grade (E grade), specializing the achievement of nidus embolization. E grade A was defined as sufficient nidus embolization with more than half of the total number of feeders achieving nidus penetration. E grade B was defined as less than half achievement of nidus embolization, and E grade C was defines as failure to perform nidus embolization. Results Forty-three patients were included in the S group, and 29 patients were included in the N group. The size and Spetzler-Martin grade of AVM and the rate of diffuse type was higher in the N group without statistical significance. The embolization performance level according to E grade indicated a significantly higher rate of successful embolization with more than 50% of nidus penetration in the S group (P<0.001). This difference was also confirmed in the subanalysis for limited cases, excluding smaller AVMs with complete occlusion with SRS alone (P=0

  3. Physical examination of the hemodialysis arteriovenous fistula to detect early dysfunction.

    PubMed

    Abreo, Kenneth; Amin, Bakhtiar M; Abreo, Adrian P

    2018-04-01

    The maintenance of vascular access patency for end-stage renal disease patients on hemodialysis is necessary for survival. Many nephrologists, nurse practitioners, and nurses have limited experience with the physical examination of the arteriovenous fistula. In this review, we define key terms used in the assessment of an arteriovenous fistula. We discuss the arteriovenous fistula physical exam, including details of inspection, palpation, and auscultation. Using these concepts, we review the abnormal findings that can assist practitioners in determining the location of a stenosis. We review the existing literature that validates physical exam findings with gold standard tests such as ultrasound and angiography. Finally, we review data supporting the value of training physicians and nurses in arteriovenous fistula physical examination.

  4. Endovascular management of sigmoid sinus dural arteriovenous fistula associated with sinus stenosis in an infant.

    PubMed

    Cohen, José E; Gomori, John M; Benifla, Moni; Itshayek, Eyal; Moscovici, Samuel

    2013-01-01

    A 4-month-old female presented with a dural arteriovenous fistula (DAVF), which was successfully managed using endovascular techniques. There are very few case series reporting DAVF in infants younger than 12 months and, to our knowledge, only 60 pediatric patients with DAVF have been reported to date. Although most DAVF have a benign course, they can result in life-threatening hemorrhage. Endovascular therapies are usually indicated in the management of these neurosurgical vascular malformations. Endovascular therapy of DAVF in neonatal patients presents some major issues. Gaining arterial access may be problematic in femoral arteries too small for the introduction of a sizeable guiding catheter. The volumes of contrast and infused fluids must be carefully monitored to prevent fluid overload. Radiation exposure should be restricted as far as possible. This report contributes to the limited body of evidence on neonatal DAVF and its endovascular management. Copyright © 2012 Elsevier Ltd. All rights reserved.

  5. [The current approach to hemangiomas and vascular malformations of the head and neck].

    PubMed

    Raveh, E; Waner, M; Kornreich, L; Segal, K; Ben-Amitai, D; Kalish, E; Lapidot, M; Mimon, S; Shalev, B; Feinmesser, R

    2002-09-01

    Though most hemangiomas do not need treatment, a significant minority are associated with complications and external deformities that demand intervention. Steroids play an important role in therapy, but not infrequently afford only partial and temporary benefit. Thanks to improvements in the surgical approach and equipment, hemostasis control devices and laser techniques, we can now treat patients who would otherwise go untreated. Moreover, in certain cases, we can now recommend earlier intervention, saving patients from years of living with deformities and the concomitant psychosocial problems. Vascular anomalies of the head and neck include venular, venous and arteriovenous malformations. These lesions are slow growing vascular ectasia that never involute spontaneously and almost always require intervention. Treatment includes laser therapy, injection of sclerosing agents, embolization through angiography and surgery, which in many cases is the only definitive treatment. We present the current treatment approach and describe our experience in the treatment of 16 patients.

  6. Definition of the key target volume in radiosurgical management of arteriovenous malformations: a new dynamic concept based on angiographic circulation time.

    PubMed

    Valle, Ramiro Del; Zenteno, Marco; Jaramillo, José; Lee, Angel; De Anda, Salvador

    2008-12-01

    The cumulative experience worldwide indicates complete radiosurgical obliteration rates of brain arteriovenous malformations (AVMs) ranging from 35 to 90%. The purpose of this study was to propose a strategy to increase the obliteration rate for AVMs through the dynamic definition of the key target volume (KTV). A prospective series of patients harboring an AVM was assessed using digital subtraction angiography in which a digital counter was used to measure the several stages of the frame-by-frame circulation time. All the patients were analyzed using dynamic measurement planning to define the KTV, corresponding to the volume of the shunt with the least vascular resistance and the earliest venous drainage. All patients underwent catheter-based angiography, a subgroup was additionally assessed by means of a superselective catheterization, and among these a further subgroup received embolization. The shunts were also categorized according to their angioarchitectural type: fistulous, plexiform, or mixed. The authors applied the radiosurgery-based grading system (RBGS) as well to find a correlation with the obliteration rate. This series includes 44 patients treated by radiosurgery; global angiography was performed for all patients, including dynamic measurement planning. Eighty-four percent of them underwent superselective catheterization, and 50% of the total population underwent embolization. In the embolized arm of the study, the pretreatment volume was up to 120 ml. In patients with a single treatment, the mean volume was 8.5 ml, and the median volume was 6.95 +/- 4.56 ml (mean +/- standard deviation), with a KTV of up to 15 ml. For prospectively staged radiosurgery, the mean KTV was 28 ml. The marginal radiation dose was 18-22 Gy, with a mean of dose 20 Gy. The mean RBGS score was 1.70. The overall obliteration rate was 91%, including the repeated radiosurgery group (4 patients), in which 100% showed complete obliteration. The overall permanent deficit was 2 of

  7. Successful treatment of acquired uterine arterial venous malformation using N-butyl-2-cyanoacrylate under balloon occlusion

    PubMed Central

    Ogasawara, Go; Ishida, Kenichiro; Fujii, Kaoru; Yamane, Takuro; Nishimaki, Hiroshi; Matsunaga, Keiji; Inoue, Yusuke

    2014-01-01

    We present two cases of acquired uterine arterial venous malformation (AVM) which was diagnosed because of massive genital bleeding successfully treated with transcatheter arterial embolization (TAE), using N-butyl-2-cyanoacrylate (NBCA) under balloon occlusion. Balloon occlusion at the uterine artery was performed in both patients for diffuse distribution of NBCA in multiple feeding branches, as well as to the pseudoaneurysm, and for the prevention of NBCA reflux. In one of our patients, balloon occlusion of the draining vein was simultaneously performed to prevent NBCA migration through accompanying high-flow arteriovenous fistula (AVF). Doppler ultrasound at 6 months of both patients documented persistent complete occlusion of AVM. Complete and safe obliteration of acquired uterine AVM was accomplished using NBCA as embolic agent, under balloon occlusion at the communicating vessels of acquired uterine AVM. PMID:25346850

  8. Superficial temporal arteriovenous fistula as a complication of rhytidectomy.

    PubMed

    Kominami, Shushi; Watanabe, Akira; Akimoto, Masahiro; Kobayashi, Shiro; Teramoto, Akira

    2012-03-01

    A 67-year-old woman who had undergone rhytidectomy 5 years before her presentation experienced increasing pulsatile tinnitus on the left side that had begun 2 years earlier. Angiography revealed a direct arteriovenous fistula between the superficial temporal artery and superficial temporal vein in front of her left ear. There was a scar from the earlier cosmetic surgery at the site. The fistula was embolized with N-butyl cyanoacrylate, and her tinnitus disappeared. We posit that the fistula was a complication of rhytidectomy and that a small arteriovenous fistula formed at the time of surgery and enlarged over time. This case indicates that arteriovenous fistulae can occur as a delayed complication of cosmetic surgery.

  9. Iatrogenic arteriovenous fistula of the superficial temporal artery after reduction malarplasty.

    PubMed

    Kim, Jun Hyuk; Yoon, Seok Mann; Choi, Hwan Jun

    2015-01-01

    A 34-year-old woman who had a history of undergoing reduction malarplasty at a local clinic about 1 year ago developed gradually increasing pulsatile tinnitus in the right preauricular area since the last 6 months. On physical examination, there were an approximately 1 × 0.5-cm nontender, soft, pulsatile mass with a palpable thrill and a continuous machinery-like buzzing sound in synchrony with the heartbeat. She had a fine scar near the mass, which was supposed to be a postoperative scar of reduction malarplasty. A three-dimensional computed tomographic angiogram revealed a direct arteriovenous fistula between the superficial temporal artery and superficial temporal vein in the right preauricular area. The arteriovenous fistula was embolized using Tornado coils. After coiling, the thrill and disturbing tinnitus disappeared immediately, and postembolization angiography confirmed obliteration of the arteriovenous shunt. This is the first case of an arteriovenous fistula of the superficial temporal artery after reduction malarplasty, and it indicates that arteriovenous fistula can occur as a delayed complication of reduction malarplasty.

  10. Percutaneous Creation of a Central Iliac Arteriovenous Anastomosis for the Treatment of Arterial Hypertension.

    PubMed

    Bertog, Stefan C; Sobotka, Nathan A; Sobotka, Paul A; Lobo, Melvin D; Sievert, Kolja; Vaskelyte, Laura; Sievert, Horst; Schmieder, Roland E

    2018-03-19

    Provision of a summary on the physiologic effects of arteriovenous fistula creation and description of previously published human data on the efficacy of a percutaneously implanted device creating an arteriovenous fistula. Though antihypertensive therapy is effective, some patient's blood pressure remains poorly controlled despite adherence to optimal medical therapy. Moreover, some patients are not compliant with recommended medical therapy due to side effects or personal decision. This has prompted exploration of alternative, device-based antihypertensive therapies including, among others, the percutaneous creation of an arteriovenous fistula. An arteriovenous fistula is accompanied by a number of favorable physiologic changes that may lower blood pressure. These physiologic changes, conduction of the procedure, and previously published human experience are summarized in this review article. The results of a recently published trial comparing arteriovenous fistula creation and standard antihypertensive therapy versus standard antihypertensive therapy alone are summarized. Creation of an arteriovenous fistula is accompanied by a significant blood pressure reduction likely related to a reduction in total arterial resistance, perhaps blood volume reduction, inhibition of the baroreceptor reflex, and release of natriuretic peptides. These findings foster further interest in studying the impact of an arteriovenous fistula and arterial blood pressure. The design of a large randomized trial comparing arteriovenous fistula creation to sham control is outlined.

  11. Live-cell imaging to detect phosphatidylserine externalization in brain endothelial cells exposed to ionizing radiation: implications for the treatment of brain arteriovenous malformations.

    PubMed

    Zhao, Zhenjun; Johnson, Michael S; Chen, Biyi; Grace, Michael; Ukath, Jaysree; Lee, Vivienne S; McRobb, Lucinda S; Sedger, Lisa M; Stoodley, Marcus A

    2016-06-01

    OBJECT Stereotactic radiosurgery (SRS) is an established intervention for brain arteriovenous malformations (AVMs). The processes of AVM vessel occlusion after SRS are poorly understood. To improve SRS efficacy, it is important to understand the cellular response of blood vessels to radiation. The molecular changes on the surface of AVM endothelial cells after irradiation may also be used for vascular targeting. This study investigates radiation-induced externalization of phosphatidylserine (PS) on endothelial cells using live-cell imaging. METHODS An immortalized cell line generated from mouse brain endothelium, bEnd.3 cells, was cultured and irradiated at different radiation doses using a linear accelerator. PS externalization in the cells was subsequently visualized using polarity-sensitive indicator of viability and apoptosis (pSIVA)-IANBD, a polarity-sensitive probe. Live-cell imaging was used to monitor PS externalization in real time. The effects of radiation on the cell cycle of bEnd.3 cells were also examined by flow cytometry. RESULTS Ionizing radiation effects are dose dependent. Reduction in the cell proliferation rate was observed after exposure to 5 Gy radiation, whereas higher radiation doses (15 Gy and 25 Gy) totally inhibited proliferation. In comparison with cells treated with sham radiation, the irradiated cells showed distinct pseudopodial elongation with little or no spreading of the cell body. The percentages of pSIVA-positive cells were significantly higher (p = 0.04) 24 hours after treatment in the cultures that received 25- and 15-Gy doses of radiation. This effect was sustained until the end of the experiment (3 days). Radiation at 5 Gy did not induce significant PS externalization compared with the sham-radiation controls at any time points (p > 0.15). Flow cytometric analysis data indicate that irradiation induced growth arrest of bEnd.3 cells, with cells accumulating in the G2 phase of the cell cycle. CONCLUSIONS Ionizing radiation

  12. The breast: from Ancient Greek myths to Hippocrates and Galen.

    PubMed

    Iavazzo, C R; Trompoukis, C; Siempos, I I; Falagas, M E

    2009-01-01

    This is a historical article about Ancient Greek literature from mythological times until the first centuries AD with regard to the female breast. We endeavoured to collect several elegant narratives on the topic as well as to explore the knowledge of Ancient Greek doctors on the role, physiology and pathology of breast and the treatment of its diseases. We identified such descriptions in myths regarding Amazons, Hercules, Zeus, Hera and Amaltheia. Furthermore, descriptions on the topic were also found in the work of Hippocrates, Aristoteles, Soranos, Alexander of Aphrodisias, Celsus, Archigenis, Leonides, Galen and Oribasius. We may conclude that some of today's medical knowledge or practice regarding the breast was also known in the historical period.

  13. Intraoperative direct puncture and embolization (IOPE) using a glue material for spinal cord arteriovenous fistula: a case report.

    PubMed

    Shin, Hong Kyung; Suh, Dae Chul; Jeon, Sang Ryong

    2015-05-01

    Spinal arteriovenous fistula (AVF) is treated by embolization or surgery. However, transarterial embolization or surgery is difficult in rare cases when the fistula site is very complicated to access especially as in fistular nidus supplied by posterior and anterior spinal artery. We present the case which was treated with intraoperative direct puncture and embolization (IOPE) using glue material, since the usual transarterial or transvenous neurointerventional approach was difficult to embolize the AVF. A 36-year-old woman presented with progressive leg weakness and pain after a 20-year history of lower back pain. She had pelvic and spinal AVF combined with arteriovenous malformation (AVM). Despite prior treatment of the pelvic lesion with radiotherapy and coil embolization, the spinal lesion persisted and caused repeated subarachnoid hemorrhages. A spinal angiogram revealed a tortuous and long feeder of the AVF which had growing venous sac, as well as AVM. Two embolization trials failed because of the long tortuosity and associated anterior spinal artery. Four months later, drastic leg weakness and pain occurred, and IOPE was performed using a glue material. The subsequent recovery of the patient was rapid. One month later, the use of a strong opioid could be discontinued, and the patient could walk with aid. A follow-up spinal angiogram revealed that the venous sac of the AVF had disappeared. In spinal AVF which is not feasible to access by usual intervention approach and to dissect surgically, IOPE with glue material can be considered for the treatment.

  14. Transcatheter Amplatzer vascular plug-embolization of a giant postnephrectomy arteriovenous fistula combined with an aneurysm of the renal pedicle by through-and-through, arteriovenous access

    PubMed Central

    Kayser, Ole; Schäfer, Philipp

    2013-01-01

    Although endovascular transcatheter embolization of arteriovenous fistulas is minimally invasive, the torrential flow prevailing within a fistula implies the risk of migration of the deployed embolization devices into the downstream venous and pulmonary circulation. We present the endovascular treatment of a giant postnephrectomy arteriovenous fistula between the right renal pedicle and the residual renal vein in a 63-year-old man. The purpose of this case report is to demonstrate that the Amplatzer vascular plug (AVP) can be safely positioned to embolize even relatively large arteriovenous fistulas (AVFs). Secondly, we illustrate that this occluder can even be introduced to the fistula via a transvenous catheter in cases where it is initially not possible to advance the deployment-catheter through a tortuous feeder artery. Migration of the vascular plug was ruled out at follow-up 4 months subsequently to the intervention. Thus, the Amplatzer vascular plug and the arteriovenous through-and-through guide wire access with subsequent transvenous deployment should be considered in similar cases. PMID:23326248

  15. Neurological morbidity and mortality associated with the endovascular treatment of cerebral arteriovenous malformations before and during the Onyx era.

    PubMed

    Crowley, R Webster; Ducruet, Andrew F; Kalani, M Yashar S; Kim, Louis J; Albuquerque, Felipe C; McDougall, Cameron G

    2015-06-01

    The widespread implementation of the embolic agent Onyx has changed the endovascular management of cerebral arteriovenous malformations (AVMs). Recent data suggest that outcomes following embolization and resection may have worsened in the Onyx era. It has been hypothesized that there may be increased complications with Onyx embolization and increased surgical aggressiveness in patients treated with Onyx. In this study the authors analyzed their institutional experience with the endovascular treatment of cerebral AVMs prior to and after the introduction of Onyx to determine factors associated with periprocedural neurological morbidity and mortality. A retrospective review was performed of all patients with cerebral AVMs undergoing embolization at the Barrow Neurological Institute from 1995 to 2012. Endovascular treatment of 342 cerebral AVMs was performed over 446 treatment sessions (mean age 37.8 years, range 1-83 years). Clinical presentation included hemorrhage in 47.6%, seizures in 21.9%, headaches in 11.1%, and no symptoms in 10% of cases. The endovascular pretreatment strategy was preoperative in 78.9%, preradiosurgery in 9.1%, palliative in 5.3%, targeted in 4.4%, and curative in 2.3%. The median Spetzler-Martin grade was III. The mean number of arteries embolized was 3.5 (range 0-13 arteries), and the mean number of treatment sessions was 1.3 (range 1-4 sessions). Onyx was used in 105 AVMs (30.7%), and N-butyl cyanoacrylate (NBCA) without Onyx was used in 229 AVMs (67%). AVMs treated with Onyx had a higher mean number of arterial pedicles embolized than did NBCA cases (4.3 ± 2.7 vs 3.2 ± 2.4, respectively; p < 0.001) and a greater number of sessions (1.5 ± 0.7 vs 1.2 ± 0.5, respectively; p < 0.05). Unexpected immediate postprocedural permanent neurological deficits were present in 9.6% of AVMs, while transient deficits were present in 1.8%. There was 1 death (0.3%). Spetzler-Martin grade was not associated with differences in outcome, as permanent

  16. Brain AVM (Arteriovenous Malformation)

    MedlinePlus

    ... of Privacy Practices Notice of Nondiscrimination Manage Cookies Advertising Mayo Clinic is a not-for-profit organization and proceeds from Web advertising help support our mission. Mayo Clinic does not ...

  17. De novo development of dural arteriovenous fistula after endovascular embolization of pial arteriovenous fistula.

    PubMed

    Paramasivam, Srinivasan; Toma, Naoki; Niimi, Yasunari; Berenstein, Alejandro

    2013-07-01

    The development of de novo dural arteriovenous fistula(s) following endovascular embolization of a prior high-flow pial arteriovenous fistula (PAVF) has not previously been reported and the natural history is unknown. The anatomic basis, pathophysiologic mechanism, management and outcome are discussed. Treatment-completed congenital PAVFs treated at our center between January 2005 and August 2011 were analyzed retrospectively. Among 16 cases of PAVFs treated by endovascular embolization, four developed de novo dural arteriovenous fistulas during treatment or on follow-up that were not present before treatment. Information was collected from the clinical case records, imaging by MRI on presentation and during follow-up, all angiographic images and records during each of the procedures and during follow-up. The time interval between the last embolization and identification of a dural fistula ranged from 3 to 14 months. Ten fistulas were identified in four patients, seven of which were embolized, four with glue, two with Onyx18 and one with absolute alcohol. None recanalized, while one patient developed fistula in an adjacent location that was subsequently treated with radiosurgery. Not all fistulas need treatment; small fistulas with a minimal flow can safely be observed. De novo dural fistulas following endovascular embolization of high-flow PAVFs is not an uncommon development. They are mostly asymptomatic and develop anywhere along the drainage of the fistula, maturing over time and diagnosed during follow-up studies, emphasizing the need for follow-up angiography. They can be effectively treated by endovascular embolization. Localized refractory dural fistulas can be dealt with by radiosurgery.

  18. Reflections on religious belief and prosociality: Comment on Galen (2012).

    PubMed

    Myers, David G

    2012-09-01

    Luke Galen (2012) offers a timely analysis of associations between religiosity and prosocial and antisocial attitudes and behaviors. After identifying 10 points of agreement, I raise 8 questions for further reflection and research: (1) Is ingroup giving and volunteerism not prosocial? (2) Are religion-related prosocial norms part of the religious factor? (3) Is social support also appropriately considered part of the religious factor? (4) Are self-report data from more and less religious people invalid? (5) How should we disentangle gender and religiosity? (6) How might we resolve "the religious engagement paradox"? (7) Does religion serve an adaptive, evolutionary function? And (8) Might research further explore religiosity, in its varieties, and prosociality? (PsycINFO Database Record (c) 2012 APA, all rights reserved).

  19. Contralateral posterior interhemispheric approach to deep medial parietooccipital vascular malformations: surgical technique and results.

    PubMed

    Burkhardt, Jan-Karl; Winkler, Ethan A; Lawton, Michael T

    2017-07-21

    OBJECTIVE Deep medial parietooccipital arteriovenous malformations (AVMs) and cerebral cavernous malformations (CCMs) are traditionally resected through an ipsilateral posterior interhemispheric approach (IPIA), which creates a deep, perpendicular perspective with limited access to the lateral margins of the lesion. The contralateral posterior interhemispheric approach (CPIA) flips the positioning, with the midline positioned horizontally for retraction due to gravity, but with the AVM on the upper side and the approach from the contralateral, lower side. The aim of this paper was to analyze whether the perpendicular angle of attack that is used in IPIA would convert to a parallel angle of attack with the CPIA, with less retraction, improved working angles, and no significant increase in risk. METHODS A retrospective review of pre- and postoperative clinical and radiographic data was performed in 8 patients who underwent a CPIA. RESULTS Three AVMs and 5 CCMs were resected using the CPIA, with an average nidus size of 2.3 cm and CCM diameter of 1.7 cm. All lesions were resected completely, as confirmed on postoperative catheter angiography or MRI. All patients had good neurological outcomes, with either stable or improved modified Rankin Scale scores at last follow-up. CONCLUSIONS The CPIA is a safe alternative approach to the IPIA for deep medial parietooccipital vascular malformations that extend 2 cm or more off the midline. Contralaterality and retraction due to gravity optimize the interhemispheric corridor, the surgical trajectory to the lesion, and the visualization of the lateral margin, without resection or retraction of adjacent normal cortex. Although the falx is a physical barrier to accessing the lesion, it stabilizes the ipsilateral hemisphere while gravity delivers the dissected lesion through the transfalcine window. Patient positioning, CSF drainage, venous preservation, and meticulous dissection of the deep margins are critical to the safety of

  20. [Polythelia and renal malformation].

    PubMed

    Jójárt, G; Seres, E

    1992-07-12

    The authors found 241 polythelia (5.86) among 4113 schoolchildren (aged 6-14 years). They investigated 236 of the 241 with ultrasound and found 10 renal malformations (4.24%). Among 280 controls with respiratory infection, accident or tonsillectomy they found 9 renal malformations (3.21%). With screening of 1635 neonates they found 66 with accessory nipples (4.05%). Two of the 66 had renal malformations (3.03%), while among the 1957 control neonates 37 had renal malformations (1.89%). In the hospital and ambulancy the authors found 106 polythelia, five of them had renal abnormalities (4.72%). The authors did not found association of polythelia and renal malformation with ultrasound investigation of 408 children with polythelia.

  1. A regional survey of malformed frogs in Minnesota (USA) (Minnesota malformed frogs).

    PubMed

    Vandenlangenberg, Susan M; Canfield, Jeffrey T; Magner, Joseph A

    2003-02-01

    In late 1995, school children discovered malformed frogs in a south central Minnesota pond. Press coverage resulted in numerous citizen reports of frog malformation across Minnesota in 1996. After some initial site investigation, 3 affected frog sites and 4 nearby reference sites were selected for more detailed evaluation. Field biologists made 89 visits to study sites beginning spring 1997 through fall 1999 to examine the number and type of frog malformations. Over 5,100 Leopard frogs (Rana pipiens) were captured and examined at all study sites. Water elevations and associated littoral inundation were recorded from 1997-2000. Results indicate that malformation occurred at all study sites above historical background levels. Rana pipiens malformation across all sites over three seasons averaged 7.9% and ranged from 0 to 7% at reference sites and 4 to 23% at affected sites. At one northern Minnesota site, mink frog (Rana septentrionalis) malformation was 75% in 1998. A site characteristic common to the most affected sites was an elastic zone of littoral inundation. Climate driven hydrologic variation likely influenced water depth and associated breeding locations.

  2. Amplatzer vascular plug for arteriovenous hemodialysis access occlusion: initial experience.

    PubMed

    Bui, J T; Gaba, R C; Knuttinen, M G; West, D L; Owens, C A

    2009-01-01

    The Amplatzer Vascular Plug (AVP; AGA Medical, Golden Valley, MN) is a recently developed self-expanding metallic device indicated for peripheral vascular embolizations. Herein, we describe use of this device in the treatment of vascular complications related to arteriovenous hemodialysis fistulas and grafts. This HIPAA compliant retrospective study was approved by the institutional review board with informed consent waived. Six patients with problematic arteriovenous access underwent access occlusion using the AVP. Procedure indications included vascular steal syndrome in five patients, and enlarging vascular aneurysms in one patient. Contraindications for surgical correction were determined by the referring surgeon. AVP embolizations were performed using devices oversized by 50% introduced through vascular sheaths positioned within vein segments just beyond the arteriovenous anastomoses. Noninvasive evaluation of the involved extremity was performed pre- and post-embolization in addition to clinical follow-up examinations. Measured outcomes included success of angiographic occlusion, improvement in distal arterial flow, AVP number, AVP diameter, time to access occlusion, and clinical symptomatic improvement. Technical success was 100%, with complete arteriovenous access occlusion accomplished in all cases, with an average of 1.5 AVPs used per patient. Mean time to access occlusion was 19.3 minutes. Angiographic improvement in distal arterial flow was immediately evident and resolution of clinical symptoms occurred in all patients, with mean long-term follow-up of 16 months. No procedure-related complications were encountered. The Amplatzer Vascular Plug provides a minimally invasive and efficacious method for embolization of problematic arteriovenous hemodialysis access.

  3. PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia.

    PubMed

    Ola, Roxana; Dubrac, Alexandre; Han, Jinah; Zhang, Feng; Fang, Jennifer S; Larrivée, Bruno; Lee, Monica; Urarte, Ana A; Kraehling, Jan R; Genet, Gael; Hirschi, Karen K; Sessa, William C; Canals, Francesc V; Graupera, Mariona; Yan, Minhong; Young, Lawrence H; Oh, Paul S; Eichmann, Anne

    2016-11-29

    Activin receptor-like kinase 1 (ALK1) is an endothelial serine-threonine kinase receptor for bone morphogenetic proteins (BMPs) 9 and 10. Inactivating mutations in the ALK1 gene cause hereditary haemorrhagic telangiectasia type 2 (HHT2), a disabling disease characterized by excessive angiogenesis with arteriovenous malformations (AVMs). Here we show that inducible, endothelial-specific homozygous Alk1 inactivation and BMP9/10 ligand blockade both lead to AVM formation in postnatal retinal vessels and internal organs including the gastrointestinal (GI) tract in mice. VEGF and PI3K/AKT signalling are increased on Alk1 deletion and BMP9/10 ligand blockade. Genetic deletion of the signal-transducing Vegfr2 receptor prevents excessive angiogenesis but does not fully revert AVM formation. In contrast, pharmacological PI3K inhibition efficiently prevents AVM formation and reverts established AVMs. Thus, Alk1 deletion leads to increased endothelial PI3K pathway activation that may be a novel target for the treatment of vascular lesions in HHT2.

  4. Radial Artery Approach to Salvage Nonmaturing Radiocephalic Arteriovenous Fistulas

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hsieh, Mu-Yang; Lin, Lin; Tsai, Kuei-Chin

    2013-08-01

    PurposeTo evaluate the usefulness of an approach through the radial artery distal to the arteriovenous anastomosis for salvaging nonmaturing radiocephalic arteriovenous fistulas.MethodsProcedures that fulfilled the following criteria were retrospectively reviewed: (1) autogenous radiocephalic fistulas, (2) fistulas less than 3 months old, (3) distal radial artery approach for salvage. From 2005 to 2011, a total of 51 patients fulfilling the above criteria were enrolled. Outcome variables were obtained from angiographic, clinical and hemodialysis records, including the success, complication, and primary and secondary patency rates.ResultsThe overall anatomical and clinical success rates for the distal radial artery approach were 96 and 94 %,more » respectively. The average procedure time was 36 {+-} 19 min. Six patients (12 %) experienced minor complications as a result of extravasations. No arterial complication or puncture site complication was noted. The postinterventional 6-month primary patency rate was 51 %, and the 6-month secondary patency rate was 90 %. When the patients were divided into a stenosed group (20 patients) and an occluded group (31 patients), there were no differences in the success rate, complication rate, or primary and secondary patency rates.ConclusionAn approach through the radial artery distal to the arteriovenous anastomosis is an effective and safe alternative for the salvage of nonmaturing radiocephalic arteriovenous fistulas, even for occluded fistulas.« less

  5. The Maastricht-Duke bridge: An era of mentoring in clinical research - A model for mentoring in clinical research - A tribute to Dr. Galen Wagner.

    PubMed

    Meijs, Loek; Zusterzeel, Robbert; Wellens, Hein Jj; Gorgels, Anton Pm

    With the passing of Dr. Galen Wagner, an exceptional collaboration between Maastricht University Medical Center, The Netherlands, and Duke Clinical Research Institute, USA, has come to an end. This article focuses on the background of what Galen coined the Maastricht-Duke bridge (MD-bridge), its merits, limitations and development throughout the years, and his special role. Between 2004 and 2015, 23 Maastricht University medical students and post-graduate students were enrolled in the 4-month research elective, mentored by Galen and the Maastricht co-mentor. They were asked to complete a survey about their MD-bridge experience. Sixteen out of the 23 students responded. None but 1 participant had prior research experience. Following their MD bridge-program most participants published 1 or more manuscripts and/or presented their research in an international setting. They felt they had full responsibility as a leader of their project with all participants developing meaningful skills useful in their current job. Fourteen out of 16 would recommend the MD-bridge experience to others. Participants considered the program of great value for their personal growth and independence, giving a feeling of achievement. In addition, for some participants it led to careers in foreign countries including medical practice and research, or obtaining PhDs. With Galen's impressive career of mentoring students, including the 23 MD-bridge participants, he has left behind an amazing concept of self-development in research and personal life. The successes of the MD-bridge prove that it is possible for students to be young investigators during or just after medical school with the potential to contribute to developing meaningful skills and noteworthy careers. Collaborations between international universities, such as the MD-bridge, are feasible and should be embraced by other institutions. Published by Elsevier Inc.

  6. Arteriovenous carboxyhemoglobin difference in critical illness: fiction or fact?

    PubMed

    Westphal, Martin; Eletr, Dina; Bone, Hans Georg; Ertmer, Christian; Weber, Thomas Peter; Aken, Hugo Van; Booke, Michael

    2002-12-06

    It is still unclear whether the paradoxical arteriovenous carboxyhemoglobin (COHb) difference found in critical illness is due to increased COHb production by the lung, or whether this gradient is caused by technical artifacts using spectrophotometry. In healthy and matched endotoxemic sheep, blood gases were analyzed with a standard ABL 625 and the updated version, an ABL 725. The latter one was accurately calibrated for COHb wavelengths (SAT 100) to eliminate the FCOHb dependency on oxygen tension. All endotoxemic sheep exhibited a hypotensive-hyperdynamic circulation and a pulmonary hypertension. Interestingly, arteriovenous COHb difference occurred in both healthy and endotoxemic sheep (P<0.001 each). Arterial and central venous COHb concentrations determined with the ABL 625 were significantly lower than those measured with the ABL 725 (P<0.001 each). We conclude that (a) arteriovenous COHb difference per se does not reflect critical illness and (b) measurements with an ABL 625 underestimate COHb concentrations.

  7. Cerebrovascular Disease and Stroke in Korean Male Adolescent

    PubMed Central

    Oh, Chang Hyun; Chung, Joonho; Hyun, Dongkeun; Kim, Eunyoung

    2012-01-01

    Purpose This study aimed to estimate the prevalence and incidence of cerebrovascular disease (CVD) and stroke in Korean male adolescents. Materials and Methods The authors reviewed all medical certificates, medical records, and radiologic images from the examinations of Korean military conscription from January 2008 to May 2011. Results Of the 101156 examinees, 40 had CVD and stroke during adolescence. The overall prevalence and incidence of CVD and stroke was 39.54 cases per 100000 adolescents and 2.08 cases per 100000 adolescents per year, respectively and these were similar to the worldwide data. There were 3 cases of aneurysm, 3 cases of dural arteriovenous fistula, 11 cases of arteriovenous malformation, 4 cases of cavernous hemangioma, 4 cases of cerebrovascular infarction, 16 cases of Moyamoya disease, and 1 case of missing data. The incidence of arteriovenous malformation (0.57 cases per 100000 adolescents per year) was lower than the incidence for the worldwide general population. The incidence of Moyamoya disease was higher than that in any other country (15.82 cases per 100000 adolescents, vs. 0.83 cases per 100000 adolescents per year). Conclusion We observed ischemic and hemorrhagic stroke, each accounting for approximately half of cases, and high incidence of Moyamoya disease with low incidence of arteriovenous malformation in Korean male adolescents. PMID:22476988

  8. Genetics Home Reference: capillary malformation-arteriovenous malformation syndrome

    MedlinePlus

    ... JB, Enjolras O, Cordisco MR, Burrows PE, Clapuyt P, Hammer F, Dubois J, Baselga E, Brancati F, Carder R, Quintal JM, Dallapiccola B, Fischer G, Frieden IJ, Garzon M, Harper J, Johnson-Patel J, Labrèze C, Martorell L, Paltiel HJ, Pohl ...

  9. Galen: a third generation terminology tool to support a multipurpose national coding system for surgical procedures.

    PubMed

    Trombert-Paviot, B; Rodrigues, J M; Rogers, J E; Baud, R; van der Haring, E; Rassinoux, A M; Abrial, V; Clavel, L; Idir, H

    1999-01-01

    GALEN has developed a new generation of terminology tools based on a language independent concept reference model using a compositional formalism allowing computer processing and multiple reuses. During the 4th framework program project Galen-In-Use we applied the modelling and the tools to the development of a new multipurpose coding system for surgical procedures (CCAM) in France. On one hand we contributed to a language independent knowledge repository for multicultural Europe. On the other hand we support the traditional process for creating a new coding system in medicine which is very much labour consuming by artificial intelligence tools using a medically oriented recursive ontology and natural language processing. We used an integrated software named CLAW to process French professional medical language rubrics produced by the national colleges of surgeons into intermediate dissections and to the Grail reference ontology model representation. From this language independent concept model representation on one hand we generate controlled French natural language to support the finalization of the linguistic labels in relation with the meanings of the conceptual system structure. On the other hand the classification manager of third generation proves to be very powerful to retrieve the initial professional rubrics with different categories of concepts within a semantic network.

  10. Spectrum of prenatally detected central nervous system malformations: Neural tube defects continue to be the leading foetal malformation.

    PubMed

    Siddesh, Anjurani; Gupta, Geetika; Sharan, Ram; Agarwal, Meenal; Phadke, Shubha R

    2017-04-01

    Prenatal diagnosis of malformations is an important method of prevention and control of congenital anomalies with poor prognosis. Central nervous system (CNS) malformations amongst these are the most common. The information about the prevalence and spectrum of prenatally detected malformations is crucial for genetic counselling and policymaking for population-based preventive programmes. The objective of this study was to study the spectrum of prenatally detected CNS malformations and their association with chromosomal abnormalities and autopsy findings. This retrospective study was conducted in a tertiary care hospital in north India from January 2007 to December 2013. The details of cases with prenatally detected CNS malformations were collected and were related with the foetal chromosomal analysis and autopsy findings. Amongst 6044 prenatal ultrasonographic examinations performed; 768 (12.7%) had structural malformations and 243 (31.6%) had CNS malformations. Neural tube defects (NTDs) accounted for 52.3 per cent of CNS malformations and 16.5 per cent of all malformations. The other major groups of prenatally detected CNS malformations were ventriculomegaly and midline anomalies. Chromosomal abnormalities were detected in 8.2 per cent of the 73 cases studied. Foetal autopsy findings were available for 48 foetuses. Foetal autopsy identified additional findings in eight foetuses and the aetiological diagnosis changed in two of them (4.2%). Amongst prenatally detected malformations, CNS malformations were common. NTD, which largely is a preventable anomaly, continued to be the most common group. Moreover, 60 per cent of malformations were diagnosed after 20 weeks, posing legal issues. Chromosomal analysis and foetal autopsy are essential for genetic counselling based on aetiological diagnosis.

  11. Surgical management of venous malformations.

    PubMed

    Loose, D A

    2007-01-01

    Among vascular malformations, the predominantly venous malformations represent the majority of cases. They form a clinical entity and therefore need clear concepts concerning diagnosis and treatment. This paper presents an overview of contemporary classification as well as tactics and techniques of treatment. According to the Hamburg Classification, predominantly venous malformations are categorized into truncular and extratruncular forms, with truncular forms distinguished as obstructions and dilations, and extratruncular forms as limited or infiltrating. The tactics of treatment represent surgical and non-surgical methods or combined techniques. Surgical approaches utilize different tactics and techniques that are adopted based on the pathologic form and type of the malformation: (I) operation to reduce the haemodynamic activity of the malformation; (II) operation to eliminate the malformation; and (III) reconstructive operation. As for (I), a type of a tactic is the operation to derive the venous flow. In (II), the total or partial removal of the venous malformation is demonstrated subdivided into three different techniques. In this way, the infiltrating as well as the limited forms can be treated. An additional technique is dedicated to the treatment of a marginal vein. Approach (III) involves the treatment of venous aneurysms, where a variety of techniques have been successful. Long-term follow-up demonstrates positive results in 91% of the cases. Congenital predominantly venous malformations should be treated according to the principles developed during the past decades in vascular surgery, interventional treatment and multidisciplinary treatment. The days of predominantly conservative treatment should be relegated to the past. Special skills and experiences are necessary to carry out appropriate surgical strategy, and the required operative techniques should be dictated by the location and type of malformation and associated findings.

  12. Some old drugs improve late primary patency rate of native arteriovenous fistulas in hemodialysis patients.

    PubMed

    Righetti, Marco; Ferrario, GianMichele; Serbelloni, Paola; Milani, Silvana; Tommasi, Adalberto

    2009-01-01

    Vascular access failure causes 20% of all hospitalizations of dialysis patients. Native arteriovenous fistulas, the best type of dialysis vascular access, have a 1-year primary patency rate that is extremely variable, ranging 40-80%. Neointimal hyperplasia is the most important cause of arteriovenous fistula late primary dysfunction. In recent years the arteriovenous fistula late primary patency rate has not improved because of the increase of old uremic patients with a high number of comorbidities and the lack of new therapeutic interventions. Therefore, we performed a long-term case-control study to analyze which factors or drugs may affect native arteriovenous fistula late primary patency rate in 60 incident hemodialysis patients. The arteriovenous fistula late primary patency rate was 75.1% after 12 months, 58.5% after 24 months, and 50% after 987 days. Homocysteine levels during follow-up had a significant direct association with vascular access failure (event vs. event-free 28.5+/-1.9 vs. 22.3+/-1.2 micromol/L, p<0.01). Folate values had a trend toward an inverse relationship with arteriovenous fistula failure (event vs. event-free 11.5+/-1.2 vs. 14.6 vs. 1.1 ng/mL, p=0.06). Patients treated with folic acid and/or statin had an arteriovenous fistula late primary patency rate significantly higher than patients without folic acid and statin therapy, respectively, 81.7% vs. 66% after 1 year and 71.5% vs. 39.1% after 2 years (p=0.02). Many other factors were not associated with vascular access failure. Statin and homocysteine-lowering folic acid therapy is associated with prolonged arteriovenous fistula survival. It is important to perform randomized trials to verify our observation.

  13. Bendectin and human congenital malformations.

    PubMed

    Shiono, P H; Klebanoff, M A

    1989-08-01

    The relationship between Bendectin exposure during the first trimester of pregnancy and the occurrence of congenital malformations was prospectively studied in 31,564 newborns registered in the Northern California Kaiser Permanente Birth Defects Study. The odds ratio for any major malformation and Bendectin use was 1.0 (95% confidence interval 0.8-1.4). There were 58 categories of congenital malformations; three of them were statistically associated with Bendectin exposure (microcephaly--odds ratio = 5.3, 95% confidence interval = 1.8-15.6; congenital cataract--odds ratio = 5.3, 95% confidence interval = 1.2-24.3; lung malformations (ICD-8 codes 484.4-484.8)--odds ratio = 4.6, 95% confidence interval = 1.9-10.9). This is exactly the number of associations that would be expected by chance. An independent study (the Collaborative Perinatal Project) was used to determine whether vomiting during pregnancy in the absence of Bendectin use was associated with these three malformations. Two of the three (microcephaly and cataract) had strong positive associations with vomiting in the absence of Bendectin use. We conclude that there is no increase in the overall rate of major malformations after exposure to Bendectin and that the three associations found between Bendectin and individual malformations are unlikely to be causal.

  14. Vascular anomalies of the head and neck: a review of genetics.

    PubMed

    Yadav, Prashant; De Castro, Dawn K; Waner, Milton; Meyer, Lutz; Fay, Aaron

    2013-01-01

    Vascular anomalies comprise malformations, hemangiomas, and rare tumors. The commonality among these lesions is their origin in vascular endothelia. Most occur sporadically, but occasional inheritance is observed and thus allows genetic research and insight into etiology. This review highlights those vascular anomalies in which genetic inheritance has been demonstrated. A comprehensive literature search was performed on PubMed. Fifty-five full-length articles were reviewed. Five categories of vascular anomalies with patterned inheritance were identified: arteriovenous malformation (AVM), capillary malformation (CM), lymphatic malformation (LM), venous malformation (VM), and infantile hemangioma (IH). Capillary and arteriovenous malformation subtypes are associated with a RASA-1 gene mutation and show autosomal dominant inheritance. VEGFR3 mutations have been associated with generalized forms of LM and lymphedema. Mutations in TIE2/TEK genes cause inherited forms of venous malformations also with autosomal dominant inheritance. Familial clustering and atopic disease are associated with infantile hemangioma, and gene expression varies with the developmental stage of these lesions. Most vascular anomalies occur sporadically, but several genes and genetic disorders have been associated with them. Specific forms of capillary malformation appear to be most convincingly associated with genomic errors. Further research promises new insights into the development of this diverse group of disorders.

  15. Galen Wagner, M.D., Ph.D. (1939-2016) as international mentor of young investigators in electrocardiology.

    PubMed

    Swenne, Cees A; Pahlm, Olle; Atwater, Brett D; Bacharova, Ljuba

    This paper describes a substantial part of the international mentoring network of students and young investigators in electrocardiology that developed around Dr. Galen Wagner (1939-2016), including many experiences of his mentees and co-mentors. The paper is meant to stimulate thinking about international mentoring as a means to achieve important learning experiences and personal development of young investigators, to intensify international scientific cooperation, and to stimulate scientific production. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  16. Vesalius on the anatomy and function of the recurrent laryngeal nerves: medical illustration and reintroduction of a physiological demonstration from Galen.

    PubMed

    Lanska, Douglas J

    2014-01-01

    The purpose of this article is to review the anatomical illustrations and physiological demonstrations of sixteenth-century Flemish-born anatomist and physician Andreas Vesalius concerning the recurrent laryngeal nerves. Although Vesalius was primarily an anatomist, he also used vivisection as a pedagogical device to help his students understand the function of structures within the fabric of the body that they had previously studied in anatomical detail. Vesalius's masterwork, De humani corporis fabrica or simply the Fabrica (1543, 1555), was ostensibly an anatomy text, but Vesalius included textual and figural references to his use of vivisection to explicate the function of specific structures. Even as he began to criticize the errors in Galen's anatomical works, Vesalius nevertheless adopted some of Galen's classic physiological demonstrations, in particular the ligation (and subsequent release) of the recurrent laryngeal nerves of a pig to demonstrate their role in generating the pig's squeal. Vesalius's illustrations concerning the recurrent laryngeal nerve in the Fabrica were of two types: elegant anatomical woodcut plates-unsurpassed for their clarity, accuracy, and detail - and the distinctly inelegant historiated initial Q, depicting a throng of putti busily engaged in vivisecting a pig. Vesalius' anatomical plates were heavily plagiarized while the historiated initials, showing the rough work of an anatomist or surgeon, were largely ignored and remain little recognized today. While Vesalius' anatomical illustrations of the recurrent laryngeal nerves contained some errors, they were a dramatic departure from prior meager efforts at medical illustration and indeed far surpassed all contemporary published illustrations by others. Vesalius was also influential in reviving Galen's approach to vivisection, at least for pedagogical purposes, if not really then yet as a full-fledged investigative technique.

  17. Lower Limb Arteriovenous Communications in Diabetes Mellitus: A Potential Reason for Aggravation of Ischemic Symptoms

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Hamady, Mohamad S.; Yu, Dominic F.; Sayer, Gabriel L.

    2006-10-15

    The aim of this study was to assess the prevalence and to evaluate the clinical associations of arteriovenous communications in patients with diabetes mellitus (DM) and lower limb peripheral arterial ischemia. Peripheral arteriography of DM patients from an eight-year period (1993-2000) was evaluated retrospectively by two observers. The presence of arteriovenous communications, defined as occurring without evidence of a preceding precipitating event, and the distribution and severity of the vascular disease were evaluated. The type (non-insulin-dependent DM or insulin-dependent DM) and the duration of the DM, the presenting symptoms, and the presence of a peripheral neuropathy were documented by amore » review of the clinical records. A total of 348 arteriography studies in 285 DM patients were evaluated (duration of DM: median, 16 years; range, 7-42 years); an arteriovenous communication was present in 14/285 patients (4.9%), 9 male and 5 female (median age, 71 years; range, 17-84 years). Symptoms were those of a peripheral leg ulcer (n = 11), claudication (n = 3), and gangrene (n = 1), with symptoms ipsilateral to the side of the arteriovenous communication in 13/14 patients. The sites of the arteriovenous communications were infra popliteal (n = 7), popliteal (n = 3), superficial femoral artery (n = 3), and common femoral artery (n = 1). Features of a peripheral neuropathy were found in 12/14 and ipsilateral to the side of the communication in 11/12. Arteriovenous communications in the peripheral femoral arterial system of patients with DM is an uncommon finding. Although not proven in the current study, arteriovenous communications might be associated with more severe symptoms than that attributable to the underlying vascular disease alone.« less

  18. A hemorrhagic complication after Onyx embolization of a tentorial dural arteriovenous fistula: A caution about subdural extension with pial arterial supply.

    PubMed

    Sato, Kenichi; Matsumoto, Yasushi; Endo, Hidenori; Tominaga, Teiji

    2017-06-01

    We report a case of tentorial dural arteriovenous fistula (DAVF) with a severe intracranial hemorrhage occurring after Onyx embolization. A 40-year-old man presented with an asymptomatic tentorial DAVF on angiography. Transarterial embolization with Onyx was performed via the middle meningeal artery, and the cast filled the fistula itself and its proximal draining vein. Postoperative angiography confirmed complete occlusion of the DAVF. A computed tomography scan performed immediately after the procedure demonstrated an acute subdural hematoma with the temporal hemorrhage. Emergency craniotomy revealed continuous arterial bleeding from a viable glomus-like vascular structure around the proximal part of the embolized draining vein, fed by a pial artery arising from the posterior cerebral artery. Pathologic findings suggested diagnosis of vascular malformation extending into the subdural space. Tentorial DAVFs can extend to the subdural space along their drainage route, and may be involved in severe hemorrhagic complications of curative endovascular treatment using Onyx, particularly those with pial arterial supply.

  19. Pseudoaneurysm with Arteriovenous Fistula after Arthroscopic Procedure: A Rare Complication of Arthroscopy

    PubMed Central

    Jin, Moran; Lee, Yang-Haeng; Yoon, Young Chul; Han, Il-Yong; Park, Kyung-Taek; Wi, Jin Hong

    2015-01-01

    Pseudoaneurysm with arteriovenous fistula is a rare complication of arthroscopy, and can be diagnosed by ultrasonography, computed tomography, magnetic resonance imaging, or angiography. This condition can be treated with open surgical repair or endovascular repair. We report our experience with the open surgical repair of a pseudoaneurysm with an arteriovenous fistula in a young male patient who underwent arthroscopy five months previously. PMID:26290846

  20. Magnetic Resonance Imaging of Malformations of Midbrain-Hindbrain.

    PubMed

    Abdel Razek, Ahmed Abdel Khalek; Castillo, Mauricio

    2016-01-01

    We aim to review the magnetic resonance imaging appearance of malformations of midbrain and hindbrain. These can be classified as predominantly cerebellar malformations, combined cerebellar and brain stem malformations, and predominantly brain stem malformations. The diagnostic criteria for the majority of these morphological malformations are based on neuroimaging findings. The predominantly cerebellar malformations include predominantly vermian hypoplasia seen in Dandy-Walker malformation and rhombencephalosynapsis, global cerebellar hypoplasia reported in lissencephaly and microlissencephaly, and unilateral cerebellar hypoplasia seen in PHACES, vanishing cerebellum, and cerebellar cleft. Cerebellar dysplasias are seen in Chudley-McCullough syndrome, associated with LAMA1 mutations and GPR56 mutations; Lhermitte-Duclos disease; and focal cerebellar dysplasias. Cerebellar hyperplasias are seen in megalencephaly-related syndromes and hemimegalencephaly with ipsilateral cerebellomegaly. Cerebellar and brain stem malformations include tubulinopathies, Joubert syndrome, cobblestone malformations, pontocerebellar hypoplasias, and congenital disorders of glycosylation type Ia. Predominantly brain stem malformations include congenital innervation dysgenesis syndrome, pontine tegmental cap dysplasia, diencephalic-mesencephalic junction dysplasia, disconnection syndrome, and pontine clefts.

  1. Brainstem and cerebellar cavernous malformations.

    PubMed

    Atwal, Gursant S; Sarris, Christina E; Spetzler, Robert F

    2017-01-01

    Cavernous malformations are vascular lesions that occur throughout the central nervous system, most commonly in the supratentorial location, with brainstem and cerebellar cavernous malformations occurring more rarely. Cavernous malformations are associated with developmental venous anomalies that occur sporadically or in familial form. Patients with a cavernous malformation can present with headaches, seizures, sensorimotor disturbances, or focal neurologic deficits based on the anatomic location of the lesion. Patients with infratentorial lesions present more commonly with a focal neurologic deficit. Cavernous malformations are increasingly discovered incidentally due to the increasing use of magnetic resonance imaging. Understanding the natural history of these lesions is essential to their management. Observation and surgical resection are both reasonable options in the treatment of patients with these lesions. The clinical presentation of the patient, the location of the lesion, and the surgical risk assessment all play critical roles in management decision-making. © 2017 Elsevier B.V. All rights reserved.

  2. Congenital heart defects and extracardiac malformations.

    PubMed

    Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

    2013-06-01

    To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

  3. Transarterial Embolization of a Renal Artery Aneurysm Concomitant With Renal Arteriovenous Fistula.

    PubMed

    Hongsakul, Keerati; Bannangkoon, Kittipitch; Boonsrirat, Ussanee; Kritpracha, Boonprasit

    2018-01-01

    Congenital renal artery aneurysm is uncommon. Moreover, renal artery aneurysm concomitant with a congenital renal arteriovenous fistula is extremely rare. Transarterial embolization is the first-line treatment for these conditions. We report a case of a patient with congenital renal artery aneurysm concomitant with a congenital renal arteriovenous fistula of the upper polar left renal artery which was successfully treated by transarterial embolization with coil, glue, and Amplatzer vascular plug.

  4. CASE NOTES AND CLINICIANS: GALENS COMMENTARY ON THE HIPPOCRATIC EPIDEMICS IN THE ARABIC TRADITION

    PubMed Central

    Pormann, PE

    2010-01-01

    Galens Commentaries on the Hippocratic Epidemics constitute one of the most detailed studies of Hippocratic medicine from Antiquity. The Arabic translation of the Commentaries by Ḥunayn ibn Isḥāq (d. c. 873) is of crucial importance because it preserves large sections now lost in Greek, and because it helped to establish an Arabic clinical literature. The present contribution investigate the translation of this seminal work into Syriac and Arabic. It provides a first survey of the manuscript tradition, and explores how physicians in the medieval Muslim world drew on it both to teach medicine to students, and to develop a framework for their own clinical research. PMID:20852724

  5. [Pulmonary arteriovenous fistula with Rendu-Osler-Weber disease].

    PubMed

    Segawa, Masataka; Touge, Masayoshi; Seki, Kouji; Kusajima, Yoshinori; Saito, Katsuhiko

    2012-09-01

    A 36-year-old man was admitted to our hospital for examination of a nodular shadow in the left lung. Chest 3-dimensional computed tomography (3D-CT) revealed a pulmonary arteriovenous fistula (PAVF) of 21 mm in diameter composed of the feeding artery (A4) and the draining vein (V4) in the left S4. Abdominal enhanced CT revealed multiple hepatic arteriovenous fistula. Brain CT revealed a cavernous hemangioma in right occipital cerebrum. He had a family history, habitual epistaxis, and oral telangiectasia and was diagnosed as Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia:HHT). According to his family history, PAVF was likely to be a risk factor of brain infarction and abscess, and the wedge resection of the lingual lobe was performed to remove PAVF.

  6. Management of a case with misdiagnosed spinal dural arterio-venous fistula.

    PubMed

    Lien, Chia-Yi; Lui, Chun-Chung; Lu, Cheng-Hsien; Chang, Wen-Neng

    2014-03-01

    Spinal dural arterio-venous fistula (SDAVF) is an uncommon and easily misdiagnosed vascular malformation. We aimed to report the diagnosis and management of a case with SDAVF. A 62-year-old man suffered from acute paraparesis about 15 months before this study. He underwent a neurosurgical procedure for herniated discs of the cervical spine at that time but there was no relief and the symptoms worsened despite the surgery. Neurologically, he had spastic paraparesis and decreased vibration sensation of the lower limbs. Spinal magnetic resonance imaging (MRI) revealed intra-medullary hyper-intensity lesion at T8 to T10 levels and tubular-like signal void structures in the corresponding posterior sub-arachnoid space. Further trans-arterial angiography through right T6 intercostal artery catheterization revealed engorged veins, thereby confirming the diagnosis of SDAVF. The patient was treated via endovascular embolization (18% Onyx, EV3TM MIT, Bonn, Germany) through spinal angiography and the results showed a marked decrease in engorged veins. After a 4-month follow-up, the patient was symptomatic but stable. Follow-up MRI showed a complete disappearance of the hyper-intensity change of the spinal cord. Spinal MR angiography did not reveal any recurrence of SDAVF. This case study demonstrated the easily misdiagnosed state of SDAVF. Serial neuroimage studies including spinal MRI, endovascular embolization through spinal angiography and MR angiography can be useful tools for its diagnostic confirmation, management and follow-up study.

  7. Cerebellar and Brainstem Malformations.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2016-08-01

    The frequency and importance of the evaluation of the posterior fossa have increased significantly over the past 20 years owing to advances in neuroimaging. Conventional and advanced neuroimaging techniques allow detailed evaluation of the complex anatomic structures within the posterior fossa. A wide spectrum of cerebellar and brainstem malformations has been shown. Familiarity with the spectrum of cerebellar and brainstem malformations and their well-defined diagnostic criteria is crucial for optimal therapy, an accurate prognosis, and correct genetic counseling. This article discusses cerebellar and brainstem malformations, with emphasis on neuroimaging findings (including diagnostic criteria), neurologic presentation, systemic involvement, prognosis, and recurrence. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Galen-In-Use: using artificial intelligence terminology tools to improve the linguistic coherence of a national coding system for surgical procedures.

    PubMed

    Rodrigues, J M; Trombert-Paviot, B; Baud, R; Wagner, J; Meusnier-Carriot, F

    1998-01-01

    GALEN has developed a language independent common reference model based on a medically oriented ontology and practical tools and techniques for managing healthcare terminology including natural language processing. GALEN-IN-USE is the current phase which applied the modelling and the tools to the development or the updating of coding systems for surgical procedures in different national coding centers co-operating within the European Federation of Coding Centre (EFCC) to create a language independent knowledge repository for multicultural Europe. We used an integrated set of artificial intelligence terminology tools named CLAssification Manager workbench to process French professional medical language rubrics into intermediate dissections and to the Grail reference ontology model representation. From this language independent concept model representation we generate controlled French natural language. The French national coding centre is then able to retrieve the initial professional rubrics with different categories of concepts, to compare the professional language proposed by expert clinicians to the French generated controlled vocabulary and to finalize the linguistic labels of the coding system in relation with the meanings of the conceptual system structure.

  9. Headache in children with Chiari I malformation.

    PubMed

    Toldo, Irene; Tangari, Marta; Mardari, Rodica; Perissinotto, Egle; Sartori, Stefano; Gatta, Michela; Calderone, Milena; Battistella, Pier Antonio

    2014-05-01

    Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (<5 mm, N = 12), mild (5-9 mm, N = 27), and severe tonsillar ectopia (≥10 mm, N = 6), respectively. Twenty-four out of 33 (73%) cases with Chiari 1 malformation complained of headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia. © 2014 American Headache Society.

  10. Transarterial onyx embolization of cranial dural arteriovenous fistulas: long-term follow-up.

    PubMed

    Chandra, R V; Leslie-Mazwi, T M; Mehta, B P; Yoo, A J; Rabinov, J D; Pryor, J C; Hirsch, J A; Nogueira, R G

    2014-09-01

    Endovascular therapy with liquid embolic agents is a common treatment strategy for cranial dural arteriovenous fistulas. This study evaluated the long-term effectiveness of transarterial Onyx as the single embolic agent for curative embolization of noncavernous cranial dural arteriovenous fistulas. We performed a retrospective review of 40 consecutive patients with 41 cranial dural arteriovenous fistulas treated between March 2006 and June 2012 by using transarterial Onyx embolization with intent to cure. The mean age was 57 years; one-third presented with intracranial hemorrhage. Most (85%) had cortical venous drainage. Once angiographic cure was achieved, long-term treatment effectiveness was assessed with DSA and clinical follow-up. Forty-nine embolization sessions were performed; 85% of cranial dural arteriovenous fistulas were treated in a single session. The immediate angiographic cure rate was 95%. The permanent neurologic complication rate was 2% (mild facial palsy). Thirty-five of the 38 patients with initial cure underwent short-term follow-up DSA (median, 4 months). The short-term recurrence rate was only 6% (2/35). All patients with occlusion at short-term DSA undergoing long-term DSA (median, 28 months) had durable occlusion. No patient with long-term clinical follow-up (total, 117 patient-years; median, 45 months) experienced hemorrhage. Transarterial embolization with Onyx as the single embolic agent results in durable long-term cure of noncavernous cranial dural arteriovenous fistulas. Recurrence rates are low on short-term follow-up, and all patients with angiographic occlusion on short-term DSA follow-up have experienced a durable long-term cure. Thus, angiographic cure should be defined at short-term follow-up angiography instead of at the end of the final embolization session. Finally, long-term DSA follow-up may not be necessary if occlusion is demonstrated on short-term angiographic follow-up. © 2014 by American Journal of Neuroradiology.

  11. [Focus on Dandy-Walker malformation].

    PubMed

    Klein, O; Pierre-Kahn, A

    2006-09-01

    The aim of this review is to summarize our knowledge about Dandy-Walker malformation (DWM) and introduce recent notions about its prognosis. DWM is a malformation associating hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses and anterio-posterior enlargement of the posterior fossa. It is frequently associated with genetic anomalies, brain malformations (anomalies of gyration, grey matter heterotopias, meningoceles, corpus callosum agenesis...) or systemic malformations (heart, orthopedic, intestinal, urogenital and facial anomalies). It is also part of many syndromes. Its rarity, the difficulty of its diagnosis, the fact that the malformation is mainly known through hospitalized patients, mainly from neurosurgical departments, have made its definition variable and prevents us from having an accurate knowledge of its natural history and prognosis. Hydrocephalus, so frequent in neurosurgical series that some have included it in the definition, is actually rare as has been seen in prenatal series. Treatment, when necessary, is still controversial, the main options remaining cyst fenestration, ventriculo- and/or cystoperitoneal shunts and more recently endoscopic third ventriculostomy. The prognosis, commonly said to be unpredictable, is actually foreseeable after scrutinous observation of the brain anatomy, and mainly of the vermis. A cerebellar vermis with three groups of lobes and two main fissures, as identified on MRI T2 sagittal views, not only has the greatest chance to not be associated with other malformation but also to have a favorable neurocognitive outcome. On the contrary, a deeply dysgenetic vermis with only two or one recognizable lobes is not only constantly associated with other brain malformation but also with poor prognosis. This is a recent advance that may be important for those involved in prenatal counselling.

  12. [The use of labelled albumin microspheres in the study of arteriovenous shunting in varicosities of the lower limbs (author's transl)].

    PubMed

    Serise, J M; Le Héron, D; Le Héron, G; Bonnet, A; Busquet, J; Basse-Cathalinat, B; Tingaud, R

    1982-01-01

    The greater proportion of oxygen in the blood circulating in veins of the lower limbs bought to attention the possibility of pre-capillary arteriovenous shunting. The intra-arteriel injection of Technetium labelled serum albumin microspheres enables one to quantify arteriovenous shunts greater than 25 microns. The authors made a comparative study of arterio-venous shunting by blood gases analysis and isotopic techniques in twenty patients. The results seem to deny the existence of arteriovenous shunts in the genesis of essential varicose veins in the lower limb.

  13. Neuro-ophthalmology of type 1 Chiari malformation

    PubMed Central

    Shaikh, Aasef G.; Ghasia, Fatema F.

    2016-01-01

    Chiari malformation is a congenital deformity leading to herniation of cerebellar tonsils. Headache is a typical symptom of this condition, but patients with Chiari malformation often present with double vision and vertigo. Examination of eye movements in such patients often reveals nystagmus and strabismus. Eye movement deficits in the context of typical symptomatic presentation are critical clinical markers for the diagnosis of Chiari malformation. We will review eye movement deficits that seen in patients with type 1 Chiari malformation. We will then discuss the underlying pathophysiology and therapeutic options for such deficits. PMID:26843890

  14. A developmental classification of malformations of the brainstem.

    PubMed

    Barkovich, A James; Millen, Kathleen J; Dobyns, William B

    2007-12-01

    With advances in imaging and genetics, malformations of the brainstem are being more commonly identified. We describe and classify brainstem anomalies in 138 patients ascertained over a period of 10 years Magnetic resonance imaging studies and, where available, clinical records of the patients were retrospectively reviewed. Malformations were segregated according to magnetic resonance findings and classified when possible by embryological mechanisms The most common location for anomalies was the pons, which was involved in 114 patients. The midbrain was involved in 45 patients, whereas the medulla was involved in 14. In 53 patients, more than 1 region was affected (all 3 regions in 6 patients, midbrain and pons in 39, and medulla and pons in 8). The malformations were divided into four groups: (1) malformations with abnormal brainstem segmentation, (2) malformations with segmental hypoplasia, (3) postsegmentation malformations, and (4) malformations associated with abnormal cortical organization The malformations of the brainstem identified in this study were diverse and complex. This proposed classification organizes them into groupings based on known genetics and embryological events. Use of this system will help clinicians and scientists to better understand these disorders and, ultimately, to better counsel families of affected patients.

  15. Associated malformations among infants with anophthalmia and microphthalmia.

    PubMed

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2012-03-01

    Infants with anophthalmia and microphthalmia frequently have other associated congenital anomalies. The reported frequency and types of associated malformations vary among different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with anophthalmia and microphthalmia in a geographically well defined population from 1979 to 2004 of 346,831 consecutive births. Of the 87 infants with anophthalmia and microphthalmia born during this period (prevalence at birth, 2.5 per 10,000), 90% had associated malformations. Infants with associated malformation were divided into recognizable conditions (22 infants [25%] with chromosomal and 15 infants [17%] with nonchromosomal conditions), and nonrecognizable conditions (41 infants [47%] with multiple malformations). Trisomies 13 and 18 were the most frequent chromosomal abnormalities. Amniotic bands sequence, CHARGE syndrome, Meckel-Gruber syndrome, and VACTERL association were most often present in recognizable nonchromosomal conditions. Malformations in the musculoskeletal, cardiovascular, and central nervous systems were the most common other anomalies in infants with multiple malformations and nonrecognizable conditions. The frequency of associated malformations in infants with anophthalmia or microphthalmia emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations-especially musculoskeletal, cardiac, and central nervous system anomalies-may need to be considered in infants with anophthalmia or microphthalmia, and referral of these infants for genetics evaluation and counseling seems warranted. Copyright © 2012 Wiley Periodicals, Inc.

  16. Treatment of human head lice infestations in a single application with a new galenic lotion.

    PubMed

    Militão de Sousa, F; Vasconcelos, A W; de Nadon, J; Duhot, P-Y

    2010-10-01

    To evaluate the efficiency and safety of a new galenic meta-emulsion for the treatment of human head lice (Pediculus capitis) in a single application. A controlled clinical study was conducted comparing lice infestation and nit hatching observed before and after a treatment in a single application. Eighty-two of the 87 children included completed the study. An infestation control was performed on 36 and 46 children, 8 and 24 h respectively after application. Five days later, a meticulous hair examination was carried out to check that the lice infestation was completely cured. After a single application of the lotion being tested, an examination of the scalp with a head lice detection comb, as well as an examination of the rinsing water and the towel used for drying after washing, showed that out of the total 1285 lice, there were no live lice. The percentage of nits hatching before treatment was close to 70%. In comparison, after an 8-h treatment (t(+8)), the percentage of nits hatching was 2.1%, with only 0.35% of living nymphs. After a 24-h treatment (t(+24)), 1.9% hatched with 0.38% living nymphs. Nymphs were revealed to be non-viable. After 5 days (t(+120)), no living adult or immature lice were found on the subjects tested. Moreover, observation of tolerance levels to this treatment at days 1, 5 and 12 showed no side effects. The specific galenic lotion completely cured head lice infestation in the population studied in a single application. The lotion, a patented meta-emulsion, has a mechanical action that asphyxiates lice and nits. Considering the advantages of the single application, the possibility of complete concomitant therapeutics for a whole school population within only 1 day and the high level of tolerance to this treatment, this approach seems simple and promising.

  17. [Lymphatic malformations in the head and neck area].

    PubMed

    Wiegand, S; Werner, J A

    2016-02-01

    Lymphatic malformations are congenital malformations of the lymphatic system. They are mainly located in the head and neck area, and grow proportional to the patients' body growth. Depending on the morphology, it can be distinguished between macrocystic, microcystic and mixed lymphatic malformations. Due to their infiltrative growth, microcystic lymphatic malformations are particularly difficult to treat. Therapeutic approaches include conventional surgical resection, laser therapy, sclerotherapy and systemic drug therapies.

  18. Dandy-Walker Malformation Presenting with Psychological Manifestations

    PubMed Central

    Dahanayake, Dulangi Maneksha Amerasinghe

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment. PMID:27493822

  19. Dandy-Walker Malformation Presenting with Psychological Manifestations.

    PubMed

    Rohanachandra, Yasodha Maheshi; Dahanayake, Dulangi Maneksha Amerasinghe; Wijetunge, Swarna

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment.

  20. GALEN: a third generation terminology tool to support a multipurpose national coding system for surgical procedures.

    PubMed

    Trombert-Paviot, B; Rodrigues, J M; Rogers, J E; Baud, R; van der Haring, E; Rassinoux, A M; Abrial, V; Clavel, L; Idir, H

    2000-09-01

    Generalised architecture for languages, encyclopedia and nomenclatures in medicine (GALEN) has developed a new generation of terminology tools based on a language independent model describing the semantics and allowing computer processing and multiple reuses as well as natural language understanding systems applications to facilitate the sharing and maintaining of consistent medical knowledge. During the European Union 4 Th. framework program project GALEN-IN-USE and later on within two contracts with the national health authorities we applied the modelling and the tools to the development of a new multipurpose coding system for surgical procedures named CCAM in a minority language country, France. On one hand, we contributed to a language independent knowledge repository and multilingual semantic dictionaries for multicultural Europe. On the other hand, we support the traditional process for creating a new coding system in medicine which is very much labour consuming by artificial intelligence tools using a medically oriented recursive ontology and natural language processing. We used an integrated software named CLAW (for classification workbench) to process French professional medical language rubrics produced by the national colleges of surgeons domain experts into intermediate dissections and to the Grail reference ontology model representation. From this language independent concept model representation, on one hand, we generate with the LNAT natural language generator controlled French natural language to support the finalization of the linguistic labels (first generation) in relation with the meanings of the conceptual system structure. On the other hand, the Claw classification manager proves to be very powerful to retrieve the initial domain experts rubrics list with different categories of concepts (second generation) within a semantic structured representation (third generation) bridge to the electronic patient record detailed terminology.

  1. Early application of an intermittent pneumatic compression device is safe and results in proximal arteriovenous fistula enlargement.

    PubMed

    Desai, Sanjay; Mitra, Amit; Arkans, Ed; Singh, Tej M

    2018-05-01

    Delays in arteriovenous fistula maturation can cause care delays and increased costs. Increased distention pressure and intermittent wall shear stress may dilate veins based on prior research. Early use of non-invasive devices may help assist clinical arteriovenous fistula dilation. This was an Institutional Review Board approved study. After arteriovenous fistula creation, a novel, intermittent pneumatic compression device (Fist Assist ® ) was applied 15 cm proximal to arteriovenous fistula enabling 60 mmHg of cyclic compression for 6 h daily for 30 days. Among the patients who completed 1 month follow-up, 30 (n = 30) arteriovenous fistula patients were in the study arm to test vein dilation with Fist Assist. Controls (n = 16) used a sham device. Vein size was measured and recorded at baseline and after 30 days by duplex measurement. Clinical results (percentage increase) were recorded and tested for significance. No patients experienced thrombosis or adverse effects. Patient compliance and satisfaction was high. After 1 month, the mean percentage increase in vein diameter in the Fist Assist treatment group was significantly larger (p = 0.026) than controls in the first 5 mm segment of the fistula after the anastomosis. All fistulas treated with Fist Assist are still functional with no reported thrombosis or extravasations. Early application of an intermittent pneumatic compression device may assist in arteriovenous fistula dilation and are safe. Non-invasive devices like Fist Assist may have clinical utility to help fistulae development and decrease costs as they may eventually assist maturation.

  2. Study of placenta of children born with congenital malformations.

    PubMed

    Stoll, Claude; Alembik, Yves; Dott, Béatrice; Roth, Marie-Paule

    2003-01-01

    The malformations in this study were observed in a series of 279,642 consecutive births of known outcome registered in our Registry of congenital anomalies. For each case, more than 50 factors included in the registration forms were studied. One of the factors studied was the placenta. For each malformed child, a control was chosen. Cases with maternal known factors impairing placenta function, i.e. vasculopathy and diabetes, were excluded. In each category of malformations studied, the malformed children were divided into isolated and non-isolated (multiple malformed) cases. The weight of placenta of isolated cases was not lower than the weight of placenta of the controls. In contrast, the weight of placenta of the cases with non-isolated malformations was lower than the weight of placenta of the controls and of the isolated cases, for all categories of malformations but gastroschisis and omphalocele. The mean weights at birth of the cases with multiple malformations were also lower than those of the controls. The human placenta discounts a principal functional part, the maternal blood in the intervillous space. Congenital malformations may interact with this function.

  3. Hydrocephalus in Dandy-Walker malformation.

    PubMed

    Spennato, Pietro; Mirone, Giuseppe; Nastro, Anna; Buonocore, Maria Consiglio; Ruggiero, Claudio; Trischitta, Vincenzo; Aliberti, Ferdinando; Cinalli, Giuseppe

    2011-10-01

    Even if the first description of Dandy-Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of "Dandy-Walker". An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed. The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

  4. Brain Malformations

    MedlinePlus

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  5. What Is an Arteriovenous Malformation (AVM)?

    MedlinePlus

    ... sensory information, such as interpretation of pain and temperature, light touch, vibration and more. The temporal lobe functions to process things related to hearing, memory, learning and receptive speech. The occipital lobe functions to ...

  6. 21 CFR 878.4010 - Tissue adhesive.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ... DEVICES GENERAL AND PLASTIC SURGERY DEVICES Surgical Devices § 878.4010 Tissue adhesive. (a) Tissue... intended for use in the embolization of brain arteriovenous malformation or for use in ophthalmic surgery...

  7. 21 CFR 878.4010 - Tissue adhesive.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ... DEVICES GENERAL AND PLASTIC SURGERY DEVICES Surgical Devices § 878.4010 Tissue adhesive. (a) Tissue... intended for use in the embolization of brain arteriovenous malformation or for use in ophthalmic surgery...

  8. 21 CFR 878.4010 - Tissue adhesive.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... DEVICES GENERAL AND PLASTIC SURGERY DEVICES Surgical Devices § 878.4010 Tissue adhesive. (a) Tissue... intended for use in the embolization of brain arteriovenous malformation or for use in ophthalmic surgery...

  9. Suicide by severing the arterio-venous subclavian dialysis catheter.

    PubMed

    Edirisinghe, P A S; Busuttil, A

    2006-02-01

    Haemodialysis access is an essential requirement for haemodialysis treatment in end-stage renal disease. The common forms are arteriovenous fistula (AVF) and arteriovenous grafts in ante-cubital fossa, forearm and upper thigh. Sometimes temporary or immediate access is created via a subclavian catheter or internal jugular catheter. This report is on a 79-year-old man who was suffering from chronic renal failure with a non-functional peripheral AVF; he was being dialysed through a permanent subclavian catheter and he became depressed due to continuing deterioration of his health. He used the easily accessible haemodialysis site as the method of suicide by cutting the tube that connected with the main vessel in his chest and bled to death. This highlights the requirement to assess carefully the patient's mental state in those on chronic haemodialysis, even though very few similar fatal cases have been previously reported.

  10. Placement of Upper Extremity Arteriovenous Access in Patients with Central Venous Occlusions: A Novel Technique.

    PubMed

    Murga, Allen G; Chiriano, Jason T; Bianchi, Christian; Sheng, Neha; Patel, Sheela; Abou-Zamzam, Ahmed M; Teruya, Theodore H

    2017-07-01

    Central venous occlusion is a common occurrence in patients with end-stage renal disease. Placement of upper extremity arteriovenous access in patients with occlusion of the brachiocephalic veins is often not an option. Avoidance of lower extremity vascular access can decrease morbidity and infection. The central venous lesions were crossed centrally via femoral access. The wire was retrieved in the neck extravascularly. A Hemodialysis Reliable Outflow catheter was then placed in the right atrium and completed with an arterial anastomosis. We describe a novel technique for placing upper extremity arteriovenous access in patients with occlusion of the brachiocephalic veins. This technique was utilized in 3 patients. The technical success was 100%. The placement of upper extremity arteriovenous access in patients with central venous occlusions is technically feasible. Published by Elsevier Inc.

  11. Acquired arteriovenous fistula in a grizzly bear (Ursus arctos horribilis).

    PubMed

    Tuttle, Allison D; MacLean, Robert A; Linder, Keith; Cullen, John M; Wolfe, Barbara A; Loomis, Michael

    2009-03-01

    A captive adult male grizzly bear (Ursus arctos horribilis) was evaluated due to multifocal wounds of the skin and subcutaneous tissues sustained as a result of trauma from another grizzly bear. On presentation, one lesion that was located in the perineal region seemed to be a deep puncture with purple tissue protruding from it. This perineal wound did not heal in the same manner or rate as did the other wounds. Twenty-five days after initial detection, substantial active hemorrhage from the lesion occurred and necessitated anesthesia for examination of the bear. The entire lesion was surgically excised, which later proved curative. An acquired arteriovenous fistula was diagnosed via histopathology. Arteriovenous fistulas can develop after traumatic injury and should be considered as a potential complication in bears with nonhealing wounds.

  12. Technique to Avoid Hemodialysis Catheter in Patients with Failing Aneurysmal Arteriovenous Fistula by Creating a Concomitant New Arteriovenous Fistula.

    PubMed

    Lara, Kelly A; Chua, Rochelle Anne; Vo, Trung D

    2018-05-01

    Autogenous arteriovenous fistula (AVF) is the primary recommended access for hemodialysis. Long-term use will not uncommonly result in AVF aneurysmal degeneration. Aneurysm-associated complications encompass pain, skin ulceration, infection, thrombosis, cannulation difficulties, and life-threatening bleeding. Various methods to repair aneurysmal AVFs have been described. However, there may be circumstances when this is not possible and require insertion of a temporary hemodialysis catheter (HDC) until a new arteriovenous access is created. We describe a case series of creating a new simultaneous AVF while continuing to use the primary failing aneurysmal AVF to avoid placement of an HDC. Once the new AVF becomes operational, the primary aneurysmal AVF can be abandoned. Six patients underwent simultaneous new AVF creation, 4 ipsilateral, and 2 contralateral. None of the patients developed symptomatic steal syndrome or congestive heart failure. Five of 6 patients had successful usage of the new AVF, and subsequently underwent ligation and excision of the aneurysmal AVF, thus avoiding a temporary HDC. Close monitoring for skin compromise and bleeding in the aneurysmal AVF is recommended while the new AVF matures. Copyright © 2018 Elsevier Inc. All rights reserved.

  13. Congenital malformations of the skull and meninges.

    PubMed

    Kanev, Paul M

    2007-02-01

    The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

  14. [Renal cirsoid aneurysm (congenital arteriovenous fistula): a rare cause of severe hematuria].

    PubMed

    Pereira Arias, José Gregorio; Ullate Jaime, Vicente; Pereda Martínez, Esther; Gutiérrez Díez, José María; Ateca Díaz-Obregón, Ricardo; Ramírez Rodríguez, Maria Mar; Berreteaga Gallastegui, José Ramón

    2007-06-01

    Congenital arteriovenous fistulas are an exceptional clinical feature. Although they are frequently asymptomatic, their presentation as severe hematuria pose an excellent diagnostic exercise and often immediate therapeutic action. We report the case of a 75-year-old female patient presenting with severe hematuria producing anaemia, high blood pressure and congestive heart failure. Image tests revealed right ureteral-hydronephrosis with bladder blockage by blood clots. The endoscopic study (cystoscopy and ureterorenoscopy) alerted about the origin of the hematuria from the right kidney, finally requiring nephrectomy as definitive treatment. Pathology revealed the presence of a round formation with multiple vascular channels, arterial and venous, in the pyelocalicial submucosa, with focal epithelial erosion, compatible with congenital arteriovenous fistula. We review the diagnostic and therapeutic features in the literature. Renal congenital arteriovenous fistulas represent a diagnostic dilemma. They may present asymptomatic or condition clinical features derived from the shunt and high cardiac output (hypertensive cardiopathy and congestive heart failure) or from the erosion and acute hemorrhage into the urinary tract (severe renal hematuria). Treatment should be conservative with embolization or supraselective sclerosis. Nevertheless, in cases of big fistulas, post embolization revascularization, or hemodynamic instability nephrectomy is an excellent option.

  15. Chiari I Malformation in Nephropathic Cystinosis

    PubMed Central

    Rao, Kavya I; Hesselink, John; Trauner, Doris A

    2015-01-01

    Objective To determine the relative incidence of Chiari I malformations in children with cystinosis compared with those in the general population. Study design Magnetic resonance imaging (MRI) scans were performed on 53 patients with nephropathic cystinosis and 120 controls, age range 3-18 years. Results Ten of 53 (18.9%) cystinosis patients had Chiari I or tonsillar ectopia, and only 2 of 120 controls (1.6%) had a similar finding. At least 2 of the patients had symptoms or signs thought to be related to the malformation, and one had surgical decompression. Two had an associated cervical syrinx. Conclusions Children with cystinosis have a 12-fold higher prevalence of Chiari I malformations than the general pediatric population. Chiari I malformations should be high on the differential diagnosis when individuals with cystinosis develop neurologic signs and symptoms, and MRI scans should be performed on children with cystinosis who present with new-onset headache, ataxia, incontinence, or other unexplained neurologic symptoms. PMID:26265281

  16. Clinical presentation, imaging findings, and prognosis of spinal dural arteriovenous fistula.

    PubMed

    Lee, Jookyung; Lim, Young-Min; Suh, Dae Chul; Rhim, Seung Chul; Kim, Sang Joon; Kim, Kwang-Kuk

    2016-04-01

    Spinal dural arteriovenous fistula (SDAVF) is a relatively common acquired vascular malformation of the spinal cord. Assessment of a SDAVF is often difficult because of non-specific findings on non-invasive imaging modalities. Diagnosis of a SDAVF is often delayed, and some patients receive unnecessary treatment and treatment delays, often resulting in a poor outcome. The aim of this study was to characterize the clinical presentation, typical imaging findings, and long-term outcome of SDAVF. Forty patients (13 women, 27 men; mean age 58.18 ± standard deviation 14.75 years) who were treated at our hospital from June 1992 to March 2014 were retrospectively reviewed. We investigated the baseline characteristics, clinical presentation, imaging findings, treatment modalities, and outcome of the patients. The most common clinical presentation was a sensory symptom (80%), followed by motor weakness (70%), and sphincter dysfunction (62.5%). Roughly one-third (32.5%) of patients had a stepwise progression of fluctuating weakness and sensory symptoms, but the most common presentation was chronic progressive myelopathic symptoms (47.5%). Thirty-four patients (85%) had T2 signal change on the spinal cord MRI, indicative of cord edema. Thirty-eight patients had typical perimedullary vessel flow voids on T2-weighted MRI. Twenty-eight patients were treated with endovascular embolization, five patients underwent surgery, and four patients underwent both. Clinical outcome was determined by severity of initial deficit (p=0.008), extent of cord edema (p=0.010), treatment failure (p=0.004), and a residual fistula (p=0.017). SDAVF causes a treatable myelopathy, so early diagnosis and intervention is essential. Copyright © 2015 Elsevier Ltd. All rights reserved.

  17. GalenOWL: Ontology-based drug recommendations discovery

    PubMed Central

    2012-01-01

    Background Identification of drug-drug and drug-diseases interactions can pose a difficult problem to cope with, as the increasingly large number of available drugs coupled with the ongoing research activities in the pharmaceutical domain, make the task of discovering relevant information difficult. Although international standards, such as the ICD-10 classification and the UNII registration, have been developed in order to enable efficient knowledge sharing, medical staff needs to be constantly updated in order to effectively discover drug interactions before prescription. The use of Semantic Web technologies has been proposed in earlier works, in order to tackle this problem. Results This work presents a semantic-enabled online service, named GalenOWL, capable of offering real time drug-drug and drug-diseases interaction discovery. For enabling this kind of service, medical information and terminology had to be translated to ontological terms and be appropriately coupled with medical knowledge of the field. International standards such as the aforementioned ICD-10 and UNII, provide the backbone of the common representation of medical data, while the medical knowledge of drug interactions is represented by a rule base which makes use of the aforementioned standards. Details of the system architecture are presented while also giving an outline of the difficulties that had to be overcome. A comparison of the developed ontology-based system with a similar system developed using a traditional business logic rule engine is performed, giving insights on the advantages and drawbacks of both implementations. Conclusions The use of Semantic Web technologies has been found to be a good match for developing drug recommendation systems. Ontologies can effectively encapsulate medical knowledge and rule-based reasoning can capture and encode the drug interactions knowledge. PMID:23256945

  18. Genetic variation in the functional ENG allele inherited from the non-affected parent associates with presence of pulmonary arteriovenous malformation in hereditary hemorrhagic telangiectasia 1 (HHT1) and may influence expression of PTPN14.

    PubMed

    Letteboer, Tom G W; Benzinou, Michael; Merrick, Christopher B; Quigley, David A; Zhau, Kechen; Kim, Il-Jin; To, Minh D; Jablons, David M; van Amstel, Johannes K P; Westermann, Cornelius J J; Giraud, Sophie; Dupuis-Girod, Sophie; Lesca, Gaetan; Berg, Jonathan H; Balmain, Allan; Akhurst, Rosemary J

    2015-01-01

    HHT shows clinical variability within and between families. Organ site and prevalence of arteriovenous malformations (AVMs) depend on the HHT causative gene and on environmental and genetic modifiers. We tested whether variation in the functional ENG allele, inherited from the unaffected parent, alters risk for pulmonary AVM in HHT1 mutation carriers who are ENG haploinsufficient. Genetic association was found between rs10987746 of the wild type ENG allele and presence of pulmonary AVM [relative risk = 1.3 (1.0018-1.7424)]. The rs10987746-C at-risk allele associated with lower expression of ENG RNA in a panel of human lymphoblastoid cell lines (P = 0.004). Moreover, in angiogenically active human lung adenocarcinoma tissue, but not in uninvolved quiescent lung, rs10987746-C was correlated with expression of PTPN14 (P = 0.004), another modifier of HHT. Quantitative TAQMAN expression analysis in a panel of normal lung tissues from 69 genetically heterogeneous inter-specific backcross mice, demonstrated strong correlation between expression levels of Eng, Acvrl1, and Ptpn14 (r2 = 0.75-0.9, P < 1 × 10(-12)), further suggesting a direct or indirect interaction between these three genes in lung in vivo. Our data indicate that genetic variation within the single functional ENG gene influences quantitative and/or qualitative differences in ENG expression that contribute to risk of pulmonary AVM in HHT1, and provide correlative support for PTPN14 involvement in endoglin/ALK1 lung biology in vivo. PTPN14 has been shown to be a negative regulator of Yap/Taz signaling, which is implicated in mechanotransduction, providing a possible molecular link between endoglin/ALK1 signaling and mechanical stress. EMILIN2, which showed suggestive genetic association with pulmonary AVM, is also reported to interact with Taz in angiogenesis. Elucidation of the molecular mechanisms regulating these interactions in endothelial cells may ultimately provide more rational choices for HHT therapy.

  19. Statins Improve Long Term Patency of Arteriovenous Fistula for Hemodialysis

    PubMed Central

    Chang, Hao-Hsiang; Chang, Yu-Kang; Lu, Chia-Wen; Huang, Chi-Ting; Chien, Chiang-Ting; Hung, Kuan-Yu; Huang, Kuo-Chin; Hsu, Chih-Cheng

    2016-01-01

    The protective effects of statins against stenosis for permanent hemodialysis access have been repeatedly demonstrated in animal studies, but remain controversial in human studies. This study aims to evaluate the association between statin use and permanent hemodialysis access patency using a nationwide hemodialysis cohort. A total of 9862 pairs of statin users and non-users, matched by age and gender, were selected for investigation from 75404 new hemodialysis patients during 2000–2008. The effect of statins on permanent hemodialysis access patency was evaluated using Cox proportional hazards models. Compared with non-users, statin users had an overall 18% risk reduction in the composite endpoint in which angioplasty and recreation were combined (adjusted hazard ratio = 0.82 [95%CI, 0.78–0.87]) and 21% in recreation of permanent hemodialysis access (adjusted hazard ratio = 0.79 [95%CI, 0.69–0.80]). Specifically, the protective effect was found for arteriovenous fistula (adjusted hazard ratio = 0.78[95% CI, 0.73–0.82] for composite endpoint and 0.74 [95% CI, 0.69–0.80] for vascular recreation), but not for arteriovenous grafts (adjusted hazard ratio = 1.10 [95% CI, 0.98–1.24] and 0.94 [95% CI, 0.83–1.07]). Statins possess a protective effect for arteriovenous fistula against the recreation of permanent hemodialysis access. The results provide a pharmaco-epidemiologic link between basic research and clinical evidence. PMID:26902330

  20. Dural arteriovenous fistula as a treatable dementia.

    PubMed

    Enofe, Ikponmwosa; Thacker, Ike; Shamim, Sadat

    2017-04-01

    Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms.

  1. Choledochal malformations: the Scottish experience.

    PubMed

    Yeung, Baldwin Po Man; Broadis, Emily; Maguire, Kirsty; Bradnock, Timothy J; Munro, Fraser D; Driver, Chris P; Haddock, Graham

    2012-06-01

    Excisional surgery for choledochal malformations in Scotland is currently performed in three specialist pediatric surgical centers using open or laparoscopic-assisted techniques. We reviewed the outcome of children who had excisional surgery in Scotland between 1992 and 2010. Case notes for all patients undergoing excisional surgery in any of the three specialist pediatric surgical centers in Scotland between 1992 and 2010 were retrospectively reviewed. A total of 25 patients were identified, with a female preponderance of 4:1. Of these, three patients (12%) were diagnosed by antenatal ultrasound scan. The commonest presenting symptoms were anorexia (56%), abdominal pain (52%), and jaundice (52%). Only 20% had the classical triad of abdominal pain, jaundice, and a palpable mass. Using the King's College Hospital classification, 14 patients had type 1 malformations, 8 had type 4 malformations, and 3 had type 2 malformations. Median age at operation was 2 years (range 35 days to 13.5 years). Two centers performed open excision while the third center used primarily a laparoscopic-assisted technique. Median follow-up was 2.1 years (range 30 days to 11.9 years). Three patients (12%) required repeat laparotomy. The wound infection rate was 8% (n=2). The recurrent cholangitis rate was 8% (n=2). There was one late death due to adhesive small bowel obstruction, 4 years after surgery. To date, no patient has developed biliary tree stones or liver failure. Choledochal malformation excisional surgery, either open or laparoscopic assisted, can be safely performed in appropriately equipped, pediatric surgical centers in Scotland by experienced pediatric surgeons. Copyright © 2012 by Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  2. Embolization of Brain Aneurysms and Fistulas

    MedlinePlus

    ... Resources Professions Site Index A-Z Embolization of Brain Aneurysms and Arteriovenous Malformations/Fistulas Embolization of brain ... Brain Aneurysms and Fistulas? What is Embolization of Brain Aneurysms and Fistulas? Embolization of brain aneurysms and ...

  3. Genetics Home Reference: megalencephaly-capillary malformation syndrome

    MedlinePlus

    ... the prognosis of a genetic condition? Genetic and Rare Diseases Information Center Frequency The prevalence of MCAP is ... Brain Malformations Health Topic: Vascular Diseases Genetic and Rare Diseases Information Center (1 link) Megalencephaly-capillary malformation syndrome ...

  4. Dural Sinus Malformations (DSM) with Giant Lakes, in Neonates and Infants. Review of 30 Consecutive Cases.

    PubMed

    Barbosa, M; Mahadevan, J; Weon, Y C; Yoshida, Y; Ozanne, A; Rodesch, G; Alvarez, H; Lasjaunias, P

    2003-12-20

    Abstract: Background and Purpose. Dural Arteriovenous Shunt (DAVS) in children include Dural sinus malformation (DSM), infantile and adult types. They are rare and seldom reported. Our purpose was to highlight the angiographic features of the DSM sub group for prognosis of clinical evolution and outcome and to lay guidelines for management. From a dedicated neurovascular data bank, there were 52 cases of arteriovenous dural shunts in children from 1985 to 2003. Of these, there were 30 patients with DSM, which we analysed the various angioarchitecture, presentation and neurological outcome. Children clinical status was evaluated and scored at admission and follow up. Results. There was an overall male dominance of 2:1. Antenatal diagnosis was obtained in 8/30 (26.7%) cases. Mean age of diagnosis was 5 months. Mean age at first consultation was 8.7 months. No patient was diagnosed during childhood. The most common clinical presentations were macrocrania 76.7%, seizures 23.3% and mental retardation 23.3%. In 14/30 (35.7%) of the patients, the therapeutic decision was to manage conservatively; in 5/14 (30.7%) with predictable favourable evolution and in 9/14 (64.3%) with irreversible poor neurological outcome. In the remaining 16/30 (53.3%) patients, endovascular treatment was performed. In 12/16 (75.0%) patients the neurological outcome was good, 3/16 (18.8%) patients had unfavourable evolution despite embolization. There was no morbidity mortality related to the procedures themselves. 1/16 (6.3%) patient was lost to follow-up. Overall 12/29 (45.8%) patients had an unfavourable neurological outcome with 11 patients dead and 1 with severe neurological deficit. In the surviving group of children, 17/18 (94.4%) have a good neurological outcome; in 10/18 (55.5%) the lesion is morphologically excluded. Conclusion. DSM is rare disease with high mortality. They usually proceed to either total or partial spontaneous thrombosis before the age of 2 thus compromising normal

  5. Dandy-Walker Malformation Presenting with Affective Symptoms.

    PubMed

    Batmaz, Mert; Balçik, Zeynep Ezgi; Özer, Ürün; Hamurişçi Yalçin, Burcu; Özen, Şakir

    2017-09-01

    Dandy-Walker malformation is defined by enlarged posterior fossa, cystic dilatation of the fourth ventricle, and cerebellar hypoplasia. Although developmental delay and mental retardation are common in Dandy-Walker malformation cases, other comorbid psychiatric conditions have been rarely reported. There are limited numbers of case reports about comorbidity of bipolar disorder with Dandy-Walker malformation in the literature. Herein, a Dandy-Walker malformation case presenting affective symptoms is reported, and psychiatric symptoms which might be seen in this rare malformation are discussed along with diagnosis, treatment, and follow-up processes. A 27-year-old male patient, hospitalized for compulsory treatment, had been diagnosed with Dandy-Walker malformation in childhood. First complaints were attention deficiency, behavioral problems, learning difficulties; and manic and depressive episodes have occurred during follow-ups. He recently complained of decreased need for sleep, irritability, and increased speed of thought, and psychiatric examination was consistent with manic episode. Cranial computed tomography (CT) revealed bilateral ventriculomegaly, enlarged third and fourth ventricles with posterior fossa cyst, and cerebellar hypoplasia. His treatment included 30 mg/day aripiprazole, 1000 mg/day valproic acid, 200 mg/day quetiapine, 4 mg/day biperiden, and 100 mg/month paliperidone palmitate. Beside its traditional role in the regulation of coordination and motor functions, cerebellum is increasingly emphasized for its involvement in the mood regulation. Thus, as seen in Dandy-Walker malformation, cerebellar anomalies are suggested to play a role in the pathophysiology of mood disorders. Further studies are needed to better understand the relationship between mood disorders and cerebellum. Moreover, treatment options should be considered carefully in terms of resistance to treatment and potential side effects, for psychiatric disorders occurring in these

  6. Congenital Vascular Malformation

    MedlinePlus

    ... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel–Trenaunay Syndrome) of the limbs, frequently benefit from elastic garments and bandages used for com- ...

  7. Dural arteriovenous fistula as a treatable dementia

    PubMed Central

    Enofe, Ikponmwosa; Thacker, Ike

    2017-01-01

    Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Although rare, dural arteriovenous fistulas (DAVFs) could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms. DAVFs represent a potentially treatable and reversible cause of dementia. Here, we report the case of an elderly woman diagnosed with a DAVF after presenting with new-onset seizures, deteriorating neurocognitive function, and Parkinson-like symptoms. PMID:28405088

  8. Computed tomography of congenital brain malformations

    DOE Office of Scientific and Technical Information (OSTI.GOV)

    Sarwar, M.

    1984-01-01

    This book is illustrated showing each condition. This book is designed to correlate the pathology of CNS malformations with their CT scan appearance, mainly on the axial images. The author has drawn upon his personal experience and the information gleaned from the literature dealing with the description of the CT scan findings of these malformations. The emphasis is on simplicity of description. Since a large degree of morphological variation exists in each entity, numerous illustrations (wherever applicable) are shown to depict those variations. When appropriate, deficiency of the CT scan in the evaluation of these CNS malformations also is indicated.more » A description of CNS embryology is included as well.« less

  9. Management of lymphatic malformations in children.

    PubMed

    Bagrodia, Naina; Defnet, Ann M; Kandel, Jessica J

    2015-06-01

    To review the literature on lymphatic malformations and to provide current opinion about the management of these lesions. Current treatment options include nonoperative management, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New therapies are emerging, including sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management centers on the patient's quality of life. Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, in addition to knowledge gained from clinical practice. A patient-centered approach should guide timing and modality of treatment. Continued study of lymphatic malformations will increase and solidify a treatment algorithm for these complicated lesions.

  10. Update on the management of anorectal malformations.

    PubMed

    Bischoff, Andrea; Levitt, Marc A; Peña, Alberto

    2013-09-01

    Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a "Pandora's box" that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period.

  11. Quantifying the Cerebral Hemodynamics of Dural Arteriovenous Fistula in Transverse Sigmoid Sinus Complicated by Sinus Stenosis: A Retrospective Cohort Study.

    PubMed

    Guo, W-Y; Lee, C-C J; Lin, C-J; Yang, H-C; Wu, H-M; Wu, C-C; Chung, W-Y; Liu, K-D

    2017-01-01

    Sinus stenosis occasionally occurs in dural arteriovenous fistulas. Sinus stenosis impedes venous outflow and aggravates intracranial hypertension by reversing cortical venous drainage. This study aimed to analyze the likelihood of sinus stenosis and its impact on cerebral hemodynamics of various types of dural arteriovenous fistulas. Forty-three cases of dural arteriovenous fistula in the transverse-sigmoid sinus were reviewed and divided into 3 groups: Cognard type I, type IIa, and types with cortical venous drainage. Sinus stenosis and the double peak sign (occurrence of 2 peaks in the time-density curve of the ipsilateral drainage of the internal jugular vein) in dural arteriovenous fistula were evaluated. "TTP" was defined as the time at which a selected angiographic point reached maximum concentration. TTP of the vein of Labbé, TTP of the ipsilateral normal transverse sinus, trans-fistula time, and trans-stenotic time were compared across the 3 groups. Thirty-six percent of type I, 100% of type IIa, and 84% of types with cortical venous drainage had sinus stenosis. All sinus stenosis cases demonstrated loss of the double peak sign that occurs in dural arteriovenous fistula. Trans-fistula time (2.09 seconds) and trans-stenotic time (0.67 seconds) in types with cortical venous drainage were the most prolonged, followed by those in type IIa and type I. TTP of the vein of Labbé was significantly shorter in types with cortical venous drainage. Six patients with types with cortical venous drainage underwent venoplasty and stent placement, and 4 were downgraded to type IIa. Sinus stenosis indicated dysfunction of venous drainage and is more often encountered in dural arteriovenous fistula with more aggressive types. Venoplasty ameliorates cortical venous drainage in dural arteriovenous fistulas and serves as a bridge treatment to stereotactic radiosurgery in most cases. © 2017 by American Journal of Neuroradiology.

  12. Arterial oxygen content is precisely maintained by graded erythrocytotic responses in settings of high/normal serum iron levels, and predicts exercise capacity: an observational study of hypoxaemic patients with pulmonary arteriovenous malformations.

    PubMed

    Santhirapala, Vatshalan; Williams, Louisa C; Tighe, Hannah C; Jackson, James E; Shovlin, Claire L

    2014-01-01

    Oxygen, haemoglobin and cardiac output are integrated components of oxygen transport: each gram of haemoglobin transports 1.34 mls of oxygen in the blood. Low arterial partial pressure of oxygen (PaO2), and haemoglobin saturation (SaO2), are the indices used in clinical assessments, and usually result from low inspired oxygen concentrations, or alveolar/airways disease. Our objective was to examine low blood oxygen/haemoglobin relationships in chronically compensated states without concurrent hypoxic pulmonary vasoreactivity. 165 consecutive unselected patients with pulmonary arteriovenous malformations were studied, in 98 cases, pre/post embolisation treatment. 159 (96%) had hereditary haemorrhagic telangiectasia. Arterial oxygen content was calculated by SaO2 x haemoglobin x 1.34/100. There was wide variation in SaO2 on air (78.5-99, median 95)% but due to secondary erythrocytosis and resultant polycythaemia, SaO2 explained only 0.1% of the variance in arterial oxygen content per unit blood volume. Secondary erythrocytosis was achievable with low iron stores, but only if serum iron was high-normal: Low serum iron levels were associated with reduced haemoglobin per erythrocyte, and overall arterial oxygen content was lower in iron deficient patients (median 16.0 [IQR 14.9, 17.4]mls/dL compared to 18.8 [IQR 17.4, 20.1]mls/dL, p<0.0001). Exercise tolerance appeared unrelated to SaO2 but was significantly worse in patients with lower oxygen content (p<0.0001). A pre-defined athletic group had higher Hb:SaO2 and serum iron:ferritin ratios than non-athletes with normal exercise capacity. PAVM embolisation increased SaO2, but arterial oxygen content was precisely restored by a subsequent fall in haemoglobin: 86 (87.8%) patients reported no change in exercise tolerance at post-embolisation follow-up. Haemoglobin and oxygen measurements in isolation do not indicate the more physiologically relevant oxygen content per unit blood volume. This can be maintained for SaO2

  13. Arterial Oxygen Content Is Precisely Maintained by Graded Erythrocytotic Responses in Settings of High/Normal Serum Iron Levels, and Predicts Exercise Capacity: An Observational Study of Hypoxaemic Patients with Pulmonary Arteriovenous Malformations

    PubMed Central

    Santhirapala, Vatshalan; Williams, Louisa C.; Tighe, Hannah C.; Jackson, James E.; Shovlin, Claire L.

    2014-01-01

    Background Oxygen, haemoglobin and cardiac output are integrated components of oxygen transport: each gram of haemoglobin transports 1.34 mls of oxygen in the blood. Low arterial partial pressure of oxygen (PaO2), and haemoglobin saturation (SaO2), are the indices used in clinical assessments, and usually result from low inspired oxygen concentrations, or alveolar/airways disease. Our objective was to examine low blood oxygen/haemoglobin relationships in chronically compensated states without concurrent hypoxic pulmonary vasoreactivity. Methodology 165 consecutive unselected patients with pulmonary arteriovenous malformations were studied, in 98 cases, pre/post embolisation treatment. 159 (96%) had hereditary haemorrhagic telangiectasia. Arterial oxygen content was calculated by SaO2 x haemoglobin x 1.34/100. Principal Findings There was wide variation in SaO2 on air (78.5–99, median 95)% but due to secondary erythrocytosis and resultant polycythaemia, SaO2 explained only 0.1% of the variance in arterial oxygen content per unit blood volume. Secondary erythrocytosis was achievable with low iron stores, but only if serum iron was high-normal: Low serum iron levels were associated with reduced haemoglobin per erythrocyte, and overall arterial oxygen content was lower in iron deficient patients (median 16.0 [IQR 14.9, 17.4]mls/dL compared to 18.8 [IQR 17.4, 20.1]mls/dL, p<0.0001). Exercise tolerance appeared unrelated to SaO2 but was significantly worse in patients with lower oxygen content (p<0.0001). A pre-defined athletic group had higher Hb:SaO2 and serum iron:ferritin ratios than non-athletes with normal exercise capacity. PAVM embolisation increased SaO2, but arterial oxygen content was precisely restored by a subsequent fall in haemoglobin: 86 (87.8%) patients reported no change in exercise tolerance at post-embolisation follow-up. Significance Haemoglobin and oxygen measurements in isolation do not indicate the more physiologically relevant oxygen content

  14. Congenital malformations of human dermatoglyphs

    PubMed Central

    David, T. J.

    1973-01-01

    A classification for congenital malformations of dermatoglyphs is presented, dividing them into ridge aplasia, ridge hypoplasia, ridge dissociation, ridges-off-the-end, and a combination of the last two. The medical and genetic significance of these are considered in the light both of previous published cases and of new material. Malformations of dermatoglyphs are important as physical signs in paediatric diagnosis. ImagesFIG. 1FIG. 2FIG. 3FIG. 4FIG. 5FIG. 6FIG. 7 PMID:4693462

  15. Atherosclerosis and Stroke

    MedlinePlus

    ... Stroke and atherosclerosis There are two types of ischemic stroke caused by blood clots, narrowing of blood vessels ... 2 What Is an Arteriovenous Malformation (AVM)? 3 Ischemic Strokes (Clots) 4 Hemorrhagic Strokes (Bleeds) 5 What You ...

  16. Dandy-Walker malformation: analysis of 38 cases.

    PubMed

    Pascual-Castroviejo, I; Velez, A; Pascual-Pascual, S I; Roche, M C; Villarejo, F

    1991-04-01

    Thirty-eight cases of Dandy-Walker malformation (DWM) are presented. A female predominance of 3:1 was found. Thirty-two cases (84%) were diagnosed within the 1st year of life. Of these, 17 cases (44.7%) were diagnosed at birth. Ten (26%) were delivered by cesarean section. Thirteen infants (34%) had a birth weight below 3000 g. Several associated malformations were observed, the most frequent being capillary angioma (6 cases); cardiac malformations, ophthalmic anomalies, agenesis of the corpus callosum, malformed limbs, and occipital meningocele were also seen. These observations indicate that DWM represents a disorder of the midline central nervous system indicative of marked genetic and etiologic heterogeneity with the possibility of showing clinical and pathological alterations intra- and extracranially. Macrocephaly was the most frequent physical finding, appearing in 31 cases (82%). Seventeen (44.7%) patients died, 11 before 6 months of age, 3 between 6 and 12 months, and 3 after 1 year. Postmortem studies were performed in 13 patients. Three cases have been lost to follow-up. Mental retardation (IQ below 70) was found in 11 cases (58% of survivors), low intellect (IQ between 70 and 85) in 4, and only 2 patients showed normal intellectual development (IQ more than 85). The high incidence of malformations having several genetic and environmental origins, as well as the high early mortality of patients with DWM, indicate the complexity of this syndrome, which involves the midline developmental field structures. It is not an isolated malformation of the posterior fossa in most cases.

  17. The presentation and management of complex female genital malformations.

    PubMed

    Acién, Pedro; Acién, Maribel

    2016-01-01

    Common uterine anomalies are important owing to their impact on fertility, and complex mesonephric anomalies and certain Müllerian malformations are particularly important because they cause serious clinical symptoms and affect woman's quality of life, in addition to creating fertility problems. In these cases of complex female genital tract malformations, a correct diagnosis is essential to avoid inappropriate and/or unnecessary surgery. Therefore, acquiring and applying the appropriate embryological knowledge, management and therapy is a challenge for gynaecologists. Here, we considered complex malformations to be obstructive anomalies and/or those associated with cloacal and urogenital sinus anomalies, urinary and/or extragenital anomalies, or other clinical implications or symptoms creating a difficult differential diagnosis. A diligent and comprehensive search of PubMed and Scopus was performed for all studies published from 1 January 2011 to 15 April 2015 (then updated up to September 2015) using the following search terms: 'management' in combination with either 'female genital malformations' or 'female genital tract anomalies' or 'Müllerian anomalies'. The MeSH terms 'renal agenesis', 'hydrocolpos', 'obstructed hemivagina' 'cervicovaginal agenesis or atresia', 'vaginal agenesis or atresia', 'Herlyn-Werner-Wunderlich syndrome', 'uterine duplication' and 'cloacal anomalies' were also used to compile a list of all publications containing these terms since 2011. The basic embryological considerations for understanding female genitourinary malformations were also revealed. Based on our experience and the updated literature review, we studied the definition and classification of the complex malformations, and we analysed the clinical presentation and different therapeutic strategies for each anomaly, including the embryological and clinical classification of female genitourinary malformations. From 755 search retrieved references, 230 articles were analysed and

  18. Slide tracheoplasty outcomes in children with congenital pulmonary malformations.

    PubMed

    DeMarcantonio, Michael A; Hart, Catherine K; Yang, Christina J; Tabangin, Meredith; Rutter, Michael J; Bryant, Roosevelt; Manning, Peter B; de Alarcón, Alessandro

    2017-06-01

    Evaluate and compare surgical outcomes of slide tracheoplasty for the treatment of congenital tracheal stenosis in children with and without pulmonary malformations. Retrospective chart review at a tertiary care pediatric medical center. We identified patients with tracheal stenosis who underwent slide tracheoplasty from 2001 to 2014, and a subset of these patients who were diagnosed with congenital pulmonary malformations. Hospital course and preoperative and postoperative complications were recorded. One hundred thirty patients (18 with pulmonary malformations, 112 with normal pulmonary anatomy) were included. Pulmonary malformations included unilateral pulmonary agenesis (61%) and hypoplasia (39%). Children with pulmonary malformations had a greater median age compared to their normal lung anatomy counterparts. Preoperatively, patients with pulmonary malformations more frequently required preoperative mechanical ventilation (55.6% vs. 21.3%, P = .007), extracorporeal membrane oxygenation (ECMO) (11% vs. 0.9%, P = .05), and tracheostomy (22.2% vs. 3.6%, P = .01). Postoperatively, patients with pulmonary malformations more frequently required mechanical ventilation >48 hours (78% vs. 37%, P =.005) and ECMO use (11% vs. 0.9%, P = .05). Pulmonary malformation patients and children with normal anatomy did not differ in terms of postoperative tracheostomy (16.7% vs. 4.4%, P > .05), dehiscence (6% vs. 0%, P > .05%), restenosis (11% vs. 6%, P > .05) or postoperative figure 8 deformity (6% vs. 3%, P > .05). Mortality, however, was significantly increased (22.2% vs. 3.6%, P = .01) in children with pulmonary malformations. Although slide tracheoplasty can be successfully performed in patients with abnormal pulmonary anatomy, surgeons and families should anticipate a more difficult postoperative course, with possible associated prolonged mechanical ventilation, ECMO use, and higher mortality than in children with tracheal stenosis alone. 4. Laryngoscope, 127:1283-1287, 2017

  19. Chronic cerebral herniation in shunted Dandy-Walker malformation.

    PubMed

    Naidich, T P; Radkowski, M A; McLone, D G; Leestma, J

    1986-02-01

    A review of serial computed tomography (CT) scans of 25 patients with the Dandy-Walker malformation revealed six patients with chronic downward transincisural herniation of the cerebrum after shunt decompression of the posterior fossa cyst or malfunction of a lateral ventricular drainage catheter, or both. Chronic cerebral herniation was detected postmortem in a seventh patient with the Dandy-Walker malformation. The CT findings and autopsy appearance of this previously undescribed feature of shunted Dandy-Walker malformation are illustrated.

  20. Dandy-Walker Malformation Presenting with Affective Symptoms

    PubMed Central

    BATMAZ, Mert; BALÇIK, Zeynep Ezgi; ÖZER, Ürün; HAMURİŞÇİ YALÇIN, Burcu; ÖZEN, Şakir

    2017-01-01

    Dandy-Walker malformation is defined by enlarged posterior fossa, cystic dilatation of the fourth ventricle, and cerebellar hypoplasia. Although developmental delay and mental retardation are common in Dandy-Walker malformation cases, other comorbid psychiatric conditions have been rarely reported. There are limited numbers of case reports about comorbidity of bipolar disorder with Dandy-Walker malformation in the literature. Herein, a Dandy-Walker malformation case presenting affective symptoms is reported, and psychiatric symptoms which might be seen in this rare malformation are discussed along with diagnosis, treatment, and follow-up processes. A 27-year-old male patient, hospitalized for compulsory treatment, had been diagnosed with Dandy-Walker malformation in childhood. First complaints were attention deficiency, behavioral problems, learning difficulties; and manic and depressive episodes have occurred during follow-ups. He recently complained of decreased need for sleep, irritability, and increased speed of thought, and psychiatric examination was consistent with manic episode. Cranial computed tomography (CT) revealed bilateral ventriculomegaly, enlarged third and fourth ventricles with posterior fossa cyst, and cerebellar hypoplasia. His treatment included 30 mg/day aripiprazole, 1000 mg/day valproic acid, 200 mg/day quetiapine, 4 mg/day biperiden, and 100 mg/month paliperidone palmitate. Beside its traditional role in the regulation of coordination and motor functions, cerebellum is increasingly emphasized for its involvement in the mood regulation. Thus, as seen in Dandy-Walker malformation, cerebellar anomalies are suggested to play a role in the pathophysiology of mood disorders. Further studies are needed to better understand the relationship between mood disorders and cerebellum. Moreover, treatment options should be considered carefully in terms of resistance to treatment and potential side effects, for psychiatric disorders occurring in these

  1. Appreciating the broad clinical features of SMAD4 mutation carriers: a multicenter chart review.

    PubMed

    Wain, Karen E; Ellingson, Marissa S; McDonald, Jamie; Gammon, Amanda; Roberts, Maegan; Pichurin, Pavel; Winship, Ingrid; Riegert-Johnson, Douglas L; Weitzel, Jeffrey N; Lindor, Noralane M

    2014-08-01

    Heterozygous loss-of-function SMAD4 mutations are associated with juvenile polyposis syndrome and hereditary hemorrhagic telangiectasia. Some carriers exhibit symptoms of both conditions, leading to juvenile polyposis-hereditary hemorrhagic telangiectasia syndrome. Three families have been reported with connective tissue abnormalities. To better understand the spectrum and extent of clinical findings in SMAD4 carriers, medical records of 34 patients (20 families) from five clinical practices were reviewed. Twenty-one percent of the patients (7/34) had features suggesting a connective tissue defect: enlarged aortic root (n = 3), aortic and mitral insufficiency (n = 2), aortic dissection (n = 1), retinal detachment (n = 1), brain aneurysms (n = 1), and lax skin and joints (n = 1). Juvenile polyposis-specific findings were almost uniformly present but variable. Ninety-seven percent of the patients had colon polyps that were generally pan-colonic and of variable histology and number. Forty-eight percent of the patients (15/31) had extensive gastric polyposis. Hereditary hemorrhagic telangiectasia features, including epistaxis (19/31, 61%), mucocutaneous telangiectases (15/31, 48%), liver arteriovenous malformation (6/16, 38%), brain arteriovenous malformation (1/26, 4%), pulmonary arteriovenous malformation (9/17, 53%), and intrapulmonary shunting (14/23, 61%), were documented in 76% of the patients. SMAD4 carriers should be managed for juvenile polyposis and hereditary hemorrhagic telangiectasia because symptoms of both conditions are likely yet unpredictable. Connective tissue abnormalities are an emerging component of juvenile polyposis-hereditary hemorrhagic telangiectasia syndrome, and larger studies are needed to understand these manifestations.

  2. A developmental and genetic classification for midbrain-hindbrain malformations

    PubMed Central

    Millen, Kathleen J.; Dobyns, William B.

    2009-01-01

    Advances in neuroimaging, developmental biology and molecular genetics have increased the understanding of developmental disorders affecting the midbrain and hindbrain, both as isolated anomalies and as part of larger malformation syndromes. However, the understanding of these malformations and their relationships with other malformations, within the central nervous system and in the rest of the body, remains limited. A new classification system is proposed, based wherever possible, upon embryology and genetics. Proposed categories include: (i) malformations secondary to early anteroposterior and dorsoventral patterning defects, or to misspecification of mid-hindbrain germinal zones; (ii) malformations associated with later generalized developmental disorders that significantly affect the brainstem and cerebellum (and have a pathogenesis that is at least partly understood); (iii) localized brain malformations that significantly affect the brain stem and cerebellum (pathogenesis partly or largely understood, includes local proliferation, cell specification, migration and axonal guidance); and (iv) combined hypoplasia and atrophy of putative prenatal onset degenerative disorders. Pertinent embryology is discussed and the classification is justified. This classification will prove useful for both physicians who diagnose and treat patients with these disorders and for clinical scientists who wish to understand better the perturbations of developmental processes that produce them. Importantly, both the classification and its framework remain flexible enough to be easily modified when new embryologic processes are described or new malformations discovered. PMID:19933510

  3. Brainstem conundrum: the Chiari I malformation.

    PubMed

    Mueller, D

    2001-04-01

    To describe the Chairi I Malformation in relation to the anatomy of the brain and spinal cord, the common manifestations of the condition, diagnostic considerations, and management for the primary care provider. Extensive review of the world-wide scientific literature on the condition, supplemented with actual case studies. The adult Chairi I Malformation is an insidious congenital brainstem anomaly that consists of caudal displacement of the cerebellar tonsils, brainstem and fourth ventricle into the upper cervical space, resulting in overcrowding of the posterior fossa. Due to the vague, and often ambiguous presenting symptoms of Chiari I Malformation, many patients are misdiagnosed with conditions such as multiple sclerosis, fibromyalgia, chronic fatigue syndrome, or psychiatric disorders. Patients frequently experience symptoms months to years prior to accurate diagnosis and often incur irreversible neurologic deficits.

  4. Field guide to malformations of frogs and toads: with radiographic interpretations

    USGS Publications Warehouse

    Meteyer, Carol U.

    2000-01-01

    In 1995, students found numerous malformed frogs on a field trip to a Minnesota pond. Since that time, reports of malformed frogs have increased dramatically. Malformed frogs have now been reported in 44 states in 38 species of frogs, and 19 species of toads. Estimates as high as 60% of the newly metamorphosed frog populations have had malformations at some ponds (NARCAM, ’99). The wide geographic distribution of malformed frogs and the variety of malformations are a concern to resource managers, research scientists and public health officials. The potential for malformations to serve as a signal of ecosystem disruption, and the affect this potential disruption might have on other organisms that share those ecosystems, has not been resolved. Malformations represent an error that occurred early in development. The event that caused the developmental error is temporally distant from the malformation we see in the fully developed animal. Knowledge of normal developmental principles is necessary to design thoughtful investigations that will define the events involved in abnormal development in wild frog populations.Development begins at the time an egg is fertilized and progresses by chemical communication between cells and cell layers. This communication is programmed through gene expression. Malformations represent primary errors in development, errors in chemical communication or translation of genetic information. Deformations arise later in development and usually result from the influence of mechanical factors (such as amputation) that alter shape or anatomy of a structure that has developed normally. The occurrence and the type of malformations are influenced by the type of error or insult as well as the timing of the error (the developmental stage at which the error occurred). The appearance of the malformation can therefore provide clues that suggest when the error may have occurred. If the malformation is an incomplete organ, such as an incomplete limb, the

  5. CONGENITAL HEART MALFORMATIONS IN NEWBORN BABIES WITH LOW BIRTH WEIGHT.

    PubMed

    Luca, Alina-Costina; Holoc, Andreea-Simona; Iordache, C

    2015-01-01

    Congenital heart malformations represent a public health problem, holding a significant percentage of the total of heart diseases. Beside the elevated frequency of the malformations, we also notice their occurrence in newborn babies with low birth weight, increasing, thus, the risk of complications and late therapeutic approach. The goal of the study was to highlight the general and particular aspects of cardiovascular malformations epidemiology in newborn babies with low weight at birth, the correlation of the malformations with implied genetic and environmental factors, assessing the complications and their procedures on the therapeutic management. Our study was performed on a group of 271 patients, hospitalized in the Department of Pediatric Cardiology of "Sf. Maria" Emergency Clinical Hospital for Children of Iasi, during January 2011-December 2013. The patients were assessed based on anamnesis, clinical, biological and imagistic exam. The study lot was divided according to the type of the structural defect: 95% of the patients were diagnosed with non-cyanogenic congenital heart malformations and 5% with cyanogenic congenital heart malformations. Regarding the patient's origin background, we notice an elevated frequency of the rural environment (71%). The incidence of the malformations was high in premature low birth weight (48%), followed by premature very low birth weight (22%). In evolution, congenital heart malformations often get more complicated heart failure, arterial hypertension and respiratory infections being most often met. Mortality was maximum in the first year of life, a third of the cases being associated with chromosomal malformations. Congenital heart malformations in newborn patients with low weight at birth represented an elevated percentage of 44.13% of the total of the cases hospitalized for cardiovascular diseases from the Department of Pediatric Cardiology of Iasi. Many cases were associated with other congenital malformations or

  6. Abernethy malformation: a case report

    PubMed Central

    2012-01-01

    Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4 weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients. PMID:22642663

  7. Adding a PECS II block for proximal arm arteriovenous access - a randomised study.

    PubMed

    Quek, K H; Low, E Y; Tan, Y R; Ong, A S C; Tang, T Y; Kam, J W; Kiew, A S C

    2018-05-01

    Brachial plexus block is often utilised for proximal arm arteriovenous access creation. However, the medial upper arm and axilla are often inadequately anaesthetised, requiring repeated, intraoperative local anaesthetic supplementation, or conversion into general anaesthesia. We hypothesised that the addition of a PECS II block would improve anaesthesia and analgesia for proximal arm arteriovenous access surgery. In this prospective, double-blinded, randomised proof-of-concept study, 36 consenting adults with end-stage renal disease aged between 21 and 90 years received either a combined supraclavicular and PECS II block (Group PECS, n = 18), or combined supraclavicular and sham block (Group SCB, n = 18) for proximal arm arteriovenous access surgery. Primary outcome was whether patients required intraoperative local anaesthetic supplementation by the surgeon. In Group PECS, 33.3% (6/18) needed local anaesthetic supplementation vs. 100% (18/18) in Group SCB. Group SCB had three times (RR 3.0, 95% CI 1.6-5.8; P < 0.001) the risk of requiring intraoperative local anaesthetic supplementation. Group PECS required lower volume of supplemental local anaesthetic compared to Group SCB (0.0 ml, IQR 0.0-6.3 ml vs. 15.0 ml, IQR 7.4-17.8 ml; P < 0.001). Group SCB had twice [RR 2.2, 95% CI 1.1-4.4; (P = 0.019)] the risk of needing additional sedation or analgesia. There were no significant differences between the groups with respect to postoperative visual analogue scale pain scores, time to first rescue analgesia or patient satisfaction. The results suggest that adding a PECS II block to a supraclavicular block improves regional anaesthesia for patients with end-stage renal disease undergoing proximal arm arteriovenous access surgery. © 2018 The Acta Anaesthesiologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.

  8. Dandy-Walker malformation: a rare association with hypoparathyroidism.

    PubMed

    Coban, Dilek; Akin, Mustafa Ali; Kurtoglu, Selim; Oktem, Suat; Yikilmaz, Ali

    2010-12-01

    Dandy-Walker malformation is characterized by cystic dilatation of the fourth ventricle and an enlarged posterior cranial fossa with upward displacement of the tentorium, lateral sinuses, and torcular, with agenesis or hypoplasia of the cerebellar vermis. Dandy-Walker malformation occurs in approximately the 4th week of gestation and is associated with various abnormalities involving the cardiac, skeletal, genitourinary, and gastrointestinal systems. The parathyroid gland also forms in the 3rd and 4th gestational weeks. Reported here is the case of a male infant with Dandy-Walker malformation with ventricular and atrial septal defect, unilateral renal agenesis, and hypoparathyroidism. To our knowledge, this rare association with neural crest events during the development of Dandy-Walker malformation has not been reported previously. Crown Copyright © 2010. Published by Elsevier Inc. All rights reserved.

  9. cis-Regulatory Mutations Are a Genetic Cause of Human Limb Malformations

    PubMed Central

    VanderMeer, Julia E.; Ahituv, Nadav

    2011-01-01

    The underlying mutations that cause human limb malformations are often difficult to determine, particularly for limb malformations that occur as isolated traits. Evidence from a variety of studies shows that cis-regulatory mutations, specifically in enhancers, can lead to some of these isolated limb malformations. Here, we provide a review of human limb malformations that have been shown to be caused by enhancer mutations and propose that cis-regulatory mutations will continue to be identified as the cause of additional human malformations as our understanding of regulatory sequences improves. PMID:21509892

  10. Dose fractionated gamma knife radiosurgery for large arteriovenous malformations on daily or alternate day schedule outside the linear quadratic model: Proof of concept and early results. A substitute to volume fractionation.

    PubMed

    Mukherjee, Kanchan Kumar; Kumar, Narendra; Tripathi, Manjul; Oinam, Arun S; Ahuja, Chirag K; Dhandapani, Sivashanmugam; Kapoor, Rakesh; Ghoshal, Sushmita; Kaur, Rupinder; Bhatt, Sandeep

    2017-01-01

    To evaluate the feasibility, safety and efficacy of dose fractionated gamma knife radiosurgery (DFGKRS) on a daily schedule beyond the linear quadratic (LQ) model, for large volume arteriovenous malformations (AVMs). Between 2012-16, 14 patients of large AVMs (median volume 26.5 cc) unsuitable for surgery or embolization were treated in 2-3 of DFGKRS sessions. The Leksell G frame was kept in situ during the whole procedure. 86% (n = 12) patients had radiologic evidence of bleed, and 43% (n = 6) had presented with a history of seizures. 57% (n = 8) patients received a daily treatment for 3 days and 43% (n = 6) were on an alternate day (2 fractions) regimen. The marginal dose was split into 2 or 3 fractions of the ideal prescription dose of a single fraction of 23-25 Gy. The median follow up period was 35.6 months (8-57 months). In the three-fraction scheme, the marginal dose ranged from 8.9-11.5 Gy, while in the two-fraction scheme, the marginal dose ranged from 11.3-15 Gy at 50% per fraction. Headache (43%, n = 6) was the most common early postoperative complication, which was controlled with short course steroids. Follow up evaluation of at least three years was achieved in seven patients, who have shown complete nidus obliteration in 43% patients while the obliteration has been in the range of 50-99% in rest of the patients. Overall, there was a 67.8% reduction in the AVM volume at 3 years. Nidus obliteration at 3 years showed a significant rank order correlation with the cumulative prescription dose (p 0.95, P value 0.01), with attainment of near-total (more than 95%) obliteration rates beyond 29 Gy of the cumulative prescription dose. No patient receiving a cumulative prescription dose of less than 31 Gy had any severe adverse reaction. In co-variate adjusted ordinal regression, only the cumulative prescription dose had a significant correlation with common terminology criteria for adverse events (CTCAE) severity (P value 0.04), independent of age, AVM volume

  11. Contribution of Rare Copy Number Variants to Isolated Human Malformations

    PubMed Central

    Serra-Juhé, Clara; Rodríguez-Santiago, Benjamín; Cuscó, Ivon; Vendrell, Teresa; Camats, Núria; Torán, Núria; Pérez-Jurado, Luis A.

    2012-01-01

    Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n = 7) or very uncommon (n = 15, <1/2,000) in the control population] in 20/95 fetuses with congenital malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases. PMID:23056206

  12. Klippel-Feil syndrome and Dandy-Walker malformation.

    PubMed

    Karaman, A; Kahveci, H

    2011-01-01

    The Klippel-Feil deformity is a complex of osseous and visceral anomalies, which include low hairline, platybasia, fused cervical vertebrae with a short neck, and deafness. Associated central nervous system abnormalities include occipital cephalocele, Chiari I malformation, syrinx, microcephaly, and hydrocephalus. Herein, we report a case with Klippel-Feil syndrome and Dandy-Walker malformation.

  13. The Chiari 3 Malformation and a Systemic Review of the Literature.

    PubMed

    Young, Richard M; Shafa, Justin S; Myseros, John S

    2015-01-01

    Chiari type 3 is a rare hindbrain malformation that has been reported in the literature primarily as case reports and case series. Radiological, pathophysiological and surgical definitions of the malformation are inconsistent in the literature and subsequently can be confusing, and outcomes have also been uniformly poor. The definition of this rare malformation will be clarified through a case presentation. A retrospective review of prior publications in the PubMed and MEDLINE databases was performed looking for reports of 'Chiari 3 +/- malformation' and 'occipital encephalocele'. Relevant papers were reviewed and compiled into table format with associated descriptions of a Chiari type 3 malformation. A case illustration is presented with radiological and intraoperative imaging to reinforce and clarify the definition. Upon review of the prior publications in the detail of the descriptions and imaging associated with each article, there is a wide range of variability in the description of what is considered a Chiari 3 malformation. Occipital, occipitocervical and high cervical defects have all been described as Chiari 3 malformation. Our case illustration presents a patient with an occipitocervical encephalocele with neural elements, which is the classic and accepted definition of the Chiari 3 malformation. Chiari type 3 is a rare congenital malformation, and prior publications describing this developmental disorder have not demonstrated a consensus in its definition. In addition, outcomes have traditionally been reported as poor. This case illustration of a Chiari type 3 enforces the definition of an occipitocervical encephalocele with hindbrain herniation, and with proper management not all Chiari 3 malformation patients have bad outcomes. © 2015 S. Karger AG, Basel.

  14. Angiolymphoid Hyperplasia with Eosinophilia: A Case Report

    PubMed Central

    Esteves, Paola; Barbalho, Marcella; Lima, Tiago; Quintella, Leonardo; Niemeyer-Corbellini, João Paulo; Ramos-e-Silva, Marcia

    2015-01-01

    Angiolymphoid hyperplasia with eosinophilia is a rare disease considered as being a vascular malformation resulting from a subjacent arteriovenous shunt. It affects mostly the head of women between 20 and 40 years old and may present spontaneous involution. PMID:26120306

  15. Posterior axial corneal malformation and uveoretinal angiodysgenesis--a neurocristopathy?

    PubMed

    Mooy, C M; Clark, B J; Lee, W R

    1990-01-01

    This clinicopathological report describes an unusual combination of axial corneal malformation and angiodysgenesis in the uvea, retina and optic nerve in three eyes. In each specimen there was hypocellularity in the posterior axial stroma, with corresponding loss of the corneal endothelium. The vascular malformation consisted of numerous telangiectatic endothelium-lined tubes with inconspicuous or absent media. One globe was obtained from a stillborn fetus (36 weeks) in which renal agenesis and a sireniform malformation (mermaid fetus) occurred in conjunction with a Fallot's tetralogy, pulmonary hypoplasia and atresia of the trachea and duodenum. Eyes with almost identical malformations were obtained from a 39-week female neonate who died after 5 h as a consequence of renal agenesis and pulmonary hypoplasia. This combination of ocular tissue malformations can be explained by embryological studies, which have shown that the corneal stroma and endothelium and the ocular periendothelial vascular tissues are derived from the neural crest.

  16. Recurrent Hyperammonemia After Abernethy Malformation Type 2 Closure: a Case Report.

    PubMed

    Li, Hui; Ma, Zhi; Xie, Ying; Tian, Feng

    The Abernethy malformation is a rare congenital malformation defined by the presence of an extrahepatic portosystemic shunt. Although most patients are asymptomatic, clinical encephalopathy is present in 15% of cases. We present a patient with type 2 Abernethy malformation, hyperammonemia, and encephalopathy. Shunt closure was performed successfully using interventional angiography; however, hyperammonemia recurred 3 months later. The diagnosis of Abernethy malformation can be made easily, but the ideal patient management strategy has not yet been established. This is the first reported patient with recurrence of hyperammonemia after interventional treatment; we discuss the therapeutic options for Abernethy malformation.

  17. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation.

    PubMed

    Lisovsky, Mikhail; Konstas, Angelos A; Misdraji, Joseph

    2011-09-01

    Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins. Aside from the complete or near-complete absence of portal veins, other histologic features of Abernethy malformation have not been evaluated in the literature. The goal of this study was to detail the hepatic histopathology in 5 patients with Abernethy malformation diagnosed at our institution. Paraffin-embedded tissue sections from 1 explant, 2 liver tumor resections, and 2 liver biopsies were evaluated using hematoxylin and eosin stains, reticulin, elastic, and trichrome stains, and immunohistochemistry for D2-40. Histologic findings included absence of portal veins in small portal tracts, absent or hypoplastic portal veins in medium-sized and large-sized portal tracts, isolated capillaries and arterioles in the lobules, hypertrophy of hepatic artery branches, remodeling of the liver architecture, and nodular regenerative hyperplasia in 1 case. Two patients had hepatocellular carcinoma without cirrhosis, and 2 had focal nodular hyperplasia. In addition to loss of portal veins, Abernethy malformation is characterized by multiple abnormalities due to remodeling of the hepatic vasculature. Abernethy malformation may also be associated with hepatocellular carcinoma and focal nodular hyperplasia in some patients.

  18. Neuroimaging of Dandy-Walker malformation: new concepts.

    PubMed

    Correa, Gustavo Gumz; Amaral, Lázaro Faria; Vedolin, Leonardo Modesti

    2011-12-01

    Dandy-Walker malformation (DWM) is the most common human cerebellar malformation, characterized by hypoplasia of the cerebellar vermis, cystic dilation of the fourth ventricle, and an enlarged posterior fossa with upward displacement of the lateral sinuses, tentorium, and torcular. Although its pathogenesis is not completely understood, there are several genetic loci related to DWM as well as syndromic malformations and congenital infections. Dandy-Walker malformation is associated with other central nervous system abnormalities, including dysgenesis of corpus callosum, ectopic brain tissue, holoprosencephaly, and neural tube defects. Hydrocephalus plays an important role in the development of symptoms and neurological outcome in patients with DWM, and the aim of surgical treatment is usually the control of hydrocephalus and the posterior fossa cyst. Imaging modalities, especially magnetic resonance imaging, are crucial for the diagnosis of DWM and distinguishing this disorder from other cystic posterior fossa lesions. Persistent Blake's cyst is seen as a retrocerebellar fluid collection with cerebrospinal fluid signal intensity and a median line communication with the fourth ventricle, commonly associated with hydrocephalus. Mega cisterna magna presents as an extraaxial fluid collection posteroinferior to an intact cerebellum. Retrocerebellar arachnoid cysts frequently compress the cerebellar hemispheres and the fourth ventricle. Patients with DWM show an enlarged posterior fossa filled with a cystic structure that communicates freely with the fourth ventricle and hypoplastic vermis. Comprehension of hindbrain embryology is of utmost importance for understanding the cerebellar malformations, including DWM, and other related entities.

  19. [Coexistence of female sexual organ malformation and urinary tract anomalies].

    PubMed

    Rzymski, P; Szpakowska-Rzymska, I; el Yubi, R; Wilczak, M; Sajdak, S; Opala, T

    2001-02-01

    The aim of the study was to estimate the correlation between sexual organs and urinary tract malformation. The retrospective analysis of clinical data obtained from 50 patients with sexual organs anomalies diagnosed in the Academic Gynaecological Centre between 1992-1999 was performed. Material included 24 patients with the Meyer-Rokitansky-Küster-Hauser syndrome and 26 with other sexual organs' malformations. Frequency of urinary tract anomalies was 42%, 11 patients with Meyer-Rokitansky-Küster-Hauser syndrome and 11 with other malformations. The gravity of urinary tract anomalies showed no statistical significant difference dependent on the type and symmetry of genital malformation. Urinary tract anomalies were more frequent in cases of asymmetric genital malformation and the difference was statistically significant. Intravenous urography proofed to be more sensitive than ultrasonography in diagnosing urinary tract anomalies.

  20. Angiotensin-Converting Enzyme Inhibitors and the Risk of Congenital Malformations

    PubMed Central

    Bateman, Brian T; Patorno, Elisabetta; Desai, Rishi J; Seely, Ellen W; Mogun, Helen; Dejene, Sara Z; Fischer, Michael A; Friedman, Alexander M; Hernandez-Diaz, Sonia; Huybrechts, Krista F

    2016-01-01

    Objective To examine the association between first-trimester angiotensin-converting enzyme (ACE) inhibitor exposure and the risk for overall major congenital, cardiac, and central nervous system (CNS) malformations. Methods We used a cohort of completed pregnancies linked to liveborn infants derived from Medicaid claims from 2000 to 2010. We examined the risk of malformations associated with first-trimester exposure to an ACE inhibitor. Propensity score based methods were used to control for potential confounders including maternal demographics, medical conditions, exposure to other medications, and measures of health care utilization. Results The cohort included 1,333,624 pregnancies, of which 4,107 (0.31%) were exposed to ACE inhibitors during the first trimester. The prevalence of overall malformations in the ACE inhibitor–exposed was 5.9% versus 3.3% in the unexposed (unadjusted relative risk (RR), 1.82; 95% confidence interval (CI) 1.61 to 2.06), of cardiac malformations was 3.4% versus 1.2% (RR 2.95; 95% CI 2.50 to 3.47), and of CNS malformations was 0.27% versus 0.18% (RR 1.46; 95% CI 0.81 to 2.64). After restricting the cohort to pregnancies complicated by chronic hypertension (both exposed and unexposed) and accounting for other confounding factors, there was no significant increase in the risk for any of the outcomes assessed. Relative risks associated with first-trimester ACE inhibitor exposure were 0.89 (95% CI 0.75 to 1.06) for overall malformations, 0.95 (95% CI 0.75 to 1.21) for cardiac malformations, and 0.54 (95% CI 0.26 to 1.11) for CNS malformations. Conclusions After accounting for confounders, among women with hypertension, exposure to ACE inhibitors during the first trimester was not associated with an increased risk of major congenital malformations. PMID:27926639