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Sample records for intracranial vascular malformations

  1. Sudden Death From Ruptured Intracranial Vascular Malformations During Mechanical Asphyxia: A Domestic Violence Case Report.

    PubMed

    Wu, Xue-Mei; Zhang, Xu-Dong; Yun, Li-Bing; Liu, Min; Yi, Xu-Fu

    2017-03-01

    Smothering and manual strangulation are not uncommon in domestic violence against women; however, no report on the combination of mechanical asphyxia and intracranial vascular malformations has been previously published. We report a middle-aged woman who was smothered and manually strangled by her husband and subsequently died from subarachnoid hemorrhage due to ruptured intracranial vascular malformations, rather than direct mechanical asphyxiation. Smothering and manual strangulation are considered provocative conditions for rupture and contributory causes of death. In this case study, we underline the importance of meticulous autopsy in cases of mechanical asphyxia and intracranial hemorrhage. Exclusion of underlying diseases that may have caused or contributed to death is also required, despite serious asphyxiation signs and neck injuries. Postmortem angiography is a valuable complement to autopsy to detect vascular pathology, with good prospects for further development in China.

  2. Clinical results of stereotactic heavy-charged-particle radiosurgery for intracranial angiographically occult vascular malformations

    SciTech Connect

    Levy, R.P.; Fabrikant, J.I.; Phillips, M.H.; Frankel, K.A.; Steinberg, G.K.; Marks, M.P.; DeLaPaz, R.L.; Chuang, F.Y.S.; Lyman, J.T.

    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, clinical and neuroradiologic results and complications encountered, and discusses the strengths and limitations of the method. 10 refs., 1 fig.

  3. Trends in the Management of Intracranial Vascular Malformations in the USA from 2000 to 2007

    PubMed Central

    Choi, Jae H.; Pile-Spellman, John; Brisman, Jonathan

    2012-01-01

    Objective. To assess prevalence, clinical characteristics, trends in treatment pattern, and outcome in patients with intracranial vascular malformations (IVMs). Methods. Nationwide inpatient sample. Patients with the diagnosis of an IVM admitted to US hospitals from 2000 to 2007. Results. In 58,051 IVM-related admissions (detection rate 2.4/100,000 person-years; mean age 49 ± 17 years; 52% women) major diagnoses were intracranial hemorrhage (ICrH) in 15%, seizure 32%, ischemia 5%, and headache 9%. Procedures included surgery (13%), embolization (13%), radiation therapy (2%), aneurysm clipping (1%), and mechanical ventilation (6%). Ventilation and ICrH were associated with death (2%), whereas ventilation, ICrH, surgery, seizure, and ischemia were associated with unfavorable outcome (20%). IVM detection rate and hospital outcome remained stable over time, whereas mean age and comorbid diagnosis of cerebral ischemia increased (ICrH and seizure decreased). Conclusion. IVMs are infrequent and present in 1/6 patients with some form of ICrH. Overall, seizure is the dominant comorbid diagnosis (1/3 patients). IVMs are equally prevalent among race-ethnic groups and are increasingly detected later in life. The inpatient care of IVM patients results in death or discharge into specialized care in 1/5 patients. PMID:22550618

  4. [Multiple intracranial arteriovenous malformation].

    PubMed

    Gelabert-González, Miguel; Santin-Amo, José María; Román-Pena, Paula; Vázquez Herrero, Fernando

    2015-01-01

    Multiple cerebral arteriovenous malformations (AVMs) are thought to be exceedingly rare lesions and have usually been reported as single cases. The incidence of multiple cerebral AVMs in major series ranges from 0.3% to 9% and, in the majority of cases, these malformations are associated with other vascular anomalies of the brain or soft tissues. We report a 62-year-old woman that presented with a left temporal haemorrhage. Angiography showed 3 AVMs located in the left temporal lobe, left cerebellar hemisphere and right temporal lobe. The lesions were treated with radiosurgery.

  5. [Intracranial arteriovenous malformations in Taiwan].

    PubMed

    Lin, L S; Shih, C J

    1993-12-01

    This paper analyzes the available literature on intracranial arteriovenous malformations (AVM) in Taiwan. The incidence and symptoms of the disease are studied with a view to assisting practitioners in its recognition. The incidence of intracranial AVM in patients who have suffered hemorrhagic stroke in Taiwan is 2.5% to 4.8%, with the male to female ratio being 1.5:1. The peak age at which bleeding from intracranial AVM occurred ranged from 10 to 40 years; bleeding showed no seasonal variation. Sudden headaches, vomiting, and disturbance of consciousness were the commonest presenting symptoms of AVM, similar to the rupture of intracranial aneurysms. However, the possibility of focal neurological deficit among patients with intracranial AVM was higher than in patients with intracranial aneurysms. Risk factors, such as hypertension, diabetes mellitus, heart disease, smoking and alcohol intake showed no close relationship to bleeding in intracranial AVM. Pregnancy is not a risk factor in female patients with intracranial AVM with no history of hemorrhage. Small intracranial AVM are more likely to bleed. Since 1961 the majority of Taiwan's intracranial AVM patients have been treated surgically, while before that date general medicine was the treatment of choice. In recent years, several developments such as operation microscope, microsurgical instruments and microsurgical techniques have enhanced the efficacy of surgical intervention in the treatment of AVM. When the mortality and morbidity rates resulting from the two forms of treatment are compared, surgical treatment shows a better prognosis for the treatment of intracranial AVM.

  6. Vascular Malformations: A Review

    PubMed Central

    Cox, Joshua A.; Bartlett, Erica; Lee, Edward I.

    2014-01-01

    Identification and treatment of vascular malformations is a challenging endeavor for physicians, especially given the great concern and anxiety created for patients and their families. The goal of this article is to provide a review of vascular malformations, organized by subtype, including capillary, venous, lymphatic and arteriovenous malformations. Only by developing a clear understanding of the clinical aspects, diagnostic tools, imaging modalities, and options for intervention will appropriate care be provided and results maximized. PMID:25045330

  7. Pelvic Vascular Malformations

    PubMed Central

    Christenson, Brian M.; Gipson, Matthew G.; Smith, Mitchell T.

    2013-01-01

    Vascular malformations (VMs) comprise a wide spectrum of lesions that are classified by content and flow characteristics. These lesions, occurring in both focal and diffuse forms, can involve any organ and tissue plane and can cause significant morbidity in both children and adults. Since treatment strategy depends on the type of malformation, correct diagnosis and classification of a vascular lesion are crucial. Slow-flow VMs (venous and lymphatic malformations) are often treated by sclerotherapy, whereas fast-flow lesions (arteriovenous malformations) are generally managed with embolization. In addition, some cases of VMs are best treated surgically. This review will present an overview of VMs in the female pelvis as well as a discussion of endovascular therapeutic techniques. PMID:24436563

  8. Nevus vascularis mixtus (cutaneous vascular twin nevi) associated with intracranial vascular malformation of the Dyke-Davidoff-Masson type in two patients.

    PubMed

    Ruggieri, Martino; Milone, Pietro; Pavone, Piero; Falsaperla, Raffaele; Polizzi, Agata; Caltabiano, Rosario; Fichera, Marco; Gabriele, Anna Lia; Distefano, Angela; De Pasquale, Rocco; Salpietro, Vincenzo; Micali, Giuseppe; Pavone, Lorenzo

    2012-11-01

    The term twin spotting refers to phenotypes characterized by the spatial and temporal co-occurrence of two (or more) different nevi arranged in variable cutaneous patterns, and can be associated with extra-cutaneous anomalies. Several examples of twin spotting have been described in humans including nevus vascularis mixtus, cutis tricolor, lesions of overgrowth, and deficient growth in Proteus and Elattoproteus syndromes, epidermolytic hyperkeratosis of Brocq, and the so-called phacomatoses pigmentovascularis and pigmentokeratotica. We report on a 28-year-old man and a 15-year-old girl, who presented with a previously unrecognized association of paired cutaneous vascular nevi of the telangiectaticus and anemicus types (naevus vascularis mixtus) distributed in a mosaic pattern on the face (in both patients) and over the entire body (in the man) and a complex brain malformation (in both patients) consisting of cerebral hemiatrophy, hypoplasia of the cerebral vessels and homolateral hypertrophy of the skull and sinuses (known as Dyke-Davidoff-Masson malformation). Both patients had facial asymmetry and the young man had facial dysmorphism, seizures with EEG anomalies, hemiplegia, insulin-dependent diabetes mellitus (IDDM), autoimmune thyroiditis, a large hepatic cavernous vascular malformation, and left Legg-Calvé-Perthes disease (LCPD) [LCPD-like presentation]. Array-CGH analysis and mutation analysis of the RASA1 gene were normal in both patients.

  9. Congenital Vascular Malformation

    MedlinePlus

    ... reason these artery- to-vein connections, or a cluster of them persist. Such connections are called arteriovenous fistulas (AVFs), or if there is a cluster of them they are called arteriovenous malformations (AVMs). ...

  10. [Surgical treatment of vascular malformations].

    PubMed

    Fernández Alonso, L

    2004-01-01

    In spite of the numerous advances made over the last two decades, the treatment of congenital vascular malformations continues to be one of the greatest enigmas facing modern medicine. There are no clear criteria concerning the indications to be followed, and even less concerning the most appropriate therapeutic procedures for each type of lesion. The results of a strictly surgical approach are discouraging and today it is accepted that congenital vascular malformations should be attended to and treated by multidisciplinary units, combining the efforts of all the specialists involved in its treatment. This paper reviews the general principles of surgical treatment of congenital vascular malformations, without losing sight of the fact that the traditional role of isolated surgery in the treatment of congenital vascular malformations has been replaced by a multidisciplinary approach to this type of lesions, making it possible to integrate embolization, sclerotherapy and surgery to improve the results. The combination of these techniques reduces the risk and complications that existed when they were applied in an isolated form. Thus, surgical treatment should not be considered as an independent tool of treatment but as a therapeutic weapon integrated in the ensemble of measures directed at improving the quality of life of the patient with a congenital vascular malformation.

  11. [Sclerosing treatment of vascular malformations].

    PubMed

    Cabrera, J; Redondo, P

    2004-01-01

    Traditional sclerotherapy with liquid sclerosants has been used for many years in the treatment of venous, lymphatic and low flow vascular malformations; it is efficient only with those vascular malformations of reduced size as a pre or post-operational complement. The use of liquid sclerosants has the limitations of their dilution and progressive inactivation in a great haematic volume, the irregular distribution of the sclerosant on the endothelium, the handling of the sclerosant once injected and its imperceptibility to the echo-Doppler. In their turn, both ethanol and sodium morrhuate - the most habitually employed sclerosants - produce important secondary effects. On the contrary, the use of sclerosants, specifically polidocanol in microfoam form, significantly improves the procedure, since the microfoam displaces the blood instead of mixing and diluting itself in it, thus facilitating an homogeneous distribution of the sclerosant over the endothelial surface. Finally, the echogenicity of the microbubbles, which makes them directly visible, together with their manageable consistency, means that it can be distributed more easily throughout the treated area. We comment on our experience with 50 patients with venous or low flow vascular malformations, treated with this new form of sclerosant. Similarly, the use of OK-432 (picibanil) - as the recommended sclerosant treatment in lymphatic vascular, especially macrocystic, malformations - is reviewed and its protocol given.

  12. Thoracolumbar Arteriovenous Malformations Presenting with Intracranial Subarachnoid Hemorrhage: Case Series and Review of Literature.

    PubMed

    Cerejo, Russell; John, Seby; Grabowski, Matthew; Bauer, Andrew; Chaudhry, Burhan; Toth, Gabor; Hui, Ferdinand; Bain, Mark

    2016-04-01

    Cryptogenic intracranial subarachnoid hemorrhage accounts for approximately 15% of all subarachnoid hemorrhage cases. Diagnostic workup after negative cerebral digital subtraction angiogram typically includes magnetic resonance imaging of the brain and cervical spine for arteriovenous malformations, tumors, and fistulae. Only a few cases of thoracolumbar spinal vascular malformations have been associated with intracranial subarachnoid hemorrhage. Case series and review of the literature. We found 3 patients at our institution who had nontraumatic, nonaneurysmal intracranial subarachnoid hemorrhage with isolated spinal vascular malformation in the thoracolumbar region. Including our 3 cases, we found a total of 15 similar cases in the literature. Most of the patients were younger, most having concurrent spinal cord symptoms of radiculopathy (27%), myelopathy (20%), or bladder bowel involvement (20%). Most of the spinal vascular malformations were intramedullary or conus medullaris type. Locations of intracranial subarachnoid hemorrhage were mostly isolated to the perimesencephalic area and posterior fossa. In younger populations presenting with nonaneurysmal intracranial subarachnoid hemorrhage and symptoms related to the spinal cord, evaluation for thoracolumbar spinal vascular malformations must be included in the initial workup. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Intracranial aneurysms: links among inflammation, hemodynamics and vascular remodeling

    PubMed Central

    Hashimoto, Tomoki; Meng, Hui; Young, William L.

    2009-01-01

    Abnormal vascular remodeling mediated by inflammatory cells has been identified as a key pathologic component of various vascular diseases, including abdominal aortic aneurysms, brain arteriovenous malformations and atherosclerosis. Based on findings from observational studies that analysed human intracranial aneurysms and experimental studies that utilized animal models, an emerging concept suggests that a key component of the pathophysiology of intracranial aneurysms is sustained abnormal vascular remodeling coupled with inflammation. This concept may provide a new treatment strategy to utilize agents to inhibit inflammation or cytokines produced by inflammatory cells such as matrix metalloproteinases. Such an approach would aim to stabilize these vascular lesions and prevent future expansion or rupture. PMID:16759441

  14. CONGENITAL MACROVESSEL ASSOCIATED WITH CYSTOID MACULAR EDEMA AND AN IPSILATERAL INTRACRANIAL VENOUS MALFORMATION

    PubMed Central

    Sanfilippo, Christian J.

    2015-01-01

    Background/Purpose: To report a case of congenital retinal macrovessel associated with cystoid macular edema and an ipsilateral intracranial venous malformation. Methods: Case report. Results: A 58-year-old woman with decreased vision was found to have a congenital retinal venous macrovessel associated with cystoid macular edema because of tributary venous occlusion. The patient underwent neuroimaging and an ipsilateral venous malformation of the frontal lobe was discovered. Conclusion: Congenital retinal macrovessel can occasionally be complicated by vascular occlusion and macular edema. The authors report a case of congenital retinal macrovessel associated with an intracranial venous malformation. Clinicians should be aware of this potential association, and further studies are warranted. PMID:26421894

  15. Genetic causes of vascular malformations.

    PubMed

    Brouillard, Pascal; Vikkula, Miikka

    2007-10-15

    Vascular malformations are localized defects of vascular development. They usually affect a limited number of vessels in a restricted area of the body. Although most malformations are sporadic, inheritance is observed, enabling genetic analysis. Usually, sporadic forms present with a single lesion whereas multiple lesions are observed in familial cases. The last decade has seen unraveling of several causative genes and beginning of elucidation of the pathophysiological pathways involved in the inherited forms. In parallel, definition of the clinical phenotypes has improved and disorders such as Parkes-Weber syndrome (PKWS), first thought to be sporadic, is now known to be part of a more common inheritable phenotype. In addition, the concept of double-hit mechanism that we proposed earlier to explain the incomplete penetrance, variable expressivity and multifocality of lesions in inherited venous anomalies is now becoming confirmed, as some somatic mutations have been identified in venous, glomuvenous and cerebral cavernous malformations. It is thus tempting to suggest that familial forms of vascular malformations follow paradominant inheritance and that sporadic forms, the etiopathogenic causes of which are still unelucidated, are caused by somatic mutations in the same genes.

  16. [Intracranial arteriovenous malformations in pregnant women].

    PubMed

    Perquin, D A; Kloet, A; Tans, J T; Witte, G N; Dörr, P J

    1999-03-06

    Three women, aged 27, 32 and 30 years, respectively, suffered from headache, nausea and neurological abnormalities and were found to have an intracranial arteriovenous malformation (AVM). One of them after diagnosis had two pregnancies, both ended by caesarean section with good results. Another woman was 32 weeks pregnant when the AVM manifested itself with a haemorrhage; she recovered well and was delivered by caesarean section. After the AVM proved radiologically to have been obliterated, she delivered after her subsequent pregnancy by the vaginal route with vacuum extraction. The third woman was 15 weeks pregnant when major abnormalities developed. There was a large intracerebral haematoma with break-through to the ventricular system; this patient died. Intracranial haemorrhage during pregnancy is rate. It can result in maternal and foetal morbidity and mortality. It appears that pregnancy does not increase the rate of first cerebral haemorrhage from an AVM. The management of AVM rupture during pregnancy should be based primarily on neurosurgical rather than on obstetric considerations. Close collaboration with a team of neurologists, neurosurgeons, obstetricians and anaesthesiologists is mandatory.

  17. Cryptic vascular malformations involving the brainstem

    SciTech Connect

    Yeates, A.; Enzmann, D.

    1983-01-01

    Six patients with angiographically cryptic vascular malformations involving the brainstem were examined with computed tomography (CT). The clinical and CT findings of cryptic vascular malformations of the brainstem are described and distinguished from those of brainstem glioma and multiple sclerosis. Calcification within a brainstem lesion that displays relatively little mass effect and shows little contrast enhancement, particularly when associated with a long history of waxing and waning brainstem symptoms, should suggest a vascular malformation.

  18. MR imaging of cerebral vascular malformations.

    PubMed

    Lee, B C; Herzberg, L; Zimmerman, R D; Deck, M D

    1985-01-01

    Fifteen vascular malformations, including six supratentorial arteriovenous malformations (AVMs), three venous malformations, and six brainstem vascular malformations, were examined on 0.5 T magnetic resonance (MR) and GE 9800 and 8800 computed tomographic (CT) scanners. All the malformations were shown by MR, and the arterial and venous drainage of AVMs was precisely delineated. Hematoma was always differentiated from calcification by MR signal characteristics. Increased signal in the brain parenchyma was often seen adjacent to AVMs. The signal of blood within venous malformations altered with spin-echo techniques using various repetition times and was distinguished from rapidly flowing blood in AVMs that lacked signal in all imaging sequences. Brainstem malformations were seldom demonstrated by angiography. Hemorrhage was common and was invariably associated with multiple areas of absent signal that may have represented abnormal vessels. These appearances are distinct from those of intrinsic tumors and are probably pathognomonic of brainstem vascular malformations.

  19. Vascular Malformations: Approach by an Interventional Radiologist

    PubMed Central

    Pimpalwar, Sheena

    2014-01-01

    Children with vascular malformations are best managed with a multidisciplinary team of specialists. Interventional radiology may deliver primary treatment such as staged sclerotherapy and embolization for malformations that are poor candidates for primary surgical resection or play a supportive role such as preoperative or intraoperative embolization. A thorough understanding of vascular morphology and flow dynamics is imperative to choosing the best treatment tool and technique. In this review, the author discusses the selection of techniques and tools used to treat vascular malformations based on their angiographic morphology. PMID:25045335

  20. [Idiopathic intracranial hypertension in a patient with Chiari I malformation].

    PubMed

    Santos-Bueso, E; Porta-Etessam, J; Díaz-Valle, D; Benítez-del-Castillo, J M; Gegúndez-Fernández, J A; Vinuesa-Silva, J M; García-Sánchez, J

    2015-04-01

    The case involves a 22-year-old woman who presented with headache and decreased vision. She showed asymmetric papilledema, and a 6-mm tonsillar descent was observed in the image tests. She was diagnosed with secondary intracranial hypertension coinciding with the symptoms of a Chiari malformation (MC). Chiari malformation type I is the most common in this group of malformations, and is characterized by a greater than 5mm descent of the tonsils, being able to cause increased intracranial pressure and papilledema by blocking the flow of the cerebrospinal fluid. In this case, the MC was not the responsible for triggering the secondary intracranial hypertension, but a mere coincidence of both processes. Copyright © 2013 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.

  1. Clinical-radiological evaluation of sequelae of stereotactic radiosurgery for intracranial arteriovenous malformations

    SciTech Connect

    Levy, R.P.; Fabrikant, J.I.; Frankel, K.A.; Phillips, M.H.; Steinberg, G.K.; Marks, M.P.; DeLaPaz, R.L.; Chuang, F.Y.S.

    1989-12-01

    Stereotactic heavy-charged-particle Bragg peak radiosurgery has been used to treat 322 patients with surgically-inaccessible intracranial vascular malformations. (The clinical results of this method for the treatment of angiographically demonstrable arteriovenous malformations (AVMs) and angiographically occult vascular malformations (AOVMs) of the brain are described in separate reports of this symposium). The great majority of patients have had an uneventful post-treatment course with satisfactory health outcomes. However, several categories of delayed sequelae of stereotactic radiosurgery have been identified, involving the vascular structures essential for the integrity of the brain tissue and the brain parenchyma directly. These categories reflect both reaction to injury and to alterations in regional hemodynamic status, and include vasogenic edema, occlusion of functional vasculature, radiation necrosis, and local or remote effects on cerebral arterial aneurysms. 10 refs., 7 figs., 1 tab.

  2. Stereotactic helium-ion radiosurgery for the treatment of intracranial arteriovenous malformations

    SciTech Connect

    Fabrikant, J.I.; Levy, R.P.; Frankel, K.A.; Phillips, M.H.; Lyman, J.T.; Chuang, F.Y.S.; Steinberg, G.K.; Marks, M.P.

    1989-12-01

    One of the more challenging problems of vascular neurosurgery is the management of surgically-inaccessible arteriovenous malformations (AVMs) of the brain. At Lawrence Berkeley Laboratory, we have developed the method of stereotactic heavy-charged-particle (helium-ion) Bragg peak radiosurgery for treatment of inoperable intracranial AVMs in over 300 patients since 1980 (Fabrikant et al. 1989, Fabrikant et al. 1985, Levy et al. 1989). This report describes patient selection, treatment method, clinical and neuroradiologic results and complications encountered. 4 refs.

  3. Retroperitoneal vascular malformation mimicking incarcerated inguinal hernia.

    PubMed

    Dubey, Indu Bhushan; Sharma, Anuj; Singh, Ajay Kumar; Mohanty, Debajyoti

    2011-01-01

    A 30-year-old man presented to the Department of Surgery with a painful groin swelling on right side. Exploration revealed a reddish-blue hemangiomatous mass in the scrotum extending through inguinal canal into the retroperitoneum. On further dissection swelling was found to be originating from right external iliac vein. The swelling was excised after ligating all vascular connections. The histopathological examination of excised mass confirmed the diagnosis of venous variety of vascular malformation. This is the first reported case of vascular malformation arising from retroperitoneum and extending into inguinoscrotal region, presenting as incarcerated inguinal hernia.

  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. Total knee arthroplasty in vascular malformation

    PubMed Central

    Bhende, Harish; Laud, Nanadkishore; Deore, Sandeep; Shashidhar, V

    2015-01-01

    In Klippel–Trenaunay syndrome, vascular malformations are not only in skin and superficial soft tissues but also in deep tissues like muscles bones and joints. It is well documemted that these recurrent intraarticular bleeds can cause early arthritis and joint pain. Performing arthroplasty in such patients is difficult and fraught with complications. We describe such a case where navigated total knee arthroplasty was performed with success to avoid the problems of intra medullary alignment used in the presence of intra medullary vascular malformations. We also suggest certain measures when knee arthroplasty is considered in such patients. PMID:26538765

  6. Birt-Hogg-Dubé syndrome and intracranial vascular pathologies.

    PubMed

    Kapoor, Rahul; Evins, Alexander I; Steitieh, Diala; Bernardo, Antonio; Stieg, Philip E

    2015-12-01

    Birt-Hogg-Dubé syndrome, first described in 1977, is a rare autosomal dominant condition that commonly presents with skin lesions, including fibrofolliculomas and trichodiscomas; pulmonary cysts; spontaneous pneumothoraces; and renal cancer. We present the only known cases of intracranial vascular pathologies in patients with Birt-Hogg-Dubé syndrome. We present three cases (three female; age range 18-50) of intracranial vascular lesions in Birt-Hogg-Dubé patients, including two aneurysms and one arteriovenous malformation, and review one previously reported case of carotid aplasia. Due to the rarity of Birt-Hogg-Dubé syndrome and significant variations in its clinical presentation, it is difficult to assess whether or not Birt-Hogg-Dubé patients are predisposed to intracranial vascular pathologies. We hypothesize that increased transcription of hypoxia-inducible factor 1-alpha, resulting from a mutated form of the protein folliculin transcribed by the Birt-Hogg-Dubé gene, may be associated with vascular pathogenesis in Birt-Hogg-Dubé patients and thus provide a possible molecular basis for a link between these two conditions.

  7. Oral vascular malformations: laser treatment and management

    NASA Astrophysics Data System (ADS)

    Romeo, U.; Rocchetti, F.; Gaimari, G.; Tenore, G.; Palaia, G.; Lo Giudice, G.

    2016-03-01

    Vascular malformations are a very heterogeneous group of circulatory system's diseases that can involve different kind of vessels: arterial, venous or lymphatic ones. Many treatments, such as conventional surgery, embolization, steroid therapy and laser therapy, are available for vascular lesions. The laser approach relies more therapeutic techniques: the transmucosal thermophotocoagulation, intralesional photocoagulation, the excisional biopsy. Today laser is demonstrated to be the gold standard technique to treat vascular lesions that allows a safe and efficient treatment and a lower post-operative healing time. The only disadvantage is the risk of carbonization that could be avoided by using the multiple-spot single pulsed wave technique.

  8. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    SciTech Connect

    Cornelis, F.; Neuville, A.; Labreze, C.; Kind, M.; Bui, B.; Midy, D.; Palussiere, J.; Grenier, N.

    2013-06-15

    The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

  9. Hemangiomas and Vascular Malformations: Current Theory and Management

    PubMed Central

    Richter, Gresham T.; Friedman, Adva B.

    2012-01-01

    Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumor. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. This paper reviews current theory and practice in the etiology, diagnosis, and treatment of these more common vascular anomalies. PMID:22611412

  10. Systemic vascular responses to increased intracranial pressure

    PubMed Central

    Fitch, William; McDowall, D. Gordon

    1977-01-01

    This paper details the results of experimental studies, on 16 dogs with artificially-induced intracranial space-occupying lesions, of the systemic vascular responses and the intracranial pressure changes (both in the supratentorial and infratentorial compartments) induced by increasing intracranial pressure. The changes produced were divided into two phases such that phase 1 detailed the alterations observed from the start of the balloon inflation up to the initiation of the systemic pressor response. Phase 2 recorded those alterations which occurred during, and immediately after, the period of systemic hypertension (see Fitch et al., 1977). The changes observed during phase 1, and presented in this communication, were those of increasing intracranial pressures and decreasing mean arterial pressure and heart rate. These alterations were associated with decreases in supratentorial perfusion pressure and increases in transtentorial pressure gradient and arrhythmia index. PMID:599360

  11. Solitary vascular malformation of the clitoris.

    PubMed

    Haritharan, T; Islah, M; Zulfiqar, A; Thambi Dorai, C R

    2006-06-01

    Isolated involvement of the clitoris by vascular malformation (VM) is very rare. Clinically, the lesion simulates female pseudohermaphroditism. A five-year-old girl presented with clitoromegaly and a clinical diagnosis of solitary VM of the clitoris was made. Magnetic resonance imaging showed characteristic features and confirmed the diagnosis and the extent of the VM. This is the first reported case of isolated involvement of the clitoris by VM to be diagnosed preoperatively.

  12. Severe Epistaxis from an Intracranial Vascular Bleed from Grenade Injury

    DTIC Science & Technology

    2008-09-01

    Severe Epistaxis from an Intracranial Vascular Bleed from Grenade Injury Radiology Corner Case 27 Severe Epistaxis from an Intracranial ...neck injuries. In particular, this case focuses on an intracranial vascular injury generated by a hand grenade with the diagnosis assisted by...4. TITLE AND SUBTITLE Severe Epistaxis from an Intracranial Vascular Bleed from Grenade Injury 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM

  13. BIOLOGY OF VASCULAR MALFORMATIONS OF THE BRAIN

    PubMed Central

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

    2009-01-01

    Background and Purpose 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 (CCM), sporadic brain arteriovenous malformation (AVM), and the AVMs of hereditary hemorrhagic telangiectasia (HHT). Summary of Review The identification of gene mutations and genetic risk factors associated with CCM, HHT, and sporadic AVM 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 CCM lesion formation involves a genetic two-hit mechanism, in which a germline mutation in one copy of a CCM 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. Conclusions 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 progress will be facilitated by collaborative expansion and standardization of these. PMID:19834013

  14. Extracranial vascular malformations (hemangiomas and vascular malformations) in children and adolescents – diagnosis, clinic, and therapy

    PubMed Central

    Eivazi, Behfar; Werner, Jochen A.

    2014-01-01

    The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be

  15. Oral encapsulated vascular malformation: An undescribed presentation in the mouth

    PubMed Central

    Dias, Márcio-Américo; Dias, Pedro-de Souza; Martínez-Martínez, Marisol; Sena-Filho, Marcondes; de Almeida, Oslei-Paes

    2016-01-01

    Vascular lesions have been classified in two broad categories, hemangiomas and malformations. Encapsulated vascular lesions have not been reported in the oral cavity, but they were described in other sites, mainly in the orbit. Herein, we present a case of an oral encapsulated vascular lesion located in the right buccal mucosa of a 69-year-old male, including histological and immunohistochemical description and a literature review. Key words:Buccal mucosa, hemangioma, vascular malformation, oral cavity. PMID:26855712

  16. Amplatzer vascular plugs in congenital cardiovascular malformations

    PubMed Central

    Barwad, Parag; Ramakrishnan, Sivasubramanian; Kothari, Shyam S; Saxena, Anita; Gupta, Saurabh K; Juneja, Rajnish; Gulati, Gurpreet Singh; Jagia, Priya; Sharma, Sanjiv

    2013-01-01

    Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 – 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow. PMID:24688229

  17. Laser treatment of oral vascular malformations

    NASA Astrophysics Data System (ADS)

    Romeo, U.; Gaimari, G.; Mohsen, M.; Tenore, G.; Palaia, G.

    2014-01-01

    Oral Vascular Malformations (OVM) are congenital anomalies characterized by morph-structural and/or functional changes of nature in severity and extension. OVM can affect any type of vessels arterial, venous or lymphatic and any capillary or anatomical. They are divided into two categories: low and high flow. In this study were treated 40 patients with OVM with a range size from 2 mm to 44 mm; they were subjected to clinical examination supported by Colour-Doppler Ultrasound instrumental examination and only for doubt cases the Magnetic Resonance Imaging (MRI) was prescribed. Only low flow venous and capillary malformations were treated by GaAlAs laser (Wiser®, Lambda, Brindole,Italy, 980nm) and KTP laser (SmartLite®, DEKA, Florence, Italy, 532nm) with two different techniques: the Transmucosal Thermophotocoagulation (TMT) and the Intralesional Photocoagulation (ILP). These techniques permitted a good control of haemostasis, avoiding bleeding both during surgery and in the postoperative. It is obtained an excellent and good healing respectively in 10% and 60% of cases, a moderate and poor resolution respectively in 22.5% and 7.5% of cases. A clear diagnosis allowed the management of Venous malformations (VM) by laser devices with wavelengths highly absorbed in haemoglobin in safety and efficacy and according to the principles of minimal invasive surgery. The aim of this study was to verify if the laser is effective in the treatment of OVM for the purpose of the clinical findings and the postoperative course. The Authors concluded that the laser can be considered the "gold standard" for treating OVM.

  18. Vascular permeability in cerebral cavernous malformations

    PubMed Central

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao; Girard, Romuald; Shenkar, Robert; Guo, Xiaodong; Shah, Akash; Larsson, Henrik BW; Tan, Huan; Li, Luying; Wishnoff, Matthew S; Shi, Changbin; Christoforidis, Gregory A; Awad, Issam A

    2015-01-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive familial CCM disease had greater WMF permeability compared to those with milder disease phenotype, but similar lesion permeability. Subjects receiving statin medications for routine cardiovascular indications had a trend of lower WMF, but not lesion, permeability. This is the first demonstration of brain vascular hyperpermeability in humans with an autosomal dominant disease, as predicted mechanistically. Brain permeability, more than lesion permeability, may serve as a biomarker of CCM disease activity, and help calibrate potential drug therapy. PMID:25966944

  19. Vascular permeability in cerebral cavernous malformations.

    PubMed

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao; Girard, Romuald; Shenkar, Robert; Guo, Xiaodong; Shah, Akash; Larsson, Henrik B W; Tan, Huan; Li, Luying; Wishnoff, Matthew S; Shi, Changbin; Christoforidis, Gregory A; Awad, Issam A

    2015-10-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive familial CCM disease had greater WMF permeability compared to those with milder disease phenotype, but similar lesion permeability. Subjects receiving statin medications for routine cardiovascular indications had a trend of lower WMF, but not lesion, permeability. This is the first demonstration of brain vascular hyperpermeability in humans with an autosomal dominant disease, as predicted mechanistically. Brain permeability, more than lesion permeability, may serve as a biomarker of CCM disease activity, and help calibrate potential drug therapy.

  20. Intralesional laser therapy for vascular malformations.

    PubMed

    Ma, Linda W; Levi, Benjamin; Oppenheimer, Adam J; Kasten, Steven J

    2014-11-01

    Intralesional laser therapy for the treatment of vascular malformations (VMs) has been previously reported for select patient populations. Larger studies, over a wider variety of indications, are needed to better define the potential role of this technology. In the current study, a 12-year, retrospective review of 44 patients who underwent 73 intralesional Nd:YAG or diode laser treatments of VMs was performed. The most commonly encountered lesions were venous malformations (66%) and the most commonly involved anatomic locations were the head and neck regions (41%) and lower extremity (39%). Primary indications for treatment were enlargement (73%) and pain (52%). Lesion size was reduced in 94% of cases after treatment and pain was improved in 91% of cases. Minor postoperative complications occurred in 16 (36%) patients. There was no difference in treatment response among various VM subtypes or anatomic locations (P=0.497, P=0.866) or in the incidence of complications (P=0.531, P=0.348). Age was the only factor associated with an increased risk of complications (odds ratio, 1.034; P=0.038). When used in accordance with the suggested guidelines, intralesional laser therapy is a safe and effective treatment modality for VMs of varying compositions and locations.

  1. 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

  2. Emerging Techniques for Evaluation of the Hemodynamics of Intracranial Vascular Pathology

    PubMed Central

    Huang, Melissa; Chien, Aichi

    2015-01-01

    Advances in imaging modalities have improved the assessment of intracranial hemodynamics using non-invasive techniques. This review examines new imaging modalities and clinical applications of currently available techniques, describes pathophysiology and future directions in hemodynamic analysis of intracranial stenoses, aneurysms and arteriovenous malformations and explores how hemodynamic analysis may have prognostic value in predicting clinical outcomes and assist in risk stratification. The advent of new technologies such as pseudo-continuous arterial spin labeling, accelerated magnetic resonance angiography (MRA) techniques, 4D digital subtraction angiography, and improvements in clinically available techniques such as phase-contrast MRA may change the landscape of vascular imaging and modify current clinical practice guidelines. PMID:25924168

  3. Klippel-Trénaunay Syndrome with Intracranial Arteriovenous Malformation: A Rare Presentation

    PubMed Central

    Sadiq, Mahniya F.; Tiwana, Muhammad H.; Johnson, Jamlik-Omari; Khosa, Faisal

    2014-01-01

    Klippel-Trénaunay syndrome (KTS) is a rare vascular congenital anomaly affecting less than 200,000 people in the United States. Vascular malformations associated with KTS tend to affect slow flow systems: venous, capillary, and lymphatic systems. The nature of the syndrome leads to a higher risk for the development of arteriovenous malformations. Our case presentation describes a patient with KTS and an associated rare presentation of intraventricular arteriovenous malformation (AVM). PMID:24653849

  4. [Application of cyanoacrylate glue and ethylene vinyl alcohol copolymer for the treatment of vascular malformations of the central nervous system].

    PubMed

    Guziński, Maciej; Kurcz, Jacek; Bereza, Sławomir; Garcarek, Jerzy; Sasiadek, Marek

    2010-01-01

    Arterio-venous malformations (AVMs) and dural arterio-venous fistulas (AVFs) are relatively rare developmental vascular system disorders and constitute the majority of vascular malformations of the nervous system. The malformations are characterized by a large variety of vascular architecture. Intracranial or intramedullary hemorrhage constitute the most serious complications of the malformations. The algorithm of management aiming at complete recovery or hemorrhage prevention has not been completely established yet due to considerable individual variability of malformations. The obliteration of malformations pathological vessels using cyanoacrylate glue or ethylene vinyl alcohol copolymer (EVOH)--so called endovascular embolization--has been the most dynamically developing treatment method recently. The procedure, unlike classical surgical resection of pathological vessels, is significantly less invasive and associated with shorter hospitalization period. The features and properties of embolization agents (cyanoacrylate and EVOH) as well as application of the agents for treatment of the vascular pathologies of the central nervous system are discussed in the paper. The procedure of endovascular introduction of embolization agents into the lumen of malformation vessels is also presented in the article. The analyzed literature and own experiences allow to claim that the application of cyanoacrylate and EVOH is a relatively safe therapeutic method that in part of the cases enables complete embolization of pathological vessels. The development of endovascular systems and the advances in embolization agents should trigger further intensive improvement of the technique.

  5. Vascular malformations: an update on imaging and management.

    PubMed

    Sierre, Sergio; Teplisky, Darío; Lipsich, José

    2016-04-01

    Vascular malformations comprise a broad and heterogeneous range of lesions that often represent a diagnostic and therapeutic challenge for the pediatrician. For a long time, the use of an inaccurate nomenclature has led to confusion. Since management depends on the specific vascular malformation, a proper classification and identification is critical. The objective of this article is to provide the necessary information about the current classification and terminology of vascular anomalies, including basic concepts about available imaging diagnostic and therapeutic tools for the management of such complex condition.

  6. Congenital vascular malformations: a series of five prenatally diagnosed cases.

    PubMed

    Connell, Fiona; Homfray, Tessa; Thilaganathan, Baskaran; Bhide, Amarnath; Jeffrey, Iona; Hutt, Renata; Mortimer, Peter; Mansour, Sahar

    2008-10-15

    In the literature there are single case reports of mediastinal/chest and limb combined vascular malformations (previously labeled "hemangiolymphangiomas"). A variable outcome in such prenatally diagnosed cases is reported. Presented here is the only series of patients reported with these macrocystic, predominantly lymphatic malformations. Prenatal ultrasound scan and post-mortem examination findings are described. In our experience the outcome has been poor and this highlights the dilemma faced by clinicians and parents when these lesions are diagnosed prenatally. We present a series of five, prenatally diagnosed vascular (combined vascular malformations and simple localized lymphatic malformations) malformations. Three cases had lower leg involvement with extension into the abdomen and two cases had lymphatic malformations of the chest wall with involvement of the upper limb(s). Management of a twin pregnancy, in which one twin was affected, is described. In two cases, termination of pregnancy was undertaken because of the extensive nature of the lesion. One case died in utero and one in the neonatal period. The fifth case is an 11-year-old boy, whose lower limb deformity illustrates the considerable morbidity associated with this condition. 2008 Wiley-Liss, Inc.

  7. Assessing Intracranial Vascular Compliance Using Dynamic Arterial Spin Labeling

    PubMed Central

    Yan, Lirong; Liu, Collin Y.; Smith, Robert X.; Jog, Mayank; Langham, Michael; Krasileva, Kate; Chen, Yufen; Ringman, John M.; Wang, Danny J.J.

    2015-01-01

    Vascular compliance (VC) is an important marker for a number of cardiovascular diseases and dementia, which is typically assessed in central and peripheral arteries indirectly by quantifying pulse wave velocity (PWV), and/or pulse pressure waveform. To date, very few methods are available for the quantification of intracranial VC. In the present study, a novel MRI technique for in-vivo assessment of intracranial VC was introduced, where dynamic arterial spin labeling (ASL) scans were synchronized with the systolic and diastolic phases of the cardiac cycle. VC is defined as the ratio of change in arterial cerebral blood volume (ΔCBV) and change in arterial pressure (ΔBP). Intracranial VC was assessed in different vascular components using the proposed dynamic ASL method. Our results show that VC mainly occurs in large arteries, gradually decreases in small arteries and arterioles. The comparison of intracranial VC between young and elderly subjects shows that aging is accompanied by a reduction of intracranial VC, in good agreement with the literature. Furthermore, a positive association between intracranial VC and cerebral perfusion measured using pseudo-continuous ASL with 3D GRASE MRI was observed independent of aging effects, suggesting loss of VC is associated with a decline in perfusion. Finally, a significant positive correlation between intracranial and central (aortic arch) VC was observed using an ungated phase-contrast 1D projection PWV technique. The proposed dynamic ASL method offers a promising approach for assessing intracranial VC in a range of cardiovascular diseases and dementia. PMID:26364865

  8. Endovascular embolization of life threatening intracranial arterio-venous malformation.

    PubMed

    Khan, S U; Rahman, K M; Siddiqui, M R; Hoque, M A; Mondol, B A; Hussain, S; Mohammad, Q D

    2010-07-01

    Haemorrhagic stroke from cerebral arteriovenous malformations (AVMs) represents 2% of all hemorrhagic strokes. A clear understanding of the diagnostic and treatment algorithms of cerebral AVM management is very important, because AVMs are a cause of hemorrhage in young adults. Surgery, endovascular therapy, and radiosurgery can be used alone or in combination to treat an AVM. We reported a 40 years old man of cerebral arteriovenous malformation (AVM), complicated with intracerebral hemorrhage (ICH). Digital subtraction angiogram was done for diagnosis and endovascular embolization for treatment of the case. This is the first successful cerebral arteriovenous malformations (AVMs) embolization in any government hospital of Bangladesh. The aim of this case report is to inform about this new technologies and emerging treatment strategies in these areas.

  9. Awake Surgery for Brain Vascular Malformations and Moyamoya Disease.

    PubMed

    Aoun, Rami James N; Sattur, Mithun G; Krishna, Chandan; Gupta, Amen; Welz, Matthew E; Nanney, Allan D; Koht, Antoun H; Tate, Matthew C; Noe, Katherine H; Sirven, Joseph I; Anderies, Barrett J; Bolton, Patrick B; Trentman, Terry L; Zimmerman, Richard S; Swanson, Kristin R; Bendok, Bernard R

    2017-09-01

    Although a significant amount of experience has accumulated for awake procedures for brain tumor, epilepsy, and carotid surgery, its utility for intracranial neurovascular indications remains largely undefined. Awake surgery for select neurovascular cases offers the advantage of precise brain mapping and robust neurologic monitoring during surgery for lesions in eloquent areas, avoidance of potential hemodynamic instability, and possible faster recovery. It also opens the window for perilesional epileptogenic tissue resection with potentially less risk for iatrogenic injury. Institutional review board approval was obtained for a retrospective review of awake surgeries for intracranial neurovascular indications over the past 36 months from a prospectively maintained quality database. We reviewed patients' clinical indications, clinical and imaging parameters, and postoperative outcomes. Eight consecutive patients underwent 9 intracranial neurovascular awake procedures conducted by the senior author. A standardized "sedated-awake-sedated" protocol was used in all 8 patients. For the 2 patients with arteriovenous malformations and the 3 patients with cavernoma, awake brain surface and white matter mapping was performed before and during microsurgical resection. A neurological examination was obtained periodically throughout all 5 procedures. There were no intraoperative or perioperative complications. Hypotension was avoided during the 2 Moyamoya revascularization procedures in the patient with a history of labile blood pressure. Postoperative imaging confirmed complete arteriovenous malformation and cavernoma resections. No new neurologic deficits or new-onset seizures were noted on 3-month follow-up. Awake surgery appears to be safe for select patients with intracranial neurovascular pathologies. Potential advantages include greater safety, shorter length of stay, and reduced cost. Copyright © 2017. Published by Elsevier Inc.

  10. 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.

  11. The pathobiology of vascular malformations: insights from human and model organism genetics.

    PubMed

    Wetzel-Strong, Sarah E; Detter, Matthew R; Marchuk, Douglas A

    2017-01-01

    Vascular malformations may arise in any of the vascular beds present in the human body. These lesions vary in location, type, and clinical severity of the phenotype. In recent years, the genetic basis of several vascular malformations has been elucidated. This review will consider how the identification of the genetic factors contributing to different vascular malformations, with subsequent functional studies in animal models, has provided a better understanding of these factors that maintain vascular integrity in vascular beds, as well as their role in the pathogenesis of vascular malformations. Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  12. Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis.

    PubMed

    Ellis, Michael J; Armstrong, Derek; Dirks, Peter B

    2011-01-01

    The management of large and giant arteriovenous malformations (AVMs) in patients presenting with nonhemorrhagic neurological deficits secondary to vascular steal phenomenon is challenging and controversial. In many cases, large AVMs cannot be completely excised or cured, leaving patients with residual or partially treated AVMs, the natural history of which is unknown. Additionally, large, diffuse vascular malformations with multiple, small feeders, slow flow, or so-called cerebral proliferative angiopathy represent a related but distinct clinical and angiographic entity that may require a different therapeutic approach than traditional brain AVMs. The current management of children with other conditions of chronic cerebral hypoperfusion, such as moyamoya disease, involves consideration of surgical revascularization to enhance blood flow to the compromised hemisphere. Here, the authors present the case of a young child with a large thalamic vascular malformation who presented with clinical and radiological features of vascular steal and ischemia. In an effort to augment flow to the hypoperfused brain and protect against future ischemia, the authors treated the child with unilateral pial synangiosis. At 12 months, postoperative angiography demonstrated robust neovascularization, and the child has not sustained any further ischemic events. The authors discuss concept of vascular malformation-related hypoperfusion and the utility of indirect revascularization for inoperable vascular malformations presenting with ischemic symptoms.

  13. Alcohol sclerotherapy to treat vascular malformations in the oral cavity.

    PubMed

    Talens Ferrando, A; Ferrer Mengual, S; González-Cruz Soler, A; Martínez Sanjuán, V; Poveda Roda, R; Sanchis Bielsa, J M; Bagán Sebastián, J V

    2013-01-01

    To present our experience in treating vascular malformations in the oral cavity solely by injecting ethanol into the lesions. We treated 26 patients (12 men and 14 women) with oral malformations. The diagnosis was based on clinical findings (n=26), magnetic resonance imaging studies (n=19), angiography findings (n=5), and direct puncture venography (n=2). To achieve sclerosis, we administered absolute ethanol through direct puncture. All interventions were performed under deep sedation. The vascular malformations treated ranged from 7mm to 60mm (median: 24.5mm) in maximum diameter and had been present in the oral cavity for 0.2 to 54 years (mean: 13.6 years). The median age of the patients was 44.5 years (range: 12-87 years). The reason for treatment of the malformation was: an increase in size (n=8), local bleeding (n=11), risk of bleeding during dental extraction (n=5), pain (n=1), and esthetic purposes (n=3). Lesions were located in the mucosa of the cheek (n=12), in the facial gingiva (n=5), in the labial mucosa (n=6), in the tongue (n=3), in the pterygomandibular region (n=1), and in the palate (n=1). The median dose of ethanol was 3.2mL. Twenty lesions disappeared after a single injection session, five after two sessions, two after three sessions, and one after five sessions. In 20 cases all signs of the lesions disappeared, in 6 a bluish macule persisted, and in 2 a mass effect persisted. The symptoms improved in all patients. Only transient complications of sclerotherapy were observed: local inflammation, perioral paresthesia in two patients, and necrosis of the mucosa of the cheek in one. Alcohol sclerotherapy is an efficacious procedure for treating vascular malformations in the oral cavity. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  14. [A multidisciplinary approach to the management of cerebral vascular malformations].

    PubMed

    Estupiñán, B; López, G; Morales, L; Ochoa, L; García, I; Guerra, E; Zaldivar, M

    Cerebral vascular malformations (CVM) are a heterogeneous group of lesions. One way of classifying them is according to histological criteria, clinical features, imaging findings, electroencephalography and distinctive pathology. To report the results obtained in 16 patients clinically diagnosed as having CVM and operated on in the Centro Internacional de Restauración Neurológica (La Habana, Cuba) between March 1995 and October 1998. We also consider the usefulness of diagnostic tools for neurosurgical management and anatomo-pathological diagnosis. We review the clinical findings, images, electroencephalograms and diagnostic histology of 16 patients. The gender distribution was 10 men and 6 women who were aged between 9 and 48 years. Stereotaxic resection guided by CAT and angiography was done in all cases. The predominant symptoms were headache and generalized tonic-clonic convulsions. CAT and angiography were helpful in determining the clinical diagnosis and location. The most frequent electroencephalographic finding was focal slowing associated with inactive epileptiform disorders. Histological study showed that there were 3 cases of arteriovenous malformations (AVM), one angioma cavernosa and one mixed vascular malformation (AVM plus angioma cavernosa). The malformation was not resected (it was treated with a clip) in the remaining case. Our results show the importance of structural imaging studies and their relationship to functional studies in the presumptive diagnosis of CVM corroborated by post-operative histological diagnosis.

  15. Concurrent intracranial and spinal arteriovenous malformations: Report of two pediatric cases and literature review

    PubMed Central

    Shallwani, Hussain; Tahir, Muhammad Z.; Bari, Muhammad E.; Tanveer-ul-Haq

    2012-01-01

    Background: Concurrent intracranial and spinal arteriovenous malformations (AVMs) are very rare with only a few cases being reported in literature. Two of the rare concurrent intracranial and spinal AVM cases are presented. Case Description: Case 1 is a 12-year-old girl with headache and motor disturbances in the lower limbs. Her spinal and brain angiogram was done and she was diagnosed to have a spinal AVM at level T8–T9 and an intracranial AVM in the left mesial temporal lobe. Her spinal AVM was embolized, while no treatment was given for her intracranial AVM. Case 2 is a 10-year-old girl who presented with headache and quadriparesis. Her brain and spinal angiogram revealed an intracranial AVM in the left parietal lobe and a spinal AVM at level C2, respectively. Craniotomy and excision was done for her intracranial AVM and embolization for the spinal AVM. Conclusion: It is proposed that multiple AVMs may be a result of yet unrevealed pathogenesis or strong embryogenetic anomaly, which may be different from that involved in single AVM. With lack of consensus over the best therapeutic strategy, multimodality treatment based on the individual's needs is suggested. PMID:22629488

  16. Diagnosis and management of extensive vascular malformations of the lower limb: part I. Clinical diagnosis.

    PubMed

    Redondo, Pedro; Aguado, Leyre; Martínez-Cuesta, Antonio

    2011-11-01

    There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients.

  17. Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism

    PubMed Central

    Pang, Chang Hwan; Kim, Chi Heon; Chung, Chun Kee

    2015-01-01

    The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery. PMID:26512271

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

    PubMed Central

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

    2017-01-01

    Introduction 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. Methods and analysis 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. Ethics and dissemination 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. Trial registration

  19. Vascular malformations and arteriovenous fistulas of the kidney.

    PubMed

    Cura, Marco; Elmerhi, Fadi; Suri, Rajeev; Bugnone, Alejandro; Dalsaso, Timothy

    2010-03-01

    Vascular malformations of the kidney are disease processes that involve renal veins and arteries and include congenital arteriovenous malformations (AVMs) and arteriovenous fistulas. AVMs are congenital communications between arteries and veins with a vascular nidus that bypass the capillary bed. Congenital AVMs are rare and subclassified in cirsoid, angiomatous, and aneurysmal types. Congenital AVMs are different from iatrogenic or traumatic arteriovenous fistulas (AVF), which are characterized by a single direct communication between an artery and a vein without an intervening vascular nidus. These lesions may present with a wide range of signs and symptoms that vary from hypertension to renal masses. Imaging is valuable in the detection and characterization of AVM and AVF. The presence of arteriovenous shunting characterizes AVM and AVF. These lesions represent an important group of entities for diagnostic consideration, and understanding the vascular anatomy helps in guiding for proper treatment. This article describes the imaging features of each lesion that help to differentiate it from the others and the endovascular therapies to treat these vascular processes and their possible complications.

  20. Sleep-disordered breathing in pediatric head and neck vascular malformations.

    PubMed

    Durr, Megan L; Meyer, Anna K; Kezirian, Eric J; Mamlouk, Mark D; Frieden, Ilona J; Rosbe, Kristina W

    2017-09-01

    To determine the prevalence of sleep-disordered breathing (SDB) symptoms among children with head and neck vascular malformations and to compare obstructive sleep apnea (OSA)-18 scores between children with head and neck vascular malformations and children with non-head and neck vascular malformations. Retrospective cohort and prospective cross-sectional studies METHODS: Forty-three pediatric subjects with head and neck vascular malformations evaluated at a tertiary-care multidisciplinary vascular anomalies center were included in a retrospective cohort study. Eighty-three consecutive pediatric subjects with vascular malformations evaluated at the same center were included in the prospective cross-sectional study. In the retrospective cohort study, 20 (47%) subjects with head and neck malformations had documented SDB symptoms. Of those with SDB symptoms, five (25%) required long-term tracheotomy. The children with SDB symptoms had greater vascular malformation size, more extensive pharyngeal involvement, greater vascular malformation mass effect on airway, and closer proximity of malformation to airway when compared to children without SDB symptoms. For the prospective cross-sectional study, 23% of pediatric subjects had malformations of the head and neck. Those with head and neck malformations had a higher OSA-18 score and a lower overall quality of life (QOL) score when compared to subjects with non-head and neck malformations. Nearly half of children with head and neck vascular malformations have SDB symptoms. Children with head and neck vascular malformations have a higher OSA-18 score and lower overall QOL score when compared to children with non-head and neck vascular malformations. 4. Laryngoscope, 127:2159-2164, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  1. Vascular structure and binomial statistics for response modeling in radiosurgery of cerebral arteriovenous malformations

    NASA Astrophysics Data System (ADS)

    Andisheh, Bahram; Bitaraf, Mohammad A.; Mavroidis, Panayiotis; Brahme, Anders; Lind, Bengt K.

    2010-04-01

    Radiation treatment of arteriovenous malformations (AVMs) has a slow and progressive vaso-occlusive effect. Some studies suggested the possible role of vascular structure in this process. A detailed biomathematical model has been used, where the morphological, biophysical and hemodynamic characteristics of intracranial AVM vessels are faithfully reproduced. The effect of radiation on plexiform and fistulous AVM nidus vessels was simulated using this theoretical model. The similarities between vascular and electrical networks were used to construct this biomathematical AVM model and provide an accurate rendering of transnidal and intranidal hemodynamics. The response of different vessels to radiation and their obliteration probability as a function of different angiostructures were simulated and total obliteration was defined as the probability of obliteration of all possible vascular pathways. The dose response of the whole AVM is observed to depend on the vascular structure of the intra-nidus AVM. Furthermore, a plexiform AVM appears to be more prone to obliteration compared with an AVM of the same size but having more arteriovenous fistulas. Finally, a binomial model was introduced, which considers the number of crucial vessels and is able to predict the dose response behavior of AVMs with a complex vascular structure.

  2. PHACE syndrome is associated with intracranial cavernous malformations.

    PubMed

    Foster, Kimberly A; Ares, William J; Tempel, Zachary J; McCormick, Andrew A; Panigrahy, Ashok; Grunwaldt, Lorelei J; Greene, Stephanie

    2016-08-01

    PHACE syndrome is a neurocutaneous disorder involving large facial hemangiomas in association with posterior fossa abnormalities, cerebral arterial anomalies, cardiac defects, and eye abnormalities. A recent consensus statement has delineated criteria necessary for the diagnosis of PHACE syndrome. Extracutaneous manifestations of PHACE syndrome predominately affect the cerebrovascular system. To date, there are no reports of cerebral cavernous malformations (CCMs) in children with PHACE syndrome. We reviewed the charts of children admitted to the Children''s Hospital of Pittsburgh who met criteria for PHACE syndrome, and evaluated neuroimaging for cerebrovascular abnormalities, including the finding of CCMs. Six children met criteria for PHACE syndrome at our institution over a 10-year period. All children were female. All children had cerebrovascular abnormalities sufficient to meet major criteria for diagnosis. Four children (66.7 %) were found incidentally to have CCMs; all lesions measured less than 5 mm at the time of diagnosis and were asymptomatic. At present, CCMs are not listed among the diagnostic criteria for PHACE syndrome, and they have not previously been reported in association with PHACE syndrome. Hypoxic injury in utero may be the common denominator in the pathogenesis of many of the abnormalities already accepted in the criteria for PHACE syndrome and the formation of CCMs. In the setting of PHACE syndrome, we encourage clinicians to evaluate children for CCMs, which are readily apparent on the already-recommended screening MRIs.

  3. [Proteus syndrome: Case report of bladder vascular malformation causing massive hematuria].

    PubMed

    Abbo, O; Bouali, O; Galinier, P; Moscovici, J

    2012-02-01

    Proteus syndrome is a rare, sporadic disorder consisting of disproportionate overgrowth of multiple tissues, vascular malformations, and connective tissue or epidermal nevi. Due to mosaic pattern of distribution, the phenotypes are variable and diverse. Vascular malformations are part of the major criteria used to define and diagnose this syndrome. It can involve the gastrointestinal tract, spleen, or the urinary tract but bladder malformations are rare. We report here a case of bladder vascular malformation in a 12-year-old boy known to have Proteus syndrome and review the literature on bladder malformations or tumors in this syndrome. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  4. Evidence of redistribution of cerebral blood flow during treatment for an intracranial arteriovenous malformation

    SciTech Connect

    Batjer, H.H.; Purdy, P.D.; Giller, C.A.; Samson, D.S. )

    1989-10-01

    The presence of an intracranial arteriovenous malformation has a dramatic impact on local circulatory dynamics. Treatment of some arteriovenous malformations can result in disastrous hyperemic states caused by redistribution of previously shunted blood. This report describes serial hemodynamic measurements of both cerebral blood flow and flow velocity in 3 patients during treatment for arteriovenous malformations. Measurements of cerebral blood flow were made by computed tomographic scan employing the stable xenon inhalation technique; flow velocity, including autoregulatory characteristics, was measured by transcranial Doppler ultrasonogram. Substantial hyperemia developed in one patient (Case 1) after resection and in another (Case 3) after embolization. Embolization resulted in restoration of normal regional cerebral blood flow in a patient who demonstrated hypoperfusion before treatment (Case 2). In Patient 1, postoperative hyperemia was associated with persistently elevated flow velocities, and may have been accompanied by hemispheric neurological deficits. Sequential hemodynamic measurements may predict patients at risk of perioperative complications, and may become useful clinical guidelines for the extent and timing of embolization and for the timing of surgery after intracranial hemorrhage or preoperative embolization procedures.

  5. Nontraumatic intracranial hemorrhage.

    PubMed

    Fischbein, Nancy J; Wijman, Christine A C

    2010-11-01

    Nontraumatic (or spontaneous) intracranial hemorrhage most commonly involves the brain parenchyma and subarachnoid space. This entity accounts for at least 10% of strokes and is a leading cause of death and disability in adults. Important causes of spontaneous intracranial hemorrhage include hypertension, cerebral amyloid angiopathy, aneurysms, vascular malformations, and hemorrhagic infarcts (both venous and arterial). Imaging findings in common and less common causes of spontaneous intracranial hemorrhage are reviewed.

  6. [Extracranial vascular anomalies (hemangiomas and vascular malformations) in children and adolescents--diagnosis, clinic, and therapy].

    PubMed

    Eivazi, B; Werner, J A

    2014-03-01

    The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be

  7. Treatment of vascular malformation of the gastrointestinal tract

    NASA Astrophysics Data System (ADS)

    Waldschmidt, Juergen; Stroedter, L.; Doede, T.; Kischkel, A.

    2000-06-01

    Vascular malformations of the gastrointestinal tract are rare phenomenon. They are generally manifested by upper or lower GI - bleeding and do not resolve spontaneously. Emergency intervention is necessary. This paper reports on 10 cases, treated in the Dept. of Pediatric surgery of the FU Berlin, recorded from 1981 to 1999. We use the Nd:YAG laser 1064 nm, Fibertom 5100, Dornier, Germany, with a 600nm barefiber. Reduction in size of the hemangiomas and stop of the GI-bleeding was achieved in all cases.

  8. Pediatric stroke: the importance of cerebral arteriopathy and vascular malformations.

    PubMed

    Beslow, Lauren A; Jordan, Lori C

    2010-10-01

    Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 person-years. More than half of children who have had a stroke have long-term neurological sequelae. The goal of this article is to review recent literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations as stroke risk factors. Additionally, we review diagnostic studies for childhood stroke, outcome data, and regional and geographic practice differences. PubMed was searched using the terms child, childhood, pediatric, stroke, ischemic, intracerebral hemorrhage, vasculopathy, and vascular malformation. Reference lists of these articles were reviewed for additional key publications. Preference was given to articles published since the year 2000; however, seminal articles in the field were also reviewed. Pediatric stroke is a heterogeneous disorder and a major cause of morbidity in the pediatric population. Five-year recurrence risk is estimated to be 5-19%. Children with cerebrovascular abnormalities are at the highest risk of recurrence (66% at 5 years for ischemic stroke in one study). Furthermore, cerebral arteriopathy including arterial dissection may account for up to 80% of childhood stroke in otherwise healthy children. In many cases, evaluation and treatment of pediatric stroke is not evidence-based, and regional and geographic variations in practice patterns exist. Ultimately multicenter cohort studies and dedicated pediatric clinical trials are essential to establish comprehensive evidence-based guidelines for pediatric stroke care.

  9. 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.

  10. Three-dimensional dynamic time-resolved contrast-enhanced MRA using parallel imaging and a variable rate k-space sampling strategy in intracranial arteriovenous malformations.

    PubMed

    Petkova, Mina; Gauvrit, Jean-Yves; Trystram, Denis; Nataf, François; Godon-Hardy, Sylvie; Munier, Thierry; Oppenheim, Catherine; Meder, Jean-François

    2009-01-01

    To evaluate the effectiveness of three-dimensional (3D) dynamic time-resolved contrast-enhanced MRA (TR-CE-MRA) using a combination of a parallel imaging technique (ASSET: array spatial sensitivity encoding technique) and a time-resolved method (TRICKS: time-resolved imaging of contrast kinetics) and to compare it with 3D dynamic TR-CE-MRA using ASSET alone in the assessment of intracranial arteriovenous malformations (AVMs). Twenty consecutive patients with angiographically confirmed AVMs were investigated using both 3D dynamic TR-CE-MRA techniques. Examinations were compared with respect to image quality, spatial resolution, number and type of feeders and drainers, nidus size, presence of early venous filling and temporal resolution. Digital subtraction angiography was used as standard of reference. The higher temporal and spatial resolution of 3D dynamic TR-CE-MRA TRICKS ASSET allowed a better assessment of intracranial vascular malformations, namely better depiction of feeders, drainers and better detection of early venous drainage. There was no significant difference between them in terms of nidus size. 3D dynamic TR-CE-MRA combining parallel imaging and a time-resolved method with subsecond and submillimeter resolution could become the first-line investigation technique in both diagnosis and follow-up of intracranial AVMs.

  11. Emerging role of contrast-enhanced MRI in diagnosing vascular malformations.

    PubMed

    Turley, Ryan S; Lidsky, Michael E; Markovic, Jovan N; Shortell, Cynthia K

    2014-07-01

    Vascular malformations comprise a diverse and rare group of lesions which generally pose a formidable treatment challenge. Requisite for optimal surgical planning are imaging modalities capable of delineating involved anatomy and malformation flow characteristics. In this regard, we and others have purported the advantages of contrast-enhanced MRI. Here, we review the current body of literature regarding the emerging of role of contrast enhanced MRI for the management of vascular malformations.

  12. Advances in non-invasive imaging of intracranial vascular disease.

    PubMed Central

    Jäger, H. R.; Grieve, J. P.

    2000-01-01

    Intra-arterial catheter angiography has, in the past, been the mainstay for the investigation of intracranial vascular disease. It is, however, invasive, usually requires in-patients admission, and is associated with a rate of neurological complications between 1% and 3%. In recent years, magnetic resonance angiography (MRA) and CT angiography (CTA) have emerged as non-invasive alternatives for imaging blood vessels and have made a significant impact on neuroradiological investigations. It is the purpose of this article to explain the basic technical principles of these two methods and to give an overview of their current clinical applications. PMID:10700757

  13. Association between Venous Angioarchitectural Features of Sporadic Brain Arteriovenous Malformations and Intracranial Hemorrhage.

    PubMed

    Alexander, M D; Cooke, D L; Nelson, J; Guo, D E; Dowd, C F; Higashida, R T; Halbach, V V; Lawton, M T; Kim, H; Hetts, S W

    2015-05-01

    Intracranial hemorrhage is the most serious outcome for brain arteriovenous malformations. This study examines associations between venous characteristics of these lesions and intracranial hemorrhage. Statistical analysis was performed on a prospectively maintained data base of brain AVMs evaluated at an academic medical center. DSA, CT, and MR imaging studies were evaluated to classify lesion side, drainage pattern, venous stenosis, number of draining veins, venous ectasia, and venous reflux. Logistic regression analyses were performed to identify the association of these angiographic features with intracranial hemorrhage of any age at initial presentation. Exclusively deep drainage (OR, 3.42; 95% CI, 1.87-6.26; P < .001) and a single draining vein (OR, 1.98; 95% CI, 1.26-3.08; P = .002) were associated with hemorrhage, whereas venous ectasia (OR, 0.52; 95% CI, 0.34-0.78; P = .002) was inversely associated with hemorrhage. Analysis of venous characteristics of brain AVMs may help determine their prognosis and thereby identify lesions most appropriate for treatment. © 2015 by American Journal of Neuroradiology.

  14. Association Between Venous Angioarchitectural Features of Sporadic Brain Arteriovenous Malformations and Intracranial Hemorrhage

    PubMed Central

    Alexander, Matthew D.; Cooke, Daniel L.; Nelson, Jeffrey; Guo, Diana E.; Dowd, Christopher F.; Higashida, Randall T.; Halbach, Van V.; Lawton, Michael T.; Kim, Helen; Hetts, Steven W.

    2015-01-01

    Background and Purpose Intracranial hemorrhage is the most serious outcome for brain arteriovenous malformations (AVM). This study examines associations between venous characteristics of these lesions and intracranial hemorrhage. Materials and Methods Statistical analysis was performed on a prospectively maintained database of brain AVMs evaluated at an academic medical center. DSA, CT, and MRI studies were evaluated to classify lesion side, drainage pattern, venous stenosis, number of draining veins, venous ectasia, and venous reflux. Logistic regression analyses were performed to identify association of these angiographic features with intracranial hemorrhage of any age at initial presentation. Results Exclusively deep drainage (OR 3.42, 95% CI 1.87–6.26, p<0.001) and a single draining vein (OR 1.98, 95% CI 1.26–3.08, p=0.002) were associated with hemorrhage, whereas venous ectasia (OR 0.52, 95% CI 0.34–0.78, p=0.002) was inversely associated with hemorrhage. Conclusion Analysis of venous characteristics of brain AVMs may help determine their prognosis and thereby identify lesions most appropriate for treatment. PMID:25634722

  15. Radionuclide-labeled red blood cell imaging of vascular malformations in children

    SciTech Connect

    Sloan, G.M.; Bolton, L.L.; Miller, J.H.; Reinisch, J.F.; Nichter, L.S.

    1988-09-01

    Vascular malformations, particularly in the absence of cutaneous changes, can be difficult to distinguish from other soft tissue masses in children. We have used technetium-99m-labeled red blood cell scintigraphy to study 47 lesions in 43 children. Thirty-nine lesions showed increased flow and were, therefore, diagnosed as vascular malformations. Subsequent biopsy of 10 of these lesions confirmed that diagnosis. The other 29 lesions with increased flow were followed for 10 months to 5 years and the clinical course was consistent with vascular malformation in every case. Eight lesions showed no increased flow on technetium scan. One of these subsequently proved to be a hemangioma. The others have turned out not to be vascular malformations. Therefore, in our experience, the technetium-99m-labeled red blood cell scan has had 98% sensitivity and 100% specificity in diagnosing vascular malformations in children.

  16. A Vascular Malformation Presenting as a Peripheral Nerve Sheath Tumor

    PubMed Central

    Parmar, Vikas; Haldeman, Clayton; Amaefuna, Steve; Hanna, Amgad S.

    2016-01-01

    We present the case of a venous malformation (VM) masquerading as a schwannoma. VMs are thin-walled vascular dilations of various sizes that typically present as soft, compressible, blue masses that are associated with pain or dysesthesia. VMs are commonly found in the head and neck as well as the distal extremities. Notably, slow-flow VMs are hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging, and enhance markedly with contrast. However, VMs tend to be poorly circumscribed and fraught with venous lakes and phleboliths. Conservative therapy and sclerotherapy are the primary treatment options. In this case report, we present a VM presenting near the neurovascular bundle of the upper extremity axilla. Our case is unique in that the patient presented with symptoms and imaging qualities characteristic for a peripheral nerve schwannoma. PMID:28077959

  17. Comparative observational study on the clinical presentation, intracranial volume measurements, and intracranial pressure scores in patients with either Chiari malformation Type I or idiopathic intracranial hypertension.

    PubMed

    Frič, Radek; Eide, Per Kristian

    2017-04-01

    OBJECTIVE Several lines of evidence suggest common pathophysiological mechanisms in Chiari malformation Type I (CMI) and idiopathic intracranial hypertension (IIH). It has been hypothesized that tonsillar ectopy, a typical finding in CMI, is the result of elevated intracranial pressure (ICP) combined with a developmentally small posterior cranial fossa (PCF). To explore this hypothesis, the authors specifically investigated whether ICP is comparable in CMI and IIH and whether intracranial volumes (ICVs) are different in patients with CMI and IIH, which could explain the tonsillar ectopy in CMI. The authors also examined whether the symptom profile is comparable in these 2 patient groups. METHODS The authors identified all CMI and IIH patients who had undergone overnight diagnostic ICP monitoring during the period from 2002 to 2014 and reviewed their clinical records and radiological examinations. Ventricular CSF volume (VV), PCF volume (PCFV), and total ICV were calculated from initial MRI studies by using volumetric software. The static and pulsatile ICP scores during overnight monitoring were analyzed. Furthermore, the authors included a reference (REF) group consisting of patients who had undergone ICP monitoring due to suspected idiopathic normal-pressure hydrocephalus or chronic daily headache and showed normal pressure values. RESULTS Sixty-six patients with CMI and 41 with IIH were identified, with comparable demographics noted in both groups. The occurrence of some symptoms (headache, nausea, and/or vomiting) was comparable between the cohorts. Dizziness and gait ataxia were significantly more common in patients with CMI, whereas visual symptoms, diplopia, and tinnitus were significantly more frequent in patients with IIH. The cranial volume measurements (VV, PCFV, and ICV) of the CMI and IIH patients were similar. Notably, 7.3% of the IIH patients had tonsillar descent qualifying for diagnosis of CMI (that is, > 5 mm). The extent of tonsillar ectopy was

  18. Cerebral Cavernous Malformations: Somatic Mutations in Vascular Endothelial Cells

    PubMed Central

    Gault, Judith; Awad, Issam A.; Recksiek, Peter; Shenkar, Robert; Breeze, Robert; Handler, Michael; Kleinschmidt-DeMasters, Bette Kay

    2009-01-01

    OBJECTIVE Germline mutations in three genes have been found in familial cases of cerebral cavernous malformations (CCM). We previously discovered somatic and germline truncating mutations in the KRIT1 gene supporting the “two-hit” mechanism of CCM lesion formation in a single lesion. The purpose of this study was to screen for somatic, nonheritable, mutations in three more lesions from different patients and identify the cell type(s) in which somatic mutations occur. METHODS Somatic mutations were sought in DNA from three surgically excised, fresh-frozen CCM lesions by cloning and screening PCR products generated from KRIT1 or PDCD10 coding regions. Laser capture microdissection (LCM) was used to isolated endothelial and nonendothelial cells in order to determine if somatic mutations were found in endothelial cells. RESULTS A CCM lesion harbored somatic and germline KRIT1 mutations on different chromosomes and are therefore biallelic. Both mutations are predicted to truncate the protein. The KRIT1 somatic mutations (novel c.1800delG mutation and previously identified 34 nucleotide deletion) in CCMs from two different patients were only found in the vascular endothelial cells lining caverns. No obvious somatic mutations were identified in the two other lesions; however, the results were inconclusive possibly due to the technical limitations or the fact that these specimens had a small proportion of vascular endothelial cells lining pristine caverns. CONCLUSION The “two-hit” mechanism occurs in vascular endothelial cells lining CCM caverns from two patients with somatic and Hispanic-American KRIT1 germline mutations. Methods for somatic mutation detection should focus on vascular endothelial cells lining pristine caverns. PMID:19574835

  19. Congenital vascular malformation associated with multiple cranial, vertebral and upper limb skeletal abnormalities.

    PubMed

    Marsden, N; Shokrollahi, K; Maw, K; Sierakowski, A; Bhat, F A; Mathur, B

    2010-07-01

    The association between congenital vascular malformations and altered bone growth, the so-called vascular bone syndrome, is well documented. Various eponymous syndromes each with their individual traits, such as Klippel-Trenaunay, Parkes-Weber and Servelle-Martorell syndrome have been described, along with variations. We report on a previously undescribed case of congenital vascular malformation associated with multiple skeletal abnormalities affecting the skull, vertebrae and right upper limb, and discuss the literature.

  20. Racial/Ethnic differences in longitudinal risk of intracranial hemorrhage in brain arteriovenous malformation patients.

    PubMed

    Kim, Helen; Sidney, Stephen; McCulloch, Charles E; Poon, K Y Trudy; Singh, Vineeta; Johnston, S Claiborne; Ko, Nerissa U; Achrol, Achal S; Lawton, Michael T; Higashida, Randall T; Young, William L

    2007-09-01

    Race/ethnicity is associated with overall incidence of intracranial hemorrhage (ICH), but its impact in patients with brain arteriovenous malformation is unknown. We evaluated whether race/ethnicity was a risk factor for ICH in the natural course in a large, multiethnic cohort of patients with brain arteriovenous malformation followed longitudinally. Data were collected prospectively for patients with brain arteriovenous malformation evaluated at the University of California, San Francisco (n=436) and retrospectively through databases and chart review in the 20 hospitals of the Kaiser Permanente Medical Care Program (n=1028). Multivariate Cox regression was performed to assess the influence of race/ethnicity on subsequent ICH, adjusting for risk factors. Cases were censored at first treatment, loss to follow-up, or death. Average follow up was 4.7+/-8.0 years for Kaiser Permanente Medical Care Program patients and 2.8+/-7.3 years for University of California, San Francisco patients with no difference in time to ICH between cohorts (log rank P=0.57). The annualized 5-year ICH rate was 2.1% (3.7% for ruptured at presentation; 1.4% for unruptured). Initial ICH presentation (hazard ratio: 3.0, 95% CI: 1.9 to 4.9, P<0.001) and Hispanic race/ethnicity (hazard ratio: 1.9, 95% CI: 1.1 to 3.3, P=0.02) were independent predictors of ICH, adjusting for age, gender, cohort, and a cohort-age interaction. The ICH risk for Hispanics versus whites increased to 3.1 (95% CI: 1.3 to 7.4, P=0.013) after further adjusting for arteriovenous malformation size and deep venous drainage in a subset of cases with complete data. Similar trends were observed for blacks (hazard ratio: 2.1, 95% CI: 0.9 to 4.8, P=0.09) and Asians (hazard ratio: 2.4, 95% CI: 0.8 to 7.1, P=0.11), although nonsignificant. This study reports the first description of race/ethnic differences in brain arteriovenous malformation, with Hispanics at an increased risk of subsequent ICH compared with whites.

  1. [Screening for systemic manifestations of vascular malformations in patients with hereditary haemorrhagic telangiectasia (Osler disease)].

    PubMed

    Cerra Pohl, Ana; Werner, Jochen Alfred; Folz, Benedikt Josef

    2008-11-01

    Hereditary haemorrhagic telangiectasia (Rendu-Osler- Weber syndrome) is a disease characterized by systemic vascular malformations. Typical clinical manifestations are recurrent epistaxis and telangiectases of the skin and the mucous membranes. The syndrome is furthermore characterized by its hereditary aspect. The disease seems to be much more complicated than previously thought, mainly because of the accompanying vascular malformations in vital organs, like the liver, the kidney, the lung, the brain, and the eyes. The diagnosis and treatment of systemic vascular malformations requires interdisciplinary management.

  2. Management of Low-Flow Vascular Malformations: Clinical Presentation, Classification, Patient Selection, Imaging and Treatment

    SciTech Connect

    McCafferty, Ian

    2015-10-15

    This review article aims to give an overview of the current state of imaging, patient selection, agents and techniques used in the management of low-flow vascular malformations. The review includes the current classifications for low-flow vascular malformations including the 2014 updates. Clinical presentation and assessment is covered with a detailed section on the common sclerosant agents used to treat low-flow vascular malformations, including dosing and common complications. Imaging is described with a guide to a simple stratification of the use of imaging for diagnosis and interventional techniques.

  3. Laser therapy and sclerotherapy in the treatment of oral and maxillofacial hemangioma and vascular malformations

    NASA Astrophysics Data System (ADS)

    Crişan, Bogdan; BǎciuÅ£, Mihaela; BǎciuÅ£, Grigore; Crişan, Liana; Bran, Simion; Rotar, Horatiu; Moldovan, Iuliu; Vǎcǎraş, Sergiu; Mitre, Ileana; Barbur, Ioan; Magdaş, Andreea; Dinu, Cristian

    2016-03-01

    Hemangioma and vascular malformations in the field of oral and maxillofacial surgery is a pathology more often found in recent years in patients. The aim of this study was to evaluate the efficacy of the laser photocoagulation performed with a diode laser (Ga-Al-As) 980 nm wavelength in the treatment of vascular lesions which are located on the oral and maxillofacial areas, using color Doppler ultrasonography for evaluation of the results. We also made a comparison between laser therapy and sclerotherapy in order to establish treatment protocols and recommendations associated with this pathology. We conducted a controlled study on a group of 92 patients (38 male and 54 female patients, with an average age of 36 years) having low flow hemangioma and vascular malformations. Patients in this trial received one of the methods of treatment for vascular lesions such as hemangioma and vascular malformations: laser therapy or sclerotherapy. After laser therapy we have achieved a reduction in size of hemangioma and vascular malformations treated with such a procedure, and the aesthetic results were favorable. No reperfusion or recanalization of laser treated vascular lesions was observed after an average follow-up of 6 to 12 months. In case of sclerotherapy a reduction in the size of vascular lesions was also obtained. The 980 nm diode laser has been proved to be an effective tool in the treatment of hemangioma and vascular malformations in oral and maxillofacial area. Laser therapy in the treatment of vascular lesions was more effective than the sclerotherapy procedure.

  4. Imaging and therapeutic approach of hemangiomas and vascular malformations in the pediatric age group.

    PubMed

    Dubois, J; Garel, L

    1999-12-01

    Terminology regarding the vascular lesions of the soft tissues remains confusing. A single classification is necessary in order to decide on the proper investigation and the best treatment. At the Workshop on Vascular Anomalies in Rome in June 1996, the membership accepted the Mulliken and Glowacki classification, which differentiates vascular lesions into vascular tumors, including hemangiomas and vascular malformations. At Sainte-Justine, we have set up a multidisciplinary clinic for the discussion of problem patients with vascular anomalies, both in terms of diagnosis and treatment. In this review, we present our experience regarding the classification, the imaging modalities and the treatment of vascular anomalies. In our experience, Doppler ultrasound should be the initial imaging modality for recognizing vascular tumors from vascular malformations. CT scan or magnetic resonance imaging is best to evaluate the extent of the lesions prior to treatment. A multidisciplinary approach is essential to establish a correct diagnosis and define accordingly the appropriate treatment and follow-up.

  5. Pediatric Stroke: The Importance of Cerebral Arteriopathy and Vascular Malformations

    PubMed Central

    Beslow, Lauren A.; Jordan, Lori C.

    2011-01-01

    Stroke is an important cause of neurologic morbidity in childhood. Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 children. This article will review recent literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations as stroke risk factors. Additional risk factors include congenital heart disease, sickle cell disease, and hematologic abnormalities among others. Outcomes are variable and are related to the severity of presentation, associated illnesses, and other factors. More than half of children who have had a stroke have long-term neurological sequelae. Five-year recurrence risk is estimated to be 5–19%. Children with cerebrovascular abnormalities are at the highest risk of recurrence (66% at 5 years for ischemic stroke in one study). Furthermore, cerebral arteriopathy including arterial dissection may account for up to 80% of childhood stroke in otherwise healthy children. In many cases, evaluation and treatment of pediatric stroke is not evidence-based, and regional and geographic variations in practice patterns exist. Multicenter cohort studies and ultimately dedicated pediatric clinical trials will be essential to establish comprehensive evidence-based guidelines for pediatric stroke care. PMID:20625743

  6. Structure and vascular function of MEKK3–cerebral cavernous malformations 2 complex

    SciTech Connect

    Fisher, Oriana S.; Deng, Hanqiang; Liu, Dou; Zhang, Ya; Wei, Rong; Deng, Yong; Zhang, Fan; Louvi, Angeliki; Turk, Benjamin E.; Boggon, Titus J.; Su, Bing

    2015-08-03

    Cerebral cavernous malformations 2 (CCM2) loss is associated with the familial form of CCM disease. The protein kinase MEKK3 (MAP3K3) is essential for embryonic angiogenesis in mice and interacts physically with CCM2, but how this interaction is mediated and its relevance to cerebral vasculature are unknown. Here we report that Mekk3 plays an intrinsic role in embryonic vascular development. Inducible endothelial Mekk3 knockout in neonatal mice is lethal due to multiple intracranial haemorrhages and brain blood vessels leakage. We discover direct interaction between CCM2 harmonin homology domain (HHD) and the N terminus of MEKK3, and determine a 2.35 Å cocrystal structure. We find Mekk3 deficiency impairs neurovascular integrity, which is partially dependent on Rho–ROCK signalling, and that disruption of MEKK3:CCM2 interaction leads to similar neurovascular leakage. We conclude that CCM2:MEKK3-mediated regulation of Rho signalling is required for maintenance of neurovascular integrity, unravelling a mechanism by which CCM2 loss leads to disease.

  7. Structure and vascular function of MEKK3–cerebral cavernous malformations 2 complex

    PubMed Central

    Fisher, Oriana S.; Deng, Hanqiang; Liu, Dou; Zhang, Ya; Wei, Rong; Deng, Yong; Zhang, Fan; Louvi, Angeliki; Turk, Benjamin E.; Boggon, Titus J.; Su, Bing

    2015-01-01

    Cerebral cavernous malformations 2 (CCM2) loss is associated with the familial form of CCM disease. The protein kinase MEKK3 (MAP3K3) is essential for embryonic angiogenesis in mice and interacts physically with CCM2, but how this interaction is mediated and its relevance to cerebral vasculature are unknown. Here we report that Mekk3 plays an intrinsic role in embryonic vascular development. Inducible endothelial Mekk3 knockout in neonatal mice is lethal due to multiple intracranial haemorrhages and brain blood vessels leakage. We discover direct interaction between CCM2 harmonin homology domain (HHD) and the N terminus of MEKK3, and determine a 2.35 Å cocrystal structure. We find Mekk3 deficiency impairs neurovascular integrity, which is partially dependent on Rho–ROCK signalling, and that disruption of MEKK3:CCM2 interaction leads to similar neurovascular leakage. We conclude that CCM2:MEKK3-mediated regulation of Rho signalling is required for maintenance of neurovascular integrity, unravelling a mechanism by which CCM2 loss leads to disease. PMID:26235885

  8. Surgical Management of Giant Intracranial Arteriovenous Malformations: A Single Center Experience over 32 years.

    PubMed

    Reinard, Kevin A; Pabaney, Aqueel H; Basheer, Azam; Phillips, Scott B; Kole, Max K; Malik, Ghaus M

    2015-12-01

    Treatment of giant intracranial arteriovenous malformations (gAVMs) is a formidable challenge for neurosurgeons and carries significant morbidity and mortality rates for patients compared with smaller AVMs. In this study, we reviewed the treatments, angiographic results, and clinical outcomes in 64 patients with gAVMs who were treated at Henry Ford Hospital between 1980 and 2012. The arteriovenous malformation (AVM) database at our institution was queried for patients with gAVMs (≥ 6 cm) and data regarding patient demographics, presentation, AVM angioarchitecture, and treatments were collected. Functional outcomes as well as complications were analyzed. Of the 64 patients, 33 (51.6%) were female and 31 (48.4%) were male, with an average age of 45.7 years (SD ± 15.5). The most common symptoms on presentation were headaches (50%), seizures (50%), and hemorrhage (41%). The mean AVM size was 6.65 cm (range, 6-9 cm). Only 6 AVMs (9.4%) were located in the posterior fossa. The most common Spetzler-Martin grade was V, seen in 64% of patients. Of the 64 patients, 42 (66%) underwent surgical excision, 10 (15.5%) declined any treatment, 8 (12.5%) were deemed inoperable and followed conservatively, 2 (3%) had stand-alone embolization, 1 (1.5%) had embolization before stereotactic radiosurgery, and 1 (1.5%) received stereotactic radiosurgery only. Complete obliteration was achieved in 90% of the surgical patients. Mortality rate was 19% in the surgical cohort compared with 22% in the observation cohort (P = 0.770). Treatment of gAVMs carries significant morbidity and mortality; however, good outcomes are attainable with a multimodal treatment approach in carefully selected patients. Copyright © 2015 Elsevier Inc. All rights reserved.

  9. Diagnosis and management of extensive vascular malformations of the lower limb: part II. Systemic repercussions [corrected], diagnosis, and treatment.

    PubMed

    Redondo, Pedro; Aguado, Leyre; Martínez-Cuesta, Antonio

    2011-11-01

    At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. Extensive vascular malformations are often more complex than they appear and require a multidisciplinary therapeutic approach. Vascular malformations may be associated with underlying disease or systemic anomalies. Part II of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limb highlights the systemic repercussions [corrected] (bone, articular, visceral, and hematologic involvement), diagnosis, and treatment of these lesions.

  10. Microsurgery for intracranial arteriovenous malformation: Long-term outcomes in 445 patients

    PubMed Central

    Zeng, Yunhui; Liu, Zhiyong; Liu, Hao; Xu, Jianguo

    2017-01-01

    Background The management of intracranial arteriovenous malformations(AVMs) poses challenges to the cerebrovascular specialists. Objective To review the long-term outcomes of intracranial AVMs treated with microsurgical resections. Methods We performed a retrospective review of 445 patients with intracranial AVMs treated in our hospital from January 1st, 2008 to December 31st, 2014. The extracted data included demographic characteristics, clinical presentations, Spetzler-Martin (SM) grades, Supplemented Spetzler-Martin (SM-Supp) Grades, treatment modalities, long-term outcomes, and obliteration rates. Outcome was assessed with a post-operative modified Rankin Scale (mRS) score at the last follow-up visit. Results Of the 445 patients treated with microsurgery, 298 (67.0%) patients initially presented with hemorrhage. Based on the SM grading system, the patients were graded as follows: 83(18.6%) Grade I, 156(35.1%) Grade II, 132(29.7%) Grade III, 61(13.7%) Grade IV and 13(2.9%) Grade V. Overall, 344(77.3%) patients had a favorable outcome (mRS score of 0–2). The favorable outcome for Grade I and II were 92.8% and 85.9%, respectively, sharply reducing to 52.5% in patients with Grade IV and 15.4% in patients with Grade V AVMs. 388(87.2%) patients achieved complete obliteration of the AVMs. 63(14.2%) patients experienced recurrent hemorrhage, and the frequency of rehemorrhage was highest in Grade V patients (77.0%), dropping to 3.6% and 3.8% in patients with Grade I and II lesions, respectively. Permanent neurological deficits occurred in 66(14.8%) patients and death in 35(7.9%) patients. There was no difference of AUROC values between SM grading system and SM-supp grading system (0.726 and 0.734, respectively, p = .715). Conclusion The Spetzler-Martin grading system is a simple and effective method to estimate the risk of surgery and to evaluate the prognosis. Microsurgical resection for AVMs depends on the SM grades, and the morbidity-mortality rate increases with

  11. Outcome after Gamma Knife surgery for intracranial arteriovenous malformations in children.

    PubMed

    Tamura, Noriko; Hayashi, Motohiro; Chernov, Mikhail; Tamura, Manabu; Horiba, Ayako; Konishi, Yoshiyuki; Muragaki, Yoshihiro; Iseki, Hiroshi; Okada, Yoshikazu

    2012-12-01

    The focus of the present study was the evaluation of outcomes after unstaged and staged-volume Gamma Knife surgery (GKS) in children harboring intracranial arteriovenous malformations (AVMs). Twenty-two children (median age 9.5 years) underwent GKS for AVMs and were followed up for at least 2 years thereafter. The disease manifested with intracranial hemorrhage in 77% of cases. In 68% of patients the lesion affected eloquent brain structures. The volume of the nidus ranged from 0.1 to 6.7 cm(3). Gamma Knife surgery was guided mainly by data from dynamic contrast-enhanced CT scans, with preferential targeting of the junction between the nidus and draining vein. The total prescribed isodose volume was kept below 4.0 cm(3), and the median margin dose was 22 Gy (range 20-25 Gy). If the volume of the nidus was larger than 4.0 cm(3), a second radiosurgical session was planned for 3-4 years after the first one. Nine patients in the present series underwent unstaged radiosurgery, whereas staged-volume treatment was scheduled in 13 patients. Complete obliteration of the AVM was noted in 17 (77%) of 22 patients within a median period of 47 months after the last radiosurgical session. Complete obliteration of the lesion occurred in 89% of patients after unstaged treatment and in 62.5% after staged GKS. Four (67%) of 6 high-grade AVMs were completely obliterated. Complications included 3 bleeding episodes, the appearance of a region of hyperintensity on T(2)-weighted MR images in 2 patients who had no symptoms, and reappearance of the nidus in the vicinity of the completely obliterated AVM in 1 patient. Radiosurgery is a highly effective management option for intracranial AVMs in children. For larger lesions, staged GKS may be applied successfully. Initial targeting of the nidus adjacent to the draining vein and application of a sufficient radiation dose to a relatively small volume (≤ 4 cm(3)) provides a good balance between a high probability of obliteration and a low risk

  12. Bleeding recurrence in patients with gastrointestinal vascular malformation after thalidomide.

    PubMed

    Chen, Haiying; Fu, Sengwang; Feng, Nan; Chen, Huimin; Gao, Yunjie; Zhao, Yunjia; Xue, Hanbing; Zhang, Yao; Li, Xiaobo; Dai, Jun; Fang, Jingyuan; Ge, Zhizheng

    2016-08-01

    Thalidomide may be used for the treatment of gastrointestinal vascular malformation (GIVM), but the long-term response and adverse effects are unknown. Aim to study the recurrence rate of GIVM bleeding after thalidomide treatment, the response to treatment, and the adverse effects.This was a retrospective study of 80 patients with GIVM treated with thalidomide between November 2003 and November 2013. Patients received a course of 100 mg/day of thalidomide for 4 months and were followed up for at least 1 year. The response rate during follow-up, the recurrence rate after the 1st course of treatment, and the rate of retreatment were assessed. Comorbidities, the need for blood transfusion, yearly bleeding episodes, hemoglobin levels, hospitalization after thalidomide treatment, and the rate of adverse effects were also examined.The overall response rate during follow-up was 79.5% (62/78). The recurrence rate was 21.0% after the 1st course of thalidomide. The response rate of retreatment was 100%. After thalidomide treatment, yearly blood transfusion amounts, yearly bleeding episodes, and yearly hospitalization numbers were significantly decreased, while hemoglobin levels were significantly increased (P < 0.001). Adverse effects were observed in 60.0% (48/80) of the patients. Serious adverse effects were reported in 31.3% (25/80). The overall response rate was 76.7% (23/30) in 30 patients with comorbidities, while the rate was 78.0% (39/50) in patients without comorbidities (P = 0.55). The rate of serious adverse effects was similar between the comorbidities (33.3%) and no-comorbidities groups (30.0%) (P = 0.76).Thalidomide showed a good response rate and low adverse effect rate in patients with recurrent gastrointestinal bleeding due to GIVM.

  13. Delayed expansion of an intracranial cyst induced by ventriculoperitoneal shunt in a patient with Chiari malformation type 2.

    PubMed

    Maki, Yoshinori; Ohta, Tsuyoshi; Onishi, Hirokazu; Fukui, Naoki; Morimoto, Masanori

    2017-02-11

    A male neonate was referred to our hospital for the treatment of myelomeningocele and hydrocephalus related to Chiari malformation type 2. After the placement of ventriculoperitoneal (VP) shunt for hydrocephalus, an intracranial cyst, which was asymptomatic at first, expanded and caused clinical symptoms due to increased intracranial pressure. The expansion of the cyst was confirmed dominantly in the right lateral ventricle where the VP shunt was inserted. Intraoperative findings through a neuroendoscope suggested that the expanded cyst was an isolated lesion. The pressure reduction in the lateral ventricle through the shunt tube, not the malfunction of the VP shunt, might have attributed to increased intracranial pressure with the expanded cyst. The authors present this rare case, speculating the cause of the expansion of the cyst.

  14. 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.

  15. 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

  16. Multispectral imaging of pigmented and vascular cutaneous malformations: the influence of laser treatment

    NASA Astrophysics Data System (ADS)

    Kuzmina, Ilona; Diebele, Ilze; Asare, Lasma; Kempele, Anna; Abelite, Anita; Jakovels, Dainis; Spigulis, Janis

    2010-11-01

    The paper investigates influence and efficacy of laser therapy on pigmented and vascular cutaneous malformations by multispectral imaging technique. Parameter mapping of skin pigmented and vascular lesions and monitoring of the laser therapy efficacy are performed by multispectral imaging in wavelength range 450-700nm by scanning step - 10nm. Parameter maps of the oxyhemoglobin deoxyhemoglobin and melanin derived from the images are presented. Possibility of laser therapy efficacy monitoring by comparison of the parameter maps before and after laser treatment has been demonstrated. As both cutaneous pigmented and vascular malformations are commonly found lesions, the parameter mapping would be a valuable method to use routinely.

  17. Optic chiasmal cavernous angioma: A rare suprasellar vascular malformation

    PubMed Central

    Abou-Al-Shaar, Hussam; Bahatheq, Ayman; Takroni, Radwan; Al-Thubaiti, Ibrahim

    2016-01-01

    Background: Suprasellar cavernous malformation in the optic pathway is not commonly encountered. To date, there are only few reports present in the literature. Case Description: The authors report a rare case of suprasellar optic pathway cavernous malformation in a 33-year-old female who presented with progressive visual loss. Her imaging revealed a large heterogeneous, hyperintense, hemorrhagic right suprasellar extra-axial complex cystic structure, causing mass effect on the adjacent hypothalamus and third ventricle displacing these structures. Gross total resection of the lesion was achieved utilizing a right frontal craniotomy approach. Histopathological examination confirmed the diagnosis of suprasellar chiasmal cavernous malformation. Conclusion: Although visual pathway cavernous malformation is a rare event, it should be included in the differential diagnosis of lesions occurring suprasellarly in the visual pathway and hypothalamus. PMID:27583178

  18. A Multicenter Retrospective Study of Frameless Robotic Radiosurgery for Intracranial Arteriovenous Malformation

    PubMed Central

    Oermann, Eric K.; Murthy, Nikhil; Chen, Viola; Baimeedi, Advaith; Sasaki-Adams, Deanna; McGrail, Kevin; Collins, Sean P.; Ewend, Matthew G.; Collins, Brian T.

    2014-01-01

    Introduction: CT-guided, frameless radiosurgery is an alternative treatment to traditional catheter-angiography targeted, frame-based methods for intracranial arteriovenous malformations (AVMs). Despite the widespread use of frameless radiosurgery for treating intracranial tumors, its use for treating AVM is not-well described. Methods: Patients who completed a course of single fraction radiosurgery at The University of North Carolina or Georgetown University between 4/1/2005–4/1/2011 with single fraction radiosurgery and received at least one follow-up imaging study were included. All patients received pre-treatment planning with CTA ± MRA and were treated on the CyberKnife (Accuray) radiosurgery system. Patients were evaluated for changes in clinical symptoms and radiographic changes evaluated with MRI/MRA and catheter-angiography. Results: Twenty-six patients, 15 male and 11 female, were included in the present study at a median age of 41 years old. The Spetzler-Martin grades of the AVMs included seven Grade I, 12 Grade II, six Grade III, and one Grade IV with 14 (54%) of the patients having a pre-treatment hemorrhage. Median AVM nidal volume was 1.62 cm3 (0.57–8.26 cm3) and was treated with a median dose of 1900 cGy to the 80% isodose line. At median follow-up of 25 months, 15 patients had a complete closure of their AVM, 6 patients had a partial closure, and 5 patients were stable. Time since treatment was a significant predictor of response, with patients experience complete closure having on average 11 months more follow-up than patients with partial or no closure (p = 0.03). One patient experienced a post-treatment hemorrhage at 22 months. Conclusion: Frameless radiosurgery can be targeted with non-invasive MRI/MRA and CTA imaging. Despite the difficulty of treating AVM without catheter angiography, early results with frameless, CT-guided radiosurgery suggest that it can achieve similar results to frame-based methods at these time

  19. Vascular malformations of the spinal cord (angiodysgenetic myelomalacia): a critique on its pathogenesis.

    PubMed

    Badejo, L; Sangalang, V E

    1979-02-01

    Two cases of angiodysgenetic myelomalacia are presented. Both patients had progressive weakness and sensory deficits in the lower extremities and vascular malformations of their spinal cords. The lesions were located on the dorsum of the spinal cord and the dorso-spinal roots. We believe the symptoms that developed later in life were due to spinal cord ischemia resulting from late degenerative changes in the vessels of the malformation and an ever increasing spinal "steal".

  20. Percutaneous Sclerotherapy of Congenital Slow-Flow Vascular Malformations of the Orbit

    SciTech Connect

    Chiramel, George Koshy Keshava, Shyamkumar Nidugala Moses, Vinu Mammen, Suraj; David, Sarada; Sen, Sudipta

    2015-04-15

    PurposeThis manuscript describes the clinical features, imaging findings, treatment details, and short-term outcomes of a series of congenital slow-flow vascular malformations.MethodsThis was a prospective study of congenital slow-flow vascular malformations involving the orbital region treated at a single institution with percutaneous sclerotherapy.ResultsTen patients presented during the study period, comprising eight venous malformations, one lymphatic malformation, and one veno-lymphatic malformation. Nine patients underwent percutaneous sclerotherapy under digital subtraction angiography guidance, of which three developed marked rise in intraocular pressure requiring lateral canthotomy. The treatments were performed in the presence of an ophthalmologist who measured the intraorbital pressure during and after the procedure. On follow-up, some of the patients required repeat sessions of sclerotherapy. All patients had improvement of symptoms on follow up after the procedure.ConclusionCongenital slow-flow vascular malformations of the orbital region are rare lesions that should be treated using a multidisciplinary approach. Monitoring of the intraorbital pressure is required both during and after the procedure to decide about the need for lateral canthotomy to reduce the transiently increased intraorbital pressure.

  1. The Efficiency of Sclerotherapy in the Treatment of Vascular Malformations: A Retrospective Study of 63 Patients

    PubMed Central

    Rautio, Riitta; Giordano, Salvatore; Koskivuo, Ilkka; Savolainen, Otto

    2016-01-01

    Background and Aims. Vascular malformations are a vast group of congenital malformations that are present at birth. These malformations can cause pain, pressure, and cosmetic annoyance as well as downturn growth and development in a child in the case of high flow. Sclerotherapy has become an important tool in the treatment of vascular malformations. However, little is known about the success rate of sclerotherapy. Material and Methods. In this study, the efficiency of sclerotherapy in the treatment of vascular anomalies was investigated retrospectively in 63 patients treated in Turku University Hospital between 2003 and 2013. Results. Out of the 63 patients investigated, 83% (53) had venous malformations (VMs) and 9% (5) were defined as having arteriovenous malformations (AVMs). Patients with a VM were operated on, in 14% (8) out of all VM cases. Hence 86% (45) of patients with a VM received adequate help to their symptoms solely from sclerotherapy. The duration of treatment for the 14% of the VM patients that needed a surgical procedure was prolonged by 7–9 months, that is, by 41%. Conclusions. Sclerotherapy is an effective method in the treatment of VMs with a satisfactory clinical response in patients symptoms in 84% of cases. PMID:28074157

  2. Functional MRI-guided microsurgery of intracranial arteriovenous malformations: study protocol for a randomised controlled trial.

    PubMed

    Zhao, Bing; Cao, Yong; Zhao, Yuanli; Wu, Jun; Wang, Shuo

    2014-10-23

    Intracranial arteriovenous malformations (AVMs) are associated with high morbidity and mortality. Modern microsurgery has improved the results of surgical treatment of AVMs; however, the treatment of AVMs, particularly eloquently located AVMs, still carries a high risk. Functional MRI (fMRI) has been reported to be used for the preoperative evaluation of AVMs in small case series. The purpose is to identify the utility and efficacy of fMRI-guided microsurgery of AVMs in a large randomised controlled trial. The study is a prospective, randomised controlled clinical trial. This study will enrol a total of 600 eligible patients. These eligible patients will be randomised to the standard microsurgery group and the fMRI-guided microsurgery group in a 1:1 ratio. Patient baseline characteristics and AVM architecture and characteristics will be described. In the fMRI-guided group, fMRI mapping of an eloquent cortex in all AVMs will be identified. Surgical complications and outcomes at pretreatment, post-treatment, at discharge and at 1-month, 3-month and 6-month follow-up intervals will be analysed using the modified Rankin Scale (mRS). This trial will determine whether fMRI-guided microsurgery could improve outcomes in patients with AVMs and also identify the safety and efficacy of fMRI-guided microsurgery. The study protocol and written informed consent were reviewed and approved by the Institutional Review Board of Beijing Tiantan Hospital Affiliated to Capital Medical University (ky2012-016-02). Study findings will be disseminated in the printed media. ClinicalTrials.gov NCT01758211. 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.

  3. Compression therapy for congenital low-flow vascular malformations of the extremities: A systematic review.

    PubMed

    Langbroek, Ginger B; Horbach, Sophie Er; van der Vleuten, Carine Jm; Ubbink, Dirk T; van der Horst, Chantal Mam

    2016-12-19

    Introduction Low-flow vascular malformations are congenital abnormalities of the veins, capillaries or lymphatic vessels or a combination of the previous. Compressive garments are frequently used as a first-line treatment option for low-flow vascular malformations of the extremities with the purpose of relieving symptoms. Yet, the benefits and harms of compression stockings remain unclear. Methods A systematic search was performed in MEDLINE, Embase, Cochrane Central Register of Controlled Trials including a hand search for studies measuring the benefits and harms of compression garments in treating low-flow vascular malformations. Two investigators performed study selection, quality assessment and data extraction independently. Results Of the 565 studies found, eventually five (totalling 101 patients with venous malformations or Klippel-Trenaunay syndrome receiving compression therapy) observational studies were included in the systematic review. Although the overall quality of these studies was poor, results suggest that compression garments might lessen intravascular coagulation, improve symptoms and appearance, diminish oedema, and protect against minor trauma. None of the studies quantified any harms of compression therapy. Conclusion Even though compression therapy is commonly used in the treatment of low-flow vascular malformations, available literature does not provide high-quality evidence to validate its use. We therefore advocate the need for prospective comparative trials with standardised outcome measures to study the benefits and harms of this treatment option.

  4. Foot Drop after Ethanol Embolization of Calf Vascular Malformation: A Lesson on Nerve Injury

    SciTech Connect

    Tay, Vincent Khwee-Soon; Mohan, P. Chandra; Liew, Wendy Kein Meng; Mahadev, Arjandas; Tay, Kiang Hiong

    2013-08-01

    Ethanol is often used in sclerotherapy to treat vascular malformations. Nerve injury is a known complication of this procedure. However, the management of this complication is not well described in literature. This case describes a 10-year-old boy with a slow flow vascular malformation in the right calf who underwent transarterial ethanol embolization following prior unsuccessful direct percutaneous sclerotherapy. The development of a dense foot drop that subsequently recovered is described, and the management of this uncommon but distressful complication is discussed.

  5. Cerebral cavernous malformations as a disease of vascular permeability: from bench to bedside with caution.

    PubMed

    Yadla, Sanjay; Jabbour, Pascal M; Shenkar, Robert; Shi, Changbin; Campbell, Peter G; Awad, Issam A

    2010-09-01

    Tremendous insight into the molecular and genetic pathogenesis of cerebral cavernous malformations (CCMs) has been gained over the past 2 decades. This includes the identification of 3 distinct genes involved in familial CCMs. Still, a number of unanswered questions regarding the process from gene mutation to vascular malformation remain. It is becoming more evident that the disruption of interendothelial junctions and ensuing vascular hyperpermeability play a principal role. The purpose of this review is to summarize the current understanding of CCM genes, associated proteins, and functional pathways. Promising molecular and genetic therapies targeted at identified molecular aberrations are discussed as well.

  6. Foot drop after ethanol embolization of calf vascular malformation: a lesson on nerve injury.

    PubMed

    Tay, Vincent Khwee-Soon; Mohan, P Chandra; Liew, Wendy Kein Meng; Mahadev, Arjandas; Tay, Kiang Hiong

    2013-08-01

    Ethanol is often used in sclerotherapy to treat vascular malformations. Nerve injury is a known complication of this procedure. However, the management of this complication is not well described in literature. This case describes a 10-year-old boy with a slow flow vascular malformation in the right calf who underwent transarterial ethanol embolization following prior unsuccessful direct percutaneous sclerotherapy. The development of a dense foot drop that subsequently recovered is described, and the management of this uncommon but distressful complication is discussed.

  7. A long-Segmental Vascular Malformation in the Small Bowel Presenting With Gastrointestinal Bleeding in a Preschool-Aged Child

    PubMed Central

    Lee, Yeoun Joo; Hwang, Jae-Yeon; Cho, Yong Hoon; Kim, Yong-Woo; Kim, Tae Un; Shin, Dong Hoon

    2016-01-01

    Gastrointestinal (GI) bleeding in pediatric patients has several causes. Vascular malformation of the small bowel is a rare disease leading to pediatric GI bleeding. To our knowledge, few reports describe ultrasound and computed tomography findings of venous malformations involving the small bowel. We present a case of long-segmental and circumferential vascular malformation that led to GI bleeding in a pre-school aged child, focusing on the radiologic findings. Although vascular malformation including of the GI tract is rare in children, it should be considered when GI bleeding occurs in pediatric patients. PMID:27110342

  8. Chronic daily headache in the adults: differential diagnosis between symptomatic Chiari I malformation and spontaneous intracranial hypotension.

    PubMed

    Mea, Eliana; Chiapparini, Luisa; Leone, Massimo; Franzini, Angelo; Messina, Giuseppe; Bussone, Gennaro

    2011-12-01

    This article briefly reviews the spectrum of headaches associated with Chiari type I malformation (CMI) and specifically analyzes the current data on the possibility of this malformation as an etiology for some cases of chronic daily headache (CDH). CMI is definitely associated with cough headache and not with primary episodic headaches, with the rare exception of basilar migraine-like cases. With regard to CDH, there is no clear evidence supporting an association with CMI. A magnetic resonance imaging (MRI) study would be justified only in patients showing either a Valsalva-aggravating component or cervicogenic features. Hydrocephalus and low-intracranial pressure syndrome should be ruled out in patients showing tonsillar herniation in an MRI study and consulting due to daily headache.

  9. beta-amyloid protein of Alzheimer's disease is found in cerebral and spinal cord vascular malformations.

    PubMed Central

    Hart, M. N.; Merz, P.; Bennett-Gray, J.; Menezes, A. H.; Goeken, J. A.; Schelper, R. L.; Wisniewski, H. M.

    1988-01-01

    Congo/Red deposition with birefringence to polarized light was demonstrated focally in cerebrovascular malformations removed surgically from 4 older patients (ages 85, 74, 74, and 63), and in a spinal cord vascular malformation in a 76-year-old patient. Lesser degrees of Congophilic change were observed in cerebrovascular malformations screened from 4 of 10 patients between the ages of 30 and 59. No Congophilic change was seen in 10 cerebrovascular malformations removed from patients under 30 years of age. Congophilic areas in all cases decorated with W-2 and 85/45 polyclonal antibodies raised to peptide sequences of cerebrovascular beta-amyloid and beta-amyloid of senile plaques from patients with Alzheimer's disease. Thus, the amyloid in these vascular malformations is immunologically related to beta-amyloid protein. This finding provides another indication that vascular beta-amyloid deposition is not specific for Alzheimer's disease and suggests that an existing abnormality of vessels may be a predisposing factor. Images Figure 1 Figure 2A Figure 2B Figure 3 Figure 4 PMID:3293463

  10. Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: case report.

    PubMed

    Rosenow, J; Rawanduzy, A; Weitzner, I; Couldwell, W T

    2000-05-01

    Type IVc arteriovenous malformations (AVMs) of the spinal cord consist of multiple high-flow feeding vessels, and they often present a challenging management situation. Their location is intradural and extramedullary, and they are rare malformations that are difficult to treat owing to the risk of thrombosis of the anterior spinal artery. The authors report a case of Type IVc spinal AVM in a patient with a family history of three siblings with pulmonary AVMs. Spinal AVMs have been reported to be associated with inherited syndromes such as familial cutaneous hemangiomas and Kartagener's syndrome, but an association with pulmonary AVMs has not previously been described. A 27-year-old man presented with sudden onset of occipital headache with cervical radiation while weightlifting. Results of computed tomography of the brain were normal, but lumbar puncture revealed a subarachnoid hemorrhage. The patient had a 1-year history of a neurogenic bladder and exhibited marked left calf muscle wasting. The patient underwent spinal magnetic resonance imaging, which revealed the AVM in the conus region. Selective spinal angiography was performed for diagnostic purposes. A laminectomy was performed, and the vessels feeding the AVM were clipped, as was the fistula. The patient remained neurologically stable, and angiography confirmed obliteration of the AVM. This is the first case report of a patient with a spinal AVM who had multiple siblings with pulmonary malformations or AVMs.

  11. Thermographic Assessment of a Vascular Malformation of the Hand: A New Imaging Modality

    PubMed Central

    Hardwicke, Joseph T.; Titley, O. Garth

    2016-01-01

    Vascular malformations of the hand are rare. Angiography is the current Gold Standard imaging modality. Thermal imaging is an emerging noninvasive, noncontact technology that does not require intravenous contrast agents. We present the case of a patient with an arteriovenous malformation affecting the hand in which thermal imaging has been used as an adjunct to capture baseline images to allow monitoring of progression. We suggest that thermal imaging provides an adjunct that can be used in addition to clinical examination and/or angiography for the diagnosis and routine follow-up of conservatively managed arteriovenous malformations, to monitor progression or vascular steal, and also for recording recurrence after surgical excision for which there is known to be a significant incidence. With the benefit of being a noninvasive imaging modality that does not require intravenous contrast, or ionizing radiation exposure, office-based thermal imaging may become commonplace. PMID:27195175

  12. Time-resolved contrast-enhanced MR angiography of spinal vascular malformations.

    PubMed

    Amarouche, M; Hart, J L; Siddiqui, A; Hampton, T; Walsh, D C

    2015-02-01

    The diagnosis of spinal vascular malformations may be challenging on conventional MR imaging because neither the location of the signal abnormality in the spinal cord nor the level of the abnormal flow voids correlates with the level of the fistula. We conducted a retrospective evaluation of the utility of using a time-resolved imaging of contrast kinetics sequence in the diagnosis, characterization, and localization of spinal vascular malformations, comparing it with the criterion standard of spinal DSA. Fifty-five consecutive patients with a suspected diagnosis of spinal vascular malformation underwent time-resolved imaging of contrast kinetics followed by spinal DSA. All scans were performed on a 1.5T scanner by using a standard 8-channel spine coil and were reported by a neuroradiologist before the DSA was performed. Forty-seven lesions were confirmed on time-resolved imaging of contrast kinetics and classified as spinal dural arteriovenous fistulas (n = 33, with 1 patient having a type Ib fistula), perimedullary spinal cord arteriovenous fistulas (n = 10), and intramedullary arteriovenous malformations (n = 3). One patient had an extradural spinal vascular malformation. Time-resolved imaging of contrast kinetics identified the location of the arterial feeder to within 1 vertebral level in 27/33 patients (81.8%) with spinal dural arteriovenous fistulas and correctly predicted the side in 22/33 (66.6%) patients. Perimedullary spinal cord arteriovenous fistulas were erroneously considered to represent spinal dural arteriovenous fistulas before spinal DSA. The anatomy of the arterial supply to intramedullary arteriovenous malformations was also poorly characterized on time-resolved contrast-enhanced MR angiography. It has been our experience that time-resolved imaging of contrast kinetics is a useful confirmatory tool when a spinal vascular malformation is suspected on the basis of clinical and conventional MR imaging findings. As experience with the technique

  13. How do the type and location of a vascular malformation influence growth in Klippel-Trénaunay syndrome?

    PubMed

    Funayama, Emi; Sasaki, Satoru; Oyama, Akihiko; Furukawa, Hiroshi; Hayashi, Toshihiko; Yamamoto, Yuhei

    2011-01-01

    Although Klippel-Trénaunay syndrome is a mixed vascular malformation characterized by abnormal growth in the extremities, no uniform diagnostic criteria have been established because of the variety in its manifestation. Consequently, no anatomical analysis based on a comparison study has been reported. In this study, the authors determine the frequency of various vascular malformations and abnormal growth and assess any statistical relationship between vascular malformation type/location and abnormal growth in terms of length and girth. Thirty-five patients with Klippel-Trénaunay syndrome satisfying the criteria proposed by Oduber et al. in 2008 were enrolled. The type and location of the vascular malformation and abnormal circumferential growth were assessed by magnetic resonance imaging and ultrasonography. Bone girth was assessed by axial magnetic resonance imaging/computed tomography. Plain radiographs of the long bones were used to measure growth in length. The spectrum of vascular types was similar to that in previous reports. There was no significant association between leg length and vascular malformation type or location. Leg bone circumferential hypoplasia was observed in 50 percent of cases and was significantly related to the presence of intramuscular lesions. A single venous malformation in the subcutaneous tissue was significantly associated with the presence of subcutaneous hypertrophy. Patients with intramuscular lymphatic malformations had a significantly higher frequency of muscle hypoplasia. The type and location of certain vascular malformations were significantly associated with abnormal subcutaneous tissue, muscle, and bone growth.

  14. [Vascular malformations in the Williams-Beuren syndrome: report of three new cases].

    PubMed

    Sator, Hicham; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Rhouni, Fatima Ezzahra; Benjouad, Ibitihale; Dafiri, Rachida; Chat, Latifa

    2016-01-01

    The Williams-Beuren syndrome is a rare genetic disease. It combines classically specific facial dysmorphism, cardiovascular malformations and specific neuropsychological profile. We report three cases of Williams-Beuren syndrome in children with particular emphasis on vascular abnormalities observed on CT angiography and MR angiography.

  15. RGB imaging system for monitoring of skin vascular malformation's laser therapy

    NASA Astrophysics Data System (ADS)

    Jakovels, Dainis; Kuzmina, Ilona; Berzina, Anna; Spigulis, Janis

    2012-06-01

    A prototype RGB imaging system for mapping of skin chromophores consists of a commercial RGB CMOS sensor, RGB LEDs ring-light illuminator and orthogonally orientated polarizers for reducing specular reflectance. The system was used for monitoring of vascular malformations (hemagiomas and telangiectasias) therapy.

  16. [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.

  17. Intracranial Hemorrhage

    PubMed Central

    2011-01-01

    Intracranial hemorrhage is a life-threatening condition, the outcome of which can be improved by intensive care. Intracranial hemorrhage may be spontaneous, precipitated by an underlying vascular malformation, induced by trauma, or related to therapeutic anticoagulation. The goals of critical care are to assess the proximate cause, minimize the risks of hemorrhage expansion through blood pressure control and correction of coagulopathy, and obliterate vascular lesions with a high risk of acute rebleeding. Simple bedside scales and interpretation of computed tomography scans assess the severity of neurological injury. Myocardial stunning and pulmonary edema related to neurological injury should be anticipated, and can usually be managed. Fever (often not from infection) is common and can be effectively treated, although therapeutic cooling has not been shown to improve outcomes after intracranial hemorrhage. Most functional and cognitive recovery takes place weeks to months after discharge; expected levels of functional independence (no disability, disability but independence with a device, dependence) may guide conversations with patient representatives. Goals of care impact mortality, with do-not-resuscitate status increasing the predicted mortality for any level of severity of intraparenchymal hemorrhage. Future directions include refining the use of bedside neuromonitoring (electroencephalogram, invasive monitors), novel approaches to reduce intracranial hemorrhage expansion, minimizing vasospasm, and refining the assessment of quality of life to guide rehabilitation and therapy. PMID:22167847

  18. 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

  19. MR imaging of soft-tissue vascular malformations: diagnosis, classification, and therapy follow-up.

    PubMed

    Flors, Lucía; Leiva-Salinas, Carlos; Maged, Ismaeel M; Norton, Patrick T; Matsumoto, Alan H; Angle, John F; Hugo Bonatti, Md; Park, Auh Whan; Ahmad, Ehab Ali; Bozlar, Ugur; Housseini, Ahmed M; Huerta, Thomas E; Hagspiel, Klaus D

    2011-01-01

    Vascular malformations and tumors comprise a wide, heterogeneous spectrum of lesions that often represent a diagnostic and therapeutic challenge. Frequent use of an inaccurate nomenclature has led to considerable confusion. Since the treatment strategy depends on the type of vascular anomaly, correct diagnosis and classification are crucial. Magnetic resonance (MR) imaging is the most valuable modality for classification of vascular anomalies because it accurately demonstrates their extension and their anatomic relationship to adjacent structures. A comprehensive assessment of vascular anomalies requires functional analysis of the involved vessels. Dynamic time-resolved contrast material-enhanced MR angiography provides information about the hemodynamics of vascular anomalies and allows differentiation of high-flow and low-flow vascular malformations. Furthermore, MR imaging is useful in assessment of treatment success and establishment of a long-term management strategy. Radiologists should be familiar with the clinical and MR imaging features that aid in diagnosis of vascular anomalies and their proper classification. Furthermore, they should be familiar with MR imaging protocols optimized for evaluation of vascular anomalies and with their posttreatment appearances. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.315105213/-/DC1.

  20. Angiography of Hepatic Vascular Malformations Associated with Hereditary Hemorrhagic Telangiectasia

    SciTech Connect

    Hashimoto, Manabu Tate, Etuko; Nishii, Toshiaki; Watarai, Jiro; Shioya, Takanobu; White, Robert I.

    2003-04-15

    Purpose: To describe the angiographic features of hepatic involvement in hereditary hemorrhagictelangiectasia (HHT), particularly the presence of portovenous shunts. Methods: We reviewed the angiographic findings of seven patients with HHT. The patients comprised three women and four men with a mean age of 51 years. Results: Intrahepatictelangiectasias were found in all seven patients and shunts between three vascular channels were found in six of seven patients. In the four patients who had portovenous shunts combined with arterioportal shunts, the portovenous shunts were large. Three patients had noportovenous shunts. Two of these patients had arteriovenous shunts, and one had no shunt. The mean age (69 years) of the patients with portovenous shunts was older than those without portovenous shunts (26 years). Conclusions: Hepatic vascular lesions in HHT are varied, ranging from telangiectasias to large shunts between three vascular channels. In an advanced stage of involvement, large portovenous shunts are present.

  1. [Diagnosis of uterine vascular malformation using Doppler ultrasound].

    PubMed

    Deckner, C; Schiesser, M; Bastert, G

    2004-04-01

    We present the case of a 56-year-old woman, who was admitted to our clinic for diagnostic laparoscopy because of a cystic uterine tumour of uncertain dignity. In the patient's history three curettages due to recurrent acyclic premenopausal vaginal bleeding were reported without specific histological findings. The preceding MRI described the structure as a myoma. During preoperative diagnostics an arteriovenous malformation was suspected by transvaginal Doppler sonography. Consequently the procedure was changed and a laparotomy performed. The sonographic findings were confirmed during surgery and by histological examination. This case points out the important role of transvaginal sonography combined with colour-flow-mapping. By confirming the diagnosis preoperatively and changing the management a low-risk procedure could be ensured.

  2. Imaging Spectrum of Hemangioma and Vascular Malformations of the Head and Neck in Children and Adolescents

    PubMed Central

    Bhat, Venkatraman; Salins, Paul C; Bhat, Varun

    2014-01-01

    Vascular lesions of the head and neck region in children constitute an interesting group of lesions that benefit immensely from imaging techniques. Imaging is essential for identification, characterization, and delineation of the extent of lesion and subsequent follow-up. Infantile hemangiomas, which are vascular tumors with a specific evolution pattern, constitute a large majority of these lesions. On the other hand, there are vascular malformations, which are anomalies of the vascular system, consisting of a range of vascular tissues associated with various flow patterns. When diagnosis is clinically evident, imaging should utilize non-radiation techniques and address the issues necessary for management. Timing and interpretation of imaging methods employed in assessing childhood vascular lesion should also take into consideration the natural history so that imaging is performed to address a specific question. This review highlights the typical appearance of a hemangioma and a group of vascular malformations of the head and neck. For descriptive purpose, an attempt has been made to group lesions into specific subsites, with each one having specific clinical significance. Cases included illustrate the spectrum of the disease ranging from classical form in young children to slightly differing manifestations of the disease in adolescents and adults. The illustrations also provide a novel way of presenting image data using volume-rendering techniques of 3D data. Multi-modality team interaction and management strategies of these complex lesions are also emphasized. PMID:25161800

  3. Novel Application of Postmortem CT Angiography for Evaluation of the Intracranial Vascular Anatomy in Cadaver Heads.

    PubMed

    van Eijk, Ruben P A; van der Zwan, Albert; Bleys, Ronald L A W; Regli, Luca; Esposito, Giuseppe

    2015-12-01

    Postmortem CT angiography is a common procedure used to visualize the entire human vasculature. For visualization of a specific organ's vascular anatomy, casting is the preferred method. Because of the permanent and damaging nature of casting, the organ cannot be further used as an experimental model after angiography. Therefore, there is a need for a minimally traumatic method to visualize organ-specific vascular anatomy. The purpose of this study was to develop and evaluate a contrast enhancement technique that is capable of visualizing the intracranial vascular anatomy while preserving the anatomic integrity in cadaver heads. Seven human heads were used in this study. Heads were prepared by cannulating the vertebral and internal carotid arteries. Contrast agent was injected as a mixture of tap water, polyethylene glycol 600, and an iodinated contrast agent. Postmortem imaging was executed on a 64-MDCT scanner. Primary image review and 3D reconstruction were performed on a CT workstation. Clear visualization of the major cerebral arteries and smaller intracranial branches was achieved. Adequate visualization was obtained for both the anterior and posterior intracranial circulation. The minimally traumatic angiography method preserved the vascular integrity of the cadaver heads. A novel application of postmortem CT angiography is presented here. The technique can be used for radiologic evaluation of the intracranial circulation in cadaver heads. After CT angiography, the specimen can be used for further experimental or laboratory testing and teaching purposes.

  4. Multiple cavernous malformations with supravermian arachnoid cyst.

    PubMed

    Unalp, Aycan; Uran, Nedret

    2007-11-01

    Cerebral cavernous malformation are congenital vascular abnormalities that have been reported in 0.4% of the population; they represent 5-13% of all cerebrovascular malformations. Onset of cerebral cavernous malformations may be associated with seizures, intracranial hemorrhages, focal neurological deficit or migraine-type headaches. Some patients may require surgical intervention due to hemorrhage. Multiple cavernomas in childhood have been reported in the literature, but they are rare. This manuscript presents a 12-year-old girl with multiple cavernomas accompanied by supravermian arachnoid cyst detected by neuroimaging techniques. This is the first report that demonstrates a case of pediatric multiple cavernous malformation coexisting with arachnoid cyst of the supravermian cistern.

  5. Diffuse capillary malformation with overgrowth: a clinical subtype of vascular anomalies with hypertrophy.

    PubMed

    Lee, Margaret S; Liang, Marilyn G; Mulliken, John B

    2013-10-01

    Categorization of vascular anomalies with overgrowth is evolving rapidly with the aid of massively parallel genomic sequencing; however, accurate clinical diagnosis is still essential. We identified a group of patients with an extensive, diffuse, reticulate capillary malformation (CM) and variable hypertrophy without major complications. We sought to study a subset of patients with diffuse CM to better define prognosis and management. Chart review identified 73 patients with diffuse CM who did not fit the criteria for known disorders with CM and/or overgrowth. Soft-tissue or bony overgrowth did not correlate with location, morphology, or intensity of the vascular stain. Patients required periodic follow-up to monitor for leg length discrepancy. They were found to exhibit normal neurologic development and proportionate overgrowth rather than progressive, disproportionate asymmetry or vascular complications. This retrospective review was limited to observations documented at clinic visits; these patients require long-term assessment. Further studies are necessary to accurately assess Wilms tumor risk and clinical outcomes in older adults. We propose the term "diffuse capillary malformation with overgrowth" to designate this extensive reticular vascular staining with proportionate overgrowth. We differentiate diffuse capillary malformation with overgrowth from other disorders with CM and hypertrophy. Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

  6. Percutaneous and combined percutaneous and intralesional Nd:YAG-laser therapy for vascular malformations.

    PubMed

    Wimmershoff, M B; Landthaler, M; Hohenleutner, U

    1999-01-01

    The numerous types of vascular abnormality are classified in groups according to their pathological and anatomical features. We present case histories of 2 patients who had vascular malformations of the face since birth or early childhood. Application methods, side-effects and complications of percutaneous and intra-lesional Nd:YAG-laser therapy are reviewed for these patients. A 54-year-old woman was treated percutaneously with the Nd: YAG-laser at 1064 nm, with 20 30 W, cw 1-5 s pulses and 2 - 3 mm spot size. A 59-year-old woman was treated with the combined percutaneous and intralesional laser therapy with 30 W, cw 1-5 s pulses and 2-3 mm spot size. In both cases, percutaneous or combined percutaneous and intra-lesional Nd: YAG-laser application resulted in a significant shrinking of the lesion. The Nd:YAG-laser radiation at 1064 nm presents an effective treatment of vascular malformations due to its deep penetration into the tissue. No standardized guidelines for Nd: YAG-laser therapy exist and the treatment parameters should be chosen individually according to the type of vascular malformation.

  7. Smell and taste in patients with vascular malformation of the extracranial head and neck region.

    PubMed

    Steinbach, Silke; Fasunla, Ayotunde J; Lahme, Carolin M E; Schäfers, Sophia P; Hundt, Walter; Wolf, Petra; Mandic, Robert; Werner, Jochen A; Eivazi, Behfar

    2014-01-01

    Olfactory and gustatory functions have not been investigated in patients with vascular malformation of the extracranial head and neck region with validated smell and taste tests. Although olfactory and gustatory deficiencies are often not outwardly apparent, they substantially affect daily life. Smell and taste tests using sniffin sticks and taste strips were administered in 40 patients. For all age groups and both sexes, odor threshold (THR) values were, on average, lower in patients than in healthy individuals; whereas, values of odor identification and discrimination were not significantly lower. Regarding odor THR, 33 (82.5%) patients were hyposmic. Taste values (sweet, sour, salty, bitter, and total taste) were, on average, lower in patients than in healthy individuals; 21 (52.5%) patients were hypogeusic. Disease duration did not correlate with smell and taste test values. Patients with and without tongue involvement had decreased odor threshold and taste values. No significant differences were identified when taste values on the left and right sides of the tongue were compared in patients without tongue involvement and with unilateral and bilateral tongue involvement. Patients with venous malformations had lower smell test values, and patients with lymphatic malformations had lower taste test values than patients with other malformations. Patients exhibit significantly reduced olfactory and gustatory function even when the nose and/or tongue are not malformed. Patients should be tested with validated smell and taste tests to adequately inform and advise them about overcoming smell and taste deficits.

  8. Cerebral vasospasm. Part I. In cerebral vascular malformations.

    PubMed

    Mohr, J P; Kase, C S

    1983-01-01

    This review enumerates the many proposed mechanisms of vasospasm, including cellular elements, agents derived from the blood and injured cerebral tissues, alteration of calcium: magnesium ratios, free radical reactions, hypothalamic injury, clogging of the subarachnoid space, obstructions of the vasa vasorum and necrosis of the media with subintimal proliferation and intraluminal acidosis. At present, no single agent has been demonstrated as the only source of vasospasm, and whether the disorder is spasm or a chronic arteriopathy remains the subject of argument. The factors influencing the frequency, timing, severity and distribution of angiographically documented vasospasm are discussed, including data from our own population-based study over a 3 year period showing an incidence of vasospasm of 73%. Special emphasis is given to the observation that differences in patient populations play a major role in the incidence and severity of reported vasospasm: those from non-selective populations show a higher incidence of vasospasm and a greater severity of the syndromes attributed to spasm. Hypotheses are offered to account for the low frequency of vasospasm in hemorrhages from arteriovenous malformations and mycotic aneurysms. Clinical syndromes of vasospasm are reviewed, with special emphasis on our own material. The mode of onset and subsequent course of syndromes include those of sudden onset consistent with embolism, and those of gradual onset suggesting a low flow state. Their relationship to the severity of the subarachnoid hemorrhage and the vasospasm is presented. The paucity of syndromes of isolated deep infarcts of the lacunar type is noted. An account is given of the many failed therapies and the future hopes for early surgery. Innovations in medical therapy, including the use of some platelet inhibitors available only in some countries, and rationales for the use of aspirin and even heparin is discussed.

  9. [Intestinal venous vascular malformation: Unusual etiology of gastrointestinal bleeding in pediatrics. Case report].

    PubMed

    Ninomiya, Inés S; Steimberg, Clarisa; Udaquiola, Julia; González, Lucio; Liberto, Daniel; Cieri, Patricio; Peralta, Oscar; Orsi, Marina

    2016-06-01

    Intestinal vascular malformations, especially those in the right colon, are a frequent cause of lower gastrointestinal bleeding in adults, but they are a very rare condition in children. Symptoms include acute hemorrhage, intestinal obstruction, or chronic anemia of uncertain etiology, which is the most frequent form of presentation but the most difficult to diagnose and thus properly treat. We report the case of an 11 year old boy admitted to the Emergency Room with abdominal pain, vomits, hemodynamic decompensation, who required expansion and blood transfusion. With history ofrecurrent bloody stools since infancy with repeated normal endoscopies and Tc99 scintigraphy with chronic anemia and no improvement despite adequate treatment. In the last admission, the videocolonoscopy detected a venous vascular malformation in the ileocecal region. The angiography and the entero multislice computer tomography scanner were valuable tools to confirm the diagnosis and to select the appropriate surgical procedure for this rare condition. Sociedad Argentina de Pediatría.

  10. Percutaneous Treatment of Peripheral Vascular Malformations in Children: Long-Term Clinical Outcome

    SciTech Connect

    Linden, Edwin van der; Otoide-Vree, Marleen; Pattynama, Peter M. T.

    2012-04-15

    Purpose: This study was designed to assess the rate of complications and clinical failure at 3 and 12 months after percutaneous treatment of vascular malformations in children. Furthermore, we describe patient satisfaction of treatment results during 5 years of follow-up. Methods: In a retrospective cohort study, we evaluated 26 patients younger than aged 19 years who were treated for symptomatic vascular malformations. Data on treatment outcomes and patient satisfactions were obtained with a precoded structured questionnaire. Patient files and imaging data were retrieved to obtain information regarding the vascular malformations and treatment. Clinical success was defined as disappearance or partial improvement of the complaints. Patient satisfaction was declared whenever patients answered in the questionnaire that they were satisfied with the treatment results. Results: Of 26 eligible patients, we included 23 (88%). The mean follow-up was 36 (range, 15-127) months. Posttreatment, 87% (20/23; 95% confidence interval (CI), 66-97%) of patients reported clinical success at 3 months. At 1, 2, 3, 4, and 5 years of follow-up this percentage was 74%, 59%, 59%, 59%, and 59%, respectively. Eleven (48%, 95% CI 27-69%) patients had experienced complications and 22% (95% CI 7-44%) had major complications, of which 5 had required additional treatment. In all, 83% (19/23) of the patients reported satisfaction with the treatment. Conclusions: Percutaneous treatment of vascular malformations improved clinical symptoms in 87% of the patients at 3 months and were sustainable for half of all patients during a 5-year follow-up period. However, major complications were seen in 22%.

  11. Focal congenital lipoatrophy and vascular malformation: a mild form of inverse Klippel-Trenaunay syndrome?

    PubMed

    Cappuccio, Gerarda; Brunetti-Pierri, Nicola

    2012-12-01

    A 2 year-and seven month-old male boy with vascular malformation and localized soft tissue hypotrophy is reported. Such presentation could represent the mildest end of the spectrum of 'inverse' Klippel-Trenaunay syndrome, a recently proposed disorder presenting with tissue hypotrophy as opposed to partial or complete limb overgrowth of classic Klippel-Trenaunay syndrome. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  12. Role of endovascular embolization for trigeminal neuralgia related to cerebral vascular malformation.

    PubMed

    Ge, Huijian; Lv, Xianli; Jin, Hengwei; He, Hongwei; Li, Youxiang

    2016-10-01

    The objective of this article is to describe the trigeminal neuralgia related to cerebral vascular malformation that is rarely reported and the experience referring to endovascular treatment. A total of 10 patients who had cerebral vascular malformation (AVM and dAVF) in a single center presented with trigeminal neuralgia. Clinical and angiographic presentations as well as their clinical outcomes after embolization were reviewed. Of the 10 cases, seven dAVFs and three AVMs were detected. In contrast to the dilated feeding arteries, an ectasia of the draining vein that is adjacent to the root entry zone of the trigeminal nerve such as the petrosal vein and lateral mesencephalic vein has the major role in causing the trigeminal neuralgia. All of these patients had relief of facial pain after endovascular embolization during follow-up (mean 57.3 months, range 5 to 100 months). There were no permanent neurological deficits. Endovascular embolization is an effective method in treating trigeminal neuralgia related to cerebral vascular malformation. © The Author(s) 2016.

  13. Spontaneous thrombosis of vein of Galen malformation

    PubMed Central

    Kariyappa, Kalpana Devi; Krishnaswami, Murali; Gnanaprakasam, Francis; Ramachandran, Madan; Krishnaswamy, Visvanathan

    2016-01-01

    Vein of Galen malformation (VOGM) is a rare intracranial vascular malformation. Before the advent and advancement of various endovascular techniques, there was dismal prognosis. Rarely, this condition may spontaneously thrombose without the need for surgical or endovascular treatment with good prognosis. We report a case of an 8-month-old infant who had serial imaging, suggestive of VOGM and presented to us for further management, wherein the imaging revealed spontaneous thrombosis. PMID:27857804

  14. [State of the art of classification, diagnostics and therapy for cervicofacial hemangiomas and vascular malformations].

    PubMed

    Werner, J A; Eivazi, B; Folz, B J; Dünne, A-A

    2006-12-01

    The successful treatment of vascular anomalies depends on profound knowledge of the biologic behavior of vascular lesions and their correct classification. On the base of the clinical course Mulliken and Glowacki developed a biologic classification that was accepted as official classification by the ISSVA (International Society for the Study of Vascular Anomalies). Based on an extended literature research, this manuscript will give an overview of different internationally accepted treatment concepts. Even if a wait-and-see strategy can be recommended in many cases of uneventful hemangiomas in infants the proliferative growth of such lesions requires an adequate treatment indication. Vascular malformations that persist lifelong require treatment in the majority of the cases, especially when clinical symptoms occur. Based on individual parameters such as the diameter, location or growth behavior, different therapeutic options as cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgical intervention and/or embolisation can be performed successfully. None of those treatment concepts, however, represents the only treatment method of choice.

  15. Noncontrast dynamic MRA in intracranial arteriovenous malformation (AVM), comparison with time of flight (TOF) and digital subtraction angiography (DSA).

    PubMed

    Yu, Songlin; Yan, Lirong; Yao, Yuqiang; Wang, Shuo; Yang, Mingqi; Wang, Bo; Zhuo, Yan; Ai, Lin; Miao, Xinyuan; Zhao, Jizong; Wang, Danny J J

    2012-07-01

    Digital subtraction angiography (DSA) remains the gold standard to diagnose intracranial arteriovenous malformations (AVMs) but is invasive. Existing magnetic resonance angiography (MRA) is suboptimal for assessing the hemodynamics of AVMs. The objective of this study was to evaluate the clinical utility of a novel noncontrast four-dimensional (4D) dynamic MRA (dMRA) in the evaluation of intracranial AVMs through comparison with DSA and time-of-flight (TOF) MRA. Nineteen patients (12 women, mean age 26.2±10.7 years) with intracranial AVMs were examined with 4D dMRA, TOF and DSA. Spetzler-Martin grading scale was evaluated using each of the above three methods independently by two raters. Diagnostic confidence scores for three components of AVMs (feeding artery, nidus and draining vein) were also rated. Kendall's coefficient of concordance was calculated to evaluate the reliability between two raters within each modality (dMRA, TOF, TOF plus dMRA). The Wilcoxon signed-rank test was applied to compare the diagnostic confidence scores between each pair of the three modalities. dMRA was able to detect 16 out of 19 AVMs, and the ratings of AVM size and location matched those of DSA. The diagnostic confidence scores by dMRA were adequate for nidus (3.5/5), moderate for feeding arteries (2.5/5) and poor for draining veins (1.5/5). The hemodynamic information provided by dMRA improved diagnostic confidence scores by TOF MRA. As a completely noninvasive method, 4D dMRA offers hemodynamic information with a temporal resolution of 50-100 ms for the evaluation of AVMs and can complement existing methods such as DSA and TOF MRA. Copyright © 2012 Elsevier Inc. All rights reserved.

  16. Stabiliztin of VEGFR2 Signaling by Cerebral Cavernous Malformation 3 is Critical for Vascular Development

    SciTech Connect

    Y He; H Zhang; L Yu; M Gunel; T Boggon; H Chen; W Min

    2011-12-31

    Cerebral cavernous malformations (CCMs) are human vascular malformations caused by mutations in three genes of unknown function: CCM1, CCM2, and CCM3. CCM3, also known as PDCD10 (programmed cell death 10), was initially identified as a messenger RNA whose abundance was induced by apoptotic stimuli in vitro. However, the in vivo function of CCM3 has not been determined. Here, we describe mice with a deletion of the CCM3 gene either ubiquitously or specifically in the vascular endothelium, smooth muscle cells, or neurons. Mice with global or endothelial cell-specific deletion of CCM3 exhibited defects in embryonic angiogenesis and died at an early embryonic stage. CCM3 deletion reduced vascular endothelial growth factor receptor 2 (VEGFR2) signaling in embryos and endothelial cells. In response to VEGF stimulation, CCM3 was recruited to and stabilized VEGFR2, and the carboxyl-terminal domain of CCM3 was required for the stabilization of VEGFR2. Indeed, the CCM3 mutants found in human patients lacking the carboxyl-terminal domain were labile and were unable to stabilize and activate VEGFR2. These results demonstrate that CCM3 promotes VEGFR2 signaling during vascular development.

  17. Stabilization of VEGFR2 signaling by cerebral cavernous malformation 3 is critical for vascular development.

    PubMed

    He, Yun; Zhang, Haifeng; Yu, Luyang; Gunel, Murat; Boggon, Titus J; Chen, Hong; Min, Wang

    2010-04-06

    Cerebral cavernous malformations (CCMs) are human vascular malformations caused by mutations in three genes of unknown function: CCM1, CCM2, and CCM3. CCM3, also known as PDCD10 (programmed cell death 10), was initially identified as a messenger RNA whose abundance was induced by apoptotic stimuli in vitro. However, the in vivo function of CCM3 has not been determined. Here, we describe mice with a deletion of the CCM3 gene either ubiquitously or specifically in the vascular endothelium, smooth muscle cells, or neurons. Mice with global or endothelial cell-specific deletion of CCM3 exhibited defects in embryonic angiogenesis and died at an early embryonic stage. CCM3 deletion reduced vascular endothelial growth factor receptor 2 (VEGFR2) signaling in embryos and endothelial cells. In response to VEGF stimulation, CCM3 was recruited to and stabilized VEGFR2, and the carboxyl-terminal domain of CCM3 was required for the stabilization of VEGFR2. Indeed, the CCM3 mutants found in human patients lacking the carboxyl-terminal domain were labile and were unable to stabilize and activate VEGFR2. These results demonstrate that CCM3 promotes VEGFR2 signaling during vascular development.

  18. Coupling hemodynamics with vascular wall mechanics and mechanobiology to understand intracranial aneurysms

    PubMed Central

    Humphrey, J.D.

    2009-01-01

    Arteries exhibit a remarkable ability to adapt in response to sustained alterations in hemodynamic loading, to heal in response to injuries, and to compensate in response to diverse disease conditions. Nevertheless, such compensatory adaptations are limited and many vascular disorders, if untreated, lead to significant morbidity or mortality. Parallel advances in vascular biology, medical imaging, biomechanics, and computational methods promise to provide increased insight into many arterial diseases, including intracranial aneurysms. In particular, although it may be possible to identify useful clinical correlations between either the blood flow patterns within or the shape of aneurysms and their rupture-potential, our ultimate goal should be to couple studies of hemodynamics with those of wall mechanics and the underlying mechanobiology so that we can understand better the mechanisms by which aneurysms arise, enlarge, and rupture and thereby identify better methods of treatment. This paper presents one such approach to fluid-solid-growth (FSG) modeling of intracranial aneurysms. PMID:20526461

  19. Effects of physiotherapy combined with sirolimus in a patient with vascular malformation: A case report.

    PubMed

    Akbayrak, Türkan; Orhan, Ceren; Baran, Emine; Kaya, Serap; Coskun, Gürsoy; Varan, Ali

    2016-01-01

    The aim of the present case report was to investigate the effects of a physiotherapy program combined with sirolimus in a child patient with upper extremity edema and joint limitation due to low-flow vascular malformation. This case report included an 11-year-old male patient (26 kg, 130 cm) diagnosed with congenital lymphovascular malformation on the left and right chest. The patient, who had edema on the upper left extremity and experienced joint limitations, was administered complete decongestive therapy (CDT) and manual therapy in combination with sirolimus. Physiotherapy included a total of 24 sessions, 3 sessions a week for 8 weeks. Following the physiotherapy, the patient was assigned to a home therapy program, and then the maintenance phase of the CDT was initiated. Evaluations were carried out at baseline, at the end of week 8, and after 12 months. Following the physiotherapy program combined with sirolimus, a decrease in extremity volume, an increase in joint movement range, and an improvement in disease-related complaints were observed. Physiotherapy methods combined with sirolimus may be an effective treatment method in patients with vascular malformations. However, further studies with larger sample size are warranted.

  20. Toxicity of Gamma Knife Radiosurgery in the Treatment of Intracranial Tumors in Patients With Collagen Vascular Diseases or Multiple Sclerosis

    SciTech Connect

    Lowell, Dot; Tatter, Stephen B.; Bourland, J. Daniel; Guzman, Allan F. de; Ekstrand, Kenneth E.; Ellis, Thomas L.; Lovato, James F.; McMullen, Kevin P.; Munley, Michael T.; Shaw, Edward G.; Urbanic, James J.; Chan, Michael D.

    2011-11-15

    Purpose: To assess toxicity in patients with either a collagen vascular disease (CVD) or multiple sclerosis (MS) treated with intracranial radiosurgery. Methods and Materials: Between January 2004 and April 2009, 6 patients with MS and 14 patients with a CVD were treated with Gamma Knife radiosurgery (GKRS) for intracranial tumors. Treated lesions included 15 total brain metastases in 7 patients, 11 benign brain tumors, 1 low grade glioma, and 1 cavernous malformation. Toxicities were graded by the Radiation Therapy Oncology Group Acute/Late Radiation Morbidity Scoring Criteria. 'Rare toxicities' were characterized as those reported in the scientific literature at an incidence of <5%. Results: Median follow-up time was 16 months. Median dose to the tumor margin was 13.0 Gy (range, 12-21 Gy). Median size of tumor was 5.0 cm{sup 3} (range, 0.14-7.8 cm{sup 3}). Of the 14 patients with CVD, none experienced a Grade 3 or 4 toxicity or a toxicity characterized as rare. Of the 6 patients with MS, 3 experienced rare toxicities, and two of these were Grade 3 toxicities. Rare complications included a patient experiencing both communicating hydrocephalus and facial nerve palsy, as well as 2 additional patients with motor cranial nerve palsy. High-grade toxicities included the patient with an acoustic neuroma requiring ventriculoperitoneal shunt placement for obstructive hydrocephalus, and 1 patient with a facial nerve schwannoma who experienced permanent facial nerve palsy. Interval between radiosurgery and high-grade toxicities ranged from 1 week to 4 months. Conclusions: Our series suggests that patients with MS who receive GKRS may be at increased risk of rare and high-grade treatment-related toxicity. Given the time course of toxicity, treatment-related edema or demyelination represent potential mechanisms.

  1. Congenital Vascular Malformations of the Liver: An Association With Trisomy 21.

    PubMed

    Burdall, Oliver C; Grammatikopoulos, Tassos; Sellars, Maria; Hadzic, Nedim; Davenport, Mark

    2016-12-01

    A link between congenital vascular malformation (CVM) of the liver and trisomy 21 has been suggested. We reviewed all children with trisomy 21 referred for investigation to a specialist pediatric hepatobiliary unit (1985-2015). Forty-five children with trisomy 21 were identified; 7 (15%) had a defined CVMs (4 girls). All such infants were also diagnosed with a range of cardiac defects. CVMs were divided according to the nature of the vascular connection. Group (i) (n = 3): Abnormal venovenous anomaly. This included portocaval shunt and patent ductus venosus (n = 2). Group (ii) (n = 4): Involvement of all 3 vascular systems. Two infants had arterioportal hypertension caused by hepatic arteries feeding into a left portal vein aneurysm within the umbilical fissure. Two infants had more complex hepatic artery to hepatic vein shunts developing early cardiorespiratory failure with progressive jaundice. Our series shows a clear association between cardiac anomalies and CVM in children with trisomy 21.

  2. [Diagnosis and differential diagnosis of cerebro-vascular malformations by CT (author's transl)].

    PubMed

    Schumacher, M; Stoeter, P; Voigt, K

    1980-03-01

    In 38 patients, the diagnosis of a cerebrovascular malformation (17 arteriovenous agniomas including one low-flow- and two venous angiomas; 10 aneurysms; 4 arteriovenous fistulae of the cavernous sinus, the tentorium and one of the Great Vein of Galen; 6 megadolical basilar arteries) was initially made by computertomographic (CT) examination, including contrast enhancement. The characteristic and pathognomonic CT findings are described and compared with those of cerebral angiography also done in these cases. The problems of differential diagnosis and the reasons for a false CT diagnosis in 5 other patients with a cerebro-vascular malformation are investigated; and the diagnostic value of cerebral angiography and CT is discussed and their complementary functions are being pointed out.

  3. Novel Vascular Malformation in an Affected Newborn with Deletion Del(4)(q31.3)

    PubMed Central

    de León Ojeda, Norma Elena; Soriano-Torres, Michel; Cabrera, Mercedes J.; Benítez Ramos, Dunia Bárbara

    2012-01-01

    We report on a newborn male patient with a terminal deletion in the long arm of the chromosome 4 with a congenital heart defect unreported before in association with this syndrome. The patient had multiple congenital anomalies including a pointed duplicated fingernail, low set posteriorly rotated ears, large anterior fontanel, micrognathia, glabellar capillary vascular malformation, and Interrupted Aortic Arch type C. The patient died due to multiple congenital malformations; a peripheral chromosome analysis showed 46, XY, del(4)(q31.3) de novo. The only reported case with the same deletion was a male newborn that exhibited the pattern of minor anomalies of deletion 4q31 syndrome. The parents were cytogenetically normal. We compare clinical signs to other cases with a deletion in long arm of chromosome 4. PMID:23320208

  4. Results of a Conservative Dose Plan Linear Accelerator-Based Stereotactic Radiosurgery for Pediatric Intracranial Arteriovenous Malformations.

    PubMed

    Rajshekhar, Vedantam; Moorthy, Ranjith K; Jeyaseelan, Visalakshi; John, Subhashini; Rangad, Faith; Viswanathan, P N; Ravindran, Paul; Singh, Rabiraja

    2016-11-01

    To evaluate the obliteration rate and clinical outcome following linear accelerator (LINAC)-based stereotactic radiosurgery (SRS) for intracranial arteriovenous malformation (AVM) in pediatric patients (age ≤18 years). Factors associated with the obliteration rate and neurologic complications were studied retrospectively in pediatric patients who underwent LINAC-based SRS for AVM between June 1995 and May 2014. The study cohort comprised 36 males and 33 females, with a median age at the time of SRS of 14 years (range, 7-18 years). The mean AVM volume was 8.5 ± 8.7 cc (range, 0.6-41.8 cc). The median marginal dose of radiation delivered was 15 Gy (range, 9-20 Gy). Magnetic resonance imaging (MRI) demonstrated complete obliteration of the AVM in 44 of the 69 patients (63.8%), at a mean follow up of 27.5 months (range, 12-90 months). On subgroup analysis, 41 of the 53 AVMs of ≤14 cc in volume (77.3%) were obliterated. AVMs with a modified AVM radiosurgery score <1 had significantly shorter obliteration times from the time of SRS (P = .006). On multivariate analysis, the mean marginal dose of radiation delivered to the AVM was the sole significant predictor of obliteration (odds ratio, 1.6; 95% confidence interval, 1 to 2.4). A modest median marginal dose of 15 Gy (16 Gy in the obliterated AVM group vs. 12 Gy in the nonobliterated group) resulted in an obliteration rate of 66.7% after LINAC-based SRS for intracranial AVM, with low rate. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Overexpression of Notch1 ectodomain in myeloid cells induces vascular malformations through a paracrine pathway.

    PubMed

    Li, Xiujie; Calvo, Ezequiel; Cool, Marc; Chrobak, Pavel; Kay, Denis G; Jolicoeur, Paul

    2007-01-01

    We previously reported that truncation of Notch1 (N1) by provirus insertion leads to overexpression of both the intracellular (N1(IC)) and the extracellular (N1(EC)) domains. We produced transgenic (Tg) mice expressing N1(EC) in T cells and in cells of the myeloid lineage under the regulation of the CD4 gene. These CD4C/N1(EC) Tg mice developed vascular disease, predominantly in the liver: superficial distorted vessels, cavernae, lower branching of parenchymal vessels, capillarized sinusoids, and aberrant smooth muscle/endothelial cell topography. The disease developed in lethally irradiated normal mice transplanted with Tg bone marrow or fetal liver cells as well as in Rag-/- Tg mice. In nude mice transplanted with fetal liver cells from (ROSA26 x CD4C/N1(EC)) F1 Tg mice, abnormal vessels were of recipient origin. Transplantation of Tg peritoneal macrophages into normal recipients also induced abnormal vessels. These Tg macrophages showed impaired functions, and their conditioned medium inhibited the proliferation of liver sinusoid endothelial cells in vitro. The Egr-1 gene and some of its targets (Jag1, FIII, FXIII-A, MCP-1, and MCP-5), previously implicated in hemangioma or vascular malformations, were overexpressed in Tg macrophages. These results show that myeloid cells can be reprogrammed by N1(EC) to induce vascular malformations through a paracrine pathway.

  6. Pathologically confirmed cryptic vascular malformation as a cause of convexity subarachnoid hemorrhage: case report.

    PubMed

    Mutoh, Tatsushi; Kobayashi, Shinya; Ishikawa, Tatsuya; Moroi, Junta; Miyata, Hajime; Suzuki, Akifumi; Yasui, Nobuyuki

    2012-05-01

    We report a rare case of pathologically confirmed cryptic vascular malformation as a cause of primary convexity subarachnoid hemorrhage (SAH) of unknown etiology. A 48-year-old woman presented with sudden severe headache. Localized right convexity SAH was observed on computed tomography (CT) scan, but the origin could not be detected despite extensive workup covering the entire head by using 3.0-Tesla magnetic resonance (MR) imaging with MR angiography and CT angiography combined with venous-phase imaging with a 320-detector row CT scanner. Subsequent digital subtraction angiography (DSA) performed 2.5 hours after admission failed to reveal any cause of SAH; however, a right frontoparietal avascular region was suspected to be due to a newly developed intracerebral hematoma. The lesion was simultaneously confirmed by angiographic cone-beam CT imaging. Because she remained neurologically intact, we decided to perform a follow-up study later with medical management. However, she developed left hemiparesis 3 hours after DSA. CT scan demonstrated progression of the hematoma, and her symptoms gradually worsened. Emergent surgical exploration along the SAH superficial to the postcentral sulcus and hematoma evacuation were performed, with favorable functional outcome. Pathological examination confirmed cryptic vascular malformation with several abnormally dilated arterioles within the subarachnoid space surrounded by a thick SAH clot. It is important to consider the possibility of ruptured cryptic vascular malformation as a cause of nontraumatic nonaneurysmal convexity SAH when recurrent hemorrhage occurs despite thorough diagnostic workup, because surgical resection may be the only curative treatment option to eliminate the risk of rebleeding and disabling symptoms.

  7. Shared Decision-Making in the Management of Congenital Vascular Malformations.

    PubMed

    Horbach, Sophie E R; Ubbink, Dirk T; Stubenrouch, Fabienne E; Koelemay, Mark J W; van der Vleuten, Carine J M; Verhoeven, Bas H; Reekers, Jim A; Schultze Kool, Leo J; van der Horst, Chantal M A M

    2017-03-01

    In shared decision-making, clinicians and patients arrive at a joint treatment decision, by incorporating best available evidence and the patients' personal values and preferences. Little is known about the role of shared decision-making in managing patients with congenital vascular malformations, for which preference-sensitive decision-making seems obvious. The authors investigated preferences regarding decision-making and current shared decision-making behavior during physician-patient encounters. In two Dutch university hospitals, adults and children with congenital vascular malformations facing a treatment-related decision were enrolled. Before the consultation, patients (or parents of children) expressed their preference regarding decision-making (Control Preferences Scale). Afterward, participants completed shared decision-making-specific questionnaires (nine-item Shared Decision-Making Questionnaire, CollaboRATE, and satisfaction), and physicians completed the Shared Decision-Making Questionnaire-Physician questionnaire. Consultations were audiotaped and patient involvement was scored by two independent researchers using the five-item Observing Patient Involvement instrument. All questionnaire results were expressed on a scale of 0 to 100 (optimum shared decision-making). Fifty-five participants (24 parents and 31 adult patients) were included. Two-thirds preferred the shared decision-making approach (Control Preferences Scale). Objective five-item Observing Patient Involvement scores were low (mean ± SD, 31 ± 15), whereas patient and physician Shared Decision-Making Questionnaire scores were high, with means of 68 ± 18 and 68 ± 19, respectively. The median CollaboRATE score was 93. There was no clear relationship between shared decision-making and satisfaction scores. Although adults and parents of children with vascular malformations express a strong desire for shared decision-making, objective shared decision-making behavior is still lacking, most

  8. 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

  9. Surgical outcomes of Majewski osteodysplastic primordial dwarfism Type II with intracranial vascular anomalies.

    PubMed

    Teo, Mario; Johnson, Jeremiah N; Bell-Stephens, Teresa E; Marks, Michael P; Do, Huy M; Dodd, Robert L; Bober, Michael B; Steinberg, Gary K

    2016-12-01

    OBJECTIVE Majewski osteodysplastic primordial dwarfism Type II (MOPD II) is a rare genetic disorder. Features of it include extremely small stature, severe microcephaly, and normal or near-normal intelligence. Previous studies have found that more than 50% of patients with MOPD II have intracranial vascular anomalies, but few successful surgical revascularization or aneurysm-clipping cases have been reported because of the diminutive arteries and narrow surgical corridors in these patients. Here, the authors report on a large series of patients with MOPD II who underwent surgery for an intracranial vascular anomaly. METHODS In conjunction with an approved prospective registry of patients with MOPD II, a prospectively collected institutional surgical database of children with MOPD II and intracranial vascular anomalies who underwent surgery was analyzed retrospectively to establish long-term outcomes. RESULTS Ten patients with MOPD II underwent surgery between 2005 and 2012; 5 patients had moyamoya disease (MMD), 2 had intracranial aneurysms, and 3 had both MMD and aneurysms. Patients presented with transient ischemic attack (TIA) (n = 2), ischemic stroke (n = 2), intraparenchymal hemorrhage from MMD (n = 1), and aneurysmal subarachnoid hemorrhage (n = 1), and 4 were diagnosed on screening. The mean age of the 8 patients with MMD, all of whom underwent extracranial-intracranial revascularization (14 indirect, 1 direct) was 9 years (range 1-17 years). The mean age of the 5 patients with aneurysms was 15.5 years (range 9-18 years). Two patients experienced postoperative complications (1 transient weakness after clipping, 1 femoral thrombosis that required surgical repair). During a mean follow-up of 5.9 years (range 3-10 years), 3 patients died (1 of subarachnoid hemorrhage, 1 of myocardial infarct, and 1 of respiratory failure), and 1 patient had continued TIAs. All of the surviving patients recovered to their neurological baseline. CONCLUSIONS Patients with MMD

  10. Orbital Hemangioma with Intracranial Vascular Anomalies and Hemangiomas: A New Presentation of PHACE Syndrome?

    PubMed

    Antonov, Nina K; Spence-Shishido, Allyson; Marathe, Kalyani S; Tlougan, Brook; Kazim, Michael; Sultan, Sally; Hess, Christopher P; Morel, Kimberly D; Frieden, Ilona J; Garzon, Maria C

    2015-01-01

    We present two cases of infants with a similar constellation of clinical findings: retro-orbital infantile hemangioma (IH), internal carotid artery (ICA) arteriopathy, and intracranial IH. In both cases, intracranial vascular anomalies and hemangiomas were found incidentally during evaluation of unilateral proptosis. Neither infant had evidence of cutaneous segmental IH of the face or neck, which might have provided a clue to the diagnosis of PHACE syndrome or of intracranial hemangiomas. In one case, intracranial involvement was particularly extensive and function threatening, with mass effect on the brain parenchyma. These cases serve to highlight the fact that clinical findings of proptosis, globe deviation, and strabismus should prompt immediate imaging to confirm the presence of orbital IHs and to exclude other diagnoses. Moreover, based on our cases and the embryologic origin of the orbit as a unique developmental unit, patients with confirmed retro-orbital IHs should undergo evaluation for anomalies associated with PHACE syndrome. Patients with orbital IHs and an additional major criterion for PHACE syndrome should be considered to have definite, and not just possible, PHACE syndrome.

  11. [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.

  12. Stereotactic radiosurgery of angiographically occult vascular malformations: Indications and preliminary experience

    SciTech Connect

    Kondziolka, D.; Lunsford, L.D.; Coffey, R.J.; Bissonette, D.J.; Flickinger, J.C. )

    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 where 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.

  13. Stereotactic heavy-charged-particle Bragg peak radiosurgery for the treatment of intracranial arteriovenous malformations in childhood and adolescence

    SciTech Connect

    Levy, R.P.; Fabrikant, J.I.; Frankel, K.A.; Phillips, M.H.; Lyman, J.T.

    1989-06-01

    Forty patients aged 6 to 18 years have now been treated for inoperable intracranial arteriovenous malformations (AVMs) using stereotactic heavy-charged-particle Bragg peak radiosurgery at the Lawrence Berkeley Laboratory 184-inch Synchrocyclotron at the University of California, Berkeley. This paper describes the procedures for selection of patients, the treatment protocol, and the neurological and neuroradiological responses to stereotactic radiosurgery in this age group. The volumes of the treated AVMs ranged from 265 mm/sup 3/ to 60,000 mm/sup 3/. The results are favorable: thus far, 20 of 25 patients have experienced greater than or equal to 50% obliteration of their AVMs within 1 year after treatment, and 14 of 18 patients have experienced total obliteration of the AVM by 2 years after treatment. Two patients hemorrhaged from radiosurgically treated AVMs within 12 months after treatment, but none thereafter. Complications include vasogenic edema and arterial occlusion; three patients have had neurological worsening as definite or possible sequelae of treatment. The strengths and limitations of the method are discussed.

  14. Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity

    PubMed Central

    Stockton, Rebecca A.; Shenkar, Robert; Awad, Issam A.

    2010-01-01

    Endothelial cell–cell junctions regulate vascular permeability, vasculogenesis, and angiogenesis. Familial cerebral cavernous malformations (CCMs) in humans result from mutations of CCM2 (malcavernin, OSM, MGC4607), PDCD10 (CCM3), or KRIT1 (CCM1), a Rap1 effector which stabilizes endothelial cell–cell junctions. Homozygous loss of KRIT1 or CCM2 produces lethal vascular phenotypes in mice and zebrafish. We report that the physical interaction of KRIT1 and CCM2 proteins is required for endothelial cell–cell junctional localization, and lack of either protein destabilizes barrier function by sustaining activity of RhoA and its effector Rho kinase (ROCK). Protein haploinsufficient Krit1+/− or Ccm2+/− mouse endothelial cells manifested increased monolayer permeability in vitro, and both Krit1+/− and Ccm2+/− mice exhibited increased vascular leak in vivo, reversible by fasudil, a ROCK inhibitor. Furthermore, we show that ROCK hyperactivity occurs in sporadic and familial human CCM endothelium as judged by increased phosphorylation of myosin light chain. These data establish that KRIT1–CCM2 interaction regulates vascular barrier function by suppressing Rho/ROCK signaling and that this pathway is dysregulated in human CCM endothelium, and they suggest that fasudil could ameliorate both CCM disease and vascular leak. PMID:20308363

  15. Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity.

    PubMed

    Stockton, Rebecca A; Shenkar, Robert; Awad, Issam A; Ginsberg, Mark H

    2010-04-12

    Endothelial cell-cell junctions regulate vascular permeability, vasculogenesis, and angiogenesis. Familial cerebral cavernous malformations (CCMs) in humans result from mutations of CCM2 (malcavernin, OSM, MGC4607), PDCD10 (CCM3), or KRIT1 (CCM1), a Rap1 effector which stabilizes endothelial cell-cell junctions. Homozygous loss of KRIT1 or CCM2 produces lethal vascular phenotypes in mice and zebrafish. We report that the physical interaction of KRIT1 and CCM2 proteins is required for endothelial cell-cell junctional localization, and lack of either protein destabilizes barrier function by sustaining activity of RhoA and its effector Rho kinase (ROCK). Protein haploinsufficient Krit1(+/-) or Ccm2(+/-) mouse endothelial cells manifested increased monolayer permeability in vitro, and both Krit1(+/-) and Ccm2(+/-) mice exhibited increased vascular leak in vivo, reversible by fasudil, a ROCK inhibitor. Furthermore, we show that ROCK hyperactivity occurs in sporadic and familial human CCM endothelium as judged by increased phosphorylation of myosin light chain. These data establish that KRIT1-CCM2 interaction regulates vascular barrier function by suppressing Rho/ROCK signaling and that this pathway is dysregulated in human CCM endothelium, and they suggest that fasudil could ameliorate both CCM disease and vascular leak.

  16. Photosclerosis of cutaneous vascular malformations with a pulsed 810-nm diode laser

    NASA Astrophysics Data System (ADS)

    Bass, Lawrence S.

    1995-05-01

    Various continuous wave lasers have been effectively used for sclerosis of cutaneous vascular lesions. The risk of scarring has been as high as 15% in some applications, however. Pulse dye lasers have virtually eliminated scarring as a complication, substituting high cost and cumbersome operation. Pre-clinical studies have demonstrated the ability of the 810 nm gallium-aluminum-arsenide diode laser in photosclerosis. In this study, a small group of patients (n equals 6) were treated with a pulsed 810 nm diode laser (Surgimedics, The Woodlands, TX). Pulse widths used were 5 - 15 msec with corresponding energy densities during the square wave pulse of 14 - 42 J/cm2. Pulse interval was 32 msec. Lesions treated include telangiectasias (n equals 5), spider veins, (n equals 4), capillary dermal malformation (n equals 1) and a cutaneous venous malformation (n equals 1). Lower extremity spider veins were the most resistent to treatment, rarely disappearing entirely. Telangiectasias were most responsive, usually disappearing after one treatment. No scarring was noted and textural changes were seen in only one patient. While these data are preliminary and parameters have not been optimized, pulsed diode laser exposure can be an effective treatment for cutaneous vascular lesions.

  17. Massive lower gastrointestinal bleeding due to 'Dieulafoy's vascular malformation' of the jejunum: case report.

    PubMed Central

    Goins, W. A.; Chatman, D. M.; Kaviani, M. J.

    1995-01-01

    Dieulafoy reported three cases of massive gastric hemorrhage due to a dilated submucosal artery in 1898, and since then, more than 100 cases of this gastric vascular malformation have been reported in the literature. These same pathologic lesions are even a rarer occurrence in the small bowel. This article reports a 38-year-old hypotensive male who presented to the hospital after an acute onset of massive lower gastrointestinal hemorrhage; superior mesenteric angiography demonstrated an actively bleeding lesion in a proximal jejunal branch. Intraoperative small bowel endoscopy via an enterotomy demonstrated a 4 mm bleeding submucosal lesion 30 cm distal to the ligament of Treitz. A literature review revealed six other cases of Dieulafoy's vascular malformation that occurred in the small bowel, with the lesions located in the proximal jejunum between 15 cm and 45 cm distal to the ligament of Treitz. The cause of these lesions is unknown. This case demonstrates the importance of preoperative angiography and intraoperative endoscopy when massive lower gastrointestinal hemorrhage is suspected to be from a small bowel source. Images Figure 1 Figure 2 Figure 3 PMID:7473854

  18. The contribution of prenatal diagnosis to the understanding of malformative intracranial cysts: state of the art.

    PubMed

    Pierre-Kahn, A; Hanlo, P; Sonigo, P; Parisot, D; McConnell, R S

    2000-11-01

    This review evaluates the contribution of prenatal diagnosis to the understanding of intracranial cysts. We describe the outcome of 54 fetuses in which prenatal investigations indicated the presence of such lesions. The cysts were diagnosed between 20 and 30 weeks of gestation. Most (63%) were supratentorial and interhemispheric. There was only a single sylvian cyst. In the infratentorial compartment, median retrocerebellar cysts were predominant. Incisural cysts accounted for 14.8% of the series. Nine pregnancies were interrupted because of the presence of associated brain disorders. Forty-five children are alive. Thirty-four had neuropsychological tests. Cysts rarely progressed, most frequently stabilized and often regressed postnatally. Hydrocephalus was rare. In two cases delivery was precipitated at 36 weeks to allow urgent treatment of rapidly evolving cysts. Thirteen children (28.2%) were treated postnatally, in general for developing cysts. The median follow-up for the whole series exceeds 4 years. Behavior, neurological development, and intelligence are normal in 88% of the cases, and 91% have a normal neurological status. Prognosis at the time of the prenatal consultation was correct in 89% of the cases. We emphasize the value of prenatal magnetic resonance imaging and karyotype studies to limit risks of incorrect prognosis.

  19. 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.

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

    PubMed

    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.

  1. Rare presentation of intracranial vascular blowout after tumor resection and radiation therapy

    PubMed Central

    Alaraj, Ali; Behbahani, Mandana; Valyi-Nagy, Tibor; Aardsma, Nathan; Aletich, Victor A

    2014-01-01

    A middle-aged patient presented with a rapidly growing right dural-based extra-axial posterior clinoid mass extending to the right cavernous sinus that was surgically resected. Histological examination showed solid growth of primitive neuroectodermal tumor arising from the third nerve. Following surgical resection, the patient was further managed by radiation and chemotherapy. Two years later the patient developed new intracranial hemorrhage in the area adjacent to the previous surgical cavity. A cerebral angiogram showed contrast extravasation at the junction of the posterior communicating artery (Pcom) and the right posterior cerebral artery (PCA), with an expanding pseudoaneurysm. This was managed with N-butyl cyanoacrylate embolization. Autopsy showed microscopic recurrence of tumor into the PCA/PCom region with invasion of the wall of the Pcom. This case report illustrates the concept of vascular blowout in intracranial cerebral vasculature. It appears that, in the presence of risk factors that contribute to weakening of vessel walls (surgery, radiation, tumor recurrence), a blowout can occur intracranially. PMID:24748141

  2. Sex-dichotomous effects of NOS1AP promoter DNA methylation on intracranial aneurysm and brain arteriovenous malformation.

    PubMed

    Wang, Zhepei; Zhao, Jikuang; Sun, Jie; Nie, Sheng; Li, Keqing; Gao, Feng; Zhang, Tiefeng; Duan, Shiwei; Di, Yazhen; Huang, Yi; Gao, Xiang

    2016-05-16

    The goal of this study was to investigate the contribution of NOS1AP-promoter DNA methylation to the risk of intracranial aneurysm (IA) and brain arteriovenous malformation (BAVM) in a Han Chinese population. A total of 48 patients with IAs, 22 patients with BAVMs, and 26 control individuals were enrolled in the study. DNA methylation was tested using bisulfite pyrosequencing technology. We detected significantly higher DNA methylation levels in BAVM patients than in IA patients based on the multiple testing correction (CpG4-5 methylation: 5.86±1.04% vs. 4.37±2.64%, P=0.006). In women, CpG4-5 methylation levels were much lower in IA patients (3.64±1.97%) than in BAVM patients (6.11±1.20%, P<0.0001). However, in men, CpG1-3 methylation levels were much higher in the controls (6.92±0.78%) than in BAVM patients (5.99±0.70%, P=0.008). Additionally, there was a gender-based difference in CpG1 methylation within the controls (men vs. women: 5.75±0.50% vs. 4.99±0.53%, P=0.003) and BAVM patients (men vs. women: 4.70±0.74% vs. 5.50±0.87%, P=0.026). A subgroup analysis revealed significantly higher CpG3 methylation in patients who smoked than in those who did not (P=0.041). Our results suggested that gender modulated the interaction between NOS1AP promoter DNA methylation in IA and BAVM patients. Our results also confirmed that regular tobacco smoking was associated with increased NOS1AP methylation in humans. Additional studies with larger sample sizes are required to replicate and extend these findings.

  3. Intracranial arteriovenous malformations treated utilizing a linear accelerator-based patient rotator or commercially available radiosurgery system.

    PubMed

    Scarbrough, Todd J; Crocker, Ian R; Davis, Lawrence W; Barrow, Daniel L; Fowler, B Zach; Oyesiku, Nelson M

    2005-01-01

    To report a single-institution experience with intracranial arteriovenous malformations (AVMs) treated utilizing a linear accelerator-based patient rotator (PR) or BrainLAB (BL) radiosurgery system (BrainLAB AG, Heimstetten, Germany). Since 1989, 84 evaluable patients were treated. PR patients (n = 45) were planned/localized on the basis of biplane angiography and treated between 1989 and 2000. BL patients (n = 39) were planned/localized on the basis of CT/MRI and treated since 2000. Kaplan-Meier analyses of survival, nidus obliteration (NO), and any radiographic improvement were undertaken with Cox regression of dose and volume effects. No significant complication, survival, previous embolization incidence, AVM location or size differences existed between BL/PR patients. The groups differed significantly in prescribed dose (PR: 16.2 Gy, BL: 17.3 Gy, p = 0.004) and isodose (PR: 62%, BL: 79%, p < 0.0001). Estimated 2-year NO rate was 87% for BL patients, 12% for PR patients (p < 0.0001). Ultimate PR NO rate was 67% at 6 years. Dose (p = 0.037) and isodose (p = 0.014) significantly affected PR NO outcome; volume was of borderline significance (p = 0.069). No factors significantly affected BL outcome. Analyses of small (< or = 4.0 cm3), high-dose (> or = 17.0 Gy) PR patients (PR1 group) vs. BL patients still demonstrated greater NO (p = 0.04) and radiographic improvement (p = 0.0004) rates for the BL group. PR1 patients had a 76% 3-year NO rate. BL-based radiosurgery achieved a high NO rate, the PR method did not. Differences in outcomes between PR/BL groups may be due to localization methods or an inherent advantage with the BL system. Copyright 2005 S. Karger AG, Basel.

  4. Vascular angular remodeling by kissing-Y stenting in wide necked intracranial bifurcation aneurysms.

    PubMed

    Melber, Katharina; Meila, Dan; Draheim, Philipp; Grieb, Dominik; Greling, Björn; Schlunz-Hendann, Martin; Brassel, Friedhelm

    2016-12-05

    Single stent deployment leads to a change in vascular geometry in wide necked bifurcation aneurysms. In some complex cases, the use of the single stent technique might not be sufficient or may not be feasible. The kissing-Y stenting technique appears to be an alternative endovascular treatment option. The aim of this study was to evaluate the effects of the kissing-Y stenting technique on vascular angular remodeling. 21 patients with wide necked intracranial bifurcation aneurysms at different sites (10 anterior communicating artery, 6 middle cerebral artery, 3 basilar artery, 1 vertebral artery/posterior inferior cerebellar artery, 1 internal carotid artery/posterior communicating artery) were treated with 44 closed cell stents (follow-up 2012-2016) using the kissing-Y stenting technique. We analyzed vascular angle geometry between the mother and both affected daughter vessels by digital subtraction angiography, before and after stent deployment, using standard working projections. Endovascular treatment of wide necked intracranial aneurysms using the kissing-Y stenting technique significantly decreased the angle between the bifurcation branches from 130.4±9.5° to 91.5±9.1° (p<0.0001). Kissing-Y stenting in wide necked bifurcation aneurysms leads to vascular angular remodeling of both affected branches. The resulting straightening of the bifurcation angle may prevent aneurysmal recurrence. 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/.

  5. Cardiopulmonary bypass in surgery for complex-combined vascular malformation of the lower limb: case report.

    PubMed

    Ismail, M S; Sharaf, I; Thambidorai, C R; Zainal, A; Somasundaram, S; Adeeb, S; Sajjad, M Y; Bilkis, B; Felicia, L S K

    2005-05-01

    A 16-year-old boy was referred with features of Parkes Weber syndrome (PWS) involving the right lower limb. He had presented at birth with cutaneous vascular malformations (VM) in the right thigh and at the age of 7 years developed congestive cardiac failure, which was controlled with drugs. He received alpha interferon and steroids during this period without any benefit. He defaulted follow-up and at 12 years of age presented with further enlargement of the VM in the right thigh and leg with skin and soft tissue thickening. At this stage, embolization and subsequent excision of the VM were tried, but the surgery was abandoned because of massive hemorrhage. Over the next 4 years, the boy became totally bedridden because of massive increase in the size of the limb, repeated hemorrhages, and secondary infection of the VM. Right hip disarticulation was considered the best option to improve his quality of life. To prevent uncontrollable hemorrhage during surgery, the disarticulation was done under cardiopulmonary bypass with low circulatory flow. Postoperatively, the patient required intensive care nursing for a week. He is presently ambulatory with crutches. Cardiopulmonary bypass with low flow has been used for treating posttraumatic arteriovenous malformations. However, its use in surgery for PWS has not been reported earlier.

  6. Vascular malformations of central nervous system: A series from tertiary care hospital in South India

    PubMed Central

    Karri, Sudhir Babu; Uppin, Megha S.; Rajesh, A.; Ashish, K.; Bhattacharjee, Suchanda; Rani, Y. Jyotsna; Sahu, B. P.; Saradhi, M Vijaya; Purohit, A. K.; Challa, Sundaram

    2016-01-01

    Aims and Objectives: To describe clinicopathological features of surgically resected vascular malformations (VMs) of central nervous system (CNS). Materials and Methods: Histologically diagnosed cases of VMs of CNS during April 2010–April 2014 were included. Demographic data, clinical and radiological features were obtained. Hematoxylin and eosin slides were reviewed along with Verhoeff-Van Gieson (VVG), Masson's trichrome, periodic acid-Schiff, and Perls' stains. Morphologically, cavernomas and arteriovenous malformations (AVMs) were distinguished on the basis of vessel wall features on VVG and intervening glial parenchyma. Results: Fifty cases were diagnosed as VMs of CNS with an age range of 14–62 years. These included 36 cavernomas, 12 AVMs, 2 mixed capillary-cavernous angiomas. Most of the cavernoma patients (15/36) presented with seizures, whereas AVM patients (8/12) had a headache as the dominant symptom. Twenty-nine patients were reliably diagnosed on radiological features. Microscopic evidence of hemorrhage was seen in 24/36 cavernomas and 6/12 AVMs, as opposed to radiologic evidence of 10 and 4, respectively. Reactive gliosis was seen in 16 cavernomas. Conclusions: Histological features are important for classifying the VMs of CNS as there are no specific clinical and radiological features. Type of VM has a bearing on management, prognosis, and risk of hemorrhage. PMID:27114659

  7. Application of indocyanine green videoangiography in surgery for spinal vascular malformations.

    PubMed

    Misra, Basant K; Purandare, Harshad R

    2012-06-01

    We present our recent experience with indocyanine green videoangiography (ICGVA) in intra-operative evaluation of two patients with dorsal spinal dural arteriovenous fistula (SDAVF) and one patient with conus medullaris arteriovenous malformation (AVM). To our knowledge, the latter is the first report of this in the literature. Intra-operative ICGVA was used to identify an early filling vessel and to obliterate the site of fistulous connection. This was confirmed by a repeat ICGVA study and correlated with post-operative digital subtraction angiography (DSA). The abnormal fistulous site was identified in all three patients and disconnected. Complete obliteration was confirmed in all patients using ICGVA and with post-operative imaging. There was no untoward reaction to the dye injection. We conclude that ICGVA is a useful adjunct in surgical treatment of spinal vascular malformations since it is a real-time, non-invasive, radiation-free technique with good image resolution, and is repeatable and easily reproducible. Technical disadvantages can be minimized by proper exposure of the operative field. Copyright © 2011 Elsevier Ltd. All rights reserved.

  8. Prospective evaluation of the incidence of uterine vascular malformations developing after abortion or delivery.

    PubMed

    Yazawa, Hiroyuki; Soeda, Syu; Hiraiwa, Tsuyoshi; Takaiwa, Masayo; Hasegawa-Endo, Sumiko; Kojima, Manabu; Fujimori, Keiya

    2013-01-01

    To describe the incidence of uterine vascular malformations (UVMs) including uterine arteriovenous malformations (AVMs) in patients after abortion or delivery and in outpatients. Prospective study (Canadian Task Force classification II-3). Fukushima Red Cross Hospital. Six patients with a UVM including 1 with an AVM. Clinical screening of patients using transvaginal color Doppler ultrasonography between April 2010 and March 2012. The incidence of UVM developing after abortion or delivery or in outpatients was prospectively evaluated using transvaginal color Doppler ultrasonography. From 959 patients, we identified 6 (0.63%) with UVMs, including 1 (0.10%) with a uterine AVM. Specifically, we detected UVMs in 4 of 77 patients (5.2%) after abortion, 1 of 458 patients (0.22%) after delivery, and 1 of 424 outpatients (0.24%). Four patients after abortion and 1 after delivery reported mild symptoms, which were treated conservatively; however, the outpatient had a severe uterine AVM, which was confirmed via 3-dimensional computed tomography angiography. The incidence of UVMs was relatively higher, in particular in the patients after abortion, and was significantly higher than that in postpartum or outpatient groups. Therefore, it is important to consider the possibility of UVMs in any patient with episodes of unexplained uterine bleeding and to perform follow-up analysis using color Doppler ultrasonography. Such an approach will facilitate accurate diagnosis and lead to appropriate clinical management to prevent unnecessary dangerous repeat curettage, which might induce profuse uterine bleeding. Copyright © 2013 AAGL. Published by Elsevier Inc. All rights reserved.

  9. Optimizing treatment parameters for the vascular malformations using 1064-nm Nd:YAG laser

    NASA Astrophysics Data System (ADS)

    Gong, Wei; Lin, He; Xie, Shusen

    2010-02-01

    Near infrared Nd:YAG pulsed laser treatment had been proved to be an efficient method to treat large-sized vascular malformations like leg telangiectasia for deep penetrating depth into skin and uniform light distribution in vessel. However, optimal clinical outcome was achieved by various laser irradiation parameters and the key factor governing the treatment efficacy was still unclear. A mathematical model in combination with Monte Carlo algorithm and finite difference method was developed to estimate the light distribution, temperature profile and thermal damage in epidermis, dermis and vessel during and after 1064 nm pulsed Nd:YAG laser irradiation. Simulation results showed that epidermal protection could be achieved during 1064 nm Nd:YAG pulsed laser irradiation in conjunction with cryogen spray cooling. However, optimal vessel closure and blood coagulation depend on a compromise between laser spot size and pulse duration.

  10. 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.

  11. Proptosis Reduction Using Sirolimus in a Child With an Orbital Vascular Malformation and Blue Rubber Bleb Nevus Syndrome.

    PubMed

    Gildener-Leapman, Juliana R; Rosenberg, Jamie B; Barmettler, Anne

    A 15-month-old boy with left congenital proptosis presented to the emergency department with melena. Upper GI endoscopy and magnetic resonance angiography revealed vascular lesions, consistent with gastrointestinal tract manifestations of blue rubber bleb nevus syndrome. MRI revealed vascular malformations in both orbits, with mass effect on the left side. The patient was started on a trial of the antiangiogenic agent sirolimus (also known as rapamycin), and after 6 months of treatment showed clinical improvement in proptosis supported by radiologic evidence of regression in the larger, left orbital mass, with stability of the smaller, right orbital mass. There are 11 published cases of orbital blue rubber bleb nevus syndrome in the English literature. To our knowledge, this is the first reported case of successful, long-term treatment with sirolimus causing a reduction in the size of an orbital vascular malformation.

  12. Cerebral Cavernous Malformation

    MedlinePlus

    ... cavernous malformations (CCMs) are vascular lesions comprised of clusters of tightly packed, abnormally thin-walled small blood ... cavernous malformations (CCMs) are vascular lesions comprised of clusters of tightly packed, abnormally thin-walled small blood ...

  13. Intraoperative Angiography Using Portable Fluoroscopy Unit in the Treatment of Vascular Malformation.

    PubMed

    Hayashi, Kentaro; Horie, Nobutaka; Morofuji, Yoichi; Fukuda, Shuji; Yamaguchi, Susumu; Izumo, Tsuyoshi

    2015-01-01

    Intraoperative angiography (IOA) is employed for the treatment of the complicated cases in neurological surgery. The IOA is usually performed with OEC portable digital subtraction angiography (DSA) unit. We are performing IOA with portable fluoroscopy unit with simple DSA function and report its usefulness on neurosurgical treatment. IOA or hybrid treatment with mobile fluoroscopy system was performed for 9 cases [cerebral arteriovenous malformation (AVM), 3; cranial dural arteriovenous fistula (AVF), 2; and spinal AVM/AVF, 4]. Thus, ex vivo analysis was performed comparing image quality of portable fluoroscopy unit and conventional DSA system. Although the resolution of portable fluoroscopy unit is not so high compared to conventional DSA system, the existence of the vascular lesions such as cerebral aneurysm, cerebral AVM, and spinal dural AVF were detected. The operation of portable fluoroscopy unit was simple and no special assistance was required. The complication related to the catheterization or IOA did not occur. IOA with portable fluoroscopy unit was useful for the identification of vascular lesion and has advantage on the cost benefit.

  14. [Is argon plasma coagulation an efficient treatment for digestive system vascular malformation and radiation proctitis?].

    PubMed

    Rolachon, A; Papillon, E; Fournet, J

    2000-12-01

    Argon beam coagulation is an innovative no-touch electrocoagulation technique in which high-frequency monopolar alternating current is delivered to the tissue through ionized argon gas. The aim of this prospective study was to evaluate the efficacy and safety of argon plasma coagulation (APC) for the treatment of hemorrhagic digestive vascular malformations and hemorrhagic radiation proctosigmoiditis. From March 1998 through April 1999, we used endoscopic APC (ERBE, Lyon, France, argon gas source ICC 300, high-frequency electrosurgical generator ICC 200, gas flow 1 L/min, power setting 50 W) to treat 39 consecutive patients (mean age 70.3 +/- 10 years). The indications for treatment were anemia (n =10), active or oozing haemorrhage (n =15) from digestive angiodysplastic lesions (n =25), hemorrhagic antral telangiectatic vascular lesions (n =2), and hemorrhagic radiation proctosigmoiditis (n =12) after failure of medical treatments (5-aminosalicylic acid, corticosteroids, or sucralfate enemas). The efficacy of APC treatment was evaluated on symptoms, transfusion requirement, bleeding recurrence, hemoglobin value before and 6 months after APC therapy. On the average, 1 +/- 0.5 sessions per patient was required to treat digestive vascular malformations. Definitive haemostasis of digestive angiodysplastic lesions with active or oozing haemorrhage was achieved in one session in all patients. No bleeding recurrence was observed during the follow-up period of 6 months. Anemia recurrence was observed in 2 patients (7%). Average hemoglobin levels recorded before and 6 months after APC therapy were 78.8 +/- 21.2 g/L and 108 +/- 13.7 g/L, respectively (P<0.05). On the average, 2.8 +/- 0.8 sessions per patient were required to treat hemorrhagic radiation proctosigmoiditis. Ten patients (83%) reported improvement or cessation of rectal bleeding, most of them immediately after APC therapy. Endoscopic control was performed one month after APC therapy and showed complete

  15. Vascular permeability and iron deposition biomarkers in longitudinal follow-up of cerebral cavernous malformations.

    PubMed

    Girard, Romuald; Fam, Maged D; Zeineddine, Hussein A; Tan, Huan; Mikati, Abdul Ghani; Shi, Changbin; Jesselson, Michael; Shenkar, Robert; Wu, Meijing; Cao, Ying; Hobson, Nicholas; Larsson, Henrik B W; Christoforidis, Gregory A; Awad, Issam A

    2016-08-05

    OBJECTIVE Vascular permeability and iron leakage are central features of cerebral cavernous malformation (CCM) pathogenesis. The authors aimed to correlate prospective clinical behavior of CCM lesions with longitudinal changes in biomarkers of dynamic contrast-enhanced quantitative permeability (DCEQP) and quantitative susceptibility mapping (QSM) assessed by MRI. METHODS Forty-six patients with CCMs underwent 2 or more permeability and/or susceptibility studies in conjunction with baseline and follow-up imaging and clinical surveillance during a mean 12.05 months of follow-up (range 2.4-31.27 months). Based on clinical and imaging features, cases/lesions were classified as stable, unstable, or recovering. Associated and predictive changes in quantitative permeability and susceptibility were investigated. RESULTS Lesional mean permeability and QSM values were not significantly different in stable versus unstable lesions at baseline. Mean lesional permeability in unstable CCMs with lesional bleeding or growth increased significantly (+85.9% change; p = 0.005), while mean permeability in stable and recovering lesions did not significantly change. Mean lesional QSM values significantly increased in unstable lesions (+44.1% change; p = 0.01), decreased slightly with statistical significance in stable lesions (-3.2% change; p = 0.003), and did not significantly change in recovering lesions. Familial cases developing new lesions during the follow-up period showed a higher background brain permeability at baseline (p = 0.001), as well as higher regional permeability (p = 0.003) in the area that would later develop a new lesion as compared with the homologous contralateral brain region. CONCLUSIONS In vivo assessment of vascular permeability and iron deposition on MRI can serve as objective and quantifiable biomarkers of disease activity in CCMs. This may be applied in natural history studies and may help calibrate clinical trials. The 2 techniques are likely applicable in

  16. Large, segmental, circular vascular malformation of the small intestine (in a female toddler with hematochezia): unusual presentation in a child.

    PubMed

    Kalmar, Peter I; Petnehazy, Thomas; Wießpeiner, Ulrike; Beer, Meinrad; Hauer, Almuthe C; Till, Holger; Riccabona, Michael

    2014-02-26

    Failure to thrive and hematochezia in children may be alarm signs warranting endoscopy. In contrast, vascular malformations of the small intestine are uncommon in this age group. We report on a female toddler in whom various imaging techniques revealed an unusually large segmental vascular malformation of the ileum as the cause of the child's main clinical symptoms. A 19 months old girl presented with severe anemia (Hb 3 mmol/l), failure to thrive and chronic diarrhea. Diagnostics for intestinal blood loss and pathogens were negative. The child had duodenoscopy, also for histological diagnosis of celiac disease, with negative results. A dietary protocol was suggestive for inadequate iron intake and she was supplemented. After symptomless four-months the child presented again, now with mild abdominal pain and, for the first time, hematochezia. An orienting abdominal ultrasound (US) study showed a suspicious tumorous bowel condition. A subsequent detailed abdominal US supplemented by a saline enema during investigation (i.e., "hydrocolon", to improve outlining of the formation's localization) revealed a large circumferential cystiform vascular mass of the ileum causing segmental ileal obstruction.Complementing preoperative abdominal hydro-MRI, planned based on the findings of the US study, confirmed the suspected vascular malformation of the ileum and exquisitely outlined the extent, location and anatomy.The patient was successfully operated laparoscopically, the affected ileum segment with the mass was completely removed as proven by histology, and the child recovered well. The huge segmental vascular malformation of the distal ileum described here is an extreme rarity in young children. Although the reported child's presenting symptoms malabsorption and malnutrition could have been responsible for its severe anemia, this was obviously caused by blood losses from the ileal vascular malformation. It was due to incipient abdominal pain rather than hematochezia that

  17. Angiomatous lesion and delayed cyst formation after gamma knife surgery for intracranial meningioma: case report and review of literatures.

    PubMed

    Liu, Zhiyong; He, Min; Chen, Hongxu; Liu, Yi; Li, Qiang; Li, Lin; Li, Jin; Chen, Haifeng; Xu, Jianguo

    2015-01-01

    Gamma Knife has become a major therapeutic method for intracranial meningiomas, vascular malformations and schwannomas with exact effect. In recent years an increasing number of delayed complications after Gamma Knife surgery have been reported, such as secondary tumors, cystic changes or cyst formation. But angiomatous lesion and delayed cyst formation after Gamma Knife for intracranial lesion has rarely been reported. Here we report the first case of angiomatous lesion and delayed cyst formation following Gamma Knife for intracranial meningioma and discuss its pathogenesis.

  18. Newly delineated syndrome of congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) in seven patients.

    PubMed

    Sapp, Julie C; Turner, Joyce T; van de Kamp, Jiddeke M; van Dijk, Fleur S; Lowry, R Brian; Biesecker, Leslie G

    2007-12-15

    We present a series of seven patients who were previously diagnosed with Proteus syndrome, but who do not meet published diagnostic criteria for this disorder and whose natural history is distinct from that of Proteus syndrome. This newly recognized phenotype comprises progressive, complex, and mixed truncal vascular malformations, dysregulated adipose tissue, varying degrees of scoliosis, and enlarged bony structures without progressive bony overgrowth. We have named this condition congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) on a heuristic basis. In contrast to the bony distortion so characteristic of Proteus syndrome, distortion in CLOVE syndrome occurs only following major or radical surgery. Here, we contrast differences and similarities of CLOVE syndrome to Proteus syndrome. (c) 2007 Wiley-Liss, Inc.

  19. [Arteriovenous malformation-glioma association: study of four cases].

    PubMed

    Borges, Lia Raquel R; Malheiros, Suzana M F; Pelaez, Maria Paula; Stávale, João Norberto; Santos, Adrialdo J; Carrete, Henrique; Nogueira, Roberto Gomes; Ferraz, Fernando A P; Gabbai, Alberto A

    2003-06-01

    We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years) presented with progressive headache with clinical evidence of intracranial hypertension (in 3) and partial seizures (in 1). CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.

  20. 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.

  1. PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia

    PubMed Central

    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

    2016-01-01

    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. PMID:27897192

  2. Periodontal growth in areas of vascular malformation in patients with Sturge-Weber syndrome: a management protocol.

    PubMed

    Pontes, Flávia Sirotheau Corrêa; Conte Neto, Nicolau; da Costa, Rodrigo Moreira Bringel; Loureiro, Arlison Miranda; do Nascimento, Liliane Silva; Pontes, Hélder Antônio Rebelo

    2014-01-01

    Sturge-Weber syndrome (SWS) is a very rare condition characterized by abnormal vascular formations that encompass several manifestations: cutaneous, neurologic, ocular, and oral. Neurologic conditions are the most important factor, especially epilepsy, which frequently leads patients to make use of anticonvulsants. These drugs are capable of inducing abnormal tissue growth in the oral cavity that can be situated over areas with vascular malformation, requiring special attention by the clinician. This report describes 1 case of SWS and performs a literature review of treatments for this condition, providing a protocol of treatment for these clinical situations.

  3. Disorganized vascular structures in sporadic venous malformations: a possible correlation with balancing effect between Tie2 and TGF-β

    PubMed Central

    Chen, Gang; Ren, Jian-Gang; Zhang, Wei; Sun, Yan-Fang; Wang, Feng-Qin; Li, Rui-Fang; Zhang, Jian; Zhao, Yi-Fang

    2014-01-01

    Venous malformations (VMs) are among the most common slow-flow vascular malformations characterized by irregular venous channels, luminal thrombi, and phleboliths. To systematically manifest the disorganized vascular structures in sporadic VMs, we initially evaluated histopathological characteristics, perivascular cell coverage, adhesion molecules expression and vascular ultrastructures. Then, the expression of Tie2 and TGF-β in VMs was detected. Meanwhile, the in vitro studies were performed for mechanism investigation. Our data showed that the perivascular α-SMA+ cell coverage and expression of adhesion molecules in VMs were significantly decreased compared with those in the normal skin tissues. We also found that the expression and phosphorylation levels of Tie2 were upregulated, whereas TGF-β was downregulated in VMs, and they were negatively correlated. Moreover, the in vitro results also revealed a possible balancing effect between Tie2 and TGF-β, as demonstrated by the findings that Ang-1 (agonist of Tie2) treatment significantly downregulated TGF-β expression, and treatment with recombinant TGF-β could also suppress Tie2 expression and phosphorylation. This study provided strong evidence supporting the disorganized vascular structures and dysregulation of related molecules in sporadic VMs, and demonstrated a possible balancing effect between Tie2 and TGF-β, which might help to develop novel therapeutics for vascular disorganization-related disorders. PMID:24966004

  4. CLOVE Syndrome (Congenital Lipomatous Overgrowth, Vascular Malformations, and Epidermal Nevi): CNS Malformations and Seizures may be a Component of this Disorder

    PubMed Central

    Gucev, Zoran S.; Tasic, Velibor; Jancevska, Aleksandra; Konstantinova, Marina Krstevska; Pop-Jordanova, Nada; Trajkovski, Zoran; Biesecker, Leslie G.

    2010-01-01

    A newborn girl was found to have a massive lymphatic truncal vascular malformation with overlying cutaneous venous anomaly associated with overgrown feet and splayed toes. These manifestations comprise the recently described CLOVE syndrome. She also had cranial asymmetry and developed generalized seizures, which were treated with anticonvulsants. Cranial CT showed encephalomalacia, widening of the ventricles and the sulci, hemimegalencephaly (predominantly white matter) and partial agenesis of corpus callosum. Review of the literature identified several other patients with CLOVE syndrome, some of whom were misdiagnosed as having Proteus syndrome, with strikingly similar manifestations. We conclude that CNS manifestations including hemimegalencephaly, dysgenesis of the corpus callosum, neuronal migration defects, and the consequent seizures, may be a more common manifestation of CLOVE syndrome than is presently appreciated. PMID:18816642

  5. Diode laser to treat small oral vascular malformations: A prospective case series study.

    PubMed

    Bacci, Christian; Sacchetto, Luca; Zanette, Gastone; Sivolella, Stefano

    2017-09-14

    The current work examined a consecutive series of patients presenting vascular malformations (VMs) and venous lakes (VLs) of the lip and oral mucosa who were treated with transmucosal diode laser applications and assessed over a 1 year period. Fifty-nine patients (31 males and 28 females) presenting low-flow VMs or VLs of the oral cavity were treated transmucosally using a diode laser (with an 830 nm operating wavelength and 1.6 W output power) with a 320 µm diameter flexible fiber. All the lesions were assessed 7 days, 30 days, and 1 year after the laser treatment, and the lesion reduction percentage was scored on a one to five scale. The patients were also asked to assess their pain perception daily during the 7 days following the treatment using a visual analog scale (VAS). There were no procedure-related intra- or post-operative complications; only modest pain intensity was reported. Thirty days after the treatment, lesion reduction was described as excellent or good in 52 cases; it was fair or poor in 7. Six patients (F:M ratio 2:4) required a second diode laser application. At the 1 year follow-up, volume reduction was complete in 48 out of 59 patients; there were five recurrences (F:M ratio 3:2). No relevant gender-related differences were noted. The use of diode laser application to treat small oral VMs and VLs was associated to shorter operating times and fewer postoperative complications with respect to the scapel surgery approach. More than one session may nevertheless be required if the anomaly is larger than 10 mm. Lasers Surg. Med. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

  6. Exsanguination by intent: controlled phlebotomy during resection of a giant vascular malformation in a 22-month-old child.

    PubMed

    Choi, Ellen; Landrigan-Ossar, Mary; Fishman, Steven J; Seefelder, Christian

    2011-11-01

    Resection of large vascular malformations may require transection across the lesion, resulting in uncontrollable bleeding with the risk of exsanguination or massive transfusion-related complications such as hyperkalemic cardiac arrest. We present the anesthetic management of a 22-month-old child with a giant vascular malformation who required surgical intervention because of increasing pain and bleeding from the lesion. As a standard resection carried a high risk of mortality for the patient, a novel surgical approach was performed, consisting of gradual compression of the lesion, reducing its base to allow transection across the smallest possible area. This compression resulted in acute massive autotransfusion managed by therapeutic phlebotomy of more than twice the circulating blood volume of the patient, guided by CVP and blood pressure. Although subsequent resection was still associated with large blood loss, the hemodynamic course of the patient was stable, and both bleeding and massive transfusion occurred in a controlled fashion allowing safe and successful resection of the malformation. © 2011 Blackwell Publishing Ltd.

  7. Occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of the lung: report of a case.

    PubMed

    Sa, Young Jo; Kim, Young Du; Moon, Seok-Whan; Kim, Chi-Kyung; Ki, Chang Seok

    2013-12-01

    An 8-year-old male presented with a cystic lung lesion in the left lower lobe, which was initially detected during surgery for a spontaneous rupture of the sigmoid colon at the age of 6 years. Tissue fragility and a tendency to bleed easily were noted during the surgery, which strongly suggested vascular Ehlers-Danlos syndrome. Although there was no abnormality in the hemostasis screening test, or any suspicious hereditary problem in his pedigree, genetic gene testing for vascular Ehlers-Danlos syndrome was recommended, and showed a de novo mutation in the COL3A1 gene. This report presents the case of patient with occlusive vascular Ehlers-Danlos syndrome accompanying a congenital cystic adenomatoid malformation of lung, in addition to a duplicated infrarenal vena cava.

  8. Differential Vascular Pathophysiologic Types of Intracranial Atherosclerotic Stroke: A High-Resolution Wall Magnetic Resonance Imaging Study.

    PubMed

    Ryoo, Sookyung; Lee, Mi Ji; Cha, Jihoon; Jeon, Pyoung; Bang, Oh Young

    2015-10-01

    Intracranial atherosclerotic stroke (ICAS) has various stroke mechanisms, including branch occlusive disease (BOD), subcortical infarcts caused by parent arterial disease occluding the perforator's orifice, and non-BOD, infarcts beyond the subcortical area caused by artery-to-artery embolism. To test whether these 2 types of ICAS had different vascular pathophysiologies, we compared the high-resolution magnetic resonance imaging characteristics between BOD and non-BOD ICAS. Eighty patients with acute infarcts caused by ICAS of proximal middle cerebral artery or basilar artery without carotid/cardiac embolic sources or nonatherosclerotic causes were enrolled (36 BOD and 44 non-BOD patients). The steno-occlusive intracranial artery at the maximal stenosis was analyzed for vascular remodeling and wall enhancement. BOD had distinct radiological features in terms of vascular morphology and enhancement. BOD showed a milder stenosis than non-BOD (P<0.001). Positive remodeling was more frequently observed in non-BOD than in BOD (P=0.005). Wall area index was also lower in BOD. Plaque enhancement was observed in all but one non-BOD patient and in one fourth of BOD patients (P=0.003). Although both types showed an eccentric enhancement, this enhancement was more frequently distributed in the BOD group on the side where the perforators arose. As the number of asymptomatic intracranial stenosis increased, the degree of stenosis (rho=0.513, P=0.003) increased in the BOD group, whereas enhanced plaque area (rho=0.343, P=0.030) increased in the non-BOD group. Our data indicate that BOD is a common and unique form of ICAS, distinct from non-BOD. These 2 types of ICAS have different vascular pathophysiologies in terms of vascular remodeling and plaque characteristics. © 2015 American Heart Association, Inc.

  9. Vascular Cures

    MedlinePlus

    ... Contact Us Vascular Disease What is Vascular Disease? Education and Awareness Vascular Diseases Abdominal Aortic Aneurysm Aortic Dissection Arteriovenous Malformation Atherosclerosis Buerger's Disease Carotid Artery Disease ...

  10. Efficacy of intra-lesional alcohol injection as alternative and/or complementary treatment of vascular malformations: a systematic review.

    PubMed

    Prasetyono, Theddeus O H; Kreshanti, Prasetyanugraheni

    2010-07-01

    The purpose of this article is to discuss whether intra-lesional alcohol injection is beneficial in the treatment of vascular malformations. A systematic review through literature search was conducted for English-language studies in PubMed. This search was conducted in April 2007 using EndNote 7.0 with keywords: 'vascular malformation*' and 'sclerotherapy'. Both retrospective and prospective studies on the efficacy of intra-lesional alcohol injection in the treatment of vascular malformations in the head and neck and/or upper extremity region published from 1997 to 2006 are included. Exclusion criteria are letters to editor, article, comment, discussion, literature research, publication review, prevalence study, animal study and lecture. Evaluation is aimed at level of clinical improvement or cure, number of injections required to achieve the desired clinical result and local or other complications. Thirty publications with usable information were retrieved. In 25 publications, a total of 567 patients (mean age: 18.6 years; 43.4% male, 56.6% female) received intra-lesional absolute alcohol injection, either as an independent therapy or as an adjunctive to other treatment modalities. From 13 publications, the clinical results are as follows: excellent 74 (22.3%), good 224 (67.5%) and poor 34 (10.2%). Mean number of injections required is 2.63. Minor complications are predominantly skin damage (21.1%). Major complications are mostly tissue fibrosis (1.9%). Intra-lesional alcohol injection as an independent treatment or in combination with other treatment modalities has good-to-excellent results. This intervention applied in fast-flow malformations is as effective as in slow-flow malformations. Preoperative and/or postoperative embolo/sclerotherapy provides substantial improvement on the overall results of the therapy. Intra-lesional alcohol injection is a relatively safe treatment modality with minor complications mostly involving the skin and some, but relatively

  11. Multimodality Image-Guided Sclerotherapy of Low-Flow Orbital Vascular Malformations: Report of Single-Center Experience.

    PubMed

    Harmoush, Samer; Chinnadurai, Ponraj; El Salek, Kamel; Metwalli, Zeyad; Herce, Honey; Bhatt, Amit; Steinkuller, Paul; Vece, Timothy; Siddiqui, Shakeel; Pimpalwar, Ashwin; Marx, Douglas; Mawad, Michel; Pimpalwar, Sheena

    2016-07-01

    To evaluate the role of multimodality imaging tools for intraprocedural guidance and outcome evaluation during sclerotherapy of low-flow orbital vascular malformations. A retrospective review was performed of 17 consecutive patients with low-flow orbital malformations (14 lymphatic, two venous, and one venolymphatic) who underwent multimodality image-guided sclerotherapy between November 2012 and May 2015. Sclerotherapy technique, image guidance tools, and complications were recorded. Sclerotherapy outcome was evaluated using clinical response, magnetic resonance (MR) image-based lesion volumetry, and proptosis quantification. There were 22 sclerotherapy sessions performed. Intraprocedural ultrasound (US), fluoroscopy, cone-beam computed tomography (CT) and MR image fusion were used for image guidance with 100% technical success. Resolution of presenting symptoms was observed in all patients at 1-month follow-up. Four major sclerotherapy complications were successfully managed. Statistically significant reduction in lesion volume (P = .001) and proptosis (P = .0117) by MR image analysis was achieved in all patients in whom 3-month follow-up MR imaging was available (n = 13/17). There was no lesion recurrence at a median follow-up of 18 months (range, 8-38 mo). Multimodality imaging tools, including US, fluoroscopy, cone-beam CT, and MR fusion, during sclerotherapy of low-flow orbital malformations provide intraprocedural guidance and quantitative image-based evaluation of treatment outcome. Copyright © 2016 SIR. Published by Elsevier Inc. All rights reserved.

  12. Comparing monomodality treatments of low-grade intracranial arteriovenous malformation at Hospital Kuala Lumpur between 2008 and 2011: A retrospective study.

    PubMed

    Bin Rosli, Fadzlishah Johanabas; Mohammed Haspani, Mohammed Saffari; Izaini Ab Ghani, Abdul Rahman

    2016-01-01

    Intracranial arteriovenous malformations (AVMs) of Spetzler-Martin grades (SMGs) I-III are treated using either monomodality treatments of microsurgical excision, embolization or stereotactic radiosurgery (SRS), or a combination of two or more of these treatment options. At Hospital Kuala Lumpur, we still practice monomodality treatments for AVMs of these three grades. In this study, we wanted to achieve an understanding whether monomodality treatments can achieve a satisfactory outcome of AVM nidi for patients, for up to 3 years, and to gather an objective data for AVM treatment for the Malaysian population. This is a retrospective review of records study. The data are acquired from case notes of patients with intracranial AVM of SMGs I to III who underwent monomodality treatment at Hospital Kuala Lumpur between 2008 and 2011. The patients were followed up with imaging for up to 3 years from the date of treatment. A total of 81 patients were recruited in this study, where 30 underwent microsurgical treatment, 27 underwent embolization, and 24 underwent SRS. Total obliteration of AVM nidus was achieved in 96.7% of patients who underwent microsurgery, 8.7% of patients who underwent embolization, and 79.2% of patients who underwent SRS. The modified Rankin scale (mRS) for all three groups showed an improving trend, with the microsurgery group showing the best improvement (from 70% at 3 months to 92.3% at 3 years showing favorable mRS scores). The AVM nidus obliteration for each treatment group is comparable to the meta-analysis published in 2011. Each modality had its own set of complications; however, most of the patients in all three groups had either static or improved mRS at the end of the 3-year follow-up.

  13. The role of serum basic fibroblast growth factor, estradiol and urine basic fibroblast growth factor in differentiating infantile haemangiomas from vascular malformations.

    PubMed

    Yang, X J; Jiang, Y H; Zheng, J W; Hong, L; Zhou, Q; Qin, Z P

    2011-08-01

    To investigate the role of serum basic fibroblast growth factor (bFGF), estradiol (E2) and urine bFGF in differentiating infantile haemangiomas from vascular malformations. Between October 2007 and January 2009, 97 patients with haemangiomas and 25 patients with vascular malformations who had not been treated previously were included in this prospective study. Forty-eight patients with cleft lip and/or palate were selected as controls. The age of all subjects ranged from 1 to 30 months. The serum and urine levels of bFGF were determined by enzyme-linked immunosorbent assay (ELISA). The serum levels of E2 were examined via radioimmunoassay. All data were analysed with SPSS 11.5 software package. The concentration of serum and urine bFGF was significantly different among the three groups (haemangiomas, vascular malformations and controls) (P = 0.027, P = 0.001). Significantly different urine bFGF levels were found in patients with proliferating and involuting haemangiomas (P = 0.04). The serum E2 levels were significantly higher in patients with haemangiomas than vascular malformations (P = 0.001) and controls (P = 0.001). Serum bFGF and E2 as well as urine bFGF can be used to supplement the clinical diagnosis of congenital vascular anomalies. Urine bFGF combined with serum E2 may be the most potential markers for diagnosing haemangiomas and determining the proliferating stage of haemangiomas.

  14. Effect of electrochemical treatment on high-flow vascular malformations in the maxillofacial region.

    PubMed

    Xue, Lei; Qin, Xing-Jun; Wang, Xu-Kai; Wang, Hua; Jia, Rui; Zhai, Qin-Kai

    2011-12-01

    We explored the effect of electrochemical therapy for the treatment of high-flow venous malformations in the maxillofacial region in 32 patients. We used a method of anaesthesia that was suitable to the site and size of the lesion, and then inserted platinum needles into the lesion in a sterile environment. We protected the normal skin by inserting the cannulas into plastic sheaths, and connected the needles to an electrochemical machine. The common voltage, electric current, and amount of electricity were 6-8 V, 80-100 mA, and 10-20 C/cm(2), respectively. During a follow up period of two months to three years, 18 (56%) patients had a complete response, and 14 a partial response (44%). Electrochemical treatment is a simple, relatively atraumatic method of dealing with high-flow venous malformations that leaves no scars.

  15. [Endovascular treatment of intracranial arteriovenous malformation with n-butyl cyanoacrylate. Working methods and experience in 58 cases].

    PubMed

    Jordan González, José A; Llibre Guerra, Juan Carlos; Prince López, José A; Vázquez Luna, Frank; Rodríguez Ramos, Raúl Marino; Ugarte Suarez, José Carlos

    2013-01-01

    Arteriovenous malformations (AVMs) constitute malformative lesions of the central nervous system vasculature and cause significant morbidity and mortality. Endovascular embolization with n-butyl cyanoacrylate is a well established modality of AVM treatment, usually combined with surgery or radiosurgery. The purpose of this study was to characterise the AVMs that were treated endovascularly with n-butyl cyanoacrylate and to evaluate the post-embolization results in the Cuban population. From February 2006 to February 2011, a group of 58 consecutive patients with brain AVMs were embolized using n-butyl cyanoacrylate in the endovascular therapy unit of the Medical Surgical Research Centre in Havana (Cuba). In all, 91sessions were carried out with intranidal embolization and mainly partial devascularization, 25-30% per session, and closing 123 arterial pedicles. Safety times for n-butyl cyanoacrylate injection were established by calculating the polymerisation times for different dilutions, using post-embolisation hypotension systematically, as well as a superselective test with propofol to determine cerebral eloquence. Haemorrhagic signs were the initial presentation in 68.8% of the patients, 24.1% presented with epileptic episodes and 1.7% with ischemic stroke. Of the AVMs, 93.2% were supratentorial; according to the Spetzler and Martin classification, 13.8% were grade II, 56.9% were grade III, 22.4% were grade IV and 6.8%, grade V. One hundred and twenty-eight selective tests with propofol were performed and 118 (92.2%) of those were negative. Partial devascularization (20-30%) prevailed; complete obliteration was achieved in 17.2% of the patients and 70%-99% in 27.5% of the patients. Safety times for n-butyl cyanoacrylate injection were established and the use of post-procedure hypotension was settled. Morbidity of 17.2%, with 6.9% haemorrhagic complications and mortality of 3.4% were registered in the whole series. The rates of total occlusion and of morbidity and

  16. Vascular Steal Syndrome, Optic Neuropathy, and Foreign Body Granuloma Reaction to Onyx-18 Embolization for Congenital Orbito-Facial Vascular Malformation

    PubMed Central

    Liu, Catherine Y.; Yonkers, Marc A.; Liu, Tiffany S.; Minckler, Don S.; Tao, Jeremiah P.

    2016-01-01

    A 34-year-old patient presented with a right orbito-facial mass since childhood, consistent with a congenital arteriovenous (AV) malformation. Prior to presentation, she had multiple incomplete surgical resections and embolizations with N-butyl acetyl acrylate and Onyx-18. The patient reported gradual, progressive vision loss shortly after Onyx-18 embolization. Five months after embolization, she presented with decreased vision, disfigurement and mechanical ptosis relating to a large subcutaneous mass affecting the medial right upper eyelid and forehead. Significant exam findings included a visual acuity of 20/400 (20/60 prior to embolization), an afferent pupillary defect, and optic disc pallor. MRI and angiography revealed a persistent AV malformation with feeders from the ophthalmic artery and an absent choroidal flush to the right eye. Pathology from surgical resection showed a significant foreign body giant cell reaction to the embolization material adjacent to the vessels. We suggest that an incomplete embolization with Onyx-18 may have caused vascular steal syndrome from the ophthalmic artery. PMID:27239463

  17. The vascular steal phenomenon is an incomplete contributor to negative cerebrovascular reactivity in patients with symptomatic intracranial stenosis.

    PubMed

    Arteaga, Daniel F; Strother, Megan K; Faraco, Carlos C; Jordan, Lori C; Ladner, Travis R; Dethrage, Lindsey M; Singer, Robert J; Mocco, J; Clemmons, Paul F; Ayad, Michael J; Donahue, Manus J

    2014-09-01

    'Vascular steal' has been proposed as a compensatory mechanism in hemodynamically compromised ischemic parenchyma. Here, independent measures of cerebral blood flow (CBF) and blood oxygenation level-dependent (BOLD) magnetic resonance imaging (MRI) responses to a vascular stimulus in patients with ischemic cerebrovascular disease are recorded. Symptomatic intracranial stenosis patients (n=40) underwent a multimodal 3.0T MRI protocol including structural (T1-weighted and T2-weighted fluid-attenuated inversion recovery) and hemodynamic (BOLD and CBF-weighted arterial spin labeling) functional MRI during room air and hypercarbic gas administration. CBF changes in regions demonstrating negative BOLD reactivity were recorded, as well as clinical correlates including symptomatic hemisphere by infarct and lateralizing symptoms. Fifteen out of forty participants exhibited negative BOLD reactivity. Of these, a positive relationship was found between BOLD and CBF reactivity in unaffected (stenosis degree<50%) cortex. In negative BOLD cerebrovascular reactivity regions, three patients exhibited significant (P<0.01) reductions in CBF consistent with vascular steal; six exhibited increases in CBF; and the remaining exhibited no statistical change in CBF. Secondary findings were that negative BOLD reactivity correlated with symptomatic hemisphere by lateralizing clinical symptoms and prior infarcts(s). These data support the conclusion that negative hypercarbia-induced BOLD responses, frequently assigned to vascular steal, are heterogeneous in origin with possible contributions from autoregulation and/or metabolism.

  18. Factors predicting language lateralization in patients with perisylvian vascular malformations. Clinical article.

    PubMed

    Lee, Darrin J; Pouratian, Nader; Bookheimer, Susan Y; Martin, Neil A

    2010-10-01

    The authors conducted a study to determine the factors associated with right-sided language dominance in patients with cerebrovascular malformations. 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. 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. 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.

  19. 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

  20. 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

  1. Volumetric Modulated Arc-Based Hypofractionated Stereotactic Radiotherapy for the Treatment of Selected Intracranial Arteriovenous Malformations: Dosimetric Report and Early Clinical Experience

    SciTech Connect

    Subramanian, Sai; Srinivas, Chilukuri; Ramalingam, K.; Babaiah, M.; Swamy, S. Thirumalai; Arun, G.; Kathirvel, M.; Ashok, S.; Clivio, Alessandro; Fogliata, Antonella; Nicolini, Giorgia; Rao, K. Srinivasa; Reddy, T. Pratap; Amit, Jotwani; Vanetti, Eugenio; Cozzi, Luca

    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. Delivery 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

  2. Imaging of Spontaneous Ventriculomegaly and Vascular Malformations in Wistar rats: implications for Preclinical Research

    PubMed Central

    Tu, Tsang-Wei; Turtzo, L. Christine; Williams, Rashida A.; Lescher, Jacob D.; Dean, Dana D.; Frank, Joseph A.

    2014-01-01

    Wistar rats are widely used in biomedical research and commonly serve as a model organism in neuroscience studies. In most cases when noninvasive imaging is not utilized, studies assume a consistent baseline condition in rats that lack visible differences. While performing a series of traumatic brain injury studies, we discovered mild spontaneous ventriculomegaly in 70/162 (43.2%) of Wistar rats that had been obtained from 2 different vendors. Advanced magnetic resonance (MR) imaging techniques, including MR angiography and diffusion tensor imaging, were utilized to evaluate the rats. Multiple neuropathologic abnormalities, including presumed arteriovenous malformations, aneurysms, cysts, white matter lesion and astrogliosis were found in association with ventriculomegaly. Postmortem micro-CT and immunohistochemical staining confirmed the presence of aneurysms and arteriovenous malformations. Diffusion tensor imaging significant decreases in fractional anisotropy and increases in mean diffusivity, axial diffusivity, and radial diffusivity in multiple white matter tracts (p < 0.05). These results could impact the interpretation, e.g. of a pseudo-increase of axon integrity and a pseudo-decrease of myelin integrity, based on characteristics intrinsic to rats with ventriculomegaly. We suggest the use of baseline imaging to prevent the inadvertent introduction of a high degree of variability in preclinical studies of neurological disease or injury in the Wistar rats. PMID:25383642

  3. Stereotactic radiosurgery for pediatric patients with intracranial arteriovenous malformations: variables that may affect obliteration time and probability.

    PubMed

    Galván De la Cruz, O O; Ballesteros-Zebadúa, P; Moreno-Jiménez, S; Celis, M A; García-Garduño, O A

    2015-02-01

    It is debatable whether pediatric patients diagnosed with arteriovenous malformations (AVMs) should be treated as adults. Several indexes to classify AVMs have been proposed in the literature, and most try to predict the outcome for each specific treatment. The indexes differ in the variables considered, but they are all based in adult populations. In this study, we analyzed the variables that influence the obliteration time and probability of occurrence in a Mexican pediatric population diagnosed with an AVM and treated with stereotactic radiosurgery (SRS). We analyzed 45 pediatric patients (<18 years) with a minimum follow-up of 10 months and a maximum of 112 months. We used logistic regression analysis and Kaplan-Meier curves to evaluate the influence of age, AVM volume, prescribed dose, minimum dose, maximum dose, time of follow-up, sex, previous hemorrhage, venous drainage, treatment technique, previous treatment and location. We also evaluated the predictive power of the following indexes: Spetzler-Martin, RBAS, or K index dose deviation. We found that the radiation technique used may influence the obliteration occurrence (p=0.057). The data suggests that circular arcs are a more efficient treatment technique than dynamic arcs. However, no relationship of dose or volume with treatment technique could be found. Obliteration was also dependent on follow-up time and after three years of follow-up, the obliteration probability decreases (p=0.024). According to Kaplan-Meier analysis, the nidus obliteration time was related with the location according to the Spetzler-Martin index. If the nidus was located in a non-eloquent region, there was a tendency of a shorter obliteration time (p=0.071). None of the previously proposed indexes for adults predict obliteration in this pediatric population. Treatment technique, eloquence and follow up time were the only variables that showed influence in obliteration. Since the highest probability of obliteration occurs during

  4. 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 (signalmax), time intervals between onset of arterial enhancement and lesion enhancement (tonset), and time intervals between beginning of lesion enhancement and maximum percentage of lesion enhancement (tmax) 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 signalmax 737 %, IQR [interquartile range] = 511 - 1182 %; median tonset 5 s, IQR = 5 - 10 s; median tmax 35 s, IQR = 26 - 40 s) and without AVFs (median signalmax 284 %, IQR = 177-432 %; median tonset 23 s, IQR = 15 - 30 s; median tmax 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.

  5. Expression of CD34 and CD105 as markers for angiogenesis in oral vascular malformations and pyogenic granulomas.

    PubMed

    Vasconcelos, Marcelo Gadelha; Alves, Pollianna Muniz; Vasconcelos, Rodrigo Gadelha; da Silveira, Éricka Janine Dantas; Medeiros, Ana Miryam Costa; de Queiroz, Lélia Maria Guedes

    2011-08-01

    The objective of this study was to assess angiogenic activity by analyzing anti-CD105 and anti-CD34 immunostaining in 20 cases of vascular malformations (VMs) and 20 cases of oral pyogenic granulomas (OPG). In addition, the usefulness of these markers for the differential diagnosis of these two oral tumors was evaluated. The results showed no significant difference in mean microvessel count between the anti-CD105 (P = 0.803) and anti-CD34 (P = 0.279) antibody. The mean number of vessels was 18.75 and 59.72 for oral VMs immunostained with anti-CD105 and anti-CD34 antibody, respectively, whereas in OPG the mean number was 20.22 and 48.09, respectively. CD34 was found to be more effective than CD105 in identifying blood vessels. However, the anti-CD105 antibody seems to be more related to vascular neoformation. Overall, this study supports the role of angiogenic factors in the etiopathogenesis of oral VMs and PG, but the results showed that quantification of angiogenesis cannot be used as a marker for the differential diagnosis of these two types of lesions.

  6. Proteus Syndrome with Arteriovenous Malformation

    PubMed Central

    Asilian, Ali; Kamali, Atefeh Sadat; Riahi, Nabet Tajmir; Adibi, Neda; Mokhtari, Fatemeh

    2017-01-01

    Proteus syndrome is a rare sporadic disorder that appears with localized macrosomia, congenital lipomatosis, and slow flow vascular malformations, connective tissue nevus, and epidermal nevus. There are usually some manifestations at birth. The vascular abnormalities that have been reported in Proteus syndrome are capillary and slow flow venous malformation. We report a case of a 10-year-old boy with confirmed Proteus syndrome characterized by high flow vascular malformation (arteriovenous [AV] malformation) unlike the usual vascular malformations seen in this syndrome. This case adds a new perspective to the established clinical findings of the Proteus syndrome. PMID:28401074

  7. Large laryngeal vascular malformation in a 5-year-old child

    PubMed Central

    Danstrup, Christian Sander; Madsen, Mette Hjørringgaard; Bille, Jesper

    2015-01-01

    A 5-year-old boy was admitted with stridor, which was initially interpreted as subglottic laryngitis. He had a history of prolonged hoarseness and his voice was deep for his age. The stridor persisted despite treatment with epinephrine inhalations and intravenous glucocorticoids. A direct laryngoscopy and blood work up did not support the suspected diagnosis. A MRI was then carried out, followed by angiography. The latter revealed an arteriovenous malformation (AVM) involving the lingual and superior thyroid arteries bilaterally. Owing to the rareness and extent of such an AVM, the patient was referred to Paris for further treatment. This case presents the difficulties in diagnostics and emphasises the importance of diagnostics and multidisciplinary approaches with regard to treatment of AVMs. PMID:25795744

  8. Flow-related intracranial aneurysms associated with unfused arterial twigs relevant to different vascular anomalies: embryologic and hemodynamic considerations.

    PubMed

    Shin, Hee Sup; Lee, Seung Hwan; Ryu, Chang-Woo; Koh, Jun Seok

    2014-09-01

    Cerebrovascular anomalies resulting from the persistence of unfused embryonic twig-like vessels are associated with intracranial aneurysms. All records of patients with ruptured intracranial aneurysms who were treated at our institution were retrospectively reviewed for the presence of aneurysm-associated, unfused, twig-like vessels in the middle cerebral artery (MCA). Such vessels were recorded as twig-like MCA (T-MCA) or twig-like networks of an anomalous collateral artery (T-NACA). Additionally, we sought to characterize vulnerable intracranial aneurysms associated with those vascular anomalies. A total of 442 ruptured aneurysms were treated from June 2006 to November 2013; of these, 4 ruptured aneurysms exhibited the presence of ipsilateral, unfused, twig-like vessels. Computed tomography (CT) scans, three-dimensional CT angiography, and digital subtraction angiography (DSA) were performed immediately after the initial ictus. Data analysis included age, sex, Hunt and Hess grade (HHG), Fisher grade (FG), medical risk factors, angiographic architecture, operative methods and findings, radiologic outcomes, and Glasgow outcome scale (GOS). The average follow-up period was 26 months. Patient ages ranged from 26 to 49 years with a mean age of 41; there were two females and two males. All four patients showed FG IV, and three patients had unfavorable HHG (IV in 2 and V in one) at admission. An M1 segmental occlusion and an adjacent small aneurysmal pouch were detected with three-dimensional CT angiography in three patients. Hypertension was recorded in all patients. The initial DSA revealed T-MCA in one patient and T-NACA in three patients. Six aneurysms in all, including two unruptured aneurysms, were found; three ruptured aneurysms existed inside of the twigs. All but one patient required diverse treatment modalities, and four of the five aneurysms were completely occluded after treatment. The remaining aneurysm, treated only with gluing, disappeared during follow

  9. Large plaque-like glomuvenous malformation (glomangioma) simulating venous malformation.

    PubMed

    Vercellino, N; Nozza, P; Oddone, M; Bava, G L

    2006-07-01

    Glomuvenous malformations and venous malformations are vascular lesions that can be distinguished on the basis of clinical and pathological features. A vascular lesion of the skin and superficial and deep soft tissues of a lower limb in a 5-year-old child is described. The clinical and radiological features, including skeletal muscle involvement, were typical of venous malformation, whereas the histopathological features were those of a glomuvenous malformation. The clinical and histopathological features are briefly discussed.

  10. Crystal Structure of CCM3, a Cerebral Cavernous Malformation Protein Critical for Vascular Integrity

    SciTech Connect

    Li, X.; Zhang, R; Zhang, H; He, Y; Ji, W; Min, W; Boggon, T

    2010-01-01

    CCM3 mutations are associated with cerebral cavernous malformation (CCM), a disease affecting 0.1-0.5% of the human population. CCM3 (PDCD10, TFAR15) is thought to form a CCM complex with CCM1 and CCM2; however, the molecular basis for these interactions is not known. We have determined the 2.5 {angstrom} crystal structure of CCM3. This structure shows an all {alpha}-helical protein containing two domains, an N-terminal dimerization domain with a fold not previously observed, and a C-terminal focal adhesion targeting (FAT)-homology domain. We show that CCM3 binds CCM2 via this FAT-homology domain and that mutation of a highly conserved FAK-like hydrophobic pocket (HP1) abrogates CCM3-CCM2 interaction. This CCM3 FAT-homology domain also interacts with paxillin LD motifs using the same surface, and partial CCM3 co-localization with paxillin in cells is lost on HP1 mutation. Disease-related CCM3 truncations affect the FAT-homology domain suggesting a role for the FAT-homology domain in the etiology of CCM.

  11. Crystal structure of CCM3, a cerebral cavernous malformation protein critical for vascular integrity.

    PubMed

    Li, Xiaofeng; Zhang, Rong; Zhang, Haifeng; He, Yun; Ji, Weidong; Min, Wang; Boggon, Titus J

    2010-07-30

    CCM3 mutations are associated with cerebral cavernous malformation (CCM), a disease affecting 0.1-0.5% of the human population. CCM3 (PDCD10, TFAR15) is thought to form a CCM complex with CCM1 and CCM2; however, the molecular basis for these interactions is not known. We have determined the 2.5 A crystal structure of CCM3. This structure shows an all alpha-helical protein containing two domains, an N-terminal dimerization domain with a fold not previously observed, and a C-terminal focal adhesion targeting (FAT)-homology domain. We show that CCM3 binds CCM2 via this FAT-homology domain and that mutation of a highly conserved FAK-like hydrophobic pocket (HP1) abrogates CCM3-CCM2 interaction. This CCM3 FAT-homology domain also interacts with paxillin LD motifs using the same surface, and partial CCM3 co-localization with paxillin in cells is lost on HP1 mutation. Disease-related CCM3 truncations affect the FAT-homology domain suggesting a role for the FAT-homology domain in the etiology of CCM.

  12. Monro-Kellie 2.0: The dynamic vascular and venous pathophysiological components of intracranial pressure

    PubMed Central

    2016-01-01

    For 200 years, the ‘closed box’ analogy of intracranial pressure (ICP) has underpinned neurosurgery and neuro-critical care. Cushing conceptualised the Monro-Kellie doctrine stating that a change in blood, brain or CSF volume resulted in reciprocal changes in one or both of the other two. When not possible, attempts to increase a volume further increase ICP. On this doctrine’s “truth or relative untruth” depends many of the critical procedures in the surgery of the central nervous system. However, each volume component may not deserve the equal weighting this static concept implies. The slow production of CSF (0.35 ml/min) is dwarfed by the dynamic blood in and outflow (∼700 ml/min). Neuro-critical care practice focusing on arterial and ICP regulation has been questioned. Failure of venous efferent flow to precisely match arterial afferent flow will yield immediate and dramatic changes in intracranial blood volume and pressure. Interpreting ICP without interrogating its core drivers may be misleading. Multiple clinical conditions and the cerebral effects of altitude and microgravity relate to imbalances in this dynamic rather than ICP per se. This article reviews the Monro-Kellie doctrine, categorises venous outflow limitation conditions, relates physiological mechanisms to clinical conditions and suggests specific management options. PMID:27174995

  13. BOLD fMRI integration into radiosurgery treatment planning of cerebral vascular malformations

    SciTech Connect

    Stancanello, Joseph; Cavedon, Carlo; Francescon, Paolo; Causin, Francesco; Avanzo, Michele; Colombo, Federico; Cerveri, Pietro; Ferrigno, Giancarlo; Uggeri, Fulvio

    2007-04-15

    Functional magnetic resonance imaging (fMRI) is used to distinguish areas of the brain responsible for different tasks and functions. It is possible, for example, by using fMRI images, to identify particular regions in the brain which can be considered as 'functional organs at risk' (fOARs), i.e., regions which would cause significant patient morbidity if compromised. The aim of this study is to propose and validate a method to exploit functional information for the identification of fOARs in CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery treatment planning; in particular, given the high spatial accuracy offered by the CyberKnife system, local nonrigid registration is used to reach accurate image matching. Five patients affected by arteriovenous malformations (AVMs) and scheduled to undergo radiosurgery were scanned prior to treatment using computed tomography (CT), three-dimensional (3D) rotational angiography (3DRA), T2 weighted and blood oxygenation level dependent echo planar imaging MRI. Tasks were chosen on the basis of lesion location by considering those areas which could be potentially close to treatment targets. Functional data were superimposed on 3DRA and CT used for treatment planning. The procedure for the localization of fMRI areas was validated by direct cortical stimulation on 38 AVM and tumor patients undergoing conventional surgery. Treatment plans studied with and without considering fOARs were significantly different, in particular with respect to both maximum dose and dose volume histograms; consideration of the fOARs allowed quality indices of treatment plans to remain almost constant or to improve in four out of five cases compared to plans with no consideration of fOARs. In conclusion, the presented method provides an accurate tool for the integration of functional information into AVM radiosurgery, which might help to minimize undesirable side effects and to make radiosurgery less invasive.

  14. Gamma knife radiosurgery for arteriovenous malformations of basal ganglia, thalamus and brainstem--a retrospective study comparing the results with that for AVMs at other intracranial locations.

    PubMed

    Kiran, Narayanam Anantha Sai; Kale, Shashank Sharad; Kasliwal, Manish Kumar; Vaishya, Sandeep; Gupta, Aditya; Singh Sharma, Manish; Shankar Sharma, Bhawani; Kumar Mahapatra, Ashok

    2009-12-01

    The objective of this retrospective study was to study the outcome in patients with basal ganglia, thalamus and brainstem (central/deep) arteriovenous malformations (AVMs) treated with gamma knife radiosurgery (GKS) and to compare the results with that for AVMs at other intracranial locations. The results of 53 patients with central AVMs and 255 patients with AVMs at other locations treated with GKS at our center between April 1997 and March 2005 with minimum follow-up of 1 year were analyzed. CENTRAL AVMS: Forty of these 53 AVMs were Spetzler-Martin grade III, 11 were grade IV, and 2 were grade V. The mean AVM volume was 4.3 cm(3) (range 0.1-36.6 cm(3)). The mean marginal dose given was 23.3 Gy (range 16-25 Gy). The mean follow-up was 28 months (range 12-96 months). Check angiograms were advised at 2 years after GKS and yearly thereafter in the presence of residual AVM till 4 years. Presence of a residual AVM on an angiogram at 4 years after radiosurgery was considered as radiosurgical failure. Complete obliteration of the AVM was documented in 14 (74%) of the 19 patients with complete angiographic follow-up. Significantly lower obliteration rates (37% vs. 100%) were seen in larger AVMs (>3 cm(3)) and AVMs of higher (IV and V) Spetzler-Martin grades (28% vs. 100%). The 3- and 4-year actuarial rates of nidus obliteration were 68% and 74%, respectively. Eight patients (15%) developed radiation edema with a statistically significantly higher incidence in patients with AVM volume >3 cm(3) and in patients with Spetzler-Martin grade IV and V AVMs. Five patients (9.4%) had hemorrhage in the period of latency. COMPARISON OF RESULTS WITH AVMS AT OTHER LOCATIONS: Patients with central AVMs presented at a younger age (mean age 22.7 years vs. 29 years), with a very high proportion (81% vs. 63%) presenting with hemorrhage. Significantly higher incidence of radiation edema (15% vs. 5%) and lower obliteration rates (74% vs. 93%) were seen in patients with central AVMs. GKS is an

  15. Severe holocord syrinx in a child with megalencephaly-capillary malformation syndrome.

    PubMed

    Segal, Devorah; Heary, Robert F; Sabharwal, Sanjeev; Barry, Maureen T; Ming, Xue

    2016-07-01

    The authors present the case of a child with megalencephaly-capillary malformation syndrome who developed a rapidly progressive holocord syringomyelia that was treated surgically. A 3-year-old boy with megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome presented with several months of right leg weakness, worsening scoliosis, and increased seizures. An MRI study of the brain demonstrated a Chiari I malformation and massively dilated syringomyelia extending from C-2 to the conus medullaris. The patient underwent an urgent suboccipital craniectomy with C1-3 laminectomies to relieve the CSF outflow obstruction with significant clinical improvement. Surgery was complicated by bleeding from intracranial vascular malformations. This report describes a very rapidly developing, massive holocord syringomyelia related to CSF obstruction due to an unusual congenital brain malformation and associated vascular overgrowth at the site. Serial, premorbid MRI studies demonstrated the very rapid progression from no Chiari malformation, to progressively greater cerebellar tonsillar herniation, to holocord syrinx. This complication has never been reported in MCAP syndrome and should be considered in any affected MCAP patient with a progressive neurological decline, even if previous spine imaging findings were normal. Surgical complications due to hemorrhage also need to be considered in this vascular brain malformation.

  16. Management of cerebral cavernous malformations in the pediatric population: a literature review and case illustrations.

    PubMed

    Kosnik-Infinger, L; Carroll, C; Greiner, H; Leach, J; Mangano, F T

    2015-09-01

    Cavernous malformations (CM) are vascular malformations of the central nervous system that may occur in the brain and spinal cord. They are one of the four major types of vascular malformations that also includes developmental venous anomalies (DVA)s, arteriovenous malformations (AVMs), and capillary telangiectasias. CMs are a common vascular malformation, and 25% of them occur in the pediatric age group. They can present with acute or chronic symptoms including headache, neurologic deficits secondary to hemorrhage, mass effect, or epilepsy. This review will focus on the neurosurgical management of intracranial cavernous malformations in children. Pediatric CMs have special considerations different from CM that occur in the adult population which are highlighted throughout this review. Characteristics specific to pediatric CM epidemiology, genetics, presentation, pathology, location, size, epilepsy, and management will be discussed. Specific considerations must be entertained with the diagnosis of pediatric CM in that management needs to include consideration of the lifetime risk of hemorrhage, as well as the possibility of development of epilepsy. If in an accessible location, most cavernomas should be surgically removed in a timely fashion to provide lifelong cure for pediatric patients. The review closes with the discussion of two interesting cavernous malformation cases occurring in a 12-year old male and a 12-year old female that exhibit many of the important aspects specific to the management of a pediatric patient with CM, highlighting the importance of a multidisciplinary approach to treatment.

  17. Potential of fluid-attenuated inversion recovery MRI as an alternative to contrast-enhanced MRI for oral and maxillofacial vascular malformations: experimental and clinical studies.

    PubMed

    Sasaki, Yoshinori; Sakamoto, Junichiro; Otonari-Yamamoto, Mika; Nishikawa, Keiichi; Sano, Tsukasa

    2013-10-01

    To determine the potential of fluid-attenuated inversion recovery (FLAIR) imaging of oral and maxillofacial vascular malformations as an alternative to contrast-enhanced magnetic resonance imaging (MRI), we investigated the influence of differences in T1 and T2 values on image contrast in FLAIR images and evaluated the diagnostic utility of such images. FLAIR imaging and heavily T2-weighted (hT2-weighted) imaging were performed using a phantom. FLAIR and hT2-weighted images of 32 lesions (11 mucous cysts, 12 vascular malformations, and 9 tumors) were also studied retrospectively. The contrast-to-noise ratios (CNRs) and CNR change ratios were compared. All aqueous solutions except those with a short T2 value were discriminated by CNR change ratio (P < .05). All 3 types of lesions were discriminated by CNR change ratio (P < .05). FLAIR imaging has potential as an alternative to contrast-enhanced MRI in differentiating vascular malformations from other types of lesions in the oral and maxillofacial region. Copyright © 2013 Elsevier Inc. All rights reserved.

  18. 320-Row Detector Dynamic 4D-CTA for the Assessment of Brain and Spinal Cord Vascular Shunting Malformations. A Technical Note.

    PubMed

    D'Orazio, Federico; Splendiani, Alessandra; Gallucci, Massimo

    2014-12-01

    Shunting vascular malformations of the brain and spinal cord are traditionally studied using digital subtraction angiography (DSA), the current gold standard imaging method routinely used because of its favourable combination in terms of spatial and temporal resolution. Because DSA is relatively expensive, time-consuming and carries a risk of silent embolic events and a small risk of transient or permanent neurologic deterioration, a non-invasive alternative angiographic method is of interest. New 320 row-detector CT scanners allow volumetric imaging of the whole brain with temporal resolution up to ≌ 3 Hz. Those characteristics make computed tomography angiography (CTA) an affordable imaging method to study the haemodynamics of the whole brain and can also be applied to the study of limited portions of the spinal cord. The aim of this paper is to make a brief summary of our experience in studying shunting vascular malformation of the brain and spinal cord using dynamic 4D-CTA, explaining the technical details of the studies performed at our institution, and the state-of-the-art major advantages and drawbacks of this new technique. We found that dynamic 4D-CTA is able to depict the main architectural characteristics of previously untreated vascular shunting malformations both in brain and spinal cord (i.e. their main arterial feeders and draining veins) allowing their correct diagnosis and exhaustive classification, limiting the use of DSA for therapeutic purposes.

  19. Real-time ultrasonography as a monitoring technique for interstitial Nd:YAG laser treatment of voluminous hemangiomas and vascular malformations

    NASA Astrophysics Data System (ADS)

    Werner, Jochen A.; Gottschlich, Stefan; Lippert, Burkard M.; Folz, Benedikt J.

    1998-01-01

    Voluminous vascular anomalies of the head and neck region are still treated with conventional surgery although Neodymium:Yttrium-Aluminum-Garnet (Nd:YAG) laser therapy is an effective treatment method. One hundred thirty give patients with voluminous hemangiomas and vascular malformations were treated with interstitial Nd:YAG laser therapy, partly complemented by a non-contact mode Nd:YAG laser light application. The vascular tumors had a diameter of more than 3 cm in two or all three dimensions. Treatment was carried out under ultrasound and manual control. Nearly 60% of the patients showed a complete clinical regression of the vascular tumor, a third of the patients had a partial regression and were satisfied with the treatment outcome. Four patients were treated unsuccessfully with the laser and three of them subsequently underwent conventional surgery. Only 10 patients showed cosmetic and functional deficits. These results on the interstitial Nd:YAG laser therapy of voluminous hemangiomas and vascular malformations in a large patient group demonstrated the high effectiveness of this novel and innovative therapy modality.

  20. What Is Vascular Disease?

    MedlinePlus

    ... Contact Us Vascular Disease What is Vascular Disease? Education and Awareness Vascular Diseases Abdominal Aortic Aneurysm Aortic Dissection Arteriovenous Malformation Atherosclerosis Buerger's Disease Carotid Artery Disease ...

  1. Vascular Disease Foundation

    MedlinePlus

    ... Contact Us Vascular Disease What is Vascular Disease? Education and Awareness Vascular Diseases Abdominal Aortic Aneurysm Aortic Dissection Arteriovenous Malformation Atherosclerosis Buerger's Disease Carotid Artery Disease ...

  2. Longitudinal MRI Evaluation of Intracranial Development and Vascular Characteristics of Breast Cancer Brain Metastases in a Mouse Model

    PubMed Central

    Zhou, Heling; Chen, Min; Zhao, Dawen

    2013-01-01

    Longitudinal MRI was applied to monitor intracranial initiation and development of brain metastases and assess tumor vascular volume and permeability in a mouse model of breast cancer brain metastases. Using a 9.4T system, high resolution anatomic MRI and dynamic susceptibility contrast (DSC) perfusion MRI were acquired at different time points after an intracardiac injection of brain-tropic breast cancer MDA-MB231BR-EGFP cells. Three weeks post injection, multifocal brain metastases were first observed with hyperintensity on T2-weighted images, but isointensity on T1-weighted post contrast images, indicating that blood-tumor-barrier (BTB) at early stage of brain metastases was impermeable. Follow-up MRI revealed intracranial tumor growth and increased number of metastases that distributed throughout the whole brain. At the last scan on week 5, T1-weighted post contrast images detected BTB disruption in 160 (34%) of a total of 464 brain metastases. Enhancement in some of the metastases was only seen in partial regions of the tumor, suggesting intratumoral heterogeneity of BTB disruption. DSC MRI measurements of relative cerebral blood volume (rCBV) showed that rCBV of brain metastases was significantly lower (mean  = 0.89±0.03) than that of contralateral normal brain (mean  = 1.00±0.03; p<0.005). Intriguingly, longitudinal measurements revealed that rCBV of individual metastases at early stage was similar to, but became significantly lower than that of contralateral normal brain with tumor growth (p<0.05). The rCBV data were concordant with histological analysis of microvascular density (MVD). Moreover, comprehensive analysis suggested no significant correlation among tumor size, rCBV and BTB permeability. In conclusion, longitudinal MRI provides non-invasive in vivo assessments of spatial and temporal development of brain metastases and their vascular volume and permeability. The characteristic rCBV of brain metastases may have a diagnostic value. PMID

  3. [A case of the anterior choroidal artery aneurysm combined with the abnormal intracranial vascular network (author's transl)].

    PubMed

    Takeyama, E; Matsumori, K; Sugimori, T; Kagawa, M

    1976-11-01

    A case of the anterior choroidal artery aneurysm combined with the abnormal intracranial vascular net-work was reported. The patient was 43-year-old male who was attacked by subarachnoid hemorrhage in February 25th 1975. When he was admitted to our clinic 30 days after the ictus, his general status was good and the neurological examination showed no particular findings except only slightly accelerated deep tendon reflexes on the left side. Cerebrospinal fluid still remained xanthochromic although no nuchal rigidity was denoted. Based on the angiographic four vessel studies, the circulatory condition of the patient's brain was summarized as follows; the internal carotid arteries were stenosed or occluded between the C1 and C2 segment on both sides, and abundant collateral circulation was developed mainly around the circle of Willis making an angiographically peculiar vascular net-work in tha base of the brain. Another angiographic finding to be noticed was a berry aneurysm which originated from the distal part of the left anterior choirdal artery. No special treatment was performed on him. He was discharged without any neurological residuals. It would be difficult to find out any hemodynamic relationship between the occlusion of the internal carotid arteries and occurence of the aneurysm. But the abnormally dilated anterior choroidal artery might suggest that the vessel wall of this artery would be burdened in the abnormal distension stress due to the increased transaxial pressure in this artery. Unqder such a hemodynamically stressed state, it would be possible the aneurysm like outpouching of the vessel wall being developed in some fragile portion of the artery functioning as a prominent collateral circulation.

  4. Spontaneous thrombosis of a vein of galen malformation

    PubMed Central

    Mohanty, Chandan B.; Srinivas, Dwarakanath; Sampath, Somanna

    2016-01-01

    Vein of Galen malformation (VOGM) is a rare congenital vascular anomaly, comprising about 1% of all intracranial vascular anomalies, predominantly affecting the children less than 1 year of age. A 6-month-old infant presented with complaints of increasing head size of 3 months duration and multiple episodes of vomiting associated with refusal to feed since 7 days. He was a known case of VOGM who had initially refused treatment. Investigations revealed a spontaneously thrombosed VOGM with obstructive hydrocephalous. Child improved uneventfully with ventriculoperitoneal shunt. Spontaneous thrombosis of a VOGM is a rare occurrence and carries a better prognosis. The relevant literature is discussed with emphasis on etiopathogenesis, mechanism, and management of spontaneous thrombosis of the malformation. PMID:26889287

  5. Feasibility of stereotactic MRI-based image guidance for the treatment of vascular malformations: a phantom study.

    PubMed

    Schwalbe, Marius; Haine, Axel; Schindewolf, Marc; von Tengg-Kobligk, Hendrik; Williamson, Tom; Weber, Stefan; Baumgartner, Iris; Fuss, Torsten

    2016-12-01

    Treatment of vascular malformations requires the placement of a needle within vessels which may be as small as 1 mm, with the current state of the art relying exclusively on two-dimensional fluoroscopy images for guidance. We hypothesize that the combination of stereotactic image guidance with existing targeting methods will result in faster and more reproducible needle placements, as well as reduced radiationexposure, when compared to standard methods based on fluoroscopy alone. The proposed navigation approach was evaluated in a phantom experiment designed to allow direct comparison with the conventional method. An anatomical phantom of the left forearm was constructed, including an independent control mechanism to indicate the attainment of the target position. Three interventionalists (one inexperienced, two of them frequently practice the conventional fluoroscopic technique) performed 45 targeting attempts utilizing the combined and 45 targeting attempts utilizing the standard approaches. In all 45 attempts, the users were able to reach the target when utilizing the combined approach. In two cases, targeting was stopped after 15 min without reaching the target when utilizing only the C-arm. The inexperienced user was faster when utilizing the combined approach and applied significantly less radiation than when utilizing the conventional approach. Conversely, both experienced users were faster when using the conventional approach, in one case significantly so, with no significant difference in radiation dose when compared to the combined approach. This work presents an initial evaluation of a combined navigation fluoroscopy targeting technique in a phantom study. The results suggest that, especially for inexperienced interventionalists, navigation may help to reduce the time and the radiation dose. Future work will focus on the improvement and clinical evaluation of the proposed method.

  6. Systemic Expression of Vascular Endothelial Growth Factor in Patients with Cerebral Cavernous Malformation Treated by Stereotactic Radiosurgery

    PubMed Central

    Park, Sang-Jin

    2016-01-01

    Objective Increased expression of angiogenic factors, such as vascular endothelial growth factor (VEGF), is associated with the pathogenesis of cerebral cavernous malformations (CCMs). The purpose of this study was to investigate plasma levels of VEGF in normal subjects and in patients with CCM and to evaluate change in these levels following stereotactic radiosurgery (SRS). Methods Peripheral venous blood was collected from 6 patients with CCM before SRS using Gamma Knife and at the 1 week, 1 month, 3month, and 6 month follow-up visits. Plasma VEGF levels were measured using commercially available enzyme-linked immunosorbent assay kits. Peripheral blood samples were obtained from 10 healthy volunteers as controls. Results Mean plasma VEGF level of 41.9 pg/mL (range, 11.7–114.9 pg/mL) in patients with CCM at baseline was higher than that of the healthy controls (29.3 pg/mL, range, 9.2–64.3 pg/mL), without significant differences between CCM patients and controls (p=0.828). Plasma VEGF level following SRS dropped to 24.6 pg/mL after 1 week, and decreased to 18.5 pg/mL after 1 month, then increased to 24.3 pg/mL after 3 months, and 32.6 pg/mL after 6 months. Two patients suffering from rebleeding after SRS showed a higher level of VEGF at 6 months after SRS than their pretreatment level. Conclusion Plasma VEGF levels in patients with CCM were elevated over controls at baseline, and decreased from baseline to 1 month after SRS and increased further for up to 6 months. Theses results indicated that anti-angiogenic effect of SRS might play a role in the treatment of CCMs. PMID:27651861

  7. Systemic Expression of Vascular Endothelial Growth Factor in Patients with Cerebral Cavernous Malformation Treated by Stereotactic Radiosurgery.

    PubMed

    Park, Sang-Jin; Park, Seong-Hyun

    2016-09-01

    Increased expression of angiogenic factors, such as vascular endothelial growth factor (VEGF), is associated with the pathogenesis of cerebral cavernous malformations (CCMs). The purpose of this study was to investigate plasma levels of VEGF in normal subjects and in patients with CCM and to evaluate change in these levels following stereotactic radiosurgery (SRS). Peripheral venous blood was collected from 6 patients with CCM before SRS using Gamma Knife and at the 1 week, 1 month, 3month, and 6 month follow-up visits. Plasma VEGF levels were measured using commercially available enzyme-linked immunosorbent assay kits. Peripheral blood samples were obtained from 10 healthy volunteers as controls. Mean plasma VEGF level of 41.9 pg/mL (range, 11.7-114.9 pg/mL) in patients with CCM at baseline was higher than that of the healthy controls (29.3 pg/mL, range, 9.2-64.3 pg/mL), without significant differences between CCM patients and controls (p=0.828). Plasma VEGF level following SRS dropped to 24.6 pg/mL after 1 week, and decreased to 18.5 pg/mL after 1 month, then increased to 24.3 pg/mL after 3 months, and 32.6 pg/mL after 6 months. Two patients suffering from rebleeding after SRS showed a higher level of VEGF at 6 months after SRS than their pretreatment level. Plasma VEGF levels in patients with CCM were elevated over controls at baseline, and decreased from baseline to 1 month after SRS and increased further for up to 6 months. Theses results indicated that anti-angiogenic effect of SRS might play a role in the treatment of CCMs.

  8. 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

  9. Role of intraoperative indocyanine green video-angiography to identify small, posterior fossa arteriovenous malformations mimicking cavernous angiomas. Technical report and review of the literature on common features of these cerebral vascular malformations.

    PubMed

    Barbagallo, Giuseppe M V; Certo, Francesco; Caltabiano, Rosario; Chiaramonte, Ignazio; Albanese, Vincenzo; Visocchi, Massimiliano

    2015-11-01

    To illustrate the usefulness of intraoperative indocyanine green videoangiography (ICG-VA) to identify the nidus and feeders of a small cerebellar AVM resembling a cavernous hemangioma. To review the unique features regarding the overlay between these two vascular malformations and to highlight the importance to identify with ICG-VA, and treat accordingly, the arterial and venous vessels of the AVM. A 36-year old man presented with bilateral cerebellar hemorrhage. MRI was equivocal in showing an underlying vascular malformation but angiography demonstrated a small, Spetzler-Martin grade I AVM. Surgical resection of the AVM with the aid of intraoperative ICG-VA was performed. After hematoma evacuation, pre-resection ICG-VA did not reveal tortuous arterial and venous vessels in keeping with a typical AVM but rather an unusual blackberry-like image resembling a cavernous hemangioma, with tiny surrounding vessels. Such intraoperative appearance, which could also be the consequence of a "leakage" of fluorescent dye from the nidal pathological vessels, with absent blood-brain barrier, into the surrounding parenchymal pathological capillary network, is important to be recognized as an unusual AVM appearance. Post-resection ICG-VA confirmed the AVM removal, as also shown by postoperative and 3-month follow-up DSAs. Despite technical limitations associated with ICG-VA in post-hemorrhage AVMs, this case together with the intraoperative video, demonstrates the useful role of ICG-VA in identifying small AVMs with peculiar features. Copyright © 2015 Elsevier B.V. All rights reserved.

  10. Glomuvenous malformations.

    PubMed

    Henning, J Scott; Kovich, Olympia I; Schaffer, Julie V

    2007-01-27

    A 9-year-old girl presented with a congenital, blue-purple, partially compressible plaque with a cobblestone surface on the left lateral foot and ankle. Similar, solitary, blue nodules later appeared elsewhere on the extremities. The lesions were tender to palpation and were associated with spontaneous paroxysms of pain and paresthesias. Histopathologic evaluation of a skin biopsy specimen showed rows of glomus cells that surrounded thin-walled vascular channels, which confirmed the diagnosis of glomuvenous malformations. This autosomal dominant condition, which is due to mutations in the GLMN gene, presents with clinical findings that are distinct from those of familial, multiple, cutaneous and mucosal venous malformations. Treatment options include excision, sclerotherapy, and laser therapy (ablative or pulsed dye).

  11. Imaging of Intracranial Hemorrhage

    PubMed Central

    Heit, Jeremy J.; Iv, Michael; Wintermark, Max

    2017-01-01

    Intracranial hemorrhage is common and is caused by diverse pathology, including trauma, hypertension, cerebral amyloid angiopathy, hemorrhagic conversion of ischemic infarction, cerebral aneurysms, cerebral arteriovenous malformations, dural arteriovenous fistula, vasculitis, and venous sinus thrombosis, among other causes. Neuroimaging is essential for the treating physician to identify the cause of hemorrhage and to understand the location and severity of hemorrhage, the risk of impending cerebral injury, and to guide often emergent patient treatment. We review CT and MRI evaluation of intracranial hemorrhage with the goal of providing a broad overview of the diverse causes and varied appearances of intracranial hemorrhage. PMID:28030895

  12. Management of vasospasm in ruptured unsecured intracranial vascular lesions: review of 10 cases.

    PubMed

    Serrone, Joseph; Jimenez, Lincoln; Andaluz, Norberto; Abruzzo, Todd A; Zuccarello, Mario; Ringer, Andrew J

    2014-03-01

    Risks associated with endovascular management remain unaddressed for post-hemorrhagic cerebral vasospasm (PHCV) caused by pathologies that cannot be secured or identified before vasospasm treatment. This retrospective study reviews our 10 year experience in the difficult scenario of subarachnoid hemorrhage (SAH) with vasospasm, including intra-arterial vasodilators or percutaneous transluminal angioplasty (PTA) to vessels feeding a ruptured unsecured lesion. 10 SAH patients with ruptured unsecured vascular lesions underwent 44 endovascular treatments for PHCV (2002-2011). We defined unsecured as an untreated aneurysm/dissection, incompletely coiled aneurysm, dissection covered with self-expanding nitinol stents, or angiographically negative SAH. Treatments were categorized by location of the ruptured unsecured (partial or complete) lesion relative to the vessel treated for vasospasm. Our 10 patients with four aneurysms, four dissections, and two angiographically negative SAH accounted for 10.3% of SAH patients who underwent angiography for vasospasm. No procedure related complications occurred when treating vessels not supplying the index lesion or with angiographically negative SAH. Of the endovascular treated vessels supplying partially secured lesions, one (6.3%) fatal complication occurred; none of these patients receiving only vasodilators had complications. With endovascular treatment of PHCV with completely unsecured lesions, one (33%) complication was fatal. Endovascular treatment appeared safe for PHCV for vessels not supplying the index arterial lesion and for angiographically negative SAH. Vasodilators were safe for vessels harboring partially secured, ruptured lesions (eg, incompletely coiled aneurysms, stented dissections). Following two major complications, the safety of administering vasodilators or performing PTA to vessels supplying completely unsecured vascular lesions remains inconclusive and should be used cautiously.

  13. Rho kinase as a target for cerebral vascular disorders

    PubMed Central

    Bond, Lisa M; Sellers, James R; McKerracher, Lisa

    2015-01-01

    The development of novel pharmaceutical treatments for disorders of the cerebral vasculature is a serious unmet medical need. These vascular disorders are typified by a disruption in the delicate Rho signaling equilibrium within the blood vessel wall. In particular, Rho kinase overactivation in the smooth muscle and endothelial layers of the vessel wall results in cytoskeletal modifications that lead to reduced vascular integrity and abnormal vascular growth. Rho kinase is thus a promising target for the treatment of cerebral vascular disorders. Indeed, preclinical studies indicate that Rho kinase inhibition may reduce the formation/growth/rupture of both intracranial aneurysms and cerebral cavernous malformations. PMID:26062400

  14. Intracranial microvascular free flaps.

    PubMed

    Levine, Steven; Garfein, Evan S; Weiner, Howard; Yaremchuk, Michael J; Saadeh, Pierre B; Gurtner, Geoffrey; Levine, Jamie P; Warren, Stephen M

    2009-02-01

    Large acquired intracranial defects can result from trauma or surgery. When reoperation is required because of infection or tumor recurrence, management of the intracranial dead space can be challenging. By providing well-vascularized bulky tissue, intracranial microvascular free flaps offer potential solutions to these life-threatening complications. A multi-institutional retrospective chart and radiographic review was performed of all patients who underwent microvascular free-flap surgery for salvage treatment of postoperative intracranial infections between 1998 and 2006. A total of six patients were identified with large intracranial defects and postoperative intracranial infections. Four patients had parenchymal resections for tumor or seizure and two patients had posttraumatic encephalomalacia. All patients underwent operative debridement and intracranial free-flap reconstruction using the latissimus dorsi muscle (N=2), rectus abdominis muscle (N=2), or omentum (N=2). All patients had titanium (N=4) or Medpor (N=2) cranioplasties. We concluded that surgery or trauma can result in significant intracranial dead space. Treatment of postoperative intracranial infection can be challenging. Vascularized free tissue transfer not only fills the void, but also provides a delivery system for immune cells, antibodies, and systemically administered antibiotics. The early use of this technique when intracranial dead space and infection coexist is beneficial.

  15. Selection of Endovascular Approach of Vein of Galen Aneurysmal Malformation

    PubMed Central

    Ito, O.; Goto, K.; Ogata, N.; Utsunomiya, H.; Sato, S.; Fukumura, A.

    2001-01-01

    Summary Vein of Galen aneurysmal malformation (VGAM) is one of the most difficult intracranial vascular lesions because this disease consists of extremely high flow shunts and affects infants and small children. Thanks to the development of various diagnostic modalities, early diagnosis became possible allowing us to prepare appropriately according to the patients' general and neurological conditions. Recent improvements of endovascular techniques and materials enabled both transarterial and transvenous approaches even to the newborn infants, widening therapeutic windows. In this article, we discuss the selection of endovascular approaches based upon angioarchitecture of VGAM presenting four representative cases from our file. PMID:20663400

  16. Cardiac arteriovenous malformation causing sudden death.

    PubMed

    Aguilera, Beatriz; Suárez-Mier, M Paz; Argente, Trinidad

    2004-01-01

    Cardiac vascular malformations are rare. We report a subendocardial arteriovenous malformation (AVM), associated with extensive myocardial fibrosis, causing sudden death in a 25-year-old woman. To our knowledge, this is the first autopsy case reported.

  17. [Congenital anomalies of cerebral artery and intracranial aneurysm].

    PubMed

    Nakajima, K; Ito, Z; Hen, R; Uemura, K; Matsuoka, S

    1976-02-01

    It is well known that congenital anomalies such as polycystic kidney, aortic coarctation, Marfan syndrome, Ehler-Danlos syndrome are apt to be complicated by intracranial aneurysms. In this report we attempt to reveal the relation and incidence between cerebrovascular anomalies and intracranial aneurysms. The etiology of aneurysms has been discussed, too. 12 cases of persistent trigeminl artery, 2 cases of persistent hypoglossal artery and 11 cases of fenestration were obtained from 3841 patients who were angiographically examined in our clinic for 5 years. The incidence is 0.31%, 0.05% and 0.29%, respectively. Persistent trigeminal arteries were complicated by 2 cases of intracranial aneurysms and one case of arterivenous malformations (AVM), persistent hypoglossal arteries were complicated by one case of aneurysm, and fenestrations were complicated by 2 cases of aneurysms and one case of AVM. One case of congenital agenesis of right internal carotid artery was obtained which was complicated by aneurysm of anterior communicating artery. Totally, 8 cases of aneurysms and AVM were obtained from 26 cases of cerebrovascular anomalies (incidence 30.8%). On the other hand, thalamic or caudate hemorrhage revealed the highest incidence of complication of intracranial aneurysms among intracerebral hematomas (10.7%). Compared with the incidence of aneurysms between cerebro vascular anomalies (30.8%) and thalamic or caudate hemorrhage (10.7%), the difference is statistically signigicant (P less than 0.05). The cause of intracranial aneurysm has not yet been clarified. But it is well accepted that the defect of tunica media vasorum is most responsible factor as to the occurrence of intracranial aneurysms. We concluded that the genetic error of cerebral vessels including defect of media caused intracranial aneurysms, and this result was supported from the evidence that cerebrovascular anomalies showed statistically high incidence of complication of intracranial aneurysms.

  18. Assessment of Cerebral Blood Flow with Micro-Doppler Vascular Reduces the Risk of Ischemic Stroke During the Clipping of Intracranial Aneurysms.

    PubMed

    Pereira, Benedito J A; Holanda, Vanessa M; Giudicissi-Filho, Miguel; Borba, Luiz Alencar B; de Holanda, Carlos Vanderlei M; de Oliveira, Jean G

    2015-12-01

    To analyze the impact of the introduction of Micro-Doppler vascular (MDV) as a method of cerebral blood flow analysis during microsurgical clipping of intracranial aneurysms to check the partial occlusion of the aneurysm and the occurrence of stenosis by comparing these results with those provided by the postoperative digital subtraction angiography (DSA) scan as well as the occurrence of ischemic infarction on the postoperative computed tomography (CT) images. We reviewed retrospectively the last 50 patients operated on before the introduction of the MDV (group 1) compared with the first 50 patients operated on using this technique (group 2). Nine (18%) of the 50 patients evaluated in the group 1 showed a new hypodensity in the postoperative CT images, whereas only 2 (4%) patients showed infarction in the group 2 (P = 0.02). In addition, in the group 1, 10 (20%) patients presented unexpected findings on DSA images (residual aneurysms, stenosis, and arterial occlusion), whereas in the group 2, those unexpected DSA findings were observed in only 3 (6%) patients (P = 0.023). MDV is an excellent method for cerebral blood flow assessment during the microsurgical clipping of intracranial aneurysms, reducing the unexpected angiographic results (residual aneurysms, stenosis, and arterial occlusion), as well as reducing the incidence of ischemic infarction on postoperative CT images, evidence of the positive impact of this method in the microsurgical treatment of intracranial aneurysms. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Intracranial-to-intracranial vascular anastomosis created using a microanastomotic device for the treatment of distal middle cerebral artery aneurysms. Technical note.

    PubMed

    Newell, David W; Schuster, James M; Avellino, Anthony M

    2002-08-01

    The use of a microanastomotic device for direct connection of intracranial vessels can be helpful to facilitate removal of distally located middle cerebral artery (MCA) aneurysms. The authors report on two patients who presented for treatment with large aneurysms distally located on the MCA. The aneurysms were completely excised and the proximal and distal portions of the parent vessel were connected in an end-to-end fashion by using a microanastomotic device. The time required to crossclamp the vessel for excision of the aneurysm and primary anastomosis was 10 minutes in one case and 15 minutes in the other. The short crossclamp time and high-quality anastomosis afforded by this device may be useful in the treatment of these difficult lesions and the prevention of cerebral ischemia.

  20. Intracranial Vascular Treatments

    MedlinePlus

    ... an imaging area where a magnetic resonance imaging (MRI) scan will be performed to show the exact location ... performed instead of, or in addition to, an MRI scan. If you are having treatment for an arteriovenous ...

  1. Intracranial Vascular Treatments

    MedlinePlus

    ... information. top of page How does the procedure work? Endovascular therapy: Embolization: Using x-ray imaging and ... radiotherapy using a relocatable frame, which is an advantage for large tumors or particularly critical locations. For ...

  2. Sulindac metabolites decrease cerebrovascular malformations in CCM3-knockout mice.

    PubMed

    Bravi, Luca; Rudini, Noemi; Cuttano, Roberto; Giampietro, Costanza; Maddaluno, Luigi; Ferrarini, Luca; Adams, Ralf H; Corada, Monica; Boulday, Gwenola; Tournier-Lasserve, Elizabeth; Dejana, Elisabetta; Lampugnani, Maria Grazia

    2015-07-07

    Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe neurological consequences. At present, the only recommended treatment of CCM is surgical. Because surgery is often not applicable, pharmacological treatment would be highly desirable. We describe here a murine model of the disease that develops after endothelial-cell-selective ablation of the CCM3 gene. We report an early, cell-autonomous, Wnt-receptor-independent stimulation of β-catenin transcription activity in CCM3-deficient endothelial cells both in vitro and in vivo and a triggering of a β-catenin-driven transcription program that leads to endothelial-to-mesenchymal transition. TGF-β/BMP signaling is then required for the progression of the disease. We also found that the anti-inflammatory drugs sulindac sulfide and sulindac sulfone, which attenuate β-catenin transcription activity, reduce vascular malformations in endothelial CCM3-deficient mice. This study opens previously unidentified perspectives for an effective pharmacological therapy of intracranial vascular cavernomas.

  3. Sulindac metabolites decrease cerebrovascular malformations in CCM3-knockout mice

    PubMed Central

    Bravi, Luca; Rudini, Noemi; Cuttano, Roberto; Giampietro, Costanza; Maddaluno, Luigi; Ferrarini, Luca; Adams, Ralf H.; Corada, Monica; Boulday, Gwenola; Tournier-Lasserve, Elizabeth; Dejana, Elisabetta; Lampugnani, Maria Grazia

    2015-01-01

    Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe neurological consequences. At present, the only recommended treatment of CCM is surgical. Because surgery is often not applicable, pharmacological treatment would be highly desirable. We describe here a murine model of the disease that develops after endothelial-cell–selective ablation of the CCM3 gene. We report an early, cell-autonomous, Wnt-receptor–independent stimulation of β-catenin transcription activity in CCM3-deficient endothelial cells both in vitro and in vivo and a triggering of a β-catenin–driven transcription program that leads to endothelial-to-mesenchymal transition. TGF-β/BMP signaling is then required for the progression of the disease. We also found that the anti-inflammatory drugs sulindac sulfide and sulindac sulfone, which attenuate β-catenin transcription activity, reduce vascular malformations in endothelial CCM3-deficient mice. This study opens previously unidentified perspectives for an effective pharmacological therapy of intracranial vascular cavernomas. PMID:26109568

  4. Undersized angioplasty and stenting of symptomatic intracranial tight stenosis with Enterprise: Evaluation of clinical and vascular outcome

    PubMed Central

    Lee, Kun-Yu; Chen, David Yen-Ting; Hsu, Hui-Ling; Chen, Chi-Jen

    2015-01-01

    Background Severe intracranial arterial stenosis results in more than 10% incidence of stroke and transient ischemic attack. Using undersized angioplasty with off-label closed-cell Enterprise stent may be a feasible alternative option for treating patients with intracranial atherosclerotic disease who fail dual-antiplatelet medical therapy. The results of the authors’ study are presented in this paper. Materials and methods Between January 2013 and July 2014, 24 symptomatic patients with a total of 30 intracranial arterial stenotic lesions refractory to medical therapy, who underwent undersized angioplasty and Enterprise stenting, were retrospectively reviewed in the authors’ institution. The results evaluated include technical success rate, clinical outcome measured as modified Rankin Scale at presentation and follow-up, peri-procedural morbidity within 30 days and 1 year, and follow-up vessel patency. Results Stent deployment was successfully achieved in all stenotic lesions (30/30). Mean pre-stent and post-stent diameter residual stenosis was 81% and 18%, respectively. The peri-procedural complication rate during 30 days after stenting was 10% per lesion (3/30), including intracranial hemorrhage, in-stent thrombosis and ischemic stroke. No further thromboembolic event or complication occurred in any patient more than 30 days after stenting. Modified Rankin scale ≤ 2 was observed in 64% and 83% of patients at initial presentation and follow-up (mean 15.8 months), respectively. Imaging follow-up was available in 17 of 24 patients (70.8%) and 20 of 30 treated lesions (66.6%) with a mean follow-up period of 15.4 months. Only one asymptomatic in-stent restenosis occurred in 20 available lesions (5.0%). Conclusion This preliminary study suggests that using undersized angioplasty and Enterprise stenting may effectively treat high-degree symptomatic intracranial arterial stenosis with favorable clinical and angiographic outcome. PMID:26542728

  5. [Hemangioma and superficial arteriovenous malformations].

    PubMed

    Brevière, G M; Piette, F; Beregi, J P; Rey, C

    1999-05-01

    Haemangiomas are different from true superficial vascular malformations. The haemangiomas, mainly affecting the newborn and small babies, will, after a phase of progression, sometimes regress completely. Therapeutic abstention is the rule except in high risk angiomas when steroid therapy may be effective. Visceral involvement poses problems. Superficial vascular malformations, on the other hand, arise at all ages and may affect any blood vessel. Each type has a specific clinical presentation, complementary investigations and appropriate treatment. Some are slowly progressive, for example capillary, venous and lymphatic malformations. Others are haemodynamically active, such as the arteriovenous malformations. Capillary malformations are flat angiomas with aesthetic consequences, apart from the Sturge-Weber-Krabbe syndrome. Cold, blue venous malformations confirmed by ultrasonography and magnetic resonance imaging, when necessary, require treatment adapted to their site and size: compression, embolisation, surgery or abstention. Lymphatic malformations may be cystic or tissular: the cystic lymphangioma, a soft swelling of often healthy skin, with compartments separated by septa on ultrasound scan, is usually treated by ethibloc embolisation. Arteriovenous malformations, warm and pulsatile, demonstrated at arteriography, may progress rapidly and treatment by surgery or embolisation, when necessary, has to be complete. Finally, there are complex vascular malformations which pose very difficult problems of management.

  6. A locus for cerebral cavernous malformations maps to chromosome 7q in two families

    SciTech Connect

    Marchuk, D.A.; Gallione, C.J.; Morrison, L.A.; Davis, L.E.; Clericuzio, C.L.

    1995-07-20

    Cavernous malformations (angiomas) affecting the central nervous system and retina can be inherited in autosomal dominant pattern (OMIM 116860). These vascular lesions may remain clinically silent or lead to a number of neurological symptoms including seizure, intracranial hemorrhage, focal neurological deficit, and migraine. We have mapped a gene for this disorder in two families, one of Italian-American origin and one of Mexican-American origin, to markers on proximal 7q, with a combined maximum lod score of 3.92 ({theta} of zero) with marker D7S479. Haplotype analysis of these families places the locus between markers D7S502 proximally and D7S515 distally, an interval of approximately 41 cM. The location distinguishes this disorder from an autosomal dominant vascular malformation syndrome where lesions are primarily cutaneous and that maps to 9p21. 16 refs., 3 figs., 1 tab.

  7. Use of a Polytetrafluoroethylene-Coated Vascular Plug for Focal Intracranial Parent Vessel Sacrifice for Fusiform Aneurysm Treatment.

    PubMed

    Carlson, Andrew P; Abbas, Mohammad; Hall, Patricia; Taylor, Christopher

    2017-10-01

    Fusiform intracranial aneurysms are challenging due to the circumferential nature of the disease. Endovascular parent vessel sacrifice with coils may be a treatment option, but typically requires a long vessel segment to induce complete cessation of flow.   We evaluate early clinical experience with the intracranial use of the microvascular plug (MVP; Medtronic, Dublin, Ireland) device and to compare to previous coil-only techniques for vertebral artery sacrifice for fusiform vertebral aneurysm. We reviewed patients treated with the MVP for intracranial aneurysms at our institution. As a case-control study, we located 6 control patients who underwent coiling alone for vertebral artery sacrifice. The number of implants, fluoroscopy time, and procedural charges were compared using unpaired t -tests. Twelve patients underwent vessel sacrifices with MVP. Eight were for vertebral artery dissecting aneurysms. Comparing only vertebral aneurysms, the mean implants was 7 in the MVP group (n = 8) and 19.5 in the coiling group (n = 6; P = .0015). Mean fluoroscopy time was 17.62 min in the MVP group compared to 24.2 min in the coiling group ( P = .07). Procedural costs were less in the MVP group ($19 667.38) compared to coiling ($44 909.50, P = .05). There were no technical failures and no cases with persistent flow in the parent vessel at the end of the procedure. The MVP is a cost-effective device for focal intracranial vessel occlusion in select patients. This is an important tool for cerebrovascular surgeons, particularly in cases of ruptured dissecting vertebral aneurysms.

  8. Hypoxia-inducible factor and vascular endothelial growth factor are expressed more frequently in embolized than in nonembolized cerebral arteriovenous malformations.

    PubMed

    Sure, Ulrich; Battenberg, Elmar; Dempfle, Astrid; Tirakotai, Wuttipong; Bien, Siegfried; Bertalanffy, Helmut

    2004-09-01

    In previous studies, we documented a marked neoangiogenesis and endothelial proliferation in cerebral arteriovenous malformations (AVMs) that were embolized before surgery compared with those that were not embolized. We hypothesized that embolization caused a local hypoxia that promotes neoangiogenesis as a possible pathomechanism. To support this hypothesis, we now examined the angiogenesis-related proteins in a larger cohort of patients. In addition, we investigated hypoxia-inducible factor-1 alpha as a possible protein operative during neoangiogenesis of cerebral AVMs. Paraffin-embedded specimens of 56 AVMs obtained from surgical resection and 14 brain tissue controls were immunohistochemically stained with antibodies to proliferating cell nuclear antigen, MIB-1, vascular endothelial growth factor, Flk1, and hypoxia-inducible factor-1 alpha by standard protocols. In AVMs treated with embolization before surgery (n = 35, 63%), the expression of hypoxia-inducible factor-1 alpha (P = 0.0101) and vascular endothelial growth factor (P = 0.0007) was significantly higher (Fisher's exact test) than in patients who did not have previous endovascular treatment. Differences in the expression of Flk-1 (P = 0.0798) and proliferating cell nuclear antigen (P = 0.0423) were in the same direction but were not significant when corrected for multiple testing. Our results provide circumstantial evidence that a partial occlusion of cerebral AVMs might induce local hypoxia-related neoangiogenesis. To support these data, future animal studies should be performed.

  9. MR imaging of cerebrospinal fluid dynamics in health and disease. On the vascular pathogenesis of communicating hydrocephalus and benign intracranial hypertension.

    PubMed

    Greitz, D; Hannerz, J; Rähn, T; Bolander, H; Ericsson, A

    1994-05-01

    The CSF flows in the aqueduct and at the foramen magnum were examined in 5 patients with communicating hydrocephalus (HC) and in 10 with benign intracranial hypertension (BIH) as well as in 5 healthy volunteers. As compared to normal individuals, the aqueductal flow in HC was about 10 times larger and the cervical flow was half as large. In BIH the CSF flows were not different from those of normal volunteers. The decreased arterial expansion as reflected in the reduced cervical flow in HC may be due to pathologic changes in the arteries and paravascular spaces. The large aqueductal flow in HC reflects a large brain expansion, causing increased transcerebral mantle pressure gradient and ventricular dilatation. In BIH there is a normal brain expansion (aqueductal flow) and consequently no ventricular dilatation. It is argued that BIH be caused by an obstruction on the venous side, as opposed to the vascular alterations in HC, which are on the arterial side.

  10. 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.

  11. Percutaneous Image-Guided Cryoablation as Second-Line Therapy of Soft-Tissue Venous Vascular Malformations of Extremities: A Prospective Study of Safety and 6-Month Efficacy.

    PubMed

    Cornelis, F H; Labrèze, C; Pinsolle, V; Le Bras, Y; Castermans, C; Bader, C; Thiebaut, R; Midy, D; Grenier, N

    2017-09-01

    To report the safety and short-term efficacy of percutaneous image-guided cryoablation performed as second-line therapy of venous vascular malformations (VVM) of extremities. In this non-blinded, no-randomized trial, cryoablation was proposed in 14 patients presenting with symptomatic VVM for recurrences after treatment. Eligibility criteria were: cryoablation feasible, localization at least 5 mm from skin and nerves, absence of contra-indication for anesthesia. Safety was evaluated by the common terminology criteria for adverse events (AE). Clinical response was assessed by evaluating pain at day 7, month 2 and 6 using visual analog scale; quality of life before cryoablation and at 2 and 6 months after using questionnaire. Evolution of volume was evaluated by MRI at 6 months. Comparison was performed using the Wilcoxon test. A technical success was observed in all cases. While 11 patients (78.6%) presented AE (13 grade 1-2 and 3 grade 3), only two severe AE (grade 3) related to cryoablation occurred in two patients (14.3%) during the 6-month follow-up: one immediate sciatic paralysis and one delayed paresthesia. A clinical response was observed in 12 patients (85.7%) at 6 months. Pain decreased significantly from 42.5 ± 14.2 mm before the intervention to 11.8 ± 17.9 mm at 6 months (P = 0.002). A significant decrease in the mean volume from 12.8 ± 14.3 to 3 ± 2.7 cm(3) was observed at 6 months (P = 0.002). Percutaneous cryoablation is a promising alternative treatment for sclerotherapy-resistant venous malformations. However, to improve safety, careful patient selection and treatment planning will be mandatory.

  12. Intracranial endoscopy.

    PubMed

    Schroeder, H W; Gaab, M R

    1999-04-15

    The authors' intention is to reduce the invasiveness of intracranial procedures while avoiding traumatization of brain tissue, to decrease the risk of neurological and mental deficits. Intracranial endoscopy is a minimally invasive technique that provides rapid access to the target via small burr holes without the need for brain retraction. Craniotomy as well as microsurgical brain splitting and dissection can often be avoided. Furthermore, because obstructed cerebrospinal fluid pathways can be physiologically restored, the need for shunt placement is eliminated. The ventricular system and subarachnoid spaces provide ideal conditions for the use of an endoscope. Therefore, a variety of disorders, such as hydrocephalus, small intraventricular lesions, and arachnoid and parenchymal cysts can be effectively treated using endoscopic techniques. With the aid of special instruments, laser fibers, and bipolar diathermy, even highly vascularized lesions such as cavernomas may be treated. Moreover, during standard microsurgical procedures, the endoscopic view may provide valuable additional information ("looking around a corner") about the individual anatomy that is not visible with the microscope. In transsphenoidal pituitary surgery, transseptal dissection can be avoided if an endonasal approach is taken. In the depth of the intrasellar space, the extent of tumor removal can be more accurately controlled, especially in larger tumors with para- and suprasellar growth. The combined use of endoscopes and computerized neuronavigation systems increases the accuracy of the approach and provides real-time control of the endoscope tip position and approach trajectory. In the future, the indications for neuroendoscopy will certainly expand with improved technical equipment.

  13. [Intraoperative fluid therapy in infants with congestive heart failure due to intracranial pial arteriovenous fistula].

    PubMed

    Arroyo-Fernández, F J; Calderón-Seoane, E; Rodríguez-Peña, F; Torres-Morera, L M

    2016-05-01

    Pial arteriovenous fistula is a rare intracranial congenital malformation (0.1-1: 100,000). It has a high blood flow between one or more pial arteries and drains into the venous circulation. It is usually diagnosed during the childhood by triggering an intracranial hypertension and/or congestive heart failure due to left-right systemic shunt. It is a rare malformation with a complex pathophysiology. The perioperative anaesthetic management is not well established. We present a 6-month-old infant diagnosed with pial arteriovenous fistula with hypertension and congestive heart failure due to left-right shunt. He required a craniotomy and clipping of vascular malformation. Anaesthetic considerations in patients with this condition are a great challenge. It must be performed by multidisciplinary teams with experience in paediatrics. The maintenance of blood volume during the intraoperative course is very important. Excessive fluid therapy can precipitate a congestive heart failure or intracranial hypertension, and a lower fluid therapy may cause a tissue hypoxia due to the bleeding. Copyright © 2015 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

  14. Endovascular Balloon-Assisted Embolization of Intracranial and Cervical Arteriovenous Malformations Using Dual Lumen Co-axial Balloon Microcatheters and Onyx: Initial Experience.

    PubMed

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

    2013-02-25

    BACKGROUND:: Ethylene vinyl alcohol co-polymer (Onyx) is widely used for the embolization of arteriovenous malformations of the brain, head, and neck. Balloon-assisted Onyx embolization may provide additional unique advantages in the treatment of AVMs when compared to traditional catheter-based techniques. OBJECTIVE:: To report our initial experience in performing balloon-assisted AVM embolization for brain and neck AVMs using the new Scepter-C and Scepter-XC co-axial dual lumen balloon microcatheters. METHODS:: Balloon-assisted trans-arterial embolization was carried out in a series of seven patients with AVMs (4 with brain AVMs, one with a dural AVF, and two with neck AVMs) using Onyx delivered through the lumen of Scepter-C or Scepter XC co-axial balloon microcatheters. Following the initial balloon-catheter navigation into a feeding artery and the subsequent inflation of the balloon, the embolization was performed using Onyx 18, Onyx 34, or both. RESULTS:: A total of twelve embolization sessions were performed via 17 arterial feeders in these 7 patients. In one patient, there was an arterial perforation from inflation of the balloon; in all others, the embolization goals were successfully achieved with no adverse events. CONCLUSION:: 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 to conventional embolization methods.

  15. Safety and Efficacy Study of Sirolimus in Complicated Vascular Anomalies

    ClinicalTrials.gov

    2015-02-03

    Kaposiform Hemangioendotheliomas; Tufted Angioma; Capillary Venous Lymphatic Malformation; Venous Lymphatic Malformation; Microcystic Lymphatic Malformation; Mucocutaneous Lymphangiomatosis and Thrombocytopenia; Capillary Lymphatic Arterial Venous Malformations; PTEN Overgrowth Syndrome With Vascular Anomaly; Lymphangiectasia Syndromes

  16. Comprehensive Treatment of Primary Orbital Arteriovenous Malformation.

    PubMed

    Xie, Jizi; Xu, Shiqiong; Shi, Yinyun; Li, Tianyuan; Jia, Renbing; Fan, Xianqun

    2017-09-01

    Primary orbital arteriovenous malformations are a rare kind of vascular malformation lesions. The authors present a 17-year-old man presented with swelling and pulsation in the left upper eyelid. The angiogram of the left internal carotid artery showed that arteriovenous malformations at the left upper eyelid area were supplied with one of the branches of ophthalmic artery. In this report, the authors elaborated the comprehensive treatments of primary arteriovenous malformation.

  17. Cerebral venous etiology of intracranial hypertension and differentiation from idiopathic intracranial hypertension.

    PubMed

    Iencean, Stefan Mircea; Poeata, Ion; Iencean, Andrei Stefan; Tascu, Alexandru

    2015-03-01

    This study presents the characteristics that distinguish between idiopathic intracranial hypertension (ICH) and ICH caused by intracranial vascular damage. Twenty-one patients with ICH were included in this study. The analysis of the symptomatology correlated with the values of intracranial pressure, and the imaging findings revealed significant differences between these two types of ICH. ICH caused by intracranial venous vascular damage is named vascular ICH. Vascular ICH has a known etiology, such as cerebral vascular illness, and a relatively rapid increase in intracranial pressure of approximately 21 cmH2O and imaging findings show characteristic images of thrombosis or stenosis of the intracranial venous system, while all brain images (computed tomography, magnetic resonance imaging, angio-magnetic resonance imaging) are normal in idiopathic ICH. The treatment of vascular ICH is etiologic, pathogenic, and symptomatic, but that of idiopathic ICH is only symptomatic. Copyright © 2015. Published by Elsevier Taiwan.

  18. Relative risk of hemorrhage during pregnancy in patients with brain arteriovenous malformations.

    PubMed

    van Beijnum, Janneke; Wilkinson, Tim; Whitaker, Heather J; van der Bom, Johanna G; Algra, Ale; Vandertop, W Peter; van den Berg, René; Brouwer, Patrick A; Rinkel, Gabriël Je; Kappelle, L Jaap; Al-Shahi Salman, Rustam; Klijn, Catharina Jm

    2017-10-01

    Background It is unclear whether the risk of bleeding from brain arteriovenous malformations is higher during pregnancy, delivery, or puerperium. We compared occurrence of brain arteriovenous malformation hemorrhage in women during this period with occurrence of hemorrhage outside this period during their fertile years. Methods We included all women with ruptured brain arteriovenous malformations (16-41 years) from a retrospective database of patients with brain arteriovenous malformations in four Dutch university hospitals (n = 95) and from the population-based Scottish Audit of Intracranial Vascular Malformations (n = 44). We estimated the relative rate of brain arteriovenous malformation rupture (before any treatment) during exposed time (pregnancy, delivery, puerperium) versus non-exposed time during fertile years, using the case-crossover design as primary analysis, and the self-controlled case-series design as secondary analysis. Results In 17 of 95 Dutch women and in 3 of 44 Scottish women, hemorrhages occurred while pregnant; none occurred during delivery or puerperium. In Dutch women, the relative rate of brain arteriovenous malformation rupture during pregnancy, delivery, or puerperium was 6.8 (95% confidence interval 3.6-13) according to the case-crossover method and 7.1 (95% confidence interval 3.4-13) using the self-controlled case-series method. In Scottish women, the relative rate was 1.3 (95% confidence interval 0.39-4.1) using the case-crossover method and 1.7 (95% confidence interval 0.0-4.4) according to the self-controlled case-series method. Because of limited overlap of confidence intervals, we refrained from pooling the cohorts. Conclusions Case-crossover and self-controlled case series analyses reveal an increase in relative rate of brain arteriovenous malformation rupture during pregnancy in the Dutch cohort but not in the Scottish cohort. Since point estimates varied between both cohorts and numbers are relatively small, the

  19. The relationship of cortical folding and brain arteriovenous malformations

    PubMed Central

    Shah, Manish N.; Smith, Sarah E.; Dierker, Donna L.; Herbert, Joseph P.; Coalson, Timothy S.; Bruck, Brent S.; Zipfel, Gregory J.; Van Essen, David C.; Dacey, Ralph G.

    2016-01-01

    Background The pathogenesis of human intracranial arteriovenous malformations (AVMs) is not well understood; this study aims to quantitatively assess cortical folding in patients with these lesions. Methods Seven adult participants, 4 male and 3 female, with unruptured, surgically unresectable intracranial AVMs were prospectively enrolled in the study, with a mean age of 42.1 years and Spetzler-Martin grade range of II–IV. High-resolution brain MRI T1 and T2 sequences were obtained. After standard preprocessing, segmentation and registration techniques, three measures of cortical folding, the depth difference index (DDI), coordinate distance index (CDI) and gyrification index (GI)), were calculated for the affected and unaffected hemispheres of each subject as well as a healthy control subject set. Results Of the three metrics, CDI, DDI and GI, used for cortical folding assessment, none demonstrated significant differences between the participants and previously studied healthy adults. There was a significant negative correlation between the DDI ratio between affected and unaffected hemispheres and AVM volume (correlation coefficient r = −0.74, p = 0.04). Conclusion This study is the first to quantitatively assess human brain cortical folding in the presence of intracranial AVMs and no significant differences between AVM-affected versus unaffected hemispheres were found in a small dataset. We suggest longitudinal, larger human MRI-based cortical folding studies to assess whether AVMs are congenital lesions of vascular development or de novo, dynamic lesions. PMID:28009020

  20. Perfusion computed tomography of intracranial meningiomas: In vivo correlation of cerebral blood volume and vascular permeability.

    PubMed

    Granata, Francesca; Morabito, Rosa; Alafaci, Concetta; Barresi, Valeria; Tomasello, Francesco; Vinci, Sergio; Mormina, Enricomaria; Calamuneri, Alessandro; Grasso, Giovanni; Salpietro, Francesco Maria; Longo, Marcello

    2015-06-01

    A noninvasive method to predict the grade of a meningioma would be desirable since it would anticipate information about tumour nature, recurrence and improve tumour management and outcomes. The aim of the present study was to assess the ability of perfusion computed tomography (PCT) technique in predicting the meningioma grade before surgery. Data from PCT, such as cerebral blood volume (CBV) and permeability surface (PS), were correlated with immunohistolopathological information. Twenty-three patients with a diagnosis of intracranial meningioma underwent PCT for pre-surgical evaluation of CBV and PS. During surgery, samples from the centre and periphery of the tumour were obtained. Two correspondent regions of interest (ROIs) were drawn on CBV and PS maps. Central and peripheral CBV and PS mean values were calculated. PCT parameters were correlated to CD-34 and endoglin. There was a positive correlation between PS and CD-34. No correlation was found between PS values and endoglin, CBV values and CD-34 and endoglin values. Our findings suggest that PCT may support conventional morphological imaging in predicting meningioma grading before surgery. © The Author(s) 2015.

  1. [Unruptured intracranial aneurysms associated with vascular disorder of the vertebro-basilar system: report of five cases].

    PubMed

    Yamamoto, M; Jimbo, M; Ide, M; Kasai, T; Tanaka, N; Takeyama, E

    1987-12-01

    Among fifty-two angiographically examined patients of vertebro-basilar disorder, incidental intracranial aneurysms were found in five cases. These were four males and a female, ages ranging from 42-67 year old. Vertebro-basilar disorders were ischemic origin in three cases and hemorrhage in two cases. Locations of incidental aneurysms were the internal carotid artery, middle cerebral artery, anterior communicating artery and basilar artery. They were multiple in three cases. They were operated on radically, except one case whose status seemed to be untolerable to the operation. Intervals between strokes and operations were 51-81 days. In spite of successful operation, mild to moderate disturbances of consciousness appeared in three cases. In two cases they were subsided in a week, but in one case it persisted for two months. Fortunately, final results in all operative cases were good. These transient deterioration of consciousness level might probably be ascribed to vertebro-basilar insufficiency once subsided but resumed due to the operative affections. Through a small experience of four cases, three points would be discussed to prevent such hazards. 1) Extreme systemic hypotension as well as hypertension should be avoided during operation. 2) GOF might be an anesthesia of choice in such cases. 3) Operation should be favorably waited for more than six months after the ictus.

  2. Rapid growth of an infectious intracranial aneurysm with catastrophic intracranial hemorrhage.

    PubMed

    Koffie, Robert M; Stapleton, Christopher J; Torok, Collin M; Yoo, Albert J; Leslie-Mazwi, Thabele M; Codd, Patrick J

    2015-03-01

    Infectious intracranial aneurysms are rare vascular lesions that classically occur in patients with infective endocarditis. We present a 49-year-old man with altered mental status and headache with rapid growth and rupture of an infectious intracranial aneurysm with catastrophic intracranial hemorrhage, and review issues related to open neurosurgical and endovascular interventions.

  3. Supernova hemorrhage: obliterative hemorrhage of brain arteriovenous malformations following γ knife radiosurgery.

    PubMed

    Alexander, Matthew D; Hetts, Steven W; Young, William L; Halbach, Van V; Dowd, Christopher F; Higashida, Randall T; English, Joey D

    2012-09-01

    Hemorrhage represents the most feared complication of cerebral arteriovenous malformations (AVMs) in both untreated patients and those treated with gamma knife radiosurgery. Radiosurgery does not immediately lead to obliteration of the malformation, which often does not occur until years following treatment. Post-obliteration hemorrhage is rare, occurring months to years after radiosurgery, and has been associated with residual or recurrent AVM despite prior apparent nidus elimination. Three cases are reported of delayed intracranial hemorrhage in patients with cerebral AVMs treated with radiosurgery in which no residual AVM was found on catheter angiography at the time of delayed post-treatment hemorrhage. That the pathophysiology of these hemorrhages involves progressive venous outflow occlusion is speculated and the possible mechanistic link to subsequent vascular rupture is discussed.

  4. Low-dose contrast-enhanced time-resolved MR angiography at 3T: diagnostic accuracy for treatment planning and follow-up of vascular malformations.

    PubMed

    Anzidei, M; Cavallo Marincola, B; Napoli, A; Saba, L; Zaccagna, F; Lucatelli, P; Fanelli, F; Bassetti, E; Salvatori, F M; Catalano, C; Passariello, R

    2011-12-01

    To assess the accuracy of low-dose contrast-enhanced time-resolved 3T magnetic resonance angiography (MRA) for the morphological and functional assessment of vascular malformations (VM), and to evaluate its diagnostic potential for the depiction of treatment-induced changes. Twenty-five patients with known VM underwent MRA to evaluate the location and extent of lesions and their haemodynamic characteristics. Three-dimensional (3D) T1-weighted time-resolved sequences were acquired following the administration of 0.05mmol/kg of gadobenate dimeglumine. VM were classified according to their morphology and haemodynamic characteristics. All patients thereafter underwent conventional angiography to confirm the diagnosis and to treat the lesions (embolization or sclerotherapy). Follow-up MRA was performed 30 days after treatment to assess morphological and functional changes. A visual analogue scale (VAS) was used to clinically assess the severity of symptoms before and after therapy. Based on haemodynamic characteristics, VM were classified as predominantly arterial [4 (16%)], artero-venous [19 (76%)] or venous [2 (8%)]. Twenty-three (92%) lesions were classified as high-flow VM and two (8%) as low-flow VM. Intralesional thrombosis was present in 17 (68%) lesions before therapy and in 10 lesions (40%) after therapy. The median VAS scores were 5±1 before treatment and 4±2 after treatment. Very good correlation (Spearman's correlation coefficient: rho=0.87; p=0.000) was noted between the reduction of lesion size on follow-up MRA and pain relief as assessed by VAS. Low-dose contrast-enhanced time-resolved 3T MRA can be used to define morphological and functional aspects of VM accurately during treatment planning and follow-up, and can identify post-therapy changes that positively correlate with treatment outcome. 2011 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

  5. Ohtahara syndrome associated with hemimegalencephaly and intracranial lipoma

    PubMed Central

    Gowda, Vykuntaraju K.; Bhat, Ashwini; Bhat, Maya; Ramaswamy, Premalatha

    2015-01-01

    Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types. We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma. PMID:26167232

  6. Ohtahara syndrome associated with hemimegalencephaly and intracranial lipoma.

    PubMed

    Gowda, Vykuntaraju K; Bhat, Ashwini; Bhat, Maya; Ramaswamy, Premalatha

    2015-01-01

    Hemimegalencephaly is a disorder of cortical malformation and is associated with various disorders including various neurocutaneous syndromes and many seizure types. We present a case of hemimegalencephaly associated with Ohtahara syndrome and intracranial and facial lipoma.

  7. Idiopathic intracranial hypertension in children: Diagnostic and management approach.

    PubMed

    Albakr, Abdulrahman; Hamad, Muddathir H; Alwadei, Ali H; Bashiri, Fahad A; Hassan, Hamdy H; Idris, Hiyam; Hassan, Saeed; Muayqil, Taim; Altweijri, Ikhlass; Salih, Mustafa A

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. If not treated properly, it may lead to severe visual dysfunction. Here we review the etiology, clinical presentation, diagnostic criteria and management of IIH in children through illustration of the clinical and radiological presentation of a 13-year-old overweight girl who presented with severe headache, diplopia and bilateral papilledema. Otherwise, she had unremarkable neurological and systemic examinations. Lumbar puncture showed a high CSF opening pressure (360-540 mmH2O). Her investigations showed normal complete blood count (CBC), normal renal, liver, and thyroid function tests. Cerebrospinal fluid (CSF) and blood chemistry were unremarkable. Magnetic resonant image (MRI) of the brain demonstrated empty sella turcica, tortuous optic nerves, and flattening of the posterior sclera. Magnetic resonant venography (MRV) showed focal narrowing of the distal transverse sinuses and absence of venous sinus thrombosis. She required treatment with acetazolamide and prednisolone. With medical treatment, weight reduction, and exercise, our patient had a remarkable improvement in her symptoms with resolution of papilledema in two months. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss.

  8. Idiopathic intracranial hypertension in children: Diagnostic and management approach

    PubMed Central

    Hamad, Muddathir H; Alwadei, Ali H; Bashiri, Fahad A; Hassan, Hamdy H; Idris, Hiyam; Hassan, Saeed; Muayqil, Taim; Altweijri, Ikhlass; Salih, Mustafa A

    2016-01-01

    Idiopathic intracranial hypertension (IIH) is a rare neurological disorder in children. It is characterized by raised intracranial pressure (ICP) in the absence of brain parenchymal lesion, vascular malformations, hydrocephalus, or central nervous system (CNS) infection. The diagnosis is usually confirmed by high opening pressure of cerebrospinal fluid (CSF) with exclusion of secondary causes of intracranial hypertension. If not treated properly, it may lead to severe visual dysfunction. Here we review the etiology, clinical presentation, diagnostic criteria and management of IIH in children through illustration of the clinical and radiological presentation of a 13-year-old overweight girl who presented with severe headache, diplopia and bilateral papilledema. Otherwise, she had unremarkable neurological and systemic examinations. Lumbar puncture showed a high CSF opening pressure (360–540 mmH2O). Her investigations showed normal complete blood count (CBC), normal renal, liver, and thyroid function tests. Cerebrospinal fluid (CSF) and blood chemistry were unremarkable. Magnetic resonant image (MRI) of the brain demonstrated empty sella turcica, tortuous optic nerves, and flattening of the posterior sclera. Magnetic resonant venography (MRV) showed focal narrowing of the distal transverse sinuses and absence of venous sinus thrombosis. She required treatment with acetazolamide and prednisolone. With medical treatment, weight reduction, and exercise, our patient had a remarkable improvement in her symptoms with resolution of papilledema in two months. This review highlights the importance of early recognition and management of IIH to prevent permanent visual loss. PMID:28096561

  9. 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, ...

  10. Intracranial stenosis in cognitive impairment and dementia.

    PubMed

    Hilal, Saima; Xu, Xin; Ikram, M Kamran; Vrooman, Henri; Venketasubramanian, Narayanaswamy; Chen, Christopher

    2017-06-01

    Intracranial stenosis is a common vascular lesion observed in Asian and other non-Caucasian stroke populations. However, its role in cognitive impairment and dementia has been under-studied. We, therefore, examined the association of intracranial stenosis with cognitive impairment, dementia and their subtypes in a memory clinic case-control study, where all subjects underwent detailed neuropsychological assessment and 3 T neuroimaging including three-dimensional time-of-flight magnetic resonance angiography. Intracranial stenosis was defined as ≥50% narrowing in any of the intracranial arteries. A total of 424 subjects were recruited of whom 97 were classified as no cognitive impairment, 107 as cognitive impairment no dementia, 70 vascular cognitive impairment no dementia, 121 Alzheimer's Disease, and 30 vascular dementia. Intracranial stenosis was associated with dementia (age/gender/education - adjusted odds ratios (OR): 4.73, 95% confidence interval (CI): 1.93-11.60) and vascular cognitive impairment no dementia (OR: 3.98, 95% CI: 1.59-9.93). These associations were independent of cardiovascular risk factors and MRI markers. However, the association with Alzheimer's Disease and vascular dementia became attenuated in the presence of white matter hyperintensities. Intracranial stenosis is associated with vascular cognitive impairment no dementia independent of MRI markers. In Alzheimer's Disease and vascular dementia, this association is mediated by cerebrovascular disease. Future studies focusing on perfusion and functional markers are needed to determine the pathophysiological mechanism(s) linking intracranial stenosis and cognition so as to identify treatment strategies.

  11. MiR-29b Downregulation Induces Phenotypic Modulation of Vascular Smooth Muscle Cells: Implication for Intracranial Aneurysm Formation and Progression to Rupture.

    PubMed

    Sun, Liqian; Zhao, Manman; Zhang, Jingbo; Lv, Ming; Li, Youxiang; Yang, Xinjian; Liu, Aihua; Wu, Zhongxue

    2017-01-01

    Our previous microarray results identified numerous microRNAs (miRNAs), including miR-29b, that were differentially expressed in the serum of intracranial aneurysm (IA) patients. The current study aimed to investigate whether miR-29b downregulation in IA could promote the phenotypic modulation of vascular smooth muscle cells (VSMCs) involved in the pathogenesis of aneurysm by activating ATG14-mediated autophagy. First, the levels of miR-29b and autophagy related genes (ATGs) between IA patients and normal subjects were compared. Next, we modified the level of miR-29b via lentivirus particles in the VSMCs and examined the effects of miR-29b on proliferation, migration, and phenotypic modulation of VSMCs from a contractile phenotype to a synthetic phenotype, as well as the levels of autophagy. Finally, the binding of miR-29b to the 3'UTR of ATG14 mRNA and its effects on ATG14 expression were analysed by a luciferase reporter assay and Western blot, respectively. The level of miR-29b was decreased, and autophagy markers were increased in the IA patients compared to that of the normal subjects. Knockdown of miR-29b significantly promoted VSMCs proliferation and migration and, more importantly, induced the phenotypic modulation associated with autophagy activation, whereas miR-29b overexpression showed the opposite effects. The luciferase reporter assay demonstrated that ATG14 was a functional target gene of miR-29b. Notably, knockdown of ATG14 by siRNA apparently abrogated miR-29b inhibition-mediated phenotypic modulation. Downregulation of miR-29b induced VSMCs phenotypic modulation by directly activating ATG14-mediated autophagy, which is associated with the formation, growth and rupture of IAs. © 2017 The Author(s) Published by S. Karger AG, Basel.

  12. A Japanese pedigree of familial cerebral cavernous malformations--a case report.

    PubMed

    Imada, Yasutaka; Yuki, Kiyoshi; Migita, Keisuke; Sadatomo, Takashi; Kuwabara, Masashi; Yamada, Toru; Kurisu, Kaoru

    2014-12-01

    Familial cerebral cavernous malformations (FCCM) are autosomal-dominant vascular malformations. At present, 3 cerebral cavernous malformation genes (KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3) have been identified. Few genetic analyses of Japanese FCCM have been reported. A Japanese pedigree of 4 patients with FCCM has been reported that includes the genetic analysis of one of the patients. All 4 patients showed multiple lesions in the brain. Surgical removal was performed at our hospital due to enlargement or hemorrhage of the intracranial lesions in a 21-year-old female (Case 1) and a 30-year-old male (Case 2). The histological diagnoses were cavernous malformations. A 62-year-old female (Case 4), the mother of Cases 1, 2, and 3, suffered from intramedullary hemorrhage at T6-7 and surgical removal was performed at another hospital. Only one patient, a 32-year-old female (Case 3), did not show symptoms. The genetic analysis of Case 2 demonstrated heterozygous partial deletions of exons 12-15 of the KRIT1 gene.

  13. Diagnostic cerebral angiography in spontaneous intracranial haemorrhage: a guide for developing countries.

    PubMed

    Abu Bakar, Ishak; Shuaib, Ibrahim Lutfi; Mohd Ariff, Abdul Rahman; Naing, Nyi Nyi; Abdullah, Jafri Malin

    2005-01-01

    Spontaneous intracranial haemorrhage constitutes 18-40% of all stroke cases. Indications for cerebral angiography to find underlying potentially treatable vascular abnormalities are not clear. This study determined which intracranial haemorrhage patients need cerebral angiography by correlating computed tomography (CT) findings, age and hypertension history with cerebral angiography findings. A total of 54 patients (8-79 years) with intracranial haemorrhage who underwent both CT examination and six-vessel cerebral angiography were studied over a 2-year period. Cerebral angiography was repeated within 6 weeks if the first angiogram was negative. Angiography detected vascular lesions in 50% of cases (aneurysm 38.9% and arteriovenous malformation, AVM, 11.1%). In the aneurysm group, angiographic yield was 34.3% whereas in the AVM group, it was 37.9%. Subarachnoid haemorrhage (SAH) combined with other types of haemorrhage (such as intracerebral haemorrhage, ICH) was not significantly correlated with the likelihood of finding a vascular lesion, both aneurysm and AVM (p = 0.157). Age less than 50 years had significant correlation (p = 0.021) in the AVM group as well as in the aneurysm group (p < 0.001). A history of hypertension was associated with both aneurysm (p = 0.039) and AVM (p = 0.008). No patients with deep intracerebral haematoma had vascular lesions. The presence of an intravascular haemorrhage (IVH) had significant correlation with aneurysm (p = 0.008) but not AVM. There was no significant difference in mean age between patients with and without a vascular lesion (p = 0.134). Cerebral angiography is justified in patients with ICH accompanied by pure SAH (p = 0.001). Other factors associated with finding a vascular lesion were a history of hypertension and the presence of IVH. Diagnostic cerebral angiography is indicated for patients with ICH and SAH and IVH with a history of hypertension, regardless of age.

  14. Arteriovenous malformation of the uterus.

    PubMed

    Dodia, Nazera; George, Suku

    2015-09-17

    We present the case of a 54-year-old woman with intermittent right-sided abdominal pain. Ultrasound scans showed an unusual vascular appearance of the uterus with a thinned endometrium. Contrast CT led to a strong suspicion of an arteriovenous malformation of the uterus. The patient was successfully treated with a hysterectomy with salpingo-oophorectomy. 2015 BMJ Publishing Group Ltd.

  15. The ACVRL1 c.314-35A>G Polymorphism is Associated with Organ Vascular Malformations in Hereditary Hemorrhagic Telangiectasia Patients with ENG Mutations, but not in Patients with ACVRL1 Mutations

    PubMed Central

    Pawlikowska, Ludmila; Nelson, Jeffrey; Guo, Diana E.; McCulloch, Charles E.; Lawton, Michael T.; Young, William L.; Kim, Helen; Faughnan, Marie E.

    2015-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is characterized by the presence of vascular malformations (VMs) and caused by mutations in TGFβ/BMP9 pathway genes, most commonly ENG or ACVRL1. Patients with HHT have diverse phenotypes related to skin and mucosal telangiectases and organ VMs, including arteriovenous malformations (AVM). The clinical heterogeneity of HHT suggests a potential role for genetic modifier effects. We hypothesized that the common polymorphisms ACVRL1 c.314-35A>G and ENG c.207G>A, previously associated with sporadic brain AVM, are also associated with organ VM in HHT. We genotyped ACVRL1 c.314-35A>G and ENG c.207G>A in 716 patients with HHT recruited by the Brain Vascular Malformation Consortium and evaluated association of genotype with presence of any organ VM, and specifically with brain VM, liver VM and pulmonary AVM, by multivariate logistic regression analyses stratified by HHT mutation. Among all patients with HHT, neither polymorphism was significantly associated with presence of any organ VM; ACVRL1 c.314-35A>G showed a trend toward association with pulmonary AVM (OR=1.48, p=0.062). ACVRL1 c.314-35A>G was significantly associated with any VM among patients with HHT with ENG (OR=2.66, p=0.022), but not ACVRL1 (OR=0.79, p=0.52) mutations. ACVRL1 c.314-35A>G was also significantly associated with pulmonary AVM and liver VM among ENG mutation carriers. There were no significant associations between ENG c.207G>A and any VM phenotype. These results suggest that common polymorphisms in HHT genes other than the mutated gene modulate phenotype severity of HHT disease, specifically presence of organ VM. PMID:25847705

  16. Raised intracranial pressure and cerebral blood flow

    PubMed Central

    Johnston, I. H.; Rowan, J. O.

    1974-01-01

    Pressure changes within the venous outflow tract from the brain were studied in anaesthetized baboons. Segmental vascular resistance changes were also calculated and the results correlated with the changes in cerebral blood flow, measured by the 133Xenon clearance method. Three different methods were used to raise intracranial pressure: cisterna magna infusion, a supratentorial subdural balloon, and an infratentorial subdural balloon. A close correlation was found between the cortical vein pressure and intracranial pressure with all methods of raising intracranial pressure: the overall correlation coefficient was 0·98. In the majority of animals sagittal sinus pressure showed little change through a wide range of intracranial pressure. In three of the six animals in the cisterna magna infusion group, however, sagittal sinus pressure increased to levels approaching the intracranial pressure during the later stages of intracranial hypertension. Jugular venous pressure showed little change with increasing intracranial pressure. The relationship between cerebral prefusion pressure and cerebral blood flow differed according to the method of increasing intracranial pressure. This was due to differing patterns of change in prevenous vascular resistance as venous resistance increased progressively with increasing pressure in all three groups. The present results confirm, therefore, the validity of the current definition of cerebral perfusion pressure—that is, cerebral perfusion pressure is equal to mean arterial pressure minus mean intracranial pressure—by demonstrating that intracranial pressure does represent the effective cerebral venous outflow pressure. Images PMID:4209160

  17. Arteriovenous Malformation Management

    SciTech Connect

    Yakes, Wayne F.; Rossi, Plinio; Odink, Henk

    1996-11-15

    Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the initial noninvasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is performed under general anesthesia. Depending on the size of the lesion, additional Swan-Ganz line and arterial line monitoring are performed. Patients are usually observed overnight and uneventfully discharged the following day if no complication occurs. Patients are followed at periodic intervals despite cure of their lesion. Long-term follow-up is essential in AVM management.

  18. Intracranial Hematoma

    MedlinePlus

    ... one that causes only a brief lapse of consciousness (concussion) — can be minor, an intracranial hematoma is ... Increasing headache Vomiting Drowsiness and progressive loss of consciousness Dizziness Confusion Unequal pupil size Slurred speech As ...

  19. 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.

  20. Congenital plaque-type glomuvenous malformation associated with chylous ascites.

    PubMed

    Tejedor, Maria; Martín-Santiago, Ana; Gómez, Cristina; Fiol, Miquel; Benítez-Segura, Ignacio

    2010-01-01

    Congenital plaque-type glomuvenous malformation (GVM) is caused by loss of function mutations in glomulin gene. We report a newborn with this rare vascular disorder associated with chylous ascites. The common mesenchymal origin of GVM and lymphatic vessels as well as the glomulin expression in vascular smooth muscle cells in utero could help explain this unusual prenatal complication of glomuvenous malformations.

  1. Intracranial hypertension: classification and patterns of evolution

    PubMed Central

    Iencean, SM

    2008-01-01

    Intracranial hypertension (ICH) was systematized in four categories according to its aetiology and pathogenic mechanisms: parenchymatous ICH with an intrinsic cerebral cause; vascular ICH, which has its aetiology in disorders of cerebral blood circulation; ICH caused by disorders of cerebro–spinal fluid dynamics and idiopathic ICH. The increase of intracranial pressure is the first to happen and then intracranial hypertension develops from this initial effect becoming symptomatic; it then acquires its individuality, surpassing the initial disease. The intracranial hypertension syndrome corresponds to the stage at which the increased intracranial pressure can be compensated and the acute form of intracranial hypertension is equivalent to a decompensated ICH syndrome. The decompensation of intracranial hypertension is a condition of instability and appears when the normal intrinsic ratio of intracranial pressure – time fluctuation is changed. The essential conditions for decompensation of intracranial hypertension are: the speed of intracranial pressure increase over normal values, the highest value of abnormal intracranial pressure and the duration of high ICP values. Medical objectives are preventing ICP from exceeding 20 mm Hg and maintaining a normal cerebral blood flow. The emergency therapy is the same for the acute form but each of the four forms of ICH has a specific therapy, according to the pathogenic mechanism and if possible to aetiology. PMID:20108456

  2. Glomuvenous malformation: magnetic resonance imaging findings.

    PubMed

    Flors, Lucia; Norton, Patrick T; Hagspiel, Klaus D

    2015-02-01

    We report a case of a glomuvenous malformation involving the dorsal aspect of the right hand and distal forearm in an 11-year-old boy. He had a history of multiple vascular anomalies since birth and presented with increasing right hand pain. MRI played an important role in characterizing and determining the extent of the lesion. In particular, dynamic time-resolved contrast-enhanced MR angiography precisely defined its vascularity. The diagnosis was made histopathologically after partial resection of the lesion. Glomuvenous malformation is a rare developmental hamartoma that originates from the glomus body. Clinically they usually resemble a venous malformation but they are a different entity. In the appropriate clinical setting this rare condition must be included in the differential diagnosis of a vascular malformation, especially when subtle arterial enhancement, early venous shunting and progressive filling of dilated venous spaces are depicted on MRA.

  3. Inner Ear Conductive Hearing Loss and Unilateral Pulsatile Tinnitus Associated with a Dural Arteriovenous Fistula: Case Based Review and Analysis of Relationship between Intracranial Vascular Abnormalities and Inner Ear Fluids

    PubMed Central

    Cassandro, Ettore; Cassandro, Claudia; Sequino, Giuliano; Scarpa, Alfonso; Petrolo, Claudio; Chiarella, Giuseppe

    2015-01-01

    While pulsatile tinnitus (PT) and dural arteriovenous fistula (DAVF) are not rarely associated, the finding of a conductive hearing loss (CHL) in this clinical picture is unusual. Starting from a case of CHL and PT, diagnosed to be due to a DAVF, we analyzed relationship between intracranial vascular abnormalities and inner ear fluids. DAVF was treated with endovascular embolization. Following this, there was a dramatic recovery of PT and of CHL, confirming their cause-effect link with DAVF. We critically evaluated the papers reporting this association. This is the first case of CHL associated with PT and DAVF. We describe the most significant experiences and theories reported in literature, with a personal analysis about the possible relationship between vascular intracranial system and labyrinthine fluids. In conclusion, we believe that this association may be a challenge for otolaryngologists. So we suggest to consider the possibility of a DAVF or other AVMs when PT is associated with CHL, without alterations of tympanic membrane and middle ear tests. PMID:26693371

  4. Notch receptor expression in human brain arteriovenous malformations.

    PubMed

    Hill-Felberg, Sandra; Wu, Hope Hueizhi; Toms, Steven A; Dehdashti, Amir R

    2015-08-01

    The roles of the Notch pathway proteins in normal adult vascular physiology and the pathogenesis of brain arteriovenous malformations are not well-understood. Notch 1 and 4 have been detected in human and mutant mice vascular malformations respectively. Although mutations in the human Notch 3 gene caused a genetic form of vascular stroke and dementia, its role in arteriovenous malformations development has been unknown. In this study, we performed immunohistochemistry screening on tissue microarrays containing eight surgically resected human brain arteriovenous malformations and 10 control surgical epilepsy samples. The tissue microarrays were evaluated for Notch 1-4 expression. We have found that compared to normal brain vascular tissue Notch-3 was dramatically increased in brain arteriovenous malformations. Similarly, Notch 4 labelling was also increased in vascular malformations and was confirmed by western blot analysis. Notch 2 was not detectable in any of the human vessels analysed. Using both immunohistochemistry on microarrays and western blot analysis, we have found that Notch-1 expression was detectable in control vessels, and discovered a significant decrease of Notch 1 expression in vascular malformations. We have demonstrated that Notch 3 and 4, and not Notch 1, were highly increased in human arteriovenous malformations. Our findings suggested that Notch 4, and more importantly, Notch 3, may play a role in the development and pathobiology of human arteriovenous malformations.

  5. Venous malformations: classification, development, diagnosis, and interventional radiologic management.

    PubMed

    Legiehn, Gerald M; Heran, Manraj K S

    2008-05-01

    Venous malformations are categorized as low-flow vascular malformations within the domain of vascular anomalies and are the most common vascular malformation encountered clinically. Venous malformations are by definition present at birth, undergo pari passu growth, and present clinically because of symptoms related to mass effect or stasis. Although diagnosis can usually be made by clinical history and examination, differentiation from other vascular and nonvascular entities often requires an imaging work-up that includes ultrasound, CT, MR imaging, and diagnostic phlebography. All decisions regarding imaging work-up and decision to treat must be coordinated though referral and discussions with a multidisciplinary team and be based on clearly defined clinical indications. Percutaneous image-guided sclerotherapy has become the mainstay of treatment for venous malformations and involves the introduction of any one of a number of endothelial-cidal sclerosants into the vascular spaces of the lesion, with each sclerosant possessing its own unique spectrum of advantages and disadvantages.

  6. Glioblastoma Mimicking an Arteriovenous Malformation

    PubMed Central

    Khanna, Arjun; Venteicher, Andrew S.; Walcott, Brian P.; Kahle, Kristopher T.; Mordes, Daniel A.; William, Christopher M.; Ghogawala, Zoher; Ogilvy, Christopher S.

    2013-01-01

    Abnormal cerebral vasculature can be a manifestation of a vascular malformation or a neoplastic process. We report the case of a patient with angiography-negative subarachnoid hemorrhage (SAH) who re-presented 3 years later with a large intraparenchymal hemorrhage. Although imaging following the intraparenchymal hemorrhage was suggestive of arteriovenous malformation, the patient was ultimately found to have an extensive glioblastoma associated with abnormal tumor vasculature. The case emphasizes the need for magnetic resonance imaging to investigate angiography-negative SAH in suspicious cases to rule out occult etiologies, such as neoplasm. We also discuss diagnostic pitfalls when brain tumors are associated with hemorrhage and abnormal vasculature. PMID:24137154

  7. Vertebrobasilar insufficiency. Part 2. Microsurgical treatment of intracranial vertebrobasilar disease.

    PubMed

    Hopkins, L N; Martin, N A; Hadley, M N; Spetzler, R F; Budny, J; Carter, L P

    1987-05-01

    Posterior circulation transient ischemic attacks have an associated risk of subsequent infarction of approximately 5% per year. Intracranial vertebrobasilar thrombo-occlusive lesions appear particularly likely to result in repetitive ischemic symptoms and in infarction due to hemodynamic insufficiency. The authors present their experience with 45 patients with symptomatic intracranial vertebrobasilar vascular disease despite maximal medical therapy. The specific operative approaches for intracranial vertebral artery endarterectomy and extracranial to intracranial posterior circulation revascularization procedures are outlined.

  8. Spontaneous intracranial hemorrhage and multiple intracranial aneurysms in a patient with Roberts/SC phocomelia syndrome.

    PubMed

    Wang, Anthony C; Gemmete, Joseph J; Keegan, Catherine E; Witt, Cordelie E; Muraszko, Karin M; Than, Khoi D; Maher, Cormac O

    2011-11-01

    Roberts/SC phocomelia syndrome (RBS) is a rare but distinct genetic disorder with an autosomal recessive inheritance pattern. It has been associated with microcephaly, craniofacial malformation, cavernous hemangioma, encephalocele, and hydrocephalus. There are no previously reported cases of RBS with intracranial aneurysms. The authors report on a patient with a history of RBS who presented with a spontaneous posterior fossa hemorrhage. Multiple small intracranial aneurysms were noted on a preoperative CT angiogram. The patient underwent emergency craniotomy for evacuation of the hemorrhage. A postoperative angiogram confirmed the presence of multiple, distal small intracranial aneurysms.

  9. [Prolonged hypothermia in refractory intracranial hypertension. Report of one case].

    PubMed

    Rovegno, Maximiliano; Valenzuela, José Luis; Mellado, Patricio; Andresen, Max

    2012-02-01

    The use of hypothermia after cardiac arrest caused by ventricular fibrillation is a standard clinical practice, however its use for neuroprotection has been extended to other conditions. We report a 23-year-old male with intracranial hypertension secondary to a parenchymal hematoma associated to acute hydrocephalus. An arterial malformation was found and embolized. Due to persistent intracranial hypertension, moderate hypothermia with a target temperature of 33°C was started. After 12 hours of hypothermia, intracranial pressure was controlled. After 13 days of hypothermia a definitive control of intracranial pressure was achieved. The patient was discharged 40 days after admission, remains with a mild hemiparesia and is reassuming his university studies.

  10. Vascular ring

    MedlinePlus

    ... Stanton BF, St Geme JW, Schor NF. Other congenital heart and vascular malformations. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics . 20th ed. Philadelphia, ... AN. Congenital heart disease. In: Mann DL, Zipes DP, Libby ...

  11. Hemorrhage Rates From Brain Arteriovenous Malformation in Hereditary Hemorrhagic Telangiectasia Patients

    PubMed Central

    Kim, Helen; Nelson, Jeffrey; Krings, Timo; terBrugge, Karel G.; McCulloch, Charles E.; Lawton, Michael T.; Young, William L.; Faughnan, Marie E.

    2015-01-01

    Background and Purpose Hereditary hemorrhagic telangiectasia (HHT) is a systemic disease characterized by mucocutaneous telangiectasias, epistaxis, and arteriovenous malformations (AVM). Intracranial hemorrhage (ICH) rates in this population are not well described. We report ICH rates and characteristics in HHT patients with brain arteriovenous malformations (HHT-BAVM). Methods We studied the first 153 HHT-BAVM patients with follow-up data enrolled in the Brain Vascular Malformation Consortium HHT Project. We estimated ICH rates after BAVM diagnosis. Results The majority of patients were female (58%) and Caucasian (98%). The mean age at BAVM diagnosis was 31±19 years (range: 0–70), with 61% of cases diagnosed upon asymptomatic screening. Overall, 14% presented with ICH; among symptomatic cases, 37% presented ruptured. During 493 patient-years of follow-up, 5 ICH events occurred yielding a rate of 1.02% per-year (95% CI: 0.42–2.44%). ICH-free survival differed significantly by ICH presentation (P=0.003); ruptured cases had a higher ICH rate (10.07%, 95% CI: 3.25–31.21%) than unruptured cases (0.43%, 95% CI: 0.11–1.73%). Conclusions HHT-BAVM patients who present with hemorrhage are at a higher risk for re-hemorrhage compared to BAVMs detected pre-symptomatically. PMID:25858236

  12. Intracranial Arteries - Anatomy and Collaterals.

    PubMed

    Liebeskind, David S; Caplan, Louis R

    2016-01-01

    Anatomy, physiology, and pathophysiology are inextricably linked in patients with intracranial atherosclerosis. Knowledge of abnormal or pathological conditions such as intracranial atherosclerosis stems from detailed recognition of the normal pattern of vascular anatomy. The vascular anatomy of the intracranial arteries, both at the level of the vessel wall and as a larger structure or conduit, is a reflection of physiology over time, from in utero stages through adult life. The unique characteristics of arteries at the base of the brain may help our understanding of atherosclerotic lesions that tend to afflict specific arterial segments. Although much of the knowledge regarding intracranial arteries originates from pathology and angiography series over several centuries, evolving noninvasive techniques have rapidly expanded our perspective. As each imaging modality provides a depiction that combines anatomy and flow physiology, it is important to interpret each image with a solid understanding of typical arterial anatomy and corresponding collateral routes. Compensatory collateral perfusion and downstream flow status have recently emerged as pivotal variables in the clinical management of patients with atherosclerosis. Ongoing studies that illustrate the anatomy and pathophysiology of these proximal arterial segments across modalities will help refine our knowledge of the interplay between vascular anatomy and cerebral blood flow. Future studies may help elucidate pivotal arterial factors far beyond the degree of stenosis, examining downstream influences on cerebral perfusion, artery-to-artery thromboembolic potential, amenability to endovascular therapies and stent conformation, and the propensity for restenosis due to biophysical factors. © 2016 S. Karger AG, Basel.

  13. Intracranial, intradural aneurysmal bone cyst.

    PubMed

    Afnan, Jalil; Snuderl, Matija; Small, Juan

    2015-01-01

    Aneurysmal bone cysts (ABCs) are benign, expansile, blood-filled, osteolytic lesions with internal septations that may be intraosseous or extraosseous. The cysts may cause local mass effect, and changes in the regional vascular supply necessitating intervention. A case of an intracranial, intradural ABC in a young male patient with progressively severe headaches is presented. This is only the third recorded intradural case, the majority of these rare lesions being extracranial and only a minute fraction intracranial. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Arteriovenous malformation of brain with stroke in Down syndrome: a case report

    PubMed Central

    Prasad, Rajniti; Singh, Utpal Kant; Mishra, Om Prakash

    2009-01-01

    Down syndrome is a common chromosomal aberration in children. A variety of associated malformations have been reported in the literature, including vascular malformations of pelvic organs. The vascular malformations of brain with Down syndrome have not been reported in the literature. Therefore, we report a child with Down syndrome, with associated arteriovenous malformation in the brain, who developed stroke and was treated successfully. PMID:21686822

  15. Intracranial dural arteriovenous fistulas: clinical characteristics and management based on location and hemodynamics.

    PubMed

    Oh, Jung Tae; Chung, Seung Young; Lanzino, Giuseppe; Park, Ki Seok; Kim, Seong Min; Park, Moon Sun; Kim, Han Kyu

    2012-09-01

    A dural arteriovenous fistula (DAVF) generally refers to a vascular malformation of the wall of a major venous sinus. These lesions have diverse symptoms according to the location and venous drainage, and require multidisciplinary treatment. We report on our experience and analyze the treatment outcome of intracranial DAVFs for a nine-year period. Between January 2000 and December 2008, 95 patients with intracranial DAVFs were enrolled in this study. A retrospective review of clinical records and imaging studies of all patients was conducted. Endovascular embolization, surgical interruption, gamma knife stereotactic radiosurgery (GKS), or combinations of these treatments were performed based on clinical symptoms, lesion location, and venous drainage pattern. Borden type I, II, and III were 34, 48, and 13 patients, respectively. Aggressive presentation was reported in 6% of Borden type I, 31% of Borden type II, and 77% of Borden type III DAVFs, respectively, and DAVFs involving transverse, sigmoid, and superior sagittal sinus. Overall, the rate of complete obliteration was 68%. The complete occlusion rates with a combination treatment of endovascular embolization and surgery, surgery alone, and endovascular embolization were 89%, 86%, and 80%, respectively. When GKS was used with embolization, the obliteration rate was 83%, although it was only 54% in GKS alone. Spontaneous obliteration of the DAVF occurred in three patients. There were a few complications, including hemiparesis (in microsurgery), intracranial hemorrhage (in endovascular embolization), and facial palsy (in GKS). The hemorrhagic risk of DAVFs is dependent on the location and hemodynamics of the lesions. Strategies for treatment of intracranial DAVFs should be decided according to the characteristic of the DAVFs, based on the location and drainage pattern. GKS can be used as an optional treatment for intracranial DAVFs.

  16. Venous malformations: clinical diagnosis and treatment.

    PubMed

    Behravesh, Sasan; Yakes, Wayne; Gupta, Nikhil; Naidu, Sailendra; Chong, Brian W; Khademhosseini, Ali; Oklu, Rahmi

    2016-12-01

    Venous malformation (VM) is the most common type of congenital vascular malformation (CVM). They are present at birth and are often symptomatic, causing morbidity and pain. VMs can be challenging to diagnose and are often confused with hemangioma in terminology as well as with imaging. An accurate clinical history and cross-sectional imaging are critical for diagnosis and for devising management. This manuscript will review imaging approaches to diagnosing VMs and current treatment strategies.

  17. Venous malformations: clinical diagnosis and treatment

    PubMed Central

    Behravesh, Sasan; Yakes, Wayne; Gupta, Nikhil; Naidu, Sailendra; Chong, Brian W.; Khademhosseini, Ali

    2016-01-01

    Venous malformation (VM) is the most common type of congenital vascular malformation (CVM). They are present at birth and are often symptomatic, causing morbidity and pain. VMs can be challenging to diagnose and are often confused with hemangioma in terminology as well as with imaging. An accurate clinical history and cross-sectional imaging are critical for diagnosis and for devising management. This manuscript will review imaging approaches to diagnosing VMs and current treatment strategies. PMID:28123976

  18. Effectiveness of 2-methoxyestradiol in alleviating angiogenesis induced by intracranial venous hypertension.

    PubMed

    Zou, Xiang; Zhou, Liangfu; Zhu, Wei; Mao, Ying; Chen, Liang

    2016-09-01

    OBJECT Intracranial dural arteriovenous fistulas (DAVFs) are complex intracranial vascular malformations that can lead to hemorrhage. The authors recently found that chronic local hypoperfusion seems to be the main cause of angiogenesis in the dura mater, which leads to the formation of DAVFs. As a natural derivative of estradiol, 2-methoxyestradiol (2-ME) has an antiangiogenic effect and can be used safely in patients with advanced carcinoid tumors. This study was conducted to examine the antiangiogenic effects of 2-ME on a rat DAVF model. METHODS Male Sprague-Dawley rats (n = 72) were used in the experiments. Intracranial venous hypertension was induced for modeling, and 2-ME was used in the early or late stage for treatment. The effects were examined by immunohistochemistry, Western blot analysis, and quantitative real-time polymerase chain reaction assays. RESULTS 2-Methoxyestradiol significantly reduced angiogenesis in the dura in early- and late-intervention treatment groups, as proven by the results of immunohistochemical staining, Western blotting, real-time polymerase chain reaction assays, and microvessel density counts. The antiangiogenic effect even lasted for up to 2 weeks after 2-ME cessation. CONCLUSIONS These data collectively suggest that 2-ME can reduce the angiogenic effect caused by venous hypertension in a rat DAVF model, mainly by suppressing the inhibitor of differentiation 1 (ID-1) and hypoxia-inducible factor 1α (HIF-1α) pathways.

  19. Vascular Tumors

    PubMed Central

    Sepulveda, Abel; Buchanan, Edward P.

    2014-01-01

    Vascular anomalies are divided into two main groups: tumors and malformations. Vascular tumors are a large and complex group of lesions, especially for clinicians with none or little experience in this field. In the past, these lesions caused a great deal of confusion because many appear analogous to the naked eye. Thankfully, recent advances in diagnostic techniques have helped the medical community to enhance our comprehension, accurately label, diagnose, and treat these lesions. In this article, we will review the most frequent vascular tumors and provide the reader with the tools to properly label, diagnose, and manage these complex lesions. PMID:25045329

  20. [Pathogenesis and genetics of vascular anomalies].

    PubMed

    Vikkula, M

    2006-01-01

    Vascular anomalies, divided into vascular tumors and vascular malformations, are localized defects of angiogenesis. Hemangiomas appear soon after birth, grow quickly, and then spontaneously, but slowly, disappear. In contrast, vascular malformations are congenital defects of vascular development that grow proportionately with the child. Most vascular anomalies are considered non-hereditary. However, due to detailed analysis inherited forms have been observed, which has led to identify mutations in three genes causing familial vascular malformations: in the angiopoietin receptor TIE2 in mucocutaneous venous malformations (VMCM), in glomulin in glomuvenous malformations (GVM) and in RASA1 in the newly recognized phenotype capillary malformation-arteriovenous malformation (CM-AVM). Identification of the causative genes has permitted more precise diagnosis and differential diagnosis, evaluation of phenotypic variability among patients with a proven mutation, study of used treatments in more homogeneous patient groups, and elucidation of the etiopathogenic mechanisms behind vascular malformations. Further studies are needed to unravel the role of genetic variations in the various vascular malformations and to unravel the precise molecular mechanisms that lead to development of these vascular lesions. This should provide development of new-targeted therapies.

  1. The ACVRL1 c.314-35A>G polymorphism is associated with organ vascular malformations in hereditary hemorrhagic telangiectasia patients with ENG mutations, but not in patients with ACVRL1 mutations.

    PubMed

    Pawlikowska, Ludmila; Nelson, Jeffrey; Guo, Diana E; McCulloch, Charles E; Lawton, Michael T; Young, William L; Kim, Helen; Faughnan, Marie E

    2015-06-01

    Hereditary hemorrhagic telangiectasia (HHT) is characterized by vascular malformations (VMs) and caused by mutations in TGFβ/BMP9 pathway genes, most commonly ENG or ACVRL1. Patients with HHT have diverse manifestations related to skin and mucosal telangiectases and organ VMs, including arteriovenous malformations (AVM). The clinical heterogeneity of HHT suggests a role for genetic modifiers. We hypothesized that the ACVRL1 c.314-35A>G and ENG c.207G>A polymorphisms, previously associated with sporadic brain AVM, are associated with organ VM in HHT. We genotyped these variants in 716 patients with HHT and evaluated association of genotype with presence of any organ VM, and specifically with brain VM, liver VM and pulmonary AVM, by multivariate logistic regression analyses stratified by HHT mutation. Among all patients with HHT, neither polymorphism was significantly associated with presence of any organ VM; ACVRL1 c.314-35A>G showed a trend toward association with pulmonary AVM (OR = 1.48, P = 0.062). ACVRL1 c.314-35A>G was significantly associated with any VM among patients with HHT with ENG (OR = 2.66, P = 0.022), but not ACVRL1 (OR = 0.79, P = 0.52) mutations. ACVRL1 c.314-35A>G was also associated with pulmonary AVM and liver VM among ENG mutation heterozygotes. There were no significant associations between ENG c.207G>A and any VM phenotype. These results suggest that common polymorphisms in HHT genes other than the mutated gene modulate phenotype severity of HHT disease, specifically presence of organ VM. © 2015 Wiley Periodicals, Inc.

  2. Intracranial cavernoma and speckled lentiginous nevus: extending the spectrum of phakomatoses?

    PubMed

    Boncoraglio, Giorgio B; Parati, Eugenio A; Ciceri, Elisa; Rinaldi, Rosa; Capella, Giovanni L

    2010-12-01

    Phakomatosis refers to several malformation syndromes with simultaneous involvement of the skin, the eye, and the central nervous system by developmental lesions. Speckled lentiginous nevus (SLN), a subtype of congenital melanocytic nevi, is usually an isolate, harmless finding. Here, we report the case of a 52-year-old woman with congenital left laterocervical SLN associated with an ipsilateral intracranial extra-axial cavernous angioma, a yet not described association to date. After revision of the literature, we suggest that both these lesions could be correlated in the setting of an atypical, yet unclassifiable form of phakomatosis, such as phakomatosis pigmentovascularis or SLN syndrome. We also propose that patients with bizarre, geometrical, pigmented or vascular cervicocranial skin lesions should undergo a thorough neurologic and ophthalmologic evaluation.

  3. Interhemispheric Lipoma, Callosal Anomaly, and Malformations of Cortical Development: A Case Series.

    PubMed

    Niwa, Tetsu; de Vries, Linda S; Manten, Gwendolyn T R; Lequin, Maarten; Cuppen, Inge; Shibasaki, Jun; Aida, Noriko

    2016-04-01

    Intracranial lipomas are rare congenital malformations. The most common type of intracranial lipoma is the interhemispheric lipoma, which is frequently associated with callosal anomalies such as hypogenesis or agenesis of the corpus callosum. In contrast, interhemispheric lipomas are less often accompanied with malformations of cortical development (MCD). We report magnetic resonance imaging findings of three infants with an interhemispheric lipoma, associated with a callosal anomaly, and MCD: two infants with nodular interhemispheric lipoma, agenesis of the corpus callosum, and polymicrogyria, and one infant with interhemispheric curvilinear lipoma, hypoplasia of the corpus callosum, and heterotopias. An association was suggested regarding the occurrence of these malformations.

  4. 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

  5. Anorectal malformations

    PubMed Central

    Levitt, Marc A; Peña, Alberto

    2007-01-01

    Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life. PMID:17651510

  6. Venous Malformation: update on etiopathogenesis, diagnosis & management

    PubMed Central

    Dompmartin, Anne; Vikkula, Miikka; Boon, Laurence M

    2011-01-01

    The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area. PMID:20870869

  7. Disappearance of a small intracranial aneurysm as a result of vessel straightening and in-stent stenosis following use of an Enterprise vascular reconstruction device.

    PubMed

    Takemoto, Koichiro; Tateshima, Satoshi; Rastogi, Sachin; Gonzalez, Nestor; Jahan, Reza; Duckwiler, Gary; Vinuela, Fernando

    2013-01-17

    In-stent stenosis after stent-assisted coil embolization is a rare but well-known complication. A 32-year-old woman with an unruptured wide-necked left internal carotid artery (ICA) terminus aneurysm and an ipsilateral very small anterior choroidal artery aneurysm underwent stent-assisted coil embolization for the ICA terminus aneurysm. The 4-month follow-up angiography revealed diffuse in-stent stenosis and disappearance of the untreated anterior choroidal artery aneurysm, retaining the patency of the anterior choroidal artery. To our knowledge, this is the first report to demonstrate the course of in-stent stenosis and disappearance of an untreated small intracranial aneurysm as a result. We report this unique case and discuss the interesting mechanism underlying this phenomenon, and also provide a review of the relevant literature.

  8. Disappearance of a small intracranial aneurysm as a result of vessel straightening and in-stent stenosis following use of an Enterprise vascular reconstruction device.

    PubMed

    Takemoto, Koichiro; Tateshima, Satoshi; Rastogi, Sachin; Gonzalez, Nestor; Jahan, Reza; Duckwiler, Gary; Vinuela, Fernando

    2014-01-01

    In-stent stenosis after stent-assisted coil embolization is a rare but well-known complication. A 32-year-old woman with an unruptured wide-necked left internal carotid artery (ICA) terminus aneurysm and an ipsilateral very small anterior choroidal artery aneurysm underwent stent-assisted coil embolization for the ICA terminus aneurysm. The 4-month follow-up angiography revealed diffuse in-stent stenosis and disappearance of the untreated anterior choroidal artery aneurysm, retaining the patency of the anterior choroidal artery. To our knowledge, this is the first report to demonstrate the course of in-stent stenosis and disappearance of an untreated small intracranial aneurysm as a result. We report this unique case and discuss the interesting mechanism underlying this phenomenon, and also provide a review of the relevant literature.

  9. Intracranial calcified pseudocyst reaction to a shunt catheter.

    PubMed

    Yowtak, June; Hughes, Douglas; Heger, Ian; Macomson, Samuel D

    2014-02-01

    A 9-year-old boy with spina bifida, Chiari II malformation, and hydrocephalus presented with signs of increased intracranial pressure consistent with a shunt malfunction. Radiological investigations revealed an intracranial calcified lesion along the ventricular catheter. A shunt tap revealed a translucent milky white fluid. The patient underwent a ventriculostomy and, eventually, a shunt revision. Pathology findings were consistent with the formation of dystrophic calcification and a pseudocyst around the shunt catheter. Postoperatively, the patient returned to his neurological baseline. This is, to the best of the authors' knowledge, the first report of an intracranial calcified pseudocyst in a patient with normal renal function.

  10. Pathogenesis of Brain Arteriovenous Malformations

    PubMed Central

    KOMIYAMA, Masaki

    2016-01-01

    Brain arteriovenous malformations (bAVMs) represent a high risk of intracranial hemorrhages, which are substantial causes of morbidity and mortality of bAVMs, especially in children and young adults. Although a variety of factors leading to hemorrhages of bAVMs are investigated extensively, their pathogenesis is still not well elucidated. The author has reviewed the updated data of genetic aspects of bAVMs, especially focusing on clinical and experimental knowledge from hereditary hemorrhagic telangiectasia, which is the representative genetic disease presenting with bAVMs caused by loss-of-function in one of the two genes: endoglin and activin receptor-like kinase 1. This knowledge may allow us to infer the pathogensis of sporadic bAVMs and in the development of new medical therapies for them. PMID:27076383

  11. Genetically diagnosed Birt-Hogg-Dubé syndrome and familial cerebral cavernous malformations in the same individual: a case report.

    PubMed

    Whitworth, James; Stausbøl-Grøn, Brian; Skytte, Anne-Bine

    2017-01-01

    When faced with an unusual clinical feature in a patient with a Mendelian disorder, the clinician may entertain the possibilities of either the feature representing a novel manifestation of that disorder or the co-existence of a different inherited condition. Here we describe an individual with a submandibular oncocytoma, pulmonary bullae and renal cysts as well as multiple cerebral cavernous malformations and haemangiomas. Genetic investigations revealed constitutional mutations in FLCN, associated with Birt-Hogg-Dubé syndrome (BHD) and CCM2, associated with familial cerebral cavernous malformation. Intracranial vascular pathologies (but not cerebral cavernous malformation) have recently been described in a number of individuals with BHD (Kapoor et al. in Fam Cancer 14:595-597, 10.1007/s10689-015-9807-y , 2015) but it is not yet clear whether they represent a genuine part of that conditions' phenotypic spectrum. We suggest that in such instances of potentially novel clinical features, more extensive genetic testing to consider co-existing conditions should be considered where available. The increased use of next generation sequencing applications in diagnostic settings is likely to lead more cases such as this being revealed.

  12. Anthelmintic induced congenital malformations in sheep embryos using netobimin.

    PubMed

    Navarro, M; Cristofol, C; Carretero, A; Arboix, M; Ruberte, J

    1998-01-24

    Benzimidazole compounds have teratogenic effects in domestic and experimental animals. In this study, 14 Manchega ewes were treated orally, under controlled conditions, with 20 mg netobimin (a prodrug of a benzimidazole compound) per/kg bodyweight on the 17th day of pregnancy. Congenital malformations and abortions affected 60 per cent of the lambs. The main malformations were skeletal and renal, but vascular malformations were observed for the first time. The abnormalities were investigated using radiological, dissection and vascular injection techniques, and associations among them were recorded. The anomalies are discussed in terms of embryological considerations.

  13. Vascular anomalies in children.

    PubMed

    Weibel, L

    2011-11-01

    Vascular anomalies are divided in two major categories: tumours (such as infantile hemangiomas) and malformations. Hemangiomas are common benign neoplasms that undergo a proliferative phase followed by stabilization and eventual spontaneous involution, whereas vascular malformations are rare structural anomalies representing morphogenetic errors of developing blood vessels and lymphatics. It is important to properly diagnose vascular anomalies early in childhood because of their distinct differences in morbidity, prognosis and need for a multidisciplinary management. We discuss a number of characteristic clinical features as clues for early diagnosis and identification of associated syndromes.

  14. Intradural spinal arteriovenous malformation in a 13-month-old female: A case report.

    PubMed

    McLaughlin, Matthew; Green, Michael

    2015-01-01

    Describe the case of a spinal arteriovenous malformation (AVM), which represents a rare cause for tetraplegia in the infant population. This patient underwent treatment with surgical clipping of an intradural AVM, intravenous steroids, and inpatient rehabilitation. Although AVMs are a congenital vascular malformation, spinal AVMs are extremely rare with only a few case reports published in the pediatric literature. Generally AVMs are diagnosed intracranially which would lead to cerebral infarction; however, in this case, the AVM was in the cervical spinal cord leading to tetraplegia. With medical and rehabilitation interventions, the patient's function improved significantly; however, long-term prognostication remained difficult given the lack of standardized assessments and the inaccuracy of the American Spinal Injury Association (ASIA) examination for this age group. Spinal AVMs are extremely rare in the pediatric population; however, with this case the use of surgical intervention helped prevent further loss of neurologic impairment, and inpatient rehabilitation served to increase the patient's function. She continued to participate in outpatient rehabilitation to improve developmental milestones, mobility, posture, positioning, and upper limb function.

  15. FAM222B Is Not a Likely Novel Candidate Gene for Cerebral Cavernous Malformations.

    PubMed

    Spiegler, Stefanie; Kirchmaier, Bettina; Rath, Matthias; Korenke, G Christoph; Tetzlaff, Fabian; van de Vorst, Maartje; Neveling, Kornelia; Acker-Palmer, Amparo; Kuss, Andreas W; Gilissen, Christian; Fischer, Andreas; Schulte-Merker, Stefan; Felbor, Ute

    2016-07-01

    Cerebral cavernous malformations (CCMs) are prevalent slow-flow vascular lesions which harbour the risk to develop intracranial haemorrhages, focal neurological deficits, and epileptic seizures. Autosomal dominantly inherited CCMs were found to be associated with heterozygous inactivating mutations in 3 genes, CCM1 (KRIT1), CCM2 (MGC4607), and CCM3 (PDCD10) in 1999, 2003 and 2005, respectively. Despite the availability of high-throughput sequencing techniques, no further CCM gene has been published since. Here, we report on the identification of an autosomal dominantly inherited frameshift mutation in a gene of thus far unknown function, FAM222B (C17orf63), through exome sequencing of CCM patients mutation-negative for CCM1-3. A yeast 2-hybrid screen revealed interactions of FAM222B with the tubulin cytoskeleton and STAMBP which is known to be associated with microcephaly-capillary malformation syndrome. However, a phenotype similar to existing models was not found, neither in fam222bb/fam222ba double mutant zebrafish generated by transcription activator-like effector nucleases nor in an in vitro sprouting assay using human umbilical vein endothelial cells transfected with siRNA against FAM222B. These observations led to the assumption that aberrant FAM222B is not involved in the formation of CCMs.

  16. FAM222B Is Not a Likely Novel Candidate Gene for Cerebral Cavernous Malformations

    PubMed Central

    Spiegler, Stefanie; Kirchmaier, Bettina; Rath, Matthias; Korenke, G. Christoph; Tetzlaff, Fabian; van de Vorst, Maartje; Neveling, Kornelia; Acker-Palmer, Amparo; Kuss, Andreas W.; Gilissen, Christian; Fischer, Andreas; Schulte-Merker, Stefan; Felbor, Ute

    2016-01-01

    Cerebral cavernous malformations (CCMs) are prevalent slow-flow vascular lesions which harbour the risk to develop intracranial haemorrhages, focal neurological deficits, and epileptic seizures. Autosomal dominantly inherited CCMs were found to be associated with heterozygous inactivating mutations in 3 genes, CCM1 (KRIT1), CCM2 (MGC4607), and CCM3 (PDCD10) in 1999, 2003 and 2005, respectively. Despite the availability of high-throughput sequencing techniques, no further CCM gene has been published since. Here, we report on the identification of an autosomal dominantly inherited frameshift mutation in a gene of thus far unknown function, FAM222B (C17orf63), through exome sequencing of CCM patients mutation-negative for CCM1-3. A yeast 2-hybrid screen revealed interactions of FAM222B with the tubulin cytoskeleton and STAMBP which is known to be associated with microcephaly-capillary malformation syndrome. However, a phenotype similar to existing models was not found, neither in fam222bb/fam222ba double mutant zebrafish generated by transcription activator-like effector nucleases nor in an in vitro sprouting assay using human umbilical vein endothelial cells transfected with siRNA against FAM222B. These observations led to the assumption that aberrant FAM222B is not involved in the formation of CCMs. PMID:27587990

  17. Chiari-I malformation in two fighter pilots.

    PubMed

    Akin, Ahmet; Canakci, Zafer; Sen, Ahmet; Tore, Hasan F

    2003-07-01

    This report describes two cases of Chiari Malformation Type I (Chiari-I) in fighter pilots of the Turkish Air Force. Chiari-I is a congenital malformation characterized by herniation of cerebellar tonsils through the foramen magnum. Patients have symptoms and signs related to dysfunction of the brainstem, spinal cord, and cerebellum. They generally are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years. Symptoms can be provoked by increasing intracranial pressure (Valsalva or straining). We report on two pilots with Chiari-I malformation who had no symptoms or signs in their daily activities. Furthermore, these pilots had successfully completed physiological training, including centrifuge training, without any symptoms. However, they suffered from headache, neck spasms, and/or disequilibrium under +Gz during flight training sorties. The clinical presentation, diagnosis, treatment, possibility of acquired cases, and aeromedical disposition of Chiari malformations are discussed.

  18. Colonic venous malformation and portal hypertension: association, management, and review of the literature.

    PubMed

    Pierce, James R; Hunter, Catherine J; Naik-Mathuria, Bindi; Stanley, Philip; Ford, Henri R; Genyk, Yuri; Shaul, Donald B; Panossian, Andre; Anselmo, Dean M

    2012-04-01

    We present a case of an adolescent with lower gastrointestinal bleeding caused by a colorectal venous malformation (VM) with concomitant portal hypertension. After an episode of massive gastrointestinal bleeding, we performed an extended right hemicolectomy and resection of the VM and selective portosystemic shunt. Here, we present the case and review the literature regarding portal hypertension and gastrointestinal vascular malformations. Additionally, we discuss the physiologic and hemodynamic effects of gastrointestinal vascular malformations on the portal system.

  19. [Treatments strategies for intracranial cavernomas].

    PubMed

    Stienen, Martin N; Bijlenga, Philippe; Schaller, Karl; Hildebrandt, Gerhard; Gautschi, Oliver P

    2014-11-26

    Cerebral cavernomatous malformations (CCM) are a subgroup of low-pressure cerebral vascular malformation. They have an estimated prevalence of 0,1-4% of the population and account for 8-15% of all cerebral vascular malformations. Depending on their size and localization (supratentorial or infratentorial, brain stem), CCM may present with focal bleedings with or without neurological deficits, as epileptic seizures, or are diagnosed incidentally by MRI performed for unspecific symptoms (e.g. headache, vertigo), especially in younger patients (mostly in 2nd or 3rd decade). The decision-making in patients with CCM can be classified into four categories: surgical or conservative treatment of symptomatic or incidental lesions. The decision regarding the optimal therapy in the individual patient must be made in the knowledge of the natural history (risk for bleeding or epilepsy) on the one hand, and the surgical possibilities (accessibility of the lesion) including their estimated outcome on the other hand. Therefore, careful patient selection and appropriate experience of the centre is warranted in the management of CCM.

  20. Management of Intracranial Hypertension

    PubMed Central

    Rangel-Castillo, Leonardo; Gopinath, Shankar; Robertson, Claudia S.

    2008-01-01

    Effective management of intracranial hypertension involves meticulous avoidance of factors that precipitate or aggravate increased intracranial pressure. When intracranial pressure becomes elevated, it is important to rule out new mass lesions that should be surgically evacuated. Medical management of increased intracranial pressure should include sedation, drainage of cerebrospinal fluid, and osmotherapy with either mannitol or hypertonic saline. For intracranial hypertension refractory to initial medical management, barbiturate coma, hypothermia, or decompressive craniectomy should be considered. Steroids are not indicated and may be harmful in the treatment of intracranial hypertension resulting from traumatic brain injury. PMID:18514825

  1. Pineal Cavernous Malformations: Report of Two Cases

    PubMed Central

    Kim, Dong-Seok; Shim, Kyu-Won; Kim, Tae-Gon; Chang, Jong-Hee; Park, Yong-Gou

    2005-01-01

    Pineal hemorrhage only occurs in rare cases, and this known to have several different causes such as germ cell tumors, pineal cysts and vascular malformations, including the cavernous malformations. Pineal cavernous malformations are extremely rare: to date only fifteen cases have been reported worldwide. Although the diagnosis of pineal cavernous malformation is not easy because of the extreme rareness of this condition, the presence of this lesion can be suspected based on its typical radiological findings. Case 1. A 42-year- old man presented with a limitation in his upward gazing. Radiologic examinations showed acute hemorrhage in the pineal region. He underwent ventriculo-peritoneal (VP) shunting but the patient's condition deteriorated after the shunting surgery. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. Case 2. A 37-year-old man presented with diplopia. Radiologic examinations showed acute hemorrhage in the third ventricle. He underwent VP shunting, and after this procedure the diplopia was aggravated. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. If there is no doubt about the pineal cavernous malformation on MR imaging, we strongly recommend early surgical intervention without performing a risky biopsy. In this study, we describe our experiences for the diagnosis of cavernous malformations in the pineal region with special emphasis on the radiological aspects and the clinical course of this disease. PMID:16385664

  2. Management strategies for neoplastic and vascular brain lesions presenting during pregnancy: A series of 29 patients

    PubMed Central

    Pereira, Celestino Esteves; Lynch, Jose Carlos

    2017-01-01

    Background: The occurrence of a brain tumor or intracranial vascular lesion during pregnancy is a rare event, but when it happens, it jeopardizes the lives of both the mother and infant. It also creates challenges of a neurosurgical, obstetric, and ethical nature. A multidisciplinary approach should be used for their care. Methods: Between 1986 and 2015, 12 pregnant women diagnosed with brain tumors and 17 women with intracranial vascular lesion underwent treatment at the Neurosurgery Department of the Servidores do Estado Hospital and Rede D’Or/São Luis. The Neurosurgery Department teamed up with Obstetrics Anesthesiology Departments in establishing the procedures. The patients’ records, surgical descriptions, imaging studies, and histopathological material were reviewed. Results: Among 12 patients presenting with brain tumors, there were neither operative mortality nor fetal deaths. Among the vascular lesions, aneurysm rupture was responsible for bleeding in 6 instances. Arteriovenous malformation was diagnosed in 7 patients. In this subgroup, the maternal and fetal mortality rates were 11.7% and 23.7%, respectively. Conclusions: We can assert that the association between a brain tumor and vascular lesions with pregnancy is a very unusual event, which jeopardizes both the lives of the mother and infant. It remains incompletely characterized due to the rare nature of these potentially devastating events. Knowing the exact mechanism responsible for the interaction of pregnancy and with these lesions will improve the treatment of these patients. PMID:28303207

  3. Intracranial aneurysms.

    PubMed

    Puskar, G; Ruggieri, P M

    1995-08-01

    MR angiography provides a rapid, accurate, and extremely flexible noninvasive evaluation of intracranial aneurysms without the cost and risk of conventional angiography. TOF and phase contrast techniques each have specific advantages and disadvantages that can be selectively exploited to optimize aneurysm evaluation. Present indications for MR angiography in aneurysm evaluation include: (1) the presence of incidental findings on a CT or MR examination that suggest the possibility of aneurysm (Figs. 7 and 8), (2) when angiography is contraindicated or when the risk is too high, (3) non-invasive follow-up of patients with known aneurysms, (4) patient refusal of contrast angiography, and (5) evaluation of patients with specific clinical symptoms (i.e., third cranial nerve palsy) or patients with non-specific subacute symptoms in whom an aneurysm might explain the clinical presentation. Although MR angiography certainly can detect aneurysms with a high rate of sensitivity and specificity, detailed decision analyses generally have not supported the overall benefit of this type of screening. Future technical advances as well as advances in the overall understanding of aneurysms may one day prove unequivocally the benefit of MR angiography in screening high-risk patient groups. MR angiography has not yet been clinically evaluated as a tool in the evaluation of acute subarachnoid hemorrhage. Potential obstacles to such an evaluation include the clinical instability of SAH patients, limited spatial resolution of the MR angiography acquisitions, the potential for subarachnoid blood or focal intraparenchymal hematomas to obscure or mimic small aneurysms, and the unreliability of MR angiography in demonstrating vasospasm. Currently these factors continue to provide an integral role for contrast angiography in aneurysm evaluation.

  4. Multifocal vascular lesions.

    PubMed

    Levin, Laura E; Lauren, Christine T

    2016-09-01

    Multifocal vascular lesions are important to recognize and appropriately diagnose. Generally first noticed on the skin, multifocal vascular lesions may have systemic involvement. Distinguishing among the different types of multifocal vascular lesions is often based on clinical features; however, radiological imaging and/or biopsy are frequently needed to identify distinct features and guide treatment. Knowledge of the systemic associations that can occur with different vascular anomalies may reduce life-threatening complications, such as coagulopathy, bleeding, cardiac compromise, and neurologic sequelae. This review provides a synopsis of the epidemiology, pathogenesis, presentation, workup, and treatment of several well-recognized multifocal vascular tumors and malformations.

  5. Massive Intracerebral Hemorrhage Caused by a Cavernous Malformation

    PubMed Central

    Kim, Young Soo; Lee, Jae Il; Choi, Chang Hwa

    2012-01-01

    We present a rare case of massive intracerebral hemorrhage resulting from a small, superficially-located supratentorial cavernous malformation, or cavernoma. These lesions rarely lead to massive, life-threatening intracerebral hemorrhages. A 17-year-old female presented with a 3-week history of declining mental status. Brain computed tomography and magnetic resonance imaging revealed a sizable intracranial hemorrhage, within the right occipital region, associated with a small nodule at the hematoma's posterior margin. An emergency operation removed the entire hematoma and nodule. Histological examination of the nodule was compatible with a diagnosis of cavernous malformation. The patient's post-operative course was uneventful. PMID:22396841

  6. Chiari type 1 malformation in a pseudotumour cerebri patient: is it an acquired or congenital Chiari malformation?

    PubMed

    Istek, Seref

    2014-06-04

    Chiari malformation type 1 (CM1) is a developmental abnormality of the cerebellar tonsils. Patients with CM1 commonly present with headache. Papilloedema is rarely seen in CM1. However, a 52-year-old woman presented to the hospital with a headache and her ophthalmological examination revealed bilateral papilloedema. Her cranial MRI was compatible with borderline CM1. Bilateral papilloedema and headache suggested idiopathic intracranial hypertension (IIH) as the preliminary diagnosis. IIH is a rare case in CM1. This article argues about this association and discusses as to whether it is an acquired or congenital Chiari malformation.

  7. 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.

  8. Pathogenesis of Vascular Anomalies

    PubMed Central

    Boon, Laurence M.; Ballieux, Fanny; Vikkula, Miikka

    2010-01-01

    Vascular anomalies are localized defects of vascular development. Most of them occur sporadically, i.e. there is no familial history of lesions, yet in a few cases clear inheritance is observed. These inherited forms are often characterized by multifocal lesions that are mainly small in size and increase in number with patient’s age. On the basis of these inherited forms, molecular genetic studies have unraveled a number of inherited mutations giving direct insight into the pathophysiological cause and the molecular pathways that are implicated. Genetic defects have been identified for hereditary haemorrhagic telangiectasia (HHT), inherited cutaneomucosal venous malformation (VMCM), glomuvenous malformation (GVM), capillary malformation - arteriovenous malformation (CM-AVM), cerebral cavernous malformation (CCM) and some isolated and syndromic forms of primary lymphedema. We focus on these disorders, the implicated mutated genes and the underlying pathogenic mechanisms. We also call attention to the concept of Knudson’s double-hit mechanism to explain incomplete penetrance and the large clinical variation in expressivity of inherited vascular anomalies. This variability renders the making of correct diagnosis of the rare inherited forms difficult. Yet, the identification of the pathophysiological causes and pathways involved in them has had an unprecedented impact on our thinking of their etiopathogenesis, and has opened the doors towards a more refined classification of vascular anomalies. It has also made it possible to develop animal models that can be tested for specific molecular therapies, aimed at alleviating the dysfunctions caused by the aberrant genes and proteins. PMID:21095468

  9. [Venous malformations: clinical characteristics and differential diagnosis].

    PubMed

    Casanova, D; Boon, L-M; Vikkula, M

    2006-01-01

    Venous malformations (VM) are localized defects of blood vessels that are due to vascular dysmorphogenesis. These slow-flow lesions can affect any tissue or organ. Clinically, a cutaneous VM is characterized by a bluish mass that is compressible on palpation. Phleboliths are commonly present. Symptoms depend on location and size. VM are often sporadic and isolated, however, they can be associated with other malformations and be part of a syndrome; Klippel-Trenaunay (capillary-lymphatico-venous malformation with limb hypertrophy) is the most common. Glomuvenous malformation (GVM) is another type of venous anomaly. In contrast to VM, GVM is often painful on palpation and not compressible. Clinical diagnosis of VM is often made in the presence of a bluish cutaneous lesion: however, other lesions can mimick VM. The most frequent anomalies are a blue naevus, a hemorrhagic lymphatic malformation, a sub-cutaneous hemangioma or even the presence of dilated superficial normal veins due to underlying venous stenoses. This chapter will detail the clinical characteristics of venous anomalies and their differential diagnosis.

  10. Glioma coexisting with angiographically occult cerebrovascular malformation: A case report

    PubMed Central

    Chen, Junhui; Chen, Lei; Zhang, Chunlei; He, Jianqing; Li, Peipei; Zhou, Jingxu; Zhu, Jun; Wang, Yuhai

    2016-01-01

    Angiographically occult cerebrovascular malformation (AOVM) is a type of complex cerebrovascular malformation that is not visible on digital subtraction angiography (DSA). Vascular malformation coexisting with glioma is clinically rare, and glioma coexisting with AOVM is even more rare. To the best of our knowledge, the present study is the first to report glioma coexisting with AOVM in the literature. The present study reports a rare case of glioma coexisting with AOVM in a 30-year-old male patient. Computed tomography (CT) scan revealed calcification, hemorrhage and edema in the right frontal lobe. CT angiography revealed a vascular malformation in the right frontal lobe, which was not observed on DSA. Finally, glioma coexisting with AOVM was confirmed by 2.0T magnetic resonance imaging and postoperative pathological examination. The present patient had a positive outcome and no neurological dysfunctions during the 6-month follow-up subsequent to surgery. PMID:27698825

  11. Malformations of cortical development: genetic mechanisms and diagnostic approach

    PubMed Central

    2017-01-01

    Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development. PMID:28203254

  12. Malformations of cortical development: genetic mechanisms and diagnostic approach.

    PubMed

    Lee, Jeehun

    2017-01-01

    Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development.

  13. Vascular anomalies: classification, imaging characteristics and implications for interventional radiology treatment approaches

    PubMed Central

    Prajapati, H J S; Martin, L G; Patel, T H

    2014-01-01

    The term vascular anomaly represents a broad spectrum of vascular pathology, including proliferating vascular tumours and vascular malformations. While the treatment of most vascular anomalies is multifactorial, interventional radiology procedures, including embolic therapy, sclerotherapy and laser coagulation among others, are playing an increasingly important role in vascular anomaly management. This review discusses the diagnosis and treatment of common vascular malformations, with emphasis on the technique, efficacy and complications of different interventional radiology procedures. PMID:24588666

  14. Capillary malformation-arteriovenous malformation: a clinical review of 45 patients.

    PubMed

    Larralde, Margarita; Abad, María Eugenia; Luna, Paula Carolina; Hoffner, Mariana Viktoria

    2014-04-01

    Capillary malformation-arteriovenous malformation (CM-AVM) is a recently described autosomal dominant disorder that results from mutations in RASA1. It has been initially described as multiple CMs affecting several members of the same family, associated with fast-flow malformations in at least one family member. To report and analyze clinical data on 45 patients with CM-AVM assessed at the Department of Pediatric Dermatology, Ramos Mejía Hospital (Buenos Aires, Argentina). Retrospective clinical review of all the patients clinically diagnosed as having CM-AVM over a period of eight years. Forty-five patients were recorded (24 females and 21 males). The age ranged from one month to 44 years. In 36 patients, the stains were congenital; progressive acquired lesions were observed in 39. Family history was positive in 32 subjects. Well defined, round to oval, pink-purple or reddish-brown macules were found in all the patients; pinpoint red lesions with a pale halo were found in nine cases. The macules were warmer than normal skin in 15 cases and surrounded by a white halo in 26 cases. Three subjects presented associated overgrowth, lymphatic malformation was present in one case, retinal vascular lesion in one patient, and isolated port wine stain in two cases. Three patients also had infantile hemangioma. We had no cases of fast-flow vascular malformation or combined vascular syndromes. CM-AVM is a heterogeneous disorder with phenotypic variability, from fast-flow malformation, limb enlargement, or Parkes Weber syndrome to multiple CMs without internal involvement. © 2013 The International Society of Dermatology.

  15. Arteriovenous malformations: epidemiology and clinical presentation.

    PubMed

    Laakso, Aki; Hernesniemi, Juha

    2012-01-01

    Arteriovenous malformations (AVMs) of the brain are relatively rare congenital developmental vascular lesions. They may cause hemorrhagic stroke, epilepsy, chronic headache, or focal neurologic deficits, and the incidence of asymptomatic AVMs is increasing due to widespread availability of noninvasive imaging methods. Since the most severe complication of an AVM is hemorrhagic stroke, most epidemiologic studies have concentrated on the hemorrhage risk and its risk factors. In this article, the authors discuss the epidemiology, presenting symptoms, and hemorrhage risk associated with brain AVMs.

  16. Embolization and radiosurgery for arteriovenous malformations

    PubMed Central

    Plasencia, Andres R.; Santillan, Alejandro

    2012-01-01

    The treatment of arteriovenous malformations (AVMs) requires a multidisciplinary management including microsurgery, endovascular embolization, and stereotactic radiosurgery (SRS). This article reviews the recent advancements in the multimodality treatment of patients with AVMs using endovascular neurosurgery and SRS. We describe the natural history of AVMs and the role of endovascular and radiosurgical treatment as well as their interplay in the management of these complex vascular lesions. Also, we present some representative cases treated at our institution. PMID:22826821

  17. Fetal Magnetic Resonance Imaging of Malformations Associated with Heterotaxy

    PubMed Central

    Shah, Parinda H; Anderson, Robert H

    2015-01-01

    Magnetic resonance imaging (MRI) is increasingly used as an investigation during fetal life, particularly for assessment of intracranial masses, congenital diaphragmatic hernia, myelomeningocele, and abdominal masses. As the number of scans increases, so is the variety of congenital malformations being recognized. It is axiomatic that interpretation of the findings is enhanced when attention is paid to the likely findings in the setting of known syndromes, this information then dictating the need for additional acquisition of images. One such syndrome is so-called “visceral heterotaxy”, in which there is typically an isomeric, rather than a lateralized, arrangement of the thoracic and abdominal organs. Typically associated with complex congenital cardiac malformations, heterotaxy can also involve the central nervous system, and produce pulmonary, gastrointestinal, immunologic, and genitourinary malformations. In this review, we discuss how these findings can be demonstrated using fetal MRI.  PMID:26180693

  18. Increased intracranial pressure

    MedlinePlus

    ... brain. Many conditions can increase intracranial pressure. Common causes include: Aneurysm rupture and subarachnoid hemorrhage Brain tumor Encephalitis Head injury Hydrocephalus (increased fluid around ...

  19. Cerebral Cavernous Malformations (CCM)

    MedlinePlus

    ... Contact Registry Interest Form Contact Us | Login Disorder Definitions Learn More > Disorder Definitions Cerebral Cavernous Malformations (CCM) ... until it is too late to salvage vision. Routine screening is very important, even if there are ...

  20. Multiple spinal cavernous malformations in Klippel-Trenaunay-Weber syndrome.

    PubMed

    Göksu, Ethem; Alpsoy, Erkan; Uçar, Tanju; Tuncer, Recai

    2012-01-01

    Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital vascular disorder characterized by cutaneous haemangiomas, venous varicosities, and hypertrophy of the osseous and soft tissue. Various vascular anomalies of the central nervous system have been described in this syndrome. Two previous associations between KTWS and spinal cord cavernous malformations have been reported in the English literature. In this report, we present a patient in whom multiple cavernous malformations located in the conus medullaris region and cauda equina were associated with KTWS. General physical examination as well as neuroradiological and operative findings are described.

  1. A method for semi-automatic segmentation and evaluation of intracranial aneurysms in bone-subtraction computed tomography angiography (BSCTA) images

    NASA Astrophysics Data System (ADS)

    Krämer, Susanne; Ditt, Hendrik; Biermann, Christina; Lell, Michael; Keller, Jörg

    2009-02-01

    The rupture of an intracranial aneurysm has dramatic consequences for the patient. Hence early detection of unruptured aneurysms is of paramount importance. Bone-subtraction computed tomography angiography (BSCTA) has proven to be a powerful tool for detection of aneurysms in particular those located close to the skull base. Most aneurysms though are chance findings in BSCTA scans performed for other reasons. Therefore it is highly desirable to have techniques operating on standard BSCTA scans available which assist radiologists and surgeons in evaluation of intracranial aneurysms. In this paper we present a semi-automatic method for segmentation and assessment of intracranial aneurysms. The only user-interaction required is placement of a marker into the vascular malformation. Termination ensues automatically as soon as the segmentation reaches the vessels which feed the aneurysm. The algorithm is derived from an adaptive region-growing which employs a growth gradient as criterion for termination. Based on this segmentation values of high clinical and prognostic significance, such as volume, minimum and maximum diameter as well as surface of the aneurysm, are calculated automatically. the segmentation itself as well as the calculated diameters are visualised. Further segmentation of the adjoining vessels provides the means for visualisation of the topographical situation of vascular structures associated to the aneurysm. A stereolithographic mesh (STL) can be derived from the surface of the segmented volume. STL together with parameters like the resiliency of vascular wall tissue provide for an accurate wall model of the aneurysm and its associated vascular structures. Consequently the haemodynamic situation in the aneurysm itself and close to it can be assessed by flow modelling. Significant values of haemodynamics such as pressure onto the vascular wall, wall shear stress or pathlines of the blood flow can be computed. Additionally a dynamic flow model can be

  2. Shining Pearls Sign: A New Identity for Venous Malformations on Computed Tomographic Imaging.

    PubMed

    Bhat, Venkatraman; Bhat, Varun

    2016-12-01

    Vascular malformations, in particular venous malformations (VM), are common lesions involving the pediatric and adolescent population. VM occur at approximately 1:5,000 to 10,000; approximately 40% of them occur in the head and neck regions. Classical appearance of VM on imaging is a demonstration of near-normal-sized or mildly dilated feeding arteries which subsequently lead to dilated venous structures of varying caliber, thus constituting the malformation. Phleboliths are the hallmark of VM. Plain radiography has been an established modality for demonstrating phleboliths. The emergence of computed tomographic (CT) as a preferred imaging modality for the evaluation of a complex vascular malformation necessitates familiarity with the spectrum of CT appearances of this lesion. This presentation illustrates an additional CT sign, "shining pearls sign," highlighting the striking display of phleboliths in the vascular malformations, prompting correct diagnosis.

  3. Mechanisms of vascular stability and the relationship to human disease

    PubMed Central

    Smith, Matthew C.P.; Li, Dean Y.; Whitehead, Kevin J.

    2010-01-01

    Purpose of review The genetic basis for a variety of vascular malformation syndromes have been described, with an increasing functional understanding of the associated genes. Recent findings Genes responsible for familial vascular malformation syndromes have increasingly been shown to be involved in the control of vascular stability. Summary Genes involved in vascular stability pathways are good candidates for causing vascular malformation syndromes. Although these findings confirm the biologic importance of the involved pathways, further explanations are required to describe the focal nature of disease. PMID:20308891

  4. 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).

  5. Angioplasty and Stenting for Atherosclerotic Intracranial Stenosis: Rationale for a Randomized Clinical Trial

    PubMed Central

    Derdeyn, Colin P.; Chimowitz, Marc I.

    2007-01-01

    Synopsis Atherosclerotic disease of the major intracranial arteries is a frequent cause of stroke. In addition, many patients with symptomatic intracranial stenosis are at very high risk for recurrent stroke. A recently completed medical treatment trial, the Warfarin versus Aspirin for Symptomatic Intracranial Stenosis (WASID) trial, showed that aspirin was as effective and safer than warfarin for preventing stroke or vascular death in these patients, and that patients with 70%-99% intracranial stenosis are at particularly high risk of stroke despite antithrombotic therapy and usual management of vascular risk factors. Preliminary studies suggest that angioplasty and stenting may reduce the risk of stroke in patients with severe stenosis of intracranial arteries. However, data for angioplasty and stenting consists of case series: no randomized studies have been completed to date. These data will be reviewed and the rationale for a randomized trial of angioplasty and stenting versus best medical management for patients with symptomatic intracranial stenosis will be discussed. PMID:17826637

  6. RASA1 mutations may cause hereditary capillary malformations without arteriovenous malformations.

    PubMed

    Hershkovitz, D; Bercovich, D; Sprecher, E; Lapidot, M

    2008-05-01

    Capillary malformation (CM), a common vascular abnormality, is often present among family members. Recently a rare form of hereditary vascular malformation termed capillary malformation-arteriovenous malformation (CM-AVM) was shown to be caused by heterozygous mutations in RASA1, encoding RAS p21 protein activator 1. CM-AVM is characterized by multiple, small CMs associated with either AVM or arteriovenous fistula (AVF) in affected individuals or at least one of their family members. The purpose of the study was to find out whether CMs in the absence of AVM/AVF are associated with RASA1 mutations. We assessed three families comprising 14 affected individuals with CMs. Linkage to the RASA1 locus was evaluated using microsatellite markers. The RASA1 gene was scrutinized for pathogenic mutations using denaturing high-performance liquid chromatography screening and direct sequencing. AVM/AVF was identified in one of three affected families. CM without AVM/AVF was found to map in one large kindred to the RASA1 locus. Direct sequencing revealed novel heterozygous mutations segregating with CM in all three families. The mutations are predicted to result in premature termination of translation and RASA1 haplo-insufficiency. We have demonstrated that the spectrum of clinical manifestations due to mutations in RASA1 is wider than previously thought and also includes typical CMs not associated with AVM/AVF.

  7. Neurofibromatosis type 1 with intracranial hemorrhage and horseshoe kidney.

    PubMed

    Jat, Kana Ram; Marwaha, Ram Kumar; Panigrahi, Inusha; Gupta, Vivek

    2008-10-01

    A 12-year-old boy presented with a history of sudden-onset vomiting, headache, and giddiness. Two members of his family manifested neurofibromatosis type 1. On examination, the child had multiple café-au-lait spots, bilateral axillary freckles, and Lisch nodules in both eyes. A central nervous system examination revealed raised intracranial pressure. Computed tomography of the cranium revealed an intracranial hemorrhage in the right parietal region, without a midline shift. Magnetic resonance imaging of the brain revealed a hemorrhage and a neurofibromatosis bright object. Magnetic resonance angiography and digital subtraction angiography revealed no evidence of arteriovenous malformation or aneurysm. Ultrasonography of the abdomen revealed a horseshoe kidney, as confirmed by a 99m technetium dimercaptosuccinic acid renal cortical scan. He responded to treatment for the raised intracranial pressure, and remained asymptomatic during follow-up.

  8. Congenital plaque-type glomuvenous malformations associated with fetal pleural effusion and ascites.

    PubMed

    Goujon, Elisa; Cordoro, Kelly M; Barat, Muriel; Rousseau, Thierry; Brouillard, Pascal; Vikkula, Miikka; Frieden, Ilona J; Vabres, Pierre

    2011-01-01

    Glomuvenous malformations are hereditary vascular anomalies, usually without extracutaneous involvement. We report two cases of extensive thoracic plaque-type glomuvenous malformation in newborns who had previously been diagnosed in utero with pleural effusion and ascites, suggesting a pathogenic link between the two conditions.

  9. Malformations of Cortical Development: From Postnatal to Fetal Imaging.

    PubMed

    Lerman-Sagie, Tally; Leibovitz, Zvi

    2016-09-01

    Abnormal fetal corticogenesis results in malformations of cortical development (MCD). Abnormal cell proliferation leads to microcephaly or megalencephaly, incomplete neuronal migration results in heterotopia and lissencephaly, neuronal overmigration manifests as cobblestone malformations, and anomalous postmigrational cortical organization is responsible for polymicrogyria and focal cortical dysplasias. MCD comprises various congenital brain disorders, caused by different genetic, infectious, or vascular etiologies and is associated with significant neurological morbidity. Although MCD are rarely diagnosed prenatally, both dedicated multiplanar neurosonography and magnetic resonance imaging enable good demonstration of fetal cortical development. The imaging signs of fetal MCD are: delayed or absent cerebral sulcation; premature abnormal sulci; thin and irregular hemispheric parenchyma; wide abnormal overdeveloped gyri; wide opening of isolated sulci; nodular bulging into the lateral ventricles; cortical clefts; intraparenchymal echogenic nodules; and cortical thickening. The postnatal and prenatal imaging features of four main malformations of cortical development-lissencephaly, cobblestone malformations, periventricular nodular heterotopia, and polymicrogyria-are described.

  10. Arteriovenous malformation in chronic gastrointestinal bleeding.

    PubMed Central

    Cavett, C M; Selby, J H; Hamilton, J L; Williamson, J W

    1977-01-01

    Arteriovenous malformations of the gastrointestinal tract are uncommon and treatment is problematic because routine barium contrast studies and endoscopy fail to demonstrate the lesion. Diagnosis is by selective mesenteric arteriography, demonstrating a characteristic vascular tuft and very early venous phase. Two cases of arteriovenous malformation are presented and 47 other reported cases are reviewed. Forty-five per cent were found in the cecum; 37, or 80%, involved the distal ileum, cecum ascending colon, or hepatic flexure. Seventy-five per cent of all patients fall into the 50--80 year age range. The literature reveals a recurring pattern of chronic gastrointestinal blood loss, anemia, and delay (even negative abdominal explorations) before the diagnosis is finally made. A more aggressive approach to chronic gastrointestinal bleeding is suggested through the use of selective mesenteric arteriography. Images Fig. 1. Fig. 2. Fig. 3. Fig. 4. Fig. 5. PMID:299801

  11. [Idiopathic intracranial hypertension].

    PubMed

    Bäuerle, J; Egger, K; Harloff, A

    2017-02-01

    This review describes the clinical findings as well as thes diagnostic and therapeutic options for idiopathic intracranial hypertension (pseudotumor cerebri). Furthermore, the pathophysiological concepts are discussed. Idiopathic intracranial hypertension is characterized by signs and symptoms of raised intracranial pressure with no established pathogenesis. Common symptoms include headaches, visual loss and pulsatile tinnitus. Treatment has two major goals: the alleviation of headaches and the preservation of vision. Weight loss and acetazolamide are the cornerstones in the treatment of the disorder. Drainage of cerebrospinal fluid, optic nerve sheath fenestration and stent angioplasty of a sinus stenosis can be employed in severe cases.

  12. Intracranial dural based chondroma.

    PubMed

    Reinshagen, Clemens; Redjal, Navid; Sajed, Dipti P; Nahed, Brian V; Walcott, Brian P

    2016-03-01

    Intracranial chondromas are benign, slow-growing, cartilaginous tumors, which comprise only about 0.2% of all intracranial tumors. The majority of these lesions occur at the base of the skull, where they are thought to arise from residual embryonic chondrogenic cells along the basal synchondrosis. Very rarely, they may also originate from the convexity dura, falx cerebri, or the brain parenchyma. We present a patient with a dural based chondroma to highlight the technical considerations of surgical resection. The recent literature on intracranial chondromas regarding incidence, pathophysiologic origin, clinical symptoms, imaging, histopathology and prognosis is reviewed.

  13. Spitz nevus arising upon a congenital glomuvenous malformation.

    PubMed

    Arica, Deniz A; Arica, Ibrahim E; Yayli, Savas; Cobanoglu, Umit; Akay, Bengu N; Anadolu, Rana; Bahadir, Sevgi

    2013-01-01

    There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.

  14. Bleomycin sclerotherapy for lymphatic malformation after unsuccessful surgical excision: case report.

    PubMed

    Vlahovic, A; Gazikalovic, A; Adjic, O

    2015-10-01

    Lymphatic malformations (LMs) are benign cystic masses resulting from the abnormal development of lymphatic channels. Lymphatic malformations occur primarily in the head and neck region. Surgical excision of lymphatic malformation is followed by high rate of recurrence and a high risk of complications. Bleomycin is an established antineoplastic drug. It can be used as a sclerosing agent in vascular anomalies. We present a child who was unsuccessfully treated with four surgical resections, with peripheral palsy of facial nerve as complication. The lymphatic malformation was successfully treated in our institution with intralesional administration of bleomycin.

  15. Intracranial pressure monitoring

    MedlinePlus

    ... head. The monitor senses the pressure inside the skull and sends measurements to a recording device. ... are 3 ways to monitor pressure in the skull (intracranial pressure). INTRAVENTRICULAR CATHETER The intraventricular catheter is ...

  16. Hidradenoma with intracranial involvement.

    PubMed

    Bradbury, P G; Diengdoh, J V; Crockard, H A; Stern, G M

    1984-06-01

    A case of recurrent hidradenoma of the external ear with intracranial spread is described. The presentation, classification and management of this rare tumour are discussed and the importance of adequate long term review is stressed.

  17. Peripheral intracranial aneurysms: management challenges in 60 consecutive cases.

    PubMed

    Nussbaum, Eric S; Madison, Michael T; Goddard, James K; Lassig, Jeffrey P; Nussbaum, Leslie A

    2009-01-01

    The authors report the management and outcomes of 55 patients with 60 intracranial aneurysms arising distal to the major branch points of the circle of Willis and vertebrobasilar system. Between July 1997 and December 2006, the authors' neurovascular service treated 2021 intracranial aneurysms in 1850 patients. The database was reviewed retrospectively to identify peripherally located intracranial aneurysms. Aneurysms that were mycotic and aneurysms that were associated with either an arteriovenous malformation or an atrial myxoma were excluded from review. The authors encountered 60 peripheral intracranial aneurysms in 55 patients. There were 42 small, 7 large, and 11 giant lesions. Forty-one (68%) were unruptured, and 19 (32%) had bled. Fifty-three aneurysms were treated surgically by using direct clip reconstruction in 26, trapping or proximal occlusion with distal revascularization in 21, excision with end-to-end anastomosis in 3, and circumferential wrap/clip reconstruction in 3. Coils were used to treat 6 aneurysms, and 1 was treated by endovascular parent artery occlusion. Overall, 49 patients had good outcomes, 4 were left with new neurological deficits, and 2 died. Peripherally situated intracranial aneurysms are rare lesions that present unique management challenges. Despite the fact that in the authors' experience these lesions were rarely treatable with simple clipping of the aneurysm neck or endovascular coil occlusion, preservation of the parent artery was possible in most cases, and the majority of patients had a good outcome.

  18. Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies.

    PubMed

    Wassef, Michel; Blei, Francine; Adams, Denise; Alomari, Ahmad; Baselga, Eulalia; Berenstein, Alejandro; Burrows, Patricia; Frieden, Ilona J; Garzon, Maria C; Lopez-Gutierrez, Juan-Carlos; Lord, David J E; Mitchel, Sally; Powell, Julie; Prendiville, Julie; Vikkula, Miikka

    2015-07-01

    Vascular anomalies represent a spectrum of disorders from a simple "birthmark" to life- threatening entities. Incorrect nomenclature and misdiagnoses are commonly experienced by patients with these anomalies. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Classification schemes provide a consistent terminology and serve as a guide for pathologists, clinicians, and researchers. One of the goals of the International Society for the Study of Vascular Anomalies (ISSVA) is to achieve a uniform classification. The last classification (1997) stratified vascular lesions into vascular malformations and proliferative vascular lesions (tumors). However, additional disease entities have since been identified that are complex and less easily classified by generic headings, such as capillary malformation, venous malformation, lymphatic malformation, etc. We hereby present the updated official ISSVA classification of vascular anomalies. The general biological scheme of the classification is retained. The section on tumors has been expanded and lists the main recognized vascular tumors, classified as benign, locally aggressive or borderline, and malignant. A list of well-defined diseases is included under each generic heading in the "Simple Vascular Malformations" section. A short definition is added for eponyms. Two new sections were created: one dealing with the malformations of individually named vessels (previously referred to as "truncular" malformations); the second groups lesions of uncertain or debated nature (tumor versus malformation). The known genetic defects underlying vascular anomalies are included in an appendix. This classification is meant to be a framework, acknowledging that it will require modification as new scientific information becomes available. Copyright © 2015 by the American Academy of Pediatrics.

  19. Echoing Plaque Activity of the Coronary and Intracranial Arteries in Patients With Stroke.

    PubMed

    Chung, Jong-Won; Bang, Oh Young; Lee, Mi Ji; Hwang, Jaechun; Cha, Jihoon; Choi, Jin-Ho; Choe, Yeon Hyeon

    2016-06-01

    Atherosclerosis is a systemic disease, and both coronary and intracranial atherosclerosis are common in the elderly. Unlike coronary artery disease (CAD), intracranial atherosclerotic disease can cause intracranial atherosclerotic stroke by branch occlusive disease (B-type) and coronary-type rupture of plaque (C-type). We hypothesized that plaque characteristics of intracranial arteries are associated with those of coronary arteries. Eighty-one patients with acute cerebral infarcts caused by intracranial atherosclerotic disease without history of CAD were analyzed. Asymptomatic CAD burden (number and degree of stenosis) and plaque characteristics (calcified, mixed, and noncalcified) were measured with multidetector computed tomography, whereas the asymptomatic intracranial atherosclerotic disease burden was measured using magnetic resonance angiography. The symptomatic intracranial artery was analyzed using high-resolution magnetic resonance imaging for vascular morphology (stenosis degree, remodeling index, and wall index) and plaque activation (enhancement pattern and volume). The asymptomatic CAD burden was correlated with the asymptomatic intracranial atherosclerotic disease burden. The overall CAD burden did not differ between B- and C-type intracranial atherosclerotic stroke. However, the prevalence of noncalcified coronary plaque was much higher in C-type intracranial atherosclerotic stroke and the presence of coronary noncalcified plaque was independently associated with C-type intracranial atherosclerotic stroke (odds ratio, 3.38; 95% confidence interval, 1.05-10.85; P=0.041). As the number of coronary noncalcified plaques increased, positive remodeling and plaque enhancement increased in the symptomatic intracranial artery on high-resolution magnetic resonance imaging. Plaques within the intracranial and coronary arteries behave in similar ways. Our results suggest the need to evaluate and treat other vascular trees in patients with vulnerable plaques

  20. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance.

    PubMed

    Gault, Judith; Sarin, Hemant; Awadallah, Nabil A; Shenkar, Robert; Awad, Issam A

    2004-07-01

    Cerebrovascular malformations affect more than 3% of the population, exposing them to a lifetime risk of hemorrhagic stroke, seizures, and focal neurological deficits. Cerebral cavernous malformations (CCMs) exhibit an immature vessel wall, a brittle hemorrhagic tendency, and epileptogenesis, whereas arteriovenous malformations (AVMs) lack capillary beds and manifest apoplectic bleeding under high-flow conditions. There are also more benign venous anomalies, capillary malformations, and lesions with mixed and transitional features. Advances have been made toward understanding the natural history, radiological and pathological correlates, and clinical management. Yet, mechanisms of lesion genesis and clinical manifestations remain largely unknown, and the clinical behavior in individual patients is highly unpredictable. Lesion pathogenesis likely involves abnormal assembly or maintenance of blood vessels, resulting in dysmorphic vessel phenotypes. Familial CCM disease is in part caused by mutations in a cytoskeletal-related protein that is likely integral to interendothelial cell connectivity and maturation of the vascular wall. Rare familial forms of AVM disease have been correlated with two different transforming growth factor-beta receptor components, possibly causing disturbance in signaling during vascular assembly. Relevance of these mechanisms to the more common and otherwise identical sporadic CCM and AVM lesions is being explored. In this report, basic mechanisms of vasculogenesis and angiogenesis and how they possibly relate to the common cerebrovascular malformation lesions are reviewed. Novel concepts are discussed related to the cellular, molecular, and genetic substrates in CCM and AVM as well as to how this knowledge can be applied to predict, explain, and possibly modify clinical disease manifestations.

  1. Intracranial Artery Calcification and Its Clinical Significance

    PubMed Central

    Wu, Xiao Hong; Wang, Li Juan; Wong, Ka Sing

    2016-01-01

    Intracranial arterial calcification (IAC) is an easily identifiable entity on plain head computed tomography scans. Recent studies have found high prevalence rates for IAC worldwide, and this may be associated with ischemic stroke and cognitive decline. Aging, traditional cardiovascular risk factors, and chronic kidney disease have been found to be associated with IAC. The severity of IAC can be assessed using different visual grading scales or various quantitative methods (by measuring volume or intensity). An objective method for assessing IAC using consistent criteria is urgently required to facilitate comparisons between multiple studies involving diverse populations. There is accumulating evidence from clinical studies that IAC could be utilized as an indicator of intracranial atherosclerosis. However, the pathophysiology underlying the potential correlation between IAC and ischemic stroke—through direct arterial stenosis or plaque stability—remains to be determined. More well-designed clinical studies are needed to explore the predictive values of IAC in vascular events and the underlying pathophysiological mechanisms. PMID:27165425

  2. Ventricular Tract Hemorrhage Following Intracranial Nail Removal: Utility of Real-time Endovascular Assistance

    PubMed Central

    Rennert, Robert C.; Steinberg, Jeffrey A.; Sack, Jayson; Pannell, J. Scott; Khalessi, Alexander A.

    2016-01-01

    Penetrating brain trauma commonly results in occult neurovascular injury. Detailed cerebrovascular imaging can evaluate the relationship of intracranial foreign bodies to major vascular structures, assess for traumatic pseudoaneurysms, and ensure hemostasis during surgical removal. We report a case of a self-inflicted intracranial nail gun injury causing a communicating ventricular tract hemorrhage upon removal, as well as a delayed pseudoaneurysm. Pre- and post-operative vascular imaging, as well as intra-operative endovascular assistance, was critical to successful foreign body removal in this patient. This report demonstrates the utility of endovascular techniques for the assessment and treatment of occult cerebrovascular injuries from intracranial foreign bodies. PMID:27471490

  3. Arteriovenous malformation in temporal lobe presenting as contralateral ocular symptoms mimicking carotid-cavernous fistula.

    PubMed

    Mohd-Tahir, Fadzillah; Siti-Raihan, Ishak; Wan Hazabbah, W H

    2013-01-01

    Aim. To report a rare case of arteriovenous malformation in temporal lobe presenting as contralateral orbital symptoms mimicking carotid-cavernous fistula. Method. Interventional case report. Results. A 31-year-old Malay gentleman presented with 2-month history of painful progressive exophthalmos of his left eye associated with recurrent headache, diplopia, and reduced vision. Ocular examination revealed congestive nonpulsating 7 mm exophthalmos of the left eye with no restriction of movements in all direction. There was diplopia in left lateral gaze. Left IOP was elevated at 29 mmHg. Left eye retinal vessels were slightly dilated and tortuous. CT scan was performed and showed right temporal arteriovenous malformation with a nidus of 3.8 cm × 2.5 cm with right middle cerebral artery as feeding artery. There was dilated left superior ophthalmic vein of 0.9 mm in diameter with enlarged left cavernous sinus. MRA and carotid angiogram confirmed right temporal arteriovenous malformation with no carotid-cavernous fistula. Most of the intracranial drainage was via left cavernous sinus. His signs and symptoms dramatically improved following successful embolisation, completely resolved after one year. Conclusion. Intracranial arteriovenous malformation is rarely presented with primary ocular presentation. Early intervention would salvage the eyes and prevent patients from more disaster morbidity or fatality commonly due to intracranial haemorrhage.

  4. 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.

  5. A review of cavernous malformations with trigeminal neuralgia.

    PubMed

    Adachi, Kazuhide; Hasegawa, Mitsuhiro; Hayashi, Takuro; Nagahisa, Shinya; Hirose, Yuichi

    2014-10-01

    Cavernous malformation with trigeminal neuralgia is relatively rare; only 10 cases have been reported. In deciding treatment strategies, it is helpful to classify cavernous malformation according to its origin, as follows: in the Gasserian ganglion (Type G); between the cisternal and intra-axial portions of the trigeminal nerve root (Type C); in the intra-axial trigeminal nerve root in the pons (Type P); or in the spinal tract of the trigeminal nerve root (Type S). A 62-year-old male presented with left trigeminal neuralgia (V2 area) and left facial hypoesthesia. Imaging studies revealed a cerebellopontine angle mass lesion with characteristics of a cavernous malformation and evidence of hemorrhage. The lesion was completely removed via a left anterior transpetrosal approach. The mass was attached to the trigeminal nerve root; it was located between the cisternal and intra-axial portions of the nerve root, and feeding off microvessels from the trigeminal nerve vascular plexus. Histological examination confirmed a cavernous malformation. In this case, the cavernous malformation was Type C. We review cases of cavernous malformation with trigeminal neuralgia and discuss therapeutic strategies according to the area of origin. Copyright © 2014 Elsevier B.V. All rights reserved.

  6. 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

  7. Congenital cardiovascular malformations and the fetal circulation.

    PubMed

    Rudolph, A M

    2010-03-01

    After birth, gas exchange is achieved in the lung, whereas prenatally it occurs in the placenta. This is associated with differences in blood flow patterns in the fetus as compared with the postnatal circulation. Congenital cardiovascular malformations are associated with haemodynamic changes in the fetus, which differ from those occurring postnatally. Obstruction to cardiac outflow may alter myocardial development, resulting in progressive ventricular hypoplasia. Alteration of oxygen content may profoundly influence pulmonary vascular and ductus arteriosus responses. Interference in blood flow and oxygen content may affect cerebral development as a result of inadequate oxygen or energy substrate supply. The circulatory effects may be gestational dependent, related to maturation of vascular responses in different organs. These prenatal influences of congenital cardiac defects may severely affect immediate, as well as longterm, postnatal prognosis and survival. This has stimulated the development of techniques for palliation of disturbed circulation during fetal life.

  8. Large pure intracranial vagal schwannoma.

    PubMed

    Gazzeri, Roberto; Galarza, Marcelo; Costanzo, De Bonis; Carotenuto, Vincenzo; D'Angelo, Vincenzo

    2009-04-01

    We report a patient with a large, pure intracranial vagal schwannoma, compressing the medulla who presented with essential hypertension. Based on this and on previous cases, we suggest that a differentiation of pure intracranial schwannomas (subtype A1) from intracranial schwannomas with some extension in the jugular foramen (type A) should be used.

  9. Cerebral cavernous malformations: from molecular pathogenesis to genetic counselling and clinical management.

    PubMed

    Haasdijk, Remco A; Cheng, Caroline; Maat-Kievit, Anneke J; Duckers, Henricus J

    2012-02-01

    Cerebral cavernous (or capillary-venous) malformations (CCM) have a prevalence of about 0.1-0.5% in the general population. Genes mutated in CCM encode proteins that modulate junction formation between vascular endothelial cells. Mutations lead to the development of abnormal vascular structures.In this article, we review the clinical features, molecular and genetic basis of the disease, and management.

  10. Intracranial blister aneurysms: clip reconstruction techniques.

    PubMed

    Barrow, Daniel L; Pradilla, Gustavo; McCracken, D Jay

    2015-07-01

    Intracranial blister aneurysms are difficult to treat cerebrovascular lesions that typically affect the anterior circulation. These rare aneurysms can lead to acute rupture which usually cannot be treated via endovascular methods, but still require urgent surgical intervention. Surgical options are limited given their unique pathology and often require a combination of wrapping and clip reconstruction. In this video we present two patients with acute subarachnoid hemorrhage secondary to ruptured blister aneurysms. We demonstrate several surgical techniques for repairing the vascular defect with and without intraoperative rupture. The video can be found here: http://youtu.be/nz-JM45uKQU.

  11. [A temporal head injury involving intracranial penetration by glass].

    PubMed

    Nakagawa, Atsuhiro; Su, Ching-Chan; Yamashita, Yoji; Endo, Toshiki; Shirane, Reizo

    2002-05-01

    The authors report a rare case of intracranial glass injury due to a temporal head injury. This 72-year-old man slipped on a bathroom floor, impacting a glass door with his head and right shoulder. His right temporal scalp and right shoulder were cut by the broken glass. He visited our emergency unit four hours after sustaining the injury. Physical and neurological examinations showed no abnormalities except for two lacerated wounds on both the right temporal scalp (1.5 cm) and the right shoulder skin (10 cm). Foreign bodies were not palpable around the lacerated wounds. Skull X-ray and CT studies disclosed a single, 5-cm long, radiopaque foreign body penetrating the temporal skull bone into the right temporal lobe, but no evidence of intracranial bleeding was found. Under the diagnosis of intracranial glass injury, total removal of the foreign body with dural repair was carried out. On surgical exploration, glass penetrating the skull bone 5-mm distant anteriorly to the scalp laceration was observed. Postoperative angiography showed no vascular lesions, and one-week later he was discharged with no complications. According to the literature, most of the intracranial foreign bodies occur around the orbital, the frontal sinus, and the nasal areas. To our knowledge, this is the first report of an intracranial glass penetrating injury to the temporal lobe. Since the clinical manifestations occasionally do not correspond to the appearance of the laceration after glass penetrating injuries, serious caution concerning patients with intracranial glass penetrating injuries is important.

  12. Uterine Vascular Lesions

    PubMed Central

    Vijayakumar, Abhishek; Srinivas, Amruthashree; Chandrashekar, Babitha Moogali; Vijayakumar, Avinash

    2013-01-01

    Vascular lesions of the uterus are rare; most reported in the literature are arteriovenous malformations (AVMs). Uterine AVMs can be congenital or acquired. In recent years, there has been an increasing number of reports of acquired vascular lesions of the uterus following pregnancy, abortion, cesarean delivery, and curettage. It can be seen from these reports that there is confusion concerning the terminology of uterine vascular lesions. There is also a lack of diagnostic criteria and management guidelines, which has led to an increased number of unnecessary invasive procedures (eg, angiography, uterine artery embolization, hysterectomy for abnormal vaginal bleeding). This article familiarizes readers with various vascular lesions of the uterus and their management. PMID:24340126

  13. Advances in intracranial monitoring.

    PubMed

    Blount, Jeffrey P; Cormier, Jason; Kim, Hyunmi; Kankirawatana, Pongkiat; Riley, Kristen O; Knowlton, Robert C

    2008-09-01

    Intracranial monitoring using electroencephalography (IC-EEG) continues to play a critical role in the assessment of patients with medically intractable localization-related epilepsy. There has been minimal change in grid or electrode design in the last 15-20 years, and the surgical approaches for implantation are unchanged. Intracranial monitoring using EEG allows detailed definition of the region of ictal onset and defines the epileptogenic zone, particularly with regard to adjacent potentially eloquent tissue. Recent developments of IC-EEG include the coregistration of functional imaging data such as magnetoencephalography to the frameless navigation systems. Despite significant inherent limitations that are often overlooked, IC-EEG remains the gold standard for localization of the epileptogenic cortex. Intracranial electrodes take a variety of different forms and may be placed either in the subdural (subdural strips and grids, depth electrodes) or extradural spaces (sphenoidal, peg, and epidural electrodes). Each form has its own advantages and shortcomings but extensive subdural implantation of electrodes is most common and is most comprehensively discussed. The indications for intracranial electrodes are reviewed.

  14. Spontaneous intracranial hypotension.

    PubMed Central

    Renowden, S A; Gregory, R; Hyman, N; Hilton-Jones, D

    1995-01-01

    The clinical features and radiological appearances of spontaneous intracranial hypotension are described in three patients and the medical literature is reviewed. Awareness of this condition and its differentiation from more sinister meningitic processes is important to avoid unnecessary invasive investigations and to allow prompt diagnosis and effective treatment. Images PMID:8530936

  15. Arteriovenous Malformation Underlying a Plexiform Neurofibroma: An Unusual Presentation.

    PubMed

    Abbas, Zaheer; Khani, Sepideh; Zare, Javad

    2017-01-01

    Vascular abnormalities associated with neurofibromatosis type 1 are well described in the literature, however, arteriovenous malformation is a very rare finding in neurofibromatosis type 1. We report the case of an 11-year-old girl who presented with a soft mass on the right flank. Provisional diagnosis of plexiform neurofibroma was made on the basis of clinical and histological observations. Because the lesion was warm on palpation, imaging studies were performed to evaluate further and arteriovenous malformation was detected underlying the plexiform neurofibroma. This report emphasizes the importance of careful examination and proper investigations of the plexiform neurofibroma prior to treatment strategies to avoid future complications. The rarity of plexiform neurofibroma in association with arteriovenous malformation at the same site was also highlighted in this report.

  16. Arteriovenous Malformation Underlying a Plexiform Neurofibroma: An Unusual Presentation

    PubMed Central

    Abbas, Zaheer; Khani, Sepideh; Zare, Javad

    2017-01-01

    Vascular abnormalities associated with neurofibromatosis type 1 are well described in the literature, however, arteriovenous malformation is a very rare finding in neurofibromatosis type 1. We report the case of an 11-year-old girl who presented with a soft mass on the right flank. Provisional diagnosis of plexiform neurofibroma was made on the basis of clinical and histological observations. Because the lesion was warm on palpation, imaging studies were performed to evaluate further and arteriovenous malformation was detected underlying the plexiform neurofibroma. This report emphasizes the importance of careful examination and proper investigations of the plexiform neurofibroma prior to treatment strategies to avoid future complications. The rarity of plexiform neurofibroma in association with arteriovenous malformation at the same site was also highlighted in this report. PMID:28405554

  17. Chiari-like Malformation.

    PubMed

    Loughin, Catherine A

    2016-03-01

    Chiari-like malformation is a condition of the craniocervical junction in which there is a mismatch of the structures of the caudal cranial fossa causing the cerebellum to herniate into the foramen magnum. This herniation can lead to fluid buildup in the spinal cord, also known as syringomyelia. Pain is the most common clinical sign followed by scratching. Other neurologic signs noted are facial nerve deficits, seizures, vestibular syndrome, ataxia, menace deficit, proprioceptive deficits, head tremor, temporal muscle atrophy, and multifocal central nervous system signs. MRI is the diagnostic of choice, but computed tomography can also be used.

  18. Intracranial dural arteriovenous fistula as a cause for symptomatic superficial siderosis: A report of two cases and review of the literature

    PubMed Central

    Baum, Griffin R.; Turan, Nefize; Buonanno, Ferdinando S.; Pradilla, Gustavo; Nogueira, Raul G.

    2016-01-01

    Background: Superficial siderosis (SS) is the occult deposition of hemosiderin within the cerebral cortex due to repeat microhemorrhages within the central nervous system. The collection of hemosiderin within the pia and superficial cortical surface can lead to injury to the nervous tissue. The most common presentation is occult sensorineural hearing loss although many patients have been misdiagnosed with diseases such as multiple sclerosis and amyotrophic lateral sclerosis before being diagnosed with SS. Only one case report exists in the literature describing an intracranial dural arteriovenous fistula (dAVF) as the putative cause for SS. Case Description: We describe two cases of SS caused by a dAVF. Both patients had a supratentorial, cortical lesion supplied by the middle meningeal artery with venous drainage into the superior sagittal sinus. In both patients, symptoms improved after endovascular embolization. The similar anatomic relationship of both dAVFs reported presents an interesting question about the pathogenesis of SS. Similar to the pathologic changes seen in the formation of intracranial arterial aneurysms; it would be possible that changes in the blood vessel lining and wall might predispose a patient to chronic, microhemorrhage resulting in SS. Conclusions: We describe the second and third cases of a dAVF as the cause of SS, and the first cases of successful treatment of SS-associated dAVF with endovascular embolization. As noninvasive imaging techniques become more sensitive and easily obtained, one must consider their limitations in detecting occult intracranial vascular malformations such as dAVF as a possible etiology for SS. PMID:27127712

  19. The "seagull cry" in a patient with a cerebral arteriovenous malformation.

    PubMed

    Morelli, N; Rota, E; Michieletti, E; Guidetti, D

    2012-09-01

    The "seagull cry" is an acoustic phenomenon heard during duplex ultrasound. It is caused by harmonic covibrations of a vessel wall in the presence of high-velocity blood flow. It has been reported in a few cases of cerebrovascular disease, such as severe intracranial stenosis, vasospasm or carotid-cavernous fistula. A 35-year-old man underwent transcranial color-coded sonography (TCCS) for work-up of a severe new-onset headache. Doppler spectral analysis of the right intracranial carotid bifurcation revealed multiple pairs of mirror-image parallel strings, and a high-frequency seagull cry was heard. Computed tomography-angiography and magnetic resonance imaging of the brain showed an arteriovenous malformation in the right temporal lobe. The seagull cry is a "musical murmur" with single or multiple frequency that sounds like a musical tone. This is the first report of this phenomenon in a cerebral arteriovenous malformation.

  20. Thromboembolism and congenital malformations: from Duane syndrome to thalidomide embryopathy.

    PubMed

    Parsa, Cameron F; Robert, Matthieu P

    2013-04-01

    To propose a pathophysiologic mechanism to unify a variety of disparate sporadic congenital malformations. Inductive and deductive analyses to correlate malformation laterality with asymmetries in thoracic anatomy, critical analysis of malformations with female predominance, and concepts of hydrodynamic pressure gradients in vascular growth were applied to the ensuing development of guiding tissue scaffolds for cellular proliferation, differentiation, and apoptosis. Duane syndrome may develop following a focal vascular insult to the sixth nerve trunk with axonal degeneration, allowing for substitutive innervation from third nerve axons to the lateral rectus muscle. Causative fibrin clots may originate from the venous system and paradoxically migrate through physiological right-to left shunts, or they may arise directly from the heart. Hence, the unilateral, left-sided, and female predominance of Duane syndrome results from the asymmetry in the thoracic anatomy and from thrombosis risk factors. Embolic occlusions may also alter local hemodynamic pressure gradients, leading to the compensatory enlargement and persistence of the fetal vasculature and may dysregulate tissue growth. Within the eye, this results in forms of Peters anomaly, unilateral congenital cataracts, and the morning glory disc anomaly, all in the vascular territory of the carotid arteries that also share a propensity for left-sided involvement in girls. Most aberrant misinnervation phenomena (eg, jaw-winking syndrome, crocodile tear syndrome, Brown syndrome, and congenital fibrosis syndrome) and, by extrapolation, the hypoplasia or dysgenesis of noncephalic anatomical structures (including limbs) may be similarly explained. Such malformations will occur more frequently under thrombogenic conditions, such as those induced by thalidomide. Fibrin emboli and focal hypoperfusion may explain the development of many sporadic congenital malformations.

  1. Seizure risk from cavernous or arteriovenous malformations

    PubMed Central

    Josephson, C.B.; Leach, J.-P.; Duncan, R.; Roberts, R.C.; Counsell, C.E.

    2011-01-01

    Objectives: To determine the risk of epileptic seizures due to a brain arteriovenous malformation (AVM) or cavernous malformation (CM). Methods: In a prospective population-based study of new diagnoses of AVMs (n = 229) or CMs (n = 139) in adults in Scotland in 1999–2003, we used annual medical records surveillance, general practitioner follow-up, and patient questionnaires to quantify the risk of seizures between clinical presentation and AVM/CM treatment, last follow-up, or death. Results: The 5-year risk of first-ever seizure after presentation was higher for AVMs presenting with intracranial hemorrhage or focal neurologic deficit (ICH/FND: n = 119; 23%, 95% confidence interval [CI] 9%–37%) than for incidental AVMs (n = 40; 8%, 95% CI 0%–20%), CMs presenting with ICH/FND (n = 38; 6%, 95% CI 0%–14%), or incidental CMs (n = 57; 4%, 95% CI 0%–10%). For adults who had never experienced ICH/FND, the 5-year risk of epilepsy after first-ever seizure was higher for CMs (n = 23; 94%, 95% CI 84%–100%) than AVMs (n = 37; 58%, 95% CI 40%–76%; p = 0.02). Among adults who never experienced ICH/FND and presented with or developed epilepsy, there was no difference in the proportions achieving 2-year seizure freedom over 5 years between AVMs (n = 43; 45%, 95% CI 20%–70%) and CMs (n = 35; 47%, 95% CI 27%–67%). Conclusions: AVM-related ICH confers a significantly higher risk of a first-ever seizure compared to CMs or incidental AVMs. Adults with a CM have a high risk of epilepsy after a first-ever seizure but achieve seizure freedom as frequently as those with epilepsy due to an AVM. PMID:21536634

  2. Venous malformation and haemangioma: differential diagnosis, diagnosis, natural history and consequences.

    PubMed

    Lee, B B

    2013-03-01

    Venous malformation (VM) is the most common form of congenital vascular malformation (CVM). VM presents at birth as an inborn vascular defect and never disappears/regresses spontaneously through the rest of life; it will continue to grow slowly at a rate that is proportional to the growth rate of the body. Haemangioma is not a vascular malformation but one of the vascular tumours originating from the endothelial cells; it develops after birth mostly in the infantile/neonatal period with a distinctive growth cycle: a proliferation phase of early rapid growth followed by an involutional phase of slow regression. Although the vascular malformation and vascular tumour belong to the 'vascular anomaly' together, both conditions are fundamentally different not only in their anatomical, histological and pathophysiological findings but also in their clinical courses. Therefore, an appropriate differential diagnosis of the VM is mandated not only from other kinds of CVMs but also from 'genuine' haemangioma. Appropriate diagnosis and assessment of VMs can be made based on clinical presentation and a proper combination of basic non-invasive studies in general but the presence of a mixed lesion involving other types of CVM lesions and the type of VM lesion, extratruncular and truncular, will dictate the need for further work-up with additional non- to less-invasive study or angiography. Otherwise, angiography is usually reserved for therapeutic planning and treatment.

  3. Intraoperative Angiography for Arteriovenous Malformation Resection in the Prone and Lateral Positions, Using Upper Extremity Arterial Access.

    PubMed

    Nossek, Erez; Chalif, David J; Buciuc, Razvan; Gandras, Eric J; Anderer, Erich G; Insigna, Sal; Dehdashti, Amir R; Setton, Avi

    2017-06-01

    Intraoperative angiography is routinely utilized for aneurysms and arteriovenous malformations (AVMs) to verify complete occlusion and resection. Surgery for spinal and posterior fossa neurovascular lesions is usually performed in prone position. Intraoperative angiography in the prone position is challenging and there is no standardized protocol for this procedure. To describe our experience with intraoperative angiography in the prone and lateral positions, using upper extremity arterial access. We reviewed our experience with intraoperative angiography in the prone position between 2014 and 2015, where vascular access was obtained via the upper extremity arteries. Patients were treated in a hybrid endovascular operating room. High cervical and intracranial lesions were studied via brachial or radial access. All accesses were obtained using ultrasonographic guidance and a small caliber arterial sheath (4F). Five patients were treated in the prone and lateral positions using brachial/radial artery access. Patients harbored cerebellar AVM, lateral medullary AVM, cervical arteriovenous fistula (AVF), tentorial dural AVF, and tentorial-incisural dural AVF. Patients were positioned prone (n = 2), semiprone (n = 2), and lateral (n = 1) for the surgery. Three patients were treated via right brachial artery access. Two patients were treated via radial arteries access. All patients tolerated the procedures without technical or clinical complications. Intraoperative angiography verified complete occlusion and resection in all cases prior to surgical closure. Intraoperative angiography in the prone and lateral positions using upper extremity access is an important adjunct. Brachial or radial access can be obtained safely and provides comfortable and quick approaches.

  4. Retinal Vascular Changes are a Marker for Cerebral Vascular Diseases.

    PubMed

    Moss, Heather E

    2015-07-01

    The retinal circulation is a potential marker of cerebral vascular disease because it shares origin and drainage with the intracranial circulation and because it can be directly visualized using ophthalmoscopy. Cross-sectional and cohort studies have demonstrated associations between chronic retinal and cerebral vascular disease, acute retinal and cerebral vascular disease, and chronic retinal vascular disease and acute cerebral vascular disease. In particular, certain qualitative features of retinopathy, retinal artery occlusion, and increased retinal vein caliber are associated with concurrent and future cerebrovascular events. These associations persist after accounting for confounding variables known to be disease-causing in both circulations, which supports the potential use of retinal vasculature findings to stratify individuals with regards to cerebral vascular disease risk.

  5. A familial venous malformation locus is on chromosome 9p

    SciTech Connect

    Boon, L.M.; Mulliken, J.B.; Vikkula, M.

    1994-09-01

    Venous malformation is the most common vascular malformation affecting 0.2% of the population. Depending upon size and location, these slow-flow lesions may cause pain, anatomic distortion and threaten life. Most venous malformations occur sporadically and present as solitary lesions. For this reason, determining their pathogenic bases has proven elusive. However, venous malformations also occur in several rare syndromes, some of which demonstrate Mendelian inheritance. As a first step towards identifying the pathogenic bases for these lesions, we have mapped a locus for an autosomal dominant disorder in a three generation family that manifests as multiple cutaneous and mucosal venous malformations. This locus lies within a 24.5 cM interval on chromosome 9p, defined by the markers D9S157 and D9S163. A maximum LOD score of 4.11 at {theta} = 0.05 is obtained with several markers within the interval. The interferon gene cluster, which has previously been implicated in angiogenesis, and the multiple tumor suppressor gene, responsible for several types of malignant tumors, also lie within this interval and are potential candidates.

  6. Sporadic Multifocal Venous Malformations of the Head and Neck

    PubMed Central

    Amato, Michael V.; Patel, Neha A.; Hu, Shirley; Pantelides, Harry

    2015-01-01

    Objective. To report a case of unusually widespread sporadic venous malformations of the head and neck associated with normal D-dimer levels and, due to the protean clinical manifestations and increased risk of coagulopathy of these lesions, to review their diagnosis and clinical management. Case Report. A 25-year-old man presented with a one-year history of intermittent right-sided neck swelling and tongue swelling. Physical exam revealed additional lesions present throughout the head and neck. There was no family history suggestive of heritable vascular malformations. Radiographic imaging demonstrated 15 lesions located in various tissue layers consistent with venous malformations. A coagulation screen showed a normal prothrombin time, activated partial thromboplastin time, international normalized ratio, D-dimer level, and fibrinogen level. It was determined that the patient was not at increased risk for intraoperative coagulopathy and preoperative heparin administration would not be necessary. The patient's buccal and tongue lesions were subsequently excised with no complications. The patient also underwent sclerotherapy evaluation for his neck mass. Conclusion. This case describes a unique presentation of sporadic multifocal venous malformations. It also emphasizes the importance of prompt diagnosis and workup when multiple venous malformations are present to prevent morbidity during surgical excision secondary to intravascular coagulopathy. PMID:26483982

  7. Local Model of Arteriovenous Malformation of the Human Brain

    NASA Astrophysics Data System (ADS)

    Nadezhda Telegina, Ms; Aleksandr Chupakhin, Mr; Aleksandr Cherevko, Mr

    2013-02-01

    Vascular diseases of the human brain are one of the reasons of deaths and people's incapacitation not only in Russia, but also in the world. The danger of an arteriovenous malformation (AVM) is in premature rupture of pathological vessels of an AVM which may cause haemorrhage. Long-term prognosis without surgical treatment is unfavorable. The reduced impact method of AVM treatment is embolization of a malformation which often results in complete obliteration of an AVM. Pre-surgical mathematical modeling of an arteriovenous malformation can help surgeons with an optimal sequence of the operation. During investigations, the simple mathematical model of arteriovenous malformation is developed and calculated, and stationary and non-stationary processes of its embolization are considered. Various sequences of embolization of a malformation are also considered. Calculations were done with approximate steady flow on the basis of balanced equations derived from conservation laws. Depending on pressure difference, a fistula-type AVM should be embolized at first, and then small racemose AVMs are embolized. Obtained results are in good correspondence with neurosurgical AVM practice.

  8. A Review of Vascular Anomalies: Genetics and Common Syndromes

    PubMed Central

    Killion, Elizabeth; Mohan, Kriti; Lee, Edward I.

    2014-01-01

    Vascular tumors and malformations are unique in that affected cells exhibit disrupted angiogenesis. The current treatment options often yield suboptimal results. New insight into the genetics and molecular basis of vascular anomalies may pave the way for potential development of targeted therapy. The authors review the genetic and molecular basis of vascular anomalies and common associated syndromes. PMID:25045331

  9. Atypical MRI features in soft-tissue arteriovenous malformation: a novel imaging appearance with radiologic-pathologic correlation.

    PubMed

    Patel, Anand S; Schulman, Joshua M; Ruben, Beth S; Hoffman, William Y; Dowd, Christopher F; Frieden, Ilona J; Hess, Christopher P

    2015-09-01

    The absence of a discrete mass, surrounding signal abnormality and solid enhancement are imaging features that have traditionally been used to differentiate soft-tissue arteriovenous malformations from vascular tumors on MRI. We have observed that these findings are not uncommon in arteriovenous malformations, which may lead to misdiagnosis or inappropriate treatment. To estimate the frequency of atypical MRI features in soft-tissue arteriovenous malformations and assess their relationship to lesion size, location, tissue type involved and vascular architecture. Medical records, MRI and histopathology were reviewed in consecutive patients with soft-tissue arteriovenous malformations in a multidisciplinary vascular anomalies clinic. Arteriovenous malformations were divided into those with and without atypical MRI findings (perilesional T2 signal abnormality, enhancement and/or a soft-tissue mass). Lesion location, size, tissue involved and vascular architecture were also compared between groups. Tissue stains were reviewed in available biopsy or resection specimens to assess relationships between MRI findings and histopathology. Thirty patients with treatment-naïve arteriovenous malformations were included. Fifteen lesions demonstrated atypical MRI. There was no difference in age, gender, lesion size or involved body part between the groups. However, more than half of the atypical lesions demonstrated multicompartmental involvement, and tiny intralesional flow voids were more common in atypical arteriovenous malformations. Histopathology also differed in atypical cases, showing densely packed endothelial cells with connective tissue architectural distortion and edema. Arteriovenous malformations may exhibit features of a vascular tumor on MRI, particularly when multicompartmental and/or containing tiny internal vessels. These features are important to consider in suspected fast-flow vascular malformations and may have implications with respect to their treatment.

  10. Arteriovenous malformation embocure score: AVMES.

    PubMed

    Lopes, Demetrius K; Moftakhar, Roham; Straus, David; Munich, Stephan A; Chaus, Fahad; Kaszuba, Megan C

    2016-07-01

    Cerebral arteriovenous malformations (CAVMs) may be treated with microsurgery, radiosurgery, endovascular surgery, or a combination of these modalities. Grading scales are available to aid the assessment of curative risk for microsurgery and radiosurgery. No grading system has been developed to assess the curative risk of endovascular surgery. To report our retrospective application of the AVM embocure score to patients treated at our institution between 2005 and 2011 METHODS: We performed a retrospective review of 39 patients with CAVM treated at our institution between 2005 and 2011 with the primary aim of achieving a curative embolization. After reviewing all the different variables associated with the conventional Onyx embolization technique for CAVMs, we identified the following as the most relevant characteristics influencing the chances for complete angiographic embolization and complication risk: the number of arterial pedicles and draining veins, size of AVM nidus, and vascular eloquence. We sought to develop a scoring system to assess the complication risk for a curative embolization of CAVM with liquid embolic Onyx (Covidien, Irvine, California, USA). We developed the AVM embocure score (AVMES). This scoring system ranges from 3 to 10 and is the arithmetic sum of the number of arterial pedicles feeding the AVM (≤3, 4-6, >6), the number of draining veins (≤3, 4-6, >6), the size of the AVM nidus in centimeters (≤3, 4-6, >6), and the vascular eloquence (0-1). We applied AVMES to the same cohort of patients and validated the predictability of complete angiographic embolization and expected clinical risk of complication. In lesions with an AVMES of 3 (n=8), there was a 100% rate of complete AVM obliteration and 0% rate of major complications. In AVMES 4 (n=12) lesions, there was 75% complete obliteration rate, with 8% major morbidity. In AVMES 5 (n=9) lesions, there was 78% complete obliteration and 11% major morbidity. In AVMES >5 (n=10) there was 20

  11. Early experience on peripheral vascular application of the vascular plugs

    PubMed Central

    Rohit, Manoj Kumar; Sinha, Alok Kumar; Kamana, Naveen Krishna

    2013-01-01

    Background Transcatheter closure of various congenital and acquired vascular malformations with Amplatzer Vascular plugs I and II has been established. Here we present our experience with device closure. Materials and methods Between October 2006 and August 2012, nine (three males and six females) patients aged between 11 months and 62 years (mean age 19 years) underwent percutaneous device closure with AVP I and II vascular plugs for congenital and acquired arteriovenous malformation and cardiac diverticulum are presented here. Results One case of coronary cameral fistula, four cases of pulmonary arteriovenous fistula, one case of large major aortopulmonary collaterals (in tetralogy of Fallot closed before intracardiac repair), one case of congenital cardiac diverticulum, one case of fistula between external carotid artery and internal jugular vein and one case of iatrogenic carotid jugular fistula were successfully closed with AVP I and II plugs. Overall in nine cases, 16 AVP I and II plugs were deployed to occlude feeding vessels and one cardiac diverticulum. The technical success rate was 100%. No major complications were observed. Conclusion Amplatzer vascular plugs can be used successfully for closure of various congenital and acquired vascular malformations with good result. PMID:24206877

  12. Transverse Sinus Stenosis in Adult Patients With Chiari Malformation Type I

    PubMed Central

    Saindane, Amit M.; Bruce, Beau B.; Desai, Nilesh K.; Roller, Lauren A.; Newman, Nancy J.; Biousse, Valérie

    2014-01-01

    OBJECTIVE Transverse sinus stenosis is an imaging finding very highly associated with elevated intracranial pressure (ICP). Patients with the Chiari I malformation may potentially have elevated ICP due to impairment of CSF flow at the foramen magnum. This study evaluated whether patients with Chiari I malformation have transverse sinus stenosis and other imaging findings indicative of elevated ICP. MATERIALS AND METHODS Thirty patients with Chiari I malformation treated surgically and 76 control subjects were identified retrospectively. All control subjects and all patients with Chiari I malformation (preoperatively) underwent standardized contrast-enhanced brain MRI including a contrast-enhanced 3D T1-weighted sequence from which curved reformats of the transverse sinuses were generated. Two different readers blinded to the diagnosis then independently evaluated these curved reformats for severity of transverse sinus stenosis. Orbital and skull-base findings previously described in association with elevated ICP were also evaluated. Frequency of MRI findings between the two groups was compared. RESULTS Patients with Chiari I malformation had significantly greater frequency of unilateral or bilateral transverse sinus stenosis than did control subjects (p < 0.001). There was complete interreader agreement on presence or absence of transverse sinus stenosis by patient (κ = 1.0 [95% CI, 0.89–1.0]). Logistic regression analysis controlling for age, sex, and body mass index found that transverse sinus stenosis significantly predicted Chiari I malformation versus control status (odds ratio, 11.2 [95% CI, 2.1–59.0]; p = 0.004) but that no other features were significantly associated with the Chiari I malformation. Patients with Chiari I malformation who had transverse sinus stenosis had significantly greater pituitary flattening than did those without transverse sinus stenosis (p = 0.02). CONCLUSION Patients with Chiari I malformation have higher likelihood of trans

  13. [Vascular "tumors" and the rules of their surgical management].

    PubMed

    Enjolras, O; Deffrennes, D; Borsik, M; Diner, P; Laurian, C

    1998-08-01

    After defining vascular tumors and malformations, formerly called angiomas, the authors adopt the classification of the International Society for the Study of vascular Anomalies. The various groups of malformations, after definition of the subject and the problems raised, are illustrated by a number of clinical cases and the rules of surgical management are discussed. Capillary malformations, formerly called portwine stains, can be treated by dye laser, but sometimes tissue and orthognathic reconstructive surgery in the presence of a skeletal malformation. Lymphatic malformations (lymphangiomas), optimally assessed by CT or MRI, can be treated by a variety of approaches: sclerotherapy, surgery. Venous malformations raise very different problems in the cervicocephalic region and on the limbs and trunk, but always require a multidisciplinary approach with, according to the site, size and repercussions, Ethibloc percutaneous sclerotherapy, embolization, surgery. Arteriovenous malformations require complete surgical treatment, usually preceded by embolization; reconstruction consists of local flaps or skin expansion in simple cases, and revascularized free flaps in difficult cases. If the malformation is cosmetically and functionally acceptable, the authors propose conservative management. The first-line treatment of haemangiomas is pharmacological (corticosteroids, interferon), but surgery may be indicated in two situations: early and late. There is a renewed interest in early surgery, subtended by several factors including cosmetic concerns and the development of new technologies, including the Cavitron. Late surgery retains its classical cosmetic and functional indications. Two key-words dominate the rules of therapeutic management of all types of vascular malformations: a multidisciplinary approach and modesty.

  14. Intracranial Trigeminal Schwannoma

    PubMed Central

    2015-01-01

    Intracranial trigeminal schwannomas are rare tumors. Patients usually present with symptoms of trigeminal nerve dysfunction, the most common symptom being facial pain. MRI is the imaging modality of choice and is usually diagnostic in the appropriate clinical setting. The thin T2-weighted CISS 3D axial sequence is important for proper assessment of the cisternal segment of the nerve. They are usually hypointense on T1, hyperintense on T2 with avid enhancement post gadolinium. CT scan is supplementary to MRI, particularly for tumors located in the skull base. Imaging plays a role in diagnosis and surgical planning. In this pictorial essay, we retrospectively reviewed imaging findings in nine patients with pathologically proven trigeminal schwannoma. Familiarity with the imaging findings of intracranial trigeminal schwannoma may help to diagnose this entity. PMID:25924170

  15. A rare case of pulmonary arterio-venous malformation with recurrent anemia: Hereditary hemorrhagic telangiectasia

    PubMed Central

    Nataraju, Kamalesh Tagadur; Mukherjee, Tirthankar; Doddaiah, Ramachandra Prabhu Hosahalli; Nanjappa, Nagesh Gabbadi; Narasegowda, Lakshmikanth

    2015-01-01

    Arteriovenous malformation (AVM) is a rare vascular anomaly of the lung, which manifests predominantly as dyspnea (due to right to left shunting) and paradoxical embolism. Hereditary Hemorrhagic Telangiectasia (HHT) being a rare genetic disorder is one of the most common causes of pulmonary arteriovenous malformation (PAVM). Here we report an interesting case of recurrent anemia in an elderly female, who was subsequently found to have multiple cutaneous and mucosal telangiectasias and a large pulmonary AVM. PMID:26180392

  16. [Diagnostic imaging of peripheral renal vascular disorders].

    PubMed

    Hélénon, O; Correas, J M; Eiss, D; Khairoune, A; Merran, S

    2004-02-01

    Peripheral vascular disorders of the kidney involve the intrarenal branches of the renal vascular tree. It include occlusive (infarction and cortical necrosis) and non-occlusive vascular lesions (acquired arteriovenous fistulas, arteriovenous malformation, false aneurysms and microaneurysms). Initial diagnosis relies on color Doppler US and CT angiography. Angiography plays a therapeutic role. MR imaging provides useful diagnostic information on perfusion disorders especially in patients with renal insufficiency.

  17. Management of venous malformations.

    PubMed

    Richter, Gresham T; Braswell, Leah

    2012-12-01

    Venous malformations (VMs) frequently occur in the head and neck with a predilection for the parotid gland, submandibular triangle, buccal space, muscles of mastication, lips, and upper aerodigestive tract. They are composed of congenitally disrupted ectatic veins with inappropriate connections and tubular channels. Because VMs have poorly defined boundaries and a tendency to infiltrate normal tissue, they require calculated treatment decisions in the effort to preserve surrounding architecture. Sclerotherapy, surgical excision, neodymium:yttrium aluminum garnet laser therapy, or a combination of these modalities is employed in the management of VMs. Although many small VMs can be cured, the objective is often to control the disease with periodic therapy. Location, size, and proximity to vital structures dictate the type of therapy chosen. Vigilance with long-term follow up is important. This review outlines current diagnostic and therapeutic approaches to simple and extensive cervicofacial VMs. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  18. Intracranial pial arteriovenous fistulas.

    PubMed

    Lee, Ji Yeoun; Son, Young-Je; Kim, Jeong Eun

    2008-08-01

    Intracranial pial arteriovenous fistula (AVF) is a rare cerebrovascular lesion that has only recently been recognized as a distinct pathological entity. A 41-year-old woman (Patient 1) presented with the sudden development of an altered mental state. Brain CT showed an acute subdural hematoma. A red sylvian vein was found intraoperatively. A pial AVF was revealed on postoperative angiography, and surgical disconnection of the AVF was performed. A 10-year-old boy (Patient 2) presented with a 10-day history of paraparesis and urinary incontinence. Brain, spinal MRI and angiography revealed an intracranial pial AVF and a spinal perimedullary AVF. Endovascular embolization was performed for both lesions. The AVFs were completely obliterated in both patients. On follow-up, patient 1 reported having no difficulty in performing activities of daily living. Patient 2 is currently able to walk without assistance and voids into a diaper. Intracranial pial AVF is a rare disease entity that can be treated with surgical disconnection or endovascular embolization. It is important for the appropriate treatment strategy to be selected on the basis of patientspecific and lesion-specific factors in order to achieve good outcomes.

  19. Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus.

    PubMed Central

    Obrador, S; Soto, M; Silvela, J

    1975-01-01

    Arteriovenous malformations or fistulae shunting arterial blood from branches of the external and internal carotid and vertebral arteries into the transverse-sigmoid sinus may produce different clinical syndromes. The literature is reviewed with 96 patients including six personal cases. Usually these malformations have a congenital origin and only in 4% of the series was there a previous history of a severe head injury. Clinical groups are defined and the role of angiography assessed. Direct surgical approach with occlusion or removal of the vascular malformation is the treatment of choice. Possible methods of treatment by selective embolization are discussed. Images PMID:1097602

  20. A case of prenatally diagnosed extrapulmonary arteriovenous malformation associated with a complex heart defect

    PubMed Central

    Jeong, Ba-Da; An, Suah; Kim, Ji Yeon; Lee, Mi-Young; Kim, Eun Na; Kim, Jung-Sun; Kim, Chong Jai

    2016-01-01

    Pulmonary arteriovenous malformations are rare vascular anomalies of the lung, only a few cases of which have been diagnosed prenatally. The diagnostic clue for prenatal diagnosis was cardiomegaly with a particularly enlarged left atrium. All previous cases of pulmonary arteriovenous malformations diagnosed prenatally have been reported as an isolated anomaly or in association with simple heart defects. We here describe the first case of a pulmonary arteriovenous malformation with a complex heart defect that was diagnosed prenatally at 21.0 weeks of gestation and confirmed by postmortem autopsy. PMID:27896260

  1. PHACE Syndrome: Persistent Fetal Vascular Anomalies

    PubMed Central

    Prochazka, V.; Hrbac, T.; Chmelova, J.; Skoloudik, D.; Prochazka, M.

    2005-01-01

    Summary PHACE(S) syndrome is an acronym for neurocutaneous disease encompassing the expression of (P) posterior cranial fossa malformations, (H) facial haemangiomas, (A) arterial anomalies, (C) aortic coarctaion and other cardiac defects, (E) eye abnormalities and (S) for sternal malformation or stenotic arterial diseases. We report on a case of PHACE syndrome complete expression with persistent fetal vascular anomalies unusually in a 55-year-old women with large bilateral facial and neck haemangioma and posterior fossa circulation insufficiency. PMID:20584448

  2. Intracranial chondroma: a rare entity.

    PubMed

    Maheshwari, Veena; Mehdi, Ghazala; Varshney, Manoranjan; Jain, Anshu; Vashishtha, Sonal; Gaur, Kavita; Srivastava, Vinod Kumar

    2011-05-12

    Intracranial chondroma is a rare benign cartilaginous tumour with an incidence of less than 1% of all primary intracranial tumours. The authors are reporting here a case of intracranial chondroma in a 40-year-old man who presented with 5-month history of headache and gradual diminution of vision. A tentative diagnosis of chondroma was made on imprint cytology which was confirmed on histopathological examination.

  3. Early Support of Intracranial Perfusion

    DTIC Science & Technology

    2012-10-01

    associated with intractable intracranial hypertension and cerebral hypoperfusion At the close of Year 3  Recruitment of targeted 50 subjects...Determination of serum and CSF biomarkers that predict worsening of cerebral hypoperfusion, intracranial hypertension , and cerebral hypoxia. At the close of Year...rate variation is associated with intractable intracranial hypertension and cerebral hypoperfusion. Kahraman S, Dutton R, Hu P, Stansbury L, Xiao Y

  4. Cerebral cavernous malformations: from CCM genes to endothelial cell homeostasis.

    PubMed

    Fischer, Andreas; Zalvide, Juan; Faurobert, Eva; Albiges-Rizo, Corinne; Tournier-Lasserve, Elisabeth

    2013-05-01

    Cerebral cavernous malformations (CCMs) are vascular lesions that can occur sporadically or as a consequence of inherited loss-of-function mutations, predominantly in the genes CCM1 (KRIT1), CCM2 (MGC4607, OSM, Malcavernin), or CCM3 (PDCD10, TFAR15). Inherited, familial CCM is characterized by the development of multiple lesions throughout a patient's life leading to recurrent cerebral hemorrhages. Recently, roles for the CCM proteins in maintaining vascular barrier functions and quiescence have been elucidated, and in this review we summarize the genetics and pathophysiology of this disease and discuss the molecular mechanisms through which CCM proteins may act within blood vessels.

  5. Mouse models of intracranial aneurysm.

    PubMed

    Wang, Yutang; Emeto, Theophilus I; Lee, James; Marshman, Laurence; Moran, Corey; Seto, Sai-wang; Golledge, Jonathan

    2015-05-01

    Subarachnoid hemorrhage secondary to rupture of an intracranial aneurysm is a highly lethal medical condition. Current management strategies for unruptured intracranial aneurysms involve radiological surveillance and neurosurgical or endovascular interventions. There is no pharmacological treatment available to decrease the risk of aneurysm rupture and subsequent subarachnoid hemorrhage. There is growing interest in the pathogenesis of intracranial aneurysm focused on the development of drug therapies to decrease the incidence of aneurysm rupture. The study of rodent models of intracranial aneurysms has the potential to improve our understanding of intracranial aneurysm development and progression. This review summarizes current mouse models of intact and ruptured intracranial aneurysms and discusses the relevance of these models to human intracranial aneurysms. The article also reviews the importance of these models in investigating the molecular mechanisms involved in the disease. Finally, potential pharmaceutical targets for intracranial aneurysm suggested by previous studies are discussed. Examples of potential drug targets include matrix metalloproteinases, stromal cell-derived factor-1, tumor necrosis factor-α, the renin-angiotensin system and the β-estrogen receptor. An agreed clear, precise and reproducible definition of what constitutes an aneurysm in the models would assist in their use to better understand the pathology of intracranial aneurysm and applying findings to patients.

  6. Unusual association of Arnold-Chiari malformation and vitamin B12 deficiency.

    PubMed

    Verma, Rajesh; Praharaj, Heramba Narayan

    2012-07-09

    Arnold-Chiari malformations are a group of congenital or acquired defects associated with the displacement of cerebellar tonsils into the spinal canal. First described by Chiari (1891), this has various grades of severity and involves various parts of neuraxis, for example, cerebellum and its outputs, neuro-otological system, lower cranial nerves, spinal sensory and motor pathways. The symptomatology of Arnold-Chiari malformations may mimic multiple sclerosis, primary headache syndromes, spinal tumours and benign intracranial hypertension. We highlighted a case of Chiari type I malformation, who presented with posterolateral ataxia associated with significant vitamin B(12) deficiency. The patient was supplemented with vitamin B(12) injections and showed remarkable improvement at follow-up after 3 months.

  7. Molecular diagnosis in cerebral cavernous malformations.

    PubMed

    Mondejar, R; Lucas, M

    2017-10-01

    Cerebral cavernous malformations (CCMs; OMIM 116860) are enlarged vascular cavities without intervening brain parenchyma whose estimated prevalence in the general population is between 0.1% and 0.5%. Familial CCM is an autosomal dominant disease with incomplete clinical and radiological penetrance. Three genes have been linked to development of the lesions: CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10. The aetiological mutation is not detected in a large percentage of cases and new approaches are therefore needed. The aim of this review is to analyse current molecular techniques and the possible mutations or variations which can be detected in a molecular genetics or molecular biology laboratory. Likewise, we will analyse other alternatives that may help detect mutations in those patients showing negative results. A molecular diagnosis of cerebral cavernous malformations should provide at least the copy number variation and sequencing of CCM genes. In addition, appropriate genetic counselling is a crucial source of information and support for patients and their relatives. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  8. Critical roles of macrophages in the formation of intracranial aneurysm

    PubMed Central

    Kanematsu, Yasuhisa; Kanematsu, Miyuki; Kurihara, Chie; Tada, Yoshiteru; Tsou, Tsung-Ling; van Rooijen, Nico; Lawton, Michael T.; Young, William L.; Liang, Elena I.; Nuki, Yoshitsugu; Hashimoto, Tomoki

    2011-01-01

    Background and Purpose Abnormal vascular remodeling triggered by hemodynamic stresses and inflammation is believed to be a key process in the pathophysiology of intracranial aneurysms. Numerous studies have shown infiltration of inflammatory cells, especially macrophages, into intracranial aneurysmal walls in humans. Using a mouse model of intracranial aneurysms, we tested whether macrophages play critical roles in the formation of intracranial aneurysms. Methods Intracranial aneurysms were induced in adult male mice using a combination of a single injection of elastase into the cerebrospinal fluid and angiotensin-II-induced hypertension. Aneurysm formation was assessed three weeks later. Roles of macrophages were assessed utilizing clodronate liposome-induced macrophage depletion. In addition, the incidence of aneurysms was assessed in mice lacking monocyte chemotactic protein-1 (MCP-1, CCL2), and mice lacking matrix metalloproteinase-12 (MMP-12, macrophage elastase). Results Intracranial aneurysms in this model showed leukocyte infiltration into the aneurysmal wall, the majority of leukocytes being macrophages. Mice with macrophage depletion had a significantly reduced incidence of aneurysms compared to control mice (1/10 vs. 6/10; P < 0.05). Similarly, there was a reduced incidence of aneurysms in mice lacking MCP-1, compared to incidence of aneurysms in wild-type mice (2/10 vs. 14/20, P < 0.05). There was no difference in the incidence of aneurysms between mice lacking MMP-12 and wild-type mice. Conclusions These data suggest critical roles of macrophages and proper macrophage functions in the formation of intracranial aneurysms in this model. PMID:21106959

  9. Rebound intracranial hypertension: a complication of epidural blood patching for intracranial hypotension.

    PubMed

    Kranz, P G; Amrhein, T J; Gray, L

    2014-06-01

    Rebound intracranial hypertension is a complication of epidural blood patching for treatment of intracranial hypotension characterized by increased intracranial pressure, resulting in potentially severe headache, nausea, and vomiting. Because the symptoms of rebound intracranial hypertension may bear some similarity to those of intracranial hypotension and literature reports of rebound intracranial hypertension are limited, it may be mistaken for refractory intracranial hypotension, leading to inappropriate management. This clinical report of 9 patients with confirmed rebound intracranial hypertension reviews the clinical characteristics of patients with this condition, emphasizing factors that can be helpful in discriminating rebound intracranial hypertension from refractory spontaneous intracranial hypotension, and discusses treatment.

  10. Lymphatic malformations: current cellular and clinical investigations.

    PubMed

    Perkins, Jonathan A; Manning, Scott C; Tempero, Richard M; Cunningham, Michael J; Edmonds, Joseph L; Hoffer, Fredric A; Egbert, Mark A

    2010-06-01

    Summarize current knowledge of lymphatic malformation development, biology, and clinical outcome measures. Panel presentation of lymphatic malformation biology and measurement of head and neck malformation treatment outcomes. Characterization of lymphatic malformation endothelial and stromal cells may lead to biologically based treatment. Traditionally, lymphatic malformation treatment outcomes have been measured according to reduction of malformation size. Currently, methods to measure functional outcomes following lymphatic malformation treatment are lacking. This is particularly apparent when the malformation directly involves the upper aerodigestive tract. The etiology and pathogenesis of head and neck lymphatic malformations are poorly understood, but understanding is improving through ongoing investigation. Reduction of lymphatic malformation size is generally possible, but further work is necessary to optimize methods for measuring therapeutic outcomes in problematic areas. Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

  11. Embolization of uterine arteriovenous malformation

    PubMed Central

    Chen, Yan; Wang, Guoyun; Xie, Fubo; Wang, Bo; Tao, Guowei; Kong, Beihua

    2013-01-01

    Background: Uterine arteriovenous malformation is a rare but potential life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can be lead to massive hemorrhage. Case: We describe here a case of uterine arteriovenous malformation. A 32-year-old woman presented abnormal vaginal bleeding following the induced abortion. A diagnosis of uterine arteriovenous malformation made on the basis of Doppler ultrasonraphy was confirmed through pelvic angiography. The embolization of bilateral uterine arteries was performed successfully. Conclusion: Uterine arteriovenous malformation should be suspected in patient with abnormal vaginal bleeding, especially who had the past medical history incluing cesarean section, induced abortion, or Dillation and Curethage and so on. Although angiography remains the gold standard, Doppler ultrasonography is also a good noninvasive technique. The transcatheter uterine artery embolization offers a safe and effective treatment PMID:24639742

  12. [Diagnostics of genetic malformations in small ruminants].

    PubMed

    Ganter, M

    2013-01-01

    The epidemic occurrence of the Schmallenberg virus has induced numerous congenital malformations in small ruminants. Because of this high incidence of malformed lambs, an overview of the different causes of congenital malformations is provided. The most frequent infectious and physical causes as well as mineral and vitamin deficiencies and toxic agents which can induce congenital malformations are indicated. This list is supplemented by advice on sampling and laboratory diagnosis for an etiological diagnosis of the malformations.

  13. Atypical causes of nontraumatic intracranial subarachnoid hemorrhage.

    PubMed

    Carvi y Nievas, Mario Nazareno; Archavlis, Eleftherios

    2009-05-01

    To analyze the management and outcome of patients presenting with atypical causes of intracranial subarachnoid hemorrhage (SAH). We performed a review of our last 820 nontraumatic-SAH patients and analyzed the management and outcome of patients where the SAH origin was not a ruptured aneurysm. The Glasgow Outcome Scale (GOS) was used to assess outcome 3 months after event. Thirty-two patients had atypical causes of SAH. In 15 patients with Hunt and Hess (H&H) scores from 1 to 3 without focal neurological deficit (FND), 8 perimesencephalic non-aneurysmal SAH, 4 blood coagulation disorders, 1 sinus thrombosis, 1 vasculitis, and 1 unknown-origin-SAH (UOS) were diagnosed. Fourteen (93%) of these 15 patients were conservatively treated. In 17 patients with H&H scores from 3 to 5 and FND, 8 tumors, 1 cavernoma, 1 sinus thrombosis, 1 arteriovenous malformation, 1 blood coagulation disorders, 2 UOS, and 3 dural fistulas were diagnosed. Fifteen (88%) of these 17 patients were interventionally treated. The neurological condition 3 months later was good (GOS 4 and 5) in 12 of the 15 cases (80%) admitted with low-H&H scores, as well as in 13 of the 17 cases (76%) admitted with high-H&H scores. Three patients died and four developed a severe disability. Patients presenting with atypical causes of SAH and high-H&H scores at admission are likely to harbor an intracranial organic process producing the bleeding. Despite this poor initial condition, their 3-month outcome can be similar to those of patients with low-H&H scores if the origin of the bleeding is properly treated.

  14. Multiple intracranial enterogenous cysts.

    PubMed Central

    Walls, T J; Purohit, D P; Aji, W S; Schofield, I S; Barwick, D D

    1986-01-01

    The case of a 40-year-old woman with increasing ataxia is described. Although the clinical presentation and evoked response studies raised the possibility of multiple sclerosis, further investigation revealed multiple cystic intracranial lesions. Surgical excision of one of the lesions relieved the patient's symptoms. Histological examination revealed that this was an enterogenous cyst. Although single cysts of this type have rarely been reported occurring in the posterior cranial fossa, the occurrence of multiple lesions, some in the supratentorial compartment, appears to be unique. Images PMID:3701354

  15. Ruptured intracranial dermoid cyst.

    PubMed

    Oursin, C; Wetzel, S G; Lyrer, P; Bächli, H; Stock, K W

    1999-09-01

    Intradural dermoids are rare congenital tumors representing approximately 0.05% of all intracranial lesions. These benign tumors have a typical appearance on CT and MR due to their lipid components. The complication caused by rupture are the spillage of the fatty material into the cerebrospinal fluid. We report a case of a ruptured dermoid cyst showing fat/fluid levels in both side ventricles and fatty material in the subarachnoid space on CT and MR-imaging and the follow-up over four years after incomplete resection of the tumor.

  16. Pediatric intracranial aneurysms.

    PubMed

    Tripathy, L N; Singh, S N

    2009-01-01

    The incidence of subarachnoid haemorrhage from intracranial aneurysms in the paediatric age group is extremely rare. Interestingly, occurrence of vasospasm has been reported to be less in comparison to the adults. Both coiling and clipping have been advocated in selected cases. Because of the thinness of the wall of the arteries, utmost care should be taken while handling these arteries during surgery. The overall results of surgery in children have been reported to be better than their adult counterparts. We present four such cases from our own experience. All these children were operated upon, where the solitary aneurysm in each case was clipped and all of them made a good recovery.

  17. Preoperative Assessment of Adult Patients for Intracranial Surgery

    PubMed Central

    Sivanaser, Vanitha; Manninen, Pirjo

    2010-01-01

    The preoperative assessment of the patient for neurosurgical and endovascular procedures involves the understanding of the neurological disease and its systemic presentation, and the requirements of the procedure. There is a wide spectrum of different neurosurgical disorders and procedures. This article provides an overview of the preoperative evaluation of these patients with respect to general principles of neuroanesthesia, and considerations for specific intracranial and vascular neurosurgical and interventional neuroradiological procedures. PMID:20700431

  18. Intracranial abscess in Ectopia Cordis.

    PubMed

    Merola, Joseph; Tipper, Geoffrey Adrian; Hussain, Zakier; Balakrishnan, Venkataraman; Gan, Peter

    2014-08-25

    We present a case of intracranial abscess in a young female with Ectopia Cordis, an exceptionally rare cardiac condition. The neurosurgical implication is the predisposition to intracranial abscess formation. A heightened awareness of this association will aid diagnosis in similar clinical scenarios.

  19. In vitro model test and preliminary clinical application of a new method of ultrasonographic imaging: Vascular enhancement technology.

    PubMed

    Liu, Xi; Duan, Yun You; Wang, Jia; Sun, Si Guo; Li, Juan; Hou, Wei Hua; Cao, Tie Sheng

    2009-09-01

    Vascular enhancement technology (VET) is a new form of ultrasonographic technology that can optimize images by enhancing the B-mode display with information derived from power Doppler. We designed an in vitro model to evaluate the accuracy and application method of VET and to apply this technology preliminarily in vivo in the vascular periphery. An in vitro model was designed with a flow pump system to simulate blood flow in soft tissue and the intracranial vasculature. Modeling vessels were imaged by traditional B mode, color Doppler flow imaging and VET. The diameter of the various silicon tubes was measured to verify the accuracy of VET. For in vivo application, 15 normal subjects and 26 patients suspected of having carotid artery plaques and cerebrovascular disease were examined using these three image modes. The imaging effects were observed and compared. VET imaging could clarify the lumens of the modeling vessels and reduce artifacts. The caliber of three sizes of silicon tubing was also measured accurately by VET. Of 15 normal subjects, sound artifacts in large vessels were inhibited and the intermedia membrane was clearly displayed by VET. The boundaries of carotid plaques were manifested by VET with well-defined edges. Three cases of hypoechoic soft plaque on the anterior wall missed in B-mode imaging were detected by VET. Intracranial scanning with VET identified cerebral vascular disease, including cerebral stenosis, arteriovenous malformations and aneurysms. The size and shape of the focus displayed by VET coincided with that observed using digital subtraction arteriography. VET is helpful in improving detection of the boundary of vessels and visualization of the microvasculature.

  20. Pediatric Intracranial Hypertension.

    PubMed

    Aylward, Shawn C; Reem, Rachel E

    2017-01-01

    Primary (idiopathic) intracranial hypertension has been considered to be a rare entity, but with no precise estimates of the pediatric incidence in the United States. There have been attempts to revise the criteria over the years and adapt the adult criteria for use in pediatrics. The clinical presentation varies with age, and symptoms tending to be less obvious in younger individuals. In the prepubertal population, incidentally discovered optic disc edema is relatively common. By far the most consistent symptom is headache; other symptoms include nausea, vomiting tinnitus, and diplopia. Treatment mainstays include weight loss when appropriate and acetazolamide. Furosemide may exhibit a synergistic benefit when used in conjunction with acetazolamide. Surgical interventions are required relatively infrequently, but include optic nerve sheath fenestration and cerebrospinal fluid shunting. Pain and permanent vision loss are the two major complications of this disorder and these manifestations justify aggressive treatment. Once intracranial hypertension has resolved, up to two thirds of patients develop a new or chronic headache type that is different from their initial presenting headache.

  1. Intracranial Hypertension Is Painless!

    PubMed

    Manet, R; Fabre, N; Moyse, E; Laurent, B; Schmidt, E A

    2016-01-01

    Headache is usually considered a key symptom of intracranial hypertension (ICHT). However, there are no published experimental data to support the concept that increased intracranial pressure (ICP) is painful in humans. This prospective study was performed in 16 patients with suspected normal-pressure hydrocephalus, necessitating a lumbar infusion test with measurement of cerebrospinal fluid (CSF) hydrodynamics. During the test, ICP was increased from baseline to a plateau. Headache was scored on a visual analog scale (VAS) (0 = no pain, 10 = very severe pain) at baseline ICP and when ICP plateaued. At baseline, mean ICP was 11 ± 3.6 mmHg and VAS was 0. At plateau, mean ICP was 28 ± 9.5 mmHg and VAS was 0. There was a significant increase in ICP (p <0.001), but no increase in headache intensity (VAS). An acute (20-min) moderate increase in ICP was not accompanied by a headache. We demonstrate that an acute, isolated increase in CSF pressure does not produce a headache. To occur, a headache needs activation of the pain-sensitive structures (dura and venous sinuses) or central activation of the cerebral nociceptive structures. This peripheral or central activation does not occur with an isolated increase in CSF pressure.

  2. Endovascular correction of an infantile intracranial venous outflow obstruction.

    PubMed

    Soltanolkotabi, Maryam; Rahimi, Shahram; Hurley, Michael C; Bowman, Robin M; Russell, Eric J; Ansari, Sameer A; Shaibani, Ali

    2013-12-01

    The authors report on the case of a 7-year-old boy who presented with a reduced level of activity, macrocephaly, prominent scalp veins, and decreased left-sided visual acuity. Imaging workup demonstrated generalized cerebral volume loss, bilateral chronic subdural hematomas, absent left sigmoid sinus, hypoplastic left transverse sinus, and severe focal weblike stenosis of the right sigmoid sinus. Right sigmoid sinus angioplasty and stent insertion was performed, with an immediate reduction in the transduced intracranial venous pressure gradient across the stenosis (from 22 to 3 mm Hg). Postprocedural diminution of prominent scalp and forehead veins and spinal venous collateral vessels was followed by a progressive improvement in visual acuity and physical activity over a 1-year follow-up period, supporting the efficacy of angioplasty and stent insertion in intracranial venous outflow obstruction. There are multiple potential causes of intracranial venous hypertension in children. Development of dural sinus stenosis in infancy may be one such cause, mimicking the clinical presentation of other causes such as vein of Galen malformations. This condition can be ameliorated by early endovascular revascularization.

  3. The utility of GLUT1 as a diagnostic marker in cutaneous vascular anomalies: A review of literature and recommendations for daily practice.

    PubMed

    van Vugt, Lieke J; van der Vleuten, Carine J M; Flucke, Uta; Blokx, Willeke A M

    2017-06-01

    To assess the utility of GLUT1 as an immunohistochemical marker in the diagnostics of cutaneous vascular anomalies. A systematic literature search was conducted for studies on GLUT1 staining patterns in cutaneous vascular lesions. Data was grouped according to the latest ISSVA classification for vascular anomalies. Vascular tumors: GLUT1 staining was positive in 368/386 (95%) of infantile hemangiomas. Congenital hemangiomas (16 cases) and kaposiform hemangioendotheliomas (62 cases) were all negative for GLUT1. Angiosarcomas were GLUT1 positive in 12/39 (31%) and epithelioid hemangioendotheliomas in 2/27 (7%) of cases. Vascular malformations: All vascular malformations (33 arteriovenous malformations, 16 capillary malformations, 64 lymphatic malformations, 54 venous malformations, 3 venous-lymphatic malformations and 3 capillary venous-lymphatic malformations) were negative for GLUT1 staining. Unclassified vascular anomalies: Angiokeratomas were GLUT1 positive in 1/15 (7%) and verrucous hemangiomas in 71/100 (71%) of cases. Microvenular hemangiomas were negative for GLUT1 in all 9 cases. GLUT1 can be used as an additional diagnostic tool in cutaneous vascular lesions. A negative GLUT1 stain renders a diagnosis of infantile hemangioma unlikely. A positive GLUT1 stain excludes vascular malformations and is suggestive of infantile hemangioma. One must be cautious, however, that the final diagnosis is made through interpretation of all clinical and diagnostic features, and not based on GLUT1 staining alone. Copyright © 2017 Elsevier GmbH. All rights reserved.

  4. Pulmonary Arteriovenous Malformations

    PubMed Central

    2014-01-01

    Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ∼1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Paradoxical emboli lead to strokes and cerebral abscesses, and these commonly occur in individuals with previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of maternal death in pregnancy. PAVM occlusion by embolization is the standard of care to reduce these risks. However, recent data demonstrate that currently recommended management protocols can result in levels of radiation exposure that would be classified as harmful. Recent publications also provide a better appreciation of the hematologic and cardiovascular demands required to maintain arterial oxygen content and oxygen consumption in hypoxemic patients, identify patient subgroups at higher risk of complications, and emphasize the proportion of radiologically visible PAVMs too small to treat by embolization. This review, therefore, outlines medical states that exacerbate the consequences of PAVMs. Chief among these is iron deficiency, which is commonly present due to concurrent hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia compensations by restricting erythropoiesis and increases the risk of ischemic strokes. Management of periodontal disease, dental interventions, pulmonary hypertension, and pregnancy also requires specific consideration in the setting of PAVMs. The review concludes by discussing to what extent previously recommended protocols may benefit from modification or revision. PMID:25420112

  5. Embolization of Arteriovenous Malformation

    PubMed Central

    Nagashima, H.; Hongo, K.; Kobayashi, S.; Takamae, T.; Okudera, H.; Koyama, J.I.; Oya, F.; Matsumoto, Y.

    2004-01-01

    Summary Treatment options for cerebral arteriovenous malformation (AVM) are still controversial due to the recent result of stereotactic radiosurgery and the improved result of microsurgical resection. We investigated previously treated AVM cases and discussed the efficacy and safety of preoperative embolization especially for microsurgical resection of high-grade AVM in the Spetzler-Martin grading. Efficacy of preoperative embolization was evaluated based on 126 previously treated AVM cases at Shinshu University Hospital during the last 25 years. The safety of embolization was evaluated based on our previously-embolized 58 AVM cases (91 procedures) in the last 11 years after introduction of preoperative embolization for AVM. In all 126 cases, 82 were treated before introduction of embolization and 44 were treated after introduction of embolization. In 82 cases of the pre-embolization era, 63 lesions were removed totally in 63 AVMs (77%), partially resected in 11 (13%) and untreated in eight (10%). In 74 surgically removed cases, 11 (15%) cases showed severe intra/postoperative bleeding. In 44 cases of the embolization era, lesions were removed totally in 29 AVMs (66%), disappeared only with embolization in one (2%), disappeared with radiosurgery in seven (16%) and were untreated in five (11%). In 32 surgically removed cases, only one (2%) case showed severe intra/postoperative bleeding. In all 58 embolized cases, 44 were surgically removed, six were treated with radiosurgery, one was eliminated with embolization alone and six were partially obliterated and followed up for their location. In 91 procedures for 58 cases, two haemorrhagic and three ischemic complications occurred, three were transient and two remained having neurological deficits. The introduction of preoperative embolization improved the total removal rate and reduced the intra/postoperative bleeding rate in surgical removal of AVM. The total risk of embolization is low and well-designed preoperative

  6. The population-based Barcelona-Asymptomatic Intracranial Atherosclerosis Study (ASIA): rationale and design

    PubMed Central

    2011-01-01

    Background Large-artery intracranial atherosclerosis may be the most frequent cause of ischemic stroke worldwide. Traditional approaches have attempted to target the disease when it is already symptomatic. However, early detection of intracranial atherosclerosis may allow therapeutic intervention while the disease is still asymptomatic. The prevalence and natural history of asymptomatic intracranial atherosclerosis in Caucasians remain unclear. The aims of the Barcelona-ASymptomatic Intracranial Atherosclerosis (ASIA) study are (1) to determine the prevalence of ASIA in a moderate-high vascular risk population, (2) to study its prognostic impact on the risk of suffering future major ischemic events, and (3) to identify predictors of the development, progression and clinical expression of this condition. Methods/Design Cross-over and cohort, population-based study. A randomly selected representative sample of 1,503 subjects with a mild-moderate-high vascular risk (as defined by a REGICOR score ≥ 5%) and with neither a history of cerebrovascular nor ischemic heart disease will be studied. At baseline, all individuals will undergo extracranial and transcranial Color-Coded Duplex (TCCD) ultrasound examinations to detect presence and severity of extra and intracranial atherosclerosis. Intracranial stenoses will be assessed by magnetic resonance angiography (MRA). Clinical and demographic variables will be recorded and blood samples will be drawn to investigate clinical, biological and genetic factors associated with the presence of ASIA. A long-term clinical and sonographic follow-up will be conducted thereafter to identify predictors of disease progression and of incident vascular events. Discussion The Barcelona-ASIA is a population-based study aiming to evaluate the prevalence and clinical importance of asymptomatic intracranial large-artery atherosclerosis in Caucasians. The ASIA project may provide a unique scientific resource to better understand the dynamics of

  7. Venous malformation: update on aetiopathogenesis, diagnosis and management.

    PubMed

    Dompmartin, A; Vikkula, M; Boon, L M

    2010-10-01

    The aim of this review was to discuss the current knowledge on aetiopathogenesis, diagnosis and therapeutic management of venous malformations (VMs). VMs are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneomucosal VMs or glomuvenous malformations), combined (e.g. capillaro-venous and capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, blue rubber bleb naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of VMs within vascular anomalies. Those associated with pain are often responsive to low-molecular-weight heparin, which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose-ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area.

  8. Multiple cavernous malformations presenting in a patient with Poland syndrome: A case report.

    PubMed

    Lizarraga, Karlo J; De Salles, Antonio Af

    2011-09-20

    Poland syndrome is a congenital disorder related to chest and hand anomalies on one side of the body. Its etiology remains unclear, with an ipsilateral vascular alteration (of unknown origin) to the subclavian artery in early embryogenesis being the currently accepted theory. Cavernous malformations are vascular hamartomas, which have been linked to a genetic etiology, particularly in familial cases, which commonly present with multiple lesions. Our case report is the first to describe multiple cavernous malformations associated with Poland syndrome, further supporting the vascular etiology theory, but pointing to a genetic rather than a mechanistic factor disrupting blood flow in the corresponding vessels. A 41-year-old Caucasian man with Poland syndrome on the right side of his body presented to our hospital with a secondary generalized seizure and was found to have multiple cavernous malformations distributed in his brain, cerebellum, and brain stem, with a predominance of lesions in the left hemisphere. The distribution of cavernous malformations in the left hemisphere and the right-sided Poland syndrome in our patient could not be explained by a mechanistic disruption of one of the subclavian arteries. A genetic alteration, as in familial cavernous malformations, would be a more appropriate etiologic diagnosis of Poland syndrome in our patient. Further genetic and pathological studies of the involved blood vessels in patients with Poland syndrome could lead to a better understanding of the disease.

  9. Novel Magnetic Resonance Imaging Findings in Children With Intracranial Hypertension.

    PubMed

    Hirfanoglu, Tugba; Aydin, Kursad; Serdaroglu, Ayse; Havali, Cengiz

    2015-08-01

    Increased intracranial hypertension is defined as elevated intracranial pressure with absence of hydrocephalus, vascular or structural abnormalities, and normal cerebrospinal fluid content. Magnetic resonance imaging abnormalities of the optic nerve and sheath that have been described in adults include increased nerve tortuosity, flattening in posterior aspect of globe, intraocular protrusion of the optic nerve, and enlarged optic nerve sheath. We evaluated accuracy of those proposed markers on magnetic resonance imaging in children with increased intracranial hypertension that are described in adults. Eleven patients between 3 and 15 years of age with intracranial hypertension were selected for re-evaluation of magnetic resonance imaging that had been previously described as normal to determine the presence of: (1) increased tortuosity and elongation of the optic nerve; (2) enlargement of the optic nerve sheath on axial and coronal T2 so called by us "target sign" and postcontrast T1 sequences; (3) flattening in posterior aspect of the globe; and (4) intraocular protrusion of the optic nerve head. Of the 11 patients, tortuosity of the optic nerve (10/11, 90.9%) and enlarged optic nerve sheath--target sign (7/11, 63.6%)--were the most common findings. Flattening in the posterior aspect of globe (5/11, 45.5%) and intraocular protrusion (3/11, 27.3%) were also detected as a novel magnetic resonance imaging findings. Magnetic resonance imaging findings of the optic nerve and sheath include valuable signs of intracranial hypertension not only in adults but also in children. This is the first detailed analysis of the magnetic resonance imaging findings in children with increased intracranial hypertension. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. The unusual angiographic course of intracranial pseudoaneurysms

    PubMed Central

    Zanaty, Mario; Chalouhi, Nohra; Jabbour, Pascal; Starke, Robert M.; Hasan, David

    2015-01-01

    Although rare, traumatic intracranial pseudoaneurysms remain one of the most difficult vascular lesions to diagnose and treat. A 55-year-old male patient underwent endoscopic endonasal transphenoidal resection for a pituitary macroadenoma. The operation was complicated by an arterial bleed. The initial angiogram revealed pseudoaneurysm of the anterior choroidal artery. Although the pseudoaneurysm completely disappeared on the second angiogram, it was surprisingly found to have enlarged on the third angiogram. The lesion was successfully treated with flow-diversion using a pipeline embolization device. The present case demonstrates that the natural history of iatrogenic pseudoaneurysms may be unpredictable and misleading. Traumatic pseudoaneurysms should, therefore, be carefully followed when conservative treatment is elected or when the lesion seems to have spontaneously regressed. Flow-diversion seems to be a reasonable treatment option. PMID:26425168

  11. Primary intracranial choriocarcinoma: MR imaging findings.

    PubMed

    Lv, X-F; Qiu, Y-W; Zhang, X-L; Han, L-J; Qiu, S-J; Xiong, W; Wen, G; Zhang, Y-Z; Zhang, J

    2010-11-01

    PICCC is the rarest, most malignant primary intracranial GCT. The purpose of this study was to describe and characterize the MR imaging findings in a series of 7 patients (6 males and 1 female; mean age, 11.9 years) with pathologically proved PICCC in our institution from 2004 to 2009. All tumors were located within the pineal (n = 6) or suprasellar (n = 1) regions. On T2-weighted MR imaging, the lesions appeared markedly heterogeneous with areas of both hypointensity and hyperintensity reflecting the histologic heterogeneity, including hemorrhage, fibrosis, cysts, or necrosis. Heterogeneous (n = 7), ringlike (n = 4), and/or intratumoral nodular (n = 3) enhancement was noted on T1-weighted images with gadolinium. These MR imaging findings, combined with patient age and serum β-HCG levels, may prove helpful in distinguishing PICCC from the more common primary brain tumors, thereby avoiding biopsy of this highly vascular tumor.

  12. Telemetry of intracranial pressure

    NASA Technical Reports Server (NTRS)

    Fryer, T. B.; Corbin, S. D.; Silverberg, G. D.; Schmidt, E. V.; Ream, A. K.

    1978-01-01

    A completely implantable epidural pressure telemetry system designed for accurate measurement of intracranial pressure (ICP) is described. The implant device is batteryless, providing unlimited operating life. The described system uses a capacitive pressure transducer with excellent long-term stability. Once detected with the transducer and converted to a frequency with the oscillator electronics, the pressure signal is digitized. It is then telemetered without the possibility of further degradation. After detection with the small external module, the data can be retransmitted by a radio link for complete patient mobility or the energizer signal pickup module can be wired to a bedside readout unit. Continuous data are available from the system so that the dynamic ICP changes reflecting arterial blood pressure can be observed and used for diagnosis.

  13. Traumatic Arteriovenous Malformation of the Superficial Temporal Artery.

    PubMed

    Fard, Mahmoud Omrani; Yousofnejad, Omid; Heydari, Mohammadbagher

    2017-01-01

    Most of the vascular lesions on head and neck soft tissue are congenital, but a rare cause can be trauma. A 23-year-old man came to our clinic with a wide pulsatile tortuous mass in the left temporofrontal area. That mass was appeared since 10 years ago. Ten years before his coming to our clinic, he had a blunt trauma in that area. After that, a small wound appeared there and healed gradually. In clinical examination, mass was large and pulsatile, and a fine murmur was detected from it. In paraclinical examination, computed tomography scan with intravenous contrast and sonography revealed a vascular mass with arteriovenous (AV) fistula in soft tissue only in that area. We operated him and vascular mass completely excised without recurrency. Pathologic report was AV malformation (AVM). According to our study, also rare trauma is one of the causes of AVMs, and we able to resection it completely without recurrency.

  14. Kernohan's phenomenon associated with left ruptured occipital arteriovenous malformation.

    PubMed

    Fujimoto, A; Sato, H; Katayama, W; Nakai, K; Tsunoda, T; Kobayashi, E; Nose, T

    2004-05-01

    A 23-year-old woman presented with ipsilateral hemiparesia due to rupture of a left occipital arteriovenous malformation (AVM). Emergency decompression (the onset-operation interval was 46 minutes,) was carried out and the patient could leave the hospital upon recovery without neurological deficits. In general, Kernohan's phenomenon is caused by the gradual displacement of the cerebral peduncle against the tentorial edge caused by compression by the contralateral mass. This phenomenon is very rare among the cases with spontaneous intracranial hemorrhage and only three cases including the present one have been reported in the literature. In all cases the onset-operation intervals of were very short. Kernohan's phenomenon associated with a ruptured AVM is a rare condition and emergency decompression is required.

  15. Glomuvenous malformations with smooth muscle and eccrine glands: unusual histopathologic features in a familial setting.

    PubMed

    Borroni, Riccardo G; Grassi, Sara; Concardi, Monica; Puccio, Ignazio; Giordano, Calogero; Agozzino, Manuela; Caspani, Clelia; Grasso, Maurizia; Diegoli, Marta; Arbustini, Eloisa

    2014-03-01

    Glomuvenous malformations (OMIM 138000) are hamartomas presenting in childhood as multiple, bluish papules and nodules in the skin, which are characterized histopathologically by irregular vascular spaces surrounded by typical glomus cells. Glomuvenous malformations are caused by autosomal dominant mutations of the GLMN gene. A 34-year-old woman and her 16-year-old son presented with bluish papules and nodules since childhood. Biopsy specimens from both patients showed histopathologic features of glomuvenous malformations, unusually in consistent and close association with smooth muscle, hair follicles and eccrine glands. Sequencing of the GLMN gene revealed the p.C36X (c.108C>A) mutation in germline DNA from both patients. This is probably the first report describing the hamartomatous features of familial glomuvenous malformations consistently associated with a prominent smooth muscle component and eccrine glands.

  16. Vein of Galen malformation in a neonate: A case report and review of endovascular management

    PubMed Central

    Puvabanditsin, Surasak; Mehta, Rajeev; Palomares, Kristy; Gengel, Natalie; Da Silva, Christina Ferrucci; Roychowdhury, Sudipta; Gupta, Gaurav; Kashyap, Arun; Sorrentino, David

    2017-01-01

    Vein of Galen malformation (VOGM) is a rare congenital vascular malformation caused by the maldevelopment of its embryonic precursor, the median prosencephalic vein of Markowski. VOGM results in neonatal morbidity and mortality, and premature delivery does not improve the outcome. We report a term female neonate in whom a vein of Galen malformation was diagnosed prenatally at 37 wk of gestation during a growth ultrasound and confirmed by fetal magnetic resonance imaging. Signs of cardiac decompensation were evident in the fetus. Multiple interventional radiology embolizations of the feeding vessels were performed successfully on days 7, 10, 12, 14 and 19. A review of the literature on the endovascular management of neonates with these malformations is presented herein. PMID:28224101

  17. Unruptured Intracranial Aneurysms

    PubMed Central

    Raymond, J.; Guillemin, F.; Proust, F.; Molyneux, A.J.; Fox, A.J.; Claiborne, J.S.; Meder, J.-F.; Rouleau, I.

    2008-01-01

    Summary The preventive treatment of unruptured aneur­ysms has been performed for decades despite the lack of evidence of a clinical benefit. Reports of observational studies such as the International Study of Unruptured Intracranial Aneurysms (ISUIA) suggest that preventive treatments are rarely justified. Are these reports compelling enough to guide clinical practice? The ISUIA methods and data are reviewed and analysed in a more conventional manner. The design of the appropriate clinical research program is approached by steps, reviewing potential problems, from the formulation of the precise research question to the interpretation of subgroup analyses, including sample size, representativity, duration of observation period, blin­ding, definition of outcome events, analysis of cross-overs, losses to follow-up, and data reporting. Unruptured intracranial aneurysms observed in ISUIA ruptured at a minimal annual rate of 0.8% (0.5-1%), despite multiple methodological difficulties biased in favour of a benign natural history. Available registries do not have the power or the design capable of providing normative guidelines for clinical decisions. The appropriate method to solve the clinical dilemma is a multicentric trial comparing the incidence of a hard clinical outcome events in approximately 2000 patients randomly allocated to a treatment group and a deferred treatment group, all followed for ten years or more. Observational studies have failed to provide reliable evidence in favour or against the preventive treatment of unruptured aneurysms. A randomized trial is in order to clarify what is the role of prevention in this common clinical problem. PMID:20557790

  18. A somatic GNA11 mutation is associated with extremity capillary malformation and overgrowth.

    PubMed

    Couto, Javier A; Ayturk, Ugur M; Konczyk, Dennis J; Goss, Jeremy A; Huang, August Y; Hann, Steve; Reeve, Jennifer L; Liang, Marilyn G; Bischoff, Joyce; Warman, Matthew L; Greene, Arin K

    2017-08-01

    Capillary malformation is a cutaneous vascular anomaly that is present at birth, darkens over time, and can cause overgrowth of tissues beneath the stain. The lesion is caused by a somatic activating mutation in GNAQ. In a previous study, we were unable to identify a GNAQ mutation in patients with a capillary malformation involving an overgrown lower extremity. We hypothesized that mutations in GNA11 or GNA14, genes closely related to GNAQ, also may cause capillary malformations. Human capillary malformation tissue obtained from 8 patients that had tested negative for GNAQ mutations were studied. Lesions involved an extremity (n = 7) or trunk (n = 1). Droplet digital PCR (ddPCR) was used to detect GNA11 or GNA14 mutant cells (p.Arg183) in the specimens. Single molecule molecular inversion probe sequencing (smMIP-seq) was performed to search for other mutations in GNA11. Mutations were validated by subcloning and sequencing amplimers. We found a somatic GNA11 missense mutation (c.547C > T; p.Arg183Cys) in 3 patients with a diffuse capillary malformation of an extremity. Mutant allelic frequencies ranged from 0.3 to 5.0%. GNA11 or GNA14 mutations were not found in 5 affected tissues or in unaffected tissues (white blood cell DNA). GNA11 mutations are associated with extremity capillary malformations causing overgrowth. Pharmacotherapy that affects GNA11 signaling may prevent the progression of capillary malformations.

  19. Pediatric lymphatic malformations: evolving understanding and therapeutic options.

    PubMed

    Defnet, Ann M; Bagrodia, Naina; Hernandez, Sonia L; Gwilliam, Natalie; Kandel, Jessica J

    2016-05-01

    Multimodal treatment of lymphatic malformations continues to expand as new information about the biology and genetics of these lesions is discovered, along with knowledge gained from clinical practice. A patient-centered approach, ideally provided by a multidisciplinary medical and surgical team, should guide timing and modality of treatment. Current treatment options include observation, surgery, sclerotherapy, radiofrequency ablation, and laser therapy. New medical and surgical therapies are emerging, and include sildenafil, propranolol, sirolimus, and vascularized lymph node transfer. The primary focus of management is to support and optimize these patients' quality of life. Researchers continue to study lymphatic malformations with the goal of increasing therapeutic options and developing effective clinical pathways for these complicated lesions.

  20. Successful breastfeeding with breast malformations.

    PubMed

    Faridi, M M A; Dewan, Pooja

    2008-11-01

    Congenital and acquired malformations of the breast can present with difficulty in breastfeeding. The authors report on 3 mothers of Indian origin who successfully breastfed their babies after appropriate counseling despite having different breast malformations. One of the mothers had bilateral ectopic axillary breasts but was able to breastfeed after expressing them. The other 2 mothers had burns scars over the chest and breasts, with a flat nipple in 1 of them, and a psychological barrier was seen in both of them. Both mothers were able to successfully breastfeed after counseling and some practical help.

  1. Arteriovenous malformation (AVM) of the maxilla complicated by a local pathogen infection

    PubMed Central

    Ella, Bruno; Lanotte, Aurelie; Sedarat, Cyril; Fricain, Jean-Christophe

    2013-01-01

    Arteriovenous malformations (AVM) are usually observed on the skin, but they can also rarely affect the visceral organs and the maxillofacial area. In the maxillofacial area, these lesions can spread and they are potentially dangerous, as a biopsy or even a simple tooth extraction can lead to a catastrophic haemorrhage that may eventually cause death. There are several indications for the treatment, including age, size and type of vascular malformation. The purpose of this report is to describe an AVM on the maxilla, first diagnosed as an epulis, which has been treated for several years as a chronic local periodontitis. The lesion was finally stabilised after an antibiotic therapy. Improved knowledge of the development of these lesions and collaboration between dentists, radiologists and surgeons are necessary to improve the diagnosis and treatment of vascular malformations. PMID:23376660

  2. Understanding venous malformations of the head and neck: a comprehensive insight.

    PubMed

    Colletti, Giacomo; Ierardi, Anna Maria

    2017-03-01

    Venous malformations (VMs) are congenital vascular malformations. They are very often misnamed and thus incorrectly managed. The aim of the present paper is to provide the reader with the most updated literature available and to offer a detailed description of each single aspect of this disease. In detail, the paper discusses the epidemiology, the embryological origins and the physiopathology of VMs. Then, the clinical features of sporadic, inherited and syndromic VMs are discussed. The instrumental diagnosis is presented, and the role of US, CT, MRI and phlebography is pointed out. Differential diagnoses with other vascular malformations and tumors are described. The clinical session ends with the staging of VMs relying on MRI and rheological features. All aspects of treatment are described: conservative measures, medical treatment, sclerotherapy, laser and surgery are thoroughly discussed. A section is reserved to bony VMs. Their clinical aspects and the appropriate treatment are presented.

  3. [Classification of superficial vascular anomalies].

    PubMed

    Enjolras, Odile; Soupre, Véronique; Picard, Arnaud

    2010-04-01

    All superficial vascular abnormalities are not angiomas even though this term continues - incorrectly - to be used. Because the suffix "oma" implies a tumor, it is necessary to differentiate true vascular tumors, such as infantile hemangioma, from vascular malformations. From a hemodynamic perspective, there are two types of vascular malformations: slow- and fast-flow. In addition to the functioning of the impaired or severely damaged vessels, we discuss slow-flow capillary, venous, or lymphatic malformations and rapid flow arterial and arteriovenous malformations. All combinations are possible. There are several types of childhood vascular tumors with different courses and different prognoses. Infantile hemangioma is by far the most frequent (8 to 10 children/100). The diverse other vascular tumors in children are sufficiently rare that they are described as orphan diseases. Since the end of the last century, a simple endothelial marker, GLUT-1, is available. This immunophenotype is present in all cases of infantile hemangioma at every stage and is negative in other tumors. Kasabach-Merritt syndrome and its accompanying severe thrombocytopenia never complicate childhood hemangioma, contrary to what has been said for nearly 60 years. When it is present, the tumor is either a tufted angioma or kaposiform hemangioendothelioma, and the GLUT1 marker can distinguish them from infantile hemangioma if the histologic diagnosis is uncertain (GLUT 1 is negative in both the latter cases). There are a wide variety of rare vascular tumors; many of them are benign, isolated, or limited; some are locally aggressive and recur after excision. A small number are low-grade malignant lesions with a risk of multivessel expansion, metastasis, and sometimes a fatal outcome. Major progress has been made in the imaging of these vascular abnormalities. Magnetic resonance imaging (MRI) in particular has revolutionized the non-invasive and especially the non-irradiating exploration of many of

  4. Genetic Screening of Pediatric Cavernous Malformations.

    PubMed

    Merello, Elisa; Pavanello, Marco; Consales, Alessandro; Mascelli, Samantha; Raso, Alessandro; Accogli, Andrea; Cama, Armando; Valeria, Capra; De Marco, Patrizia

    2016-10-01

    Cerebral cavernous malformations (CCMs) are vascular malformations mostly located within the central nervous system. Heterozygous loss of function mutations in CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10 genes are identified in about 90 % of familial cases of CCMs and two thirds of sporadic cases with multiple lesions. In this study, we performed genetic screening of a cohort of 31 patients, mainly pediatric. We analyzed the CCM1, CCM2, and CCM3 genes by multiplex ligation-dependent probe amplification (MLPA) and direct sequencing of exons and intronic boundaries. A total of 9 typical pathogenic loss-of-function mutations were identified in 10 out 31 patients (32 %). The 75 % of familial cases were mutated and the percentage reached to 85 % when we consider only pediatric cases. Detection rate in sporadic cases with multiple lesions was considerably lower (16 %). We identified a novel variant of CCM3, the c.130-131insT (p.R45Efs*8), in 1 pediatric sporadic case with multiple lesions that introduced a premature termination codon into the messenger RNA (mRNA), most likely leading to mRNA decay. Similar to other CCM pediatric series, the main symptoms associated to clinical debut consisted of cerebral hemorrhage. In conclusion, the penetrance of CCM mutations in familial pediatric cases is high (85 %). The genetic workup could improve clinical and genetic counseling in CCM patients. Moreover, we confirmed the high risk of hemorrhage in children with CCMs.

  5. An intratesticular arteriovenous malformation identified incidentally during ultrasound evaluation of scrotal trauma

    PubMed Central

    Jafarpishefard, Mohammad Saleh; Momeni, Mohammad; Baradaran Mahdavi, Mohammad Mehdi; Momeni, Fatame; Kamal, Sima

    2016-01-01

    Intratesticular arteriovenous malformation (AVM) is an extremely rare benign testicular lesion. Ultrasonography (US) usually reveals a hypoechoic solid vascular mass within the testicular parenchyma. Herein, we report our experience with a young patient in whom an intratesticular AVM was found incidentally by US during the workup of scrotal trauma. PMID:28217640

  6. Combined laser and surgical treatment of giant port wine stain malformation - Case report

    NASA Astrophysics Data System (ADS)

    Siewiera, I.; Drozdowski, P.; Wójcicki, P.

    2012-10-01

    Background:Port-wine stains (PWS) are vascular malformations of the skin concerning about 0,3% of the population. Though various laser systems have been used for various treatment regimens the treatment of PWS of large size is especially difficult and demanding from aesthetic and psychological point of view.

  7. Possible association between acetazolamide administration during pregnancy and multiple congenital malformations

    PubMed Central

    Al-Saleem, Afnan I; Al-Jobair, Asma M

    2016-01-01

    Congenital malformations might occur because of environmental or genetic factors, and sometimes occur because of unknown causes. Acetazolamide is a carbonic anhydrase inhibitor that is used to treat idiopathic intracranial hypertension, glaucoma, and epilepsy. The use of acetazolamide has not been recommended for pregnant women because of reported teratogenic risks. Congenital malformations, such as ectrodactyly, syndactyly, cleft lip/palate, and retarded incisor teeth development, have been reported in experimental animals. However, tooth agenesis due to the use of acetazolamide has not been reported yet. Oligodontia is a severe type of tooth agenesis involving six or more congenitally missing teeth. The causes of oligodontia are attributed to environmental factors, such as irradiation, drugs, trauma, tumors, infection, genetic factors, or a combination. There is no credible evidence of undesirable effects of acetazolamide use in human pregnancy. However, we report a case of a 12-year-old Saudi boy who was exposed to maternal acetazolamide (1,000 mg/day) for treatment of idiopathic intracranial hypertension before pregnancy, during the first trimester, and throughout the pregnancy. This treatment might have resulted in some congenital malformations, such as ectrodactyly, syndactyly, and oligodontia. PMID:27143854

  8. [SURGICAL TREATMENT OF THE FACE CAPILLARY MALFORMATION].

    PubMed

    Galich, S P; Gindich, O A; Dabizha, A Yu; Ogorodnik, Ya P

    2015-08-01

    Results of surgical treatment of 37 patients for the head and neck capillary malformations were analyzed. Optimal surgical tactics, depending on the malformation form and localization, was proposed. Restitution of the tissues defect after excision of malformation, using the flaps transposition, have permitted to achieve good esthetic results.

  9. Intracranial artery stenosis and progression from mild cognitive impairment to Alzheimer disease.

    PubMed

    Zhu, Jie; Wang, Yanjiang; Li, Jing; Deng, Juan; Zhou, Huadong

    2014-03-11

    To assess the impact of intracranial arterial stenosis on the progression from mild cognitive impairment (MCI) to Alzheimer disease (AD). A total of 423 participants with MCI were included and evaluated with clinical and neuropsychological examinations annually for 4 years. The incidence of dementia due to AD was investigated. CT angiography was used to measure the stenosis of major intracranial arteries in the studied population. A mixed-effects regression model was used to analyze the association between intracranial arterial stenosis and the progression of MCI, which was assessed with the Mini-Mental State Examination and the Activities of Daily Living scale. Cox proportional hazards models were used to identify the association between intracranial arterial stenosis and dementia progression. At the end of the follow-up, 116 participants had progressed to dementia due to AD, while 223 remained in the MCI stage. Participants with moderate or severe intracranial arterial stenosis had a faster decline in cognition and function relative to participants without such stenosis. The presence of moderate or severe intracranial arterial stenosis significantly increased the risk of dementia progression, even after controlling for age, sex, education, vascular risk factors, and silent MRI lesions. Intracranial arterial stenosis increased the risk of developing AD dementia after MCI.

  10. Intracranial extra-axial hemangioma in a newborn: A case report and literature review

    PubMed Central

    Dalsin, Marcos; Silva, Rafael Sodré; Galdino Chaves, Jennyfer Paula; Oliveira, Francine Hehn; Martins Antunes, Ápio Cláudio; Vedolin, Leonardo Modesti

    2016-01-01

    Background: Congenital hemangiomas are benign vascular tumors, and the intracranial counterpart was described in very few cases. Case Description: A newborn presented with an intracranial tumor associated with an arachnoid cyst, diagnosed by antenatal ultrasound at 37 weeks of gestation. Surgery was indicated due to increased head circumference and bulging fontanelle, and a complete resection of an extra-axial red–brown tumor was performed at the 3rd week of life. Microscopy revealed a hemangioma. Conclusion: Hemangioma is a rare differential diagnosis that must be considered in extra-axial intracranial tumors affecting infants and neonates. The radiological features are not helpful in differentiating from other tumors, and surgery is indicated when the diagnosis is uncertain or whenever there are signs of increased intracranial pressure. PMID:27274403

  11. Reconstruction of middle ear malformations

    PubMed Central

    Schwager, Konrad

    2008-01-01

    Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients. PMID:22073077

  12. 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.

  13. Intralesional radiofrequency in venous malformations.

    PubMed

    Garg, S; Kumar, S; Singh, Y B

    2015-03-01

    Venous malformations are usually asymptomatic and managed conservatively. Treatment, in the form of laser, sclerotherapy, or resection, is needed only if lesions present with symptoms or cosmetic deformity. The aim of this study was to find out how effective radiofrequency ablation was in patients with incomplete or unsatisfactory resolution of a venous malformation after an intralesional injection of bleomycin. During the 5 year period 2008-2012, we organised a prospective, clinical study at a tertiary care centre. Patients were selected from the outpatient department of the Lady Hardinge Medical College and associated hospitals, New Delhi, India. Five patients with venous malformations were treated by intralesional injection of bleomycin in a dose of 0.5U/kg body weight, which was repeated every 2 weeks for a total of 8 injections. They then had multiple intralesional radiofrequency ablation every 2 months until a satisfactory outcome was achieved. After the initial 8 doses the reduction in the size of the lesions was minimal (less than 50%). After 2-4 applications of radiofrequency ablation there was appreciable reduction in the size of the lesions (about 80%) with good functional and cosmetic outcomes. Radiofrequency ablation is an effective adjunct for patients with venous malformations of the head and neck that have not responded satisfactorily to intralesional injection of bleomycin. To our knowledge radiofrequency ablation after intralesional injection of bleomycin has not previously been described as a treatment for venous malformations. Copyright © 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

  14. Compressive hematoma and deep arteriovenous malformation: Emergency endovascular occlusion via a venous approach with surgical evacuation of the hematoma

    PubMed Central

    Chau, Yves; Sachet, Marina; Cattet, Florian; Lonjon, Michel

    2016-01-01

    Compressive hematoma associated with deep arterio-venous malformation is a difficult surgery. Arterial presurgical embolization is often indicated but rarely effective. Endovascular occlusion via a venous approach is a technique that has recently been undertaken successfully to treat certain types of sub-pial vascular malformation. The venous endovascular approach has succeeded in a 20-year-old man of who presented with a compressive hematoma due to rupture of a deep arteriovenous malformation. By fully endovascular occlusion, the surgery consisted of surgical removal of the hematoma, with reduced cortical damage. PMID:26825136

  15. Compressive hematoma and deep arteriovenous malformation: Emergency endovascular occlusion via a venous approach with surgical evacuation of the hematoma.

    PubMed

    Sedat, Jacques; Chau, Yves; Sachet, Marina; Cattet, Florian; Lonjon, Michel

    2016-02-01

    Compressive hematoma associated with deep arterio-venous malformation is a difficult surgery. Arterial presurgical embolization is often indicated but rarely effective. Endovascular occlusion via a venous approach is a technique that has recently been undertaken successfully to treat certain types of sub-pial vascular malformation. The venous endovascular approach has succeeded in a 20-year-old man of who presented with a compressive hematoma due to rupture of a deep arteriovenous malformation. By fully endovascular occlusion, the surgery consisted of surgical removal of the hematoma, with reduced cortical damage.

  16. Risk Factors Associated with Severity and Location of Intracranial Arterial Stenosis

    PubMed Central

    Turan, Tanya N.; Makki, Achraf A.; Tsappidi, Samuel; Cotsonis, George; Lynn, Michael J.; Cloft, Harry J.; Chimowitz, Marc I.

    2010-01-01

    Background and Purpose We sought to determine vascular risk factors and demographic features associated with the severity and location of intracranial stenosis. Methods Data on patients enrolled in the Warfarin-Aspirin Symptomatic Intracranial Disease (WASID) trial were used for the analyses. Demographic features and vascular risk factors were compared in patients with moderate stenosis (n=336) vs. severe stenosis (n=225) and according to location of intracranial stenosis (MCA, ICA, basilar, or vertebral). Results History of a lipid disorder (77% in severe vs. 67% in moderate, p=0.01), metabolic syndrome (63% in severe vs. 53% in moderate, p=0.05), and diabetes (43% in severe vs. 35% in moderate, p=0.04) were more common in patients with severe intracranial stenosis in univariate analyses. History of lipid disorder was independently associated with severe stenosis (OR 1.62 (95% CI 1.09–2.42), p=0.02). The distribution of stenosis location differed among age groups (p=0.0015), gender (p=0.0001), race (p=0.0243), qualifying event (p=0.0156), diabetes (p=0.0030), coronary artery disease (p=0.0030), and hyperlipidemia (p=0.054). Patients with basilar stenosis were older and more likely to have hyperlipidemia. Patients with MCA stenosis were more likely to be women and black. Patients with ICA stenosis were more likely to have diabetes. Patients with vertebral stenosis were more likely to have coronary artery disease. Conclusions Lipid disorder has the strongest association with severity of intracranial stenosis and should be the target of prevention therapies. Different locations of intracranial stenosis are associated with different vascular risk factors and demographic features, suggesting there may be a difference in the underlying pathophysiology of stenosis among the intracranial arteries. PMID:20616323

  17. Intracranial pial arteriovenous fistula.

    PubMed

    Yamashita, Kentaro; Ohe, Naoyuki; Yoshimura, Shin-ichi; Iwama, Toru

    2007-12-01

    A 33-year-old woman presented with a rare intracranial pial arteriovenous fistula manifesting as monoparesis and hypesthesia of the right lower extremity. Computed tomography demonstrated an approximately 10-mm diameter subcortical hematoma in the left postcentral gyrus. Two months after suffering the ictus, angiography demonstrated a pial arteriovenous fistula in the late arterial phase fed by the left paracentral artery and drained into the left precentral vein. No nidus or dural arteriovenous fistula was detected. Left parietal craniotomy was performed and the pial arteriovenous fistula was extirpated by electrocoagulation. Intraoperative angiography demonstrated disappearance of the fistula. She experienced no postoperative neurological deterioration, but hypesthesia of the right leg persisted. Obliteration of the pial arteriovenous fistula was reconfirmed by postoperative angiography. She suffered no rebleeding episodes during the 36-month follow-up period. Pial arteriovenous fistula causing mild symptoms should be treated by flow disconnection because the direct arteriovenous shunt and attendant high blood flow usually results in huge venous varices. To determine whether direct surgery or endovascular treatment is appropriate, the position and shape of the lesion must be known.

  18. Nonlocal Intracranial Cavity Extraction

    PubMed Central

    Manjón, José V.; Eskildsen, Simon F.; Coupé, Pierrick; Romero, José E.; Collins, D. Louis; Robles, Montserrat

    2014-01-01

    Automatic and accurate methods to estimate normalized regional brain volumes from MRI data are valuable tools which may help to obtain an objective diagnosis and followup of many neurological diseases. To estimate such regional brain volumes, the intracranial cavity volume (ICV) is often used for normalization. However, the high variability of brain shape and size due to normal intersubject variability, normal changes occurring over the lifespan, and abnormal changes due to disease makes the ICV estimation problem challenging. In this paper, we present a new approach to perform ICV extraction based on the use of a library of prelabeled brain images to capture the large variability of brain shapes. To this end, an improved nonlocal label fusion scheme based on BEaST technique is proposed to increase the accuracy of the ICV estimation. The proposed method is compared with recent state-of-the-art methods and the results demonstrate an improved performance both in terms of accuracy and reproducibility while maintaining a reduced computational burden. PMID:25328511

  19. Focal stenosis of the sigmoid sinus causing intracranial venous hypertension: Case report, endovascular management, and review of the literature.

    PubMed

    Honarmand, Amir R; Hurley, Michael C; Ansari, Sameer A; Alden, Tord D; Kuhn, Ryan; Shaibani, Ali

    2016-04-01

    Regardless of the underlying pathology, elevated intracranial pressure is the endpoint of any impairment in either cerebrospinal fluid (CSF) absorption (including arachnoid villi) or intracranial venous drainage. In all age groups, the predominant final common pathway for CSF drainage is the dural venous sinus system. Intracranial venous hypertension (ICVH) is an important vascular cause of intracranial hypertension (and its subsequent sequelae), which has often been ignored due to excessive attention to the arterial system and, specifically, arteriovenous shunts. Various anatomical and pathological entities have been described to cause ICVH. For the second time, we present a unique case of severe focal stenosis in the distal sigmoid sinus associated with concurrent hypoplasia of the contralateral transverse sinus causing a significant pressure gradient and intracranial hypertension, which was treated with endovascular stent placement and angioplasty.

  20. Vasospasm after intraventricular hemorrhage caused by arteriovenous malformation

    PubMed Central

    Tseng, Wei-Lung; Tsai, Yi-Hsin

    2015-01-01

    Intraventricular hemorrhage (IVH) induced vasospasm is rare in clinical practice. We report a case with vasospasm 10 days after IVH due to thalamic bleeding from an arteriovenous malformation. The vasospasm was so severe that transarterial embolization failed. Follow-up angiography could not reveal the vascular lesion and spontaneous thrombosis was considered. The discussion includes literature review and possible mechanism of such phenomenon. IVH-related vasospasm is rare but should always be kept in mind. Early detection with proper treatment provides better clinical outcome in such cases. PMID:25972942

  1. Radiation-induced large intracranial vessel occlusive vasculopathy

    SciTech Connect

    Brant-Zawadzki, M.; Anderson, M.; DeArmond, S.J.; conley, F.K.; Jahnke, R.W.

    1980-01-01

    Two patients who developed large intracranial vessel occlusion after standard radiation therapy for brain tumor are described. This form of vascular occlusion is usually seen in patients who have previously been treated by radiotherapy for intracranial tumor who then develop a relatively acute change in neurologic status. Histology of the lesion mimics accelerated focal arteriosclerosis. The clinical and radiographic manifestations of one case were highly atypical. The vasculopathy became evident shortly after termination of radiation therapy for a fourth ventricular ependymoma, and the angiographic picture stimulated a diffuse arteritis. The second patient was more typical, with clinical symptoms developing 12 years after radiation therapy for an oligodendroglioma. Occlusion of a proximal vessel that had been included in the radiation port was demonstrated radiographically and confirmed by pathologic examination. The clinical, angiographic, and histologic features of these two cases are discussed and previously reported cases are reviewed.

  2. Cavernous malformations of the central nervous system (CNS) in children: clinico-radiological features and management outcomes of 36 cases.

    PubMed

    Bilginer, Burcak; Narin, Firat; Hanalioglu, Sahin; Oguz, Kader Karlı; Soylemezoglu, Figen; Akalan, Nejat

    2014-08-01

    Cavernous malformations (CMs) of the central nervous system (CNS) are angiographically occult vascular lesions that affect approximately 0.5 % of the general population, and one quarter of all CMs occurs in children. We retrospectively analyzed demographic, clinical, radiological, management, and follow-up data of 36 pediatric patients with CMs from a single institution. The mean age of the children at first presentation and at operation was 8.7 and 9.6 years, respectively. However, a bimodal age distribution was found with peak under 4 years and above 12 years. Seizure was the most common single presenting symptom (38.9 %), and 61.1 % of patients had at least one seizure before the admission. Focal neurological deficits (410.7 %), intracranial hypertension (27.8 %), and headache (2.8 %) were the other manifestations. Acute/subacute hemorrhage was evident at presentation in 63.9 %. The patients under 6 years of age were found to have significantly more giant cavernomas (69 vs 20 %; p = 0.011), and more overt hemorrhages (81 vs 47 %; p = 0.065) at diagnosis than those patients above 12 years. Surgery was performed in 31 patients (32 CMs), with 26 total and 6 incomplete resections. Mean follow-up duration was 6.9 ± 4.1 years. Of all patients, 63.8 % had excellent and 30.5 % had good clinical outcomes, and also 90.9 % of the epileptic patients were seizure-free (Engel Class I) at the last follow-up. Younger children tend to harbor larger CMs and present with hemorrhage more frequently than older ones. Microsurgical resection should be the treatment of choice in symptomatic and accessible CMs.

  3. Vascular anomalies of the head and neck in children.

    PubMed

    Mahady, Kate; Thust, Stefanie; Berkeley, Rupert; Stuart, Sam; Barnacle, Alex; Robertson, Fergus; Mankad, Kshitij

    2015-12-01

    Sixty percent of vascular anomalies in children are found in the head and neck. These lesions can present throughout antenatal, perinatal and childhood development. They broadly fall into two categories: vascular tumours and vascular malformations. Their clinical and, often, psychological impact is determined by both pathological type and location: many lesions follow an uncomplicated natural course and other more complex, extensive or progressive lesions can present a threat to life from mass effect, haemorrhage or large volume arteriovenous shunting. Vascular tumours include infantile haemangioma (IH), congenital haemangioma (CH) and kaposiform hemangioendothelioma (KH); of which IH is the most common. Management options for vascular tumours include conservative approaches, oral medications and surgical intervention as determined by tumour type, location and associated complications. Vascular malformations can be categorised into low flow and high flow lesions. Low flow lesions include capillary, venous and lymphatic malformations (LMs). High flow lesions describe the arteriovenous malformations (AVMs), a highly heterogeneous group of lesions which can present in a variety of ways-the mainstay of treatment for these dynamic lesions is endovascular or surgical obliteration. We provide a practical framework for clinical classification of vascular anomalies of the head and neck in children. We also explore principles of their clinical and radiological assessment along with management, highlighting the importance of a multi-disciplinary approach.

  4. Bilateral Vocal Cord Palsy with Arnold Chiari Malformation: A Rare Case Series.

    PubMed

    Arora, Nikhil; Juneja, Ruchika; Meher, Ravi; Bhargava, Eishaan K

    2016-09-01

    Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition.

  5. Bilateral Vocal Cord Palsy with Arnold Chiari Malformation: A Rare Case Series

    PubMed Central

    Arora, Nikhil; Meher, Ravi; Bhargava, Eishaan K.

    2016-01-01

    Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition. PMID:27790480

  6. Guidelines for the treatment of head and neck venous malformations

    PubMed Central

    Zheng, Jia Wei; Mai, Hua Ming; Zhang, Ling; Wang, Yan An; Fan, Xin Dong; Su, Li Xin; Qin, Zhong Ping; Yang, Yao Wu; Jiang, Yin Hua; Zhao, Yi Fang; Suen, James Y

    2013-01-01

    Venous malformation is one of the most common benign vascular lesions, with approximately 40% of cases appearing in the head and neck. They can affect a patient’s appearance and functionality and even cause life-threatening bleeding or respiratory tract obstruction. The current methods of treatment include surgery, laser therapy, sclerotherapy, or a combined. The treatment of small and superficial venous malformations is relatively simple and effective; however, the treatment of deep and extensive lesions involving multiple anatomical sites remains a challenge for the physicians. For complex cases, the outcomes achieved with one single treatment approach are poor; therefore, individualized treatment modalities must be formulated based on the patient’s condition and the techniques available. Comprehensive multidisciplinary treatments have been adapted to achieve the most effective results. In this paper, based on the national and international literature, we formulated the treatment guidelines for head and neck venous malformations to standardize clinical practice. The guideline will be renewed and updated in a timely manner to reflect cutting-edge knowledge and to provide the best treatment modalities for patients. PMID:23724158

  7. Low-Flow Arterialized Venous Malformations of the Orbit.

    PubMed

    Callahan, Alison B; Meyers, Philip M; Garrity, James A; Son, Jung H; Petris, Carisa; Kazim, Michael

    Orbital vascular malformations are classified by their hemodynamic properties, either high or low flow. Low-flow lesions may be simple venous, lymphatic, or combined lymphaticovenous malformations. The authors report a series of cases in which predominantly low flow, venous lesions were unexpectedly noted to have arterial feeders. A retrospective chart review of patients identified by the authors as having orbital varices with arterial components was conducted. The authors identified 7 such cases. After careful review, 2 cases were excluded due to inconclusive neuroradiographic findings. The authors review the clinical, radiologic, histopathologic, and surgical information from the remaining 5 cases and discuss their clinical significance. All 5 cases were most consistent with variceal lesions: 3 as clinically distensible lesions and 2 as thrombosed lesions. Additional arterial feeder vessels were noted by angiography (3) or intraoperative visualization (2). The arterial contribution varied from faint vessels to distinct branches of the ophthalmic artery. Ages ranged from 13 to 61 years without predilection for gender. Treatments consisted of excision, embolization, and observation. Two poignant cases are highlighted: the first illustrating that an angiogram in isolation of its clinical picture can be misleading and result in treatment intervention with undue risk, and the second illustrating that inadequate treatment of unrecognized arterial components may contribute to recurrences. Low-flow orbital variceal lesions may have less prominent, arterial components. This type of combined arterialized venous malformation is largely unrecognized in the ophthalmic literature. Correct identification of these lesions is critical in providing safe, effective, and durable treatment.

  8. Development of an intracranial dural arteriovenous fistula after venous sinus stenting for idiopathic intracranial hypertension.

    PubMed

    Buell, Thomas J; Raper, Daniel M; Ding, Dale; Chen, Ching-Jen; Liu, Kenneth C

    2017-09-26

    We report a case in which an intracranial dural arteriovenous fistula (DAVF) developed after endovascular treatment of a patient with idiopathic intracranial hypertension with venous sinus stenting (VSS). The pathogenesis may involve hemodynamic alterations secondary to increased poststenting venous sinus pressure, which may cause new arterial ingrowth into the fistulous sinus wall without capillary interposition. Despite administration of dual antiplatelet therapy, there may also be subclinical cortical vein thrombosis that contributed to DAVF formation. In addition to the aforementioned mechanisms, increased inflammation induced by VSS may upregulate vascular endothelial growth factor and platelet-derived growth factor expression and also promote DAVF pathogenesis. Since VSS has been used to obliterate DAVFs, DAVF formation after VSS may seem counterintuitive. Previous stents have generally been closed cell, stainless steel designs used to maximize radial compression of the fistulous sinus wall. In contrast, our patient's stent was an open cell, self-expandable nitinol design (Protégé Everflex). Neurointerventionalists should be aware of this potential, although rare complication of DAVF formation after VSS. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  9. Prenatal diagnosis of cloacal malformation.

    PubMed

    Peiro, Jose L; Scorletti, Federico; Sbragia, Lourenco

    2016-04-01

    Persistent cloaca malformation is the most severe type of anorectal and urogenital malformation. Decisions concerning the surgical treatment for this condition are taken during the first hours of life and may determine the quality of life of these patients. Thus, prenatal diagnosis becomes important for a prompt and efficient management of the fetus and newborn, and accurate counseling of the parents regarding its consequences and the future of the baby. Careful evaluation by ultrasonography, and further in-depth analysis with MRI, allow prenatal detection of characteristic findings, which can lead to diagnose or at least suspect this condition. We reviewed our experience and the literature in order to highlight the most important clues that can guide the physician in the differential diagnosis. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. [Congenital malformations: care or predict?].

    PubMed

    Pellerin, D

    1993-02-01

    Spectacular scientific and technological advances made in the last decade have had such a profound impact on biological and medical science that they have dramatically modified the citizen's behaviour concerning life events, especially congenital malformation. Prenatal diagnosis (PND) leads to do the diagnosis of almost all fetal internal and external malformations. The matter is, not only to care, but, first to know. The positive efficiency of PND is sometimes preparing the best cares and, of course, to recognize many severe anomalies postnatally diagnosed before PND time, and carrying wellknown 50% rate mortality by neo-natal surgery. Congenital diaphragmatic hernia is pointed out as a good example of it, and of hopes and disappointing in utero foetal surgery. New protocol of assessment of fetal renal function is an appreciated method to do prognosis of some fetal uropathies before late in utero drainage, for a short time waiting for necessary maturation of lungs allowing premature delivery. The possibility to do PND of small and benign malformation leads to ask for the question of utility of to know. In spite of the respect of quality of life, can we really allow this type of human selection to be made? The next knowledges of the human genoma map bring us into the predictive medicine. Using "compulsory" PND is a real risk to practice dangerously, a soft eugenism. PND must be, and remain an outstanding advance to provide better treatment.

  11. Arteriovenous malformation in the retina of a monkey.

    PubMed

    Horiuchi, T; Gass, D M; David, N J

    1976-12-01

    A vascular abnormality of the retina of a rhesus monkey was studied with fluorescein angiography, microvascular examination after silicone rubber injection, and histological examination. Fluorescein angiography revealed that this abnormality was an arteriovenous shunt. Microvascular examination showed a vascular abnormality on the sclera and an end-to-end communication of the arteriovenous malformation that was a continuation of abnormally large central retinal vessels observed just before their insertion into the optic nerve in the orbit. Histologic examination proved that the arteriovenous shunt originated from the central retinal vessels in the orbit; that degeneration of the retina and the choroid was extensive near the abnormal vessels; that the abnormal vessels had normal endothelium and adventitia but remarkably widened media; and that the cavernous hamangioma-like structure in the optic disk was clearly distinguishable from a cavernous hamangioma.

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

    SciTech Connect

    Cil, Barbaros E. E-mail: barbaros@hacettepe.edu.tr; Erdogan, Cueneyt; Akmangit, Ilkay; Cekirge, Saruhan; Balkanci, Ferhun

    2004-11-15

    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 coil migration during the transcatheter occlusion of PAVMs.

  13. Coil Embolotherapy of Unilateral Diffuse Pulmonary Arteriovenous Malformations in a Nineteen-Year-Old Woman

    PubMed Central

    Rokni Yazdi, Hadi; Abtahi, Hamidreza; Saberi, Hazhir; Salahi, Mona

    2015-01-01

    Pulmonary arteriovenous malformations (AVMs) are rare vascular malformations of the lung that usually led to a notable risk of serious and life-threatening complications. There is considerable debate about the best management of strategies for the group of patients with diffuse AVMs. Several therapeutic options have been reported for management of this abnormality among which coil embolization is currently the preferred ones. This report describes our experience with the use of coiling method for treatment of multiple AVMs in an adult patient. PMID:26528389

  14. Intracranial calcification in central diabetes insipidus.

    PubMed

    Al-Kandari, Salwa Ramadan; Pandey, Tarun; Badawi, Mona H

    2008-01-01

    Intracranial calcification is a known but extremely rare complication of diabetes insipidus. To date, only 16 patients have been reported and all had the peripheral (nephrogenic) type of diabetes insipidus. We report a child with intracranial calcification complicating central diabetes insipidus. We also report a child with nephrogenic diabetes insipidus, and compare the patterns of intracranial calcification.

  15. Alternating Skew Deviation from Traumatic Intracranial Hypotension

    PubMed Central

    Moster, Stephen J.; Moster, Mark L.

    2014-01-01

    Abstract A 56-year-old woman developed progressive headache, mental status changes, and diplopia after trauma. She was diagnosed with alternating skew deviation caused by intracranial hypotension. This is the first case of alternating skew deviation reported from intracranial hypotension and perhaps a differential pressure between intracranial and intraspinal spaces plays a role in the development of these findings. PMID:27928294

  16. Fetal intracranial teratoma. A review.

    PubMed

    Isaacs, Hart

    2014-01-01

    A literature and institutional review of fetal intracranial teratomas yielded 90 tumors. The mean age at ultrasound diagnosis was 32 weeks, ranging from 21 to 41 weeks. Males and females were equally affected. The average, maximum tumor size was 10 cm, varying between 3.5 and 23 cm. Forty-two percent of patients died within the first week of life. Death rate was exceptionally high before 30 weeks gestation where almost half the affected fetuses expired. The overall survival rate for 90 fetuses with intracranial teratoma was only 7.8%.

  17. Surgical management of scalp arterio-venous malformation and scalp venous malformation: An experience of eleven cases

    PubMed Central

    Chowdhury, Forhad Hossain; Haque, Mohammod Raziul; Kawsar, Khandkar Ali; Sarker, Mainul Haque; Momtazul Haque, A. F. M.

    2013-01-01

    Aims: Scalp arterio-venous malformation (AVM) and scalp venous malformation (SVM) are rare conditions that usually need surgical treatment. Here, we have reported our experience of the surgical management of such lesions with a short review of the literature. Materials and Methods: In this prospective study, 11 patients with scalp AVM and SVM, who underwent surgical excision of lesion in our hospital from 2006 to 2012, were included. All suspected high-flow AVM were investigated with the selective internal and external carotid digital subtraction angiogram (DSA) ± computed tomography (CT) scan of brain with CT angiogram or magnetic resonance imaging (MRI) of brain with MR angiogram, and all suspected low-flow vascular malformation (VM) was investigated with MRI of brain + MR angiogram. Eight were high-flow and three were low-flow VM. Results: All lesions were successfully excised. Scalp cosmetic aspects were acceptable in all cases. There was no major post-operative complication or recurrence till last follow-up. Conclusions: With preoperative appropriate surgical planning, scalp AVM and SVM can be excised without major complication. PMID:23960313

  18. [Progress in diagnosis and treatment of intracranial hypertension and hydrocephalus in children with intracranial infections].

    PubMed

    He, Fang; Peng, Jing; Yin, Fei

    2015-06-01

    Intracranial infections are one of the most common neurological diseases in children and are associated with high mortality and morbidity. Intracranial hypertension and hydrocephalus are the common, fatal complications of intracranial infections, so early diagnosis and timely treatment are the keys to saving patients' lives and reducing neurological sequelae. This paper introduces the progress in the etiology, diagnosis, and treatment of intracranial hypertension and hydrocephalus in children with intracranial infections.

  19. Contemporary vascular smartphone medical applications.

    PubMed

    Carter, Thomas; O'Neill, Stephen; Johns, Neil; Brady, Richard R W

    2013-08-01

    Use of smartphones and medical mHealth applications (apps) within the clinical environment provides a potential means for delivering elements of vascular care. This article reviews the contemporary availability of apps specifically themed to major vascular diseases and the opportunities and concerns regarding their integration into practice. Smartphone apps relating to major vascular diseases were identified from the app stores for the 6 most popular smartphone platforms, including iPhone, Android, Blackberry, Nokia, Windows, and Samsung. Search terms included peripheral artery (arterial) disease, varicose veins, aortic aneurysm, carotid artery disease, amputation, ulcers, hyperhydrosis, thoracic outlet syndrome, vascular malformation, and lymphatic disorders. Forty-nine vascular-themed apps were identified. Sixteen (33%) were free of charge. Fifteen apps (31%) had customer satisfaction ratings, but only 3 (6%) had greater than 100. Only 13 apps (27%) had documented medical professional involvement in their design or content. The integration of apps into the delivery of care has the potential to benefit vascular health care workers and patients. However, high-quality apps designed by clinicians with vascular expertise are currently lacking and represent an area of concern in the mHealth market. Improvement in the quality and reliability of these apps will require the development of robust regulation. Copyright © 2013 Elsevier Inc. All rights reserved.

  20. The Horizon for Treating Cutaneous Vascular Lesions

    PubMed Central

    Patel, Amit M.; Chou, Elizabeth L.; Findeiss, Laura; Kelly, Kristen M.

    2013-01-01

    Dermatologists encounter a wide range of cutaneous vascular lesions, including infantile hemangiomas, port-wine stain birthmarks, arteriovenous malformations, venous malformations, Kaposi sarcomas, angiosarcomas, and angiofibromas. Current treatment modalities to reduce these lesions include topical and/or intralesional steroids, laser therapy, surgical resection, and endovascular therapy. However, each method has limitations owing to recurrence, comorbidities, toxicity, or lesion location. Photodynamic therapy, antiangiogenic therapy, and evolving methods of sclerotherapy are promising areas of development that may mitigate limitations of current treatments and offer exciting options for patients and their physicians. PMID:22640429

  1. The horizon for treating cutaneous vascular lesions.

    PubMed

    Patel, Amit M; Chou, Elizabeth L; Findeiss, Laura; Kelly, Kristen M

    2012-06-01

    Dermatologists encounter a wide range of cutaneous vascular lesions, including infantile hemangiomas, port-wine stain birthmarks, arteriovenous malformations, venous malformations, Kaposi sarcomas, angiosarcomas, and angiofibromas. Current treatment modalities to reduce these lesions include topical and/or intralesional steroids, laser therapy, surgical resection, and endovascular therapy. However, each method has limitations owing to recurrence, comorbidities, toxicity, or lesion location. Photodynamic therapy, antiangiogenic therapy, and evolving methods of sclerotherapy are promising areas of development that may mitigate limitations of current treatments and offer exciting options for patients and their physicians.

  2. Computed tomographic spectrum of intracranial mycosis: correlation with histopathology

    SciTech Connect

    Whelan, M.A.; Stern, J.; deNapoli, R.A.

    1981-12-01

    Four cases of intracerebral fungal infection are reviewed. The clinical course is outlined, and the computed tomographic (CT) characteristics are analyzed in light of known pathological data. The CT appearance of intracranial mycosis is dependent on the type of fungus as well as the dominant infecting form, i.e., yeast or hyphae. The hyphal form leads predominantly to a CT pattern consistent with vascular occlusion and secondary abscess formation; the yeast form generally results in noncaseating granulomas, which appear on CT scan as nodular enhancing lesions. If the patient survives the acute infective process, these fungal lesions undergo a prolonged subacute phase, and may eventually calcify.

  3. Cervicofacial Lymphatic Malformations: A Retrospective Review of 40 Cases

    PubMed Central

    Cho, Byung Chae; Kim, Jae Bong; Lee, Jeong Woo; Choi, Kang Young; Yang, Jung Dug; Lee, Seok-Jong; Kim, Yong-Sun; Lee, Jong Min; Huh, Seung

    2016-01-01

    Background Lymphatic malformation (LM) is a form of congenital vascular malformation with a low incidence. Although LM has been studied, no consensus has emerged regarding its cause or treatment. Methods In this study, we retrospectively evaluated 40 patients who visited our vascular anomalies center for the treatment of cervicofacial LM, which is a common manifestation of LM. The medical records of patients over a period of 12 years were reviewed and analyzed for commonalities regarding the diagnosis and the results of treatment. Results Suspected cervicofacial LM was confirmed through imaging studies. No difference in incidence was observed according to sex, and 73% of patients first presented with symptoms before the age of two years. The left side and the V2–V3 area were most commonly affected. No significant differences in incidence were observed among the macrocystic, microcystic, and combined types of LM. A total of 28 out of 36 patients received sclerotherapy as the first choice of treatment, regardless of the type of lesion. Complete resolution was achieved in only 25% of patients. Conclusions LM is important to confirm the diagnosis early and to choose an appropriate treatment strategy according to the stage of the disease and each individual patient's symptoms. When treatment is delayed or an incorrect treatment is administered, patient discomfort increases as the lesion gradually spreads. Therefore, more so than is the case for most other diseases, a team approach on a case-by-case basis is important for the accurate and appropriate treatment of LM. PMID:26848440

  4. Establishment of the intracranial hemodynamic model based on contrast medium and clinical applications

    PubMed Central

    Cheng, Yaoer; He, Wen

    2016-01-01

    Abstract Ischemic cerebrovascular diseases are one of the most common vascular diseases in aged people and CT perfusion (CTP) is a very popular tool to detect the ischemic changes in brain vascular. The present study aims to establish a novel intracranial hemodynamic model to simulate anterior cerebral artery blood flow, and compare the actual and simulated hemodynamic parameters of healthy people and patients with carotid stenosis or occlusion. A mathematical model of the intracranial hemodynamic was generated using MATLAB software, and data from patients with or without infarct disease (57 and 44 cases, respectively) were retrospectively collected to test the new model. The actual time-density curve (TDC) of anterior cerebral artery was obtained from the original intracranial CTP data, and simulated TDC was calculated from our intracranial hemodynamic model. All model parameters were adjusted according to patients’ sex, height, and weight. Time to peak enhancement (TTP), maximum enhancement (ME), and mean transit time (MTT) were selected to evaluate the status of hemodynamics. In healthy people, there were no significant differences of TTP and ME between actual and simulated curves. For patients with infarct symptoms, ME was significantly decreased in actual data compared with simulated curve, while there was no obvious difference of TTP between actual and simulated data. Moreover, MTT was delayed in infarct patients compared with healthy people. Our group generated a computer-based, physiologic model to simulate intracranial hemodynamics. The model successfully simulated anterior cerebral artery hemodynamics in normal healthy people and showed noncompliant ME and MTT in infarct patients, reflecting their abnormal cerebral hemodynamic status. The digital model is reliable and may help optimize the protocol of contrast medium enhancement in intracranial CT, and provide a solid tool to study intracranial hemodynamics. PMID:27930555

  5. Establishment of the intracranial hemodynamic model based on contrast medium and clinical applications.

    PubMed

    Cheng, Yaoer; He, Wen

    2016-12-01

    Ischemic cerebrovascular diseases are one of the most common vascular diseases in aged people and CT perfusion (CTP) is a very popular tool to detect the ischemic changes in brain vascular. The present study aims to establish a novel intracranial hemodynamic model to simulate anterior cerebral artery blood flow, and compare the actual and simulated hemodynamic parameters of healthy people and patients with carotid stenosis or occlusion.A mathematical model of the intracranial hemodynamic was generated using MATLAB software, and data from patients with or without infarct disease (57 and 44 cases, respectively) were retrospectively collected to test the new model. The actual time-density curve (TDC) of anterior cerebral artery was obtained from the original intracranial CTP data, and simulated TDC was calculated from our intracranial hemodynamic model. All model parameters were adjusted according to patients' sex, height, and weight. Time to peak enhancement (TTP), maximum enhancement (ME), and mean transit time (MTT) were selected to evaluate the status of hemodynamics.In healthy people, there were no significant differences of TTP and ME between actual and simulated curves. For patients with infarct symptoms, ME was significantly decreased in actual data compared with simulated curve, while there was no obvious difference of TTP between actual and simulated data. Moreover, MTT was delayed in infarct patients compared with healthy people.Our group generated a computer-based, physiologic model to simulate intracranial hemodynamics. The model successfully simulated anterior cerebral artery hemodynamics in normal healthy people and showed noncompliant ME and MTT in infarct patients, reflecting their abnormal cerebral hemodynamic status. The digital model is reliable and may help optimize the protocol of contrast medium enhancement in intracranial CT, and provide a solid tool to study intracranial hemodynamics.

  6. Management of patients with brain arteriovenous malformations.

    PubMed

    Söderman, Michael; Andersson, Tommy; Karlsson, Bengt; Wallace, M Christopher; Edner, Göran

    2003-06-01

    Arteriovenous malformations (AVMs) of the brain, which are probably genetically determined, are errors in the development of the vasculature that, together with the effects of blood flow, may lead to a focal arteriovenous shunt. Clinically, the adult patient may present with acute or chronic neurological symptoms-fixed or unstable-such as deficits, seizures or headache. Sometimes the lesion is an incidental finding. In about half of the patients, the revealing event is an intracranial haemorrhage. The prevalence of AVM in the western world is probably <0.01% and the detection rate is about one per 100,000 person-years. Most AVMs are revealed in patients 20-40 years of age. Therefore, the risk of developing neurological symptoms from an AVM, usually because of haemorrhage, increases with patient age. In the young adult population, AVMs are significant risk factors for hemorrhagic stroke. This risk increases with AVM volume and is higher in centrally located AVMs. Almost all patients with AVM are subjected to treatment, either by surgery, radiosurgery or embolisation, with the functional aim of reducing the risk of haemorrhage or to alleviate neurological symptoms with an acceptable treatment risk. Few neurocentres have physicians highly skilled in all treatment modalities. Therefore, the prescribed treatment may not be defined from an objective assessment of what is optimal for each individual patient, but rather from local expertise. In this context, more and better data about the natural history and the outcome of different treatments, as well as predictive models, would be valuable to help to optimise the management. Management strategies obviously differ according to local preferences, but results presented in the literature suggest the following strategy: (I) cortically located AVMs with a nidus volume <10 ml could be operated, with or without presurgical embolisation, unless there is a single feeder that can easily be catheterised and embolised for

  7. Amphetamine abuse and intracranial haemorrhage.

    PubMed Central

    Buxton, N; McConachie, N S

    2000-01-01

    Amphetamines taken by any route can cause cerebral vasculitis and intracranial haemorrhage. 8 cases were seen in a neurosurgical unit over 3.5 years. The published work indicates that those who experience these complications, mainly young adults, have poor outcomes. PMID:11089483

  8. [Multiple intracranial tuberculomas in infancy].

    PubMed

    Serrano, M; Campistol, J; Chávez, B; Caritg, J; Fortuny, C; Costa, J M

    Tuberculous involvement of the CNS is most frequent in children aged between 6 months and 6 years, although it may occur at any age. It may present as meningoencephalitis, basal arachnoiditis or intracranial tuberculomas. Whilst meningitis is typical of infancy, tuberculomas and arachnoiditis are commoner in adults. It has been estimated that tuberculomas make up 3% of the cases of neurotuberculosis. The increasing use of CAT and MR has been a great help for diagnosis of this serious complication of tuberculosis. A 5 month old patient presented with tuberculous meningitis which had been treated with streptomycin, isoniazid, pyrazinamide and rifampicin at the usual dosage. One month later, after good initial progress, triventricular hydrocephaly was diagnosed and a ventriculoperitoneal shunt inserted. Three months after this, there was an episode of intracranial hypertension. Cranial CAT showed considerable zones of hypodense parenchyma without ventricle dilatation. On MR there were multiple, disseminated, rounded areas which were hyperintense on T2 and compatible with intracranial tuberculomas. After fresh insertion of a ventricular shunt, the patient progressed but still had a residual right hemiparesia and retarded development. Although intracranial tuberculomas usually occur in adults, they may be seen in children following meningoencephalitis. Occasionally, following a good initial response to tuberculostatic drugs, tuberculomas appear, although not present before, as happened in our patient. This usually occurs within the first three months, and although the mechanism is unknown, it is believed to be due to the accumulation of lymphocytes and macrophages at preexisting microscopic foci when treatment is started.

  9. Intracranial tuberculoma mimicking brain metastasis.

    PubMed

    Salaskar, Abhijit L; Hassaneen, Wael; Keenan, Cheryl H; Suki, Dima

    2015-01-01

    To our knowledge, this is the first report of an intracranial tuberculoma in an immunocompetent patient with a solid primary tumor outside the central nervous system. This case is important because the patient underwent treatment for a presumed brain metastasis, based on the knowledge that a solid extracranial primary tumor was present, but before the brain lesion pathology was determined.

  10. Imaging findings of vascular lesions in the head and neck

    PubMed Central

    Güneyli, Serkan; Ceylan, Naim; Bayraktaroğlu, Selen; Acar, Türker; Savaş, Recep

    2014-01-01

    Vascular lesions of the head and neck include vascular neoplasms, vascular malformations, and hypervascular lesions, derived from nonvascular soft-tissue elements. We retrospectively evaluated magnetic resonance imaging and computed tomography images of vascular lesions located in the head and neck. Twelve patients (seven males, five females) aged 1–68 years (mean age, 35.25 years) were included in this study. Most of the vascular lesions in our study were histologically diagnosed. The lesions were as follows: a hemangioma located in the parotid space (n=1); a hemangioendothelioma located in the parotid space (n=1); a hemangiopericytoma located in the larynx (n=1); a juvenile angiofibroma located in the nasopharynx (n=1); a glomus tumor located in the carotid bifurcation (n=1); venous malformations located in the parapharyngeal space, the pterygoid area, the orbital space, and the larynx (n=4); lymphatic malformations located in the parotid space and the supraclavicular area (n=2); and an arteriovenous malformation located in the infratemporal fossa (n=1). We present rare vascular lesions of the head and neck, which have typical radiological findings. PMID:25010372

  11. An Unusual Case of Post-Traumatic Headache Complicated by Intracranial Hypotension

    PubMed Central

    Siavoshi, Sara; Dougherty, Carrie; Ailani, Jessica; Yadwadkar, Kaustubh; Berkowitz, Frank

    2016-01-01

    We present a case of post-traumatic headache complicated by intracranial hypotension resulting in an acquired Chiari malformation and myelopathy with syringomyelia. This constellation of findings suggest a possible series of events that started with a traumatic cerebral spinal fluid (CSF) leak, followed by descent of the cerebellar tonsils and disruption of CSF circulation that caused spinal cord swelling and syrinx. This unusual presentation of post-traumatic headache highlights the varying presentations and the potential sequelae of intracranial hypotension. In addition, the delayed onset of upper motor neuron symptoms along with initially normal head computerized tomography scan (CT) findings, beg the question of whether or not a post-traumatic headache warrants earlier magnetic resonance imaging (MRI). PMID:28036062

  12. [Effects of xenon anesthesia on cerebral blood flow in neurosurgical patients without intracranial hypertension].

    PubMed

    Rylova, A V; Beliaev, A Iu; Lubnin, A Iu

    2013-01-01

    Among anesthetic agents used in neurosurgery xenon appears to be the most advantageous. It preserves arterial blood pressure, assures rapid recovery and neuroprotection. But the data is lacking on xenon effect upon cerebral blood flow under anesthetic conditions. We measured flow velocity in middle cerebral artery in neurosurgical patients without intracranial hypertension during closed circuit xenon anesthesia comparing propofol and xenon effect in the same patients. In our study xenon didn't seem to induce clinically relevant changes in cerebral blood flow and preserved cerebral vascular reactivity thus proving its safety in patients without intracranial hypertension.

  13. Diverse imaging characteristics of a mandibular intraosseous vascular lesion.

    PubMed

    Handa, Hina; Naidu, Giridhar S; Dara, Balaji Gandhi Babu; Deshpande, Ashwini; Raghavendra, Raju

    2014-03-01

    Intraosseous vascular lesions of the maxillofacial region are rare, and the differential diagnosis of intraosseous vascular malformations from other jaw lesions can be challenging. In the present case, magnetic resonance imaging and three-dimensional computed tomographic angiography (CTA) was used for diagnosis, and the lesion was treated wih surgical excision. Diverse characteristics such as the "honeycomb" and "sunburst" radiographic appearances and the absence of major peripheral feeder vessels in the CTA were noted. Intraosseous vascular malformations have a varied radiographic appearance, and the nomenclature of these lesions is equally diverse, with several overlapping terms. Pathologists do not generally differentiate among intraosseous vascular lesions on the basis of histopathology, although these lesions may present with contrasting immunohistochemical and clinical behaviors requiring varied treatment strategies. This case report highlights the need for multiple imaging modalities to differentiate among vascular lesions, as well as to better understand the behaviors of these unique lesions.

  14. Vein of Galen malformation presenting as persistent pulmonary hypertension of newborn (PPHN)

    PubMed Central

    Tiwary, Sangeeta; Geethanath, Ruppa Mohanram; Abu-Harb, Majd

    2013-01-01

    Arteriovenous malformation is a recognised cause of persistent pulmonary hypertension in a newborn (PPHN). Vein of Galen malformation (VOGM) is a rare vascular malformation which can be life-threatening if not diagnosed and treated early. We describe a case of a term baby who presented at day 4 of life with PPHN secondary to VOGM. The neonate underwent two transarterial interventional embolisation procedures on day 9 and then another one due to developing ventricular dilation on day 44. He remains stable since and was doing well at clinical review at 10 weeks and 4 months of age. VOGM usually presents in the neonatal period with high-output cardiac failure. In a baby who presents atypically with pulmonary hypertension, a cranial ultrasound scan should be considered to look for extracardiac shunting in the brain, especially, VOGM. PMID:24072831

  15. The Vanishing Twin Syndrome: Two Cases of Extreme Malformations Associated With Vanished Twins.

    PubMed

    Shinnick, Julia K; Khoshnam, Nasim; Archer, Sydney R; Quigley, Philip C; Robinson, Haynes; Keene, Sarah; Santore, Matthew T; Hill, Sarah; Patel, Binita; Shehata, Bahig M

    2017-01-01

    Two cases of devastating fetal malformations associated with vanished monochorionic twins were identified upon review of pathology files. A 35-year-old G1P0 woman and 36-year-old G3P1 woman were both diagnosed with an intrauterine twin gestation via transvaginal ultrasound at 10 weeks. The spectrum of fetal anomalies ranged from omphalocele, bilateral upper extremity, and unilateral lower extremity hypoplasia, to craniofacial malformation with diaphragmatic hernia. On histopathologic examination, the placentas demonstrated vascular anastomoses between the surviving co-twin and the "vanished" fetal sac. We propose anastomotic placental vasculature as a contributing factor to the observed fetal malformations. Additionally, genetic or teratogenic factors may have been attributed to the demise of the first twin and the anomalies seen in the other twin. While such instances are rare, they are important to consider when counseling patients regarding outcomes associated with a monochorionic vanished twin.

  16. Outcomes of Surgical Treatment of Vascular Anomalies on the Vermilion

    PubMed Central

    Park, Sang Min; Lee, Jae Woo; Kim, Hoon Soo; Lee, In Sook

    2016-01-01

    Background The vermilion plays an important role in both the aesthetic and functional aspects of facial anatomy. Due to its structural features, the complete excision of vascular anomalies on the vermilion is challenging, making it difficult to determine the appropriate treatment strategy. Thus, the authors analyzed the results of surgical treatment of vascular anomalies on the vermilion. Methods The medical records of 38 patients with vascular anomalies on the vermilion who underwent surgery from 1995 to 2013 were analyzed. Nine of the cases had an involuted hemangioma, and 29 cases had a vascular malformation; of the vascular malformations, 13, 11, one, and four cases involved were capillary malformations (CMs), venous malformations (VMs), lymphatic malformations (LMs), and arteriovenous malformations (AVMs), respectively. We investigated the surgical methods used to treat these patients, the quantity of surgical procedures, complications and instances of recurrence, and self-assessed satisfaction scores. Results A total of 50 operations were carried out: 28 horizontal partial excisions, eight vertical partial excisions, and 14 operations using other surgical methods. All cases of AVM underwent complete excision. Six cases experienced minor complications and one case of recurrence was observed. The overall average satisfaction score was 4.1 out of 5, while the satisfaction scores associated with each lesion type were 4.2 for hemangiomas, 3.9 for CMs, 4.2 for VMs, 5.0 for LMs, and 4.0 for AVMs. Conclusions It is difficult to completely excise vascular anomalies that involve the vermilion. This study suggests that partial excision focused on correcting the overall contour of the lips is effective and leads to satisfactory results. PMID:26848441

  17. Vascular Diseases

    MedlinePlus

    The vascular system is the body's network of blood vessels. It includes the arteries, veins and capillaries that carry ... to and from the heart. Problems of the vascular system are common and can be serious. Arteries ...

  18. 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.

  19. The “seagull cry” in a patient with a cerebral arteriovenous malformation

    PubMed Central

    Morelli, N.; Rota, E.; Michieletti, E.; Guidetti, D.

    2012-01-01

    Introduction The “seagull cry” is an acoustic phenomenon heard during duplex ultrasound. It is caused by harmonic covibrations of a vessel wall in the presence of high-velocity blood flow. It has been reported in a few cases of cerebrovascular disease, such as severe intracranial stenosis, vasospasm or carotid-cavernous fistula. Material and methods A 35-year-old man underwent transcranial color-coded sonography (TCCS) for work-up of a severe new-onset headache. Results Doppler spectral analysis of the right intracranial carotid bifurcation revealed multiple pairs of mirror-image parallel strings, and a high-frequency seagull cry was heard. Computed tomography-angiography and magnetic resonance imaging of the brain showed an arteriovenous malformation in the right temporal lobe. Discussion The seagull cry is a “musical murmur” with single or multiple frequency that sounds like a musical tone. This is the first report of this phenomenon in a cerebral arteriovenous malformation. PMID:23459331

  20. Vascular tumours in infants. Part I: benign vascular tumours other than infantile haemangioma.

    PubMed

    Hoeger, P H; Colmenero, I

    2014-09-01

    Vascular anomalies can be subdivided into vascular tumours and vascular malformations (VMs). While most VMs are present at birth and do not exhibit significant postnatal growth, vascular tumours are characterized by their dynamics of growth and (sometimes) spontaneous regression. This review focuses on benign vascular tumours other than infantile haemangiomas (IHs), namely pyogenic granuloma, eruptive pseudoangiomatosis, glomangioma, rapidly involuting and noninvoluting congenital haemangioma, verrucous haemangioma and spindle cell haemangioma. While some of them bear clinical resemblance to IH, they can be separated by age of appearance, growth characteristics and/or negative staining for glucose transporter 1. Separation of these tumours from IH is necessary because their outcome and therapeutic options are different. Semimalignant and malignant vascular tumours will be addressed in a separate review.