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Sample records for vein vascular malformation

  1. Pulmonary vascular malformations.

    PubMed

    Liechty, Kenneth W; Flake, Alan W

    2008-02-01

    Pulmonary vascular malformations have historically been diagnosed in a wide range of age groups, but the extensive use of prenatal imaging studies has resulted in the majority of lesions being diagnosed in utero. Among this group of lesions, bronchopulmonary sequestrations (BPS), hybrid lesions with both congenital cystic adenomatoid malformation (CCAM) and BPS, aberrant systemic vascular anastomoses, and pulmonary arteriovenous malformations (PAVM), are the most common. The biologic behavior of these lesions and the subsequent therapy is, in large part, determined by the age of the patient at diagnosis. In the fetus, large BPS or hybrid lesions can result in fetal hydrops and in utero fetal demise. In the perinatal period, pulmonary hypoplasia from the mass effect or air trapping within the cystic component of hybrid lesions can result in life-threatening respiratory distress. In the postnatal period, communication of the lesion with the aero-digestive system can result in recurrent pneumonia. Alternatively, increased pulmonary blood flow from the systemic arterial supply can result in hemorrhage, hemoptysis, or high output cardiac failure. In addition, there have been several reports of malignant degeneration. Finally, the broad spectrum encompassed by these lesions makes classification and subsequent communication of the lesions confusing and difficult. This paper will review the components of these lesions, their associated anomalies, the diagnosis and natural history, and finally, current concepts in the management of pulmonary vascular malformations. PMID:18158137

  2. 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. PMID:21633582

  3. Systemic to pulmonary vascular malformation.

    PubMed

    Pouwels, H M; Janevski, B K; Penn, O C; Sie, H T; ten Velde, G P

    1992-11-01

    A case is reported of life-threatening haemoptysis as a result of an anomalous communication between a bronchial artery and pulmonary vein, demonstrated by angiography. The patient recovered following bilobectomy of the right lower and middle lobes. When a systemic artery is involved in an arteriovenous malformation of the lung, haemodynamics are different compared with those present in malformations fed by the pulmonary artery. This implicates other clinical features, options for surgical intervention and prognosis. In reviewing the literature, a relationship with Rendu-Osler-Weber disease is absent in these specific malformations. PMID:1486979

  4. Overgrowth syndromes with vascular malformations.

    PubMed

    Hagen, Solveig L; Hook, Kristen P

    2016-03-01

    This review provides a clinically-oriented summary of the most commonly encountered overgrowth syndromes associated with vascular malformations. This manuscript will outline morphologic features, clinical evaluation and management of this complex group of patients. Recent genetic advances have aided in classification and help to explain overlapping clinical features in many cases. PMID:27607325

  5. 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.

  6. Spontaneous thrombosis of a vein of galen malformation.

    PubMed

    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

  7. 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

  8. Megadolicho vascular malformation of the intracranial arteries.

    PubMed

    Lodder, J; Janevski, B; van der Lugt, P J

    1981-01-01

    A patient is presented suffering a hemiparesis. Megadolicho-vascular malformation of the intracranial part of the internal carotid arteries and some of its branches and of the basilar artery was suggested by CT and confirmed by angiography. The value of CT compared with angiography in relation to intracranial megadolicho vascular malformations is discussed. PMID:6273040

  9. 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

  10. Lymphangiosarcoma complicating extensive congenital mixed vascular malformations.

    PubMed

    Al Dhaybi, Rola; Agoumi, Mehdi; Powell, Julie; Dubois, Josée; Kokta, Victor

    2010-09-01

    Pediatric hepatic angiosarcoma is a very rare malignant vascular tumor. A few cases have shown pediatric hepatic angiosarcoma occurring on a background of preexisting vascular lesions. We report the case of a newborn girl who presented extensive limbs and upper trunk cutaneous mixed vascular malformations at birth. These malformations were associated with thrombocytopenia. Cutaneous biopsies revealed complex vascular malformations with a significant lymphatic component. Compressive body suit therapy led to regression of the limbs' cutaneous vascular malformations. At the age of 9 months, the patient presented multiple heterogeneous hepatosplenic nodules. Aggressive treatment with prednisone, vincristine, and hepatosplenic embolizations resulted in initial improvement of the hepatosplenic lesions for few months, followed by an increase of the lesions with failure of response to treatment despite adding alpha-interferon-2b to treatment. The patient died at the age of 19 months. The autopsy's pathological examination revealed a hepatic-based angiosarcoma with plurimetastatic dissemination to the spleen, lungs, peritoneum, pleura, mesenteric linings as well as the serosa of the stomach and small intestine. Multiple cutaneous and visceral complex capillaro-lymphatico-venous malformations were also identified. We hypothesize that these multiple extensive mixed vascular malformations were associated with chronic lymphedema which probably predisposed to the development of the angiosarcoma in our patient. PMID:20863270

  11. Congenital vascular malformations in scintigraphic evaluation

    PubMed Central

    Pilecki, Stanisław; Gierach, Marcin; Gierach, Joanna; Świętaszczyk, Cyprian; Junik, Roman; Lasek, Władysław

    2014-01-01

    Summary Background Congenital vascular malformations are tumour-like, non-neoplastic lesions caused by disorders of vascular tissue morphogenesis. They are characterised by a normal cell replacement cycle throughout all growth phases and do not undergo spontaneous involution. Here we present a scintigraphic image of familial congenital vascular malformations in two sisters. Material/Methods A 17-years-old young woman with a history of multiple hospitalisations for foci of vascular anomalies appearing progressively in the upper and lower right limbs, chest wall and spleen. A Parkes Weber syndrome was diagnosed based on the clinical picture. Due to the occurrence of new foci of malformations, a whole-body scintigraphic examination was performed. A 12-years-old girl reported a lump in the right lower limb present for approximately 2 years, which was clinically identified as a vascular lesion in the area of calcaneus and talus. Phleboscintigraphy visualized normal radiomarker outflow from the feet via the deep venous system, also observed in the superficial venous system once the tourniquets were released. In static and whole-body examinations vascular malformations were visualised in the area of the medial cuneiform, navicular and talus bones of the left foot, as well as in the projection of right calcaneus and above the right talocrural joint. Conclusions People with undiagnosed disorders related to the presence of vascular malformations should undergo periodic follow-up to identify lesions that may be the cause of potentially serious complications and to assess the results of treatment. Presented scintigraphic methods may be used for both diagnosing and monitoring of disease progression. PMID:24567769

  12. 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.

  13. 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.

  14. Pretreatment imaging of peripheral vascular malformations

    PubMed Central

    Johnson, Joshua B; Cogswell, Petrice M; McKusick, Michael A; Binkovitz, Larry A; Riederer, Stephen J; Young, Phillip M

    2015-01-01

    Peripheral vascular malformations (VMs) are complex and diverse vascular lesions which require individualized pretreatment planning. Pretreatment imaging using various modalities, especially magnetic resonance imaging and time-resolved magnetic resonance angiography, is a valuable tool for classifying peripheral VMs to allow proper diagnosis, demonstrate complete extent, identify the nidus, and distinguish between low-flow and high-flow dynamics that determines the treatment approach. We discuss pretreatment imaging findings in four patients with peripheral VMs and how diagnostic imaging helped guide management. PMID:25625123

  15. Advanced noninvasive imaging of spinal vascular malformations

    PubMed Central

    Eddleman, Christopher S.; Jeong, Hyun; Cashen, Ty A.; Walker, Matthew; Bendok, Bernard R.; Batjer, H. Hunt; Carroll, Timothy J.

    2010-01-01

    Spinal vascular malformations (SVMs) are an uncommon, heterogeneous group of vascular anomalies that can render devastating neurological consequences if they are not diagnosed and treated in a timely fashion. Imaging SVMs has always presented a formidable challenge because their clinical and imaging presentations resemble those of neoplasms, demyelination diseases, and infection. Advancements in noninvasive imaging modalities (MR and CT angiography) have increased during the last decade and have improved the ability to accurately diagnose spinal vascular anomalies. In addition, intraoperative imaging techniques have been developed that aid in the intraoperative assessment before, during, and after resection of these lesions with minimal and/or optimal use of spinal digital subtraction angiography. In this report, the authors review recent advancements in the imaging of SVMs that will likely lead to more timely diagnoses and treatment while reducing procedural risk exposure to the patients who harbor these uncommon spinal lesions. PMID:19119895

  16. GI-Associated Hemangiomas and Vascular Malformations

    PubMed Central

    Yoo, Stephen

    2011-01-01

    Hemangiomas and vascular malformations of the gastrointestinal tract, rare clinical entities, present as overt or occult bleeding. They can be distributed throughout the intestinal digestive system, or present as a singular cavernous hemangioma or malformation, which is often located in the rectosigmoid region. Misdiagnosis is common despite characteristic radiographic features such as radiolucent phleboliths on plain film imaging and a purplish nodule on endoscopy. Adjunctive imaging such as computed tomography and magnetic resonance imaging are suggested as there is potential for local invasion. Endorectal ultrasound with Doppler has also been found to be useful in some instances. Surgical resection is the mainstay of treatment, with an emphasis on sphincter preservation. Nonsurgical endoscopic treatment with banding and sclerotherapy has been reported with success, especially in instances where an extensive resection is not feasible. PMID:22942801

  17. Benign Vascular Malformation at the Ischial Tuberosity.

    PubMed

    Said, Rami; Bevelaqua, Anna-Christina

    2016-07-01

    A 31-year-old female student was referred to physical therapy with a chief complaint of proximal, posterior left thigh pain that began insidiously 12 months prior, and progressively worsened while training for a half-marathon. A mobile, soft mass was identified just inferior to the ischial tuberosity that was tender and painful to palpation, recreating the patient's chief complaint. Radiographic findings were negative for a suspected avulsion fracture at the ischial tuberosity. Therefore, the physician performed musculoskeletal ultrasonography, which revealed a superficial hypoechoic mass with vascular flow. Magnetic resonance imaging and a subsequent biopsy led to the diagnosis of a benign vascular malformation. J Orthop Sports Phys Ther 2016;46(7):607. doi:10.2519/jospt.2016.0410. PMID:27363574

  18. 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

  19. US and MRI features in venous vascular malformation of the abdominal wall. A case report

    PubMed Central

    Alessandrino, F.; Maira, A.; Tarantino, C.C.

    2012-01-01

    Vascular anomalies are classified as vascular tumors and vascular malformations. Venous vascular malformations are the most common type of vascular malformation. They may be isolated or multiple and they rarely affect the trunk. The authors report a rare case of isolated venous vascular malformation of the abdominal wall with an emphasis on the related MRI and ultrasound (US) features. PMID:23450707

  20. 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

  1. 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.

  2. Intracranial vascular malformations: MR and CT imaging

    SciTech Connect

    Kucharczyk, W.; Lemme-Pleghos, L.; Uske, A.; Brant-Zawadzki, M.; Dooms, G.; Norman, D.

    1985-08-01

    Twenty-four patients with 29 cerebrovascular malformations were evaluated with a combination of computed tomography (CT), angiography, and magnetic resonance (MR) imaging. Characteristics of the malformations on MR images were reviewed retrospectively, and a comparative evaluation of MR and CT images was made. Of 14 angiographically evident malformations, 13 intra-axial lesions were detected on both CT and MR images, and one dural malformation gave false-negative results on both modalities. The appearance of parenchymal lesions on MR images closely mirrored characteristic CT findings. Angiographically evident malformations have a highly characteristic appearance on MR images. MR may be more sensitive than CT in the detection of small hemorrhagic foci associated with cryptic arteriovenous malformations and may add specificity in the diagnosis of occult malformations in some cases, but MR is less sensitive than CT for the detection of small calcified malformations.

  3. Sonographic Diagnosis of Fetal Adrenal Hemorrhage Complicating a Vein of Galen Aneurysmal Malformation

    PubMed Central

    Kütük, Mehmet Serdar; Doğanay, Selim; Özdemir, Ahmet; Görkem, Süreyya Burcu; Öztürk, Adnan

    2016-01-01

    Background: The vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which causes end-organ ischemia or venous congestion due to heart failure. Adrenal hemorrhage associated with VGAM has not been reported in the literature. We present the imaging findings of a fetal VGAM with adrenal hemorrhage. Case Report: A 26 year-old primigravida woman whose fetus with VGAM and mild cardiomegaly was scanned in the 34th week. On fetal ultrasound, a hyperechoic, well-circumscribed mass in the left suprarenal region was shown. Fetal and postnatal magnetic resonance imaging confirmed the diagnosis of adrenal hemorrhage. The baby died after delivery. Conclusion: Adrenal hemorrhage can complicate VGAM in fetuses with severe heart failure. Evaluation of the adrenal gland in affected cases may contribute to the prenatal counseling, and postnatal management. PMID:26966627

  4. High Temporal and Spatial Resolution Imaging of Peripheral Vascular Malformations

    PubMed Central

    Mostardi, Petrice M.; Young, Phillip M.; McKusick, Michael A.; Riederer, Stephen J.

    2013-01-01

    Purpose To assess the performance of a recently developed 3D time-resolved CE-MRA technique, Cartesian Acquisition with Projection-Reconstruction-like sampling (CAPR), for accurate characterization and treatment planning of vascular malformations of the periphery. Materials and Methods Twelve patient studies were performed (eight female, four male; average age, 33 years). The protocol consisted of three-dimensional (3D) time-resolved CE-MRA followed by a single late phase T1-weighted acquisition. Vascular malformations were imaged in the forearm, hand, thigh, and foot. Imaging evaluation was performed for accurate characterization of lesion type, identification of feeding and draining vessels, involvement with surrounding tissue, overall quality for diagnosis and treatment planning, and correlation with conventional angiography. Results Time-resolved CE-MRA allowed for characterization of malformation flow and type. Feeding and draining vessels were identified in all cases. Overall quality for diagnosis and treatment planning was 3.58/4.0, and correlation with conventional angiography was scored as 3.89/4.0. Conclusion The CAPR time series has been shown to portray the temporal dynamics and structure of vascular malformations as well as the normal vasculature with high quality. CAPR time-resolved imaging is able to accurately characterize high and low flow lesions, allowing for pretreatment lesion assessment and treatment planning. Delayed imaging is important to capture complete filling of very slow flow vascular malformations. PMID:22674646

  5. Preserved saphenous vein allografts for vascular access.

    PubMed

    Piccone, V A; Sika, J; Ahmed, N; LeVeen, H H; DiScala, V

    1978-09-01

    Preserved venous allografts were used as an alternate access procedure in 70 patients receiving dialysis during a three year period. The clinical experience with allograft fistulas revealed an extremely high initial patency rate; absence of infection postoperatively and during three years of dialysis; suitability for dialysis a week after implantation, thus greatly obviating the need for Silastic shunts; a low long term thrombosis rate and the weakly antigenic allograft veins produced no accelerated rejection of subsequently transplanted kidneys. Surviving patients average 172 dialysis treatments per allograft. Allograft fistulas constituted 45 per cent of the last 100 vascular procedures, an indication of the extent of usage. Microscopic examination of grafts retrieved from patients who died during the late follow-up period demonstrated that structural components of the wall of the vein were still identifiable. Allograft venous fistulas offer dependable, safe vascular access, especially in the infection prone patient with diabetes who is receiving dialysis treatment. The clinical results of allograft fistulas suggests a major role for this technique in vascular access operations. PMID:684591

  6. Vascular Integrity in the Pathogenesis of Brain Arteriovenous Malformation

    PubMed Central

    Zhang, Rui; Zhu, Wan

    2015-01-01

    Brain arteriovenous malformation (bAVM) is an important cause of intracranial hemorrhage (ICH), particularly in the young population. ICH is the first clinical symptom in about 50 % of bAVM patients. The vessels in bAVM are fragile and prone to rupture, causing bleeding into the brain. About 30 % of unruptured and non-hemorrhagic bAVMs demonstrate microscopic evidence of hemosiderin in the vascular wall. In bAVM mouse models, vascular mural cell coverage is reduced in the AVM lesion, accompanied by vascular leakage and microhemorrhage. In this review, we discuss possible signaling pathways involved in abnormal vascular development in bAVM. PMID:26463919

  7. Vascular tumors and malformations in children, Introduction.

    PubMed

    Maguiness, Sheilagh M

    2016-03-01

    Over the past decade, I have been amazed at the growth in the field of vascular anomalies. The recognition of vascular birthmarks as a defined area of medicine is a relatively recent event. The International Society for the Study of Vascular Anomalies (ISSVA) was founded by Drs John Mulliken and Anthony Young in the late 1970s. Mulliken and Glowacki's sentinel 1982 paper on the biologic classification of vascular anomalies further established the field, by providing clarity of nomenclature and unifying concepts that had previously been lacking. PMID:27607317

  8. [Congenital vascular malformations: epidemiology, classification and therapeutic basis].

    PubMed

    Pereira Albino, J

    2010-01-01

    Congenital vascular malformations are part of the rare diseases of angiology and vascular surgery and can present in a variety of forms. They rise a lot of doubts and many diagnostic and therapeutic challenges. Treatment options are widely variable and often debated; surgeons usually have doubts about the best procedure to adopt. It is also an area of great anatomic and functional variability where the confusion regarding the nomenclature and classifications has been frequent, rendering difficult to adopt standardized measures. There have been significant advances in the recent years towards reaching a consensus. Based on his practical clinical experience and past work on these issues, the author reviews the epidemiology, the classifications and the therapeutic basis of this pathology. The author emphasizes the fact that the International Society for the Study of Vascular Anomalies (ISSVA) classification provides a useful framework for classifying vascular anomalies, as well as the therapeutic percutaneous embolization using polidocanol foam to control venous malformations. PMID:20972487

  9. Sclerotherapy using 1% sodium tetradecyl sulfate to treat a vascular malformation: a report of two cases

    PubMed Central

    2015-01-01

    Vascular malformations are the most common congenital and neonatal vascular anomalies in the head and neck region. The demand for simple and esthetic vascular malformation treatments have increased more recently. In this study, two patients that were diagnosed with venous malformations were treated with sodium tetradecyl sulfate as a sclerosing agent. Recurrence was not found one year after the surgery. This article gives a brief case report of sclerotherapy as an effective approach to treat vascular malformations in the oral cavity. PMID:26734559

  10. Neuropilin-2 and vascular endothelial growth factor receptor-3 are up-regulated in human vascular malformations.

    PubMed

    Partanen, Taina A; Vuola, Pia; Jauhiainen, Suvi; Lohi, Jouko; Salminen, Päivi; Pitkäranta, Anne; Häkkinen, Sanna-Kaisa; Honkonen, Krista; Alitalo, Kari; Ylä-Herttuala, Seppo

    2013-01-01

    Despite multiple previous studies in the field of vascular anomalies, the mechanism(s) leading to their development, progression and maintenance has remained unclear. In this study, we have characterized the expression levels of vascular endothelial growth factors and their receptors in 33 human vascular anomalies. Analysis with quantitative real-time PCR and gene-specific assays showed higher expression of neuropilin-2 (NRP2) and VEGF-receptor-3 (VEGFR-3) mRNAs in vascular malformations (VascM) as compared to infantile hemangiomas (Hem). In addition, the expression levels of PlGF and VEGF-C mRNA were significantly higher in venous VascM when compared to the other VascM and Hem. Higher expression of NRP2 and VEGFR-3 were confirmed by immunohistochemistry. To further study the importance of NRP2 and VEGFR-3, endothelial cell (EC) cultures were established from vascular anomalies. It was found that NRP2 and VEGFR-3 mRNA levels were significantly higher in some of the VascM ECs as compared to human umbilical vein ECs which were used as control cells in the study. Furthermore, adenoviral delivery of soluble decoy NRP2 prevented the proliferation of ECs isolated from most of the vascular anomalies. Our findings suggest that NRP2 functions as a factor maintaining the pathological vascular network in these anomalies. Thus, NRP2 could become a potential therapeutic target for the diagnosis and treatment of vascular anomalies. PMID:22961441

  11. The “Little AVM”: A New Entity in High-flow versus Low-flow Vascular Malformations

    PubMed Central

    Stein, Michael; Guilfoyle, Regan; Courtemanche, Douglas J.; Moss, Wendy; Bucevska, Marija

    2014-01-01

    Background: Arteriovenous malformations (AVMs) are high-flow lesions with abnormal connections between arteries and veins without an intervening capillary bed. Infrequently, the radiographic diagnosis of a vascular lesion will not support the clinical diagnosis of an AVM. These “discrepant” lesions are not adequately captured within the current classification system and represent a treatment dilemma. The purpose of this study is to review our center’s experience with vascular malformations where incongruity in a patient’s clinical and radiographic presentation produces a diagnostic and therapeutic challenge. Methods: A retrospective chart review of patients with atypical AVM pre sen ta tions was performed. Parameters reviewed included patient history and demogra phics, clinical presentation, radiological imaging, and treatment modalities. Results: Over a 15-year period, we identified 7 cases of vascular malformations with discrepant clinical and radiological findings concerning flow characteristics. All patients were treated based on their radiological diagnosis and most were managed with sclerotherapy. No lesions evolved into a high-flow process, and there was no recurrence at a minimum of 24 months of follow-up. Conclusions: We have identified and described a unique subcategory of vascular malformations that have clinical features of high-flow malformations but radiological features of low-flow malformations. These lesions behave like low-flow malformations and should be treated as such. We propose that complex vascular malformations are best evaluated by both clinical and specialized diagnostic radiological means; the radiologic diagnoses should supplant what is found clinically, and ultimately treatment should be preferentially based on a radiological diagnosis. PMID:25426370

  12. Brain Vascular Malformation Consortium: Overview, Progress and Future Directions

    PubMed Central

    Akers, Amy L.; Ball, Karen L.; Clancy, Marianne; Comi, Anne M.; Faughnan, Marie E.; Gopal-Srivastava, Rashmi; Jacobs, Thomas P.; Kim, Helen; Krischer, Jeffrey; Marchuk, Douglas A.; McCulloch, Charles E.; Morrison, Leslie; Moses, Marsha; Moy, Claudia S.; Pawlikowska, Ludmilla; Young, William L.

    2013-01-01

    Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in “research silos” with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation, and HHT Foundation International. Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations. PMID:25221778

  13. Brain Vascular Malformation Consortium: Overview, Progress and Future Directions.

    PubMed

    Akers, Amy L; Ball, Karen L; Clancy, Marianne; Comi, Anne M; Faughnan, Marie E; Gopal-Srivastava, Rashmi; Jacobs, Thomas P; Kim, Helen; Krischer, Jeffrey; Marchuk, Douglas A; McCulloch, Charles E; Morrison, Leslie; Moses, Marsha; Moy, Claudia S; Pawlikowska, Ludmilla; Young, William L

    2013-04-01

    Brain vascular malformations are resource-intensive to manage effectively, are associated with serious neurological morbidity, lack specific medical therapies, and have no validated biomarkers for disease severity and progression. Investigators have tended to work in "research silos" with suboptimal cross-communication. We present here a paradigm for interdisciplinary collaboration to facilitate rare disease research. The Brain Vascular Malformation Consortium (BVMC) is a multidisciplinary, inter-institutional group of investigators, one of 17 consortia in the Office of Rare Disease Research Rare Disease Clinical Research Network (RDCRN). The diseases under study are: familial Cerebral Cavernous Malformations type 1, common Hispanic mutation (CCM1-CHM); Sturge-Weber Syndrome (SWS); and brain arteriovenous malformation in hereditary hemorrhagic telangiectasia (HHT). Each project is developing biomarkers for disease progression and severity, and has established scalable, relational databases for observational and longitudinal studies that are stored centrally by the RDCRN Data Management and Coordinating Center. Patient Support Organizations (PSOs) are a key RDCRN component in the recruitment and support of participants. The BVMC PSOs include Angioma Alliance, Sturge Weber Foundation, and HHT Foundation International. Our networks of clinical centers of excellence in SWS and HHT, as well as our PSOs, have enhanced BVMC patient recruitment. The BVMC provides unique and valuable resources to the clinical neurovascular community, and recently reported findings are reviewed. Future planned studies will apply successful approaches and insights across the three projects to leverage the combined resources of the BVMC and RDCRN in advancing new biomarkers and treatment strategies for patients with vascular malformations. PMID:25221778

  14. Thoracolumbar spinal vascular malformation as a rare cause of isolated intraventricular hemorrhage.

    PubMed

    Marlin, Evan S; Entwistle, John J; Arnold, Michael A; Pierson, Christopher R; Governale, Lance S

    2014-07-01

    Spinal vascular malformations are rare vascular lesions that most frequently present with back pain, radiculopathy, and/or myelopathy. Neurological decline is typically secondary to progressive radiculopathy, myelopathy, venous thrombosis, and stroke. Few case reports have described thoracolumbar spinal vascular malformations that present with both subarachnoid and intraventricular hemorrhage. This is the first reported case of a thoracolumbar spinal vascular malformation presenting with isolated intraventricular hemorrhage on initial imaging followed by acute and fatal rehemorrhage. PMID:24784978

  15. Stereotactic microresection of small cerebral vascular malformations (SCVM).

    PubMed

    Lerch, K D; Schaefer, D; Palleske, H

    1994-01-01

    Between 1988-1993 we performed CT-stereotactic guided microsurgical resection as a one-session-procedure in 46 patients bearing small (< 30 mm) cerebral vascular malformations (SCVM). The location of the SCVM was deep subcortical in 38 patients, temporal medio- basal in 3 and brainstem in 5. The surgical technique intended to minimise invasiveness by reducing the operative approach to a size less than the diameter of the lesion concerned. The mean diameter of our SCVM's was 20 mm ranging from 10 to 30 mm. Histologically we found 23 arteriovenous malformations, 22 cavernous malformations and 1 capillary telangiectasia (capillary malformation). Clinical symptomatology consisted mainly of seizures, (progressive) neurological deficit and (minute) acute intracerebral bleeding. The SCVMs could be demonstrated by contrast-enhanced CT as well as by MRI. 15 of the AVM's revealed as angiographically occult. Complete microsurgical resection of the SCVM was accomplished in all cases with a surgical morbidity of 6.5% and no operative mortality. In 14 patients, most of them with initial acute intracerebral haemorrhage, the pronounced focal neurological deficit improved. During the follow-up period (1/2-5 years) no rebleeding occurred. As far as epileptic seizures were concerned 13 patients became seizure-free without anticonvulsants and 11 patients seizure-free with anticonvulsant, in the remaining 4 patients seizures were reduced in frequency. PMID:7725939

  16. Malformation of cortical and vascular development in one family with parietal foramina determined by an ALX4 homeobox gene mutation.

    PubMed

    Valente, Marcelo; Valente, Kette D; Sugayama, Sofia S M; Kim, Chong Ae

    2004-01-01

    Vascular and cortical anomalies have been found in a family with parietal foramina type 2 (PFM2), which is determined by the ALX4 gene. It is believed that ALX4 has a bone-restricted expression. We report a case of PFM with age-related size variation in a 4-year-old boy, as well as in his mother, aunt and grandfather. MR imaging of the child demonstrates prominent malformations of cortical (polymicrogyric cortex with an unusual infolding pattern) and vascular development (persistence median prosencephalic vein), associated with high tentorial incisure periatrial white matter changes. PMID:15569759

  17. Trigeminal Neuralgia Due to Red Vein Draining a Supratentorial Arteriovenous Malformation: Case Report

    PubMed Central

    Inoue, Takuro; Shima, Ayako; Hirai, Hisao; Suzuki, Fumio; Matsuda, Masayuki

    2016-01-01

    Trigeminal neuralgia (TGN) is rarely caused by arteriovenous malformation (AVM). The AVMs causing TGN are reported mostly in the ipsilateral posterior fossa. The culprit vessels are dilated feeding artery or nidus itself. We present a rare case of TGN caused by dilated draining veins from a supratentorial AVM. The patient suffered from TGN with an incidentally found large AVM, which had been left untreated. The neuralgia was successfully relieved by microvascular decompression. Dilated red veins compressed the nerve at the root entry zone and distant cisternal portion of the nerve. Technically, transposition is not practical for fragile, dilated red veins with high pressure. Interposition is safer method in this case. PMID:27390665

  18. Trigeminal Neuralgia Due to Red Vein Draining a Supratentorial Arteriovenous Malformation: Case Report.

    PubMed

    Inoue, Takuro; Shima, Ayako; Hirai, Hisao; Suzuki, Fumio; Matsuda, Masayuki

    2016-07-01

    Trigeminal neuralgia (TGN) is rarely caused by arteriovenous malformation (AVM). The AVMs causing TGN are reported mostly in the ipsilateral posterior fossa. The culprit vessels are dilated feeding artery or nidus itself. We present a rare case of TGN caused by dilated draining veins from a supratentorial AVM. The patient suffered from TGN with an incidentally found large AVM, which had been left untreated. The neuralgia was successfully relieved by microvascular decompression. Dilated red veins compressed the nerve at the root entry zone and distant cisternal portion of the nerve. Technically, transposition is not practical for fragile, dilated red veins with high pressure. Interposition is safer method in this case. PMID:27390665

  19. The Typical Presentation Spectrum of Deep Vein Thrombosis Associated with Inferior Vena Cava Malformations

    PubMed Central

    El Lakis, Mustapha A.; Chamieh, Jad

    2016-01-01

    Congenital malformations of the inferior vena cava (IVC) are rare and underreported. They can be a risk factor for deep venous thrombosis (DVT) as a result of inadequate venous drainage of the lower extremities through collateral circulation. The significant number of cases reported in the literature highlights their importance, warranting investigating their existence in younger individuals with idiopathic DVT of the lower extremities and pelvic veins. In this systematic review, we depict the typical presentation of IVC malformations, their management, and the management of their associated DVT. PMID:27478635

  20. Hyperhomocysteinemia promotes vascular remodeling in vein graph in mice.

    PubMed

    Tan, Hongmei; Shi, Chengwei; Jiang, Xiaohua; Lavelle, Muriel; Yu, Caijia; Yang, Xiaofeng; Wang, Hong

    2014-01-01

    This study investigated the role and mechanism of Hyperhomocysteinemia (HHcy) on vascular remodeling in mice. We assessed the effect of HHcy on vascular remodeling using a carotid arterial vein patch model in mice with the gene deletion of cystathionine-beta-synthase (Cbs). Vein grafts were harvested 4 weeks after surgery. Cross sections were analyzed using Verhoeff-van Gieson staining, Masson`s Trichrome staining, and immunostaining for morphological analysis and protein level assessment. The effect of Hcy on collagen secretion was examined in cultured rat aortic smooth muscle cells (RASMC). We found that Cbs-/- mice with severe HHcy exhibited thicker neointima and a higher percentage of luminal narrowing in vein grafts. In addition, severe HHcy increased elastin and collagen deposition in the neointima. Further, severe HHcy increases CD45 positive cells and proliferative cells in vein grafts. Finally, Hcy increases collagen secretion in RASMC. These results demonstrate that HHcy increases neointima formation, elastin and collagen deposition following a carotid arterial vein patch. The capacity of Hcy to promote vascular fibrosis and inflammation may contribute to the development of vascular remodeling. PMID:24896329

  1. Turner syndrome with spinal hemorrhage due to vascular malformation

    PubMed Central

    Yu, Min Kyung; Jung, Mo Kyung; Kim, Ki Eun; Kwon, Ah Reum; Kim, Duk Hee; Kim, Ho-Seong

    2015-01-01

    Turner syndrome (TS) is a relatively common chromosomal disorder and is associated with a range of comorbidities involving the cardiovascular system. Vascular abnormalities, in particular, are a common finding in cases of TS. However, dissection involving the vertebral arteries is rare. Here, we report the case of a 9-year-old girl with TS who had been treated with growth hormone replacement therapy for the past 3 years. She presented with weakness of both lower legs, and was ultimately diagnosed with spinal hemorrhage due to vascular malformation. We treated her with intravenous high dose dexamethasone (0.6 mg/kg) and she could walk without assistance after 6 days of treatment. In conclusion, when a patient with TS shows sudden weakness of the lower limbs, we should consider the possibility of spinal vessel rupture and try to take spine magnetic resonance imaging as soon as possible. We suggest a direction how to make a proper diagnosis and management of sudden vertebral artery hemorrhage in patients with TS. PMID:26817012

  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. Thrombosed vascular malformation within the flexor tendon sheath of the index finger in the palm

    PubMed Central

    Schonauer, Fabrizio; Nele, Gisella; La Rusca, Ivan

    2015-01-01

    Vascular malformations are relatively infrequent among benign lesions of the hand. We report the case of an arteriovenous malformation in a 48-year-old white woman presenting a mass in her left hand without any symptoms. The diagnosis was confirmed by histopathology and the lesion completely removed by surgery. PMID:27252973

  5. Intralesional bleomycin injection in management of low flow vascular malformations in children.

    PubMed

    Mohan, Anita T; Adams, Saleigh; Adams, Kevin; Hudson, Donald A

    2015-04-01

    Low flow vascular malformations are challenging to manage, particularly with their propensity to grow, and can lead to severe disfigurement and dysfunction. Traditional surgical excision is fraught with tedious dissection and complications, particularly in the head and neck region. Trends toward less invasive techniques, such as intralesional sclerotherapy, are proving to be successful independent treatments or adjuncts in management in low flow vascular malformations. This study was a retrospective case note review, over an 8-year period, reporting the outcomes of 32 children (mean = 5.8 years, range = 5 months-11.5 years) with radiologically confirmed low flow vascular malformations, treated with serial intralesional bleomycin injection (IBI) therapy. Patient demographics, lesion characteristics, imaging findings, treatment course, radiological and clinical response to treatment were recorded. An overall 91% (n = 29) response rate was achieved, with 28% obtaining complete resolution for low flow vascular malformations. Lesions were sub-categorized into venous malformation, including mixed venous-capillary (n = 27) or lymphatic malformation (LM) (n = 5). Twenty-seven of 32 children experienced no complications. Local complications included superficial skin infection (n = 2), skin necrosis (n = 1), hyperpigmentation, and minor contour deformity. There was no recurrence and no systemic side-effects to bleomycin. Mean follow-up was 38 months (range = 6-95 months). In conclusion, serial intralesional bleomycin injections can be effective and also safe in a paediatric population for the successful management of symptomatic or disfiguring low flow vascular malformations. PMID:25204206

  6. 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

  7. Vascular Malformation and Common Keratinocytic Nevus of the Soft Type: Phacomatosis Pigmentovascularis Revisited

    PubMed Central

    Laureano, André; Carvalho, Rodrigo; Amaro, Cristina; Freitas, Isabel; Cardoso, Jorge

    2014-01-01

    Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type. PMID:25506441

  8. Vein of Galen Aneurysmal Malformation: Prognostic Markers Depicted on Fetal MRI

    PubMed Central

    Wagner, Matthias W; Vaught, Arthur J; Poretti, Andrea; Blakemore, Karin J

    2015-01-01

    Fetal magnetic resonance imaging (MRI) serves a dual role in the prenatal diagnostic work up of a vein of Galen aneurysmal malformation (VGAM). First, it may confirm the prenatal ultrasound findings and secondly it may identify prognostically important secondary complications of the VGAM. Progressive heart failure with development of fetal hydrops and hemispheric white matter injuries are associated with a poor outcome in children with a VGAM. We present the prenatal findings using both ultrasound and MRI of a fetus with VGAM including bilateral injury of the cerebral hemispheres, severe dilatation of the jugular veins, cardiomegaly, and hydrops fetalis. The neonate died within 30 minutes after delivery. Moreover, fetal MRI revealed complete placenta praevia, uterine fibroids, and wrapping of the umbilical cord around the fetal neck. This additional information is unrelated to the fetal pathology, but could have been of importance to plan the delivery. PMID:25924177

  9. [Quality standards for ultrasonographic assessment of peripheral vascular malformations and vascular tumors. Report of the French Society for Vascular Medicine].

    PubMed

    Laroche, J-P; Becker, F; Khau-Van-Kien, A; Baudoin, P; Brisot, D; Buffler, A; Coupé, M; Jurus, C; Mestre, S; Miserey, G; Soulier-Sotto, V; Tissot, A; Viard, A; Vignes, S; Quéré, I

    2013-02-01

    THE QUALITY STANDARDS OF THE FRENCH SOCIETY OF VASCULAR MEDICINE FOR THE ULTRASONOGRAPHIC ASSESSMENT OF VASCULAR MALFORMATIONS ARE BASED ON THE TWO FOLLOWING REQUIREMENTS: Technical know-how: mastering the use of ultrasound devices and the method of examination. Medical know-how: ability to adapt the methods and scope of the examination to its clinical indication and purpose, and to rationally analyze and interpret its results. AIMS OF THE QUALITY STANDARDS: To describe an optimal method of examination in relation to the clinical question and hypothesis. To achieve consistent practice, methods, glossary, and reporting. To provide good practice reference points, and promote a high-quality process. ITEMS OF THE QUALITY STANDARDS: The three levels of examination; their clinical indications and goals. The reference standard examination (level 2), its variants according to clinical needs. The minimal content of the examination report; the letter to the referring physician (synthesis, conclusion and proposal for further investigation and/or therapeutic management). Commented glossary (anatomy, hemodynamics, semiology). Technical bases. Settings and use of ultrasound devices. Here, we discuss the methods of using ultrasonography for the assessment of peripheral vascular malformations and tumors. PMID:23312609

  10. 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

  11. 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". PMID:424976

  12. 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.

  13. Venous vascular malformation of the floor of mouth masquerading as a dermoid tumor.

    PubMed

    Dmytriw, Adam A; Song, Jin Soo A; Gullane, Patrick; terBrugge, Karel G; Yu, Eugene

    2016-01-01

    Venous vascular malformations (VVMs) are described as abnormal post-capillary lesions which exhibit low flow. These are typically malleable and may grow with endocrine fluctuations. A VVM that mimics the classic appearance of dermoid tumor on imaging has never been reported. We encountered a 43-year-old woman with intermittent dysphagia relating to a firm submandibular mass. Physical exam and cross-sectional imaging revealed features consistent with variant dermoid cyst. However, catheter angiography eventually demonstrated a VVM which possessed vessels of variable size and partial thrombosis. We report the case and propose that catheter angiography remains important in cases where vascular malformation is considered. PMID:26851131

  14. 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.

  15. 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

  16. 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

  17. Cortical actin regulation modulates vascular contractility and compliance in veins

    PubMed Central

    Saphirstein, Robert J; Gao, Yuan Z; Lin, Qian Qian; Morgan, Kathleen G

    2015-01-01

    Abstract The literature on arterial mechanics is extensive, but far less is known about mechanisms controlling mechanical properties of veins. We use here a multi-scale approach to identify subcellular sources of venous stiffness. Portal vein tissue displays a severalfold decrease in passive stiffness compared to aortic tissues. The α-adrenergic agonist phenylephrine (PE) increased tissue stress and stiffness, both attenuated by cytochalasin D (CytoD) and PP2, inhibitors of actin polymerization and Src activity, respectively. We quantify, for the first time, cortical cellular stiffness in freshly isolated contractile vascular smooth muscle cells using magnetic microneedle technology. Cortical stiffness is significantly increased by PE and CytoD inhibits this increase but, surprisingly, PP2 does not. No detectable change in focal adhesion size, measured by immunofluorescence of FAK and zyxin, accompanies the PE-induced changes in cortical stiffness. Probing with phospho-specific antibodies confirmed activation of FAK/Src and ERK pathways and caldesmon phosphorylation. Thus, venous tissue stiffness is regulated both at the level of the smooth muscle cell cortex, via cortical actin polymerization, and by downstream smooth muscle effectors of Src/ERK signalling pathways. These findings identify novel potential molecular targets for the modulation of venous capacitance and venous return in health and disease. Key points Most cardiovascular research focuses on arterial mechanisms of disease, largely ignoring venous mechanisms. Here we examine ex vivo venous stiffness, spanning tissue to molecular levels, using biomechanics and magnetic microneedle technology, and show for the first time that venous stiffness is regulated by a molecular actin switch within the vascular smooth muscle cell in the wall of the vein. This switch connects the contractile apparatus within the cell to adhesion structures and facilitates stiffening of the vessel wall, regulating blood flow return

  18. 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.

  19. Venolymphatic vascular malformation of the parotid gland extending into the parapharyngeal space: A rare presentation

    PubMed Central

    Khatib, Yasmeen; Dande, Madhura; Patel, Richa D; Gite, Vinod

    2016-01-01

    Vascular malformations (VMs) are structural malformations of vascular development causing soft tissue abnormality with functional and esthetic impairment. They are named by their predominant vessel type as arterial, venous, lymphatic or mixed types. VMs of the parotid gland are extremely rare and constitute a distinct entity of parotid pathology that requires specific diagnostic tools and management. Till 2013, only fifty cases of VMs of the parotid have been described in the literature. We present a case of a venolymphatic malformation of the parotid gland extending into the parapharyngeal space in a 21-year-old male who presented with a swelling on the left side of the face extending into the neck. Diagnosis was suggested by ultrasonography and computed tomography scan and was confirmed by magnetic resonance imaging examination. Complete surgical excision of the lesion was done with a favorable outcome. Diagnosis was confirmed based on histopathology and immunohistochemical studies. PMID:27601828

  20. 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

  1. 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.

  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. ACVRL1 gene variant in a patient with vein of Galen aneurysmal malformation

    PubMed Central

    Chida, Ayako; Shintani, Masaki; Wakamatsu, Hajime; Tsutsumi, Yoshiyuki; Iizuka, Yuo; Kawaguchi, Nanako; Furutani, Yoshiyuki; Inai, Kei; Nonoyama, Shigeaki; Nakanishi, Toshio

    2013-01-01

    Although mutations in the RASA1 gene in vein of Galen aneurysmal malformation (VGAM) and an endoglin gene mutation in a VGAM patient with a family history of hereditary hemorrhagic telangiectasia (HHT) have been identified, most VGAM cases have no mutation in these genes. We sought to detect mutations in other genes related to HHT. We screened for mutations in RASA1 and three genes (endoglin, activin receptor-like kinase 1 (ACVRL1), encoding ALK1, and SMAD4) related to HHT in four VGAM patients. One variant (c.652 C>T p.R218W) in ACVRL1 was identified. Immunoblotting revealed that the ALK1-R218W protein could not promote SMAD1/5/8 phosphorylation by BMP9 stimulation. On the other hand, wild-type ALK1 could enhance the phosphorylation as expected. Furthermore, the transcriptional activation of ALK1-R218W was less efficient than that of wild-type ALK1. We identified 1 variant in ACVRL1 in a VGAM patient. These findings suggest that the ACVRL1 variant-R218W may be associated with the pathogenesis of VGAM.

  4. [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. PMID:27164348

  5. Intra-vascular leiomyoma of the popliteal vein.

    PubMed Central

    Grimer, R. J.; Armstrong, G. R.

    1988-01-01

    A large mass in the popliteal fossa was found to be a leiomyoma of the popliteal vein with portions of tumour both inside and outside the vein. This is only the second recorded case of a benign smooth muscle tumour of a peripheral vein. Images Figure 1 PMID:3174546

  6. 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%.

  7. [Biotinidase deficiency and vascular ring malformation: case report].

    PubMed

    González-Salazar, Francisco; Gabino Gerardo-Aviles, José; Rodríguez Jacobo, Sofía; Vargas-Camacho, Gerardo

    2014-10-01

    Biotinidase deficiency is an autosomal recessive metabolic disorder that affects the cleavage of biotin. Family studies of the index case found that both parents are usually carriers and siblings have the altered gene, but only homozygotes have manifestations that vary depending on the deficiency grade. Mothers may have moderate deficiency and be asymptomatic; biotin deficiency in pregnant women causes defects in children. In a study, using human cells exposed to biotin deficiency, cell growth decreased contributing to the development of cleft palate. In newborns, biotinidase deficiency has been associated with VACTERL syndrome and annular pancreas. The case of an infant with biotinidase deficiency and congenital defect of the vascular ring is presented. This defect surrounds and compresses the trachea and esophagus, disturbing swallowing and breathing. Infant was supplemented with biotin and surgically intervened with excellent results. PMID:25192539

  8. 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.

  9. Angiographically occult vascular malformation of the intracranial accessory nerve: case report.

    PubMed

    Terterov, Sergei; McLaughlin, Nancy; Vinters, Harry; Martin, Neil A

    2016-07-01

    Angiographically occult cerebral vascular malformations (AOVMs) are usually found in the supratentorial brain parenchyma. Uncommonly, AOVMs can be found within the cavernous sinus or basal cisterns and can be associated with cranial nerves. AOVMs involving the intracranial segment of the spinal accessory nerve have not been described. A 46-year-old female patient presented with a history of episodic frontal headaches and episodes of nausea and dizziness, as well as gait instability progressing over 6 months prior to evaluation. Imaging revealed a well-circumscribed 3-cm extraaxial T1-weighted isointense and T2-weighted hyperintense contrast-enhancing mass centered in the region of the right lateral cerebellomedullary cistern. The patient underwent resection of the lesion. Although the intraoperative appearance was suggestive of a cavernous malformation, some histological findings were atypical, leading to the final diagnosis of vascular malformation, not otherwise specified. The patient's postoperative course was uneventful with complete resolution of symptoms. To the authors' knowledge, this is the first report of an AOVM involving the intracranial portion of the accessory nerve. For any AOVM located within the cerebellomedullary cistern or one suspected of involving a cranial nerve, the authors recommend including immunohistochemistry with primary antibody to neurofilament in the histopathology workup. PMID:26566204

  10. Endovascular management of a vein of Galen aneurysmal malformation in an infant with challenging femoral arterial access.

    PubMed

    Jagadeesan, Bharathi D; Zacharatos, Haralabos; Nascene, David R; Grande, Andrew W; Guillaume, Daniel J; Tummala, Ramachandra P

    2016-08-01

    A 5-month-old infant was to be treated with elective transarterial embolization for a vein of Galen aneurysmal malformation (VGAM). A team of endovascular surgical neuroradiologists, pediatric interventional radiologists, and pediatric cardiologists attempted conventional femoral arterial access, which was unsuccessful given the small caliber of the femoral arteries and superimposed severe vasospasm. Thereafter, eventual arterial access was achieved by navigating from the venous to the arterial system across the patent foramen ovale following a right femoral venous access. Embolization was then successfully performed. At a later date, the child underwent successful transvenous balloon-assisted embolization and eventual arterial embolization with cure of the VGAM. PMID:27058455

  11. The high risks of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood: case report and literature review.

    PubMed

    Jea, Andrew; Bradshaw, Tina J; Whitehead, William E; Curry, Daniel J; Dauser, Robert C; Luerssen, Thomas G

    2010-08-01

    Little to no pediatric or neurosurgical literature has been published about the complications of ventriculoperitoneal shunt procedures for hydrocephalus associated with vein of Galen malformations in childhood. The interventional neuroradiology literature, however, suggests that ventriculoperitoneal shunting as first-line treatment for hydrocephalus in children with vein of Galen malformations is fraught with short- and long-term dangers, including status epilepticus, intraventricular hemorrhage, subdural hematoma and hygroma, venous infarction, malignant dystrophic calcification, and worsening developmental delay. We present a single pediatric case where a ventriculoperitoneal shunt procedure for symptomatic hydrocephalus seemed to be the major contributing factor to the rapid neurological deterioration and eventual death of an infant with a vein of Galen malformation. Based on this experience and our review of the literature, we suggest the use of endovascular embolization of the vein of Galen malformation to reestablish a balance in hydrovenous dynamics as first-line treatment rather than directly addressing hydrocephalus with CSF diversion. The ventriculoperitoneal shunt procedure should be reserved for cases with symptomatic hydrocephalus in which the patient is a poor candidate for embolization, or for cases where endovascular therapy has already been maximized. The role of endoscopy in the treatment of hydrocephalus associated with vein of Galen malformations is not clear. PMID:20664304

  12. [Cerebral arteriovenous malformations: value of the non invasive vascular imaging techniques].

    PubMed

    Leclerc, X; Gauvrit, J Y; Trystram, D; Reyns, N; Pruvo, J P; Meder, J F

    2004-12-01

    Imaging evaluation of cerebral arteriovenous malformations (AVM) requires selective visualization of the different compartments of the malformation in order to select the therapeutic management. Conventional angiography remains the reference to analyze intracranial vessel conspicuity but non-invasive methods constitute an excellent alternative. Among these techniques, CT angiography is rarely used because of the need to inject iodinated contrast material and because of irradiation. MR angiography provides useful information and can be performed using several techniques: time of flight with or without contrast material injection, phase contrast, three-dimensional (3D) gradient echo acquisition after contrast material injection and, more recently, MR digital subtraction angiography. The purpose of this review article is to summarize the different non-invasive techniques for vascular imaging and to analyze the usefulness of these techniques for the assessment of brain AVMs. PMID:15687950

  13. Primary intra- and juxta-articular vascular malformations of the temporomandibular joint: a clinical analysis of 8 consecutive patients

    PubMed Central

    Zhou, Qin; Yang, Chi; Chen, Min-Jie; Qiu, Ya-Ting; Qiu, Wei-Liu; Zheng, Jia-Wei

    2015-01-01

    Objective: To analyze primary intra- and juxta-articular vascular malformations of the temporomandibular joint. Patients and methods: This study retrospectively reviewed eight patients (seven venous malformations and one lymphatico-venous malformation) who were treated for intra- or juxta-articular vascular malformations of the temporomandibular joint from November 2005 to January 2011. All patients underwent magnetic resonance imaging (MRI) preoperatively. Results: According to MRI findings, vascular malformations involving TMJ could be divided into 3 types; homogenous, lacunar and mixed types. All patients underwent surgical resection, and the final clinical diagnoses were confirmed by postoperative histopathology and immunohistochemical examinations. All treated patients had no clinical or radiographic signs of recurrence. Conclusion: Owing to the lower incidence and nonspecific clinical presentations, preoperative diagnosis of vascular malformations involving the TMJ region is very difficult. The classification based on MRI manifestations is proposed first, then it may greatly help in the initial diagnosis. Surgical resection is considered the first option for these TMJ lesions with excellent results. PMID:25932158

  14. Georges Brohee Prize. Oestrogen-progesterone, a new therapy of bleeding gastrointestinal vascular malformations.

    PubMed

    Van Cutsem, E

    1993-01-01

    Gastrointestinal vascular malformations can cause haemorrhage requiring multiple transfusions. Surgical or endoscopic therapy are ineffective when the vascular malformations are spread diffusely over the gastrointestinal tract, when lesions escape identification or are not accessible for treatment. Several reports suggest that oestrogen-progesterone therapy is effective in the treatment of epistaxis in hereditary haemorrhagic telangiectasia. The aim of our studies is to test the hypothesis that oestrogen-progesterone is an effective treatment for bleeding gastrointestinal vascular malformations in patients with a very high transfusion need and in whom surgical or endoscopic treatment failed or could not be performed. The mean transfusion requirement prior to entering the studies amounted to 35.4 units packed per patient. In an uncontrolled trial oestrogen-progesterone therapy diminished significantly transfusion requirements from 3.4 units packed cells per patient per month to 0.1 units packed cells (p = 0.02). Haemorrhage ceased totally in 3 of 7 patients. A double-blind randomized placebo-controlled, cross-over trial shows that therapy with 0.050 mg ethinyloestradiol and 1 mg norethisterone is very effective in reducing transfusion requirements: 2.8 versus 11.2 units packed cells over a 6 month treatment period (p = 0.002). Only 3 of 13 patients treated with ethinyloestradiol and norethisterone required transfusions for persistent bleeding, while 12 of 13 patients in the placebo group had transfusion requirements (p = 0.001). After stopping hormonal therapy, no transfusions were needed for a mean of 10 months. This indicates a long-lasting effect of ethinyloestradiol and norethisterone on bleeding and transfusion requirements.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8342398

  15. Vascular corrosion casting: analyzing wall shear stress in the portal vein and vascular abnormalities in portal hypertensive and cirrhotic rodents.

    PubMed

    Van Steenkiste, Christophe; Trachet, Bram; Casteleyn, Christophe; van Loo, Denis; Van Hoorebeke, Luc; Segers, Patrick; Geerts, Anja; Van Vlierberghe, Hans; Colle, Isabelle

    2010-11-01

    Vascular corrosion casting is an established method of anatomical preparation that has recently been revived and has proven to be an excellent tool for detailed three-dimensional (3D) morphological examination of normal and pathological microcirculation. In addition, the geometry provided by vascular casts can be further used to calculate wall shear stress (WSS) in a vascular bed using computational techniques. In the first part of this study, the microvascular morphological changes associated with portal hypertension (PHT) and cirrhosis in vascular casts are described. The second part of this study consists of a quantitative analysis of the WSS in the portal vein in casts of different animal models of PHT and cirrhosis using computational fluid dynamics (CFD). Microvascular changes in the splanchnic, hepatic and pulmonary territory of portal hypertensive and cirrhotic mice are described in detail with stereomicroscopic examination and scanning electron microscopy. To our knowledge, our results are the first to report the vascular changes in the common bile duct ligation cirrhotic model. Calculating WSS using CFD methods is a feasible technique in PHT and cirrhosis, enabling the differentiation between different animal models. First, a dimensional analysis was performed, followed by a CFD calculation describing the spatial and temporal WSS distributions in the portal vein. WSS was significantly different between sham/cirrhotic/pure PHT animals with the highest values in the latter. Up till now, no techniques have been developed to quantify WSS in the portal vein in laboratory animals. This study showed for the first time that vascular casting has an important role not only in the morphological evaluation of animal models of PHT and cirrhosis, but also in defining the biological response of the portal vein wall to hemodynamic changes. CFD in 3D geometries can be used to describe the spatial and temporal variations in WSS in the portal vein and to better understand

  16. Exogenous vascular endothelial growth factor induces malformed and hyperfused vessels during embryonic neovascularization.

    PubMed Central

    Drake, C J; Little, C D

    1995-01-01

    Vascular endothelial growth factor (VEGF) is a potent and specific endothelial mitogen that is able to induce angiogenesis in vivo [Leung, D. W., Cachianes, G., Kuang, W.-J., Goeddel, D. V. & Ferrara, N. (1989) Science 246 1306-1309]. To determine if VEGF also influences the behavior of primordial endothelial cells, we used an in vivo vascular assay based on the de novo formation of vessels. Japanese quail embryos injected with nanomolar quantities of the 165-residue form of VEGF at the onset of vasculogenesis exhibited profoundly altered vessel development. In fact, the overall patterning of the vascular network was abnormal in all VEGF-injected embryos. The malformations were attributable to two specific endothelial cell activities: (i) inappropriate neovascularization in normally avascular areas and (ii) the unregulated, excessive fusion of vessels. In the first instance, supernumerary vessels directly linked the inflow channel of the heart to the aortic outflow channel. The second aberrant activity led to the formation of vessels with abnormally large lumens. Ultimately, unregulated vessel fusion generated massive vascular sacs that obliterated the identity of individual vessels. These observations show that exogenous VEGF has an impact on the behavior of primordial endothelial cells engaged in vasculogenesis, and they strongly suggest that endogenous VEGF is important in vascular patterning and regulation of vessel size (lumen formation). Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 PMID:7543999

  17. 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.

  18. Major and minor arterial malformations in patients with cutaneous vascular abnormalities.

    PubMed

    Pascual-Castroviejo, Ignacio; Pascual-Pascual, Samuel I; Viaño, Juan; López-Gutierrez, Juan C; Palencia, Rafael

    2010-05-01

    The association of persistent embryonic arteries and the absence of 1 carotid or vertebral arteries with facial or neck hemangioma or vascular malformation have been frequently described. The abnormalities can involve major or minor vessels. Of 22 patients of our series with this neurocutaneous syndrome, 20 had the origin of both anterior cerebral arteries from the same internal carotid artery. Thirteen patients showed absence or hypoplasia of 1 carotid artery and 10 of 1 vertebral artery; 10 showed persistence of the trigeminal artery; 3 had persistent proatlantal artery; 6 showed the absence of the posterior communicating artery; and 4 had hypoplastic posterior cerebral artery. Other less frequent abnormalities were found in 7 patients. Intellectual level of most patients was either borderline or below normal. Abnormalities in the vascularization and perfusion of the frontal lobes may contribute to the borderline or lower mental level of these patients. PMID:19808986

  19. Assessment of PTEN-associated vascular malformations in a patient with Bannayan-Riley-Ruvalcaba syndrome.

    PubMed

    Anusic, Sandra; Clemens, Robert Karl Josef; Meier, Thomas Oleg; Amann-Vesti, Beatrice Ruth

    2016-01-01

    Misdiagnosis of phosphatase and tensin homologue hamartoma syndromes is common. Correct diagnosis has a relevant impact on patients, as the risk of malignancies is high and treatment options are limited. We report the case of a 24-year-old man who presented with symptomatic vascular intramuscular lesions of the left forearm and right calf, macrocephaly, post Hashimoto thyroiditis, a multicystic intracranial paratrigonal lesion, lentiginous hyperpigmented maculae on the foreskin and multiple skin lesions. MRI showed extended fibrofatty changes and malformed vessels in the forearm and calf lesions, also, arteriovenous shunting was present in these lesions. The patient had been treated by embolisation and surgically in the past, with limited results. A multidisciplinary assessment and genetic counselling were undertaken and a surveillance programme was initiated. Treatment options of the symptomatic vascular lesions include excision or possibly cryoablation. Physiotherapy to prevent progression of the contractures should be initiated meanwhile. PMID:27358095

  20. Loss-of-Function Mutations in ELMO2 Cause Intraosseous Vascular Malformation by Impeding RAC1 Signaling.

    PubMed

    Cetinkaya, Arda; Xiong, Jingwei Rachel; Vargel, İbrahim; Kösemehmetoğlu, Kemal; Canter, Halil İbrahim; Gerdan, Ömer Faruk; Longo, Nicola; Alzahrani, Ahmad; Camps, Mireia Perez; Taskiran, Ekim Zihni; Laupheimer, Simone; Botto, Lorenzo D; Paramalingam, Eeswari; Gormez, Zeliha; Uz, Elif; Yuksel, Bayram; Ruacan, Şevket; Sağıroğlu, Mahmut Şamil; Takahashi, Tokiharu; Reversade, Bruno; Akarsu, Nurten Ayse

    2016-08-01

    Vascular malformations are non-neoplastic expansions of blood vessels that arise due to errors during angiogenesis. They are a heterogeneous group of sporadic or inherited vascular disorders characterized by localized lesions of arteriovenous, capillary, or lymphatic origin. Vascular malformations that occur inside bone tissue are rare. Herein, we report loss-of-function mutations in ELMO2 (which translates extracellular signals into cellular movements) that are causative for autosomal-recessive intraosseous vascular malformation (VMOS) in five different families. Individuals with VMOS suffer from life-threatening progressive expansion of the jaw, craniofacial, and other intramembranous bones caused by malformed blood vessels that lack a mature vascular smooth muscle layer. Analysis of primary fibroblasts from an affected individual showed that absence of ELMO2 correlated with a significant downregulation of binding partner DOCK1, resulting in deficient RAC1-dependent cell migration. Unexpectedly, elmo2-knockout zebrafish appeared phenotypically normal, suggesting that there might be human-specific ELMO2 requirements in bone vasculature homeostasis or genetic compensation by related genes. Comparative phylogenetic analysis indicated that elmo2 originated upon the appearance of intramembranous bones and the jaw in ancestral vertebrates, implying that elmo2 might have been involved in the evolution of these novel traits. The present findings highlight the necessity of ELMO2 for maintaining vascular integrity, specifically in intramembranous bones. PMID:27476657

  1. Cerebral vascular malformations: Time-resolved CT angiography compared to DSA

    PubMed Central

    Lum, Cheemun; Chakraborty, Santanu; dos Santos, Marlise P

    2015-01-01

    Purpose The purpose of this article is to prospectively test the hypothesis that time-resolved CT angiography (TRCTA) on a Toshiba 320-slice CT scanner enables the same characterization of cerebral vascular malformation (CVM) including arteriovenous malformation (AVM), dural arteriovenous fistula (DAVF), pial arteriovenous fistula (PAVF) and developmental venous anomaly (DVA) compared to digital subtraction angiography (DSA). Materials and methods Eighteen (eight males, 10 females) consecutive patients (11 AVM, four DAVF, one PAVF, and two DVA) underwent 19 TRCTA (Aquillion one, Toshiba) for suspected CVM diagnosed on routine CT or MRI. One patient with a dural AVF underwent TRCTA and DSA twice before and after treatment. Of the 18 patients, 13 were followed with DSA (Artis, Siemens) within two months of TRCTA. Twenty-three sequential volume acquisitions of the whole head were acquired after injection of 50 ml contrast at the rate of 4 ml/sec. Two patients with DVA did not undergo DSA. Two TRCTA were not assessed because of technical problems. TRCTAs were independently reviewed by two neuroradiologists and DSA by two other neuroradiologists and graded according to the Spetzler-Martin classification, Borden classification, overall diagnostic quality, and level of confidence. Weighted kappa coefficients (k) were calculated to compare reader’s assessment of DSA vs TRCTA. Results There was excellent (k = 0.83 and 1) to good (k = 0.56, 0.61, 0.65 and 0.67) agreement between the different possible pairs of neuroradiologists for the assessment of vascular malformations. Conclusion TRCTA may be a sufficient noninvasive substitute for conventional DSA in certain clinical situations. PMID:26246101

  2. Longitudinal distribution of vascular resistance in the pulmonary arteries, capillaries, and veins

    PubMed Central

    Brody, Jerome S.; Stemmler, Edward J.; DuBois, Arthur B.

    1968-01-01

    A new method has been described for measuring the pressure and resistance to blood flow in the pulmonary arteries, capillaries, and veins. Studies were performed in dog isolated lung lobes perfused at constant flow with blood from a donor dog. Pulmonary artery and vein volume and total lobar blood volume were measured by the ether plethysmograph and dyedilution techniques. The longitudinal distribution of vascular resistance was determined by analyzing the decrease in perfusion pressure caused by a bolus of low viscosity liquid introduced into the vascular inflow of the lobe. The pulmonary arteries were responsible for 46% of total lobar vascular resistance, whereas the pulmonary capillaries and veins accounted for 34 and 20% of total lobar vascular resistance respectively. Vascular resistance was 322 dynes ·sec·cm-5/ml of vessel in the lobar pulmonary arteries, 112 dynes·sec·cm-5/ml in the pulmonary capillaries, and 115 dynes·sec·cm-5/ml in the lobar pulmonary veins. Peak vascular resistivity (resistance per milliliter of volume) was in an area 2 ml proximal to the capillary bed, but resistivity was high throughout the pulmonary arterial tree. The pulmonary arteries accounted for approximately 50% of vascular resistance upstream from the sluice point when alveolar pressure exceeded venous pressure. The method described provides the first measurements of pulmonary capillary pressure. Mid-capillary pressure averaged 13.3 cm H2O, pulmonary artery pressure averaged 20.4 cm H2O, and pulmonary vein pressure averaged 9.2 cm H2O. These techniques also provide a way of analyzing arterial, capillary, and venous responses to various pharmacologic and physiologic stimuli. PMID:4868032

  3. Co-occurrence of a cerebral cavernous malformation and an orbital cavernous hemangioma in a patient with seizures and visual symptoms: Rare crossroads for vascular malformations

    PubMed Central

    Choudhri, Omar; Feroze, Abdullah H.; Lad, Eleonora M.; Kim, Jonathan W.; Plowey, Edward D.; Karamchandani, Jason R.; Chang, Steven D.

    2014-01-01

    Background: Cerebral cavernous malformations (CCMs) are angiographically occult vascular malformations of the central nervous system. As a result of hemorrhage and mass effect, patients may present with focal neurologic deficits, seizures, and other symptoms necessitating treatment. Once symptomatic, most often from hemorrhage, CCMs are treated with microsurgical resection. Orbital cavernous hemangiomas (OCHs) are similar but distinct vascular malformations that present within the orbital cavity. Even though CCMs and OCHs are both marked by dilated endothelial-lined vascular channels, they are infrequently seen in the same patient. Case Description: We provide a brief overview of the two related pathologies in the context of a patient presenting to our care with concomitant lesions, which were both resected in full without complication. Conclusion: This is the first known report that describes a case of concomitant CCM and OCH and explores the origins of two pathologies that are rarely encountered together in neurosurgical practice. Recognition of disparate symptomatologies is important for properly managing these patients. PMID:25071938

  4. [A case of vein of Galen aneurysmal malformation with a newly developed dural arteriovenous fistula after successful embolization].

    PubMed

    Asano, Takeshi; Kuroda, Satoshi; Houkin, Kiyohiro; Yoshida, Daisuke; Cho, Kazutoshi; Shiraishi, Hideaki; Saito, Shinji

    2012-06-01

    We report a case of vein of Galen aneurysmal malformation (VGAM) with a newly developed dural arteriovenous fistula (AVF) subsequent to successful embolization. A male neonate diagnosed as VGAM with prenatal ultrasonography and MRI presented severe cardiac and respiratory failure soon after birth. Five sessions of transarterial embolization using NBCA were performed during the first 6 months of his life. The shunt flow was effectively reduced and heart failure was resolved after the treatment. Follow-up angiography performed 2.5 years after the last embolization revealed complete obliteration of VGAM and newly developed small dural AVF on the wall of the thrombosed falcorial sinus. We believe that the dural AVF in this case was caused by local venous hypertension or induction of angiogenic factor during the thrombosing process of VGAM. PMID:22647511

  5. Experimental Branch Retinal Vein Occlusion Induces Upstream Pericyte Loss and Vascular Destabilization

    PubMed Central

    Dominguez, Elisa; Raoul, William; Calippe, Bertrand; Sahel, José-Alain; Guillonneau, Xavier; Paques, Michel; Sennlaub, Florian

    2015-01-01

    Aims Branch retinal vein occlusion (BRVO) leads to extensive vascular remodeling and is important cause of visual impairment. Although the vascular morphological changes following experimental vein occlusion have been described in a variety of models using angiography, the underlying cellular events are ill defined. Methods and Results We here show that laser-induced experimental BRVO in mice leads to a wave of TUNEL-positive endothelial cell (EC) apoptosis in the upstream vascular network associated with a transient edema and hemorrhages. Subsequently, we observe an induction of EC proliferation within the dilated vein and capillaries, detected by EdU incorporation, and the edema resolves. However, the pericytes of the upstream capillaries are severely reduced, which was associated with continuing EC apoptosis and proliferation. The vascular remodeling was associated with increased expression of TGFβ, TSP-1, but also FGF2 expression. Exposure of the experimental animals to hypoxia, when pericyte (PC) dropout had occurred, led to a dramatic increase in endothelial cell proliferation, confirming the vascular instability induced by the experimental BRVO. Conclusion Experimental BRVO leads to acute endothelial cells apoptosis and increased permeability. Subsequently the upstream vascular network remains destabilized, characterized by pericyte dropout, un-physiologically high endothelial cells turnover and sensitivity to hypoxia. These early changes might pave the way for capillary loss and subsequent chronic ischemia and edema that characterize the late stage disease. PMID:26208283

  6. Biological distinctions between juvenile nasopharyngeal angiofibroma and vascular malformation: an immunohistochemical study.

    PubMed

    Zhang, Mengjun; Sun, Xicai; Yu, Huapeng; Hu, Li; Wang, Dehui

    2011-10-01

    The exact nature of juvenile nasopharyngeal angiofibroma (JNA) is still in dispute. In recent years, the main controversy of its nature has focused on hemangioma and vascular malformation. In this study, the immunolocalization of vascular endothelial growth factor (VEGF), VEGF receptor-1/fms-like tyrosine kinase-1 (VEGFR-1/Flt-1), VEGF receptor-2/fetal liver kinase-1 (VEGFR-2/Flk-1), proliferating cell nuclear antigen (PCNA), and CD34 was investigated in 28 cases of JNA and 20 cases of orbital cavernous hemangiomas (OCH). The immunostaining levels of VEGF, Flt-1, and Flk-1 were higher and more frequent in vascular endothelial cells of JNA than those of OCH (p<0.05). The average microvessel density (MVD) marked by CD34 in JNA was (49.3 ± 9.1)/HPF (high power field), which was higher than OCH (29.1 ± 6.7)/HPF (p<0.05). Immunoreactivity of PCNA was localized in both endothelial and stromal cell components of JNA, but was predominantly seen in the stromal cells. However, no PCNA immunoreactivity was identified in any of the stromal and endothelial cells in cases of OCH. The immunostaining levels of CD34, VEGF, Flt-1, Flk-1, and PCNA in JNA were higher than those in OCH. These data support the view that JNA has biological characteristics of an angiogenic histogenetic tumor. In the future, anti-angiogenic therapy may represent a novel treatment strategy for JNA. PMID:20688360

  7. 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

  8. 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.

  9. Peripheral limb vascular malformations: an update of appropriate imaging and treatment options of a challenging condition

    PubMed Central

    Farrant, J; Chhaya, N; Anwar, I; Marmery, H; Platts, A; Holloway, B

    2015-01-01

    Peripheral vascular malformations encompass a wide spectrum of lesions that can present as an incidental finding or produce potentially life- or limb-threatening complications. They can have intra-articular and intraosseous extensions that will result in more diverse symptomology and present greater therapeutic challenges. Developments in classification, imaging and interventional techniques have helped to improve outcome. The onus is now placed on appropriate detailed preliminary imaging, diagnosis and classification to direct management and exclude other more common mimics. Radiologists are thus playing an increasingly important role in the multidisciplinary teams charged with the care of these patients. By fully understanding the imaging characteristics and image-guided procedures available, radiologists will be armed with the tools to meet these responsibilities. This review highlights the recent advances made in imaging and the options available in interventional therapy. PMID:25525685

  10. Intracranial vascular malformations: imaging of charged-particle radiosurgery. Part II. Complications

    SciTech Connect

    Marks, M.P.; Delapaz, R.L.; Fabrikant, J.I.; Frankel, K.A.; Phillips, M.H.; Levy, R.P.; Enzmann, D.R.

    1988-08-01

    Seven of 24 patients with intracranial vascular malformations who were treated with helium-ion Bragg-peak radiosurgery had complications of therapy. New symptoms and corresponding radiologic abnormalities developed 4-28 months after therapy. Five patients had similar patterns of white matter changes and mass effect on computed tomographic scans and magnetic resonance images. The abnormalities were centered in the radiation field. Gray matter changes and abnormal enhancement in the thalamus and hypothalamus outside the radiation field developed in one patient. This patient also had vasculopathic changes on angiograms. Rapidly progressive large vessel vasculopathy developed in another patient and caused occlusion of major vessels. Thus, different mechanisms may be involved in the complications of heavy-ion radiosurgery.

  11. Hemangiomas, angiosarcomas, and vascular malformations represent the signaling abnormalities of pathogenic angiogenesis.

    PubMed

    Arbiser, J L; Bonner, M Y; Berrios, R L

    2009-11-01

    Angiogenesis is a major factor in the development of benign, inflammatory, and malignant processes of the skin. Endothelial cells are the effector cells of angiogenesis, and understanding their response to growth factors and inhibitors is critical to understanding the pathogenesis and treatment of skin disease. Hemangiomas, benign tumors of endothelial cells, represent the most common tumor of childhood. In our previous studies, we have found that tumor vasculature in human solid tumors expresses similarities in signaling to that of hemangiomas, making the knowledge of signaling in hemangiomas widely applicable. These similarities include expression of reactive oxygen, NFkB and akt in tumor vasculature. Furthermore, we have studied malignant vascular tumors, including hemangioendothelioma and angiosarcoma and have shown distinct signaling abnormalities in these tumors. The incidence of these tumors is expected to rise due to environmental insults, such as radiation and lumpectomy for breast cancer, dietary and industrial carcinogens (hepatic angiosarcoma), and chronic ultraviolet exposure and potential Agent Orange exposure. I hypothesize that hemangiomas, angiosarcomas, and vascular malformations represent the extremes of signaling abnormalities seen in pathogenic angiogenesis. PMID:19925405

  12. Transhepatic Preoperative Portal Vein Embolization Using the Amplatzer Vascular Plug: Report of Four Cases

    SciTech Connect

    Ringe, Kristina I. Weidemann, Juergen; Rosenthal, Herbert; Keberle, Marc; Chavan, Ajay; Baus, Stefan; Galanski, Michael

    2007-11-15

    The Amplatzer Vascular Plug (AVP) is a device originally intended for arterial and venous embolization in peripheral vessels. From December 2004 to March 2007 we implanted a total of 8 AVPs in the portal venous system in our institution for preoperative portal vein embolization in 4 patients (55-71 years) prior to right hemihepatectomy. AVP implantation was successful in all patients. Total occlusion of the embolized portal vein branches was achieved in all patients. There were no major complications associated with the embolization.

  13. The suitability of the femoral vein for permanent vascular access.

    PubMed

    Pecorari, M

    2004-01-01

    In our dialysis unit, we commonly position permanent catheters (the Tesio twin catheter) in femoral veins with 'high' exit sites, in the abdominal area. We prefer this method because of its intrinsic safety in patients immediately requiring hemodialysis (HD), because there is no need for ultrasound guidance and a low incidence of complications during and after positioning. In addition, we consider that this method is suitable for older patients without good native vessels for an arteriovenous fistula (AVF). Sixty-nine femoral catheters were positioned in 61 patients (mean age 70.8 yrs): 307 observational months/patients (mean duration 4.45 months) demonstrated the following results: a) no incidents during catheter positioning; b) four iliac-femoral thromboses (in three cases complete recanalization with heparin treatment); c) two catheters were removed due to an infection of the tunnel; d) no generalized, life-threatening sepsis. Therefore, we recommend this solution as a 'bridge-access' between the immediate need for HD and the successive best strategy for the treatment of end-stage renal disease (ESRD). PMID:16596552

  14. Saphenous vein forearm grafts and gortex thigh grafts as alternative forms of vascular access.

    PubMed

    Bhandari, S; Wilkinson, A; Sellars, L

    1995-11-01

    To compare the survival and complication rates of saphenous vein forearm grafts and gortex thigh grafts. Retrospective study over a twelve-year period with review of case-notes. Saphenous vein forearm grafts were constructed in 17 males and 12 females, mean age 61 years and gortex thigh grafts in 24 males and 22 females (49 grafts), mean age 49 years. Grafts were the primary form of access in 9 patients in each group. Follow-up was 45.6 and 135.2 patient years on dialysis for forearm grafts and thigh grafts respectively. One-year total survival was 89.4% (89.4% at 2 years and 71.5% at 3 years) and 84.9% (82.3% at 2 years and 70.4% at 3 years) for saphenous vein forearm grafts and gortex thigh grafts respectively. The overall complication rates were 0.22 and 0.61 per patient year on dialysis for saphenous vein forearm grafts and gortex thigh grafts respectively. Thrombosis occurred in 10% and 52%, infection in 0% and 35% and no complications in 62% and 24% of saphenous vein forearm grafts and gortex thigh grafts respectively. Both saphenous vein forearm grafts and gortex thigh grafts can provide satisfactory vascular access. The survival is similar at one year but gortex thigh grafts have a higher complication rate. PMID:8605714

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

    PubMed Central

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

    2016-01-01

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

  16. 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.

  17. Structure and vascular function of MEKK3-cerebral cavernous malformations 2 complex.

    PubMed

    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

  18. 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

  19. Arterial and venous coronary pressure-flow relations in anesthetized dogs. Evidence for a vascular waterfall in epicardial coronary veins.

    PubMed

    Uhlig, P N; Baer, R W; Vlahakes, G J; Hanley, F L; Messina, L M; Hoffman, J I

    1984-08-01

    The coronary circulation of anesthetized dogs was tested for the presence of vascular waterfalls by manipulating coronary arterial and coronary venous pressures. The left main coronary artery and the coronary sinus were cannulated, and relationships between coronary artery pressure, coronary sinus pressure, and coronary flow were studied. Experiments were conducted during diastolic arrests, under steady state conditions, in the absence of autoregulation. Relations of coronary flow to coronary sinus pressure at constant coronary artery pressure were consistent with the presence of a vascular waterfall in the coronary sinus. When the great cardiac vein was cannulated, relations of great vein flow to great vein pressure at constant coronary artery pressure were consistent with the presence of a vascular waterfall in the great vein, indicating that waterfall behavior can occur in epicardial veins other than the coronary sinus. In dogs on right heart bypass, with the coronary sinus and great vein uncannulated, the relationship between right atrial pressure and coronary sinus pressure showed a waterfall pattern, indicating that the waterfall is not an artifact of venous cannulation. In the right heart bypass experiments, venous waterfall behavior was seen in beating hearts as well as during diastolic arrests. We conclude that a vascular waterfall is present in epicardial coronary veins which can significantly influence coronary blood flow. PMID:6611215

  20. Combined Lymphedema and Capillary Malformation of the Lower Extremity

    PubMed Central

    Maclellan, Reid A.; Chaudry, Gulraiz

    2016-01-01

    Background: Primary lymphedema and capillary malformation are independent vascular malformations that can cause overgrowth of the lower extremity. We report a series of patients who had both types of malformations affecting the same leg. The condition is unique but may be confused with other types of vascular malformation overgrowth conditions (eg, Klippel–Trenaunay and Parkes Weber). Methods: Our Vascular Anomalies Center and Lymphedema Program databases were searched for patients with both capillary malformation and lymphedema. Diagnosis of lymphedema–capillary malformation was made by history, physical examination, and imaging studies. Because lymphedema–capillary malformation has phenotypical overlap with other conditions, only patients who had imaging confirming their diagnosis were included in the analysis. Clinical and radiological features, morbidity, and treatment were recorded. Results: Eight patients (4 females and 4 males) had confirmed lymphedema–capillary malformation. Referring diagnosis was Klippel–Trenaunay syndrome (n = 4), diffuse capillary malformation with overgrowth (n = 3), or lymphatic malformation (n = 1). The condition was unilateral (n = 6) or bilateral (n = 2). Morbidity included infection (n = 6), difficulty fitting clothes (n = 6), bleeding or leaking vesicles (n = 5), leg length discrepancy (n = 4), and difficulty ambulating (n = 3). All patients were managed with compression regimens. Operative management was liposuction (n = 3), treatment of phlebectatic veins (n = 3), staged skin/subcutaneous excision (n = 1), and/or epiphysiodesis (n = 1). Conclusions: Lymphedema and capillary malformation can occur together in the same extremity. Both conditions independently cause limb overgrowth primarily because of subcutaneous adipose deposition. Compression garments and suction-assisted lipectomy can improve the condition. Lymphedema–capillary malformation should not be confused with other vascular malformation overgrowth

  1. HIF-1α and HIF-2α induced angiogenesis in gastrointestinal vascular malformation and reversed by thalidomide.

    PubMed

    Feng, Nan; Chen, Haiying; Fu, Sengwang; Bian, Zhaolian; Lin, Xiaolu; Yang, Li; Gao, Yunjie; Fang, Jingyuan; Ge, Zhizheng

    2016-01-01

    Thalidomide is used in clinical practice to treat gastrointestinal vascular malformation (GIVM), but the pathogenesis of GIVM is not clear. Hypoxia inducible factor 1 alpha (HIF-1α) and 2 alpha (HIF-2α/EPAS1) are in the same family and act as master regulators of the adaptive response to hypoxia. HIF-1α and HIF-2α are up-regulated in vascular malformations in intestinal tissues from GIVM patients, but not in adjacent normal vessels. Therefore, we investigated the role of HIF-1α and HIF-2α during angiogenesis and the mechanism of thalidomide action. In vitro experiments confirmed that vascular endothelial growth factor (VEGF) was a direct target of HIF-2α and that HIF-1α and HIF-2α regulated NOTCH1, Ang2, and DLL4, which enhanced vessel-forming of endothelial cells. Thalidomide down-regulated the expression of HIF-1α and HIF-2α and inhibited angiogenesis. In vivo zebrafish experiments suggested that HIF-2α overexpression was associated with abnormal subintestinal vascular (SIV) sprouting, which was reversed by thalidomide. This result indicated that thalidomide regulated angiogenesis via the inhibition of HIF-1α and HIF-2α expression, which further regulated downstream factors, including VEGF, NOTCH1, DLL4, and Ang2. The abnormally high expression of HIF-1α and HIF-2α may contribute to GIVM. PMID:27249651

  2. HIF-1α and HIF-2α induced angiogenesis in gastrointestinal vascular malformation and reversed by thalidomide

    PubMed Central

    Feng, Nan; Chen, Haiying; Fu, Sengwang; Bian, Zhaolian; Lin, Xiaolu; Yang, Li; Gao, Yunjie; Fang, Jingyuan; Ge, Zhizheng

    2016-01-01

    Thalidomide is used in clinical practice to treat gastrointestinal vascular malformation (GIVM), but the pathogenesis of GIVM is not clear. Hypoxia inducible factor 1 alpha (HIF-1α) and 2 alpha (HIF-2α/EPAS1) are in the same family and act as master regulators of the adaptive response to hypoxia. HIF-1α and HIF-2α are up-regulated in vascular malformations in intestinal tissues from GIVM patients, but not in adjacent normal vessels. Therefore, we investigated the role of HIF-1α and HIF-2α during angiogenesis and the mechanism of thalidomide action. In vitro experiments confirmed that vascular endothelial growth factor (VEGF) was a direct target of HIF-2α and that HIF-1α and HIF-2α regulated NOTCH1, Ang2, and DLL4, which enhanced vessel-forming of endothelial cells. Thalidomide down-regulated the expression of HIF-1α and HIF-2α and inhibited angiogenesis. In vivo zebrafish experiments suggested that HIF-2α overexpression was associated with abnormal subintestinal vascular (SIV) sprouting, which was reversed by thalidomide. This result indicated that thalidomide regulated angiogenesis via the inhibition of HIF-1α and HIF-2α expression, which further regulated downstream factors, including VEGF, NOTCH1, DLL4, and Ang2. The abnormally high expression of HIF-1α and HIF-2α may contribute to GIVM. PMID:27249651

  3. Intraoral venous malformation with phleboliths

    PubMed Central

    Mohan, Ravi Prakash S.; Dhillon, Manu; Gill, Navneet

    2011-01-01

    The most common type of vascular malformation is the venous malformation and these are occasionally associated with phleboliths. We report a case of a 45 year old woman with intraoral venous malformation with phleboliths. PMID:24151422

  4. Late-presenting left internal mammary to great cardiac vein fistula treated with a vascular plug.

    PubMed

    Franco-Gutierrez, Raul; Estevez-Loureiro, Rodrigo; Calviño-Santos, Ramon

    2010-10-01

    A 73-year-old patient, who underwent triple bypass surgery in another hospital in 1991 after suffering an acute myocardial infarction, was admitted to our institution after an episode of unstable angina. Evidence of a left internal mammary artery grafted to the great cardiac anterior interventricular vein (GCV), with Qp:Qs > 1.5:1, was demonstrated by angiography. We report the closure of said fistula using a percutaneous left radial artery access and the deployment of an Amplatzer vascular occlusion device via the antegrade approach. PMID:20944199

  5. Lymphatic and other vascular malformative/overgrowth disorders are caused by somatic mutations in PIK3CA

    PubMed Central

    Luks, Valerie L.; Kamitaki, Nolan; Vivero, Matthew P.; Uller, Wibke; Rab, Rashed; Bovée, Judith V.M.G.; Rialon, Kristy L.; Guevara, Carlos J.; Alomari, Ahmad I.; Greene, Arin K.; Fishman, Steven J.; Kozakewich, Harry P.W.; Maclellan, Reid A.; Mulliken, John B.; Rahbar, Reza; Spencer, Samantha A.; Trenor, Cameron C.; Upton, Joseph; Zurakowski, David; Perkins, Jonathan A.; Kirsh, Andrew; Bennett, James T; Dobyns, William B; Kurek, Kyle C.; Warman, Matthew L.; McCarroll, Steven A.; Murillo, Rudy

    2015-01-01

    Objectives To test the hypothesis that somatic PIK3CA mutations would be found in patients with more common disorders including isolated lymphatic malformation (LM) and Klippel-Trenaunay syndrome (KTS). Study design We used next generation sequencing, droplet digital PCR (ddPCR), and single molecule molecular inversion probes (smMIPs) to search for somatic PIK3CA mutations in affected tissue from patients seen at Boston Children’s Hospital who had an isolated LM (n=17), KTS (n=21), fibro-adipose vascular anomaly (FAVA; n=8), or congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies syndrome (CLOVES; n = 33), the disorder for which we first identified somatic PIK3CA mutations. We also screened 5 of the more common PIK3CA mutations in a second cohort of patients with LM (n=31) from Seattle Children’s Hospital. Results Most individuals from Boston Children’s Hospital who had isolated LM (16/17) or LM as part of a syndrome, such as KTS (19/21), FAVA (4/8), and CLOVES (30/32) were somatic mosaic for PIK3CA mutations, with 5 specific PIK3CA mutations accounting for ~ 80% of cases. Seventy-four percent of patients with LM from Seattle Children’s Hospital also were somatic mosaic for 1 of 5 specific PIK3CA mutations. Many affected tissue specimens from both cohorts contained fewer than 10% mutant cells. Conclusions Somatic PIK3CA mutations are the most common cause of isolated lymphatic malformations and disorders in which lymphatic malformation is a component feature. Five PIK3CA mutations account for most cases. The search for causal mutations requires sampling of affected tissues and techniques that are capable of detecting low-level somatic mosaicism, because the abundance of mutant cells in a malformed tissue can be low. PMID:25681199

  6. Mathematical modeling of selective photothermolysis to aid the treatment of vascular malformations and hemangioma with pulsed dye laser.

    PubMed

    Shafirstein, Gal; Buckmiller, Lisa M; Waner, Milton; Bäumler, Wolfgang

    2007-06-01

    Pulsed dye lasers (PDL) are the standard of care in the treatment of cutaneous vascular disorders such as the port-wine strains or hemangiomas of infancy. Nonetheless, there is still uncertainty regarding the specific laser parameters that are likely to yield optimal clinical outcomes. Using mathematical modeling, we explain and associate clinical outcomes with laser wavelength, radiant exposure, and pulse time and shape. The model's prediction that a continuous PDL pulse of 0.45 ms with a radiant exposure of 6 J/cm(2) is equivalent to delivering a 1.5-ms pulse consisting of three pulses with a radiant exposure of 12 J/cm(2) is in agreement with clinical studies. The model also suggests that for vascular malformations involving vessel diameters in the range of 150-500 microm, one should use a PDL at a wavelength of 595 nm with a radiant exposure of at least 12 J/cm(2) and pulse time of 1.5 ms, delivered in three pulses. Whereas it is calculated that malformations with vessels smaller than 50 microm will not respond to PDL in any clinical setting, an excellent response to PDL treatment at either a 585- or 595-nm wavelength can be expected for malformations with vessel diameters of 50-150 microm. Epidermal cooling is highly recommended for all settings to minimize pain and the risk of side effects. Finally, the model is used to generate a reference table that suggests specific PDL parameters for the treatment of various malformations and hemangiomas. The table cannot replace a clinician's experience with respect to which and how parameters should be changed, but provides a defined window of parameters that should be tried to improve clinical response. PMID:17268765

  7. Vascular endothelial growth factor signaling regulates the segregation of artery and vein via ERK activity during vascular development

    SciTech Connect

    Kim, Se-Hee; Schmitt, Christopher E.; Woolls, Melissa J.; Holland, Melinda B.; Kim, Jun-Dae; Jin, Suk-Won

    2013-01-25

    Highlights: ► VEGF-A signaling regulates the segregation of axial vessels. ► VEGF-A signaling is mediated by PKC and ERK in this process. ► Ectopic activation of ERK is sufficient to rescue defects in vessel segregation. -- Abstract: Segregation of two axial vessels, the dorsal aorta and caudal vein, is one of the earliest patterning events occur during development of vasculature. Despite the importance of this process and recent advances in our understanding on vascular patterning during development, molecular mechanisms that coordinate the segregation of axial vessels remain largely elusive. In this report, we find that vascular endothelial growth factor-A (Vegf-A) signaling regulates the segregation of dorsal aorta and axial vein during development. Inhibition of Vegf-A pathway components including ligand Vegf-A and its cognate receptor Kdrl, caused failure in segregation of axial vessels in zebrafish embryos. Similarly, chemical inhibition of Mitogen-activated protein kinase kinase (Map2k1)/Extracellular-signal-regulated kinases (Erk) and phosphatidylinositol 3-kinases (PI3 K), which are downstream effectors of Vegf-A signaling pathway, led to the fusion of two axial vessels. Moreover, we find that restoring Erk activity by over-expression of constitutively active MEK in embryos with a reduced level of Vegf-A signaling can rescue the defects in axial vessel segregation. Taken together, our data show that segregation of axial vessels requires the function of Vegf-A signaling, and Erk may function as the major downstream effector in this process.

  8. Vascular Lesions.

    PubMed

    Jahnke, Marla N

    2016-08-01

    Vascular lesions in childhood are comprised of vascular tumors and vascular malformations. Vascular tumors encompass neoplasms of the vascular system, of which infantile hemangiomas (IHs) are the most common. Vascular malformations, on the other hand, consist of lesions due to anomalous development of the vascular system, including the capillary, venous, arterial, and lymphatic systems. Capillary malformations represent the most frequent type of vascular malformation. IHs and vascular malformations tend to follow relatively predictable growth patterns in that IHs grow then involute during early childhood, whereas vascular malformations tend to exhibit little change. Both vascular tumors and vascular malformations can demonstrate a wide range of severity and potential associated complications necessitating specialist intervention when appropriate. Evaluation and treatment of the most common types of vascular lesions are discussed in this article. [Pediatr Ann. 2016;45(8):e299-e305.]. PMID:27517358

  9. Fluctuating nature of an orbital venous-lymphatic anomaly in association with intracranial vascular malformations: a classical presentation.

    PubMed

    Kanagalingam, Sivashakthi; Wyse, Emily; Merbs, Shannath L; Pearl, Monica Smith

    2015-01-01

    Venous-lymphatic anomalies (VLA) are rare and benign congenital lesions of the lymphatic system, composed of endothelial-lined lymphatic cysts. They are most frequently located in the region of the head and neck, and represent 4% of all orbital masses. In those patients with extensive orbital VLAs, a strong association with intracranial vascular anomalies has been reported. Factors known to suddenly increase the size of these lesions include upper respiratory tract infections or intralesional haemorrhage; however, complete spontaneous regression is rare. We report on the classic presentation of a patient with a fluctuating right orbital VLA in association with an intracranial cavernous malformation and intracranial developmental venous anomaly. PMID:26438679

  10. Congenital internal jugular vein aneurysm in an infant: A rare entity.

    PubMed

    Awasthy, Neeraj; Khandelwal, Nidhi; Iyer, Krishna S

    2016-05-01

    A 1-month old baby boy presented with a mass at the root of the neck. On investigation, a saccular aneurysm arising from the internal jugular vein was diagnosed. The aneurysm was excised after ligating the patent internal jugular vein above and below the origin of the aneurysm. Histopathology confirmed the diagnosis of a vascular malformation. Vascular malformation of the internal jugular vein, presenting as neck mass, is extremely rare with no case described in neonates. We present one such interesting case. PMID:25425716

  11. Intrahepatic Left to Right Portoportal Venous Collateral Vascular Formation in Patients Undergoing Right Portal Vein Ligation

    SciTech Connect

    Lienden, K. P. van; Hoekstra, L. T.; Bennink, R. J.; Gulik, T. M. van

    2013-12-15

    Purpose: We investigated intrahepatic vascular changes in patients undergoing right portal vein ligation (PVL) or portal vein embolization (PVE) in conjunction with the ensuing hypertrophic response and function of the left liver lobe. Methods: Between December 2008 and October 2011, 7 patients underwent right PVL and 14 patients PVE. Computed tomographic (CT) volumetry to assess future remnant liver (FRL) and functional hepatobiliary scintigraphy were performed in all patients before and 3 weeks after portal vein occlusion. In 18 patients an intraoperative portography was performed to assess perfusion through the occluded portal branches. Results: In all patients after initially successful PVL, reperfused portal veins were observed on CT scan 3 weeks after portal occlusion. This was confirmed in all cases during intraoperative portography. Intrahepatic portoportal collaterals were identified in all patients in the PVL group and in one patient in the PVE group. In all other PVE patients, complete occlusion of the embolized portal branches was observed on CT scan and on intraoperative portography. The median increase of FRL volume after PVE was 41.6 % (range 10-305 %), and after PVL was only 8.1 % (range 0-102 %) (p = 0.179). There were no differences in FRL function between both groups. Conclusion: Preoperative PVE and PVL are both methods to induce hypertrophy of the FRL in anticipation of major liver resection. Compared to PVE, PVL seems less efficient in inducing hypertrophy of the nonoccluded left lobe. This could be caused by the formation of intrahepatic portoportal neocollateral vessels, through which the ligated portal branches are reperfused within 3 weeks.

  12. A vascular contractility bioassay using bovine right ruminal artery and vein.

    PubMed

    Klotz, J L; Bush, L P; Strickland, J R

    2011-06-01

    Endophyte-infected (Neotyphodium coenophialum) tall fescue (Lolium arundinaceum) produces ergot alkaloids that are associated with peripheral vasoconstriction in grazing animals, and ingestion of these alkaloids may affect splanchnic vasculature. Peripheral effects of ergot alkaloids have been well documented previously in cattle using a lateral saphenous vein bioassay. Because of significant differences in morphological and functional characteristics between vasculature supporting digestive and peripheral tissues, the bovine foregut vascular model required validation. Experiments were conducted, using dose-responses to norepinephrine and serotonin that were normalized to either 0.12 M KCl, or 0.1 mM norepinephrine or serotonin, to compare responses of vessels equilibrated at different tensions on the day of collection or the day after collection. Segments of a branch of right ruminal artery and vein were collected from the ventral coronary groove of healthy cattle of mixed breed, age, and sex (n = 20) at local abattoirs. Cross-sections of the artery and vein were suspended on luminal supports in a chamber of a multimyograph containing continuously oxygenated Krebs-Henseleit buffer (95% O(2)/5% CO(2), pH 7.4; 37°C). Vessels were allowed to equilibrate at either 0.5 or 1.0 g of tension for 1.5 h before exposure to a reference compound. Increasing concentrations of each biogenic amine were administered in 15-min intervals after buffer replacement. Data were normalized as a percentage of the contractile response induced by the reference compound for each tension and day of analysis. The ruminal artery and vein were both more responsive to KCl as a reference compound (P < 0.05) than to norepinephrine or serotonin and did not differ between days when normalized with KCl. Ruminal arteries had greater contractile responses (P < 0.05) when tension was set to 1.0 g, compared with 0.5 g, during equilibration. The ruminal vein response had a more stable maintenance of

  13. R132C IDH1 mutations are found in spindle cell hemangiomas and not in other vascular tumors or malformations.

    PubMed

    Kurek, Kyle C; Pansuriya, Twinkal C; van Ruler, Maayke A J H; van den Akker, Brendy; Luks, Valerie L; Verbeke, Sofie L J; Kozakewich, Harry P; Sciot, Raf; Lev, Dina; Lazar, Alexander J; Fletcher, Christopher D M; Bovée, Judith V M G

    2013-05-01

    Spindle cell hemangioma (SCH) is a rare, benign vascular tumor of the dermis and subcutis. The lesions can be multifocal and are overrepresented in Maffucci syndrome, in which patients also have multiple enchondromas. Somatic mosaic R132C IDH1 hotspot mutations were recently identified in Maffucci syndrome. We evaluated the presence of mutations in solitary and multiple SCHs in patients without multiple enchondromas and tested a range of other vascular lesions that enter into the differential diagnosis. The R132C IDH1 mutation was identified by hydrolysis probes assay and confirmed by Sanger sequencing in 18 of 28 (64%) SCHs; of the 10 negative cases, 2 harbored a mutation in IDH2 (R172T and R172M) by Sanger sequencing. None of 154 other vascular malformations and tumors harbored an IDH1 R132C mutation, and R132H IDH1 mutations were absent in all 182 cases. All 16 SCHs examined by immunohistochemistry were negative for expression of HIF-1α. In conclusion, 20 of 28 (71%) SCHs harbored mutations in exon 4 of IDH1 or IDH2. Given that mutations were absent in 154 other vascular lesions, the mutation seems to be highly specific for SCH. The mutation does not induce expression of HIF-1α in SCH, and therefore the exact mechanism by which mutations in IDH1 or IDH2 lead to vascular tumorigenesis remains to be established. PMID:23485734

  14. Percutaneous Transsplenic Access to the Portal Vein for Management of Vascular Complication in Patients with Chronic Liver Disease

    SciTech Connect

    Chu, Hee Ho; Kim, Hyo-Cheol Jae, Hwan Jun; Yi, Nam-Joon; Lee, Kwang-Woong; Suh, Kyung-Suk; Chung, Jin Wook; Park, Jae Hyung

    2012-12-15

    Purpose: To evaluate the safety and feasibility of percutaneous transsplenic access to the portal vein for management of vascular complication in patients with chronic liver diseases. Methods: Between Sept 2009 and April 2011, percutaneous transsplenic access to the portal vein was attempted in nine patients with chronic liver disease. Splenic vein puncture was performed under ultrasonographic guidance with a Chiba needle, followed by introduction of a 4 to 9F sheath. Four patients with hematemesis or hematochezia underwent variceal embolization. Another two patients underwent portosystemic shunt embolization in order to improve portal venous blood flow. Portal vein recanalization was attempted in three patients with a transplanted liver. The percutaneous transsplenic access site was closed using coils and glue. Results: Percutaneous transsplenic splenic vein catheterization was performed successfully in all patients. Gastric or jejunal varix embolization with glue and lipiodol mixture was performed successfully in four patients. In two patients with a massive portosystemic shunt, embolization of the shunting vessel with a vascular plug, microcoils, glue, and lipiodol mixture was achieved successfully. Portal vein recanalization was attempted in three patients with a transplanted liver; however, only one patient was treated successfully. Complete closure of the percutaneous transsplenic tract was achieved using coils and glue without bleeding complication in all patients. Conclusion: Percutaneous transsplenic access to the portal vein can be an alternative route for portography and further endovascular management in patients for whom conventional approaches are difficult or impossible.

  15. [Antithrombotic property of new growing endothelial cells on the prosthetic vascular grafts--experimental study of portal vein replacement].

    PubMed

    Ohtake, S

    1997-01-01

    On experimental portal vein replacement of mongrel dogs with high porosity EPTFE grafts (fibril length: 60 microns) wrapped in omental pedicle flap, it has been reported that healing process is promoted. But it is not clear on the antithrombotic properties of the newly growing endothelial cells on prosthetic vascular grafts. PGI2 and NO are known as antithrombotic factors that restrain the agglutination of platelets. We evaluated PGI2 and NO production from the newly growing endothelial cells on prosthetic vascular grafts. High porosity EPTFE grafts were implanted at the portal veins of mongrel dogs. After 3 months we removed the grafts and tried to procure the new growing endothelial cells by trypsinization and culture them. We confirmed by using Dil-Ac-LDL that endothelial-like cells on prosthetic vascular grafts were as same as the endothelial cells. As control cells, we used the endothelial cells of canine portal vein and infra vena cava. The basal PGI2 production from each cells and the thrombin-stimulated production of PGI2 were measured by radioimmuno assay. Thrombin concentration was established at 2 unit/ml. On the basal PGI2 production there was no significant difference between the grafts and the portal veins, and between the grafts and the infra vena cava. The PGI2 production of the grafts increased significantly by stimulating with thrombin. On the thrombin-stimulated production, there was no significant difference between the grafts and the portal veins, and between the grafts and the infra vena cava. NO production of the grafts was measured by the griess reaction. On NO production there was no significant difference between the grafts and the portal veins. But the grafts produced significantly more NO than the infra vena cava (P < 0.05). This study suggests that the newly growing endothelial cells on the prosthetic vascular grafts may have the antithrombotic properties equal to the endothelial cells on the portal vein and the infra vena cava. PMID

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

    PubMed

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

    2016-04-01

    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. 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

  18. Arteriovenous malformation of vein of Galen as a rare non-hypoxic cause of changes in fetal heart rate pattern during labor.

    PubMed

    Biringer, Kamil; Zubor, Pavol; Kudela, Erik; Kolarovszki, Branislav; Zibolen, Mirko; Danko, Jan

    2016-03-01

    The aim of this case report is to describe a rare non-hypoxic cause of pathological changes in fetal heart rate pattern during labor, and to determine management, including a description of important prenatal aspects when pathologic cardiotocographic recording is performed during labor. A fetus with rare arteriovenous malformation of the vein of Galen, which represents less than 1% of all intracranial arteriovenous malformations, was monitored by intrapartum external cardiotocography in the 37 + 5 gestational week. The baby was born by cesarean section because of signs of imminent intrauterine hypoxia on cardiotocography. However, metabolic acidosis was not confirmed in umbilical cord blood sampling. Despite intensive neonatal care management, the newborn died 31 h after delivery because of progressive cardiac decompensation, hypotension and multi-organ failure. Precise diagnosis of the abovementioned pathology, a pre-labor plan for delivery and postnatal prognosis assessment can significantly contribute to the avoidance of a misdiagnosis of fetal hypoxia and unnecessary operative delivery with marked medico-legal consequences. PMID:26694901

  19. 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

  20. Successful segmental thermal ablation of varicose saphenous veins in a patient with confirmed vascular Ehlers-Danlos syndrome.

    PubMed

    Frank, Michael; Says, Jerome; Denarié, Nicolas; Sapoval, Marc; Messas, Emmanuel

    2016-04-01

    We describe here the successful scheduled treatment of varicose veins by radiofrequency segmental thermal ablation in a 43-year-old patient with vascular Ehlers-Danlos syndrome. Her venous disease started at the age of 16 years, 1 year prior to her first major Ehlers-Danlos syndrome-related event which led to the diagnosis of her genetic condition. Surgical stripping was contra-indicated because of Ehlers-Danlos syndrome at the age of 18 years. More than 20 years later, her venous disease had become highly symptomatic despite daily compression and pain medication. Venous reassessment evidenced incompetent right and left great saphenous and left small saphenous veins, with increased diameters of both sapheno-femoral and sapheno-popliteal junctions. Radiofrequency endovenous ablation rather than surgery was considered because of its minimally invasive nature and because of standardized energy delivery.All intended-to-be-treated incompetent saphenous vein segments were occluded successfully, followed by an important improvement of clinical disease severity at day 30, persistent at 1 year post-treatment. Duplex ultrasound confirmed closure and fibrotic retraction of all treated venous segments at 1 year. This report shows that radiofrequency endovenous ablation may be a safe and effective therapy of varicose veins in patients with diagnosed vascular Ehlers-Danlos syndrome. PMID:25926429

  1. 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.

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

    PubMed Central

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

    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 Å crystal structure of CCM3. This structure shows an all α-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. PMID:20489202

  3. Meandering right pulmonary vein associated with severe and progressive "idiopathic-like" pulmonary hypertensive vascular disease.

    PubMed

    Cuenca, Sofia; Bret, Montserrat; Del Cerro, Maria Jesus

    2016-03-01

    Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure. PMID:26374461

  4. Genetic and epigenetic mechanisms in the development of arteriovenous malformations in the brain.

    PubMed

    Thomas, Jaya Mary; Surendran, Sumi; Abraham, Mathew; Rajavelu, Arumugam; Kartha, Chandrasekharan C

    2016-01-01

    Vascular malformations are developmental congenital abnormalities of the vascular system which may involve any segment of the vascular tree such as capillaries, veins, arteries, or lymphatics. Arteriovenous malformations (AVMs) are congenital vascular lesions, initially described as "erectile tumors," characterized by atypical aggregation of dilated arteries and veins. They may occur in any part of the body, including the brain, heart, liver, and skin. Severe clinical manifestations occur only in the brain. There is absence of normal vascular structure at the subarteriolar level and dearth of capillary bed resulting in aberrant arteriovenous shunting. The causative factor and pathogenic mechanisms of AVMs are unknown. Importantly, no marker proteins have been identified for AVM. AVM is a high flow vascular malformation and is considered to develop because of variability in the hemodynamic forces of blood flow. Altered local hemodynamics in the blood vessels can affect cellular metabolism and may trigger epigenetic factors of the endothelial cell. The genes that are recognized to be associated with AVM might be modulated by various epigenetic factors. We propose that AVMs result from a series of changes in the DNA methylation and histone modifications in the genes connected to vascular development. Aberrant epigenetic modifications in the genome of endothelial cells may drive the artery or vein to an aberrant phenotype. This review focuses on the molecular pathways of arterial and venous development and discusses the role of hemodynamic forces in the development of AVM and possible link between hemodynamic forces and epigenetic mechanisms in the pathogenesis of AVM. PMID:27453762

  5. 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.

  6. Vein Graft-Coated Vascular Stents: A Feasibility Study in a Canine Model

    SciTech Connect

    Schellhammer, Frank; Haberstroh, Joerg; Wakhloo, Ajay K.; Gottschalk, Eva; Schumacher, Martin

    1998-03-15

    Purpose: To evaluate different vein grafts for luminal coating of endovascular stents in normal canine arteries. Methods: Twenty-four tantalum Strecker stents were coated with either autologous (n= 10), denatured heterologous (n= 11), or denatured homologous vein grafts (n= 3). The carotid artery (n= 11) and the iliac artery (n= 13) were stented using a transfemoral approach. Angiograms were performed at days 0, 7, and 21, and months 3, 6, and 9. All grafts underwent histological examination. Results: Eight of 10 autologous vein grafts showed patency during the whole observation period of 9 months, without histological signs of inflammation. Denatured heterologous vein grafts revealed acute (n= 3), subacute (n= 5), or delayed (n= 3) vessel occlusion. Hyaloid transformation of the vein graft and lympho-plasmacellular formations were seen. Denatured homologous vein grafts showed acute vessel occlusion. Although significant inflammatory tissue response was seen, no host-versus-graft reaction was present. Conclusion: Autologous vein graft-coated stents showed good biocompatibility in canine arteries. Preparation was cumbersome and required surgical venae-sectio. Denatured vein grafts, however, were limited by inflammatory reactions.

  7. G-Protein-Coupled Receptor 35 Mediates Human Saphenous Vein Vascular Smooth Muscle Cell Migration and Endothelial Cell Proliferation

    PubMed Central

    McCallum, Jennifer E.; Mackenzie, Amanda E.; Divorty, Nina; Clarke, Carolyn; Delles, Christian; Milligan, Graeme; Nicklin, Stuart A.

    2016-01-01

    Vascular smooth muscle cell (VSMC) migration and proliferation is central to neointima formation in vein graft failure following coronary artery bypass. However, there are currently no pharmacological interventions that prevent vein graft failure through intimal occlusion. It is hence a therapeutic target. Here, we investigated the contribution of GPR35 to human VSMC and endothelial cell (EC) migration, using a scratch-wound assay, and also the contribution to proliferation, using MTS and BrdU assays, in in vitro models using recently characterized human GPR35 ortholog-selective small-molecule agonists and antagonists. Real-time PCR studies showed GPR35 to be robustly expressed in human VSMCs and ECs. Stimulation of GPR35, with either the human-selective agonist pamoic acid or the reference agonist zaprinast, promoted VSMC migration in the scratch-wound assay. These effects were blocked by coincubation with either of the human GPR35-specific antagonists, CID-2745687 or ML-145. These GPR35-mediated effects were produced by inducing alterations in the actin cytoskeleton via the Rho A/Rho kinase signaling axis. Additionally, the agonist ligands stimulated a proliferative response in ECs. These studies highlight the potential that small molecules that stimulate or block GPR35 activity can modulate vascular proliferation and migration. These data propose GPR35 as a translational therapeutic target in vascular remodeling. PMID:27064272

  8. Immunomodulation of vascular endothelium: Effects of ultraviolet B irradiation on vein allograft rejection

    SciTech Connect

    Marin, M.L.; Hardy, M.A.; Gordon, R.E.; Reemtsma, K.; Benvenisty, A.I. )

    1990-01-01

    Prosthetic grafts of vein allografts are inadequate as small-diameter vessel substitutes. We have applied ultraviolet B (UVB) irradiation to modulate the immunogenicity of vein allografts to avoid immunologic injury. The veins of male ACI rats were irradiated with UVB (60 mJ/cm2) in situ and transplanted to male ACI rats (autografts) and female Lewis rats (allografts). Nonirradiated veins served as controls. At 4, 7, 14, and 28 days, all grafts were patent and were studied for morphologic changes by scanning electron microscopy and for immunogold labeling of major histocompatibility complex class II antigen expression. In autografts, scanning electron microscopy demonstrated minimal endothelial loss after grafting, regardless of UVB irradiation. Untreated allografts showed severe endothelial injury 4, 7, and 14 days after transplantation. UVB irradiation of veins protected allografts from injury to the endothelium and basement membrane. Major histocompatibility complex class II-positive endothelial cells were not seen in autografts but were seen in 40% of cells 4 days after transplantation in untreated allografts. UVB-treated allografts showed MHC class II antigen expression labeling of 20% of the endothelial cells. Barr body analysis demonstrated the donor origin of these endothelial cells. UVB irradiation of rat vein allografts prolongs endothelial survival while decreasing endothelial surface expression of class II antigens. These data suggest that modification of vein immunogenicity with UVB irradiation may permit functional survival of small-vessel allografts without chronic immunosuppression.

  9. Anti-vascular endothelial growth factor for macular edema secondary to central retinal vein occlusion

    PubMed Central

    Braithwaite, Tasanee; Nanji, Afshan A; Greenberg, Paul B

    2014-01-01

    Background Central retinal vein occlusion (CRVO) is a common retinal vascular disorder in which macular edema (ME) may develop, with a consequent reduction in visual acuity. The visual prognosis in CRVO-ME is poor in a substantial proportion of patients, especially those with the ischemic subtype, and until recently there has been no treatment of proven benefit. Macular grid laser treatment is ineffective, and whilst a few recent randomized controlled trials (RCTs) suggest short-term gains in visual acuity with intravitreal steroids for patients with non-ischemic CRVO-ME, there is no established treatment for ischemic CRVO-ME. Anti-vascular endothelial growth factor (anti-VEGF) agents have been used to treat ME resulting from a variety of causes and may represent a treatment option for CRVO-ME. Objectives To investigate the effectiveness and safety of intravitreal anti-VEGF agents in the treatment of CRVO-ME. Search strategy We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2010, Issue 8), MEDLINE (January 1950 to August 2010), EMBASE (January 1980 to August 2010), Latin American and Caribbean Health Sciences Literature Database (LILACS) (January 1982 to August 2010), Cumulative Index to Nursing and Allied Health Literature (CINAHL) (January 1937 to August 2010), OpenSIGLE (January 1950 to August 2010), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com) and ClinicalTrials.gov (www.clinicaltrials.gov). There were no language or date restrictions in the search for trials. The electronic databases were last searched on 10 August 2010. Selection criteria We considered RCTs that compared intravitreal anti-VEGF agents of any dose or duration to sham injection or no treatment. We focused on studies that included individuals of any age or gender with unilateral or bilateral disease and a minimum of six months follow up. Secondarily, we

  10. Immunomodulation of vascular endothelium. 1. Ultrastructural changes following ultraviolet B irradiation of peripheral veins

    SciTech Connect

    Marin, M.L.; Gordon, R.E.; Hardy, M.A.; Reemtsma, K.; Benvenisty, A.I. )

    1990-02-01

    Immunologic function of endothelial cells is especially important in consideration of vein allografting for arterial reconstruction and in organ allotransplantation. Ultraviolet B radiation (UVB) has previously been shown to modulate graft immunogenicity, and to alter cell surface receptor function. In this study, superficial epigastric veins were UVB irradiated with 10, 24, 40, 80, and 150 mJ/cm2 while control veins were not irradiated; all specimens were examined for endothelial ultrastructural changes. Veins were perfuse-fixed at 1, 3, 7, 14, and 28 days after irradiation, and were evaluated by transmission electron microscopy and scanning electron microscopy. Control veins had a normal appearing endothelial lining, composed of elongated, attenuated endothelial cells. Veins irradiated with more than 24 mJ/cm2 displayed injured endothelial cells characterized by altered microvilli, defects in the cell surface, and a change in cell shape. The degree of cell damage correlated closely with increasing UVB dose. At doses of 80 mJ/cm2 or greater there was moderate to severe endothelial cell separation from the underlying basement membrane and an increase in cellular lysosomes. The effects of UVB were maximal at 3 days with virtual recovery in resurfacing of all specimens with endothelium 28 days after irradiation. These data suggest that UVB has a dose-dependent effect on venous endothelium that is morphologically reversible with time. Cell membrane changes seen following exposure to UVB may contribute to altered cell surface receptor function.

  11. Long term results of treatment of vascular malformations of the gastrointestinal tract by neodymium Yag laser photocoagulation.

    PubMed Central

    Rutgeerts, P; Van Gompel, F; Geboes, K; Vantrappen, G; Broeckaert, L; Coremans, G

    1985-01-01

    The effect of Yag laser photocoagulation on the course of bleeding of gastrointestinal vascular malformations was studied in 59 patients, with a total of 482 lesions. The lesions were located in the upper gastrointestinal tract alone in 25 patients, in the lower tract alone in 31 patients and in both the lower and the upper gastrointestinal tract in three patients. In the month before laser therapy the number of bleeding episodes averaged 1.09 +/- 0.6 (SD) per patient (n = 57) and the transfusion requirements 2.4 +/- 2.6 red blood cells units per patient, while in the month after treatment the bleeding incidence averaged 0.16 +/- 0.5 and the transfusion requirements 0.21 +/- 0.8 (both p less than 0.001). Long term results were analysed considering for each patient an equally long pretreatment and follow up period. After a mean follow up period of 11.5 months (1-48 months), 17 of the 57 patients available for follow up rebled. The reduction of the bleeding rate was statistically significant at one, six, 12, and 18 months of follow up, while transfusion rate was significantly decreased at one, six, and 12 months. The results were disappointing in patients with Osler-Weber-Rendu (n = 4) and in patients with angiomas associated with Von Willebrand's disease (n = 3), who all rebled. In angiodysplasia the treatment was successful in 82% of the 49 patients. The more numerous the lesions, the less effective the reduction in bleeding rate by laser treatment was. Histological studies showed that the haemostatic effect of Yag laser photocoagulation was obtained by destruction of the lesion. Rebleeding was due to lesions missed at the first treatment, incompletely treated lesions and recurrence of new lesions. In two patients a free caecal perforation necessitated a right hemicolectomy. In both patients numerous or very large lesions had been treated in the caecum. Images Fig. 3 PMID:3874122

  12. [Search for Factors Related to Vascular Pain Expression upon Administration of Oxaliplatin into a Peripheral Vein].

    PubMed

    Takagi, Akiko; Yonemoto, Nao; Aoyama, Yuuya; Touma, Yuri; Kajiwara, Michiko; Watanabe, Kosuke; Miyazaki, Yoshiko; Koinuma, Masayoshi

    2015-07-01

    We investigated the relationship between vascular pain and various characteristics (age, sex, cancer stage, performance status [PS], height, weight, body mass index [BMI], body surface area, oxaliplatin dose, and presence and absence of the initial administration of dexamethasone) in colorectal cancer patients who were administered initial doses of oxaliplatin intravenously. The study population included 29 patients treated at Higashi Totsuka Memorial Hospital between June 2010 and April 2014. One-way analysis of variance showed that vascular pain was significantly associated with weight (p=0.015), body surface area (p=0.013), and oxaliplatin doses (p=0.0026), where the significance level was p=0.05. Logistic regression analysis and the likelihood ratio test demonstrated that the likelihood of vascular pain increased with the increase in the oxaliplatin dose. According to the cut-off value of vascular pain determined using the receiver operating characteristic (ROC) analysis, a single dose of oxaliplatin was determined to be 175 mg or more. According to the cut-off value established using the ROC analysis, a single dose of oxaliplatin at which vascular pain is expressed was determined to be 175 mg or more. At this dose, 13 patients complained of vascular pain and 8 did not. At doses less than 175 mg, none of the 8 patients complained of vascular pain. These results suggest that lowering the diluted concentration and reducing the infusion rate of intravenously administered oxaliplatin may reduce vascular pain. PMID:26197757

  13. Anti-vascular endothelial growth factor for macular oedema secondary to central retinal vein occlusion

    PubMed Central

    Braithwaite, Tasanee; Nanji, Afshan A; Lindsley, Kristina; Greenberg, Paul B

    2014-01-01

    Background Central retinal vein occlusion (CRVO) is a relatively common retinal vascular disorder in which macular oedema may develop, with a consequent reduction in visual acuity. Until recently there has been no treatment of proven benefit, but growing evidence supports the use of anti-vascular endothelial growth factor (anti-VEGF) agents. Objectives To investigate the effectiveness and safety of anti-VEGF therapies for the treatment of macular oedema secondary to CRVO. Search methods We searched CENTRAL (which contains the Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2013, Issue 10), Ovid MEDLINE (January 1950 to October 2013), EMBASE (January 1980 to October 2013), Latin American and Caribbean Health Sciences Literature Database (LILACS) (January 1982 to October 2013), Cumulative Index to Nursing and Allied Health Literature (CINAHL) (January 1937 to October 2013), OpenGrey, OpenSIGLE (January 1950 to October 2013), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), Clinical-Trials.gov (www.clinicaltrials.gov), the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en) and Web of Science Conference Proceedings Citation Index-Science (CPCI-S). There were no language or date restrictions in the electronic search for trials. The electronic databases and clinical trials registers were last searched on 29th October 2013. Selection criteria We considered randomised controlled trials (RCTs) that compared intravitreal anti-VEGF agents of any dose or duration to sham injection or no treatment. We focused on studies that included individuals of any age or gender and a minimum of six months follow-up. Data collection and analysis Two review authors independently assessed trial quality and extracted data. The primary outcome was the proportion of participants with a gain in best-corrected visual acuity (BCVA) from baseline of

  14. Early experience with X-ray magnetic resonance fusion for low-flow vascular malformations in the pediatric interventional radiology suite.

    PubMed

    Hwang, Tiffany J; Girard, Erin; Shellikeri, Sphoorti; Setser, Randolph; Vossough, Arastoo; Ho-Fung, Victor; Cahill, Anne Marie

    2016-03-01

    This technical innovation describes our experience using an X-ray magnetic resonance fusion (XMRF) software program to overlay 3-D MR images on real-time fluoroscopic images during sclerotherapy procedures for vascular malformations at a large pediatric institution. Five cases have been selected to illustrate the application and various clinical utilities of XMRF during sclerotherapy procedures as well as the technical limitations of this technique. The cases demonstrate how to use XMRF in the interventional suite to derive additional information to improve therapeutic confidence with regards to the extent of lesion filling and to guide clinical management in terms of intraprocedural interventional measures. PMID:26681438

  15. Vascular geometry and oxygen diffusion in the vicinity of artery-vein pairs in the kidney.

    PubMed

    Ngo, Jennifer P; Kar, Saptarshi; Kett, Michelle M; Gardiner, Bruce S; Pearson, James T; Smith, David W; Ludbrook, John; Bertram, John F; Evans, Roger G

    2014-11-15

    Renal arterial-to-venous (AV) oxygen shunting limits oxygen delivery to renal tissue. To better understand how oxygen in arterial blood can bypass renal tissue, we quantified the radial geometry of AV pairs and how it differs according to arterial diameter and anatomic location. We then estimated diffusion of oxygen in the vicinity of arteries of typical geometry using a computational model. The kidneys of six rats were perfusion fixed, and the vasculature was filled with silicone rubber (Microfil). A single section was chosen from each kidney, and all arteries (n = 1,628) were identified. Intrarenal arteries were largely divisible into two "types," characterized by the presence or absence of a close physical relationship with a paired vein. Arteries with a close physical relationship with a paired vein were more likely to have a larger rather than smaller diameter, and more likely to be in the inner-cortex than the mid- or outer cortex. Computational simulations indicated that direct diffusion of oxygen from an artery to a paired vein can only occur when the two vessels have a close physical relationship. However, even in the absence of this close relationship oxygen can diffuse from an artery to periarteriolar capillaries and venules. Thus AV oxygen shunting in the proximal preglomerular circulation is dominated by direct diffusion of oxygen to a paired vein. In the distal preglomerular circulation, it may be sustained by diffusion of oxygen from arteries to capillaries and venules close to the artery wall, which is subsequently transported to renal veins by convection. PMID:25209866

  16. Congenital Vascular Malformation

    MedlinePlus

    ... types. The more primitive ones appear as thinwalled lakes in which venous blood collects and when they ... collection of grape-like clusters of these venous lakes. This type usually does not affect the venous ...

  17. Muscarinic receptors of the vascular bed: radioligand binding studies on bovine splenic veins.

    PubMed

    Brunner, F; Kukovetz, W R

    1986-01-01

    Despite an obvious lack of parasympathetic innervation to the spleen, pharmacological evidence suggests the presence of cholinergic receptors in isolated bovine splenic veins. We therefore studied muscarinic cholinergic binding sites in a bovine splenic vein preparation by direct radioligand binding techniques using [3H]quinuclidinyl benzilate ([3H]QNB) as radioactive probe. Saturation experiments indicated one homogeneous class of high-affinity binding sites, with a KD of 0.11 nM and a binding site density Bmax of 55 fmol/mg protein. The rate constants at 37 degrees C for formation and dissociation of the [3H]QNB receptor complex were 2.7 X 10(9) M-1 h-1 and 0.38 h-1, respectively, yielding a KD of 0.14 nM. The binding sites showed a high stereospecificity, which was evident from competition experiments with dexetimide (KI = 1.3 nM) and levetimide (KI = 4.6 microM). In competition experiments with muscarinic and nicotinic antagonists and some antidepressants, only one binding site was found, whereas with muscarinic agonists, two binding sites were detected. In the presence of 0.1 mM guanyl-imido-diphosphate, only one binding site could be identified with the muscarinic agonist carbamylcholine. The affinity of [3H]QNB, on the other hand, was slightly decreased, and Bmax values were unchanged. It is concluded that specific, saturable, high-affinity muscarinic binding sites in the bovine splenic vein have been identified and characterized that exhibit properties similar to cholinergic receptors of brain and peripheral tissues and probably mediate acetylcholine-induced relaxation of splenic veins. PMID:2427809

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

    PubMed

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

    2015-10-01

    A polymer based material was developed to act as an embolic agent and drug reservoir for the treatment of arteriovenous malformations (AVM) and hyper vascularized solid tumors. The aim was to combine the blocking of blood supply to the target region and the inhibition of the embolization-stimulated angiogenesis. The material is composed of an ethanolic solution of a linear acrylate based copolymer and acrylate calibrated microparticles containing nanospheres loaded with sunitinib, an anti-angiogenic agent. The precipitation of the linear copolymer in aqueous environment after injection through microcatheter results in the formation of an in-situ embolization gel whereas the microparticles serve to increase the cohesive properties of the embolization agent and to form a reservoir from which the sunitinib-loaded nanospheres are released post-embolization. The swollen state of the microparticles in contact with aqueous medium results in the release of the nanospheres out of microparticles macromolecular structure. After the synthesis, the formulation and the characterization of the different components of the material, anti-angiogenic activity was evaluated in vitro using endothelial cells and in vivo using corneal neovascularization model in rabbit. The efficiency of the arterial embolization was tested in vivo in a sheep model. Results proved the feasibility of this new system for vascular embolization in association with an in situ delivery of anti-angiogenic drug. This combination is a promising strategy for the management of arteriovenous malformations and solid tumors. PMID:26386355

  19. Significant Correlation between Retinal Venous Tortuosity and Aqueous Vascular Endothelial Growth Factor Concentration in Eyes with Central Retinal Vein Occlusion

    PubMed Central

    Yasuda, Shunsuke; Kachi, Shu; Kondo, Mineo; Ueno, Shinji; Kaneko, Hiroki; Terasaki, Hiroko

    2015-01-01

    Purpose To determine whether the degree of venous tortuosity is significantly correlated with the aqueous vascular endothelial growth factor (VEGF) concentration in eyes with a central retinal vein occlusion (CRVO). Methods We reviewed the medical records of 32 eyes of 32 patients who had macular edema due to a CRVO. All of the patients were examined at the Nagoya University Hospital and were scheduled to receive an intravitreal injection of bevacizumab (IVB) or ranibizumab (IVR) within 12 weeks of the onset of the CRVO to treat the macular edema. Aqueous humor was collected just before the IVB or IVR, and the VEGF concentration was determined by enzyme-linked immunosorbent assay (ELISA). The venous tortuosity index was calculated by dividing the length of the retinal veins by the chord length of the same segment. The correlation between the mean tortuosity index of the inferotemporal and supratemporal branches of the retinal vein and the aqueous VEGF concentration was determined. Results The mean aqueous VEGF concentration was 384 ± 312 pg/ml with a range of 90 to 1077 pg/ml. The degree of venous tortuosity was significantly correlated with the VEGF concentration in the aqueous. (r = 0.49, P = 0.004), with the foveal thickness (r = 0.40, P = 0.02), and with the best-corrected visual acuity (r = 0.38, P = 0.03). Conclusions The significant correlation between the aqueous VEGF concentration and the venous tortuosity indicates that the degree of retinal venous tortuosity can be used to identify eyes that are at a high risk of developing neovascularization. PMID:26214803

  20. OUABAIN- AND MARINOBUFAGENIN-INDUCED PROLIFERATION OF HUMAN UMBILICAL VEIN SMOOTH MUSCLE CELLS AND A RAT VASCULAR SMOOTH MUSCLE CELL LINE, A7R5

    Technology Transfer Automated Retrieval System (TEKTRAN)

    We studied the growth-promoting effects of 2 sodium pump-selective cardiotonic steroids, ouabain and marinobufagenin, on cultured cells from vascular smooth muscle (VSMCs) from human umbilical vein and a rat VSMC line, A7r5. Both ouabain and marinobufagenin activated proliferation of these cells in...

  1. The care of patients with varicose veins and associated chronic venous diseases: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum.

    PubMed

    Gloviczki, Peter; Comerota, Anthony J; Dalsing, Michael C; Eklof, Bo G; Gillespie, David L; Gloviczki, Monika L; Lohr, Joann M; McLafferty, Robert B; Meissner, Mark H; Murad, M Hassan; Padberg, Frank T; Pappas, Peter J; Passman, Marc A; Raffetto, Joseph D; Vasquez, Michael A; Wakefield, Thomas W

    2011-05-01

    The Society for Vascular Surgery (SVS) and the American Venous Forum (AVF) have developed clinical practice guidelines for the care of patients with varicose veins of the lower limbs and pelvis. The document also includes recommendations on the management of superficial and perforating vein incompetence in patients with associated, more advanced chronic venous diseases (CVDs), including edema, skin changes, or venous ulcers. Recommendations of the Venous Guideline Committee are based on the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system as strong (GRADE 1) if the benefits clearly outweigh the risks, burden, and costs. The suggestions are weak (GRADE 2) if the benefits are closely balanced with risks and burden. The level of available evidence to support the evaluation or treatment can be of high (A), medium (B), or low or very low (C) quality. The key recommendations of these guidelines are: We recommend that in patients with varicose veins or more severe CVD, a complete history and detailed physical examination are complemented by duplex ultrasound scanning of the deep and superficial veins (GRADE 1A). We recommend that the CEAP classification is used for patients with CVD (GRADE 1A) and that the revised Venous Clinical Severity Score is used to assess treatment outcome (GRADE 1B). We suggest compression therapy for patients with symptomatic varicose veins (GRADE 2C) but recommend against compression therapy as the primary treatment if the patient is a candidate for saphenous vein ablation (GRADE 1B). We recommend compression therapy as the primary treatment to aid healing of venous ulceration (GRADE 1B). To decrease the recurrence of venous ulcers, we recommend ablation of the incompetent superficial veins in addition to compression therapy (GRADE 1A). For treatment of the incompetent great saphenous vein (GSV), we recommend endovenous thermal ablation (radiofrequency or laser) rather than high ligation and inversion stripping

  2. Vascular resection in pancreatic adenocarcinoma with portal or superior mesenteric vein invasion

    PubMed Central

    Pan, Gang; Xie, Kun-Lin; Wu, Hong

    2013-01-01

    AIM: To evaluate long-term survival after the Whipple operation with superior mesenteric vein/portal vein resection (SMV/PVR) in relation to resection length. METHODS: We evaluated 118 patients who underwent the Whipple operation for pancreatic adenocarcinoma at our Department of Hepatobiliary Pancreatic Surgery between 2005 and 2010. Fifty-eight of these patients were diagnosed with microscopic PV/SMV invasion by frozen-section examination and underwent SMV/PVR. In 28 patients, the length of SMV/PVR was ≤ 3 cm. In the other 30 patients, the length of SMV/PVR was > 3 cm. Clinical and survival data were analyzed. RESULTS: SMV/PVR was performed successfully in 58 patients. There was a significant difference between the two groups (SMV/PVR ≤ 3 cm and SMV/PVR > 3 cm) in terms of the mean survival time (18 mo vs 11 mo) and the overall 1- and 3-year survival rates (67.9% and 14.3% vs 41.3% and 5.7%, P < 0.02). However, there was no significant difference in age (64 years vs 58 years, P = 0.06), operative time (435 min vs 477 min, P = 0.063), blood loss (300 mL vs 383 mL, P = 0.071) and transfusion volume (85.7 mL vs 166.7 mL, P = 0.084) between the two groups. CONCLUSION: Patients who underwent the Whipple operation with SMV/PVR ≤ 3 cm had better long-term survival than those with > 3 cm resection. PMID:24379594

  3. The value of primary vascular stents in management of early portal vein stenosis after liver transplantation.

    PubMed

    Chang, Wen-Tsan; Kuo, Yu-Ting; Lee, King-Teh; Shih, Ming-Chen; Huang, Jian-Wei; Su, Wen-Lung; Chen, Chau-Yun; Huang, Yu-Ling; Wang, Shen-Nien; Chuang, Shih-Chang; Kuo, Kung-Kai; Chen, Jong-Shyone

    2016-03-01

    If portal vein stenosis (PVS) occurs within 1 month after liver transplantation (LT), especially within 1 week, it can be catastrophic and result in rapid loss of the grafts and mortality. Although surgical treatments have been considered standard treatment for PVS, patients are usually unable to receive operations or re-transplantations, because of their critical conditions and a shortage of grafts. Recently, primary percutaneous transhepatic portal vein stents (PTPS) were suggested as alternative and less-invasive treatments of PVS. However, because lethal complications may follow these primary stent placements for patients in early stages after LT, primary PTPS placements for patients suffering PVS 1 month after LT has been suggested. From November 2009 to July 2015, 38 consecutive adult patients underwent LT at our institution. Among them, six recipients suffered PVS within 1 month after LT. Technical success was achieved in all six patients. Clinical success was obtained in two of the four patients suffering PVS within 1 week after LT, and in the other two patients suffering PVS>1 week after LT. All surviving patients and their grafts were in good condition, and their stents remained patent. Our experience showed that primary PTPS placements can be used to effectively treat patients with PVS encountered within 1 month, and even within 1 week, after LT with acceptable short-term results. However, possible fatal complications should be kept in mind. Long-term results of these procedures need further follow-up. PMID:27106002

  4. Don't all veins look alike? Comprehensively attending to diversity within the vascular surgical specialty.

    PubMed

    Hancock, Ange-Marie; Hancock, Charles R

    2010-04-01

    Prior research has established diversity as a topic of empirical analysis in the vascular surgery literature. Building on the work of previously published articles on diversity in the Journal of Vascular Surgery and elsewhere, this article engages in a broad discussion of diversity in two interrelated arenas: educational/workplace diversity and culturally competent care. Interdisciplinary review of the literature indicates that diversity is often thought of as an end-state to be accomplished. A more fruitful way to encompass the changing aspects of diversity work is to think of diversity as a set of processes that can be adjusted based on a set of interrelated goals that matter differently to different groups. In considering diversity as a process, an approach to diversity emerges that considers both independent effects of gender and race/ethnicity as well as interactive effects between the two variables to address future trends in medical education. Such trends are diagnosed and multiple courses of intervention are offered as reasonable options for future efforts. A comprehensive definition of diversity will be established in order to encompass two different arenas in which diversity concerns arise: educational diversity and culturally competent patient care. Second, a discussion of the rationales for attention to diversity among vascular surgeons will provide different avenues into a conversation about diversity in the profession. In so doing, three successful efforts will be briefly discussed: the Ohio State University's MED-Path program, the Keck School of Medicine's chair-centered approach to diversity in residency training, and the American Association of Orthopedic Surgeons' (AAOS) approach to culturally competent care. PMID:20346338

  5. Acquired vascular malformation in ruptured caesarean section scar: a rare cause of life-threatening vaginal bleeding

    PubMed Central

    Hilal, Kiran; Masroor, Imrana; Aziz, Aliya; Sayani, Raza

    2013-01-01

    Uterine arteriovenous malformations (AVM) are rare and potentially life-threatening lesions. Patients present with severe vaginal bleeding which usually does not respond to conservative management and on most of occasions requires radiological or surgical intervention. We report a case of acquired AVM in a ruptured caesarean section scar. The patient presented with life-threatening vaginal bleeding and was treated with gonadotropin releasing hormone agonist and subsequent angioembolisation coiling. PMID:23839607

  6. The Dural AV-Fistula (DAVF), the Most Frequent Acquired Vascular Malformation of the Central Nervous System (CNS).

    PubMed

    Wanke, I; Rüfenacht, D A

    2015-10-01

    Acquired arteriovenous malformations, such as is the case with dural arteriovenous fistulae (DAVF), are the consequence of a pathological new arterial ingrowth into venous spaces that reaches directly the venous lumen, without interposition of a capillary network, thereby creating an AV-shunt.The following concise text will provide elements in regards to diagnosis, indication for treatment discussion and choice of endovascular treatment (EVT) method. PMID:26308245

  7. Walter E. Dandy's contributions to vascular neurosurgery.

    PubMed

    Kretzer, Ryan M; Coon, Alexander L; Tamargo, Rafael J

    2010-06-01

    Although Walter E. Dandy (1886-1946) is appropriately credited with the first surgical clipping of an intracranial aneurysm in 1937--a procedure that established the modern field of vascular neurosurgery--his numerous other contributions to this specialty are not as well known. Dandy can be credited with the first detailed description of the vein of Galen malformation, the first description of x-ray visualization of an intracranial aneurysm, the first characterization of basilar artery dolichoectasia, and the publication of the first comprehensive operative case series of arteriovenous malformations, cavernous malformations, and developmental venous anomalies. In addition, Dandy performed the first surgical trapping of a cavernous internal carotid artery (ICA) aneurysm by clipping the supraclinoid ICA and ligating the cervical ICA, and he also executed the first intracranial surgical clipping of the ICA to treat a carotid-cavernous fistula. In this article the authors describe Dandy's contributions to the field of vascular neurosurgery. PMID:20515365

  8. Occlusion of Arteriovenous Fistulas of In-Situ Saphenous Vein Bypass Grafts Using the Amplatzer Vascular Plug 4: Initial Experience

    SciTech Connect

    Libicher, Martin; Reichert, V.; Schwabe, H.; Matoussevitch, V.; Gawenda, M.

    2011-06-15

    We examined the safety and efficacy of vessel occlusion of the Amplatzer Vascular Plug 4 (AVP-4) in patients with arteriovenous fistulas after in-situ saphenous vein bypass grafts. We treated 18 fistulas of seven patients (four women, mean {+-} standard deviation age 76 {+-} 7 years, range 63-88 years). All fistulas were detected within 14 days after surgery. Initial diagnosis and follow-up was established by sonography. We measured the diameter of the feeding vessel and the time of vessel occlusion after plug deployment. Additionally, we recorded procedure time and the dose area product. Additional interventional procedures were necessary in three patients. We successfully used 19 AVP-4 for occlusion of all fistulas without thromboembolic complications. There was no need for recapturing the device, and we did not observe dislocation. Mean occlusion time was 9.6 min (range 5-22 min). Mean diameter of the feeding vessels was 3.5 mm (range 2.6-5.1 mm). Plug sizes ranged from 4-8 mm (mean 5.5 mm) resulting in an oversizing of 33-88%. Mean procedure time for patients with and without additional intervention was 91 {+-} 38 min and 35 {+-} 18 min, respectively. Mean dose area product was 11,790 cGy/cm{sup 2} (range 1,850-23,500 cGy/cm{sup 2}). Permanent occlusion of the fistulas was confirmed by ultrasound after a mean follow-up of 4 months (1-6 months). Occlusion of arteriovenous fistulas with an AVP-4 seems to be effective and safe in patients with in-situ saphenous vein bypass grafts. The AVP-4 is well suited for this purpose because of the appropriate diameter of the feeding vessels.

  9. Evaluation of decellularized human umbilical vein (HUV) for vascular tissue engineering - comparison with endothelium-denuded HUV.

    PubMed

    Mangold, Silvia; Schrammel, Siegfried; Huber, Georgine; Niemeyer, Markus; Schmid, Christof; Stangassinger, Manfred; Hoenicka, Markus

    2015-01-01

    Human umbilical vessels have been recognized as a valuable and widely available resource for vascular tissue engineering. Whereas endothelium-denuded human umbilical veins (HUVs) have been successfully seeded with a patient-derived neoendothelium, decellularized vessels may have additional advantages, due to their lower antigenicity. The present study investigated the effects of three different decellularization procedures on the histological, mechanical and seeding properties of HUVs. Vessels were decellularized by detergent treatment (Triton X-100, sodium deoxycholate, IGEPAL-CA630), osmotic lysis (3 m NaCl, distilled water) and peroxyacetic acid treatment. In all cases, nuclease treatments were required to remove residual nucleic acids. Decellularization resulted in a partial loss of fibronectin and laminin staining in the subendothelial layer and affected the appearance of elastic fibres. In addition to removing residual nucleic acids, nuclease treatment weakened all stainings and substantially altered surface properties, as seen in scanning electron micrographs, indicating additional non-specific effects. Detergent treatment and osmotic lysis caused failure stresses to decrease significantly. Although conditioned medium prepared from decellularized HUV did not severely affect endothelial cell growth, cells seeded on decellularized HUV did not remain viable. This may be attributed to the partial removal of essential extracellular matrix components as well as to changes of surface properties. Therefore, decellularized HUVs appear to require additional modifications in order to support successful cell seeding. Replacing the vessels' endothelium may thus be a superior alternative to decellularization when creating tissue-engineered blood vessels with non-immunogenic luminal interfaces. PMID:23038628

  10. Branch retinal vein occlusion.

    PubMed

    Hamid, Sadaf; Mirza, Sajid Ali; Shokh, Ishrat

    2008-01-01

    Retinal vein occlusions (RVO) are the second commonest sight threatening vascular disorder. Branch retinal vein occlusion (BRVO) and central retinal vein occlusion (CRVO) are the two basic types of vein occlusion. Branch retinal vein occlusion is three times more common than central retinal vein occlusion and- second only to diabetic retinopathy as the most common retinal vascular cause of visual loss. The origin of branch retinal vein occlusion undoubtedly includes both systemic factors such as hypertension and local anatomic factors such as arteriovenous crossings. Branch retinal vein occlusion causes a painless decrease in vision, resulting in misty or distorted vision. Current treatment options don't address the underlying aetiology of branch retinal vein occlusion. Instead they focus on treating sequelae of the occluded venous branch, such as macular oedema, vitreous haemorrhage and traction retinal detachment from neovascularization. Evidences suggest that the pathogenesis of various types of retinal vein occlusion, like many other ocular vascular occlusive disorders, is a multifactorial process and there is no single magic bullet that causes retinal vein occlusion. A comprehensive management of patients with retinal vascular occlusions is necessary to correct associated diseases or predisposing abnormalities that could lead to local recurrences or systemic event. Along with a review of the literature, a practical approach for the management of retinal vascular occlusions is required, which requires collaboration between the ophthalmologist and other physicians: general practitioner, cardiologist, internist etc. as appropriate according to each case. PMID:19385476

  11. Vascular malformation with phleboliths involving the parotid gland: A case report with a review of the literature.

    PubMed

    Ho, Caleb; Judson, Benjamin L; Prasad, Manju L

    2015-01-01

    Phleboliths within the parotid gland are exceedingly rare. We report a case of a venous malformation with multiple phleboliths that involved the left parotid gland and extended into the extraparotid tissue in a 43-year-old woman. We also review 13 similar cases that have been reported since 1948, and we highlight the significance of distinguishing phleboliths from sialoliths because management of the two can be entirely different. Phleboliths in and around the salivary glands primarily affect the parotid and submandibular glands in adult women. Patients can present with recurrent, intermittent swelling that may be associated with food intake and hyposalivation. Any sialolithiasis located outside of its most common location in the submandibular glands must be investigated thoroughly before surgery. PMID:26535823

  12. Galenic arteriovenous malformation with precocious puberty.

    PubMed

    Ventureyra, E C; Badejo, A

    1984-01-01

    Pineal lesions may appear with precocious puberty. In this report, a patient with precocious puberty and macrogenitosomia caused by an arteriovenous malformation in the pineal region is presented. This vascular malformation was not visualized during investigations 3 years before the present series. It appears that the vascular malformation increased considerably in size within a 3-year period. This case suggests that some arteriovenous malformations may take a malignant course, increasing rapidly in size and behaving like tumors by causing destruction and compression of surrounding structures. This case seems to be unique because, to the best of our knowledge, an arteriovenous malformation associated with precocious puberty has never been described previously. PMID:6689808

  13. Amphibian malformations

    USGS Publications Warehouse

    National Wildlife Health Center

    1998-01-01

    Frog malformations have been reported from 42 states. The broad geographic distribution of these malformations warrants national attention. Scientists at the USGS National Wildlife Health Center in Madison, Wisconsin are studying this problem in an effort to document its scope and to determine the causes of the observed malformations.

  14. 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

  15. Genetic basis for vascular anomalies.

    PubMed

    Kirkorian, A Yasmine; Grossberg, Anna L; Püttgen, Katherine B

    2016-03-01

    The fundamental genetics of many isolated vascular anomalies and syndromes associated with vascular anomalies have been elucidated. The rate of discovery continues to increase, expanding our understanding of the underlying interconnected molecular pathways. This review summarizes genetic and clinical information on the following diagnoses: capillary malformation, venous malformation, lymphatic malformation, arteriovenous malformation, PIK3CA-related overgrowth spectrum (PROS), Proteus syndrome, SOLAMEN syndrome, Sturge-Weber syndrome, phakomatosis pigmentovascularis, congenital hemangioma, verrucous venous malformation, cutaneomucosal venous malformation, blue rubber bleb nevus syndrome, capillary malformation-arteriovenous malformation syndrome, Parkes-Weber syndrome, and Maffucci syndrome. PMID:27607321

  16. Orthopedic issues in vascular anomalies.

    PubMed

    Spencer, Samantha A; Sorger, Joel

    2014-08-01

    Vascular malformations impact the musculoskeletal system depending on the tissue involved (skin, subcutis, muscle, cartilage, or bone), the extent of involvement, and the type of anomalous vessels (arteries, capillaries, veins, or lymphatics). These malformations can cause a multitude of musculoskeletal problems for the patient and their Orthopedic Surgeon to manage. Leg-length discrepancy, intra-articular involvement, muscular lesions, and primary or secondary scoliosis are just to name a few. All of these problems can cause pain, deformity, and a range of functional limitations. Surgical and nonsurgical treatment plans both have a role in the care of these patients. Patients with vascular malformations may also suffer from life-threatening cardiovascular and hematologic abnormalities. For those patients who undergo surgery, thromboembolic risk is elevated, wound breakdown and infection are much more common, and bleeding risk continues well into the postoperative course. Because of the complex nature of these disorders, the clinician must have a full understanding of the types of lesions, their natural history, appropriate diagnostic studies, associated medical problems, indications for treatment, and all the treatment options. For severe malformations, especially syndromes such as CLOVES and Klippel-Trenaunay syndrome, interdisciplinary team management is essential for the best outcomes. PMID:25241103

  17. Arteriovenous malformation of the pancreas: a case report.

    PubMed

    Abe, Tsuyoshi; Suzuki, Nobuyasu; Haga, Junichirou; Azami, Ayaka; Todate, Yukitoshi; Waragai, Mitsuru; Sato, Atai; Takano, Yoshinao; Kawakura, Kenji; Imai, Shigeki; Sakuma, Hideo; Teranishi, Yasushi

    2016-12-01

    Arteriovenous malformation (AVM) of the pancreas is uncommon in the gastrointestinal tract. We present a case of AVM of the pancreatic head in a 59-year-old male. He was admitted to a hospital with hematemesis and tarry stool and referred to our hospital in March 2014 on the diagnosis of pancreatic artery pseudoaneurysm. A computed tomography scan showed the presence of irregular dilated and/or stenotic vessels with meandering in the pancreatic head. Magnetic resonance imaging showed strong enhancement of the conglomeration in the pancreatic head. Selective angiography showed the proliferation of a vascular network in the pancreatic head and an early visualization of the portal vein at the arterial phase. The patient qualified for surgery with a preoperative diagnosis of AVM of the pancreatic head. We performed pylorus-preserving pancreaticoduodenectomy. The histological results confirmed the presence of irregular dilated tortuous arteries and veins in the pancreatic head. Surgical treatment may represent definitive management of symptomatic AVM. PMID:26943682

  18. Developmental venous anomaly, capillary telangiectasia, cavernous malformation, and arteriovenous malformation: spectrum of a common pathological entity?

    PubMed

    Kalani, M Yashar S; Zabramski, Joseph M; Martirosyan, Nikolay L; Spetzler, Robert F

    2016-03-01

    Vascular malformations of the central nervous system are thought to originate from abnormal developmental processes during embryogenesis. Reports have cited the dynamic nature of these lesions and their "maturation" into other types of malformations. Herein we report on three patients with vascular malformations who exhibited dynamic alterations with stepwise progression of their lesions. These cases lend support to the hypothesis that these malformations may constitute the spectrum of a single disease caused by alterations in a common developmental program and that accumulating injury (e.g., by radiation) may allow one malformation type to mature into another. This concept warrants further investigation. PMID:26743915

  19. Inferior mesenteric vein serves as an alternative guide for transection of the pancreatic body during pancreaticoduodenectomy with concomitant vascular resection: a comparative study evaluating perioperative outcomes

    PubMed Central

    2014-01-01

    Background Tumors of the pancreatic head often involve the superior mesenteric and portal veins. The purpose of this study was to assess perioperative outcomes after pancreaticoduodenectomy (PD) with concomitant vascular resection using the inferior mesenteric vein (IMV) as a guide for transection of the pancreatic body (Whipple at IMV, WATIMV). Methods One hundred thirty-seven patients had segmental vein resection during PD between January 2006 and June 2013. Depending on whether the standard approach of creating a tunnel anterior to the mesenterico-portal vein (MPV) axis was achieved for pancreatic transection, patients were subjected to a standard PD with vein resection procedure (s-PD + VR, n = 75) or a modified procedure (m-PD + VR, n = 62). Within the m-PD + VR group, 28 patients underwent the WATIMV procedure, while 34 patients underwent the usual procedure of transection, or ‘central pancreatectomy’ (c-PD + VR). Results The volume of intraoperative blood loss and the blood transfusion requirements were significantly greater, and the venous wall invasion and neural invasion frequency were significantly higher in the m-PD + VR group compared with the s-PD + VR group. There were no significant differences in the length of hospitalization, postoperative morbidity, and grades of complications between the two groups. Multivariate logistic regression identified intraoperative blood transfusion (P = 0.004) and vascular invasion (P = 0.008) as the predictors of postoperative morbidity. Further stratification of the entire cohort of 62 (45%) patients who underwent m-PD + VR showed a higher rate of negative resection margins (96.4%) in the WATIMV group compared with the c-PD + VR group (76.5%) (P = 0.06). The volume of intraoperative blood loss (P = 0.013), and intraoperative blood transfusion requirements (P = 0.07) were significantly greater in the c-PD + VR group compared with the WATIMV group

  20. [Combined treatment of arteriovenous malformations of the head and neck].

    PubMed

    Galich, S P; Dabizha, A Iu; Gindich, O A; Ogorodnik, Ia P; Al'tman, I V; Gomoliako, I V; Guch, A A

    2015-01-01

    An arteriovenous malformation (AVM) is a vascular developmental abnormality conditioned by impaired embryonic morphogenesis and characterized by the development of an abnormal connection between arteries and veins. More than 50% of the total number of patients suffering from this pathology are those having the pathological foci localizing in the area of the head and neck. At present, a combined method is both a generally accepted and the most radical one used for treatment for AVM. However, in the majority of cases, excision of the malformation leaves an extensive and complicated defect of tissues, whose direct closure leads to coarse cicatricious deformities. Over the period from 2004 to 2012, we followed up a total of 37 patients presenting with arteriovenous malformations of the head and neck. At admission the patients underwent preoperative examination including clinical tests, ultrasound duplex scanning, arteriography, MRT, and computed tomography. 24-72 hours prior to the operative intervention the patients were subjected to embolisation of the main vessels supplying the vascular malformation. Excision of the AVM was in 8 cases followed by primary closure of the postoperative wound, in 17 patients the defect was closed by transposition of the axial flaps, and 12 subjects underwent free transplantation of composite complexes of tissues. Relapse of the disease was revealed in 17 patients. In the majority of cases, relapses developed during the first year after the operative intervention (10 cases). The control of the disease's course was obtained in 20 patients. In 8 of the 12 patients with free transplantation of flaps we managed to obtain long-term control over the disease's course (more than 5 years). Hence, free microsurgical transplantation of compound complexes of tissues may be considered as a method of choice for closing the defect after excising an AVM in the area of the head and neck. Replacement of the defect with a well-vascularized tissue complex

  1. [Ultrasound mapping of lower-limb vascular system with regard to occurrence and anatomy of additional front great saphenous vein].

    PubMed

    Musil, D; Herman, J

    2006-12-01

    A primary survey study involving a set of 113 lower limbs of 66 patients was focused on the prevalence of additional front great saphenous vein (AFGSV) and the measurement of the distance between its start in the great saphenous vein (GSV) and the saphenofemoral junction (SFJ), the measurement of the distance between the passage of AFGSV through the saphenous fascia and its start in GSV, and compared the width of AFGSV anechogenous lumen with the calibre of GSV. The prevalence of AFGSV in the observed set was 42.5%. Reflux was proved in 6 AFGSVs (12.5%). The width of the AFGSV anechogenous lumen was on an average 0.29 cm (M 0.26 cm, SD +/- 0.15 cm), and the width of GSV anechogenous lumen measured on lower limbs with simultaneous occurrence of AFGSV was 0.38 cm (M 0.38 cm, SD +/- 0.11 cm) on an average. Comparison of the anechogenous lumen width of the two veins showed a statistically significant difference (p < 0,001). AFGSV in the observed set of lower limbs started in VS at an average distance of 0.8 cm from the saphenofemoral junction (M 0.8 cm, SD +/- 0.46 cm). The minimum start point distance was 0.1 cm, the maximum was 1.84 cm. In 3 cases (6.3%), AFGSV started directly in the common femoral vein (v. femoralis communis), which was the place where also the saphenous vein begins. The distance between the passage of AFGSV through saphenous fascia to upper subcutaneous layers and the start of the vein in GSV was on an average 13.2 cm (M 14.5 cm, SD +/- 6.5 cm). The maximum distance between the passage of AFGSV through saphenous fascia and the start of the vein in GSV was 4 cm, the maximum distance being 30 cm. PMID:17299907

  2. Control of proliferation of human vascular endothelial cells. Characterization of the response of human umbilical vein endothelial cells to fibroblast growth factor, epidermal growth factor, and thrombin.

    PubMed

    Gospodarowicz, D; Brown, K D; Birdwell, C R; Zetter, B R

    1978-06-01

    Because the response of human endothelial cells to growth factors and conditioning agents has broad implications for our understanding of wound healing angiogenesis, and human atherogenesis, we have investigated the responses of these cells to the fibroblast (FGF) and epidermal growth factors (EGF), as well as to the protease thrombin, which has been previously shown to potentiate the growth response of other cell types of FGF and EGF. Because the vascular endothelial cells that form the inner lining of blood vessels may be expected to be exposed to high thrombin concentrations after trauma or in pathological states associated with thrombosis, they are of particular interest with respect to the physiological role of this protease in potentiating cell proliferation. Our results indicate that human vascular endothelial cells respond poorly to either FGF or thrombin alone. In contrast, when cells are maintained in the presence of thrombin, their proliferative response to FGF is greatly increased even in cultures seeded at a density as low as 3 cells/mm2. Human vascular endothelial cells also respond to EGF and thrombin, although their rate of proliferation is much slower than when maintained with FGF and thrombin. In contrast, bovine vascular endothelial cells derived from vascular territories as diverse as the bovine heart, aortic arch, and umbilical vein respond maximally to FGF alone and neither respond to nor bind EGF. Furthermore, the response of bovine vascular endothelial cells to FGF was not potentiated by thrombin, indicating that the set of factors controlling the proliferation of vascular endothelial cells could be species-dependent. The requirement of cultured human vascular endothelial cells for thrombin could explain why the human cells, in contrast to bovine endothelial cells, are so difficult to maintain in tissue culture. Our results demonstrate that by using FGF and thrombin one can develop cultures of human vascular endothelial cells capable of

  3. Acquired pulmonary arteriovenous malformation secondary to hydatid cyst operation.

    PubMed

    Gezer, S; Turut, H; Oz, G; Demirag, F; Tastepe, I

    2007-10-01

    Pulmonary arteriovenous malformations are abnormal communications between pulmonary arteries and pulmonary veins. The majority of the cases are congenital in origin, and acquired pulmonary arteriovenous malformations are very rare. We present a case here, which - to the best of our knowledge - is the first acquired pulmonary arteriovenous malformation secondary to a hydatid cyst operation in the literature, and we discuss the etiology, clinical presentation, diagnostic modalities and treatment of acquired pulmonary arteriovenous malformations. PMID:17902072

  4. Change in vascular smooth muscle response to 5-HT due to short- or long-term endothelial denudation of the bovine digital vein.

    PubMed

    Punzi, Simona; Belloli, Chiara; Gogny, Marc; Desfontis, Jean-Claude; Mallem, Mohamed Y

    2016-01-01

    Several chronic progressive vascular diseases, such as laminitis, show vasocontractile dysfunction that might evolve into reperfusion injury and/or vessel structural remodelling, which may be traced back to aberrant endothelial function. In the present study, the vasomotor responses of bovine digital veins (BDVs) to 5-hydroxytryptamine (5-HT) were investigated in blood vessels, with and without endothelium present, and in samples deprived of endothelium before or after overnight incubation in tissue culture medium, to evaluate the effects of short- and long-term endothelial damage on vascular smooth muscle (VSM) reactivity. No significant effects were observed in the blood vessels tested immediately after the removal of endothelium. In contrast, a significant increase in VSM reactivity to 5-HT was seen in vessels incubated without endothelium. This long-term change in smooth muscle reactivity was prevented by exposure to the nitric oxide (NO) donor nitroprusside (P < 0.01), suggesting that the long-term lack of inhibitory control exerted by endothelium-derived NO is involved in increased VSM reactivity. The RhoA/ROCK pathway inhibitor fasudil reduced VSM hyper-contractility to ~65% (P < 0.001), the superoxide dismutase-mimetic tempol normalised the vascular response and the non-selective COX-inhibitor indomethacin exerted a moderate inhibitory effect (P < 0.05). Thus, over-activation of the RhoA/ROCK pathway and production of reactive oxygen species could account for VSM hyper-reactivity, triggered by long-term endothelium-deprivation in BDVs, suggesting that these biochemical mechanisms are potential targets for controlling the progressive vasocontractile dysfunction of digital veins in animals affected with laminitis. PMID:26670334

  5. Hepatic angiomyolipoma: differential diagnosis from other liver tumors in a special reference to vascular imaging - importance of early drainage vein.

    PubMed

    Yoshioka, Masato; Watanabe, Go; Uchinami, Hiroshi; Kudoh, Kazuhiro; Hiroshima, Yuko; Yoshioka, Toshiaki; Nanjo, Hiroshi; Funaoka, Masato; Yamamoto, Yuzo

    2015-12-01

    A 51-year-old female had been diagnosed with a hemangioma in the hepatic segment 6 (S6). After a 6-year follow-up, enlargement of the tumor was detected. The tumor was clearly enhanced in the arterial phase, and the enhancement remained in the portal phase on computed tomography (CT). Although the primary differential diagnosis on CT was hepatocellular carcinoma (HCC), we worried about the possibility of other vessel system tumors because the tumor remained to be enhanced at the portal phase for HCC and all tumor markers of HCC were negative. We performed angiography to determine the tumor nature and to seek other tumors. Angiography showed tumor stain at the hepatic S6 with an early obvious drainage vein from the tumor flowing through the right hepatic vein into the inferior vena cava. In addition to tumor stain and the drainage vein, there were many small poolings of contrast medium in the whole liver, which were suspected as dilatation of the hepatic peripheral artery. We suspected the tumor as a benign tumor such as hepatocellular adenoma or focal nodular hyperplasia, but the possibility of HCC could not be ruled out. Hepatic posterior sectionectomy was done to completely remove the drainage vein with the tumor. Intraoperative histological examination revealed the tumor as not malignant and not HCC. Later, immunohistochemical analysis uncovered that the tumor had high expression of HMB-45 and, therefore, the final diagnosis was angiomyolipoma. We think that detecting an early drainage vein from the tumor would be a key point for diagnosing hepatic angiomyolipoma. PMID:26943379

  6. Photopatterning of vascular endothelial growth factor within collagen-glycosaminoglycan scaffolds can induce a spatially confined response in human umbilical vein endothelial cells.

    PubMed

    Alsop, Aurora T; Pence, Jacquelyn C; Weisgerber, Daniel W; Harley, Brendan A C; Bailey, Ryan C

    2014-11-01

    Biomolecular signals within the native extracellular matrix are complex, with bioactive factors found in both soluble and sequestered states. In the design of biomaterials for tissue engineering applications it is increasingly clear that new approaches are required to locally tailor the biomolecular environment surrounding cells within the matrix. One area of particular focus is strategies to improve the speed or quality of vascular ingrowth and remodeling. While the addition of soluble vascular endothelial growth factor (VEGF) has been shown to improve vascular response, strategies to immobilize such signals within a biomaterial offer the opportunity to optimize efficiency and to explore spatially defined patterning of such signals. Here we describe the use of benzophenone (BP) photolithography to decorate three-dimensional collagen-glycosaminoglycan (CG) scaffolds with VEGF in a spatially defined manner. In this effort we demonstrate functional patterning of a known agonist of vascular remodeling and directly observe phenotypic effects induced by this immobilized cue. VEGF was successfully patterned in both stripes and square motifs across the scaffold with high specificity (on:off pattern signal). The depth of patterning was determined to extend up to 500 μm into the scaffold microstructure. Notably, photopatterned VEGF retained native functionality as it was shown to induce morphological changes in human umbilical vein cells indicative of early vasculogenesis. Immobilized VEGF led to greater cell infiltration into the scaffold and the formation of immature vascular network structures. Ultimately, these results suggest that BP-mediated photolithography is a facile method to spatially control the presentation of instructive biological cues to cells within CG scaffolds. PMID:25016280

  7. Chiari Malformation

    MedlinePlus

    Chiari malformations (CMs) are structural defects in the cerebellum. The cerebellum is the part of the brain that controls balance. With CM, brain tissue extends into the spinal canal. It can happen when part of the skull is too ...

  8. Arteriovenous Malformation

    MedlinePlus

    ... to the formation of brain AVMs. NIH Patient Recruitment for Arteriovenous Malformation Clinical Trials At NIH Clinical Center Throughout the U.S. and Worldwide NINDS Clinical Trials Organizations Column1 Column2 Brain Aneurysm Foundation 269 Hanover Street, ...

  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. Intraneural Venous Malformations of the Median Nerve

    PubMed Central

    González Rodríguez, Alba; Midón Míguez, José

    2016-01-01

    Venous malformations arising from the peripheral nerve are a rare type of vascular malformation. We present the first case of an intraneural venous malformation of the median nerve to be reported in a child and review the previous two cases of median nerve compression due to a venous malformation that have been reported. These cases presented with painless masses in the volar aspect of the wrist or with symptoms suggestive of carpal tunnel syndrome. Clinical suspicion should lead to the use of Doppler ultrasonography as the first-line diagnostic tool. Magnetic resonance imaging and histopathology can confirm the diagnosis, as phleboliths are pathognomonic of venous malformations. Surgical treatment appears to be the only modality capable of successfully controlling the growth of an intraneural malformation. Sclerotherapy and radiotherapy have never been used to treat this type of malformation. PMID:27462571

  11. Classification of vascular disorders in the skin and selected data on new evaluation and treatment.

    PubMed

    Wollina, Uwe; Unger, Leonore; Haroske, Gunter; Heinig, Birgit

    2012-01-01

    Cutaneous vascular disorders are common. They include arteries, veins, and lymphatic vessels, or a mixture of them. In this review, we discuss classification, new developments in understanding and treatment of vascular diseases. We focus on infantile hemangiomas and drug therapy, vasculitides with new vasculitic syndromes, yellow nail syndrome and localized lymphatic malformations. Benign cutaneous vascular lesions may be a sign of severe internal diseases. In many cases multidisciplinary treatment is important. The dermatologist can often act as a pilot for these patients. PMID:22950555

  12. Varicose Veins

    MedlinePlus

    Varicose veins are swollen, twisted veins that you can see just under the skin. They usually occur in ... of the body. Hemorrhoids are a type of varicose vein. Your veins have one-way valves that help ...

  13. Mitochondrial Ca²⁺ handling is crucial for generation of rhythmical Ca²⁺ waves in vascular interstitial cells from rabbit portal vein.

    PubMed

    Harhun, Maksym I

    2015-09-01

    Vasomotion is the rhythmical changes in vascular tone of various blood vessels. It was proposed that in rabbit portal vein (RPV) the spontaneous contractile activity is driven by vascular interstitial cells (VICs), since RPV VICs generate rhythmical changes in intracellular Ca(2+) concentration ([Ca(2+)]i) associated with membrane depolarisation in these cells. In this work, using confocal imaging in Fluo-3 loaded RPV VICs we studied if generation of rhythmical [Ca(2+)]i changes is affected when Ca(2+) handling by mitochondria is compromised. We also visualised mitochondria in VICs using Mito Tracker Green fluorescent dye. Our results showed that freshly dispersed RPV VICs generated rhythmical [Ca(2+)]i oscillations with a frequency of 0.2-0.01 Hz. Imaging of VICs stained with Mito Tracker Green revealed abundant mitochondria in these cells with a higher density of the organelles in sub-plasmalemmar region compared to the central region of the cell. Oligomycin, an ATP synthase inhibitor, did not affect the amplitude and frequency of rhythmical [Ca(2+)]i oscillations. In contrast, two uncoupling agents, carbonylcyanide-3-chlorophenylhydrazone (CCCP) and carbonylcyanide-4-trifluoromethoxyphenylhydrazone (FCCP) effectively abolished rhythmical [Ca(2+)]i changes with simultaneous increase in basal [Ca(2+)]i in RPV VICs. These data suggest that in RPV VICs mitochondrial Ca(2+) handling is important for the generation of rhythmical [Ca(2+)]i changes which underlie the spontaneous rhythmical contractile activity in this vessel. PMID:26104918

  14. Chiari Malformation

    MedlinePlus

    ... Ones & When? Smart School Lunches Emmy-Nominated Video "Cerebral Palsy: Shannon's Story" 5 Things to Know About Zika & ... spine that can lead to nerve damage or paralysis. Chiari malformations also can happen after brain or spinal surgery. Doctors haven't identified a ...

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

    SciTech Connect

    Lv, Ming-ming; Fan, Xin-dong; Su, Li-xin

    2013-10-15

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

  16. 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.

  17. [Medullary venous malformation with azygos anterior cerebral artery aneurysm: a case report].

    PubMed

    Harada, K; Kobayashi, S; Sigemori, M; Watanabe, M; Kuramoto, S

    1987-03-01

    A case of medullary venous malformation with azygos anterior cerebral artery aneurysm is reported, which was associated with the leptomenigeal angiomatosis on the cortex of the right frontal lobe. A 62-year-old female was admitted to the Kurume University Hospital on June 21, 1985, because of a convulsive seizure. On admission, neurological and general examinations, except for slightly liver function disorder, was no abnormality. Plain CT scan showed the cortical atrophy and two small high-density areas in the right frontal lobe. Enhanced CT scan showed a high-density area in the genu of the corpus callosum. MRI (magnetic resonance imaging), on T2 weighted image, showed a high signal intensity area in the right paraventricular deep white matter and the right frontal region. A right internal carotid angiogram in the venous phase showed numerous enlarged medullary veins in the deep frontal region, converging into a single large draining vein that empties into the basal vein of Rosenthal. An azygos anterior cerebral artery was visualized on the right and left carotid angiograms. The aneurysm was situated at the distal end of the azygos artery. Twenty eight days after admission, a right frontal cranioplastic craniotomy was performed. During operation, the surface of the right frontal lobe was covered by fine, vascular networks, which was reddish brown. A right frontal lobectomy, including venous malformation and vascular networks, was performed. The aneurysm was clipped via an interhemispheric approach. Histologically, the malformation vessels had spread into the subarachnoid space.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3600991

  18. Vascular Injury Post Stent Implantation: Different Gene Expression Modulation in Human Umbilical Vein Endothelial Cells (HUVECs) Model

    PubMed Central

    Campolo, Jonica; Vozzi, Federico; Penco, Silvana; Cozzi, Lorena; Caruso, Raffaele; Domenici, Claudio; Ahluwalia, Arti; Rial, Michela; Marraccini, Paolo; Parodi, Oberdan

    2014-01-01

    To explore whether stent procedure may influence transcriptional response of endothelium, we applied different physical (flow changes) and/or mechanical (stent application) stimuli to human endothelial cells in a laminar flow bioreactor (LFB) system. Gene expression analysis was then evaluated in each experimental condition. Human umbilical vein endothelial cells (HUVECs) were submitted to low and physiological (1 and 10 dyne/cm2) shear stress in absence (AS) or presence (PS) of stent positioning in a LFB system for 24 h. Different expressed genes, coming from Affymetrix results, were identified based on one-way ANOVA analysis with p values <0.01 and a fold changed >3 in modulus. Low shear stress was compared with physiological one in AS and PS conditions. Two major groups include 32 probes commonly expressed in both 1AS versus 10AS and 1PS versus 10PS comparison, and 115 probes consisting of 83 in addition to the previous 32, expressed only in 1PS versus 10PS comparison. Genes related to cytoskeleton, extracellular matrix, and cholesterol transport/metabolism are differently regulated in 1PS versus 10PS condition. Inflammatory and apoptotic mediators seems to be, instead, closely modulated by changes in flow (1 versus 10), independently of stent application. Low shear stress together with stent procedure are the experimental conditions that mainly modulate the highest number of genes in our human endothelial model. Those genes belong to pathways specifically involved in the endothelial dysfunction. PMID:24587287

  19. Portal Vein Embolization Using a Nitinol Plug (Amplatzer Vascular Plug) in Combination with Histoacryl Glue and Iodinized Oil: Adequate Hypertrophy with a Reduced Risk of Nontarget Embolization

    SciTech Connect

    Bent, Clare L. Low, Deborah; Matson, Matthew B.; Renfrew, Ian; Fotheringham, Tim

    2009-05-15

    The purpose of this study was to assess whether portal vein embolization (PVE) using a nitinol vascular plug in combination with histoacryl glue and iodinized oil minimizes the risk of nontarget embolization while obtaining good levels of future liver remnant (FLR) hypertrophy. Between November 2005 and August 2008, 16 patients (8 females, 8 males; mean age, 63 {+-} 3.6 years), each with a small FLR, underwent right ipsilateral transhepatic PVE prior to major hepatectomy. Proximal PVE was initially performed by placement of a nitinol vascular plug, followed by distal embolization using a mixture of histoacryl glue and iodinized oil. Pre- and 6 weeks postprocedural FLR volumes were calculated using computed tomographic imaging. Selection for surgery required an FLR of 0.5% of the patient's body mass. Clinical course and outcome of surgical resection for all patients were recorded. At surgery, the ease of hepatectomy was subjectively assessed in comparison to previous experience following PVE with alternative embolic agents. PVE was successful in all patients. Mean procedure time was 30.4 {+-} 2.5 min. Mean absolute increase in FLR volume was 68.9% {+-} 12.0% (p = 0.00005). There was no evidence of nontarget embolization during the procedure or on subsequent imaging. Nine patients proceeded to extended hepatectomy. Six patients demonstrated disease progression. One patient did not achieve sufficient hypertrophy in relation to body mass to undergo hepatic resection. At surgery, the hepatobiliary surgeons observed less periportal inflammation compared to previous experience with alternative embolic agents, facilitating dissection at extended hepatectomy. In conclusion, ipsilateral transhepatic PVE using a single nitinol plug in combination with histoacryl glue and iodinized oil simplifies the procedure, offering short procedural times with minimal risk of nontarget embolization. Excellent levels of FLR hypertrophy are achieved enabling safe extended hepatectomy.

  20. Transvenous Embolization for Carotid-Cavernous Fistula in a Patient with Vascular Type of Ehlers-Danlos Syndrome—Direct Superior Ophthalmic Vein Approach: Case Report

    PubMed Central

    TANAKA, Teppei; HAYAKAWA, Motoharu; SADATO, Akiyo; ADACHI, Kazuhide; WATABE, Takeya; MAEDA, Shingo; OHMURA, Masahiro; HIROSE, Yuichi

    2014-01-01

    The vascular type of Ehlers-Danlos syndrome (vEDS) is an autosomal dominant hereditary disease characterized by connective tissue fragility throughout the body, including the arteries, viscera, and gastrointestinal tract. We report a case in which we performed transvenous embolization (TVE) via direct superior ophthalmic vein (SOV) approach to treat a direct carotid-cavernous fistula (CCF) in a patient with Ehlers-Danlos syndrome (EDS). The patient was a 37-year-old woman who developed tinnitus in her left ear and a headache during examination in the outpatient clinic of another hospital in order to make a definitive diagnosis of vEDS, and she was referred to our hospital and examined. Based on the results of all of the studies she was diagnosed with a CCF. Conservative treatment was attempted, but was not very effective. Because of progressing aphasia, TVE was performed via the SOV direct cut. There were no intraoperative or postoperative complications. It has been reported that cerebral angiography is generally contraindicated in vEDS and that the morbimortality associated with endovascular treatment is very high. When performing treatment it is necessary to be sufficiently aware of the risks it entails. PMID:24418783

  1. Acquired Jugular Vein Aneurysm

    PubMed Central

    Hopsu, Erkki; Tarkkanen, Jussi; Vento, Seija I.; Pitkäranta, Anne

    2009-01-01

    Venous malformations of the jugular veins are rare findings. Aneurysms and phlebectasias are the lesions most often reported. We report on an adult patient with an abruptly appearing large tumorous mass on the left side of the neck identified as a jugular vein aneurysm. Upon clinical examination with ultrasound, a lateral neck cyst was primarily suspected. Surgery revealed a saccular aneurysm in intimate connection with the internal jugular vein. Histology showed an organized hematoma inside the aneurysmal sac, which had a focally thinned muscular layer. The terminology and the treatment guidelines of venous dilatation lesions are discussed. For phlebectasias, conservative treatment is usually recommended, whereas for saccular aneurysms, surgical resection is the treatment of choice. While an exact classification based on etiology and pathophysiology is not possible, a more uniform taxonomy would clarify the guidelines for different therapeutic modalities for venous dilatation lesions. PMID:20107571

  2. Varicose Veins

    MedlinePlus

    ... page from the NHLBI on Twitter. What Are Varicose Veins? Español Varicose (VAR-i-kos) veins are swollen, ... can form in other parts of the body. Varicose veins are a common condition. They usually cause few ...

  3. Heart and vascular services

    MedlinePlus

    ... gov/ency/article/007459.htm Heart and vascular services To use the sharing features on this page, ... blood vessels (arteries and veins). Heart and vascular services refers to the branch of medicine that focuses ...

  4. Anorectal malformations

    PubMed Central

    Gangopadhyay, Ajay Narayan; Pandey, Vaibhav

    2015-01-01

    Anorectal malformations (ARMs) are among the more frequent congenital anomalies encountered in paediatric surgery, with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live births. Antenatal diagnosis of an isolated ARM is rare. Most cases are diagnosed in the early neonatal period. There is a wide spectrum of presentation ranging from low anomalies with perineal fistula having simple management to high anomalies with complex management. Advances in the imaging techniques with improvement in knowledge of the embryology, anatomy and physiology of ARM cases have refined diagnosis and initial management. There has been marked improvement in survival of such patient over the last century. The management of ARM has moved forward from classical procedures to PSARP to minimal invasive procedures. But still the fecal and urinary incontinence can occur even with an excellent anatomic repair, mainly due to associated problems. There has been a paradigm shift in approach to these patients which involves holistic approach to the syndrome of Anorectal malformations with a long term goal of achievement of complete fecal and urinary continence with excellent quality of life. PMID:25552824

  5. Endothelial cells in the context of brain arteriovenous malformations.

    PubMed

    Sammons, Vanessa; Davidson, Andrew; Tu, Jian; Stoodley, Marcus A

    2011-02-01

    A subset of brain arteriovenous malformations (AVM) cannot be treated using today's treatment paradigms. Novel therapies may be developed, however, as the underlying pathophysiology of these lesions becomes better understood. Endothelial cells (EC) are the subject of new biological therapies, such as radiosensitisation and vascular targeting. This work reviews the current research surrounding EC in the context of brain AVM, including both in vitro and AVM specimen analysis, with a particular focus on the effect of radiation on EC. EC are heterogeneous with no recognised common phenotype, which leads to difficulties in applying the results of the common studies using human umbilical vein endothelial cells to AVM research. Human brain EC are observed to have a high rate of proliferation and also have a reduced apoptotic response to inflammatory mediators such as transforming growth factor-beta. The angiogenic factors vascular endothelial growth factor and endothelin-1 (ET-1) are not normally produced by quiescent brain vasculature, but are produced by AVM EC. Radiation causes EC to separate and become disrupted. Leucocyte and platelet adherence is increased for several days post-irradiation due to increased E-selectin and P-selectin and intercellular adhesion molecule-1 expression. ET-1 is highly expressed in irradiated AVM EC. Radiosurgery produces local radiation-induced changes in EC, which may allow these changes to be harnessed in conjunction with other techniques such as vascular targeting. PMID:21167719

  6. 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

  7. Management of hemangiomas and other vascular tumors.

    PubMed

    Greene, Arin K

    2011-01-01

    Vascular tumors of childhood are typically benign. The 4 most common types are infantile hemangioma (IH), congenital hemangioma (CH), kaposiform hemangioendothelioma (KHE), and pyogenic granuloma (PG). Vascular tumors must be differentiated from vascular malformations. Although tumors and malformations may appear as raised, blue, red, or purple lesions, their management differs significantly. PMID:21095471

  8. The economics of vein disease.

    PubMed

    Sales, Clifford M; Podnos, Joan; Levison, Jonathan

    2007-09-01

    The management of cosmetic vein problems requires a very different approach than that for the majority of most other vascular disorders that occur in a vascular surgery practice. This article focuses on the business aspects of a cosmetic vein practice, with particular attention to the uniqueness of these issues. Managing patient expectations is critical to the success of a cosmetic vein practice. Maneuvering within the insurance can be difficult and frustrating for both the patient and the practice. Practices should use cost accounting principles to evaluate the success of their vein work. Vein surgery--especially if performed within the office--can undergo an accurate break-even analysis to determine its profitability. PMID:17911565

  9. Focus on Varicose Veins

    MedlinePlus

    ... Other veins often mistaken for varicose veins are spider veins and reticular veins, which are the visible ... greenish-blue veins that appear in our legs. Spider veins or teleangiectesias are tiny veins that you ...

  10. Combined neuroradiological and neurosurgical treatment of intracerebral arteriovenous malformations.

    PubMed

    Adelt, D; Brückmann, H; Krenkel, W; Hacke, W; Zeumer, H

    1988-07-01

    Four patients with intracerebral vascular malformations underwent preoperative butylcyanoacrylate embolization via a calibrated leak catheter, in order to reduce the risks of surgery alone. In three cases the malformation was removed without causing neurological deficits. One patient died later from recurrent bleeding. PMID:3171616

  11. A Meta-Analysis of Anti-Vascular Endothelial Growth Factor Remedy for Macular Edema Secondary to Central Retinal Vein Occlusion

    PubMed Central

    Huang, Peirong; Niu, Wenquan; Ni, Zhentian; Wang, Renzuo; Sun, Xiaodong

    2013-01-01

    Background Central retinal vein occlusion (CRVO) associates with severe vision outcome and no proven beneficial treatment. Our meta-analysis intended to appraise the efficacy and safety of anti-vascular endothelial growth factor (anti-VEGF) agents in macular edema (ME) following CRVO. Methods Data were collected and analyzed by Review Manager 5.2.1. We employed a random-effects model to eliminate between-study heterogeneity. Nfs (called fail-safe number) was calculated to evaluate the publication bias. Results We included 5 trials consisting 323 cases and 281 controls. Primary outcomes showed that overall comparison of anti-VEGF agents with placebo control yielded a 374% and 136% increased tendency for a gain of 15 letters or more on Early Treatment Diabetic Retinopathy Study (ETDRS) chart (95% confidence interval [95% CI]: 2.43–9.23; P<0.00001; I2 = 59%, 95% CI: 1.60–3.49; P<0.0001; I2 = 0%, respectively) at 6 and 12 months. Secondary outcomes showed that a 90% and 77% decreased risk at 6 and 12 months for a loss of 15 letters or more. The overall mean difference showed a statistically significance in best-corrected visual acuity (BCVA) on each time point. However, changes of central retinal thickness (CRT) lost significance at 12 months after 6-month as-needed treatment. The incidence of adverse events (AEs) had no statistical difference between anti-VEGF and placebo groups. Subgroup analyses indicated that patients receiving Aflibercept got the highest tendency to gain 15 letters or more (OR = 9.78; 95% CI: 4.43–21.56; P<0.00001). Age controlled analysis suggested a weaken tendency of BCVA improvement in age over 50 (MD = 12.26; 95% CI: 7.55–16.98; P<0.00001). Subgroup analysis by clinical classification showed a strengthen difference of BCVA changes at 6 months in ischemic type (MD = 19.65 letters, 95% CI: 13.15 to 26.14 letters, P<0.00001). Conclusions Our results showed that anti-VEGF agents were superior to placebo in CRVO

  12. 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

  13. Development of the Pulmonary Vein and the Systemic Venous Sinus: An Interactive 3D Overview

    PubMed Central

    van den Berg, Gert; Moorman, Antoon F. M.

    2011-01-01

    Knowledge of the normal formation of the heart is crucial for the understanding of cardiac pathologies and congenital malformations. The understanding of early cardiac development, however, is complicated because it is inseparably associated with other developmental processes such as embryonic folding, formation of the coelomic cavity, and vascular development. Because of this, it is necessary to integrate morphological and experimental analyses. Morphological insights, however, are limited by the difficulty in communication of complex 3D-processes. Most controversies, in consequence, result from differences in interpretation, rather than observation. An example of such a continuing debate is the development of the pulmonary vein and the systemic venous sinus, or “sinus venosus”. To facilitate understanding, we present a 3D study of the developing venous pole in the chicken embryo, showing our results in a novel interactive fashion, which permits the reader to form an independent opinion. We clarify how the pulmonary vein separates from a greater vascular plexus within the splanchnic mesoderm. The systemic venous sinus, in contrast, develops at the junction between the splanchnic and somatic mesoderm. We discuss our model with respect to normal formation of the heart, congenital cardiac malformations, and the phylogeny of the venous tributaries. PMID:21779373

  14. Genetics Home Reference: cerebral cavernous malformation

    MedlinePlus

    ... R, Awad IA, Ginsberg MH. Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity. J Exp Med. 2010 Apr 12;207(4):881-96. doi: 10.1084/jem.20091258. Epub 2010 Mar 22. Citation on ... CCM1 and CCM2 protein interactions in cell signaling: implications for cerebral cavernous ...

  15. Arteries and Veins of the Sylvian Fissure and Insula: Microsurgical Anatomy.

    PubMed

    Delion, Matthieu; Mercier, Philippe; Brassier, Gilles

    2016-01-01

    We present a vascular anatomical study of the arteries and veins of the sylvian fissure and insula.A good knowledge of the sylvian fissure, the insula, and their vascular relationship would seem mandatory before performing surgery in this area, whatever the type of surgery (aneurysms, arteriovenous malformations, insular tumors).We start with the sylvian fissure and insula morphology, followed by the MCA description and its perforators, with special attention paid to the insular perforators. We demonstrate that the long insular perforators penetrating in the superior part of the posterior short gyrus and long gyri vascularize, respectively, the corticonuclear and corticospinal fasciculi. We particularly insist too on three anatomical constants regarding the vascularization of the insula, already described in the literature: The superior periinsular sulcus is the only sulcus on the lateral surface of the brain without an artery along its axis; the superior branch of the MCA supplies the anterior insular pole and both the anterior and middle short gyri in 100 % of cases; in at least 90 % of cases, the artery that supplied the central insular sulcus continued on to become the central artery.We end with the anatomical study of the veins and cisterns. PMID:26508410

  16. What Causes Varicose Veins?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Causes Varicose Veins? Weak or damaged valves in the veins can ... space. These are varicose veins. Normal Vein and Varicose Vein Figure A shows a normal vein with a ...

  17. Apoptosis, cell proliferation and modulation of cyclin-dependent kinase inhibitor p21(cip1) in vascular remodelling during vein arterialization in the rat.

    PubMed

    Borin, Thaiz Ferraz; Miyakawa, Ayumi Aurea; Cardoso, Leandro; de Figueiredo Borges, Luciano; Gonçalves, Giovana Aparecida; Krieger, Jose Eduardo

    2009-06-01

    Neo-intima development and atherosclerosis limit long-term vein graft use for revascularization of ischaemic tissues. Using a rat model, which is technically less challenging than smaller rodents, we provide evidence that the temporal morphological, cellular, and key molecular events during vein arterialization resemble the human vein graft adaptation. Right jugular vein was surgically connected to carotid artery and observed up to 90 days. Morphometry demonstrated gradual thickening of the medial layer and important formation of neo-intima with deposition of smooth muscle cells (SMC) in the subendothelial layer from day 7 onwards. Transmission electron microscopy showed that SMCs switch from the contractile to synthetic phenotype on day 3 and new elastic lamellae formation occurs from day 7 onwards. Apoptosis markedly increased on day 1, while alpha-actin immunostaining for SMC almost disappeared by day 3. On day 7, cell proliferation reached the highest level and cellular density gradually increased until day 90. The relative magnitude of cellular changes was higher in the intima vs. the media layer (100 vs. 2 times respectively). Cyclin-dependent kinase inhibitors (CDKIs) p27(Kip1) and p16(INKA) remained unchanged, whereas p21(Cip1) was gradually downregulated, reaching the lowest levels by day 7 until day 90. Taken together, these data indicate for the first time that p21(Cip1) is the main CDKI protein modulated during the arterialization process the rat model of vein arterialization that may be useful to identify and validate new targets and interventions to improve the long-term patency of vein grafts. PMID:19563615

  18. Cerebral arteriovenous malformation presenting as visual deterioration in a child.

    PubMed

    Kaye, L C; Kaye, S B; Lagnado, R; Boothroyd, A; Morton, C; May, P

    2000-10-01

    A rare case of visual loss as the presenting feature of a central arteriovenous malformation involving the vein of Galen is reported. A 5-year-old girl with a history of deteriorating vision for the past 6 months was examined. Ocular examination showed a left hemianopia, left optic atrophy, and dilated vessels of the right optic disc. MRI revealed a massive deep-seated central arteriovenous malformation involving the vein of Galen. The mechanism of visual loss is likely to be a combination of ischaemic optic atrophy associated with a steal phenomenon and direct compression of the right optic radiation. PMID:11085301

  19. Arteriovenous malformations of the head and neck: current concepts in management.

    PubMed

    Fowell, Christopher; Jones, Robert; Nishikawa, Hiroshi; Monaghan, Andrew

    2016-06-01

    The field of vascular anomalies and their clinical management is expanding and developments in the last few years have improved the understanding of haemangiomas and vascular malformations. In this paper, the first in the series of 3 educational reviews, we review the current trends and evidence-based management of arteriovenous malformations. PMID:27020371

  20. Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013.

    PubMed

    Lee, B B; Baumgartner, I; Berlien, P; Bianchini, G; Burrows, P; Gloviczki, P; Huang, Y; Laredo, J; Loose, D A; Markovic, J; Mattassi, R; Parsi, K; Rabe, E; Rosenblatt, M; Shortell, C; Stillo, F; Vaghi, M; Villavicencio, L; Zamboni, P

    2015-04-01

    Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and

  1. 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

  2. Abnormal patterns of the renal veins

    PubMed Central

    Azari, Hassan; Abedinzadeh, Mehdi

    2012-01-01

    Knowledge of the renal vascular anatomy may greatly contribute to the success of surgical, invasive and radiological procedures of the retroperitoneal region. Here, morphometric and histological studies of a human cadaveric specimen presented a complex, anomalous pattern of renal veins. The left renal vein had an oblique retro-aortic course and received two lumbar veins. It bifurcated near its drainage point into the inferior vena cava. The right renal vein received the right testicular vein. In addition, the left kidney was located at a low position. The spleen was enlarged. The present case is unique and provides information that may help surgeons or angiologists to apply safer interventions. PMID:22536553

  3. Vein Problems Related to Varicose Veins

    MedlinePlus

    ... your doctor if you think you have them. Spider Veins Spider veins are a smaller version of varicose veins and a less serious type of telangiectasias. Spider veins involve the capillaries, the smallest blood vessels ...

  4. Personal Authentication Analysis Using Finger-Vein Patterns in Patients with Connective Tissue Diseases—Possible Association with Vascular Disease and Seasonal Change -

    PubMed Central

    Kono, Miyuki; Miura, Naoto; Fujii, Takao; Ohmura, Koichiro; Yoshifuji, Hajime; Yukawa, Naoichiro; Imura, Yoshitaka; Nakashima, Ran; Ikeda, Takaharu; Umemura, Shin-ichiro; Miyatake, Takafumi; Mimori, Tsuneyo

    2015-01-01

    Objective To examine how connective tissue diseases affect finger-vein pattern authentication. Methods The finger-vein patterns of 68 patients with connective tissue diseases and 24 healthy volunteers were acquired. Captured as CCD (charge-coupled device) images by transmitting near-infrared light through fingers, they were followed up in once in each season for one year. The similarity of the follow-up patterns and the initial one was evaluated in terms of their normalized cross-correlation C. Results The mean C values calculated for patients tended to be lower than those calculated for healthy volunteers. In midwinter (February in Japan) they showed statistically significant reduction both as compared with patients in other seasons and as compared with season-matched healthy controls, whereas the values calculated for healthy controls showed no significant seasonal changes. Values calculated for patients with systemic sclerosis (SSc) or mixed connective tissue disease (MCTD) showed major reductions in November and, especially, February. Patients with rheumatoid arthritis (RA) and patients with dermatomyositis or polymyositis (DM/PM) did not show statistically significant seasonal changes in C values. Conclusions Finger-vein patterns can be used throughout the year to identify patients with connective tissue diseases, but some attention is needed for patients with advanced disease such as SSc. PMID:26701644

  5. Pulmonary arteriovenous malformation in chronic thromboembolic pulmonary hypertension

    PubMed Central

    Sharma, Arun; Gulati, Gurpreet S; Parakh, Neeraj; Aggarwal, Abhinav

    2016-01-01

    Chronic thromboembolic pulmonary hypertension is a morbid condition associated with complications such as hemoptysis, right heart failure, paradoxical embolism, and even death. There is no known association of chronic thromboembolic pulmonary hypertension with pulmonary arteriovenous malformation. Possible hypothesis for this association is an increased pulmonary vascular resistance leading to the compensatory formation of pulmonary arteriovenous malformation. We present one such case presenting with hemoptysis that was managed with endovascular treatment. PMID:27413264

  6. The anatomy of the cardiac veins in mice

    PubMed Central

    Ciszek, Bogdan; Skubiszewska, Daria; Ratajska, Anna

    2007-01-01

    Although the cardiac coronary system in mice has been the studied in detail by many research laboratories, knowledge of the cardiac veins remains poor. This is because of the difficulty in marking the venous system with a technique that would allow visualization of these large vessels with thin walls. Here we present the visualization of the coronary venous system by perfusion of latex dye through the right caudal vein. Latex injected intravenously does not penetrate into the capillary system. Murine cardiac veins consist of several principal branches (with large diameters), the distal parts of which are located in the subepicardium. We have described the major branches of the left atrial veins, the vein of the left ventricle, the caudal veins, the vein of the right ventricle and the conal veins forming the conal venous circle or the prepulmonary conal venous arch running around the conus of the right ventricle. The venous system of the heart drains the blood to the coronary sinus (the left cranial caval vein) to the right atrium or to the right cranial caval vein. Systemic veins such as the left cranial caval, the right cranial caval and the caudal vein open to the right atrium. Knowledge of cardiac vein location may help to elucidate abnormal vein patterns in certain genetic malformations. PMID:17553104

  7. 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. PMID:27587990

  8. Varicose vein - noninvasive treatment

    MedlinePlus

    Sclerotherapy; Laser therapy - varicose veins; Radiofrequency vein ablation; Endovenous thermal ablation; Ambulatory phlebectomy; Transilluminated power phlebotomy; Endovenous laser ablation; Varicose vein ...

  9. Cerebellar and Brainstem Malformations.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2016-08-01

    The frequency and importance of the evaluation of the posterior fossa have increased significantly over the past 20 years owing to advances in neuroimaging. Conventional and advanced neuroimaging techniques allow detailed evaluation of the complex anatomic structures within the posterior fossa. A wide spectrum of cerebellar and brainstem malformations has been shown. Familiarity with the spectrum of cerebellar and brainstem malformations and their well-defined diagnostic criteria is crucial for optimal therapy, an accurate prognosis, and correct genetic counseling. This article discusses cerebellar and brainstem malformations, with emphasis on neuroimaging findings (including diagnostic criteria), neurologic presentation, systemic involvement, prognosis, and recurrence. PMID:27423798

  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. PMID:11469062

  11. Varicose vein stripping

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/002952.htm Varicose vein stripping To use the sharing features on this ... enable JavaScript. Vein stripping is surgery to remove varicose veins in the legs. Description Varicose veins are swollen, ...

  12. [Radical removal of a totally thrombosed vein of Galen aneurysm preoperatively diagnosed as a pineal neoplasm].

    PubMed

    Roszkowski, M; Barszcz, S; Grajkowska, W; Kowalska, A

    1993-01-01

    A case of an eight-year-old with signs of a pineal region tumor who underwent radical removal of a totally thrombosed vein of Galen aneurysm through the infratentorial supracerebellar approach. Pathophysiology of vein of Galen malformations including variety of clinical course, angiographic appearance and prognosis was described. Predisposition to spontaneous thrombosis in that type of malformations indicates necessity of taking into account such cases in the differential diagnosis of pineal region tumors. PMID:8115002

  13. Varicose vein - noninvasive treatment

    MedlinePlus

    Sclerotherapy; Laser therapy - varicose veins; Radiofrequency vein ablation; Endovenous thermal ablation; Ambulatory phlebectomy; Transilluminated power phlebotomy; Endovenous laser ablation; Varicose ...

  14. RASA1 somatic mutation and variable expressivity in capillary malformation/arteriovenous malformation (CM/AVM) syndrome.

    PubMed

    Macmurdo, Colleen F; Wooderchak-Donahue, Whitney; Bayrak-Toydemir, Pinar; Le, Jenny; Wallenstein, Matthew B; Milla, Carlos; Teng, Joyce M C; Bernstein, Jonathan A; Stevenson, David A

    2016-06-01

    Germline mutations in RASA1 are associated with capillary malformation-arteriovenous malformation (CM-AVM) syndrome. CM-AVM syndrome is characterized by multi-focal capillary malformations and arteriovenous malformations. Lymphatic anomalies have been proposed as part of the phenotype. Intrafamilial variability has been reported, suggesting modifiers and somatic events. The objective of the study was to identify somatic RASA1 "second hits" from vascular malformations associated with CM-AVM syndrome, and describe phenotypic variability. Participants were examined and phenotyped. Genomic DNA was extracted from peripheral blood on all participants. Whole-exome sequencing was performed on the proband. Using Sanger sequencing, RASA1 exon 8 was PCR-amplified to track the c.1248T>G, p.Tyr416X germline variant through the family. A skin biopsy of a capillary malformation from the proband's mother was also obtained, and next-generation sequencing was performed on DNA from the affected tissue. A familial germline heterozygous novel pathogenic RASA1 variant, c.1248T>G (p.Tyr416X), was identified in the proband and her mother. The proband had capillary malformations, chylothorax, lymphedema, and overgrowth, while her affected mother had only isolated capillary malformations. Sequence analysis of DNA extracted from a skin biopsy of a capillary malformation of the affected mother showed a second RASA1 somatic mutation (c.2245C>T, p.Arg749X). These results and the extreme variable expressivity support the hypothesis that somatic "second hits" are required for the development of vascular anomalies associated with CM-AVM syndrome. In addition, the phenotypes of the affected individuals further clarify that lymphatic manifestations are also part of the phenotypic spectrum of RASA1-related disorders. © 2016 Wiley Periodicals, Inc. PMID:26969842

  15. Medical management of vascular anomalies.

    PubMed

    Trenor, Cameron C

    2016-03-01

    We have entered an exciting era in the care of patients with vascular anomalies. These disorders require multidisciplinary care and coordination and dedicated centers have emerged to address this need. Vascular tumors have been treated with medical therapies for many years, while malformations have been historically treated with endovascular and operative procedures. The recent serendipitous discoveries of propranolol and sirolimus for vascular anomalies have revolutionized this field. In particular, sirolimus responses are challenging the dogma that vascular malformations are not biologically active. While initially explored for lymphatic anomalies, sirolimus is now being used broadly throughout the spectrum of vascular anomalies. Whether medical therapies are reserved for refractory patients or used first line is currently dependent on the experience and availability of alternative therapies at each institution. On the horizon, we anticipate new drugs targeting genes and pathways involved in vascular anomalies to be developed. Also, combinations of medications and protocols combining medical and procedural approaches are in development for refractory patients. PMID:27607327

  16. Comparison of anti-vascular endothelial growth factors, laser treatments and a combination of the both for treatment of central retinal vein occlusion

    PubMed Central

    Pikkel, Yoav Y.; Sharabi-Nov, Adi; Beiran, Itzchak; Pikkel, Joseph

    2016-01-01

    AIM To compare changes in visual acuity and macular edema in patients with central retinal vein occlusion (CRVO) treated with intravitreal injections of bevacizumab, macular grid photocoagulation combined with pan retinal photocoagulation (PRP), or both (bevacizumab+grid+PRP). METHODS Our study is a retrospective cohort clinical study that examined patients that suffered from ischemic CRVO with macular edema. Study inclusion criteria were ischemic CRVO with macula edema and the availability of complete medical records for at least 12mo after treatment. Excluded were patients with diabetes or any other retinal disease. We reviewed the medical records of patients treated in one ophthalmology department-comparing changes in visual acuity and macular edema in patients treated with intravitreal injections of bevacizumab vs those that were treated with macular grid photocoagulation and PRP or both. The main outcome measures were the differences in best corrected visual acuity (BCVA) and in macular thickness, as assessed by optical coherence tomography, between the enrollment and the final follow up visits. RESULTS Sixty-five patients met inclusion criteria. There were no statistically significant differences among the three groups in the mean changes in macular thickness as measured by ocular coherence tomography (131.5±41.2, 108.6±29.2, and 121.1±121.1, P=0.110), or in visual acuity (0.128±0.077, 0.088±0.057, and 0.095±0.065), for intravitreal injections, macular grid photocoagulation+PRP and a combination of the treatments, respectively, P=0.111. The proportions of patients with macular edema after treatment were: 26.1%, 28.6%, and 14.3%, respectively, P=0.499. CONCLUSION Similar benefit was observed for intravitreal injections, laser photocoagulation, or a combined regimen in the treatment of CRVO. A non-statistically significant trend for reduction in macular edema was observed following combined treatment. PMID:27158615

  17. Coexistent arteriovenous malformation and hippocampal sclerosis.

    PubMed

    Prayson, Richard A; O'Toole, Elizabeth E

    2016-06-01

    Cavernous angiomas or cavernomas have been occasionally described in patients presenting with medically intractable epilepsy. Reports of cavernomas associated with a second pathology potentially causative of seizures have rarely been documented; most commonly, the second pathology is focal cortical dysplasia or less frequently, hippocampal sclerosis. To our knowledge, cases of arteriovenous malformation arising in this clinical setting and associated with hippocampal sclerosis have not been previously described. We report a 56-year-old woman who initially presented at age 24years with staring spells. Imaging studies revealed an arteriovenous malformation in the right parietal lobe. At age 51years, she represented with signs and symptoms related to a hemorrhage from the malformation. The patient underwent Gamma Knife radiosurgery (Elekta AB, Stockholm, Sweden) of the lesion. She subsequently developed seizures, refractory to medical management. MRI studies showed atrophy in the right hippocampus. She underwent resection of the right parietal lobe and hippocampus. Histopathologic examination of the right parietal lesion revealed an arteriovenous malformation marked by focally prominent vascular sclerosis, calcification and adjacent hemosiderin deposition. The hippocampus was marked by prominent neuronal loss and gliosis in the CA1 region, consistent with CA1 sclerosis or hippocampal sclerosis International League Against Epilepsy type 2. PMID:26899356

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

    PubMed Central

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

    2012-01-01

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

  19. Effect of CPU-XT-008, a combretastatin A-4 analogue, on the proliferation, apoptosis and expression of vascular endothelial growth factor and basic fibroblast growth factor in human umbilical vein endothelial cells

    PubMed Central

    XIONG, RUI; SUN, JING; LIU, KUN; XU, YUNGEN; HE, SHUYING

    2016-01-01

    The present study investigated the effect of the combretastatin A-4 analogue CPU-XT-008 on the proliferation, apoptosis and expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (FGF-2) in human umbilical vein endothelial cells (HUVECs). The proliferation capacity of HUVECs was analyzed with a cell viability assay, while their apoptosis and migration abilities were evaluated via flow cytometry and monolayer denudation assay, respectively. The mRNA and protein expression levels of VEGF and FGF-2 in these cells were determined by reverse transcription-polymerase chain reaction, and cell-based ELISA, western blotting and immunocytochemistry, respectively. The results demonstrated that CPU-XT-008 inhibited proliferation and migration, and induced apoptosis in HUVECs in a dose-dependent manner. In addition, CPU-XT-008 downregulated the mRNA and protein expression levels of VEGF and FGF-2 in these cells. These findings suggest that CPU-XT-008 exerts anti-angiogenic effects in HUVECs, which may explain the inhibition of cell proliferation and migration, induction of apoptosis, and reduction in the mRNA and protein expression levels of VEGF and FGF-2 observed in the present study. PMID:26870239

  20. Pars Plana Vitrectomy Combined with Internal Limiting Membrane Peeling to Treat Persistent Macular Edema after Anti-Vascular Endothelial Growth Factor Treatment in Cases of Ischemic Central Retinal Vein Occlusion

    PubMed Central

    Shirakata, Yukari; Fujita, Tomoyoshi; Nakano, Yuki; Shiraga, Fumio; Tsujikawa, Akitaka

    2016-01-01

    Objective To evaluate the efficacy of pars plana vitrectomy (PPV) combined with internal limiting membrane (ILM) peeling in cases of ischemic central retinal vein occlusion (CRVO) where macular edema (ME) persisted after anti-vascular endothelial growth factor (anti-VEGF) treatment. Methods Fifteen eyes with ischemic CRVO-related ME were included in the study. Nine were treated with panretinal photocoagulation after initial examination. Anti-VEGF agents were injected intravitreally. Persistent ME was treated with PPV combined with ILM peeling. During surgery, laser photocoagulation was further applied to the non-perfused area. Results Mean retinal thickness gradually decreased after surgery (p = 0.024 at 6 months), although visual acuity did not improve significantly during the follow-up period (14.7 ± 11.6 months). Neovascular glaucoma subsequently developed in three cases and a trabeculectomy was performed in one case. Conclusion In eyes with ischemic CRVO, PPV combined with ILM peeling contributed to a reduction in persistent ME. However, there was no significant improvement in visual acuity. PMID:26889152

  1. Synergistic protective effects of escin and low‑dose glucocorticoids against vascular endothelial growth factor‑induced blood‑retinal barrier breakdown in retinal pigment epithelial and umbilical vein endothelial cells.

    PubMed

    Zhang, Fenglan; Man, Xuejing; Yu, Huajun; Liu, Limei; Li, Yuanbin

    2015-02-01

    Previous studies have shown that escin possesses glucocorticoid (GC)‑like anti‑edematous and anti‑inflammatory effects. The present study was designed to investigate whether escin exhibits synergistic protective effects against blood‑retinal barrier (BRB) breakdown when combined with GC in an in vitro monolayer BRB model, based on retinal pigment epithelial (RPE) cells and human umbilical vein endothelial cells (HUVECs). The results showed that low concentrations of escin and triamcinolone acetonide (TA) administered separately did not affect BRB trans‑endothelial (epithelium) resistance (TEER). However, when administered together, escin and TA significantly inhibited reduced BRB TEER following treatment with vascular endothelial growth factor (VEGF). Furthermore, low‑concentrations of escin and TA administered together significantly increased the expression levels of occludin and ZO‑1. This demonstrates that escin and GC have synergistic protective effects against BRB breakdown, and the molecular mechanisms may be related to the upregulation of occludin and ZO‑1 expression. The combination of escin with GC indicates a potential beneficial strategy for the treatment of breakdown of the BRB. PMID:25370688

  2. Embryonic development and malformation of lymphatic vessels.

    PubMed

    Wilting, Jörg; Buttler, Kerstin; Rössler, Jochen; Norgall, Susanne; Schweigerer, Lothar; Weich, Herbert A; Papoutsi, Maria

    2007-01-01

    In the human, malformations of lymphatic vessels can be observed as lymphangiectasia, lymphangioma and lymphangiomatosis, with a prevalence of 1.2-2.8 per thousand. Their aetiology is unknown and a causal therapy does not exist. We investigated the origin of lymphatic endothelial cells (LECs) in avian and murine embryos, and compared the molecular profile of LECs from normal and malformed lymphatics of children. In avian embryos, Prox1+ lymphangioblasts are located in the confluence of the cranial and caudal cardinal veins, where the jugular lymph sac (JLS) forms. Cell lineage studies show that the JLS is of venous origin. In contrast, the lymphatics of the dermis are derived from mesenchymal lymphangioblasts located in the dermatomes, suggesting a dual origin of LECs in avian embryos. The same may hold true for murine embryos, where Lyve1+ LEC precursors are found in the cardinal veins, and in the mesenchyme. The mesenchymal cells express the pan-leukocyte marker CD45, indicating a cell type with lymphendothelial and leukocyte characteristics. In the human, such cells might give rise to Kaposi's sarcoma. Microarray analyses of LECs from lymphangiomas of children show a large number of regulated genes, such as VEGFR3. Our studies show that lymphvasculogenesis and lymphangiogenesis occur simultaneously in the embryo, and suggest a function for VEGFR3 in lymphangiomas. PMID:18300425

  3. An Unusual Cause of Dysphagia: A Large Expectorated Arteriovenous Malformation

    PubMed Central

    Griffin, Aaron S.; Gunasena, Rivindi; Schaefer, Nathan R.; Kennedy, Edmund

    2015-01-01

    Background Vascular malformations are generally detected in childhood or adolescence with first presentations in adulthood being rare. Case Report We report the case of a 52-year-old female with threatened compromise of her airway after expectorating a massive arteriovenous malformation anchored at the supraglottis. The only preceding symptom was dysphagia. The lesion was resected, the patient had a quick recovery, and she has shown no evidence of recurrence. Conclusion Although uncommon, vascular malformations of the supraglottis or hypopharynx should be considered in the differential diagnosis of a patient presenting with dysphagia because of the potential to cause disastrous airway compromise. Although a lesion presenting acutely mandates a definitive airway plan, when clinically possible, computed tomography scan and indirect laryngoscopy can provide useful information for the airway and operative teams. PMID:26130989

  4. Surgical management of arteriovenous malformation.

    PubMed

    Visser, Anniek; FitzJohn, Trevor; Tan, Swee T

    2011-03-01

    This article presents our experience in managing a series of consecutive patients with arteriovenous malformation (AVM) referred to our Vascular Anomalies Centre over a 14-year period. These patients were culled from our prospective Vascular Anomalies Database 1996-2010. The medical records of these patients were reviewed to supplement the data collected. Out of 1131 patients with vascular anomalies, 53 patients (22 males, 31 females) with AVM were identified. Their mean age was 29 (range: 3-88) years with 14 stage-III, 34 stage-II and five stage-I AVMs, affecting the head and neck area (n=32), lower limb (n=13), upper limb (n=7) and trunk (n=1). Eight patients with eight stage-III and 14 patients with 15 stage-II AVMs underwent definitive surgery following preoperative embolisation in 10 patients. Seventeen patients required reconstruction with free flaps (n=8) or local or regional flaps (n=9), tissue expansion (n=4), tendon recession (n=1), tendon transfer (n=1), osseo-integration (n=1) and skin grafting (n=5). Fourteen patients required a combination of reconstructive techniques. During an average follow-up of 54 (range: 10-135) months, two (8.7%) lesions recurred but were improved following surgery. One patient with life-threatening stage-III AVM underwent 'palliative' surgery following preoperative embolisation and the lesion had improved and remained stable during the 4-year follow-up period. AVM is a challenging clinical problem that requires a multidisciplinary team approach. Complete surgical excision remains the gold-standard treatment and immediate reconstruction is an integral part of definitive surgery for AVM. The heterogeneous nature of AVM requires treatment to be tailored for individual patients and the complex excision defects necessitate expertise in a variety of reconstructive techniques. Our experience shows a recurrence rate of 8.7% following definitive surgery for AVM. PMID:20663728

  5. Corpus callosum arteriovenous malformation with persistent trigeminal artery.

    PubMed

    Mohanty, Chandan B; Devi, B Indira; Somanna, Sampath; Bhat, Dhananjaya I; Dawn, Rose

    2011-12-01

    A 13-year-old boy presented with an intracerebral haematoma secondary to a large corpus callosal arteriovenous malformation (AVM) with an associated persistent trigeminal artery, and was treated with Gamma-Knife Radiosurgery for the AVM. This report discusses the embryological basis, radiological features and various classifications of this rare vascular anomaly. PMID:21501055

  6. Giacomini vein: thigh extension of the small saphenous vein - report of two cases and review of the literature

    PubMed Central

    Natsis, K; Paraskevas, G; Lazaridis, N; Sofidis, G; Piagkou, M

    2015-01-01

    Background Varicose vein surgery is very commonly performed. Also, it is very frequently employed for recurrent disease. The recognition of the normal or variant veins, inducing incompetency, is a prerequisite for effective treatment. The thigh extension of the small saphenous vein, the so-called Giacomini vein, was extensively described in 1873 by Carlo Giacomini in an incidence of 72%. However, such a vein is usually underestimated in classic surgical textbooks. Description of cases We present two cases of Giacomini vein found in two cadavers, dissected for academic purposes in the Department of Anatomy, where the small saphenous vein displayed a thigh extension without terminating into the popliteal vein. In one case Giacomini vein drained into the large saphenous vein, while in the other case the vein divided into two branches, separately draining into the large saphenous vein and the subcutaneous tissue of the gluteal region. Conclusion Due to the fact that Giacomini vein could be incompetent, associated with or without varicose saphenous vein trunks, the vascular surgeon should keep in mind that anatomical entity, to include it in preoperative ultrasound scanning control. Moreover, this vein could be utilized as an autologous graft, when the large saphenous is not available. Hippokratia 2015; 19 (3): 263-265.

  7. Diagnostic imaging in the evaluation of vascular birthmarks.

    PubMed

    Burrows, P E; Laor, T; Paltiel, H; Robertson, R L

    1998-07-01

    This article reviews the role of modern diagnostic imaging in the evaluation of patients with vascular birthmarks. There are two main categories of vascular anomalies: hemangiomas and vascular malformations. The findings on plain radiography, sonography, computed tomography (CT), magnetic resonance imaging (MRI) and angiography, and the appropriate sequence of investigation for the different vascular anomalies are discussed. PMID:9704205

  8. Deep Vein Thrombosis

    MedlinePlus

    MENU Return to Web version Deep Vein Thrombosis Overview What is deep vein thrombosis? Deep vein thrombosis (also called DVT) is a blood clot in a vein deep inside your body. These clots usually occur in your leg veins. While DVT is a fairly common condition, it is ...

  9. Deep Vein Thrombosis

    MedlinePlus

    Deep vein thrombosis, or DVT, is a blood clot that forms in a vein deep in the body. Most deep vein clots occur in the ... vein swells, the condition is called thrombophlebitis. A deep vein thrombosis can break loose and cause a serious problem ...

  10. Deep Vein Thrombosis

    MedlinePlus

    Deep vein thrombosis, or DVT, is a blood clot that forms in a vein deep in the body. Most deep vein clots occur in the lower leg or ... vein swells, the condition is called thrombophlebitis. A deep vein thrombosis can break loose and cause a ...

  11. Umbilical intra-abdominal vein varix: a case report and review of the literature.

    PubMed

    Virdis, G; Gulotta, A; Cherchi, C; Spanedda, G; Olzai, M G; Ambrosini, G; Dessole, S; Capobianco, G

    2016-01-01

    Fetal umbilical intra-abdominal vein varix (FIUV) is a rare congenital malformation characterized by focal dilatation of the umbilical vein. The authors report a case of pregnant woman at 32 weeks of gestation with a fetus affected by dilatation of an intra-abdominal portion of the umbilical vein. They performed continuous ultrasound and cardiotocographic monitoring, from admission to the delivery. They describe the case and perform a review of the literature. PMID:27132426

  12. Etiologies of uterine malformations.

    PubMed

    Jacquinet, Adeline; Millar, Debra; Lehman, Anna

    2016-08-01

    Ranging from aplastic uterus (including Mayer-Rokitansky-Kuster-Hauser syndrome) to incomplete septate uterus, uterine malformations as a group are relatively frequent in the general population. Specific causes remain largely unknown. Although most occurrences ostensibly seem sporadic, familial recurrences have been observed, which strongly implicate genetic factors. Through the study of animal models, human syndromes, and structural chromosomal variation, several candidate genes have been proposed and subsequently tested with targeted methods in series of individuals with isolated, non-isolated, or syndromic uterine malformations. To date, a few genes have garnered strong evidence of causality, mainly in syndromic presentations (HNF1B, WNT4, WNT7A, HOXA13). Sequencing of candidate genes in series of individuals with isolated uterine abnormalities has been able to suggest an association for several genes, but confirmation of a strong causative effect is still lacking for the majority of them. We review the current state of knowledge about the developmental origins of uterine malformations, with a focus on the genetic variants that have been implicated or associated with these conditions in humans, and we discuss potential reasons for the high rate of negative results. The evidence for various environmental and epigenetic factors is also reviewed. © 2016 Wiley Periodicals, Inc. PMID:27273803

  13. 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. PMID:27035547

  14. Vascular wall extracellular matrix proteins and vascular diseases

    PubMed Central

    Xu, Junyan; Shi, Guo-Ping

    2014-01-01

    Extracellular matrix proteins form the basic structure of blood vessels. Along with providing basic structural support to blood vessels, matrix proteins interact with different sets of vascular cells via cell surface integrin or non-integrin receptors. Such interactions induce vascular cell de novo synthesis of new matrix proteins during blood vessel development or remodeling. Under pathological conditions, vascular matrix proteins undergo proteolytic processing, yielding bioactive fragments to influence vascular wall matrix remodeling. Vascular cells also produce alternatively spliced variants that induce vascular cell production of different matrix proteins to interrupt matrix homeostasis, leading to increased blood vessel stiffness; vascular cell migration, proliferation, or death; or vascular wall leakage and rupture. Destruction of vascular matrix proteins leads to vascular cell or blood-borne leukocyte accumulation, proliferation, and neointima formation within the vascular wall; blood vessels prone to uncontrolled enlargement during blood flow diastole; tortuous vein development; and neovascularization from existing pathological tissue microvessels. Here we summarize discoveries related to blood vessel matrix proteins within the past decade from basic and clinical studies in humans and animals — from expression to cross-linking, assembly, and degradation under physiological and vascular pathological conditions, including atherosclerosis, aortic aneurysms, varicose veins, and hypertension. PMID:25045854

  15. 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

  16. Pediatric intraoral high-flow arteriovenous malformation: a diagnostic challenge.

    PubMed

    Petel, Roy; Ashkenazi, Malka

    2014-01-01

    Arteriovenous malformations (AVMs) are rarely reported in the dental pediatric literature. They may develop adjacent to primary molars and can be life-threatening due to their potential for massive bleeding. The most common symptom associated with documented cases of AVMs is spontaneous gingival bleeding. Other clinical signs include pain, erythematous gingiva, resorption and mobility of teeth, soft tissue discoloration, facial swelling, and asymmetry. Radiographically, AVMs are osteolytic lesions. The purpose of this report was to describe the challenge of diagnosis of a high-flow arteriovenous malformation located in the primary maxillary molar region, which was misdiagnosed as a dentoalveolar abscess adjacent to previously treated primary molars. A decision to extract a tooth with gingival swelling and associated spontaneous bleeding should be made after the differential diagnosis of a vascular malformation has been ruled out. PMID:25303512

  17. Treatment of congenital malformations.

    PubMed

    Brucker, Sara Yvonne; Rall, Katharina; Campo, Rudi; Oppelt, Peter; Isaacson, Keith

    2011-03-01

    The prevalence of müllerian malformations is 1 in 200, or 0.5%. A third of the anomalies are septate, a third bicornuate uteri, 10% arcuate uterus, 10% didelphis and unicornuate uterus, and < 5% uterine and vaginal aplasia. Correct diagnosis of the malformation is most important but often very difficult. Correct treatment can only be performed if the malformation is clear. Longitudinal vaginal septums have to be removed due to potential obstetric problems. Transverse vaginal septums can cause hematocolpos and pain and have to be incised crosswise and excised so as not to shorten the vagina at the same time. Congenital vaginal agenesis occurs in Mayer-Rokitansky-Kuster-Hauser syndrome patients and in androgen insensitivity syndrome. The first choice for surgical treatment should be the new laparoscopic-assisted creation of a neovagina. Septate uterus has to be distinguished from a bicornuate uterus. Even if it is not proven to be a cause for infertility, the chance of miscarriage can be diminished by performing hysteroscopic metroplasty. Repair of a uterine septum in infertility patients often improves pregnancy rates. In contrast, surgical repair of a bicornuate uterus requires an abdominal metroplasty. This should only be performed if the patient has recurrent fetal loss due to the uterine structural defect. In a unicornuate uterus it is most important to determine if there is a second uterine horn that can cause cyclic pain if it has functioning endometrium. The only surgical option in these cases is to remove the rudimentary uterus with endometrium and hematometra, respectively. PMID:21437824

  18. On the phenomenology of the perforating vein of the elbow.

    PubMed

    Lomonte, Carlo; Basile, Carlo

    2009-01-01

    The perforating veins, as the name suggests, are the veins that perforate the muscular aponeuroses ensuring communication between the superficial and the deep veins. Located at the bend of the elbow, almost constantly, there is a vein, named perforating vein of the elbow, which is of great interest to the nephrologist who is responsible for the management of vascular access for hemodialysis (HD). It represents, in fact, because of its intrinsic characteristics and topographical reasons, a valuable resource for the creation of a vascular access for HD, especially in obese patients, elderly patients, diabetics, and patients affected by peripheral vasculopathy. Arterio-venous fistulae (AVF) constructed with the perforating vein of the elbow are relatively easy to perform, have an excellent patency rate, a low incidence of early thrombosis, adequate flows, and a low incidence of the steal syndrome. In other types of AVFs, the perforating vein of the elbow subtracts flow to the superficial veins slowing or preventing their full maturation, and can become a problem in measuring the blood flow of the AVF. But still, its presence can maintain patent a fistula in case of poor compliance of the superficial veins, while awaiting for interventional procedures able to accelerate a subsequent maturation. This review intends to explore the role that the perforating vein of the elbow plays in the physiology and pathology of the AVF, in relation to the issues that most frequently occur in clinical practice. PMID:19573011

  19. 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. PMID:26631589

  20. 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

  1. 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.

  2. 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

  3. 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

  4. Portal Vein Thrombosis

    PubMed Central

    Mallet, Thierry; Soltys, Remigiusz; Loarte, Pablo

    2015-01-01

    Portal vein thrombosis (PVT) is the blockage or narrowing of the portal vein by a thrombus. It is relatively rare and has been linked with the presence of an underlying liver disease or prothrombotic disorders. We present a case of a young male who presented with vague abdominal symptoms for approximately one week. Imaging revealed the presence of multiple nonocclusive thrombi involving the right portal vein, the splenic vein, and the left renal vein, as well as complete occlusion of the left portal vein and the superior mesenteric vein. We discuss pathogenesis, clinical presentation, and management of both acute and chronic thrombosis. The presence of PVT should be considered as a clue for prothrombotic disorders, liver disease, and other local and general factors that must be carefully investigated. It is hoped that this case report will help increase awareness of the complexity associated with portal vein thrombosis among the medical community. PMID:25802795

  5. Preventing Deep Vein Thrombosis

    MedlinePlus

    ... Patient Education FAQs Preventing Deep Vein Thrombosis Patient Education Pamphlets - Spanish Preventing Deep Vein Thrombosis FAQ174, August 2011 PDF ... Your Practice Patient Safety & Quality Payment Reform (MACRA) Education & Events Annual ... Pamphlets Teen Health About ACOG About Us Leadership & ...

  6. 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

  7. Deep Vein Thrombosis

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Deep Vein Thrombosis? Español Deep vein thrombosis (throm-BO-sis), or DVT, is a blood clot that forms in a vein deep in the body. Blood clots occur when blood ...

  8. Brain arteriovenous malformations.

    PubMed

    Ferrara, Adi R

    2011-01-01

    An arteriovenous malformation (AVM) is a particular abnormality of blood vessels. Brain AVMs are congenital, but symptoms usually do not appear until the second decade of life - if at all. The most common presenting symptom is a brain hemorrhage, but other possible symptoms include neurological deficits, seizures and headaches. Until recently, the gold standard for diagnosing AVM was conventional angiography. However, computed tomography and magnetic resonance angiography are now the first-line diagnostic tools for AVMs. This article reviews the presenting symptoms, diagnostic procedures and treatment options for brain AVMs, including embolization, micro-surgery and radiosurgery. This article is a Directed Reading. Your access to Directed Reading quizzes for continuing education credit is determined by your CE preference. For access to other quizzes, go to www.asrt.org/store. PMID:21771938

  9. Varicose veins and venous insufficiency

    MedlinePlus

    Varicose veins are swollen, twisted, and enlarged veins that you can see under the skin. They are often ... from the blood that collects there, which causes varicose veins. Smaller varicose veins that you can see on ...

  10. Abdominopelvic vascular injuries.

    PubMed

    Sriussadaporn, S

    2000-01-01

    The clinical records of 25 patients with 32 abdominopelvic vascular injuries were reviewed. Sixty per cent of patients sustained blunt trauma and 40 per cent sustained penetrating trauma. Nineteen patients (76%) were in shock on arrival, 2 of them underwent ER thoracotomy when they first arrived in the emergency room. Nine patients (36%) had signs of lower extremity ischemia. The Injury Severity Score (ISS) ranged from 16-50, mean 29 +/- 10.0. Nineteen patients (76%) had 35 associated injuries. Of the 32 injured vessels; 8 were external iliac artery, 5 were renal vein, 4 were abdominal aorta, 3 were common iliac artery, common iliac vein, external iliac vein and inferior vena cava, and 1 was superior mesenteric artery, superior mesenteric vein and median sacral artery. Treatments included: 13 lateral repair, 4 prosthetic grafting, 4 nephrectomy, 3 ligation, 3 reversed saphenous vein grafting, 2 end to end anastomosis, 1 internal iliac artery grafting, 1 intravascular shunt and packing and 1 perihepatic packing. Nine patients (36%) died. High mortality was observed in injuries to the abdominal aorta (75%), inferior vena cava (66.7%), common iliac vein (66.7%) and associated major pelvic fractures (50%). Factors significantly associated with mortality were the presence of shock on arrival, associated injuries and high Injury Severity Score. The author concludes that short prehospital time, effective resuscitation and proper surgical decision making are important for survival in these critically injured patients. PMID:10710864

  11. The umbilical and paraumbilical veins of man.

    PubMed Central

    Martin, B F; Tudor, R G

    1980-01-01

    During its transit through the umbilicus structural changes occur in the thick wall of the extra-abdominal segment of the umbilical vein whereby the components of the intra-abdominal segment acquire an essentially longitudinal direction and become arranged in fibro-elastic and fibro-muscular zones. The vein lumen becomes largely obliterated by asymmetrical proliferation of loose subendothelial conective tissue. The latter forms a new inner zone within which a small segment of the lumen persists in an eccentric position. This residual lumen transmits blood to the portal system from paraumbilical and systemic sources, and is retained in the upper part of the vein, even in old age. A similar process of lumen closure is observed in the ductus venosus. In early childhood the lower third of the vein undergoes breakdown, with fatty infiltration, resulting in its complete division into vascular fibro-elastic strands, and in old age some breakdown occurs in the outermost part of the wall of the upper two thirds. The paraumbilical veins are thick-walled and of similar structure to the umbilical vein. Together they constitute an accessory portal system which is confined between the layers of the falciform ligament and is in communication with the veins of the ventral abdominal wall. The constituents form an ascending series, namely, Burow's veins, the umbilical vein, and Sappey's inferior and superior veins. The main channel of Sappey's inferior veins may be the remnant of the right umbilical vein since it communicates with the right rectus sheath and often communicates directly with the portal system within the right lobe of the liver. The results are of significance in relation to clinical usage of the umbilical vein. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 Fig. 8 Fig. 9 Fig. 10 Fig. 11 Fig. 12 Fig. 13 Fig. 14 Fig. 15 Fig. 16 Fig. 17 Fig. 18 Fig. 19 Fig. 20 Fig. 21 Fig. 22 Fig. 23 Fig. 24 Fig. 25 Fig. 26 Fig. 27 Fig. 28 Fig. 29 PMID:7400038

  12. 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.

  13. Unusual Presentation of Cerebral Cavernous Malformation.

    PubMed

    Kim, Won-Hyung; Lim, Dong-Jun; Choi, Jong-Il; Ha, Sung-Kon; Kim, Sang-Dae; Kim, Se-Hoon

    2015-09-01

    Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage. PMID:26523262

  14. Unusual Presentation of Cerebral Cavernous Malformation

    PubMed Central

    Kim, Won-Hyung; Choi, Jong-Il; Ha, Sung-Kon; Kim, Sang-Dae; Kim, Se-Hoon

    2015-01-01

    Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage. PMID:26523262

  15. Varicose Veins and Other Vein Disorders

    MedlinePlus

    ... Share Glossary Basic Facts & Information Causes & Symptoms Diagnosis & Tests Care & Treatment Lifestyle & Management Other Resources Caregiving How To's Related Topics Peripheral Artery Disease Join our e-newsletter! Aging & Health A to Z Varicose Veins and Other ...

  16. Arteriovenous malformations of the bladder.

    PubMed

    Grunberger, I; DeAsis, A; Torno, R; Godec, C J

    1989-01-01

    We report a rare case of a localized arteriovenous malformation of the bladder mimicking a bladder tumor and presenting with gross hematuria. The mass was successfully resected transurethrally. PMID:2908934

  17. 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

  18. Vascular trauma in civilian practice.

    PubMed Central

    Golledge, J.; Scriven, M. W.; Fligelstone, L. J.; Lane, I. F.

    1995-01-01

    Vascular trauma is associated with major morbidity and mortality, but little is known about its incidence or nature in Britain. A retrospective study of 36 patients requiring operative intervention for vascular trauma under one vascular surgeon over a 6-year period was undertaken. Twenty-four patients suffered iatrogenic trauma (median age 61 years); including cardiological intervention (19), radiological intervention (2), varicose vein surgery (1), umbilical vein catherisation (1) and isolated hyperthermic limb perfusion (1). There were 23 arterial and three venous injuries. Twelve patients had accidental trauma (median age 23 years). Three of the ten patients with blunt trauma were referred for vascular assessment before orthopaedic intervention, two after an on-table angiogram and five only after an initial orthopaedic procedure (range of delay 6 h to 10 days). Injuries were arterial in nine, venous in two and combined in one. Angiography was obtained in six patients, and in two patients with multiple upper limb fractures identified the site of injury when clinical localisation was difficult. A variety of vascular techniques were used to treat the injuries. Two patients died postoperatively and one underwent major limb amputation. Thirty-two (89%) remain free of vascular sequelae after a median follow-up of 48 months (range 3-72 months). Vascular trauma is uncommon in the United Kingdom. To repair the injuries a limited repertoire of vascular surgery techniques is needed. Therefore, vascular surgical assessment should be sought at an early stage to prevent major limb loss. PMID:8540659

  19. Auxin Is Required for Leaf Vein Pattern in Arabidopsis1

    PubMed Central

    Sieburth, Leslie E.

    1999-01-01

    To investigate possible roles of polar auxin transport in vein patterning, cotyledon and leaf vein patterns were compared for plants grown in medium containing polar auxin transport inhibitors (N-1-naphthylphthalamic acid, 9-hydroxyfluorene-9-carboxylic acid, and 2,3,5-triiodobenzoic acid) and in medium containing a less well-characterized inhibitor of auxin-mediated processes, 2-(p-chlorophynoxy)-2-methylpropionic acid. Cotyledon vein pattern was not affected by any inhibitor treatments, although vein morphology was altered. In contrast, leaf vein pattern was affected by inhibitor treatments. Growth in polar auxin transport inhibitors resulted in leaves that lacked vascular continuity through the petiole and had broad, loosely organized midveins, an increased number of secondary veins, and a dense band of misshapen tracheary elements adjacent to the leaf margin. Analysis of leaf vein pattern developmental time courses suggested that the primary vein did not develop in polar auxin transport inhibitor-grown plants, and that the broad midvein observed in these seedlings resulted from the coalescence of proximal regions of secondary veins. Possible models for leaf vein patterning that could account for these observations are discussed. PMID:10594105

  20. Auxin is required for leaf vein pattern in Arabidopsis.

    PubMed

    Sieburth, L E

    1999-12-01

    To investigate possible roles of polar auxin transport in vein patterning, cotyledon and leaf vein patterns were compared for plants grown in medium containing polar auxin transport inhibitors (N-1-naphthylphthalamic acid, 9-hydroxyfluorene-9-carboxylic acid, and 2,3,5-triiodobenzoic acid) and in medium containing a less well-characterized inhibitor of auxin-mediated processes, 2-(p-chlorophynoxy)-2-methylpropionic acid. Cotyledon vein pattern was not affected by any inhibitor treatments, although vein morphology was altered. In contrast, leaf vein pattern was affected by inhibitor treatments. Growth in polar auxin transport inhibitors resulted in leaves that lacked vascular continuity through the petiole and had broad, loosely organized midveins, an increased number of secondary veins, and a dense band of misshapen tracheary elements adjacent to the leaf margin. Analysis of leaf vein pattern developmental time courses suggested that the primary vein did not develop in polar auxin transport inhibitor-grown plants, and that the broad midvein observed in these seedlings resulted from the coalescence of proximal regions of secondary veins. Possible models for leaf vein patterning that could account for these observations are discussed. PMID:10594105

  1. Malformations of cortical development

    PubMed Central

    Pang, Trudy; Atefy, Ramin; Sheen, Volney

    2012-01-01

    Background Malformations of cortical development (MCD) are increasingly recognized as an important cause of epilepsy and developmental delay. MCD encompass a wide spectrum of disorders with various underlying genetic etiologies and clinical manifestations. High resolution imaging has dramatically improved our recognition of MCD. Review Summary This review will provide a brief overview of the stages of normal cortical development, including neuronal proliferation, neuroblast migration, and neuronal organization. Disruptions at various stages lead to characteristic MCD. Disorders of neurogenesis give rise to microcephaly (small brain) or macrocephaly (large brain). Disorders of early neuroblast migration give rise to periventricular heterotopia (neurons located along the ventricles), whereas abnormalities later in migration lead to lissencephaly (smooth brain) or subcortical band heterotopia (smooth brain with a band of heterotopic neurons under the cortex). Abnormal neuronal migration arrest give rise to over-migration of neurons in cobblestone lissencephaly. Lastly, disorders of neuronal organization cause polymicrogyria (abnormally small gyri and sulci). This review will also discuss the known genetic mutations and potential mechanisms that contribute to these syndromes. Conclusion Identification of various gene mutations has not only given us greater insight into some of the pathophysiologic basis of MCD, but also an understanding of the processes involved in normal cortical development. PMID:18469675

  2. Pulmonary Vein Stenosis Mimicking Nonspecific Interstitial Pneumonia

    PubMed Central

    Linga, Karthika R.; Khoor, Andras; Phelan, Jonathan A.; Mira-Avendano, Isabel

    2015-01-01

    Pulmonary vein stenosis (PVS) is a known complication after catheter ablation of arrhythmias. Surprisingly, little information is available on its manifestations in the lung. We describe the case of a 39-year-old woman who presented from an outside hospital with worsening shortness of breath after catheter ablation of pulmonary veins for atrial fibrillation. After an initial diagnosis of pneumonia and its nonimprovement with antibiotics, a surgical lung biopsy was done and interpreted as nonspecific interstitial pneumonia (NSIP) with vascular changes consistent with pulmonary arterial hypertension. Later, she was admitted to our institution where a transthoracic echocardiogram (TTE) and subsequent computed tomography (CT) angiogram of the heart showed severe stenosis of all four pulmonary veins. The previous lung biopsy was rereviewed and reinterpreted as severe parenchymal congestion mimicking NSIP. Our case demonstrates that PVS is an underrecognized complication of catheter ablation, and increased awareness among both clinicians and pathologists is necessary to avoid misdiagnosis. PMID:26779359

  3. A case of combined soft tissue and intraosseous venous malformation of the thumb treated with sclerotherapy using a bone marrow aspiration needle

    PubMed Central

    Ishikawa, Kosuke; Sasaki, Satoru; Furukawa, Hiroshi; Nagao, Munetomo; Iwasaki, Daisuke; Fujita, Munezumi; Saito, Noriko; Oyama, Akihiko; Yamamoto, Yuhei

    2015-01-01

    Abstract Vascular malformations of bone are complex lesions that can cause deformity and pain. A combined soft tissue and intraosseous venous malformation of the left thumb in a girl was treated with two sessions of ethanol sclerotherapy using a bone marrow aspiration needle under fluoroscopic guidance.

  4. Retinal vascular changes are a marker for cerebral vascular diseases

    PubMed Central

    Moss, Heather E.

    2016-01-01

    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. PMID:26008809

  5. [How to do: central vein catheterization].

    PubMed

    Allgäuer, Sebastian

    2016-03-01

    The cannulation of a central vein is a standard acces to the vascular system of critically ill patients. It can be used for administration of medication and parenteral nutrition, haemodynamic monitoring as well as hemodialsis via Shaldon catheter.The technique of implantation of a central venous catheter is described step by step in this article. Moreover, advantages and disadvantages of the different techniques and puncture sites as well as indications and contraindications are critically discussed regarding the most recent literature. PMID:26939103

  6. Posttransplant Complex Inferior Venacava Balloon Dilatation After Hepatic Vein Stenting

    SciTech Connect

    Kohli, Vikas; Wadhawan, Manav; Gupta, Subhash; Roy, Vipul

    2010-02-15

    Orthotopic and living related liver transplantation is an established mode of treatment of end-stage liver disease. One of the major causes of postoperative complications is vascular anastomotic stenosis. One such set of such complications relates to hepatic vein, inferior vena cava (IVC), or portal vein stenosis, with a reported incidence of 1-3%. The incidence of vascular complications is reported to be higher in living donor versus cadaveric liver transplants. We encountered a patient with hepatic venous outflow tract obstruction, where the hepatic vein had been previously stented, but the patient continued to have symptoms due to additional IVC obstruction. The patient required double-balloon dilatation of the IVC simultaneously from the internal jugular vein and IVC.

  7. Pulmonary arteriovenous malformations.

    PubMed

    Shovlin, Claire L

    2014-12-01

    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

  8. 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

  9. Treatment of complex periorbital venolymphatic malformation in a neonate with a combination therapy of sirolimus and prednisolone.

    PubMed

    Kim, David; Benjamin, Latanya; Wysong, Ashley; Hovsepian, David; Teng, Joyce

    2015-01-01

    Venolymphatic malformations (VLMs) are vascular anomalies consisting of both veins and lymph vessels. A 2-week-old newborn presented with large VLMs on the left forehead, temple, preauricular area, and orbit. Patient was at imminent risk for permanent vision loss due to a localized mass effect. Surgical excision or debulking was contraindicated due to its complexity and proximity to the left eye, and the patient failed to respond to the sildenafil treatment and sclerotherapy. Patient was subsequently started on oral sirolimus 0.8 mg/m(2) twice daily in combination with prednisolone 2 mg/kg daily. The patient had an excellent therapeutic outcome for 7 months with complete preservation of vision before treatment was discontinued. However, 2 months after the medical treatments were discontinued, her VLM rebounded. She responded to the combination therapy again after a failed treatment with the mTOR inhibitor alone. This case demonstrates that the sirolimus and prednisolone combination therapy could be beneficial for treatment of complex VLM intractable to other treatments. PMID:25753853

  10. Revealing Maximal Diameter of Upper Limb Superficial Vein with an Elevated Environmental Temperature.

    PubMed

    Irfan, Hira; Ooi, Guo Shen; Kyin, May M; Ho, Pei

    2016-01-01

    Ultrasonography is the primary tool for preoperative analysis of vein morphology for fistula creation in patients with end-stage renal disease. This study examines the effect of environmental temperature on the superficial vein size. Superficial veins of thirteen healthy volunteers were marked at three sites: cephalic vein in left lateral arm near cubital fossa, cephalic vein in left forearm at wrist, and basilic vein in left medial arm near cubital fossa. Mean diameters were recorded using ultrasound probe at 26°C and 43°C. Body temperature was increased using a Bair Hugger blanket. Mean values from the two temperatures were analyzed using paired sample t-test. All three superficial vein sites displayed statistically significant increase in diameter when the temperature was increased from 26°C to 43°C. Paired t-test showed p values of 0.001 for cephalic vein at wrist, 0.01 for cephalic vein near cubital fossa, and 0.01 for basilic vein near cubital fossa. This study proved that environmental temperature exerts a statistically significant effect on vein size measured by ultrasound during preoperative assessment for vascular access. Not to the extent of 43°C, the authors would recommend setting the room temperature higher during ultrasound vascular assessment to avoid underestimating the superficial vein size. PMID:27597987

  11. Revealing Maximal Diameter of Upper Limb Superficial Vein with an Elevated Environmental Temperature

    PubMed Central

    Ooi, Guo Shen; Kyin, May M.

    2016-01-01

    Ultrasonography is the primary tool for preoperative analysis of vein morphology for fistula creation in patients with end-stage renal disease. This study examines the effect of environmental temperature on the superficial vein size. Superficial veins of thirteen healthy volunteers were marked at three sites: cephalic vein in left lateral arm near cubital fossa, cephalic vein in left forearm at wrist, and basilic vein in left medial arm near cubital fossa. Mean diameters were recorded using ultrasound probe at 26°C and 43°C. Body temperature was increased using a Bair Hugger blanket. Mean values from the two temperatures were analyzed using paired sample t-test. All three superficial vein sites displayed statistically significant increase in diameter when the temperature was increased from 26°C to 43°C. Paired t-test showed p values of 0.001 for cephalic vein at wrist, 0.01 for cephalic vein near cubital fossa, and 0.01 for basilic vein near cubital fossa. This study proved that environmental temperature exerts a statistically significant effect on vein size measured by ultrasound during preoperative assessment for vascular access. Not to the extent of 43°C, the authors would recommend setting the room temperature higher during ultrasound vascular assessment to avoid underestimating the superficial vein size. PMID:27597987

  12. 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. PMID:26815877

  13. Why study human limb malformations?

    PubMed Central

    Wilkie, Andrew OM

    2003-01-01

    Congenital limb malformations occur in 1 in 500 to 1 in 1000 human live births and include both gross reduction defects and more subtle alterations in the number, length and anatomy of the digits. The major causes of limb malformations are abnormal genetic programming and intra-uterine disruption to development. The identification of causative gene mutations is important for genetic counselling and also provides insights into the mechanisms controlling limb development. This article illustrates some of the lessons learnt from the study of human limb malformation, organized into seven categories. These are: (1) identification of novel genes, (2) allelic mutation series, (3) pleiotropy, (4) qualitative or (5) quantitative differences between mouse and human development, (6) physical and teratogenic disruption, and (7) unusual biological phenomena. PMID:12587917

  14. Retinal vein occlusion: current treatment.

    PubMed

    Lattanzio, Rosangela; Torres Gimeno, Ana; Battaglia Parodi, Maurizio; Bandello, Francesco

    2011-01-01

    Retinal vein occlusion (RVO) is a pathology noted for more than 150 years. Although a lot has been written on the matter, it is still a frequent condition with multifactorial etiopathogenesis with many unclear aspects. The RVO pathogenesis has varied systemic and local implications that make it difficult to elaborate treatment guidelines. The management of the patient with RVO is very complex and a multidisciplinary approach is required in order to identify and correct the associated risk factors. Laser therapy remains the gold standard in RVO, but only modest functional improvement has been shown in branch retinal occlusion forms. Multicenter studies of intravitreal drugs present them as an option to combine with laser. Anti-vascular endothelial growth factor, corticosteroids and sustained-release implants are the future weapons to stop disease progression and get a better visual outcome. Consequently, it is useful to clarify some aspects of the pathology that allow a better patient management. PMID:20938213

  15. 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

  16. A novel technique for hepatic vein reconstruction during hepatectomy.

    PubMed

    Surjan, Rodrigo C; Basseres, Tiago; Pajecki, Denis; Puzzo, Daniel B; Makdissi, Fabio F; Machado, Marcel A C; Battilana, Alexandre Gustavo Bellorio

    2016-01-01

    Surgical resection is the treatment of choice for malignant liver tumours. Nevertheless, surgical approach to tumours located close to the confluence of the hepatic veins is a challenging issue. Trisectionectomies are considered the first curative option for treatment of these tumours. However, those procedures are associated with high morbidity and mortality rates primarily due to post-operative liver failure. Thus, maximal preservation of functional liver parenchyma should always be attempted. We describe the isolated resection of Segment 8 for the treatment of a tumour involving the right hepatic vein and in contact with the middle hepatic vein and retrohepatic vena cava with immediate reconstruction of the right hepatic vein with a vascular graft. This is the first time this type of reconstruction was performed, and it allowed to preserve all but one of the hepatic segments with normal venous outflow. This innovative technique is a fast and safe method to reconstruct hepatic veins. PMID:27076622

  17. A novel technique for hepatic vein reconstruction during hepatectomy

    PubMed Central

    Surjan, Rodrigo C.; Basseres, Tiago; Pajecki, Denis; Puzzo, Daniel B.; Makdissi, Fabio F.; Machado, Marcel A.C.; Battilana, Alexandre Gustavo Bellorio

    2016-01-01

    Surgical resection is the treatment of choice for malignant liver tumours. Nevertheless, surgical approach to tumours located close to the confluence of the hepatic veins is a challenging issue. Trisectionectomies are considered the first curative option for treatment of these tumours. However, those procedures are associated with high morbidity and mortality rates primarily due to post-operative liver failure. Thus, maximal preservation of functional liver parenchyma should always be attempted. We describe the isolated resection of Segment 8 for the treatment of a tumour involving the right hepatic vein and in contact with the middle hepatic vein and retrohepatic vena cava with immediate reconstruction of the right hepatic vein with a vascular graft. This is the first time this type of reconstruction was performed, and it allowed to preserve all but one of the hepatic segments with normal venous outflow. This innovative technique is a fast and safe method to reconstruct hepatic veins. PMID:27076622

  18. Evolutionary origin of cardiac malformations.

    PubMed

    Taussig, H B

    1988-10-01

    The author has proposed in previous publications that isolated cardiac malformations have an evolutionary origin. This is partly supported by the fact that isolated cardiac malformations found in humans occur also in other placental mammals as well as in birds. External gross examination of the heart in just over 5,000 birds was carried out during a 3 year period. Anomalies included one instance of duplicate hearts, two specimens in which no heart could be identified and in a fourth, a yellow-rumped warbler, the heart lay in the neck outside of the thoracic cavity. Published reports of similar occurrences of an ectopically placed heart concern birds, cattle and humans. The fact that various species of both placental mammals and birds show evidence of heritability for heart defects, and that these species cannot interbreed, combined with the fact that birds and mammals have many similar malformations, points to either a common external causative factor or a common origin. Genes that code the malformed heart must be transmitted with that part of the genetic makeup common to all birds and mammals. Malformations caused by teratogens produce widespread organ injury to a potentially normal embryo whereas the evolutionary malformation is an organ-specific anomaly in an otherwise normal mammal or bird and occurs in widely separated species. The implications of this theory are important for parents of children with an isolated congenital heart defect who may have ingested one or another drug or chemical or have been exposed to toxins or infectious agents before or after conception of the affected offspring. PMID:3047192

  19. Varicose veins and venous insufficiency

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/001109.htm Varicose veins and venous insufficiency To use the sharing features on this page, please enable JavaScript. Varicose veins are swollen, twisted, and enlarged veins that you ...

  20. Imaging of congenital pulmonary malformations.

    PubMed

    Praticò, Francesco Emanuele; Corrado, Michele; Della Casa, Giovanni; Parziale, Raffaele; Russo, Giuseppe; Gazzani, Silvia Eleonora; Rossi, Enrica; Borgia, Daniele; Mostardi, Maurizio; Bacchini, Emanuele; Cella, Simone; De Filippo, Massimo

    2016-01-01

    Congenital pulmonary malformations represent a broad spectrum of anomalies that may result in varied clinical and pathologic pictures, ranging from recurrent pulmonary infections and acute respiratory distress syndrome, which require timely drug therapy, up to large space-occupying lesions needing surgical treatment. This classification includes three distinct anatomical and pathological entities, represented by Congenital Cystic Adenomatoid Malformation, Bronchopulmonary Sequestration and Congenital Lobar Emphysema. The final result in terms of embryological and fetal development of these alterations is a Congenital Lung Hypoplasia. Since even Bronchial Atresia, Pulmonary Bronchogenic Cysts and Congenital Diaphragmatic Hernias are due to Pulmonary Hypoplasia, these diseases will be discussed in this review (1, 2). PMID:27467867

  1. Novel Vein Patterns in Arabidopsis Induced by Small Molecules1[OPEN

    PubMed Central

    Cutler, Sean

    2016-01-01

    The critical role of veins in transporting water, nutrients, and signals suggests that some key regulators of vein formation may be genetically redundant and, thus, undetectable by forward genetic screens. To identify such regulators, we screened more than 5000 structurally diverse small molecules for compounds that alter Arabidopsis (Arabidopsis thaliana) leaf vein patterns. Many compound-induced phenotypes were observed, including vein networks with an open reticulum; decreased or increased vein number and thickness; and misaligned, misshapen, or nonpolar vascular cells. Further characterization of several individual active compounds suggests that their targets include hormone cross talk, hormone-dependent transcription, and PIN-FORMED trafficking. PMID:26574596

  2. Genetics Home Reference: Dandy-Walker malformation

    MedlinePlus

    ... All Close All Description Dandy-Walker malformation affects brain development, primarily development of the cerebellum , which is the ... Walker malformation , signs and symptoms caused by abnormal brain development are present at birth or develop within the ...

  3. Arteriovenous malformation of nose-revision surgery.

    PubMed

    Srinivas, C V; Kailash, N; Kailas, Gayattre; Divya Jyothi, N

    2012-12-01

    Areteriovenous malformations are rare in the head and neck region and generally arise from intracranial vessels. We present one rare case with spontaneous arteriovenous malformations related to the nose. PMID:24294582

  4. 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.

  5. Demonstration of normal and dilated testicular veins by multidetector computed tomography.

    PubMed

    Karcaaltincaba, Musturay

    2011-04-01

    Recent advances in multidetector computed tomography (MDCT) technology enabled better visualization of testicular (gonadal) vein using submillimeter slice thickness and three-dimensional images. Normally, the testicular vein measures 1-3 mm and drains into the inferior vena cava and left renal vein on the right and left sides, respectively. They can be seen in most patients during MDCT studies. Curved planar and volume-rendered images can be used to display testicular veins. We aim to demonstrate MDCT findings of normal testicular vein and its pathologies including varicocele, varices, the testicular vascular pedicle sign, and phlebolith. The testicular vein can be dilated owing to varicocele or portal hypertension and in patients with intraabdominal seminomas arising from undescended testis. The testicular vein can also cause ureteral compression at the crossing point. Understanding MDCT findings of the normal testicular vein and its various pathologies can allow a correct diagnosis, thereby avoiding further diagnostic tests. PMID:21519988

  6. Pulmonary vascular diseases.

    PubMed

    Mélot, C; Naeije, R

    2011-04-01

    Diseases of the pulmonary vasculature are a cause of increased pulmonary vascular resistance (PVR) in pulmonary embolism, chronic thromboembolic pulmonary hypertension (CTEPH), and pulmonary arterial hypertension or decreased PVR in pulmonary arteriovenous malformations on hereditary hemorrhagic telangiectasia, portal hypertension, or cavopulmonary anastomosis. All these conditions are associated with a decrease in both arterial PO2 and PCO2. Gas exchange in pulmonary vascular diseases with increased PVR is characterized by a shift of ventilation and perfusion to high ventilation-perfusion ratios, a mild to moderate increase in perfusion to low ventilation-perfusion ratios, and an increased physiologic dead space. Hypoxemia in these patients is essentially explained by altered ventilation-perfusion matching amplified by a decreased mixed venous PO2 caused by a low cardiac output. Hypocapnia is accounted for by hyperventilation, which is essentially related to an increased chemosensitivity. A cardiac shunt on a patent foramen ovale may be a cause of severe hypoxemia in a proportion of patients with pulmonary hypertension and an increase in right atrial pressure. Gas exchange in pulmonary arteriovenous malformations is characterized by variable degree of pulmonary shunting and/or diffusion-perfusion imbalance. Hypocapnia is caused by an increased ventilation in relation to an increased pulmonary blood flow with direct peripheral chemoreceptor stimulation by shunted mixed venous blood flow. PMID:23737196

  7. A new venture with sclerotherapy in an oral vascular lesion.

    PubMed

    Sitra, G; Kayalvizhi, E B; Sivasankari, T; Vishwanath, R

    2014-12-01

    Vascular malformations are one of the most common lesions of the oral cavity. The lesion may be a congenital malformation observed in neonates or arteriovenous malformation observed in adults. Various surgical and medical managements are possible for vascular lesions which include surgical excision, laser therapy, cryotherapy, selective embolization, intralesional sclerosing agents, β-blockers and steroid therapy. Here we report a case of oral vascular lesion where intralesional injection with 30 mg/ml of sodium tetradecyl sulfate (STS) was given, which resulted in local complications with severe inflammatory response including pain, swelling, and surface ulceration that remained for 2 weeks. Sclerotherapy with STS still remain an effective agent in treating benign oral vascular lesions and provides alternative or support for surgical methods. Sometimes it can lead to undesirable complications like allergic reactions, local inflammatory response, etc. PMID:25538471

  8. Congenital subcutaneous arteriovenous malformation in a puppy: diagnosis with CT angiography.

    PubMed

    Proks, Pavel; Stehlik, Ladislav; Paninarova, Michaela; Irova, Katarina; Lorenzova, Jana; Necas, Alois

    2015-10-01

    A 4-month-old, 20 kg, intact male, cane corso dog was presented with a slowly growing subcutaneous lesion on the left caudoventral abdominal wall. Ultrasound and computed tomography angiography revealed a subcutaneous plexus of aberrant tortuous vessels directly connected with the superficial branch of the deep circumflex iliac artery and vein. The arteriovenous malformation (AVM) was successfully surgically removed. Early recognition and surgical removal of AVM can have excellent cosmetic results and prevents potential cardiovascular complications. PMID:26175066

  9. Reconstruction of portal vein and superior mesenteric vein after extensive resection for pancreatic cancer

    PubMed Central

    Kim, Suh Min; Park, Daedo; Min, Sang-Il; Jang, Jin-Young; Kim, Sun-Whe; Ha, Jongwon; Kim, Sang Joon

    2013-01-01

    Purpose Tumor invasion to the portal vein (PV) or superior mesenteric vein (SMV) can be encountered during the surgery for pancreatic cancer. Venous reconstruction is required, but the optimal surgical methods and conduits remain in controversies. Methods From January 2007 to July 2012, 16 venous reconstructions were performed during surgery for pancreatic cancer in 14 patients. We analyzed the methods, conduits, graft patency, and patient survival. Results The involved veins were 14 SMVs and 2 PVs. The operative methods included resection and end-to-end anastomosis in 7 patients, wedge resection with venoplasty in 2 patients, bovine patch repair in 3 patients, and interposition graft with bovine patch in 1 patient. In one patient with a failed interposition graft with great saphenous vein (GSV), the SMV was reconstructed with a prosthetic interposition graft, which was revised with a spiral graft of GSV. Vascular morbidity occurred in 4 cases; occlusion of an interposition graft with GSV or polytetrafluoroethylene, segmental thrombosis and stenosis of the SMV after end-to-end anastomosis. Patency was maintained in patients with bovine patch angioplasty and spiral vein grafts. With mean follow-up of 9.8 months, the 6- and 12-month death-censored graft survival rates were both 81.3%. Conclusion Many of the involved vein segments were repaired primarily. When tension-free anastomosis is impossible, the spiral grafts with GSV or bovine patch grafts are good options to overcome the size mismatch between autologous vein graft and portomesenteric veins. Further follow-up of these patients is needed to demonstrate long-term patency. PMID:23741692

  10. 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

  11. Perivascular mast cells regulate vein graft neointimal formation and remodeling

    PubMed Central

    Grassia, Gianluca; Cambrook, Helen; Ialenti, Armando; MacRitchie, Neil; Carberry, Jaclyn; Lawrence, Catherine

    2015-01-01

    Objective. Emerging evidence suggests an important role for mast cells in vein graft failure. This study addressed the hypothesis that perivascular mast cells regulate in situ vascular inflammatory and proliferative responses and subsequent vein graft neointimal lesion formation, using an optimized local mast cell reconstitution method. Methods and Results. Neointimal hyperplasia was induced by insertion of a vein graft into the right carotid artery in wild type and mast cell deficient KitW−sh/W−sh mice. In some experiments, mast cells were reconstituted systemically (tail vein injection of bone marrow-derived mast cells) or locally (directly into the right neck area) prior to vein grafting. Vein graft neointimal lesion formation was significantly (P < 0.05) reduced in KitW−sh/W−sh mice. Mast cell deficiency reduced the number of proliferating cells, and inhibited L-selectin, CCL2, M-CSF and MIP-3α expression in the vein grafts. Local but not systemic mast cell reconstitution restored a perivascular mast cell population that subsequently promoted neointimal formation in mast cell deficient mice. Conclusion. Our data demonstrate that perivascular mast cells play a key role in promoting neointima formation by inducing local acute inflammatory and proliferative responses. These results suggest that ex vivo intraoperative targeting of mast cells may have therapeutic potential for the prevention of pathological vein graft remodeling. PMID:26312183

  12. Retinal vein occlusion

    MedlinePlus

    ... Berrocal MH, Rodriguez FJ, et al. Pan-American Collaborative Retina Study Group (PACORES). Comparison of two doses ... retinal vein occlusion: results from the Pan-American Collaborative Retina Study Group at 6 months of follow- ...

  13. Deep vein thrombosis - discharge

    MedlinePlus

    You were treated for deep venous thrombosis (DVT). This is a condition in which a blood clot forms in a vein that is not on ... especially if it gets worse upon taking a deep breath in You cough up blood

  14. Popliteal vein aneurysm.

    PubMed

    Falkowski, A; Poncyljusz, W; Zawierucha, D; Kuczmik, W

    2006-06-01

    The incidence of a popliteal vein aneurysm is extremely low. Two cases of this rare venous anomaly are described. The epidemiology, morphology, and diagnostic methods are discussed and the potentially dangerous complications and treatment methods are presented. PMID:16796307

  15. Vascular anomalies of the head and neck in children

    PubMed Central

    Mahady, Kate; Thust, Stefanie; Berkeley, Rupert; Stuart, Sam; Barnacle, Alex; Robertson, Fergus

    2015-01-01

    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. PMID:26807370

  16. 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. PMID:26807370

  17. Surgical and Endovascular Treatment for Spinal Arteriovenous Malformations

    PubMed Central

    ENDO, Toshiki; ENDO, Hidenori; SATO, Kenichi; MATSUMOTO, Yasushi; TOMINAGA, Teiji

    2016-01-01

    Spinal arteriovenous malformation (AVM) is a broad term that constitutes diverse vascular pathologies. To date, various classification schemes for spinal AVM have been proposed in literature, which helped neurosurgeons understand the pathophysiology of the disease and determine an optimal treatment strategy. To discuss indications and results of surgical and endovascular interventions for spinal AVM, this article refers to the following classification proposed by Anson and Spetzler in 1992: type I, dural arteriovenous fistula (AVF); type II, glomus intramedullary AVM; type III, juvenile malformations; and type IV, perimedullary AVF. In general, complete obliteration of the fistula is a key for better outcome in type I dural and type IV perimedullary AVFs. On the other hand, in type II glomus and type III juvenile malformations, functional preservation, instead of pursuing angiographical cure, is the main goal of the treatment. In such cases, reduction of the shunt flow can alleviate clinical symptoms. Proper management of spinal AVM should start with neurological examination and understanding of angioarchitectures, which provide critical information that guides the indication and modality of intervention. Finally, close collaboration of the microsurgical and endovascular teams are mandatory for successful treatment. PMID:26948701

  18. 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

  19. Pain evoked by polymodal stimulation of hand veins in humans.

    PubMed

    Arndt, J O; Klement, W

    1991-01-01

    1. To explore the function of the sensory innervation of veins in humans we used a psychophysical approach to study painful and non-painful sensations by applying polymodal stimuli (electrical, stretch, cold/heat and osmotic) inside vascularly isolated hand vein segments before and after blockade of either venous or cutaneous afferents. 2. All modes of stimulation elicited pain, which showed only slight adaptation during 10 min of maintained stimulation. Pain increased monotonically with stimulus intensity between threshold and the maximally tolerable pain. 3. The exponents of the power functions of the pain magnitude-stimulus strength relations for five stimulus modes ranged between 2.5 and 3.3 but did not significantly differ from one another (P = 0.3). 4. Pain evoked by all stimuli was reported to be of similar quality, i.e. sharp, aching and unpleasant; it was accompanied by non-painful sensations (skin movements on stretching, warm and cold sensation with intravenous thermal stimulation) unless the skin above the stimulated vein segment was numbed with benzocaine ointment. 5. Pain could no longer be evoked in the presence of 0.4-0.8% procaine within the stimulated vein segment. 6. These observations are consistent with the view that veins are invested with polymodal nociceptors only, which in all likelihood are connected with thinly myelinated afferents of the A delta group. 7. The vascularly isolated vein segment may open a new avenue for pain research in humans. PMID:1804973

  20. Medical Therapy for Pediatric Vascular Anomalies

    PubMed Central

    Margolin, Judith F.; Soni, Heather Mills; Pimpalwar, Sheena

    2014-01-01

    Vascular anomalies (VAs) comprise a large variety of individual diagnoses that in different phases of treatment require a diverse number of medical specialists to provide optimal care. Medical therapies include agents usually associated with cancer chemotherapy, such as vincristine, as well more immunomodulatory types of drugs, such as glucocorticoids and sirolimus. These immunomodulating drugs are being successfully applied in cases that are typically categorized as vascular tumors, including kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), as well as some of the more invasive types of vascular malformations (i.e., microcystic lymphatic malformations and blue rubber bleb nevus syndrome (BRBNS). These therapies need to be combined with good supportive care, which often involves anticoagulation, antimicrobial prophylaxis, and comprehensive pain and symptom-relief strategies, as well as appropriate drug monitoring and management of side effects of medical treatment. The optimal care of these patients frequently involves close collaboration between surgeons, interventional and conventional radiologists, medical subspecialists, and nurses. PMID:25045333

  1. Ileocolic Arteriovenous Fistula with Superior Mesenteric Vein Aneurism: Endovascular Treatment

    SciTech Connect

    Gregorio, Miguel Angel de; Gimeno, Maria Jose; Medrano, Joaquin; Schoenholz, Caudio; Rodriguez, Juan; D'Agostino, Horacio

    2004-09-15

    We report a case of a venous aneurysm secondary to an acquired ileocolic arteriovenous fistula in a 64-year-old woman with recurrent abdominal pain and history of appendectomy. The aneurysm was diagnosed by ultrasound and computed tomography. Angiography showed an arteriovenous fistula between ileocolic branches of the superior mesenteric artery and vein. This vascular abnormality was successfully treated with coil embolization.

  2. Stereotactic radiosurgery for intramedullary spinal arteriovenous malformations.

    PubMed

    Kalani, Maziyar A; Choudhri, Omar; Gibbs, Iris C; Soltys, Scott G; Adler, John R; Thompson, Patricia A; Tayag, Armine T; Samos, Cindy H; Chang, Steven D

    2016-07-01

    Spinal cord arteriovenous malformations (AVM) are rare lesions associated with recurrent hemorrhage and progressive ischemia. Occasionally a favorable location, size or vascular anatomy may allow management with endovascular embolization and/or microsurgical resection. For most, however, there is no good treatment option. Between 1997 and 2014, we treated 37 patients (19 females, 18 males, median age 30years) at our institution diagnosed with intramedullary spinal cord AVM (19 cervical, 12 thoracic, and six conus medullaris) with CyberKnife (Accuray, Sunnyvale, CA, USA) stereotactic radiosurgery. A history of hemorrhage was present in 50% of patients. The mean AVM volume of 2.3cc was treated with a mean marginal dose of 20.5Gy in a median of two sessions. Clinical and MRI follow-up were carried out annually, and spinal angiography was repeated at 3years. We report an overall obliteration rate of 19% without any post-treatment hemorrhagic events. In those AVM that did not undergo obliteration, significant volume reduction was noted at 3years. Although the treatment paradigm for spinal cord AVM continues to evolve, radiosurgical treatment is capable of safely obliterating or significantly shrinking most intramedullary spinal cord AVM. PMID:26869363

  3. A giant frontal cavernous malformation with review of literature

    PubMed Central

    Sharma, Arvind; Mittal, Radhey Shyam

    2016-01-01

    Cavernous malformations (CMs) are vascular anomalies with dilated spaces called caverns. These spaces are lined by endothelial cells and collage and devoid of smooth muscle or intervening neural tissue, and filled with blood at various stages of stasis, thrombosis, organization, and calcification. Most CMs are relatively small in size but when they are large enough they can produce sing of mass effect and may simulate neoplastic, vascular, inflammatory pathology. Giant CM (size >6 cm) are very rare lesions and very few cases are reported in world literature. We are reporting such a rare case of a 16 year male. Our case is also unique in the sense that it is the largest reported CM in Indian population. PMID:27114662

  4. 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.

  5. Congenital malformations in diabetic offspring.

    PubMed

    Temesio, P; Belitzky, R; Gallego, L; Martell, M; Pose, S V

    1977-01-01

    A retrospective study of 215 deliveries in diabetic mothers at Hospital de Clínicas (Montevideo, Uruguay) has been performed. Presence of congenital malformations (CM) was considered in relation to age of the mothers, class of maternal diabetes, maternal angiopathy, treatment and metabolic control. The prevalence of CM in the series was 9.8%. None of the factors analyzed seems to be statistically linked to the prevalence of CM. PMID:613685

  6. Extrinsic and intrinsic veins of the equine hoof wall.

    PubMed Central

    Mishra, P C; Leach, D H

    1983-01-01

    The extrinsic and intrinsic venous drainage of the equine hoof have previously been poorly described. There is also an absence of information about the venous organisation of both primary and secondary dermal lamellae of the hoof wall. The purpose of this study was to describe the normal venous pattern of the horse hoof, especially that associated with the dermal lamellae of the wall. The venous organisation of 46 pairs of hooves from clinically healthy horses was studied using standard plastic vascular corrosion casts and histological sections. A consistent pattern of venous organisation was observed in the toe, quarter and heel regions. Veins in the proximal half of the wall corium form an exteriorly and an interiorly located venous plexus, while in the distal wall corium only an exteriorly located venous plexus is present. The wall corium of the toe and quarters is drained primarily by veins contributing to the coronary venous plexus and to the circumflex vein. In the heel regions this venous drainage network is supplemented by tributaries of the caudal hoof vein, independent superficial vein and anastomotic branches from the circumflex vein to the inner venous plexus. Because of this supplementary venous return the heel region is apparently better drained than other areas of the hoof wall. The wall corium is drained distally by the circumflex vein. The coronary venous plexus is composed of an exteriorly situated short superficial collecting venous plexus and an interiorly situated deep collecting venous plexus. The short superficial collecting veins, which drain the proliferating zone of epidermis of the coronary border, connect directly to the coronary and subcoronary veins. The deep collecting veins drain only the proximal wall corium. Most of the veins that drain the major portions of venous blood from the hoof are situated at the palmar aspect of the digit. These veins drain venous blood from the coronary venous plexus, the inner venous plexus and the

  7. [Maternal imagination and congenital malformations].

    PubMed

    Van Heiningen, Teunis Willem

    2011-01-01

    Since antiquity philosophers and scientists tried to explain the cause of congenital malformations. In early modern medicine maternal imagination was largely accepted as their true cause, This concept was rejected by Blondel, a London physician. Around 1750 Wolff introduced the Hemmungsbildung as the cause of congenital malformations, a concept adopted in 1781 by Blumenbach. Later on Soemmerring (1784), Crichton (1785) and Meckel the younger adopted Blumenbach's concept. In 1824 Suringar further developed it. More and more the excessive development of fetal blood vessels or nerves was rejected as a possible cause, although from time to time these ideas were adopted again. In the early 1800s Etienne Geoffroy Saint-Hilaire (1811) and Vrolik (1817) developed a classification of monstra. These attempts urged Isidore Geoffroy Saint-Hilaire (Etienne's son) and Vrolik the younger (Gerard's son) to develop it further. Nevertheless, around 1840 Vrolik had to admit that although we are well acqainted with the various malformations, we are still ignorant of the primary cause of these phenomena. Meanwhile the dispute between the adherents of the theory of preformation and those who had adopted the concept of epigenesis exercised many minds. In the second half of the eighteenth century the latter theory became more and more adopted and this fact cleared the way for the ideas introduced by Wolff and Blumenbach, because it was consistent with the idea of a gradual development of fetal structures. PMID:22073754

  8. 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

  9. 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

  10. Vascular trauma historical notes.

    PubMed

    Rich, Norman M

    2011-03-01

    This article provides a brief historical review of treatment of vascular trauma. Although methods for ligation came into use in the second century, this knowledge was lost during the Dark Ages and did not come back until the Renaissance. Many advances in vascular surgery occurred during the Balkan Wars, World War I, and World War II, although without antibiotics and blood banking, the philosophy of life over limb still ruled. Documenting and repairing both arteries and veins became more common during the Korean and Vietnam conflicts. Increased documentation has revealed that the current conflicts have resulted in more arterial injuries than in previous wars, likely because of improved body armor, improvised explosive device attacks, tourniquet use, and improved medical evacuation time. This brief review emphasizes the great value of mentorship and the legacy of the management of arterial and venous injuries to be passed on. PMID:21502112

  11. Vascular Thoracic Outlet Syndrome.

    PubMed

    Hussain, Mohamad Anas; Aljabri, Badr; Al-Omran, Mohammed

    2016-01-01

    Two distinct terms are used to describe vascular thoracic outlet syndrome (TOS) depending on which structure is predominantly affected: venous TOS (due to subclavian vein compression) and arterial TOS (due to subclavian artery compression). Although the venous and arterial subtypes of TOS affect only 3% and <1% of all TOS patients respectively, the diagnostic and management approaches to venous and arterial TOS have undergone considerable evolution due to the recent emergence of minimally invasive endovascular techniques such as catheter-directed arterial and venous thrombolysis, and balloon angioplasty. In this review, we discuss the anatomical factors, etiology, pathogenesis and clinical presentation of vascular TOS patients. In addition, we use the most up to date observational evidence available to provide a contemporary approach to the diagnosis and management of venous TOS and arterial TOS patients. PMID:27568153

  12. Pelvic Vein Embolisation in the Management of Varicose Veins

    SciTech Connect

    Ratnam, Lakshmi A.; Marsh, Petra; Holdstock, Judy M.; Harrison, Charmaine S.; Hussain, Fuad F.; Whiteley, Mark S.; Lopez, Anthony

    2008-11-15

    Pelvic vein incompetence is common in patients with atypical varicose veins, contributing to their recurrence after surgery. Therefore, refluxing pelvic veins should be identified and treated. We present our experience with pelvic vein embolisation in patients presenting with varicose veins. Patients presenting with varicose veins with a duplex-proven contribution from perivulval veins undergo transvaginal duplex sonography (TVUS) to identify refluxing pelvic veins. Those with positive scans undergo embolisation before surgical treatment of their lower limb varicose veins. A total of 218 women (mean age of 46.3 years) were treated. Parity was documented in the first 60 patients, of whom 47 (78.3%) were multiparous, 11 (18.3%) had had one previous pregnancy, and 2 (3.3%) were nulliparous. The left ovarian vein was embolised in 78%, the right internal iliac in 64.7%, the left internal iliac in 56.4%, and the right ovarian vein in 42.2% of patients. At follow-up TVUS, mild reflux only was seen in 16, marked persistent reflux in 6, and new reflux in 3 patients. These 9 women underwent successful repeat embolisation. Two patients experienced pulmonary embolisation of the coils, of whom 1 was asymptomatic and 1 was successfully retrieved; 1 patient had a misplaced coil protruding into the common femoral vein; and 1 patient had perineal thrombophlebitis. The results of our study showed that pelvic venous embolisation by way of a transjugular approach is a safe and effective technique in the treatment of pelvic vein reflux.

  13. Ovarian vein thrombosis

    PubMed Central

    Jenayah, Amel Achour; Saoudi, Sarra; Boudaya, Fethia; Bouriel, Ines; Sfar, Ezzeddine; Chelli, Dalenda

    2015-01-01

    Ovarian vein thrombosis (OVT) is a rare cause of abdominal pain that may mimic a surgical abdomen. It is most often diagnosed during the postpartum period. In this report, we present four cases of postoperative ovarian vein thrombosis. The complications of OVT can be significant, and the diagnosis relies on a careful examination of the radiographic findings. It can occur with lower quadrant abdominal pain, especially in the setting of recent pregnancy, abdominal surgery, pelvic inflammatory disease, or malignancy. Diagnosis can be made with confidence using ultrasound, computed tomography or magnetic resonance imaging. Treatment of ovarian vein thrombosis is particularly important in the post-partum patients, with anticoagulation therapy being the current recommendation. PMID:26526119

  14. Transsplenic portal vein reconstruction-transjugular intrahepatic portosystemic shunt in a patient with portal and splenic vein thrombosis.

    PubMed

    Salsamendi, Jason T; Gortes, Francisco J; Shnayder, Michelle; Doshi, Mehul H; Fan, Ji; Narayanan, Govindarajan

    2016-09-01

    Portal vein thrombosis (PVT) is a potential complication of cirrhosis and can worsen outcomes after liver transplant (LT). Portal vein reconstruction-transjugular intrahepatic portosystemic shunt (PVR-TIPS) can restore flow through the portal vein (PV) and facilitate LT by avoiding complex vascular conduits. We present a case of transsplenic PVR-TIPS in the setting of complete PVT and splenic vein (SV) thrombosis. The patient had a 3-year history of PVT complicated by abdominal pain, ascites, and paraesophageal varices. A SV tributary provided access to the main SV and was punctured percutaneously under ultrasound scan guidance. PV access, PV and SV venoplasty, and TIPS placement were successfully performed without complex techniques. The patient underwent LT with successful end-to-end anastomosis of the PVs. Our case suggests transsplenic PVR-TIPS to be a safe and effective alternative to conventional PVR-TIPS in patients with PVT and SV thrombosis. PMID:27594947

  15. [Vascular anomaly in the midcheek region of an infant--review of the diagnostic procedure].

    PubMed

    Rossler, L; Sander, V; Teuber, I; Stücker, M; Kreuter, A; Stricker, I; Hamelmann, E

    2015-05-01

    Clinical history, physical examination, evolution and imaging findings (Colour Doppler sonography, MRI if available) are of pivotal importance in the diagnostic pathway of an infantile vascular anomaly. Histopathology with specific stains and markers is contributive in difficult cases. Differentiation between vascular tumors (hemangioma) and vascular malformations is now well known and integrated into the ISSVA classification. We report here a 6-months-old boy, who presented with a localized cutaneous and expansive vascular birthmark in the left cheek and developed bleedings at the age of 18 months. Diagnostic features of a hemangioma were not evident, and the final diagnosis of a venous malformation was confirmed by histopathology. PMID:25985453

  16. Varicose vein surgery.

    PubMed

    Kendler, Micheal; Fellmer, Peter T; Wetzig, Tino

    2012-03-01

    Venous diseases are common in the general population. After a comprehensive diagnostic evaluation, an individual therapeutic approach should be selected on the basis of the findings, with the aim of treating the diseased vein segments and improving quality of life. Numerous therapeutic options are available for the treatment of varicose veins. In addition to conservative methods such as compression therapy, exercise or drugs, surgical procedures such as traditional surgery, thermal ablation techniques or sclerotherapy can be performed. Recent developments include the use of endoluminal water vapor or mechano-chemical endovenous ablation. PMID:22222053

  17. Somatic Activating PIK3CA Mutations Cause Venous Malformation.

    PubMed

    Limaye, Nisha; Kangas, Jaakko; Mendola, Antonella; Godfraind, Catherine; Schlögel, Matthieu J; Helaers, Raphael; Eklund, Lauri; Boon, Laurence M; Vikkula, Miikka

    2015-12-01

    Somatic mutations in TEK, the gene encoding endothelial cell tyrosine kinase receptor TIE2, cause more than half of sporadically occurring unifocal venous malformations (VMs). Here, we report that somatic mutations in PIK3CA, the gene encoding the catalytic p110α subunit of PI3K, cause 54% (27 out of 50) of VMs with no detected TEK mutation. The hotspot mutations c.1624G>A, c.1633G>A, and c.3140A>G (p.Glu542Lys, p.Glu545Lys, and p.His1047Arg), frequent in PIK3CA-associated cancers, overgrowth syndromes, and lymphatic malformation (LM), account for >92% of individuals who carry mutations. Like VM-causative mutations in TEK, the PIK3CA mutations cause chronic activation of AKT, dysregulation of certain important angiogenic factors, and abnormal endothelial cell morphology when expressed in human umbilical vein endothelial cells (HUVECs). The p110α-specific inhibitor BYL719 restores all abnormal phenotypes tested, in PIK3CA- as well as TEK-mutant HUVECs, demonstrating that they operate via the same pathogenic pathways. Nevertheless, significant genotype-phenotype correlations in lesion localization and histology are observed between individuals with mutations in PIK3CA versus TEK, pointing to gene-specific effects. PMID:26637981

  18. Chiari Malformations and Syringohydromyelia in Children.

    PubMed

    Poretti, Andrea; Boltshauser, Eugen; Huisman, Thierry A G M

    2016-04-01

    Chiari malformations are a heterogeneous group of disorders with distinct clinical anatomical features all of which involve the hindbrain. Our understanding of Chiari malformations increased tremendously over the past decades, and progress in neuroimaging was instrumental for that. Conventional and advanced neuroimaging of the brain and spine play a key role in the workup of children with suspected Chiari malformations. In addition, neuroimaging studies in Chiari malformations may guide the management, serve as a predictor of outcome, and shed light on the pathogenesis. PMID:27063663

  19. 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

  20. Vascular ring

    MedlinePlus

    ... with aberrant subclavian and left ligamentum ateriosus; Congenital heart defect - vascular ring; Birth defect heart - vascular ring ... accounts for less than 1% of all congenital heart problems. The condition occurs as often in males ...

  1. Nasopharyngeal venous malformation: A rare condition managed with Nd:YAG laser.

    PubMed

    Lee, David R; Richter, Gresham T

    2015-10-01

    Venous malformations are benign but symptomatic vascular lesions of the head and neck that undergo soft tissue infiltration and relentless growth. Here we present a 31-year-old female referred for an obstructing and painful right posterior nasal mass. Flexible nasopharyngoscopy and magnetic resonance imaging demonstrated a 3 cm × 4 cm enhancing mucosal and submucosal venous malformation of the nasopharynx and oropharynx. Three staged episodes of transnasal endoscopically guided Nd:YAG laser therapy were performed with dramatic and sustained reduction in size and symptoms. No bleeding or complications occurred. Nd:YAG laser treatment of nasopharyngeal venous malformations is a safe and effective alternative to open surgical excision and sclerotherapy. PMID:25684672

  2. Surgical management of cavernous malformation of the optic nerve with canalicular extension

    PubMed Central

    Gonçalves, Vítor M.; Gonçalves, Victor

    2014-01-01

    Background: Cavernous malformations arising in a single optic nerves paring the chiasm (intracranial prechiasmatic optic nerve) and expanding into the optic canal are extremely rare lesions. Published series or case reports regarding the surgical removal of these vascular malformations within this specific location are scarce. Case Description: We present the first case to be published, of an intracranial optic nerve cavernous malformation with a contiguous canalicular component that was totally and successfully removed through a microsurgical pterional approach with excellent clinical outcome. Conclusion: This pathology should always be considered in the differential diagnosis of optic neuropathy and visual loss. Early detection and surgical proposal are mandatory, warranting the prevention of permanent damage to visual pathways. Radical resection is challenging, but usually curative and associated with favorable visual outcomes. PMID:25422788

  3. Diffuse malformations of cortical development.

    PubMed

    Bahi-Buisson, Nadia; Guerrini, Renzo

    2013-01-01

    Malformations of cortical development (MCD) represent a major cause of developmental disabilities and severe epilepsy. Advances in imaging and genetics have improved the diagnosis and classification of these conditions. Up to now, eight genes have been involved in different types of MCD. Lissencephaly-pachygyria and subcortical band heterotopia (SBH) represent a malformative spectrum resulting from mutations of either LIS1 or DCX genes. LIS1 mutations cause a more severe malformation in the posterior brain regions. DCX mutations usually cause anteriorly predominant lissencephaly in males and SBH in female patients. Additional forms are X-linked lissencephaly with corpus callosum agenesis and ambiguous genitalia associated with mutations of the ARX gene. Lissencephaly with cerebellar hypoplasia (LCH) encompass heterogeneous disorders named LCH types a to d. LCHa is related to mutation in LIS1 or DCX, LCHb with mutation of the RELN gene, and LCHd could be related to the TUBA1A gene. Polymicrogyria encompasses a wide range of clinical, etiological, and histological findings. Among several syndromes, recessive bilateral fronto-parietal polymicrogyria has been associated with mutations of the GPR56 gene. Bilateral perisylvian polymicrogyria has been associated with mutations in the SRPX2 gene in a few individuals and with linkage to chromosome Xq28 in a some other families. X-linked bilateral periventricular nodular heterotopia (PNH) consists of PNH with focal epilepsy in females and prenatal lethality in males. Filamin A (FLNA) mutations have been reported in some families and in sporadic patients. It is possible to infer the most likely causative gene by brain imaging studies and other clinical findings. PMID:23622213

  4. Portal vein thrombosis.

    PubMed

    Chawla, Yogesh K; Bodh, Vijay

    2015-03-01

    Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion. PMID:25941431

  5. Portal Vein Thrombosis

    PubMed Central

    Chawla, Yogesh K.; Bodh, Vijay

    2015-01-01

    Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion. PMID:25941431

  6. Elastomechanical properties of bovine veins.

    PubMed

    Rossmann, Jenn Stroud

    2010-02-01

    Veins have historically been discussed in qualitative, relative terms: "more compliant" than arteries, subject to "lower pressures". The structural and compositional differences between arteries and veins are directly related to the different functions of these vessels. Veins are often used as grafts to reroute flow from atherosclerotic arteries, and venous elasticity plays a role in the development of conditions such as varicose veins and valvular insufficiency. It is therefore of clinical interest to determine the elastomechanical properties of veins. In the current study, both tensile and vibration testing are used to obtain elastic moduli of bovine veins. Representative stress-strain data are shown, and the mechanical and failure properties reported. Nonlinear and viscoelastic behavior is observed, though most properties show little strain rate dependence. These data suggest parameters for constitutive modeling of veins and may inform the design and testing of prosthetic venous valves as well as vein grafts. PMID:20129420

  7. Living with Deep Vein Thrombosis

    MedlinePlus

    ... page from the NHLBI on Twitter. Living With Deep Vein Thrombosis NHLBI Resources Pulmonary Embolism (Health Topics) Non-NHLBI Resources Deep Vein Thrombosis (MedlinePlus) Pulmonary Embolism (MedlinePlus) Clinical Trials ...

  8. [Jaboulay, vascular surgeon at Lyon].

    PubMed

    Bouchet, Mathieu

    2010-01-01

    Mathieu Jaboulay was an excellent surgeon. He was the first to come up with the principles of vascular surgery as he made a surgical anastomosis between two arteries of a dog by an eversion circular suture. In 1902, he implemented a suture between an artery and a vein in an arteritis by obliteration. Jaboulay also tried the graft of an animal kidney on the crease of a human elbow in 1906. Jaboulay was an initiator for Alexis Carrel. PMID:20527332

  9. NONICHEMIC CENTRAL RETINAL VEIN OCCLUSION ASSOCIATED WITH HEREDITARY THROMBOPHYLIA.

    PubMed

    Fişuş, Andreea Dana; Pop, Doina Suzana; Rusu, Monica Blanka; Vultur, Florina; Horvath, Karin Ursula

    2015-01-01

    Retinal vein occlusion (RVO) is the second most common retinal vein disease with significant visual loss via thrombus or compression of vein wall. Thrombophilia is the predisposition to vascular thrombosis with the existence of genetic defect that leads to blood hypercoagulability. This report describes the case of a 55 year old male patient, with an active life who presented himself at the emergency room with acute visual lose, insidious and progressive visual field constriction, without any known history of neurological or vascular diseases. The examinations revealed unilateral optic nerve head edema, the fluorescein angiography was specific for nonischemic central retinal vein occlusion CRVO complicated with macular edema. Blood examinations has emphasized the presence of the heterozygous mutation A1298C in the methylenetetrahydrofolate reductase gene (MTHFR), the only one presented from the thrombophilia screen panel and a slightly elevated cholesterol level. During the follow-up period, the patient received anti-VEGF treatment (Bevacizumab, 3x 0.1 ml intravitreal injections) with improved visual acuity and amendment of macular edema. The complex etiology calls for interdisciplinary approach to determine better the cause of this ophthalmological disease. Although studies have found a correlation between some thrombophilia mutations and retinal vein occlusion, more studies that contain a larger number of patients are necessary in order to determine the final role of these gene variants. PMID:26978887

  10. Radiographic Findings Associated with Vascular Anomalies

    PubMed Central

    Masand, Prakash

    2014-01-01

    Imaging of patients with vascular tumors and malformations has been sufficiently refined to answer pertinent questions when making treatment decisions in this challenging subgroup of pediatric patients. The imaging modalities at hand include conventional radiography, Doppler ultrasound, and magnetic resonance imaging with time-resolved, contrast-material enhanced magnetic resonance angiography. This review article will focus on the characteristic imaging features of some focal and diffuse vascular lesions, which have been classified by their clinical history and physical exam, and further labeled as a vascular tumor or slow-flow versus high-flow vascular malformation based on the updated classification system proposed by the International Society for the Study of Vascular Anomalies. The recent advances in knowledge regarding the biology of these vascular anomalies have led to increased awareness of the current nomenclature. Moreover, with better understanding of the imaging features, the radiologist has become a key player in the multidisciplinary approach offered at various institutions where appropriate treatment algorithms and interventional strategies are put together. This is crucial in avoiding misdiagnosis and improper management. PMID:25045332

  11. A clinical and experimental overview of sirenomelia: insight into the mechanisms of congenital limb malformations

    PubMed Central

    Garrido-Allepuz, Carlos; Haro, Endika; González-Lamuño, Domingo; Martínez-Frías, María Luisa; Bertocchini, Federica; Ros, Maria A.

    2011-01-01

    Sirenomelia, also known as sirenomelia sequence, is a severe malformation of the lower body characterized by fusion of the legs and a variable combination of visceral abnormalities. The causes of this malformation remain unknown, although the discovery that it can have a genetic basis in mice represents an important step towards the understanding of its pathogenesis. Sirenomelia occurs in mice lacking Cyp26a1, an enzyme that degrades retinoic acid (RA), and in mice that develop with reduced bone morphogenetic protein (Bmp) signaling in the caudal embryonic region. The phenotypes of these mutant mice suggest that sirenomelia in humans is associated with an excess of RA signaling and a deficit in Bmp signaling in the caudal body. Clinical studies of sirenomelia have given rise to two main pathogenic hypotheses. The first hypothesis, based on the aberrant abdominal and umbilical vascular pattern of affected individuals, postulates a primary vascular defect that leaves the caudal part of the embryo hypoperfused. The second hypothesis, based on the overall malformation of the caudal body, postulates a primary defect in the generation of the mesoderm. This review gathers experimental and clinical information on sirenomelia together with the necessary background to understand how deviations from normal development of the caudal part of the embryo might lead to this multisystemic malformation. PMID:21504909

  12. Impaired cerebral vasoreactivity after embolization of arteriovenous malformations: assessment with serial acetazolamide challenge xenon CT

    SciTech Connect

    Tarr, R.W.; Johnson, D.W.; Horton, J.A.; Yonas, H.; Pentheny, S.; Durham, S.; Jungreis, C.A.; Hecht, S.T. )

    1991-05-01

    Embolization of a portion of the nidus of an arteriovenous malformation not only may alter hemodynamics within the nidus, but also may change blood flow dynamics in adjacent normal vessels. Sequential acetazolamide-challenge xenon CT cerebral blood flow studies were performed in eight patients before and after embolization of arteriovenous malformations to assess the hemodynamic effects on the major vascular territories supplying the malformation. Acetazolamide is a potent cerebral vasodilator, and its administration combined with cerebral blood flow studies allows assessment of cerebral vasoreactivity. In seven of the eight patients, one or more parenchymal areas exhibited a normal cerebral blood flow augmentation response to acetazolamide before embolization, but diminished acetazolamide flow augmentation was seen after embolization, indicating abnormal vasoreactivity. We found that the decrease in vasoreactivity peaked 6-10 days after embolization. In one of the eight patients, a temporary delayed neurologic deficit developed during a period of impaired cerebral vasoreactivity following embolization. Our results suggest that embolization of an arteriovenous malformation can induce vasoreactivity changes in adjacent normal vessels. Because these changes appear to be somewhat time-dependent, an appropriate interval should be observed between embolization stages or before surgical resection of an arteriovenous malformation following embolization to allow hemodynamic equilibration to occur. Acetazolamide challenge combined with serial cerebral blood flow studies following embolization enables determination of this hemodynamic equilibration.

  13. Visual Fixation in Chiari Type II Malformation

    PubMed Central

    Salman, Michael S.; Sharpe, James A.; Lillakas, Linda; Dennis, Maureen; Steinbach, Martin J.

    2011-01-01

    Chiari type II malformation is a congenital deformity of the hindbrain. Square wave jerks are horizontal involuntary saccades that interrupt fixation. Cerebellar disorders may be associated with frequent square wave jerks or saccadic oscillations such as ocular flutter. The effects of Chiari type II malformation on visual fixation are unknown. We recorded eye movements using an eye tracker in 21 participants with Chiari type II malformation, aged 8 to 19 years while they fixated a target for 1 minute. Thirty-eight age-matched healthy participants served as controls. Square wave jerks’ parameters were similar in the 2 groups. Saccadic oscillations were not seen. Chiari type II malformation is not associated with pathological square wave jerks or abnormal saccadic oscillations. The congenital nature of this deformity may permit compensation that preserves stable visual fixation. Alternatively, the deformity of Chiari type II malformation may spare parts of the cerebellum that usually cause fixation instability when damaged. PMID:19182152

  14. 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.

  15. Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection

    PubMed Central

    Verma, Sudeep; Subramanian, Anand; Saileela, Rajan; Koneti, Nageswara Rao

    2015-01-01

    Background: Vertical vein is left patent in some cases of supra-cardiac total anomalous pulmonary venous connection (TAPVC) when there is hemodynamic instability due to noncompliant left atrium and ventricle. After the peri-operative period, this results in features of pre-tricuspid shunt. Materials and Methods: Three cases with patent vertical vein following repair of supra-cardiac TAPVC presented with features of pre-tricuspid shunt on follow-up. Trans-catheter closure of patent vertical vein was performed using vascular plug in all three subjects. Results: The procedure was technically successful in all the patients. There was a complication related to catheter tip breakage in one of them, which was successfully managed. There was no impingement on pulmonary vein in any of the patients. Conclusion: Patent vertical vein following TAPVC repair results in features of pre-tricuspid shunt. Transcatheter closure of the patent vein is feasible. PMID:26556968

  16. Vulvar vascular tumors: a clinicopathologic study of 85 patients.

    PubMed

    Papalas, John A; Sangueza, Omar P; Puri, Puja K; Robboy, Stanley J; Selim, Maria A

    2013-02-01

    The subepidermal hormonally sensitive tissue of the vulva is anatomically unique and may give rise to a wide variety of vascular tumors. As a consequence, classifying vulvar vascular lesions has been challenging due both to the wide variety of lesions that may be encountered and the heterogeneity in reporting across several disciplines. The purpose of this study is to present an institutional experience of vulvar vascular lesions. Overall, 85 patients were identified over a 26-year period. Vascular lesions belonging to the following classes included (n, %total) benign vascular tumors (32, 38%), dilatations of preexisting vessels (31, 36%), hyperplasia/reactive (7, 8%), tumors with significant vascular component (11, 13%), malformations (3, 4%), and malignant vascular tumors (1, 1%). Two reaction patterns based on vulvar lymphatic pathology were identified: one is a stromal dominant pattern and the other is a vascular dominant pattern. Vulvar vascular malformations and true vascular malignancies, although rare, may have associated high morbidity. To accurately classify vulvar lymphatic lesions, the pathologist must carefully consider the patient's clinical history taking into account features such as preexisting lymphedema. PMID:23348141

  17. Spontaneous Iliac Vein Rupture

    PubMed Central

    Kim, Dae Hwan; Park, Hyung Sub; Lee, Taeseung

    2015-01-01

    Spontaneous iliac vein rupture (SIVR) is a rare entity, which usually occurs without a precipitating factor, but can be a life-threatening emergency often requiring an emergency operation. This is a case report of SIVR in a 62-year-old female who presented to the emergency room with left leg swelling. Workup with contrast-enhanced computed tomography revealed a left leg deep vein thrombosis with May-Thurner syndrome and a hematoma in the pelvic cavity without definite evidence of arterial bleeding. She was managed conservatively without surgical intervention, and also underwent inferior vena cava filter insertion and subsequent anticoagulation therapy for pulmonary thromboembolism. This case shows that SIVR can be successfully managed with close monitoring and conservative management, and anticoagulation may be safely applied despite the patient presenting with venous bleeding. PMID:26217647

  18. 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.

  19. [Duplication of the superior vena cava and other malformations discovered at insertion of a port-a-cath].

    PubMed

    Hammerer, V; Jeung, M; Mennecier, B; Demian, M; Pauli, G; Quoix, E

    2005-09-01

    We report a clinical case of a persistent left superior vena cava discovered in a 50-year-old female patient when a port-a-cath was inserted. This already seldom malformation was associated with an arteria lusoria and polysplenia with left inferior vena cava with hemiazygos continuation, right-sided stomach, short pancreas, preduodenal portal vein and intestinal malrotation, but without any cardiac abnormalities. PMID:16208194

  20. Hans von Haberer: a forgotten pioneer in vascular surgery.

    PubMed

    Mönig, S P; Walter, M; Erasmi, H; Pichlmaier, H

    1997-03-01

    After carrying out the first free vein graft transplantation on an aneurysm of the axillary vein by Lexer in 1907, many attempts were made to reconstruct arterial injuries with direct vascular suture technique or vein graft transplants during the Balkan War (1912) and the First World War (1914-1918). Hans von Haberer gained wide experience in the reconstructive surgery of traumatic aneurysms at the Department of Surgery at the University of Innsbruck. During this period, he operated on a total of 201 vascular aneurysms, mainly using a direct circular vascular suture technique. In 1914, von Haberer described the first reconstruction of a carotid aneurysm. First experiences with vein bypasses were made, but not pursued in the following years. PMID:9181777

  1. Bleeding Scrotal Vascular Lesions: Interventional Management with Transcatheter Embolization

    SciTech Connect

    Jaganathan, Sriram; Gamanagatti, Shivanand Mukund, Amar; Dhar, Anita

    2011-02-15

    Vascular lesions of the scrotum are uncommon; the most common among them are varicocele lesions. The other vascular lesions that may involve the scrotum are hemangioma, lymphangioma, and arteriovenous malformations, which are exceedingly rare. The imaging modalities useful in the diagnosis and management of scrotal vascular lesions are grayscale sonography, color Doppler sonography, magnetic resonance imaging, magnetic resonance angiography, and digital subtraction angiography. We present two cases of scrotal vascular lesions involving the extratesticular scrotal soft tissues. Patients presented with bleeding and were treated by radiological interventional technique. We emphasize the importance of superselective catheterization and distal embolization.

  2. 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

  3. MicroRNA and vascular remodelling in acute vascular injury and pulmonary vascular remodelling

    PubMed Central

    McDonald, Robert A.; Hata, Akiko; MacLean, Margaret R.; Morrell, Nicholas W.; Baker, Andrew H.

    2012-01-01

    Vascular remodelling is an integral pathological process central to a number of cardiovascular diseases. The complex interplay between distinct cell populations in the vessel wall following vascular injury leads to inflammation, cellular dysfunction, pro-growth signals in the smooth muscle cell (SMC) compartment, and the acquisition of a synthetic phenotype. Although the signals for vascular remodelling are diverse in different pathological contexts, SMC proliferation and migration are consistently observed. It is therefore critical to elucidate key mechanisms central to these processes. MicroRNAs (miRNAs) are small non-coding sequences of RNA that have the capacity to regulate many genes, pathways, and complex biological networks within cells, acting either alone or in concert with one another. In diseases such as cancer and cardiac disease, the role of miRNA in disease pathogenesis has been documented in detail. In contrast, despite a great deal of interest in miRNA, relatively few studies have directly assessed the role of miRNA in vascular remodelling. The potential for modulation of miRNA to achieve therapeutic benefits in this setting is attractive. Here, we focus on the role of miRNA in vascular inflammation and remodelling associated with acute vascular injury (vein graft disease, angioplasty restenosis, and in-stent restenosis) as well as in vascular remodelling associated with the development of pulmonary arterial hypertension. PMID:22065733

  4. Azygos Vein Dialysis Catheter Placement Using the Translumbar Approach in a Patient with Inferior Vena Cava Occlusion

    SciTech Connect

    Jaber, Mohammad R.; Thomson, Matthew J.; Smith, Douglas C.

    2008-07-15

    We describe percutaneous, translumbar placement of a 14-Fr dialysis catheter into an ascending lumbar vein to achieve tip position in an enlarged azygos vein. The patient had thrombosis of all traditional vascular sites, as well as the inferior vena cava. This catheter functioned well for 7 months before fatal catheter-related infection developed.

  5. 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

  6. Perceptual enhancement of arteriovenous malformation in MRI angiography displays

    NASA Astrophysics Data System (ADS)

    Abhari, Kamyar; Baxter, John S. H.; Eagleson, Roy; Peters, Terry; de Ribaupierre, Sandrine

    2012-02-01

    The importance of presenting medical images in an intuitive and usable manner during a procedure is essential. However, most medical visualization interfaces, particularly those designed for minimally-invasive surgery, suffer from a number of issues as a consequence of disregarding the human perceptual, cognitive, and motor system's limitations. This matter is even more prominent when human visual system is overlooked during the design cycle. One example is the visualization of the neuro-vascular structures in MR angiography (MRA) images. This study investigates perceptual performance in the usability of a display to visualize blood vessels in MRA volumes using a contour enhancement technique. Our results show that when contours are enhanced, our participants, in general, can perform faster with higher level of accuracy when judging the connectivity of different vessels. One clinical outcome of such perceptual enhancement is improvement of spatial reasoning needed for planning complex neuro-vascular operations such as treating Arteriovenous Malformations (AVMs). The success of an AVM intervention greatly depends on fully understanding the anatomy of vascular structures. However, poor visualization of pre-operative MRA images makes the planning of such a treatment quite challenging.

  7. COMPUTED TOMOGRAPHIC AND ULTRASONOGRAPHIC CHARACTERISTICS OF CAVERNOUS TRANSFORMATION OF THE OBSTRUCTED PORTAL VEIN IN SMALL ANIMALS.

    PubMed

    Specchi, Swan; Pey, Pascaline; Ledda, Gianluca; Lustgarten, Meghann; Thrall, Donald; Bertolini, Giovanna

    2015-01-01

    In humans, the process of development of collateral vessels with hepatopetal flow around the portal vein in order to bypass an obstruction is called "cavernous transformation of the portal vein." The purpose of this retrospective, cross-sectional, multicentric study was to describe presumed cavernous transformation of the portal vein in small animals with portal vein obstruction using ultrasound and multidetector-row computed tomography (MDCT). Databases from three different institutions were searched for patients with an imaging diagnosis of cavernous transformation of the portal vein secondary to portal vein obstruction of any cause. Images were retrieved and reanalyzed. With MDCT-angiography, two main portoportal collateral pathways were identified: short tortuous portoportal veins around/inside the thrombus and long portoportal collaterals bypassing the site of portal obstruction. Three subtypes of the long collaterals, often coexisting, were identified. Branches of the hepatic artery where involved in collateral circulation in nine cases. Concomitant acquired portosystemic shunts were identified in six patients. With ultrasound, cavernous transformation of the portal vein was suspected in three dogs and one cat based on visualization of multiple and tortuous vascular structures corresponding to periportal collaterals. In conclusion, the current study provided descriptive MDCT and ultrasonographic characteristics of presumed cavernous transformation of the portal vein in a sample of small animals. Cavernous transformation of the portal vein could occur as a single condition or could be concurrent with acquired portosystemic shunts. PMID:25877678

  8. The Significance of Neuregulin-1/ErbB Expression in Autogenous Vein Grafts in a Diabetic Rat Model.

    PubMed

    Huang, Qiangxin; Zhang, Jueyu; Liang, Ludong; Lan, Zhicun; Huo, Tianming; Li, Shikang

    2015-09-01

    Diabetes mellitus (DM) is an important risk factor for increased vein graft failure after bypass surgery. The neuregulin-1 (NRG-1)/ErbB signaling system plays a critical role in neointimal formation after vascular injury as well as the proliferation and migration of mitogen-induced vascular smooth muscle cells; however, changes in NRG-1/ErbB signaling leading to vein grafts attrition in DM remain largely unexplored. Therefore, the aim of this study was to investigate changes in NRG-1/ErbB signaling in vein grafts in diabetic rats. To do this, a rat model of DM was established by streptozotocin injection followed by engraftment of autologous jugular veins to carotid arteries to induce intimal hyperplasia. After vein graft harvest, a pathohistological examination was performed; changes in NRG-1 and ErbB expression were also assessed. NRG-1 and ErbB expression localized to endothelial cells and vascular smooth muscle cells, which is consistent with the arterialization of vein grafts. NRG-1, ErbB2, and ErbB4 expression significantly decreased in vein grafts over time. Our findings show that NRG-1/ErbB signaling is impaired in vein grafts of diabetic rats, suggesting an important role for this pathway in the pathogenesis of intimal hyperplastic lesions in vein grafts of patients with DM. PMID:25978692

  9. The use of internal jugular vein as interposition graft for femoral vein reconstruction following traumatic venous injury: a useful approach in selected cases.

    PubMed

    Woodson, J; Rodriguez, A A; Menzoian, J O

    1990-09-01

    Complex venous injuries remain a controversial and interesting challenge to the vascular and trauma surgeon. Data from the Vietnam Vascular Registry, combined with experience from recent civilian series, seem to indicate that the best results are obtained when venous repair is undertaken. This is especially true of combined arterial and venous injury where compromised venous outflow may lead to limb loss in spite of patent arterial reconstruction. The larger size of veins, however, has required the construction of complex and time-consuming panel and spiral-vein grafts. This makes them far from ideal in the trauma treatment setting, where minimization of blood loss and operating room time are high priorities. We present a case of combined injury to both femoral artery and vein, where the femoral vein injury was repaired using autologous internal jugular vein as interposition graft while the arterial injury was repaired with autologous saphenous vein from the opposite limb. The avoidance of prosthetics, ease of harvest, size match, and little associated morbidity all make a strong case for use of the internal jugular vein where speedy reconstruction of large venous conduits is indicated. PMID:2223549

  10. Advanced Imaging of Chiari 1 Malformations.

    PubMed

    Fakhri, Akbar; Shah, Manish N; Goyal, Manu S

    2015-10-01

    Type I Chiari malformations are congenital deformities involving cerebellar tonsillar herniation downward through the foramen magnum. Structurally, greater than 5 mm of tonsillar descent in adults and more than 6 mm in children is consistent with type I Chiari malformations. However, the radiographic severity of the tonsillar descent does not always correlate well with the clinical symptomatology. Advanced imaging can help clinically correlate imaging to symptoms. Specifically, cerebrospinal fluid (CSF) flow abnormalities are seen in patients with type I Chiari malformation. Advanced MRI involving cardiac-gated and phase-contrast MRI affords a view of such CSF flow abnormalities. PMID:26408061

  11. Radiological assessment of vascular access in haemodialysis patients.

    PubMed

    Kamper, Lars; Frahnert, Michael; Grebe, Scott-Oliver; Haage, Patrick

    2014-01-01

    Clinical examination is still the most important diagnostic tool and duplex ultrasonography is the imaging method of first choice. Radiological assessment of vascular access for haemodialysis includes preoperative analysis of vessel anatomy and postoperative surveillance for access maturation as well as diagnosis in vascular access insufficiency. Compared to ultrasonography digital subtraction angiography is superior for the evaluation of the central veins and allows diagnosis and treatment in one session. Computed tomography should only be used in patients with inconclusive ultrasonography results, for example, for the assessment of the central veins and visualization of the vascular tree. Gadolinium-enhanced magnetic resonance imaging is no longer recommended in dialysis patients, because it may trigger nephrogenic systemic fibrosis. In patients with a history of previous central venous catheters additional preoperative imaging of the central veins should be performed. In this article we review the different radiological imaging methods for preoperative assessment and suspected vascular access dysfunction. PMID:24817452

  12. ACR Appropriateness Criteria Clinically Suspected Pulmonary Arteriovenous Malformation.

    PubMed

    Hanley, Michael; Ahmed, Osmanuddin; Chandra, Ankur; Gage, Kenneth L; Gerhard-Herman, Marie D; Ginsburg, Michael; Gornik, Heather L; Johnson, Pamela T; Oliva, Isabel B; Ptak, Thomas; Steigner, Michael L; Strax, Richard; Rybicki, Frank J; Dill, Karin E

    2016-07-01

    Pulmonary arteriovenous malformations are often included in the differential diagnosis of common clinical presentations, including hypoxemia, hemoptysis, brain abscesses, and paradoxical stroke, as well as affecting 30% to 50% of patients with hereditary hemorrhagic telangiectasia (HHT). Various imaging studies are used in the diagnostic and screening settings, which have been reviewed by the ACR Appropriateness Criteria Vascular Imaging Panel. Pulmonary arteriovenous malformation screening in patients with HHT is commonly performed with transthoracic echocardiographic bubble study, followed by CT for positive cases. Although transthoracic echocardiographic bubble studies and radionuclide perfusion detect right-to-left shunts, they do not provide all of the information needed for treatment planning and may remain positive after embolization. Pulmonary angiography is appropriate for preintervention planning but not as an initial test. MR angiography has a potential role in younger patients with HHT who may require lifelong surveillance, despite lower spatial resolution compared with CT. The ACR Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed every three years by a multidisciplinary expert panel. The guideline development and review include an extensive analysis of current medical literature from peer-reviewed journals and the application of a well-established consensus methodology (modified Delphi) to rate the appropriateness of imaging and treatment procedures by the panel. In those instances in which evidence is lacking or not definitive, expert opinion may be used to recommend imaging or treatment. PMID:27209598

  13. PDCD10 Gene Mutations in Multiple Cerebral Cavernous Malformations

    PubMed Central

    Cigoli, Maria Sole; Avemaria, Francesca; De Benedetti, Stefano; Gesu, Giovanni P.; Accorsi, Lucio Giordano; Parmigiani, Stefano; Corona, Maria Franca; Capra, Valeria; Mosca, Andrea; Giovannini, Simona; Notturno, Francesca; Ciccocioppo, Fausta; Volpi, Lilia; Estienne, Margherita; De Michele, Giuseppe; Antenora, Antonella; Bilo, Leda; Tavoni, Antonietta; Zamponi, Nelia; Alfei, Enrico; Baranello, Giovanni; Riva, Daria; Penco, Silvana

    2014-01-01

    Cerebral cavernous malformations (CCMs) are vascular abnormalities that may cause seizures, intracerebral haemorrhages, and focal neurological deficits. Familial form shows an autosomal dominant pattern of inheritance with incomplete penetrance and variable clinical expression. Three genes have been identified causing familial CCM: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3. Aim of this study is to report additional PDCD10/CCM3 families poorly described so far which account for 10-15% of hereditary cerebral cavernous malformations. Our group investigated 87 consecutive Italian affected individuals (i.e. positive Magnetic Resonance Imaging) with multiple/familial CCM through direct sequencing and Multiplex Ligation-Dependent Probe Amplification (MLPA) analysis. We identified mutations in over 97.7% of cases, and PDCD10/CCM3 accounts for 13.1%. PDCD10/CCM3 molecular screening revealed four already known mutations and four novel ones. The mutated patients show an earlier onset of clinical manifestations as compared to CCM1/CCM2 mutated patients. The study of further families carrying mutations in PDCD10/CCM3 may help define a possible correlation between genotype and phenotype; an accurate clinical follow up of the subjects would help define more precisely whether mutations in PDCD10/CCM3 lead to a characteristic phenotype. PMID:25354366

  14. Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke.

    PubMed

    Bertram, Kelly L; Madan, Anoop; Frayne, Judith

    2016-07-01

    Young onset stroke is uncommon, and may be due to conditions other than traditional vascular risk factors. A 42-year-old woman with an ischaemic stroke was found to have left atrial bubble study positivity on transthoracic echocardiogram (TTE) suggestive of patent foramen ovale, however she also had low peripheral oxygen saturation. Investigation revealed an isolated pulmonary arteriovenous malformation (PAVM), visible on admission chest radiograph. This can cause embolic stroke and is an alternate cause of the TTE findings. The PAVM was able to be closed via endovascular intervention, removing the shunt and therefore removing her risk of recurrent stroke events. This is a rare cause of embolic stroke in young people which can be easily missed on investigation yet is amenable to treatment. PMID:26896908

  15. Histopathological Features of Brain Arteriovenous Malformations in Japanese Patients.

    PubMed

    Hermanto, Yulius; Takagi, Yasushi; Yoshida, Kazumichi; Ishii, Akira; Kikuchi, Takayuki; Funaki, Takeshi; Mineharu, Yohei; Miyamoto, Susumu

    2016-06-15

    Clinical features of high risk brain arteriovenous malformations (BAVMs) are well characterized. However, pathological evidences about the differences that are possessed by high risk patients are still lacking. We reviewed archived routine hematoxylin-eosin specimens from a total of 54 surgical treated BAVMs. The histopathological features in nidus were semi-quantitatively analyzed. We obtained the pathological differences of BAVMs nidus between several clinical features. Among the analyzed pathological features, the significant differences were observed in degree of venous enlargement and intimal hyperplasia. Juvenile, female, diffuse nidus, high Spetzler-Martin grade, and low flow patients had a lesser degree of those parameters compared to adult, male, compact nidus, low Spetzler-Martin grade and high flow patients. High risk profiles of BAVMs patients were well-reflected in the nidus pathology. Therefore, juvenile, female, diffuse nidus, and low flow in Japanese BAVMs patients might have different vascular remodeling process that predispose to higher tendency of hemorrhage. PMID:27053330

  16. Intramedullary cavernous malformation of the spinal cord in two dogs.

    PubMed

    MacKillop, E; Olby, N J; Linder, K E; Brown, T T

    2007-07-01

    Intramedullary cavernous malformations (CVMs) of the spinal cord were diagnosed in 2 adult dogs that presented for paraparesis. An intramedullary spinal cord lesion was identified on a myelogram in the first dog, and expansion of the vertebral canal was evident on radiographs in the second. Extensive intraparenchymal hemorrhage was found on gross postmortem examination in both dogs, and a distinct lobulated intramedullary mass was evident in the second dog. Microscopically, both lesions were composed of dilated, thin-walled vascular channels with little-to-no intervening neural parenchyma. Both dogs had evidence of channel thrombosis along with perilesional hemorrhage and hemosiderin accumulation. The second dog had additional degenerative changes, including thickened fibrous channel walls with hyalinization, foci of mineralization, and occasional tongues of entrapped gliotic neuropil. CVMs appear to be an uncommon cause of both acute and chronic spinal cord disease in the dog. PMID:17606517

  17. Successful arterial substitution with modified human umbilical vein.

    PubMed

    Dardik, H; Dardik, I I

    1976-03-01

    Human umbilical veins were prepared as vascular grafts by tanning with dialdehyde starch or gluteraldehyde and by structural re-inforcement with an outer polyester fiber mesh. These grafts were implanted in baboons in the aortoiliac position for periods of 3 days to 9 months. There was no aneurysm formation and there was an excellent maintenance of patency as well as function. Histologic evaluation of the graft materials showed a dense collagen layer within the encircling polyester fiber mesh. A multicellular subintimal layer formed a discrete inner capsule. The inflammatory response with the umbilical component was insignificant. There was no evidence of rejection. This study suggests that the modified umbilical vein can serve as a satisfactory blood conduit. Experience with three short-term clinical implants supports the applicability and potential for this new vascular graft. PMID:816263

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

    NASA Astrophysics Data System (ADS)

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

    2016-06-01

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

  19. Cardiovascular malformations and other cardiovascular abnormalities in neurofibromatosis 1.

    PubMed

    Lin, A E; Birch, P H; Korf, B R; Tenconi, R; Niimura, M; Poyhonen, M; Armfield Uhas, K; Sigorini, M; Virdis, R; Romano, C; Bonioli, E; Wolkenstein, P; Pivnick, E K; Lawrence, M; Friedman, J M

    2000-11-13

    Although it is well recognized that a peripheral vasculopathy may occur in patients with neurofibromatosis 1 (NF1), it is unclear whether cardiovascular abnormalities are more common. We reviewed the frequency of cardiovascular abnormalities, in particular, cardiovascular malformations (CVMs), among 2322 patients with definite NF1 in the National Neurofibromatosis Foundation International Database from 1991-98. Cardiovascular malformations were reported in 54/2322 (2.3%) of the NF1 patients, only 4 of whom had Watson syndrome or NF1-Noonan syndrome. There was a predominance of Class II "flow" defects [Clark, 1995: Moss and Adams' Heart Disease in Infants, Children, and Adolescents Including the Fetus and Young Adult. p 60-70] (43/54, 80%) among the NF1 patients with CVMs. Pulmonic stenosis, that was present in 25 NF1 patients, and aortic coarctation, that occurred in 5, constitute much larger proportions of all CVMs than expected. Of interest was the paucity of Class I conotruncal defects (2 patients with tetralogy of Fallot), and the absence of atrioventricular canal, anomalous pulmonary venous return, complex single ventricle and laterality defects. Besides the 54 patients with CVMs, there were 27 patients with other cardiac abnormalities (16 with murmur, 5 with mitral valve prolapse, 1 with intracardiac tumor, and 5 with electrocardiogram abnormalities). No patient in this study had hypertrophic cardiomyopathy. There were 16 patients who had a peripheral vascular abnormality without an intracardiac CVM, plus an additional 4 patients among those with a CVM who also had a peripheral vascular abnormality. PMID:11078559

  20. Stereotactic proton beam therapy for intracranial arteriovenous malformations

    SciTech Connect

    Vernimmen, Frederik J.A.I. . E-mail: fv@sun.ac.za; Slabbert, Jacobus P.; Wilson, Jennifer A.; Fredericks, Shaheeda

    2005-05-01

    Purpose: To investigate hypofractionated stereotactic proton therapy of predominantly large intracranial arteriovenous malformations (AVMs) by analyzing retrospectively the results from a cohort of patients. Methods and Materials: Since 1993, a total of 85 patients with vascular lesions have been treated. Of those, 64 patients fulfilled the criteria of having an arteriovenous malformation and sufficient follow-up. The AVMs were grouped by volume: <14 cc (26 patients) and {>=}14 cc (38 patients). Treatment was delivered with a fixed horizontal 200 MeV proton beam under stereotactic conditions, using a stereophotogrammetric positioning system. The majority of patients were hypofractionated (2 or 3 fractions), and the proton doses are presented as single-fraction equivalent cobalt Gray equivalent doses (SFEcGyE). The overall mean minimum target volume dose was 17.37 SFEcGyE, ranging from 10.38-22.05 SFEcGyE. Results: Analysis by volume group showed obliteration in 67% for volumes <14 cc and 43% for volumes {>=}14 cc. Grade IV acute complications were observed in 3% of patients. Transient delayed effects were seen in 15 patients (23%), becoming permanent in 3 patients. One patient also developed a cyst 8 years after therapy. Conclusions: Stereotactic proton beam therapy applied in a hypofractionated schedule allows for the safe treatment of large AVMs, with acceptable results. It is an alternative to other treatment strategies for large AVMs. AVMs are likely not static entities, but probably undergo vascular remodeling. Factors influencing angiogenesis could play a new role in a form of adjuvant therapy to improve on the radiosurgical results.

  1. Deep vein thrombosis following prolonged kneeling: a case report.

    PubMed

    van Beeck, J Looringh; Versfeld, K; Ehrlich, R

    2014-06-01

    This report describes a fibreglass mould maker in the yacht building industry who developed a deep vein thrombosis (DVT) after 6 weeks of working in a kneeling position. We propose that his prolonged kneeling combined with constrictive knee pad straps caused vascular compression, precipitating his DVT. A hypercoagulability diathesis was suspected but not confirmed. Operator and employer education, modified work practices and strapless knee pads are suggested as possible preventive measures. PMID:24727563

  2. Female Pelvic Vein Embolization: Indications, Techniques, and Outcomes

    SciTech Connect

    Lopez, Anthony James

    2015-08-15

    Until recently, the main indication for pelvic vein embolization (PVE) in women was to treat pelvic venous congestion syndrome (PVC) but increasingly, patients with refluxing pelvic veins associated with leg varicosities are also being treated. A more unusual reason for PVE is to treat pelvic venous malformations, although such lesions may be treated with sclerotherapy alone. Embolotherapy for treating PVC has been performed for many years with several published studies included in this review, whilst an emerging indication for PVE is to treat lower limb varicosities associated with pelvic vein reflux. Neither group, however, has been subjected to an adequate randomized, controlled trial. Consequently, some of the information presented in this review should be considered anecdotal (level III evidence) at this stage, and a satisfactory ‘proof’ of clinical efficacy remains deficient until higher-level evidence is presented. Furthermore, a wide range of techniques not accepted by all are used, and some standardization will be required based on future mandatory prospective studies. Large studies have also clearly shown an unacceptably high recurrence rate of leg varicose veins following venous surgery. Furthermore, minimally or non-invasive imaging is now revealing that there is a refluxing pelvic venous source in a significant percentage of women with de novo leg varicose veins, and many more with recurrent varicosities. Considering that just over half the world’s population is female and a significant number of women not only have pelvic venous reflux, but also have associated leg varicosities, minimally invasive treatment of pelvic venous incompetence will become a common procedure.

  3. Update on the management of anorectal malformations.

    PubMed

    Bischoff, Andrea; Levitt, Marc A; Peña, Alberto

    2013-09-01

    Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a "Pandora's box" that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period. PMID:23913263

  4. Revision surgery for Chiari malformation decompression.

    PubMed

    Mazzola, Catherine A; Fried, Arno H

    2003-09-15

    Chiari malformations comprise four different hindbrain anomalies originally described by Hans Chiari, a professor of pathology at the German University in Prague. There are four basic Chiari malformations. The reasons for revision of Chiari malformation decompression may be for conservative or inadequate initial decompression or the development of postoperative complications. Another reason involves cases of both hindbrain herniation and syringomyelia in patients who have undergone adequate posterior fossa decompression without resolution of symptoms, signs, or radiological appearance of their syrinx cavity. Additionally, symptom recurrence has been reported in association with various types of dural grafts. Reoperation or revision surgery for patients with Chiari malformations is common and may not be due to technical error or inadequate decompression. The types of revision surgeries, their indications, and initial presentations will be reviewed. PMID:15347221

  5. Malformation and plastic surgery in childhood

    PubMed Central

    Siegert, Ralf; Magritz, Ralph

    2014-01-01

    Malformations of the head and neck show a huge variety of clinical symptoms with functional and esthetic consequences. Often times its rehabilitation requires multi-staged and multi-disciplinary procedures and concepts. These must consider eating, speech, mimic expression, hearing and “esthetics” or at least “normality”. A survey of the most common head and neck malformations and their treatment options are presented here. PMID:25587361

  6. The Microcephaly-Capillary Malformation Syndrome

    PubMed Central

    Mirzaa, Ghayda M.; Paciorkowski, Alex R.; Smyser, Christopher D.; Willing, Marcia C.; Lind, Anne C.; Dobyns, William B.

    2012-01-01

    We report on three children from two families with a new pattern recognition malformation syndrome consisting of severe congenital microcephaly (MIC), intractable epilepsy including infantile spasms, and generalized capillary malformations that was first reported recently in this journal [Carter et al. (2011); Am J Med Genet A 155: 301–306]. Two of our reported patients are an affected brother and sister, suggesting this is an autosomal recessive severe congenital MIC syndrome. PMID:21815250

  7. Spontaneous arteriovenous malformations in the cervical area

    PubMed Central

    Greenberg, J.

    1970-01-01

    Four patients with spontaneous arteriovenous malformations of cervical vessels have been presented. The embryology of these vessels has been discussed in order to suggest an explanation for the apparent difference in the incidence of arteriovenous malformations involving the internal carotid artery and those involving either the vertebral or the external carotid arteries. A fifth case (S.T.) is presented as a probable iatrogenic arteriovenous fistula and is to be added to the steadily growing reports of this phenomenon. Images PMID:5431722

  8. [Coronary veins and coronary sinus tributary veins in Africans].

    PubMed

    Yangni-Angate, H; Kokoua, A; Kouassi, R; Kassanyou, S; Gnagne, Y; Guessan, G N; Cowppli-Bony, P; Memel, J B

    1995-01-01

    This anatomical study carried out on 40 African adults hearts studied branches of the coronary sinus. By using of injection of the coronary arteries and corrosion of the myocardium, the study identified certain peculiarities of the small coronary vein and the posterior descending interventricular vein in Africans. PMID:8519704

  9. Vascular rings.

    PubMed

    Backer, Carl L; Mongé, Michael C; Popescu, Andrada R; Eltayeb, Osama M; Rastatter, Jeffrey C; Rigsby, Cynthia K

    2016-06-01

    The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time. PMID:27301603

  10. Endovascular Treatment of Acute Portal Vein Thrombosis After Liver Transplantation in a Child

    SciTech Connect

    Carnevale, Francisco Cesar Borges, Marcus Vinicius; Moreira, Airton Mota; Cerri, Giovanni Guido; Maksoud, Joao Gilberto

    2006-06-15

    Although operative techniques in hepatic transplantation have reduced the time and mortality on waiting lists, the rate of vascular complications associated with these techniques has increased. Stenosis or thrombosis of the portal vein is an infrequent complication, and if present, surgical treatment is considered the traditional management. This article describes a case of acute portal vein thrombosis after liver transplantation from a living donor to a child managed by percutaneous techniques.

  11. Deep vein thrombosis detection by 99m TC-MDP scanning

    SciTech Connect

    Moallem, A.; Lichsztral, R.

    1984-08-01

    A new observation is reported wherein 99m Tc-MDP is incorporated into recanalizing thrombophlebitis. In one patient with subacute disease, inflammatory vascularity is appreciated during the first one to two minutes after injection. Both patients showed evidence of late phosphate uptake in their greater saphenous veins two hours later. Intravenous isotope injections may be given in an arm vein: foot injections are not required.

  12. A new site for venous access: superficial veins of portal collateral circulation.

    PubMed

    Turc, Jean; Gergelé, Laurent; Attof, Rachid; Mottard, Nicolas; Bérend, Michel; David, Jean-Stéphane

    2012-01-01

    In case of failure of peripheral vascular access, classical alternatives are central venous or intraosseous access. We report a new site of vascular access necessitating no specific material. A 53-year-old patient with cirrhosis-induced coagulopathy, portal hypertension, and collateral abdominal portosystemic circulation required parenteral antibiotherapy. After failure of peripheral vein catheterization, he was addressed to our resuscitation room for central venous access. To avoid the risks associated with this invasive procedure, we chose an alternative approach. After skin preparation, a 20-gauge peripheral venous catheter was inserted in a dilated subcutaneous vein of abdominal wall. To our knowledge, it is the first human report of insertion of a catheter in a superficial vein of abdominal wall. It could be an alternative approach for vascular access after failure of peripheral venipuncture in patients with portal hypertension. PMID:21159464

  13. [Does saphenous vein saving surgery have a role in the therapy of primary varices?].

    PubMed

    Recek, C

    2003-11-01

    Venous grafts retrieved from long saphenous veins are the best conduits for vascular and coronary reconstructions. The demand for such grafts rise continuously with the development of vascular and coronary surgery. Surgeons were soon confronted with the problem whether the saphenous trunk in patients with varicose veins may be used for grafting or not. Saphenous vein saving surgery means intentional renouncement of removing the saphenous trunk during varicose vein surgery and saving it for a possible graft in the future. Opinions whether such procedures are substantiated differ widely and concern both the suitability of such grafts and the fact that lowering the radicality of varicose vein surgery increases the probability of varicose vein recurrence. The saphenous trunk in primary varicose veins is not diffusely degenerated, it usually shows only a few local bulges and is basically compatible for the use as a vascular or coronary artery conduit; it does not dilate aneurysmatically when transplanted into the arterial circulation. Crossectomy disconnects saphenofemoral junction, the most frequent source of reflux, abolishes even the most serious venous derangement and restores normal venous hemodynamics; stripping brings about no further immediate amelioration. Recurrence of varicose veins is more frequent when crossectomy alone is performed in comparison with crossectomy and stripping, but nor crossectomy combined with stripping is able to reliably prevent recurrence, because the distinct tendency to recur is a characteristic feature of varicose disease. Saphenous vein saving surgery can be efficiently supplemented by sclerotherapy during follow-up. The excellent hemodynamic improvement achieved immediately after crossectomy can be preserved for many years during follow-up by repeated applications of sclerotherapeutic agents. In this way necessary conditions for a rapid and mostly a definitive healing of varicose ulcers can be established. In addition, preservation

  14. Disc edge veins of Kraupa associated with optic disc drusen

    PubMed Central

    Díaz, Andrea; Almela, Miguel Angel

    2014-01-01

    Objective: Disc edge veins of Kraupa are a rare anomaly of the retinal venous system in which the main trunk of the retinal vein disappeared into the margin of the optic disc instead of its centre. Methods: A 40-year-old woman was detected to have an anomaly in her left optic disc in a routine eye examination. The eyes had an anomaly of the retinal venous system in which all branches of the retinal vein joined in a common trunk that entered the disc margin inferonasally. The central retinal artery issued from the centre of the disc separately of the venous system. B-scan ultrasonografhy revealed the presence of hyperechoic imaging at the optic nerve head in both eyes. Results: We describe the association of disc edge veins of Kraupa with optic disc drusen. Conclusion: Vascular complications of optic disc drusen hav been described. We don’t know the implication of disc edge veins in the pathogenesis of these complications.

  15. Reduction of intimal hyperplasia and enhanced reactivity of experimental vein bypass grafts with verapamil treatment.

    PubMed Central

    el-Sanadiki, M N; Cross, K S; Murray, J J; Schuman, R W; Mikat, E; McCann, R L; Hagen, P O

    1990-01-01

    in platelet or endothelium function between untreated and verapamil-treated animals, we examined the direct effect of verapamil on smooth muscle. Verapamil significantly inhibited [3H]-thymidine incorporation into DNA in vascular smooth muscle cells in culture in a dose-dependent manner. Verapamil treatment significantly reduces intimal hyperplasia in experimental vein grafts and inhibits smooth muscle cell proliferation in culture. Furthermore the enhanced reactivity to norepinephrine and histamine in the verapamil-treated vessels has no detrimental effect on the patency rate at 4 weeks. Thus by inhibiting intimal hyperplasia, calcium antagonists may improve the long-term patency of vein bypass grafts. Images Figs. 1A-C. PMID:2363608

  16. An Automated Mouse Tail Vascular Access System by Vision and Pressure Feedback

    PubMed Central

    Chang, Yen-Chi; Berry-Pusey, Brittany; Yasin, Rashid; Vu, Nam; Maraglia, Brandon; Chatziioannou, Arion X.; Tsao, Tsu-Chin

    2015-01-01

    This paper develops an automated vascular access system (A-VAS) with novel vision-based vein and needle detection methods and real-time pressure feedback for murine drug delivery. Mouse tail vein injection is a routine but critical step for preclinical imaging applications. Due to the small vein diameter and external disturbances such as tail hair, pigmentation, and scales, identifying vein location is difficult and manual injections usually result in poor repeatability. To improve the injection accuracy, consistency, safety, and processing time, A-VAS was developed to overcome difficulties in vein detection noise rejection, robustness in needle tracking, and visual servoing integration with the mechatronics system. PMID:26478693

  17. Infrared imaging of varicose veins

    NASA Astrophysics Data System (ADS)

    Noordmans, Herke Jan; de Zeeuw, Raymond; Verdaasdonk, Ruud M.; Wittens, Cees H. A.

    2004-06-01

    It has been established that varicose veins are better visualized with infrared photography. As near-infrared films are nowadays hard to get and to develop in the digital world, we investigated the use of digital photography of varicose veins. Topics that are discussed are illumination setup, photography and digital image enhancement and analysis.

  18. Vascular Diseases

    MedlinePlus

    ... heart and blood vessels, such as diabetes or high cholesterol Smoking Obesity Losing weight, eating healthy foods, being active and not smoking can help vascular disease. Other treatments include medicines and surgery.

  19. Congenital Agenesis of the Internal Jugular Vein: An Extremely Rare Anomaly

    PubMed Central

    Kayiran, Oguz; Calli, Caglar; Emre, Abdulkadir; Soy, Fatih Kemal

    2015-01-01

    Vascular anomalies of major venous vessels are rarely seen. Moreover, congenital absence of internal jugular vein is extremely uncommon. In our case, a female patient presented with primary unknown left cervical mass. Cervical ultrasonography demonstrated absence of right internal jugular vein. In addition, computed tomography and dynamic magnetic resonance imaging scans confirmed this diagnosis. Compensatory left internal jugular vein enlargement mimicked sort of cervical mass. Venous magnetic resonance imaging images revealed the absence of right internal jugular vein with compensatory left internal jugular vein dominance. In the literature, the agenesis of IJV was mentioned in a case with concomitant multiple problems. Here, an asymptomatic case is reported with an incident diagnosis. No interventions were planned upon the patient's request. It should be kept in mind that any kind of anomalies can be seen during venous access and neck surgery. PMID:25821625

  20. Quantification of Hepatic Vascular and Parenchymal Regeneration in Mice

    PubMed Central

    Xie, Chichi; Schwen, Lars Ole; Wei, Weiwei; Schenk, Andrea; Zafarnia, Sara; Gremse, Felix; Dahmen, Uta

    2016-01-01

    Background Liver regeneration consists of cellular proliferation leading to parenchymal and vascular growth. This study complements previous studies on cellular proliferation and weight recovery by (1) quantitatively describing parenchymal and vascular regeneration, and (2) determining their relationship. Both together are needed to (3) characterize the underlying growth pattern. Methods Specimens were created by injecting a polymerizing contrast agent in either portal or hepatic vein in normal or regenerating livers after 70% partial hepatectomy. 3D image data were obtained through micro-CT scanning. Parenchymal growth was assessed by determining weight and volume of the regenerating liver. Vascular growth was described by manually determined circumscribed parameters (maximal vessel length and radius of right inferior portal/hepatic vein), automatically determined cumulative parameters (total edge length and total vascular volume), and parameters describing vascular density (total edge length/volume, vascular volume fraction). The growth pattern was explored by comparing the relative increase of these parameters to the increase expected in case of isotropic expansion. Results Liver volume recovery paralleled weight recovery and reached 90% of the original liver volume within 7 days. Comparing radius-related vascular parameters immediately after surgical resection and after virtual resection in-silico revealed a slight increase, possibly reflecting the effect of resection-induced portal hyperperfusion. Comparing length-related parameters between post-operative day 7 and after virtual resection showed similar vascular growth in both vascular systems investigated. In contrast, radius-related parameters increased slightly more in the portal vein. Despite the seemingly homogeneous 3D growth, the observed vascular parameters were not compatible with the hypothesis of isotropic expansion of liver parenchyma and vascular structures. Conclusion We present an approach for

  1. The Management of Varicose Veins

    PubMed Central

    Lin, Fan; Zhang, Shiyi; Sun, Yan; Ren, Shiyan; Liu, Peng

    2015-01-01

    This study aimed to review the current management modalities for varicose veins. There are a variety of management modalities for varicose veins. The outcomes of the treatment of varicose veins are different. The papers on the management of varicose veins were reviewed and the postoperative complications and efficacy were compared. Foam sclerotherapy and radiofrequency ablation were associated with less pain and faster recovery than endovenous laser ablation and surgical stripping. Patients undergoing endovenous laser ablation and radiofrequency ablation are most likely to have a faster recovery time and earlier return to work in comparison with those undergoing conventional high ligation and stripping. A randomized controlled study in multiple centers is warranted to verify which approach is better than others for the treatment of varicose veins. PMID:25594661

  2. Extraglandular and intraglandular vascularization of canine prostate.

    PubMed

    Stefanov, Miroslav

    2004-03-01

    The literature on the vascularization of the canine prostate is reviewed and the clinical significance of prostate morphology is described. Scanning Electron Microscopy (SEM), combined with improved corrosion casting methods, reveal new morphological details that promise better diagnostics and treatment but also require expansion of clinical nomenclature. A proposal is made for including two previously unnamed veins in Nomina Anatomica Veterinaria (NAV). The canine prostate has two lobes with independent vascularization. Each lobe is supplied through the left and right a. prostatica, respectively. The a. prostatica sprouts three small vessels (cranial, middle, and caudal) towards the prostate gland. A. prostatica is a small-size artery whose wall structure is similar to the arteries of the muscular type. V. prostatica is a small-size valved vein. The canine prostate has capsular, parenchymal, and urethral vascular zones. The surface vessels of the capsule are predominantly veins and the diameter of arterial vessels is larger than that of the veins. The trabecular vessels are of two types: direct and branched. The prostate parenchyma is supplied by branches of the trabecular vessels. The periacinary capillaries are fenestrated and form a net in a circular pattern. The processes of the myoepithelial cells embrace both the acins and the periacinar capillaries. In the prostate ductal system. there are spermatozoa. The prostatic part of the urethra is supplied by an independent branch of a. prostatica. The prostatic urethral part is drained by v. prostatica, the vein of the urethral bulb and the ventral prostate veins. M. urethralis begins as early as the urethral prostatic part. The greater part of the white muscle fibers in m. urethralis suggest an enhanced anaerobic metabolism. PMID:14988915

  3. Tetrahydrobiopterin Role in human umbilical vein endothelial dysfunction in maternal supraphysiological hypercholesterolemia.

    PubMed

    Leiva, Andrea; Fuenzalida, Bárbara; Salsoso, Rocío; Barros, Eric; Toledo, Fernando; Gutiérrez, Jaime; Pardo, Fabián; Sobrevia, Luis

    2016-04-01

    Maternal physiological hypercholesterolemia (MPH) allows a proper foetal development; however, maternal supraphysiological hypercholesterolemia (MSPH) associates with foetal endothelial dysfunction and early development of atherosclerosis. MSPH courses with reduced endothelium-dependent dilation of the human umbilical vein due to reduced endothelial nitric oxide synthase activity compared with MPH. Whether MSPH modifies the availability of the nitric oxide synthase cofactor tetrahydrobiopterin is unknown. We investigated whether MSPH-associated lower umbilical vein vascular reactivity results from reduced bioavailability of tetrahydrobiopterin. Total cholesterol <7.2mmol/L was considered as maternal physiological hypercholesterolemia (n=72 women) and ≥7.2mmol/L as MSPH (n=35 women). Umbilical veins rings were used for vascular reactivity assays (wire myography), and primary cultures of human umbilical vein endothelial cells (HUVECs) to measure nitric oxide synthase, GTP cyclohydrolase 1, and dihydrofolate reductase expression and activity, as well as tetrahydrobiopterin content. MSPH reduced the umbilical vein rings relaxation caused by calcitonine gene-related peptide, a phenomenon partially improved by incubation with sepiapterin. HUVECs from MSPH showed lower nitric oxide synthase activity (l-citrulline synthesis from l-arginine) without changes in its protein abundance, as well as reduced tetrahydrobiopterin level compared with MPH, a phenomenon reversed by incubation with sepiapterin. Expression and activity of GTP cyclohydrolase 1 was lower in MSPH, without changes in dihydrofolate reductase expression. MSPH is a pathophysiological condition reducing human umbilical vein reactivity due to lower bioavailability of tetrahydrobiopterin leading to lower NOS activity in the human umbilical vein endothelium. PMID:26826019

  4. High-resolution MR venography of cerebral arteriovenous malformations.

    PubMed

    Essig, M; Reichenbach, J R; Schad, L R; Schoenberg, S O; Debus, J; Kaiser, W A

    1999-12-01

    The purpose of this study was to evaluate the diagnostic potential of a high-resolution magnetic resonance (MR) venography technique in patients with cerebral arteriovenous malformations (AVMs). A high-resolution 3D gradient echo sequence was used with a long echo time TE to obtain venous information down to sub-pixel sized vessel diameters of several hundred microns. The method is based on the paramagnetic property of deoxyhemoglobin, and the resulting developing phase difference between veins and brain parenchyma at long echo times which leads to signal cancellation. The reconstructed venograms were compared with time-of-flight (TOF)-MR angiography using qualitative and quantitative criteria with the conventional digital subtraction angiography serving as the reference gold standard. In 17 patients with angiographically proven cerebral AVMs, the method indicates its potential in clinical applications. Venography was able to detect all AVMs whereas TOF-MRA failed in three patients. In the delineation of venous drainage patterns MR venography was superior to TOF-MRA, however, the method failed in the detection of about half of the main feeding arteries, as expected. Due to susceptibility artifacts at air/tissue boundaries and interference with paramagnetic hemosiderin, venography was limited with respect to the delineation of the exact nidus sizes and shapes in ten patients with AVMs located close to the skull base or having suffered from previous bleeding. Although the visualization of draining veins represents an important prerequisite in the surgical and radiosurgical treatment planning of cerebral AVMs, application of high resolution MR venography may be limited in the diagnostic work-up in some of these patients. On the other hand, it may be of special importance in the early detection and assessment of small AVMs that are difficult to diagnose with other MR methods. PMID:10609990

  5. Changes in blood flow in conduit artery and veins of the upper arm during leg exercise in humans.

    PubMed

    Ooue, Anna; Ichinose, Tomoko K; Inoue, Yoshimitsu; Nishiyasu, Takeshi; Koga, Shunsaku; Kondo, Narihiko

    2008-06-01

    This study investigated changes in blood flow in the conduit artery, superficial vein, and deep vein of the upper arm during increase in internal temperature due to leg cycling. Additionally, we sought to demonstrate the contributions of blood velocity and vessel diameter on blood flow responses. Fourteen subjects performed supine cycling exercise at 60-69% maximal oxygen uptake for 30 min at an ambient temperature of 28 degrees C and relative humidity of 50%. Blood velocity and diameter in the brachial artery, basilic vein (superficial vein), and brachial vein (deep vein) were measured using ultrasound Doppler, and blood flow was calculated. Blood flow in the artery and superficial vein increased linearly with rising oesophageal temperature (DeltaT (oes)) after DeltaT (oes) was about 0.3 degrees C (within threshold), as well as cutaneous vascular conductance on the forearm. Changes in blood velocity in these vessels were similar to those in blood flow. Conversely, the brachial artery and superficial vein diameter did not affect the blood flow response. Blood flow variables in the deep vein did not change remarkably with rising DeltaT (oes). These results suggest that blood flow response, by an increase in velocity, in the conduit artery with rising DeltaT (oes) during exercise is similar to that in the superficial vein, but not deep vein. Also, it is indicated that these increases in blood flow relate to the increase in skin blood flow on the forearm with the rise in body temperature during exercise. PMID:18369659

  6. Sagittal vein thrombosis caused by central vein catheter.

    PubMed

    Sabzi, Feridoun; Karim, Hosein; Heydar Pour, Behzad; Faraji, Reza

    2015-01-01

    Cerebral venous thrombosis, including thrombosis of cerebral veins and major dural sinuses, is an uncommon disorder in the general population. However, it has a higher frequency among patients younger than 40 years of age, patients with thrombophilia, pregnant patients or those receiving hormonal contraceptive therapy or has foreign body such as catheter in their veins or arterial system. In this case report, we described clinical and radiological findings in a patient with protein C-S deficiency and malposition of central vein catheter. PMID:25796028

  7. Ultrasound examination of the liver: Normal vascular anatomy

    PubMed Central

    Draghi, F.; Rapaccini, G.L.; Fachinetti, C.; de Matthaeis, N.; Battaglia, S.; Abbattista, T.; Busilacchi, P.

    2007-01-01

    Various treatments for liver diseases, including liver transplant (particularly partial liver resection from a living donor), treatment of liver tumors, and TIPS, require detailed knowledge of the complex vascular anatomy of the liver. The hepatic artery and portal vein provide the organ with a double blood supply whereas venous drainage is furnished by the hepatic veins. Multislice computed tomography and magnetic resonance imaging provide undeniably excellent information on these structures. On ultrasound, the inferior vena cava, the openings of the hepatic veins, and the main branch of the portal vein can always be visualized, but intrasegmental vessels (portal, arterial, accessory hepatic venous branches) can be only partially depicted and in some cases not at all. In spite of its difficulty and limitations, hepatic sonography is frequently unavoidable, particularly in critically ill patients, and the results are essential for defining diagnostic and therapeutic strategies. For this reason, a thorough knowledge of the sonographic features of hepatic vascular anatomy is indispensable. PMID:23396216

  8. Spectrum of urorectal septum malformation sequence.

    PubMed

    Shah, Krupa; Nayak, Shalini S; Shukla, Anju; Girisha, Katta M

    2016-05-01

    Urorectal septum malformation sequence (URSMS) is a rare spectrum of malformations involving various organ systems. Here, we present eight cases of URSMS, noted in autopsy, with different degrees of complexity, seven being the complete type and one being the partial type. All cases had gastrointestinal tract malformation in the form of the imperforate anus and indeterminate genitalia. Other gastrointestinal tract anomalies were anal agenesis in two cases, anorectal agenesis in two cases, and malformed lower intestinal tract in four cases. The associated renal abnormality was noted in five cases, which were unilateral renal agenesis, dysplastic kidney, hydronephrosis, horseshoe kidney, and unilateral hypoplastic ectopic kidney. External genital malformation, present in both male and female fetuses, included a knob-like structure at perineum in female fetuses, genital fold hypoplasia and penile aplasia or hypoplasia in male fetuses. Skeletal abnormalities included two cases of sacral agenesis and one case of lumbosacral dysraphism. Other anomalies included a case with alobar holoprosencephaly, truncus arteriosus with hypoplastic lungs in one case, and three cases with abdominal wall defects. It is our attempt to delineate a spectrum of abnormalities associated with URSMS. PMID:26663027

  9. Vascular Diseases of the Spinal Cord: Infarction, Hemorrhage, and Venous Congestive Myelopathy.

    PubMed

    Vuong, Shawn M; Jeong, William J; Morales, Humberto; Abruzzo, Todd A

    2016-10-01

    Vascular pathologies of the spinal cord are rare and often overlooked. This article presents clinical and imaging approaches to the diagnosis and management of spinal vascular conditions most commonly encountered in clinical practice. Ischemia, infarction, hemorrhage, aneurysms, and vascular malformations of the spine and spinal cord are discussed. Pathophysiologic mechanisms, clinical classification schemes, clinical presentations, imaging findings, and treatment modalities are considered. Recent advances in genetic and syndromic vascular pathologies of the spinal cord are also discussed. Clinically relevant spinal vascular anatomy is reviewed in detail. PMID:27616317

  10. Control of leaf and vein development by auxin.

    PubMed

    Scarpella, Enrico; Barkoulas, Michalis; Tsiantis, Miltos

    2010-01-01

    Leaves are the main photosynthetic organs of vascular plants and show considerable diversity in their geometries, ranging from simple spoon-like forms to complex shapes with individual leaflets, as in compound leaves. Leaf vascular tissues, which act as conduits of both nutrients and signaling information, are organized in networks of different architectures that usually mirror the surrounding leaf shape. Understanding the processes that endow leaves and vein networks with ordered and closely aligned shapes has captured the attention of biologists and mathematicians since antiquity. Recent work has suggested that the growth regulator auxin has a key role in both initiation and elaboration of final morphology of both leaves and vascular networks. A key feature of auxin action is the existence of feedback loops through which auxin regulates its own transport. These feedbacks may facilitate the iterative generation of basic modules that underlies morphogenesis of both leaves and vasculature. PMID:20182604

  11. Vein matching using artificial neural network in vein authentication systems

    NASA Astrophysics Data System (ADS)

    Noori Hoshyar, Azadeh; Sulaiman, Riza

    2011-10-01

    Personal identification technology as security systems is developing rapidly. Traditional authentication modes like key; password; card are not safe enough because they could be stolen or easily forgotten. Biometric as developed technology has been applied to a wide range of systems. According to different researchers, vein biometric is a good candidate among other biometric traits such as fingerprint, hand geometry, voice, DNA and etc for authentication systems. Vein authentication systems can be designed by different methodologies. All the methodologies consist of matching stage which is too important for final verification of the system. Neural Network is an effective methodology for matching and recognizing individuals in authentication systems. Therefore, this paper explains and implements the Neural Network methodology for finger vein authentication system. Neural Network is trained in Matlab to match the vein features of authentication system. The Network simulation shows the quality of matching as 95% which is a good performance for authentication system matching.

  12. [Dynamic MRA in the evaluation of intracranial vascular diseases].

    PubMed

    Gauvrit, J Y; Oppenheim, C; Savage, J; Nataf, F; Reyns, N; Pruvo, J P; Meder, J F; Leclerc, X

    2005-01-01

    Conventional catheter angiography (CCA) remains the gold standard for the evaluation of most intracranial vascular malformations. MRA techniques such as Time of Flight, Phase Contrast or 3D contrast-enhanced MRA, provide anatomic evaluation but without hemodynamic information. Recently developed, dynamic MRA is based on dynamic acquisition of images and image subtraction; these two principal characteristics produce images comparable to those obtained by CCA. The purpose of this review is to explain the principles, advantages and drawbacks of this technique in the evaluation of arteriovenous malformations, arteriovenous fistulas, aneurysms and venous thrombosis. PMID:15798609

  13. 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. PMID:21990534

  14. Treatment of left ventricular assist device-associated arteriovenous malformations with thalidomide.

    PubMed

    Ray, Ranjan; Kale, Parag P; Ha, Richard; Banerjee, Dipanjan

    2014-01-01

    Gastrointestinal bleeding because of arteriovenous malformations (AVMs) is an increasingly recognized complication of continuous flow left ventricular assist devices (LVADs). Currently, therapeutic options for LVAD-associated AVMs are limited and often require repeated endoscopic procedures and reduction or cessation of anticoagulation. Thalidomide has been utilized in the treatment of refractory bleeding because of gastrointestinal vascular malformations. Here we describe the case of a 66-year-old man with severe ischemic cardiomyopathy implanted with a continuous flow HeartMate II. His postoperative course was complicated by multiple hospital admissions for gastrointestinal bleeding because of LVAD-associated AVMs refractory to repeated argon plasma laser coagulation. Anticoagulation was discontinued with subsequent pump stoppage because of thrombus requiring urgent surgical pump exchange. Following this, thalidomide was initiated and anticoagulation with warfarin was continued. Since initiation of thalidomide, the patient has not had further gastrointestinal bleeding or evidence of pump thrombus in the subsequent 1 year. PMID:24830804

  15. Meningitis after cochlear implantation in Mondini malformation.

    PubMed

    Page, E L; Eby, T L

    1997-01-01

    Although the potential for CSF leakage and subsequent meningitis after cochlear implantation in the malformed cochlea has been recognized, this complication has not been previously reported. We report a case of CSF otorhinorrhea and meningitis after minor head trauma developing 2 years after cochlear implantation in a child with Mondini malformation. Leakage of CSF was identified from the cochleostomy around the electrode of the implant, and this leak was sealed with a temporalis fascia and muscle plug. Although this complication appears to be rare, care must be taken to seal the cochleostomy in children with inner ear malformations at the initial surgery, and any episode of meningitis after surgery must be thoroughly investigated to rule out CSF leakage from the labyrinth. PMID:9018266

  16. Sex hormone exposure during pregnancy and malformations.

    PubMed

    Briggs, M H; Briggs, M

    1979-01-01

    This general review of the effects of exposure to sex hormones during pregnancy and subsequent fetal malformation presents summaries of animal studies, develops the data indicating virilization and feminization in humans, documents chromosome abnormalities, and presents data on the connection of steroid exposure in utero and somatic malformations. Fetal exposure can occur 3 different ways, through hormonal pregnancy test, via obstetrical use of hormones, or because of continued maternal use of oral contraceptives after conception. In the latter case, an ongoing prospective study indicates that accidental ingestion of oral contraceptives after conception is not harmful to the fetus if taken during early pregnancy. Tables present summaries of numerous large surveys and retrospective studies linking particular sex hormones (exogenous) to particular fetal malformations including neural tube defects and other constellations of developmental problems. The question of exogenous hormone effects on the personality of infants who were exposed in utero is addressed. PMID:400321

  17. Anorectal Malformation: Paediatric Problem Presenting in Adult.

    PubMed

    Chavan, Rahulkumar N; Chikkala, Bhargav; Das, Cinjini; Biswas, Somak; Sarkar, Diptendra Kumar; Pandey, Sushil Kumar

    2015-01-01

    This is a case report of 22-year-old girl admitted with abdominal distension, vomiting, and chronic constipation since birth. Abdomen was distended, and perineal examination revealed imperforate anus with vestibular fistula (ARM). So far worldwide very few cases have been reported about anorectal malformation presenting in adulthood, and thus extremely little data is available in the literature about an ideal management of anorectal malformation in adults. In our case in the treatment instead of conventional procedure of posterior sagittal anorectoplasty (PSARP) anal transposition was done and till two years after the definitive treatment during follow-up patient has been doing well with Kelly's score of six. Our experience suggests that anal transposition provides satisfactory outcome in adults presenting late with anorectal malformation. PMID:26539301

  18. Anorectal Malformation: Paediatric Problem Presenting in Adult

    PubMed Central

    Chavan, Rahulkumar N.; Chikkala, Bhargav; Das, Cinjini; Biswas, Somak; Sarkar, Diptendra Kumar; Pandey, Sushil Kumar

    2015-01-01

    This is a case report of 22-year-old girl admitted with abdominal distension, vomiting, and chronic constipation since birth. Abdomen was distended, and perineal examination revealed imperforate anus with vestibular fistula (ARM). So far worldwide very few cases have been reported about anorectal malformation presenting in adulthood, and thus extremely little data is available in the literature about an ideal management of anorectal malformation in adults. In our case in the treatment instead of conventional procedure of posterior sagittal anorectoplasty (PSARP) anal transposition was done and till two years after the definitive treatment during follow-up patient has been doing well with Kelly's score of six. Our experience suggests that anal transposition provides satisfactory outcome in adults presenting late with anorectal malformation. PMID:26539301

  19. Effect of Intravenous (IV) Assistive Device (VeinViewer) on IV Access Attempts, Procedural Time, and Patient and Nurse Satisfaction.

    PubMed

    Ramer, Lois; Hunt, Pauline; Ortega, Erin; Knowlton, Jessica; Briggs, Raymond; Hirokawa, Shinichi

    2016-07-01

    This study evaluated the effectiveness of VeinViewer for peripheral vascular accessing a pediatric hematology oncology clinic. After obtaining consent, 53 patients were randomly assigned to either the VeinViewer group (n = 27) or standard methods group (n = 26). Data on number of attempts, procedural time, access complications, and patient and nurse satisfaction were collected. Patients randomized to the VeinViewer group required significantly less time to access a vein as compared with the standard methods group (P ≤ .05). Additionally, these patients rated nurses as having significantly more skill than nurses who did not use VeinViewer (P ≤ .05) and assigned significantly higher scores for "overall experience"(P ≤ .05). Responses by nurses using VeinViewer overall saw the device in a positive light. PMID:26510643

  20. Congenital Malformations Leading to Paradoxical Embolism.

    PubMed

    Bruckheimer, Elchanan

    2016-05-01

    The absolute separation of the right and left circulations and the filtration of blood by the pulmonary circulation are essential to prevent the passage of thrombotic material from the venous system into the systemic arterial circulation. Any breach of the intracardiac septae or circumvention of the pulmonary capillary network may cause a paradoxical embolus. The most common causes are atrial septal defects and pulmonary arteriovenous malformations. This article discusses unusual connections and pathways related to congenital malformations. Although anticoagulation is necessary to prevent paradoxical emboli, the hematologic disturbances and the most appropriate therapy in these patients warrant further investigation. PMID:27150173

  1. Mayer-Rokitansky syndrome and anorectal malformation.

    PubMed

    Patankar, Shreeprasad P; Kalrao, Vijay; Patankar, Shilpa S

    2004-12-01

    Mayer Rokitansky Kuster Houser syndrome (MRKH syndrome) is characterized by Mullerian duct structures agenesis, vaginal atresia being the commonest variant. It can be associated with renal, skeletal, spine and other malformations. Patient with Mayer Rokitansky syndrome has a varied presentation from newborn period to adolescence. Thorough investigations are required for classification of the syndrome and diagnosis of associated anomalies. The MRKH syndrome patient may require complex vaginal reconstructive surgery and a detailed counseling about the potentials of menstruation and fertility. Here we are presenting a patient having association of anorectal malformation, Mullerian duct agenesis and renal anomaly. PMID:15630325

  2. Neurodevelopmental malformations of the cerebellar vermis in genetically engineered rats

    EPA Science Inventory

    The cerebellar vermis is particularly vulnerable to neurodevelopmental malformations in humans and rodents. Sprague-Dawley, and Long-Evans rats exhibit spontaneous cerebellar malformations consisting of heterotopic neurons and glia in the molecular layer of the vermis. Malformati...

  3. In Vivo and In Vitro Assessment of Human Saphenous Vein Wall Changes

    PubMed Central

    Asbeutah, Akram M; Asfar, Sami K; Safar, Hussain; Oriowo, Mabayoje A; ElHagrassi, Ihab; Abu-Assi, Mona A; Cameron, James D; McGrath, Barry P

    2007-01-01

    Purpose: To investigate if noradrenaline (NA) and 5-hydroxyptamine (5-HT) drugs induce responses of isolated control and varicose veins are altered by removal of the endothelium. Subjects & Methods: Specimens of the great saphenous vein (GSV) were obtained from 12 subjects with primary varicose veins and 12 subjects from donor vessels at cardiac surgery. A total of 10 normal healthy volunteers were selected for comparison. The diameter changes of GSV during the resting phase, at the end of 5 minutes occlusion, and then every 30 seconds post deflation for five minutes were measured using B-mode ultrasound. Post-surgery the vein sample was collected in a tube of Krebs-Henseleit solution. Results: The repeated measure ANOVA test for the diameter, percent, and difference changes of GSV diameter from maximum diameter at different time intervals showed significance difference within and between all groups. NA and 5-HT produced concentration-dependent contractions of control and varicose saphenous vein segments. There was no significant difference in the potency of NA and for 5-HT, but the maximum response, normalized for tissue weight, was less in varicose vein segments. Removal of the endothelium had no effect on the potency of NA or 5-HT but significantly (p<0.05) reduced the maximum response to NA and 5-HT in varicose vein segments but not to 5-HT in control veins. Conclusion: The venous endothelial damage may cause vascular smooth muscle contractions dysfunction that favours dilatation and secondary valvular insufficiency. PMID:18949086

  4. [Retinal vascular diseases reflecting generalized vascular alterations. What can be mutually learnt?].

    PubMed

    Feltgen, N; Franko Zeitz, P

    2014-01-01

    Retinal vascular diseases are mostly caused by systemic vascular diseases. In some cases the systemic disease is already known but in other patients ocular anomalies often provide the first indications of a systemic disease. Treating patients with vascular fundus diseases requires close cooperation between ophthalmologists and specialists in other fields and deciding which routine and specialized diagnostic examinations are necessary in light of the potential risk factors involved requires interdisciplinary communication. This article aims to provide an overview of the most important vascular retinal diseases and which examinations are required to ensure an accurate diagnosis. The retinal vascular diseases with the highest frequency or clinical relevance are hypertensive retinopathy, diabetic retinopathy, retinal vein occlusion and retinal artery occlusion. PMID:24448809

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

    PubMed

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

    2016-06-01

    Cerebral arteriovenous malformations (AVM) are traditionally considered primary congenital lesions that result from embryological aberrations in vasculogenesis. Recent insights, however, suggest that these lesions may be secondary to a vascular insult such as ischemia or trauma. Herein, the authors present a rare case of a secondary cerebral AVM, occurring in a young girl who received prior cranial radiation therapy. At age 3years, she underwent surgical resection, chemotherapy, and photon radiation therapy for treatment of a fourth ventricular ependymoma. At age 19years, she developed new onset seizures and was found to have a left medial temporal lobe AVM. Her seizures were managed successfully with anti-epileptic medications and the AVM was treated with proton radiation therapy. This case highlights a rare but possible vascular sequela of radiation therapy and adds to the growing body of evidence that cerebral AVM may arise as secondary lesions. PMID:26860850

  6. Hepatoportography via the Umbilical Vein

    PubMed Central

    White, J. J.; Skinner, G. B.; MacLean, L. D.

    1966-01-01

    The umbilical vein in adults is patent but collapsed. There is a membranous valve at its entrance into the left portal vein. Cannulation of the portal vein via the umbilical vein permits direct access to the portal system for portography and hepatography. This procedure was performed under local or general anesthesia in 30 patients and was successful in 22. It is useful in the investigation of patients with portal hypertension, and suspected intrahepatic tumours or abscesses. It gives excellent contrast visualization of the liver and definition of lesions as small as 1.0 cm. This technique is superior to both hepatic scanning and splenoportography. ImagesFig. 2Fig. 3Fig. 4Fig. 5Fig. 6Fig. 7Fig. 8Fig. 9Fig. 10Fig. 11 PMID:5924949

  7. How Are Varicose Veins Treated?

    MedlinePlus

    ... Intramural Research Research Resources Research Meeting Summaries Technology Transfer Clinical Trials What Are Clinical Trials? Children & Clinical ... shun) therapy uses lasers or radiowaves to create heat to close off a varicose vein. Your doctor ...

  8. Automatic classification of retinal vessels into arteries and veins

    NASA Astrophysics Data System (ADS)

    Niemeijer, Meindert; van Ginneken, Bram; Abràmoff, Michael D.

    2009-02-01

    Separating the retinal vascular tree into arteries and veins is important for quantifying vessel changes that preferentially affect either the veins or the arteries. For example the ratio of arterial to venous diameter, the retinal a/v ratio, is well established to be predictive of stroke and other cardiovascular events in adults, as well as the staging of retinopathy of prematurity in premature infants. This work presents a supervised, automatic method that can determine whether a vessel is an artery or a vein based on intensity and derivative information. After thinning of the vessel segmentation, vessel crossing and bifurcation points are removed leaving a set of vessel segments containing centerline pixels. A set of features is extracted from each centerline pixel and using these each is assigned a soft label indicating the likelihood that it is part of a vein. As all centerline pixels in a connected segment should be the same type we average the soft labels and assign this average label to each centerline pixel in the segment. We train and test the algorithm using the data (40 color fundus photographs) from the DRIVE database1 with an enhanced reference standard. In the enhanced reference standard a fellowship trained retinal specialist (MDA) labeled all vessels for which it was possible to visually determine whether it was a vein or an artery. After applying the proposed method to the 20 images of the DRIVE test set we obtained an area under the receiver operator characteristic (ROC) curve of 0.88 for correctly assigning centerline pixels to either the vein or artery classes.

  9. A serotonergic system in veins: serotonin transporter-independent uptake.

    PubMed

    Linder, A Elizabeth; Ni, Wei; Szasz, Theodora; Burnett, Robert; Diaz, Jessica; Geddes, Timothy J; Kuhn, Donald M; Watts, Stephanie W

    2008-06-01

    We hypothesized that the 5-hydroxytryptamine (5-HT; serotonin) system is present and functional in veins. In vena cava (VC), the presence of the 5-HT synthesis rate-limiting enzyme tryptophan hydroxylase-1 mRNA and accumulation of the 5-HT synthesis intermediate 5-hydroxytryptophan after incubation with tryptophan supported the ability of veins to synthesize 5-HT. The presence of 5-HT and its metabolite 5-hydroxyindole acetic acid was measured by high-performance liquid chromatography in VC and jugular vein (JV), and it was compared with similarly sized arteries aorta (RA) and carotid (CA), respectively. In rats treated with the monoamine oxidase-A (MAO-A) inhibitor pargyline to prevent 5-HT metabolism, basal 5-HT levels were higher in veins than in arteries. 5-HT uptake was observed after exposure to exogenous 5-HT in all vessels. The presence of MAO-A and the 5-HT transporter (SERT) in VC was observed by immunohistochemistry and Western analysis. However, 5-HT uptake was not inhibited by the SERT inhibitors fluoxetine and/or fluvoxamine in VC and JV, as opposed to the inhibition in RA and CA. Moreover, studies performed in VC from mutant rats lacking SERT showed no differences in 5-HT uptake compared with VC from wild type. These data suggest the SERT is not functional under physiological conditions in veins. The differences in 5-HT handling between veins and arteries may represent alternative avenues for targeting the 5-HT system in the peripheral circulation for controlling vascular tone. PMID:18322152

  10. Analysis of the hand vein pattern for people recognition

    NASA Astrophysics Data System (ADS)

    Castro-Ortega, R.; Toxqui-Quitl, C.; Cristóbal, G.; Marcos, J. Victor; Padilla-Vivanco, A.; Hurtado Pérez, R.

    2015-09-01

    The shape of the hand vascular pattern contains useful and unique features that can be used for identifying and authenticating people, with applications in access control, medicine and financial services. In this work, an optical system for the image acquisition of the hand vascular pattern is implemented. It consists of a CCD camera with sensitivity in the IR and a light source with emission in the 880 nm. The IR radiation interacts with the desoxyhemoglobin, hemoglobin and water present in the blood of the veins, making possible to see the vein pattern underneath skin. The segmentation of the Region Of Interest (ROI) is achieved using geometrical moments locating the centroid of an image. For enhancement of the vein pattern we use the technique of Histogram Equalization and Contrast Limited Adaptive Histogram Equalization (CLAHE). In order to remove unnecessary information such as body hair and skinfolds, a low pass filter is implemented. A method based on geometric moments is used to obtain the invariant descriptors of the input images. The classification task is achieved using Artificial Neural Networks (ANN) and K-Nearest Neighbors (K-nn) algorithms. Experimental results using our database show a percentage of correct classification, higher of 86.36% with ANN for 912 images of 38 people with 12 versions each one.

  11. [Lymphatic malformations in the head and neck area].

    PubMed

    Wiegand, S; Werner, J A

    2016-02-01

    Lymphatic malformations are congenital malformations of the lymphatic system. They are mainly located in the head and neck area, and grow proportional to the patients' body growth. Depending on the morphology, it can be distinguished between macrocystic, microcystic and mixed lymphatic malformations. Due to their infiltrative growth, microcystic lymphatic malformations are particularly difficult to treat. Therapeutic approaches include conventional surgical resection, laser therapy, sclerotherapy and systemic drug therapies. PMID:26820157

  12. Inflammatory Cytokines in Vascular Dysfunction and Vascular Disease

    PubMed Central

    Sprague, Alexander H.; Khalil, Raouf A.

    2009-01-01

    The vascular inflammatory response involves complex interaction between inflammatory cells (neutrophils, lymphocytes, monocytes, macrophages), endothelial cells (ECs), vascular smooth muscle cells (VSMCs), and extracellular matrix (ECM). Vascular injury is associated with increased expression of adhesion molecules by ECs and recruitment of inflammatory cells, growth factors, and cytokines, with consequent effects on ECs, VSMCs and ECM. Cytokines include tumor necrosis factors, interleukins, lymphokines, monokines, interferons, colony stimulating factors, and transforming growth factors. Cytokines are produced by macrophages, T cells and monocytes, as well as platelets, ECs and VSMCs. Circulating cytokines interact with specific receptors on various cell types and activate JAK-STAT, NF-κB, and Smad signaling pathways leading to an inflammatory response involving cell adhesion, permeability and apoptosis. Cytokines also interact with mitochondria to increasie the production of reactive oxygen species. Cytokine-induced activation of these pathways in ECs modifies the production/activity of vasodilatory mediators such as nitric oxide, prostacyclin, endothelium-derived hyperpolarizing factor, and bradykinin, as well as vasoconstrictive mediators such as endothelin and angiotensin II. Cytokines interact with VSMCs to activate Ca2+, protein kinase C, Rho-Kinase, and MAPK pathways, which promote cell growth and migration, and VSM reactivity. Cytokines also interact with integrins and matrix metalloproteinases (MMPs) and modify ECM composition. Persistent increases in cytokines are associated with vascular dysfunction and vascular disease such as atherosclerosis, abdominal aortic aneurysm, varicose veins and hypertension. Genetic and pharmacological tools to decrease the production of cytokines or to diminish their effects using cytokine antagonists could provide new approaches in the management of inflammatory vascular disease. PMID:19413999

  13. Deltoid Branch of Thoracoacromial Vein

    PubMed Central

    Su, Ta-Wei; Wu, Ching-Feng; Fu, Jui-Ying; Ko, Po-Jen; Yu, Sheng-Yueh; Kao, Tsung-Chi; Hsieh, Hong-Chang; Wu, Ching-Yang

    2015-01-01

    Abstract An entry vessel is crucial for intravenous port implantation. A safe alternative entry vessel that can be easily explored is crucial for patients without feasible cephalic vein or for those who need port reimplantation because of disease relapse. In this study, we tried to analyze the safety and feasibility of catheter implantation via the deltoid branch of the thoracoacromial vein. From March 2012 to November 2013, 802 consecutive oncology patients who had received intravenous port implantation via the superior vena cava were enrolled in this study. The functional results and complications of different entry vessels were compared. The majority of patients (93.6%) could be identified as thoracoacromial vessel. The deltoid branch of the thoracoacromial vein is located on the medial aspect of the deltopectoral groove beneath the pectoralis major muscle (85.8%) and in the deep part of the deltopectoral groove (14.2%). Due to the various calibers employed and tortuous routes followed, we utilized 3 different methods for catheter implantation, including vessel cutdown (47.4%), wire assisted (17.9%), and modified puncture method (34.6%). The functional results and complication rate were similar to other entry vessels. The deltoid branch of the thoracoacromial vein is located in the neighborhood of the cephalic vein. The functional results of intravenous port implantation via the deltoid branch of the thoracoacromial vein are similar to other entry vessels. It is a safe alternative entry vessel for intravenous port implantation. PMID:25929903

  14. A somatic MAP3K3 mutation is associated with verrucous venous malformation.

    PubMed

    Couto, Javier A; Vivero, Matthew P; Kozakewich, Harry P W; Taghinia, Amir H; Mulliken, John B; Warman, Matthew L; Greene, Arin K

    2015-03-01

    Verrucous venous malformation (VVM), also called "verrucous hemangioma," is a non-hereditary, congenital, vascular anomaly comprised of aberrant clusters of malformed dermal venule-like channels underlying hyperkeratotic skin. We tested the hypothesis that VVM lesions arise as a consequence of a somatic mutation. We performed whole-exome sequencing (WES) on VVM tissue from six unrelated individuals and looked for somatic mutations affecting the same gene in specimens from multiple persons. We observed mosaicism for a missense mutation (NM_002401.3, c.1323C>G; NP_002392, p.Iso441Met) in mitogen-activated protein kinase kinase kinase 3 (MAP3K3) in three of six individuals. We confirmed the presence of this mutation via droplet digital PCR (ddPCR) in the three subjects and found the mutation in three additional specimens from another four participants. Mutant allele frequencies ranged from 6% to 19% in affected tissue. We did not observe this mutant allele in unaffected tissue or in affected tissue from individuals with other types of vascular anomalies. Studies using global and conditional Map3k3 knockout mice have previously implicated MAP3K3 in vascular development. MAP3K3 dysfunction probably causes VVM in humans. PMID:25728774

  15. Genetic basis of congenital cardiovascular malformations

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Cardiovascular malformations are a singularly important class of birth defects and due to dramatic improvements in medical and surgical care, there are now large numbers of adult survivors. The etiologies are complex, but there is strong evidence that genetic factors play a crucial role. Over the la...

  16. Magnetic resonance imaging of pediatric soft-tissue vascular anomalies.

    PubMed

    Navarro, Oscar M

    2016-05-01

    Magnetic resonance (MR) imaging can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing and assessing extent of lesions and for evaluating response to therapy. MR imaging studies often involve a combination of T1- and T2-weighted images in addition to MR angiography and fat-suppressed post-contrast sequences. The MR imaging features of these vascular anomalies when combined with clinical findings can aid in diagnosis. In cases of complex vascular malformations and syndromes associated with vascular anomalies, MR imaging can be used to evaluate accompanying soft-tissue and bone anomalies. This article reviews the MR imaging protocols and appearances of the most common pediatric soft-tissue vascular anomalies. PMID:27229506

  17. Retinal vein occlusions: a review for the internist.

    PubMed

    Marcucci, Rossella; Sofi, Francesco; Grifoni, Elisa; Sodi, Andrea; Prisco, Domenico

    2011-08-01

    Retinal vein occlusion (RVO) is a disease that is often associated with a variety of systemic disorders including arterial hypertension, diabetes mellitus, dyslipidemia and systemic vasculitis. There are various types of RVO, categorized on the basis of the site of occlusion and on the type of consequent vascular damage. Central retinal vein occlusion (CRVO) is the most clinically relevant type of RVO. In addition to well-known classical risk factors, new thrombophilic factors have been investigated in patients with RVO. Data concerning a number of the parameters remain contradictory; yet, hyperhomocysteinemia and vitamins involved in methionine metabolism appear to play a significant role in the pathogenesis of this disease. Alterations in the fibrinolysis pathway (elevated levels of PAI-1 and Lipoprotein (a)), together with haemorheologic modifications have been recently consistently associated with the disease. Medical treatment includes identification and correction of vascular risk factors. In addition, LMWHs appear to be the best therapeutic approach even if based on a limited number of trials, conducted on a limited number of patients. No data are available on the possible role of antithrombotic strategies in the long-term prevention of recurrent RVO or vascular events. PMID:21547483

  18. Asymptomatic anomalous pulmonary veins in a Siberian Husky.

    PubMed

    Abraham, L A; Slocombe, R F

    2003-07-01

    A 2-year-old, neutered male Siberian Husky presented with depression, weight loss and an inability to prehend food and water. Cerebrospinal fluid was collected under general anaesthesia prior to euthanasia. The elevated white cell count comprised mostly mononuclear cells. Histological changes within the brain were variable and multifocal. Non-suppurative meningitis secondary to lymphoma was diagnosed. At necropsy, abnormal venous drainage of the right cranial and middle lung lobes was found. A dilated major pulmonary vein from these lobes passed across the lateral aspect of the right caudal lung lobe prior to entering the heart, and subpleural veins from the affected lobes were enlarged and tortuous. These vascular abnormalities were considered incidental. There were no apparent congenital abnormalities of the heart and the animal's clinical signs were related to lymphoma of the brain. PMID:15084052

  19. Osteochondroma mimicking deep vein thrombosis in a young cricketer.

    PubMed

    Watura, Christopher; Patel, Samir

    2012-01-01

    Osteochondromas are bony outgrowths covered by thin cartilaginous caps, accounting for 35% of all benign bone tumours. The majorities are solitary and usually arise at the metaphysis of long bones. They may be associated with bursa formation, while vascular complications are rare. We report a 34-year-old man who presented with a 2-week history of pain, stiffness and swelling of the left lower leg following a cricket match. Clinically, a deep vein thrombosis (DVT) was suspected but D-dimer test was negative. Initial Doppler ultrasound scan (USS) was reported as showing a haematoma and possible DVT. A repeat Doppler USS did not detect a DVT, however the popliteal vein appeared collapsed and a pedunculated osteochondroma of the proximal tibia was found. An MRI confirmed this and also showed an extensive oedema around the calf muscles tracking distally in all compartments, most likely the result of a ruptured bursa. PMID:23188850

  20. Incidence of Central Vein Stenosis and Occlusion Following Upper Extremity PICC and Port Placement

    SciTech Connect

    Gonsalves, Carin F. Eschelman, David J.; Sullivan, Kevin L.; DuBois, Nancy; Bonn, Joseph

    2003-04-15

    .Patients with longer catheter dwell time were more likely to develop central vein abnormalities. In order to preserve vascular access for dialysis fistulae and grafts and adhere to Dialysis Outcomes Quality Initiative guidelines, alternative venous access sites should be considered for patients with chronic renal insufficiency and end-stage renal disease.

  1. [Vascular parkinsonism].

    PubMed

    Yamanouchi, H

    1997-01-01

    Critchley speculated that multiple vascular lesions of the basal ganglia must have an etiological connection to the symptoms of so-called vascular parkinsonism (VP), but without neuropathological confirmation. Some had doubts about its existence because of the lack of the pathologically confirmed case with adequate clinical correlation. At present, VP is characterized clinically by the short-stepped or frozen gait, lead-pipe rigidity, the symmetry of findings, absence of resting tremor, and negative response to levodopa in elderly patients with cerebrovascular lesions on CT/MRI. Pseudobulbar palsies, pyramidal tract findings, and/or multi-infarct dementia coexist in some of the cases. Most of clinically suspected VP patients have cerebral white matter lesions as well as basal ganglia lesions. PMID:9014431

  2. Human vascular model with defined stimulation medium - a characterization study.

    PubMed

    Huttala, Outi; Vuorenpää, Hanna; Toimela, Tarja; Uotila, Jukka; Kuokkanen, Hannu; Ylikomi, Timo; Sarkanen, Jertta-Riina; Heinonen, Tuula

    2015-01-01

    The formation of blood vessels is a vital process in embryonic development and in normal physiology. Current vascular modelling is mainly based on animal biology leading to species-to-species variation when extrapolating the results to humans. Although there are a few human cell based vascular models available these assays are insufficiently characterized in terms of culture conditions and developmental stage of vascular structures. Therefore, well characterized vascular models with human relevance are needed for basic research, embryotoxicity testing, development of therapeutic strategies and for tissue engineering. We have previously shown that the in vitro vascular model based on co-culture of human adipose stromal cells (hASC) and human umbilical vein endothelial cells (HUVEC) is able to induce an extensive vascular-like network with high reproducibility. In this work we developed a defined serum-free vascular stimulation medium (VSM) and performed further characterization in terms of cell identity, maturation and structure to obtain a thoroughly characterized in vitro vascular model to replace or reduce corresponding animal experiments. The results showed that the novel vascular stimulation medium induced intact and evenly distributed vascular-like network with morphology of mature vessels. Electron microscopic analysis assured the three-dimensional microstructure of the network containing lumen. Additionally, elevated expressions of the main human angiogenesis-related genes were detected. In conclusion, with the new defined medium the vascular model can be utilized as a characterized test system for chemical testing as well as in creating vascularized tissue models. PMID:25742497

  3. Genetic animal models of malformations of cortical development and epilepsy.

    PubMed

    Wong, Michael; Roper, Steven N

    2016-02-15

    Malformations of cortical development constitute a variety of pathological brain abnormalities that commonly cause severe, medically-refractory epilepsy, including focal lesions, such as focal cortical dysplasia, heterotopias, and tubers of tuberous sclerosis complex, and diffuse malformations, such as lissencephaly. Although some cortical malformations result from environmental insults during cortical development in utero, genetic factors are increasingly recognized as primary pathogenic factors across the entire spectrum of malformations. Genes implicated in causing different cortical malformations are involved in a variety of physiological functions, but many are focused on regulation of cell proliferation, differentiation, and neuronal migration. Advances in molecular genetic methods have allowed the engineering of increasingly sophisticated animal models of cortical malformations and associated epilepsy. These animal models have identified some common mechanistic themes shared by a number of different cortical malformations, but also revealed the diversity and complexity of cellular and molecular mechanisms that lead to the development of the pathological lesions and resulting epileptogenesis. PMID:25911067

  4. Ipsilateral leg swelling after renal transplantation as an alarming sign of Iliac vein stenosis

    PubMed Central

    Kim, Ju Hyeon; Bae, Seong Man; Park, Su-Kil

    2014-01-01

    Iliac vein stenosis is a rare vascular complication of renal transplantation that may compromise allograft function if not recognized and corrected in a timely fashion. Because chronic venous stenosis may remain undiagnosed for several years, a high index of suspicion should be maintained until diagnosing this rare disease. A 56-year-old renal transplant recipient presented with unilateral leg swelling and renal dysfunction 16 years after transplantation. Computed tomography excluded deep vein thrombosis and revealed tight iliac vein stenosis on the side of the renal transplant. Following angiographic confirmation of the stenosis, endovascular treatment was successfully performed with a purposefully designed, self-expanding, venous stent. Ipsilateral leg swelling is an alarming sign for the diagnosis of iliac vein stenosis after renal transplantation. Percutaneous intervention with venous stent placement seems to be a safe and effective treatment of this rare condition. PMID:26885480

  5. Infrahepatic inferior caval and azygos vein formation in mammals with different degrees of mesonephric development.

    PubMed

    Hikspoors, Jill P J M; Mekonen, Hayelom K; Mommen, Greet M C; Cornillie, Pieter; Köhler, S Eleonore; Lamers, Wouter H

    2016-03-01

    Controversies regarding the development of the mammalian infrahepatic inferior caval and azygos veins arise from using topography rather than developmental origin as criteria to define venous systems and centre on veins that surround the mesonephros. We compared caudal-vein development in man with that in rodents and pigs (rudimentary and extensive mesonephric development, respectively), and used Amira 3D reconstruction and Cinema 4D-remodelling software for visualisation. The caudal cardinal veins (CCVs) were the only contributors to the inferior caval (IVC) and azygos veins. Development was comparable if temporary vessels that drain the large porcine mesonephros were taken into account. The topography of the CCVs changed concomitant with expansion of adjacent organs (lungs, meso- and metanephroi). The iliac veins arose by gradual extension of the CCVs into the caudal body region. Irrespective of the degree of mesonephric development, the infrarenal part of the IVC developed from the right CCV and the renal part from vascular sprouts of the CCVs in the mesonephros that formed 'subcardinal' veins. The azygos venous system developed from the cranial remnants of the CCVs. Temporary venous collaterals in and around the thoracic sympathetic trunk were interpreted as 'footprints' of the dorsolateral-to-ventromedial change in the local course of the intersegmental and caudal cardinal veins relative to the sympathetic trunk. Interspecies differences in timing of the same events in IVC and azygos-vein development appear to allow for proper joining of conduits for caudal venous return, whereas local changes in topography appear to accommodate efficient venous perfusion. These findings demonstrate that new systems, such as the 'supracardinal' veins, are not necessary to account for changes in the course of the main venous conduits of the embryo. PMID:26659476

  6. [Summary of 2004 Chinese National Conference on oral and maxillofacial vascular anomalies].

    PubMed

    Zheng, Jia-Wei; Zhang, Zhi-Yuan; Zheng, Cang-Shang; Zhou, Qi

    2004-08-01

    Congenital vascular anomalies have been the subject of much controversy and confusion over the years, many remain to be investigated and resolved. Authorized by the Managing Director Board of Chinese Stomatological Association (CSA), 2004 Chinese National Conference on Oral and Maxillofacial Vascular Anomalies was successfully held on July 15 to 18, 2004 in Shenzhen Grand Hotel. The conference was sponsored by Chinese Society of Oral and Maxillofacial Surgery (CSOMS), and undertaken by the Second People's Hospital of Shenzhen city. Professor ZHANG Zhen-kang, president of CSA, Professor QIU Wei-liu, president of CSOMS and academician of Chinese Academy of Engineers, and Professor LIU Bao-lin, vice president of CSOMS were present and made important speech at the meeting. More than 120 delegates nationwide attended this conference. Through a 3-day of oral presentation and discussion, the terminology, classification, treatment selection and outcome measurement of oral and maxillofacial hemangioma and vascular malformations were concerted among the delegates. Hemangiomas and vascular malformations have been recognized as distinct diseases that exhibit unique properties and behavior that demand an appropriately tailored treatment plan. The classification of Waner and Suen was adopted and different treatment modalities were reviewed and advocated for different lesions. Delegates were most interested in intralesional injection of Pingyangmycin for venous malformations, Krypton laser photodynamic therapy of venular malformations, Nd:YAG laser therapy for deep head and neck venous malformations after surgical exposure of the lesions, as well as "double" embolization of large venous malformations reported by Professor QIN Zhong-ping. The conference witnessed the foundation of the Division of Vascular Anomalies, Chinese Society of Oral and Maxillofacial Surgery. PMID:15349690

  7. Extracellular matrix remodelling in response to venous hypertension: proteomics of human varicose veins

    PubMed Central

    Barallobre-Barreiro, Javier; Oklu, Rahmi; Lynch, Marc; Fava, Marika; Baig, Ferheen; Yin, Xiaoke; Barwari, Temo; Potier, David N.; Albadawi, Hassan; Jahangiri, Marjan; Porter, Karen E.; Watkins, Michael T.; Misra, Sanjay; Stoughton, Julianne; Mayr, Manuel

    2016-01-01

    Aims Extracellular matrix remodelling has been implicated in a number of vascular conditions, including venous hypertension and varicose veins. However, to date, no systematic analysis of matrix remodelling in human veins has been performed. Methods and results To understand the consequences of venous hypertension, normal and varicose veins were evaluated using proteomics approaches targeting the extracellular matrix. Varicose saphenous veins removed during phlebectomy and normal saphenous veins obtained during coronary artery bypass surgery were collected for proteomics analysis. Extracellular matrix proteins were enriched from venous tissues. The proteomics analysis revealed the presence of >150 extracellular matrix proteins, of which 48 had not been previously detected in venous tissue. Extracellular matrix remodelling in varicose veins was characterized by a loss of aggrecan and several small leucine-rich proteoglycans and a compensatory increase in collagen I and laminins. Gene expression analysis of the same tissues suggested that the remodelling process associated with venous hypertension predominantly occurs at the protein rather than the transcript level. The loss of aggrecan in varicose veins was paralleled by a reduced expression of aggrecanases. Chymase and tryptase β1 were among the up-regulated proteases. The effect of these serine proteases on the venous extracellular matrix was further explored by incubating normal saphenous veins with recombinant enzymes. Proteomics analysis revealed extensive extracellular matrix degradation after digestion with tryptase β1. In comparison, chymase was less potent and degraded predominantly basement membrane-associated proteins. Conclusion The present proteomics study provides unprecedented insights into the expression and degradation of structural and regulatory components of the vascular extracellular matrix in varicosis. PMID:27068509

  8. Portal Vein Thrombosis in Cirrhosis

    PubMed Central

    Raja, Kaiser; Jacob, Mathew; Asthana, Sonal

    2013-01-01

    Portal vein thrombosis (PVT) is being increasingly recognized in patients with advanced cirrhosis and in those undergoing liver transplantation. Reduced flow in the portal vein is probably responsible for clotting in the spleno-porto-mesenteric venous system. There is also increasing evidence that hypercoagulability occurs in advanced liver disease and contributes to the risk of PVT. Ultrasound based studies have reported a prevalence of PVT in 10–25% of cirrhotic patients without hepatocellular carcinoma. Partial thrombosis of the portal vein is more common and may not have pathophysiological consequences. However, there is high risk of progression of partial PVT to complete PVT that may cause exacerbation of portal hypertension and progression of liver insufficiency. It is thus, essential to accurately diagnose and stage PVT in patients waiting for transplantation and consider anticoagulation therapy. Therapy with low molecular weight heparin and vitamin K antagonists has been shown to achieve complete and partial recanalization in 33–45% and 15–35% of cases respectively. There are however, no guidelines to help determine the dose and therapeutic efficacy of anticoagulation in patients with cirrhosis. Anticoagulation therapy related bleeding is the most feared complication but it appears that the risk of variceal bleeding is more likely to be dependent on portal pressure rather than solely related to coagulation status. TIPS has also been reported to restore patency of the portal vein. Patients with complete PVT currently do not form an absolute contraindication for liver transplantation. Thrombectomy or thromboendovenectomy is possible in more than 75% of patients followed by anatomical end-to-end portal anastomosis. When patency of the portal vein and/or superior mesenteric vein is not achieved, only non-anatomical techniques (reno-portal anastomosis or cavo-portal hemitransposition) can be performed. These techniques, which do not fully reverse portal

  9. Management of superficial vein thrombosis.

    PubMed

    Cosmi, B

    2015-07-01

    Superficial vein thrombosis (SVT) is less well studied than deep vein thrombosis (DVT), because it has been considered to be a minor, self-limiting disease that is easily diagnosed on clinical grounds and that requires only symptomatic relief. The most frequently involved sites of the superficial vein system are the lower limbs, especially the saphenous veins, mostly in relation to varicosities. Lower-limb SVT shares the same risk factors as DVT; it can propagate into the deep veins, and have a complicated course with pulmonary embolism. Clinical diagnosis may not be accurate, and ultrasonography is currently indicated for both confirmation and evaluation of SVT extension. Treatment aims are symptom relief and prevention of venous thromboembolism (VTE) in relation to the thrombotic burden. SVT of the long saphenous vein within 3 cm of the saphenofemoral junction (SFJ) is considered to be equivalent to a DVT, and thus deserving of therapeutic anticoagulation. Less severe forms of lower-limb SVT not involving the SFJ have been included in randomized clinical trials of surgery, compression hosiery, non-steroidal anti-inflammatory drugs, unfractionated heparin, and low molecular weight heparins, with inconclusive results. The largest randomized clinical trial available, on 3004 patients with lower-limb SVT not involving the SFJ, showed that fondaparinux 2.5 mg once daily for 6 weeks is more effective than placebo in reducing the risk of the composite of death from any cause and symptomatic VTE (0.9% versus 5.9%). Further studies are needed to define the optimal management strategies for SVT of the lower limbs and other sites, such as the upper limbs. PMID:25903684

  10. 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

  11. Massive glosso-cervical arteriovenous malformation: The rationale for a challenging surgical resection

    PubMed Central

    González-García, Raúl; Moreno-García, Carlos

    2014-01-01

    Massive arterivenous malformations (AVM) in the cervico-facial area are rare but potentially life-threatening. Treatment protocols are not well-established. A 41-year old man presented large painless rubber-like mass within the entire neck, which also extended intraorally through the floor of the mouth, showing a slow growing pattern for 5 years. Angiography diagnosed it as cervicofacial AVM. Treatment approach consisted on the embolization of the right upper thyroid, lingual and facial arteries under intravenous sedation. Three days later, bilateral radical neck dissection and subtotal glossectomy was performed. A musculo-cutaneous pectoralis major pedicled flap was harvested to reconstruct the floor of the mouth. Treatment of massive AVMs in the cervico-facial area is challenging due to the associated disfigurement and frequent recurrence rate due to incomplete resection. Also, massive bleeding may be present despite pre-operative super-selective embolization. A new case is presented with focus on surgical treatment considerations. Key words:Arteriovenous malformation, high-flow vascular malformation, cervical region, tongue, surgical resection PMID:25593675

  12. Delayed presentation of an arteriovenous malformation after cerebellar hemangioblastoma resection—Case report

    PubMed Central

    Bennett, E. Emily; Otvos, Balint; Kshettry, Varun R.; Gonzalez-Martinez, Jorge

    2016-01-01

    Introduction Haemangioblastoma has been uncommonly reported to occur in coexistence either temporally or spatially with the development of an arteriovenous malformations (AVM). We present a case of a delayed AVM following haemangioblastoma resection. Presentation of case 44 year old female initially presented with a several week history of headaches, vertigo and nausea and emesis and was found to have a cystic lesion with a solid enhancing component on Magnetic Resonance Imaging (MRI) in the superior aspect of the vermis. She underwent gross total resection and final pathology was consistent with WHO grade I haemangioblastoma. One year later, patient re-presented with headaches, dizziness and left trochlear nerve palsy with rotary nystagmus. Imaging revealed a left posterior tentorial paramedian cerebellar vascular nidus with venous drainage into the left transverses sinus suspicious for arteriovenous malformation. She underwent gross total resection of the lesion. Final pathology confirmed the diagnosis of an arteriovenous malformation. Discussion Recent research supports both haemangioblastoma and AVM are of embryologic origin but require later genetic alterations to develop into symptomatic lesions. It is unclear in our case if the AVM was present at the time of the initial haemangioblastoma resection or developed de novo after tumor resection. However, given the short time between tumor resection and presentation of AVM, de novo AVM although possible, appears less likely. Conclusion AVM and haemangioblastoma rarely presents together either temporally or spatially. We present a case of a delayed AVM following haemangioblastoma resection. More research is needed to elucidate the rare intermixture of these lesions. PMID:27086272

  13. [A case of arteriovenous malformation showed onset of symptoms on the late neonatal period].

    PubMed

    Yamasaki, S; Shirakuni, T; Hojo, H

    1982-08-01

    Arteriovenous malformation ruptured in the neonatal period is rare. The authors report a case of arteriovenous malformation showed onset of symptoms on the late neonatal period. A female Japanese baby was born at full term. Neonatal history was uneventful until she had bad temper, pallor and vomiting on 23th day. Her head size increased abnormally until it was 42.5 cm when she was admitted to Shizuoka Children's Hospital with a diagnosis of possible hydrocephalus at 2 months old. CT scan showed a large cystic cavity communicated with the dilated lateral ventricles. The ventriculo-peritoneal shunt was performed, but unfortunately the shunt was removed for the suspicion of abdominal complication. At the age of 4 months, right retrograde brachial angiography showed a tangle of abnormal vascular channels in the right fronto-lateral basal region (15X13X8 mm in size), which was fed a frontopolar artery and drained to superior saggital sinus. Frontal osteoplastic craniotomy was performed and total arteriovenous malformation with a partial frontal lobe was excised. Ventriculo-peritoneal shunt was reinserted for the post-hemorrhagic hydrocephalus. She was healthy on 3 years old with mild motor deficit. PMID:7133311

  14. Effects of gravitational stress, hypokinesia and hypodynamia on the structure of the vascular bed of the spleen

    NASA Technical Reports Server (NTRS)

    Nesterenko, N. T.

    1980-01-01

    The effects of two extreme factors, hypokinesia and hypodynamia, on spleen vascular beds were studied on 180 male and female albino rats. Vessels were studied by roentgenography, microroentgenography, clearing of sections and histology. Gravity stress yielded construction of all links of arterial bed and of order 5-7 veins. Large intraorganic vein diameters changed significantly but erratically. Hypokinesia in early phases produced pronounced spleen size reduction. Veins and arteries constricted along entire length. Later hypokinetic stages showed arteries still constricted; veins began to dilate from week 4 of hypokinesia. Sinuosity, uneven contours and varicose dilations of walls in large arteries and veins occurred. Abrupt changes in parenchyma, e.g., atrophy of folliculi, narrowing of lumen of central arteries from thickening of muscular wall. After exposure to hypokinesia followed by gravitational stress, pronounced lesions such as deformation of vascular wall, including rupture, in all vessels of the spleen vascular bed.

  15. Endovascular Laser Therapy for Varicose Veins

    PubMed Central

    2010-01-01

    back pain and arthritis. Lower limb VV is a common disease affecting adults and estimated to be the seventh most common reason for physician referral in the US. There is a strong familial predisposition to VV with the risk in offspring being 90% if both parents affected, 20% when neither is affected, and 45% (25% boys, 62% girls) if one parent is affected. Globally, the prevalence of VV ranges from 5% to 15% among men and 3% to 29% among women varying by the age, gender and ethnicity of the study population, survey methods and disease definition and measurement. The annual incidence of VV estimated from the Framingham Study was reported to be 2.6% among women and 1.9% among men and did not vary within the age range (40-89 years) studied. Approximately 1% of the adult population has a stasis ulcer of venous origin at any one time with 4% at risk. The majority of leg ulcer patients are elderly with simple superficial vein reflux. Stasis ulcers are often lengthy medical problems and can last for several years and, despite effective compression therapy and multilayer bandaging are associated with high recurrence rates. Recent trials involving surgical treatment of superficial vein reflux have resulted in healing and significantly reduced recurrence rates. Endovascular Laser Therapy for VV ELT is an image-guided, minimally invasive treatment alternative to surgical stripping of superficial venous reflux. It does not require an operating room or general anesthesia and has been performed in outpatient settings by a variety of medical specialties including surgeons (vascular or general), interventional radiologists and phlebologists. Rather than surgically removing the vein, ELT works by destroying, cauterizing or ablating the refluxing vein segment using heat energy delivered via laser fibre. Prior to ELT, colour-flow Doppler ultrasonography is used to confirm and map all areas of venous reflux to devise a safe and effective treatment plan. The ELT procedure involves the

  16. Animal Models in Studying Cerebral Arteriovenous Malformation

    PubMed Central

    Xu, Ming; Xu, Hongzhi; Qin, Zhiyong

    2015-01-01

    Brain arteriovenous malformation (AVM) is an important cause of hemorrhagic stroke. The etiology is largely unknown and the therapeutics are controversial. A review of AVM-associated animal models may be helpful in order to understand the up-to-date knowledge and promote further research about the disease. We searched PubMed till December 31, 2014, with the term “arteriovenous malformation,” limiting results to animals and English language. Publications that described creations of AVM animal models or investigated AVM-related mechanisms and treatments using these models were reviewed. More than 100 articles fulfilling our inclusion criteria were identified, and from them eight different types of the original models were summarized. The backgrounds and procedures of these models, their applications, and research findings were demonstrated. Animal models are useful in studying the pathogenesis of AVM formation, growth, and rupture, as well as in developing and testing new treatments. Creations of preferable models are expected. PMID:26649296

  17. FTO variant associated with malformation syndrome.

    PubMed

    Rohena, Luis; Lawson, Michelle; Guzman, Edwin; Ganapathi, Mythily; Cho, Megan T; Haverfield, Eden; Anyane-Yeboa, Kwame

    2016-04-01

    Common FTO variants are associated with obesity. However, it has recently been shown that homozygous FTO c.947G>A variant, which predicts p.R316Q, and c.956C>T, which predicts p.S319F, are associated with a malformation syndrome inherited in an autosomal recessive pattern. We present a similar homozygous FTO c.965G>A variant that predicts p.R322Q, associated with a lethal malformation syndrome in a consanguineous Yemeni family. Functional studies showed that the p.R316Q, p.S219F, and p.R322Q variants render the FTO protein inactive. We further expand on the phenotype of homozygous FTO loss-of-function mutations to include eye abnormalities, gingival overgrowth, craniosynostosis, and cutaneous photosensitivity. PMID:26697951

  18. Introduction to cerebral cavernous malformation: a brief review.

    PubMed

    Kim, Jaehong

    2016-05-01

    The disease known as cerebral cavernous malformations mostly occurs in the central nervous system, and their typical histological presentations are multiple lumen formation and vascular leakage at the brain capillary level, resulting in disruption of the blood-brain barrier. These abnormalities result in severe neurological symptoms such as seizures, focal neurological deficits and hemorrhagic strokes. CCM research has identified 'loss of function' mutations of three ccm genes responsible for the disease and also complex regulation of multiple signaling pathways including the WNT/β-catenin pathway, TGF-β and Notch signaling by the ccm genes. Although CCM research is a relatively new and small scientific field, as CCM research has the potential to regulate systemic blood vessel permeability and angiogenesis including that of the blood-brain barrier, this field is growing rapidly. In this review, I will provide a brief overview of CCM pathogenesis and function of ccm genes based on recent progress in CCM research. [BMB Reports 2016; 49(5): 255-262]. PMID:26923303

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

    PubMed Central

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

    2014-01-01

    We present a case of a 70-year-old man with abdominal aortic aneurysm and coincident pelvic arteriovenous malformation (AVM). Before the operation for the aneurysm, we embolised the pelvic AVM that had multiple feeding arteries and an aneurysmal-dilated draining vein. After decreasing the number of the feeding arteries by coil embolisation, an n-butyl-2-cyanoacrylate/lipiodol mixture (1:1) was injected into the prominent feeding artery and nidus with proximal balloon occlusion of the right internal iliac artery to decrease the flow to the nidus. The mixture (1:4–8) was also added for the finer feeding arteries that became apparent after the initial procedure to embolise the rest of the nidus. A follow-up study showed no contrast enhancement of the nidus and aneurysmal draining vein. PMID:24907213

  20. Ischemic Colitis Due to a Mesenteric Arteriovenous Malformation in a Patient with a Connective Tissue Disorder

    PubMed Central

    Poullos, Peter D.; Thompson, Atalie C.; Holz, Grant; Edelman, Lauren A.; Jeffrey, R. Brooke

    2014-01-01

    Ischemic colitis is a rare, life-threatening, consequence of mesenteric arteriovenous malformations. Ischemia ensues from a steal phenomenon through shunting, and may be compounded by the resulting portal hypertension. Computed tomographic angiography is the most common first-line test because it is quick, non-invasive, and allows for accurate anatomic characterization. Also, high-resolution three-dimensional images can be created for treatment planning. Magnetic resonance angiography is similarly sensitive for vascular mapping. Conventional angiography remains the gold standard for diagnosis and also allows for therapeutic endovascular embolization. Our patient underwent testing using all three of these modalities. We present the first reported case of this entity in a patient with a vascular connective tissue disorder. PMID:25926912

  1. Disappearance of a Ruptured Distal Flow-Related Aneurysm after Arteriovenous Malformation Nidal Embolization.

    PubMed

    He, Lucy; Gao, Junwei; Thomas, Ajith J; Fusco, Matthew R; Ogilvy, Christopher S

    2015-11-01

    Aneurysms associated with arteriovenous malformations (AVMs) are well represented in the literature. Their exact etiology is poorly understood, but likely global hemodynamic changes coupled with vascular wall pathology play into their formation. Flow-related and intranidal aneurysms, in particular, appear to have an increased risk for hemorrhagic presentation. Treatment strategies for these aneurysms are particularly challenging. We report the case of an AVM-associated aneurysm causing intraventricular hemorrhage that disappeared after embolization of unrelated, distal feeding pedicles to the nidus, at a site distant from the aneurysm. We also review the literature with regards to these so-called "disappearing aneurysms" in the context of AVMs and other vascular pathologies. PMID:26072456

  2. Novel Paradigms for Dialysis Vascular Access: Downstream Vascular Biology–Is There a Final Common Pathway?

    PubMed Central

    2013-01-01

    Summary Vascular access dysfunction is a major cause of morbidity and mortality in hemodialysis patients. The most common cause of vascular access dysfunction is venous stenosis from neointimal hyperplasia within the perianastomotic region of an arteriovenous fistula and at the graft-vein anastomosis of an arteriovenous graft. There have been few, if any, effective treatments for vascular access dysfunction because of the limited understanding of the pathophysiology of venous neointimal hyperplasia formation. This review will (1) describe the histopathologic features of hemodialysis access stenosis; (2) discuss novel concepts in the pathogenesis of neointimal hyperplasia development, focusing on downstream vascular biology; (3) highlight future novel therapies for treating downstream biology; and (4) discuss future research areas to improve our understanding of downstream biology and neointimal hyperplasia development. PMID:23990166

  3. Statins and congenital malformations: cohort study

    PubMed Central

    Hernandez-Diaz, Sonia; Fischer, Michael A; Seely, Ellen W; Ecker, Jeffrey L; Franklin, Jessica M; Desai, Rishi J; Allen-Coleman, Cora; Mogun, Helen; Avorn, Jerry; Huybrechts, Krista F

    2015-01-01

    Objective To examine the teratogenic potential of statins. Design Cohort study. Setting United States. Participants A cohort of 886 996 completed pregnancies linked to liveborn infants of women enrolled in Medicaid from 2000 to 2007. Methods We examined the risk of major congenital malformations and organ specific malformations in offspring associated with maternal use of a statin in the first trimester. Propensity score based methods were used to control for potential confounders, including maternal demographic characteristics, obstetric and medical conditions, and use of other drugs. Results 1152 (0.13%) women used a statin during the first trimester. In unadjusted analyses, the prevalence of malformations in the offspring of these women was 6.34% compared with 3.55% in those of women who did not use a statin in the first trimester (relative risk 1.79, 95% confidence interval 1.43 to 2.23). Controlling for confounders, particularly pre-existing diabetes, accounted for this increase in risk (1.07, 0.85 to 1.37). There were also no statistically significant increases in any of the organ specific malformations assessed after accounting for confounders. Results were similar across a range of sensitivity analyses. Conclusions Our analysis did not find a significant teratogenic effect from maternal use of statins in the first trimester. However, these findings need to be replicated in other large studies, and the long term effects of in utero exposure to statins needs to be assessed, before use of statins in pregnancy can be considered safe. PMID:25784688

  4. Magnetic Resonance Imaging of Malformations of Midbrain-Hindbrain.

    PubMed

    Abdel Razek, Ahmed Abdel Khalek; Castillo, Mauricio

    2016-01-01

    We aim to review the magnetic resonance imaging appearance of malformations of midbrain and hindbrain. These can be classified as predominantly cerebellar malformations, combined cerebellar and brain stem malformations, and predominantly brain stem malformations. The diagnostic criteria for the majority of these morphological malformations are based on neuroimaging findings. The predominantly cerebellar malformations include predominantly vermian hypoplasia seen in Dandy-Walker malformation and rhombencephalosynapsis, global cerebellar hypoplasia reported in lissencephaly and microlissencephaly, and unilateral cerebellar hypoplasia seen in PHACES, vanishing cerebellum, and cerebellar cleft. Cerebellar dysplasias are seen in Chudley-McCullough syndrome, associated with LAMA1 mutations and GPR56 mutations; Lhermitte-Duclos disease; and focal cerebellar dysplasias. Cerebellar hyperplasias are seen in megalencephaly-related syndromes and hemimegalencephaly with ipsilateral cerebellomegaly. Cerebellar and brain stem malformations include tubulinopathies, Joubert syndrome, cobblestone malformations, pontocerebellar hypoplasias, and congenital disorders of glycosylation type Ia. Predominantly brain stem malformations include congenital innervation dysgenesis syndrome, pontine tegmental cap dysplasia, diencephalic-mesencephalic junction dysplasia, disconnection syndrome, and pontine clefts. PMID:26599961

  5. Vascular Tumor on the Forehead of an HIV Patient

    PubMed Central

    Ibarguren, Ander Mayor; Ramos, Jéssica González; Merino, María Beato; Pedrero, Rocío Maseda; Fernández, Cristina Gómez; Pinto, Pedro Herranz

    2015-01-01

    Cirsoid aneurysm is a small vascular proliferation characterized by small to medium-sized channels with features of arteries and veins, that present as small, blue or red asymptomatic papule. We report a case of a crisoid aneurysm on the forhead of an HIV patient that suggested a Kaposi sarcoma as a differential diagnosis. PMID:26288449

  6. Neuroembryology and brain malformations: an overview.

    PubMed

    Sarnat, Harvey B; Flores-Sarnat, Laura

    2013-01-01

    Modern neuroembryology integrates descriptive morphogenesis with more recent insight into molecular genetic programing and data enabled by cell-specific tissue markers that further define histogenesis. Maturation of individual neurons involves the development of energy pumps to maintain membrane excitability, ion channels, and membrane receptors. Most malformations of the nervous system are best understood in the context of aberrations of normal developmental processes that result in abnormal structure and function. Early malformations usually are disorders of genetic expression along gradients of the three axes of the neural tube, defective segmentation, or mixed lineages of individual cells. Later disorders mainly involve cellular migrations, axonal pathfinding, synaptogenesis, and myelination. Advances in neuroimaging now enable the diagnosis of many malformations in utero, at birth, or in early infancy in the living patient by abnormal macroscopic form of the brain. These images are complimented by modern neuropathological methods that disclose microscopic, immunocytochemical, and subcellular details beyond the resolution of MRI. Correlations may be made of both normal and abnormal ontogenesis with clinical neurological and EEG maturation in the preterm or term neonate for a better understanding of perinatal neurological disease. Precision in terminology is a key to scientific communication. PMID:23622157

  7. Malformations of cortical development and epilepsy.

    PubMed

    Leventer, Richard J; Guerrini, Renzo; Dobyns, William B

    2008-01-01

    Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures. A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etiologic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD. The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaly, classical lissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented. PMID:18472484

  8. Chiari I malformations: clinical and radiologic reappraisal.

    PubMed

    Elster, A D; Chen, M Y

    1992-05-01

    Clinical findings and magnetic resonance (MR) images in 68 patients with Chiari I malformations were retrospectively analyzed to identify those radiologic features that correlated best with clinical symptoms. A statistically significant (P = .03) female predominance of the malformation was observed, with a female: male ratio of approximately 3:2. Associated skeletal anomalies were seen in 24% of patients. Syringomyelia was detected in 40% of patients, most commonly between the C-4 and C-6 levels. Of the 25 patients who presented with spinal symptoms, 23 (92%) proved to have a syrinx at MR imaging. When the syrinx extended into the medulla (n = 3), however, brain stem symptoms predominated. Patients with objective brain stem or cerebellar signs had the largest mean tonsillar herniations. Patients with tonsillar herniations greater than 12 mm were invariably symptomatic, but approximately 30% of patients with tonsils herniating 5-10 mm below the foramen magnum were asymptomatic at MR imaging. "Incidental" Chiari I malformations are thus much more common than previously recognized, and careful clinical assessment remains the cornerstone for proper diagnosis and management. PMID:1561334

  9. Combined branch retinal vein and artery occlusion in toxoplasmosis.

    PubMed

    Aggio, Fabio Bom; Novelli, Fernando José de; Rosa, Evandro Luis; Nobrega, Mário Junqueira

    2016-01-01

    A 22-year-old man complained of low visual acuity and pain in his left eye for five days. His ophthalmological examination revealed 2+ anterior chamber reaction and a white, poorly defined retinal lesion at the proximal portion of the inferotemporal vascular arcade. There were retinal hemorrhages in the inferotemporal region extending to the retinal periphery. In addition, venous dilation, increased tortuosity, and ischemic retinal whitening along the inferotemporal vascular arcade were also observed. A proper systemic work-up was performed, and the patient was diagnosed with ocular toxoplasmosis. He was treated with an anti-toxoplasma medication, and his condition slowly improved. Inferior macular inner and middle retinal atrophy could be observed on optical coherence tomography as a sequela of ischemic injury. To our knowledge, this is the first report of combined retinal branch vein and artery occlusion in toxoplasmosis resulting in a striking and unusual macular appearance. PMID:27463632

  10. Nd:YAG laser photocoagulation of benign oral vascular lesions: a case series.

    PubMed

    Medeiros, Rui; Silva, Igor Henrique; Carvalho, Alessandra Tavares; Leão, Jair Carneiro; Gueiros, Luiz Alcino

    2015-11-01

    Vascular anomalies of the head and neck are common lesions usually associated with functional and/or aesthetic limitations. The aim of the present paper was to report a case series of oral vascular malformations treated with Nd:YAG laser photocoagulation, highlighting the clinical evolution and post-surgical complications. Fifteen patients diagnosed with oral vascular malformations were treated with Nd:YAG laser followed by three sessions of biostimulation. None of the patients presented post-surgical pain, but 6 of 15 patients (40%) experienced minimal post-surgical complications. All cases presented complete resolution of the lesions after laser treatment. More importantly, 12 out of 15 (80%) resolved after a single session. Low morbidity, minimal patient discomfort, and satisfactory aesthetic results point Nd:YAG laser photocoagulation as a promising option for the management of benign oral vascular lesions. PMID:25962368

  11. Embolisation of Small (< 3 cm) Brain Arteriovenous Malformations

    PubMed Central

    Willinsky, R.; Goyal, M.; terBrugge, K.; Montanera, W.; Wallace*, M.G; Tymianski*, M.

    2001-01-01

    Summary The role of embolisation in the treatment of small (< 3cm) brain arteriovenous malformations (AVMs) has not been elucidated. We reviewed our experience using embolisation in the treatment of small AVMs and correlated a proposed grading system based on the angioarchitecture to the percentage obliteration achieved by embolisation. Eighty-one small AVMs in 80 patients were embolised from 1984 to 1999. The age range was from 3 to 72 years. The AVMs were given a score from 0 to 6 based on the angioarchitecture. The assigned scores were as follows: nidus (fistula = 0, < 1 cm = 1,1-3 cm = 2), type of feeding arteries (cortical = 0, perforator or choroidal = 1), number of feeding arteries (single = 0, multiple -2) and number of draining veins (single = 0\\ multiple - 1). Angiographic results based on percentage obliteration were grouped into three categories: complete, 66-99%, and 0-65%. The goal of embolisation was cure in 27 AVMs, pre-surgical in 23, pre-radiosurgery in 26, and elimination of an aneurysm in five. Embolisation achieved complete obliteration in 22 (27%) of the 81 AVMs. In the AVMs where the goal was cure, 19 (70%) of 27 were completely obliterated. In the AVMs with angioarchitecture scores of 0-2, 12 (86%) of 14 were cured, with scores of 3-4, 8 (34%) of 24 were cured and with scores of 5-6, 2 (4%) of 44 were cured. Embolisation resulted in transient morbidity of 5.0%, permanent morbidity of 2.5%, and mortality of 1.2%. There were no complications in AVMs with scores of 0-2. Embolisation is an effective treatment of small AVMs when the angioarchitecture is favourable (scores 0-2). This includes pure fistulas and AVMs with a single, pial, feeding artery. PMID:20663327

  12. Vascular dementia

    PubMed Central

    Korczyn, Amos D; Vakhapova, Veronika; Grinberg, Lea T

    2012-01-01

    The epidemic grow of dementia causes great concern for the society. It is customary to consider Alzheimer’s disease (AD) as the most common cause of dementia, followed by vascular dementia (VaD). This dichotomous view of a neurodegenerative disease as opposed to brain damage caused by extrinsic factors led to separate lines of research in these two entities. Indeed, accumulated data suggest that the two disorders have additive effects and probably interact; however it is still unknown to what degree. Furthermore, epidemiological studies have shown “vascular” risk factors to be associated with AD. Therefore, a clear distinction between AD and VaD cannot be made in most cases, and is furthermore unhelpful. In the absence of efficacious treatment for the neurodegenerative process, special attention must be given to vascular component, even in patients with presumed mixed pathology. Symptomatic treatment of VaD and AD are similar, although the former is less effective. For prevention of dementia it is important to treat aggressively all factors, even in stroke survivors who do not show evidence of cognitive decline,. In this review, we will give a clinical and pathological picture of the processes leading to VaD and discuss it interaction with AD. PMID:22575403

  13. Blackberry Yellow Vein Disease Complex

    Technology Transfer Automated Retrieval System (TEKTRAN)

    A new virus disease has emerged in the Midsouth and Southeastern United States and was named blackberry yellow vein disease (BYVD). Originally, it was thought the disease was caused by Tobacco ringspot virus (TRSV) as the virus was found in many diseased plants and symptoms were very similar to thos...

  14. The Treatment of Varicose Veins

    PubMed Central

    Subramonia, S; Lees, TA

    2007-01-01

    INTRODUCTION Over the past few years, there has been a move to less invasive endoluminal methods in the treatment of lower limb varicose veins combined with a renewed interest in sclerotherapy, with the recent addition of foam sclerotherapy. The development of these new techniques has led many to question some of the more conventional teaching on the treatment of varicose veins. This review examines these new treatments for lower limb varicose veins and the current evidence for their use. MATERIALS AND METHODS An extensive search of available electronic and paper-based databases was performed to identify studies relevant to the treatment of varicose veins with particular emphasis on those published within the last 10 years. These were analysed by both reviewers independently. RESULTS There is no single method of treatment appropriate for all cases. Conventional surgery is safe and effective and is still widely practised. Whilst the new treatments may be popular with both surgeons and patients, it is important that they are carefully evaluated not only for their clinical benefits and complications when compared to existing treatments but also for their cost prior to their wider acceptance into clinical practice. PMID:17346396

  15. Diagnosis of deep vein thrombosis.

    PubMed Central

    Douketis, J. D.; Ginsberg, J. S.

    1996-01-01

    Deep vein thrombosis (DVT), a common disease, can be difficult to diagnose because its clinical features are nonspecific. Venography is the standard test, but other less expensive, easily performed, noninvasive tests are available. At present, duplex ultrasonography is the noninvasive test of choice. PMID:8616289

  16. Treatment of major vein injury with the hemostatic fleece TachoSil by interposing a peritoneal patch to avoid vein thrombosis: A feasibility study in pigs

    PubMed Central

    Dregelid, Einar B; Pedersen, Gustav

    2011-01-01

    Background: Vein lacerations in awkward locations are difficult to repair and carry high mortality. The hemostatic fleece, TachoSil, is effective in preventing intraoperative bleeding in different settings, but has not been recommended for use in large vein injury. TachoSil with a peritoneal patch interposed to avoid vein thrombosis has been reported as a method to obtain hemostasis in vein laceration, but further studies of this method are needed. Materials and Methods: A 1.5 × 1 cm defect was created in the vena cava in five pigs. A 26 × 32 mm peritoneal patch was applied on the coagulant side of a 48 × 48 mm TachoSil sheet, and used to cover the defect. Light compression with a wet sponge was applied for 3 min. No vascular suturing was performed. Results: Successful hemostasis was obtained in four out of the five pigs although the minimum TachoSil gluing zone surrounding the peritoneal patch was only 0–2 mm. The fifth pig died of hemorrhage 30 min after surgery due to a 4-mm stretch with no TachoSil gluing zone outside the peritoneal patch. At six days postoperatively the peritoneal patch was well integrated into the vein wall. After 28 days, the peritoneal patch was almost indiscernible from surrounding vein endothelium. Conclusions: Vein wall defects can be repaired using TachoSil with a peritoneal patch interposed to prevent contact between the thrombogenic TachoSil sheet and the vein lumen. An adequate TachoSil gluing zone all around the patch is essential. PMID:21633573

  17. Varicose veins - what to ask your doctor

    MedlinePlus

    ... Below are some questions you may want to ask your health care provider to help you take ... What to ask your doctor about varicose veins; Venous insufficiency - what to ask your doctor; Vein stripping - what to ask your ...

  18. [Vascular lesions of the small intestine].

    PubMed

    Yano, Tomonori; Yamamoto, Hironori

    2008-07-01

    Small-intestinal vascular lesions accounted for the bleeding source in a large percentage of the patients with mid-GI-bleeding. The progress of enteroscopy has been changing the diagnostic and therapeutic algorithm for them. There are 3 pathological conditions of vascular lesions. Angioectasia is characterized by venous/capillary lesions, Dieulafoy' s lesion is characterized by arterial lesions, and AVM is a condition in which arteries and veins are directly connected without capillary beds. We classified vascular lesions with consideration of the presence or absence of pulsatility. The presence or absence of arterial components provides important information in understanding the pathological conditions. This classification will be useful for selecting hemostatic procedure and outcome studies. PMID:18616125

  19. Developmental timing of CCM2 loss influences cerebral cavernous malformations in mice

    PubMed Central

    Boulday, Gwénola; Rudini, Noemi; Maddaluno, Luigi; Blécon, Anne; Arnould, Minh; Gaudric, Alain; Chapon, Françoise; Adams, Ralf H.; Dejana, Elisabetta

    2011-01-01

    Cerebral cavernous malformations (CCM) are vascular malformations of the central nervous system (CNS) that lead to cerebral hemorrhages. Familial CCM occurs as an autosomal dominant condition caused by loss-of-function mutations in one of the three CCM genes. Constitutive or tissue-specific ablation of any of the Ccm genes in mice previously established the crucial role of Ccm gene expression in endothelial cells for proper angiogenesis. However, embryonic lethality precluded the development of relevant CCM mouse models. Here, we show that endothelial-specific Ccm2 deletion at postnatal day 1 (P1) in mice results in vascular lesions mimicking human CCM lesions. Consistent with CCM1/3 involvement in the same human disease, deletion of Ccm1/3 at P1 in mice results in similar CCM lesions. The lesions are located in the cerebellum and the retina, two organs undergoing intense postnatal angiogenesis. Despite a pan-endothelial Ccm2 deletion, CCM lesions are restricted to the venous bed. Notably, the consequences of Ccm2 loss depend on the developmental timing of Ccm2 ablation. This work provides a highly penetrant and relevant CCM mouse model. PMID:21859843

  20. Endothelium-derived relaxing factor produced and released from artery and vein is nitric oxide

    SciTech Connect

    Ignarro, L.J.; Buga, G.M.; Wood, K.S.; Byrns, R.E.; Chaudhuri, G.

    1987-12-01

    The objective of this study was to determine whether nitric oxide (NO) is responsible for the vascular smooth muscle relaxation elicited by endothelium-derived relaxing factor (EDRF). EDRF is an unstable humoral substance released from artery and vein that mediates the action of endothelium-dependent vasodilators. NO is and unstable endothelium-independent vasodilator that is released from vasodilator drugs such as nitroprusside and glyceryl trinitrate. The authors have repeatedly observed that the actions of NO on vascular smooth muscle closely resemble those of EDRF. In the present study the vascular effects of EDRF released from perfused bovine intrapulmonary artery and vein were compared with the effects of NO delivered by superfusion over endothelium-denuded arterial and venous strips arranged in a cascade. EDRF was indistinguishable from NO in that both were labile inactivated by pyrogallol or superoxide anion, stabilized by superoxide dismutase, and inhibited by oxyhemoglobin or potassium. Both EDRF and NO produced comparable increases in cyclic GMP accumulation in artery and vein, and this cyclic GMP accumulation was inhibited by pyrogallol, oxyhemoglobin, potassium, and methylene blue. EDRF was identified chemically as NO, or a labile nitroso species, by two procedures. Thus, EDRF released from artery and vein possesses identical and biological and chemical properties as NO.

  1. Retrograde Transvenous Ethanol Embolization of High-flow Peripheral Arteriovenous Malformations

    SciTech Connect

    Linden, Edwin van der; Baalen, Jary M. van; Pattynama, Peter M. T.

    2012-08-15

    Purpose: To report the clinical efficiency and complications in patients treated with retrograde transvenous ethanol embolization of high-flow peripheral arteriovenous malformations (AVMs). Retrograde transvenous ethanol embolization of high-flow AVMs is a technique that can be used to treat AVMs with a dominant outflow vein whenever conventional interventional procedures have proved insufficient. Methods: This is a retrospective study of the clinical effectiveness and complications of retrograde embolization in five patients who had previously undergone multiple arterial embolization procedures without clinical success. Results: Clinical outcomes were good in all patients but were achieved at the cost of serious, although transient, complications in three patients. Conclusion: Retrograde transvenous ethanol embolization is a highly effective therapy for high-flow AVMs. However, because of the high complication rate, it should be reserved as a last resort, to be used after conventional treatment options have failed.

  2. An unusual presentation of arteriovenous malformation as an erosive midfoot lesion.

    PubMed

    Mohammad, Hasan Raza; Bhatti, Waqar; Pillai, Anand

    2016-01-01

    Erosive bony lesions are radiographic findings where localized bone resorption and cortical line breakage occurs. One less common cause of bone erosions is arteriovenous malformations (AVMs). This should always be included in the differentials for foot pain.A 33-year-old gentleman presented with a 5-year history of chronic left foot pain. Clinical examination was unremarkable. Magnetic resonance imaging (MRI) and computerized tomography demonstrated erosive bone changes in the navicular, the intermediate and lateral cuneiforms bones and their corresponding metatarsal bases. An ultrasound and magnetic resonance angiogram demonstrated high signal showing the abnormal communication between metatarsal artery and vein at the site of most pain confirming the AVM. This was subsequently successfully treated with sclerotherapy.Clinicians should be aware of the history, symptoms and signs of AVMs and consider the use of MRI with or without digital subtraction angiography in making a definitive diagnosis. PMID:27605662

  3. Common celiaco-mesenterico-phrenic trunk and renal vascular variations.

    PubMed

    Nayak, Satheesha

    2006-12-01

    The knowledge of vascular variations like other anatomical variations, is important during the operative, diagnostic, and endovascular procedures in abdomen. This report describes multiple variations in the upper abdominal vessels as found during the routine dissection in a 60-year-old male cadaver. The variations found were; presence of a celiaco-mesenterico-phrenic trunk, a common inferior phrenic trunk, 2 right renal arteries originating from abdominal aorta, 2 suprarenal arteries originating from the lower right renal artery, 3 right renal veins opening separately into inferior vena cava, and termination of right testicular vein into the lowest vein among the 3 right renal veins. The existence of a celiaco-mesenterico-phrenic trunk has not been reported yet. Although, other variations reported in this case exist as individual variations, a concomitant variation of them has not been reported yet. The knowledge of such variations is quite useful in planning any upper abdominal surgery. PMID:17143371

  4. Ultrasonographic diagnosis of unusual portal vascular abnormalities in two cats.

    PubMed

    McConnell, J F; Sparkes, A H; Ladlow, J; Doust, R; Davies, S

    2006-06-01

    Two cases of ascites secondary to portal vascular abnormalities associated with portal hypertension are described. In the first case a five-month-old cat was presented with recurrent ascites and investigations showed that the underlying cause was a hepatic arteriovenous fistula. Ultrasonography showed direct communication of the coeliac artery and right branch of the portal vein. There was also hepatofugal flow in the main portal vein consistent with portal hypertension. The ultrasonographic features were similar to those seen in dogs with hepatic arteriovenous fistulae. In the second case, ascites, portal hypertension and an intraluminal mass in the main portal vein was diagnosed in a 16-year-old cat that had been presented with hyperthyroidism and hepatomegaly. Acquired portosystemic collaterals involving the left renal vein were present. Additional diagnostic investigations were not permitted. Ultrasonography was useful in both cases to document portal hypertension and the underlying cause. PMID:16761986

  5. phenoVein-A Tool for Leaf Vein Segmentation and Analysis.

    PubMed

    Bühler, Jonas; Rishmawi, Louai; Pflugfelder, Daniel; Huber, Gregor; Scharr, Hanno; Hülskamp, Martin; Koornneef, Maarten; Schurr, Ulrich; Jahnke, Siegfried

    2015-12-01

    Precise measurements of leaf vein traits are an important aspect of plant phenotyping for ecological and genetic research. Here, we present a powerful and user-friendly image analysis tool named phenoVein. It is dedicated to automated segmenting and analyzing of leaf veins in images acquired with different imaging modalities (microscope, macrophotography, etc.), including options for comfortable manual correction. Advanced image filtering emphasizes veins from the background and compensates for local brightness inhomogeneities. The most important traits being calculated are total vein length, vein density, piecewise vein lengths and widths, areole area, and skeleton graph statistics, like the number of branching or ending points. For the determination of vein widths, a model-based vein edge estimation approach has been implemented. Validation was performed for the measurement of vein length, vein width, and vein density of Arabidopsis (Arabidopsis thaliana), proving the reliability of phenoVein. We demonstrate the power of phenoVein on a set of previously described vein structure mutants of Arabidopsis (hemivenata, ondulata3, and asymmetric leaves2-101) compared with wild-type accessions Columbia-0 and Landsberg erecta-0. phenoVein is freely available as open-source software. PMID:26468519

  6. Surgical Access to Jejunal Veins for Local Thrombolysis and Stent Placement in Portal Vein Thrombosis

    SciTech Connect

    Schellhammer, Frank; Esch, Jan Schulte am; Hammerschlag, Sascha; Knoefel, Wolfram Trudo; Fuerst, Guenter

    2008-07-15

    Portal vein thrombosis is an infrequent entity, which may cause high morbidity and mortality. We report a case of portal vein thrombosis due to benign stenosis following partial pancreatoduodenectomy with segmental replacement of the portal vein by a Gore-tex graft. Using a surgical access to jenunal veins, local thrombolysis, mechanical fragmentation of thrombus, and stent placement were successfully performed.

  7. Prediction of long saphenous vein graft adaptation.

    PubMed

    Davies, A H; Magee, T R; Hayward, J K; Baird, R N; Horrocks, M

    1994-07-01

    The ability of vein to dilate may allow smaller veins to be used for bypass if this change could be predicted. Sixty patients undergoing femorodistal popliteal or infrapopliteal bypass have had their long saphenous vein studied. Diameter measurements of the long saphenous vein have been performed using an ATL Duplex scanner at the groin, mid-thigh and knee. Measurements were performed preoperatively both at rest and with a venous occlusion cuff to dilate the vein and subsequently at 7 days and 3, 6, 9, 12 months after implantation. The mean diameter of the vein at the mid thigh was 4.2 mm non dilated, 5.1 mm with occlusion, 5.4 mm 7 days postoperatively and 5.5 mm at 12 months (p < 0.01 ANOVA). The mean diameter of the vein at the knee was 3.8 mm non-dilated, 4.8 mm with occlusion, 4.8 mm at 7 days and 5.0 mm at 12 months after operation (p < 0.01 ANOVA). If the minimum resting internal diameter of vein regarded as being suitable for bypass was 3 mm, this technique would have increased the vein utilisation rate by 22%. These results show that by using a technique of venous occlusion at the time of preoperative vein mapping the adaptive response of the vein can be predicted and this can result in an increased rate of vein utilisation. PMID:8088400

  8. The current approach to the diagnosis of vascular anomalies of the head and neck: A pictorial essay

    PubMed Central

    Gupta, Swati; Singh, Aarti; Prakash, Anjali; Ghosh, Sujoy; Narang, Poonam; Gupta, Sunita

    2015-01-01

    Throughout the years, various classifications have evolved for the diagnosis of vascular anomalies. However, it remains difficult to classify a number of such lesions. Because all hemangiomas were previously considered to involute, if a lesion with imaging and clinical characteristics of hemangioma does not involute, then there is no subclass in which to classify such a lesion, as reported in one of our cases. The recent classification proposed by the International Society for the Study of Vascular Anomalies (ISSVA, 2014) has solved this problem by including non-involuting and partially involuting hemangioma in the classification. We present here five cases of vascular anomalies and discuss their diagnosis in accordance with the ISSVA (2014) classification. A non-involuting lesion should not always be diagnosed as a vascular malformation. A non-involuting lesion can be either a hemangioma or a vascular malformation depending upon its clinicopathologic and imaging characteristics. PMID:26125008

  9. Effects of preserving different veins on flow-through flap survival: An experimental study

    PubMed Central

    SONG, JIAN; LI, ZONGHUAN; YU, AIXI

    2016-01-01

    Flow-through skin flap grafting is becoming widely used for the reconstruction of skin and soft tissue defects, particularly for patients with poor blood supply around the defect. However, the treatment of the veins remains controversial. In the present study, 5×2-cm skin flaps were created on the left inner thighs of rabbits in order to investigate the effects of various treatments of the veins on the survival of a flow-through skin flap. A femoral artery perforator running through the flap was preserved. Five groups were established in which no veins, one superficial vein (SV), one accompanying vein (AV), one SV plus one AV, or all trunk veins (control) were retained. The percentage of flap area survival was determined on day 10. On days 3, 5, 7 and 9, tissues were harvested from the skin flaps and immunohistochemical analysis was performed in order to count the number of microvessels. Western blot analysis was subsequently completed in order to determine the expression levels of vascular endothelial growth factor (VEGF). The flap areas in which no veins were retained demonstrated significantly reduced survival rates on day 10, as compared with those in the other four groups (P<0.01). Furthermore, flaps with the retention of a SV also demonstrated reduced survival rates, as compared with the AV, AV plus SV and all veins groups (P<0.01); however, there no significant differences were detected between the latter three groups (P>0.05). Immunohistochemical analysis on day 3 detected a greater number of microvessels in the flaps of the control group, as compared with the flaps of the other groups. Furthermore, the AV and AV plus SV groups demonstrated a greater number of microvessels, as compared with the SV and no vein groups, and he no vein group demonstrated the fewest microvessels. No significant differences were found between the AV, AV plus SV and control groups on day 5, 7 and 9. Western blot analysis on day 5 demonstrated that the expression levles of VEGF

  10. Varicose Veins and Venous Insufficiency

    MedlinePlus

    ... catheters Vertebroplasty Women and vascular disease Women's health Social Media Facebook Twitter (SIRspecialists) YouTube RSS Feeds Radiation Safety IR History Bibliographies Meetings and Education Doctor Finder/Patient information Select a State: Advanced ...

  11. Blood vessel classification into arteries and veins in retinal images

    NASA Astrophysics Data System (ADS)

    Kondermann, Claudia; Kondermann, Daniel; Yan, Michelle

    2007-03-01

    The prevalence of diabetes is expected to increase dramatically in coming years; already today it accounts for a major proportion of the health care budget in many countries. Diabetic Retinopathy (DR), a micro vascular complication very often seen in diabetes patients, is the most common cause of visual loss in working age population of developed countries today. Since the possibility of slowing or even stopping the progress of this disease depends on the early detection of DR, an automatic analysis of fundus images would be of great help to the ophthalmologist due to the small size of the symptoms and the large number of patients. An important symptom for DR are abnormally wide veins leading to an unusually low ratio of the average diameter of arteries to veins (AVR). There are also other diseases like high blood pressure or diseases of the pancreas with one symptom being an abnormal AVR value. To determine it, a classification of vessels as arteries or veins is indispensable. As to our knowledge despite the importance there have only been two approaches to vessel classification yet. Therefore we propose an improved method. We compare two feature extraction methods and two classification methods based on support vector machines and neural networks. Given a hand-segmentation of vessels our approach achieves 95.32% correctly classified vessel pixels. This value decreases by 10% on average, if the result of a segmentation algorithm is used as basis for the classification.

  12. Trigeminal neuralgia caused by brain arteriovenous malformations: A case report and literature review

    PubMed Central

    YUAN, YONGJIE; ZHANG, YANDONG; LUO, QI; YU, JINLU

    2016-01-01

    Few cases of trigeminal neuralgia (TGN) induced by brain arteriovenous malformations (bAVMs) have previously been reported. The present case report described one case of TGN caused by bAVMs in a 32-year-old male patient who suffered from recurrent pain in his right cheek for a period of two years, for whom the seizure frequency and duration of pain increased for 6 months. Magnetic resonance imaging was performed, which demonstrated flow-void signals in the abnormal vessels in the right cerebellopontine angle. Subsequent digital subtraction angiography confirmed the diagnosis of bAVMs, and showed the nidus was fed by the right superior cerebellar and the right anterior inferior cerebellar, and drained into the adjacent venous sinuses on the same side. The patient underwent an interventional embolization treatment. TGN was completely relieved following embolization of the majority of the bAVMs. Pain relief may be associated with blocking of the pulsatile compression of the feeding arteries of the bAVMs, the arterialized draining veins or the malformed niduses following embolization, which is similar to the effects induced by microvascular decompression surgery of the trigeminal nerve. In the present case study and review, the underlying mechanism and treatment strategy of TGN caused by bAVMs were discussed in the context of present case, and a literature review was carried out. PMID:27347019

  13. Rapamycin improves TIE2-mutated venous malformation in murine model and human subjects

    PubMed Central

    Boscolo, Elisa; Limaye, Nisha; Huang, Lan; Kang, Kyu-Tae; Soblet, Julie; Uebelhoer, Melanie; Mendola, Antonella; Natynki, Marjut; Seront, Emmanuel; Dupont, Sophie; Hammer, Jennifer; Legrand, Catherine; Brugnara, Carlo; Eklund, Lauri; Vikkula, Miikka; Bischoff, Joyce; Boon, Laurence M.

    2015-01-01

    Venous malformations (VMs) are composed of ectatic veins with scarce smooth muscle cell coverage. Activating mutations in the endothelial cell tyrosine kinase receptor TIE2 are a common cause of these lesions. VMs cause deformity, pain, and local intravascular coagulopathy, and they expand with time. Targeted pharmacological therapies are not available for this condition. Here, we generated a model of VMs by injecting HUVECs expressing the most frequent VM-causing TIE2 mutation, TIE2-L914F, into immune-deficient mice. TIE2-L914F–expressing HUVECs formed VMs with ectatic blood-filled channels that enlarged over time. We tested both rapamycin and a TIE2 tyrosine kinase inhibitor (TIE2-TKI) for their effects on murine VM expansion and for their ability to inhibit mutant TIE2 signaling. Rapamycin prevented VM growth, while TIE2-TKI had no effect. In cultured TIE2-L914F–expressing HUVECs, rapamycin effectively reduced mutant TIE2-induced AKT signaling and, though TIE2-TKI did target the WT receptor, it only weakly suppressed mutant-induced AKT signaling. In a prospective clinical pilot study, we analyzed the effects of rapamycin in 6 patients with difficult–to-treat venous anomalies. Rapamycin reduced pain, bleeding, lesion size, functional and esthetic impairment, and intravascular coagulopathy. This study provides a VM model that allows evaluation of potential therapeutic strategies and demonstrates that rapamycin provides clinical improvement in patients with venous malformation. PMID:26258417

  14. Rapamycin improves TIE2-mutated venous malformation in murine model and human subjects.

    PubMed

    Boscolo, Elisa; Limaye, Nisha; Huang, Lan; Kang, Kyu-Tae; Soblet, Julie; Uebelhoer, Melanie; Mendola, Antonella; Natynki, Marjut; Seront, Emmanuel; Dupont, Sophie; Hammer, Jennifer; Legrand, Catherine; Brugnara, Carlo; Eklund, Lauri; Vikkula, Miikka; Bischoff, Joyce; Boon, Laurence M

    2015-09-01

    Venous malformations (VMs) are composed of ectatic veins with scarce smooth muscle cell coverage. Activating mutations in the endothelial cell tyrosine kinase receptor TIE2 are a common cause of these lesions. VMs cause deformity, pain, and local intravascular coagulopathy, and they expand with time. Targeted pharmacological therapies are not available for this condition. Here, we generated a model of VMs by injecting HUVECs expressing the most frequent VM-causing TIE2 mutation, TIE2-L914F, into immune-deficient mice. TIE2-L914F-expressing HUVECs formed VMs with ectatic blood-filled channels that enlarged over time. We tested both rapamycin and a TIE2 tyrosine kinase inhibitor (TIE2-TKI) for their effects on murine VM expansion and for their ability to inhibit mutant TIE2 signaling. Rapamycin prevented VM growth, while TIE2-TKI had no effect. In cultured TIE2-L914F-expressing HUVECs, rapamycin effectively reduced mutant TIE2-induced AKT signaling and, though TIE2-TKI did target the WT receptor, it only weakly suppressed mutant-induced AKT signaling. In a prospective clinical pilot study, we analyzed the effects of rapamycin in 6 patients with difficult-to-treat venous anomalies. Rapamycin reduced pain, bleeding, lesion size, functional and esthetic impairment, and intravascular coagulopathy. This study provides a VM model that allows evaluation of potential therapeutic strategies and demonstrates that rapamycin provides clinical improvement in patients with venous malformation. PMID:26258417

  15. Dandy-Walker Malformation Presenting with Psychological Manifestations.

    PubMed

    Rohanachandra, Yasodha Maheshi; Dahanayake, Dulangi Maneksha Amerasinghe; Wijetunge, Swarna

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment. PMID:27493822

  16. Dandy-Walker malformation in Ellis-van Creveld syndrome.

    PubMed

    Zangwill, K M; Boal, D K; Ladda, R L

    1988-09-01

    We report on 2 Old Order Amish patients with Ellis-van Creveld (EvC) syndrome and the Dandy-Walker malformation; a similar case is noted in the literature. Pedigree analysis of our patients documents extensive inbreeding in successive generations. Considering the rarity of EvC syndrome and Dandy-Walker malformation as isolated malformations, the appearance of both in our 2 patients plus the patient in the literature suggests that Dandy-Walker malformation may be a manifestation in the EvC syndrome. However, in this isolate the coincidental occurrence of 2 rare recessive traits cannot be excluded. PMID:3223493

  17. Neuro-ophthalmology of type 1 Chiari malformation

    PubMed Central

    Shaikh, Aasef G.; Ghasia, Fatema F.

    2016-01-01

    Chiari malformation is a congenital deformity leading to herniation of cerebellar tonsils. Headache is a typical symptom of this condition, but patients with Chiari malformation often present with double vision and vertigo. Examination of eye movements in such patients often reveals nystagmus and strabismus. Eye movement deficits in the context of typical symptomatic presentation are critical clinical markers for the diagnosis of Chiari malformation. We will review eye movement deficits that seen in patients with type 1 Chiari malformation. We will then discuss the underlying pathophysiology and therapeutic options for such deficits. PMID:26843890

  18. Malformations Among the X-Linked Intellectual Disability Syndromes

    PubMed Central

    Stevenson, Roger E.; Schwartz, Charles E.; Rogers, R. Curtis

    2013-01-01

    Malformations are significant contributions to childhood mortality and disability. Their co-occurrence with intellectual disability may compound the health burden, requiring additional evaluation and management measures. Overall, malformations of greater or lesser severity occur in at least some cases of almost half of the 153 XLID syndromes. Genitourinary abnormalities are most common, but tend to contribute little or no health burden and occur in only a minority of cases of a given XLID syndrome. Some malformations (e.g., lissencephaly, hydranencephaly, long bone deficiency, renal agenesis/dysplasia) are not amenable to medical or surgical intervention; others (e.g., hydrocephaly, facial clefting, cardiac malformations, hypospadias) may be substantially corrected. PMID:24166814

  19. Opercular malformations: clinical and MRI features in 11 children.

    PubMed

    Rolland, Y; Adamsbaum, C; Sellier, N; Robain, O; Ponsot, G; Kalifa, G

    1995-11-01

    Opercular malformations are rare and complex brain malformations for which only very fragmented neuropathological descriptions have been reported. They are related to an abnormal development of both sylvian fissure and frontoparietal operculum. We report a retrospective clinical and MRI study of 11 patients presenting with opercular malformations. A congenital pseudobulbar syndrome was observed in six cases, various motor disorders in seven cases, mental retardation in six cases and epilepsy in four cases. The purpose of this study is to evaluate the main features of opercular malformations in children and to try to characterise this entity on the basis of its clinical features and MRI pattern. PMID:8577527

  20. Dandy-Walker Malformation Presenting with Psychological Manifestations

    PubMed Central

    Dahanayake, Dulangi Maneksha Amerasinghe

    2016-01-01

    Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment. PMID:27493822

  1. Arteries are formed by vein-derived endothelial tip cells

    PubMed Central

    Xu, Cong; Hasan, Sana S.; Schmidt, Inga; Rocha, Susana F.; Pitulescu, Mara E.; Bussmann, Jeroen; Meyen, Dana; Raz, Erez; Adams, Ralf H.; Siekmann, Arndt F.

    2014-01-01

    Tissue vascularization entails the formation of a blood vessel plexus, which remodels into arteries and veins. Here we show, by using time-lapse imaging of zebrafish fin regeneration and genetic lineage tracing of endothelial cells in the mouse retina, that vein-derived endothelial tip cells contribute to emerging arteries. Our movies uncover that arterial-fated tip cells change migration direction and migrate backwards within the expanding vascular plexus. This behaviour critically depends on chemokine receptor cxcr4a function. We show that the relevant Cxcr4a ligand Cxcl12a selectively accumulates in newly forming bone tissue even when ubiquitously overexpressed, pointing towards a tissue-intrinsic mode of chemokine gradient formation. Furthermore, we find that cxcr4a mutant cells can contribute to developing arteries when in association with wild-type cells, suggesting collective migration of endothelial cells. Together, our findings reveal specific cell migratory behaviours in the developing blood vessel plexus and uncover a conserved mode of artery formation. PMID:25502622

  2. Venous Endothelial Marker COUP-TFII Regulates the Distinct Pathologic Potentials of Adult Arteries and Veins

    PubMed Central

    Cui, Xiaofeng; Lu, Yao Wei; Lee, Vivian; Kim, Diana; Dorsey, Taylor; Wang, Qingjie; Lee, Young; Vincent, Peter; Schwarz, John; Dai, Guohao

    2015-01-01

    Arteries and veins have very different susceptibility to certain vascular diseases such as atherosclerosis and vascular calcification. The molecular mechanisms of these differences are not fully understood. In this study, we discovered that COUP-TFII, a transcription factor critical for establishing the venous identity during embryonic vascular development, also regulates the pathophysiological functions of adult blood vessels, especially those directly related to vascular diseases. Specifically, we found that suppression of COUP-TFII in venous ECs switched its phenotype toward pro-atherogenic by up-regulating the expression of inflammatory genes and down-regulating anti-thrombotic genes. ECs with COUP-TFII knockdown also readily undergo endothelial-to-mesenchymal transition (EndoMT) and subsequent osteogenic differentiation with dramatically increased osteogenic transcriptional program and calcium deposition. Consistently, over-expression of COUP-TFII led to the completely opposite effects. In vivo validation of these pro-atherogenic and osteogenic genes also demonstrates a broad consistent differential expression pattern in mouse aorta vs. vena cava ECs, which cannot be explained by the difference in hemodynamic flow. These data reveal phenotypic modulation by different levels of COUP-TFII in arterial and venous ECs, and suggest COUP-TFII may play an important role in the different susceptibilities of arteries and veins to vascular diseases such as atherosclerosis and vascular calcification. PMID:26537113

  3. Role of aflibercept for macular edema following branch retinal vein occlusion: comparison of clinical trials

    PubMed Central

    Oellers, Patrick; Grewal, Dilraj S; Fekrat, Sharon

    2016-01-01

    For years, the standard of care for branch-retinal-vein-occlusion-associated macular edema was initial observation followed by grid-pattern laser photocoagulation for persistent edema. Newer pharmacologic options have revolutionized the management of branch-retinal-vein-occlusion-associated macular edema, and the visual outcomes of these eyes are better than ever. However, a variety of available treatment options including intravitreal corticosteroids and intravitreal anti-vascular endothelial growth factor agents have established novel challenges with regard to appropriate drug selection. This review summarizes the available clinical studies with special emphasis on the comparison of intravitreal aflibercept with ranibizumab, bevacizumab, and steroid agents. PMID:27022238

  4. Hepatocellular carcinoma with extension to the diaphragm, falciform ligament, rectus abdominis and paraumbilical vein

    PubMed Central

    Kaur, R; Abdullah, BJJ; Rajasingam, V

    2008-01-01

    Hepatocellular carcinoma is the most common primary tumour of the liver. The most common extrahepatic metastatic sites are the lung, lymph nodes, bones and adrenal glands. All forms of HCC demonstrate a tendency for vascular invasion, producing extensive intrahepatic metastases and, occasionally, portal vein or inferior vena cava extension with spread into the right atrium in extreme cases. Tumour spread of abdominal diseases via hepatic ligaments has also been previously reported. We report a rare case of hepatocellular carcinoma with extension into the falciform ligament, overlying rectus sheath and adjacent diaphragm with concomitant infiltration into the recanalised paraumbilical vein. PMID:21611019

  5. Embolization of Incompetent Pelvic Veins for the Treatment of Recurrent Varicose Veins in Lower Limbs and Pelvic Congestion Syndrome

    SciTech Connect

    Meneses, Luis Fava, Mario; Diaz, Pia; Andia, Marcelo; Tejos, Cristian; Irarrazabal, Pablo; Uribe, Sergio

    2013-02-15

    We present our experience with embolization of incompetent pelvic veins (IPV) in women with recurrence of varicose veins (VV) in lower limbs, as well as symptoms of pelvic congestion syndrome (PCS), after first surgery. In addition, we evaluated the effects of embolization in decreasing the symptoms of VV before surgery as well as its effects on PCS symptoms. We included 10 women who had consulted a vascular surgeon because of recurrent VV in lower limbs after surgery. All of these patients were included in the study because they also had symptoms of PCS, probably due to IPV. In patients who had confirmed IPV, we performed embolization before a second surgery. VV and PCS were assessed before and at 3 months after embolization (before the second surgery) using a venous clinical severity score (VCSS) and a visual analog pain scale (VAS), respectively. Patients were controlled between 3 and 6 months after embolization. Paired Student t test analysis was used for comparing data before and after embolization. Fifteen vein segments in 10 women were suitable for embolization. There was a significant (p < 0.001) decrease of VCSS after embolization, and recurrence of VV was not detected within a period of 6 months. There was also significant (p < 0.01) relief of chronic pelvic pain related to PCS evaluated using VAS at 3 months after embolization. Embolization decreases the risk of VV recurrence after surgery and also improves PCS symptoms in women with VV in lower limbs and IPV.

  6. Malformations of the tooth root in humans.

    PubMed

    Luder, Hans U

    2015-01-01

    The most common root malformations in humans arise from either developmental disorders of the root alone or disorders of radicular development as part of a general tooth dysplasia. The aim of this review is to relate the characteristics of these root malformations to potentially disrupted processes involved in radicular morphogenesis. Radicular morphogenesis proceeds under the control of Hertwig's epithelial root sheath (HERS) which determines the number, length, and shape of the root, induces the formation of radicular dentin, and participates in the development of root cementum. Formation of HERS at the transition from crown to root development appears to be very insensitive to adverse effects, with the result that rootless teeth are extremely rare. In contrast, shortened roots as a consequence of impaired or prematurely halted apical growth of HERS constitute the most prevalent radicular dysplasia which occurs due to trauma and unknown reasons as well as in association with dentin disorders. While odontoblast differentiation inevitably stops when growth of HERS is arrested, it seems to be unaffected even in cases of severe dentin dysplasias such as regional odontodysplasia and dentin dysplasia type I. As a result radicular dentin formation is at least initiated and progresses for a limited time. The only condition affecting cementogenesis is hypophosphatasia which disrupts the formation of acellular cementum through an inhibition of mineralization. A process particularly susceptible to adverse effects appears to be the formation of the furcation in multirooted teeth. Impairment or disruption of this process entails taurodontism, single-rooted posterior teeth, and misshapen furcations. Thus, even though many characteristics of human root malformations can be related to disorders of specific processes involved in radicular morphogenesis, precise inferences as to the pathogenesis of these dysplasias are hampered by the still limited knowledge on root formation

  7. Malformations of the tooth root in humans

    PubMed Central

    Luder, Hans U.

    2015-01-01

    The most common root malformations in humans arise from either developmental disorders of the root alone or disorders of radicular development as part of a general tooth dysplasia. The aim of this review is to relate the characteristics of these root malformations to potentially disrupted processes involved in radicular morphogenesis. Radicular morphogenesis proceeds under the control of Hertwig's epithelial root sheath (HERS) which determines the number, length, and shape of the root, induces the formation of radicular dentin, and participates in the development of root cementum. Formation of HERS at the transition from crown to root development appears to be very insensitive to adverse effects, with the result that rootless teeth are extremely rare. In contrast, shortened roots as a consequence of impaired or prematurely halted apical growth of HERS constitute the most prevalent radicular dysplasia which occurs due to trauma and unknown reasons as well as in association with dentin disorders. While odontoblast differentiation inevitably stops when growth of HERS is arrested, it seems to be unaffected even in cases of severe dentin dysplasias such as regional odontodysplasia and dentin dysplasia type I. As a result radicular dentin formation is at least initiated and progresses for a limited time. The only condition affecting cementogenesis is hypophosphatasia which disrupts the formation of acellular cementum through an inhibition of mineralization. A process particularly susceptible to adverse effects appears to be the formation of the furcation in multirooted teeth. Impairment or disruption of this process entails taurodontism, single-rooted posterior teeth, and misshapen furcations. Thus, even though many characteristics of human root malformations can be related to disorders of specific processes involved in radicular morphogenesis, precise inferences as to the pathogenesis of these dysplasias are hampered by the still limited knowledge on root formation

  8. Mechanical buckling of veins under internal pressure.

    PubMed

    Martinez, Ricky; Fierro, Cesar A; Shireman, Paula K; Han, Hai-Chao

    2010-04-01

    Venous tortuosity is associated with multiple disease states and is often thought to be a consequence of venous hypertension and chronic venous disease. However, the underlying mechanisms of vein tortuosity are unclear. We hypothesized that increased pressure causes vein buckling that leads to a tortuous appearance. The specific aim of this study was to determine the critical buckling pressure of veins. We determined the buckling pressure of porcine jugular veins and measured the mechanical properties of these veins. Our results showed that the veins buckle when the transmural pressure exceeds a critical pressure that is strongly related to the axial stretch ratio in the veins. The critical pressures of the eight veins tested were 14.2 +/- 5.4 and 26.4 +/- 9.0 mmHg at axial stretch ratio 1.5 and 1.7, respectively. In conclusion, veins buckle into a tortuous shape at high lumen pressures or reduced axial stretch ratios. Our results are useful in understanding the development of venous tortuosity associated with varicose veins, venous valvular insufficiency, diabetic retinopathy, and vein grafts. PMID:20094913

  9. Mechanical Buckling of Veins under Internal Pressure

    PubMed Central

    Martinez, Ricky; Fierro, Cesar A.; Shireman, Paula K.; Han, Hai-Chao

    2010-01-01

    Venous tortuosity is associated with multiple disease states and is often thought to be a consequence of venous hypertension and chronic venous disease. However, the underlying mechanisms of vein tortuosity are unclear. We hypothesized that increased pressure causes vein buckling that leads to a tortuous appearance. The specific aim of this study was to determine the critical buckling pressure of veins. We determined the buckling pressure of porcine jugular veins and measured the mechanical properties of these veins. Our results showed that veins buckle when the transmural pressure exceeds a critical pressure that is strongly related to the axial stretch ratio in the veins. The critical pressures of the eight veins tested were 14.2 ± 5.4 mmHg and 26.4 ± 9.0 mmHg at axial stretch ratio 1.5 and 1.7, respectively. In conclusion, veins buckle into a tortuous shape at high lumen pressures or reduced axial stretch ratios. Our results are useful in understanding the development of venous tortuosity associated with varicose veins, venous valvular insufficiency, diabetic retinopathy and vein grafts. PMID:20094913

  10. Leiomyosarcoma of the renal vein.

    PubMed

    Imao, Tetsuya; Amano, Toshiyasu; Takemae, Katsurou

    2011-02-01

    A 43-year-old woman was referred to our clinic for evaluation of a left retroperitoneal mass. She presented to our internal medicine department complaining of back pain. Computed tomography (CT) scan revealed a left retroperitoneal mass 55 mm in size in the hilum of the left kidney. Enhanced CT scan and magnetic resonance imaging (MRI) disclosed a poorly staining mass. Metaiodobenzylguanidine scintigraphy demonstrated no accumulation in the mass; moreover, endocrinologic examination was normal. Laparoscopic resection of the left retroperitoneal tumor was attempted; however, strong adhesion between the tumor and the left renal vein was encountered. Thus, left nephrectomy after open conversion was performed. Histological findings indicated leiomyosarcoma originating from the left renal vein. The postoperative course has been uneventful; neither recurrence nor metastasis is evident 2 years postsurgery. PMID:20694494

  11. Congenital malformation and maternal occupational exposure to glycol ethers. Occupational Exposure and Congenital Malformations Working Group.

    PubMed

    Cordier, S; Bergeret, A; Goujard, J; Ha, M C; Aymé, S; Bianchi, F; Calzolari, E; De Walle, H E; Knill-Jones, R; Candela, S; Dale, I; Dananché, B; de Vigan, C; Fevotte, J; Kiel, G; Mandereau, L

    1997-07-01

    Glycol ethers are found in a wide range of domestic and industrial products, many of which are used in women's work environments. Motivated by concern about their potential reproductive toxicity, we have evaluated the risk of congenital malformations related to glycol ether exposure during pregnancy as part of a multicenter case-control study, conducted in six regions in Europe. The study comprised 984 cases of major congenital malformations and 1,134 controls matched for place and date of birth. Interviews of the mothers provided information about occupation during pregnancy, sociodemographic variables, and other potential risk factors (medical history, tobacco, alcohol, drugs). A chemist specializing in glycol ethers evaluated exposure during pregnancy, using the job description given by the mother, without knowledge of case or control status. We classified malformations into 22 subgroups. The overall odds ratio (OR) of congenital malformation associated with glycol ether exposure was 1.44 [95% confidence interval (CI) = 1.10-1.90], after adjustment for several potential confounders. The association with exposure to glycol ethers appeared particularly strong in three subgroups: neural tube defects (OR = 1.94; 95% CI = 1.16-3.24), multiple anomalies (OR = 2.00; 95% CI = 1.24-3.23), and cleft lip (OR = 2.03; 95% CI = 1.11-3.73). In this last subgroup, risk, especially of an isolated defect, tended to increase with level of exposure. PMID:9209847

  12. Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature

    PubMed Central

    Rameshbabu, Chittapuram Srinivasan; Gupta, Kanchan Kumar; Qasim, Muhammad; Gupta, Om Prakash

    2015-01-01

    The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with unique vascular anomalies in a 35 year old male patient evaluated initially for nephrolithiasis by ultrasonography, and intravenous pyelography. Further evaluation by multidetector row computed tomography showed bilateral bilobed lungs with hyparterial bronchi, cardiac apex to the left, five branches from left-sided aortic arch with retroesophageal right subclavian artery, interrupted inferior vena cava with azygos continuation, left renal vein continuing as hemiazygos vein and replaced common hepatic artery arising from the superior mesenteric artery. Other vascular anomalies include right internal iliac vein joining the left common iliac vein and precaval course of the single main right renal artery. Anomalies involving abdominal organs include right-sided stomach, midline liver, multiple splenules (polysplenia) in right upper quadrant of abdomen, short truncated pancreas, intestinal malrotation, inversion of superior mesenteric vessels and a preduodenal portal vein. To the best of our knowledge this is the first report of association of left renal vein continuing as hemiazygos vein, precaval right renal artery and anomalous branching pattern of aortic arch with heterotaxy syndrome. PMID:26629295

  13. Heterotaxy Polysplenia Syndrome In An Adult With Unique Vascular Anomalies: Case Report With Review Of Literature.

    PubMed

    Rameshbabu, Chittapuram Srinivasan; Gupta, Kanchan Kumar; Qasim, Muhammad; Gupta, Om Prakash

    2015-07-01

    The pattern of anatomical organization of the thoraco-abdominal visceral and vascular structures which is not the expected normal arrangement, is called as situs ambiguous or heterotaxy syndrome. Patients with heterotaxy syndrome exhibit a wide spectrum of anatomical variations involving thoraco-abdominal structures. We present here an incidental finding of heterotaxy syndrome associated with unique vascular anomalies in a 35 year old male patient evaluated initially for nephrolithiasis by ultrasonography, and intravenous pyelography. Further evaluation by multidetector row computed tomography showed bilateral bilobed lungs with hyparterial bronchi, cardiac apex to the left, five branches from left-sided aortic arch with retroesophageal right subclavian artery, interrupted inferior vena cava with azygos continuation, left renal vein continuing as hemiazygos vein and replaced common hepatic artery arising from the superior mesenteric artery. Other vascular anomalies include right internal iliac vein joining the left common iliac vein and precaval course of the single main right renal artery. Anomalies involving abdominal organs include right-sided stomach, midline liver, multiple splenules (polysplenia) in right upper quadrant of abdomen, short truncated pancreas, intestinal malrotation, inversion of superior mesenteric vessels and a preduodenal portal vein. To the best of our knowledge this is the first report of association of left renal vein continuing as hemiazygos vein, precaval right renal artery and anomalous branching pattern of aortic arch with heterotaxy syndrome. PMID:26629295

  14. Clinical Characteristics and Treatment Options of Infantile Vascular Anomalies

    PubMed Central

    Yang, Bin; Li, Li; Zhang, Li-xin; Sun, Yu-juan; Ma, Lin

    2015-01-01

    Abstract To analyze the clinical characteristics and treatment outcomes of vascular anomalies, and determine which therapy is safe and effective. The data of vascular anomalies pediatric patients who arrived at Beijing children's Hospital from January 2001 to December 2014 were analyzed retrospectively, including the influence of gender, age, clinical manifestation, diagnosis, treatment options, and outcomes. As to infantile hemangiomas, the outcomes of different treatments and their adverse reactions were compared. As to spider angioma and cutaneous capillary malformation, the treatment effect of 595 nm pulsed dye laser (PDL) is analyzed. A total number of 6459 cases of vascular anomalies were reclassified according to the 2014 ISSVA classification system. Among them, the gender ratio is 1:1.69, head-and-neck involved is 53.3%, the onset age within the first month is 72.4%, the age of initial encounter that younger than 6 months is 60.1%. The most common anomalies were infantile hemangiomas (42.6%), congenital hemangiomas (14.1%), and capillary malformations (29.9%). In treating infantile hemangiomas, laser shows the lowest adverse reactions rate significantly. Propranolol shows a higher improvement rate than laser, glucocorticoids, glucocorticoids plus laser, and shows no significant difference with propranolol plus laser both in improvement rate and adverse reactions rate. The total improvement rate of 595 nm PDL is 89.8% in treating spider angioma and 46.7% in treating cutaneous capillary malformation. The improvement rate and excellent rate of laser in treating cutaneous capillary malformation are growing synchronously by increasing the treatment times, and shows no significant difference among different parts of lesion that located in a body. Vascular anomalies possess a female predominance, and are mostly occurred in faces. Definite diagnosis is very important before treatment. In treating infantile hemangioma, propranolol is recommended as the first

  15. [ENDOVENOUS LASER TREATMENT FOR VARICOSE VEINS].

    PubMed

    Tezuka, Masahiro; Kanaoka, Yuji; Ohki, Takao

    2015-05-01

    Varicose veins are a common condition attecting approximately 10 million patients in Japan. The main cause of varicose veins is reflux of the saphenous vein, and conventional treatment for several decades was stripping the affected saphenous vein and phlebectomy. Endovenous laser treatment (EVLT) is a less-invasive treatment method in which the saphenous vein is ablated with a laser under local anesthesia. EVLT has been approved by the Japanese Ministry of Health, Labor and Welfare since 2011, and we have performed EVLT on 5,160 legs with saphenous insufficiency with no severe complications including deep vein thrombosis except for one case of arteriovenous fistula. EVLT appears to be a safe, effective treatment option for varicose veins with saphenous insufficiency. PMID:26281654

  16. [Early stage of a cloverleaf skull malformation].

    PubMed

    Fischer, G; Hori, A; Ulbrich, R; Rath, W

    1982-12-01

    Cloverleaf skull anomaly was diagnosed sonographically and in the fetogram, together with concomitant chondrodystrophy. This resulted in an indication for intentional abortion in the 29th week. Consequently, this rare form of skull monstrosity could be examined pathologico-anatomically for the first time in a very early stage of foetal development. Contrary to the widely held opinion that the reason for such hideous malformation is a hydrocephalus internus due to a deformation of the skull base, we found a practically negligible hydrocephalus, although the cloverleaf skull had already developed in a very marked manner. Hence, this case contradicts the generally adopted formal pathogenetic interpretation of cloverleaf skull monstrosity. PMID:7178767

  17. Spinal Arteriovenous Malformation Masquerating Zoster Sine Herpete

    PubMed Central

    Lee, Ji Young; Ok, Se Jin; Oh, Chang Keun; Park, Sun Kyung; Kim, Do Wan

    2013-01-01

    Zoster sine herpete (ZSH) is difficult to diagnosis during an acute period due to the absence of the characteristic zosteriform dermatomal rash; therefore, progression to postherpetic neuralgia is more common than typical zoster. In addition, misdiagnosis of other neuropathic pain as ZSH is common in clinical situations. Here, we report a case of spinal arteriovenous malformation that mimics ZSH. This is a rare condition; therefore, high clinical suspicion for a correct diagnosis and proper examination are not easy. However, early diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality. PMID:23342212

  18. Role of Embolization for Cerebral Arteriovenous Malformations

    PubMed Central

    Ellis, Jason A.; Lavine, Sean D.

    2014-01-01

    Cerebral arteriovenous malformations (AVMs) are complex high-flow lesions that can result in devastating neurological injury when they hemorrhage. Embolization is a critical component in the management of many patients with cerebral AVMs. Embolization may be used as an independent curative therapy or more commonly in an adjuvant fashion prior to either micro- or radiosurgery. Although the treatment-related morbidity and mortality for AVMs—including that due to microsurgery, embolization, and radiosurgery—can be substantial, its natural history offers little solace. Fortunately, care by a multidisciplinary team experienced in the comprehensive management of AVMs can offer excellent results in most cases. PMID:25624978

  19. Toward postnatal reversal of ocular congenital malformations

    PubMed Central

    Sahel, José-Alain; Marazova, Katia

    2013-01-01

    Aniridia is a panocular disorder that severely affects vision in early life. Most cases are caused by dominantly inherited mutations or deletions of the PAX6 gene, which encodes a transcription factor that is essential for the development of the eye and the central nervous system. In this issue of the JCI, Gregory-Evans and colleagues demonstrate that early postnatal topical administration of an ataluren-based formulation reverses congenital malformations in the postnatal mouse eye, providing evidence that manipulation of PAX6 after birth may lead to corrective tissue remodeling. These findings offer hope that ataluren administration could be a therapeutic paradigm applicable to some major congenital eye defects. PMID:24355915

  20. Microlissencephaly: a heterogeneous malformation of cortical development.

    PubMed

    Barkovich, A J; Ferriero, D M; Barr, R M; Gressens, P; Dobyns, W B; Truwit, C L; Evrard, P

    1998-06-01

    We report the neonatal courses, early postnatal development, and neuroimaging findings of 17 patients with marked microcephaly and simplified cerebral gyral patterns, a condition that we call microlissencephaly. Retrospective analyses of the clinicoradiologic features of these patients allowed segregation of the patients into 5 distinct groups with varying outcomes. The apparent discreteness of these groups suggests multiple etiologies of this malformation, although there appears to be a strong genetic component with probable autosomal recessive inheritance. Utilizing the neonatal course and neuroradiologic features of these infants allows classification of specific subsets, which may be useful to predict outcome. PMID:9706619