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Sample records for cerebral arteriovenous malformation

  1. Gamma knife radiosurgery for cerebral arteriovenous malformations.

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    Kemeny, A A; Radatz, M W R; Rowe, J G; Walton, L; Hampshire, A

    2004-01-01

    Since its introduction, gamma knife radiosurgery has become an important treatment modality for cerebral arteriovenous malformations. This paper is a brief overview of the technique used, of the clinical results achieved and of the experience gained in Sheffield.

  2. Gamma Knife treatment for cerebral arteriovenous malformations.

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    Kemeny, Andras A; Radatz, Matthias W R; Rowe, Jeremy G; Walton, Lee; Vaughan, Paul

    2007-01-01

    One of the earliest indications for Gamma Knife treatment, radiosurgery for cerebral arteriovenous malformations, has stood the test of time. While initially only the ideal cases (small, compact nidus in a non-eloquent site) were chosen, increasingly larger, more complex AVMs were treated. Combination treatment with embolisation and surgery enables most lesions to be treated with success and remarkably low complication rate. This paper is a brief overview of the experience gained in Sheffield.

  3. Unique double recurrence of cerebral arteriovenous malformation.

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    Nagm, Alhusain; Horiuchi, Tetsuyoshi; Ichinose, Shunsuke; Hongo, Kazuhiro

    2015-09-01

    Surgically treated patients with arteriovenous malformations (AVMs) are considered cured when the postoperative angiogram proves complete resection. However, despite no residual nidus or early draining vein on postoperative angiogram, rare instances of AVM recurrence have been reported in adults. In this paper, the authors present a case of a 24-year-old woman with asymptomatic double recurrence of her cerebral AVM after angiographically proven complete resection. To the authors' knowledge, this patient represents the first case with double de novo asymptomatic recurrence of Spetzler-Martin grade I AVM. Also, she represents the first case with unique AVM criteria in each recurrence.

  4. Coexistence of a single cerebral arteriovenous malformation and spinal arteriovenous malformation

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    Wang Yabing

    2009-01-01

    Full Text Available The coexistence of a cerebral and a spinal arteriovenous malformation (AVM together is extremely rare. We present a 31-year-old woman, who suffered from severe root pains in the left upper extremity. Magnetic resonance imaging (MRI revealed the abnormal vessels in the left occipital lobe and upper cervical segment of spinal cord. Cerebral angiography and spinal angiogram revealed two AVMs: One was in the right occipital lobe and the other was located in the C1-C2 segments of cervical cord. She had no other vascular lesions, and nor did her other family members. As the primary problem in her was left upper extremity root pains, which we considered was related to the spinal AVM, the first therapeutic treatment was focused on spinal AVM. The cerebral AVM of the right occipital lobe was surgically resected after part embolization.

  5. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    OpenAIRE

    Andrew Conger; Charles Kulwin; Lawton, Michael T; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Cerebral arteriovenous malformations (AVMs) can be a heterogeneous pathological entity whose management requires a complex decision-making process due to the risks associated with their treatment and natural history. Despite the recently published conclusions of the aborted Randomized Trial of Brain Unruptured AVMs (ARUBA) trial, the authors of this article believe multimodality intervention in general and microsurgical resection in particular continue to play a major role in the ...

  6. Pre- and postoperative changes of regional cortical cerebral blood flow in patients with cerebral arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    石广志; 赵继宗; 王硕; 王永刚; 陆铮

    2003-01-01

    Objective To investigate pre- and postoperative changes of regional cerebral cortical blood flow in patients with cerebral arteriovenous malformation. Method Twenty-two adult patients with arteriovenous malformation(AVM) were recruited into this study at Beijing Tiantan Hospital from September 2001 to May 2002. Eight patients had giant cerebral AVM and the other 14 had a small one. Cortical cerebral blood flow (CBF) was measured by laser Doppler flowmetry (LDF) before and after AVM resections. After surgery, the probe of LDF was implanted adjacent to the area of AVM and monitored for 24 hours.Results CBF increased significantly after the resection in all patients regardless of AVM size. In patients with small AVM, CBF returned to the baseline level within 4 hours, but in patients with giant AVM, CBF remained high even after 24 hours.Conclusions Monitoring CBF is helpful to understand pre- and postoperative changes of regional cortical CBF in patients with cerebral AVM.

  7. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    Science.gov (United States)

    Conger, Andrew; Kulwin, Charles; Lawton, Michael T.; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Cerebral arteriovenous malformations (AVMs) can be a heterogeneous pathological entity whose management requires a complex decision-making process due to the risks associated with their treatment and natural history. Despite the recently published conclusions of the aborted Randomized Trial of Brain Unruptured AVMs (ARUBA) trial, the authors of this article believe multimodality intervention in general and microsurgical resection in particular continue to play a major role in the management of carefully selected ruptured or unruptured AVMs. Methods: The authors provide an overview of their methodology for endovascular intervention and microsurgical resection and share their technical nuances for successful embolization and microsurgical resection of AVMs with special emphasis on complication avoidance. Results: The authors have achieved successful outcomes in embolization and resection of cerebral AVMs when using their methodology. Conclusions: These lesions are among the most technically difficult pathological entities handled by the cerebrovascular specialist, and an overview of technical concepts to help systematize this challenging and variable endeavor can improve the safety of their treatment. PMID:25883831

  8. Surgical strategy for cerebral arteriovenous malformation with acute hemorrhage

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Presently, there have been craniocerebral operation, interventional embolization,stereotactic radiotherapy and other methods in treating cerebral arteriovenous malformation (AVM).However, the standard of different therapeutic regimens of cerebral AVM at the acute stage of hemorrhage has not been completely identified.OBJECTIVE: To observe the clinical characteristics and therapeutic effects of AVM at the acute stage of hemorrhage in patients, and to analyze corresponding therapeutic strategies.DESIGN: Non-randomized clinical observation.SETTING: Department of Neurosurgery, Foshan First People's Hospital, Sun Yat-sen University.PARTICIPANTS: Forty-six patients with cerebral AVM complicated by hemorrhage admitted to Department of Neurosurgery, Foshan First People's Hospital between January 1999 and December 2006,were involved in this study. All the patients were confirmed as cerebral AVM complicated by hemorrhage by brain angiography or/and postoperational pathology. The involved patients, 32 males and 14 females,averaged 25 years old, ranging from 6 to 62 years. Informed consents of therapeutic items were obtained from the relatives of all the patients.METHODS: ①On admission, skull CT and brain angiography were conducted in the involved subjects. ②The therapeutic method was confirmed according to the consciousness, hematoma region, hematoma volume,imageological results following comprehensive analysis: DSA examination was permitted to identify the size and position of abnormal vessel mass, and the distribution of feeding artery and draining vein. Craniocerebral operation was carried out as early as possible in patients with severe or progressive conscious disturbance, in which most of hematoma with obvious occupied effect or cerebral hernia was located in lobe of brain. The primary thing was to clean intracerebral hematoma for in time decompression. According to different situations, corresponding therapeutic measures were used for resecting abnormal

  9. Spontaneous occlusion of cerebral arteriovenous malformation following partial embolization with Onyx.

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    Nas, Omer Fatih; Ozturk, Kerem; Gokalp, Gokhan; Hakyemez, Bahattin

    2017-02-01

    Management options for brain arteriovenous malformations (AVMs) are surgery, radiosurgery, and endovascular embolization. The aim of partial embolization in endovascular treatment is to make total resection possible. However, increased risk of bleeding in partial embolization creates some controversies about treatment options. Spontaneous total occlusion of cerebral AVMs following partial obliteration with embolization agents is a rarely seen condition. We present a case with an AVM vanishing from right posterior cerebral artery which spontaneously occluded following partial embolization with Onyx liquid agent.

  10. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

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    Moraes, Paulo L.; Dias, Rodrigo S.; Weltman, Eduardo; Giordani, Adelmo J.; Benabou, Salomon; Segreto, Helena R. C.; Segreto, Roberto A.

    2015-01-01

    Background: The aim of this study was to evaluate the clinical outcome of patients undergoing single-dose reirradiation using the Linear Accelerator (LINAC) for brain arteriovenous malformations (AVM). Methods: A retrospective study of 37 patients with brain AVM undergoing LINAC reirradiation between April 2003 and November 2011 was carried out. Patient characteristics, for example, gender, age, use of medications, and comorbidities; disease characteristics, for example, Spetzler–Martin grading system, location, volume, modified Pollock–Flickinger score; and treatment characteristics, for example, embolization, prescription dose, radiation dose–volume curves, and conformity index were analyzed. During the follow-up period, imaging studies were performed to evaluate changes after treatment and AVM cure. Complications, such as edema, rupture of the blood–brain barrier, and radionecrosis were classified as symptomatic and asymptomatic. Results: Twenty-seven patients underwent angiogram after reirradiation and the percentage of angiographic occlusion was 55.5%. In three patients without obliteration, AVM shrinkage made it possible to perform surgical resection with a 2/3 cure rate. A reduction in AVM nidus volume greater than 50% after the first procedure was shown to be the most important predictor of obliteration. Another factor associated with AVM cure was a prescription dose higher than 15.5 Gy in the first radiosurgery. Two patients had permanent neurologic deficits. Factors correlated with complications were the prescription dose and maximum dose in the first procedure. Conclusion: This study suggests that single-dose reirradiation is safe and feasible in partially occluded AVM. Reirradiation may not benefit candidates whose prescribed dose was lower than 15.5 Gy in the first procedure and initial AVM nidus volume did not decrease by more than 50% before reirradiation. PMID:26110078

  11. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

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    Paulo L Moraes

    2015-01-01

    Full Text Available Background: The aim of this study was to evaluate the clinical outcome of patients undergoing single-dose reirradiation using the Linear Accelerator (LINAC for brain arteriovenous malformations (AVM. Methods: A retrospective study of 37 patients with brain AVM undergoing LINAC reirradiation between April 2003 and November 2011 was carried out. Patient characteristics, for example, gender, age, use of medications, and comorbidities; disease characteristics, for example, Spetzler-Martin grading system, location, volume, modified Pollock-Flickinger score; and treatment characteristics, for example, embolization, prescription dose, radiation dose-volume curves, and conformity index were analyzed. During the follow-up period, imaging studies were performed to evaluate changes after treatment and AVM cure. Complications, such as edema, rupture of the blood-brain barrier, and radionecrosis were classified as symptomatic and asymptomatic. Results: Twenty-seven patients underwent angiogram after reirradiation and the percentage of angiographic occlusion was 55.5%. In three patients without obliteration, AVM shrinkage made it possible to perform surgical resection with a 2/3 cure rate. A reduction in AVM nidus volume greater than 50% after the first procedure was shown to be the most important predictor of obliteration. Another factor associated with AVM cure was a prescription dose higher than 15.5 Gy in the first radiosurgery. Two patients had permanent neurologic deficits. Factors correlated with complications were the prescription dose and maximum dose in the first procedure. Conclusion: This study suggests that single-dose reirradiation is safe and feasible in partially occluded AVM. Reirradiation may not benefit candidates whose prescribed dose was lower than 15.5 Gy in the first procedure and initial AVM nidus volume did not decrease by more than 50% before reirradiation.

  12. The role of SDF-1/CXCR4 in the vasculogenesis and remodeling of cerebral arteriovenous malformation

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    Wang L

    2015-09-01

    Full Text Available Lingyan Wang,1 Shaolei Guo,2 Nu Zhang,2 Yuqian Tao,3 Heng Zhang,1 Tiewei Qi,2 Feng Liang,2 Zhengsong Huang2 1Department of Neurosurgery ICU, 2Department of Neurosurgery, 3Department of Neurology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, People’s Republic of China Background: Cerebral arteriovenous malformation (AVM involves the vasculogenesis of cerebral blood vessels and can cause severe intracranial hemorrhage. Stromal cell-derived factor-1 (SDF-1 and its receptor, CXCR4, are believed to exert multiple physiological functions including angiogenesis. Thus, we investigated the role of SDF-1/CXCR4 in the vasculogenesis of cerebral AVM.Methods: Brain AVM lesions from surgical resections were analyzed for the expression of SDF-1, CXCR4, VEGF-A, and HIF-1 by using immunohistochemical staining. Flow cytometry was used to quantify the level of circulating endothelial progenitor cells (EPCs. Further, in an animal study, chronic cerebral hypoperfusion model rats were analyzed for the expression of SDF-1 and HIF-1. CXCR4 antagonist, AMD3100, was also used to detect its effects on cerebral vasculogenesis and SDF-1 expression.Results: Large amounts of CXCR4-positive CD45+ cells were found in brain AVM lesion blood vessel walls, which also have higher SDF-1 expression. Cerebral AVM patients also had higher level of EPCs and SDF-1. In chronic cerebral hypoperfusion rats, SDF-1, HIF-1, and CD45 expressions were elevated. The application of AMD3100 effectively suppressed angiogenesis and infiltration of CXCR4-positive CD45+ cells in hypoperfusion rats compared to controls.Conclusion: The SDF-1/CXCR4 axis plays an important role in the vasculogenesis and migration of inflammatory cells in cerebral AVM lesions, possibly via the recruitment of bone marrow EPCs. Keywords: cerebral arteriovenous malformation, SDF-1/CXCR4, chronic cerebral hypoperfusion, endothelial progenitor cells

  13. Moyamoya syndrome associated with γ knife surgery for cerebral arteriovenous malformation: case report.

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    Uozumi, Yoichi; Sumitomo, Masaki; Maruwaka, Mikio; Araki, Yoshio; Izumi, Takashi; Miyachi, Shigeru; Kato, Takenori; Hasegawa, Toshinori; Kida, Yoshihisa; Okamoto, Sho; Wakabayashi, Toshihiko

    2012-01-01

    A 30-year-old female developed moyamoya syndrome after gamma knife surgery (GKS) for cerebral arteriovenous malformation (AVM), and was treated with bypass surgery. She suffered from flittering scotoma, right transient hemianopsia, and headache for 1 year. Cerebral angiography revealed a Spetzler-Martin grade III AVM located in the left occipital lobe. After staged embolization, GKS was performed with a minimum dose of 20 Gy to the periphery of the nidus at the 50% isodose level of the maximum target dose. Gradual nidus regression was achieved, and the clinical symptoms disappeared completely. However, at 30 months after GKS, the patient suffered transient ischemic attack. Cerebral angiography showed left middle cerebral artery occlusion with moyamoya vessels. The patient underwent direct and indirect bypass surgery. After surgery, the patient was free from ischemic symptoms. Chronic inflammation and long-term changes in expression of cytokines and growth factors after GKS may have triggered this case.

  14. The Rare Association of Moyamoya Disease and Cerebral Arteriovenous Malformations: a Case Report

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    Wu, Te Chang [Chi-Mei Foundation Hospital, Tainan (China); Guo, Wan Yuo; Wu, Hsiu Mei; Chang, Feng Chi; Shiau, Cheng Ying; Chung, Wen Yuh [Taipei Veterans General Hospital, Taipei (China)

    2008-07-15

    A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow- up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.

  15. MRI evidence for preserved regulation of intracranial pressure in patients with cerebral arteriovenous malformations

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    Meinel, Felix G.; Fischer, Judith; Pomschar, Andreas; Wöhrle, Natalie; Koerte, Inga K.; Steffinger, Denise [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany); Laubender, Rüdiger P. [Institute of Medical Informatics, Biometry and Epidemiology, Ludwig-Maximilians-University, Marchioninistr. 15, 81377 Munich (Germany); Muacevic, Alexander [European Cyberknife Center Munich, 81377 Munich (Germany); Reiser, Maximilian F. [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany); Alperin, Noam [Department of Radiology, Miller School of Medicine, University of Miami, Miami, FL 33136 (United States); Ertl-Wagner, Birgit, E-mail: birgit.ertl-wagner@med.uni-muenchen.de [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany)

    2014-08-15

    Purpose: The purpose of this study was to investigate intracranial pressure and associated hemo- and hydrodynamic parameters in patients with cerebral arteriovenous malformations AVMs. Methods: Thirty consecutive patients with arteriovenous malformations (median age 38.7 years, 27/30 previously treated with radiosurgery) and 30 age- and gender-matched healthy controls were investigated on a 3.0 T MR scanner. Nidus volume was quantified on dynamic MR angiography. Total arterial cerebral blood flow (tCBF), venous outflow as well as aqueductal and craniospinal stroke volumes were obtained using velocity-encoded cine-phase contrast MRI. Intracranial volume change during the cardiac cycle was calculated and intracranial pressure (ICP) was derived from systolic intracranial volume change (ICVC) and pulse pressure gradient. Results: TCBF was significantly higher in AVM patients as compared to healthy controls (median 799 vs. 692 mL/min, p = 0.007). There was a trend for venous flow to be increased in both the ipsilateral internal jugular vein (IJV, 282 vs. 225 mL/min, p = 0.16), and in the contralateral IJV (322 vs. 285 mL/min, p = 0.09), but not in secondary veins. There was no significant difference in median ICP between AVM patients and control subjects (6.9 vs. 8.6 mmHg, p = 0.30) and ICP did not correlate with nidus volume in AVM patients (ρ = −0.06, p = 0.74). There was a significant positive correlation between tCBF and craniospinal CSF stroke volume (ρ = 0.69, p = 0.02). Conclusions: The elevated cerebral blood flow in patients with AVMs is drained through an increased flow in IJVs but not secondary veins. ICP is maintained within ranges of normal and does not correlate with nidus volume.

  16. Clinical and pathological changes in cerebral arteriovenous malformations after stereotactic radiosurgery failure

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    LIU Wei-ming; YE Xun; ZHAO Yuan-li; WANG Shuo; ZHAO Ji-zong

    2008-01-01

    Background Stereotactic radiosurgery is an alternative to resection of intracraniaI cerebral arteriovenous malformations (AVMs),while it will failin some cases.This study aimed to evaluate the changes after stereotactic radiosurgery for AVMs.Methods Nineteen cases with cerebral AVMs had failure after stereotactic radiosurgery therapy.The symptoms and angiography were assessed.All patients underwent microsurgery.Pathologic examination was performed for all cases and electron microscopic examination was carried out in 6 patients.Reaults Seven cases had hemorrhage from 12 to 98 months after stereotactic radiosurgery,5 had headache.4 had refractory encephalon edema,2 had epilepsy as a new symptom and 1 had a pressure cyst 5 years after radiosurgery.Angiography in 18 cases,8-98 months after radiation therapy,demonstrated no significant changes in 5 cases.slight reduction in 9,near complete obliteration in 1 and complete obliteration in 3.An abnormal vessel was found on pathologic examination in 17 cases,even one case had obliterated in angiography.Electron microscopy examination showed vessel wall weakness,but the vessels remained open and blood circulated.One case died because of a moribund state before surgery.The other 18 cases had no new neurological deficiencies,seizure control and no hemorrhage occurred after microsurgery at an average follow-up of 3 years.Conclusion Stereotactic radiotherapy for AVMs should have a long period follow-up.If serious complications occur,microsurgery can be performed as salvage treatment.

  17. Severe pulmonary oedema following therapeutic embolization with Onyx for cerebral arteriovenous malformation

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    Murugesan, C.; Saravanan, Sundararaj; Rajkumar, John; Prasad, Jagadish; Banakal, Sanjay; Muralidhar, Kanchi [Narayana Hrudayalaya Institute of Medical Sciences, Bangalore (India)

    2008-05-15

    Acute respiratory distress syndrome (ARDS) is characterized by sudden onset of respiratory distress, infiltrates on radiographs consistent with pulmonary oedema, hypoxaemia and increased work in breathing. Infiltrates on radiographs are bilateral, but may be patchy or diffuse and fluffy or dense. It is associated with absence of left heart failure and a PaO{sub 2}/FiO{sub 2} ratio of {<=}200. Ethylene vinyl alcohol copolymer dissolved in dimethyl sulfoxide (DMSO), which was approved by the US FDA in July 2005, is used as an embolic agent for cerebral arteriovenous malformation (AVM). It is a biocompatible liquid polymer that precipitates and solidifies on contact with blood, thus forming a soft and spongy embolus. We report a case of ARDS following therapeutic embolization with ethylene vinyl alcohol copolymer for cerebral AVM under general anaesthesia. Experienced perioperative physicians adopted standard anaesthetic technique and monitoring for this procedure. Acute respiratory distress and hypoxaemia developed in the patient following extubation of the trachea. Infiltrates seen on postprocedural chest radiographs were consistent with pulmonary oedema. DMSO, the solvent for the ethylene vinyl alcohol copolymer, is excreted via the lungs after administration and we postulate that DMSO was the possible cause of ARDS in this patient. Monitoring of haemodynamic parameters (invasive blood pressure, electrocardiography) and ventilatory parameters (ETCO{sub 2}, SpO{sub 2}, airway pressure monitoring) are important in the recognition of this possible event. One should be vigilant and anticipate this complication following therapeutic embolization with ethylene vinyl alcohol polymer for the treatment of cerebral AVM. (orig.)

  18. Using a Machine Learning Approach to Predict Outcomes after Radiosurgery for Cerebral Arteriovenous Malformations.

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    Oermann, Eric Karl; Rubinsteyn, Alex; Ding, Dale; Mascitelli, Justin; Starke, Robert M; Bederson, Joshua B; Kano, Hideyuki; Lunsford, L Dade; Sheehan, Jason P; Hammerbacher, Jeffrey; Kondziolka, Douglas

    2016-01-01

    Predictions of patient outcomes after a given therapy are fundamental to medical practice. We employ a machine learning approach towards predicting the outcomes after stereotactic radiosurgery for cerebral arteriovenous malformations (AVMs). Using three prospective databases, a machine learning approach of feature engineering and model optimization was implemented to create the most accurate predictor of AVM outcomes. Existing prognostic systems were scored for purposes of comparison. The final predictor was secondarily validated on an independent site's dataset not utilized for initial construction. Out of 1,810 patients, 1,674 to 1,291 patients depending upon time threshold, with 23 features were included for analysis and divided into training and validation sets. The best predictor had an average area under the curve (AUC) of 0.71 compared to existing clinical systems of 0.63 across all time points. On the heldout dataset, the predictor had an accuracy of around 0.74 at across all time thresholds with a specificity and sensitivity of 62% and 85% respectively. This machine learning approach was able to provide the best possible predictions of AVM radiosurgery outcomes of any method to date, identify a novel radiobiological feature (3D surface dose), and demonstrate a paradigm for further development of prognostic tools in medical care.

  19. Gene expression signatures in the peripheral blood after radiosurgery of human cerebral arteriovenous malformations

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    Zabel-du Bois, Angelika [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Dept. of RadioOncology, Univ. of Heidelberg (Germany); Wagner-Ecker, Mechthild; Schwager, Christian; Wirkner, Ute; Huber, Peter E. [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Milker-Zabel, Stefanie; Debus, Juergen [Dept. of RadioOncology, Univ. of Heidelberg (Germany); Abdollahi, Amir [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Dept. of RadioOncology, Univ. of Heidelberg (Germany); Center of Cancer Systems Biology, Tufts Univ. School of Medicine, Boston, MA (United States)

    2010-02-15

    Purpose: To unravel biological mechanisms potentially resulting in the obliteration process after radiosurgery (RS) of human cerebral arteriovenous malformations (AVMs) by investigating molecular signatures on the transcriptomic level in peripheral blood of patients. Patients and Methods: Venous blood samples were obtained at definite points of time before and after RS. The samples were tested for radiation-induced changes regarding biological markers (mRNA) using cDNA and oligo-microarray technology. The corresponding expression profiles were correlated with clinical data and obliteration signs in radiologic imaging. Results: The proof of principle that RS outcome can be successfully correlated with transcriptomics of cellular blood components as disease parameter was demonstrated. The authors identified 76 differentially regulated genes (p < 0.001) after RS. Interestingly, in particular genes with known roles in antiangiogenic and procoagulative pathways were identified as potentially relevant. In particularly, the authors found a significant downregulation of neuropilin-2, protein C inhibitor and cyclin-dependent kinase 6. They also found that low pretreatment blood mRNA levels of TLR4 (toll-like receptor 4) and STAT3 (signal transducer and activator of transcription 3) correlated with fast obliteration of AVMs. Conclusion: The authors report on a novel technique for molecular biological analysis of blood from patients with cerebral AVM treated with RS. Differential regulation of genes in peripheral blood was successfully correlated with RS and time to obliteration of AVMs. The identified genes indicate a potential new methodology to monitor RS, which may result in an individualized therapy and optimized follow-up. (orig.)

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

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    Skjøth-Rasmussen, Jane; Roed, Henrik; Ohlhues, Lars;

    2010-01-01

    Primarily, gamma knife centers are predominant in publishing results on arteriovenous malformations (AVM) treatments including reports on risk profile. However, many patients are treated using a linear accelerator-most of these at smaller centers. Because this setting is different from a large...... gamma knife center, the risk profile at Linac departments could be different from the reported experience. Prescribed radiation doses are dependent on AVM volume. This study details results from a medium sized Linac department center focusing on risk profiles....

  1. Arteriovenous Malformation of the Pancreas

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    Alexandros Charalabopoulos

    2011-01-01

    Full Text Available Pancreatic arteriovenous malformation (PAVM is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.

  2. Non-enhanced MR imaging of cerebral arteriovenous malformations at 7 Tesla

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    Wrede, Karsten H.; Dammann, Philipp [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Neurosurgery, Essen (Germany); Johst, Soeren; Maderwald, Stefan [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); Moenninghoff, Christoph; Forsting, Michael [University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); Schlamann, Marc [University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); University Hospital Giessen, Department of Neuroradiology, Giessen (Germany); Sandalcioglu, I.E. [University Hospital Essen, Department of Neurosurgery, Essen (Germany); Nordstadtkrankenhaus Hannover, Department of Neurosurgery, Hannover (Germany); Ladd, Mark E. [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); German Cancer Research Center (DKFZ), Division of Medical Physics in Radiology (E020), Heidelberg (Germany); Sure, Ulrich [University Hospital Essen, Department of Neurosurgery, Essen (Germany); Umutlu, Lale [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany)

    2016-03-15

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

  3. Radiosurgery with a linear accelerator in cerebral arteriovenous malformations Radiocirurgia com acelerador linear em malformações arteriovenosas cerebrais

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    Sérgio Carlos Barros Esteves

    2008-04-01

    Full Text Available OBJECTIVE: To evaluate results achieved with radiosurgery and complications of the procedure when treating arteriovenous malformations with linear accelerator. METHODS: This retrospective study was conducted between October 1993 and December 1996. Sixty-one patients with arteriovenous malformations were treated with radiosurgery utilizing a 6MV energy linear accelerator. Ages of the 32 female and 29 male patients ranged from 6 to 54 years (mean: 28.3 years. The most frequent initial symptom was cephalea (45.9%, followed by neurological deficit (36.1%. Cerebral hemorrhage diagnosed by image was observed in 35 patients (57.3%. Most arteriovenous malformations (67.2% were graded Spetzler III and IV. Venous stenosis (21.3% and aneurysm (13.1% were the most frequent angioarchitecture changes. The dose administered varied from 12 to 27.5Gy in the periphery of the lesion. RESULTS: Out of twenty-eight patients that underwent conclusive angiography control, complete obliteration was achieved in 18 (72% and treatment failed in 7 (absence of occlusion with more than 3 years of follow-up. Four were submitted to a second radiosurgery, and one of these has shown obliteration after 18 months of follow-up. DISCUSSION: Several factors were analyzed regarding the occlusion rate (gender, age, volume, localization, Spetzler, flow, embolization, total of isocenters, prescribed dose and chosen isodose and complications (total of isocenters, localization, volume, maximum dose, prescribed dose and chosen isodose. Analyzed variables showed no statistical significance for obliteration of the vessel, as well as for treatment complications. The largest diameter of the arteriovenous malformation, its volume and the dose administered did not influence time of obliteration. CONCLUSION: Radiosurgery is effective in the treatment of arteriovenous malformations and can be an alternative for patients with clinical contraindication or with lesions in eloquent areas. In the studied

  4. Cerebral hemodynamics and the role of transcranial Doppler applications in the assessment and management of cerebral arteriovenous malformations.

    Science.gov (United States)

    Busch, Kathryn J; Kiat, Hosen; Stephen, Michael; Simons, Mary; Avolio, Alberto; Morgan, Michael Kerin

    2016-08-01

    Dramatic hemodynamic changes occur following resection of brain arteriovenous malformations (AVM). Transcranial Doppler (TCD) records non-invasive velocity and pulsatility parameters. We undertook a systematic review to assess AVM hemodynamics including the time course of changes in velocity and pulsatility in patients undergoing AVM resection. The review employed the Embase and Medline databases. A search strategy was designed. An initial title search for clinical series on AVM and TCD was performed followed by a search for reports on AVM and TCD. A total of 283 publications were selected. Full text analysis produced 54 studies with extractable data regarding AVM, velocity and pulsatility. Two TCD techniques were utilized: conventional "blind" TCD (blind TCD); and transcranial color duplex Doppler (TCCD). Of these, 23 publications reported on blind TCD and seven on TCCD. The presence of high velocity and low pulsatility within AVM feeding arteries preoperatively followed by a postoperative decrease in velocity and subsequent increase in pulsatility of feeding arteries is established. The time sequence of hemodynamic changes following AVM resection using TCD remains uncertain, confounded by variations in methodology and timing of perioperative measurements. Of the two techniques, TCCD reported qualitative aspects including improved differentiation of feeding arteries from draining veins. However, there are a limited number of studies supporting this conclusion. Furthermore, none report reproducible changes with time from treatment. TCCD appears to be a useful technique to analyze the hemodynamic changes occurring following treatment of AVM, however little data is available. This is a field of research that is appropriate to pursue.

  5. Rare malformation of glans penis: arteriovenous malformation.

    Science.gov (United States)

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

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

  6. Occlusion of pulmonary arteriovenous malformations by use of vascular plug

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D

    2007-01-01

    Pulmonary arteriovenous malformations are commonly treated by embolization with coils or balloons to prevent cerebral complications and to raise the oxygenation of the blood. The Amplatzer vascular plug is a new occlusive device made of a self-expanding cylindrical nitinol mesh. It is fast and safe...

  7. Severe pulmonary oedema following therapeutic embolization with Onyx for cerebral arteriovenous malformation.

    Science.gov (United States)

    Murugesan, C; Saravanan, Sundararaj; Rajkumar, John; Prasad, Jagadish; Banakal, Sanjay; Muralidhar, Kanchi

    2008-05-01

    Acute respiratory distress syndrome (ARDS) is characterized by sudden onset of respiratory distress, infiltrates on radiographs consistent with pulmonary oedema, hypoxaemia and increased work in breathing. Infiltrates on radiographs are bilateral, but may be patchy or diffuse and fluffy or dense. It is associated with absence of left heart failure and a PaO2/FiO2 ratio of SpO2, airway pressure monitoring) are important in the recognition of this possible event. One should be vigilant and anticipate this complication following therapeutic embolization with ethylene vinyl alcohol polymer for the treatment of cerebral AVM.

  8. What Is an Arteriovenous Malformation (AVM)?

    Science.gov (United States)

    ... T. Quiz 5 Things to Know About Stroke What Is an Arteriovenous Malformation (AVM)? Updated:Nov 21, ... About AVMs Symptoms and Bleeding Diagnosis and Treatment What is a brain AVM? Normally, arteries carry blood ...

  9. Cerebellar arteriovenous malformations in children

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

    1998-05-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

  10. 儿童脑动静脉畸形出血危险因素分析%Rick Factors for Cerebral Arteriovenous Malformation Hemorrhage in Pediatric Patients

    Institute of Scientific and Technical Information of China (English)

    陈晓霖; 马力; 王书磊; 赵元立

    2015-01-01

    Objective To investigate the clinical and morphological features of cerebral arteriovenous malformation (CAVM) in children and to assess its risk factors of hemorrhage in pediatric patients. Methods The clinical data of pediatric patients admitted to Beijing Tiantan Hospital for CAVMs between 2012 and 2014 were retrospectively analyzed. All patients were diagnosed using digital subtraction angiography. The effects of demographic characteristics and CAVM morphological characteristics on hemorrhage presentation were studied using univariate and multivariate Logistic regression analysis. The characteristics including the gender, age, deep location, malformation diameter, AVM side, venous drainage, associated aneurysms. Results Seventy-three pediatric CAVM cases were identified, 49 (67.1%) cases presented with hemorrhage. The malformation diameter had signiifcant difference on the malformation, (odds ratio [OR] 0.96, 95% confidence interval [CI] 0.93~0.99,P<0.05). The gender, age, AVM side, deep location, deep venous drainage, associated aneurysms, venous drainage did not have significant difference on the malformation. Conclusion Hemorrhagic brain arteriovenous malformations relate with the malformation diameter, and the malformations with small AVM diameter should be treated early to reduce morbidity and mortality.%目的:研究儿童脑动静脉畸形(cerebral arteriovenous malformation,CAVM)临床特点及形态特点,评估其破裂出血的相关因素。方法回顾性分析2012年1月~2014年12月首都医科大学附属北京天坛医院收治年龄小于14岁CAVM患者73例,均经数字减影血管造影(digital subtraction angiography,DSA)检查明确诊断。采用单变量及多变量Logistic回归分析儿童入院时CAVM破裂出血与患者性别、年龄、CAVM侧别、DSA最大径、是否合并动脉瘤、病变是否位于深部、是否纯深静脉引流及静脉引流类型(浅静脉、深静脉及浅静脉合并深静脉)

  11. Arteriovenous malformation within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma

    Directory of Open Access Journals (Sweden)

    Grace Lai

    2015-01-01

    Full Text Available Background: The co-occurrence of intracranial arteriovenous malformations (AVMs and cerebral neoplasms is exceedingly rare but may harbor implications pertaining to the molecular medicine of brain cancer pathogenesis. Case Description: Here, we present a case of de novo AVM within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma (WHO Grade III and review the potential contribution of this mutation to aberrant angiogenesis as an interesting case study in molecular medicine. Conclusion: The co-occurrence of an IDH1 mutated neoplasm and AVM supports the hypothesis that IDH1 mutations may contribute to aberrant angiogenesis and vascular malformation.

  12. Endovascular embolization of pulmonary arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    LIU Feng-yong; WANG Mao-qiang; FAN Qing-sheng; DUAN Feng; WANG Zhi-jun; SONG Peng

    2010-01-01

    had persistent blood oxygen saturation between 85% and 90%. Symptoms of hypoxia never recurred in the other cases. Conclusions Endovascular embolization of pulmonary arteriovenous malformations can significantly improve blood oxygen saturation and reduce serious complications such as cerebral infaction. Therefore, this kind of interventional procedure is a safe and effective treatment method with a low complication rate.

  13. Endothelial Gene Expression and Molecular Changes in Response to Radiosurgery in In Vitro and In Vivo Models of Cerebral Arteriovenous Malformations

    Directory of Open Access Journals (Sweden)

    Jian Tu

    2013-01-01

    Full Text Available Radiosurgery for cerebral arteriovenous malformations (AVMs is limited to 2-year latency. There is no early marker to monitor whether the lesion is responsive to radiosurgery. In this study, we examined endothelial gene expression and molecular changes in response to radiosurgery. Gene expression of E- and P-selectin, ICAM-1, PECAM-1, VCAM-1, tissue factor, and vWF in human cerebral microvascular endothelial cells was quantified by RT-qPCR at different radiation doses and time points. Soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in an animal model of AVMs were quantified by ELISA at different time after radiosurgery. We found that gene expression of E- and P-selectin, ICAM-1, PECAM-1, and VCAM-1 was upregulated by radiation in a dose-dependent manner (P<.05. Gene expression of E- and P-selectin and ICAM-1 was more sensitive to irradiation than that of PECAM-1 and VCAM-1. Radiosurgery induced gene expression of P-selectin, ICAM-1, PECAM-1, and VCAM-1 was linearly correlated with time (P<.05. Radiosurgery induced elevation of soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in a rat model of AVMs (P<.05. Thus, a combination of these molecules measured at different time points may serve as an early predictor of responsiveness of AVMs to radiosurgery.

  14. Endothelial gene expression and molecular changes in response to radiosurgery in in vitro and in vivo models of cerebral arteriovenous malformations.

    Science.gov (United States)

    Tu, Jian; Hu, Zhiqiang; Chen, Zhongbin

    2013-01-01

    Radiosurgery for cerebral arteriovenous malformations (AVMs) is limited to 2-year latency. There is no early marker to monitor whether the lesion is responsive to radiosurgery. In this study, we examined endothelial gene expression and molecular changes in response to radiosurgery. Gene expression of E- and P-selectin, ICAM-1, PECAM-1, VCAM-1, tissue factor, and vWF in human cerebral microvascular endothelial cells was quantified by RT-qPCR at different radiation doses and time points. Soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in an animal model of AVMs were quantified by ELISA at different time after radiosurgery. We found that gene expression of E- and P-selectin, ICAM-1, PECAM-1, and VCAM-1 was upregulated by radiation in a dose-dependent manner (P rat model of AVMs (P < .05). Thus, a combination of these molecules measured at different time points may serve as an early predictor of responsiveness of AVMs to radiosurgery.

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

    Science.gov (United States)

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

    2017-04-01

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

  16. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge;

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  17. Arteriovenous Malformation Detected by Small Bowel Endoscopy

    Directory of Open Access Journals (Sweden)

    Takaaki Fujii

    2014-10-01

    Full Text Available Gastrointestinal bleeding that originates in the small intestine is often difficult to diagnose. When successful diagnosis reveals a lesion that can be localized preoperatively, the laparoscopic approach is an appropriate and beneficial treatment modality for small bowel resection. A 69-year-old man presented with a 6-month history of gastrointestinal bleeding and symptomatic transfusion-dependent anemia. Upper and lower endoscopy were normal. Double-balloon endoscopy established the source of the bleeding as a 0.5-cm polypoid mass appearing as a submucosal tumor with redness and pulsation in the lower ileum, suggesting a vascular lesion. Laparoscopic small bowel resection was successful in removing the mass in the ileum. Histological evaluation of the mass revealed an arteriovenous malformation. Preoperative small bowel endoscopy can be useful for diagnosing the cause and localization of arteriovenous malformation in the small intestine.

  18. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele

    Energy Technology Data Exchange (ETDEWEB)

    Weon, Y.C.; Roh, H.G.; Byun, H.S. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul (Korea); Chung, J.I. [Medimoa Hospital, Department of Radiology, Seoul (Korea); Eoh, W. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea)

    2005-10-01

    Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed. (orig.)

  19. Diagnosis and evaluation of intracranial arteriovenous malformations

    OpenAIRE

    Andrew Conger; Charles Kulwin; Lawton, Michael T; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Ideal management of intracranial arteriovenous malformations (AVMs) remains poorly defined. Decisions regarding management of AVMs are based on the expected natural history of the lesion and risk prediction for peritreatment morbidity. Microsurgical resection, stereotactic radiosurgery, and endovascular embolization alone or in combination are all viable treatment options, each with different risks. The authors attempt to clarify the existing literature′s understanding of the natu...

  20. Postoperative visual loss following cerebral arteriovenous malformation surgery: a case report [v2; ref status: indexed, http://f1000r.es/308

    Directory of Open Access Journals (Sweden)

    Nicolai Goettel

    2014-03-01

    Full Text Available We report the case of a 46 year-old woman presenting with unilateral postoperative visual loss after right frontal craniotomy for resection of an arteriovenous malformation in the supine position. The intraoperative course was uneventful with maintenance of hemodynamic stability. Blood loss was 300 ml; postoperative hemoglobin was 12.4 g/dl. In the recovery room, the patient reported loss of vision in her right eye. Ophthalmologic examination revealed decreased visual acuity, color vision, and visual field. Assessment of the retina was normal, but the patient showed a relative afferent pupillary defect consistent with the clinical diagnosis of ischemic optic neuropathy. Postoperative computer tomogram showed normal perfusion of ophthalmic artery and vein, no hemorrhage or signs of cerebral ischemia or edema. The patient recovered most of her vision 3 months after surgery. Anesthesiologists should be aware that this condition may follow uncomplicated intracranial surgeries in the supine position, and should obtain prompt ophthalmologic consultation when a patient develops postoperative visual loss.

  1. Giant Arteriovenous Malformation of the Neck

    Directory of Open Access Journals (Sweden)

    P. A. Dieng

    2015-01-01

    Full Text Available Arteriovenous malformations (AVM have a wide range of clinical presentations. Operative bleeding is one of the most hazardous complications in the surgical management of high-flow vascular malformations. In the cervical region, the presence of vital vascular structures, such as the carotid artery and jugular vein, may increase this risk. This is a case of massive arteriovenous malformation deforming the neck and the face aspect of this aged lady and growing for several years. A giant mass of the left neck occupied the carotid region and the subclavian region. The AVM was developed between the carotid arteries, jugular veins, and vertebral and subclavian vessels, with arterial and venous flux. The patient underwent surgery twice for the cure of that AVM. The first step was the ligation of the external carotid. Seven days later, the excision of the mass was done. In postoperative period the patient presented a peripheral facial paralysis which completely decreased within 10 days. The first ligation of the external carotid reduces significantly the blood flow into the AVM. It permitted secondarily the complete ablation of the AVM without major bleeding even though multiple ligations were done.

  2. Plasticity of motor function and surgical outcomes in patients with cerebral arteriovenous malformation involving primary motor area:insight from fMRI and DTI

    Institute of Scientific and Technical Information of China (English)

    Lijun Wang; Fuxin Lin; Jun Wu; Yuming Jiao; Yong Cao; Yuanli Zhao; Shuo Wang

    2016-01-01

    Background:Patients who have a cerebral arteriovenous malformation (cAVMs) in the motor cortex can have displaced function. The finding and its relationship to recovery from surgery is not known. Methods:We present the five cases with cAVMs involving precentral knob and/or paracentral lobule and without preoperative motor deficits. We used motor activation areas derived from Functional functional MRI (fMRI) as a region of interesting (ROI) to launch the plasticity of cerebrospinal tracts (CST). All the results were incorporated into the neuronavigation platform for surgical treatment. Intraoperative electric cortical stimulation (ECS) was used to map motor areas. Modified Rankin Scale (mRS) of hands and feets were performed on postoperative day 2, 7 and at month 3, 6 during follow-up period. All the patients suffered from motor deficits regardless of cortical activation patterns. Results:Three patients showed functionally seeded CST in or around the AVM, and were validated by intraoperative electrical stimulation (ECS). Patient 4 had two aberrant functionally seeded fiber tracts away from the lesion, but were proved to be non-functional by postoperative motor deficits. Patient 3 with motor cortex and fiber tract within a diffuse AVMs nidus, complete paralysis of upper extremity after operation and has a persistent motor deficit during 6-month follow-up period. Conclusions:The plasticity of motor cortex on fMRI doesn’t prevent post-operative motor deficits. Functionally mapped fiber tract within or abutting AVM nidus predicts transient and persistent motor deficit.

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

    Directory of Open Access Journals (Sweden)

    Faisal Shamim

    2012-01-01

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

  4. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Pugash, R.A. [Univ. of Toronto, St. Michael' s Hospital, Dept. of Medical Imaging, Toronto, Ontario (Canada)

    2001-04-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized

  5. Advantages of T2 reversed fast spin-echo image and enhanced three-dimensional surface MR angiography for the diagnosis of cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Tanabe, Sumiyoshi; Honmou, Osamu; Minamida, Yoshihiro; Hashi, Kazuo [Sapporo Medical Univ. (Japan). School of Medicine

    2001-09-01

    Although the anatomical investigation of cerebral arteriovenous malformation (AVM) with conventional neuro-imagings considerably supports the preoperative evaluation, it is still hard to dissect the detailed anatomical conformations of AVMs such as location of nidus, identification of feeding arteries or draining veins, and the three-dimensional configuration of nidus in sulci or gyri. In this study, we investigated the efficacy of enhanced three-dimensional surface MR angiography (surface MRA) and T2 reversed image (T2R image) in the diagnosis and surgical planning for cerebral AVMs. The diagnostic accuracy was studied in twelve AVMs: four AVMs closed to motor area, one to Broca area, one to Wernicke area, four in temporal lobe, and two in occipital lobe. Images were obtained with a SIGNA HORIZON LX 1.5T VER 8.2. To construct T2R, the brain is scanned by fast SE method with long TR and was displayed with the reversed gray scale, which seemed similar to T1WI. Surface MRA is a fusion image of MRA and surface image in the workstation. The original data was obtained by enhanced 3D-SPGR method. MRA image was reconstructed with MIP method, and surface image was manipulated with a volume rendering method. T2R images demonstrated seven sulcal AVMs, three gyral AVMs, and two sulco-gyral AVMs; five AVMs located on cortex, four extended to subcortex, and three to paraventricular brain. The images clearly showed six AVMs had hypervascular network such as modja-modja vascular formation. Surface MRA represented nidus adjacent to eloquent area. They were present in central sulcus, precentral sulcus, intraparietal sulcus, inferior frontal sulcus, sylvian fissure, superior temporal sulcus, inferior temporal sulcus, superior temporal gyrus, inferior temporal gyrus, medial temporal gyrus, premotor area and superior frontal sulcus, precuneus and parieto-occipital sulcus. It was easy to identify the point of feeding arteries going down into the sulcus and the junction-point of nidus

  6. Notch receptor expression in human brain arteriovenous malformations.

    Science.gov (United States)

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

    2015-08-01

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

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

    Science.gov (United States)

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

    2003-06-01

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

  8. Spontaneous obliteration highlights the dynamic nature of cerebral arteriovenous malformations: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Su Lone Lim

    2016-01-01

    Conclusion: In our center′s 20-year experience of treatment of cerebral AVMs (approximately 600 cases, this is the only case that has been aborted due to spontaneous obliteration leading us to infer that the incidence of spontaneous AVM obliteration is <1%. Spontaneous obliteration of AVM is a rare but well-established phenomenon that bears testimony to the dynamics of this vascular disorder.

  9. Cerebral malformations without antenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Girard, Nadine J. [Diagnostic Neuroradiology, Hopital Timone, Marseille (France)

    2010-06-15

    Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms), or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital hemiparesis, epilepsy and mental or psychomotor retardation. MRI is the imaging method of choice to assess cerebral malformations. (orig.)

  10. Novel cases of amyotrophic lateral sclerosis after treatment of cerebral arteriovenous malformationss.

    Science.gov (United States)

    Linnebank, Michael; McDougall, Cameron G; Krueger, Stefanie; Biskup, Saskia; Neumann, Manuela; Weller, Michael; Valavanis, Antonios; Prudlo, Johannes

    2016-01-01

    Previous case studies reported nine patients with cerebral arteriovenous malformations (AVM) who developed amyotrophic lateral sclerosis (ALS) after AVM embolisation. Here, we describe three novel cases of ALS which developed 13-34 years after treatment, including embolisation, of cerebral AVM. This study provides further arguments supporting the thesis that embolisation of cerebral AVM might influence the risk of later ALS development.

  11. Kernohan's phenomenon associated with left ruptured occipital arteriovenous malformation.

    Science.gov (United States)

    Fujimoto, A; Sato, H; Katayama, W; Nakai, K; Tsunoda, T; Kobayashi, E; Nose, T

    2004-05-01

    A 23-year-old woman presented with ipsilateral hemiparesia due to rupture of a left occipital arteriovenous malformation (AVM). Emergency decompression (the onset-operation interval was 46 minutes,) was carried out and the patient could leave the hospital upon recovery without neurological deficits. In general, Kernohan's phenomenon is caused by the gradual displacement of the cerebral peduncle against the tentorial edge caused by compression by the contralateral mass. This phenomenon is very rare among the cases with spontaneous intracranial hemorrhage and only three cases including the present one have been reported in the literature. In all cases the onset-operation intervals of were very short. Kernohan's phenomenon associated with a ruptured AVM is a rare condition and emergency decompression is required.

  12. Diagnosis and evaluation of intracranial arteriovenous malformations

    Science.gov (United States)

    Conger, Andrew; Kulwin, Charles; Lawton, Michael T.; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Ideal management of intracranial arteriovenous malformations (AVMs) remains poorly defined. Decisions regarding management of AVMs are based on the expected natural history of the lesion and risk prediction for peritreatment morbidity. Microsurgical resection, stereotactic radiosurgery, and endovascular embolization alone or in combination are all viable treatment options, each with different risks. The authors attempt to clarify the existing literature's understanding of the natural history of intracranial AVMs, and risk-assessment grading scales for each of the three treatment modalities. Methods: The authors conducted a literature review of the existing AVM natural history studies and studies that clarify the utility of existing grading scales available for the assessment of peritreatment risk for all three treatment modalities. Results: The authors systematically outline the diagnosis and evaluation of patients with intracranial AVMs and clarify estimation of the expected natural history and predicted risk of treatment for intracranial AVMs. Conclusion: AVMs are a heterogenous pathology with three different options for treatment. Accurate assessment of risk of observation and risk of treatment is essential for achieving the best outcome for each patient. PMID:25984390

  13. Pulmonary arteriovenous malformation in cryptogenic liver cirrhosis.

    Science.gov (United States)

    Afzal, Noureen; Akhtar, Saleem; Ahmed, Shakeel; Atiq, Mehnaz

    2013-09-01

    The cause in 10 - 20% cases of liver cirrhosis (LC) cannot be elucidated, and are thus termed cryptogenic. Pulmonary arteriovenous malformations (PAVMs) are relatively rare, but the most common anomaly involving the pulmonary tree. Although the rare correlation between LC and PAVM is well-known, there have been no reports of PAVMs occurring in cryptogenic LC. We report a case of PAVM that occurred in cryptogenic liver cirrhosis in a 3 years old male child. The child presented with complaints of malena, hematemesis and variceal bleed. The examination revealed a child with respiratory distress, irritability, tachycardia, clubbing and abdominal distention. He was worked up for recurrent variceal bleeding secondary to portal hypertension but the oxygen saturation during hospital stay kept deteriorating. The diagnosis of hepatopulmonary syndrome as the cause of persistent hypoxemia in the absence of other cardio-pulmonary causes was then made by enhanced echocardiogram using agitated saline. He improved significantly after liver transplantation performed abroad. At a 6 months follow-up, the child was stable with no evidence of intrapulmonary shunting on repeat echo.

  14. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report.

    Science.gov (United States)

    Chen, Kuo-Wei; Kuo, Meng-Fai; Lee, Chung-Wei; Tu, Yong-Kwang

    2015-03-01

    A case of acquired Chiari malformation type I with frontal fistulous arteriovenous malformation (AVM) is presented, and the pathophysiology is discussed. The tonsillar herniation and hydrocephalus both resolved after AVM was excised. This case provides some insight into the complex hemodynamic change exerted by the fistulous AVM and the mechanism of the development of acquired Chiari malformation type I.

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

    Science.gov (United States)

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

    2009-01-01

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

  16. Hypersexuality from resection of left occipital arteriovenous malformation.

    Science.gov (United States)

    Cao, Yong; Zhu, Zhaohui; Wang, Rong; Wang, Shuo; Zhao, Jizong

    2010-01-01

    The authors report their experience on one patient with hypersexuality from resection of left occipital arteriovenous malformation. To the best of our knowledge, this is the first case reported in the literature. A 35-year-old right-handed female farmer suffered a sudden left occipital hemorrhage with subarachnoid and subdural hemorrhages of the left hemisphere. Transient left uncal herniation occurred at the onset and was released by conservative treatment. Digital subtraction angiography showed a brain left occipital arteriovenous malformation. After microsurgical resection of the arteriovenous malformation, the patient developed hypersexual behavior. Positron emission tomography showed hypermetabolism in the left frontal region and left posterior hippocampal gyrus and hypometabolism in the left anterior hippocampal gyrus and the left occipital surgical area. Theories concerning normal pressure perfusion breakthrough and specific areas in the brain responsible for the human sexual response are discussed.

  17. Fistulectomy as a surgical option for pulmonary arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    ZHANG Shao-yan; ZHANG Zhi-tai; OU Song-lei; HU Yan-sheng; SONG Fei-qiang; LI Xin; MA Xu-chen; MA Xin-xin; LIANG Lin; LI Dong; GUO Lin; SUN Zhen

    2009-01-01

    Background Surgical resection remains the treatment of choice for pulmonary arteriovenous malformation but removes some normal lung parenchyma. This study aimed to evaluate the effect and safety of the lung-saving procedure of fistulectomy as an alternative to lung resection.Methods From July 2003 to July 2008, 6 selected patients with pulmonary arteriovenous malformations underwent fistulectomies. Among them, 1 patient underwent emergency operation and 2 underwent bilateral operations. One patient received postoperative embolotherapy.Results No hospital deaths or postoperative morbidity occurred. PaO_2 increased significantly after operation. All patients were free of symptoms and hypoxia during a follow-up for 9 months to 5 years.Conclusions Fistulectomy is a safe and effective procedure for patients with pulmonary arteriovenous malformation and may be an alternative to lung resection.

  18. Arteriovenous Malformation in Temporal Lobe Presenting as Contralateral Ocular Symptoms Mimicking Carotid-Cavernous Fistula

    Directory of Open Access Journals (Sweden)

    Fadzillah Mohd-Tahir

    2013-01-01

    Full Text Available Aim. To report a rare case of arteriovenous malformation in temporal lobe presenting as contralateral orbital symptoms mimicking carotid-cavernous fistula. Method. Interventional case report. Results. A 31-year-old Malay gentleman presented with 2-month history of painful progressive exophthalmos of his left eye associated with recurrent headache, diplopia, and reduced vision. Ocular examination revealed congestive nonpulsating 7 mm exophthalmos of the left eye with no restriction of movements in all direction. There was diplopia in left lateral gaze. Left IOP was elevated at 29 mmHg. Left eye retinal vessels were slightly dilated and tortuous. CT scan was performed and showed right temporal arteriovenous malformation with a nidus of 3.8 cm × 2.5 cm with right middle cerebral artery as feeding artery. There was dilated left superior ophthalmic vein of 0.9 mm in diameter with enlarged left cavernous sinus. MRA and carotid angiogram confirmed right temporal arteriovenous malformation with no carotid-cavernous fistula. Most of the intracranial drainage was via left cavernous sinus. His signs and symptoms dramatically improved following successful embolisation, completely resolved after one year. Conclusion. Intracranial arteriovenous malformation is rarely presented with primary ocular presentation. Early intervention would salvage the eyes and prevent patients from more disaster morbidity or fatality commonly due to intracranial haemorrhage.

  19. Incidence of cerebral edema in patients with cerebral arteriovenous malformations treated by radiosurgery%脑动静脉畸形γ刀放射治疗后脑水肿发生情况分析

    Institute of Scientific and Technical Information of China (English)

    利晞; 梁舜尧; 谭理连; 胡滨; 侯仲军; 陈耀棠; 于晓君; 曹兵艺

    2013-01-01

    目的 探讨脑动静脉畸形γ刀放射治疗后不同随访阶段放射性脑水肿的发生率.方法 回顾性分析广州医学院第二附属医院放射科自1995年1月至2010年12月十余年间内经γ刀治疗的脑动静脉畸形患者的临床资料,共经磁共振检查随访351例次.统计γ刀术后不同随访阶段放射性脑水肿发生率并采用采用x2检验分析. 结果 γ刀放射治疗后本组患者放射性脑水肿阳性率为36.2%;不同随访阶段放射性脑水肿阳性率分别为:≤12月43.0%,13~36月45.3%,37~60月23.1%,61~120月13.6%,>120月5.9%;各随访阶段间放射性脑水肿的发生率比较差异有统计学意义(x2=26.514,P=0.000). 结论 放射性脑水肿是脑动静脉畸形γ刀治疗后最常见的并发症之一,部分患者可出现顽固性放射性脑水肿.γ刀治疗后第一阶段(≤12月)、第二阶段(13~36月)放射性脑水肿发生率较高,应临床密切关注.%Objective To study the incidence of cerebral edema in patients with cerebral arteriovenous malformations (AVMs) treated by Gamma knife.Methods The clinical data of 351 patients with AVMs,admitted to and treated with gamma knife in our hospital from January 1995 to December 2010,were retrospectively analyzed.MR imaging was performed on these patients to review the characteristics of cerebral edema and compare the different incidences of cerebral edema at different follow up periods; chi-square analysis was employed on these data.Results After being treated with gamma knife,the total positive rate of cerebral edema was 36.2%.At different follow-up periods,the positive rates of cerebral edema were different:43.0% within 12 months of treatment,45.3% at 13-36 months after treatment,23.1% at 37-60 months after treatment,13.6% at 61-120 months after treatment,and 5.9% more than 120 months after treatment; significant difference was noted between each two follow up periods (x2=26.514,P=0.000).Conclusion

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

    Energy Technology Data Exchange (ETDEWEB)

    Lv, Ming-ming, E-mail: lvmingming001@163.com [Ninth People' s Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China); Fan, Xin-dong, E-mail: fanxindong@yahoo.com.cn [Ninth People' s Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China); Su, Li-xin, E-mail: sulixin1975@126.com [Ninth People' s Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)

    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.

  1. Prospective comparison of late 3T MRI with conventional angiography in evaluating the patency of cerebral arteriovenous malformations treated with stereotactic radiosurgery

    Energy Technology Data Exchange (ETDEWEB)

    Khandanpour, Nader [National Hospital for Neurology and Neurosurgery, London (United Kingdom); Griffiths, Paul; Hoggard, Nigel [University of Sheffield, Academic Unit of Radiology, C Floor, Royal Hallamshire Hospital, Sheffield (United Kingdom); Warren, Daniel [University of Sheffield, C Floor, Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2013-06-15

    Risk of further haemorrhage in patients suffering from arteriovenous malformation (AVM) would be eliminated only if complete obliteration of the AVM is obtained. Therefore, these patients frequently need long-term follow-up. Conventional catheter angiography (CCA) with a risk of 0.5 %.to 1.6 % of significant neurological complications has traditionally been used for this purpose. However, magnetic resonance imaging (MRI) at 3T may be a safer alternative. The aim of this study was to evaluate if MRI at 3T can accurately evaluate closure of AVM in 2 years after stereotactic radiosurgery. Twenty-three patients with both MRI at 3T and a CCA study were examined. The residual AVMs were evaluated by MRI at 3T against CCA in a prospective study. The time interval between radiosurgery and neuroimaging was on average of 25 months (range, 15-30 months) for MRI study and 33 months (range, 25-46 months) for CCA study. Ten patients showed closure of the AVM on MRI, all of which were confirmed on CCA. There was a complete agreement between late MRI at 3T scan and CCA in evaluation of AVM patency. (orig.)

  2. Capillary malformation--arteriovenous malformation syndrome: review of the literature, proposed diagnostic criteria, and recommendations for management.

    Science.gov (United States)

    Orme, Charisse M; Boyden, Lynn M; Choate, Keith A; Antaya, Richard J; King, Brett A

    2013-01-01

    Capillary malformation-arteriovenous malformation syndrome is an autosomal dominant disorder caused by mutations in the RASA1 gene and characterized by multiple small, round to oval capillary malformations with or without arteriovenous malformations. Ateriovenous malformations occur in up to one-third of patients and may involve the brain and spine. Although making the diagnosis is straightforward in some patients, there are other patients for whom diagnostic criteria may be helpful in their evaluation. Here we review the literature regarding capillary malformation-arteriovenous malformation syndrome, propose diagnostic criteria, and discuss the care of patients with this condition.

  3. Appendicitis Presenting Concurrently with Cecal Arteriovenous Malformation in a Child

    Directory of Open Access Journals (Sweden)

    Sahil P Parikh

    2015-09-01

    Full Text Available Acute appendicitis is a commonly diagnosed surgical problem in the pediatric population. Arterio-venous malformations (AVM of the colonic tract are rarely reported in the pediatric literature. A 13-year old boy who presented with acute appendicitis with concurrent cecal AVM is reported in whom appendectomy was done. Later on radiological investigations AVM was confirmed.

  4. Umbilical arteriovenous malformation in a healthy neonate with umbilical hernia.

    Science.gov (United States)

    Marcelo Boglione, Mariano; D Alessandro, Pablo; Reusmann, Aixa; Rubio, Martín; Lipsich, José; Goldsmith, Gustavo

    2013-01-01

    We describe the case of a neonate with an umbilical hernia and persistent wet umbilicus. Examination revealed a pulsatile umbilical cord with palpable thrill. Doppler ultrasound suspected umbilical arteriovenous malformation and contrast-enhanced computed tomography was performed leading to a definitive diagnosis. Surgery was successfully performed on day 27.

  5. Umbilical Arteriovenous Malformation in a Healthy Neonate with Umbilical Hernia

    OpenAIRE

    2013-01-01

    We describe the case of a neonate with an umbilical hernia and persistent wet umbilicus. Examination revealed a pulsatile umbilical cord with palpable thrill. Doppler ultrasound suspected umbilical arteriovenous malformation and contrast-enhanced computed tomography was performed leading to a definitive diagnosis. Surgery was successfully performed on day 27.

  6. Congenital subclavian arteriovenous malformation causing cardiac failure in an adult.

    Science.gov (United States)

    Anoop, T M; Sreejith, P; Thomas, Joby K; Gailin, B; Jabbar, P K; Ittycheria, Cherian C; George, Raju

    2009-07-01

    Congenital arteriovenous malformations (AVMs) of the thoracic region are rarely reported in adults. The authors report an unusual case of a 30-year-old man who presented with a large congenital AVM and heart failure. The diagnosis was made using transthoracic Doppler echocardiography and computed tomography. Embolization followed by surgical resection of the AVM resulted in the prompt relief of heart failure.

  7. Local Model of Arteriovenous Malformation of the Human Brain

    Science.gov (United States)

    Nadezhda Telegina, Ms; Aleksandr Chupakhin, Mr; Aleksandr Cherevko, Mr

    2013-02-01

    Vascular diseases of the human brain are one of the reasons of deaths and people's incapacitation not only in Russia, but also in the world. The danger of an arteriovenous malformation (AVM) is in premature rupture of pathological vessels of an AVM which may cause haemorrhage. Long-term prognosis without surgical treatment is unfavorable. The reduced impact method of AVM treatment is embolization of a malformation which often results in complete obliteration of an AVM. Pre-surgical mathematical modeling of an arteriovenous malformation can help surgeons with an optimal sequence of the operation. During investigations, the simple mathematical model of arteriovenous malformation is developed and calculated, and stationary and non-stationary processes of its embolization are considered. Various sequences of embolization of a malformation are also considered. Calculations were done with approximate steady flow on the basis of balanced equations derived from conservation laws. Depending on pressure difference, a fistula-type AVM should be embolized at first, and then small racemose AVMs are embolized. Obtained results are in good correspondence with neurosurgical AVM practice.

  8. Spontaneous intracranial hemorrhage in children – ruptured lobar arteriovenous malformations: report of two cases

    Directory of Open Access Journals (Sweden)

    Tascu A.

    2015-03-01

    Full Text Available Brain arteriovenous malformations (AVMs are lesions thought to be primarily congenital in origin, consisting of fistulous connections of abnormal arteries and veins, without normal intervening capillary beds and no cerebral parenchyma between vessels. In the pediatric population, AVMs represent the most common cause of spontaneous intracranial hemorrhage (ICH, with a high recurrent bleeding risk. The aim of this paper is to report 2 cases of ruptured lobar AVMs in children, presenting with spontaneous ICH. Due to the patients’ neurological status, the only imaging examination performed preoperatively was a CT scan, showing intraparenchymal hemorrhage. Thus, there was no MRI/angiographic examination to prove the existence of a brain AVM prior to the surgical interventions. Also, the cerebral angiography performed after the surgery showed, in both patients, no signs of residual vascular malformations. Therefore, the diagnosis of AVM was certified by macroscopic and microscopic pathological findings, with no brain imaging suggestive of a vascular malformation.

  9. An Unusual Case Of Urinary Bladder Arteriovenous Malformation

    LENUS (Irish Health Repository)

    Gnanappiragasam, D

    2016-07-01

    A 45-year-old male presented with haematuria and urinary frequency. Computed Tomography (CT) urogram revealed gross thickening of the left bladder wall. Histology showed large vessels cuffed by eosinophonilic material suggestive of urinary bladder arteriovenous malformation (AVM). No further intervention was carried out as symptoms resolve after the resection. Follow up rigid cystoscopy and CT at 3 months showed resolution of all visible pathology and no evidence of recurrence.

  10. Anesthesia considerations and intraoperative monitoring during surgery for arteriovenous malformations and dural arteriovenous fistulas.

    Science.gov (United States)

    Miller, Christina; Mirski, Marek

    2012-01-01

    The anesthetic considerations for surgical resection of arteriovenous malformations (AVMs) and dural arteriovenous fistulas (DAVFs) incorporate many principles that are common to craniotomies for other indications. However, a high-flow, low-resistance shunt results in chronic hypoperfusion of adjacent brain tissue that is vulnerable to ischemia and at high risk for hyperemia and hemorrhage as resection of the lesion redirects blood flow. A comprehensive understanding of AVM pathophysiology and rapidly titratable anesthetic and vasoactive agents allow the anesthesiologist to alter blood pressure targets as resection evolves for optimal patient outcome. Intensive management is continued post-operatively as the brain acclimatizes to new parameters.

  11. Emergency hepatectomy for hepatic arteriovenous malformation combined with pulmonary hypertension in an infant

    Directory of Open Access Journals (Sweden)

    Naruhiko Murase

    2015-12-01

    Full Text Available Patients with hepatic arteriovenous malformations rarely present with pulmonary hypertension. We report the case of a 3-month-old boy who developed severe pulmonary hypertension due to a hepatic arteriovenous malformation. The use of pulmonary vasodilators to treat the patient's pulmonary hypertension worsened his high-output heart failure. This is the first case in which emergency hepatectomy rescued a patient with hepatic arteriovenous malformations who developed pulmonary hypertension.

  12. Arteriovenous malformations in hereditary haemorrhagic telangiectasia: looking beyond ALK1-NOTCH interactions.

    Science.gov (United States)

    Peacock, Hanna M; Caolo, Vincenza; Jones, Elizabeth A V

    2016-02-01

    Hereditary haemorrhagic telangiectasia (HHT) is characterized by the development of arteriovenous malformations--enlarged shunts allowing arterial flow to bypass capillaries and enter directly into veins. HHT is caused by mutations in ALK1 or Endoglin; however, the majority of arteriovenous malformations are idiopathic and arise spontaneously. Idiopathic arteriovenous malformations differ from those due to loss of ALK1 in terms of both location and disease progression. Furthermore, while arteriovenous malformations in HHT and Alk1 knockout models have decreased NOTCH signalling, some idiopathic arteriovenous malformations have increased NOTCH signalling. The pathogenesis of these lesions also differs, with loss of ALK1 causing expansion of the shunt through proliferation, and NOTCH gain of function inducing initial shunt enlargement by cellular hypertrophy. Hence, we propose that idiopathic arteriovenous malformations are distinct from those of HHT. In this review, we explore the role of ALK1-NOTCH interactions in the development of arteriovenous malformations and examine a possible role of two signalling pathways downstream of ALK1, TMEM100 and IDs, in the development of arteriovenous malformations in HHT. A nuanced understanding of the precise molecular mechanisms underlying idiopathic and HHT-associated arteriovenous malformations will allow for development of targeted treatments for these lesions.

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

    Science.gov (United States)

    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.

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

    LENUS (Irish Health Repository)

    McCarthy, Claire

    2012-01-01

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

  15. Arterio-venous malformations of uterus - diagnostic and management dilemmas.

    Science.gov (United States)

    Narang, H K; Puri, M; Patra, S; Trivedi, S S

    2015-01-01

    Keeping in mind the life-threatening consequences of curettage in cases of undiagnosed uterine arterio-venous malformation (AVM), its possibility should be considered in patients presenting with abnormal heavy uterine bleeding and negative Human Chorionic Gonadotropin (β-hCG) values. We collected a series of cases in which the patients presented with abnormal heavy uterine bleeding, some not responding to conservative treatment. In the presence of declining or low serum β-hCG levels and ultrasound Doppler showing increased vascularity, patients were investigated to detect the possible presence of uterine AVM. In those patients in whom angiographic confirmation of uterine AVM was made, embolisation was done and the outcome was followed. In those patients in whom hysterectomy was done the histopathogy specimen was studied for the possible cause of increased vascularity. Arterio-venous shunting seen on ultrasound does not always imply a uterine AVM and some cases can present diagnostic and management dilemmas.

  16. Value of embolization of brain arteriovenous malformations with ethylene vinyl alcohol copolymer

    Institute of Scientific and Technical Information of China (English)

    YANG Xin-xin; SONG Lu; WU Na; LIU Zhen-guo

    2010-01-01

    @@ T° the Editor We are interested in a recent article "Embolization of brain arteriovenous malformations with ethylene vinyl alcohol copolymer: technical aspects" written by GAO et al.1 Ethylene vinyl alcohol copolymer (Onyx) is a novel liquid embolic material used to cure brain arteriovenous malformations. They performed the embolization on 115 patients and they found that the complications of the treatment are scarce.

  17. Eruption of blood: Arteriovenous malformation of the penile urethra

    Science.gov (United States)

    White, Joshua T.; Baverstock, Richard J.

    2017-01-01

    While arteriovenous malformations (AVMs) are a common congenital or post-traumatic abnormality, male genital AVMs are rare and have been described in the scrotum or penis in pediatric patients.1,2 We describe a 34-year-old male presenting with recurrent spontaneous penile urethral bleeding found to have an AVM of the penile urethra. While angiography has traditionally been helpful, magnetic resonance imaging (MRI) can aid in the diagnosis and characterization of these lesions.3 Each case of male genital AVM provides a unique challenge to manage depending on the presenting complaint, as there are no guidelines to direct treatment.4 PMID:28163810

  18. Classical Triad in Pulmonary Arteriovenous Malformation: Clubbing, Cyanosis and Policytemia

    Directory of Open Access Journals (Sweden)

    Hikmet Tekin Nacaroğlu

    2013-08-01

    Full Text Available Pulmonary arteriovenous malformations (PAVM are generally congenital lesions that results from an abnormal capillary development. Lesions can be presented as an isolated single anomaly, or may be multiple when accompanying with autosomal dominant hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber Syndrome; ROWS. These cases may be asymptomatic, but exertional dyspnea, palpitations and easy fatigability may also be seen. The classic radiological appearance is a round, well-circumscribed lesions. Computed tomography of thorax and angiography are also useful for diagnosis. Herein, we present 2 and 13 years old girls with the diagnosis of PAVM with clubbing and cyanosis. (Journal of Current Pediatrics 2013;11:92-5

  19. Uterine Arteriovenous Malformation with Sudden Heavy Vaginal Hemmorhage

    Directory of Open Access Journals (Sweden)

    Marianne Haughey

    2013-09-01

    Full Text Available Dysfunctional uterine bleeding (DUB is a common presentation in the emergency department and has a wide differential. Most presentations of DUB are in hemodynamically stable patients and can be evaluated as an outpatient. Uterine arteriovenous malformation (AVM is one presentation that can result in a life-threatening medical emergency with unexpected sudden and massive vaginal bleeding. We describe a case of a 24-year-old female with sudden heavy vaginal bleeding requiring a blood transfusion, ultrasound evidence of uterine AVM, and a treatment method of expectant management using an intrauterine device in an attempt to preserve fertility. [West J Emerg Med. 2013;14(5:411-414.

  20. Analysis on Diagnosis Results of Cerebral Arteriovenous Malformation by dual Phase Cerebrovascular Imaging Interventional Treatment%16层螺旋CT双期脑血管成像诊断脑动静脉畸形介入治疗结果分析

    Institute of Scientific and Technical Information of China (English)

    郝绪滨

    2014-01-01

    Objective To study the application value of 16 layer spiral CT dual phase cerebrovascular imaging interventional treatment application value in diagnosis of cerebral arteriovenous malformations,analysis the cerebral imaging results of interventional therapy and scanning characteristics.Methods Selecting 29 cases diagno-sised as cerebral arteriovenous malformation by DSA in our hospital neurosurgery,they were given 16 layer spiral CT dual phase cerebrovascular intervention treatment, analysis the imaging results.Results 16 layer spiral CT scan results suggest that cerebral arteries venous malformations are 28 cases,1 case was missed diagnosis.Conclu-sion 16 layer spiral CT dual phase cerebrovascular imaging interventional treatment has the advantages of noninvasive ,convenient operation,short time consuming,low cost,high resolution,good 3-d imaging effect and so on,which has high diagnosis rate in patients with cerebral arteriovenous malformation ,so it is worthy of populariza-tion and application.%目的:探讨研究16层螺旋CT双期脑血管成像在诊断脑动静脉畸形中的应用价值,分析脑血管成像介入治疗的结果及扫描特征。方法选取我院神经外科经DSA明确诊断为脑动静脉畸形的患者29例,给予16层螺旋CT双期脑血管成像介入治疗,分析所得图像结果。结果16层螺旋CT扫描结果提示,脑动脉静脉畸形者28例,有1例漏诊。结论16层螺旋CT双期脑血管成像介入治疗具有无创、操作简便、耗时短、费用低、分辨率高、三维成像效果好等优点,对脑动静脉畸形患者确诊率高,值得推广应用。

  1. Spontaneous Regression of Uterine Arteriovenous Malformations with Conservative Management

    Science.gov (United States)

    Oishi, Sugiko; Akamine, Kozue; Heshiki, Chiaki

    2017-01-01

    Uterine arteriovenous malformation (AVM) can cause massive hemorrhage and is often treated with uterine artery embolization (UAE), which may lead to ovarian insufficiency. Thus, avoiding UAE should be considered, particularly in women undergoing fertility treatments. We present three women diagnosed with postmiscarriage AVM on color Doppler by transvaginal ultrasound imaging. They had no genital bleeding and a small mass, measuring 16–22 mm. If estradiol was >300 pg/mL when AVM was diagnosed, then a gonadotropin-releasing hormone agonist was administered. All three women underwent follow-up observation, revealing spontaneous mass disappearance. To avoid ovarian insufficiency risk with UAE, conservative management and close follow-up observation should be considered in patients with AVM without bleeding, particularly during the fertility treatment. PMID:28299220

  2. Experimental model of arteriovenous malformation in vitro using biological grafts

    Directory of Open Access Journals (Sweden)

    Sandu Aurelia Mihaela

    2015-06-01

    Full Text Available Introduction: Brain arteriovenous malformations (AVMs represent a serious health problem all around the world. Experimental models help to better understand the pathophysiology of these lesions. Experiment: We performed an experimental model of AVM using biological grafts, arteries and veins harvested from chicken wings at the elbow joint. We used 14 vessels and we performed 20 end-to-end anastomoses to create a nidus with a single feeding artery and a single draining vein. The system was irrigated with colored solution. The experiment was done according with law in force regarding experimental research activity. Conclusions: Experimental models allow us to understand the hemodynamics and predict the outcome of brain AVMs in humans. This experimental model is a useful tool in understanding the hemodynamic properties of brain AVMs. It is very useful in vascular anastomosis training

  3. Partially trombosed glomus type spinal arteriovenous malformation – case presentation

    Directory of Open Access Journals (Sweden)

    Gorgan M.R.

    2014-12-01

    Full Text Available Spinal AVM are very rare vascular lesions and most o the studies give reports on only a few cases. Given their localization between the spinal tracts and the gray matter core of the medulla and their multiple feeders from posterior and anterior spinal arteries they are amongst the most difficult surgical pathology in the nervous sistem. We present the case of a 60 years old male with a glomus type T10-L2 spinal AVM that presented with motor deficit in the lower limbs and urinary incontinence. The partially thrombosed lesion was completely resected without previous embolisation, and the patient was walking with assistance at discharge. This is a rare case that presented with progressive neurological deterioration consistent with an ischemic rather than a hemorrhagic event. Microsurgery is a better option than conservative treatment in these rare cases of spontaneous occlusion of intramedullary arteriovenous malformations.

  4. Intestinal arteriovenous malformation involving the descending colon: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Jin; Park, Young Chan; Lee, Young Hwan; Jung, Kyung Jae; Kim, Ho Kyun [Catholic University of Daegu, Daegu (Korea, Republic of)

    2007-08-15

    Arteriovenous malformations (AVMs) comprising a feeding artery, nidus, and draining vein rarely develop in the gastrointestinal tract. Although almost all AVMs are asymptomatic, they cause massive painless rectal bleeding and subsequent chronic anemia. The definitive diagnosis of AVM is achieved by selective mesenteric angiography, and surgical resection is the treatment of choice. We detected an intestinal AVM involving the descending colon in a patient with severe hematochezia. The diagnosis was made by CT angiography performed using a 64-channel MDCT and the obtained 3D reconstruction images. The AVM showed an extensive vascular network on CT images, and it was treated by surgical resection. Here, we report this case of an intestinal AVM along with its imaging findings.

  5. Cerebral malformations in Carpenter syndrome.

    Science.gov (United States)

    Taravath, S; Tonsgard, J H

    1993-01-01

    The inherited forms of craniosynostosis can be divided into 4 groups: isolated craniosynostosis, craniosynostosis with syndactyly, craniosynostosis with polydactyly and syndactyly, and craniosynostosis with other somatic abnormalities. Acrocephalopolysyndactyly or Carpenter syndrome consists of craniosynostosis, short fingers, soft tissue syndactyly, preaxial polydactyly, congenital heart disease, hypogenitalism, obesity, and umbilical hernia. As many as three-fourths of the patients have some degree of intellectual impairment. The etiology of mental retardation in this syndrome has not been explored. A patient is reported with the features of Carpenter syndrome who has profound developmental delay and cerebral malformations demonstrated by magnetic resonance imaging and computed tomography. Because mental retardation is not an invariable feature of this syndrome or other craniosynostosis syndromes, neuroradiologic examination may help in predicting the intellectual outcome in these patients.

  6. Gamma knife radiosurgery for arteriovenous malformations located in eloquent regions of the brain

    Directory of Open Access Journals (Sweden)

    Javalkar Vijayakumar

    2009-12-01

    Full Text Available Background : Stereotactic radiosurgery is an effective treatment strategy for selected group of patients with cerebral arteriovenous malformations (AVMs. Aim : The aim of this study was to evaluate the obliteration rates, complications, and patient outcomes after Gamma knife radiosurgery for cerebral arteriovenous malformations (AVMs located in eloquent regions of the brain with an emphasis on neurological morbidity. Materials and Methods : Between 2000 and December 2005, 37 patients with AVMs in eloquent locations (sensory, motor, speech, visual cortex, basal ganglia, and brain stem underwent stereotactic radiosurgery. We retrospectively reviewed the clinical data of these patients to asses the outcomes. Of the 37 patients, only two patients had prior embolization. Three underwent prospective staged volume radiosurgery. Two patients needed redo-radiosurgery for residual AVM. Mean target volume was 9.1 cc. Three lesions had nidus volume more than 20 cc. Average marginal dose was 18.75 Gy. The median duration of follow-up was 23 months (range, 6-60 months. 15 patients had follow-up of more than 36 months. Results : A total of 15 patients had follow-up of more than 36 months, thus available for evaluation of angiographic obliteration rates. Complete angiographic obliteration was documented in seven patients (46.7%. Four patients experienced hemorrhage during the latency period. One patient who had subsequent hemorrhage on follow-up developed worsening of neurological deficit. One patient developed significant sensory symptoms which resolved after steroids. No additional clinical deterioration related to treatment was noted in rest of the patients. Conclusions : AVMs located in eloquent and in deep locations can be treated safely with stereotactic radiosurgery with acceptable obliteration rates and minimal morbidity.

  7. Arterial desaturation due to pulmonary arteriovenous malformations after the Kawashima Operation

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2016-01-01

    Full Text Available Arterial desaturation may occur after the Kawashima procedure and, in the absence of venovenous collaterals is usually due to pulmonary arteriovenous malformations. Stenting of the pulmonary arteries, oxygen therapy, and pulmonary vasodilators such as sildenafil have not been able to resolve the arterial desaturation and the only way to do this has been Fontan completion. The time course of the formation of these malformations after the Kawashima and the progression of cyanosis and its resolution after the Fontan has only been demonstrated in case reports and small case series. We pool the available data to model arterial saturations in patients with pulmonary arteriovenous malformations after the Kawashima procedure.

  8. Intracranial arteriovenous malformation and dural arteriovenous fistula embedded in a meningioma—case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Moujahed Labidi

    2015-12-01

    Full Text Available The association between a vascular malformation and a meningioma is a rare occurrence intracranially. We document the case of a 59-year-old man who presented with a right parietal extra-axial mass with headaches and seizures. Hemangiopericytoma was initially suspected on the basis of an unusual vascular pattern and rapid lesion progression. Angiography revealed abnormal vessels and an early draining vein associated with the mass. Arterial feeders were primarily from the middle cerebral artery with discrete contribution from both middle meningeal arteries. Craniotomy and Simpson 0 resection of the lesion were undertaken and revealed the coexistence of a dural based tumor with an AVM and a dural arteriovenous fistula (dAVF. Histology and immunohistochemistry were consistent with the diagnosis of meningioma and associated AVM. This case represents the eleventh report of an AVM associated with a meningioma, among which only 6 were contiguous. Such cases illustrate the pathogenic roles of angiogenesis and inflammation that is common to AVMs, dAVF and meningiomas.

  9. Stereotactic radiosurgery for intracranial arteriovenous malformations: A review

    Directory of Open Access Journals (Sweden)

    Ranjith K Moorthy

    2015-01-01

    Full Text Available Stereotactic radiosurgery (SRS has proven to be an effective strategy in the management of intracranial arteriovenous malformations (AVMs in children and adults over the past three decades. Its application has resulted in lowering the morbidity and mortality associated with treatment of deep-seated AVMs. SRS has been used as a primary modality of therapy as well as in conjunction with embolization and microsurgery in the management of AVMs. The obliteration rate after SRS has been reported to range from 35% to 92%. Smaller AVMs receiving higher marginal doses have obliteration rates of 70% and more. The median follow-up reported in most series is approximately 36–40 months. The median time to obliteration has been reported to be approximately 24–36 months in most series. Radiation-induced neurological complications have been reported in less than 10% of patients, with a 1.5%–6% risk of developing a new permanent neurological deficit. The bleeding rate during the latency to obliteration has been reported to be approximately 5%. This review describes the experience reported in literature with respect to the indications, dosage, factors affecting obliteration rate of AVMs, and complications after SRS.

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

    Energy Technology Data Exchange (ETDEWEB)

    Su, Li-xin, E-mail: sulixin1975@126.com [Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial & Head and Neck Oncology, Ninth People’s Hospital (China); Jia, Ren-Bing, E-mail: jrb19760517@hotmail.com [Shanghai Jiao Tong University School of Medicine, Department of Ophthalmology, Ninth People’s Hospital (China); Wang, De-Ming, E-mail: wdmdeming@hotmail.com; Lv, Ming-Ming, E-mail: lvmingming001@163.com; Fan, Xin-dong, E-mail: fanxindong@aliyun.com [Shanghai Jiao Tong University School of Medicine, Department of Radiology, Ninth People’s Hospital (China)

    2015-06-15

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

  11. Prevalence of pulmonary arteriovenous malformations (PAVMs) and occurrence of neurological symptoms in patients with hereditary haemorrhagic telangiectasia (HHT)

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Oxhøj, H; Andersen, P E;

    2000-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms.......Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease. HHT is characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs) and neurological symptoms....

  12. Radiologic diagnosis and treatment of iatrogenic acquired uterine arteriovenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Kwon, Jung Hyeok [College of Medicine, Keimyung Univ., Taegu (Korea, Republic of)

    2002-05-01

    To analyze gray-scale US, color and duplex Doppler US, and angiographic findings in patients with acquired uterine arteriovenous malformations (AVMs), and to evaluate the usefulness of these modalities in the diagnosis of this disease and the effect of transcatheter arterial embolization in its treatment. During a recent seven-year period, we diagnosed 21 cases of acquired uterine AVM. Nineteen of these patients had a history of causative D and C (between one and seven D and C procedures per patient), one had a history of causative cesarean section, and one had cervical conization. All patients underwent transabdominal and endovaginal gray-scale, color Doppler, and duplex Doppler US and angiography, with therapeutic embolization of bilateral uterine arteries. The majority underwent follow-up Doppler US after embolization. The gray-scale US morphology of uterine AVMs included subtle myometrial inhomogeneity and multiple distinct, small anechoic spaces in the thickened myometrium or endometrium. Color Doppler US showed a tangle of tortuous vessels with multidirectional, high-velocity arterial flow, which was focally or asymmetrically distributed. Duplex Doppler US depicted a waveform of fast arterial flow with low resistance, while angiography demonstrated a complex tangle of vessels supplied by enlarged uterine arteries, in association with early venous drainage during the arterial phase, and staiss of contrast medium within abnormal vasculature. Where AVMs were combined with a pseudoaneurysm, this finding was observed. Transcatheter arterial embolization provided a complete cure, without recurrence. Color and duplex Doppler US in an appropriate modality for the detection and diagnosis of uterine AVMs and for follow-up after embolization. Transcatheter arterial embolization is a safe and effective method of treating this disease.

  13. Gamma knife radiosurgery for arteriovenous malformation in children

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Takayuki; Kobayashi, Tatsuya [Komaki City Hospital, Aichi (Japan)

    1995-04-01

    Intracranial arteriovenous malformations (AVM) of 38 children were treated by gamma knife radiosurgery. Their clinical courses and the effect of the treatment in 19 patients who had angiographies are described. There were 21 boys and 17 girls with a mean age of 11.2 years. The initial signs and symptoms were: intracranial hemorrhage in 32, seizures in 4, and headache in 2. Prior to the radiosurgery, craniotomy was performed in 10 patients, ventricular drainage, in 8, ventriculoperitoneal shunting in 4, and intravascular embolization in 3. The AVM was in the parietal lobe in 8 patients, in the thalamus in 7, in the occipital lobe in 5, in the temporal lobe in 4, in the basal ganglia in 4, in the corpus callosum in 3, and in other locations in 8. The mean diameter of the nidus was 18.9 mm. According to Spetzler`s classification of AVM, 23 (60.5%) were grade III, 10 were grade II, 3 were grade IV, and 2 were grade VI. The mean maximum dose was 36.3 Gy, and the mean peripheral dose was 20.2 Gy. Follow-up angiography was done in 19 patients during a mean follow-up period of 14.9 months. Complete occlusion of the nidus was obtained within 1 year in 10 of 15 patients (67%). All 6 patients who underwent angiography 2 years after treatment showed complete obliteration of the AVM. Only 1 patient developed hemiparesis due to radiation necrosis. It is considered that occlusion of AVMs by gamma knife radiosurgery is probably more effective and safer in children than in adults. (author).

  14. Selection of the methods used in MR angiography with arteriovenous malformation, carotid cavernous fistula, moyamoya disease, and cerebral aneurysms; A comparison of 3-D phase-contrast MRA with 3-D time-of-flight MRA

    Energy Technology Data Exchange (ETDEWEB)

    Ikawa, Fusao; Uozumi, Tohru; Kuwabara, Satoshi (Hiroshima Univ. (Japan). School of Medicine) (and others)

    1993-02-01

    The methods used in MR angiography (MRA) can be roughly divided into two groups: time-of-flight methods (TOF methods) which make use of the influx effect of the flow, and the phase-contrast method of Dumoulin, Souza, and their collaborators (PC method), which utilizes phase differences induced by the flow. In this study, the selection of the MRA method was determined by a comparison of the three-dimensional (3-D) PC and the 3-D TOF method in normal and clinical cases. The tool used was SIGNA Advantage (1.5T) made by GE. The 3-D PC method was applied under a pulse sequence generated by gradient-recalled acquisition in the steady state (GRASS); the pulse sequence used for the 3-D TOF method was spoiled GRASS (SPGR) in a total of 28 cases. The following conclusions were reached: the TOF method should be used when aneurysms, arteriovenous malformations (AVM), and moyamoya disease have been diagnosed, and the PC method should be used when AVM and carotid cavernous fistulae (CCF) are to be evaluated in the flow and when CCFs have been diagnosed. (author).

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

    DEFF Research Database (Denmark)

    Khan, Sabrina; Amin, Faisal Mohammad; Hauerberg, John

    2016-01-01

    BACKGROUND: Though endovascular therapy (EVT) is increasingly applied in the treatment of intracranial vascular lesions, little is known about the effect of EVT on post-procedure headache. We aimed to investigate the prevalence of headache in patients who have undergone EVT for cerebral arteriove......BACKGROUND: Though endovascular therapy (EVT) is increasingly applied in the treatment of intracranial vascular lesions, little is known about the effect of EVT on post-procedure headache. We aimed to investigate the prevalence of headache in patients who have undergone EVT for cerebral...... arteriovenous malformations (AVMs) and aneurysms. METHODS: A total of 324 patients underwent EVT treatment for aneurysms and AVMs at the Danish National Hospital from January 2012 to December 2014. We applied strict exclusion criteria in order to minimize the effect of other factors on headache occurrence, e.......g., craniotomy. Eligible subjects were phone-interviewed using a purpose-developed semi-structured questionnaire. Headaches were classified according to ICHD-III beta criteria. RESULTS: The 59 patients underwent treatment of aneurysms (n = 43), cranial dural fistulas (n = 11), and AVMs (n = 5...

  16. 荧光造影和B超在脑浅表动静脉畸形手术中的联合应用%Intraoperative integration of B-ultrasound and indocyanine green angiography in the surgery of superficial cerebral arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    王硕; 赵洪洋; 刘泠; 赵元立

    2011-01-01

    Objective To evaluate the surgical effect of intraoperative combined use of B-ultrasound and indocyanine green angiography (ICGA) in the surgery of superficial cerebral arteriovenous malformations (AVMs). Methods Sixteen patients with superficial cerebral AVMs,admitted to our hospital from January 2009 to December 2009, were performed surgery with the help of intraoperative B-ultrasound and ICGA; the clinical data of these patients were retrospectively collected.The ability of intraoperative B-ultrasound and ICGA in distinguishing AVMs' vessels (draining veins,feeding and nidal arteries) from each other and from normal vessel was both evaluated.Results The combination of B-ultrasound and ICGA allowed accurate and real-time localization of the AVMs' nidus,helped to define the boundary and identify the draining veins and feeding arteries. Total lesion removal was achieved by 16 successful surgeries in 16 patients, which was confirmed by postoperative DSA.Conclusion The combination of B-ultrasound and ICGA in the surgery of superficial cerebral arteriovenous malformations allows accurate and real-time localization of the AVMs' indus and helps to assess the completeness of lesion removal, indicating its value in the surgery of superficial cerebral AVMs.%目的 探讨B超和吲哚菁绿血管造影(ICGA)在脑浅表动静脉畸形(AVMs)手术中的应用价值.方法 回顾性分析自2009年1月至12月北京天坛医院神经外科血管组联合应用B超和ICGA辅助切除的16例脑浅表AVMs患者临床资料,同时分析2种术中辅助技术对AVMs定位、边界确定及血管类型鉴别的作用.结果 术中联合应用ICGA和B超能有效帮助定位AVMs,确定其边界,帮助辨认供血动脉和引流静脉.16例脑浅表AVMs患者共行开颅手术16次,均全切病灶,手术后经DSA证实AVMs无残留.结论 脑浅表AVMs手术中联合应用ICGA和B超能有效帮助准确切除病灶,判断有无畸形残留,具有较高的临床应用价值.

  17. Increased expression of osteopontin in brain arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    XU Hong-zhi; QIN Zhi-yong; GU Yu-xiang; ZHOU Ping; XU Feng; CHEN Xian-cheng

    2012-01-01

    Background The precise mechanisms responsible for the development and growth of intracranial arteriovenous malformations (AVMs) remain unclear.Osteopontin (OPN) is a phosphorylated glycoprotein with diverse functions.This study aimed to analyze the expression of OPN in human brain AVMs.Methods The AVM nidus was surgically obtained from patients with AVM,whereas control brain artery specimens were surgically obtained from patients with epilepsy.Reverse transcription-polymerase chain reaction (RT-PCR) was used to examine the expression of OPN mRNA in biopsy specimens.OPN protein expression was localized by immunohistochemistry.The statistical differences between different groups were assessed by two-way analysis of variance (ANOVA).Results We analyzed 36 brain AVM specimens and 8 control brain artery specimens.Eleven patients with brain AVM received embolization treatment,and five underwent gamma knife radiotherapy before resection.Nineteen patients with brain AVM had a history of hemorrhage from AVMs.The expression of OPN mRNA was significantly higher in AVMs than that in the control specimens (25.76±2.71 vs.21.46±2.01,P <0.01).There was no statistically significant difference in the extent of OPN mRNA expression between the AVM group with and that without history of hemorrhage (26.13±2.45 vs.25.34±2.99) or gamma knife radiotherapy (24.39±2.10 vs.24.53±1.85).However,the difference between the AVM group with and that without embolization treatment history was statistically significant (24.39±2.10 vs.28.80±1.13,P <0.01).In the group with gamma knife radiotherapy history,OPN expression was found in arteries with early-stage radio-effect.Conclusions OPN may contribute to the vascular instability of brain AVMs.It may play an important role in the pathophysiological process related to embolization treatment.

  18. 多层螺旋CT血管成像在脑动静脉畸形诊断及治疗中的价值%Value of multi-slice spiral CT angiography in diagnosis and treatment of cerebral arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    余海浪; 庹秀均; 李战辉; 张应和

    2013-01-01

    目的 探讨脑动静脉畸形(CAVM)在多层螺旋CT血管造影(MSCTA)中的表现及MSCTA的临床价值.方法 收集36例行MSCTA检查的CAVM患者的资料,采用容积再现重组(VR)血管生长技术(AV)进行血管重建,分析供血动脉、引流静脉的表现.结果 36例CAVM患者中,病变累及枕叶10例,颞叶9例,额叶2例,顶叶3例,枕叶、小脑半球7例,颞、枕叶3例,额、顶叶2例,均清楚显示畸形血管团、供血动脉及引流静脉;4例合并血管畸形内动脉瘤,2例合并血管畸形外动脉瘤,3例蛛网膜下腔出血,6例脑出血.结论 MSCTA能清楚显示CAVM的畸形血管团、供血动脉、引流静脉及合并的动脉瘤.%Objective To evaluate the manifestation of cerebral arteriovenous malformations (CAVM) on multi-slice spiral CT (MSCT) angiography and the clinical value of this examinaion.Methods The data on 36 patients with CAVM undergoing MSCT angiography were collected.Vascular reconstruction were performed by volume rendering and AV.The manifestations of feeding arteries and draining veins were then analyzed.Results Among these 36 patients with cerebral arteriovenous malformations,ten patients had the lesion in occipital lobe,nine in temporal lobe,two in frontal lobe,three in parietal lobe,seven in occipital lobe and cerebellar hemisphere,three in occipital and temporal lobe,and two in frontal and parietal lobe,with a clear display of masses of deformed vessels,feeding arteries and draining veins; 4 patients were complicated by intra-aneurysm of CAVM,2 were complicated by extra-aneurysm of CAVM,3 concurred with subarachnoid hemorrhage,and 6 concurred with cerebral hemorrhage.Conclusions Multi-slice spiral angiography can clearly detect masses of deformed vessels,feeding arteries,draining veins,and coexisting aneurysms.

  19. An intratesticular arteriovenous malformation identified incidentally during ultrasound evaluation of scrotal trauma

    Directory of Open Access Journals (Sweden)

    Mohammad Saleh Jafarpishefard

    2016-01-01

    Full Text Available Intratesticular arteriovenous malformation (AVM is an extremely rare benign testicular lesion. Ultrasonography (US usually reveals a hypoechoic solid vascular mass within the testicular parenchyma. Herein, we report our experience with a young patient in whom an intratesticular AVM was found incidentally by US during the workup of scrotal trauma.

  20. Quality of life, anxiety, and depression in patients with an untreated intracranial aneurysm or arteriovenous malformation

    NARCIS (Netherlands)

    van der Schaaf, IC; Brilstra, EH; Rinkel, GJE; Bossuyt, PM; van Gijn, J

    2002-01-01

    Background and Purpose-The objective of this study was to assess the health-related quality of life and symptoms of anxiety and depression in patients who are aware of the presence of a patent aneurysm or arteriovenous malformation. Methods-Participants were retrospectively identified and invited to

  1. Imbalance of matrix metalloproteinase-9 and matrix metalloproteinase tissue inhibitor-1 may contribute to hemorrhage in cerebellar arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    Fei Di; Tongyan Chen; Hongli Li; Jizong Zhao; Shuo Wang; Yuanli Zhao; Dong Zhang

    2012-01-01

    In this study,we determined the expression levels of matrix metalloproteinase-2 and -9 and matrix metalloproteinase tissue inhibitor-1 and -2 in brain tissues and blood plasma of patients undergoing surgery for cerebellar arteriovenous malformations or primary epilepsy (control group).Immunohistochemistry and enzyme-linked immunosorbent assay revealed that the expression of matrix metalloproteinase-9 and matrix metalloproteinase tissue inhibitor-1 was significantly higher in patients with cerebellar arteriovenous malformations than in patients with primary epilepsy.The ratio of matrix metalloproteinase-9 to matrix metalloproteinase tissue inhibitor-1 was significantly higher in patients with hemorrhagic cerebellar arteriovenous malformations compared with those with non-hemorrhagic malformations.Matrix metalloproteinase-2 and matrix metalloproteinase tissue inhibitor-2 levels were not significantly changed.These findings indicate that an imbalance of matrix metalloproteinase-9 and matrix metalloproteinase tissue inhibitor-1,resulting in a relative overabundance of matrix metalloproteinase-9,might be the underlying mechanism of hemorrhage of cerebellar arteriovenous malformations.

  2. 流体切应力对脑动静脉畸形内皮细胞增殖与c-myc表达的影响%Effect of flow shear stress on endothelial cell proliferation and c-myc expression in cerebral arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    赵明光; 李彦兵; 吕博川; 梁勇; 薛洪利; 赵丽萍; 王丹玲

    2007-01-01

    BACKGROUND:Shear stress can directly mediate the expression of endothelial cells, especially some cytokine genes whose codes are related to angiogenesis. Otherwise, flow shear stress of blood plays an importantly biological role in regulating vascular structure and function.OBJECTIVE: To observe the effects of laminar flow shear stress on the proliferation of vascular endothelial cells and the expression of protooncogene c-myc in human cerebral arteriovenous malformation.DESIGN: Randomized controlled study.SETTING: Department of Neurosurgery, Shenyang General Hospital of Military Area Command of Chinese PLA.MATERIALS: The experiment was carried out in the Neuromedical Institute, General Hospital of Shenyang Military Area Command of Chinese PLA from November 2006 to February 2007. Fresh samples of human cerebral arteriovenous malformation were derived from 20 patients who were of grade Spetzler Ⅱ -Ⅲ and received resection of human cerebral arteriovenous malformation in the Department of Neurosurgery, General Hospital of Shenyang Military Area Command of Chinese PLA in 2006. All cases were diagnosed with whole-brain angiography before operation. The main reagents and equipments were detailed as follows: M199 culture media (Gilbco BRL), quality fetal bovine serum (HyClone), endothelial cell growth supplement (ECGS; Sigma, USA), CO2 incubator (Forma Scientific, USA), flow cytometry analysis of cell cycle kit (BD Company), flow cytometer (FACS Calibur, BD Company), rat-anti-human c-myc monoclonal antibody (Santa Cruz Company, USA), and reverse transcription polymerase chain reaction (RT-PCR) kit (Promega).METHODS: Tissue explants adherent method was used to culture vascular endothelial cells of human cerebral arteriovenous malformation, and then the cells were classified into 4 groups based on degree of shear stress, including control group, low shear stress group, moderate shear stress group and high shear stress group. Cultured endothelial cells of human cerebral

  3. Eloquent area in the gamma knife treatment of arteriovenous malformations of the brain

    Energy Technology Data Exchange (ETDEWEB)

    Kobayashi, Tatsuya; Kida, Yoshihisa; Tanaka, Takayuki; Yoshida, Kazuo; Mori, Yoshimasa; Ohsuga, Koji; Hasegawa, Toshinori; Kondo, Toshiki [Komaki City Hospital, Aichi (Japan)

    1999-06-01

    It has been long since cerebral arteriovenous malformation (AVM) could be cured by gamma radiosurgery. In this study, it has been found that the complete obliteration of AVM by radiosurgery is depended on the factors such as the size of the nidus, the marginal dose, the location and the age of patients. It is also true that higher the marginal dose, higher the obliteration rate and also higher the risk of radiation injury. Therefore the marginal dose has to be limited by the radiosensitivity (tolerance) of surrounding brain, which means the obliteration rate is largely depended on the location of AVM. The definition of the eloquent area of AVM by microsurgery is based upon the anatomical and functional importance of the brain. However, the eloquency in radiosurgery is different in that it depends upon radiosensitivity of the surrounding brain around AVM. From this definition, the most eloquent area by radiosurgery is brain around the optic pathway, followed by cochlear nerve, other cranial nerves, brain stem and basal ganglia-thalamus. (author)

  4. Atypical MRI features in soft-tissue arteriovenous malformation: a novel imaging appearance with radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Anand S. [University of California, San Francisco, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States); University of California, San Francisco, Department of Interventional Radiology, San Francisco, CA (United States); Schulman, Joshua M.; Ruben, Beth S. [University of California, San Francisco, Departments of Pathology and Dermatology, San Francisco, CA (United States); Hoffman, William Y. [University of California, San Francisco, Department of Plastic Surgery, Birthmarks and Vascular Anomalies Clinic, San Francisco, CA (United States); Dowd, Christopher F. [University of California, San Francisco, Department of Interventional Neuroradiology, Birthmarks and Vascular Anomalies Clinic, San Francisco, CA (United States); Frieden, Ilona J. [University of California, San Francisco, Department of Dermatology, Birthmarks and Vascular Anomalies Clinic, San Francisco, CA (United States); Hess, Christopher P. [University of California, San Francisco, Department of Neuroradiology, Birthmarks and Vascular Anomalies Clinic, San Francisco, CA (United States)

    2015-09-15

    The absence of a discrete mass, surrounding signal abnormality and solid enhancement are imaging features that have traditionally been used to differentiate soft-tissue arteriovenous malformations from vascular tumors on MRI. We have observed that these findings are not uncommon in arteriovenous malformations, which may lead to misdiagnosis or inappropriate treatment. To estimate the frequency of atypical MRI features in soft-tissue arteriovenous malformations and assess their relationship to lesion size, location, tissue type involved and vascular architecture. Medical records, MRI and histopathology were reviewed in consecutive patients with soft-tissue arteriovenous malformations in a multidisciplinary vascular anomalies clinic. Arteriovenous malformations were divided into those with and without atypical MRI findings (perilesional T2 signal abnormality, enhancement and/or a soft-tissue mass). Lesion location, size, tissue involved and vascular architecture were also compared between groups. Tissue stains were reviewed in available biopsy or resection specimens to assess relationships between MRI findings and histopathology. Thirty patients with treatment-naive arteriovenous malformations were included. Fifteen lesions demonstrated atypical MRI. There was no difference in age, gender, lesion size or involved body part between the groups. However, more than half of the atypical lesions demonstrated multicompartmental involvement, and tiny intralesional flow voids were more common in atypical arteriovenous malformations. Histopathology also differed in atypical cases, showing densely packed endothelial cells with connective tissue architectural distortion and edema. Arteriovenous malformations may exhibit features of a vascular tumor on MRI, particularly when multicompartmental and/or containing tiny internal vessels. These features are important to consider in suspected fast-flow vascular malformations and may have implications with respect to their treatment

  5. Intraparenchymal brain hemorrhage and remote soft tissue arteriovenous malformation in a newborn infant.

    Science.gov (United States)

    Jee, Sandra H; Grow, Jennifer L; Faix, Roger G

    2002-12-01

    Congenital arteriovenous malformations (AVMs) often present with congestive heart failure. Such pathologic vascular structures typically occur in cranial, hepatic, or pulmonary locations and are usually associated with overlying external visible, tactile, or audible abnormalities. These vascular anomalies may also be associated with such complications as thromboembolic events, coagulopathy, and localized hemorrhage. We present a newborn infant with an occult but hemodynamically significant parascapular AVM who presented with an intraparenchymal brain hemorrhage, which we suspect to be a remote complication of the AVM.

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

    Science.gov (United States)

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

    2014-01-01

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

  7. Transcatheter coil embolisation of pulmonary arteriovenous malformations; Transkatheterembolisation pulmonaler arterio-venoeser Fisteln

    Energy Technology Data Exchange (ETDEWEB)

    Wingen, M.; Guenther, R.W. [Technische Hochschule Aachen (Germany). Klinik fuer Radiologische Diagnostik

    2001-07-01

    Evaluation of technical success, complications and long-term results of transcatheter coil embolisation in pulmonary arteriovenous malformations (pAVMs). Material and Methods: Transcatheter embolisations of 46 pAVMs in 14 patients were analysed retrospectively, and, 5 years after treatment, the patients were interviewed by telefone concerning persistent symptoms and complications. Main symptoms before embolisation were dyspnoe (86%), hypoxaemia (100%), cerebral ischemia (21%), and hemoptysis (14%); 11 patients (79%) suffered from hereditary hemorrhagic telangiectasia. Results: Embolisation with an average of 3,9 coils (min. 1, max. 19 coils) per pAVM yielded technical success in all cases. Only two minor complications, transitory pleuritis and a small lung infarction were observed. On follow up examination after 5 years either no residual complaints or substantial improvement of dyspnoe were reported; no patient suffered from neurologic or hemorrhagic complications after the embolisation. Conclusion: Transcatheter embolisation is a safe and minimally invasive therapy for pAVMs and has rightfully replaced surgical resection as the therapy of choice. (orig.) [German] Bewertung der Spiralembolisation pulmonaler AV-Fisteln hinsichtlich Erfolgsrate, Komplikationen und Langzeitergebnissen. Material und Methoden: Retrospektiv wurden die Transkatheterembolisationen von 46 pulmonalen AV-Fisteln bei 14 Patienten ausgewertet und die Patienten bzw. ihre Hausaerzte im Mittel 5 Jahre nach der Intervention telefonisch nachbefragt. Fuehrende Symptome vor der Behandlung waren Belastungsdyspnoe (86%), Hypoxaemie (100%), zerebrale Ischaemien (21%) und Haemoptysen (14%); 11 der Patienten (79%) litten an M. Osler. Ergebnisse: die Embolisation mit im Mittel 3,9 Spiralen (minimal 1, maximal 19 Spiralen) pro Fistel war in allen Faellen technisch erfolgreich, Komplikationen waren mit einer leichten Pleuritis und einer Infarktpneumonie selten und nicht gravierend. Alle nachbefragten

  8. Ethanol embolization of arteriovenous malformations: results and complications of 33 cases

    Energy Technology Data Exchange (ETDEWEB)

    Jeon, Yong Hwan; Do, Young Soo; Shin, Sung Wook; Liu, Wei Chiang; Cho, Jae Min; Lee, Min Hee; Kim, Dong Ik; Lee, Byung Boong; Choo, Sung Wook; Choo, In Wook [School of Medicine, Sungkyunkwan Univ., Seoul (Korea, Republic of)

    2003-10-01

    To assess the effectiveness of ethanol embolization for the treatment of arteriovenous malformation (AVM), and the complications, if any, arising. Thirty-three patients with AVMs underwent 145 staged sessions of ethanol embolization. AVMs were located in an upper extremity (n=14), a lower extremity (n=10), the pelvis (n=7), the thorax (n=1), or the abdomen (n=1). Eighty-five transcatheter embolizations and 60 direct percutaneous puncture embolizations were performed, and seven patients underwent additional coil embolization of the dilated outflow vein. The therapeutic effectiveness of embolization was evaluated in terms of the extent to which an AVM was obliterated between baseline and the final angiogram. Complications were classified as minor or major. In 13 patients (39%), AVMs were totally obliterated. In eight patients (24%), more than 75% were obliterated; in three (9%), the proportion was 50-75%; and in four (12%), less than 50%. Five patients (15%), were not treated. The reasons for failure were the difficulty of approaching the nidus due to previous surgical ligation or coil embolization of the feeding artery, the subcutaneous location of an AVM, post-procedural infection, and massive bleeding during the follow-up period. Twenty-one minor complications such as focal skin necrosis or transient nerve palsy developed during 145 sessions of (an incidence of 14%), but these were relieved by conservative treatment. The five major complications arising (3%) were cerebral infarction, urinary tract infection, acute renal failure due to rhabdomyolysis, permanent median nerve palsy, and infection. Ethanol embolization by direct percutaneous puncture or using a transcatheter technique is an effective approach to the treatment of an AVM. However, to overcome the considerable number of complications, arising, further investigation is required.

  9. Vascular permeability in cerebral cavernous malformations

    DEFF Research Database (Denmark)

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao;

    2015-01-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observ......Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case......-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability...... correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive...

  10. Successful management with glue injection of arterial rupture seen during embolization of an arteriovenous malformation using a flow-directed catheter: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Jong Won; Baik, Seung Kug; Shin, Mi Jung; Choi, Han Yong; Kim, Bong Gi [Wallace Memorial Baptist Hospital, Pusan (Korea, Republic of)

    2000-12-01

    We present a case in which an arterial rupture occurring during embolization of an arteriovenous malformation of the left occipital lobe with a flow-directed micro-catheter, was successfully sealed with a small amount of glue. We navigated a 1.8-Fr Magic catheter through the posterior cerebral artery, and during superselective test injection, extravasation was observed at the parieto-occipital branch. The catheter was not removed and the perforation site was successfully sealed with a small amount of glue injected through the same catheter. Prompt recognition and closure of the perforation site is essential for good prognosis. (author)

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

    Energy Technology Data Exchange (ETDEWEB)

    Grasso, Rosario Francesco, E-mail: r.grasso@unicampus.it; Cazzato, Roberto Luigi; Luppi, Giacomo; Faiella, Eliodoro; Del Vescovo, Riccardo; Giurazza, Francesco [University ' Campus Bio-Medico of Rome' , Department of Radiology (Italy); Borzomati, Domenico; Coppola, Roberto [University ' Campus Bio-Medico of Rome' , Department of General Surgery (Italy); Beomonte Zobel, Bruno [University ' Campus Bio-Medico of Rome' , Department of Radiology (Italy)

    2012-08-15

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

  12. Pulmonary Arteriovenous Malformation in Cryptogenic Liver Cirrhosis Associated with Turner's Syndrome.

    Science.gov (United States)

    Kim, Ji Hoon; Jung, Young Kul; Jeong, Eun Seok; Seo, Yeon Seok; Yim, Hyung Joon; Yeon, Jong Eun; Shim, Jae Jeong; Byun, Kwan Soo; Lee, Chang Hong

    2010-06-01

    Turner's syndrome is a genetic disorder of the sex chromosomes (e.g., 45,X or 45,X/46,XX) that manifests as various congenital anomalies. Despite its numerous extragonadal manifestations and frequent accompanying abnormalities in liver function tests, liver cirrhosis associated with Turner's syndrome has not been reported in Korea. Moreover, pulmonary arteriovenous malformations (PAVMs) have rarely been reported in association with liver cirrhosis, but there have been no reports of PAVMs occurring in cryptogenic liver cirrhosis associated with Turner's syndrome. We report a case of PAVM that occurred in cryptogenic liver cirrhosis associated with Turner's syndrome.

  13. Transcatheter closure of a pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia.

    Science.gov (United States)

    Parker, Christopher; Rousan, Talla A; Abu-Fadel, Mazen

    2015-07-01

    Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Over 50 % of the cases are associated with hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu Syndrome. Untreated PAVMs progressively enlarge and can cause significant right-to-left shunting. Surgical- and catheter-based approaches have been used in the management of PAVM. We report a case of a 74-year-old man who presented with dyspnea and hypoxia and was found to have a large right-sided PAVM. He underwent percutaneous closure of the PAVM with an Amplatzer device with significant improvement of his symptoms.

  14. Treatment of Residual Facial Arteriovenous Malformations after Embolization with Percutaneous Cryotherapy.

    Science.gov (United States)

    Woolen, Sean; Gemmete, Joseph J

    2016-10-01

    This report presents 4 patients (mean age, 22 y; range, 17-26 y) with facial arteriovenous malformations (AVMs) who underwent arterial ethanol and N-butyl cyanoacrylate embolization followed by percutaneous cryoablation of residual inaccessible AVMs. After the procedure, minor complications classified as type B according to the Society of Interventional Radiology (SIR) classification system occurred in 75% (3/4) of patients. One patient achieved 90% obliteration of AVM, and 3 patients had complete obliteration of AVM at 1-year follow-up. This reports shows that percutaneous ablation may be a viable treatment option for residual facial AVMs after ethanol and glue embolization.

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

    Science.gov (United States)

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

    2014-11-01

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

  16. High-flow orbital arteriovenous malformation in a child: current management and options.

    Science.gov (United States)

    Trombly, Ryan; Sandberg, David I; Wolfe, S Anthony; Ragheb, John

    2006-07-01

    Vascular malformations of the orbit cause significant morbidity such as chronic pain, diplopia, amblyopia, and cosmetic disfigurement. They are rare lesions which require multidisciplinary care, and in the modern era results of treatment have been greatly improved with the assistance of endovascular therapy. Other treatment options include laser therapy, percutaneous embolization, open surgery, or a combination of these modalities. Nevertheless some patients suffer poor results despite modern medical advances. A case of an orbital arteriovenous malformation (AVM) initially treated independently by a dermatologist, a plastic surgeon, and a neuroendovascular interventionalist is presented. When treating patients with these rare but disabling lesions it is of the highest importance to coordinate efforts between all pertinent specialists in order to promote the best possible result.

  17. Evaluation of the degree of arteriovenous shunting in intracranial arteriovenous malformations using pseudo-continuous arterial spin labeling magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Sunwoo, Leonard; Park, Sun-Won [Seoul Metropolitan Government - Seoul National University Boramae Medical Center, Department of Radiology, Seoul (Korea, Republic of); Seoul National University College of Medicine, Department of Radiology, Seoul (Korea, Republic of); Sohn, Chul-Ho; Yun, Tae Jin; Choi, Seung Hong; Cho, Young Dae; Kim, Ji-hoon; Han, Moon Hee [Seoul National University College of Medicine, Department of Radiology, Seoul (Korea, Republic of); Seoul National University Hospital, Department of Radiology, Seoul (Korea, Republic of); Lee, Jong Young [Kangdong Sacred Heart Hospital, Department of Neurosurgery, Seoul (Korea, Republic of); Yi, Kyung Sik [Chungbuk National University Hospital, Department of Radiology, Cheongju (Korea, Republic of); Paek, Sun Ha; Kim, Yong Hwy; Kim, Jin Wook; Chung, Hyun-Tai; Kim, Dong Gyu [Seoul National University Hospital, Department of Neurosurgery, Seoul (Korea, Republic of)

    2015-08-15

    Intracranial arteriovenous malformations (AVMs) display venous signals on arterial spin labeling (ASL) magnetic resonance (MR) imaging due to the presence of arteriovenous shunting. Our aim was to quantitatively correlate AVM signal intensity on ASL with the degree of arteriovenous shunting estimated on digital subtraction angiography (DSA) in AVMs. MR imaging including pseudo-continuous ASL at 3 T and DSA were obtained on the same day in 40 patients with intracranial AVMs. Two reviewers assessed the nidus and venous signal intensities on ASL images to determine the presence of arteriovenous shunting. Interobserver agreement on ASL between the reviewers was determined. ASL signal intensity of the AVM lesion was correlated with AVM size and the time difference between normal and AVM venous transit times measured from the DSA images. Interobserver agreement between two reviewers for nidus and venous signal intensities was excellent (κ = 0.80 and 1.0, respectively). Interobserver agreement regarding the presence of arteriovenous shunting was perfect (κ = 1.0). AVM signal intensity showed a positive relationship with the time difference between normal and AVM venous transit times (r = 0.638, P < 0.001). AVM signal intensity also demonstrated a positive relationship with AVM size (r = 0.561, P < 0.001). AVM signal intensity on ASL in patients with AVM correlates well with the degree of early vein opacification on DSA, which corresponds to the degree of arteriovenous shunting. (orig.)

  18. Vascular permeability in cerebral cavernous malformations.

    Science.gov (United States)

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

    2015-10-01

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

  19. Treatment for arteriovenous malformation of the brain Comparison between microsurgery and gamma knife

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Microsurgery and gamma knife are the mainly ways to treat arteriovenous malformation of brain in grade Spetzler-Martin Ⅰ Ⅲ; however, therapeutic effects of them need to be further studied.OBJECTIVE: To compare the therapeutic effects between microsurgery and gamma knife on the treatment of arteriovenous malformation of brain in grade Spetzler-Martin Ⅰ-Ⅲ.DESIGN: Retrospective analysis.SETTING: Department of Neurosurgery, the Third Hospital Affiliated to Sun Yat-sen University;Guangdong Microinvasion Center.PARTICIPANTS: A total of 86 patients with arteriovenous malformation of the brain were selected from the Department of Neurosurgery, the Third Hospital Affiliated to Sun Yat-sen University and Guangdong Microinvasion Center from January 1997 to February 2007. After DSA, CT and/or MRI examinations,patients were evaluated in grade Spetzler-Martin Ⅰ - Ⅲ. All patients were divided into microsurgery group (n = 34) and gamma knife group (n =52). There were 22 males and 12 females in the microsurgery group and their mean age was 26 years, while there were 34 males and 18 females in the gamma knife group and their mean age was 28 years. The grade of Spetzler-Martin was comparable in the two groups. All their relatives provided the confirmed consent and the study was allowed by ethics committee of our hospital.METHODS: Under complete anesthesia, patients were given microsurgery and the operative approach was chosen based on diseased regions. Firstly, feeding artery was blocked; secondly, it was separated along band of gliosis between malformation vessel mass and brain tissue; finally, draining vein was cut off and malformation vessel mass was resected. On the other hand, patients in the gamma knife group received Leksell-2300B gamma knife treatment. Leksell-G stereotaxis headframe was installed; GE1.5TMR scanning device was used for localization; r-Plan5.2 workstation was used for target design and dosage program;Leksell B gamma knife was used

  20. Computer simulation of Cerebral Arteriovenous Malformation—validation analysis of hemodynamics parameters

    Science.gov (United States)

    Mehta, Shashi Bhushan; Ramachandra, Manjunath

    2017-01-01

    Problem The purpose of this work is to provide some validation methods for evaluating the hemodynamic assessment of Cerebral Arteriovenous Malformation (CAVM). This article emphasizes the importance of validating noninvasive measurements for CAVM patients, which are designed using lumped models for complex vessel structure. Methods The validation of the hemodynamics assessment is based on invasive clinical measurements and cross-validation techniques with the Philips proprietary validated software’s Qflow and 2D Perfursion. Results The modeling results are validated for 30 CAVM patients for 150 vessel locations. Mean flow, diameter, and pressure were compared between modeling results and with clinical/cross validation measurements, using an independent two-tailed Student t test. Exponential regression analysis was used to assess the relationship between blood flow, vessel diameter, and pressure between them. Univariate analysis is used to assess the relationship between vessel diameter, vessel cross-sectional area, AVM volume, AVM pressure, and AVM flow results were performed with linear or exponential regression. Discussion Modeling results were compared with clinical measurements from vessel locations of cerebral regions. Also, the model is cross validated with Philips proprietary validated software’s Qflow and 2D Perfursion. Our results shows that modeling results and clinical results are nearly matching with a small deviation. Conclusion In this article, we have validated our modeling results with clinical measurements. The new approach for cross-validation is proposed by demonstrating the accuracy of our results with a validated product in a clinical environment. PMID:28149675

  1. Arteriovenous Malformation

    Science.gov (United States)

    ... bleeding into surrounding tissue (hemorrhage) that can cause stroke or brain damage, and by compressing or displacing parts of the ... bleeding into surrounding tissue (hemorrhage) that can cause stroke or brain damage, and by compressing or displacing parts of the ...

  2. Cerebral abscesses among Danish patients with hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Tørring, P M; Nissen, H;

    2013-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs), which due to paradoxical embolization may cause cerebral abscess.......Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs), which due to paradoxical embolization may cause cerebral abscess....

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

    Energy Technology Data Exchange (ETDEWEB)

    Uchikawa, Yoko, E-mail: jauchikawa@gmail.com [University of Tsukuba Hospital, Department of Radiology (Japan); Mori, Kensaku, E-mail: moriken@md.tsukuba.ac.jp [University of Tsukuba, Department of Radiology, Faculty of Medicine (Japan); Shiigai, Masanari, E-mail: m-41gai@yahoo.co.jp [Tsukuba Medical Center Hospital, Department of Radiology (Japan); Konishi, Takahiro, E-mail: soratobukangaruu@gmail.com [University of Tsukuba Hospital, Department of Radiology (Japan); Hoshiai, Sodai, E-mail: hoshiai@sb4.so-net.ne.jp [Ibaraki Prefectural Central Hospital, Department of Radiology (Japan); Ishigro, Toshitaka, E-mail: suzutokei@gmail.com; Hiyama, Takashi, E-mail: med-tak@hotmail.com [University of Tsukuba Hospital, Department of Radiology (Japan); Nakai, Yasunobu, E-mail: nakaiya@tmch.or.jp [Tsukuba Medical Center Hospital, Department of Neurosurgery (Japan); Minami, Manabu, E-mail: mminami@md.tsukuba.ac.jp [University of Tsukuba, Department of Radiology, Faculty of Medicine (Japan)

    2015-10-15

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

  4. Development of a huge varix following endovascular embolization for cerebellar arteriovenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Mineura, K.; Sasajima, H.; Itoh, Y.; Kowada, M. [Akita Univ. Hospital (Japan). Neurosurgical Service; Tomura, N. [Akita Univ. Hospital (Japan). Dept. of Radiology; Goto, K. [Iizuka Hospital, Fukuoka (Japan). Dept. of Interventional Neuroradiology

    1998-03-01

    We report on the case of a huge varix that developed after the endovascular embolization of a cerebellar arteriovenous malformation (AVM) with a single drainer. A 21-year-old male presented with trigeminal neuralgia which was caused by the dilated drainer of the AVM. A varix was found at the basal vein of Rosenthal 2 months after an initial stage of embolization with polyvinyl alcohol particles; it diminished after the surgical extirpation of the AVM. The varix formation might have been facilitated by the stenosis in the vein of Galen and by the dynamic changes that followed the embolization. This rare complication should be kept in mind when embolization is performed for AVMs with impaired venous outlets. (orig.).

  5. Hypofractionated Stereotactic Radiosurgery in a Large Bilateral Thalamic and Basal Ganglia Arteriovenous Malformation

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    Janet Lee

    2013-01-01

    Full Text Available Purpose. Arteriovenous malformations (AVMs in the basal ganglia and thalamus have a more aggressive natural history with a higher morbidity and mortality than AVMs in other locations. Optimal treatment—complete obliteration without new neurological deficits—is often challenging. We present a patient with a large bilateral basal ganglia and thalamic AVM successfully treated with hypofractionated stereotactic radiosurgery (HFSRS with intensity modulated radiotherapy (IMRT. Methods. The patient was treated with hypofractionated stereotactic radiosurgery to 30 Gy at margin in 5 fractions of 9 static fields with a minimultileaf collimator and intensity modulated radiotherapy. Results. At 10 months following treatment, digital subtraction angiography showed complete obliteration of the AVM. Conclusions. Large bilateral thalamic and basal ganglia AVMs can be successfully treated with complete obliteration by HFSRS with IMRT with relatively limited toxicity. Appropriate caution is recommended.

  6. Endovascular treatment of brain-stem arteriovenous malformations: safety and efficacy

    Energy Technology Data Exchange (ETDEWEB)

    Liu, H.M.; Wang, Y.H.; Chen, Y.F.; Huang, K.M. [Department of Medical Imaging, National Taiwan University Hospital, 7 Chung-Shan South Road, 10016, Taipei (Taiwan); Tu, Y.K. [Division of Neurosurgery, Department of Surgery, National Taiwan University Hospital, 7 Chung-Shan South Road, 1001, Taipei (Taiwan)

    2003-09-01

    Our purpose was to evaluate the safety and efficacy of endovascular treatment of brain-stem arteriovenous malformations (AVMs), reviewing six cases managed in the last 5 years. There were four patients who presented with bleeding, one with a progressive neurological deficit and one with obstructive hydrocephalus. Of the six patients, one showed 100%, one 90%, two 75% and two about 50% angiographic obliteration of the AVM after embolisation; the volume decreased about 75% on average. Five patients had a good outcome and one an acceptable outcome, with a mild postprocedure neurological deficit; none had further bleeding during midterm follow-up. Endovascular management of a brain-stem AVM may be an alternative to treatment such as radiosurgery and microsurgery in selected cases. It may be not as risky as previously thought. Embolisation can reduce the size of the AVM and possibly make it more treatable by radiosurgery and decrease the possibility of radiation injury. (orig.)

  7. ArterioVenous Malformation within Jejunal Diverticulum: An Unusual Cause of Massive Gastrointestinal Bleeding

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    Jeffrey K. Lee

    2009-01-01

    Full Text Available Massive gastrointestinal (GI bleeding can occur with multiple jejunal diverticulosis. However, significant bleeding in the setting of few diverticulae is very unusual and rare. We report a case of massive gastrointestinal bleeding from an arteriovenous malformation (AVM within a jejunal diverticulum to underscore the significance of such coexisting pathologies. Mesenteric angiogram was chosen to help identify the source of bleeding and to offer an intervention. Despite endovascular coiling, emergent intestinal resection of the bleeding jejunal segment was warranted to ensure definitive treatment. However several reports have shown jejunal diverticulosis as a rare cause of massive GI bleeding. The coexistence of jejunal diverticulum and AVM is rare and massive bleeding from an acquired Dieulafoy-like AVM within a diverticulum has never previously been described. Awareness of Dieulafoy-like AVM within jejunoileal diverticulosis is useful in preventing delay in treatment.

  8. Endoscopic Resection of Asymptomatic, Colonic, Polypoid Arteriovenous Malformations: Two Case Reports and a Literature Review

    Science.gov (United States)

    Lee, Han-Hee; Kwon, Hyuk-Min; Gil, Sanghyun; Kim, Young-Shin; Cho, Minjung; Seo, Kyung-Jin; Chae, Hiun-Suk; Cho, Young-Seok

    2017-01-01

    A colonic arteriovenous malformation (AVM) is a significant vascular lesion of the gastrointestinal tract and a common cause of lower gastrointestinal bleeding. AVMs are usually identified endoscopically as bright red, flat lesions. AVMs with a polypoid appearance are extremely rare in the large intestine. We present two cases of colonic polypoid AVM, which were detected incidentally during screening colonoscopy. Both the patients had no history of gastrointestinal bleeding such as melena or hematochezia. Colonoscopy revealed pedunculated polyps overlaid by hyperemic mucosa in the ascending colon and proximal sigmoid colon. Microscopic examination showed aberrant vessels with thickened, hypertrophic walls in the mucosa and the submucosa, and arteries were directly connected to veins without capillary beds. These features were compatible with a diagnosis of AVM with a polypoid appearance. No immediate or delayed bleeding was noted after polypectomy. PMID:28139503

  9. A spindle cell hemangioendothelioma on the head resembling an arteriovenous malformation.

    Science.gov (United States)

    Higashino, Takuya; Hirai, Rintaro

    2014-07-01

    A spindle cell hemangioendothelioma is a relatively uncommon lesion, especially on the head and neck. Recurrence occurs after local excision of 50% to 60% of these lesions; therefore, it is important to recognize this unusual neoplasm and avoid misdiagnosis. Here, we report a rare case of a spindle cell hemangioendothelioma of the head. A 37-year-old woman presented with a soft subcutaneous mass, 2.5 cm in size, on her right occipital region. The mass pulsated strongly and a thrill was present. Magnetic resonance imaging showed that some dilated feeding arteries flowed into the mass and that a flow-void sign was present. The lesion looked like an arteriovenous malformation, and a marginal resection was performed. Histologically, there was a mix of cavernous vascular cavities and Kaposi sarcomalike spindle cell vascular zones, which is compatible with a spindle cell hemangioendothelioma.

  10. Orthodeoxia and postural orthostatic tachycardia in patients with pulmonary arteriovenous malformations: a prospective 8-year series.

    Science.gov (United States)

    Santhirapala, V; Chamali, B; McKernan, H; Tighe, H C; Williams, L C; Springett, J T; Bellenberg, H R; Whitaker, A J; Shovlin, C L

    2014-11-01

    Postural changes in 258 patients with pulmonary arteriovenous malformations (PAVMs) reviewed between 2005 and 2013 were evaluated prospectively using validated pulse oximetry methods. Of the 257 completing the test, 75 (29%) demonstrated orthodeoxia with an oxygen saturation fall of at least 2% on standing. None described platypnoea (dyspnoea on standing). The heart rate was consistently higher in the erect posture: 74 (29%) had a postural orthostatic tachycardia of ≥20 min(-1), and in 25 (10%) this exceeded 30 min(-1). Orthostatic tachycardia was more pronounced in PAVM patients than controls without orthodeoxia (age-adjusted coefficient 5.5 (95% CIs 2.6, 8.4) min(-1), ppostural orthostatic tachycardia syndrome, in this population, there was a trend for more pronounced orthostatic tachycardia to be associated with better exercise tolerance.

  11. [Hyperkalemia caused by intravenous administration of mannitol in a patient with arteriovenous malformation: case report].

    Science.gov (United States)

    Kimura, Shigeyoshi; Ogawa, Haruhiko; Katayama, Yoichi

    2006-01-01

    We experienced a case in which hyperkalemia was induced by mannitol administration. The medication with mannitol was given to a 15-year-old male patient who underwent a removal operation for arteriovenous malformation under general anesthesia. Following the mannitol infusion, his arterial blood gas and electrolyte analysis revealed severe metabolic acidosis and an increase in serum potassium. Furthermore, a change in his electrocardiogram was observed. The hyperkalemia was quickly normalized by medication with calcium gluconate and sodium bicarbonate. We stopped the removal operation with the aim of giving priority to the patient's safety. It is speculated that the hyperkalemia was caused by the administration of mannitol. Checks of electrolyte levels, arterial blood gas analysis and electrocardiogram monitoring should therefore be carried out when using mannitol, especially in an emergency situation such as intracranial hemorrhage.

  12. Arteriovenous Malformations in the Pediatric Population: Review of the Existing Literature

    Science.gov (United States)

    El-Ghanem, Mohammad; Kass-Hout, Tareq; Kass-Hout, Omar; Alderazi, Yazan J.; Amuluru, Krishna; Al-Mufti, Fawaz; Prestigiacomo, Charles J.; Gandhi, Chirag D.

    2016-01-01

    Arteriovenous malformations (AVMs) in the pediatric population are relatively rare but reportedly carry a higher rate of rupture than in adults. This could be due to the fact that most pediatric AVMs are only detected after rupture. We aimed to review the current literature regarding the natural history and the clinical outcome after multimodality AVM treatment in the pediatric population, as optimal management for pediatric AVMs remains controversial. A multidisciplinary approach using multimodality therapy if needed has been proved to be beneficial in approaching these lesions in all age groups. Microsurgical resection remains the gold standard for the treatment of all accessible pediatric AVMs. Embolization and radiosurgery should be considered as an adjunctive therapy. Embolization provides a useful adjunct therapy to microsurgery by preventing significant blood loss and to radiosurgery by decreasing the volume of the AVM. Radiosurgery has been described to provide an alternative treatment approach in certain circumstances either as a primary or adjuvant therapy.

  13. Imaging findings of arteriovenous malformations involving lung and liver in hereditary hemorrhagic telangiectasia(Osler-weber-rendu disease): two cases report

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Jeong Geun; Lee, Joo Hyuk; Seong, Su Ok [Cheongju St. Mary' s Hospital, Cheongju (Korea, Republic of)

    1999-09-01

    Hereditary hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu disease is an autosomal dominant disorder characterized by repeated episodes of bleeding. Multiple telangiectases consisting of thin-walled, dilated vascular channels with arteriovenous communication may involve, for example, mucocutaneous tissue, the gastrointestinal tract, and the liver, lung, and brain. We report the imaging findings of two cases of HHT involving arteriovenous malformation of both the lungs and liver, a rare condition. Chest radiography revealed a round mass, while helical CT showed a feeding artery and draining vein with arteriovenous malformation in the lung. Color Doppler sonography revealed an enlarged and tortuous hepatic artery with high systolic velocity. CT demonstrated an enlarged hepatic artery, arteriovenous shunt, and early draining hepatic vein in the liver. Celiac angiography showed arteriovenous malformation.

  14. 3D-Printing of Arteriovenous Malformations for Radiosurgical Treatment: Pushing Anatomy Understanding to Real Boundaries.

    Science.gov (United States)

    Conti, Alfredo; Pontoriero, Antonio; Iatì, Giuseppe; Marino, Daniele; La Torre, Domenico; Vinci, Sergio; Germanò, Antonino; Pergolizzi, Stefano; Tomasello, Francesco

    2016-04-29

    Radiosurgery of arteriovenous malformations (AVMs) is a challenging procedure. Accuracy of target volume contouring is one major issue to achieve AVM obliteration while avoiding disastrous complications due to suboptimal treatment. We describe a technique to improve the understanding of the complex AVM angioarchitecture by 3D prototyping of individual lesions. Arteriovenous malformations of ten patients were prototyped by 3D printing using 3D rotational angiography (3DRA) as a template. A target volume was obtained using the 3DRA; a second volume was obtained, without awareness of the first volume, using 3DRA and the 3D-printed model. The two volumes were superimposed and the conjoint and disjoint volumes were measured. We also calculated the time needed to perform contouring and assessed the confidence of the surgeons in the definition of the target volumes using a six-point scale. The time required for the contouring of the target lesion was shorter when the surgeons used the 3D-printed model of the AVM (p=0.001). The average volume contoured without the 3D model was 5.6 ± 3 mL whereas it was 5.2 ± 2.9 mL with the 3D-printed model (p=0.003). The 3D prototypes proved to be spatially reliable. Surgeons were absolutely confident or very confident in all cases that the volume contoured using the 3D-printed model was plausible and corresponded to the real boundaries of the lesion. The total cost for each case was 50 euros whereas the cost of the 3D printer was 1600 euros. 3D prototyping of AVMs is a simple, affordable, and spatially reliable procedure that can be beneficial for radiosurgery treatment planning. According to our preliminary data, individual prototyping of the brain circulation provides an intuitive comprehension of the 3D anatomy of the lesion that can be rapidly and reliably translated into the target volume.

  15. The MDCT and MRI Findings of a Pancreatic Arteriovenous Malformation Combined with Isolated Dissection of the Superior Mesenteric Artery: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong Soo; Jeong, Woo Kyoung [Hanyang University Guri Hospital, Seoul (Korea, Republic of); Kim, Jin Oo [Naval Pohang Hospital, Pohang (Korea, Republic of); Oh, Ji Young; Song, Soon Young [Hanyang University Medical College, Seoul (Korea, Republic of)

    2010-03-15

    Pancreatic arteriovenous malformation and isolated spontaneous dissection of the superior mesenteric artery are both rare maladies, and now they can be easily diagnosed due to the development of such noninvasive modalities as multi-detector computed tomography and magnetic resonance imaging. We report here on the multi-detector computed tomography and magnetic resonance imaging findings of a rare case of pancreatic arteriovenous malformation combined with isolated dissection of the superior mesenteric artery.

  16. A rare case of arteriovenous malformation following hysterectomy in a case of choriocarcinoma

    Directory of Open Access Journals (Sweden)

    Suchitra R

    2015-10-01

    Full Text Available A uterine arteriovenous malformation (AVM is a rare cause of uterine bleeding. It may have varied presentations ranging from being completely asymptomatic; to features of congestive heart failure, to vaginal bleeding which may at times life be threatening. Clinical findings in such cases are often un-reliable; requiring a high index of suspicion to make the diagnosis. We report a case of a 46-year-old lady who presented with heavy vaginal bleeding. She has undergone hysterectomy with a histopathology of choriocarcinoma one and half months back. She has received chemotherapy and 8 fractions of radiotherapy for the same. AVM was diagnosed following a CT angiogram and was managed by embolization. We also discuss in brief about this uncommon but serious condition which the radiologist/gynaecologist may encounter in their practice. AV Malformation is a rare but potentially life-threatening cause of vaginal bleeding which must be kept in the differential diagnosis of sudden and massive vaginal bleeding. It requires a high index of clinical suspicion. Despite its rarity, early recognition of an AVM is imperative to enable timely diagnosis and intervention. [Int J Reprod Contracept Obstet Gynecol 2015; 4(5.000: 1561-1564

  17. Trigeminal Neuralgia Caused by Cerebellopontine Angle Arteriovenous Malformation Treated With Gamma Knife Radiosurgery.

    Science.gov (United States)

    Işik, Semra; Ekşi, Murat Şakir; Yilmaz, Baran; Toktaş, Zafer Orkun; Akakin, Akin; Kiliç, Türker

    2016-01-01

    Trigeminal neuralgia is a facial pain syndrome characterized as sudden onset and lightening-like sensation over somatosensorial branch(es) of fifth cranial nerve. Rarely, some underlying diseases or disorders could be diagnosed, such as multiple sclerosis, brain tumors, and vascular malformations. The authors present a 47-year-old man with trigeminal neuralgia over left V2 and V3 dermatomes. He had a previous transarterial embolization and long use of carbamazepine with partial response to treatment. Gamma knife radiosurgery (GKR) was planned. A marginal dose of 15 Gy was given to 50% isodose line. His pain was relieved by GKR in 1.5 years. Treatment of posterior fossa arteriovenous malformations causing trigeminal neuralgia, with GKR has a very limited use in the literature. It, however, is obvious that success rate as pain relief, in a very challenging field of functional neurosurgery, is satisfactory. Large series, however, are in need to make a more comprehensive statement about efficacy and safety of the procedure in these pathologies.

  18. Susceptibility-weighted imaging in the evaluation of brain arteriovenous malformations

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    George Uttam

    2010-01-01

    Full Text Available Background: Digital subtraction angiography (DSA remains the gold standard in the evaluation of arteriovenous malformations (AVMs. Susceptibility-weighted imaging (SWI, a relatively new magnetic resonance imaging (MRI technique, exploits the magnetic susceptibility differences of various tissues, such as blood, iron and calcification. Earlier studies have shown that the magnitude and phase information of SWI offers improved sensitivity, revealing low-flow vascular malformations that are invisible on conventional gradient-echo (GRE sequences. Aim: To evaluate the imaging appearance of AVMs on SWI. Materials and Methods: In this retrospective study, the appearance of the various components (feeding artery, nidus, and draining veins of AVMs on the phase, magnitude, and minimal intensity projection (minIP images of SWI were analyzed in 14 patients with AVM and compared with conventional sequences. Results: Detection and delineation of various components of AVMs was best achieved in the magnitude images. Although minIP was most effective in detecting hemorrhage and calcification, it was the magnitude image that could separate the hemorrhagic and calcified component in the nidus from the remaining nidus. The minIP was less effective in detecting the AVM components, especially nidus and draining vein, whereas conspicuity was poor with the phase images. Conclusion: The magnitude images of the SWI help in differentiating the different components of AVM and also helps in differentiating nidus from hemorrhage and calcification.

  19. Twenty-seven years follow-up of a patient with congenital retinocephalofacial vascular malformation syndrome and additional congenital malformations (Bonnet-dechaume-blanc syndrome or wyburn-mason syndrome

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    Schmidt D

    2010-02-01

    Full Text Available Abstract Purpose Follow-up of vascular changes in a patient with congenital retinocephalofacial vascular malformation syndrome. Methods MRI and cerebral angiography. Results In a 36-year-old man, magnetic resonance im aging of the skull and cerebral angiography revealed left intracranial arteriovenous malformations. Follow-up observation of 27 years revealed no essential change of retinal and cerebral arteriovenous malformations. Additional congenital deficits in this patient were described. Conclusion Patients with retinal arteriovenous malformations should be early examined with neuroradiological methods.

  20. Successful laparoscopic bipolar coagulation of a large arteriovenous malformation due to invasive trophoblastic disease: a case report.

    Science.gov (United States)

    Corusic, Ante; Barisic, Dubravko; Lovric, Helena; Despot, Albert; Planinic, Pavao

    2009-01-01

    We report the case of an acquired large arteriovenous malformation due to invasive gestational trophoblastic tumor that was treated successfully with laparoscopic surgery. After 4 cycles of methotrexate chemotherapy, a vascular tangle (volume, 28 cm(3)) was noted that emerged from the right uterine horn, invading the broad ligament adjacent to the uterine artery. Doppler ultrasonography along with magnetic resonance arteriography confirmed the diagnosis. The location, size and relation of this arteriovenous malformation to the uterine vasculature demanded urgent intervention. Laparoscopy was performed, and bipolar coagulation of the ovarian and uterine artery feeding branches was achieved after surgical resection of the tumor. The defect in the uterine wall with an intact uterine cavity was reconstructed using sutures. There were no intraoperative or postoperative complications. The patient underwent chemotherapy, and at 2-month follow-up was cured and has since had regular menstrual cycles.

  1. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    Directory of Open Access Journals (Sweden)

    Andrew Conger

    2015-01-01

    Conclusions: These lesions are among the most technically difficult pathological entities handled by the cerebrovascular specialist, and an overview of technical concepts to help systematize this challenging and variable endeavor can improve the safety of their treatment.

  2. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    NARCIS (Netherlands)

    de Wit, Olga A; den Dunnen, Wilfred Fa; Sollie, Krystyne M; Muñoz, Rosa Iris; Meiners, Linda C; Brouwer, Oebele F; Rodríguez, Esteban M; Sival, Deborah A

    2008-01-01

    BACKGROUND: Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denud

  3. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    Directory of Open Access Journals (Sweden)

    Brouwer Oebele F

    2008-03-01

    Full Text Available Abstract Background Fetal spina bifida aperta (SBA is characterized by a spinal meningomyelocele (MMC and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA with MMC and 6 fetuses with normal cerebral development (22–41 week GA were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US. After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation

  4. A case report of pulsatile tinnitus as a symptom of brain arteriovenous malformation

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    M. Sayadnasiri

    2015-12-01

    Full Text Available Pulsatile tinnitus is the result of blood flow related sounds transmitted to the inner ear and coincides with heartbeat. Although pulsatile tinnitus is a rare entity, this symptom is most often indicative of a serious underlying disease in central nervous system. Unfortunately, this symptom is often not properly assessed by clinician that leads to delayed diagnosis of underlying brain pathology. In this report, a patient is presented with chief complaint of tinnitus that had many medical visits for 2 years. Finally, a cerebral vascular malformation was diagnosed with regards to physical examination and neuroimaging findings.

  5. Abordagem anestésica de grávida com malformação arteriovenosa cerebral e hemorragia subaracnoidea durante a gravidez: relato de caso Abordaje anestésico de embarazada con malformación arteriovenosa cerebral y hemorragia subaracnoidea durante el embarazo: relato de caso Anesthetic approach of pregnant woman with cerebral arteriovenous malformation and subarachnoid hemorrhage during pregnancy: case report

    Directory of Open Access Journals (Sweden)

    Catarina Santos Carvalho

    2013-04-01

    de 39 semanas, sana antes del embarazo, con antecedentes de HSA a las 22 semanas de gestación que se manifestó por medio de cefaleas, vómitos y mareos, sin la pérdida de la consciencia u otros déficits a la hora de su entrada en el servicio de urgencia. La resonancia magnética (RM arrojó MAV frontal izquierda. Después de un breve período de ingreso para la estabilización y el diagnóstico, se decidió mantener el embarazo y el acompañamiento ambulatorio multidisciplinario por neurocirugía y obstetricia en consulta de alto riesgo. Se optó por realizar la cesárea electiva a las 39 semanas bajo anestesia epidural lumbar. En el intraoperatorio ocurrió un episodio de hipotensión que fue rápidamente revertido con fenilefrina. El Índice de Apgar del recién nacido fue de 10/10. El catéter epidural fue usado para la analgesia postoperatoria, que también cursó sin intercurrencias. CONCLUSIONES: Son muy raros los casos publicados de abordaje anestésico de embarazadas con MAV sintomáticas. Todas las decisiones tomadas por el equipo multidisciplinario, desde optar por continuar con el embarazo, hasta el momento ideal para intervenir la MAV, pasando por el tipo de anestesia y analgesia, fueron sopesadas en función del riesgo de daño cerebral. Desde el punto de vista anestésico, los autores enfatizan la necesidad de estabilidad hemodinámica.BACKGROUND AND OBJECTIVES: Subarachnoid hemorrhage (SAH during pregnancy is a rare event, and about half the cases are due to arteriovenous malformations (AVM. The authors describe the anesthetic approach of a 39 week pregnant patient scheduled for cesarean section, with a history of SAH due to AVM at 22 week gestation. CASE REPORT: 39 week pregnant patient, healthy prior to pregnancy, with a history of SAH at 22 week gestation, manifested by headache, vomiting, and dizziness without loss of consciousness or other deficits on admission to the emergency room. Magnetic resonance imaging (MRI revealed a left frontal AVM

  6. [Macro- and microscopic systematization of cerebral cortex malformations in children].

    Science.gov (United States)

    Milovanov, A P; Milovanova, O A

    2011-01-01

    For the first time in pediatric pathologicoanatomic practice the complete systematization of cerebral cortex malformations is represented. Organ, macroscopic forms: microencephaly, macroencephaly, micropolygyria, pachygyria, schizencephaly, porencephaly, lissencephaly. Histic microdysgenesis of cortex: type I includes isolated abnormalities such as radial (IA) and tangential (I B) subtypes of cortical dislamination; type II includes sublocal cortical dislamination with immature dysmorphic neurons (II A) and balloon cells (II B); type III are the combination focal cortical dysplasia with tuberous sclerosis of the hippocampus (III A), tumors (III B) and malformations of vessels, traumatic and hypoxic disorders (III C). Band heterotopias. Subependimal nodular heterotopias. Tuberous sclerosis. Cellular typification of cortical dysplasia: immature neurons and balloon cells.

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

    Energy Technology Data Exchange (ETDEWEB)

    Orlowski, Piotr; Noble, Alison [University of Oxford, Institute of Biomedical Engineering, Department of Engineering Science, Oxford (United Kingdom); Mahmud, Imran; Kamran, Mudassar; Byrne, James V. [University of Oxford, John Radcliffe Hospital, Nuffield Department of Surgical Sciences, Oxford (United Kingdom); Summers, Paul [University of Oxford, John Radcliffe Hospital, Nuffield Department of Surgical Sciences, Oxford (United Kingdom); University of Modena and Reggio Emilia, Department of Biomedical, Metabolic and Neural Sciences, Modena (Italy); Ventikos, Yiannis [University College London, Department of Mechanical Engineering, London (United Kingdom)

    2014-03-15

    There is currently no standardised approach to arteriovenous malformation (AVM) reporting. Existing AVM classification systems focuses on angioarchitectural features and omit haemodynamic, anatomical and topological parameters intuitively used by therapists. We introduce a symbolic vocabulary to represent the state of an AVM of the brain at different stages of treatment. The vocabulary encompasses the main anatomic and haemodynamic features of interest in treatment planning and provides shorthand symbols to represent the interventions themselves in a schematic representation. The method was presented to 50 neuroradiologists from14 countries during a workshop and graded 7.34 ± 1.92 out of ten for its usefulness as means of standardising and facilitating communication between clinicians and allowing comparisons between AVM cases. Feedback from the survey was used to revise the method and improve its completeness. For an AVM test case, participants were asked to produce a conventional written report and subsequently a diagrammatic report. The two required, on average, 6.19 ± 2.05 and 5.09 ± 3.01 min, respectively. Eighteen participants said that producing the diagram changed the way they thought about the AVM test case. Introduced into routine practice, the diagrams would represent a step towards a standardised approach to AVM reporting with consequent benefits for comparative analysis and communication as well as for identifying best treatment strategies. (orig.)

  8. Concomitant pulmonary arteriovenous and inferior vena cava malformations. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hawass, N.D.; Kolawole, T.M.; Badawi, M.G.

    1988-05-01

    A case of pulmonary arterio-venous malformation (AVM) presenting with dyspnoea diagnosed by computer tomography (CT) and pulmonary angiography is reported. Venous anomalies in the form of agenesis of the iliac veins and the inferior vena cava (IVC), were discovered through femoral venograms performed after technical difficulties were encountered at pulmonary angiography performed via the femoral route. These venous anomalies co-existed with normal but dilated azygos and hemiazygos systems, and with the azygos appearing as a right hilar mass lesion which showed the 'candy cane sign' on the lateral view on venography. A dilated varix at the confluence of the right renal vein and the IVC collaterals was also noted. This association of a pulmonary AVM and IVC agenesis has not been previously reported in the literature. The embryogenesis of the inferior vena cava and the azygos system is presented in an attempt to explain the rare association of these anomalies. The various techniques of diagnosing the disease entities, expecially by CT, are discussed. The rare co-existence of pulmonary AVM and the absence of the IVC is emphasized.

  9. Comparison of 2 approaches for determining the natural history risk of brain arteriovenous malformation rupture.

    Science.gov (United States)

    Kim, Helen; McCulloch, Charles E; Johnston, S Claiborne; Lawton, Michael T; Sidney, Stephen; Young, William L

    2010-06-15

    Estimating risk of intracranial hemorrhage (ICH) for patients with unruptured brain arteriovenous malformations (AVMs) in the natural course is essential for assessing risks and benefits of treatment. Traditionally, the survival period starts at the time of diagnosis and ends at ICH, but most patients are quickly censored because of treatment. Alternatively, a survival period from birth to first ICH, censoring at the date of diagnosis, has been proposed. The authors quantitatively compared these 2 timelines using survival analysis in 1,581 Northern California brain AVM patients (2000-2007). Time-shift analysis of the birth-to-diagnosis timeline and maximum pseudolikelihood identified the point at which the 2 survival curves overlapped; the 95% confidence interval was determined using bootstrapping. Annual ICH rates per 100 patient-years were similar for both the birth-to-diagnosis (1.27, 95% confidence interval (CI): 1.18, 1.36) and the diagnosis-to-ICH (1.17, 95% CI: 0.89, 1.53) timelines, despite differences in curve morphology. Shifting the birth-to-diagnosis timeline an optimal amount (10.3 years, 95% CI: 3.3, 17.4) resulted in similar ICH survival curves (P = 0.979). These results suggest that the unconventional birth-to-diagnosis approach can be used to analyze risk factors for natural history risk in unruptured brain AVM patients, providing greater statistical power. The data also suggest a biologic change around age 10 years influencing ICH rate.

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

    Science.gov (United States)

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

    2014-11-01

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

  11. A case report of congenital umbilical arteriovenous malformation complicated with liver failure after surgical excision

    Science.gov (United States)

    Han, Ji-Won; Kim, Hyun-Young; Jung, Sung-Eun

    2017-01-01

    Abstract Rationale: Few case reports of umbilical arteriovenous malformation (AVM) have been reported. Herein, we report a neonatal case of umbilical AVM who underwent liver failure after surgical excision. Patient concerns: The patient was a girl delivered at a gestational age of 39+5 weeks showing cyanosis and heart murmur. Diagnoses: Cardiac echography, abdominal ultrasonography (USG), and computed tomography revealed suspecting the umbilical AVM. Interventions: On the eighth day after birth, because of the aggravation of heart failure, emergency surgery for excision of umbilical AVM was performed. Outcomes: In postoperative state, worsened laboratory test of liver function and coagulopathy indicated the liver failure. Abdominal USG revealed that the portal vein (PV) flow primarily occurred from the left PV to the inferior vena cava via ductus venosus and coarse hepatic echogenicity. After conservative management, laboratory findings of liver function and the flow direction of the left PV were normal, as demonstrated by abdominal USG within 50th postoperative day. Lessons: Careful preoperative evaluation of an AVM of a large size with significant blood flow should be performed, and the possibility of liver failure after surgery should always be considered. PMID:28178121

  12. Gamma knife radiosurgery for arteriovenous malformations located in the sensorimotor cortex

    Energy Technology Data Exchange (ETDEWEB)

    Yamanaka, Kazuhiro; Iwai, Yoshiyasu; Komiyama, Masaki; Nakajima, Hideki; Yasui, Toshihiro; Nishikawa, Misao; Sakamoto, Hiroaki; Morikawa, Toshie [Osaka City General Hospital (Japan)

    2002-05-01

    The goal of this study was to define treatment results of gamma knife radiosurgery (GKS) for arteriovenous malformation (AVM) located in the sensorimotor cortex. We analyzed 27 patients followed up for at least 12 months. The onset of AVM was convulsion 10, clinical hemorrhage 7, neurological deficits 4, and headache 3. The mean diameter of the AVM nidus was 22.2 mm (range 8.5-33.6 mm) and mean volume was 7.4 ml (range 0.32-19.9 ml). According to the Spetzler-Martin scale, the AVMs were Grade II in 13, Grade III in 10, and Grade IV in 4 of the patients. GKS was performed with a mean dose of 19.0 Gy (range 14-25 Gy) to the margin of the nidus. The mean follow-up period was 29 months (range 12-72 months). Six AVMs showed complete obliteration angiographically and 5 AVMs showed obliteration on magnetic resonance image. Sixteen AVMs showed nidus shrinkage. Eight (53%) of 15 patients followed up for more than 24 months showed obliteration. Complications consisted of 32 (11.5%) of 27 patients with evidence of radiation injury to the brain parenchyma. Symptoms of slight hemiparesis resolved completely in all patients within several months. (author)

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

    Science.gov (United States)

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

    2015-01-01

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

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

    OpenAIRE

    Beslow, Lauren A.; Jordan, Lori C

    2010-01-01

    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 ar...

  15. Late clinical and radiological complications of stereotactical radiosurgery of arteriovenous malformations of the brain

    Energy Technology Data Exchange (ETDEWEB)

    Parkhutik, Vera [Hospital Universitario la Fe, Department of Neurology, Valencia (Spain); Universidad Autonoma de Barcelona, PhD Program of the Department of Medicine, Barcelona (Spain); Lago, Aida; Vazquez, Juan Francisco; Tembl, Jose Ignacio [Hospital Universitario la Fe, Department of Neurology, Valencia (Spain); Aparici, Fernando; Guillen, Lourdes; Mainar, Esperanza; Vazquez, Victor [Hospital Universitario la Fe, Department of Neuroradiology, Valencia (Spain)

    2013-04-15

    Post-radiation injury of patients with brain arteriovenous malformations (AVM) include blood-brain barrier breakdown (BBBB), edema, and necrosis. Prevalence, clinical relevance, and response to treatment are poorly known. We present a series of consecutive brain AVM treated with stereotactic radiosurgery describing the appearance of radiation injury and clinical complications. Consecutive patients with annual clinical and radiological follow-up (median length 63 months). Edema and BBBB were classified in four groups (minimal, perilesional, moderate, or severe), and noted together with necrosis. Clinical symptoms of interest were intracranial hypertension, new neurological deficits, new seizures, and brain hemorrhages. One hundred two cases, median age 34 years, 52 % male. Median irradiated volume 3.8 cc, dose to the margin of the nidus 18.5 Gy. Nineteen patients underwent a second radiosurgery. Only 42.2 % patients remained free from radiation injury. Edema was found in 43.1 %, blood-brain barrier breakdown in 20.6 %, necrosis in 6.9 %. Major injury (moderate or severe edema, moderate or severe BBBB, or necrosis) was found in 20 of 102 patients (19.6 %). AVM diameter >3 cm and second radiosurgery were independent predictors. Time to the worst imaging was 60 months. Patients with major radiation injury had a hazard ratio for appearance of focal deficits of 7.042 (p = 0.04), of intracranial hypertension 2.857 (p = 0.025), hemorrhage into occluded nidus 9.009 (p = 0.079), appearance of new seizures not significant. Major radiation injury is frequent and increases the risk of neurological complications. Its late appearance implies that current follow-up protocols need to be extended in time. (orig.)

  16. A genome-wide investigation of copy number variation in patients with sporadic brain arteriovenous malformation.

    Directory of Open Access Journals (Sweden)

    Nasrine Bendjilali

    Full Text Available BACKGROUND: Brain arteriovenous malformations (BAVM are clusters of abnormal blood vessels, with shunting of blood from the arterial to venous circulation and a high risk of rupture and intracranial hemorrhage. Most BAVMs are sporadic, but also occur in patients with Hereditary Hemorrhagic Telangiectasia, a Mendelian disorder caused by mutations in genes in the transforming growth factor beta (TGFβ signaling pathway. METHODS: To investigate whether copy number variations (CNVs contribute to risk of sporadic BAVM, we performed a genome-wide association study in 371 sporadic BAVM cases and 563 healthy controls, all Caucasian. Cases and controls were genotyped using the Affymetrix 6.0 array. CNVs were called using the PennCNV and Birdsuite algorithms and analyzed via segment-based and gene-based approaches. Common and rare CNVs were evaluated for association with BAVM. RESULTS: A CNV region on 1p36.13, containing the neuroblastoma breakpoint family, member 1 gene (NBPF1, was significantly enriched with duplications in BAVM cases compared to controls (P = 2.2×10(-9; NBPF1 was also significantly associated with BAVM in gene-based analysis using both PennCNV and Birdsuite. We experimentally validated the 1p36.13 duplication; however, the association did not replicate in an independent cohort of 184 sporadic BAVM cases and 182 controls (OR = 0.81, P = 0.8. Rare CNV analysis did not identify genes significantly associated with BAVM. CONCLUSION: We did not identify common CNVs associated with sporadic BAVM that replicated in an independent cohort. Replication in larger cohorts is required to elucidate the possible role of common or rare CNVs in BAVM pathogenesis.

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

    Science.gov (United States)

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

    2017-01-01

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

  18. Embolization of brain arteriovenous malformations with ethylene vinyl alcohol copolymer:technical aspects

    Institute of Scientific and Technical Information of China (English)

    GAO Kun; YANG Xin-jian; MU Shi-qing; LI You-xiang; ZHANG You-ping; L(U) Ming; WU Zhong-xue

    2009-01-01

    Background Endovascular therapy plays an important role in the treatment of brain arteriovenous malformations (BAVMs).Ethylene vinyl alcohol copolymer (Onyx) is a novel liquid embolic material.This study aimed to summarize our experience of using Onyx for embolization of BAVMs with the focus on embolization technique.Methods From September 2003 to November 2007,115 patients (43 women and 72 men,with a mean age of 29 years)with BAVMs were endovascularly treated with Onyx in our department.The following features of all AVMs were evaluated prior to treatment:type of nidus and shunt,draining veins,and feeding arteries.A total of 196 endovascular procedures were performed.Results The course of endovascular treatment was completed in 88 patients.Additional sessions were planned in 27 patients.Of the 88 patients,total occlusion was obtained in 23 patients (26.1%),near-total (>80% of the original volume) occlusion was obtained in 35 patients (39.8%) and partial occlusion (<80% of the original volume) was obtained in 30 patients (34.1%) using embolization as the sole therapeutic technique.Mean volume reduction was 72% (range 30%-100%) in 115 patients.Thirty four patients (38.6%,34/88) underwent radiosurgical treatment.Additional embolization sessions were planned in 27 patients.Complications occurred in 19 patients (16.5%,19/115),leading to death in one patient (mortality 0.9%) and permanent disabling in 3 patients (morbidity 2.6%).Conclusions Onyx was shown to be feasible and safe for embolization of BAVMs.Proper use of the Onyx injection technique largely improved the endovascular treatment of BAVMs.Large AVMs can be adequately reduced in size through the use of additional treatment.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2014-07-01

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

  20. Peripheral Arteriovenous Malformations with a Dominant Outflow Vein: Results of Ethanol Embolization

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Sung Ki; Do, Young Soo; Kim, Dong Ik; Kim, Young Wook; Shin, Sung Wook; Park, Kwang Bo; Ko, Justin Sang; Lee, Ae Ryoung; Choo, Sung Wook; Choo, In Wook [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2008-06-15

    To assess retrospectively the treatment results of ethanol embolization of peripheral arteriovenous malformations (AVMs) with a dominant outflow vein (DOV). Nineteen patients who had peripheral AVMs with a DOV were enrolled in this study (mean age, 29.7 years; range, 15 42 years). Fifty-one ethanol embolizations (mean, 2.7; range, 1 8) were performed by direct puncture (n = 29), the transarterial approach (n = 13), the transvenous approach (n = 5), or a combination of methods (n = 4) under general anesthesia. Coil and/or core-removed guide wire embolization of the DOV or another flow occlusion technique (i.e., use of an external pneumatic pressure cuff) to achieve vascular stasis were required in all patients during ethanol embolization. Clinical follow-up (mean, 22.2 months; range, 1 53 months) was performed for all patients, and imaging follow-up (mean, 22.1 months; range, 2 53 months) from the last treatment session was performed for 14 patients. The therapeutic outcome (cure, improvement, no change, or aggravation) was assessed according to the clinical response and the degree of devascularization at angiography. Ethanol embolization was considered as an effective procedure in all patients. Thirteen (68%) of 19 patients were cured and six displayed improvement. Three of six patients with improvement needed further treatment sessions for residual AVMs. Four patients (21%) experienced a total of eight complications. Five complications (three events of a distal embolism and one event each of a urinary bladder necrosis and a brain infarct related to the accidental cannulation of the common carotid artery during insertion of the Swan-Ganz catheter) were major and three complications (skin necrosis) were minor. Peripheral AVMs with a DOV can be effectively treated with a high cure rate by the use of ethanol embolization alone or in conjunction with the use of coil and/or core-removed guide wire embolization.

  1. Early neonatal complications from pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: case report and review of the literature.

    Science.gov (United States)

    Gludovacz, Karoy; Vlasselaer, Jozef; Mesens, Tinne; Van Holsbeke, Caroline; Van Robays, Johan; Gyselaers, Wilfried

    2012-08-01

    Hereditary hemorrhagic telangiectasia (HHT) is a rare but life-threatening disease characterized by multi system telangiectasias and arteriovenous malformations (AVM). Complications in adults have been reported extensively, but neonatal (NN) complications have only been published in incidental case reports. In this paper, we present a literature review on NN pulmonary AVM related to HHT, following our own experience with a NN death due to this disease. As prenatal diagnosis of pulmonary AVM is feasible, we recommend that a family history of HHT should be an indication for expertise prenatal anomaly scanning, in order to organise optimal NN support at birth.

  2. Direct Puncture Embolization of Scalp Arteriovenous Malformation in a Patient with Severe Hemophilia A: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Mi; Kim, Eui Jong [Dept. of Radiology, Kyung Hee University Hospital, Kyung Hee University Graduate School of Medicine, Seoul (Korea, Republic of); Park, Bong Jin [Dept. of Neurosurgery, Kyung Hee University Hospital, Kyung Hee University Graduate School of Medicine, Seoul (Korea, Republic of); Kim, Keon Ha [Dept. of Radiology, Samsug Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2011-09-15

    We present a case of scalp arteriovenous malformation (AVM) in a patient with severe hemophilia A. The 22-year-old man presented with a pulsatile right parietal scalp mass. Digital subtraction angiography revealed an AVM in the right parietal scalp, supplied by superficial temporal and occipital arteries that drained into multiple venous structures. We successfully performed direct puncture embolization followed by surgical resection of the scalp AVM in conjunction with supplemental infusion of coagulation factor VIII before, during and after the embolization and the operation.

  3. Surgical Management of Combined Intramedullary Arteriovenous Malformation and Perimedullary Arteriovenous Fistula within the Hybrid Operating Room after Five Years of Performing Focus Fractionated Radiotherapy: Case Report

    Science.gov (United States)

    GEKKA, Masayuki; SEKI, Toshitaka; HIDA, Kazutoshi; OSANAI, Toshiya; HOUKIN, Kiyohiro

    2014-01-01

    Perimedullary arteriovenous fistula (AVF) shunts occur on the spinal cord surface and can be treated surgically or by endovascular embolization. In contrast, the nidus of an intramedullary arteriovenous malformation (AVM) is located in the spinal cord and is difficult to treat surgically or by endovascular techniques. The benefits of radiotherapy for treating intramedullary AVM have been published, but are anecdotal and consist largely of case reports. We present a case of combined cervical intramedullary AVM and perimedullary AVF which received surgical treatment within a hybrid operating room (OR) after 5 years of focus fractionated radiotherapy. A 37-year-old male presented with stepwise worsening myelopathy. Magnetic resonance imaging and spinal angiography revealed intramedullary AVM and perimedullary AVF at the C3 to C5 levels. In order to reduce nidus size and blood flow, we first performed focal fractionated radiotherapy. Five years later, the lesion volume was reduced. Following this, direct surgery was performed by an anterior approach using corpectomy in the hybrid OR. The spinal cord was monitored by motor-evoked potential throughout the surgery. Complete obliteration of the fistulous connection was confirmed by intraoperative indocyanine green video-angiography and intraoperative angiography, preserving the anterior spinal artery. We conclude that surgical treatment following focal fractionated radiotherapy may become one strategy for patients who are initially deemed ineligible for endovascular embolization and surgical treatment. Furthermore, the hybrid OR enables safe and precise treatment for spinal vascular disorders in the fields of endovascular treatment and neurosurgery. PMID:25367581

  4. Surgical management of combined intramedullary arteriovenous malformation and perimedullary arteriovenous fistula within the hybrid operating room after five years of performing focus fractionated radiotherapy: case report.

    Science.gov (United States)

    Gekka, Masayuki; Seki, Toshitaka; Hida, Kazutoshi; Osanai, Toshiya; Houkin, Kiyohiro

    2014-01-01

    Perimedullary arteriovenous fistula (AVF) shunts occur on the spinal cord surface and can be treated surgically or by endovascular embolization. In contrast, the nidus of an intramedullary arteriovenous malformation (AVM) is located in the spinal cord and is difficult to treat surgically or by endovascular techniques. The benefits of radiotherapy for treating intramedullary AVM have been published, but are anecdotal and consist largely of case reports. We present a case of combined cervical intramedullary AVM and perimedullary AVF which received surgical treatment within a hybrid operating room (OR) after 5 years of focus fractionated radiotherapy. A 37-year-old male presented with stepwise worsening myelopathy. Magnetic resonance imaging and spinal angiography revealed intramedullary AVM and perimedullary AVF at the C3 to C5 levels. In order to reduce nidus size and blood flow, we first performed focal fractionated radiotherapy. Five years later, the lesion volume was reduced. Following this, direct surgery was performed by an anterior approach using corpectomy in the hybrid OR. The spinal cord was monitored by motor-evoked potential throughout the surgery. Complete obliteration of the fistulous connection was confirmed by intraoperative indocyanine green video-angiography and intraoperative angiography, preserving the anterior spinal artery. We conclude that surgical treatment following focal fractionated radiotherapy may become one strategy for patients who are initially deemed ineligible for endovascular embolization and surgical treatment. Furthermore, the hybrid OR enables safe and precise treatment for spinal vascular disorders in the fields of endovascular treatment and neurosurgery.

  5. Radiation-induced changes of brain tissue after radiosurgery in patients with arteriovenous malformations: dose/volume-response relations

    Energy Technology Data Exchange (ETDEWEB)

    Levegruen, S.; Schlegel, W. [Dept. of Medical Physics, German Cancer Research Center (DKFZ), Heidelberg (Germany); Hof, H.; Debus, J. [Dept. of Radiation Oncology, German Cancer Research Center (DKFZ), Heidelberg (Germany); Essig, M. [Dept. of Radiology, German Cancer Research Center (DKFZ), Heidelberg (Germany)

    2004-12-01

    Purpose: to evaluate late radiation effects in the brain after radiosurgery of patients with cerebral arteriovenous malformations (AVMs) and to quantify dose/volume-response relations for radiation-induced changes of brain tissue identified on follow-up neuroimaging. Patients and methods: data from 73 AVM patients who had stereotactic linac radiosurgery at DKFZ (German Cancer Research Center), Heidelberg, Germany, were retrospectively analyzed. The endpoint of radiation-induced changes of brain tissue on follow-up magnetic resonance (MR) neuroimaging (i.e., edema and blood-brain-barrier breakdown [BBBB]) was evaluated. Each endpoint was further differentiated into three levels with respect to the extent of the image change (small, intermediate, and large). A previous analysis of the data found correlation of the endpoints with several dose/volume variables (DV) derived from each patient's dose distribution in the brain, including the mean dose in a volume of 20 cm{sup 3} (Dmean20) and the absolute brain volume (including the AVM target) receiving a dose of at least 12 Gy (V12). To quantify dose/volume-response relations, patients were ranked according to DV (i.e., Dmean20 and V12) and classified into four groups of equal size. For each group, the actuarial rates of developing the considered endpoints within 2.5 years after radiosurgery were determined from Kaplan-Meier estimates. The dose/volume-response curves were fitted with a sigmoid-shape logistic function and characterized by DV{sub 50}, the dose for a 50% incidence, and the slope parameter k. Results: dose/volume-response relations, based on two alternative, but correlated, dose distribution variables that are a function of both dose and volume, were observed for radiation-induced changes of brain tissue. DV{sub 50} values of fitted dose/volume-response curves for tissue changes of large extent (e.g., V12{sub 50} = 22.0 {+-} 2.6 cm{sup 3} and Dmean20{sub 50} = 17.8 {+-} 2.0 Gy for the combined endpoint

  6. Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment

    Directory of Open Access Journals (Sweden)

    Nikolaos Mouchtouris

    2015-01-01

    Full Text Available Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment—microsurgical resection, stereotactic radiosurgery, and conservative management—depending on the lesion characteristics.

  7. Brain arteriovenous malformation multiplicity predicts diagnosis of Hereditary Hemorrhagic Telangiectasia: Quantitative assessment

    Science.gov (United States)

    Bharatha, Aditya; Faughnan, Marie E.; Kim, Helen; Pourmohamad, Tony; Krings, Timo; Bayrak-Toydemir, Pinar; Pawlikowska, Ludmila; McCulloch, Charles E.; Lawton, Michael T.; Dowd, Christopher F.; Young, William L.; Terbrugge, Karel G.

    2013-01-01

    Purpose To quantitatively estimate the relationship between multiplicity of brain arteriovenous malformations (bAVMs) and the diagnosis of hereditary hemorrhagic telangiectasia (HHT). Methods We combined databases from two large North American bAVM referral centers, including demographics, clinical presentation and angiographic characteristics, and compared HHT patients with non-HHT patients. Logistic regression analysis was performed to quantify the association between bAVM multiplicity and odds of HHT diagnosis. Sensitivity, specificity, positive and negative predictive value (PPV, NPV), and positive and negative likelihood ratios (LR) were calculated to determine accuracy of bAVM multiplicity for screening HHT. Results Prevalence of HHT was 2.8% in the combined group. bAVM multiplicity was present in 39% of HHT patients and was highly associated with diagnosis of HHT in univariate (OR=83, 95% CI:40–173, P<0.0001) and multivariable (OR=87, 95% CI: 38–195, P<0.001) models, adjusting for age at presentation (P=0.013), non-symptomatic presentation (P=0.029) and cohort site (P=0.021). bAVM multiplicity alone was associated with high specificity (99.2%, 95%CI: 98.7–99.6%) and NPV (98.3%, 95% CI: 97.6–98.8%), and low sensitivity (39.3%, 95% CI: 26.5–53.2%) and PPV (59.5%, 95% CI: 42.1–75.2%). Positive and negative LR was 51 and 0.61, respectively, for diagnosis of HHT. HHT bAVMs were also more often smaller in size (<3 cm), non-eloquent in location and associated with superficial venous drainage, compared to non-HHT bAVMs. Conclusion Multiplicity of bAVMs is highly predictive of the diagnosis of HHT. The presence of multiple bAVMs should alert the clinician to the high probability of HHT and lead to comprehensive investigation for this diagnosis. PMID:22034007

  8. Dosimetric measurements of an n-butyl cyanoacrylate embolization material for arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Labby, Zacariah E., E-mail: zelabby@humonc.wisc.edu [Department of Human Oncology, University of Wisconsin–Madison, 600 Highland Avenue, Madison, Wisconsin 53792 (United States); Chaudhary, Neeraj [Division of Neurointerventional Radiology, Departments of Radiology and Neurosurgery, University of Michigan Hospital and Health Systems, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109 (United States); Gemmete, Joseph J. [Division of Neurointerventional Radiology, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Michigan Hospital and Health Systems, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109 (United States); Pandey, Aditya S. [Department of Neurosurgery, University of Michigan Hospital and Health Systems, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109 (United States); Roberts, Donald A. [Radiation Physics Division, Department of Radiation Oncology, University of Michigan Hospital and Health Systems, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109 (United States)

    2015-04-15

    Purpose: The therapeutic regimen for cranial arteriovenous malformations often involves both stereotactic radiosurgery and endovascular embolization. Embolization agents may contain tantalum or other contrast agents to assist the neurointerventionalists, leading to concerns regarding the dosimetric effects of these agents. This study investigated dosimetric properties of n-butyl cyanoacrylate (n-BCA) plus lipiodol with and without tantalum powder. Methods: The embolization agents were provided cured from the manufacturer with and without added tantalum. Attenuation measurements were made for the samples and compared to the attenuation of a solid water substitute using a 6 MV photon beam. Effective linear attenuation coefficients (ELAC) were derived from attenuation measurements made using a portal imager and derived sample thickness maps projected in an identical geometry. Probable dosimetric errors for calculations in which the embolized regions are overridden with the properties of water were calculated using the ELAC values. Interface effects were investigated using a parallel plate ion chamber placed at set distances below fixed samples. Finally, Hounsfield units (HU) were measured using a stereotactic radiosurgery CT protocol, and more appropriate HU values were derived from the ELAC results and the CT scanner’s HU calibration curve. Results: The ELAC was 0.0516 ± 0.0063 cm{sup −1} and 0.0580 ± 0.0091 cm{sup −1} for n-BCA without and with tantalum, respectively, compared to 0.0487 ± 0.0009 cm{sup −1} for the water substitute. Dose calculations with the embolized region set to be water equivalent in the treatment planning system would result in errors of −0.29% and −0.93% per cm thickness of n-BCA without and with tantalum, respectively. Interface effects compared to water were small in magnitude and limited in distance for both embolization materials. CT values at 120 kVp were 2082 and 2358 HU for n-BCA without and with tantalum, respectively

  9. Intestinal lymphangiectasia associated with chylothorax and multiple lower extremity arteriovenous malformation:A case report and literature review

    Institute of Scientific and Technical Information of China (English)

    Haojie Li; Daoyu Hu; Lili Liang; Yao Hu; Zhen Li

    2015-01-01

    Intestinal lymphangiectasia (IL) is an uncommon protein losing enteropathy, characterized by smal intes-tinal mucosa or serosa lymphangiectasia and intestine lymph loss. Currently, IL is a very rare disease in children or adults, with typical clinical symptoms including hypoalbuminemia, absolute lymphocyte reduc-tion, ascites, edema, etc. We report a case of an adult with intestinal lymphatic ectasia accompanied by chylothorax and multiply arteriovenous malformations of the hip and lower extremity. CT and MRI revealed difuse edema and thickening of the smal intestine, accompanied by splenomegaly and pleural efusion. Extensive nodularity of lower ileum and the ileocecal region could be seen during intestinal endoscopy. Finaly, smal intestinal lamina propria lymphangiectasis was confirmed by pathological examination. To raise awareness of the disease, here we compare our case and those previously reported, and discuss the diagnosis and management of IL.

  10. Renal Cell Carcinoma Initially Presenting as an Arteriovenous Malformation: A Case Presentation and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Samuel Volin

    2013-01-01

    Full Text Available We describe a case of a patient who presented with hematuria and was diagnosed with a renal arteriovenous malformation (AVM. Transcatheter arterial embolization subsequently was performed on this lesion multiple times. Follow-up imaging demonstrated that the AVM was masking an underlying, rapidly growing renal cell carcinoma (RCC. We describe the pathological and radiographic characteristics of AVMs and RCC. We describe the strengths and weaknesses of computed tomography (CT and magnetic resonance imaging (MRI to detect and characterize RCC and AVM. We recommend initial and follow-up MR imaging in patients with an AVM to establish a baseline, monitor treatment response, and survey lesions for underlying and obscured malignancy.

  11. Concurrent Pancreatic Head and Tail Arteriovenous Malformations in a 40-Year-Old Gentleman: The First Published Report

    Directory of Open Access Journals (Sweden)

    Asma Alnajjar

    2014-05-01

    Full Text Available Context Pancreatic arteriovenous malformations (AVMs are uncommon in the gastrointestinal tract. Less than 100 cases have been identified in the medical literature. Approximately 10% of all pancreatic AVMs are sporadic. Case Report Herein, we report the first documented case of sporadic concurrent pancreatic head and tail AVMs in a 40-year-old gentleman who presented with a 10-day history of epigastric pain and one episode of hematemesis. Patient denied any history of traumatic incidents, cigarette smoking, alcohol abuse, chronic gastric/duodenal ulcer, chronic pancreatitis, chronic hepatic disease, difficulty swallowing, respiratory compromise, or weight loss. Physical examination and laboratory results were unremarkable. Contrast-enhanced computed tomography scan showed two hypervascular masses involving the pancreatic head and tail. The celiac trunk angiogram showed proliferating vascular networks involving the pancreatic head and tail. The superior mesenteric angiogram demonstrated significant vascular contribution to the pancreatic head arteriovenous malformation only. Due to the extreme locations of pancreatic AVMs in the head and tail, surgical resection of both lesions(leaving behind the normal pancreatic body was not possible. Instead, patient underwent intraoperative irradiation therapy (IORT. During the procedure, patient was surgically operated to retract healthy organs/tissues, and then a single concentrated dose of radiation therapy was precisely applied to both pancreatic head and tail AVM lesions. Patient had an uneventful postoperative recovery and was discharged home on the second postoperative day in stable condition. The patient is to be seen in clinic in a 4-month-period during which patient will be completing a 12-month period of postoperative IORT. Conclusion This is the first documented case of sporadic concurrent pancreatic head and tail AVMs. Angiography is the gold standard diagnostic modality.

  12. Development of a de novo arteriovenous malformation after bilateral revascularization surgery in a child with moyamoya disease.

    Science.gov (United States)

    Fujimura, Miki; Kimura, Naoto; Ezura, Masayuki; Niizuma, Kuniyasu; Uenohara, Hiroshi; Tominaga, Teiji

    2014-06-01

    The development of a de novo arteriovenous malformation (AVM) in patients with moyamoya disease is extremely rare. A 14-year-old girl developed an AVM in the right occipital lobe during the 4-year postoperative period following successful bilateral revascularization surgeries. She suffered a transient ischemic attack with hemodynamic compromise of the bilateral hemispheres at the age of 10 years. Results of an initial examination by 1.5-T MRI and MR angiography satisfied the diagnostic criteria of moyamoya disease but failed to detect any vascular malformation. Bilateral direct and indirect revascularization surgeries in the anterior circulation relieved her symptoms, and she underwent MRI and MR angiography follow-up every year after surgery. Serial T2-weighted MRI revealed the gradual appearance of flow voids in the right occipital lobe during the follow-up period. Magnetic resonance angiography ultimately indicated the development of an AVM 4 years after these surgeries when catheter angiography confirmed the diagnosis of an AVM in the right occipital lobe. The AVM remained asymptomatic, and the patient remained free of cerebrovascular events during the time she was observed by the authors. Acquired AVM in moyamoya disease is extremely rare, with only 3 pediatric cases including the present case being reported in the literature. The development of a de novo AVM in a postoperative patient with moyamoya disease appears to be unique, and this case may provide insight into the dynamic pathology of AVMs.

  13. Arteriovenous Malformations and Other Vascular Lesions of the Central Nervous System

    Science.gov (United States)

    ... Malformations and Other Vascular Lesions of the Central Nervous System Fact Sheet Table of Contents (click to jump ... other types of vascular lesions affect the central nervous system? What causes vascular lesions? How are AVMs and ...

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

    DEFF Research Database (Denmark)

    Girard, Romuald; Fam, Maged D; Zeineddine, Hussein A;

    2017-01-01

    OBJECTIVE Vascular permeability and iron leakage are central features of cerebral cavernous malformation (CCM) pathogenesis. The authors aimed to correlate prospective clinical behavior of CCM lesions with longitudinal changes in biomarkers of dynamic contrast-enhanced quantitative permeability (...

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

    NARCIS (Netherlands)

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

    2016-01-01

    Cerebral cavernous malformations (CCMs) are prevalent slow-flow vascular lesions which harbour the risk to develop intracranial haemorrhages, focal neurological deficits, and epileptic seizures. Autosomal dominantly inherited CCMs were found to be associated with heterozygous inactivating mutations

  16. An arteriovenous malformation in the suprapatellar fat pad of the knee associated with Klippel-Trenaunay-Weber syndrome: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Mi Hyun; Kwon, Soon Tae; Shin, Byung Seok; Kim, Young Mo [Chungnam National University Hospital, Daejeon (Korea, Republic of)

    2006-01-15

    Klippel-Trenaunay-Weber syndrome (KTWS) is a vascular disorder that has significant arteriovenous malformation (AVM). We report a case of an AVM in the suprapatellar fat pad of the knee in a patient with the characteristic manifestations of KTWS, including cutaneous hemangioma, limb hypertrophy, and varicose veins. Magnetic resonance imaging, color Doppler sonography, and subsequent angiography demonstrated an AVM in the supra-patellar fat pad of the right knee causing painful swelling of the knee.

  17. Unilateral moyamoya disease associated with cerebellar arteriovenous malformation:one case report

    Institute of Scientific and Technical Information of China (English)

    DENG Zheng-hai; WANG Shuo; LI Zhong; ZHAO Ji-zong

    2008-01-01

    @@ As a non-atherosclerotic chronic cerebral vasculopathy,moyamoya disease is characterized by progressive steno-occlusion of the arteries of the circle of Willis with a developed collateral vascular network mainly at the cerebral base.1 And it is named moyamoya disease because of a characteristic "puff-of-smoke" angiographic appearance.

  18. Endoscopic and Angiographic Diagnosis and Management of a Gastric Arteriovenous Malformation

    Directory of Open Access Journals (Sweden)

    Jeffrey B. McCrary

    2014-04-01

    Conclusions: We propose that these two minimally invasive technologies can be used to manage AVM in the gut: endoscopic therapy to control luminal bleeding and interventional radiology to define the full extent of the malformation and to decrease arterial pressure and flow to the point that hemostasis can occur, without creating symptomatic ischemia.

  19. Cerebral cavernous malformations: clinical insights from genetic studies.

    Science.gov (United States)

    Mindea, Stefan A; Yang, Benson P; Shenkar, Robert; Bendok, Bernard; Batjer, H Hunt; Awad, Issam A

    2006-07-15

    Familial disease is responsible for one third to one half of cerebral cavernous malformation (CCM) cases presenting to clinical attention. Much has been learned in the past decade about the genetics of these cases, which are all inherited in an autosomal dominant pattern, at three known chromosome loci. Unique features of inherited CCMs in Hispanic-Americans of Mexican descent have been described. The respective genes for each locus have been identified and preliminary observations on disease pathways and mechanisms are coming to light, including possible explanations for selectivity of neural milieu and relationships to endothelial layer abnormalities. Mechanisms of lesion genesis in cases of genetic predisposition are being investigated, with evidence to support a two-hit model emerging from somatic mutation screening of the lesions themselves and from lesion formation in transgenic murine models of the disease. Other information on potential inflammatory factors has emerged from differential gene expression studies. Unique phenotypic features of solitary versus familial cases have emerged: different associations with venous developmental anomaly and the exceptionally high penetrance rates that are found in inherited cases when high-sensitivity screening is performed with gradient echo magnetic resonance imaging. This information has changed the landscape of screening and counseling for patients and their families, and promises to lead to the development of new tools for predicting, explaining, and modifying disease behavior.

  20. ADVANCED MAGNETIC RESONANCE IMAGING OF CEREBRAL CAVERNOUS MALFORMATIONS

    Science.gov (United States)

    Shenkar, Robert; Venkatasubramanian, Palamadai N.; Wyrwicz, Alice M.; Zhao, Jin-cheng; Shi, Changbin; Akers, Amy; Marchuk, Douglas A.; Awad, Issam A.

    2008-01-01

    Objective We sought to assess the appearance of cerebral cavernous malformations (CCMs) on magnetic resonance (MR) imaging in murine Ccm1 and Ccm2 gene knockout models, and to develop a technique of lesion localization for correlative pathobiologic studies Methods Brains from eighteen CCM mutant mice (Ccm1+/-Trp53-/- and Ccm2+/-Trp53-/-) and 28 controls were imaged by gradient recalled echo (T2*)-weighted MR at 4.7 T and 14.1 T in vivo and/or ex vivo. After MR imaging, the brains were removed and stained with hematoxylin and eosin and cells were laser microdissected for molecular biologic studies. Results T2*-weighted MR imaging of brains in vivo and ex vivo revealed lesions similar to human CCMs in mutant mice, but not in control animals. Stereotactic localization and hematoxylin and eosin-staining of correlative tissue sections confirmed lesion histology, and revealed other areas of dilated capillaries in the same brains. Some lesions were identified by MR imaging at 14.1 T, but not at 4.7 T. PCR amplification from Ccm1 and β-actin genes was demonstrated from nucleic acids extracted from laser microdissected lesional and perilesional cells. Conclusions The high field MR imaging techniques offer new opportunities for further investigation of disease pathogenesis in vivo, and the localization, staging and histobiologic dissection of lesions, including the presumed earliest stages of CCM lesion development. PMID:18981891

  1. Combined Intralesional Neodymium-Doped Yttrium Aluminium Garnet Laser and Intratumoral Ligation as Curative Treatment for Craniofacial Arteriovenous Malformations.

    Science.gov (United States)

    Rojvachiranonda, Nond; Lerdlum, Sukalaya; Mahatumarat, Charan

    2016-03-01

    Craniofacial arteriovenous malformation (AVM), although very rare, has been a very difficult problem to treat especially when it is large and involves important structures. Surgical resection often results in unacceptable complications but still not curative. At our institution, treatment by combined intralesional neodymium-doped yttrium aluminium garnet laser and intratumoral ligation has been successful in venous malformation. This minimally invasive technique was then applied to more challenging AVM on the head and neck. Disease control was studied using clinical parameters and magnetic resonance imaging.Four patients with moderate-to-severe (Schobinger 2-4) craniofacial AVM were treated by this technique from 2001 to 2011. Patient age ranged from 2 to 51 years (mean: 25 years). After 2 to 4 treatments and follow-up period of 1456 days, 3 (75%) were cured. One of them was infant with huge mass and secondary pulmonary hypertension. Clinical cure was achieved after 3 treatments without residual cardiovascular compromise. The other patient (25%) had cheek mass with intraorbital involvement. The authors did not treat periorbital lesion so as to avoid triggering intraorbital spreading. The rest of the cheek lesion was clinically and radiologically cured.Laser energy setting, ablative technique, and skin cooling are the main factors determining the success. Individualized laser settings and properly set endpoints can increase treatment effectiveness in shorter period. In conclusion, this minimally invasive technique was successful in curing AVM without complication. With more clinical study and development of soft tissue monitoring tools, it is possible that intralesional laser could become the treatment of choice for all cutaneous AVM.

  2. Massive hematuria due to a congenital renal arteriovenous malformation mimicking a renal pelvis tumor: a case report

    Directory of Open Access Journals (Sweden)

    Sountoulides P

    2008-05-01

    Full Text Available Abstract Introduction Congenital renal arteriovenous malformations (AVMs are very rare benign lesions. They are more common in women and rarely manifest in elderly people. In some cases they present with massive hematuria. Contemporary treatment consists of transcatheter selective arterial embolization which leads to resolution of the hematuria whilst preserving renal parenchyma. Case presentation A 72-year-old man, who was heavy smoker, presented with massive hematuria and flank pain. CT scan revealed a filling defect caused by a soft tissue mass in the renal pelvis, which initially led to the suspicion of a transitional cell carcinoma (TCC of the upper tract, in view of the patient's age and smoking habits. However a subsequent retrograde study could not depict any filling defect in the renal pelvis. Selective right renal arteriography confirmed the presence of a renal AVM by demonstrating abnormal arterial communication with a vein with early visualization of the venous system. At the same time successful selective transcatheter embolization of the lesion was performed. Conclusion This case highlights the importance of careful diagnostic work-up in the evaluation of upper tract hematuria. In the case presented, a congenital renal AVM proved to be the cause of massive upper tract hematuria and flank pain in spite of the initial evidence indicating the likely diagnosis of a renal pelvis tumor.

  3. A rare association of cerebral dural arteriovenous fistula with venous aneurysm and contralateral flow-related middle cerebral artery aneurysm.

    Science.gov (United States)

    Onu, David O; Hunn, Andrew W; Harle, Robin A

    2013-09-19

    The association of cerebral dural arteriovenous fistula (DAVF) and ipsilateral flow related aneurysm has infrequently been reported. We describe a male patient who presented with an acute haemorrhagic stroke and was found to have a large right fronto-parietal intra-parenchymal haemorrhage from the ruptured Borden type II DAVF in addition to a large venous aneurysm and a flow related intraosseous aneurysm of the contralateral middle meningeal artery (MMA) all clearly delineated by CT and DSA. He underwent emergency stereotactic evacuation of the intraparenchymal haemorrhage and successful surgical treatment of all the vascular lesions at the same time with residual neurological deficit. To our knowledge, this is the first such reported case. We discuss the challenging surgical treatment, emphasising the role of CT/DSA in management, and provide a literature review.

  4. The use of 4D-CTA in the diagnostic work-up of brain arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Willems, Peter W.A. [Toronto Western Hospital, UHN, Division of Neuroradiology, Department of Medical Imaging, Toronto, Ontario (Canada); Leiden University Medical Center, Department of Radiology, Leiden (Netherlands); Taeshineetanakul, Patamintita; Terbrugge, Karel G.; Krings, Timo [Toronto Western Hospital, UHN, Division of Neuroradiology, Department of Medical Imaging, Toronto, Ontario (Canada); Schenk, Barry; Brouwer, Patrick A. [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands)

    2012-02-15

    We aimed to evaluate the use of time-resolved whole-head CT angiography (4D-CTA) in patients with an untreated arteriovenous malformation of the brain (bAVM), as demonstrated by catheter angiography (DSA). Seventeen patients with a DSA-proven bAVM were enrolled. These were subjected to 4D-CTA imaging using a 320 detector row CT scanner. Using a standardized scoring sheet, all studies were analyzed by a panel of three readers. This panel was blind to the DSA results at the time of reading the 4D-CTA. 4D-CTA detected all bAVMs. With regard to the Spetzler-Martin grade, 4D-CTA disagreed with DSA in only one case, where deep venous drainage was missed. Further discrepancies between 4D-CTA and DSA analyses included underestimation of the nidus size in small lesions (four cases), misinterpretation of a feeding vessel (one case), misinterpretation of indirect feeding through pial collaterals (three cases) and oversight of mild arterial enlargement (two cases). 4D-CTA correctly distinguished low-flow from high-flow lesions and detected dural/transosseous feeding (one case), venous narrowing (one case) and venous pouches (nine cases). In this series, 4D-CTA was able to detect all bAVMs. Although some angioarchitectural details were missed or misinterpreted when compared to DSA, 4D-CTA evaluation was sufficiently accurate to diagnose the shunt and classify it. Moreover, 4D-CTA adds cross-sectional imaging and perfusion maps, helpful in treatment planning. 4D-CTA appears to be a valuable new adjunct in the non-invasive diagnostic work-up of bAVMs and their follow-up when managed conservatively. (orig.)

  5. Stereotactic radiosurgery planning based on time-resolved CTA for arteriovenous malformation: a case report and review of the literature.

    Science.gov (United States)

    Turner, Ryan C; Lucke-Wold, Brandon P; Josiah, Darnell; Gonzalez, Javier; Schmidt, Matthew; Tarabishy, Abdul Rahman; Bhatia, Sanjay

    2016-08-01

    Stereotactic radiosurgery has long been recognized as the optimal form of management for high-grade arteriovenous malformations not amenable to surgical resection. Radiosurgical plans have generally relied upon the integration of stereotactic magnetic resonance angiography (MRA), standard contrast-enhanced magnetic resonance imaging (MRI), or computed tomography angiography (CTA) with biplane digital subtraction angiography (DSA). Current options are disadvantageous in that catheter-based biplane DSA is an invasive test associated with a small risk of complications and perhaps more importantly, the two-dimensional nature of DSA is an inherent limitation in creating radiosurgical contours. The necessity of multiple scans to create DSA contours for radiosurgical planning puts patients at increased risk. Furthermore, the inability to import two-dimensional plans into some radiosurgery programs, such as Cyberknife TPS, limits treatment options for patients. Defining the nidus itself is sometimes difficult in any of the traditional modalities as all draining veins and feeding arteries are included in the images. This sometimes necessitates targeting a larger volume, than strictly necessary, with stereotactic radiosurgery for treatment of the AVM. In this case report, we show the ability to use a less-invasive and three-dimensional form of angiography based on time-lapsed CTA (4D-CTA) rather than traditional DSA for radiosurgical planning. 4D-CTA may allow generation of a series of images, which can show the flow of contrast through the AVM. A review of these series may allow the surgeon to pick and use a volume set that best outlines the nidus with least interference from feeding arteries or draining veins. In addition, 4D-CTA scans can be uploaded into radiosurgery programs and allow three-dimensional targeting. This is the first reported case demonstrating the use of a 4D CTA and an MRI to delineate the AVM nidus for Gamma Knife radiosurgery, with complete

  6. [Arteriovenous malformations of the brain: personal experience with 121 patients treated with microsurgery].

    Science.gov (United States)

    Vilalta, Jordi

    2015-12-01

    Introduccion. Las malformaciones arteriovenosas (MAV) se pueden encontrar en el 0,1% de la poblacion, y la forma de presentacion mas frecuente es la hemorragia intracraneal. Objetivo. Analisis descriptivo de una serie de MAV operadas por un neurocirujano. Pacientes y metodos. De un registro hospitalario se han seleccionado los casos de MAV cerebrales operados por el autor en el periodo 1990-mayo 2014, revisando los aspectos clinicos, demograficos y angiograficos, y los resultados clinicorradiologicos seis meses despues de la cirugia. Resultados. De los 400 pacientes tratados en el mismo periodo, 121 fueron intervenidos por el autor, con una media de edad de 34,3 años (rango: 5-75 años). El 61,7% de los pacientes se diagnostico por una hemorragia cerebral, y la segunda forma de presentacion mas frecuente, el 19,8%, por crisis epilepticas. La localizacion superficial supratentorial, en 97 casos (80,2%), fue la mas frecuente. Mas de la mitad de los enfermos presentaba una MAV de grados bajos, I-II, segun la escala de Spetzler y Martin. Doce pacientes (10%) tenian aneurismas no intranidales asociados. En la mayoria de los casos, 109 (90,1%), se realizo cirugia electiva. La reseccion completa de la MAV despues del ultimo examen angiografico se obtuvo en 118 pacientes (97,5%). Los resultados clinicos fueron buenos (buena recuperacion y moderada incapacidad) en 114 casos (95%), y malos (gran incapacidad y muerte), en seis (5%). Conclusiones. La cirugia para las MAV cerebrales, con un criterio de seleccion apropiado, es la mejor opcion terapeutica para muchos pacientes.

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

    Science.gov (United States)

    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.

  8. Retrospective analysis of linac-based radiosurgery for arteriovenous malformations and testing of the Flickinger formula in predicting radiation injury

    Energy Technology Data Exchange (ETDEWEB)

    Cetin, I.A. [Universitair Ziekenhuis Brussel (Belgium). Dept. of Radiation Oncology; Marmara Univ., Ustkaynarca/Pendik (Turkey). Dept. of Radiation Oncology; Ates, R.; Dhaens, J.; Storme, G. [Universitair Ziekenhuis Brussel (Belgium). Dept. of Radiation Oncology

    2012-12-15

    Background and purpose: The aim of the study was to validate the use of linac-based radiosurgery in arteriovenous malformation (AVM) patients and to predict complications using an integrated logistic formula (ILF) in comparison with clinical outcomes. Patients and methods: The results of radiosurgery in 92 AVM patients were examined. All patients were treated with linac-based radiosurgery. Of these, 70 patients were followed for 12-45 months (median, 24 months) and were analyzed. The treated volume varied from 0.09 to 26.95 cm{sup 3} (median, 2.3 cm{sup 3}) and the median marginal dose was 20 Gy (range, 10.4-22). The median 12-Gy volume was 9.94 cm{sup 3} (range, 0.74-60.09 cm{sup 3}). Patients and lesion characteristics potentially affecting nidus obliteration and excellent outcome were evaluated by performing a log-rank test and univariate and multivariate analyses. The risk for radiation injury (RRI) was calculated with an integrated logistic formula. The predictive power of the RRI was assessed by calculating the area under the receiver operating characteristic (ROC) curve. Results: Follow-up magnetic resonance (MR) angiography revealed complete AVM obliteration in 56 of 70 patients. The MR angiography confirmed an obliteration rate of 80%. The annual hemorrhage rate was 1.4% for the first 2 years after radiosurgery and 0% thereafter. The number of patients with an excellent outcome was 48 (68%). Factors associated with better obliteration were higher radiation dose to the lesion margins [12-Gy volume (V12) > 10 cm{sup 3}], small volume, and a Pollock-Flickinger score less than 1.49; those predicting excellent outcomes were V12 < 10 cm{sup 3}, small volume, and Pollock-Flickinger score less than 1.49, as determined by multivariate analyses. Factors associated with radiation injury were V12 > 10 cm{sup 3} (p=0.03) and volume greater than 2 cm{sup 3} (p=0.001), as determined by a univariate analysis. The analyses showed an ROC of 0.66. Conclusion: These data

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

    Science.gov (United States)

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

    2010-09-01

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

  10. Associação de malformação vascular e gliomas: estudo de quatro casos Arteriovenous malformation-glioma association: study of four cases

    Directory of Open Access Journals (Sweden)

    Lia Raquel R. Borges

    2003-06-01

    Full Text Available Entre os pacientes operados no Hospital São Paulo e acompanhados pelo setor de neuro-oncologia no período de 1991 a 2000, avaliamos a apresentação clínica, aspectos de imagem e características histopatológicas de 4 pacientes (2 homens; idade entre 15 e 52 anos cujo diagnóstico histológico foi malformação vascular associada a glioma. O quadro inicial foi cefaléia progressiva com características de hipertensão intracraniana (em 3 e crises parciais motoras (em 1. O diagnóstico tomográfico inicial foi processo expansivo, sem que houvesse suspeita de malformação vascular pelo aspecto da imagem em nenhum caso. O exame histológico mostrou neoplasias de linhagem astrocítica associadas a malformações vasculares. Em nenhum paciente o componente vascular esteve localizado na intimidade da neoplasia. A associação de malformação vascular e gliomas é rara e deve ser caracterizada por nítida separação entre a malformação e a neoplasia, independente da vascularização própria do tumor.We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years presented with progressive headache with clinical evidence of intracranial hypertension (in 3 and partial seizures (in 1. CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.

  11. Brain AVM (Arteriovenous Malformation)

    Science.gov (United States)

    ... a brain scan for another health issue or after the blood vessels rupture and cause bleeding in the brain (hemorrhage). Once diagnosed, a brain AVM can often be treated successfully to prevent complications, such as brain damage or stroke. Find out why Mayo Clinic is the best ...

  12. Detection of Novel Mutation in Ccm3 Causes Familial Cerebral Cavernous Malformations.

    Science.gov (United States)

    Scimone, Concetta; Bramanti, Placido; Ruggeri, Alessia; Katsarou, Zoe; Donato, Luigi; Sidoti, Antonina; D'Angelo, Rosalia

    2015-11-01

    Cerebral cavernous malformations are vascular lesions that usually involve brain micro-vessels. They can occur both in a sporadic form and familial one. Causes of familial forms are mutations at three loci: CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10. Here, we describe a novel CCM3 missense mutation (c.422T>G) detected in two Greek brothers showing multiple lesions at magnetic resonance imaging; to date, only the youngest is symptomatic. Bioinformatics tools showed this novel variant causes a loss of function in Pdcd10 protein due to its localization in the eighth helix and, particularly, affects Leu141, a highly conserved amino acid. Roles of Pdcd10 in angiogenesis regulation and its association with early development of cerebral cavernous malformations were also considered.

  13. Contemporary role of minimally invasive thoracic surgery in the management of pulmonary arteriovenous malformations: report of two cases and review of the literature

    Science.gov (United States)

    Wang, Stephani C.; Rosen, Jonathan M.

    2016-01-01

    Pulmonary arteriovenous malformations (PAVM) can have potentially serious neurological and cardiac consequences if left untreated. Embolization has supplanted surgical resection as the first line treatment modality. However, this technique is not always successful and carries risks of air embolism, migration of the coil, myocardial rupture, vascular injury, pulmonary hypertension, and pulmonary infarction. We present two patients with symptomatic PAVM despite multiple embolizations: the first one with recurrent and persistent hemoptysis who underwent a thoracoscopic lobectomy, and the second one with chronic debilitating pleuritic pain subsequent to embolization who underwent a thoracoscopic wedge resection. Video-assisted thoracoscopic surgery (VATS) with lung resection was successfully performed in both patients, with complete resolution of their symptoms. We also review the literature regarding the contemporary role of surgery in PAVM, particularly thoracoscopy. PMID:26904229

  14. Contemporary role of minimally invasive thoracic surgery in the management of pulmonary arteriovenous malformations: report of two cases and review of the literature.

    Science.gov (United States)

    Bakhos, Charles T; Wang, Stephani C; Rosen, Jonathan M

    2016-01-01

    Pulmonary arteriovenous malformations (PAVM) can have potentially serious neurological and cardiac consequences if left untreated. Embolization has supplanted surgical resection as the first line treatment modality. However, this technique is not always successful and carries risks of air embolism, migration of the coil, myocardial rupture, vascular injury, pulmonary hypertension, and pulmonary infarction. We present two patients with symptomatic PAVM despite multiple embolizations: the first one with recurrent and persistent hemoptysis who underwent a thoracoscopic lobectomy, and the second one with chronic debilitating pleuritic pain subsequent to embolization who underwent a thoracoscopic wedge resection. Video-assisted thoracoscopic surgery (VATS) with lung resection was successfully performed in both patients, with complete resolution of their symptoms. We also review the literature regarding the contemporary role of surgery in PAVM, particularly thoracoscopy.

  15. 脑动静脉畸形破裂出血并脑疝的急诊手术治疗%Emergent Surgical Treatment of Acute Hemorrhagic Intracranial Arteriovenous Malformation with Intracerebral Herniation

    Institute of Scientific and Technical Information of China (English)

    魏健; 刘维生; 王永和; 曹培成; 卜振富

    2013-01-01

      目的探讨脑动静脉畸形(AVMs)破裂出血并脑疝的急诊手术问题。方法回顾性分析我院17例AVMs急性破裂出血合并脑疝并行急诊手术治疗的病例资料。结果患者术后半年GOS评定疗效,良好4例,中残6例,重残2例,植物生存2例,死亡3例。术后并发肺部感染4例,皮瓣下积液2例,颅内感染并脑积水1例,迟发血肿1例。结论急诊手术治疗是AVMs破裂出血并脑疝首选治疗方法,能够提高病人生存率,降低致残率。%Objective To study the emergent operative treatment of acute hemorrhagic cerebral arteriove-nous malformations(AVMs) with cerebral herniation.Methods A retrospective analysis of 17 patients with acute hemor-rhagic cerebral AVMs with cerebral herniation was carried out ,who were treated with emergent operative treatment .Re-sults According to GOS scale six month after operation ,4 patients recovered well,moderately disabled in 6 cases,se-verely disabled in 2 cases,vegetably survived in 2 cases,3 patients died after operation,4 cases with pneumonia after op-eration,2 cases with subdural hydroma ,1 case with intracranial infection and hydrocephalus ,1 case with recurrent hema-toma.Conclusion The emergent hematoma debridement and AVMs excision by microneurosurgery is the optimal effec-tive method to treat AVMs with cerebral herniation because it can heighten survival rate and decrease disability rate .

  16. Image-guided resection of cerebral cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    毛颖; 周良辅; 杜固宏; 陈亮

    2003-01-01

    Objective To evaluate retrospectively the effectiveness of image-guided navigation techniques in the management of cerebral CMs.Methods Between July 1997 and January 2001, 44 patients underwent image-guided resection of cerebral CMs. To counteract brain shift, a small silicon catheter was implanted as a guide in the case of deep-seated lesions (except in the case of brain stem CMs) and before excision of multiple lesions. Results A total of 27 men and 17 women with a mean age of 35 years underwent surgical procedures (5 patients had multiple lesions). The lesions were located in the frontal (n=14), lobe temporal lobe (n=12), parietal lobe (n=6), cerebellum (n=6), thalamus (n=5), pons (n=5), and orbital region (n=1). Under the guidance of a StealthStation navigator, total removal of the lesions was achieved in all patients. Follow-up revealed marked improvement of preoperative symptoms in 26 patients and no additional deficits in 13 patients. Five patients suffered from additional neurological deficits, but two of them gradually improved during the follow-up period. Conclusions With the assistance of an image-guided surgical system, functional areas can be effectively avoided and surgical injury can be decreased. This system is well suited for accurate localization and safe resection of small, deep-seated CMs.

  17. Novel, mutable site in the cerebral cavernous malformation-1 gene in Chinese sporadic intracranial cavernous malformation patients

    Institute of Scientific and Technical Information of China (English)

    Rong Xie; Xiancheng Chen

    2009-01-01

    BACKGROUND: A cerebral cavernous malformation-1 (CCM1) gene mutation might result in functional loss of KREV interaction trapped-1 (KRIT1), which is related to onset of cavernous malformations (CM). However, data addressing sporadic CM in Chinese patients remains limited to date.OBJECTIVE: To analyze CCM1 mutation of Chinese patients with sporadic intracranial CM.DESIGN, TIME AND SETTING: Genetics experiment was performed in the Department of Neurosurgery, Huashan Hospital Affiliated to Fudan University between January 2004 and December 2005.PARTICIPANTS: Ninety patients with sporadic CM served as the CM group, and 30 healthy subjects were considered to be the control group.METHODS: Peripheral blood was collected from patients with CM and from control group subjects.Genomic DNA was extracted, and exons 8, 9, 11, 12, 13, 15, 16, 17, and 18, as well as the related introns, were amplified using polymerase chain reaction. DNA sequences were compared with GeneBank.MAIN OUTCOME MEASURES: Abnormal mutable site of CCM1 gene in the two groups.RESULTS: Four exclusive mutations of CCM1 were detected in the CM group, with a sporadic CM mutational rate of 32% (6/19). Of the four exclusive mutations, there was one missense mutation [exon 12, 1172C→T (S391F)], one insertion mutation [exon 8, 704insT (K246stop)], one intervening sequence mutation (IVS12-4C→T), and one synonymous mutation (exon 17,1875C→T). With the exception of 1875C→T, all mutations detected in the CM group led to functional changes of the KRIT1 protein, which was encoded by the CCM1 gene. Gene mutations were not detected in the control group.CONCLUSION: Four exclusive mutations of the CCM1 gene were determined in Chinese patients with sporadic CM, which led to functional changes or loss of the encoding KRIT1 protein. KRIT1 protein is considered to be the genetic basis of CM occurrence.

  18. Acute Hemiparesis in a Child as a Presenting Symptom of Hemispheric Cerebral Proliferative Angiopathy

    Directory of Open Access Journals (Sweden)

    J. J. Gold

    2013-01-01

    Full Text Available A 9-year-old girl with a several-month history of unilateral intermittent headaches presented to the hospital with worsening headaches and unsteadiness. Neurologic exam was positive for a mild right hemiparesis and right homonymous hemianopsia. Noncontrast computed tomography revealed an engorged sagittal and straight sinus with prominent cortical veins concerning an arteriovenous malformation and the patient was admitted to the pediatric intensive care unit. Computed tomography angiogram demonstrated a left hemispheric vascular malformation, without evidence of dural arteriovenous fistula on conventional angiogram consistent with a diagnosis of cerebral proliferative angiopathy. There was no evidence of infarct on magnetic resonance imaging, and the patient’s symptoms were completely resolved within 24 hours. Cerebral proliferative angiopathy is a rare but important vascular malformation distinct from classic arteriovenous malformations that may present with stroke-like symptoms in childhood.

  19. Cerebral malformation induced by prenatal X-irradiation: an autoradiographic and Golgi study

    Energy Technology Data Exchange (ETDEWEB)

    Ferrer, I.; Xumetra, A.; Santamaria, J. (Neuropatologia, Depto. Anatomia Patologica, C.S. ' Principes de Espana' , Hospitalet de Llobregat, Barcelona (Spain))

    1984-01-01

    Brain malformations are produced after X-irradiation at different post-conceptional ages in the rat. Malformed cortical patterns result from abnormal organisation and capricious orientation of the neurons, while a radical migratory pattern of neuroblasts outwards to the cerebral cortex is preserved in animals irradiated on the fourteenth, sixteenth or eighteenth days of gestation. Migratory disturbances are restricted to the large subcortical ectopic masses found in rats irradiated on the fourteenth gestational day and to pyramidal ectopic nodules in the hippocampus in rats irradiated on the sixteenth gestational day. Subcortical ectopic masses develop from ectopic germinal rosettes and are formed by several types of cortical neuron distributed in a stereotyped pattern. The presence of large numbers of intrinsic, afferent and efferent connections are indicative of integrative functions of the subcortical masses.

  20. A Rare Case of Acroangiodermatitis Associated with a Congenital Arteriovenous Malformation (Stewart-Bluefarb Syndrome) in a Young Veteran: Case Report and Review of the Literature.

    Science.gov (United States)

    Archie, Mark; Khademi, Saieh; Aungst, David; Nouvong, Aksone; Freeman, Shanna; Gelabert, Hugh; Rigberg, David; deVirgilio, Christian; Lewis, Michael; O'Connell, Jessica

    2015-10-01

    Acroangiodermatitis (AD) is a rare angioproliferative disease manifesting with cutaneous lesions clinically similar to Kaposi's sarcoma. AD is a benign hyperplasia of preexisting vasculature and may be associated with acquired or congenital arteriovenous malformations (AVM), or severe chronic venous insufficiency (because of hypostasis, elevated venous pressure, arteriovenous shunting). Stewart-Bluefarb syndrome is the rare syndrome in which AD is associated with a congenital AVM. We present the case of a young veteran with a painful, chronic nonhealing ulcer and ipsilateral popliteal artery occlusion likely because of trauma, who elected transmetatarsal amputation for symptomatic relief. A 24-year-old male veteran presented with a 5-year history of a nonhealing dorsal left foot ulcer, resulting from a training exercise injury. He ultimately developed osteomyelitis requiring antibiotics, frequent debridements, multiple trials of unsuccessful skin substitute grafting, and severe unremitting pain. He noted a remote history of left digital deformities treated surgically as a child, and an AVM, previously endovascularly treated at an outside facility. Arterial duplex revealed somewhat dampened left popliteal, posterior tibial (PT), and dorsalis pedis (DP) artery signals with arterial brachial index of 1.0. CT angiography showed occlusion of the proximal to mid popliteal artery with significant calcifications felt initially to be a result of prior trauma. Pedal pulses were palpable and transcutaneous oxygen measurements revealed adequate oxygenation. Because of unremitting pain, the patient opted for amputation. Pathology revealed vascular proliferation consistent with AD. This case illustrates an unusual diagnosis of acroangiodermatitis, and a rare syndrome when associated with his underlying AVM (Stewart-Bluefarb syndrome). This resulted in a painful, chronic ulcer and was further complicated by trauma-related arterial occlusive disease. AD disease can hinder wound

  1. The “focus on aneurysm” principle: Classification and surgical principles of management of concurrent arterial aneurysm with arteriovenous malformation causing intracranial hemorrhage

    Science.gov (United States)

    Jha, Vikas; Behari, Sanjay; Jaiswal, Awadhesh K.; Bhaisora, Kamlesh Singh; Shende, Yogesh P.; Phadke, Rajendra V.

    2016-01-01

    Context: Concurrent arterial aneurysms (AAs) occurring in 2.7-16.7% patients harboring an arteriovenous malformation (AVM) aggravate the risk of intracranial hemorrhage. Aim: We evaluate the variations of aneurysms simultaneously coexisting with AVMs. A classification-based management strategy and an abbreviated nomenclature that describes their radiological features is also proposed. Setting: Tertiary care academic institute. Statistics: Test of significance applied to determine the factors causing rebleeding in the groups of patients with concurrent AVM and aneurysm and those with only AVMs. Subjects and Methods: Sixteen patients (5 with subarachnoid hemorrhage and 11 with intracerebral/intraventricular hemorrhage; 10 with low flow [LF] and 6 with high flow [HF] AVMs) underwent radiological assessment of Spetzler Martin (SM) grading and flow status of AA + AVM. Their modified Rankin's score (mRS) at admission was compared with their follow-up (F/U) score. Results: Pre-operative mRS was 0 in 5, 2 in 6, 3 in 1, 4 in 3 and 5 in 1; and, SM grade I in 5, II in 3, III in 3, IV in 4 and V in 1 patients, respectively. AA associated AVMs were classified as: (I) Flow-related proximal (n = 2); (II) flow-related distal (n = 3); (III) intranidal (n = 5); (IV) extra-intranidal (n = 2); (V) remote major ipsilateral (n = 1); (VI) remote major contralateral (n = 1); (VII) deep perforator related (n = 1); (VIII) superficial (n = 1); and (IX) distal (n = 0). Their treatment strategy included: Flow related AA, SM I-III LF AVM: aneurysm clipping with AVM excision; nidal-extranidal AA, SM I-III LF AVM: Excision or embolization of both AA + AVM; nidal-extranidal and perforator-related AA, SM IV-V HF AVM: Only endovascular embolization or radiosurgery. Surgical decision-making for remote AA took into account their ipsilateral/contralateral filling status and vessel dominance; and, for AA associated with SM III HF AVM, it varied in each patient based on diffuseness of AVM nidus, flow

  2. Immune complex formation and in situ B-cell clonal expansion in human cerebral cavernous malformations.

    Science.gov (United States)

    Shi, Changbin; Shenkar, Robert; Kinloch, Andrew; Henderson, Scott G; Shaaya, Mark; Chong, Anita S; Clark, Marcus R; Awad, Issam A

    2014-07-15

    Cerebral cavernous malformations (CCMs) represent clusters of dilated vascular channels, predisposing to hemorrhagic stroke and seizures. They are associated with defective blood brain barrier, hemorrhages of different ages and a robust inflammatory cell infiltrate. We report for the first time evidence of co-localized IgG and complement membrane attack complexes in CCM lesions. CD4(+) and CD8(+) T-cells are aggregated with CD20(+) B-cells. And IgG repertoire analyses demonstrate in situ B-cell clonal expansion and antigen-driven affinity maturation in CCMs. These results suggest an organ-intrinsic adaptive immune response in CCMs that should be further characterized as a potential therapeutic target.

  3. Unilateral facial palsy in an infant: an unusual presentation of familial multiple cerebral cavernous malformation.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Cerebral cavernous malformation (CCM) in infants tends to have genetic predisposition. These cavernomas have a progressive course of events and associated neurological symptoms with increase in age. They most commonly present with seizure and syndrome of increased intracranial pressure comprising of headache, vomiting and focal neurological signs. We describe a case of a 7-month-old infant who presented with an acute onset of right facial paralysis with a background of familial CCM. The CT and MRI scan revealed fresh haemorrhage in the right cerebellar and pontine cavernomas with surrounding oedema and no evidence of obstructive hydrocephalus. These two cavernomas re-bled in a week duration causing episodes of incessant crying and irritability. After discussing the pros and cons of treatment, owing to stable clinical status, the patient is currently been managed conservatively.

  4. An interesting case of cerebral abscess.

    Science.gov (United States)

    Rafiq, Muhammad Khizar

    2009-01-01

    An immigrant from Romania was referred to the neurosurgical unit with a cerebral abscess. On examination she was cyanosed and had clubbing of her fingers. A cardiovascular system examination revealed a systolic murmur heard all over the precordium. However, the diagnosis was not congenital cyanotic heart disease. The patient had a history of frequent nosebleeds and had multiple telangiectases on her body, leading to the diagnosis of hereditary haemorrhagic telangiectasia (HHT). A search was carried out for the presence of arteriovenous malformations in internal organs. Large arteriovenous malformations were found in the lungs, causing her cyanosis due to right-to-left shunting of blood and cerebral abscess due to paradoxical septic embolisation into cerebral circulation.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  6. A novel CCM2 gene mutation associated with family cerebral cavernous malformation

    Directory of Open Access Journals (Sweden)

    Wen-Qing Huang

    2016-09-01

    Full Text Available Background: Cerebral cavernous malformations (CCMs are common vascular malformations that predominantly arise in the central nervous system and are mainly characterized by enlarged vascular cavities without intervening brain parenchyma. Familial CCMs (FCCMs is inherited in an autosomal dominant pattern with incomplete penetrance and variable symptoms. Methods: Mutations of three pathogenic genes, CCM1, CCM2 and CCM3, were investigated by direct DNA sequencing in a Chinese family with multiple CCM lesions. Results: Four heterozygous variants in the CCM2 gene, including one deletion (c.95delC, a missense mutation (c.358G>A, p.V120I, one silent mutation (c.915G>A, p.T305T, and a substitution (c. *1452 T>C, were identified in the subjects with multiple CCM lesions, but not in a healthy sibling. Among these variants, the c.95delC deletion is a novel mutation which is expected to cause a premature termination codon. It is predicted to produce a truncated CCM2 protein lacking the PTB and C-terminal domains, thus disrupting the molecular functions of CCM2. Conclusions: The novel truncating mutation in the CCM2 gene, c.95delC, may be responsible for multiple CCM lesions in a part of FCCM. In addition, it may represent a potential genetic biomarker for early diagnosis of FCCM.

  7. Sporadic Cerebral Cavernous Malformations: Report of Further Mutations of CCM Genes in 40 Italian Patients

    Directory of Open Access Journals (Sweden)

    Rosalia D’Angelo

    2013-01-01

    Full Text Available Cerebral cavernous malformations (CCMs are vascular lesions characterized by abnormally enlarged capillary cavities, affecting the central nervous system. CCMs can occur sporadically or as a familial autosomal dominant condition with incomplete penetrance and variable clinical expression attributable to mutations in three different genes: CCM1 (K-Rev interaction trapped 1 (KRIT1, CCM2 (MGC4607, and CCM3 (PDCD10. CCMs occur as a single or multiple malformations that can lead to seizures, focal neurological deficits, hemorrhagic stroke, and headache. However, patients are frequently asymptomatic. In our previous mutation screening, performed in a cohort of 95 Italian patients, both sporadic and familial, we have identified several mutations in CCM genes, three of which in three distinct sporadic patients. In this study, representing further molecular screening of the three CCM genes, in a south Italian cohort of CCM patients enrolled by us in the last three years, we report the identification of other four new mutations in 40 sporadic patients with either single or multiple CCM.

  8. Sporadic Cerebral Cavernous Malformations: Report of Further Mutations of CCM Genes in 40 Italian Patients

    Science.gov (United States)

    D'Angelo, Rosalia; Alafaci, Concetta; Scimone, Concetta; Ruggeri, Alessia; Salpietro, Francesco Maria; Bramanti, Placido; Tomasello, Francesco; Sidoti, Antonina

    2013-01-01

    Cerebral cavernous malformations (CCMs) are vascular lesions characterized by abnormally enlarged capillary cavities, affecting the central nervous system. CCMs can occur sporadically or as a familial autosomal dominant condition with incomplete penetrance and variable clinical expression attributable to mutations in three different genes: CCM1 (K-Rev interaction trapped 1 (KRIT1)), CCM2 (MGC4607), and CCM3 (PDCD10). CCMs occur as a single or multiple malformations that can lead to seizures, focal neurological deficits, hemorrhagic stroke, and headache. However, patients are frequently asymptomatic. In our previous mutation screening, performed in a cohort of 95 Italian patients, both sporadic and familial, we have identified several mutations in CCM genes, three of which in three distinct sporadic patients. In this study, representing further molecular screening of the three CCM genes, in a south Italian cohort of CCM patients enrolled by us in the last three years, we report the identification of other four new mutations in 40 sporadic patients with either single or multiple CCM. PMID:24058906

  9. Surveying genetic variants and molecular phylogeny of cerebral cavernous malformation gene, CCM3/PDCD10.

    Science.gov (United States)

    Kumar, Abhishek; Bhandari, Anita; Goswami, Chandan

    2014-12-01

    The three cerebral cavernous malformations (CCMs) genes namely CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10 have been identified for which mutations cause cerebral cavernous malformations. However, the protein products of these genes involved in forming CCM signaling, are still poorly understood imposing an urgent need to understand these genes and their signaling processes in details. So far involvement of CCM3/PDCD10 in the cavernous angioma has been characterized from biochemical and biophysical analyses. However, there is no comprehensive study illustrating the phylogenetic history and comprehensive genetic variants of CCM3/PDCD10. Herein, we explored the phylogenetic history and genetic variants of CCM3/PDCD10 gene. Synteny analyses revealed that CCM3/PDCD10 gene shared same genomic loci from Drosophila to human and the gene structure of CCM3/PDCD10 is conserved from human to Branchiostoma floridae for about 500 MYs with some changes in sea urchin and in insects. The conserved CCM3/PDCD10 is characterized by presence of indels in the N-terminal dimerization domain. We identified 951 CCM3/PDCD10 variants by analysis of 1092 human genomes with top three variation classes belongs to 84% SNPs, 6.9% insertions and 6.2% deletions. We identified 22 missense mutations in the human CCM3/PDCD10 protein and out of which three mutations are deleterious. We also identified four stop-codon gaining mutations at the positions E34*, E68*, E97* and E140*, respectively. This study is the first comprehensive analysis of the CCM3/PDCD10 gene based on phylogenetic origin and genetic variants. This study corroborates that the evolution of CCM proteins with tubular organization evolvements by endothelial cells.

  10. Brain arteriovenous malformation diagnosis: value of time-resolved contrast-enhanced MR angiography at 3.0T compared to DSA

    Energy Technology Data Exchange (ETDEWEB)

    Machet, A.; Kadziolka, K.; Robin, G.; Lanoix, O.; Pierot, L. [Maison Blanche Hospital, University of Reims, Department of Radiology, Reims (France); Portefaix, C. [Maison Blanche Hospital, University of Reims, Department of Radiology, Reims (France); University of Reims, CReSTIC SIC EA3804, Reims (France)

    2012-10-15

    This study was conducted in order to evaluate the value of time-resolved contrast-enhanced magnetic resonance angiography (TR-CE-MRA) with a 3.0-T magnetic field compared to digital subtraction angiography (DSA) as the reference standard for the diagnosis of brain arteriovenous malformation (bAVM). Nineteen patients with 19 angiographically confirmed untreated bAVM were investigated with both DSA and TR-CE-MRA for the initial diagnosis. Examinations were compared by two independent readers. Interobserver agreement and intermodality agreement with respect to nidus size, arterial feeders, and venous drainage were determined using the K statistic test. Also, the quality of the TR-CE-MRA images was evaluated. Seventeen of the 19 bAVM (89.5%) detected with DSA were diagnosed with TR-CE-MRA. Interobserver agreement for TR-CE-MRA was good for nidus size, venous drainage, and arterial feeders (K = 0.75, 95% CI 0.50-1.00; K = 0.77, 95% CI 0.54-1.00; and K = 0.80, 95% CI 0.59-1.00 respectively). Intermodality agreement was good for nidus size and venous drainage (K = 0.75, 95% CI 0.49-1.00 and K = 0.77, 95% CI 0.54-1.00, respectively) and moderate for arterial feeders (K = 0.44, 95% CI 0.17-0.70). TR-CE-MRA at 3.0 T has a good sensitivity for bAVM detection and good agreement with DSA for determining nidus size and the type of venous drainage, suggesting that TR-CE-MRA is potentially a reliable tool for the diagnosis and assessment of bAVMs. However, it still suffers from low spatial resolution and vessel superposition, making differentiation of the arterial feeders of the nidus difficult at times. (orig.)

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

    Energy Technology Data Exchange (ETDEWEB)

    Fisher, Oriana S. [Yale Univ., New Haven, CT (United States); Deng, Hanqiang [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China); Liu, Dou [Yale Univ. School of Medicine, New Haven, CT (United States); Zhang, Ya [Yale Univ. School of Medicine, New Haven, CT (United States); Wei, Rong [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China); Deng, Yong [Yale Univ. School of Medicine, New Haven, CT (United States); Zhang, Fan [Yale Univ. School of Medicine, New Haven, CT (United States); Louvi, Angeliki [Yale Univ. School of Medicine, New Haven, CT (United States); Turk, Benjamin E. [Yale Univ., New Haven, CT (United States); Boggon, Titus J. [Yale Univ., New Haven, CT (United States); Su, Bing [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China)

    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.

  12. Whole-brain CT digital subtraction angiography of cerebral dural arteriovenous fistula using 320-detector row CT

    Energy Technology Data Exchange (ETDEWEB)

    Fujiwara, Hirokazu; Momoshima, Suketaka; Kuribayashi, Sachio [Keio University, Department of Diagnostic Radiology, School of Medicine, Tokyo (Japan); Akiyama, Takenori [Keio University, Department of Neurosurgery, School of Medicine, Tokyo (Japan)

    2013-07-15

    The purpose of this study was to evaluate the usefulness of CT digital subtraction angiography (CTDSA) by using 320-detector row CT in the diagnosis and classification of cerebral dural arteriovenous fistula (dAVF) and comparing it with DSA as the standard reference. A total of 29 CTDSA/DSA from 25 patients with dAVF were retrospectively evaluated by two neuroradiologists. The presence, Cognard classification, and feeding arteries of dAVFs on CTDSA were assessed according to DSA. DSA depicted 33 dAVFs in 28 cases. By consensus reading, CTDSA correctly detected 32 dAVFs in 27 cases and properly graded 31 lesions. The intermodality agreement for the presence and classification of dAVFs was excellent (kappa = 0.955 and 0.921, respectively). CTDSA detected 77 of 109 feeding arteries (70.6 %) in 25 cases. The intermodality agreement for the feeding arteries was good (kappa = 0.713). Although CTDSA is limited in temporal and spatial resolution in comparison with DSA, it is an effective non-invasive tool for the detection and classification of dAVF. (orig.)

  13. Cerebral cavernous malformations arise from endothelial gain of MEKK3-KLF2/4 signalling.

    Science.gov (United States)

    Zhou, Zinan; Tang, Alan T; Wong, Weng-Yew; Bamezai, Sharika; Goddard, Lauren M; Shenkar, Robert; Zhou, Su; Yang, Jisheng; Wright, Alexander C; Foley, Matthew; Arthur, J Simon C; Whitehead, Kevin J; Awad, Issam A; Li, Dean Y; Zheng, Xiangjian; Kahn, Mark L

    2016-04-07

    Cerebral cavernous malformations (CCMs) are common inherited and sporadic vascular malformations that cause strokes and seizures in younger individuals. CCMs arise from endothelial cell loss of KRIT1, CCM2 or PDCD10, non-homologous proteins that form an adaptor complex. How disruption of the CCM complex results in disease remains controversial, with numerous signalling pathways (including Rho, SMAD and Wnt/β-catenin) and processes such as endothelial-mesenchymal transition (EndMT) proposed to have causal roles. CCM2 binds to MEKK3 (refs 7, 8, 9, 10, 11), and we have recently shown that CCM complex regulation of MEKK3 is essential during vertebrate heart development. Here we investigate this mechanism in CCM disease pathogenesis. Using a neonatal mouse model of CCM disease, we show that expression of the MEKK3 target genes Klf2 and Klf4, as well as Rho and ADAMTS protease activity, are increased in the endothelial cells of early CCM lesions. By contrast, we find no evidence of EndMT or increased SMAD or Wnt signalling during early CCM formation. Endothelial-specific loss of Map3k3 (also known as Mekk3), Klf2 or Klf4 markedly prevents lesion formation, reverses the increase in Rho activity, and rescues lethality. Consistent with these findings in mice, we show that endothelial expression of KLF2 and KLF4 is increased in human familial and sporadic CCM lesions, and that a disease-causing human CCM2 mutation abrogates the MEKK3 interaction without affecting CCM complex formation. These studies identify gain of MEKK3 signalling and KLF2/4 function as causal mechanisms for CCM pathogenesis that may be targeted to develop new CCM therapeutics.

  14. Cerebral cavernous malformations arise from endothelial gain of MEKK3-KLF2/4 signaling

    Science.gov (United States)

    Zhou, Zinan; Tang, Alan T.; Wong, Weng-Yew; Bamezai, Sharika; Goddard, Lauren M.; Shenkar, Robert; Zhou, Su; Yang, Jisheng; Wright, Alexander C.; Foley, Matthew; Arthur, J. Simon C.; Whitehead, Kevin J.; Awad, Issam A.; Li, Dean Y.; Zheng, Xiangjian; Kahn, Mark L.

    2016-01-01

    Cerebral cavernous malformations (CCMs) are common inherited and sporadic vascular malformations that cause stroke and seizures in younger individuals1. CCMs arise from endothelial cell loss of KRIT1, CCM2, or PDCD10, non-homologous proteins that form an adaptor complex2. How disruption of the CCM complex results in disease remains controversial, with numerous signaling pathways (including Rho3,4, SMAD5 and Wnt/β-catenin6) and processes such as endothelial-mesenchymal transition (EndMT)5 proposed to play causal roles. CCM2 binds MEKK37–11, and we have recently demonstrated that CCM complex regulation of MEKK3 is essential during vertebrate heart development12. Here, we investigate this mechanism in CCM disease pathogenesis. Using a neonatal mouse model of CCM disease, we find that expression of the MEKK3 target genes KLF2 and KLF4, as well as Rho and ADAMTS protease activity, are increased in the endothelial cells of early CCM lesions. In contrast, we find no evidence of EndMT or increased SMAD or Wnt signaling during early CCM formation. Endothelial-specific loss of Mekk3, Klf2, or Klf4 dramatically prevents lesion formation, reverses the increase in Rho activity, and rescues lethality. Consistent with these findings in mice, we demonstrate that endothelial expression of KLF2 and KLF4 is elevated in human familial and sporadic CCM lesions, and that a disease-causing human CCM2 mutation abrogates MEKK3 interaction without affecting CCM complex formation. These studies identify gain of MEKK3 signaling and KLF2/4 function as causal mechanisms for CCM pathogenesis that may be targeted to develop new CCM therapeutics. PMID:27027284

  15. B-Cell Depletion Reduces the Maturation of Cerebral Cavernous Malformations in Murine Models.

    Science.gov (United States)

    Shi, Changbin; Shenkar, Robert; Zeineddine, Hussein A; Girard, Romuald; Fam, Maged D; Austin, Cecilia; Moore, Thomas; Lightle, Rhonda; Zhang, Lingjiao; Wu, Meijing; Cao, Ying; Gunel, Murat; Louvi, Angeliki; Rorrer, Autumn; Gallione, Carol; Marchuk, Douglas A; Awad, Issam A

    2016-06-01

    Cerebral cavernous malformations (CCMs) are relatively common vascular malformations, characterized by increased Rho kinase (ROCK) activity, vascular hyper-permeability and the presence of blood degradation products including non-heme iron. Previous studies revealed robust inflammatory cell infiltration, selective synthesis of IgG, in situ antigen driven B-cell clonal expansion, and deposition of immune complexes and complement proteins within CCM lesions. We aimed to evaluate the impact of suppressing the immune response on the formation and maturation of CCM lesions, as well as lesional iron deposition and ROCK activity. Two murine models of heterozygous Ccm3 (Pdcd10), which spontaneously develop CCM lesions with severe and milder phenotypes, were either untreated or received anti-mouse BR3 to deplete B cells. Brains from anti-mouse BR3-treated mice exhibited significantly fewer mature CCM lesions and smaller lesions compared to untreated mice. B cell depletion halted the progression of lesions into mature stage 2 lesions but did not prevent their genesis. Non-heme iron deposition and ROCK activity was decreased in lesions of B cell depleted mice. This represents the first report of the therapeutic benefit of B-cell depletion in the development and progression of CCMs, and provides a proof of principle that B cells play a critical role in CCM lesion genesis and maturation. These findings add biologics to the list of potential therapeutic agents for CCM disease. Future studies would characterize the putative antigenic trigger and further define the mechanism of immune response in the lesions.

  16. Three-dimensional assessment of the effects of high-density embolization material on the absorbed dose in the target for Gamma Knife radiosurgery of arteriovenous malformations.

    Science.gov (United States)

    Watanabe, Yoichi; Sandhu, Divyajot; Warmington, Leighton; Moen, Sean; Tummala, Ramachandra

    2016-12-01

    OBJECTIVE Arteriovenous malformation (AVM) is an intracranial vascular disorder. Gamma Knife radiosurgery (GKRS) is used in conjunction with intraarterial embolization to eradicate the nidus of AVMs. Clinical results indicate that patients with prior embolization tend to gain less benefit from GKRS. The authors hypothesized that this was partly caused by dosimetric deficiency. The actual dose delivered to the target may be smaller than the intended dose because of increased photon attenuation by high-density embolic materials. The authors performed a phantom-based study to quantitatively evaluate the 3D dosimetric effect of embolic material on GKRS. METHODS A 16-cm-diameter and 12-cm-long cylindrical phantom with a 16-cm-diameter hemispherical dome was printed by a 3D printer. The phantom was filled with radiologically tissue-equivalent polymer gel. To simulate AVM treatment with embolization, phantoms contained Onyx 18. The material was injected into an AVM model, which was suspended in the polymer gel. The phantom was attached to a Leksell frame by standard GK fixation method, using aluminum screws, for imaging. The phantom was scanned by a Phillips CT scanner with the standard axial-scanning protocol (120 kV and 1.5-mm slice thickness). CT-based treatment planning was performed with the GammaPlan treatment planning system (version 10.1.1). The plan was created to cover a fictitious AVM target volume near the embolization areas with eleven 8-mm shots and a prescription dose of 20 Gy to 50% isodose level. Dose distributions were computed using both tissue maximum ratio (TMR) 10 and convolution dose-calculation algorithms. These two 3D dose distributions were compared using an in-house program. Additionally, the same analysis method was applied to evaluate the dosimetric effects for 2 patients previously treated by GKRS. RESULTS The phantom-based analyses showed that the mean dose difference between TMR 10 and convolution doses of the AVM target was no larger than

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

    Energy Technology Data Exchange (ETDEWEB)

    Subramanian, Sai; Srinivas, Chilukuri; Ramalingam, K.; Babaiah, M.; Swamy, S. Thirumalai; Arun, G.; Kathirvel, M.; Ashok, S. [Yashoda Super Specialty Hospital, Hyderabad (India); Clivio, Alessandro [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland); Fogliata, Antonella, E-mail: antonella.fogliata-cozzi@eoc.ch [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland); Nicolini, Giorgia [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland); Rao, K. Srinivasa; Reddy, T. Pratap; Amit, Jotwani [Yashoda Super Specialty Hospital, Hyderabad (India); Vanetti, Eugenio; Cozzi, Luca [Oncology Institute of Southern Switzerland, Bellinzona (Switzerland)

    2012-03-01

    Purpose: To evaluate, with a dosimetric and clinical feasibility study, RapidArc (a volumetric modulated arc technique) for hypofractionated stereotactic radiotherapy treatment of large arteriovenous malformations (AVMs). Methods and Materials: Nine patients were subject to multimodality imaging (magnetic resonance, computed tomography, and digital subtraction angiography) to determine nidus and target volumes, as well as involved organs at risk (optical structures, inner ear, brain stem). Plans for multiple intensity-modulated arcs with a single isocenter were optimized for a fractionation of 25 Gy in 5 fractions. All plans were optimized for 6-MV photon beams. Dose-volume histograms were analyzed to assess plan quality. Delivery parameters were reported to appraise technical features of RapidArc, and pretreatment quality assurance measurements were carried out to report on quality of delivery. Results: Average size of AVM nidus was 26.2 cm{sup 3}, and RapidArc plans provided complete target coverage with minimal overdosage (V{sub 100%} = 100% and V{sub 110%} < 1%) and excellent homogeneity (<6%). Organs at risk were highly spared. The D{sub 1%} to chiasm, eyes, lenses, optic nerves, and brainstem (mean {+-} SD) was 6.4 {+-} 8.3, 1.9 {+-} 3.8, 2.3 {+-} 2.2, 0.7 {+-} 0.9, 4.4 {+-} 7.2, 12.2 {+-} 9.6 Gy, respectively. Conformity index (CI{sub 95%}) was 2.2 {+-} 0.1. The number of monitor units per gray was 277 {+-} 45, total beam-on time was 2.5 {+-} 0.3 min. Planning vs. delivery {gamma} pass rate was 98.3% {+-} 0.9%. None of the patients developed acute toxicity. With a median follow-up of 9 months, 3 patients presented with deterioration of symptoms and were found to have postradiation changes but responded symptomatically to steroids. These patients continue to do well on follow-up. One patient developed headache and seizures, which was attributed to intracranial bleed, confirmed on imaging. Conclusion: Hypofractionated stereotactic radiotherapy can be

  18. Live-cell imaging to detect phosphatidylserine externalization in brain endothelial cells exposed to ionizing radiation: implications for the treatment of brain arteriovenous malformations.

    Science.gov (United States)

    Zhao, Zhenjun; Johnson, Michael S; Chen, Biyi; Grace, Michael; Ukath, Jaysree; Lee, Vivienne S; McRobb, Lucinda S; Sedger, Lisa M; Stoodley, Marcus A

    2016-06-01

    OBJECT Stereotactic radiosurgery (SRS) is an established intervention for brain arteriovenous malformations (AVMs). The processes of AVM vessel occlusion after SRS are poorly understood. To improve SRS efficacy, it is important to understand the cellular response of blood vessels to radiation. The molecular changes on the surface of AVM endothelial cells after irradiation may also be used for vascular targeting. This study investigates radiation-induced externalization of phosphatidylserine (PS) on endothelial cells using live-cell imaging. METHODS An immortalized cell line generated from mouse brain endothelium, bEnd.3 cells, was cultured and irradiated at different radiation doses using a linear accelerator. PS externalization in the cells was subsequently visualized using polarity-sensitive indicator of viability and apoptosis (pSIVA)-IANBD, a polarity-sensitive probe. Live-cell imaging was used to monitor PS externalization in real time. The effects of radiation on the cell cycle of bEnd.3 cells were also examined by flow cytometry. RESULTS Ionizing radiation effects are dose dependent. Reduction in the cell proliferation rate was observed after exposure to 5 Gy radiation, whereas higher radiation doses (15 Gy and 25 Gy) totally inhibited proliferation. In comparison with cells treated with sham radiation, the irradiated cells showed distinct pseudopodial elongation with little or no spreading of the cell body. The percentages of pSIVA-positive cells were significantly higher (p = 0.04) 24 hours after treatment in the cultures that received 25- and 15-Gy doses of radiation. This effect was sustained until the end of the experiment (3 days). Radiation at 5 Gy did not induce significant PS externalization compared with the sham-radiation controls at any time points (p > 0.15). Flow cytometric analysis data indicate that irradiation induced growth arrest of bEnd.3 cells, with cells accumulating in the G2 phase of the cell cycle. CONCLUSIONS Ionizing radiation

  19. Involvement of the visual pathway is not a risk factor of visual field deficits in patients with occipital arteriovenous malformations:an fMRI study

    Institute of Scientific and Technical Information of China (English)

    Xianzeng Tong; Jun Wu; Fuxin Lin; Yong Cao; Yuanli Zhao; Zhen Jin; Shuo Wang

    2015-01-01

    Background: Occipital arteriovenous malformations (AVMs) are still one of neurosurgery's most intriguing and challenging pathologies.In this study, we reviewed our series of patients with occipital AVMs admitted in Beijing Tiantan Hospital from June 2013 through January 2015 and attempted to evaluate the risk factors of visual field deficits (VFDs) in these patients at presentation.Methods: Forty-two consecutive patients with occipital AVMs were included in our study.Patient parameters (age, sex, and history of hemorrhage) and AVM characteristics (size, side, venous drainage, Spetzler-Martin grade, and diffuseness) were collected.VFDs were quantified using an Octopus perimetry.Conventional MRI,blood oxygen level dependent fMRI (BOLD-fMRI) of the visual cortex, and diffusion tensor imaging (DTI) of the optic radiation were performed.The least distances from the AVM to the optic radiation (AVM-OR) and from the AVM to the visual cortex (AVM-VC) were measured.Univariate analyses were used to correlate initial VFDs with patient parameters, AVM characteristics, AVM-OR, and AVM-VC distances.Results: VFDs were identified in 14 patients, among which 12 patients presented with a history of hemorrhage and 2 patients presented with nonhemorrhagic chronic headache.VFDs were more common (P =0.000003) in patients with ruptured AVMs.VFD frequency was not associated with patient age, sex, and AVM characteristics (size, side,venous drainage, S-M grade, and diffuseness).Unlike other lesions involving the optic radiation and visual cortex, the frequency of VFDs in occipital AVMs did not correlate with the AVM-OR and AVM-VC distances (P =0.640 and 0.638,respectively).Conclusions: A history of hemorrhage is an independent risk factor of VFDs in occipital AVMs.Most unruptured occipital AVMs may present with chronic headache and seizures other than VFDs.The distances from the AVMs to the optic radiation and the visual cortex are not associated with preexisting VFDs.Our results prompt us

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

    Science.gov (United States)

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2010-04-12

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

  2. Update on Novel CCM Gene Mutations in Patients with Cerebral Cavernous Malformations.

    Science.gov (United States)

    Scimone, Concetta; Bramanti, Placido; Alafaci, Concetta; Granata, Francesca; Piva, Francesco; Rinaldi, Carmela; Donato, Luigi; Greco, Federica; Sidoti, Antonina; D'Angelo, Rosalia

    2017-02-01

    Cerebral cavernous malformations (CCMs) are lesions affecting brain microvessels. The pathogenesis is not clearly understood. Conventional classification criterion is based on genetics, and thus, familial and sporadic forms can be distinguished; however, classification of sporadic cases with multiple lesions still remains uncertain. To date, three CCM causative genes have been identified: CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10. In our previous mutation screening, performed in a cohort of 95 Italian patients, with both sporadic and familial cases, we identified several mutations in CCM genes. This study represents further molecular screening in a cohort of 19 Italian patients enrolled by us in the few last years and classified into familial, sporadic and sporadic with multiple lesions cases. Direct sequencing and multiplex ligation-dependent probe amplification (MLPA) analysis were performed to detect point mutations and large genomic rearrangements, respectively. Effects of detected mutations and single-nucleotide polymorphisms (SNPs) were evaluated by an in silico approach and by western blot analysis. A novel nonsense mutation in CCM1 and a novel missense mutation in CCM2 were detected; moreover, several CCM2 gene polymorphisms in sporadic CCM patients were reported. We believe that these data enrich the mutation spectrum of CCM genes, which is useful for genetic counselling to identify both familial and sporadic CCM cases, as early as possible.

  3. Cerebral Cavernous Malformation: A Portuguese Family with a Novel CCM1 Mutation

    Science.gov (United States)

    Marto, João Pedro; Gil, Inês; Calado, Sofia; Viana-Baptista, Miguel

    2016-01-01

    Introduction Cerebral cavernous malformation (CCM) is a vascular disorder characterized by the presence of central nervous system cavernomas. In familial forms, mutations in three genes (CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10) were identified. We describe a Portuguese family harboring a novel CCM1 mutation. Case Presentation The proband is a woman who at the age of 55 years started to have complex partial seizures and episodic headache. Although nothing was found during her neurological examination, brain MRI showed bilateral, supra- and infratentorial cavernomas. She had a sister who, at the age 61 years, suffered a tonic-clonic seizure. Neurological examination was normal and imaging investigation demonstrated a right frontal intracerebral hemorrhage and multiple cavernomas. In the following years, she suffered several complex partial seizures and had a new intracerebral hemorrhage located in the right temporal lobe. Genetic analysis was performed and a novel nucleotide substitution, i.e. c.1927C>T (p.Gln643*) within the exon 17 of the CCM1 gene, was detected in both sisters. The substitution encodes a stop codon, with a consequent truncated KRIT1 protein, therefore supporting its pathogenic role. Further affected family members were detected, suggesting an autosomal dominant pattern of inheritance. Conclusion We report a Portuguese family with a novel CCM1 (KRIT1) mutation – c.1927C>T (p.Gln643*). A better knowledge of the phenotype-genotype correlation is needed to improve the management of CCM patients.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  5. Congenital cerebral malformations and dysfunction in fetuses and newborns following the 2013 to 2014 Zika virus epidemic in French Polynesia.

    Science.gov (United States)

    Besnard, Marianne; Eyrolle-Guignot, Dominique; Guillemette-Artur, Prisca; Lastère, Stéphane; Bost-Bezeaud, Frédérique; Marcelis, Ludivine; Abadie, Véronique; Garel, Catherine; Moutard, Marie-Laure; Jouannic, Jean-Marie; Rozenberg, Flore; Leparc-Goffart, Isabelle; Mallet, Henri-Pierre

    2016-01-01

    We detected an unusual increase in congenital cerebral malformations and dysfunction in fetuses and newborns in French Polynesia, following an epidemic of Zika virus (ZIKV), from October 2013 to March 2014. A retrospective review identified 19 cases, including eight with major brain lesions and severe microcephaly, six with severe cerebral lesions without microcephaly and five with brainstem dysfunction without visible malformations. Imaging revealed profound neurological lesions (septal and callosal disruption, ventriculomegaly, abnormal neuronal migration, cerebellar hypoplasia, occipital pseudocysts, brain calcifications). Amniotic fluid was drawn from seven cases at gestation weeks 20 to 29. ZIKV RNA was detected by RT-PCR and infectious ZIKV isolates were obtained in four of five microcephalic, but not in two non-microcephalic cases with severe brain lesions. Medical termination of pregnancy was performed in eleven cases; two cases with brainstem dysfunction died in the first months of life; six cases are alive, with severe neurological impairment. The results show that four of seven tested fetuses with major neurological injuries were infected with ZIKV in utero. For other non-microcephalic, congenital abnormalities we were not able to prove or exclude ZIKV infection retrospectively. The unusual occurrence of brain malformations or dysfunction without microcephaly following a ZIKV outbreak needs further studies.

  6. [Arteriovenous malformation in the mandibula

    NARCIS (Netherlands)

    Kruizinga, E.H.; Meijer, G.J.; Koole, R.; Es, R.J. van

    2007-01-01

    A 15-year-old autistic boy of subnormal intelligence appeared at the office of his dental practitioner complaining about pain in the third quadrant. Investigation revealed that the second molar could be intruded easily. No other irregularities, such as caries or periodontal disease were apparent. Du

  7. Demonstration of cerebral vessels by multiplane computed cerebral angiotomography

    Energy Technology Data Exchange (ETDEWEB)

    Asari S.; Satch, T.; Sakurai, M.; Yamamoto, Y. (Matsuyama Shimin Hospital, Matsuyama (Japan)); Sadamoto, K.

    1981-06-01

    1. Cerebral arteries and veins were demonstrated by multiplane computed cerebral angiotomography (combination of axial, modified coronal, half axial (Towne), and semisagittal planes). The vessels which were demonstrated by various planes were as follows: Axial plane: Willis ring, middle cerebral arteries (horizontal and insular portions), anterior cerebral arteries (Horizontal and ascending portions), posterior cerebral arteries, basal vein of Rosenthal, internal cerebral veins (and the subependymal veins which join the ICV), and vein of Galen. Coronal plane: intermal carotid arteries (supraclinoid portion), anterior cerebral arteries (horizontal portion), middle cerebral arteries (horizontal and insular portions), lenticulostriate arteries, basal vein of Rosenthal (and the subependymal veins which join this vessel), internal cerebral veins, and vein of Galen. Half axial plane (Towne projection): basilar artery, vertebral arteries, posterior cerebral arteries, superior cerebellar arteries, middle cerebral arteries (horizontal portion), and anterior cerebral arteries (horizontal and ascending portions). Semisagittal plane: internal carotid artery (supraclinoid portion), posterior communicating artery, posterior carebral artery, superior cerebellar artery, internal cerebral vein, basal vein of Rosenthal, vein of Galen, and straight shinus. 2. A detailed knowledge of normal cerebrovascular structures acquired by computed tomography (CT) is essential in detecting and more precisely localizing lesions such as cerebrovascular disease, neoplasm or abscess, in differentiating these lesions from the normal contrast-enhanced structures, and in understanding the spatial relationship between the mass lesion and the neighboring vessels. In addition, it will be possible to discover such asymptomatic cerebrovascular diseases as non-ruptured aneurysms, arteriovenous malformations, and Moyamoya disease by means of computed cerebral angiotomography.

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

    Science.gov (United States)

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

    2016-08-05

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

  9. ADVANCED MAGNETIC RESONANCE IMAGING OF CEREBRAL CAVERNOUS MALFORMATIONS: I. HIGH FIELD IMAGING OF EXCISED HUMAN LESIONS

    Science.gov (United States)

    Shenkar, Robert; Venkatasubramanian, Palamadai N.; Zhao, Jin-cheng; Batjer, H. Hunt; Wyrwicz, Alice M.; Awad, Issam A.

    2008-01-01

    Objectives We hypothesized that structural details would be revealed in cerebral cavernous malformations (CCMs) through the use of high field magnetic resonance (MR) and confocal microscopy, which have not been described previously. The structural details of CCMs excised from human patients were sought by examination with high field MR imaging, and correlated with confocal microscopy of the same specimens. Novel features of CCM structure are outlined, including methodological limitations, venues for future research and possible clinical implications. Methods CCM lesions excised from four patients were fixed in 2% paraformaldehyde and subjected to high resolution MR imaging at 9.4 or 14.1 Tesla by spin-echo and gradient recalled echo methods. Histological validation of angioarchitecture was conducted on thick sections of CCM lesions using fluorescent probes to endothelium under confocal microscopy. Results Images of excised human CCM lesions were acquired with proton density-weighted, T1-weighted, T2-weighted spin echo and T2*-weighted gradient-recalled echo MR. These images revealed large “bland” regions with thin walled caverns, and “honeycombed” regions with notable capillary proliferation and smaller caverns surrounding larger caverns. Proliferating capillaries and caverns of various sizes were also associated with the wall of apparent larger blood vessels in the lesions. Similar features were confirmed within thick sections of CCMs by confocal microscopy. MR relaxation times in different regions of interest suggested the presence of different states of blood breakdown products in areas with apparent angiogenic proliferative activity. Conclusions The high field MR imaging techniques demonstrate novel features of CCM angioarchitecture, visible at near histological resolution, including regions with apparently different biologic activity. These preliminary observations will motivate future research, correlating lesion biologic and clinical activity with

  10. 儿童期颅内动静脉畸形临床及形态学分析%Clinical and morphological analysis of intracranial arteriovenous malformation in childhood

    Institute of Scientific and Technical Information of China (English)

    沈文俊; 李秋平; 李昊

    2014-01-01

    Objective To discuss the clinical manifestations and the morphological features of intracranial arteriovenous malformation (AVM) in childhood. Methods During the period from Jan. 2012 to June 2013, a total of 14 AVM patients under the age of 14 years were admitted to authors’ hospital. Of the 14 patients, 11 had a history of spontaneous intracerebral hemorrhage (78.6%), 7 complained of headache (50.5% ), 5 showed signs of unilateral limb weakness (35.7% ), and 2 was accompanied with epileptic attacks (14.3% ). The clinical characteristics, the preoperative imaging features and DSA findings were retrospectively analyzed. Results DSA showed that terminal feeding branches were visualized in 9 cases and blood supply from perforating branches was seen in the remaining 5 cases. In seven cases the feeding artery was single, while in the remaining 7 cases the feeding artery was multiple, and in four of them combination blood supply from both anterior and posterior circulation was found. The lesions were situated at the lateral or convex surface of cerebral hemisphere (n = 5), lateral ventricle (n = 4), cerebral bottom (n = 3) or medial surface of cerebral hemisphere (n = 2). The lesion’s diameter was < 30 mm in 10 cases and (30 -60) mm in 4 cases. According to Spetzler- Martin grading, grade Ⅰ was seen in 4 cases, grade Ⅱ in 2 cases, grade Ⅲ in 5 cases and grade Ⅳ in 3 cases. Conclusion Although in childhood AVM lesion is small in size and lower in grading, its onset is early and the lesion is prone to bleeding. DSA findings indicate that most AVMs in childhood take the form of immature type. Nevertheless, its natural course needs to be further studied.%目的:探讨儿童期颅内动静脉畸形(AVM)的临床特点及形态学表现。方法2012年1月-2013年6月收治14岁以下颅内 AVM 患儿14例,其中11例(78.6%)曾有自发性脑出血病史,头痛7例(50.0%),单侧肢体乏力5例(35.7%),2例(14.3%)伴有癫痫

  11. A tiny dural arteriovenous fistula

    Institute of Scientific and Technical Information of China (English)

    ZHANG Peng 张 鹏; ZHU Fengshui 朱风水; LING Feng 凌 锋; Christophe COGNARD

    2003-01-01

    @@ Pulsatile tinnitus is commonly encountered in approximately 10% of a given population.1 Since causes of the disease vary, selecting appropriate protocols of imaging strategies is quite challenging.2 Vascular anormalies or diseases including anormalies of the carotid arteries and jugular veins, intracranial arteriovenous malformation and dural arteriovenous fistula (DAVF) are major causative factors of the disease. Before imaging studies, history inquiry and physical examination are important for detect the possible causes of pulsatile tinnitus. Different imaging examinations are depended on histories and clinical signs of different patients.

  12. Surgical management of a pial arteriovenous fistula with giant varix in an infant

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    Pillai Ashok

    2006-01-01

    Full Text Available A seven-month-old infant presented with a generalized seizure. The radiological evaluation revealed a large arteriovenous fistula in the left sylvian region. The fistula connected the left middle cerebral artery and the vein of Trolard. A giant varix was present at the venous end. The child underwent craniotomy, complete disconnection of the fistula and excision of the varix. Except for recurrent seizures, which were eventually controlled on anticonvulsants, the child′s neurological development has been good on long-term follow-up of three years. Pial arteriovenous fistulae are rare intracranial vascular malformations. Though significant operative risks exist, they can be successfully managed surgically with good long-term prognosis.

  13. Arteriovenous Fistula

    Science.gov (United States)

    ... home. Accessed Feb. 23, 2015. Vascular access for hemodialysis. National Kidney and Urologic Diseases Information Clearinghouse. http:// ... www.mayoclinic.org/diseases-conditions/arteriovenous-fistula/basics/definition/CON-20034876 . Mayo Clinic Footer Legal Conditions and ...

  14. Pediatric central nervous system vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

    2015-09-15

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

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

    Energy Technology Data Exchange (ETDEWEB)

    Marchuk, D.A.; Gallione, C.J. [Duke Univ. Medical Center, Durham, NC (United States); Morrison, L.A.; Davis, L.E.; Clericuzio, C.L. [Univ. of New Mexico School of Medicine, Albuquerque, NM (United States)] [and others

    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.

  16. Developmental malformations of the cerebral cortex; Heterotopie, Polymikrogyrie, Lissenzephalie und Co. Malformationen der kortikalen Hirnentwicklung

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    Reiss-Zimmermann, Martin [Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie, Universitaetsklinikum Leipzig AoeR (Germany); Weber, D.; Sorge, I.; Hirsch, W. [Abt. Paediatrische Radiologie, Universitaetsklinikum Leipzig AoeR (Germany); Merkenschlager, A. [Universitaetsklinik und Poliklinik fuer Kinder und Jugendliche, Universitaetsklinikum Leipzig AoeR (Germany)

    2010-06-15

    Migration disorders (MD) are increasingly recognized as an important cause of epilepsy and developmental delay. Up to 25% of children with refractory epilepsy have a cortical malformation. MD encompass a wide spectrum with underlying genetic etiologies and clinical manifestations. Research regarding the delineation of the genetic and molecular basis of these disorders has provided greater insight into the pathogenesis of not only the malformation but also the process involved in normal cortical development. Diagnosis of MD is important since patients who fail three antiepileptic medications are less likely to have their seizures controlled with additional trials of medications and therefore epilepsy surgery should be considered. Recent improvements in neuroimaging have resulted in a significant increase in the recognition of MD. Findings can be subdivided in disorders due to abnormal neurogenesis, neuronal migration, neuronal migration arrest and neuronal organization resulting in different malformations like microcephaly, lissencephaly, schizencephaly and heterotopia. The examination protocol should include T1-w and T2-w sequences in adequate slice orientation. T1-w turbo-inversion recovery sequences (TIR) can be helpful to diagnose heterotopia. Contrast agent is needed only to exclude other differential diagnoses. (orig.)

  17. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance.

    Science.gov (United States)

    Gault, Judith; Sarin, Hemant; Awadallah, Nabil A; Shenkar, Robert; Awad, Issam A

    2004-07-01

    Cerebrovascular malformations affect more than 3% of the population, exposing them to a lifetime risk of hemorrhagic stroke, seizures, and focal neurological deficits. Cerebral cavernous malformations (CCMs) exhibit an immature vessel wall, a brittle hemorrhagic tendency, and epileptogenesis, whereas arteriovenous malformations (AVMs) lack capillary beds and manifest apoplectic bleeding under high-flow conditions. There are also more benign venous anomalies, capillary malformations, and lesions with mixed and transitional features. Advances have been made toward understanding the natural history, radiological and pathological correlates, and clinical management. Yet, mechanisms of lesion genesis and clinical manifestations remain largely unknown, and the clinical behavior in individual patients is highly unpredictable. Lesion pathogenesis likely involves abnormal assembly or maintenance of blood vessels, resulting in dysmorphic vessel phenotypes. Familial CCM disease is in part caused by mutations in a cytoskeletal-related protein that is likely integral to interendothelial cell connectivity and maturation of the vascular wall. Rare familial forms of AVM disease have been correlated with two different transforming growth factor-beta receptor components, possibly causing disturbance in signaling during vascular assembly. Relevance of these mechanisms to the more common and otherwise identical sporadic CCM and AVM lesions is being explored. In this report, basic mechanisms of vasculogenesis and angiogenesis and how they possibly relate to the common cerebrovascular malformation lesions are reviewed. Novel concepts are discussed related to the cellular, molecular, and genetic substrates in CCM and AVM as well as to how this knowledge can be applied to predict, explain, and possibly modify clinical disease manifestations.

  18. Advances in diagnosis and treatment of cerebral venous system diseases

    Directory of Open Access Journals (Sweden)

    Xiao-yun LIU

    2016-11-01

    Full Text Available Cerebral venous system diseases include cerebral venous thrombosis (CVT, venous sinus stenosis, carotid cavernous fistula (CCF, intracranial arteriovenous malformation (AVM and so on. In recent years, due to the rapid development of neuroimaging and interventional technology, more and more cerebral venous system diseases have been timely diagnosed and treated, such as magnetic resonance black-blood thrombus imaging (MRBTI in the diagnosis of CVT, stenting in the treatment of venous sinus stenosis, micro coil plus Onyx glue or covered stents in the treatment of CCF, which allow us to make a deeper recognition of cerebral venous system diseases. Therefore, this paper will introduce the latest diagnosis and treatment of cerebral venous system diseases. DOI: 10.3969/j.issn.1672-6731.2016.11.006

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

    Science.gov (United States)

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

    2011-01-01

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

  20. A novel mouse model of cerebral cavernous malformations based on the two-hit mutation hypothesis recapitulates the human disease.

    Science.gov (United States)

    McDonald, David A; Shenkar, Robert; Shi, Changbin; Stockton, Rebecca A; Akers, Amy L; Kucherlapati, Melanie H; Kucherlapati, Raju; Brainer, James; Ginsberg, Mark H; Awad, Issam A; Marchuk, Douglas A

    2011-01-15

    Cerebral cavernous malformations (CCMs) are vascular lesions of the central nervous system appearing as multicavernous, blood-filled capillaries, leading to headache, seizure and hemorrhagic stroke. CCM occurs either sporadically or as an autosomal dominant disorder caused by germline mutation of one of the three genes: CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10. Surgically resected human CCM lesions have provided molecular and immunohistochemical evidence for a two-hit (germline plus somatic) mutation mechanism. In contrast to the equivalent human genotype, mice heterozygous for a Ccm1- or Ccm2-null allele do not develop CCM lesions. Based on the two-hit hypothesis, we attempted to improve the penetrance of the model by crossing Ccm1 and Ccm2 heterozygotes into a mismatch repair-deficient Msh2(-/-) background. Ccm1(+/-)Msh2(-/-) mice exhibit CCM lesions with high penetrance as shown by magnetic resonance imaging and histology. Significantly, the CCM lesions range in size from early-stage, isolated caverns to large, multicavernous lesions. A subset of endothelial cells within the CCM lesions revealed somatic loss of CCM protein staining, supporting the two-hit mutation mechanism. The late-stage CCM lesions displayed many of the characteristics of human CCM lesions, including hemosiderin deposits, immune cell infiltration, increased endothelial cell proliferation and increased Rho-kinase activity. Some of these characteristics were also seen, but to a lesser extent, in early-stage lesions. Tight junctions were maintained between CCM lesion endothelial cells, but gaps were evident between endothelial cells and basement membrane was defective. In contrast, the Ccm2(+/-)Msh2(-/-) mice lacked cerebrovascular lesions. The CCM1 mouse model provides an in vivo tool to investigate CCM pathogenesis and new therapies.

  1. The Role of Hemosiderin Excision in Seizure Outcome in Cerebral Cavernous Malformation Surgery: A Systematic Review and Meta-Analysis.

    Directory of Open Access Journals (Sweden)

    Di Ruan

    Full Text Available Whether the excision of hemosiderin surrounding cerebral cavernous malformations (CCMs is necessary to achieve a seizure-free result has been the subject of debate. Here, we report a systematic review of related literature up to Jan 1, 2015 including 594 patients to assess the effect of hemosiderin excision on seizure outcome in patients with CCMs by meta-analysis.Ten studies comparing extended hemosiderin excision with only lesion resection were identified by searching the English-language literature. Meta-analyses, subgroup analyses and sensitivity analysis were conducted to determine the association between hemosiderin excision and seizure outcome after surgery.Seizure outcome was significantly improved in the patients who underwent an extended excision of the surrounding hemosiderin (OR, 0.62; 95% CI: 0.42-0.91; P = 0.01. In subgroup analysis, studies from Asia (OR, 0.42; 95% CI: 0.25-0.71; P = 0.001, male-majority (female ratio 1 year before surgery (OR, 0.43; 95% CI: 0.22-0.84; P = 0.01, lesion diameter > 2 cm (OR, 0.41; 95% CI: 0.19-0.87; P = 0.02 and short-term (< 3 years follow-up (OR, 0.48; 95% CI: 0.29-0.80; P = 0.005 tended to correlate with a significantly favorable outcome.Patients who underwent extended surrounding hemosiderin excision could exhibit significantly improved seizure outcomes compared to patients without hemosiderin excision. However, further well-designed prospective multiple-center RCT studies are still needed.

  2. Features of a Chinese family with cerebral cavernous malformation induced by a novel CCM1 gene mutation

    Institute of Scientific and Technical Information of China (English)

    WANG Xue; LIU Xue-wu; Nora Lee; LIU Qi-ji; LI Wen-na; HAN Tao; WEI Kun-kun

    2013-01-01

    Background Familial cerebral cavernous malformations (CCMs),characterized by hemorrhagic stroke,recurrent headache and epilepsy,are congenital vascular anomalies of the central nervous system.Familial CCMs is an autosomal dominant inherited disorder and three CCM genes have been identified.We report a Chinese family with CCMs and intend to explore clinical,pathological,magnetic resonance imaging (MRI) features and pathogenic gene mutation of this family.Methods Totally 25 family members underwent brain MRI examination and clinical check.Two patients with surgical indications had surgical treatment and the specimens were subjected to histopathological and microstructural examination.In addition,polymerase chain reaction (PCR) and direct sequencing were performed with genomic DNA extracted from 25 family members' blood samples for mutation detection.Results Brain MRI identified abnormal results in seven family members.All of them had multiple intracranial lesions and four cases had skin cavernous hemangioma.T2-weighted sequence showed that the lesions were typically characterized by an area of mixed signal intensity.Gradient-echo (GRE) sequence was more sensitive to find microcavernous hemangiomas.There was a wide range in the clinical manifestations as well as the age of onset in the family.The youngest patient was an 8-year-old boy with least intracranial lesions.Histopathological and microstructural examination showed that CCMs were typically discrete multi-sublobes of berry-like lesions,with hemorrhage in various stages of illness evolution.They were formed by abnormally enlarged sinusoids and the thin basement membranes.A novel T deletion mutation in exon 14 of CCM1 gene was identified by mutation detection in the seven patients.But unaffected members and healthy controls did not carry this mutation.Conclusions The clinical manifestations were heterogenic within this family.We identified a novel mutation (c.1396delT) was the disease-causing mutation for this

  3. The Rendu-Osler-Weber Disease Revealed by a Refractory Hypoxemia and Severe Cerebral Fat Embolism

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    Leonel Barreto

    2013-01-01

    Full Text Available The Rendu-Osler-Weber disease is a genetic disease which may lead to severe hemorrhage and less frequently to severe organ dysfunction. We report the case of a 22-year-old patient with no personal medical history who was involved in a motorcycle accident and exhibited severe complications related to large arteriovenous pulmonary shunts during his ICU stay. The patient developed an unexplained severe hypoxemia which was attributed to several arteriovenous shunts of the pulmonary vasculature by a contrast study during a transesophageal echocardiographic examination. The course was subsequently complicated by a prolonged coma associated with hemiplegia which was attributed to a massive paradoxical fat embolism in the setting of an untreated femoral fracture. In addition to hemorrhagic complications which may lead to intractable shock, arteriovenous malformations associated with the Rendu-Osler-Weber disease may involve the pulmonary vasculature and result in unexpected complications, such as hypoxemia or severe cerebral fat embolism in high-risk patients.

  4. Assessment of the arteriovenous cerebrovascular system by multi-slice CT. A single-bolus, monophasic protocol

    Energy Technology Data Exchange (ETDEWEB)

    Klingebiel, R.; Zimmer, C. [Charite CM, Humboldt Univ., Berlin (Germany). Neuroradiology Section; Rogalla, P.; Kivelitz, D. [Charite CM, Humboldt Univ., Berlin (Germany). Dept. of Radiology; Bohner, G. [Charite CM, Humboldt Univ., Berlin (Germany). Neuroradiology Section; Goetze, R. [Charite CM, Humboldt Univ., Berlin (Germany). Dept. of Radiology; Lehmann, R. [Charite CM, Humboldt Univ., Berlin (Germany). Neuroradiology Section

    2001-11-01

    Purpose: We present a protocol for the non-invasive angiographic assessment of the arterial and venous cerebrovascular (CV) system by multi-slice CT. Material and Methods: Data acquisition was performed in a multi-slice CT scanner with a scan range from the carotid bifurcation to the vertex and manual scan start following i.v. administration of 120 ml iodinated contrast medium with a flow rate of 4 ml/s. This protocol was applied in 12 patients with symptoms of acute CV insufficiency. Results: In all patients, comprehensive imaging of the arteriovenous CV system was achieved including the common carotid bifurcation, the third segment of the major cerebral arteries, the dural sinus and the internal cerebral veins. Various CV pathologies, such as a territorial artery occlusion, a thrombotic obstruction of the internal carotid artery, an intracranial arteriovenous malformation and a sinus vein thrombosis, were successfully evaluated. Conclusion: Comprehensive assessment of the arteriovenous CV system is possible by the use of a single-bolus, monophasic multi-slice scan technique.

  5. Variable expression of cerebral cavernous malformations in carriers of a premature termination codon in exon 17 of the Krit1 gene

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    Gamero Miguel A

    2003-07-01

    Full Text Available Abstract Background Cerebral cavernous malformations (CCM present as either sporadic or autosomal dominant conditions with incomplete penetrance of symptoms. Differences in genetic and environmental factors might be minimized among first-degree relatives. We therefore studied clinical expression in a family with several affected members. Methods We studied a three-generation family with the onset of CCM as a cerebral haemorrhage in the younger (four-year-old sibling. Identification and enumeration of CCMs were performed in T2-weighted or gradient-echo MRIs of the whole brains. Genetic analysis comprised SCCP, sequencing and restriction polymorphism of the Krit1 gene in the proband and at risk relatives. Results The phenotypes of cerebral cavernous malformations (CCMs in carriers of Krit1 mutations were very variable. We identified a novel frameshift mutation caused by a 1902A insertion in exon 17 of the Krit1 gene, which leads to a premature TAA triplet and predicts the truncating phenotype Y634X. A very striking finding was the absence of both clinical symptoms and CCMs in the eldest sibling harbouring the 1902insA. Conclusions Patients in this family, harbouring the same mutation, illustrate the very variable clinical and radiological expression of a Krit1 mutation. The early and critical onset in the proband contrasts with minor clinical findings in affected relatives. This consideration is important in genetic counselling.

  6. Color-coded digital subtraction angiography in the management of a rare case of middle cerebral artery pure arterial malformation. A technical and case report.

    Science.gov (United States)

    Feliciano, Caleb E; Pamias-Portalatin, Eva; Mendoza-Torres, Jorge; Effio, Euclides; Moran, Yadira; Rodriguez-Mercado, Rafael

    2014-12-01

    The advent of flow dynamics and the recent availability of perfusion analysis software have provided new diagnostic tools and management possibilities for cerebrovascular patients. To this end, we provide an example of the use of color-coded angiography and its application in a rare case of a patient with a pure middle cerebral artery (MCA) malformation. A 42-year-old male chronic smoker was evaluated in the emergency room due to sudden onset of severe headache, nausea, vomiting and left-sided weakness. Head computed tomography revealed a right basal ganglia hemorrhage. Cerebral digital subtraction angiography (DSA) showed a right middle cerebral artery malformation consisting of convoluted and ectatic collateral vessels supplying the distal middle cerebral artery territory-M1 proximally occluded. An associated medial lenticulostriate artery aneurysm was found. Brain single-photon emission computed tomography with and without acetazolamide failed to show problems in vascular reserve that would indicate the need for flow augmentation. Twelve months after discharge, the patient recovered from the left-sided weakness and did not present any similar events. A follow-up DSA and perfusion study using color-coded perfusion analysis showed perforator aneurysm resolution and adequate, albeit delayed perfusion in the involved vascular territory. We propose a combined congenital and acquired mechanism involving M1 occlusion with secondary dysplastic changes in collateral supply to the distal MCA territory. Angiographic and cerebral perfusion work-up was used to exclude the need for flow augmentation. Nevertheless, the natural course of this lesion remains unclear and long-term follow-up is warranted.

  7. Arterial oxygen content is precisely maintained by graded erythrocytotic responses in settings of high/normal serum iron levels, and predicts exercise capacity: an observational study of hypoxaemic patients with pulmonary arteriovenous malformations.

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    Vatshalan Santhirapala

    Full Text Available BACKGROUND: Oxygen, haemoglobin and cardiac output are integrated components of oxygen transport: each gram of haemoglobin transports 1.34 mls of oxygen in the blood. Low arterial partial pressure of oxygen (PaO2, and haemoglobin saturation (SaO2, are the indices used in clinical assessments, and usually result from low inspired oxygen concentrations, or alveolar/airways disease. Our objective was to examine low blood oxygen/haemoglobin relationships in chronically compensated states without concurrent hypoxic pulmonary vasoreactivity. METHODOLOGY: 165 consecutive unselected patients with pulmonary arteriovenous malformations were studied, in 98 cases, pre/post embolisation treatment. 159 (96% had hereditary haemorrhagic telangiectasia. Arterial oxygen content was calculated by SaO2 x haemoglobin x 1.34/100. PRINCIPAL FINDINGS: There was wide variation in SaO2 on air (78.5-99, median 95% but due to secondary erythrocytosis and resultant polycythaemia, SaO2 explained only 0.1% of the variance in arterial oxygen content per unit blood volume. Secondary erythrocytosis was achievable with low iron stores, but only if serum iron was high-normal: Low serum iron levels were associated with reduced haemoglobin per erythrocyte, and overall arterial oxygen content was lower in iron deficient patients (median 16.0 [IQR 14.9, 17.4]mls/dL compared to 18.8 [IQR 17.4, 20.1]mls/dL, p<0.0001. Exercise tolerance appeared unrelated to SaO2 but was significantly worse in patients with lower oxygen content (p<0.0001. A pre-defined athletic group had higher Hb:SaO2 and serum iron:ferritin ratios than non-athletes with normal exercise capacity. PAVM embolisation increased SaO2, but arterial oxygen content was precisely restored by a subsequent fall in haemoglobin: 86 (87.8% patients reported no change in exercise tolerance at post-embolisation follow-up. SIGNIFICANCE: Haemoglobin and oxygen measurements in isolation do not indicate the more physiologically relevant

  8. Malformação arteriovenosa uterina após doença trofoblástica gestacional Uterine arteriovenous malformation after gestational trophoblastic disease

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    Paulo Belfort

    2006-02-01

    Full Text Available OBJETIVO: investigar a presença e resultados de malformações vasculares uterinas (MAVU após doença trofoblástica gestacional (DTG. MÉTODOS: estudo retrospectivo com inclusão de casos diagnosticados entre 1987 e 2004; 2764 pacientes após DTG foram acompanhadas anualmente com ultra-sonografia transvaginal e Doppler colorido no Centro de Neoplasia Trofoblástica Gestacional da Santa Casa da Misericórdia (Rio de Janeiro, RJ, Brasil. Sete pacientes tiveram diagnóstico final de MAVU baseado em análise ultra-sonográfica - índice de pulsatilidade (IP, índice de resistência (IR e velocidade sistólica máxima (VSM - e achados de imagens de ressonância nuclear magnética (RNM. Dosagens negativas de beta-hCG foram decisivas para estabelecer o diagnóstico diferencial com DTG recidivante. RESULTADOS: a incidência de MAVU após DTG foi 0,2% (7/2764. Achados ultra-sonográficos de MAVU: IP médio de 0,44±0,058 (extremos: 0,38-0,52; IR médio de 0,36±0,072 (extremos: 0,29-0,50; VSM média de 64,6±23,99 cm/s (extremos: 37-96. A imagem de RNM revelou útero aumentado, miométrio heterogêneo, espaços vasculares tortuosos e vasos parametriais com ectasia. A apresentação clínica mais comum foi hemorragia transvaginal, presente em 52,7% (4/7 dos casos. Tratamento farmacológico com 150 mg de acetato de medroxiprogesterona foi empregado para controlar a hemorragia, após a estabilização hemodinâmica. Permanecem as pacientes em seguimento, assintomáticas até hoje. Duas pacientes engravidaram com MAVU, com gestações e partos exitosos. CONCLUSÃO: presente sangramento transvaginal em pacientes com beta-hCG negativo e história de DTG, deve-se considerar a possibilidade de MAVU e solicitar avaliação ultra-sonográfica com dopplervelocimetria. O tratamento conservador é a melhor opção na maioria dos casos de MAVU pós-DTG.PURPOSE: to investigate the presence and outcome of uterinevascular malformations (UVAM after gestational

  9. MRI对脑静脉畸形的诊断价值%Diagnostic value of MRI in cerebral venous malformation

    Institute of Scientific and Technical Information of China (English)

    牛磊; 朱蒙蒙; 王明皓; 崔文; 刘圆圆; 朱斌

    2011-01-01

    目的:探讨MRI对脑静脉畸形(CVM)的诊断价值.方法:收集本院18例脑静脉畸形病例,均行磁共振T1WI、T2WI、FLAIR、SWI、DWI、MRA及T1 Wl增强扫描的资料.结果:CVM位于幕下5例,幕上13例,18例均呈典型的“海蛇头”征.MRI显示髓静脉呈长T1长T2信号,引流静脉呈短T1稍长T2信号或长T1长T2信号.FLAIR上髓静脉为稍高信号,引流静脉为低信号或稍高信号.SWI及DWI上髓静脉和引流静脉均为低信号.增强呈“海蛇头”样改变,即多条髓静脉呈辐射状汇入粗大的引流静脉.结论:MRI能明确诊断脑静脉畸形.T1WI增强扫描及SWI均较敏感.SWI对CVM的诊断价值明显优于常规MRI序列.%Objective:To investigate the diagnostic value of MRI in cerebral venous malformations (CVM). Methods: 18 patients of CVM were collected, and all of them underwent T1 WI, T2WI. FLAIR. SWI. DWI. MRA. And T,W1 enhancement scans. Results:CVMs were located in infra in five cases, and supra tentorium in thirteen eleven patients. Eighteen presented the typical " caput medusae" appearance. As for MRI plain scans.the medullary veins showed hypointensity in T1 WI and hyperintensity in T2WI, while, the drainage veins featured hyperintensity in T1 WI and hyperintensity in T2 WI. FLAIR sequences manifested less medullary veins with hyperintensity and drainage veins with hypointensity or hyperintensity. Both the medullary and the drainage veins appeared hypointensity in DWI and SWI. After enhancement, numerous radiating medullary veins gathered together into one or two larger drainage veins and flowed into superficial or deeper veins. Demonstrating the so-called "Caput Medusae" appearance. Conclusion: MRI is the first choice for suspected CVM patients thus providing more diagnostic information. T1 WI enhancement and SWI are sensitive to manifesting CVMs. SWI diagnosis of CVM is significantly superior to conventional MRI sequences.

  10. Brainstem cavernous malformations: a review with two case reports Malformações cavernosas do tronco cerebral: uma revisão com relato de dois casos

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    Adolfo Ramírez-Zamora

    2009-09-01

    Full Text Available Central nervous system (CNS cavernous malformations (CMs are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.Malformações cavernosas (MFC do sistema nervoso central são malformações do desenvolvimento do leito vascular com múltiplas apresentações clínicas devido a sua natureza dinâmica. Apresentamos dois casos de malformações cavernosas do tronco cerebral: o primeiro após radioterapia e o segundo em paciente grávida com hemorragia intracraniana sintomática. MFC são responsáveis por cerca de 8-15% de todas as malformações vasculares. Embora tradicionalmente sejam genéticas, as MFC podem também ser adquiridas, particularmente após radioterapia. As manifestações clínicas são variáveis e o tratamento cirúrgico deve ser considerado para pacientes com quadros neurológicos progressivos.

  11. Cerebral Cavernous Malformations (CCM)

    Science.gov (United States)

    ... all children with HHT should have the same screening for lung AVMs recommended for adults. Children can be safely treated with embolization. ? What are the symptoms or signs of HHT in the gastrointestinal tract (stomach and intestines)? Unexplained anemia or low blood count, ...

  12. Renal cirsoid arteriovenous malformation masquerading as neoplasia.

    Science.gov (United States)

    Silverthorn, K; George, D

    1988-12-01

    A woman with renal colic and microscopic hematuria had filling defects in the left renal collecting system detected on excretory urography. A nephrectomy, performed because of suspected malignancy, might have been averted by renal angiography.

  13. Diagnosis and evaluation of intracranial arteriovenous malformations

    Directory of Open Access Journals (Sweden)

    Andrew Conger

    2015-01-01

    Conclusion: AVMs are a heterogenous pathology with three different options for treatment. Accurate assessment of risk of observation and risk of treatment is essential for achieving the best outcome for each patient.

  14. Onyx embolization by distant injection combined with gamma-knife surgery in treatment of brain arte-riovenous malformations%远距离注射 Onyx 胶介入栓塞联合伽玛刀治疗脑动静脉畸形

    Institute of Scientific and Technical Information of China (English)

    张龙; 刘保国; 李伟光; 冯文峰; 李平安; 冯金福; 肖罡; 何黎明

    2015-01-01

    目的:探讨采用远距离注射Onyx胶介入栓塞联合伽玛刀治疗脑动静脉畸形(AVM)的临床应用价值。方法采用远距离注射 Onyx 胶介入栓塞13例脑 AVM,残留畸形血管团行伽玛刀治疗。采用格拉斯哥预后量表(GOS)评估出院情况,使用改良 Rankin 量表(mRS)评价预后。结果13例脑 AVM患者共计实施16次栓塞手术,栓塞结束后即刻复查脑血管造影,畸形血管团体积缩小25%~85%,平均缩小(50.3±17.9)%。均未出现与栓塞相关的并发症,1例伽玛刀治疗后出现放射性脑水肿。出院时,GOS 达5分11例(85%)。随访6~18个月,其中11例脑 AVM完全不显影,2例脑 AVM较出院时明显缩小,mRS 小于3分12例。结论采用远距离注射 Onyx 胶介入栓塞治疗脑AVM安全可行,可减少术者射线暴露风险,联合伽玛刀治疗可获得理想的临床疗效。%Objective To evaluate the clinical value of Onyx embolization by distant injection in combination with gamma-knife surgery in treating brain arteriovenous malformation (AVM).Methods Thir-teen patients with brain AVMwere treated with distant injection of Onyx and the residual brain AVMwas sub-ject to gamma-knife surgery.Clinical status at discharge was evaluated using Glasgow Outcome Scale (GOS). Clinical prognosis was assessed by modified Rankin Scale (mRS).Results All 13 AVM patients underwent embolization surgery for 16 times.Cerebral angiography was performed immediately after surgery and revealed that the volume of the AVMwas reduced by 25%-85%,(50.3 ±17.9)on average.No embolization-related complications were noted.Brain edema was found in one patient after undergoing gamma-knife surgery.Upon discharge,11 patients (85%)had GOS score >5.Postoperative follow-up endured from 6 to 18 months. During follow-up,AVMof 11 cases were completely occluded,the volume of AVMnidus was obviously shrank in 2 cases,and 12 patients had mRS score <3

  15. Perkutan transluminal embolisering af pulmonale arteriovenøse misdannelser

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Andersen, P E; Oxhøj, H

    1998-01-01

    A series of nine patients with pulmonary arteriovenous malformations (PAVM) treated with embolotherapy at Odense University Hospital is presented. In all patients the arterial oxygen tension increased after embolisation. PAVM causes right-to-left shunting, which may result in severe hypoxaemia, and...

  16. The return of an old worm: cerebral paragonimiasis presenting with intracerebral hemorrhage.

    Science.gov (United States)

    Koh, Eun Jung; Kim, Seung-Ki; Wang, Kyu-Chang; Chai, Jong-Yil; Chong, Sangjoon; Park, Sung-Hye; Cheon, Jung-Eun; Phi, Ji Hoon

    2012-11-01

    Paragonimiasis is caused by ingesting crustaceans, which are the intermediate hosts of Paragonimus. The involvement of the brain was a common presentation in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain imaging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by the hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. This case alerts practicing clinicians to the domestic transmission of a forgotten parasitic disease due to environmental changes.

  17. Optimizing Arteriovenous Fistula Maturation

    OpenAIRE

    2009-01-01

    Autogenous arteriovenous fistulas are the preferred vascular access in patients undergoing hemodialysis. Increasing fistula prevalence depends on increasing fistula placement, improving the maturation of fistula that fail to mature and enhancing the long-term patency of mature fistula. Percutaneous methods for optimizing arteriovenous fistula maturation will be reviewed.

  18. Contrast-enhanced sonography as a novel tool for assessment of vascular malformations

    Directory of Open Access Journals (Sweden)

    Oe Yukiko

    2010-11-01

    Full Text Available Abstract Background Vascular malformations with arteriovenous shunt components can cause significant disability, chronic pain, and functional impairment. Effective treatment may require serial procedures, yet an imaging modality optimized to control cost and reduce radiation exposure in this predominantly pediatric population has not yet been identified. Methods and Results We describe the use of contrast-enhanced sonography as a novel tool to define vascular anatomy and localize arteriovenous shunting in a young patient with a symptomatic vascular malformation. Conclusions This method may effectively reduce radiation exposure and cost, and additionally provide unique information about arteriovenous shunting, offering a novel imaging application for patients with these conditions.

  19. Consensus Document of the International Union of Angiology (IUA)-2013. Current concept on the management of arterio-venous management

    NARCIS (Netherlands)

    Lee, B.B.; Baumgartner, I.; Berlien, H.P.; Bianchini, G.; Burrows, P.; Do, Y.S.; Ivancev, K.; Schultze Kool, L.J.; Laredo, J.; Loose, D.A.; Lopez-Gutierrez, J.C.; Mattassi, R.; Parsi, K.; Rimon, U.; Rosenblatt, M.; Shortell, C.; Simkin, R.; Stillo, F.; Villavicencio, L.; Yakes, W.

    2013-01-01

    Arterio-venous malformations (AVMs) are congenital vascular malformations (CVMs) that result from birth defects involving the vessels of both arterial and venous origins, resulting in direct communications between the different size vessels or a meshwork of primitive reticular networks of dysplastic

  20. 辅助技术在颅内动静脉畸形手术中的应用%Strategy for assisted cerebral arteriovenons malformation surgery

    Institute of Scientific and Technical Information of China (English)

    王硕; 刘泠; 赵元立; 张东; 王嵘; 赵继宗

    2010-01-01

    目的 探讨B超、神经导航以及吲哚菁绿血管造影在颅内动静脉畸形手术中的应用价值.方法 回顾性分析2009年1至10月北京天坛医院神经外科脑血管病区收治的43例颅内动静脉畸形患者临床资料.其中男26例,女17例;年龄8~53岁,平均26.51岁.所有患者在手术中分别或联合采用B超、神经导航或吲哚菁绿血管造影辅助病灶切除,且术后全部病例行脑血管造影(DSA)或CTA检查,同时记录术后新发并发症及恢复情况.结果 所有患者均全切病灶,术中辅助技术检测结果均与术后DSA或CTA吻合.术后新发神经功能障碍3例(7.0%),其中2例出现双侧视野同向性偏盲,另1例为复视.43例患者KPS(Karnofsky Performance Scale)评分均高于80分.结论 采用B超、神经导航或吲哚菁绿血管造影技术辅助颅内动静脉畸形手术切除,均有助于鉴别血管性质和反映病灶边界.但其侧重各有不同.因此,该3种辅助技术有选择的联合应用可以使术者增加术中判断的准确性,从而收到最佳手术效果.%Objective To evaluate the surgical effects of such adjuvant techniques as type B ultrasound, neuronavigation and intraoperative indocyanine green vedioangiography (ICGA) on intracranial arteriovenous malformation ( AVM) surgery. Methods From January to October 2009, 43 AVM patients treated at our center were retrospectively studied, including 26 males and 17 females with an age range of 8 to S3 years old (mean; 26. 51). Type B ultrasound, neuronavigation or ICCA were employed during surgical procedures in all cases. Intraoperative findings were compared with those of postoperative digital subtraction angiography ( DSA) or computed tomography angiography (CTA) . And the clinical outcomes were evaluated. Results As confirmed by postoperative DSA or CTA, the lesions of all 43 patients were totally removed. Only 3 patients (7.0%) suffered from new neurological deficits, including homonymous hemianopia

  1. Lesions from patients with sporadic cerebral cavernous malformations harbor somatic mutations in the CCM genes: evidence for a common biochemical pathway for CCM pathogenesis.

    Science.gov (United States)

    McDonald, David A; Shi, Changbin; Shenkar, Robert; Gallione, Carol J; Akers, Amy L; Li, Stephanie; De Castro, Nicholas; Berg, Michel J; Corcoran, David L; Awad, Issam A; Marchuk, Douglas A

    2014-08-15

    Cerebral cavernous malformations (CCMs) are vascular lesions affecting the central nervous system. CCM occurs either sporadically or in an inherited, autosomal dominant manner. Constitutional (germline) mutations in any of three genes, KRIT1, CCM2 and PDCD10, can cause the inherited form. Analysis of CCM lesions from inherited cases revealed biallelic somatic mutations, indicating that CCM follows a Knudsonian two-hit mutation mechanism. It is still unknown, however, if the sporadic cases of CCM also follow this genetic mechanism. We extracted DNA from 11 surgically excised lesions from sporadic CCM patients, and sequenced the three CCM genes in each specimen using a next-generation sequencing approach. Four sporadic CCM lesion samples (36%) were found to contain novel somatic mutations. Three of the lesions contained a single somatic mutation, and one lesion contained two biallelic somatic mutations. Herein, we also describe evidence of somatic mosaicism in a patient presenting with over 130 CCM lesions localized to one hemisphere of the brain. Finally, in a lesion regrowth sample, we found that the regrown CCM lesion contained the same somatic mutation as the original lesion. Together, these data bolster the idea that all forms of CCM have a genetic underpinning of the two-hit mutation mechanism in the known CCM genes. Recent studies have found aberrant Rho kinase activation in inherited CCM pathogenesis, and we present evidence that this pathway is activated in sporadic CCM patients. These results suggest that all CCM patients, including those with the more common sporadic form, are potentially amenable to the same therapy.

  2. Vascular malformations of the mandible (intraosseous haemangiomas)

    Energy Technology Data Exchange (ETDEWEB)

    Guibert-Tranier, F.; Piton, J.; Caille, J.M.; Riche, M.C.; Merland, J.J.

    1982-11-01

    Haemangiomas of the mandible are relatively rare arteriovenous malformations: 85 cases have been described since 1849. The severe risk of bleeding during tooth extraction or biopsy led us to describe the radiological and angiographic features of this condition. The afferent vessles are the inferior dental, the lingual and the facial arteries. Preoperative embolization reduces the risk of bleeding at operation. Surgical treatment must assure the complete removal of the lesion. Radiotherapy and vascular ligatures are useless and dangerous.

  3. Brain Malformations

    Science.gov (United States)

    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, ...

  4. Application and value of MRI in the diagnosis of cerebral venous malformation.%脑静脉性血管畸形MRI应用及价值

    Institute of Scientific and Technical Information of China (English)

    王秀兰; 田为中; 李瑗; 陈锦华; 陈小云

    2011-01-01

    Objective To explore the application and evaluation of a variety of imaging sequences of magnetic resonance (MR) imaging in diagnosing cerebral venous malformation (CVM). Methods The distribution of 22 CVM cases and corresponding MR features were retrospectively analyzed. All the patients underwent conventional routine MRI sean and following examinations including 18 cases with extra enhanced TIWI, 8 cases with contrast enhanced magnetic resonance angiography (CE MRA), 3 eases with 3D time of flight magnetic resonance angiography (3D TOFMRA) and 6 cases with susceptibility-weighted imaging (SWI) were processed. Results he conventional routine MRI scans of draining veins were shown as long T1 and long T2 signal intensities except for few short T2 signal intensifies. Most oft. he conventional routine MRI scans of medullary veins were unclear and shown as long T1 and long T2 or iso T2 signal intensities, and after enhancement, several radial medullary veins were dearly shown afflux into bulky drain veins, demonstrating typical “jellyfish head” appearance or “centipede” and “roots shape” changes.The SWI was rather susceptible to CVM with low intensity on draining veins and medullary veins. Conclusion The characteristic representation of CVM can be achieved by combining a variety of imaging methods, which provided significant value in the diagnosis of CVM and assessment of corresponding eomplieatious.%目的 探讨MRI多种成像序列在脑静脉性畸形诊断中的应用及评价.方法 回顾性分析22例CVM分布及MR征象.常规平扫后,其中18例加做增强后TWI扫描,CE-MRA 8例,3D-TOFMRA 3例,SWI 6例.结果 MRI平扫显示引流静脉(或导静脉)通常为长T长T信号影,少数为短T信号;髓静脉为长T、长T或等T信号,大部分显示不佳,增强后可清晰显示多根髓静脉呈放射状汇入粗大的引流静脉,呈典型"水母头"状或"蜈蚣"、"树根状"改变.SWI显示CVM更为敏感,引流静脉和髓

  5. Gastrointestinal malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Loane, Maria; Dolk, Helen;

    2007-01-01

    of mortality for the prenatally diagnosed infants. Clinicians need to balance the risk of early delivery against the benefits of clinical convenience when making case management decisions after prenatal diagnosis. Very few studies have been able to show benefits of prenatal diagnosis of congenital...... malformations for liveborn infants. This may be because the benefits of prenatal diagnosis are outweighed by the problems arising from a lower GA at birth....

  6. Influence of Patient Age on Angioarchitecture of Brain Arteriovenous Malformations

    Science.gov (United States)

    Hetts, Steven W.; Cooke, Daniel L.; Nelson, Jeffrey; Gupta, Nalin; Fullerton, Heather; Amans, Matthew R.; Narvid, Jared A.; Moftakhar, Parham; McSwain, Hugh; Dowd, Christopher F.; Higashida, Randall T.; Halbach, Van V.; Lawton, Michael T.; Kim, Helen

    2014-01-01

    Background and Purpose To determine if clinical and angioarchitectural features of brain AVMs differ between children and adults. Materials and Methods A prospectively collected institutional database of all patients diagnosed with brain AVMs since 2001 was queried. Demographic, clinical, and angioarchitecture information was summarized and analyzed with univariable and multivariable models. Results Results often differed when age was treated as a continuous variable as opposed to dividing subjects into children (≤18 years; n=203) versus adults (>18 years; n=630). Children were more likely to present with AVM hemorrhage than adults (59% vs. 41%, p6 cm compared to 37.1 years for <3 cm), this was not significantly different between children and adults (p=0.069). Exclusively deep venous drainage was more common in younger subjects both when age was treated continuously (p=0.04), or dichotomized (p<0.001). Venous ectasia was more common with increasing age (mean, 39.4 years with ectasia compared to 31.1 years without ectasia) and when adults were compared to children (52% vs. 35%, p<0.001). Patients with feeding artery aneurysms presented at later average age (44.1 years) than those without such aneurysms (31.6 years); this observation persisted when comparing children to adults (13% vs. 29%, p<0.001). Conclusion Although children with brain AVMs were more likely to come to clinical attention due to hemorrhage than adults, venous ectasia and feeding artery aneurysms were underrepresented in children, suggesting that these particular high risk features take time to develop. PMID:24627452

  7. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  8. MRI与DSA影像融合联合电生理监测对脑动静脉畸形伴癫痫手术的价值%Value of MRI and 3D-DSA images fusion combined with intraoperative neuro-electrophysiological technique to surgery for intracranial arteriovenous malformation associated with epilepsy

    Institute of Scientific and Technical Information of China (English)

    孙荣辉; 徐国政; 杜浩; 宋健; 黄河; 赵曰圆; 马廉亭

    2015-01-01

    目的:探讨MRI与3D-DSA三维影像融合联合电生理监测在脑动静脉畸形伴癫痫显微手术中的应用价值。方法将1例脑动静脉畸形合并癫痫患者的MRI与3D-DSA影像数据输入神经导航进行影像融合,对病灶进行精准定位,联合术中电生理监测,完成脑动静脉畸形及致痫灶显微切除术。结果本例通过MRI与3D-DSA影像融合,精确定位显示病灶范围,术中实时导航找到主要供血动脉及引流静脉,成功切断主要供血动脉,完整切除畸形血管团,同时结合术中神经电生理技术定位脑功能区及致痫灶,术后患者无神经功能缺损,复查DSA示畸形无残留,脑电监测颅内未见异常放电。结论MRI与3D-DSA影像融合结合术中电生理监测联合应用,可以既能完全切除脑动静脉畸形病灶,又能同时清除致痫灶,保护脑重要功能区,为重要功能区脑动静脉畸形继发癫痫的治疗提供了一种安全有效的新方法。%Objective To investigate the value of MRI and 3D-DSA images fusion combined with intraoperative neuro-electrophysiological technique to the surgery for intracranial arteriovenous malformation (AVM) associated with epilepsy. Methods MRI and 3D-DSA images fusion was performed in 1 patient with epilepsy induced by AVM, in whom, AVM and epileptogenic zone were resected by neuronavigator-assisted surgery under eletroophysiological monitoring. Results The lesion was exactly located by MRI and 3D-DSA images fusion. The main arteries supplying blood to AVM and veins dainaging from AVM were found by the real-time navigation and then were ligated and cut off. AVM was totally resected. The epileptogenic zone and the functional cortex were accurately located by intraoperative eletrophysiological technique. The epileptogenic zone were successfully resected without neurological functional deficits. Conclusions MRI and 3D- DSA images fusion technology, which can clearly show

  9. Hereditary haemorrhagic telangiectasia: a population-based study of prevalence and mortality in Danish patients

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Vase, P; Green, A

    1999-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by telangiectatic lesions. The disease manifestations are variable and include epistaxis, gastrointestinal bleeding, pulmonary arteriovenous malformations and cerebral arteriovenous malformations. Early d...

  10. Transcranial Doppler blood flow measurement during cesarean section in two patients with cerebral vascular disease.

    Science.gov (United States)

    Smiley, R M; Ridley, D M; Hartmann, A; Ciliberto, C F; Baxi, L

    2002-07-01

    We present two cases of neurovascular disease in pregnancy in which transcranial Doppler was used to assess the status of the cerebral circulation during cesarean section under regional anesthesia. One woman had been found to have moyamoya disease, following a series of transient ischemic attacks during her first pregnancy, which ended in spontaneous abortion. On this occasion she was delivered by cesarean section under slowly-induced epidural anesthesia, using ephedrine to maintain the blood pressure, and transcranial Doppler revealed no change in signal in her left middle cerebral artery. Both mother and baby had an uneventful post natal course. The second case involved a primiparous woman with a large arteriovenous malformation that had been detected following generalized seizures, which were treated with valproic acid. Her cesarean section was conducted under spinal anesthesia, and her blood pressure maintained with ephedrine. Again transcranial Doppler revealed no change in signal in her middle cerebral artery during the procedure. We believe this is a potentially useful technique to monitor the cerebral circulation intraoperatively in the presence of cerebrovascular disease.

  11. 脑动静脉畸形治疗方法选择(附36例报告)%Study on choice of treatment of Cerebral Arteriovenous Malformation: a review of 36 cases

    Institute of Scientific and Technical Information of China (English)

    丁轩; 鲍洪; 吴开华; 招建华; 兰蒙; 骆太顺

    2005-01-01

    目的探讨各种类型脑动静脉畸形(AVM)治疗方法的选择为临床治疗提供依据.方法对36例脑AVM临床治疗进行回顾性分析,并结合文献复习.结果 36例脑AVM病人,手术切除9例和血管内栓塞后手术切除2例畸形血管团消失,γ-刀治疗4例,复查血管团消失或染色变淡,血管内栓塞10例和血管内栓塞后γ-刀治疗11例中栓塞>95%4例,>80%7例,>50%8例.复查GOS预后分级5级25例(69.4%);4级7例(19,4%),其中后遗头痛3例,癫痫4例;3级3例(8.3%)后遗神经功能缺失,主要为偏瘫、失语等;死亡1例(2.8%).结论根据脑AVM的Spetzler-Martin分级和临床类型选择合理的治疗方法可减少致残率和病死率.

  12. 儿童脑型肺吸虫病合并颅内动静脉畸形临床分析%Clinical analysis on cerebral paragonimiasis accompanied with intracranial arteriovenous malformations in children

    Institute of Scientific and Technical Information of China (English)

    吴宣萱; 梁平; 李映良; 李禄生; 周渝冬; 翟瑄

    2015-01-01

    目的 探讨儿童脑型肺吸虫病合并颅内动静脉畸形的临床表现、诊断及治疗方法.方法 回顾2011-2013年我科收治的6例脑型肺吸虫病合并颅内动静脉畸形患儿资料,对其病因、临床表现、辅助检查、诊断、治疗及愈后进行分析总结.结果 6例脑型肺吸虫病合并颅内动静脉畸形患儿,其中男性4例,女性2例,年龄5~8岁,平均6.4岁,均来自疫区,以颅内出血和感染为主要表现,肺吸虫抗体(+),增强CT或计算机断层扫描血管成像(CTA)提示脑内异常血管团,给予驱虫和手术切除治疗.切除病变组织病理检查提示异常血管,驱虫治疗后复查肺吸虫抗体(-),随访1~4年,颅内未再出血.结论 详细病史和增强CT扫描能确诊儿童脑型肺吸虫病合并颅内动静脉畸形,早治疗能有效降低颅内再出血风险和保护神经功能.

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

    Science.gov (United States)

    2017-01-01

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

  14. Anorectal malformations

    Directory of Open Access Journals (Sweden)

    Peña Alberto

    2007-07-01

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

  15. Cerebral hemorrhage associated with sildenafil (Revatio) in an infant.

    Science.gov (United States)

    Samada, Kazunori; Shiraishi, Hirohiko; Aoyagi, Jun; Momoi, Mariko Y

    2009-10-01

    A case of cerebral hemorrhage associated with sildenafil (Revatio) use in an infant is presented. Sildenafil is increasingly used in the treatment of primary and secondary pulmonary arterial hypertension and pulmonary arteriovenous fistula. In the reported case, sildenafil used to treat pulmonary arteriovenous fistula improved right-to-left shunting across the pulmonary fistula but resulted in cerebral hemorrhage. Cerebral hemorrhage, a previously reported complication of sildenafil, developed in an infant after a rapid increase in dose, to 4.7 mg/kg/day. Therefore, sildenafil doses must be increased only with care, and cerebral hemorrhage must be considered a potential complication.

  16. Delayed postoperative dural arteriovenous fistula presenting with progressive dementia.

    Science.gov (United States)

    Higashida, Tetsuhiro

    2015-12-09

    A 64-year-old woman underwent right suboccipital craniotomy to treat spontaneous cerebrospinal fluid (CSF) otorrhoea. Although the CSF leak was cured, the patient developed pulsatile tinnitus, ambulatory disturbance and progressive dementia. Four years after the surgery, she presented with generalised seizure. Cerebral angiography showed a dural arteriovenous fistula at the right transverse-sigmoid sinus, which was associated with sinus occlusion, retrograde blood flow in the sinus and diffuse intracranial cortical venous reflux. A combination of endovascular transarterial embolisation and transvenous embolisation with direct sinus puncture was performed. After the procedure, the patient's mental status and cognitive function improved significantly.

  17. Cerebral venous thrombosis: Update on clinical manifestations, diagnosis and management

    Directory of Open Access Journals (Sweden)

    Leys Didier

    2008-01-01

    Full Text Available Cerebral venous thrombosis (CVT has a wide spectrum of clinical manifestations that may mimic many other neurological disorders and lead to misdiagnoses. Headache is the most common symptom and may be associated with other symptoms or remain isolated. The other frequent manifestations are focal neurological deficits and diffuse encephalopathies with seizures. The key to the diagnosis is the imaging of the occluded vessel or of the intravascular thrombus, by a combination of magnetic resonance imaging (MRI and magnetic resonance venography (MRV. Causes and risk factors include medical, surgical and obstetrical causes of deep vein thrombosis, genetic and acquired prothrombotic disorders, cancer and hematological disorders, inflammatory systemic disorders, pregnancy and puerperium, infections and local causes such as tumors, arteriovenous malformations, trauma, central nervous system infections and local infections. The breakdown of causes differs in different parts of the world. A meta-analysis of the most recent prospectively collected series showed an overall 15% case-fatality or dependency rate. Heparin therapy is the standard therapy at the acute stage, followed by 3-6 months of oral anticoagulation. Patients with isolated intracranial hypertension may require a lumbar puncture to remove cerebrospinal fluid before starting heparin when they develop a papilloedema that may threaten the visual acuity or decompressive hemicraniectomy. Patients who develop seizures should receive antiepileptic drugs. Cerebral venous thrombosis - even pregnancy-related - should not contraindicate future pregnancies. The efficacy and safety of local thrombolysis and decompressive hemicraniectomy should be tested

  18. SDF-1α及其受体CXCR4与HIF-1α、VEGF在脑动静脉畸形中的表达%Expressions of stromal-cell derived factor-1α and its receptor CXCR4, hypoxia inducible factor-1α and vascular endothelial growth factor in brain arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    王凌雁; 郭少雷; 齐铁伟; 梁丰; 黄正松

    2014-01-01

    Objective To investigate the expressions ofstromal-cell derived factor-1α (SDF-1α)and its receptor CXCR4 in brain arteriovenous malformation (AVM) and to explore the relationships of SDF-1α with hypoxia inducible factor-1α (HIF-1α) and vascular endothelial growth factor (VEGF).Methods Surgical specimens from 48 patients accepted brain AVM resection,collected in our hospital from January 2012 to December 2013,were studied for expressions ofSDF-1α,CXCR4,VEGF and HIF-1α by immunohistochemical staining.The relationships of SDF-1α with VEGF and HIF-1α were analyzed and influences of embolism,hemorrhage and Spetzer-Martin classification in SDF-1α expression were assessed.Results SDF-1α and CXCR4 expressed in 100% and 83.3% AVM specimens,respectively.The positive staining for SDF-1α was observed in the cytoplasm of vascular endothelium within the nidus and smooth muscle cells of vascular wall.CXCR4 expressed in vascular endothelium and perivascular cells located in the space between the abnormal vessels.SDF-1α expression was significantly associated with VEGF and HIF-1α (r=0.537 and 0.437,respectively,P<0.05).SDF-1α showed more intense expression in embolized patients than that in non-embolized patients (P< 0.05),while no significant difference was noted between patients with and without hemorrhage and between patients of different Spetzer-Martin classifications (P>0.05).Conclusion SDF-1α and its receptor CXCR4 highly express in brain AVM; preoperative embolization might induce expression of SDF-1α.%目的 观察脑动静脉畸形(AVM)病灶内间质细胞衍生因子-1α(SDF-1α)及其受体CXCR4的表达情况,以及SDF-1α与低氧诱导因子-1α(HIF-1α)和血管内皮生长因子(VEGF)表达的关系. 方法 选择中山大学附属第一医院神经外科自2012年1月至2013年12月经手术切除并经病理组织学证实的脑AVM标本共48例,应用免疫组化染色方法检测SDF-1α、CXCR4、HIF-1α和VEGF的表达情况,

  19. Malformación arteriovenosa pulmonar: Características clínicas, diagnóstico y rol del tratamiento quirúrgico en pacientes tratados con cirugía resectiva pulmonar Pulmonary arteriovenous malformation: Clinical features, diagnosis and role of surgical management in patients with lung resection surgery

    Directory of Open Access Journals (Sweden)

    ROBERTO GONZÁLEZ L

    2011-03-01

    Full Text Available Introducción: Las malformaciones arteriovenosas pulmonares (MAVP son infrecuentes y la cirugía tiene un rol en casos seleccionados. Objetivos: Describir las características clínicas, métodos diagnósticos y rol del tratamiento quirúrgico en pacientes tratados con cirugía por MAVP. Materialy Método: Revisión retrospectiva, incluyendo todos los pacientes con diagnóstico de MAVP en quienes se realizó cirugía en el Instituto Nacional del Tórax, desde febrero de 2005 a febrero de 2010. El seguimiento fue por control médico o contacto telefónico. Resultados: Se analizó 8 pacientes, seis mujeres (relación 3:1, edad entre 16 y 68 años. Los síntomas y signos más frecuentes fueron: disnea, cianosis y acropaquia. La localización más frecuente fue el lóbulo inferior derecho. Cuatro pacientes tenían MAVP múltiples y cuatro cumplían con criterios de Enfermedad de Rendu-Osler-Weber Seis tenían policitemia y dos anemia. La radiografía fue anormal en todos los casos y la tomogrqfía computada definió la anatomía en siete. Angiogrqfia se realizó en tres casos. Dos tenían ecocardiograma con contraste y cuatro cintigrama. La cirugía más frecuente fue la lobectomía. La indicación de cirugía fue: tamaño de la MAVP en cinco, falla de embolización en dos y hallazgo intra operatorio en uno, intervenido por otra razón. Uno presentó hemorragia post-operatoria. La estadía post-operatoria fue entre 2 y 10 días. No hubo mortalidad. En el último control todos los pacientes estaban asintomáticos. Conclusión: Las MAVPpueden presentarse en un amplio espectro clínico y anatómico. Pueden generar síntomas y complicaciones graves, por esto se recomienda tratarlas. El estudio pre-operatorio se basa en demostrar el shunt y en determinar las características anatómicas de la lesión. En algunos casos seleccionados está indicada la cirugía resectiva pulmonar.Background: Pulmonary arteriovenous malformations (PA VM are rare and surgery

  20. Differential Notch Signalling Pathway Gene Expression of Nidus and Adjacent Brain Tissure Parts in Human Brain Arteriovenous Malformations%Notch信号通路基因在人脑动静脉畸形和周围脑组织中的差异表达

    Institute of Scientific and Technical Information of China (English)

    郑名哲; 陈衔城; 汤海亮; 谢清; 宫晔

    2012-01-01

    Aim: To investigate the expression of genes involved in Notch signalling pathway of different parts in human brain arteriovenous malformations (BAVM) by using Notch signalling pathway microarray. Methods: Five BAVM cases with stroke history were collected, whose sample of nidus (N) and adjacent brain tissure (B) were obtained in surgery. Signal intensity of the genes was examed and calculated by using Oligo GE Array Human Notch Signaling Pathway Microarray OHS-059. The genes with N/B ratio larger than 1.5 or smaller than 0.67 were counted as markablly changed, two of which were verified by realtime PCR. Results: Twenty seven of 113 genes involved in Notch signalling pathway were found differentially expressed. Four genes were up-regulated, and twenty three genes were down-regulated. Most important genes include Notch signalling pathway ligands DLL1/DLL3, key genes of cleavage ADAM10/ADAM17, Notch signalling pathway target gene HESS, Notch related Sonic Hedgehog pathway gene GLI1, Wnt signalling pathway gene FZD1, oncogene LM02. The data from two genes DLL1 and HESS by realtime PCR is basically consisted with the data from the gene chip. Some important genes involved in Notch signalling pathway did not show differential expression at the two parts of BAVM. Conclusion: Many of the genes involved in Notch signalling pathway were differential expressed at nidus and adjacent brain tissue parts in BAVM, which indicated BAVM occurrence and developing have something to do with Notch signalling pathway. Notch signallling pathway gene expression pattern might be different at adjacent brain tissure. Differential expressed genes can contribute new ways to BAVM research.%目的:应用基因芯片研究人脑动静脉畸形(BAVM)的畸形团及周围脑组织的Notch信号通路相关基因的差异表达.方法:收集有出血史的5例BAVM标本,在畸形团、周围脑组织分别取材,进行Notch信号通路基因芯片杂交,得到基因芯片上各基因点数值型信

  1. Germline Mutations in RASA1 Are Not Found in Patients with Klippel-Trenaunay Syndrome or Capillary Malformation with Limb Overgrowth.

    Science.gov (United States)

    Revencu, N; Boon, L M; Dompmartin, A; Rieu, P; Busch, W L; Dubois, J; Forzano, F; van Hagen, J M; Halbach, S; Kuechler, A; Lachmeijer, A M A; Lähde, J; Russell, L; Simola, K O J; Mulliken, J B; Vikkula, M

    2013-04-01

    The RASA1 gene encodes p120RASGAP, a multidomain cytoplasmic protein that acts as a negative regulator of the RAS signalling pathway. Heterozygous loss-of-function RASA1 mutations were identified in patients with Parkes Weber syndrome and multifocal capillary malformations. This syndrome is characterised by a capillary blush on an extremity, arteriovenous microfistulas, and bony and soft tissue hypertrophy. The aim of this study was to test RASA1 in 2 disorders characterised by asymmetric limb enlargement and vascular malformations, namely Klippel-Trenaunay syndrome and regional capillary malformation with overgrowth. We did not identify any clear pathogenic change in these patients. Thus, besides clinical and radiological criteria, RASA1 testing constitutes an additional tool to differentiate Parkes Weber syndrome of capillary malformation-arteriovenous malformation (CM-AVM) from overlapping disorders.

  2. Arteriovenous Patterns in Beaked Whales

    Science.gov (United States)

    2015-09-30

    1 DISTRIBUTION STATEMENT A. Approved for public release; distribution is unlimited. Arteriovenous patterns in beaked whales Alexander M...from which future morphological (e.g. acoustic pathways), pathophysiological (e.g. decompression sickness and embolus formation) and mathematical...extramandibular fat body (EMFB), intramandibular fat body (IMFB) and pterygoid venous lake. 2) To describe the vascular morphology of the pulmonary system

  3. What the Neurosurgeon needs to know about Cerebral Developmental Venous anomalies

    Directory of Open Access Journals (Sweden)

    Alvis-Miranda Hernando Raphael

    2014-06-01

    Full Text Available Venous Angiomas or Developmental venous anomalies (DVA are extreme variations of normal transmedullary veins that are necessary for the drainage of white and gray matter, also are one type of cerebrovascular malformation (CVM, sharing category with capillary telangiectesias, cavernous malformations (CM, and arteriovenous malformations (AVM, each of which may also be associated with a DVA. DVA are the most commonly encountered CVM, accounting for up to 60% of all CVM. We present a review of the literatura

  4. Digital subtraction angiography of the cerebral vessels by intraarterial injection

    Energy Technology Data Exchange (ETDEWEB)

    Nagata, Izumi; Kikuchi, Haruhiko; Karasawa, Jun; Mitsugi, Toru; Naruo, Yoshito; Takamiya, Makoto (National Cardiovascular Center, Suita, Osaka (Japan))

    1984-10-01

    Three hundred and fifty-seven digital subtraction angiography (DSA) were performed in 184 neurosurgical patients by intraarterial injection. Examinations consisted of 192 carotid angiography, 110 vertebral angiography, 23 aortography, 11 spinal angiography and 21 other angiography. In all examinations, visualization of the vessels was excellent and the complications were never experienced. High contrast sensitivity of DSA resulted in better visualization of tumor stains, phlebogram, and arteries in cerebral arteriovenous malformations with large shunt blood flow than conventional angiography. Selective catheterization into each cerebral arteries was not necessarily demanded for good opacification of the vessels because of high sensitivity. High contrast sensitivity also permitted low concentration of contrast material, small dose of contrast material, and slow injection rate. Low concentration of contrast material reduced pain and heat during injection especially in the external carotid and vertebral angiography. Using slow injection, recoiling of catheter into the aorta was reduced, so that injection from the innominate and subclavian arteries for visualization of origin of the cerebral arteries were always successful. Full study of cerebral arteries by Seldinger's method, if necessary, was easily achieved using DSA even in patient with high age or with severe atherosclerosis. Bolus injection of small dose of contrast material as well as serial imaging was helpful in evaluating hemodynamics in the lesion. Real time display of DSA reduced the time required for angiography and was very convenient for artificial embolization. Besides these advantages, DSA became comparable to conventional angiography in spacial resolution by use of intraarterial injection and could be a preoperative genuine examination as well as a screening method.

  5. 3D磁敏感加权成像在脑静脉畸形诊断中的价值%The role of 3D susceptibilitv weighted imaging in the evaluation of cerebral venous malformation

    Institute of Scientific and Technical Information of China (English)

    祁鸣; 王德杭

    2011-01-01

    Objective To discuss the value of 3D susceptibility weighted imaging(SWI) in the diagnosis of cerebral venous malformation (CVM). Methods Thirty CVM patients were examined with a 3. OT MRI scanner. The sequences included the conventional MRI, 3D SWI and contrast enhanced T1WL Comparisons were made among SWI, contrast enhanced T1W1 and conventional sequences. The image quality of CVM was graded. Results SWI could demonstrate conspicuously both numerous deep medullary veins and collector veins as the contrast enhanced MRI did, which were hardly visible on Tl-weighted images or T2-weighted images. Conclusion SWI is a sensitive sequence for detecting CVMs. It allows better visualization of CVMs without contrast agents.%目的 探讨3D磁敏感加权成像(SWI)技术对脑静脉畸形(CVM)的诊断价值.方法 30例CVM形患者采用3.0T磁共振常规MRI序列(T1WI、T2WI)、3D SWI及T1WI增强扫描,根据CVM血管显影情况对常规MRI序列、3D SWI及T1WI增强扫描进行分级比较.结果 SWI能显著显示常规T1WI、T2WI很难显示的髓静脉及引流静脉,并与增强扫描图像有很好的对应.结论 SWI是检出CVM较为敏感的序列,在不用造影剂情况下使静脉畸形血管获得较好的显示.

  6. Device and methods for "gold standard" registration of clinical 3D and 2D cerebral angiograms

    Science.gov (United States)

    Madan, Hennadii; Likar, Boštjan; Pernuš, Franjo; Å piclin, Žiga

    2015-03-01

    Translation of any novel and existing 3D-2D image registration methods into clinical image-guidance systems is limited due to lack of their objective validation on clinical image datasets. The main reason is that, besides the calibration of the 2D imaging system, a reference or "gold standard" registration is very difficult to obtain on clinical image datasets. In the context of cerebral endovascular image-guided interventions (EIGIs), we present a calibration device in the form of a headband with integrated fiducial markers and, secondly, propose an automated pipeline comprising 3D and 2D image processing, analysis and annotation steps, the result of which is a retrospective calibration of the 2D imaging system and an optimal, i.e., "gold standard" registration of 3D and 2D images. The device and methods were used to create the "gold standard" on 15 datasets of 3D and 2D cerebral angiograms, whereas each dataset was acquired on a patient undergoing EIGI for either aneurysm coiling or embolization of arteriovenous malformation. The use of the device integrated seamlessly in the clinical workflow of EIGI. While the automated pipeline eliminated all manual input or interactive image processing, analysis or annotation. In this way, the time to obtain the "gold standard" was reduced from 30 to less than one minute and the "gold standard" of 3D-2D registration on all 15 datasets of cerebral angiograms was obtained with a sub-0.1 mm accuracy.

  7. Estimation of blood volume difference between bilateral cerebral hemispheres by means of subtraction method

    Energy Technology Data Exchange (ETDEWEB)

    Ueno, Ichiro (Tokyo Women' s Medical Coll. (Japan))

    1983-05-01

    It would be admitted that by measuring radioactivity of the head after intravenous injection of RI some information could be afforded concerning the cerebral vascular bed. After intravenous injection of sup(99m)Tc-pertechnetate (15mCi), an anterior view scintiscanning was made using a gamma camera combined with a computer system. Two ROIs of about 30 cm/sup 2/ were set bilateral symmetrically and a count rate curve of each ROI was obtained. The brain transit time (BTT) was calculated from first derivative of the initial count rate curve. As an index devoting difference between vascular bed of each hemisphere, the vascular bed difference index (VBDI) was introduced BTT and VBDI were calculated in 104 subjects including 11 normal controls. In most of brain tumor, cerebral hemorrhage, severe head injury and cerebral aneurysm BTTs were prolonged in affected hemispheres. However, in a half of cerebral infarction cases BTTs were rather shortened on the affected side. In normal controls, the absolute value of VBDI was (0.16 +- 0.06(S.D.)). In cases of brain tumor, cerebral hemorrhage and severe head injury, VBDI showed a significant deviation from the normal range, taking plus value. This results suggest, according to the definition of VBDI, vascular beds of the affected hemisphere are abnormally decreased compared with those of non-affected side. In cases of arteriovenous malformation VBDIs were increased on the affected side, reflecting enlarged tangle of arteriols and venous vessels. On the other hand, in cases of cerebral infarction, VBDI varied from case to case, making it difficult to get some conclusion from the present data. VBDI, however, invariably decreased in cases in which the occlusion of arteries were definitely confirmed by angiography.

  8. Spinal dural arteriovenous fistula: Imaging features and its mimics

    Energy Technology Data Exchange (ETDEWEB)

    Jeog, Ying; Ting, David Yen; Hsu, Hui Ling; Huang, Yen Lin; Chen, Chi Jen; Tseng, Ting Chi [Dept. of Radiology, aipei Medical University-Shuang Ho Hospital, New Taipei City, Taiwan (China)

    2015-10-15

    Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation, however it is still rare and underdiagnosed. Magnetic resonance imaging findings such as spinal cord edema and dilated and tortuous perimedullary veins play a pivotal role in the confirmation of the diagnosis. However, spinal angiography remains the gold standard in the diagnosis of SDAVF. Classic angiographic findings of SDAVF are early filling of radicular veins, delayed venous return, and an extensive network of dilated perimedullary venous plexus. A series of angiograms of SDAVF at different locations along the spinal column, and mimics of serpentine perimedullary venous plexus on MR images, are demonstrated. Thorough knowledge of SDAVF aids correct diagnosis and prevents irreversible complications.

  9. Spinal dural arteriovenous fistulas. Diagnostics and therapy; Spinale durale arteriovenoese Fisteln. Diagnostik und Therapie

    Energy Technology Data Exchange (ETDEWEB)

    Reith, W.; Kettner, M.; Simgen, A.; Yilmaz, U. [Universitaetsklinikum des Saarlandes, Klinik fuer Diagnostische und Interventionelle Neuroradiologie, Homburg/Saar (Germany)

    2012-05-15

    Spinal dural arteriovenous fistulas are rare spinal vascular malformations which can cause progressive paraparesis and paraplegia if not treated. As symptoms are unspecific diagnosis is often delayed and clinical outcome is dependent on early therapy. While magnetic resonance imaging (MRI) is the first choice imaging procedure, selective spinal digital subtraction angiography is necessary to analyze the angioarchitecture and to plan the treatment. This article provides an overview on the epidemiology, etiology, clinical aspects and imaging features as well as therapeutic aspects of spinal dural arteriovenous fistulas. Knowledge of spinal vascular anatomy is the basis for understanding spinal dural arteriovenous fistulas. (orig.) [German] Spinale durale arteriovenoese Fisteln (dAVF) sind seltene spinale vaskulaere Malformationen, die unbehandelt zu einer progredienten Paraparese und Paraplegie fuehren koennen. Da die klinischen Symptome oft unspezifisch sind, werden sie haeufig erst in einem spaeteren Stadium diagnostiziert. Die Erkrankungshaeufigkeit ist mit 5-10 Neuerkrankungen/1 Mio. Einwohner/Jahr relativ selten, ueber 80% der Betroffenen sind Maenner. Der unbehandelt schlechte klinische Verlauf der dAVF sowie die Moeglichkeit der Therapie, deren Erfolg von einer fruehzeitigen Behandlung abhaengt, macht sie jedoch zu einer wichtigen Erkrankung. Die Diagnose ist haeufig im MRT zu stellen, zur genauen Darstellung der Fistel ist eine selektive spinale Subtraktionsangiographie jedoch notwendig. Ziel dieses Artikels ist, einen Ueberblick ueber die Epidemiologie, Aetiologie, Klinik und bildgebende Verfahren sowie therapeutischen Moeglichkeiten dieser spinalen vaskulaeren Malformation zu geben. Voraussetzung zum grundlegenden Verstaendnis der duralen AVF sind genaue Kenntnisse der vaskulaeren spinalen Gefaessversorgung. (orig.)

  10. [Childhood transverse sinus dural arteriovenous fistula treated with endovascular and direct surgery: a case report].

    Science.gov (United States)

    Niizuma, Kuniyasu; Sakata, Hiroyuki; Koyama, Shinya; Kon, Hiroyuki; Chonan, Masashi; Sasaki, Tatsuya; Nishijima, Michiharu; Ezura, Masayuki; Tominaga, Teiji

    2012-11-01

    Infantile dural arteriovenous fistula is a rare cerebrovascular malformation carrying a poor prognosis with an anatomic cure of only 9%. Endovascular embolization is mainly selected to treat this entity, aiming to obtain normal development of the patients. We present a case of a 20-month-old girl with epilepsy. Digital subtraction angiography revealed a dural arteriovenous fistula involving the right transverse sinus. The arteriovenous fistula was fed by multiple dural branches from the middle meningeal, occipital, meningohypophyseal, and anteroinferior cerebellar arteries. The right transverse sinus was transvenously embolized with platinum coils. Although the shunt flow remained, the patient was liberated from epilepsy. Nine months later, the patient suffered from a recurrence of epilepsy. Digital subtraction angiography demonstrated some increase in shunt flow. Right middle meningeal, occipital, posterior deep temporal, and tentorial arteries were transarterially embolized using N-butyl cyanoacrylate, followed by complete surgical resection of the right transverse sinus. The shunt flow disappeared after surgery, and her epilepsy improved significantly. Our experience suggests that the combination of endovascular and surgical treatment is effective for recurrent infantile dural arteriovenous fistula.

  11. Hydrocephalus in a patient with an unruptured pial arteriovenous fistula: hydrodynamic considerations, endovascular treatment, and clinical course.

    Science.gov (United States)

    Morales-Gómez, Jesús A; Garza-Oyervides, Vicente V; Arenas-Ruiz, José A; Mercado-Flores, Mariana; Elizondo-Riojas, C Guillermo; Boop, Frederick A; de León, Ángel Martínez-Ponce

    2017-03-01

    Intracranial pial arteriovenous fistulas, also known as nongalenic fistulas, are rare vascular malformations affecting predominantly the pediatric population. Hydrocephalus is an unusual presentation in which the exact pathophysiology is not fully understood. The aim of treatment in these cases is occlusion of the fistula prior to considering ventricular shunting. Here, the authors describe the hydrodynamic considerations of the paravascular pathway and the resolution of hydrocephalus with endovascular treatment of the fistula.

  12. Microscope-assisted endoscopic interlaminar ligation of spinal arteriovenous fistulas: technical note.

    Science.gov (United States)

    Wang, Chen; Chen, Chien-Min; Shen, Fang; Fang, Xiao-Dong; Ying, Guang-Yu; Ren, Yu-Cheng; Yu, Dan-Feng; Zhu, Liang-Liang; Zhu, Yong-Jian; Zhang, Jian-Min

    2016-09-01

    Spinal dural arteriovenous fistulas (SDAVFs) are the most common type of spinal arteriovenous malformations, and microsurgical ligation is the treatment modality most frequently used for these lesions. Developments in endoscopic techniques have made endoscopy an even less invasive alternative to routine microsurgical approaches in spine surgery, but endoscopic management of SDAVF or other intradural spinal lesions has not been reported to date. The authors describe the use of a microscope-assisted endoscopic interlaminar approach for the ligation of the proximal draining vein of an L-1 SDAVF in a 58-year-old man. A complete cure was confirmed by postoperative angiography. The postoperative course was uneventful, and short-term follow-up showed improvements in the patient's neurological function. The authors conclude that the endoscopic interlaminar approach with microscope assistance is a safe, minimally invasive, innovative technique for the surgical management of SDAVFs in selected patients.

  13. A rare cause of progressive paraparesis and urinary retention: Spinal dural arteriovenous fistula- Case report

    Directory of Open Access Journals (Sweden)

    Sırma Geyik

    2016-12-01

    Full Text Available Spinal arteriovenous fistulas (AVF, are rarely seen clinical pathology, have serious morbidity in cases without treatment although spinal AVF are the most common types of spinal arteriovenous malformation. Fifty years old male patient suffered from urine retention and paraparesis after lifted a heavy object. Spinal magnetic resonance images (MRI showed diffuse hyper intense lesion from midthoracic spinal cord segment to conus medullaris in T2A sequance. Spinal angiography revealed a long segmental dorsal AVF on the right side of T 7-8 level in spinal cord. Because of the low flow and a small AVF neurosurgical Department decided to perform an operation for spinal AVF. We should keep in mind spinal AVF, in which prognosis is well after prompt and appropriate theraphy, as a differential diagnosis in patients presented with progressive spinal symptoms.

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

    Science.gov (United States)

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

    2016-05-01

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

  15. Dural arteriovenous fistula of the lesser sphenoid wing region treated with Onyx: technical note

    Energy Technology Data Exchange (ETDEWEB)

    Rezende, Marco Tulio Salles; Piotin, Michel; Mounayer, Charbel; Spelle, Laurent; Abud, Daniel Giansante [Fondation Rothschild, Department of Interventional Neuroradiology, Paris (France); Moret, Jacques [Fondation Rothschild, Department of Interventional Neuroradiology, Paris (France); Hopital de la Fondation Rothschild, Service de Neuroradiologie Interventionnelle, Paris, CEDEX 19 (France)

    2006-02-15

    A 45-year-old man presented with acute right orbital pain and right-sided headache. Magnetic resonance imaging (MRI) and cerebral angiography revealed a dural arteriovenous fistula (DAVF) of the lesser sphenoid wing region. The lesion was endovascularly treated by transarterial embolization with Onyx (ethyl vinyl alcohol; Micro Therapeutics, Irvine, Calif.). We review some anatomical and therapeutic features involving DAVFs of this region and describe the feasibility of the use of Onyx in the treatment of these lesions. (orig.)

  16. UNILATERAL RETINAL VASCULAR MALFORMATION IN HEREDITARY HEMORRHAGIC TELANGIECTASIA

    Directory of Open Access Journals (Sweden)

    Ananth Bhandary

    2014-07-01

    Full Text Available Hereditary hemorrhagic telangeiectasia (HHT is an autosomal dominant genetic disorder that leads to vascular malformations. It was first recognized in the 19th century as a familial disorder with abnormal vascular structures causing bleeding from the nose and gastrointestinal tract. HHT is characterized by telangiectatic lesions of the nose, lips, lungs, brain, and spinal cord. The reported incidence in Europe and Japan is between 1:5000 and 1:8000; but is widely variable in other regions. It is seen more frequently in whites. Ocular involvement has been reported in patients with HHT. Although conjunctival telangiectasia is the most common manifestation, rarely intraocular vascular lesions such as retinal telangiectasia and arteriovenous malformations in the retina, are seen. We describe a patient with HHT who had an abnormal unilateral retinal vascular abnormality along with tortuous conjunctival vasculature in the other eye, which has not been reported till date

  17. Management of extensive intraparotid vascular malformation: a case report

    Directory of Open Access Journals (Sweden)

    Katerina Anesti

    2014-06-01

    Full Text Available Treatment of large soft tissue vascular lesions remains one of the greatest challenges in modern plastic surgery. The extent of the disease and the involved structures, but also the expectations of the patients are important in determining the way of treatment. The effective management of hemangiomas and vascular malformations of the head and neck requires a team approach, in order to understand the biologic behavior of the lesion, complete the diagnostic studies necessary to define the area of involvement, and understand the benefits and limitations of interventional radiologic and surgical procedures. The synthesis of this knowledge can help determine the best treatment. The strategic plan and subsequent management of a 34-year-old Maori man with an extensive arteriovenous intraparotid malformation is presented.

  18. Paroxysmal supraventricular tachycardia as first manifestation of right atrial hemangioma during endovascular treatment of intracranial arteriovenous fistulas.

    Science.gov (United States)

    Spanò, Francesca; Cereda, Alberto; Moreo, Antonella; Bonacina, Edgardo; Peritore, Angelica; Roghi, Alberto; Giannattasio, Cristina; Pedrotti, Patrizia

    2015-06-10

    We report the description of a cardiac mass occupying almost the entire right atrium in a young man who developed paroxysmal supraventricular tachycardia during endovascular treatment of intracranial arteriovenous fistulas. The mass was detected at echocardiographic examination, its tissue characteristics were defined with cardiac magnetic resonance and it was successfully surgically removed. The histopathological findings were consistent with a mixed type cavernous-capillary hemangioma of the heart. The intriguing co-existence of cardiac hemangioma and cerebral arteriovenous fistulas, to the best of our knowledge, has not been previously reported in English Literature.

  19. Split Cord Malformations

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    Yurdal Gezercan

    2015-06-01

    Full Text Available Split cord malformations are rare form of occult spinal dysraphism in children. Split cord malformations are characterized by septum that cleaves the spinal canal in sagittal plane within the single or duplicated thecal sac. Although their precise incidence is unknown, split cord malformations are exceedingly rare and represent %3.8-5 of all congenital spinal anomalies. Characteristic neurological, urological, orthopedic clinical manifestations are variable and asymptomatic course is possible. Earlier diagnosis and surgical intervention for split cord malformations is associated with better long-term fuctional outcome. For this reason, diagnostic imaging is indicated for children with associated cutaneous and orthopedic signs. Additional congenital anomalies usually to accompany the split cord malformations. Earlier diagnosis, meticuolus surgical therapy and interdisciplinary careful evaluation and follow-up should be made for good prognosis. [Cukurova Med J 2015; 40(2.000: 199-207

  20. Genetics Home Reference: cerebral cavernous malformation

    Science.gov (United States)

    ... Ibrahim R, Irthum B, Jacquet G, Lonjon M, Moreau JJ, Neau JP, Parker F, Tremoulet M, Tournier- ... Lapierre F, Loiseau H, Menei P, Mercier P, Moreau JJ, Nivelon-Chevallier A, Parker F, Redondo AM, ...

  1. Intracranial pial arteriovenous fistula in infancy: a case report and literature review.

    Science.gov (United States)

    Kraneburg, U M; Nga, V D W; Ting, E Y S; Hui, F K H; Lwin, S; Teo, C; Chou, N; Yeo, T T

    2014-02-01

    Intracranial pial arteriovenous fistulas (AVF) are rare vascular malformation especially in the first 2 years of life. The pathology in this age group is associated with greater morbidity and mortality. We report a rare case of 36-day-old male infant with a pial AVF associated with an arterial aneurysm, who presented with intraventricular hemorrhage and hydrocephalus. In addition, an online review of the literatures on pediatric pial AVF was performed using PubMed on published case reports and articles from 1980 to April 2013.

  2. Paradoxical embolism following thromboaspiration of an arteriovenous fistula thrombosis: a case report

    Directory of Open Access Journals (Sweden)

    Amara Brahim

    2010-10-01

    Full Text Available Abstract Introduction Paradoxical embolism is an increasingly reported cause of arterial embolism. Several embolic sources have been described, but thrombosis of an arteriovenous fistula as a paradoxical emboligenic source has not, to the best of our knowledge, been reported. Case presentation A 50-year-old Caucasian woman received a renal graft for primary hyperoxaluria. After transplantation, she was maintained on daily hemodialysis. Thrombosis of her arteriovenous fistula occurred two weeks post-transplantation and was treated by thromboaspiration, which was partially successful. During a hemodialysis session immediately following thromboaspiration, she developed a coma with tetraplegia requiring intensive cardiorespiratory resuscitation. Brain magnetic resonance imaging revealed various hyperdense areas in the vertebrobasilar territory resulting from bilateral occlusion of posterior cerebral arteries. Transesophageal echocardiographic examination showed a patent foramen ovale, while pulse echography of the arteriovenous fistula revealed the persistence of extensive clots that were probably the embolic source. A paradoxical embolus through a patent foramen ovale was suggested because of the proximity of the neurological event to the thrombectomy procedure. Conclusions The risk of paradoxical embolism in a hemodialyzed patient with a patent foramen ovale deserves consideration and requires careful evaluation in situations of arteriovenous fistula thrombosis.

  3. Dural arteriovenous fistulae after forehead knife-cut injury

    Institute of Scientific and Technical Information of China (English)

    朱刚; 陈志; 冯华

    2004-01-01

    @@ Dural arteriovenous fistulae (DAVF) is a rare intracranial vascular disease. It is pathologically characterized by direct shunting of the intracranial artery and vein, which results in cerebral ischemia, intracranial hemorrhage, neural deficit and intracranial murmur. The etiological mechanism of DAVF is not well known, but most researchers think it is associated with congenital abnormal development, especially abnormal development of dural blood vessels at the stage of embryogenesis. Recently, some researchers have found that DAVF is also associated with some acquired factors. This article reports a case who developed DAVF within 2 years after debridement of frontal bone fragmentation, depressed fracture, left frontal lobe contusion and superior sagittal sinus injury due to forehead knife-cut injury. The pathogenic mechanism was explored through a review of the related literatures.

  4. Congenital Vascular Malformation

    Science.gov (United States)

    ... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel– ... 3) non-profit organization focused on providing public education and improving awareness about vascular diseases. For more ...

  5. Observation and nursing of perioperative cerebral angiography%脑血管造影围术期的观察及护理

    Institute of Scientific and Technical Information of China (English)

    李存珍; 张慧; 朱明艳; 吴建红

    2013-01-01

    目的:总结脑血管造影患者围术期的护理。方法:在阅读相关文件的同时,回顾分析总结45例脑血管造影围术期的全程护理体会。结果:检出动脉瘤24例,动静脉畸形3例,动脉闭塞2例,颅内动静脉瘘4例,脊髓动静脉瘘1例,烟雾病2例,未见异常9例,无神经系统并发症,阳性率77%,阳性率高,有利于准确诊断疾病。结论:脑血管造影是一种有创性检查,具有较高的操作风险。围术期严密观察、精心护理,术中密切配合医生,是脑血管造影术成功的重要保证。%Objective To summarize the perioperative nursing care of patients with cerebral angiography .At the same time in reading re-lated documents ,retrospectively analyzed and summarized the experience of 45 cases of nursing in perioperative period of cerebral angiogra-phy .Results there were 24 cases of aneurysm ,arteriovenous malformation in 3 cases ,arterial occlusion in 2 cases ,intracranial arteriovenous fistula in 4 cases ,spinal cord arteriovenous fistula in 1 cases ,2 cases of moyamoya disease ,9 cases were normal ,no nervous system complica-tion ,the positive rate was 77% ,the positive rate is high ,is conducive to the accurate diagnosis of disease .Conclusion cerebral angiography is an invasiveoperation ,with higher risk .Wai close observation ,perioperative care ,close cooperation during operation doctor ,is an important guarantee for the success ofthe cerebral angiography .

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

    Energy Technology Data Exchange (ETDEWEB)

    Hammer, Simone; Fellner, Claudia; Wohlgemuth, Walter A. [University Hospital Regensburg, Department of Radiology, Regensburg (Germany); Uller, Wibke [Boston Children' s Hospital and Harvard Medical School, Division of Vascular and Interventional Radiology, Boston, MA (United States); University Hospital Regensburg, Department of Radiology, Regensburg (Germany); Manger, Florentine [University Hospital Wuerzburg, Department of Nuclear Medicine, Wuerzburg (Germany); Zeman, Florian [University Hospital Regensburg, Center for Clinical Trials, Regensburg (Germany)

    2017-01-15

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

  7. Splenic arteriovenous fistula treated with percutaneous transarterial embolization

    DEFF Research Database (Denmark)

    Madsen, M.A.; Frevert, S.; Madsen, P.L.;

    2008-01-01

    Splenic arteriovenous fistula is a rare complication following splenectomy. We report a case of a large splenic arteriovenous fistula 23 years after splenectomy in a 50-year old male with abdominal pain, gastro-intestinal bleeding, ascites, diarrhoea, dyspnoea, portal hypertension and heart failure....... The arteriovenous fistula was successfully treated with percutaneous transarterial embolization and the patient gained almost complete recovery. This case demonstrates the usefulness of embolization of an otherwise surgical demanding arteriovenous fistula Udgivelsesdato: 2008/11...

  8. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  9. Stent graft placement for dysfunctional arteriovenous grafts

    Energy Technology Data Exchange (ETDEWEB)

    Jeon, Gyeong Sik [Dept. of Radiology, CHA Bundang Medical Center, College of Medicine, CHA University, Seongnam (Korea, Republic of); Shin, Byung Seok; Ohm, Joon Young; Ahn, Moon Sang [Chungnam National University Hospital, Daejeon (Korea, Republic of)

    2015-07-15

    This study aimed to evaluate the usefulness and outcomes of stent graft use in dysfunctional arteriovenous grafts. Eleven patients who underwent stent graft placement for a dysfunctional hemodialysis graft were included in this retrospective study. Expanded polytetrafluoroethylene covered stent grafts were placed at the venous anastomosis site in case of pseudoaneurysm, venous laceration, elastic recoil or residual restenosis despite the repeated angioplasty. The patency of the arteriovenous graft was evaluated using Kaplan-Meier analysis. Primary and secondary mean patency was 363 days and 741 days. Primary patency at 3, 6, and 12 months was 82%, 73%, and 32%, respectively. Secondary patency at the 3, 6, 12, 24, and 36 months was improved to 91%, 82%, 82%, 50%, and 25%, respectively. Fractures of the stent graft were observed in 2 patients, but had no effect on the patency. Stent graft placement in dysfunctional arteriovenous graft is useful and effective in prolonging graft patency.

  10. Noncavernous arteriovenous shunts mimicking carotid cavernous fistulae

    Science.gov (United States)

    Kobkitsuksakul, Chai; Jiarakongmun, Pakorn; Chanthanaphak, Ekachat; Singhara Na Ayudya, Sirintara (Pongpech)

    2016-01-01

    PURPOSE The classic symptoms and signs of carotid cavernous sinus fistula or cavernous sinus dural arteriovenous fistula (AVF) consist of eye redness, exophthalmos, and gaze abnormality. The angiography findings typically consist of arteriovenous shunt at cavernous sinus with ophthalmic venous drainage with or without cortical venous reflux. In rare circumstances, the shunts are localized outside the cavernous sinus, but mimic symptoms and radiography of the cavernous shunt. We would like to present the other locations of the arteriovenous shunt, which mimic the clinical presentation of carotid cavernous fistulae, and analyze venous drainages. METHODS We retrospectively examined the records of 350 patients who were given provisional diagnoses of carotid cavernous sinus fistulae or cavernous sinus dural AVF in the division of Interventional Neuroradiology, Ramathibodi Hospital, Bangkok between 2008 and 2014. Any patient with cavernous arteriovenous shunt was excluded. RESULTS Of those 350 patients, 10 patients (2.85%) were identified as having noncavernous sinus AVF. The angiographic diagnoses consisted of three anterior condylar (hypoglossal) dural AVF, two traumatic middle meningeal AVF, one lesser sphenoid wing dural AVF, one vertebro-vertebral fistula (VVF), one intraorbital AVF, one direct dural artery to cortical vein dural AVF, and one transverse-sigmoid dural AVF. Six cases (60%) were found to have venous efferent obstruction. CONCLUSION Arteriovenous shunts mimicking the cavernous AVF are rare, with a prevalence of only 2.85% in this series. The clinical presentation mainly depends on venous outflow. The venous outlet of the arteriovenous shunts is influenced by venous afferent-efferent patterns according to the venous anatomy of the central nervous system and the skull base, as well as by architectural disturbance, specifically, obstruction of the venous outflow. PMID:27767958

  11. Immunohistochemical study in dural arteriovenous fistula and possible role of ephrin-B2 for development of dural arteriovenous fistula

    Institute of Scientific and Technical Information of China (English)

    Wuttipong Tirakotai 王宝隆; BIAN Liu-guan 卞留贯; Helmut Bertalanffy; Siegfried Bien; Ulrich Sure

    2004-01-01

    Background Although there were several clinical and experimental studies discussing the pathogenesis of dural arteriovenous fistula (DAVF), the pathological process leading to intracranial DAVF so far remains unknown. In this study, we investigated the expression of vascular growth factors in order to elucidate the possible role of these factors for the development of DAVF and to study the biological activity of this uncommon lesion.Methods We examined the histological features, proliferative and angiogenic capacities of the tissue specimens obtained from 6 patients who underwent surgery at our institution. Immunohistochemical staining for vascular endothelial growth factor (VEGF), its receptors Flk-1 and Flt-1, ephrin-B2, MIB-1 and proliferating cell nuclear antigen (PCNA) was performed using standard immunohistochemical techniques. Results A positive immunostaining was found for all antibodies studied except MIB-1, whereas nuclear endothelial expression of PCNA was observed in only 3/6 cases. VEGF stained positive in all of the available specimens (6/6). Flk-1 showed a positive immunoreaction in only 2/6 cases and Flt-1 in 4/6 cases. Ephrin-B2 was expressed in the majority (5/6) of the cases.Conclusions These results support the hypothesis that DAVFs might be acquired dynamic vascular malformations with low biological activity. Vascular growth factors like VEGF and ephrin-B2 might play a pivotal role in the formation of DAVF.

  12. Congenital pial arteriovenous fistula in the temporal region draining into cavernous sinus: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Ziyin; Wang, Chaohua; Zhang, Changwei; Xie, Xiaodong [Dept. of Neurosurgery, West China Hospital of Sichuan University, Chengdu (China); Wang, Kun; Tang, Jianjian [Dept. of Neurosurgery, Affiliated Hospital of Hainan Medical College, Haikou (China)

    2013-06-15

    This report concerns a 4-month-old infant with progressive prominent and redness of his left eye since birth. This report concerns a 4-month-old infant with progressive prominent redness of his left eye since birth. Angiography revealed a congenital pial arteriovenous fistula between the temporal branch of the left posterior cerebral artery and left cavernous sinus through the sphenoparietal sinus, a condition not reported in the literature. The fistula was successfully occluded with two micro-coils by vertebrobasilar approach.

  13. Familial Chiari malformation: case series.

    Science.gov (United States)

    Schanker, Benjamin D; Walcott, Brian P; Nahed, Brian V; Kahle, Kristopher T; Li, Yan Michael; Coumans, Jean-Valery C E

    2011-09-01

    Chiari malformations (Types I-IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%-0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

  14. Periodontal manifestations and management of a patient with AV malformation

    Directory of Open Access Journals (Sweden)

    Sumit Narang

    2012-01-01

    Full Text Available Arterio-venous malformation (AVM is an abnormal communication between an artery and a vein. The incidence of its occurrence in oral and maxillofacial region is rare, and if present, the most common sign is gingival bleeding. A 12-year-old female patient presented with an extra oral swelling in relation with upper lip. Intra oral examination showed non tender gingival swelling with spontaneous bleeding associated with maxillary arch. On initiation of phase I therapy using hand instruments, spontaneous brisk bleeding was encountered which was difficult to control. Because of severe nature of hemorrhage encountered, some type of vascular abnormality was suspected. Ultrasonography followed by angiography confirmed AVM in relation with upper lip. Embolization of lesion was followed by gingivectomy procedure and no recurrence was reported during one year of follow-up. Thus, proper recognition and therapeutic intervention is essential to avoid serious complications and potentially tragic outcome in such situations.

  15. Coronary Arteriovenous Fistula Causing Hydrops Fetalis

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    Nilüfer Çetiner

    2014-01-01

    Full Text Available Fetal heart failure and hydrops fetalis may occur due to systemic arteriovenous fistula because of increased cardiac output. Arteriovenous fistula of the central nervous system, liver, bone or vascular tumors such as sacrococcygeal teratoma were previously reported to be causes of intrauterine heart failure. However, coronary arteriovenous fistula was not reported as a cause of fetal heart failure previously. It is a rare pathology comprising 0.2–0.4% of all congenital heart diseases even during postnatal life. Some may remain asymptomatic for many years and diagnosed by auscultation of a continuous murmur during a routine examination, while a larger fistulous coronary artery opening to a low pressure cardiac chamber may cause ischemia of the affected myocardial region due to steal phenomenon and may present with cardiomyopathy or congestive heart failure during childhood. We herein report a neonate with coronary arteriovenous fistula between the left main coronary artery and the right ventricular apex, who presented with hydrops fetalis during the third trimester of pregnancy.

  16. Endovascular treatment of hemodialysis arteriovenous fistulas

    DEFF Research Database (Denmark)

    Heerwagen, Søren T; Hansen, Marc A; Schroeder, Torben V;

    2012-01-01

    Purpose: The purpose of this study was to investigate if the immediate hemodynamic outcome of an endovascular intervention on a dysfunctional hemodialysis arteriovenous fistula is a prognostic factor for primary patency. Methods: This was a prospective observational study including 61 consecutive...

  17. Scimitar syndrome with pulmonary arteriovenous fistulas.

    Science.gov (United States)

    Le Rochais, J P; Icard, P; Davani, S; Abouz, D; Evrard, C

    1999-10-01

    Right abnormal pulmonary venous return into the inferior vena cava associated with abnormal fissure, dextrocardia, and systemic arterial supply of a variable degree, are the characteristics of the scimitar syndrome. We report on a patient in whom this rare syndrome was associated with pulmonary arteriovenous fistulas within the involved lung.

  18. Vector Volume Flow in Arteriovenous Fistulas

    DEFF Research Database (Denmark)

    Hansen, Peter Møller; Heerwagen, Søren; Pedersen, Mads Møller;

    2013-01-01

    , but is very challenging due to the angle dependency of the Doppler technique and the anatomy of the fistula. The angle independent vector ultrasound technique Transverse Oscillation provides a new and more intuitive way to measure volume flow in an arteriovenous fistula. In this paper the Transverse...

  19. Vascular malformations of the upper limb: a review of 270 patients.

    Science.gov (United States)

    Upton, J; Coombs, C J; Mulliken, J B; Burrows, P E; Pap, S

    1999-09-01

    Vascular malformations of the upper limb were once thought to be impossible to properly diagnose and treat. We reviewed our experience with these malformations of the upper limb in 270 patients seen over a 28-year period. These anomalies were slightly more common in females than males (ratio, 1.5:1.0). The malformations were categorized as either slow flow (venous, n = 125; lymphatic, n = 47; capillary, n = 32; combined, n = 33) or fast flow (arterial, n = 33). Three categories of fast-flow malformations were identified and designated as types A, B, and C. Over 90% of these lesions could be properly diagnosed by their appearance and growth pattern within the first 2 years of life. Additional radiographic studies were used to confirm this diagnosis and to define specific characteristics. Magnetic resonance imaging with and without contrast best demonstrated site, size, flow characteristics, and involvement of contiguous structures for all types of malformations. Algorithms for treatment of both slow-flow and fast-flow anomalies are presented. Two hundred sixty surgical resections were performed in 141 patients, including 24 of 33 fast-flow anomalies. Preoperative angiographic assessment, with magnified views, was an important preoperative adjunct before any well-planned resection of fast-flow arteriovenous malformations. The surgical strategy in all groups was to thoroughly extirpate the malformation, with preservation of nerves, tendons, joints, and uninvolved muscle, and microvascular revascularization and skin replacement as required. Resections were always restricted to well-defined regions and often completed in stages. Symptomatic slow-flow malformations and types A and B fast-flow anomalies were resected without major sequelae. Type C arterial anomalies, diffuse, pulsating lesions with distal vascular steal, and involvement of all tissues, including bone, progressed clinically and resulted in amputation in 10 of 14 patients. The complication rate was 22% for

  20. Medida da diferença artério-venosa de oxigênio na monitorização de pacientes com hemorragia subaracnóidea por aneurisma cerebral Measurement of arteriovenous oxygen difference in the monitoring of patients with subarachnoid haemorrhage due to cerebral aneurysm

    Directory of Open Access Journals (Sweden)

    Ronaldo Sérgio Santana Pereira

    1997-01-01

    Full Text Available A diferença artério-venosa de oxigênio (DAVO2, pelo fato de estar relacionada com o metabolismo cerebral, reflete alterações que ocorrem em determinadas situações patológicas, entre elas as causadas pela hemorragia subaracnóidea espontânea (HSAE. Com a finalidade de avaliar a relação entre alterações na DAVO2 com o quadro clínico e a evolução de pacientes com HSAE, devido à ruptura de aneurisma cerebral, este método foi utilizado em 30 pacientes portadores desta patologia, admitidos na Unidade de Neurocirurgia do HBDF. A HSAE foi confirmada por CT de crânio em 17 pacientes e por punção lombar em 13. Dezoito pacientes foram admitidos com Hunt & Hess (H&H I ou II, sete com H&H III e cinco com H&H IV ou V. A medida da DAVO2 baseou-se na equação de Fick e os resultados clínicos foram avaliados pela escala de seqüelas de Glasgow. Dezenove pacientes apresentaram DAVO2 normais (inicialmente e durante a evolução, sendo que três faleceram; cinco tiveram valores de DAVO2 sempre baixos e três faleceram; os restantes seis pacientes tiveram valores da DAVO2 sempre elevados e dois faleceram. Os pacientes com DAVO2 normais tiveram melhor evolução clínica e índice de mortalidade menor, quando comparados com os pacientes com valores anormais da DAVO2 (pThe arterious venous oxygen difference (AVDO2 due to the close relationship with cerebral metabolic rate of oxygen and cerebral blood flow shows metabolic alterations that occur in some pathological situations in the brain including subarachnoid haemorrhage. The AVDO2 was calculated by the Fick equation and the results evaluated by the Glasgow outcome scale. Measurements of arteriojugular oxygen difference were carried out in 30 patients with subarachnoid haemorrhage due to rupture of intracranial aneurysms, as an attempt to monitor the relationship between changes in AVDO2, clinical picture, and evolution of the patients. The subarachnoid haemorrhage was diagnosed by CT scan in

  1. Endovascular management of sigmoid sinus dural arteriovenous fistula associated with sinus stenosis in an infant.

    Science.gov (United States)

    Cohen, José E; Gomori, John M; Benifla, Moni; Itshayek, Eyal; Moscovici, Samuel

    2013-01-01

    A 4-month-old female presented with a dural arteriovenous fistula (DAVF), which was successfully managed using endovascular techniques. There are very few case series reporting DAVF in infants younger than 12 months and, to our knowledge, only 60 pediatric patients with DAVF have been reported to date. Although most DAVF have a benign course, they can result in life-threatening hemorrhage. Endovascular therapies are usually indicated in the management of these neurosurgical vascular malformations. Endovascular therapy of DAVF in neonatal patients presents some major issues. Gaining arterial access may be problematic in femoral arteries too small for the introduction of a sizeable guiding catheter. The volumes of contrast and infused fluids must be carefully monitored to prevent fluid overload. Radiation exposure should be restricted as far as possible. This report contributes to the limited body of evidence on neonatal DAVF and its endovascular management.

  2. Endovascular treatment of congenital arteriovenous fistulae of the internal maxillary artery

    Energy Technology Data Exchange (ETDEWEB)

    Kim, B.S. [Department of Radiology, Catholic University of Korea, Seoul (Korea); Lee, S.K.; terBrugge, K.G. [Department of Medical Imaging, Toronto Western Hospital, Fell Pavilion 3-210, 399 Bathurst St, Toronto, Ontario M5T2S8 (Canada)

    2003-07-01

    Congenital arteriovenous fistulae (AVF) of the internal maxillary artery (IMA) are rare. We present the angiographic findings and management of six AVF of the IMA, selected from 147 patients with facial vascular malformations. The fistula was thought to be congenital in all six in view of a life-long history, with no recorded trauma. Our analysis included angioarchitecture, treatment modality, embolic material, treatment results and follow-up. All patients had angiography showing an AVF originating from the IMA and draining to the jugular vein. Five patients underwent endovascular treatment with detachable balloons; a combination of Guglielmi detachable coils and N-acetyl-2-cyanoacrylate (NBCA) was used in one child. We successfully closed the AVF in all cases, without procedure-related complications, except for delayed transient facial numbness in one patient. No recurrence was observed on follow-up of 5 months to 7 years (mean 44 months). (orig.)

  3. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  4. Measurement of cerebral circulation times by means of the analysis of the time-density curve using digital subtraction angiography

    Energy Technology Data Exchange (ETDEWEB)

    Tazawa, Toshiaki; Karasawa, Jun; Touho, Hajime; Kobayashi, Keisuke; Nakagawara, Jyoji; Nakauchi, Mikio; Kuriyama, Yoshihiro

    1987-04-01

    Five patients who had suffered a transient ischemic attack (TIA) with a normal angiogram were selected as a control group for the study of the internal-carotid-artery system, while 2 patients were selected as the controls in the study of the vertebral-artery system in order to evaluate the effect of cerebrovascular lesions. The cerebral circulation time of the internal carotid artery system was defined as the difference in the arrival times of the intraarterial contrast bolus between Fischer's C1 portion and the middle of the transverse sinus, while that of the vertebral artery system was defined as the difference in arraival times between Krayenbuhl's third or fourth segment and the middle of the transverse sinus. In the internal-carotid-artery system, the circulation time (as the time difference between the two points for the control) was 4.76 +- 0.28 (mean +- SD) sec. in TTP and 4.66 +- 0.11 sec. in MTT. In the vertebral-artery system, it was 3.97 sec. on the average in TTP and 4.16 sec. in MTT. Eleven patients with middle cerebral artery stenosis or occlusion and one patient with ''MoyaMoya'' disease were selected for a study of the ischemic lesions, while one patient with an arteriovenous malformation (AVM) was selected for a study of the effect of an arteriovenous shunt. In the symptomatic patients with middle-cerebral-artery occlusion or stenosis, the circulation time was slower than that of the control group both in TTP and in MTT. There was a significant correlation between the values of the circulation time in TTP and that in MTT. The circulation time of the patient with ''MoyaMoya'' disease was significantly slower than that of the control. In the patient with an AVM, the circulation time was much faster than in the control in TTP, while it was normal in MTT. (J.P.N.).

  5. Brain Abscesses Associated with Asymptomatic Pulmonary Arteriovenous Fistulas

    Science.gov (United States)

    Nam, Taek-Kyun; Park, Yong-sook; Kwon, Jeong-taik

    2017-01-01

    Brain abscess commonly occurs secondary to an adjacent infection (mostly in the middle ear or paranasal sinuses) or due to hematogenous spread from a distant infection or trauma. Pulmonary arteriovenous fistulas (AVFs) are abnormal direct communications between the pulmonary artery and vein. We present two cases of brain abscess associated with asymptomatic pulmonary AVF. A 65-year-old woman was admitted with a headache and cognitive impairment that aggravated 10 days prior. An magnetic resonance (MR) imaging revealed a brain abscess with severe edema in the right frontal lobe. We performed a craniotomy and abscess removal. Bacteriological culture proved negative. Her chest computed tomography (CT) showed multiple AVFs. Therapeutic embolization of multiple pulmonary AVFs was performed and antibiotics were administered for 8 weeks. A 45-year-old woman presented with a 7-day history of progressive left hemiparesis. She had no remarkable past medical history or family history. On admission, blood examination showed a white blood cell count of 6290 cells/uL and a high sensitive C-reactive protein of 2.62 mg/L. CT and MR imaging with MR spectroscopy revealed an enhancing lesion involving the right motor and sensory cortex with marked perilesional edema that suggested a brain abscess. A chest CT revealed a pulmonary AVF in the right upper lung. The pulmonary AVF was obliterated with embolization. There needs to consider pulmonary AVF as an etiology of cerebral abscess when routine investigations fail to detect a source. PMID:28061502

  6. Classification and diagnosis of ear malformations

    OpenAIRE

    Bartel-Friedrich, Sylva; Wulke, Cornelia

    2008-01-01

    In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations) or acquired in nature. Malformations can affect the outer ear (pinna and externa...

  7. Congenital cardiovascular malformations and the fetal circulation.

    Science.gov (United States)

    Rudolph, A M

    2010-03-01

    After birth, gas exchange is achieved in the lung, whereas prenatally it occurs in the placenta. This is associated with differences in blood flow patterns in the fetus as compared with the postnatal circulation. Congenital cardiovascular malformations are associated with haemodynamic changes in the fetus, which differ from those occurring postnatally. Obstruction to cardiac outflow may alter myocardial development, resulting in progressive ventricular hypoplasia. Alteration of oxygen content may profoundly influence pulmonary vascular and ductus arteriosus responses. Interference in blood flow and oxygen content may affect cerebral development as a result of inadequate oxygen or energy substrate supply. The circulatory effects may be gestational dependent, related to maturation of vascular responses in different organs. These prenatal influences of congenital cardiac defects may severely affect immediate, as well as longterm, postnatal prognosis and survival. This has stimulated the development of techniques for palliation of disturbed circulation during fetal life.

  8. Anorectal malformations : A multidisciplinary approach

    NARCIS (Netherlands)

    D. van den Hondel (Desiree)

    2015-01-01

    markdownabstractAbstract The research described in this thesis was performed with the aim to evaluate and improve multidisciplinary treatment of anorectal malformation patients. An overview of current literature on treatment of anorectal malformations is given in the Preface section, which also inc

  9. Gradient-based 3D-2D registration of cerebral angiograms

    Science.gov (United States)

    Mitrović, Uroš; Markelj, Primož; Likar, Boštjan; Miloševič, Zoran; Pernuš, Franjo

    2011-03-01

    Endovascular treatment of cerebral aneurysms and arteriovenous malformations (AVM) involves navigation of a catheter through the femoral artery and vascular system into the brain and into the aneurysm or AVM. Intra-interventional navigation utilizes digital subtraction angiography (DSA) to visualize vascular structures and X-ray fluoroscopy to localize the endovascular components. Due to the two-dimensional (2D) nature of the intra-interventional images, navigation through a complex three-dimensional (3D) structure is a demanding task. Registration of pre-interventional MRA, CTA, or 3D-DSA images and intra-interventional 2D DSA images can greatly enhance visualization and navigation. As a consequence of better navigation in 3D, the amount of required contrast medium and absorbed dose could be significantly reduced. In the past, development and evaluation of 3D-2D registration methods received considerable attention. Several validation image databases and evaluation criteria were created and made publicly available in the past. However, applications of 3D-2D registration methods to cerebral angiograms and their validation are rather scarce. In this paper, the 3D-2D robust gradient reconstruction-based (RGRB) registration algorithm is applied to CTA and DSA images and analyzed. For the evaluation purposes five image datasets, each comprised of a 3D CTA and several 2D DSA-like digitally reconstructed radiographs (DRRs) generated from the CTA, with accurate gold standard registrations were created. A total of 4000 registrations on these five datasets resulted in mean mTRE values between 0.07 and 0.59 mm, capture ranges between 6 and 11 mm and success rates between 61 and 88% using a failure threshold of 2 mm.

  10. Stent-graft repair of a recurrent popliteal arteriovenous fistula

    NARCIS (Netherlands)

    Tielliu, IFJ; Verhoeven, ELG; Prins, TR; van Det, M; van den Dungen, JJAM

    2002-01-01

    Purpose: To describe the successful endovascular treatment of a popliteal arteriovenous fistula with a stent-graft. Case Report: A 54-year-old man was referred to our hospital with a distal popliteal arteriovenous fistula following an arthroscopic meniscectomy 6 years earlier. Three surgical attempt

  11. 颅内发育性静脉异常伴海绵状血管畸形三例患者的临床及影像学特点%Clinical and imaging feature of 3 patients with combined developmental venous anomalies and cerebral cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    刘建军; 熊葶; 李尊波; 李盛彧; 贾颐

    2012-01-01

    Objective To investigate the clinical,neuroradiologic characteristics and possible causes in 3 patients with combined developmental venous anomalies (DVAs) and cerebral cavernous malformations (CCM).Methods The clinical examination,magnetic resonance imaging (MRI) T1-weighted (T1 WI),T2-weighted (T2WI),susceptibility-weighted imaging (SWI) or T2 fast field echo (T2 FFE),contrast-enhanced MRI at 1.5 T field strength and digital substrate angiography were performed in 3 patients.Results Three patients presented with the seizure,vertigo,and dizziness respectively.MRI findings of reticulated “popcorn like” lesion with complete hemosiden rim showed typical sign of CCM.DSA,contrast-enhanced MRI and MRI-SWI revealed the caput medusae of the medullary veins and collected veins which was drained into subcortical and deep venous system,which indicated DVAs in 3 patients.The angulated medullary veins and collected veins in approaching distal zone of CCM were observed.Conclusion DVAs can be combined with CCM.The angulated medullary veins and collected veins combined with CCM in same territory reveals that the angioarchitectural factors is a key factor in pathogenesis of cavernous malformation.%目的 探索颅内发育性静脉异常(DVAs)并发海绵状血管畸形(CCM)的临床及影像学特点.方法 对分别以癫痫、头昏、头晕发病的3例患者行头颅MRI磁敏感成像(MRI-SWI)或T2快速梯度回波(T2FFE)、钆喷酸葡胺增强、MRA及全脑血管造影检查.结果 MRI-SWI或T2FFE可见边缘低信号、中心为不同强度混杂信号的爆米花样或网格状的病灶,分布于额叶皮质及皮质下,提示CCM;在MRI增强及全脑血管造影中可见水母头样表现,提示有DVAs.在分析DVAs和CCM的关系中发现CCM病灶均发生在DVAs区域,可见引流静脉与CCM病灶呈成角弯曲.结论 DVAs可并发CCM,影像学可提供有特征性的改变,DVAs血管因素可能是CCM形成的原因之一.

  12. Pericallosal lipoma and middle cerebral artery aneurysm: a coincidence?

    Energy Technology Data Exchange (ETDEWEB)

    Sommet, Julie; Schiff, Manuel; Evrard, Philippe [Hopital Robert Debre, APHP, Department of Paediatric Neurology and Metabolic Diseases, Paris Cedex 19 (France); Blanc, Raphael [Fondation Rothschild, Department of Interventional Radiology, Paris (France); Elmaleh-Berges, Monique [Hopital Robert Debre, Department of Paediatric Radiology, Paris (France)

    2010-08-15

    Intracranial lipomas are rare congenital malformations that can often be seen in association with other brain malformations; agenesis or dysgenesis of the corpus callosum is the most frequently associated brain anomaly. They are usually pericallosal asymptomatic midline lesions. Intracranial lipomas associated with a non-contiguous cerebral aneurysm are extremely rare. We report an infant with partial agenesis of the corpus callosum and pericallosal lipoma associated with cerebral haemorrhage due to a distal middle cerebral artery aneurysm. Such an association is probably not fortuitous and could suggest a pathogenic relationship. (orig.)

  13. Hereditary hemorrhagic telangiectasia with bilateral pulmonary vascular malformations: A case report

    Directory of Open Access Journals (Sweden)

    Lončarević Olivera

    2016-01-01

    Full Text Available Introduction. Hereditary hemorrhagic telangiectasia (HHT also known as Osler-Weber-Rendu syndrome is an autosomal dominant disease that occurs due to vascular dysplasia associated with the disorder in the signaling pathway of transforming growth factor β (TGF-β. The clinical consequence is a disorder of blood vessels in multiple organ systems with the existence of telangiectasia which causes dilation of capillaries and veins, are present from birth and are localized on the skin and mucosa of the mouth, respiratory, gastrointestinal and urinary tract. They can make a rupture with consequent serious bleeding that can end up with fatal outcome. Since there is a disruption of blood vessels of more than one organic system, the diagnosis is very complex and requires a multidisciplinary approach. Case report. We reported a 40-year-old female patient with a long-time evolution of problems, who was diagnosed and treated at the Clinic for Lung Diseases of the Military Medical Academy in Belgrade, Serbia, because of bilaterally pulmonary arteriovenous malformations associated with HHT. Embolization was performed in two acts, followed with normalization of clinical, radiological and functional findings with the cessation of hemoptysis, effort intolerance with a significant improvement of the quality of life. Conclusion. HHT is a rare dominant inherited multisystem disease that requires multidisciplinary approach to diagnosis and treatment. Embolization is the method of choice in the treatment of arteriovenous malformations with minor adverse effects and very satisfying therapeutic effect.

  14. Imaging of spontaneous ventriculomegaly and vascular malformations in Wistar rats: implications for preclinical research.

    Science.gov (United States)

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

    2014-12-01

    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 used, 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 (43.2%) of 162 Wistar rats that had been obtained from 2 different vendors. Advanced magnetic resonance (MR) imaging techniques, including MR angiography and diffusion tensor imaging, were used to evaluate the rats. Multiple neuropathologic abnormalities, including presumed arteriovenous malformations, aneurysms, cysts, white matter lesions, and astrogliosis were found in association with ventriculomegaly. Postmortem microcomputed tomography and immunohistochemical staining confirmed the presence of aneurysms and arteriovenous malformations. Diffusion tensor imaging showed significant decreases in fractional anisotropy and increases in mean diffusivity, axial diffusivity, and radial diffusivity in multiple white matter tracts (p ventriculomegaly. We suggest the use of baseline imaging to prevent the inadvertent introduction of a high degree of variability in preclinical studies of neurologic disease or injury in Wistar rats.

  15. Acquired uterine vascular malformations: radiological and clinical outcome after transcatheter embolotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Maleux, Geert; Heye, Sam; Wilms, Guy [University Hospitals Gasthuisberg, Department of Radiology, Leuven (Belgium); Timmerman, Dirk [University Hospitals Gasthuisberg, Department of Obstetrics and Gynecology, Leuven (Belgium)

    2006-02-01

    The purpose of this retrospective study is to assess the radiological and clinical outcome of transcatheter embolization of acquired uterine vascular malformations in patients presenting with secondary postpartum or postabortion vaginal hemorrhage. In a cohort of 17 patients (mean age: 29.7 years; standard deviation: 4.23; range: 25-38 years) 18 embolization procedures were performed. Angiography demonstrated a uterine parenchymal hyperemia with normal drainage into the large pelvic veins (''low-flow uterine vascular malformation'') in 83% (n=15) or a direct arteriovenous fistula (''high-flow uterine vascular malformation'') in 17% (n=3). Clinically, in all patients the bleeding stopped after embolization but in 1 patient early recurrence of hemorrhage occurred and was treated by hysterectomy. Pathological analysis revealed a choriocarcinoma. During follow-up (mean time period: 18.8 months; range: 1-36 months) 6 patients became pregnant and delivered a healthy child. Transcatheter embolization of the uterine arteries, using microparticles, is safe and highly effective in the treatment of a bleeding acquired uterine vascular malformation. In case of clinical failure, an underlying neoplastic disease should be considered. Future pregnancy is still possible after embolization. (orig.)

  16. Nonsurgical treatment of congenital vascular malformations of the trunk and limb by transcatheter embolization

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Choi, Dae Seob; Han, Joon Koo; Chung, Jin Wook; Han, Moon Hee; Goo, Jin Mo; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-01-15

    We evaluated the effectiveness of embolization in the management of congenital vascular malformations(CVMs). A retrospective analysis was performed in 10 patients who underwent embolotherapy for their soft tissue CVMs(eight arteriovenous malformations, one hemangioma, one venous malformation) involving the extremity or back. In nine patients, intraarterial embolization was performed with multiple embolic agents including Gelfoam, Ivalon, IBCA, ethanol mixed with Lipiodol, and micro-coils. In one patient with venous malformation, direct puncture and embolization of the lesion were performed with absolute ethanol mixed with Lipiodol. In four patients, who subsequently underwent surgery, preoperative embolization decreased operative morbidity and facilitated a thorough extirpation. Five of six patients with inoperable CVMs experienced dramatic reduction of symptoms by single or repeated embolization, with a mean follow-up period of four months. We believe that preoperative embolization may decrease operative morbidity and facilitate a thorough extirpation in the patients with extensive but resectable CVMs and repeated embolizations are effective for symptom control in the patients with inoperable CVMs.

  17. Microsurgical management of deep ruptured arteriovenous malformations of the basal ganglia and thalamus

    Directory of Open Access Journals (Sweden)

    Sandu Aurelia Mihaela

    2014-03-01

    Full Text Available Introduction : Deep AVMs are more difficult to operate compared with other supratentorial locations due to the long working corridor through the brain, location in eloquent areas and vicinity with brain critical structures.

  18. Ruptured spinal arteriovenous malformation: Presenting as stunned myocardium and neurogenic shock

    Directory of Open Access Journals (Sweden)

    Tasneem H Mehesry

    2015-01-01

    Conclusions: Spinal AVM rupture can present as neurogenic shock, stunned myocardium, and pulmonary edema. Early recognition of AVM rupture and prompt surgical intervention, as well as aggressive treatment of shock, may enhance recovery and decrease the long-term morbidity.

  19. Stereotactic Radiosurgery with Neoadjuvant Embolization of Larger Arteriovenous Malformations: An Institutional Experience

    Directory of Open Access Journals (Sweden)

    Richard Dalyai

    2014-01-01

    Full Text Available Objective. This study investigates the safety and efficacy of a multimodality approach combining staged endovascular embolizations with subsequent SRS for the management of larger AVMs. Methods. Ninety-five patients with larger AVMs were treated with staged endovascular embolization followed by SRS between 1996 and 2011. Results. The median volume of AVM in this series was 28 cm3 and 47 patients (48% were Spetzler-Martin grade IV or V. Twenty-seven patients initially presented with hemorrhage. Sixty-one patients underwent multiple embolizations while a single SRS session was performed in 64 patients. The median follow-up after SRS session was 32 months (range 9–136 months. Overall procedural complications occurred in 14 patients. There were 13 minor neurologic complications and 1 major complication (due to embolization while four patients had posttreatment hemorrhage. Thirty-eight patients (40% were cured radiographically. The postradiosurgery actuarial rate of obliteration was 45% at 5 years, 56% at 7 years, and 63% at 10 years. In multivariate analysis, larger AVM size, deep venous drainage, and the increasing number of embolization/SRS sessions were negative predictors of obliteration. The number of embolizations correlated positively with the number of stereotactic radiosurgeries (P<0.005. Conclusions. Multimodality endovascular and radiosurgical approach is an efficacious treatment strategy for large AVM.

  20. Embolotherapy for pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D; Oxhøj, H

    1998-01-01

    with transcatheter embolization of 20 PAVMs using 12 detachable silicone balloons and 26 steel coils. RESULTS: All PAVMs were completely occluded and we observed a significant rise in PaO2 after treatment and a significant decrease in right-to-left shunt estimated by contrast echocardiography. All patients...

  1. Clinical and radiological long-term follow-up after embolization of pulmonary arteriovenous malformations

    DEFF Research Database (Denmark)

    Andersen, Poul Erik; Kjeldsen, Anette D

    2005-01-01

    . Outcome parameters at follow-up were PaO(2) and patients' satisfaction. During follow-up, the patients had a clinical examination, measurement of arterial blood gases, chest X-ray, and contrast echocardiography performed and were asked to fill in a questionnaire exploring experience of the treatment...

  2. Ruptured spinal arteriovenous malformation: Presenting as stunned myocardium and neurogenic shock

    OpenAIRE

    Mehesry, Tasneem H.; Nissar Shaikh; Malmstrom, Mohammad F.; Marcus, Marco A.E.; Adnan Khan

    2015-01-01

    Background: Neurogenic pulmonary edema (NPE) is a clinical syndrome usually defined as an acute pulmonary edema occurring shortly after a central neurologic insult. NPE was identified 100 years ago, but it is still underappreciated in the clinical setup. NPE usually appears within minutes to hours after the injury. It has a high mortality rate if not recognized early and treated appropriately. Similarly, neurogenic shock is a known complication of spinal cord injury reported incidence is more...

  3. A Developmental and Genetic Classification for Malformations of Cortical Development: Update 2012

    Science.gov (United States)

    Barkovich, A. James; Guerrini, Renzo; Kuzniecky, Ruben I.; Jackson, Graeme D.; Dobyns, William B.

    2012-01-01

    Malformations of cerebral cortical development include a wide range of developmental disorders that are common causes of neurodevelopmental delay and epilepsy. In addition, study of these disorders contributes greatly to the understanding of normal brain development and its perturbations. The rapid recent evolution of molecular biology, genetics…

  4. Evaluation of flow characteristics of soft-tissue vascular malformations using technetium-99m labelled red blood cells

    Energy Technology Data Exchange (ETDEWEB)

    Inoue, Yusuke; Yoshikawa, Kohki; Yoshioka, Naoki; Ohtake, Tohru; Ohtomo, Kuni [Tokyo Univ. (Japan). Dept. of Radiology; Wakita, Shinichi; Kaji, Nobuyuki; Harii, Kiyonori [Dept. of Plastic Surgery, Univ. of Tokyo (Japan)

    1999-04-29

    The estimation of intralesional haemodynamics is crucial in determining appropriate treatment for soft-tissue vascular malformations. The aim of this study was to develop a method to evaluate the flow characteristics of soft-tissue vascular malformations using technetium-99m labelled red blood cells ({sup 99m}Tc-RBCs). Seventy-nine soft-tissue vascular malformations, including 20 arteriovenous malformations and 59 venous malformations, in 57 patients were examined. Following the intravenous injection of {sup 99m}Tc-RBCs, dynamic imaging was performed for 30 min with the lesion in the field of view ({sup 99m}Tc-RBC flow study). A time-activity curve was generated for the lesion, and the lesion was categorized as a high-flow or low-flow lesion by visual inspection of the curve. In low-flow lesions, mean vascular transit time (MTT) was calculated by curve fitting based on a two-compartment model. Twenty-nine lesions in 19 patients were examined twice, and reproducibility was assessed. In 23 venous malformations in 16 patients, {sup 99m}Tc-Sn colloid was percutaneously injected into the intravascular space of the lesion, and dynamic data of 5-min duration were acquired (direct puncture scintigraphy). MTT was estimated from the washout curve and compared with MTT estimated by {sup 99m}Tc-RBC flow study. {sup 99m}Tc-RBC flow study classified all 20 arteriovenous malformations as high-flow lesions and all 59 venous malformations as low-flow lesions. In the low-flow lesions, MTT estimated by {sup 99m}Tc-RBC flow study ranged from 61.2 to 2174.9 s. In the reproducibility study, complete concordance in classification and high correlation in MTT were shown between the first and second examinations. MTT estimated by {sup 99m}Tc-RBC flow study was significantly correlated with that estimated by direct puncture scintigraphy. In summary, {sup 99m}Tc-RBC flow study provides a quantitative indicator of intralesional haemodynamics in low-flow lesions in addition to accurate

  5. Udredning og behandling af morbus Osler

    DEFF Research Database (Denmark)

    Kjeldsen, Anette Drøhse; Andersen, Poul Erik; Tørring, Pernille Mathiesen

    2011-01-01

    such as pulmonary arteriovenous malformations (PAVM), cerebral arteriovenous malformations (CAVM) and gastrointestinal bleeding. Collaboration between various medical specialties is essential in order to provide an up-to-date treatment and thorough work-up in the individual patient. Disregard of symptoms may result...

  6. Study of Congenital Malformations in Central Nervous System AND Gastro- Intestinal Tract

    Directory of Open Access Journals (Sweden)

    Saiyad SS

    2012-04-01

    Full Text Available Introduction: Congenital malformations comprise 8% of the perinatal mortality in India. They rank fifth as a cause of perinatal mortality, after asphyxia, respiratory problems, infections and cerebral trauma. However, the pattern is changing rapidly with improvement in health care and living standards. Material & Method: In the present study, authors have tried to study the cases of congenital malformations specially related to Central nervous system and Gastro-intestinal system. 5240 cases of newborn babies were studied and results were analyzed and classified in to various categories. Findings: The results show that malformations are more common in still birth, more in female babies and more in central nervous system In live born babies the percentage of malformation is0.63 % whereas in still born baby it is6.53 %. Conclusions: Chances of having malformations increases as the age advances. Parity of mother also influences the incidence. Exposure to radiation & drugs also influences malformations. Incidence of congenital malformation is highest in central nervous system. [National J of Med Res 2012; 2(2.000: 121-123

  7. Anorectal malformations in neonates

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2011-01-01

    Full Text Available Background : Anorectal malformations (ARM are associated with congenital anomalies and other risk factors, yielding a poor prognosis, especially in neonatal life. Objectives: This study was performed to identify the congenital anomalies as a factor of poor prognosis (mortality in such patients. Settings: Department of Pediatric surgery, The Children′s Hospital and The Institute of Child Health, Lahore. Design: Prospective observational study, with statistical support. Materials and Methods: The information on the demography, clinical features, investigations, management performed, and outcome was entered in the designed proforma and analysed with the help of statistical software EpiInfo version 3.5.1. Statistical test: Chi-square test was used to determine statistical significance of the results. Results : Of 100 neonates with ARM, 77 were male and 23, female (3.4:1. The mean age at presentation was 3.4 days (range, 12 hrs to 28 days. In 60 patients (60%, the presentation was imperforate anus without a clinically identified fistula. In 28 patients (28%, associated anomalies were present. The common associated anomalies were urogenital (10%, cardiovascular (8%, and gastrointestinal (6%. Down′s syndrome was present in 8 (8% patients. A total of 15 (15% deaths occurred in this study. In patients having associated congenital anomalies, 11 deaths occurred, whereas, 4 deaths were in patients without associated anomalies (P < 0.5. Conclusion : The mortality is higher in neonates with ARM having associated congenital anomalies.

  8. EFFECT OF HEPARIN ON THE PATENCY OF ARTERIOVENOUS FISTULA

    Directory of Open Access Journals (Sweden)

    H Ravari

    2008-11-01

    Full Text Available "nPatients with end stage renal disease need a good vascular access for hemodialysis. Arteriovenous fistula is the method of choice for vascular access in these patients. However, failure of arteriovenous fistula due to thrombosis is a major problem. The aim of this study was to evaluate the effect of the heparin on the patency of the arteriovenous fistula. This prospective interventional case control study was performed from November 2003 through May 2005 in vascular surgery ward in Imam Reza Hospital. All the patients who underwent a surgery in order to perform an arteriovenous fistula in cubital or snuff box areas for the dialysis means were enrolled. They were randomly divided into two groups. The case group (n = 96 received intraoperative heparin whereas the controls (n = 102 did not. Early observation of arteriovenous fistula (immediately after surgery showed patency in 89% of heparin group and in 87% of the control group. The patency rate 2 weeks after the surgery was 85% in heparin group versus 74% in the control group, resulting in a statistically significant difference (P value = 0.046. According to higher patency rate of arteriovenous fistula in 2 weeks following surgery in case group, we recommend intraoperative use of heparin in arteriovenous fistula operations.

  9. Improvement of depression after treatment of dural arteriovenous fistula: a case report and a review.

    Science.gov (United States)

    Nakagawa, Minoru; Sugiu, Kenji; Tokunaga, Koji; Sakamoto, Chihoko; Fujiwara, Kenjiro

    2012-01-01

    Patients with dural arteriovenous fistulas (DAVFs) in the transverse-sigmoid sinus suffer from several symptoms: bruit, headache, visual impairment, and so on. But depression is rare in patients with DAVF. The authors reported a rare case presenting the improvement of depression after the treatment of a dural arteriovenous fistula in the left transverse-sigmoid sinus. A 46-year-old male had suffered from depression and was treated with antidepressants at a local hospital for four years. The patient was temporarily laid off due to his depression. Afterwards, he had Gerstmann's syndrome and came to our hospital. A DAVF in the left transverse-sigmoid sinus was demonstrated on the angiogram. The DAVF was successfully treated with endovascular surgery, coil embolization of the isolated diseased sinus through the mastoid emissary vein which was a draining vein from the fistula. After this treatment, his depression as well as Gerstmann's syndrome was improved and the quantity of the antidepressants decreased. The patient returned to work without any antidepressant two years after the treatment. DAVFs might be one of the causes of depression. It may be necessary to evaluate cerebral vessels in patients suffering from depression by using MRA or 3D-CTA even if there are not any abnormal findings on plain CT scans.

  10. A dural arteriovenous fistula in cavernous sinus developed from viral meningitis.

    Science.gov (United States)

    Hai, Jian; Zhang, Lin; Wan, Jue-Feng; Su, Shao-Hua; Wang, Fei; Zhang, Gui-Yun

    2011-06-01

    Although hormonal influences, inflammation, trauma, sinus thrombosis, venous hypertension, and congenital origin have been proposed as sources of dural arteriovenous fistulas (DAVFs) in cavernous and sigmoid sinuses, the etiology of these lesions remains controversial. We present a case with a cavernous sinus DAVF developed from viral meningitis which has not been previously described. A 24-year-old male was admitted to our institute because of periorbital pain, decreased vision, pulsatile tinnitus, chemosis, and exophthalmos on the right side after he had suffered viral meningitis four months before. Cerebral angiography demonstrated a cavernous sinus DAVF, which was successfully obliterated with several platinum coils using a transvenous approach. The viral meningitis most likely caused the inflammation, that may be responsible for the occurrence of the cavernous sinus DAVF. Prompt treatment for inflammation may help to prevent the development of DAVFs.

  11. 容易误诊漏诊的硬脑膜动静脉瘘9例分析%Dural arteriovenous fistula with easily inaccurate or missed diagnosis: analysis of 9 cases

    Institute of Scientific and Technical Information of China (English)

    廖焕权; 王鸿轩; 陈红兵; 杨智云; 王莹; 贺涓涓; 尚文锦; 洪华

    2011-01-01

    目的 提高对容易误诊漏诊的硬脑膜动静脉瘘(dural arteriovenous fistula,DAVF)的诊断水平.方法 回顾性总结9例DAVF的临床资料,结合文献进行分析.结果 9例主要临床表现及误诊情况分别为:视朦或视力下降4例,无颅内高压症状,误诊为视神经炎、颅内结核感染或特发性颅内压增高;记忆力下降1例,误诊为病毒性脑炎;双下肢无力1例,误诊为急性脊髓炎、神经鞘瘤;反复头痛1例,误诊为偏头痛、特发性颅内压增高;反复抽搐1例,误诊为正常颅内压脑积水;饮水呛咳、吞咽困难1例,误诊为脑梗死.3例满足于静脉窦血栓的诊断而忽略了DAVF.所有病例影像学均漏诊,但如仔细观察仍可发现细微的血管形态改变,提示动静脉畸形.结论 DAVF临床表现缺乏特异性,加上临床及放射科医师对本病认识不足,诊断思路狭窄,造成本病误诊、漏诊率高.%Objective To improve the diagnostic capability of dural arteriovenous fistula(DAVF) which easily leads to inaccurate or missed diagnosis.Methods The clinical information of 9 cases of DAVF was summarized retrospectively, and analyzed with a literature review.Results The main clinical manifestations and misdiagnosis of 9 cases were: 4 cases presenting with only amaurosis fugax or vision loss without symptoms of increased intracranial pressure were misdiagnosed as optic neuritis,intracranial tuberculosis infection and idiopathic intracranial hypertension;1 case presenting with memory decline was misdiagnosed as viral encephalitis;1 case presenting with bilateral lower limbs weakness was misdiagnosed as acute myelitis and neurilemmoma; 1 case presenting with recurrent headache was misdiagnosed as migraine and idiopathic intracranial hypertension;1 case presenting with repeating seizures was misdiagnosed as normal intracranial pressure hydrocephalus;1 case presenting with choking water and swallowing difficulty was misdiagnosed as cerebral infarction.3

  12. Endovascular treatment for bilateral vertebral arteriovenous fistulas in neurofibromatosis 1.

    Science.gov (United States)

    Siddhartha, W; Chavhan, Govind B; Shrivastava, Manish; Limaye, Uday S

    2003-12-01

    We report a rare case of a 36-year-old woman with neurofibromatosis 1 (NF1) with bilateral vertebro-vertebral arteriovenous fistulas. The patient presented with quadriparesis and had neck pain. Angiography revealed vertebral arteriovenous fistulas bilaterally with dilated epidural venous plexuses compressing the cervical cord resulting in quadriparesis. Endovascular treatment using coils and balloons resulted in successful occlusion of both fistulas. At 6-months postembolization, the patient had improved significantly and is now able to walk with support.

  13. Vascular access for hemodialysis: arteriovenous fistula.

    Science.gov (United States)

    Malovrh, Marko

    2005-06-01

    The long-term survival and quality of life of patients on hemodialysis (HD) is dependant on the adequacy of dialysis via an appropriately placed vascular access. The optimal vascular access is unquestionably the autologous arteriovenous fistula (AVF), with the most common method being the conventional radio-cephalic fistula at the wrist. Recent clinical practice guidelines recommend the creation of native fistula or synthetic graft before the start of chronic HD therapy to prevent the need for complication-prone dialysis catheters. This could also have a beneficial effect on the rapidity of worsening kidney failure. A multidisciplinary approach (nephrologists, surgeons, radiologists and nurses) should improve the HD outcome by promoting the use of AVF. An important additional component of this program is the Doppler ultrasound for preoperative vascular mapping. Such an approach may be realized without unsuccessful surgical explorations, with a minimal early failure rate and a high maturation, even in patients with diabetes mellitus.

  14. Investigation of Brain Arterial Circle Malformations Using Electrical Modelling and Simulation

    Directory of Open Access Journals (Sweden)

    Klara Capova

    2006-01-01

    Full Text Available The paper deals with the cerebral arterial system investigation by means of electrical modelling and simulations. The main attention is paid to the brain arterial circle malformations (stenoses and aneurysms and their determination and evaluation by computer-aided methods as tools of a non-invasive diagnostics. The compensation possibilities of brain arterial circle in case of presence of concrete arterial malformations are modelled and simulated. The simulation results of brain arteries blood pressures and volume flow velocities time dependences are presented and discussed under various health conditions.

  15. Ataque cerebral

    OpenAIRE

    Takeuchi Tan, Yuri; Fundación Valle de Lili

    1998-01-01

    ¿Qué es un ataque cerebral?/¿Qué tipos de ataque cerebral existen?/¿Cuáles son los síntomas de un ataque cerebral?/Factores de riesgo para un ataque cerebral/Tratamiento médico del ataque cerebral/¿por qué es importante acudir temprano cuando se presentan las señales de alarma?/ Manejo preventivo del ataque cerebral isquémico/Tratamiento quirúrgico del ataque cerebral/Enfermedad vascular cerebral hemorrágica/¿Cómo está constituido el grupo de ataque cerebral de la fundación Clínica Valle d...

  16. 两种磁共振血管成像序列对脑静脉畸形的比较影像学研究%Comparative Imaging Study of Two Sequences of MR Angiography in Dioagnosis of Cerebral Venous Malformation

    Institute of Scientific and Technical Information of China (English)

    王任水; 李晶; 曲林涛

    2012-01-01

    Objective To discuss the diagnostic value of Venous Blood Oxygenation Dependent MR1 (VEN_BOLD) and time of flight MR angiography(TOF-MRA) in cerebral venous malformation (CVM). Methods Using 1.5T superconducting MRI scanner, 13 cases of our hospital are diagnosed by the contrast imaging analysis of TOF-MRA and VENBOLD. Results Original axial images of TOF-MRA not only could display drainage vein of all cases, but also could observe 3D images of maximum intensity prolection(MIP). Among these original axial images, only 6 cases could show few pulp vein, however, VENBOLD could clear display drainage vein and more pulp veins of all CVM cases. Conclusion TOF-MRA could directly show 3D images of drainage vein of CVM, however, VEN_BOLD could clear display drainage vein and more pulp veins of CVM, The combining of TOF-MRA and VENBOLD could be the necessary supplement sequence for CVM diagnosis.%目的 探讨磁共振静脉血氧水平依赖成像(VEN-BOLD)和时间飞跃法血管成像(TOF-MRA)对静脉畸形(CVM)的诊断价值.方法 采用1.5T超导磁共振扫描仪对我院收治的13例CVM患者进行TOF-MRA和VEN-BOLD对比成像分析.结果 TOF-MRA原始轴位图像可显示全部13例CVM的引流静脉,并可进行最大密度投影重建(MIP) 3D图像观察,但仅6例原始图像可显示稀少的髓静脉,而VEN-BOLD可清晰显示全部13例CVM的引流静脉及更多的髓静脉.结论 TOF-MRA可对CVM的引流静脉进行3D直观显示;而VEN-BOLD可清晰准确地显示CVM的引流静脉及髓静脉,二者结合可作为CVM诊断必要的补充序列.

  17. 3D-2D registration of cerebral angiograms based on vessel directions and intensity gradients

    Science.gov (United States)

    Mitrovic, Uroš; Špiclin, Žiga; Štern, Darko; Markelj, Primož; Likar, Boštjan; Miloševic, Zoran; Pernuš, Franjo

    2012-02-01

    Endovascular treatment of cerebral aneurysms and arteriovenous malformations (AVM) involves navigation of a catheter through the femoral artery and vascular system to the site of pathology. Intra-interventional navigation is done under the guidance of one or at most two two-dimensional (2D) X-ray fluoroscopic images or 2D digital subtracted angiograms (DSA). Due to the projective nature of 2D images, the interventionist needs to mentally reconstruct the position of the catheter in respect to the three-dimensional (3D) patient vasculature, which is not a trivial task. By 3D-2D registration of pre-interventional 3D images like CTA, MRA or 3D-DSA and intra-interventional 2D images, intra-interventional tools such as catheters can be visualized on the 3D model of patient vasculature, allowing easier and faster navigation. Such a navigation may consequently lead to the reduction of total ionizing dose and delivered contrast medium. In the past, development and evaluation of 3D-2D registration methods for endovascular treatments received considerable attention. The main drawback of these methods is that they have to be initialized rather close to the correct position as they mostly have a rather small capture range. In this paper, a novel registration method that has a higher capture range and success rate is proposed. The proposed method and a state-of-the-art method were tested and evaluated on synthetic and clinical 3D-2D image-pairs. The results on both databases indicate that although the proposed method was slightly less accurate, it significantly outperformed the state-of-the-art 3D-2D registration method in terms of robustness measured by capture range and success rate.

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

    Science.gov (United States)

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

    2015-07-07

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

  19. 青年脑出血128例的病因、危险因素及预后分析%Analysis of the etiology,risk factors and prognosis of youth cerebral hemorrhage in 128 cases

    Institute of Scientific and Technical Information of China (English)

    徐新良

    2015-01-01

    Objective:To explore the etiology,risk factors and prognosis of youth cerebral hemorrhage.Methods:The clinical data of 128 patients with youth cerebral hemorrhage were retrospectively analyzed.Results:99 cases(77.34% ) had definite etiology, including 43 cases(33.59% ) of hypertension,36 cases(28.13% ) of arteriovenous malformation,12 cases(9.38% ) of internal medicine diseases,6 cases(4.69%) of hematological system diseases,2 cases(1.56%) of pregnancy status.29 cases(22.67%) were unknown cause.The main risk factors were smoking,drinking.32 cases(25% ) were cured,50 cases(39.06% ) were improved,22 cases(17.19%) were unrecovered,24 cases(18.75%) were death.Conclusion:Hypertension,cerebral vascular malformation are the main causes of youth cerebral hemorrhage,its clinical symptoms are heavier,most patients have good prognosis.%目的:探讨青年脑出血的病因、危险因素及预后。方法:对128例青年脑出血患者的临床资料进行回顾性分析。结果:有明确病因99例(77.34%),其中高血压43例(33.59%),动静脉畸形36例(28.13%),内科疾病12例(9.38%),血液系统疾病6例(4.69%),妊娠状态2例(1.56%);原因不明29例(22.67%)。主要危险因素有吸烟、饮酒。痊愈32例(25%),好转50例(39.06%),未愈22例(17.19%),死亡24例(18.75%)。结论:高血压病、脑血管畸形是青年脑出血的主要病因,临床症状表现较重,大多数患者预后良好。

  20. Contemporary diagnosis of venous malformation

    Directory of Open Access Journals (Sweden)

    Lee BB

    2013-11-01

    Full Text Available BB Lee,1 I Baumgartner21Department of Surgery, George Washington University, Washington, DC, USA; 2Swiss Cardiovascular Center, University Hospital Bern, Bern, SwitzerlandAbstract: Venous malformation is a congenital vascular malformation resulting from defective development during various stages of embryogenesis and selectively affecting the venous system. Depending on the embryologic stage when the developmental arrest occurred, the clinical presentation of venous malformation is extremely variable in location, extent, severity, natural progression, and hemodynamic impact. Extratruncular lesions occur in the earlier stages of embryonic life, and retain characteristics unique to mesenchymal cells (angioblasts, growing and proliferating when stimulated internally (eg, by menarche, pregnancy, and hormones or externally (eg, by trauma or surgery. These lesions also have a significant hemodynamic impact on the venous system involved, in addition to the risk of localized intravascular coagulopathy. However, truncal lesions, as defective developments along the late stage, no longer carry the risk of proliferation and recurrence due to lack of mesenchymal characteristics. Although, they often have serious hemodynamic consequences due to direct involvement of the main vein trunk. Therefore, a thorough clinical history and careful physical examination should be followed by an appropriate combination of noninvasive and less invasive tests (eg, Doppler ultrasonography, magnetic resonance imaging, computed tomography to confirm the clinical impression as well as to define the extent and severity of the venous malformation. Invasive tests, eg, phlebography or angiography, are seldom needed for the diagnosis per se. Additional evaluation for coagulation abnormalities, eg, D-dimer and fibrinogen levels, is generally recommended, especially for the treatment of surgery and endovascular candidates with extensive lesions to assess the localized intravascular

  1. Cerebral Palsy

    Science.gov (United States)

    Cerebral palsy is a group of disorders that affect a person's ability to move and to maintain balance ... do not get worse over time. People with cerebral palsy may have difficulty walking. They may also have ...

  2. Classification and diagnosis of ear malformations

    Directory of Open Access Journals (Sweden)

    Bartel-Friedrich, Sylva

    2007-01-01

    Full Text Available In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90% and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations or acquired in nature. Malformations can affect the outer ear (pinna and external auditory canal, EAC, middle ear and inner ear, not infrequently in combination. Formal classification is advisable in order to be able to predict the prognosis and compare treatment schedules. Various classifications have been proposed: pinna and EAC malformations according to Weerda [1], middle ear malformations according to Kösling [2], and inner ear malformations according to Jackler [3], [4], to Marangos [5] and to Sennaroglu [6]. Additionally, we describe Altmann’s classification of atresia auris congenita [7] and the Siegert-Mayer-Weerda score [8] for EAC and middle ear malformations, systems of great practicability that are in widespread clinical use. The diagnostic steps include clinical examination, audiological testing, genetic analysis and, especially, CT and MRI. These imaging methods are most usefully employed in combination. Precise description of the malformations by means of CT and MRI is indispensable for the planning and successful outcome of operative ear reconstruction and rehabilitation procedures, including cochlear implantation.

  3. Flow Field Characterization Inside an Arteriovenous Graft-to-Vein Anastomosis Under Pulsatile Flow Conditions

    Science.gov (United States)

    2007-11-02

    1 FLOW FIELD CHARACTERIZATION INSIDE AN ARTERIOVENOUS GRAFT- TO-VEIN ANASTOMOSIS UNDER PULSATILE FLOW CONDITIONS Nurullah Arslan1, Francis Loth2...the relationship between the distribution of turbulence intensity and the localization of stenoses inside the venous anastomosis of arteriovenous (A...found to be greatest downstream of the anastomosis . KEYWORDS: Arteriovenous graft, dialysis, turbulence, stenosis I. INTRODUCTION

  4. Congenital malformations and damage in early infancy of the central nervous system; Fehlbildungen und fruehkindliche Schaedigungen des ZNS

    Energy Technology Data Exchange (ETDEWEB)

    Jansen, O. [Universitaetsklinikum Schleswig-Holstein, Kiel (Germany). Sektion Neuroradiologi; Stephani, U. (eds.) [Universitaetsklinikum Schleswig-Holstein, Kiel (Germany). Universitaetskinderklinik - Klinik fuer Neuropaediatrie

    2007-07-01

    Congenital malformations and cerebral damage in early infancy cause complex morphological and clinical changes. Modern imaging techniques, and especially NMR, have provided deeper knowledge of these diseases in the past few years. Based on the neuroradiological findings, the book presents a complete picture of congenital malformations of the central nervous systems and cerebral damage in early infancy; it describes the underlying pathomechanisms, clinical symptoms and therapies. Neurologists and neuropaediatricians are enabled to diagnose malformations correctly and to develop optimal therapy strategies in cooperation with other medical disciplines. Neuroradiologists and radiologists, on the other hand, will find a manual for correct interpretation and differential diagnosis of their findings and a guide for interpreting the findings and deciding further therapeutic or diagnostic interventions. (orig.)

  5. Variants in CUL4B are Associated with Cerebral Malformations

    NARCIS (Netherlands)

    Vulto-van Silfhout, Anneke T.; Nakagawa, Tadashi; Bahi-Buisson, Nadia; Haas, Stefan A.; Hu, Hao; Bienek, Melanie; Vissers, Lisenka E. L. M.; Gilissen, Christian; Tzschach, Andreas; Busche, Andreas; Muesebeck, Joerg; Rump, Patrick; Mathijssen, Inge B.; Avela, Kristiina; Somer, Mirja; Doagu, Fatma; Philips, Anju K.; Rauch, Anita; Baumer, Alessandra; Voesenek, Krysta; Poirier, Karine; Vigneron, Jacqueline; Amram, Daniel; Odent, Sylvie; Nawara, Magdalena; Obersztyn, Ewa; Lenart, Jacek; Charzewska, Agnieszka; Lebrun, Nicolas; Fischer, Ute; Nillesen, Willy M.; Yntema, Helger G.; Jarvela, Irma; Ropers, Hans-Hilger; de Vries, Bert B. A.; Brunner, Han G.; van Bokhoven, Hans; Raymond, F. Lucy; Willemsen, Michel A. A. P.; Chelly, Jamel; Xiong, Yue; Barkovich, A. James; Kalscheuer, Vera M.; Kleefstra, Tjitske; de Brouwer, Arjan P. M.

    2015-01-01

    Variants in cullin 4B (CUL4B) are a known cause of syndromic X-linked intellectual disability. Here, we describe an additional 25 patients from 11 families with variants in CUL4B. We identified nine different novel variants in these families and confirmed the pathogenicity of all nontruncating varia

  6. Variants in CUL4B are associated with cerebral malformations

    NARCIS (Netherlands)

    Vulto-van Silfhout, A.T.; Nakagawa, T.; Bahi-Buisson, N.; Haas, S.A.; Hu, H; Bienek, M.; Vissers, L.E.L.M.; Gilissen, C.F.H.A.; Tzschach, A.; Busche, A.; Musebeck, J.; Rump, P.; Mathijssen, I.B.; Avela, K.; Somer, M.; Doagu, F.; Philips, A.K.; Rauch, A.; Baumer, A.; Voesenek, K.E.J.; Poirier, K.; Vigneron, J.; Amram, D.; Odent, S.; Nawara, M.; Obersztyn, E.; Lenart, J.; Charzewska, A.; Lebrun, N.; Fischer, U.; Nillesen, W.M.; Yntema, H.G.; Jarvela, I.; Ropers, H.H.; Vries, B. de; Brunner, H.G.; Bokhoven, H. van; Raymond, F.L.; Willemsen, M.A.A.P.; Chelly, J.; Xiong, Y.; Barkovich, A.J.; Kalscheuer, V.M.; Kleefstra, T.; Brouwer, A.P.M. de

    2015-01-01

    Variants in cullin 4B (CUL4B) are a known cause of syndromic X-linked intellectual disability. Here, we describe an additional 25 patients from 11 families with variants in CUL4B. We identified nine different novel variants in these families and confirmed the pathogenicity of all nontruncating varia

  7. Cerebral malaria Malaria cerebral

    Directory of Open Access Journals (Sweden)

    Silvia Blair Trujillo

    2003-03-01

    Full Text Available Is the most common complication of P. falciparum malaria; nearly 90% of people who have suffered CM can recover without neurological problems. Currently there are four hypotheses that explain pathogenesis of CM: cytoadherence and sequestering of parasitized red blood cells to cerebral capillaries; rosette formation and parasitized red blood cells agglutination; production of cytokines and activation of second messengers and opening of the blood-brain barrier. However the main question remains to be answered; how the host-parasite interaction in the vascular space interferes transiently with cerebral function? Recently, the beta amyloid precursor peptide has been employed as marker of neural injury in CM. It is expected that the beta amyloid precursor peptide will help to understand the pathogenesis of CM in complicated patients of endemic areas of Colombia. La malaria Cerebral (MC es la complicación más frecuente de la malaria por P. falciparum; aproximadamente el 90% de las personas que la han padecido se recuperan completamente sin secuelas neurológicas. Aún no se conoce con claridad su patogénesis pero se han postulado cuatro hipótesis o mecanismos posibles: 1 citoadherencia y secuestro de glóbulos rojos parasitados en la microvasculatura cerebral; 2 formación de rosetas y aglutinación de glóbulos rojos parasitados; 3 producción de citoquinas y activación de segundos mensajeros y, 4 apertura de la barrera hematoencefálica. Sin embargo, queda un interrogante sin resolver aún: ¿qué proceso se lleva a cabo para que el parásito, desde el espacio microvascular, pueda interferir transitoriamente con la función cerebral? Recientemente se ha utilizado el precursor de la proteína b-Amiloide como un marcador de daño neuronal en MC; este precursor será de gran ayuda en futuras investigaciones realizadas en nuestro medio que aporten información para comprender la patogénesis de la MC.

  8. Magnetic Resonance Perfusion Imaging in Malformations of Cortical Development

    Energy Technology Data Exchange (ETDEWEB)

    Widjaja, ED.; Wilkinson, I.D.; Griffiths, P.D. [Academic Section of Radiolog y, Univ. of Sheffield, Sheffield (United Kingdom)

    2007-10-15

    Background: Malformations of cortical development vary in neuronal maturity and level of functioning. Purpose: To characterize regional relative cerebral blood volume (rCBV) and difference in first moment transit time (TTfm) in polymicrogyria and cortical tubers using magnetic resonance (MR) perfusion imaging. Material and Methods: MR imaging and dynamic T2*-weighted MR perfusion imaging were performed in 13 patients with tuberous sclerosis complex, 10 with polymicrogyria, and 18 controls with developmental delay but no macroscopic brain abnormality. Regions of interest were placed in cortical tubers or polymicrogyric cortex and in the contralateral normal-appearing side in patients with malformations. In 'control' subjects, regions of interest were placed in the frontal and parietal lobes in both hemispheres. The rCBV and TTfm of the tuber/contralateral side (rCBVRTSC and TTFMTSC) as well as those of the polymicrogyria/contralateral side (rCBVRPMG and TTFMPMG) were assessed. The right-to-left asymmetry of rCBV and TTfm in the control group was also assessed (rCBVRControls and TTFMControls). Results: There was no significant asymmetry between right and left rCBV or TTfm (P>0.05) in controls. There was significant reduction in rCBVRTSC compared to rCBVRControls (P<0.05), but no significant difference in TTFMTSC compared to TTFMControls (P>0.05). There were no significant differences between rCBVRPMG and rCBVRControls (P>0.05) or TTFMPMG and TTFMControls (P>0.05). Conclusion: Our findings imply that cerebral blood volume of polymicrogyria is similar to normal cortex, but there is reduced cerebral blood volume in cortical tubers. The lower rCBV ratio of cortical tubers may be related to known differences in pathogenetic timing of the underlying abnormalities during brain development or the presence of gliosis.

  9. Unusual MRI findings of dural arteriovenous fistula: Isolated perfusion lesions mimicking TIA

    Directory of Open Access Journals (Sweden)

    Kim Yong-Won

    2012-08-01

    Full Text Available Abstract Background The diagnosis of transient ischemic attack (TIA based on clinical history and objective findings, even including multiparametric MRI, can be misleading. We report two patients who presented with TIA-like deficits with isolated perfusion lesions in corresponding areas but were finally diagnosed as transient neurological symptoms associated with dural arteriovenous fistula (dAVF. Case presentation Two patients presented with transient focal neurological symptoms lasting less than one hour. An isolated perfusion deficit with no diffusion change in the clinically relevant area was shown on brain MRI, indicating transient ischemia as the most plausible cause of neurological symptoms. However, cerebral angiography let to diagnosis of dAVF in both cases. Intracerebral hemorrhage occurred after the initial diagnosis of TIA in one patient, and the small area of perfusion abnormality accompanied by the enlarged cortical vein in the other case helped to identify the dAVF through the further investigation. The pattern of perfusion-weighted imaging in both cases revealed increase of mean transit time and relative cerebral blood volume denoting the venous congestion in a clinically corresponding area. Conclusion Reported cases are uncommon clinical presentation of a dAVF, which can be misdiagnosed as TIA on clinical grounds. In rare cases, the isolated perfusion deficits could be attributable to venous congestion, despite the similar pattern of clinical presentation, such as with TIA.

  10. CLINICAL STUDY OF ANORECTAL MALFORMATIONS

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    Umesh

    2015-01-01

    Full Text Available BACKGROUND: A norectal malformations are relatively encountered anomalies. Presentations may vary from mild to severe and bowel control is t he main concern. AIM: To study the modes of presentation , types of anomalies , associated anomalies , reliability of clinical signs and radiological investigations in the diagnosis and the prognosis and continence in the post - operative in relation to type of anomaly and associated anomaly (s. MATERIAL AND METHODS: 50 cases of anorectal malformations admit ted to Department of Paediatric Surgery , in Medical Coll ege and Research Institute , were included in the study. Data related to the objectives of the study were collected. RESULTS: Commonest mode of presentation was failure to pass meconium 50%. 59% of mal es had high anomalies , while 53% females had intermediate anomalies. The diagnosis of low anomaly was made clinically , while high and intermediate anomalies needed further investigations. Associated anomalies were noted in 46.6% of the cases. 71.42% of the se patients had either a high or intermediate ARM. All patients with high anomalies underwent a 3 stage procedure , while low anomalies underwent a single stage procedure followed by anal dilatations. Rectal mucosal prolapse (2 cases , wound infection (4 ca ses , stenosis (3 cases , retraction of neo anus (1 case was seen. All the patients with low anomalies had a good functional result post operatively , while 57% and 28% of patients with intermediate and high anomalies had good results. CONCLUSION : Anorectal malformations are common congenital anomalies. Males are more commonly affected (1.3:1. Low anomalies are the commonest lesions noted in both the sexes (36.67%. High anomalies are more frequent in males. Invertogram offer an accurate diagnosis for planning management in patients with anorectal malformations. Low anomalies have a better outcome following surgery. For intermediate and high anomalies a staged repair offers better results

  11. Embryological Consideration of Dural Arteriovenous Fistulas

    Science.gov (United States)

    TANAKA, Michihiro

    2016-01-01

    The topographical distribution of dural arteriovenous fistulas (DAVFs) was analyzed based on the embryological anatomy of the dural membrane. Sixty-six consecutive cases of intracranial and spinal DAVFs were analyzed based on the angiography, and each shunt point was identified according to the embryological bony structures. The area of dural membranes was categorized into three different groups: a ventral group located on the endochondral bone (VE group), a dorsal group located on the membranous bone (DM group) and a falcotentorial group (FT group) located in the falx cerebri, tentorium cerebelli, falx cerebelli, and diaphragm sellae. The FT group was designated when the dural membrane was formed only with the dura propria (meningeal layer of the dura mater) and not from the endosteal dura. Cavernous sinus, sigmoid sinus, and anterior condylar confluence was categorized to VE group, which had a female predominance, more benign clinical presentations, and a lower rate of cortical and spinal venous reflux. Transverse sinus, confluence, and superior sagittal sinus belonged to the DM group. Olfactory groove, falx, tent of the cerebellum, and nerve sleeve of spinal cord were categorized to the FT group, which presented later in life and which had a male predominance, more aggressive clinical presentations, and significant cortical and spinal venous reflux. The DAVFs was associated with the layers of the dural membrane characterized by the two different embryological bony structures. The FT group was formed only with the dura propria as an independent risk factor for aggressive clinical course and hemorrhage of DAVFs. PMID:27250699

  12. A Patient with Recurrent Arteriovenous Graft Thrombosis.

    Science.gov (United States)

    Allon, Michael

    2015-12-01

    Arteriovenous grafts (AVGs) are prone to frequent thrombosis that is superimposed on underlying hemodynamically significant stenosis, most commonly at the graft-vein anastomosis. There has been great interest in detecting AVG stenosis in a timely fashion and performing preemptive angioplasty, in the belief that this will prevent AVG thrombosis. Three surveillance methods (static dialysis venous pressure, flow monitoring, and duplex ultrasound) can detect AVG stenosis. Whereas observational studies have reported that surveillance with preemptive angioplasty substantially reduces AVG thrombosis, randomized clinical trials have failed to confirm such a benefit. There is a high frequency of early AVG restenosis after angioplasty caused by aggressive neointimal hyperplasia resulting from vascular injury. Stent grafts prevent AVG restenosis better than balloon angioplasty, but they do not prevent AVG thrombosis. Several pharmacologic interventions to prevent AVG failure have been evaluated in randomized clinical trials. Anticoagulation or aspirin plus clopidogrel do not prevent AVG thrombosis, but increase hemorrhagic events. Treatment of hyperhomocysteinemia does not prevent AVG thrombosis. Dipyridamole plus aspirin modestly decreases AVG stenosis or thrombosis. Fish oil substantially decreases the frequency of AVG stenosis and thrombosis. In patients who have exhausted all options for vascular access in the upper extremities, thigh AVGs are a superior option to tunneled internal jugular vein central vein catheters (CVCs). An immediate-use AVG is a reasonable option in patients with recurrent CVC dysfunction or infection. Tunneled femoral CVCs have much worse survival than internal jugular CVCs.

  13. Large spinal intraosseous arteriovenous fistula: case report.

    Science.gov (United States)

    Imajo, Yasuaki; Kanchiku, Tsukasa; Yoshida, Yuichiro; Nishida, Norihiro; Taguchi, Toshihiko

    2015-04-01

    Here the authors report the case of a fresh vertebral body fracture with a large spinal intraosseous arteriovenous fistula (AVF). A 74-year-old woman started to experience low-back pain following a rear-end car collision. Plain radiography showed diffuse idiopathic skeletal hyperostosis (DISH). Sagittal CT sections revealed a transverse fracture of the L-4 vertebral body with a bone defect. Sagittal fat-suppressed T2-weighted MRI revealed a flow void in the fractured vertebra. Spinal angiography revealed an intraosseous AVF with a feeder from the right L-4 segmental artery. A fresh fracture of the L-4 vertebral body with a spinal intraosseous AVF was diagnosed. Observation of a flow void in the vertebral body on fat-suppressed T2-weighted MRI was important for the diagnosis of the spinal intraosseous AVF. Because conservative treatment was ineffective, surgery was undertaken. The day before surgery, embolization through the right L-4 segmental artery was performed using 2 coils to achieve AVF closure. Posterolateral fusion with instrumentation at the T12-S2 vertebral levels was performed without L-4 vertebroplasty. The spinal intraosseous AVF had disappeared after 4 months. At 24 months after surgery, the bone defect was completely replaced by bone and the patient experienced no limitations in daily activities. Given their experience with the present case, the authors believe that performing vertebroplasty or anterior reconstruction may not be necessary in treating spinal intraosseous AVF.

  14. Congenital Chiari malformations: A review

    Directory of Open Access Journals (Sweden)

    Vannemreddy Prasad

    2010-01-01

    Full Text Available Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available.

  15. Pediatric neuroradiology: Cerebral and cranial diseases

    Energy Technology Data Exchange (ETDEWEB)

    Diebler, C.; Dulac, O.

    1987-01-01

    In this book, a neuroradiologist and a neuropediatrician have combined forces to provide the widest possible knowledge in investigating cranial and cerebral disorders in infancy and childhood. Based on more than 20,000 pediatric CT examinations, with a follow-up time often exceeding ten years, the book aims to bridge interdisciplinary gaps and help radiologists, pediatricians and neurosurgeons solve the various problems of pediatric neuroradiology that frequently confront them. For each disease, the etiology, clinical manifestation, pathological lesions and radiological presentations are discussed, supported by extensive illustrations. Malformative, vascular, traumatic, tumoral, infectious and metabolic diseases are reviewed. Miscellaneous conditions presenting particular symptoms or syndromes are also studied, such as hydrocephalus and neurological complications of leukemia. Contents: Cerebral and cranial malformations; neurocutaneous syndromes; inherited metabolic diseases; infectious diseases - vascular disorders; intracranial tumors; cranial trauma - miscellaneous and subject index.

  16. Urokinase treatment for arteriovenous fistulae declotting in dialyzed patients.

    Science.gov (United States)

    Mangiarotti, G; Canavese, C; Thea, A; Segoloni, G P; Stratta, P; Salomone, M; Vercellone, A

    1984-01-01

    Urokinase treatment, previously employed with success in the declotting of deep venous thrombosis and arteriovenous shunts in patients undergoing regular dialytic treatment (RDT), was used in 23 cases of arteriovenous fistula thrombotic occlusion in 18 RDT patients. The treatment was successful in 65.2% of the cases without any negative side effects, except 1 case which may have developed a pulmonary embolism. Patients with severe hypofibrinolysis may need larger doses or may have a recurrence of the thrombotic episode. All therapeutic failures correlated with the presence of fibrosis or sclerosis.

  17. Volume Flow in Arteriovenous Fistulas Using Vector Velocity Ultrasound

    DEFF Research Database (Denmark)

    Hansen, Peter Møller; Olesen, Jacob Bjerring; Pihl, Michael Johannes;

    2014-01-01

    Volume flow in arteriovenous fistulas for hemodialysis was measured using the angle-independent ultrasound technique Vector Flow Imaging and compared with flow measurements using the ultrasound dilution technique during dialysis. Using an UltraView 800 ultrasound scanner (BK Medical, Herlev......, Denmark) with a linear transducer, 20 arteriovenous fistulas were scanned directly on the most superficial part of the fistula just before dialysis. Vector Flow Imaging volume flow was estimated with two different approaches, using the maximum and the average flow velocities detected in the fistula. Flow...

  18. Congenital malformations of the skull and meninges.

    Science.gov (United States)

    Kanev, Paul M

    2007-02-01

    The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

  19. Spontaneous ileal perforation complicating low anorectal malformation

    Directory of Open Access Journals (Sweden)

    TiJesuni Olatunji

    2015-01-01

    Full Text Available Anorectal malformation is a common anomaly in neonates. Although colorectal perforations have been reported as a complication, ileal perforation is rarely encountered. This is a report of a 2-day-old boy presenting with a low anorectal malformation, complicated with ileal perforation, necessitating laparotomy and ileal repair. Anoplasty was done for the low anomaly. Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication.

  20. Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Hannah E. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Moore, Kevin R. [University of Utah School of Medicine, Department of Radiology, Salt Lake City, UT (United States); Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2014-11-15

    Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords ''Chiari'' and ''Chiari 1'' to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in

  1. Skeletal malformations in fetuses with Meckel syndrome

    DEFF Research Database (Denmark)

    Kjaer, K W; Fischer Hansen, B; Keeling, J W;

    1999-01-01

    four types, based on the number and morphology of metacarpals and metatarsals. In the individual fetus there was more often similarity in the pattern of malformation in the two hands or in the two feet than there was between the pattern of malformation seen in the hands and that seen in the feet. Only...... one foot was normal. Malformations of the cranial base (the basilar part of the occipital bone or the postsphenoid bone) occurred in five cases, and the vertebral bodies in the lumbar region of the spine were malformed (cleft) in three cases. It is proposed that a skeletal analysis be included...

  2. Chiari II malformation. Pt. 4

    Energy Technology Data Exchange (ETDEWEB)

    Naidich, T.P.; McLone, D.G.; Fulling, K.H.

    1983-08-01

    Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary ''kinking''; anterior displacement and sequential sagittal compression of each protrusion by the protrusions posterior to it; compression of all protrusions by the posterior lip of foramen magnum and the posterior arch of C1; and associated cervical hydromyelia, cervical diastematomyelia, and cervical arachnoid cysts.

  3. Four cases of trisomy 18 syndrome with limb reduction malformations.

    OpenAIRE

    Christianson, A L; Nelson, M. M.

    1984-01-01

    Limb reduction malformations of the arms are well documented in the trisomy 18 syndrome. Four cases of trisomy 18 syndrome with limb reduction malformations of the legs are described and compared with the upper limb malformations.

  4. A mathematical model of continuous arterio-venous hemodiafiltration (CAVHD)

    NARCIS (Netherlands)

    E. Akcahuseyin (Emin); H.H. Vincent (Jeroen); F.J. van Ittersum (Frans); W.A. van Duyl (Wim); M.A.D.H. Schalekamp (Maarten)

    1990-01-01

    textabstractAbstract Continuous arterio-venous hemodiafiltration (CAVHD) differs from conventional hemofiltration and dialysis by the interaction of convection and diffusion, the use of very low dialysate flow rates and by the deterioration of membrane conditions during the treatment. In order to st

  5. Radial Artery Approach to Salvage Nonmaturing Radiocephalic Arteriovenous Fistulas

    Energy Technology Data Exchange (ETDEWEB)

    Hsieh, Mu-Yang; Lin, Lin; Tsai, Kuei-Chin; Wu, Chih-Cheng, E-mail: chihchengwumd@gmail.com [National Taiwan University Hospital, Department of Cardiology (China)

    2013-08-01

    PurposeTo evaluate the usefulness of an approach through the radial artery distal to the arteriovenous anastomosis for salvaging nonmaturing radiocephalic arteriovenous fistulas.MethodsProcedures that fulfilled the following criteria were retrospectively reviewed: (1) autogenous radiocephalic fistulas, (2) fistulas less than 3 months old, (3) distal radial artery approach for salvage. From 2005 to 2011, a total of 51 patients fulfilling the above criteria were enrolled. Outcome variables were obtained from angiographic, clinical and hemodialysis records, including the success, complication, and primary and secondary patency rates.ResultsThe overall anatomical and clinical success rates for the distal radial artery approach were 96 and 94 %, respectively. The average procedure time was 36 {+-} 19 min. Six patients (12 %) experienced minor complications as a result of extravasations. No arterial complication or puncture site complication was noted. The postinterventional 6-month primary patency rate was 51 %, and the 6-month secondary patency rate was 90 %. When the patients were divided into a stenosed group (20 patients) and an occluded group (31 patients), there were no differences in the success rate, complication rate, or primary and secondary patency rates.ConclusionAn approach through the radial artery distal to the arteriovenous anastomosis is an effective and safe alternative for the salvage of nonmaturing radiocephalic arteriovenous fistulas, even for occluded fistulas.

  6. Arteriovenous shunt graft ulceration with sinus and graft epithelialization

    Directory of Open Access Journals (Sweden)

    Pooja Singhal

    2015-03-01

    Full Text Available Arteriovenous fistula and grafts are used as access sites for patients with chronic kidney disease and are prone for complications. Stent grafts are used to treat access site complications. We report a rare and unusual finding of epithelialization of the sinus tract and the lumen of a polytetrafluoroethylene graft, following ulceration of the overlying skin.

  7. Effects of arteriovenous fistulas on cardiac oxygen supply and demand

    NARCIS (Netherlands)

    Bos, W.J.W.; Zietse, R.; Wesseling, K.H.; Westerhof, N.

    1999-01-01

    Background. Arteriovenous (AV) fistulas used for hemodialysis access may affect cardiac load by increasing the preload while decreasing the afterload. In dogs, AV fistulas have also been shown to affect coronary perfusion negatively. We investigated the net effect of AV fistulas on cardiac oxygen su

  8. [Right renal arteriovenous fistula after nephrectomy with streptococcal endarteritis].

    Science.gov (United States)

    Natali, J; Emerit, J; Reynier, P; Maraval, M

    1975-01-18

    The authors add a new case, to the 41 already published, of arterio-venous fistula of the renal pedicle after nephrectomy, with the peculiarity of its presentation as a prolonged fever resulting from streptococcal bacterial endarteritis at the site of the fistula (3rd case in the literature). Surgical treatment in association with massive and prolonged antibiotic therapy resulted in recovery.

  9. Venous aneurysm complicating arteriovenous fistula access and matrix metalloproteinases

    Directory of Open Access Journals (Sweden)

    Serra Raffaele

    2015-01-01

    Full Text Available Introduction: An arteriovenous fistula (AVF for placed for hemodialysis may be burdened by one particular complication-the formation of a venous aneurysm. It has been shown that matrix metalloproteinases (MMPs and neutrophil gelatinase-associated lipocalin (NGAL could represent markers of disease in both venous and arterial vessels.

  10. [Spondylodiscitis after embolization of an extramedullary intraspinal arteriovenous fistula].

    Science.gov (United States)

    Baudrillard, J C; Toubas, O; Lerais, J M; Auquier, F; Gatfosse, M; Bernard, M H

    1985-04-01

    The authors report a case of spondylitis Th11-Th12 occurred 1 month after embolization of an intraspinal extramedullary arteriovenous fistulae; this fistulae was fed by 11th left intercostal artery. The infecting organism isolated from the affected intervertebral disc was streptococcus sanguis a common agent of dental abscess.

  11. Traumatic basilar pseudoaneurysm with a basilar-cavernous arteriovenous fistula

    Energy Technology Data Exchange (ETDEWEB)

    Connor, S.E.J.; Deasy, N.P.; Jeffree, M.A. [Dept. of Neuroradiology, King' s Coll. Hospital, London (United Kingdom); Martin, A.J.; Strong, A.J. [Dept. of Neurosurgery, King' s Coll. Hospital, London (United Kingdom)

    2001-03-01

    A traumatic pseudoaneurysm of the basilar artery with a basilar-cavernous sinus arteriovenous fistula was diagnosed in a 12-year-old girl using CT, MRI and angiography. It was successfully treated by coil embolisation. We speculate on the mode of formation of this rare traumatic lesion. (orig.)

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

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    Sudhir Babu Karri

    2016-01-01

    Full Text Available 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.

  14. Efficacy and safety of embolization in iatrogenic traumatic uterine vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Z.; Chen, J.; Shi, H.; Zhou, K.; Sun, H. [Department of Radiology, Peking Union Medical College Hospital, Beijing (China); Li, X., E-mail: pumch005@sina.com [Department of Radiology, Peking Union Medical College Hospital, Beijing (China); Pan, J.; Zhang, X.; Liu, W.; Yang, N.; Jin, Z. [Department of Radiology, Peking Union Medical College Hospital, Beijing (China)

    2012-06-15

    Aim: To retrospectively evaluate the efficacy of embolotherapy in patients with iatrogenic traumatic uterine arteriovenous malformations (AVMs). Materials and methods: A retrospective review of all patients who underwent uterine arterial embolization in Peking Union Medical College Hospital between January 2000 and December 2010 was performed. Forty-two patients were diagnosed with a uterine vascular malformation. All patients had obstetric manipulations before. Serial beta-human chorionic gonadotropin ({beta}-HCG) levels were measured to exclude gestational trophoblastic neoplasia. All patients underwent transcatheter embolization of bilateral uterine arteries. The complications, control of haemorrhage, and outcome of subsequent pregnancies were assessed. Results: A total of 49 embolization procedures were performed in 42 patients. Seven patients required repeated embolizations for recurrence of bleeding. The technical success rate of embolization was 100%. Bleeding was controlled in 35 of 42 patients (83%) after the first embolization procedures, and bleeding was controlled in another two patients who underwent repeat embolization at a median follow-up of 29 months (range 3 months to 5 years). The overall clinical success rate was 88% (37/42). Thirteen patients subsequently became pregnant and eight of 13 patients had uneventful intrauterine pregnancies carried to term. Seven patients had post-embolization syndrome and no other complication occurred. Conclusion: Percutaneous embolotherapy is a safe and effective treatment for traumatic AVMs. Future pregnancy is still possible after embolization.

  15. Subfascial involvement in glomuvenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Shaikh, Raja; Alomari, Ahmad I.; Chaudry, Gulraiz [Boston Children' s Hospital, Division of Interventional Radiology, Boston, MA (United States); Mulliken, John B. [Boston Children' s Hospital, Division of Plastic Surgery, Boston, MA (United States); Fishman, Steven J. [Boston Children' s Hospital, Department of Surgery, Boston, MA (United States); Kozakewich, Harry P.W. [Boston Children' s Hospital, Department of Pathology, Boston, MA (United States)

    2014-07-15

    Glomuvenous malformation (GVM) is an inherited autosomal dominant trait. The lesions, which appear as bluish nodules or plaque-like cutaneous elevations, are usually tender and more firm than sporadic venous malformations. Conventionally, the lesions are thought to be limited to the cutaneous and subcutaneous tissue planes. The objective was to characterize the depth of involvement of GVM lesions. Magnetic resonance imaging (MRI) findings in GVM were retrospectively evaluated by two radiologists. The signal characteristics, tissue distribution, pattern of contrast enhancement of the lesions in GVM were documented. Thirty patients (19 female) aged 1-35 years (mean 18 years) were diagnosed with GVM based on clinical features (n = 20) and/or histopathological findings (n = 10). The lesions were present in the lower extremity (n = 15), upper extremity (n = 6), cervico-facial region (n = 6), pelvis (n = 2), and chest wall (n = 1). All patients had skin and subcutaneous lesions. Fifty percent of the patients (n = 15) demonstrated subfascial intramuscular (n = 15), intra-osseous (n = 1), and intra-articular involvement (n = 1). Contrary to the conventional belief that GVMs are generally limited to the skin and subcutaneous tissue, deep subfascial extension of the lesions is common. (orig.)

  16. Vascular tumors and malformations of the colon

    Institute of Scientific and Technical Information of China (English)

    Israel Fernandez-Pineda

    2009-01-01

    The term "hemangioma" refers to the common tumor of infancy that exhibits rapid postnatal growth and slow regression during childhood. It may cause confusion with venous malformations that are often incorrectly called "cavernous hemangioma". Venous malformations comprise abnormally formed channels that are lined by quiescent endothelium. Accurate diagnosis is required for selecting the appropriate treatment.

  17. Arteriovenous Relationships in the Pathogenesis of Encephalopathy

    Directory of Open Access Journals (Sweden)

    Alexander G. Kruglov

    2015-09-01

    Full Text Available The study aims at investigating the disturbance in intra- and extracranial interaction of arterial and venous vessels in stable arterial hypertension (SAH and building a model of vascular relationships in the system: inflow – the exchange field of cerebral blood flow – outflow. Baseline data were obtained by catheterization through a probe that was wedged in the superior bulb of the internal jugular vein, where the hemodynamic and biochemical parameters of cerebral blood flow were obtained. Arterial blood was collected from the thoracic aorta. We performed the correlation and factor analyses of the relationship between the parameters of inflow and outflow to the skull in SAH patients compared with those in the control group. The identified differences led to the following conclusions: There is a loss of homeostatic control for the hemodynamic (extra- and intracerebral and biochemical regulation in SAH; the high-energy processes of the aortic chamber (systolic and pulse pressure spread to the bloodstream of the brain; the damping function of carotid siphons is impaired; cerebral venous stasis is formed; increased pressure in the microvascular venous network of the brain is defined; and a loss of the homeostatic control of the rheological properties of blood is defined. The loss of extracranial regulation of intracranial venous pressure in SAH leads to venous plethora of the intracerebral vessels, increasing the “booster" pressure in the microvasculature, and circulatory hypoxia of brain tissues. The consequences of these changes are metabolic and hemodynamic disturbances in energy supply for activated neurons, as well as circulatory hypoxia resulting in disturbances of the regulatory function of the nervous system and mental activity, and the development of hypertonic angioencephalopathy.

  18. Arteriovenous Fistula Complicated by Popliteal Venous Access for Endovascular Thrombolytic Therapy of Deep Vein Thrombosis

    Energy Technology Data Exchange (ETDEWEB)

    Byun, Sung Su; Kim, Jeong Ho; Park, Chul Hi; Hwang, Hee Young; Kim, Hyung SiK [Gacheon University Gil Medical Center, Gacheon (Korea, Republic of); Jeon, Young Sun; Kim, Won Hong [Inha University College of Medicine, Incheon (Korea, Republic of)

    2008-10-15

    We report a case of an iatrogenic arteriovenous fistula complicated by catheter- directed thrombolytic therapy in a patient with acute deep vein thrombosis of a lower extremity. To the best of our knowledge, this is the first report of an arteriovenous fistula between the sural artery and popliteal vein in that situation. As the vessels have a close anatomical relationship, the arteriovenous fistula seems to be a potential complication after endovascular thrombolytic therapy of acute deep vein thrombosis.

  19. Characterization of Live Birth with Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Carlos Acosta Batista

    2015-06-01

    Full Text Available Background: the congenital malformations constitute the first cause of infantile death in developed countries, as well as the second cause of death in Cuba, in younger children of an elderly year. Objective: characterizing the live birth newborns with congenital malformations at Marianao municipality during the year 2011. Methods: descriptive, cross-section study of 30- live birth with congenital malformations at Marianao municipality in Havana, during the year 2011. Some of analyzed variables were: sex, affected system, congenital malformation, type of malformation, severity, birth weight, gestational age, prenatal diagnosis, family history of congenital malformation, maternal age, among others. Results: the masculine sex was the more affected, with 18 cases that represented the 60 %. The Polydactyl was the malformation further frequent, with 23.3 %, followed by the pre-aural appendix, with 10 %. The 20 % was born pre-term and only in the 10 % of the cases was obtained a positive result in the tests of prenatal diagnosis. Conclusions: the live birth with MC were characterized to be males with isolated MC and less severe, full term, normopeso, without family history of the aforementioned affection and with a negative prenatal diagnosis. The majority of mothers belonged to 20-35's age group years, they suffered from hypertension and during pregnancy, the principal diseases that they presented were the sepsis and anemia.

  20. Dependence of transcutaneous oxygen tension on local arteriovenous pressure gradient in normal subjects.

    Science.gov (United States)

    Wyss, C R; Matsen, F A; King, R V; Simmons, C W; Burgess, E M

    1981-05-01

    1. We studied the relationship between transcutaneous oxygen tension at the foot and local arteriovenous pressure difference in 15 normal men and women; arteriovenous pressure difference was varied by changing the height of the foot with respect to the heart and by applying external pressure to the foot. 2. Control transcutaneous oxygen tension was 67 +/- 9 SD mmHg (8.9 +/- 1.2 kPa) at a control arteriovenous pressure difference of 80 +/- 6 SD mmHg (10.6 +/- 0.8 kPa). 3. In every subject transcutaneous oxygen tension fell non-linearly with a decrease in arteriovenous pressure difference; transcutaneous oxygen tension was relatively insensitive to changes in arteriovenous pressure difference when arteriovenous pressure difference was high, but always fell sharply to zero at some positive arteriovenous pressure difference [range 13-34 mmHg (1.7-4.5 kPa)]. 4. An analysis of the data indicated that transcutaneous oxygen tension varied with arteriovenous pressure difference approximately as the oxygen tension of cutaneous venous blood under the sensor varied (in the absence of changes in cutaneous vascular resistance and oxygen consumption). 5. This analysis was supported by studies in three subjects in whom the oxygen tension of superficial venous drainage from a warmed hand or foot was measured along with transcutaneous oxygen tension while arteriovenous pressure difference was varied.

  1. Outcome of Kidney Allografts in Recipients With a Femoral Arteriovenous Fistula: Report of Two Cases

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    Denise M.D. Özdemir-van Brunschot

    2016-09-01

    Full Text Available Two patients, who were on hemodialysis over a femoral arteriovenous fistula, were transplanted in our center. Despite adequate blood pressure, perfusion of the renal allograft remained poor after completion of the vascular anastomoses. Ligation of the femoral arteriovenous fistula (1.6 L/min led to adequate perfusion. Initial graft function was good. Although it remains unclear whether ischemia of a renal allograft is caused by venous hypertension or vascular steal due to a femoral arteriovenous fistula, it might be necessary to ligate a femoral arteriovenous fistula to obtain adequate graft perfusion.

  2. Superior cerebellar artery infarction in endovascular treatment for tentorial dural arteriovenous fistulas

    Energy Technology Data Exchange (ETDEWEB)

    Zhang Jingbo; Lv Xianli; Jiang Chuhan; Li Youxiang [Beijing Neurosurgical Institute and Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan, Xili, Chongwen, 100050, Beijing (China); Wu Zhongxue, E-mail: ttyyzjb@sina.co [Beijing Neurosurgical Institute and Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan, Xili, Chongwen, 100050, Beijing (China)

    2010-06-15

    Background: Superior cerebellar artery (SCA) syndrome shows ipsilateral cerebellar ataxia and Horner's syndrome, contralateral superficial sensory disturbance, as well as nystagmus toward the impaired side, vertigo, and nausea. Occasionally, unilateral lesions may produce bilateral hypogeusia and contralateral hypoacusia. Objective: To report 2 patients with unilateral lower midbrain ischemic lesions of the inferior colliculus level caused by transarterial embolization for tentorial dural arteriovenous fistulas (TDAVFs). Methods: Hospital records for 21 patients with TDAVFs mainly treated by endovascular techniques between 2005 and 2008 were reviewed. Two patients with MRI evidence of unilateral SCA territory infarction were investigated. Results: Of 21 patients, 2 treated transarterially with Onyx-18 (a nonahesive liquid embolic agent) developed infarctions in the territory of SCA. One patient had lateral SCA infarction characterized by ipsilateral gait ataxia, contralateral hemihypoesthesia, with additional ipsilateral ocular motor palsy and bilateral gustatory loss. And the other patient had medial SCA infarction characterized by ipsilateral ataxia contralateral hemihypoesthesia with additional contralateral hypoacusia. Conclusion: SCA infarction can be caused by transarterial injection of Onyx-18 via SCA or the posterior cerebral artery (PCA) for TDAVFs and additionally presented with gustatory loss and deafness, which is generally not a feature of the SCA syndrome.

  3. Medullary Venous Hypertension Secondary to a Petrous Apex Dural Arteriovenous Fistula: A Case Report

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    Meghan Murphy

    2012-11-01

    Full Text Available Background: Dural arteriovenous fistulae (dAVF are common intracranial vascular lesions typically becoming symptomatic with cortical venous hypertension and possible hemorrhage. Here, we present a case illustration of a petrous apex dAVF with marked medullary venous hypertension and a unique clinical presentation. Methods: Case report. Results: A 72-year-old female, whose clinical progression was significant for altered mental status and progressive weakness, presented with diplopia, right leg paresis, and ataxia. Magnetic resonance imaging revealed edema involving the medulla. On digital subtraction cerebral angiogram, the patient was found to have a petrous apex dAVF, Cognard type IV. Following treatment with Onyx embolization, her symptoms rapidly improved, with complete resolution of diplopia and drastic improvement of her ataxia. Conclusion: The importance of this case is in the presentation and deterioration of the clinical exam, resembling an acute ischemic event. Further, this case illustrates that dAVF may cause venous hypertension with rapid onset of focal neurologic symptoms not exclusive to cortical locations.

  4. Improvement of Depression after Treatment of Dural Arteriovenous Fistula: A Case Report and a Review

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    Minoru Nakagawa

    2012-01-01

    the treatment of a dural arteriovenous fistula in the left transverse-sigmoid sinus. A 46-year-old male had suffered from depression and was treated with antidepressants at a local hospital for four years. The patient was temporarily laid off due to his depression. Afterwards, he had Gerstmann's syndrome and came to our hospital. A DAVF in the left transverse-sigmoid sinus was demonstrated on the angiogram. The DAVF was successfully treated with endovascular surgery, coil embolization of the isolated diseased sinus through the mastoid emissary vein which was a draining vein from the fistula. After this treatment, his depression as well as Gerstmann's syndrome was improved and the quantity of the antidepressants decreased. The patient returned to work without any antidepressant two years after the treatment. DAVFs might be one of the causes of depression. It may be necessary to evaluate cerebral vessels in patients suffering from depression by using MRA or 3D-CTA even if there are not any abnormal findings on plain CT scans.

  5. Vasoespasmo cerebral

    Directory of Open Access Journals (Sweden)

    Antonio A. F. de Salles

    1987-09-01

    Full Text Available Vasoespasmo cerebral ocorre em patologias como enxaqueca, hemorragia subaracnóidea, trauma de crânio, após isquemia e/ou hipoxia. A fisiopatologia do vasoespasmo cerebral nestas patologias não está completamente desvendada. Neste artigo são analisados os fatores neuroquímicos e morfológicos responsáveis pelo controle circulatório cerebral. As alterações circulatórias que seguem a hemorragia subaracnóidea são utilizadas como exemplo. Conclui-se que fatores bioquímicos, fisiológicos e morfológicos são responsáveis pelas manifestações vasculares que ocorrem após a hemorragia subaracnóidea. Alternativas de tratamento do vasoespasmo cerebral são discutidas.

  6. Computed tomography of congenital brain malformations

    Energy Technology Data Exchange (ETDEWEB)

    Sarwar, M.

    1984-01-01

    This book is illustrated showing each condition. This book is designed to correlate the pathology of CNS malformations with their CT scan appearance, mainly on the axial images. The author has drawn upon his personal experience and the information gleaned from the literature dealing with the description of the CT scan findings of these malformations. The emphasis is on simplicity of description. Since a large degree of morphological variation exists in each entity, numerous illustrations (wherever applicable) are shown to depict those variations. When appropriate, deficiency of the CT scan in the evaluation of these CNS malformations also is indicated. A description of CNS embryology is included as well.

  7. Anterior commissure absence without callosal agenesis: a new brain malformation.

    Science.gov (United States)

    Mitchell, T N; Stevens, J M; Free, S L; Sander, J W; Shorvon, S D; Sisodiya, S M

    2002-04-23

    The authors report a novel human brain malformation characterized by the absence of the anterior commissure without callosal agenesis, but associated with gross unilateral panhemispheric malformation incorporating subependymal heterotopia, subcortical heterotopia, and gyral abnormalities including temporal malformation and polymicrogyria. In contrast, a normal anterior commissure was found in 125 control subjects and in 113 other subjects with a range of brain malformations.

  8. Cerebral Paragonimiasis.

    Science.gov (United States)

    Miyazaki, I

    1975-01-01

    The first case of cerebral paragonimiasis was reported by Otani in Japan in 1887. This was nine years after Kerbert's discovery of the fluke in the lungs of Bengal tigers and seven years after a human pulmonary infection by the fluke was demonstrated by Baelz and Manson. The first case was a 26-year-old man who had been suffering from cough and hemosputum for one year. The patient developed convulsive seizures with subsequent coma and died. The postmortem examination showed cystic lesions in the right frontal and occipital lobes. An adult fluke was found in the occipital lesion and another was seen in a gross specimen of normal brain tissue around the affected occipital lobe. Two years after Otani's discovery, at autopsy a 29-year-old man with a history of Jacksonian seizure was reported as having cerebral paragonimiasis. Some time later, however, it was confirmed that the case was actually cerebral schistosomiasis japonica. Subsequently, cases of cerebral paragonimiasis were reported. However, the majority of these cases were not confirmed histologically. It was pointed out that some of these early cases were probably not Paragonimus infection. After World War II, reviews as well as case reports were published. Recently, investigations have been reported from Korea, with a clinicla study on 62 cases of cerebral paragonimiasis seen at the Neurology Department of the National Medical Center, Seoul, between 1958 and 1964. In 1971 Higashi described a statistical study on 105 cases of cerebral paragonimiasis that had been treated surgically in Japan.

  9. Acquired arteriovenous fistula in a grizzly bear (Ursus arctos horribilis).

    Science.gov (United States)

    Tuttle, Allison D; MacLean, Robert A; Linder, Keith; Cullen, John M; Wolfe, Barbara A; Loomis, Michael

    2009-03-01

    A captive adult male grizzly bear (Ursus arctos horribilis) was evaluated due to multifocal wounds of the skin and subcutaneous tissues sustained as a result of trauma from another grizzly bear. On presentation, one lesion that was located in the perineal region seemed to be a deep puncture with purple tissue protruding from it. This perineal wound did not heal in the same manner or rate as did the other wounds. Twenty-five days after initial detection, substantial active hemorrhage from the lesion occurred and necessitated anesthesia for examination of the bear. The entire lesion was surgically excised, which later proved curative. An acquired arteriovenous fistula was diagnosed via histopathology. Arteriovenous fistulas can develop after traumatic injury and should be considered as a potential complication in bears with nonhealing wounds.

  10. Perfusion-weighted MRI of spinal dural arteriovenous fistula

    Energy Technology Data Exchange (ETDEWEB)

    Yanaka, K.; Matsumaru, Y.; Uemura, K.; Matsumura, A.; Nose, T. [Department of Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki (Japan); Anno, I. [Department of Radiology, Institute of Clinical Medicine, University of Tsukuba, Ibaraki (Japan)

    2003-10-01

    A 72-year-old woman was admitted with rapidly progressive paraplegia and sphincter disturbance. T2-weighted images of the thoracic spine showed intramedullary high signal with flow voids suggesting dilated medullary veins. Conventional spinal angiography demonstrated a dural arteriovenous fistula draining into perimedullary veins. Perfusion-weighted MRI demonstrated a prolonged mean transit time and increased blood volume in the high-signal area. The loss of normal perfusion gradient and venous hypertension and were thought to produce these differences. The time-to-peak was almost identical in the high-signal and isointense areas, although the bolus of contrast medium arrived earlier in the former. Arteriovenous shunting was thought to cause faster inflow. These changes may have resulted in increased blood volume in the spinal cord. The high signal has been attributed to oedema due to venous congestion, but there has been no histological confirmation. Perfusion MRI in this case supports this hypothesis. (orig.)

  11. Congenital arteriovenous fistula of the horseshoe kidney with multiple hemangiomas

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    Lazić Miodrag

    2012-01-01

    Full Text Available Introduction. Congenital renal arteriovenous fistulas (AVF are rare, especially if they are associated with other developmental renal anomalies. Case Outline. A 34-year-old female was hospitalized due to total painless hematuria and bladder tamponade. Excretory urography revealed a horseshoe kidney with normal morphology of pyelocaliceal system and ureters. Aortography and selective renovasography detected a cluster-like vascular formation with multiple arteriovenous fistulas (AVF. Due to a large AVF gauge and poor flow of the efferent vein to the inferior vena cava, a surgical procedure of two renal artery segmentary branches ligation and division was performed. During the operative procedure, the presence of multiple superficial renal hemangiomas was detected. Conclusion. Although selective arterial embolization represents the preferable treatment option, conventional surgery remains favorable alternative in selected cases with large and complex AVF.

  12. Arteriovenous fistula: An evidence based practice in nursing care

    OpenAIRE

    2013-01-01

    The arteriovenous fistula (AVF) is the most frequent form of vascular access for patients undergoing haemodialysis because it ensures good quality of dialysis and reduce haemodialysis mortality. For this reason, the nephrology nurse plays an important role in the appropriate care of fistula with a view to promoting the longevity and prevention of complications. Purpose: The purpose of this review was to investigate the role of Nephrology nurse in the appropriate care of fistula, promoting lon...

  13. Care of arteriovenous fistula by patients with chronic renal failure.

    OpenAIRE

    Monteiro Furtado, Angelina; Elisângela Teixeira Lima, Francisca

    2008-01-01

    This descriptive study aimed at identifying care of arteriovenous fistula (AVF) performed patients under hemodialysis. Twenty one hemodialysis patients were randomly selected in a clinic in Fortaleza, Ceara, Brazil:11 female and 11 male patients, age between 30 and 50 yearold, and most were single, retired, and had low education level. Semi-structured interview was applied. The following procedures were mentioned: cleaning the fistula arm, thrill palpation, avoiding lifting weight with AVF...

  14. Surgical management of multiple posttraumatic arteriovenous fistulas of femoral vessels.

    Science.gov (United States)

    Pokrovsky, A V; Shubin, A A; Kuntsevich, G I; Subbotin, V V; Suntsov, D S

    2008-01-01

    Presented herein are two case reports concerning surgical management of posttraumatic arteriovenous fistulas of femoral vessels. Case 1. A 45-year-old female patient attended with a history of a shotgun injury wound of her left femur and crus sustained when a girl of eight. She sought medical attention for a progressively deteriorating condition, accompanied by pain, and breathlessness dyspnea at rest. Detected were multiple fistulas between the deep femoral artery and superficial femoral artery and femoral vein. Management consisted in separation of the arteriovenous fistulas, followed by prosthetic repair of the deep femoral artery. Case 2. A 32-year-old male patient after an accidentally inflicted shotgun injury of the his left femur underwent within a time period of 3 year three vascular operations including ligation of the deep femoral artery and femoral vein followed by having later on developed secondary lymphedema of his left lower limb and pronounced manifested chronic venous insufficiency. Management included dissociation of the numerous arteriovenous fistulas between the branches of the deep femoral artery and the common femoral artery, as well as between the superficial femoral artery and femoral vein.

  15. [Central nervous system malformations: neurosurgery correlates].

    Science.gov (United States)

    Jiménez-León, Juan C; Betancourt-Fursow, Yaline M; Jiménez-Betancourt, Cristina S

    2013-09-06

    Congenital malformations of the central nervous system are related to alterations in neural tube formation, including most of the neurosurgical management entities, dysraphism and craniosynostosis; alterations of neuronal proliferation; megalencefaly and microcephaly; abnormal neuronal migration, lissencephaly, pachygyria, schizencephaly, agenesis of the corpus callosum, heterotopia and cortical dysplasia, spinal malformations and spinal dysraphism. We expose the classification of different central nervous system malformations that can be corrected by surgery in the shortest possible time and involving genesis mechanisms of these injuries getting better studied from neurogenic and neuroembryological fields, this involves connecting innovative knowledge areas where alteration mechanisms in dorsal induction (neural tube) and ventral induction (telencephalization) with the current way of correction, as well as the anomalies of cell proliferation and differentiation of neuronal migration and finally the complex malformations affecting the posterior fossa and current possibilities of correcting them.

  16. Vein of Galen Malformation: Outcome after Embolization

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-12-01

    Full Text Available The neurodevelopmental outcome after endovascular treatment of vein of Galen malformation (VOGM in 27 patients seen between 1983 and 2002 was assessed by chart review and parental questionnaires at the University of California, San Francisco.

  17. Human malformations induced by environmental noxae

    Energy Technology Data Exchange (ETDEWEB)

    Hecker, W.C.; Angerpointner, T.A.

    1980-01-01

    The paper reviews congenital malformations in humans and presents possible causes. 60% of all malformations are a result of environmental and other factors; i.e. not hereditary or caused by a disease of the mother. The teratogenic effects of ionizing radiation, drugs, alcohol, polyvinyl chloride and trichlorophenol are discussed as well as the effect of the mother's working in certain fields, e.g. clinical laboratories or printing offices; in the latter case the teratogenic noxae are still unknown. Efficient research requires centralized storage of all data on children born with malformations and on the mother's health situation during pregnancy, and the legislator is asked to do so while observing the law on data protection. Foundation of a German Institute of Teratology is recommended. In order to intensify research, it is suggested to set up groups or departments for research on malformations in some major paediatric hospitals.

  18. Inner ear malformations: a practical diagnostic approach.

    Science.gov (United States)

    Mazón, M; Pont, E; Montoya-Filardi, A; Carreres-Polo, J; Más-Estellés, F

    2016-12-29

    Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted. And finally, the radiological findings of each type of malformation are concisely described and depicted. Computed tomography and magnetic resonance imaging play a crucial role in the characterization of inner ear malformations and allow the assessment of the anatomical structures that enable the selection of appropriate treatment and surgical approach.

  19. Presenting Symptoms of Chiari Type I Malformation

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    J Gordon Millichap

    2009-06-01

    Full Text Available Clinical and radiographic predictors of neurologic symptoms were investigated in a population-based retrospective study of 51 children identified with Chiari I malformation at the University of California, and Kaiser Department of Radiology, San Francisco.

  20. Glomuvenous malformation: magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Flors, Lucia; Norton, Patrick T.; Hagspiel, Klaus D. [University of Virginia Health System, Department of Radiology and Medical Imaging, Charlottesville, VA (United States)

    2014-07-05

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

  1. Animal models for human craniofacial malformations.

    Science.gov (United States)

    Johnston, M C; Bronsky, P T

    1991-01-01

    Holoprosencephaly malformations, of which the fetal alcohol syndrome appears to be a mild form, can result from medial anterior neural plate deficiencies as demonstrated in an ethanol treated animal model. These malformations are associated with more medial positioning of the nasal placodes and resulting underdevelopment or absence of the medial nasal prominences (MNPs) and their derivatives. Malformations seen in the human retinoic acid syndrome (RAS) can be produced by administration of the drug 13-cis-retinoic acid in animals. Primary effects on neural crest cells account for most of these RAS malformations. Many of the malformations seen in the RAS are similar to those of hemifacial microsomia, suggesting similar neural crest involvement. Excessive cell death, apparently limited to trigeminal ganglion neuroblasts of placodal origin, follows 13-cis retinoic acid administration at the time of ganglion formation and leads to malformations virtually identical to those of the Treacher Collins syndrome (TCS). Secondary effects on neural crest cells in the area of the ganglion appear to be responsible for the TCS malformations. Malformations of the DiGeorge Syndrome are similar to those of the RAS and can be produced in mice by ethanol administration or by "knocking out" a homeobox gene (box 1.5). Human and animal studies indicate that cleft lips of multifactorial etiology may be generically susceptible because of small MNP)s or other MNP developmental alterations, such as those found in A/J mice, that make prominence contact more difficult. Experimental maternal hypoxia in mice indicates that cigarette smoking may increase the incidence of cleft lip by interfering with morphogenetic movements. Other human cleft lips may result from the action of a single major gene coding for TGF-alpha variants. A study with mouse palatal shelves in culture and other information suggest that a fusion problem may be involved.

  2. Syringomyelia associated with Chiari malformation in children

    Energy Technology Data Exchange (ETDEWEB)

    Sakamoto, Hiroaki; Kitano, Shouhei; Nishikawa, Misao; Yasui, Toshihiro [Osaka City General Hospital (Japan); Fujitani, Ken; Hakuba, Akira; Nakanishi, Naruhiko

    1997-02-01

    Among 28 patients with myelomeningocele (MMC group), the myelomeningocele in all patients was repaired shortly after birth, and a shunt was implanted for the associated hydrocephalus in 18 patients. MRI of the group of 28 indicated 20 were afflicted with Chiari II malformation, and the remaining 8 by Chiari I malformation. Among 8 patients lacking myelomeningocele (non-MMC group), seven demonstrated a large syrinx at the cervical and cervico-thoracic level; only one had a syrinx extending from the cervical level down to the lumbar level. None of these patients had hydrocephalus. Surgical decompression to improve cerebrospinal fluid (CSF) flow at the major cistern improved neurological signs in 7 patients. MRI indicated 4 patients were afflicted with Chiari I malformation, and the remaining 4 with Chiari II malformation. In the MMC group, the initial development of the syrinx at the lumbar level may be the result of a combination of occlusion of the caudal end of the central canal brought about by repair of the myelomeningocele and CSF flow into the hydromyelic cavity via the patent proximal portion of the central canal. In the non-MMC group, the syringomyelia may be considered an early onset type of syringomyelia associated with adult type Chiari malformation because the location of the syrinx was quite similar to that found in adult type Chiari malformation, and decompressive surgery was quite effective. In the non-MMC group, turbulence of the CSF now at the major cistern caused by the herniated cerebellum plays an important role in the enlargement of the syringomyelia. To offer greater appropriate management of pediatric Chiari malformation accompanied by syringomyelia, the malformation should be classified not by degree of the herniated brain tissue but by its association with neural tube defect (myelomeningocele). (K.H.)

  3. Terminal hemimyelocystocele associated with Chiari II malformation

    Directory of Open Access Journals (Sweden)

    Umamaheswara Reddy V.

    2014-06-01

    Full Text Available Terminal myelocystocele (TMC results from failure of embryonic CSF to drain outside the neural tube creating CSF reservoir within a dorsal meningocele. Association of Chiari II malformation with diastematomyelia and myelocystocele is extremely rare. Myelocystoceles do not have neural tissue so they have good prognosis after treatment, however when associated with hydromelia and Chiari malformation they present with neurological deficits. We present details of a 2 year old female who presented to us with this rare anomaly.

  4. Congenital pseudoarthrosis associated with venous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

    2007-06-15

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  5. Exsanguinating upper GI bleeds due to Unusual Arteriovenous Malformation (AVM of stomach and spleen: a case report

    Directory of Open Access Journals (Sweden)

    Baqai Mohammad

    2009-05-01

    Full Text Available Abstract Background In this paper we are reporting one case of exsanguinating upper gastrointestinal tract (GIT bleed requiring massive blood transfusion and immediate life saving surgery. Case presentation A 30 years old female, 12 weeks pregnant was referred to our hospital from the earth-quake affected area of Kashmir with history of upper abdominal pain, haematemesis and melaena for one week. After stabilizing the patient, upper gastro-intestinal endoscopy was performed. It revealed gastric ulcer just distal to the gastro-esophageal junction on the lesser curvature. Biopsy from the ulcer edge led to profuse spurting of the blood and patient went into state of shock. Immediate resuscitation led to rebleeding and recurrence of post haemorrahagic shock. Conclusion The patient was immediately explored and total gastrectectomy with splenectomy concluded as life saving procedure. A review of literature was conducted to make this report possible.

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

    OpenAIRE

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

    2015-01-01

    Pericytes regulate vessel stability and pericyte dysfunction contributes to retinopathies, stroke, and cancer. Here we define Notch as a key regulator of pericyte function during angiogenesis. In Notch1 +/−; Notch3 −/− mice, combined deficiency of Notch1 and Notch3 altered pericyte interaction with the endothelium and reduced pericyte coverage of the retinal vasculature. Notch1 and Notch3 were shown to cooperate to promote proper vascular basement membrane formation and contribute to endothel...

  7. Middle Meningeal Arteriovenous Fistula and Its Spontaneous Closure: A Case Report and Review of the Literature

    OpenAIRE

    2007-01-01

    Middle meningeal artery pseudo-aneurysms and arteriovenous fistulas are usually post-traumatic, although occasional iatrogenic cases have been reported. The treatment has been obliteration of the fistula by surgical or endovascular means. Spontaneous closure of fistula is uncommon. We report a case of non-traumatic middle meningeal arteriovenous fistula in a patient with alcoholism, which resolved spontaneously without treatment.

  8. Vasoespasmo cerebral

    OpenAIRE

    1987-01-01

    Vasoespasmo cerebral ocorre em patologias como enxaqueca, hemorragia subaracnóidea, trauma de crânio, após isquemia e/ou hipoxia. A fisiopatologia do vasoespasmo cerebral nestas patologias não está completamente desvendada. Neste artigo são analisados os fatores neuroquímicos e morfológicos responsáveis pelo controle circulatório cerebral. As alterações circulatórias que seguem a hemorragia subaracnóidea são utilizadas como exemplo. Conclui-se que fatores bioquímicos, fisiológicos e morfológi...

  9. The accuracy and utility of contrast-enhanced MR angiography for localization of spinal dural arteriovenous fistulas: the Toronto experience

    Energy Technology Data Exchange (ETDEWEB)

    Lindenholz, Arjen [University Medical Center Groningen, Department of Neurosurgery AB71, PO Box 30001, Groningen (Netherlands); Toronto Western Hospital, Department of Medical Imaging, Division of Neuroradiology, Toronto, ON (Canada); TerBrugge, Karel G.; Farb, Richard I. [Toronto Western Hospital, Department of Medical Imaging, Division of Neuroradiology, Toronto, ON (Canada); Dijk, J.M.C. van [University Medical Center Groningen, Department of Neurosurgery AB71, PO Box 30001, Groningen (Netherlands)

    2014-11-15

    The purpose of this study was to determine the accuracy and utility of contrast-enhanced MR angiography (CE-MRA) in spinal dural arteriovenous fistulas (SDAVF). A retrospective analysis from 1999-2012 identified 70 patients clinically suspected of harboring a SDAVF. Each patient underwent consecutive conventional MR-imaging, CE-MRA, and digital subtraction angiography (DSA). The presence or absence of serpentine flow voids, T2-weighted hyperintensity, and cord enhancement were evaluated, as well as location of the fistula as predicted by CE-MRA. DSA was used as the reference standard. Of the 70 cases, 53 were determined to be a SDAVF, 10 cases were shown to be other forms of vascular malformation, and 7 were DSA-negative. On MRI, all reported cases of SDAVF showed serpentine flow voids (100 %). T2-weighted hyperintensity was seen in 48 of 50 cases (96 %), extending to the conus in 41 of 48 cases (85 %). Cord enhancement was seen in 38 of 41 cases (93 %). CE-MRA correctly localized the SDAVF in 43 of the 53 cases (81 %). CE-MRA is a useful non-invasive examination for the detection and localization of SDAVF. CE-MRA facilitates but does not replace DSA as confirmation of location, fistula type, and arterial detail, which are required before treatment. (orig.)

  10. KRIT1 mutations in three Japanese pedigrees with hereditary cavernous malformation

    Science.gov (United States)

    Hirota, Kengo; Akagawa, Hiroyuki; Kikuchi, Asami; Oka, Hideki; Hino, Akihiko; Mitsuyama, Tetsuryu; Sasaki, Toshiyuki; Onda, Hideaki; Kawamata, Takakazu; Kasuya, Hidetoshi

    2016-01-01

    Cerebral cavernous malformation is a neurovascular abnormality that can cause seizures, focal neurological deficits and intracerebral hemorrhage. Familial forms of this condition are characterized by de novo formation of multiple lesions and are autosomal-dominantly inherited via CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10 mutations. We identified three truncating mutations in KRIT1 from three Japanese families with CCMs: a novel frameshift mutation, a known frameshift mutation and a known splice-site mutation that had not been previously analyzed for aberrant splicing. PMID:27766163

  11. Malformation of the fetal brain in thanatophoric dysplasia: US and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Fink, A.M. [The Royal Children' s Hospital, Department of Medical Imaging, Melbourne, Victoria (Australia); The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); The University of Melbourne, Department of Radiology, Melbourne, Victoria (Australia); Hingston, Tania; Sampson, Amanda [The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); Ng, Jessica [The Royal Children' s Hospital, Department of Anatomical Pathology, Melbourne, Victoria (Australia); The Royal Women' s Hospital, Department of Anatomical Pathology, Melbourne, Victoria (Australia); Palma-Dias, Ricardo [The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); The University of Melbourne, Department of Obstetrics and Gynaecology, Melbourne (Australia)

    2010-12-15

    We present a case in which the unusual cerebral malformations of thanatophoric dysplasia (TD) were identified on a 21-week fetal US and confirmed by antenatal MRI, postmortem imaging and autopsy. TD is the most common lethal skeletal dysplasia and is characterized by short long bones, which are often bowed (type 1), a small thorax, and skull deformities. There is also a recognised constellation of abnormalities of the brain primarily affecting the temporal lobes that, although well described in the postmortem setting, are not widely recognized in fetal imaging. Familiarity with this appearance will facilitate accurate antenatal diagnosis. (orig.)

  12. Ethanol sclerotherapy of peripheral venous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Rimon, U. E-mail: rimonu@sheba.health.gov.il; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B

    2004-12-01

    Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur.

  13. Microcephaly; Correlation between cerebral CT index and developmental quotient

    Energy Technology Data Exchange (ETDEWEB)

    Azuma, Ototaka; Onozaki, Michihiko; Hidano, Fumio; Mizuguchi, Susumu; Kodama, Kimio (Akita Medical Center for Disabled Children, Kawajiri (Japan)); Komatsu, Eiko; Sakemi, Kikuo; Yamashita, Jun; Sawaishi, Ukio

    1991-09-01

    Thirty one children with microcephaly were referred to Akita Medical Center for Disabled Chilren. Of these children, 28 underwent cerebral computed tomography (CT). Cerebral CT indices were examined in relation to developmental quotient and underlying diseases. Mental retardation, cerebral palsy, epilepsy, and congenital malformations were associated with microcephaly. The most common abnormal CT finding was the ventricular-brain ratio (92.9%, 26/28). CT indices, including Evans' index, the caudal nuclei ratio, transverse width of the third ventricle, the ventricular/intracranial area ratio, the brain/intracranial area ratio, the basal cistern ratio, width of the cerebral longitudinal fissure, and integrated brain CT index, were all significantly correlated with developmental quotient. (N.K.).

  14. CEREBRAL VENOUS THROMBOSIS AND TURNER SYNDROME: A RARE REPORTED ASSOCIATION.

    Science.gov (United States)

    Guler, A; Alpaydin, S; Bademkiran, F; Sirin, H; Celebisoy, N

    2015-01-01

    Turner Syndrome is the only known viable chromosomal monosomy, characterised by the complete or partial absence of an X chromosome. It's the most common chromosomal abnormality in females. Apart from the well known dysmorphic features of the syndrome, it has been associated with a number of vascular pathologies; mainly involving the cardiovascular, renovascular, peripheral vascular and cerebrovascular system. It seems striking that thromboembolism is not considered as a feature of the syndrome. Most of the thromboembolism cases are related to the arterial vascular system; except for some rare reported portal venous thrombosis cases, peripheral venous thrombosis cases and to the best of our knowledge a single case of cerebral venous thrombosis with Dandy Walker malformation and polymicrogyria. We herein report a cerebral venous thrombosis case with Turner Syndrome. With no other found underlying etiology, we want to highlight that Turner Syndrome, itself, may have a relationship not only with the cerebral arterial vascular system pathologies but also with the cerebral venous thrombosis.

  15. Novalis Stereotactic Radiosurgery for Spinal Dural Arteriovenous Fistula.

    Science.gov (United States)

    Sung, Kyoung-Su; Song, Young-Jin; Kim, Ki-Uk

    2016-07-01

    The spinal dural arteriovenous fistula (SDAVF) is rare, presenting with progressive, insidious symptoms, and inducing spinal cord ischemia and myelopathy, resulting in severe neurological deficits. If physicians have accurate and enough information about vascular anatomy and hemodynamics, they achieve the good results though the surgery or endovascular embolization. However, when selective spinal angiography is unsuccessful due to neurological deficits, surgery and endovascular embolization might be failed because of inadequate information. We describe a patient with a history of vasospasm during spinal angiography, who was successfully treated by spinal stereotactic radiosurgery using Novalis system.

  16. [Arteriovenous fistulae of posttraumatic origin: apropos 7 cases].

    Science.gov (United States)

    Lerma, R; Galego, G; Lisbona, C; Martorell, A; Silveira, P; Callejas, J M

    1992-01-01

    We present our experience with arterio-venous traumatic fistulas. Seven cases, between 6 patients are reviewed. Respective etiologies were: 4 cases after an accidental traumatism, 2 cases produced by iatrogenicity and 1 case of idiopathic etiology. Respective localizations were: 6 cases at lower limbs (85.7%) and one case at cervical column (14.2%). All patients underwent surgical treatment. There was any case of mortality and treatment results were satisfactory in all cases. Patients were followed and, up to now, no recidives have been registered. follow-time ranged from 1.5 to more than 6 years.

  17. [Arteriovenous fistula in patients with a complicated postphlebitic syndrome].

    Science.gov (United States)

    Quiñones Castro, M; Fernández de la Vara Sánchez, L; Perera González, F; Alvarez Sánchez, J A

    1991-01-01

    An investigation, which goal is to evidencing AV fistulas in patients with a complicated postthrombotic syndrome, as well as to determinate the efficacy of noninvasive methods used for the study of AV fistulas in such patients, is presented. So, patients with a complicated postphlebitic syndrome interned into the Instituto de Angiología were studied. Twenty-five patients were included in the study, 9 of these patients (36%) had arteriovenous fistulas, arteriographically evidenced. Haemodynamic study do not support the evidence of such communications.

  18. Combined arteriovenous thrombolytic infusion for refractory renal vein thrombosis.

    Science.gov (United States)

    Heafner, Thomas A; Scott, Daniel; Watson, J Devin; Propper, Brandon; Johnson, Chatt; Arthurs, Zachary M

    2014-08-01

    Acute renal vein thrombosis can rapidly lead to significant impairment and eventual loss of renal function. Classically presenting with flank pain, hematuria, and laboratory markers consistent with acute kidney injury, therapeutic anticoagulation is the mainstay of treatment. However, endovascular surgery offers a safe and effective alternative for renal salvage in the setting of acute renal vein thrombosis. Described is the use of combined arteriovenous thrombolytic infusion for refractory renal vein thromboses to quickly and effectively decrease clot burden in the micro- and macrovenous circulations while limiting systemic exposure.

  19. Posterior cranial fossa arteriovenous fistula with presenting as caroticocavernous fistula

    Energy Technology Data Exchange (ETDEWEB)

    Liu, H.M.; Shih, H.C.; Huang, Y.C.; Wang, Y.H. [Dept. of Medical Imaging, National Taiwan University Hospital, Taipei (Taiwan)

    2001-05-01

    We report cases of posterior cranial fossa arteriovenous fistula (AVF) with presenting with exophthalmos, chemosis and tinnitus in 26- and 66-year-old men. The final diagnoses was vertebral artery AVF and AVF of the marginal sinus, respectively. The dominant venous drainage was the cause of the unusual presentation: both drained from the jugular bulb or marginal sinus, via the inferior petrosal and cavernous sinuses and superior ophthalmic vein. We used endovascular techniques, with coils and liquid adhesives to occlude the fistulae, with resolution of the symptoms and signs. (orig.)

  20. Intracranial dural arterio-venous fistula presenting with progressive myelopathy.

    LENUS (Irish Health Repository)

    Ogbonnaya, Ebere Sunny

    2011-01-01

    Spinal dural arterio-venous fistula (DAVF) is rare and usually involves the thoracic segments. The classical presentation is a slowly progressive ataxia. Clinical presentation of intracranial DAVF depends on the site of the DAVF, as well as the vessels involved. Patients may present with pulsatile tinnitus, occipital bruit, headache, dementia, visual impairment as well as neurological deterioration distant from the DAVF as a result of venous hypertension and cortical haemorrhage. The authors present a rare case of progressive myelopathy secondary to an intracranial DAVF.

  1. Chiari type I malformation in children.

    Science.gov (United States)

    Massimi, L; Novegno, F; di Rocco, C

    2011-01-01

    The diagnosis of Chiari type I malformation (CIM) is more and more frequent in clinical practice due to the wide diffusion of magnetic resonance imaging. In many cases, such a diagnosis is made incidentally in asymptomatic patients, as including children investigated for different reasons such as mental development delay or sequelae of brain injury. The large number of affected patients, the presence of asymptomatic subjects, the uncertainties surrounding the pathogenesis of the malformation, and the different options for its surgical treatment make the management of CIM particularly controversial.This paper reports on the state of the art and the recent achievements about CIM aiming at providing further information especially on the pathogenesis, the natural history, and the management of the malformation, which are the most controversial aspects. A historial review introduces and explains the current classification. Furthermore, the main clinical, radiological, and neurophysiological findings of CIM are described to complete the picture of this heterogeneous and complex disease.

  2. Imaging of head and neck venous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Flis, Christine M.; Connor, Stephen E. [King' s College Hospital, Neuroradiology Department, London (United Kingdom)

    2005-10-01

    Venous malformations (VMs) are non proliferative lesions that consist of dysplastic venous channels. The aim of imaging is to characterise the lesion and define its anatomic extent. We will describe the plain film, ultrasound (US) (including colour and duplex Doppler), computed tomography (CT), magnetic resonance imaging (MRI), conventional angiographic and direct phlebographic appearances of venous malformations. They will be illustrated at a number of head and neck locations, including orbit, oral cavity, superficial and deep facial space, supraglottic and intramuscular. An understanding of the classification of such vascular anomalies is required to define the correct therapeutic procedure to employ. Image-guided sclerotherapy alone or in combination with surgery is now the first line treatment option in many cases of head and neck venous malformations, so the radiologist is now an integral part of the multidisciplinary management team. (orig.)

  3. Cochlear Implantation in Children with Cochlear Malformation.

    Science.gov (United States)

    Saikawa, Etsuko; Takano, Kenichi; Ogasawara, Noriko; Tsubomatsu, Chieko; Takahashi, Nozomi; Shirasaki, Hideaki; Himi, Tetsuo

    2016-01-01

    Cochlear implantation (CI) has proven to be an effective treatment for severe bilateral sensorineural hearing loss (SNHL). Inner ear malformation is a rare anomaly and occurs in approximately 20% of cases with congenital SNHL. In cases with cochlear malformation, CI can be successfully performed in nearly all patients, the exceptions being those with complete labyrinthine and cochlear aplasia. It is important to evaluate the severity of inner ear deformity and other associated anomalies during the preimplantation radiological assessment in order to identify any complication that may potentially occur during the surgery and subsequent patient management.

  4. Papilloedema due to Chiari I malformation.

    Science.gov (United States)

    Zhang, Jason Chao; Bakir, Belal; Lee, Andrew; Yalamanchili, Sushma S

    2011-10-16

    The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation.

  5. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    Energy Technology Data Exchange (ETDEWEB)

    Cornelis, F., E-mail: francoiscornelis@hotmail.com [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France)

    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.

  6. Diagnosis, treatment, and prevention of cerebral palsy.

    Science.gov (United States)

    O'Shea, Thomas Michael

    2008-12-01

    Cerebral palsy is the most prevalent cause of persisting motor function impairment with a frequency of about 1/500 births. In developed countries, the prevalence rose after introduction of neonatal intensive care, but in the past decade, this trend has reversed. A recent international workshop defined cerebral palsy as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain." In a majority of cases, the predominant motor abnormality is spasticity; other forms of cerebral palsy include dyskinetic (dystonia or choreo-athetosis) and ataxic cerebral palsy. In preterm infants, about one-half of the cases have neuroimaging abnormalities, such as echolucency in the periventricular white matter or ventricular enlargement on cranial ultrasound. Among children born at or near term, about two-thirds have neuroimaging abnormalities, including focal infarction, brain malformations, and periventricular leukomalacia. In addition to the motor impairment, individuals with cerebral palsy may have sensory impairments, cognitive impairment, and epilepsy. Ambulation status, intelligence quotient, quality of speech, and hand function together are predictive of employment status. Mortality risk increases incrementally with increasing number of impairments, including intellectual, limb function, hearing, and vision. The care of individuals with cerebral palsy should include the provision of a primary care medical home for care coordination and support; diagnostic evaluations to identify brain abnormalities, severity of neurologic and functional abnormalities, and associated impairments; management of spasticity; and care for associated problems such as nutritional deficiencies, pain, dental care, bowel and bladder continence, and orthopedic complications. Current strategies to decrease the risk of cerebral palsy include interventions to

  7. Effect of Pingyangmycin on human venous malformation endothelial cells

    Institute of Scientific and Technical Information of China (English)

    Yi Fang Zhao; Zhi Jun Sun; Yu Lin Jia; Jun Jia; Ya Meng Si; Ji Hong Zhao; Wen Feng Zhang

    2008-01-01

    @@ Purpose: Venous malformations are common vascular anomalies with a propensity of the head and neck. Intralesional injection of Pingyangmycin (PYM, bleomycin A5 hydrochloride) is a widely used sclerotherapy method for the treatment of venous malformation.

  8. Endophthalmitis: a rare complication of arteriovenous fistula infection.

    Science.gov (United States)

    Desai, Madhav; Rapoor, Ram; Gudithi, Swarna Latha; Kumar, Ravi; Prasad, Neela; Dakshinamurty, Kaligotla Venkata

    2008-04-01

    Vascular access infection is a frequent problem in patients undergoing maintenance hemodialysis. Infection of arteriovenous fistula (AVF) is less common than dialysis catheter-associated infection. Previous case reports described endophthalmitis secondary to hemodialysis catheter-related infection, but not secondary to native AVF infection. We report a rare patient of endophthalmitis as a metastatic infection of AVF cannulation site abscess. A 19-year-old girl on maintenance hemodialysis for the past 2 years has presented with a history of fever, chills, and rigor of 3-days duration and painful dimness of vision in the left eye of 1-night duration. It was followed by redness of the eye, photophobia, and ocular discharge. On examination, the patient was febrile with an abscess near cannulation site of AVF. There was no perception of light in the left eye, conjunctiva was congested, cornea was clear, hypopyon present, and pupil was mid-dilated, not reacting to light. Lens was clear. Vitreitis and exudative retinal detachment was present. Methicillin sensitive Staphylococcus aureus was isolated from blood, pus from AVF abscess and vitreous fluid. Diagnosis of endophthalmitis was confirmed by B-scan ultrasound. She was treated with both intravenous and intraocular antibiotics and drainage of pus from AVF abscess and therapeutic vitrectomy. Though arteriovenous abscess responded to sensitive antibiotics and drainage, vision has not improved much. Strict aseptic precautions during regular AVF cannulation are required. Lapses may lead to loss of vision apart from described complications like access closure, endocarditis, and osteomyelitis.

  9. Treatment of postcatheterization femoral arteriovenous fistulas with simple prolonged bandaging

    Institute of Scientific and Technical Information of China (English)

    ZHOU Tao; LIU Zhen-jiang; ZHOU Sheng-hua; SHEN Xiang-qian; LIU Qi-ming; FANG Zhen-fei; HU Xin-qun; LI Jiang; L(U) Xiao-lin

    2007-01-01

    Background The methods for the treatment of postcatheterization femoral arteriovenous fistulas (AVF-s) - simple observation, ultrasound guided compression, covered stents implantation and coil embolization have poor outcome.Surgery is the standard method for treatment of femoral AVFs, but it is a traumatic operation. In this study, we report the results of the treatment of postcatheterization femoral AVFs by simple prolonged compressing bandage.Methods To treat iatrogenic femoral AVFs caused by transfemoral catheterization, prolonged binding with elastic or common bandage was applied in 16 cases. Catheterization was performed in 7 cases for radiofrequency current catheter ablation, in 4 for occlusion of congenital heart disease, in 3 for percutaneous coronary intervention, in 1 for coronary angiography and in 1 for right heart catheterization.Results All iatrogenic femoral AVFs were healed after simple binding with elastic or common bandage for 4-46 days (mean (15±10) days). During the period of binding, local skins ulceration occurred at puncture site in two cases and femoral vein thrombus was found in one patient. During 6-24 months (mean (11.8±3.6) months) followup with colour Doppler ultrasonography, no recurrent arteriovenous shunting or other complications were observed.Conclusion The results suggest that simple prolonged bandaging for postcatheterization femoral AVFs is an effective and economical procedure.

  10. Cervical Myelopathy Caused by Intracranial Dural Arteriovenous Fistula

    Science.gov (United States)

    Kim, Won Young; Kim, Jin Bum; Nam, Taek Kyun; Kim, Young Baeg

    2016-01-01

    Intracranial dural arteriovenous fistula (dAVF) usually results in various problems in the brain. But it can be presented as a myelopathy, which may make early diagnosis and management to be difficult. We recently experienced a case of cervical myelopathy caused by intracranial dAVF. A 60-year-old man presented with a 3-year history of gait disturbance due to a progressive weakness of both legs. Neurological examination revealed spastic paraparesis (grade IV) and Babinski sign on both sides. Magnetic resonance imaging showed serpentine vascular signal voids at C2-T1 on T2-weighted image with increased signal intensity and swelling of spinal cord at C1-C4. We performed a brain computed tomography angiography and found intracranial dAVF with multiple arteriovenous shunts. Venous drainages were noted at tentorial veins and cervical perimedullary veins. After Onyx embolization, the patient showed gradual improvement in motor power and gait disturbance. The venous drainage pattern is a well-known prognostic factor of dAVF. In our case, the intracranial dAVF drained to spinal perimedullary vein, which seemed to result in the ischemic myelopathy. Although it is rare condition, it sometimes can cause serious complications. Therefore, we should keep in mind the possibility of intracranial dAVF when a patient presents myelopathy. PMID:27437016

  11. An unusual case of fistula formation and thrombosis between arteriovenous graft and a native vein

    Directory of Open Access Journals (Sweden)

    Young Sub Kim

    2016-03-01

    Full Text Available Arteriovenous graft for hemodialysis vascular access is a widely used technique with many advantages. However, it has crucial complications with graft thrombosis and infection. We recently experienced an unusual case of arteriovenous graft complication involving graft thrombosis related to fistula formation between the graft and the natural vein with infection. We diagnosed this condition using Doppler ultrasound and computed tomography angiography. Successful surgical treatment including partial graft excision and creation of a secondary arteriovenous fistula using an inadvertently dilated cephalic vein was performed. The dialysis unit staff should keep this condition in mind and try to prevent this complication.

  12. A STUDY OF POSTERIOR FOSSA MALFORMATIONS: MR IMAGING

    Directory of Open Access Journals (Sweden)

    Ravi

    2015-02-01

    Full Text Available AIMS AND OBJECTIVES: The aim of our study is to describe the imaging findings of various posterior fossa malformations and to evaluate the supratentorial abnormalities associated with posterior fossa malformations. MATERIALS AND METHODS: MR images of 30 patients wi th posterior fossa malformations detected in the department of Radiodiagnosis, BMCRI over a period of two years, from December 2012 to December 2014 were evaluated retrospectively. The various posterior fossa malformations were evaluated. Associated suprat entorial abnormalities were noted. RESULTS: 30 patients with posterior fossa malformations were included in the study. The age group of patients ranged from 1year to 53years. There were 18 males and 12 females. The various posterior fossa malformations det ected were Dandy Walker malformation (1 case, Dandy Walker variant (2 cases, mega cisterna magna (8 cases, arachnoid cysts (5 cases, Chiari 1 malformation (5 cases, Chairi 2 malformation (2 cases, Joubert malformation (1 case, lipoma (2 cases, verm ian and/or cerebellar hypoplasia without posterior fossa CSF collection or cyst (4 cases. Associated supratentorial abnormalities were seen in 8 cases . CONCLUSION: MRI is the imaging modality of choice in the evaluation of posterior fossa malformations. I t is very important to know the imaging findings of these malformations and to have knowledge about the various supratentorial and spinal abnormalities associated with them so as to provide an accurate diagnosis which is very essential for predicting the p rognosis and planning further management.

  13. RTTN mutations link primary cilia function to organization of the human cerebral cortex

    NARCIS (Netherlands)

    S.K. Kia; E. Verbeek (Elly); M.P. Engelen (Erik); R. Schot (Rachel); R.A. Poot (Raymond); I.F.M. de Coo (René); M. Leguin (Maarten); C.J. Poulton (Cathryn); F. Pourfarzad, F. (Farzin); F.G. Grosveld (Frank); A. Brehm (António); M.C.Y. de Wit (Marie Claire); R. Oegema (Renske); W.B. Dobyns (William); F.W. Verheijen (Frans); G.M.S. Mancini (Grazia)

    2012-01-01

    textabstractPolymicrogyria is a malformation of the developing cerebral cortex caused by abnormal organization and characterized by many small gyri and fusion of the outer molecular layer. We have identified autosomal-recessive mutations in RTTN, encoding Rotatin, in individuals with bilateral diffu

  14. The role of preoperative angiography in the management of giant meningiomas associated to vascular malformation

    Directory of Open Access Journals (Sweden)

    Fabio Papacci

    2015-01-01

    Full Text Available Background: The role of preoperative digital subtraction angiography (DSA in meningiomas is currently under discussion because of the introduction of noninvasive magnetic resonance imaging (MRI angiography to study vascular anatomy associated to the tumor. Preoperative DSA is mainly performed to obtain embolization of the lesion, although a number of complications have been reported after this procedure. Nonetheless, the coexistence of meningiomas with vascular malformations has previously been reported and it has been evidenced that this event could be underestimated because of neglect of preoperative DSA. Here, we report on two challenging cases of giant meningiomas associated to vascular malformations and we discuss the pertinent literature. Case Descriptions: In the first case: A large right temporal meningioma with erosion of the sphenoid greater wing and extension toward infratemporal fossa and right orbit - a large pseudoaneurysm of right middle cerebral artery branch was found end embolized during DSA. In the second case: A giant parieto-temporal meningioma - DSA permitted the full visualization of an abnormal drainage of superior sagittal sinus like a "sinus pericranii" that was respected during the following surgery. Conclusion: We think that MRI angiography is the exam of choice to study vascular anatomy in meningiomas. Nonetheless, DSA remains a useful tool in giant meningiomas not only to embolizate the lesion but also to treat tumor associated vascular malformation and to achieve the full knowledge of vascular anatomy. We think that a wide communication between interventionalist and surgeon is essential for the optimal management of these patients.

  15. Lymphatic malformations: a proposed management algorithm.

    LENUS (Irish Health Repository)

    Oosthuizen, J C

    2012-02-01

    OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors\\' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

  16. Congenital Malformations in River Buffalo (Bubalus bubalis

    Directory of Open Access Journals (Sweden)

    Sara Albarella

    2017-02-01

    Full Text Available The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital malformations most frequently reported in literature or signaled by breeders to the Department of Veterinary Medicine and Animal Production of the University Federico II (Naples, Italy in river buffalo are: musculoskeletal defects (transverse hemimelia, arthrogryposis, umbilical hernia and disorders of sexual development. In conclusion this review put in evidence that river buffalo have a great variety of malformations due to genetic causes, and TH and omphalocele are the most frequent and that several cases are still not reported, leading to an underestimation of the real weight of genetic diseases in this species.

  17. Fetal MRI clues to diagnose cloacal malformations

    Energy Technology Data Exchange (ETDEWEB)

    Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Patel, Manish N.; Kraus, Steven [Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States); Levitt, Marc A.; Pena, Alberto [Cincinnati Children' s Hospital Medical Center, Colorectal Center for Children, Pediatric Surgery, Cincinnati, OH (United States); Lim, Foong-Yen; Crombleholme, Timothy M. [Cincinnati Children' s Hospital Medical Center, Fetal Care Center of Cincinnati, Pediatric Surgery, Cincinnati, OH (United States); Linam, Leann E. [Arkansas Children' s Hospital, Department of Radiology, Little Rock, AR (United States)

    2011-09-15

    Prenatal US detection of cloacal malformations is challenging and rarely confirms this diagnosis. To define the prenatal MRI findings in cloacal malformations. We performed a retrospective study of patients with cloacal malformations who had pre- and post-natal assessment at our institution. Fetal MRI was obtained in six singleton pregnancies between 26 and 32 weeks of gestation. Imaging analysis was focused on the distal bowel, the urinary system and the genital tract and compared with postnatal clinical, radiological and surgical diagnoses. The distal bowel was dilated and did not extend below the bladder in five fetuses. They had a long common cloacal channel (3.5-6 cm) and a rectum located over the bladder base. Only one fetus with a posterior cloacal variant had a normal rectum. Three fetuses had increased T2 signal in the bowel and two increased T1/decreased T2 signal bladder content. All had renal anomalies, four had abnormal bladders and two had hydrocolpos. Assessment of the anorectal signal and pelvic anatomy during the third trimester helps to detect cloacal malformations in the fetus. The specificity for this diagnosis was highly increased when bowel fluid or bladder meconium content was identified. (orig.)

  18. Idiopathic hepatic arterial malformation: a case report

    Institute of Scientific and Technical Information of China (English)

    郑蔚巍; 周康荣; 王佩芬; 陈祖望

    2003-01-01

    @@ Hepatic arterial malformation is a rare disorder which either origi nates idiopathically or may be associated with hereditary hemorrhagic telangiect asia (also known as Osler-Weber-Rendu disease). Although previous reports presented only descriptions of sonographic and angiographic findings,1-6 we present a case of splenic infarct caused by this disorder with CT and CTA findi ngs.

  19. New concepts on posterior fossa malformations

    Energy Technology Data Exchange (ETDEWEB)

    Jaspan, Tim [Imaging Centre, University Hospital, Nottingham (United Kingdom)

    2008-06-15

    A full description of the embryology of the posterior fossa (PF) is beyond the scope of this review; several recent publications are recommended. Specific aspects of the processes involved are, however, reviewed as a background to malformations that involve defects or errors occurring at critical stages during the embryogenesis of the PF structures. (orig.)

  20. Ketogenic Diet for Epilepsy and Focal Malformation

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-09-01

    Full Text Available The efficacy and long-term treatment outcome of a classic ketogenic diet (KD addon treatment (4:1 lipid/nonlipid ratio, without initial fasting and fluid restriction were evaluated retrospectively in 47 children with intractable epilepsy and focal malformation of cortical development, in a study at Severance Children’s and Sanggye Park Hospitals, Seoul, Korea.

  1. [A woman with a rare vascular malformation

    NARCIS (Netherlands)

    Koning, G.G.; Vries, M. de

    2015-01-01

    A 30-year-old woman with trisomy 8 syndrome and coagulopathy was diagnosed with a malformation of the vena cava superior. This is a rare anatomical variation, which originates from a non-development of Marshall's ligament during the 8th week of gestation (prevalence: 0.3%).

  2. Percutaneous Treatment of Peripheral Vascular Malformations

    NARCIS (Netherlands)

    E. van der Linden (Edwin)

    2011-01-01

    textabstractVascular malformations arise from errors in the morphological processes that shape the embryonic vascular system during fetal development. These developmental errors result in abnormal clusters of blood vessels. Although these lesions are present at birth, they might not become visible u

  3. Congenital spinal malformations; Kongenitale spinale Malformationen

    Energy Technology Data Exchange (ETDEWEB)

    Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

    2001-12-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

  4. Phosphorus magnetic resonance spectroscopy in malformations of cortical development

    Directory of Open Access Journals (Sweden)

    Celi Santos Andrade

    2013-07-01

    Full Text Available Introduction Malformations of cortical development (MCD result from disruptions in the dynamic process of cerebral corticogenesis and are important causes of epilepsy, motor deficits and cognitive impairment. Objectives The aim of this study was to evaluate phospholipids metabolism in vivo in a series of patients with epilepsy and MCD. Methods Thirty-seven patients with MCD and 31 control subjects were studied using three-dimensional phosphorus magnetic resonance spectroscopy (31P-MRS at a 3.0 T scanner. Quantification methods were applied to the following resonances: phosphoethanolamine (PE, phosphocholine (PC, glycerophosphoethanolamine (GPE, glycerophosphocholine (GPC, inorganic phosphate (Pi, phosphocreatine (PCr, and a-, b-, and g-adenosine triphosphate (ATP. The magnesium (Mg2+ levels and pH were calculated based on PCr, Pi and b-ATP chemical shifts. Results Compared to controls, the MCD lesions exhibited lower pH values and higher Mg2+ levels (p<0.05. The lesions also presented significant reduction of GPC and PDE, and an increased PME/PDE ratio. The otherwise normal appearing parenchyma also demonstrated lower pH values in the frontoparietal cortex and bilateral centrum semiovale. Conclusions Our data support the idea that metabolic impairments occur in the lesions of MCD, with propagation to remote normal appearing parenchyma. The results also suggest that there are membrane turnover disturbances in MCD lesions.

  5. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Hedlund, Gary [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2006-07-15

    The newborn, infant, or young child who presents with a midline frontonasal mass often poses a diagnostic challenge to the clinician. The most pressing issue is whether the mass extends intracranially. The development of the frontonasal region or anterior neuropore is complex. Aberrant embryogenesis leads to three main types of anomalies: nasal dermal sinus, anterior cephalocele, and nasal glioma. Understanding the developmental anatomy of the anterior neuropore and postnatal maturation will serve the radiologist well when it comes to imaging frontonasal masses. Pitfalls particularly common to CT imaging interpretation include the evolving ossification of the frontal, nasal and ethmoid bones in the first year of life, morphology and size of the foramen cecum, and the natural intumescence of the anterior nasal septum. Determination of the presence of a connection between the frontonasal mass and the anterior cranial fossae is crucial in the imaging assessment and clinical management. In the case of the nasal dermal sinus, failure to appreciate the intracranial components of the malformation can lead to fatal meningitis. MR imaging is the modality of choice for assessing the pediatric frontonasal region. Its advantages include multiplanar imaging, distinguishing the interface among cartilage, bone, brain and fluid, diffusion imaging to detect epidermoid tumors, and the capacity to evaluate the brain for associated cerebral anomalies. (orig.)

  6. Congenital Malformations in River Buffalo (Bubalus bubalis)

    Science.gov (United States)

    Albarella, Sara; Ciotola, Francesca; D’Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

    2017-01-01

    Simple Summary Congenital malformations (due to genetic causes) represent a hidden danger for animal production, above all when genetic selection is undertaken for production improvements. These malformations are responsible for economic losses either because they reduce the productivity of the farm, or because their spread in the population would decrease the total productivity of that species/breed. River buffalo is a species of increasing interest all over the world for its production abilities, as proved by the buffalo genome project and the genetic selection plans that are currently performed in different countries. The aim of this review is to provide a general view of different models of congenital malformations in buffalo and their world distribution. This would be useful either for those who performed buffalo genetic selection or for researchers in genetic diseases, which would be an advantage to their studies with respect to the knowledge of gene mutations and interactions in this species. Abstract The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital

  7. Effects of vascular endothelial growth factor on angiogenesis of the endothelial cells isolated from cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    TAN YuZhen; ZHAO Yao; WANG HaiJie; ZHOU LiangFu; MAO Ying; LIU Rui; SHU Jia; WANG YongFei

    2008-01-01

    Human cerebral cavernous malformation (CM) is a common vascular malformation of the central nervous system. We have investigated the biological characteristics of CM endothelial cells and the cellular and molecular mechanisms of CM angiogenesis to offer new insights into exploring effective measures for treatment of this disease. The endothelial cells were isolated from CM tissue masses dissected during operation and expanded in vitro. Expression of VEGFR-1 and VEGFR-2 was examined with immunocytochemical staining. Proliferation, migration and tube formation of CM endothelial cells were determined using MTT, wounding and transmigration assays, and three-dimensional collagen type Ⅰ gel respectively. The endothelial cells were successfully isolated from the tissue specimens of 25 CMs dissected without dipolar electrocoagulation. The cells show the general characteristics of the vascular endothelial cells. Expression of VEGFR-1 and VEGFR-2 on the cells is higher than that on the normal cerebral microvascular endothelial cells. After treatment with VEGF, numbers of the proliferated and migrated cells, the maximal distance of cell migration and the length and area of capillary-like struc-tures formed in the three-dimensional collagen gel increase significantly. These results demonstrate that expression of VEGFR-1 and VEGFR-2 on CM endothelial cells is up-regulated. By binding to re-ceptors, VEGF may activate the downstream signaling pathways and promote proliferation, migration and tube formation of CM endothelial cells. VEGF/VEGFR signaling pathways play important regulating roles in CM angiogenesis.

  8. The effect of arteriovenous fistulas on in situ saphenous vein bypasses

    DEFF Research Database (Denmark)

    Rørdam, Peter; Jensen, Leif Panduro; Schroeder, T;

    1991-01-01

    Intraoperative identification and later development of arteriovenous fistulas were investigated prospectively in 70 in situ saphenous vein bypass procedures. Surveillance was performed by completion arteriography and intra- and postoperative continuous wave Doppler examination. The intraoperative...

  9. [Brainstem auditory evoked potentials and somatosensory evoked potentials in Chiari malformation].

    Science.gov (United States)

    Moncho, Dulce; Poca, María A; Minoves, Teresa; Ferré, Alejandro; Rahnama, Kimia; Sahuquillo, Juan

    2013-06-16

    Introduccion. La malformacion de Chiari (MC) incluye una serie de anomalias congenitas que tienen como comun denominador la ectopia de las amigdalas del cerebelo por debajo del foramen magno, lo que puede condicionar fenomenos compresivos del troncoencefalo, la medula espinal alta y los nervios craneales, alterando las respuestas de los potenciales evocados auditivos del tronco cerebral (PEATC) y de los potenciales evocados somatosensoriales (PESS). Sin embargo, las indicaciones de ambas exploraciones en las MC han sido motivo de estudio en un numero limitado de publicaciones, centradas en series cortas y heterogeneas de pacientes. Objetivo. Revisar los hallazgos de los PEATC y los PESS en los estudios publicados en pacientes con MC tipo 1 (MC-1) o tipo 2 (MC-2), y su indicacion en el diagnostico, tratamiento y seguimiento, especialmente en la MC-1. Desarrollo. Es un estudio de revision realizado mediante analisis de los estudios publicados en Medline desde 1966, localizados mediante PubMed, utilizando combinaciones de las palabras clave 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' y 'somatosensory evoked potentials', asi como informacion de pacientes con MC-1 valorados en los servicios de neurocirugia y neurofisiologia clinica del Hospital Universitari Vall d'Hebron. Conclusiones. Los hallazgos mas comunes de los PESS son la reduccion en la amplitud cortical para el nervio tibial posterior, la reduccion o ausencia del potencial cervical del nervio mediano y el aumento del intervalo N13-N20. En el caso de los PEATC, los hallazgos mas frecuentes descritos son el aumento del intervalo I-V y la alteracion periferica o coclear.

  10. Hämodynamische Analyse und Klassifikation der Gefäßstrukturen bei Patienten mit zerebralen arteriovenösen Malformationen

    Directory of Open Access Journals (Sweden)

    Möller, Dietmar

    2009-08-01

    Full Text Available Hintergrund: Eine zerebrale arteriovenöse Malformation (AVM ist eine Gefäßmissbildung im Gehirn, die sich durch das Fehlen eines kapillaren Gefäßbettes mit abnormem Kurzschluss zwischen dem arteriellen und dem folgendem venösen System auszeichnet, dem sog. Nidus. Die veränderten hämodynamischen Bedingungen resultieren in neurologischen Ausfällen sowie in dysplastischen Veränderungen der zu- und abführenden Gefäße und daraus folgenden erhöhten Blutungsrisiko. Zielsetzung: Für die diagnostische Beurteilung der AVM sind Informationen über die individuelle Gefäßstruktur und die Hämodynamik von besonderem Interesse. In diesem Beitrag wird ein Verfahren zur Extraktion von Parametern zur Beschreibung der Hämodynamik präsentiert. Aufbauend hierauf werden Verfahren zur automatischen Detektion des Nidus der arteriovenösen Malformation sowie der zuleitenden (Feeder, ableitenden (Drainagevenen und „en passage“-Gefäße vorgestellt. Als Eingabe hierfür dienen hochaufgelöste 3D- sowie zeitlich-räumliche 4D-MRT-Bildsequenzen. Methoden: Bei der vorgestellten Methode wird zunächst in den 3D-MRT-Bilddaten das Gefäßsystem semi-automatisch segmentiert. Auf Basis eines neuen Verfahrens zur Charakterisierung der Hämodynamik durch Bestimmung des Einflusszeitpunktes des Kontrastmittels mittels referenzbasierter Kurvenanpassung wird in einem weiteren Schritt in den zeitlich-räumlichen MR-Bildfolgen für jedes Voxel der zeitliche Signalverlauf analysiert. Zusätzlich wird die Flussgeschwindigkeit des Kontrastmittels diskret approximiert. Anschließend werden die extrahierten Parameterbilder mittels eines nicht-linearen Registrierungsverfahrens automatisch auf das segmentierte Gefäßsystem übertragen. Durch eine kombinierte Analyse der Intensität, der Geschwindigkeit und des relativen Einflusszeitpunktes des Blutes werden Gefäßstrukturen automatisch charakterisiert. Ergebnisse: Zur Evaluation der vorgestellte Methode standen 19

  11. A rare case of Chiari type-1 malformation accompanied by symptomatic cerebrospinal fluid hypovolemia: comparison of congenital Chiari type-1 malformation and acquired Chiari malformation secondary to cerebrospinal fluid hypovolemia: case report.

    Science.gov (United States)

    Hatae, Ryusuke; Kohri, Ryusuke; Maeda, Kazushi; Miyazono, Masayuki

    2014-01-01

    A 23-year-old woman was injured in a rear-end collision. She had general malaise and posterior neck pain, which were more severe when she was in an upright position. Magnetic resonance imaging (MRI) revealed the presence of cerebellar tonsil descensus and syringomyelia in the spinal cord. Radioisotope (RI) cisternography showed signs of an early accumulation of RI in the bladder, and a delayed accumulation of RI in the cerebral fornix. We considered the possibilities of cerebrospinal fluid (CSF) hypovolemia and congenital Chiari type-1 malformation as being responsible for her headache. To obtain a definitive diagnosis, we performed gadolinium (Gd)-enhanced MR cisternography and found evidence of CSF leakage. We performed an epidural blood patch (EBP), and her symptoms resolved. In 2 years since the episode, her symptoms have not recurred, and additional treatment has not been required. In addition, MRI performed 2 years after the EBP did not reveal any changes. There seems no previous report which described successful differentiation of pre-existing congenital Chiari type-1 malformation from the acquired one caused by symptomatic CSF hypovolemia. Because treatment protocols differ between these two conditions, the establishment of a correct diagnosis is important.

  12. Jugular-axillary vein bypass for salvage of arteriovenous access.

    Science.gov (United States)

    Fulks, K D; Hyde, G L

    1989-01-01

    Stenosis or occlusion of the subclavian vein can cause incapacitating upper extremity swelling and venous hypertension in the patient with an arteriovenous (AV) access. A case of subclavian vein occlusion is reported that was treated with internal jugular-axillary vein bypass. This procedure resulted in salvage of the access and rapid resolution of the associated upper extremity swelling. It was concluded that jugular-axillary vein bypass should be considered in patients who have massive upper extremity edema resulting from a functioning AV access and ipsilateral subclavian vein occlusion. Patients undergoing creation of an AV access who have had previous temporary subclavian catheters or previous early failure of an AV access should have phlebography before surgery.

  13. Arteriovenous fistulas aggravate the hemodynamic effect of vein bypass stenoses

    DEFF Research Database (Denmark)

    Nielsen, T G; Djurhuus, C; Pedersen, Erik Morre;

    1996-01-01

    PURPOSE: The purpose of this study was to assess the impact of arteriovenous fistulas combined with varying degrees of stenosis on distal bypass hemodynamics and Doppler spectral parameters. METHODS: In an in vitro flow model bypass stenoses causing 30%, 55%, and 70% diameter reduction were induced...... 10 cm upstream of a fistula with low outflow resistance. Flow and intraluminal pressure were measured proximal to the stenosis and downstream of the fistula. The waveform parameters peak systolic velocity, end-diastolic velocity, pulsatility index, and pulse rise time were determined from midstream...... Doppler spectra obtained 10 cm downstream of the fistula. All measurements were carried out with open and clamped fistula. RESULTS: At 30% diameter reducing stenosis opening of the fistula induced a 12% systolic pressure drop across the stenosis but had no adverse effect on the Doppler waveform parameters...

  14. Surgical treatment of complicated traumatic aneurysm and arteriovenous fistula

    Institute of Scientific and Technical Information of China (English)

    乔正荣; 时德

    2003-01-01

    Objective: To evaluate the surgical methods and the outcome of management for traumatic arterial aneurysm (TAA) and traumatic arteriovenous fistula (TAVF). Methods: A total of 121 patients with TAA or TAVF were treated by surgery. Clinical, operative and postoperative data were collected and analyzed retrospectively. Results: The surgical techniques included aneurysmectomy and arterial end-to-end anastomosis or vascular grafting or artery ligation, aneurysm ligation and bypass, vascular repair, fistula excision and vascular ligation or vascular grafting or repair and so on. One patient died (0.83%). The follow-up rates of TAA and TAVF were 65.7% and 60% respectively. Conclusions: Complicated TAA and TAVF in different sites should be treated with different methods.

  15. Acute paraplegia following embolization of spinal dural arteriovenous fistula

    Institute of Scientific and Technical Information of China (English)

    HUANG Cheng-guang; QI Xiang-qian; CHEN Huai-rui; L(U) Li-quan; WU Xiao-jun; BAI Ru-lin; LU Yi-cheng

    2011-01-01

    Embolization therapy has been used as the initial treatment for spinal dural arteriovenous fistula (SDAVF) only for certain patients or in certain medical institutions due to its minimal invasiveness, but the recurrence of embolization remains a clinical challenge. The recurrent patient usually exhibits a gradual onset of symptoms and progressive deterioration of neurological function. Developing paraplegia several hours after embolization is commonly seen in patients with venous thrombosis-related complications, for which anticoagulation therapy is often administered. This article reports on a SDAVF patient who had weakness of both lower extremities before embolization and developed complete paraplegia several hours after embolization therapy, later confirmed by angiography as fistula recurrence. The symptoms were relieved gradually after second embolization. The pathophysiology of this patient is also discussed.

  16. Intracranial dural arteriovenous fistula with spinal medullary venous drainage

    Energy Technology Data Exchange (ETDEWEB)

    Wiesmann, M.; Padovan, C.S.; Pfister, H.W.; Yousry, T.A. [Muenchen Univ. (Germany). Abt. fuer Neuroradiologie

    2000-10-01

    We report on a 46-year-old patient in whom an intracranial dural arteriovenous (AV) fistula, supplied by a branch of the ascending pharyngeal artery, drained into spinal veins and produced rapidly progressive symptoms of myelopathy and brainstem dysfunction including respiratory insufficiency. Magnetic resonance imaging studies demonstrated brainstem oedema and dilated veins of the brainstem and spinal cord. Endovascular embolization of the fistula led to good neurological recovery, although the patient had been paraplegic for 24 h prior to embolization. This case demonstrates the MRI characteristics of an intracranial dural AV fistula with spinal drainage and illustrates the importance of early diagnosis and treatment. Even paraplegia may be reversible, if angiography is performed and the fistula treated before ischaemic and gliotic changes become irreversible. (orig.)

  17. Renal aneurysm and arteriovenous fistula; Management with transcatheter embolization

    Energy Technology Data Exchange (ETDEWEB)

    Savastano, S.; Feltrin, G.P.; Miotto, D.; Chiesura-Corona, M. (Padua Univ. (Italy). Ist. di Radiologia Padua Univ. (Italy). Ist. di Fisioterapia)

    1990-01-01

    Embolization was performed in six patients with renal artery aneurysms (n=2) and arteriovenous fistulas (AVF) (n=5). The aneurysms were observed in one patient with fibromuscular dysplasia and in another with Ehlers-Danlos syndrome. All the AVFs were intraparenchymal and secondary to iatrogenic trauma. Elective embolization was performed in five patients with good clinical results at follow-up between 1 and 9 years. Because of rupture of the aneurysm emergency embolization was attempted without success in the patient with Ehlers-Danlos syndrome, and nephrectomy was carried out. A postembolization syndrome complicated three procedures in which Gelfoam and polyvinyl alcohol were used; in two of these cases unexpected reflux of the particulate material occurred, resulting in limited undesired ablation of the ipsilateral renal parenchyma. Embolization is the most reliable and effective treatment for intrarenal vascular abnormalities since it minimizes the parenchymal damage. (orig.).

  18. Pre- and postnatal MRI of the fetus with complex intracranial vascular malformation; Prae- und postnatale MRT des Fetus bei komplexer intrakranieller vaskulaerer Malformation

    Energy Technology Data Exchange (ETDEWEB)

    Blondin, D.; Turowski, B.; Moedder, U.; Schaper, J. [Universitaetsklinikum Duesseldorf (Germany). Institut fuer Diagnostische Radiologie

    2006-02-15

    Intracranial vascular malformations (IVM) on the whole occur in neonates very rarely. In such cases of IVM two different kinds can and must be discerned: (1) dural arteriovenous shunts (DAVS) and (2) vein of Galen aneurysmal malformations (VGAM). The latter seem to represent the majority of IVM of newborns. Several classifications are known for both types. Mortality of neonates and babies due to DAVS exceeds the average mortality from DAVS in adults. VGAM and DAVS can be diagnosed by prenatal ultrasonography only when the vessels behind the shunt are vasodilated because of the increasing flow stress. For that reason VGAM and DAVS are generally not recognized before the last trimenon or even postnatally. The prognosis of an IVM is influenced by the shunt capacity and its resulting cardiac stress as well as by the child's age. To plan the mostly interventional therapy, postnatal angiography (DSA) and MRI are employed and to an increasing extent also prenatal MRI examinations. We describe the case of a very complex IVM, the full dimensions of which could only be discerned by using pre- and postnatal MRI supplementary to the ultrasonography. Besides DAVS, flows from the pericallosal arteries as well as from the thalamic branches had to be considered. As a consequence DAVS and in addition VGAM existed in parallel. (orig.) [German] Intrakranielle vaskulaere Malformationen (IVM) des Neugeborenen sind insgesamt selten. Hierbei koennen und muessen durale arteriovenoese Shunts (DAVS) und aneurysmatische Malformationen der V. Galeni (VGAM) unterschieden werden, letztere stellt die wohl haeufigste IVM des Neugeborenen dar. Fuer beide existieren mehrere Klassifizierungen. Die Mortalitaet der DVAS ist im Neugeborenenalter ueberdurchschnittlich hoch. Erst wenn durch eine erhoehte Flussbelastung sich die dem Gefaesskurzschluss nachgeschalteten Gefaesse im Verlauf der Schwangerschaft erweitern, koennen VGAM und DVAS im Ultraschall diagnostiziert werden. Daher werden sie in der

  19. PET in malformations of cortical development; La tomographie d'emission de positons (TEP) dans les malformations corticales de developpement

    Energy Technology Data Exchange (ETDEWEB)

    Bouilleret, V.; O' Brien, T.J. [Department of medicine, the Royal Melbourne Hospital, Royal Parade, Parkville, 3005 Victoria (Australia); Bouilleret, V. [Unite de neurophysiologie clinique et d' epileptologie, AP-HP, CHU Bicetre, 94275 Paris (France); Bouilleret, V.; Chiron, C. [Service hospitalier Frederic-Joliot, DRM, CEA, 4, place du General-Leclerc, 91401 Orsay cedex (France); Chiron, C. [Inserm U663, AP-HP, hopital Necker, 75015 Paris (France); University Paris-Descartes, 11, rue Pierre-et-Marie-Curie, 75005 Paris (France)

    2009-01-15

    Within the group of malformations of cortical development, focal cortical dysplasia (FCD) are an increasingly recognized cause of intractable epilepsy that can be cured by surgery. The success of cortical resection for intractable epilepsy is highly dependent on the accurate pre-surgical delineation of the regions responsible for generating seizures. [{sup 18}F]-FDG PET, which images cerebral metabolism studying brain glucose uptake, is the most established functional imaging modality in the evaluation of patients with epilepsy. The aim of this article is to review [{sup 18}F]-FDG PET usefulness as a pre-surgical tool in the evaluation of medically refractory partial epilepsy. It has an established place in assisting in the localisation and definition of FCD in patients with no lesion, or only a subtle abnormality, on MRI. The role of FDG-PET in defining the extent of the surgical resection is still uncertain and needs to be the focus of future research. (authors)

  20. Clinical Outcome Measures in Chiari I Malformation.

    Science.gov (United States)

    Yarbrough, Chester K; Greenberg, Jacob K; Park, Tae Sung

    2015-10-01

    Chiari malformation type 1 (CM-I) is a common and often debilitating neurologic disease. Reliable evaluation of treatments has been hampered by inconsistent use of clinical outcome measures. A variety of outcome measurement tools are available, although few have been validated in CM-I. The recent development of the Chicago Chiari Outcome Scale and the Chiari Symptom Profile provides CM-I-specific instruments to measure outcomes in adults and children, although validation and refinement may be necessary.

  1. Radiological features of childhood giant cavernous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Ozgen, Burce; Senocak, Efsun; Oguz, Kader K. [Hacettepe University, Department of Radiology, Faculty of Medicine, Ankara (Turkey); Soylemezoglu, Figen [Hacettepe University, Department of Pathology, School of Medicine, Ankara (Turkey); Akalan, Nejat [Hacettepe University, Department of Neurosurgery, School of Medicine, Ankara (Turkey)

    2011-04-15

    Giant cavernous malformations (GCM) are very large, low-flow vascular malformations, which usually have atypical imaging features and are commonly misdiagnosed preoperatively as neoplasms or vascular malformations. These lesions have mostly been reported in children. As cavernomas show different features in children compared to adults, we evaluated the imaging features of pediatric GCMs in order to help in the preoperative diagnosis of these malformations. Brain MR studies of nine children (mean age of 4 years; 8 months-9 years) with biopsy-proven GCM were retrospectively evaluated. We defined GCMs as cavernomas of {>=}4 cm. Lesions were evaluated regarding their size, location, signal characteristics, general appearance (uni/multilocular) as well as regarding the presence of mass effect, edema, and fluid-fluid levels and were classified according to the Mottolese classification of pediatric cavernomas. Lesion locations were parietal (n = 5), frontal (n = 2), temporal, and intraventricular. Seven lesions were in the periventricular region (with five in the periatrial region). Six patients had T1 hyperintense multilobulated lesions with ''bubbles of blood'' appearance and three patients had heterogeneous lesions with reticular core. All lesions had mass effect, edema (marked in four cases), and peripheral hemosiderin rim. Fluid-fluid levels were also common (n = 7). Most of our lesions (six of nine) were classified as type IIIA, two as type IIIC, and one as type IA. In children, a GCM should be considered in case of very large hemorrhagic intra-axial mass with ''bubbles of blood'' multicystic appearance, surrounding hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect, especially in the periatrial location. (orig.)

  2. Magnetic Resonance Imaging of Malformations of Midbrain-Hindbrain.

    Science.gov (United States)

    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.

  3. Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

    Energy Technology Data Exchange (ETDEWEB)

    Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

    2006-04-15

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  4. Malformations of cortical development and neocortical focus.

    Science.gov (United States)

    Luhmann, Heiko J; Kilb, Werner; Clusmann, Hans

    2014-01-01

    Developmental neocortical malformations resulting from abnormal neurogenesis, disturbances in programmed cell death, or neuronal migration disorders may cause a long-term hyperexcitability. Early generated Cajal-Retzius and subplate neurons play important roles in transient cortical circuits, and structural/functional disorders in early cortical development may induce persistent network disturbances and epileptic disorders. In particular, depolarizing GABAergic responses are important for the regulation of neurodevelopmental events, like neurogenesis or migration, while pathophysiological alterations in chloride homeostasis may cause epileptic activity. Although modern imaging techniques may provide an estimate of the structural lesion, the site and extent of the cortical malformation may not correlate with the epileptogenic zone. The neocortical focus may be surrounded by widespread molecular, structural, and functional disturbances, which are difficult to recognize with imaging technologies. However, modern imaging and electrophysiological techniques enable focused hypotheses of the neocortical epileptogenic zone, thus allowing more specific epilepsy surgery. Focal cortical malformation can be successfully removed with minimal rim, close to or even within eloquent cortex with a promising risk-benefit ratio.

  5. Fiber-optic laser-Doppler anemometer microscope applied to the cerebral microcirculation in rats.

    Science.gov (United States)

    Seki, J; Sasaki, Y; Oyama, T; Yamamoto, J

    1996-01-01

    We have applied our developed fiber-optic laser-Doppler anemometer microscope (FLDAM) for the study of the cerebral microcirculation in the rat. The red cell velocity in single pial microvessels was successfully measured through a closed cranial window for the vessel diameter range from 7.8 to 230 microns. The temporal resolution of the FLDAM was sufficiently high to detect the pulsation in the arterioles. Arterio-venous distributions of the temporal mean red cell velocity and wall shear rate are also described.

  6. 糖尿病肾病血液净化患者动静脉内瘘护理研究%Study on the nursing of arteriovenous fistula in patients with diabetic nephropathy

    Institute of Scientific and Technical Information of China (English)

    杨光

    2015-01-01

    目的:研究探讨对糖尿病肾病血液透析患者动静脉内瘘护理的有效方式。方法选取自2012年1月—2014年6月期间收治的78例糖尿病肾病透析患者,对其动静脉内瘘情况进行有针对性护理,然后观察。结果经过科学的系统化护理,动静脉内瘘使用良好的有75例(96.15%);使用3年后闭塞的2例(2.6%);由脑出血导致死亡的1例(1.3%);均未发生内瘘感染。结论对糖尿病肾病血液透析患者,准确和熟练掌握动静脉内瘘的穿刺方法,并认真观察透析过程,正确把握好动静脉内瘘的启用时机,同时注意结束时,正确按压血管以及严格无菌操作等,都是保护动静脉内瘘的关键所在。%Objective To study the effective method of nursing of arteriovenous fistula in hemodialysis patients with diabetic nephropathy. Methods From January 2012 to June 2014, 78 patients with diabetic nephropathy were treated with dialysis patients, and the arteriovenous fistula in the case of targeted nursing, and then observe. Results After scientific systematic nursing, 75 cases (96.15%) were used in the arteriovenous fistula (3), 2 cases (2.6%) were occluded after years, 1 cases (1.3%) died of cerebral hemorrhage. Conclusion On diabetic nephropathy in hemodialysis patients, accurate and skilled master dynamic internal arteriovenous fistula puncture method, and careful observation of the dialysis process, correctly grasp the opportunity for the active vein fistula opening, also pay attention to the end, the correct pressing blood vessel and strict aseptic operation, is to protect animals, arteriovenous fistula is the key.

  7. Otosclerosis associated with type B-1 inner ear malformation

    OpenAIRE

    De Stefano, A.; DISPENZA, F.; Aggarwal, N.; Russo, A.

    2010-01-01

    Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossi...

  8. "COCHLEAR IMPLANTATION IN PATIENTS WITH INNER EAR MALFORMATIONS"

    OpenAIRE

    P. Borghei S. Abdi; M. Motesaddi Zari; Khalessi MH

    2004-01-01

    Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. This study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. Six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. The average follow-up period was 27 months. Malformations included one incomplete par...

  9. [Diagnosis of fetal malformations with ultrasound--state of development].

    Science.gov (United States)

    Fendel, M; Fendel, H

    1983-01-01

    Ultrasonography is of great importance for the prenatal diagnosis of fetal malformations and abnormalities. An early diagnosis in the second trimester is of great interest for an intrauterine or an extrauterine therapy planning (the choice of the time and mode of delivery). Defects of the neural tube including hydrocephalus, malformations of the extremities, the gastrointestinal tract, omphaloceles, the urogenital and cardiac system are described. Four cases of fetal malformations are presented: fetal myelomeningocele, hydrocephalus, bilateral hydronephrosis and lymphangioma with fetal ascites.

  10. Does the risk of cerebral palsy increase or decrease with increasing gestational age?

    Directory of Open Access Journals (Sweden)

    Murphy-Kaulbeck Lynn

    2003-12-01

    Full Text Available Abstract Background It is generally accepted that the risk of cerebral palsy decreases with increasing gestational age of live born infants. However, recent studies have shown that cerebral palsy often has prenatal antecedents including congenital malformations, vascular insults and maternal infection. Cerebral palsy is therefore better viewed as occurring among fetuses, rather than among infants. We explored the epidemiologic implications of this change in perspective. Methods We used recently published data from Shiga Prefecture, Japan and from North-East England to examine the pattern of gestational age-specific rates of cerebral palsy under these alternative perspectives. We first calculated gestational age-specific rates of cerebral palsy as per convention, by dividing the number of cases of cerebral palsy identified among live births within any gestational age category by the number of live births in that gestational age category. Under the alternative formulation, we calculated gestational age-specific rates of cerebral palsy by dividing the number of cases of cerebral palsy identified among live births within any gestational age category by the number of fetuses who were at risk of being born at that gestation and being afflicted with cerebral palsy. Results Under the conventional formulation, cerebral palsy rates decreased with increasing gestational age from 63.9 per 1,000 live births at Conclusions The fetuses-at-risk approach is the appropriate epidemiologic formulation for calculating the gestational age-specific rate of cerebral palsy from a causal perspective. It shows that the risk of cerebral palsy increases as gestational duration increases. This compelling view of cerebral palsy risk may help refocus research aimed at understanding and preventing cerebral palsy.

  11. Dandy-Walker Malformation Presenting with Psychological Manifestations

    Directory of Open Access Journals (Sweden)

    Yasodha Maheshi Rohanachandra

    2016-01-01

    Full Text Available 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.

  12. The significance of changes in cerebral oxygen and glucose metabolism in patients with cerebral hemorrhage caused by acute hypertension%急性高血压脑出血患者脑糖氧代谢变化及意义

    Institute of Scientific and Technical Information of China (English)

    马骏; 陈锷峰; 屠传建; 钱辉; 骆明; 顾志伟; 张建民

    2014-01-01

    Objective To study the clinical significance of early changes in cerebral oxygen and glucose metabolism in patients with cerebral hemorrhage and with Glasgow coma score (GCS) of 5-8 caused by acute hypertension in order to find relationship between those changes and prognosis.Methods From January 1,2011 to June 30,2012,a cohort of 43 patients with cerebral hemorrhage caused by acute hypertension were enrolled for retrospective study.Radial artery and internal jugular vein were separately cannulated retrogradely for collecting blood for blood gas analysis and blood glucose tests carried out 24 hours after the onset of the cerebral hemorrhage and then every 6-8 hours and as any major changes in physical signs of patients occurred.And this monitoring kept for consecutive 3 days.The data of these laboratory findings were analyzed and calculated to determine internal jugular vein oxygen saturation (SjVO2),cerebral oxygen utilization rate (CEO2),cerebral arterio-venous oxygen difference (AVDO2),arterio-venous blood glucose difference (V-Aglu),arterio-venous lactic acid difference (V-Alac) and absolute value of carbon dioxide pressure difference between jugular vein and artery (V-APCO2).All patients met the diagnostic criteria of hypertensive cerebral hemorrhage revised by the 4th National Academic Conference on cerebrovascular disease in 1995 requiring diagnosis confirmed by brain CT,admitted within 24 hours of onset,Glasgow coma score (GCS) 5-8 and a history of hypertension.Exclusion criteria were:cerebral hemorrhage caused by traumatic intracranial hematoma,spontaneous subarachnoid hemorrhage,arteriovenous malformation and Moyamoya disease,intracranial tumor apoplexy,cerebral bleeding derived from the disturbance of blood coagulation system,and cerebral hemorrhagic infarction.According to the short-term prognosis,the patients were divided into the death group and the survival group.Then the differences in biomarkers mentioned above between two groups were compared to

  13. Treatment and Outcome of Epileptogenic Temporal Cavernous Malformations

    Institute of Scientific and Technical Information of China (English)

    Yong-Zhi Shan; Xiao-Tong Fan; Liang Meng; Yang An; Jian-Kun Xu; Guo-Guang Zhao

    2015-01-01

    Background:The aim of this study is to explore the treatment and outcome ofepileptogenic temporal lobe cavernous malformations (CMs).Methods:We analyzed retrospectively the profiles of 52 patients diagnosed as temporal lobe CMs associated with epilepsy.Among the 52 cases,11 underwent a direct resection of CM along with the adjacent zone of hemosiderin rim without electrocorticogram (ECoG) monitoring while the other 41 cases had operations under the guidance of ECoG.Forty-six patients were treated by lesionectomy + hemosiderin rim while the other six were treated by lesionectomy + hemosiderin rim along with extended epileptogenic zone resection.The locations of lesions,the duration of illness,the manifestation,the excision ranges and the outcomes of postoperative follow-up were analyzed,respectively.Results:All of the 52 patients were treated by microsurgery.There was no neurological deficit through the long-term follow-up.Outcomes of seizure control are as follows:42 patients (80.8%) belong to Engel Class Ⅰ,5 patients (9.6%) belong to Engel Class Ⅱ,3 patients (5.8%) belong to Engel Class Ⅲ and 2 patients (3.8%) belong to Engel Class Ⅳ.Conclusion:Patients with epilepsy caused by temporal CMs should be treated as early as possible.Resection of the lesion and the surrounding hemosiderin zone is necessary.Moreover,an extended excision of epileptogenic cortex or cerebral lobes is needed to achieve a better prognosis if the ECoG indicates the existence of an extra epilepsy onset origin outside the lesion itself.

  14. Electrocorticography-Guided Surgical Treatment of Solitary Supratentorial Cavernous Malformations with Secondary Epilepsy

    Institute of Scientific and Technical Information of China (English)

    Chao Wang; Chao You; Guo-qiang Han; Jun Wang; Yun-biao Xiong; Chuang-xi Liu

    2014-01-01

    Objective To evaluate the efficacy of electrocorticographic (ECoG) monitoring and the application of different surgical approaches in the surgical treatment of solitary supretentorial cavernous malformations with secondary epilepsy. Methods This study enrolled a consecutive series of 36 patients with solitary supratentorial cavernous malformations and secondary epilepsy who underwent surgery with intraoperative ECoG monitoring in the Department of Neurosurgery between January 2004 and January 2008. The patients were composed of 15 males and 21 females, aged between 8 and 52 years (mean age 27.3±2.8 years) at the time of surgery. Epilepsy history, the type of epilepsy at the presentation, lesion location, the incidence of residual epileptiform discharges, and postoperative outcomes were evaluated. Results Histopathological examination indicated cavernous malformations and hippocampal sclerosis in 36 and 5 cases, respectively. Neuronal degeneration, glial cell proliferation, and neurofibrillary tangles were found in all the resected cerebral tissues of extended lesionectomy of residual epileptic foci. Lesionectomy, anterior temporal lobectomy, anterior temporal lobectomy plus cortical thermocoagulation, extended lesionectomy, extended lesionectomy plus cortical thermocoagulation were performed in 4, 4, 1, 14, and 13 cases, respectively. Residual epileptiform discharges were captured in 9 out of the 14 patients who had additional cortical thermocoagulation. According to Engle class for postoperative outcomes, 27 cases were class I (75.00%), 5 were class II (13.89%), 2 were class III (5.56%), and 2 were class IV (5.56%), thus the total effective rate (class I+class II) was 88.89%. Neither of epilepsy history, the type of epilepsy, and the location of cavernous malformation was significantly related to outcomes (P>0.05). A significant relationship was found between the incidence of residual epileptiform discharges and outcomes (P=0.041). Conclusions Intraoperative ECo

  15. Efficacy of preoperative US vascular mapping for arteriovenous fistula in patients with hemodialysis

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Min Sun; Hwang, Ji Young; Kang, Byung Chul; Baek, Seung Yon [College of Medicine, Ewha Womans University, Seoul (Korea, Republic of)

    2007-07-15

    The purpose of this study is to assess the efficacy of preoperative US vascular mapping to predict postoperative patency of the arteriovenous fistula for hemodialysis. Sixty-six patients who underwent hemodialysis for end-stage renal failure (M:F = 34:32, mean age, 58.8 years) were observed prospectively from January 2001 to April 2003. The patients were divided into two groups: the vascular mapping group and the control group. A comparative analysis of the re-operation rate between the two groups was determined by use of the chi-square rest, efficacy of preoperative US vascular mapping according to the type of surgery. A comparative analysis of the secondary patency after percutaneous transluminal angioplasty was determined by the use of Fischer exact test, and a comparative analysis of the diminution of patency during the follow-up periods was determined by the use of the Logrank test. In the mapping group, the diameters of intraoperatively selected vessels were investigated and compared with the recommended diameter on preoperative US vascular mapping determined statistically by the use of Fisher's exact test. The preoperative US vascular mapping group had relatively lower re-operation rates (11.8%) than the control group (28.1%) ({rho} = 0.09). The preventive role of US vascular mapping in more effective in decreasing the re-operation rate for a native arteriovenous fistula (7.4%) than for a synthetic arteriovenous graft (25.9%) ({rho} = 0.06). For patients than had an interventional procedure, the failure rate to obtain a secondary patency was smaller than in the mapping group (33.3%), compared with the control group (46.3%) ({rho} = 0.37). Patients in the mapping group had a higher patency than the control group patients for a native arteriovenous fistula (92.0%) and a synthetic arteriovenous graft (71.4%) at one year following surgery ({rho} = 0.10, {rho} = 0.79). The arteriovenous fistulas in the mapping group had a higher patency for both a native

  16. Percutaneous Intervention in Axillary Loop-Configured Arteriovenous Grafts for Chronic Hemodialysis Patients

    Energy Technology Data Exchange (ETDEWEB)

    Park, Beom Jin; Chung, Hwan Hoon; Sung, Deuk Jae; Park, Sang Joon; Son, Ho Sung; Jo, Sang Kyung; Kim, Yun Hwan; Cho, Sung Bum [College of Medicine, Korea University, Seoul (Korea, Republic of); Kim, Hyoung Rae [Kangwon National University, Chuncheon (Korea, Republic of)

    2010-04-15

    The purpose of this study was to evaluate the fistulographic features of malfunctioning axillary loop-configured arteriovenous grafts and the efficacy of percutaneous interventions in failed axillary loop-configured arteriovenous grafts. Ten patients with axillary loop-configured arteriovenous grafts were referred for evaluation of graft patency or upper arm swelling. Fistulography and percutaneous intervention, including thrombolysis, percutaneous transluminal angioplasty and stent placement, were performed. Statistical analysis of the procedure success rate and the primary and secondary patency rates was done. Four patients had graft related and subclavian venous stenosis, two patients had graft related stenosis and another four patients had subclavian venous stenosis only. Sixteen sessions of interventional procedures were performed in eight patients (average: 2 sessions / patient) until the end of follow-up. An interventional procedure was not done in two patients with central venous stenosis. The overall procedure success rate was 69% (11 of 16 sessions). The post-intervention primary and secondary patency rates were 50% and 63% at three months, 38% and 63% at six months and 25% and 63% at one year, respectively. Dysfunctional axillary loop-configured arteriovenous grafts almost always had subclavian venous and graft-related stenosis. Interventional treatments are helpful to overcome this and these treatments are expected to play a major role in restoring and maintaining the axillary loop-configured arteriovenous loop grafts

  17. Treatment of an Immature Autogenous Arteriovenous Fistula with Percutaneous Transluminal Angioplasty

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyoung Rae [Kangwon National University, Chuncheon (Korea, Republic of)

    2008-11-15

    The purpose of this study was to evaluate the efficacy of percutaneous transluminal angioplasty (PTA) in facilitating maturation of autogenous arteriovenous fistulae. There were 12 immature autogenous arteriovenous fistulae. We performed 15 PTAs transvenously. Post-intervention anatomic and clinical successes were estimated, and the 6-month and 1-year primary and secondary patency rates were calculated using Kaplan-Meier analysis. All immature fistulae had underlying stenosis (n=20): arteriovenous anastomosis (n=1) and venous outflow (n=19): 1) within 5 cm from the anastomosis (n=10); 2) more than 5 cm but less than 10 cm from the anastomosis (n=5); 3) more than 10 cm from the anastomosis, including central veins (n=4). Six fistulae had two or more stenoses. Repeat intervention was necessary in two patients. The anatomical success rate was 94.3%, and the clinical success rate was 86.7%. The 6-month and 1-year primary patency rates were 72.7% and 54.5%, and the secondary patency rates were 100% and 81.8%, respectively. All immature hemodialysis fistulae have underlying stenosis, most of which are located near the arteriovenous anastomosis. Early interventional procedures are helpful in the salvage and maintenance of immature arteriovenous fistulae, with a high degree of success

  18. Malformations of the tooth root in humans

    Directory of Open Access Journals (Sweden)

    Hans Ulrich eLuder

    2015-10-01

    Full Text Available 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

  19. [Recurrent meningitis in inner ear malformations].

    Science.gov (United States)

    Claros, Pedro; Matusialk, Monika

    2008-01-01

    Authors present two cases of children with reccurent meningitis and unilateral deafness. Implemented diagnostics (CT, NMR, ABR) revealed one side inner ear congenital malformation in one case and anterior fossa bony defect accompanied by labirynthine deformation in the other case. The presence of perilymphatic fistulae in oval and round windows and cerebrospinal fluid leakage has been confirmed in both cases during surgery. Carefull obliteration of the Eustachian tube and both windows has been performed. Non- complicated postoperative course (2 months and 6 years - respectively) has prooved the effectiveness of applied treatment.

  20. Congenital malformations of the temporal bone.

    Science.gov (United States)

    Mukerji, Shraddha S; Parmar, Hemant A; Ibrahim, Mohannad; Mukherji, Suresh K

    2011-08-01

    Congenital ear or temporal bone malformations are a diagnostic challenge to radiologists and surgeons alike. Newer imaging techniques can detect subtle changes in middle ear and cochlear anatomy. This information is invaluable with increasing use of hearing restoration surgeries and/or cochlear implants in such patients. This article discusses the embryogenesis, classification system, and salient imaging findings of congenital outer, middle ear, and inner ear anomalies in children. Both high-resolution computerized tomography and magnetic resonance imaging scans of the temporal bones are described.