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Sample records for arteriovenous malformation case

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

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    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.

  2. Treatment of pulmonary arteriovenous malformation using platinum coils: case report

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    Kim, Man Deuk; Kim, Jeung Sook; Lim, Chang Young [College of Medicine, Pochon CHA University, Pochon (China)

    2005-07-15

    Pulmonary arteriovenous malformation (PAVM) is an abnormal direct communication between the pulmonary arteries and veins without any capillary network. The patients may be completely asymptomatic or may they develop serious complications including hemoptysis and brain abscess. We present here a case of incidentally found PAVM in a 33-year-old male who underwent embolization using platinum coils. Coil embolization is a safe, highly effective procedure that should be considered more often for the treatment of PAVM.

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

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    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.

  4. Partially trombosed glomus type spinal arteriovenous malformationcase presentation

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    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.

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

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    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.

  6. 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.

  7. Pulmonary Arteriovenous Malformations

    DEFF Research Database (Denmark)

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

    1999-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited disease with a high prevalence of pulmonary arteriovenous malformations (PAVMs). The first symptom of HHT may be stroke or fatal hemoptysis associated with the presence of PAVM....

  8. Proteus Syndrome with Arteriovenous Malformation

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    Ali Asilian

    2017-01-01

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

  9. 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.

  10. Familial Brain Arteriovenous Malformations

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    J Gordon Millichap

    2007-11-01

    Full Text Available Researchers at University Medical Centre Utrecht, the Netherlands, reviewed the literature on patients with familial brain arteriovenous malformations (BAVMs, and their age, sex, and clinical presentation were compared with those in population-based patients with sporadic BAVMs.

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

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

    2016-05-01

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

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

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    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.

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

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    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

  14. Rare malformation of glans penis: arteriovenous malformation.

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    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.

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

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    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.

  16. Cerebellar arteriovenous malformations in children

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    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.

  17. 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.

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

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    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

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

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    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.

  20. 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.

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

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    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.

  2. Pulmonary arteriovenous malformation treated by lobectomy.

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    Georghiou, Georgios P; Berman, Marius; Vidne, Bernardo A; Saute, Milton

    2003-08-01

    Pulmonary arteriovenous malformation (PAVM) may occur primarily or in association with hereditary hemorrhagic telangiectasia. We present a case of PAVM in the central lower lobe of the left lung of a 75-year-old woman, which was successfully treated by lobectomy. Contrast echocardiography is an excellent tool for evaluation of this uncommon lesion. Advances in interventional radiology have led to the introduction of obliterative techniques for the treatment of PAVM. However, in the presence of a large solitary malformation centrally located, as in our case, and in high-risk patients, surgery is still a safe and effective first option.

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

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    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.

  4. 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.

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

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    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.

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

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    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.

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

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    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

  8. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele

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    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.)

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

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    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.

  10. 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.

  11. 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.

  12. 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 ...

  13. 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.

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

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    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.

  15. 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.

  16. 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.

  17. 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.

  18. 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

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. 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.

  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. 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

    Background The major consequence of pulmonary arteriovenous malformations (PAVMs) is the direct inflow of blood from the pulmonary artery to the pulmonary vein which induces hypoxemia. Severe complications include transient ischemic attacks, paradoxical embolization in the central nervous system, massive hemoptysis or hemothorax, etc. The conventional treatment is surgical intervention. However, this can be very traumatic and dangerous. Endovascular embolization has advantages over surgery such as a faithful therapeutic effect, a low complication rate, repeatability, etc. Methods Patients (n=23) with symptomatic PAVMs underwent endovascular embolization; 11 were males and 12 were females, with ages ranging from 6 months to 58 years. During the embolization, microcoils were applied in 6 cases and standard steel coils were used in 17 cases.Results Multiple PAVMs lesions were found in 16 cases and single PAVMs lesion was found in 7 cases. Embolotherapy was carried out 28 times for 23 patients. The success rate was 100%. The results of pulmonary arteriography after treatment showed that single lesion disappeared completely while the main abnormal vessels in multiple lesions also disappeared. The mean blood oxygen saturation increased from (78.04±8.22)% to (95.13±3.67)% after the procedure. A correlated groups t test showed changes in blood oxygen saturation before and after embolization (fe=9.101, P <0.001). Symptoms of cardiac insufficiency disappeared in 5 cases and vascular murmur in the chest disappeared in 13 cases. After embolization, mild chest pain occurred in 11 cases, small amounts of pleural effusion occurred in 5 cases, and 1 patient died 2 months later because of a pyogenic infection secondary to the pulmonary infarction. Among the 22 remaining cases, with overall follow-up ranging from 18 months to 12 years, general conditions were fine, daily lives were normal and there were no neurologic symptoms or signs, except for 3 patients with diffused PAVMs who

  8. 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.

  9. 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.

  10. 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.

  11. Embolization of brain arteriovenous malformations: results of 42 cases treated with N. B. C. A. and flow-guided microcatheter

    Energy Technology Data Exchange (ETDEWEB)

    Han, Moon Hee; Chang, Kee Hyun; Kim, Dong Gyu; Lee, Sang Hyung; Han, Dae Hee [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1994-04-15

    We report the results of embolization in 42 cases of brain arteriovenous malformation and discuss the technical problems. Flow-guided microcatheter for the superselection of the feeding arteries and n-butyl cyanoacrylate as an embolic agent were used. Wire-guided microcatheter and microparticle were used in two exceptional cases. As preembolization functional evaluation, 30-50 mg of thiopental sodium solution was injected into the feeding arteries through superselected microcatheters in 40 cases with supratentorial lesions. There was no local arterial complication by injection of thiopental solution. Embolization caused a permanent neurologic deficit(mild to moderated hemiparesis) in 2 patients (4.8%) by a reflux of embolic agent or probable spasm of main arterial trunk. In 8 patients(19%), the AVMs were completely obliterated and 25 patients(60%) showed angiographic obliterations of 50-90% of the lesions. Embolization-induced venous occlusion was demonstrated at post-embolization angiography in 6 patients, and 3 patients of them showed delayed and transient neurologic deficits. Embolization of cerebral AVM can be performed effectively and safely by a superselective method and appropriate functional evaluation. Superselective thiopental sodium injection is a safe and reliable test for the evaluation of local brain function before embolization.

  12. 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...

  13. 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.

  14. 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.

  15. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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

  18. 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.

  19. 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)

  20. 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.

  1. 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.

  2. 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

  3. 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

  4. 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.

  5. 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

  6. Seizure risk from cavernous or arteriovenous malformations

    Science.gov (United States)

    Josephson, C.B.; Leach, J.-P.; Duncan, R.; Roberts, R.C.; Counsell, C.E.

    2011-01-01

    Objectives: To determine the risk of epileptic seizures due to a brain arteriovenous malformation (AVM) or cavernous malformation (CM). Methods: In a prospective population-based study of new diagnoses of AVMs (n = 229) or CMs (n = 139) in adults in Scotland in 1999–2003, we used annual medical records surveillance, general practitioner follow-up, and patient questionnaires to quantify the risk of seizures between clinical presentation and AVM/CM treatment, last follow-up, or death. Results: The 5-year risk of first-ever seizure after presentation was higher for AVMs presenting with intracranial hemorrhage or focal neurologic deficit (ICH/FND: n = 119; 23%, 95% confidence interval [CI] 9%–37%) than for incidental AVMs (n = 40; 8%, 95% CI 0%–20%), CMs presenting with ICH/FND (n = 38; 6%, 95% CI 0%–14%), or incidental CMs (n = 57; 4%, 95% CI 0%–10%). For adults who had never experienced ICH/FND, the 5-year risk of epilepsy after first-ever seizure was higher for CMs (n = 23; 94%, 95% CI 84%–100%) than AVMs (n = 37; 58%, 95% CI 40%–76%; p = 0.02). Among adults who never experienced ICH/FND and presented with or developed epilepsy, there was no difference in the proportions achieving 2-year seizure freedom over 5 years between AVMs (n = 43; 45%, 95% CI 20%–70%) and CMs (n = 35; 47%, 95% CI 27%–67%). Conclusions: AVM-related ICH confers a significantly higher risk of a first-ever seizure compared to CMs or incidental AVMs. Adults with a CM have a high risk of epilepsy after a first-ever seizure but achieve seizure freedom as frequently as those with epilepsy due to an AVM. PMID:21536634

  7. 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

  8. Giant congenital intercostal arteriovenous malformation with extensive involvement of chest wall and ribs: surgical experience.

    Science.gov (United States)

    Parashi, Hrishikesh Sukhadeo; Bhosle, Krishnarao Narayan; Thakare, Nitin Dashrath; Sharma, Ajay; Potwar, Sushrut Suhas

    2013-06-01

    Intercostal arteriovenous malformations (AVMs) are rare lesions. Review of literature shows that most reported cases are secondary to trauma or iatrogenic in origin. Congenital intercostal AVMs are extremely rare. We believe that only 1 case report of congenital intercostal arteriovenous malformation has been reported previously in the literature. We present an exceedingly rare case of giant congenital intercostal AVM in a young patient diagnosed on contrast-enhanced computed tomography of the thorax and treated by surgical resection of the involved chest wall and ribs with reconstruction of the surgical defect. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  9. 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,…

  10. 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,…

  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. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  14. 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.

  15. 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.

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

    Science.gov (United States)

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

    2014-04-01

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

  17. Spontaneous occlusion of a cerebral arteriovenous malformation after subtotal endovascular embolisation

    Directory of Open Access Journals (Sweden)

    Chiriac A.

    2014-06-01

    Full Text Available We present a case of a patient with a ruptured temporo-occipital arteriovenous malformation, grade 3 on Spetzler-Martin scale, with an unpredictable evolution. A spontaneous occlusion of the malformation was found at 4 months after the initial partial endovascular embolization. We reviewed and discuss some aspects concerning clinical and angiographic finding, the angioarchitectural remodelling and evolution of the AVMs after partial occlusion treatment.

  18. Diagnosis, management, and pathophysiology of post-Fontan hypoxaemia secondary to Glenn shunt related pulmonary arteriovenous malformation

    OpenAIRE

    Premsekar, R; Monro, J; SALMON, A

    1999-01-01

    An 8 year old child with tricuspid atresia had developed right sided pulmonary arteriovenous malformations following a previous classic Glenn procedure. These became clinically manifest immediately after Fontan conversion because of severe systemic desaturation. The pathophysiology and postoperative medical management of this case is described and related to current understanding of the aetiology of acquired pulmonary arteriovenous malformations following cavopulmonary shunt.


Keywords: clas...

  19. 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.

  20. 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...

  1. 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

  2. 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.

  3. 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.

  4. 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.

  5. 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.

  6. 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 ...

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

    Science.gov (United States)

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

    2016-01-01

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

  8. 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.

  9. 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.

  10. Intraoperative Micro-Doppler in Cerebral Arteriovenous Malformations.

    Science.gov (United States)

    Burkhardt, Till; Siasios, Giannis; Schmidt, Nils Ole; Reitz, Mathias; Regelsberger, Jan; Westphal, Manfred

    2015-11-01

    Intraoperative micro-Doppler (IOMD), intraoperative digital substraction angiography (DSA), and microscope-integrated indocyanine green angiography are methods that guide neurosurgical resection of arteriovenous malformations (AVMs) in the brain and minimize the trauma of healthy tissue. In this study we emphasize the use of IOMD in AVM surgery, analyzing the advantages and the limitations of this method. Patients and A total of 32 patients were diagnosed with an AVM. Supplying arteries and draining veins were analyzed regarding hemodynamic profiles, flow velocities, pulsatility index (PI), and resistance index (RI). Venous drainages were accompanied by arterial blood flow disturbances that showed typical characteristics in all cases. We set an angle of 60 degrees between the examined vessel and the probe to achieve a more reliable and comparable measurement. Postoperative DSA was performed in all patients. Supplying arterial blood vessels of AVMs could be identified by their characteristic blood flow profiles with PI < 0.7 and RI < 0.55. Drainage veins in all 32 cases showed normalized venous flow patterns without arterial flow turbulences at the end of the surgical procedure. Postoperative DSA revealed a residual AVM in one patient. IOMD constitutes a safe, accurate, and low-cost imaging modality for evaluating blood flow velocities and for optimal stepwise AVM elimination without unnecessary sacrifice of veins. PI and RI are reliable parameters in diagnosing cerebrovascular malformations, but systolic and diastolic flow velocities may vary to a greater extent. This phenomenon has never been elucidated previously and therefore needs to be emphasized when using this technique intraoperatively. Georg Thieme Verlag KG Stuttgart · New York.

  11. Seizure Outcomes in Patients With Surgically Treated Cerebral Arteriovenous Malformations.

    Science.gov (United States)

    von der Brelie, Christian; Simon, Matthias; Esche, Jonas; Schramm, Johannes; Boström, Azize

    2015-11-01

    Epilepsy is the second most common symptom in cerebral arteriovenous malformation (AVM) patients. The consecutive reduction of life quality is a clinically underrated problem because treatment usually focuses on the prevention of intracerebral hemorrhage. To evaluate postoperative seizure outcome with the aim of more accurate counseling for postoperative seizure outcome. From 1985 to 2012, 293 patients with an AVM were surgically treated by J.S. One hundred twenty-six patients with preoperative seizures or epilepsy could be identified; 103 of 126 had a follow-up of at least 12 months and were included in the analysis. The different epilepsy subtypes were categorized (sporadic seizures, chronic epilepsy, drug-resistant epilepsy [DRE]). Preoperative workup and surgical technique were evaluated. Seizure outcome was analyzed by using International League Against Epilepsy classification. Sporadic seizures were identified in 41% of patients (chronic epilepsy and DRE were identified in 36% and 23%, respectively). Detailed preoperative epileptological workup was done in 13%. Seizure freedom was achieved in 77% (79% at 5 years, 84% at 10 years). Outcome was significantly poorer in DRE cases. More extensive resection was performed in 11 cases with longstanding symptoms (>24 months) and resulted in better seizure outcome as well as the short duration of preoperative seizure history. Patients presenting with AVM-associated epilepsy have a favorable seizure outcome after surgical treatment. Long-standing epilepsy and the progress into DRE markedly deteriorate the chances to obtain seizure freedom and should be considered an early factor in establishing the indication for AVM removal.

  12. 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.

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

    Science.gov (United States)

    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.

  14. 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.

  15. 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.

  16. 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

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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

  2. Medical image of the week: pulmonary arteriovenous malformations

    Directory of Open Access Journals (Sweden)

    Stawter C

    2014-10-01

    Full Text Available A 34 year old woman presented to the clinic with exertional dyspnea since childhood. Oxygen saturations in clinic were 92% on room air. On review of systems she admitted to recurrent epistaxis and her daughter also suffered from frequent epistaxis. Bubble contrast echocardiography showed severe right to left shunting without evidence of intracardiac shunt (Figure 1. Computed tomography angiogram of the chest revealed multiple bilateral arteriovenous malformations (AVM’s, the largest measuring 9mm on coronal images (Figure 2. MRI brain was negative for AVM’s. She was referred to interventional radiology for microcoil embolization. She met two of four Curaçao criteria for the diagnosis of hereditary hemorrhagic telangiectasia (HHT, giving her “possible HHT”. She was referred for genetic testing to confirm the diagnosis.

  3. 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

  4. 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.

  5. Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia

    DEFF Research Database (Denmark)

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

    1999-01-01

    Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited disease with a high prevalence of pulmonary arteriovenous malformations (PAVMs). The first symptom of HHT may be stroke or fatal hemoptysis associated with the presence of PAVM....

  6. Chronic Encapsulated Expanding Thalamic Hematoma Associated with Obstructive Hydrocephalus following Radiosurgery for a Cerebral Arteriovenous Malformation: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Jun Takei

    2016-01-01

    Full Text Available Chronic encapsulated intracerebral hematoma is a unique type of intracerebral hematoma accompanied by a capsule that is abundant in fragile microvasculature occasionally causing delayed regrowth. A 37-year-old man who had undergone radiosurgery for an arteriovenous malformation (AVM causing intracerebral hematoma in the left parietal lobe presented with headache, vomiting, and progressive truncal ataxia due to a cystic lesion that had been noted in the left thalamus, leading to progressive obstructive hydrocephalus. He underwent left frontal craniotomy via a transsylvian fissure approach, and the serous hematoma was aspirated. The hematoma capsule was easy to drain and was partially removed. Pathological findings demonstrated angiomatous fibroblastic granulation tissue with extensive macrophage invasion. The concentration of vascular endothelial growth factor (VEGF was high in the hematoma (12012 pg/mL. The etiology and pathogenesis of encapsulated hematoma are unclear, but the gross appearance and pathological findings are similar to those of chronic subdural hematoma. Based on the high concentration of VEGF in the hematoma, expansion of the encapsulated hematoma might have been caused by the promotion of vascular permeability of newly formed microvasculature in the capsule.

  7. 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.

  8. 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.

  9. 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.

  10. 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

  11. 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.

  12. 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.

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

    Science.gov (United States)

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

    2016-06-01

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

  14. Sarcoidosis with arteriovenous malformation in a 15 year old girl—the rarest of the rare

    Directory of Open Access Journals (Sweden)

    Iman eQaiser

    2015-09-01

    Full Text Available Introduction: Sarcoidosis is an uncommon multi-system disorder with many possible complications. Arteriovenous malformations are a rare vascular complication of sarcoidosis.Case Description: A 15 year old girl presented to the Pediatric Clinic at AKUH with pulmonary, hepatic, joint and skin manifestations. Physical examination and investigations pointed towards sarcoidosis, including raised ESR, ACE and ALT. An incidental finding of pulmonary arteriovenous malformation (PAVM was noticed on echocardiography. She responded to oral corticosteroids, her ACE and ALT levels improved. There was lack of indication for pulmonary angio-embolization for her PAVM. On 4-year follow up, her condition improved and she is clinically well.Discussion: PAVM is an extremely rare complication of sarcoidosis, especially among the pediatric population. Hence, this is the first reported case of its kind. The relation between sarcoidosis and PAVM is difficult to establish, however, there are some theories. This condition may be treated depending on the symptoms. Since our patient did not have any significant symptoms of PAVM, she was treated for the underlying disease, i.e. sarcoidosis.Conclusion: While dealing with patients having multi-system disorders like sarcoidosis, one must be very vigilant so as not to miss out on any complication. Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

  15. 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

  16. 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.

  17. Cerebral Arteriovenous Malformation Flow Is Associated With Venous Intimal Hyperplasia.

    Science.gov (United States)

    Shakur, Sophia F; Hussein, Ahmed E; Amin-Hanjani, Sepideh; Valyi-Nagy, Tibor; Charbel, Fady T; Alaraj, Ali

    2017-04-01

    The pathogenesis of venous intimal hyperplasia and venous outflow stenosis associated with cerebral arteriovenous malformation (AVM) draining veins is poorly understood. We sought to determine the relationship between maximum vein wall thickness and AVM flow. Patients who underwent AVM surgical resection and had flow measured before treatment using quantitative magnetic resonance angiography were retrospectively reviewed. Specimens were mounted on slides and stained with elastin special stain. Perinidal veins were identified, and maximum wall thickness was measured from digitized images. Relationship between maximum vein wall thickness and AVM flow was assessed. Twenty-eight patients were included. Spearman correlation revealed a statistically significant relationship between maximum vein wall thickness and total AVM flow (ρ=+0.51; P=0.006), AVM flow per draining vein (ρ=+0.41; P=0.03), and mean intranidal vessel diameter (ρ=+0.39; P=0.04). Maximum vein wall thickness increases with higher total AVM flow and AVM flow per draining vein. This finding implicates chronically high AVM inflow in venous intimal hyperplasia. © 2017 American Heart Association, Inc.

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

    Science.gov (United States)

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

    2014-04-01

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

  19. Pulmonary Arteriovenous Fistula: Clinical and Histologic Spectrum of Four Cases

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    Soomin Ahn

    2016-09-01

    Full Text Available Pulmonary arteriovenous fistula (PAVF is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female and 2 (a 54-old-female presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.

  20. Recombinant factor VII (NovoSeven in intraoperative blood saving during neurosurgical treatment of the brain arteriovenous malformation

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    Novak Vesna

    2007-01-01

    Full Text Available Background. Cerebral arteriovenous (AV malformation causes, due to the increased blood flow through a malformation, a massive intraoperative bleeding complicating, so, surgical treatment. The use of intraoperative blood saving apparatus during surgery and a recombinant factor VII-a (NovoSeven significantly reduce complications during surgical treatment. Case report. We reported a case of surgical treatment of the patient with AV malformation of IV stage according to the Spetzler-Martin scale, in the brain. Due to a possible heavy bleeding we used a apparatus for intrasurgical blood recovery, Cell Saver, Sequestra 1 000, Medtronic, U.S.A., and recombinant human factor VIIa (rFVIIa - NovoSeven, NovoNordisk, Denmark to control bleeding and restore an adequate hemostasis. Conclusion. The use of an apparatus for intraoperative blood saving, as well as the NovoSeven preparation in the management of AV malformation of IV stage, showed to be successful.

  1. Fatal rupture of pulmonary arteriovenous malformation in hereditary haemorrhagic telangiectasis and severe PAH

    Directory of Open Access Journals (Sweden)

    D. Montani

    2009-03-01

    Full Text Available 29-yr-old patient with hereditary haemorrhagic telangiectasia was referred to the present authors’ centre with progressive exertional dyspnoea. Pulmonary arterial hypertension (PAH was suspected on Doppler echocardiography and confirmed by right heart catheterisation demonstrating severe PAH. Genetic analysis found an activin receptor-like kinase-1 gene missense mutation. Chest radiography and computed tomodensitometry of the chest revealed a pulmonary arteriovenous malformation with a 5-mm diameter feeding artery in the right lower lobe. Embolisation of the arteriovenous malformation was discussed, but was considered a very high-risk procedure that could aggravate PAH and was therefore not performed. Haemodynamics were improved by dual endothelin receptor antagonist and inhaled iloprost but the patient subsequently died suddenly of a rupture of the arteriovenous malformation into the pleural cavity. Severe PAH is generally considered a contraindication to performing pulmonary arteriovenous malformation embolisation because of the risk of worsening of PAH. However, given the significant risk of rupture, paradoxical embolism and haemoptysis, and the lack of data regarding the evolution of pulmonary pressure after embolisation in PAH, pulmonary arteriovenous malformation embolisation should not be absolutely contraindicated and might be considered in patients with stable PAH.

  2. 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....

  3. Radiosurgery with linac based photon knife in cerebral arteriovenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jin Hee; Choi, Tae Jin [College of Medicine, Keimyung Univ., Taegu (Korea, Republic of)

    2003-03-01

    The purpose of this study was to analyze the effect of a linear accelerator based Photon Knife Radiosurgery System developed by the staff of Keimyung University Dongsan Medical Center for the treatment of cerebral arteriovenous malformation. Between December 1993 and October 2000, 30 patients with cerebral arteriovenous malformation (AVM) were treated with the Linac based Photon Knife Radiosurgery System in the Department of Therapeutic Radiology at Keimyung University Dongsan Medical Center. The median age was 34, ranging from 7 to 63 years, with a 2: 1 male to female ratio. The locations of the AVM nidi were the frontal lobe (motor cortex), parietal lobe, and the thalamus, in that order. The diameters of the AVM nidi ranged 1.2 to 5.5 cm with a mean of 2.9 cm, and target volumes of between 0.5 and 20.6 cc, with a mean of 6.8 cc. The majority of patients received radiation doses of between 1,500 and 2,500 cGy, with a mean of 2,000 cGy, at 8O% the isodose line. Twenty-five patients were treated with one isocenter, 4 with two, and 1 with four. The follow-up radiological evaluations were performed with cranial computed tomogram (CT) or MRI between 6 month and one year interval, and if the AVM nidus had completely disappeared in the CT or MRI, we confirmed this was a complete obliteration, with a cerebral or magnetic resonance angiogram (MRA). The median follow-up period was 39 months with a range of 10 to 103 months. Twenty patients were radiologically followed up for over 20 months, with complete obliteration observed in 14 (70%). According to the maximal diameter. all four of the small AVM (<2 cm) completely obliterated, 8 of the 10 patients with a medium A VM (2 - 3 cm) showed a complete obliteration, and two showed partial obliteration, Among the patients with a large AVM (>3 cm), only one showed complete obliteration, and 5 showed partial obliteration, but 3 of these underwent further radiosurgery 3 years later. One who followed up for 20 months following

  4. Scattered depressions with temporal preponderance in visual field test coexisting with optic disc temporal atrophy in cerebral arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    Gokce G

    2013-10-01

    Full Text Available Gokcen Gokce,1 Nisa Cem Oren,2 Osman Melih Ceylan,3 Tarkan Mumcuoglu,3 Volkan Hurmeric3 1Sarikamis Military Hospital, Department of Ophthalmology, 2Department of Radiology, Kars, 3Gulhane Military Medical Academy, Department of Ophthalmology, Ankara, Turkey Abstract: In this article, the unusual association of optic disc temporal atrophy associated with scattered depressions with temporal preponderance in visual field test resembling incomplete bitemporal hemianopsia is reported. A 22-year-old man with cerebral arteriovenous malformation (AVM, which was located adjacent to the inferomedial portion of the posterior limb of the right internal capsule at the level of lateral ventricle, revealed interesting and unexpected ophthalmological findings. Possible mechanisms including anatomical variant, previously larger AVM, and retrograde optic neuropathy were mentioned. This case also highlighted that the usual complaint of visual disturbance might associate with unusual visual field defect in cerebral AVMs. Keywords: arteriovenous malformation, bitemporal hemianopsia, optic chiasm, optic nerve, vascular steal

  5. 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).

  6. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

    Science.gov (United States)

    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

  7. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.)

  9. 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.

  10. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.).

  13. 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.

  14. 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

  15. 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.

  16. 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.

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

    Institute of Scientific and Technical Information of China (English)

    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.

  18. 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.

  19. 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.

  20. 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.

  1. 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

  2. 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.

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

    DEFF Research Database (Denmark)

    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....

  4. 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). Copyright © 2014 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  5. 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.)

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

    Science.gov (United States)

    Cabrilo, Ivan; Bijlenga, Philippe; Schaller, Karl

    2014-09-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1989-12-01

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

  8. Therapeutic management of pregnant women with intracranial haemorrhage related to most commonly occurring arteriovenous malformations

    Directory of Open Access Journals (Sweden)

    Adrianna Jarych

    2016-03-01

    Full Text Available Intracranial haemorrhage in pregnancy poses a serious threat to both the mother and the foetus. It is associated with a high mortality and it is the third among non-obstetric causes of death in this group of patients, accounting for 5–12% of all deaths of the pregnant. Vascular malformations, mainly intracranial aneurysms and arteriovenous malformations observed also in the general population, are the most common cause of bleeding in pregnant women. So far, there have been no clear guidelines for the treatment of pregnant women with intracranial haemorrhage in the course of the above mentioned malformations, therefore the objective of this study is to summarize the knowledge about the specificity of therapeutic management in those cases. The study discusses the location of vascular lesions, physiological processes in a pregnant woman that must be taken into consideration during a therapeutic intervention, the problem of selecting the treatment (surgical treatment, endovascular therapy, radiosurgery, method and time of delivery, the use of ionizing radiation and drugs during endovascular procedures. In the available literature a special attention is paid to the mother’s welfare during the undertaken treatment, including the maintenance of normal uteroplacental perfusion, which has an effect on the general condition of the foetus. Therefore, it is essential to know the discussed physiological changes in a pregnant women as they condition specific therapeutic management. The safety of applied endovascular procedures in pregnant women is equally important due to the use of both ionizing radiation and certain medications. It is believed that these procedures are acceptable in pregnant women when appropriate radiological protection is used, radiation doses are reduced and medications are selected properly. However, surgical intervention is still considered to be the main method of treatment in this group of patients. The decision about the

  9. 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.

  10. 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.

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

    Science.gov (United States)

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

    2016-06-01

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

  12. 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.

  13. 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.)

  14. Micromultileaf collimator-based stereotactic radiosurgery for selected arteriovenous malformations: Technique and preliminary experience

    Directory of Open Access Journals (Sweden)

    Jalali Rakesh

    2009-01-01

    Full Text Available Purpose : To report our experience of stereotactic radiosurgery (SRS in consecutively treated patients with arteriovenous malformations (AVMs. Materials and Methods : Of the 87 patients, 23 patients qualified and were treated with SRS as per predefined protocol according to AVM size, location, neurological status, prior bleeding, and the AVM score. All had Spletzer-Martin grade II/III and AVM scores < 2.5. Patients underwent SRS using micromultileaf collimators delivering multiple noncoplanar fixed fields. Doses were prescribed using the Flickinger model. Patients were followed up with magnetic resonance angiography (MRA and digitally subtracted angiography (DSA. Results : The mean nidus volume was 3.65 cc. The mean prescribed maximum dose was 22 Gy and the marginal dose was 19.24 Gy; 12 Gy normal brain volume was 8.39 cc and 12 Gy marginal volume was 5.03 cc. Mean dose to brain stem, pituitary hypothalamic axis, and optic chiasm was 2.5, 0.72, and 0.49 Gy, respectively. At a median follow-up of 22 months (range 1.5-71.2 months, 7 of 10 patients presenting with a neurological deficit showed significant improvement. All 15 patients who underwent MRA 1.5-2 years after SRS had no residual nidus yielding an MRA complete obliteration rate of 100%. Twelve patients also underwent a check DSA, which confirmed obliteration in 11 of them resulting in an accuracy of MRA of 92%. One patient after SRS had transient deterioration of motor power, which resolved completely after a short course of steroids and another had mild worsening of the hemiparesis. All patients are able to lead an active functional life. Conclusions : Careful selection of cases suitable for SRS provides optimum obliteration rates with low toxicity.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  16. 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

  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. 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

  19. Management of Brain Arteriovenous Malformations: A Scientific Statement for Healthcare Professionals From the American Heart Association/American Stroke Association.

    Science.gov (United States)

    Derdeyn, Colin P; Zipfel, Gregory J; Albuquerque, Felipe C; Cooke, Daniel L; Feldmann, Edward; Sheehan, Jason P; Torner, James C

    2017-08-01

    The aim of this statement is to review the current data and to make suggestions for the diagnosis and management of both ruptured and unruptured brain arteriovenous malformations. The writing group met in person and by teleconference to establish search terms and to discuss narrative text and suggestions. Authors performed their own literature searches of PubMed, Medline, or Embase, specific to their allocated section, through the end of January 2015. Prerelease review of the draft statement was performed by expert peer reviewers and by the members of the Stroke Council Scientific Oversight Committee and Stroke Council Leadership Committee. The focus of the scientific statement was subdivided into epidemiology; diagnosis; natural history; treatment, including the roles of surgery, stereotactic radiosurgery, and embolization; and management of ruptured and unruptured brain arteriovenous malformations. Areas requiring more evidence were identified. Brain arteriovenous malformations are a relatively uncommon but important cause of hemorrhagic stroke, especially in young adults. This statement describes the current knowledge of the natural history and treatment of patients with ruptured and unruptured brain arteriovenous malformations, suggestions for management, and implications for future research. © 2017 American Heart Association, Inc.

  20. 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.

  1. Ruptured Arteriovenous Malformation Presenting with Kernohan’s Notch

    Directory of Open Access Journals (Sweden)

    Christopher F. Dibble

    2015-01-01

    Full Text Available AVMs are congenital lesions that predispose patients to intracranial hemorrhage and resultant neurological deficits. These deficits are often focal and due to the presence of local neurologic disruption from hemorrhage in the contralateral cerebral hemisphere. We present a rare case of a patient with ipsilateral neurological deficits due to Kernohan’s Notch phenomenon resulting from hemorrhage from an AVM. A 31-year-old woman with seizures underwent MR and angiographic imaging which confirmed an unruptured left parietal AVM. The patient declined treatment and presented with obtundation 4 years later. Imaging revealed an acute left parietal ICH and SDH with significant mass effect. The patient underwent emergent hemicraniectomy and hematoma evacuation. Postoperatively, she made significant improvement and was following commands contralaterally with ipsilateral hemiplegia. MR imaging revealed right Kernohan’s Notch. The patient had significant rehabilitation with neurological improvement. She eventually underwent elective embolization followed by subsequent surgical resection and bone replacement. Three years from the initial hemorrhage, the patient had only mild left-sided weakness and ambulates without assistance. A false localizing sign, Kernohan’s Notch phenomenon, should be considered in the setting of AVM hemorrhage with paradoxical motor impairment and can be identified through MRI.

  2. 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 ...

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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.

  6. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Andisheh, Bahram; Mavroidis, Panayiotis; Brahme, Anders; Lind, Bengt K [Department of Medical Radiation Physics, Karolinska Institutet and Stockholm University, Stockholm (Sweden); Bitaraf, Mohammad A [Department of Neurosurgery, Tehran University of Medical Sciences and Iran Gamma Knife Center, Tehran (Iran, Islamic Republic of)

    2010-04-07

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

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

    Science.gov (United States)

    Akhigbe, T; Zolnourian, A; Bulters, D

    2017-05-01

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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  11. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    Hattangadi-Gluth, Jona A. [Department of Radiation Medicine and Applied Sciences, University of California San Diego, San Diego, California (United States); Chapman, Paul H. [Department of Neurosurgery, Massachusetts General Hospital, Boston, Massachusetts (United States); Kim, Daniel; Niemierko, Andrzej; Bussière, Marc R.; Stringham, Alison; Daartz, Juliane [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Ogilvy, Christopher [Department of Neurosurgery, Beth Israel Deaconess Medical Center, Boston, Massachusetts (United States); Loeffler, Jay S. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Shih, Helen A., E-mail: hshih@partners.org [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2014-06-01

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

  13. Delayed angiography in the investigation of intracerebral hematomas caused by small arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Willinsky, R.A. (Dept. of Radiology, Toronto Hospital, Western Div., Toronto, ON (Canada) Univ. of Toronto, Brain Vascular Malformation Study Group, ON (Canada)); Fitzgerald, M. (Dept. of Radiology, Toronto Hospital, Western Div., Toronto, ON (Canada)); TerBrugge, K. (Dept. of Radiology, Toronto Hospital, Western Div., Toronto, ON (Canada) Univ. of Toronto, Brain Vascular Malformation Study Group, ON (Canada)); Montanera, W. (Dept. of Radiology, Toronto Hospital, Western Div., Toronto, ON (Canada)); Wallace, M. (Div. of Neurosurgery, Dept. of Surgery, Toronto Hospital, Western Div., ON (Canada) Univ. of Toronto, Brain Vascular Malformation Study Group, ON (Canada))

    1993-04-01

    We reviewed the clinical and radiological features of ten patients with small arteriovenous malformations that caused intracerebral hematomas. In six patients, angiography showed a small nidus (less than 1 cm in diameter) with a shunt at the site of the hematoma, and in four only an early-filling vein was evident. Six patients had only delayed angiography (4 weeks or more after the ictus). In three, angiography within 2 days of the ictus failed to reveal the cause of the bleed, but repeat angiography showed an early-filling vein in two, and a nidus with shunting in one. In only one patient did early angiography reveal the malformation. MRI was obtained in eight patients, and in two prominent vessels were evident in the wall of the hematoma cavity. In investigation of an unexplained intracerebral hematoma, MRI may be useful to exclude a neoplasm or cavernoma, although the latter may be not be evident in the presence of a recent hematoma. We suggest early MRI and angiography for investigation of an unexplained, nonhypertensive intracerebral bleed, with follow-up MRI and dealyed angiography if the initial studies fail to reveal the cause. (orig.)

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  16. Extravascular injection of sclerotic agents does not affect vessels in the rat: experimental implications for percutaneous sclerotherapy of arteriovenous malformations.

    Science.gov (United States)

    Sato, D; Kurita, M; Ozaki, M; Kaji, N; Takushima, A; Harii, K

    2012-07-01

    Sclerotherapy is useful for the treatment of arteriovenous vascular malformations. However, intravascular administration of sclerotic agents into small arteriovenous niduses is often difficult. Extravascular administration of sclerotic agents causes reduction of vascular flow on Doppler echo during clinical sclerotherapy. Therefore, we aimed to investigate whether the extravascular injection of sclerotic agents affects tiny vessels. Animal study. The effect of extravascular injection of sclerotic agents on vessels was investigated using rat femoral and superficial inferior epigastric vessels. After surgical exposure of vessels, absolute ethanol, 5% ethanolamine oleate and 3% polidocanol were injected into perivascular surrounding tissues, and their effect on vessels was evaluated after 14 days using histology and coloured silicone rubber injection. The integrity of the vascular lumen, endothelial cells and vascular patency were not affected by injection of sclerotic agents. Attenuation of vascular flow of an arteriovenous shunt after extravascular injection of sclerotic agents is transient and/or trivial and does not cause disruption of vessels. Therefore, sclerotic agents should be delivered to obtain sufficient destruction of arteriovenous malformation lesions and blood flow. Copyright © 2012 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

  17. Phosphatidylserine Translocation after Radiosurgery in an Animal Model of Arteriovenous Malformation.

    Science.gov (United States)

    Raoufi Rad, Newsha; McRobb, Lucinda S; Zhao, Zhenjun; Lee, Vivienne S; Patel, Nirav J; Qureshi, Anas Sarwar; Grace, Michael; McHattan, Joshua J; Amal Raj, Jude V; Duong, Hong; Kashba, Saleh R; Stoodley, Marcus A

    2017-06-01

    Phosphatidylserine (PS) is asymmetrically distributed across the plasma membrane, located predominantly on the inner leaflet in healthy cells. Translocation of PS to the outer leaflet makes it available as a target for biological therapies. We examined PS translocation after radiosurgery in an animal model of brain arteriovenous malformation (AVM). An arteriovenous fistula was created by end-to-side anastomosis of the left external jugular vein to the common carotid artery in 6-week-old, male Sprague Dawley rats. Six weeks after AVM creation, 15 rats underwent Gamma Knife stereotactic radiosurgery receiving a single 15 Gy dose to the margin of the fistula; 15 rats received sham treatment. Externalization of PS was examined by intravenous injection of a PS-specific near-infrared probe, PSVue-794, and in vivo fluorescence optical imaging at 1, 7, 21, 42, 63 and 84 days postirradiation. Fluorescent signaling indicative of PS translocation to the luminal cell surface accumulated in the AVM region, in both irradiated and nonirradiated animals, at all time points. Fluorescence was localized specifically to the AVM region and was not present in any other anatomical sites. Translocated PS increased over time in irradiated rats (P < 0.001) but not in sham-irradiated rats and this difference reached statistical significance at day 84 (P < 0.05). In summary, vessels within the mature rat AVM demonstrate elevated PS externalization compared to normal vessels. A single dose of ionizing radiation can increase PS externalization in a time-dependent manner. Strict localization of PS externalization within the AVM region suggests that stereotactic radiosurgery can serve as an effective priming agent and PS may be a suitable candidate for vascular-targeting approaches to AVM treatment.

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

    Science.gov (United States)

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

    2017-07-06

    Radiosurgery is an established technique to treat cerebral arteriovenous malformations (AVMs). Obliteration of larger AVMs (> 10-15 cm(3) or diameter > 3 cm) in a single session is challenging with current radiosurgery platforms due to toxicity. We present a novel technique of multistage stereotactic radiosurgery (SRS) for large intracranial arteriovenous malformations (AVM) using the Gamma Knife system. Eighteen patients with large (> 10-15 cm(3) or diameter > 3 cm) AVMs, which were previously treated using a staged SRS technique on the Cyberknife platform, were retrospectively selected for this study. The AVMs were contoured and divided into 3-8 subtargets to be treated sequentially in a staged approach at half to 4 week intervals. The prescription dose ranged from 15 Gy to 20 Gy, depending on the subtarget number, volume, and location. Gamma Knife plans using multiple collimator settings were generated and optimized. The coordinates of each shot from the initial plan covering the total AVM target were extracted based on their relative positions within the frame system. The shots were regrouped based on their location with respect to the subtarget contours to generate subplans for each stage. The delivery time of each shot for a subtarget was decay corrected with (60) Co for staging the treatment course to generate the same dose distribution as that planned for the total AVM target. Conformality indices and dose-volume analysis were performed to evaluate treatment plans. With the shot redistribution technique, the composite dose for the multistaged treatment of multiple subtargets is equivalent to the initial plan for total AVM target. Gamma Knife plans resulted in an average PTV coverage of 96.3 ± 0.9% and a PITV of 1.23 ± 0.1. The resulting Conformality indices, V12Gy and R50 dose spillage values were 0.76 ± 0.05, 3.4 ± 1.8, and 3.1 ± 0.5 respectively. The Gamma Knife system can deliver a multistaged conformal dose to treat large AVMs when correcting for

  19. Volume-staged radiosurgery for large arteriovenous malformations: an evolving paradigm.

    Science.gov (United States)

    Seymour, Zachary A; Sneed, Penny K; Gupta, Nalin; Lawton, Michael T; Molinaro, Annette M; Young, William; Dowd, Christopher F; Halbach, Van V; Higashida, Randall T; McDermott, Michael W

    2016-01-01

    OBJECT Large arteriovenous malformations (AVMs) remain difficult to treat, and ideal treatment parameters for volume-staged stereotactic radiosurgery (VS-SRS) are still unknown. The object of this study was to compare VS-SRS treatment outcomes for AVMs larger than 10 ml during 2 eras; Era 1 was 1992-March 2004, and Era 2 was May 2004-2008. In Era 2 the authors prospectively decreased the AVM treatment volume, increased the radiation dose per stage, and shortened the interval between stages. METHODS All cases of VS-SRS treatment for AVM performed at a single institution were retrospectively reviewed. RESULTS Of 69 patients intended for VS-SRS, 63 completed all stages. The median patient age at the first stage of VS-SRS was 34 years (range 9-68 years). The median modified radiosurgery-based AVM score (mRBAS), total AVM volume, and volume per stage in Era 1 versus Era 2 were 3.6 versus 2.7, 27.3 ml versus 18.9 ml, and 15.0 ml versus 6.8 ml, respectively. The median radiation dose per stage was 15.5 Gy in Era 1 and 17.0 Gy in Era 2, and the median clinical follow-up period in living patients was 8.6 years in Era 1 and 4.8 years in Era 2. All outcomes were measured from the first stage of VS-SRS. Near or complete obliteration was more common in Era 2 (log-rank test, p = 0.0003), with 3- and 5-year probabilities of 5% and 21%, respectively, in Era 1 compared with 24% and 68% in Era 2. Radiosurgical dose, AVM volume per stage, total AVM volume, era, compact nidus, Spetzler-Martin grade, and mRBAS were significantly associated with near or complete obliteration on univariate analysis. Dose was a strong predictor of response (Cox proportional hazards, p < 0.001, HR 6.99), with 3- and 5-year probabilities of near or complete obliteration of 5% and 16%, respectively, at a dose < 17 Gy versus 23% and 74% at a dose ≥ 17 Gy. Dose per stage, compact nidus, and total AVM volume remained significant predictors of near or complete obliteration on multivariate analysis. Seventeen

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

    Science.gov (United States)

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

    2015-05-01

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

  1. [Hyperintensity on T2 MRI and size as predictors of obliteration in radiosurgically treated arteriovenous malformations].

    Science.gov (United States)

    Arrese Regańón, Ignacio; Alday, R; González, P A; Campollo, J; Ramos, A; Domínguez, P; Fernández-Letón, J P; Pérez-Núñez, A; Lobato, R D

    2009-04-01

    The aim of this study was to determine which factors were statistically related to radiological and clinical outcomes following radiosurgical treatment of arteriovenous malformations (AVMs). The data of 59 patients receiving radiosurgical treatment at our department were retrospectivelly reviewed. Different clinical and biological data, including Spetzler-Martin grade, the presentation of symptoms, radiation dose, number of isocenters and both radiological and clinical outcome, were subjected to multivariate analysis. AVM obliteration was achieved in 77% of patients, the majority of them occurring between 3-5 years after treatment. Ten patients (17%) showed either acute or delayed complications. Only one patient died due tor hemorrhage during the follow-up after radiosurgery. A multivariate analysis showed that, hyperintensity on T2 MRI and a nidus smaller than 3 cm were the only factors statistically related to oclusion of the AVM (p=0.03 and p=0.05, respectively). The nidus size and the development of hyperintensity on T2 MRI after the treatment were the strongest predictive factors of obliteration in our series of AVMs radiosurgically treated. Moreover, given that many AVMs showed complete obliteration between 3-5 years after treatment, we recommend to wait untill 5 years after treatment before considering a new terapeuthic approach in patients showing small residual nidus at control imaging.

  2. Exome sequencing identifies a novel intronic mutation in ENG that causes recurrence of pulmonary arteriovenous malformations.

    Science.gov (United States)

    Saji, Naoki; Kawarai, Toshitaka; Miyamoto, Ryosuke; Sato, Takahiro; Morino, Hiroyuki; Orlacchio, Antonio; Oki, Ryosuke; Kimura, Kazumi; Kaji, Ryuji

    2015-05-15

    Hereditary hemorrhagic telangiectasia (HHT) occasionally can be discovered in patients with cerebrovascular disease. Pulmonary arteriovenous malformation (PAVM) is one of the complications in HHT and occasionally is causative for life-threatening embolic stroke. Several genetic defects have been reported in patients with HHT. The broad spectrum of phenotype and intrafamilial phenotype variations, including age-at-onset of vascular events, often make an early diagnosis difficult. We present here a Japanese family with a novel intronic heterozygous mutation of ENG, which was identified using whole exome sequencing (WES). The intronic mutation, IVS3+4delAGTG, results in in-frame deletion of exon 3 and would produce a shorter ENG protein lacking the extracellular forty-seven amino acid sequences, which is located within the orphan domain. Our findings highlight the importance of the domain for the downstream signaling pathway of transforming growth factor-beta and bone morphogenesis protein superfamily receptors. Considering the phenotype variations and the available treatment for vascular complications, an early diagnosis using genetic testing, including WES, should be considered for individuals at risk of HHT. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. 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...

  4. 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.

  5. 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.

  6. 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)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  8. 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

  9. 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)

  10. 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.)

  11. Superselective Intra-Arterial Ethanol Sclerotherapy of Feeding Artery and Nidal Aneurysms in Ruptured Cerebral Arteriovenous Malformations.

    Science.gov (United States)

    Settecase, F; Hetts, S W; Nicholson, A D; Amans, M R; Cooke, D L; Dowd, C F; Higashida, R T; Halbach, V V

    2016-04-01

    In the endovascular treatment of cerebral arteriovenous malformations, ethanol sclerotherapy is seldom used due to safety concerns. However, when limited reflux of an embolic agent is permissible or when there is a long distance to the target, ethanol may be preferable. We reviewed 10 patients with 14 cerebral AVM feeding artery aneurysms or intranidal aneurysms treated with intra-arterial ethanol sclerotherapy at our institution between 2005 and 2014. All patients presented with acute intracranial hemorrhage. Thirteen of 14 aneurysms were treated primarily with 60%-80% ethanol into the feeding artery. Complete target feeding artery and aneurysm occlusion was seen in all cases; 8/13 (62%) were occluded by using ethanol alone. No retreatments or recurrences were seen. One permanent neurologic deficit (1/13, 7.7%) and no deaths occurred. In a subset of ruptured cerebral AVMs, ethanol sclerotherapy of feeding artery aneurysms and intranidal aneurysms can be performed with a high degree of technical success and a low rate of complication. © 2016 by American Journal of Neuroradiology.

  12. 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.

  13. [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.

  14. 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)

  15. Headway Duo microcatheter for cerebral arteriovenous malformation embolization with n-BCA.

    Science.gov (United States)

    Heit, Jeremy J; Faisal, Abigail G S; Telischak, Nicholas A; Choudhri, Omar; Do, Huy M

    2016-11-01

    Cerebral arteriovenous malformations (AVMs) are uncommon vascular lesions, and hemorrhage secondary to AVM rupture results in significant morbidity and mortality. AVMs may be treated by endovascular embolization, and technical advances in microcatheter design are likely to improve the success and safety of endovascular embolization of cerebral AVMs. To describe our early experience with the Headway Duo microcatheter for embolization of cerebral AVMs with n-butyl-cyanoacrylate (n-BCA). Consecutive patients treated by endovascular embolization of a cerebral AVM with n-BCA delivered intra-arterially through the Headway Duo microcatheter (167 cm length) were identified. Patient demographic information, procedural details, and patient outcome were determined from electronic medical records. Ten consecutive patients undergoing cerebral AVM embolization using n-BCA injected through the Headway Duo microcatheter were identified. Presenting symptoms included headache, hemorrhage, seizures, and weakness. Spetzler Martin grades ranged from 1 to 5, and AVMs were located in the basal ganglia (2 patients), parietal lobe (4 patients), frontal lobe (1 patient), temporal lobe (1 patient), an entire hemisphere (1 patient), and posterior fossa (1 patient). 50 arterial pedicles were embolized, and all procedures were technically successful. There was one post-procedural hemorrhage that was well tolerated by the patient, and no other complications occurred. Additional AVM treatment was performed by surgery and radiation therapy. The Headway Duo microcatheter is safe and effective for embolization of cerebral AVMs using n-BCA. The trackability and high burst pressure of the Headway Duo make it an important and useful tool for the neurointerventionalist during cerebral AVM embolization. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  16. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    Science.gov (United States)

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

    2016-03-01

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

  19. 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.

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

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    A Raghunath

    2016-01-01

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

  1. 357 Gamma Knife Stereotactic Radiosurgery in the Management of Large Cerebral Arteriovenous Malformations.

    Science.gov (United States)

    Singh, Manmohan; Aggarwal, Deepak; Kale, Shashank Sharad

    2016-08-01

    Large brain arteriovenous malformations (AVMs) pose management challenge in neurosurgical practice. Management remains conservative unless these AVMs bleed or present with neurological deficits. For patients who require treatment, options are limited with very high treatment-related morbidity. Gamma knife radiosurgery remains an excellent noninvasive option for such AVMs. This is a retrospective study of 74 patients with large AVMs who were treated with gamma knife radiosurgery over 16 years. Sixty-five patients were treated with single-fraction and 9 patients were treated with volume-staged gamma knife radiosurgery. Average follow-up was 4.2 years. In the single-fraction group, 90% patients had SM grade 3 and 4 AVMs while 10% patients had SM grade 5 AVMs. Mean marginal radiation dose was 23.66 Gy. Overall obliteration rate of 66% was observed. Four percent of patients rebled in this group. Postradiation edema was observed in 18% of patient who presented with headache, new-onset seizures, and motor deficits. The symptoms improved with steroid therapy in most patients. Delayed cyst formations were seen in 2 patients. Interestingly, 41% patients showed seizure control after gamma knife radiosurgery. In volume-staging group, 60% patients had SM grade 4 AVMs and 40% patients has SM grade 5 AVMs. All patients were treated in 2 sittings. Average marginal dose used was 23 Gy. These patients showed nearly 80% reduction in the nidus volume at follow-up. No new-onset deficit was observed in this group, and all patients tolerated the treatment very well. Large-volume AVMs can be managed with gamma knife radiosurgery with acceptable risks. Single-fraction gamma knife radiosurgery should be considered for moderate-size AVMs away from critical structures. Large-volume lesions should be treated with volume-staged gamma knife radiosurgery.

  2. 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

  3. Retrospective study of long-term outcome after brain arteriovenous malformation rupture: the RAP score.

    Science.gov (United States)

    Shotar, Eimad; Debarre, Matthieu; Sourour, Nader-Antoine; Di Maria, Federico; Gabrieli, Joseph; Nouet, Aurélien; Chiras, Jacques; Degos, Vincent; Clarençon, Frédéric

    2017-01-20

    OBJECTIVE The authors aimed to design a score for stratifying patients with brain arteriovenous malformation (BAVM) rupture, based on the likelihood of a poor long-term neurological outcome. METHODS The records of consecutive patients with BAVM hemorrhagic events who had been admitted over a period of 11 years were retrospectively reviewed. Independent predictors of a poor long-term outcome (modified Rankin Scale score ≥ 3) beyond 1 year after admission were identified. A risk stratification scale was developed and compared with the intracranial hemorrhage (ICH) score to predict poor outcome and inpatient mortality. RESULTS One hundred thirty-five patients with 139 independent hemorrhagic events related to BAVM rupture were included in this analysis. Multivariate logistic regression followed by stepwise analysis showed that consciousness level according to the Glasgow Coma Scale (OR 6.5, 95% CI 3.1-13.7, p RAP) score, was a stronger predictor of a poor long-term outcome (area under the receiver operating characteristic curve [AUC] 0.87, 95% CI 0.8-0.92, p = 0.009) and inpatient mortality (AUC 0.91, 95% CI 0.85-0.95, p = 0.006) than the ICH score. For a RAP score ≥ 6, sensitivity and specificity for predicting poor outcome were 76.8% (95% CI 63.6-87) and 90.8% (95% CI 81.9-96.2), respectively. CONCLUSIONS The authors propose a new admission score, the RAP score, dedicated to stratifying the risk of poor long-term outcome after BAVM rupture. This easy-to-use scoring system may help to improve communication between health care providers and consistency in clinical research. Only external prospective cohorts and population-based studies will ensure full validation of the RAP scores' capacity to predict outcome after BAVM rupture.

  4. 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.

  5. Outcomes and Complications After Endovascular Treatment of Brain Arteriovenous Malformations: A Prognostication Attempt Using Artificial Intelligence.

    Science.gov (United States)

    Asadi, Hamed; Kok, Hong Kuan; Looby, Seamus; Brennan, Paul; O'Hare, Alan; Thornton, John

    2016-12-01

    To identify factors influencing outcome in brain arteriovenous malformations (BAVM) treated with endovascular embolization. We also assessed the feasibility of using machine learning techniques to prognosticate and predict outcome and compared this to conventional statistical analyses. A retrospective study of patients undergoing endovascular treatment of BAVM during a 22-year period in a national neuroscience center was performed. Clinical presentation, imaging, procedural details, complications, and outcome were recorded. The data was analyzed with artificial intelligence techniques to identify predictors of outcome and assess accuracy in predicting clinical outcome at final follow-up. One-hundred ninety-nine patients underwent treatment for BAVM with a mean follow-up duration of 63 months. The commonest clinical presentation was intracranial hemorrhage (56%). During the follow-up period, there were 51 further hemorrhagic events, comprising spontaneous hemorrhage (n = 27) and procedural related hemorrhage (n = 24). All spontaneous events occurred in previously embolized BAVMs remote from the procedure. Complications included ischemic stroke in 10%, symptomatic hemorrhage in 9.8%, and mortality rate of 4.7%. Standard regression analysis model had an accuracy of 43% in predicting final outcome (mortality), with the type of treatment complication identified as the most important predictor. The machine learning model showed superior accuracy of 97.5% in predicting outcome and identified the presence or absence of nidal fistulae as the most important factor. BAVMs can be treated successfully by endovascular techniques or combined with surgery and radiosurgery with an acceptable risk profile. Machine learning techniques can predict final outcome with greater accuracy and may help individualize treatment based on key predicting factors. Copyright © 2016 Elsevier Inc. All rights reserved.

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

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    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.

  7. 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.

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

    Science.gov (United States)

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

    2015-04-01

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

  9. 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.

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

    Science.gov (United States)

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

    2014-09-01

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

  11. Autodestrucción espontánea de una malformación arteriovenosa cerebral Spontaneous self-destruction of a cerebral arteriovenous malformation

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    Antonio Guevara Melcón

    2011-12-01

    Full Text Available Se presenta una paciente que fue investigada y operada en el Hospital Universitario "Gral. Calixto García", portadora de una malformación arteriovenosa cerebral, que desapareció espontáneamente después de un cuadro neurológico de intensa gravedad. Arteriográficamente se comprobó que estaba asociada a un aneurisma arterial en su mismo sistema vascular cerebral. La paciente había sufrido 3 eventos neurológicos en orden ascendente de gravedad, y fue el último motivo de ingreso en la Unidad de Terapia Intensiva, con un nivel de 5 en la escala de Glasgow para el coma y sin signos meníngeos evidenciables. La lesión valorada como malformación arteriovenosa cerebral en las tomografías axiales cumputarizadas previas, no se evidenció en la panangiografía cerebral realizada en la fase de mejoría clínica relativa después de su etapa más crítica. Dicha angiografía demostró un aneurisma arterial homolateral a la malformación arteriovenosa ya desaparecida, el cual fue excluido de la circulación por presillamiento de su cuello sin eventos transoperatorios ni posoperatorios relevantes. Se realiza una revisión de la literatura al respecto.Authors present the case of a patient researched and operated on in the "General Calixto García" University Hospital presenting with a cerebral arteriovenous malformation disappeared spontaneously after a neurologic picture of intense severity. From the arteriographic point of view it was verified that it was associated with an arterial aneurysm present in her same cerebral vascular system. The patient has had three neurological events in rising order of severity which was the last reason for admission in the Intensive Therapy Unit (ITU with a level of 5 in the Glasgow scale for the coma and without obvious meningeal signs. The injury classified as a cerebral arteriovenous malformation according to the previous computerized axial tomographies was not evidenced in the cerebral panangiograpy carried out

  12. Novel brain arteriovenous malformation mouse models for type 1 hereditary hemorrhagic telangiectasia.

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    Eun-Jung Choi

    Full Text Available Endoglin (ENG is a causative gene of type 1 hereditary hemorrhagic telangiectasia (HHT1. HHT1 patients have a higher prevalence of brain arteriovenous malformation (AVM than the general population and patients with other HHT subtypes. The pathogenesis of brain AVM in HHT1 patients is currently unknown and no specific medical therapy is available to treat patients. Proper animal models are crucial for identifying the underlying mechanisms for brain AVM development and for testing new therapies. However, creating HHT1 brain AVM models has been quite challenging because of difficulties related to deleting Eng-floxed sequence in Eng(2fl/2fl mice. To create an HHT1 brain AVM mouse model, we used several Cre transgenic mouse lines to delete Eng in different cell-types in Eng(2fl/2fl mice: R26CreER (all cell types after tamoxifen treatment, SM22α-Cre (smooth muscle and endothelial cell and LysM-Cre (lysozyme M-positive macrophage. An adeno-associated viral vector expressing vascular endothelial growth factor (AAV-VEGF was injected into the brain to induce focal angiogenesis. We found that SM22α-Cre-mediated Eng deletion in the embryo caused AVMs in the postnatal brain, spinal cord, and intestines. Induction of Eng deletion in adult mice using R26CreER plus local VEGF stimulation induced the brain AVM phenotype. In both models, Eng-null endothelial cells were detected in the brain AVM lesions, and formed mosaicism with wildtype endothelial cells. However, LysM-Cre-mediated Eng deletion in the embryo did not cause AVM in the postnatal brain even after VEGF stimulation. In this study, we report two novel HHT1 brain AVM models that mimic many phenotypes of human brain AVM and can thus be used for studying brain AVM pathogenesis and testing new therapies. Further, our data indicate that macrophage Eng deletion is insufficient and that endothelial Eng homozygous deletion is required for HHT1 brain AVM development.

  13. 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

  14. Treatment of arteriovenous malformations with linear accelerator-based radiosurgery compared with Gamma Knife surgery.

    Science.gov (United States)

    Orio, Peter; Stelzer, Keith J; Goodkin, Robert; Douglas, James G

    2006-12-01

    The authors sought to compare the outcomes of patients with arteriovenous malformations (AVMs) treated by Gamma Knife surgery (GKS) with those of patients treated by linear accelerator-based (LINAC) radiosurgery. One hundred and eighty-seven patients with AVMs were treated at our institution between 1992 and 2003. Ninety-one patients were treated with GKS and 96 patients were treated with LINAC radiosurgery. Patient and treatment characteristics in the two groups included the following. In the LINAC group, the median age was 33 years (range 9-66 years); the median dose was 16 Gy (70% isodose line); the median treated AVM volume was 5.5 cm3; and 46% of patients in this group were treated after hemorrhage. In the GKS group, the median age was 38 years (range 6-63 years); the median dose was 20 Gy (50% isodose line); the median treated AVM volume was 4.3 cm3; and 44% of patients in this group were treated after hemorrhage. Obliteration of AVMs was determined by performing computed tomography (CT) angiography and/or magnetic resonance (MR) angiography and angiography. Patient follow-up evaluation included obtaining an MR angiogram/MR image or CT angiogram at 6 months, at 1 year, and then annually thereafter. Angiography was performed to confirm obliteration when MR angiography and/or CT angiography no longer revealed evidence of an AVM. The 5-year estimated AVM obliteration rate was 66% in the entire patient group; the LINAC group was 60%; the GKS group was 72%; this difference was not statistically significant (p = 0.97). Twelve patients who underwent treatment with LINAC radiosurgery underwent retreatment with GKS and one was retreated with LINAC radiosurgery. The obliteration rate was 82%. Six patients treated with GKS were retreated with GKS, but the follow-up time is of short duration. Chronic toxicity occurred in 8% of both the GKS and the LINAC groups (p = 0.61). Posttreatment hemorrhage during the time of risk before AVM obliteration was 13% in the GKS group

  15. Multimodality Management of Spetzler-Martin Grade 3 Brain Arteriovenous Malformations with Subgroup Analysis.

    Science.gov (United States)

    Abecassis, Isaac Josh; Nerva, John D; Feroze, Abdullah; Barber, Jason; Ghodke, Basavaraj V; Kim, Louis J; Sekhar, Laligam N

    2017-06-01

    Spetzler-Martin grade 3 (SM3) lesions entail 4 distinct subtypes described based on size, eloquence, and deep venous drainage (3A-3D). The ideal management of each is contentious, and the results of A Randomized Trial of Unruptured Brain AVMs (ARUBA) introduced additional controversy and attention toward management strategies of unruptured brain arteriovenous malformations (bAVMs). We retrospectively reviewed 114 patients with treated SM3 bAVMs, including both ruptured and unruptured lesions. Primary outcomes included modified Rankin score at most recent follow-up, angiographic cure, and permanent treatment-related complications (morbidity). Other outcomes included mortality, bAVM recurrence or rebleed, and transient treatment-related complications. We used univariate and multivariate modeling to determine whether any specific features were predictive of outcomes. For unruptured bAVMs, an "ARUBA eligible" subgroup analysis was performed. We also reviewed the literature on management of ruptured and unruptured SM3 bAVMs. Of the 114 identified SM3 bAVMs, 40% were unruptured. Most (43.5%) lesions in the unruptured group were type 3C, whereas most ruptured bAVMs (66.2%) were type 3A. Unruptured lesions were mostly managed with radiosurgery (47.8%) and ruptured ones with preoperative embolization and surgery (36.7%). Surgical intervention was predictive of angiographic cure in multivariate modeling, even after controlling for ≥2 years of follow-up, although associated with a slightly higher rate of morbidity. Focal neurological deficit was the only predictor of a worse (modified Rankin score ≥2) functional outcome in follow-up for unruptured bAVMs. For ruptured bAVMs, superficial and cerebellar locations were predictive of better outcomes in multivariate models, in the absence of a focal neurological deficit at presentation and new after surgery deficit. ARUBA SM3 bAVMs specifically underwent more embolization as a monotherapy and less microsurgical resection than

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

    Science.gov (United States)

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

    2015-09-01

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

  17. 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

  18. Dosimetric effects of Onyx embolization on Gamma Knife arteriovenous malformation dose distributions.

    Science.gov (United States)

    Schlesinger, David J; Nordström, Håkan; Lundin, Anders; Xu, Zhiyuan; Sheehan, Jason P

    2016-12-01

    OBJECTIVE Patients with arteriovenous malformations (AVMs) treated with Gamma Knife radiosurgery (GKRS) subsequent to embolization suffer from elevated local failure rates and differences in adverse radiation effects. Onyx is a common embolic material for AVMs. Onyx is formulated with tantalum, a high atomic number (Z = 73) element that has been investigated as a source of dosimetric uncertainty contributing to the less favorable clinical results. However, prior studies have not modeled the complicated anatomical and beam geometries characteristic of GKRS. This study investigated the magnitude of dose perturbation that can occur due to Onyx embolization using clinically realistic anatomical and Gamma Knife beam models. METHODS Leksell GammaPlan (LGP) was used to segment the AVM nidus and areas of Onyx from postcontrast stereotactic MRI for 7 patients treated with GKRS postembolization. The resulting contours, skull surface, and clinically selected dose distributions were exported from LGP in DICOM-RT (Digital Imaging and Communications in Medicine-radiotherapy) format. Isocenter locations and dwell times were recorded from the LGP database. Contours were converted into 3D mesh representations using commercial and in-house mesh-editing software. The resulting data were imported into a Monte Carlo (MC) dose calculation engine (Pegasos, Elekta Instruments AB) with a beam geometry for the Gamma Knife Perfexion. The MC-predicted dose distributions were calculated with Onyx assigned manufacturer-reported physical constants (MC-Onyx), and then compared with corresponding distributions in which Onyx was reassigned constants for water (MC-water). Differences in dose metrics were determined, including minimum, maximum, and mean dose to the AVM nidus; selectivity index; and target coverage. Combined differences in dose magnitude and distance to agreement were calculated as 3D Gamma analysis passing rates using tolerance criteria of 0.5%/0.5 mm, 1.0%/1.0 mm, and 3.0%/3.0 mm

  19. Potential improvement of three dimension treatment planning and proton beams in fractonated radiotherapy of large cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Miralbell, R.; Urie, M. (Harvard Univ., Boston, MA (United States))

    1993-01-15

    The treatment of large cerebral arteriovenous malformations is a surgical challenge, especially for deep seated brain locations. Furthermore, these lesions are unfit for radiosurgical approaches due to a high risk of complications secondary to high radiation doses to large brain volumes. Fractionated precision radiotherapy can potentially deliver high, uniform, target-contoured dose distributions optimizing the dose reduction to the critical surrounding brain. The results of a study are presented in such a way that dose distributions achievable with proton beams are compared to those with 10 MV x-rays; and the potential improvements with protons evaluated, relying heavily on dose-volume histograms to examine the coverage of the lesion as well as the dose to the normal brain, brain-stem, and optic chiasm.

  20. Neuraxis Cavernous Malformations: a Four Cases Report

    Directory of Open Access Journals (Sweden)

    Yandy Prieto Leyras

    2013-04-01

    Full Text Available Cavernous malformations are benign vascular tumors, with a raspberry-like morphology, commonly described as part of the group of the so called cryptic vascular malformations, which are rare neurological presentations. They are considered to be a controversial chapter in neuroscience due to the clinical-topographic variability of their presentation. Their management remains controversial. We present the cases of four patients with neuraxis cavernomas who are characterization from clinical and imaging standpoint, up to their final outcome. Early detection, understanding, and better management of the neuraxis cavernous malformations are the result of contemporary technological advances. The historical review on the subject shows the difference between the few cases that could be clinically diagnosed through conventional radiology and the largest number of lesions detected at autopsy or during surgery.

  1. 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.

  2. [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.

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

    Directory of Open Access Journals (Sweden)

    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. 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.

  5. 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.

  6. 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.

  7. The role of time-resolved 3D contrast-enhanced MR angiography in the assessment and grading of cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Oleaga, L., E-mail: lauraoleaga@gmail.co [Radiology Department, Hospital Clinic, Villarroel 170, Barcelona 08036 (Spain); Dalal, S.S.; Weigele, J.B.; Hurst, R.W. [Radiology Department, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104 (United States); Lee, J. [Radiology Department, Hospital Clinic, Villarroel 170, Barcelona 08036 (Spain); Neurosurgery Department, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104 (United States); Voorhees, A. [Siemens Medical Solutions USA, Inc., Malvern, PA (United States); Melhem, E.R. [Radiology Department, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104 (United States)

    2010-06-15

    Purpose: To assess the role of three-dimensional (3D) contrast-enhanced, time-resolved MR angiography (CE TR MRA) in patients with intracranial arteriovenous malformations (AVMs). Methods: We studied 12 patient with intracranial AVMs on a 3.0 T MR imaging system (Magentom TIM Trio, Siemens Medical Solutions, Erlangen, Germany) using 3D CE TR MRA with autocalibrating partially parallel acquisitions and echo sharing schemes, which provided temporal resolution of 0.58 or 1.7 s and near isotropic voxels. We qualitatively assessed image quality of the 3D CE TR MRA and compared the grading of the AVMs based on modified Spetzler-Martin system for 3D CE TR MRA and catheter digital subtraction angiography (DSA). Results: CE TR MRA provided good quality images in the 3 standard orthogonal planes, and good arterial-venous separation in all cases. All AVMs were correctly graded by CE TR MRA when compared with DSA. 3D CE TR MRA provides a non-invasive alternative to DSA for the evaluation of cerebral AVMs.

  8. 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.

  9. Enhanced analysis of intracerebral arterioveneous malformations by the intraoperative use of analytical indocyanine green videoangiography: technical note.

    Science.gov (United States)

    Faber, Florian; Thon, Niklas; Fesl, Gunther; Rachinger, Walter; Guckler, Roland; Tonn, Jörg-Christian; Schichor, Christian

    2011-11-01

    In cerebral arterioveneous malformations (AVMs) detailed intraoperative identification of feeding arteries, nidal vessels and draining veins is crucial for surgery. Intraoperative imaging techniques like indocyanine green videoangiography (ICG-VAG) provide information about vessel architecture and patency, but do not allow time-dependent analysis of intravascular blood flow. Here we report on our first experiences with analytical indocyanine green videoangiography (aICG-VAG) using FLOW 800 software as a useful tool for assessing the time-dependent intraoperative blood flow during surgical removal of cerebral AVMs. Microsope-integrated colour-encoded aICG-VAG was used for the surgical treatment of a 38-year-old woman diagnosed with an incidental AVM, Spetzler Martin grade I, of the left frontal lobe and of a 26-year-old man suffering from seizures caused by a symptomatic AVM, Spetzler Martin grade III, of the right temporal lobe. Analytical ICG-VAG visualization was intraoperatively correlated with in situ micro-Doppler investigation, as well as preoperative and postoperative digital subtraction angiography (DSA). Analytical ICG-VAG is fast, easy to handle and integrates intuitively into surgical procedures. It allows colour-encoded visualization of blood flow distribution with high temporal and spatial resolution. Superficial major and minor feeding arteries can be clearly separated from the nidus and draining veins. Effects of stepwise vessel obliteration on velocity and direction of AVM blood flow can be objectified. High quality of visualization, however, is limited to the site of surgery. Colour-encoded aICG-VAG with FLOW 800 enables intraoperative real-time analysis of arterial and venous vessel architecture and might, therefore, increase efficacy and safety of neurovascular surgery in a selected subset of superficial AVMs.

  10. 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.

  11. 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.

  12. 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.

  13. 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.

  14. 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

  15. 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

  16. Clinical aspects, management and outcome of brain arteriovenous malformations – results with microsurgery first policy

    Directory of Open Access Journals (Sweden)

    Sandu Aurelia Mihaela

    2014-12-01

    Full Text Available We performed a retrospective study, including patients operated for brain AVMs between 1999 and 2014, in the Clinic of Neurosurgery, Emergency Clinical Hospital Bagdasar-Arseni, Bucharest. 277 patients underwent surgery for brain AVMs. Mean age was 29.82 years. 195 patients (70.40% presented with hemorrhage and 86 cases (31.05% were admitted with seizures. We performed total resection of AVMs in 228 cases (82.31% and subtotal resection in 49 cases (17.69%. Regarding patients with residual nidus, 16 of them underwent second surgery, 27 stereotactic radiosurgery Gamma Knife, 3 embolization and 3 refused further treatment. Modified Rankin Scale (mRS improved following surgery (Z = -9.248, p = 0.000. Early complications (0-30 days were encountered in 84 patients (30.32%. We found the following risk factors for postoperative complications occurrence: motor deficit (p = 0.006, co-morbidities (p = 0.023, higher mRS (p = 0.005, lower Karnofsky score (p = 0.003, lower GCS (p = 0.016, profound nidus (p = 0.001, eloquent aria (p = 0.000, large nidus (p = 0.000, multiple arterial territory (p = 0.000, deep feeding arteries (p = 0.000, higher number of feeding arteries (p = 0.000, deep venous drainage (p = 0.000, multiple draining veins (p = 0.000, higher Spetzler- Martin grade (p = 0.006, high flow (p = 0.000, vascular steel (p = 0.000, associated aneurysms (p = 0.010 and decompressive craniectomy (p = 0.019. Mortality was 6.1%. Microsurgery is the treatment of choice for brain AVMs. Surgical results are excellent, with low morbidity and mortality. Patients with poor surgical results belonged to the group admitted with severe altered general state, state of consciousness, massive hematomas and acute brainstem dysfunction. If part of the nidus cannot be safely surgical resected, stereotactic radiosurgery can provide definitive cure of the lesion.

  17. 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.

  18. ARTERIO-VENOUS FISTULA IN THE TEMPORAL REGIONS – A CLINICAL CASE

    Directory of Open Access Journals (Sweden)

    Irina Dobrin

    2011-12-01

    Full Text Available The arterio-venous fistulae in the temporal regions, to be first described by Bartholin around 1730, were considered as tardy post-traumatic lesions correlated with the impact of tough objects in the above-mentioned area. The superficial temporal arterio-venous axis is usually interested at the level of the pterional region, while establishment of some communication between the artery and the vein generates an arterio-venous fistula with a systolodiastolic murmur and the tendency for progressive growth. The study discusses a clinical case and its surgical solution.

  19. 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 ...

  20. Stent explantation from an arteriovenous fistula for hemodialysis: a case report.

    Science.gov (United States)

    Nano, Giovanni; Dalainas, Ilias; Casana, Renato; Bianchi, Paolo; Lupattelli, Tommaso; Malacrida, Giovanni; Tealdi, Domenico G

    2006-01-01

    Percutaneous transluminal angioplasty is the first treatment of a dysfunctional vascular access for hemodialysis. A case of stenting of a native arteriovenous hemodialysis fistula is reported that was treated with a stent placement at the anastomosis level, with explantation of the stent after complete thrombosis of the fistula 48 hours after the procedure. It is preferable to treat arteriovenous fistulas with simple balloon dilatation, avoiding stenting of the fistula, especially in the anastomosis site.

  1. 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

  2. Double spinal dural arteriovenous fistulas: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Krings, T. [Department of Neuroradiology, University Hospital of the University of Technology, Aachen, Pauwelsstrasse 30, 52057, Aachen (Germany); Department of Neurosurgery, University Hospital of the University of Technology, Aachen, Pauwelsstrasse 30, 52057, Aachen (Germany); Mull, M.; Thron, A. [Department of Neuroradiology, University Hospital of the University of Technology, Aachen, Pauwelsstrasse 30, 52057, Aachen (Germany); Reinges, M.H.T. [Department of Neurosurgery, University Hospital of the University of Technology, Aachen, Pauwelsstrasse 30, 52057, Aachen (Germany)

    2004-03-01

    Spinal dural arteriovenous fistula (SDAVF) is the most common spinal vascular malformation. It mainly affects men after the fifth decade and is usually an acquired lesion with an unknown etiology. We report on a patient with the unusual finding of two separate SDAVFs at the level of L1 on the right and L2 on the left side. Initial selective spinal digital subtraction angiography (DSA) was terminated with demonstration of a SDAVF at the level of L1 but incomplete demonstration of all segmental arteries. Due to a recurrent deterioration of the patient's neurological status, and persistent pathological vessels seen on MRI, a second spinal DSA was performed 6 years later, demonstrating the second fistula at the level of L2 on the left side with a separate venous drainage pattern. A retrospective analysis of the angiographic films suggested that both fistulas had already been present 6 years previously. This conclusion is justified because of a transient and faint opacification of the left L2 fistula demonstrated on the films after injection of the right L2 segmental artery. We conclude that in the case of incomplete angiography and persistent clinical and MR findings not only reopening of the treated SDAVF has to be taken into account but also the existence of a second fistula. Since this is the first case of a double fistula in our series of 129 SDAVFs, and given the few reported cases of double SDAVFs, we do not think that completion of selective spinal DSA has to be postulated routinely after a fistula has been found. However, repeat angiography should be performed in patients who continue to deteriorate, fail to improve with persisting MRI pathologies, or demonstrate delayed deterioration after a period of improvement. (orig.)

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

    Science.gov (United States)

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

    2009-01-01

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

  4. Hybrid management of a spontaneous ilio-iliac arteriovenous fistula: a case report

    LENUS (Irish Health Repository)

    O'Brien, Gavin C

    2011-08-22

    Abstract Introduction Spontaneous iliac arteriovenous fistulae are a rare clinical entity. Such localized fistulation is usually a result of penetrating traumatic or iatrogenic injury. Clinical presentation can vary greatly but commonly includes back pain, high-output congestive cardiac failure and the presence of an abdominal bruit. Diagnosis, therefore, is often incidental or delayed. Case presentation We report a case of a spontaneous ilio-iliac arteriovenous fistula in a 68-year-old Caucasian man detected following presentation with unilateral claudication and congestive cardiac failure. Following computed tomography evaluation, the fistula was successfully treated with a combined endovascular (aorto-uni-iliac device) and open (femoro-femoral crossover) approach. Conclusion Endovascular surgery has revolutionized the management of such fistulae and we report an interesting case of a high-output iliac arteriovenous fistulae successfully treated with a hybrid vascular approach.

  5. Therapeutic clues in spinal dural arteriovenous fistulas - a 30 year experience of 156 cases.

    Science.gov (United States)

    Hessler, C; Regelsberger, J; Grzyska, U; Illies, T; Zeumer, H; Westphal, M

    2010-02-01

    Spinal dural arteriovenous fistulae (SDAVF) are rare but remain the most common type of spinal vascular malformations. Treatment options for SDAVF include endovascular embolization, microsurgical dissection or a combination of both. But the optimal treatment paradigm has yet to be defined and may well be an individualized interdisciplinary combinatorial approach. From 1980 to 2008, 156 patients with the diagnosis of SDAVF were treated by neuroradiological and neurosurgical means. Based on the procedure-related complications we retrospectively analyzed our data to elucidate the reasons for endovascular failure and the evolution of the surgical technique. 156 patients were included in this study. There were 31 (19.9%) female and 125 (80.1%) male patients. Average age at the time of diagnosis was 60.8 years. 102 out of 156 (65.4%) underwent endovascular obliteration, 54 (34.6%) patients were treated primarily by surgery. 134 (85.9%) underwent follow-up examination. A total of 29 (18.6%) out of 156 patients could not be treated successfully by endovascular (9.4%) or surgical (4.1%) means. Microsurgery can be recommended as the first choice treatment when the fistula's point is unmistakably identified intradurally. Endovascular obliteration may be justifiable in cases with an easy access to a monoradicular feeding artery during diagnostic angiography. Surgery is a definitive treatment with stable long-term results in which procedure-related morbidity is low. During evolution of the combined approach, endovascular coil placement for correct localization of the fistula and the use of intraoperative micro-Doppler was found to be very helpful in increasing the safety of the surgical procedure and minimizing surgical exposure. Georg Thieme Verlag KG Stuttgart * New York.

  6. Two Cases of Arnold-Chiari Malformation with Respiratory Failure

    Directory of Open Access Journals (Sweden)

    Sinem Iliaz

    2014-03-01

    Full Text Available Arnold–Chiari malformation is defined as downward displacement of the brainstem and cerebellum through the foramen magnum. It has different clinical presentations and four subtypes. It is known that downward migration of posterior fossa components through the foramen magnum and associated lower cranial nerve palsy and brainstem compression can cause respiratory failure. Acute respiratory failure could mark the onset of the disease. Posterior fossa decompression performed to treat primary disease can improve the central sleep abnormalities. As respiratory failure is rarely seen, this paper presents two cases of Arnold–Chiari malformation with respiratory failure.

  7. 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).

  8. Dural arteriovenous fistula at the foramen magnum: Report of a case and clinical-anatomical review.

    Science.gov (United States)

    Llácer, José L; Suay, Guillermo; Piquer, José; Vazquez, Victor

    2016-01-01

    Arterial supply and venous drainage at the foramen magnum is variable. Two main forms of clinical presentation, intracranial and spinal, can be differentiated when a dural arteriovenous fistula (DAVF) is found at this level. We describe a case of a 68-year-old patient with a progressive paraparesis, diagnosed of dural arteriovenous fistula located at the posterior lip of foramen magnum. We review, in this setting, the vascular radiological anatomy of those fistulas and its important correlation with neurologic clinical symptoms. Copyright © 2016 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  9. [Arterial steal via an arteriovenous fistula for hemodialysis. A clinical case and review of the literature].

    Science.gov (United States)

    Juliá Montoya, J; Lozano Vilardell, P; Corominas Roura, C; Blanes Mompó, I; Flores López, D; Manuel-Rimbau Muñoz, E; García de la Torre, A

    1993-01-01

    We related a case of arterial atrappment in the left upper limb by an arteriovenous humerus-cephalic hyperfunctioning fistula. Surgical procedure consisted on the insertion of a PTFE's banding around the arterialized vein obtaining satisfactory clinic and functional results. We review in the literature, the frequency the pathogeny and the therapeutics possibilities.

  10. 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.

  11. 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.)

  12. 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.

  13. Selective Transvenous Coil Embolization of Dural Arteriovenous Fistula: A Report of Three Cases

    OpenAIRE

    2007-01-01

    We herein report three cases of dural arteriovenous fistula (DAVF) in which the venous outlet immediately adjacent to the fistula was selectively embolized. Case 1: A 69-year-old man presented with a subarachnoid hemorrhage (SAH). Angiography demonstrated a DAVF in the left superior petrous sinus. Case 2: A 59-year-old woman presented with dizziness. Angiography demonstrated a DAVF adjacent to great vein of Galen. The DAVF drained through the great vein of Galen with retrograde leptomeningeal...

  14. 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。

  15. 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

  16. 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.

  17. 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.)

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

    Science.gov (United States)

    Machet, A; Portefaix, C; Kadziolka, K; Robin, G; Lanoix, O; Pierot, L

    2012-10-01

    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.

  19. Feasibility of Non-contrast-enhanced MR Angiography Using the Time-SLIP Technique for the Assessment of Pulmonary Arteriovenous Malformation.

    Science.gov (United States)

    Hamamoto, Kohei; Matsuura, Katsuhiko; Chiba, Emiko; Okochi, Tomohisa; Tanno, Keisuke; Tanaka, Osamu

    2016-07-11

    The purpose of this study was to evaluate the diagnostic performance of non-contrast-enhanced magnetic resonance angiography with time-spatial labeling inversion pulse (time-SLIP MRA) in the assessment of pulmonary arteriovenous malformation (PAVM). Eleven consecutive patients with 38 documented PAVMs underwent time-SLIP MRA with a 3-tesla unit. Eight patients with 25 lesions were examined twice, once before and once after embolotherapy. The lesions were divided into two groups-initial diagnosis (n = 35) and follow-up (n = 28)-corresponding to untreated and treated lesions, respectively, and were evaluated separately. To evaluate the initial diagnosis group, two reviewers assessed image quality for visualization of PAVMs by using a qualitative 4-point scale (1 = not assessable to 4 = excellent). The location and classification of PAVMs were also evaluated. The results were compared with those from digital subtraction angiography. For evaluation of the follow-up group, the reviewers assessed the status of treated PAVMs. Reperfusion and occlusion were defined respectively as visualization or disappearance of the aneurysmal sac. The diagnostic accuracy of time-SLIP MRA was assessed and compared with standard reference images. Interobserver agreement was evaluated with the κ statistic. In the initial diagnosis group, time-SLIP MRA correctly determined the PAVMs in all but one patient with one lesion who had image degradation due to irregular breath. Image quality was considered excellent (median = 4) and the κ coefficient was 0.85. Additionally, both readers could correctly localize and classify the PAVMs on time-SLIP MRA images with both κ coefficient of 1.00. In the follow-up group, the sensitivity and specificity of time-SLIP MRA for reperfusion of PAVMs were both 100%, and the κ coefficient was 1.00. Time-SLIP MRA is technically and clinically feasible and represents a promising technique for noninvasive pre- and post-treatment assessment of PAVMs.

  20. Detection of residual brain arteriovenous malformations after radiosurgery: diagnostic accuracy of contrast-enhanced four-dimensional MR angiography at 3.0 T

    Science.gov (United States)

    Lim, H K; Choi, C G; Kim, S M; Kim, J L; Lee, D H; Kim, S J; Suh, D C

    2012-01-01

    Objective To evaluate the diagnostic accuracy of four-dimensional MR angiography (4D-MRA) at 3.0 T for detecting residual arteriovenous malformations (AVMs) after Gamma Knife® (Elekta Instrument AB, Stockholm, Sweden) radiosurgery (GKRS). Methods We assessed 36 angiographically confirmed AVMs in 36 patients who had been treated with GKRS. 4D-MRA was performed after GKRS and the time intervals were 39.4±26.0 months [mean±standard deviation (SD)]. 4D-MRA was obtained at 3.0 T after contrast injection, with a measured voxel size of 1×1×1 mm and a temporal resolution of 1.1 s (13 patients) or a voxel size of 1×1×2 mm and a temporal resolution of 0.98 s (23 patients). X-ray angiography was performed as the standard reference within 53±47 days (mean±SD) after MRA. To determine a residual AVM, the 4D-MRA results were independently reviewed by two readers blinded to the X-ray angiography results. We evaluated diagnostic sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of 4D-MRA for detection of a residual AVM. Results A residual AVM was identified in 13 patients (13/36, 36%) on X-ray angiography. According to Readers 1 and 2, 4D-MRA had a sensitivity of 79.6% and 64.3%, a specificity of 90.9% and 100%, a PPV of 84.6% and 100% and an NPV of 90% and 81.5%, respectively, and a diagnostic accuracy of 86.1% for Readers 1 and 2, for detecting residual AVMs after GKRS. Conclusion The diagnostic accuracy of 4D-MRA at 3.0 T seems high, but there is still the possibility of further improving the spatiotemporal resolution of this technique. PMID:22294705

  1. Cerebral abscess associated with odontogenic bacteremias, hypoxemia, and iron loading in immunocompetent patients with right-to-left shunting through pulmonary arteriovenous malformations.

    Science.gov (United States)

    Boother, E J; Brownlow, S; Tighe, H C; Bamford, K B; Jackson, J E; Shovlin, C L

    2017-04-19

    Cerebral abscess is a recognised complication of pulmonary arteriovenous malformations (PAVMs) that allow systemic venous blood to bypass the pulmonary capillary bed through anatomic right-to-left shunts. Broader implications and mechanisms remain poorly explored. Between June 2005 and December 2016, at a single institution, 445 consecutive adult patients with CT-scan confirmed PAVMs (including 403 (90.5%) with hereditary haemorrhagic telangiectasia) were recruited to a prospective series. Multivariate logistic regression, and detailed peri-abscess histories were evaluated to identify potential associations with cerebral abscess. Rates were compared to an earlier non-overlapping series. Thirty-seven (8.3%) of the 445 patients experienced a cerebral abscesses at median age 50 (range 19-76) years. The rate adjusted for ascertainment bias was 27/435 (6.2%). 29/37 (78.4%) abscess patients had no PAVM diagnosis prior to their abscess, a rate unchanged from earlier UK series. 21/37 (56.7%) suffered residual neurological deficits, most commonly memory/cognition impairment; hemiparesis, and visual defects. Isolation of periodontal microbes, and precipitating dental and other interventional events emphasised potential sources of endovascular inoculations. In multivariate logistic regression, cerebral abscess was associated with low oxygen saturation (indicating greater right-to-left shunting); higher transferrin iron saturation index; intravenous iron use for anemia (adjusted odds ratio 5.4 [95% confidence intervals 1.4, 21.1]); male gender; and venous thromboemboli. There were no relationships with anatomic attributes of PAVMs, or red cell indices often increased due to secondary polycythemia. Greater appreciation of the risk of cerebral abscess in undiagnosed PAVMs is required. Lower SaO2 and intravenous iron may be modifiable risk factors.

  2. 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

  3. Traumatic arteriovenous fistula after kickboxing injury: a case report and review of the literature.

    Science.gov (United States)

    Rezvani, Masoud

    2014-03-01

    A traumatic arteriovenous fistula (AVF) after repetitive blunt trauma has not been described previously. In a 34-year-old male, the first reported case of such an injury after repetitive blunt trauma is described. A 34-year-old gentleman presented with a non-healing ulcer near his medial malleolus. A bone scan was performed and then treated for presumed osteomyelitis. An arteriogram confirmed an AVF, and coil embolization was performed with complete occlusion of the AVF. Subsequently, the ulcer healed rapidly with no complication. Along with the cause of AVF, this case is notable for symptom presentation. Arteriovenous fistula after blunt trauma can present as a non-healing venous stasis ulcer, which could be treated non-invasively.

  4. Systemic-pulmonary arteriovenous fistula of traumatic origin: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Hirsch, M.; Maroko, I.; Gueron, M.; Goleman, L.

    1983-08-01

    Arteriovenous fistulas between the systemic circulation and the pulmonary artery are extremely rare. Continuous precordial murmur is the usual clinical sign while unilateral rib notching may be the only radiologic manifestation of this condition. Selective angiographic investigation is necessary to localize the site of such an arteriovenous (AV) fistula before surgery is performed. In a review of the literature of 15 published cases, the majority were of congenital origin, with four of these systemic-pulmonary AV fistulas of traumatic origin, of which one occurred after insertion of an intercostal catheter. We describe one case of traumatic origin 9 years after percutaneous thoracic drainage for spontaneous pneumothorax, in which transcatheter embolic occlusion of the feeding arteries of an AV fistula was attempted. The advantages and the disadvantages of the non-surgical and surgical therapeutic approaches are discussed.

  5. Dural arteriovenous fistulas of the cavernous sinus - clinical case and treatment

    Directory of Open Access Journals (Sweden)

    Chiriac A.

    2014-06-01

    Full Text Available The purpose of our article is to present the results of our treatment of dural arteriovenous fistula of the cavernous sinus by glue embolization of the external carotid artery feeders. By this case presentation we try to clarify the clinical course, with the dural carotid cavernous fistula (CCF, characterizing a pallet of symptoms, paying special attention to radiological finding and endovascular treatment.

  6. Paraplegia in a chiropractic patient secondary to atraumatic dural arteriovenous fistula with perimedullary hypertension: case report.

    Science.gov (United States)

    Foreman, Stephen M; Stahl, Michael J; Schultz, Gary D

    2013-07-08

    Intracranial dural arteriovenous fistulas are abnormal communications between higher-pressure arterial circulation and lower-pressure venous circulation. This abnormal communication can result in important and frequently misdiagnosed neurological abnormalities.A case of rapid onset paraplegia following cervical chiropractic manipulation is reviewed. The patient's generalized spinal cord edema, lower extremity paraplegia and upper extremity weakness, were initially believed to be a complication of the cervical spinal manipulation that had occurred earlier on the day of admission. Subsequent diagnostic testing determined the patient suffered from impaired circulation of the cervical spinal cord produced by a Type V intracranial arteriovenous fistula and resultant venous hypertension in the pontomesencephalic and anterior spinal veins.The clinical and imaging findings of an intracranial dural arteriovenous fistula with pontomesencephalic venous congestion and paraplegia are reviewed.This case report emphasizes the importance of thorough and serial diagnostic imaging in the presence of sudden onset paraplegia and the potential for error when concluding atypical neurological presentations are the result of therapeutic misadventure.

  7. Arnold-Chiari Type II Malformation: A Case Report and Review of Prenatal Sonographic Findings

    Directory of Open Access Journals (Sweden)

    Maryam Nik Nejadi

    2008-01-01

    Full Text Available The Arnold-Chiari malformation is a congenital abnormality of CNS, characterized by downwarddisplacement the parts of the cerebellum, fourth ventricle, pons and medulla oblongata into thespinal canal. This malformation is one of causative factor of death in neonates and infants. Athorough understanding of the direct and indirect sonographic findings is necessary for diagnosis ofChiari II malformation in the developing fetus.In this case report, we present a Chiari malformation II detected at 23 weeks of gestation by routinelysonographic screening. The Role of prenatal sonography in recognition of the malformation andprognostic value of these features are discussed.

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

    Science.gov (United States)

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

    2012-02-01

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

  9. A Case of Femoral Arteriovenous Fistula Causing High-Output Cardiac Failure, Originally Misdiagnosed as Chronic Fatigue Syndrome

    Directory of Open Access Journals (Sweden)

    J. Porter

    2014-01-01

    Full Text Available Percutaneous arterial catheterisation is commonly undertaken for a range of diagnostic and interventional procedures. Iatrogenic femoral arteriovenous fistulas are an uncommon complication of these procedures. Most are asymptomatic and close spontaneously, but can rarely increase in size leading to the development of symptoms. We report a case of an iatrogenic femoral arteriovenous fistula, causing worsening congestive cardiac failure, in a 34-year-old marathon runner. This was originally diagnosed as chronic fatigue syndrome. Following clinical examination, duplex ultrasound, and CT angiography a significant arteriovenous fistula was confirmed. Elective open surgery was performed, leading to a dramatic and rapid improvement in symptoms. Femoral arteriovenous fistulas have the potential to cause significant haemodynamic effects and can present many years after the initial procedure. Conservative, endovascular, and open surgical management strategies are available.

  10. Isolated oculomotor nerve palsy caused by cavernous sinus dural arteriovenous fistula: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Ihn, Yon Kwon; Jung, Won Sang [The Catholic Univ. of Korea, Suwon (Korea, Republic of); Kim, Bum Soo [The Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2012-10-15

    Cavernous dural arteriovenous fistula (DAVF), which usually presents with conjunctival injection, proptosis, loss of visual acuity, and ophthalmoplegia, is a rare cause of ophthalmoplegia. Thus, it may be overlooked when the typical symptoms are lacking. There have been some cavernous DAVF case reports presenting with isolated oculomotor, abducens and trochlear nerve palsy. We report a patient presenting with isolated oculomotor palsy, caused by cavernous DAVF, which was treated by transvenous coil embolization. This case suggests that cavernous DAVF should be considered in the differential diagnosis of isolated oculomotor nerve palsy and for which case - selective angiography and embolization may be helpful in reaching a diagnosis and providing a guide for optimal treatment.

  11. 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.

  12. 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

  13. 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

  14. 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

  15. Results of volume-staged fractionated Gamma Knife radiosurgery for large complex arteriovenous malformations: obliteration rates and clinical outcomes of an evolving treatment paradigm.

    Science.gov (United States)

    Franzin, Alberto; Panni, Pietro; Spatola, Giorgio; Vecchio, Antonella Del; Gallotti, Alberto L; Gigliotti, Carmen R; Cavalli, Andrea; Donofrio, Carmine A; Mortini, Pietro

    2016-12-01

    OBJECTIVE There are few reported series regarding volume-staged Gamma Knife radiosurgery (GKRS) for the treatment of large, complex, cerebral arteriovenous malformations (AVMs). The object of this study was to report the results of using volume-staged Gamma Knife radiosurgery for patients affected by large and complex AVMs. METHODS Data from 20 patients with large AVMs were prospectively included in the authors' AVM database between 2004 and 2012. A staging strategy was used when treating lesion volumes larger than 10 cm(3). Hemorrhage and seizures were the presenting clinical feature for 6 (30%) and 8 (40%) patients, respectively. The median AVM volume was 15.9 cm(3) (range 10.1-34.3 cm(3)). The mean interval between stages (± standard deviation) was 15 months (± 9 months). The median margin dose for each stage was 20 Gy (range 18-25 Gy). RESULTS Obliteration was confirmed in 8 (42%) patients after a mean follow-up of 45 months (range 19-87 months). A significant reduction (> 75%) of the original nidal volume was achieved in 4 (20%) patients. Engel Class I-II seizure status was reported by 75% of patients presenting with seizures (50% Engel Class I and 25% Engel Class II) after radiosurgery. After radiosurgery, 71.5% (5/7) of patients who had presented with a worsening neurological deficit reported a complete resolution or amelioration. None of the patients who presented acutely because of hemorrhage experienced a new bleeding episode during follow-up. One (5%) patient developed radionecrosis that caused sensorimotor hemisyndrome. Two (10%) patients sustained a bleeding episode after GKRS, although only 1 (5%) was symptomatic. High nidal flow rate and a time interval between stages of less than 11.7 months were factors significantly associated with AVM obliteration (p = 0.021 and p = 0.041, respectively). Patient age younger than 44 years was significantly associated with a greater than 75% reduction in AVM volume but not with AVM obliteration (p = 0

  16. SU-E-T-382: Evaluation of Clinical Application and Dosimetric Comparison for Treatment Plans of Gamma Knife and CyberKnife for Arteriovenous Malformations

    Energy Technology Data Exchange (ETDEWEB)

    Kuo, C [Department of Radiation Oncology, Shuang Ho Hospital, Taipei Medical Univer, New Taipei City, Taiwan (R.O.C.) (China)

    2015-06-15

    Purpose: To analyze and compare the characteristics of dose distributions between Gamma Knife (GK) and CyberKnife (CK), in treating arteriovenous malformations (AVMs), and evaluate the influences on their clinical applications. Methods: Twenty four patients with AVMs treated with CK of prescribed dose (PD) of 16–25 Gy in single fraction were selected. Each patient’s CT images used for CK treatment planning with contours of targets and critical organs were exported and then loaded into the GK planning system. GK treatment plan with the same PD used in CK was generated for each patient. The metrics for dose comparison between GK and CK included conformity index (CI), gradient index (GI) of 75%, 50% and 25% of the PD, heterogeneity index (HI), volume of brain tissues covered by 10 Gy and 12 Gy, maximum dose to brainstem and beam-on time. Paired Samples t-test was used to analyze these metrics for significance (p value). Results: The CI were 0.744 ± 0.075 (GK) and 0.768 ± 0.086 (CK), p = 0.281. The GI75%, GI50%, and GI25% in GK and CK were 1.735 ± 0.100 and 2.439 ± 0.338 (p < 0.001), 3.169 ± 0.265 and 4.972 ± 0.852 (p < 0.001), and 8.650 ± 0.914 and 14.261 ± 2.476 (p < 0.001). The HI were 0.728 ± 0.072 (GK) and 0.313 ± 0.069 (CK), p < 0.001. There were significant differences both for volume of brain tissues covered by 10 Gy and 12 Gy in GK and CK (p < 0.001). GK had smaller maximum dose to brainstem. CK had shorter beam-on time. Conclusion: GK has similar dose conformity as CK, and has better normal tissue sparing but is less efficient than CK.

  17. 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

  18. 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 .

  19. Urinary lithiasis and urinary tract malformations in children: A retrospective study of 34 cases

    Directory of Open Access Journals (Sweden)

    Jamila Chahed

    2011-01-01

    Full Text Available Background: Although the association of urinary lithiasis and urinary tract malformation is not rare, their management poses challenges. The aim of this study was to evaluate the relationship between urolithiasis and malformations of the urinary system. There were 34 patients (19 males and 15 females with a mean age of 4.8 years (range, 2 months to 14 years. All patients had urinary lithiasis with a urinary tract malformation. Abdominal pain was the most frequent clinical symptom (38%. Urinary infection was found in 7 patients (21% and macroscopic haematuria was present in 10 patients (29%. The most frequent urinary tract malformations were megaureter (8 cases, uretero-pelvic junction obstruction (7 cases and vesico-ureteric reflux (8 cases, but its malformative origin could not be confirmed. Treatment consisted of lithiasis extraction in 32 cases associated with specific treatment of the uropathy in 27 cases. Postoperative outcome was uneventful in all cases. In fact, urinary lithiasis and urinary tract malformation association is not rare. Indeed, 9-34% of urinary lithiasis are noted to be associated with urinary tract malformation. Positive diagnosis relies specifically on kidney ultrasound, intravenous urography, and urethrocystography. Treatment depends on the type of urinary tract malformation, localisation and size of the urinary lithiasis. Conclusion: In conclusion, urinary lithiasis and urinary tract malformation association is a frequent eventuality. Surgical intervention is the usual mode of treatment.

  20. Three cases of hypertension and renal arteriovenous fistula with a de novo fistula.

    Science.gov (United States)

    Melo, Natalia Correa Vieira; Mundim, Juliano Sacramento; Costalonga, Elerson Carlos; Lucon, Antonio Marmo; Santello, Jose Luiz; Praxedes, Jose Nery

    2009-05-01

    The Renal Arteriovenous Fistula (RAVF) is a rare and potentially reversible cause of hypertension and kidney and/or heart failure. The treatment of RAVF aims at preserving the most of the renal parenchyma and, concomitantly, eradicating the symptoms and hemodynamic effects caused by the RAVF. The present study reports three cases of RAVF, including one case of a de novo idiopathic RAVF, which presented with hypertension and kidney and/or heart failure and describes the therapeutic measures used to treat these patients as well as the outcomes.

  1. [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.

  2. [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

  3. Clinical and radiobiological advantages of single-dose stereotactic light-ion radiation therapy for large intracranial arteriovenous malformations. Technical note.

    Science.gov (United States)

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

    2009-11-01

    Radiation treatment of large arteriovenous malformations (AVMs) remains difficult and not very effective, even though seemingly promising methods such as staged volume treatments have been proposed by some radiation treatment centers. In symptomatic patients harboring large intracranial AVMs not amenable to embolization or resection, single-session high-dose stereotactic radiation therapy is a viable option, and the special characteristics of high-ionization-density light-ion beams offer several treatment advantages over photon and proton beams. These advantages include a more favorable depth-dose distribution in tissue, an almost negligible lateral scatter of the beam, a sharper penumbra, a steep dose falloff beyond the Bragg peak, and a higher probability of vascular response due to high ionization density and associated induction of endothelial cell proliferation and/or apoptosis. Carbon ions were recently shown to be an effective treatment for skull-base tumors. Bearing that in mind, the authors postulate that the unique physical and biological characteristics of light-ion beams should convey considerable clinical advantages in the treatment of large AVMs. In the present meta-analysis the authors present a comparison between light-ion beam therapy and more conventional modalities of radiation treatment with respect to these lesions. Dose-volume histograms and data on peripheral radiation doses for treatment of large AVMs were collected from various radiation treatment centers. Dose-response parameters were then derived by applying a maximum likelihood fitting of a binomial model to these data. The present binomial model was needed because the effective number of crucial blood vessels in AVMs (the number of vessels that must be obliterated to effect a cure, such as large fistulous nidus vessels) is low, making the Poisson model less suitable. In this study the authors also focused on radiobiological differences between various radiation treatments. Light

  4. Dural arteriovenous fistula at the foramen magnum presenting with subarachnoid hemorrhage: case reports and literature review.

    Science.gov (United States)

    Guo, L-M; Zhou, H-Y; Xu, J-W; Wang, G-S; Tian, X; Wang, Y; Qiu, Y-M; Jiang, J-Y

    2010-05-01

    Spinal dural arteriovenous fistulas (DAVFs) may arise at any level from the foramen magnum to the sacrum. Only a few case series of DAVFs at the foramen magnum have been reported, especially with patients presenting with subarachnoid hemorrhage (SAH). We performed a retrospective study of four such cases and summarize experiences in the diagnosis and surgical treatment of a DAVF at the foramen magnum. Four male patients, aged from 35 to 51 years, were admitted with severe headache. The cranial computerized tomography scans of all four patients showed SAH, with hemorrhage in the fourth ventricle with or without hemorrhage in the occipital horns of the lateral ventricles. Pre-operative digital subtraction cerebral angiography showed a DAVF at the foramen magnum draining to medullary veins and/or the straight sinus and the confluence of sinuses. Two DAVFs were fed by the vertebral artery, whilst the others were fed by dural branches of the occipital artery and/or the ascending pharyngeal artery. Three patients underwent direct microsurgical electrocoagulation and disconnection of the arteriovenous shunt via an enlargement of the foramen magnum and a hemilaminectomy at C1 by the far lateral suboccipital approach. Post-operative angiography confirmed complete obliteration of the fistula. Cerebral digital subtraction angiography is an effective and accurate method for examination of a DAVF at the foramen magnum. It can be treated effectively and with minimal surgical trauma by microsurgical electrocoagulation and disconnection of the shunt.

  5. Dural Arteriovenous Fistula Following Translabyrinthine Resection of Cerebellopontine Angle Tumors: Report of Two Cases

    Science.gov (United States)

    Li, Peter M.M.C.; Fischbein, Nancy J.; Do, Huy M.; Blevins, Nikolas H.

    2011-01-01

    We describe two cases of dural arteriovenous fistula (DAVF) developing in a delayed fashion after translabyrinthine resection of cerebellopontine angle tumors. Two patients in an academic tertiary referral center, a 46-year-old woman and a 67-year-old man, underwent translabyrinthine resection of a 2-cm left vestibular schwannoma and a 4-cm left petrous meningioma, respectively. Both patients subsequently developed DAVF, and in each case the diagnosis was delayed despite serial imaging follow-up. In one patient, cerebrospinal fluid diversion before DAVF was identified as the cause of her intracranial hypertension; the other patient was essentially asymptomatic but with a high risk of hemorrhage due to progression of cortical venous drainage. Endovascular treatment was effective but required multiple sessions due to residual or recurrent fistulas. Dural arteriovenous fistula is a rare complication of translabyrinthine skull base surgery. Diagnosis requires a high index of clinical suspicion and an understanding of subtle imaging findings that may be present on follow-up studies performed for tumor surveillance. Failure to recognize this complication may lead to misguided interventions for treatment of hydrocephalus and other complications, as well as ongoing risks related to venous hypertension and intracranial hemorrhage. As this condition is generally curable with neurointerventional and/or surgical methods, timely diagnosis and treatment are essential. PMID:23984203

  6. 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.

  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.

    Directory of Open Access Journals (Sweden)

    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. 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.

  9. Pulmonale arteriovenøse malformationer. Moderne behandlingsprincipper

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D

    2001-01-01

    Pulmonary arteriovenous malformations are congenital vascular malformations in the lungs, which act as shunts so that the blood is not oxygenated or filtered. These patients are typically hypoxaemic with exercise intolerance and are at high risk of paradoxical emboli to the brain. About 25...... technical success and few complications. Embolisation prevents cerebral stroke and abscess and pulmonary haemorrhage and further raises the functional level. Screening for pulmonary arteriovenous malformations in patients at risk is recommended....

  10. A severe case of astrogliosis and encephalomalacia in a neonate with a massive vein of Galen malformation (VGM

    Directory of Open Access Journals (Sweden)

    F Ismail

    2011-06-01

    Full Text Available Vein of Galen malformations (VGMs are rare anomalies of intracranial circulation that constitute 1% of all intracranial vascular malformations. We describe a case of severe encephalomalacia associated with a VGM, which has not been previously described.

  11. Fetal magnetic resonance imaging. Diagnostics in cases of congenital cystadenomatoid malformation; Fetale Magnetresonanztomographie. Diagnostik bei zystadenomatoider Malformation der Lunge (CCAM)

    Energy Technology Data Exchange (ETDEWEB)

    Buesing, K.A.; Kilian, A.K.; Neff, K.W. [Fakultaet fuer Klinische Medizin der Universitaet Heidelberg, Institut fuer Klinische Radiologie, Universitaetsklinikum Mannheim, Mannheim (Germany); Schaible, T. [Fakultaet fuer Klinische Medizin der Universitaet Heidelberg, Kinderklinik, Universitaetsklinikum Mannheim, Mannheim (Germany)

    2006-02-15

    Despite advancing therapeutic strategies, congenital cystadenomatoid malformation of the fetal lung is still a potentially life-threatening anomaly. Antenatally, the development of hydrops as well as the natural history of the malformation is of particular therapeutic and prognostic importance. Postnatally, respiratory distress due to pulmonary hypoplasia counts as a crucial limiting factor. Owing to its feasibility and practicability, as well as a high sensitivity, antenatal ultrasound is still the screening method of choice for the detection of fetal thoracic malformations. However, particularly in cases of indistinguishable sonographic findings, fetal MRI is the modality of choice for proving the diagnosis and preliminary appraisal of intensive care therapy and extracorporal membrane oxygenation postnatally. Furthermore, fetal MRI often facilitates assessment and planning of intrauterine surgical procedures. These two features frequently require a close transfer to an expert neonatal centre. (orig.) [German] Trotz verbesserter Therapien stellt die kongenitale zystadenomatoide Malformation der Lunge fuer den Feten weiterhin eine potenziell lebensbedrohliche Missbildung dar. Praenatal sind insbesondere ein Hydrops fetalis sowie der Spontanverlauf von prognostischer und therapeutischer Relevanz. Postnatal kommt der Lungenhypoplasie eine entscheidende Bedeutung zu. Aufgrund der hohen Verfuegbarkeit und Sensitivitaet bleibt die Primaerdiagnostik weiterhin dem Ultraschall vorbehalten. Die fetale MRT hingegen hat sich insbesondere bei unklarer sonographischer Befundlage als Methode der Wahl etabliert. Neben der Diagnosesicherung erleichtert sie durch eine uebersichtliche Darstellung der Befundausdehnung haeufig die Abschaetzung einer postnatalen Intensivtherapie. Mit zunehmender Erfahrung beim Einsatz fetalchirurgischer Verfahren kommt ihr ferner eine wachsende Bedeutung in der Indikationsstellung und Planung praenataler Therapien zu. Beides erfordert u. U. die

  12. Outcome of Kidney Allografts in Recipients With a Femoral Arteriovenous Fistula: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    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.

  13. Fetal macrosomia as an important indicator of fetal malformation syndrome: ultrasonic findings of two cases

    NARCIS (Netherlands)

    PA de Jong; MD E.J.M. Wouters; EA Pley

    1989-01-01

    Two extraordinary cases of fetal macrosomia are presented. It is discussed that extreme fetal growth should raise the suspicion of a malformation syndrome and deserves thorough antenatal ultrasonographic examination.

  14. [A case of multiple arteriovenous fistulae in a postphlebitic syndrome of longterm evolution].

    Science.gov (United States)

    Delgado Daza, R; Moga Donadeu, L; Mañosa Bonamich, J; Muncunill Gil, J; Vidal Conde, V

    1992-01-01

    Authors report a case of a 77-years-old man who, after an accident of traffic on 1968, presented a left ileo-femoral deep venous thrombosis. Consequently, the patient suffered and important postphlebitic syndrome, with several varicose packs which were treated by surgical procedure in other centre. During several years, patient presented severe trophic diseases. Seventeen years after the beginning of his pathology, and during an angiologic examination, multiple arteriovenous fistulas at the left ileofemoral area have been shown. An skeletalization was impossible because of the severe ulcerations of the leg; so a left iliac arterial ligature and a Dacron Banding in primitive iliac artery, reducing a 50% the diameter, were made. The postoperative result was excellent, with an spectacular reduction of the fistulous communications and a complete remission of the cutaneous ulcerations.

  15. Cardiovascular Malformations in CHARGE Syndrome with DiGeorge Phenotype: Two Case Reports

    Directory of Open Access Journals (Sweden)

    Kazushi Yasuda

    2016-01-01

    Full Text Available Both CHARGE syndrome and DiGeorge anomaly are frequently accompanied by cardiovascular malformations. Some specific cardiovascular malformations such as interrupted aortic arch type B and truncus arteriosus are frequently associated with 22q11.2 deletion syndrome, while conotruncal defects and atrioventricular septal defects are overrepresented in patients with CHARGE syndrome. CHD7 gene mutation is identified in approximately two-thirds of patients with CHARGE syndrome, and chromosomal microdeletion at 22q11.2 is found in more than 95% of patients with 22q11.2 deletion syndrome. CHARGE syndrome is occasionally accompanied by DiGeorge phenotype. We report two patients with dysmorphic features of both CHARGE syndrome and 22q11.2 deletion syndrome. Although both of the two cases did not have 22q11.2 deletion, they had typical dysmorphic features of 22q11.2 deletion syndrome including cardiovascular malformations such as interrupted aortic arch type B. They also had characteristic features of CHARGE syndrome including ear malformation, genital hypoplasia, limb malformation, and endocrinological disorders. CHD7 gene mutation was confirmed in one of the two cases. When a patient with cardiovascular malformations frequently associated with 22q11.2 deletion syndrome does not have 22q11.2 deletion, we suggest that associated malformations characteristic of CHARGE syndrome should be searched for.

  16. 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

  17. Uncommon cavernous malformation of the optic chiasm: a case report

    Directory of Open Access Journals (Sweden)

    Ning Xianbin

    2012-08-01

    Full Text Available Abstract Cavernous malformation (CM is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans.

  18. Multiple de novo vascular malformations in relation to diffuse venous occlusive disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Desal, H.A. [Hopital Laennec, University of Nantes, Department of Neuroradiology, Nantes (France); Toronto Western Hospital, Department of Medical Imaging, University Health Network, Toronto, Ont. (Canada); Lee, S.K.; Kim, B.S.; TerBrugge, K.G. [Toronto Western Hospital, Department of Medical Imaging, University Health Network, Toronto, Ont. (Canada); Raoul, S.; Tymianski, M. [Toronto Western Hospital, Department of Neurosurgery, University Health Network, Toronto, Ont. (Canada)

    2005-01-01

    Brain vascular malformations are dynamic disorders. Although mostly considered to be of congenital origin, the improvement of clinical imaging and vasculogenesis knowledge has shown that they might also result from a biological dysfunction of the remodeling process after birth. Venous occlusive disease and ishemia may represent powerful revealing triggers and support the capillary venous origin of some vascular malformations. We report a unique case of the development of multiple de novo vascular malformations (transverse sinus dural fistula and posterior fossa cavernomas) following acoustic neuroma surgery. (orig.)

  19. High anorectal malformation in a five-month-old boy: a case report

    Directory of Open Access Journals (Sweden)

    Pandey Anand

    2010-08-01

    Full Text Available Abstract Introduction Anorectal malformation, one of the most common congenital defects, may present with a wide spectrum of defects. Almost all male patients present within first few days of life. Case presentation A five-month-old baby boy of Indian origin and nationality presented with anal atresia and associated rectourethral prostatic fistula. The anatomy of the malformation and our patient's good condition permitted a primary definitive repair of the anomaly. A brief review of the relevant literature is included. Conclusion Delayed presentation of a patient with high anorectal malformation is rare. The appropriate treatment can be rewarding.

  20. Tentorial dural arteriovenous fistula successfully treated with surgical interruption of leptomeningeal venous drainage:a case report

    Institute of Scientific and Technical Information of China (English)

    LI Mei-hua; XU Geng-sheng; LI Yi-yun

    2005-01-01

    @@ Tentorial dural arteriovenous fistula (DAVF) is a rare disease, accounting for some 4% of all cases of intracranial DAVF.1 Because of a high risk of intracranial hemorrhage, patients with DAVF need aggressive treatment. Despite recent advances in endovascular technology, many researchers2-4 advocate open surgery for the treatment of tentorial DAVF. In this report, we present a case of tentorial DAVF with pure leptomeningeal venous drainage successfully treated by interruption of the draining vein.

  1. Congenital Cystic Adenomatoid Malformation of Lung-Rare Case Report

    Directory of Open Access Journals (Sweden)

    N. S. Kamakeri

    2016-10-01

    Full Text Available Congenital cystic adenomatoid malformation of lung associated with Cystic dysplasia of kidney, cystic disease of liver with mixed gonadal dysgenesis is rare and is not reported in literature so far. Hence an attempt is made to present this rarest entity.

  2. Isolated unilateral trismus as a presentation of Chiari malformation: case report.

    Science.gov (United States)

    Feinberg, Michelle; Babington, Parker; Sood, Shawn; Keating, Robert

    2016-05-01

    The authors present a case of Chiari malformation manifesting as isolated trismus, describe the typical symptoms associated with Chiari malformation, and discuss the potential anatomical causes for this unique presentation. A 3-year-old boy presented with inability to open his jaw for 6 weeks with associated significant weight loss. The results of medical and radiological evaluation were negative except for Type I Chiari malformation with cerebellar tonsils 12 mm below the level of the foramen magnum. The patient underwent Chiari decompression surgery. Postoperatively, his ability to open his mouth was significantly improved, allowing resumption of a regular diet. Postoperative MRI revealed almost complete resolution of the syringobulbia. To the best of the authors' knowledge, this is the first reported case of isolated trismus from Chiari malformation with syringobulbia.

  3. Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases.

    Science.gov (United States)

    Holekamp, Terrence F; Mollman, Matthew E; Murphy, Rory K J; Kolar, Grant R; Kramer, Neha M; Derdeyn, Colin P; Moran, Christopher J; Perrin, Richard J; Rich, Keith M; Lanzino, Giuseppe; Zipfel, Gregory J

    2016-06-01

    Nonhemorrhagic neurological deficits are underrecognized symptoms of intracranial dural arteriovenous fistulas (dAVFs) having cortical venous drainage. These symptoms are the consequence of cortical venous hypertension and portend a clinical course with increased risk of neurological morbidity and mortality. One rarely documented and easily misinterpreted type of nonhemorrhagic neurological deficit is progressive dementia, which can result from venous hypertension in the cortex or in bilateral thalami. The latter, which is due to dAVF drainage into the deep venous system, is the less common of these 2 dementia syndromes. Herein, the authors report 4 cases of dAVF with venous drainage into the vein of Galen causing bithalamic edema and rapidly progressive dementia. Two patients were treated successfully with endovascular embolization, and the other 2 patients were treated successfully with endovascular embolization followed by surgery. The radiographic abnormalities and presenting symptoms rapidly resolved after dAVF obliteration in all 4 cases. Detailed descriptions of these 4 cases are presented along with a critical review of 15 previously reported cases. In our analysis of these 19 published cases, the following were emphasized: 1) the clinical and radiographic differences between dAVF-induced thalamic versus cortical dementia syndromes; 2) the differential diagnosis and necessary radiographic workup for patients presenting with a rapidly progressive thalamic dementia syndrome; 3) the frequency at which delays in diagnosis occurred and potentially dangerous and avoidable diagnostic procedures were used; and 4) the rapidity and completeness of symptom resolution following dAVF treatment.

  4. Endoscopic endonasal transclival resection of a ventral pontine cavernous malformation: technical case report.

    Science.gov (United States)

    Gómez-Amador, Juan Luis; Ortega-Porcayo, Luis Alberto; Palacios-Ortíz, Isaac Jair; Perdomo-Pantoja, Alexander; Nares-López, Felipe Eduardo; Vega-Alarcón, Alfredo

    2016-10-21

    Brainstem cavernous malformations are challenging due to the critical anatomy and potential surgical risks. Anterolateral, lateral, and dorsal surgical approaches provide limited ventral exposure of the brainstem. The authors present a case of a midline ventral pontine cavernous malformation resected through an endoscopic endonasal transclival approach based on minimal brainstem transection, negligible cranial nerve manipulation, and a straightforward trajectory. Technical and reconstruction technique advances in endoscopic endonasal skull base surgery provide a direct, safe, and effective corridor to the brainstem.

  5. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report

    OpenAIRE

    Luis Rafael Moscote-Salazar; Nasly Zabaleta-Churio; Gabriel Alcala-Cerra; Andres M. Rubiano; Willem Guillermo Calderon-Miranda; Hernando Raphael Alvis-Miranda; Amit Agrawal

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomy...

  6. Medullary Venous Hypertension Secondary to a Petrous Apex Dural Arteriovenous Fistula: A Case Report

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

  8. Auditory dysfunction in selected syndromes and patterns of malformations: review and case findings.

    Science.gov (United States)

    Hall, J W; Prentice, C H; Smiley, G; Werkhaven, J

    1995-01-01

    Hundreds of syndromes may be associated with hearing impairment. Within recent years, there has been heightened research and clinical interest in hearing and syndromes, probably due to both advances in molecular genetics and improved techniques for auditory assessment of infants and young children. An example of the current appreciation for the possible relation between auditory dysfunction and syndromes or patterns of malformations is the inclusion of syndromes as a risk factor for hearing impairment by the 1990 Joint Committee on Infant Hearing. In this paper, we review syndromes and patterns of malformation that are frequently or occasionally associated with hearing impairment. In addition, we illustrate, in case studies, auditory findings that may be associated with three of these syndromes and patterns of malformation: Carpenter syndrome, ichthyosiform dermatosis, and Townes syndrome. We conclude with a discussion of strategies for audiologic assessment and management of infants and children with syndromes and patterns of malformations.

  9. Congenital malformations and maternal consumption of benzodiazepines: a case-control study.

    Science.gov (United States)

    Laegreid, L; Olegård, R; Conradi, N; Hagberg, G; Wahlström, J; Abrahamsson, L

    1990-05-01

    This study assessed potential teratogenic properties of benzodiazepine (BZD) intake during early pregnancy. Four neonatal diagnoses of congenital malformations (embryopathy and fetopathy, unspecified; unspecified congenital malformations of the nervous system; cleft palate and cleft lip; congenital malformations of the urinary tract) were selected. The authors' previous clinical experience had shown these diagnoses to be characteristic of infants born to mothers with excessive intake of BZD in early pregnancy. The selected diagnoses were present in 25 of 10,646 liveborn infants (2.3 per 1000) delivered by mothers living in the city of Gothenburg in 1985 and 1986. In 18 of these cases, it was possible to analyse maternal plasma, and eight samples (44 per cent) were found to be BZD-positive. Of 60 controls, two maternal blood samples (3 per cent) were positive for BZD. The difference is highly significant and suggests an association between these congenital malformations and BZD consumed during early gestation.

  10. Giant Pseudoaneurysm Associated with Arteriovenous Fistula of the Brachial and Femoral Arteries following Gunshot Wounds: Report of Two Cases

    Science.gov (United States)

    Daniel, Handy Eone; Firmin, Ankouane; Angele, Pondy O.; Esthelle, Minka Ngom; Freddy, Bombah; Bernadette, Ngo Nonga

    2015-01-01

    Posttraumatic pseudoaneurysm associated with arteriovenous fistula of the upper or lower limb is exceptional. We are reporting herein the history of two cases in civil life that have been followed and repaired in our service. Both patients were shot more than a year before being referred to our tertiary hospital for an enlarging mass which was a pseudoaneurysm associated with an arteriovenous fistula. The aneurysm was repaired and the fistula closed. Due to the absence of well-trained professionals, vascular injuries and their complications are usually discovered late in Cameroon while these pseudoaneurysms can reach very dramatic sizes. This presentation intends to raise the attention on a careful clinical exam and search of vascular lesion in the case of penetrating wound of the limb associated with profuse bleeding. PMID:25705543

  11. Giant Pseudoaneurysm Associated with Arteriovenous Fistula of the Brachial and Femoral Arteries following Gunshot Wounds: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Handy Eone Daniel

    2015-01-01

    Full Text Available Posttraumatic pseudoaneurysm associated with arteriovenous fistula of the upper or lower limb is exceptional. We are reporting herein the history of two cases in civil life that have been followed and repaired in our service. Both patients were shot more than a year before being referred to our tertiary hospital for an enlarging mass which was a pseudoaneurysm associated with an arteriovenous fistula. The aneurysm was repaired and the fistula closed. Due to the absence of well-trained professionals, vascular injuries and their complications are usually discovered late in Cameroon while these pseudoaneurysms can reach very dramatic sizes. This presentation intends to raise the attention on a careful clinical exam and search of vascular lesion in the case of penetrating wound of the limb associated with profuse bleeding.

  12. 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.)

  13. 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

  14. Endovascular management of early-onset post-nephrectomy renal arteriovenous fistula: A report of two cases

    Directory of Open Access Journals (Sweden)

    Henghui Yin

    2015-12-01

    Full Text Available Purpose: Here, we report two cases of early-onset post-nephrectomy renal arteriovenous fistula who were successfully managed by implantation of patent ductus arteriosus occluders. Case report: Both patients were female, aged 38 and 36 years. They received left renal nephrectomy 9 and 6 months, respectively, with a complaint of chest congestion and dyspnea before admitting to our center. Computed tomographic angiography revealed a huge arteriovenous fistula of the left renal pedicle with a renal venous aneurysm in both patients. The fistulas were isolated by implanting patent ductus arteriosus occluders in the renal artery stumps. Clinical symptoms disappeared after intervention. Computed tomographic angiography confirmed the effectiveness of the occluders during follow-up time. The venous aneurysms shrank to normal size. Conclusion: Our experience indicates that post-nephrectomy renal arteriovenous fistula can present as an early complication which can be efficiently managed by endovascular occlusion of the arterial stump by patent ductus arteriosus occluder.

  15. Posterior cranial fossa single-hole arteriovenous fistulae in children: 14 consecutive cases

    Energy Technology Data Exchange (ETDEWEB)

    Yoshida, Y.; Weon, Y.C.; Sachet, M.; Mahadevan, J.; Alvarez, H.; Rodesch, G.; Lasjaunias, P. [Service de Neuroradiologie Diagnostique et Therapeutique, CHU de Bicetre, 78 rue du General Leclerc, 94275, Le Kremlin Bicetre (France)

    2004-06-01

    We report 14 consecutive children with 23 posterior cranial fossa arteriovenous fistula (AVF); six had multifocal lesions, involving the supratentorial brain in three and the spinal cord in one. There were two boys and four girls with a family history compatible with hereditary haemorrhagic telangiectasia. The diagnosis was made in infancy in eight cases and in a further six before the age of 12 years; mean age at diagnosis was 3.5 years. The male-to-female ratio was 1.8:1. Presenting features were macrocrania in four cases, haemorrhage or headache in three and nonhaemorrhagic neurological deficits or and cardiac overload in two. Dominant supply to the symptomatic fistula arose from the posterior inferior cerebellar artery in five cases, anterior inferior cerebellar artery in two and the upper basilar artery system in seven. All children were primarily treated by transarterial embolisation. We treated thirteen children (93%) by transarterial embolisation alone; one older child with a history of haemorrhage also underwent radiosurgery. We obtained 100% exclusion of the fistula(e) in six children, 95-80% in five, 80-50% in one and <50% in one. Of the incompletely treated cases, three had conservative management, and two with 80% and one with 60% reduction of their lesion are scheduled for elective treatment; two partially treated case died. There was no morbidity due to the endovascular procedures. Follow-up since referral is 6 months-10 years (mean 4.5 years). Ten children are neurologically normal, two have persistent (pre-existing) neurological deficits and two are dead. (orig.)

  16. Perkutan transluminal embolisation af pulmonale arteriovenøse malformationer

    DEFF Research Database (Denmark)

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

    2001-01-01

    The aim was to evaluate the effect of embolisation of pulmonary arteriovenous malformations (PAVM), as estimated by contrast echocardiography, arterial blood-gas analyses and functional level, and further to evaluate procedure-related and late complications.......The aim was to evaluate the effect of embolisation of pulmonary arteriovenous malformations (PAVM), as estimated by contrast echocardiography, arterial blood-gas analyses and functional level, and further to evaluate procedure-related and late complications....

  17. 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 ...

  18. 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

  19. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report

    Directory of Open Access Journals (Sweden)

    Luis Rafael Moscote-Salazar

    2016-01-01

    Full Text Available Chiari malformation Type I (CM-I is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition.

  20. [Maxillary sinus malformations (double wall bone) lead to chronic sinusitis in a case].

    Science.gov (United States)

    Pang, Chaofu; Gu, Feng; Fang, Yingqi

    2014-09-01

    Chronic rhinosinusitis is a common otorhinolaryngological disease, although the incidence of chronic sinusitis is the result of many factors, the local anatomic abnormalities is one of the most important reasons. When maxillary sinus dysplasia that sinus cavity becomes small. These was some sinus cavity partial or complete bony septum malformation used to be reported occasionally, according to reports in the literature of this malformation rate is below 2%, bony divides sinus cavity is divided into 2 to 3 independent lacunar deformity are very rare, next we will introduce a case of sinus cavity that been divided into double deck by bone wall.

  1. Symptomatic Chiari Malformation with Syringomyelia after Severe Traumatic Brain Injury: Case Report.

    Science.gov (United States)

    Moscote-Salazar, Luis Rafael; Zabaleta-Churio, Nasly; Alcala-Cerra, Gabriel; M Rubiano, Andres; Calderon-Miranda, Willem Guillermo; Alvis-Miranda, Hernando Raphael; Agrawal, Amit

    2016-01-01

    Chiari malformation Type I (CM-I) is a congenital disorder, which is basically a tonsillar herniation (≥ 5 mm) below the foramen magnum with or without syringomyelia. The real cause behind this malformation is still unknown. Patients may remain asymptomatic until they engender a deteriorating situation, such as cervical trauma. The objective of this case report is to give a broad perspective on CM-I from the clinical findings obtained in a patient with asymptomatic non-communicating syringomyelia associated with a CM-I exacerbated within 2 years of a TBI, and to discuss issues related to that condition.

  2. The clinico-radiological spectrum of masseteric venous malformation: Case report.

    Science.gov (United States)

    Agarwal, Kavita; Umarji, Hemant R; Kadam, Sonali; Agrawal, Neeraj

    2016-11-01

    Due to its location, intra-masseteric venous malformation often mistaken for a parotid swelling and clinical examination alone frequently underestimates the deep extension of the lesion and rarely gives accurate pre-operative diagnosis. But once it is diagnosed, the feasibility of the treatment and the plan of approach depend on accurate delineation of the extent, size and location of the lesion. Therefore, complimentary radiographic studies are essential for its management. In this case report, typical features of venous malformation within the masseter muscle, including clinical findings (turkey wattle sign) and imaging are presented. Ultrasonography and CT scan were non-contributory in the diagnosis, while on MRI, masseteric venous malformations have a typical appearance that allowed early identification, patient education and its management.

  3. The Vanishing Twin Syndrome: Two Cases of Extreme Malformations Associated With Vanished Twins.

    Science.gov (United States)

    Shinnick, Julia K; Khoshnam, Nasim; Archer, Sydney R; Quigley, Philip C; Robinson, Haynes; Keene, Sarah; Santore, Matthew T; Hill, Sarah; Patel, Binita; Shehata, Bahig M

    2017-01-01

    Two cases of devastating fetal malformations associated with vanished monochorionic twins were identified upon review of pathology files. A 35-year-old G1P0 woman and 36-year-old G3P1 woman were both diagnosed with an intrauterine twin gestation via transvaginal ultrasound at 10 weeks. The spectrum of fetal anomalies ranged from omphalocele, bilateral upper extremity, and unilateral lower extremity hypoplasia, to craniofacial malformation with diaphragmatic hernia. On histopathologic examination, the placentas demonstrated vascular anastomoses between the surviving co-twin and the "vanished" fetal sac. We propose anastomotic placental vasculature as a contributing factor to the observed fetal malformations. Additionally, genetic or teratogenic factors may have been attributed to the demise of the first twin and the anomalies seen in the other twin. While such instances are rare, they are important to consider when counseling patients regarding outcomes associated with a monochorionic vanished twin.

  4. Meckel-Gruber syndrome concomitant with Dandy-Walker malformation: prenatal sonographic diagnosis in two cases.

    Science.gov (United States)

    Yapar, E G; Ekici, E; Dogan, M; Gökmen, O

    1996-10-01

    Meckel-Gruber syndrome is an autosomal recessive disorder which comprises a characteristic triad of major abnormalities: renal cystic dysplasia, occipital encephalocele, and postaxial polydactyly. Because of the recessive inheritance, prenatal sonographic diagnosis is paramount for informed genetic counselling of affected pregnancies. However, Meckel-Gruber syndrome may demonstrate variation in phenotypic expression when some malformations are different from those traditionally accepted and cases may be evaluated as a different syndrome. The aim of this paper is to emphasise the phenotypic variability in Meckel-Gruber syndrome, and the importance of the prenatal sonography in the diagnosis. We also suggest that Dandy-Walker malformation or Dandy-Walker variant be accepted as one of the malformations which occur in the central nervous system as a part of the syndrome.

  5. Intrauterine exposure to carbamazepine and specific congenital malformations : systematic review and case-control study

    NARCIS (Netherlands)

    Jentink, Janneke; Dolk, Helen; Loane, Maria A.; Morris, Joan K.; Wellesley, Diana; Garne, Ester; de Jong-van den Berg, Lolkje

    2010-01-01

    Objective To identify specific major congenital malformations associated with use of carbamazepine in the first trimester of pregnancy. Design A review of all published cohort studies to identify key indications and a population based case-control study to test these indications. Setting Review of P

  6. [Intracranial dural arteriovenous fistulae. Experience after 81 cases and literature review].

    Science.gov (United States)

    Paredes, Igor; Martinez-Perez, Rafael; Munarriz, Pablo M; Castaño-Leon, Ana María; Campollo, Jorge; Alén, Jose F; Lobato, Ramiro D; Lagares, Alfonso

    2013-01-01

    To analyse the clinical, radiological and therapeutic variables of intracranial dural arteriovenous fistulae (DAVF) treated at our institution, and to assess the validity of the Borden and Cognard classifications and their correlation with the presenting symptoms. The DAVF identified were retrospectively analysed. They were classified according to their location, drainage pattern and the Borden and Cognard classifications. We recorded the different treatments, their complications and efficacy. There were 81DAVF identified between 1975 and 2012. The cavernous sinus (CS) location was the most frequent one. The Borden and Cognard classifications showed an interobserver Kappa index of 0.72 and 0.76 respectively. The odds ratio of aggressive presentation in the presence of cortical venous drainage (CVD) was 19.3 (2.8-132.4). No location, once adjusted by venous drainage pattern, showed significant association with an aggressive presentation. Endovascular transarterial treatment of cavernous sinus DAVF achieved symptomatic improvement of 78%, with a complication rate of 5%. The DAVF of non-CS locations, with CVD, treated surgically were angiographically shown cured in 100% of the cases, with no treatment-related complications. The presence of CVD was significantly associated with aggressive presentations. The Borden and Cognard classifications showed little interobserver variability. Endovascular treatment for CS DAVF is safe and relatively effective. Surgical treatment of non-CS DAVF with CVD is safe, effective and the first choice treatment in our environment. Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  7. Multimodality evaluation of dural arteriovenous fistula with CT angiography, MR with arterial spin labeling, and digital subtraction angiography: case report.

    Science.gov (United States)

    Alexander, Matthew; McTaggart, Ryan; Santarelli, Justin; Fischbein, Nancy; Marks, Michael; Zaharchuk, Greg; Do, Huy

    2014-01-01

    Dural arteriovenous fistulae (DAVF) are cerebrovascular lesions with pathologic shunting into the venous system from arterial feeders. Digital subtraction angiography (DSA) has long been considered the gold standard for diagnosis, but advances in noninvasive imaging techniques now play a role in the diagnosis of these complex lesions. Herein, we describe the case of a patient with right-side pulsatile tinnitus and DAVF diagnosed using computed tomography angiography, magnetic resonance with arterial spin labeling, and DSA. Implications for imaging analysis of DAVFs and further research are discussed.

  8. Genetic variation in the functional ENG allele inherited from the non-affected parent associates with presence of pulmonary arteriovenous malformation in hereditary hemorrhagic telangiectasia 1 (HHT1) and may influence expression of PTPN14.

    Science.gov (United States)

    Letteboer, Tom G W; Benzinou, Michael; Merrick, Christopher B; Quigley, David A; Zhau, Kechen; Kim, Il-Jin; To, Minh D; Jablons, David M; van Amstel, Johannes K P; Westermann, Cornelius J J; Giraud, Sophie; Dupuis-Girod, Sophie; Lesca, Gaetan; Berg, Jonathan H; Balmain, Allan; Akhurst, Rosemary J

    2015-01-01

    HHT shows clinical variability within and between families. Organ site and prevalence of arteriovenous malformations (AVMs) depend on the HHT causative gene and on environmental and genetic modifiers. We tested whether variation in the functional ENG allele, inherited from the unaffected parent, alters risk for pulmonary AVM in HHT1 mutation carriers who are ENG haploinsufficient. Genetic association was found between rs10987746 of the wild type ENG allele and presence of pulmonary AVM [relative risk = 1.3 (1.0018-1.7424)]. The rs10987746-C at-risk allele associated with lower expression of ENG RNA in a panel of human lymphoblastoid cell lines (P = 0.004). Moreover, in angiogenically active human lung adenocarcinoma tissue, but not in uninvolved quiescent lung, rs10987746-C was correlated with expression of PTPN14 (P = 0.004), another modifier of HHT. Quantitative TAQMAN expression analysis in a panel of normal lung tissues from 69 genetically heterogeneous inter-specific backcross mice, demonstrated strong correlation between expression levels of Eng, Acvrl1, and Ptpn14 (r2 = 0.75-0.9, P ENG gene influences quantitative and/or qualitative differences in ENG expression that contribute to risk of pulmonary AVM in HHT1, and provide correlative support for PTPN14 involvement in endoglin/ALK1 lung biology in vivo. PTPN14 has been shown to be a negative regulator of Yap/Taz signaling, which is implicated in mechanotransduction, providing a possible molecular link between endoglin/ALK1 signaling and mechanical stress. EMILIN2, which showed suggestive genetic association with pulmonary AVM, is also reported to interact with Taz in angiogenesis. Elucidation of the molecular mechanisms regulating these interactions in endothelial cells may ultimately provide more rational choices for HHT therapy.

  9. 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.

  10. Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder

    Directory of Open Access Journals (Sweden)

    Shantanu Kumar

    2011-01-01

    Full Text Available Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

  11. A case report of a pediatric traumatic aneurysm with arteriovenous (A-V) fistula CASE-BASED UPDATE.

    Science.gov (United States)

    Anami, Hidenori; Aihara, Yasuo; Kawashima, Akitsugu; Yamaguchi, Koji; Nagahara, Ayumi; Okada, Yoshikazu

    2014-12-01

    Traumatic intracranial aneurysms (TICAs) are rare, representing less than 5 % of all intracranial aneurysms. Most TICAs are located within the anterior circulation, and less than 10 % of TICAs occur in the posterior circulation. Histopathologically, most TICAs are false aneurysms and have a high risk of rupture or re-bleeding. When they are discovered, careful observation or treatment may be required. Once they are enlarged, they have a high risk of rupture, and immediate treatment is essential. For the first time in the literature, we report a rare case of an 8-year-old boy with a TICA in anterior inferior cerebellar artery (AICA) and arteriovenous (A-V) fistula after severe head trauma. Trapping of AICA and resection of the aneurysm was performed. Postoperatively, he has peripheral facial palsy but is otherwise neurologically normal. Surgical treatment for traumatic aneurysms is challenging because most of TICAs are histopathologically pseudoaneurysms. It may be technically difficult to perform direct surgery of aneurysms especially located in the posterior circulation, but when the treatment is successful, the outcome was favorable.

  12. Rendu-Osler-Weber syndrome: what radiologists should know. Literature review and three cases report; Sindrome de Rendu-Osler-Weber: o que o radiologista precisa saber: revisao da literatura e apresentacao de tres casos

    Energy Technology Data Exchange (ETDEWEB)

    Agnollitto, Paulo Moraes; Barreto, Andre Rodrigues Facanha; Barbieri, Raul Fernando Pinsetta; Junior, Jorge Elias; Muglia, Valdair Francisco, E-mail: agno53@gmail.com [Universidade de Sao Paulo (HC/FMRP/USP), Ribeirao Preto, SP (Brazil). Fac. de Medicina. Hospital das Clinicas

    2013-05-15

    Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia is an autosomal dominant vascular disease involving multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Most common symptoms include skin and mucosal telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. The key imaging finding is the presence of visceral arteriovenous malformations. The diagnosis is based on clinical criteria and can be confirmed by molecular biology techniques. Treatment includes measures for management of epistaxis, as well as surgical excision, radiotherapy and embolization of arteriovenous malformations, with emphasis on endovascular treatment. The present pictorial essay includes a report of three typical cases of this entity and a literature review. (author)

  13. Congenital Malformations of the Inner Ear: Case Series and Review of the Literature.

    Science.gov (United States)

    Piromchai, Patorn; Kasemsiri, Pornthep; Thanawirattananit, Panida; Yimtae, Kwanchanok

    2015-08-01

    Patients with craniofacial anomalies often present to doctors due to their noticeable disfigurement and are routinely assessed by otolaryngologists for hearing evaluation. However, small percentage of craniofacial anomaly patients may present with delayed speech though they may not have initial obvious external deformation. The objective of case series is to identify the congenital inner ear malformation. The series of clinical presentation, physical examination, investigations, treatments and follow-up results were demonstrated followed by the discussion.

  14. [Neonatal pneumothorax and urinary tract malformation. 3 cases].

    Science.gov (United States)

    Chabrolle, J P; Brioude, R; Guerin, J; de Montis, G

    1976-10-01

    Three newborn male infants are reported who presented with a tension pneumothorax in the first few hours of life. Early in one case and after intervals of one month and eight months in the others attention was drawn to the urinary tract. Radiological investigations revealed morphological abnormalities of the kidneys and the urinary tract itself. The pathogenesis is discussed and an intravenous pyelogram is recommended before the development of clinical features. In this way early diagnosis might allow treatment prior to renal involvement.

  15. Traumatic coronary arteriovenous fistula communicating the left main coronary artery to pulmonary artery, associated with pulmonary valvular insufficiency and endocarditis: case report.

    Science.gov (United States)

    Rangel, A; Badui, E; Verduzco, C; Valdespino, A; Enciso, R

    1990-02-01

    The authors present a case of a seventeen-year-old white male who suffered from a knife chest wound and secondarily developed a traumatic coronary arteriovenous fistula communicating the left main coronary artery to the pulmonary artery, associated with pulmonary valvular insufficiency and endocarditis.

  16. Bilateral Vocal Cord Palsy with Arnold Chiari Malformation: A Rare Case Series

    Science.gov (United States)

    Arora, Nikhil; Meher, Ravi; Bhargava, Eishaan K.

    2016-01-01

    Stridor in paediatric age group is not an uncommon presentation to the ENT emergency. The range of differential diagnosis is vast. The presentation may vary from noisy breathing to severe respiratory distress and apnea. Early and meticulous diagnosis is crucial for the management as the condition may be life threatening. We report a rare case series of 3 infants with Arnold Chiari Malformation who presented to the hospital with stridor and were diagnosed with bilateral vocal cord palsy. These 3 infants had similar underlying neurological condition with hydrocephalus and raised intracranial pressure. Chiari malformation is the one of the most common congenital central nervous system anomaly associated with bilateral vocal cord paralysis. However, the presentation is rare. This article, thus, emphasizes the significance of early diagnosis and immediate management of this condition. PMID:27790480

  17. [Pyelonephritis with massive renal tissue necrosis in child with urinary tract malformation--a case report].

    Science.gov (United States)

    Pawlak-Bratkowska, Monika; Finke, Daria; Olejniczak, Dariusz; Midel, Anna; Tkaczyk, Marcin

    2009-04-01

    The aim of the case report is presentation of unusual and heavy clinical course of pyelonephritis with renal tissue necrosis in a child with urinary tract malformation. Nine month old girl was admitted to hospital in heavy clinical status due to pyelonephritis--urosepsis. It was complicated by acute renal insufficiency. Patient was treated by broad-spectrum antibiotics and parenteral nutrition. She was feverish for 14 days. Computed tomography done in order to exclude abdominal abscess showed massive renal tissue necrosis of on both sides. Antibiotic treatment was successful after 6 weeks. Urological evaluation revealed bilateral vesico-ureteral refluxes grade IV. Scintigraphy showed multiple scars. Patient was treated Deflux injections (twice). We noted 5 urinary tract recurrences despite antibiotic profilaxis. GFR of 75 ml/min/1.73 m2 was estimated at age of 16 m. Immunodeficiency or malignancy as background of clinical course were excluded. The case we describe presents severe clinical course of pyelonephritis due to complex urinary tract malformation that is to be considered despite based on modern publications "sparing" strategies of diagnosis and profilaxis in urinary tract malformations.

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

    Directory of Open Access Journals (Sweden)

    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

  19. 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.

  20. Superior mesenteric arteriovenous fistula presenting as gastrointestinal bleeding: case report and literature review

    Directory of Open Access Journals (Sweden)

    Chong Wang

    Full Text Available Superior mesenteric arteriovenous fistula (SMAVF is a rare vascular disorder usually following penetrating abdominal trauma or gastrointestinal surgery. Percutaneous endovascular treatment such as embolization, has been widely used to treat this disease. We report a patient, who was presented with melena at the onset of his symptoms, then an acute hematemesis in shock. A SMAVF was diagnosed on an angiogram after a large mesenteric vein was seen on CT. The patient had a successful emergency endoscopic variceal ligation (EVL to stop bleeding. Then the patient received fistula embolization with covered stent.

  1. Dose fractionated gamma knife radiosurgery for large arteriovenous malformations on daily or alternate day schedule outside the linear quadratic model: Proof of concept and early results. A substitute to volume fractionation.

    Science.gov (United States)

    Mukherjee, Kanchan Kumar; Kumar, Narendra; Tripathi, Manjul; Oinam, Arun S; Ahuja, Chirag K; Dhandapani, Sivashanmugam; Kapoor, Rakesh; Ghoshal, Sushmita; Kaur, Rupinder; Bhatt, Sandeep

    2017-01-01

    To evaluate the feasibility, safety and efficacy of dose fractionated gamma knife radiosurgery (DFGKRS) on a daily schedule beyond the linear quadratic (LQ) model, for large volume arteriovenous malformations (AVMs). Between 2012-16, 14 patients of large AVMs (median volume 26.5 cc) unsuitable for surgery or embolization were treated in 2-3 of DFGKRS sessions. The Leksell G frame was kept in situ during the whole procedure. 86% (n = 12) patients had radiologic evidence of bleed, and 43% (n = 6) had presented with a history of seizures. 57% (n = 8) patients received a daily treatment for 3 days and 43% (n = 6) were on an alternate day (2 fractions) regimen. The marginal dose was split into 2 or 3 fractions of the ideal prescription dose of a single fraction of 23-25 Gy. The median follow up period was 35.6 months (8-57 months). In the three-fraction scheme, the marginal dose ranged from 8.9-11.5 Gy, while in the two-fraction scheme, the marginal dose ranged from 11.3-15 Gy at 50% per fraction. Headache (43%, n = 6) was the most common early postoperative complication, which was controlled with short course steroids. Follow up evaluation of at least three years was achieved in seven patients, who have shown complete nidus obliteration in 43% patients while the obliteration has been in the range of 50-99% in rest of the patients. Overall, there was a 67.8% reduction in the AVM volume at 3 years. Nidus obliteration at 3 years showed a significant rank order correlation with the cumulative prescription dose (p 0.95, P value 0.01), with attainment of near-total (more than 95%) obliteration rates beyond 29 Gy of the cumulative prescription dose. No patient receiving a cumulative prescription dose of less than 31 Gy had any severe adverse reaction. In co-variate adjusted ordinal regression, only the cumulative prescription dose had a significant correlation with common terminology criteria for adverse events (CTCAE) severity (P value 0.04), independent of age, AVM volume

  2. Yawning as a presenting symptom of Chiari malformation Type I: report of 2 cases.

    Science.gov (United States)

    Zebian, Bassel; Hogg, Florence Rosie Avila; Fu, Richard Zhiming; Sivakumaran, Ramanan; Stapleton, Simon

    2015-06-01

    Yawning is thought to be a behavior regulated by the brainstem. Although excessive yawning has been reported in brainstem strokes, demyelination, and tumors, the cases presented here are the first reports of excessive yawning in patients with Chiari malformation Type I (CM-I). The authors believe that brainstem compression at the craniocervical junction and ensuing edema were implicated in this curious symptomatology. They describe excessive yawning as a presenting feature of CM-I in 2 adolescent females. The presentation was acute in the first case and more chronic in the second. Both patients underwent foramen magnum decompression, which resulted in complete cessation of the excessive yawning.

  3. Sylvian fissure lipoma with angiomatous component and associated brain malformation: A case report.

    Directory of Open Access Journals (Sweden)

    Shruti Thakur

    2013-12-01

    Full Text Available Intracranial lipomas are congenital malformations. These uncommon lesions have an incidence of 0.1 to 1.7% of all intracranial tumors. Most cases are located at midline and 5% are along the sylvian fissures. If symptomatic, seizures are the most common symptom. These tumors are slow growing and have favorable outcome. We report a case of a 25-year-old man whose CT and MRI revealed a lesion in right sylvian fissure suggesting a lipoma with abnormal vasculature and overlying cortical dysplasia.

  4. 儿童Abernethy畸形%Comprehensive Review of Abernethy Malformation Cases in China

    Institute of Scientific and Technical Information of China (English)

    王亚妹; 周艳茹; 陶于洪

    2013-01-01

    Objective To acquire more knowledge about Abernethy malformation (congenital extrahepatic portosystemic shunt,including type Ⅰa,type Ⅰb and type Ⅱ) in children.Methods All children with Abernethy malformation published previously in China were reviewed.The clinical,radiological and pathological manifestations and treatment were summarized.Results From January 2001 to November 2012,13 children diagnosed to have Abemethy malformation were reported.Among them,11 were diagnosed as type Ⅰ Abernethy malformation,including 4 cases of type Ⅰa and 7 cases of type Ⅰb.However,only 2 children were diagnosed to be with type Ⅱ Abernethy malformation.Of the 11 type Ⅰcases,6 occurred by the age of one year old,and the duration of pre-diagnosis in 6 children was over 5 years.These 11 cases with type Ⅰ presented with hematochezia (n=3),hematemesis (n=4),liver dysfunction (n=4),liver cirrhosis (n=l),hepatic encephalopathy (n=2),hepatic nodule (n=3),splenomegaly (n=4),hypersplenism (n=3),portal hypertension (n=3) and other associated malformations (n=3).In the 2 type Ⅱ cases,one child presented with hematochezia,while another one had liver dysfunction accompanied with liver cirrhosis,splenomegaly and hypersplenism.No hematemesis or other associated malformations was found in children with type Ⅱ.The sites of drainage for portal vein system were the inferior vena cava (2/11),internal iliac vein (4/11),left renal vein (1/11),azygos vein (1/11),right atrium (2/11) and pelvic venous plexus (1/11),and there were no records of specific draining sites in two cases.The majority of these cases received conservative treatment.Surgical techniques such as splenectomy and surgical ligation of the veins of sigmoid were used to treat splenomegaly,hematochezia or hematemesis.But liver transplantation and resection of liver nodule were not reported.Conclusion Most Chinese children with Abemethy malformation are type Ⅰ Abernethy malformation and the onset is at early stage

  5. Combined hepatocellular-cholangiocarcinoma in a patient with Abernethy malformation and tetralogy of Fallot: A case report.

    Science.gov (United States)

    Happaerts, Sofie; Foucault, Amélie; Billiard, Jean Sébastien; Nguyen, Bich; Vandenbroucke-Menu, Franck

    2016-11-01

    Abernethy malformation is a rare congenital anomaly of the portal vein where the portal blood bypasses the liver. We report the first case of a patient with Abernethy malformation and tetralogy of Fallot associated with nodular regenerative hyperplasia and focal nodular hyperplasia (FNH), which finally evolved to a giant hepatocellular-cholangiocarcinoma (HCC-CC) of the liver, successfully resected. (Hepatology 2016;64:1800-1802). © 2016 by the American Association for the Study of Liver Diseases.

  6. Congestive myelopathy (Foix-Alajouanine Syndrome) due to intradural arteriovenous fistula of the filum terminale fed by anterior spinal artery: Case report and review of literature.

    Science.gov (United States)

    Krishnan, Prasad; Banerjee, Tapas Kumar; Saha, Manash

    2013-07-01

    Spinal arteriovenous fistulas are rare entities. They often present with congestive myelopathy but are infrequently diagnosed as the cause of the patients' symptoms. Only one such case has been described previously in Indian literature. We describe one such case who presented to us after a gap of 3 years since symptom onset and following a failed laminectomy where the cause was later diagnosed to be an intradural fistula in the filum terminale fed by the anterior spinal artery and review the available literature.

  7. Anesthesia management for pregnant patient with Arnold-Chiari malformation type I: A case report

    Directory of Open Access Journals (Sweden)

    Şaban Yalçın

    2012-06-01

    Full Text Available The Arnold-Chiari I malformation (ACM is a congenitalanomaly characterized by downward herniation of thecerebellar tonsils into the spinal canal. The anomaly maypresent in a variety of ways with vague symptoms suchas head and limb pains, vertigo, hypoesthesia, weaknessin the extremities. Diagnosis is often difficult and thereforedelayed. Measurement of intracranial pressure in thesepatients is an important measure thus precautions shoulbe taken for avoidance of increased intracranial pressure.In this case report, we discussed the anesthetic managementof a pregnant patient with Arnold Chiari I malformationwho underwent an emergency caesarean section.

  8. Aplasia cutis congenita associated with type I split cord malformation: Unusual case.

    Science.gov (United States)

    Abuzayed, Bashar; Erdincler, Pamir

    2014-01-01

    A full-term newborn girl born with large skin, muscle, bone and dural defect in the lumbo-sacral area. The lesion included a split spinal cord by a perpendicular bony spur and connected from its tip to the upper lamina. Patient was diagnosed with aplasia cutis congenita (ACC) associated with type I split cord malformation (SCM). Neurological examination of the lower extremities was normal. Spinal X-rays showed a bony spur on the L2 vertebral column and laminar defect in the lumbo-sacral area. Lesion was operated and closed according to anatomic layers. Clinical and intraoperative findings of this extremely rare case are discussed.

  9. 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.

  10. 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.

  11. 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....

  12. MURCS association and anorectal malformation: Case report of a female newborn

    Directory of Open Access Journals (Sweden)

    Sofia Morão

    2017-03-01

    Full Text Available MURCS association is rare, first described by Duncan et al. in 1979, including Müllerian duct aplasia, renal aplasia and cervicothoracic somite dysplasia. Levitt and Peña described in 2007 a classification of syndromic anorectal malformation (ARM that associates these two entities. The reported case is the first one described in neonatal period. We describe a case of a female newborn with suspected diagnosis of anorectal and renal malformations. Physical and radiologic investigation revealed agenesis of sacrum and coccyx, tethered cord, left multicystic renal dysplasia, absence of vaginal orifice and hymen, normally placed urethral orifice and abnormal anal opening at the vaginal introitus as a rectovestibular type fistula. Also, she had right uterine, tube and ovary agenesis with a normal 46, XX female karyotype. A left diversing colostomy was done in first day of life and four months later, was performed a posterior sagittal anorectoplasty (PSARP, with intra-operative identification of a duplication of the distal rectum (related with caudal regression syndrome type 2. There were no complications in postoperative period. A staged management strategy is a viable option avoiding further complications in an already poor prognosis situation.

  13. 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

  14. Improvement of Depression after Treatment of Dural Arteriovenous Fistula: A Case Report and a Review

    Directory of Open Access Journals (Sweden)

    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.

  15. Split cord malformation (scm in paediatric patients : outcome of 19 cases.

    Directory of Open Access Journals (Sweden)

    Kumar R

    2001-04-01

    Full Text Available There had been considerable debate regarding the surgical outcome of neuro-orthopaedic syndromes (NOS and neurological syndromes in cases of split cord malformation (SCM. On retrospective analysis of 19 cases of SCM, thirteen were grouped under (Pang type I and 6 in type II. Their age ranged from 1 month to 9 years (mean 3.5 years. 14 of these were male children. The NOS without neurological signs was detected in 6 cases where as pure neurological signs without NOS were seen in 8 patients. However, the rest 5 had mixed picture of NOS and neurological dysfunction. Nine of 19 cases presented with cutaneous stigmata, mainly in the form of hairy patch. 18 cases had other associated craniospinal anomalies i.e. hydrocephalus, meningomyelocoele, syrinx, dermoid, teratoma etc. Detethering of cord was done in all cases by removal of fibrous/bony septum. Associated anomalies were also treated accordingly. Follow up of these cases ranged from 6 months to 6 years. Six cases of NOS group neither showed deterioration nor improvement, and remained static on follow up. However, four of 8 children with neurological signs showed improvement in their motor weakness, and 1 in saddle hypoaesthesia as well as bladder/bowel function. In 5 cases of mixed group, two had improvement in their weakness and one in hypoaesthesia, but no change was noticed in NOS of this group as well. Hence surgery seemed to be effective, particularly in patients with neurological dysfunction.

  16. Middle and inner ear malformations in two siblings exposed to valproic acid during pregnancy: a case report.

    Science.gov (United States)

    Van Houtte, Evelyne; Casselman, Jan; Janssens, Sandra; De Kegel, Alexandra; Maes, Leen; Dhooge, Ingeborg

    2014-11-01

    Valproic acid (VPA) is a known teratogenic drug. Exposure to VPA during the pregnancy can lead to a distinct facial appearance, a cluster of major and minor anomalies and developmental delay. In this case report, two siblings with fetal valproate syndrome and a mild conductive hearing loss were investigated. Radiologic evaluation showed middle and inner ear malformations in both children. Audiologic, vestibular and motor examination was performed. This is the first case report to describe middle and inner ear malformations in children exposed to VPA.

  17. 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

    cases were satisfied with the diagnosis of venous sinus thrombosis while ignoring DAVF.Images in all cases were missed diagnosed.However, read carefully, subtle changes of vascular morphology suggesting arteriovenous malformation could still be found.Conclusions The lack of specificity of the clinical manifestations of DAVF, and clinician and radiologist with inadequate knowledge and narrow thinking about the disease, may lead to missed or inaccurate diagnosis.

  18. Pulmonary arteriovenous fistula

    Science.gov (United States)

    ... medlineplus.gov/ency/article/001090.htm Pulmonary arteriovenous fistula To use the sharing features on this page, please enable JavaScript. Pulmonary arteriovenous fistula is an abnormal connection between an artery and ...

  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

    Directory of Open Access Journals (Sweden)

    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. Characteristics, diagnosis and treatment of hypoglossal canal dural arteriovenous fistula: report of nine cases

    Energy Technology Data Exchange (ETDEWEB)

    Manabe, Shinji; Satoh, Koichi; Matsubara, Shunji; Satomi, Junichiro; Hanaoka, Mami; Nagahiro, Shinji [University of Tokushima, Department of Neurosurgery, Tokushima (Japan)

    2008-08-15

    We report the characteristics, diagnosis and treatment of dural arteriovenous fistula (DAVF) of the hypoglossal canal in nine patients with this relatively rare vascular disorder. Of 248 patients with intracranial DAVFs managed at our institution, nine patients (3.6%; four men, five women; mean age 62 years) were diagnosed with hypoglossal canal DAVF. We investigated patient characteristics with respect to clinical symptoms, neuroradiological findings, efficacy and complications related to endovascular treatment. Seven patients had experienced head injury. All patients presented with pulsatile tinnitus. One patient displayed ipsilateral hypoglossal nerve palsy before treatment. MR angiography showed a 'magic wand' appearance between the affected hypoglossal canal and the internal jugular vein in four patients. Angiography demonstrated an AV fistula on the medial aspect of the superior jugular bulb, mostly arising from the bilateral occipital, ascending pharyngeal and vertebral arteries with drainage to the internal jugular vein via the anterior condylar vein. Contralateral carotid injection accurately clarified the shunting point. Five patients underwent endovascular treatment: transarterial embolization (TAE; n=2), transvenous embolization (TVE; n=2), and TAE/TVE (n=1). Complete shunt obliteration was achieved in four patients and shunt reduction in one. The remaining four patients were treated conservatively and the shunt had disappeared at follow-up. Postoperative hypoglossal nerve palsy occurred in one patient after TVE, possibly due to coil overpacking. The incidence of hypoglossal canal DAVF was not very low in our series. Contralateral carotid injection is an essential examination to provide an accurate diagnosis. TVE should be considered when access is available, although TAE is also appropriate for shunt reduction. (orig.)

  1. 脑干血管畸形的显微手术治疗(附19例报告)%Microsurgery for brainstem vascular malformation:report of 19 cases

    Institute of Scientific and Technical Information of China (English)

    赵丛海; 高宇飞; 赵航; 于伟东; 李东原

    2011-01-01

    Objective To investigate the microsurgical treatment of brainstem vascular malformation and evaluate the surgical outcome. Method Brain stem vascular malformations in 19 cases were resected by microsurgical techniques. Six cases of cavernous malformations ( CM ) in the dorsal of pons and medulla underwent operations via through the base of the forth ventrical approach. Another nine cases of CM in lateral and ventral lateral pons were treated via suboccipito - retrosigmoid approach. Three cases of arteriovenous malformation(AVM) in cerebellomedullary fissure were treated via telovelar approach. One case of AVM in the dosal medulla were resected via transvermian approach. Results All the lesions were totally resected. Pathologic diagnosis were CM( 15 cases) and AVM(4 cases). The diameter of all the CM were less than 1 centimeter. One AVM was 1.5 centimeter in diameter, the other two lesions was 2. 0 centimeter, the last one was 2. 5 centimeter. The functional disorders were improved after operation in 13 cases. Complications appeared in five patients, which improved between one week and three months. One patient died of sever pneumonia. During two months to six years after operation, the brainstem vascular malformation didn't recrudesce and re - bleed. No one patient appeared new syndromes. Conclusions The microsurgical management of brainstem vascular malformation can effectively prevent re - bleeding. Selecting different surgical approach basing on the locations can reduce the neurofunction damage. In order to accelerate the recovery of damaged brainstem, early surgery should be choosen for the patients with surgical indications. Basing on the correct choice of surgical indications, using the extensive knowledge of microanatomy,new concept of minimal invasive surgery and skillful microsurgical techniques, the surgical results of brainstem vascular malformation are satisfactory.%目的 探讨脑干血管畸形的显微外科手术治疗方法和预后.方法 显微

  2. Split cord malformation – A study of 300 cases at AIIMS 1990– 2006

    Directory of Open Access Journals (Sweden)

    A K Mahapatra

    2011-01-01

    Full Text Available Background: Split cord malformation (SCM is a rare condition. With decreasing incidence of neural tube defect (NTD in the West, the reports of SCM are getting lesser and lesser. However, in India, spinal dysraphism is still a major problem encountered by the neurosurgeons. Objective: Our aim was to analyze 300 patients of SCM for their clinical features, radiological findings and outcome of surgery, which can throw light on the subject to others, who have less scope of finding these cases frequently. Materials and Methods: Over a 16-year period, we encountered 300 cases of SCM at AIIMS. Over the same period, more than 1500 cases of NTD were managed. SCM was noticed in 20% of cases with NTD. Skin stigmata were noted in two-third of the cases, and scoliosis and foot deformity were observed in 50% and 48% cases, respectively. Motor and sensory deficits were observed in 80% and 70% cases, respectively. Commonest site affected was lumbar or dorsolumbar (55% and 23%, respectively. In 3% cases, it was cervical in location. Magnetic resonance imaging (MRI scan revealed a large number of anomalies like lipoma, neuroenteric cyst, thick filum and dermoid or epidermoid cysts. All the patients were surgically treated. In type I, bony spurs were excised, and in type II, bands tethering the cord were released. Associated anomalies were managed in the same sitting. Patients were followed up from 3 months to 3 years. Results: Overall improvement was noticed in 50% and stabilization in 44% cases and deterioration of neurological status was recorded in 6% cases. However, 50% of those who deteriorated improved to preop status prior to discharge, 7-10 days following surgery. Conclusions: SCM is rare and not many large series are available. We operated 300 cases and noticed a large number of associated anomalies and also multilevel and multisite splits. Improvement or stabilization was noted in 94% and deterioration in 6% cases. We recommended prophylactic surgery

  3. A Case Report of Coronary Arteriovenous Fistulas with an Unruptured Coronary Artery Aneurysm Successfully Treated by Surgery

    Directory of Open Access Journals (Sweden)

    Nobuhiro Takeuchi

    2012-01-01

    Full Text Available A 58-year-old female with a history of Wolff-Parkinson-White syndrome presented at our institution with palpitations and chest pain. Electrocardiography revealed paroxysmal supraventricular tachycardia with a heart rate of 188 beats/min. Antiarrhythmic drugs were ineffective, and tachycardia was resolved by electrical cardioversion. Transthoracic echocardiography revealed abnormal vessels around the right coronary artery (RCA and pulmonary artery (PA; in addition, we suspected coronary arteriovenous fistula (CAVF. Coronary angiography and coronary computed tomography revealed dilated fistula vessels, with a 1 cm saccular aneurysm around the RCA, originating from the proximal RCA and left anterior descending artery into the main trunk of PA. Therefore, we confirmed the diagnosis of CAVF with an unruptured aneurysm. We surgically ligated and clipped the fistula vessels and resected the aneurysm. The resected aneurysm measured  cm in size. Pathological examination of the resected aneurysm revealed hypertrophic walls comprising proliferating fibroblasts cells thin elastic fibers. Very few atherosclerotic changes manifested in the aneurysm walls. We report the case of a patient with CAVF and an unruptured coronary artery aneurysm who was successfully treated by surgery.

  4. [Treatment of advanced periodontal and periapical lesion caused by malformed lingual groove with guided tissue regeneration: report of one case].

    Science.gov (United States)

    Yu, Yi-ming; Sun, Qing-Feng; Yang, Pi-shan

    2005-08-01

    This paper reported one case with severe periodontal lesions caused by malformed lingual groove treated by guided tissue regeneration. The periodontal lesion was exposed palatally after the tooth had been treated with root canal therapy, the alveolar bone and the root surface was prepared, an unabsorbable member of e-PTFE was placed into the wound,and removed after 4 weeks, the patient was followed up for 3 years. The lesion recovered well three years after the operation, all of the periodontal tissue was in a healthy condition. It is advisable that guided periodontal tissue regeneration can be used as a new method to treat periodontal destruction induced by malformed lingual groove.

  5. Anorectal malformations and pregnancy-related disorders : a registry-based case-control study in 17 European regions

    NARCIS (Netherlands)

    Wijers, C. H. W.; van Rooij, I. A. L. M.; Bakker, M. K.; Marcelis, C. L. M.; Addor, M. C.; Barisic, I.; Beres, J.; Bianca, S.; Bianchi, F.; Calzolari, E.; Greenlees, R.; Lelong, N.; Latos-Bielenska, A.; Dias, C. M.; McDonnell, R.; Mullaney, C.; Nelen, V.; O'Mahony, M.; Queisser-Luft, A.; Rankin, J.; Zymak-Zakutnia, N.; de Blaauw, I.; Roeleveld, N.; de Walle, H. E. K.

    2013-01-01

    Objective To identify pregnancy-related risk factors for different manifestations of congenital anorectal malformations (ARMs). Design A population-based case-control study. Setting Seventeen EUROCAT (European Surveillance of Congenital Anomalies) registries, 1980-2008. Population The study populati

  6. Anorectal malformations and pregnancy-related disorders: a registry-based case-control study in 17 European regions

    NARCIS (Netherlands)

    Wijers, C.H.W.; Rooij, I.A.L.M. van; Bakker, M.K.; Marcelis, C.L.M.; Addor, M.C.; Barisic, I.; Beres, J.; Bianca, S.; Bianchi, F.; Calzolari, E.; Greenlees, R.; Lelong, N.; Latos-Bielenska, A.; Dias, C.M.; McDonnell, R.; Mullaney, C.; Nelen, V.; O'Mahony, M.; Queisser-Luft, A.; Rankin, J.; Zymak-Zakutnia, N.; Blaauw, I. de; Roeleveld, N.; Walle, H.E. de

    2013-01-01

    OBJECTIVE: To identify pregnancy-related risk factors for different manifestations of congenital anorectal malformations (ARMs). DESIGN: A population-based case-control study. SETTING: Seventeen EUROCAT (European Surveillance of Congenital Anomalies) registries, 1980-2008. POPULATION: The study popu

  7. 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...

  8. Congestive myelopathy (Foix-Alajouanine Syndrome due to intradural arteriovenous fistula of the filum terminale fed by anterior spinal artery: Case report and review of literature

    Directory of Open Access Journals (Sweden)

    Prasad Krishnan

    2013-01-01

    Full Text Available Spinal arteriovenous fistulas are rare entities. They often present with congestive myelopathy but are infrequently diagnosed as the cause of the patients′ symptoms. Only one such case has been described previously in Indian literature. We describe one such case who presented to us after a gap of 3 years since symptom onset and following a failed laminectomy where the cause was later diagnosed to be an intradural fistula in the filum terminale fed by the anterior spinal artery and review the available literature.

  9. Scala tympani drill-out technique for oval window atresia with malformed facial nerve:A report of three cases

    Institute of Scientific and Technical Information of China (English)

    Yang Liu a; Feng Yang b

    2015-01-01

    Objective:To report a scala tympani drill-out technique for managing malformed facial nerve covering the entire oval window (OW). Methods:Data from three cases with OW atresia, malformed stapes and abnormal facial nerve courses were reported, in which a scala tympani drill-out technique was employed with a TORP between the tympanic membrane and scala tympani fenestration for hearing reconstruction. Results: Air conduction hearing improved in two of the three cases following surgery. In the third case, there was no improvement in air conduction hearing following a canal wall up mastoidectomy and tympanoplasty. There were no vertigo, tinnitus or sensorineural hearing loss in the three cases. Conclusion:The scala tympani drill-out technique, which is basically fenestration at the initial part of the basal turn, provides a choice in hearing reconstruction when the OW is completely covered by abarrently coursed facial nerve.

  10. Silent neurenteric cyst with split cord malformation at conus medullaris: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Hanuma Srinivas

    2014-01-01

    Full Text Available Split cord malformations (SCM are a common pediatric abnormality where children present with features of tethering and backache along with varying neurological deficits. Multiple neural tube defects may co-exist in children having defects of primary and/or secondary neurulation. Co-existent neurenteric cysts along with type 1 SCM have been described very rarely in the literature. We report a case of silent neurenteric cyst at conus medullaris with SCM type 1 where the cyst was missed in the preoperative imaging. Until date, only 8 such cases of neurenteric cysts with SCM at lumbar region have been reported. We review the literature regarding co-existing dual pathologies of neurenteric cysts and type 1 SCMs in light of limited capacity of imaging modalities to detect small neurenteric cysts in presence of co-existent neural tube defects. Multiple spinal neural tube defects in children need more attention and precise microneurosurgical skills as management differs in each of them. Co-existence of such pathologies detected intra-operatively may need modifications in preoperative planning to achieve the best possible outcomes.

  11. Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Roya Memarpour

    2015-01-01

    Full Text Available Neuroarthropathy (neuropathic osteoarthropathy, also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis. We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

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

    Science.gov (United States)

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

    2016-04-01

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

  13. Paraplegia in a chiropractic patient secondary to atraumatic dural arteriovenous fistula with perimedullary hypertension: case report

    National Research Council Canada - National Science Library

    Foreman, Stephen M; Stahl, Michael J; Schultz, Gary D

    2013-01-01

    .... This abnormal communication can result in important and frequently misdiagnosed neurological abnormalities.A case of rapid onset paraplegia following cervical chiropractic manipulation is reviewed...

  14. Seizure and unilateral facial nerve paralysis in a newborn with Dandy-Walker malformation – A case report

    Directory of Open Access Journals (Sweden)

    Subhajit Bhakta

    2014-01-01

    Full Text Available The Dandy-Walker syndrome (DWS is a rare posterior fossa malformation. It can have a varied presentation depending on the age. A newborn presenting with neonatal seizure along with unilateral facial nerve palsy is rather a rare presentation of DWS and very few such cases were reported in the past. We are reporting a case of a newborn male baby presenting with neonatal seizure within 48 hrs of birth along with right sided LMN type facial nerve palsy which on due course of investigation revealed as a case of Dandy–Walker malformation. Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-1, 48-51 DOI: http://dx.doi.org/10.3126/jcmsn.v9i1.9673

  15. Successful treatment of two cases of urorectal septum malformation sequence with oligohydramnios and severe respiratory failure

    Directory of Open Access Journals (Sweden)

    Sekiguchi K

    2013-03-01

    Full Text Available Kazuhito Sekiguchi, Daijiro Takahashi, Takehiko Hiroma, Tomohiko Nakamura Division of Neonatology, Nagano Children's Hospital, Nagano, Japan Abstract: Urorectal septum malformation sequence can be diagnosed prenatally. We report herein the successful treatment of severe respiratory failure in two patients with urorectal septum malformation sequence and prenatally diagnosed oligohydramnios. The patients showed significant hypoxic deterioration and persistent pulmonary artery hypertension soon after birth. We used high-frequency ventilation (HFO, nitric oxide (NO, and extracorporeal membrane oxygenation (ECMO. The patients were successfully weaned from ECMO and artificial ventilation and discharged to home. Although termination of the pregnancy has often been selected for fetuses with oligohydramnios-related urorectal septum malformation sequence, our results suggest the opportunity for these fetuses to be treated using respiratory supports including HFO, NO, and ECMO after birth. Keywords: urorectal septum malformation sequence, oligohydramnios, pulmonary hypoplasia

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

    OpenAIRE

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

    2016-01-01

    Intracranial lipomas are rare congenital malformations. The most common type of intracranial lipoma is the interhemispheric lipoma, which is frequently associated with callosal anomalies such as hypogenesis or agenesis of the corpus callosum. In contrast, interhemispheric lipomas are less often accompanied with malformations of cortical development (MCD). We report magnetic resonance imaging findings of three infants with an interhemispheric lipoma, associated with a callosal anomaly, and MCD...

  17. Middle and inner ear malformations in two cases of velocardiofacial syndrome

    OpenAIRE

    Tabith Junior,Alfredo; Haetinger, Rainer Guilherme; Silva,Fernando Leite de Carvalho e; Gudmon, Monica de Castro

    2009-01-01

    Objective: To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Method: Audiometric evaluation, computerized tomography of the temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome. Results: Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the latter with the use of ...

  18. 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.

  19. A retrospective review of cases preoperatively diagnosed by radiologic imaging as cavernous venous malformations.

    Science.gov (United States)

    Jayaram, Anupam; Cohen, Liza M; Lissner, Gary S; Karagianis, Achilles G

    2017-04-03

    The purpose of this study is to examine orbital lesions identified on preoperative radiologic imaging as cavernous venous malformations (CVMs), identify their imaging characteristics, and determine if these may help differentiate CVMs from other intraorbital masses. An IRB-approved retrospective chart review over 30 years was undertaken identifying lesions "consistent with cavernous hemangioma" on radiologic studies, which were subsequently surgically resected with a tissue diagnosis. All radiologic images (CT and MRI) obtained preoperatively were re-reviewed by a single masked neuroradiologist. The pattern of contrast enhancement on sequential MRI views was used to determine whether the enhancing characteristics helped identify CVMs compared to other intraorbital masses. Fifty-seven orbital lesions consistent with a CVM were identified on imaging. Fourteen (25%) of them were resected, of which nine (64%) were found to be CVMs on pathologic examination. Five (36%) were found to be a different lesion, most commonly schwannoma (21%). On imaging, CVMs tended to display heterogeneous progressive enhancement, whereas other tumors, in particular schwannomas, enhanced at their maximum level immediately. Based on these characteristics, on re-review, the masked neuroradiologist was able to differentiate a CVM versus other tumors for all 14 imaging cases. This study suggests that examining the pattern of contrast enhancement may help to correctly differentiate a CVM from other isolated, encapsulated orbital lesions on CT/MR imaging.

  20. 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.

  1. 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.

  2. 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

  3. Potential of transcranial power Doppler imaging in the diagnosis of cerebral arteriovenous malformation%经颅能量多普勒血流显像在脑动静脉畸形诊断中的价值

    Institute of Scientific and Technical Information of China (English)

    庄磊; 段云友; 曹铁生; 阮骊韬

    2000-01-01

    Objective To evaluate potential of transcranial power Doppler imaging(PDI)in the diagnosis of cerebral artefiovenous malformation(AVM).Methods Compared with the results of cerebral an~ography.Twenty-five cases(16 moles,9 females),of cerebral AVM were involved.Transcranial PDI and color Doppler flow imaging(CDFI) examination were performed through skull acoustic windows. Hemodynamics parameters such as Vmax,Vmin and RI were measured in abnormal vessels.Results Both transcranial PDI and CDFI detected 23 cases of AVM,the size and location of lesions were well correlated to the results obtained by cerebral angiography.The number of AVM-feedmg arteries,-main feeding arteries and-draining veins counted by PDl were 42,29,10,respectively,and those by CDFI were 37,28,6,respectively.Conclusions There ware no much difference in the diagnostic accuracy of AVM between transcranial PDI and CDFI.Transcranial PDI shows more advantage than CDFI in displaying atypical AVM-feeding arteries and draining veins.%目的 探讨经颅能量多普勒血流显像(PDI)在脑动静脉畸形(AVM)诊断中的应用价值.方法 对临床诊断为脑动静脉畸形患者25例,分别采用经颅能量多普勒和彩色多普勒血流显像(CDFI)进行探测,测量病变血管的Vmax、Vmin及RI,并与脑血管造影结果进行对比分析.结果 CDFI诊断23例脑AVM,检出供血动脉37支(80.4%),主要供血动脉28支(90.3%),引流静脉6支(30.O%);PDI诊断23例AVM,检出供血动42支(91.3%),主要供血动脉29支(93.5%),引流静脉10支(50.0%).PDI和CDFI对AVM大小及位置的判断与脑血管造影结果基本一致(P>0.05).结论 经颅PDI对脑AVM诊断率与CDFI基本一致,但在显示非主要供血动脉和引流静脉方面优于CDFI,可为临床诊断脑AVM提供更丰富的血流动力学资料.

  4. Color Doppler Ultrasound Study of Glomuvenous Malformations with its Clinical and Histologic Correlations.

    Science.gov (United States)

    Wortsman, X; Millard, F; Aranibar, L

    2017-07-03

    Glomuvenous malformations are hamartomatous lesions characterized by the presence of glomus cells in the vascular smooth muscle. We present the clinical and color Doppler ultrasound features of a series of 13 cases of histologically confirmed glomuvenous malformations. In all cases, the ultrasound study revealed moderately delimited superficial dermal and hypodermal pseudonodular structures of mixed echogenicity, with hypoechoic and heterogeneous areas and anechoic, pseudocystic tubular and lacunar zones. Arterial and venous vessels, mainly with a low flow (≤ 15cm/s) were observed in 85% of patients, but no arteriovenous shunts were present. Deeper structures were not affected and no phleboliths were detected. The clinical and ultrasound findings could facilitate diagnosis, surgical planning, and noninvasive follow-up in these tumors. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Transvenous Coil Embolization for Dural Arteriovenous Fistulas of the Ophthalmic Sheath: Report of Two Cases and Review of the Literature

    Science.gov (United States)

    Hwang, Juyoung; Jo, Kyung-Il; Yeon, Je Young; Hong, Seung-Chyul

    2016-01-01

    We present two patients with a dural arteriovenous fistula (dAVF) of the ophthalmic sheath who developed progressive exophthalmos, conjunctival chemosis, and visual loss. These symptoms mimic those of cavernous sinus dAVFs. Dural AVFs of the ophthalmic sheath are extremely rare and their clinical management is controversial. We successfully treated these two patients by transvenous coil embolization. Transvenous embolization appears to be a safe and effective method to treat dAVFs of the ophthalmic sheath.

  6. 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.

  7. Automated double-cone-beam CT fusion technique. Enhanced evaluation of glue distribution in cases of spinal dural arteriovenous fistula (SDAVF) embolisation

    Energy Technology Data Exchange (ETDEWEB)

    Farago, Giuseppe [Foundation Neurological Institute ' ' C. Besta' ' , Department of Interventional Neuroradiology, Milan (Italy); Fondazione IRCCS Istituto Neurologico Carlo Besta, Department of Interventional Neuroradiology, Milan (Italy); Caldiera, V. [Foundation Neurological Institute ' ' C. Besta' ' , Department of Interventional Neuroradiology, Milan (Italy); Antozzi, C.; Bellino, A. [Foundation Neurological Institute ' ' C. Besta' ' , Department of Neuroimmunology and Neuromuscular Diseases, Milan (Italy); Innocenti, A. [Foundation Neurological Institute ' ' C. Besta' ' , Department of Neuro-Oncology, Milan (Italy); Ciceri, E. [Foundation Neurological Institute ' ' C. Besta' ' , Department of Interventional Neuroradiology, Milan (Italy); Azienda Ospedaliera Universitaria Integrata Borgo Trento, Department of Neuroradiology, Verona (Italy)

    2017-05-15

    Spinal dural arteriovenous fistulas (SDAVFs) are acquired diseases that represent the majority of all arteriovenous spinal shunts, leading to progressive and disabling myelopathy. Treatment is focused on accurately disconnecting the fistula point. We present our experience with the double-cone-beam CT fusion technique successfully applied to evaluate treatment results in a series of SDAVFs. Between November 2011 and December 2015 we performed double-DynaCT acquisition (pre- and post-embolisation) in 12 cases of SDAVF. A successful DynaCT fusion technique was only achieved in the group of patients with pre- and post-treatment images acquired at the same time as the treatment session, under general anaesthesia (4/12). DynaCT performed on different days proved to be inadequate for the automated fusion technique because of changes in the body position (8/12). A pre-treatment flat-panel cone-beam CT with contrast, at the time of diagnostic angiography, can be very helpful to detect the correct level of the fistula and the relationship between the fistula and the surrounding structures. In case of the endovascular approach, additional post-treatment native acquisition merged with the pre-treatment acquisition (double-cone-beam CT fusion technique) permits to immediately evaluate the distribution of the glue cast and to confirm the success of the procedure. (orig.)

  8. Intracranial dural arteriovenous fistula as a cause for symptomatic superficial siderosis: A report of two cases and review of the literature

    Directory of Open Access Journals (Sweden)

    Griffin R Baum

    2016-01-01

    Conclusions: We describe the second and third cases of a dAVF as the cause of SS, and the first cases of successful treatment of SS-associated dAVF with endovascular embolization. As noninvasive imaging techniques become more sensitive and easily obtained, one must consider their limitations in detecting occult intracranial vascular malformations such as dAVF as a possible etiology for SS.

  9. Delayed-onset heparin-induced thrombocytopenia presenting with multiple arteriovenous thromboses: case report

    Directory of Open Access Journals (Sweden)

    Omran Abbas

    2007-11-01

    Full Text Available Abstract Background Delayed-onset heparin-induced thrombocytopenia with thrombosis, albeit rare, is a severe side effect of heparin exposure. It can occur within one month after coronary artery bypass grafting (CABG with manifestation of different thrombotic events. Case presentation A 59-year-old man presented with weakness, malaise, bilateral lower limb pitting edema and a suspected diagnosis of deep vein thrombosis 18 days after CABG. Heparin infusion was administered as an anticoagulant. Clinical and paraclinical work-up revealed multiple thrombotic events (stroke, renal failure, deep vein thrombosis, large clots in heart chambers and 48 ×103/μl platelet count, whereupon heparin-induced thrombocytopenia was suspected. Heparin was discontinued immediately and an alternative anticoagulant agent was administered, as a result of which platelet count recovered. Heparin-induced thrombocytopenia, which causes thrombosis, is a serious side effect of heparin therapy. It is worthy of note that no case of delayed-onset heparin-induced thrombocytopenia with thrombosis associated with cardiopulmonary bypass surgery has thus far been reported in Iran. Conclusion Delayed-onset heparin-induced thrombocytopenia should be suspected in any patient presenting with arterial or venous thromboembolic disorders after recent heparin therapy, even though the heparin exposure dates back to more than a week prior to presentation; and it should be ruled-out before the initiation of heparin therapy.

  10. Cardiac Arrest Secondary to Bilateral Pulmonary Emboli following Arteriovenous Fistula Thrombectomy: A Case Report with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Avni Shah

    2012-01-01

    Full Text Available Number of patients with End Stage Renal Disease (ESRD is growing worldwide. Hemodialysis remains the main modality of renal replacement therapy for ESRD patients. A patent hemodialysis access (arteriovenous fistula or arteriovenous graft plays a key role in successful delivery of hemodialysis. Common vascular access issues encountered by patients and nephrologists are thrombosis and infection. The thrombosed access is declotted by various percutaneous techniques these days by multiple outpatient access centers in a timely fashion. Thrombolysis can give rise to various complications, a few of which can be life threatening. A young hemodialysis patient underwent percutaneous thrombolysis of his clotted arteriovenous fistula. Outpatient access thrombectomy was complicated immediately afterwards with cardiac arrest requiring cardiac resuscitation in the recovery room. The patient was admitted to intensive care unit after life sustaining care. Work up revealed multiple pulmonary emboli to both lung fields on CT scan of the chest. Patient was anticoagulated and discharged from the hospital. Thrombolysis of clotted hemodialysis access is associated commonly with occurrences of pulmonary embolic which are usually asymptomatic. Massive pulmonary embolization due to access thrombolysis is rare. Nephrologists and radiologists should be aware of this dangerous complication particularly in patients with preexisting cardiopulmonary disease.

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

    Science.gov (United States)

    Deckner, C; Schiesser, M; Bastert, G

    2004-04-01

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

  12. Case Report: Microsurgical excision of grade 5 cerebral AVM [version 2; referees: 1 approved, 2 approved with reservations

    Directory of Open Access Journals (Sweden)

    Sunil Munakomi

    2016-07-01

    Full Text Available In this case report, we discuss the microsurgical management of a Spetzler-Martin grade 5 arteriovenous malformation (AVM in a young boy who presented with a hemorrhagic episode and had a high calculated risk of rebleeding. We also outline the rationale for choosing the management option.

  13. A case report of aphallia with urorectal septum malformation sequence in a newborn: a very rarely seen condition

    Directory of Open Access Journals (Sweden)

    Sharma D

    2015-12-01

    Full Text Available Deepak Sharma,1 Ravinder Singh,2 Sweta Shastri3 1Department of Neonatology, Fernandez Hospital, Hyderabad, 2Department of Pediatrics, Civil Hospital, Hisar, Haryana, 3Department of Pathology, NKP Salve Medical College, Nagpur, Maharashtra, India Abstract: Aphallia (absence of penis is an extremely rare abnormality which has rarely been described in medical literature and can be part of the urorectal septum malformation sequence (URSMS. URSMS has hardly been reported in medical literature and includes the absence of perineal and anal openings in association with ambiguous genitalia and urogenital, colonic, and lumbosacral anomalies. This case report tells the importance of detailed examination of infants that are diagnosed with aphallia. We report a case of a newborn who was diagnosed as aphallia with the URSMS syndrome after birth. The neonate had an endocardial cushion defect (atrial septal defect and ventricular septal defect and bilateral agenesis of the kidney. The neonate succumbed to death secondary to hypoplastic lung leading to respiratory failure. Keywords: aphallia, urorectal septum malformation sequence, cloacal membrane, hindgut development, congenital aphallia, anorectal malformation

  14. 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.

  15. Chiari malformation type I: a case-control association study of 58 developmental genes.

    Directory of Open Access Journals (Sweden)

    Aintzane Urbizu

    Full Text Available Chiari malformation type I (CMI is a disorder characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa (PCF, often causing progressive neurological symptoms. The etiology of CMI remains unclear and is most likely multifactorial. A putative genetic contribution to CMI is suggested by familial aggregation and twin studies. Experimental models and human morphometric studies have suggested an underlying paraxial mesoderm insufficiency. We performed a case-control association study of 303 tag single nucleotide polymorphisms (SNP across 58 candidate genes involved in early paraxial mesoderm development in a sample of 415 CMI patients and 524 sex-matched controls. A subgroup of patients diagnosed with classical, small-PCF CMI by means of MRI-based PCF morphometry (n = 186, underwent additional analysis. The genes selected are involved in signalling gradients occurring during segmental patterning of the occipital somites (FGF8, Wnt, and retinoic acid pathways and from bone morphogenetic proteins or BMP, Notch, Cdx and Hox pathways or in placental angiogenesis, sclerotome development or CMI-associated syndromes. Single-marker analysis identified nominal associations with 18 SNPs in 14 genes (CDX1, FLT1, RARG, NKD2, MSGN1, RBPJ1, FGFR1, RDH10, NOG, RARA, LFNG, KDR, ALDH1A2, BMPR1A considering the whole CMI sample. None of these overcame corrections for multiple comparisons, in contrast with four SNPs in CDX1, FLT1 and ALDH1A2 in the classical CMI group. Multiple marker analysis identified a risk haplotype for classical CMI in ALDH1A2 and CDX1. Furthermore, we analyzed the possible contributions of the most significantly associated SNPs to different PCF morphometric traits. These findings suggest that common variants in genes involved in somitogenesis and fetal vascular development may confer susceptibility to CMI.

  16. The Impact of Different Case Ascertainment Definitions on the Prevalence of Major Congenital Malformations and their Association with Asthma During Pregnancy.

    Science.gov (United States)

    Eltonsy, Sherif; Forget, Amelie; Blais, Lucie

    2017-03-01

    Objectives To compare the prevalence of major malformations using different case ascertainment definitions and to evaluate their impact on maternal asthma-major malformations association. Methods A cohort of pregnancies with and without asthma between 1990 and 2010 was formed. We used two classification methods: the Two step Congenital Malformation Classification (TCMC) and the Canadian Congenital Anomalies Surveillance System (CCASS). Within each method, three case definitions were compared: (1) ≥1 diagnosis in the hospital database; (2) ≥1 diagnosis in the hospital database or ≥2 in the medical claims; and (3) ≥1 diagnosis in the hospital database or ≥1 in the medical claims. We calculated the prevalence of major malformations and adjusted odds ratios (aORs) for maternal asthma association. Results Of 467,946 pregnancies, 12.3 % were with active asthma. The prevalence estimates were: TCMC 5.10-7.08 % and CCASS 7.03-10.57 %. Asthma-major malformations association was weaker with the CCASS (aOR 1.14-1.20) versus TCMC (aOR 1.22-1.26). Discussion The case ascertainment definitions with ≥1 hospitalization are likely to be the most reliable in similar administrative databases. The case ascertainment definition had a considerable impact on the prevalence of major malformations, but hardly influenced the aORs. Future studies should formally assess the validity of the case ascertainment definitions and allow generalizability to other maternal exposures.

  17. 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...

  18. 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

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

    NARCIS (Netherlands)

    Niwa, Tetsu; de Vries, Linda S.; Manten, GTR; Lequin, Maarten; Cuppen, Inge; Shibasaki, Jun; Aida, Noriko

    2016-01-01

    Intracranial lipomas are rare congenital malformations. The most common type of intracranial lipoma is the interhemispheric lipoma, which is frequently associated with callosal anomalies such as hypogenesis or agenesis of the corpus callosum. In contrast, interhemispheric lipomas are less often acco

  2. Endoscopic approaches to brainstem cavernous malformations: Case series and review of the literature

    Directory of Open Access Journals (Sweden)

    Nikhil R Nayak

    2015-01-01

    Conclusion: The endoscope is a promising adjunct to the neurosurgeon′s ability to approach difficult to access brainstem cavernous malformations. It allows the surgeon to achieve well-illuminated, panoramic views, and by combining approaches, can provide minimally invasive access to most regions of the brainstem.

  3. Dyssynergic defecation may aggravate constipation : results of mostly pediatric cases with congenital anorectal malformation

    NARCIS (Netherlands)

    van Meegdenburg, Maxime M.; Heineman, Erik; Broens, Paul M. A.

    2015-01-01

    BACKGROUND: Most patients with congenital anorectal malformation suffer from mild chronic constipation. To date, it is unclear why a subgroup of patients develops a persistent form of constipation. Because dyssynergic defecation is a common cause of constipation in the general population, we hypothe

  4. 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.

  5. Middle and inner ear malformations in two cases of velocardiofacial syndrome

    Directory of Open Access Journals (Sweden)

    Tabith Junior, Alfredo

    2009-03-01

    Full Text Available Objective: To describe audiometric characteristics and middle and inner ear malformations in two patients with velocardiofacial syndrome. Method: Audiometric evaluation, computerized tomography of the temporal bones and analysis of DNA for multiple markers of 22q11 region were performed in two patients with clinical signs of velocardiofacial syndrome. Results: Conductive hearing loss related to chronic otites media and middle and inner ear malformations were found, the latter with the use of reformations based on multislice acquisitions on of the computerized tomography of the temporal bones. Conclusion: We consider it to be highly important to carry out a thorough evaluation and monitoring of the hearing evolution, as well as the occurrence of symptoms related to the vestibular function in patients with velocardiofacial syndrome. From the radiological point of view, attention should be given to the use of high quality techniques for the tomographic study of temporal bones.

  6. Myelomeningocele associated with split cord malformation type I -three case reports-.

    Science.gov (United States)

    Higashida, Tetsuhiro; Sasano, Mari; Sato, Hironobu; Sekido, Ken'ichi; Ito, Susumu

    2010-01-01

    Three neonates presented with split cord malformation (SCM) associated with myelomeningocele (MMC), complicated with various coexisting anomalies. All patients were female and classified as SCM type I. All patients had a syrinx located rostral to the SCM. One patient had hydrocephalus and Chiari malformation causing serious respiratory problems. Two patients had partial hypertrichosis located close to the MMC, suggesting association with SCM. One patient had sacral hypoplasty and right kidney agenesis, suggesting that some embryologic errors may affect not only neural but also mesodermal development. All patients underwent surgical treatment for SCM after detailed evaluation and management of concomitant anomalies, and developed no new neurological deficits. Delayed surgery is an alternative treatment strategy for SCM in patients with both SCM and MMC with similar complications.

  7. 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, ...

  8. 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.

  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. A extremely rare case of cervical intramedullary granuloma due to Brucella accompanied by Chiari Type-1 malformation.

    Science.gov (United States)

    Tufan, Kadir; Aydemir, Fatih; Sarica, Feyzi Birol; Kursun, Ebru; Kardes, Özgür; Cekinmez, Melih; Caner, Hakan

    2014-01-01

    Chiari Type-1 malformation is displacement of the cerebellar tonsils through the foramen magnum into the cervical spine and usually does not exceed the level of C2. It is 50-70% associated with syringomyelia. Nervous system involvement due to brucellosis is called neurobrucellosis, and neurological involvement rate has been reported an average of 3-5%, ranging between 3% and 25% at different series. Intramedullary abscess or granuloma due to Brucella is extremely rare. Hence far, six cases have been reported in the literature and only two of these cases were reported as intramedullary granuloma. This case is presented in order to remind the importance of the cervical cord granuloma which was presented once before in the literature and to emphasize the importance of evaluation of patient history, clinical and radiological findings together in the evaluation of a patient.

  11. Aplasia cutis congenita associated with type I split cord malformation: Unusual case

    OpenAIRE

    Bashar Abuzayed; Pamir Erdincler

    2014-01-01

    A full-term newborn girl born with large skin, muscle, bone and dural defect in the lumbo-sacral area. The lesion included a split spinal cord by a perpendicular bony spur and connected from its tip to the upper lamina. Patient was diagnosed with aplasia cutis congenita (ACC) associated with type I split cord malformation (SCM). Neurological examination of the lower extremities was normal. Spinal X-rays showed a bony spur on the L2 vertebral column and laminar defect in the lumbo-sacral area....

  12. 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

  13. 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.)

  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. 婴儿Chiari畸形II型1例报告%Chiari II malformation in infant:a case report

    Institute of Scientific and Technical Information of China (English)

    胡凤娥

    2014-01-01

    目的:分析婴儿Chiari畸形Ⅱ型的临床资料,加深儿科医师的认识。方法回顾性分析1例确诊为Chiari畸形Ⅱ型患儿的临床、实验室、影像学资料,并复习相关文献。结果患儿,男,2个月,因颈部肿物2个月入院。颅脑MRI示小脑扁桃体部分疝入枕骨大孔。予手术治疗后一直间断发热,不能竖头、独坐,前囟门进行性增大,CT诊断脑积水。结论 Chi-ari畸形Ⅱ型会导致神经功能异常,预后差,病死率高;早期诊断,积极手术,可改善预后。%Objective To analyze the clinical data of an infant with Chiari II malformation so as to deepen understanding of this disease in pediatrics. Methods The clinical, laboratory and imaging data of an infant with Chiari II malformation were retrospectively analyzed and a literature review was performed. Results A 2-month-old male infant was admitted to our hospital due to a neck tumor for two months. Brain magnetic resonance imaging (MRI) showed herniation of the cerebellar tonsils into the foramen magnum. The infant was finally diagnosed with Chiari II malformation based on the result of MRI and the presence of myelomeningocele. After repair of spinal cord and meninges and decompression of pillow macroporous by surgery, the infant presented with intermittent fever and progressively enlarged anterior fontanel, with culture-positive cerebrospinal fluid for bacte-ria and hydrocephalus diagnosed by CT, and intracranial infection was considered. Ventriculoperitoneal shunt was not performed due to uncontrolled intracranial infection after treatment, and motor retardation was found during the follow-up. Conclusions Chiari II malformation may results in nervous system dysfunction with poor diagnosis and high case fatality rate. Early diagnosis and operation may be helpful to the improvement of prognosis.

  16. 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.

  17. Transcatheter embolization of a congenital intrahepatic arterioportal venous malformation: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Sing, T.M.Y.S.; Wong, K.P.; Young, N. [Westmead Hospital, Westmead, NSW, (Australia). Department of Radiaology; Le, S.D.V. [Bankstown-Lidcombe Hospital, Bankstown, NSW, (Australia). Department of Nuclear Medicine and Ultrasound

    1997-08-01

    Congenital intrahepatic arterioportal venous malformations (APVM) are uncommon lesions. A congenital intrahepatic APVM found incidentally in a 51 -year-old man during pre-operative aortography for an abdominal aortic aneurysm is reported here. This was successfully treated by transcatheter embolization of the involved hepatic artery prior to surgical repair of the aortic aneurysm. A 51-year-old smoker was admitted for pre-operative aortography of an abdominal aortic aneurysm (AAA). Liver function tests showed a mildly elevated alkaline phosphatase. There was no previous history of liver disease or trauma. Aortography demonstrated a large infra-renal AAA measuring 10 cm in diameter and 20 cm in length. The coeliac axis was noted to be grossly dilated with tortuous veins seen to the right side of the lower thoracic spine on delayed images. Coeliac angiography revealed a dilated intrahepatic vascular abnormality in the left lobe of the liver with late opacification of the portal vein. Contrast abdominal CT demonstrated the AAA and the dilated coeliac axis feeding a large vascular malformation in the lateral aspect of the left lobe of the liver. The arterial inflow was via the left hepatic artery and a large vein was seen leading into the left portal vein. Endoscopy showed no oesophageal varices. (authors). 11 refs., 7 figs.

  18. 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.)

  19. 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. Copyright © 2012 Elsevier Ltd. All rights reserved.

  20. A three-year-old boy with X-linked adrenoleukodystrophy and congenital pulmonary adenomatoid malformation: a case report

    Directory of Open Access Journals (Sweden)

    Cakan Nedim

    2009-12-01

    Full Text Available Abstract Introduction X-linked adrenoleukodystrophy leads to demyelination of the nervous system, adrenal insufficiency, and accumulation of long-chain fatty acids. Most young patients with X-linked adrenoleukodystrophy develop seizures and progressive neurologic deficits, and die within the first two decades of life. Congenital or acquired disorders of the respiratory system have not been previously described in patients with X-linked adrenoleukodystrophy. Case presentation A 3-year-old Arabic boy from Yemen presented with discoloration of the mucous membranes and nail beds, which were considered cyanoses due to methemoglobinemia. He also had shortness of breath, fatigue, emesis and dehydration episodes for which he was admitted to our hospital. Chest radiograph and chest computed tomography scans showed congenital pulmonary adenomatoid malformation. A few weeks before the removal of the malformation, he had a significant episode of hypotension and hypoglycemia. This development required further in-hospital evaluation that led to the diagnosis of adrenal insufficiency and the initiation of treatment with corticosteroids. One year later, he developed seizures and loss of consciousness. Magnetic resonance imaging of his head showed diffuse demyelination secondary to X-linked adrenoleukodystrophy. He was treated with anti-seizure and anti-oxidants, and was referred for bone marrow transplant evaluation. Conclusion The presence of adrenal insufficiency, neurologic deficits and seizures are common manifestations of X-linked adrenoleukodystrophy. The association of congenital lung disease with X-linked adrenoleukodystrophy or Addison's disease has not been described previously.

  1. 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

  2. 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

  3. Wide variation in anal sphincter muscles in cases of high- and intermediate-type male anorectal malformation.

    Science.gov (United States)

    Watanabe, Yoshio; Takasu, Hidemi; Sumida, Wataru; Mori, Kensaku

    2013-04-01

    The distribution of sphincter muscle complex in anorectal malformation (ARM) needs to be investigated on a case-by-case basis. This study was undertaken to demonstrate the differences in the anal sphincter muscles between patients with the same type of ARM. Computed tomography (CT) data from cases of high- and intermediate-type male patients with ARM were reviewed using three-dimensional (3D) image analysis. Twenty-seven male patients with ARM (18 high and 9 intermediate) before anorectoplasty were assessed using multidetector-row helical CT (MRH-CT). A 3D reconstruction was made using volume rendering method. The multi-dimensional sections of the 3D reconstructed images of the pelvic muscles were then analyzed and compared with schematic drawings from the literature. The sphincters in the high and intermediate types of ARM could be divided into five groups. In 13 out of 18 cases in the high type and 7 out of 9 cases in the intermediate type, images of the sphincter muscles appeared different from schematic drawings appearing in the literature. In both high and intermediate types of ARM, more than 2/3 of cases demonstrated unexpectedly displaced and deformed hypoplastic sphincters. Therefore, we recommend that variations in anal sphincter should be investigated on an individual basis prior to surgery.

  4. Suboccipital craniectomy with opening of the fourth ventricle and duraplasty: study of 192 cases of craniovertebral malformations

    Directory of Open Access Journals (Sweden)

    Jose Alberto Goncalves da Silva

    2013-09-01

    Full Text Available The prime objective in the surgical treatment of basilar impression (BI, Chiari malformation (CM, and/or syringomyelia (SM is based on restoration of the normal cerebrospinal fluid (CSF dynamics at the craniovertebral junction and creation of a large artificial cisterna magna, avoiding the caudal migration of the hindbrain. It is observed that a large craniectomy might facilitate an upward migration of the posterior fossa structures. There are many surgical techniques to decompress the posterior fossa; however, a gold standard approach remains unclear. The authors present the results of 192 cases of BI, CM, and SM treated between 1975 and 2008 and whose surgical treatment was characterized by a large craniectomy without tonsillectomy with the patient in the sitting position, large opening of the fourth ventricle, and duraplasty.

  5. Posterior fossa decompression with tonsillectomy in 104 cases of basilar impression, Chiari malformation and/or syringomyelia

    Directory of Open Access Journals (Sweden)

    José Alberto Gonçalves da Silva

    2011-10-01

    Full Text Available The prime objective in the surgical treatment of basilar impression (BI, Chiari malformation (CM and/or syringomyelia (SM is based on the restoration of the normal cerebrospinal fluid (CSF dynamics at the craniovertebral junction through the creation of a large artificial cisterna magna. A small suboccipital craniectomy has been emphasized to avoid caudal migration of the hindbrain structures into the vertebral canal. Nevertheless, the results showed downward migration of the hindbrain related to that type of craniectomy. The authors present, otherwise, the results of 104 cases of BI, CM and/or SM, whose surgical treatment was characterized by a large craniectomy with the patient in the sitting position, tonsillectomy, large opening of the fourth ventricle and duraplasty with creation of a large artificial cisterna magna. A significant upward migration of the posterior fossa structures was detected by postoperative magnetic resonance imaging.

  6. Co-existence of Chiari malformation type I and Epstein-Barr virus meningoencephalitis in a 3-year-old child: case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Solomou, E.K.; Kotsarini, C.; Badra, F.A. [University Hospital of Patras, Department of Radiology, Rio (Greece); Krepis, A.; Papanastasiou, D. [University Hospital of Patras, Pediatric Clinic, Patras (Greece); Patriarcheas, G. [Diagnostic Centre B Diagnosis, Patras (Greece)

    2005-01-01

    In the present report we describe an unusual case of a 3-year-old girl who was admitted to our hospital with Epstein-Barr virus meningoencephalitis. Brain magnetic resonance imaging revealed diffuse abnormalities in white matter and Chiari I malformation with cervical and thoracic hydro-syringomyelia. (orig.)

  7. Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Jea Andrew

    2011-08-01

    Full Text Available Abstract Introduction Cystic fibrosis, an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator gene, is not generally associated with malformations of the central nervous system. We review eight previously published reports detailing an infrequent association between cystic fibrosis and Chiari I malformation. Case presentation To the best of our knowledge, our report describes only the ninth case of a baby presenting with a new diagnosis of cystic fibrosis and Chiari I malformation, in this case in a 10-month-old, full-term Caucasian baby boy from the United States of America. Neurosurgical consultation was obtained for associated developmental delay, macrocephaly, bulging anterior fontanel, and papilledema. An MRI scan demonstrated an extensive Chiari I malformation with effacement of the fourth ventricle, obliteration of the outlets of the fourth ventricle and triventricular hydrocephalus without aqueductal stenosis. Our patient was taken to the operating room for ventriculoperitoneal shunt placement. Conclusions It is possible that the cystic fibrosis transmembrane conductance regulator gene may play a previously unrecognized role in central nervous system development; alternatively, this central nervous system abnormality may have been acquired due to constant valsalva from recurrent coughing or wheezing or metabolic and electrolyte imbalances that occur characteristically in cystic fibrosis.

  8. 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.

  9. Congenital malformation of fetus in a pregnancy following spontaneous ovulation in a case of premature ovarian failure

    Directory of Open Access Journals (Sweden)

    Priya Selvaraj

    2010-01-01

    Full Text Available Premature ovarian failure (POF, that is, amenorrhea before 40 years of age can be attributed to a variety of etiologies. Approximately 1% of women before 30 years are diagnosed with POF. Spontaneous ovulation leading to pregnancy in POF is even a rarer entity. We report a case where congenital malformations were diagnosed in a fetus following spontaneous ovulation in a case of POF. A 33-year-old woman presented to our center with primary infertility. On complete work up, she was diagnosed with POF and conceived with hormone replacement therapy and donor oocyte program. She delivered a healthy female baby through caesarean section. The patient reviewed later with amenorrhea of 40 days and pregnancy was confirmed. However, during antenatal follow-up congenital anomalies in fetus were diagnosed sonographically. The decision for termination of pregnancy was taken. To conclude, we recommend large-scale retrospective analysis that would define medical guidelines in cases of pregnancy following spontaneous ovulation in POF.

  10. 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.

  11. Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

    Science.gov (United States)

    Sgulò, Francesco Giovanni; Spennato, Pietro; Aliberti, Ferdinando; Di Martino, Giuliana; Cascone, Daniele; Cinalli, Giuseppe

    2017-01-01

    Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

  12. Neurofibromatosis type 1 and Chiari type 1 malformation: A case report and literature review of a rare association

    Science.gov (United States)

    Pozetti, Marianne; Belsuzarri, Telmo Augusto Barba; Belsuzarri, Natalia C. B.; Seixas, Naira B.; Araujo, João F. M.

    2016-01-01

    Background: The association between neurofibromatosis type 1 (NF-I) and Chiari I malformation (CMI) is rare, and not many studies are reported in the literature. Performing magnetic resonance imaging (MRI) in patients with NF-1 is essential because several cases of Chiari type I are completely asymptomatic. We emphasize the need for inclusion of Chiari I as diagnosis in association with NF-1. Case Description: The patient was a 51-year-old black man who presented with complaints of pain and decreased motion and sensibility of his hands, wrists, and forearms, along with progressive dysarthria. Even though the computed tomography (CT) scan of the skull did not show changes, the MRI showed hydro/syringomyelia in the cervical spine area. Midline suboccipital craniectomy with total laminectomy of c1 and partial laminectomy of c2 was performed; tonsillectomy was also performed for cistern expansion because of intense thickening and obliteration of the obex by the cerebellar tonsils. Following treatment, the patient showed remission of symptoms. Conclusion: NF-1 in association with CMI is rare, and early diagnosis and surgical treatment are essential to slow down the myelopathy; although they prevent neurological damages, patients with NF-1 must remain under doctor's attention in case of association with CMI. Our literature review showed that symptoms can vary and include headache, gait disturbance, and sensory/motor diminution, until asymptomatic patients. Moreover, the incidence of NF-1 is considerably higher in CMI patients in comparison to the global incidence (8.6–11.8% and 0.775%, respectively). The surgical technique must be evaluated case by case according to the degree of cerebrospinal fluid obstruction. PMID:27500008

  13. Headache in children with Chiari I malformation.

    Science.gov (United States)

    Toldo, Irene; Tangari, Marta; Mardari, Rodica; Perissinotto, Egle; Sartori, Stefano; Gatta, Michela; Calderone, Milena; Battistella, Pier Antonio

    2014-05-01

    Headache is the most common symptom of Chiari 1 malformation, a condition characterized by the herniation of cerebellar tonsils through the foramen magnum. However, the headache pattern of cases with Chiari 1 malformations is not well defined in the literature, especially in children. The aim of this retrospective chart review was to evaluate the frequency and the characteristics of headache in children with Chiari 1 malformation at initial evaluation and during follow up. Forty-five cases with tonsillar ectopia were selected among 9947 cases under 18 years of age who underwent neuroimaging between 2002 and 2010. A semistructured clinical interview (mean follow-up: 5.2 years) was conducted. Headache was classified according to the second edition of the International Classification of Headache Disorders. Possible associations between clinical picture, in particular headache pattern, but also other signs and symptoms attributable to Chiari 1 malformation, and the extent of tonsillar ectopia were found for 3 different groups: those with borderline (headache, and 9/33 (27%) of those patients (5 with mild and 4 with severe tonsillar ectopia) reported headache attributed to Chiari 1 malformation. In our studied pediatric population, the most common symptom for cases diagnosed with Chiari 1 malformation was headache, and headache attributed to Chiari 1 malformation was the most common headache pattern in patients with Chiari 1 malformation. The presence of headache attributed to Chiari 1 malformation along with 3 other signs or symptoms of Chiari 1 malformation were highly predictive of severe tonsillar ectopia. © 2014 American Headache Society.

  14. Syndrome of absent abdominal muscles: two cases with microcephaly, polymicrogyria, and cerebellar malformations

    Science.gov (United States)

    Heffner, Reid R.

    1970-01-01

    Two unique cases of the syndrome of absent abdominal muscles with central nervous system involvement are presented. Microcephaly, polymicrogyria, and cerebellar heterotopiae were present in both. In case 1 there was also absence of the corpus callosum and agenesis of the cerebellar vermis. In case 2 a count of anterior horn cells in the spinal cord showed a reduction of approximately 50% in the lower thoracic region. The pertinent literature is briefly discussed. The findings in the nervous system suggest that the syndrome is the result of defective embryogenesis during the first trimester. Images PMID:4250700

  15. A case-control study of maternal periconceptual and pregnancy recreational drug use and fetal malformation using hair analysis.

    Directory of Open Access Journals (Sweden)

    Anna L David

    Full Text Available OBJECTIVE: Maternal recreational drug use may be associated with the development of fetal malformations such as gastroschisis, brain and limb defects, the aetiology due to vascular disruption during organogenesis. Using forensic hair analysis we reported evidence of recreational drug use in 18% of women with a fetal gastroschisis. Here we investigate this association in a variety of fetal malformations using the same method. METHODS: In a multi-centre study, women with normal pregnancies (controls and those with fetal abnormalities (cases gave informed consent for hair analysis for recreational drug metabolites using mass spectrometry. Hair samples cut at the root were tested in sections corresponding to 3 month time periods (pre and periconceptual period. RESULTS: Women whose fetus had gastroschisis, compared to women with a normal control fetus, were younger (mean age 23.78 ± SD4.79 years, 18-37 vs 29.79 ± SD6 years, 18-42, p = 0.00001, were more likely to have evidence of recreational drug use (15, 25.4% vs 21, 13%, OR2.27, 95thCI 1.08-4.78, p = 0.028, and were less likely to report periconceptual folic acid use (31, 53.4% vs 124, 77.5%, OR0.33, 95thCI 0.18-0.63, p = 0.001. Age-matched normal control women were no less likely to test positive for recreational drugs than women whose fetus had gastroschisis. After accounting for all significant factors, only young maternal age remained significantly associated with gastroschisis. Women with a fetus affected by a non-neural tube central nervous system (CNS anomaly were more likely to test positive for recreational drugs when compared to women whose fetus was normal (7, 35% vs 21, 13%, OR3.59, 95th CI1.20-10.02, p = 0.01. CONCLUSIONS: We demonstrate a significant association between non neural tube CNS anomalies and recreational drug use in the periconceptual period, first or second trimesters, but we cannot confirm this association with gastroschisis. We confirm the association of

  16. Combined Spinal-Epidural Analgesia for Laboring Parturient with Arnold-Chiari Type I Malformation: A Case Report and a Review of the Literature

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    Clark K. Choi

    2013-01-01

    Full Text Available Anesthetic management of laboring parturients with Arnold-Chiari type I malformation poses a difficult challenge for the anesthesiologist. The increase in intracranial pressure during uterine contractions, coughing, valsalva maneuvers, and expulsion of the fetus can be detrimental to the mother during the process of labor and delivery. No concrete evidence has implicated high cerebral spinal fluid pressure on maternal and fetal complications. The literature on the use of neuraxial techniques for managing parturients with Arnold-Chiari is extremely scarce. While most anesthesiologists advocate epidural analgesia for management of labor pain and spinal anesthesia for cesarean section, we are the first to report the use of combined spinal-epidural analgesia for managing labor pain in a pregnant woman with Arnold-Chiari type I malformation. Also, we have reviewed the literature and presented information from case reports and case series to support the safe usage of neuraxial techniques in these patients.

  17. Intraoperative somatosensory evoked potential recovery following opening of the fourth ventricle during posterior fossa decompression in Chiari malformation: case report.

    Science.gov (United States)

    Grossauer, Stefan; Koeck, Katharina; Vince, Giles H

    2015-03-01

    The most appropriate surgical technique for posterior fossa decompression in Chiari malformation (CM) remains a matter of debate. Intraoperative electrophysiological studies during posterior fossa decompression of Type I CM (CM-I) aim to shed light on the entity's pathomechanism as well as on the ideal extent of decompression. The existing reports on this issue state that significant improvement in conduction occurs after craniotomy in all cases, but additional durotomy contributes a further improvement in only a minority of cases. This implies that craniotomy alone might suffice for clinical improvement without the need of duraplasty or even subarachnoid manipulation at the level of the craniocervical junction. In contrast to published data, the authors describe the case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. Postoperatively, the patient recovered completely from her preoperative neurological deficits. To the authors' knowledge, this is the first report of significant SSEP recovery after opening the fourth ventricle in the decompression of a CM-I. The electrophysiological and operative techniques are described in detail and the findings are discussed in the light of available literature. The authors conclude that there might be a subset of CM-I patients who require subarachnoid dissection at the level of the craniocervical junction to benefit clinically. Prospective studies with detailed electrophysiological analyses seem warranted to answer the question regarding the best surgical approach in CM-I decompression.

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

    Science.gov (United States)

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

    2017-05-01

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

  19. The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association.

    Science.gov (United States)

    Goswami, Dibyajyoti; Kusre, Giriraj; Dutta, Hemonta Kumar; Sarma, Adity

    2013-08-01

    The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism. On the other hand, the urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence. A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig's cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing knowledge on the prune belly syndrome.

  20. Influence of patient age on angioarchitecture of brain arteriovenous malformations.

    Science.gov (United States)

    Hetts, S W; Cooke, D L; Nelson, J; Gupta, N; Fullerton, H; Amans, M R; Narvid, J A; Moftakhar, P; McSwain, H; Dowd, C F; Higashida, R T; Halbach, V V; Lawton, M T; Kim, H

    2014-07-01

    The imaging characteristics and modes of presentation of brain AVMs may vary with patient age. Our aim was to determine whether clinical and angioarchitectural features of brain AVMs differ between children and adults. A prospectively collected institutional data base 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 often differed when age was treated as a continuous variable as opposed to dividing subjects into children (18 years or younger; n = 203) versus adults (older than 18 years; n = 630). Children were more likely to present with AVM hemorrhage than adults (59% versus 41%, P 6 cm compared with 37.1 years for <3 cm), this feature was not significantly different between children and adults (P = .069). Exclusively deep venous drainage was more common in younger subjects when age was treated continuously (P = .04) or dichotomized (P < .001). Venous ectasia was more common with increasing age (mean, 39.4 years with ectasia compared with 31.1 years without ectasia) and when adults were compared with children (52% versus 35%, P < .001). Patients with feeding artery aneurysms presented at a later average age (44.1 years) than those without such aneurysms (31.6 years); this observation persisted when comparing children with adults (13% versus 29%, P < .001). 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 under-represented in children, suggesting that these particular high-risk features take time to develop. © 2014 by American Journal of Neuroradiology.

  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. 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

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

    Science.gov (United States)

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

    2015-02-01

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

  4. Congenital cystic adenomatoid malformation of the lung. Presentation of 16 cases

    Energy Technology Data Exchange (ETDEWEB)

    Beluffi, G. (IRCCS Politechnico S. Matteo, Pavia (Italy)); Brokensha, C. (Alder Hey Children' s Hospital, Liverpool (UK)); Kozlowski, K. (Royal Alexandra Hospital for Children, Sydney (Australia)); Lucaya, J. (Ciudad Sanitaria de la Seguridad Social, Barcelona (Spain). Clinica Infantil); Masel, J. (Royal Children' s Hospital, Brisbane (Australia)); Morris, L. (Adelaide Children' s Hospital (Australia)); Rosso, R. (Pavia Univ. (Italy). Inst. of Anatomic Patology); Stronati, M. (Pavia Univ. (Italy). Div. of Neonatal Pathology); Thomson, R. (Princess Margaret Hospital for Children, Perth (Australia))

    1989-05-01

    Sixteen cases of CCAM are presented, one with bilateral disease, diagnosed at different times, and one with an associated prune belly syndrome, to be added to the 405 already reported in the literature, and their clinical, radiological and pathological features are described. (orig./MG).

  5. 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...

  6. Chiari 1 malformation presenting as central sleep apnea during pregnancy: a case report, treatment considerations, and review of the literature

    Directory of Open Access Journals (Sweden)

    Erik K. St. Louis

    2014-10-01

    Full Text Available Purpose: Chiari malformation (CM type 1 frequently causes obstructive or central sleep-disordered breathing (SDB in both adults and children, although SDB is relatively rare as a presenting manifestation in the absence of other neurological symptoms. The definitive treatment of symptomatic CM is surgical decompression. We report a case that is, to our knowledge, a novel manifestation of central sleep apnea (CSA due to CM type 1 with severe exacerbation and initial clinical presentation during pregnancy.Methods: Case report from tertiary care comprehensive sleep medicine center with literature review of sleep-disordered breathing manifestations associated with CM type 1. PubMed search was conducted between January 1982 and October 2013. Results: We report a 25-year-old woman with severe central sleep apnea initially presenting during her first pregnancy that eventually proved to be caused by CM type 1. The patient was successfully treated preoperatively by adaptive servoventilation (ASV, with effective resolution of sleep-disordered breathing following surgical decompression, and without recurrence in a subsequent pregnancy.Our literature review found that 58% of CM patients with SDB had OSA alone, 28% had CSA alone, 8 (10% had mixed OSA/CSA, and 6 (8% had hypoventilation. Of CM patients presenting with SDB, 50% had OSA, 42% had CSA, 8% had mixed OSA/CSA, and 10.4% had hypoventilation. We speculate that CSA may develop in CM patients in whom brainstem compression results in excessive central chemoreflex sensitivity with consequent hypocapnic CSA.Conclusions: CM type 1 may present with a diversity of SDB manifestations, and timely recognition and surgical referral are necessary to prevent further neurological deficits. ASV therapy can effectively manage CSA caused by CM type 1, which may initially present during pregnancy.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. A STUDY OF POSTERIOR FOSSA MALFORMATIONS: MR IMAGING

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    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.

  9. 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.

  10. 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

  11. Congestive heart failure: a review and case report from a chiropractic teaching clinic.

    Science.gov (United States)

    Osterhouse, Melanie D; Kettner, Norman W; Boesch, Ron

    2005-06-01

    To discuss the case of a 62-year-old woman with congestive heart failure (CHF), precipitated by a previous arteriovenous malformation, and to review the clinical presentation, pathophysiology, and treatment options for patients with CHF. The patient complained of pain, rapid weight gain, and shortness of breath. The index event for this patient was known to be an arteriovenous malformation. Biventricular cardiomegaly with pulmonary venous hypertension was evident on chest radiographs. The patient received both medical care (drug therapy) and chiropractic care (manipulation and soft tissue techniques to alleviate symptoms and discomfort). Patients with known and undiagnosed CHF may visit the chiropractic physician; thus, knowledge of comprehensive care, differential diagnosis, and continuity of care are important. Chiropractic management may be helpful in alleviating patient discomfort. Further clinical investigations may help to clarify the role of complementary and alternative care in the diagnosis and treatment of CHF.

  12. Fístula axilo-cava para hemodiálise: relato de caso Axillary arteriovenous fistula for hemodialysis: case report

    Directory of Open Access Journals (Sweden)

    Yosio Nagato

    2009-12-01

    Full Text Available Na confecção de fístula arteriovenosa (FAV para hemodiálise, condutos venosos autógenos demonstram performance superior quando comparados com material protético em relação à perviedade primária ou secundária. A prótese de politetrafluoroetileno (PTFE é reservada para casos de falência de material autógeno e é geralmente utilizada em fístulas em membros superiores. Descrevemos o caso de uma paciente de 52 anos que, após falência de acessos para hemodiálise e impossibilidade de realização de diálise peritoneal em razão de peritonite bacteriana, foi submetida à confecção de FAV entre a artéria axilar direita e a veia cava inferior com prótese de PTFE de 6 mm. O acesso foi utilizado para hemodiálise 1 mês após sua criação e permanece pérvio após 24 meses. Até o momento, não houve complicações infecciosas, sinais de insuficiência cardíaca ou síndrome de roubo em membro superior direito.With regards to the creation of an arteriovenous fistula (AV fistula for hemodialysis, autogenous venous grafts clearly show high performance when compared with prosthetic material in terms of primary or secondary patency. Polytetrafluoroethylene (PTFE grafts for the reconstruction of AV fistulae must be restricted to cases of failure of the autogenous material, which is generally used in upper limb fistulae. We describe a case of a 52-year-old patient, who, after access failure for hemodialysis and the impossibility of performing peritoneal dialysis due to bacterial peritonitis, underwent the reconstruction of an AV fistula between the right axillary artery and the cava vein using a 6-mm PTFE prosthesis. One month after surgery, this AV fistula started to be used for hemodialysis. The AV fistula remains patent 24 months after its creation. No infectious complications, cardiac insufficiency symptoms, or steal syndromes of right upper limb were detected.

  13. Spontaneous hemothorax caused by rupture of an intercostal artery aneurysm in neurofibromatosis Type I: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Shim, Chang Min; Na, Jae Beom; You, Jin Jong; Chung, Sung Hoon [Gyeongsang National Univ. College of Medicine, Pusan (Korea, Republic of)

    2001-01-01

    Neurofibromatosis type I (NF-1) is the most common neurocutaneous syndrome. Associated vascular abnormalities are arterial occlusion, aneurysm, ectasia and arteriovenous malformation. Spontaneous massive hemothorax due to rupture of an arterial aneurysm is rare but fatal. It is, therefore, essential to determine the location of an aneurysm and provide immediate surgical or the interventional treatment. We report a case of spotaneous hemothorax caused by rupture of an intercostal arterial aneurysm diagnosed by CT and angiography.

  14. 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.

  15. Human fetal malformations associated with the use of an angiotensin II receptor antagonist: Case Report

    Directory of Open Access Journals (Sweden)

    Henri Augusto Korkes

    2014-09-01

    Full Text Available Introduction: The potential risks related to drug exposure during pregnancy represent a vast chapter in modern obstetrics and data regarding the safety of antihypertensive drugs during pregnancy are relatively scarce. Case report: A 37-year-old patient discovered her fifth pregnancy at our hospital after 26 weeks and 4 days of gestation. She reported a history of hypertension and was currently being treated with Losartan. Hospitalization was recommended for the patient and further evaluation of fetal vitality was performed. On the fourth day an ultrasound was performed, resulting in a severe oligohydramnios, fetal centralization and abnormal ductus venosus. After 36 hours, the newborn died. Pathologic evaluation: At autopsy, the skullcap had large fontanels and deficient ossification. The kidneys were slightly enlarged. A microscopic examination detected underdevelopment of the tubules and the presence of some dilated lumens. Immunohistochemical detection of epithelial membrane antigen was positive. Immunoreactivity of CD 15 was also assayed to characterize the proximal tubules, and lumen collapse was observed in some regions. Discussion: Angiotensin-converting enzyme inhibitors (ACEIs and angiotensin receptor antagonists (ARAs are among the most widely prescribed drugs for hypertension. They are often used by hypertensive women who are considering become pregnant. While their fetal toxicity in the second or third trimesters has been documented, their teratogenic effect during the first trimester has only recently been demonstrated. Conclusion: Constant awareness by physicians and patients should be encouraged, particularly in regard to the prescription of antihypertensive drugs in women of childbearing age who are or intend to become pregnant.

  16. A traumatic dural arteriovenous fistula between the inferolateral trunk of the internal carotid artery and the ophthalmic vein: A case of transvenous coil embolization via the facial vein

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jun Young; Hong, Chang Ki; Suh, Sang Hyun [Dept. of Radiology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Dong Ik [Dept. of of Radiology, CHA Bundang Medical Center, CHA University, Seongnam (Korea, Republic of)

    2017-05-15

    A 31-year-old man was admitted with exophthalmos. He suffered from progressive exophthalmos, bruit and conjunctival chemosis 7 days after head trauma caused by falling down. Cerebral angiography showed a dural arteriovenous fistula (DAVF) draining into the ophthalmic vein caused by tear in the inferolateral trunk, which is a rare presentation of traumatic DAVF. Selective transvenous coil embolization was performed via the facial vein without neurologic complications.

  17. A case of a spontaneous intraorbital arteriovenous fistula: clinico-radiological findings and treatment by transvenous embolisation via the superior ophthalmic vein.

    Science.gov (United States)

    Naqvi, Jawad; Laitt, Roger; Leatherbarrow, Brian; Herwadkar, Amit

    2013-04-01

    A 72-year-old male presented with progressive right axial proptosis and red eye. Catheter angiography demonstrated an intraorbital arteriovenous fistula (IAVF) distal to the central retinal artery (CRA). Transvenous embolisation following direct surgical exposure of the superior ophthalmic vein (SOV) resulted in rapid resolution of his symptoms and signs. Transvenous embolisation via the SOV is a safe, effective alternative to transarterial embolisation for treating spontaneous IAVF where transarterial embolisation poses a risk of CRA occlusion.

  18. Tetralogy of Fallot associated with macrocephaly-capillary malformation syndrome: a case report and review of the literature

    OpenAIRE

    2009-01-01

    Abstract Introduction Macrocephaly-capillary malformation syndrome is characterized by cutaneous vascular lesions, including cutis marmorata telangiectatica and hemangiomas, associated with congenital anomalies, including macrocephaly, macrosomia, asymmetry and mental retardation. In addition to these cardinal signs, several other clinical conditions have been reported in people with this condition. However, to the best of our knowledge, the presence of tetralogy of Fallot has not previously ...

  19. Malformación aneurismática de la vena de Galeno: Reporte de un caso Vein of Galen aneurysmal malformation: a case report

    Directory of Open Access Journals (Sweden)

    Luis F Carvajal

    2011-06-01

    Full Text Available Las malformaciones aneurismáticas de la vena de Galeno representan aproximadamente un tercio de todas las malformaciones vasculares intracraneales en la edad pediátrica; sin embargo, existe poca referencia en el medio. Los pacientes con este tipo de malformación presentaban altas tasas de morbimortalidad hasta la década de los noventa; desde entonces el desarrollo en las técnicas de diagnóstico prenatal y de terapia endovascular han permitido modificar el pronóstico de esta población. Se describe el caso de un paciente en quien se hizo diagnóstico prenatal, se realizó tratamiento con terapia endovascular a los cuatro meses de vida, y se obtuvieron buenos resultados. Se hace enfásis en el diagnóstico temprano y en la prevención de secuelas a través de una intervención oportuna.Aneurysmal malformations of the vein of Galen represent approximately one third of all intracranial vascular malformations in children. However, in our country we have few references. Patients with this type of malformation had high morbi-mortality rates until the early nineties; since then, the development of techniques of prenatal diagnosis and endovascular therapy have allowed to modify the prognosis of these patients. We describe the case of a patient in whom prenatal diagnosis of aneurysmal malformation of the vein of Galen was made, and was managed at four months of age with endovascular therapy with good results. We emphasize on early diagnosis and prevention of sequelae through appropriate intervention.

  20. [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.

  1. 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.

  2. Lateral sacral artery supply to an intramedullary arteriovenous fistula at the conus medullaris

    Energy Technology Data Exchange (ETDEWEB)

    Mochizuki, T.; Nemoto, Y.; Inoue, Y.; Tashiro, T. (Osaka City Univ. Medical School (Japan). Dept. of Radiology); Sakanaka, H. (Osaka City Univ. Medical School (Japan). Dept. of Orthopedics)

    1991-10-01

    In this 25-year-old woman, severe low back pain, flaccid paraparesis, sensory disturbance of both legs and sphincter dysfunction resulted from an intramedullary conal arteriovenous malformation fed by an anterior spinal artery arising from the left sixth intercostal artery and by the left lateral sacral artery, a branch of the left internal iliac artery. Magnetic resonance (MR) images showed low signal (flow void) within an intramedullary mass that expanded the conus from T12 to L1. (orig./GDG).

  3. 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

  4. 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.

  5. 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.

  6. Giant cavernous malformation in the ventrolateral midbrain with extension into the thalamus: a case report of a paramedian supracerebellar transtentorial approach.

    Science.gov (United States)

    Duan, Hongzhou; Hara, Yosuke; Goto, Tetsuya; Chiba, Akihiro; Hongo, Kazuhiro

    2016-08-01

    Cavernous malformations (CMs) of the midbrain and thalamus are relatively rare and particularly difficult to be resected given their location in eloquent tissues. Here, we report a case of a 14-year-old boy who experienced repeated and progressive right hemiparesis. Image examinations showed a gradually enlarged CM originated in the left ventrolateral midbrain extending to the left thalamus with repeated hemorrhage. By performing a paramedian supracerebellar transtentorial approach, the CM was totally removed, and the patient recovered without any new neurological deficit. The authors' experience suggests that this approach is eminent in treating giant lesions involving the ventrolateral midbrain and thalamus.

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

    Science.gov (United States)

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

    2010-10-01

    The authors conducted a study to determine the factors associated with right-sided language dominance in patients with cerebrovascular malformations. Twenty-two patients with either arteriovenous malformations (AVMs [15 cases]) or cavernous malformations (7 cases) underwent functional MR (fMR) imaging studies of language function; a 3.0-T head-only unit was used. Lateralization indices were calculated separately for Broca and Wernicke areas. Lesion size, Spetzler-Martin grade, and the distance between the lesion and anatomically defined language cortex were calculated for each patient. Right-sided language dominance occurred in 5 patients, all of whom had AVMs within 10 mm of canonical language areas. Three patients had right-sided language dominance in the Wernicke area alone whereas 2 had right-sided language dominance in both Broca and Wernicke areas. Wada testing and intraoperative electrocortical stimulation were performed as clinically indicated to corroborate fMR imaging findings. The primary factor associated with right-sided language dominance was the AVM being within 10 mm of anatomically defined language areas. The lesion size and the Spetzler-Martin grade were not significant factors. Anomalous fMR imaging laterality was typically confined to the language area proximate to the lesion, with the distal language area remaining in the left hemisphere dominant. This study emphasizes the need to map each case individually in patients with left perisylvian AVMs. Assumptions about eloquent cortex based on anatomical landmarks (a key component of Spetzler-Martin grading) may have to be reconsidered.

  8. 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.

  9. [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.

  10. 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,

  11. Sediment quality assessment using survival and embryo malformation tests in amphipod crustaceans: The Gulf of Riga, Baltic Sea AS case study

    Science.gov (United States)

    Strode, Evita; Jansons, Mintauts; Purina, Ingrida; Balode, Maija; Berezina, Nadezhda A.

    2017-08-01

    The aim of this study was to assess the toxicity of bottom sediment and to estimate the potential effects of contaminated sediment on health of benthic organisms in the Gulf of Riga (eastern Baltic Sea). Two endpoints were used: survival rate (acute toxicity test) of five crustacean amphipod species and frequency of embryo malformation (samples were collected from the field) in the two species. Toxic resistance of living animals to sediment quality was measured as survival rate (%) at 25 study sites from 2010-2012. Significant differences in the toxic resistance between species were found: 80-100% for Monoporeia affinis, 70-95% for Corophium volutator, 38-88% for Pontogammarus robustoides, 38-100% for Bathyporeia pilosa and 60-100% for Hyalella azteca. Reproductive disorders, measured as percentage (%) of malformed embryos per female, varied in the ranges of 0.0-9.5% in deep water species M. affinis and 0.3-7.5% in littoral species P. robustoides. Both the acute toxicity test and embryo malformation test (only M. affinis was used) indicated moderate and poor sediment quality at 20% and 12% accordingly in the study sites, low toxicity of sediment was estimated in 64% of cases, and no toxicity was recorded in the rest of the cases (4%). Additionally, sediment toxicity test using aquatic organisms was combined with sediment chemical analysis (trace metals) and the Benthic Quality Index (macrozoobenthos) was based on data collected from 13 sites in the Gulf of Riga in 2010 and used for triad sediment quality assessment. According to this combined approach, 23% of the bottom sediments were classified as likely impacted and 23% as possibly impacted (central and southern part of the Gulf). However, the remaining 54% was identified as likely un-impacted. The sediment quality assessment with single survival test or chemical analyses showed better sediment quality in the Gulf than the triad method. The embryo malformation test appeared to be more sensitive to pollution than

  12. 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.)

  13. Focal dermal hypoplasia: report of a case with myelomeningocele, Arnold-Chiari malformation and hydrocephalus with a review of neurologic manifestations of Goltz syndrome.

    Science.gov (United States)

    Peters, Tess; Perrier, Renee; Haber, Richard M

    2014-01-01

    Focal dermal hypoplasia (Goltz syndrome, Online Mendelian Inheritance in Man [OMIM] 305600) is a rare X-linked dominant congenital disorder involving defects of mesodermal- and ectodermal-derived structures. It is associated with mutations in the PORCN gene, a regulator of Wnt signaling proteins. The phenotype is highly variable, although all describe characteristic skin findings as a primary diagnostic feature. To date there are few case reports of focal dermal hypoplasia associated with central nervous system abnormalities. We report the second case of focal dermal hypoplasia associated with myelomenigocele, Arnold-Chiari malformation and hydrocephalus and the first in a male. Genetic testing identified a novel mosaic three base pair deletion within the PORCN gene (c.853_855delACG). This case highlights the importance of neurological evaluation in focal dermal hypoplasia and consideration of other syndromes more commonly associated with central nervous system abnormalities. In this report we summarize the literature on neurological manifestations in Goltz syndrome.

  14. Splenic arteriovenous fistula and sudden onset of portal hypertension as complications of a ruptured splenic artery aneurysm: Successful treatment with transcatheter arterial embolization. A case study and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Dimitrios Siablis; Zafiria G Papathanassiou; Dimitrios Karnabatidis; Nikolaos Christeas; Konstantinos Katsanos; Constantine Vagianos

    2006-01-01

    Splenic arteriovenous fistula (SAVF) accounts for an unusual but well-documented treatable cause of portal hypertension[1-4]. A case of a 50-year-old multiparous female who developed suddenly portal hypertension due to SAVF formation is presented. The patient suffered from repeated episodes of haematemesis and melaena during the past twelve days and thus was emergently admitted to hospital for management. Clinical and laboratory investigations established the diagnosis of portal hypertension in the absence of liver parenchymal disease. Endoscopy revealed multiple esophageal bleeding varices. Abdominal computed tomography (CT)and transfemoral celiac arteriography documented the presence of a tortuous and aneurysmatic splenic artery and premature filling of an enlarged splenic vein, findings highly suggestive of an SAVF. The aforementioned vascular abnormality was successfully treated with percutaneous transcatheter embolization. Neither recurrence nor other complications were observed.

  15. Malformación vascular intraósea en el maxilar que se presenta en forma de sangrado gingival Intra-osseous vascular malformation in the maxilla

    Directory of Open Access Journals (Sweden)

    Naveenjayakumar

    2009-12-01

    Full Text Available Las malformaciones arterio-venosas intraóseas (MAVs en la región maxilofacial son entidades clínicas poco frecuentes. Ofrecen una amplia gama de presentación clínica y no siempre se diagnostican sin sorpresa. El tratamiento de estas lesiones siempre ha representado un reto para el cirujano debido a su intensa vascularización y a la elevada incidencia de recurrencia. Se presenta el caso de una malformación arterio-venosa intraósea en el maxilar superior que se manifiesta como sangrado gingival, cuyos exámenes radiológicos de rutina no fueron concluyentes. Se llevó a cabo la excisión quirúrgica y el empaquetado del hueso con cera, y se realizó el seguimiento del paciente durante 1 año sin que se produjera recurrencia. Las malformaciones vasculares deben tenerse en consideración en el diagnóstico diferencial del sangrado gingival con radiografías dentales no concluyentes.Intraosseous arterio-venous malformations (AVM in the maxillofacial region are rare clinical entities. They have a wide range of clinical presentation and are not always diagnosed without a surprise. Treatment of these lesions has always been a challenge to the surgeon due to extreme vascularity and the high recurrence rate. The case presented is that of an intraosseous arterio-venous malformation in the maxilla that started as gingival bleeding and routine radiographic examinations were inconclusive. Surgical excision and bone wax packing was performed there were no recurrences during 1-year of follow-up. Vascular malformations may be considered in differential diagnosis of gingival bleeding with inconclusive dental radiographs.

  16. The ejaculatory duct ectopically invading the bladder with multiple congenital malformations of the homolateral urogenital system: a report of a rare case and an embryological review

    Institute of Scientific and Technical Information of China (English)

    Feng Wang; Hong-Fei Wu; Jie Yang

    2009-01-01

    We report a rare case of a left ejaculatory duct that allotropically protrudes towards or invades the left vesicletriangular area with its dead end. The patient simultaneously exhibited multiple congenital malformations of thehomolateral urogenital system, such as absence of the left kidney, dysplasia and allotopia of the left seminal vesicle,absence of the left ureterostoma, separation between the left testis and the epididymis tail, and maldevelopment ofthe left testis. According to all clinical and laboratory evidence, the case represented a new syndrome, which wenamed Wuyang's syndrome. It involved a rare phenomenon in embryonic development; the dysplastic proximalvas precursor, having intruded into a common mesonephric duct and accidentally encroaching on the ureteric budposition, resulted in the absence or dysplasia of the homolateral urinary tract and ectopic invasion of the bladder bythe homolateral seminal tract.

  17. Cognard Type V intracranial dural arteriovenous fistula presenting in a pediatric patient with rapid, progressive myelopathy.

    Science.gov (United States)

    Jermakowicz, Walter J; Weil, Alexander G; Vlasenko, Artyom; Bhatia, Sanjiv; Niazi, Toba N

    2017-08-01

    Cognard Type V dural arteriovenous fistulas (dAVFs) are a unique type of cranial vascular malformation characterized by congestion of the perimedullary venous system that may lead to devastating spinal cord pathology if left untreated. The authors present the first known case of a pediatric patient diagnosed with a Type V dAVF. A 14-year-old girl presented with a 3-week history of slowly progressive unilateral leg weakness that quickly progressed to bilateral leg paralysis, sphincter dysfunction, and complete sensory loss the day of her presentation. MRI revealed an extensive T2 signal change in the cervical spine and tortuous perimedullary veins along the entire length of the cord. An emergency cranial angiogram showed a Type V dAVF fed by the posterior meningeal artery with drainage into the perimedullary veins of the cervical spine. The fistula was not amenable to embolization because vascular access was difficult; therefore, the patient underwent urgent suboccipital craniotomy and ligation of the arterialized venous drainage from the fistula. The patient's clinical course immediately reversed; she had a complete recovery over the course of a year, and she remains asymptomatic at the 2-year follow-up. This report adds to a growing body of evidence that describes the diverse and unpredictable nature of Type V dAVFs and highlights the need to obtain a cranial angiogram in pediatric patients with unexplained myelopathy and cervical cord T2 signal change on MRI.

  18. Case report: Manual lymphatic drainage and kinesio taping in the secondary malignant breast cancer-related lymphedema in an arm with arteriovenous (A-V) fistula for hemodialysis.

    Science.gov (United States)

    Chou, Ya-Hui; Li, Shu-Hua; Liao, Su-Fen; Tang, Hao-Wei

    2013-08-01

    Lymphedema is a dreaded complication of breast cancer treatment. The standard care for lymphedema is complex decongestive physiotherapy, which includes manual lymphatic drainage (MLD), short stretch bandaging, exercise, and skin care. The Kinesio Taping could help to improve lymphatic uptake. We reported a patient with unilateral secondary malignant breast cancer-related lymphedema and arteriovenous (A-V) fistula for hemodialysis happened in the same arm, and used kinesio taping, MLD, and exercise to treat this patient because no pressure could be applied to the A-V fistula. The 12-session therapy created an excellent effect. We do not think the kinesio taping could replace short stretch bandaging, but it could be another choice for contraindicating pressure therapy patients, and we should pay attention to wounds induced by kinesio tape.

  19. Characterization of Live Birth with Congenital Malformations

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    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. Living fetus without congenital malformation in a singleton partial hydatidiform molar pregnancy: a case report and review of the literature

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    Babita Ramani

    2014-08-01

    Full Text Available A 36 years old lady gravida 2 para 1, came to our emergency ward at 9:30 pm on 12th June 2010 with complains of bleeding p/v for 2 hours with history of bleeding at 12 weeks. On examination she was anemic and uterus was 32 weeks size with good fetal heart sound. Bleeding was coming through os, on p/s examination. Ultrasonography showed a single live fetus of 1033 grams and thickened placenta (79 mm thickness showing multiple cystic lesion with peripheral hypervascularity, giving an impression of partial hydatidiform mole. She was managed conservatively and delivered a live preterm male child of 960 grams on 13th June 2010 evening without any congenital malformation. Placental weight was 1800 grams with multiple small vesicles. Now the boy is 4 years old and going to school with normal developmental milestone. [Int J Reprod Contracept Obstet Gynecol 2014; 3(4.000: 1130-1133

  1. Upper airway dimensions in patients with craniocervical junction malformations with and without sleep apnea. A pilot case-control study

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    Ramon Barbalho Guerreiro

    2015-04-01

    Full Text Available Objective Patients with craniocervical junction malformations (CCJM tend to suffer more frequently from sleep respiratory disturbances, which are more frequent and severe in patients with basilar invagination. Here we evaluate if patients with CCJM and sleep respiratory disorders (SRD present smaller airway dimensions than patients without SRD. Method Patients with CCCM with and without sleep respiratory disturbances were evaluated clinically by Bindal's score, modified Mallampati classification, full-night polysomnography and upper airway cone beam tomography. Results Eleven patients had sleep respiratory disorders (SRD, and nine patients performed control group without SRD. CCJM patients with SRD were predominantly female, older, had higher BMI, were more likely to have Mallampati grades 3 and 4 and had statistically significant smaller anteroposterior diameter of the upper airway than patients without SRD. Conclusion Patients with CCJM and sleep respiratory disturbances have higher BMI, higher Mallampati score and smaller anterior posterior diameter of the upper airway.

  2. The efficacy of resection of an intradural extramedullary foramen magnum cavernous malformation presenting with repeated subarachnoid hemorrhage: a case report.

    Science.gov (United States)

    Oishi, Tomoya; Sakai, Naoto; Sameshima, Tetsuro; Kawaji, Hiroshi; Namba, Hiroki

    2017-03-09

    Intradural extramedullary cavernous angiomas of the central nervous system are a rare type of cavernous angioma, but they can cause fatal subarachnoid hemorrhage. The efficacy of resection for this type of cavernous malformations remains uncertain. This is the first report to recommend surgical resection of these types of lesions regardless of the fatal condition. Our patient was a 70-year-old Japanese man who experienced a sudden onset of an occipital headache, followed by bilateral abducens nerve palsy. Magnetic resonance imaging revealed a small amount of hemorrhage in both of the lateral ventricles and an intradural extramedullary mass lesion in the left side of his foramen magnum. Two weeks after the appearance of initial symptoms, he became comatose. A computed tomography scan showed an increase in the subarachnoid intraventricular hemorrhaging and of the acute hydrocephalus. Following ventricular drainage, total tumor resection was performed using the lateral suboccipital transcondylar approach in conjunction with a first cervical hemilaminectomy. We observed a grape-like vascular-rich tumor with calcification that was adhering tightly to the wall of his left vertebral artery. A histopathological examination of the surgery specimen identified it as a cavernous angioma. After placement of a ventriculoperitoneal shunt and 2 months of rehabilitation, he recovered completely. An intradural extramedullary foramen magnum cavernous malformation is quite rare. The fragile surface of our patient's lesion was causing repeated subarachnoid hemorrhage and consequently progressive fatal neurological deterioration. Surgical resection of the lesion to prevent repeated hemorrhage was performed and he recovered fully. Therefore, we recommend surgical resection of the lesion regardless of the potentially fatal condition.

  3. 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.

  4. Uterine vascular lesions: a rare cause of abnormal uterine bleeding, reporting of two cases

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    Sunita Arora

    2014-06-01

    Full Text Available Uterine vascular lesions in the form of arteriovenous malformation or pseudo aneurysm are rare but potential life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manner are essential because instrumentation that is often used for other causes of uterine bleeding can lead to massive hemorrhage. We describe two cases of uterine vascular malformation, one presenting as postabortal hemorrhage and other as postpartum hemorrhage. Case one presented as postabortal hemorrhage after induced abortion following dilatation and curettage. Case two presented as delayed postpartum hemorrhage after six weeks following cesarean section. In both cases diagnosis of uterine arteriovenous malformation was made on Doppler ultrasonography which was subsequently confirmed on pelvic angiography. The embolization of affected uterine arteries was performed successfully in both cases. Uterine vascular lesion should be suspected in patient with abnormal vaginal bleeding, especially who has recent medical history of induced abortion or dilatation and curettage or cesarean section and so on. Although angiography remains the gold standard for making diagnosis, Doppler ultrasonography is also a good noninvasive technique for the same. Uterine artery embolization offers a safe and effective treatment. [Int J Reprod Contracept Obstet Gynecol 2014; 3(3.000: 749-753

  5. [Monitoring of Somatosensory Evoked Potentials during Foramen Magnum Decompression of Chiari Malformation Type I Complicated with Syringomyelia: A Report of Two Cases].

    Science.gov (United States)

    Kadoya, Tatsuo; Takenaka, Ichiro; Kinoshita, Yuki; Shiraishi, Munehiro; Uehara, Hirofumi; Yamamoto, Toshinori; Joyashiki, Takeshi

    2015-03-01

    Few reports exist on anesthetic management for foramen magnum decompression (FMD) of Chiari malformation type I (CM I) complicated with syringomyelia. In two such cases we monitored somatosensory evoked potentials (SEP). Case 1 : A 40-year-old woman presented with occipital headache and nuchal pain for 2 months; numbness and muscular weakness of bilateral upper limbs for a month. Magnetic resonance imaging (MRI) scan showed CM I complicated with syringomyelia. Case 2 : A 32-year-old man presented with numbness and muscular weakness of bilateral upper limbs for 5 months; numbness and muscular weakness of lower limbs for 2 months. MRI scan showed CM I complicated with syringomyelia. They underwent FMD. In both cases, general anesthesia was induced with remifentanil, propofol and rocuronium, and was maintained with oxygen, air, remifentanil and propofol. Moreover, we monitored SEP. Their operative courses were uneventful. In case 1, SEP latency became shorter after FMD. Her preoperative neurologic symptoms disappeared on first postoperative day. In contrast there was no change of SEP latency after FMD in case 2. His preoperative neurologic symptoms showed no change on fifth postoperative day. SEP monitoring may be a useful index for prediction of early recovery of neurologic symptoms after FMD.

  6. 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.)

  7. 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.

  8. 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

  9. Análisis de 200 casos pediátricos de malformaciones anorectales Analysis of 200 cases of Pediatric Anorectal Malformation

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    Norma Ceciliano-Romero

    2010-06-01

    of the opening: bladder neck (12 cases, prostate (12 cases, bulbar (18 cases, vestibular (26 cases, perineal (92 cases, anal stenosis (12 cases. Those who had no fistula: high (16 cases or low (3 cases. Finally, the cloacae malformations, both high cases (2 cases and low cases (7cases. All of the patients underwent surgery using the Peña technique with the corresponding modifications. No patients were treated with the laparoscopic technique. Conclusion: Because we consider these cases to be highly complex, they must be studied holistically due to the fact that associated malformations bear much weight in the final results and in the morbimortality rate. In the majority of cases, satisfactory results are obtained. Long term follow up care that includes all necessary procedures, is essential in order for the patient to lead a normal life.

  10. Case Report: Acute obstructive hydrocephalus associated with infratentorial extra-axial fluid collection following foramen magnum decompression and durotomy for Chiari malformation type I.

    Science.gov (United States)

    Munakomi, Sunil; Bhattarai, Binod; Chaudhary, Pramod

    2016-01-01

    Acute obstructive hydrocephalus due to infratentorial extra-axial fluid collection (EAFC) is an extremely rare complication of foramen magnum decompression (FMD) and durotomy for Chiari malformation type I. Presence of infratentorial  EAFC invariably causes obstruction at the level of the fourth ventricle or aqueduct of Silvius, thereby indicating its definitive role in hydrocephalus. Pathogenesis of EAFC is said to be a local arachnoid tear as a result of durotomy, as this complication is not described in FMD without durotomy. Controversy exists in management. Usually EAFC is said to resolve with conservative management; so hydrocephalus doesn't require treatment. However, in this case EAFC was progressive and ventriculo-peritoneal shunting (VPS) was needed for managing progressive and symptomatic hydrocephalus.

  11. Vascular Malformations of the Orofacial Region

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    Daya K Jangam

    2010-01-01

    Full Text Available Vascular malformations are the benign lesion of the blood vessels or vascular elements and are considered errors of vascular morphogenesis, displaying abnormal dilatations, and channels but no i