Giant vein of Galen malformation in an adult.
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ABSTRACT:
SUBMITTER: Kerolus MG
PROVIDER: S-EPMC5551915 | biostudies-other | 2017 Sep
REPOSITORIES: biostudies-other
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Project description:Genital arteriovenous malformations are rare and present unique surgical challenges in preserving urogenital function, abdominal wall integrity, and lower limb perfusion. A 32-year-old man with a giant abdominoscrotal arteriovenous malformation presented with recurrent heavy bleeding. Due to the high risk of rebleeding and fatal hemorrhage, surgery with curative intent was proposed and the patient was counseled on the risks of ischemia to the lower limb, testes, and penis. Preoperative embolization of the feeding vessels was performed. Three days later, surgical excision of the mass with the affected scrotum, left rectus muscle, sheath, and overlying abdominal skin followed. The testes were dissected from the malformation and preserved along with the right internal pudendal artery. The left thigh skin was advanced to the scrotal remnants and a neoscrotum created. The resulting large abdominal wall defect was reconstructed in layers with a pedicled anterolateral thigh flap, including innervated vastus lateralis muscle, to prevent herniation. Recovery was uneventful, and a 4-year follow-up revealed no significant clinical or radiological recurrence with recovery of flap sensation, retained erectile function, and no herniation. We report this case due to rarity of giant abdominoscrotal arteriovenous malformations and present preoperative embolization, surgical resection, and functional anterolateral thigh flap reconstruction as a valuable treatment option of this life-threatening illness.
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Project description:Arteriovenous malformations (AVMs) are rare congenital vascular anomalies where rupture can be fatal. This report describes a case of a 69-year-old woman who presented with sudden severe lower back pain radiating to her left buttock and thigh and an inability to move her left leg. Computed tomography angiography (CTA) showed an extensive ruptured retroperitoneal AVM on the left paravertebral side with active bleeding and a large hematoma. Cardiopulmonary resuscitation with considerable blood transfusion was required. The AVM was effectively treated with coil embolization of the feeding lumbar arteries. Shortly after the intervention, the patient developed abdominal compartment syndrome. Urgent laparotomy with evacuation of the retroperitoneal hematoma was required. The patient was discharged 16 days after admission in a good physical condition and has been free of symptoms for more than a year after intervention. However, follow-up CTA showed residual contrast enhancement in the AVM and further surveillance is required.
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Project description:ImportanceMajor neurological complications from the embolization of vein of Galen malformations (VOGMs) are poorly understood. We provide a detailed analysis of contributors to periprocedural neurological complications and lessons learned.ObjectiveTo assess the rate of major periprocedural neurological complications following VOGM embolization with major procedural and strategic contributors.Design, setting, and participantsThis retrospective cohort study was conducted at a quarternary referral pediatric hospital (Hospital for Sick Children; Toronto, Ontario, Canada) from January 1999 to December 2018 with a mean clinical follow-up of 44.7 months; all children with VOGM diagnosed and/or treated were eligible (n = 48). Thirty-three patients who underwent endovascular treatment were included.InterventionsEndovascular staged transarterial embolization performed in 33 patients over 91 sessions.Main outcomes and measuresThe primary outcome was the rate of periprocedural neurological complications (occurring within 1 week of embolization). The secondary outcomes were mortality, long-term neurological outcomes, and contributing anatomical and management factors to neurological complications.ResultsOf 33 patients who underwent embolization (31 boys [64.6%]; 17 girls [35.4%]; median age at first embolization, 4 months [range, 0-29 months]), 10 patients (30.3%) developed major periprocedural neurological complications. Five of these patients died. Univariate logistic regression analyses identified internal cerebral vein drainage to the main venous sac of the VOGM and use of a microcatheter with a distal outer diameter of more than 2.0F as significant predictors of poor neurological outcomes. Lessons learned from our experience include the need to assess the internal cerebral vein drainage pattern on preprocedural magnetic resonance venography, avoidance of excessive embolization into the venous sac, treatment of more distal fistulae before proximal fistulae to avoid a sump effect, and preferably use of smaller (<2.0F outer diameter) microcatheters in neonatal embolization procedures.Conclusions and relevanceIn this cohort, 10 patients with VOGM treated with embolization (30.3%) experienced major periprocedural neurological complications, half of whom died. While these outcomes are superior to historic conservative and surgical treatment results, ongoing improvements in treatment and pretreatment diagnostic approaches are needed. Awareness of the lessons learned from our experience can help to avoid similar complications in the future for this vulnerable population.
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Project description:A term baby presented on the 7th day of life in cardiogenic shock due to vein of Galen aneurysmal malformation. A successful embolization of the malformation was performed through transarterial route on day 12 of life after a period of initial stabilization.
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Project description:ObjectiveDescribe the course and outcomes in a UK national cohort of neonates with vein of Galen malformation identified before 28 days of life.MethodsNeonates with angiographically confirmed vein of Galen malformation presenting to 1 of 2 UK treatment centers (2006-2016) were included; those surviving were invited to participate in neurocognitive assessment. Results in each domain were dichotomized into "good" and "poor" categories. Cross-sectional and angiographic brain imaging studies were systematically interrogated. Logistic regression was used to explore potential outcome predictors.ResultsOf 85 children with neonatal vein of Galen malformation, 51 had survived. Thirty-four participated in neurocognitive assessment. Outcomes were approximately evenly split between "good" and "poor" categories across all domains, namely, neurological status, general cognition, neuromotor skills, adaptive behavior, and emotional and behavioral development. Important predictors of poor cognitive outcome were initial Bicêtre score???12 and presence of brain injury, specifically white matter injury, on initial imaging; in multivariate analysis, only Bicêtre score???12 remained significant.InterpretationDespite modern supportive and endovascular treatment, more than one-third of unselected newborns with vein of Galen malformation did not survive. Outcome was good in around half of survivors. The importance of white matter injury suggests that abnormalities of venous as well as arterial circulation are important in the pathophysiology of brain injury. Ann Neurol 2018;84:547-555.
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Project description:We report the case of a 74-year old male who was evaluated for progressively enlarging right heart border on serial chest radiographs. Computed tomography of the chest revealed a pseudoaneurysm arising from the saphenous vein graft (SVG) to the posterior descending artery with mass effect on the right atrium. Coronary angiography showed severely compromised distal flow and an angiographically small territory at risk. Using a minimally invasive, catheter-based approach, an Amplatzer Vascular Plug II occlusion device was utilized successfully for embolizing the SVG pseudoaneurysm.
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Project description:BackgroundAbdominopelvic arteriovenous malformation is an uncommon congenital vascular lesion, for which the diagnosis and treatment are usually difficult. Though embolization and sclerotherapy are the primary treatment strategies, traditional surgical resection remains a valuable option.Case presentationHerein, we present a 32-year-old female diagnosed with a massive abdominopelvic arteriovenous malformation that originates from the splenic artery and drains into the portal vein. The vascular lesion was evaluated with multiple imaging modalities and then surgically resected successfully. The patient was discharged post-operatively on day 6 and free of symptoms during the 12-month follow-up.ConclusionTo our knowledge, the presented abdominopelvic arteriovenous malformation is the first to be reported in the literature, with such a rare condition originating from the splenic artery and draining into the portal vein.
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Project description:The patient was a 67-year-old man who had undergone coronary artery bypass graft surgery using a saphenous vein graft (SVG) 22 years before. Computed tomography angiogram revealed a large aneurysm of the SVG (38 × 42 mm in diameter; 80-mm long) and total occlusion of the left anterior descending artery (LAD). We first performed percutaneous coronary intervention for chronic total occlusion of the native LAD with bi-directional approach via the SVG. One month later, we performed the trans-catheter embolization of the SVG and occluded the SVG using multiple coils. This case demonstrates that trans-catheter embolization after recanalization of native coronary artery is an effective strategy to treat an SVG aneurysm. © 2015 Wiley Periodicals, Inc.
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Project description:Brain arteriovenous malformations (bAVMs) are an important cause of hemorrhagic stroke. The underlying mechanisms are not clear. No animal model for adult bAVM is available for mechanistic exploration. Patients with hereditary hemorrhagic telangiectasia type 2 (HHT2) with activin receptor-like kinase 1 (ALK1; ACVRL1) mutations have a higher incidence of bAVM than the general population. We tested the hypothesis that vascular endothelial growth factor (VEGF) stimulation with regional homozygous deletion of Alk1 induces severe dysplasia in the adult mouse brain, akin to human bAVM.Alk1(2f/2f) (exons 4-6 flanked by loxP sites) and wild-type (WT) mice (8-10 weeks old) were injected with adenoviral vector expressing Cre recombinase (Ad-Cre; 2 × 10(7) plaque forming units [PFU]) and adeno-associated viral vectors expressing VEGF (AAV-VEGF; 2 × 10(9) genome copies) into the basal ganglia. At 8 weeks, blood vessels were analyzed.Gross vascular irregularities were seen in Alk1(2f/2f) mouse brain injected with Ad-Cre and AAV-VEGF. The vessels were markedly enlarged with abnormal patterning resembling aspects of the human bAVM phenotype, displayed altered expression of the arterial and venous markers (EphB4 and Jagged-1), and showed evidence of arteriovenous shunting. Vascular irregularities were not seen in similarly treated WT mice.Our data indicate that postnatal, adult formation of the human disease, bAVM, is possible, and that both genetic mutation and angiogenic stimulation are necessary for lesion development. Our work not only provides a testable adult mouse bAVM model for the first time, but also suggests that specific medical therapy can be developed to slow bAVM growth and potentially stabilize the rupture-prone abnormal vasculature.
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