Giant vein of Galen malformation in an adult.
Ontology highlight
ABSTRACT:
SUBMITTER: Kerolus MG
PROVIDER: S-EPMC5551915 | biostudies-other | 2017 Sep
REPOSITORIES: biostudies-other
Similar Datasets
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.
| S-EPMC7253270 | biostudies-literature
Project description: Not available
| S-EPMC7756164 | biostudies-literature
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.
| S-EPMC7119147 | biostudies-literature
Project description:Pulmonary arteriovenous malformations (PAVMs) may manifest with bleeding or embolic events necessitating intervention. Transcatheter coil embolization through the pulmonary artery (PA) is an established approach. We present a case of recurrent PAVMs despite numerous PA coil embolizations. PAVM occlusion was achieved through plug placement by a transseptal and pulmonary venous approach. (Level of Difficulty: Advanced.).
| S-EPMC8853956 | biostudies-literature
Project description:BackgroundAneurysmal dilatation of saphenous vein grafts used for coronary artery bypass grafting is a rare complication. These aneurysms are often large in calibre and pose a risk of rupture with significant haemorrhage.Case summaryWe describe a case whereby a large saphenous vein graft aneurysm is closed percutaneously using a vascular plug to cease flow and promote thrombosis of the aneurysm whilst reconstructing the occluded native artery to negate ischaemia.ConclusionSaphenous vein graft aneurysms following coronary artery bypass graft are rare and late complications. The preferred modality of closure is via percutaneous approach that requires meticulous planning to achieve a good outcome.
| S-EPMC10586192 | biostudies-literature
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.
| S-EPMC7186914 | biostudies-literature
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.
| S-EPMC6146857 | biostudies-other
Project description:Normal vascular development includes the formation and specification of arteries, veins, and intervening capillaries. Vein of Galen malformations (VOGMs) are among the most common and severe neonatal brain arterio-venous malformations, shunting arterial blood into the brain's deep venous system through aberrant direct connections. Exome sequencing of 55 VOGM probands, including 52 parent-offspring trios, revealed enrichment of rare damaging de novo mutations in chromatin modifier genes that play essential roles in brain and vascular development. Other VOGM probands harbored rare inherited damaging mutations in Ephrin signaling genes, including a genome-wide significant mutation burden in EPHB4. Inherited mutations showed incomplete penetrance and variable expressivity, with mutation carriers often exhibiting cutaneous vascular abnormalities, suggesting a two-hit mechanism. The identified mutations collectively account for ∼30% of studied VOGM cases. These findings provide insight into disease biology and may have clinical implications for risk assessment.
| S-EPMC10292091 | biostudies-literature
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.
| S-EPMC6221157 | biostudies-literature
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.
| S-EPMC3380964 | biostudies-literature