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

Journal of Neurosurgery. Pediatrics
Walter J JermakowiczToba N Niazi

Abstract

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 g...Continue Reading

References

Jun 3, 2009·Journal of Neurosurgery. Pediatrics·R Webster CrowleyAaron S Dumont
May 13, 2011·Interventional Neuroradiology : Journal of Peritherapeutic Neuroradiology, Surgical Procedures and Related Neurosciences·S Aixut LorenzoJ M Macho Fernández

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Citations

Feb 3, 2018·Journal of Neurosurgery. Pediatrics·Thomas Sorenson, Giuseppe Lanzino
May 8, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Helen J ZhangCatherine A Mazzola
Mar 30, 2019·The Neuroradiology Journal·Guangming WangJinlu Yu

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