Title

Complex dural arteriovenous fistula in Bannayan-Riley-Ruvalcaba syndrome

Document Type

Article

Abstract

In this paper the authors report the case of a complex dural arteriovenous fistula (dAVF) with high-risk features in a 14-year-old girl with Bannayan-Riley-Ruvalcaba syndrome (BRRS), a phosphatase and tensin homolog-associated syndrome, presenting with signs and symptoms of increased intracranial pressure (ICP) that had previously been attributed to pseudotumor cerebri. This fistula was obliterated following 2 stages of embolization, and the patient experienced immediate symptomatic improvement. At the 2-month follow-up evaluation, the fistula remained angiographically occluded, and her symptoms continue to improve. This is the third reported case of an intracranial dAVF in a patient with BRRS. Because high-risk dAVFs can result in devastating morbidity, early detection with vascular imaging is crucial for patients with BRRS presenting with signs of increased ICP. Goals of treatment should include complete fistula obliteration whenever possible.

Medical Subject Headings

Adolescent; Angiography; Angiography, Digital Subtraction; Balloon Occlusion; Central Nervous System Vascular Malformations (complications, diagnostic imaging, therapy); Chemoembolization, Therapeutic (methods); Developmental Disabilities (etiology); Diagnosis, Differential; Drug Combinations; Embolization, Therapeutic (methods); Female; Genetic Testing; Hamartoma Syndrome, Multiple (complications, diagnosis, therapy); Humans; Magnetic Resonance Imaging; Megalencephaly (complications); Mutation; PTEN Phosphohydrolase (genetics); Polyvinyls (administration & dosage); Tantalum (administration & dosage)

Publication Date

7-1-2013

Publication Title

Journal of neurosurgery. Pediatrics

E-ISSN

1933-0715

Volume

12

Issue

1

First Page

87

Last Page

92

PubMed ID

23662932

Digital Object Identifier (DOI)

10.3171/2013.3.PEDS12551

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