Bannayan-Riley-Ruvalcaba syndrome

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by hamartomatous intestinal polyposis, lipomas, macrocephaly and genital lentiginosis.

Increased intracranial pressure

An increase of the pressure inside the cranium (skull) and thereby in the brain tissue and cerebrospinal fluid.


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PMID (PMCID)
23662932
FEMALE
Complex dural arteriovenous fistula in Bannayan-Riley-Ruvalcaba syndrome.
Moon K, Ducruet AF, Crowley RW, Klas K, Bristol R, Albuquerque FC.
J Neurosurg Pediatr. 2013;12(1):87-92.
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.