Developmental venous anomalies and sinus pericranii in the blue rubber-bleb nevus syndrome. Case report

Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous syst...

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Bibliographic Details
Published inJournal of neurosurgery Vol. 99; no. 2; p. 409
Main Authors Gabikian, Patrik, Clatterbuck, Richard E, Gailloud, Philippe, Rigamonti, Danielle
Format Journal Article
LanguageEnglish
Published United States 01.08.2003
Subjects
Cerebral Angiography
Female
Hemangioma, Cavernous - diagnostic imaging
Hemangioma, Cavernous - pathology
Humans
Magnetic Resonance Imaging
Middle Aged
Nevus, Blue - diagnostic imaging
Nevus, Blue - pathology
Skin Neoplasms - diagnostic imaging
Skin Neoplasms - pathology
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Summary:Blue rubber-bleb nevus syndrome (BRBNS) is a developmental disorder that originally was identified by the presence of distinctive cutaneous and gastrointestinal hemangiomas. More recently it has been recognized that the number of affected organs is larger and that BRBNS includes central nervous system vascular malformations. A 52-year-old woman in whom intracranial vascular malformations had been diagnosed earlier presented for evaluation. At birth, several blue nevi had been noted on her tongue, lips, and neck. Cerebral angiography demonstrated an extensive network of developmental venous anomalies and a left anterior sinus pericranii. The literature on BRBNS and developmental venous anomalies is reviewed.
ISSN:0022-3085
DOI:10.3171/jns.2003.99.2.0409