Orbital Varix Thrombosis and Review of Orbital Vascular Anomalies in Blue Rubber Bleb Nevus Syndrome

Pari N. Shams, Sudha Cugati, Tim Wells, Shyamala Huilgol, Dinesh Selva

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

A 57-year-old woman with cutaneous manifestations of (BRBNS) presented with acute left proptosis and venous congestion secondary to thrombosis within a superior ophthalmic vein varix. Multiple phleboliths were noted in the contralateral right orbit, and an incidental right middle cerebral artery aneurysm. Her symptoms spontaneously resolved within a few days. An English literature review found 7 reported cases of orbital vascular lesions in association with BRBNS from 1950 to 2012. All lesions showed contrast enhancement on CT or MRI: 4 had small orbital calcifications and 3 were distensible with raised venous pressure. The occurrence of a thrombosed orbital varix or cerebral artery aneurysm in BRBNS, to the best of the authors' knowledge, has not been previously reported. Although orbital vascular lesions in BRBNS have been described as hemangiomas, the biologic behavior and histology of most of the reported orbital lesions are most compatible with venous malformations.

Original languageEnglish
Pages (from-to)e82-e86
Number of pages5
JournalOphthalmic Plastic and Reconstructive Surgery
Volume31
Issue number4
DOIs
Publication statusPublished - Jul 2015
Externally publishedYes

Keywords

  • BRBNS

Fingerprint Dive into the research topics of 'Orbital Varix Thrombosis and Review of Orbital Vascular Anomalies in Blue Rubber Bleb Nevus Syndrome'. Together they form a unique fingerprint.

  • Cite this