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May 1996

Presumed Orbital Hemangioma Associated With the Blue Rubber Bleb Nevus Syndrome

Author Affiliations

New York, NY

Arch Ophthalmol. 1996;114(5):618-619. doi:10.1001/archopht.1996.01100130610024

In 1958, Bean described the association of multiple cutaneous and enteric bluish hemangiomas with enteric bleeding.12 It has subsequently been called the blue rubber bleb nevus syndrome. He noted inheritance in an autosomal dominant pattern in several families. Bean described 3 forms of bluish cutaneous hemangiomas: large disfiguring cavernous angiomata, compressible blood sacs that resemble blue rubber nipples, and irregular blue punctate spots. Two to hundreds of hemangiomas may be seen, and they generally increase in size and number with age. Their size varies from 1 mm to several centimeters. Lesions can be partly or completely emptied on compression and refill slowly. In addition to cutaneous involvement, hemangiomas can be found in the gastrointestinal tract from the oral to the anal mucosa,3 and they may cause chronic occult blood loss or acute hemorrhage. Ophthalmologic involvement has been described in 2 previous cases; in 1 there was an orbital

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