Massive craniofacial cystic angiomatosis and extra-skeletal angiomatosis: A unique case report and brief review of literature

Abstract

Cystic angiomatosis is a rare vascular disorder, characterized by multifocal hemangiomatous and/or lymphangiomatous lesions of the skeleton with possible visceral organ involvement. The aim of the present communication is to report a unique case of massive craniofacial skeletal cystic angiomatosis in a 15-year-old boy with history of progressive enlargement of all craniofacial bones since birth associated with severe anemia secondary to splenomegaly. The computed tomography and magnetic resonance imaging techniques revealed hypertrophy of the craniofacial skeleton secondary to multiple cystic lesions and also disclosed cystic lesions in cervical, lumbar, sacral vertebrae and the spleen. Histologically, it was analogous to cavernous hemangioma. This was an unusual case of classical bilateral, craniofacial skeletal angiomatosis; till date a total of three cases have been reported but the present case was diverse from the reported ones.