Maffucci's Syndrome: A Case Report

Lee, Duk Yong; Chung, Moon Sang; Lee, Soo Yong

Abstract

Maffuccis syndrome was first reported by Maffucci, an Italian, in 1881. It is manifested by unilateral multiple hemangiomas and enchondromas. It is congenital but is not hereditary. We encountered a case of this rare syndrome, with which bleeding tendency was combined. All who have multiple hemangioma on his unilateral side of the body should be checked with bone X-rays to rule out this syndrome. This syndrome is noted for transformation to malignant tumors. When there is pain or enlargenent of the tumor without any significant trauma, biopsy is in order. There can develop bleeding tendency due to microangiopathic coagulopathy which is associated with throm-bocytopenia and hypofibrinogenemia.