Maffucci's syndrome (dyschondroplasia with hemangiomas): a case with early osseous changes.

Abstract

MAFFUCCI, in 1881, 1 was the first to describe an entity characterized by dyschondroplasia and multiple hemagiomas. Since that time a total of 27 cases have been reported in the literature. 2 Other characteristic observations of this syndrome include the following: the disease is nonfamilial; the patient is usually normal at birth; involvement is unilateral or extremely asymmetric; there is uneven growth of the two sides; the disease is commoner in males; its onset is before puberty; there is a susceptibility of the patient to fractures, and phleboliths in hemangiomas are not uncommon. The dyschondroplasia is primarily in the form of defects in ossification, with one or more of the long bones having short shafts. The deformity varies according to the severity of involvement. On a review of the previous literature the incidence of chondrosarcoma was found to be approximately 20 per cent, particularly in those patients in whom enchondroma