Maffucci syndrome

An 18-year-old man visited the Department of Plastic Surgery at our hospital complaining of functional and aesthetic problems with his hands caused by soft-tissue swelling. He particularly had problems using his left hand when working on the computer. He had developed multiple palpable nodular masses on both hands since the age of 2 years.

Image from (link)

Oblique radiographs of the hands obtained at the age of 18 years show the typical ring-and-arc appearance of chondroid lesions in the matrix of the osseous lesions. The well-defined round calcifications in the soft-tissue masses are typical of phleboliths. (from radiographics)

Maffucci syndrome was first described in 1881. This rare, nonhereditary syndrome is characterized by multiple enchondromas combined with hemangiomas and less commonly lymphangiomas.  Maffucci syndrome is sometimes confused with Ollier disease, which was described 19 years later and consists of multiple enchondromas without hemangiomas. (from radiographics)

Other similar posts
This entry was posted in Bones, Disease and tagged , , , .