The Maffucci syndrome.
Maffucci syndrome consists of subcutaneous hemangiomata and multiple enchondromata. The incidence of malignancy associated with the disease is 23%, of which 15.2% is a malignant transformation of enchondromata to chondrosarcoma. Periodic follow-up of the patient is advised. When malignant transformation is suspected, a bone scan may be useful for diagnosis, especially when done sequentially. Ninety-four percent of the patients can live a reasonably normal life, although two-thirds of them require some surgical intervention.
Ben-Itzhak
I
I
Department of Orthopaedic Surgery, University of the Witwatersrand, Johannesburg, South Africa.
Denolf
F A
FA
Versfeld
G A
GA
Noll
B J
BJ
D002648:Child; D003937:Diagnosis, Differential; D004687:Enchondromatosis / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis; D010009:Osteochondrodysplasias / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging; D011859:Radiography; D012008:Recurrence