MAFFUCCI SYNDROME, UNILATERAL LOWER LIMB INVOLVEMENT WITH GROSS SHORTENING: A CASE REPORT

Maffuci syndrome is a rare type of multiple enchondromatosis associated with hemangiomas in soft tissues. The presence of hemangiomas and a very high risk of malignant transformation of enchondromas to chondrosarcomas differentiates Maffucci syndrome from Olliers disease.We report a case of9 years old girl child with multiple enchondromas and hemangiomas. She had shortening and deformity of left lower limb, fracture of left tibia following a trivial trauma, and a history of surgical interventions to correct valgus deformity at the knee by figure of 8 plating and nailing and bone grafting for fracture tibia that failed to unite. X-rays were suggestive of multiple enchondromas affecting the bones unilaterally, of only the left lower limbs with multiple lesions in the left femur, tibia and fibula. The right lower limb was normal. The resultant shortening was 6.5 inches (16.5 centimeters.).There was, in addition, a painful, warty growth in the lower third of the posterior aspect of the left thigh measuring 1.5 cm x 1.5 cm.An excisional biopsy was done and the histopathology report was a Hemangioma. The child was diagnosed as a case of Maffucci syndrome. The child was fitted with a KAFO with an extension prosthesis (a double decker).