ENCHONDROMATOSIS WITH FEATURES OF DYSSPONDYLOENCHONDROMATOSIS AND MAFFUCCI-SYNDROME

We report a girl,vith multiple enchondromatosis, unequal leg length, s hort stature, congenital scoliosis, lymphangioma, and cutaneous hemang iomata. The skeletal findings were consistent with the clinical and ra diological features of dysspondyloenchondromatosis except that short s tature was not apparent in the neonatal period. Dysspondyloenchondroma tosis is a rare disorder, one of the several types of multiple enchond romatosis with spinal abnormalities. In previous reports of this condi tion the association of vascular lesions usually found in Maffucci syn drome has not been described.