Therapy in unilateral sirenomelia: report of one case.

Abstract

A 17-month-old male infant was referred to us with a unilateral sirenoid malformation in 1970. Besides urogenital and lumbosacral vertebral defects, the right knee was flexed 160 degrees, and the hip externally rotated and abducted. The thigh and calf were connected by a soft tissue bridge with the heel fixed to the ischial region. Arteriography showed that the foot and lower leg were supplied by the profunda femoris artery through the soft tissue bridge. The lower leg had only a very small supply from the popliteal artery. Therefore, an amputation by disarticulation at the knee joint was performed. A few months later, in addition, the hip flexion contracture was released. The boy was taught to walk with a prosthesis. At the age of 31 years, he has a sedentary job and is more troubled by his urogenital than his orthopaedic defects. He prefers to walk with crutches and for a few years has no longer used his prosthesis.