Kasabach-Merritt Syndrome and Left Trans-Femoral Amputation
DR. DAVID BERBRAYER, M.D., AND MR. LINH LE, C.P.O.(c)
Asha has Kasabach-Merritt syndrome with a large cavernous hemangioma of her left thigh since she was born.
In March 1976, she fell and sustained a pathological fracture of the left femur. She sustained a second fracture in October 1976 after having a fall. During this interim period, she was prescribed with a knee ankle foot orthosis. The fracture healed reasonably well again.
In 1977, she had an arteriogram performed which revealed the presence of a large hemangioma extending from the perineum right down to the left knee joint. In October 1978, she sustained a third fracture which associated with narrowing of the femur. This fracture did not heal despite immobilization, intramedullary nailing and bone graft. She was left with significant leg length discrepancy of approximately 14 cm. As a result of these complications, her left knee became ankylosed and she had difficulty with ambulation. During this period, she was fitted with above knee extension prosthesis, with quadralateral socket, without knee joint and OTTO BOCK SACH foot and silesian belt suspension.
On March 31, 1982, a knee disarticulation amputation was recommended to improve her gait. On May 10, 1982, she was seen in Prosthetic Clinic and ready to be casted for the new prosthesis. Her new one was fabricated with a definitive above knee prosthesis; quadrilateral molded socket with anterior opening flexible wall and velcro straps closures in order to accommodate the changing shape of her stump, silesian belt suspension, constant friction knee joint and Kingsley sculptured toes SACH foot and finished endoskeletal modular system. She tended to walk with her left hip in internal rotation, so a pelvic joint and pelvic band were added in March 1983. She appeared to be ambulating quite well but from time to time she has had a problem with pain in her stump.
In May 1986, she was casted for the new prosthesis due to growth and also provided a night resting socket as well. Everything seemed fine until the end of June 1987, when she was aggravated by the development of persistent pain over the lateral aspect of her stump.
In both medical and prosthetic attempts were made to alleviate the pain in her stump. On June 1988, Asha was recasted and was fitted with an experimental above knee prosthesis with molded CAT-CAM socket, single axis constant friction knee joint, Kingsley sculptured toes SACH foot and silesian belt suspension and finished endoskeletal modular system with light weight foam fairing. At the same time she also has a night resting socket. It is possible that the hemangioma may be undergoing spontaneous atrophy and since wearing CAT-CAM socket she has had no further pain in her stump. However, the pain is present without the prosthesis on. Therefore the prosthesis is substituted by a night resting splint when she retires to bed.
Hugh MacMillan Rehabilitation Centre, 350 Rumsey Road, Toronto, Ontario, Canada M4G 1R8