Sacral Agenesis Prosthetic and Physical Therapy Management of a Patient with Sacral Agenesis


Treatment of patients with sacral agenesis can be very difficult because of the neural involvement that occurs to varying degrees in the lower limbs. Frequently, surgical ablation of a portion of the lower limbs is accomplished at a very young age. Contractures often aggravate problems with respect to prosthetic fitting and eventual ambulation of the patient. Fixed orthopedic deformity may preclude fitting. A possible solution to widely abducted hips with fixed deformity is presented and the prosthetic techniques are described.

Case Study

J.J. is a 9-year-old girl who has a diagnosis of multiple congenital anomalies and lumbosacral agenesis. Elective above-knee amputation on the basis of deformity of the lower limbs was accomplished at the age of 6 (Fig. 1 ). The patient has bladder and urinary problems secondary to lumbosacral agenesis. She also has significant muscle weakness in her trunks and hips.

The patient was initially seen in our facility at age 9 (she had been referred from another facility due to inability to walk and inability to perform activities of daily living). The initial physical therapy evaluation showed she had inadequate prostheses for walking. She was unable to perform a consistent "swing-through" gait. She had widely abducted hips because of weakness in the hip adductors and extensors and the "natural" abducted position of the hip joints due to the sacral agenesis. She lacked the ability to move from a chair to standing and lacked gait independence. She was started on trunk balance skills. Once she progressed to the point of some independent balance, it was felt that the prostheses could be ordered. The prostheses were to be made with the legs maintained in the abducted position. The knees were aligned in a somewhat wide base with manual knee locks. A pelvic band was incorporated into the prostheses (Fig. 2 ). Prior to completion of the prostheses, the patient learned to rise from a sitting to a standing position and return independently. She can lock and unlock the knee joints and don and doff the prostheses. She is able to use the prostheses for social occasions and walk independently with fair endurance with a roller walker. She is beginning training in community ambulation, including inclines, and now walks independently in school with stubbies and short crutches.

The importance of the prostheses is that they do not attempt to force the legs into a neutral position. The sockets were fabricated to allow for the abduction which the patient presented and the knee centers were placed below with locks on both sides.

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