Management Of Combined Lumbosacral-Pelvic And Lower-Extremity Anomalies
Virginia M. Badger, M.D. Warren G. Stamp, M.D.
The rare pelvic and spinal anomalies of agenesis of the ilium or agenesis or partial absence of the sacrum with partial absence of the lower lumbar spine present special problems in amputation surgery and prosthetic management. Typically, children with these anomalies also have such anomalies of the lower extremities as congenital clubfoot (talipes equinovarus), congenital webbing of joints (pterygium), knee flexion deformities, and/or incomplete rotation of the limbs, frequently associated with motor and sensory deficits from the hips distalward. Moreover, developmental failure of the spinal cord in its distal portion usually leaves the child without bowel or bladder control, even though the genitourinary system is intact otherwise. Areas of partial anesthesia about the buttocks and hips must also be inspected regularly for abrasion or redness caused by abnormal pressures.
Three patients in the Shriners Hospital, St. Louis Unit Amputee Clinic, present varying treatment challenges for lumbosacral-pelvic anomalies. Treatment of the condition has also been discussed by VanDerwerker in previous issues of the Inter-Clinic Information Bulletin1,2.
Two of the St. Louis patients were first reported by Stamp, Mahon, and Morgan in 1965(3). Their follow-up case histories reveal the following problems:
Case 1 (D.K.)
This boy, who was born March 29, 1953, has agenesis of the lumbar spine and sacrum (Fig. 1 ,Fig. 2 and 3 ). He has had bilateral subtrochanteric amputations because of severe flexion contractures of the hips and knees, and decubiti, which could not be healed, over the knees (Fig. 4 and 5 ,Fig. 6 ). Sensation was present only in the trochanteric areas. The boy wears a bucket-type prosthesis with bilateral Canadian-type hip joints and articulated knees (Fig. 7-9 ). His legs have been lengthened about 1 1/2 inches per year, the amount being determined by using the tenth percentile of the anthropometric chart developed by the Children's Medical Center, Boston. To assist the boy maintain his balance, total height has been limited to 5 feet.
He has had only one episode of skin breakdown over the right medial iliac region, which cleared following relief of his socket. He wears his prosthesis eight hours daily, uses a tripod gait, and is able to sit down onto a chair and get up by using his powerful upper extremities. His major problems have been prosthetic repairs and recurrent urinary tract infections.
Case 2 (R.G.)
Born on November 21, 1957, this boy also has an absent sacrum and lumbar spine segments with the same deformities as D.K. (Fig. 10 and 11 and Fig. 12 ). He had bilateral knee disarticulation amputations when he was four years of age. Clinical evaluation revealed that sensation in the knees was present only in spots. One month postoperatively he was fitted with a platform-type prosthesis, Canadian-type hip joints, and nonarticulated pylons (Fig. 13 and 14 ). These components he learned to control well, using a tripod gait. Six months later, his hip flexion contractures were surgically released, and three months following that procedure he was fitted with bilateral partial knee-bearing sockets, reverse-bail knee locks, hip locks, and a leather corset (Fig. 15-17 ).
One of his problems has been to obtain satisfactory control of hip abduction following correction of the contractures. The hip locks and corset have served to maintain correction as well as to provide him with additional hip stability in extension, since the hip extensor muscles are weak. Functionally, he too uses a tripod gait, and sits and stands without assistance. However, it was difficult to lock the bail locks after they were unlocked. Therefore, these locks have been changed to a custom-made device similar to the outside elbow lock-i.e., the boy pulls a cable to unlock the knee, but when the knee is extended it locks automatically (Fig. 18 ). He has had no problem with skin breakdown.
Case 3 (L.C.)
This girl, who is now six years of age, was born with four lumbar vertebrae, spina bifida, partial absence of the right sacrum, absent right ilium, and right phocomelia (Fig. 19 and 20 , Fig. 21 and 22 ). On the left lower extremity she had an intercalary paraxial tibial hemimelia. At age two an attempt was made to relocate the left fibula at the knee in order to preserve length of the extremity. Since the right phocomelic foot was nonfunctioning, it was amputated three months later (Fig. 21 and 22 ). On the same hospital admission, an attempt was made to relocate the left talus and calcaneus under the fibula. Following these procedures, the patient was fitted with a modified Canadian-type hip disarticulation prosthesis on the right and a long-leg brace on the left with limited knee and ankle motion.
Over the next two years, however, the left knee flexion contracture and foot deformity recurred, no function could be demonstrated in the left "knee," and problems with asensory skin led to a heel ulceration. In November 1966 a decision was reached to disarticulate the left knee. She has subsequently been fitted with a quadrilateral socket on the left and is now able to use crutches for short distances (Fig. 23 and 24 ). She needs an elastic strap between the sockets to prevent drift of the left leg into abduction. The major problem has been to achieve good socket fit on a chubby six-year-old. A "bikini-type" hip disarticulation socket has been found to be most satisfactory. Skin breakdown has not been a problem.
These patients represent complex problems in management. Important principles to observe in treatment are:
As much functional length of an extremity as possible should be saved, taking into account the prosthetic replacement necessary to obtain optimal functional gait.
Areas where the skin has sensation should be used for weight-bearing .
Surgical conversion of a deformed extremity will usually provide more efficient locomotion and better cosmetic acceptance.
To obtain maximum motor skill development, complete evaluation of sensory and motor function and early surgery followed by early fitting is advised.
In the described treatment of cases we gratefully acknowledge the assistance of Dr. George E. Scheer, who is the Chief Surgeon of the St. Louis Shriners Hospital Unit. Mr. Leo Tippy, certified orthotist, designed and constructed the prostheses illustrated in Fig. 13-14 and 23-24.
Virginia M. Badger, M.D. is the Associate Surgeon and Warren G. Stamp, M.D. is the Assistant Chief Surgeon at the Amputee Clinic Shriners Hospital for Crippled Children St. Louis, Missouri
1. VanDerwerker, Earl E., Jr., "Iliolumbar Fusion in the Management of Sacral Agenesis-A Preliminary Report," ICIB, May 1966.
2. VanDerwerker, Earl E., Jr., "Iliolumbar Fusion in the Management of Sacral Agenesis-A Follow-up Report," ICIB, October 1967.
3. Stamp, Warren G., Mahon, Sharon, and Morgan, Harry C., "Problems of Management of the Child With Multiple Amputations," Arch. Phys. Med, and Rehab., 46:354, 1965.