Surgical And Prosthetic Management Of Three Congenital Child Amputees
John E. Hall, F.R.C.S.(C) William F. Sauter, C.P.O.
In treating children with severe congenital limb deficiencies at the Ontario Crippled Children's Centre, our close association with the Prosthetic Research and Training Unit has been most helpful. Advanced experimental devices developed by Mr. Colin McLaurin and his staff have been readily available to us in dealing with our most difficult cases, sometimes in association with surgery, sometimes not. Three cases are presented to illustrate this close working relationship.
Case 1 (S.W.)
This child was born January 3, 1963, with phocomelia of both upper extremities. She was first seen at the Ontario Crippled Children's Centre in July 1963 at the age of six months, at which time it was found that she had good sitting balance and appeared to be in good health, aside from her upper-extremity defects.
Her left arm was shorter than her right. On the left side there was no obvious bony nucleus that represented the humerus, but there did seem to be an ulna with three finger rays, two of them functional. On the right side there was a rudimentary humerus and ulna and four fingers, of which the ulnar three were functional.
Function Without Prosthesis
Prior to the fitting of a prosthesis, the child appeared to have little interest in self-help skills. The use of her feet was strongly encouraged, but with little response, and she rarely used compensatory patterns-mouth, knees, etc.--to compensate for lack of grasp. She attacked activities almost exclusively with her right hand, using a spherical type grasp, and only started to use her left hand when its utilization was required to activate the switches on her prosthesis. She was completely dependent for feeding, although there was some attempt to finger feed.
Socially, the child was somewhat passive and lacked initiative. She was content to sit and play with objects handed to her rather than take an active interest in the environment.
When she was two years of age (Fig. 1 and 2 ), the child was fitted with a wrist-elbow coordinated arm, which was designed specifically to aid independent feeding (Fig. 1 and 2 ). The arm consisted of a powered elbow unit which was linked to a wrist component in a parallelogram fashion, so that a spoon could be raised from the table without tipping. The control was a simple rocker switch placed within reach of the existing fingers on the left side. A Dorrance hook could be opened by pulling a cable with the teeth.
The prosthesis was satisfactory for such foods as mashed potatoes and ice cream, and the child was able to use the arm for holding cookies, sandwiches, etc. In addition to the prosthesis, the child required special equipment for feeding. This included a dish with vertical sides, a bent spoon, and a waist-high table. Thus feeding away from the home was difficult.
The prosthesis proved very useful for play purposes, as the child was able to use it in opposition to her right hand when holding large objects, and for such tasks as carrying a shopping bag, pulling a wagon, etc.
The arm proved to be extremely rugged, with little or no servicing required other than routine battery charging. The child adapted quickly to the device and wore it routinely, but was hampered by the lack of an activated terminal device.
At three and a half years of age, the child was fitted with her present prosthesis (Fig. 3 ). This is an electrically powered coordinated arm with a three-fingered terminal device. The arm has a powered shoulder joint connected mechanically to the elbow joint, and moves in a natural, sweeping motion from the mouth to table level, and thence down to the side and around behind the back. This motion is powered by a motor in the shoulder; a second motor in the forearm powers the terminal device. The motors are controlled by two switches-a rocker type to move the arm and a press switch to open the fingers.
The child quickly accepted the arm and wears it during all her waking hours. She is now completely independent for feeding, provided the food is chopped up for her, although she still requires a certain amount of special equipment. She uses the arm spontaneously in all types of play and for increased reach, stabilization, and body support.
The prosthesis has significantly improved the child's ability to cope with everyday activities. Although she still uses her right hand predominantly, she is now adept at such bimanual tasks as drying dishes, stringing beads, etc., and she has responded to the interest in her performance by becoming increasingly outgoing and adventurous .
The arm has proved to be extremely rugged, and occasional wire breakages have been the only maintenance problems .
Case 2 (R.C.)
This child was born in May 1962, with very severe quadrilateral phocomelia. His deformities were the result of thalidomide ingestion during his mother's pregnancy. When he was first seen in November 1962 he was making rather poor progress, having a tendency toward repeated chest infection and high fevers. He was unable to sit up or roll over. His legs were rudimentary, with eight-toed feet, but had no real power in them. His arms had rather short proximal segments, but he was able to bring them together in front of his chest. Associated anomalies present were bilateral inguinal hernia, which required repair, and a congenital heart defect which has not proved to be a problem to date (Fig. 4 ). In 1964 the child had an operation on his eyes for divergence strabismus, and in September 1965 he underwent surgery for ptosis of his eyelids, which considerably improved his appearance.
In December 1962 the child was provided with a bucket for sitting purposes, and this also improved his chest problem. He quickly adapted to the bucket, and in July 1963 it was mounted onto a cart to make him a little more mobile. In September of that year it was attached to short legs terminating in fore and aft rockers (Fig. 5 ). At this time he was taught to use a series of devices, including a rocking board, bouncing table, and lastly a side-to-side rocker and a saucer to help develop his balance (Fig. 6 ). He was finally fitted with a device known as a wobble walker. Early in 1965 the child was able to use the wobble walker with reasonable proficiency, and three months later he was fitted with a device known as a swivel walker (Fig. 7 and 8 ). In October 1966 he was fitted with his present device, which is a more sophisticated version of the swivel walker, using articulated legs (Fig. 9 and 10 ).
When using the swivel walker, the child progresses by transferring his weight to one foot by leaning sideways, and then swivels forward about this foot, using the force of gravity. Stops are provided to limit forward or backward swing, while springs return the foot to the neutral position when it has been raised clear of the floor by leaning in the opposite direction. The socket of this appliance is mounted on a platform which is attached to two legs with conventional hip and knee joints, and terminates in a swivel ankle joint attached to the foot piece.
The child quickly adapted to his swivel walker, and uses it all his waking hours. He is able to walk at about 30 feet per minute on floors and walks, manages shallow ramps slowly and under supervision, and can step up small steps of about one inch. Walking on grass or other uneven terrain has not been possible, nor is it possible for him to rise or sit down without assistance. This makes a powered wheelchair unsuitable, but some thought has been given to a battery-powered scooter with a self-raising platform to enable him to proceed at a normal speed in school, on sidewalks, and in other public places.
Although initially the child's feet seemed useless, they were left alone and have since developed considerably in size and strength, and he now uses his right foot to hold his penis for urination.
Prior to his fitting with the swivel walker, the child appeared quite retarded because of his slowness of response, but once he became upright and started to move around, his interest in the environment increased, and his reactions became more normal. He now likes to be independent, and becomes easily frustrated when he attempts tasks beyond his capabilities.
The child was not provided with any prostheses for his upper extremities because it was felt that his function could not be materially improved. He is able to make his hands touch in front of his body, and reach his mouth for feeding purposes. Although he has limited grasp, and primarily uses a lateral pinch between the two ulnar fingers, he is capable of grasping onto a bar, holding a golf club, etc. However, his bimanual hand skills are below normal for his age level.
Case 3 (K.B.)
This child was born in January 1962 with quadrilateral phocomelia as a result of thalidomide ingestion by her mother during pregnancy. Her lower extremities were rather short and were folded across her abdomen. However, they contained good segments of fibula, and she had reasonably normal feet, with five extra toes. Her upper extremities had a good humeral segment, an absent radial ray, with severe clubbing at the wrist, and four reasonably functional digits (Fig. 11 and 12 and Fig. 13 ).
K.B. was first seen at the Ontario Crippled Children's Centre in May 1962, and her programme was envisaged as a combination of surgery and prosthetic fitting. After a series of corrective plasters, an operation consisting of a dorsal approach to the area of the wrist joint was performed on her left arm in September 1962. The lunate and capitate bones were removed, and the ulna was transferred into the defect that was created. Since the ulna was too large for this defect, the intermetacarpal ligament between the two middle rays was split to provide more room, rather than trimming down the ulnar head, since this would have prejudiced its blood supply. A pin was used to hold position, and this pin was removed at the end of three months.
In November 1963 an operation similar to the one performed on the left arm was performed on the right. At this time good function had developed on the left side, with a reasonably normal-looking arm segment. A week after the operation on her right arm, a release procedure was done on both legs, to get them away from their position of being folded across the lower part of the abdomen. The procedure involved release of the soft tissues posteriorly and excision of the talus and navicular of both feet, putting the remnant of the carpus in position at the lower end of the fibula. The purpose of this procedure was to get the legs pointing downward, so that they could be used as attachments for prostheses (Fig. 14 ).
In April 1965 an operation was performed on the second finger of her right hand, which had a rather marked contracture of the proximal interphalangeal joint. This finger was explored through a lateral incision, and the tightest structure was found to be the flexor sheath, as well as the capsule of the proximal interphalangeal joint. These structures were divided, the finger was splinted out straight for two weeks, then active exercises were begun, supplemented by a small spring-loaded night splint. This operation resulted in straightening of the finger, with ability to flex it to 90 degrees, and got it out of the way of her palm, where it had been interfering with her grasp. In January 1966 eye surgery was performed to correct a strabismus.
In April 1964 the child was fitted with plaster prostheses with short legs (Fig. 15 ), which were gradually raised in height until by August 1964 she was up to nearly normal height, with plastic laminated prostheses without knee joints (Fig. 16 ). However, she did not walk well, and the leg length severely hindered her ability to get up from the floor. When new prostheses were required (due to growth), short temporary devices were again employed. By August 1965 she was again up to nearly normal height using similar prostheses. During 1966 the child gradually increased her proficiency in walking and was raised 2 inches to put her on a comparable height with her classmates.
In October 1966 she was fitted with prostheses with knee joints, which she is still wearing (Fig. 17 ). The knee joints are secured with elastic webbing, so that they remain fully extended while walking, but allow sitting. Ankle rotators which allowed about 10 degrees rotation about a vertical axis were fitted, and this rotation appears to have been of assistance in walking. Her walking ability is now quite satisfactory. At some future date it is hoped to fit her with knee joints that will provide stability at heel contact while allowing normal flexion during swing phase.
The basic socket pattern has changed little since the first fitting. Most of the weight has been taken in the ischial area, and the legs have been held in extension while making the plaster wrap. The anterior distal part of the socket has been left open for easy entry, and suspension has been achieved by padded Velcro straps over the proximal dorsum of the foot.
In the early fittings, the socket was extended upward posterior to the ischial seat to help maintain an upright posture. This extension later became unnecessary.
A typical socket pattern is shown in Fig. 18 . The most recent sockets have been fabricated of Polysar X414 thermoplastic sheet. With this material minor modifications can be readily carried out and a more comfortable fit achieved.
John E. Hall, F.R.C.S.(C) and William F. Sauter, C.P.O. are associated with the Ontario Crippled Children's Centre Toronto, Canada