Weinstein Lovell and Winters Pediatric Orthopaedics 7e

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CHAPTER 30  |  The Child with a Limb Deficiency

Children with bilateral amelia often walk late. They will need help in pushing to stand. In addition, the fear of falling, because they cannot protect themselves, comes early. Helmets or some protective headgear are needed until the child is indepen- dent in gait. Therapy concentrates on trunk control and strength, along with training in the use of their feet for all activities. These children are also prone to develop a progressive scoliosis, often before adolescence. This presents a difficult problem. Treatment of the scoliosis, either by bracing or by surgical fusion, restricts the use of their feet in ADL. However, often these curves will be progressive, and in such cases, a selective fusion is preferable. Phocomelia.  In phocomelia, the distal portion of the extremity appears to attach directly to the body (Fig. 30-37). Although typically described as an intercalary deficiency, a recent study suggests that the limb almost always exhibits a longitudinal deficiency proximal and distal to the intercalary defect (Goldfarb et al.). There are wide variations in the severity of this deficiency. In some, the hands may be close to normal, with some remnants of the arm bones, whereas in others the hand may be no more than a single functionless digit with near complete absence of the arm. Patients with phocomelia usu- ally have some mobility in their residual limbs and therefore differ in one significant way from the child with bilateral ame- lia—they have a sensate limb often capable of some grasping function. The function of these limbs depends on the function

of the hand, the length and function of the arm itself, and the ability to bring the hands together within the field of vision. As mentioned previously, those children who cannot bring their hands to the midline or to the mouth will use their feet to substitute in the ADL (Fig. 30-38A–D). They should be encouraged from an early age to develop their foot skills and the body strength that is necessary to use the feet. In those children with longer residual limbs and better function, little treatment other than providing adaptive equipment to aid in dressing and so on may be necessary (Fig. 30-38E). Therapy to increase the range of motion of the scapula and limbs and to strengthen any muscle power in the residual digits may be beneficial. Adaptive equipment can be very use- ful for some activities such as feeding, dressing, and so on. The residual limbs can manipulate switches for powered prostheses, giving rise to the temptation to find a prosthetic solution to their problem. However, like the child with bilateral amelia, these children will function in most activities by substitut- ing foot function for what they cannot do with their upper extremities. Fitting the older child with a unilateral prosthesis for a specific function may be indicated, but routine wear is not common. Transverse Complete Humeral Deficiency.  There is little published experience in this deficiency. Although some children with unilateral above-elbow amputation will develop surprising facility with a prosthesis, most will often wear it only for specific activities, such as sports, or for cosmetic reasons in social situations. In children with a long-enough humeral seg- ment, the humeral–thoracic pinch provides useful assistance for the normal opposite extremity. Prosthetic use in these chil- dren generally relates to specific tasks. These may be as limited as for riding a bicycle or full-time use at school. An attempt at prosthetic fitting is warranted. Patients with bilateral congenital transhumeral deficien- cies are more inclined to use their own body, rather than a prosthesis. These children may benefit more from assistive devices than from prostheses. Such patients will often prefer prosthetic fitting on one side, where they use the humeral– thoracic pinch, with intact sensory feedback on the other side and in their feet. For the patient with bilateral above-elbow transhumeral deficiencies, Marquardt has recommended an angulation oste- otomy of the humerus (197). The osteotomy angles the dis- tal 3 to 5 cm of the humerus anteriorly by 70 to 90 degrees. This allows for suspension of the prosthesis without the usual shoulder cap, makes it easier to put on, permits better shoulder motion, and gives better control of rotation. This procedure should only be performed if the humeral length is sufficient and there is a need for a unilateral prosthesis. Transverse Complete Forearm Deficiency (Congenital Below-elbow Amputation).  The congenital below-elbow amputation is the most common of all of the upper extremity deficiencies. It is more often the left arm (Fig. 30-39). It is sporadic and without known cause.

FIGURE 30-37.  Clinical photograph of a patient with bilateral upper extremity phocomelia, with the hands seemingly arising directly at the level of the shoulder joint. Patients with phocomelia often have the ability to use their residual limbs, but in cases like this they rely on their lower extremities for ADLs.