Weinstein Lovell and Winters Pediatric Orthopaedics 7e

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CHAPTER 30  |  The Child with a Limb Deficiency

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B C FIGURE 30-39. A : A typical patient with a congenital below-elbow amputation. There is usually enough length to fit a prosthetic arm and still permit good active elbow motion. B,C : Two different children with transverse incomplete forearm deficiency fitted with a myoelectric-powered hand performing common functions of childhood. Many of the children who use the prosthesis develop amazing skills in its use.

­fitted after, had stopped using their prosthesis. However, of those who continued to use their prosthesis, there was no dif- ference in the amount of use between the two groups. The most common age at which patients discontinued use of their prosthesis was at 13 years, most commonly because the pros- thesis was viewed as cosmetically unacceptable and function- ally superfluous. Sorbye (200) reported that, of the patients in their clinic who were younger than 24 years, 87% were using their myoelectric prosthesis, and 65% of these used it all day and for all activities. Hubbard et al. (201), reporting on the Toronto experience, found that 70% of the below- elbow amputees were using their prosthesis, whereas 30% had rejected it. With this and other evidence, it is now the usual practice to recommend fitting around the age of 4 to 6 months with a pas- sive hand to aid in normal development. This lightweight pros- thesis helps the child become comfortable with a prosthesis and acquaints them with the two-handed activities that a normal child would perform. The hope is that the child will develop the central cortical pathways necessary for bimanual dexterity. Depending on the child’s acceptance and use of this pas- sive prosthesis, a more functional terminal device is fit between 15 and 18 months (31, 202). Today, there are a number of ter- minal devices available (203). There are two choices to power the device: battery (myoelectric) and body (cables). Although there will be many factors to consider in the selection (cost and funding, clinic philosophy, and parent choice), virtually all centers today in North America are fitting most children with myoelectric powered terminal devices (Fig. 30-39). Table 30.4 compares the advantages and disadvantages of myoelectric and

body-powered terminal devices for the child with a congenital below-elbow amputation. Surgical Treatment Recommendations.  One surgical option in the treatment of patients with bilateral transverse forearm deficiency is the Krukenberg operation. This opera- tion separates the radius and ulna to create a forearm capable of pinch and grasp between sensate ends (Fig. 30-40). This was invented in 1916 to treat World War I upper extrem- ity traumatic limb deficiency patients, and there are several favorable reports of function after this surgery for traumatic amputation in the literature (204–207). The procedure has also been proposed for patients with congenital transverse forearm amputation with similar good results (208, 209). This procedure has been accepted in third-world countries for both congenital and traumatic bilateral upper extremity amputations. In the Western world, concerns over the cos- metic appearance of the arm after surgery have limited its use, much like the Van Nes rotationplasty. Current surgical indi- cations are limited to the blind bilateral upper extremity limb deficiency patient. The advantages of the Krukenberg procedure are that the child gains sensory feedback with pincer function between the distal radius and ulna, which cannot occur with a prosthesis. Moreover, the operation does not preclude prosthetic fitting. At the author’s institution, the experience with the Krukenberg procedure is that patient’s Krukenberg limb becomes the domi- nant functioning extremity. Patients occasionally will choose to wear a passive hand cosmetic prosthesis in certain social situ- ations over their Krukenberg limb. Complications including