Treatment of Congenital Differencesof the Upper Extremity:

Therapist's Commentary

Mia Fuller, MS, OTR, CHTSite CoordinatorHealthSouth Atlanta HandAtlanta, Georgia

R ehabili tation of the patient with a congen­ital upper extremity disorder creates many

challenges and rewards for the hand therapist. In­tervention not only includes the infant or child withthe condition but must also address the emotionsof the family. The major goals of hand therapy inthe treatment of congenital anomalies include op­timizing reach-and-grasp functions, enhancing theappearance of the upper extremity following sur­gical intervention, maximizing independence indaily living activities, including play, and assistingthe family and child in overcoming social and emo­tional obstacles.

Therapeutic intervention for the treatment ofupper extremity congenital disorders can be em­ployed pre- and postoperatively.l" Evaluation in­cludes a thorough medical history and detailsabout range of motion, strength, and functionalhand use related to age, sensibility, and scar orwound status, as applicable. Splint fabrication isemployed pre- and postoperatively to assist in thecorrection of contractures as well as maintain sur­gical gains. Splinting a pediatric patient requiresspecialized skill and training. Manipulating tiny ex­tremities to specified angles and ensuring proper fitare common challenges faced by the hand thera­pist. Low-temperature plastics, such as Aquaplast(Smith & Nephew), allow the splinting material tobe pinched around the extremity, freeing the ther­apist's hands to position the joints as needed. Thismaterial also comes in a multitude of colors thatare exceptionally pleasing to children. The use ofDycem (Smith & Nephew) to line the inside ofsplints greatly assists in preventing slippage. Thematerial is easily removed and replaced for hygieneand is relatively inexpensive. Velcro straps aloneare rarely sufficient to maintain a splint on the ex­tremity. A circumferential wrap (e.g., Coban) fol­lowed occasionally by stockinette can be used tosecure the orthosis. A Coban-like product is avail-

This paper is preceded, on p. 164, by a paper presenting a handsurgeon's perspective on the same subject.

Correspondence and reprint requests to Mia Fuller, MS, OTR,CHT, HealthSouth Atlanta Hand, 980 Johnson Ferry Road, Suite1000, Atlanta, GA 30342.

174 JOURNAL OF HAND THERAPY

able at pet stores under the name "Vet Wrap" andis easily attainable and inexpensive. In addition,parents have reported that thin socks work just aswell as stockinette. Parents should be providedwith a written splint instruction sheet that includesa picture of the splint and landmarks to ensureproper donning and doffing.

Children with limb deficiencies are at risk forpsychological and social adjustment problems.T"Hand therapists can assist the patient and familyin overcoming some of these obstacles through ed­ucation and training. Varni and Setoguchi' suggestthat parents who answer questions about theirchild's limb deficiency in a "simple, matter-of-fact,positive manner" set an excellent example for thechild to accept their physical differences. In addi­tion, coordinating parentichild/teacher confer­ences as the child reaches school age can alleviatethe teacher's concerns and answer questions aboutthe child's functional abilities." The hand therapistcan also encourage and teach cooperative play andthe use of role-playing in response to questionsabout a child's condition.

Although hand therapy intervention in thetreatment of congenital anomalies is frequently re­quested and expected by referring physicians, lit­erature documenting the effectiveness of such in­tervention is lacking. Therapists must work closelywith the treating hand surgeon to optimize patientcare, as each case is unique. The following disor­ders are commonly seen by hand therapists.

RADIAL LONGITUDINALDEFICIENCY

The patient with radial club hand presentswith shortened, tight radial structures that usuallyrequire immediate therapeutic intervention once adiagnosis is made. Initial treatment includes cast­ing, followed by splinting to stretch the soft tissueand improve the deformity. This is frequently per­formed prior to surgical intervention to gain max­imal range of motion. When fabricating the orthosisthe objective is to centralize the hand over the ulna(Figure 1).9 The splint is based radially and includes80% of the volar and dorsal surface of the hand and

forearm. The splint is cut out volarly and dorsallyat the distal ulna in a small semicircle. A Velcrostrap is then placed across this area with thick pad­ding on the strap at the cutout to allow pressure atthe ulna, thus pulling the wrist into ulnar devia­tion. Additional straps are placed proximally anddistally, to secure the splint. The elbow is left freeto move; however, the proximal portion of thesplint has "wings" that come just below the epi­condyles to include as much of the forearm as pos­sible, while still allowing elbow range of motion. Ifa thumb is present it is also left free, along with thedigits, to promote mobility. The splint is worn full­time, with the exception of removal for hygiene andrange-of-motion exercises.

Parents should be instructed in passive rangeof motion (PROM) techniques for the digits, wrist,elbow, and shoulder in all planes of movement. Thedigits frequently present with decreased flexionability, and the elbow joint may be stiff or anky­losed. When performing PROM, PlateO instructsthat, ft ••• as the elbow is being bent, the hand mustalso be corrected on the forearm both by distractionand ulnar deviation." It is difficult for parents toinflict discomfort on their child, and they must beproperly trained in mild, prolonged, slow stretch­ing. This can be incorporated into a daily warmbath routine to increase comfort and compliance.

Postoperative care includes the splint de­scribed above, which is fabricated after removal ofthe initial cast. It is imperative that the patient re­turn to the therapist on an ongoing basis for ad­justments and refabrication as the child grows. Thesplint is worn until skeletal maturity is achieved.In a long-term review of sur~ical treatment of radialclub hand, Bayne and Klug1 emphasize the impor­tance of postoperative splinting, indicating that halfthe patients with unsatisfactory results in theirstudy were noncompliant with splinting. With therecent use of external fixation, pin-care and digitrange-of-motion exercise to prevent stiffness are im­plemented.

The development of prehensile patterns, theperformance of activities of daily living, and theability to play are affected by this condition. Com-

FIGURE 1. Postoperative splint for centralization.

pensatory patterns such as grasping objects withthe forearms or manipulation in extremes of wristflexion may be present. Parents are taught to en­courage gross and fine prehension. Bilateral activi­ties can encourage functional use immediately aftersurgery. Progressing from larger to smaller toys willrefine prehension. Built-up handles and wash mittsshould be used to improve independence in dailyliving. Children are followed through school age,and then appropriate recommendations may bemade for further intervention within the school sys­tem.

SYNDACTYLY

Syndactyly is the most common congenitalhand disorder and can range from simple to com­plex, based on the degree of webbing and status ofbony fusion." Hand therapy can be employed be­fore surgery with the goal of educating parents andother caregivers in proper stretching techniques.This may serve to widen the skin web space, thuspossibly making surgical intervention easier.10

Following syndactyly separation, postopera­tive rehabilitation focuses on scar and wound man­agement and the prevention of web creep. A staticforearm-based splint is fabricated, positioning thewrist in slight extension and the digits in extensionand abduction. This splint offers protection, post­operative rest, and compression. The splint materialis brought between the digits. An Blastomere insertis fit in the splint and should touch each involvedweb space (Figure 2). It is sometimes necessary tobring the Elastomere completely around the digit(i.e., forming a ring).

An additional obstacle in treating very youngchildren is maintaining the digits in the correct po­sition within the splint. Placing straps at each digit(instead of strapping across the dorsum of thehand) helps secure the digits in place. The holes forthe strap must be placed strategically so that themost proximal digit strap is close to the web space.The splint is worn full-time initially, except whenthe patient is exercising or bathing. The child isgradually weaned from the splint and encouragedto use the extremity. The child may continue withnighttime splinting for six to twelve months. Cus­tom-fit compression garments may also be used tooffer continuous compression and to stabilize Elas­tomere inserts. These are available in a variety ofcolors and can be made to exclude the uninvolveddigits. These garments allow functional prehensionwhile compressing the scar. Parents should be in­structed in passive range of motion exercises to theentire upper extremity. The therapist should alsoprovide age-appropriate play guidelines to pro­mote functional pinch and grasp. Scar massage isincluded in the home program, as well as avoid­ance of sun exposure. Desensitization ideas are pro­vided to the parents, utilizing household itemssuch as a cotton ball, washcloth, and infant emeryboard.

April-June 1999 175

FIGURE 2. Elastomere-insert postoperative syndactyly release.

THE CONGENITALLY ABSENTTHUMB

A child born without a thumb is "severelyhandicapped in social and educational develop­ment and in future ability to earn a living."lD Pol­licization is the surgical procedure designed to cre­ate a thumb from an existing digit. Because of theextensive mobilization and reattachment of mus­cles, the greatest postoperative challenge to thehand therapist is prevention of an adduction con­tracture. Referring physicians may allow unre­stricted thumb range of motion at six to eightweeks postoperatively. It is at this time that the in­itial cast is removed and the patient is placed in ac-web spacer splint to maintain the web spaceachieved in surgery. Aquaplast splinting materialconforms nicely and allows the therapist to pinchthe material together during fabrication, which isuseful with young children who have difficulty sit­ting still. This gives the therapist an additionalhand to fit the material to the web space. In addi­tion, an Elastomere insert is fit in the web space, tobe worn in conjunction with the splint to assist inscar remodeling.

Parents are educated in scar massage andstretching of the web space. If joint limitations arepresent in other digits, the parents are shown pas­sive range of motion techniques to these involvedareas. Encouragement of functional grasp andpinch is emphasized. Bilateral play activities (e.g.,large ball, pat-a-cake, swinging a bat) are useful forencouraging initial use of the recently operatedhand. Age-appropriate prehensile tasks (e.g., pegsof varying sizes, play dough, beads, cards) are sug­gested to stimulate fine motor skills. It may be use­ful for short periods of play to suspend the ulnardigits into the palm to further encourage use of thenew thumb against the radial digits. Coban or pa­per tape is useful when maintaining the ulnardigits. The patient is followed monthly for the firstyear, and then annually until skeletal maturity isreached, to ensure progression of range of motion,scar remodeling, and functional hand use.

176 JOURNAL OF HAND THERAPY

THUMB DUPLICATION

The goal of surgical intervention with a dupli­cate thumb is to ablate the more hypoplastic thumband create a functional and cosmetically appealingresult with the remaining thumb. One of the mostcommon postoperative complications is a zig-zagdeformity, consisting of ulnar deviation of the prox­imal phalanx with radial deviation of the distalphalanx. This deformity may be a result of inade­quate bony alignment, tendon imbalance, or scarcontracture." A forearm-based thumb spica splintwith the interphalangeal (IP) joint included shouldbe fabricated after the initial postoperative dressingis removed. When fabricating this splint, three­point pressure is needed to properly align the pha­langes. Two points of pressure are applied proxi­mally and distally to the IP joint on the radialsurface of the thumb, with counter-pressure ap­plied at the ulnar IP joint. The web space must bemaintained within the splint. The splint is wornfull-time for three months and then nightly for anadditional three months. Monitoring is needed toavoid the zig-zag deformity. The revised thumb of­fers excellent support and stability, but the childwill need encouragement with grasp-and-pinch ac­tivities, as well as functional use of the affected ex­tremity. Hand therapists receive training in motordevelopment and can assist in achieving develop­mental milestones. In addition to therapeutic inter­vention postoperatively, Iwasawal et a1. 13 reportedeffective results with the use of splinting preoper­atively. This study describes an increase of 10° to30° at the IP joint with preoperative splinting,thereby simplifying the patient's later surgical pro­cedure.

CONCLUSION

In summary, as we approach the 21st centuryit is evident that we now have a vast array ofsurgical options and interventions for treating thepatient who presents with a congenital difference.Postoperative rehabilitation with specialized hand

therapists can playa vital role in a patient's recov­ery. The therapists' ultimate responsibility is to en­sure that parents get the training they need to con­duct a thorough home program, including scarmanagement techniques, range of motion andstrengthening exercises, play facilitation, promotionof gross and fine motor skills, splint wear, andmonitoring of their child's progress. Assisting thefamily and child in coping with functional andemotional barriers is needed. Unfortunately, re­search to support this type of therapeutic interven­tion in this population is still lacking. The challengeto therapists in the new millennium is to gatherdata, perform individual case studies, and furthersubstantiate the value of therapeutic interventionfor congenital upper extremity differences.

REFERENCES

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Camptodactyly: classification and results of nonoperativetreatment. J Pediatr Orthop. 1994;14:814-9.

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4. Varni 1, Setoguchi Y. Psychosocial factor in the managementof children with limb deficiencies. Phys Med Rehabil ClinNorth Am. 1991;2:395-404.

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6. Varni J, Rubenfeld L, Talbot D, Setoguchi Y. Family func­tioning, temperament, and psychologic adaptation in chil­dren with congenital or acquired limb deficiencies. Pediat­rics. 1989;84:323-30.

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8. Varni J, Setoguchi S. Screening for behavioral and emotionalproblems in children and adolescents with congenital or ac­quired limb deficiencies. Am J Dis Child. 1992;146:103-7.

9. Lourie GM, Lins RE. Radial longitudinal deficiency: a re­view and update. Hand Clinics. 1998;14:85-99.

10. Flatt AE. The Care of Congenital Hand Anomalies. 2nd ed.St. Louis, Mo.: Quality Medical Publishing, 1994.

11. Bayne LG, Klug MS. Long-term review of the surgical treat­ment of radial deficiencies. J Hand Surg. 1987;12A:169-79.

12. Cohen MS. Thumb duplication. Hand Clinics. 1998;14:17­28.

13. Iwasawa M, Matsuo K, Hirose T, Sakaguchi Y.Improvementin the surgical results of treatment of duplicated thumb bypreoperative splinting. J Hand Surg. 1989;14A:941-5.

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