Journal of Pediatric Orthopaedics 22:44–47 © 2002 Lippincott Williams & Wilkins, Inc., Philadelphia Arthrogryposis Wrist Deformities: Results of Infantile Serial Casting *Dean W. Smith, M.D., and †James C. Drennan, M.D. Study conducted at Carrie Tingley Hospital, Albuquerque, New Mexico, U.S.A. Summary: Arthrogryposis multiplex congenita involving the functionally independent at final follow-up, and had no recur- upper extremity can be associated with significant contractures rence of deformity. Patients with classic arthrogryposis had of major joints. Treatment options to maximize upper extremity rigid wrist flexion contractures and a 75% incidence of defor- motion and function include passive joint stretching, serial cast- mity recurrence after casting. At final follow-up, these patients ing, or surgical intervention. This study reviewed all patients at remained functionally dependent, requiring >50% assistance Carrie Tingley Hospital with arthrogrypotic wrist flexion con- with activities of daily living, and had less improvement in tractures treated with passive stretching, serial casting, and cus- wrist motion. The authors recommend early casting of infant tom wrist orthotics to determine the effect on wrist position and wrist deformities for both forms of arthrogryposis. If the wrist function. Seventeen infant patients with distal and classic ar- deformity recurs, repeat serial casting is unlikely to improve throgryposis used this regimen. Average follow-up was 6 years. wrist extension. Other treatment options may be considered in The greatest gain in wrist motion occurred after the first casting the older child. Key Words: Arthrogryposis—Serial casting— session for both groups. Patients with distal arthrogryposis had Wrist deformity. the largest improvement in passive wrist motion, were more Although the etiology of arthrogryposis remains un- Distal arthrogryposis was described as a separate syn- known, restriction of intrauterine movement is probably drome in 1982 and is characterized by congenital con- responsible for the severe joint stiffness (6,7,10). The tractures of distal joints. Clenched hands with overlap- contractures and muscle loss correlate with specific seg- ping of digits, wrist contractures, foot deformities, and mental neurologic motor deficits and decreased anterior variable large joint involvement are found; the elbow horn cell populations (3,5,6). tends to be spared. The inheritance pattern is recognized Classic arthrogryposis multiplex congenita was first as autosomal dominant. reported in 1907 as a condition characterized by multiple Our retrospective study reviews 17 infants with mod- joint contractures. The hand, wrist, elbow, and shoulder erate to severe wrist flexion contractures treated with as well as major lower extremity joints are commonly passive stretching, serial casting, and custom wrist or- involved. The forearm flexors/extensors, deltoid, and bi- thotics. We report their response to the treatment proto- ceps may be aplastic and can have abnormal insertion col and interim outcomes of wrist extension and func- sites. The upper extremities may lack skin and joint tional independence. There are no previous reports in the creases, are tubular, and retain normal sensation. Com- literature that measure upper extremity function using a mon contracture patterns include internal rotation of the standard functional score after serial casting of infant shoulder, extension of the elbow, pronation of the fore- arthrogrypotic wrist deformities. The differentiation be- arm, and wrist/finger flexion. The contractures progress tween the classic and distal arthrogrypotic response to as limb growth occurs. No specific genetic inheritance early serial casting is also reported for the first time. pattern is recognized, and associated congenital anoma- lies are often present. METHODS Address correspondence and reprint requests to Dr. James C. Twenty-three patients with arthrogryposis were Drennan, Carrie Tingley Hospital, 1127 University Boulevard, NE, treated at Carrie Tingley Hospital between 1981 and Albuquerque, NM 87102, U.S.A. 1999. Six patients were excluded from this study: four From the *Department of Orthopaedics & Rehabilitation, University had minimal or no upper extremity involvement and two of New Mexico School of Medicine, Albuquerque; and †Carrie Tingley Hospital, University of New Mexico Health Sciences Center, and the underwent most of their treatment at other facilities. Sev- Department of Orthopaedics and Pediatrics, University of New Mexico enteen patients (nine girls, eight boys) had moderate to School of Medicine, Albuquerque, New Mexico, U.S.A. severe wrist flexion contractures along with other asso- 44 ARTHROGRYPOSIS WRIST DEFORMITIES 45 contracture was 25°.Chartswereretrospectivelyre- viewed and patients or families were contacted. Patients and their families completed the WeeFIM (8) instrument for independent function in children at final clinical fol- low-up or by telephone interview. Wrist extension was measured and recorded after each casting session and clinical visit. RESULTS The average number of casting sessions per patient/extremity was four, with an average length of 56 days per session. All patients with distal arthrogryposis required a single casting session to gain significant pas- sive wrist extension. Nine of the 12 (75%) patients in the classic group had recurrence of deformity, whereas none in the distal group had recurrence. Most patients with classic arthrogryposis underwent four or more casting sessions. FIG. 1. Newborn hand shows the typical index finger pronation The most common associated upper extremity defor- and overlap of the long finger that is associated with distal ar- mities included contracture of the proximal interphalan- throgryposis. geal joint, elbow, adducted thumb, metacarpophalangeal joint, ulnar wrist and pronation deformity, and internal rotation of the shoulders (Fig. 4). Three patients in the ciated upper extremity contractures (Fig. 1). Twelve classic group eventually underwent flexor carpi ulnaris were diagnosed as having classic and five as having dis- transfer that delayed but did not prevent recurrence of tal arthrogryposis. their wrist deformities. Compliance with the prolonged All patients underwent serial casting sessions, fol- treatment plan was a problem in 60% of the cases. Fam- lowed by a stretching program and custom orthotics to ily and patients stated that the continuous use of casts or maintain wrist position (Fig. 2). The gentle sequential orthotics interfered with activities of daily living and casting technique addressed the digital, metacarpopha- writing in older children. langeal joint, and wrist contractures with short arm casts The largest gain in passive wrist extension occurred extending from the forearm to the phalanges. To prevent after the first casting session (Fig. 5). The average final maceration, cotton padding was placed between the pha- passive wrist extension in both groups was 9° on the left langes and felt pads were used to protect the skin during and 7° on the right, with a 33° mean correction of the three-point molding of the casts. No skin problems or wrist deformity. Recurrences undergoing additional se- secondary digital fixed contractures were noted with this rial casting failed to be corrected past neutral. Patients technique. with distal arthrogryposis achieved a greater final pas- Fourteen of the 17 infants began treatment within 6 sive wrist extension (27°) than the classic group (1°) months of age, with 11 starting treatment at 1 month of (Fig. 6). Final follow-up ranged from 1 to 16 years (av- age or younger (Fig. 3). The average initial wrist flexion erage 6). FIG. 2. A: Correction of the deformity proceeds serially from distal to proximal joints. The introduction of cotton cast padding between the fingers decreases the risk of skin maceration. B: A felt pad is placed over the dorsum of the wrist. Wide thumb abduction and slight ulnar deviation of the wrist increase the width of the first interosseous space. C: Gentle pressure is applied to volar metacarpophalangeal and dorsal wrist areas. Additional wrist dorsiflexion was obtained with further serial casts. JPediatrOrthop,Vol.22,No.1,2002 46 D. W. SMITH AND J. C. DRENNAN FIG. 5. The dramatic improvement that follows the initial casting is followed by more limited improvements in subsequent casting series. of the five patients in the distal group were younger than 36 months old at the time of final follow-up. The distal group also reported improvement in upper extremity FIG. 3. Three-year-old patient with arthrogryposis multiplex con- function with age, whereas the classic group tended to- genita shows uncorrected wrist deformity. Note the absence of ward static functional deficits. the digital skin creases, which reflects absence of active motion. DISCUSSION WeeFIM functional independence scores for the chil- The goal of treatment for the upper extremity in both dren were completed at final follow-up (8). Complete forms of arthrogryposis is to increase motion and perfor- independence is demonstrated by a score of 7, whereas mance in activities of daily living, such as feeding and total functional dependence has a score of 1 (Table 1). personal hygiene tasks. Caution must be taken that use of Fourteen of 17 patients/parents completed the WeeFIM a specific treatment protocol to improve one functional questionnaire on upper extremity function and self-care, activity does not interfere with another important func- including personal