Occupational Therapy in the Habilitation of The Child with Arthrogryposis

Arthrogryposis multiplex congenital is a non-progressive syndrome characterized by poorly developed and contracted muscles and deformed joints with thickened periarticular capsules. The sensory system is not affected. Upper limbs usually present internal rotation of the shoulder, elbow extension, wrist flexion and ulnar deviation, hyperadducted thumb, and contractures of the distal interphalangeal joints.

At the Greenville Unit of Shriners Hospitals for Crippled Children we have treated 188 children with a diagnosis of arthrogryposis from 1930 to the present. Forty-eight cases are still active; patients range in age from 9 months to 21 years of age. Twelve individuals with involvement limited to the lower limbs were not included in this report. Of the remaining 36 patients, four display only upper limb involvement, while the other 32 present multiple limb involvement with a wide range of severity. This article deals specifically with the management of 36 children and adolescents who have involvement of the upper limbs, with or without lower-limb deformities.

Twenty patients were reevaluated for this report. Assessment included examination of the child and a structured interview of the child and parents. They responded to a questionnaire, providing information regarding birth history, genetic background, other diagnoses, age of appearance of developmental milestones, and previous treatment, including surgery, orthoses, and adaptive equipment. Children were examined to determine their current ability to perform daily activities and their mode of locomotion. Information on patients who were not able to return to the clinic for personal examination was obtained through chart review.

Other Disorders

Patients presented other disorders, many of which complicated management. Four developed scoliosis, two demonstrated torticollis, and one had congenital amputation; other associated diagnoses were Moebius syndrome in one child and undescended testes in two boys.

Orthopedic Surgery

Only two of the 36 patients in this series underwent orthopedic surgery. One child had unilateral deepening of the thumb web space. The other patient, who had congenital above-elbow limb deficiency, had a Green procedure, involving transfer of the wrist flexor tendons to the extensor surface. Examination of all patients with arthrogryposis includes consideration of orthopedic surgery; however, most children did not have surgery because the likelihood of benefit was small or nonexistent, as compared with results anticipated with a conservative program.

Occupational Therapy

Children were managed with a multidisciplinary approach, in which occupational therapy had major responsibility for enhancement of the patient's upper-limb, function. The impact of occupational therapy was influenced by the physician, physical therapist, nurse, orthotist, social worker, family, and community resources.

The goals of the occupational therapy program were to:

  1. Increase and maintain joint range of motion
  2. Develop functional sitting and standing posture
  3. Foster achievement of gross motor competence
  4. Achieve maximum independence in the skills required for activities of daily living

These goals were accomplished by means of a program which emphasized range of motion exercises, augmented by appropriate splinting; adapted seating with trunk and lower-limb orthoses, as required; and special equipment for feeding, dressing, toileting, and play and school activities, whenever necessary.

The fragile bone structure of the child with arthrogryposis was taken into consideration when the comprehensive occupational therapy regimen was designed. Risk of fractures of all limbs is very high. Nevertheless, conscientious safeguarding resulted in only two humeral fractures, both in infants younger than 1 month of age.

Occupational therapy was prescribed for infants as early as 2 weeks old. Regardless of age, patients engaged in an intensive program intended to increase range of motion. Those who were in-patients were treated four times a day, for half hour sessions. Most children remained in the hospital for 6 to 8 weeks. They were discharged with detailed home programs intended to continue the gains already achieved. Home programs consisted of stretching, splinting, and developmental activities. Family compliance was imperative. Mothers were urged to conduct the program on a daily basis. The nursing service and the family were involved in the exercise program while the child was an in-patient, in order to reinforce parental training to be used after discharge.

Range of Motion

Static hand, elbow, and knee splints were fabricated to provide constant stretch on deformed joints. As joint excursion increased, the orthoses were altered appropriately. Initially, patients wore upper-limb splints at all times, except for bathing and exercise. Wearing times were decreased to naps and nights as children became more interested in their hands, using them for playing, beginning to grasp, and when rolling skills were initiated. A physician casted the children's feet to reduce deformities and prepare patients for secure standing and walking (Fig. 2 ). All seven patients treated before the age of 2 years benefitted from the program. They showed progressive increase in range of motion through their hospitalizations, and the gains were maintained with the home programs.

Motor Skills and Activities of Daily Living

Fine motor skills were achieved by encouraging children to use either lateral pinch or bimanual grasping. Ten patients demonstrated poor to fair biceps power; the others learned a total body pattern sufficient to enable hand to mouth activity (Fig. 3 ). Although formal intelligence testing was not conducted, most patients displayed evidence of normal or above normal intelligence, even in the presence of obvious delays in fine and gross motor tasks. Two children, however, appeared to have mental retardation; both were referred to developmental centers where they showed improvement.

Most patients could feed themselves without the use of adaptive equipment. A few use various types of feeders, ranging from commercially available devices, such as the Eatery* and modified sandwich holder** (Fig. 4 ) to a custom made clothespin feeder (Fig. 5 ).

Commercially available adaptive equipment designed to aid dressing and personal hygiene often proved too heavy or bulky for children with arthrogryposis. Consequently, independence was difficult to acquire. Clothing adaptations, such as Velcro closures in place of buttons, loops on the trouser waistband and at lateral mid thighs to assist in pulling trousers up, key ring zipper pulls, and elastic suspenders, have assisted some children toward independent dressing.

Head pointers (Fig. 6 ), mouthsticks, and pencil holders purchased from Fred Sammons, Inc. have been used to improve communication and play performance. Approximately half of the patients older than 9 years became independent in daily activities. Those who required assistance were either still leaming techniques, or were severely involved.

Functional Sitting, Standing, and Locomotion

Seating modifications, orthoses, and adaptive equipment enabled patients to sit, stand, and move about. Strollers (see Fig. 2 ) and corner seats adapted with inserts improved the positioning of younger children. One child used a travel chair; he was very difficult to position in a stable manner. Small tilt tables and standing boxes were effective in aiding children to stand and increase their weight-bearing tolerance.

For patients who could not walk independently, we have utilized the electric wheelchair, Greenville Swivel Bucket (Fig. 7 ), and a modified parapodium. Wheelchairs, used by five patients, had various control adaptations, such as chin and foot controls. Two children managed with the custom made Greenville Swivel Bucket. Maneuvering with lateral trunk motion, they could traverse 61 in (200 feet) or more. This orthosis was used constantly as the main form of mobility at home and school. When not in the bucket, children travelled from one place to another by log rolling themselves.

One young girl uses a special parapodium mainly for standing (Fig. 8 ). The basic Canadian design was adapted into an appliance custom made by a local orthotist. In the parapodium, the patient has improved her fine motor skins. She was also fitted with knee-ankle-foot orthoses and may eventually be able to ambulate with them.

School Activities

Children in this series attended developmental centers, preschools, and regular elementary and high schools. One young man expressed a desire to study art in college. Children with arthrogryposis reported participating in a wide range of extracurricular activities, including dancing and school plays.

Conclusions

Review of 36 patients with arthrogryposis involving the upper limbs, with or without associated lower-limb deformities, indicates that:

  1. A multidisciplinary approach with the patient and family is essential. The physician, nurse, physical therapist, orthotist, social worker, and community resources complement the occupational therapy program.
  2. The child with arthrogryposis benefits from early and intensive therapy, consisting of stretching, range of motion exercises, and splinting.
  3. Conservative management generally precludes the need for orthopedic surgery.
  4. Adaptive equipment for feeding, dressing, hygiene, communication, and play activities should be used to maximize independence in daily activities. Seating devices, trunk and lower-limb orthoses, and commercial and custom mobility aids foster independent sitting, standing, and mobility.
  5. Seating devices, trunk and lower-limb orthoses, and commercial and custom mobility aid foster independent sitting, standing, and mobility.
  6. Family involvement and compliance with the home program is imperative to maintain the results from therapy and improve the child's developmental status and independence.

EDITOR'S NOTE:

Readers may also be interested in "A Special Devices Program for an Arthrogrypotic Child," by E. Trefler and R. Fulford which appeared in InterClinic Information Bulletin 13:6:1-4,10, 1974.

*Maddak, Inc., Pequannock, NJ 07440-1993

**Fred Sammons, Inc., Box 32, Brookfield, IL 60513-0032