Physical therapy management of patients with juvenile rheumatoid arthritis.

NJ Rhodes, MPH, PT, is Senior Physical Therapist, Newington Children's Hospital, 181 Cedar St, Newington, CT 06111 (USA).

Juvenile rheumatoid arthritis (JRA) is the most common pediatric rheumatoid disease in North America and a major cause of childhood disability.(1) Up to 25% of children with JRA have unremitting disease, which may lead to contracture and deformity,(2) and approximately 10% will enter adulthood with severe functional disabilities.1 Significant visual impairment may also occur as a consequence of this illness, secondary to iridocyclitis, a chronic eye inflammation affecting the iris and ciliary body.

Etiology, Incidence, and Clinical Manifestations

Although the cause of JRA is unknown, the disease is thought to have a multifactorial etiology; possibly an infectious agent triggers a genetically predisposed autoimmune response, which results in joint inflammation.(1,3)

There have been several epidemiologic studies that have attempted to determine the incidence of JRA. Recent data from the Mayo Clinic show an incidence of 13.9 cases per 100,000 individuals per year and a prevalence of 113.4 cases per 100,000 individuals.(1) A 1986 Finnish study(4) estimated that the incidence is 19.6 cases per 100,000 individuals per year. Most recently, Andersson-Gore and Fasth(5) presented data from Goteborg, Sweden, that showed an incidence of 11 cases per 100,000 individuals per year and a point prevalence (number of total cases at a given point in time) of 82 cases per 100,000 individuals. juvenile rheumatoid arthritis affects girls 2:1 over boys.(1,3) There are three distinct modes of onset in JRA, however, and sex ratios are strongly affected by onset type.1,3,6 The American College of Rheumatology,'s criteria for classification of JRA are listed in the Appendix. (1)

Certain manifestations of JRA differ from those of adult rheumatoid arthritis (RA). Systemic disease is more common in children with JRA than in adults with RA. Unlike adult RA, chronic eve inflammation iridocvclitis) may be a symptom of JRA. In children with JRA, there is predominantly large joint involvement and frequent cervical spine anklyosis. Typical wrist and hand deformities in children with JRA include wrist subluxation and radial deviation with ulnar deviation of fingers. In adults with RA, wrist ulnar deviation with finger radial deviation is the usual pattern. Additionally, rheumatoid factor RF), an abnormal immune complex in the blood, is seen in fewer than 15% of young children with JRA, but is seen in 80% to 85% of adults with RA.(3)

Inflammation occurs naturally within the body as part of the immune system's defense against disease and trauma. Antigens (bacteria, viruses, and other foreign substances) are initially processed by macrophages and mononuclear cells. Cellular chemicals, such as cytokines, attract the humoral (B cell) and cellular (T cell) arms of the immune response. These lymphocytes may then work in concert or independently to immobilize the antigen. B cells produce specific antibodies that may attach to the antigen, attract complement proteins, and form immune complexes. These complexes may then activate other components of the inflammatory system, including phagocytic neutrophils, kinins, and proteolytic enzymes.

In the development of inflammatory arthritis, either immune complexes or a putative antigen invades the synovium, initiating the cascade of inflammatory events. Lymphocytes lose their ability to distinguish between antigens and healthy tissue. Lysosomal enzymes and collagenase released into the joint fluid destroy synovial membrane cells.

Synovial proliferation results from the inflammatory reaction, creating a mass, or pannus, that can then overlie the articular surface and soften and weaken the cartilage. Destruction of the bone can occur at the articular margins through blood vessel foramina.(7) Effusion and synovial thickening lead to increased intra-articular pressures. Surrounding soft tissues undergo fibrosis and contracture. Uncontrolled disease may lead to joint fibrosis or even bony ankylosis.

After bone destruction has occurred, the synovial membrane and periarticular tissues contain products of bone and cartilage destruction. This contamination may further contribute to synovial and periarticular fibrosis, as well as direct involvement of tendons with adhesions and contracture.(6) Readers wishing further information regarding pathogenesis should refer Radiologic Changes in the Rheumatoid Joint

The following changes may be noted by observing radiographs of affected joints: (1) opaque-looking bone, suggesting osteoporosis; (2) decreased joint space, suggesting erosion of cartilage (Fig. 1); (3) variations in bony outline, indicating bone cysts, erosions, or spurs; and (4) loss of normal axial alignment of adjoining bones." Cartilage loss may be seen by magnetic resonance imaging before appearing on radiographs. There may be hypertrophy, accelerated growth, or irregular growth of bone around an involved joint, probably attributable to an increased blood supply secondary to inflammation. Premature fusion of epiphyses also commonly occurs. (1-3,8)

Characteristics of the Three Onset Types

There are three distinct onset types of JRA. Because the course of the disease, well as the prognosis, varies among the onset types, it is very important to understand the differences among them.

Systemic-onset JRA, also known as Still's disease, accounts for 10% of all cases and occurs throughout childhood, with a 1:1 female-to-male sex ratio. Clinical findings may include an intermittent spiking fever, which may be as high as 104[deg]105[deg]F, and a fleeting rash of pale erythematous macules is present in the majority of patients.(1,3) The rash appears on the trunk and proximal extremities and less commonly on the distal extremities (Fig. 2). The rash may recur for months or years, even in the absence of other signs of disease activity. Systemic symptoms may include pericarditis, hepatosplenomegaly, and lymphadenopathy. Laboratory findings may include anemia, an increased white blood cell count, and an elevated erythrocyte sedimentation rate, a measure of inflammation. Most children with Still's disease develop chronic polyarthritis. Approximately half those diagnosed with systemiconset JRA have a progressive increase in the number of joints involved, and they eventually have moderate to severe disability.(1)

Polyarticular JRA, the second subtype, occurs in 40% of all children with JRA. It occurs throughout childhood, peaking at 1 to 3 years of age, with the female-to-male ratio being 3:1.1 Clinical findings include acute or insidious symmetrical arthritis of the upper and lower extremities, with more than four joints involved. There is an absence of systemic symptoms. Laboratory findings include an elevated erythrocyte sedimentation rate and may include a positive rheumatoid factor.(1,3) Children with rheumatoid factor-positive polyarticular JRA have a poor prognosis, with a greater possibility of erosive arthritis. Their arthritis more closely resembles adult RA, with frequent findings of subcutaneous nodules attached to the tendon sheaths of periarticular structures.

The third subgroup of JRA is known as pauciarticular JRA, or oligoarticular JRA, with four or less joints involved. This type occurs in 50% of all children with JRA. it usually occurs in children under 10 years of age, peaking at 1 to 2 years of age, with the male-to-female ratio being 5:1.1 In pauciarticular JRA of the lower extremities, involvement is often asymmetrical. The knee is the most commonly affected joint, followed by the ankle and the elbow.9 Laboratory findings include an elevated sedimentation rate and may include a positive antinuclear antibody (ANA) blood test. The presence of immunoglobulins, which act against nuclear cellular material, results in ANAs. In pauciarticular ANA-positive JRA, the child is at high risk for development of iridocyclitis, a chronic eye inflammation. Iritis may lead to impaired vision: 15% to 30% of children with iritis will eventually develop functional blindness.(1) Iridocyclitis is treated with ophthalmologic corticosteroid preparations and pupil-dilating drops, which prevent scarring of the pupil and iris.

The prognosis in pauciarticular arthritis is good. Few joints are affected, and functional disability is uncommon. In addition, a greater percentage of these children have early remission.(1)

Principles of Care in Juvenile Rhournatoid Arthritis

The treatment program for children with JRA must be individualized. A team approach that involves die family and child as key members is ideal. Professionals who may become involved with the child include pediatric rheumatologists, nurses, physical therapists, occupational therapists, social workers, psychologists, ophthalmologists, orthopedists, orthotists, dietitians, and cardiologists. The goals of team management are to decrease joint inflammation, relieve pain, achieve or maintain an optimal level of function, and educate the patient and family as to the course of die disease and the care required.

Principles of care include a long-term program supervised by a rheumatologist, adequate rest and good health habits, an exercise program directed by a physical therapist, medications to control joint inflammation, and school support and intervention where needed.

Medications play a major role in the management of the patient with JRA. The drugs of choice are the anti-inflammatories, which include salicylates (aspirin) and other nonsteroidal anti-inflammatory drugs such as ibuprofen and naproxen. These drugs provide symptomatic relief of pain, stiffness, and swelling. They act, in part, by interfering with prostaglandin synthesis in the inflammatory process.(1,3)

The second-line drugs are called remitting agents, or slow-acting or disease-modifying antirheumatic drugs, because they are believed to interfere with the autoimmune process itself.3 These drugs include gold salts, hydroxychloroquine (Plaquenil(R)*), D-penicillamine, and sulfasalazine. Immunosuppressive drugs, such as methotrexate, and corticosteroicls have selected roles in management.(1,3) Experimental therapies include intravenous immune globulin and biologic agents such as specific targeted (monoclonal) antibodies.(10,11)

Principles of exercise and activity are of prima importance to physical therapists working with children with JRA. The Affiliated Children's Arthritis Centers of New England have developed physical therapy standards for the care of children with chronic arthritis. This program, funded by a Bureau of Maternal and Child Health and Resources Development Special Projects of Regional and National Significance (SPRANS) grant, consists of a network of 13 tertiary pediatric institutions located throughout the six New England states. Readers should consult these standards for ongoing guidance in the assessment, problem and goal identification, and treatment planning for children who have JRA. Generally, children with arthritis are encouraged to participate in age-appropriate physical activities, with limitations imposed only if the child experiences discomfort during or following an activity.

Physical therapists should remember that normal joint function cannot be maintained in the absence of motion and weight bearing.(13) Additionally, I believe that in young children who continue to mature motorically, physical exploration is essential for the acquisition of balance, coordination, perceptual skills, and self-confidence. Modifications of activities may be necessary, such as restricting gymnastic activities (eg, tumbling), for children with severe wrist disease or cervical spine involvement or limiting contact sports in children with extensive arthritis.(14) Such restrictions may be necessary to prevent exacerbation of inflammation or to avoid injury or fracture at a joint with limited mobility.

Children and their families should be educated in basic joint protection techniques. Static flexion positions should be avoided. When at rest, involved joints should he maintained in an extended position. Total body weight should not be placed on non-weightbearing joints if these joints are affected; activities such as cartwheels, handstands, chin-ups, and rope climbing should be avoided. (14) joint stress can he minimized in the upper extremities by carrying items close to the body and by using both hands; stress can be decreased in the fingers by using the palms of the hands for carrying items and for turning doorknobs, faucet handles, and lids. When ankles or metatarsals are involved, shoes with heels are to be avoided in order to prevent excessive stress on metatarsal heads as well as to maintain length of the Achilles tendons.

Physical Therapy Evaluation

Physical therapy evaluation begins with a baseline examination. Serial reevaluations are used to chart the progress of the disease and the effect of treatments, including medications and physical therapy. Because there is no single measure of disease activity, assessment of pain, swelling, range of motion (ROM), muscle strength (as determined by manual muscle testing), and mobility are critical in determining the overall benefits of team treatment and are essential in goal setting and treatment planning.

The first element of the evaluation should be the patient history. information, such as the presence and duration of morning stiffness, should he obtained. Additionally, the patient's (and parents') perception of pain and fatigue are noted. Information is gathered about the child's independence in activities of daily living, functional mobility at home and in school, and endurance throughout the day. The child's ability to participate in physical education, sports, and recreational activities is discussed. Any specialized equipment, splinting, or exercise programs already in place are observed and evaluated.

Next, an examination of the spine and lower-extremity joints is performed (upper-extremity, joints as well, if the child is not also seen by an occupational therapist). joints are evaluated for the presence of effusion, synovial thickening, heat, and redness. Swelling of the hip is not easily detected unless the child is thin. Knee swelling is most commonly seen over the medial superior and lateral aspects of the patella. Swelling can be detected at the knee by the use of "patellar tap" and "bulge" signs. The patellar tap is performed by stabilizing and slightly distracting the patella by the use of the thumb and forefinger proximally. The other forefinger is used to press quickly and firmly down on the patella. The patellar tap is positive if a rush of fluid is felt escaping from under the patella. The bulge sign is seen by firmly "milking" fluid away from the anatomical depression medial to the patella, then pushing gently against the patella laterally. if fluid is seen to refill the depression, an effusion is believed to be present. Therapists are cautioned regarding the use of girth measurements to assess swelling at the knee. Bony overgrowth of the femoral condyles commonly accompanies knee arthritis; therefore, even in the absence of effusion, an involved knee may be larger circumferentially than an uninvolved knee, if measured at the joint line. If girth measurements are taken, they are best performed proximal to the superior patellar border, above any effusion, to avoid the effect of bony overgrowth.

Ankle effusions are seen most frequently around the malleoli and dorsum of the joint.3 if swelling is present, the normal indentation between the anterior tibial tendon and the medial malleolus is lost. When the tarsal area is inflamed, swelling appears over the dorsum of the foot.(3) Swelling in the metatarsophalangeal joints can he assessed through observation and palpation, although this swelling may be difficult to detect. Swelling is often assessed using a three-point scale: 1+ = minimal, 2 + = moderate, and 3 +

=severe.

Pain is first evaluated by asking the child about pain at rest. Pain on motion and weight bearing is then assessed. Tenderness is differentiated from pain in that tenderness is determined deep palpation of the tissue about the joint.(3)

Measurement of ROM of the spine and lower extremities is then performed by the use of goniometry.(15) Linear measures, such as the Schober test of lumbosacral mobility and the incisor gap, are also helpful.(1) The Schober test is performed, with the child in a standing position, by measuring 5 cm below and 10 cm above a surface landmark (eg, a line between the dimples of Venus). The patient then flexes forward maximally, and the change in the linear measurement is recorded. Normal values are available for comparison.1 The incisor gap, a measure of temporomandibular joint mobility, is assessed by recording the distance between upper and lower incisors. The distinction between true joint limitation and muscle tightness should be made. When assessing joint ROM, care must be taken to place any two-point muscle in a shortened position over one of the joints.(3) For example, knee flexion is measured with the child in a supine position, as opposed to a prone position, in which knee flexion could be limited by rectus femoris muscle tightness. Ankle dorsiflexion is measured during knee flexion, as opposed to during knee extension, to eliminate the effects of gastrocnemius muscle shortening on ROM.

A muscle examination includes manual muscle testing, observation of muscle atrophy, and measurement of thigh and calf girth to determine whether the effect is asymmetrical. if a child is too young to participate in manual muscle testing, the child's ability to perform within the developmental sequence may give an indication of weakened areas.

A postural assessment is completed from anterior, sagittal, and posterior views. Postural deviations are noted, including the presence of scoliosis (structural versus functional), forward head, rounded shoulders, scapular winging," lordosis, kyphosis, and pelvic obliquity. Additional postural deviations/deformities common to JRA include femoral anteversion, genu valgum, tibial torsion, pes cavus or valgus, hallux valgus, and hammertoes.(16) Possible leg-length discrepancies are noted, because such discrepancies are common with unilateral knee arthritis.

Gait is usually assessed by observation. Using gait laboratory analysis, Lechner and colleagues'6 found significantly decreased velocity, cadence, and stride length in their subjects with JRA as compared with a group of children without JRA. The anterior pelvic tilt of the subjects with JRA was significantly increased throughout the gait cycle. Hip extension at terminal stance and ankle plantar flexion at toe-off were significantly decreased. Gait assessment should include observation of walking on level surfaces, stairs, and inclines, as well as assessment of distance that can be covered and the use of any assistive devices.

Functional mobility must be assessed on the basis of the following four measures: (1) proficiency, or the child's ability to independently complete a task from beginning to end; (2) movement quality, or the need to substitute muscle groups, revert to a lower developmental pattern, or use two hands or other body parts to assist in completion of a task; (3) speed of performance; and (4) endurance, or the ability to perform multiple repetitions of an activity.(12) An example of pediatric functional assessment tools that are used for the JRA population is the Pediatric Evaluation of Disability Inventory PEDI).(17) Currently undergoing normalization for ages birth through 7 years, this tool evaluates the areas of self-care, mobility, and social functions, as well as caregiver assistance required and modifications needed.

Several investigators have attempted to develop assessment tools for use specifically with the JRA population. These instruments include a modified version of the adult Arthritis Impact Measurement Scales AIMS)(18); the Newington Children's Hospital JRA Evaluation, a movement quality-oriented developmentally based tool for use in children 1 to 10 years of age(19); and the Juvenile Arthritis Functional Assessment Scale,(20) a task-oriented, timed assessment aimed at children 7 to 18 years of age. For discussion of the reliability and validity of the AIMS, the reader should refer to Coulton et al. (18) The juvenile Arthritis Functional Assessment Scale has an internal reliability of .85 (Cronbach's coefficient of alpha) and a convergent validity of .40 between score and total number of joints involved. A functional mobility assessment should include evaluation of developmental milestones and gross motor skills, bed/mat mobility, transfer abilities, and wheelchair mobility, if appropriate.

The child should also be assessed for splinting needs. Splints are indicated for joint contractures that are unresponsive to exercise. Lower-extremity splints are usually resting splints used at night and at nap times. The most commonly required lower-extremity splint is for knee extension. This type of splint is required because of the frequency of knee joint involvement in all subtypes of JRA, the weakening of the quadriceps femoris muscle mechanism in the presence of knee-swelling,(21) and the tendency for the knee joint to be flexed for comfort. Nighttime splinting of ankles to prevent heelcord shortening and in-shoe orthotic devices to correct forefoot or hindfoot deviations may also be helpful.(12)

Finally, the need for adaptive equipment is noted. This equipment may include a raised toilet seat, tub transfer seat, grab bars, ambulation aids, or a wheelchair.

Physical Therapy Techniques

The physical therapy program must be individualized for each child. In acute phases of JRA, the goal of treatment is to prevent loss of motion, disuse atrophy, and osteoporosis with loss of activities of daily living and functional mobility. During this time, appropriate treatment techniques include measures to provide comfort; active, active-assistive, and passive ROM exercises (no vigorous stretching); metric exercises; and contract/ relax techniques. Care should be taken to avoid excessive fatigue; rest is felt to be important when recovering from a flare of the disease. Therapeutic activities should not increase the child's symptoms, and painful exercises are to be avoided.

During chronic phases of JRA, goals need to be modified. The careful addition of progressive resistive exercises is permitted as long as an increase in symptoms (eg, pain, stiffness, swelling) does not occur. The child's activity level is gradually increased as tolerated. General conditioning programs such as swimming and bicycling are encouraged.

Various physical therapy modalities may be helpful in easing joint symptoms, especially stiffness and protective muscle spasm in acute stages of the disease. Moist heat in the form of a tub bath or shower, whirlpool, hot packs, or warm swimming pool appears to be beneficial. A paraffin bath can be used for hands or feet. Retaining body warmth overnight through use of warm pajamas or a heated water bed or sleeping bag can help to minimize morning stiffness.(14) Deep heating techniques, such as ultrasound or diathermy, are not recommended.(22) in addition to concern about the effects of these modalities on the epiphyseal plate in growing children, deep heating techniques may increase joint inflammation. The use of cold in the form of ice massage or cold packs can also decrease joint pain by providing local anesthesia; however, in my experience, most children with arthritis prefer heat.

Another useful method, if a patient has one or two very painful joints, is transcutaneous electrical nerve stimulation (TENS). This modality may provide enough pain relief to allow active or active-assistive exercise during a flare of JRA in order to maintain joint ROM. I have also found TENS to be very effective for use in early mobilization postoperatively. When TENS is used, the patient should be old enough to communicate discomfort and not be fearful of the procedure.

During any stage of the disease, biofeedback may be helpful in increasing joint ROM by providing information to the patient regarding correct muscle activation and by encouraging optimal effort. in already restricted joints, biofeedback may be necessary to obtain correct movement without muscle substitution and to overcome spasm.

Gait training in children with JRA should seek to minimize or eliminate observed deviations. This may be done through postural training; weight-bearing activities; and attention to symmetry, form, and cadence.(12) Primary deformities, such as femoral anteversion or genu valgum, may lead to secondary problems such as tibial torsion or pes cavum. Orthotic management may assist in easing areas of painful pressure and providing proper alignment.

Assistive devices may be used as training aids for short-term rehabilitation following surgery or to sustain independent ambulation in a patient with severe involvement. The assistive device should maintain good postural alignment. In the child with multiple joint involvement, care must be taken not to place the upper extremities in positions that may exacerbate the child's symptoms (eg, pain, stiffness, swelling). The use of a platform attachment for a walker or a crutch is a good alternative to abnormal weight-bearing stresses on the wrist and hand.

Improving functional mobility is a key goal of rehabilitation. Ongoing mobility and transfer assessments are essential. In my view, normal, age-appropriate motor patterns are the guide for training; however, modifications may sometimes be necessary. For example, excessive work on half-kneel-to-stand transitions or reciprocal stair climbing may be inappropriate in the presence of severe knee inflammation.

To assess aerobic fitness, Jasso and colleagues (23) compared the physical work capacity of children with JRA, as determined by measurement of maximal oxygen uptake, with that of children without JRA. The 16 children with JRA were found to be significantly less fit than their age-matched controls. Jasso and associates concluded that the amount of physical work that children with JRA are able to accomplish is related to the severity of their articular disease. This research also established that children with JRA have a problem in maintaining normal levels of physical fitness.

At Newington Children's Hospital (Newington, Conn), we conducted a study of aerobic exercise in children with JRA NU Rhodes, L Zemel; unpublished data; 1987). The purpose of the study was to determine whether aerobic capacity, as measured by maximal oxygen uptake, could be increased through training without adverse effects on involved joints or overall physical status. Seven subjects, aged 4 to 18 years, underwent 20 minutes of aerobic in-pool training three times weekly for 12 weeks, and six age-matched controls participated in a nonaerobic pool exercise program for the same duration and frequency. Although we were unable to demonstrate a statistically significant difference in maximal oxygen uptake (prestudy mean of 28.7 mL/kg/min; poststudy mean of 27.5 ndag/min) in the subjects involved in the aerobic exercise program, we did show a significant decrease in resting heart rate (decrease in means from 118 to 104 bpm). All children stayed well during the study period, without deterioration in selected variables of interest, including joint count (a measure of ROM and swelling),24 sedimentation rate, and morning stiffness. This research indicates the need to consider nonstressful means of aerobic conditioning in a pool or on a bicycle when designing exercise programs for children with JRA. Improving physical fitness does not need to be time-consuming. Harkcom and colleagues(25) found that as little as 15 minutes of exercise three times a week is sufficient to improve aerobic capacity in women with RA.

Orthopedic Management

Orthopedic manifestations of JRA are common, in that the hip joint is involved in 10% to 38% of children with JRA. 26 Within I year of onset of disease, the hips of 9% of all children with JRA are affected.(27) Figure 3 demonstrates the results of severe hip disease, including loss of joint space and cartilage and irregular articulating surfaces. Typical hip deformities include hip flexion contractures and loss of hip extension, abduction, and rotation. Hip movement may be painful at end ranges and with weight bearing.

Prone lying for at least 20 minutes daily is used to prevent or minimize hip flexion contracture.(15) Occasionally, an abduction orthosis may be prescribed to prevent adduction contractures. The length of the iliotibial band should be monitored by use of serial Ober's tests.(15) Acute synovitis of the hip may be relieved by skin traction, applied with the pull toward abduction, to distract and rest the joint.(7) Prolonged sitting at a desk or in a wheelchair increases the likelihood of losing ROM. A tricycle or bicycle may also be used to maintain ROM and muscle strength if ambulation becomes difficult.

If soft tissues surrounding the hip joint become contracted, surgical releases may be indicated to restore ROM. Adductor and psoas muscle tenotomies are most often performed, with success noted in increasing ROM and relieving pain.(7) Joint integrity, however, must be present. In hips with significant deterioration, with loss of joint space and irregular articular surfaces, nothing will be gained by soft tissue release. Espada et al(28) have shown a marked drop in flexion contractures and an improvement in joint motion up to the first 3 years postsurgery. They concluded that soft tissue release is a beneficial option in preserving alignment and function in hip and knee flexion deformities affecting patients with JRA.

Indications for total hip arthroplasty in children with JRA include marked functional impairment or severe disabling pain.(29) Several authors(7,3-32) have described successful outcomes in hip replacement surgery in this population. Custom-made or special miniature prostheses are required by as many as 50% of all children with JRA; these devices can be specially fabricated using computerized tomography linked with a computer-driven lathe.(29) Component wear is usually not a significant problem because of the small stature of children with JRA, although loosening is the most common reason for failure of prostheses.(29)

Postoperative physical therapy care for the patient with total hip arthroplasty includes an emphasis on early mobilization, with ambulation (with partial weight bearing on the operated hip) beginning as early as the second day. A positioning program teaches avoidance of hip adduction/ external rotation and flexion beyond 90 degrees for 6 weeks following surgery because of risk of subluxation. Hyperextension is not permitted, an abductor pillow is used in bed and while sitting, and a raised toilet seat and chair are used for ease of transfer. Exercises include active and active-assistive hip flexion and extension and active hip abduction exercises in a supine position.(33)

Common knee joint problems include effusion with flexion contracture (Fig. 4) and quadriceps femoris muscle atrophy, valgus deformity, and leg-length discrepancy (Fig. 5). The leg-length discrepancy is often secondary to asymmetric knee arthritis, leading to overgrowth of the affected side attributable to stimulation of the growth plate by chronic inflammation.", Treatment emphasizes maintaining knee extension and quadriceps femoris muscle strength.

Placement of pillows under the knees should be avoided, Bicycling and swimming may be helpful in maintaining muscle strength and ROM.

Knee flexion contractures are usually managed conservatively by nighttime extension splinting or short-term serial casting. Dynamic splinting, such as the use of Dynasplints(TM)*[dagger] to achieve knee flexion or extension, can be used to reduce contractures that do not respond to static splinting splinting with the joint held in one position without allowing free motion). Skin traction may also be helpful.(9) Because of muscle spasm or contracture, the tibia frequently moves backward relative to the femur, leading to a tightened anterior cruciate ligament. Hamstring muscle releases are advised early to prevent such contractures and subluxation; as movement is regained, the joint is better nourished and larger articular surfaces make contact. This can lead to healing if the disease remits.(7)

Leg-length discrepancy may he monitored by serial orthoroentgenograms. If necessary, an epiphysiodesis may he performed to halt overgrovall of the longer limb. Leg-length discrepancy is often treated with shoe lifts to maintain postural alignment. A partial epiphysiodesis or osteotomy may be necessary to correct genu valgum deformity. For some patients, Ilizarov instrumentation can correct leg-length discrepancies or valgus deformities.(34)

Knee synoxectomy can be performed to remove overgrown, inflamed synovium and relative joint inflammation.(35) Although the synovium will eventually grow back, this can he a useful procedure to relieve pain and may assist in gaining motion. Some authors,(7,35) however, disagree on the procedure's overall effectiveness in this young population.

Total knee arthroplasty is indicated for older children with JRA for preservation of ambulation and is effective in reducing pain and increasing functional mobility. Postoperatively, a knee immobilizer is used, with isometric quadriceps femoris muscle strengthening and active-assistive ROM beginning on the second day and ambulation on the third day. Continuous passive motion machines are often used to restore mobility. The goal of this approach is to achieve 90 degrees of knee flexion and full active extension by the 14th day postoperatively.(36)

Ankle and foot deformities also occur in children with JRA. In severe cases of the disease, an equinovarus deformity may be seen (Fig. 6). This condition may necessitate surgical correction of calcaneal deformity (such as a Dwyer osteotomy) and soft tissue releases for correction of alignment. in milder cases, valgus or pronated feet are often apparent. Shoe inserts or custom-molded orthoses may be useful in improving weight-bearing position. Heel cups may be indicated for painful heel spurs. Metatarsal bars may he used in shoes to relieve pressure from painful metatarsal heads. Another common foot deformity is hallux valgus with hammertoes. Custom-molded shoes may be indicated for painful deformities such as these, which are not responsive to a more conservative orthotic approach.

Conclusions

Physical therapists play a vital role in the care and management of children with JRA throughout the course of the disease. The physical therapist's serial evaluations can provide a barometer of disease activity, assisting the rheumatologist in decision making. Goal setting should be comprehensive and address all problem areas, which may include ROM, strength, pain, posture, aerobic conditioning, gait, and functional mobility. Physical therapy plays a crucial role in the team management of children with JRA. Components of this program include center-based treatment, as well as home exercises and guidelines for joint protection; positioning; and appropriate recreational activities. The physical therapist also participates in postoperative management of surgical interventions, which may include soft tissue releases, synovectomies, epiphysiodeses, arthrodeses, and joint replacements. Understanding the many facets of JRA, including the effects of exercise and activity, is important in attaining optimal functional outcomes for these patients.

Appendix. American College of Rbeumatology Criteria for Classification of Juvenile Rheurmtoid Arthritis (JRA)

1 . Age of onset less than 16 years.

2. Presence of arthritis in one or more joints defined as swelling or effusion, or. in the absence of swelling, by the presence of at least two of the following:

Limitation of range of motion

Tenderness or pain on motion

Increased heat

3. Duration of disease of at least 6 weeks,

4. Type of onset in the first 6 months characterized as one of the three onset types of JRA.

5. Exclusion of other rheumatic or viral diseases that may mimic JRA.

Acknowledgments

I thank Dr Lawrence Zemel for his support as teacher and friend and for his technical assistance in writing this article. I also thank Mary Gail Horelick, JD, MS, PT, and joan Page, MA, PF, for their support and editorial assistance and Eleanor Fox for typing the manuscript.

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10 Silverman E, Isacovics E, Schneider R, et al. Effect of intravenous gamma globulin on immunoglobulin production in systemic onset juvenile arthritis. Arthritis Rheum. 1988;31:527.

11 Burmester GR, Horneff G, Emmrich F, Kalden JR. Immunomodulatory treatment of rheumatoid arthritis with an anti-CD4 (anti-helper T cell) monoclonal antibody. Podium presentation at the annual meeting of the American College of Rheumatology; 1990; Seattle, Wash.

12 DeNardo BS, Rhodes Nu, Gibbons B, et al Physical Therapy Standards of Care for Children with Chronic Arihritis. Boston, Mass: The Affiliated Children's Arthritis Centers of New England; 1990.

13 Kiviranta I, jurvelin J, Tammi M, et al. Weight bearing controls glycosaminoglycan concentration and articular cartilage in the knee joints of young beagle dogs. Arthritis Rheum. 1987;30:801-808.

14 Scull SA, Dow MB, Athreya BH. Physical and occupational therapy for children with rheumatic diseases Pediatr Clin North Am. 1986;33:1053-1077.

15 Scull S. juvenile rheumatoid arthritis. in: Tecklin JS. Pediatric Physical Therapy. Philadelphia, Pa; JB Lippincott Co; 1989:216-236.

16 Lechner DE, McCarthy CF, Holden MK. Gait deviations in patients with juvenile rheumatoid arthritis. Phys Ther. 1987;67:1335-1341.

17 Haley S, Faas RM, Coster WJ, et al. Pediatric Evaluation of Disability Inventory. Boston, Mass; New England Medical Center; 1989

18 Coulton CJ, Zborowky E, Lipton J, Newnan J. Assessment of the reliability and validity of the arthritis impact measurement scales for children with juvenile arthritis. Arthritis Rheum. 1987;30:819-824.

19 Rhodes X), Pumphrey KF, Zemel L. Development of a functional assessment tool for children with juvenile rheumatoid arthritis. Arthritis Rheum. 1988;31 (suppl 4):5151. Abstract.

20 Lovell DJ, Howe S, Shear E, Hartner S. Development of a disability measurement tool for juvenile rheumatoid arthritis. Arthritis Rheum. 1989;32:1390-1395

21 DeAndrade JR, Grant C, Dixon AS. joint distension and reflex muscle inhibition in the knee. J Bone Joint Surg [Am]. 1965;47:313-322

22 Koch B. Rehabilitation of the child with joint disease. In: Molnar GE, ed. Pediatric Rehabilitation. Baltimore, Md: Williams & Wilkins; 1985:233-271.

23 Jasso MS, Protas EJ, Giannini EH, Brewer EJ. Physical work capacity (PWC) in juvenile rheumatoid arthritis (JRA) patients and healthy children. Presented at the Annual Meeting of the American Rheumatism Association/American Health Planning Association; 1986; New Orleans, La,

24 Weinblatt ME, Coblyn JS, Fox DA, et al; Efficacy of low-dose methotrexate in rheumatoid arthritis. N Fngl J Med. 1985;312:818-822.

25 Harkcom TM, Lampman RM, Banwell BF, Castor CW, Therapeutic value of graded aerobic exercise training in rheumatoid arthritis. Arthritis Rheum. 1985;28:32-39

26 Isdale IC. Hip disease in juvenile rheumatoid arthritis. Ann Rheum Dis. 1970;29: 603-638

27 Ansell BM: Introduction. In: Arden GP, Ansell BM, eds. Surgical Management of Juzenile Chronic Polyarthritis. New York, NY: Grune & Stratton Inc; 1978:1-7.

28 Espada G, Alvarez mm, Gagliardi S. Longterm results of soft tissue release surgery for hips and knees in juvenile chronic arthritis (JCA). Presented at the Third international Pediatric Rheumatology Conference; March 1991; Park City., Utah.

29 Scott RD. Total hip and knee arthroplasty in juvenile rheumatoid arthritis. Clin Orthop. 1990;259:83-91.

30 Lachiewicz PF, McCaskill B, Inglis A, et al. Total hip arthroplzlsty in juvenile rheumatoid arthritis: two-to-eleven-year results. J Bone joint Surg [Am] 1986;68:502-508.

31 Ruddlesdon C, Ansell BM, Arden GP Swann M. Total hip replacement in children with juvenile chronic arthritis J Bone Joint Surg [Br]. 1986;68:218-222.

32 Gudmundsson GH, Harving S, Pilgaard S. The Charaley total hip arthroplasty in juvenile rheumatoid arthritis patients. Orthopedics. 1989;12:385-388,

33 University of Connecticut Health Center Multidisciplinary Total Hip Replacement Protocol. Farmington, conn: University of Connecticut Health Center.

34 Hizarov GA. The principles of the Hizarov method. Bull Hosp Jt Dis Orthop Inst. 1988. 8(2):1-11.

35 Rydholm U, Elborgh R, Ranstam J, Schroder A Synovectomy of the knee in juvenile chronic arthritis. J. Bone Joint Surg [Br]. 1986;68:223-228.

36 Carmichael E. Chaplin DM. Total Knee arthroplasty in juvenile rheumatoid arthritis: a seven-year follow-up study. Clin Orthop. 1986;210; 192-210, prosthetic management of children with amputations. Because most pediatric limb deficiencies are from congenital causes and because many children with congenital amputations have multiple limb involvement, these epidemiologic factors often direct clinical decisions.

Prevalence

The National Center for Health Statistics and other agencies estimate that about one tenth of 1% of the US population have a "major limb reduction" (ie, more than a missing toe or part of a finger) and that 13% of these individuals are younger than 21 years of age.4,5 Therefore, the estimated prevalence of major pediatric amputation in the United States is 32,500. Congenital loss accounts for the majority of pediatric limb reductions., however, trauma, tumor, and other diseases, in descending order, are the causes for most postnatal pediatric xnputations.(5-6)

Incidence

The only published report examining the incidence of pediatric amputations in North America was completed several years ago, so its conclusions may be outdated compared with current amputation incidence.(6) That census was limited to 4,105 children with limb deficiencies treated at specialty clinics. Thus, it is not known to what extent that census reflects the total North American population. Given these limitations, however, the rate of new cases treated by the pediatric clinics was about 17% of the total caseload, with three fifths of these 17% being children with congenital limb reductions.(6) Applying this rate to federal population estimates suggests that the United States would likely experience 5,525 new cases of childhood amputations per year, with about 3,315 of these amputations being congenital. Sampling estimate errors and childhood deaths may explain the numeric discrepancies between the incidence and prevalence data.

Characteristics

The population of children with amputations differs from the population of adults with amputations in several ways. Few childhood amputations are caused by the vascular disorders so prevalent among older persons, and, unlike adults, most children with amputations have upper-limb deficiency. In treating children, decisions should emphasize developmental function and avoidance of related residuum (formerly called the "stump") anomalies such as terminal spike and distal bone overgrowth,(1) rather than residuum care and length preservation.

Congenital anomalies are taxonomically diverse and are classified as being either transverse, in which all skeletal elements distal to the level of loss are absent, or longitudinal, in which some distal skeletal elements remain.(7) Transyerse deficiencies resemble surgical amputations, although there is no scar. Longitudinal deficiencies are more variable. For example, in fibular absence-a rather common lower-limb longitudinal deficiency--the foot may be intact, but often the fourth and fifth rays may be missing, with tarsal abnormalities and a markedly bowed tibia.

Among the most challenging lower-limb anomalies are proximal femoral focal deficiency (PFFD), which includes a foreshortened femur and a reasonably normal tibia, fibula, and foot, resulting in severe reduction in limb length, and phocomelia, in which the foot is articulated with the pelvis. In both anomalies, the pelvis is abnormally formed. Comparable longitudinal deficiencies appear in the upper limb, particularly absence of the ulna.(7)

Level of deficiency and residuum characteristics. Although most adult patients have unilateral lower-limb amputations, fully 20% of children with amputations have bilateral, quadrimembral, or trimembral reductions-many of these reductions include complex congenital anomalies such as PFFD.(6) Cosmetic considerations notwithstanding, acceptable surgical (such as the Van Nes rotationplasty) and conservative management and expert rehabilitative care can enable these children to be quite functional.(8,9) Although a given limb reduction is usually fitted prosthetically similarly to that of an adult amputation of the same level, both prosthetic and surgical options should be carefully discussed with the child's parents, and, if possible, with the child.2.3 Conversion of a congenital anomaly to a more proximal amputation through surgery should be undertaken only after all members of the clinic team have participated in the decision. Consideration should be given to the potential limb length, current and future prosthetic options, and cosmetic and functional expectations of the child and family. Rarely are postsurgical joint stiffness and healing major factors., children virtually always regain full joint mobility, and, because few such residua have vascular impediments to healing, wound recovery is usually prompt.

Of the children most frequently seen in pediatric amputation clinics, most have left upper-limb deficiency, and most of these children have below-elbow reductions.(6) Nearly one quarter of all children with amputations have below-elbow deficiencies; all except 13% of these children have had congenital anomalies (Fig. 1). Therefore, physical therapists who work with such children should become familiar with upper-limb treatment options.(6)

Approximately 7% of all children with amputations have three or more limb reductions. Children with congenital amputations and those with very early surgically acquired, bilateral upper-extremity amputations often learn to feed and otherwise care for themselves by developing "prehensile toes" (as one of our patients referred to his ability to use his feet as most of us use our hands). Children with trimembral amputations, and especially those with quadruple amputations, however, invariably need special assistive devices. Technology is rapidly enhancing the ability of these individuals to be more independent. Nonspecialist therapists should seek assistance from pediatric amputation centers to help these patients achieve their full potential with current technological options.

If the residuum contains vestigial articulations and epiphyseal growth plates, it is critically important to observe the distal end for bone overgrowth. Bony overgrowth can eventually cause skin penetration in cases in which the initial symptoms (eg, sharp and clearly located pain) and signs (eg, a palpable and irregular stiff spike immediately subcutaneous to the distal-most surgical flap or congenital residuum end) are ignored.

Controversy exists concerning the utility of firm prosthetic contact, such as that provided by distal end-bearing sockets, for treating or preventing bony overgrowth. Recent evidence suggests that appositional bone growth from the periosteal residuum is a causative agent in both congenital and acquired (postpartum) osseous spikes." Until conclusive evidence emerges on the effects of mechanical stimulation in general, and distal contact in particular, on the genesis of residuum overgrowth, we believe that end-bearing and mechanical loading of the remaining epiphyses contribute to the long-term well-being of the residual limb.

Age. About two thirds of all pediatric limb reductions are due to perinatal (usually congenital) events. The remaining third occur over the first two decades of life. About half of all children seeking care for the first time at a typical amputation specialty clinic are less than 3 years old. About 16% of children with congenital amputations do not receive such care until they are about to begin school.6 We believe clinicians need to be more proactive in identifying young patients who may benefit from early prosthetic fitting.

Gender, side, and etiology. more boys (59%) than girls (41%) comprise the population of children with amputations, regardless of age. Overall, most limb reductions occur on the body's left side (Fig. 2); this sinistral predilection can be explained in part by congenital teratologic side-specificity.(11) Teratogens also probably explain the excess peril to boys prenatally,(12,13) but excessive risk-taking and poor parental supervision account for many gun-related, farm,(14) and railway(15) acquired amputations. Such data highlight the important role physical therapists can play in preventing accidental amputations.

Boys account for well over half of amputations in which the etiology is cancer, including the most common carcinoma among children: osteosarcoma.(16) Most carcinoma-related limb reductions occur in the second decade of life. New treatment methods can be used to address the desire of appearance-conscious adolescents for nonprosthetic limb salvage. Options include en-bloc resections, in which a major portion of the femur is replaced with an endoprosthesis, and rotationplasty.(9) En-bloc salvage procedures would seem to offer superior appearance, because the natural external shape and size of the thigh is retained, and reasonable performance, because the natural knee is preserved. Few data, however, have been reported to support this supposition. Indeed, the operative risks attendant to pediatric surgery should always be a part of the many considerations guiding surgical management options.

Prostheses and Prescription Options

Children's prosthetic needs are dictated by their small size and physical and psychosocial growth and development. Parental concerns also affect prosthetic prescription. Some families are primarily interested in disguising the amputation, particularly upper-limb reductions. Personal financial resources play a key role in prescription, especially when private insurance and public funding are meager. A list of commercial vendors of prosthetic components is provided in the Appendix.

Lower-limb Prostheses

Children with acquired below-knee amputations or congenital transverse deficiencies at the below-knee level wear prostheses having four elements: (1) a foot-ankle assembly, (2) a shank, (3) a socket, and (4) a suspension. A modified below-knee prosthesis is also used by patients with some congenital longitudinal deficiencies, such as fibular hemimelia, which are functionally comparable to below-knee amputations.(17)

Ankle-foot assemblies. Infants are given flat solid-ankle cushion heel (SACH) assemblies[.sup.*,[dagger]]; this simple foot provides a stable standing base and, when the infant walks, flat initial contact. By 1.5 years of age, nearly all nondisabled toddlers can contact the floor with the heel(18,19) and progress with a heel-toe transition; with a basic prosthetic foot, the pediatric amputee should be able to perform similarly.

The smallest commercial prosthetic foot is 9 cm (3.5 in) long, equal to a juvenile size I shoe, and is suitable for an infant approximately 4 months old. This foot has an external keel and a smooth distal border. The 10-cm (4-in) SACH assembly is available with an internal keel and molded toes. The SACH feet are durable, inexpensive, and lightweight. The smallest Syme's model of the SACH foot is somewhat longer (14 cm [5.5 in]).

For school-aged children and adolescents, energy storing-releasing feet are used. They have flexible keels, which bend upon loading in early stance to store energy and recoil in late stance to release energy. This feature affords the wearer more spring when walking, running, and jumping. The smallest components are 12 cm (4.7 in) long; they are the "Stored Energy" (STEN)* (Fig. 3), Flex-foot[double dagger] (Fig. 4),20 and Flex-walk[double dagger] (Fig. 4) feet. The new split-toe option of the Flex-foot greatly improves maneuvering over rough terrain and hills. The smallest child's model of the Seattle LightFoots[Section] (Fig. 5) is 13 cm (5 in) long, and that of the Springlite~~ is 14 cm long.

Initially, a prosthetic foot may be aligned anteriorly relative to the socket to promote stability. As the wearer gains confidence, the alignment can be altered to favor mobility.

Shanks. The remainder of the below-knee prosthesis is custom-made, regardless of the size of the patient. The shank, connecting the foot and the socket, may be an endoskeletal or exoskeletal device. Exoskeletal devices are usually carved from wood and then covered with a protective plastic coating. Exoskeletal constructions are relatively inexpensive, resist abrasions, are durable, and cannot be saturated by urine from young children who are not toilet trained. An endoskeletal shank consists of an aluminum and steel, or ultralightweight carbon-fiber or titanium, central pylon. Endoskeletal devices are covered with foam rubber shaped to match the contralateral limb; the rubber may not be durable enough for children who engage in vigorous games.

Below-knee Sockets. These sockets are molded of rigid plastic, usually polyester laminate, with a liner of polyethylene foam. The socket should contact the entirety of the amputation limb to maximize sensory feedback and to ensure that moderate pressure is applied to the distal end of the residuum, thereby preventing circulatory and dermatologic disorders. Even in the presence of skin grafts, children can generally tolerate more distal loading than can adults.(21)

The socket must be aligned in greater abduction and flexion to complement the infantile gait pattern, which lacks bimodal knee flexion/extension movement until the child is 2 years of age.18 Young children with amputations walk with excessively flexed knees and hips during the stance phase.19 Prostheses for toddlers should be aligned to accommodate the wide walking base and external hip rotation that persist in children until they are 3 years of age.(19)

Below-knee prosthetic suspensions. Suspension can be achieved in several ways. A baby may require a supracondylar cuff joined to an anterior elastic strap, which, in turn, buckles to a waist belt or harness. The strap maintains the prosthesis in place even when the wearer reverts to crawling. Some toddlers need a harness to counteract attempts at unsupervised undressing. As the skeleton matures, the femoral epicondyles become more prominent, making supracondylar or supracondylar/suprapatellar suspensions good choices. The supracondylar/suprapatellar suspension, in particular, decreases pistoning and enhances gait stability, even for children with an absent fibula or a short residual limb.(22) The one-piece construction eliminates the risk of losing the supracondylar device's medial suspension wedge.(23) Teenagers often opt for an elastic sleeve in place of the cuff or to augment supracondylar suspension; the sleeve provides suspension that is advantageous during athletics and creates a smooth silhouette under snug clothing. Suction, particularly with the Silicone Suction Socket, maximizes security. The wearer rolls a silicone sock over the amputation limb. The sock has a distal metal ring that is secured to the medial and lateral socket walls with a transverse rod.(24) Thigh corset suspension is rarely necessary, unless the anatomic knee is unstable or the child has Van Nes rotationplasty.(9,25)

Above-knee components. The above-knee prosthesis has five components, two of which-the foot and the shank-are essentially the same as for the below-knee appliance. Recently introduced endoskeletal shank systems include the Otto Bock aluminum and steel endoskeletal modular system[dagger] designed for children 2 to 14 years of age who weigh less than 40 kg (99 lb). The Endolite system,# manufactured of carbon fiber and titanium, is lighter, but appreciably more expensive, than the aluminum and steel components.

Knee units. An infant's first prosthesis may lack a knee unit and have an ischial bearing socket mounted on a pair of side bars above a foot.(26) The creative prosthetist can fashion a pair of plastic overlap knee hinges or use small hinges intended for a below-elbow prosthesis.

For school-aged wearers, knee units, with or without a stabilizing mechanism, are manufactured as small as 24 cm (9.5 in) in circumference at the calf, with the knee bolt 6.5 cm (2.5 in) wide. Active preteenagers and adolescents often use hydraulic and pneumatic knee units, in which frictional control of knee swing adjusts automatically to changes in walking or running velocity.

Above-knee sockets. Plastic total-contact sockets are essential for virtually all children with amputations, whether infants in diapers or adolescents who perspire profusely while engaged in strenuous activities. "Ischial socket contours," which have a narrower width than in the anteroposterior dimension, are used by many children, whereas other children use quadrilateral sockets, which are narrower in the anteroposterior dimension. Regardless of shape, the flexible polyethylene socket in a carbon-fiber reinforced frame is appropriate for children.(27) The thin socket is comfortable, dissipates heat well, yields to the chair seat when the wearer sits, and conforms to changes in muscle contour when the wearer walks. In addition, the flexible polyethylene clings to the skin to enhance suspension and is translucent, enabling clinicians to visualize fit. The socket is very easy to adjust to accommodate for growth.(27)

Above-knee prosthetic suspensions. Very young patients usually need a combination of suction and some form of auxiliary suspension, typically a Silesian bandage, to retain the prosthesis on soft subcutaneous tissue. Toddlers may require the continued use of a Silesian bandage to aid reapplication of the prosthesis when there is accidental loss of suction. By age 6 years, children can use full suction suspension, which eliminates the necessity of straps around the torso.(25) Although some clinicians do not recommend total suction suspension until the patient is 14 years of age, believing there may he problems with independent donning,(28), in our experience, children as young as 5 years of age can manage prostheses with flexible sockets, suction suspension, and a Silesian bandage.(27)

Prostheses for children with congenital deformities. Children with hip anomalies, such as PFFD, often require a funnel-shaped socket to provide full contact. The distal end of the socket is similar to that for the child with surgical amputation. If the foreshortened PFFD limb has not been revised surgically, however, the socket may accommodate the anatomic foot. The anatomic foot is plantar flexed to minimize the bulkiness of the thigh section of the prosthesis. Ordinarily, a Silesian bandage is attached. The patient with unilateral phocomelia can wear a modified hip disarticulation prosthesis or, depending on the strength of the foot, a variant above-knee prosthesis; in both instances, the foot aids suspension and prosthetic control.(26) Several youngsters with bilateral amelia or phocomelia or with sacral agenesis have been fitted successfully with a modified reciprocating gait orthosis.(29)

Adjustments for growth. Adjustments growth should be anticipated at the time of prosthesis prescription to extend the life of the prosthesis and reduce both expense and office visits, which disrupt school and home routines. In addition to these adjustments, children's prostheses are replaced more frequently than those for adults because the young wearers' greater activity takes its toll on movable components and the external finish. Growth occurs in spurts, particularly in infancy and in preadolescence, necessitating a new prosthesis every 1 to 2 years.(30) Persistent redness of the skin and a tissue roll over the edge of the socket are signs of tightness. Maintaining the prosthesis equal to the length of the sound limb is important to prevent the patient from walking with excessive lateral trunk bending. Purchasing larger shoes so that the sound foot can be fitted properly is another event signaling the need to revise the prosthesis. For the individual with bilateral amputations, the impetus for lengthening prostheses often comes from the youngster's concern about conforming to one's peers or being taller than younger siblings. Frequent inspection of the amputation limb is required to determine when adjustment or a new prescription is needed. We recommend quarterly clinic visits for young patients, although in our experience, adolescents generally do not require more than semiannual examination.

Approximately a third of lower-limb growth occurs at the proximal femoral epiphysis(31); consequently, most of the limb length will be below the socket. Exoskeletal shanks can be lengthened by prosthetists, who section the shanks near the ankle unit and insert shims of appropriate thickness. Ordinarily, the girth of the shank is not increased. Annual calf growth is normally only 0.3 cm for children between 2 and 6 years of age and 0.2 cm for 7- to 10-year-olds.(32) Endoskeletal shanks are lengthened by substituting longer piping for the outgrown pylon. By the time the shank needs lengthening, a new cosmetic cover will be required to replace the old, often damaged, cover. Alternatively, for both types of shank, a 1-cm (0.5-in) insert can be placed in the shoe on the prosthetic side.

Socket length and circumference can be increased in several ways. When the prosthesis is fabricated, two concentric sockets can be nested in the outer socket, with the inner sockets removed as the amputation limb grows. Extra liners can be used in the same manner. Both approaches, however, require that the new prosthesis be relatively bulky. in addition, because the amputation limb does not grow uniformly, the final socket version may not fit precisely. A socket with an adjustable section held by several straps33 is another option. The ideal arrangement involves a flexible socket that can readily he modified. The polyethylene above-knee socket can be heated to be reshaped as much as 4 cm 1.5 in) on the lateral aspect. The rigid frame is constructed with a thick lamination proximally and 2-cm (0.7-in) polyurethane filler distally. The prosthetist can grind away material as the child grows.(27)

Prosthetic foot size should keep pace with the anatomic foot, which for children 4 to 14 years of age, grows approximately one size per year.(32) The ipsilateral knee will be more difficult to flex if the child walks with a small prosthetic foot inside a large shoe.

Upper-Limb Prostheses

Most upper-Limb prostheses include a terminal device (TD), which replaces the missing hand, a wrist unit, and a custom-made socket. Depending on the mode of TD control, the prosthesis may also include an individually fashioned harness and a cable system.

Terminal devices. The tiniest TD resembling a hand is the passive mitten" (Fig. 6), 8.6 cm (3.4 in) in circumference across the "metacarpophalangeal joints," suitable for infants approximately 6 months old. Although it disguises the amputation, the pink or brown mitten does not provide for unilateral prehension, nor does it allow the baby to stabilize the limb on a crib rail to pull up to stand. The device is replaced when the child is ready for active prosthetic control.(34) The smallest passive hand is 10.2 cm (4 in) in circumference at the knuckles" and is covered with a glove, manufactured in nine colors approximating skin tones.

The smallest voluntary-opening, cable-operated hand is 5 cm (2 in) wide, intended for children 2 to 6 years of age.(35) At the hand's minimum spring setting, it does not close tightly over paper; at its maximum setting, pinch force is so great that the wearer must exert substantial force on the harness to open the hand.36

Children 18 months of age have been fitted with myoelectrically controlled hands[double dagger] (Figs. 7, 8), 5 cm wide,37 although there is no convincing support for fitting prior to 2.5 years of age.(38) This battery-powered component eliminates the need for harnessing and provides greater grasp force than do cable-operated hands, but the myoelectric unit is heavier and more fragile than passive or cable-operated TDs. A very small child will have to wear the battery in a chest or waist hoister if space in the forearm shell between the distal end of the socket and the proximal end of the hand is insufficient to house the battery. Some patients with very short residual limbs complain about the distal weight. All prosthetic hands, whether myoelectric or body-powered, require a glove, which matches the wearer's skin color and protects any mechanism in the component. The gloves tear and stain easily and require constant upkeep.

Hook TDs enable infants to appreciate bimanual prehension. When the toddler is 12 to 15 months old, a cable is added to the TD to prepare for independent activation.39,40 The smallest voluntary-opening model-the Hosmer Dorrance infant's 12P hook" (Fig. 9)-is 7 cm (2.7 in) long and has a pink or brown plastic covering. Pinch force is regulated by the number of rubber bands worn on the hook; increasing closing force increases the harness force needed to open the hook. The smallest voluntary-closing hook, the Anatomically Designed-engineered Polymer Technology (ADEPT) hook*$ (Fig. 10), is tan and of similar size to the Hosmer Dorrance infant's 12P hook; grasp depends on the amount of force exerted on the cable at the harness. One report(41) Suggested that fitting a voluntary-closing hook at 6 months of age improves gross motor development.

An intermediate TD design provides the prehensile capability of a hook with a more anthropomorphic contour. one such device-the Child Amputee Prosthetics Project (CAPP) voluntary-opening TD** (Fig. 11)--is manufactured in two sizes: The smaller device is 0.5 cm longer than the smallest hook and is available in brown and pink. Other options for young patients include electric switch-controlled hands and hooks** (Fig. 12) and an electric prehension actuator, which can be used with any voluntary-opening TD.

Wrist units. The TD is screwed into a wrist unit, which provides passive pronation and supination. Two commercially available units** also enable the child to palmar flex the TD.

Proximal components. The socket, harness, and cable system of prostheses are custom-made. The flexible polyethylene socket in a rigid frame has been used for all levels, from wrist disarticulation to above-elbow, although older-style laminated sockets are also popular.(42)

Above-elbow prosthetic components. The above-elbow prosthesis is composed of a TD, wrist unit, forearm section, elbow unit, socket, harness, and cable system. The forearm section may be custom-made or manufactured as part of the elbow unit. The smallest elbow units are 5.1 cm (1.5 in) in diameter. The simplest model has a fraction joint, whereas the more complex type has a cable that permits locking the elbow at the desired angle. Electric switch-controlled elbows** (Fig. 13) manufactured for toddlers respond to minimum force on a push or pull activator, but are appreciably heavier than simpler models.(37)

Adjustments for growth. Progressively larger TDs and forearm sections must be supplied to maintain symmetry between the amputated and intact limbs. Wrist units are available in small, medium, and large sizes. The below- or above-elbow socket can be enlarged in ways similar to those used with lower-limb prostheses.42-44

Other Appliances

The pediatric patient may benefit from other appliances at various ages. Toddlers with unilateral lower-limb amputations generally manage independent walking, but, like nondisabled toddlers, may ride in a stroller to traverse long distances. The child with bilateral lower-limb deficiencies may need to use a wheelchair until proficiency with the prostheses is gained, or when prostheses are being repaired or the child has medical problems that interfere with walking. Although individuals with complete absence of both lower limbs can be fitted with a pair of prostheses or with two artificial limbs joined to a pelvic socket, the energy cost of ambulation is so great and walking is so slow and awkward that, by adolescence, most of these individuals will opt for a wheelchair. Children with quadrimembral limb deficiencies usually depend on special crutches(45) and adaptive equipment for daily activities.(46)

We believe prostheses designed for recreation should be part of the child's equipment. Many pursuits, such as bicycling and running, do not necessitate special prostheses. Children with unilateral leg amputations generally ski without a prosthesis, using ski poles with small rudders at the end. Similarly, most of these children swim without prostheses; however, waterproof prostheses are useful at the beach or water's edge. Several recreational TDs (Fig. 14) are manufactured, including the Super Sport flexible mitt" for gymnastics and appliances for bowling, holding ski poles, using a baseball mitt, and managing golf clubs and cameras.

Prosthetic Training

The fundamental differences between adults and children with amputations-growth, responsibility, and functional needs-make pediatric prosthetic training a complex process of assessment, training, and intermittent upgrading of functional activities suited to the child's stage of development. The rehabilitation process must also focus on maintaining prosthetic durability and function.(47)

Role of Parents in Rehabilitation

Whether the patient is born with a limb deficiency or acquires an amputation during childhood, successful pediatric rehabilitation requires the intensive interaction and cooperation of parents.(41) Parental attendance at and participation in therapy sessions are vital to the child's welfare. Parents should be taught how and when to assist their child, and they should be provided with a home program that enhances prosthetic wearing tolerance and emphasizes activities important to fostering prosthetic use.(41,49) Rehabilitation also includes teaching parents to operate, care for, and maintain the prosthesis.(28,47,50) Parents of children with acquired amputations should be provided a postoperative and preprosthetic program of residuum care and exercises for maintaining range of motion (ROM) and strength.(28,47)

Therapists must incorporate parents' needs and expectations in order to establish trust and a reciprocal working relationship.(51) Providing accurate and current information to the family about the child's prosthesis and prosthetic management will enable the family to participate constructively in decision making and to encourage prosthetic use at home.(52,53)

Age of Fining

Lower limb. Developmental readiness is an important consideration when fitting children with lower-extremity amputations, There is little functional need for a prosthesis prior to standing at about 6 months of age.(28) The first fitting generally occurs when the child shows interest in pulling to a standing position (ie, at about 6-9 months of age), which is presumed to assist the developmental progression toward independent ambulation.(28,47,49,54)

Children with bilateral lower-limb deficiencies move independently, using their arms and residual limbs. Low "stubbies" (short, nonarticulated pegs with rocker-bottom distal ends), however, aid postural stability for upright ambulation. By increasing the height of the stubbies as balance improves (47,50) the child is encouraged to perform standing activities.(47)

Children with an acquired amputation can be fitted with a prosthesis immediately following wound healing for effective early rehabilitation. The temporary pylon equalizes leg length and allows gait acquisition to proceed.(47,50)

Upper limb. Although many authories(47,49,51-53,55,56) advocate early prosthetic fitting to improve prehensile skill and prosthetic acceptance, recent studies' indicate no relationship between early fitting and prosthetic skill or spontaneous use of prosthetic devices among children with unilateral below-elbow amputations. A better rationale for early fitting is that it enables the infant to use both arms for gross motor developmental tasks, such as prone propping, creeping, coming to sitting position, and pulling to standing position, and consequently that equalizing limb length should provide a functional advantage for development.4(47

tting should commence when independent sitting is

this stage of development, about 6 to 8 months of age, nondisabled children begin to use their hands to explore and manipulate objects. Although the infant is too young to learn to operate a TD,(28) a passive device to hold objects inserted by an adult will draw the child's attention to the prosthesis (Fig. 15).(28,47,48,52,53)

Children with bilateral upper-extremity amputations, although delaved in achieving developmental milestones (51,60)learn to compensate with the head, trunk, and legs to attain gross motor skills such as sitting and pulling to a standing position.(52) Because foot function is essential to their development,"(55) prosthetist spebcialists generally agree to fit these children later than children with unilateral amputations, based on their needs and abilities and when a prosthesis can he used effectively for light grasping activities.(28,48,52)

Determination of the readiness of children with unilateral or bilateral amputations to use a cable-activated or myoelectric TD is based on the following criteria: the ability to follow two-step directions, an attention span of 5 to 10 minutes, an interest in bimanual activities, and the ability to perform the TD control

Children with multiple limb deficiencies present greater complexities for rehabilitation. Each child's development readiness and abilities, as well as functional need for prostheses, should guide the timing of fitting the first prosthesis.(28,48,52)

Developmental Approach to Treatment

Because treatment should be coordinated with normal growth and development (28,47-49,52) some relevant developmental skills from birth to 6 years of age that can be incorporated into the intervention services are listed in the Table.

Preprosthetic program. Observation of the infant's level of development and the interaction between parent and child during the therapist's initial evaluation enables the therapist to plan activities suited to the child's age and stage of development, regardless of site and level of limb deficienCy.(28,52) A preprosthetic program consisting of ROM activities to increase mobility of the limbs and trunk, a general strengthening program using positioning to assist the child in maintaining postural stability against gravity, and stimulating equilibrium reactions to foster the balance required for trunk control will help prepare the child for maximal independent functioning with and without a prosthesis.(48,60) Development activities, including rolling, crawling, kneeling, standing, and falling, can be incorporated into a mat exercise program.(47) Following visits at regular intervals prior to the initial prosthetic fitting will enable the therapist to monitor the child's development and prepare the child and family for a prosthesis.(28,51,52,60)

For children with bilateral upper-limb deficiency, treatment typically focuses on opportunities for tactile exploration. Although the child will develop tactile awareness through the feet without formal training, this skill can be enhanced by keeping the feet uncovered, placing toys on the floor for grasping by the toes, and providing a variety of objects for learning to differentiate different textures, shapes, and weights (Fig. 16). (55,59)

Common principles. Once the definitive prosthesis is received by a child, both a mechanical and a functional evaluation should be completed to determine whether the prosthesis fits correctly and the components work properly and to ensure that the materials and construction are satisfactory.(28,51,52) Treatment often consists of games and play activities to stimulate use of the prosthesis.(47,48,56,59) initial training: lower limb. Training children with lower-extremity amputations through play activities in an open area with tables and chairs of varying heights encourages getting up and down from the floor, "cruising" (walking along furniture), and standing and playing. toys that require bimanual manipulation at various positions on a table allows the child to develop weight shifting and balance in standing (Fig.

Gait training requires knowledge of the gait pattern typical of the child's age. nondisabled child demonstrates characteristic gait differences from year to year, with an adult pattern evidenced by age 5 years.18 Exercises that are believed to activate innate motor patterns appropriate for the age-specific gait pattern expected should be incorporated into the training program.(62) Many children with below-knee amputations, both unilateral and bilateral, do not require assistive devices for independent ambulation however, initial training with crutches using a four-point gait enables the therapist to emphasize a reciprocal gait pattern and heel-strike at foot contact.(18-47) For the very young child, doll carriages and pushcarts can be used temporarily as ambulatory aids.(28,47)

Initial training: upper limb. Initial training of TD activation for the child with an upper-extremity amputation focuses on teaching the child to open the hand or hook TD. If the prosthesis is cable operated and has a voluntary-opening TD, the therapist places an object in the TD, then passively moves the child's arm to open the TD (Fig. 18).28 This is followed by helping the child open the TD actively, close it on an object, and then release the object(28,49,52) For the voluntary-closing TD, the therapist first moves the wearer's arm to put tension on the cable to close the hook. The child is then guided to close the TD actively on an object and relax to open the book. When the child successfully learns the control motions of the TD, training is directed toward activities of daily living.(50) All activities should promote a problem-solving approach through trial and error.(28,52) Developmentally appropriate activities combining play and the activities of daily living should provide the child with a variety of gross and fine motor exercises.63 Allowing the child ample time to perform the activity, assisting only when necessary, and avoiding fatigue should stimulate spontaneous and functional use of the prosthesis.48 The child with an above-elbow prosthesis must also be provided with reaching and placing activities that require active elbow positiotiing.(28,49,52)

Although the young child with bilateral deficiencies can learn activities-of-daily-living skills, independent prosthetic performance may be limited.(47.48,55) Training that combines use of the feet and the prostheses will help maximize independence (Fig. 19).- The preschooler often needs assistive clothing and toileting and other hygienic devices to enhance functional independence.(47,48) With the absence of arms for protection, the child needs to learn to fall safely.

Continued training considerations. with growth and maturity, higher-level purposeful activities are incorporated into the program. Functional needs change with age. Play activities, such as painting, tool use, paper cutting, and simple games with cards or checkers, improve the basic upper-extremity motor skills. Lower-extremity games that incorporate kicking, walking backward and sideways, purposeful walking, climbing, and running enhance functional ambulation.28,47,49,50,54 Training should eventually incorporate all adolescent and adult activities of daily living (eg, tying a necktie, fastening a brassiere) and vocational skills.

Summary

Physical characteristics determine the range of prosthetic management options available to each child, and epidemiologic factors are important in allocating health resources. Experienced multidisciplinary treatment teams are required to treat all except those children with the least complex limb reductions. Current surgical management, prosthetic fitting options, and training regimens have progressed substantially over the past several decades, and clinicians involved in the care of children with amputations should be familiar with recent technological options to provide the best possible care. The plethora of small components available for lower- and upper-limb prostheses emphasizes the desirability of managing juvenile patients in an interdisciplinary clinic setting where the relative advantages and disadvantages of prosthetic options can be related to the needs and wishes of each young patient. Although prostheses may never be the avatars of normal limbs, they can enhance the child's functional abilities. Prosthetic training, coordinated with normal growth, parental participation, and developmental expectations, can enable the young patient to achieve maximum function.

Well-founded advice is probably the most important contribution clinicians provide to children with limb deficiencies. With well-fitting prostheses, many children will teach themselves the functional skills they need to provide their current needs-. Experienced clinicians, however, can offer advice on how current choices affect future function.

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