INTRODUCTION

Juvenile rheumatoid arthritis (JRA) is a disabling condition that affects nearly a quarter of a million children in the United States.^3,45 It has been defined by the American Rheumatology Association³ as arthritis that has been present in at least one joint for 6 weeks to 3 months when the patient is younger than 16 years old. Patients with this affliction often present with stiff, painful elbows, and some may have disabling elbow ankylosis. This condition has implications regarding treatment. Just as the clinical manifestations of JRA may differ from those of adult rheumatoid arthritis in the hip, knee, and shoulder,^26,28,45 the course of this disease process also differs in the elbow.²⁰ Patients often have severe osseous atrophy, deformity, and soft tissue contractures about the elbow that require special consideration and may adversely affect treatment outcomes.^{8,9,11,14,15,20,21} We have also reconstructed extremely small joints and narrow intramedullary cavities.

Most patients with JRA who present with stiff, painful elbows have had prior treatment of other symptomatic joints and are functionally limited to only activities of daily living.⁶ The painful impairment of elbow function may have been overlooked by the patient or minimized by the physician because of the severity of other joint involvement as well as a general lack of awareness regarding treatment options and results. In one study, 15 of 19 patients with JRA had had an average of five (range, two to eight) previous arthroplasties or arthrodeses of other major joints (e.g., hip, knee, shoulder) before presentation for total elbow arthroplasty.⁶

EVALUATION

As with all inflammatory conditions, clinical assessment of patients with JRA must include a detailed evaluation of the ipsilateral shoulder, wrist, and hand. The assumption that shoulder motion compensates for loss of elbow flexion and extension is invalid (see Chapter 5). Thus, range of motion of the shoulder and elbow should be carefully and independently assessed. Although some controversy exists regarding the ideal order of surgical management in patients with multiple joint involvement, the most painful and functionally disabled joints should have priority.¹⁸ In the event that all joints are equally symptomatic, the next priority is usually given to the hand and wrist, because the function of the shoulder and elbow is to place a functional hand in space.

There are differences of opinion if the decision focuses on the priority of shoulder or elbow arthroplasty. Although one study suggests that elbow arthroplasty should have precedence over shoulder arthroplasty,¹⁶ others believe that shoulder arthroplasty should be performed first.³⁸ Suggested reasons for proceeding with shoulder arthroplasty before elbow arthroplasty include the following³⁸:

Our opinion has changed since we demonstrated in our surgical practice that greater functional benefit derives from the elbow replacement.¹⁸ This is shown by a markedly extended period before a patient requested to replace the shoulder after the elbow compared with requesting the elbow replacement after the shoulder is done first. Therefore, if the elbow had been replaced first, sufficient improvement often exists, so the shoulder operation can be delayed for a larger period than if the shoulder is replaced first.

The radiographs that are essential for evaluation of the JRA elbow are the anteroposterior and lateral views. The radiographic presentation of the JRA elbow has five distinct stages^6,32: grade I indicates no radiographic changes with the exception of osteopenia associated with active synovitis; grade II, symmetric narrowing of the joint space with the architecture intact; grade III, alteration of the architecture of the joint that may be relatively mild (type A) or extensive (type B); grade IV, gross destruction of most or all of the articular architecture. A grade V, radiographic change was defined if a review of the JRA radiograph revealed an absence of an identifiable ulnohumeral joint on anteroposterior and lateral radiographs or by mature bony trabeculation crossing the ulnohumeral joint (Fig. 55-1). If severe bony atrophy is present, calibrated markers may be necessary for preoperative templating purposes.

FIGURE 55-1 The type V radiographic change with rheumatoid arthritis is one of ankylosis demonstrated by complete trabeculation across the joint. Anteroposterior (A) and lateral (B) views.

TREATMENT ALTERNATIVES

Open communication between the rheumatologist, orthopedist, and physical therapist helps provide ideal care for these patients. The nonsurgical management of JRA differs little from that of adult rheumatoid arthritis. Improved results appear to be occurring from the use of disease-remitting agents (see Chapter 74). Efforts should be geared toward pain relief and especially maintenance of joint motion. Elbow splinting is unpredictable. Resting or night splints can be helpful. Judicious use of corticosteroid injections in the elbow joint may also be helpful for early symptoms. Gentle exercises should be performed regularly for the all important goal of maintaining mobility and muscle strength.

When nonoperative management fails to provide adequate relief of symptoms, surgical treatment alternatives include surgical or clinical synovectomy, interposition arthroplasty, and joint replacement. The relative roles of synovectomy and interposition arthroplasty of the rheumatoid elbow are discussed in Chapters 68 and 69 of this text. As previously emphasized, elbows with JRA have a propensity toward stiffness. Thus, if a synovectomy or interposition arthroplasty is proposed, concomitant anterior and posterior capsular releases may be necessary to re-establish functional range of motion. This was recently reaffirmed by a series of 24 synovectomies for JRA patients. The outcome was considered satisfactory in 72% at 5 years.³⁰ Of importance was that motion did not improve in these patients. On the other hand, a more aggressive approach to débridement and removal of capsule and bony impingement might improve results, as was shown in the Mayo experience.⁷

If the rather stringent indications for synovectomy and interposition arthroplasty are not met, elbow arthroplasty is recommended.