Juvenile idiopathic arthritis

To consolidate terminology for classifying chronic childhood arthritis, the International League Against Rheumatism adopted the term juvenile idiopathic arthritis (JIA), which encompasses the diseases previously known as juvenile rheumatoid arthritis and juvenile chronic arthritis [111]. In patients who have JIA, the wrist is a very commonly involved joint, second only to the knee, and is affected in approximately 54% to 59% of patients [112,113]. Initial radiographic

Scaphoid Ischemia

Fig. 13. (A-C) Preiser's disease (scaphoid ischemic necrosis) in a patient who has VACTERL syndrome anomalies, including scoliosis, anal atresia, tracheoesophageal fistula, and thumb hypoplasia. (Fig. 13C from Light TR. Injury to the immature carpus. Hand Clin 1988;4(3):421; with permission. Case provided by Neil Marcus, MD, Detroit, Michigan.)

Fig. 13. (A-C) Preiser's disease (scaphoid ischemic necrosis) in a patient who has VACTERL syndrome anomalies, including scoliosis, anal atresia, tracheoesophageal fistula, and thumb hypoplasia. (Fig. 13C from Light TR. Injury to the immature carpus. Hand Clin 1988;4(3):421; with permission. Case provided by Neil Marcus, MD, Detroit, Michigan.)

changes include premature ossification of the carpus, narrowing of the intercarpal joint spaces, and early closure of the distal ulnar physis (Fig. 14A,B) [114]. The resulting negative ulnar variance is associated with ulnar translocation and ultimately dislocation of the carpus, described by Chaplin [112] as the ''bayonet deformity.''

Treatment for JIA consists mainly of drug therapy designed to attenuate the inflammatory response. Aspirin, NSAIDs, gold salts, metho-trexate, hydroxychloroquine, penicillamine, and systemic corticosteroids are all currently prescribed [115]. In a study by Harel and colleagues [116], methotrexate improved wrist symptoms in 74% of patients, 65% of which showed actual improvement in carpal length on radiographs. In wrist disease that is unresponsive to medication, steroid injection is generally effective for reducing synovitis [114]. Synovectomy has also been shown to improve symptoms of boggy swollen joints, but should not be performed in patients who have minimal synovial thickening where fibrous anky-losis is already present [117]. Distraction lengthening of the shortened ulna has been proposed as a method of decreasing ulnar translation of the carpus [114]. Although the procedure has been shown to correct pre-existing deformity, its long-term effectiveness is not yet known.

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