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Jeune syndrome, or asphyxiating thoracic dysplasia, is an autosomal recessive osteochondrodysplasia. Four forms of Jeune syndrome have been proposed: lethal, severe, mild, and latent. In the severe form, respiratory failure leads to death in early infancy. We present 2 cases of a mild variant of Jeune syndrome in a 14-year-old girl and her 9-year-old brother, who were referred to us because of characteristic concave deformities of bilateral middle-lower chest walls without cardiopulmonary distress or renal failure. In addition, both showed short statue (-2.1 and -2.5 SD), progressive retinal dystrophy, and metaphyseal dysplasia (cone-shaped metaphysis and metacarpal brachydactyly). The chest wall deformity was treated at the age of 9 years in the sister and at the age of 7 years in the brother. According to the Nuss procedure, 2 bent bars were inserted into the thoracic cavity from each lateral intercostal space at the midaxillary line and pulled out over the lower end of the sternum. The ends of inserted bars were fixed to soft tissue over the sternum with nonabsorbable sutures. Conjoined costal cartilage around the sternum restricts the number of bars that can be positioned. A single bar for deformity of each side could not achieve complete reconstruction, but the patients and their parents were satisfied with the results cosmetically.

FROM THE ORIGINAL ARTICLE BACKGROUND: Three previous studies have investigated the long-term outcome of radial osteotomy in the treatment of Kienbock disease. However, none used patient-based assessment. The purpose of this study was to investigate the long-term clinical and radiographic outcomes of this osteotomy, including the subjective evaluation of the patient with use of the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. METHODS: A DASH questionnaire was sent to nineteen patients with Kienbock disease who had undergone a radial shortening osteotomy, and thirteen replied. The mean age at the time of surgery was thirty-nine years. On the basis of the Lichtman classification, six patients had stage-II, four had stage-IIIA, and three had stage-IIIB disease. Prior to surgery, ulnar variance was positive in six patients, neutral in four, and negative in three. The mean duration of follow-up was twenty-one years. Clinical evaluation, including calculation of the modified Mayo wrist score, and radiographic evaluation were also performed on twelve of the thirteen patients. RESULTS: The mean DASH score was 8 points (range, 0 to 23 points), and patient satisfaction was high. Compared with the findings in the contralateral wrist, the mean range of motion was 81% in flexion and 82% in extension and mean grip strength was 88%. The mean modified Mayo wrist score was 83 points, and the clinical results were excellent in six patients, good in five, and moderate in one. The DASH scores tended to be worse in patients with Lichtman stage-IIIB disease. Follow-up radiographs revealed that the Lichtman stage had progressed in six of the twelve patients. CONCLUSIONS: Although most patients had mild wrist pain, patient satisfaction and the clinical results were satisfactory following a radial shortening osteotomy. This procedure is a reliable long-term treatment for Lichtman stage-II and IIIA disease and may be a reasonable option for patients with stage-IIIB disease. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence. ORIGINAL ABSTRACT CITATION: “Long-Term Follow-up of Radial Shortening Osteotomy for Kienbock Disease” (2008; 90:1705-11).

FROM THE ORIGINAL ARTICLE BACKGROUND: Three previous studies have investigated the long-term outcome of radial osteotomy in the treatment of Kienbock disease. However, none used patient-based assessment. The purpose of this study was to investigate the long-term clinical and radiographic outcomes of this osteotomy, including the subjective evaluation of the patient with use of the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. METHODS: A DASH questionnaire was sent to nineteen patients with Kienbock disease who had undergone a radial shortening osteotomy, and thirteen replied. The mean age at the time of surgery was thirty-nine years. On the basis of the Lichtman classification, six patients had stage-II, four had stage-IIIA, and three had stage-IIIB disease. Prior to surgery, ulnar variance was positive in six patients, neutral in four, and negative in three. The mean duration of follow-up was twenty-one years. Clinical evaluation, including calculation of the modified Mayo wrist score, and radiographic evaluation were also performed on twelve of the thirteen patients. RESULTS: The mean DASH score was 8 points (range, 0 to 23 points), and patient satisfaction was high. Compared with the findings in the contralateral wrist, the mean range of motion was 81% in flexion and 82% in extension and mean grip strength was 88%. The mean modified Mayo wrist score was 83 points, and the clinical results were excellent in six patients, good in five, and moderate in one. The DASH scores tended to be worse in patients with Lichtman stage-IIIB disease. Follow-up radiographs revealed that the Lichtman stage had progressed in six of the twelve patients. CONCLUSIONS: Although most patients had mild wrist pain, patient satisfaction and the clinical results were satisfactory following a radial shortening osteotomy. This procedure is a reliable long-term treatment for Lichtman stage-II and IIIA disease and may be a reasonable option for patients with stage-IIIB disease. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence. ORIGINAL ABSTRACT CITATION: “Long-Term Follow-up of Radial Shortening Osteotomy for Kienbock Disease” (2008; 90:1705-11).