A syndrome of dislocated hips and radial heads, carpal coalition, and short stature in Puerto Rican children

Abstract

An orthopaedic syndrome that apparently had not been reported previously was identified in twenty-three children. Characteristics shared by all twenty-three children included Hispanic descent, residence in Puerto Rico, bilateral dislocation of the hip, dislocated radial heads, short stature, and other osseous anomalies. Twelve dislocated hips in six patients were not treated. All of these hips were functioning satisfactorily at the time of the review, but only four of the children had reached skeletal maturity. Sixteen hips in eight patients remained reduced after closed reduction. Of these eight patients, the four who were skeletally immature at the time of the review had a satisfactory result, and the four who were skeletally mature had an unsatisfactory result because of discomfort or fibrous ankylosis. Eighteen hips in nine patients were treated with a reduction augmented by some form of operation. All of these hips redislocated. Of the forty-six elbows in the twenty-three children, thirty-three were dislocated, as seen clinically and radiographically; eight were normal, both clinically and radiographically; and there was dysplasia at the radiocapitellar articulation of the remaining five. Twenty of the twenty-three children were found to have carpal coalitions. Fourteen children had scoliosis, and five of them were managed with spinal arthrodesis and correction. Three patients had an anomaly of the cervical spine, with one deformity causing symptoms and signs that were treated with decompression. Eight patients had talipes cavus bilaterally, which was not treated.