The Journal of Bone and Joint Surgery, Vol 75, Issue 2 259-264, Copyright © 1993 by Journal of Bone and Joint Surgery, Inc
A syndrome of dislocated hips and radial heads, carpal coalition, and short stature in Puerto Rican children
HH Steel, RW Piston, M Clancy and RR Betz
Shriners Hospitals for Crippled Children, Philadelphia Unit, Pennsylvania.
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.
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