Congenital metacarpotalar syndrome

Three children were presented having similar facies, musculoskeletal anomalies and a common ethnic background. All had dislocated hips, scoliosis, congenital vertical tali and hand deformities. There was a very small web space between the first and second metacarpals, extension deformities at the wrist, flexion deformities at the metacarpophalangeal joints, and at one or more proximal interphalangeal joints. In addition, 2 of the 3 youngsters had marked crowding of the teeth and a small maxillary arch. All laboratory studies were normal. The most alarming finding was the poor wound healing which averaged 48%. The wound complication rate at Shriner's Hospital in San Francisco during the identical period of time these children were treated ranged from 1.2% to 1.8%. It is believed that this represents a new syndrome, as similar cases could not be found in the literature. The orthopedist may treat these anomalies in the standard fashion but should keep in mind the propensity for poor wound healing in these patients. The common ethnic background was that of Spanish-American or Chicago families.