Congenital Constriction Band Syndrome: A Seventy-Year Experience

Seventy-one cases of congenital constriction band syndrome (CCBS) were reviewed. The year of birth, sex, ethnic background, gestational history, family history, and concurrent diagnoses were obtained. The anatomic patterns of involvement, degree of impairment, and number and type of surgical interventions were recorded. The incidence of CCBS appears to be rising. The average patient had three involved limbs, with a predilection for distal, central digits of the upper extremity. Abnormal gestational histories were found in 60% of the cases; 50% had concurrent diagnoses, and nearly one-third had club-feet. The average patient had a 20% whole body impairment and required three reconstructive procedures. Distraction osteogenesis and free osteocutaneous transfer were useful. We feel that the term "early amnion rupture sequence" more accurately reflects the true pathogenesis of CCBS.