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Congenital constriction band syndrome: a seventy-year experience
1Division of Orthopaedics, John A. Burns School of Medicine, University of Hawaii, Honolulu.
Insights
Congenital constriction band syndrome (CCBS) incidence is rising, often affecting multiple limbs and requiring multiple surgeries. Early amnion rupture sequence may better explain its cause.
Area of Science:
- Orthopedics
- Developmental Biology
- Medical Genetics
Background:
- Congenital constriction band syndrome (CCBS) is a rare condition affecting limb development.
- Understanding its incidence, patterns, and pathogenesis is crucial for effective management.
- Previous studies have varied in scope and focus, necessitating a comprehensive review.
Purpose of the Study:
- To review a cohort of CCBS cases to elucidate incidence, clinical features, and outcomes.
- To identify common patterns of limb involvement and associated anomalies.
- To evaluate the effectiveness of surgical interventions and propose a more accurate pathogenetic term.
Main Methods:
- Retrospective review of 71 CCBS cases.
- Data collection included demographics, gestational and family history, concurrent diagnoses, and CCBS characteristics.
- Anatomic patterns, impairment levels, and surgical interventions were recorded.
Main Results:
- CCBS incidence appears to be increasing.
- The average patient had three involved limbs, predominantly distal upper extremity digits.
- Abnormal gestational histories (60%), concurrent diagnoses (50%), and club feet (approx. 33%) were common; average impairment was 20%, requiring three procedures.
- Distraction osteogenesis and free osteocutaneous transfer were effective surgical techniques.
Conclusions:
- CCBS is increasingly recognized, with characteristic limb involvement and frequent comorbidities.
- Surgical interventions, including distraction osteogenesis, are vital for managing CCBS.
- The term "early amnion rupture sequence" is proposed as a more accurate descriptor of CCBS pathogenesis.
Abstract:
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.