Characterization of Limb Involvement in Constriction Band Syndrome
Leah Demetri, MD, University of California, San Francisco, San Francisco, CA, Ana G Starcevich, MS, Shriners Hospitals for Children Northern California, Sacramento, CA, Mary Claire B Manske, MD, Shriners Hospital for Children Northern California and University of California Davis, Sacramento, CA and Michelle A. James, MD, Department of Orthopaedic Surgery, Shriners Hospital for Children Northern California, Sacramento, CA
Introduction: Constriction band syndrome (CBS) is an uncommon congenital condition causing limb deformity, with varying clinical presentation. We sought to characterize the pattern of limb involvement in CBS by analyzing a large cohort of patients.
Methods & Methods: We performed a retrospective review of all patients with CBS presenting to our tertiary medical center between January 1, 1998 and December 31, 2018. Patients were identified by ICD-9 and ICD-10 codes. Medical records were reviewed for demographic data and associated conditions. Clinical photographs and radiographs were reviewed to determine the pattern of limb involvement.
Results: 138 children with CBS were identified. Upper and lower extremities were affected equally (80% and 79%, respectively). The most prevalent feature was distal limb amputation (89%) followed by constriction bands (79%) and acrosyndactyly (68%). Amputations were characterized by hypoplasia and deformed appearance of the amputated bone on radiographs. There was a strong predilection for involvement of central digits of the hands and feet with sparing of the thumb/great toe and small finger/toe. Although most features of CBS were limited to the distal extremities, some children had limb amputations proximal to the digits (17%) and proximal constriction bands (39%). The average number of involved extremities was 2.5 limbs per child, however 33% had involvement of only one limb. Children with involvement of a single limb were significantly less likely to have an associated diagnosis compared to children with multiple involved limbs (36% vs. 56%, p=0.047). The most common associated conditions were clubfoot (34%) and craniofacial anomalies (12%).
Conclusion: Children with CBS can be categorized as having either mild or severe involvement based on the number of involved limbs and associated conditions. This characterization may provide implications for prognosis and treatment options. Future investigation of prenatal risk factors may further elucidate the etiology of this heterogenous condition.
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