Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis

Abstract

Objective: To determine the types and prevalence of musculoskeletal involvement in children with prune-belly syndrome, and to analyze the pathogenesis of the syndrome in relationship to the musculoskeletal deformities.

Design: A retrospective review of charts and roentgenograms along with a comprehensive review of 188 cases from the literature.

Setting: Tertiary care children's hospital.

Participants: Twelve boys treated between 1975 and 1990.

Measurements/main results: The prevalence of musculoskeletal involvement in patients was 45%. The involvement can be congenital (eg, clubfeet, limb deficiencies, teratologic hip dysplasia, and vertebral malformations) or developmental (eg, renal osteodystrophy, scoliosis, and pectus excavatum and/or pectus carinatum). The embryologic characteristics of congenital musculoskeletal problems correlate better with the embryologic theory of the prune-belly syndrome (an aberration of mesenchymal development around 6 weeks of gestation) than with the distal urinary tract obstructive theory.

Conclusion: Since children with prune-belly syndrome are now living into adulthood, these musculoskeletal aspects will become important regarding potential morbidity.