Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis.

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.