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Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis
R T Loder1, J P Guiboux, D A Bloom
1Section of Orthopaedic, University of Michigan School of Medicine, Ann Arbor.
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.