Musculoskeletal Aspects of Prune-Belly Syndrome: Description and Pathogenesis

Randall T. Loder, Jean Paul Guiboux, David A. Bloom, Robert N. Hensinger

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29 Scopus citations

Abstract

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. —A retrospective review of charts and roentgenograms along with a comprehensive review of 188 cases from the literature. —Tertiary care Children's hospital. —Twelve boys treated between 1975 and 1990. —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. —Since children with prune-belly syndrome are now living into adulthood, these musculoskeletal aspects will become important regarding potential morbidity.

Original languageEnglish (US)
Pages (from-to)1224-1229
Number of pages6
JournalAmerican Journal of Diseases of Children
Volume146
Issue number10
DOIs
StatePublished - Oct 1992

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ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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