Musculoskeletal Aspects of Prune-Belly Syndrome

Description and Pathogenesis

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

Research output: Contribution to journalArticle

28 Citations (Scopus)

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 - 1992
Externally publishedYes

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Prune Belly Syndrome
Funnel Chest
Chronic Kidney Disease-Mineral and Bone Disorder
Clubfoot
Hip Dislocation
Scoliosis
Tertiary Healthcare
Urinary Tract
Extremities
Morbidity
Pregnancy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Musculoskeletal Aspects of Prune-Belly Syndrome : Description and Pathogenesis. / Loder, Randall; Guiboux, Jean Paul; Bloom, David A.; Hensinger, Robert N.

In: American Journal of Diseases of Children, Vol. 146, No. 10, 1992, p. 1224-1229.

Research output: Contribution to journalArticle

Loder, Randall ; Guiboux, Jean Paul ; Bloom, David A. ; Hensinger, Robert N. / Musculoskeletal Aspects of Prune-Belly Syndrome : Description and Pathogenesis. In: American Journal of Diseases of Children. 1992 ; Vol. 146, No. 10. pp. 1224-1229.
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