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Musculoskeletal Aspects of Prune-Belly SyndromeDescription and Pathogenesis
Randall T. Loder, MD;
Jean-Paul Guiboux, MD;
David A. Bloom, MD;
Robert N. Hensinger, MD
Am J Dis Child. 1992;146(10):1224-1229.
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.
(AJDC. 1992;146:1224-1229)
Author Affiliations
From the Sections of Orthopaedic (Drs Loder, Guiboux, and Hensinger) and Urological (Dr Bloom) Surgery, The University of Michigan School of Medicine, Ann Arbor.
Footnotes
Accepted for publication May 26, 1992.
Reprint requests to The University of Michigan Hospitals, 1500 E Medical Center Dr, 2912 G Taubman Center, Box 0328, Ann Arbor, Ml 48109-0328 (Dr Loder).
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