Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis
R. T. Loder, J. P. Guiboux, D. A. Bloom and R. N. Hensinger
Section 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.
