Musculoskeletal aspects of prune-belly syndrome. Description and pathogenesis.
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.


METHODS
A retrospective review of charts and roentgenograms along with a comprehensive review of 188 cases from the literature.


METHODS
Tertiary care children's hospital.


METHODS
Twelve boys treated between 1975 and 1990.


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.


CONCLUSIONS
Since children with prune-belly syndrome are now living into adulthood, these musculoskeletal aspects will become important regarding potential morbidity.
Loder
R T
RT
Section of Orthopaedic, University of Michigan School of Medicine, Ann Arbor.
Guiboux
J P
JP
Bloom
D A
DA
Hensinger
R N
RN
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006776:Hospitals, Pediatric; D006785:Hospitals, University; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008824:Michigan / Q000453:epidemiology; D009140:Musculoskeletal Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000628:therapy; D015995:Prevalence; D011535:Prune Belly Syndrome / Q000150:complications* / Q000196:embryology / Q000453:epidemiology; D011859:Radiography; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome