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Prune‐Belly Syndrome in Two Children and Review of the Literature
Author(s) -
Bogart Megan M.,
Arnold Holly E.,
Greer Kenneth E.
Publication year - 2006
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2006.00265.x
Subject(s) - prune belly syndrome , medicine , orchiopexy , abdominal wall , urinary system , gastrointestinal tract , pulmonary hypoplasia , congenital disorder , hypoplasia , incidence (geometry) , surgery , abdominal wall defect , disease , anatomy , pregnancy , fetus , genetics , physics , biology , optics
Prune‐belly syndrome is a congenital disorder characterized by abdominal wall musculature deficiency, urinary tract anomalies, and bilateral cryptorchidism. Because of the defect in the musculature, the abdominal skin has a peculiar wrinkled appearance. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Developmental delays and growth retardation have also been reported. The incidence of prune belly syndrome is approximately 1:40,000 live births. Over 95% of patients are men. Urinary tract disease is the major prognostic factor, with the complications of pulmonary hypoplasia and end stage renal disease resulting in a mortality rate of 60%. Treatment involves surgical correction of the abdominal wall defect and urinary tract abnormalities, early orchiopexy, and supportive management of associated defects.