Prune-belly syndrome
Abstract
Prune-belly syndrome, also known as Eagle-Barretsyndrome, is a congenital anomaly comprising
three clinical findings: deficient abdominal
musculature, urinary tract anomalies, and
bilateral cryptorchidism. Other clinical findings involving
respiratory, skeletal, digestion and cardiovascular system
may also accompany the syndrome. The incidence is
approximately 1 : 30,000 to 40,000 live births and 95%
of cases occur in boys. Pulmonary hypoplasia and kidney
failure are important prognostic factors that contribute
to 60% of mortality rate. Treatment includes surgical
correction of the abdominal wall and urinary tract,
orchidopexy and other supportive managements.l-4 We
report 4 cases on typical Prune-belly syndrome, together
with other clinical variants.
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Accepted 2016-09-11
Published 2009-10-31