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Prune-belly syndrome consists of abdominal muscle deficiency, urinary tract abnormalities, and intra-abdominal undescended testes.
The name prune-belly syndrome derives from the characteristic wrinkled appearance of the abdominal wall in neonates. The cause of this congenital syndrome, which occurs primarily but not exclusively in males, is unclear. Urinary abnormalities may include hydronephrosis, megaureters, vesicoureteral reflux, and urethral abnormalities. Severe cases may involve renal failure, bronchopulmonary dysplasia, and stillbirth.
Diagnosis is often made during routine prenatal ultrasonography. Further evaluation includes voiding cystourethrography, ultrasonography, and isotope renography.
Urinary tract abnormalities may require open surgical reconstruction; orchidopexy can be done at the same time. If no urinary intervention is necessary, laparoscopic orchidopexy is done in childhood.
Last full review/revision January 2010 by Ronald Rabinowitz, MD
Content last modified January 2010
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