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Prune-belly syndrome

2005-11-15-11 Heron Werner, MD*, Pedro Daltro, MD*, Dorothy Bulas, MD# * Clínica de Diagnóstico por Imagem (CDPI) & Instituto Fernandes Figueira (IFF) – FIOCRUZ – Rio de Janeiro – Brazil
# Professor of Radiology and Pediatrics – Children"s National Medical Center – George Washington University Medical Center – 111 Michigan Ave, NW, Washington D.C. 20010

The term prune belly Is often used to describe the appearance of a neonate born with urethral obstruction megacystis, and, after drainage, the appearance of a prune belly. There is however, a syndrome that includes megacystis, severe hydro ureter and hydronephrosis, agenesis of the rectus muscles, and cryptorchidism without necessarily urethral obstruction.

The Prune-belly syndrome is a pathology of doubtful cause. It consists of the triad:

A plausible explanation for Prune-Belly is a precocious urethral obstruction that will raise the pressure on the abdominal muscles causing atrophy. An important vesical distension will also hinder the testicles from coming down. In some cases, an important oligohydramnios is noticeable. Nevertheless, polyhydramnios can be present. The contradiction of a severely obstructed tract sonographically with normal or often increased amniotic fluid volume is most likely prune belly syndrome. These babies typically do not develop pulmonary hypoplasia, and survival is expected. The prognosis depends on the severity of the urinary distension and oligohydramnios as the cause of pulmonary hypoplasia.

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