prune belly syndrome


prune bel·ly syn·drome

a syndrome of deficient abdominal muscle, undescended testes, large hypotonic bladder and dilated, tortuous ureters. Synonym(s): Eagle-Barrett syndrome, triad syndrome

prune belly syndrome

Eagle-Barrett syndrome Pediatrics A condition characterized by congenital lack of abdominal muscles, which imparts a rugose, prune-like appearance to the flaccid abdominal wall; 97% occur in ♂ and are accompanied by GU anomalies–eg, bilateral cryptorchidism, hypoplastic and dysplastic kidneys; affected ♀ have uterine defects; although considered an X-linked disease, no chromosome defect has been identified, and PBS may represent a 'sequence' initiated by in utero urethral obstruction, causing urinary tract anomalies–megaureters, megabladder, patent urachus or urachal cyst; other findings include Potter's facies, talipes, hip dislocation, musculoskeletal and cardiac defects Treatment Corsets, excision of redundant tissue Prognosis Oligohydramnios may arise in utero, causing fetal pulmonary hypoplasia, 20% are stillborn, 50% die in infancy. See Oligohydramnios.

Eagle,

J.F., Jr., 20th century U.S. physician. Eagle-Barrett syndrome - Synonym(s): prune belly syndrome

Barrett,

Norman Rupert, English surgeon, 1903-1979. adenocarcinoma in Barrett esophagus - an adenocarcinoma arising in the lower third of the esophagus that has become columnar cell lined (Barrett mucosa) due to gastroesophageal reflux.Barrett epithelium - columnar esophageal epithelium seen in Barrett syndrome.Barrett esophagus - chronic peptic ulceration of the lower esophagus acquired as a result of long-standing chronic esophagitis. Synonym(s): Barrett syndrome; Barrett ulcerBarrett syndrome - Synonym(s): Barrett esophagusBarrett ulcer - Synonym(s): Barrett esophagusEagle-Barrett syndrome - Synonym(s): prune belly syndrome

Obrinsky,

William, U.S. physician, 1913–. Obrinsky syndrome - aplasia or hypoplasia of the abdominal wall. Synonym(s): prune belly syndrome