prune belly 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.