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prune belly syndrome |
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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. Want to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit the webmaster's page for free fun content. |
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