Potentially life-threatening emergency
Prune belly syndrome
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Synopsis
Life-threatening to fetus and potential cause of stillbirth, prune belly (Eagle-Barrett) syndrome is a rare congenital disorder characterized by a triad of abnormalities including deficient or absent abdominal muscles, severe urinary tract malformations, and bilateral cryptorchidism. Presentation may include renal dysplasia and pulmonary hypoplasia. Condition will be present at birth; affected patients have a swollen abdomen with wrinkly skin. Ultrasound may reveal the condition. Etiology is unknown. Mainly affects males but female cases have been reported. Possible complications include chronic constipation, recurrent infections, growth retardation, chronic kidney disease, and delays sitting and walking. Surgery is typically required.
Codes
ICD10CM:
Q79.4 – Prune belly syndrome
SNOMEDCT:
5187006 – Prune belly syndrome
Q79.4 – Prune belly syndrome
SNOMEDCT:
5187006 – Prune belly syndrome
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Last Updated:04/30/2023
Potentially life-threatening emergency
Prune belly syndrome