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Potentially life-threatening emergency
Prune belly syndrome
Other Resources UpToDate PubMed
Potentially life-threatening emergency

Prune belly syndrome

Contributors: David Sullo MD
Other Resources UpToDate PubMed

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

Differential Diagnosis & Pitfalls

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Best Tests

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References

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Last Updated:04/30/2023
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Potentially life-threatening emergency
Prune belly syndrome
A medical illustration showing key findings of Prune belly syndrome : Creatinine elevated
Clinical image of Prune belly syndrome - imageId=2362444. Click to open in gallery.  caption: 'A protruding abdomen with furrowing and wrinkling.'
A protruding abdomen with furrowing and wrinkling.
Copyright © 2024 VisualDx®. All rights reserved.