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Potentially life-threatening emergency
Prune belly syndrome in Infant/Neonate
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Potentially life-threatening emergency

Prune belly syndrome in Infant/Neonate

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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.

For more information, see OMIM.

Codes

ICD10CM:
Q79.4 – Prune belly syndrome

SNOMEDCT:
5187006 – Prune belly syndrome

Differential Diagnosis & Pitfalls

Posterior urethral valves

Best Tests

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References

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Last Updated: 03/29/2017
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Potentially life-threatening emergency
Prune belly syndrome in Infant/Neonate
Print 4 Images
View all Images (4)
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Prune belly syndrome : Creatinine elevated
Clinical image of Prune belly syndrome
Copyright © 2018 VisualDx®. All rights reserved.