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Virtual Pediatric Hospital: Paediapaedia: Prune-Belly Syndrome (Eagle-Barrett Syndrome) Paediapaedia: Genitourinary Diseases

Prune-Belly Syndrome (Eagle-Barrett Syndrome)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Triad of deficient or absent abdominal musculature, undescended testicles, and dysplasia of the urinary tract. Physical exam shows a wrinkled anterior abdominal wall.

Etiology/Pathophysiology:
Etiology is unknown.Twenty percent are still born or die as neonates, and an additional 50% die in the first 2 years of life. Other associated anomalies include hip dislocations / club foot / limb deformities (40% ), cardiac defects(30% ), and malrotation (30% ).

Pathology:
Aplasia of the muscles of the lower medial abdominal wall. The kidneys have a decreased number of functioning nephrons with variable cystic dysplasia. The dilated ureters and bladder have a patchy absence of smooth muscle.

Imaging Findings:
The ureters and bladder are dilated. Urethral abnormalities that can be seen include posterior urethral dilation, diverticula, stenosis / atresia, and megaurethra. They can have umbilical-bladder attachment or a patent urachus.

DDX:

References:
See References Chapter.

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