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Bilateral Single System Ureteroceles
Joseph Junewick, MD FACR
over 7 years ago
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ARSt Case Repository

Prune Belly Syndrome

Case Detail

Anatomy: Genitourinary
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags:
Modality/Study Types: CR FL
Activities:
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History

Newborn male with large soft abdomen.


Case Images


Diagnosis

Prune Belly Syndrome

Findings

CR – Flaccid abdominal wall with bulging flanks.

FL – Large, elongated urinary bladder with dilated prostatic urethra.

Discussion

Prune belly syndrome (Eagle-Barrett syndrome) is a multisystem disorder which almost exclusively affects males. Clinically the abdominal wall lax and wrinkled due to deficient rectus abdominus muscles. Genitourinary abnormalities include undescended testes, dilated ureters, megalocystis, dilated prostatic urethra, urethral obstruction (atresia or valves) and renal dysmorphism. The bladder is often enlarged and elongated but lacks trabeculation. The wall of the bladder is thickened by replacement of normal smooth muscle with connective tissue. A similar process involving the ureters produces ureteral dilatation, although ureteral dilatation may be secondary to reflux.

Reference

Berrocal T, Lopez-Pereira P, Arjonilla A, et al. Anomalies of the Distal Ureter, Bladder, and Urethra in Children: Embryologic, Radiologic, and Pathologic Feature. Radiographics (2002); 22:1139-1164.



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