Prune belly syndrome

Case contributed by Dr Bronwyn Shin

Presentation

Acute hematuria on a background of recurrent constipation.

Patient Data

Age: 2 years old
Gender: Male

One year ago

X-ray

Bilateral bulging flanks with intraperitoneal structures spread out laterally.

Renal tract ultrasound

Ultrasound

The right kidney is small, but the corticomedullary differentiation is preserved with no hydronephrosis.
The left kidney demonstrates marked hydronephrosis and hydroureter extending to the vesicoureteric junction. Real-time imaging also demonstrated left vesicoureteral reflux.
There is thickened appearance of the bladder wall.

Case Discussion

Prune belly syndrome is characterized by three findings:

  • lack of abdominal musculature leading to "prune belly" appearance
  • bilateral cryptorchidism
  • urinary tract abnormalities

This patient was diagnosed with prune belly syndrome in the antenatal period with all of the above findings.

The patient did not have any urinary tract symptoms until this presentation when he presented with large hematuria. An MAG-3 scan was performed later which demonstrated a dysplastic right kidney providing 14% of overall renal function (left kidney - 86%).

The patient had previously been operated on for bilateral cryptorchidism.

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