Prune belly syndrome

Case contributed by Dr Corey Thompson

Presentation

Haematuria, flank pain and fever.

Patient Data

Age: 50 years
Gender: Male
Modality: CT

​Absence of abdominal wall musculature.

Grossly distended bladder with wall thickening and diverticulum. Bilateral hydronephrosis and hydroureter. Grossly distended and tortuous ureters.

Non-obstructing right kidney calculus.

Case Discussion

Prune belly syndrome is classically described by a triad of clinical features: abdominal wall aplasia/deficiency, urinary tract abnormalities and bilateral cryptorchidism.

This patient reports the diagnosis at birth. Interestingly, this patient engages in paid manual labour despite the absence of abdominal wall musculature.

The diagnosis is based on clinical features. These are commonly identified on the second-trimester antenatal ultrasound.

Differentials include posterior urethral valves and megacystis microcolon intestinal hypoperistalsis syndrome.

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Case Information

rID: 51319
Case created: 10th Feb 2017
Last edited: 21st Mar 2017
Inclusion in quiz mode: Included

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