Prune belly syndrome
Hematuria, flank pain and fever.
Loading Stack -
0 images remaining
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.
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 labor 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.
- Hassett S, Smith GH, Holland AJ. Prune belly syndrome. Pediatric surgery international. 28 (3): 219-28. doi:10.1007/s00383-011-3046-6 - Pubmed
- Tonni G, Ida V, Alessandro V, Bonasoni MP. Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal and pediatric pathology. 31 (1): 13-24. doi:10.3109/15513815.2012.659411 - Pubmed