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Published on: January 7, 2019
Urethral atresia and the prune belly syndrome. Report of 6 cases
Y Reinberg1, G Chelimsky, R Gonzalez
1Department of Urologic Surgery, University of Minnesota Hospital and Clinic, Minneapolis.
Insights
Urethral atresia in prune belly syndrome (PBS) affects 18% of patients. Survival is linked to vesicocutaneous fistulas, but most survivors develop end-stage renal failure.
Area of Science:
- Pediatric Urology
- Congenital Abnormalities
- Renal Physiology
Background:
- Prune belly syndrome (PBS) is a rare congenital disorder characterized by abdominal muscle deficiency, urinary tract abnormalities, and undescended testes.
- Urethral atresia, a severe obstructive anomaly, is occasionally associated with PBS, posing significant management challenges.
Observation:
- This study reviewed 34 patients with PBS, identifying 6 cases (18%) with urethral atresia (3 males, 3 females).
- Two neonates died from pulmonary insufficiency, and one was stillborn, highlighting the critical condition of these patients.
- A spontaneous vesicocutaneous fistula was a common feature in the 3 surviving patients (2 males, 1 female).
Findings:
- Pulmonary insufficiency due to oligohydramnios affected two neonates.
- Early urinary tract decompression via vesicostomy was performed in two survivors.
- The surviving female achieved normal renal function after complex reconstructive surgery, including an ileocaecal bladder substitute and appendiceal urethra.
- The surviving males experienced renal insufficiency; one received a successful renal transplant, while the other underwent urethral reconstructive surgery and progressive catheter dilation.
Implications:
- Survival in PBS with urethral atresia correlates with the presence of a vesicocutaneous fistula.
- Despite surgical interventions, two-thirds of survivors develop end-stage renal failure, emphasizing the need for long-term renal monitoring and management.
- This study underscores the critical interplay between urethral atresia, PBS, and renal outcomes, informing future treatment strategies.
Abstract:
An association between urethral atresia and the prune belly syndrome (PBS) has been recognised, but few reports discuss the outcome of treatment for these gravely ill patients. Of the 34 patients with prune belly syndrome evaluated at our institutions, 6 had urethral atresia (3 males and 3 females). Two of these patients died as neonates as a result of pulmonary insufficiency and 1 was stillborn. The common feature of the 3 surviving patients (2 males and 1 female) was the presence of a vesicocutaneous fistula. As neonates, 2 suffered from pulmonary insufficiency due to oligohydramnios. The mean length of follow-up for these 3 patients was 37 months (range 13-58). The urinary tract was decompressed in 2 patients by a formal vesicostomy performed early in life. The surviving girl has normal renal function following reconstructive surgery. Because both the bladder and urethra were absent in this patient, an ileocaecal bladder substitute and an appendiceal urethra were constructed. The 2 surviving boys both have renal insufficiency. One has received a renal transplant from a living relative and is doing well. In one of the boys, urethral atresia was initially managed by perineal urethrostomy and then by reconstructive surgery. Progressive catheter dilation was used on the other boy. Urethral atresia occurred in 18% of our patients with PBS; the incidence was equal in males and females. Survival correlated with the development of a spontaneous vesicocutaneous fistula. Two-thirds of the survivors developed end-stage renal failure.
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