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Renal transplantation in prune-belly syndrome
Fabio Fusaro1, Giovanni F Zanon, Anna M Ferreli
1Division of Pediatric Surgery, University of Padova, via Giustiniani 3, 35128, Padova, Italy. fabiofusaro@libero.it
Insights
Prune-belly syndrome (PBS) patients show good renal transplant outcomes, with 5-year graft survival at 66.7%. Surgical correction of urinary and abdominal wall issues improves outcomes and reduces urinary tract infections (UTIs).
Area of Science:
- Pediatric Nephrology
- Urology
- Transplantation Surgery
Background:
- Prune-belly syndrome (PBS) is a congenital disorder characterized by abdominal wall defects, urinary tract anomalies, and undescended testes.
- Renal transplantation is a treatment option for end-stage renal disease in PBS patients, but outcomes can be affected by the syndrome's complexities.
Purpose of the Study:
- To evaluate the efficacy and outcomes of renal transplantation in patients with prune-belly syndrome.
- To identify factors influencing graft survival, patient survival, and complications in this specific population.
Main Methods:
- A retrospective analysis of six renal transplantations performed in five boys with PBS.
- Assessment of graft survival, graft function, lower urinary tract dysfunction, urinary tract infections (UTIs), and patient survival at 1 and 5 years post-transplant.
Main Results:
- The 1-5-year renal graft survival rate was 66.7%, with mean serum creatinine levels between 98-103 micromol/l.
- Surgical intervention for bladder obstruction and abdominal wall defects reduced UTIs, enabling patients to retain their native urinary tracts without prophylactic antibiotics.
- One patient experienced a mechanical complication due to abdominal wall deficiency, which was successfully managed with Monfort's abdominal wall reconstruction.
Conclusions:
- Renal transplantation offers a good prognosis for patients with prune-belly syndrome.
- Pre-transplant evaluation of urinary tract anomalies and abdominal wall musculature is crucial for optimizing treatment strategies and long-term graft outcomes.
Abstract:
We assess the effect of the prune-belly syndrome (PBS) on renal transplantation outcome. Six renal transplantations were performed in five boys affected by PBS (median age 5.8+/-2.1 years, median weight 13.6+/-2.4kg). Renal graft survival, graft function, lower urinary tract dysfunction, urinary tract infections (UTIs), associated complications and patients' survival after 1 and 5 years of follow-up were analysed. The rate for 1-5-year graft survival was 66.7% (mean serum creatinine 98-103 micromol/l). The surgical treatment of the documented bladder obstruction (two patients) and the severe abdominal wall deficit (one patient) led to a reduction of UTI: the patients maintained their native urinary tract and none received prophylactic antibiotics. The lack of abdominal wall musculature led to severe mechanical complication in one patient, but Monfort's abdominal wall reconstruction was able to restore the graft's function. The outcome of patients with PBS who undergo renal transplantation is good. Before the transplant, an accurate assessment of urinary tract anomalies and deficiency of the abdominal wall musculature is mandatory, to program the appropriate treatment and obtain a good long-term prognosis for the renal graft.
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