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Published on: January 7, 2019
The long-term outcomes after staged repair of exstrophy-epispadias complex
1Department of Urology, Institute of Kidney Diseases and Research Center and Dr. H L Trivedi Institute of Transplantation Sciences, Civil Hospital Campus, Ahmedabad, Gujarat, India.
Insights
Staged reconstruction for bladder exstrophy (BE) shows promising results for urinary continence and acceptable cosmetic outcomes. This approach minimizes renal scarring, offering a better quality of life for affected children.
Area of Science:
- Pediatric Urology
- Genitourinary Reconstructive Surgery
- Congenital Malformations
Background:
- Classic bladder exstrophy (BE) is a rare genitourinary malformation affecting 1 in 50,000 to 100,000 live births.
- Surgical reconstruction of the bladder exstrophy-epispadias complex presents significant challenges for pediatric specialists.
Purpose of the Study:
- To evaluate the success of staged reconstruction for bladder exstrophy (BE).
- To assess the long-term effects of BE repair on the upper urinary tract, renal function, and urinary continence.
Main Methods:
- Retrospective study of 30 patients with BE undergoing stage 1 repair between 1994 and 2013.
- Included epispadias repair in 18 males and bladder neck reconstruction in 13 patients.
- Three patients required augmentation cystoplasty.
Main Results:
- Out of 17 patients assessed, 14 achieved social continence.
- Four patients require clean intermittent self-catheterization for bladder emptying; four await further continence procedures.
- Two patients with augmentation cystoplasty developed renal failure requiring hemodialysis; one had altered renal function.
Conclusions:
- Modern staged repair for bladder exstrophy (BE) is associated with a low risk of renal scarring.
- The procedure offers acceptable opportunities for achieving urinary continence.
- Acceptable cosmetic appearance of external genitalia in both males and females is achieved.
Introduction:
Classic bladder exstrophy (BE) is a rare malformation of the genito-urinary tract affecting 1:50,000 to 1:100,000 live births. The surgical reconstruction of the BE-epispadias complex is challenging for the most experienced pediatric urologists, surgeons, and orthopedists.
Purpose:
To assess the success of staged reconstruction of the BE and long-term effects on the upper urinary tract, renal function, and continence.
Materials And Methods:
This is retrospective study; between 1994 and 2013, 30 patients with BE have undergone stage 1 repair at the institute. Eighteen male patients have been operated for epispadias repair and thirteen patients have undergone Guy Leadbetter bladder neck reconstruction. Three patients required augmentation cystoplasty one child is continent after epispadias repair only and one child attained continence after single-stage repair.
Results:
Hence, out of 17 patients, 14 are socially continent, four patients require clean intermittent self-catheterization for bladder emptying. Four patients, who are coming for regular follow-up, are awaiting continence procedure. Two patients who underwent augmentation cystoplasty are on hemodialysis for renal failure and one child has altered renal function.
Conclusion:
In our experience, the modern staged repair offers a low risk of renal scarring with acceptable continence opportunity with acceptable cosmetic appearance of external genitalia in the males and females.
