The long-term outcomes after staged repair of exstrophy-epispadias complex.

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.