Can a complete primary repair approach be applied to cloacal exstrophy?

Surgical reconstruction for children with cloacal exstrophy remains challenging. The operative approach to cloacal exstrophy has expanded with the addition of the complete primary exstrophy repair. We assessed the safety and efficacy of complete primary exstrophy repair for this complex condition.

We performed a retrospective review of children treated from birth for cloacal exstrophy between March 1, 1994 and January 1, 2003 at our institution. We evaluated associated anomalies, method of closure, complications and urinary continence.

Seven patients with cloacal exstrophy were initially treated at our institution. One mortality occurred before complete primary exstrophy repair was attempted. Six patients were converted to a classic exstrophy appearance and underwent closure using complete primary exstrophy repair principles within 7 to 182 days (mean 68, median 32) postoperatively. Postoperative development of moderate hydronephrosis was seen in 1 patient and severe hydronephrosis in 1. Three of six patients had vesicoureteral reflux. Six patients had dry intervals and spontaneous voids before toilet training. Two patients had stress urinary incontinence. Two patients have been treated with bladder neck injections. One has undergone bladder neck reconstruction and construction of a nonorthotopic channel for clean intermittent catheterization (Mitrofanoff). One patient reported complete dryness after toilet training. One child has undergone bladder augmentation.

This series represents our initial efforts to use complete primary exstrophy repair for cloacal exstrophy. The application of the principles of complete primary exstrophy repair in a sequential fashion appears to be a viable and safe addition to the surgical armamentarium in this challenging patient population.