Mobilization of pelvic musculature and its effect on continence in classical bladder exstrophy: a single-center experience of 38 exstrophy repairs.

UNLABELLED

Soft tissue mobilization of pelvic musculature in bladder exstrophy repair and its effect on continence. A single-center experience of 38 exstrophy repairs in children.

INTRODUCTION/BACKGROUND: Bladder exstrophy is characterized by infra-umbilical abdominal wall defects, evaginated bladder plate of varying size, epispadias, abnormality of genitalia and bony pelvis. The goal of repair is to provide satisfactory continence, which should preferably be done in specialized centers dedicated to exstrophy management. The concept of functional reconstruction rather than urinary diversion is the gold standard worldwide, which can be accomplished by staged or one-stage procedures. Our technique of mobilization of pelvic musculature is based on the concept that continence in bladder exstrophy can be achieved by repairing the disorganized/splayed tissues involved in normal continence (as first advocated by J.H. Kelly) without osteotomy.

OBJECTIVES

A systematic review of outcomes of neonatal bladder closure followed by mobilization of pelvic musculature in bladder exstrophy repair in children.

STUDY DESIGN

A retrospective chart review of all exstrophy repairs conducted over a 10-year period (between 2001 and 2011). Repairs were done in two stages: bladder closure in the neonatal period (stage 1); and mobilization of pelvic musculature and epispadias repair (stage 2), preferably done between 4 and 6 months of age. The data on complications and continence were evaluated.

RESULTS

Thirty-eight patients had completed all the stages of repair. Mean follow-up was 4.5 years (range 2.5-8 years). The following complications were noted: bladder dehiscence in eight patients after stage 1 repair, penopubic fistula occurred in four patients following stage 2 repair. Vulval scarring and vulval dehiscence (2 patients) were complications seen in girls. Twenty-four of the 38 patients (63.5%) achieved complete continence. Functional continence was attained by 31 of the 38 patients (82%). Older age at bladder closure affected continence, while the number of attempts at closure did not affect outcome. The age at pelvic mobilization was not a significant factor in outcome.

DISCUSSION

The pelvic floor musculature and urethral sphincters are essential for voluntary control of micturition. In bladder exstrophy, these components are splayed out and our technique is based on reorganizing these components in the second stage. The voluntary urethral sphincter is a delicate complex of musculature located dorsal to the opened urethral plate and spread over the corpora. These are identified using a muscle stimulator and repaired around the tubularized urethra. Normally the levator ani muscle, by its attachment to the pubic bone, forms a loop, by which it compresses the urethra, providing additional aid in continence. In bladder exstrophy with wide pubic diastasis, this loop configuration is lost and becomes a hammock configuration and in fact becomes a pushing force. By mobilizing the pelvic musculature and repairing it in front of the bladder neck, this loop configuration is re-established and further aids in continence. After a successful second stage, patients may have increased frequency and dribbling initially, which improves with age as bladder capacity increases. Perineal exercises aimed at strengthening the pelvic floor musculature are an integral part of our bladder exstrophy management, which begins once the child can understand the technique. The results of our technique are shown in comparison with other series employing the original Kelly's technique (see Table). None of our patients have undergone additional bladder neck repair or permanent augmentation.

CONCLUSIONS

Neonatal bladder closure followed by mobilization and repair of the pelvic musculature, produce satisfactory continence in exstrophic children. Proper identification and repair of the external sphincter and levatorplasty correct the altered anatomy by relocating the bladder neck and posterior urethra deep in the pelvis, simulating normal micturition. Early neonatal bladder closure improves outcome. The results are reproducible if basic principles governing continence are followed and when done in a specialized centers.