V09-11: Safe and Sound: Principles for Successful Complete Primary Repair of Bladder Exstrophy (CPRE) in the Boy

Video

INTRODUCTION

Bladder exstrophy (BE) is rare, complex and presents many challenges to optimal surgical reconstruction and outcome in the boy. We detail important elements of CPRE technique with specific considerations for safety, limitation of tissue injury, and optimization of functional and cosmetic outcomes.

METHODS

Boys with BE underwent CPRE and were followed per Multi-Institutional Bladder Exstrophy Consortium (MIBEC) IRB protocol. General technical principles included use of skin hook gentle retraction vs. repetitive forceps grasping of skin, sharp dissection vs. electrocautery, and intermittent topical dilute epinephrine or fine absorbable suture for hemostasis vs. epinephrine injection or monopolar electrocautery. Urethral tubularization and bladder closure were performed by incorporating serosa with little or no mucosa, inverting mucosa to decrease fistula risk. Specific principles included exploitation of fat planes to dissect skin from fascia, and in turn, fascia from bladder. Urethral plate dissection with initial ventral approach using manual traction to &[Prime]roll&[Prime] the corpora laterally, sharp dissection and bipolar electrocautery facilitates separation of spongiosum from cavernosum. The urethral width is carried proximally to the level of the bladder neck to promote continence. Glans color and perfusion pre- and post pubis approximation is carefully assessed to decrease potential ischemic injury. Post-CPRE pelvic immobilization is with spica cast.

RESULTS

From February 2013 to November 2017 MIBEC surgeons completed successful CPRE with bilateral pelvic osteotomy in 29 consecutive boys with BE. No bladder dehiscence occurred, penopubic skin loss was noted in 1 boy, but otherwise no tissue loss or urinary retention was observed. Hypospadias was result at CPRE in 6. In several boys 3 years plus post-CPRE, toilet training has commenced or completed, voiding without straining observed, and cystometrogram with normal capacity, compliance and detrusor voiding contraction has been documented. Urethrocutaneous fistula occurred in 5 boys including 2 with a large posterior urethra/ penopubic fistula. One of these boys has undergone reoperation and the other is awaiting same. Pyelonephritis occurred in 2. There were no complications with spica cast use.

CONCLUSION

Based on our evolving experience, including real time coaching and case video review, we have found that the principles developed and discussed promote safety, limit tissue injury, and optimize functional and cosmetic outcomes. The MIBEC has promoted ongoing refinement of CPRE technique.

Funding: none