Complete repair of bladder exstrophy in the newborn: complications and management

Complete repair of bladder exstrophy in the newborn includes bladder, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While reports have shown the need for early reimplantation of the ureters in 50% of patients and repair of hypospadias in those in whom the urethral plate does not reach the tip of the glans, to my knowledge none has described immediate postoperative complications in these patients.

Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehiscence with loss of the proximal urethra and a significant amount of penile skin occurred in 2 male patients. Bladder prolapse with loss of the urethra well onto the penis occurred in 2 newborns, of whom 1 had undergone 2 closures before referral and 1 had partial loss of the glans on 1 side. One patient had only minor bladder prolapse but lost most of the urethra and penile skin. The final male patient lost most of the urethra and had separation of the pubis but the bladder maintained a subcutaneous position and did not prolapse. The female patient had major prolapse of the bladder and loss of the urethrovaginal septum.

The 2 patients with complete dehiscence underwent reclosure with bilateral innominate and vertical iliac osteotomy with replacement of the urethral plate with a skin graft and await epispadias repair. The patient with major prolapse and 2 prior closures was treated with reclosure, bladder augmentation and continent stoma reconstruction, and awaits epispadias repair. In the male patient with marked prolapse and partial loss of the glans has undergone reclosure with bilateral innominate and vertical iliac osteotomy was done and he awaits epispadias repair. The patient with minor prolapse was treated with reclosure with bilateral innominate and vertical iliac osteotomy, and reclosure with epispadias repair under testosterone stimulation. The patient in whom the pubis was separated and the urethra was lost underwent reclosure, epispadias repair and combined osteotomy under testosterone stimulation. The female patient was treated with reclosure with combined bilateral osteotomy and mobilization of an anterior bladder tube to use as a urethra with no attempt to establish continence.

Complete repair of bladder exstrophy in the newborn period requires experience with the exstrophy condition, appropriate pelvic and patient immobilization, and excellent postoperative care. Complications with this repair are similar to those with modern versions of the staged repair, and its failures are similar. However, penile skin loss with dehiscence or prolapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimulation are valuable adjuncts. Other than the patient who underwent augmentation and continent stoma construction, none has undergone any type of continence procedure, all are awaiting an increase in bladder capacity and followup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy surgeon. While earlier reports of this procedure seem encouraging, complications are real and difficult to manage, even in experienced hands.