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

Purpose: Complete repair of bladder exstrophy in the newborn includes bladd er, and posterior and penile urethral closure, along with epispadias repair and abdominal wall closure without bladder neck reconstruction. While repo rts have shown the need for early reimplantation of the ureters in 50% of p atients 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 immediat e postoperative complications in these patients. Materials and Methods: Six males and 1 female were referred after "complete repair" procedure in the newborn period without osteotomy. Complete dehisc ence with loss of the proximal urethra and a significant amount of penile s kin 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 closu res before referral and 1 had partial loss of the glans on 1 side. One pati ent 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 o f 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. Results: The 2 patients with complete dehiscence underwent reclosure with b ilateral innominate and vertical iliac osteotomy with replacement of the ur ethral plate with a skin graft and await epispadias repair. The patient wit h major prolapse and 2 prior closures was treated with reclosure, bladder a ugmentation and continent stoma reconstruction, and awaits epispadias repai r. In the male patient with marked prolapse and partial loss of the glans h as undergone reclosure with bilateral innominate and vertical iliac osteoto my was done and he awaits epispadias repair. The patient with minor prolaps e was treated with reclosure with bilateral innominate and vertical iliac o steotomy, and reclosure with epispadias repair under testosterone stimulati on. The patient in whom the pubis was separated and the urethra was lost un derwent reclosure, epispadias repair and combined osteotomy under testoster one stimulation. The female patient was treated with reclosure with combine d bilateral osteotomy and mobilization of an anterior bladder tube to use a s a urethra with no attempt to establish continence. Conclusions: Complete repair of bladder exstrophy in the newborn period req uires experience with the exstrophy condition, appropriate pelvic and patie nt immobilization, and excellent postoperative care. Complications with thi s repair are similar to those with modern versions of the staged repair, an d its failures are similar. However, penile skin loss with dehiscence or pr olapse is particularly difficult because of the paucity of skin already due to the exstrophy condition, for which skin grafting and testosterone stimu lation are valuable adjuncts. Other than the patient who underwent augmenta tion and continent stoma construction, none has undergone any type of conti nence procedure, all are awaiting an increase in bladder capacity and follo wup is short. Complete repair of bladder exstrophy, whether in the newborn or older child, is a formidable task and not for the occasional exstrophy s urgeon. While earlier reports of this procedure seem encouraging, complicat ions are real and difficult to manage, even in experienced hands.