Prior to 2002, all patients underwent cystectomy and creation of a catheterizable Mainz pouch. The patient was taught self-catheterization and advised to practice the same 2 weeks after the surgery. After 2002, patients having a classical exstrophy were also assessed for surgical correction of bladder exstrophy. On bladder biopsies showing no evidence of dysplastic or neoplastic changes, bladder reconstruction and repair was done in two stages. The first stage included ileocytoplasty to increase the bladder capacity, Young Dees bladder neck reconstruction and a secure abdominal wall closure using flaps. The epispadias was repaired by the modified Cantwell-Ransley method at the second stage 6 months later. All these patients had their drain, suprapubic catheter and urethral stent removed at an appropriate time. The patients were advised self-clean intermittent catheterization.

Various social organizations, insurance companies, and charitable institutions supported these patients financially. Four patients presenting prior to 2002 underwent cystectomy with creation of catheterizable modified Mainz pouch. Postoperatively all these patients did well. Self-intermittent catheterization of the abdominal stoma was started from the second week. All these patients underwent epispadias repair 6 months later. There were no intraoperative or postoperative complications. All these four patients have been on follow-up ranging from 6 to 15 years. The patients needed to catheterize once in 4-5 h. The Mainz pouch capacity ranged from 350 to 450 cc. All patients have remained dry during the daytime and only one patient has occasional nighttime urinary leak. The patients have improved in their self-esteem, confidence, and social interaction. Two of these four have been married and have satisfactory erections and sexual relations. The remaining two also have adequate erections.

Since 2002 two patients underwent bladder reconstruction in the first stage which included, augmentation ileocystoplasty, Young Dees bladder neck reconstruction and a secure abdominal wall closure and one patient underwent cystectomy with creation of catheterizable modified Mainz pouch. The patient who underwent cystectomy was the one who had a very small bladder template. All these three patients underwent epispadias repair 6 months later. All these three patients were on clean self-intermittent catheterization. These three patients have similarly improved in their outlook, self-esteem, confidence, and social interaction following surgery. All these three patients have adequate erections. All these three patients are on regular follow-up. The two patientsx who underwent bladder reconstruction had a near normal appearing bladder on cystoscopy a year later. These two patients are on regular follow-up with ultrasonography and urinary examination to monitor the bladder. Interval endoscopic assessment of the bladder has been advised to these patients.

The abdominal wall closure was achieved primarily in all five patients who underwent cystectomy followed by catheterizable Mainz pouch. No flaps were necessary in any of these patients. Two patients who underwent bladder reconstruction, required flaps to secure abdominal wall closure. We have used crossed rectus flaps and rectus sheath flaps for securing abdominal wall closure.

Wound complications occurred specifically in patients with bladder reconstruction which included wound infection and partial wound dehiscence not requiring any active surgical intervention.

Historically, ureterosigmoidostomy was the first form of diversion to be popularized for patients with exstrophy. Although the initial series was associated with multiple metabolic problems, results improved markedly with newer techniques of reimplantation.[4] Ureterosigmoidostomy is favored by some because of the lack of an abdominal stoma. One should be cautious before advising this form of diversion and is certain that anal continence is normal and after the family has been made aware of the potential serious complications, including pyelonephritis, hyperkalemic acidosis, rectal incontinence, ureteral obstruction, and delayed development of malignancy.[6,7]

Matsuda et al.[8] reported a case of a bladder exstrophy in an adult female, treated with cystectomy, construction of a Kock's continent ileal reservoir, and closure of the abdominal fascial defect using alloplastic material. The Kock's ileal reservoir improved the quality of her life not only physically, but also mentally by affording her urinary continence. The Kock's ileal continent reservoir overcomes the problems associated with ureterosigmoidostomy, namely, recurrent infections, pyelonephritis, metabolic and electrolyte disturbances, fecal incontinence, and colonic neoplasia.[7]

Gulati et al.[9] reported management of bladder exstrophy in adulthood. Two adult females presenting with untreated bladder exstrophy underwent cystectomy and modified Mainz pouch. The abdominal wall defect was closed primarily. Both these patients had improved quality of life and renal function. Stein and associates[4,10] treated a group of 128 patients with bladder exstrophy-epispadias with the Mainz technique for ureterosigmoidostomy. D'Elia et al.[11] reported on 26 patients with the exstrophy-epispadias complex who showed excellent continence rates with long-term upper tract preservation when compared with standard ureterosigmoidostomy.

Ozdiler et al. [12] reported a 49-year-old female who presented with exstrophy of bladder. Quattara et al.[13] also reported a 39-year-old male who had presented with exstrophy of bladder.

In our series five patients underwent cystectomy with creation of modified Mainz pouch, four of these underwent this procedure prior to 2002. Due to rarity of these cases, patients were offered cystectomy to remove the bladder plate as well as creation of modified Mainz pouch to render the patient continent. The main concern of these patients at the time of presentation was urinary incontinence, urinary smell, and social ostracism. None of these patients showed any interest regarding management of epispadias component at this time primarily because of ignorance, financial constraint, and the concern for urinary incontinence. However, all five patients showed an interest in epispadias repair once continence was achieved. Second, these patients being in adolescent/young adulthood started appreciating erections and felt the need for sexual relationship/marriage. The two patients who underwent augmentation cystoplasty with bladder neck reconstruction and abdominal wall closure were offered management of epispadias as a staged procedure by us.

Pathak et al.[14] reported the treatment of classic exstrophy of bladder in four adult patients using ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with flaps. Three patients were treated in two stages. The first stage included ileocystoplasty, bladder neck reconstruction, and abdominal wall closure with the use of flaps. The epispadias was repaired in the second stage. In one patient, the reconstruction was completed in a single stage. All patients were continent at the last follow-up visit, with three using self-catheterization and one voiding spontaneously. After surgery, all demonstrated improved social interaction. They concluded that vesical preservation with primary reconstruction of bladder exstrophy in adults was safe and feasible in the absence of significant histologic changes in the bladder mucosa. Pathak et al.[14] believe that although the bladder mucosa will be, inflamed before closure, in the absence of dysplastic changes, it is suitable for bladder neck reconstruction and augmentation and the inflammation subsides with proper closure. Pathak et al.[14] also opined that the patients demonstrated increased self-confidence and improved social interaction as a result of being continent and having a normally placed meatus and a cosmetically improved phallus following surgery.