Neonatal total reconstruction of bladder exstrophy-epispadias complex is the treatment of choice. Primary reconstruction is increasingly performed with good results. Adults presenting with exstrophy is very rare, as the obvious deformity with leaking urine cannot go unnoticed and can lead to difficulties due to upper tract dysfunction, difficulty in secure abdominal closure, and malignant potential of the bladder remnant.
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
Matsuda et al
] 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
] 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
] treated a group of 128 patients with bladder exstrophy-epispadias with the Mainz technique for ureterosigmoidostomy. D'Elia et al
] 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
] reported a 49-year-old female who presented with exstrophy of bladder. Quattara et al
] 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
] 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
] 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
] 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.
] expressed that it was important to save and use bladder template for it had two advantages. First, using bladder template meant taking less bowel and second, if ureteral reimplantation was necessary, the bladder template was a much better substrate for reimplantation than a bowel wall. Though we have not compared the patients undergoing the two different procedures, we feel that the patients get well adjusted psychosexually and psychologically primarily because they become dry in both these procedures.