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The staged approach to bladder exstrophy closure and the role of osteotomies.

Since the 1970's, the staged reconstruction of bladder exstrophy has yielded consistent surgical success. The Johns Hopkins Hospital approach begins with early pelvic ring approximation with abdominal wall, bladder, and posterior urethral closure. Within the first 72 hours of life, the malleable pelvis can sometimes be approximated without osteotomies. Beyond this age, the author's prefer a combined vertical iliac and horizontal innominate osteotomy. Second, we typically perform the epispadias closure at 1 year of age. A modified Cantwell-Ransley technique is performed, usually yielding an increase in bladder capacity and very satisfactory results. In the last phase, the modified Young-Dees-Leadbetter continence procedure along with transtrigonal/cephalotrigonal ureteroneocystostomies are performed when the urethra is catheterizable, the bladder capacity is 60 cc or greater, and the child will participate in a postoperative voiding program (typically 4-5 years of age). This applied approach usually results in a continent, voiding patient with pleasing external genitalia and preserved renal function.

Bladder Exstrophy↗

Lower urinary tract reconstruction in patients with bladder exstrophy after failed primary treatment in early childhood.

The results of neonatal surgery for bladder exstrophy are not very satisfactory. A significant percentage of patients present in later childhood or adolescence for correction of their residual deformities. We have reconstructed 26 patients, correcting their entire urogenital and cosmetic deformity in a one-stage procedure. The results show that one-stage total reconstruction is possible and is preferable to serial correction of the various individual abnormalities. The principles of surgical reoperation in adolescence are the same as those now established for the primary correction in neonatal life.

Adolescent↗

Abnormalities of colonic mucin secretion and metabolic changes after internal urinary diversion for bladder exstrophy. A prospective study.

Ten patients with different types of internal urinary diversion for bladder exstrophy were studied prospectively in order to assess metabolic abnormalities and morphological, histochemical and lectin binding changes in the colorectal mucosa. The histochemical and/or lectin binding changes which were found in the majority of patients were identical to those observed in premalignant and malignant conditions of the colon. In some cases they were detectable 3 years after the initial examination but were completely absent from the colorectal mucosa of normal subjects. Metabolic disturbances (metabolic acidosis, increased anion gap, hyperchloraemia) were observed in a substantial number of asymptomatic patients. These findings stress the need for regular endoscopic, histological and metabolic follow-up in these patients and for life-long treatment with bicarbonate or citrate.

Adolescent↗

Factors predisposing to renal scarring: following staged reconstruction of classical bladder exstrophy.

Preservation of renal function is one of the major goals of staged reconstruction of bladder exstrophy. In 68 exstrophy patients who have completed staged reconstruction, 10 (14.7%) have developed renal scarring. A multiple factorial analysis of these patients showed the following factors to be statistically related to the development of renal scarring: (1) one or more febrile urinary tract infections prior to bladder neck reconstruction (P less than .001); (2) failure to utilize antibiotic prophylaxis following initial bladder closure (P less than .005); (3) elevated urinary residuals greater than 50 mL (P less than .02); (4) a diagnosis of elevated urinary residuals greater than 6 months following the onset of continence (P less than .001); and (5) one or more febrile urinary tract infections following bladder neck reconstruction (P less than .005).

Anti-Bacterial Agents↗

[Pyeloureteral lieberkuhn adenocarcinoma after trans-intestinal ureterostomy for bladder exstrophy].

The authors report a case of pyeloureteric adenocarcinoma in a urinary diversion inserted for bladder exstrophy and in a context of chronic pyelonephritis secondary to renal stones. The various aetiopathogenic hypotheses are discussed. Renal stones and chronic inflammation very probably played an important role in the development of this type of adenocarcinoma in the urothelium.

Adenocarcinoma↗

Horizontal pelvic osteotomies for bladder exstrophy. A preliminary report.

We have treated 11 patients aged three days to 15 years with bladder exstrophy by horizontal osteotomies of the innominate bones. The operation was originally used for older patients with severe deformity or failed previous surgery but is now applied as a primary procedure in the first week of life. The osteotomies enable the complex malformations to be corrected in a single operation without turning the patient: the pubic bones can be brought together, the abdominal wall repaired and the bladder closed with reconstruction of the urethra and external genitalia. The early results have been very satisfactory in all cases with only minor complications; we felt that a preliminary report should be made, despite a mean follow-up of only seven months.

Adolescent↗

Long-term results of surgical treatment of bladder exstrophy.

A review of the long-term results of the treatment of 18 cases of bladder exstrophy is reported. Five cases have been operated on before 1961 by ureterosigmoidostomy; 4 (80%) patients have good renal function and have not had any complications, although 1 patient has anal incontinence; 1 patient presented bilateral renoureteral complications, requiring several corrective operations, and has anal incontinence too. Out of 13 cases operated on after 1961, 10 have been followed up from 3 to 14 years. In 1 patient an ileal conduit was performed because of the failure of an ureterosigmoidostomy performed at another hospital 10 years before. In 9 patients a personal technique based on the bladder substitution by a colonic loop plus creation of a sphincteric mechanism by trigonal tubulization was used (enterotrigonal urethroplasty). Morbidity has been high (66.6%), but the end results are very satisfactory: 7 patients (77.7%) are currently living a normal life, with normal renal function and good continence; 1 patient is incontinent and has bilateral reflux and a left retracted kidney; 1 patient has been secondarily diverted by means of a cutaneous Y transuretero-ureterostomy due to gross lreteral dilatation, renal damage and incontinence.

Adolescent↗

Ureterosigmoidostomy in bladder exstrophy.

Experience with ureterosigmoidostomy (US) using a reflux prevention technique in 38 of 48 cases of bladder exstrophy is reviewed. During a follow-up period of 14 years, the results were excellent as to morphological changes of the upper urinary tract, incontinence, psychological development and social behavior. Within their families the patients are considered healthy. Even in children with benign disease, US with reflux prevention performed during the second year of life is the operation of choice for urinary diversion. In the postoperative follow-up it is recommended that sigmoidoscopy be performed once a year in addition to routine controls of the upper urinary tract and metabolic balance.

Adolescent↗

Nephrogenic adenoma with bladder exstrophy and immunosuppression.

Nephrogenic adenoma, an unusual proliferative lesion, often occurs in association with chronic inflammation of the bladder and is rarely found in children. Two cases are reported, one occurring in a young child born with bladder exstrophy, and one in a young female, with both interstitial cystitis and chronic use of immunosuppressive therapy. We believe these are the first case reports of nephrogenic adenoma occurring in this specific clinical setting.

Adenoma↗

Pregnancy in women after repair of bladder exstrophy. Two case reports.

Improved techniques in urinary diversion operations have enabled a large number of patients with bladder exstrophy to achieve urinary control. They can live fairly normal lives and some have achieved pregnancy. Case reports of such exist in the literature. Two more cases are reported in this paper, the pregnancies and their outcome are discussed.

Adult↗