The modern staged repair of bladder exstrophy in the female: A contemporary series

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Abstract

Objective

Many changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken.

Patients and methods

From an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988–2005, at a mean age of 2 months (range 4 h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39–65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80–179 cc).

Results

Thirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3 h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1 h day and night. The mean time to daytime continence was 12 months (4–16 months) and to night-time continence was 19 months (10–28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure.

Conclusions

Female classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.

Introduction

Despite the findings of population-based studies suggesting that the incidence of classic bladder exstrophy is equal between the genders, most major reports on the management of bladder exstrophy have a predominance of males in their cohorts [1]. Interestingly, Hugh Hampton Young at our institution performed the first successful closure of classic bladder exstrophy in a female [2]. Since that time, the treatment has undergone significant improvement [3]. Currently, several techniques are being offered in the primary treatment of this disease: complete primary repair of exstrophy as advocated by Grady and Mitchell [4], radical soft-tissue mobilization by Kelly [5] and Cuckow, the Baka-Jacubiak repair [6], to name a few. While enthusiasm abounds, very little in the way of long-term data exists for the above repairs other than the Warsaw repair. The modern staged repair of bladder exstrophy (MSRE) began at our institution [7], and there currently exists a large cohort of patients who have completed repair. The data herein represent a single-institution, single surgeon experience of female patients born with classic exstrophy, and managed with MSRE and a minimum follow up of 5 years after bladder neck repair (BNR).

Section snippets

Patients and methods

A search of an institutionally approved exstrophy database at our institution revealed 198 patients in whom primary exstrophy closure had been performed by one surgeon between 1988 and 2005. Of these, 139 patients underwent MSRE before 2001. Forty-one females were identified in whom MSRE was completed and 5 years of follow-up data were available. The mean follow-up period was 9 years 1 month (range 5–17 years), with 24 patients having more than 10 years of follow up. All patients had undergone

Results

Primary closure was successful in all patients and there were no major complications. Minor complications (n = 5, 12%) were

  • 1.

    premature dislodgement of suprapubic tube, in one case requiring return to the operating room for replacement;

  • 2.

    febrile UTI in one case requiring enteral antibiotics and hospitalization;

  • 3.

    superficial pin-site infections in one case requiring oral antibiotics and wound cleaning;

  • 4.

    superficial wound infection requiring incision and drainage, and oral antibiotics;

  • 5.

    erosion of intrapubic

Discussion

In this treatise, the authors report on a cohort of 41 female exstrophy patients managed by one surgeon at a single center who had completed MSRE prior to 2001 and had a minimum of 5-year follow-up data available for analysis. Voided continence achieved by MSRE was seen in 74% of cases (31 females). Most patients seemed to reach daytime continence within a year of BNR, but the chance of achieving continence has not been found to be associated with the age at which the bladder neck is repaired.

Conclusions

In this paper the authors report on the long-term results of MSRE in a pure group of female classic exstrophy patients. These are mature data with a minimum of 5 years and in many cases greater than 10 years of follow up available. A large number of exstrophy patients treated in the more modern staged fashion are socially continent (84%) and a lesser number (74%) are totally dry both day and night.

The authors believe that these very favorable results can be obtained in all major centers with

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