Coping with bladder exstrophy: diverse results from early attempts at functional urinary tract surgery

Abstract

OBJECTIVESTo report the long-term surgical outcome in a group of patients with bladder exstrophy treated from 1960 to 1982, and to assess physical health, social integration and sexual function, as attempts at functional closure during the development of this surgery resulted in patients with differing surgical status of the genitourinary organs. PATIENTS AND METHODSA review of medical record archives revealed 36 patients; of these, seven had died, six were untraceable and six declined to participate, leaving 15 evaluable subjects (seven men and eight women, mean age at follow-up, 35 years). Data were collected from medical records and direct interviews were conducted using a semi-structured questionnaire, after obtaining informed consent. The Short Form-36 (SF-36) v2 Health Survey instrument was used to assess health and well-being. RESULTSThree patients have retained the use of their bladder, and 11 had initial bladder closure but required (at a mean age of 3.1 years) various urinary diversions. Eight patients had a pelvic osteotomy. Of 27 functioning renal units, 25 are in reasonable to good condition. Six men and six women had genital reconstruction.Four men are capable of penetrative intercourse and ejaculate; six women manage penetrative intercourse and five have orgasms. All patients attended mainstream school and 13 achieved examination success. Nine patients took vocational training and 11 work full-time. Five patients are married and five are in long-term relationships. Two men have achieved three pregnancies and one women has had a child. The mean total SF-36 score (maximum 3600) was 2763 in men and 2235 in women. CONCLUSIONSSurgery for bladder exstrophy has been developing for more than 40 years and the legacy of early attempts at functional closure is a population of adults who have a diversity of lower urinary tracts, good preservation of renal function overall and acceptable sexual function. They are usually robust, healthy and well-adjusted individuals functioning well in society, often in full-time employment and long-term relationships. Adolescent followup must be clearly focused, incorporating a multidisciplinary team approach to facilitate a seamless transition into adulthood. KEYWORDSbladder exstrophy, reconstruction, function, outcome, follow up Authors from Liverpool address the issue of quality of life in exstrophy patients, and assess the long-term results of surgery for this condition. Their study not only yields interesting results, but the reader is also made aware of the problems there can be in conducting such a study.Risk factors for developing chronic renal failure in patients with highgrade VUR are described by authors from Rome. All patients were diagnosed in the first year of life, and the mean (range) follow-up was 6.3 (1-16) years. The findings were of interest, although the reader may not be very surprised to hear that they found bilateral high-grade reflux and existing renal damage to be the main predictors of subsequent chronic renal failure.

Short and long-term quality of life after reconstruction of bladder exstrophy in infancy: preliminary results of the QUALEX (QUAlity of Life of bladder EXstrophy) study

“…Many studies have been published reporting the long-term outcome of reconstruction [5][6][7][8] to assess which kind of reconstruction gives the best results in continence or sexual function [9]. Few studies have evaluated patient QOL [10,11], and only some have used health-related QOL (HRQOL) questionnaires [11][12][13]. The major difficulty is that because of the rarity of this malformation, most studies only have small sample sizes and, therefore, lack the power to identify factors that influence QOL.…”

Bladder exstrophy and male fertility: pregnancies after ICSI with ejaculated or epididymal sperm

“…Their orgasm is normal. Most patients describe an abnormal ejaculation, with one out of seven patients incapable of ejaculation at all (10). A complete absence of ejaculation is rare, even after genital reconstruction, but the emission may be slow and continuous over several hours after orgasm.…”

“…Bennet reported on 3 of 32 (13), Baird et al on 2 out of 5 (10), and Woodhouse on 6 out 72 (9) men with a ureterosigmoidostomy. From the finding it has been clearly revealed that only a few men have penetrative intercourse with subsequent ejaculation followed by a pregnancy (10).…”

“…A complete absence of ejaculation is rare, even after genital reconstruction, but the emission may be slow and continuous over several hours after orgasm. Spermiograms reveal oligoastenoteratospermia (10).…”

“…According to the detailed reports of the patients recruited for the present study, two of five patients without reconstruction fathered four children (10). Our first patient, who had the most extensive reconstructive operations, needed an epididymal punction to collect viable semen.…”