Bladder exstrophy–epispadias complex (BEEC) is a rare spectrum of genitourinary malformations. Children risk long-term urinary and genital dysfunctions. To achieve a comprehensive understanding, this study aimed to review the literature on generic and disease-specific health-related quality of life (HRQOL) in BEEC patients, and methodologies used. A literature search was conducted in Pubmed/CINAHL/Embase/PsycINFO/Cochrane, from inception to May 2018. A meta-analysis of HRQOL in BEEC patients compared to healthy references was performed. Twenty-one articles (published 1994–2018), describing HRQOL of children and adolescents (n = 5) and adults only (n = 5), or integrated age populations (n = 11), were identified (median sample size 24, loss to follow-up 43%, response rate 84%). Overall HRQOL was reduced in BEEC patients compared to healthy references in 4/4 studies. Impaired physical or general health in BEEC patients has been described in 9 articles, diminished mental health in 11, restricted social health in 10, and sexual health/functioning or body perception impairments in 13 articles. Urinary incontinence was the most common factor related to worse HRQOL (12 studies). In six studies, HRQOL was better than healthy norms. In eligible studies (n = 5), the pooled estimate of the effect of BEEC indicated worse HRQOL for children and adults (0 > effect sizes < 0.5). Thirty-six HRQOL assessments were used, none developed and validated for BEEC. HRQOL in BEEC patients may be negatively impacted, particularly considering mental and social HRQOL. Sexual health/functioning or body perception impairments may be present in adolescents and adults. However, HRQOL is heterogeneously assessed and subsequent findings are differently reported. Additional research is warranted and can be improved.

Health-related quality of life among children, adolescents, and adults with bladder exstrophy-epispadias complex: a systematic review of the literature and recommendations for future research.

The bladder exstrophy-epispadia complex (BEEC) includes malformations with midline closing defects of the lower abdomen and external genitalia. Long-term consequences with urinary incontinence and sexual dysfunction, in spite of multiple surgical interventions, are common and expected to affect the patient's health-related quality of life (HRQOL). The extent and the predictive factors are, however, not known. New patient-reported outcome research is emerging, but valid and reliable condition-specific HRQOL are still missing. The aim of this review is to summarize and discuss the latest published reports (2015-2019) on HRQOL in patients with the BEEC and its relationship to incontinence and sexual factors.

Health-Related Quality of Life in Patients with the Bladder Exstrophy-Epispadias Complex and Relationship to Incontinence and Sexual Factors: A Review of the Recent Literature.

Bladder exstrophy: we need to improve. A lot

This group of anomalies comprises a spectrum of lower midline disorders affecting the pelvic ring, the lower abdominal wall, the bladder, and the genitalia. Predominantly occurring in males, the commonest is classic bladder exstrophy which presents antenatally or at birth with an open bladder plate continuous with a urethral plate which is open on the dorsum of the penis. Various operations or combinations of operations are described to address closing the bladder and reconstructing the penis/genitalia and these have evolved over the past 20 years with spontaneous voiding continence as a primary goal. Cloacal Exstrophy is much rarer and combines bladder exstrophy, exstrophy of the hindgut with a more severe abdominal wall and genital defect. This most challenging group of patients may also have a myriad of other non-urogenital abnormalities which affect their outcomes and necessitate a coordinated multi-disciplinary approach, although most will survive into adult life. The surgical goals are to establish a sound abdominal wall closure and obtain control of bowel and bladder function with a permanent stoma and a catheterizable reservoir, respectively, optimizing function and quality of life. Primary epispadias is perhaps wrongly considered to be a minor variant of the spectrum, whose continence outcome is additionally affected by a degree of dysplasia/hypoplasia of the bladder outlet. Voiding continence may be hard to achieve in these patients, and satisfactory genital outcomes are a challenge, especially in boys. The rare nature of these conditions (with a combined incidence of around 1 in 20,000 births) suggests that they are better treated in specialized centers where focusing the experience and skills of fewer surgeons will provide the starting point for further developments. 

Bladder Exstrophy, Epispadias, and Cloacal Exstrophy

Objective This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function. Design Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire. Setting Maryland, United States. Participants Review of a single-institution exstrophy-epispadias complex database resulted in 25 adult female patients with a history of POP treated at the authors' institution. Eleven patients participated and were included in the analysis. Main outcome measures Urinary continence and sexual function. Results All participants underwent surgical repair for prolapse, with 7 (63.6%) experiencing unsuccessful initial repair and subsequent recurrence. Median total number of POP repairs was 2.5 (1-4). After correction of POP, patients reported a median improvement in International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form scores of 11 (21 to -1) of 21 and in POP-Urinary Incontinence Sexual Questionnaire scores of 9.5 (6.5-33.0) of 48.0. With regard to urinary continence, 6 (54.5%) patients presently reported no incontinence, 3 (27.3%) reported mild incontinence, and 2 (18.2%) reported continuous incontinence. Conclusions Pelvic organ prolapse poses significant reductions in quality of life for women born with exstrophy, with effects on urinary continence and sexual function. Identification and correction of prolapse seems to result in notable improvements in the lives of these patients.

Patient-Reported Impact of Pelvic Organ Prolapse on Continence and Sexual Function in Women With Exstrophy-Epispadias Complex.

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Patient-Reported Impact of Pelvic Organ Prolapse on Continence and Sexual Function in Women With Exstrophy-Epispadias Complex.