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DOI: 10.1055/s-0040-1713178
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
Publication History
24 April 2020
08 May 2020
Publication Date:
21 June 2020 (online)
![](https://www.thieme-connect.de/media/ejps/202003/lookinside/thumbnails/10-1055-s-0040-1713178_205251rev-1.jpg)
Abstract
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.
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References
- 1 Ebert AK, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis 2009; 4: 23
- 2 Jayachandran D, Bythell M, Platt MW, Rankin J. Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol 2011; 186 (05) 2056-2060
- 3 Reinfeldt Engberg G, Mantel Ä, Fossum M, Nordenskjöld A. Maternal and fetal risk factors for bladder exstrophy: A nationwide Swedish case-control study. J Pediatr Urol 2016; 12 (05) 304.e1-304.e7
- 4 Baka-Jakubiak M. Combined bladder neck, urethral and penile reconstruction in boys with the exstrophy-epispadias complex. BJU Int 2000; 86 (04) 513-518
- 5 Cuckow P. The bladder exstrophy epispadias complex. In: Thomas DFM, Duffy PG, Rickwood AMK. , eds. Essentials of Pediatric Urology London. United Kingdom: Informa Health Care; 2008: 199-212
- 6 Gearhart JP. Bladder exstrophy: staged reconstruction. Curr Opin Urol 1999; 9 (06) 499-506
- 7 Grady RW, Carr MC, Mitchell ME. Complete primary closure of bladder exstrophy. Epispadias and bladder exstrophy repair. Urol Clin North Am 1999; 26 (01) 95-109
- 8 Kertai MA, Rösch WH, Brandl R, Hirschfelder H, Zwink N, Ebert AK. Morphological and functional hip long-term results after exstrophy repair. Eur J Pediatr Surg 2016; 26 (06) 508-513
- 9 Mushtaq I, Garriboli M, Smeulders N. , et al. Primary bladder exstrophy closure in neonates: challenging the traditions. J Urol 2014; 191 (01) 193-197
- 10 Stein R, Fisch M, Stöckle M, Hohenfellner R. Urinary diversion in bladder exstrophy and incontinent epispadias: 25 years of experience. J Urol 1995; 154 (03) 1177-1181
- 11 Woodhouse CR, North AC, Gearhart JP. Standing the test of time: long-term outcome of reconstruction of the exstrophy bladder. World J Urol 2006; 24 (03) 244-249
- 12 Maruf M, Manyevitch R, Michaud J. , et al. Urinary continence outcomes in classic bladder exstrophy: a long-term perspective. J Urol 2020; 203 (01) 200-205
- 13 Diseth TH, Bjordal R, Schultz A, Stange M, Emblem R. Somatic function, mental health and psychosocial functioning in 22 adolescents with bladder exstrophy and epispadias. J Urol 1998; 159 (05) 1684-1689
- 14 Rubenwolf P, Thomas C, Thüroff JW, Stein R. Sexual function and fertility of women with classic bladder exstrophy and continent urinary diversion. J Urol 2016; 196 (01) 140-145
- 15 Rubenwolf P, Thomas C, Thüroff JW, Stein R. Sexual function, social integration and paternity of males with classic bladder exstrophy following urinary diversion. J Urol 2016; 195 (02) 465-470
- 16 Deans R, Banks F, Liao LM, Wood D, Woodhouse C, Creighton SM. Reproductive outcomes in women with classic bladder exstrophy: an observational cross-sectional study. Am J Obstet Gynecol 2012; 206 (06) 496.e1-496.e6
- 17 Cervellione RM, Phillips T, Baradaran N, Asanuma H, Mathews RI, Gearhart JP. Vaginoplasty in the female exstrophy population: outcomes and complications. J Pediatr Urol 2010; 6 (06) 595-599
- 18 Nakhal RS, Deans R, Creighton SM, Wood D, Woodhouse CR. Genital prolapse in adult women with classical bladder exstrophy. Int Urogynecol J Pelvic Floor Dysfunct 2012; 23 (09) 1201-1205
- 19 Pennison MC, Mednick L, Grant R. , et al. A survey to assess body and self-image in individuals with bladder exstrophy: a call for psychosocial support. J Urol 2013; 190 (04) 1572-1576
- 20 Park W, Zwink N, Rösch WH. , et al. Sexual function in adult patients with classic bladder exstrophy: a multicenter study. J Pediatr Urol 2015; 11 (03) 125.e1-125.e6
- 21 Wilson CJ, Pistrang N, Woodhouse CR, Christie D. The psychosocial impact of bladder exstrophy in adolescence. J Adolesc Health 2007; 41 (05) 504-508
- 22 Gobet R, Weber D, Horst M, Yamamoto S, Fischer J. Long-term followup (37 to 69 years) in patients with bladder exstrophy treated with ureterosigmoidostomy: psychosocial and psychosexual outcomes. J Urol 2009; 182 (04) 1819-1823
- 23 Suominen JS, Santtila P, Taskinen S. Sexual function in patients operated on for bladder exstrophy and epispadias. J Urol 2015; 194 (01) 195-199
- 24 Cella DF. Quality of life: the concept. J Palliat Care 1992; 8 (03) 8-13
- 25 Bullinger M. [The concept of quality of life in medicine: its history and current relevance] (in German). Z Evid Fortbild Qual Gesundhwes 2014; 108 (2-3): 97-103
- 26 Dellenmark-Blom M, Sjöström S, Abrahamsson K, Holmdahl G. 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. Qual Life Res 2019; 28 (06) 1389-1412
- 27 Benjamin K, Vernon MK, Patrick DL, Perfetto E, Nestler-Parr S, Burke L. Patient-reported outcome and observer-reported outcome assessment in rare disease clinical trials: an ISPOR COA emerging good practices task force report. Value Health 2017; 20 (07) 838-855
- 28 Amesty MV, Chocarro G, Lobato R. , et al. Quality of life in female epispadias. Eur J Pediatr Surg 2016; 26 (03) e1
- 29 Baumgartner TS, Lue KM, Sirisreetreerux P. , et al. Long-term sexual health outcomes in men with classic bladder exstrophy. BJU Int 2017; 120 (03) 422-427
- 30 Bujons A, Lopategui DM, Rodríguez N, Centeno C, Caffaratti J, Villavicencio H. Quality of life in female patients with bladder exstrophy-epispadias complex: Long-term follow-up. J Pediatr Urol 2016; 12 (04) 210.e1-210.e6
- 31 da Cruz JA, de Mattos B, Srougi M. , et al. Quality of life in young adult patients treated for bladder exstrophy. Cent European J Urol 2016; 69 (02) 221-224
- 32 Deans R, Liao LM, Wood D, Woodhouse C, Creighton SM. Sexual function and health-related quality of life in women with classic bladder exstrophy. BJU Int 2015; 115 (04) 633-638
- 33 Di Grazia M, Pellizzoni S, Tonegatti LG, Rigamonti W. Psychosexual development management of bladder exstrophy epispadias in complex patients. J Pediatr Urol 2017; 13 (02) 199.e1-199.e5
- 34 Ebert AK, Lange T, Reutter H. , et al. Evaluation of sexual function in females with exstrophy-epispadias-complex: A survey of the multicenter German CURE-Net. J Pediatr Urol 2017; 13 (02) 183.e1-183.e6
- 35 Everett RG, Lue KM, Reddy SS. , et al. Patient-reported impact of pelvic organ prolapse on continence and sexual function in women with exstrophy-epispadias complex. Female Pelvic Med Reconstr Surg 2017; 23 (06) 377-381
- 36 Hurrell RA, Fullwood C, Keys J. , et al. Psychosocial screening at paediatric BEEC clinics: a pilot evaluation study. J Pediatr Urol 2015; 11 (02) 79.e1-79.e6
- 37 Reynaud N, Courtois F, Mouriquand P. , et al. Male sexuality, fertility, and urinary continence in bladder exstrophy-epispadias complex. J Sex Med 2018; 15 (03) 314-323
- 38 Taskinen S, Suominen JS, Mattila AK. Health-related quality of life and mental health in adolescents and adults operated for bladder exstrophy and epispadias. Urology 2015; 85 (06) 1515-1519
- 39 Traceviciute J, Zwink N, Jenetzky E. , et al. Sexual function and quality of life in adult male individuals with exstrophy-epispadias complex-a survey of the German CURE-Network. Urology 2018; 112: 215-221
- 40 Zhu X, Klijn AJ, de Kort LMO. Urological, sexual, and quality of life evaluation of adult patients with exstrophy-epispadias complex: long-term results from a dutch cohort. Urology 2019; 136: 272-277
- 41 Lloyd JC, Spano SM, Ross SS, Wiener JS, Routh JC. How dry is dry? A review of definitions of continence in the contemporary exstrophy/epispadias literature. J Urol 2012; 188 (05) 1900-1904
- 42 Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. Incontinence affects health-related quality of life in children and adolescents with spina bifida. J Pediatr Urol 2018; 14 (03) 279.e1-279.e8
- 43 Szymanski KM, Cain MP, Whittam B, Kaefer M, Rink RC, Misseri R. All incontinence is not created equal: impact of urinary and fecal incontinence on quality of life in adults with Spina Bifida. J Urol 2017; 197 (3 Pt 2): 885-891
- 44 Anderson DL, Murray CD, Hurrell R. Experiences of intimacy among people with bladder exstrophy. Qual Health Res 2013; 23 (12) 1600-1612
- 45 Seliner B, Gobet R, Metzenthin P, Spirig R. [Living with bladder exstrophy-the patients' perspective]. Pflege 2010; 23 (03) 163-172
- 46 Di Grazia M, Pellizzoni S, Tonegatti LG, Rigamonti W. Qualitative questionnaire on the psychosocial wellbeing of mothers of children with BEEC. J Pediatr Urol 2017; 13 (01) 55.e1-55.e6
- 47 EuroQol Group. EuroQol--a new facility for the measurement of health-related quality of life. Health Policy 1990; 16 (03) 199-208
- 48 Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 2001; 39 (08) 800-812
- 49 Ware Jr JE, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care 1992; 30 (06) 473-483
- 50 McHorney CA, Ware Jr. JE, Raczek AE. The MOS 36-Item Short-Form Health Survey (SF-36): II. Psychometric and clinical tests of validity in measuring physical and mental health constructs. Med Care 1993; 31 (03) 247-263
- 51 McHorney CA, Ware Jr. JE, Lu JF, Sherbourne CD. The MOS 36-item Short-Form Health Survey (SF-36): III. Tests of data quality, scaling assumptions, and reliability across diverse patient groups. Med Care 1994; 32 (01) 40-66
- 52 Avery K, Donovan J, Peters TJ, Shaw C, Gotoh M, Abrams P. ICIQ: a brief and robust measure for evaluating the symptoms and impact of urinary incontinence. Neurourol Urodyn 2004; 23 (04) 322-330
- 53 Brookes ST, Donovan JL, Wright M, Jackson S, Abrams P. A scored form of the Bristol Female Lower Urinary Tract Symptoms questionnaire: data from a randomized controlled trial of surgery for women with stress incontinence. Am J Obstet Gynecol 2004; 191 (01) 73-82
- 54 Barry MJ, Fowler Jr FJ, O'Leary MP. , et al; The Measurement Committee of the American Urological Association. The American Urological Association symptom index for benign prostatic hyperplasia. J Urol 1992; 148 (05) 1549-1557