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Pelvic Organ Prolapse in Patients with Congenital Genitourinary Conditions

  • Stress Incontinence and Prolapse (S Reynolds, Section Editor)
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Abstract

Purpose of Review

We discuss the etiology of pelvic organ prolapse in women with congenital conditions, the features and considerations that distinguish it from that of the general population, and the treatment strategies. We aim to provide the adult urologist with the latest data to guide diagnosis, counseling, and treatment of pelvic organ prolapse.

Recent Findings

Pelvic organ prolapse may be asymptomatic due to altered sensation in this population, but it may also present atypically, including with bladder and bowel dysfunction, difficulty with clean intermittent catheterization, hygiene challenges, and ulceration of skin and soft tissues. Thus, pelvic organ prolapse in those with congenital genitourinary conditions warrants regular assessment. Management should be case- and etiology-dependent, based on incorporation of physiologic and anatomic risks as well as patient goals. Spina bifida and bladder exstrophy are the conditions in which the prevalence, etiology, and management of pelvic organ prolapse have been studied in most detail.

Summary

As clinical care has improved over the past 50 years, individuals with congenital genitourinary conditions now have longer life expectancy and quality of life as adults. As a result of their underlying congenital conditions and prior reconstructive urological procedures, many of these women are uniquely subject to acquired urologic conditions such as pelvic organ prolapse as they age.

Clinical Trial Registration

This study is not a clinical trial and thus does not warrant registration as such.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Wood D. Adolescent urology: develo** lifelong care for congenital anomalies. Nat Rev Urol. 2014;11(5):289–96.

    Article  Google Scholar 

  2. Roth J, Elliott S, Szymanski K, Cain M, Misseri R. The need for specialized training for adults with congenital urologic conditions: differences in opinion among specialties. Central European Journal of Urology. 2020;73(1):62.

    Google Scholar 

  3. •• Wood D, Baird A, Carmignani L, et al. Lifelong congenital urology: the challenges for patients and surgeons. Eur Urol. 2019;75(6):1001–7. Evaluation of the barriers to and strategies for a successful transition from pediatric to adult urologic care for those with congenital genitourinary conditions.

    Article  Google Scholar 

  4. Berry JG, Bloom S, Foley S, Palfrey JS. Health inequity in children and youth with chronic health conditions. Pediatrics. 2010;126(Supplement_3):S111–9.

    Article  Google Scholar 

  5. •• Lai J, Meza J, Oot A, et al. Management of pelvic organ prolapse in the adult congenital genitourinary patient. Urology. 2022;161:142–5. Retrospective review of pelvic organ prolapse management and outcomes in individuals with congenital genitourinary conditions.

    Article  Google Scholar 

  6. Loftus C, Wood H. Emerging concepts in transitional care of congenital genitourinary reconstructions. Current Bladder Dysfunction Reports. 2015;10(4):427–35.

    Article  Google Scholar 

  7. Loftus CJ, Wood HM. Congenital causes of neurogenic bladder and the transition to adult care. Trans Androl Urol. 2016;5(1):39.

    Google Scholar 

  8. Doshani A, Teo RE, Mayne CJ, Tincello DG. Uterine prolapse. BMJ. 2007;335(7624):819–23.

    Article  Google Scholar 

  9. Vergeldt TF, Weemhoff M, IntHout J, Kluivers KB. Risk factors for pelvic organ prolapse and its recurrence: a systematic review. Int Urogynecol J. 2015;26(11):1559–73.

    Article  Google Scholar 

  10. Liu JS, Vo AX, Doolittle J, Hamoui N, Lewicky-Gaupp C, Kielb SJ. Characterizing pelvic organ prolapse in adult spina bifida patients. Urology. 2016;97:273–6.

    Article  Google Scholar 

  11. •• Kaufman MR. Pelvic organ prolapse and pregnancy in the female bladder exstrophy patient. Curr Urol Rep. 2018;19(3):1–7. Literature review of the epidemiology, etiology, and management of pelvic organ prolapse and pregnancy in those with a history of bladder exstrophy.

    Article  Google Scholar 

  12. • De Win G, Dautricourt S, Deans R, et al. Fertility and sexuality issues in congenital lifelong urology patients: female aspects. World J Urol. 2021;39(4):1021–7. Literature review regarding the fertility and sexuality in females with congenital genitourinary conditions including bladder exstrophy and spina bifida.

    Article  Google Scholar 

  13. Nakhal RS, Deans R, Creighton SM, Wood D, Woodhouse CR. Genital prolapse in adult women with classical bladder exstrophy. Int Urogynecol J. 2012;23(9):1201–5.

    Article  Google Scholar 

  14. Lamers BH, Broekman BM, Milani AL. Pessary treatment for pelvic organ prolapse and health-related quality of life: a review. Int Urogynecol J. 2011;22(6):637–44.

    Article  Google Scholar 

  15. •• Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers. 2015;1(1):1–18. Detailed and definitive synopsis of spina bifida.

    Article  Google Scholar 

  16. Rintoul NE, Sutton LN, Hubbard AM, et al. A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics. 2002;109(3):409–13.

    Article  Google Scholar 

  17. Henn EW, Juul L, van Rensburg K. Pelvic organ prolapse in the neonate: report of two cases and review of the literature. Int Urogynecol J. 2015;26(4):613–5.

    Article  Google Scholar 

  18. El Akri M, Brochard C, Hascoet J, et al. Risk of prolapse and urinary complications in adult spina bifida patients with neurogenic acontractile detrusor using clean intermittent catheterization versus Valsalva voiding. Neurourol Urodyn. 2019;38(1):269–77.

    Article  Google Scholar 

  19. •• Khavari R, Tokaz MC, Stewart JN, Boone TB. Pelvic organ prolapse in female patients presenting to transitional urology care clinic. J Urol. 2015;194(6):1654–8. Retrospective review regarding presentations and treatment of pelvic organ prolapse in adults with a history of congenital genitourinary conditions.

    Article  Google Scholar 

  20. Gill EJ, Hurt WG. Pathophysiology of pelvic organ prolapse. Obstet Gynecol Clin North Am. 1998;25(4):757–69.

    Article  CAS  Google Scholar 

  21. Summers A, Winkel LA, Hussain HK, DeLancey JO. The relationship between anterior and apical compartment support. Am J Obstet Gynecol. 2006;194(5):1438–43.

    Article  Google Scholar 

  22. Mouritsen L, Larsen JP. Symptoms, bother and POPQ in women referred with pelvic organ prolapse. Int Urogynecol J. 2003;14(2):122–7.

    Article  Google Scholar 

  23. Beverly CM, Walters MD, Weber AM, Piedmonte MR, Ballard LA. Prevalence of hydronephrosis in patients undergoing surgery for pelvic organ prolapse. Obstet Gynecol. 1997;90(1):37–41.

    Article  CAS  Google Scholar 

  24. • Streur CS, Schafer CL, Garcia VP, Quint EH, Sandberg DE, Wittmann DA. “If everyone else is having this talk with their doctor, why am I not having this talk with mine?”: the experiences of sexuality and sexual health education of young women with spina bifida. J Sex Med. 2019;16(6):853–9. (Qualitative study regarding the perceptions of women with spina bifida regarding experiences with sexuality and sexual health education)

    Article  Google Scholar 

  25. Ebert A-K, Reutter H, Ludwig M, Rösch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis. 2009;4(1):1–17.

    Article  Google Scholar 

  26. •• Gearhart JP, Mathews R. Exstrophy-epispadias complex. Campbell-Walsh. Urology. 2007;4:3325–78. Detailed and definitive synopsis of bladder exstrophy.

    Google Scholar 

  27. Stec AA. Embryology and bony and pelvic floor anatomy in the bladder exstrophy-epispadias complex. Elsevier; 2011:66–70.

  28. • Peng Z, Huang Y, Tang W, et al. Pelvic floor anatomical variations in children with exstrophy-epispadias complex using MRI. Urology. 2022. Anatomical comparison between children with exstrophy and controls using magnetic resonance imaging.

  29. Mathews R, Gan M, Gearhart J. Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int. 2003;91(9):845–9.

    Article  CAS  Google Scholar 

  30. Anusionwu I, Baradaran N, Trock BJ, Stec AA, Gearhart JP, Wright EJ. Is pelvic osteotomy associated with lower risk of pelvic organ prolapse in postpubertal females with classic bladder exstrophy? J Urol. 2012;188(6):2343–6.

    Article  Google Scholar 

  31. 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(1):140–5.

    Article  Google Scholar 

  32. Deans R, Banks F, Liao L-M, 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(6):496.e1-496.e6.

    Article  Google Scholar 

  33. Ebert AK, Falkert A, Brandl R, Hirschfelder H, Koller M, Rösch WH. Pelvic-floor imaging using three-dimensional ultrasonography and magnetic resonance imaging in the long term follow-up of the bladder-exstrophy-epispadias complex. BJU Int. 2010;105(2):248–53.

    Article  Google Scholar 

  34. Muir TW, Aspera AM, Rackley RR, Walters MD. Recurrent pelvic organ prolapse in a woman with bladder exstrophy: a case report of surgical management and review of the literature. Int Urogynecol J. 2004;15(6):436–8.

    Article  Google Scholar 

  35. Benson AD, Kramer BA, McKenna PH, Schwartz BF. Robot-assisted laparoscopic sacrouteropexy for pelvic organ prolapse in classical bladder exstrophy. J Endourol. 2010;24(4):515–9.

    Article  Google Scholar 

  36. Miles-Thomas J, Gearhart JP, Gearhart SL. An initial evaluation of pelvic floor function and quality of life of bladder exstrophy patients after ureterosigmoidostomy. J Gastrointest Surg. 2006;10(4):473–7.

    Article  Google Scholar 

  37. Fisch M. Commentary on: Urogynaecological and obstetric issues in women with the exstrophy-epispadias complex. BJU Int. 2003;91(9):845–9.

    Article  Google Scholar 

  38. Feldkamp ML, Botto LD, Amar E, et al. Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. Wiley Online Library; 2011:333–343.

  39. Catti M, Paccalin C, Rudigoz R-C, Mouriquand P. Quality of life for adult women born with bladder and cloacal exstrophy: a long-term follow up. J Pediatr Urol. 2006;2(1):16–22.

    Article  Google Scholar 

  40. Wood RJ, Reck-Burneo CA, Levitt MA. Cloacal malformations: technical aspects of the reconstruction and factors which predict surgical complexity. Front Pediatr. 2019;7:240.

    Article  Google Scholar 

  41. Tonni G, Ida V, Alessandro V, Bonasoni MP. Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal Pediatr Pathol. 2013;32(1):13–24.

    Article  Google Scholar 

  42. Achour R, Bennour W, Ksibi I, et al. Prune belly syndrome: approaches to its diagnosis and management. Intractable Rare Dis Res. 2018;7(4):271–4.

    Article  Google Scholar 

  43. Wu YW, Day SM, Strauss DJ, Shavelle RM. Prognosis for ambulation in cerebral palsy: a population-based study. Pediatrics. 2004;114(5):1264–71.

    Article  Google Scholar 

  44. Marciniak CM, Lee J, Jesselson M, Gaebler-Spira D. Cross-sectional study of bowel symptoms in adults with cerebral palsy: prevalence and impact on quality of life. Arch Phys Med Rehabil. 2015;96(12):2176–83.

    Article  Google Scholar 

  45. Hendren WH, Crawford JD. Adrenogenital syndrome: the anatomy of the anomaly and its repair. Some new concepts. J Pediatric Surg. 1969;4(1):49–58.

    Article  CAS  Google Scholar 

  46. Fuchs J, Warmann SW, Seitz G, Schäfer J, Schröder M, Obermayr F. Laparoscopically assisted vaginal pull-through for high urogenital sinus: a new surgical technique. Urology. 2012;79(5):1180–3.

    Article  Google Scholar 

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Authors and Affiliations

  1. Department of Urology, Glickman Urological and Kidney Institute, Cleveland Clinic Foundation, Cleveland, OH, 44195, USA

    Glenn T. Werneburg, Sandip P. Vasavada & Hadley M. Wood

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  1. Glenn T. Werneburg
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  3. Hadley M. Wood
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Correspondence to Hadley M. Wood.

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Conflict of Interest

GW: no disclosures.

SPV: consultant: Medtronic, Allergan, BlueWind, Axonics.

HW: Site-PI, Boston Scientific; Physician Advisory Council member, Spina Bifida Association; Site-PI, National Spina Bifida Patient Registry US Centers for Disease Control.

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Werneburg, G.T., Vasavada, S.P. & Wood, H.M. Pelvic Organ Prolapse in Patients with Congenital Genitourinary Conditions. Curr Bladder Dysfunct Rep 17, 263–270 (2022). https://doi.org/10.1007/s11884-022-00669-0

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