One-stage correction of epispadia to Macedo in a girl 3 years old

Introduction. Epispadias is an extremely rare disease. Epispadias in women is 5–6 times less common than in men. The incidence of female epispadias ranges from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed by a thorough examination of the genitals. Treatment of epispadias is only surgical, which is quite difficult and requires experience. The literature that discusses female epispadias is very limited. In this article, we would like to report on the surgical treatment of isolated epispadias in a 3-year-old girl at the Almazov National Medical Research Center.

Clinical case. A 3-year-old girl presented with the main complaint of urinary incontinence since birth, during the day and at night. Urinary incontinence was not caused by physical activity and was not aggravated by eating/drinking. Physical examination showed that the external genital organs of the patient have underdeveloped labia minora, abnormal urethra, bifurcated clitoris. Laboratory results were within the normal range. Voiding cystourethrography revealed urine leakage during the filling phase. The bladder wall was normal, there was no vesicoureteral reflux. The patient underwent simultaneous surgical interventions, consisting in plastic surgery of the urethra and reconstruction of the bladder neck, plastic surgery of the clitoris and labia minora. There were no intraoperative and postoperative complications. After 1 week and 6 months of observation, the patient achieved urinary retention, the surgical wound healed primarily.

Discussion. Epispadias is a rare condition that can vary in severity from mild to severe. Severely, splitting of the entire urethra occurs, involving the bladder neck, causing permanent urinary incontinence in the patient. Cases of epispadias are quite difficult to diagnose. The doctor should dilute the labia majora and carefully conduct a physical examination. The objective goals of the surgical treatment of epispadias are to achieve urinary retention, restore the anatomy, function and cosmetic appearance of the urethra and genitals. One-stage reconstruction is a modern surgical technique used to treat isolated epispadias in women.

Conclusion. Epispadias in women is a rare congenital anomaly that is often not detected in childhood. For isolated epispadias in a girl, one-stage surgical correction is currently preferred.

1. Anand S, Lotfollahzadeh S. Epispadias [cited 2021 Jun 13]. Available from, in: StatPearls [Internet], StatPearls Publishing, Treasure Island (FL), 2021, http://www.ncbi.nlm.nih.gov/books/NBK563180/.

2. Epispadias [Internet], Available from, 2021 Jun 13, https://www.hopkinsmedicine.org/health/conditionsand-diseases/epispadias.

3. Allen L, Rodjani A, Kelly J, et al. Female epispadias: are we missing the diagnosis? BJU Int. 2004 Sep;94(4):613–5. DOI: 10.1111/j.14644096.2004.04920.x.

4. Jayachandran D, Bythell M, Platt MW, Rankin J. Bladder exstrophies and epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival in the north of England, Pediatr. Res. 2011 Nov;70 (5):86–86.

5. Atilgan D, Uluocak N, Erdemir F, Parlaktas BS. Female epispadias: a case report and review of the literature. Kaohsiung J Med Sci. 2009 Nov;25(11):613–6. DOI: 10.1016/S1607-551X(09)70565-0.

6. Jonuzi A, Popovic N, Zvizdic Z, et al. Female Epispadias Presenting as Urinary Incontinence. APSP J Case Rep. 2017 Mar 18;8(2):10. DOI: 10.21699/ajcr.v8i2.548.

7. Shetty MV, Bhaskaran A, Sen TK. Female epispadias. Afr J Paediatr Surg. 2011 May-Aug;8(2):215–7. DOI: 10.4103/0189-6725.86066.

8. Ali S, Moosa H, Khan SA. Isolated Female Epispadias with Urinary Incontinence. J Coll Physicians Surg Pak. 2020 Apr;30(4):438–439. DOI: 10.29271/jcpsp.2020.04.438.

9. Arslan MS, Arslan S, Zeytun H, et al. A case of isolated female epispadias and our approach to treatment, Afr. J. Urol. 2016 Sep 1;22(3):193–195.

10. Urinalysis [Internet], Available from, 2021 Jun 16, https://webpath.med.utah.edu/TUTORIAL/URINE/URINE.html.

11. Gearhart JP. Exstrophy, epispadias and other bladder anomalies, in: P.C. Walsh, In. (Eds.), Campbell’s Urology, 7th ed., WB Saunders, Philadelphia, 1998, p. 2136.

12. Bansal, Modified Cantwell-Ransley Repair in Penopubic Epispadias With Continent Bladder [Internet], 2021 Jun 15. http://www.astrocyte.in/article.asp?issn=2349-0977;year=2015;volume=2;issue=1;spage=49;epage=51;aulast=Bansal#ref3.

13. Yoham AL, Casadesus D. Embryology, urethral folds [cited 2021 Jun 16]. Available from, in: StatPearls [Internet], StatPearls Publishing, Treasure Island (FL), 2021, http://www.ncbi.nlm.nih.gov/books/NBK560572/.

14. Alyami F, Fernandez N, Lee L, et al. Long-term follow-up after traditional versus modified perineal approach in the management of female epispadias. J Pediatr Urol. 2017 Oct;13(5):497.e1–497.e5. DOI: 10.1016/j.jpurol.2017.02.012.

15. Bhat AL, Bhat M, Sharma R, Saxena G. Singlestage perineal urethroplasty for continence in female epispadias: a preliminary report. Urology. 2008 Aug;72(2):300–3; discussion 303–4. DOI: 10.1016/j.urology.2007.09.073.

16. Young HH. An operation for the cure of incontinence associated with epispadias, J. Urol. 1922 Jan; 7(1):1–32.

17. Mitchell ME, Brito CG, Rink RC. Cloacal exstrophy reconstruction for urinary continence. J Urol. 1990 Aug;144(2 Pt 2):554–8; discussion 562–3. DOI: 10.1016/s0022-5347(17)39521-6.

18. Marshall VF, Marchetti AA, Krantz KE. The correction of stress incontinence by simple vesicourethral suspension. Surg Gynecol Obstet. 1949 Apr;88(4):509–18.

19. King LR, Wendel RM. A new application for transvaginal plication in the treatment of girls with total urinary incontinence due to epispadias or hypospadias. J Urol. 1969 Dec;102(6):278–82.

20. Tanagho EA. Bladder neck reconstruction for total urinary incontinence: 10 years experience. J Urol. 1981 Mar;125(3):321–6. DOI: 10.1016/s0022-5347(17)55024-7.

21. Gearhart JP, Peppas DS, Jeffs RD. Complete genitourinary reconstruction in female epispadias. J Urol. 1993 May;149(5):1110–3. DOI: 10.1016/s00225347(17)36310-3.

22. Hendren WH, Congenital female epispadias with incontinence, J. Urol. 1981 Apr;125 (4):558–564.

23. Kramer SA, Kelalis PP. Surgical correction of female epispadias. Eur Urol. 1982;8(6):321–4. DOI: 10.1159/000473547.

24. Leclair MD, Faraj S, Villemagne T, et al. Primary female epispadias: Perineal approach or Kelly repair? J Pediatr Urol. 2018 Feb;14(1):33–39. DOI: 10.1016/j.jpurol.2017.08.017.

25. de Jong TP, Dik P, Klijn AJ. Female epispadias repair: a new 1-stage technique. J Urol. 2000 Aug;164(2):492–4. DOI: 10.1097/00005392-20000800000070.

26. Manzoni GR, Ransley PG. Primary surgical intervention for female epispadias, J. Pediatr. Urol. (2007;3:s73.) In.

27. Kajbafzadeh AM, Talab SS, Elmi A, Tourchi A. Single-stage subsymphyseal cystoscopic-guided bladder neck plication and urethrogenitoplasty in female epispadias: presentation of long-term follow-up. BJU Int. 2011 Oct;108(7):1200–7. DOI: 10.1111/j.1464410X.2010.10022.x.

28. Macedo A Jr, Leal da Cruz M, Trivelato R, et al. Complete female epispadia: the case for perineal approach. J Pediatr Urol. 2015 Feb;11(1):49–50. DOI: 10.1016/j.jpurol.2014.08.013.

29. Yadav SS, Agarwal N, Kumar S, et al. Single-stage Female Epispadias Repair by Combined Infrasymphyseal Bladder Neck Plication and Urethrogenitoplasty: A Novel Technique. Urology. 2017 Feb;100:240–245. DOI: 10.1016/j.urology.2016.09.002.

30. Ferrer FA, Tadros YE, Gearhart J. Modified YoungDees-Leadbetter bladder neck reconstruction: new concepts about old ideas. Urology. 2001 Nov;58(5):791– 6. DOI: 10.1016/s0090-4295(01)01345-0.

31. Cheikhelard A, Aigrain Y, Lottmann H, LortatJacob S. Female epispadias management: perineal urethrocervicoplasty versus classical Young-Dees procedure. J Urol. 2009 Oct;182(4 Suppl):1807–11. DOI: 10.1016/j.juro.2009.04.087.

32. Agha RA, Franchi T, Sohrabi C, et al. SCARE Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines. Int J Surg. 2020 Dec;84:226–230. DOI: 10.1016/j.ijsu.2020.10.034.