Feminizing genitoplasty in childhood: aiming for achievable outcomes
Tóm tắt
Early genital reconstruction may be recommended in cases of congenital adrenal hyperplasia to avoid the stigma that can affect these individuals with variable degrees through different cultures. However, the separation and mobilization of a high vagina has remained challenging with less satisfactory surgical outcomes. Therefore, the value of early vaginal reconstruction has been questioned in favour of delaying the whole repair after puberty. In this report, the author has adopted the third option in-between, which comprises early reconstruction of the external genitalia and delaying the more challenging vaginal reconstruction (if needed) to be performed after puberty. The study included ten consecutive cases of CAH who underwent feminizing genitoplasty during the period 2016 through 2019. Their age at operation ranged from 8 to 84 months (mean 31; median 15). In five cases (50%), the technique of limited urogenital sinus mobilization adopted in this report succeeded in bringing the vaginal introitus down to the perineum. Those cases had originally a low vagina. In the rest of cases, labial retraction showed a common but wide urogenital introitus perfectly lined by urogenital mucosa. The outcomes have been considered satisfactory to a great extent for both parents and doctors regarding cosmesis and lack of functional complications (voiding problems). Longer follow up is still needed to assess the sexual function when these girls become sexually active. In cases of congenital adrenal hyperplasia, reduction clitoroplasty combined with partial urogenital sinus mobilization can achieve predictable and satisfactory outcomes. In about 50% of cases, this approach is sufficient to bring the vagina down to the perineum. In the other half of cases with higher vagina, the possibility of satisfactory sinus intercourse can be studied when these girls grow and become sexually active; otherwise, a delayed vaginal reconstruction may turn to be necessary.
Tài liệu tham khảo
Lottman H, Thomas D. Disorders of sexual development. In: Thomas D, Duffy PG, Rickwood A, editors. Essentials of paediatric urology. London: Informa; 2008. p. 275–93.
Ganesan A, Smith GHH, Broome K, Steinberg A. Congenital adrenal hyperplasia: preliminary observations of the urethra in 9 cases. J Urol. 2002;167:275–9.
Witchel SF. Congenital adrenal hyperplasia. J Pediatr Adolesc Gynecol. 2017;30:520–34.
Mouriquand PDE, Gorduza DB, Gay CL, et al. Surgery in disorders of sex development (DSD) with a gender issue: if (why), when and how? J Pediatr Urol. 2016;12:139–49.
AbouZeid AA, Mohammad SA. Transformation of the female genitalia in congenital adrenal hyperplasia: MRI study. J Pediatr Surg. 2020;55:977–84.
AbouZeid AA, Mohammad SA. The 'Bifid bulb' sign to differentiate between high and low vagina in congenital adrenal hyperplasia. https://doi.org/10.26044/ecr2020/C-05754.
Hendren WH, Crawford JD. Adrenogenital syndrome: the anatomy of the anomaly and its repair. Some new concepts. J Pediatr Surg. 1969;4:49.
Passerini-Glazel G. A new 1-stage procedure for clitoroplasty in severely masculinized female pseudohermaphrodites. J Urol. 1989;142:565 e8.
Ludwikowski B, Oesch Hayward I, Gonzalez R. Total urogenital sinus mobilization: expanded applications. BJU Int. 1999;83:820 e2.
Hamza AF, Soliman HA, Hay SA, et al. Total urogenital sinus mobilization in the repair of cloacal anomalies and congenital adrenal hyperplasia. J Pediatr Surg. 2001;36:1656–8.
Dòmini R, Rossi F, Ceccarelli PL, et al. Anterior sagittal transanorectal approach to the urogenital sinus in adrenogenital syndrome: preliminary report. J Pediatr Surg. 1997;32:714–6.
Rink RC, Metcalfe PD, Kaefer MA, Casale AJ, Meldrum KK, Cain MP. Partial urogenital mobilization: a limited proximal dissection. J Pediatr Urol. 2006;2:351–6.
Lee P, Schober J, Nordenstrom A, et al. Review of recent outcome data of disorders of sex development (DSD): emphasis on surgical and sexual outcomes. J Pediatr Urol. 2012;8:611–5..
Pena A, Bischoff A: History of the treatment of anorectal malformations. In: Surgical treatment of Colorectal problems in children. Springer 2015; pp: 1-16.
El-Agwany AS. Asymptomatic uretheral intercourse associated with Mayer Rokitansky Küster Hauser syndrome type II: a case with crossed fused renal ectopia and scoliosis. African J Urol. 2017;23:48–51.
Rumley-Dawson E. A case of complete transverse septum of the vagina impeding delivery; urethral coitus. Lancet. 1900;156(4032):1646–7.
Ryckman J, Black A, Fleming N. Adolescent urethral coitus: 2 cases and review of the literature. J Pediatr Adolesc Gynecol. 2014;27:e9–e12.
Puppo V. Embryology and anatomy of the vulva: the female orgasm and women’s sexual health. European Journal of Obstetrics & Gynecology and Reproductive Biology. 2011;154:3–8.
Crouch NS, Liao LM, Woodhouse CR, Conway GS, Creighton SM. Sexual function and genital sensitivity following feminizing genitoplasty for congenital adrenal hyperplasia. J Urol. 2008;179(2):634–8.
Oshiba A, Ashour K, Khairi A, et al. Evaluation of the early outcomes of partial urogenital sinus mobilization in females with congenital adrenal hyperplasia. Ann Pediatr Surg. 2020;16:13 https://doi.org/10.1186/s43159-020-00024-0.