The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

Alexander V. Anikiev

doi:10.14341/serg12707

The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

Alexander V. Anikiev

https://doi.org/10.14341/serg12707

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Abstract

The choice of tactics for feminizing plastic surgery (one- or two-stage) remains an actually issue. To operate early, in the first months of a child’s life, when endocrinological stabilization is achieved, or to postpone the intervention until puberty? Despite the large number of approaches to surgical treatment, still not all results can be recognized as positive. There are complications that affect the quality of life of patients, not only of a surgical but also of a psychological nature. The advantages of one or another approach are currently impossible to prove, since this will require a prospective multicenter multidisciplinary study of long-term results with each of the approaches. Therefore, pediatric surgeons for a long time in the choice of tactics will be based on accumulated experience and logical reasoning. Given the extensive experience of multidisciplinary rehabilitation, the positive long-term results of two-stage feminization remains the preferred tactic for surgical treatment of girls with CAH. The increased risk of complications from the genitourinary system in the form of infections of the urinary tract, hematocolpos and hematometra in girls with a narrow common urogenital canal, which is inherent in virilization of 3, 4 and 5 degrees according to Prader, can be considered as a reason for an early one-stage feminizing plasty.

Keywords

early feminizing plasty, congenital adrenal hyperplasia, urinary tract infection

About the Author

Alexander V. Anikiev

Endocrinology Research Centre
Russian Federation

MD, PhD, eLibrary SPIN: 9880-7682

11 Dm. Ulyanova street, 117036 Moscow, Russia

References

1. DiSandro M, Merke D, Rink R. Review of Current Surgical Techniques and Medical Management Considerations in the Treatment of Pediatric Patients with Disorders of Sex Development. Horm Metab Res. 2015;47(05):321-328. doi: https://doi.org/10.1055/s-0035-1547292

2. Creighton S, Chernausek SD, Romao R, Ransley P, Salle JP. Timing and nature of reconstructive surgery for disorders of sex development – Introduction. J Pediatr Urol. 2012;8(6):602-610. doi: https://doi.org/10.1016/j.jpurol.2012.10.001

3. Anikiev AV, Volodko ЕА, Brovin DN, et al. Differentsirovannyi podkhod k lecheniyu gipospadii u devochek. Voprosy Ginekol akušerstva i Perinatol. 2019;18(4):5-11. (In Russ.). doi: https://doi.org/10.20953/1726-1678-2019-4-5-11

4. Anikiev AV, Volodko EA, Brovin DN, et al. Complications of the genitourinary system in girls with disorders of sex development and hypospadias. Endocrine Surgery. 2019;13(2):89-94. (In Russ.). doi: https://doi.org/10.14341/serg10104

5. Nabhan ZM, Eugster EA. Upper-Tract Genitourinary Malformations in Girls With Congenital Adrenal Hyperplasia. Pediatrics. 2007;120(2):e304-e307. doi: https://doi.org/10.1542/peds.2006-2993

6. Gurkin YuA. Treatment of vulvovaginites in girls. Voprosy Ginekol akusherstva i Perinatol. 2008;7(2):92-99. (In Russ.).

7. Nefrologiya detskogo vozrasta. Ed. by Tabolin VA, Bel’mer SV, Osmanov IM. — Moscow: ID MEDPRAKTIKA-M; 2005. 712 p. (In Russ.).

8. Lesma A, Bocciardi A, Corti S, et al. Sexual Function in Adult Life Following Passerini-Glazel Feminizing Genitoplasty in Patients with Congenital Adrenal Hyperplasia. J Urol. 2014;191(1):206-211. doi: https://doi.org/10.1016/j.juro.2013.07.097

9. Creighton SM, Minto CL, Steele SJ. Objective cosmetic and anatomical outcomes at adolescence of feminising surgery for ambiguous genitalia done in childhood. Lancet. 2001;358(9276):124-125. doi: https://doi.org/10.1016/S0140-6736(01)05343-0

10. Tammar-Mattis A. Exceptions to the rule: curing the law’s failure to protect intersex infants. Berk J Gend Law Justice. 2013;21:59-110.

11. Fagerholm R, Santtila P, Miettinen P, et al. Sexual function and attitudes toward surgery after feminizing genitoplasty. J Urol. 2011;185:1900-1904. doi: https://doi.org/10.1016/j.juro.2010.12.099

12. Crouch NS, Liao LM, Woodhouse CRJ, et al. Sexual Function and Genital Sensitivity Following Feminizing Genitoplasty for Congenital Adrenal Hyperplasia. J Urol. 2008;179(2):634-638. doi: https://doi.org/10.1016/j.juro.2007.09.079

13. Stikkelbroeck NMML, Beerendonk CCM, Willemsen WNP, et al. The long term outcome of feminizing genital surgery for congenital adrenal hyperplasia: anatomical, functional and cosmetic outcomes, psychosexual development, and satisfaction in adult female patients. J Pediatr Adolesc Gynecol. 2003;16(5):289-296. doi: https://doi.org/10.1016/S1083-3188(03)00155-4

14. Krege S, Walz KH, Hauffa BP, et al. Long-term follow-up of female patients with congenital adrenal hyperplasia from 21-hydroxylase deficiency, with special emphasis on the results of vaginoplasty. BJU Int. 2000;86(3):253-258. doi: https://doi.org/10.1046/j.1464-410x.2000.00789.x

15. Binet A, Lardy H, Geslin D, et al. Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype? J Pediatr Surg. 2016;51(3):465-468. doi: https://doi.org/10.1016/j.jpedsurg.2015.10.004

16. Szymanski KM, Whittam B, Kaefer M, et al. Parental decisional regret and views about optimal timing of female genital restoration surgery in congenital adrenal hyperplasia. J Pediatr Urol. 2018;14(2):156.e1-156.e7. doi: https://doi.org/10.1016/j.jpurol.2017.11.012

17. Jesus LE. Feminizing genitoplasties: Where are we now? J Pediatr Urol. 2018;14(5):407-415. doi: https://doi.org/10.1016/j.jpurol.2018.03.020

18. Nidal S, Kocherov S, Jaber J, et al. Sexual function and voiding status following one stage feminizing genitoplasty. J Pediatr Urol. 2020;16(1):97.e1-97.e6. doi: https://doi.org/10.1016/j.jpurol.2019.11.017

19. Dangle PP, Lee A, Chaudhry R, Schneck FX. Surgical Complications Following Early Genitourinary Reconstructive Surgery for Congenital Adrenal Hyperplasia—Interim Analysis at 6 Years. Urology. 2017;101(1):111-115. doi: https://doi.org/10.1016/j.urology.2016.11.027

20. Stites J, Bernabé KJ, Galan D, et al. Urinary continence outcomes following vaginoplasty in patients with congenital adrenal hyperplasia. J Pediatr Urol. 2017;13(1):38.e1-38.e7. doi: https://doi.org/10.1016/j.jpurol.2016.10.012

21. Houben C, Tsui S, Mou J, et al. Reconstructive surgery for females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a review from the Prince of Wales Hospital. Hong Kong Med J. 2014;20:481-485. doi: https://doi.org/10.12809/hkmj144227

22. Elsayed S, Badawy H, Khater D, et al. Congenital adrenal hyperplasia: Does repair after two years of age have a worse outcome? J Pediatr Urol. 2020;16(4):424.e1-424.e6. doi: https://doi.org/10.1016/j.jpurol.2020.06.010

23. Bailez MM, Cuenca ES, Dibenedetto V. Urinary continence following repair of intermediate and high urogenital sinus (UGS) in CAH. Experience with 55 cases. Front Pediatr. 2014;2:67. doi: https://doi.org/10.3389/fped.2014.00067

24. Dobrowolska-Glazar B, Honkisz I, Sulislawski J, Tyrawa K, Wolnicki M, Chrzan R. Sexual function and health status in adult patients with Congenital Adrenal Hyperplasia. J Pediatr Urol. 2020;16(4):464.e1-464.e6. doi: https://doi.org/10.1016/j.jpurol.2020.05.162

25. Crouch NS, Creighton SM. Long-term functional outcomes of female genital reconstruction in childhood. BJU Int. 2007;100(2):403-407. doi: https://doi.org/10.1111/j.1464-410X.2007.06855.x

26. Fomina MA, Bizhanova DA, Arzamastseva LV, et al. Otsenka rezul’tatov feminiziruyushchei plastiki u devochek-podrostkov s vrozhdennoi disfunktsiei kory nadpochechnikov v otdalennom posleoperatsionnom periode. Detskaya bol’nitsa. (In Russ.).

Supplementary files

	1. Figure 1. Vocational cystourethrogram in a young girl with a salt-wasting form of congenital adrenal cortex dysfunction. Urethro-vaginal reflux is determined (when urinating, the vagina (ВЛ) is filled with a contrast agent) and a long, convoluted, male-shaped common urogenital canal (ОМК). «МП» — bladder.
	Subject
	Type	Исследовательские инструменты
	View (137KB)	Indexing metadata ▾

	2. Figure 2. Series of MRI sections of the small pelvis in a 12-year-old girl with a salt-wasting form of congenital adrenal cortex dysfunction.
	Subject
	Type	Исследовательские инструменты
	View (208KB)	Indexing metadata ▾

	Title	Figure 2. Series of MRI sections of the small pelvis in a 12-year-old girl with a salt-wasting form of congenital adrenal cortex dysfunction.
	Type	Исследовательские инструменты
Brief description	The diagnosis was made at birth on the basis of a high 17-hydroxyprogesterone level, female karyotype, and virilization of the external genital organs of the 5th degree. At an early age, plastic surgery of the hypertrophied clitoris and sinusotomy were performed. At the age of 12, in connection with gross hematuria, signs of hematocolpos (blue arrow) and hematometers (yellow arrow), confirmed by MRI, were revealed. Thanks to the prompt actions of endocrinologists at the place of residence and parents, the girl was promptly hospitalized and operated on at the Institute of Endocrinology. Due to the high form of the urethro-vaginal fistula and insufficient estrogenization and therefore the high risk of complications, it was decided to abandon uncoupling introitoplasty in favor of sinusovaginotomy. A clinical example demonstrates the ineffectiveness of a sinusotomy performed in the pre-pubertal period - despite the dissection of the common urogenital canal, a complication from the genital tract in the form of hematocolpos and hematomas was obtained, the cause of which was a narrow entrance to the vagina.
	Date	2021-10-06
Digital Object Identifier	https://doi.org/10.14341/serg12707-5910

Review

For citations:

Anikiev A.V. The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya. Endocrine Surgery. 2020;14(4):30-35. (In Russ.) https://doi.org/10.14341/serg12707

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND 4.0).

ISSN 2306-3513 (Print)
ISSN 2310-3965 (Online)

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	Title	Figure 1. Vocational cystourethrogram in a young girl with a salt-wasting form of congenital adrenal cortex dysfunction. Urethro-vaginal reflux is determined (when urinating, the vagina (ВЛ) is filled with a contrast agent) and a long, convoluted, male-shaped common urogenital canal (ОМК). «МП» — bladder.
	Type	Исследовательские инструменты
	Date	2021-10-06
Digital Object Identifier	https://doi.org/10.14341/serg12707-5908

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Endocrine Surgery

The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

Full Text:

Abstract

Keywords

About the Author

References

Supplementary files

Review

For citations:

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