Genital masculinizing reconstruction for untreated intersex individuals in adulthood: a case report.

Clement Parat, Aurelie Schirmann, Nicolas Morel-Journel, Laetitia Martinerie, Thomas Blanc, Etienne Suply, Paul Neuville, Fran��ois-Xavier Madec
Author Information
  1. Clement Parat: Department of Urology, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France. ORCID
  2. Aurelie Schirmann: Department of Urology, Foch Hospital, Paris, France. ORCID
  3. Nicolas Morel-Journel: Department of Urology, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France.
  4. Laetitia Martinerie: Pediatric Endocrinology Department, CHU Robert Debr��, Assistance Publique-H��pitaux de Paris, Paris, France. ORCID
  5. Thomas Blanc: Universit�� Paris Cit��, Facult�� de Sant��, UFR de M��decine, Paris, France. ORCID
  6. Etienne Suply: Department of Pediatric Surgery, CHU de La R��union (F��lix Guyon), La R��union, France. ORCID
  7. Paul Neuville: Department of Urology, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France. ORCID
  8. Fran��ois-Xavier Madec: Department of Urology, Foch Hospital, Paris, France. ORCID

Abstract

Background: Partial androgen insensitivity syndrome (PAIS) is a rare cause of variations in sexual and genital development characterized by severe hypospadias, micropenis, and a bifid scrotum. Sex assignment and determination of gender are significant challenges both from societal and surgical considerations. This case report describes the surgical management of masculinizing genital reconstruction without urethroplasty in an adult patient with PAIS, demonstrating that achieving penile straightening and lengthening is possible as an initial surgery in adulthood.
Case Description: A 30-year-old patient born with PAIS exhibited genitalia variation with severe perineoscrotal hypospadias and a microphallus. Assigned female at birth, he developed gender dysphoria in adolescence and was referred to our institution for surgery in adulthood. The surgery consisted of cavernoplasty with a double-Y albugineal incision covered by a patch using Surgisis and metoidioplasty implants to correct the penile curvature. The urethral plate was transected, a penoscrotal urethrostomy was performed and a scrotal flap was harvested. The patient reported a high level of satisfaction with sexual function.
Conclusions: This case demonstrates that it is possible to delay genital reconstruction surgery and raises the question of the societal acceptability of the intersex situation during childhood. Following to its European neighbors, France has recently legislated on the management of these patients, promoting self-determination before any gender-affirming surgery.

Keywords

References

  1. Urology. 2023 Jun;176:206-212 [PMID: 37004847]
  2. Int J Transgend Health. 2022 Sep 6;23(Suppl 1):S1-S259 [PMID: 36238954]
  3. J Sex Marital Ther. 2002 May-Jun;28(3):269-74 [PMID: 11995605]
  4. Soc Biol. 2006 Spring-Summer;53(1-2):4-12 [PMID: 21516946]
  5. Semin Pediatr Surg. 2020 Jun;29(3):150929 [PMID: 32571514]
  6. J Biol Chem. 2000 Aug 25;275(34):26164-71 [PMID: 10840043]
  7. Med Law Rev. 2019 Aug 1;27(3):482-508 [PMID: 30544143]
  8. J Endocrinol Invest. 2016 Nov;39(11):1207-1224 [PMID: 27287420]
  9. Arch Pediatr. 2023 Jan;30(1):10-13 [PMID: 36462990]
  10. J Clin Res Pediatr Endocrinol. 2016 Jun 5;8(2):232-5 [PMID: 27087292]
  11. J Neonatal Surg. 2017 Aug 10;6(3):58 [PMID: 28920018]
  12. J Surg Case Rep. 2023 Dec 06;2023(12):rjad652 [PMID: 38076312]
  13. J Pediatr Urol. 2016 Jun;12(3):139-49 [PMID: 27132944]
  14. J Pediatr Urol. 2021 Feb;17(1):39-47 [PMID: 33246831]
  15. Horm Behav. 2016 Nov;86:8-20 [PMID: 27576114]

Word Cloud

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