Neovagina in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Vaginoplasty Using Ileal Flap

Authors

  • Sílvia Serrano Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Centro Hospitalar Universitário Lisboa Norte. Lisbon. https://orcid.org/0000-0002-0544-2698
  • Inês Pereira Department of Gynecology and Obstetrics. Hospital CUF Descobertas. Lisbon; Department of Gynecology and Obstetrics. Hospital CUF Torres Vedras. Torres Vedras.
  • Alexandra Henriques Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Centro Hospitalar Universitário Lisboa Norte. Lisbon; Lisbon School of Medicine. Lisbon Academical Medical Center. Lisbon.
  • Alexandre Valentim Lourenço Department of Obstetrics, Gynecology and Reproductive Medicine. Hospital de Santa Maria. Centro Hospitalar Universitário Lisboa Norte. Lisbon; Lisbon School of Medicine. Lisbon Academical Medical Center. Lisbon.

DOI:

https://doi.org/10.20344/amp.18563

Keywords:

46, XX Disorders of Sex Development/surgery, Reconstructive Surgical Procedures, Vagina/surgery

Abstract

Introduction: Surgical treatment of patients with malformations of the female genital tract is a complex problem and there are different techniques described in the literature. The goal of all these techniques is the reconstruction of a neovagina that is anatomically similar to a vagina, with adequate length to facilitate sexual functioning and with the lowest risk of possible complications. The aim of this study is to describe the surgical technique for the reconstruction of a neovagina from an ileal segment without a vascular pedicle.
Material and Methods: Description of a surgical technique developed in our tertiary university center in a patient with Mayer-Rokitansky-Küster-Hauser syndrome.
Results: The vaginoplasty surgery using ileal flap was performed in three steps. In the first part of the intervention a laparoscopic hysterectomy with bilateral salpingectomy was performed. The second step consisted of isolating the ileal segment, ileal anastomosis and preparing the ileal segment. After the isolated segment was prepared, it was repositioned in a vagina mold to configure the neovagina. Finally, the third step included the adaptation of the vaginal mold with the ileal mucosa to the vesicorectal space.
Conclusion: Ileal vaginoplasty without a vascular pedicle is an option that can be used to reconstruct the vagina, because it provides an excellent tissue for vaginal replacement. This technique can be used in patients with genital malformations of the genital tract with absence or vaginal hypoplasia.

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References

Fontana L, Gentilin B, Fedele L, Gervasini C, Miozzo M. Genetics of Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome. Clin Genet. 2017;91:233-46. DOI: https://doi.org/10.1111/cge.12883

Morcel K, Camborieux L, Programme de recherches sur les aplasies Mullériennes, Guerrier D. Mayer-Rokitansky-Kuster- Hauser (MRKH) syndrome. Orphanet J Rare Dis. 2007;2:1-13. DOI: https://doi.org/10.1186/1750-1172-2-13

Callens N, de Cuypere G, de Sutter P, Monstrey S, Weyers S, Hoebeke P, et al. An update on surgical and non-surgical treatments for vaginal hypoplasia. Hum Reprod Update. 2014;20:775-801. DOI: https://doi.org/10.1093/humupd/dmu024

Sultan C, Biason-Lauber A, Philibert P. Mayer–Rokitansky–Kuster–Hauser syndrome: recent clinical and genetic findings. Gynecol Endocrinol. 2009;25:8-11. DOI: https://doi.org/10.1080/09513590802288291

Weijenborg PT, TerKuile MM. The effect of a group programme on women with the Mayer-Rokitansky-Kuster-Hauser syndrome. BJOG. 2000;107:365-8. DOI: https://doi.org/10.1111/j.1471-0528.2000.tb13232.x

Pizzo A, Lagana AS, Sturlese E, Retto G, Retto A, Dominici R, et al. Mayer-Rokitansky-Kuster-Hauser syndrome: embryology, genetics and clinical and surgical treatment. ISRN Obstet Gynecol. 2013;2013:628717. DOI: https://doi.org/10.1155/2013/628717

Marin JS, Lara LA, Silva AV, Reis RM, Junqueira FR, Rosa-e-Silva AC. Surgical and non-surgical treatment of vaginal agenesis: analysis of a series of cases. Rev Bras Ginecol Obstet. 2012;34:274–7.

Maniglio P, Ricciardi E, Lagana AS, Triolo O, Caserta D. Epigenetic modifications of primordial reproductive tract: a common etiologic pathway for Mayer-Rokitansky-Kuster-Hauser syndrome and endometriosis? Med Hypotheses. 2016;90:4-5. DOI: https://doi.org/10.1016/j.mehy.2016.02.015

McIndoe A. The treatment of congenital absence and obliterative conditions of the vagina. Br J Plast Surg. 1950;2:254-67.

Baldwin JF. Formation of an artificial vagina by intestinal transplantation. Ann Surg. 1904;40:398-403. DOI: https://doi.org/10.1097/00000658-190440030-00014

Van der Sluis WB, Pavan N, Liguori G, Bucci S, Bizic MR, Kojovic V, et al. Ileal vaginoplasty as vaginal reconstruction in transgender women and patients with disorders of sex development: an international, multicentre, retrospective study on surgical characteristics and outcomes. BJU Int. 2018;121:952-8. DOI: https://doi.org/10.1111/bju.14155

Nakhal RS, Creighton SM. Management of vaginal agenesis. J Pediatr Adolesc Gynecol. 2012;25:352-7. DOI: https://doi.org/10.1016/j.jpag.2011.06.003

Brannstrom M, Johannesson L, Bokstrom H, Kvarnstrom N, Molne J, Dahm-Kahler P, et al. Live birth after uterus transplantation. Lancet. 2015;385:607-16. DOI: https://doi.org/10.1016/S0140-6736(15)61098-4

Bean EJ, Mazur T, Robinson AD. Mayer–Rokitansky–Kuster–Hauser syndrome: sexuality, psychological effects, and quality of life. J Pediatr Adolesc Gynecol. 2008;22:339-46. DOI: https://doi.org/10.1016/j.jpag.2008.11.006

ACOG Committee on Adolescent Health Care. ACOG committee opinion no. 355: vaginal agenesis: diagnosis, management, and routine care. Obstet Gynecol. 2006;108:1605-9. DOI: https://doi.org/10.1097/00006250-200612000-00059

Edmonds DK, Rose GL, Lipton MG, Quek J. Mayer-Rokitansky-Kuster-Hauser syndrome: a review of 245 consecutive cases managed by a multidisciplinary approach with vaginal dilators. Fertil Steril. 2012;97:686-90. DOI: https://doi.org/10.1016/j.fertnstert.2011.12.038

Liguori G, Trombetta C, Bucci S, Salamè L, Bortul M, Siracusano S, et al. Laparoscopic mobilization of neovagina to assist secondary ileal vaginoplasty in male-to-female transsexuals. Urology. 2005;66:293-8. DOI: https://doi.org/10.1016/j.urology.2005.03.091

Van der Sluis WB, Bouman MB, Buncamper ME, Mullender MG, Meijerink WJ. Revision vaginoplasty: a comparison of surgical outcomes of laparoscopic intestinal versus perineal full-thickness skin graft vaginoplasty. Plast Reconstr Surg. 2016;138:793-800. DOI: https://doi.org/10.1097/PRS.0000000000002598

Burger RA, Riedmiller H, Knapstein PG, Hohenfellner VF. Ileocecal vaginal construction. Am J Obstet Gynecol. 1989;161:162-7. DOI: https://doi.org/10.1016/0002-9378(89)90256-1

Hensle TW, Chang DT. Vaginal reconstruction. Urol Clin North Am. 1999;26:39-47.

Trombetta C, Liguori G, Siracusano S, Bortul M, Bergrano E. Retubularized ileal vaginoplasty: a new application of the monti principl preliminary report. Eur Urol. 2005;48:1018–24. DOI: https://doi.org/10.1016/j.eururo.2005.05.006

Hensle TW, Gjertson CK, Reiley EA. Vaginal reconstruction. Urol Clin North Am. 1999;26:39-47. DOI: https://doi.org/10.1016/S0094-0143(99)80005-6

Kwun Kim S, Hoon Park J, Cheol Lee K, Min Park J, Tae Kim J, Chan Kim M. Longterm results in patients after rectosigmoid vaginoplasty. Plast Reconstr Surg. 2003;112:143-51. DOI: https://doi.org/10.1097/01.PRS.0000066169.78208.D4

Abbasakoor F, Mahon C, Boulos PB. Diversion colitis in sigmoid neovagina. Colorectal Dis. 2004;6:290-1. DOI: https://doi.org/10.1111/j.1463-1318.2004.00653.x

Hiroi H, Yasugi T, Matsumoto K, Fujii T, Watanabe T, Yoshikawa H, et al. Mucinous adenocarcinoma arising in a neovagina using the sigmoid colon thirty years after operation: a case report. J Surg Oncol. 2001;77:61-4. DOI: https://doi.org/10.1002/jso.1067

Ota H, Tanaka JI, Murakami M, Murata M, Fukuda J, Tanaka T, et al. Laparoscopy-assisted Ruge procedure for the creation of a neovagina in a patient with Mayer-Rokytansky-Kuster-Hauser syndrome. Fertil Steril. 2000;73:641-4. DOI: https://doi.org/10.1016/S0015-0282(99)00570-1

Mcpherson TB, Badylak SF. Characterization of fibronectin derived from porcine small intestinal submucosa. Tissue Eng. 1998;4:75-83. DOI: https://doi.org/10.1089/ten.1998.4.75

Allman AJ, McPherson TB, Timothy B, Badylak SF, Merril LC, Kallakury B, et al. Xenogeneic extracellular matrix grafts elicit a th-2-restricted immune response. Transplantation. 2001;71:1631-40. DOI: https://doi.org/10.1097/00007890-200106150-00024

Sarikaya A, Record RD, Badylak SF, Tullius B, Badylak S, Ladisch M. Antimicrobial activity associated with extracellular matrices. Tissue Eng. 2002;8:63-71. DOI: https://doi.org/10.1089/107632702753503063

Badylak S, Liang A, Record R, Tullius R, Hodde J. Endothelial cell adherence to small intestinal submucosa: an acellular bioscaffold. Biomaterials.1999;20:2257-63. DOI: https://doi.org/10.1016/S0142-9612(99)00156-8

Hodde JP, Record RD, Liang HA, Badylak SF. Vascular endothelial growth factor in porcine-derived extracellular matrix endothelium. 2001;8:11-24. DOI: https://doi.org/10.3109/10623320109063154

Filipas D, Black P, Hohenfellner R. The use of isolated caecal bowel segment in complicated vaginal reconstruction. BJU Int. 2000;85:715-9. DOI: https://doi.org/10.1046/j.1464-410x.2000.00482.x

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Published

2023-01-04

How to Cite

1.
Serrano S, Pereira I, Henriques A, Valentim Lourenço A. Neovagina in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: Vaginoplasty Using Ileal Flap. Acta Med Port [Internet]. 2023 Jan. 4 [cited 2024 May 28];36(6):408-15. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/18563

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