Methods for neovagina creation in women with Mayer-Rokitansky-Küster-Hauser syndrome for subsequent uterus transplantation

Roman Chmel,Marta Novackova,Nikoleta Chubanovova,Zlatko Pastor

doi:10.5507/bp.2021.049

Roman Chmel, Marta Novackova

Open Access

https://doi.org/10.5507/bp.2021.049

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Abstract

Mayer-Rokitansky-Küster-Hauser syndrome is a rare female congenital anomaly that presents with an inability to have coital sexual intercourse and absolute uterine factor infertility. Both surgical and nonsurgical approaches have been described for the treatment of vaginal agenesis to allow satisfactory coitus. Transplantation of the uterus has the challenge of achieving pregnancy and delivery of her own genetic and biological children in a woman without a natural uterus. Women of reproductive age with a congenital form of absolute uterine factor infertility are considered appropriate recipients of a uterus in the experimental phase of uterus transplantation trials. A neovagina in the normal anatomic position covered by natural non-keratinized mucosa is one of the main assumptions for surgical and reproductive success in transplant recipients. More than 70 uterine transplants have been performed to date, and more than 25 childbirths have been achieved by several research centers in the recipients of a uterus with uterine agenesis. In women with Mayer-Rokitansky-Küster-Hauser syndrome, skin-graft neovagina, Vecchietti's vaginoplasty, and self-dilation using Frank's and Ingram's methods are appropriate techniques to create a neovagina if transplantation of the uterus is intended in the future.

Highlights

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly characterized by agenesis of the uterus and upper two-thirds of the vagina in women with a normal female karyotype
This study is based on literature review of nonsurgical and surgical methods for neovagina creation with respect to subsequent uterus transplantation using the Web of Science, Google Scholar and Medline databases with keywords: absolute uterine infertility factor, Mayer-Rokitansky-Küster-Hauser syndrome, neovagina, uterus transplantation
In MRKHS women with only partial vaginal agenesis, attempts at sexual intercourse affects the formation of vagina in terms of length and width

Summary

Introduction

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is a rare congenital anomaly characterized by agenesis of the uterus and upper two-thirds of the vagina in women with a normal female karyotype. As the ovaries undergo normal development, women with MRKHS have normal female secondary sexual characteristics. The typical form (type I) is associated with a symmetrical developmental disorder of the caudal parts of the Müllerian ducts, which means that both the uterus and upper part of the vagina are absent and rudimentary uterine horns are often present, while other organ anomalies are not associated. Malfunctions in the other organs occur in the atypical form (type II) of MRKHS, in the renal, skeletal, and cardiac systems. The most severe form of type II is represented by Müllerian Renal Cervical Somite (MURCS) syndrome[2,3]

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