Mayer-Rokitansky-Kuster-Hauser Syndrome and related clinical events

Syggelos Nikolaos, Eleftheriadis Makarios, Panoulis Kostantinos, Sertedaki Eleni*, Salloum Ioannis*, Deligeoroglou Efthymios, Creatsas Georgios


Mayer-Rokitansky-Kuster-Hauser Syndrome (MRKH) is due to congenital hypoplasia of tissues originating from Muller’s pores, including the upper part of the vagina, uterus and fallopian tubes. It is associated with syndromes, such as VACTERL syndrome and TAR syndrome, with chromosomal abnormalities, such as Turner syndrome and Silver-Russell syndrome, with clinical manifestations, such as endometriosis, uterine fibroids and adenomyosis, with urologic disorders, such as stress urinary incontinence, malignancies, such as botryoid sarcoma, but also endocrine disorders, such as PCOS and hypergonadotrophic hypogonadism. Uterine transplantation is a promising future surgery to maintain the reproductive capacity of women with the syndrome. This study is a review of the literature over the last decade aimed at describing MRKH syndrome in combination with a set of gynecological conditions that can coexist or be associated with it through the presentation of clinical events.

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