Conversations with your Reproductive Endocrinologist: I have MRKH–do I need more gonadotropins?

As we approach Rare Disease Day, the MRKH Community is banding together to raise awareness of a condition that affects 1 in 4500 females. Mayer-Rokitansky-Küster-Hauser Syndrome represents a spectrum of phenotypes centered around the underdevelopment of the Müllerian-derived tissues of the female reproductive tract (Medicine 2015). This includes the uterus, cervix and upper portion of the vaginal canal. Most have functional ovaries, but anectdotal data from friends undergoing IVF + gestational surrogacy indicate that ovarian reserve may be lower in a subpopulation of those with MRKH. This is supported by two studies. The first shows that those with MRKH Type I (those who only have atypical development of the reproductive tract) achieve ovarian hyperstimulation using less gonadotropins and produce higher numbers of follicles, oocytes and embryos than those with MRKH Type II (those who have MRKH Type I plus kidney, skeletal, heart and/or other symptoms) (Raziel et al. 2012). Although the second has a few flaws (e.g. the number of patients with MRKH Type II is low, and the patients with MRKH Type I are not age-matched, making the findings more consistent with pilot data), it reveals that those with MRKH Type II have decreased ovarian reserve compared to age-matched controls (Ozekinci et al. 2015).

Although the ovarian reserve may be lower, Raziel and colleagues (Raziel et al. 2012) find no differences in pregnancy rates because the number of good-quality embryos were similar in those with MRKH Type I and MRKH Type II. This presents good and not so good news–those with MRKH Type II may need to undergo additional rounds of hormonal treatment.

Medicine, NLo (2015). Genetics Home Reference [Internet]. Bethesda (MD), National Library of Medicine (US). http://ghr.nlm.nih.gov/condition/mayer-rokitansky-kuster-hauser-syndrome.

Ozekinci, M, Erman Akar, M, Senol, Y, Ozdem, S, Uzun, G, Daloglu, A and Ongut, G (2015). Fertil Steril 104(1): 176-9.

Raziel, A, Friedler, S, Gidoni, Y, Ben Ami, I, Strassburger, D and Ron-El, R (2012). Hum Reprod 27(1): 126-30.

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