TY - JOUR
T1 - Primary Amenorrhea, Aplastic Uterus, and a Functional Vagina
T2 - A Rare Case of Mayer-Rokitansky-Küster-Hauser Syndrome
AU - Kirschen, Gregory W.
AU - Semenyuk, Natalie
N1 - Publisher Copyright:
© 2019 Gregory W. Kirschen and Natalie Semenyuk.
PY - 2019
Y1 - 2019
N2 - Background. Müllerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), a failure of female urogenital development, typically results in a completely stenotic or rudimentary dimple vagina, both of which are generally nonfunctional in adulthood without mechanical dilation or surgical reconstruction. Case. A 20-year-old Tanner stage V heterosexual woman with normal sexual function since coitarche presented with a chief complaint of primary amenorrhea. She was found to have aplastic uterine buds, absent endometrium/cervix, normal ovaries, and an unusually well-developed lower vagina, a rare presentation of MRKHS. We discuss mechanisms by which the anomaly may have arisen. Summary & Conclusion. This case thus expands the clinical presentation of MRKHS to include a normal appearing vagina with intact sexual function from first sexual encounter, raising interesting questions about the basic underlying embryology.
AB - Background. Müllerian agenesis, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), a failure of female urogenital development, typically results in a completely stenotic or rudimentary dimple vagina, both of which are generally nonfunctional in adulthood without mechanical dilation or surgical reconstruction. Case. A 20-year-old Tanner stage V heterosexual woman with normal sexual function since coitarche presented with a chief complaint of primary amenorrhea. She was found to have aplastic uterine buds, absent endometrium/cervix, normal ovaries, and an unusually well-developed lower vagina, a rare presentation of MRKHS. We discuss mechanisms by which the anomaly may have arisen. Summary & Conclusion. This case thus expands the clinical presentation of MRKHS to include a normal appearing vagina with intact sexual function from first sexual encounter, raising interesting questions about the basic underlying embryology.
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U2 - 10.1155/2019/3409548
DO - 10.1155/2019/3409548
M3 - Article
AN - SCOPUS:85083331537
SN - 2090-6684
VL - 2019
JO - Case Reports in Obstetrics and Gynecology
JF - Case Reports in Obstetrics and Gynecology
M1 - 3409548
ER -