ABSTRACT
The combination of Müllerian agenesis with inguinal ovaries accompanied by primary ovarian insufficiency is extremely rare. A 21-year-old Korean woman was referred to our center with primary amenorrhea. The patient was diagnosed with Müllerian agenesis with inguinal ovaries. Her hormonal profile showed hypergonadotrophic hypogonadism suggesting primary ovarian insufficiency. We performed laparoscopic neovaginoplasty using modified Davydov's procedure and reposition inguinal ovaries in the pelvic cavity. Oral estrogen replacement was applied for the treatment of primary ovarian insufficiency. This is a rare case report on Mayer-Rokitansky-Kuster-Hauser syndrome accompanied not only by inguinal ovaries but also with primary ovarian insufficiency. We present our first experience on the laparoscopic neovaginoplasty performed on the patient with müllerian agenesis accompanied by inguinal ovaries and primary ovarian insufficiency.
Subject(s)
Female , Humans , Young Adult , Amenorrhea , Estrogen Replacement Therapy , Hypogonadism , Laparoscopy , Ovary , Peritoneum , Primary Ovarian InsufficiencyABSTRACT
Congenital absence of vagina (Mayer-Rokitansky-K ster-Hauser Syndrome) is the second most common etiology of primary amenorrhea and also cause of primary infertility. Management for these women comprise of construction of neovagina for sexual life and screening for associated congenital anomalies and planning of getting her own genetic offspring. As associated assisted reproductive technologies involving in vitro fertilization and embryos transfer to surrogate mother become realizing, it is being possible for these women to have new opportunity of getting her own genetic baby. In most cases, oocyte retrieval for uterine surrogate program have been performed laparoscopically because of difficulty of oocyte retrieval via neovagina. But we have experienced a case of successful surrogate pregnancy via oocyte retrieval through the neovagina in a patient with congenital absence of vagina, so report it after following observation of the babies for 3 years.