선천성 뮬러관형성부전증은 원발성 무월경의 흔한 원인이며 그 빈도는 4,000-5,000명 여아 출생 당 1명 정도로 보고된다. 환자들은 질 형성 부전이거나 맹관 형태의 질을 가지며 자궁과 나팔관은 보통 없다. 그러나 드물게 정상적 자궁을 가지고 있지만 관상구조가 아니거나 흔적기관만 존재하기도 한다. 남성 가성 반음양과 비슷하지만 정상적인 여성 핵형을 가진다. 난소는 뮬러관 구조가 아니기 때문에, 난소의 기능은 정상이며 이차 성징 및 발달은 정상이다. 치료는 신생질의 형성으로 성교가 가능하게 하는 것이었으나, 1985년 대리모 임신의 성공으로 생리학적으로 부모가 되는 것도 가능해졌다. 정상 난소 기능을 가진 환자일 경우 기존의 체외수정시술시 사용하는 성선자극호르몬 유리호르몬 효능제 장기요법으로 과배란유도를 시도하여 체외수정술후 대리모에게 이식하여 임신을 시도할 수 있다. 저자들은 30세 뮬러관 형성부전증으로 질형성술을 시행하지 않은 환자에 있어서 대리모를 통한 성공적인 쌍태임신과 생존아의 분만을 경험하여 문헌 고찰과 함께 보고하고자 한다.

Lack of müllerian development (Mayer-Rokitansky-Kuster-Hauser Syndrome) is characterized by absence of apparent vagina and/or uterus, normal secondary sexual characteristics, normal reproductive hormonal profile, and a relatively common cause of primary amenorrhea about 1 in 4,000 female births and also cause of primary infertility. Management for these women comprise of construction of neovagina for sexual life. In 1985, the first report of a successful pregnancy through the uterine surrogacy was made. It is being possible for these women to have new opportunity of getting her own genetic offspring. Since ovarian activity is completely preserved in patients, controlled ovarian hyperstimulation is similar to any other IVF case that is with urinary or recombinant gonadotropins following GnRH agonist down regulation. Genetic offspring can be achieved by cellection of oocytes from the genetic mather, in-vitro-fertilization by the genetic father, and placement into a surrogate carrier. We have experienced a case of successful surrogate pregnancy in a patient with congenital absence of vagina and uterus.

Lack of müllerian development (Mayer-Rokitansky-Kuster-Hauser Syndrome) is characterized by absence of apparent vagina and/or uterus, normal secondary sexual characteristics, normal reproductive hormonal profile, and a relatively common cause of primary amenorrhea about 1 in 4,000 female births and also cause of primary infertility. Management for these women comprise of construction of neovagina for sexual life. In 1985, the first report of a successful pregnancy through the uterine surrogacy was made. It is being possible for these women to have new opportunity of getting her own genetic offspring. Since ovarian activity is completely preserved in patients, controlled ovarian hyperstimulation is similar to any other IVF case that is with urinary or recombinant gonadotropins following GnRH agonist down regulation. Genetic offspring can be achieved by cellection of oocytes from the genetic mather, in-vitro-fertilization by the genetic father, and placement into a surrogate carrier. We have experienced a case of successful surrogate pregnancy in a patient with congenital absence of vagina and uterus.

Mayer-Rokitansky-Kuster-Hauser Syndrome, Surrogate pregnancy