A Case of Mayer-Rokitansky-K ster-Hauser(MRKH) Syndrome with Imperforate Anus and Unilateral Renal Agenesis

The clinical features of the Mayer-Rokitansky-K ster-Hauser(MRKH) syndrome include normal female secondary sex characteristics, normal genitalia, congenital absence of vagina, rudimentary or bipartite uterus, normal ovarian function and normal ovulation, 46, XX, karyotype, frequent association of renal, skeletal and other congenital anomalies. We experienced a case of a premature infant with MRKH syndrome who had imperforate anus and unilateral renal agenesis.

A Successful Uterine Surrogate Pregnancy Via Oocyte Retrieval Through the Neovagina in a Patient with Mayer-Rokitansky-K ster-Hauser Syndrome / 대한산부인과학회잡지

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.

A Case of Mayer-Rokitansky-K ster-Hauser Syndrome combined with Unilateral Renal Agenesis and Pelvic Cystic Mass in Child / 대한비뇨기과학회지

The Mayer-Rokitansky-K ster-Hauser syndrome represents a spectrum of m llerian anomalies, including vaginal agenesis with or without renal anomalies, in genotypically and phenotypically normal female subjects with normal endocrine status. We experienced a case of this anomaly which combined with unilateral renal agenesis and pelvic cystic mass in child.