Mayer rokitansky kuster hauser syndrome with hypothyroidism with benign serous cyst adenoma of ovary

Mayer Rokitansky Kuster Hauser Syndrome is a congenital aplasia of uterus, bilateral fallopian tubes and upper 2/3 of vagina; with normal secondary sexual characters and a normal 46XX karyotype. It affects 1/4500 women and its first sign is primary amenorrhoea in young women. A 16 yr old girl presented with primary amenorrhoea with lower abdominal pain. There was blind vaginal pouch and secondary sexual characters normally present. On endocrinological profile patient was hypothyroid.MRI report showed MRKH Syndrome with 8X12cm left ovarian hemorrhagic dysgerminoma. Laparotomy was done where we found left ovarian cystic mass (around 8x12cm) filled with hemorrhagic fluid with intact capsule. Left sided salpingo oophorectomy done. Right side ovary and fallopian tube were normal. In pelvis there were two uterinovaginal bulbs connected by a band like uterus, with agenesis of upper vagina. On Karyotyping 46XX chromosomal pattern was found.