Young females come in with primary amenorrhea with normal breast, normal pubic hair. USG reveals absent uterus and cervix with short vaginal pouch and normal ovaries. Diagnosis:

Ans. B. Mullerian agenesisMULLERIAN AGENESIS* Congenital absence of both uterus and vagina is referred as mullerian aplasia, mullerian agenesis, or Mayer- Rokitansky- Kuster-Hauser syndrome.* In classic mullerian agenesis, patients have a shallow vaginal pouch, only measuring up to 1.5 inches deep. In addition, the uterus, cervix, and upper part of the vagina are absent.* Typically, a portion of the distal fallopian tubes are present. In addition, normal ovaries are present, given their separate embryonic origin.* Most patients with mullerian agenesis have only small rudimentary mullerian bulbs without endometrial activity.* Surgical excision of symptomatic rudimentary bulbs is required. With mullerian agenesis, traditional conception is impossible, but pregnancy may be achieved using sophisticated technology involving oocyte retrieval, fertilization, and implantation into a surrogate.