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Monday, 23 January 2017

Ovarian Disgenises at GG Hospital. And what is Ovarian disgenises?

Mrs. PB, aged 34 years first came to us on 09/02/1989 with complaints of primary amenorrhea. Her height, weight and vitals were normal. Her secondary sexual characteristics were tanner's stage II. Her ultrasound examination report came as Mullerian agenesis with non visualization of a midline uterus or ovaries and with normal appearing kidneys. Her Karyotyping revealed a mosaic turner (46XX/45XO) with the normal cell line in higher percentage than the abnormal cell line. Her hormonal profile showed a hypogonadotrophic state. She had her diagnostic laparoscopy later that month during which it was observed that the uterus was only like a thick fleshy fold in the midline with hypoplastic tubes and streak ovaries. The cervix was felt as a small nodule in the vault. She was then started on cyclical hormone replacement therapy with estradiol valerate and norgestrel (progyluton) for 6 cycles and progressive uterine growth was observed up to 6.2x2.8cms.


She had a second look laparoscopy one year prior to her marriage which revealed a normal sized uterus with patent fallopian tubes and hypoplastic ovaries. She subsequently got married in 1993 at the age of 20 years and came to us for infertility treatment in 1996.

All the while she was on cyclical hormone therapy to regularize her cycles and maintain optimum size of uterus. She underwent three cycles of donor oocyte programme between 1996 and 1999  with no success. She then discontinued her treatment with us. A very interesting, but unfortunate incident happened in 2004. She had a freak ovulation and conceived, but underwent an emergency laparotomy with right salpingectomy for a ruptured ectopic in February 2004 at another hospital. Following this, the patient resumed treatment with us from November 2005. She underwent her fourth cycle of donor oocyte programme on 16/02/2006 with transfer of three 4 cells, grade I-II embryos during which she conceived with twins. Owing to her previous history of hypoplastic uterus with short cervix she underwent prophylactic cervical cerclage at 15-16 weeks of pregnancy. The rest of her antenatal period was uneventful and hospitalized till delivery. An elective cesarean section was performed on 04/10/2006 and twin girls were delivered weighing 2.07 and 1.97kgs respectively. Both mother and baby did well on discharge. This is one of those interesting cases of ovarian dysgenesis with a miraculous response to cyclical hormone replacement therapy. The uterus had been grown from a thick fleshy midline structure to normal sized on measuring 6.0x3,0cms. Freak ovulation does occur in extremely rare circumstances. While another patient of ours with premature ovarian failure (POF) delivered her own second child owing to a freak ovulation while on HRT (She had already delivered her first child through donor oocyte programme). It was just unfortunate that our present patient had the luck of freak ovulation but it ended in a ectopic pregnancy.

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