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*Introduction:* The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of uterus and upper part (2/3) of vagina in women showing normal development of secondary sexual characteristics and a normal 46,XX karyotype. It affects approximately 1 of 4500 women and may be associated with vaginal atresia.
Case Report: A 24yr old female, Laxmi, presented in Gynaecology OPD with Primary amenorrhea. There was no history of periodic pain in abdomen, lump in abdomen, hirsutism, hoarseness of voice, tuberculosis.
Patient had history of Diagnostic laparoscopy 4 years back, which revealed a rectovaginal septum in between bladder and mid part of rectum, and hence diagnosis of MRKH was made. Patient was advised reconstruction surgery of Vagina before marriage. Patient admitted for Vaginoplasty.
On Examination- Secondary sexual characters well developed, average built.
Per Abdomen Examination- Soft, non-tender, no organomegaly.
Local examination- No ectopic gonads, labial folds normal, Anal sphincter in normal position, vaginal pouch < 2cm present.
Per Rectal Examination- Uterus not felt, no mass felt.
Hospital Course: After taking written and informed consent, under Ultrasonographic guidance, patient was taken for McIndoe Procedure. A transverse incision given 2cm below urinary meatus, space dissected in forward direction with finger. Approximately 8cm deep vaginal pouch created. Vaginal mould inserted and kept in situ. Catherization done for 24 hours post-operatively. Patient was discharged after 7 days, and was counselled for proper insertion and cleaning of mould. On follow up, after 1 month, patient reported to OPD and 8 cm of vaginal pouch was found intact, no post operative infection, adhesions or scarring seen.
Conclusion: Timely diagnosis and vaginoplasty under skilled hands can give good results.
