PRIMARY AMENORRHEA WITH APLASTIC UTERUS, BILATERAL NON-COMMUNICATING RUDIMENTARY HORNS WITH HEMATOMETRA IN LEFT HORN AND CERVICOVAGINAL APLASIA (U5A C4 V4) – A RAREST CASE REPORT

Congenital uterine malformations account for 5.5 % of unselected population, while mullerian agenesis has an incidence of 1 in 4500-5000 females. Mullerian agenesis occurs due to embryological underdevelopment of mullerian duct with resultant agenesis or atresia of the vagina, uterus or both. We report a rare case of 22 year old female with primary amenorrhea with aplastic uterus with bilateral rudimentary horns, hematometra in left rudimentary horn and Cervicovaginal aplasia. She came to our OPD with history of primary amenorrhea and vaginal discharge for 15 days. General physical examination revealed well developed secondary sexual characteristics and normal breast development. Axillary and pubic hair development was normal. A small dimple of 1 cm was noted at the site of Vagina. Uterus was felt like a small hypoplastic nodule on per rectal examination. Laparoscopy revealed bilateral rudimentary horns , combined agenesis of uterus , cervix and vagina. Resection of Bilateral rudimentary horns with hematometra and bilateral salpingectomy was done followed by Mc Indioe Vaginoplasty on follow up visit 3 months before her wedding. 3D Ultrasonography and MRI are gold standard for diagnosing Mullerian agenesis, but using laparoscopy aids in arriving at an accurate diagnosis. Patients should be provided with mental health support for better social, marital life and gestational surrogacy as the fertility option.