Distal Vaginal Atresia Combined with Unicornoate Uterus: A Case Report

Congenital absence of the vagina with variable uterine development known as Mullerian agenesis. We presented the case of a patient with distal vagina agenesis with presence of proximal vagina and functioning unicornoate uterus. A 13-year-old patient was referred to pelvic floor service due to primary amenorrhea and pelvic pain. She was diagnosed with the distal vaginal agenesis, functional unicornoate uterus and the ectopic right kidney. Rectoabdominal examination revealed segmental vaginal agenesis and a likely atretic cervix with a huge abdominopelvic mass of about 10 cm in size. On exploratory laparotomy, the bladder was completely dilated and the right kidney was ectopic and both kidneys had moderate hydronephrosis. On the left, a unicorn uterus containing blood and clot, and on the right, a non-functional rudimentary horn with a normal fallopian tube was seen. A neovagina was created by dissection of the space between the urethra hyatus and rectum in laparatomy. It is important to note that the patient may initially present with urinary symptoms and renal signs. Therefore, in the symptoms of urinary tract obstruction, mullerian anomalies should be considered.