A Rare Variant of OHVIRA Syndrome Managed Laparoscopically: A Case Report

Abstract

OHVIRA syndrome is a rare Mullerian duct anomaly characterized by a triad of uterine duplication, unilateral vaginal obstruction and ipsilateral renal agenesis. We report here the case of a 14 years adolescent girl with lower abdominal pain and irregular cycles for two months. Imaging demonstrated uterus didelphys with an obstructed right hemiuterus, hematometrocolpos and ipsilateral renal agenesis. Examination under anesthesia revealed a high right sided vaginal obstruction without an accessible bulging vaginal septum. Laparoscopic excision of the obstructed right hemiuterus with right salphingectomy was therefore performed. Approximately 15ml of dark blood was encountered intraoperatively after excision of the right uterine horn. Histopatholgical examination demonstrated proliferative endomyometrium, while the excised fallopian tube showed hydrosalphinx. OHVIRA syndrome should be considered in adolescent girls presenting after menarche with progressive dysmenorrhea, cyclical pelvic pain, menstrual abnormalities or a pelvic mass even when early menstruation has been apparently normal.