2020 Volume 36 Issue 2 Pages 153-157
OBJECTIVE: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital abnormality of the female urogenital tract. We report a rare case of a laparoscopic unilateral hysterectomy in a patient with OHVIRA syndrome with complete unilateral obstruction and peritonitis.
CASE: A 14-year-old girl with an imperforate hymen was referred to our hospital for treatment of double uterus and right uterine enlargement. Her medical history reported right renal agenesis noted at age seven and menarche at the age of 12, and for one and a half years afterwards, her dysmenorrhea worsened. She has one cervix, a normal left uterus and a right uterus away from the vagina. In our first consultation, she was diagnosed with OHVIRA syndrome with complete unilateral obstruction and menstrual molimina. A magnetic resonance imaging (MRI) revealed right uterine congestion and a chocolate cyst of the right ovary. We decided upon surgery as a strategy for evaluation and treatment. Low dose estrogen-progestin (LEP) was used to reduce pain while she waited for surgery, but she underwent an emergency surgery at the diagnosis of peritonitis. Laparoscopic right hysterectomy and right ovarian cystectomy were performed and the left uterus was properly maintained. The postoperative diagnosis was same as the initial diagnosis.
CONCLUSION: Early diagnosis of OHVIRA syndrome with complete unilateral obstruction is important because a delay in treatment may result in pelvic adhesions and endometriosis due to menstrual reflux. In this case, laparoscopic surgery was useful for confirming the diagnosis and treatment.
