Managing the case of Ohvira Syndrome

A report describes a case of a 28-year-old female who complained of acute urinary retention and a history of severe cyclical dysmenorrhoea along with dull, aching lower abdominal pain for three months. 

The patient was nulligravida and experienced menarche at the age of 10, with a regular menstrual cycle.

Pervaginal examination revealed a bulge extending from the mid vagina on the right side to the right fornix, resembling 12 weeks of the pregnant uterus; a small cervix was also palpable on the left side. Ultrasonography and MRI revealed a large pelvic collection, likely suggesting hematocolpos (129×88×82 mm), a didelphys uterus, and the absence of a right kidney, which prompted the diagnosis of OHVIRA syndrome. 

After Routine blood investigations, the patient was scheduled for hematocolpos, hematometra drainage, and diagnostic laparoscopy. Examination revealed the same bulge along with the left normal cervical opening. Hematometras were drained under nanoscopic guidance, revealing around 450 ml of thick, tarry, brown-colored liquid.

Laparoscopy revealed the dilation of the lower segment due to the collection of brown-colored material, which gradually decreased in size, confirming the diagnosis of uterus didel-phys. There was no evidence of hematosalpinx, endometriosis, or pelvic adhesions. Both fallopian tubes and ovaries appeared normal in morphology. Post-procedure, the patient is doing well, has received counseling, and is under regular follow-up.

Chaudhary G, Gadre S. Ohvira Syndrome: A complex and rare diagnosis, yet simple management. Indian Obstetrics and Gynecology/ 2024;14(2)