Secondary Amenorrhea in OHVIRA Syndrome




Kiefer, Emma
Pasciucco, Alison
Patel, Biren


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Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare obstructive Müllerian duct anomaly (MDA) producing pelvic pain, dysmenorrhea, and urinary retention. Exact prevalence of MDAs is difficult to ascertain, but studies quote those that are obstructive in nature at up to 7% of the general population. Case Presentation: A 21-year-old Caucasian nulligravida female presented for a well woman exam with no acute complaints and secondary amenorrhea for one year. She reported OCP use for many years but discontinued their use a year prior to her visit. A withdrawal bleed occurred following use of over the counter levonorgestrel (Plan B) a month prior to initial presentation. Menarche was reached at age 14 and cycles were irregular, occurring on average once every 40 days. Further gynecologic history revealed current sexual activity without pain or concern, no previous STIs, no prior pap smear, and no known breast disease. Medical, surgical and family histories were unrevealing, but social history detailed occasional alcohol and tobacco use. She denied pelvic pain, vaginal discharge, vaginal bleeding, or dyspareunia. The patient was afebrile and vital signs were unremarkable. A general physical examination showed no abnormalities. A genitourinary examination was significant for a large anterior mass effect to the hymen, a non-visible, non-palpable cervix, an enlarged, non-tender uterus spanning from just below the umbilicus to the distal vagina, and non-palpable adnexa. The anterior vaginal wall was bulging and exhibited loss of a rugae pattern prompting additional imaging studies. Preliminary abdominal and pelvic ultrasound indicated both adnexa were within normal limits and the mass was uterine in origin, fluid filled, and suspicious for hematometra. MRI findings revealed absence of the right-kidney and hematometrocolpos with suspicion of a complete septate uterus and vagina necessitating surgical evaluation. Surgical management entailed opening of the hemivagina, irrigation and drainage of the hematometrocolpos, and complete excision of the hemivagina. Intraoperative hysteroscopy revealed two uterine moieties with no connecting plane, an entirely normal left moiety and ostia, and normal vaginal mucosa in the previously occluded right hemivagina. There were no post-operative sequelae. A follow-up pelvic sonogram showed a uterine didelphys and the formal diagnosis of OHVIRA syndrome was given. Conclusion: This case further expands the heterogeneity of clinical presentations and contributes to the limited data pool of OHVIRA syndrome


Research Appreciation Day Award Winner - 2022 Texas College of Osteopathic Medicine, 2022 Women's Health Research Award - 1st Place
Research Appreciation Day Award Winner - 2022 Texas College of Osteopathic Medicine, 2022 TCOM Student Research Award - Best Case Study - 1st Place