Purpose or Case Report: Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome, also known as Herlyn-Werner-Wunderlich Syndrome, is a rare congenital anomaly of the female urogenital tract. OHVIRA syndrome results from abnormal development of the Mullerian duct during weeks 10-12 of gestation. OHVIRA syndrome is characterized by a classic triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Clinical presentation of OHVIRA syndrome depends on the degree of hemivaginal obstruction, with Type 1 defined as complete obstruction and Type 2 characterized by incomplete obstruction. In patients with complete hemivagina obstruction, OHVIRA syndrome may manifest in adolescence with pelvic distention and cyclic pain secondary to hematometrocolpos. Several renal anomalies have been described in OHVIRA syndrome including renal agenesis, multicystic dysplastic kidney, and duplicated collecting system. In this report, we present two cases of OHVIRA syndrome with different manifestations of unilateral renal anomaly.

The first case features a 12-year-old female who presented with heavy menstrual bleeding. Transpelvic ultrasound demonstrated possible uterine septation and a dilated cervix containing heterogeneous avascular material. Subsequent pelvic MRI revealed duplicated uterine horns with two separate cervices, consist with uterus didelphys. On MRI, the left cervix was distended with non-enhancing debris, suggestive of obstructed left hemivagina. No left kidney was identified in the left renal fossa or elsewhere in the abdomen or pelvis on renal ultrasound. Multiple non-communicating cysts were seen in the left abdomen, raising suspicion for multidysplastic left kidney with arrested ascent.

The second case involves a 10-year-old prepubertal female who presented from an outside hospital for evaluation of a uterine anomaly, which was incidentally seen on MRI pelvis performed for characterization of sacrococcygeal teratoma. Transpelvic ultrasound and pelvic MRI at our institution showed duplicated uterine horns with limited visualization of the cervices due to small size. Differential considerations included uterus didelphys and bicornuate uterus, and follow-up imaging was recommended after puberty for definitive diagnosis. MRI pelvis obtained 4 years later demonstrated complete duplication of the uterus, cervix, and vagina with obstruction of the right hemivagina. MRI pelvis also demonstrated right renal agenesis, consistent with OHVIRA syndrome.
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