Two Cases of Obstructive Hemivagina and Ipsilateral Renal Anomaly Syndrome Requiring Transvaginal Drainage in the Neonatal Period

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized by unilateral vaginal obstruction in a duplicated uterus and renal anomaly ipsilateral to the obstructed vagina. Case 1 presented with a duplicate uterovagina, left-sided vaginal obstruction, and left renal agenesis. The patient had right hydronephrosis and hydroureter with passage obstruction due to compression by a suspected vaginal hematoma. Therefore, transvaginal puncture drainage under transabdominal ultrasound guidance was performed on the obstructed vaginal wall at 5 days of age. Case 2 presented with a duplicate uterovaginal, left-sided vaginal obstruction, and left renal hypoplasia, and the ureter was opened into the left obstructed vagina. Given the risk of spontaneous perforation or infection due to urine retention in the obstructed vagina, left nephroureterectomy and drainage of the obstructed vagina were performed at 29 days of age. Both Cases 1 and 2 had no recurrence of urine retention in the obstructed vagina after surgery. OHVIRA syndrome is associated with a wide variety of pathological conditions. Therefore, it is deemed necessary to tailor the treatment plan to each individual case.