Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with septic shock: A case report

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex of structural abnormalities of the female urogenital tract. A nulliparous 37-year-old woman was referred to our department for an acute abdomen. Multiple uterine myomas and painful right vaginal bulge were found on gynecological examination. Transvaginal ultrasonography revealed a cystic mass and a right-side cervix that was hidden behind the longitudinal vaginal septum. For these findings, pyocolpos arising from OHVIRA syndrome was suspected. Her general condition gradually worsened within an hour, and she developed septic shock and was hospitalized in the intensive care unit (ICU). Laboratory data revealed disseminated intravascular coagulation (DIC) and multiple organ failure. Her general condition gradually improved as a result of intensive care and transvaginal puncture drainage of the abscess. Hysterectomy, performed 2 months after the acute septic episode, confirmed the diagnosis of septate uterus with obstructed hemivagina. To date, there is no documented case of OHVIRA syndrome with an eventual septic shock.