Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) - A Fetal Autopsy Case

Abstract

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital anomaly characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly.

Case: Autopsy performed on a third trimester stillborn fetus unexpectedly revealed uterine didelphys, an obstructed left hemivagina, and a left pelvic, atrophic, duplex kidney, with both left ureters entering the obstructed left hemivagina. Furthermore, the fetus had an imperforate anus, a single right umbilical artery, and spina bifida occulta.

Summary and conclusion: Although rare, OHVIRA is a well-documented congenital anomaly. However, prior to this case, there had been no description of OHVIRA in an autopsy or in a fetus. Furthermore, the association of OHVIRA, anorectal malformation, and spinal bifida has never been reported. By sharing this case, we hope to increase the awareness of this entity among perinatal healthcare providers and to help further elucidate genitourinary embryology, which is still not fully understood.

Keywords: Fetal autopsy; Herlyn-Werner-Wunderlich syndrome; OHVIRA; Obstructed hemivagina and ipsilateral renal anomaly; Renal agenesis; Vagina ureteral ectopia; Vaginal ureter ectopic insertion.