Alberta Children's Hospital, University of Calgary, Alberta Precision Laboratories, Calgary, AB, Canada.
University of Manitoba, Shared Health Services, Winnipeg, MB, Canada.
J Pediatr Adolesc Gynecol. 2022 Oct;35(5):593-596. doi: 10.1016/j.jpag.2022.02.003. Epub 2022 Mar 8.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital anomaly characterized by uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal anomaly.
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.
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.
单侧阴道闭锁伴同侧肾发育不全（OHVIRA）是一种罕见的先天性畸形，其特征为双子宫、单侧阴道闭锁和同侧肾发育不全。
对一名死于妊娠晚期的死胎进行尸检时，意外发现该胎儿存在双子宫、左侧阴道闭锁、左侧盆腔、萎缩性双肾盂，且两条左侧输尿管均进入左侧阴道闭锁。此外，该胎儿还存在肛门闭锁、单脐动脉和隐性脊柱裂。
尽管 OHVIRA 较为罕见，但它是一种有充分文献记载的先天性畸形。然而，在此病例之前，尚未有 OHVIRA 在尸检或胎儿中被描述的报道。此外，OHVIRA、肛门直肠畸形和脊柱裂的关联从未被报道过。通过分享此病例，我们希望提高围产期保健提供者对这一实体的认识，并有助于进一步阐明仍不完全清楚的泌尿生殖系胚胎学。
