Prenatal Diagnosis of Fetal Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Soo Jung Kim, So Yeon Shim, Hyun Hae Cho, Mi Hye Park, Kyung A. Lee

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Most cases of OHVIRA have been reported in adolescents or adults. Gartner duct cysts, including those manifesting as vaginal wall cysts, are also rare. Fetal OHVIRA syndrome and Gartner duct cysts are difficult to diagnose. Case Presentation: Here, the authors report a case of combined OHVIRA and Gartner duct cyst diagnosed prenatally by ultrasonography, along with a brief review of the relevant published reports. A 30-year-old nulliparous female was referred to our institution at 32 weeks’ gestation for fetal right kidney agenesis. Detailed ultrasonographic examinations using 2D, 3D, and Doppler ultrasounds revealed hydrocolpometra, and uterus didelphys, with a normal anus and right kidney agenesis. Conclusions: When encountering female fetuses with ipsilateral renal agenesis or vaginal cysts, clinicians should be aware of OHVIRA syndrome and Gartner duct cysts and perform systematic ultrasonographic examinations for other genitourinary anomalies.

Original languageEnglish
Article number703
JournalMedicina (Lithuania)
Volume59
Issue number4
DOIs
StatePublished - Apr 2023

Bibliographical note

Publisher Copyright:
© 2023 by the authors.

Keywords

  • Gartner duct cyst
  • Herlyn-Werner-Wunderlich syndrome
  • obstructed hemivagina and ipsilateral renal agenesis syndrome (OHVIRA)
  • prenatal diagnosis

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