The Herlyn-Werner-Wunderlich triad (OHVIRA syndrome) with good pregnancy outcome - two cases and literature review

The Herlyn-Werner-Wunderlich syndrome (HWWS) is a complex congenital malformation, originally described as a triad of symptoms: didelphys uterus, low genital obstruction and unilateral renal anomaly. The term OHVIRA is an acronym (obstructed hemivagina/hemicervix with ipsilateral renal anomaly) and refers to the same syndrome. It gained acceptance in recent years, due to reports of cases having a non-didelphys uterus (normal, septated or having other abnormalities). In the following, we report two cases with good pregnancy outcome and we provide a short discussion on published literature. We highlight differences and complications in these two cases, confirming the extreme variability of anatomic structures involved in the syndrome. Though rare, the condition allows successful pregnancies. We describe the sixth case of didelphys uterus with unique (anatomically normal) vagina and unilateral isolated hemicervix hypoplasia∕atresia. Imaging has a paramount importance in the diagnosis of HWWS∕OHVIRA, with magnetic resonance (MR) usually superseding the ultrasound (US) method, and providing other viscera details. US, MR and laparoscopy∕laparotomy complement each other, and for describing the anatomy of the obstruction a thorough clinical examination is required. The clinical course of the pathology is not standard and the management must be tailored, but term∕near-term pregnancies resulting in healthy newborns are possible in HWWS. We advocate an appropriate maternal and fetal prenatal care and long-term follow-up.