An incidental presentation of Herlyn–Werner-Wunderlich syndrome with secondary infertility

Abstract

Rationale: Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemi-vagina with ipsilateral renal agenesis (OHVIRA) syndrome, is a rare congenital anomaly of the Müllerian and Wolffian ducts. It is often asymptomatic until puberty and is usually misdiagnosed as infertility. Patient concerns: A 39-year-old woman desirous of pregnancy was referred to our radiodiagnosis unit for imaging evaluation on account of secondary infertility. Diagnosis: Incidental discovery of uterine bicornis bicollis and blind-ending hemivagina was noted at hysterosalpingography. Interventions: This necessitated the search for other genitourinary tract anomalies, and ipsilateral renal agenesis was also discovered. Outcomes: A diagnosis of Herlyn-Werner-Wunderlich syndrome was made. Lessons: Magnetic resonance imaging (MRI) is considered the gold standard imaging modality for the assessment of uterine malformations. However, in resource-poor centers where MRI is unavailable, a heightened clinical suspicion with the combination of available imaging modalities is important and reliable in making an accurate diagnosis, hence, successful management.