The Herlyn-Werner-Wunderlich Syndrome: a Rare Case Report

Abstract

Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) is an aberrated and special type of Mesonephric duct malformation and Müllerian duct anomaly. This mostly reports 5% of Müllerian duct anomalies. The majority of cases of OHVIRA are presented alongside uterine didelphys. The presence of uterus didelphys with OHVIRA are the conditions, collectively referred to as the Herlyn- WernerWunderlich Syndrome. Its cause is a complete failure of the Müllerian conduits to fuse. Initial symptoms are typically induced by secretions concentrating within the hemivaginal blockage. Patients are usually of reproductive age. Symptoms in patients with this syndrome are mostly complaints of severe dysmenorrhea, pain in the lower abdomen, palpable mass in the pelvis or vagina abrupt urinary retention, fever, or puking, in addition to atypical vaginal secretions, and abnormal vaginal discharge. Cases seldom reveal complaints of infertility in the majority. Here, we focus on the distinctive symptoms of HWW syndrome and the significance of imaging findings in the diagnosis of the condition in a case of a 23-year-old girl who visited our outpatient department primarily with concerns of infertility and periodic pelvic ache. She was initially diagnosed with OHVIRA syndrome through ultrasonography. The outcomes of her MRI are also discussed. The couple was counselled regarding the disease, complications, and treatment options however due to financial constraints they were lost to follow-up.

Keywords

• ohvira
• uterus didelphys
• renal agenesis
• hemivagina
• infertility