Atypical Presentation and Delayed Diagnosis of Herlyn-Werner-Wunderlich Syndrome: A Case Report and Literature Review

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare and complex Mullerian duct abnormality that is diagnostically challenging. It is also known as OHVIRA syndrome. The triad of uterine didelphys, obstructed hemivagina and ipsilateral renal anomaly characterises the syndrome. A 25-year-old lady was referred with prolonged foul-smelling vaginal discharge. Her periods were regular with normal flow. A vaginal examination revealed a normal cervix and fullness of left adnexa. A pelvic ultrasound showed a left adnexal mass. Computed tomography imaging of the abdomen and pelvis revealed two uterine cavities with a large cystic lesion at the level of the cervix. This lesion was communicating with the uterine cavity, raising the possibility of obstruction. Only the right kidney was present. A diagnosis of HWWS was made. The patient underwent diagnostic laparoscopy, examination under anaesthesia and excision of the vaginal septum. At follow-up, she remained well at 6 weeks and 3 months. The normal mesonephric duct plays an important role in the formation of the uterus, vagina and the upper urinary tract. HWWS occurs when one of the mesonephric ducts is absent or injured during embryogenesis. Women with HWWS usually present after menarche with varied symptoms, causing delays in diagnosis and treatment. Therefore, having a high suspicion index and awareness of Mullerian duct abnormalities is crucial. The gold standard for the evaluation of HWWS is magnetic resonance imaging. Surgical excision of the septum is the advocated treatment of choice. Prompt intervention lowers complications that impair fertility.