Herlyn-Werner-Wunderlich syndrome: clinical considerations and management

Herlyn-Werner-Wunderlich syndrome (HWWS), defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct malformation, usually diagnosed after menarche, when symptoms related to haematocolpos arise. We report a case of a 14-year-old patient who presented to the emergency department complaining of proctalgia and pelvic pain treated in our medical centre. Ultrasound and abdomino-pelvic MRI imaging studies confirmed the diagnosis. Treatment was surgical incision of the vaginal septum. At the follow-up visit, after the initial procedure, excess vaginal tissue was excised using a hysteroscopic approach during diagnostic vaginoscopy. Vaginoscopy-assisted treatment of the patient proved to be a safe and effective minimally invasive treatment modality that resulted in symptomatic relief and fertility preservation. In conclusion, although premenarche is asymptomatic in the vast majority of cases, HWWS would be optimally diagnosed in childhood to avoid acute late complications, although it is usually first diagnosed after menarche as a result of haematocolpos. Gynaecologists should consider the syndrome in the presence of pelvic mass, renal agenesis, menstrual changes and cyclic pelvic pain.