Herlyn-Werner-Wunderlich syndrome: A case report

Abstract

ntroduction. Herlyn-Werner-Wunderlich (HWW) syndrome represents a rare Mullerian and mesonephric ductal anomaly characterized by a triad of didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis complex. Case report. A 13-year-old patient was experiencing pelvic pain, dysmenorrhea. Complains of painful menarche since their onset. An initial ultrasound revealed double uterus - right 41x24mm, left 44x26mm, 63x81mm hematocolpus extends from it. The MRI scan of the abdomen and pelvis reveals duplicated reproductive organs including the uterus, cervix and vagina. On the left side, these structures appear enlarged and contain blood-filled cavities. Additionally, there is partial blockage or hemivagina affecting one-third of the left vaginal canal. A year ago this patient had a CT scan showing agenesis of the left kidney with a double uterus,similar to HWW syndrome. Sometimes there is episodic pain when urinating, as well as an imperative desire to urinate. The decision was made for the patient to undergo vaginal septum excision using laryngeal anesthesia, with the procedure being guided by ultrasound. Discussion. Symptoms like menstrual pain, vaginal bleeding, mild lower abdominal discomfort, and renal agenesis should prompt consideration of Herlyn-Werner-Wunderlich syndrome. Essential diagnostic tools such as ultrasound and MRI play a crucial role in identifying these anomalies, uncovering related complications, and guiding treatment decisions. Conclusions. The symptoms of Herlyn-Werner-Wunderlich syndrome are often nonspecific, and its manifestations can vary, which frequently leads to misdiagnosis or a delay in diagnosis. Hence, it necessitates a vigilant approach and a high level of suspicion for accurate identification.