An Unusual Appearance of the Post-pubertal Herlyn-Werner-Wunderlich Syndrome with Acute Abdominal Pain: A Case Report

Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital defect. It is detected by unilateral low vaginal obstruction, uterus didelphys, and ipsilateral kidney agenesis. It usually becomes apparent with pain, dysmenorrhea, and presence of a vaginal or pelvic mass. Purulent vaginal discharge may also happen rarely because of infective complications of the obstructed hemivagina. In this report, we describe a post-pubertal case with acute abdominal pain. Case: The patient was a 13-yr-old girl who was referred to us with acute abdominal pain one year after the onset of her menarche. In the pelvic examination, we detected hematocolpos. Abdominopelvic-computed tomography scan confirmed the presence of mullerian duct anomalies with uterus didelphys. This case of HWW syndrome along with pyocolpus was managed by vaginal septum resection, drainage of pus, and salpingectomy. Conclusion: The symptoms of HWW syndrome should be monitored in early puberty to prevent more complications. Key words: Herlyn-Werner-Wunderlich syndrome, Uterus didelphys, Kidney agenesis, Mullerian duct anomaly