Incidental diagnosis of Herlyn-Werner-Wunderlich syndrome complicated with pyocolpos

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly typically presenting after menarche, often with symptoms like abdominal pain, vaginal discharge, infertility, or spontaneous abortion. In some cases, HWWS is discovered incidentally on imaging. Here, we report the case of a 19-year-old female with a known history of a congenital single kidney who presented with delayed fertility and an abnormal ultrasound that showed a bicornuate uterus and fluid collection. Further imaging and surgical assessment revealed uterus didelphys, a right transverse vaginal septum, and pyocolpos, consistent with HWWS. Management involved draining the fluid collection, followed by pregnancy-related care. This case emphasizes the importance of routine evaluations and considering a broad differential diagnosis that includes rare congenital anomalies like HWWS. This condition results from a failure of Müllerian duct fusion and can present with various symptoms, including cyclic pelvic pain and hematocolpos due to an obstructed hemivagina. Although our patient was asymptomatic, her HWWS was identified during fertility evaluation, highlighting the challenges of diagnosing congenital anomalies and the need for surgical expertise in their management. HWWS often presents with fertility challenges and requires radiographic imaging for accurate diagnosis, with surgical intervention as the primary treatment approach.