Adolescent dysmenorrhea revealing Herlyn-Werner-Wunderlich syndrome with endometriosis: A radiologic case report

Herlyn-Werner-Wunderlich syndrome, also referred to as obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) is a rare congenital urogenital anomaly resulting from abnormal development of the Müllerian and Wolffian ducts, classically characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It typically presents after menarche with nonspecific symptoms, often leading to delayed diagnosis and increased risk of complications such as hematometra and pelvic endometriosis. Radiologic imaging plays a key role in early detection and prevention of disease progression. A 16-year-old female presented with progressive dysmenorrhea for 1.5 years and irregular menstruation. Ultrasonography demonstrated uterus didelphys, a left adnexal cyst suggestive of endometrioma, and left renal agenesis. Magnetic resonance imaging (MRI) further delineated a noncommunicating left uterine unit with cervicovaginal atresia and hematometra, consistent with type 1.2 HWWS. MRI also confirmed a left ovarian endometrioma, showing T1 hyperintensity and T2 shading. CT urography further confirmed complete left renal and ureteral agenesis with preserved contralateral renal excretory function, completing the radiologic evaluation of the associated urinary tract anomaly. These multimodality imaging findings established the diagnosis and accurately defined the extent of the congenital anomaly and its associated complications. This case emphasizes the importance of early recognition of Herlyn-Werner-Wunderlich syndrome in adolescents with persistent dysmenorrhea and underscores the pivotal role of imaging. While ultrasonography is useful for initial assessment, MRI remains the gold standard for comprehensive evaluation. Early and accurate diagnosis is essential to guide management and prevent long-term complications, including endometriosis and infertility.