Herlyn-Werner-Wunderlich Syndrome Complicated by Right Ectopic Atrophic Kidney: A Case Report from a Primary Hospital

Abstract Background Herlyn-Werner-Wunderlich syndrome (HWWS), also termed obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare congenital Müllerian duct anomaly frequently accompanied by structural abnormalities of the ipsilateral kidney and ureter. Owing to its nonspecific clinical features, which often overlap with common acute abdominal conditions such as pelvic inflammatory disease and appendicitis-HWWS is frequently misdiagnosed or missed in primary healthcare settings, particularly among post-menarchal adolescents with atypical symptoms. This case report presents a 9-year-old girl with HWWS and ipsilateral ectopic atrophic kidney associated with an ectopic ureteral orifice, and discusses the diagnostic reasoning, stepwise imaging strategy, and multidisciplinary management experience gained at a primary hospital. Case presentation: A 9-year-old girl presented with intermittent lower abdominal distending pain for 15 days. Menarche had occurred four months earlier; menses were irregular, without cyclic dysmenorrhea or vaginal purulent discharge. Initial pelvic ultrasonography demonstrated a mixed echogenic pelvic mass and absent right kidney. Subsequent contrast-enhanced pelvic MRI and CT urography (CTU) confirmed the diagnosis of type I HWWS, characterized by uterus didelphys with double cervix, right vaginal atresia with hematometra and hematocolpos, and a right ectopic atrophic kidney with an ectopic ureteral orifice. After multidisciplinary consultation involving gynecology, urology, and general surgery, the patient underwent combined hysteroscopic resection of the right vaginal oblique septum, laparoscopic resection of the ectopic right kidney and ureter, and cystoscopy. The intraoperative course was uneventful. Postoperative anti-infective therapy and vaginal care were provided; the patient recovered satisfactorily and was discharged on postoperative day 10 without abnormal vaginal discharge. Conclusion HWWS exhibits heterogeneous and often atypical manifestations in adolescent girls. Primary-care physicians should maintain a high index of suspicion and adopt an integrated genitourinary diagnostic mindset. A stepwise imaging strategy combining ultrasonography, MRI, and CTU can significantly improve early recognition. Combined minimally invasive surgery under multidisciplinary collaboration is safe and feasible, and may serve as a useful reference for the management of complex congenital genitourinary malformations in primary hospitals.