• Gaoli Niu, Yanhong Zhai, Luhong Meng, Lingli Zhao, Nannan Liu, Xuejiao Xing, Xin Wen, and Jingjing Chen.
• Department of Gynecologic Oncology, The First Affiliated Hospital of Henan Polytechnic University (The Second People's Hospital of Jiaozuo), Jiaozuo, China.
• Medicine (Baltimore). 2022 Dec 9; 101 (49): e32264e32264.

BackgroundHerlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital Mullerian duct anomaly disease that is characterized by a triad of symptoms, didelphys uterus, blind hemivagina, and ipsilateral renal agenesis. Herein, we reported a case from China.Case PresentationAn 11-year-old patient presented to our hospital with lower abdominal pain and frequent urination. Computed tomography and magnetic resonance imaging revealed hematocolpos, uterine hemorrhage, didelphys uterus, and renal agenesis on the right side. Thus, the patient was diagnosed with HWW syndrome. Laparoscopic combined with transvaginal surgery to remove the vaginal septum, the symptoms of the lesion disappeared after the blood was discharged.ConclusionAbnormal urination and other symptoms should be carefully examined in adolescent girls with abdominal pain not menarche, since they may be related to reproductive organ development disorders and other diseases. We recommend laparoscopy combined with transvaginal surgery to remove the oblique septum in HWW syndrome, which is rarely reported.Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.

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