Jia Guifeng, Chai Wei, Cui Miao, Wen Yan, Cui Lifeng, Gong Fengyan
Department of Gynecology and Obsterics, The First Hospital of Jilin University, Changchun, Jilin, China Eastgene Laboratory, Flushing, New York.
Medicine (Baltimore). 2018 Sep;97(36):e12004. doi: 10.1097/MD.0000000000012004.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital abnormality of the urogenital tract characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It is usually diagnosed after menarche, with a clinical presentation of dysmenorrhea, recurrent abdominal pain, and irregular menses. However, it is rare to diagnose it during pregnancy, subsequently resulting in spontaneous abortion.
A 22-year-old Chinese woman with HWWS whose left uterine pregnancy underwent spontaneous abortion presented with a right perforated obstructed hemivagina and right renal agenesis. The right vaginal septum was resected and the hematocolpos was drained, thereby relieving lower abdominal pain and preserving future fertility.
Co-presentation of unilateral renal agenesis and uterus didelphys should encourage clinicians to rule out HWWS. Early diagnosis and subsequent treatment can avoid possible serious complications.
赫林-韦纳-温德利希综合征（HWWS）是一种罕见的泌尿生殖道先天性畸形，其特征为双子宫、阴道半段梗阻和同侧肾缺如。通常在初潮后诊断，临床表现为痛经、反复腹痛和月经不规律。然而，在孕期诊断出该疾病的情况罕见，随后常导致自然流产。
一名22岁患有HWWS的中国女性，其左侧子宫妊娠发生自然流产，伴有右侧阴道半段梗阻穿孔及右侧肾缺如。切除右侧阴道隔并引流阴道积血，从而缓解了下腹部疼痛并保留了未来的生育能力。
单侧肾缺如与双子宫同时出现应促使临床医生排除HWWS。早期诊断及后续治疗可避免可能出现的严重并发症。
