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赫林-韦纳-温德利希综合征:一例延迟诊断病例报告及阴道纵隔切除术手术技巧的视频展示

Herlyn-Werner-Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection.

作者信息

Pittokopitou Savia, Kathopoulis Nikolaos, Protopapas Athanasios, Domali Ekaterini

机构信息

Unit of Gynecological Ultrasound, 1st Department of Obstetrics & Gynecology, National and Kapodistrian University of Athens, Alexandra Hospital, Athens, Greece.

Endoscopic Surgery Unit, 1st Department of Obstetrics & Gynecology, National and Kapodistrian University of Athens, Alexandra Hospital, Athens, Greece.

出版信息

J Obstet Gynaecol Res. 2021 Jun;47(6):2242-2245. doi: 10.1111/jog.14743. Epub 2021 Mar 15.

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of Mullerian duct development characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. We present a case of a 29-year-old nulliparous woman, who was referred to our hospital complaining of chronic pelvic pain, dyspareunia, and a palpable mass in her vagina. At the age of 12, she underwent surgery because of a didelphys uterus diagnosis. Subsequently, she was operated on twice for endometriomas. At our institution, clinical and imaging findings revealed an obstructed hemivagina setting the diagnosis of HWW syndrome. Some of the various syndrome types may go unnoticed for months or even years after the onset of menstruation. Early diagnosis, followed by proper surgical treatment, is the key to avoid potentially severe complications.

摘要

赫林-韦纳-温德利希(HWW)综合征是一种罕见的苗勒管发育先天性异常,其特征为双子宫伴盲端半阴道和同侧肾缺如。我们报告一例29岁未生育女性病例,该患者因慢性盆腔疼痛、性交困难及阴道内可触及肿物转诊至我院。12岁时,她因双子宫诊断接受了手术。随后,她因子宫内膜瘤接受了两次手术。在我院,临床和影像学检查结果显示半阴道梗阻,从而确诊为HWW综合征。某些类型的综合征在月经初潮后的数月甚至数年可能未被发现。早期诊断并随后进行适当的手术治疗是避免潜在严重并发症的关键。

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