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一名年轻女性的赫林-韦尔纳-温德利希综合征的偶然诊断：一例罕见病例报告

An Incidental Diagnosis of Herlyn-Werner-Wunderlich Syndrome in a Young Female: A Case Report of a Rare Entity.

作者信息

Gudapati Sravya, Chaudhari Kamlesh, Dave Apoorva, Shinde Dhanajay

机构信息

Department of Obstetrics and Gynaecology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND.

Department of Radiodiagnosis, Datta Meghe Institute of Higher Education and Research (DMIHER), Wardha, IND.

出版信息

Cureus. 2024 Jan 30;16(1):e53227. doi: 10.7759/cureus.53227. eCollection 2024 Jan.

DOI:10.7759/cureus.53227
PMID:38425614
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10903378/
Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a rare genetic condition of the urogenital system, is characterized by distinctive features such as ipsilateral renal agenesis, obstructed hemivagina, and uterus didelphys. It is also referred to as Herlyn-Werner-Wunderlich (HWW) syndrome. Its clinical manifestations include dysmenorrhea, consistent abdominal pain, and infrequent periods. It is typically diagnosed after menarche. We report a case of a 20-year-old female who was admitted to the casualty ward following a road accident. She was Incidentally found to have uterine didelphys with hemorrhagic cystic lesion and left renal agenesis on ultrasonography (USG). She also had gallbladder stones, along with the findings mentioned above. Clinicians should exclude HWW syndrome in cases where uterine didelphys and unilateral renal agenesis coexist. Prompt identification and treatment of the condition can help avoid potential untoward pregnancy-related issues in the future.

摘要

梗阻性半阴道并同侧肾异常（OHVIRA）综合征是一种罕见的泌尿生殖系统遗传性疾病，其特征为同侧肾缺如、梗阻性半阴道和双子宫等独特表现。它也被称为赫林 - 韦纳 - 温德利希（HWW）综合征。其临床表现包括痛经、持续性腹痛和月经不调。通常在初潮后确诊。我们报告一例20岁女性，因道路交通事故入院急诊病房。超声检查（USG）偶然发现她有双子宫伴出血性囊性病变及左肾缺如。她还伴有胆囊结石以及上述发现。临床医生在双子宫和单侧肾缺如并存的病例中应排除HWW综合征。及时识别和治疗该疾病有助于避免未来潜在的不良妊娠相关问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/e3a8cafbb738/cureus-0016-00000053227-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/cae9b63ab018/cureus-0016-00000053227-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/a616d777ed50/cureus-0016-00000053227-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/3f2dd318f459/cureus-0016-00000053227-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/5d49bd7faa9e/cureus-0016-00000053227-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/e3a8cafbb738/cureus-0016-00000053227-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/cae9b63ab018/cureus-0016-00000053227-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/a616d777ed50/cureus-0016-00000053227-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/3f2dd318f459/cureus-0016-00000053227-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/5d49bd7faa9e/cureus-0016-00000053227-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/09e8/10903378/e3a8cafbb738/cureus-0016-00000053227-i05.jpg

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本文引用的文献

1
New classification of Herlyn-Werner-Wunderlich syndrome.赫林-韦纳-温德利希综合征的新分类
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Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review.双子宫女性中梗阻性半阴道与肾发育不全的不对称侧别分布：机构病例系列及系统文献综述
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