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赫林-韦纳-温德利希综合征罕见病例报告:解析异常泌尿系统异常及文献综述

A rare case report of Herlyn-Werner-Wunderlich syndrome: Unraveling unusual urinary anomalies and literature review.

作者信息

Xu Xiaotong, Tian Yanpeng, Zhou Jingwen, Li Zhongkang, Meng Li, Huang Xianghua, Zhang Mingle

机构信息

Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China.

Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450052, PR China.

出版信息

Heliyon. 2024 Jun 25;10(13):e33558. doi: 10.1016/j.heliyon.2024.e33558. eCollection 2024 Jul 15.

DOI:10.1016/j.heliyon.2024.e33558
PMID:39071571
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11276916/
Abstract

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital genitourinary abnormality defined by uterine didelphys, obstructed hemivagina, and ipsilateral urological anomalies. Accurate diagnosis and prompt commencement of therapy can be difficult owing to heterogeneous genitourinary malformation among different patients. This is a case report of a patient with rare HWWS with uterine didelphys, obstructed hemivagina, vagina-ureteral remnant fistula (Gartner's duct cyst), and ipsilateral kidney dysgenesis who complained of intermittent abdominal pain during menstruation. The right ureteral remnant of the patient was distinctive, with three portions. The upper section was connected to the right dysplastic kidney, the lower section formed the fistulous tract with the vagina and bladder, while the middle section communicated with Gartner's Duct Cyst, which merged with the vagina and opened to the posterior cavity of hemivagina. The lower section of the right ureter was excised and ligated during laparoscopic surgery, while the upper section was excised. The patient recovered after surgery. We presented this rare case and conducted a literature review to provide a more comprehensive understanding of HWWS. This could help gynecologists effectively reduce misdiagnosis and missed diagnosis, especially when combined with complicated urinary malformation.

摘要

赫林-韦纳-温德利希综合征(HWWS)是一种罕见的先天性泌尿生殖系统异常,其特征为双子宫、阴道半段梗阻以及同侧泌尿系统异常。由于不同患者的泌尿生殖系统畸形具有异质性,准确诊断并及时开始治疗可能具有挑战性。本文报告了一例罕见的HWWS患者,该患者存在双子宫、阴道半段梗阻、阴道-输尿管残余瘘(加特纳管囊肿)以及同侧肾发育不全,患者在月经期间出现间歇性腹痛。该患者的右侧输尿管残余部分较为独特,分为三个部分。上段与右侧发育不良的肾脏相连,下段与阴道和膀胱形成瘘管,而中段与加特纳管囊肿相通,该囊肿与阴道融合并开口于阴道半段的后腔。在腹腔镜手术中切除并结扎了右侧输尿管的下段,同时切除了上段。患者术后康复。我们展示了这一罕见病例并进行了文献综述,以更全面地了解HWWS。这有助于妇科医生有效减少误诊和漏诊,尤其是在合并复杂泌尿系统畸形的情况下。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/7fbc994d1bf3/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/b855eb6377a2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/7ff7ddce5a10/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/a506c096079b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/7fbc994d1bf3/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/b855eb6377a2/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/7ff7ddce5a10/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/a506c096079b/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d87/11276916/7fbc994d1bf3/gr4.jpg

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本文引用的文献

1
Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases.《Herlyn-Werner-Wunderlich 综合征的解剖变异、治疗和结局:1673 例文献复习》
Arch Gynecol Obstet. 2023 Nov;308(5):1409-1417. doi: 10.1007/s00404-022-06856-y. Epub 2023 Feb 24.
2
Herlyn-Werner-Wunderlich Syndrome: Case Report and Review of the Literature.赫林-韦纳-温德利希综合征:病例报告及文献综述
Diagnostics (Basel). 2022 Oct 12;12(10):2466. doi: 10.3390/diagnostics12102466.
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Conservative treatment of Herlyn-Werner-Wunderlich syndrome: Analysis and long-term follow-up of 51 cases.
保守治疗赫尔林-韦纳-武德希综合征:51 例分析及长期随访。
Eur J Obstet Gynecol Reprod Biol. 2022 Aug;275:84-90. doi: 10.1016/j.ejogrb.2022.06.013. Epub 2022 Jun 15.
4
A New Case of Herlyn-Werner-Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney.1例新的赫林-韦尔纳-温德利希综合征病例:双子宫合并单侧宫颈发育不全、阴道闭锁、宫颈远端输尿管残余瘘、输尿管囊肿及对侧多囊性发育不良肾患者的肾缺如
Diagnostics (Basel). 2021 Dec 30;12(1):83. doi: 10.3390/diagnostics12010083.
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J Obstet Gynaecol Res. 2022 Mar;48(3):869-874. doi: 10.1111/jog.15148. Epub 2022 Jan 10.
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Zhonghua Fu Chan Ke Za Zhi. 2021 Nov 25;56(11):746-752. doi: 10.3760/cma.j.cn112141-20210824-00466.
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