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胎儿单侧阴道闭锁并同侧肾发育不良(OHVIRA)综合征的产前诊断。

Prenatal Diagnosis of Fetal Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome.

机构信息

Department of Obstetrics and Gynecology, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea.

Department of Pediatrics, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul 07804, Republic of Korea.

出版信息

Medicina (Kaunas). 2023 Apr 4;59(4):703. doi: 10.3390/medicina59040703.

DOI:10.3390/medicina59040703
PMID:37109661
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10146732/
Abstract

: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Most cases of OHVIRA have been reported in adolescents or adults. Gartner duct cysts, including those manifesting as vaginal wall cysts, are also rare. Fetal OHVIRA syndrome and Gartner duct cysts are difficult to diagnose. : Here, the authors report a case of combined OHVIRA and Gartner duct cyst diagnosed prenatally by ultrasonography, along with a brief review of the relevant published reports. A 30-year-old nulliparous female was referred to our institution at 32 weeks' gestation for fetal right kidney agenesis. Detailed ultrasonographic examinations using 2D, 3D, and Doppler ultrasounds revealed hydrocolpometra, and uterus didelphys, with a normal anus and right kidney agenesis. : When encountering female fetuses with ipsilateral renal agenesis or vaginal cysts, clinicians should be aware of OHVIRA syndrome and Gartner duct cysts and perform systematic ultrasonographic examinations for other genitourinary anomalies.

摘要

: 单侧阴道闭锁和同侧肾发育不全(OHVIRA)综合征,又称 Herlyn-Werner-Wunderlich 综合征,是一种罕见的综合征,其特征为双子宫、单侧阴道闭锁和同侧肾发育不全三联征。大多数 OHVIRA 病例发生在青少年或成年期。Gartner 管囊肿,包括表现为阴道壁囊肿的囊肿,也很少见。胎儿 OHVIRA 综合征和 Gartner 管囊肿很难诊断。: 本文作者报告了 1 例产前超声诊断为合并 OHVIRA 和 Gartner 管囊肿的病例,并对相关文献进行了简要复习。1 名 30 岁的初产妇因胎儿右肾发育不全,于妊娠 32 周被转诊至本院。使用二维、三维和多普勒超声进行详细的超声检查显示有水瘤和双子宫,肛门正常,右侧肾发育不全。: 当遇到单侧肾发育不全或阴道囊肿的女性胎儿时,临床医生应意识到 OHVIRA 综合征和 Gartner 管囊肿的存在,并对其他泌尿生殖系统异常进行系统的超声检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/0e635f5fe5b5/medicina-59-00703-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/64a595c82acd/medicina-59-00703-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/2d2fe0a22bac/medicina-59-00703-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/0e635f5fe5b5/medicina-59-00703-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/64a595c82acd/medicina-59-00703-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/2d2fe0a22bac/medicina-59-00703-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4617/10146732/0e635f5fe5b5/medicina-59-00703-g003.jpg

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本文引用的文献

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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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ASRM müllerian anomalies classification 2021.美国生殖医学学会 Müllerian 畸形分类 2021 版
Fertil Steril. 2021 Nov;116(5):1238-1252. doi: 10.1016/j.fertnstert.2021.09.025.
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Diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome: a case report.赫林-韦尔纳-温德利希综合征的诊断与治疗:一例报告
梗阻性半阴道合并同侧肾缺如:一例具有挑战性的病例报告及处理流程图
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Herlyn-Werner-Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection.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
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