沃尔夫导管异常的磁共振成像评估--OHVIRA 和 OSVIRA。

Q2 Medicine Journal of Human Reproductive Sciences Pub Date : 2024-04-01 Epub Date: 2024-05-28 DOI:10.4103/jhrs.jhrs_19_24
Lukshay Bansal, Suryansh Arora, Akhila Prasad
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引用次数: 0

摘要

Herlyn-Werner-Wunderlich 综合征(女性)又称阻塞性阴道和同侧肾发育不全(OHVIRA),Zinner's 综合征(男性)又称 OSVIRA,是两种罕见的影响泌尿生殖道的先天性综合征。OHVIRA是血道阻塞和同侧肾发育不全的缩写,OSVIRA是精囊阻塞和同侧肾发育不全的缩写。长期以来,由于我们对潜在的胚胎发病机制了解甚少,这两种综合征被认为是不同的实体;然而,鉴于最近人们接受了阴道的整体沃尔夫起源,这两种综合征被认为是从同一块布上剪下来的。在本病例系列中,我们分别介绍了两例 OHVIRA 和 OSVIRA 的磁共振成像特征,并讨论了它们背后胚胎发病机制的相似之处,同时驳斥了旧观念。对胚胎发病机制的深刻理解可以让临床医生和放射科医生预测疾病的表现和结果,还可以指导筛查潜在的细微缺陷,否则这些缺陷可能会被漏诊。
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Magnetic Resonance Imaging Evaluation of Wolffian Duct Anomalies - OHVIRA and OSVIRA.

Herlyn-Werner-Wunderlich syndrome in females, also known as obstructed haemivagina and ipsilateral renal agenesis (OHVIRA), and Zinner's syndrome, also known as OSVIRA in males, are two rare congenital syndromes affecting the urogenital tract. OHVIRA is obstructed haemivagina and ipsilateral renal agenesis and OSVIRA abbreviation stands for obstructed seminal vesicle and ipsilateral renal agenesis. For a long, these syndromes were considered different entities, owing to our poor understanding of underlying embryopathogenesis; however, in light of the recent acceptance of the Wolffian origin of the vagina in its entirety, these two syndromes are considered to be cut from the same cloth. In this case series, we present magnetic resonance imaging features of two cases each of OHVIRA and OSVIRA and discuss the similarities in embryopathogenesis behind them while debunking the old concepts. A deep understanding of embryopathogenesis allows a clinician and radiologist to predict the disease manifestation, and its outcome and also acts as a guide to screen for potential subtle defects, which would otherwise have been missed.

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来源期刊
Journal of Human Reproductive Sciences
Journal of Human Reproductive Sciences Medicine-Reproductive Medicine
CiteScore
2.60
自引率
0.00%
发文量
50
审稿时长
23 weeks
期刊介绍: The Journal of Human Reproductive Sciences (JHRS) (ISSN:0974-1208) a Quarterly peer-reviewed international journal is being launched in January 2008 under the auspices of Indian Society of Assisted Reproduction. The journal will cover all aspects human reproduction including Andrology, Assisted conception, Endocrinology, Physiology and Pathology, Implantation, Preimplantation Diagnosis, Preimplantation Genetic Diagnosis, Embryology as well as Ethical, Legal and Social issues. The journal will publish peer-reviewed original research papers, case reports, systematic reviews, meta-analysis, and debates.
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