Herlyn-Werner-Wunderlich综合征的晚期诊断:是否需要早期筛查?

IF 1.3 4区 医学 Q3 PEDIATRICS Congenital Anomalies Pub Date : 2023-09-22 DOI:10.1111/cga.12542
Wissam Arab, Yara Abdelkhalek, Antoine Zoghbi, David Atallah
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Late diagnosis of Herlyn-Werner-Wunderlich syndrome: Is there a need for an early screening?
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly related to an abnormal development of the Mullerian ducts during organogenesis: it consists of uterovaginal duplication with obstructed hemivagina and unilateral renal agenesis. Its incidence varies between 0.1% and 3.8%. 1 Alterations in development of both Mullerian and Woll-fian ducts lead to this anomaly: uterovaginal duplication with obstructed hemivagina is the result of lateral nonfusion of the Mullerian ducts with asymmetric obstruction, while renal agenesis results from a defect in the development of the Wollfian duct. 2 Multiple environmental and genetic factors may be involved in its development. The age of presentation of HWW syndrome can vary according to the degree of vaginal obstruction. Usually, in cases with complete obstruction, patients present few months after menarche with recurrent pelvic pain due to hematocolpos during menses. However, the septum can be initially incomplete or gets perfo-rated if very thin, leading to incomplete obstruction. The presence of fen-estration as such can delay the diagnosis because fully distended hematocolpos and its related
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来源期刊
Congenital Anomalies
Congenital Anomalies PEDIATRICS-
自引率
0.00%
发文量
49
审稿时长
>12 weeks
期刊介绍: Congenital Anomalies is the official English language journal of the Japanese Teratology Society, and publishes original articles in laboratory as well as clinical research in all areas of abnormal development and related fields, from all over the world. Although contributions by members of the teratology societies affiliated with The International Federation of Teratology Societies are given priority, contributions from non-members are welcomed.
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