Prenatal diagnosis, prediction of outcome and in utero therapy of isolated congenital diaphragmatic hernia

被引:57
|
作者
Done, Elisa [1 ]
Gucciardo, Leonardo [1 ]
Van Mieghem, Tim [1 ]
Jani, Jacques [2 ]
Cannie, Mieke [4 ]
Van Schoubroeck, Dominique [1 ]
Devlieger, Roland [1 ]
De Catte, Luc [1 ]
Klaritsch, Philipp [3 ]
Mayer, Steffi [3 ]
Beck, Veronika [3 ]
Debeer, Anne [3 ,6 ]
Gratacos, Eduardo [5 ]
Nicolaides, Kypros [2 ]
Deprest, Jan [1 ,3 ]
机构
[1] Katholieke Univ Leuven Hosp, Fetal Med Unit, Dept Obstet & Gynaecol, Div Woman & Child Univ Hosp, B-3000 Louvain, Belgium
[2] Kings Coll Hosp London, Fetal Med Unit, London, England
[3] Katholieke Univ Leuven, Fac Med, Interdepartemental Ctr Surg Technol, B-3000 Louvain, Belgium
[4] Katholieke Univ Leuven Hosp, Dept Radiol, B-3000 Louvain, Belgium
[5] Hosp Clin Barcelona, Fetal Med Unit, Barcelona, Spain
[6] Katholieke Univ Leuven Hosp, Dept Neonatol, B-3000 Louvain, Belgium
关键词
congenital diaphragmatic hernia; pulmonary hypoplasia; lung-to-head ratio; fetal tracheal occlusion;
D O I
10.1002/pd.2033
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Congenital diaphragmatic hernia (CDH) can be associated with Genetic or structural anomalies with poor prognosis. In isolated cases, survival is dependent on the degree of lung hypoplasia and liver position. Cases Should be referred in utero to tertiary care centers familiar with this condition both for prediction of outcome as well as timed delivery. The best validated prognostic indicator is the lung area to head Circumference ratio. Ultrasound is used to measure the lung area of the index case, which is then expressed as a proportion of what is expected normally (observed/expected LHR). When O/E LHR is <25% Survival chances are < 15%. Prenatal intervention. aiming to stimulate lung growth, can be achieved by temporary fetal endoscopic tracheal occlusion (FETO). A balloon is percutaneously inserted into the trachea at 26-28 weeks, and reversal Of Occlusion is planned at 34 weeks. Growing experience has demonstrated the feasibility and safety of the technique with a Survival rate of about 50%. The lung response to, and outcome after FETO, is dependent on pre-existing lung size as well gestational a,,e at birth. Early data show that FETO does not increase morbidity in survivors, when compared to historical controls. Several trials are currently under design. Copyright (C) 2008 John Wiley & Sons, Ltd.
引用
收藏
页码:581 / 591
页数:11
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