Bösenberg Adrian T, Brown Robin A
Department of Anaesthesia, Faculty of Health Sciences, South Africa.
Curr Opin Anaesthesiol. 2008 Jun;21(3):323-31. doi: 10.1097/ACO.0b013e3282f9e214.
To evaluate the impact of recent research on the management of congenital diaphragmatic hernia in the light of new theories on embryological development, earlier antenatal diagnosis, fetal and postnatal interventions together with advances in perinatal intensive care.
The year 2007 provided in excess of 200 publications that address various aspects of congenital diaphragmatic hernia. The genetic basis and the causes of pulmonary hypoplasia at the molecular level are slowly being unravelled. Fetal MRI of lung volume, lung-head ratio, liver position and size of diaphragmatic defect have all been evaluated as early predictors of outcome and with a view to prenatal counselling. The impact of fetal interventions such as fetal endoluminal tracheal occlusion, the mode of delivery, the surgical techniques and agents for treating pulmonary hypertension were evaluated. The influence of associated anomalies and therapeutic interventions on the outcome and quality of life of survivors continue to be appraised.
Deferred surgery after stabilization with gentle ventilation and reversal of pulmonary hypertension remain the cornerstones of management. Optimal presurgery and postsurgery ventilatory settings remain unproven. Continued improvement in neonatal intensive care raises the bar against which any intervention such as fetal endoluminal tracheal occlusion and extracorporeal membrane oxygenation will be judged.
根据胚胎发育新理论、更早的产前诊断、胎儿及产后干预以及围产期重症监护的进展，评估近期研究对先天性膈疝治疗的影响。
2007年发表了200多篇关于先天性膈疝各方面的论文。肺发育不全在分子水平上的遗传基础和病因正逐渐被揭示。胎儿肺容积、肺头比、肝脏位置以及膈疝缺损大小的磁共振成像（MRI）均已作为预后的早期预测指标进行评估，并用于产前咨询。评估了胎儿干预措施的影响，如胎儿气管腔内封堵、分娩方式、治疗肺动脉高压的手术技术及药物。相关畸形和治疗干预对幸存者预后及生活质量的影响仍在评估中。
在通过轻柔通气实现稳定及逆转肺动脉高压后延迟手术仍是治疗的基石。术前和术后的最佳通气设置仍未得到证实。新生儿重症监护的持续改善提高了评判诸如胎儿气管腔内封堵和体外膜肺氧合等任何干预措施的标准。
