Haroon Junaid, Chamberlain Ronald S
Saint Barnabas Medical Center, Livingston, NJ 07039, USA.
Clin Pediatr (Phila). 2013 Feb;52(2):115-24. doi: 10.1177/0009922812472249.
Congenital diaphragmatic hernia is a rare but severe condition affecting 1 in 2000 to 3000 newborns with a survival rate of 67%. Although regular antenatal screening allows prenatal diagnosis in many cases, traditionally treatment has been based on postnatal surgical repair. Recent literature has pointed out the survival benefits of initial stabilization and the use of gentle ventilation strategies prior to definitive treatment, shifting the trend from immediate to delayed surgical repair. Advances in fetal intervention have allowed the introduction of fetal endoscopic tracheal occlusion as a method to hasten lung development before birth in order to minimize postnatal morbidity. Despite appropriate treatment, the long-term outcomes of these patients are plagued with numerous complications, associated with the primary pathology and also aggressive therapeutic measures. International centers of excellence have recently come together in an effort to standardize the care of such patients in hopes of maximizing their outcomes.
先天性膈疝是一种罕见但严重的疾病,每2000至3000名新生儿中就有1例受其影响,存活率为67%。尽管常规产前筛查在许多情况下可实现产前诊断,但传统上治疗一直基于出生后手术修复。最近的文献指出了初始稳定以及在确定性治疗前采用温和通气策略对存活的益处,将趋势从立即手术修复转向延迟手术修复。胎儿干预的进展使得胎儿内镜气管闭塞得以引入,作为一种在出生前加速肺发育以尽量减少出生后发病率的方法。尽管进行了适当治疗,但这些患者的长期预后仍受到众多并发症的困扰,这些并发症既与原发性病理有关,也与积极的治疗措施有关。国际卓越中心最近联合起来,努力规范对此类患者的护理,以期实现最佳预后。