Keijzer Richard, Puri Prem
Department of Pediatric Surgery, Erasmusmc-Sophia, Rotterdam, The Netherlands.
Semin Pediatr Surg. 2010 Aug;19(3):180-5. doi: 10.1053/j.sempedsurg.2010.03.001.
Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and morbidity as the result of severe respiratory failure secondary to PH and PPH. The pathogenesis of CDH and associated PH and PPH is poorly understood. Herein, we aim to review diaphragm and pulmonary development and correlate this to the abnormalities found in CDH.
先天性膈疝(CDH)是一种先天性异常,表现为膈肌后外侧缺损,也称为博赫dalek疝。其发病率为2000至3000名新生儿中有1例,与不同程度的肺发育不全(PH)和持续性肺动脉高压(PPH)相关。尽管新生儿复苏和重症监护以及新的产后治疗策略取得了显著进展,但许多患有CDH的新生儿由于PH和PPH继发的严重呼吸衰竭,死亡率和发病率仍然很高。CDH以及相关的PH和PPH的发病机制尚不清楚。在此,我们旨在回顾膈肌和肺的发育,并将其与CDH中发现的异常相关联。