Abstract:
Congenital diaphragmatic hernia (CDH) is a rare developmental defect of the diaphragm, characterized by the herniation of abdominal contents into the chest, resulting in varying degrees of pulmonary hypoplasia and pulmonary hypertension. Significant advances in the prenatal diagnosis and identification of prognostic factors have resulted in the continued refinement of the approach to fetal therapies for CDH. In the postnatal period, protocolized approaches to lung-protective ventilation, nutrition, prevention of infection, and early aggressive management of pulmonary hypertension have led to improved outcomes in infants with CDH. Surgical repair of CDH is not urgent in most circumstances and can be delayed until the pulmonary status of the patient has stabilized. This article provides a comprehensive review of CDH, focusing on the complex pathophysiology, advances in prenatal diagnosis, fetal interventions, and optimal postnatal management of CDH.
摘要:
先天性膈疝(CDH)是一种罕见的膈肌发育缺陷,以腹部内容物突出到胸部为特征,导致不同程度的肺发育不全和肺动脉高压。产前诊断和预后因素鉴定的重大进展导致CDH胎儿治疗方法的不断完善。在产后,肺保护性通气的原生质方法,营养,预防感染,早期积极管理肺动脉高压可改善CDH婴儿的结局.CDH的手术修复在大多数情况下并不紧急,可以延迟到患者的肺部状态稳定为止。本文对CDH进行了全面的综述,专注于复杂的病理生理学,产前诊断的进展,胎儿干预,和CDH的最佳产后管理。