Chandrasekharan Praveen Kumar, Rawat Munmun, Madappa Rajeshwari, Rothstein David H, Lakshminrusimha Satyan
Department of Pediatrics, Women and Children's Hospital of Buffalo, Buffalo, NY USA.
SIGMA Hospital, Mysore, India.
Matern Health Neonatol Perinatol. 2017 Mar 11;3:6. doi: 10.1186/s40748-017-0045-1. eCollection 2017.
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes. Delivery of infants with CDH is recommended close to term gestation. Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required. The main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Although inhaled nitric oxide is not approved by FDA for the treatment of PPHN induced by CDH, it is commonly used. Extracorporeal membrane oxygenation (ECMO) is typically considered after failure of conventional medical management for infants ≥ 34 weeks' gestation or with weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors such as prematurity, associated abnormalities, severity of PPHN, type of repair and need for ECMO can affect the survival of an infant with CDH. With advances in the management of CDH, the overall survival has improved and has been reported to be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
先天性膈疝（CDH）是一种以膈肌缺损为特征的疾病，膈肌缺损导致腹腔内容物突入胸腔，干扰肺的正常发育。缺损范围可从后肌缘的小孔到膈肌完全缺失。CDH的病理生理学是肺发育不全和不成熟，伴有新生儿持续性肺动脉高压（PPHN）和心脏功能障碍。通过超声对肺头比（LHR）和肝脏位置进行产前评估，以诊断和预测预后。建议在接近足月妊娠时分娩患有CDH的婴儿。出生时的立即处理包括肠道减压，必要时避免面罩通气和放置气管内导管。处理的主要重点包括轻柔通气、血流动力学监测和肺动脉高压治疗，随后进行手术。尽管吸入一氧化氮未被美国食品药品监督管理局（FDA）批准用于治疗由CDH引起的PPHN，但它仍被广泛使用。对于孕周≥34周或体重>2 kg且无相关严重致命异常的患有CDH的婴儿，体外膜肺氧合（ECMO）通常在传统药物治疗失败后考虑使用。多种因素，如早产、相关异常、PPHN的严重程度、修复类型和对ECMO的需求，均可影响患有CDH的婴儿的生存。随着CDH管理的进展，总体生存率有所提高，据报道，非ECMO治疗的婴儿生存率为70%-90%，接受ECMO治疗的婴儿生存率高达50%。
