Buss Michael, Williams Garry, Dilley Anthony, Jones Owen
Department of Cardiology, Sydney Children's Hospital Randwick, University of New South Wales, Sydney 2031, Australia.
J Pediatr Surg. 2006 Apr;41(4):e9-11. doi: 10.1016/j.jpedsurg.2006.01.003.
Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50%. Ipsilateral pulmonary hypoplasia, increased pulmonary vascular resistance, and potential cardiac failure complicate early postnatal life. Surgical correction can either be performed on the first day of life or be deferred to a time after stabilization of the infant. Our patient presented with a left-sided Bochdalek's hernia containing large and small bowel. She required intubation and resuscitation on day 1 of life, and surgical repair had to be postponed. Further respiratory deterioration required commencement of inhaled nitric oxide and high-frequency ventilation. Pulmonary artery pressure rose to suprasystemic level. Closure of the ductus arteriosus on day 8 resulted in imminent right-sided heart failure. Commencement of alprostadil (prostaglandin E1) reopened the ductus and stabilized the patient. Surgical repair was successful 3 days later. Alprostadil should be considered as an important component of therapy in severe cases of congenital diaphragmatic hernia, where deterioration of right-sided heart function occurs.
先天性膈疝在儿童期是一种罕见疾病，死亡率高达30%至50%。同侧肺发育不全、肺血管阻力增加以及潜在的心力衰竭使出生后早期生活变得复杂。手术矫正可在出生当天进行，也可推迟到婴儿病情稳定之后。我们的患者表现为左侧Bochdalek疝，疝内容物为大小肠。她在出生第1天需要插管和复苏，手术修复不得不推迟。进一步的呼吸恶化需要开始吸入一氧化氮和高频通气。肺动脉压力升至高于体循环水平。出生第8天动脉导管关闭导致即将发生右侧心力衰竭。开始使用前列地尔（前列腺素E1）使动脉导管重新开放并使患者病情稳定。3天后手术修复成功。在先天性膈疝严重病例中，若出现右侧心功能恶化，应将前列地尔视为治疗的重要组成部分。
