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先天性膈疝

Congenital diaphragmatic hernia.

作者信息

Arora M, Bajpai M, Soni T R, Prasad T R

机构信息

Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi.

出版信息

Indian J Pediatr. 2000 Sep;67(9):665-70. doi: 10.1007/BF02762181.

Abstract

Over the last two decades there has been a constant improvement in the understanding of the pathophysiology of Congenital Diaphragmatic Hernia (CDH) and its management. However, the ideal treatment remains elusive. The earlier management strategy of immediate surgery is replaced by the principle of physiological stabilisation and delayed surgery. Conventional mechanical ventilatory techniques, with high pressures and hyperventilation to reverse ductal shunting and cause alkalinization, are being questioned because of the risks of barotrauma and consequent broncho-pulmonary dysplasia. It has also been shown that paralysis with pancuronium bromide for patients on conventional mechanical ventilation results in increased incidence of sensorineural hearing loss in childhood survivors of CDH. With the introduction of the concept of permissive hypercapnia and high frequency oscillation ventilation, the complications of pulmonary barotrauma are circumvented. Although ECMO therapy is invasive, yet has improved survival by about 15% independently, especially in critically ill infants who have the predictive mortality rate of more than 80%. Further insights into the pathophysiology of CDH and the introduction of less invasive therapeutic techniques in the form of high frequency oscillation ventilation, inhalation nitric oxide, surfactant, and perfluorocarbon liquid ventilation may even make the need for ECMO redundant.

摘要

在过去二十年里,人们对先天性膈疝(CDH)的病理生理学及其治疗的理解不断进步。然而,理想的治疗方法仍然难以捉摸。早期立即手术的治疗策略已被生理稳定和延迟手术的原则所取代。传统的机械通气技术,通过高压力和过度通气来逆转导管分流并导致碱化,由于存在气压伤和随之而来的支气管肺发育不良的风险而受到质疑。研究还表明,接受传统机械通气的患者使用潘库溴铵麻痹会导致CDH儿童幸存者感音神经性听力损失的发生率增加。随着允许性高碳酸血症和高频振荡通气概念的引入,肺气压伤的并发症得以避免。尽管体外膜肺氧合(ECMO)治疗具有侵入性,但它独立地将生存率提高了约15%,特别是在预测死亡率超过80%的重症婴儿中。对CDH病理生理学的进一步深入了解以及以高频振荡通气、吸入一氧化氮、表面活性剂和全氟化碳液体通气等形式引入的侵入性较小的治疗技术,甚至可能使ECMO不再必要。

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