Kane T D, Greenberg J M, Bove K E, Warner B W
Division of Pediatric Surgery, Children's Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA.
Pediatr Surg Int. 1998 Nov;14(1-2):89-91. doi: 10.1007/s003830050445.
Respiratory failure in the neonate that is refractory to maximal medical management is a frequent indication for extracorporeal life support (ECLS). Alveolar capillary dysplasia with misalignment of the pulmonary veins is an irreversible cause of respiratory failure that cannot be diagnosed on clinical grounds alone and would not be expected to respond to ECLS therapy. A recent experience with a patient prompted us to review the literature regarding this condition for the purpose of identifying factors suggestive of this diagnosis. This condition should be considered in neonates with presumed pulmonary hypertension who cannot be weaned from ECLS. If the diagnosis is made by antemortem open-lung biopsy, a costly, protracted, and unnecessary continued course of ECLS may be avoided.
对于采用最大程度药物治疗仍无效的新生儿呼吸衰竭，体外生命支持（ECLS）是常见的治疗手段。肺泡毛细血管发育异常并伴有肺静脉错位是一种不可逆的呼吸衰竭病因，仅靠临床症状无法诊断，且预计对ECLS治疗无反应。最近一名患者的经历促使我们查阅有关该病症的文献，以确定提示该诊断的因素。对于无法从ECLS撤机的疑似肺动脉高压新生儿，应考虑这种病症（肺泡毛细血管发育异常并伴有肺静脉错位）。如果通过生前开胸肺活检确诊，或许可以避免代价高昂、旷日持久且不必要的ECLS持续治疗过程。
