体外循环膜氧合支持下全肺灌洗治疗肺泡蛋白沉积症一例并文献复习

目的提高对肺泡蛋白沉积症(PAP)患者肺灌洗治疗过程中低氧血症危险性的认识。方法介绍1例经病理证实的PAP患者体外循环膜氧合下的全肺灌洗治疗过程，并复习相关文献。结果57岁男性患者，因咳嗽和进行性呼吸困难12个月入院，动脉血氧分压(PaO2)为46mmHg(1mmHg=0．133kPa)，脉搏容积血氧饱和度(SpO2)85％～88％。胸部CT、经纤维支气管镜支气管肺泡灌洗和肺活检病理检查符合PAP。在手术室常规静脉全麻后，经口腔插入双腔管，分隔为通气肺和灌洗肺。两肺机械通气SpO2为80％～90％；当右侧单侧肺机械通气SpO2为68％～80％。于右股动脉和右股静脉插管，建立体外循环通道，开始静脉-动脉体外循环膜氧合(ECMO)支持，右侧单侧肺机械通气SpO2为89％～97％。左侧肺用总计20800ml生理盐水灌洗，灌洗期间SpO2为80％～94％；灌洗后，患者无呼吸困难。28d后未用ECMO完成右肺灌洗。1个月后复查CT示双肺浸润影基本消失。结论当PAP患者全肺灌洗操作前出现顽固的低氧血症，应考虑使用ECMO支持，以避免患者在全肺灌洗治疗中的危险低氧血症。

Pulmonary alveolar proteinosis treated with whole-lung lavage utilizing extracorporeal membrane oxygenation：a case report and review

Objective To improve the treatment of severe hypoxaemia in patients with pulmonary alveolar proteinosis(PAP). Methods The clinical data of a patient with pathologically proven PAP treated with whole-lung lavage utilizing extracorporeal membrane oxygenation(ECMO)were described and the literature was reviewed. Results This 57-year-old man was admitted because of cough and progressive dyspnea for 12 months. His PaO_2 was 46 mm Hg(1 mm Hg=0.133 kPa) and saturation of pulse oximeter(SpO_2) was from 85% to 88% with oxygen 5 L/min by nasal cannula. His chest CT,bronchoscopy with bronchoalveolar lavage and transbronchial biopsies were consistent with PAP. Whole-lung lavage was performed in the operation room under general anesthesia. A double-lumen tube was intubated in order to selectively ventilate and lavage a single lung independently. During mechanical ventilation for both lungs,the SpO_2 was from 80% to 90%,but when a single right lung ventilation was tried,the SpO_2(from 68%to 80%) dropped significantly. To ensure adequate oxygen supply during lavage,a veno-arterial ECMO was set up by inserting catheters percutaneously into the right femoral artery and right femoral vein respectively. Then the SpO_2 improved,from 89% to 97% during single right lung ventilation. The left lung was lavaged with a total of 20.8 L of normal saline. The SpO_2 ranged from 80% to 94% during the lavage. After the lavage,the patient no longer experienced shortness of breath. Then 28 days later the right lung was lavaged without the aid of ECMO. A month after the second lavage,his chest CT showed marked improvement in infiltrates of both lungs. Conclusion When a patient with PAP has refractory hypoxemia prior to the lavage procedure,ECMO should be considered in order to avoid severe hypoxaemia with fatal consequences during lavage.