Pulmonary Alveolar Proteinosis Treated with Multiple Selective Lobar Lavage by Fiberoptic Bronchoscopy with Non-Invasive Ventilation under Procedural Sedation: A Case Report

非侵入性正壓通氣及鎮靜下多次支氣管內視鏡選擇性肺葉沖洗術治療肺泡蛋白質沉著症－病例報告

林倡葦(Chang-Wei Lin) ； 林定佑(Ting-Yu Lin) ； 羅友倫(Yu-Lun Lo)

《胸腔醫學》 30卷2期 (2015/04) Pp. 111-117

摘要

肺泡蛋白質沉著症（pulmonary alveolar proteinosis, PAP）是少見的疾病。全肺沖洗術（whole lung lavage, WLL）仍是現今標準的治療方式。支氣管內視鏡選擇性肺葉沖洗術是另一個選擇的方式。但仍有些病人需插管及麻醉下進行此方式。我們報告一個蛋白質沉著症患者在非侵入性正壓通氣及鎮靜下，使用支氣管內視鏡作多次肺葉沖洗術的經驗。因為病人拒絕插管及無法忍受支氣管內視鏡所造成的不舒服，病患無法執行全肺沖洗術。我們根據電腦斷層選擇受影響嚴重的肺葉，並在設計過的非侵入性正壓通氣面罩及鎮靜使用下，分別進行左下肺葉及右中下肺葉的支氣管內視鏡肺葉沖洗術。每次以生理食鹽水從支氣管鏡內注入及洗出。病患對執行過程忍受度良好。我們的經驗顯示，支氣管內視鏡肺葉沖洗術在非侵入性正壓通氣及鎮靜使用下，對無法容忍全肺沖洗術的蛋白質沉著症患者或無法忍受支氣管內視鏡所造成的不舒服，是有效，安全的治療方式且可以改善病人生活品質，甚至可在門診執行以減少醫療花費。

關鍵字

肺泡蛋白質沉著症 ； 支氣管內視鏡肺葉沖洗術 ； 非侵入性正壓通氣 ； 處置過程鎮靜

並列摘要

Pulmonary alveolar proteinosis (PAP) is a rare disease. The current standard treatment for PAP is whole-lung lavage (WLL). An alternative treatment is selective lobar lavage via fiberoptic bronchoscopy (FOB). However, some patients still require intubation with sedation for this procedure. Herein, we report a case of multiple selective lobar lavage via FOB with non-invasive positive pressure ventilation (NIPPV) support under procedural sedation for the treatment of a patient with PAP diagnosed by wedged resection via video-assisted thoracoscopic surgery. As the patient refused to undergo WLL and could not tolerate FOB, we performed lobar lavage by FOB for the affected segment, according to chest CT images, with designed NIPPV and procedural sedation. This new technique is safe and effective, improves patients' quality of life, and can possibly be performed at outpatient clinics to decrease treatment cost.

並列關鍵字

pulmonary alveolar proteinosis ； bronchofiberscopic lobar lavage ； non-invasive positive pressure ventilation ； procedural sedation