肺泡蛋白質沉積症進行全肺部沖洗術之病例報告

pulmonary Alveolar Proteinosis Undergoing Whole Lung Lavage in South Taiwan-- A Case Report

肺泡蛋白質沉積症在台灣是非常罕見的疾病，根據文獻上之記載，盛行率大約為兩百萬分之一，男與女的比例則為三比一左右。肺泡蛋白質沉積症的病理組織學呈現肺泡中充滿蛋白質類的物質，它臨床上被區分為原發性及繼發性兩種。在從前，這個疾病要靠開肺取樣術才能診斷。但自從肺葉沖洗術被廣為接受後，用開肺取樣術來診斷肺泡蛋白質沉積症已經比較不常見了。而至於對這個疾病最有效也最正確的治療方式則是全肺沖洗術。治療性的全肺沖洗術必須經由全身麻醉及置放雙口徑氣管內管，而這個治療過程常碰到三個常見的問題：1.低血氧2.心肺循環問題3.無法適切置放雙口徑氣管內管於正確位置。我們在此報告一個高雄榮民總醫院進行全肺沖洗術的病例，這個病人曾有肺結核病史，並經由開肺取樣術證實為肺泡蛋白質沉積症。因為她的肺部功能已經無法應付日常生活所需，所以決定接受全肺沖洗術。第一次左肺的沖洗結果非常好，但是第二次要進行另一邊時，卻因為氣管狹窄，無法放入雙口徑氣管內管而作罷。我們用這個病例來討論肺泡蛋白質沉積症以及全肺沖洗術。

Pulmonary alveolar proteinosis (PAP) is rarely seen in Taiwan. According to the literature the incidence is one in two million with a male to female ratio of 3 to 1. The pathohistology of PAP is associated with the filling of the alveoli by a proteinaceous material. PAP can be divided into primary and secondary forms. PAP was once diagnosed by open lung biopsy, but since the introduction of bronchoalveolar lavage, open lung biopsy is less common now for the diagnosis of PAP. The most definite and effective therapy for PAP is whole lung lavage (WLL). Therapeutic WLL is performed under one-lung general anesthesia with a double lumen tube (DLT). This procedure is associated with three problems: 1. hypoxia. 2. circulatory disturbance. 3. difficulty in positioning the DLT. We present a case undergoing WLL in our hospital who had contracted pulmonary TB was diagnosed as a case of PAP by open lung biopsy. She was convinced to receive WLL because of intolerance of daily activity. The result of the left lung lavage was excellent, but the attempt on the other lung at a later time was cancelled because of difficulty in positioning the DLT due to tracheal stenosis. Here we would like to give an account of our management and discuss about PAP and WLL after literature review.