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先天性肺臓囊腫腺瘤狀畸形併發肺積膿:一病例報告

Congenital Cystic Adenomatoid Malformation of the Lung with Abscess Formation: A Case Report

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摘要


本文係報告一個一歲半女性幼童,在患支氣管性肺炎併發肺部積膿。病人胸部X光片顯示縱膈往左移位,右下肺葉有非均勻之軟組織浸潤影像,並出現空氣-水界面。經置胸管及抗生素ampicillin及gentamicin 治療後症狀改善,但胸部X光片卻始終未曾進步,多次血液及膿液抽取培養均爲陰性。經過胸部超音波、核醫灌流攝影((上標99m)Tc-MAA lung perfusion scintigram)及胸部電腦斷層切片檢查後,懷疑可能爲先天性肺臓囊腫疾病併發肺部積膿。在作完主動脈血管攝影後,將肺臓胚皮遺留(pulmonary sequestration)的可能性排除,便實施手術將右下肺葉病灶去除。患部外觀鼓脹且凹凸不平,部分呈充血狀。組織切面有大小不等的囊性空間,互相通連並有小梁散布其間。內部充滿膿液及黏液。此膿液經細菌培養後發現有鏈球菌Streptococcus viridans且對ampicillin及 gentamicin 均有敏感性。病理切片顯示細小支氣管發育不良,呈腺瘤狀增生,以織毛柱狀或立方上皮向囊內突出呈乳頭狀。囊壁間質中有彈性纖維及平滑肌增生,偶見黏液腺細胞存在。病人於手術後一週康復出院。先天性腫臓囊腫腺瘤狀畸形係一罕見先天性肺臓囊腫病。一般發病均較早,且發生次發性細菌感染的病例非常少見。筆者參考文獻,將其病因、病理、臨床症狀及X光片特徵和鑑別診斷作一討論以供參考。

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並列摘要


A one-and-a-half-year-old female had bronchopneumonia complicated by lung abscess for a few days. Chest roentgenogram revealed a heterogenous shadow of increased density with air-fluid level in the right lower lung field. After ampicillin and gentamicin treatment, the general condition improved; the chest X-ray abnormality showed no improvement. Congenital pulmonary cystic disease with lung abscess over the right lower lobe was a strong suspicion after taking chest echogram, (superscript 99m)Tc-MMA lung perfusion scintigram and chest CT scan. A pulmonary sequestration was ruled out by aortic angiogram. When a right lower lobe lobectomy was carried out, the external surface of the right lower lobe was found to be irregularly bulged, with local congestion. The cut surface demonstrated cystic cavities of various sizes within the pulmonary parenchyma and in communication with one another. A large amount of yellow mucopurulent and mucinous material was found in the cavities and small bronchi. Streptococcus viridans was isolated and found to be sensitive to ampicillin and gentamicin. Microscopically, the pulmonary cystic spaces were lined by ciliated columnar or cuboid epithelium with isolated mucus cells. Numerous papillary projections were noted arising from the lining epithelium. The cystic walls were surrounded by a fine network of elastic fibers, connective tissue and smooth muscle. The patient was discharged in good condition one week after the operation. Congenital cystic adenomatoid malformation is a rare congenital cystic lung disease. Early diagnosis and early operation are the most effective ways of treatment.

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