Lymphangioleiomyomatosis with Chylothorax-A Case Report

淋巴血管平滑肌瘤併發乳糜胸之病例報告及文獻回顧

龔昱中(Yu-Chung Kung) ； 陳培然(Pei-Jan Chen) ； 陳碧芳(Be-Fong Chen) ； 劉洪彰(Hung-Chang Liu)

《胸腔醫學》 21卷1期 (2006/02) Pp. 101-107

https://doi.org/10.29806/TM.200602.0016

摘要

肺淋巴血管平滑肌瘤是一個原因不明且罕見的疾病，侵犯對象以生育年齡女性為主。以肺實質及其他部位的異常平滑肌細胞增生為其特徵，並導致肺功能逐漸喪失。臨床表現有呼吸困難、胸痛、咳嗽、咳血、反覆性氣胸、乳糜胸，甚至呼吸衰竭，進而死亡。我們報告一位46歲女性罹患淋巴血管平滑肌瘤合併乳糜胸，出現咳嗽、呼吸困難，胸部x光片呈現左側肋膜腔積液，經胸腔穿刺術檢查確定是乳糜胸。而電腦斷層上則見到許多囊狀空泡的病變。經胸腔鏡手術與病理組織檢查後確定診斷為淋巴血管平滑肌瘤。乳糜胸則在肋膜沾黏術治療後，獲得改善並消失。

關鍵字

淋巴血管平滑肌瘤 ； 乳糜胸 ； 肋膜沾黏術

並列摘要

Pulmonary lymphangioleiomyomatosis (LAM) is an uncommon disorder of unknown etiology affecting women of childbearing age. It is characterized by the nonneoplastic proliferation of atypical smooth muscle cells within the lung parenchyma and elsewhere, leading to progressive loss of lung function. Clinical features include exertional dyspnea, cough, chest pain, recurrent pneumothorax, chylous pleural effusion, hemoptysis, eventual respiratory failure and, ultimately, death. We report a case of pulmonary LAM with chylothorax that developed in a 46-year-old woman. This patient suffered from cough and exertional dyspnea, and the chest X-ray showed left pleural effusion. Thoracocentesis demonstrated chylous effusion. The chest computed tomography (CT) scan revealed multiple cystic lesions. The clinical diagnosis, based on histological examinations with biopsy specimens, was pulmonary LAM. The chylothorax resolved after pleurodesis.

並列關鍵字

lymphangioleiomyomatosis ； chylothorax ； pleurodesis