Two Cases of Bilateral Diffuse Cystic Lesion

양측성 미만성 소낭포성 병변 2예

Lymphangioleiomyomatosis(LAM) is a rare disease of unknown etiology that occurs mainly in woman in her reproductive age. We recently experienced two cases of bilateral diffuse cystic lesion of the lung on chest X-ray and HRCT. The first case, a 26-year-old female, who had been diagnosed with tuberous sclerosis by the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to a recently developed hemoptysis. Chest PA showed diffuse ground-glass opacity with radiolucent cystic lesions of various sizes on both lung fields. HRCT showed innumerable small cystic lesions with suspicious diffuse ground-glass opacity on both lung fields The second case, a 30-year-old fema1e was admitted due to dyspnea and spewing of blood-tinged sputum for 2 weeks, shortly after delivery. Chest PA showed diffuse reticular and ground-glass opacities on both lung field. HRCT showed multiple well-defined and relatively uniform size air cysts with a uniform wall thickness on entire both lung fields, with small amount of right pleural effusion. By thoracoscopic lung biopsy she was diagnosed with pulmonary lymphangioleiomyomatosis. We report these cases with a brief review of the literatures.

저자들은 양측성 신혈관근지방종, 경련, 지능저하 및 피지선종을 동반한 결절성 경화증으로 진단된 26세 여자 환자에서 객혈을 주증상으로 하여 흉부 X-선 및 고해상도 흉부전산화단층 촬영상 폐림프관평활근종증이 의심되었던 1예와 분만후 호흡곤란과 객혈을 주소로 내원한 30세 여자 환자에서 흉부 X-선 촬영, 고해상도 흉부전산화단층 촬영 및 폐생검을 통해 확진한 폐임파관 평활근종증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.