The current patient was a 22-year-old woman with dyspnea on exertion that had exacerbated over the past two to three years. Chest X-ray indicated pneumothorax in the right lung, and chest computed tomography showed diffuse microcysts in both lungs. The patient was diagnosed with lymphangiomyomatosis based on pleural biopsy by intrathoracic endoscopy. Systemic testing revealed complications of tuberous sclerosis and abdominal aortic aneyrysm, and a prosthetic graft replacement was perfomed following pneumothorax treatment. Aortic wall tissue images showed hamartomatous changes in smooth muscle tissue, similar to the pulmonary lesion, and this was thought to have caused vascular fragility and aneurysmal change.
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