• Journal of Chest Surgery
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• v.26 no.2
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• pp.160-162
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• 1993

Pulmonary lymphangiomyomatosis is rare but extremely interesting condition caused by progressive widespread smooth muscle proliferation in the perilymphatic regions throughout the lungs. The patient was a 25-year-old female. She had angiofibromas in the face, and angiomyolipoma in the left kidney which was removed 5 years earlier. Three years ago she started having severe dyspnea with bilateral pneumothoraces. Treatment was initiated with bilateral closed thoracotomies followed by open thoracotomy through median sternotomy and lung biopsy, which revealed the diagnosis of pulmonary lymphangiomyomatosis. Recurrence of pneumothorax was treated by repeated chemical pleurodesis with tetracycline. She has been in good condition during medroxyprogesterone administration for 3 years.

• Korean Journal of Radiology
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• v.1 no.2
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• pp.84-90
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• 2000

Objective: To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. Materials and Methods: The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. Results: All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. Conclusion: In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary.