Diffuse pulmonary hamartoangiomyomatosis associated with pneumothorax; a report of three cases.

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Three cases of diffuse pulmonary hamartoangiomyomatosis with pneumothorax are reported here, and the 56 cases reported to date in the Japanese literature are reviewed.<BR>Our three patients were young or middle aged women with repeated pneumothorax. Chest X-ray films showed granulo-reticular shadows. Two cases of them had diffuse cystic shadows on chest CT scans. Diffuse pulmonary hamartoangiomyomatosis was suspected. Thoracotomy was performed both to treat the pneumothorax and to obtain tissue for a histological diagnosis.<BR>The 56 patients reported in the Japanese literature until 1990 were all women aged 1758 (mean 33 years). In 39 of them (70 %) repeated episodes of pneumothorax had been noted.<BR>Pneumothorax associated with diffuse pulmonary hamartoangiomyomatosis requires thoracotomy for confirmation of the diagnosis and for treatment.
特定非営利活動法人日本呼吸器外科学会の論文