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- Akagawa Shinobu
- Department of Internal Medicine, Ohme-shi General Hospital
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- Sakamoto Yasumi
- Department of Internal Medicine, Ohme-shi General Hospital
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- Ozaki Toku
- Department of Internal Medicine, Surgery, Ohme-shi General Hospital
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- Uda Tohhei
- Department of Internal Medicine, Surgery, Ohme-shi General Hospital
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- Kobayashi Ichizou
- Department of Internal Medicine, Pathology, Ohme-shi General Hospital
Bibliographic Information
- Other Title
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- 多発性気しゅ性のう胞を合併したＲｅｃｋｌｉｎｇｈａｕｓｅｎ病の１例
Abstract
A 46 year-old male was admitted because of recurrent right spontaneous pneumothorax after an interval of 8 years. He had multiple cutaneous neurofibromas and cafe-au-lait spots, and a diagnosis of Recklinghausen's disease was made. Chest X-ray film showed collapse of the right lung. Contrast thoracography revealed some thumb sized cysts in the lung apex, and cobblestone appearance on the lung surface. At thoracoscopy numerous cysts were seen all over the lung. Open lung biopsy and resection of large cysts was performed. Microscopically the cysts were bullae and there were emphysematous changes arround them, therefore the pathological diagnosis was emphysematous bullae. Six months after operation, chest CT revealed multiple peripheral lung cysts bilaterally, but respiratory function tests showed no abnormality.<br>Although Recklinghausen's disease associated with multiple lung cysts is rare in Japan, some cases were reported in international journals. Many of them had interstitial pneumonitis and it has been considered that alveoli were destroyed by fibrosis and accumulated to form bullae. However in our case, emphysematous changes with neither interstitial pneumonitis nor airway obstruction were seen. Therefore we consider that these findings are very interesting and may have some relation to Recklinghausen's disease.
Journal
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- The Japanese journal of thoracic diseases
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The Japanese journal of thoracic diseases 23 (7), 819-823, 1985
The Japanese Respiratory Society