気胸手術を契機に診断したBirt-Hogg-Dubé症候群の3例

Birt-Hogg-Dubé Syndrome Diagnosed with Surgery；Report of Three Cases

はじめに　Birt-Hogg-Dubé（BHD）症候群は1977年にBirtらが最初に報告した顔面頭頸部皮疹，多発肺囊胞，腎腫瘍を3徴とする常染色体優性遺伝の疾患である．本疾患の肺病変の特徴としては囊胞が肺尖部だけでなく中下葉や葉間部に散在性に認められることが多く，気胸の発症頻度は非罹患者と比較して約50倍と報告されている1）．われわれは3例のBHD症候群を経験したので報告する．

Birt-Hogg-Dubé （BHD） syndrome is an autosomal and predominantly inherited disorder. We report 3 cases of BHD syndrome.

Case 1：A 24-year-old man was admitted to our hospital due to left-sided spontaneous pneumothorax. He had a previous drainage history of right-sided spontaneous pneumothorax. In operation, we resected the cyst at segment 8 of the left lung and covered the lung using polyglycolic acid （PGA） sheet.

Case 2：A 47-year-old man was admitted to our hospital due to right-sided spontaneous pneumothorax. He had a previous surgical history of right-sided spontaneous pneumothorax and left-sided spontaneous pneumothorax. In operation, we resected the cyst at segment 8 of the right lung and covered the lung using fibrin glue-coated collagen fleece.

Case 3：A 60-year-old man was admitted to our hospital due to left-sided spontaneous pneumothorax. He had 2 times of previous drainage history of left-sided spontaneous pneumothorax. In operation, we resected the cysts at lingular division of the lung and covered the lung using PGA sheet.

On genetic analysis, all 3 cases were diagnosed with the BHD syndrome.