Straight Back症候群を伴った先天性左側心膜全欠損症の1例

A Case of Complete Absence of the Left Pericardium Coexisting with Straight Back Syndrome

　症例は30歳の女性．1989（平成元年）12月の検診の胸部X線写真で心陰影の拡大を指摘され，当科を受診した．正面像で左2,3弓の突出と心陰影の著明な左方偏位を，側面像でStraight Back syndrome（以下SBSと省略する）を認めた．心エコーでは，心室中隔の奇異性運動，左室後壁の過剰運動および右室腔の拡大を認めた．以上よりSBSを合併した先天性左側心膜全欠損症が疑われた．人工気縦隔術後のCTとMRIで主肺動脈の突出，肺組織の縦隔組織内陥入に加え心嚢気腫を認め確診した．本例は無症状のため，無治療にて経過観察中である．我々の検索した範囲では，SBSと先天性心膜欠損症の合併の報告はなく，本例が第1例目と思われた．先天性心膜欠損症の診断に，従来の人工気胸術と同様，人工気縦隔術が有用であった．

A case of complete absence of the left pericardium coexisting with straight back syndrome in a 30-year-old female is presented. She had been asymptomatic, but was referred to our hospital because of an abnormal finding on a chest X-ray obtained during a routine physical examination. The posteroanterior view of the chest X-ray showed displacement of the right and left cardiac border to the left and bulging of the left cardiac border. The lateral view showed loss of normal thoracic curvature and also demonstrated the straight back syn-drome.