乳び心膜症に対する胸腔鏡手術治療例

A Case Report of Thoracoscopic Surgical Treatment for Primary Chylopericardium

　患者は16歳，女性．高校入学時健診で心拡大を指摘され当院受診．心エコーで大量の心嚢液貯留を認め心嚢ドレナージを施行，排液は乳白色に混濁し，白球数16,300／μl（リンパ球98％），中性脂肪2,077mg／dlと高値で，乳び心膜症と診断した．リンパ管造影を行ったところ，胸管は左静脈角流入部で閉塞し，網状側副路が認められ，その一部は心嚢に交通していた．中鎖脂肪食を試みたところ，心嚢液は黄色透明となり中性脂肪258mg／dlと低下したが，排液量は約200ml／日持続するため外科治療の適応と判断．胸腔鏡下に胸管クリッピングおよび心膜開窓術を施行したところ心嚢液貯留は改善した．乳び心膜症の報告は世界で約120例と極めて稀で，胸腔鏡による治療は過去に報告がなく，非開胸低侵襲手術として貴重と思われた．

A 16-year-old woman was referred to our hospital for evaluation of cardiomegaly (cardiothoracic ratio, 0.67), and a large amount of pericardial effusion was detected by echocardiography. She had no history of chest trau-ma or thoracic operations. The diagnosis of primary chylopericardium was established by an analysis of themilky-colored chylous fluid obtained by pericar-diocentesis, draining 900 ml of fluid. It contained 16,300/ white blood cells (lymphocytes 98%), high triglycer-ide (2,077 mg/di) and protein content (6.0 g/d1) . Lymph-angiography demonstrated that the thoracic duct was occluded before drainage into the left subclavian vein. There were a lot of small collaterals instead of a normal duct. Some of the collaterals communicated with the pericardial space. A medium chain triglyceride diet was instituted after diagnosis, the fluid became yellowish transparent and triglyceride decreased to 258 mg/dl but 200 ml of fluid had to be drained off daily, so we decided surgical treatment might be appropriate for the patient. Clipping of the thoracic duct and parietal pericar-diectomy were performed by thoracoscopy. Echocardio-graphy and CT were repeated to check for accumulation of the fluid, but chylous fluid was evacuated and reac-cumulation was prevented. Primary chylopericardium is an extremely rare entity (about 120 case reports in the world), and there is no report of surgical treatment by thoracoscopy so far. This report is the first successful case of thoracoscopic surgery for primary chylopericar-dium in the world.