Objective To analyze the clinical characteristics of idiopathic chylopericardium (CP) and improve the diagnostic and therapeutic level of this rare disease. Methods The clinic and follow-up data of one patient with idiopathic CP were analyzed. Then we retrieved MEDLINE database and CHINAINFO (Wanfang) database for all reported cases of idiopathic CP in the past 55 years (from January 1957 to June 2012) , of which the clinical manifestation, etiology, diagnosis and treatment were conducted. Results A total of 89 reported cases of idiopathic CP were identified through a systematic literature search. There was no gender bias in the incidence of CP. Age at diagnosis varied from 0 to 68 years with a mean age of ( 25. 5 ± 15. 8 ) years. The common clinical manifestations included dyspnea ( 53. 9% ) , cough (25. 8% ) ,chest pain or epigastric pain (6. 7% ) , fever (6. 7% ) , palpitation (3. 4% ) , edema (3. 4% ), fatigue (1. 1% ) , syncope or tamponade also could be the first symptom. The diagnosis was made with positive chylus test and high triglyceride level ( > 12.9 mmol/L ) of pericardial effusion. Lymphoscintigraphy and lymphangiography were helpful to find leak site and etiology of idiopathic CP, the positive rates of these two methods were 80.8% and 74. 1% , respectively. The common causes were occlusion or dysplasia of thoracic, lymphangiectasis, other deformity of lymphatic vessels. Conservative therapy included low fat or medium chain triglyceride ( MCT) diet and total parenteral nutrition (TPN) , but failed in 70. 8% of the patients, in whom subsequent surgery was necessary. Thoracic duct ligation with creation of a pericardial window was the most common and effective surgical procedure, of which the successful rate arrived 92. 4% . Follow-up was reported in 70 of the cases: no recurrence in 88. 6% of patients, 10% were uncured including one patient developing constrictive pericarditis 4 weeks after surgery. All patients survived during follow-up. Conclusions Idiopathic chylopericardium is a rare disease and always presents nonspecific clinical manifestations. Correct diagnosis can be made with detailed test of pericardial effusion. Lymphoscintigraphy and lymphangiography are helpful to find leak site and etiology. Surgical management is the most successful method with a favorable prognosis.%目的 介绍1例特发性乳糜心包积液并进行文献复习,以提高对该病的认识.方法 对1例特发性乳糜心包积液患者的诊治过程进行分析,并检索Medline及万方中文数据库近55年国内外报道的特发性乳糜心包积液病例,分析总结其临床表现、病因、诊断和治疗的特点.结果 共检出89例特发性乳糜性心包积液的患者.男性49例、女性40例,患病无性别差异,年龄6周至68岁,平均(25.5±15.8)岁.其中无症状者22例(24.7％).常见临床表现包括:呼吸困难48例(53.9％),咳嗽23例(25.8％),胸背痛或上腹痛6例(6.7％),发热6例(6.7％),心慌3例(3.4％),水肿3例(3.4％),乏力1例(1.1％),并可以晕厥、心脏压塞为首发症状.多数通过心包积液乳糜试验阳性、心包积液三酰甘油＞ 12.9 mmol/L而诊断,淋巴管核素显像以及淋巴管造影有助于发现淋巴漏出部位及病因,本研究中阳性率分别为80.8％ (21/26)和74.1％(20/27).常见病因包括胸导管闭塞或发育不良、胸导管/淋巴管异常扩张,其他局部异常和发育异常等.特发性乳糜心包积液保守治疗包括低脂饮食、中链脂肪酸饮食、全胃肠外营养等,但效果差,70.8％(46/65)的患者转而行手术治疗.最常用、有效的术式为胸导管低位结扎及心包开窗术,治愈率可达92.4％ (61/66).89例患者中70例有随访资料:88.6％(62/70)无复发,10％ (7/70)未愈,其中1例发生缩窄性心包炎.随访期内无死亡病例.结论 特发性乳糜心包积液发病率很低,临床表现缺乏特异性,心包积液检查可以确诊.淋巴管影像学检查有助于确定病变部位及病因.手术是有效的治疗方法,预后良好.
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