布加综合征9例临床分析及诊治经验

Clinical analysis and experience in diagnosis and treatment of 9 cases of Budd-Chiari syndrome

目的分析布加综合征(Budd-Chiari syndrome,BCS)的临床特点及诊治经验,为临床诊断治疗提供参考。方法收集整理上海中医药大学附属曙光医院2017至2021年9例布加综合征患者的病史资料,对其主要临床表现、肝功能、影像学检查结果、误诊情况等进行归纳整理。结果9例BCS患者临床表现以肝/脾肿大、食管静脉曲张、腹胀、腹水、胁痛、腹壁静脉曲张、上消化道出血、胁胀为主;肝功能无明显异常;影像学检查结果以下腔静脉阻塞/狭窄者为主;临床误诊率较高(7/9),常被误诊为肝硬化。结论布加综合征临床表现复杂多变,易漏诊误诊;诊断除了血管影像学检查,肝活检病理也具有重要价值;结合益气活血的中药治疗该病可获良效。

Objective To analyze the clinical features,experience in diagnosis and treatment of Budd-Chiari syndrome(BCS),and to provide reference for clinical diagnosis and treatment.Methods The clinical data of 9 patients with Budd-Chiari syndrome in Shuguang Hospital affiliated to Shanghai University of traditional Chinese Medicine from 2017 to 2021 were collected,and their main clinical manifestations,liver function,imaging results and misdiagnosis situation were summarized.Results The main clinical manifestations of 9 patients with BCS were hepatomegaly/splenomegaly,esophageal varices,abdominal distension,ascites,costal pain,abdominal wall varices,upper gastrointestinal bleeding and distending pain in hypochondrium;no obvious abnormality of liver function;imaging examination showed that the patients with inferior vena cava obstruction/stenosis were dominant;the clinical misdiagnosis rate was high(77.8%)and was often misdiagnosed as liver cirrhosis.Conclusion The clinical manifestations of BCS are complex and changeable,and it is easy to be missed and misdiagnosed;Besides vascular imaging examination,liver biopsy pathology also has important value;good effect can be obtained in the treatment of this disease when combined with the traditional Chinese medicine of invigorating qi and activating blood.