NEJM：西京医院报道表现为腹壁静脉曲张的布加氏综合症一例

此病例发表在今天的NEJM上，患者表现为腹壁静脉曲张的布加氏综合症。

57岁男，双下肢间歇性水肿5年，因右侧腹壁和胸壁静脉曲张入院（图A）。过去18年里，静脉曲张的大小和数量逐渐增加。

实验室检查发现以下异常结果：

总胆红素44.0umol/L（正常值3.4-20.5umol/L）、白蛋白28.2g/L（正常值40-55g/L)、凝血酶原时间17.1s（正常9.8-12s）。腹部增强CT发现下腔静脉的肝上、肝后静脉血栓形成，右侧腹壁多发静脉曲张。未发现腹水。腹部超声及下腔静脉血管造影进一步证实下腔静脉血栓形成，因此确诊为布加氏综合征伴下腔静脉血栓形成。

随后进行了经皮经腔球囊导管扩张及支架置入术，下腔静脉成功再通。患者接受了为期6个月的抗凝治疗后，临床反应良好。术后6个月，腹壁静脉曲张出现显著改善（图B）。

英文阅读：

A 57-year-old man with a 5-year history of intermittent edema of the legs was admitted to the hospital because of varices in the right abdominal and chest walls (Panel A). The varices had been gradually increasing in size and number over a period of 18 years. Abnormal results of laboratory tests included a total bilirubin level of 44.0 μmol per liter (2.6 mg per deciliter; normal range, 3.4 to 20.5 μmol per liter [0.2 to 1.2 mg per deciliter]), a serum albumin level of 28.2 g per liter (normal range, 40 to 55), and a prothrombin time of 17.1 seconds (normal range, 9.8 to 12.1). Contrast-enhanced computed tomography of the abdomen revealed a thrombus in suprahepatic and posthepatic portions of the inferior vena cava and multiple varices in the right abdominal wall. No ascites was found. Thrombosis of the inferior vena cava was further confirmed by abdominal ultrasonography and angiography of the inferior vena cava. Thus, a diagnosis of the Budd–Chiari syndrome with thrombosis of the inferior vena cava was made. Subsequent percutaneous recanalization of the inferior vena cava by means of balloon angioplasty and stent placement was successful; after the percutaneous recanalization, the patient received anticoagulation therapy for 6 months and had an excellent clinical response. At the sixth postoperative month, the abdominal-wall varices had improved markedly (Panel B).

Xingshun Qi, M.D., and Guohong Han, M.D.Abdominal-Wall Varices in the Budd–Chiari Syndrome.N Engl J Med 2014; 370:1829May 8, 2014DOI: 10.1056/NEJMicm1308567