IgG4硬化性胆管炎误诊为肝门胆管癌及术后消化道出血MDT讨论

IgG4 sclerosing cholangitis misdiagnosed as hilar cholangiocarcinoma and MDT discussion on postoperative gastrointestinal hemorrhage

目的探讨免疫球蛋白G4硬化性胆管炎(IgG4SC)误诊为肝门胆管癌以及术后胃十二指肠假性动脉瘤破裂的临床表现、诊断和治疗措施,以提高对该病的认识及合理诊治。方法回顾性分析四川大学华西医院诊治的1例IgG4SC误诊为肝门胆管癌且术后消化道出血患者的临床资料。结果该例患者以肝门胆管癌收入院,术前影像学检查表现为典型的肝门胆管癌影像学特征,遂行肝门胆管癌根治术,术后病理结果诊断为IgG4SC,补查血清IgG4仅轻度升高。术后1个月突发呕血,动脉造影显示胃十二指肠假性动脉瘤破裂出血,经栓塞治疗后痊愈。结论术前诊断IgG4SC较为困难,特别应与肝门胆管癌鉴别,可避免不必要的手术。胃十二指肠假性动脉瘤形成的原因主要是胃十二指肠动脉术中被损伤,肝胆疾病手术行动脉骨骼化过程中需注意动脉保护。临床怀疑假性动脉瘤形成时,应尽早行血管介入诊断并同时行栓塞治疗。

Objective To explore clinical manifestation, diagnosis and treatment of IgG4 sclerosing cholangitis developed postoperative gastroduodenal hemorrhage, so as to improve awareness and treatment of this disease. Method The clinical data of a case of IgG4 sclerosing cholangitis misdiagnosed as the hilar cholangiocarcinoma which developed postoperative gastrointestinal hemorrhage in this hospital were analyzed retrospectively. Results This patient was misdiagnosed as the hilar cholangiocarcinoma and accepted the radical resection, while the postoperative pathology proved to be the IgG4 sclerosing cholangitis. One month later, the patient developed the acute gastrointestinal hemorrhage and it was resolved by using the endovascular embolization. Conclusions Preoperative distinguishing IgG4 sclerosing cholangitis from hilar cholangiocarcinoma can avoid an unnecessary surgery. Endovascular intervention is both a useful measure of diagnosis and treatment for gastroduodenal pseudoaneurysm. Attention should be paid to arterial protection during process of arterial osteogenesis in hepatobiliary operation.