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目的探讨小儿先天性胆管扩张症的临床发病特点和误诊原因。方法分析近15a来经手术及病理检查确诊的小儿先天性胆管扩张症26例的临床表现、实验检查及误诊情况。结果具有“腹块、腹痛、黄症”三联松典型表现者4例，可有肝转氨酶异常，胆红素升高，血尿淀粉酶升高，ALP、γ－GT升高等。其中16例误诊，误诊的主要疾病为病毒性肝炎。误诊主要原因：①对本病认识不足;②疾病的临床表现多不典型；③检查手段受限制;④满足于常见病的诊断。结论具有三联征典型表现病例不多，表现缺乏特异性，易误诊。故凡有胆道系统症状、腹块、不明原因的腹痛、胆汁性腹膜炎、不典型的急性胰腺炎在鉴别诊断时均应警惕本病。
Objective To investigate the clinical features and misdiagnosis of children with congenital bile duct dilatation. Methods The clinical manifestations, laboratory tests and misdiagnosis of 26 cases of pediatric congenital biliary dilatation confirmed by operation and pathology in the past 15 years were analyzed. Results with “abdominal block, abdominal pain, yellow syndrome,” triple pine typical performance in 4 cases, may have abnormal liver transaminases, bilirubin, hematuria, amylase, ALP, γ-GT increased. 16 cases of misdiagnosis, misdiagnosis of the main disease is viral hepatitis. The main causes of misdiagnosis: ① lack of knowledge of the disease; ② clinical manifestations of the disease are atypical; ③ means of examination is limited; ④ meet the diagnosis of common diseases. Conclusion There are three typical cases of triad typical cases, the lack of specificity of performance, easy to misdiagnosis. Where there are biliary system symptoms, abdominal block, unexplained abdominal pain, biliary peritonitis, atypical acute pancreatitis should be vigilant in the differential diagnosis of the disease.