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目的：为了全面系统地掌握先天性胆总管囊肿的发病规律，为该病的诊断和治疗提供可参考的资料。方法：对１５９例先天性胆总管囊肿病例进行回顾性研究和分析，找出其发病规律。结果：女性多于男性。婴幼儿期就诊者占４９．７％，学龄前就诊者占７２．３％。主要症状依次为腹痛（６６．７％）、黄疸（５０．３％）、肿块（４４．７％）。具有腹痛、黄疸、肿块“三联征”者仅占１５．１％。年龄不同表现主要症状的先后次序不同。经术中造影合并胆胰管合流异常者占７９．４％。囊肿型者８５．４％，其中３岁以下者占５５．２％。梭型者虽比例较小，但儿童组占７３．９％。结论：诊断和治疗本病时应结合不同年龄的特点和发病规律。
Objective: To provide a reference for the diagnosis and treatment of congenital choledochal cyst in order to grasp the regularity of the disease. Methods: 159 cases of congenital choledochal cyst were retrospectively studied and analyzed to find out the pathogenesis. Results: More women than men. 49.7% of infants and young children were treated, pre-school visits accounted for 72.3%. The main symptoms were abdominal pain (66.7%), jaundice (50.3%) and mass (44.7%). With abdominal pain, jaundice, tumor “triple sign” accounted for only 15.1%. Different age performance of the main symptoms in different order. The intraoperative angiography combined pancreatic duct anomalies accounted for 79.4%. Cyst type 85.4%, of which 3 years of age accounted for 55.2%. Although a smaller proportion of shuttle, but the children accounted for 73.9%. Conclusion: The diagnosis and treatment of this disease should be combined with different age characteristics and incidence of law.