梭形先天性胆管扩张症35例诊断分析

Diagnosis characteristics of congenital fusiform bile duct dilatation-report of 35 cases

目的总结梭形先天性胆管扩张症的诊断特点，提高其诊断的准确性。方法2011年1月至2013年10月我们共收治梭形先天性胆管扩张症患儿35例，其中男性8例，女性27例，平均年龄3．1（1—8）岁，术前常规行血常规、肝功能、腹部彩超及上腹部增强CT或磁共振等检查；术中行胆道造影检查，同时测定胆管内胆汁胰淀粉酶值。患儿均实施扩张胆管切除、胆肠吻合术。结果患儿主要临床表现为反复腹痛及腹部不适（100％），其次为黄疸（25％）；术前血清总胆红素、血白细胞计数及术中胆汁胰淀粉酶测定平均值分别为（54．99±83．90）mol／L、（8．39±2．74）×10^9／L、（28418．82±41459．41）IU／L；术前腹部CT或磁共振检查及术中胆道造影均提示胆总管轻中度扩张，最大扩张直径平均为（1．96±0．55）cm；影像学检查发现胰胆合流异常28例（80％），包括胰胆合流共同管延长[（10．28±5．05）mm]、胰胆汇合角度增大（70．39°±10．61°）及共同管开口位置异常（8例，占22．9％）。结论依据典型的临床表现及规范的辅助检查是确诊梭形先天性胆管扩张症的关键。

Objetive To explore the diagnostic features of congenital fusiform bile duct dilatation, and to improve the diagnostic accuracy. Methods Thirty-five cases of fusiform bile duct dilatation were conducted in our hospital from January 2011 to October 2013. The patients （8 boys, 27 girls） had an average age of 3. 1 years （ 1 - 8 years）. Blood test, liver function, ultrasound, and imaging examination were checked before surgery; cholangiography examination was conducted intraoperatively, at the same time, the bile pancreatic amylase value was determined. All patients were given a dilative bile duct resection and biliary-enteric anastomosis. Results The main clinical manifestation of fusiform bile duct dilatation was repeatedly abdominal pain （100%） and jaundice （25%）. The average value of serum total bilirubin, white blood cell count, and bile pancreatic amylase were（54. 99 ±83. 90） tool/L, （8. 39 ±2. 74） ×10^9/L, and（28 418. 82 ±41 459.41 ）IU/ L, respectively. Imaging and cholangiography examination showed mild to moderate common bile duct dilatation. The average value of maximum diameter of common bile duct was （ 1.96 ± 0.55 ） cm; Twenty-eight （80%） pancreaticobiliary maljunction （PBM） were found involving prolonged common duct [ average of （ 10. 28 ± 5.05 ）mm ], increased angle of pancreatobiliary convergence （ average of 70.39°±10.61° ）, and abnormal openings of common duct （8 cases, 22. 9% ）. Conclusions Accurate diagnosis of congenital fusiform bile duct dilatation is relying on typical chnical manifestation and specific auxiliary examination.