CAJ | 학술논문

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）×109／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。
목적：총결사형선천성담관확장증적진단특점，제고기진단적준학성。방법2011년1월지2013년10월아문공수치사형선천성담관확장증환인35례，기중남성8례，녀성27례，평균년령3.1（1～8）세，술전상규행혈상규、간공능、복부채초급상복부증강 CT 혹자공진등검사；술중행담도조영검사，동시측정담관내담즙이정분매치。환인균실시확장담관절제、담장문합술。결과환인주요림상표현위반복복통급복부불괄（100％），기차위황달（25％）；술전혈청총담홍소、혈백세포계수급술중담즙이정분매측정평균치분별위（54.99±83.90）mol／L、（8.39±2.74）×109／L、（28418.82±41459.41）IU ／L；술전복부 CT 혹자공진검사급술중담도조영균제시담총관경중도확장，최대확장직경평균위（1.96±0.55）cm；영상학검사발현이담합류이상28례（80％），포괄이담합류공동관연장[（10.28±5.05）mm]、이담회합각도증대（70.39°±10.61°）급공동관개구위치이상（8례，점22.9％）。결론의거전형적림상표현급규범적보조검사시학진사형선천성담관확장증적관건。
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 201 1 to October 201 3.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 sur-gery;cholangiography examination was conducted intraoperatively,at the same time,the bile pancreatic amyl-ase 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 (1 00%)and jaundice (25%).The average value of serum total bilirubin,white blood cell count,and bile pancreatic amylase were(54.99 ±83.90)mol/L,(8.39 ±2.74)×1 09 /L,and(28 41 8.82 ±41 459.41 )IU /L,respectively.Imaging and cholangiography examination showed mild to moderate common bile duct dilata-tion.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 (1 0.28 ±5.05)mm],increased angle of pancreatobiliary convergence (average of 70.39°±1 0.61 °),and abnormal openings of common duct (8 cases,22.9%). Conclusions Accurate diagnosis of congenital fusi-form bile duct dilatation is relying on typical clinical manifestation and specific auxiliary examination.