先天性胆管扩张症合并共同管内蛋白栓的诊断和处理

目的 探讨先天性胆管扩张症合并共同管蛋白栓的诊断和清除方法.方法 2001年6月至2009年1月,在收治的先天性胆管扩张症203例患儿中,经手术前超声、CT、MRCP和术中胆道造影检查发现22例合并胰胆合流异常患儿的共同管内有蛋白栓存在,年龄1岁8个月至12岁,平均6.1岁.对其临床表现、影像学特点及手术中蛋白栓的清除方法进行分析,并对其术后症状、生化检测和胆道改变进行随访.结果 22例患儿均以腹痛症状为主,并发呕吐,其中13例患儿合并黄疸,2例囊肿穿孔.19例患儿腹痛的发作期,血和尿中的胰淀粉酶升高.术中胆道造影22例均发现共同管内充盈缺损和扩张,胰管显影,其中9例合并胰管扩张.采用插管和尿道镜冲洗清除后,共同管的直径回缩,胰管不再显影.蛋白栓非常脆软,很容易被水流的冲力破碎成较小的颗粒.经再次共同管造影证实,蛋白栓清除干净.其中8例经小儿尿道镜直视下证实蛋白栓清除干净.患儿随访3个月至8年,22例患儿经血生化和超声检查,无胰淀粉酶升高和再发共同管和胰管内结石者.结论 1岁以上先天性胆管扩张症患儿,以腹痛为主,发作时血和尿中的胰淀粉酶升高,影像学共同管充盈缺损和扩张者,应怀疑共同管内蛋白栓.术中胆道造影是可靠的诊断方法.插管冲洗或尿道镜下清除蚩白栓安全有效,远期预后好.

Diagnosis and treatment of protein plugs of the common channel in children with congenital biliary dilatation

Objective To investigate the method of diagnosis and removal for protein plugs in the common channel in children with congenital biliary dilatation.Methods The clinical presentation,radiological features and surgical treatment of 22 cases with congenital biliary dilatation(CBD) with protein plugs of the common channel were analyzed.The postoperative symptoms,laboratory examination and bile duct changes were evaluated during follow-up term.Results The 22 children had an average age of 6.1 years,ranging from 1 year 8 months to 12 years.The symptoms included abdominal pain (22),vomiting(22),jaundiee(13) and cyst perforation(2).In 19 patients,the levels of serum and urinary amylase increased when abdominal pain appeared.Intraoperative cholangiography showed filling defect and dilatation of the common channel and pancreatic ducts visualization in 22 cases,including nine cases of pancreatic duct dilatation.After removal of protein plugs,no dilatation of the common channel and pancreatic ducts decreased.The laparoscopy revealed protein plugs were fragile and could be easily broken into the smaller particles by the irrigation with saline.The repeat cholangiography confirmed protein plugs were removed completely.Under urethroseopy,protein plugs were cleared in 8 cases.Patients were followed up for 3 months to 8 years.The biochemical and ultrasound examination showed no increase in pancreatic amylase and recurrence of the stones in the common channel and pancreatic duct.Conclusions The children over 1 year of age with CBD,abdominal pain,with high levels of serum and urinary amylase when abdominal pain appeared and filling defect and dilatation of the common channel showed by radiologic findings could be suffering from protein plugs in the common channel.Intraoperative cholangiography is a reliable method of diagnosis.Laparoscopic assisted intubation and irrigation or urethroscopic assisted removal of protein plugs is safe and effective,with a good long-term effect.