Cussenot O, Valayer J, Gauthier F
Chir Pediatr. 1987;28(1):8-19.
During a period of 18 years, 41 cases of congenital dilatation of the common bile duct in young patients (11 of them were less than 12 months of age) were treated at Bicetre Hospital. A retrospective analysis of these cases underline the following points. A close etiopathogenic relation exists between the presence of a common hepatico-pancreatic duct and a congenital dilatation of the common bile duct (in this study, 16 cases demonstrated the anomaly out of 20 cases for which the common bile duct could be analysed). Early surgical treatment must be undertaken before occurrence of liver complications (6 patients have cirrhosis when first treated, 4 of them were children less than 12 months of age). Abdominal echotomography (100% of preoperative diagnosis) and transparietal cholangiogram are the best investigations for providing pictures of hepatico-pancreatic abnormalities and collecting bile samples for bacteriological and chemical study. The treatment of congenital dilatation of common bile duct by cyst excision and Roux en Y hepaticojejunostomy has good long term results. After 20 cases of complete cyst excision, 6 of them were children under 12 months, and 17 cases or partial cyst excision respecting the cyst fundus when they were intraduodenal and retropancreatic in position (4 were children under 2 months) we have noted that 83% of these children had a good outcome with a mean follow up of 7 years. Complications include hepatico-jejunal stenosis at the anastomosis site in 2 patients, for which they were reoperated and cholangitis with hepatico-jejunal anastomosis in 6 cases. There were 3 deaths in children under 12 months who already developed hepatic cirrhosis and episodes of severe cholangitis when preoperative diagnosis was first proved. In conclusion, the future life of the majority of these children is expected to be uneventful inasmuch that bile stasis has been corrected, with resolution of inflammatory lesions which are known to expose to secondary malignancies of the biliary tract.
在18年的时间里,比塞特尔医院共治疗了41例年轻患者的先天性胆总管扩张症(其中11例年龄小于12个月)。对这些病例的回顾性分析突出了以下几点。肝胰管共同通道的存在与先天性胆总管扩张之间存在密切的病因学关系(在本研究中,20例可分析胆总管的病例中有16例显示出该异常)。必须在肝脏并发症出现之前尽早进行手术治疗(6例患者首次治疗时已出现肝硬化,其中4例为年龄小于12个月的儿童)。腹部超声检查(术前诊断率为100%)和经皮肝穿刺胆管造影是提供肝胰异常图像以及采集胆汁样本进行细菌学和化学研究的最佳检查方法。通过囊肿切除和Roux-en-Y肝空肠吻合术治疗先天性胆总管扩张症具有良好的长期效果。在20例完全囊肿切除病例中,6例为12个月以下儿童,17例在囊肿位于十二指肠内和胰后位置时进行了保留囊肿底部的部分囊肿切除(其中4例为2个月以下儿童),我们注意到这些儿童中有83%预后良好,平均随访7年。并发症包括2例患者吻合口处肝空肠狭窄,为此进行了再次手术,6例发生肝空肠吻合术后胆管炎。12个月以下已出现肝硬化且术前首次确诊时发生严重胆管炎的儿童中有3例死亡。总之,可以预期,由于胆汁淤积已得到纠正,炎症病变已消退,而炎症病变已知会增加胆道继发恶性肿瘤的风险,这些儿童中的大多数未来生活将平安无事。