先天性胆管扩张症新分型——CBD华西分型法

A new classification of congenital biliary dilatation—HUAXI CBD classification

目的拟提出一种新的更简明、更适用于先天性胆管扩张症(congenital biliary dilatation,CBD)患者诊断和治疗的临床新分型—CBD华西分型,同时探索CBD华西分型的可行性及有效性。方法基于2015年1月至2018年12月期间四川大学华西医院小儿外科收治的CBD患者的术前影像学资料,根据胆管扩张病变累及胆管树的部位进行分型—CBD华西分型,将CBD分为4型:Ⅰ型,即远端肝外胆管扩张型;Ⅱ型,即远端肝外胆管合并左右一级肝管扩张型;Ⅲ型,即肝外胆管合并二级及以上肝管扩张型;Ⅳ型,即单纯肝内胆管扩张型。同时分析CBD华西分型应用的可行性及有效性。结果本研究共纳入300例未成年CBD患者,根据CBD华西分型法:Ⅰ型240例,Ⅱ型48例,Ⅲ型10例,Ⅳ型2例。Ⅰ型患者中236例采取肝外扩张胆管切除+肝总管空肠吻合术,4例非手术治疗;Ⅱ型患者全部采取肝外扩张胆管切除+肝门胆管成型+肝胆管空肠吻合术;Ⅲ型患者中有9例采取了肝外扩张胆管切除+肝总管空肠吻合术(其中5例肝内扩张局限于部分肝叶内予以肝部分切除+扩张肝管空肠吻合),1例仅实施经皮经肝胆道引流术;Ⅳ型患者2例均施行经皮经肝胆道引流术。Ⅰ型患者术后肝功能恢复好,总体预后较好;Ⅱ型患者中37例(77.1%)患者肝功能恢复良好,11例(22.9%)患者随访期间出现不同程度术后并发症;Ⅲ、Ⅳ型患者术后胆管炎(分别为6/10、2/2)与胆管结石(分别为5/10、2/2)发生率较高,特别对于肝内弥漫性扩张者,预后较差,最终发展为肝硬化而需要肝移植。结论CBD华西分型是主要针对以未成年患者为主体的CBD分型,以此为指导下的治疗原则合理、效果较好,并且分型简洁易记忆,能有效指导CBD临床治疗策略的选择。

Objective To intend to propose a new clinical classification of congenital biliary dilatation(CBD which is more concise and suitable for diagnosis and treatment,and explore feasibility and reliability of the new classification.Methods Based on the preoperative imaging data of patients with CBD admitted to the Department of Pediatric Surgery of West China Hospital of Sichuan University,from January 2015 to December 2018,a new classification of CBD was accomplished according to the site of bile duct dilatation lesions,which was named HUAXI CBD classification.The CBD was classified into 4 types:typeⅠ(distal extra-hepatic bile duct dilatation),typeⅡ(distal extra-hepatic combined with right and left primary hepatic bile duct dilatation),typeⅢ(extra-hepatic combined with secondary and above hepatic bile duct dilatation),and typeⅣ(intra-hepatic bile duct dilatation).Meanwhile,the feasibility and reliability of the HUAXI CBD classification were analyzed.Results A total of 300 patients with CBD were included in this study.According to the HUAXI CBD classification method,240 cases were typeⅠ,48 cases were typeⅡ,10 cases were typeⅢ,and 2 cases were typeⅣ.For typeⅠ,236 patients underwent cholecystectomy and Roux-en-Y hepaticojejunostomy and 4 cases were treated non-operatively;For typeⅡ,48 patients underwent cholecystectomy,central hepatic duct reconstruction and Roux-en-Y hepaticojejunostomy;For typeⅢ,9 patients underwent cholecystectomy and Roux-en-Y hepaticojejunostomy,5 of which had intrahepatic dilatation confined to part of the liver lobe and underwent partial hepatectomy with intra-hepaticojejunostomy.One case was treated with percutaneous transhepatic cholangial drainage(PTCD)only;two typeⅣpatients were treated with PTCD only.Patients with typeⅠhad a normal hepatobiliary function after surgery and a good prognosis;patients with typeⅡhad good recovery of liver function in 37 cases(77.1%),but 11 cases(22.9%)had postoperative complications during follow-up;patients with typeⅢandⅣhad high incidence of cholangitis(6/10,2/2,respectively)and choledocholithiasis(5/10,2/2,respectively)after surgery,especially those with diffuse intrahepatic dilatation having a poor prognosis,eventually developing cirrhosis and necessitating liver transplantation.Conclusion The HUAXI CBD classification is consistent with treatment principles,concise and easy to remember,and more suitable for pediatric clinical application,which can effectively assist in the selection of clinical treatment strategies for children with BD.