先天性胆管扩张症的新临床分类 - 华山 CBD 分类。

A new clinical classification of congenital biliary dilatation - HUAXI CBD classification.

机构信息

Department of Pediatric Surgery, West China Hospital of Sichuan University, #37 Guo-Xue-Xiang, Chengdu, 610041, China.

Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, 610041, China.

出版信息

BMC Pediatr. 2024 Nov 19;24(1):750. doi: 10.1186/s12887-024-05220-0.

DOI:10.1186/s12887-024-05220-0

PMID:39563279

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11575198/

Abstract

BACKGROUND

Current biliary dilatation (BD) classifications are complex and based on cases including secondary BD, leading to unclear distinctions. Notably, congenital and secondary BD differ in etiology, symptoms, and prognosis.

OBJECTIVE

To propose a more concise and more suitable classification of congenital biliary dilatation (CBD), and exploring the feasibility and effectiveness of this classification in diagnosis and treatment.

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: type I (distal extra-hepatic bile duct dilatation), type II (distal extra-hepatic combined with right and left primary hepatic bile duct dilatation), type III (extra-hepatic combined with secondary and above hepatic bile duct dilatation), and type IV (intra-hepatic bile duct dilatation). Meanwhile, the feasibility and reliability of the HUAXI CBD classification were analyzed by analyzing the clinical treatment strategies, perioperative complications and long-term follow-up results of different subtypes.

RESULTS

A total of 300 patients with CBD were included in this study. According to the HUAXI CBD classification method, 240 cases were type I, 48 cases were type II, 10 cases were type III, and 2 cases were type IV. After treatment, patients with type I had a normal hepatobiliary function after surgery and a good prognosis; patients with type II had good recovery of liver function in 37 cases (77.1%), but 11 cases (22.9%) had postoperative complications during follow-up; patients with type III and IV had 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.

CONCLUSIONS

The HUAXI CBD classification is consistent with treatment principles, concise and easy to remember, and more suitable for CBD clinical application.

摘要

背景

目前的胆道扩张（BD）分类较为复杂，且基于包括继发性 BD 在内的病例，导致分类不够清晰。值得注意的是，先天性和继发性 BD 在病因、症状和预后方面存在差异。

目的

提出一种更简洁、更适用于先天性胆管扩张（CBD）的分类方法，并探讨该分类方法在诊断和治疗中的可行性和有效性。

方法

根据 2015 年 1 月至 2018 年 12 月期间在四川大学华西医院小儿外科就诊的 CBD 患者的术前影像学资料，根据胆管扩张病变部位，提出一种新的 CBD 分类方法，命名为“华西 CBD 分类”。CBD 分为 4 型：Ⅰ型（肝外远端胆管扩张）、Ⅱ型（肝外远端合并左右肝内胆管扩张）、Ⅲ型（肝外合并二级及以上肝内胆管扩张）和Ⅳ型（肝内胆管扩张）。同时，通过分析不同亚型的临床治疗策略、围手术期并发症和长期随访结果，分析“华西 CBD 分类”的可行性和可靠性。

结果

本研究共纳入 300 例 CBD 患者。根据“华西 CBD 分类”方法，240 例为Ⅰ型，48 例为Ⅱ型，10 例为Ⅲ型，2 例为Ⅳ型。治疗后，Ⅰ型患者术后肝胆功能正常，预后良好；Ⅱ型患者肝功能恢复良好 37 例（77.1%），但随访期间发生术后并发症 11 例（22.9%）；Ⅲ型和Ⅳ型患者术后胆管炎（分别为 6/10、2/2）和胆总管结石（分别为 5/10、2/2）发生率较高，尤其是弥漫性肝内扩张患者预后较差，最终发展为肝硬化，需要进行肝移植。