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

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

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.

Keywords: Classification; Congenital biliary dilation; Diagnosis; Imaging; Prognosis.

MeSH terms

  • Bile Duct Diseases* / classification
  • Bile Duct Diseases* / congenital
  • Bile Duct Diseases* / diagnostic imaging
  • Child
  • Child, Preschool
  • Dilatation, Pathologic / congenital
  • Feasibility Studies
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Retrospective Studies