A long-term appreciation of cystic biliary atresia (1990-2025).

BACKGROUND

Cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA),characterized by cystic dilatation within an obliterated extrahepatic biliary system. The aim of this study was to characterize the clinical, diagnostic, and postoperative features of CBA, identify relevant subgroups, and assess prognostic outcomes and predictors of long-term surgical success.

METHODS

Single-center retrospective study of patients with CBA who underwent Kasai portoenterostomy (KPE) over a 35-year period (1990-2024). Patients were subdivided into 2 anatomical subgroups based on the patency (Sub-Group A) or obstruction/hypoplasia (Sub-Group B) of the proximal hepatic duct. Clinical characteristics, short and long-term outcomes were analyzed in the overall cohort and compared across the 2 sub-groups.

RESULTS

Among 886 infants with BA, 62 (7%) (n = 39, 63% female) were identified as CBA; 20 (32%) belonging to Sub Group A and 42 (68%) to Sub-Group B. Infants with antenatally detected CBA (n= 28, 45%) underwent surgery earlier than those detected postnatally [40 (24-50) days vs. 57 (44-67) days; P = 0.003]. Clearance of jaundice after KPE was achieved in 52 (84%) infants, with no significant difference between sub-groups (P = 0.29). Two (10%) children in Sub-Group A and 16 (38%) in Sub-Group B required liver transplantation, with comparable native liver survival curves (P = 0.16).The mean number of cholangitis episodes per patient was similar between the 2 subgroups.Gastrointestinal bleeding occurred exclusively in Sub-Group B (8/46, 19%,P=0.04), and clinically significant varices were also more prevalent in this group (4 vs. 24, P =0.02).

CONCLUSION

CBA represents a clinically favourable variant of classical BA. It incorporates 2 anatomical sub-groups, with similar long-term outcomes but with more complications related to portal hypertension in those with obstruction/hypoplasia of the proximal hepatic duct.