Purpose: Infants and children with congenital dilatation of the bile duct (CDBD) usually also have some degree of liver fibrosis, which occasionally persists after diversion surgery, leading to liver cirrhosis. We conducted this study to evaluate the histological degree of liver fibrosis using intraoperative biopsy and to determine whether it affects the postoperative clinical course.
Methods: The subjects were 43 infants and children with CDBD. The age at operation ranged from 1 month to 14 years. Histological classification was defined as follows: grade 0, no fibrosis; grade 1, fibrosis confined to the portal area; grade 2, bridge formation with the neighboring portal area; grade 3, widened bridging fibrosis; and grade 4, pseudolobule formation, representative of cirrhosis.
Results: There were 21 (48.8%) patients with grade 0, 17 (39.5%) with grade 1, 4 (9.3%) with grade 2, 1 (2.3%) with grade 3, and 0 (0%) with grade 4 fibrosis. There were no differences in preoperative serum total bilirubin, total bile acid, aspartate aminotransferase, or gamma-glutamyl transpeptidase levels among the four groups. On the other hand, the grade 2 and 3 groups tended to include patients with a lower age at operation, and lower amylase levels in the serum and bile. Postoperatively, clinical symptoms resolved and laboratory data normalized in all patients. Two patients underwent liver biopsy for other reasons 3 and 4 years after surgery, which confirmed disappearance of the liver fibrosis. These patients had originally had grade 1 and grade 2 fibrosis, respectively.
Conclusion: Mild to moderate liver fibrosis is present in almost half of all children with CDBD; however, if it is graded as less than moderate, it will probably not affect the postoperative clinical course.