Prof. Shilpa Sharma - Best of the Best in Pediatric Surgery 2024

Biliary atresia is a progressive cholangiopathy that leads to morbidity and end stage cirrhosis.

In developing countries with delayed presentation, advanced cirrhosis, and hepatic failure, most infants with biliary atresia succumb to hepatopulmonary syndrome, portal hypertension, intraventricular hemorrhage, and aspiration due to massive ascites.

The liver may regenerate after physiological insults and resection, with hepatic resection acting as a trigger for regeneration.

The liver can regenerate only to a size that is proportional to its original size.

In biliary atresia, hepatic stellate cells activate and lead to a pro-fibrogenic pathway involving transforming growth factor, platelet derived growth factor, and interleukins.

In hepatic resection, hematopoietic stem cells lead to proliferation with the help of hepatocyte growth factor receptor, epidermal growth factor receptor, and cytokines like tumor necrosis factor.

Starting in 2015, the team began resecting segments 2 and 3 of the liver in a non-anatomical fashion to stimulate an alternative pathway of stem cell proliferation for hepatic regeneration.

The study compared outcomes of segmentectomy combined with Kasai procedure versus Kasai alone, measuring bilirubin and APRI at preoperative, day 7, and 1st, 3rd, 6th, and 12th month time points.

The median age at presentation was similar in both groups: 96 days in one group and 89 days in the other.

Serum bilirubin levels were significantly different between the Kasai-plus-resection group and Kasai-alone group at 1 month and 3 months postoperatively.

APRI (AST to Platelet Ratio Index) was significantly different between groups on postoperative day 7.

Mortality was lower in the study group (Kasai plus resection) at 56% compared to 93% in the control group (Kasai alone).

The jaundice-free percentage was 91% in the study group compared to 25% in the control group.

Resection of a part of the liver may trigger an alternative pathway of stem cell proliferation for hepatic regeneration with reduced fibrosis and improved survival.