Impact of decompensated cirrhosis in children: A population-based study.

Background: We describe the proportion of children with compensated cirrhosis who develop decompensation in Ontario, Canada over the past two decades.

Methods: This is a retrospective population-based cohort study using routinely collected health care data from Ontario, Canada held at ICES during 1997-2017. Diagnosis of cirrhosis was made using validated ICES definition, and decompensation events were defined according to validated coding. Rates of decompensation, type of decompensation, and incidence of liver transplantation after decompensation were analyzed. Databases were linked at the individual level and analyzed at ICES-Queen's.

Results: A total of 2,755 children with compensated cirrhosis were included and 9% (253) developed decompensation over a median follow-up of 7 years. Children most likely to suffer decompensation were younger (median age 10 versus 4 years, p < 0.001) and female (45% versus 52%, p = 0.03). Ascites (137/253, 54%) was the most frequent complication. 199/2755 (7%) of children with cirrhosis received liver transplantation, of which 64% (128/199) occurred after a decompensation event. Overall, a total of 132 (4.7%) deaths occurred during the study period, with 55 deaths following a decompensating event.

Conclusion: We present the first study to describe rates of decompensation, type, and rate of liver transplantation after decompensation in pediatric cirrhosis at the population level. To improve the care of children with liver disease, early detection of liver disease, early initiation of specific treatments as well as identification of children who are at risk of becoming decompensated are crucial.