BACKGROUND AND OBJECTIVES: Despite the implementation of screening guidelines to 
identify infants at risk for hyperbilirubinemia, chronic bilirubin 
encephalopathy (CBE) continues to be reported worldwide in otherwise healthy 
infants. The incidence of CBE in Canada is unknown. The objectives of this study 
were to establish the incidence of CBE in Canada and identify epidemiological 
and medical risk factors associated with its occurrence.
METHODS: Data on infants were collected prospectively through the Canadian 
Pediatric Surveillance Program. Infants born between January 1, 2007 and 
December 31, 2008 were included if they either had symptoms of CBE and a history 
of hyperbilirubinemia, or if they presented in the newborn period with severe 
hyperbilirubinemia and an abnormal MRI finding as per the reporting physician.
RESULTS: During the study period, 20 cases were identified; follow-up data were 
available for 14 of these. The causes for the hyperbilirubinemia included 
glucose-6-phosphate dehydrogenase deficiency (n = 5), sepsis (n = 2), ABO 
incompatibility and other red blood cell antibodies (n = 7). Fifteen infants had 
abnormal brain MRI findings during the neonatal period. At follow-up, 5 infants 
developed classic choreoathetoid cerebral palsy, 6 had spectrum of neurologic 
dysfunction and developmental delay (as described by the reporting physician), 
and 3 were healthy.
CONCLUSIONS: CBE continues to occur in Canada at an incidence that appears to be 
higher than previously reported.