Paediatrics: Bilirubin encephalopathy (kernicterus)
A clinical syndrome resulting from the development of excessive neuro-toxic unconjugated bilirubin levels.
Bilirubin encephalopathy (kernicterus)
A clinical syndrome resulting from the development of excessive neuro-toxic unconjugated bilirubin levels. Toxic levels lead to selective damage of the cerebellum, basal ganglia, and brainstem auditory pathways. It may occur in the healthy neonate if serum bilirubin is >360µmol/L, but usually only occurs at significantly higher serum levels (>430µmol/L after 48hr of life) unless:
- The infant is <24hr old.
- The infant is preterm.
- The infant is severely ill (any cause).
- The infant is acidotic.
- Caused by iso-immunization haemolytic disease.
- Reduced albumin binding caused by drugs or hypoalbuminaemia.
- Lethargy progressing to hypertonia then hypotonia.
- Poor feeding.
- High-pitched cry.
- Seizures and coma.
The main differential diagnosis is meningoencephalitis/sepsis. Neonatal tetany may also present with opisthotonos.
- Supportive (likely to require full intensive care).
- Urgent reduction of serum bilirubin by intensive phototherapy and exchange transfusion.
- Give IV immunoglobulin.
- Treat underlying cause.
The majority survives, but there is a high risk of athetoid cerebral palsy, deafness, and low IQ.