Paediatrics: Bilirubin encephalopathy (kernicterus)

2021-03-05 12:00 AM

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.

Presentation

  • Lethargy progressing to hypertonia then hypotonia.
  • Poor feeding.
  • Fever.
  • High-pitched cry.
  • Opisthotonos.
  • Seizures and coma.

The main differential diagnosis is meningoencephalitis/sepsis. Neonatal tetany may also present with opisthotonos.

Treatment

  • Supportive (likely to require full intensive care).
  • Urgent reduction of serum bilirubin by intensive phototherapy and exchange transfusion.
  • Give IV immunoglobulin.
  • Treat underlying cause.

Prognosis

The majority survives, but there is a high risk of athetoid cerebral palsy, deafness, and low IQ.