Kernicterus

A form of brain damage caused by excessive jaundice in infancy.

Etiology:

damage to the brain centers of infants caused by increased levels of unconjugated bilirubin in the tissues.

Bilirubin builds up in the body to toxic levels and precipitates into tissues as the liver fails to metabolize it and the kidneys fail to excrete it from the body.

Pathophysioogy:

Pathologic diagnosis is characterized by bilirubin staining of the brainstem nuclei and cerebellum. Called bilirubin encephalopathy.

Manifestations:

Infant presents with yellowed skin tone and yellow discolouration to sclera of eyes.

In addition to the mild symptoms of jaundice listed above, severe brain damaging jaundice presents as follows:

  1. Excessively lethargy –  The infant is too sleepy, and they are difficult to arouse – either they don’t wake up from sleep easily like a normal baby, or they don’t wake up fully, or they can’t be kept awake.
  2. They have a high-pitched cry
  3. decreased muscle tone, becoming hypotonic or floppy)
  4. have episodes of increased muscle tone (hypertonic) and arching of the head and back backwards.
  5. As the damage continues, they may develop fever, may arch their heads back into a very contorted position known as opisthotonus or retrocollis.
Health promotion and prevention of hyperbilirubinemia
  1. Promote and support successful breastfeeding
  2. Establish protocols for the assessment of hyperbilirubinemia
  3. Measure total serum bilirubinemia or trancutaneous bilirubinemia of jaundiced infants within 24hrs
  4. Visual estimation of jaundice degree can lead to inaccurate assessment
  5. Interpret labs results of hyperbilirubinemia with respect to infant’s age in hours old
  6. Infants <38 weeks gestation, particularly those with inadequate milk intake in poor breastfeeding, those with congenital defects and infections such as hepatitis are at higher risk, requiring heightened monitoring.
  7. Perform a systematic assessment on all infants before discharge for severe hyperbilirubinemia risk.
  8. Educate parents to assess for newborn jaundice
  9. Provide follow-up and home visits for those at higher risk
  10. Treat newborns with severe hyperbilirubinemia with phototherapy and or exchange blood transfusion.

Management and Treatment:

Phototherapy. Light of the blue coloured wavelength in visible light alters the bilirubin chemical structure transforming it from a toxic form to a water soluble, non-toxic form that can be eliminated easily by the body.

At higher, more dangerous levels of bilirubin, or in certain situations where the bilirubin is expected to rise very rapidly, such as Rh or other hemolytic diseases of the newborn, exchange transfusions may be performed, to rapidly remove toxic bilirubin from the blood.

American Academy of Pediatrics, Clincical Practice Guideline, Subcommittee on Hyperbilirubinemai. (2004). Management of Hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics. 114 (1) 297-316.

Sharpiro, Steven. 2007. Jaundice and Kernicterus. Retrieved September 23, 2010 from http://www.kernicterus.org/

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