A rare but serious type of brain damage caused by severe jaundice in newborns
What is Kernicterus?
Kernicterus is a form of brain damage that occurs when there are extremely high levels of bilirubin in a newborn’s blood, leading to the yellow pigment crossing the blood-brain barrier and damaging brain tissues, particularly in areas that control movement and hearing. It is a preventable condition that usually results from untreated or poorly managed neonatal jaundice.
What causes Kernicterus?
Kernicterus is caused by excess bilirubin, a yellow substance produced when red blood cells break down. Normally, the liver processes bilirubin and eliminates it from the body. In some newborns, especially those with certain risk factors, bilirubin levels become dangerously high and enter the brain.
Common causes include:
- Hemolytic diseases (like Rh or ABO incompatibility)
- Premature birth (underdeveloped liver)
- G6PD deficiency
- Sepsis or infection
- Birth trauma causing bruising or bleeding
- Genetic or metabolic disorders affecting bilirubin clearance
Who is at risk?
- Premature infants (especially under 37 weeks gestation)
- Babies with blood group incompatibilities
- Breastfeeding difficulties leading to dehydration
- Siblings of infants who had severe jaundice
- Babies of East Asian, Mediterranean, or African descent (due to G6PD deficiency risk)
What are the symptoms of Kernicterus?
Kernicterus symptoms typically appear in stages as bilirubin levels increase.
Early signs (acute bilirubin encephalopathy):
- Severe jaundice (yellowing of skin and eyes, especially within first 24 hours)
- Poor feeding
- High-pitched crying
- Lethargy or floppy muscles
- Arching of the back and neck (opisthotonos)
- Fever
Later signs (chronic kernicterus):
- Hearing loss
- Cerebral palsy (especially involving involuntary movements)
- Gaze abnormalities
- Intellectual disabilities
- Tooth enamel problems
How is Kernicterus diagnosed?
Diagnosis is based on clinical presentation, history of high bilirubin levels, and neurological signs.
Diagnostic tools include:
- Total serum bilirubin (TSB) test
- Neurological examination
- Auditory Brainstem Response (ABR) for hearing evaluation
- MRI or CT scan to detect changes in brain tissue
- Blood tests for causes like hemolysis or infection
How is Kernicterus treated?
Kernicterus is not reversible, but early treatment of jaundice can prevent its development.
Treatments for high bilirubin include:
- Phototherapy: blue light therapy that helps break down bilirubin in the skin
- Exchange transfusion: replacing the baby’s blood to rapidly lower bilirubin
- Intravenous immunoglobulin (IVIG): used in cases of blood group incompatibility
- Hydration and feeding support: to help flush out bilirubin
- Monitoring bilirubin levels frequently in at-risk newborns
What is the prognosis for Kernicterus?
- Kernicterus often results in permanent neurological damage, including movement disorders, hearing loss, and developmental delays
- With early recognition and proper jaundice management, kernicterus is largely preventable
- Supportive therapies like physical therapy, occupational therapy, and speech therapy may help improve quality of life
Can Kernicterus be prevented?
Yes, kernicterus is largely preventable through early detection and treatment of neonatal jaundice.
Prevention strategies:
- Monitor bilirubin levels in all newborns, especially in the first few days
- Initiate phototherapy when bilirubin reaches concerning levels
- Encourage frequent breastfeeding to maintain hydration
- Educate parents to watch for signs of worsening jaundice
- Provide early follow-up visits after discharge