What is the definition of Bilirubin Encephalopathy?

Bilirubin encephalopathy is a rare neurological condition that occurs in some newborns with severe jaundice.

What are the alternative names for Bilirubin Encephalopathy?

Bilirubin-induced neurologic dysfunction (BIND); Kernicterus

What are the causes for Bilirubin Encephalopathy?

Bilirubin encephalopathy (BE) is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created as the body gets rid of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (jaundice).

If the level of bilirubin is very high or a baby is very ill, the substance will move out of the blood and collect in the brain tissue if it is not bound to albumin (protein) in the blood. This can lead to problems such as brain damage and hearing loss. The term "kernicterus" refers to the yellow staining caused by bilirubin. This is seen in parts of the brain on autopsy.

This condition most often develops in the first week of life, but may be seen up until the third week. Some newborns with Rh hemolytic disease are at high risk for severe jaundice that can lead to this condition. Rarely, BE can develop in seemingly healthy babies.

What are the symptoms for Bilirubin Encephalopathy?

The symptoms depend on the stage of BE. Not all babies with kernicterus on autopsy have had definite symptoms.

Early stage:

  • Extreme jaundice
  • Absent startle reflex
  • Poor feeding or sucking
  • Extreme sleepiness (lethargy) and low muscle tone (hypotonia)

Middle stage:

  • High-pitched cry
  • Irritability
  • May have arched back with neck hyperextended backwards, high muscle tone (hypertonia)
  • Poor feeding

Late stage:

  • Stupor or coma
  • No feeding
  • Shrill cry
  • Muscle rigidity, markedly arched back with neck hyperextended backwards
  • Seizures

What are the current treatments for Bilirubin Encephalopathy?

Treatment depends on how old the baby is (in hours) and whether the baby has any risk factors (such as prematurity). It may include:

  • Light therapy (phototherapy)
  • Exchange transfusions (removing the child's blood and replacing it with fresh donor blood or plasma)

What is the outlook (prognosis) for Bilirubin Encephalopathy?

BE is a serious condition. Many infants with late-stage nervous system complications die.

What are the possible complications for Bilirubin Encephalopathy?

Complications may include:

  • Permanent brain damage
  • Hearing loss
  • Death

When should I contact a medical professional for Bilirubin Encephalopathy?

Get medical help right away if your baby has signs of this condition.

How do I prevent Bilirubin Encephalopathy?

Treating jaundice or conditions that may lead to it can help prevent this problem. Infants with the first signs of jaundice have bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).

All newborns have a follow-up appointment within 2 to 3 days after leaving the hospital. This is very important for late preterm or early term babies (born more than 2 to 3 weeks before their due date).

Kernicterus

REFERENCES

Hamati AI, Felker MV. Neurological complications of systemic disease: children. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier; 2022:chap 59.

Hansen TWR. Pathophysiology of kernicterus. In: Polin RA, Abman SH, Rowitch, DH, Benitz WE, eds. Fetal and Neonatal Physiology. 6th ed. Philadelphia, PA: Elsevier; 2022:chap 163.

Kaplan M, Wong RJ, Burgis JC, Sibley E, Stevenson DK. Neonatal jaundice and liver disease. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 11th ed. Philadelphia, PA: Elsevier; 2020:chap 91.

Marcdante KJ, Kliegman RM. Anemia and hyperbilirubinemia. In: Marcdante KJ, Kliegman RM, eds. Nelson Essentials of Pediatrics. 8th ed. Elsevier; 2019:chap 62.