Bilirubin encephalopathy
Bilirubin-induced neurologic dysfunction (BIND); KernicterusBilirubin encephalopathy is a rare neurological condition that occurs in some newborns with severe jaundice.
Newborns with severe jaundice
Newborn jaundice occurs when a baby has a high level of bilirubin in the blood. Bilirubin is a yellow substance that the body creates when it replac...
Causes
Bilirubin encephalopathy (BE) is caused by very high levels of bilirubin in the blood and body. 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 in the blood 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.
Rh hemolytic disease
Hemolytic disease of the newborn (HDN) is a blood disorder in a fetus or newborn infant. In some infants, it can be fatal. Normally, red blood cells...
Symptoms
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)
High muscle tone
Spasticity is stiff or rigid muscles. It may also be called unusual tightness or increased muscle tone. Reflexes (for example, a knee-jerk reflex) ...
ImageRead Article Now Book Mark Article - Poor feeding
Late stage:
- Stupor or coma
Stupor or coma
Decreased alertness is a state of reduced awareness and is often a serious condition. A coma is the most severe state of decreased alertness in which...
Read Article Now Book Mark Article - No feeding
- Shrill cry
- Muscle rigidity, markedly arched back with neck hyperextended backwards
- Seizures
Seizures
A seizure is the physical changes in behavior that occurs during an episode of specific types of abnormal electrical activity in the brain. The term ...
ImageRead Article Now Book Mark Article
Exams and Tests
A blood test will show a high bilirubin level (greater than 20 to 25 mg/dL). However, there is not a direct link between bilirubin level and degree of injury.
Normal value ranges may vary slightly among different laboratories. Talk to your health care provider about the meaning of your specific test results.
Treatment
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)
Outlook (Prognosis)
BE is a serious condition. Many infants with nervous system complications die.
Possible Complications
Complications may include:
- Permanent brain damage
- Hearing loss
- Death
When to Contact a Medical Professional
Get medical help right away if your baby has signs of this condition.
Prevention
Treating jaundice or conditions that may lead to it can help prevent this problem. Infants with the first signs of jaundice have their 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 should 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).
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, Fox WW, eds. Fetal and Neonatal Physiology. 6th ed. Philadelphia, PA: Elsevier; 2022:chap 163.
Kaplan M, Wong RJ, Bensen R, Sibley E, Stevenson DK. Neonatal jaundice and liver disease. In: Martin RJ, Fanaroff AA, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 95.
Ryan KS, Kliegman RM. Jaundice and hyperbilirubinemia in the newborn. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 137.
Kernicterus - illustration
Kernicterus is a condition where very high bilirubin levels in the blood are deposited in the brain tissue causing irreversible damage to the brain. In the newborn, early diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent kernicterus.
Kernicterus
illustration
Kernicterus - illustration
Kernicterus is a condition where very high bilirubin levels in the blood are deposited in the brain tissue causing irreversible damage to the brain. In the newborn, early diagnosis and treatment of jaundice or conditions that lead to jaundice may help prevent kernicterus.
Kernicterus
illustration
Review Date: 4/5/2025
Reviewed By: Charles I. Schwartz, MD, FAAP, Clinical Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, General Pediatrician at PennCare for Kids, Phoenixville, PA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
All rights reserved.