Kernicterus in Newborn Babies

NewbornKernicterus is a very serious medical condition affecting the brain that can potentially result if infant jaundice is not properly managed and treated after birth.

Kernicterus is a rare type of infant brain damage that can result from excessive levels of bilirubin in the brain caused by untreated cases of jaundice. Bilirubin is a bi-product substance naturally produced in the body during the disposal of old red blood cells. When levels of bilirubin in the blood become too high, however, it can be toxic - a condition referred to as hyperbilirubinemia. Jaundice occurs when hyperbilirubinemia progresses beyond a moderate level. The excessive accumulation of bilirubin caused by jaundice can eventually damage the brain and nervous system.

Newborn babies are particularly susceptible to jaundice because their liver is not fully developed at birth. The underdeveloped liver of a newborn can quickly become overworked causing bilirubin levels to increase and result in jaundice. Jaundice in newborns is a very common condition. About 6 out of every 10 newborn babies will experience some degree of jaundice during the first few weeks of life.

Infant jaundice is typically pretty harmless and short lived. Usually all you need to do is expose the baby to some sunlight regularly and the jaundice goes away quickly. In some cases, however, infant jaundice does not go away easily at which point it can become a very serious concern. If a case of infant jaundice becomes very severe and is not properly managed or treated, the excessive bilirubin levels in the baby's blood can cause a particular type of infant brain damage known as kernicterus.

Symptoms of Kernicterus

Kernicterus is a progressive condition that gets worse as the brain damage advances. The specific symptoms of kernicterus differ depending on what stage of progression the condition is in.

Stage I Symptoms
  • Jaundice: severe and/or prolonged infant jaundice is the root cause of kernicterus, so anytime a newborn has a particularly serious case of infant jaundice that does not clear up on its own it can be a symptom of kernicterus.
  • Poor Feeding: newborns with early-stage kernicterus will often demonstrate significant difficulties with feeding, latching on and sucking (with both breast and bottle feeding).
  • Hypotonia: hypotonia (often called "floppy baby syndrome") is a condition characterized by extreme lack of muscle tone. Babies with hypotonia may feel floppy, like a rag-doll when picked up.
  • Chronic Fatigue: abnormal levels of lethargy or apparent fatigue in a newborn (e.g., lack of intense crying, not getting up at night to feed, etc.) can be early symptoms of kernicterus.
Stage II Symptoms
  • Seizures: as the neurologic damage of kernicterus advances, babies may experience sudden, uncontrolled infant seizures.
  • Head Bulge: babies with kernicterus may develop a noticeable bulge in the "soft spots" or fontanel areas of the head.
Stage III Symptoms
  • Increased Seizures: as kernicterus reaches the most advanced stages, a baby's seizures will often increase in both frequency and intensity.
  • Hearing Loss: a common symptom of advanced kernicterus is extensive high frequency hearing loss. Of course this can be difficult to identify in newborn infants.
  • Stiffness: in early stages kernicterus may cause hypotonia (floppy baby syndrome). In the late stages, however, the condition will often result in excessive muscle stiffness and problems with movement and body control.
Causes & Risk Factors for Kernicterus

As explained above, kernicterus is caused by extreme, unmanaged jaundice that causes bilirubin levels in the blood to become so high that it damages the brain. Bilirubin is continuously manufactured inside the body from the constant breakdown and disposal of dead red blood cells. When old red blood cells get broken down (a process called hemolysis) most of the byproducts such as iron are reused elsewhere by the body. Bilirubin is the one byproduct of hemolysis that they body has no use for and must be regularly disposed of.

When bilirubin is initially created from red blood cell breakdown it is in the unconjugated form. Unconjugated bilirubin is toxic because it is not water-soluble and it will build up in the body's tissues. The liver filters unconjugated bilirubin out of the bloodstream and converts into conjugated bilirubin. Conjugated bilirubin from the liver is water-soluble which means it can be removed from the body through urine.

When many babies are first born, however, their liver is often not able to efficiently filter and convert unconjugated bilirubin into conjugated bilirubin fast enough to keep up with production. This invariably causes a backlog of unconjugated bilirubin in bloodstream of many newborn babies which leads to infant jaundice.

Infant jaundice is usually short-lived as the baby's liver is quickly able to get caught up with bilirubin conversion. However, in some babies the conversion and disposal of bilirubin by the liver never seems to get back on pace and their jaundice become more severe. If not properly managed and treated this can cause kernicterus where unconjugated bilirubin in the blood to infiltrates and damages brain tissue. There are certain known factors that will put a baby at increased risk of severe jaundice and kernicterus:

  • Premature Birth: the liver is one of the last organ to develop during gestation, so when a baby is born before week 37 their liver will be less efficient at bilirubin conversion and will take longer to get caught up.
  • Poor Feeding: feeding problems can prolong infant jaundice because frequent stools and urination helps accelerate bilirubin disposal.
  • Genetic Predisposition: babies with a family history of severe infant jaundice (i.e. siblings or parents who had serious jaundice as an infant) are at increased risk.
  • Maternal Blood Type: research has found that mothers with type O or Rh-negative blood types are more prone to have babies with high bilirubin levels.
Diagnosing Kernicterus

A definitive diagnosis of kernicterus is almost impossible to make without an autopsy. Diagnosis of kernicterus is usually made presumptively. Diagnosis of kernicterus in a newborn is a process that usually begins with a standard physical exam and the identification of jaundice indications (yellow eyes, skins discolor, etc.).

Treatment Options for Kernicterus

Sadly once kernicterus occurs there is currently no effective medical treatment. Kernicterus results from damage to the brain which cannot be reversed or repaired. Treatment of kernicterus is basically focused on prevention of hyperbilirubinemia / jaundice which causes the condition.

The good news is that newborn jaundice is something that can be very effectively treated. The first level of treatment for jaundice involves phototherapy (or light therapy) in which the baby is exposed to natural sunlight or a special artificial light. Light exposure accelerates the filtration and conversion of bilirubin inside the body. A second level of treatment for infant jaundice can involve blood exchange transfusions. This involves removal of some of the baby's blood and replacement with donor blood.