Kernicterus – Symptoms, Causes and Treatment

Kernicterus is a form of brain damage caused by excessive jaundice. The substance which causes jaundice, bilirubin, is so high that it can move out of the blood into brain tissue. When babies begin to be affected by excessive jaundice, when they begin to have brain damage, they become excessively lethargic. They are 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. They have a high-pitched cry, and decreased muscle tone, becoming hypotonic or floppy) with episodes of increased muscle tone (hypertonic) and arching of the head and back backwards.

Symptoms

The most common sign that a baby is at risk for Kernicterus is yellowing of the skin, especially in the first 24 hours after birth. Sometime, the whites of the baby’s eyes may turn yellow too. Other signs are a baby who is not alert or who is difficult to wake up. Babies with Kernicterus often have a shrill, high pitched cry and may appear weak or limp. Sometimes they lie unnaturally in bed with their bodies arched upward when lying on their backs.

Causes

Kernicterus is caused by very high levels of bilirubin. Bilirubin is a yellow pigment that is created in the body during the normal recycling of old red blood cells. High levels of bilirubin in the body can cause the skin to look yellow (which is called jaundice).In some cases when there are extremely high levels of bilirubin in the body or the baby is extremely ill, the substance will move out of the blood and collect in the brain tissue.

kernicterus was once the leading cause of athetoid (writhing) cerebral palsy. Because of the largely effective control of newborn jaundice, athetosis is now a relatively rare form of cerebral palsy. The syndrome in term infants is marked by refusal to feed, a high-pitched cry, stiffness, and arching of the back. Survivors usually suffer not only athetoid cerebral palsy, but high-frequency hearing loss, paralysis of upward gaze, and dental enamel malformations.

Kernicterus is caused by a high level of bilirubin in a baby’s blood. If left untreated, the bilirubin can then spread into the brain, where it causes long-term damage. A low-level buildup of bilirubin is normal. This is called mild jaundice, and it gives a newborn a slightly yellowish tint to the skin and sometimes the eyes.

Treatment

Quick treatment may help prevent further brain damage. Treatment may start with light therapy and fluids given through a needle into a vein (intravenous fluid replacement). Sometimes a baby may also have a tube placed down his or her throat or into the stomach for feeding with a special type of formula. A baby will also have a blood type test so that he or she can quickly get a blood transfusion if it is needed. A blood transfusion may be given to help remove extra bilirubin from the baby’s blood.

Treatment depends on the severity of the condition. Usually treatment for Kernicterus focuses on decreasing the amount of unconjugated bilirubin in the blood. Once the amount of unconjugated bilirubin in the blood decreases, jaundice should go away.