Kernicterus

 Kernicterus

Overview

Kernicterus is a rare condition that affects your baby’s brain when they have too much bilirubin in their blood (hyperbilirubinemia). Bilirubin is a yellow waste product that your body makes. Sometimes, your liver can’t remove enough bilirubin to keep you healthy. Too much bilirubin can cause jaundice. This is when your skin, the whites of your eyes and your gums or the area underneath your tongue (mucous membranes) appear yellow.

Symptoms of kernicterus progress in stages. In addition to jaundice, symptoms usually affect newborns and include irritability, poor feeding and seizures. Complications can lead to hearing loss and permanent brain damage. If you notice changes to your newborn’s behavior or appearance, contact their healthcare provider immediately.

You may hear your healthcare provider call kernicterus “bilirubin encephalopathy.”

Jaundice is common in newborns. Healthcare providers will monitor newborn jaundice to decrease your baby’s risk of developing kernicterus, which is why the condition is rare.

While the condition most often affects newborns, rare cases may affect adults.

Symptoms

The first sign of kernicterus is jaundice. If your baby has jaundice, the following parts of their body will have a yellow tone:

Skin.

The white part of their eyes.

Mucous membranes in their mouth.

Healthcare providers may diagnose this condition one to three weeks after birth.

In addition to jaundice, signs of kernicterus may include:

Lack of energy (lethargy).

Fever.

Vomiting.

Involuntary muscle movements.

Other signs and symptoms of kernicterus vary based on the stage of the condition.

Kernicterus stages

There are three stages of kernicterus, each with different symptoms.

Early stage: Difficulty feeding and sucking, very sleepy, low muscle tone and no response to loud sounds (startle reflex).

Middle stage: Irritability, a loud or high-pitched cry and tense muscles (a high muscle tone).

Late stage: Not feeding, stiff muscles (arched back with your neck bent backward) and seizures.

Late-stage kernicterus is very serious and life-threatening. If you notice your child has jaundice in addition to any other symptoms, contact their healthcare provider immediately.

Causes

Very high levels of bilirubin in your blood (hyperbilirubinemia) cause kernicterus. This happens when your newborn’s liver can’t get rid of enough bilirubin.

Causes of hyperbilirubinemia may include:

Polycythemia: Too many red blood cells in your body.

Hemolysis: Your body destroys red blood cells. This is a natural part of a cell’s lifecycle. Sometimes, your body destroys cells too soon or more than usual.

Injury during birth: Your baby may develop an injury during the birthing process like a collection of blood under their scalp (cephalohematoma), bleeding between their skin and skull (subgaleal hemorrhage) or bruising.

An underlying condition that affects your child’s blood cells,like Rh hemolytic disease or a condition that affects your child’s liver, like Crigler-Najjar syndrome.

When your liver isn’t able to get rid of bilirubin through your poop, it collects in your blood before moving into your brain tissue. Bilirubin in your brain can cause severe complications.

Complications

Complications of kernicterus can be life-threatening and include:

Hearing loss.

Cerebral palsy.

Problems with cognitive development.

Permanent brain damage.

Coma.

Risk factors

Your newborn is more at risk of developing kernicterus if they:

Have newborn jaundice.

Have a darker skin tone, as it can be difficult to detect yellowing skin, which is the first sign of the condition.

Were born before 37 weeks of pregnancy.

Aren’t eating well and, therefore, aren’t pooping to get rid of bilirubin.

Have a biological family history of newborn jaundice.

Children who are healthy may develop jaundice. Severe jaundice can lead to kernicterus.

Diagnosis

A healthcare provider will diagnose kernicterus after a physical exam and testing. Two tests include:

A light meter test.

A bilirubin blood test.

A light meter is a device that shines a medical-grade light on your baby’s skin. It calculates a bilirubin level based on how the light reflects off of your baby’s skin onto the device.

To confirm a diagnosis, a healthcare provider will perform a bilirubin blood test. For this test, a healthcare provider will remove a small sample of blood from your newborn’s heel. A lab will run the test to determine how much bilirubin is in your child’s body.

Imaging tests, like a head ultrasound, CT scan or MRI aren’t necessary but they may help rule out conditions with similar symptoms.

Treatment

Treatment for kernicterus can include:

Light therapy (phototherapy): Bright, ultraviolet lights shine on your newborn’s skin. These lights are medical-grade and won’t harm your child. Your newborn will rest in a bed with a light directed toward their body. Your baby will wear protective eye coverings during the treatment session.

Exchange blood transfusions: Donor blood and/or plasma will replace your newborn’s blood. A healthcare provider will place a small tube into a vein in your child’s arm or a catheter into your child’s umbilical stump (a piece of umbilical cord left on the body that a healthcare provider cut after birth). They’ll remove and replenish your newborn’s blood supply.

Intravenous immunoglobulins (IVIG): If your newborn has Rh disease, a healthcare provider may prescribe IVIG. This is an antibody therapy treatment. Your child will receive this treatment through an infusion into a vein in their arm.

Type of Doctor Department : Neurologist