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A case of kernicterus
An article for physicians by physicians
Originally published June 2007
IS0769-E

Of interest to all physicians

In recent years, re-emergence of kernicterus has resulted in a number of litigations against physicians and hospitals.

Why this topic is important

Many physicians believe kernicterus was eliminated in the 1980s. However, clinical experience indicates a re-emergence of this disease, and this is reflected in CMPA medico-legal files.

About the case

While neonatal hyperbilirubinemia is common, kernicterus remains rare. This case presents an adverse outcome for a newborn, and medico-legal difficulties for the physicians as a result of the failure to track the total serum bilirubin after the discharge from hospital.

Medico-legal case study

A woman of Asian origin had been followed by her family physician for a number of years. She had been treated for hypothyroidism and became euthyroid. Her first child, born at 36 weeks’ gestation, had hyperbilirubinemia treated successfully with phototherapy. Her second child, born at term, also needed phototherapy. Her third pregnancy was complicated by urinary tract infections. These were treated with nitrofurantoin on three occasions, the last course being given at 32 weeks’ gestation. Gestational diabetes was diagnosed at 34 weeks’ gestation and was treated with diet alone.

Labour started spontaneously at 35 5/7 weeks’ gestation and after a low-mid vacuum extraction a healthy male infant was born. Apgar scores were 8 and 9 and the birth weight was 3,500 g. There was no acidosis and no need for resuscitation at birth. He had a bruised forehead and a caput succedaneum.

Because of tachypnea, the baby was initially observed in the neonatal intensive care unit. The blood sugar remained stable and the transient tachypnea resolved. After a few hours, the child was transferred to the regular nursery under the care of a family physician. The next day, examination by the family physician revealed no clinical abnormality. The child was sucking and feeding well. As the parents were experienced, the child was discharged home at 29 hours of age to be followed by his own family physician one week later. This discharge occurred despite the knowledge that a total bilirubin requested by a nurse that morning was 143 µmol/l. The subsequent day, the child was seen at home by a public health nurse who noted the child was acting normally, was pink in colour and had white sclera.

Three days later, at five days of age, the child was brought back to the hospital and was found to be very hypotonic and apneic, requiring intubation and ventilation. The child was transferred to a tertiary care centre where he was noted to be markedly jaundiced, with a total bilirubin of 584 µmol/l. He underwent a double volume exchange transfusion, which needed to be repeated because of a rebound hyperbilirubinemia despite phototherapy. The bilirubin subsequently stabilized, then declined. However, the child developed seizures and bilirubin encephalopathy with hearing loss and dental dysplasia. An MRI showed bilateral abnormality of the globus pallidus, consistent with kernicterus.

The cause of the hyperbilirubinemia was not found despite extensive investigations. For example: there was no blood group incompatibility, the mother was O Rh positive and the baby was O Rh negative; there was no hemoglobin abnormality; G6PD, requested because of the mother’s ethnicity, was negative; metabolic studies were normal; the TSH was normal.

It was felt that the likely cause of the hyperbilirubinemia was an exaggerated jaundice of prematurity.

The family began litigation naming a number of the physicians involved in the care.

Expert opinion

Several neonatologists acting as experts for the plaintiffs were critical of the early discharge and lack of follow-up of the bilirubin. The experts noted that although the physicians were reassured by the large size of the baby, his good feeding ability and the experience of the parents, there were a number of factors that might have alerted the physicians to the risk of severe hyperbilirubinemia, including:

• previous siblings had required phototherapy,
• the ethnicity of the mother was Asian,
• the mother had gestational diabetes,
• this was a pre-term infant who was large for dates,
• the mother had undergone an instrumental delivery and the child had bruising and caput succedaneum,
• the visual assessment of the severity of jaundice is unreliable, particularly in individuals with more deeply pigmented skin,
• the infant’s hyperbilirubinemia had started in the first 24 hours as evidenced by the total serum bilirubin obtained on the day of discharge.

Experts commenting on the care provided in this case also noted:

• The high total serum bilirubin at discharge should have been addressed.
• The parents should have been educated about the risks of jaundice and the importance of following up with the appropriate physician or health professional after discharge.
• The care and instructions provided should have been documented.
• The Canadian Paediatric Society and the American Academy of Paediatrics have published guidelines regarding management of hyperbilirubinemia in newborn infants.

Legal outcome

Experts were unanimous in their criticism of the failure to address the bilirubin level. They also commented that the only treatment for kernicterus is prevention.

The case was settled and compensation paid by the CMPA on behalf of the neonatologist and the family physician who had seen the child in hospital initially. All the other physicians were let out of the action prior to settlement.

The bottom line

This case study demonstrates the importance of following up the bilirubin in a newborn, especially if the baby is pre-term or if there are any number of other risk factors for hyperbilirubinemia.