Fact-sheet: Kernicterus


Updated on 04/23/2018 at 9:29 AM

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Definition

Bilirubin encephalopathy (kernicterus).

Rare, but the leading cause of preventable encephalopathy.

Clinical features

On day 2-5 of life.

Clinical signsNonspecific: onsetProgression: moderate toxicityAdvanced stage: overt toxicityConsciousnessdrowsylethargy or irritabilitysemi-coma or seizureToneslightly decreasedhypotonia/hypertonia, retrocollisopisthotonos, decreased toneCryhigh-pitchedshrillinconsolable

Outcome: risk of sequelae

- Intellectual disability

- Motor: extrapyramidal syndrome, choreoathetosis, oculomotor paresis

- Hearing loss

Laboratory findings

Hyperbilirubinemia.

Causes:

  • maternal-fetal incompatibility
  • Crigler-Najjar syndrome
  • hyperhemolysis: G6PD deficiency
  • Minkowski-Chauffard disease

Worsened or more frequent in premature or low-birth-weight infants.

MRI

Lesions:

  • pallidal ++
  • substantia nigra
  • hippocampus
  • tegmentum
  • subthalamic nucleus
  • dentate nucleus

The signal abnormalities found depend on the timing of the MRI:

  • Acute phase: T1 hyperintensity
  • Chronic phase: T2 hyperintensity

Management

Treatment:

  • phototherapy
  • exchange transfusion

Differential diagnosis

Numerous, guided by clinical presentation and history

Involvement of the basal ganglia:

  • vascular cause
  • toxic and metabolic disorders
  • degenerative and hereditary disorders
  • tumoral origin
  • infectious disease
  • systemic disease