Kernicterus

Sahisnuta Basnet
Neurological syndrome resulting from the
depostion of unconjugated bilirubin in
brain cells
etiology
 1. prematurity
 2. hemolytic disease of the newborn
 3. congenital familial non hemolytic
jaundice
 4. neonatal hepatitis
 5. congenital spherocytosis
Pathophysiology
 Precise level above which indirect
reacting bilirubin will be toxic for an
individual infant is unpredictable but
kernicterus is rare in healthy term
infants if serum level is under 25mg/dl
 Less mature the infant the greater the
susceptibility to kernicterus
 Asphyxia, acidosis, infection, and
hyperosmolality increases the
susceptibility- in these situations levels
as low as 8-12mg/dl may cause
kernicterus BBB is damaged by
asphyxia, acidosis, hyperosmolality
KERNICTERUS
 Yellow staining of brain
assc with neuronal
injury
 Affects basal ganglia,
cranial nerve nuclei,
brain stem nuclei,
hippocampus and AHC
of spinal cord
 Necrosis, neuronal loss
and gliosis …
pathological findings
Clinical manifestation
 Early signs: may be subtle. Lethargy,
poor feeding, loss of moro reflex
 Acute bilirubin encephalopathy:
 Stage 1: hypotonia, lethargy, high pitched
cry, poor suck, poor Moro
 Stage 2: hypertonia of extensor muscles,
ophisthotonus, rigidity, retrocollis, fever,
twitching of face or limbs, bulging fontanel,
seizures
 Stage 3: hypotonia replaces hypertonia
after about 1 wk of age
 Many die, survivors develop chronic
bilirubin encephalopathy
 Chronic bilirubin encephalopathy:
 Chreoathetosis with involuntary muscle spasms
 Extrapyramidal signs
 Seizures
 Mental deficiency
 Dysarthric speech
 Hearing loss
 Squints and defective upward gaze
Treatment
 C/F suggestive of bilirubin toxicity is an
indication for EXCHANGE TRANSFUSION
 Once established kernicterus is
irreversible and management is only
symptomatic