OBJECTIVE: We report serial magnetic resonance (MR) and sonographic
behavior of globus pallidus in 5 preterm and 3 term infants with
kernicterus and describe the clinical context in very low birth weight
preterm infants. On the basis of this information, we suggest means of
diagnosis and prevention. METHODS: Charts and MR and ultrasound images of
5 preterm infants and 3 term infants with suspected bilirubin-associated
brain damage were reviewed. Included were preterm infants with severe
hearing loss, quadriplegic hypertonia, and abnormal hypersignal of globus
pallidus on T2-weighted MR imaging (MRI). In 1 infant who died on day 150,
the diagnosis was confirmed during the neonatal period. The others were
picked up as outpatients and scanned at 12 or 22 months' corrected age.
Three instances of term kernicterus were included for comparison of serial
MRI in the neonatal period and early infancy: they were caused by
glucose-6-phosphate dehydrogenase deficiency, urosepsis, and dehydration
plus fructose 1-6 biphosphatase deficiency. RESULTS: Five preterm infants
of 25 to 29 weeks' gestational age presented with total serum bilirubin
(TSB) levels below exchange transfusion thresholds commonly advised. Mixed
acidosis was present in 3 infants around the TSB peak. The
bilirubin/albumin molar ratio was >0.5 in all, in the absence of
displacing drugs. All failed to pass bedside hearing screen tests and had
severe hearing loss on auditory brain response testing. Symmetrical
homogeneous hyperechogenicity of globus pallidus was the alerting feature
in 1 infant. Globus pallidus was hyperintense on T1-weighted MR images in
this child. The other infants presented with severe developmental delay as
a result of dyskinetic quadriplegia and hearing loss. Globus pallidus was
normal on T1- but hyperintense on T2-weighted MR images at 12