Changes in Globus Pallidus With (Pre)Term Kernicterus

Abstract

ABSTRACT. 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 or 22 months' corrected age. Subthalamic involvement was documented in coronal fluid attenuated inversion recovery MRI in 2 infants. The term infants with classical clinical presentation in the neonatal period had MR behavior similar to the preterms, but pallidal injury was not recognized with targeted sonographic examination. Their

Unbound (Free) Bilirubin: Improving the Paradigm for Evaluating Neonatal Jaundice

“…3B. The increased extravascular UCB often reached levels sufficient to cause ABE despite the low B T (23 ), a phenomenon that still haunts clinical practice (37,38 ).…”

“…There is now unequivocal evidence that UCB-induced ABR changes are predicted by B f but not B T in both premature and term newborns (54 -57 ). In addition, UCB injury in susceptible areas of the CNS can now be documented using newer imaging techniques in vivo such as MRI (37,58 ), providing yet another new outcome measure for documenting UCB injury.…”

An approach to the management of hyperbilirubinemia in the preterm infant less than 35 weeks of gestation

“…[13][14][15] In past decades, autopsy proven kernicterus was reported in sick, very low birth weight infants who were exposed to low TSB levels (the so-called 'low bilirubin kernicterus') 16,17 and recent case series document that this remains a clinical risk. [18][19][20][21] In a recent study from the Netherlands, 18 5 sick, preterm infants (25 to 29 weeks gestation) with peak TSB levels ranging from 8.7 to 11.9 mg dl À1 (148 to 204 mmol l À1 ) developed the classical MRI findings of kernicterus. Serum albumin levels in these infants were strikingly low, ranging from 1.4 to 2.1 g dl À1 .…”

Diffuse basal ganglia or thalamus hyperechogenicity in preterm infants

“…1,2,[8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] Basal ganglia hemorrhage results from periventricular hemorrhagic infarction or hemorrhagic necrosis. In preterm infants <28 weeks, diffuse caudate hemorrhage is difficult to differentiate from hemorrhage in the germinal matrix over the body of the caudate nucleus (grade I).…”