RESUMO
Moderate hypothermia therapy (HT) after perinatal asphyxia of the newborn has clearly demonstrated its efficacy in reducing both mortality and long-term neurosensory sequelae. HT has now been introduced in many developed countries as a standard of care for term infants meeting the entry criteria for therapeutic cooling. However, this new therapy is only effective in case of an acute perinatal hypoxic-ischemic event. Since a number of potentially deleterious complications have been described during cooling, a strict evaluation of the newborn's status is mandatory. To help clinicians reliably select newborns who may benefit from HT, amplitude-integrated electroencephalography (aEEG) is today strongly recommended. The indication criteria for cooling include aEEG in addition to delivery history, Apgar score, cord pH and lactates, and neurological scoring for encephalopathy. We report a clinical case of a term baby girl, considered for HT in our unit, because of a clinical feature of severe neonatal encephalopathy, metabolic acidosis on cord pH, and a history of fetal distress on fetal heart rate monitoring. However, despite all these criteria, her early aEEG, like her classic EEG, showed no signs of hypoxic-ischemic encephalopathy (HIE). She was then denied HT and her early magnetic resonance imaging (MRI) exam showed no signs of HIE but typical features of a metabolic disorder (Zellweger-like syndrome). Thanks to the HT strict protocols, including early aEEG and MRI exam, the right diagnosis was rapidly made and the hypothesis of a hypoxic-ischemic event during delivery was finally ruled out.
