Summary Purpose Traumatic brain injury (TBI) is an important cause of morbidity and mortality in children and early post-traumatic seizures (EPTS) are a contributing factor to ongoing acute damage. Continuous video EEG monitoring (cEEG) was utilized to assess the burden of clinical and electrographic EPTS. Methods Eighty-seven consecutive, unselected (mild – severe), acute TBI patients requiring pediatric intensive care unit (PICU) admission at 2 academic centers were prospectively monitored with cEEG per established clinical TBI protocols. Clinical and subclinical seizures and status epilepticus (SE, clinical and subclinical) were assessed for their relation to clinical risk factors and short-term outcome measures. Key findings Of all patients, 42.5% (37/87) had seizures. Younger age (p=0.002) and mechanism (abusive head trauma - AHT, p<0.001) were significant risk factors. Subclinical seizures occurred in 16.1% (14/87), 6 of whom had only subclinical seizures. Risk factors for subclinical seizures included: younger age (p<0.001), AHT (p<0.001) and intraaxial bleed (p<0.001). Status Epilepticus (SE) occurred in 18.4% (16/87) with risk factors including: younger age (p<0.001), AHT (p<0.001), and intraaxial bleed (p=0.002). Subclinical SE was detected in 13.8% (12/87) with significant risk factors including: younger age (p<0.001), AHT (p=0.001), and intraaxial bleed (p=0.004). Subclinical seizures were associated with lower discharge KOSCHI score (p=0.002). SE and subclinical SE were associated with increased hospital length of stay (p=0.017 and p=0.041 respectively) and lower hospital discharge KOSCHI (p=0.007 and p=0.040 respectively). Significance cEEG monitoring significantly improves detection of seizures/SE and is the only way to detect subclinical seizures/SE. cEEG may be indicated after pediatric TBI, particularly in younger children, AHT cases, and those with intraaxial blood on CT.

An increasing body of evidence demonstrates the importance of establishing age-dependent guidelines for physiological monitoring, pharmacological intervention, management of intracranial pressure and facilitating recovery of function.

Traumatic brain injury induces a complex pathophysiological cascade of cellular events. Central components of this response include increases in cerebral glucose uptake, reductions in cerebral blood flow, indiscriminate excitatory neurotransmitter release, ionic disequilibrium, and intracellular calcium accumulation. Acute glutamate release and nonspecific neuronal depolarization induce threatening perturbations in neuronal function. Restoration of homeostasis requires significant increases in glucose metabolism; however, there is often a concomitant reduction in cerebral blood flow, resulting in an uncoupling of supply and demand. Understanding the nature and timing of these processes provides the practicing clinician with a mechanistic rationale for acute physiological monitoring, aggressive interventions to address and minimize secondary injuries, implementation of advanced neuroimaging techniques, and careful monitoring return to normal activity in head injured patients.

We report a case of a 16-yr-old male with Danon disease caused by a novel mutation in the LAMP-2 gene. Mutations in the LAMP-2 gene result in the absence of LAMP-2 on immunohistochemical staining of muscle tissue, thus defining Danon disease, a rare X-linked myopathy. It is characterized clinically by HCM or left ventricular hypertrophy, a WPW pattern on ECG, variable degrees of muscular weakness (skeletal myopathy), mental retardation, and retinal changes. The patient presented with severe skeletal muscular weakness and respiratory failure. He also had a history of two OHTs, the first one for severe HCM and the second for allograft rejection. The patient's myopathy was initially presumed to be exclusively related to steroid-induced "critical care myopathy." However, further evaluation with a thigh muscle biopsy revealed autophagic vacuoles with sarcolemnal features suggestive of a lysosomal storage disorder. DNA analysis ultimately identified a previously unreported hemizygous IVS6+3_+6delGAGT splice site deletion mutation in the LAMP-2 gene located within the 5' splice site of intron 6, consistent with Danon disease.

Acute viral bronchiolitis is a leading cause of admission to pediatric intensive care units, but research on the care of these critically ill infants has been limited. Pathology of viral bronchiolitis revealed respiratory obstruction due to intraluminal debris and edema of the airways and vasculature. This and clinical evidence suggest that airway clearance interventions such as hypertonic saline nebulizers and pulmonary toilet devices may be of benefit, particularly in situations of atelectasis associated with bronchiolitis. Research to distinguish an underlying asthma predisposition in wheezing infants with viral bronchiolitis may one day lead to guidance on when to trial bronchodilator therapy. Considering the paucity of critical care research in pediatric viral bronchiolitis, intensive care practitioners must substantially rely on individualization of therapies based on bedside clinical assessments. However, with the introduction of new diagnostic and respiratory technologies, our ability to support critically ill infants with acute viral bronchiolitis will continue to advance.

Neurogenic stunned myocardium is an uncommon event after neurosurgical procedures in children. Pediatric intensivists need to consider this diagnosis in a patient with signs of myocardial dysfunction in the neurosurgical postoperative period. The management of neurogenic stunned myocardium involves close monitoring and establishing the absence of other causes of myocardial ischemia.

No abstract

The primary goal in treating any pediatric patient with severe traumatic brain injury (TBI) is the prevention of secondary insults such as hypotension, hypoxia, and cerebral edema. Despite the publication of guidelines, significant variations in the treatment of severe TBI continue to exist, especially in regards to intracranial pressure (ICP)-guided therapy. This variability in treatment results mainly from a paucity of data from which to create standards and from the heterogeneity inherent in pediatric TBI. The approach to management of severe TBI based on the published guidelines should be focused on ICP control, which should ultimately improve cerebral perfusion pressure. After identifying and surgically evacuating expanding hematomas, the first-tier treatment approach requires placing an ICP monitor. This is accompanied by medical management of elevated ICP, initially with simple maneuvers such as elevating the head of the bed to improve venous drainage, instituting sedation and analgesia to decrease metabolic demands of the brain, and draining cerebrospinal fluid. If these measures fail, then further first-tier interventions include hyperosmolar therapy to decrease cerebral edema and controlled ventilation to decrease cerebral blood volume. For elevations of ICP resistant to first-tier therapies, treatment escalates to second-tier therapy, which includes more aggressive measures such as placing jugular catheters to measure cerebral oxygenation with moderate hyperventilation, placing lumbar drains to remove more cerebrospinal fluid, administering high-dose barbiturates to suppress cerebral electrical activity, inducing hypothermia as a protective measure, and performing decompressive craniectomy to open the cranial vault. To properly execute these interventions, appropriate neuromonitoring is essential, starting from standard physiological parameters such as ICP, mean arterial blood pressure, and temperature. Additional modalities of neurologic monitoring are becoming more readily available and can provide additional clinically useful information about the pediatric patient with TBI; these include cerebral oxygenation, continuous electroencephalography, noninvasive blood flow monitoring, and advanced neuroimaging.