Paediatric anaesthesia
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Paediatric anaesthesia · May 2018
Perioperative management of children with glycogen storage disease type II-Pompe disease.
Pompe disease is a rare metabolic disorder caused by a deficiency of the lysosomal enzyme acid α-glucosidase. Glycogen accumulation damages skeletal, cardiac, and smooth muscles, causing a progressive and debilitating muscle weakness and cardiomyopathy. As life expectancy has much improved since the introduction of enzyme replacement therapy an increasing number of patients are referred for surgical procedures. Due to the potential cardiopulmonary complications, these patients form a high-risk group for the anesthesiologist. ⋯ Despite the potentially high anesthetic risk for children with Pompe disease under enzyme replacement therapy, the incidence of perioperative complications in our study was relatively low. Our data suggest that with proper precautionary measures and a critical choice of timing of the operation, general anesthesia in children with Pompe disease could be relatively safe nowadays.
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Paediatric anaesthesia · May 2018
Lung ultrasound reclassification of chest X-ray data after pediatric cardiac surgery.
Lung ultrasound is gaining consensus for the diagnosis of some pulmonary conditions. Pulmonary complications are common in pediatric cardiac surgery. However, its use remains limited in this setting. Our aim was to test the feasibility of lung ultrasound following pediatric cardiac surgery and to compare lung ultrasound and chest X-ray findings, assessing whether lung ultrasound may provide additional information. ⋯ Lung ultrasound is feasible and accurate for the diagnosis of common pulmonary conditions after pediatric cardiac surgery, allowing reclassification of chest X-ray findings in a significant number of patients.