Paediatric anaesthesia
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Paediatric anaesthesia · Feb 2006
Letter Case ReportsAnesthesia management of a child with adrenoleukodystrophy.
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Paediatric anaesthesia · Jan 2006
Clinical TrialLeigh syndrome: anesthetic management in complicated endoscopic procedures.
Leigh's syndrome, a disorder of infancy and childhood, is characterized by gray matter degeneration and focal brainstem necrosis. It presents with special clinical features such as developmental delay, nervous system dysfunction, respiratory abnormalities, and hypertrophic cardiomyopathy that can be a real challenge to the anesthesiologist. Anesthesia or sedation has rarely been reported in patients with Leigh disease. We report our experience in sedating five children with Leigh syndrome in seven procedures undertaken in the endoscopy suite (outside the operating room). ⋯ This rare mitochondrial disease presents unique management problems to the anesthesiologist when using general anesthesia. Our patients were managed appropriately before endoscopy and underwent the procedure under deep sedation. No complications occurred. We concluded that deep sedation in the endoscopy suite was safe in this small series of patients with this rare disease.