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Risks of anaesthesia in children with Angelman syndrome


Authors: Hladíková Andrea 1;  Olos Tomáš 2;  Hladík Michal 2;  Šilhánová Eva 1
Authors‘ workplace: Oddělení lékařské genetiky, FN Ostrava 1;  Klinika dětského lékařství FN Ostrava a LF Ostravské univerzity 2
Published in: Anest. intenziv. Med., 23, 2012, č. 4, s. 183-187
Category: Anaesthesiology - Review articles

Overview

The authors would like to make you aware of the unpredictable effects of anaesthetic agents in children with Angelman syndrome (a rare neuro-genetic disorder characterized by intellectual and developmental disability, epilepsy, sleep disturbance, seizures, jerky movements – especially hand-flapping, frequent laughter or smiling, usually a happy demeanour, microcephaly, plagiocephaly, prominent mandible, wide mouth, wide-spaced teeth, hypopigmentation, tongue thrusting, suck/swallowing disorders). Especially vagal hypertonia resulting in cardiac rhythm disturbances and asystole should be considered in the peri-operative care of these patients. Their recovery may be unpredictable and complicated.

Keywords:
Angelman syndrome – anaesthetic risk – difficult airway – vagal hypertonia


Sources

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10. Ohshita, N. et al. Anesthetic management of a child with Angelman’s syndrome. Masui, 2010, 59, 4, s. 484–486.

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Labels
Anaesthesiology, Resuscitation and Inten Intensive Care Medicine
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