Moebius syndrome: a review of the anaesthetic implications.
Moebius syndrome is a rare congenital anomaly characterized by multiple cranial nerve palsies, orofacial malformations and limb anomalies. This study retrospectively reviewed the anaesthetic records of 19 children with Moebius syndrome who had anaesthesia at the Royal Alexandra Hospital for Children over a 15 year period and analysed the complications which occurred. Affected children most commonly present for anaesthesia for correction of strabismus, or for orthopaedic procedures to improve limb function. Despite the abnormal facies and drooling of saliva, these children are almost always of normal intelligence. Problems can arise during anaesthesia, with a high incidence of difficult or failed intubation. The use of a facemask and spontaneous breathing technique, where appropriate, seems to present no problems and maintaining an airway in this way appears to be safe. However, the potential for problems with aspiration of oral secretions should be remembered and the use of antisialogogue premedication is recommended. Affected children have a high incidence of other anomalies, including congenital cardiac disease, spinal anomalies, corneal abrasions and peripheral neuropathies and a careful preoperative assessment is essential.
Ferguson
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D000293:Adolescent; D000768:Anesthesia, General; D002648:Child; D002675:Child, Preschool; D003316:Corneal Diseases / Q000150:complications; D003389:Cranial Nerve Diseases / Q000151:congenital* / Q000503:physiopathology; D005121:Extremities / Q000601:surgery; D005145:Face / Q000002:abnormalities; D005158:Facial Paralysis / Q000151:congenital* / Q000503:physiopathology; D006330:Heart Defects, Congenital / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007360:Intelligence; D007442:Intubation, Intratracheal / Q000009:adverse effects; D017880:Limb Deformities, Congenital; D008397:Masks; D009056:Mouth Abnormalities / Q000473:pathology; D009886:Ophthalmoplegia / Q000151:congenital* / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000150:complications; D011015:Pneumonia, Aspiration / Q000517:prevention & control; D011292:Premedication; D012119:Respiration; D012189:Retrospective Studies; D012463:Saliva / Q000378:metabolism; D012472:Salivation / Q000187:drug effects; D013131:Spine / Q000002:abnormalities; D013285:Strabismus / Q000601:surgery; D013577:Syndrome