Prise en charge anesthésique d'un enfant atteint de deltasarcoglycanopathie

A 3-year-old boy of North African decent was seen in the anaesthetic preoperative clinic prior to a scheduled adenoidectomy. His history revealed that his older brother suffered from an unknown form of muscular dystrophy. On clinical exam, no signs of muscular dystrophy were present. However in light of the patient's family history a blood sample for CPK was taken. This was found to be elevated and a neurological consultation was obtained. The patient was diagnosed with a deltasarcoglycanopathy, a rare form of limb girdle muscular dystrophy. A non triggering anaesthetic technique was used, avoiding halogenated anaesthetics and succinylcholine. The preoperative evaluation for a child with a suspected myopathy and the implications for its anaesthetic management are reviewed in this article. (c) 2007 Elsevier Masson SAS. Tous droits reserves