title: Anaesthesia for diagnostic muscle biopsy in an infant with Pompe's disease.
abstract: The anaesthetic management of children with glycogen-storage disease type IIa (Pompe's disease) presents a variety of challenges. A modification of a femoral nerve block, the inguinal paravascular block, as described by Winnie, was used in conjunction with intravenous ketamine to provide anaesthesia for a diagnostic muscle biopsy in a 5.5-month-old infant with Pompe's disease. A peripheral nerve stimulator was used to locate the femoral nerve in lieu of eliciting a paraesthesia.
authors.forename: K R
authors.initials: KR
authors.lastname: Rosen
authors.forename: L M
authors.initials: LM
authors.lastname: Broadman
mesh_terms: D000758:Anesthesia / Q000379:methods*; D001706:Biopsy; D005260:Female; D006008:Glycogen Storage Disease / Q000473:pathology*; D006009:Glycogen Storage Disease Type II / Q000150:complications / Q000473:pathology*; D006801:Humans; D007223:Infant; D007649:Ketamine / Q000009:adverse effects; D009132:Muscles / Q000473:pathology*; D009407:Nerve Block
chemical_list: D007649:Ketamine