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Canadian Journal of Anesthesia, Vol 33, 790-794, Copyright © 1986 by Canadian Anesthesiologists' Society

Anaesthesia For Diagnostic Muscle Biopsy in an Infant With Pompe'S Disease

KATHLEEN R. ROSEN MD1 and LYNN M. BROADMAN MD1

1 Department of Anesthesiology and Child Health and Development, Children's Hospital National Medical Center and the George Washington University Medical Center, Washington, D.C.

Address correspondence to: Dr. Lynn M. Broadman, Children's Hospital National Medical Center, 111 Michigan Avenue, Northwest, Washington, D.C. 20010.

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.

Key Words: METABOLISM: glycogenosis, glycogen-storage disease, Pompe's disease • ANAESTHESIA, CONDUCTION: femoral nerve block • ANAESTHESIA, INTRAVENOUS: ketamine • ANAESTHESIA: paediatric






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Copyright © 1986 by the Canadian Anesthesiologists' Society.