Anesthesia for a Muscle Biopsy：肌肉活检的麻醉.pptx

Case You are assigned to do an anesthetic for a muscle biopsy. The patient is a 13 month old male toddler with delayed motor milestones. H e can not sit unsupported, roll over, cruise or walk yet. He was delivered at 35 weeks PCA and spent 1 week in the NICU for feeding difficulties. There were never any respiratory difficulties. Per the parents the toddler has not had any prior anesthetics. There is no family history of muscular weakness or any problems with anesthesia although mom tells you that she was adopted and knows nothing about her biological family. What are you going to do? ? Are there any questions you would like to ask? ? What are your concerns? ? What is your anesthetic plan? Muscular Dystrophy vs Mitochondrial Myopathy Muscular Dystrophy Mitochondrial Disease Incidence 1:3200 male births 1:4000 births MH Susceptible NO NO Anesthesia Associated Hyperkalemia Yes No Family History Yes No Location of Weakness Proximal Proximal 4 Malignant Hyperthermia 56 MH Susceptible Diseases King Denborough Syndrome with and without mutations. Neuromuscular Disorders. Vol 21, Iss 6, June 2011 420-7. 7 TIVA for muscle biopsy ? What are baseline goals for all patients with mitochondrial myopathies ?? Should a TIVA be used then? ? How would a propofol based TIVA affect the child with mitochondrial myopathies? Types of Mitochondrial Myopathies ? Types of Mitochondrial Myopathies ? Respiratory chain deficiencies ? Mitochondrial DNA mutations ? Mitochondrial deletions 9 Anesthetic Goals of Mitochondrial Myopathies ? Understand the disorder ? Reduced fast ? IV fluids with glucose and avoids lactate ? Avoid stress that can aggravate energy production ? Monitor acid-base status ? Maintain temperature ? Maximize pain relief 10