Alyssa Brzenski ANESTHESIA FOR A MUSCLE BIOPSY

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. He 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 DystrophyMitochondrial Disease Incidence1:3200 male births1:4000 births MH SusceptibleNO Anesthesia Associated Hyperkalemia YesNo Family HistoryYesNo Location of WeaknessProximal

Malignant Hyperthermia

MH Susceptible Diseases King Denborough Syndrome with and without mutations. Neuromuscular Disorders. Vol 21, Iss 6, June

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 Respiratory chain deficiencies Mitochondrial DNA mutations Mitochondrial deletions

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

Why avoid Propofol? All anesthetics depress mitochondrial function Propofol inhibits mitochondrial function at complex I, uncouple oxidative phosphorylation and is associated with propofol infusion syndrome Patients with mitochondrial disease are at increased risk for toxic effect, namely cardiac depression and respiratory failure (during surgery and later).

What would you do?

Sources 1. Allison KR: Muscular dystrophy versus mitochondrial myopathy: the dilemma of the undiagnosed hypotonic child. Paediatr Anaesth 2007; 17: Benca J, Hogan K: Malignant hyperthermia, coexisting disorders, and enzymopathies: risks and management options. Anesth Analg 2009; 109: Davis PJ, Brandom BW: The association of malignant hyperthermia and unusual disease: when you're hot you're hot or maybe not. Anesth Analg 2009; 109: Driessen J, Willems S, Dercksen S, Giele J, van der Staak F, Smeitink J: Anesthesia- related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects. Paediatr Anaesth 2007; 17: Driessen JJ: Neuromuscular and mitochondrial disorders: what is relevant to the anaesthesiologist? Curr Opin Anaesthesiol 2008; 21: Footitt EJ, Sinha MD, Raiman JA, Dhawan A, Moganasundram S, Champion MP: Mitochondrial disorders and general anaesthesia: a case series and review. Br J Anaesth 2008; 100: Gurnaney H, Brown A, Litman RS: Malignant hyperthermia and muscular dystrophies. Anesth Analg 2009; 109: Klingler W, Rueffert H, Lehmann-Horn F, Girard T, Hopkins PM: Core myopathies and risk of malignant hyperthermia. Anesth Analg 2009; 109: