Alyssa Brzenski
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 Dystrophy
Mitochondrial Disease
1:3200 male births
1:4000 births
MH Susceptible
Anesthesia Associated
Family History
Location of Weakness
Malignant Hyperthermia
MH Susceptible Diseases
King Denborough Syndrome with and without mutations. Neuromuscular Disorders.
Vol 21, Iss 6, June 2011 420-7.
TIVA for muscle biopsy
• What are baseline goals for all patients with mitochondrial
• 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
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
• 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?
1. Allison KR: Muscular dystrophy versus mitochondrial myopathy: the dilemma of the
undiagnosed hypotonic child. Paediatr Anaesth 2007; 17: 1-6
2. Benca J, Hogan K: Malignant hyperthermia, coexisting disorders, and enzymopathies:
risks and management options. Anesth Analg 2009; 109: 1049-53
3. 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: 1001-3
4. Driessen J, Willems S, Dercksen S, Giele J, van der Staak F, Smeitink J: Anesthesiarelated morbidity and mortality after surgery for muscle biopsy in children with
mitochondrial defects. Paediatr Anaesth 2007; 17: 16-21
5. Driessen JJ: Neuromuscular and mitochondrial disorders: what is relevant to the
anaesthesiologist? Curr Opin Anaesthesiol 2008; 21: 350-5
6. 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: 436-41
7. Gurnaney H, Brown A, Litman RS: Malignant hyperthermia and muscular dystrophies.
Anesth Analg 2009; 109: 1043-8
8. Klingler W, Rueffert H, Lehmann-Horn F, Girard T, Hopkins PM: Core myopathies and
risk of malignant hyperthermia. Anesth Analg 2009; 109: 1167-73

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