alyssa brzenski anesthesia for a muscle biopsy. case you are assigned to do an anesthetic for a...
TRANSCRIPT
![Page 1: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/1.jpg)
Alyssa Brzenski
ANESTHESIA FOR A MUSCLE BIOPSY
![Page 2: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/2.jpg)
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.
![Page 3: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/3.jpg)
What are you going to do?• Are there any questions you would like to ask?•What are your concerns?•What is your anesthetic plan?
![Page 4: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/4.jpg)
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
![Page 5: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/5.jpg)
Malignant Hyperthermia
![Page 6: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/6.jpg)
![Page 7: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/7.jpg)
MH Susceptible Diseases
King Denborough Syndrome with and without mutations. Neuromuscular Disorders. Vol 21, Iss 6, June 2011 420-7.
![Page 8: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/8.jpg)
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?
![Page 9: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/9.jpg)
Types of Mitochondrial Myopathies• Types of Mitochondrial Myopathies• Respiratory chain deficiencies• Mitochondrial DNA mutations • Mitochondrial deletions
![Page 10: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/10.jpg)
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
![Page 11: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/11.jpg)
![Page 12: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/12.jpg)
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).
![Page 13: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/13.jpg)
![Page 14: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/14.jpg)
![Page 15: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/15.jpg)
What would you do?
![Page 16: 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](https://reader030.vdocuments.net/reader030/viewer/2022032703/56649cfe5503460f949ce658/html5/thumbnails/16.jpg)
Sources1. 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: Anesthesia-related 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