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The Anesthetic Management of a patient with Mitochondrial Myopathy

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The Anesthetic Management of a patient with Mitochondrial Myopathy Powered By Docstoc
					 The Anesthetic Management
of a patient with Mitochondrial
           Myopathy
            A Case Study
          By Krista Douglass

   USC Program of Nurse Anesthesia
        Mitochondrial Myopathy
   Mitochondrial principal source of cellular
    energy metabolism.
   Necessary for Krebs cycle, fatty acid
    oxidation, oxidative phosphorylation
   Affects tissues requiring most energy-
    muscle and nerve
   Mitochondrial defects- maternal
   Manifestations can be mild to severe
   Related to ratio of mutated genes to
    normal genes
                 Pathology

   Heterogeneous group of diseases
   Effect mitochondrial energy metabolism
   Result in decreased aerobic ATP
    production, impairment of energy-
    requiring functions
   Affects tissues and organs w/ highest
    energy demand, i.e. skeletal, cardiac, CNS
                 Pathology
   Hundreds of histologic manifestations,
    RNA/ DNA mutations
   Mutations along mitochondrial respiratory
    chain, myoadenylate deaminase deficincy,
    base pair substitutions (C-T, T-C), mtDNA
    point mutations, thymidine kinase 2 gene/
    deoxyguanosine kinase gene mutations
   These mutations result in mitochondrial
    respiratory chain dysfunctions
              Mitochondrial Myopathy
   Diagnosis is by muscle biopsy
   Hallmark- Ragged Red Fibers seen with Gomori
    trichrome stain
    Fibers also contain excess glycogen and
    increased lipid droplets, cytochrome c oxidase
    deficiency
   Presence of elevated lactic acid levels in blood
    and CSF can be helpful but not all patients have
    this
   Thompson, Viginia A. MD; Wahr, Joyce A. MD. (1997). Anesthetic considerations I patients presenting with
    mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (melas) syndrome. Anesthesia
    & Analgesia. 85(6); pp1404-1406.
               Common Manifestations
   Leigh’s Synd- seizures, hypotonia, fatigue,
    nystagmus, poor reflexes, swallowing
    difficulties, respiratory fatigue, poor motor
    function, ataxia.
   MELAS (mitochondrial encephalopathy
    lactic acidosis and stroke-like episodes)-
    short stature, seizures, stroke-like episodes
    w/ neurological deficits, HA, cognitive
    regression and disease progression.
http://www.specialchild.com. Mitochondrial Myopathy. November 11, 2003.
 http://www.thedoctorsdoctor.com. Mitochondrial Myopathy. April 5, 2004.
              Common Manifestations
   Kearns-Sayre Syndrome- progressive external
    ophthalmoplegia, pigmentary retinopathy, heart
    block and high CSF protein concentration
   MERRF (myoclonus epilepsy w/ ragged red
    fibers)- myoclonus, epilepsy, progressive ataxia,
    muscle weakness/ degeneration, deafness and
    dementia. Hallmark- ragged red fiber.

http://www.specialchild.com. Mitochondrial Myopathy. November 11, 2003.
http://www.thedoctorsdoctor.com. Mitochondrial Myopathy. April 5, 2004.
                     Case Study
   8 year old girl
   Admitted for chronic constipation & cyclic
    vomiting
   Scheduled for laparoscopic
    appendacostomy
       A stoma is made from the appendix
       Patient is able to irrigate the GI tract daily
       Prevention of constipation
   At birth, Apgar scores at 1 and 5 mins
    were 8/9
                      Case Study
   At 3 months of age- patient had a GI procedure
    (mother could not recall procedure, not on
    chart)
   At 6 months of age- Dx with a mild form of
    Mitochondrial Myopathy
   Recent past medical history- episodes of cyclic
    vomiting
       Chronic constipation
       Exercise intolerance
       Heat exhaustion
   Symptoms of disease limited to GI tract
    (chronic constipation and vomiting)
        Anesthetic Management
   Premed- 2mg Midazolam PO
   IV induction, Propofol 100mg
   ETT 5.5 placed without NDMR
   Air:O2 1:1
   Maintenance TIVA, propofol infusion between
    100-200 mcg/kg/min, Remifentanil 0.150
    mcg/kg/min
   TPN continued from ward
   Normal Saline used for intravenous fluid
    requirements
             Anesthesia cont
   Nitrous Oxide was used for first 45 mins
    then discontinued
   No muscle relaxant used
   No other inhalational gases used
   Temp within normal limits throughout
   Blood sugar checked after 1 hr= 112
   Procedure performed successfully
   Pt. emerged from anesthesia without
    complications
            Goals of Anesthesia
              for this patient
   Provide a continuous level of deep
    anesthesia without MH triggering agents
   No inhalational gases; no muscle relaxants
   Optimal functioning for mitochondria by
    keeping pt warm, continued TPN, NS
    instead of LR
   Avoid lactic acidosis
     Anesthetic Considerations
for Mitochondrial Myopathy patients
    Mitochondrial dysfunction affects function
     of nervous and muscular system
    Volatile anesthetics affect same systems-
     these patients have abnormal sensitivity
    General anesthetics shown to depress
     oxidative phosphorylation in isolated
     mitochondria
Morgan et al. (2002) Mitochondrial defects and anesthetic sensitivity. Anesthesiology. 96(5):1268-1272.
          Anesthetic Considerations
   Myopathy patients reported to be sensitive
    to thiopental
   Susceptible to develop AV blocks, BBB to
    3rd degree block
   Decreased ventilatory responds to hypoxia
    and hypercapnia


Naguib et al (1996). Sensitivity to mivacurium in a patient with mitochondrial myopathy. Anesthesiology. 84(6):1506-
  1509.
      Anesthetic Considerations

   Conflicting reports of response to
    neuromuscular blocking drugs
   Naguib et al (2002)- myopathy patient
    with extreme sensitivity to mivacurium
   Robertson et al (1984)- extreme sensitivity
    to d-tubocurarine with lack of reversal by
    neostigmine
            Anesthetic Considerations
    Lessel et al (1969)- sensitivity to succinylcholine
     in woman with ocular myopathy

    D’Ambra et al (1979)- “suggested mitochondrial
     myopathy did not involve NM junction”; pt
     showed normal repose to pancuronium and
     succinylcholine.


Naguib et al. (1996). Sensitivity to mivacurium in a patient with mitochondrial myopathy. Anesthesiology.
    84(6):pp1506-1509.
                Anesthetic Considerations
    Spinal anesthesia report to be safe
    Although local anesthetics disrupt oxidative
     phosphorylation – these patients reported
     to do well
    Increased MH susceptibility related to
     mitochondrial involvement in pathogenesis
     of MH
    Mitochondrial Ca+ efflux- perpetuation of
     MH

Fricker et al. (2002). Positive malignant hyperthermia susceptiblity in vitro test in a patient with mitochondrialmyopahty
     and myoadenylate deaminase deficiency. Anesthesiology. 97(6):1635-1637.
                                          Summary
    Preoperative assessment of functional organ
     system reserve rather than disease presence
    Reduced anesthetic requirement
    Susceptibility to prolonged NS depression
    Enhanced sensitivity to NM blockade
    Increased risk of MH
    Increased risk of sudden death from conduction
     abnormalities
Ciccotelli, et al (1997). An adult with inherited mitochondrial encephalomyopathy: report of a case. Anesthesiology.
     87(5):1240-1242.
                          References
   Ciccotelli, Kristine Kloss MD, RPh; Prak, Eline Luning AB;
         Muravchick, S. MD, PhD. (1997) An adult with
         inherited mitochondrial encephalmyopathy; report of a
         case. Anesthesiology, 87(5);1240-1242.

   Frickler, Ruth MD; Rafffelsberger, Thomas MD; Rauch-Shorny,
         Sigrid MD; Finsterer, Josef MD; Muller-Reible,
         Clemens PhD; Gilly, Hermann PhD; Bittner, Reginald MD.
         (2002). Positive malignant hyperthermia susceptiblity in
         vitro test in a patient with mitochondrial myopathy and
         ayoadenylate deaminase deficiency. Anesthesiology,
         97(6); 1635-1637.
                        References
   Garcia-Velasco, A.; Gomez-Escalonilla, C.; Guerra-
        Vales, J.M.; Cabella, A.; Campos, Y.; Arenas, J.
        (203). Intestinal pseudo-obstruction and urinary
        retention: cardinal features of a mitochonrial DNA related
        disease. Journal of Internal Medicine. 253(3); 381-385.

    Mancuso, Michangelo MD; Filosto, Massimiliano MD;
       Bonilla, Eduardo MD; Hirano, Michio MD; Shanske, Sara
       PhD; Vu, Tuan H. MD; DiMauro, Salvatore MD. (2003).
       Mitochondrial myopathy of childhood associated with
       mitochondrial dna depletion and homozygous mutation in
       the tk2 gene. Archives Neurology. 60(7); 1007-1009.
                        References
   Morgan, Phil G. MD; Hoppel, Charles L. MD; Sedensky,
        Margaret M. MD. (2002). Mitochondrial defects and
        anesthestic sensitivity. Anesthesiology. 96(5); pp. 1268-
        1270.

   Naguib, Mohamed MB.; el Dawlatly, Abdel Azim MB MD;
        Ashour, Mahmoud MB; Al-Bunyan, Muneera MB. (1996).
        Sensitivity to mivacurium in a patient with mitochondrial
        myopathy. Anesthesiology. 84(6); pp. 1506-1509.

   Thompson, Viginia A. MD; Wahr, Joyce A. MD. (1997).
       Anesthetic considerations I patients presenting with
       mitochondrial myopathy, encephalopathy, lactic acidosis,
       and stroke-like episodes (melas) syndrome. Anesthesia &
       Analgesia. 85(6); pp1404-1406.
                       References
   Savarese JJ, Ali HH, Basta SJ, Embree PB, Scott RPF, Sunder
        N, Weakly JN, Wastila WB, El-Sayad HA. (1998) The
        clinical neuromuscular pharmacology of mivacurium
        chloride; a short-acting nondepolarizing ester
        neuromuscular blocking drug. Anesthesiology. 68:723-32.

   http://www.specialchild.com. Mitochondrial Myopathy.
    November 11, 2003.

   http://www.mitomap.org. The Report of the Committee on
    the Human Mitochondrial Genome. April 3, 2004.

   http://www.thedoctorsdoctor.com. Mitochondrial Myopathy.
    April 5, 2004.

				
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