Case conference

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Case conference

• Postoperatvie Hyperthermia, Rhabdomyolysis,
  Critical Temperature, and Death in a Former
  Premature Infant After His Ninth General
  Anesthetic
• AA 2007 vol.
  105, No. 4 Oct

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Introduction

• An 8-month-old boy died 12h after an uneventful
  general anesthetic with sevo, fentanyl for
  closure of ileostomy
• Fever up to 43 degree, acidosis, increased end
  tidal CO2 and post-OP sepsis like symptoms and
  signs were noted
• Duchennes muscular dystrophy (DMD) was confirmed
  after autopsy

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Admission course

• 8-month-old boy
• Accepted the ninth general anesthesia
• Weight 4.1 Kg
• Pre-OP medication
• IV hyperalimentation via CVC
• Lasix causally
• Nasal cannula 3L? SPO291
• Gas before operation pH7.28 pCO245 pO250
  BE5 Na140 k3.6 Cl90 HCO331
• Vital sign HR160 BP90/50 mmHg RR20

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Previous GA
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Induction

• Propofol
• Rocuronium
• ?Intubation
• Maintain
• Sevoflurane
• O2
• Air
• Depolarizing muscle relaxant was not used

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Maintenance

• ET-CO2?60-70 torr
• Change in ventilation rate? useless
• Flow ?? useless
• Tachycardia 140-160
• Deepen the anesthetic with Fentanyl and Sevo?
  useless
• IV hydration with 15ml/Kg/H30ml 5 albumin
• No evidence of increased oxygen consumption
• Duration of anesthesia was 3H and 20 mins

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NICU course
On arrival

• On endotracheal tube, BT36.8 HR173 BP86/43
• Profound hypokalemia (K2.3), hypocalcemia
  (Ca0.5), hypomagnesemia (Mg0.3), metabolic
  acidosis, no urine output
• Began increasing BT
• Forehead skin BT up to 38, Cr1.1, coagulopathy
  developed PT25.9, APTT 93.7, INR2.4
• Focal seizure? phenobarbital
• Acute sepsis was Dx? antibiotic therapy was given
• Hypotension and inotropic agent usage
• BT up to 43
• Persistent acidosis, TTE poor contractility of
  bi-ventricle? expired

1H latter
5H latter
6H latter
Following
8H latter
12H latter
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Postmortem examination

• Immunohistochemical stain? DMD (Duchennes
  muscular dystrophy)
• Kidney section? dilated tubules, myoglobin stain
  positive in dilated tubules
• CK/LDH 21093/21393
• Multiple tissue hematomas and petechia,
  cholestatic hepatomegaly

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Discussion
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DMD(Duchennes muscular dystrophy)

• DMD incidence rate 1/3500
• Asymptomatic before age of 5 year
• Anesthesia? usually uncomplicated
• ? can exacerbate the chronic rhadomyolysis
• ? hyperkalemic cardiac arrest can occur
  during or after anesthesia
• In the absence of family history? hard to
  diagnosed DMD

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DMD with perioperative cardiac arrest

• Hyperkalemia (metabolic acidosis)
• Intra-, extra- cellular fluid shifting??
• If absence of SCC? rare case
• SCC?
• In this case? hypokalemia was noted initial
• The youngest DMD reported died within 24h after
  surgery

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Conclusion
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• Adverse events can occur in other myopathies,
  even in the absence of potent inhaled anesthetics
• Suspected malignant hyperthermia in a child with
  merosin deficiency in the absence of a triggering
  agent Ped ane 200616462
• Genetic tests of DMD should be applied whenever a
  positive family history

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Final diagnosis of death

• DMD
• Rhabdomyolysis and associated critical fever
• The author have no further explanation why these
  complication

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• Pediatric anesthesiologist must routinely inquire
  family history
• For stigma attached to the presence of this
  familial disease

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More .
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• Could this be malignant hyperthermia??
• Dantrolene therapy starting?
• Need more test to rule out MH?
• Ex halothane and caffeine contracture test?
• Holding inhalational anesthetics in myopathies
  patients?

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Letter to the editorAA vol 106 No4 April 2008

• Dantrolene may be a useful agent to dissipate
  heat? and might be helpful in this case
• Dantrolene
• Directly interferes with muscle contraction by
  binding the Ryr1 receptor calcium channel and
  inhibiting calcium ion release from sarcoplasmic
  reticulum.
• Consult the MH hotline? prefer underlying
  problem and coexisting metabolic issues

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Caffeine Halothane Contracture Test (CHCT)

• The gold standard for diagnosing MH
• Remain doubt Post-operative
• biopsy of living skeletal muscle expose to
  Caffeine Halothane
• 10-20 false positive rate
• Why no further MH test?
• Do not contract normally in myopathies patient
• MH consultant center not suggest
• Blood test
• Gene chromosomes 1,3,7,17,19(Ryr1)
• Positive test for mutation test is minimal

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Myopathies patient

• MH

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Thanks for your attention!!