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Muscle Biopsies and Anaesthesia. BCH Data 2005-2008. So what is the problem?. Links between muscular disorders and anaesthetics MH risk and volatiles 25% linkage to CCD Weak linkage to minicore disease Propofol and mitochondria?

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so what is the problem
So what is the problem?
  • Links between muscular disorders and anaesthetics
    • MH risk and volatiles
      • 25% linkage to CCD
      • Weak linkage to minicore disease
    • Propofol and mitochondria?
  • How can we decide what anaesthetic to give in the absence of a confirmed diagnosis?
anaesthetic database and ice lab results
Anaesthetic Database and ICE lab results
  • Anaesthetic given and histological diagnosis
  • Searched anaesthetic database for all procedures including muscle biopsy where full data is available (2005 >)
    • Pre op conditions
    • Anaesthetic details
  • Searched ICE for muscle biopsy histology
results 1
Results 1
  • 35 cases identified
  • Histology available in 32
  • Median age 2 (IQR 0.5-8)
  • 33 anaesthetised by Consultant
  • 2 anaesthetised by SpR
results 3 anaesthetics
Induction

Sevoflurane 17

Propofol 16

Ketamine 1

Spinal 1

Maintenance

Volatile 30

Isoflurane 18

Sevoflurane 12

Propofol 2

Propofol / ketamine 1

Ketamine 1

Spinal 1

Results 3: Anaesthetics
results 4 local blocks
Results 4: local blocks
  • Infiltration 25
  • Regional 6
    • Caudal 4
    • Epidural 1 (other surgery also)
    • Spinal 1
  • None stated 2
did the pre op diagnosis match the histology
Did the Pre-op diagnosis match the histology?
  • Yes 10
  • No 12
  • Unstated 11
  • No report 3
  • For 2 CCD:
    • no diagnosis recorded
  • For 3 MCD:
    • 1 minicore, 1 cong. myopathy, 1 none recorded
search of all cases on database where there is risk of mh
Search of all cases on database where there is risk of MH
  • Central core disease 6
    • 25% linkage
      • Induction: 2 propofol 4 sevo
      • Maintenance: 1 propofol 5 volatile
  • Minicore disease 8
    • Weak linkage
      • Induction: 7 propofol 1 sevo
      • Maintenance: 4 propofol 4 volatile
duchenne muscular dystrophy
Duchenne Muscular Dystrophy
  • Risk of rhabdomyolysis with volatiles?
  • 17 cases recorded (9 spine surgery)
      • Induction: 14 propofol 3 sevo
      • Maintenance: 8 propofol 9 volatile (2 both)
conclusions and questions
Conclusions and Questions
  • ? Recording of pre existing conditions
  • Pre op diagnosis wrong >50% of time
  • CCD or MCD and potential MH
    • 5/35 of muscle biopsies had this diagnosis
    • 9/14 CCD or MCD patients received volatiles
  • 9/17 DMD patients received volatiles
  • What should we do for muscle biopsies where diagnosis is unknown?
  • What should we do for CCD, MCD and DMD where diagnosis is known?
anaesthesia for muscle biopsies

Anaesthesia for Muscle Biopsies

Rob Alcock

RJAH Orthopaedic and General Hospital NHS Trust

anaesthesia for muscle biopsies15
Anaesthesia for Muscle Biopsies
  • What Should We Do for Muscle Biopsies Where Diagnosis is Unknown?
  • What Should We Do for CCD, MCD and DMD Where Diagnosis is Known?
  • What Neuromuscular Diseases are Out There? What are their Frequencies?
  • What Problems Might We Encounter?
  • What are the Risks?
what conditions are biopsied
What conditions are biopsied?
  • Muscular Dystrophies
  • Congenital Myopathies
  • Mitochondrial Myopathies
  • Metabolic muscle disease
  • Myositis and Dermatomyositis
  • Periodic Paralysis
  • Myotonias and Myotonic Dystrophy
muscular dystrophies
Muscular Dystrophies
  • Duchenne Muscular Dystophy (DMD) 1:5,000
  • Becker Muscular Dystrophy 1:18,000
  • Emery Dreyfuss Dystrophy 1: 100,000
  • Fascioscapulohumeral Dystrophy 1:20,000
congenital myopathies
Congenital Myopathies
  • Incidence 1:1000
  • 6000 in the W Midlands
  • Main Symptom is Hypotonia
  • Only 14% of Hypotonic infants
congenital myopathies20
Congenital Myopathies
  • Nemaline Rod Myopathy 20%
  • Central Core Myopathy 16%
  • Centronuclear Myopathy 14%
  • Minimulticore Myopathy 10%
  • Disproportionate Fibre Type Myopathy 21%
  • Rare Forms 19%
what are we worrying about
What Are We Worrying About?
  • Malignant Hyperpyrexia
  • Conditions Associated with Malignant Hyperpyrexia
  • Muscular Dystrophy
  • General Considerations
malignant hyperpyrexia mh
Malignant Hyperpyrexia (MH)
  • Spectrum of Pharmacogenetic Disorders
  • Disorder of Calcium Homeostasis
  • Triggered by Suxamethonium and Volatile Anaesthetics
  • Frequently associated with Ryanodine Ca Efflux Channel on the Sarcoplasmic Reticulum
  • Previous Uneventful Exposure to Triggers does not rule out MH
  • Diagnosed by In vitro Contracture Test
masseter spasm
Masseter Spasm
  • Defined as lasting > 2 mins after Administration of Suxamethonium
  • 30% may prove to have MH
  • Wait
  • Resort to Trigger Free Anaesthesia
genetics of mh
Genetics of MH
  • 19q11.2-13.2 Ryanodine (RyR1):- Release of Ca2+stores from sarcoplasmic reticulum
  • 17q11.2-q24:- Altered sodium channel functioning
  • 7q21.1 Dihydropyridine (DHP):- voltage sensor for RyR1
  • 1q32 CACNL1A3 gene encoding the alpha 1-subunit of the voltage-gated DHP receptor that interacts with RyR1
conditions associated with mh
Conditions Associated with MH
  • Central Core Myopathy
  • Minicore or Multiminicore Myopathy
  • King Denborough syndrome
central core myopathy
Central Core Myopathy
  • The most common presentation is at birth or in early childhood with weakness and hypotonia, slowly progressive.
  • Also present in adolescence as slowly progressive limb-girdle syndrome
  • Skeletal Abnormalities are Common
  • Asymptomatic individuals may present with CK or MH
  • 25% of patients are susceptible to MH
muscular dystrophy
Muscular Dystrophy
  • Malignant Hyperthermia Association of the United States (MHAUS)
  • 3 Cases Life Threatening Hyperkaemia
  • Duchenne & Becker
  • Following Use of Volatile Agents
general considerations
General Considerations
  • Avoid Suxamethonium in Children with Neuromuscular Disease
  • Avoid Hypothermia
  • Cardiac Problems associated with Dystrophies?
  • Respiratory muscle weakness
fulminant mh abortive mh overall incidence
Fulminant MH Abortive MH Overall Incidence

Incidence of Different Forms of MH

in Relation to Type of Anesthesia

  • - Total Number of Anesthetics 1:251,063 1:17,435 1:16,303
  • - General Anesthesia 1:221,811 1:15,404 1:14,403
  • - Anesthesia with Inhalation Agent 1:84,488 1:6,653 1:6,167
  • - With Sux 1:61,961 1:4,506 1:4,201
  • Without Sux 1:174,597 1:20,541 1:18,379
  • Anesthesia with Sux 1:140,006 1:8,819 1:8,297
anaesthesia for biopsy
Anaesthesia for Biopsy?
  • Randall et al Paediatric Anaesthesia 2007;17:22-27
  • 351 Patients with a Variety of NM Disorders
  • 274 Received Volatile Agents
  • 3 Received Sux!
  • No Cases of MH or Rhabdomyolysis
  • Conclusion: Risk of MH < 1%
anaesthesia for biopsy34
Anaesthesia for Biopsy?
  • Carr et al Can. J Anaes. 1995;42: 281-286
  • 2,214 Pts with suspected MH Sensitivity Undergoing Muscle Biopsy
  • Trigger Free Anaesthesia
  • 97% GA
  • 1082 were positive
  • 5 Patients had MH reactions
mitocondrial myopathies
Mitocondrial Myopathies
  • Case Reports of Resp and CV Depression, Lactic Acidosis and Rhabdomyolysis after Prolonged Propofol Anaesthesia
  • Propofol is Highly Metabolised
  • Volatiles are Minimally Metabolised
  • Should Propofol be Avoided?
conclusion
Conclusion
  • Patients for Bx Should Ideally be Anaesthetisd in the Absence of Volatiles.
  • Patients with Known CCD, MCD and DMD Should be Anaesthetised without Volatiles.
  • Patients with Known Mitochondrial Disease Should be Anaesthetised with Volatiles.
  • No-one with NMD Should be given Sux!