- charcoal filters placed in the anesthesia
breathing system will rapidly purge the system
INHERITED DISORDERS
MALIGNANT HYPERTHERMIA
- pharmacogenetic disorder of skeletal muscle
that when triggered results in a hypermetabolic
process associated with significant morbidity
and mortality
- commonly triggered by succinylcholine or
halogenated inhaled anesthetics
- associated with extreme physiologic stress or
heat exhaustion
- individuals susceptible have a mutation of the
ryanodine receptor that permits the
uncontrolled release of calcium from the
sarcoplasmic reticulum  sustained muscle
contraction/rigidity, metabolic and respiratory
acidosis, hypercarbia, tachycardia,
hyperthermia, rhabdomyolysis, hemodynamic
instability
- incidence of 1:40,000 to 1:250,000 but may be
as high as 1:15,000 in children, mortality rate of
9.5%
MANAGEMENT OF ACUTE MH
- the earlier it is identified, the better the
outcome
- FIRST SIGN OF MH is usually an increase in
ETCO2 that does not respond to an appropriate
increase in ventilation
- nonspecific signs: tachycardia, tachypnea,
hypertension
- muscle rigidity, masseter spasm, and
respiratory and metabolic acidosis develop
subsequently
- hyperthermia may occur early or late
- mimics: sepsis, hyperthyroidism, and NMS
- ANY HALOGENATED INHALED ANESTHETIC
SHOULD BE DISCONTINUED
- hyperventilation with 100% oxygen
- INSERT SCORING SYSTEM
- surgical team should be informed and the
procedure aborted or terminated as quickly as
possible under intravenous anesthesia
of any halogenated anesthetic
- MOST DEFINITIVE TREATMENT: dantrolene, a
hydantoin derivative that inhibits the pathologic
release of Ca2+ (IV dose of 2.5 mg/kg repeated
until it subsides)
- serial ABG
- supportive measures
MANAGEMENT IN A SUSCEPTIBLE PATIENT
- most definitive test: CAFFEINE-HALOTHANE
CONTRACTURE TEST
- avoidance of triggering agents is CRUCIAL
- IV agents propofol, benzodiazepines ,opioids,
NDMB, and NO are SAFE
- prepare anes machine: removal or closure of
vaporizers, change of all disposable
components, and flushing the machine with
100% oxygen (may require >2 hours)
- charcoal filters attached to both limbs of the
anesthesia breathing circuit before and during
the procedure are effective for reducing
halogenated anesthetics to less than trace
amounts
- prophylactic dantrolene is NOT recommended
PORPHYRIA
- caused by enzymatic deficiencies in the heme
synthesis pathway
- each of the porphyrias is caused by a
deficiency of one of the eight enzymes that
results in an accumulation of porphyrin
precursors with toxic effects
- four acute porphyrias that result in acute
attacks: acute intermittent porphyria (most
common), hereditary coproporphyria,
variegate porphyria, and ALA-dehydratase-
deficient porphyria (rarest)
- AIP typically occurs in young adults and is
more common in women
- features of AIP: fever, tachycardia, nausea,
emesis, severe abdominal pain, weakness,
seizures, confusion, hallucinations
- weakness can be so severe that respiratory
failure ensues
- hyponatremia secondary to inappropriate
secretion of ADH, rarely severe hypertension
and encephalopathy
- attacks may last for 1-2 weeks and can be
triggered by hormone changes during the
menstrual cycle, fasting, infections, and
exposure triggering drugs (INSERT DA TABLE)
- treatment removal of triggers, resolution of
infection, supportive care for skeletal muscle
weakness
- specific therapy: infusion of hemin solution 
inhibits 5-aminolevulinic acid synthetase and
decreases production of toxic intermediates

MANAGEMENT OF ANESTHESIA
- goal is to avoid drugs that may trigger acute
prophryia
- Propofol, isoflurane, sevoflurane, desflurane,
fentanyl, morphine,
and ketamine have been administered without
complications. Succinylcholine,
cisatracurium, and rocuronium are acceptable
for muscle relaxation. Regional
anesthesia may be administered as well