Re: Clinical discussion... as requested! Originally Posted by EmeraldNYL
Okay, so a few months ago I was in the ENT room at my clinical site doing some peds cases. A 2 yr. old presents for BMT (ear tubes), otherwise healthy besides seasonal allergies and freq. ear infections. Upon reviewing the anesthesia pre-op assessment that was completed in pre-admission testing, it is noted that the child has a questionable family history of malignant hyperthermia. Supposedly, the child's aunt has been diagnosed with MH. The child himself has never had anesthesia and has never been tested. The child's mother recently had a lap chole but does not have access to her records and is unaware if she was given a non-triggering anesthetic. The anesthesiologist I am with would like to cancel the case and have the child tested for MH before proceeding, but the parents become very upset and insist that the procedure be completed because the child is miserable and having very severe hearing difficulties. Unfortunately, this is not the first case of the day and the questionable history of MH has not been picked up by the physician assistant in PAT. So, what now?
Questions:
Would you proceed with the case, and if so, how would you proceed?
How does one test for MH exactly?
Describe the pathophysiology of MH.
List the triggering agents for MH.
Describe the treatment of suspected intra-op MH.
Which patients are at risk of MH?
1. Only after consulting further w/ parents, surgeon, and another anesthesia provider.
2. "The Caffeine Halothane Contracture Test (CHCT), a test performed on freshly biopsied muscle, is the “gold standard” for diagnosis of MH."
http://www.mhaus.org/index.cfm/fusea...DB27999FE5.cfm
3. What Drugs trigger MH and are Not Safe?
Inhaled General Anesthetics
Choroform (Trichloromethane, Methyltrichloride)
Desflurane
Enflurane
Halothane
Isoflurane
Methoxyflurane
Sevoflurane
Trichloroethylene
Xenon (Rarely Used)
Depolarizing Muscle Relaxants Trigger MH
Succinylcholine (Suxamethonium)
Also, soda lime, breathing tubes, and breathing bags should be new since trace amounts could be detrimental to the patient or changed out immediately if MH is suspected.
http://www.mhaus.org/index.cfm/fusea...theticList.cfm
4. Treatment is dantrolene, ventilation with 100% O2, and cooling measures if fever is present.
5. "MH or MH-like events however, have occurred in patients with underlying muscle diseases, such as muscular dystrophy and myotonia. Such patients typically display muscle weakness. MH has been linked to a rare disorder of muscle called Central Core disease and King Denborough Syndrome, a rarer muscle syndrome.
Additionally, patients with certain forms of muscular dystrophy may develop life-threatening disturbances and muscle destruction on exposure to the triggering agents for MH. The clinical event may resemble MH in many ways, but is not considered “true” MH. In patients with Duchenne muscular dystrophy, succinylcholine should always be avoided or rhabdomyolysis may occur. Potent volatile agents may produce rhabdomyolysis in time, but most believe that brief exposure is a small risk. Patients with muscle disorders should be carefully evaluated by their anesthesiologist prior to surgery.
Hypokalemic and hyperkalemic periodic paralysis are also associated with risk for MH. Hyperkalemic cardiac arrest may occur when MH-trigger agents are administered to muscular dystrophy patients.
Patients with osteogenesis imperfecta often develop fever during anesthesia. Myotonic patients will develop muscle rigidity with succinylcholine. There have been a few MH cases reported in patients with carnitine palmityl transferase deficiency and it is recommended to stay away from MH triggers in such patients. Neuroleptic Malignant Syndrome (NMS) is a syndrome that resembles MH but is precipitated by drugs acting centrally on dopaminergic pathways in the brain."
http://www.mhaus.org/index.cfm/fusea...edicalFAQs.cfm
Great Questions!!
Keep 'em coming I start CRNA school next month!
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