Anaesthesia Related Problems,Acute Malignant Hyperthermia Treatment

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CONGENITAL MYOPATHIES AND ANESTHESIA

Dr Sunita Goel
Lecturer in Anaesthesiology
BJ Wadia Children's Hospital

Continued...

ANESTHESIA RELATED PROBLEMS:

The various problems faced by us are:

Proneness to Malignant Hyperthermia
Cardiovascular problems ranging from arrhythmias to heart block
Respiratory failure
Problems due to other congenital abnormalities

Choice Of Anaesthesia:

Regional or General Malignant Hyperthermia: Malignant hyperthermia is a syndrome of a chain reaction of signs, which include a greatly increased body metabolism, muscle rigidity and high fever. Death or brain damage may result from cardiac arrest, internal haemorrhage or failure of other body systems. As all these myopathies are prone to malignant hyperthermia, it is important to know which are its prime triggers. All volatile anaesthetics - Halothane, Isoflurane, enflurane, Desflurane and Sevoflurane are triggers for malignant hyperthermia, while nitrous oxide is NOT. Amongst muscle relaxants Succinylcholine is a potent trigger. Thus for short surgeries, it is safer to use total intravenous anaesthesia using

Propofol and fentanyl or
Ketamine with supplements of nitrous oxide

Regional anaesthesia is still the anaesthesia of choice in these cases unless there are its contraindications. Various nerve blocks can be given for pain relief such as Lateral Cutaneous, Common peroneal, Brachial plexus blocks and Lower limb blocks.

Treatment Of Acute Malignant Hyperthermia:

STOP all volatile anaesthetics and Succinylcholine.
Hyperventilate with 100% oxygen.
Give 2.5mg/kg of dantrolene sodium IV.
Treat acidosis with bicarbonate.
Monitor core temperature - if hyperthermic, cool by nasogastric, rectal lavage and surface cooling.
Treat hyperkalemia with glucose-insulin, bicarbonate and calcium chloride. Avoid calcium channel blockers, most arrhythmias respond to correction of hyperkalemia and acidosis by hyperventilation, dantrolene and bicarbonate.
Continue dantrolene for at least 24 hours after control of episode
Ensure adequate urine output by hydration and diuretics since myoglobinuria is common
Follow coagulation profile- DIC may occur
suddenly cardiac arrest occurs, HYPERKALEMIA should be considered as the prime cause. Treatment is with glucose - insulin, sodium bicarbonate, calcium chloride and hyperventilation.
There should be a high index of suspicion for cardiac arrhythmias either brady or tachyarrhythmias which have to be treated accordingly. They can even have heart blocks which need treatment accordingly.

Last updated on 01-02-2005 Vol 2 Issue 2 Art # 7

How to cite this url

Goel S.Congenital Myopathies And Anesthesia. Pediatric Oncall [serial online] 2005 [cited 2005 February 1];2. Art # 7. Available from:

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