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Table 13 Considerations and management of general anesthesia

From: Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia

Elective procedures Emergency operations and major procedures (>30 minutes)
- Check that the child is healthy 48 hours before surgery. If not, postpone the operation. - Seek specialist advice
- Last metabolic work-up must have taken place less than 3 months - On admission
  • Check plasma ammonia, pH, blood gases
  • If ammonia >100 μmol/l, pH < 7.30 or base deficit > 10 mmol/l) or the child is unwell, cancel elective procedures and/or seek specialist advice
- Stop feeds according to minimal anesthetic requirements and replace by clear carbohydrate containing fluids or intravenous 10% glucose and appropriate electrolytes at a rate allowing to block lipolysis:
 • 8-10 mg/kg/min for neonates and infants
 • 6-7 mg/kg/min for children
 • 5-6 mg/kg/min for adolescents
 • 4-5 mg/kg/min for adults
- For B12 responsive patients administer hydroxocobalamin 1mg parenterally 24 h before and on the day of the procedure
- Add intravenous L-carnitine: 100 mg/kg/ day (max. 12 g for adults)
The infusion and treatment should be maintained during the whole surgical procedure. The use of intravenous lipid solution may be considered for longer procedures (1 to 2 g/kg/day IV).
Post-operative management
- Following the operation, feed depending on metabolic stability at the time you would feed any other patient following an equivalent procedure.
- Give whatever oral medicines the patient may be due at the same time
- Discontinue the intravenous infusion ONLY after the child has been seen to tolerate food.
- Remove the cannula ONLY when normal feeding has been achieved.
- If recovery is delayed or complicated by vomiting:
 • check blood gases, electrolytes and ammonia
 • consider using antiemetic drugs (ondansetron, avoid metoclopramide)
 • continue glucose and L-carnitine infusion and add IV lipids (1-2 g/kg/d)
 • IV amino acids should be added if the patient cannot be fed (0.5-0.8 g/kg/d)
- Discharge ONLY after full recovery and normal metabolic results. This will often be the following day.