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 |
Management | |
- 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. | |