A patient with maple syrup urine disease (MSUD) requires careful management during the perioperative period. In general procedures are best performed by a medical team with experience in managing these patients and adequate resources.

Enzymatic defect: branched-chain alpha ketoacid dehydrogenase


Untreated patients have accumulation of leucine, isoleucine, valine and amino acid metabolites.


Triggers for metabolic decompensation: infection, dehydration, pain, fever, vomiting, surgery, trauma, inflammation


Metabolic decompensation may include:

(1) ketoacidosis

(2) hypoglycemia

(3) hyperammonemia

(4) dehydration

(5) cerebral edema with neurologic decompensation, apnea and seizures


Other problems may include:

(1) iatrogenic electrolyte abnormalities (hypokalemia, hypophosphatemia)

(2) malnutrition with nutritional deficiencies


Issues in the perioperative period:

(1) Avoid anything that may increase the intracranial pressure.

(2) Avoid any drugs that may precipitate a seizure.

(3) Maintain normovolemia and normoglycemia, avoiding fluid overload.

(4) Avoid perioperative nausea and vomiting with antiemetics.

(5) Metabolic monitoring for evidence of ketoacidosis, hyperammonemia, or hypoglycemia.

(6) Avoid or minimize the use of central venous catheters.

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