Acronym for Very Long Chain-Acyl-coenzyme A dehydrogenase Deficiency.

Rare. Mitochondrial metabolic disease. Autosomal recessive transmission of a deficiency of the enzyme that initiates the β-oxidation of  very long chain fatty acids (C14 - 20). 

Clinical presentations are variable according to the level of active enzyme:

-         neonatal, severe form: hypertrophic cardiomyopathy (reversible if adequate treatment is quickly introduced), metabolic acidosis, hypoglycemia, myopathy (lipid clusters at the biopsy)

-         infantile form: outbreaks of hypoglycemic coma without ketonemia in case of fasting or fever, acute fatty liver  disease (Reye-like syndrome), sudden infant death syndrome, epilepsy with organic aciduria  

-         little or asymptomatic form, known as the adolescent or adult form: muscle cramps in case of prolonged fasting or effort. Keep that possibility in mind in case of a rhabdomyolysis or a post-anesthetic coma.

Treatment: avoid any catabolism with a diet rich in slow carbohydrates and with a low content in fat (only short or medium chain fatty acids are allowed) and proteins; exogenous supply in carnitine (and occasionally riboflavin) is associated to correct a primary or secondary deficiency

Anesthetic implications: 

preoperative echocardiography; basal level of CPK and renal function;  glucose 10% IV administration, to avoid lipolysis, at the start of the fasting period: 6 mg/kg/h in children (8 mg/kg/h in case of stress) and 2 mg/kg/h for adults [in case of hyperglycemia, add insulin rather than decrease the intake of glucose]; the usual dose of carnitine on the morning of the intervention; monitor blood glucose levels.

Anxiolytic premedication to avoid undue stress.

Use of propofol ? Use preferably a solution with medium chain fatty acids (Propofol® and Diprivan® formulations  mainly contain long chain fatty acids) and avoid using it in continuous infusion because propofol inhibits complex II of the respiratory chain and the  intramitochondrial transport of long-chain fatty acids by carnitine. Increased risk of 'propofol infusion syndrome' (PRIS) ?

No risk of rhabdomyolysis with halogenated agents. Avoid succinylcholine: risk of rhabdomyolysis. Tourniquets ?

Caution with NSAIDs.

References : 

• Steiner LA, Studer W, Baumgarten ER, Frei FJ. 
  Perioperative management of a child with very-long-chain acyl-coenzyme A dehydrogenase deficiency. 
  Paediatr Anaesth 2002; 12: 187-91.
• Roe CR, Wiltse HE, Sweetman L, Alvarado LL. 
  Death caused by perioperative fasting and sedation in a child with unrecognized very long chain acyl-coenzymz A dehydrogenase deficiency. 
  Pediatrics 2000; 136: 397-9.
• Iwata K, Tanabe K, Sugiyama Y, Tanaka M et al. 
  Anesthetic management for a patient with very-long-chain-acyl-coenzyme A dehydrogenase deficiency. 
  J Anesth 2012; 26: 957-8.
• Redshaw C, Stewart C. 
  Anesthetic agents in patients with very long-chain acyl-coenzyme A dehydrogenase deficiency: a literature review. 
  Pediatr Anesth 2014; 24: 1115-9.
• Hess AS, Rice GM, Jochman JD, Muldowney BL.
  Volatile anesthesia for a pediatric patient with very long-chain acyl-coenzyme A dehydrogenase deficiency : a case report.
  Pediatr Anesth 2018 ; 28 : 296-7

Updated: February 2018