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

Incidence estimated at 1 : 30,000-400,000 live births. Newborn screening in many countries. 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  

-         pauci or asymptomatic form, known as the adolescent or adult form: muscle cramps in case of prolonged fasting or effort. That possibility should be kept 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) with rhabdomyolysis. Cases of total IV anesthesia with ketamine, lidocaine and remifentanil have been described.

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-coenzyme 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 
• Tanaka A, Cai T, Platten M, Tollinche LE, DeJoy SJ.
  Anesthetic management and neuromonitoring in a patient with Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency undergoing scoliosis surgery: a case report and review of literature.
  Case Reports in Anesthesiology 2024 ; 1050279, doi.org/10.1155/2024/1050279

Updated: April 2024