Anesthetic Management for Fracture Head of Radius in a Child with Glutaric Aciduria type-1

Abstract

Glutaric aciduria Type 1 (GA-1) is an autosomal recessive metabolic disorder that results from deficiency of enzyme glutaryl-CoA dehydrogenase. This gives rise to elevated neurotoxic glutaric acid and 3-hydroxyglutaric acid as well as nontoxic glutarylcarnitine in body fluids. The enzyme defect leads to secondary damage to central nervous system due to the accumulation of glutaric acid. Approximately 90% people will develop the neurological disease during a finite period of brain development (3-36 months) following an acute encephalopathic crisis often precipitated by gastroenteritis, immunization, surgical intervention, and intercurrent febrile illness. GA-1 can also develop insidiously without clinically apparent crisis in 10%-20% of the patients. We present a 10-year-old male child with GA-1 who required anesthetic care for fracture (left) neck of radius. Strategies for anesthetic management should include prevention of hypoglycemia, dehydration, electrolyte imbalance, and sufficient analgesia to prevent surgical stress.

Keywords: 3-Hydroxyglutaric acid; encephalopathic crisis; glutaric acid; glutaryl CoA dehydrogenase; glutarylcarnitine.