Perioperative management of a patient with glutaric aciduria: a case report and literature review

ABSTRACT

Glutaric aciduria type-1 [GA-1] is an autosomal recessive metabolic disorder due to the deficiency of the enzyme glutaryl-CoA dehydrogenase. The enzymatic defect leads to secondary damage to the central nervous system due to the accumulation of glutaric acid. Progressive neurologic effects with spasticity and orthopedic deformities necessitate frequent surgical and anesthetic care. We present a 13-year-old girl with glutaric academia type-1 who required anesthetic care for posterior spinal fusion. Previous reports of anesthetic care for these patients are reviewed, the end-organ involvement discussed, and options for anesthetic care presented