Perioperative Management of a Patient With Glutaric Aciduria

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. Due to the progressive neurologic effects with spasticity and orthopedic deformities, surgical and anesthetic cares are frequently required. We present a 13-year-old girl with glutaric acidemia type 1 who required anesthetic care for posterior spinal fusion. Previous reports of anesthetic care for these patients are reviewed, the end-organ involvement is discussed, and options for anesthetic care are presented.




J Med Cases. 2015;6(6):257-263
doi: http://dx.doi.org/10.14740/jmc2096w