DOI: 10.36347/sjmcr.2014.v02i06.013

Pages: 391-393

Ornithine transcarbamylase deficiency (OTCD) is an X-linked, inherited condition within the urea cycle characterized by failure of ammonia detoxification and urea formation. This may lead to hyperammonemic encephalopathy that, if uncontrolled, results in brain injury and death. Individuals susceptible to this disorder are at risk for hyperammonemic crises if a catabolic state is precipitated. We present an adult female patient with OTCD who underwent L5-S1 transforaminal lumbar interbody fusion. Since the NPO status and the stress of surgery predisposed the body to a catabolic state, the anesthetic management of this patient aimed at avoiding conditions that could stimulate protein catabolism.