Anesthetic Management of the Surgical Correction of Idiopathic Scoliosis in a Teenager With Ornithine Transcarbamalyse Deficiency

Ornithine transcarbamylase (OTC) deficiency is the most common genetic disorder of the urea cycle. These disorders are characterized by an inability to metabolize ammonia into urea, leading to hyperammonemia with variable physiological consequences and presenting important anesthetic challenges, especially the perioperative prevention of hyperammonemia and management of its consequences, should it occur. Idiopathic scoliosis (IS) is the most common spinal deformity requiring surgical treatment. This paper presents the case of a 16-year-old female with OTC deficiency who underwent spinal fusion for IS. The chosen anesthetic strategy was combined anesthesia with total intravenous general anesthesia using target-controlled infusion pumps, an erector spinae plane block (ESPB), and a multi-pronged approach to ensure metabolic control while avoiding hyperammonemia. The existing literature regarding major surgery in patients with OTC deficiency is sparse, and this paper provides one of the first case reports of a scoliosis correction surgery, as well as one of the first descriptions of prolonged propofol infusion and locoregional anesthesia with an erector spinae plane block in this context.