Anesthetic Management in a Child With Citrullinemia: A Case Report

INTRODUCTION: Citrullinemia is a defect in the urea cycle that causes ammonia to accumulate in the blood. We describe the anesthetic management of a patient with citrullinemia, who experienced an unexpected 10 day hospital admission. CASE PRESENTATION: We anesthetized a 3.5 year-old boy with citrullinemia who was scheduled for a dentistry procedure. Perioperative precautions included minimizing fasting period, hypothermia prevention, relieving anxiety and pain, perioperative infusion of D10W and benzoate sodium, as well as a pediatric endocrinology consultation. The operation lasted 4 hours and its course was uneventful. He had a delayed recovery from anesthesia and was discharged from hospital after 10 days. CONCLUSIONS: General anesthesia and surgery can be a risk factor for exacerbating the course of the disease in patients with citrullinemia. It appears that administering short acting sedatives and analgesics in these patients would be of more benefit. Further studies are required to identify a safe method for anesthesia in citrullinemia.