Dexmedetomidine, Ketamine, and Midazolam Used for IV Moderate Sedation in a Pediatric Patient With Asplenia Syndrome

Abstract

Asplenia syndrome is a rare congenital abnormality associated with a nonfunctional or no spleen, cardiac defects, and abdominal organ malpositioning. This report describes an 11-year-old boy diagnosed with asplenia syndrome and pulmonary hypertension who underwent intravenous (IV) moderate sedation for dental treatment. He presented with cyanosis secondary to a single right ventricle and bilateral origin of the great vessels and had undergone multiple cardiac interventions including a Glenn procedure. Owing to his high pulmonary vascular resistance, a Fontan procedure could not be performed, so he was maintained on oral medications and supplemental oxygen. Conventional dental treatment was difficult due to his intellectual disability, so IV moderate sedation was utilized with midazolam (1.5 mg) to start, followed by a loading infusion of dexmedetomidine (DEX; 1 mcg/kg) for 10 minutes and a maintenance infusion at 0.5 mcg/kg/h. Five minutes after starting the DEX infusion, a ketamine (25 mg) bolus was administered. IV ketamine (12.5 mg) was administered 25 and 50 minutes after procedure initiation, which was completed uneventfully. The combination of midazolam, DEX, and ketamine may provide safe IV moderate sedation for pediatric patients with asplenia syndrome and pulmonary hypertension.