The King-Denborough syndrome in the paediatric patient

Abstract

King Denborough syndrome is a rare myopathy with characteristic skeletal and craniofacial abnormalities and a susceptibility to malignant hyperthermia (MH). We describe the management of two children with a diagnosis of King Denborough syndrome. The first case is that of a 23-month-old term male infant requiring repair of a cleft palate. After flushing the anaesthetic machine, infusions of remifentanil at 0.25 μ/kg/min and propofol at 12 mg/kg/hr were commenced. These were subsequently changed to 0.40 μ/kg/min and 10 mg/kg/hour followed by 8 mg/kg/hour respectively. The case proceeded uneventfully and the patient was managed in the ward postoperatively. The second case was a three-year-old, 5.7kg, former 34-week premature male infant with a dysplastic kidney and hydronephrosis, and recurrent urinary tract infections requiring ureteric re-implantation and repair of the hypospadias. Cardiac echocardiography confirmed the atrial septal defect (ASD: secundum type). Remifentanil and propofol were also used for this case. The patient was fully awake within 10 minutes and managed in the ward postoperatively. These case reports describe the successful use of total intravenous anaesthesia with propofol and remifentanil in paediatric patients who are susceptible to MH.