Anaesthetic management in syndromic paediatric patients undergoing dental procedures: A case series

Rare congenital disease patients are a challenge to the anaesthesiologist especially when conducted under general anaesthesia. A thorough clinical examination and accurate history regarding previous anaesthetic management needs to be sought. This is a case series of paediatric patients who presented with congenital rubella, West syndrome, Down’s syndrome, mucopolysaccharidosis (MPS) and Pelizaeus-Merzbacher disease (PMD) for dental procedures. The key to managing West syndrome patients is to continue antiepileptic drugs perioperatively and avoiding epileptogenic drugs. Patients with PMD have poor pharyngeal muscle control and subsequent airway complications. Down’s syndrome is associated with multi-system comorbidities and atlantoaxial instability. Accumulation of glycosaminoglycans results in anatomical abnormalities and organ dysfunction in MPS patients. Congenital rubella syndrome poses challenges like unanticipated difficult airway and uncorrected cardiac defects.