General Anesthesia for a Child With Sjögren-Larsson Syndrome

This case report describes the dental treatment performed under general anesthesia for a boy with Sjögren-Larsson syndrome (SLS), a rare autosomal recessive genetic disorder. The main symptoms of SLS are congenital ichthyosis, limb paraplegia or tetraplegia, and intellectual disability. The main concerns regarding the administration of general anesthesia to patients with SLS include difficulty with adhesion of the electrocardiogram electrodes and intravenous (IV) catheter dressings due to ichthyosis and heat intolerance secondary to hypohidrosis. Although it was first described approximately 70 years ago, there is little in the existing literature on general anesthesia for patients with SLS. We administered deep sedation/general anesthesia using continuous IV infusions of propofol and remifentanil along with a laryngeal mask airway in a 6-year-old boy with SLS to successfully obtain radiographic studies and extract a supernumerary tooth. No problems occurred other than difficulties with adhesives and soft tissue obstruction of the airway.