Anesthesia in a patient with mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome)

We report a case of anesthesia during surgery to enlarge the foramen magnum in a pediatric patient with an extremely rare form of mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome). Airway control was unexpectedly easy, and intraoperative anesthetic management with total intravenous anesthesia went smoothly. However, the disease is progressive, with no guarantee that future anesthetic management of this patient will remain easy. If repeated surgery is required, thorough testing should be conducted over time to assess both airway and systemic complications. Nevertheless, we found that safe anesthetic management of affected patients is possible with anesthetics currently used in a clinical setting.