Conducta perioperatoria en un paciente con síndrome de San Filippo sometido a exodoncia múltiple

Abstract

San Filippo syndrome is a hereditary lysosomal disorder resulting in the accumulation of mucopolysaccharides (mucopolysaccharidosis type II). A deficit in the enzyme required to break down heparan sulfate leads to its deposition in the connective tissue of many organs, particularly the brain, liver, heart, and spleen. The first symptoms-including mental deterioration, dimorphism, and behavioral changes such as hyperkinesis and aggressivity-present in childhood. Because this rare disorder has many anesthetic implications, we report the case of a 20-year-old man with San Filippo syndrome who underwent multiple tooth extraction under general combined anesthesia and a block of the second and third branches of the trigeminal nerve. This anesthetic combination provided satisfactory surgical conditions and recovery from anesthesia was rapid. Following surgery the patient developed a respiratory infection that led to severe respiratory failure and death.