Perioperative care of a child with Sanfilippo syndrome for ade- notonsillectomy

Abstract

The mucopolysaccharidoses (MPS) are a group of inherited conditions with lysosomal enzymatic deficiencies re- sulting in the inability to metabolize glycosaminoglycans. The MPS are separated into 7 major types, based on the underlying enzymatic defect. MPS type III or Sanfil- ippo syndrome is an autosomal recessive disorder, resulting from the congenital deficiency of one of the four enzymes involved in the catabolism of glycosaminoglycan heparin sulfate. Given the multisystem involvement including the airway, respiratory, cardiovascular, and cen- tral nervous systems, patients with MPS are at increased risk during anesthetic care or procedural sedation related to airway obstruction, excessive airway secretions, skeletal restriction of cervical spine movement, infiltration of airway structures, neurologic impairment, and frequent respiratory infections. We present a 5-year-old child with MPS type III (Sanfilippo syndrome) who required anesthetic care during adenotonsillectomy. The perioperative implications of Sanfilippo syndrome are presented, previous reports of anesthetic care reviewed, and options for anesthetic care discussed.

Keywords

• mucopolysaccharidosis type iii
• sanfilippo syndrome