Anesthetic care for the child with Morquio syndrome: general versus regional anesthesia

Abstract

Morquio syndrome is a subtype of the mucopolysaccharidoses (MPS IV) characterized by the intracellular accumulation of keratin sulfate. Anesthetic issues related to the primary disease process include infiltration of tissues with keratin sulfate leading to distortion of upper airway anatomy with difficult or impossible endotracheal intubation, infiltration of the cervical spine with odontoid hypoplasia placing these patients at risk for atlanto-axial subluxation and quadriparesis, as well as alterations in cardiorespiratory function. Two children with Morquio syndrome who required anesthetic care during lower extremity orthopedic procedures are presented. The anesthetic implications of Morquio syndrome are reviewed. General anesthesia was used in one case, whereas continuous spinal anesthesia, which was used in the second case, is reported for the first time in a child with mucopolysaccharidoses.