Lysosomal Storage Disorders

Individually rare, but as a group the incidence is approximately 1:8000.

Difficult airway management

Cardiac or respiratory failure

Hemorrhage

Difficult or failed intubation

Coexisting cardiac or respiratory disease

Difficult vascular access

Neurologic involvement and pt cooperation

Lysosomal storage disorders are rare, inherited, metabolic connective tissue disorders (often autosomal recessive) with variable anesthetic risk.

Caused by a variety of mutations in lysosomal enzymes in macrophages, impairing normal cellular debris scavenging.

Retained debris thus accumulates in various tissues throughout the body.

Includes MPS types I to VII and lipid storage disorders (including Tay-Sachs and Gaucher disease).

Depending on disease type and severity, many die in early childhood; others can expect to survive well into adulthood.

MPS most feared from anesthetic point of view primarily due to airway issues (thus the primary focus of this chapter is on these conditions).