Combined mucopolysaccharidosis type VI and congenital adrenal hyperplasia in a child: Anesthetic considerations

Abstract

We present a child posted for magnetic resonance imaging of brain under general anesthesia with the rare combination of mucopolysachharidosis type VI and congenital adrenal hyperplasia. The presence of both these disorders has important anesthetic implications. The pathophysiology of this rare combination of disease is reviewed with emphasis on the anesthesia management.

Keywords

• Congenital adrenal hyperplasia
• mucopolysaccharidosis
• steroid supplementation
• stress dose