Adrenoleukodystrophy, X-linked type

General

• Two types: X-linked, and Neonatal (autosomal recessive)

Airway

• Hypotonia/airway obstruction

Respiratory

• Airway obstruction

Gastrointestinal

• Liver function abnl
• GERD

Endocrine

• Hyperpigmentation due to ACTH over secretion

Neurological

• Motor/mental retardation
• Hypotonia
• Seizures
• Spastic paraplegia

Musculoskeletal

• Hypotonia

Considerations

• Premedication/hypotonia bad combination
• Perioperative aspiration
• Possible excess K+ release with succinylcholine
• Positioning nightmare
• Post-anesthetic airway obstruction
• Liver induction from anticonvulsants
• Perioperative stress-dose steroids
• Avoid dopaminergic blocker drugs
• Ondansetron is your friend!

Links

• Adrenomyeloneuropathy
• Metachromatic leukodystrophy
• Krabbe disease
• Canavan disease
• Pelizaeus-Merzbacher disease
• Alexander disease