Adrenoleukodystrophy, Neonatal

General

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

Airway

• High arched palate

Gastrointestinal

• Liver function abnl
• GERD

Endocrine

• Hyperpigmentation due to ACTH oversecretion

Neurological

• Mental retardation
• Motor delay
• Seizures

Musculoskeletal

• Motor delay

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

• Zellweger syndrome
• Refsum disease