Anesthetic management of an adult patient with childhood x-linked adrenoleukodystrophy

Abstract

X-linked adrenoleukodystrophy is a disorder of peroxisomal fatty acid beta-oxidation causing the accumulation of very long chain fatty acids (VLCFA) in tissues. The hallmarks of the disease are CNS demyelination and primary adrenal insufficiency. The anesthetic considerations include mental retardation, seizure disorder, impaired adrenocortical function, immunosuppression, risk of iatrogenic fractures, hypotonia and delayed awakening. The anesthetic plan should be case specific. Pre-operative sedation should be avoided because of hypotonia of pharyngeal muscles. Corticosteroids should be  given intra-operatively. We anesthetized our case using titrated doses of short acting intravenous and inhalational agents. We secured the airway with i-gel. Few cases have been reported in literature. Still there exists no established anesthetic management plan for these patients. To the best of our knowledge, this is the first reported use of titrated doses of anesthetic agents under i-gel supraglottic airway device for a case of x-linked adrenoleukodystrophy posted for orchidectomy, and with a favorable outcome.