Anaesthesia for a child with adrenoleukodystrophy: A case report and review of the literature

• Main Authors: Sien Hui Tan, Vivienne HY Ng
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2014-01-01
• Series: Indian Journal of Anaesthesia
• Subjects: Adrenoleukodystrophy; anaesthesia; fatty acids; X-linked cerebral adrenoleukodystrophy
• Online Access: http://www.ijaweb.org/article.asp?issn=0019-5049;year=2014;volume=58;issue=1;spage=63;epage=65;aulast=Tan

We present a 9-year-old boy with X-linked cerebral adrenoleukodystrophy (X-linked ALD) and previous umbilical cord transplant who required general anaesthesia. An anaesthetic plan for each individual should be tailored to ensure the best possible anaesthetic care for these patients. The anaesthetic considerations include mental retardation, seizure disorder, hypotonia, liver function abnormalities, gastro-oesophageal reflux, impaired adrenocortical function and immunosuppression. Pre-operative sedation should be avoided because of hypotonia of the pharyngeal muscles. Anti-convulsants are continued, and potentially epileptogenic anaesthetic agents are avoided. The patient was intubated using a modified rapid sequence induction with a head up position of 30 degrees. Four other cases have been reported in literature. Nevertheless, there is still no established anaesthetic management for these patients, and total intravenous anaesthesia can be considered as a safe and alternative method of anaesthesia. To the best of our knowledge, this is the first reported use of total intravenous anaesthesia with propofol and remifentanil in a case of cerebral adrenoleukodystrophy, and with a favourable outcome.