J Korean Dent Soc Anesthesiol.  2010 Jun;10(1):45-49. 10.17245/jkdsa.2010.10.1.45.

Ambulatory Anesthesia Management of the Patient with Adrenoleukodystrophy for Dental Treatment: A Case Report

• Seo KS
• Bak S
• Shin TJ
• Kim HJ

• KMID: 2345096
• DOI: http://doi.org/10.17245/jkdsa.2010.10.1.45

Abstract

Adrenoleukodystrophy (ALD) is a rare X-linked genetic disorder associated with various central nervous system problems and adrenal insufficiency. It is common in childhood and symptoms include loss of previously acquired neurological abilities, seizures, ataxia, Addison's disease as well as degeneration of visual and auditory function. These children frequently require anesthesia during imagining procedure such as MRI or during some surgical procedures like gastrostomy. There is special need for careful management because of numerous anesthetic challenges like difficulty in cooperation, seizure disorders, life-threatening airway obstruction, copious oral secretion and possibility of aspiration. In addition, adrenal involvement and hypofunction must be considered for safe anesthesia management. We report a successful anesthetic management in a patient with ALD for dental procedures.

Keyword

Adrenoleukodystrophy (ALD) Dental treatment; General Anesthesia; Cooperation difficulty

MeSH Terms

Addison Disease
Adrenal Insufficiency
Adrenoleukodystrophy*
Airway Obstruction
Anesthesia*
Anesthesia, General
Ataxia
Central Nervous System
Child
Epilepsy
Gastrostomy
Humans
Magnetic Resonance Imaging
Seizures