If physicians pay close attention to the comorbidities associated with adrenoleukodystrophy (ALD) — particularly gastroesophageal reflux disease (GERD), adrenal insufficiency, and seizures — children with the disease may safely undergo anesthesia during therapeutic or surgical procedures without an increased risk of complications, a study found.
This also is true for those managing their symptoms by taking regular doses of corticosteroids, known to trigger muscle contractions and acid reflux, the findings show.
The study’s findings were reported in “Anesthetic management of pediatric patients diagnosed with X‐linked adrenoleukodystrophy: A single‐center experience,” published in the journal Pediatric Anesthesia.
ALD is a rare, genetic, neurodegenerative disorder that can cause a wide range of symptoms, including changes in motor skills, learning, and behavior, weight loss, and weakness.
This wide range of symptoms, and other concurrent medical conditions, can complicate the use of anesthesia during surgery. Such complications can be further exacerbated by the use of corticosteroids, since these medications can trigger acid reflux in the esophagus, muscle contractions, or loss of muscle tone. All of these contribute in increasing the risk of aspiration, or breathing liquids into the airways, during anesthesia.
However, there is a lack of reports investigating the use of anesthetics in pediatric patients with ALD.
To learn more, a team of researchers from the University of Minnesota Masonic Children’s Hospital, in the U.S., examined in-house hospital records of children diagnosed with ALD who underwent either surgery or diagnostic and/or therapeutic procedures between 2014 and 2016 that required the use of anesthetics.
The study included data from 38 children with ALD, between the ages of two months and 15 years, who received anesthetics 166 times over the course of three years. Each child had between one and 11 medical procedures that required the use of anesthetic agents, including sevoflurane, isoflurane, propofol, and ketamine.
Most procedures were performed on patients with severe systemic disease (73.5%), and in a sedation unit (75.9%), while the remainder occurred in the main operating rooms. In most procedures (86.1%), the children received a total intravenous anesthetic — use of intravenous, or into-the-vein therapies for induction and maintenance of anesthesia — with natural airway ventilation.
Among the 166 procedures analyzed, 145 (87.3%) were performed on children with adrenal insufficiency — a condition in which the adrenal glands are unable to produce normal amounts of certain hormones, including cortisol — who were taking regular doses of corticosteroids.
In 103 procedures (62%), patients were also taking antiepileptic medications as part of their routine maintenance treatment. GERD was reported in 11 (6%) of the procedures analyzed, and stress-dose steroids were administered in 117 (70.5%) of the performed anesthetics procedures.
The rate of complications associated with anesthesia was low (6%). Complications included an episode of agitation following surgery in one patient, side effects caused by a different treatment in another child, and pneumonia in a third patient. In this last case, because the first signs of lung infection were already present before the procedure, the likelihood of the event being aspiration pneumonia was low, according to the team.
No complications related to adrenal insufficiency and corticosteroid use were reported. Stress-dose steroids were well-tolerated, and no anesthesia-related deaths were reported.
According to the team, the results show that “with appropriate attention to the associated comorbidities, specifically adrenal insufficiency, GERD and seizure disorder, [ALD] patients can safely undergo anesthesia with a variety of techniques and anesthetic medications.”
“With the availability of skilled pediatric anesthesia care, children with X-linked adrenoleukodystrophy can undergo procedures under anesthesia in sedation units and regular operating rooms with low overall anesthesia risk,” the team concluded.
