Batten disease and perioperative complications: a retrospective descriptive study

Area of Science:

• Pediatric Neurology
• Anesthesiology
• Rare Diseases

Background:

• Batten disease, a rare neurodegenerative disorder, primarily affects children.
• Perioperative complications in Batten disease patients are poorly understood, with limited data from case reports.
• Understanding anesthetic risks is crucial for managing this vulnerable population.

Purpose of the Study:

• To investigate perioperative complications in a large cohort of children with Batten disease.
• To characterize anesthetic management, including propofol use, in these patients.
• To assess the association between anesthetic agents and adverse perioperative events.

Main Methods:

• Retrospective descriptive study of patients with Batten disease (ICD10 E75.4) receiving anesthetic care.
• Data collected from electronic medical records between December 2014 and May 2019.
• Analysis of 93 anesthetic encounters in 35 pediatric patients.

Main Results:

• The most frequent adverse events were hypotension (6.5%) and bradycardia (7.5%), requiring medical treatment.
• Other events included oxygen desaturation, seizures, hypothermia, and agitation.
• No statistical association was found between intraoperative propofol use and adverse events (OR 1.03, 95% CI 0.42-2.51).

Conclusions:

• Most pediatric patients with Batten disease can be managed with few significant perioperative complications.
• Common adverse events include bradycardia, hypotension, and hypothermia.
• Routine avoidance of propofol in Batten disease patients is not supported by this data.