Safety of anesthesia for children with mucopolysaccharidoses: A retrospective analysis of 54 patients.

Background: Complications are common during anesthesia for patients with mucopolysaccharidoses. San Gerardo Hospital (Italy) is a reference center for mucopolysaccharidoses with a dedicated pediatric anesthesia service.

Aims: This study aims to evaluate the safety of anesthesia for mucopolysaccharidoses patients, describe their anesthetic management at our institution, and assess risk factors for complications.

Methods: The anesthetic charts of mucopolysaccharidoses patients admitted from January 1999 to December 2014 were retrospectively analyzed. We retrieved patients' demographics; location and type of the procedure; anesthetic approach airway management and occurrence of difficult intubation and complications and outcome at hospital discharge. A generalized linear mixed model was performed to assess risk factors for complications and difficult intubation.

Results: Fifty-four consecutive children were included. The anesthetic charts of 232 procedures (52% radio-diagnostics, 15% orthopedics, 15% ear-nose-throat surgery, 10% neurosurgery, and 8% general surgery) were analyzed. Each patient underwent a median of 4 (1-6) procedures. The median age at the first procedure was 2 (1-5), and overall age was 5 (2-8) years old. One hundred and twenty-five (54%) procedures were performed in remote locations. General anesthesia was utilized for 100 (43%) procedures. No death was recorded. Twenty-one (9%) procedures had respiratory complications. Remote location anesthesia was associated with increased risk for complications (odds ratio 5.405 [1.355-28.571], P = .016). All planned intubations (n = 65) were successful. Nineteen (29%) of those were defined difficult. All emergency intubations (n = 3) failed and were rescued by laryngeal mask airways. Older age was associated with an increased risk of difficult intubation (OR 1.200 [1.019-1.436], P = .028).

Conclusion: Patients with mucopolysaccharidoses are at high risk for anesthesia-related complications. Remote location anesthesia is associated with increased risk for complications, and older age is associated with increased risk for difficult intubation.