• Lauren U Madoff, Anna Kordun, and Joseph P Cravero.
• Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, Massachusetts.
• Paediatr Anaesth. 2019 Jun 1; 29 (6): 620-627.

BackgroundPatients with mucopolysaccharidoses (MPS) have physical changes to their airways over time. Due to the natural progression of their disease, these patients become more difficult to intubate as they get older.AimsThe aims of this study were to evaluate the difficulty of airway management in MPS patients over time, and to evaluate the effect of bone marrow transplant and/or enzyme replacement therapy on airway difficulty.MethodsA retrospective review of MPS patients presenting for surgery from January 2012 to May 2018 was performed. Patients were assigned to groups based on their ages at the time of surgery, number of intubation attempts, equipment used for intubation, difficulty of mask ventilation, and difficulty of laryngeal mask airway placement. The same designations were applied to patients with a history of a bone marrow transplant (BMT) and/or enzyme replacement therapy, and they were compared to patients of similar ages who had received no treatment. Logistic regression was used to determine the odds of difficult intubation.ResultsOne hundred and twenty-eight anesthetic records were reviewed. In 27 cases, the patient had a BMT and in 54 cases, the patient had received enzyme replacement therapy. Adults (18 years and older) had the highest likelihood of difficult intubation (OR 13.44, CI 1.45-124.86, P = 0.022). Mask ventilation and laryngeal mask airway placement were not significantly more difficult in any age groups. Bone marrow transplant did not improve airway management. A history of enzyme replacement therapy was associated with an increased risk of difficult intubation in patients under the age of 12.ConclusionsAs patients with MPS get older, there is a progression toward difficult intubation. Mask ventilation and laryngeal mask airway placement does not become more difficult with age. Bone marrow transplantation did not affect airway difficulty in our population, while enzyme replacement therapy was associated with difficult intubations in younger patients.© 2019 John Wiley & Sons Ltd.

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