Airway management in patients with mucopolysaccharidoses: The progression toward difficult intubation

Abstract

Background:Patients 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. Aims:The 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. Methods: A retrospective review of MPS patients presenting for surgery fromJanuary 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. Results:One 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,, 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. Conclusions:As 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. K E Y W O R D Sairway management, anesthesia, bone marrow transplantation, enzyme replacement therapy, intubation, mucopolysaccharidoses | 621 MADOFF et Al.

Airway management and perioperative adverse events in children with mucopolysaccharidoses and mucolipidoses: A retrospective cohort study

“…In nonsyndromic children, the risk for perioperative critical events decreases with age . However, it has been proposed that due to disease progression during the natural history of MPS, the risk for intubation problems might paradoxically increase . Nevertheless, after adjustment for multiple confounders, we found that perioperative adverse events did not increase with age in our multivariate analysis.…”

“…Previous case series addressed the issue of airway techniques in MPS, but did not provide direct comparative assessments taking essential confounding factors into account …”

“…Respiratory complications are the most frequently reported adverse events . However, perioperative morbidity and mortality are dramatically higher among children with MPS/ML . In MPS type I, a perioperative morbidity of up to 30% and a 30‐day risk of death per procedure of approximately 0.7% have been reported.…”

“…[7][8][9] However, perioperative morbidity and mortality are dramatically higher among children with MPS/ ML. [4][5][6][9][10][11] In MPS type I, a perioperative morbidity of up to 30% 5,6 and a 30-day risk of death per procedure of approximately 0.7% 12,13 have been reported.…”

“…Airway management is challenging in children with MPS/ML. [4][5][6]10,11,13,14 Fiberoptic intubation through a supraglottic airway and videolaryngoscopy are frequently used as indirect intubation techniques with high success rates in children with difficult airways. 15 Although it is recognized that MPS/ML poses unique anesthesia-related challenges, the standard of care for airway management in these children still remains unclear, and the impact of the disease subtype and primary airway approach on perioperative adverse events is unknown.…”