Perioperative complications in patients diagnosed with mucopolysaccharidosis and the impact of enzyme replacement therapy followed by hematopoietic stem cell transplantation at early age

Megens, J.H.A.M.; Wit, Michel de; Hasselt, Peter M. van; Boelens, Jaap Jan; Werff, Desirée B. M. van der; Graaff, Jurgen C. de

doi:10.1111/pan.12370

Cited by 23 publications

(31 citation statements)

References 26 publications

(39 reference statements)

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“…The risk for perioperative adverse events was significantly higher in MPS type I and II than in MPS type III. Previous case series reported high event rates in MPS types I and II, but did not provide a direct comparison between MPS subtypes with an adjustment for relevant, confounding factors. Although case numbers for very rare disease are too low to draw clear conclusions, we found a high rate of perioperative adverse events in children with ML.…”

Section: Discussionmentioning

confidence: 97%

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

Section: Discussionmentioning

confidence: 99%

“…Registry data show that almost half of children with MPS I undergo two or more surgeries until the age of four . Many clinical manifestations of MPS/ML, especially progressive airway obstruction, restrictive lung disease, thorax deformity, adenotonsillar hypertrophy, obstructive and central sleep apnea, macroglossia, cervical spinal canal stenosis, dens hypoplasia, atlantoaxial instability, spinal cord compression, and skeletal dysplasia, have important implications for anesthesia …”

Section: Introductionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

“…[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.…”

Section: Introductionmentioning

confidence: 99%

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

Dohrmann

Muschol

Sehner

et al. 2020

Pediatric Anesthesia

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Background Children suffering from mucopolysaccharidoses (subtypes I, II, III, IV, VI, and VII) or mucolipidoses often require anesthesia, but are at high risk for perioperative adverse events. However, the impact of the disease subtype and the standard of care for airway management are still unclear. Aims This study aimed to assess independent risk factors for perioperative adverse events in individuals with mucopolysaccharidoses/mucolipidoses and to analyze the interaction with the primary airway technique implemented. Methods This retrospective study included individuals with mucopolysaccharidoses/mucolipidoses who underwent anesthesia at two high‐volume centers from 2002 to 2016. The data were analyzed in a multivariate hierarchical model, accounting for repeated anesthesia procedures within the same patient and for multiple events within a single anesthesia. Results Of 141 identified inpatients, 67 (63 mucopolysaccharidoses and 4 mucolipidoses) underwent 269 anesthesia procedures (study cases) for 353 surgical or diagnostic interventions. At least one perioperative adverse event occurred in 25.6% of the cases. The risk for perioperative adverse events was higher in mucopolysaccharidoses type I (OR 8.0 [1.5‐42.7]; P = .014) or type II (OR 8.8 [1.3‐58.6]; P = .025) than in type III. Fiberoptic intubation through a supraglottic airway was associated with the lowest risk for perioperative adverse events and lowest conversion rate. Direct laryngoscopy was associated with a significantly higher risk for airway management problems than indirect techniques (estimated event rates 47.8% vs 10.1%, OR 24.05 [5.20‐111.24]; P < .001). The risk for respiratory adverse events was significantly higher for supraglottic airway (22.6%; OR 31.53 [2.79‐355.88]; P = .001) and direct laryngoscopy (14.8%; OR 14.70 [1.32‐163.44]; P = .029) than for fiberoptic intubation through a supraglottic airway (2.1%). Conclusions The disease subtype and primary airway technique were the most important independent risk factors for perioperative adverse events. Our findings indicate that in MPS/ML children with predicted difficult airway indirect techniques should be favored for the first tracheal intubation attempt.

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Section: Discussionmentioning

confidence: 97%

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

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%