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

Scaravilli, Vittorio; Zanella, Alberto; Ciceri, Valentina; Bosatra, Mariagrazia; Flandoli, Claudia; Bruna, Alessia La; Sosio, Simone; Parini, Rossella; Gasperini, Serena; Artigas, Antonio; Moretto, Alessandra

doi:10.1111/pan.13379

Cited by 12 publications

(21 citation statements)

References 21 publications

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“…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: 66%

“…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.…”

Section: Discussionmentioning

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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“…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: 66%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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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“…In particular, patients with MPS type II have increased incidence of difficult airway compared to the general patient population and require repeated MRI to follow the progression of the disease . Moreover, upper airway obstruction becomes more troublesome as disease progresses and with increasing age . Therefore, patients with MPS II require sedative drugs that provide reliable levels of sedation without causing airway obstruction.…”

Section: Discussionmentioning

confidence: 99%

A retrospective comparison of propofol to dexmedetomidine for pediatric magnetic resonance imaging sedation in patients with mucopolysaccharidosis type II

Kang

Shin

Gil

et al. 2018

Pediatric Anesthesia

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Summary Background Mucopolysaccharidosis type II patients are reported to have an elevated incidence of difficult airway. Propofol is a commonly used sedative for magnetic resonance imaging in pediatric patients, but patients who receive it may exhibit dose‐dependent upper airway obstruction and respiratory depression. Dexmedetomidine also provides adequate procedural sedation with a relatively low risk of airway obstruction. Accordingly, we introduced the use of dexmedetomidine in our practice to reduce the risk of airway obstruction during magnetic resonance imaging procedures. Aims The aim of this study was to evaluate the incidence of artificial airway interventions in patients sedated with propofol and compare it to that in patients sedated with dexmedetomidine in patients with mucopolysaccharidosis type II during magnetic resonance imaging procedures. Methods All mucopolysaccharidosis type II patients undergoing magnetic resonance imaging at our institution between April 2014 and February 2018 were included in this study. The patients were divided into two groups according to whether they were managed before and after the introduction of dexmedetomidine: those who were sedated with propofol (group P) and those who were sedated with dexmedetomidine (group D). Results Forty‐six sedations were performed in 27 patients. Artificial airway interventions were significantly more frequent during propofol‐based than dexmedetomidine‐based sedation: 14 of 32 (43.8%) in group P and 1 of 14 (7.1%) in group D (odds ratio, 10.11; 95% confidence interval, 1.18‐86.85; P = 0.018). Time to awake and time to discharge were similar between groups. Changes in hemodynamic variables also did not significantly differ between groups. Conclusion Dexmedetomidine provides an adequate level of sedation and is associated with lower rates of artificial airway interventions compared to propofol. Therefore, dexmedetomidine may offer advantages for preserving the native airway compared to propofol when administered during magnetic resonance imaging scans in patients with mucopolysaccharidosis type II.

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Successful spinal anesthesia in a patient with mucopolysaccharidosis type I under femoral fracture reduction and external fixation

Sun

Jian-min

Zhao

2019

Pediatric Investigation

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Introduction Mucopolysaccharidosis (MPS) is an inherited lysosomal storage disorders with glycosaminoglycans accumulation in tissues. MPS patients undergoing intratracheal intubation anesthesia show high mortality, with serious anesthetic complications associated with airway thickness and narrow. The regional anesthesia is a useful alternative to general anesthesia, however performing spinal anesthesia in MPS patients are rarely documented. Case presentation We report a case of a boy with MPS type I undergoing femoral reduction and external fixation under spinal anesthesia in combination with sevoflurane inhalational induction, getting rid of difficulties associated with intubation. Conclusion Sevoflurane inhalational induction with spinal anesthesia without tracheal intubation is a safe choice for MPS I patient.

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

Abstract: 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.

Cited by 12 publications

References 21 publications

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

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

A retrospective comparison of propofol to dexmedetomidine for pediatric magnetic resonance imaging sedation in patients with mucopolysaccharidosis type II

Successful spinal anesthesia in a patient with mucopolysaccharidosis type I under femoral fracture reduction and external fixation

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