Abstract:A combination of dexmedetomidine and propofol provided effective general anesthesia with a native airway during the procedures. Although upper airway obstruction was noted, it resolved with simple airway maneuvers without further airway intervention.
“…Furthermore, our literature review suggests that mask ventilation is less likely encountered in MPS III patients. Kamata et al [13] reported no difficulties in mask ventilation in a cohort of MPS III patients, albeit mild upper airway obstruction was noted during 14 procedures (33%), which was resolved with simple head/jaw maneuvering/positioning. Another review of a large MPS III series reported no problems with mask ventilation in 86 anesthetics [12].…”
Section: Discussionmentioning
confidence: 95%
“…The systematic review of the literature identified nine case series, and their airway management is summarized in Table 2 [5,[8][9][10][11][12][13][14][15]. In addition, we identified 27 individual case reports and these patients' characteristics and their airway management is summarized in Tables 3 and 4 [7 -10,16-38].…”
Mucopolysaccharidoses (MPS) are rare, inherited, lysosomal storage diseases that cause accumulation of glycosaminoglycans, resulting in anatomic abnormalities and organ dysfunction that can increase the risk of anesthesia complications. We conducted a systematic review of the literature in order to describe the anesthetic management and perioperative outcomes in patients with MPS. We reviewed English-language literature search using an OVID-based search strategy of the following databases: 1) PubMed (1946-present), 2) Medline (1946-present), 3) EMBASE (1946-present), and 4) Web of Science (1946-present), using the following search terms: mucopolysaccharidosis, Hurler, Scheie, Sanfilippo, Morquio, Maroteaux, anesthesia, perioperative, intubation, respiratory insufficiency, and airway. The review of the literature revealed nine case series and 27 case reports. A substantial number of patients have facial and oral abnormalities posing various challenges for airway management, however, evolving new technologies that include videolaryngoscopy appears to substantially facilitate airway management in these patients. The only type of MPS that appears to have less difficulty with airway management are MPS III patients, as the primary site of glycosaminoglycan deposition is in the central nervous system. All other MPS types have facial and oral characteristics that increase the risk of airway management. To mitigate these risks, anesthesia should be conducted by experienced anesthesiologists with expertise in using of advanced airway intubating devices.
“…Furthermore, our literature review suggests that mask ventilation is less likely encountered in MPS III patients. Kamata et al [13] reported no difficulties in mask ventilation in a cohort of MPS III patients, albeit mild upper airway obstruction was noted during 14 procedures (33%), which was resolved with simple head/jaw maneuvering/positioning. Another review of a large MPS III series reported no problems with mask ventilation in 86 anesthetics [12].…”
Section: Discussionmentioning
confidence: 95%
“…The systematic review of the literature identified nine case series, and their airway management is summarized in Table 2 [5,[8][9][10][11][12][13][14][15]. In addition, we identified 27 individual case reports and these patients' characteristics and their airway management is summarized in Tables 3 and 4 [7 -10,16-38].…”
Mucopolysaccharidoses (MPS) are rare, inherited, lysosomal storage diseases that cause accumulation of glycosaminoglycans, resulting in anatomic abnormalities and organ dysfunction that can increase the risk of anesthesia complications. We conducted a systematic review of the literature in order to describe the anesthetic management and perioperative outcomes in patients with MPS. We reviewed English-language literature search using an OVID-based search strategy of the following databases: 1) PubMed (1946-present), 2) Medline (1946-present), 3) EMBASE (1946-present), and 4) Web of Science (1946-present), using the following search terms: mucopolysaccharidosis, Hurler, Scheie, Sanfilippo, Morquio, Maroteaux, anesthesia, perioperative, intubation, respiratory insufficiency, and airway. The review of the literature revealed nine case series and 27 case reports. A substantial number of patients have facial and oral abnormalities posing various challenges for airway management, however, evolving new technologies that include videolaryngoscopy appears to substantially facilitate airway management in these patients. The only type of MPS that appears to have less difficulty with airway management are MPS III patients, as the primary site of glycosaminoglycan deposition is in the central nervous system. All other MPS types have facial and oral characteristics that increase the risk of airway management. To mitigate these risks, anesthesia should be conducted by experienced anesthesiologists with expertise in using of advanced airway intubating devices.
“…MPS I, II, and VI show very similar aspects; MPS IVA also shares these characteristics, although skeletal involvement is more apparent. Patients with MPS III may also have disease processes which can complicate anesthesiological management, although somatic manifestations are generally less severe [ 3 , 5 – 7 ]. Fig.…”
BackgroundPatients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management of the airway. The evidence base for anesthesia management is often limited to case reports and small case series.AimsTo identify useful information about experience with each subtype of mucopolysaccharidosis reported in the literature and propose a guide on the best options for airway management to the anesthesiologists who take care of these patients.MethodsA query of the PubMed database specific for “anesthesia” and “mucopolysaccharidosis” and a further query specific for “mucopolysaccharidosis and difficult airway management” was conducted. We looked for those items that offered practical guidance to anesthesiological management. We did not exclude case reports, especially those that reported a specific technique, because of their practical suggestions.ResultsWe identified 15 reviews, 17 retrospective case series, 5 prospective studies, and 28 case reports that focused on airway managements in anesthesia or had practical suggestions for preoperative evaluation and risk assessment. An accurate preoperative evaluation and the need for an experienced team are emphasized in all the reviewed articles and for each type of mucopolysaccharidosis. Many suggestions on how to plan the perioperative period have been highlighted. Insertion of a laryngeal mask airway generally improves ventilation and facilitates intubation with a fiberoptic bronchoscope. Furthermore, the videolaryngoscope is very useful in making intubation easier and facilitating bronchoscope passage.ConclusionsPatients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However, a multidisciplinary approach, combined with expertise in the use of new techniques and new devices for airway management, makes anesthesiological management safer. Further research with prospective studies would be useful.Electronic supplementary materialThe online version of this article (10.1186/s13052-018-0554-1) contains supplementary material, which is available to authorized users.
“…Mucopolysaccharidosis (MPS) type II, also known as Hunter syndrome, is a rare X‐lined recessive disease caused by deficiencies of lysosomal enzymes that lead to the accumulation of glycosaminoglycans (GAGs) . Accumulated GAGs in the upper airway can lead to severe airway obstruction due to macroglossia, tonsil and adenoid hypertrophy, vocal cord enlargement, supraglottic narrowing, and tracheomalacia . In particular, MPS II patients have a higher incidence of difficult airway than other types of MPS patients .…”
Section: Introductionmentioning
confidence: 99%
“…1 Accumulated GAGs in the upper airway can lead to severe airway obstruction due to macroglossia, tonsil and adenoid hypertrophy, vocal cord enlargement, supraglottic narrowing, and tracheomalacia. 2,3 In particular, MPS II patients have a higher incidence of difficult airway than other types of MPS patients. 4,5 Therefore, it can be challenging to achieve the desired level of sedation while maintaining a patent airway and ensuring ventilation during deep sedation of such patients.…”
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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