Bronchoscopy and airway management in patients with mucopolysaccharidoses (MPS).

INTRODUCTION: Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders characterized by tissue deposition of glycosaminoglycans (GAG). Their musculoskeletal abnormalities and the GAG storage in the airway result in increased risk for patients undergoing anesthesia. This study evaluates a multi-disciplinary airway management approach and reports upper and lower airway findings of flexible bronchoscopy performed during these procedures. METHODS: This is a retrospective study over 10 years evaluating approaches to and outcomes of airway management and bronchoscopic findings in this patient group. RESULTS: Thirty-one patients underwent a total of 105 anesthetic events of which 74 involved multiple surgical services. The majority of patients were either MPS I (n = 9) or MPS II (n = 19). The median age was 8.6 years (range 1.1-24 years). Airway management by anesthesiologists alone occurred in 31 cases including natural airway (n = 7), perilaryngeal airway (n = 7), oral or nasal intubation (n = 7) or tracheostomy (n = 6) and emergent fiberoptic intubation in four cases. In 74 of the procedures, flexible bronchoscopy was performed which included fiberoptic intubation in 22 cases. Post-operative complications occurred in eight cases mostly when prolonged airway instrumentation had occurred. The most frequent findings on bronchoscopy were GAG deposits/adenoid hypertrophy in 72%, laryngomalacia in 31% and lower airway deposits and/or tracheobronchomalacia in 46% of procedures. Deposits of GAG were seen in patients as young as 4 years of age. CONCLUSION: Our experience demonstrates that a multidisciplinary approach and combined surgeries in MPS provides for safe airway management and allows diagnostic assessments for further patient care without added risks. Significant, multi-factorial airway compromise may occur already in early childhood including upper and lower airway GAG deposits.

The effects of isoflurane and desflurane titrated to a bispectral index of 60 on the cortical somatosensory evoked potential during pediatric scoliosis surgery.

In this study, we compared the effect of isoflurane and desflurane on the posterior tibial somatosensory evoked potential recorded by scalp electrodes during correction of idiopathic scoliosis in pediatric patients. Depth of sedation was controlled by maintaining bispectral index (BIS) at 60 throughout the study. Comparison of patients breathing desflurane and isoflurane showed an evoked cortical amplitude (N37-P45) of 0.53 +/- 0.3 microV versus 1.3 +/- 0.8 microV (P = 0.014), respectively. In addition to this comparison, a crossover design was included whereby the desflurane or isoflurane received in the first part of the study was changed to the other anesthetic. Substituting one anesthetic for another confirmed our initial finding that the cortical evoked amplitude is greater with isoflurane than with desflurane. No differential effect was found between desflurane and isoflurane on the evoked subcortical (N31-P34) amplitude or the P37 latency.

Tracheal extubation of deeply anesthetized pediatric patients: a comparison of desflurane and sevoflurane.

UNLABELLED: In this study, we examined the emergence characteristics of children tracheally extubated while deeply anesthetized with desflurane (Group D) or sevoflurane (Group S). Forty-eight children were randomly assigned to one of the two groups. At the end of the operation, all subjects were tracheally extubated while breathing 1.5 times the minimal effective concentration of assigned inhaled anesthetic. Recovery characteristics and complications were noted. Group D patients had higher arousal scores on arrival to the postanesthesia care unit than Group S patients. Later arousal scores were not significantly different. No serious complications occurred in either group. Coughing episodes and the overall incidence of complications after extubation were more frequent in Group D. Readiness for discharge and actual time to discharge were not significantly different between groups. Emergence agitation was common in both groups (33% overall, 46% for Group D, and 21% for Group S). Narcotic administration in the postanesthesia care unit occurred more frequently in Group D (10 of 24 patients) versus Group S (3 of 24 patients). Premedication with oral midazolam resulted in significantly longer emergence times regardless of the potent inhaled anesthetic administered. IMPLICATIONS: Deep extubation of children can be performed safely with desflurane or sevoflurane. Airway problems occur more frequently with desflurane. Awakening occurs more quickly with desflurane. Midazolam premedication has a greater effect on emergence times than does the choice of inhaled anesthetic. Emergence agitation occurs frequently with either technique.