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Chapter 48 - Hurler Syndrome

from Section 7 - Miscellaneous Lesions and Syndromes

Published online by Cambridge University Press:  09 September 2021

Laura K. Berenstain
Affiliation:
Cincinnati Children's Hospital Medical Center
James P. Spaeth
Affiliation:
Cincinnati Children's Hospital Medical Center
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Summary

The mucopolysaccharidoses are a diverse group of lysosomal storage diseases that present multiple challenges to the anesthesiologist. Key features include difficult mask ventilation, difficult intubation, cardiac disease, coronary ischemia, obstructive sleep apnea, and skeletal abnormalities. Careful and thorough preoperative evaluation is crucial for anesthetic planning and safe management. It should emphasize airway management along with the cardiovascular, pulmonary, and neurologic systems. Cardiac valvulopathies and ventricular dysfunction are common and must be managed appropriately. Postoperative consideration should be given to caring for these patients in a high-acuity environment depending on the type of surgery and the severity of the underlying disease.

Type
Chapter
Information
Congenital Cardiac Anesthesia
A Case-based Approach
, pp. 367 - 373
Publisher: Cambridge University Press
Print publication year: 2021

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References

Suggested Reading

Braunlin, E. A., Harmatz, P. R., Scarpa, M., et al. Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Metab Dis 2011; 34: 1183–97.Google Scholar
Burjek, N. E., Nishisaki, A., Fiadjoe, J. E., et al. Videolaryngoscopy versus fiber-optic intubation through a supraglottic airway in children with a difficult airway: an analysis from the multicenter Pediatric Difficult Intubation Registry. Anesthesiology 2017; 127: 432–40.CrossRefGoogle ScholarPubMed
Donaldson, M. D. C., Pennock, C. A., Berry, P. J., et al. Hurler syndrome with cardiomyopathy in infancy. J Pediatr 1989; 114: 430–2.Google Scholar
Fiadjoe, J. E., Nishisaki, A., Jagannathan, N., et al. Airway management complications in children with difficult tracheal intubation from the Pediatric Difficult Intubation (PeDI) registry: a prospective cohort analysis. Lancet Respir Med 2016; 4: 3748.CrossRefGoogle ScholarPubMed
Kandil, A. I., Petit, C. S., Berry, L. N., et al. Tertiary pediatric academic institution’s experience with intraoperative neuromonitoring for nonspinal surgery in children with mucopolysaccharidosis, based on a novel evidence-based care algorithm. Anesth Analg 2019: DOI: 10.1213/ANE.000000000004215, PMID: 31082970.Google Scholar
Osthaus, W. A., Harendza, T., Witt, L. H., et al. Paediatric airway management in mucopolysaccharidosis 1: a retrospective case review. Eur J Anaesthesiol 2012; 29: 204–7.CrossRefGoogle ScholarPubMed
Sawamoto, K., Stapleton, M., Almeciga-Diaz, C. J., et al. Therapeutic options for mucopolysaccharidoses: current and emerging treatments. Drugs 2019; 79: 1103–34.CrossRefGoogle ScholarPubMed
Stapleton, M., Arunkumar, N., Kubaski, F., et al. Clinical presentation and diagnosis of mucopolysaccharidoses. Mol Genet Metab 2018; 125: 417.CrossRefGoogle ScholarPubMed
Walker, R., Belani, K. G., Braunlin, E. A., et al. Anesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis 2013; 36: 211–19.CrossRefGoogle ScholarPubMed

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