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aerodigestive evaluation

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Chapter 48 - Hurler Syndrome
from Section 7 - Miscellaneous Lesions and Syndromes
- By Kirk Lalwani, Erin Conner
Edited by Laura K. Berenstain, James P. Spaeth
Book:

Congenital Cardiac Anesthesia

Published online:

09 September 2021

Print publication:

16 September 2021, pp 367-373
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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.