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Latest revision as of 02:17, 25 September 2026
A 7 year old 35 kg girl with Hurler syndrome for adenotonsillectomy is at increased risk compared to a healthy 7 year old for the following:
a) neurological injury from neck extension
b) postoperative respiratory events
c) difficult face mask ventilation on induction
d) all of the above
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Answer
Answer d.
Hurler syndrome, or mucopolysaccharidosis I, is an autosomal recessive disorder where the congenital absence of the lysosomal enzyme alpha- L-iduronidase leads to the build-up of the glycosaminoglycans heparan sulfate and dermatan sulfate in the tissues; the tongue, pharyngeal connective tissue and skeletal structures are involved, leading to propensity for upper airway obstruction and development of an unstable cervical spine.
Notes
- This question originally printed in the Pediatric Anesthesiology Review Topics kindle book series, and appears courtesy of Naerthwyn Press, LLC.
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