TYK128

A 10-year-old male diagnosed with Hurler syndrome is scheduled to undergo suboccipital craniectomy for cervicomedullary decompression under general anesthesia. Which of the following best describes his airway management?

a) The likelihood of airway difficulties in this patient is low if there is a history of prior uneventful anesthesia and intubation at age 2

b) This patient is at risk for difficult airway management due to his disease condition; appropriate airway devices and possibly otolaryngology back-up should be available prior to induction

c) Patients with Hurler syndrome are considered difficult airway because of presence of micrognathia/retrognathia, mandibular hypoplasia and glossoptosis

d) An emergent tracheostomy can be easily performed in this patient if needed

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Answer

Answer b

Hurler syndrome is an inherited metabolic disorder due to deficiency in lysosomal enzyme. These patients are known to have difficult airway which progressively worsens due to increasing infiltration of airway with glycosaminoglycans. They are prone to intraoperative airway difficulties secondary macroglossia, tonsillar and adenoid hypertrophy, narrow nasopharynx, infiltration of upper airway mucosa, temporomandibular joint stiffness, limited jaw thrust, short neck, and distorted airway anatomy due to infiltrates making fiberoptic intubation and tracheostomy challenging for these patients.

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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