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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