2014
DOI: 10.4103/0970-9185.142862
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Management of an anticipated difficult airway in Hurler′s syndrome

Abstract: Management of an anticipated difficult airway in Hurler syndrome. Hurler syndrome is a subtype of Mucopolysaccharidosis (MPS) type 1. Mucopolysaccharidosis (lysosomal storage diseases) are a group of inherited disorders caused by deficiency of specific lysosomal enzyme required for a normal degradation of glycosaminoglycons (GAGs). Administration of general anaesthesia in patients who have congenital syndromes such as Hurler's is often a challenge because of progressive airway, craniofacial and skeletal abnorm… Show more

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Cited by 6 publications
(3 citation statements)
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“…Difficulty with nasal FOB is to be expected because of the narrow nasopharyngeal path and GAG infiltration of the adenoids [ 4 , 9 , 20 ]. Intubation can also be obtained by passing the fiberscope through the LMA [ 11 , 43 , 44 ], and the new supraglottic airway device makes this procedure even easier [ 46 , 47 ]. The equipment is shown in Fig.…”
Section: Discussionmentioning
confidence: 99%
“…Difficulty with nasal FOB is to be expected because of the narrow nasopharyngeal path and GAG infiltration of the adenoids [ 4 , 9 , 20 ]. Intubation can also be obtained by passing the fiberscope through the LMA [ 11 , 43 , 44 ], and the new supraglottic airway device makes this procedure even easier [ 46 , 47 ]. The equipment is shown in Fig.…”
Section: Discussionmentioning
confidence: 99%
“…, ankylosing spondylitis, degenerative osteoarthritis, Treacher–Collins, Klippel–Feil, Down syndrome, mucopolysaccharidosis, and airway masses) (Category B4-H evidence ). 71–122…”
Section: Guidelinesmentioning
confidence: 99%
“…1 Craniofacial and skeletal abnormalities in these patients include macroglossia, tonsil and adenoid hypertrophy, odontoid hypoplasia, and atlantoaxial instability. [2][3][4] Because these anomalies can complicate airway management, intubation with flexible bronchoscopy (FB) has traditionally been used in our practice in selected MPS patients. In 2009, video laryngoscopy (VL) was introduced to our practice.…”
mentioning
confidence: 99%