Safe intubation in Morquio-Brailsford syndrome: A challenge for the anesthesiologist

Morquio-Brailsford syndrome is a type of mucopolysaccharidoses. It is a rare disease with features of short stature, atlantoaxial instability with risk of cord damage, odontoid hypoplasia, pectus carinatum, spine deformities, hepatomegaly, and restrictive lung disease. Neck movements during intubati...

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Bibliographic Details
Published in:Journal of anaesthesiology, clinical pharmacology clinical pharmacology, 2013-04, Vol.29 (2), p.258-261
Main Authors: Chaudhuri, Souvik, Handigodu Duggappa, Arun, Mathew, Shaji, Venkatesh, Sandeep
Format: Article
Language:English
Subjects:
Bronchoscopy
Care and treatment
Case Report
Cervical instability
classic laryngeal mask airway
cord damage
difficult airway
Intubation
Methods
Mucopolysaccharidosis
Pediatric anesthesia
Trachea
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Summary:Morquio-Brailsford syndrome is a type of mucopolysaccharidoses. It is a rare disease with features of short stature, atlantoaxial instability with risk of cord damage, odontoid hypoplasia, pectus carinatum, spine deformities, hepatomegaly, and restrictive lung disease. Neck movements during intubation are associated with the risk of quadriparesis due to cervical instability. This, along with the distortion of the airway anatomy due to deposition of mucopolysaccharides makes airway management arduous. We present our experience in management of difficult airway in a 3-year-old girl with Morquio-Brailsford syndrome posted for magnetic resonance imaging and computerized tomography scan of a suspected unstable cervical spine. As utmost sagacity during intubation is required, the child was intubated inside operation theatre in the presence of experienced anesthesiologists and then shifted to the peripheral location. Intubation was done with an endotracheal tube railroaded over a pediatric fibreoptic bronchoscope passed through the lumen of a classic laryngeal mask airway, keeping head in neutral position.
ISSN:0970-9185
2231-2730
DOI:10.4103/0970-9185.111666