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

Chaudhuri S, Duggappa AK, Mathew S, Venkatesh S - J Anaesthesiol Clin Pharmacol (2013)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesia, Kasturba Medical College, Manipal, Karnataka, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

CT of the child with nonossification of the dens
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Figure 5: CT of the child with nonossification of the dens

Mentions: The ETT with LMA was fixed as a single unit after confirming the ETT tip position by visualization and auscultation. Care was taken to avoid kinking of the ETT at the point of exit from LMA. Throughout the procedure, saturation was maintained between 96% and 100%. A total of 3mg IV atracurium was then given and child was shifted to MRI suite on oxygen trolley with monitors. Anesthesia during the MRI and CT was uneventful, after which child was shifted to pediatric intensive care unit and was extubated in ICU. MRI showed cord compression involving the cervicomedullary junction, hypoplastic split atlas, and posterior atlantooccipital fusion [Figure 4]. CT confirmed hypoplasia of C1 vertebra, split atlas, and nonossification of the dens [Figure 5]. Child was then put on a cervical collar and eventually discharged.


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

Chaudhuri S, Duggappa AK, Mathew S, Venkatesh S - J Anaesthesiol Clin Pharmacol (2013)

CT of the child with nonossification of the dens
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3713682&req=5

Figure 5: CT of the child with nonossification of the dens
Mentions: The ETT with LMA was fixed as a single unit after confirming the ETT tip position by visualization and auscultation. Care was taken to avoid kinking of the ETT at the point of exit from LMA. Throughout the procedure, saturation was maintained between 96% and 100%. A total of 3mg IV atracurium was then given and child was shifted to MRI suite on oxygen trolley with monitors. Anesthesia during the MRI and CT was uneventful, after which child was shifted to pediatric intensive care unit and was extubated in ICU. MRI showed cord compression involving the cervicomedullary junction, hypoplastic split atlas, and posterior atlantooccipital fusion [Figure 4]. CT confirmed hypoplasia of C1 vertebra, split atlas, and nonossification of the dens [Figure 5]. Child was then put on a cervical collar and eventually discharged.

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesia, Kasturba Medical College, Manipal, Karnataka, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus