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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

Shape of classic LMA changed like a PLMA for better position at laryngeal aperture after insertion in a neutral head position
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Figure 2: Shape of classic LMA changed like a PLMA for better position at laryngeal aperture after insertion in a neutral head position

Mentions: Intubation was done inside the operation theatre (OT) in presence of senior anesthesiologists and then shifted to the MRI suite. Different sizes of endotracheal tubes (ETTs), laryngeal mask airways (LMAs), proseal LMA, and pediatric fibreoptic bronchoscope were kept in the difficult airway cart. An intravenous (IV) access was secured and child was shifted to OT after administration of 0.1mg IV glycopyrrolate and 0.5mg IV midazolam. Baseline vitals were heart rate of 150/min, blood pressure 94/60mmHg and oxygen saturation (SpO2) of 99%. Anesthesia was induced with titrating doses of propofol along with 3% sevoflurane in 100% oxygen; ensuring spontaneous ventilation was maintained while monitoring SpO2 and end tidal carbon dioxide (EtCO2). A total of 10μg IV fentanyl was given and after adequate depth of anesthesia, a size 2 classic LMA which was shaped like a proseal LMA using stylet was gently inserted with manual in-line stabilization (MILS) [Figure 2]. LMA position was confirmed by adequate chest expansion and presence of capnogram. A right-angle tracheal tube connector with seal was attached to the LMA [Figure 3]. This allowed fibreoptic bronchoscope to be inserted through the LMA while the child was ventilated with 100% oxygen and 2%-3% sevoflurane.


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

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

Shape of classic LMA changed like a PLMA for better position at laryngeal aperture after insertion in a neutral head position
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Shape of classic LMA changed like a PLMA for better position at laryngeal aperture after insertion in a neutral head position
Mentions: Intubation was done inside the operation theatre (OT) in presence of senior anesthesiologists and then shifted to the MRI suite. Different sizes of endotracheal tubes (ETTs), laryngeal mask airways (LMAs), proseal LMA, and pediatric fibreoptic bronchoscope were kept in the difficult airway cart. An intravenous (IV) access was secured and child was shifted to OT after administration of 0.1mg IV glycopyrrolate and 0.5mg IV midazolam. Baseline vitals were heart rate of 150/min, blood pressure 94/60mmHg and oxygen saturation (SpO2) of 99%. Anesthesia was induced with titrating doses of propofol along with 3% sevoflurane in 100% oxygen; ensuring spontaneous ventilation was maintained while monitoring SpO2 and end tidal carbon dioxide (EtCO2). A total of 10μg IV fentanyl was given and after adequate depth of anesthesia, a size 2 classic LMA which was shaped like a proseal LMA using stylet was gently inserted with manual in-line stabilization (MILS) [Figure 2]. LMA position was confirmed by adequate chest expansion and presence of capnogram. A right-angle tracheal tube connector with seal was attached to the LMA [Figure 3]. This allowed fibreoptic bronchoscope to be inserted through the LMA while the child was ventilated with 100% oxygen and 2%-3% sevoflurane.

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