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

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.

Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management

“…AM: Mask ventilation difficult; LMA FOI guide wire ureteral dilator wire removed LMA removed ETT railroaded over dilator 42/F, NS [29] Tracheal stenosis, CTLSA. AM: Refused awake FOI; oral fiberscope unable to visualize glottis opening asleep nasal FOI 3/F, Radiology [30] CTLSA. AM: FOI through LMA 31/M, Orthopedic [31] MG, CFA and CSA, and lumbar abnormalities (scoliosis).…”

Anesthetic Challenges in a Patient of Morquio Syndrome Associated with Acromegaly

“…Anesthetic implications of Morquio syndrome relate to the end-organ dysfunction and anatomical distortions associated with KS's intracellular accumulation. 2 This case was particularly challenging because of the accompanying systemic sequelae of acromegaly, i.e., cardiovascular effects, respiratory effects, osteoarthropathy, metabolic complications (insulin resistance, hyperglycemia, and hyperlipidemia), and hypopituitarism.…”

“…Anesthetic implications of Morquio syndrome relate to the end-organ dysfunction and anatomical distortions associated with KS’s intracellular accumulation. 2 …”

“…Unique anesthetic concerns include distortion of upper airway anatomy secondary to infiltration of respiratory tissues with KS causing difficult airway and alterations in cardiorespiratory function. 2 Infiltration of the cervical spine with odontoid hypoplasia places these patients at risk for atlantoaxial subluxation.…”

Anaesthesia management in craniovertebral junctional anomalies

“…There may be congestion of face and tongue, and pressure sore on malar prominences owing to horseshoe headrest. [3] Extreme flexion of the neck may cause endobronchial intubation because of short trachea and intraoral kinking of endotracheal tube (ETT); hence, armoured ETTs are preferred. Excessive flexion or extension of head may cause brainstem compression in patients with Arnold–Chiari malformation.…”