Novel airway management in a child with Goldenhar Syndrome at a remote location

A six year old female child with Goldenhar syndrome, dextrocardia, a single functional ventricle, transposition of great arteries, pulmonary and subpulmonary stenosis and atrial and ventricular septal defects, obstructive sleep apnea, failure to thrive, speech and developmental delay presented to our institution requiring a cardiac magnetic resonance imaging (MRI) in order to delineate her cardiac anatomy. She was being evaluated by the otolaryn-gology, ophthalmology and orthopedics services for further reconstructive surgery. Recent echocardiography demonstrated a single ventricle with normal function, mild aortic valve regurgitation, and pulmonic sub-valvular stenosis. Prior surgery included a Blalock-Taussig shunt and subsequent Glenn procedure (Bidirectional cavo-pulmonary shunt), and multiple cleft lip/ palate procedures. Her prior anesthetic history was significant for inability to obtain a definitive airway. Physical examination revealed right hemifacial microsomia, max-illary and mandibular hypoplasia, microtia, significant alar collapse, right anophthal-mia, and severe kyphoscoliosis with hemi-vertebra and right thoracic hump. Given her difficult airway and remote location , the airway was secured before going to MRI. Attempts with the Glidescope using the pediatric blade provided subopti-mal visualization compared to the direct laryngoscopy (the blade was too large for her oral cavity). Fiberoptic bronchoscopy allowed visualization of the vocal cords. However, passing the scope through the small glottic opening was unsuccessful. A size 1.5 layrngeal mask airway (LMA) was then placed, achieving adequate seal and ventilation. Subsequently the fiberoptic bronchoscope (FOB) was passed through the LMA, and vocal cords were visualized. A 3.5 mm cuffed endotracheal tube (ETT) was successfully advanced into the trachea through the LMA. Although the ETT was well positioned, the end of the LMA was too close to the end of the ETT, thus making it impossible to secure the tube safely for transport. The decision was made to remove the LMA. However, the length of the tube was limiting ; another 3.5 mm cuffed ETT was threaded over the existing ETT, and then Keywords: pediatrics, airway management, Golden-har syndrome.