Airway Management of a Patient with Plexiform Neurofibromatosis with Asymptomatic Supraglottic Involvement

Plexiform neurofibromas represent an uncommon variant (30%) of neurofibromatosis 1 (NF1). However, glottic involvement is rare (0.03–0.1%). We are reporting a case of a 31-year-old male, a known case of plexiform NF was posted for scalp lesion excision. Examination showed multiple lobulated soft swellings over the scalp, neck, and limbs. The airway assessment appeared normal externally. Flexible bronchoscopy showed lateral pharyngeal wall bulge, proliferative lesion over left aryepiglottic fold obscuring view of vocal cords which was moving toward glottic opening with each inspiration. The initial plan was to remove the airway lesion as well. However, as he was explained about the possibility of tracheostomy while removing the glottic tumor, he wanted scalp neurofibroma excision only as he never had any airway symptoms. We planned for awake fiberoptic bronchoscopic (FOB) assisted intubation. The airway was topicalized, and using FOB patient was intubated with a 7.0 mm ID nasal Ring Adair Elwyn tube using adult FOB. The patient was then induced, and steroid was given to reduce airway edema. At the end of the surgery, neuromuscular blockade was reversed, and a smooth extubation was performed once the patient was fully awake. The postoperative period was uneventful. It is concluded that in patients with supraglottic mass for incidental surgery, thorough preoperative airway assessment and preparedness for emergency tracheostomy perioperatively are essential.