Page 19 - CASA Bulletin of Anesthesiology Vol 9 (4) 2022 (3)

Vol. 9, No 4, 2022

the lesion in a hemostatic manner, with precision to maintain the medial mucosa of the
aryepiglottic fold. The lesion was debulked until the glottis aperture was readily visible and the
right aryepiglottic fold dimension was similar to the normal left aryepiglottic fold (Figure 4).
Patient remained intubated in the PICU, and on post-operative day (POD) 2, the patient was
successfully weaned off sedation and extubated without complications. No stridor or respiratory
distress was noted. Patient was transferred to the floor on POD 3 and discharged home on POD
4 after a bedside flexible fiberoptic laryngoscopy confirmed airway patency and lack of
significant edema.

The pathology revealed a plexiform neurofibroma without mitotic activity or nuclear
pleomorphism. A ki67 immunostain showed low proliferative activity (<5%). Due to the high
risk of recurrence the patient will undergo serial laryngoscopies by his local otolaryngologist for
subsequent management. It is possible that he may need an open excision of potential residual
neurofibroma.

Discussion

Airway obstruction can become very severe and result in cyanosis, respiratory distress and
fatigue, pneumothorax, and pneumomediastinum, and death. The most significant anatomic risk
factor for obstructive sleep apnea in non-obese healthy children is adenoid and or tonsillar
hypertrophy. Disproportionate proliferation of the adenoids and tonsils can occur in children
with allergic rhinitis, asthma, and children exposed to cigarette smoking or upper airway
respiratory infections, particularly viruses . Several other medical conditions are known to be
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associated with obstructive breathing in children including upper airway narrowing due to
craniofacial anomalies, neuromuscular abnormalities or hypotonia, Down syndrome, Prader
Willi syndrome, mucopolysaccharide storage disease, allergic rhinitis, epiglottitis, viral croup,
laryngomalacia, choanal atresia/stenosis, micrognathia, cleft palate after surgical repair, turbinate
hypertrophy, deviated septum and maxillary constriction, as well as hypothyroidism. Although
benign tumors occurring in the peripheral nerves are seen throughout the body, neurogenic
tumors arising primarily from the larynx are rare and include neurofibroma, schwannoma, and
malignant peripheral nerve sheath tumor. When neurofibromas occur in the larynx, they may be
solitary and nonsyndromic, or most commonly, associated with neurofibromatosis (NF) type 1 or
2 .
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An estimated 5% of patients with NF1 have an intra‐oral manifestation of the disease .
8
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Discrete neurofibromas may involve the tongue or the larynx . When the latter is involved, it is
often the aryepiglottic fold or arytenoids that are affected, presumably reflecting those areas most
rich in terminal nerve plexuses. Discrete and plexiform neurofibromas commonly occur in the
cervical region and large tumors of the parapharyngeal space may result in distortion of the
airway. Pathologies in NF1 that affect the tongue, larynx and cervical tissues may cause
obstruction. Symptoms of dyspnea, stridor, loss or change of voice or dysphagia should warn the
anesthesiologist of potential airway problems. Suspicion warrants specialist examination with
indirect laryngoscopy and CT or MR imaging.

Airway obstruction after induction of anesthesia, as observed in this case, has been reported
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in patients with a tongue neurofibroma and a neurofibroma involving the laryngeal inlet .
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