Page 20 - CASA Bulletin of Anesthesiology Vol 9 (4) 2022 (3)

CASA Bulletin of Anesthesiology

Both patients required emergent tracheotomy. Even if intra‐oral pathology is recognized pre‐
operatively, elective awake fiberoptic tracheal intubation may fail because of a grossly distorted
anatomy . However, successful intubation after inhalation anesthesia with sevoflurane has been
12
described .
2
Although the manifestations of NF1 are often mild, there may be associated pathology of
direct relevance and importance to the anesthetic management of patients with the disease. It is
therefore important to have a working knowledge of the clinical manifestations of the disease so
that a systematic approach to the pre‐operative assessment of these patients can result in safe
perioperative management. In this case, this patient’s OSA was actually due to his laryngeal
plexiform neurofibroma and not adenoid hypertrophy. Despite the large size of the laryngeal
lesion, he remained asymptomatic until laying supine further confusing the etiology. From this
experience, we recommend that NF1 patients with OSA or other airway symptoms, regardless of
severity or presumed etiology, undergo a flexible fiberoptic laryngoscopy by ENT prior to
elective procedures requiring sedation or general anesthesia.

Teaching point

1. Laryngeal lesions, including plexiform neurofibromas, can be relatively asymptomatic
until the induction of anesthesia. The presence of airway neurofibromas should be considered in
NF-1 patients with pre-operative airway symptoms.

2. It is of utmost importance that NF-1 children with OSA or sleep disordered breathing be
evaluated by pediatric otolaryngology prior to receiving anesthesia for elective procedures or
studies.

3. Even supposed simple sedation cases can take an ominous turn and it is imperative to
plan accordingly to convert to a general endotracheal anesthetic.

4. It is very important to have more than one anesthesia provider available for all off-site
procedures involving pediatric anesthesia for the rare but rapidly deteriorating emergency
situation.

Disclosures/ Conflicts of interest

Conflict of Interest: No conflicts of interest declared. Informed written consent was obtained
for the research protocol and all publications that come out of the study. The natural history
protocol is research funded through the intramural research program. A sentence should be
added:” This research was, in part, funded through the NCI CCR intramural research program.

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