Page 16 - CASA Bulletin of Anesthesiology Vol 9 (4) 2022 (3)

CASA Bulletin of Anesthesiology

Anesthetic implications in NF1 patients with an undiagnosed laryngeal

plexiform neurofibroma: A case report

1
1
2
Muhammad H. Yousef MD, Xiaowei Lu MD, Marci J. Neidich 2, Nancy M. Bauman MD,
3
1
Brigitte C Widemann MD，Andrew Mannes MD.
1 Department of Perioperative Medicine, the National Institutes of Health Clinical Center,
Bethesda, MD.
2 Division of Pediatric Otolaryngology, Children’s National Medical Center, Washington, DC.
3 Pediatric Oncology Branch, NCI, NIH, Bethesda, MD.

Correspondence: Andrew Mannes, Department of Perioperative Medicine. Bldg 10/2C-525. 10
Center Dr Bethesda, MD 20892. (301) 545-7320, (301) 480-1699 (fax)

Abstract

Neurofibromatosis is an autosomal dominant genetic disorder characterized by cutaneous
café au lait spots, central and peripheral nervous system neurofibromas, and other tumors arising
from the central nervous system. Airway obstruction from pharyngeal or laryngeal involvement
is rare, but when incidentally found, can cause great difficulty with airway management under
anesthesia as illustrated in this case.

As part of a National Institutes of Health (NIH) Neurofibromatosis (NF) protocol study, a 3
year old African American male enrolled in the NF Type 1 natural history protocol presented for
an MRI under anesthesia to assess interval progression of the disease. The patient was recently
diagnosed with obstructive sleep apnea (OSA) presumed secondary to enlarged adenoids and an
otolaryngology consultation was pending. Pre-anesthesia evaluation performed by the Pre-
Anesthesia Clinic Anesthesiologist was otherwise normal.

Initially, the magnetic resonance imaging study (MRI) was to be performed under
Monitored Anesthesia Care/Sedation (MAC) with propofol infusion. Due to difficulties
maintaining adequate ventilation, even with the use of head positioning maneuvers,
oropharyngeal and nasopharyngeal airways, and varying Laryngeal Mask Airways (LMA), this
sedation case was emergently converted to general endotracheal anesthesia. Direct laryngoscopy
revealed a large submucosal mass in the right supraglottis that nearly completely obstructed the
glottic inlet. The patient was intubated and admitted to National Institutes of Health Clinical
Center Intensive Care Unit (ICU) and later transferred to Children’s National Medical Center
Pediatric ICU. An Otolaryngology consultation was obtained for management of the obstructing
laryngeal mass, presumed to be a plexiform neurofibroma. This case report highlights the need to
recognize the rare occurrence of obstructing airway neurofibromas in patients with NF-1 to
minimize the risk of airway obstruction under sedation and general anesthesia.

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