At the Crossroads

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Identifier walsh_2019_s3_c4-abstract
Title At the Crossroads
Creator Konstantinos Douglas; Michael Yoon; Frederick Jakobiec; Joseph Rizzo III; Bart Chwalisz
Affiliation (KD) (MY) (FJ) (JR) (BC) Massachusetts Eye and Ear Infirmary, Boston, Massachusetts
Subject Intracranial Tumors; Cranial Nerve Palsies; Facial Pain; Perineural Invasion
History A 54-year-old female former smoker presented with vertical diplopia, left facial pain and left forehead weakness. She previously had thyroidectomy for a noncancerous nodule and abdominal hysterectomy for fibroids. She reported a two-year history of worsening facial pain and numbness that started as a sore spot above the left eyebrow, and over months progressed to involve the left forehead and cheek. This pain was initially responsive to steroids but became persistent, severe, and unresponsive to treatment. Within a few months of the onset of the pain, the patient developed oblique binocular diplopia that also worsened over time. In addition, she lost the ability to move the forehead on that side, although the rest of the face appeared normal. Exam was notable for left-sided cranial nerve findings: partial CNIII palsy, complete CNIV and CNVI palsies, left-sided hypesthesia in the V1-2 distribution, and weakness of the temporal branch of left frontal nerve. Later, left eye chemosis, injection and inferior keratopathy were also present. Skin and ENT exams were normal. MRI with contrast revealed asymmetric enhancement of left cavernous sinus; on further review there was extension along the left frontal and vidian nerves. CT chest/abdomen/pelvis showed a benign hepatic cyst. PET scan only showed reduced uptake in the extraocular muscles, secondary to the cranial nerve palsies. An extensive laboratory work-up was unrevealing, including serologies for connective tissue diseases, ANCA, sarcoidosis, Lyme and syphilis. Four lumbar punctures yielded normal CSF and cell counts, including cytology and flow. The patient and was managed with escalating doses of steroids, which were initially given orally but then transitioned to IV. She was admitted to an outside hospital for severe refractory pain, and transferred to our institution. A diagnostic left frontal nerve biopsy was obtained, and showed moderately differentiated, keratinizing squamous cell carcinoma with intraneural and perineural involvement.
Disease/Diagnosis Intracranial squamous cell carcinoma with unknown primary origin, which in addition to involvement of the cranial nerves in the cavernous sinus (III, IV, VI, V1, V2) included branches of the ipsilateral facial nerve.
Date 2019-03
References [1] Pierre R Bourque and others, ‘Combined Isolated Trigeminal and Facial Neuropathies from Perineural Invasion by Squamous Cell Carcinoma: A Case Series and Review of the Literature', Journal of Clinical Neuroscience, 2017 <http://dx.doi.org/https://doi.org/10.1016/j.jocn.2016.09.022>. [2] Serkan Erkan and others, ‘Clinical Perineural Invasion of the Trigeminal and Facial Nerves in Cutaneous Head and Neck Squamous Cell Carcinoma: Outcomes and Prognostic Implications of Multimodality and Salvage Treatment', Head and Neck, 2017 <http://dx.doi.org/10.1002/hed.24607>.
Language eng
Format application/pdf
Type Text
Source 2019 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of NANOS Annual Meeting Frank B. Walsh Sessions; 2019
Collection Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6gn2rc6
Setname ehsl_novel_fbw
ID 1431972
Reference URL https://collections.lib.utah.edu/ark:/87278/s6gn2rc6
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