Juniper Publishers-Journal of Ophthalmology
A number of medical conditions
may present to the emergency department involving a change or loss of
vision. Many of these conditions are sudden in onset and may result in
complete or partial loss of vision, such as stroke or multiple
sclerosis. A 53 year old male with a past history of oral squamous cell
carcinoma presented with a headache and changes in color hue in his
right eye. CT imaging revealed recurrent oral squamous cell carcinoma
involving the right sphenoid and ethmoid sinuses with extension into the
right middle cranial fossa and orbit. It is important to note that a
change in color vision may be from optic nerve dysfunction, which was
found to be the case for this patient. The patient was diagnosed with
optic neuropathy secondary to recurrent oral squamous cell carcinoma,
which represents the first case report of such condition.
Keywords: Human Papillomavirus
Abbreviations: OSCC : Oral Squamous Cell Carcinoma
Introduction
Oral squamous cell carcinoma (OSCC) represents the
most frequent malignancy of the oral mucosa [1,2]. Tobacco and alcohol
are strong risk factors [2], and a number of studies have commented on
the association between human papilloma virus and OSCC [3,4]. It
represents a significant clinical challenge, requiring aggressive
surgical resection with staging of the tumor. Chemotherapy and radiation
therapy are required to achieve remission [5] and management requires
evaluation by MRI and PET [6]. The most frequent spread of OSCC is to
the cervical lymph nodes and lung, followed by metastasis to bone and
liver [7]. Nasopharyngeal spread may occur in up to 28% of cases;
however secondary ocular spread is rare [8]. We report a patient who
presented to the emergency room with symptoms of color vision changes
which was found to be secondary to metastatic spread of OSCC.
Materials and Methods
Biomicroscopy, color vision testing, visual field
testing, dilated indirect ophthalmoloscopy, optical coherent tomography,
fundus photography, and fluorescein angiography was performed.
Subsequent CT and PET imaging was also obtained.
Case Report
A 53 year old male presented to the emergency room
with headaches and decreased contrast sensitivity in his right eye. He
was a nonsmoker, but would occasionally drink alcohol and use
recreational drugs. Family history was unremarkable for malignancy.
Medical history included controlled hypertension and OSCC involving the
tongue, with spread into the cervical lymph node. After undergoing
surgical resection, he underwent chemotherapy and radiation therapy to
the head, neck, and lungs. Two years later he was noted to have a
recurrence of OSCC, which involved his left maxillary and ethmoid
sinuses with extension into the left orbit. He underwent left orbital
exenteration, left external sphenoidectomy, left ethmoidectomy including
resection of tumor at cribriform plate, and left medial maxillectomy.
The patient concurrently received an additional three month course of
chemotherapy and radiation therapy.
The patient was referred for ophthalmologic
consultation. Due to the patient’s unusual presentation, there was
concern for recurrence of OSCC in the right orbit. The patient was sent
for MRI imaging of the brain and orbit. Due to surgical clips from the
previous orbital exenteration, a CT scan was performed instead. He was
referred to oncology for further evaluation, and received a PET scan and
another CT scan. The patient elected for hospice care rather than
further surgical or medical treatment.
Results
Ophthalmic examination disclosed a best corrected
visual acuity of 20/25-2 in the right eye, and an intraocular pressure
of 10 mmHg. There were no abnormalities of the right eyelids,
conjunctiva, cornea, lens, pupillary response, or extraocular eye
motility. There was no proptosis or pain or resistance on retropulsion.
There were no abnormalities of color vision by Ishihara plate testing.
Humphrey 24-2 visual field testing revealed an enlarged blind spot and
small peripheral abnormalities (Figure 1). After dilation, the optic
nerve showed mild hyperemia and mild retinal venous congestion (Figure
2A). No abnormalities of the macula, peripheral retina, or vitreous were
noted. A fluorescein angiogram was performed and disclosed mild late
staining of the optic nerve, with no other abnormalities (Figure 2B).
Evaluation of the optic nerve by optical coherent tomography
demonstrated mild optic nerve elevation, consistent with optic
neuropathy (Figure 3).
The CT scan disclosed sphenoid and posterior ethmoid
sinus disease (Figure 4). PET scan imaging demonstrated progression of
malignancy at the level of the right skull base with extension into the
right cavernous sinus and extension to the posterior nasopharynx and
adenoid region. Extensive new skeletal metastatic disease was also
noted. Within a month, he presented with proptosis and an orbital apex
syndrome. Extensive tumor invasion into the right orbit was noted by an
additional CT (Figure 5). The patient succumbed to his illness within
three weeks of this examination.
Discussion
Visual complaints can be a common presentation to the
emergency department. This patient presented with a headache and
changes in color hue, and was diagnosed with optic neuropathy secondary
to recurrent OSCC. Given the ophthalmologic findings, CT and PET
studies, it is likely the optic neuropathy was secondary to tumor spread
in the cavernous sinus giving rise to the venous dilation and direct
tumor involvement of the optic foramen from the sphenoid sinus. We
suspect the patient’s headache was secondary to meningeal involvement.
Macrophage polarization and regional spread to the cervical lymph nodes,
both of which this patient demonstrated, have been indicators of a poor
prognosis [9]. Fewer than 10 cases involving ocular spread have been
reported, which also carries a poor prognosis [7]. None of the reported
cases have presented as an optic neuropathy. In 2007, Feng et al. [10]
reported the first case of acute visual loss in a head and neck cancer
patient with ocular metastasis and sphenoid pyocele, however he points
out that the visual loss occurred from compression of the optic nerve
and the sphenoid pyocele.
This case serves to illustrate the unusual nature of
visual complaints that may present to the emergency department and helps
to illustrate the effectiveness of the multispecialty team approach in
assessing complex patients.
Acknowledgments
The authors thank Dr. Vijay Suhag (Department of
Oncology, Sutter Roseville Medical Center), Dr. Christopher Markus
(Emergency Department, Sutter Roseville Medical Center), Dr. Randall Ow
(Department of Otolaryngology, Sutter Roseville Medical Center), and Dr.
Michael Kaplan (Department of Otolaryngology, Head and Neck Surgery,
Stanford University Medical Center).
Figures and Tables
Figure 1: An
enlarged blind spot and peripheral visual field defects in the right eye
formally tested using the Humphrey 24-2 testing algorithm.
Figure 2: Fundus
photography displaying mild hyperemia in the right optic nerve and
retinal venous congestion (A), followed by fluorescein angiography
displaying mild late staining of the optic nerve without other
abnormalities of the macula or retinal vasculature (B).
Figure 3: Cirrus-HD optical coherence tomography demonstrating slight optic nerve elevation consistent with right optic neuropathy.
Figure 4: Computed tomography disclosing sphenoid and posterior ethmoid sinus disease.
Figure 5: Computed tomography demonstrating proptosis and an orbital apex syndrome with extensive tumor invasion into the right orbit.
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