Woman presents with progressive left eye proptosis, upper lid edema
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A 41-year-old woman was referred to the neuro-ophthalmology service at the New England Eye Center for left-sided proptosis, left upper lid edema and progressively worsening vision in the left eye for about 5 months.
Her symptoms started after giving birth to her child. She did not have any double vision but was having intermittent dull, throbbing bitemporal and bifrontal headaches that worsened with stress. Her medical history was significant for prior Graves’ disease although she was currently euthyroid and not on medications. She also had a history of gestational diabetes and pre-eclampsia. She was otherwise healthy and did not take any medications. Her ocular history included myopia and astigmatism for which she wore glasses.
Examination
Best corrected visual acuity was 20/25 in the right eye and 20/50 in the left eye. The pupil examination was notable for a sluggish pupillary response and a 2+ relative afferent pupillary defect of the left eye. IOPs were normal at 13 mm Hg in the right eye and 14 mm Hg in the left eye. Color vision measured via HRR testing was 4/4 in the right eye and 1.5/4 in the left eye.
Hertel measurements were 16 mm in the right eye and 21 mm in the left eye with a 5-mm relative proptosis of the left eye. Extraocular movements were full, and the patient was orthophoric. Cranial nerves V to XII were grossly intact except for mild orbicularis weakness in the left eye. There was moderate edema of the left upper lid but no palpable mass or abnormal lid position. The conjunctiva was white and quiet bilaterally without any injection or corkscrew vessels. The rest of the anterior segment examination was unremarkable. Dilated fundus examination was significant for left optic disc edema.
Humphrey visual field 30-2 SITA Fast showed full visual fields in the right eye and a superior arcuate and inferior hemifield loss in the left eye (Figure 1a). OCT of the optic nerve was normal in the right eye and confirmed left optic disc edema, with the average retinal nerve fiber layer thickness at 90 µm in the right eye and 108 µm in the left eye (Figure 1b). The ganglion cell complex was full in the right eye and diffusely thin in the left eye (Figure 1c).
Source: Yi Ling Dai, MD, and Laurel N. Vuong, MD
What is your diagnosis?
See answer below.
Proptosis, lid edema
With the patient’s history of Graves’ disease, the left-sided proptosis and eyelid edema would be concerning for thyroid eye disease with severe optic neuropathy.
However, her presentation was asymmetric, and she lacked the typical thyroid eye disease presentation of eyelid retraction and conjunctival injection. In addition, there was no limitation of her extraocular movements, which would be expected in cases of thyroid orbitopathy associated with such significant proptosis and optic neuropathy. Inflammatory etiologies such as idiopathic orbital inflammation were also considered, but again, she did not have the typical presentation of eye pain, conjunctival injection and double vision. A carotid-cavernous fistula was also low on the differential as she did not have any history of trauma or hypertension and she did not have the typical findings of corkscrew vessels, elevated IOP, mid-dilated pupils or an ocular bruit on examination. At this point, there was concern for a rapidly growing retrobulbar mass.
Workup and management
MRI of the brain and orbits with and without contrast and fat saturation was obtained urgently. It showed a large left en plaque intraosseous sphenoid wing meningioma with extension into the orbit, displacing the lateral rectus medially, compressing the optic nerve and causing proptosis (Figure 2).
Discussion
Meningiomas are slow-growing benign neoplasms that arise from outer arachnoid meningeal epithelial cells. The annual incidence of symptomatic meningioma is approximately two per 100,000. Sphenoid wing meningiomas account for 11% to 20% of intracranial meningiomas and can have significant ophthalmic manifestations due to intraorbital extension. It is twice as common in women, and the average age of onset is 50 years old. Meningiomas have been reported to grow dramatically during pregnancy, likely secondary to hormonal changes, which coincided with our patient’s course. Sixty percent of sporadic meningiomas are found to have mutations in the NF2 gene on chromosome 224.
Clinical features of sphenoid wing meningiomas vary depending on the location. Given their anatomical position, tumors can extend into the orbit through the greater wing of the sphenoid bone, the superior orbital fissure or the cavernous sinus. Patients often report uniform proptosis (79%), vision decline (20% to 25%), globe displacement, impaired extraocular motility, diplopia, eyelid edema and chemosis.
Diagnosis is confirmed with brain and orbit imaging, including CT and/or MRI. CT imaging is useful in detecting reactive hyperostosis and calcifications within the lesion, while MRI often demonstrates the characteristic dural tail or dural extension seen in meningiomas.
Management options include observation, surgical excision, radiation and, less commonly, chemotherapy. The decision to pursue intervention is often dependent on the severity of symptoms, which include proptosis, compressive optic neuropathy and motility impairment. Otherwise, the natural history of the lesion is slow growing and can be monitored with serial imaging. Complete resection of meningiomas is rarely achieved. Specifically, en plaque meningiomas, which describes a subgroup of meningiomas defined by its invasive sheet-like dural involvement and significant bone invasion, have an especially high rate of recurrence. Radiation therapy has been traditionally reserved for patients who are poor surgical candidates or as adjuvant therapy in cases of incomplete resection. Chemotherapy is rarely used in the treatment of meningiomas. However, clinical trials are currently underway evaluating the MEK inhibitor selumetinib in the treatment of neurofibromatosis type 2 tumors including meningioma.
Case resolution
After imaging revealed the meningioma, the patient was urgently referred to neurosurgery, which recommended surgical resection. She had a head CT without contrast for further evaluation and surgical planning, and she underwent left craniotomy for excision of the sphenoid wing meningioma. She tolerated the procedure well without any complications. She is scheduled for neuro-ophthalmology follow-up in 2 months.
- References:
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- Güdük M, et al. World Neurosurg. 2019;doi:10.1016/j.wneu.2018.12.175.
- Hortobágyi T, et al. Open Med (Wars). 2017;doi:10.1515/med-2017-0029.
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- Trial of selumetinib in patients with neurofibromatosis type II related tumors (SEL-TH-1601). https://clinicaltrials.gov/ct2/show/NCT03095248. Updated April 14, 2022. Accessed Aug. 5, 2022.
- For more information:
- Yi Ling Dai, MD, and Laurel N. Vuong, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Yi Ling Dai, MD, and Teresa P. Horan, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.