Case Presentation: A 48-year-old female with past medical history including obesity, T2DM, and tobacco use presented with right eye swelling, pain, and vision loss. She began noticing a dark spot in her right eye about three months prior, which progressed to a pressure-like sensation. A CTA of head and neck showed cavernous sinus hyperdensity. MRI of the brain also showed abnormalities in the cavernous sinus, orbital fissures, and right orbit. Ophthalmology evaluation showed a right afferent pupillary defect, decreased light touch sensation in CN V, and many studies came back non-contributory, including ANA, ANCA, MPO, IgG, IgG4, RPR, treponemal antibodies, Quantiferon gold, ACE, and CSF analysis. HIV and HSV testing were also negative. She went to an ER, followed by unrevealing work-up, and was discharged on steroids that improved her symptoms.She presented again after running out of steroids. While inpatient, rheumatology believed that Tolosa-Hunt syndrome was most likely given the dural-based inflammation with right orbital involvement, extension to tentorial leaflets, and thrombosis of cavernous sinuses from previous radiographic imaging. On PET and MRI, there was increased uptake in the lateral aspect of the right orbit and possible inflammatory orbital pseudotumor. The patient also had a right orbitotomy with biopsies showing inflammation, but no morphologic evidence for involvement by other common etiologies. The patient’s vision improved with IV methylprednisone and IV rituximab therapy and started on another prednisone taper outpatient.
Discussion: Cavernous Sinus Syndrome (CSS) describes any pathology of the cavernous sinus with signs/symptoms of ophthalmoplegia, autonomic dysfunction, chemosis, proptosis, vision loss, and/or sensory CN V1- CN V2 palsy. The most common etiologies of CSS include tumor and trauma. Infection, inflammation, and vascular anomalies represent more uncommon etiologies (1,2). In cases of CSS, unilateral ophthalmoplegia is the predominant presenting finding while visual field defects are uncommon. Symptoms are typically accompanied by unilateral pain (2).We report an unusual case of cavernous sinus pathology with suspected inflammatory etiology resulting in CN II deficit, CN V deficit, proptosis, and pain without the classical finding of ophthalmoplegia. Differentials included Tolosa-Hunt Syndrome and orbital pseudotumor, two inflammatory etiologies that are diagnoses of exclusion. However, Tolosa-Hunt Syndrome requires ophthalmoplegia as part of its diagnostic criteria. Therefore, the patient did not meet criteria despite having most other classic findings such as inflammation on biopsy, orbital pain, relief with corticosteroids, and MRI with dural enlargement (3,4). Of note, diagnostic criteria for Tolosa-Hunt was established prior to the common use of MRI and advances in neuroimaging (4). Similarly, the patient had many of the common orbital pseudotumor findings such as orbital pain, swelling, inflammation on biopsy, and MRI with soft tissue lesions that enhance with contrast, but no ophthalmoplegia (5,6).
Conclusions: We present a particularly unusual case of cavernous sinus pathology with other common signs of CSS, but without ophthalmoplegia. This case highlights the need for reevaluation of diagnostic criteria for CSS etiologies, and in the case of Tolosa-Hunt Syndrome, the revision of criteria with advances in knowledge of the condition.