SHM Converge Scientific Abstract ePoster Gallery-The Border of Cavernous Sinus Syndrome and Tolosa-Hunt Syndrome

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This case describes a 48-year-old woman with obesity, type 2 diabetes, asthma, COPD, fibromyalgia, and tobacco use who developed progressive right eye pain, swelling, and vision loss over three months. Imaging showed cavernous sinus and right orbital abnormalities, with cavernous sinus hyperdensity on CTA and dural-based enhancement on MRI. Ophthalmologic exam revealed a right afferent pupillary defect, but extensive laboratory and CSF testing for autoimmune, infectious, inflammatory, and malignant causes was unrevealing.<br /><br />Her symptoms temporarily improved with high-dose steroids, but recurred after she ran out of prednisone. Based on the imaging and clinical picture, rheumatology considered Tolosa-Hunt syndrome, given inflammatory involvement of the cavernous sinus and orbit with extension to the tentorial leaflets and cavernous sinus thrombosis. PET/MRI later showed increased FDG uptake in the lateral right orbit, suggesting inflammatory orbital pseudotumor.<br /><br />She ultimately underwent right orbitotomy with biopsy of the lacrimal gland and superotemporal orbital mass. Pathology showed inflammation without evidence of sarcoidosis, lymphoma, IgG4-related disease, granulomatous vasculitis, or neoplasia. Interestingly, PR3 was positive, raising concern for a vasculitic process. Her vision improved after treatment with IV methylprednisolone and IV rituximab, followed by an outpatient prednisone taper.<br /><br />The authors emphasize that this was an unusual cavernous sinus syndrome presentation because the patient had pain, proptosis, optic nerve dysfunction, and biopsy-proven inflammation, but lacked the ophthalmoplegia classically required for Tolosa-Hunt syndrome and orbital pseudotumor criteria. The case suggests that existing diagnostic criteria for cavernous sinus syndrome-related inflammatory disorders may be too narrow and may need updating to reflect modern imaging and broader clinical presentations.

Asset Subtitle

Maxwell Hershey

Meta Tag

Author List Kristin Nascimento, Maxwell Hershey, Neemit N. Shah, Pinky Jha, Shanna Su

Category Clinical Vignettes

Concept Cavernous Sinus Syndrome

Concept Tolosa-Hunt Syndrome

Concept Orbital Involvement

Concept Cranial Nerve Deficit

Concept Inflammatory Pseudotumor

Distinguished Non-Finalist

Presenter Organization Medical College of Wisconsin

Presenting Author Maxwell Hershey

Track Adult

Keywords

cavernous sinus syndrome

Tolosa-Hunt syndrome

orbital pseudotumor

right eye pain

vision loss

orbitotomy biopsy

PR3 positivity

rituximab treatment

MRI enhancement

cavernous sinus thrombosis

Cavernous Sinus Syndrome

Tolosa-Hunt Syndrome

Orbital Involvement

Cranial Nerve Deficit

Inflammatory Pseudotumor