Abstract Details

Title Trigeminal neuralgia heralding a florid case of Idiopathic Orbital Inflammation

Topic Neuro-ophthalmology/Neuro-otology

Presentation(s) Neuro-ophthalmology/Neuro-otology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 028

Background IOI is a benign inflammatory condition usually confined to the orbit. Rarely, it extends beyond orbit. The sequence of extra orbital involvement is typically through fissures or foramina from the orbit. Literature review reports ~8% of cases with extra orbital extension without information regarding the initial origin of inflammation.

Objective

To report a case of idiopathic orbital inflammation (IOI) originating in the pterygopalatine fossa presenting as trigeminal neuralgia.

Design/Methods Case report

Results 55-year-old woman presented with 6 months of worsening right facial pain and 1 month of progressive eye pain and vision loss. Visual acuity was hand motion on right and 20/100 on left. On right, extraocular movement was restricted in all directions with facial sensation decreased in V1, V2 territory. Orbits showed uniform contrast enhancing lesions involving the right lacrimal gland, lateral and inferior rectus muscles, optic nerve sheath, intraconal and extraconal fat, orbital apex, cavernous sinus, pterygopalatine fossa, and adjacent dura. An MRI performed 3 months after symptoms presented was reviewed retrospectively. It showed enhancement along nerve roots in the right pterygopalatine fossa, indicating maxillary branch of trigeminal nerve involvement prior to orbital/intracranial involvement. Lumbar puncture and bloodwork (autoimmune, sarcoid, IgG subsets) were normal. CSF cytology and flow cytometry were negative for malignancy. Biopsy from the right lacrimal gland revealed chronic non-granulomatous inflammation. Upon treatment with intravenous steroids and a slow taper of oral steroid, her symptoms improved. Visual acuity remained poor. Follow-up MRI after 6-weeks showed resolution of most of the inflammation.

Conclusions IOI is a diagnosis of exclusion and poses a diagnostic challenge due to heterogeneous clinical presentation without specific diagnostic criteria. Our case highlights extra orbital origin of IOI presenting as an isolated cranial neuropathy causing extensive intracranial and extracranial invasion.

Authors/Disclosures
Joshua Smith PRESENTER	Mr. Smith has nothing to disclose.
Prashant Natteru, MBBS (Mayo Clinic Health System)	Dr. Natteru has nothing to disclose.
Chandra Sekhar Mannyam, MBBS (Salem Health)	Dr. Mannyam has nothing to disclose.
Juebin Huang, MD, PhD (University of Mississippi Medical Center)	Dr. Huang has nothing to disclose.
Manasa Gunturu, MD (University of MS Medical Center)	Dr. Gunturu has nothing to disclose.