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Primary intraorbital inflammatory lumpy lesion: A rare case report.

Jinxin Yang¹, Qianlei Liang², Liang Han³

¹Department of Neurosurgery, The Second Hospital of Jilin University, Changchun, China.

|April 19, 2024

Summary

Eosinophilic angiocentric fibrosis (EAF) is a rare orbital disease, often linked to IgG4-related conditions. This case highlights a successful surgical treatment of IgG4-negative orbital EAF, improving vision and eye movement.

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Area of Science:

Ophthalmology
Pathology
Immunology

Background:

Eosinophilic angiocentric fibrosis (EAF) is typically a benign IgG4-related disease, commonly affecting the nasal cavity.
Orbital EAF is exceptionally rare, presenting unique diagnostic and therapeutic challenges.

Observation:

A 68-year-old male presented with a 2-month history of vision loss, proptosis, and limited extraocular motility in the left eye.
Orbital CT revealed a lesion, and subsequent examination showed impaired eyelid function and restricted eyeball movement.

Findings:

Postoperative pathology confirmed IgG4-negative EAF, a rare variant.
Surgical radical resection followed by glucocorticoid therapy resulted in improved visual acuity to 0.6 and partial relief of proptosis and motility deficits.

Implications:

This case underscores that orbital EAF can be IgG4-negative, expanding the differential diagnosis for orbital inflammatory lesions.
Successful management with the fronto orbitozygomatic approach and adjuvant therapy demonstrates a viable treatment strategy for this rare condition.