Unilateral Adult Xanthogranulomatous Infiltration of the Conjunctiva, Limbus and Sclera Leading to a Combined Ophthalmic Artery and Central Retinal Vein Occlusion.

Burak Unlu,Banu Lebe,Ziya Ayhan,Ismet Durak,Suleyman Men,Ali Osman Saatci

doi:10.2174/1874364101711010362

Burak Unlu, Banu Lebe + Show 4 more

Open Access

https://doi.org/10.2174/1874364101711010362

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Abstract

Objective:To describe the features of a female patient with a biopsy-proven xanthogranulomatous infiltration of the conjunctiva, limbus and sclera who had an exudative retinal detachment, combined ophthalmic artery and central retinal vein occlusion unilaterally.Method:A-53-year old otherwise healthy woman presenting with a painful visual loss in her right eye underwent an ophthalmic examination, meticulous systemic work-up and histopathologic assessment.Results:Ophthalmic examination revealed multiple subconjunctival masses, upper limbal infiltrations, trace cells in the anterior chamber, pale looking posterior fundus, 360 degree scattered retinal hemorrhages and marked exudative retinal detachment in her right eye. Left eye was completely normal.A biopsy taken from one of the subconjunctival masses demonstrated a diffuse infiltration of the histiocytes and this was interpreted as a xanthogranulomatous infiltration with the help of immunohistochemical staining techniques.Conclusion:Present case is the only reported adult case with xanthogranulomatous-like infiltration of the eyeball featuring both anterior and posterior segment involvement without any concomitant major systemic disturbances.

Highlights

Histiocytic disorders can be classified into three categories: Class I (Langerhans cell histiocytosis-histiocytosis X spectrum), Class II (Histiocytosis of mononuclear phagocytes other than Langerhans cells) and Class III (Malignant histiocytic disorders) [1]
Adult xanthogranulomatous ocular disease is a class II type disease [2]. It is a very uncommon disorder and generally affects the middle-aged individuals without any sex predilection. It can present as subcutaneous, subconjunctival and periocular xanthochromic infiltrates with varying sized mass formation
We hereby report a 53-year-old woman who had unilateral biopsy-proven adult xanthogranulomatous infiltration including conjunctiva, limbus and sclera resulting in a combined ophthalmic artery occlusion and central retinal vein occlusion

Summary

Results

Ophthalmic examination revealed multiple subconjunctival masses, upper limbal infiltrations, trace cells in the anterior chamber, pale looking posterior fundus, 360 degree scattered retinal hemorrhages and marked exudative retinal detachment in her right eye. Left eye was completely normal.A biopsy taken from one of the subconjunctival masses demonstrated a diffuse infiltration of the histiocytes and this was interpreted as a xanthogranulomatous infiltration with the help of immunohistochemical staining techniques

Conclusion

INTRODUCTION

REPORT OF A CASE

DISCUSSION