Sunday Okonkwo, Fariha Taimur, Amna Manzoor, Maheen Akbar, Saadullah Ahmad, Tayyab Afghani.
Unusual Features of Orbital Xanthogranuloma: a Case Series.
Al-Shifa J Ophthalmol Jan ;17(1):36-45.

Purpose: To describe previously unreported features of patients with orbital xanthogranuloma. Methods: This was a retrospective study of 5 patients with orbital xanthogranuloma treated and followed up within a twelve-year period from January 2007 to December 2018. Their clinical records were reviewed and analyzed. Results: There were 2 cases each of juvenile xanthogranuloma and adult onset xanthogranuloma and one case of Erdheim - Chester disease of the orbit. Three patients were males and 2 were females. Three patients had unilateral disease while in two cases it was bilateral. The main presenting complaints were proptosis and loss of vision seen in 4 cases. CT scan demonstrated extraconally located fairly defined masses in all except for the case of Erdheim- Chester disease it was bilateral and intraconal. There was bony erosion involving frontal bone in the two patients with adult onset xanthogranuloma. All patients underwent orbitotomy with subtotal excision of yellowish masses from the orbits. They were all followed up for at least one year (range 1 to 12 years). One of the patients with juvenile orbital xanthogranuloma had bilateral recurrence of the lesion after 8 years. Histopathologic examination revealed foamy histiocytes as prominent feature in all cases and variable number of Touton multinucleated giant cells. Conclusion: Orbital xanthogranuloma is a rare heterogeneous group of diseases with similar histopathologic profile. Diagnosis of the disease is made by considering both clinical and histopathological features. Bony destruction and presence of yellowish orbital masses in adult onset xanthogranuloma are being reported for the first time.

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