Abstract Listings 2026

Purpose

Amyloidosis is a rare disease in which abnormal proteins are deposited in the extracellular space, and involvement of the orbit and ocular adnexa is a rare form accounting for 4% of head and neck amyloidosis. The purpose of this study is to review the clinical spectrum and diagnostic approaches of this disease showing diverse clinical manifestations, and to emphasize the importance of differential diagnosis from fatal systemic involvement.

Methods

Five cases histologically confirmed at 000 Hospital from January 2015 to October 2025 were retrospectively analyzed. Sixteen articles (24 cases) published between 2004 and 2024 were collected from PubMed, KoreaMed, and KMBASE, and a total of 29 cases were subjected to integrated descriptive analysis. Mann–Whitney U test and Fisher’s exact test were used for comparison between the localized involvement group and the systemic/undetermined involvement group. The diagnosis was confirmed by apple-green birefringence under polarized light on Congo red staining.

Results

The mean age of the five institutional cases was 51 years (Range 39–63), and 60% were female. In the integrated 29 cases, the mean age was 48.2 years, 67.9% were female, and conjunctival involvement was the most common at 52.1%. In the comparison between the localized involvement group (n=21) and the systemic/undetermined involvement group (n=5), a significant difference in the rate of bilateral involvement was observed (4.8% vs 60.0%, p=0.014). In two cases, diffuse uptake in the left ventricular myocardium was observed on PET-CT, suggesting the possibility of potential cardiac involvement. The main treatment was surgical excision, and the recurrence rate was 22.7%.

Conclusion

Orbital and ocular adnexal amyloidosis requires differential diagnosis from various other diseases, and confirmation through tissue biopsy is most important. Even with localized symptoms, it is essential to evaluate the presence of systemic involvement for prognosis and treatment planning, and bilateral involvement is associated with a significantly higher possibility of systemic involvement, thus thorough systemic evaluation including PET-CT is required.

Additional Authors

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