SUN-870 - Severe Graves' Ophthalmopathy Complicated by Spontaneous Enucleation and Threatened Vision in the Contralateral Eye

Background:
Graves’ ophthalmopathy (GO), or thyroid eye disease (TED), is an autoimmune inflammatory disorder that can cause serious ocular damage. While most cases are mild, severe disease can lead to permanent vision loss. In rare, extreme cases, spontaneous enucleation can occur, underscoring the destructive potential of advanced TED. Early recognition and timely treatment are essential, especially in high risk patients.

Case:
A 55-year-old male was diagnosed with Graves’ disease and severe TED at an outside hospital, where he presented with a spontaneous enucleation of the right eye following years of progressive exophthalmos and undiagnosed hyperthyroidism, resulting in permanent blindness of that eye. Several months after this initial presentation, he was hospitalized again with thyrotoxicosis complicated by atrial fibrillation after interruption of the prescribed antithyroid therapy. While hospitalized, he required cardioversion and intensive medical management with propranolol and methimazole. At post hospital follow up in clinic, he was noted to be euthyroid. Examination revealed a contracted right orbital socket and active TED involving the left eye, including presence of exophthalmos, limited painful extraocular movement, and erythema of conjunctiva. Clinical Activity Score of the left eye was 3, consistent with active disease. Laboratory evaluation demonstrated markedly elevated thyrotropin receptor antibodies (>40 IU/L). Given monocular status and active TED, the patient was considered at high risk for bilateral blindness. After providing all available options, Teprotumumab was initiated as disease-modifying therapy to prevent progression and preserve vision in the remaining eye. An appropriate audiology evaluation and glycemic monitoring was planned due to known treatment-associated risks. Currently, euthyroid status is maintained with methimazole, while he receives teprotumumab awaiting outcomes.

Conclusion:
This case illustrates an extreme manifestation of Graves ophthalmopathy resulting in catastrophic vision loss and highlights the importance of early recognition of the disease state and timely initiation of targeted treatment for controlling hyperthyroidism and thyroid eye disease.

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