A rare differential diagnosis of the orbitopathy presenting with exophthalmus

Introduction:

Orbitopathies are rare diseases in which interdisciplinary diagnostic and therapy is required. The ENT is consulted because of e.g. an exopthalmus, or patients are referred with the question of a surgical intervention.

Case report:

A 56 year old female patient was admitted to the hospital with exophthalmus, impaired vision and nearly complete ophthalmoplegia of the left eye with the question, whether orbital decompression is necessary due to presumed Graves' orbitopathy, which was substantiated by a MRI displaying enlarged extraocular muscles without compression of the optic nerve.

The patient already presented two months earlier with similar symptoms of the right eye that subsided with medical treatment (high-dose corticosteroids). A retrobulbar irradiation was not performed since the patient showed clinical improvement, there was no histological proof of Graves' and thyroid function tests were normal.

Based on the clinical features and the radiological findings the working diagnosis of an active Graves' disease resistant to therapy led to the indication for an orbital decompression with orbital biopsy. An iv-therapy with corticoids and antibiotics was started. Laboratory testing showed negative markers as e.g. TRAb, ANA, c-/p-ANCA and IL-2-receptor. The histologic report revealed the diagnosis of an ocular myositis (OM). The therapy with corticoids was continued and reduced step by step. In the follow-up examination clinical symptoms and general condition had improved.

Conclusion:

The OM is a rare differential diagnosis (DD) of orbitopathies with unknown etiology. To proof the diagnosis other DD need to be excluded. In contrast to Graves' there is no association to thyroid abnormalities. The majority of patients show a good response to corticoids.

Publication History

Publication Date:
12 June 2019 (online)