Orbital pseudotumor: a differential diagnosis of Graves' ophthalmopathy

Abstract

SUMMARYThe objective of this study is to report and discuss a rare and inflammatory cause of exophthalmos. This report describes a patient with exophthalmos, who was initially diagnosed with euthyroid Graves' with good response to therapy. After 8 years of follow-up, she had recurrence of symptoms and a new evaluation revealed the final diagnosis of orbital pseudotumor. Orbital pseudotumor is an uncommon disorder that both radiologically and clinically mimics a malignant process or other inflammatory disease, such as Graves' ophthalmopathy. Arq Bras Endocrinol Metab. 2011;55(1):85-8 SUMÁRIO O objetivo deste estudo é relatar e discutir uma causa de exoftalmia rara e inflamatória. Este artigo relata uma paciente com exoftalmia que recebeu diagnóstico inicial de oftalmopatia de Graves eutireoideana com boa resposta à terapia. Após oito anos de seguimento, houve recorrência dos sintomas e uma nova avaliação revelou o diagnóstico final de pseudotumor orbitário. Pseudotumor orbitário é uma condição incomum que mimetiza clínica e radiologicamente uma doença maligna ou inflamatória, como a oftalmopatia de Graves. Arq Bras Endocrinol Metab. 2011;55(1):85-8 clinical case report

Atypical Early Presentation Of Orbital Pseudotumor: A Case Report

“…The proptosis, ptosis, chemosis, and painful ocular motility in our patient improve simultaneously after corticosteroid pulse therapy. Indeed, the neuro-ophthalamic deficits in previous cases of atypical OPT also responded well to corticosteroid therapy (10)(11)(12)(13)(14)(15)(16). These therapeutic findings may reflect the unique underlying inflammatory nature despite of distinct clinical manifestations or types in OPT.…”

“…In our patient, the dilemma is an absence of the typical painful eye, oculomotor deficit and ocular edema at initial, and an isolated equivocal eyelid drooling and chemosis may be misinterpreted as an local conjunctivitis, or even the ocular sympathetic dysfunction or cavernous sinus disorder. In fact, atypically early manifestation of OPT has only been mentioned in a few sporadic cases before, such as chronic visual impairment (10), painless proptosis (11,12,13), chronic palpebral edema (14), isolated abducens nerve palsy (15), and isolated oculomotor myositis (16). Therefore, OPT may present atypically and simulate any ophthalamic or neuro-ophthalmic disorder, and renders the diagnostic difficulty to general practitioner.…”

Orbital Pseudotumor Can Mimic Graves’ Ophthalmopathy

“…In 1903, Gleason, Busse and Hochheim first described a new condition known as orbital pseudotumor (OP) or idiopathic orbital inflammatory disease (5,8). OP is a benign inflammation, noninfectious, space-occupying orbital lesion with no identifiable local or systemic etiology, involving any orbit structure (myositis, dacryoadenitis, perineuritis, tendonitis, episcleritis, and localized mass) (1,5).…”

“…Some reserchers have tried to identify the pathogenesis of this inflammation, but it is still not clear. They described a real association with infection, trauma, surgery and with autoimmune disorders (Crohn's disease, Sjögren's syndrome, Behçet's disease, lupus, rheumatoid arthritis, diabetes mellitus, myasthenia gravis, and ankylosing spondylitis) suggesting an immune-mediated process (5,9).…”

“…Graves' ophthalmopathy (GO) is the most common cause of proptosis, unilateral or bilateral. Hyperthyroidism can be associated or not with GO (1,5). In some cases, the diagnostic of GO is not very clear because orbital imaging like computed tomography (CT) or magnetic resonance imaging (MRI) describe orbital inflammation masses and thyrotropin-receptor antibodies (TRAb) as normal with their high specificity and sensitivity in Graves' disease.…”