Orbitopathies affecting vision and ocular alignment

Pete Setabutr MD (Dr. Setabutr of the University of Illinois at Chicago owns shares of Allergan stock.)
Ramesh Singa MD (Dr. Singa of the University of Illinois at Chicago has no relevant financial relationships to disclose.)
Jonathan D Trobe MD, editor. (Dr. Trobe of the University of Michigan has no relevant financial relationships to disclose.)
Originally released October 12, 2000; last updated August 2, 2011; expires August 2, 2014
Notice: This article has expired and is therefore not available for CME credit.

This article includes discussion of orbitopathies affecting vision and ocular alignment, idiopathic orbital inflammatory syndrome, thyroid-related immuno-orbitopathy, thyroid-related immunoorbitopathy, malignant orbital tumors, orbital cellulitis, and orbital inflammatory syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.


Orbital pathologies can affect the optic nerve and extraocular movement that can manifest as loss of vision and diplopia. Among the most common orbital pathologies are thyroid associated orbitopathy, orbital cellulitis, orbital inflammatory disease, and malignant orbital tumors. In this article, the authors include recent developments in workup and management.

Historical note and terminology

Diseases involving the orbit that manifest in altered vision or ocular alignment can be categorized according to pathology and disease processes. The main categories include the following:

(1) inflammatory disorders

(2) orbital cellulitis and other infectious processes

(3) vascular disorders or anomalies

(4) trauma

(5) pseudoproptosis

Several studies have looked at the incidence of orbital disease (Dallow and Pratt 1994). Dallow found that Graves ophthalmopathy was the most common disorder (32%), followed by malignant entities (9% total), idiopathic orbital inflammatory syndrome (6%), pseudoptosis (6%), vascular etiologies (6%), orbital cellulitis (5%), and unknown type (5%). Rootman found thyroid-related orbitopathy in 47%, followed by malignant entities in 22%, other inflammatory disorders in 10%, and vascular lesions in 3% (Dallow and Pratt 1994). Based on these epidemiologic studies, Graves ophthalmopathy is the most common orbitopathy affecting vision and alignment. Poorly understood inflammatory disorders, tumors (usually malignant), and infection comprise the remainder.

In 1835, Graves described a patient with a now classic appearance of Graves ophthalmopathy (Netland and Dallow 1994). This patient also had a goiter and was clinically hyperthyroid. Although he was not the first to describe this constellation of symptoms, it would eventually become known as Graves disease. The ophthalmic component is often referred to as “thyroid-associated orbitopathy” or “ophthalmopathy.” It is now understood that this disorder can be associated with hypothyroidism, Hashimoto thyroiditis, and even euthyroidism (Bartley et al 1996).

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