Orbital disease in neuro-ophthalmology

Danah Albreiki MBBS (Dr. Albreiki of the Ottawa Eye Institute 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 March 9, 2017; expires March 9, 2020

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.

Overview

Orbital pathology can result in neuro-ophthalmic manifestations such as optic neuropathy and ocular misalignment, leading to vision loss and diplopia. Among the common orbital conditions are thyroid-related orbitopathy (TRO), orbital tumors, and orbital inflammatory syndromes. Other orbitopathies include IgG-4-related disease and orbital infections such as cellulitis.

Key points

 

• History taking and clinical examination of the orbit are important parts of the evaluation.

 

• Early detection may prevent or reduce visual dysfunction.

 

• Orbital pathology may lead to restrictive ocular movements manifesting as diplopia.

 

• Orbital pathology can easily be missed if the orbit is not considered as a site of pathology.

 

• Proptosis is the hallmark of orbital pathology, but it is not always present. To detect it, one could use a Hertel Exophthalmometer and check for resistance to retropulsion.

 

• The most common orbital condition is thyroid-related orbitopathy.

Historical note and terminology

Orbital pathology can be categorized according to disease mechanism:

 

1. Inflammation

 

a. Thyroid-related orbitopathy
b. Orbital inflammatory syndrome (specific or idiopathic)

2. Infection (cellulitis)
3. Tumor

 

a. Benign
b. Malignant

4. Vasculopathy
5. Anomaly

This chapter will cover thyroid-related orbitopathy, idiopathic orbital inflammatory syndrome, malignant orbital tumors, and orbital cellulitis.

The most common orbital disease is thyroid-related orbitopathy, accounting for 32% of all orbital disorders. Next in order of frequency are lymphoproliferative orbital disease (9%), idiopathic orbital inflammatory syndrome (4.3% to 6.3%), vasculopathy (6%), orbital cellulitis (5%), and conditions of unknown cause (5%). A comprehensive review reported that thyroid-related orbitopathy accounted for 47% of orbital disease, with inflammatory disorders accounting for 10% and vasculopathy for 3% (Rootman 2001).

Thyroid-related orbitopathy is also called Graves orbitopathy. The historical background behind naming the disease “Graves” goes back to 1835 when the Irish physician Robert James Graves described a patient with goiter and proptosis. The German physician Karl von Basedow reported the same group of symptoms independently in 1840. Other names for the same disease are Parry disease, Begbie disease, Flajani disease, and Marsh disease. These names are derived from physicians in the early 1800s. But in the 12th century, a Persian physician named Sayyid Ismail Al-Jurani had noted the association between goiter and proptosis, reporting it in his “Thesaurus of the Shah Khwarazm” (Ljunggren 1983).

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