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  • Updated 06.13.2023
  • Released 10.12.2000
  • Expires For CME 06.13.2026

Orbital disease in neuro-ophthalmology



Orbital pathology can produce neuro-ophthalmic manifestations, such as optic neuropathy and ocular misalignment, leading to vision loss and diplopia. Among the common orbital conditions are thyroid eye disease, lymphoproliferative disease, and idiopathic orbital inflammatory syndrome.

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 increased resistance to retropulsion.

• The most common orbital condition is thyroid eye disease.

Historical note and terminology

Orbital pathology can be categorized according to disease mechanism:

1. Inflammation

a. Thyroid eye disease
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 eye disease, idiopathic orbital inflammatory syndrome, malignant orbital tumors, and orbital cellulitis.

The most common orbital disease is thyroid eye disease, accounting for 47% of orbital disease, followed by lymphoproliferative disorders in 10.2% (39). The third most common is orbital inflammatory syndrome, accounting for approximately 5%.

Thyroid eye disease was previously 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” (26).

Idiopathic orbital inflammatory syndrome, or nonspecific orbital inflammation, was previously known as orbital pseudotumor due to a tendency to simulate orbital malignancy. It is a benign, noninfective clinical syndrome characterized by nonspecific orbital inflammation without an identifiable local or systemic cause. This condition must be differentiated from specific causes of orbital inflammation, which may produce a clinically indistinguishable syndrome. Other names for the condition include idiopathic orbital inflammation and nonspecific orbital inflammation.

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