Graves' disease (also called thyroid ophthalmopathy, dys-thyroid ophthalmopathy, or endocrine orbitopathy) is an autoimmune disease that commonly, though not always, is associated with hyperthyroidism. It is accompanied by a broad spectrum of signs and symptoms of orbital inflammation. Chief among these is exophthalmos. Graves' disease is the most common cause of exophthalmos among
adults, and it is usually a bilateral and often very asymmetric disorder. This can lead to misdiagnoses during the early stages of the disease.
Initial symptoms most commonly include foreign-body sensation, a feeling of retrobulbar pressure or pain, tearing, blurring, and photophobia. With later inflammatory involvement of the extraocular muscles, diplopia begins to appear. All of these symptoms have a diurnal cycle, with the most symptomatic period at the time of awakening in the morning. In addition, and virtually pathognomonic, is the appearance of lid signs, including lid swelling, lower lid retraction, upper lid retraction with lid lag (not moving in close synchrony with the globe during downward pursuit movements), and infrequent blinking or a staring expression (■ Fig. 9.5). These facial features are for many patients the most intolerable aspect of the disease. In addition, it can be accompanied by an acute or chronic swelling of the lacrimal gland. Other signs include conjunctival hyper-emia, chemosis, plical hyperemia, injection over the rectus muscle insertions, and exposure keratopathy. Mechanical strabismus arises from inflammation in the medial and inferior rectus muscles, which are the two muscles most commonly affected. Fibrotic foreshortening of the muscles tethers the globe, limiting the ability to elevate or abduct the eye. Finally, a secondary glaucoma can develop and retinal or choroidal signs of optic nerve compression can appear. These include relative or absolute central scotomas, relative afferent pupillary defect, profound loss of visual acuity and color perception, and optic disc edema with surface exudates may be present. This is an emergent disorder that requires immediate reversal, usually accomplished by administering high doses of corticosteroids, followed by surgical decompression of the optic canal and orbital apex from an ethmoid approach.
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