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Key Features

  • A syndrome of clinical and orbital imaging abnormalities caused by deposition of mucopolysaccharides and infiltration with chronic inflammatory cells of the orbital tissues, particularly the extraocular muscles

  • Usually occurs in association with autoimmune hyperthyroidism

  • Radioiodine therapy, possibly indirectly due to induction of hypothyroidism, and cigarette smoking increase its severity

  • Ethanol injection of thyroid nodules has been reported to be followed by severe disease

  • Ocular myasthenia and thyroid eye disease are associated and may coexist; presence of ptosis rather than eyelid retraction is characteristic of the former

Clinical Findings

  • Primary clinical features

    • Proptosis

    • Lid retraction and lid lag

    • Conjunctival chemosis and episcleral inflammation

    • Extraocular muscle dysfunction

  • Resulting symptoms

    • Cosmetic abnormalities

    • Surface irritation, which usually responds to artificial tears

    • Diplopia

  • Important complications are corneal exposure and optic nerve compression, both of which may lead to marked visual loss

Diagnosis

  • The primary imaging features are enlargement of the extraocular muscles, usually affecting both orbits

  • Clinical or laboratory evidence of thyroid dysfunction and thyroid autoantibodies may not be detectable at presentation or even on long-term follow-up

  • Clinical and imaging abnormalities of thyroid eye disease may be mimicked by dural carotico-cavernous fistula

Treatment

  • Euthyroidism should be established and maintained in all cases

  • Mild disease: Oral selenium seems to be beneficial

  • Moderately severe disease: Treatment options when there is no visual loss is controversial

    • Peribulbar corticosteroid injections have been advocated

    • Surgical decompression may be justified in patients with marked proptosis

    • Lateral tarsorrhaphy may be used for moderately severe corneal exposure

  • Severe disease: Treatment options for optic nerve compression or severe corneal exposure include

    • Intravenous pulse methylprednisolone therapy (eg, 1 g daily for 3 days, repeated weekly or monthly for up to 3 months)

    • Oral prednisolone 80–100 mg/day

    • Radiotherapy

    • Surgery (usually consisting of extensive removal of bone from the medial, inferior, and lateral walls of the orbit)

  • Other procedures are particularly useful for correcting lid retraction but should not be undertaken until the orbital disease is quiescent and orbital decompression or extraocular muscle surgery has been undertaken

  • Diplopia should be treated conservatively (eg, with prisms) in the active stages of the disease and only by surgery when the disease has been static for at least 6 months

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