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Ocular System

Thyroid eye disease (TED)

Thyroid eye disease (TED) or thyroid-associated ophthalmopathy, also known as Graves’ ophthalmopathy is a combination of adnexal and orbital findings that occurs most commonly autoimmune thyroid disease.

Introduction

Thyroid eye disease (TED) or thyroid-associated ophthalmopathy, also known as Graves’ ophthalmopathy is a combination of adnexal and orbital findings that occurs most commonly autoimmune thyroid disease.

  • M/C extrathyroidal involvement of Graves’ disease
  • M/C cause of proptosis in adults

Aetiology

Disease associations:

  • Graves’ disease (M/C, 25–50% cases)
    • Secondary to autoantibody formation to the thyrotropin receptor (TSHR) causing either hyper-stimulation or blockade of the receptor signaling.
  • Hashimoto’s thyroiditis
  • Thyroid carcinoma
  • Primary hyperthyroidism
  • Primary hypothyroidism

Clinical features

  • Upper eyelid retraction with temporal flare
  • Proptosis
  • Periorbital oedema
  • Ocular motility restriction (in order of frequency):
    1. Elevation defect (fibrosis of inferior rectus)
    2. Abduction defect (fibrosis of medial rectus)
    3. Depression defect (fibrosis of superior rectus)
    4. Adduction defect (firbrosis of lateral rectus)

Eponymous signs:

  • Dalrymple sign (M/C clinical feature): Retraction of upper eyelid d/t overaction of Muller’s muscle
  • Enroth’s sign: Oedema esp. of the upper eyelid
  • Gifford’s sign: Difficulty in eversion of upper lid
  • Joffroy’s sign: Absent creases on forehead on upward gaze
  • Kocher sign: Globe lags behind the movement of the upper eyelid on upward gaze
  • Möbius’s sign: Lack of convergence
  • Stellwag’s sign: Infrequent blinking
  • Von Graefe’s sign “lid lag”: Upper lid unable to follow downward movement of eyeball when looking down

Superior limbic keratoconjunctivitis:

Associated with thyroid eye disease
  • Conjunctival injection
  • Chemosis
a. Typical clinical appearance of a patient with TED, demonstrating lid retraction, proptosis, conjunctival injection and chemosis b. Detail demonstrating marked conjunctival injection over the insertion of the medial rectus and caruncular edema in the same patient. | Briceño, C. A., Gupta, S., & Douglas, R. S. (2013). Advances in the management of thyroid eye disease. International ophthalmology clinics, 53(3), 93–101. https://doi.org/10.1097/IIO.0b013e318293c44e

Complication

  • Compressive optic neuropathy → Vision loss

Diagnosis

CT-scan:

Investigation of choice
Orbital computed tomography images showing enlarged inferior and medial rectus muscles in a patient with thyroid-associated ophthalmopathy. The inferior rectus muscle is enlarged, mimicing an orbital tumor | Şahlı, E., & Gündüz, K. (2017). Thyroid-associated Ophthalmopathy. Turkish journal of ophthalmology, 47(2), 94–105. https://doi.org/10.4274/tjo.80688

Management

Medical management:

  • Systemic steroids (treatment of choice)
  • Teprotumumab
  • Radiation therapy
A 61-year-old female patient with infiltrative thyroid-associated ophthalmopathy. The patient exhibted significant palpebral and conjunctival edema and reported severe pain (A). The same patient showed substantial regression of clinical signs after 3 months of intravenous corticosteroid therapy (B) | Şahlı, E., & Gündüz, K. (2017). Thyroid-associated Ophthalmopathy. Turkish journal of ophthalmology, 47(2), 94–105. https://doi.org/10.4274/tjo.80688

Surgical management:

  • Orbital decompression surgery
  • Extraocular muscle surgery
  • Eyelid surgery
Coronal computed tomography of a patient with thyroid-associated ophthalmopathy (A). Coronal computed tomography images from the same patient after orbital decompression surgery (B). Postoperative images show the absence of the medial orbital wall and thinning of the cortical bone in the lateral wall | Şahlı, E., & Gündüz, K. (2017). Thyroid-associated Ophthalmopathy. Turkish journal of ophthalmology, 47(2), 94–105. https://doi.org/10.4274/tjo.80688

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