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):
- Elevation defect (fibrosis of inferior rectus)
- Abduction defect (fibrosis of medial rectus)
- Depression defect (fibrosis of superior rectus)
- 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
Complication
- Compressive optic neuropathy → Vision loss
Diagnosis
CT-scan:
Investigation of choice
Management
Medical management:
- Systemic steroids (treatment of choice)
- Teprotumumab
- Radiation therapy
Surgical management:
- Orbital decompression surgery
- Extraocular muscle surgery
- Eyelid surgery
