Thyroid orbitopathy (also known as Graves’ Disease) is an idiopathic (i.e. of uncertain etiology) autoimmune inflammatory disorder that affects the orbit (i.e. eyelids, extraocular muscles and other soft tissues surrounding the eyes). The main targets of the orbital disease is represented by the orbital muscles that control the movements of the eyeglobe and the fat that is behind the eyeglobe.
The inflammatory response that occurs in the orbital fat and/or orbital muscles causes all the changes that affect the eyes, like proptosis (the eye popping forward), eyelid retraction (excessive opening of the upper and lower eyelids that give the ‘scared look’), double vision including a 4% risk of vision loss for compression of the optic nerve.
Patients with this disorder often have an associated thyroid abnormality which may present either before, during or after the orbital signs and symptoms, with significant hormonal changes. Thyroid orbitopathy can develop and affect patients with varying degrees of severity. It mainly affects young females greatly affecting their appearance and function.
The disease can begin suddenly and progress rapidly over days to weeks or progress gradually over a longer period of time. Typically the disease has two separate phases: phase 1, the inflammatory phase, during which most patients have mild inflammation symptom including pain, ‘pink eyes’ and swelling around the eyes.
Associated with these inflammatory symptoms, other signs more specific of thyroid orbitopathy may occur and include retraction of the upper eyelids and bulging of the eyes (also known as exophthalmos).
The combination of excessive eyelid opening (retraction) and increase eye globe projection (exophthalmos) cause the common appearance of the thyroid orbithopathy.
Occasionally patients may also have varying degrees of double vision (diplopia). In the worse cases, fortunately less than 5% of the patients with thyroid orbithopathy risk visual loss due to the compression on the optic nerve from the extraocual muscle that have an increased size, especially at the apex of the orbit.
In most cases, the inflammatory process is self-limited and runs a course lasting 6 months to a few years before subsiding. After the inflammatory phase of the disease subsides, there is the phase 2, called fibrotic phase, characterized by resolution of the inflammation (redness, swelling, and pain), but may persist the periocular changes that occurred in the first phase, with eyelid retraction, proptosis and double vision.
Evaluation and Management of Thyroid orbitopathy
It is important to be evaluated by an ophthalmologist to assess the ophthalmic manifestations of the disease as well as by an endocrinologist to manage concurrent thyroid abnormalities.
Patients with mild orbitopathy are usually evaluated on an interval basis to monitor progression of the disease and managed with measures to reduce ocular symptoms. Patients with moderate to severe orbitopathy may require medical or surgical intervention to reduce inflammation or improve vision. Usually in the first phase steroids may be administered systemically or locally, with peri ocular injections.
Peri-ocular steroid injection is an excellent method alternative to systemic steroid to control orbital inflammation avoiding the risks of systemic side effects of the systemic steroid treatment. The periocular steroid injection is painless and can be performed in the office with minimal downtime. The role of steroid treatment is to accelerate the passing of the inflammatory phase and reduce the orbital changes, the ‘damage’, that occur during this phase. Systemic steroid treatment, be it oral or I.V. is administered by the endocrinologist while periocular steroid are administered by a trained ophthalmologist.
Once the inflammatory phase of the disease has subsided, steroid are not necessary and patients with eyelid abnormalities or exophthalmos may be eligible for surgical correction to improve their function and appearance. In these cases there a number of surgical techniques used to restore a normal function and appearance to the eye.
To correct the eyelid changes, including eyelid retraction and puffiness the surgery is directed to a technique to lower the upper eyelid via the upper lid crease similarly to what we do in aesthetic eyelid surgery; the desired eyelid height is evaluated in the surgical theatre and the procedure can be associated with removal of excess tissues to eliminate the swelling.
In case of exophthalmos it may be necessary to perform an orbital decompression, where more space behind the eye is created removing selected parts of the orbital bones that surround the sinuses and excess fat is removed. This procedure is performed in general anesthesia, but through invisible scars and the patients and discharged the following day.
It is possible to associate orbital decompression with eyelid surgery to achieve the best result at the same time.
Cigarette smoking has been associated with development of worsening thyroid orbitopathy, therefore cessation of smoking is universally recommended in these patients.
In general orbital surgeons tend to consider ideal to perform orbital and eyelid surgery in separate steps with
1) orbital decompression;
2) eyelid retraction;
In my practice I perform the three procedures at the same time allowing a full recovery and a complete result after just one procedure.