- Clinical science
The orbital cavity (eye socket) is the bony cavity that encloses the bulb and accessory organs of the eye such as the ocular muscles, lacrimal glands, nerves, vessels, and retrobulbar adipose tissue. Diseases of the orbital cavity include Graves ophthalmopathy, orbital cellulitis, rhabdomyosarcoma, and lacrimal sac disorders. Typical symptoms associated with these diseases include exophthalmos and diplopia. Treatment differs according to the underlying disease and includes conservative measures (antibiotics), surgery, radiotherapy, and chemotherapy.
are discussed in a separate learning card.
- Definition: Graves ophthalmopathy or orbitopathy (GO) (also known as thyroid-associated orbitopathy or ophthalmopathy, TAO) is an autoimmune condition that is generally associated with
- Sex: ♀ > ♂ Although it is more common in women, severe cases occur more frequently in men and patients > 50 years of age.
- Risk factors: family history of Graves disease and tobacco smoking
- TSH autoantibodies are present in the orbital cavity; (eye socket) → bind TSH receptor antigen; (autoimmune reaction) on cells → lymphocytic infiltration into the orbital tissues → inflammation and release of cytokines from CD4+ T cells; → stimulates fibroblasts to secrete glycosaminoglycans (hyaluronic acid), which also pulls water into the interstitial space (osmotic effect); → expansion of retro-orbital tissue due to increased fluid in extraocular muscles, lymphocytic infiltration, and adipogenesis
- Exophthalmos : can be unilateral or bilateral, often asymmetric. Retropulsion (palpation of the globe while the eyelid is closed) enable adequate examination.
- Ocular motility disturbances
Lid retraction, also known as “thyroid stare”
- Dalrymple sign: Retraction of the upper eyelid with visible sclera and extended palpebral fissure
- Von Graefe sign: Lagging of the upper eyelid on downgaze (may occur with Grove sign: resistance to pulling the retracted upper lid down)
- Stellwag sign: Infrequent and incomplete blinking (rare)
- Vigouroux sign: Eyelid fullness
- Lagophthalmos → keratitis (occurs with insufficient blinking)
- Joffroy sign: absent forehead creases during superior gaze
- Conjunctival injection and chemosis
- Ocular discomfort (pain or pressure)
- Photopsia on upward gaze
- Patient may be hyperthyroid, euthyroid, or hypothyroid.
- Loss of vision is uncommon.
- There are several severity scales, but treatment is based on mild active disease vs. severe active disease, and whether or not the patient has a risk of blindness.
- Severity is associated with level of TSAb, but not associated thyroid function.
- Laboratory analysis: ↓ TSH and ↑ free T3/T4; (to diagnose of hyperthyroidism), ↑ TSH receptor antibodies; , which are specific and sensitive to Graves disease but not widely available
- Slit lamp examination
- CT: confirmatory test that shows exophthalmos, increased fat density and inflammation and enlargement of extraocular muscles, and helps monitor progression of disease
- MRI: alternative option to CT; that can show similar findings to CT and, additionally, compression of the optic nerve
- Photo documentation
- Differential diagnosis: pseudoexophthalmos
- This is a self-limiting disease, but intervention may be necessary because of severe symptoms or risk of complications.
- Goals of therapy include treatment of hyperthyroidism, smoking cessation, eye protection, and decreasing inflammation.
- Radioiodine therapy may initially worsen the appearance and symptoms of the disease, while surgery and thioamides do not affect the disease.
- If radioiodine therapy is used, steroids are recommended before and after treatment.
- Mild disease
- Moderate to severe inflammation, nonresponders, or threatened/manifested vision loss: high-dose IV steroids
- If response is good: consider orbital radiation
If no response after 48–72 hours → surgical decompression while on steroids in the following order:
- Orbital decompression
- Strabismus correction
- Lid-lengthening surgery
- Other options for patients who are refractory to treatment or poorly tolerate glucocorticoids: rituximab, cyclosporine, octreotide, intravenous immunoglobulin, and tarsorrhaphy
- Definition: an infection of the orbital contents, such as the fat and extraocular muscles, posterior to the orbital septum (in contrast to )
- Epidemiology: more common in young children than older children and adults
- Clinical features
- Mostly clinical diagnosis
- Laboratory tests: leukocytosis, positive blood and tissue fluid cultures
- CT: confirm diagnosis and look for complications, such as orbital abscess or intracranial extension
- Definition: Preseptal cellulitis is an infection that is located anterior to the orbital septum that does not involve orbital contents.
- Clinical features
- Diagnosis: CT demonstrates only eyelid swelling
- Although preseptal cellulitis can cause severe complications, these are rare, which is why the condition is rarely life-threatening.
- Preseptal Cellulitis
- Empiric IV antibiotic treatment includes vancomycin plus one of the following: ceftriaxone, cefotaxime, ampicillin-sulbactam, piperacillin-tazobactam, or, if the patient is allergic to penicillins and cephalosporins, either ciprofloxacin or levofloxacin to cover S. aureus for 1-2 weeks
- If intracranial extension is suspected, metronidazole should be added to ceftriaxone or cefotaxime to cover anaerobes
- For treatment of mucormycosis, see
- After appropriate response in terms of fever and orbital findings, therapy can be switched to an oral antibiotic such as clindamycin alone OR clindamycin or trimethoprim-sulfamethoxazole PLUS amoxicillin-clavulanate, cefpodoxime, or cefdinir for 2–3 weeks
- Ophthalmology and otolaryngology consultation for possible surgical intervention
Reduced vision, diplopia, ophthalmoplegia, and proptosis are typical features of orbital cellulitis, and do not occur in preseptal cellulitis!
- Definition: malignant mesenchymal tumor of primitive muscle cells
- Frequently in the orbital cavity, but can also be in the head and neck, urogenital region , or extremities
- Growing orbital mass that may be painful and have potential hemorrhage
- Proptosis or dysconjugate gaze
- Open or core needle biopsy for light microscopy to look for rhabdomyoblasts
- X-ray of the primary site and chest: to determine any bone and lung involvement for staging
- CT of the primary site and chest: to search for any lung metastases and bone destruction and determine therapeutic response
- MRI: better to determine specific location of mass and any soft tissue invasion
- Surgery (complete excision) if a functional and cosmetic result is possible
- Combination of radiation and chemotherapy following a diagnostic biopsy if complete excision is not feasible
- Prognosis: more favorable for localized tumors of the orbit (> 80% 5-year survival), and less favorable for metastatic disease (< 30% 5-year event-free survival)