Orbital disorders

Last updated: May 31, 2023

Summary

The orbital cavity (eye socket) is the bony cavity that encloses the bulb and accessory organs of the eye, including 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.

Disorders of the lacrimal system are discussed in a separate article.

Graves ophthalmopathy

Definition: Graves ophthalmopathy or orbitopathy (GO) (also known as thyroid-associated orbitopathy/ophthalmopathy, TAO) is an autoimmune condition that is generally associated with Graves disease
Etiology
- TAO is not due to the thyroid disorder, rather due to an autoimmune antibody reaction.
- Associated with hyperthyroidism (most common); , euthyroidism, hypothyroidism such as Hashimoto's thyroiditis; , other autoimmune disorders, thyroid cancer, and neck irradiation.
Epidemiology
- Sex: ♀ > ♂
- Risk factors; : family history of Graves disease and tobacco smoking
Pathophysiology
- 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
Clinical features
- Exophthalmos : can be unilateral or bilateral, often asymmetric. Retropulsion (palpation of the globe while the eyelid is closed) enable adequate examination.
- Ocular motility disturbances
  - Binocular diplopia
  - Poor convergence (Moebius sign)
  - Restriction of one or more extraocular muscles (Ballet sign)
- 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.
Diagnostics
- 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
Treatment
- Usually a self-limiting disease, but intervention may be necessary because of severe symptoms or risk of complications.
- For all patients
  - Conservative local measures
    - Eye protection (e.g., artificial tears, sunglasses)
    - Sleep with the head of the bed elevated
  - Treat hyperthyroidism, if present
    - Usually with thioamides and/or surgery
    - Radioactive iodine ablation (RAIA) can be used for patients with mild disease (contraindicated in moderate-to-severe disease)
  - Avoid smoking
- Mild disease: transient or no diplopia, mild soft tissue involvement, lid retraction < 2 mm, proptosis < 3 mm
  - Conservative measures are usually sufficient
  - Selenium may be considered, if deficient
  - Quercetin
- Moderate to severe disease: inconstant or constant diplopia, moderate to severe soft tissue involvement, lid retraction ≥ 2 mm, proptosis ≥ 3 mm
  - High-dose IV steroids
  - Nonresponders or threatened/manifest vision loss
    - Orbital decompression surgery (following steroid administration)
    - Strabismus correction
    - Lid-lengthening surgery
    - Blepharoplasty
    - For patients who are refractory to treatment or poorly tolerate glucocorticoids: teprotumumab, rituximab, cyclosporine, octreotide, intravenous immunoglobulin, and tarsorrhaphy ^[1]

Goals of therapy include treatment of hyperthyroidism, smoking cessation, eye protection, and decreasing inflammation.

Graves ophthalmopathy Thyroid-associated orbitopathy (Graves ophthalmopathy)

Orbital and preseptal cellulitis

Orbital cellulitis vs. preseptal cellulitis ^[2]
	Orbital cellulitis	Preseptal cellulitis
Definition	An infection of the orbital contents, such as the fat and extraocular muscles Posterior to the orbital septum	An infection of the eyelid and periorbital soft tissues without involvement of the orbital contents Anterior to the orbital septum
Etiology	Complication of upper respiratory tract infection, most commonly due to bacterial rhinosinusitis Other causes include: Acute dacryocystitis Infection of the teeth, middle ear, or face Infected mucocele Orbital trauma Ophthalmic surgery	Complication of upper respiratory tract infection, most commonly due to bacterial rhinosinusitis Other causes include: Acute dacryocystitis Local trauma to the surrounding tissues of the face and eyelids Insect or animal bites Ophthalmic surgery
Epidemiology	More common in children than adults	More common in children than adults Much more common than orbital cellulitis
Clinical features	Proptosis and ophthalmoplegia are the cardinal signs. Fever and malaise Reduced vision: diplopia, afferent pupil defect, dyschromatopsia Other clinical features include: Ocular pain Eyelid swelling and erythema Chemosis	Ocular pain Eyelid swelling and erythema Less commonly, chemosis and fever
Diagnostics	Primarily a clinical diagnosis Laboratory tests: leukocytosis; positive blood and tissue fluid cultures CT scan: confirm diagnosis and look for complications, e.g., orbital abscess or intracranial extension	Primarily a clinical diagnosis Laboratory tests: leukocytosis; blood cultures are generally unnecessary CT scan: only eyelid swelling apparent
Treatment	Empiric IV antibiotic treatment includes vancomycin PLUS ceftriaxone OR cefotaxime OR ampicillin-sulbactam OR piperacillin-tazobactam In patients with penicillin allergy: vancomycin PLUS ciprofloxacin OR levofloxacin If an intracranial extension is suspected: add metronidazole In case of abscess formation: surgical drainage	For mild nonsystemic disease without fever, oral antibiotics and close outpatient follow-up Empiric oral antibiotic treatment: Clindamycin or trimethoprim/sulfamethoxazole PLUS Amoxicillin-clavulanic acid OR Cefpodoxime OR Cefdinir
Complications	Subperiosteal or orbital abscess Blindness due to involvement of the optic nerve Brain abscess Other rare complications: cavernous sinus thrombosis and central retinal artery occlusion	Complications are rare

Reduced vision, diplopia, ophthalmoplegia, and proptosis are typical features of orbital cellulitis. They do not occur in preseptal cellulitis.

In patients with orbital cellulitis, the development of headache, ophthalmoplegia, facial hypesthesia in regions innervated by V1 and V2, and/or seizures should raise suspicion for cavernous sinus thrombosis.

Preseptal cellulitis versus orbital cellulitis Dacryoadenitis

Rhabdomyosarcoma

Definition: malignant mesenchymal tumor of primitive skeletal muscle cells (rhabdomyoblasts) that have failed to fully differentiate
Epidemiology
- Most common soft tissue sarcoma and malignant orbital tumor in children
- Primarily occurs in the first decade of life.
Clinical features
- Frequently in the orbital cavity, but can also be in the head and neck, urogenital region , or extremities
- Minimally painful, rapidly increasing, gross swelling
- Orbital rhabdomyosarcoma
  - Growing orbital mass that may be painful and have potential hemorrhage
  - Proptosis or dysconjugate gaze
Diagnostics
- Open or core needle biopsy for light microscopy to look for rhabdomyoblasts (confirm the presence of rhabdomyosarcoma)
- 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
Treatment
- 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, and less favorable for metastatic disease

Orbital rhabdomyosarcoma

Orbital compartment syndrome

Definition: sight-threatening ophthalmological condition due to an acute increase in intraorbital pressure
Etiology ^[3]^[4]
- Intraorbital hemorrhage (most common cause)
  - Trauma (e.g., orbito-facial fractures)
  - Facial surgery
- Fluid accumulation
  - Prolonged prone surgery (e.g., spinal surgery)
  - Periocular chemical burns
- Orbital cellulitis
- Orbital emphysema (rare)
Clinical features
- Acute onset of significantly reduced visual acuity
- Eye pain
- Periorbital swelling and proptosis
- Marked resistance to retropulsion (tight orbit)
- Firm or rock-hard globe
- Increased intraocular pressure
- Subconjunctival hemorrhage
- Ophthalmoplegia
- Diplopia
- Afferent pupillary defect and absence of light perception
- Dyschromatopsia
Diagnostics: The diagnosis is mainly based on history and physical examination.
- Fundoscopy: papilledema, venous congestion
- CT scan ^[4]
  - Only indicated in patients with mild symptoms if diagnosis is uncertain
  - Orbital imaging can be performed after decompression to locate a hematoma or guide further decompression
Treatment: emergency surgical decompression via lateral canthotomy
- Involves a horizontal incision from the lateral canthus to the rim of the orbit
- After exposure of the lateral canthal tendon, inferior cantholysis is performed, and the inferior crus of the lateral canthal ligament is cut.
Prognosis ^[4]
- Treatment within 2 hours of symptom onset: good chance of final visual acuity better than 20/40
- Treatment after 2 hours of symptom onset: usually poorer visual outcomes

Orbital compartment syndrome (OCS) is a sight-threatening ophthalmological emergency that can cause vision loss. If there is a high clinical suspicion of OCS, treatment should not be delayed for further diagnostic workup. Orbital imaging may be performed after decompression. ^[4]

References

Douglas RS et al. Teprotumumab for the Treatment of Active Thyroid Eye Disease. N Engl J Med. 2020; 382 (4): p.341-352.doi: 10.1056/nejmoa1910434 . | Open in Read by QxMD
Preseptal and Orbital Cellulitis. http://www.msdmanuals.com/professional/eye-disorders/orbital-diseases/preseptal-and-orbital-cellulitis. Updated: October 1, 2017. Accessed: December 7, 2017.
Murali S, Davis C, McCrea MJ, Plewa MC. Orbital compartment syndrome: Pearls and pitfalls for the emergency physician.. Journal of the American College of Emergency Physicians open. 2021; 2 (2): p.e12372.doi: 10.1002/emp2.12372 . | Open in Read by QxMD
McCallum E, Keren S, Lapira M, Norris JH. Orbital Compartment Syndrome: An Update With Review Of The Literature.. Clinical ophthalmology (Auckland, N.Z.). 2019; 13: p.2189-2194.doi: 10.2147/OPTH.S180058 . | Open in Read by QxMD

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