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Orbital disorders

Last updated: October 28, 2020

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 or 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)
- Patient may be hyperthyroid, euthyroid, or hypothyroid.
Diagnosis
- 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
- 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
Differential diagnosis: pseudoexophthalmos
Treatment
- Principles
  - 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
  - Treatment of hyperthyroidism with thioamides and/or surgery without causing hypothyroidism
- 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

Graves ophthalmopathy Thyroid-associated orbitopathy (Graves ophthalmopathy)

References:^[1]^[2]^[3]^[4]

Orbital and preseptal cellulitis

	Orbital cellulitis	Preseptal cellulitis
Definition	An infection of the orbital contents, such as the fat and extraocular muscles Posterior to the orbital septum The eye globe is not affected.	An infection of the eyelid and periorbital soft tissues without involvement of the orbital contents Anterior to the orbital septum The eye globe is not affected.
Etiology	Complication of upper respiratory tract infection, most commonly due to bacterial rhinosinusitis Other causes include: Acute dacryocystitis Infection of the teeth, middle ear (see acute otitis media), or face Infected mucocele Orbital trauma (see emergency ophthalmology) 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 (forward protrusion of eyeball) 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 Necrotic areas in the nares and/or oral cavity in immunocompromised patients with maximally and/or ethmoid sinusitis should raise suspicion for mucormycosis.	Ocular pain Eyelid swelling and erythema Less commonly, chemosis and fever
Diagnosis	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 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 Surgical drainage is indicated in abscess formation For treatment of mucormycosis, see mucormycosis	For mild non-systemic disease without fever, oral antibiotics and close outpatient follow-up Oral amoxicillin-clavulanic acid If methicillin-resistant S. aureus (MRSA) is suspected, clindamycin, trimethoprim/sulfamethoxazole, or doxycycline should be added.
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	Although preseptal cellulitis can cause severe complications, these are rare, which is why the condition is rarely life-threatening.

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.

Orbital cellulitis

References:^[5]^[6]^[7]^[8]^[9]^[10]

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
Diagnosis
- Open or core needle biopsy for light microscopy to look for rhabdomyoblasts (confirm the presence of rhabdomyosarcoma)
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

References:^[11]^[12]^[13]^[14]^[15]

References

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Davies TF. Treatment of Graves' Orbitopathy (Ophthalmopathy). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/treatment-of-graves-orbitopathy-ophthalmopathy.Last updated: October 14, 2016. Accessed: February 18, 2017.
Ing E. Thyroid-Associated Orbitopathy. In: Roy H, Thyroid-Associated Orbitopathy. New York, NY: WebMD. http://emedicine.medscape.com/article/1218444. Updated: September 30, 2016. Accessed: February 18, 2017.
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Kwitko GM. Preseptal Cellulitis. In: Ing E, Preseptal Cellulitis. New York, NY: WebMD. https://emedicine.medscape.com/article/1218009. Updated: December 1, 2017. Accessed: December 7, 2017.
Gappy C, Archer SM, Barza M. Orbital cellulitis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/orbital-cellulitis.Last updated: January 4, 2016. Accessed: February 18, 2017.
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Cox GM, Perfect JR. Fungal Rhinosinusitis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/fungal-rhinosinusitis.Last updated: August 13, 2017. Accessed: December 7, 2017.
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.
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Okcu MF, Hicks J, Horowitz M. Rhabdomyosarcoma in childhood and adolescence: Clinical presentation, diagnostic evaluation, and staging. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/rhabdomyosarcoma-in-childhood-and-adolescence-clinical-presentation-diagnostic-evaluation-and-staging.Last updated: July 9, 2015. Accessed: February 18, 2017.
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