Lecture 1 - Inflammatory Disorders of Orbit/Adnexa

What inflammatory disorders of the orbit/adnexa are covered in this lecture?

Idiopathic Orbital Inflammatory Syndrome (IOIS), Orbital Myositis, Chronic Dacryoadenitis, and Orbital Blowout Fracture

What does the Greek root "idio-" mean?

Self; one's own

What does the Greek root "pathos" mean?

Suffering or disease

What does idiopathic mean?

A disease of its own (we don’t know what causes it)

What is Idiopathic Orbital Inflammatory Syndrome (IOIS)?

A benign, non-infectious inflammatory syndrome of the orbit that occurs without an identifiable local or systemic cause

What are other names for IOIS?

Orbital Inflammatory Pseudotumor, Idiopathic Orbital Inflammation, Nonspecific Orbital Inflammation, and Orbital Inflammatory Syndrome

What type of diagnosis is IOIS?

A diagnosis of exclusion

How does IOIS typically begin?

With a relatively rapid onset

What orbital tissues can be affected by IOIS?

Orbital fat, lacrimal gland, extraocular muscles, optic nerve, and posterior sclera

What is the etiology of IOIS?

Idiopathic

What factors have been associated with IOIS?

Higher BMI, younger age at first childbirth, and use of oral or IV bisphosphonates

What are examples of bisphosphonates associated with IOIS?

Fosamax, Boniva, and Actonel

What are bisphosphonates used for?

Prevention of bone loss in osteoporosis

Who is most commonly affected by IOIS?

Middle-aged adults, particularly women

What is the peak incidence age for IOIS?

Mid-40s

How common is IOIS in children?

Rare; approximately 6-17% of cases

Is IOIS usually unilateral or bilateral in adults?

Usually unilateral

If orbital inflammation is bilateral in an adult, what conditions should be considered?

Sarcoidosis, Wegener granulomatosis, metastases, and lymphoma

Is IOIS more commonly unilateral or bilateral in children?

More commonly bilateral

Are women or men affected more frequently by IOIS?

Women

How can the onset of IOIS vary?

It may be abrupt or insidious and may occur overnight or gradually over time

What symptoms are associated with IOIS?

Orbital swelling, pain, diplopia, and associated vision loss

What signs are associated with IOIS?

Conjunctival chemosis, conjunctival injection, increased IOP, periorbital edema, proptosis, ophthalmoplegia, and occasionally a palpable mass

What diagnostic measurement should be performed when proptosis is present in IOIS?

Exophthalmometry

What does ophthalmoplegia mean?

Paralysis or restriction of extraocular muscle movement

What imaging studies are used to diagnose IOIS?

Contrast-enhanced MRI or high-resolution CT

What is the primary treatment for IOIS?

Systemic anti-inflammatory therapy

What prednisone dosage is commonly used for IOIS?

80-100 mg oral prednisone daily for 1 week followed by a slow taper

What medication is often prescribed with high-dose steroids in IOIS?

Gastric prophylaxis

What IV steroid may be used in severe cases of IOIS?

IV Solu-Medrol

What dose of IV Solu-Medrol may be used in severe IOIS?

500 mg IV

How quickly do patients with IOIS typically respond to steroid treatment?

Within 48 hours

How are steroid-resistant cases of IOIS managed?

Steroid-sparing immunosuppressants, biologics, low-dose radiation therapy, biopsy, or surgery

What are biologics?

Drugs produced from living organisms

Which specialist commonly co-manages patients with IOIS?

A rheumatologist

Can IOIS recur?

Yes

Which patients are at greatest risk for IOIS recurrence?

Patients younger than 16 years, bilateral cases, and cases with optic disc edema

When should IOIS be strongly suspected?

In acute-onset painful unilateral ophthalmoplegia, especially in middle-aged women

What must be ruled out before diagnosing IOIS?

Orbital infection, mass lesions, and infiltrative disease

How is IOIS generally managed after diagnosis?

Aggressive steroid therapy followed by tapering and modification of risk factors to reduce recurrence

What does the Greek root "myo-" mean?

Muscle

What is orbital myositis?

Inflammation of the extraocular muscles

How does orbital myositis compare to other orbital inflammatory diseases?

It is generally a milder subtype with more clearly defined tissues and etiologies

Can orbital myositis be acute or chronic?

Yes

What are common causes of acute orbital myositis?

Lyme disease, herpes zoster virus, and Streptococcus infection

What organism causes Lyme disease associated with orbital myositis?

Borrelia burgdorferi

What are common causes of chronic orbital myositis?

Giant cell myocarditis, Crohn disease, systemic lupus erythematosus, and linear scleroderma

Are chronic cases of orbital myositis generally infectious or autoimmune?

Autoimmune

How does orbital myositis present clinically?

Essentially the same as IOIS

How is orbital myositis differentiated from IOIS?

By orbital imaging demonstrating specific extraocular muscle involvement

What structures are specifically affected in orbital myositis?

The extraocular muscles

What imaging studies are used to diagnose orbital myositis?

Contrast-enhanced MRI or high-resolution CT

What is the first management step in orbital myositis?

Rule out and address infectious causes

In which patients should infectious orbital myositis be strongly suspected?

Younger patients and those with concurrent sinusitis

Why is blood work ordered in suspected orbital myositis?

To determine whether infection is present

How are infectious cases of orbital myositis treated?

Appropriate antibiotic or antiviral therapy

How are chronic or recalcitrant cases of orbital myositis treated?

Anti-inflammatory therapy similar to IOIS

What does the Greek root "dacryo-" mean?

Tears

What does the Greek root "aden-" mean?

Gland

What is chronic dacryoadenitis?

Inflammation of the lacrimal gland

How do chronic cases of dacryoadenitis typically present?

With slow onset, minimal symptoms, and bilateral involvement

What is the usual cause of chronic dacryoadenitis?

Inflammation secondary to systemic disease

Must the underlying systemic disease already be diagnosed at presentation?

No

What is the most common cause of chronic dacryoadenitis?

Sarcoidosis

What other diseases can cause chronic dacryoadenitis?

Thyroid disease, lymphoma, adenoid cystic carcinoma, Sjögren syndrome, Wegener granulomatosis, and tuberculosis

Does chronic dacryoadenitis have a specific age, gender, or racial predilection?

No

What demographic is commonly associated with sarcoidosis-related dacryoadenitis?

African American females ages 20-50 years

Is chronic dacryoadenitis usually unilateral or bilateral?

Usually bilateral

What symptoms are associated with chronic dacryoadenitis?

Cosmetic concern about swollen eyes, puffy eyelids, little or no pain, and dry eye symptoms from reduced tear production

What signs are associated with chronic dacryoadenitis?

S-shaped swelling of the outer third of the eyelid, enlarged mobile lacrimal gland, mild ptosis, and minimal ocular signs

What is the classic eyelid finding in chronic dacryoadenitis?

S-shaped swelling of the outer one-third of the eyelid

How does the lacrimal gland appear in chronic dacryoadenitis?

Enlarged, palpable, and mobile on lid retraction

Why can ptosis occur in chronic dacryoadenitis?

Enlargement of the lacrimal gland causes lid drooping

What imaging studies are used for chronic dacryoadenitis?

MRI or CT

What laboratory tests may be ordered for chronic dacryoadenitis?

CBC with differential and serologic testing based on history and symptoms

What is the initial treatment of chronic dacryoadenitis?

Systemic corticosteroids

What prednisone dosage is commonly used in chronic dacryoadenitis?

80-100 mg daily for 1-2 weeks followed by a slow taper

What is the most important long-term management goal in chronic dacryoadenitis?

Identify and treat the underlying cause

When should biopsy be performed in chronic dacryoadenitis?

In recalcitrant cases or when malignancy is suspected

What type of biopsy may be performed in chronic dacryoadenitis?

Transcutaneous transeptal biopsy

What is an orbital blowout fracture?

A fracture of the orbital wall or floor caused by blunt facial trauma

How many bones form the orbit?

Seven

What bones form the orbit?

Frontal, ethmoid, lacrimal, palatine, sphenoid, maxillary, and zygomatic bones

Which orbital bones contain sinuses?

Frontal, ethmoid, sphenoid, and maxillary bones

Why are sinus-containing orbital bones clinically important?

They are more susceptible to inflammation and injury

Which orbital bones are most commonly fractured in blunt trauma?

Ethmoid bone (orbital wall) and maxillary bone (orbital floor)

What causes an orbital blowout fracture?

Blunt trauma to the eye or orbit

What pressure change occurs in an orbital blowout fracture?

Rapid increase in intraorbital hydraulic pressure

What force vectors are created during an orbital blowout fracture?

Downward and medial vectors

Where do most orbital blowout fractures occur?

Posteromedial orbit where the bones are thinnest

What are common causes of orbital blowout fractures?

Sports injuries, fist injuries, and motor vehicle accidents

Which structure is most frequently involved in pure blowout fractures?

The orbital floor

Why is involvement of the orbital floor noteworthy?

The medial wall is thinner, yet the floor is fractured more often

What happens to orbital contents during a blowout fracture?

They are displaced downward

Which extraocular muscle is commonly entrapped in an orbital floor fracture?

The inferior rectus muscle

What motility deficit results from inferior rectus entrapment?

Restriction of upgaze

What is orbital emphysema?

Air trapped within the orbit

What causes orbital emphysema in a blowout fracture?

Air enters the orbit through the fracture

How does air appear on MRI imaging?

Black

What imaging sign is associated with orbital emphysema?

The black eyebrow sign