Disorders of the Eyelids, Lacrimal System, Orbit, and Anophthalmic Socket
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[edit] Disorders of the Eyelids, Lacrimal System, Orbit, and Anophthalmic Socket
Murray D. Christianson
[edit] DISORDERS OF THE EYELIDS
[edit] Anatomy and Physiology
The eyelids protect the eyes and, with each blink, smooth an optically important film of tears over the cornea.
Eyelid skin is the thinnest in the body. The subcutaneous tissue is thin and areolar, swelling easily with injury or infection. The orbicularis oculi muscle is sphincterlike, closing the lid aperture when it contracts. With a facial palsy, the lids do not protect the cornea.
Near the lid margins, just posterior to the muscle, are the tarsi, tough fibrous tissue plates that extend horizontally from the lacrimal puncta to the lateral canthal angle. They are attached to bone by the medial and lateral canthal tendons, continuations of the pretarsal and preseptal orbicularis. Full-thickness lid tears are usually through the medial canthal tendons that are weakened by the lacrimal canaliculi passing through them.
Each tarsus has about 25 large, vertical meibomian glands that secrete sebum onto the lid margin just anterior to the posterior lid margin. Modified sebaceous glands (of Zeis) and modified sweat glands (of Moll) empty into or beside the eyelash follicles.
The eyelashes (cilia), 100 to 150 in the upper lid and half that number in the lower, form two or three rows at the anterior lid margins. They curve anteriorly away from the cornea. Each has an average life span of about 5 months; its replacement is fully grown in 10 weeks.
The conjunctiva has three parts: bulbar, covering the globe; tarsal, lining the eyelids; and forniceal, in the cul-de-sacs inferiorly and superiorly. The bulbar portion is exposed to sunlight and may develop degenerative and neoplastic conditions. When the tarsal conjunctiva scars and shrinks, it rotates the margin of the eyelid posteriorly causing cicatricial entropion. The forniceal conjunctiva may prolapse between the eyelids with orbital swelling.
The eyelids are richly vascularized. Lymphatic drainage is to the preauricular and submandibular nodes.
Mueller's muscle and the levator muscle lift the upper lid. Blepharoptosis occurs when Mueller's muscle is denervated, or when the levator muscle is denervated, diseased, damaged by age or injury, or congenitally fibrotic.
The capsulopalpebral fascia connects the inferior rectus muscle capsule to the inferior border of the tarsal plate. In Graves' disease, pull on the capsulopalpebral fascia by the diseased inferior rectus muscle often causes lower eyelid retraction. The orbital septum extends from the periosteum at the orbital rim into the upper and lower eyelids, separating the “eyelid” from the orbit, holding back orbital fat and acting as a barrier to infection.
Orbital fat is packed around the globe, extraocular muscles, nerves, and blood vessels, cushioning and supporting them. The eyebrows normally sit at or above the bony superior orbital rim.
[edit] Clinical Evaluation of Eyelid Disease
Clinical evaluation of eyelid disease should begin with a personal, occupational, and social profile; past medical history; list of medications and allergies; and family history. Obtain a careful chronologic history of the disorder, inquiring about inciting factors, associated symptoms and signs, and response to treatments. Examine the face from the top down, noting: frontalis use in attempting to raise the lids; position and asymmetry of the brows; upper lid skin irritation, redundancy, or tumors; position of the upper lid skin crease; lash orientation; upper lid level and contour; lid excursion measurements from full down-gaze to full up-gaze (normally about 15 millimeters); vertical palpebral aperture measurements; lower lid position and contour; herniated orbital fat pockets in both upper and lower lids; lacrimal punctal position; lower lid skin laxity, inflammation, or tumor; and cheek abnormalities. Sketch the pathology. Examine, with magnification if available, the lid margin, and palpebral and bulbar conjunctiva. When indicated, palpate the preauricular and submandibular lymph nodes.
[edit] Eyelid Malpositions
See Table 174-1.
Table 174-1 Eyelid Malpositions
| Malposition | Symptoms | Signs | Treatment |
|---|---|---|---|
| Trichiasis (normal lid position, but lashes directed posteriorly) | Irritation; tearing; red eye | Eyelid: no entropion; scarring; inflammation | For inflammation: lid scrubs; hot compresses; antibiotic drops |
| Eyelash: against the eye | For lashes: epilation (temporary); electroepilation (permanent); excision of root(s); cryodestruction | ||
| Eye: red; corneal ulcer | |||
| Entropion | Off and on? | Eyelid: lid flipped in, lashes and skin against the eye | For lid: tape (temporary); surgical repair (permanent) |
| Congenital; acquired (cicatricial, mechanical, _senile) | Irritation; tearing; red eye | ||
| Eye: red; corneal ulcer | |||
| Ectropion | Irritation; tearing; red eye; red lid | Eyelid: eyelid flipped out; irritated, exposed conjunctiva | For eye: lubricate |
| Congenital; acquired (cicatricial, paralytic, senile) | For lid: surgical repair | ||
| Eye: dry, irritated | |||
| Blepharoptosis (neurogenic neuromuscular junction; myogenic; aponeurotic; mechanical) | Upper lid droop | Frontalis contraction; brow elevation; excess skin; high lid crease; droopy lid | For underlying disease |
| For lid (surgically): restore anatomy; suspend lid | |||
| Lid retraction (Graves' disease?) | Stare; eye bigger; irritation; tearing | Lid: upper pulled up; lower pulled down | For underlying disease |
| For lid: surgically lower upper lid and/or raise lower lid | |||
| Eye: red, exposed | |||
| Blepharospasm | Eyes close; can't see | Lid spasms; facial twitches | Botulinum toxin Surgery |
[edit] Eyelid Defects and Reconstruction
[edit] Cosmetic Eyelid Surgery.
With age, forehead and eyelid skin and subcutaneous supporting structures sag. The brows may fall below the superior orbital rims, and the upper lid skin may hang down, obstructing vision, or push the lashes into the cornea. The upper lid crease may collapse, and orbital fat may herniate through orbital septal defects, giving lid bags. Lower lids may sag. Dramatic improvement in function and appearance may result from lifting the brows, removal of excess eyelid skin and prolapsing fat, re-creation of the upper lid crease, and eyelid tightening.
[edit] Lower and Upper Eyelid Defects.
Skin defects may be repaired with flaps or free, full-thickness skin grafts from the upper eyelid or behind the ear. Full-thickness lid repairs replace skin, tarsus, and conjunctiva with local flaps, and free grafts of skin, tarsus, cartilage, and mucous membrane are options.
[edit] Canthal Tendons: Medial and Lateral.
After tumor resection, the canthal tendons must be reanchored to bone.
[edit] Eyelid Injuries
After injury, assess and treat more pressing systemic problems, then protect the eye and vision. Marked eyelid swelling and bruising are the rule. They usually resolve over 1 to 2 weeks. Unless exposure threatens the cornea, eyelid repair can usually wait 24 to 36 hours (Box 174-1). The necessary expertise, instruments, lighting, magnification, and anesthetic must be available.
| Box 174-1 - Eyelid Lacerations |
May Repair Without Referral
|
Gently clean thermal eyelid burns and apply light, moist dressings. Aggressively, immediately, and copiously irrigate chemical burns and refer as emergencies.
[edit] Infections, Infestations, and Inflammations
Eyelid lumps can be infectious, inflammatory, cystic, xanthomatous, or neoplastic. Lid infections can be viral, bacterial, or fungal; lid infestations can be from lice or mites (Table 174-2). Lid inflammations include marginal blepharitis and associated disorders, dermatitis, and foreign body granulomas (Table 174-3).
Table 174-2 Eyelid Infections and Infestations
| Lid infection | Symptoms | Signs | Treatment |
|---|---|---|---|
| Viral | |||
| Molluscum contagiosum | Mildly contagious; bumps on lids; more bumps coming; itchy eyes | One or many dome-shaped, skin-colored, umbilicated nodules, 1-3 mm diam; cheesy, sebum-like material expressed; may give follicular conjunctivitis | Incise |
| Excise | |||
| Curet | |||
| Swab with alcohol | |||
| Verruca | Warty lid bumps; often from inoculation from warts on hands | Verruca vulgaris: round, warty | Excise |
| Verruca plana: flat | |||
| Verruca digitata: fingerlike, horny, capped filaments, grouped on narrow base | |||
| Verruca filiformis: threadlike, normal skin covering | |||
| Herpex simplex | Spread by kissing, two infection peaks—6 mo-4 yr, 16-25 yr; blisters on lids; “cold sore” on lid | Primary: subclinical or unilateral crop of pinhead-sized vesicles on swollen, slightly red base, usually lower lid; may have fever; resolve in 7 days, leave no scars; may give keratitis or conjunctivitis; in atopic patients, may give severe systemic infection, Kaposi's varicelliform eruption | Refer |
| Acyclovir 300 mg po five times daily | |||
| Vidarabine 3% ointment five times daily | |||
| Recurrent herpes (cold sore) may affect the eyelids; benign unless eye infected | |||
| Herpes zoster | Headache, sudden fever, malaise; in 3-4 days swelling and blistering on lid and forehead; usually adults and aged, sometimes the young; at increased risk are immunosuppressed patients; postherpetic neuralgia may be helped with antidepressants | Vesicles in V1 or V2 distribution, respect midline, one third involve lids and eye; vesicles filled with clear, then turbid fluid, quickly burst, giving eschar in nerve distribution, inviting secondary infection, healing in a week or two; may be marked eye inflammation; pain often severe, may persist for years (postherpetic) in elderly; skin often left mildly numb, yet giving pain on the slightest provocation (anesthesia dolorosa) | Refer |
| If early diagnosis: oral antiviral for 7 days (acyclovir, famciclovir, valacyclovir) | |||
| Bacterial | |||
| Hordeolum (much less common than chalazia) | Stye: pain and lid margin swelling and redness | Rapidly forms abscess, may point and drain, often secondary to staphylococcal blepharitis | Refer |
| Hot compresses | |||
| Oral penicillinase-resistant antibiotics | |||
| External hordeolum, furuncle of lash follicles and adjacent glands | |||
| Surgical drainage if necessary | |||
| Internal hordeola involve meibomian glands | |||
| Fungal | |||
| Tinea corporis | Often spreads from face; red spot | Red spot with centrifugal extension and central healing; ring shape | Topical antifungal twice daily, continue for 10 days after lesions disappear |
| Infestation | |||
| Crab lice (Phthirus pubis) | Often in children; chronic, itchy blepharitis | Blepharitis | Refer |
| Dramatic: multiple nits (eggs) glued to lash bases, several lice gripping lash bases or crawling about the shafts | Wash all clothes and bed linen in hot, soapy water | ||
| Use an anti-lice shampoo on all affected individuals | |||
| Subtle; only one or two nits | |||
| Pick off the nits and lice at the slit lamp | |||
| Demodex mites | Red, itchy lids; infest the lid margin pilosebaceous units | Chronic blepharitis and meibomianitis | Lid scrubs |
| Antibiotic ointment | |||
Table 174-3 Eyelid Inflammations
| Inflammation | History | Signs | Treatment |
|---|---|---|---|
| Staphylococcal marginal blepharitis | Often begins in childhood; may last a lifetime; women>men; irritated, red, crusting eyelids; recurrent styes, often associated with seborrhea and acne rosacea | Dilated skin vessels; brittle, yellow crusts at lash roots leaving a bleeding ulcer when removed; recurrent hordeolum | Dandruff shampoo if seborrheic |
| Wash with warm, soapy water twice a day | |||
| “Eyelid scrubs” with face cloth or cotton-tipped applicator twice a day; remove all crusts | |||
| If chronic may give entropion or ectropion, lid thickening or notching; lash loss, misdirection or whitening; papillary conjunctivitis, keratitis | |||
| Flare-ups or resistant cases: scrubs, then massage antibiotic ointment into lid margin | |||
| If severe: antibiotic/corticosteroid ointment; oral tetracycline | |||
| Seborrheic marginal blepharitis | Associated seborrheic dermatitis of the scalp, nasolabial folds, brow, retroauricular area, and presternum | Reddened lid margins, soft, greasy scales, not clustered at the lash roots, and not giving bleeding microulcers when removed, foamy tear meniscus, meibomian orifices swollen or plugged by oil globules, chalazia, secondary chronic papillary conjunctivitis, interpalpebral corneal epithelial punctate erosions | As above |
| Chalazion | Stye; one or many; variable growth rate; variable pain, less than hordeolum; sebaceous gland inflammation from duct obstruction, spontaneous or secondary lid margin disease; associated with seborrheic dermatitis or acne rosacea | Usually painless, rounded, slowly enlarging, subcutaneous lid mass that may wax and wane in size; may rupture posteriorly, giving a polypoid, conjunctival mass of granulation tissue on the conjunctiva, or anteriorly, giving a subcutaneous mass | For acne rosacea or seborrheic dermatitis |
| For lids: hot, moist compresses for 15 min, four times a day | |||
| For resistant or chronic chalazia: incision and curettage, with subconjunctival triamcinolone injection | |||
| Marginal chalazia resist conservative treatment | |||
| Dermatitis | Common | Red, irritated, thickened, scaling lid skin | Remove irritant |
| Acute: red, itches, burns, swollen, flakes; blisters if severe | Cool compresses | ||
| Hydrocortisone 0.5% in Aquaphor four times a day | |||
| Chronic: itchy, thick, scaling skin | |||
| Dermatologic referral if widespread | |||
| Atopic dermatitis | Personal or family history of asthma, hay fever, atopic dermatitis | As above | As above |
| Contact dermatitis | Cosmetics, aerosols, nail polish, soap, eye drops—especially neomycin | As above | As above |
| Foreign body granuloma | Lid lump, grows slowly; old injury | Quiet lid lump | Excise |
Marginal blepharitis is a common chronic condition associated with staphylococcal infection, seborrhea, acne rosacea, and dry eyes. It eventually gives secondary lid margin scarring, with misdirection of lashes causing corneal damage. Patients complain of chronic burning, itching, and redness of their lids, which are often worse in the morning. Symptoms are often out of proportion to signs. Although often a mixed staphylococcal/seborrheic condition, each type has its hallmarks.
[edit] Eyelid Neoplasms
[edit] Epithelial Neoplasms.
See Table 174-4.
Table 174-4 Primary Epithelial Neoplasms of the Eyelids
| Neoplasm | History | Signs | Treatment |
|---|---|---|---|
| Benign | |||
| Squamous cell papilloma (most common benign eyelid lesion) | Chronic lid bump | Sessile or pedunculated, skin colored, often multiple, often involve lid margin | Observe or excise |
| Seborrheic keratosis (dermatosis papulosa nigra, in Blacks) | Common eyelid and facial skin lesions in the middle-aged or older | Caucasians: tan to light brown, sharply circumscribed, wartlike, soft, friable, “stuck on” the skin | Curettage |
| Cryotherapy | |||
| Excise if pedunculated | |||
| Blacks: darkly pigmented lesions | |||
| Keratocanthoma | Uncommon; in aged; grows rapidly | Begins as a reddish papule, grows rapidly over weeks or a few months into a firm nodule with a keratin-filled crater | Excisional biopsy |
| Precancerous | |||
| Actinic keratosis (solar, senile keratosis; untreated, about 12% develop squamous cell carcinoma) | Most common precancerous skin lesion; older Caucasians; sun-exposed skin | Scaly, keratotic, flat or warty, brownish, circumscribed | Consider biopsy |
| Exfoliative agents | |||
| Cutaneous horns (also from squamous papillomata, seborrheic keratosis, inverted follicular keratosis, and verruca vulgaris) | Curettage | ||
| Excision | |||
| Cancers | |||
| Basal cell carcinoma (Jacob's ulcer; most common eyelid malignancy) | Fair-skinned; commonly lower lids and medical canthi; often other facial lesions; almost never metastasizes; slowly grows, invading and destroying (“rodent ulcer”) | Varies, often misleading | Look for second lesions |
| Common types: | Biopsy | ||
| 1. Noduloulcerative: raised, firm, pearly nodule with telangiectatic vessels, central ulcer, and bleeding with minor trauma | Excise with frozen section control | ||
| Mohs' | |||
| Radiation | |||
| 2. Pigmented: noduloulcerative with pigment | |||
| 3. Sclerosing or morpheaform: pale, firm plaque with ill-defined borders | |||
| 4. Superficial: erythematous scaling patches with a fine pearly border | |||
| Squamous cell carcinoma (second most common lid malignancy; in the upper eyelid and lateral canthal areas, more common than basal cell; arises de novo or from actinic keratosis) | Elderly, fair-skinned; lower eyelid margin | Elevated, firm plaque or nodule, often ulcerates, irregular borders; grows faster than basal cell, invades and destroys locally, may metastasize to regional lymph nodes | Biopsy and wide excision |
| Radiotherapy | |||
[edit] Melanocytic Neoplasms.
Nevi (moles) vary in size and pigmentation, and can be flat, elevated, papillomatous, dome-shaped, and pedunculated. Appearing and growing rapidly in childhood, they grow more slowly during adolescence, and become stable in adulthood. They should be excised if troublesome.
Freckles (ephelides) are little, circumscribed, brown spots, seen usually in adolescents following sun exposure. They may be transient or permanent and need no treatment.
Lentigo senilis occurs in 90% of elderly Caucasians and resembles seborrheic keratosis, giving multiple, dark-brown macules with irregular outlines. It should be observed.
Malignant melanoma of the eyelid skin is rare.
[edit] Sebaceous Neoplasms.
Sebaceous carcinoma arising from the meibomian glands or the glands of Zeis or the caruncle is the third most common eyelid malignancy, occurring usually in the elderly or in younger patients with irradiated lids. Presentation varies, with the sebaceous carcinoma appearing as: a small, firm nodule like a chalazion; an atypical or recurrent chalazion; a diffuse, plaquelike tarsal thickening; a fungating or papillomatous growth; or a persistent unilateral conjunctivitis, blepharitis, or meibomitis (masquerade syndrome). Spread is local, lymphatic, and hematogenous. Prognosis is good unless diagnosis is late. The physician should biopsy and excise widely.
[edit] Vascular Eyelid Lesions
See Table 174-5.
Table 174-5 Vascular Lesions of the Eyelids
| Lesion | History | Signs | Treatment |
|---|---|---|---|
| Capillary hemangioma | Develops just after birth, grows rapidly for about 1 year, then stabilizes, then involutes until about age 5 years | Raised, dimpled, intensely red “strawberry mark”; bulges with Valsalva's maneuver; blanches with pressure | Observe and await involution if possible |
| Treat if amblyopia threatens | |||
| Local injections of steroid | |||
| Excision | |||
| Nevus Flammeus or port-wine stain (may be part of Sturge-Weber syndrome) | Always present at birth; does not enlarge; often becomes darker with time | Flat, port-wine colored mark of varying size; no bulging with Valsalva's maneuver; no blanching | Cosmetics |
| Photoablation | |||
| Check for ocular and intracranial involvement if Sturge-Weber syndrome | |||
| Cavernous hemangioma | Usually develops in the second to fourth decade; grows slowly; does not involute | Raised, red or purplish lesion | Excise |
| Pyogenic granuloma (most common acquired eyelid lesion) | Follows minor trauma or surgery; grows rapidly; bleeds easily | Scarlet, brown, or blue-black nodule; friable surface | Excise |
| Varices | Grow slowly; often with other facial venous anomalies | Dark blue, deeper, soft lesions | Debulk as necessary |
| Often recur | |||
| Lymphangiomas | Grow slowly; no spontaneous regression; intralesional bleeding may cause enlargement | Yellowish-tan or reddish swelling; deep component | Observe |
| Excise | |||
| Often recur | |||
| Kaposi's sarcoma | In patients with AIDS | Reddish vascular nodule of conjunctiva or lid | Treat AIDS |
| Biopsy and excise as necessary |
[edit] Neurogenic Neoplasms
Neurogenic neoplasms occur with neurofibromatosis, type 1. Plexiform neurofibromas are unencapsulated, diffuse, intertwining bundles of Schwann cells, axons, and endoneural fibroblasts in a perineural sheath that usually grow along sensory nerves. The most common and complex orbital peripheral nerve tumors, they give elephantiasis neuromatosa, with hypertrophic skin, lid, and face; proptosis and disfigurement; and on palpation, a “bag of worms.” The patient should be referred to an ophthalmologist.
Molluscum fibrosum usually occur in great numbers over the body and involve the lids, they occasionally become pedunculated and hang down over the cheek. They should be excised if indicated.
[edit] Miscellaneous Eyelid Lesions
[edit] Xanthomatous Lesions.
Xanthelasma is the most common cutaneous xanthoma, and the only common lid xanthoma. It occurs in the middle-aged and elderly patients, two thirds of whom are normolipemic. Usually bilateral and at the inner canthi, they are flat or slightly raised, yellowish-tan, soft plaques. The patient should be referred to an ophthalmologist or the xanthelasma should be excised with care.
[edit] Cystic Lesions of the Eyelids.
See Table 174-6.
Table 174-6 Cystic Lesions of the Eyelids✢
| Lesion | History | Signs | Treatment |
|---|---|---|---|
| Hidrocystomas (retention cysts either of the apocrine [Moll's gland] or eccrine sweat glands) | Common; chronic, painless lid margin cyst | Occasionally multiple, but usually solitary; lid margin translucent, cystic nodule; often bluish, transilluminates | Excise |
| Pilosebaceous cysts, milia | Asymptomatic, multiple white lid bumps | Rounded, white; sharply circumscribed, pinhead-sized, pearly nodules | Excision, diathermy, or electrolysis |
| Pilosebaceous cysts, sebaceous or pilar cysts | Subcutaneous lid bump; stable or grow slowly | Globular, subcutaneous masses, attached to skin, often of brow; plugged pore at the summit often | Excise |
| Epidermal inclusion cysts | After trauma or surgery; grow slowly | Firm, globular, painless, mobile, dermal or subcutaneous masses; skin intact | Excise |
✢Other cystic lesions include dermoid cysts, pilomatrixoma, cystic basal cell carcinoma, and dacryops (cysts from dilated ducts of accessory lacrimal glands from scarring or chronic inflammation).
[edit] Involvement of the Eyelids in Systemic Diseases
Eyelid lesions occur in primary systemic amyloidosis, sarcoidosis, leprosy, and mycosis fungoides.
[edit] DISORDERS OF THE LACRIMAL SYSTEM
[edit] Anatomy and Physiology
The lacrimal glands, both primary (orbital and palpebral lobes in the superotemporal orbit) and secondary (Krause's and Wolfring's), secrete tears except during sleep. These enter the conjunctival sac superotemporally through the lacrimal ductules. The eyelids close from medial to lateral and milk the tears toward the medial canthus. The lacrimal puncta are normally turned posteriorly into the lacrimal lakes. Capillary attraction draws tears from the lid margin menisci into the puncta, and blinking pumps them the 10 millimeters through the canaliculi to the lacrimal sac. The lacrimal sac empties under the inferior turbinate into the nose through the nasolacrimal duct. The duct's inferior end is often not yet open at birth, giving tearing and chronic purulence that usually clears when the duct opens during the first few months of life.
[edit] Clinical Evaluation of Lacrimal Diseases
Begin with a careful chronologic history of the complaint, usually moistness, or tears running down the cheek (true epiphora).
If there is no true epiphora, suspect dry eyes, with secondary irritation and reflex tearing. Ask about variations in the symptoms from day to day with aggravation by winds, cigarette smoke, automobile defrost fans, and low relative humidity from central heating. Obtain a complete medication history looking for drying medications. Inquire about systemic diseases associated with dry eyes (rheumatoid arthritis, Sjögren's syndrome, sarcoidosis). Ask about dry mouth and dry skin. If there is true epiphora, suspect nasolacrimal duct obstruction, which is more common in women. Ask about previous facial injuries, sinus or nasal surgery, or previous bouts of dacryocystitis.
Examine the eyes looking for an irritant causing hypersecretion, debris in the tear film, normal lid tautness, punctal position, and patency. Press on the lacrimal sac and look for reflux from the puncta.
[edit] Diagnosis and Treatment
[edit] Dry Eyes.
The diagnosis should be explained to the patient in some detail. Treat any treatable systemic diseases. Change any contributing systemic medication. Recommend avoiding winds, fans, low humidity, smoking, and all air pollutants. Suggest artificial tears during the day and lubricant ointment at night. Consider temporary lacrimal punctal occlusion with punctal plugs, or permanent occlusion with cautery.
[edit] Epiphora or Tearing.
Identify and treat the cause by surgically correcting lid laxity, punctal atresia, punctal ectropion, canalicular disease or obstruction, lacrimal sac, and nasolacrimal duct disease. A dacryocystorhinostomy (DCR) has a 97% success rate in primary cases, can be done as an outpatient case under local anesthetic, and leaves little scarring.
[edit] Infections.
Dacryoadenitis may be acute (viral or bacterial) or chronic (bacterial) (Table 174-7).
Table 174-7 Infections of the Lacrimal System
| Infection | History | Signs | Treatment |
|---|---|---|---|
| Lacrimal gland | |||
| Acute viral dacryoadenitis (dacryoadenitis from mumps, infectious mononucleosis, herpes zoster, measles, influenza, and dengue fever) | Fullness or pain in the upper outer orbit | Abscesslike, firm, tender lateral lid swelling, S-shaped upper lid margin; mechanical ptosis; sometimes inferonasal proptosis; preauricular lymphadenopathy; lid eversion shows gland swelling with localized chemosis | Observe |
| Resolves in 1 week | |||
| Acute bacterial dacryoadenitis (from staphylococci, streptococci, pneumococci, gonococci) | Severe pain and fullness in the upper outer orbit | Abscess in upper outer orbit; may suppurate, draining through the conjunctiva (palpebral lobe) or skin (orbital lobe) | Refer |
| Systemic antibiotic | |||
| Drainage | |||
| Support | |||
| Chronic bacterial dacryoadenitis (from trachoma, tuberculosis, leprosy, syphilis, actinomycosis) | Slow-growing orbital mass, up and out | Superotemporal orbital mass; diplopia on looking toward it, inferonasal proptosis, dry eye | Refer |
| Specific therapy | |||
| Canaliculus | |||
| Canaliculitis (from actinomyces) | Chronic unilateral tearing and irritation, resistant to topical antibiotics | Redness and swelling over canaliculus; pouting punctum | Canaliculotomy, stone removal, painting with iodine |
| Lacrimal sac | |||
| Dacryocystitis (from staphylococcus, streptococcus) | Middle-aged (females:males 3:1); acute or chronic swelling below the medial canthal tendon; irritation and tearing | From nasolacrimal duct obstruction; lacrimal sac swelling; tearing | Systemic antibiotic (oral/IV) |
| Hot compresses | |||
| Abscess drainage | |||
| Dacryocystorhinostomy (DCR) | |||
[edit] Injuries.
Facial lacerations may cut the canaliculi. Medial canthal tendon avulsions, medial to the punctum, may tear the canaliculus, displacing the punctum laterally. Repair should be meticulous to avoid permanent tearing. The sac and nasolacrimal duct are often disrupted in midfacial fractures and by sinus surgery. The normal anatomy should be restored.
[edit] Tumors.
Lacrimal gland tumors are discussed in the orbital section below. Lacrimal sac tumors are rare.
[edit] DISORDERS OF THE ORBIT
Many orbital diseases are uncommon, and patients with them are best referred to an ophthalmologist with a special interest in these conditions.
[edit] Anatomy and Pathophysiology
The orbit is the bony socket containing the globe; extraocular muscles; sensory, motor, and autonomic nerves; blood vessels; and lacrimal gland. They are all packed in orbital fat. The bony orbit is pear-shaped, with the “stem” entering the optic canal, and is wider just behind its anterior opening than at the opening itself. Its adult volume is about 30 cc, although it is much shallower in children. Space-occupying lesions may push the globe anteriorly, up or down, or nasally or temporally, or may invade orbital structures, restricting globe movement.
[edit] Epidemiology and Disease Patterns
[edit] Age Distribution.
Congenital malformations are most common in the first decade; orbital neoplasia in the first, sixth, and seventh decades; and thyroid-associated ophthalmopathy (TAO) in the fourth, fifth, and sixth decades. Structural and degenerative disease prevalence gradually increases with age, whereas prevalence of inflammatory and vascular diseases remains stable.
[edit] Clinical Presentation
[edit] History.
Onset may be hyperacute (trauma and hemorrhage), acute and subacute (inflammation, a few neoplasms), or chronic (most neoplastic and structural defects).
[edit] Physical Examination.
Presentation can be dominated by inflammation (specific and nonspecific infection, neoplasia, structural defects, vascular disorders), mass effect (low-grade inflammations such as granulomas and sarcoid, neoplasia, structural defects, vascular conditions such as varices and arteriovenous [A-V] shunts), vascular disturbances (A-V shunt, varix, ophthalmic vein thrombosis, lymphedema), or infiltration (sclerosing inflammation, neoplasia, amyloid deposition, linear scleroderma).
[edit] Clinical Evaluation
During the clinical evaluation of orbital disease the following questions should be considered:
- Where is the lesion?
- What is it doing?
- How quickly?
- What is the pathology?
- What should be done?Answering these questions, in order, directs the history, examination, investigations, and analysis, giving a diagnosis and management plan.
[edit] History.
A detailed, chronologic story should be obtained. When and how did the trouble start? Was progression catastrophic (hemorrhage, fulminant infection), less rapid but steady (inflammation, fulminant neoplasm), or insidious (low-grade inflammation, slowly growing benign or malignant neoplasm)? Does it vary (lid swelling from TAO)? Does it increase with Valsalva's maneuver (venous anomaly)? Does it pulsate (arteriovenous fistula, orbital wall defect)? Previous photographs, investigations, and treatments should be reviewed.
[edit] Examination.
A complete general examination should be done. Findings such as goiter, hair loss, tremor, hyperreflexia, pretibial myxedema, finger clubbing, tachycardia, wide pulse pressure, breast lumps, prostatic malignancy, abdominal masses, pulmonary lesions, testicular masses, and skin lesions may be associated with orbital diseases, and may help guide management.
The face should be inspected for asymmetry, proptosis, and signs of inflammation. Measure the best visual acuity, both near and far; assess eye movements; and examine the fundi. Decide on the level of urgency and refer to an ophthalmologist.
[edit] Investigations.
Thyroid function studies, computed tomography (CT), and magnetic resonance imaging (MRI) are often indicated. A biopsy may be required.
[edit] Differential Diagnosis
Limit the differential diagnosis using clinical and imaging findings (Tables 174-8 and 174-9). Orbital imaging includes ultrasound, plain films, CT, and MRI.
Table 174-8 Clinical Differential Diagnosis of Orbital Diseases
| Clinical signs | Differential diagnosis |
|---|---|
| Bilateral axial proptosis | TAO, acute and subacute idiopathic inflammation, craniofacial disorders, high myopia, lymphomas, leukemias, metastases, A-V shunts |
| Pseudoproptosis | Facial asymmetry, contralateral enophthalmos, ptosis, lid retraction, buphthalmos, high myopia |
| Nonaxial proptosis | Implicates the quadrant opposite the displacement |
| Superior | Maxillary neoplasm, lymphoma, contralateral globe ptosis, lacrimal sac tumor, lower eyelid capillary hemangioma, childhood neoplasm (rhabdomyosarcoma, Ewing's sarcoma) |
| Inferior | Lacrimal gland fossa tumors, sphenoid wing meningioma |
| Temporal | Frontoethmoidal mucocele, secondary sinus and nasopharyngeal tumors, reactive and neoplastic midline lesions, large intraconal masses, metastases, lymphomas, lacrimal sac tumors |
| Nasal | TAO, frontal mucocele, medial dermoid cysts, reactive and dysplastic bone lesions, neurofibromata, rhabdomyosarcoma, neuroblastoma |
| Enophthalmos | Bone defects: fractures, facial asymmetry, neoplastic destruction, sphenoid wing absence) |
| Fat atrophy: (trauma, irradiation, lipodystrophy, recurrent variceal swelling) | |
| Cicatrization: trauma, inflammation, sclerosing metastatic carcinoma (breast, stomach, lung, prostate) | |
| Postsurgical muscle shortening, nystagmus retractorius, sympathetic paresis, linear scleroderma | |
| Dynamic orbital diseases | Pulsate or vary with position or Valsalva's maneuver |
| Movement transmitted from brain or temporalis through bony defect: sphenoid wing absence (neurofibromatosis or meningoencephalocele); destructive lesions (massive frontal mucocele, aneurysmal cyst, reparative granuloma, xanthomata, posttraumatic/surgical dehiscence, dermoid, metastatic lytic tumors, histiocytosis X) | |
| Globe pulsations from vessels: varies during Valsalva's maneuver; shunts (capillary hemangioma, A-V shunts, and vascular tumors [nephroblastoma, thyroid, prostate]) | |
| TAO, Thyroid-associated ophthalmopathy. | |
Table 174-9 Imaging Differential Diagnosis of Orbital Diseases
| Defect | Differential diagnosis |
|---|---|
| Isolated discrete lesions | Benign: low-grade orbital or lacrimal inflammation, cavernous or capillary hemangioma, peripheral nerve sheath tumors, fibrous histiocytoma, hemangiopericytoma |
| Malignant: nodular lymphomas, carcinoid, rhabdomyosarcoma, occasionally metastatic tumors | |
| Cysts | Abscesses: microbial, parasitic (echinococcosis) |
| Degeneration in neoplasms: lymphangioma, pleomorphic adenoma, schwannoma, isolated neurofibroma, rhabdomyosarcoma, melanoma, metastasis | |
| Structural abnormalities: microphthalmos with cyst, conjunctival, sweat gland, lacrimal, dermoid | |
| Isolated infiltrative lesions | Inflammations: nonspecific or specific Neoplastic: benign (plexiform neurofibromas, lymphangiomas, capillary hemangiomas) or malignant (metastatic, fibrous histiocytomas, some lymphomas, leukemia) Depositions: amyloid |
| Extraocular muscle enlargement | Inflammatory: thyroid myopathy (muscle belly enlarged, tendon spared); myositis (muscle belly and tendon swollen) |
| Neoplastic: breast (nodular enlargement, with reticular infiltration of adjacent orbit); melanoma (uniform enlargement); lymphoma (often marked enlargement of levator, superior or medial rectus) | |
| Vascular: A-V shunts (uniform enlargement) | |
| Amyloid deposition: smooth, nodular enlargement | |
| Bone destruction | Solid: primary (reparative granuloma, aneurysmal bone cyst, Ewing's sarcoma, Wegener's granulomatosis, osteogenic sarcoma, osteoblastoma, fibrosarcoma); secondary (sinusitis, histiocytosis X, plasmacytoma, sinus and nasopharyngeal malignancies, lytic meningiomas); metastatic (neuroblastoma, prostate) |
| Cystic: dermoid, mucocele, reparative granuloma, xanthomatous | |
| Hyperostotic: osteomyelitis, meningioma, prostate metastases; primary bone tumors (fibrous dysplasia, osteoma, ossifying fibroma, osteosarcoma, chondrosarcoma) | |
| Optic nerve enlargement | Inflammation gives uniform expansion |
| Infarction gives a central, low density | |
| Neoplastic: glioma, meningioma, plexiform neurofibroma, angiomeningioma, metastasis, leukemia, meningeal spread | |
| Nonneoplastic: sheath expansion (pseudotumor cerebri, chronic papilledema, subarachnoid hemorrhage, or apical crowding of thyroid orbitopathy); nerve expansion (optic neuritis, toxoplasmosis, tuberculosis, sarcoidosis, or infarction) | |
| Calcified lesions without bone destruction | Dystrophic calcification: chronic inflammation, cartilage within dysgenic and malformed globes, phthisis bulbi, choroidal osteoma, optic nerve drusen, hyaline plaque, phlebolith, varix, lymphangioma, old thrombosis in A-V shunt or malformation, old hemorrhage, in the trochlea |
| Neoplasia: malignant and, occasionally, benign epithelial lacrimal gland tumors, extraosseous chondrosarcoma, meningioma, schwannomas, and occasionally lymphomas and neuroblastomas, osseous and cartilaginous soft tissue tumors | |
| Cysts: epithelial, dermoid, mucocele | |
| Displaced, fractured bone | |
| Calcified lesions with bone destruction | Not limited by suture lines: fibro-osseous tumors, epidermoids, mucoceles |
| Limited by suture lines: ruptured dermoids |
[edit] Management
Tailor a plan to the individual, with good communication and coordination among all involved. One physician must take charge, orchestrating care with regard to scheduling, costs, and potential dangers. Optimum care requires conscious effort in planning, communication, and coordination, and judicious, customized application of support, local treatment, medications, radiotherapy, plasmapheresis, hyperbaric oxygen, surgery, and any other treatments available.
[edit] Diagnosis and Management of Specific Diseases
[edit] Structural Defects.
See Table 174-10.
Table 174-10 Orbital Structural Defects
| Structural defect | Clinical presentation | Treatment |
|---|---|---|
| Dermoid cysts | Usually in infancy; at the orbital rim, usually superotemporally; rounded, painless, firm, nonfluctuant, immobile, 1-2 cm diameter mass | Excision using a lid crease incision if possible |
| If not excised, may rupture, causing brisk inflammation | ||
| Dermolipoma | Present from birth or infancy; have fine hairs or subepithelial dermal structures; painless, stable, smooth, yellow, soft subconjunctival masses; usually against the globe superotemporally | Observe |
| Excise only if irritating or a significant blemish | ||
| Herniated orbital fat | In men older than 60 years; more common in Blacks or after weight gain; painless, gradually enlarging, smooth, yellow, soft subconjunctival masses; usually against the globe superotemporally | Observe |
| Excise only if irritating or a significant blemish | ||
| Mucocele | Age 30-80 yr, history of facial fractures, sinusitis; sinus drainage block—slowly push the eye down, laterally, and anteriorly | Remove the mucocele and obliterate the sinus or restore sinus drainage |
| Orbital fractures | Orbital rim with or without displacement; blow-out (of floor +/− medial wall; always suspect after orbital trauma; may have lid emphysema, restriction of vertical gaze with diplopia, enophthalmos); children may have no ocular inflammation | CT scan with axial and positioned coronal views |
| Repair a significantly displaced rim fracture | ||
| Repair within 2 weeks a blow-out fracture with persistent diplopia or enophthalmos; consider repair urgently in children | ||
| Orbital foreign bodies | Always suspect with every open orbital wound; the mechanism of injury may raise the clinical suspicion | Suspect |
| CT scan with axial and coronal views and bone windows | ||
| Remove vegetable and other reactive or potentially infective material | ||
| Inert metallic fragments such as BB's are usually best left | ||
| Orbital puncture wounds | Suspect from the history of injury; sharp objects may cause orbital injuries or pass through the orbital roof with immediate hemorrhage and brain edema or delayed meningitis or brain abscess | Suspect them |
| CT scan of orbits and brain with axial and coronal views and bone windows | ||
| Most need to be removed; some inert, nonreactive materials can be left |
[edit] Atrophies, Degenerations, and Depositions.
See Table 174-11.
Table 174-11 Orbital Atrophies, Degenerations, and Depositions
| Condition | Classification |
|---|---|
| Pseudoproptosis (appearance of proptosis without anterior displacement) | Eyelid asymmetry: lid retraction as in TAO; blepharoptosis not from orbital causes |
| Unilateral globe enlargement: buphthalmos (congenital glaucoma); congenital cystic eye; scleral ectasia (scleral thinning alone); staphylomata (fused scleral and uveal thinning); high myopia | |
| Orbital asymmetry: unilateral bony hypoplasia (linear scleroderma [coup de sabre]); hemifacial atrophy of Parry-Romberg; previous trauma; radiotherapy in childhood | |
| Atrophy and enophthalmos | Microphthalmos |
| Bone loss: tumor, trauma, surgery | |
| Fat atrophy: age, lipodystrophy, facial hemiatrophy, varices; trauma (irradiation, inflammation hemorrhage, tumor removal) | |
| Cicatrization: scirrhous breast carcinoma; trauma, inflammation, surgery | |
| Oculomotor defects | Nystagmus retractorius, Duane's syndrome, muscle shortening after strabismus surgery; scarring from inflammation; progressive external ophthalmoplegia |
| Mass effect | Amyloidosis: systemic (primary—abnormal immunocytes, secondary—from inflammatory disease); localized (primary—no known local disorder, secondary—local inflammation, degeneration |
| Orbital fat prolapse |
[edit] Circulatory Disturbances.
Hemodynamics determine the pathophysiology and clinical picture of orbital vascular diseases. Flow may be lively or intermediate (A-V shunts, venous anomalies), or sluggish (cavernous hemangioma). High-flow shunts may have pulsatile bruits, visible and palpable orbital pulsations, and increased venous pressure, with dilated episcleral veins, elevated intraocular pressure (IOP), chemosis, orbital tissue swelling, and retinal engorgement. Low-flow shunts are milder, with nonpulsatile exophthalmos, no bruit, and less IOP elevation. They are prone to venous thrombosis that may help or aggravate the symptoms. Venous anomalies may distend with Valsalva's maneuver, bruise and swell episodically from spontaneous hemorrhage, and give enophthalmos when emptied.
[edit] Orbital Edema.
In orbital edema tissue fluid raises the intraorbital pressure, giving proptosis and/or optic atrophy. It may be inflammatory (reactive) or noninflammatory (static, toxic, allergic, or vasomotor). Inflammatory or reactive edema from an inflamed paranasal sinus or lacrimal gland gives proptosis, lid edema, chemosis, and mild limitation of eye movements. It may be hard to differentiate from orbital cellulitis. The physician should apply hot compresses and treat the cause. Noninflammatory edema is static (venous obstruction), toxic (exogenous [renal disease], endogenous [iodine or paraphenylenediamine poisoning], acute illnesses [trichinosis, typhus, acute myelitis, malaria, and tonsillitis]), allergic, or vasomotor (urticaria, Quincke's disease, and angioedema).
[edit] Venous Congestion and Thrombosis.
Venous congestion and thrombosis commonly accompany orbital inflammatory and neoplastic diseases, but rarely occur alone. Idiopathic thrombosis of the orbital veins without cavernous sinus thrombosis is rare and confusing, with dilation of the lid and conjunctival and episcleral veins, proptosis, retinal vein congestion with hemorrhages, and glaucoma. Idiopathic thrombosis of the cavernous sinus may affect the healthy patient, sometimes after surgical treatment for trigeminal neuralgia and internal carotid aneurysm, but usually occurs in marasmic infants and the elderly with anemia, dehydration, low blood pressure, and increased blood coagulability. Findings depend on collateral flow. Patients may have acute, sometimes pulsatile proptosis; V1 pain; III, IV, and VI palsy; dilation of retinal veins; glaucoma; and decreased vision. Spread to the other side is common. Treatment is uncertain; prognosis is variable but usually grave.
[edit] Orbital Hemorrhages.
Orbital hemorrhages result from weakened vessel walls (arteriosclerosis, scurvy, rickets), abnormal coagulability (anticoagulation, hemophilia, myelogenous leukemia), increased pressure (systemic hypertension, thoracic compression, strangulation, violent coughing, straining), or trauma (birth injury, retrobulbar injection, penetrating injury, blunt injury with or without fracture). They give sudden, painful proptosis that is axial if bleeding is into tissue, eccentric if subperiosteal; nausea and/or vomiting; mydriasis; decreased vision; retinal vascular compromise; and delayed bruising. Vision decreases transiently or permanently. Treatment is usually conservative, but orbital decompression or CT-guided aspiration or surgical drainage of a subperiosteal hematoma or blood cyst must be done if vision is threatened or resolution is delayed.
[edit] Orbital Varices.
Orbital varices give transient or intermittent proptosis. Primary varices, often with other venous anomalies, present after birth or later in life. Secondary varices result from an A-V shunt. Onset is dramatic with transient proptosis, lasting from a few seconds to a few days, recurring at intervals from a few hours to a few years, and producing proptosis from a few to 30 mm. They are always unilateral and usually left-sided, and may be produced by bending the head forward, less commonly backward or from side to side, particularly to the right; pressure on the jugular veins; stooping; a tight collar; coughing; taking a deep breath; holding the breath; and Valsalva's maneuver. Findings include pain, nausea, bruit, visible pulsations, “visual black-outs,” blindness, mydriasis, retinal engorgement, and phleboliths on plain films. Resection is difficult; prognosis is guarded. In the Klippel-Trenaunay-Weber syndrome, orbital venous varicosities may be associated with hypertrophy of soft tissues and bone.
[edit] Orbital Aneurysms.
Orbital aneurysms give pulsatile exophthalmos, as do vascular tumors, varices, A-V shunts, and bony defects (meningoceles, sphenoidal mucoceles, sphenoidal hypoplasia in neurofibromatosis). Ophthalmic artery aneurysms are fusiform or, less often, saccular. Pulsating proptosis, optic nerve dysfunction, and diplopia may occur. Angiography should be used for diagnosis; treat with ligation or excision. Lacrimal, ethmoidal, and internal maxillary artery aneurysms occur less commonly.
[edit] Arteriovenous Fistulas.
A-V shunts, the most common cause of pulsating exophthalmos, are congenital, traumatic or spontaneous (caroticocavernous or dural), and usually in the cavernous sinus. Congenital shunts are rare, occurring alone or with Wyburn-Mason's and Rendu-Osler-Weber syndromes. Traumatic cases (males > females) are due to penetrating, contrecoup, or blunt injuries with basal skull fractures. Onset may be delayed or gradual but is usually sudden, with unilateral or bilateral pulsating proptosis, a swishing noise in the head, pain, and decreased vision. Spontaneous cases (females > males, 25% in pregnancy), resulting when an aneurysm bursts into the cavernous sinus, begin dramatically with vomiting, vertigo, unconsciousness, and sometimes death. Dural shunts (postmenopausal women) have a chronic, fluctuating course with exacerbations, due to thrombosis, and spontaneous resolution in 40%. In all shunts, the degree of proptosis, pulsation, lid swelling, chemosis, venous engorgement, corneal exposure, glaucoma, cranial nerve (III, IV, V-1, VI) palsy, retinal involvement, and bruit varies from slight to marked. The proptosis, bruit, and thrill are increased on stooping or exertion, and diminished with carotid compression on the affected or both sides. CT may show proptosis, extraocular muscle and superior ophthalmic vein enlargement, and a nonenhancing, superior ophthalmic vein or cavernous sinus defect, suggesting thrombosis. Arteriography, often with superselective catheterization, is essential for diagnosis. Prognosis is poor, although some resolve spontaneously. Detachable balloon-catheter occlusion and a multidisciplinary approach have improved the outlook for high-flow cases.
[edit] Orbital Inflammations, Infections, and Infestations.
In a busy orbital clinic, 57% of cases were inflammations, infections, or infestations: 47% dysthyroid, 4.7% nonspecific, 3.7% specific infective, and 1.6% other specific. Inflammations may be nonspecific or specific; infections may be viral, bacterial, or fungal; and infestations may occur with protozoa, roundworms, flatworms, or arthropods.
[edit] Nonspecific Inflammations
[edit] Acute and subacute idiopathic inflammatory syndromes (pseudotumors).
These have a varying and confusing presentation, outcome, and histopathology. Onset is acute or subacute with pain, dilated vessels, and edema, with or without malaise. CT shows an irregular margin by the primary focus, swelling, and contrast enhancement. It may resolve rapidly with treatment or be chronic with progressive infiltration, destruction, and fibrosis. Histopathology shows fibroblasts, neutrophils, lymphocytes, plasma cells, and macrophages. Location may be anterior, diffuse, apical, myositic, or lacrimal (Table 174-12). Treatment for all these inflammations requires biopsy unless very difficult, intralesional steroids at biopsy, then oral prednisone 80 mg daily. Most respond rapidly. If recurrent with rapid tapering, restart prednisone and taper more slowly. If again recurrent with tapering, or unresponsive, biopsy is mandatory. If benign, consider radiation, with 1000 to 3000 centigray (cGy), or chemotherapy.
Table 174-12 Orbital Inflammatory Syndromes: Anatomic Patterns
| Syndrome | Presentation | Investigations | Differential diagnosis |
|---|---|---|---|
| Anterior | Young, pain, proptosis, lid swelling, ptosis, injection, and decreased vision from uveitis, sclerotenonitis, papillitis, or exudative retinal detachment | CT: diffuse anterior orbital infiltration against the eye, scleral and choroidal thickening, globe–optic nerve junction blurring, nerve sheath extension | Orbital cellulitis, a sudden event in a preexisting lesion (dermoid cyst rupture, vascular lesion hemorrhage), ocular inflammation (scleritis, uveitis), collagen vascular diseases, and, in children, rhabdomyosarcoma, neuroblastoma, and leukemia |
| Ultrasound: irregular, uniform anterior infiltrate, sclerotenonitis with Tenon's space accentuation, optic nerve shadow doubling (T sign) | |||
| Diffuse | More severe: ocular movement limitation, papillitis, exudative retinal detachment | CT: soft tissue infiltration from apex to globe, blurring of optic nerve and muscles | As for anterior |
| Apical | Pain, minimal proptosis, restricted eye movements, often decreased vision | CT: irregular apical infiltration with extension along the extraocular muscles or the optic nerve | Optic neuritis, tumors, Tolosa-Hunt syndrome |
| Myositic | Lid swelling, ptosis, chemosis and injection over the affected muscles, diplopia, restricted eye movement, and pain, worse when the affected muscles act | CT: irregular extraocular muscle swelling including the tendon (unlike Graves' disease), and localized fat infiltration | Graves' disease, Lyme disease, metastases |
| Forced duction test may be positive; often unilateral; one or more rectus, usually superior or medial; may be recurrent | |||
| Lacrimal | Female>male, any age, pain, inferonasal proptosis, S-shaped upper lid, temporal lid and conjunctival fornix injection, little motility disturbance, a tender, palpable lacrimal gland, pouting of lacrimal ducts | CT: infiltration in the superolateral orbit | Viral and bacterial dacryoadenitis, dermoid cyst rupture, sarcoidosis, Sjögren's syndrome, cysts, neoplasms |
| Chronic: few inflammatory signs, may be bilateral, recurrent, and associated with alopecia or colitis | Ultrasonography: mass with internal reflectivity, an echolucent area next to the sclera, anterior thickening of adjacent muscle |
[edit] Idiopathic sclerosing inflammation.
Idiopathic sclerosing inflammation entraps orbital structures in scar. Sometimes part of multifocal fibrosclerosis, usually unilateral, it gives diplopia, proptosis, progressive visual loss, lid retraction, mild inflammation, and pain. CT scan shows homogeneous, dense lesions, often contrast-enhancing, with regular margins, infiltrating orbital fat and muscles. Histopathology shows scarring with scattered inflammatory cells. Differential diagnosis includes chronic inflammations (Wegener's and other granulomatous inflammations), secondary and metastatic cancers (especially sclerosing types), and lymphomas. Initial response to steroids may be good, but later, relentless progression is the rule. Biopsy, then treat with prednisone (80 mg daily) plus radiotherapy (2500 to 3000 cGy).
[edit] Idiopathic noninfectious granulomatous inflammation.
Idiopathic noninfectious granulomatous inflammation is defined by its histopathology (nonnecrotizing granuloma or lipogranuloma), affecting orbital soft tissues without specific localization or systemic associations. It presents with mild inflammation, a palpable mass, and a slow onset. Treat with excision or prednisone 80 mg daily.
[edit] Specific Inflammations.
Specific inflammations include TAO, sarcoidosis, Sjögren's syndrome, vasculitides, other granulomatous and histiocytic diseases, and orbital inflammation secondary to ocular disease (Table 174-13).
Table 174-13 Specific Orbital Inflammations✢
| Inflammation | History | Findings | Treatment |
|---|---|---|---|
| Thyroid-associated ophthalmopathy (TAO) | Variable and unpredictable; 90% mild, noninfiltrative disease; 10% severe, infiltrative disease; 30%+ámily history; associated with diabetes mellitus and myasthenia gravis; TAO occurs within 18 mo of diagnosis of thyroid disease; bulging eyes, pressure behind eyes, irritated eyes, lid pulled up, lid swelling, tearing, double vision, decreased vision | Variable; exophthalmos; eye movement restriction; lid swelling; more pigment; lid lag, retraction with “scleral show,” and stare; conjunctival injection and chemosis, especially over insertion of rectus muscles; cornea exposure; compressive optic neuropathy; lacrimal gland enlargement and prolapse, tearing; CT enlarged muscles; laboratory: T3, T4, TSH, antibodies | Chronic, depressing disease, give support Stop smoking Treat thyroid disease Lubricants Corticosteroids: buy time; avoid chronic use Surgery: fix retracted lids; strabismus correction; decompress orbit Radiation Immunosuppressive drugs |
| 1. Most common cause of both unilateral and bilateral proptosis | |||
| 2. An organ-specific, autoimmune-mediated inflammation of the extraocular muscles and perhaps the periorbital connective tissue | |||
| 3. Muscles swell, pushing the globe forward, tethering the levator and rectus muscles, and squeezing the optic nerve | |||
| Sarcoidosis (multisystem, immunologic disorder, with noncaseating granulomas involving many tissues [especially hilar lymph nodes and pulmonary parenchyma] with symptoms dependent on site and degree of involvement) | Highest risk in black women age 20-40 yr in southern United States; dry eyes, swollen upper lids, lid bumps | Eyelid nodules or papules, uveitis, chorioretinitis, dry eye, lacrimal gland enlargement, conjunctival nodules, neuropathies, and, very rarely, deep orbital disease | Treat systemic disease Local corticosteroids Surgery as indicated |
| Sjögren's syndrome (chronic, systemic inflammatory disorder, unknown cause, with dry mouth, eyes, and other mucous membranes, associated with autoimmune diseases) | Dry irritated eyes, dry mouth, epistaxis, reduced sense of smell, hoarseness, recurrent bronchitis, and increased risk of malignant lymphoma and pseudolymphoma | Dry eyes, filamentary keratopathy, enlarged tender lacrimal and parotid glands, anti SS-A antibodies positive | Local lubricants |
| Systemic with systemic steroids and immunosuppressives | |||
| Vasculitides: (1) periarteritis nodosa (classical polyarteritis nodosa, allergic angiitis and granulomatosis [Churg-Strauss], and systemic necrotizing vasculitis “overlap syndrome”), (2) hypersensitivity (leukocytoclastic) angiitides (orbital vasculitis, vasculitides with connective tissue disorders, Cogan's syndrome); (3) Wegener's granulomatosis; (4) other respiratory vasculitides; and (5) giant cell arteritis. Early cases mimic nonspecific inflammations. Diagnosis can be difficult and treatment specialized. | |||
| Orbital Inflammation Secondary to Ocular Disease: These include: endophthalmitis, severe uveitis, and scleritis. The diagnosis is usually clear, but posterior scleritis can mimic other acute and subacute orbital inflammations. Suspect it in older patients, usually those with collagen-vascular diseases, presenting with orbital inflammation, severe aching pain, and typical ultrasound and CT findings. | |||
✢Other granulomatous and histiocytic diseases include eosinophilic granuloma (histiocytosis X), juvenile xanthogranuloma, Erdheim-Chester disease, necrobiotic xanthogranuloma, pseudorheumatoid nodules, and fibrous histiocytoma.
[edit] Infections.
Infections may be viral, bacterial, or fungal. Viral orbital infections produce acute dacryoadenitis. Bacterial and fungal orbital infections produce acute and chronic dacryoadenitis, and acute, subacute, and chronic orbital cellulitis (Table 174-14).
Table 174-14 Orbital Infections✢
| Infection | History | Findings | Treatment |
|---|---|---|---|
| Acute orbital cellulitis (usually from sinus; threatens vision and life; non-sinus sources include: face, teeth, meninges, ear, conjunctiva, eye, lacrimal sac or gland, bacteremia, foreign body; may be preseptal or postseptal [orbital]; cavitate forming a subperiosteal or orbital abscess, or spread, causing cavernous sinus thrombosis, intracranial abscess or thrombophlebitis, or temporalis fossa abscess) | Younger children: viral coryza, spread from the ethmoid and maxillary sinuses (not frontal), and infection with aerobes (H. influenzae) | Preseptal cellulitis (usually S. aureus or S. pyogenes) gives pain, lid swelling, chemosis, and systemic toxicity with fever and leukocytosis | Adult preseptal cellulitis: hot compresses and oral dicloxacillin 500 mg qid |
| All others: admit, IV antibiotics, infectious diseases consultation, drain abscesses as necessary | |||
| Adults: sinusitis, polyps, allergy, trauma, or recent dental extraction, spread from frontoethmoidal sinuses, and multiple microbial infection, often anaerobic | Orbital (postseptal) cellulitis adds proptosis, limited eye movement, and decreased vision | ||
| Subperiosteal abscesses, usually superior or retrobulbar, are poorly defined masses showing homogeneous, heterogeneous, or ring enhancement on CT with contrast; infrequent in children, when treated, usually resolve without drainage or sequelae; more common in adults, often require sinus drainage, and have more severe sequelae | |||
| Osteomyelitis may result | |||
| Subacute and chronic orbital infections | |||
| Orbital tuberculosis: periostitis | >20 yr, involves the zygomatic bone | Painless, indolent, red swelling that points and drains | Biopsy |
| Drugs | |||
| Generous debridement | |||
| Orbital tuberculosis: tuberculoma | Most common in women 40-60 yr; TB elsewhere | Painless, slowly enlarging mass, often on superomedial orbital wall | Biopsy, debulk, and treat with antituberculosis drugs, expecting steady improvement |
| Orbital syphilis: marginal periostitis (orbital syphilis, in tertiary disease, presents as a diffuse hyperplastic periostitis involving the margin, walls, or apex) | Most commonly involves superior rim, indolent inflammatory swelling, tenderness, intractable headache, radiating neuralgia most marked at night | Bony thickening with boss formation may be marked or the gumma may break down, leaving a persistent fistula with a depressed scar leading down to softened bone; bony absorption may be marked, especially in congenital cases | Antisyphilitic antibiotics |
| Expect rapid improvement | |||
| Orbital syphilis: periostitis of the orbital _walls | Antisyphilitic antibiotics | ||
| Orbital syphilis: gummatous apical periostitis | Antisyphilitic antibiotics | ||
| Fungal infections | |||
| Rhinoorbital mucormycosis or phycomycosis | Immunocompromised patients with diabetic ketoacidosis or AIDS, or those receiving corticosteroids systemically or as nasal sprays; sinusitis, pharyngitis, and nasal discharge; it presents with a boring orbital pain | Dramatic cellulitis, proptosis, cranial nerve pareses, visual loss, and general deterioration; thrombosing arteritis with gangrene of the nasal mucosa, turbinates, and palate, septal perforation, and intraluminal spread into the brain | Treat underlying disease, biopsy stat: ask for rapid histologic processing |
| Start IV amphotericin B | |||
| Positive biopsy, generously debride, post-operative drainage | |||
| Local amphotericin B irrigation; possibly hyperbaric oxygen (controversial) | |||
| Grave prognosis | |||
| Orbital aspergillosis (allergic and invasive) | Immunocompromised patients living in hot, humid climates; a slowly enlarging mass | Spreads to the orbit from a paranasal sinus or the lacrimal sac; biopsy: long septate filaments with dichotomous branches that stain with hematoxylin-eosin stain as well as Gomori methenamine silver | Excise |
| Amphotericin B | |||
| Orbital actinomycosis | Slowly increasing proptosis and nasal obstruction | Spreads into the orbit from the mouth, nose, paranasal sinuses conjunctiva, lids, or canaliculus | Debridement and penicillin |
✢For acute viral dacryoadenitis and acute and chronic bacterial dacryoadenitis see Table 174-7.
[edit] Infestations.
Infestations include those with protozoa (amebiasis), roundworms (nemathelminthes: filariasis, dracontiasis, trichinosis), flatworms (platyhelminthes: schistosomiasis, paragonimiasis, sparganosis, echinococcosis, cysticercosis), mollusks, and arthropods (orbital ophthalmomyiasis). Knowledge of the patient's geographic location and the life cycle of the parasite aids diagnosis.
[edit] Protozoa.
Amebiasis is a very rare cause of an orbital inflammatory mass.
[edit] Nemathelminthes.
The roundworms Wuchereria bancrofti and Dirofilaria conjunctivae can cause chronic granulomatous orbital cysts mimicking orbital tumors. Dracunculus medinensis can infest the orbit. About 11 days after an individual eats infested meat, Trichinella spiralis, during dissemination, produces a doughy edema of the eyelids, especially the upper, occasionally with chemosis and a reddish rash. Extraocular muscle invasion is early, limiting eye movement because of pain and causing a transient, pale, lemon-jelly chemosis, usually bilateral, over the insertions of the horizontal rectus muscles. Later generalized aches and systemic symptoms supervene. Death from myocarditis, encephalitis, meningitis, bronchopneumonia, or nephritis may follow. The physician should diagnose from the clinical picture, high eosinophilia, positive serology, and biopsy.
[edit] Platyhelminthes.
Flatworms Schistosoma haematobium and Paragonimus westermani may give orbital cysts. Sparganum mansoni may produce painful proptosis, lid edema, and chemosis resembling cellulitis. Treat with excision. In endemic areas suspect a hydatid cyst from Taenia echinococcus as a potential cause of unilateral proptosis of insidious onset in a young person. Treat with excision. Cysticercosis cellulosae from T. solium is the most common helminthic ocular infection in man. Subretinal, vitreous, and conjunctival involvement is frequent; orbital disease is rare, producing pea-sized anterior cysts. Treat with excision.
[edit] Arthropoda.
Various species of the fly family Calliphoridae Arthropoda produce orbital ophthalmomyiasis. Risk factors include tropical climates, poor hygiene, poverty, debilitation, malodorous gonococcal conjunctivitis, ulcerated periorbital cancer, and the extremes of age. Tissue destruction may be extensive. Treatment is difficult.
[edit] Orbital Cysts.
Except for dermoids and mucoceles, orbital cysts are rare. They include lacrimal cysts (simple palpebral and orbital lobe cysts, multiple cystic degeneration, parasitic cysts, Krause's glands' cysts) and orbital cysts (simple epithelial cysts [primary cutaneous epithelial cysts or epidermoid cysts, primary conjunctival epithelial cysts, primary respiratory epithelial cysts, implantation epithelial cysts, and sudoriferous cysts or Moll's gland cysts]; dermoid cysts; teratomas; congenital cystic eye; colobomatous cysts; meningocele; meningoencephalocele; primary optic nerve sheath cysts; mucoceles; hematoceles; hematic cysts; parasitic cysts [W. bancrofti, T. echinococcus, T. solium, and Multiceps]; and dentigerous cysts). Most of these have been discussed.
Acquired lacrimal cysts are cystic swellings of the excretory ducts. They are blue-domed; usually single, although sometimes multiple or bilateral; and visible through the conjunctiva that transilluminates. Resect carefully with out damage to other ducts.
[edit] Orbital Tumors.
Orbital tumors can be congenital, primary, secondary, or metastatic. Congenital tumors and malformations will not be discussed. Primary tumors can be neurogenic, mesenchymal, vascular, lacrimal, lymphoproliferative, or leukemic. Secondary tumors can spread from nasopharynx and paranasal sinuses, bones, intracranial tissues, eyelids, conjunctiva, lacrimal sac, or eye. Metastatic tumors commonly spread from the breast, lung, adrenal gland, prostate, GI tract, kidney, thyroid, skin, and bone.
[edit] Primary Orbital Tumors
[edit] Neurogenic tumors.
Neurogenic tumors include those of the optic nerve, meninges, peripheral nerves, and rare neuroectodermal tumors (Table 174-15).
Table 174-15 Primary Orbital Neurogenic Tumors
| Tumor | History | Findings | Management |
|---|---|---|---|
| Optic nerve tumors✢ | |||
| Juvenile optic nerve glioma; low-grade pilocytic astrocytomas | Females>males; 75% <10 yr; neurofibromatosis; decreased vision; proptosis | Chiasmal, orbitocranial, orbital, and diffuse or multifocal; grow slowly and intermittently | Excision and/or radiation is controversial |
| Malignant optic glioma/glioblastoma of adulthood | Middle aged; visual loss early; rapid progression | Decreased vision, disc edema; CT shows enlargement of nerve or chiasm | Poor prognosis |
| Meningioma | |||
| Intracranial meningiomas | Women/men = 3/1; at risk– neurofibromatosis; most common in fifth decade; vision loss, palsies, mass effect | Sphenoid ridge, suprasellar, olfactory groove; CT: bony hyperostosis and/or lysis; well-defined, homogeneous, soft-tissue mass with uniform post-contrast enhancement; a “tumor blush” | Excision, debulking, radiation, hormonal manipulation |
| Optic canal meningiomas | Women/men = 3/1; at risk– neurofibromatosis; early visual loss from optic nerve compression | CT or MRI demonstration difficult; spread over the planum sphenoidale to the opposite optic nerve | Excise before chiasm involved |
| Orbital optic nerve meningiomas | Women/men = 2/1; at risk– neurofibromatosis; bimodal peak second and fifth decade, second more aggressive; early transient visual obscurations in gaze extremes, but otherwise minimal visual impairment; later: mild proptosis, progressive field constriction | Increasing disc edema with gliosis and refractile bodies, optociliary shunts, and choroidal folds; finally, vision is lost, nerve head gliosis and atrophy increase, and the refractile bodies disappear; CT: diffuse, fusiform swelling | Excise the lesion using: a lateral orbitotomy, if it clearly spares the posterior one third of the nerve, or a neurosurgical approach otherwise |
| Consider optic canal decompression or radiotherapy when the remaining eye is involved | |||
| Peripheral nerve tumors | |||
| Neurofibromas, isolated (generally dermal) tumors | 90% do not have neurofibromatosis; often multiple; middle age | Solitary, slow-growing mass, anesthesia, paresthesia, hypesthesia | Resect, being careful not to damage a motor nerve |
| Neurofibromas, plexiform lesions | Neurofibromatosis: most common and complex orbital peripheral nerve tumors; elephantiasis neuromatosa | Hypertrophic skin, lid, and face, proptosis and disfigurement; palpation: “bag of worms” | Debulking difficult |
| Recurrence common | |||
| Orbital amputation neuromas | Previous injury, neuralgic pain | May be very small; sensitive, trigger point | Pain cured by excision |
| Schwannomas, neurilemomas | Adults age 20-50, 18% have neurofibromatosis | Intraconal: proptosis, lid swelling, globe indentation, diplopia in extremes of gaze, and central scotomata, vision fluctuation on lateral gaze; extraconal: hypesthesia | Excise Prognosis good |
| Malignant peripheral nerve sheath tumors (perineural fibrosarcoma, malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma) | Adults age 20-60 yr, males>émales: 50% neurofibromatosis | Painful superonasal mass growing over weeks to months, with paresthesia and tenderness | Biopsy and treat with exenteration or more radical “craniofacectomy” |
✢Others: medulloepitheliomas/neuroepitheliomas, secondary and metastatic tumors, primary CNS tumors, and pseudoneoplasms.
[edit] Primary mesenchymal tumors.
Primary mesenchymal tumors include those of bone and cartilage, striated and smooth muscle, fibrous tissue, histiocytes, and fat. Most are osseous, with striated muscle and histiocytic tumors next, and fat and fibrous tumors being rare. In one series primary mesenchymal tumors made up 3% of orbital cases and 16% of orbital neoplasia.
Orbital bone tumors, all rare, include dysplasia and related fibro-osseus lesions, reactive lesions, and neoplasms. Patterns of presentation include:
- A slowly growing, noninfiltrative tumor, seldom-eroding bone, with nonaxial proptosis and facial disfigurement (e.g., fibrous dysplasia, osteoma, chordoma, or low-grade chondrosarcoma).
- Bleeding into a bone-eroding dysplasia giving acute proptosis (e.g., reparative granuloma, aneurysmal bone cyst, reactive xanthomatous lesions, or brown tumor of hyperparathyroidism).
- A relentless, infiltrative neoplasm with pain, hypesthesia, muscle restriction, and optic neuropathy (e.g., osteogenic sarcoma, Ewing's sarcoma, malignant fibrous dysplasia, histiocytoma, histiocytosis X, lymphoma, or plasmacytoma).
The histopathologic diagnosis of bony tumors is difficult, requiring correlation with radiographs.
In childhood, rhabdomyosarcoma is both the most common soft tissue malignancy and the most common primary orbital malignancy (70% in first decade; most < age 7 years; male:female ratio, 5:3; 50% retrobulbar, 25% suprabulbar, 12% infrabulbar). Seldom familial, it is subacute, acute, or often fulminant, with inferolateral proptosis, no pain or decreased vision, but ptosis, lid injection, and swelling.
The histology tends to change with age: embryonal in childhood, alveolar in adolescence, and pleomorphic in adults. Embryonal and alveolar types arise in soft tissue, not extraocular muscles. Pleomorphic lesions arise from dedifferentiation of, and are associated with, adult muscles. Embryonal lesions make up two thirds of cases. Alveolar lesions, more common in the inferior orbit, have the poorest prognosis; adult pleomorphic lesions, the least common, have the best. A new classification divides them into three groups: anaplastic (ANA), monomorphous round cell (MRC), and mixed (MX), the last having the best prognosis.
Differential diagnosis includes progressive, rapidly developing tumors and inflammations, including neuroblastoma, chloroma, lymphangioma, infantile hemangioma, ruptured dermoid cysts, cellulitis, and nonspecific inflammations. Management involves biopsy, staging, limited resection, radiation (4000 to 5000 cGy), and chemotherapy, and is best done in centers with experience. Five-year survival is 65% for all rhabdomyosarcomas occurring anywhere, but 95% with lesions limited to the orbit, because of early detection and lack of lymphatic metastases. Treatment morbidity includes cataracts, keratopathy, retinopathy, ptosis, enophthalmos, lacrimal duct stenosis, facial asymmetry, bone hypoplasia, leukemia, and behavioral problems.
Smooth muscle tumors (leiomyoma and leiomyosarcoma), fibrous tissue tumors (fibroma, nodular fasciitis, fibromatoses, fibrosarcoma, congenital and infantile fibrosarcoma, myxoma), and adipose tumors (lipomas, liposarcomas) are very rare, and should be excised.
Histiocytic tumors and tumorlike lesions include fibrous histiocytoma and juvenile xanthogranuloma. Fibrous histiocytoma, the most common adult mesenchymal orbital tumor, presents in middle age as a slow-growing or aggressive mass, rarely metastasizing, that is commonly found in the upper nasal quadrant or lacrimal sac or gland. The physician should biopsy and resect. Radiotherapy is not useful. Orbital juvenile xanthogranuloma is rare.
[edit] Vascular tumors and malformations.
Vascular tumors and malformations include primary vascular tumors, A-V shunts and venous anomalies, congenital vascular anomalies (phako matoses), aneurysms, obstructions, and unclassified hemorrhages. Mass effect, vascular engorgement, orbital pulsation, and intermittent exophthalmos suggest this diagnosis.
[edit] Primary vascular tumors.
Primary vascular tumors include hamartomas, choristomas, and neoplasms. Infantile hemangiomas have high flow, cavernous hemangiomas have low flow, and lymphangiomas and some solid tumors have “no” flow.
Hamartomas include infantile capillary hemangiomas and cavernous hemangiomas. Infantile capillary hemangiomas can occur in the orbit as well as in the eyelids, and can mimic other orbital tumors. They should be treated with observation unless the behavior requires exploration. Cavernous hemangioma, the most common primary orbital tumor of adults, is a benign, well-encapsulated, slow-growing, usually intraconal, and well-tolerated orbital mass. It may give proptosis, posterior pole indentation, choroidal striae, optic nerve compression, diplopia, orbital pain, or transient gaze-induced amaurosis. CT scan shows a smooth, well-defined, oval or rounded, intraconal, poorly enhanced mass. Most are lateral, may bow the lateral orbital wall, and occasionally extend extraconally. A- and B-scans are pathognomonic. These should be observed or resected. The plump, nodular, plum-colored, relatively avascular, encapsulated mass with surface vascular channels is usually easily removed in toto or pieces.
Choristomas include lymphangiomas. Most common between ages 1 and 15 years, they are worse with upper respiratory tract infections, are sometimes associated with face and neck lesions, are relatively avascular, and may present as superficial, deep, or combined. Superficial lymphangiomas give a clear, yellowish, blood-filled, or bluish lid or conjunctival cyst or cysts that can be transilluminated. They should be resected if unsightly. Deep lymphangiomas present, usually in childhood, with intralesional hemorrhage and sudden proptosis that may gradually increase, perhaps from osmosis, and may compress orbital structures (chocolate cyst). There is no arterial or venous connection. CT scan shows low-density, cystlike masses behind orbital septum, with a thin rim of enhancement, focal-enhancing areas outside the cysts, and orbital enlargement. Combined superficial and deep lymphangiomas present, usually in infancy, sometimes with oral or intracranial lesions; enlarge slowly over many years, sometimes becoming massive; and bleed repeatedly with recurrent subconjunctival hemorrhages, periorbital ecchymoses and swelling, and progressive optic nerve damage. Resection of deep and combined lesions when indicated may be difficult because they are poorly circumscribed in a mass of orbital scar. A CO2 laser may help. Recurrence is common.
Vascular neoplasms include hemangiopericytoma, malignant hemangioendothelioma, Kaposi's sarcoma, angiolymphoid hyperplasia, and vascular leiomyoma (angiomyoma).
[edit] Arteriovenous shunts and venous anomalies.
Arteriovenous shunts and venous anomalies are discussed in Circulatory Disturbances.