The Wills Eye Manual

Chapter 6. Eyelid

6.1 Ptosis

Symptoms

Drooping upper eyelid, superior visual field compromise. Occasional visual compromise often gets worse with reading and at night. Concerning associated symptoms include diplopia, headache, and neck pain.

Signs

(See Figure 6.1.1.)

FIGURE 6.1.1 Ptosis.

Critical. Drooping upper eyelid.

Other. Concerning associated signs include anisocoria, proptosis, and ocular motility deficits. See individual entities.

Etiology

NOTE: Although the majority of ptosis is aponeurotic and of benign etiology, certain entities must be ruled out by careful examination.

1. Horner syndrome.

2. Third cranial nerve (CN) palsy (complete, partial, or aberrant CN III regeneration).

3. Myasthenia gravis.

4. Orbital tumor.

5. Eyelid/conjunctival tumor.

6. Chronic progressive external ophthalmoplegia (CPEO) (particularly Kearns-Sayre syndrome, see 10.12, Chronic Progressive External Ophthalmoplegia).

Categories:

 Myogenic: While this term is often used to describe aponeurotic ptosis, true myogenic ptosis is nearly always congenital. It is present at birth and is caused by localized dysgenesis of the levator palpebrae superioris. It results in poor levator function (0 to 5 mm) and poor or absent eyelid crease. A poor Bell phenomenon (palpebral oculogyric reflex), lagophthalmos in downgaze, and upgaze limitation may indicate a double elevator palsy. Acquired myogenic ptosis is uncommon and may be seen with muscular dystrophy and CPEO.

 Aponeurotic: Common cause of ptosis. Occurs secondary to levator dehiscence and is characterized by a high or absent eyelid crease, moderate degree of ptosis, and preserved levator function (10 to 15 mm). Often gets worse in downgaze. Levator stretching or dehiscence can result from normal aging, repetitive eye rubbing, use of rigid contact lenses (pulling on the eyelids to put in or take out), trauma, or previous intraocular surgery (speculum-related muscle damage).

 Neurogenic: Third CN palsy (often complete ptosis, never an isolated abnormality; congenital, compressive, or vasculopathic, see 10.5, Isolated Third Cranial Nerve Palsy); Horner syndrome (mild, ~2 mm upper and lower eyelid ptosis, see 10.2, Horner Syndrome); myasthenia gravis (variable degree, duration, and laterality, worsens with fatigue, see 10.11, Myasthenia Gravis); Marcus Gunn jaw-winking syndrome (ptotic eyelid elevates with jaw movement); ophthalmoplegic migraine; and multiple sclerosis.

 Mechanical: Foreign-body or retained contact lens in upper fornix; upper eyelid or forniceal inflammation (chalazion, giant papillary conjunctivitis, posttraumatic or postsurgical edema); and neoplasm.

 Traumatic: Eyelid laceration with levator involvement, levator contusion, tethering or ischemia within an orbital roof fracture, late dehiscence, or cicatricial changes.

 Pseudoptosis: Contralateral eyelid retraction or proptosis, ipsilateral enophthalmos or hypertropia, microphthalmia, phthisis bulbi, dermatochalasis, brow ptosis, eyelid tumor, edema, blepharospasm, or Duane syndrome.

Workup

1. History: Onset and duration? Present since birth? Old photographs (e.g., driver’s license) and family members' opinions are useful adjuncts to the history. Prior surgery in either eye? Trauma? Fluctuation throughout the day? Associated diplopia, headache, or neck pain? Trouble breathing or swallowing, associated drooling? History of autoimmune disease?

2. Mandatory documentation: Must carefully check and document pupillary size and extraocular motility, even if normal. If anisocoria is present, measurements should be documented under light and dark conditions. Additional pharmacologic testing may be indicated (see 10.1, Anisocoria). If extraocular muscle dysfunction is noted, additional testing with prism bars may be indicated.

3. Complete orbital examination: Measure and compare globe position with Hertel exophthalmometry, margin reflex distance, levator function (full upper eyelid excursion with frontalis muscle held inactive at the brow), and upper eyelid crease position of both eyes. Is there lagophthalmos? Associated lower eyelid “ptosis” (elevation of ipsilateral lower eyelid retractors) is often seen in Horner syndrome. Proptosis or eyelid lag may masquerade as contralateral ptosis. Signs of aberrant eyelid movements like jaw-winking, variability and/or fatigue, orbicularis weakness, and eyelid retraction with adduction and/or infraduction? Resistance to retropulsion? Palpate superior orbit to rule out a mass or superior orbital rim deformity.

4. Complete ocular examination: Flip upper eyelid to examine conjunctival surface and superior fornix. Dilated fundus examination to look for pigmentary changes in adolescents and young adults who present with ptosis, poor levator function, and external ophthalmoplegia (possible CPEO and Kearns-Sayre syndrome).

5. Corneal protective mechanisms: Document presence or absence of lagophthalmos, orbicularis function, Bell phenomenon, and tear production. Check the cornea carefully for any abnormalities or dystrophies, which may predispose the patient to keratopathy.

6. Other tests

Ice test: Apply ice pack to ptotic eye(s) for 2 minutes, measuring eyelid position before and after. Improvement in eyelid position is highly suggestive of myasthenia gravis.

 Phenylephrine test: Instill one drop of 2.5% phenylephrine in the ptotic eye(s). Patients with an improvement of ptosis after 5 to 7 minutes may be good candidates for ptosis correction by Muller muscle-conjunctival resection.

 Apraclonidine, cocaine, and hydroxyamphetamine tests. See 10.2, Horner Syndrome.

NOTE: In recent years, limited access to ophthalmic cocaine and hydroxyamphetamine has reduced their utility in the clinical setting.

7. Imaging studies: In cases where a systemic or neurologic cause is suspected:

 Computed tomography (CT) or magnetic resonance imaging (MRI) of orbit if a superior orbital mass is suspected.

 CT/computed tomography angiogram (CTA) or MRI/magnetic resonance angiogram (MRA) of the head and neck if Horner syndrome is present. This should be performed emergently if there is clinical suspicion for carotid artery dissection (neck pain, acute onset, and a history of trauma). Imaging of the head alone is inadequate. See 10.2, Horner Syndrome.

 Emergent CT/CTA, MRI/MRA, or conventional angiography to rule out posterior communicating artery aneurysm is indicated for all third CN palsies whether pupil-involving or pupil-sparing. See 10.5, Isolated Third Cranial Nerve Palsy.

 Chest CT if either Horner syndrome (to rule out apical lung mass compressing sympathetic ganglion) or myasthenia gravis (to rule out thymoma) is suspected. See 10.2, Horner Syndrome and 10.11, Myasthenia Gravis.

8. Ancillary studies:

 If myasthenia gravis is suspected, acetylcholine receptor antibody (binding, blocking, and modulating) testing, single-fiber electromyography (including the orbicularis muscle), and/or edrophonium chloride testing under monitored conditions may be indicated. See 10.11, Myasthenia Gravis.

 Urgent ECG and cardiology consult if Kearns-Sayre syndrome is suspected. These patients can have heart block, resulting in sudden death.

Treatment

1. Depends on the underlying etiology (see 10.2, Horner Syndrome; 10.5, Isolated Third Cranial Nerve Palsy; 10.11, Myasthenia Gravis).

2. Nonsurgical options: Observation. Taping upper eyelids open and eyelid crutches attached to glasses in neurogenic and myogenic ptosis. Management of chalazion with warm compresses and/or topical or intralesional steroid/antibiotic.

3. Surgical options: Excision of eyelid and/or orbital lesions, transcutaneous levator advancement, transconjunctival levator advancement, frontalis muscle suspension, Fasanella-Servat procedure, or Muller muscle-conjunctival resection (Mullerectomy). The surgical approach depends on preoperative evaluation and the underlying etiology of ptosis.

Follow Up

1. Congenital: Close follow up is required to monitor for possible amblyopia (deprivation versus refractive secondary to induced corneal astigmatism), abnormal head positioning, and exposure keratopathy.

2. Traumatic: Observation for 6 months before considering surgical intervention. Many improve or completely resolve.

3. Neurologic: Reevaluate based on particular entity.

4. Postoperative (after ptosis repair):

 Acute: Monitor for infection and hemorrhage.

 Subacute: Monitor for exposure keratopathy and asymmetry that may require postoperative readjustment. Mild lagophthalmos is common for 2 to 3 weeks after surgical repair and usually resolves.

 Chronic: Monitor for ptosis recurrence and exposure keratopathy.

6.2 Chalazion/Hordeolum

Symptoms

Acute or chronic eyelid lump, swelling, and tenderness.

Signs

(See Figure 6.2.1.)

FIGURE 6.2.1 Chalazion.

Critical. Visible or palpable, well-defined, subcutaneous nodule in the eyelid. In some cases, a nodule cannot be identified.

Other. Blocked meibomian gland orifice, eyelid swelling and erythema, focal tenderness, associated blepharitis, or acne rosacea. May also note lesion coming to a head or draining mucopurulent material.

Definitions

Chalazion: Focal, tender, or nontender inflammation within the eyelid secondary to obstruction of a meibomian gland or gland of Zeis.

Hordeolum: Acute, tender infection; can be external (abscess of a glands of Zeis on eyelid margin) or internal (abscess of the meibomian gland). Usually involves Staphylococcus species and occasionally evolves into preseptal cellulitis.

Differential Diagnosis

 Preseptal cellulitis: Eyelid and periorbital erythema, edema, and warmth. See 6.10, Preseptal Cellulitis.

 Forniceal foreign body: eyelid swelling, particularly in soft contact lens wearers or those with a history of trauma. See 3.3, Corneal and Conjunctival Foreign Bodies.

 Sebaceous carcinoma: Suspect in older patients with recurrent chalazia, eyelid thickening, madarosis, or chronic unilateral blepharitis. See 6.11, Malignant Tumors of the Eyelid.

 Pyogenic granuloma: Benign, deep-red, pedunculated conjunctival lesion often associated with chalazia, hordeola, trauma, or surgery. May be excised or treated with a topical antibiotic-steroid combination such as neomycin/polymyxin B/dexamethasone q.i.d. for no more than 1 to 2 weeks. Intraocular pressure must be monitored if topical steroids are used.

Workup

1. History: Previous ocular surgery or trauma? Previous chalazia or eyelid lesions?

2. External examination: Palpate involved eyelid for a nodule. Look for rosacea.

3. Slit lamp examination: Evaluate meibomian glands for inspissation and evert the eyelid. Assess for madarosis, poliosis, and ulceration to rule out other etiologies.

Treatment

1. Warm compresses for at least 10 minutes q.i.d. with gentle massage over the lesion.

2. Consider a short course of a topical antibiotic for hordeolum (e.g., bacitracin, tobramycin, or erythromycin ointment b.i.d. for 1 to 2 weeks) or a short course of topical antibiotic/steroid for chalazion (e.g., neomycin/polymyxin B/dexamethasone ointment b.i.d. for 1 to 2 weeks). Consider chronic low-dose doxycycline 20 to 50 mg p.o. daily to b.i.d. for its antibacterial and antiinflammatory properties (e.g., for multiple or recurrent chalazia and/or ocular rosacea).

3. If a hordeolum worsens, consider incision and drainage and management as per preseptal cellulitis (see 6.10, Preseptal Cellulitis).

4. If the chalazion fails to resolve after 3 to 4 weeks of medical therapy and the patient desires surgical intervention, incision and curettage may be performed. Alternatively, an intralesional steroid injection may be performed (e.g., 0.2 to 1.0 mL of triamcinolone 40 mg/mL mixed 1:1 with 2% lidocaine with epinephrine). Alternate steroid formulations include various combinations of betamethasone sodium phosphate and betamethasone acetate 6 mg/mL or dexamethasone sodium phosphate 4 mg/mL. Total dosage depends on the lesion size. It is recommended that all chalazia, especially recurrent or atypical chalazia, be sent for pathology upon removal.

NOTE: A steroid injection can lead to permanent depigmentation or atrophy of the skin at the injection site, especially in dark-skinned individuals. Similarly, a vigorous injection can rarely result in retrograde intra-arterial infiltration with resultant central retinal artery occlusion. Because of these risks, some manufacturers of injectable steroids (e.g., triamcinolone and betamethasone) have historically recommended against their use intraocularly and in the periocular region. Off-label use of the medications should include a detailed discussion between physician and patient.

Follow Up

Patients are not routinely seen after instituting medical therapy unless the lesion persists beyond 3 to 4 weeks. Patients who have a procedure such as incision and curettage are usually reexamined as needed.

6.3 Ectropion

Symptoms

Tearing, irritation, redness, and mucous discharge. May be asymptomatic.

Signs

(See Figure 6.3.1.)

FIGURE 6.3.1 Ectropion.

Critical. Outward turning of the eyelid margin.

Other. Superficial punctate keratopathy (SPK) from corneal exposure; conjunctival injection and thickening and eventual keratinization from chronic dryness. Eyelid scarring may be seen in cicatricial cases. Facial hemiparesis and lagophthalmos may be seen in paralytic cases.

Etiology

 Involutional: Horizontal eyelid laxity related to aging. Most common.

 Paralytic: CN VII palsy.

 Cicatricial: Anterior lamellar shortening from burn injury, prior surgery or trauma, actinic damage, chronic inflammation, skin diseases (e.g., eczema and ichthyosis), and others.

 Mechanical: Due to herniated orbital fat, eyelid tumor, and others.

 Allergic: Contact dermatitis.

 Congenital: Facial dysmorphic syndromes (e.g., Treacher Collins syndrome), Down syndrome, or isolated abnormality.

Workup

1. History: Previous surgery, trauma, chemical burn, or CN VII palsy?

2. External examination: Check orbicularis oculi function and assess horizontal eyelid laxity and punctal location. Look for an eyelid tumor, scarring, herniated orbital fat, and so on. If there is concomitant CN VII palsy and CN VIII deficit (hearing loss), consider CT or MRI brain to rule out acoustic neuroma.

3. Slit lamp examination: Evaluate for exposure keratopathy and conjunctival inflammation.

Treatment

1. Treat exposure keratopathy with lubricating agents. See 4.5, Exposure Keratopathy.

2. Treat an inflamed, exposed eyelid margin with warm compresses and antibiotic ointment (e.g., bacitracin or erythromycin q.i.d.). A short course of combination antibiotic-steroid ointment (e.g., neomycin/polymyxin B/dexamethasone) may be helpful if close follow up is ensured.

3. Taping the eyelids into position may be a temporizing measure.

4. Definitive treatment usually requires surgery. Surgery is delayed for 3 to 6 months in patients with CN VII palsy because the ectropion may resolve spontaneously (see 10.9, Isolated Seventh Cranial Nerve Palsy). Corneal exposure may make the repair more urgent.

Follow Up

Patients with corneal or conjunctival exposure are examined as needed based on the severity of signs and symptoms. If the tissues are relatively healthy, follow up is not urgent. Patients using topical steroids need to be followed up routinely for steroid-induced side effects.

6.4 Entropion

Symptoms

Irritation, foreign-body sensation, tearing, and redness.

Signs

(See Figure 6.4.1.)

FIGURE 6.4.1 Entropion.

Critical. Inward turning of the eyelid margin that pushes otherwise normal lashes onto the globe.

Other. SPK from eyelashes contacting the cornea, conjunctival injection. Corneal epithelial defect, thinning, and/or ulceration in severe cases.

Etiology

 Involutional: Age-induced horizontal eyelid laxity, retractor disinsertion, and orbicularis override.

 Cicatricial: Due to conjunctival scarring in mucous membrane pemphigoid, Stevens-Johnson syndrome, chemical burns, trauma, trachoma, and others.

 Spastic: Sustained orbicularis contraction due to surgical trauma, ocular irritation, or blepharospasm.

 Congenital.

Workup

1. History: Previous surgery, trauma, chemical burn, or infection (trachoma, herpes simplex, varicella zoster)?

2. Slit lamp examination: Check for corneal involvement and conjunctival or eyelid scarring.

Treatment

If blepharospasm is present, see 6.7, Blepharospasm.

1. Aggressive lubrication and antibiotic ointment (e.g., erythromycin or bacitracin q.i.d.).

2. Everting the eyelid margin away from the globe and taping it in place with lateral traction may be a temporizing measure.

3. For spastic entropion, a Quickert suture placed at the bedside or in the office can temporarily resolve the eyelid malposition by tightening the lower eyelid retractors and rotating the eyelid margin anteriorly.

4. Surgery is often required for permanent correction.

Follow Up

If the cornea is uninvolved, the condition does not require urgent attention or follow up. If the cornea is significantly damaged, aggressive treatment is indicated (see 4.1, Superficial Punctate Keratopathy). Follow up is determined by the severity of corneal involvement.

6.5 Trichiasis

Symptoms

Irritation, foreign-body sensation, tearing, redness, and photophobia.

Signs

Critical. Misdirected eyelashes rubbing against the globe.

Other. Conjunctival injection; SPK; corneal epithelial defect, infiltrate, or scarring.

Etiology

 Entropion: Inward turning of eyelid pushing normal lashes onto the cornea. See 6.4, Entropion.

 Epiblepharon: Congenital or familial condition in which redundant lower eyelid anterior lamella redirects lashes into a vertical position, where they may contact the globe. Most common in Asian individuals, especially children.

 Distichiasis: An aberrant second row of lashes arising from meibomian gland orifices. Most commonly acquired in the setting of trauma or chronic inflammation (e.g., blepharitis, mucous membrane pemphigoid). Congenital distichiasis is a rare, sometimes hereditary, condition in which the meibomian glands are replaced by an extra row of eyelashes.

 Idiopathic.

 Chronic blepharitis: Inflamed eyelid margin. See 5.8, Blepharitis/Meibomitis.

 Cicatricial: Eyelid scarring from trauma, surgery, mucous membrane pemphigoid (see 5.10, Mucous Membrane Pemphigoid), trachoma, Stevens-Johnson syndrome, chemical and thermal burns, medications, and others.

 Medication-induced: systemic and topical (e.g., prostaglandin analogs).

Workup

1. History: Recurrent episodes? Prior severe systemic illness or allergic reaction? Prior trauma?

2. Slit lamp examination: Evert the eyelids and inspect the palpebral conjunctiva for scarring and symblepharon. Assess eyelid position for inward rotation statically and dynamically. Check the cornea for epithelial defects, infiltrates, and scarring.

Treatment

1. Remove the misdirected lashes.

 A few misdirected lashes: Perform epilation/removal at the slit lamp with fine forceps. Recurrence is common without follicular destruction.

 Diffuse, severe, or recurrent trichiasis: Definitive therapy usually requires electrolysis, cryotherapy, radiofrequency epilation, argon laser, or eyelid surgery.

2. Treat SPK with antibiotic ointment (e.g., erythromycin or bacitracin b.i.d. to q.i.d.).

3. Treat any underlying blepharitis. See 5.8, Blepharitis/Meibomitis.

4. Address eyelid malposition if present. See 6.4, Entropion.

Follow Up

As needed based on symptom severity and corneal integrity. Closer follow up is needed if there is evidence of SPK or corneal epithelial defect.

6.6 Floppy Eyelid Syndrome

Symptoms

Chronically red, irritated eye(s) with mild mucous discharge, often worse upon awakening due to eyelid eversion during sleep. Usually bilateral, but often asymmetric. Typically seen in obese patients due to the strong association with sleep apnea, with a slight male predilection.

Signs

Critical. Upper eyelids are easily everted without an accessory finger or cotton-tipped applicator exerting counterpressure.

Other. Rubbery, atrophic superior tarsal plate with conjunctival injection and chronic papillary conjunctivitis, SPK, ptosis with lash ptosis, and/or lower eyelid laxity. Associations include obstructive sleep apnea, obesity, keratoconus, and Down syndrome.

Differential Diagnosis

The key differentiating factor is increased horizontal laxity and spontaneous eversion of the upper eyelids.

 Vernal conjunctivitis: Seasonal, itching, and giant papillary reaction. See 5.2, Chronic Conjunctivitis.

 Giant papillary conjunctivitis: Often related to contact lens wear or an exposed suture. See 4.21, Contact Lens-Induced Giant Papillary Conjunctivitis.

 Superior limbic keratoconjunctivitis: Hyperemia, thickening, and inflammation of the superior bulbar conjunctiva, limbus, and cornea. Often associated with thyroid dysfunction, keratoconjunctivitis sicca, and rheumatoid arthritis. See 5.4, Superior Limbic Keratoconjunctivitis.

 Toxic keratoconjunctivitis: Papillae or follicles are typically more pronounced inferiorly in patients using eye drops. See 5.2, Chronic Conjunctivitis.

 Ectropion: Outward turning of the eyelid margin, often leading to tearing, irritation, and eyelid thickening. See 6.3, ECTROPION.

Etiology

The underlying etiology is not definitively known. Studies have suggested locally elevated matrix metalloproteinase (MMP) levels and elastin loss. Symptoms are thought to result from spontaneous eversion of the upper eyelid during sleep, allowing the superior palpebral conjunctiva to rub against pillows or sheets. Unilateral or asymmetric symptoms occur in those who tend to sleep prone on the affected side.

Workup

1. Pull the upper eyelid toward the patient’s forehead to determine if it spontaneously everts or is abnormally lax.

2. Conduct slit lamp examination of the cornea and conjunctiva with fluorescein staining, looking for superior palpebral conjunctival papillae and SPK.

3. Ask about history of snoring and obstructive sleep apnea.

Treatment

1. Topical antibiotic ointment for any mild corneal or conjunctival abnormality (e.g., erythromycin ointment b.i.d. to q.i.d.). May change to artificial tear ointment when lesions resolve.

2. The eyelids may be taped closed during sleep, or a shield may be worn to protect the eyelid from rubbing against the pillow or bed. Patients are asked to refrain from sleeping face down. Asking patients to sleep on their contralateral side may be therapeutic as well as diagnostic.

3. Surgical horizontal tightening of the eyelid with lateral tarsal strip or wedge resection is often required for definitive treatment.

Follow Up

1. Every few days to weeks initially, followed by weeks to months as the condition stabilizes.

2. Refer to an internist, otolaryngologist, or pulmonologist for evaluation and management of possible obstructive sleep apnea. Evaluation is important because of the systemic sequelae of untreated sleep apnea and for anesthesia risk assessment before eyelid surgery.

6.7 Blepharospasm

Symptoms

Uncontrolled blinking, twitching, or closure of the eyelids. Always bilateral, but may briefly be unilateral at the first onset. Occasionally may have mid to lower face and/or neck spasms. Can also be associated with oromandibular dystonia that results in spasms in the jaw and tongue, as well as laryngeal and cervical dystonia, a condition referred to as Meige syndrome.

Signs

Critical. Bilateral, episodic, and involuntary contractions of the orbicularis oculi muscles.

Other. Disappears during sleep.

Differential Diagnosis

 Hemifacial spasm: Unilateral contractures of the entire side of the face that do not disappear during sleep. Usually idiopathic but may be related to prior CN VII palsy, injury at the level of the brainstem, or compression of CN VII by a blood vessel or tumor. MRI of the cerebellopontine angle should be obtained in all patients to rule out tumors. Treatment options include observation, botulinum toxin injections, or neurosurgical decompression of CN VII.

 Ocular irritation induced blepharospasm (e.g., corneal or conjunctival foreign body, trichiasis, blepharitis, iritis, and dry eye).

 Eyelid myokymia: Subtle eyelid twitch felt by the patient but difficult to observe, commonly brought on by stress, caffeine, alcohol, or ocular irritation. Usually unilateral lower eyelid involvement. Typically self-limited. Treat by avoiding precipitating factors and/or administering small doses of botulinum toxin.

 Tourette syndrome: Multiple compulsive muscle spasms associated with utterances of bizarre sounds or obscenities.

 Tic douloureux (trigeminal neuralgia): Acute episodes of pain in the CN V distribution, often causing a wince or tic.

 Tardive dyskinesia: Orofacial dyskinesia, often with dystonic movements of the trunk and limbs, typically from long-term use of antipsychotic medications.

 Apraxia of eyelid opening. Usually associated with Parkinson disease. Unlike blepharospasm, apraxia of eyelid opening does not feature orbicularis spasm. Instead, apraxic patients simply cannot open their eyelids voluntarily.

Etiology

 Idiopathic and likely multifactorial, possibly involving dopaminergic pathways within the basal ganglia.

Workup

1. History: Unilateral or bilateral? Does the episode involve the eyelids alone or is the lower face also involved? Are limb muscles involved? Medications?

2. Slit lamp examination: Examination for dry eye, blepharitis, or foreign body.

3. Neuroophthalmic examination to rule out other accompanying abnormalities.

4. Typical blepharospasm does not require central nervous system imaging as part of the workup. MRI of the brain with attention to the posterior fossa and path of CN VII is reserved for atypical cases or other diagnoses (e.g., hemifacial spasm).

Treatment

1. Treat any underlying eye disorder causing ocular irritation. See 4.3, Dry Eye Syndrome and 5.8, Blepharitis/Meibomitis.

2. Consider botulinum toxin (onabotulinumtoxinA, incobotulinumtoxinA, and abobotulinumtoxinA) injections into the orbicularis muscles around the eyelids if the blepharospasm is severe. Can also be used to treat orofacial dyskinesia.

3. If the spasm is not relieved with botulinum toxin injections, consider surgical excision of the orbicularis muscle (myectomy) from the upper and lower eyelids and brow.

4. Muscle relaxants and sedatives are rarely of value but can be helpful in some patients. Oral medications such as lorazepam can help, but their use is often limited by their sedative qualities. Oral methylphenidate may be helpful in patients with severe spasm.

Follow Up

Not an urgent condition, but patients with severe blepharospasm can be functionally blind.

6.8 Canaliculitis

Symptoms

Tearing or discharge, red eye, and mild tenderness over the nasal aspect of the lower or upper eyelid.

Signs

(See Figure 6.8.1.)

FIGURE 6.8.1 Canaliculitis.

Critical. Erythematous “pouting” of the punctum and erythema of the surrounding skin. Expression of mucopurulent discharge or concretions from the punctum is diagnostic.

Other. Recurrent conjunctivitis confined to the nasal aspect of the eye, gritty sensation on probing of the canaliculus, and focal injection of the nasal conjunctiva.

Differential Diagnosis

 Dacryocystitis: Infection of the lacrimal sac with more lacrimal sac swelling, tenderness, and pain than canaliculitis. See 6.9, Dacryocystitis/Inflammation of the Lacrimal Sac.

 Chalazion: Focal inflammatory eyelid nodule without discharge from punctum. See 6.2, Chalazion/Hordeolum.

 Nasolacrimal duct obstruction: Tearing, minimal-to-no erythema or tenderness around the punctum. See 8.10, Congenital Nasolacrimal Duct Obstruction.

Etiology

 Actinomyces israelii (streptothrix): Most common. Gram-positive rod with fine, branching filaments. Produces sulfur-rich stones.

 Other bacteria (e.g., Fusobacterium and Nocardia species).

 Fungal (e.g., Candida, Fusarium, and Aspergillus species).

 Viral (e.g., herpes simplex and varicella zoster).

 Retained punctal plug or foreign body.

Workup

1. Apply gentle pressure over the lacrimal sac with a cotton-tipped swab and roll it toward the punctum while observing for mucopurulent discharge or concretions.

2. Smears and cultures of the material expressed from the punctum, including slides for Gram stain and Giemsa stain. Consider thioglycolate and Sabouraud cultures.

3. Ask about a history of punctal plug placement in the past.

Treatment

1. Remove obstructing concretions or retained plug. Concretions may be expressed through the punctum at the slit lamp. A canaliculotomy is usually required for complete removal or in the setting of a retained punctal plug. If necessary, marsupialize the horizontal canaliculus from a conjunctival approach and allow the incision to heal by secondary intention.

2. If concretions are removed, consider irrigating the canaliculus with an antibiotic solution (e.g., trimethoprim sulfate/polymyxin B, moxifloxacin, penicillin G solution 100,000 units/mL, iodine 1% solution). The patient is irrigated while in the upright position, so the solution drains out of the nose and not into the nasopharynx.

3. Treat the patient with antibiotic drops (e.g., trimethoprim sulfate/polymyxin B or moxifloxacin q.i.d.) and oral antibiotics for 1 to 2 weeks (e.g., doxycycline 100 mg b.i.d.).

4. If a fungus is found on smears and cultures, nystatin 1:20,000 drops t.i.d. and nystatin 1:20,000 solution irrigation several times per week may be effective.

5. Apply warm compresses to the punctal area for 5 to 10 minutes q.i.d.

Follow Up

Five to seven days depending on severity. This is usually not an urgent condition.

6.9 Dacryocystitis/Inflammation of the Lacrimal Sac

Symptoms

Pain, redness, and swelling over the lacrimal sac in the innermost aspect of the lower eyelid. Tearing, discharge, fever, or chills may also be present. Symptoms may be recurrent.

Signs

(See Figure 6.9.1.)

FIGURE 6.9.1 Dacryocystitis.

Critical. Erythematous, tender, tense swelling over the nasal aspect of the lower eyelid and extending around the periorbital area. Mucoid or purulent discharge can be expressed from the punctum when pressure is applied over the lacrimal sac.

NOTE: Swelling in dacryocystitis is below the medial canthal tendon. Suspect lacrimal sac tumor (rare) if the mass is above the medial canthal tendon.

Other. Fistula formation from the skin below the medial canthal tendon. A lacrimal sac cyst or mucocele can occur in chronic cases. Progression to a lacrimal sac abscess, and rarely, orbital or facial cellulitis may occur.

Differential Diagnosis

• Facial cellulitis involving the medial canthal area: No discharge from punctum with pressure over the lacrimal sac. The lacrimal drainage system is patent on irrigation. See 6.10, Preseptal Cellulitis.

 Dacryocystocele: Mild enlargement of noninflamed lacrimal sac in an infant. Present at birth, but may not be detected until later. Caused by nasolacrimal duct obstruction or entrapment of mucus or amniotic fluid in the lacrimal sac and usually unilateral. If bilateral, assess breathing to rule out nasal obstruction. Conservative therapy with digital massage, antibiotic ointment, and warm compresses is usually sufficient for nonobstructive cases.

 Acute ethmoid sinusitis: Pain, tenderness, nasal obstruction, and erythema over the nasal bone, just medial to the inner canthus. Patients may be febrile. Imaging is diagnostic.

 Frontal sinus mucocele/mucopyocele: Swelling typically occurs well above the medial canthal tendon. Proptosis, downward and lateral displacement of the globe, and external ophthalmoplegia are often present. Imaging is diagnostic.

Etiology

 Almost always related to nasolacrimal duct obstruction.

 Uncommon causes include lacrimal sac diverticula, dacryoliths, nasal or sinus surgery, trauma, and rarely lacrimal sac tumors.

 Gram-positive bacteria are the most common pathogens; gramnegative and atypical organisms are seen more commonly in diabetics, immunocompromised, and nursing home patients.

Workup

1. History: Distinguish reflex tearing from epiphora. Previous episodes? Concomitant ear, nose, or throat infection? Underlying sinus disease? Prior trauma or surgery?

2. External examination: Apply gentle pressure to the lacrimal sac in the nasal corner of the lower eyelid with a cotton-tipped swab in an attempt to express discharge from the punctum. Perform bilaterally to uncover subtle contralateral dacryocystitis.

3. Evaluation for orbital signs: Assess pupillary response, extraocular motility, globe position for proptosis, and other evidence of potentially concurrent orbital cellulitis.

4. Obtain Gram stain and blood agar culture (consider chocolate agar culture in children given the higher incidence of Haemophilus influenzae) of any discharge expressed from the punctum.

5. Consider a CT scan of the orbits and paranasal sinuses in atypical cases, severe cases, and those that do not respond to appropriate antibiotics.

NOTE: Do not attempt to probe or irrigate the lacrimal system during the acute infection. If a large abscess is present superficially, incision and drainage will alleviate pain and hasten healing.

Treatment

1. Systemic antibiotics in the following regimen: Children older than 5 years and <40 kg:

 Afebrile, systemically well, mild case, and reliable parent: Amoxicillin/clavulanate: 25 to 45 mg/kg/d p.o. in two divided doses for children, with a maximum daily dose of 90 mg/kg/d.

 Alternative treatment: Cefpodoxime: 10 mg/kg/d p.o. in two divided doses for children, with a maximum daily dose of 400 mg.

 Febrile, acutely ill, moderate-to-severe case, or unreliable parent: Hospitalize and treat with cefuroxime, 50 to 100 mg/kg/d i.v. in three divided doses in consultation with an infectious disease specialist.Adults:

 Afebrile, systemically well, mild case, and reliable patient: Cephalexin 500 mg p.o. q6h or amoxicillin/clavulanate 500/125 mg t.i.d. or 875/125 mg p.o. b.i.d.

 If exposure to methicillin-resistant Staphylococcus aureus (MRSA) is suspected, then start one to two tablets double-strength trimethoprim-sulfamethoxazole 160/800 mg p.o. q12h for adults. Alternatively, start clindamycin 300 mg p.o. t.i.d. In addition to covering MRSA, this antibiotic also gives good coverage for anaerobes, streptococci, and methicillin-sensitive S. aureus.

 Febrile, acutely ill, or unreliable: Hospitalize and treat with cefazolin 1 g i.v. q8h. See 7.3.1, Orbital Cellulitis.

 The antibiotic regimen is adjusted according to the clinical response and culture/sensitivity test results. I.V. antibiotics can be changed to comparable p.o. antibiotics depending on the rate of improvement, but systemic antibiotic therapy should be continued for at least a full 10- to 14-day course.

2. Topical antibiotic drops (e.g., trimethoprim/polymyxin B q.i.d.) may be used in addition to systemic therapy. Topical therapy alone is not adequate.

3. Apply warm compresses and gentle massage to the inner canthal region for 5 to 10 minutes q.i.d.

4. Administer pain medication (e.g., acetaminophen with or without codeine) p.r.n.

5. Consider incision and drainage of a pointing abscess.

6. Once infection has resolved, evaluate patency of the nasolacrimal duct system with probing and irrigation. If an obstruction is present, consider surgical correction (e.g., dacryocystorhinostomy with silicone intubation). In cases of recurrent or chronic dacryocystitis, surgical correction is recommended.

Follow Up

Daily, until improvement is confirmed. If outpatient condition worsens, hospitalization and i.v. antibiotics are recommended. Upon resolution of acute infection, probing and irrigation are required at the follow up to assess the patency of the nasolacrimal drainage system.

6.10 Preseptal Cellulitis

Symptoms

Tenderness, redness, warmth, and swelling of the eyelid and periorbital area. Often there is a history of local skin abrasions, penetrating injury/trauma, hordeolum, or insect bites. Can be associated with sinusitis, although this is more common with postseptal infections. May complain of fever or chills.

Signs

(See Figures 6.10.1 and 6.10.2.)

FIGURE 6.10.1 Preseptal cellulitis.

FIGURE 6.10.2 CT of preseptal cellulitis.

Critical. Eyelid erythema, tense edema, warmth, and tenderness. No proptosis, no optic neuropathy, no extraocular motility restriction, usually little to no conjunctival injection, and no pain with eye movement (unlike orbital cellulitis). The patient may not be able to open the eye because of eyelid edema. Visual changes such as blurred vision or monocular diplopia attributed to swollen eyelids.

Other. Tightness of eyelid skin or fluctuant eyelid edema. The eye itself may be slightly injected but is relatively uninvolved.

Differential Diagnosis

 Orbital cellulitis: Proptosis, pain with eye movement, restricted motility, and possible visual compromise. See 7.3.1, Orbital Cellulitis.

 Chalazion: Focal inflammatory eyelid nodule. See 6.2, Chalazion/Hordeolum.

 Allergic: Sudden onset, nontender, itchy, and swollen eyelids. See 5.11, Contact Dermatitis.

 Erysipelas: Rapidly advancing streptococcal cellulitis, often with a clear demarcation line, high fever, and chills.

 Necrotizing fasciitis: Severe bacterial infection involving subcutaneous soft tissue and deep fascia. Typically due to group A beta-hemolytic streptococci and S. aureus. Gray to purple skin discoloration with local hypesthesia is characteristic. Patients are often septic and may rapidly deteriorate. This potentially fatal condition requires emergent surgical debridement with i.v. antibiotic treatment.

 Viral conjunctivitis with secondary eyelid swelling: Follicular conjunctivitis is present. See 5.1, Acute Conjunctivitis.

 Cavernous sinus thrombosis: Proptosis with multiple cranial neuropathies. See 10.10, Cavernous Sinus and Associated Syndromes (Multiple Ocular Motor Nerve Palsies).

 Varicella zoster virus: Vesicular rash that respects midline. See 4.16, Herpes Zoster Ophthalmicus/Varicella Zoster Virus.

 Herpes simplex virus: Vesicular rash, typically unilateral, but does not respect midline/dermatomes. May be associated with ipsilateral follicular conjunctivitis. See 4.15, Herpes Simplex Virus.

 Other orbital disorders: Proptosis, globe displacement, or restricted ocular motility. See 7.1, Orbital Disease.

 Others: Insect bite, angioedema, trauma, maxillary osteomyelitis, and others.

Etiology

 Adjacent infection (e.g., hordeolum or dacryocystitis).

 Trauma (e.g., puncture wound, laceration, insect bite).

Organisms

S. aureus and Streptococcus are most common, but H. influenzae should be considered in nonimmunized children. Suspect anaerobes if foulsmelling discharge or necrosis is present or if there is a history of an animal or human bite. Consider a viral cause if preseptal cellulitis is associated with a vesicular skin rash (e.g., herpes simplex or varicella zoster).

Workup

1. History: Pain with eye movements? Double vision? Prior trauma, sinus surgery or disease, cancer, chalazia, insect bites, or hair epilation? Sinus congestion or purulent nasal discharge? Recent vaccination? Prior antibiotic-resistant infections?

2. Complete ocular examination: Evaluate for ocular motility restriction, proptosis, afferent pupillary defect, dyschromatopsia, and disc edema. An eyelid speculum or Desmarres eyelid retractor may facilitate ocular examination if the eyelids are excessively swollen.

3. Check facial sensation in the distribution of first and second divisions of the trigeminal nerve.

4. Palpate the periorbital area and the head and neck lymph nodes for a mass.

5. Check vital signs.

6. Obtain Gram stain and culture of any open wound or drainage.

7. Perform CT scan of the orbits and sinuses (axial and coronal views) with contrast if there is a history of significant trauma or a concern for orbital or intraocular foreign body, orbital cellulitis, subperiosteal abscess, paranasal sinusitis, cavernous sinus thrombosis, or malignancy. Consider CT scan of the brain if any relevant history, signs, or symptoms necessitate this. Consider MRI or angiographic imaging if cavernous sinus or intracranial pathology is suspected, or if radiology recommends based on CT findings.

8. Consider obtaining a complete blood count with differential to identify a leukocytosis and blood cultures in severe cases or when fever is present.

Treatment

1. Antibiotic therapy

a. Mild preseptal cellulitis, older than 5 years (<40 kg), afebrile, reliable patient/parent:

 Amoxicillin/clavulanate: 25 to 45 mg/kg/d p.o. in two divided doses for children, a maximum daily dose of 90 mg/kg/d; 875/125 mg p.o. q12h for adults.or

 Cefpodoxime: 10 mg/kg/d p.o. in two divided doses for children, a maximum daily dose of 400 mg; 200 mg p.o. q12h for adults.or

 Cefdinir: 14 mg/mg/d p.o. in two divided doses for children with a maximum daily dose of 600 mg; 600 mg p.o. once daily for adults. If the patient is allergic to penicillin, then:

 Trimethoprim/sulfamethoxazole: 8 to 12 mg/kg/d trimethoprim with 40 to 60 mg/kg/d sulfamethoxazole p.o. in two divided doses for children; 160 to 320 mg trimethoprim with 800 to 1600 mg sulfamethoxazole (one to two double-strength tablets) p.o. b.i.d. for adults.or

 Moxifloxacin 400 mg p.o. daily (contraindicated in children). If exposure to MRSA is suspected, then:

 Trimethoprim/sulfamethoxazole: 8 to 12 mg/kg/d trimethoprim with 40 to 60 mg/kg/d sulfamethoxazole p.o. in two divided doses for children; one to two tablets double-strength trimethoprim-sulfamethoxazole 160/800 mg p.o. q12h for adults.or

 Doxycycline: 100 mg p.o. b.i.d. (contraindicated in children, pregnant women, and nursing mothers).or

 Clindamycin: 10 to 30 mg/kg/d p.o. in three to four divided doses for children; 450 mg p.o. t.i.d. for adults. In addition to covering MRSA, this antibiotic also gives good coverage for streptococci and methicillin-sensitive S. aureus.

NOTE: Patients with the following risk factors should be covered for MRSA: history of MRSA infection or colonization, recurrent skin infections, contact with someone known to have MRSA, admission to a healthcare or long-term care facility within the past year, placement of a permanent indwelling catheter, on hemodialysis, i.v. drug use, incarceration within the past 12 months, participation in sports that include skin-to-skin contact or sharing of equipment and clothing, and poor personal hygiene.

NOTE: Oral antibiotics are maintained for 10 to 14 days.

2. Moderate-to-severe preseptal cellulitis or any one of the following:

 Patient appears toxic.

 Patient may be noncompliant with outpatient treatment and follow up.

 Child 5 years or younger.

 No noticeable improvement or worsening after 24 to 48 hours of oral antibiotics.Admit to the hospital for i.v. antibiotics as follows:

 Vancomycin: 10 to 15 mg/kg i.v. every 6 hours in children; 0.5 to 1 g i.v. q12h for adults. (Dose adjustment is needed in cases of impaired renal function.) Plus one of the following:

 Ampicillin/sulbactam: 300 mg/kg/d i.v. in four divided doses for children; 3.0 g i.v. q6h for adults.

 Piperacillin-tazobactam: 240 mg/kg/d i.v. in three divided doses for children; 4.5 g i.v. q6h adults.

 Ceftriaxone: 80 to 100 mg/kg/d i.v. in two divided doses for children with a maximum of 4 g/d; 2 g i.v. q12h for adults.

 Cefepime: 50 mg/kg q12h (max 2 g/dose) in children; 1 to 2 g i.v. q12h in adults.

 Ceftazidime: 90 to 150 mg/kg/day i.v. in three divided doses for children; 1 g i.v. q8-12h in adults.If the patient is allergic to penicillin, see 7.3.1, Orbital Cellulitis, for alternatives.

NOTE: Intravenous antibiotics can be changed to comparable oral antibiotics after significant improvement is observed. Systemic antibiotics are maintained for a complete 10- to 14-day course. See 7.3.1, Orbital Cellulitis, for alternative treatment. In complicated cases or patients with multiple allergies, consider consultation with an infectious disease specialist for antibiotic management.

3. Warm compresses to the inflamed area t.i.d. p.r.n.

4. Polymyxin B/bacitracin ointment to the eye q.i.d. if secondary conjunctivitis is present.

5. Tetanus toxoid if needed. (See Appendix 2, Tetanus Prophylaxis).

6. Nasal decongestants if sinusitis is present.

7. Orbital exploration and debridement are warranted if a fluctuant mass or abscess is present, or if a retained foreign body is suspected. Obtain Gram stain and culture of any drainage to guide antibiotic coverage. Avoid the orbital septum if possible. A drain may need to be placed.

8. Consider an infectious disease consult for patients who have failed oral antibiotics and require i.v. treatment.

9. Consider systemic steroids. As with orbital cellulitis, preseptal cellulitis will improve with proper antibiotic treatment of bacteria and surgical treatment of any nidus. In some cases, the inflammatory cascade caused by the infection is significant and may be slow to resolve. This may improve more quickly with a short course of steroids during or after antibiotic treatment for the infection. Steroid dosing and timing of initiation vary among clinicians. Physicians may start steroids simultaneously with antibiotics, after 24 to 48 hours of antibiotic coverage, or not at all.

Follow Up

Daily until clear and consistent improvement is demonstrated, then every 2 to 7 days until the condition has totally resolved. If preseptal cellulitis progresses despite antibiotic therapy, the patient is admitted to the hospital and a repeat (or initial) orbital CT scan is obtained. For patients already on p.o. antibiotics, switch to i.v. antibiotics (see 7.3.1, Orbital Cellulitis).

6.11 Malignant Tumors of the Eyelid

Symptoms

Asymptomatic or mildly irritating eyelid lump.

Signs

Skin ulceration and inflammation with distortion of normal eyelid anatomy. Abnormal color, texture, or persistent bleeding. Loss of eyelashes (madarosis) or whitening of eyelashes (poliosis) over the lesion. Sentinel vessels may be seen. Diplopia or external ophthalmoplegia are ominous signs of a possible orbital invasion.

Differential Diagnosis of Benign Eyelid Tumors

 Seborrheic keratosis: Middle-aged or elderly patients. Brownblack, well-circumscribed, crust-like lesion, usually slightly elevated, with or without surrounding inflammation. May be removed by shave biopsy.

 Chalazion/hordeolum: Acute, erythematous, tender, well- circumscribed lesion. See 6.2, Chalazion/Hordeolum.

 Cysts: Well-circumscribed white, yellow, or clear/skin-colored lesions on the eyelid margin or underneath the skin. Epidermal inclusion cysts, sebaceous cysts, and eccrine or apocrine hidrocystomas may be excised.

 Molluscum contagiosum: A viral infection of the epidermis, typically seen in children. Multiple small papules characterized by central umbilication. Can be severe in HIV-positive patients. May produce a chronic follicular conjunctivitis. Treatment is by unroofing and curettage. While some recommend curettage until bleeding occurs, there is no clear evidence that this is more effective. A variety of other therapies (e.g., cryotherapy, cautery, chemical peel, and laser) are used for lesions found elsewhere on the body but are typically not necessary for periocular regions and may cause injury to the ocular surface. See 5.2, Chronic Conjunctivitis.

 Nevus: Benign melanocytic neoplasm, which may involve the dermis, the dermis-epidermis junction, or both. Congenital nevi may be present at, or shortly after, birth; however, most nevi appear during childhood and may enlarge during puberty. Nevi are well-circumscribed lesions, usually round or oval, with uniform pigmentation. Melanomas may develop within preexisting nevi and may manifest as a changing pigmented lesion and/or as a pigmented lesion with asymmetry of shape, irregular borders, color variegation, and possibly pruritus or bleeding. Suspicious lesions should be biopsied, preferably with the removal of the entire lesion to the subcutaneous fat.

 Xanthelasma: Multiple, often bilateral, soft yellow plaques of lipid-laden macrophages in the medial upper and sometimes lower eyelids. Patients 40 years and younger should have a serum cholesterol and lipid profile evaluation to rule out hypercholesterolemia. Surgical excision can be performed for cosmesis; however, recurrence is possible.

 Squamous papilloma: Soft, skin-colored lesions that may be smooth, rounded, or pedunculated. May enlarge or multiply over time. Often spontaneously regress. Occasionally squamous carcinomas can appear papillomatous. Therefore, excisional biopsy should be performed for suspicious lesions.

 Actinic keratosis: Round, erythematous, premalignant lesion with a scaly surface. Found in sun-exposed areas of skin. Treated by excisional biopsy.

 Inflammatory conditions: Blepharitis and blepharoconjunctivitis.

 Allergic conditions: Chronic contact dermatitis can appear unilateral, cause cilia loss, and simulate malignancy.

 Others: Verrucae from human papillomavirus, benign tumors of hair follicles, or sweat glands (e.g., syringoma), inverted follicular keratosis, neurofibroma, neurilemmoma, capillary hemangioma, cavernous hemangioma, and pseudoepitheliomatous hyperplasia. Necrobiotic xanthogranuloma nodules of multiple myeloma appear as yellow plaques or nodules that are often mistaken for xanthelasma.

Etiology

FIGURE 6.11.1 Basal cell carcinoma.

FIGURE 6.11.2 Sebaceous carcinoma.

 Basal cell carcinoma: Most common malignant eyelid tumor, usually on the lower eyelid or medial canthus of middle-aged or elderly patients. Rarely metastasizes, but may be locally invasive, particularly when present in the medial canthal region. There are two major forms:

 Nodular: Indurated, firm mass, commonly with telangiectasias over tumor margins. Sometimes the lesion center is ulcerated (see Figure 6.11.1). On rare occasions, a cystic variant is seen.

 Infiltrative: The classic variant of this form is the morpheaform lesion. A firm, flat, subcutaneous lesion with indistinct borders. More difficult to excise and may result in a large eyelid defect.

 Squamous cell carcinoma: Variable presentation, often appearing similar to basal cell carcinoma. Regional metastasis may occur and can be extensive with the propensity for perineural invasion. A premalignant lesion, actinic keratosis, may appear either as a scaly, erythematous flat lesion or as a cutaneous horn.

 Keratoacanthoma: This lesion was previously considered to be benign and self-limiting; however, it is now regarded as a low- grade squamous cell carcinoma. Clinically may resemble basal and squamous cell carcinomas. Typically, the lesion is elevated with rolled margins and a large central ulcer filled with keratin. Rapid growth with slow regression and even spontaneous resolution has been observed. Lesions usually involve the lower eyelid and can be destructive. Complete excision is recommended.

 Sebaceous carcinoma: More common in middle-aged or elderly patients, usually elderly women. Most common on the upper eyelid but may be multifocal, involving both the upper and the lower eyelids. Often confused with recurrent chalazia or intractable blepharitis. Loss of eyelashes and destruction of the meibomian gland orifices in the region of the tumor may occur. Regional and systemic metastasis or orbital extension is possible. Can occur many decades after prior radiation exposure to the eyelids (see Figure 6.11.2).

 Others: Malignant melanoma, lymphoma, sweat gland carcinoma, metastasis (usually breast or lung), Merkel cell tumor, Kaposi sarcoma, and others.

Workup

1. History: Duration? Rapid or slow growth? Previous malignant skin lesion or other malignancies? Previous treatment of inflammatory or allergic condition? Previous radiation therapy? Associated pain?

2. External examination: Check the skin for additional lesions, palpate the preauricular, submaxillary, and cervical nodes to evaluate for metastasis.

3. Slit lamp examination: Look for telangiectasias on nodular tumors, loss of eyelashes in the region of the tumor, and meibomian orifice destruction. Evert eyelids of all patients with eyelid complaints.

4. Photograph or draw the lesion and its location for documentation.

5. Perform a biopsy of the lesion. An incisional biopsy is commonly performed when a malignancy is suspected. Depending on the depth of invasion, sentinel node biopsy may be indicated with melanoma and sebaceous carcinoma. Histopathologic confirmation must precede any extensive procedures.

6. Sebaceous carcinoma may be difficult to diagnose histopathologically. In the past, fresh tissue with oil red-O staining was recommended. This is no longer necessary if the pathologist is experienced with this malignancy.

7. For lymphoma, the pathologist would prefer the tissue to be sent fresh for flow cytometry. Contact the pathologist first. If confirmed, a systemic workup is indicated. See 7.4.2, Orbital Tumors in Adults.

Treatment

1. Basal cell carcinoma: Surgical excision with histologic evaluation of tumor margins either by frozen sections or by Mohs techniques. The entire tumor should be excised with clean margins. Cryotherapy and radiation are used rarely. Topical imiquimod, an immune modulator, might be beneficial but could be toxic to the ocular surface. Unresectable tumors can be treated by the oral Hedgehog pathway inhibitor vismodegib, although it is expensive and can cause systemic side effects. Patients are informed about the etiologic role of the sun and are advised to avoid unnecessary sunlight exposure and to use protective sunscreens.

2. Squamous cell carcinoma: Surgical excision is considered as first- line treatment. Radiation therapy is the second-best treatment after surgical excision. Topical imiquimod and topical or injectable interferon can be beneficial for elderly patients who are not surgical candidates. Patients are informed about the etiologic role of the sun. Referral to an oncologist or internist for regional and/or systemic workup and surveillance is important.

3. Sebaceous carcinoma: The approach is two-staged with stage 1 using map biopsies on the entire surface of the eye to ascertain the extent of Pagetoid spread or deep tumor. Stage 2 is performed after all biopsies are reviewed; Pagetoid spread is treated with cryotherapy whereas deep tumor requires excision. Reconstruction is then provided. Close follow up of regional nodes is indicated. Exenteration is often required when orbital invasion is present. Referral to an oncologist or internist for systemic workup and surveillance is important with attention to the lymph nodes, lungs, brain, liver, and bone.

4. Malignant melanoma: Treatment requires wide surgical excision. The margins must be free of tumor and require permanent sections. Sentinel lymph node biopsy may be required depending on tumor depth. Referral to an oncologist or internist for regional and/or systemic workup and surveillance is imperative.

NOTE: Because both melanoma and sebaceous carcinoma are difficult to diagnose by frozen section, multiple excisions utilizing permanent sectioning may be necessary until all surgical margins are free of tumor. The cornea and globe must be protected during this interim time with lubrication or temporary tarsorrhaphy.

Follow Up

Initial follow up is every 1 to 4 weeks to ensure proper healing of the surgical site. Patients are then reevaluated every 6 to 12 months, or more frequently, for more aggressive lesions. Patients who have had one skin malignancy are at greater risk for additional malignancies.