Diseases of the Lacrimal System + Miscellaneous

What are the causes of epiphora?

• Hypersecretion

  • Ocular surface irritation or inflammation

  • Infectious conjunctivitis

  • Entropion

  • Trichiasis

• Defective drainage

  • Malposition

  • Obstruction

  • Lacrimal pump failure

• Evaluate the tear meniscus height prior to eyelid manipulation or instillation of medication

• Patients with c/o excessive tearing = high meniscus

• Normally, little to no dye remains after 5 minutes

  • Prolonged retention is indicative of inadequate lacrimal drainage and can be graded from 1-4

Fluorescein disappearance test

Indicated in patients with suspected partial obstruction of the drainage system

Jones dye testing

If dye is present on Jones I is that a positive or negative test?

Positive

Identifies the probable site of partial obstruction

Jones II

If fluorescein dye is passed during Jones II, is that positive or negative?

Positive

• Injection of radio-opaque contrast medium into the canaliculi followed by capture of magnified images.

• Indications: confirm site of lacrimal drainage obstruction

• Technique:

  • Inferior puncta dilated

  • Plastic catheters inserted into inferior canaliculi

  • Contrast medium injected

  • Radiographs taken

• Interpretation

  • Failure of dye reaching nose = anatomical obstruction

Contrast dacryocystography

Inflammation of the lacrimal gland

• Acute

• Chronic

• Infectious or inflammatory

• Pathophysiology not completely understood

Dacryoadenitis

Causes of viral infectious acute dacryoadenitis…

• Mumps (common previously, especially in childhood)

• Epstein-Barr virus

• Herpes zoster

• Mononucleosis

• Cytomegalovirus

• Echoviruses

• Coxsackievirus A

• May result in severe dry eye due to lacrimal gland fibrosis

Causes of bacterial, fungal, and parasitic infectious acute dacryoadenitis…

Bacterial (less common)

• Staphylococcus aureus and Streptococcus

• Neisseria gonorrhoeae

• Treponema pallidum

• Chlamydia trachomatis

• Mycobacterium leprae

• Mycobacterium tuberculosis

• Haemophilus

• Moraxella

• Pseudomonas

Fungal (rare)

• Histoplasmosis

• Blastomycosis

Parasitic (rare)

• Schistosoma haematobium

Acute dacryoadenitis symptoms

• Unilateral pain

• Redness

• Swelling (outer 1/3 of upper lid)

• Tearing

• Discharge

Acute dacryoadenitis signs

• Erythema

• Swelling

• Tenderness

• Hyperemia of palpebral lobe of lacrimal gland

  • Prolapsed and enlarged

• S-shaped upper lid

• Fever — need CBC

• Elevated WBC

• Conjunctival injection and chemosis

• Lymphadenopathy

• Proptosis

• EOM restriction

• Parotid gland enlargement

• Upper respiratory infection

• Malaise

Chronic dacryoadenitis etiology (inflammatory)

• Sarcoidosis

• Wegener’s granulomatosis

• Graves’ disease

• Sjogren’s syndrome

• Orbital pseudotumor

• Benign lympho-epithelial lesion

Chronic dacryoadenitis clinical manifestations…

• Can be bilateral

• Painless enlargement of the lacrimal gland present for more than a month

• More common than acute

• Less severe presentation than acute

• Enlarged gland but motile

• Minimal ocular signs

• Mild ptosis

• Mild to severe dryness

How do we manage dacryoadenitis?

• CT scan for both acute and chronic

  • Should see enlargement of the gland, but NO changes to the bone or globe

    • Suspect tumor if there is

• Viral (most common): self limiting

  • Cool compress

  • NSAID

• Bacterial or infectious:

  • Keflex

    • Adults: 250-500mg PO qid 10-14 days

    • Children: 25-50mg/kg/day PO qid

  • Augmentin

    • Adults: 250-875mg bid 5-7 days

    • Children: 20mg/kg/day to 40mg/kg/day for no more than 10 days

• Inflammatory: investigate

  • Consider biopsy

• Narrowing, occlusion, closure of the punctum

• Occurs in the absence of punctal eversion

Primary punctal stenosis

Causes of primary punctal stenosis…

• Chronic blepharitis

• Idiopathic

• HSV HZO

How do we treat primary punctal stenosis?

• Dilation

• Punctoplasty

Causes of secondary punctal stenosis…

• Punctal eversion

Treatment of secondary punctal stenosis…

• Ziegler cautery

• Medial conjunctivoplasty

• Lower lid tightening

What are the causes of canalicular obstruction?

• Congenital

• Trauma

• Herpes simplex infection

• Drugs

• Irradiation

• Chronic dacryocystitis

How do we treat canalicular obstruction?

• Intubation with stents (3-6 months)

What causes canaliculitis?

Bacteria, fungi, or viruses (actinomyces israelii bacteria)

• Unilateral epiphora

• Redness

• Blurry vision

• Discomfort

• Swollen punctum

• Mucopurulent conjunctivitis

Canaliculitis

What are the signs of canaliculitis?

• Pounting punctum

• Edema of the canaliculitis

• Discharge on pressure

• Concretions

How do we treat canaliculitis?

• NEVER D&I

• Antibiotics (oral)

  • Keflex 250-500mg qid 10-14 days

  • Augmenting 250-875 bid 5-7 days

• Topical steroids

  • Lotemax qid

• Canaliculotomy

  • Linear incision into the conjunctival side of the canaliculitis and curetting of the concretions

• Stenting

Infection or inflammation of the lacrimal sac

• Usually secondary to obstruction

Dacryocyctitis

• Frequent complication of congenital dacryostenosis

• Subacute (mild to moderate tenderness on palpation)

• Chronic (painless) presentation for weeks to months

• May be unilateral or bilateral

Congenital Dacryocystitis

• Unilateral

• Acute onset with moderate to severe pain

• Epiphora

• Tenderness

• Diffuse pain around eye and orbit (headache)

• May present with pain only on firm palpation of inner canthus

Acquired Dacryocystitis

Signs of acute dacryocystitis

• Moderate swelling of sac

• Mild to moderate localized edema and erythema throughout inferior nasal region

• Purulent discharge

• Secondary conjunctivitis

• In severe cases, a secondary preseptal cellulitis

• Almost always epiphora

Signs of chronic dacryocystitis

• Less severe

• More common congenital presentation

• Purulent discharge

• May persist up to 9-12 months in congenital form with spontaneous remission (opening of the valve of Hasner)

What workup should you do for dacryocystitis?

• Problem oriented questions:

  • Previous episodes?

  • Concomitant ear, nose, or throat infection?

• Evaluation

  • Gentle compression of the lacrimal sac

  • Extraocular motility

  • Check for proptosis

  • Gran’s stain and blood agar culture

  • Consider CT scan

How do we treat dacryocystitis?

• Systemic antibiotic

  • Children: afebrile, mild case, systemically well

    • Amoxicillin clavulanate (Augmentin) 20-40mg/kg/day po

    • Cephalexin (Keflex) 25-50mg/kg/day

    • Cefaclor (Ceclor) 20-40 mg/kg/day po

  • Children: febrile or acutely ill, moderate-severe case, or unreliable parent

    • Hospitalize and treat

  • Adult: afebrile, systemically well, mild case, reliable patient

    • Cephalexin (Keflex) 500mg po qid

    • Amoxicillin clavulanate (Augmentin) 250-875mg po bid

  • Febrile or acutely ill

    • Hospitalize and treat

• Topical antibiotic drops

• Warm compresses and gentle massage (Crigler massage) qid

• Pain med (acetaminophen) PRN

• Consider surgical correction: DCR

• Follow-up every 3-5 days

Chronic, untreated dacryocystitis has inflamed the lacrimal sac to the point of perforation

Lacrimal fistula

What are the causes of nasolacrimal duct obstruction?

• Acquired or congenital

• Idiopathic stenosis

• Naso-orbital trauma

• Previous nasal and sinus surgery

• Infiltration by nasopharyngeal tumors

  • Granulomatous disease

How do we treat nasolacrimal duct obstruction?

• Dacryocystorhinostomy

• Intubation

• Stent insertion

• Balloon dilation

How do we treat congenital nasolacrimal duct obstruction?

Often resolves on its own

• Massage

• Lid hygiene

• Topical antibiotics

• Probing and/or stenting

Signs of congenital nasolacrimal duct obstruction…

• Epiphora and matting of lashes

• Gentle pressure over the lacrimal sac causes reflux of purulent material

• Uncommon

• Delayed diagnosis

• Common cause of lacrimal sac mass

• Clinical presentation

  • Epiphora

  • Dacryocystitis

  • Epistaxis (nosebleed)

  • Mass

Nasolacrimal tumor

• A number of histologies are encountered including:

  • Squamous cell papilloma

  • Squamous cell carcinoma (most common)

  • Transitional cell papilloma

  • Transitional cell carcinoma

  • Oncocytoma

  • Adenocarcinoma

  • Adenoid cystic carcinoma

  • Malignant melanoma

• Biopsy, CT and MRI scans

• Treatment — surgery, chemotherapy, immunotherapy

Nasolacrimal tumor

• Most common epithelial tumor of the lacrimal gland

• Progressive, painless, inferior and medial displacement of the globe >12 months

• Proptosis

• Firm lobular mass located near the superior orbital rim

• Treatment = removal

Pleomorphic adenoma of the lacrimal gland

• Most common malignant epithelial tumor of the lacrimal gland

• Pain

  • Perineural invasion and bond destruction

• Rapid course (<1 year)

• Extends to posterior orbit

Adenoid cystic carcinoma (ACC) of the lacrimal gland

• Nonepithelial tumor

• Usually arise in the maxillary sinus, followed by the nasopharynx

• Nasal obstruction or epiphora may be associated with tumor growth

• Characterized by proliferation of keratinizing malignant squamous cells

• Treatment = surgical excision and radiation

Primary squamous cell carcinoma and adenocarcinoma of the lacrimal gland

• Type of non-Hodgkin lymphoma

• Originates in the conjunctiva, lacrimal gland, soft tissues of the eyelids or EOMs

• Primary or secondary

• Constitutes 55% of all malignancies

• Unilateral or bilateral

Orbital lymphoma

• Vision usually preserved

• Conjunctiva: pink or red batch of swollen conjunctiva or conjunctival hyperemia (often feeder vessels)

• Orbit: painless mass in ST quadrant, proptosis, diplopia, abnormal EOMs

• LG: displaces the eye IN

• Eyelids: swelling and prolapse

Orbital lymphoma

How do you treat orbital lymphoma?

• Imaging

• Molding of tumor to normal structures

• Surgery, radiotherapy, chemotherapy, immunotherapy

• Rare

• Associated with epiphora or chronic dacryocystitis

• Spontaneous bleeding from punctum

• Tumors that invade the skin may produce ulceration with telangiectasia over the lacrimal sac

• Metastasis to reginal lymph nodes may occur

• CT and MRI

• Treatment = dacryocystectomy

Primary lacrimal sac tumor

• Adjacent to limbus— nasal & temporal 

• UV exposure 

• Epithelium may be normal, thin or thick 

• Yellow-white in color 

• Grow over time 

• Recurrent inflammation/irritation common 

• Treatment = ocular lubrication 

Pinguecula

• Wing shaped fibrovascular growth of conjunctiva involving the superficial cornea

• Caused by UV exposure, environmental insults, dryness

• Typically preceded by pinguecula

• Prevalence increases with proximity to the equator

• Irregular astigmatism with increasing size

• Measure size/topography

• Stocker line - iron line at leading edge

• Treatment - lubrication, excision is VA decreased

Pterygium

• Asymptomatic

• Congenital or acquired

  • Chronic inflammation

  • Trauma

  • Surgery

• Derived from inclusion of conjunctival epithelium in the substantia propria

• Central cavity filled with clear fluid and lined with nonkeratinized conjunctiva epithelium

• Bulbar conjunctiva or fornix

• Treatment = drain or excise

• Need to differentiate from dilated lymphatic channels

Inclusion cyst of the epithelium

• "bubble" on the eye

• "string of pearls"

• Dilated conjunctival lymphatic channel

• Unknown etiology

• Benign

• Possible connection with Fabry's Disease

Conjunctival lympangiectasis

• Chronic, recurrent inflammation involving the superior bulbar and tarsal conjunctiva as well as superior limbus and cornea

• Associated with TED

• Mechanical trauma from upper lid

• Clinical signs:

  • Papillae

  • Injection and edema of superior conjunctiva

  • (+) NaFl and LG staining

  • Hypertrophy of superior limbus

  • Blepharospasm

  • Superior corneal filaments

Superior Limbic Keratoconjunctivitis

How do you treat SLK?

• Lubrication

• Punctal plugs

• Topical cyclosporin, steroids

• Autologous serum

• BCL

• Co-manage

• Pedunculated - fleshy growth with fibrovascular core

• Sessile - found near limbus and has a flat base

  • Glistening surface with red dots

• Benign

• When caused by HPV may be carcinomatous

Conjunctival papilloma

How do you treat a conjunctival papilloma?

• Regress spontaneously

• Observe

• Excision and cryotherapy

• Dysplasia

• Premalignant that is at risk for becoming squamous cell carcinoma

• Analogous to actinic keratosis of the skin

• Does not invade underlying basement membrane

• Develops on exposed area of bulbar conjunctiva near the limbus

• Older males, light complexion

• Smokers

• Three variants

• Mild inflammation and various forms of abnormal vascularization

• Large feeder vessels - invasion to BM

• Slow growing, rapid in AIDS patient

• Can invade cornea

• Stains with LG

• Recurrent

Conjunctival Intraepithelial Neoplasia (CIN)

What is the most common conjunctival malignancy in the US?

Conjunctival Intraepithelial Neoplasia (CIN)

How do we treat conjunctival intraepithelial neoplasia (CIN)

• Excision including 3-4mm of surrounding tissue

• Chemotherapeutic agent

• Cornea involvement with minimal conjunctival involvement

• Granular, translucent, gray epithelial sheet at the limbus extending to the cornea

• Stains with LG

• No corneal neo

Corneal intraepithelial neoplasia

How do you treat corneal intraepithelial neoplasia?

• Apply absolute alcohol to the cornea for 30-40 seconds, then irrigate and debride

• Cryotherapy

• Surgical excision

• Plaque-like, gelatinous or papilliform growth

• Limbal and bulbar conjunctiva

• UV radiation, viral and genetic factors

• Compromised immunity, xeroderma pigmentosa

• Grows outward with sharp borders

• Invades through basement membrane

• Engorged conjunctival vessel feeds the tumor

• Treatment = excision followed by cryotherapy

Squamous cell carcinoma

• Benign

• Freckle

• Bulbar conjunctiva near limbus

• Present at early age

• Management: monitor

Congenital epithelial melanosis

• Increasing pigmentation of the conjunctiva

• Can extend on the cornea (striate melanokerotosis)

• Bilateral

• Sunlight exposure

• Management: monitor

Benign melanosis (racial, complexion associated)

• 1 in every 2500

• More common in Black, Hispanic,

• Asian populations

• Focal proliferation of subepithelial melanocytes (blue nevus)

• Patches of episcleral pigmentation appear slate gray

• Unilateral

• Immobile

• Management: Monitor

• 50% have ipsilateral dermal melanocytosis of periorbital skin

  • Nevus of Ota

  • Increased risk of secondary glaucoma

  • Increased risk of uveal melanoma

Ocular Melanocytosis

• 1st or 2nd decade of life

• Flat

• Tan, light brown or amelanotic

• Epithelial inclusion cysts may occur

• Secretions of mucin may cause nevus to enlarge

• Management: monitor 6-12 months

  • Biopsy if change

Conjunctival nevus

• Acquired pigmentation — hyperplasia of melanocytes

• Unilateral

• Light-skinned individuals

• Flat, brown lesion

• Changes in size may be associated with inflammation

• Pre-cancerous (rare)

  • Larger size

  • Caruncle, fornix or palpebral

  • Progressive enlargement

  • Nodular component

  • Thickening

  • Feeder vessel

• Management: monitor 8-12 months

  • Biopsy

Primary acquired melanosis (PAM)

• 2% of ocular malignancy

• European ancestry

• Arise from PAM, nevi or de novo

• Mortality rate = 25%

• 25% are amelanotic

• High recurrence rate

• Heavy vascularization

• Nodular

• Invade the globe or orbit

• May metastasize to regional lymph nodes, brain, lungs, liver and bone

Melanoma

How do we treat melanoma?

• Biopsy

• Excision

• Cryotherapy

• Orbital exteneration

• Enucleation

Abnormal increased distance between the eyes

Hypertelorism

Abnormal decreased distance between the eyes

Hypotelorism

• Remove the contents of the globe, leaving the scleral shell

• Better preservation of the anatomy

• Better motility for prosthetic

• CONTRAINDICATED in intraocular malignancy

Evisceration

• Removal of the eyeball (globe) leaving orbital content (fat & musculature) behind

• Allows for histologic examination of intact globe and optic nerve

• Indications

  • Intra ocular malignancy

  • Severe trauma

  • Blind, painful eye

Enucleation

• Removal of the entire globe and ALL orbital contents

• Indications

  • Orbital malignancy

  • Life-threatening infection

Exteneration