Diseases of the lacrimal apparatus

Dacryostenosis is a congenital or acquired obstruction of the nasolacrimal duct and manifests with epiphora. It is usually diagnosed clinically and confirmed with the fluorescein dye disappearance test. Congenital dacryostenosis is common, typically manifests in neonates, and usually resolves spontaneously within the first year of life; lacrimal sac massage is recommended to promote resolution. Acquired dacryostenosis may be idiopathic or secondary to conditions affecting the nasolacrimal duct (e.g., trauma, inflammation). Treatment includes management of the underlying cause and surgical intervention in selected cases. Dacryostenosis often causes dacryocystitis (inflammation of the lacrimal sac). Dacryocystitis may be acute or chronic and manifests with local erythema, edema, pain, epiphora, and mucopurulent discharge. Dacryoadenitis (inflammation of the lacrimal gland) is commonly idiopathic but can also be caused by infection or autoimmune or inflammatory diseases. It may be acute or chronic, unilateral or bilateral, and manifests with circumscribed swelling and erythema of the lateral upper eyelid. Features may also include a palpable mass, pain, ptosis, and/or mucopurulent discharge. Diagnosis of dacryoadenitis and dacryocystitis is clinical; laboratory studies and imaging may be obtained to evaluate for the underlying cause and to rule out orbital cellulitis. Treatment includes supportive care, antibiotics or antivirals as indicated, and surgery for selected patients. Lacrimal gland tumors are rare; benign tumors generally occur in younger patients, while malignant tumors are more common in patients > 40 years of age. Diagnosis involves imaging and biopsy of the lacrimal gland, and treatment depends on whether the tumor is benign or malignant.

See also "Lacrimal duct laceration."

Congenital dacryostenosis

Background ^[1][2]

• Definition: congenital obstruction of the nasolacrimal duct
• Incidence: occurs in 5–20% of children

Clinical features ^[1][2]

Symptoms typically manifest within the first month of life.

• Epiphora: excessive watery or mucopurulent secretions
• Possible debris on the eyelid and conjunctival irritation
• Usually unilateral but may be bilateral

Diagnosis ^[1][2]

Congenital dacryostenosis is diagnosed clinically.

• Fluorescein dye disappearance test: confirmatory test
  • Instill 1% fluorescein into the conjunctival sac.
  • The presence of fluorescein under blue light after ≥ 5 minutes indicates dacryostenosis.
• Compression of the lacrimal sac
  • Compress the lacrimal sac against its medial border.
  • Expression of mucopurulent secretions indicates likely obstruction in or distal to the lacrimal sac.

Treatment ^[1][2]

General principles

• Congenital dacryostenosis usually resolves spontaneously within the first year of life.
• Initial management consists of measures to promote spontaneous resolution.
• Invasive management is reserved for selected patients.

Refer patients with recurrent infections, amblyopia, or nonresolving dacryostenosis to ophthalmology.

Initial management

• Lacrimal sac massage: Apply gentle downward pressure below the medial canthus of the eye 3–4 times a day.
• Regular cleaning of the eyelids and warm compresses
• Antibiotics are not indicated unless there is evidence of infection.

Invasive management

• Indications
  • Initial management is unsuccessful
  • Complications: e.g., recurrent infections, chronic dacryocystitis, amblyopia
• Procedures
  • Nasolacrimal duct irrigation and probing
  • Endoscopic and/or balloon dilatation of the nasolacrimal duct
  • Dacryocystorhinostomy: surgical connection between the lacrimal sac and nasal cavity

Acquired dacryostenosis ^[1][3]

• Etiology
  • Primary acquired dacryostenosis: idiopathic
  • Secondary acquired dacryostenosis
    • Concretions in the nasolacrimal duct
    • Inflammatory conditions: e.g., granulomatous diseases
    • Scarring: e.g., secondary to viral infection or radiation
    • Trauma
    • Space-occupying lesions: e.g., tumors, encephalocele, mucocele
    • Nasal abnormalities: e.g., nasal septum deviation, rhinitis
• Clinical features and diagnostics: similar to those of congenital dacryostenosis
• Treatment: often requires surgical intervention in addition to treatment of the underlying cause

Acute dacryocystitis

Background ^[4]

• Definition: acute inflammation of the lacrimal sac
• Epidemiology: most commonly occurs in women and young infants
• Etiology: usually a staphylococcal or streptococcal infection in individuals with underlying dacryostenosis
• Pathophysiology: dacryostenosis → retention of tears and detritus in the lacrimal sac → bacterial overgrowth, causing infection

Clinical features ^[4][5][6]

• Erythema, edema, warmth, and pain below the medial canthus
• Epiphora
• Mucopurulent discharge

In dacryocystitis, applying pressure over the lacrimal sac typically elicits pain and purulent discharge from the lacrimal punctum.

Diagnostics ^[4][5][6]

Dacryocystitis is a clinical diagnosis.

• All patients: Culture and Gram stain secreted or expressed ocular discharge or aspirated fluid from the lacrimal sac.
• Further studies
  • Blood cultures: indicated in children, immunocompromised individuals, and patients with signs of sepsis
  • Additional sepsis diagnostics as indicated
  • Imaging: Consider if red flags for orbital cellulitis are present or if an abscess or sinusitis is suspected.
    • CT orbits and sinuses with contrast
    • MRI: Consider in children to avoid radiation exposure.

Treatment ^{[4][5][6] [7]}

• Mild infection in adults and children > 12 months of age
  • Initiate oral empiric antibiotic therapy: similar to oral antibiotics for preseptal cellulitis.
  • Consider concurrent topical antibiotic therapy: e.g., tobramycin. ^[4]
  • Recommend supportive therapy, i.e., analgesics, warm compresses, lacrimal duct massage.
  • Refer for ophthalmology follow-up within 3 days.
  • Surgical intervention for dacryostenosis is often pursued after resolution of the acute infection.
• Infants and/or severe infection (e.g., sepsis, orbital involvement)
  • Obtain ophthalmology consult.
  • Consider incision and drainage if an abscess is present.
  • Initiate IV empiric antibiotic therapy: similar to IV antibiotics for orbital cellulitis.
  • Consider concurrent topical antibiotic therapy with ophthalmology guidance.
  • Admit for inpatient monitoring.
  • Surgical intervention for dacryostenosis is often pursued for respiratory distress or if there is no improvement within 24–48 hours.

Consider empiric antibiotic coverage for MRSA in aggressive or atypical infections or if risk factors for MRSA are present; follow local protocols and expert advice. ^{[4] [8]}

Complications ^[5][6]

• Abscess of the lacrimal sac
• Local extension: preseptal cellulitis, orbital cellulitis, meningitis
• Thrombosis: superior ophthalmic vein thrombosis, cavernous sinus thrombosis
• Dacryocutaneous fistula
• Chronic dacryocystitis

Chronic dacryocystitis ^[4]

Background ^[4]

• Definition: chronic inflammation of the lacrimal sac
• Etiology: bacterial infection, e.g., with Staphylococcus aureus, Streptococcus pneumoniae, Haemophilus influenzae, Pseudomonas aeruginosa ^[4]
• Pathophysiology: congenital/acquired dacryostenosis → stasis of tears → secondary bacterial infection → lacrimal sac inflammation

Clinical features ^[4]

• Persistent epiphora
• Mucopurulent discharge from the punctum
• No signs of acute inflammation; no fever

Diagnostics

• Clinical diagnosis
• Culture of the discharge

Treatment

• Antibiotics (culture-specific)
• Dacryocystorhinostomy (to prevent recurrence)
• Children < 12 months of age with chronic dacryocystitis: See “Treatment” in "Congenital dacryostenosis."

Complications

• Chronic conjunctivitis
• Dacryocystocele formation

Acute dacryoadenitis

Background ^[9][10][11]

• Definition: acute inflammation of the lacrimal gland
• Epidemiology
  • Noninfectious dacryoadenitis (common): Most often seen in adults; ♀> ♂
  • Infectious dacryoadenitis (rare): More common in children and young adults
• Etiology
  • Idiopathic (common)
  • Autoimmune or inflammatory disease (common): e.g., sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease
  • Infection (rare), e.g.:
    • Viral: EBV, mumps, VZV, HSV
    • Bacterial: Staphylococcus aureus, Streptococcus spp., Gonococcus spp.

Clinical features ^[9][10]

Dacryoadenitis manifests with circumscribed swelling and erythema of the lateral upper eyelid; a palpable mass may be present.

• Noninfectious dacryoadenitis:
  • Often bilateral
  • Onset and degree of pain vary
  • Inflammatory signs (e.g., fever) typically absent
• Infectious dacryoadenitis: acute onset and painful
  • Viral
    • May be unilateral
    • Nonpurulent
    • Fever and lymphadenopathy
  • Bacterial
    • Typically unilateral
    • Purulent conjuctival discharge
    • Fever (uncommon)

Acute unilateral painful dacryoadenitis with purulent discharge suggests a bacterial cause. Consider a lacrimal duct abscess if fever is also present. ^[9]

Diagnosis ^{[9][10][11][12]}

Dacryoadenitis is a clinical diagnosis.

• All patients: Culture ocular discharge if present.
• Further studies
  • Laboratory studies: Consider based on clinical suspicion.
    • Inflammatory markers: e.g., WBC count, CRP
    • Serology: e.g., EBV serology, antineutrophil cytoplasmic antibodies
  • Imaging: in patients with red flags for orbital cellulitis or a suspected lacrimal gland abscess
    • CT orbits and sinuses with contrast
    • MRI: Consider in children to avoid radiation exposure.
  • Lacrimal gland biopsy: Consider in consultation with an ophthalmologist.
    • Often required to identify the cause
    • Usually performed transcutaneously

Consider sepsis diagnostics and orbital imaging in patients with red flags for orbital cellulitis.

Treatment ^[9][10][12]

Treatment of acute dacryoadenitis depends on the underlying cause.

• All patients: supportive care (e.g., analgesics, warm compresses)
• Viral dacryoadenitis
  • Most cases are self-limited and resolve within 4–6 weeks.
  • Antiviral therapy may be indicated for specific causes (e.g., antiviral therapy for VZV)
• Bacterial dacryoadenitis ^[7]
  • Consult ophthalmology for incision and drainage if an abscess is suspected.
  • Start empiric antibiotic therapy.
    • Mild disease: similar to oral antibiotics for preseptal cellulitis
    • Moderate or severe disease (e.g., sepsis): similar to IV antibiotics for orbital cellulitis
    • Consider early MRSA coverage (e.g., IV vancomycin), especially in patients with skin wounds and those without sinusitis or preceding upper respiratory tract infection. ^[10]
  • Transition to targeted antibiotic therapy based on culture results.
• Noninfectious dacryoadenitis
  • Consult ophthalmology to guide treatment.
  • Options include:
    • Immunosuppressive therapy: e.g., local or systemic corticosteroids
    • Debulking or excisional surgery
    • Radiotherapy

If the etiology is unclear, consider starting empiric antibiotic therapy with close monitoring to assess for a response.

Disposition ^[12]

• Patients with mild dacryoadenitis may be discharged with outpatient ophthalmology follow-up.
• Consider admission for patients who lack social support or those with moderate or severe disease.

Chronic dacryoadenitis

Background

• Definition: chronic inflammation of the lacrimal gland
• Etiology
  • Inflammatory or granulomatous conditions (most common): e.g., sarcoidosis, granulomatosis with polyangiitis
  • Autoimmune conditions: e.g., Graves disease, Sjogren syndrome
  • Neoplastic: e.g., lacrimal gland tumor, lymphomas
  • Chronic infections, e.g., fungal, mycobacterial (less common)

Clinical features

• Can be unilateral or bilateral
• Insidious onset with painless swelling over the lacrimal gland
• S-shaped ptosis; proptosis rare
• Features of underlying disease may be present (see “Etiology” above).

Diagnosis

• Eye swabs: in patients with ocular discharge
• Screening for chronic infections (e.g., tuberculosis, Chlamydia trachomatis, gonorrhea)
• CT scan: to rule out a malignant etiology
• Fine needle/incisional biopsy of the lacrimal gland: indicated only if imaging/blood tests are inconclusive
• Investigations based on the suspected etiology

Treatment

• Supportive care
• Treatment of the underlying disease

• Etiology
  • Benign tumors: pleomorphic adenoma
  • Malignant tumors
    • Adenocystic carcinoma
    • Mucoepidermoid carcinoma
  • Inflammatory tumors (pseudotumor/inflammatory dacryoadenitis): idiopathic inflammation of the lacrimal gland
• Clinical features
  • Age
    • Benign tumors manifest between 20–40 years of age
    • Malignant tumors manifest > 40 years of age
  • Unilateral swelling of the lateral orbit
  • Unilateral proptosis: painless in benign tumors and painful in malignant tumors
  • Restricted eye movement and diplopia
• Diagnostics
  • CT and/or MRI scan
    • Benign tumors: well encapsulated, no bony destruction
    • Malignant tumors
      • Poorly defined lesions with areas of calcification
      • Destruction of adjacent bones and perineural invasion
    • Pseudotumor: diffusely enlarged lacrimal gland and inflamed surrounding tissue
  • Biopsy of the lacrimal gland
• Treatment
  • Benign tumor: complete removal of the lacrimal gland
  • Malignant tumor
    • Complete removal of the gland in early disease
    • Enucleation of the eye and radiotherapy in advanced disease
  • Pseudotumor: steroids and radiotherapy

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