Keratoconjunctivitis Cornealis (Phlyctenular Keratoconjunctivitis)
Overview
Keratoconjunctivitis cornealis, more commonly known as phlyctenular keratoconjunctivitis (PKC)**, is an immuneâmediated inflammatory disease that involves the conjunctiva (the thin membrane covering the white of the eye) and the superficial layers of the cornea. Small, whiteâyellow nodulesâcalled phlyctenulesâform at the limbus (the border between the cornea and sclera) and can ulcerate if left untreated.
The condition is most frequently observed in children and young adults, especially in regions where ocular surface infections such as staphylococcal blepharitis or tuberculosis are common.
- Age group most affected: 5â30âŻyears.
- Gender: Slight male predominance in some series, but overall distribution is roughly equal.
- Geographic prevalence: Higher in developing countries and in communities with limited access to eyeâcare; prevalence estimates range from 0.2âŻ% to 2âŻ% in pediatric ophthalmology clinics in these regions[1].
Symptoms
Symptoms can vary from mild irritation to severe pain and visual disturbance. Common features include:
- Redness (hyperemia): Diffuse or localized to the limbus.
- Foreignâbody sensation: The feeling that something is in the eye.
- Burning or itching: Often worse in the evening.
- Tearing (epiphora): Excessive watery discharge.
- Photophobia: Light sensitivity that may cause squinting.
- Linear or nodular corneal infiltrates: Visible as small whiteâyellow raised lesions.
- Ulceration: If a phlyctenule ruptures, a small ulcer may develop, leading to a gritty feeling.
- Decreased visual acuity: Usually mild, but can progress if scarring occurs.
- Palpebral crusting: Especially if concurrent blepharitis is present.
Causes and Risk Factors
Underlying Mechanism
PKC is a type of delayedâtype (TypeâŻIV) hypersensitivity reaction. Antigens from bacteria, fungi, or other microorganisms trigger an immune response that results in localized inflammation at the cornealâconjunctival junction.
Common Triggers
- Staphylococcus aureus: The most frequent bacterial antigen, especially in cases associated with chronic blepharitis.
- Mycobacterium tuberculosis: Particularly in endemic regions; a positive Mantoux test is frequently found.
- Fungal spores: Aspergillus or Candida species in agricultural settings.
- Parasitic antigens: Rarely, parasites such as Demodex may act as triggers.
Risk Factors
- Age 5â30âŻyears (immune system still maturing).
- Chronic blepharitis or meibomian gland dysfunction.
- Living in crowded or unsanitary conditions that facilitate ocular surface colonization.
- History of tuberculosis or close contact with an active case.
- Contact lens wear (especially poor hygiene).
- Systemic immune dysregulation (e.g., atopic dermatitis).
Diagnosis
Diagnosis is primarily clinical, based on a characteristic slitâlamp appearance, but ancillary tests help confirm the underlying cause.
Clinical Examination
- Slitâlamp biomicroscopy: Reveals small (0.5â2âŻmm) white or yellowish nodules at the limbus with surrounding vascular injection. Older lesions may show a central ulcer with an overlying fibrinous membrane.
- Fluorescein staining: Highlights any corneal epithelial defects or ulceration.
- Eyelid inspection: Looks for blepharitis, crusting, or meibomian gland blockage.
Laboratory and Imaging Studies
- Conjunctival swab or impression cytology: To isolate bacterial, fungal, or mycobacterial organisms when infection is suspected.
- Culture and sensitivity: Guides targeted antibiotic therapy.
- Mantoux (tuberculin) test or IGRA: Screen for latent tuberculosis.
- Serologic tests for HIV: Considered in immunocompromised patients.
- Anterior segment optical coherence tomography (ASâOCT): Provides highâresolution images of infiltrate depth, useful for monitoring response to therapy.
Treatment Options
The goal is to eliminate the inciting antigen, control inflammation, and prevent scarring.
Medical Management
- Topical corticosteroids: Firstâline for active inflammation (e.g., prednisolone acetate 1âŻ%). Typical regimen: 4â6 times daily for 1âŻweek, then taper based on response. Longâterm use requires intraocular pressure (IOP) monitoring.
- Topical nonâsteroidal antiâinflammatory drugs (NSAIDs): May be used as steroidâsparing agents (e.g., ketorolac 0.5âŻ%).
- Antibiotic therapy:
- Empiric broadâspectrum topical antibiotics (e.g., moxifloxacin 0.5âŻ%) for 5â7âŻdays to cover Staphylococcus.
- If Staphylococcus is confirmed, a targeted agent such as erythromycin ointment 0.5âŻ% can be continued.
- Antitubercular therapy (ATT): When Mycobacterium tuberculosis is identified or Mantoux test is strongly positive, a standard 6âmonth regimen (isoniazid, rifampin, ethambutol, pyrazinamide) is indicated per WHO guidelines[2].
- Antifungal drops: For confirmed fungal etiology (e.g., natamycin 5âŻ%).
Procedural Interventions
- Debridement of necrotic tissue: Rarely needed but can hasten healing when a large ulcer is present.
- Amniotic membrane transplantation: Considered for persistent epithelial defects or impending perforation.
- Cycloplegic agents (e.g., cyclopentolate 1âŻ%): Reduce painful ciliary spasm and photophobia.
Lifestyle and Supportive Measures
- Warm compresses and lid hygiene (soapâfree lid scrubs twice daily) to control blepharitis.
- Avoidance of contact lenses until the inflammation fully resolves.
- Use of preservativeâfree artificial tears 4â6 times daily for comfort.
Living with Keratoconjunctivitis Cornealis (Phlyctenular Keratoconjunctivitis)
Daily Management Tips
- Adhere to medication schedule: Missing doses of steroids or antibiotics can lead to flareâups.
- Protect your eyes: Wear sunglasses outdoors to reduce photophobia and UVâinduced aggravation.
- Maintain eyelid hygiene: Use commercially available lidâscrub pads or diluted baby shampoo.
- Monitor intraâocular pressure (IOP): If you are on topical steroids for more than 2âŻweeks, have your eye doctor check IOP every 2â4âŻweeks.
- Stay up to date with vaccinations: Especially BCG in TBâendemic areas, as it may lower systemic sensitization.
- Follow up appointments: Initially weekly, then every 4â6âŻweeks once stable.
Impact on Vision & Activities
Most patients regain full visual acuity after treatment, but scarring at the limbus can cause mild astigmatism. An optometrist may need to prescribe glasses or contact lenses after the acute phase.
Prevention
- Good ocular surface hygiene: Daily lid cleaning, regular hand washing before touching eyes.
- Prompt treatment of blepharitis or skin infections: Reduces the bacterial antigen load.
- Screen for tuberculosis: In highârisk settings, a Mantoux test or IGRA should be performed annually.
- Proper contactâlens care: Use recommended solutions, replace lenses as scheduled, and avoid overnight wear unless approved.
- Environmental control: Reduce exposure to dust, smoke, and agricultural aerosols that may carry fungal spores.
Complications
If PKC is not adequately treated, several complications may arise:
- Corneal scarring: Leads to permanent visual impairment and irregular astigmatism.
- Corneal neovascularization: New blood vessels invade the cornea, further compromising transparency.
- Perforation: Rare but serious; requires surgical intervention.
- Secondary bacterial or fungal infection: Ulcerated lesions become a portal for opportunistic pathogens.
- Glaucoma: Prolonged steroid use can elevate IOP.
When to Seek Emergency Care
- Sudden, severe eye pain that does not improve with analgesics.
- Rapid loss of vision or a large area of blurry vision.
- A rapidly enlarging white spot or ulcer on the cornea.
- Signs of eye infection spreading beyond the surface (e.g., swelling of the eyelid, fever, pus discharge).
- Persistent photophobia and tearing despite medication, especially if accompanied by redness spreading to the whole eye.
These symptoms may indicate corneal perforation, acute glaucoma, or a severe secondary infection, all of which require immediate treatment.
Sources:
[1] World Health Organization. âPrevalence of ocular surface disease in children.â WHO Vision Fact Sheet, 2022.
[2] WHO. âGuidelines for the treatment of tuberculosis, 2023 Update.â
[3] Mayo Clinic. âPhlyctenular keratoconjunctivitis.â Updated 2023.
[4] American Academy of Ophthalmology. âKeratoconjunctivitis â Clinical Practice Guidelines.â 2021.
[5] National Eye Institute, NIH. âEye Health Information: Inflammatory Eye Diseases.â 2022.