Vernal Keratoconjunctivitis (Spring catarrh)

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Contents

Aetiology

Rare allergic disorder of children (prevalence in Western Europe is 3.2 per 10,000 inhabitants)
More common in some other parts of the world, e.g. Mediterranean region, parts of Africa, Indian sub-continent
Complex immune reaction with raised IgE levels in the tears and serum, and mast cells and eosinophils in the conjunctival epithelium. T cells also play a significant role

Predisposing factors

Onset usually before 10 years of age; M:F = 3-4:1
Seasonal exacerbations (hence name) but condition may be active year-round if severe
Patients usually atopic with a history of eczema and asthma
Often a family history of atopic disease

Symptoms of vernal keratoconjunctivitis

Ocular itching
Watering
Mucoid stringy discharge
Blurred vision
Photophobia
Difficulty opening eyes on waking
NB: the symptoms are often asymmetrical in the two eyes

Signs of vernal keratoconjunctivitis

Grading scales (e.g. that of Bonini et al) may be helpful in judging severity of disease
Stringy white mucous exudate
Palpebral, limbal and corneal manifestations:

Palpebral

  • hyperaemia and chemosis of conjunctiva when active
  • giant papillary hypertrophy (papillae 1mm or greater in diameter) of upper tarsus (‘cobblestone’ appearance)

Limbal

  • hyperaemic, oedematous, thickened limbus
  • Trantas’s Dots (discrete white superficial accumulations of eosinophils and degenerating epithelial cells)

Corneal (usually in upper third)

  • punctate epithelial keratopathy
  • macro-erosion (coalescent epithelial loss)
  • plaque (deposited on Bowman’s layer, preventing re-epithelialisation); ‘shield ulcer’ in US terminology
  • subepithelial scarring (often ring-shaped)

NB: the signs are often asymmetrical in the two eyes
These patients may also have keratoconus and/or atopic cataract

Differential diagnosis

Atopic keratoconjunctivitis (usually in adults; around puberty, VKC may metamorphose into this disease)

Management by optometrist

Practitioners should recognise their limitations and where necessary seek further advice or refer the patient elsewhere

Non pharmacological

Cold compresses may reduce acute symptoms
(GRADE*: Level of evidence=low, Strength of recommendation=strong)

Pharmacological

Mast cell stabilisers e.g. gutt sodium cromoglicate 2% qds, gutt lodoxamide 0.1% qds
Because of the sight-threatening nature of this condition, and the frequent need for other medical specialist involvement, maintain low threshold for referral to the ophthalmologist
(GRADE*: Level of evidence=moderate, Strength of recommendation=strong)

Management category

Mild cases (without active limbal or corneal involvement, e.g. up to Bonini Grade 2a):
B2: Alleviation or palliation; no referral
Initial management with mast cell stabilizers. VKC requires careful
monitoring for sight-threatening complications

B1: Possible prescription of drugs; routine referral
Routine referral if mast cell stabilizers fail to provide symptomatic relief

If there is active limbal or corneal involvement:
A3: First aid measures followed by urgent referral (within one week) to an ophthalmologist

Possible management by ophthalmologist

VKC often requires a multi-disciplinary approach (e.g. clinical immunologist, paediatrician). Other topical drugs used include steroids, immunosuppressants (e.g. ciclosporin, tacrolimus) and mucolytics (acetyl cysteine).
Manual or laser surgery may be required for the removal of corneal plaque

Evidence base

*GRADE: Grading of Recommendations Assessment, Development and Evaluation (www.gradeworkinggroup.org)
 

Sources of evidence

Addis H, Jeng BH. Vernal keratoconjunctivitis. Clin Ophthalmol. 2018;12:119-123

Avunduk AM, Avunduk MC, Kapicioglu Z, Akyol N, Tavli L. Mechanisms and comparison of anti-allergic efficacy of topical lodoxamide and cromolyn sodium treatment in vernal keratoconjunctivitis. Ophthalmology. 2000;107:1333-7

Bonini S, Sacchetti M, Mantelli F, Lambiase A. Clinical grading of vernal keratoconjunctivitis. Curr Opin Allergy Clin Immunol. 2007;7(5):436-41

Bremond-Gignac D, Doan S, Amrane M, Ismail D, Montero J, Németh J, Aragona P, Leonardi A; Twelve-Month Results of Cyclosporine A Cationic Emulsion in a Randomized Study in Patients With Pediatric Vernal Keratoconjunctivitis. VEKTIS Study Group. Am J Ophthalmol. 2020;212:116-126

De Smedt S, Nkurikiye J, Fonteyne Y, Tuft S, De Bacquer D, Gilbert C, Kestelyn P. Topical ciclosporin in the treatment of vernal keratoconjunctivitis in Rwanda, Central Africa: a prospective, randomised, double-masked, controlled clinical trial. Br J Ophthalmol. 2012;96:323-8

Leonardi A, Doan S, Amrane M, Ismail D, Montero J, Németh J, Aragona P, Bremond-Gignac D; VEKTIS Study Group. A Randomized, Controlled Trial of Cyclosporine A Cationic Emulsion in Pediatric Vernal Keratoconjunctivitis: The VEKTIS Study. Ophthalmology. 2019;126(5):671-681

Müller EG, Santos MSD, Freitas D, Gomes JÁP, Belfort R Jr. Tacrolimus eye drops as monotherapy for vernal keratoconjunctivitis: a randomized controlled trial. Arq Bras Oftalmol. 2017;80(3):154-158

Singhal D, Sahay P, Maharana PK, Raj N, Sharma N, Titiyal JS. Vernal Keratoconjunctivitis. Surv Ophthalmol. 2019;64(3):289-311

Vichyanond P, Pacharn P, Pleyer U, Leonardi A. Vernal keratoconjunctivitis: a severe allergic eye disease with remodeling changes. Pediatr Allergy Immunol. 2014;25(4):314-22

Plain language summary

Vernal Keratoconjunctivitis (VKC), also known as Spring Catarrh, is a rare but serious allergic disease affecting the eyes of young children, especially boys, who usually have other allergic diseases such as eczema and/or asthma. It usually begins before the age of 10 years and often disappears at puberty, though it may change at that time into another allergic eye disease known as Atopic Keratoconjunctivitis.

Children with VKC complain of itching of the eyes, watering and a stringy discharge. Their vision may be blurred and they may be abnormally sensitive to light. A typical symptom is that they may have great difficulty in opening their eyes after waking. This and the very distracting effects of the condition may cause them to miss school.

VKC produces inflammation of the eye surface. On the underside of the upper eyelids, tiny bumps shaped like cobblestones appear. Substances released from this tissue can cause damage to the cornea (the clear window of the eye). Sometimes a whitish deposit, known as plaque, may accumulate on the cornea, which may also become scarred, causing problems with vision. 

VKC is not a simple allergic condition like Seasonal Allergic Conjunctivitis (Hay Fever Conjunctivitis) as it involves various different types of immune reaction. This is why many cases need to be referred to the ophthalmologist, who in turn may refer them to other allergy specialists. Surgery is sometimes needed when plaque has accumulated on the cornea.

Vernal Keratoconjunctivitis (Spring catarrh)
Version 14
Date of search 15.09.20
Date of revision 18.12.20
Date of publication 20.08.21
Date for review 14.09.22
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