Vernal keratoconjunctivitis (VKC) is a chronic, progressive, allergic ocular surface disorder that can lead to sight-threatening complications. VKC occurs primarily in children and generally resolves about the time of puberty; however, case series and retrospective analyses indicate that approximately 10% of patients with VKC are adults, and that a subset of adult cases develop after puberty. Consequently, two age-related variants of VKC have recently been described: early-onset VKC-which manifests during childhood and persists into adult life-and late-onset disease, which emerges de novo after puberty. Although the signs and symptoms of adult and childhood VKC are similar, adult VKC is a long-lasting disease characterized by severe inflammation and increased risk of conjunctival fibrosis, which may place adult patients at higher risk for sight-threatening complications and adverse impacts on daily life. This review discusses the epidemiology, signs, symptoms, immunopathogenesis of adult VKC variants, and highlights current gaps in research and management of patients with this condition.

Vernal keratoconjunctivitis (VKC) is a chronic, bilateral corneal and conjunctival problem which typically presents in young individuals. VKC is characterized by itching, photophobia, white mucous discharge, lacrimation, foreign body sensation, and pain due to corneal involvement of shield ulcers. Vernal keratoconjunctivitis is categorized within ocular diseases. The diagnosis is clinical, as no sure biomarkers pathognomonic of the disease have yet been identified. The VKC therapy relies on different types of drugs, from antihistamines and topical steroids to cyclosporine or tacrolimus eye drops. In extremely rare cases, there is also the need for surgical treatment for the debridement of ulcers, as well as for advanced glaucoma and cataracts, caused by excessive prolonged use of steroid eye drops. We performed a systematic review of the literature, according to PRISMA guideline recommendations. We searched the PubMed database from January 2016 to June 2023. Search terms were Vernal, Vernal keratoconjunctivitis, and VKC. We initially identified 211 articles. After the screening process, 168 studies were eligible according to our criteria and were included in the review. In this study, we performed a systematic literature review to provide a comprehensive overview of currently available diagnostic methods, management of VKC, and its treatments.

BACKGROUND: Vernal keratoconjunctivitis (VKC) severely affects the quality of life of affected patients. The development of a shield ulcer is considered one of the most severe late-stage complications, which when untreated leads to irreversible vision loss. In this systematic review, we outlined the results of surgical treatments of corneal shield ulcers in VKC.
METHODS: We searched 12 literature databases on 3 April 2023 for studies of patients with VKC in which shield ulcers were treated by any surgical treatment. Treatment results were reviewed qualitatively. Assessments of the risk of bias of individual studies were made using the Clinical Appraisal Skills Programme.
RESULTS: Ten studies with 398 patients with VKC were eligible for the qualitative review. Two categories of surgical approaches were described: supratarsal corticosteroid injection and debridement with or without amniotic membrane transplantation. Almost all patients experienced resolution or improvement of their shield ulcers, regardless of treatment modality. Time to healing was faster with surgical debridement. A small proportion experienced recurrence and side effects.
CONCLUSIONS: Surgical treatment for shield ulcers in VKC seems highly effective, but careful post-operative treatment and follow-ups are necessary due to the risk of recurrence and potential side effects.

To determine the prevalence of allergic sensitization in patients with vernal keratoconjunctivitis (VKC) and to provide an overview of published studies on this topic. We systematically searched 11 literature databases on 24 May 2021, for studies with cross-sectional data on the prevalence of positive allergy tests in patients with VKC. Our main outcome of interest was the prevalence of allergic sensitization and the allergens involved. Prevalence meta-analyses were made to provide summary estimates. We identified 33 eligible studies for qualitative review with 2122 patients with VKC. Studies were predominantly based on patients seen in ophthalmology clinics. Overall, studies reported that the most prevalent positive allergen tests were the inhaled allergens house dust mites and pollen. Twenty-nine studies were eligible for the quantitative analysis. Here, we calculated the prevalence of allergen-positive patients to 57.7% (95% confidence interval: 52.5%-62.8%). Subgroup analyses of pooled estimates on sensitization based on specific testing methods found prevalence estimates of 51.4% for conjunctival provocation test, 68.7% for total tear IgE, 58.9% for specific tear IgE, and 58.2% for skin prick test. The prevalence of allergic sensitization in patients with VKC is 57.7%, and mostly towards inhaled allergens. The most frequent positive allergens are house dust mites and pollen. Identifying possible clinically relevant allergens provide information that may aid in managing VKC, such as environmental allergy-avoidance or allergy-specific treatment.

Giant papillae (1.0 mm or greater in diameter) on the upper tarsal conjunctiva are one of the most common findings in cases of vernal keratoconjunctivitis (VKC). Presently described is the case of a 3-year-old female with a unilateral giant papilla formation prolabing from the medial side of the upper tarsal conjunctiva toward the ocular surface in the left eye. A brief review of the recent literature concerning the etiology, associated risk factors, surgical options, and management of patients with giant papillae is also discussed. A significant reduction in the size of the papilla was observed after a week of medical treatment with a topical steroid and antiallergic eye drops. To the best of our knowledge, this case demonstrates the first time that a giant papilla appeared in the form of a prolapsed mass extending from the tarsal conjunctiva in a child with VKC. Ophthalmologists should keep in mind that a giant papilla can look like another form of conjunctival mass, and should not rush to excise or biopsy, as it may respond to medical treatment.

OBJECTIVE: To systematically review the literature on the treatment of vernal keratoconjunctivitis (VKC) in children and young adults and conduct comparative efficacy analysis on clinical signs and symptoms using network meta-analyses.
METHODS: We systematically searched the databases PubMed/MEDLINE, EMBASE, Cochrane Central and Web of Science on 21 October 2019 for randomized controlled trials (RCT). Studies considered had patients with VKC < 20 years of age randomized into either intervention (any medical intervention) or comparator (active treatment, placebo treatment or non-treatment control), where pre-defined outcomes (data from ≥2 weeks and as close as possible to 2 months) of symptoms (itching, tearing, photophobia and foreign body sensation) and signs (hyperaemia, punctate keratitis, Horner-Trantas dots and macropapillae) were reported. Risk of bias within studies was evaluated using the Cochrane risk of bias tool. Comparisons were made using network meta-analyses.
RESULTS: We identified 39 studies with data on 2046 individuals. Twenty-three studies were eligible for quantitative analyses. None were systemic therapy. Temporal trend analysis showed that an initial focus on topical mast cell stabilizers turned to a focus on calcineurin inhibitors and a more diverse variety of pharmacological strategies. Studies varied in population, treatment duration and quality. The quantitative analysis revealed that efficacy of different therapies differed substantially across important clinical signs and symptoms, but there was a general trend of superior efficacy when using topical corticosteroids with stronger efficacy of the more potent corticosteroids.
CONCLUSIONS: We provide an overview of RCTs comparing the efficacy of treatments for VKC in children and young adults, which we find differs across symptoms and signs. Overall, we saw a general trend of superior efficacy with topical corticosteroids. However, our findings highlight the need for better studies, consensus on core outcomes and potential for individualized therapy.

Vernal keratoconjunctivitis (VKC) is a chronic, bilateral, allergic conjunctivitis with episodes of acute exacerbations. Although VKC has a self-limiting course, chronic recurrent inflammation can cause long-term visual impairment due to corneal complications including shield ulcers, infectious keratitis, keratoconus, corneal opacities, and limbal stem cell deficiency. The initial step in the management of corneal involvement is medical treatment of the acute stage of VKC and prevention of recurrences. Giant papillae not responding to medical treatment can be removed surgically in the case of corneal involvement. Shield ulcer with no inflammatory plaque usually heals with appropriate medical therapy. For shield ulcer with inflammatory plaque, however, surgical debridement with or without amniotic membrane transplantation might be necessary. Keratoconus may develop in chronic and severe VKC. An annual evaluation of these patients with corneal topography and/or tomography is essential for early detection of keratoconus and its timely management that includes collagen cross-linking and intrastromal corneal ring segment implantation. Corneal transplantation may be required in the advanced stage of keratoconus. Both penetrating keratoplasty and deep anterior lamellar keratoplasty can result in excellent visual outcomes in keratoconic eyes with concomitant VKC. Appropriate management of inflammation in the perioperative period is crucial for achieving successful outcomes after corneal transplantation. Limbal stem cell deficiency, a rare complication of long-standing and severe VKC, might be treated with living-related conjunctival limbal allograft.

OBJECTIVE: To evaluate the early- and late-stage complications of accelerated cross-linking (A-CXL) in a large case series and investigate the relationship of A-CXL complications with ocular and systemic conditions accompanying keratoconus.
METHODS: Retrospectively, records of 2025 eyes of 1184 patients diagnosed with keratoconus between March 2013 and 2020 who underwent A-CXL (18 mW/cm2 for 5 min) treatment were reviewed. Comorbid ocular and systemic diseases other than keratoconus were recorded. In the postoperative follow-up, early and late complications and their association with accompanying diseases were examined.
RESULTS: The mean age of patients was 26.16 ± 6.05 (range 18-42), and the mean follow-up time was 3.40 ± 1.63 years (range 1-8 years). Vernal keratoconjunctivitis (3.7%) was the most common disease accompanying keratoconus. Less frequently, systemic allergic diseases (2.9%) and Down syndrome (2.3%) were observed. The most common complication in the early postoperative period was haze formation (9.1%), while failure of the treatment (4.2%) occurred in the late period. Other common complications were loss of two or more Snellen lines (2.4%) and delayed epithelial healing (1.8%). There was a significant relationship between vernal keratoconjunctivitis and delayed epithelial healing (p = 0.011). Statistically significant relationships were found between Down syndrome and haze formation (p < 0.001) and sterile infiltrate formation (p = 0.041).
CONCLUSIONS: Although A-CXL complication rates are low, vernal keratoconjunctivitis following A-CXL might increase the risk of delayed epithelial healing, and similarly corneal haze and sterile infiltrates might be observed more frequently in patients with Down syndrome.

BACKGROUND: Ocular allergy is a common disease, especially in the pediatric population, with unpleasant and long-term consequences, including corneal complications and decreased visual acuity. This study reports two cases of corneal shield ulcer due to vernal keratoconjunctivitis, with good results of surgical debridement performed after failure of long-term clinical treatment. Furthermore, this study highlights that this therapeutic approach, although less common, is efficient in treating refractory cases that cause suffering in pediatric patients.
METHODS: The first patient was a 7-year-old Caucasian boy with chronic ocular allergy, especially photophobia, who had been treated with eye drops, antihistamine, and corticosteroids for 60 days without success. Biomicroscopy of the right eye showed the presence of gelatinous limbus, giant papillae in the tarsal conjunctiva, and a shield ulcer measuring 6.0 mm vertically and 2.7 mm horizontally. Surgical debridement was performed, and the ulcer did not recur. The second patient was a 4-year-old Caucasian boy with chronic ocular allergy, especially itching and photophobia, who had been treated with eye drops for 1 year without success. Biomicroscopy of the left eye showed a shield ulcer, with a dense central corneal plaque, measuring 8 mm vertically and 3.5 mm horizontally. Surgical debridement of the ulcer was performed immediately because of the chronicity of the case and severity of the lesion, and the treatment was effective.
CONCLUSIONS: The treatment of shield ulcers caused by vernal keratoconjunctivitis in the two reported cases was curative and definitive by surgical debridement in the 7-month follow-up period. Therefore, the early debridement of shield ulcers refractory to drug treatment can considerably reduce the time of disease evolution and the probability of ocular complications caused by clinical treatment or disease chronicity. However, this approach is rarely described in the literature and needs to be included in the ophthalmologist\'s therapeutic arsenal.

Vernal keratoconjunctivitis (VKC) is a rare, recurrent and multifactorial ocular disease, which typically flares up during spring and affects especially male children and adolescents. This condition does not usually respond to common treatments with antihistamines or mast cells stabilizers, whereas corticosteroids have effective results. Corticosteroids need to be carefully administered, to avoid adverse effects, mainly the secondary development of glaucoma, cataracts, or infections. Immunosuppressive agents, such as cyclosporin (CyA) or tacrolimus are, therefore, frequently employed in VKC patients. Only the 0.1% CyA (1 mg/mL) concentration has an approved and specific clinical indication for the treatment of VKC and this drug was given the denomination of orphan drug by the European Commission (EU/3/06/360) in 2006. So far, few studies have been conducted to evaluate the efficacy and the side effects of topical 0.1% CyA. Different topical CyA concentrations, ranging from 0.05% to 2%, and various types of formulation are available at the moment. In the future, 0.1% CyA will presumably take an important part in the management of VKC. The present review focuses on eye drops containing 0.1% CyA; however, more studies will be needed to define its long-term efficacy in the natural course of this severe ocular disease.