Fig. 7.1
Giant Papillae in a patient with VKC
What Are Common Examination Findings in VKC ? What Are the Long-Term Sequelae of VKC ?
AKC and VKC have similar clinical presentations , with conjunctival hyperemia, papillary reaction, corneal epitheliopathy, and ulceration as common features of each. A trademark feature of VKC is giant “cobblestone” papillae of the upper tarsus; these are less common in AKC. These giant papillae are greater than 1 mm in diameter. A thick ropy discharge frequently accumulates in the septa in between the giant papillae. Limbal papillae may also be seen and are typically large, gelatinous, and confluent. Frequently, either tarsal papillae or limbal papillae predominate, leading to two broad categories of VKC: palpebral vernal and limbal vernal. In the limbal papillae, Horner-Trantas dots may develop, another classic feature of VKC. These are collections of degenerated epithelial debris and eosinophils that form small white dots at the limbus [5, 6].
Patients with VKC almost always have quiet, uninflamed eyelids and periorbital skin, in contrast to the nearly universal presence of periorbital atopic dermatitis seen in AKC patients. Subepithelial fibrosis of the palpebral conjunctiva and symblepharon formation are also much less common in VKC compared to AKC [8]. The mechanical rubbing of giant papillae against the cornea, along with release of inflammatory mediators, contributes to punctate epithelial erosions and sometimes macroerosions. Long-term sequelae of VKC can lead to corneal neovascularization and scarring. A pseudogerontoxon may be seen; this is a linear corneal scar, resembling a partial arcus senilis (gerontoxon). Chronic inflammation may result in severe stromal thinning and even corneal perforation. These findings are not common, and the long-term prognosis for mild VKC is generally good. However, 6% of patients will go on to develop visual impairment secondary to cataract, glaucoma, or corneal damage [4].
Slit-lamp exam revealed a white and quiet right eye and 1+ conjunctival injection in his left eye. His right cornea was clear. His left cornea showed a superior pannus and superficial neovascularization with diffuse, confluent punctate epithelial erosions ( Fig. 7.2 ). The anterior chamber was deep and quiet in both eyes.
Fig. 7.2
Superior limbal stem cell deficiency in a patient with chronic VKC
Based on the clinical history and presentation, the patient was diagnosed with VKC.
What Is the Pathophysiology of VKC? What Is Appropriate Medical Management for These Patients?
The pathogenesis of VKC is complex and not entirely understood; a thorough discussion of the immune mechanisms involved is beyond the scope of this chapter. In brief, as with allergic conjunctivitis, eosinophils and mast cells play an important role in VKC, and on cytopathologic examination these cells are seen in the conjunctiva of these patients. However, other inflammatory cells, such as lymphocytes, neutrophils, plasma cells, and macrophages, are clearly involved in VKC, as these are also found in large numbers in the conjunctiva [6]. In particular, T-helper lymphocyte cells (i.e., Th2 cells) are responsible for the production of specific interleukins, such as IL-4 and IL-5, which mediate several of the immune responses in VKC [6]. The production of interleukins and chemokines leads to recruitment of leukocytes, as well as increased production of IgE. Interleukins also promote the proliferation of fibroblasts.
Initial management for VKC is similar to that of other forms of allergic conjunctivitis and should include topical antihistamines and lubrication with artificial tears. Nonsteroidal anti-inflammatory drops may also provide some benefit in symptomatic relief. Mast cell stabilizers and preferably dual-acting agents can be useful for chronic maintenance.
Because of the numerous inflammatory mechanisms involved, these measures are usually inadequate. Topical steroids may be used for exacerbations in moderate to severe VKC and should be used judiciously with close monitoring for side effects. Topical cyclosporine eye drops are highly effective as an adjunctive therapy to decrease chronic inflammation. Cyclosporine has been shown to block T-lymphocyte proliferation, conjunctival fibroblast proliferation, and histamine release from mast cells. The commercially available concentration of 0.05% cyclosporine (Restasis, Allergan) can be used up to four times a day, though higher concentrations of 1–2% (compounded) may yield better results. Our standard treatment for these patients is to use topical cyclosporine and a dual-acting antihistamine on a long-term continuous basis. Topical steroids are used for exacerbations and at the lowest effective dose. Topical tacrolimus drops (if available) may be used as an alternative to cyclosporine and likely is more effective given that tacrolimus is 10–100 times more potent than cyclosporine.
Systemic treatment is rarely needed in VKC; however montelukast (Singulair, Merck) has been shown to be effective in reducing symptoms in VKC [9, 10]. Oral antihistamines may have minimal efficacy on VKC but may reduce systemic hyperreactivity. Systemic corticosteroids are rarely necessary but may need to be considered in vision-threatening cases. In cases where systemic steroids are needed, oral tacrolimus is a highly effective steroid-sparing agent which can be used for at least 6–12 months. Comanagement with a pediatric allergist or immunologist is highly recommended in such cases.
Case 3
A 12-year-old male is referred to you by a pediatric ophthalmologist for a chronic corneal ulcer. The child describes severe pain, tearing, and lid swelling of his left eye for approximately 1 month. The parents report that the child has had intermittent itching, tearing, and redness in both eyes for several years. He has been prescribed several different eye drops over the years, with varying effectiveness. The referral note states that the child had a small corneal ulcer in the same eye approximately 2 years ago, which resolved with topical antibiotics and steroids.
Is There Any Further History You Would Like to Elicit from the child’s family? What Is the Differential Diagnosis of a Chronic Corneal Ulcer in This Patient?
The initial evaluation of a corneal ulcer should differentiate between infectious and noninfectious etiologies. Chronic or recurrent corneal ulceration, along with long-standing symptoms of ocular surface irritation and redness, as in this child’s case, points toward an inflammatory cause as the underlying pathology. The differential diagnosis for chronic or recurring corneal ulcer in the pediatric patient includes the following common conditions: atopic and vernal keratoconjunctivitis, staphylococcal blepharitis, ocular rosacea, and herpetic viral keratitis. Pertinent history may include prior episodes of red eye, a history of allergies and/or systemic atopic disease such as eczema and asthma, frequent chalazia or hordeola, history of eyelid blisters or cold sores that may suggest a herpetic etiology, and contact lens wear. It is also important to realize that chronic ocular surface inflammation predisposes the eye to microbial superinfection, and corneal cultures should be considered in all cases of chronic corneal ulceration.
His past medical history is significant only for asthma and allergies, including a severe peanut allergy. He takes oral montelukast and uses an albuterol inhaler as needed for his asthma. He is not currently on any topical medications.
Upon exam, his vision is 20/25 in both eyes. His external lid exam shows moderate swelling of his left upper lid with secondary ptosis (Fig. 7.3 ).
Fig. 7.3
Moderate swelling of the left upper lid with secondary ptosis in a patient with VKC
Upon lid eversion of his left upper lid revealed giant papillae (Fig. 7.4 ).
Fig. 7.4
Giant Papillae in a patient with VKC
His corneal exam showed a 2.5 mm × 1 mm corneal “shield” ulcer with a plaque deposit; fluorescein staining revealed extensive filamentary and punctate keratopathy (Fig. 7.5a, b ).
Fig. 7.5
Typical central shield ulcer with a plaque in a p atient with VKC (a and b)
What Is the Pathogenesis of a Shield Ulcer ?
Shield ulcers of the cornea may develop secondarily from the mechanical injury from giant papillae, as well as chronic inflammation. Initially, a punctate epithelial keratitis develops which may lead to a frank erosion. Eventually a “vernal” plaque at the level of Bowman’s membrane may deposit in these erosions, referred to as a shield ulcer due to its appearance. These are adherent mucus plaques consisting of degraded epithelial cells, eosinophils, and inflammatory cells. The incidence of shield ulcers in VKC has been reported from 3 to 11% [4, 6, 11]. They are typically localized to the superior half of the cornea, which underlies the tarsal papillae. The plaque impedes epithelial healing, and chronic shield ulcers may result in corneal scarring, neovascularization, and stromal thinning.
The patient was diagnosed with VKC and the edges of the shield ulcer were cultured. The patient was started on moxifloxacin four times a day in the left eye and olopatadine 0.1% in both eyes two times a day. The culture had no growth at follow-up. The patient was then started on loteprednol (Lotemax, Bausch & Lomb) four times a day in the left eye.
How Should Shield Ulcers Initially Be Managed?
Shield ulcers can often be difficult to treat and may follow a chronic or relapsing course. Topical steroids should be employed to reduce surface inflammation contributing to plaque deposition, and topical antibiotics should be administered for prophylaxis. After the eye drop regimen has been optimized, debridement of the shield ulcer manually or with the use of phototherapeutic keratectomy can be attempted to stimulate re-epithelization [12, 13]. If the epithelial defect persists, surface treatments such as a bandage contact lens and amniotic membrane transplant can be considered.
The shield ulcer and papillae remained unchanged on topical therapy at the patient’s next follow-up 2 weeks later, though his symptoms of itching and pain were improved. Debridement of the plaque was performed with eventual recurrence of his shield ulcer on further follow-up.
What Are Some Other Treatment Options for Recalcitrant Shield Ulcers ?
The size of the giant papillae has been directly correlated with the persistence or worsening of symptoms [4]. In retrospect, loteprednol was likely inadequate, and more potent steroids (e.g., prednisolone acetate 1% every 2 h) may have been more effective at reducing the inflammation for our patient. Alternatively, a supratarsal injection of steroids can be considered in cases of shield ulcer unresponsive to medical therapy, both to reduce the size of the giant papillae (thereby relieving the mechanical corneal irritation) and to decrease the number of inflammatory mediators on the ocular surface [14, 15]. Multiple injections may be needed. The patient should also be treated with topical cyclosporine on a long-term basis.
Case 4
A 27-year-old male presents with decreased vision in both eyes for 5 years. He also reports a chronically itchy rash around his eyes. His medical history is significant for eczema, asthma, and allergies since childhood.
His visual acuity at presentation was 20/200 and 20/40, respectively. The external lid exam revealed an excoriated, scaly periorbital dermatitis (Fig. 7.6 ). and inspissated meibomian glands with thickened lid margins. Upon lid eversion, diffuse micropapillae were noted on the upper and lower tarsus.
Fig. 7.6
Skin changes in a patient with AKC
What Is the Typical Clinical History of Patients with AKC ? What Are Common Skin and Eyelid Findings in AKC?
Of the diseases in the allergic spectrum, AKC has the most severe, chronic course marked by significant ocular morbidity from corneal and conjunctival scarring. A thorough medical history should be obtained. Of AKC patients, 95% have concurrent eczematous dermatitis [8, 16, 17]. Other common associations include asthma and allergies, seen in up to 65–87% of patients [8, 16, 18]. AKC patients commonly present in the third to fifth decades [7]. Unlike VKC, AKC often persists, and patients may need lifelong treatment.
Clinically, AKC presents with chronic, erythematous itchy eyes with tearing [19, 20]. Pain is rarely reported; however the patient may have ocular irritation with photophobia. The external eyelid exam often reveals wrinkled, flaky, excoriated periorbital skin classic for eczematous dermatitis. Other signs include Dennie-Morgan folds, which are additional linear creases of the lower lids secondary to edema and eyelid thickening, and de Hertoghe sign, referring to the loss of hairs in the outer third of the brow [21]. Vertical corrugations near the medial canthus of the upper and lower lids may be seen. With progression of eczema, fissuring of the skin can be seen, and in long-standing AKC, ectropion, ptosis, lagophthalmos, and madarosis may result.
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