CLINICAL MANAGEMENT GUIDELINES Atopic Keratoconjunctivitis (AKC) Aetiology Severe ocular surface disease affecting some atopic individuals Complex immunopathology including T-cell mediated (type 4 hypersensitivity) Symptoms of AKC typically begin in the late teens or early twenties and can persist until the fourth or fifth decade of life. The peak incidence of AKC occurs between the ages of 30 and 50 years Sometimes follows childhood Vernal Keratoconjunctivitis (VKC) (see Clinical Management Guideline on Vernal Keratoconjunctivitis) Predisposing factors The majority of patients have a personal history of asthma, and eczema (atopic dermatitis) There may also be a family history of atopic disease Most patients have eczema affecting the eyelids and periorbital skin There is a strong association with staphylococcal lid margin disease Specific allergens may exacerbate the condition Symptoms Ocular itching, burning, watering, usually bilateral Blurred vision, photophobia White stringy mucoid discharge Onset of ocular symptoms may occur several years after onset of atopy Symptoms usually year-round, with exacerbations Signs Eyelids may be thickened, crusted and fissured Associated chronic staphylococcal blepharitis Tarsal conjunctiva: giant papillary hypertrophy, subepithelial scarring and shrinkage Entire conjunctiva hyperaemic Limbal inflammation Corneal involvement is common and may be sight-threatening: beginning with punctate epitheliopathy that may progress to macro-erosion, plaque formation (usually upper half), progressive corneal subepithelial scarring, neovascularisation, thinning, and rarely spontaneous perforation These patients are prone to develop herpes simplex keratitis (which may be bilateral), corneal ectasia such as keratoconus, atopic (anterior or posterior polar) cataracts, retinal detachment Differential diagnosis Vernal Keratoconjunctivitis Other allergic conjunctivitis, e.g. Giant Papillary Conjunctivitis (GPC) (often contact lens-related) Toxic Keratoconjunctivitis Management by Optometrist Practitioners should recognise their limitations and where necessary seek further advice or refer the patient elsewhere Non pharmacological Lid hygiene and treatment of associated staphylococcal blepharitis (see Clinical Management Guideline on Blepharitis) Cool compresses Advise avoidance of specific allergens if known, e.g. elimination of pets and carpeting, where necessary; instillation of air filtering devices and alterations to bedding materials (GRADE*: Level of evidence=low, Strength of recommendation=strong) Pharmacological Systemic antihistamines e.g. cetirizine Topical mast cell stabilisers, e.g. gutt. sodium cromoglicate 2%, gutt. lodoxamide 0.1%, or dual acting agents e.g. gutt olopatadine 0.1%, gutt Atopic Keratoconjunctivitis (AKC) Version 14, Page 1 of 3 Date of search 23.11.18; Date of revision 26.11.18; Date of publication ab.cd.ef; Date for review 22.11.20 © College of Optometrists CLINICAL MANAGEMENT GUIDELINES Atopic Keratoconjunctivitis (AKC) ketotifen 0.025%, (off-licence use; maximum duration 4 months) may also provide symptomatic relief (GRADE*: Level of evidence=low Strength of recommendation=weak) Management Category Severe corneal complications are common and potentially sight- threatening. If corneal epithelial macro-erosion or plaque are present: A3: First aid measures followed by urgent referral (within one week) to an ophthalmologist Milder cases (without active corneal involvement): B1: Possible prescription of drugs; routine referral Possible management by Ophthalmologist Often requires multidisciplinary management (ophthalmology, dermatology, immunology) Topical steroids with monitoring and management of complications, e.g. steroid glaucoma and cataract Topical/systemic antibiotics for lids Topical immunosuppression (e.g. ciclosporin, tacrolimus) Treatment of facial eczema and atopic blepharitis Surgery for atopic cataract Evidence base *GRADE: Grading of Recommendations Assessment, Development and Evaluation (see http://www.gradeworkinggroup.org/index.htm) Sources of evidence Brémond-Gignac D, Nischal KK, Mortemousque B, Gajdosova E, Granet DB, Chiambaretta F. Atopic Keratoconjunctivitis in Children: Clinical Features and Diagnosis. Ophthalmology. 2016;123(2):435-7 González-López JJ, López-Alcalde J, Morcillo Laiz R, Fernández Buenaga R, Rebolleda Fernández G. Topical cyclosporine for atopic keratoconjunctivitis. Cochrane Database of Systematic Reviews 2012;9:CD009078 Nivenius E, Montan P. Spontaneous corneal perforation associated with atopic keratoconjunctivitis: a case series and literature review. Acta Ophthalmol. 2015;93(4):383-7 Patel N, Venkateswaran N, Wang Z, Galor A. Ocular involvement in atopic disease: a review. Curr Opin Ophthalmol. 2018;29(6):576-581 Power WJ, Tugal-Tutkun I, Foster CS. Long-term follow-up of patients with atopic keratoconjunctivitis. Ophthalmology. 1998;105:637-42 Zhan H, Smith L, Calder V, Buckley R, Lightman S. Clinical and immunological features of atopic keratoconjunctivitis. Int Ophthalmol Clin. 2003;43(1):59-71 Atopic Keratoconjunctivitis (AKC) Version 14, Page 2 of 3 Date of search 23.11.18; Date of revision 26.11.18; Date of publication ab.cd.ef; Date for review 22.11.20 © College of Optometrists CLINICAL MANAGEMENT GUIDELINES Atopic Keratoconjunctivitis (AKC) LAY SUMMARY Atopic keratoconjunctivitis is a chronic (long-term) allergic condition of the eyelids and front surface of the eye. It is present in a high percentage of patients who have the skin condition, atopic dermatitis. Atopic keratoconjunctivitis requires long-term treatment to prevent sight-threatening complications such as scarring of the cornea (the clear window at the front of the eye). In the early stages of the disease, symptoms can be controlled by standard anti-allergy drugs. However, short- term use of steroid eye drops is often required when symptoms are severe. There is some evidence that cases that do not respond to steroids, or those requiring steroids eye drops long term, may benefit from ciclosporin or tacrolimus eye drops or ointment. Many cases need to be referred to the ophthalmologist for management, who in turn may refer them to specialists in other allergic diseases. Atopic Keratoconjunctivitis (AKC) Version 14, Page 3 of 3 Date of search 23.11.18; Date of revision 26.11.18; Date of publication ab.cd.ef; Date for review 22.11.20 © College of Optometrists CLINICAL MANAGEMENT GUIDELINES Basal cell carcinoma (BCC) (periocular) Aetiology Commonest (80-90%) malignant tumour of the skin of white people Commonest periocular malignancy Incidence of BCC shows marked geographic variation; has been reported as 233 per 100,000 population per annum (in Wales) while in Australia incidence has been estimated as high as 884 per 100,000 per annum Arises from the basal layer of the epidermis Rarely metastasizes (incidence <0.1%) Slow growing, locally invasive Predisposing factors Older patients (median age at diagnosis 67 years; rare under 40 years) M:F = 3:2 History of sunlight (UVB) exposure (particularly during childhood and adolescence) Outdoor workers are at significantly increased risk Fair complexion History of previous BCC or other non-melanoma skin cancer Immune compromise (e.g. HIV infection, immunosuppression) Symptoms Slow developing, non-resolving lesion of eyelid skin Usually painless, may bleed Signs Location in order of prevalence (commonest first) • lower lid • medial canthus (deeper tissue penetration more likely with possible invasion of orbit or paranasal sinuses) (may be hidden by spectacle nose pad) • upper lid • lateral canthus Three clinical presentations in order of prevalence (commonest first) • Nodular (hard nodule, pearly appearance, abnormal (telangiectatic) vessels) • Nodulo-ulcerative (as nodular but with raised rolled border surrounding central ulcer, may bleed) • Morphoeic or sclerosing (flat hardened plaque of thickened skin, without surface vascularisation, ill-defined border making it difficult to determine area of involvement) Occasional secondary infection or inflammation • overlying purulent discharge or crusting Change in lid contour/redirection or loss of eyelashes Loss of texture of surrounding skin Later cases sometimes pigmented (more prominent in dark skinned races) Differential diagnosis • Squamous cell carcinoma, chalazion, keratoacanthoma, actinic keratosis, molluscum contagiosum, papilloma • Anterior marginal blepharitis can resemble sclerosing BCC • BCC diagnosis is suspected clinically but is usually confirmed by histology which can also help to define the clinical subtype Management by Optometrist Practitioners should recognise their limitations and where necessary seek further advice or refer the patient elsewhere Non pharmacological Document with photography if possible Refer with details of location, size and history (to aid assessment of urgency) Advise patient of possible diagnosis Basal cell carcinoma Version 6, Page 1 of 3 Date of search 21.06.17; Date of revision 19.12.17; Date of publication 06.04.18; Date for review 20.06.19 © College of Optometrists CLINICAL MANAGEMENT GUIDELINES Basal cell carcinoma (BCC) (periocular) Reassure that this is a low risk skin cancer Advise on sun protection measures (GRADE*: Level of evidence=low, Strength of recommendation=strong) Pharmacological None Management Category B1: no treatment by optometrist; routine referral This slow growing neoplasia rarely metastasises but if left untreated could threaten sight (or life if BCC invades beyond orbit) Possible management by Ophthalmologist