Ocular Surface Disease
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Ocular Surface Disease Authors: Alex Lange Maciej Jesse Vista Klinik Binningen Table of Contents • Dry-Eye Syndrome • Nutritional Disorders • Rosacea • Exposure Keratopathy • Seborrhoic blepharitis • Floppy Eyelid Syndrome • Chalazion • Superior Limbic Keratokonjunctivitis • Sarcoidosis • Recurrent Corneal Erosion • Ichthyosis • Persistant Corneal Epithelial Defect • Ectodermal Dysplasia • Neurotrophic Keratopathy • Xeroderma pigmentosum • Trichiasis and Distichiasis • Noninflammantory Vascular Anomalies of • Mucus-fishing Syndrome the Conjunctiva • Dellen • Herediatary Hemorrhagic Teleangiectasia • Contact lens-related Problems • Lymphangiectasia • Limbal Stem Cell Deficiency Dry—Eye Syndrome • Definition: «Multifactorial disease of the tears and ocular surface that results in symptoms of discomfort, visual disturbance, and tear-film instability with potential damage to the ocular surface. It is accompanied by increased osmolarity of the tear film and inflammation of the ocular surface» (DEWS, 2007) • Dry eye represents a disturbance of the lacrimal functionl unit (LFU), consisting of the lacrimal glands, ocular surface (cornea, conjunctiva, and meibomian glands), eyelids and the sensory nerves that connect them • LFU regulates the components of the tear film and responds to environmental, endocrinologic, and cortical influences Quelle: https://openi.nlm.nih.gov/detailedresult.php?img=PMC3587314_IRI-32-19-g001&req=4 Dry – Eye Syndrome • Function of the LFU: - Tear-film integrity (lubricating, antimicriobial, and nutritional roles) - Ocular surface health (maintaining corneal transparency and surface stem cell population) - Quality of image projected onto the retina Mechanism of Dry Eye Core mechanisms • Tear-film hyperosmolarity • inflammatory events leading to cell death by apoptosis • loss of goblet cells • disturbance of mucin expression • tear-film instability • Tear-film instability exacerbates ocular surface hyperosmolarity -> vicious circle Dry Eye Syndrome - vicious circle Figure from AAO, Basic and Clinical Science Course, Section 8 , 2013-2014. Dry-Eye-Syndrome • Other possible reasons for tear-film instability: xerosing medication, xerophthalmia, ocular allergy, topical preservative use, contact lens • Epithelial injury stimulates corneal nerve endings leading to discomfort, increased blinking, compensatory reflex lacrimal tear secretion -> may cause neurogenic inflammation within the lacrimal gland Classification: Major Etiologic Causes of dry Eye 1. Aqueous-deficient dry eye 2. Evaporative tear dysfuntion 3. Effect of environment Figure from AAO, Basic and Clinica Science Course, Section 8 , 2013-2014. Effect of the environment • internal (low blink rate behavior, wide lid aperture gaze position, aging, low androgen pool, systemic drugs) • external (low relative humidity, high wind velocity, occupational environment) Dry-Eye Severity Grading Scheme Severity level 1 2 3 4 Discomfort, severity, Mild and/or episodic, Moderate episodic or Severe frequent or Severe and/or disabling frequency occurs under chronic, stress or no stress constant without stress and constant environmental stress Visual symptoms None or episodic mild Annoying and/or activity Annoying, chronic and/or Constant and/or possibly fatigue limiting episodic constant, limiting activity disabling Conjunctival injection None to mild None to mild +/- +/++ Conjunctival staining None to mild Variable Moderate to marked Marked Corneal staining None to mild Variable Marked central Severe punctate erosions Corneal/tear signs None to mild Mild Debris, ↓meniscus Filametary keratitis, Filamentary keratitis, mucus clumping, ↑tear mucus clumping, ↑tear debris debris, ulceration Lid/meibomian glands MGD variably present MGD variably present Frequent Trichiasis, keratinization, symblepharon TBUT (sec.) Variabele ≤10 ≤5 Immediate Schirmer score (mm/5min) Variabele ≤10 ≤5 ≤2 Dry Eye Workshop 2007 Oc Surf. 2007;5(2):75-92. Sjögren Syndrome • Can be divided into 2 clinical subsets: Primary SS and Secondary SS • Primary SS includes patients who either have illdefined systemic immune dysfunction or lack any evidence of immune dysfunction or conective tissue disease • Secondary SS occurs in patients with well-defined, generalized connective tissue disease. Most commonly associated with rheumatoid arthritis • Precise cause of aqueous tear deficiency (ATD) in SS is unknown, generally considered to be a T-cell-mediated inflammatory disease leading to destuction of the lacrimal gland • Lacrimal gland cytology: focal oder diffuse lymphocytic infiltration Sjögren Syndrome Quelle: http://dgrh.de/?id=1679 Quelle: http://www.dermis.net/dermisroot/de/39368/image.htm Non-Sjögren Syndrome • ATD due to disease of lacrimal gland, lacrimal gland obstruction, or reflex hyposecretion • Primary lacrimal disease: Rilay-Day syndrome (familial dysautomia), congenital alacrima, abscence of the lacrimal gland, anhidrotic ectodermal dysplasia, Adie syndrome, idiopathic autonomic dysfunction (Shy-Drager syndrome) • Secondary lacrimal disease: sarcoidosis, chronic graft-vs-host disease, HIV, xerophthalmia, and surgical ablation of the lacrimal gland, obstruction of lacrimal outflow (cicatricial conjunctivitis) • As a result of interruption of either the afferent or efferent limb of the reflex arc (caused by eg, HSV, VZV, contact lens wear, peripheral neuropathies, surgical disruption, PRK, PKP, ECCE, aging) Meibomian Gland Dysfunction • Result of progressive obstruction of the meibomian gland orifices due to keratinization -> reduction of lipid delivery to the ocular surface and increased inflammation of the eyelid charcterized by hyperemia of the eylelid margin and tarsal conjunctival surface. Possible meibomian gland dropout or displacement of the meibomian orifices. • MGD classified into: obstructive, resulting from blepharitis, acne rosacea, and pemphigoid and hypersecretory, resulting from meibomian seborrhea • Leads to lipid tear deficiency, which results in tear-film instability, increased rate of tear-film evaporation and elevated tear osmolarity • Management: Eyelid hygiene, systemic tetracyclines, topical cortisteroids, systemic omega-3 fatty acid supplements Meibomian Gland Dysfunction Quelle:http://www.eyecarepartners.co.uk/meibomian-gland-dysfunction-mgd.html Recommended Treatment for Aqueous Tear Deficiency Severity Therapeutic Options Mild Artificial tears up to 4x/d Lubricating ointment at bedtime Hot compresses and eyelid massage Moderate Artificial tears AT 4x/d to hourly Lubricating ointment at bedtime Cyclosporin A AT 0.05% 2x/d Reversible punctum plugs, lower puncta Severe All of the above Punctum plugs (lower and upper) Topical serum drops (20%) 4-6x/d Topical corticosteroids Moist environment Tarsorrhaphy Bandage lenses (rarely) Rosacea Chronic acneiform disorder that can affect both the skin and eyes Facial lesions • Teleangiectasias • Recurrent papules and pustules • midfacial erythema • +/- rhinophyma Quelle: https://www.aad.org/public/diseases/acne-and-rosacea/rosacea Ocular signs • Chronic conjunctivitis • excessive sebum secretion • marginal corneal infiltrates • chronic blepharitis • sterile ulceration • eyelid margin telangiectasia • episcleritis, • meibomian gland dysfunction • iridocyclits • recurrent chalazia Rosacea Causes - No proven cause - Flushing can be triggered by exposure to temperature extremes, strenuous exercise, sunlight, sunburn, stress, anxiety, cold wind, spicy foods and alcohol Management - Systemic tetracyclines, doxycycline, minocycline - In addition topical metronidazole 0.75% gel or 1% cream. - Topical corticosteroids in case of noninfectious ulcerative keratitis Seborrhoic Blepharitis • Seborrhoic Blepharitis may accur alone or in combination with staphylococcal blepharitis or MGD • Ocular signs: inflammation primarly at the anterior eyelid margin, crusting on the eyelids, eyelashes, eyebrows, scalp, increased meibomian gland secretion, associated keratitis or konjunctivitis, ATD • Management: eyelid hygiene, treatment of scalp disease (coal tar- based shampoos), topical corticosteroids , systemic antibiotics (e.g. doxycycline) in case of posterior blepharitis (eg, MGD). In case of bacteria caused blepharitis (eg, staphylococcus) topical antibiotic ointment (bacitracin, bacitracin-polymyxin B) Seborrhoic Blepharitis Quelle: https://www.google.ch/search?q=seborrheic+blepharitis&biw=1301&bih=620&source=lnms&tbm=isch&sa=X&sqi=2&ved=0ah UKEwj2gv-g3eXOAhWHWhQKHVXUDSMQ_AUIBigB#imgrc=m1T_nfRBWy_KpM%3A Chalazion • Localized lipogranulomatous inflammation involving either the meibomian or zeiss glands • Nodules develop slowly and are painless, overlying skin is erythmatous • Lesion disappears in weeks to months, a small amount of scar tissue may remain • Occasionally blurred vision secondary to astigmatism induced by chalazion • CAVE: Basal cell, squamous cell, sebaceous cell carcinoma can masquerade as chalazia -> histopathologic examination of atypical chalazia • Management: hot compresses, expression of the meibomian gland, intralesional injection of a corticosteroid, incision or drainage Chalazion Quelle:https://www.google.ch/search?q=chalazion&biw=1301&bih=620&source=lnms&tbm=isch&sa=X&sqi=2&ved=0ahUKEwiZur mB3uXOAhVEPRQKHUnZDJ8Q_AUIBigB#imgrc=av_Dl0kWQoOj_M%3A Sarcoidosis • Multisystem disorder characterized by the development of noncaseating granulomatous inflammation in affected tissues • Etiolgy seems to be linked to a genetically predetermined enhacement of cellular immune responses to a limited number of microbial pathogens • Ocular