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Ocular Surface Disease

Ocular Surface Disease

Authors: Alex Lange Maciej Jesse

Vista Klinik Binningen Table of Contents

• Dry- Syndrome • Nutritional Disorders • Rosacea • • Seborrhoic • Floppy Syndrome • • Superior Limbic Keratokonjunctivitis • • Recurrent Corneal Erosion • Ichthyosis • Persistant Corneal Epithelial Defect • Ectodermal Dysplasia • Neurotrophic Keratopathy • pigmentosum • and Distichiasis • Noninflammantory Vascular Anomalies of • Mucus-fishing Syndrome the • Dellen • Herediatary Hemorrhagic Teleangiectasia • Contact -related Problems • Lymphangiectasia • Limbal Stem Cell Deficiency Dry—Eye Syndrome

• Definition: «Multifactorial disease of the 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 (, conjunctiva, and meibomian glands), 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 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 - 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, , ocular allergy, topical preservative use, contact lens • Epithelial stimulates corneal nerve endings leading to discomfort, increased blinking, compensatory reflex lacrimal tear secretion -> may cause neurogenic inflammation within the 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 , 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 • 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, , 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 ) • 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

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 • , • 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, , 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 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 involvement in up to 50% affected patients: nodules in the eyelid skin and canthal region, conjunctival granulomas, calcific , enlargement of the lacrimal and salivary glands (->KCS), iridocyclitis, , periphlebitis, chronic cystoid , exudative , granulomatous involvement of the • Management: in case of an isolated anterior uveitis topical corticosteroids. In all other cases systemic therapy with prednisolon or immunsupression with methotrexat, azathioprin or infiximab Ichthyosis

• Represents a diverse group of hereditary skin disorders characterized by excesively dry skin and accumulation of scale • Most important forms: Ichthyosis vulgaris, an autosomal dominant trait, and the X-linked ichthyosis • Ocular signs: Eylelid scaling, cicatricial , conjunctival thickening, corneal changes such as opacities, nodular degenerations, vascularization, scarring and band keratopathy • Treatment: hydrating the skin and eyelids, removing scale, and slowing the turnover of epidermis when approciate Ichthyosis

Dry, hard and scaly skin.

Quelle: http://skinawareness.org/tag/ichthyosis/ Ectodermal Dysplasia

• Hereditary condition that displays variable defects in the morphogenesis of ectodermal structures, including hair, skin, nails, and teeth • Component in at least 150 distinct hereditary syndromes • Ocular abnormalities: sparse lashes and brows, blepharitis, , hypoplastic lacrimal ducts, diminished tear production, abnormal meibomian glands, dry conjunctivae, pterygia, corneal scarring and neovascularisation, , and . Changes may be due to limbal stem cell deficiency Ectodermal Dysplasia

Peg shaped teeth, coarse, sparse hair

Quelle: https://upload.wikimedia.org/wikipedia/commons/thumb/ d/d5/Ectodermal_dysplasia.jpg/280px- Ectodermal_dysplasia.jpg Xeroderma pigmentosum

• Recessively transmitted disease characterized by impaired ability to repair sunligt-induced damage to DNA -> skin develops areas of focal hyperpigmentation, atrophy, actinic keratosis, and telangiectasia, cutaneous neoplasms • Ophthalmic manifestations: Conjunctiva with telangiectasia and hyperpigmentation, pingueculae, pterygia, corneal complications with exposure keratitis, ulceration, neovascularization, scarring, perforation, ocular neoplasms, eyelids with atrophy, , trichiasis, scarring, symblepharon, , ectropion Xeroderma pigmentosum

Pigmented and dry skin

Quelle: http://scotdir.com/wp-content/uploads/picdir/xeroderma- pigmentosum_2.jpg Noninflammantory Vascular Anomalies of the Conjunctiva • Causes of conjunctival hyperemia - Inflammation: infection, allergy, toxicity, neoplasia - Direct irritation: foreign body, aberrant eyelashes - Reflex response: eyestrain, emotional weeping - Systemic or topical vasodilators: alcohol, oxygen, carcinoid tumor - Autonomic dysfunction: sympathetic paresis, sphenopalatine ganglion syndrome - Vascular engorgement: venous obstruction, hyperviscosity Herediatary Hemorrhagic Teleangiectasia

• Dominant inherited (but occasionally sporadic) disease with spontaneous hemorrhage from telangiectatic vessels of the skin, nasal and oral mucous membranes, gastrointestinal tract, lungs, and brain • Conjunctival telangiectasias typically involve the palpebral region • Conjunctival bleeding in most instances can be controlled with local pressure Quelle: http://clinicalgate.com/wp-content/uploads/2015/03/u04-07-9781455776443.jpg Lymphangiectasia

• Developmental anomaly or in association with trauma or inflammation • Irregularly dilated, periodically hemorrhage-filled lymphatic channely of the bulbarconjunctiva • Treatment is local excision or diathermy • Must be distinguished from ataxia-telangiectasia (Louis-Bar syndrome), in which the epibulbar and interpalpebral telangiectasia of the arteries lacks an associated lymphatic component Lymphangiectasia

Quelle: http://www.angelarteaga.es/en/imagenes/procesos/tumor_image008.jpg Deficiency

• Vitamin A is an essential fat-soluble vitamin • Results from low diatary intake or decreased absorption • Leads to xerosis (dryness of the conjunctiva and cornea), (night blindness), xerophthalmia • Xerophthalmia responsible for at least 20.000 – 100.000 new cases of blindness worldwide each year. • Systemic , best characterized by , is a medical emergency with an untreated mortality rate of 50% • Ocular manifestation: xerosis, metaplastic keratinization of the conjunctiva (Bitot spots), corneal ulcers and scars, diffuse corneal necrosis (keratomalacia) • Vitamin and protein-calorie supplements, adequate corneal lubrication and prevention of secondary infection Corneal xerosis

Drying of the cornea

Quelle: http://www.cehjournal.org/wp-content/uploads/fig3-corneal-xerosis.jpg Vit. C Deficiency

• Essential vitamin for humans, we lack its synthetic enzyme, L- gulonolactone oxidase • Cofactor in the hydroxylation of lysine and proline in ribosomal collagen synthesis • Deficiency results in unstable collagen fiber formation • In xerosis and impaired wound healing possible Exposure Keratopathy

• Results from any disease process that limits eyelid closure: - Neurogenic diseases such as seventh nerve palsy - Degenerative neurologic conditions such as Parkinson disease - Cicatricial or restrictive eyelid diseases such as ectropion - Drug abuse - Blepharoplasty - Skin disorders such as Stevens-Johnson syndrome or xeroderma pigmentosum - Ocular signs: punctate epithelial keratopathy usually involves the inferior third of the cornea. Epithelial defects, may lead to ulceration, melting, and perforation - Therapy similar to that for severe dry eye Seventh nerve palsy with lagophthalmus

Quelle: http://www.eyecasualty.co.uk/maincontent1/Rh1.jpg Floppy Eyelid Syndrome

• Patients with a flimsy, lax upper tarsus that everts with minimal upward force applied to the upper lid -> contact with the pillow during sleep • Clinical findings: papillae on the upper palpebral conjunctiva, mucus discharge, corneal involvement (punctate epithliopathy, superficial vascularisation, ) • Treatment: Shield or taping eyelids closed at night or performing surgical eyelid-thightening procedures Floppy Eyelid Syndrome

Quelle: http://www.mrcophth.com/oculoplasticgallery/floppyeyelidsyndrom/floppyeyelid3.jpg Superior Limbic Keratokonjunktivitis

• Thought to result from mechanical trauma transmitted from the upper eyelid to the superior bulbar and tarsal conjunctiva. Association with autoimmune thyroid disease • Typically develops in adult women 20-70 years of age • Condition usually resolves spontaneously • Ocular findings: often bilateral, injection and thickening of the superior bulbar conjunctiva, hypertrophy of the superior limbus, superior corneal filamentary keratopathy • Treatment: topical anti-inflammatory agents, large-diameter bandage contact lenses, superior punctal occlusion, thermocauterization of the superior bulbar conjunctiva, resection of the bulbar conjunctiva superior to the limbus, topical ciclosporine, autologous serum eyedrops, and conjunctival fixation sutures Superior Limbic Keratokonjunctivitis

SLK with hypertrophy of the superior limbus, injection and thickening of the superior bulbar conjunctiva.

Quelle. http://webeye.ophth.uiowa.edu/eyeforum/atlas/pages/Superior-limbic--SLK/SLK- LRG.jpg Recurrent Corneal Erosion

• Typically in eyes that have suffered a sudden, sharp, abrading injury or in patients with preexisting epithelial basement membrane dystrophy • Episodes usually last from 30 minutes to several hours • Slit lamp examination: subtle corneal abnormalities, epithelium loosly attached, heaped up, edematous, with pooling of fluorescein over the affected area • Management: frequent lubrication, , 5% NaCl, therapeutic CL, epithelial debridement, anterior stromal micropunction, phototherapeutic keratectomy Persistent Corneal Epitelial Defect Common causes • Herpetic corneal disease • Delayed postsurgical epithelial healing • Chemical burns • Toxicity from topically applied medications • Recurrent corneal erosiones • Dry-eye syndromes • Infections • Neuroparalytic keratopathy • Neurotrophic keratopathy • Anterior segment necrosis Ocular signs and treatment

• Characterized by central or paracentral areas of chronic nonhealing epithelium that resists maximal therapeutic endeavors • Epithelial defects with elevated, rounded edges, sometimes with stromal infiltration and corneal anesthesia. Can progress to vascularization, corneal opacification, scarring, necrosis and thinning of the stroma, perforation • Management: Treatment of the underlying disease, use of the full spectrum of approaches for dry eye and exposure keratopathy Neurotrophic keratopathy

• Results from damage to CN V, which causes corneal hypoesthesia • Damage caused by: surgical trauma (ablation of the trigeminal ganglion, PK, large limbal incisions, LASIK) cerebrovascular accidents, aneurysms, multiple sclerosis, tumors (acoustic neuroma, neurofibroma, angioma), herpes zoster ophthalmicus, keratitis, Hansen disease (Lepra), toxicity of topical medications (anesthetics, carbonic anhydrase inhibitors, NSAIDs, β-blockers), diabetes mellitus, hereditary sensory neuropathy, familial dysautonomia (Riley-Day syndrome) • Involves the central or inferior paracentral cornea • Ocular signs include recurrent epithelial defects, ulcers, perforation • Management: treatment of the underlying disease, use of the full spectrum of approaches for dry eye and exposure keratopathy Trichiasis und Distichiasis

• Trichiasis: eyelashes emerging from their normal anterior origin are curved inward the cornea • Idiopathic oder secondary to chronic inflammatory conditions, most probably the result of subtle cicatrical entropion of the eyelid margin • Distichiasis: congenital or acquired condition in which an extra row of eyelashes emerges from the ducts of meibomian glands • Aberrant eyelashes (eg. by epilation, electrolysis, cryotherapy) and poor eyelid position should be corrected Trichiasis

Quelle: https://opto.ca/sites/default/files/trichiasis_8489112576_70ca9b7a19_o.jpg Distichiasis

Quelle: http://img.medscapestatic.com/pi/meds/ckb/65/30765tn.jpg Mucus-fishing syndrome

• Increased mucus production as a response to ocular surface damage, typically in KCS • Compulsive removal of the mucus strands from the fornix heightens the ocular surface irritation, which stimulates additional mucus production -> vicious circle • Characterized by a well-circumscribed pattern of rose bengal or lissamine green staining on the nasal and inferior bulbar conjunctiva Mucus-fishing Syndrome

Patient "fishing" mucus.

Quelle: https://i.ytimg.com/vi/AH9zKG66avI/hqdefault.jpg Dellen

• Saucerlike depressions of the cornea • Desiccation of the epithelium and subepithelial tissues occurs at or near the limbus adjacent to elevations such as pterygia, filtration blebs, dermoids • Tear-film interrupted by these surface elevations • Treatment with frequent ocular lubrication or pressure patching Dellen

Quelle: http://www.rootatlas.com/wordpress/wp-content/uploads/2007/08/dellencornea.jpg Metabolic epithelial damage

• Due to contact lens overwear • Microcystic epitheliopathy most commonly in patients using extended-wear soft contact lenses • Ocular signs include central epithelial edema, epithelial erosiones, frank ulceration, acute epithelial necrosis Toxic conjunctivitis

• Proteolytic enzymes and chemicals used for cleaning contact lenses (BAC, chlorhexidine, hydrogen peroxide) can cause conjunctival injection, epithelial staining, punctate epithelial keratopathy, erosions and microcysts

• Hypersensitivity reaction to the preservative thimerosal in contact lens solutions • Declined in prevalence as a result of the replacement by other preservatives • Ocular signs include injection of the superior bulbar conjunctiva, epitheliopathy of the cornea and conjunctiva, papillary conjunctivitis, superficial pannus Neovascularization

• Neovascular ingrowth into the peripheral cornea (micropannus) common in soft lens wearers • CL should be discontinued if the neovascularization extends further than 2mm into the cornea • Probably caused by hypoxia and chronic trauma to the limbus • Deep stromal neovascularization associated with extended-wear contact lenses, especially in , symptomatic when secondary lipid deposition appears Pannus

Small pannus extending onto the superior corneal surface.

http://webeye.ophth.uiowa.edu/eyeforum/cases-i/Case77/Ocular- Rosacea-Blurry-Red-Itchy_2B.jpg Limbal Stem Cell Deficiency

• Epithelial cells of the ocular surface are permanently renewed through proliferation of stem cells • Corneal stem cells located in the basal cell layer of the limbus, conjunctival stem cells distributed throughout bulbar surface and fornices • 25-33% of the limbus must be intact to ensure normal ocular resurfacing • In case of stem cell deficiency corneal neovascularisation, conjunktivalisation of the cornea, irregular ocular surface, disturbed epithelial wound healing, recurrent ulcers • Evaluation with impression cytology (corneal surface with the presence of goblet cells and conjunctival epithelium) Etiologic Classification of Limbal Stem cell Deficiency • Idiopathic • Trauma (Chemical/thermal burns) • Iatrogenic (Surgery, radiation, contact lens use, local chemotherapy) • Systemic (Medications, Graft-vs-host disease) • Autoimmune (Stevens-Johnson syndrome, ocular cicatrical pemphigoid) • (Neoplasia und degeneration, neurotrophic keratitis, infections, atopy, peripheral corneal ulcers, anterior segment ischemic syndrome) • Congenital und hereditary (z.B. , multiple endocrine neoplasia, Xerorderma pigmentosa) Managment

• Limbal transplantation from the ipsilateral eye or from an HLA- matched living related donor with a subsequent immune supression Limball Stem Cell Deficiency

Ocular surface failure with neovaskularization and opacification due to an alkali burn

Quelle: http://www.stembook.org/sites/default/files/pubnode/06ab13370d5294906af8f5609d71afc80e09f474/Limbal_e pithelial_stem_cells_of_the_cornea/Secker05.jpg Take home message

• Dry eye syndrome is a very common disease and needs a systematic approach and good patient education for successful therapy • Recurrent erosion syndrome is often misdiagnosed as dry eye syndrome and not properly treated, detailled history and concise slitlamp exam is important • Increasing number of contact lens wearers and improper wearing habits are causing more and more contact lens associated problems (infections, hypoxia, exposure to preservatives) that need proper management

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