OCULAR SURFACE DISEASE

OCULAR SURFACE DISEASERuth Antolin 1st YearCOMMON CLINICAL FINDINGSEyelids

Common Clinical Findings of the External Eye ExamTissueFindingDescriptionMaculeSpot of skin color changePapuleSolid raised spotVesicleBlister filled with serous fluidBullaLarge blisterPustulePus filled blister

KeratosisScaling from accumulated keratinizing cellsEczemaScaly crust on a red baseErosionExcoriated epidermal defectUlcerEpidermal erosion with deeper tissue loss

Conjunctival SignsPapillaeElevated, polygonal, hyperemic moundsVascular changes seen on the palpebral conjunctivafibrous septa: anchor the conjunctiva to the tarsusHas a central red dot: dilated capillarySmooth velvety appearance With progression: surrounded by capillaries Chronic/ progressive changes: vascular tufts which obscures the underlying vesselsGiant papillae: > 0.3mm dm

FolliclesRound or oval clusters of lymphocytesFollicular conjunctivitis: involves redness and new or enlarged folliclesVessels surround and encroach the raised surfaces seen usually in the inferior and superior tarsal conjunctiva

Table 1-1. Conjunctival SignsFindingExamplesPapillary conjunctivitisAllergic BacterialFollicular conjunctivitisBacterial (Molluscum contangiosum, Chlamydia)Viral (HSV, Adenovirus)Drug inducedConjunctival pseudomembrane/ membraneSevere viral/ bacterial conjunctivitis*SJSChemical burnConjunctival granulomaCat scratch diseaseSarcoidosisForeign bodyConjunctival erosion/ ulcerationSJS Mucous membrane pemphigoidGraft vs. Host diseaseFactitious conjunctivitis

Corneal Signs

CorneaKeratitisCan be described according to the followingDistribution: diffuse, focal, multifocalDepth: epithelial, subepithelial, stromal, endothelialLocation: central, peripheralShape: dendritic, disciform etcCorneaPunctate Epithelial Keratopathy: non specific term that includes a spectrum of biomicroscopic changesPunctate epithelial Granularity - erosive and inflammatory changes

Punctate Epithelial Erosions: staining lesions of abnormal and degenerated corneal epithelial cells

Stromal inflammation: presence of new blood vesselsEpithelial defectDirect interlamellar infiltrationEndothelial injury

CorneaStromal inflammation Suppurative vs. non suppurativeDistribution: focal vs non focalLocation: central, paracentral, peripheral

Necrotizing stromal keratitis is a severe form of infiltrate without liquefaction associated with suppuration

Endothelial dysfunctionInflammatory pseudoguttae: dark areas of normal mosaic patternKeratic precipitates: clumps of inflammatory cells during active inflammationFibrin, clumped proteinsNeutrophils and lymphocytes: punctate opacitiesMacrophage: Large mutton fat clumps

CorneaProduction of corneal scar is said to be brought about by:Altered stromal keratocytesAccumulation of different complexes: calcium, lipids, proteinaceous material

NeovascularizationSuperficial stromal blood vessels from palisades of VogtPannusUsually remain at a single lamellar plane unless stroma becomes disorganized

Inflammation can lead to corneal opacification and is said to be brought about by altered stromal keratocytes that produce disorganized collagen fibers which consequently scatter light, and are non transparentCLINICAL APPROACH TO DRY EYE

16 of 80Dry EyeMulti factorial diseaseDiscomfort, visual disturbance and tear film instability with potential damage to ocular surfaceDisturbance of the LFULFU regulates the major components of tear film Responds to environmental, endocrinologic and cortical influencesOverall functionsTear film integrity (lubricant, antimicrobial, nutritional)Ocular surface health: maintains corneal transparency and surface stem cell populationImage qualityDry EyeMost common reason for ophthalmic consultPrevalence increases with ageF > MEqual amongst all racial and ethnic groups

It is associated with poor quality of life especially those patients with moderate to severe dry eyes. MECHANISM OF DRY EYEDriven by:Tear hyperosmolality breakdown of the surface epitheliumTear film instability xerophthalmia, allergy, contact lens wear, diet, prolonged use of video usage, prolonged topical medications with preservativesObstructed tear delivery (cicatricial conjunctival scarring, loss of sensory reflex drive to the lacrimal gland)InflammationEpithelial injury

*Hyperosmolality stresses the corneal epithelium, leading to release of inflammatory mediators (cytokines) subsequently disrupting the junctions between epithelial cells * Aqueous deficient vs EvaporativeAqueous Tear DeficientEvaporativeInflammation of the lacrimal glandDiminished tear productionPropagation of inflammatory mediators on the ocular surface

Primary abnormality is meibomian gland dysfunctionObstruction of the glandsAltered lipid metabolism

Tear film instabilityTear evaporationTear hyperosmolalityINFLAMMATIONAQUEOUS TEAR DEFICIENCYMild to severe disabling diseaseSymptoms tend to be worse toward the end of the dayProlonged use of eyesExposure to environmentSymptoms:Burning, dry sensation, photophobia, blurred visionStare test: blurring of vision below 8 secondsSigns:Hyperemia, decreased tear meniscus, irregular corneal surface, tear film debrisAQUEOUS TEAR DEFICIENCYAssess inferior tear meniscus: 1.00mm height and convex (0.3mm abnormal)(+) epithelial keratopathy : rose Bengal/ lissamine greenNasal/ temporal limbus, inferior paracentral corneaFilaments and mucous plaquesMarginal and paracentral thinningBand keratopathyKeratinization of the cornea and conjunctiva

Filaments strands of epithelial cells attached to the corneal surface over a core of mucus(painful)

TREATMENTImperative to carefully examine the eye for other structural disorders such as conjunctivochalasis, floppy eyelid, superior limbic keratoconjunctivitisDetermine if with systemic symptoms/ medications Artificial tear supplementation, topical cyclosporine, topical steroids, omega 3 fatty acid supplementsTREATMENT: MEDICAL MANAGEMENTAqueous Tear DeficiencyLifestyle change: smoking cessation, environment; use of moisture shields/ humidifier; discontinuation of medicationsTopical tear substitutes: mainstay of treatmentPreservative free is usually recommendedDemulcent solutions: hypromelloseBrief substitute for glycoproteins lost late in the disease processBut cannot be used for restoration of lost glycoproteinsTopical cyclosporine 0.05%: anti inflammatory; used early in the diseaseIn combination with steroidsDilute solutions of hyaluronic acid and autologous serum drops: trophic functions

Serum drops: persistent epithelial defects, neurotrophic keratopathyTREATMENT: MEDICAL MANAGEMENTFilamentary keratopathy: acetylcysteine 10% Topical low dose steroids, cyclosporine, tacrolimusContact lenses: therapeutic, soft, scleral Use of goggles, shields, moisture bubblesPharmacologic stimulation of tear secretion: pilocarpine and cevimelineApproved for xerostomia, long term benefit ?Diet: omega 3 fatty acids

Recommended Treatment for Aqueous Tear DeficiencySeverityTherapeutic OptionsMildArtificial tears with preservatives 4x/ dayLubricating ointment at bedtimeHot compresses and eyelid massageModerateArtificial tears without preservatives 4x/ day to hourlyLubricating ointment at bedtimeTopical anti inflammatory treatment (cyclosporine 0.05% 2x daily)Reversible occlusion, lower puncta plugsSevereAll of the abovePunctal occlusion, lower and upperTopical serum drops 20% 4-6x dailyTopical corticosteroids (non preserved)Moist environmentTarsorrhaphyBandage LensesEVAPORATIVE DRY EYESymptoms: burning, foreign body sensation, redness of the lids and conjunctiva, filmy vision worse in the morningConfined to the posterior eyelid margin: irregular, prominent blood vessels (telangiectatic)/ brush marksMetaplasia of the Meibomian glandsWhite plug of keratin protein found within the glandular orificeSecretions are turbid and more viscous

EVAPORATIVE DRY EYENon obvious obstructive MGDSymptomatic; no obvious clinical signs of meibomian diseaseMild compression of the glands: obstruction; filamentous secretion due to narrowing of the ductsAtrophic meibomian glandsShortening/ absence of the vertical lines of the MGsOther tear findings: Foam in tear meniscusLinear staining along inferior cornea and inferior conjunctivaEpiscleritisInfiltrates: marginal epithelial and subepithelialPannusCorneal scarring/ thinning

TEAR BREAKUP TIMEFunctional measure of tear stabilityIn MGD: rapid TBUT- less than 10 secondsFlourescein strip is used, applied to tarsal conjunctiva, then tear film evaluated with blue filter light using broad beamNo manipulation should be done prior/ instillation of any eye drops

TREATMENT: MEDICAL MANAGEMENTEvaporative Dry EyeBased on stagingEyelid hygiene: mainstay Warm compress, massage, cleaning of the eyelid margin (non irritant shampoo/ blephagel/ dilute sodium chloride solution 1tsp salt: 1 pint waterTopical antibiotics: ophthalmic azithromycinSystemic antibiotics: tetracyclines; long term Side effects: photosensitization, GI upset, azotemia; long term use: oral/ vaginal candidiasisContraindicated: children below 8 yearsAzithromycin is a lipophilic antibiotic- reduces production of bacterial lipases and improves composition of meibomian lipids;TREATMENT: MEDICAL MANAGEMENTTopical steroids: moderate to severe inflammation; corneal infiltrates/ vascularizationDiet: omega 3 1000mg 3x a day- not yet establishedGentle pulsatile pressure and thermal energy increased blood flow to the lids, open obstructed MGs

TREATMENT: SURGICAL MANAGEMENTFor patients with severe disease not refractory to meds or are impracticalPunctal occlusion: collagen implants, silicon punctal plugsCan be dislodgedCan be inadvertently inserted into the nasolacrimal systemComplications: conjunctival abrasion, granuloma formation, reduction in tear flowIrreversible punctal occlusion: disposable cautery, radiofrequency probeIndications: minimal basal tear secretion, punctate keratopathy with no significant ocular inflammation/ infectionCorrection of eyelid malpositionTreatment Algorithm for Meibomian Gland DysfunctionStage INO SYMPTOMS of ocular discomfortTx: Patient education (causes) and symptomatic management; eyelid hygiene, warm compressStage 2MINIMAL TO MILD symptomsMINIMAL TO MILD clinical signsscattered along the eyelid marginTx: Modify environmental factors, eyelid hygiene, topical meds (eye lubricants, azithromycin, emollient lubricants, oral tetracycline derivatives)

Treatment Algorithm for Meibomian Gland DysfunctionStage 3MODERATE symptoms with limitation of activitiesMODERATE signsIncreased eyelid margin features: plugging, vascularityTx: all of the above plus oral tetracycline, lubricant ointment at bedtime, (+) anti inflammatory therapy for dry eyeStage 4MARKED symptoms with definite limitation of activitiesSEVERE signs: dropout, displacement, increased signs of inflammation with hyperemia, and/or phlyctenulesTx: all of the above + anti inflame therapy

Treatment Algorithm for Meibomian Gland DysfunctionPlus diseaseCan be secondary to MGD but can also occur incidentallyExacerbated inflammatory ocular surface diseaseMucosal keratinizationPhlyctenular keratitisTrichiasisChalazionAnterior blepharitis (also includes demodex)Tx: steroids, BCL/ SCL, epilation, cryotherapy, Topical antibiotcs, tea tree oil scrubs

OTHER EYELID DISEASES ASSOCIATED WITH OCULAR SURFACE DISORDERSRosaceaPathogenesisChronic, acneiform disorder Skin and eyeNo proven cause; may be related to overexpression of cathelicidin antimicrobial keratitis Cutaneous sebaceous gland dysfunction of the face, neck and vit hemMore common in fair skinnedClinical PresentationExcessive sebum secretions with chronic blepharitisEyelid margin telangiectasia; MG distortion- chalaziaOcular surface involvement- conjunctivitis, marginal corneal infiltrates, ulceration, episcleritis, iridocyclitis, *corneal neovascularization, scarring

Cathelicidins- neutrophil recruitment, angiogenesis anad cytokine release

RosaceaClinical Presentation30- 60 years oldF > MFacial lesions: telangiectasia, recurrent papules, pustules, midfacial erythemaMalar rash associated with consumption of alcoholRhinophyma- thickening of the skin and connective tissue of the nose

Rhinophyma- thickening of the skin and connective tissue of the nose; occurs late in the disease processTetracyclines: anti inflammatory properties ( leukocyte migration, reduced production of NO and by products, RosaceaTx:Systemic tetracyclines (doxycycline, minocycline)Topical metronidazole/ azelaic acid gel for facial erythemaPapulopustular rosaceaUlcerative keratitis (sterile)- topical corticosteroidsScarrring and neovascularization- poor prognosis (PKP); conservative managementEyelid erythema- light pulse treatment

Seborrheic BlepharitisOccur alone or in association with staphylococcal blepharitis or MGDOccurs at the anterior eyelid marginCrusting Eyelids, eyelashes, scalp, eyebrowsTurbid secretion of the meibomian glandBurning sensation, chronic eyelid redness and FB sensation15%: Keratitis: punctate epithelial erosions1/3 have evaporative dry eyeTx: eyelid hygieneSelenium sulfide for patients with scalp disease

Staphylococcal BlepharitisCaused usually by S. AureusBacterial infection of the eyelids, conjunctivaYounger population Sx: burning, itching (AM)FB sensation and crusting upon awakeningSigns:

ScalesMatted crusts per ciliaUlcers Eyelid margin injection and telangiectasia

PoliosisMadarosisTrichiasis

Ulcers when crusts are removedStaphylococcal BlepharitisMay present as chronic ( > 4 week duration) conjunctivitisPapillary reaction of the tarsal conjunctiva (inferior)Bulbar, tarsal injection Scant mucopurulent dischargeATD/ lipid induced tear film instabilityS. AureusMatted golden crustsulcers inferior punctate keratopathymarginal corneal infiltrates

Phlyctenules: cell mediated/ delayed hypersensitivity caused by antigens found in the cell wall of staphylococcusStaphylococcal BlepharitisConjunctival/ corneal phlyctenules (M. tuberculosis)UnilateralLimbusRound, elevated, yellowish to grayish, hyperemic with zone of engorged vesselM. lacunataChronic angular blepharoconjunctivitis- associated with S. aureusStaphylococcal BlepharitisLaboratory evaluationinitial diagnosis is questionablerefractory to treatmentChronic unilateral casesMasquerade syndrome (conjunctival malignancy)Factitious illness Lab finding: (+) heavy confluent growth of S. AureusOther bacterial growth does not exclude the diagnosisPEK, marginal infiltrates, phlyctenulosisSusceptibility testing: refractory to empiric treatment

Staphylococcal BlepharitisTx: eyelid hygiene (base of lashes)Topical bacitracin, erythromycin or azithromycinAntibiotic treatment: narrow spectrum antibimicrobial effective against staph Anti inflammatory treatment: mild doses of topical corticosteroids*for epithelial defects, diagnostic cultures should be ascertained before steroid treatment

Hordeola and ChalaziaHordeola- painful, tender, red nodular masses near the eyelid marginExternal/ styes: glands of ZeisInternal: meibomian gland inspissationSelf limiting (1-2 weeks)

S. Aureus

HordeolaTx:Warm compress with massageAntibx ONLY if with infectious blepharoconjunctivitisSystemic: eyelid cellulitisOral doxycycline: chronic meibomitis

Hordeola and ChalaziaChalazion- evolved internal hordeolaChronic lipogranulomatous nodules (MG/ Zeis)Weeks to monthsBlurring of visionMalignancy must be ruled out!Tx:Intralesional injection of corticosteroid (0.1-0.2 triamcinolone 40mg/ml)Small, eyelid, multipleDark skin: depignmentationIncision and drainageDrainage and curettageInternal chalazia : vertical incisionsRecurrent: biopsy

STRUCTURAL AND EXOGENOUS DISORDERS ASSOCIATED WITH OCULAR SURFACE DISORDERSExposure KeratopathySecondary to lagophthalmosproptosisPunctate epithelial keratopathy (inferior)Coalesce: ulceration, melting, perforationSigns and symptoms similar to dry eye + corneal anesthesiaTx:Non preserved artificial tears AM; ointment PMLid tapingPermanent/ Temporary TarsorrhapyCorrection of eyelids for long standing eyelid malpositionGold or platinum weightsComplications: infection, shifting, extrusion, astigmatism, ptosis, inflammatory response

Floppy Eyelid SyndromeUsually seen in obese patientsFlimsy, lax upper tarsus that everts with minimal upward forceRepeated eversion of the eyelid during sleepSigns:PapillaeMucus dischargeCorneal involvement: Punctate epitheliopathy (mild); vascularizationKeratoconusTx: Metal shieldLid tapingTarsorrhapy

Superior Limbic KeratoconjunctivitisMechanical trauma from upper eyelid to the superior bulbar and tarsal conjunctivaAutoimmune thyroid disease associated chronic, recurrent condition of ocular irritation and redness20- 70 years of ageSelf limiting, recur over 1-10 yearsBilateral; 1 eye more severely affected than the otherSigns: Fine papillary reaction on superior tarsal conjunctivaInjection and thickening of superior bulbar conjunctivaHypertrophy of superior limbusFine punctate lesions and filamentary keratopathy (superior);

Superior Limbic KeratoconjunctivitisDiagnosticsHistologic:Hyperproliferation, acanthosis, loss of goblet cellsKeratinizationNuclear pyknosis with snake nucleiIncreased epithelial cytoplasm-nucleus ratio Thyroid panelTx: Topical anti inflammatoryBCLSuperior punctal occlusionThermocauterization of superior bulbar conjunctivaAutologous serum dropsAmnion/ conjunctival transplant

Recurrent Corneal Erosionhistory of trauma (sudden, sharp, abrading injury) pre existing epithelial basement membrane dystrophyRecur (days to years) spontaneously, periodicallyPoor adhesion of the epithelium Abnormality in the epithelial basement membrane and filament networkUpregulated gelatinase activity (MMP2- MMP9)MGD

Recurrent Corneal ErosionSx:Sudden onset of eye pain at night or upon first awakeningRedness, photophobia, tearingOcular discomfort out of proportion to the degree of observable pathologySlitlamp exam: epithelial cystPost traumatic vs dystrophic erosionExamine contralateral eye after maximal dilationIdentification of loosely adherent epithelium by applying pressure with surgical sponge after instillation of topical anesth - primary

Recurrent Corneal ErosionTx: Acute phaseEye lubricants non preservedAntibiotic ointmentsAM: Hypertonic saline solution (5%)PM: ointment (6-12 months)- promotes proper epithelial attachmentLow dose oral doxycyclineTopical corticosteroidsTopical antibiotics (broad spectrum)BCL: flat base and high oxygen transmissibilityTopical broad spectrum antibx 3-4x daily

Recurrent Corneal ErosionRecalcitrant casesAnterior stromal micropuncture: promotes basement membrane formationDystrophic, degenerative, severe secondary basement membrane dseEpithelial debridement: procedure of choiceAnesthesiaDebridement of loose epithelium using surgical sponge/ surgical blade/ spatulaApplication of ophthalmic diamond burrTopical antibx, cycloplegics, BCL application until Re epithelialization is complete Excimer laser phototherapeutic keratectomy: Recalcitrant recurrent erosions

Using a 25 gauge needle clinician makes numerous superficial puncture wounds firm adhesion between epithelium and stromaNeurotrophic KeratopathyCaused by:damage of CN VLeading to corneal anesthesia/ hypoesthesiaHerpetic keratitisDrugsDiabetic neuropathyCentral or paracentral areas of non healing epitheliumElevated, round/ oval, grayish edges with underlying stroma inflammationInferior, inferonasalBells phenomenonVascularization, corneal opacification, scarring Perforation

Topical anestheticsTopical NSAIDSTrifluridineB- blockersCAI inhibitorsDrops with BAK

Causes of Neurotrophic Keratopathy and Persistent Epithelial DefectsDamage to CN VCVAAneurysmsMSTumorsHerpes simplex keratitis/ herpes zoster keratitis LeprosyToxicity to topical medications (topical NSAIDs, b blockers, CAIs trifluridine)DM Familial dysautonomia (Riley Day syndrome)Neurotrophic KeratopathyTx: Discontinuation of offending agentEye lubricants preservative freeAutologous serum drops temporary or permanent punctal occlusion (dry eyes)Patching: contact lenses (low water content, highly oxygen permeable)Tarsorrhapy (lateral, medial)Systemic tetracyclines- has specific activity agains MMPsCrosslinkingAmnion graftingConjunctival flaps (last resort)

Trichiasis and DistichiasisTrichiasis: eyelashes from normal anterior origin curve inward toward the corneaSecondary to cicatricial entropion, other inflammatory conditionsIdiopathicDistichiasisExtra row of eyelashes emerges from MG ductsCongenital (AD) or acquiredTrachomaMucous membrane pemphigoidSJSChronic blepharitisChemical burns

Trichiasis and DistichiasisTxEpilationElectrolysisCryotherapy: eyelid margin thinning, loss of adjacent normal lashes, persistent lanugoShould be limited to -20 C for less than 30 secsTarsotomy with eyelid margin rotation

Factitious Ocular Surface DisordersFactitiousSelf induced injuries, intentional, assume a sick rolePatients are usually have knowledge of medicine or work in a medical settingFactitious conjunctivitis: mechanical injury to the inferior and nasal quadrants of the cornea and conjunctivaLocal anesthtetics inhibit epithelial migration and divisionLoss of microvilli and intercellular connections, swelling of mitochondria and lyososomes

Factitious Ocular Surface DisordersMucus fishing syndromeWell circumscribed pattern of rose Bengal/ lissamine green (nasal/ inferior bulbar conj)Increased mucus productionKCS inciting eventPatients demonstrate vigorous eye rubbing and compulsive removal of mucus strands from fornix (mucus fishing)

Factitious Ocular Surface DisordersTopical anesthetic abuseAnesthetics: inhibit epithelial migration and divisionFailure of corneal abrasions or infectious keratitis to respond to appropriate therapyDefects look similar to a neurotrophic appearanceMimic infectious course: KPs, hypopyonStromal edema, stromal infiltrates, large ring opacityRemoval of anesthetics

DellenDessication of the epithelium and sub epithelial tissues due to poor ocular surfaceInability to wet areas near elevated ocular surfaceSaucerlike depressions Epithelium shows punctate irregularities overlying a thinned area of dehydrated corneal stromaTx: Eye lubricantsPressure patchingScleral dellenPost op complication of pterygium removal with the use of MMCDelayed conjunctival wound closure

LIMBAL STEM CELL DEFICIENCYLimbal Stem Cell Deficiency

Limbal epithelial stem cells reside in the basal layer of the epithelium (Ep), which undulates at the limbus. Daughter transient amplifying cells (TACs) divide and migrate towards the central cornea (arrowed) to replenish the epithelium, which rests on Bowman's layer (BL). Limbal Stem Cell Deficiency25- 33% of the limbus must be intact to ensure normal ocular resurfacingNormal limbus: acts as a barrier against corneal vascularization as well as invasion of conjunctival cells from the bulbar surfaceAbsence of the limbal stem cells Ocular surface compromised migration of conjunctival cells Clinical presentation: recurrent ulceration and decreased visionCorneal neovascularization

Ocular surface is irregular, permeable epithelium

Limbal Stem Cell DeficiencyPrimary: PAX6 gene mutations (aniridia)ectrodactyly-ectodermal dysplasia clefting syndromeSclerocorneakeratitis-ichthyosis deafness syndromecongenital erythrokeratodermiaSecondary: chemical burnsRadiationcontact lens wearocular surgerymucous membrane conjunctivitis (trachoma)Pterygiumtopical medsneoplasm of the limbusLimbal Stem Cell DeficiencyTx: Mild casesDiscontinuation of inciting agent Topical steroidsSectoral: epithelium debridementSevere casesScleral contact lensLimbal transplant: contralateral eye, allograft Systemic immune suppression

SYSTEMIC CONDITIONS ASSOCIATED WITH OCULAR SURFACE DISORDERSSjogren SyndromeAssociated with hypergammaglobulinemia, RA, circulating antibodiesT cell mediated inflammatory disease leading to destruction of the lacrimal glandsSalivary glands and other mucous membranes involvementPrimaryLacks any systemic immune dysfunction/ connective tissue diseaseSecondaryWell defined generalized connective tissue disease- RA

IchthyosisHereditary skin disorders characterized by excessive dryness of skin and accumulation of scalesDiagnosed at 1st year of lifeIchthyosis vulgaris- most common hereditary scaling disorder, AD1: 250-300Ocular involvement varies with form of ichthyosisSSx: eyelid scaling, cicatricial ectropion, conjunctival thickeningPrimary corneal opacities: 50% with X-linked ichythyosisTx: hydration of the skin and lidsRemoval of scales

Ectodermal DysplasiaPresence of abnormalities at birthNon progressiveDiffuse involvement of the epidermis + 1 appendage (hair, nails, teeth, sweat glands)Various inheritance patternsOcular abnormalities:Sparse lashes, browsBlepahritisAnkyloblepharonHypoplastic lacrimal ductsCorneal scarringOcular surface disease secondary to limbal deficiency

Xeroderma PigmentosumImpaired ability to repair sunlight induced damage to DNAFocal hyperpigmentation, atrophy, actinic keratosis, telangiectasiaSkin neoplasmOphthalmic and Eyelid Photophobia, tearing, blepharospasmConjunctiva: dry, hyperpigmentedOcular neoplasm- 11%Pterygium and pingueculaCorneal complications

AtrophyMadarosisTrichiasisScarring SymblepharonEntropion/ ectropionLoss of lidsVitamin A DeficiencyXerosisSystemic: loss of mucus production in epithelial cells of GI, GU and respiratory tractsDryness of the conjunctiva and corneaLoss of mucus production of goblet cellsBitot spot: gray, triangular area on the bulbar conjunctivaKeratinized epithelium, inflamm cells, debris and Corynebacterium xerosisProlonged: corneal ulcers and scars diffuse corneal necrosis (Keratomalacia)3 stagesXIA conjunctival xerosis w/o Bitot spotXIB conjunctival xerosis w/ Bitot SpotX2 Corneal xerosisX3 Corneal ulceration with keratomalaacia X3A less than 1/3 corneal surfaceX3B more than 1/3

Not limited to the eye. Can also affect epithelial cells of GI, GU, and respiratory

Vitamin A DeficiencyXerophthlamia: 20000-100000 new cases of blindness annualyHighest risk: malnourished infants born to vitamin A deificient mothersHave had measles, diarrheaSuperficial concurrent infections: herpes simplex, measles, bacterial infectionsVitamin A deficiency : dietary practices, lifestyle related, malabsorption, surgeryKeratomalacia: systemic vitamin A deficiencyPx present with PEM Tx: Vitamin and protein calories supplements Eye lubricants, topical antibiotics THANK YOU!