The ocular presentation of systemic diseases

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The ocular presentation of systemic diseases Data collected by : Ruwida Mohammed Saeed

The eyes are actually a part of the human body sharing functions with it , and affected by factors leading to its deterioration

It can be your mirror if you knew how to use

Here are some of the main complaints that are commonly faced

Eye presentation Amaurosis FugaxUveitisCataractsAbnormal EyeMovementsPupillary AbnormalitiesRetinal vessel diseaseCorneal effects Eyelids

Amaurosis Fugax = is a painlesstransient monocular visualloss

Amaurosis FugaxEmbolicVasospastic/HaemodynamicNeurologicOcularIdiopathic

Amaurosis FugaxEmbolicVasospastic/HemodynamicNeurologicOcularIdiopathic

Optic neuritis (MS)Migraine

Optic neuritis Rapidly developing impairment of vision in 1 eye or, less commonly, both eyesDyschromatopsia ,may be more prominent than the decreased visionRetro-orbital or ocular pain exacerbated by eye movementUhthoff phenomenon, in which vision loss is exacerbated by heat or exercisePulfrich phenomenon, in which objects moving in a straight line appear to have a curved trajectory: Presumably caused by asymmetrical conduction between the optic nerves

Multiple sclerosis is the most common manifestation (usually unilateral, but may be bilateral) and the presenting feature in about 25% of MS patientsAbout 60% of patients in the 20-40 years age group who present with ON will subsequently develop evidence of systemic demyelinisation !

Amaurosis FugaxEmbolicVasospastic/HaemodynamicNeurologicOcularIdiopathic

Embolic

TIAcardiac/carotid/

Amaurosis FugaxEmbolicVasospastic/HemodynamicNeurologicOcularIdiopathic

Temporal Arteritis

Temporal arteritis is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries.The clinical criteria is :New headache (localized temporal)Abnormalities of the temporal arteries (tenderness or decreased temporal artery pulse) (ESR) of 50 mm/hPositive results of a temporal artery biopsy visual impairment may occur in as many as 60% of patients

The most common cause of vision loss is anterior ischemic optic neuropathy (AION). This results from ischemia of the optic nerve headoptic neuropathy, central retinal artery occlusion,branch retinal artery occlusion diplopia, ptosis,nystagmus, internuclear ophthalmoplegia (INO),and pupillary abnormalities.

Branch retinal vein occlusion in a patient with giant cell arteritis

Eyelids presentation Dermatochalsis Proptosis Ptosis

Proptosis InfectiousOrbital cellulitis . Mucormycosis , Concurrent sinus diseaseInflammatoryThyroidopathyVasculitisWegener granulomatosisChurg-Strauss syndrome

NeoplasticLymphomaLeukemiaMeningiomaGliomaOssifying fibromaMetastatic (breast in women, lung and prostate in men, gastrointestinal, kidney

Dermatochalsis Chronic dermatitis , as of the chronic inflammation leads to recurrent edema and redundancy of the eyelid skin.Thyroid eye disease frequently associated with dermatochalsis and steatoblepharon ,infiltration of the orbital fat and extraocular muscles with immunoglobulin complexes.Chronic renal insufficiency can be associated with periorbital edema. When chronic edema can result in stretching of the eyelid skin and redundancy of the eyelid

1. Soft tissue involvement Periorbital and lid swelling Conjunctival hyperaemia Chemosis Superior limbic keratoconjunctivitis2. Eyelid retraction3. Proptosis4. Optic neuropathy5. Restrictive myopathyTHYROID EYE DISEASE

Soft tissue involvementPeriorbital and lid swellingChemosisConjunctival hyperaemia

Superior limbic keratoconjunctivitis

Signs of eyelid retraction Occurs in about 50%

Bilateral lid retraction Bilateral proptosis Lid lag in downgaze

Proptosis Occurs in about 50% Uninfluenced by treatment of hyperthyroidism Axial and permanent in about 70% May be associated with choroidal folds

Occurs in about 40% Due to fibrotic contracture

Restrictive myopathy

Elevation defect - most common Abduction defect - less common Depression defect - uncommon Adduction defect - rare

Ptosis Any condition causing the upper eyelid to swell may cause ptosisReduced Sympathetic Activity (Horners syndrome ) Partial Ptosis , Miosis , anhydrosis Brainstem stroke or tumor , 33% of patients with brainstem lesions demonstrated Horner syndromeTumors (eg,Pancoast) or infection of the lung apexDissecting carotid aneurysm Demyelinating disease (eg, multiple sclerosis)Central venous catheterization , Chest tubesLymphadenopathy (eg, Hodgkin disease, leukemia, tuberculosis, or mediastinal tumors)Lesions of the middle ear (eg, acute otitis media

Muscular , nerve , neuromuscular junction : myotonic dystrophy , polymyositis, dermatomyositis, myasthenia, LambertEaton syndrome Oculomotor nerve palsy giant cell arteritis, Infection, cavernous sinus thrombosis ,diabetes mellitus Miller Fisher syndrome, associated with antibodies to ganglioside GQ1bIn Miller Fisher syndrome, extraocular muscle involvement is the rule but there are reports of isolated ptosis.

Pupillary AbnormalitiesHorner's syndromeThird nerve palsyHolmes-Adie syndromeArgyl RobertsonPontine stroke

Third nerve palsy

Infectious meningitis - Bacterial, fungal/parasitic, viralCarcinomatous/lymphomatous/leukemic infiltration, granulomatous inflammation (sarcoidosis, lymphomatoid granulomatosis, Wegener granulomatosis)Pituitary adenoma, meningioma, craniopharyngioma, metastatic carcinoma

is aneurological disordercharacterized by dilatedpupil that reacts slowly to light but shows a more definite response to accommodationthe classic signs of Argyll Robertson pupil, are rigidity to light and contraction to accommodationit has been seen with herpes zoster, sarcoid, vonBeonomo's encephalitis, diabetes, Lyme disease, Wernicke's encephalopathy.Tertiary syphilis

Holmes-Adie syndromeArgyl robertson pupil

The corneal changes and the systemic disease UlcerationCollagen vascular disease: rheumatoid arthritis, lupus erythematosus, polyarteritis, Sjgren's disease, Wegener's granulomatosis Sjgren's disease (keratitis sicca, marginal melting)

Alterations in contour / keratoconusAtopic diseasesDown's syndromeMarfan's syndromeDEPOSITSGout , myelomaWilson's disease (dark copper pigment in Descemet's membrane)

The lens changes are presented as cataracts

CATARACTMetabolicOther Systemic DiseasesDrug Induced

DMG6PDMuscularDystrophyNF 2DermatitisSteroids

Two types of cataracts result from diabetesEarlier onset age-related diabeticcataract ,more common type is which occurs in type I or II diabetics. It is very similar to other age-related cataracts, and may present as a cortical, posterior subcapsular

snowflakecataractRare, bilateral, occurs rapidly, and related to very high, uncontrolled DM in young type II diabetics

UveitisSystemic Dis& SyndromesInfectionsInfestationsTBCandidaHerpes Zoster

UveitisSystemic Dis& SyndromesSarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

UveitisSystemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUlcerative colitisJCA

Ulcerative colitis is characterized severe diarrhea and cramps , Fever ,Leukocytosis , Abdominal distention UC is associated with various extracolonic manifestations, as follows:UveitisPyoderma gangrenosumPleuritisErythema nodosumAnkylosing spondylitisSpondyloarthropathies

UveitisSystemic Dis& SyndromesSarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

Systemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisease UCRheumatoid arthritis

UveitisSystemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

Sacroilitis 33% of pts of AS present with acute anterior uveitis

Systemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

UrethritisArthritis

Systemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

Ocular involvement in Behet diseaseOcular involvement is seen in about 70% of patientsIn most cases, the ocular symptoms follow the oral and genital ulcers by 3-4 years, although ocular disease is the initial manifestation in about 20% of cases.The classic finding of Behet disease, iridocyclitis with hypopyon, is present in about one third of cases.Gonioscopy may reveal an occult hypopyon characterised by hypopyon that it may change position with head movement, and it may form and disappear rapidly without sequelae.Recurrent attacks may result in posterior synechiae, peripheral anterior synechiae, iris atrophy, and secondary glaucoma.

A 19-year-old boy presenting with vitreous haze, and retinal infiltrates due to Behets disease.

The classic fundus finding is retinal vasculitisretinal hemorrhages, yellow-white exudates deep in the retina, white focal retinal infiltrates, retinal edema, and optic disc edema with hyperemia. Retinal neovascularization, secondary to either retinal vein occlusion or chronic inflammation, may result in retinal or vitreous hemorrhage.Neovascular glaucomaVitreous cellular infiltration almost always is present during the acute phase.Repeated episodes of posterior segment inflammation cause sheathing of retinal vessels, chorioretinal scars, and retinal and optic nerve atrophy.

UveitisSystemic Dis& Syndromes

SarcoidBehcetsReiter'sAnkylosing Spondylitis Crohn'sDisUCJCA

Unknown Aetiology granuloma

SARCOIDOSISSystemic InvolvementLung lesions 95%Thoracic lymph nodes 50%Skin lesions 30% Eyes 30%

SARCOIDOSISOcular InvolvementConjuncti