Ocular Manifestations of Systemic Disease (DOC)

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					ICM 8:00 – Dr. Clint Gregg                                                                 Scribes: Michelle Umengan
Friday March 30, 2001                                                                               Peter Nguyen

                                       Ocular Manifestations of Systemic Disease

Scribe note: This scribe includes everything written in the PPT slides and everything said in class. I used the format
and headings of his slides.

Categories of Systemic Disease
    o Congenital, traumatic, vascular, neoplastic, autoimmune, idiopathic, infectious, metabolic/endocrine,
        drugs/toxins

Eye Examination
    o Vision
    o External – Look at eyelids, make sure everything looks normal.
    o Pupils
            Relative afferent papillary defect – (this is in the PPT slide, but he didn’t say anything about it in
                 class)
            See if pupils are reacting normally
    o Motility – Have the patient look up, down, right and left.
    o Exam of anterior segment
    o Dilated ophthalmoscopy – Examine retina, optic nerve, and vessels.
    o Visual fields – Place you finger in different places in the pt’s visual field and ask him if he sees it. Test one
       eye at a time.

Congenital Disorders
   o Down syndrome
   o Marfan syndrome
   o Myotonic dystrophy
   o Tuberous sclerosis
   o Congenital metabolic disorders
             Lysosomal storage – (He didn’t say anything about this in class….)
             Carbohydrate metabolism – (…or this)
   o Neurofibromatosis – Will see hamartomas (Lisch nodules). 95% of neurofibromatosis I pts present with
        hamartomas by age 6.

Hypertension: Factors Affecting Retinal Arterioles
   o Severity
   o Duration
             Acute – Severe hemorrhaging, exudates
             Chronic – Narrowing of arterioles, copper-wiring (looks like silver streak overlying the arteriole),
                some hemorrhaging but not as severe as in acute because the vessels have thickened over the years.

Intracranial Hypertension: Causes
Brain swelling causes pressure down the dural sheath to the optic nerve, leading to swelling of the optic nerve head.
     o Brain Tumor
     o Meningitis
     o Venous sinus thrombosis
     o Hydrocephalus
     o Idiopathic intracranial HTN – A diagnosis of exclusion. Dr. Gregg diagnoses this the most.

Central Retinal Artery Occlusion: Management
    o Internal carotid artery  ophthalmic artery  central retinal artery - Anything clogging the vasculature along
         this line will cause ischemia of the eye.
               Occlusion of ophthalmic artery – total ocular ischemia
               Central retinal artery – ischemia of the retina
               Either way there is vision loss that is typically painless
    o    Management – Goals are to lower pressure in the eye and vasodilate the vessels in the eye. The following
         treatments will do this.
               Rebreathing CO2 – Have pt breathe in a plastic bag to dilate the vessels.
               Topical β-blockers – Lower the pressure
               IV acetazolamide 500 mg
               Massaging of globe with lids closed – Massage with heel of your hand to dislodge the plaque that is
                 causing the occlusion.
               Anterior chamber paracentesis
               Calcium channel blockers
               Hyperbaric O2

Amaurosis Fugax
   o Term for symptoms caused by embolic phenomenon.
   o Monocular dimming of vision
   o Temporary arterial obstruction – As plaque moves along the vasculature, areas that were ischemic get
       reperfused, so areas of decreased vision go away.
   o Sudden, transient, painless visual loss

Amaurosis Fugax: Evaluation
   o Cardiovascular – Look at heart for source of embolization (pts with mitral valve prolapse have plaques on their
       valves that can break off)
   o Cerebrovascular – Usually look at carotid arteries
   o Ophthalmologic – Sometimes you can see the plaque, which will lead to the diagnosis.

Migraine: Visual Symptoms
   o Scintillations – Aura - Usually bilateral. Sometimes a pt will report that it’s only in their right eye, but if they
        close their right eye they can still see the scintillations. They actually mean the aura is in their right visual
        field. Usually lasts 10-45 minutes.
   o Amaurosis fugax – Sudden painless visual loss. Seen in very severe migraines.
   o Transient homonymous hemifield loss
   o Transient cortical blindness – Seen in very severe migraines.

Migraine: Types
   o With headache – Classic, common, complicated
   o Without headache - Acephalgic – All the symptoms of a classic migraine, except no headache

Migraine: Treatment
   o Prevent frequent attacks
   o Examine for visual loss
   o Consider discontinuing oral contraceptives

Blood Dyscrasias with Ocular Manifestations
    o Hyperviscosity syndromes
    o Thrombocytopenia
    o Anemia, including sickle cell anemia

Hyperviscosity Syndromes: Ocular symptoms
   o Amaurosis fugax
   o Permanent visual loss

Sickle Cell Retinopathy
    o HbSC disease (most common form) – Most common form to cause the peripheral neovascularization seen in
        sickle cell retinopathy. Causes neovascularization out in the periphery of the fundus.
              Mechanism of neovascularization – Occluded arteries in the periphery of the fundus  retina
                  becomes ischemic  retina sends out factors in response to ischemia  new blood vessels made, but
                  the new vessels leak and bleed easily.
              Treatment – Ablate the retina with laser so it produces fewer factors.
    o   HbSS disease
    o   Sickle thalassemia

Malignant Neoplasms Involving the Eye
    o Primary ocular melanoma – Most common primary tumor of the eye.
    o Large cell lymphoma
    o Metastatic carcinoma – Most common tumor of the eye.

Metastatic Carcinoma
   o Most common intraocular malignancy in adults
   o May be asymptomatic
   o May produce decreased or distorted vision
   o Less common in the iris because less vasculature here than in the choroid, for example, which has a rich
         vasculature.
   o Multiple tumors or bilateral involvement mean metastatic carcinoma.

Ocular Metastasis: Treatment
    o Local radiation
    o Chemotherapy
    o Eye wall resection
    o Enucleation if blind, painful eye

Ophthalmologist should monitor ocular metastases at regular intervals – You can observe ocular metastases to see if
treatment of the 1° tumor is working. Meaning if the tumor in the eye is growing, then the treatment is not working.

Autoimmune disorders - Connective tissue diseases, thyroid eye disease, myasthenia gravis

Connective Tissue Disorders
   o Dry eyes are the most common manifestation
   o Symptoms – Burning, foreign-body sensation, photophobia

Sjogren’s Syndrome
    o Dry eyes
    o Dry mouth
    o May have connective tissue disorder
    o You can look for specific autoantibodies if you suspect Sjogren’s syndrome.

Dry Eyes: Treatment
    o Artificial tears
    o Lubricating ointment at night
    o Punctal occlusion
    o Environmental modification – As in goggles, humidifier in the bedroom, don’t leave ceiling fan on.
    o Severe dry eyes  epithelium of the eye sloughs off  stroma underneath “melts”  perforation of the globe
        and the iris is “mashed” up against the cornea (loss of the space in between the two structures)

Ankylosing Spondylitis: Ocular Symptoms
   o Photophobia
   o Redness
   o Decreased vision
   o Iritis – Inflammation inside the eye. Can see WBC’s floating around inside the eye.
   o Refer for evaluation and treatment

Topical corticosteroids may lead to serious ocular complications – As a PCP, never treat pts with topical steroids.
    o Unless you have a slit lamp and you can really see the cornea and conjunctiva under high magnification, you’re
         going to miss some things and possibly mistreat a pt’s condition with topical steroids.
    o Treatment with topical steroids for a prolonged period of time can lead to glaucoma, cataracts, and infections.
Rheumatoid Arthritis: Ocular Manifestations
   o Dry eyes
   o Episcleritis – Inflammation of the superficial sclera.
   o Scleritis – Inflammation of the sclera
   o Corneal ulcers
   o Uveitis
   o Scleromalacia perforans – A slow, quiet inflammation; pt probably won’t know it’s there. “Melts” the sclera.
       You can see it if you lift the pt’s eyelid and have them look down. Tell the pt not to rub eyes because the
       sclera is so thin that minimal trauma can perforate it.
   o Pauci-articular – Most common form affecting the eye. So if there are only 2 or 3 joints involved, then the pt.
       is more likely to have ocular manifestations than a pt with every joint involved
   o A pt with juvenile onset RA should be followed by an ophthalmologist because findings are very subtle, and if
       untreated, it could lead to glaucoma, cataracts, and scarring inside the eye.

Iritis in Juvenile Rheumatoid Arthritis
      o Few symptoms or signs
      o Usually chronic
      o Secondary cataract and glaucoma
      o Refer for periodic ophthalmic screening

Systemic Lupus Erythematosis: Ocular Manifestation
    o Dry eyes
    o Scleritis
    o Peripheral corneal ulcers
    o Retinopathy and optic neuropathy
         Cotton-wool spots – Micro-infarction from occluded vasculature

Polyarteritis Nodosa: Ocular Manifestations
    o Dry eyes
    o Corneal ulcers
    o Scleritis
    o Hypertensive retinopathy
    o Retinal vasculitis

Giant Cell Arteritis
    o Ischemic optic neuropathy caused by GCA
         Mechanism
             Thickening of the arteries  lumen becomes smaller and smaller  artery can eventually close 
             ischemia of the optic nerve head, which is typically irreversible and untreatable.
         The only thing that we can do then is treat the temporal arteritis, and try to prevent the same thing from
             happening in the other eye. Usually leads to legal blindness in the affected eye (may not be totally blind,
             but the eye is basically useless).
    o If GCA goes untreated, pt can permanently lose vision in opposite eye w/in hours to days, so treat aggressively.
    o Symptoms
              Headache
              Scalp tenderness
              Jaw claudication
              Polymyalgia rheumatica
              Tenderness to palpation of temporal artery area
              Acute visual loss – Typically in older pt (above 60)

Giant Cell Artertis: Diagnosis
    o Clinical history
    o STAT ESR – If sed rate is high and you’re very suspicious for GCA, go ahead and treat. BUT, sed rate is
        negative in 20% of cases of GCA, so history is key.
    o Fluorescein angiogram
    o Temporal artery biopsy – Start treatment before you bx.
Giant Cell Arteritis: Treatment
    o If GCA is suspected, begin treatment immediately with high-dose corticosteroids daily.
    o A temporal artery bx can be done w/in a week after treatment with high-dose steroids has begun and still show
        positive results.
    o Do not wait for results of temporal artery biopsy to begin treatment.
    o Treatment will not produce immediate changes in the artery, but it will prevent the same thing from happening
        in the other eye.
    o Once the dx is confirmed with the bx, follow the pt with sed rates. If sed rate becomes elevated, repeat
        treatment with steroids.

Thyroid Ophthalmopathy
    o Not always correlated with serum thyroid levels
    o Can progress after thyroid function returns to normal

Thyroid Ophthalmopathy: Classes – acronym - NOSPECS
    o 0 – No signs or symptoms
    o 1 – Only signs – Such as lid retraction. Tell the pt. to look up then look down; a sign of thyroid eye disease is
        if the eyelid lags behind when the pt looks down.
    o 2 – Soft tissue involvement – May have dry eye type symptoms
    o 3 – Proptosis – Thyroid/Graves’ disease is most common cause of uni- or bilateral proptosis. If you find
        undiagnosed proptosis, look at thyroid fx.
    o 4 – Extraocular muscle involvement – Restriction of eye motility. Inferior rectus is the most common muscle
        affected.
    o 5 – Corneal damage – Due to exposure because the pt can’t close their eye. Can be prevented by maximal
        lubrication and other treatments.
    o 6 – Sight loss
         Mechanism of compressive optic neuropathy:
             Lymphocytic infiltrates in extraocular muscles  muscles get so big they take up all the space in the orbit
              muscles compress the optic nerve  sight loss

Thyroid Ophthalmopathy: Treatment of Congestive Phase (usually w/in first 2 years of onset of symptoms)
    o Tear substitutes
    o Systemic corticosteroids – To decrease inflammation in the orbit.
    o Orbital irradiation or surgical decompression
         Surgical decompression – Knock out the floor of the orbit to allow some of the orbital contents to go into
            the maxillary sinus so that the nerve is not compressed.

Thyroid Ophthalmopathy: Treatment of Cicatricial Phase (damage is done; disease not progressing anymore)
    o Lid surgery – To lower the lid so it covers the eye better.
    o Muscle surgery
    o Orbital surgery

Myasthenia Gravis: Systemic Involvement
   o Typical history – In the morning, the pt. is ok. Towards the end of the day, the pt. can’t keep his eyes open,
       has double vision.
   o Refer suspects for neurologic evaluation

Sarcoidosis
    o Idiopathic inflammation
    o More commonly affects African-Americans and Hispanics
    o Ocular involvement in about 25% of pts
    o Causes a specific form of iritis – can see WBC floating around in the eye (“mutton fat”), severe photophobia.

Refer patients with sarcoidosis if ocular involvement is suspected. Treatment may include corticosteroids.
Optic Neuritis
    o May be initial manifestation of MS
    o Acute, painful (unlike amaurosis fugax, which is painless) vision loss in one or both eyes
    o May have afferent papillary defect
    o Treat initial episode with IV methylprednisolone (controversial – some say just leave it alone)

AIDS: Ocular Manifestations
   o Dry eye
   o Cotton-wool spots – Whitish spots due to occlusion of a vessel.
   o CMV retinitis
   o Kaposi’s sarcoma involving the eyelid or conjunctiva

CMV Retinitis: Therapy
  o IV ganciclovir
  o IV foscarnet
  o Intravitreal ganciclovir
  o Implantable pellets of sustained-release ganciclovir into the vitreous

CD4 Counts
   o < 100 cells/ml: Opportunistic infections (eg CMV retinitis)
   o > 100 cells/ml: Other ocular infections (eg syphilis)

Systemic Medications – A pt’s meds can cause retinopathy and optic neuropathy. Improve situation by taking the pt off
the meds causing the problems and trying something different.
    o Toxic Retinopathies – Thioridazine, chloroquine, hydroxychloroquine, tamoxifen
    o Toxic Optic Neuropathies – Ethambutol, isoniazid, fluoroquinolones

                 Primary care physician
                             
                 Ophthalmologist

(Nothing said about this slide)