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A. Hypertension (HTN) [1] navigator

  1. Narrowing of Retinal Arteries
    1. Initial changes to occur due to HTN in eye
    2. Vasoconstriction
    3. Hypertrophy leading to narrowed vessel lumen
    4. "Copper Wire" changes, arteriovenous nicking, vascular tortuosity
  2. Later Changes as blood-retinal barrier disrupted
    1. Lipid deposits with macular edema
    2. Cotton wool spots (insult to blood flow; neural ischemia)
  3. Malignant Hypertension
    1. May cause optic neuropathy or choroidopathy
    2. Optic disc edema (papilledema)
    3. Cotton wool spots
    4. Due to fibrinoid necrosis of arterial wall
  4. Retinopathy is associated with increased risk for congestive heart failure [3]
  5. Retinopathy in patients with even early HTN is an indication for treatment

B. Hyperviscosity Retinopathy navigator

  1. Causes
    1. Monoclonal Gammopathy (Multiple Myeloma, Macroglobulinemia)
    2. Polycythemia (Polycythemia Vera, Acute Leukemia)
    3. Vascultis may also cause a hyperviscosity
  2. Signs
    1. Looks like vein occlusion - dilated, tortuous veins
    2. Superficial and deep retinal hemorrhages
    3. Macular and disc edema
    4. "Boxcar" appearance of vessels
  3. Diagnosis
    1. Blood viscosity
    2. Serum Protein electrophoresis
  4. Treatment
    1. Plasmapheresis
    2. Good oxygenation
    3. Underlying Disease therapy

C. Sickle Cell Disease navigator

  1. Arteriovenous occlusion with capillary nonperfusion due to sickling and thrombosis
  2. Serious ocular complications - more common in HbSC and Sickle-Thal than HbSS
  3. Nonproliferative Findings
    1. Intraretinal Hemorrhages
    2. Iridescent spots - old resorbed hemorrhages
    3. Black sunburst - subretinal hemorrhage with RPE hypertrophy
  4. Proliferataive Findings
    1. Neovascularization, usually in peripheral retina
    2. "Sea Fan" - new vessel formation
    3. Complications and management similar to diabetic proliferative retinopathy

D. Opportunistic Infections of the Eye navigator

  1. Patient Population
    1. Chemotherapy
    2. Rheumatologic Disease Therapy
    3. HIV Disease
    4. Diabetes mellitus
    5. Alcoholism
    6. Transplantation
  2. HIV Retinopathy
    1. Cotton Wool Spots
    2. Flame Shaped Hemorrhages
  3. Toxoplasmosis
    1. May be seen along with CNS involvement
    2. In HIV, usually ewly acquired without presence of old scar
    3. Congenital toxoplasmosis often shows early and old scarring
    4. Treat with sulfamethoxazole, pyrimethamine (with leukovorin) or clindamycin
    5. Steroid drops rarely required in HIV as vitritis is mild; needed in many non-HIV patients
  4. Kaposi's Sarcoma
    1. Lesion apparently of vascular endothelium
    2. May affect conjunctiva
  5. CMV Retinopathy [2]
    1. Most common intraocular infection in HIV
    2. White granular lesions with associated hemorrhage; "pizza-pie fundus"
    3. CD4 count nearly always <200/µL, usually <100/µL
    4. Retinal detachment often requires silicone oil tamponade to repair
    5. Therapy with Foscarnet or Ganciclovir initially; lifelong maintenance therapy required
    6. Intravitreal ganciclovir implants also in use for severe disease
    7. Oral valganciclovir is safe and effective for maintenance therapy
    8. Cidofovir intravenously prevents progression and maintains eyesite [4]
  6. Candida Endophthalmitis
    1. Mostly endogenous spread (ie. within patient) in iv drug abusers
    2. Predisposition: steroid therapy, parenteral nutrition, AIDS (ARC)
    3. "Fluffball" in retina, eventually spreading to vitreous
    4. Therapy: topical mydriatics, Amphotericin B + 5-FC, Imidazoles
    5. May need vitrectomy with intravitreal amphotericin B for vitreous abscess

E. Cerebrovascular Disease navigator

  1. Stroke may lead to cortical blindness
  2. Field loss can occur if occipital, parietal, or temporal lobes are involved
    1. Temporal lobe lesions usually denser above horizontal media with preserved optokinetic nystagmus (OKN)
    2. Parietal lobe lesions tend to be denser below horizontal media with asymmetric OKN
    3. Occipital lobe lesions tend to produce more congruous field defects
  3. Sudden transient visual loss
    1. Also called amaurosis fugax
    2. Usually due to carotid emobli to retinal circulation
    3. May affect retinal artery directly causing central or branch retinal artery occlusion
  4. Evaluation
    1. Cerebral circulation must be evaluated
    2. Carotid ultrasound
    3. MR angiography
    4. Transcranial Doppler
    5. Ophthalmodynamonetry
    6. Cerebral Angiography
    7. Hypercoagulability Disorders should be sought

F. Giant Cell (Temporal) Arteritis navigator

  1. Patients generally >60 years old
  2. Symptoms of GCA
    1. Headache, unilateral
    2. Scalp tenderness - pain while combing hair
    3. Jaw claudication - pain while chewing
    4. Transient or permanent visual loss
    5. Systemic: Fever, Weight Loss, Diaphoresis, Fatigue
    6. Highly associated with polymyalgia rheumatica (PMR)
  3. Blindness usually due to ischemic optic neuritis
    1. Posterior Ciliary Artery vasculitis most often implicated
    2. Central Retinal Artery can also be involved, with arterial occlusion
    3. Cortical blindness can occur due to CVA
    4. Diplopia may occur due to involvement of extraocular muscles and their innervations
  4. Diagnosis
    1. Symptoms and age
    2. Westergren ESR - may be >100mm/hr
    3. Temporal Artery Biopsy - should be done within 7-10 days of symptoms
    4. Retinal Changes - swollen optic nerve disc may be present
  5. Treatment
    1. If any suspicion, then oral glucocorticoids (usually prednisone, 60mg qd) indicated
    2. Steroid should NEVER be withheld if suspicion for disease is high or even moderate
    3. Methylprednisolone IV (eg. 80mg q12 hrs) for severe visual decrease or bilateral disease
    4. Fairly rapid taper to maintain ESR<40mm/hr
    5. Methotrexate is being evaluated as a steroid sparing agent in this disease

G. Sjogren Syndrome navigator

  1. Augoimmune Disease with Dry Eyes ("Sicca" Syndrome) and dry mouth
  2. Disease due to lacrimal and salivary gland inflammation and destruction
  3. Most patients are ANA+ and have either anti-Ro (SSA) and/or anti-La (SSB) antibodies
  4. May be associated with rheumatoid arthritis, lupus, or mixed connective tissue disease
  5. Can result in ulceration of cornea with perforation
  6. Diagnosis with Schirmer wetting tests and conjunctival staining
  7. Ocular Treatment
    1. Artificial tears, lubricating ointment
    2. Punctal occlusion
    3. Tarsorrhaphy if severe
    4. Cholinergic agonists (eg. bethanechol) may improve tear production
  8. Hydroxychloroquine (Plaquenil®) may be effective for various sicca symptoms

H. Rheumatoid Arthritis navigator

  1. Symmetric polyarthritis
    1. More common in women
    2. Joint pain worse in morning with improvement during day
  2. Ocular Symptoms
    1. Dry, Red Eyes - related to Sjogren's Syndrome (most common in RA, ~20% of patients)
    2. Ulcerative keratitis - corneal ulceration due to local vasculitis, inferior and peripheral
    3. Scleritis
    4. Diffuse anterior form has best therapeutic response
      1. Necrotizing scleritis without inflammation (scleromalacia perforans) - thin sclera
    5. May present with photophobia and/or change in vision
  3. Treatment of Ocular Disease
    1. Glucocorticoid drops ± systemic therapy is recommended acutely
    2. Methotrexate (7.5mg - 15mg q week) may have some preventative effects
    3. Cyclophosphamide intravenously for sight-threatening disease
    4. Cyclosporin A may be effective in disease resistant to other agents
    5. Hydroxychloroquine and/or punctal occclusion may be effective for sicca symptoms
    6. Severe scleritis may require surgical grafting
  4. Uveitis (Iritis)
    1. Very common in in Juvenile Chronic (Rheumatoid) Arthritis
    2. Often asymptomatic and may lead to blindness due to glaucoma, cataract, keratopathy
    3. Mainly in ANA+ pauciarticular disease in women
    4. Asymptomatic posterior (or anterior) uveitis may occur leading to blindness
    5. Opthalmological examinations recommended q3-4 months

I. Other Systemic Vasculitisnavigator

  1. Wegener's Granulomatosis [6]
    1. Necortizing vasculitis involving kidneys and repiratory tract
    2. Ocular findings include scleritis, optic nerve vasculitis, orbital pseudotumor
    3. Anti-neutrophil cytopaslmic antibody (ANCA) positive in >90% of active patients
    4. Rapidly fatal unless glucocorticoid and cyclophosphamide therapy is given
  2. Behcet's Syndrome
    1. Posterior Uveitis most common
    2. Associated with recurrent oral/genital ulcerations and vasculitis symptoms
    3. Treatment requires glucocorticoids and cytotoxic agents (eg. cyclophosphamide)
  3. Cogan's Syndrome
    1. Aortitis with interstitial keratitis
    2. Aortic aneurysms may occur
    3. Extremely rare condition

J. Sarcoidosis navigator

  1. Usually occurs in blacks and hispanics (more common in females)
  2. Symptoms
    1. Bilateral hilar lymphadenopathy most common
    2. May progress to interstitial lung disease
    3. ~25% of patients have ocular symptoms
    4. Non-caseating granulomas occur in almost any organ
    5. Liver, pituitary gland, salivary/lacrimal glands, may be involved
    6. Arthritis occurs in 10-15% of patients
  3. Ocular Involvement
    1. Lid and conjunctival granulomas
    2. Dry eye(s) due to lacrimal gland involvement
    3. Anterior Uveitis: iris nodules, mutton-fat keratic precipitates on corneal epithelium
    4. Posterior Uveitis: macular edema, retinal periphlebitis precipitates on corneal epi
  4. Diagnosis
    1. Presence of hilar lymphadenopathy with other symptoms
    2. Skin biopsy may show erythema nodosum
    3. Conjunctival or lacrimal biopsies may be useful
    4. Serum ACE or lysozyme levels are insensitive (~50%) and non-specific
    5. Gallium scan with specific glandular uptake may be helpful
  5. Therapy with glucocorticoids (including drops)

K. Thyroid Disease navigator

  1. Ocular Muscle Changes leading to various symptoms
    1. Graves' (more common) disease is usually underlying cause
    2. Hashimoto's Thyroiditis may coexist
  2. Etiology
    1. Generally unknown
    2. May involve anti-muscle (mitochondrial) antibodies
    3. Separate from autoimmune thyroid disease (may be hypo-, hyper-, or euthyroid)
    4. Extraocular muscles hypertrophy
    5. Congestion and edema leading to myositis and necrosis
      1. Inferior and medial rectus are most commonly involved
  3. Symptoms
    1. Typical exophthalmos - most common cause of proptosis in adults
    2. Eyelid retraction and corneal exposure results
    3. Can see "whites of eyes" completely around the iris in most people with exophthalmos
    4. Diplopia due to restrictive muslce motion
    5. Visual field defects in severe cases with compressive optic neuropathy (due to edema)
  4. Therapy
    1. Topical lubrication for dry eyes and corneal exposure
    2. Glucocorticoids (prednisone 80-100mg po qd) to suppress inflammation (myositis)
    3. Localized radiation - 1500-2000 rads
    4. Surgical decompression of orbit for compressive optic neuropathy
    5. Extraocular muscle or lid resection surgery once compressive optic neuropathy healed

L. Myasthenia Gravis navigator

  1. Anti-acetylcholine receptor antibodies block neuromuscular transmission
  2. Peripheral paralytic disease
    1. However, ~50% present with ocular signs
    2. >90% of patients with myasthenia gravis will ultimately develop ocular signs
  3. Usually affects women, often with thymus abnormalities (including thymomas)
  4. Symptoms include intermittent ptosis, diplopia, with worsening as day progresses
  5. Diagnosis confirmed with edrophonium (Tensilon) challenge

M. Cancersnavigator

  1. Intrinsic or
    1. Retinoblastoma
    2. Melanoma
  2. Metastatic
    1. Lung - most commonly in men
    2. Breast - most commonly in women
    3. Unknown Primary
    4. Gastrointestinal Tract

References navigator

  1. Wong TY and Mitchell P. 2004. NEJM. 351(22):2310 abstract
  2. Jacobson MA. 1997. NEJM. 337(2):105 abstract
  3. Wong TY, Rosamond W, chang PP, et al. 2005. JAMA. 293(3):63
  4. Lalezari JP, Stagg RJ, Kuppermann BD, et al. 1997. Ann Intern Med. 126(4):257 abstract
  5. Cunningham ET Jr and Margolis TP. 1998. NEJM. 339(4):236 abstract
  6. Schaller JG, Niles JL, Lerner LH. 1999. NEJM. 341(2):110 (Case Record)