Systemic Diseases and the Eye Department of Ophthalmology

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Systemic Diseases and the Eye

• Department of Ophthalmology
• University of Tennessee
• Health Science Center

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Diabetes Mellitus

• Diabetic retinopathy is the most common cause of
  new cases of blindness in US in patients ages 20
  to 74
• Good control of Diabetes Mellitus may slow
  progress of diabetic retinopathy
• Perfect control NOT totally protective
• General physician needs to refer patient to
  ophthalmologist for eye examination in a timely
  manner

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Diabetes MellitusReferral Guidelines

• Juvenile onset (lt 30 y/o) eye examination
  within 5 years of diagnosis and yearly thereafter
• Adult onset (gt 30 y/o) eye examination at time
  of diagnosis and yearly thereafter
• Pregnant diabetic eye examination early in the
  first trimester and every 3 months during
  pregnancy
• Gestational diabetes little known risk

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Diabetes MellitusPathophysiology

• Hyperglycemia damages basement membrane of
  retinal capillaries
• Selective loss of pericytes
• Microaneurysm formation
• Leakage of microaneurysms and capillaries
• Serum hard exudates
• RBC - hemorrhage
• Macular edema

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Diabetes MellitusPathophysiology (Continued)

• Capillary closure
• Retinal ischemia - cotton wool spot formation
• Ischemia stimulates release of Vascular
  Endothelial Growth Factor (VEGF)
• VEGF causes proliferation of new blood vessels in
  abnormal locations
• Retina traction retinal detachment
• Angle neovascular glaucoma
• Therapy is directed toward eliminating ischemia
  Laser photocoagulation of ischemic retina

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Diabetes Mellitus
Blot hemorrhage
Hard exudates
Background Diabetic Retinopathy consists of 1.
Microaneurysms 2. Dot and blot
hemorrhages 3. Hard exudates
Microaneurysms vs dot hemorrhages
Background Diabetic Retinopathy
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Diabetes Mellitus
Pre-proliferative Diabetic Retinopathy consists
of 1. Cotton wool spot (CWS) indicates retinal
ischemia 2. Venous beading
CWS
Pre-proliferative Diabetic Retinopathy
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Diabetes Mellitus
Proliferative Diabetic Retinopathy consists
of 1. New blood vessel and connective tissue
growth on the retina and/ or into the vitreous
New blood vessels may also grow in the anterior
chamber angle causing neovascular glaucoma
Neovascularization
Proliferative Diabetic Retinopathy
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Diabetes Mellitus
Proliferative Diabetic Retinopathy consists
of 1. New blood vessel and connective tissue
growth on the retina and/ or into the vitreous
New blood vessels may also grow in the anterior
chamber angle causing neovascular glaucoma
Advanced Proliferative Diabetic Retinopathy
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Diabetes MellitusOther Ophthalmic Complications

• Increased incidence of Chronic Open Angle
  Glaucoma
• Cranial nerve palsies III (with normal pupillary
  reactions), IV, VI
• Cataract formation at earlier age
• Orbital Mucormycosis (with acidosis)

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Orbital Mucormycosis See slides 44 47 on
orbital cellulitis also
Orbital Cellulitis - Mucormycosis
Lid and face necrosis - Mucormycosis
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Hypertensive Retinopathy

• Grade I mild arteriolar narrowing
• Grade II moderate arteriolar narrowing with AV
  crossing defects
• Grade III more severe arteriolar changes with
  hemorrhages and/or exudates
• Grade IV Grade III plus optic disc edema

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Hypertensive Retinopathy
Grade III Hypertensive Retinopathy
Grade IV Hypertensive Retinopathy
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Carotid AtherosclerosisOphthalmic Manifestations

• Amaurosis Fugax temporary unilateral visual
  loss 5 to 10 minutes duration
• Hollenhorst Plaque cholesterol embolus in
  retinal arteriole from carotid artery disease
• Central Retinal Artery Occlusion
• Ischemic optic neuropathy

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Hollenhorst Plaque
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Central Retinal Artery Occlusion

• Painless loss of vision
• Afferent pupillary defect
• Pale disc
• Narrowed arterioles
• Cherry Red Spot in macular region

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Central Retinal Artery Occlusion Emergency
Treatment

• Increase CO2 - breathe in bag, CO2 (resp
  therapy)
• Lower intraocular pressure
• -Acetazolamide
• -Ocular massage
• Repeat on/off at 5 second intervals
• -R/O Temporal arteritis
• -Obtain STAT sedimentation rate
• -Start steroid therapy promptly if sed rate ?
• -Get temporal artery biopsy promptly

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Ischemic Optic Neuropathy

• Painless loss of vision
• Pale swelling of the optic nerve
• Afferent pupillary defect
• R/O temporal arteritis

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Central Retinal Vein Occlusion

• Painless loss of vision
• Patient may have hypertension other vascular
  disease
• May be associated with increased blood viscosity

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Central Retinal Vein Occlusion

• Hemorrhagic infarct of retina
• Massive hemorrhage in total occlusion
• Hemorrhages along veins in partial or branch vein
  occlusion

Superior Branch Retinal Vein Occlusion
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Temporal Arteritis

• More common in females
• More common in older patients (gt55)
• Usually has elevated sed rate
• Ask about headaches, scalp tenderness, jaw
  claudication, polymyalgia rheumatica, other
  symptoms
• Consider in older patient with sudden visual
  loss, CRAO or ischemic optic neuropathy

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Graves Disease

• Female preponderance
• May appear unilateral (asymmetric)
• Thyroid status Hyper-, Hypo-, Euthyroid
• Lid retraction
• May have involvement of Inferior Rectus muscle
  with restriction of up gaze
• CT shows enlargement of extraocular muscles not
  involving muscle tendons

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Graves Disease

• Look for exposure keratopathy due to inability of
  retracted lid to cover cornea
• May develop optic neuropathy due to compression
  by enlarged extraocular muscles
• Surgery may be indicated to decompress orbit to
  preserve vision
• Surgery may be required to protect cornea,
  improve cosmesis, or attempt to correct diplopia

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Graves Disease
Note lid retraction OS and limitation of
elevation OS
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Graves Disease
Note massive enlargement of EOM in left orbit
tendons not involved
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Graves Disease
Note lid retraction OS
Note lid lag OS on down gaze
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Sarcoidosis

• Affects eye or adnexa in 20 to 40 of patients
  with sarcoidosis
• May involve visual system in many ways
• Enlarged lacrimal gland, dry eye, anterior
  uveitis, conjunctival granuloma, skin lesions on
  lid, orbital masses, optic nerve granuloma,
  retinal periphlebitis, retinal granuloma
• Conjunctiva - a source for biopsy to obtain a
  tissue diagnosis

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Sarcoidosis
Sarcoid periphlebitis candle-wax drippings
Sarcoid uveitis Note mutton fat KP on back of
cornea
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Rheumatoid Arthritis

• Dry eye (keratitis sicca)
• Dry mouth
• Cataracts from systemic steroid therapy
• Corneal melt - flat anterior chamber
• Scleritis

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Keratitis sicca Note irregular light reflections
Scleritis Note thinning of sclera superiorly
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Corneal Melt in RA with shallow Anterior Chamber
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Lupus

• Cotton wool spots in fundus
• Dry eye
• Scleritis
• Cataracts from systemic steroid therapy
• If on hydroxychloroquine (Plaquenil) -
  ophthalmologist must follow patient

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Lupus
Multiple Cotton Wool Spots in ocular fundus
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Ankylosing Spondylitis

• Anterior uveitis (iritis)
• More common in males
• Red eye
• Pain
• Photophobia
• No purulent discharge
• Small pupil, sometimes irregular
• May recur at irregular intervals

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Ankylosing Spondylitis
Anterior uveitis (iritis) in Ankylosing
Spondylitis
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Herpes Zoster Ophthalmicus

• Involvement of CN V1
• Corneal involvement common
• Hutchinsons Sign
• Vesicles at tip of the nose
• Corneal involvement likely
• Anterior uveitis
• Scleritis
• CN palsies (III, IV, VI)
• Optic neuritis

Get Ophthalmology consultation for Slit Lamp Exam
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Herpes Zoster Ophthalmicus
Hutchinsons Sign
Early
Eruption on Face
Late
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AIDS - HIV Infection

• CMV retinitis
• Herpes zoster ophthalmicus
• Molluscum contagiosum (multiple) - lids
• Keratoacanthoma - lids
• Conjunctivitis
• Kaposis sarcoma may involve lid or conjunctiva
• Retinal toxoplasmosis
• Many other infections by opportunistic organisms

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Kaposis sarcoma - conjunctiva
CMV retinitis
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The following slides do not specifically cover
systemic diseases, but do illustrate several
important infectious and neoplastic diseases of
the eye or its adnexa that are commonly
encountered by the general physician.
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Nasolacrimal Duct Obstruction

• Patient usually complains of constant tearing
• Patient has recurrent conjunctivitis unresponsive
  to antibiotics
• Infants
• Massage and observe unless large abscess formed
• Probe at about 9 months of age unless clears
  with conservative Rx
• Adults
• May present with acute dacryocystitis (abscess)
• Usually requires surgery (dacryocystorhinostomy
  DCR)

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Nasolacrimal Duct Obstruction
Dacryocystitis in adult
Dacryocystitis in infant
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Lid (pre-septal) Cellulitis

• Infected insect bite, laceration, fracture into
  sinus (subcutaneous emphysema)
• Redness, induration of lid
• Tenderness, pain
• No proptosis or limitation of eye movements
• Eye usually looks normal - not injected, etc.
• Treatment - systemic antibiotics, no nose blowing
  (if sinus fx), warm compresses

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Lid (pre-septal) Cellulitis
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Orbital (post-septal) Cellulitis

• Usually originates in infected sinus
• Pain
• Lid swelling
• Proptosis of globe
• Limitation of motion of the globe
• Chemosis (swelling of conjunctiva)
• Vision loss

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Orbital (post-septal) Cellulitis
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Orbital (post-septal) Cellulitis

• Evaluation - Visual Acuity, look for afferent
  pupillary defect, perform CT scan
• Treatment - IV antibiotics, surgery to drain
  abscess if formed (may need urgent surgery)
• Always consider Mucormycosis in a diabetic with
  orbital cellulitis (see next slide)
• Consider other fungal, opportunistic organisms in
  immunocompromised patients

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Mucormycosis
CT scan demonstrating proptosis and orbital
inflammation
Lid edema, chemosis, limitation of motion of eye,
proptosis, CRAO
Histopathology GMS Stain
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Common Ophthalmic Tumors

• Lids
• Basal Cell Carcinoma
• Intraocular - childhood
• Retinoblastoma
• Orbital - childhood
• Rhabdomyosarcoma
• Intraocular - adult
• Metastatic - lung, breast
• Primary - choroidal melanoma

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Basal Cell Carcinoma - Lid
Note raised pearly edge of the tumor and the
depressed center
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Retinoblastoma

• Most common PRIMARY intraocular malignancy of
  childhood
• Hereditary and Non-hereditary forms
• Presenting signs
• Strabismus with vision loss (may be confused with
  childhood strabismus with amblyopia)
• White pupil (amaurotic cats eye)
• Red eye

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Retinoblastoma
Note abnormal pupil reflection
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Retinoblastoma
These are some general rules about Retinoblastoma
- exceptions are not rare
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Choroidal Melanoma

• Most common PRIMARY intraocular malignancy of
  adulthood
• Presenting symptoms/signs
• None, discovered on routine eye examination
• Decreased vision
• Retinal detachment
• Blind, painful eye

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Choroidal Melanoma
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Choroidal Melanoma

• If general physician is searching for a primary
  tumor in a patient who has liver metastasis and
  no primary tumor is readily found, have the
  ophthalmologist examine patient to R/O choroidal
  melanoma (may be small, peripheral tumor)
• Liver metastasis may wait many years (20 to 30)
  to become manifest

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This concludes this presentation The faculty of
the department of Ophthalmology hope that this
presentation has been helpful in your study of
ophthalmology