SPRING EDUCATIONAL SEMINAR – 2013
PENNSYLVANIA OPTOMETRIC ASSOCIATION
CLINICAL CHALLENGES IN THE DIAGNOSIS AND MANAGEMENT OF
OCULAR PATHOLOGY
A PANEL DISCUSSI0N
MODERATED BY BERNARD H. BLAUSTEIN, O.D., F.A.A.O.
Case 1 Hyphema
Description
- An accumulation of blood in the anterior chamber
- Usually results from a tear in the blood vessels at the root of the iris secondary
to blunt trauma to the front of the eye (contracoup) but may occur secondary to
neovascularization in the iris, intraocular surgery, or bleeding diathesis
- The injury may tear the iris away from the ciliary body (iridodialysis)
Workup
- Rule out globe rupture - severe subconjunctival hemorrhage and edema along
with restricted ocular motility.
- Rule out orbital floor fracture - displaced globe, restriced elevated gaze,
reduced sensation in the distribution of the infraorbital nerve, emphysema of
the periorbital tissues, positive CT scan.
- Quantify degree of hyphema (if greater than 50%, there is an increased risk
of glaucoma and endothelial decompensation leading to blood staining of the
corneal stroma).
- Quantify flare and cells
- Applanation tonometry QD until hyphema resolves
- Dilate pupil and evaluate fundus if patient has not sustained a concussion
(with concussion, you want to make sure there is no subsequent compression
on CN III).
- No gonioscopy or scleral depression so as not to disturb clot
- Applanation tonometry
- Rule out sickle cell disease if patient is black. (sickled cells may block TM
causing IOP to rise; reduced perfusion to the optic nerve may occur at lower
IOPs)
- Attempt to prevent rebleed within 2-5 days post trauma so as to prevent
increase in IOP and decompensation of endothelium.
Management and Treatment
- Attempt to encourage clotting for at least 3 days; then encourage reabsorption
of the clot.
- Hospitalize patient if he is too young to cooperate or if systemic medications
will be administered.
-2- Advise bed rest with head elevated 30 degrees (lowers venous pressure so as
to reduce chance of rebleed, decreases IOP, facilitates settling of the blood).
- Advise against bending or lifting of heavy weights.
- Utilize Fox shield to prevent accidental jarring of eye.
- Recommend acetaminophen for pain (aspirin may induce rebleed).
- Utilize atropine 1% BID to immobilize pupil and to stabilize traumatic
iritis; utililize Pred Forte to reduce the inflammation of traumatic iritis.
- Consider aminocaproic acid (Amicar) systemically to preserve the clot.
- Reduce IOP if above 30 mm Hg in a caucasian or if above 24 mm Hg if
patient has sickle cell trait (slight elevations of IOP are beneficial as a
tamponade and tend to encourage clotting)
- Consider irrigation and aspiration if elevated IOP is nonresonsive, hyphema
is greater than 50% and corneal staining is beginning to occur, and if
hyphema has not begun to clear in 4 days.
- Tonometry 30 days post hyphyma to determine if IOP is rising due to
obstruction of the TM by degenerated RBCs (ghost cells); gonioscopy 30 days
post hyphyma to check for angle recession; dilated fundus evaluation with
scleral depression 30 days post hyphema to check for retinal holes thereafter;
Case 2 Recurrent Corneal Erosion (RCE)
Description
- A recurrent erosion of the corneal epithelium due to some disturbance in the
adhesion complex
- Usually occurs when the patient awakens from sleep
- Erosion has large, loose flap edges
Etiology
- Antecedent corneal injury from a sharp object which has injured the adhesion
complex
- Diabetes (weakens the adhesion complex)
- Any corneal dystrophy that disrupts the adhesion complex, e.g., epithelial
basement membrane dystrophy, Reis Bucklers corneal dystrophy, granular
corneal Dystrophy, macular corneal dystrophy
Epithelial Basement Membrane Dystrophy
- The most common cause of RCE
- An autosomal dominant dystrophy found most commonly found in females
over age 30
- Consists of reduplicated and thickened basement membrane that has impaired
fibrillar attachment to the underlying Bowman’s membrane
- Appears clinically as fine epithelial lines which assume the shape of finger-
prints or land formations on a map; may also manifest gray-white putty -like
formations which represent cysts of fluid and cellular debris
-3Treatment of RCE
- Debride loose epithelium; apply antibiotic; apply cycloplegia; pressure patch
for 24 hours.
- Hypertonic saline drops QID and hypertonic ointment HS for 6-8 weeks
- If RCEs continue, consider fitting patient with high-water contact lens.
- If RCEs continue, consider stromal puncture or phototherapeutic keratotomy
(PTK) to bind the basement basement membrane to the underlying Bowman’s
layer.
- The newest management approach is to have the patient utilize topical steroid,
TID for three weeks along with 50 mg doxycycline, BID for 2-3 months.
(doxcycline and steroid inhibit matrix metalloproteinase 9, an enzyme that
degrades the adhesion complex)
Case 3 Thygeson’s Superficial Punctate Keratopathy
Description
- A bilateral, but often asymmetric, coarse, punctate epitheliopathy usually
occuring in females under age 40
- Patient presents with pain, photophobia, and lacrimation
- Lesions appear as oval to circular coarse, gray, grainy, elevated dots and will
stain with FL
- There is little or no conjunctival or lid involvement, i.e. the eyes are minimally
injected, and there are no follicles or papillae
- The pathology runs a chronic course with exacerbations and remissions.
When in remission the lesions are not elevated, do not stain, and induce no
symptoms.
Management and Treatment
- Rule out EKC ( hyperemic conjunctiva, edematous lid, follicles, preauricular
lymphadenopathy).
- Rule out toxicicty, e.g. the patient admits to using eyedrops.
- Treat with low-dose topical steroids, e.g., Alrex, Lotemax, FML - relief is
dramatic often occurring in as little as one day.
Case 4 Optic Neuropathy Masquerading as Glaucoma
Anatomic Considerations – Optic Nerve
- The optic nerve consists of axons from retinal ganglion cells, i.e. the nerve
fiber layer (NFL).
- Retinal NFL is divided into 3 bundles: papillomacular bundle, arcuate bundles,
nasal radial bundle
1. Papillomacular bundle
- Fibers run a straight course from the fovea to the temporal half of the
optic disc and do not respect horizontal meridian.
-4- Lesions produce temporal pallor, central or cecocentral scotomas, VA
reduction, APD, color vision deficit, light sensitivity deficit
- Glaucoma preferentially spares the papillomacular bundle until very late
in the disease process.
- Temporal pallor is not a normal manifestation of glaucoma.
2. Arcuate bundles
- Fibers originate temporal to the macula, arch above and below the
papillomacular bundle, and enter disc at the superior and inferior poles.
- Fibers are segregated into upper and lower compartments and do not cross
the horizontal raphe.
- Glaucoma preferentially affects the arcuate bundles.
- Lesions affecting arcuate fibers near the fovea, produce paracentral
scotomas.
- Lesions affecting arcuate fibers near the optic disc, produce partial arcuate
scotomas which do not cross the nasal horizontal meridian.
- Lesions affecting fibers remote from the disc, produce nasal steps.
3. Nasal radial fibers
- Fibers originate nasal to the optic disc.
- Fibers fan into the optic disc in a radial fashion.
- Fibers do not segregate into upper and lower bundles and do cross the
horizontal raphe.
- Lesions produce wedge-shaped defects which do not respect the horizontal
meridian and do not produce a step-like depression.
Case 5 Phlyctenulosis
Description
- A pinkish-white nodule at the limbal conjunctiva; may not involve the cornea
- Nodule is vascular but centered in an area of dilated blood vessels with a local
leash of blood vessels running away from the lesion
- Usually occurs in children or young adults
Conjunctival phlyctenulosis
- Mild symptoms of discomfort
- After several days, lesion becomes necrotic and sloughs off leaving no scar
Corneal phlyctenulosis
- Lesion has a triangular shape and sits astride limbus
- Significant symptoms of pain, photophobia, and lacrimation
- Lesion may be stationary or may migrate onto cornea
- If corneal migration occurs, neovascularization and scarring may occur.
-5Etiology
- Results from a Type 4 delayed hypersensitivity reaction to a systemic antigen
- Etiology is usually staphylococcus; tuberculosis is the second most common
etiology.
- Other etiologies include intestinal parasites, chlamydia, HSV, or gonococcus.
Differential diagnosis
- Inflamed pingecula
- Nodular episcleritis
- Vernal limbal keratoconjunctivitis
- HSV - Corneal neovascularization running into a stromal infiltrate
- Ocular rosacea
Treatment
- If staphylococcal blepharitis is present – lid hygiene and antibiotic ointment
along with topical steroids, e.g. FML, Lotemax
- If rosacea is present – systemic tetracycline or erythromycin along with topical
steroid
- If TB, intestinal parasites, chlamydia, or gonococcus are present coordinate
with PCP, and continue topical steroid
Case 6 Central Serous Chorioretinopathy (CSR)
Description
- An exudative chorioretinoapthy characterized by a neurosensory retinal
detachment
- Appears as a thin, clear, blister-like lesion underneath the macula; the foveal
reflex is lost; there are often sub-retinal precipitates.
- There may be an associated retinal pigment epithelium detachment.
- A positive scotoma, i.e. metamorphopsia and /or micropsia, is present.
- An increase in hyperopia is present.
- Most cases occur in men between ages 20-50; however, pregnanacy is a known
risk in females.
- CSR is associated with Type A personality features in individuals who have
elevated stress and elevated stress hormones, i.e. corticosteroids and
epinephrine.
- Other etiologies include topical steroids, steroid inhalers, or systemic steroids.
Pathophysiology
- The inner choroidal vasculature undergoes vasoconstriction; some of the overlying RPE cells decompensate leading to a focal break between the RPE cells;
a malfunction of the RPE pump mechanism occurs, and fluid accumulates in
the space between the RPE and the sensory retina.
-6- Fluid accumulating between Bruch’s membrane and the RPE results in an RPE
detachment which has a yellow-orange appearance and is more solid-looking
and thicker than the serous neurosensory detachment.
Workup
- History of steroid use?, Amsler grid, photo-stress recovery test
- OCT – separation between RPE and sensory retina/separation between RPE
and Bruch’s membrane
- IV fluorescein angiography (IVFA); with CSR a small, diffuse
hyperfluorescent spot appears in the choroidal phase and spreads out like an ink
blot or spreads upward in a column resembling a smoke stack. The spot
increases in intensity and size. With an RPE detachment, there is an early,
even leakage with sharp borders. The leakage remains localized, increases in
intensity, but does not increase in size.
- Rule out other causes of exudative retinal detachment, e.g. choroidal
melanoma, metastasis to the choroid, renal disease acute hypertension,
choroidal hemangioma, chorio-retinal inflammations.
Treatment of CSR
- Discontinue all steroid medication
- CSR usually resolves in 3-4 months; 90% of patients recover VA to 20/20;
30-40% have recurrences.
- Micropulse laser photocoagulation of RPE leakage sites outside FAZ that have
been identified via IVFA
- Low fluence photodynamic therapy (PDT)
Case 7 Macular Pseudo-hole
Description
- A sharply circumscribed, red, macular lesion within an area of epiretinal
membrane traction; the epiretinal membrane pulls the inner portion
of the fovea toward the center causing the foveal contour to steepen into a holelike configuration.
- The outer nuclear layer and the photoreceptor layer are preserved; VA 20/4020/60
- Lamellar hole is a subcategory of pseudo-hole in which there is an irregular
foveal contour, a break in the inner fovea, a split between the inner and outer
retina, and intact foveal receptors
Differential Diagnosis
- Cystoid macular edema – often follows cataract surgery
- Full thickness macular hole
-7Pathophysiology of Macular Epiretinal Memebrane
- Develops at the vitreoretinal interface; consists of proliferating glial
cells that have gained access to the retinal surface through breaks in the ILM
(ILM)
- Breaks in the ILM may be created secondary to PVD, retinal detachment
surgery, retinal trauma, retinal vascular disease, or chronic uveitis.
- Macular epiretinal membranes are divided into cellophane maculopathy and
macular pucker.
Cellophane Maculoathy
- A thin transparent membrane of fibroglial cells which applies tangential
traction
to the retina; retinal surface is irregular with fine striae and tortuosity of
small blood vessels
- Patient may be asymptomatic or may present with mild VA deficit and mild
metamorphopsia
- Best detected with red-free light
Macular Pucker
- Caused by increased thickening and contraction of the epiretinal membrane
- Severe retinal wrinkling and distortion of the blood vessels are manifest.
- White striae may obscure the underlying blood vessels.
- Vision deficit and metamorphopsia are more severe.
- A macular pseudo-hole may form within the epiretinal membrane.
Treatment of Epiretinal Membrane
- Vitrectomy and membrane peel
Macular Hole
- Develops as a result of progressive vitreoretinal traction at the fovea
- Progresses through four stages
Stage 1a is an impending hole that appears as a yellow foveolar spot with a
loss of foveal depression.
Stage 1b results from centrifugal displacement of the foveola; characterized by
a yellow ring around the fovea and mild VA deficit and mild metamorphopsia
Stage 2 is an early, small full-thickness hole that appears as an oval or crescent
and has loss of photoreceptors and markedly reduced VA
Stage 3 is a larger full-thickness hole about the size of 1/3 of a disc diameter;
has a small cuff of subretinal fluid; the posterior cortical vitreous remains
attached.
Stage 4 is a larger full-thickness hole with a complete PVD; the hole is
surrounded by a larger cuff of subretinal fluid; often has tiny yellow flecks at
bottom of the hole
-8Diagnosis of a Full Thickness Macular Hole
- Watske-Allen test – a narrow slit beam is projected vertically and horizontally
over the center of the hole. Patients with a true hole will note that the slit beam
is broken; patients with a pseudo-hole will note that the slit is thin but not
broken.
- OCT reveals anvil-shaped edges and complete loss of photoreceptors and the
outer nuclear layer
Treatment of Macular Hole
- If the VA is less than 20/60 and the duration has been less than one year,
vitrectomy and removal of ILM followed by injection of gas into the vitreal
cavity; patient must maintain a face-down position for one to two weeks to
allow the gas bubble to tamponade the hole; success occurs in 80% of patients.
Case 8 Talc Retinopathy
Description
- Intraretinal, yellow, refractile particles in patients found in patients who
repeatedly inject drugs intended for oral use (Ritalin, Methadone) intravenously
- The oral drugs contain inert fillers such as talc (hydrous magnesium silicate).
- The number of talc particles in the retinal vessels is dependent upon the
duration of drug abuse and the number of IV injections.
Pathophysiology
- Oral drugs with talc fillers are injected IV.
- Talc passes through the right auricle and right ventricle and enters the lung.
- Some of the talc particles are bigger than the pulmonary capillaries and become
entrapped.
- Granulomas develop causing further occlusion of larger arteries; diminished
lung capacity and reduced pulmonary function result.
- Pulmonary hypertension results causing enlargement of the right ventricle (cor
pulmonale); chest pain, syncope, and sudden death may result.
- As pulmonary hypertension increases, collateral blood vessels develop in the
lung.
- The collaterals are large enough to allow the talc particles to gain access to the
systemic circulation as blood passes through the left auricle and left ventricle.
- The talc embolizes to the eye, skin, liver, kidney, and bone marrow.
- The talc can be scattered throughout the fundus but concentrates in the small
arterioles, precapillaries, and capillaries in the macular area. (There is a denser
capillary net and greater blood flow in that area.)
- Capillary occlusion may result in venous engorgement, blot hemorrhages,
cotton wool spots, and peripheral neovascularization.
-9Differential Diagnosis
- Cholesterol deposits (Hollenhorst plaques )
- Tamoxifen
- Canthaxanthine (oral tanning agent)
- Calcific drusen
Drusen
- RPE phagocytizes the tips of the outer segments of the rods and cones.
- The engulfed particles are degraded and recycled or are voided into the
choriocapillaris.
- With increasing age, Bruch’s membrane thickens and prevents the deposition
of the breakdown products into the choriocapillaris; the particles become stored
in Bruch’s membrane as drusen.
Isolated Discrete Drusen
- Small, isolated, discrete, yellowish bodies with little evidence of RPE
atrophy; VA relatively good
Confluent Soft Drusen
- Indicate significant thickening of Bruch’s membrane and RPE atrophy
- Have the highest risk of developing into advanced AMD
Hard Calcific Drusen
- Occur in long standing, indolent AMD; VA usually good
Workup of Talc Retinopathy
- Fluorescein angiography if neovscularization is suspect
- Lung x-ray and lung function tests
Case 9 Meibomian Gland Dysfunction/Dry Eye
Description
- Dry eye is a disorder of the tear film that results from decreased tear
production, excessive evaporation, or an abnormality in the mucin or
lipid components.
- The most common cause of dry eye is meibomian gland dysfunction, a
condition which causes evaporative dry eye
Signs and Symptoms of Dry Eye
- Conjunctival hyperemia, fast BUT, decreased tear meniscus, viscous tears,
debris-laden tears, SPK on inferior 1/3 of cornea, rose bengal or lissamine
green stain of cornea and intrapalpebral conjunctiva, filaments
- Patients indicate that the eyes feel sandy, itchy, gritty, or burning, and report
intermittent blurred vision, FB sensation, and photophobia.
- Symptoms increase as the day wears on.
- Symptoms often do not correlate with signs.
-10Anatomic and Physiologic Correlates
- Tears are composed of three intertwined layers: oil from the, meibomian
glands, aqueous from the lacrimal glands, and mucous from the conjunctival
goblet cells.
- Tears dilute noxious stimuli and flush away inflammatory cells, debris and
components of corneal metabolism.
- Tears supply oxygen, vitamin A, epidermal growth factors, transforming
growth factors, and antimicrobial proteins (lysozyme, lactoferrin, beta lysin).
- Tears provide a smooth refracting surface to the cornea by filling in surface
defects.
- A neural functional reflex loop governs tear secretion: continuous ocular
surface challenge induces sensory afferents to fire; secretory efferents stimulate
secretion of aqueous, mucous, and lipids.
- Dysfunction of the reflex loop alters the quantity and quality of the tear film.
Pathophysiology of Inflammatory Dry Eye (Keratoconjunctivitis Sicca)
- KCS is a chronic, immune-based, cytokine receptor inflammation
characterized by the infiltration and disruption of lacrimal gland tissue by
CD4 T lymphocytes
- The lymphocytes secrete proinflammatory cytokines that promote apoptosis
of lacrimal gland tissue and inhibit the neural reflex loop.
- Reduced androgens in perimenopausal and postmenopausal women also
induce lacrimal cell apoptosis and the accumulation of proinflammatory
cytokines.
Meibomian Gland Dysfunction (MGD)
Non-obstructive MGD
- Occurs in association with hypersecretion of the meibomian glands of the
skin (seborrheic dermatitis) and dandruff
- The meibomian gland ducts and orifices are dilated, and the meibum can be
easily expressed.
- The meibum is of normal consistency, but the amount is excessive.
- Frothy-appearing tears collect along the lid margin.
- Excessive oils adulterate the tear film causing dry spots on the cornea and
discomfort.
- Endogenous lid bacteria convert the meibum to fatty acids whose low pH
causes discomfort.
Obstructive MGD
- The gland ducts and orifices are clogged, and meibum cannot be easily
expressed.
- Aqueous portion of the tears evaporate rapidly
- Often occurs in middle-aged patients who manifest rosacea
- Lid margins are often thickened, notched, and telangiectatic.
-11Treatment of Meibomian Gland Dysfunction/Dry Eye
- Non-obstructive MGD: warm-to-hot compresses, lid scrubs, anti-dandruff
shampoos, non-preserved artificial tears to reconstitute the tear film, topical 1%
azithromycin (Azasite) to reduce inflammation, referral to a dermatologist for
the seborrheic dermatitis
- Obstructive MGD: in-office lid gland expression, at-home warm-to-hot
compresses followed by lid gland expression, topical 1% Azasite, consider a
short course of systemic tetracyclines, non-preserved artificial tears, consider
omega-3 fatty acid supplementation, consider cyclosporine A (Restasis)
Case 10 Optociliary Shunt Vessels
Description
- Optociliary shunt vessels consist of enlarged pre-existing capillaries on the
optic nerve head that shunt blood from the central venous circulation to the
peripapillary choroidal circulation.
- Occurs when there is when there is an obstruction of the normal venous
drainage behind the lamina
- The shunted blood bypasses the retinal venous circulation and passes into the
choroidal venous system; subsequently, the blood passes through the vortex
veins and into the superior and inferior ophthalmic veins.
Etiology
Obstruction of the Central Retinal Vein
- Usually occurs as a result of compression of the central retinal vein (CRV)
- Most commonly, the compression results from an arteriosclerotic central retinal
artery compressing the CRV in the common adventitial sheath.
- Compression may also occur from papilledema, an ONH tumor, or laminar
compression from elevated IOP.
Central Retinal Vein Thrombus
- May occur from autoimmune disease, excessive coagulation of the blood,
hyperviscosity, or IOP-induced laminar compression causing turbulence of the
blood and subsequent thrombus
Optic Nerve Sheath Meningioma
- Neoplasm arises from arachnoid and affects women over 40
- Involves optic nerve within the orbit causing compression and occlusion of the
veins of the optic nerve
- Causes slow, progressive vision loss