Systemic Diseases

Catherine Qui-Macaraig, MD
UST
Why Screen?
 relate survival to retinal vascular changes in the
hypertensive population
 changes correlated directly with the degree of systemic
hypertension
 correlated inversely with the prognosis for survival

Ophthalmic Complications
 Central Retinal Artery Occlusion (CRAO)
 Caused by atherosclerosis. Factors contributing to atheroma include
hyperlipidemia, hypertension and obesity.

 Retinal Branch Vein Occlusion (BRVO)

 Arterial disease, particularly hypertension – is common

 Central Retinal Vein Occlusion (CRVO)

 Increased incidence in hypertension, DM, chronic renal failure, smoking,
etc

Pathologic Changes
 Arteriosclerosis – smaller vessels
diffuse fibrosis and hyalinization

 Atherosclerosis – larger vessels

Lipid streaks / fatty infiltration, fibrosis of
the intima, fibrous plaque, ulceration, hemorrhages, thrombosis
occur, calcification

 Section of retina demonstrating marked thickening of the

arteriolar (A) wall as seen in arteriolosclerosis. Notice the
 compression of the adjacent venule (V).
Natural Course
Normal

Sudden elevation of blood pressure

Generalized uniform narrowing of retinal

arterioles
Vasoconstrictive phase

Persistent Elevated BP

Arterial Wall Damage

Hyperplasia Collagen Replacement

(muscularis) (muscularis)

Sclerotic phase
Sclerotic phase

Persistently very high BP

Marked damage to arterial walls

 Acute arteriolar necrosis

Exudative phase
Clinical findings seen in the retinal vasculature in
hypertensive retinopathy
 Arteriolar narrowing (focal and diffuse)

 Arteriovenous crossing changes

 Arterial sclerosis

 Arterial tortuosity

 Increased branching angles

fundus changes in hypertension

 A. Grade 1 -Earliest sign of hypertension showing only
generalized narrowing of the arterioles with no change in the
vessel wall thickness or reflex.
fundus changes in hypertension
 B. Grade II hypertension showing generalized narrowing plus
focal constriction and grade I arteriolar sclerosis with
widening of the reflex stripe.
AV crossing changes
fundus changes in hypertension
 C. Grade III hypertension showing generalized narrowing, focal
constriction, hemorrhages, and exudate, and grade I arteriolar
sclerosis with widening of the light reflex.
 fundus changes in hypertension
 D. Grade IV hypertension showing generalized narrowing, focal
constriction, hemorrhages, and exudates and edema of the disc
with grade I arteriolar sclerosis

Keith-Wagener-Barker Classification
Hypertensive ocular disease:
 Hypertensive choroidopathy
 Hypertensive retinopathy
 Hypertensive optic neuropathy
Hypertensive choroidopathy
 Young hypertensives
 Zones of nonperfusion of choriocapillaries (Elschnig spots)
 Focal RPE detachments, exudative RD
Hypertensive retinopathy
 a. vasoconstrictive phase
 b. sclerotic phase
 c. exudative phase
 d. complications of sclerotic phase
Hypertensive optic neuropathy
 a. optic disc edema
 b. optic atrophy
 c. ischemic optic neuropathy

Systemic Diseases
Part 2
Catherine Qui-Macaraig, MD
Instructor, UST
AIDS and the Eye
AIDS
 Retrovirus subfamily Lentivirinae
 HIV-1 à seen worldwide
 HIV-2 à western Africa
 CD4 T helper cell depletion à immune deficiency
(infectious diseases in the immunocompromised), polyclonal
B cell stimulation (lymphoma, kaposis sarcoma)
AIDS
 Transmission:
 sexual intercourse: 70%
 IV drug use: 27%
 Blood transfusion: 2-3%
 Perinatal transmission: 1%

 Diagnosis
 Screening: ELISA
 Definitive: Western blot

 CD4 levels:
 250-500 cells/mm3: oral candidiasis, disseminated TB
 150-200 cells/mm3: kaposis sarcoma, lymphoma, cryptosporidiosis
 75-125 cells/mm3: PCP, disseminated mycobacterium avium
complex, ulcerated herpes simplex, cryptococcosis, toxoplasmosis,
esophageal canididiasis
 <50 cells: CMV retinitis

 CD4< 50 should be examined by an ophthalmologist

every 6 months
Kaposi’s sarcoma of the eyelid
KS: violaceous conjunctival induration
Kaposi’s sarcoma of the conjunctiva
KAPOSI’S SARCOMA
 24-35% OF AIDS
 LID, CONJUNCTIVA, CARUNCLE & LACRIMAL SAC
 DISCRETE, DARK REDDISH MASS(CONJ)
 RESEMBLE SUBCONJUNCTIVAL HEMORRHAGE
Kaposi’s sarcoma
 occasionally seen in the conjunctiva
 may mimic a subconjunctival hemorrhage but is usually more
elevated.
molluscum contagiosum
 Multiple molluscum contagiosum should make one suspect the
diagnosis of HIV
HIV-related retinal microangiopathy (cotton wool
spots)
Cytomegalovirus (CMV) retinitis
CMV retinitis

 Latent reactivation
 White retinal infiltrates starting peripherally (late visual complaint)
 Perivascular fluffy white lesions with scattered hemorrahges
 Granular lesions with few hemorrhages and central area of clearing, with
atrophic retina
 Low grade vitritis
CMV retinitis-
“pizza pie” retinopathy

 Cytomegalovirus Retinitis is the most common opportunistic infection in HIV,

usually seen when CD4 + <50
 Retinal detachment frequently complicates CMV retinitis
CMV retinitis
 Retinal detachment in 20% of patients
 50% involvement of second eye if likewise affected
 11% progress to retinal detachment 6 mponths after diagnosis of CMV retinits
 25% involvement of the retina à 5x risk of detachment
 Treatment: GANCICLOVIR; FOSCARNET

CMV Papillitis
Pneumocystis carinii choroidopathy
 Multiple white plaque
 Rare visual loss
  Treatment: IV pentamidine

Ocular Toxoplasmosis
 retinochoroiditis
 Dx IgG and IgM
 In congenital-
focal choroiditis
Tx subconj or retrobulbar steroids

Toxoplasmosis
 Acquired:
general malaise, lymphadenopathy, sore throat, hepatosplenomegaly
Retinochoroiditis: fluffy-white areas of small to multiple retinochoroiditis, active
lesion near an old scar, cystoid macular edema, vitritis, iridocyclitis, IOP increased

Toxoplasmosis: Active
Progressive outer retinal necrosis
Syphilis
 has protean ophthalmic manifestations. CONGENITAL: Interstitial keratitis &
saddle nose

PRIMARY: Eyelid chancre, conjunctivitis

SECONDARY: Iritis, optic neuritis, retinal vasculitis

TERTIARY: Argyll Robertson pupils (small, irregular pupils reactive to near stimulus
but poorly reactive to light), Internuclear ophthalmoplegia

Interstitial Keratitis
 Nonulcerative and nonsuppurative inflammation of the corneal
stroma
  May form stromal neovascularization if untreated
 Diffuse or localized

Interstitial Keratitis
Neuro-ophthalmic manifestations

 Papillitis
 Optic neuritis
 Optic perineuritis
 Papilledema
Ocular manifestations of syphilis
 A female patient aged 73 years with syphilis. Note the optic
atrophy, arteriolar narrowing, pigment loss, and clumping.
Others
 Glaucoma almost always is associated with uveitic inflammation.
 in congenital syphilis, interstitial keratitis is associated with adult
secondary glaucoma in 15-20% of patients.
 glaucoma develops an average of 27 years after the initial episode
of interstitial keratitis has subsided.

Lids/eyebrows
 Chancre rarely occurs in the primary stage.
 Brow loss at its temporal aspect has been reported in secondary and
tertiary syphilis.
 Tarsitis or lid abscess rarely occurs in the tertiary stage.
 Pseudoepitheliomatous hyperplasia, related to the gumma of tertiary
syphilis. These lesions generally are elevated, and they have an uneven
surface that is either ulcerated or crusty, which appears similar to
squamous cell carcinoma or basal cell carcinoma .

 Conjunctiva
 Chancre in primary syphilis
 Nonspecific conjunctivitis in secondary syphilis
 Gumma in tertiary/late syphilis
  Scleritis/episcleritis
 Nodular or diffuse
 Usually present in secondary syphilis

Anterior uveitis
 Unilateral in 56% of cases
 Granulomatous with mutton-fat keratic precipitates on the
corneal endothelium in 50% of cases
 Iris nodules
 small, dilated collections of capillaries in the iris
 Treponemal emboli may cause vascular tortuosity and
dilatation, which account for the iris nodules.

Posterior uveitis
 Posterior uveitis (9-18%)
 Panuveitis (27-47%)
 Vitreal cells are typical.
 Vasculitis with or without vascular occlusion, macular edema, stellate maculopathy, disciform
macular detachment, serous macular/retinal detachment (pseudohypopyon), pseudo-RP, retinal
detachment, neuroretinitis, papillitis, diffuse chorioretinitis, uveal effusion, central retinal vein
occlusion (CRVO), subretinal neovascular membrane (SNVM) formation, and retinal necrosis (big
blind spot syndrome)
 Syphilitic posterior placoid chorioretinitis
 Present in individuals who are HIV positive
 Large, flat, dry, yellowish, or gray (with baited centers); macular or juxtapapillary placoid
lesions at the retinal pigment epithelium (RPE) layer

 Leopard spot hypofluorescence on fluorescein angiography

Ocular manifestations of syphilis

 Retinal vasculitis in a patient with ocular syphilis.
Ocular manifestations of syphilis
 Chorioretinitis
Ocular manifestations of syphilis
 Syphilis chorioretinitis in a patient aged 30 years who is mentally
retarded.
Treatment
 Parenteral penicillin is DOC for all stages of syphilis.
 Doxycycline, erythromycin, or tetracycline has been
suggested for patients allergic to penicillin
Prognosis:
Ocular syphilis, if clinically diagnosed and appropriately treated
early in its course, usually results in full visual recovery.
 If syphilitic intraocular inflammation is left untreated, chronic
progressive intraocular inflammation may ensue.
 Inflammation leads to secondary glaucoma, chronic vitritis, retinal
necrosis, and optic atrophy.

Tuberculosis and the Eye

Epidemiology
 About one third of the world's population has latent tuberculosis, caused by
Mycobacterium tuberculosis.

 Roughly 9 million cases of active tuberculosis emerge annually, resulting in 2-

3 million deaths.

 Most new cases occur in the most populated nations: India and China

 The highest rates of disease are seen in sub-Saharan Africa, the Indonesian and
Philippine archipelagos, Afghanistan, Bolivia, and Peru. In these regions case rates
typically exceed 300 cases per 100 000 per year.

Tuberculosis and the Eye

Contemporary Issues

 It is recommended that all patients with tuberculosis undergo a

test for HIV.

 Health workers are at risk of tuberculosis

 Treatment of active Tuberculosis gives cure rates above

95% provided that the strain of Mycobacterium tuberculosis
 is not multidrug resistant and that compliance is adequate.

 Non-adherence to treatment may decrease cure rates to

79% and increase the prevalence of multidrug resistant
strains

Tuberculosis and the Eye

Contemporary Issues
 New York Post 1992 headline: "TB Timebomb. Homeless contaminate public areas in
city."

 In April 1993 increasing rates of tuberculosis led to the World Health Organization
declaring a global emergency.

 Tuberculosis has enjoyed a resurgence, allied as it is to economic and social fractures

and the HIV epidemic.
Tuberculosis and the Eye Contemporary Issues

 Globally, the most common opportunistic infection and the leading cause of death
related to HIV infection is tuberculosis.

 Two main approaches to tuberculosis control: casefinding combined with

treatment of active cases, and prevention of disease in infected people.

 A recent trial conducted in Zaire compared:

A. Six month therapeutic regimen:

2 months: daily rifampin, isoniazid,
pyrazinamide, plus ethambutol
4 months: twice weekly rifampin plus
isoniazid

B. 12 month regimen: twice weekly rifampin plus

isoniazid

 The rate of relapse was higher after the shorter

regimen, but survival was comparable.

 Prophylaxis of active disease with isoniazid for patients infected

 with HIV and tuberculosis is effective, but widespread
implementation of such approach in developing countries may not be
feasible.

 The United States saw a resurgence of tuberculosis in the late

1980s and early 1990s that highlighted the devastating consequences
of cutbacks in the infrastructure of tuberculosis control programmes.

 Several outbreaks of multidrug resistant tuberculosis occurred,

primarily affecting people infected with HIV.

 In some areas, like New York city, as many as 19% of the cases of
tuberculosis were attributable to multidrug resistant tuberculosis.

 Renewed attention to tuberculosis control has begun to deal with

the problem in the USA.

Tuberculosis and the Eye Contemporary Issues

HEALTH WORKERS

 Staff working with children or immunocompromised patients should have a chest

radiograph before starting work.

 Mortuary workers, microbiology staff, and those caring for patients with
tuberculosis should have a pre-employment chest radiograph and tuberculin test.

 Those who are tuberculin negative should be given BCG.

Tuberculosis and the Eye
Pathology
 The tubercle bacillus was discovered by Robert Koch in 1882

 Mycobacteria are Gram-positive, although they are not easily stained by this
method.

 They are resistant to decolorization by mineral acids after staining with

arylmethane dyes such as carbol fuchsin, hence the term acid-fast.
Tuberculosis and the Eye
Pathology
 Immune responses: either
1. protective- leading to resolution
of disease, or
2. tissue destroying- leading to
the pathological characteristics
 of active disease

 Both types of response are cell-mediated

 The characteristic histological lesion: caseating granuloma, which consists of a chronic,

compact aggregate of activated macrophages (epithelioid cells), some of which fuse to form
multinucleate giant cells

Tuberculosis and the Eye

Pathology
 Mycobacterium tuberculosis uses a trick to invade cells.

 The body's immune system normally tags any invading bacteria with proteins that alert
macrophages to consume it. One of these proteins, C2a, then floats in the blood with no known function.

 M tuberculosis manages to associate with this discarded C2a protein and use it to create a new
label that helps the bacteria adhere to the macrophage and enter it.

 Once inside the macrophage, the mycobacteria multiply until the cell ruptures and the bacteria are
then released to repeat the process
Tuberculosis and the Eye
Pathology
 The initial pulmonary lesion, the Ghon focus, together with the hilar lymph-
adenopathy, forms the primary complex of Ranke.

 Some bacilli are disseminated through lymphatics and blood, leading in some
cases to meningeal, bone, and renal involvement.
Tuberculosis and the Eye
Standard Investigations
 Chest X-Ray

 Culture: Sputum / urine culture / stain for AAFB (acid and alkali fast
bacilli).Ziehl Neelsen stain or auramine-phenol fluorescent test are confirmatory

 Tuberculin hypersensitivity

 Mantoux

 Old Tuberculin = PPD (purified protein derivative)

 Biopsy

Tuberculosis and the Eye

Standard Investigations
Chest X Ray
 Reactivation tuberculosis

Tuberculosis and the Eye

Standard Investigations
 Radiological changes are non-specific.

 Tuberculosis may cause virtually any radiological abnormality and atypical

pictures are not uncommon, especially in HIV-positive and other immuno -compromised
persons.

 Bacteriological confirmation should always be sought.

Tuberculosis and the Eye
Ocular Manifestations

 Uveitis, commonest:
chronic granulomatous anterior uveitis,
multifocal choroiditis, exudative retinitis, Vasculitis, optic nerve
edema, papilledema

 Conjunctiva- phlyctenular conjunctivitis described in some

texbooks as allegic manifestation along with erythema nodosum.

 Cornea- phlyctenular keratoconjunctivitis, interstitial keratitis

(unilateral, sectoral, superficial vascularization)

Tuberculosis of the eye

 not common, but any ocular structure may be involved.

Examples of ocular TB include:

 eyelid tubercles
 phlyctenular conjunctivitis
 granulomatous uveitis
 scleritis
 choroidal granulomas and
 optic nerve granulomas

Tuberculous phlyctenulosis
 An elevated infiltrate is seen at the limbus with a fornix-based
leash of blood vessels.
 responds well to topical corticosteroids.
Tuberculosis and the Eye
Ocular Manifestations
 Phlyctenular conjunctivitis, which may occur in some
children within 1 year of the primary infection, consists of
small, multiple, yellow or grey conjunctival nodules near the
limbus, with a sheaf of dilated vessels.
Tuberculous interstitial keratitis
 has a prolonged course and leaves residual dense opacification.
 The central cornea usually is not affected.
Tuberculous choroiditis
 symptoms usually are blurred vision and floaters.
 This patient had a 2-month history of decreased vision and presented with a
large choroidal infiltrate.
 Systemic TB was proved by culture of Mycobacterium tuberculosis. B.
 The lesion responded dramatically to systemic antituberculous medications.
Fundus Photo- multiple vascular lesions associated with vitreous hemorrhage

Choroidal Tubercles
 Latent choroidal tubercle in a patient with multidrug resistant
TB.
Tuberculous Retinitis
 Vitritis, focal retinitis with cicatrization, focal satellites,
arteritis and venous engorgement.
Tuberculous Retinitis
 fA shows focus of retinitis as a hypofluorescent area with
venous dilatation.
 Hyperfluorescent areas are satellite lesions
Tuberculous Retinitis
 Later phases show hypofluorescent area with diffuse
hyperfluorescent borders with further diffusion of contrast
from leaking veins.
Tuberculosis of the eye
 Tuberculosis patients are frequently referred to
ophthalmology because the anti-TB meds (e.g.
ETHAMBUTOL and ISONIAZID) may cause a OPTIC
 NEUROPATHY.
Case: Ethambutol toxicity in a 43 y/o
 Ethambutol was initiated at a dosage of 15 mg/kg per
day.
 3 months later, the patient had lower extremity
neuropathy, and
 2 weeks after developed a rapidly progressive, severe
decrease in vision
 OD 20/200 OS CF

 Visual fields 3 mos.

 central scotoma with inferior temporal quadrant defects.
Ethambutol toxicity is dose related
 25 mg/kg per day have a 5% to 6% incidence of optic neuropathy,
 with dosages of 15 mg/kg per day is reportedly less than 1%.
sarcoidosis
 Non-caseating granulomatous inflammation
 The major ocular manifestation is uveitis.
 large keratic precipitates (mutton fat KP).
 Iris nodules may be seen.
 Conjunctival/eyelid nodules,
 Vitritis, Retinal periphlebitis (candle-wax drippings),
 Optic nerve infiltration, and Lacrimal gland enlargement can also occur.
 Lower motor neuron 7th nerve palsy can be seen with sarcoidosis.
Sarcoid, Lacrimal Gland:
Mikulick’s Syndrome
Sarcoid in Conjunctiva:
Millet Seeds
Sarcoid
 Hilar Adenopathy

 Increase Angiotensin Converting Enzyme (ACE)

 Increase in serum Calcium

sarcoidosis
HERPES ZOSTER
 DNA VIRUS
 TRIGEMINAL NERVE
 VESICULAR ERUPTION OF THE FRONTAL BRANCH OF OPHTHALMIC
NERVE
 RESPECT THE MIDLINE
 MARKED PAIN
 SEVERE INFLAMMATORY REACTION
 NASOCILIARY BRANCH-IRITIS & KERATITIS
 Tx: ACYCLOVIR
Varicella-Zoster Virus
 Dormant virus is found in all layers of the retina, RPE and
choroid

 Uveitis: involvement of the nasociliary nerve

 Acute Retinal Necrosis: ischemia, viral spread and

granulomatous reaction
Herpes Zoster
Herpes Zoster Keratitis: Pseudodendrite
Active Varicella Zoster
Varicella Zoster Retinitis
Varicella zoster retinitis

 Progressive outer retinal necrosis – multifocal areas of retinitis with

good preservation of the inner retina and retinal vasculature
 Treatment: ACYCLOVIR

Ophthalmic Manifestations of
 Carotid Atheromatous Disease
Clinical Manifestations of Neuro-Ophthalmic
Relevance
 transient monocular blindness [ amaurosis fugax]
 branch central retinal artery occlusion [brCRAO]
 central retinal artery occlusion [CRAO]
 retinal cholesterol emboli [Hollenhorst plaques]
 chronic ocular ischemic syndrome [venous stasis retinopathy;
anterior segment ischemia]
 acute orbital ischemic syndrome
 ischemic optic neuropathy [nonarteritic AION]
Amaurosis Fugax
Pathophysiology:
 retinal vascular embolic phenomenon
- classic presentation of ipsilateral internal carotid artery (ICA)
atheromatous disease
 retinal vascular vasospasm -presumed mechanism with
migraine-related amaurotic episodes
 retinal vascular hypoperfusion
 idiopathic
embolic amaurosis fugax
 is classically 5-10 minute duration events
 - may or may not see embolic residua between events
 greatest clinical concern lies in risk of subsequent major CVA
 asymptomatic carotid / ICA bruit - 0.1-0.4%/yr
 amaurosis fugax - 1.4-2.0%/yr
 Hollenhorst plaques / retinal vascular emboli - 3%/yr
 brCRAO’s / CRAO’s - 3%/yr
 cerebral transient ischemic episode (TIA) - 8%/yr
 {12% - year1; 8% - year2; 5%/yr thereafter}
 - 20% of patients with amaurosis fugax will end up in the idiopathic
category

Hollenhorst plaques
 [= cholesterol emboli] are typically asymptomatic & located at
bifurcations; do not produce downstream ischemic effects

CRAO
 Pallid swelling of the retina
 Usually due to AS
 Giant cell arteritis is a cause in the elderly
 Cherry red spot at center due to choroidal perfusion

CRVO
CRVO
 Usu in HPN or gl
 Dilated tortuous veins
 Retinal edema
 Hemorrhages and cotton-wool spots
 Secondary NV Gl in ischemic type
 More in DM smokers, hyperviscosity syn, CRF, hyperlipidemia
Visual Field Loss
Automated Perimetry
chiasmatic field defects
 because nasal retinal fibers [= temporal fields] decussate in the chiasm,
all chiasmatic field defects respect the vertical meridian
 all chiasmatic field defects tend to be variations on theme of
bitemporal hemianopsia
anterior, retrochiasmatic pathway
pathology
 Produces asymmetric or incongruous, homonymous
hemianopsias

retrochiasmatic visual field defects

 result in homonymous hemianopsias
 -upon entering the optic tract, but proximal to
the LGB, the visual field fibers are not precisely
arrayed

posterior, retrochiasmatic pathology

produces
 symmetric = congruous, homonymous hemianopsias
 - in the calcarine cortex, the macular fibers are represented
at the occipital lobe tip and may spare these central field fibers

 calcarine cortex is split longitudinally by fissure so that selective

involvement of one half of one occipital lobe results in a
quadrantic visual field defect

Pediatric Ophthalmology
Catherine Qui-Macaraig, MD
UST
Cardinal Santos Medical Center
American Eye Center
10 warning signs of treatable eye disease in the
newborn and infant.
 These first three of the ten warning signs of significant
eye disease in infancy and childhood have the most
serious implications and most require early definitive
treatment. Fortunately, they are rare!
 Cataract - 1 in 250
 Glaucoma - 1 in 10,000
 Retinoblastoma - 1 in 20,000

10 warning signs of treatable eye disease in the

newborn and infant.
 Should be evaluated soon
 #4 Tearing, Discharge, Redness
 #5 Strabismus
 #6 Nystagmus
 #7 Prematurity - Low Birth Weight - requires monitoring of the
retina for development
 Should be evaluated at next routine visit
 #8 Abnormal Head Posture
 #9 Pupil Defect
 #10 Anisocoria

1. White Pupil (Leukocoria)

Congenital Cataract
 Cataract can be removed in infancy but creates different problems compared to adults. Because:
 1) Diagnosis must be early to avoid amblyopia, and nystagmus if cataracts are bilateral.
 2) Surgical treatment should be early to reduce amblyopia; ideally at 2 months if unilateral and
considered visually significant, 2 to 3 months if bilateral, but before onset of nystagmus.
 3) Requires special surgical technique - different from adults.
 4) Optical rehabilitation is difficult with contact lenses or glasses. IOL's are being placed in the first
year but this treatment is undergoing study and evaluation.
 5) Any delay in treatment or lapse in optical or amblyopia treatment results in amblyopia.
 6) Even in the best of circumstances vision after congenital cataract treatment is never normal.
 There is a worldwide effort to improve treatment results in congenital cataract - but much work
remains. It is obvious that if these efforts are ever to bear fruit early diagnosis is the necessary start!

Congenital Cataracts
2 Drooping Lid
 Lump or swelling of lids with or without redness, heat, or pain
(signs of inflammation)
- this can be a sign of cellulitis, tumor, hemangioma, etc.
- occlusion of the pupil can cause amblyopia - immediate treatment
is indicated.

 Bulging of the eye - proptosis with a normal size eye indicates an

orbital mass. This requires immediate diagnosis and appropriate
treatment.

Drooping of the lid (or lids)

 covering the pupil
- if the only problem is ptosis with obstruction of the pupil
 amblyopia may result or the child may assume a chin-up head back
posture which retards motor development. Surgical or nonsurgical
elevation is indicated.

3 Enlarged Cornea of One or Both Eyes

 When a sign of congenital glaucoma, this is associated with tearing and photophobia.
Congenital glaucoma requires early and specialized surgical treatment including
goniotomy, trabeculotomy, and filtering the condition is not amenable to primary
medical treatment.
 The cause - abnormal filtration mechanism

3 Enlarged Cornea of One or Both Eyes
 Optic atrophy and blindness is the end result of
congenital glaucoma if untreated However, the incidence
of these can increase dramatically on a genetic (recessive
equivalent) basis with marriage among cousins or close
blood relatives which occurs in many parts of the world.

4 Excessive Tearing or Discharge

This can be a sign of
 - increased pressure in the eye,
 - infection,
 - light sensitivity, or
 -irritation,
all of which require attention.

4 Excessive Tearing or Discharge

 The most common cause is a blocked tear drainage system.
 treated initially with gentle pressure over the tear sac and antibiotic drops. The sac may
become infected, swollen, and have purulent discharge. When this occurs in the newborn it is
called dacryocele. This usually requires immediate probing.
 Tear duct blockage which persists after one or two courses of antiboitics is best treated
by gentle probing done before age 1 year. Persistent tearing usually requires probing with
general anesthesia often with placement of a silicone tube

5 Crossed Eyes
Intermittently crossed eyes persisting after the 2 to 4 months of age should be evaluated by an
ophthalmologist to establish a diagnosis and plan treatment. Crossing can require surgery,
done in some cases as young as 4 months.
Other crossing can require treatment with spectacles. Eyes that deviate constantly should be
evaluated immediately!

6 Nystagmus
Dancing Eyes "Dancing eyes" which move from side to side, in a rotary
pattern, or up and down.
This can be a sign of eye (vision) disease, or serious disease of the brain and
should be evaluated by an ophthalmologist soon.

7 Head Tilt, Turn,Chin up or Chin down

 Some children assume a head position most evident starting at the time of sitting up or
walking.
Head tilting can be a sign of
 eye muscle imbalance (strabismus)
 disease of the central nervous system
 an abnormality of the neck muscles
Head tilt regardless of cause should be evaluated by an ophthalmologist.

8 Defect or Missing Part of the Pupil

 A defect or missing part of the pupil can also be a sign of further defect in the retina.
The pupil is the "black" center of the eye. It enlarges in the dark and with fright or pain. The
pupil gets smaller in bright light. It should always be round if normal.
 The iris gives the eye its "color". Newborns often have a gray iris. The true eye "color"
develops during the first year.

9 Inequality of the Pupil Size

 Persistent inequality of the pupil size can be a sign of serious
disease or it may be an innocent finding.
 If persistent, inequality of pupil size should be evaluated by an
ophthalmologist.
10 Prematurity
 Infants born prematurely with a very low birthweight (below approximately 4
pounds) should have a careful retina examination for signs of retinopathy of prematurity. If
the signs of this potentially blinding condition are seen, immediate treatment by an
ophthalmologist should begin.


Vision Screening
 The purpose of vision screening is to identify chldren with one or
more of the following conditions:
 1) Decreased vision in one or both eye(s) (below an arbitrary standard)
 2) Eye conditions which could lead to decreased vision in one or both
eye(s)
 3) Strabismus (crossed or wall eye)
 4) Other eye related conditions such as: ptosis (droopy lid), nystagmus
(dancing eyes), head tilt, etc.

The best age for vision screening:

 1) As near as possible to the 3rd birthday
 2) Should be done no later than by the 5th birthday - preschool between
ages 3 and 5 yrs. (for volunteer screening)
 3) Should be done at the first visit after the 3rd birthday in pediatrician or
primary care doctor's office

The principles of vision screening:

 To find out if a child can see an object of a given size with each eye.
 If a child can see the 20/40 letter in each eye while wearing corrective
glasses, the child passes.

 each eye should see at least 20/40 with no more than 2 lines
difference between the two eyes (OD 20/20 OS 20/40) is failure
Observe for:
 eye crossing
 crossed or "wall" eye
 nystagmus (dancing eyes)
 droopy lid
 head tilt

Amblyopia
 Definition: Reduced vision in one eye or occasionally
both eyes caused by pattern vision deprivation or
abnormal binocular interaction for which no causes can be
detected by physical examination of the eye and which in
appropriate cases is reversible by therapeutic
measures (from von Noorden).
  The newborn visual system can be thought of in terms of a
"clean slate". After birth, acuity improves from less than 20/200 in
the first hours and days to a nearly normal 20/40 equivalent at 1
year, and to 20/20 by school age.
 The process of central nervous system development leading to
improved vision makes the immature visual system especially
vulnerable to the harmful effects of unequal or blurred sensory
input causing suppression of the blurred image and the visual
system that receives it.

Strabismic Amblyopia
 Constant use of the left eye causes suppression of the right eye
which produces amblyopia.

 Alternation with alternate suppression avoids amblyopia.

Anisometropic Amblyopia
 * The eyes accommodate 1.00 diopter as dictated by the OD.
Anisometropic amblyopia can be difficult to detect because there are
no obvious physical signs. The problem is discovered only after
vision testing.
Deprivation Amblyopia
 Unilateral congenital cataract is highly amblyopiagenic causing
severe amblyopia early.

 Bilateral Deprivation Amblyopia

 Occurs a few months later than unilateral, but is also severe.
Ametropic Amblyopia
 Uncorrected high hyperopia is an example of this bilateral
amblyopia.
Sensitivity to development and treatment
Visual maturity after this age, amblyopia is not likely
to occur and in the usual case is not successfully
treated.
 Clinical Behavior in Amblyopia
 Vision reduced - less than 20/40, or 2 lines
difference
 Relatively better vision in reduced illumination
(scotopic sight) compared to normal
 Vision reduced by crowding: Full line acuity lower
than single optotypes

The Incidence of Amblyopia:

 Reliable statistics for the incidence of amblyopia are
lacking

disagreement regarding the impact of amblyopia

 Lesser
 * Incidence less than 1%
 * Many have 20/40 in poorer eye which is not a handicap
 * No more a problem under binocular conditions than alternating 20/20 vision
 * Employers could (will) become more enlightened
 * Effort to detect not worth expense
 * Patching and other treatment expensive and causes emotional trauma to child
 * Valid studies of the value of vision screening and amblyopia treatment lacking

The Diagnosis of Amblyopia

 Fixation - Preference for fixation with the same eye is easily
observed in the infant

 The above observation sequence confirms better vision in the

right eye. The eye with better vision is always preferred and is called
the fixing eye. Examination is then carried out to rule out an organic
defect of the left eye in this case such as optic atrophy, coloboma,
etc. If no organic cause is found, amblyopia can be diagnosed. If
the refractive error is small and refraction is equal or nearly so
meaning no anisometropia is found, patching is started. (Glasses are
rarely needed unless the patient is highly ametropic or
anisometropic.)
Example of Occlusion Therapy
  If the child is under 1 year as shown in the example above you
 can treat as follows:
 1) Patch right eye (preferred eye with good vision) 3 days -
THEN
 2) Patch left eye 1 day (this allows good eye to maintain vision
avoiding iatrogenic amblyopia)
 3) Repeat above cycle each 4 days
 4) Examine in two to four weeks
Recognition Acuity at 3 years
Pathophysiology of amblyopia
 No retinal changes - ERG OK
 Afferent pupil response has been reported but not
common
 Lateral geniculate layers subserving amblyopic eyes
atrophic
 Cortical ocular dominance columns representing
amblyopic eye less responsive to stimulus and show
changes microscopically

strabismus

Angle Kappa
The angle bet visual and central pupillary line

A nasally centered pupillary light reflex gives the appearance of exotropia and
is physiologic.

A temporally displaced pupillary light reflex gives the appearance of esotropia

and is uncommon, occurring in some cases of very high myopia.

Extraocular Muscles
 Spiral of Tillaux
 The line of insertion where the rectus muscles insert in sclera gradually farther from the
limbus beginning with the medial rectus at 5.5mm (range 3.0 to 6.0mm), inferior rectus
6.5mm, lateral rectus 6.9mm and superior rectus 7.7mm.

 It is also the line of insertion of posterior Tenon's capsule which then proceeds to the
limbus as the episclera fused with the underlying sclera.

Recti
The rectus muscles are each 40mm long.
They receive innervation on the global surface at the junction of the middle and
posterior 1/3 of the muscle.
The pulleys are located on the orbital surface at the junction of the middle and
posterior 1/3 of the globe. The pulleys are fibromuscular structures that act as
functional origins of recti

Superior Oblique
 The superior oblique (S.O.) is made up of a 30mm muscle, and 30mm tendon with the
trochlea between.
 The S.O. has its functional origin in the trochlea. This unique structure allows an 8mm
increase in the trochlear insertion distance in upgaze and an 8mm decrease in distance
between the S.O. insertion and trochlea in downgaze.

Action
Blood Supply
A long posterior ciliary artery travels from the back of the eye in
sclera beneath the horizontal recti.
The oblique muscles do not contribute to the blood supply of the
anterior segment of the eye.

Anterior Ciliaries
EOM Innervation
lateral rectus
 innervated by the sixth cranial nerve.
 The nucleus is located in the brain stem and the nerve is
 uncrossed.
superior oblique
 innervated by the fourth cranial nerve which crosses to reach the muscle.
 The nerve passes through the "stiff" tentorium making it susceptible to
the shearing force produced by brain oscillation in closed head trauma.
third cranial nerve
Innervates
 the medial rectus
 superior rectus
 inferior rectus
 inferior oblique
 levator palpebri.

 The sympathetic nerve supply travels along the innervation to

the inferior oblique.
 The parasympathetic supply travels in the nasociliary nerve.

Muscle Fibers
 At least two types of muscle fibers are present - they differ in
several parameters
 By innervation: multiple - single
 By action: slow - fast
 By layer: global - orbital

 Normal pattern of stretch and active contraction

 Contracted/overlapping/
"stiff" muscle occurs with chronic, unopposed contraction, i.e. M.R. in chronic
VI N palsy -- "lost" contracted M.R. and after injection of local anesthetics --
as in diplopia after cataract.
Multiple fascial layers and fat compartments
 Multiple fascial layers as seen with a limbal
incision
Evaluation
Cardinal Gazes
Cardinal Gazes
Hirschberg Test
Krimsky
Cover Test
 Cover-uncover Test – shows a manifest deviation e.g.
esotropia

 Alternate Cover Test – shows latent deviations too e.g.

exophoria

 Orthophoria- no shift on cover testing

Accomodative Esotropia

Bifocals for High AC/A Accomodative ET

Exotropia
Hypertropia
Incomitant Strabismus
Deviation varies in different fields of gaze
 Duane’s
 Brown’s
 III N Palsy
  VI N Palsy
 IV N Palsy
Duane’s Type 1
The Etiology of Duane Syndrome
 IIIrd N regenerates to lateral r. causing co-contraction of LR on
attempted adduction
Non-surgical: Fresnel Prisms
Strabismus surgery

Leukocoria
Catherine O. Qui-Macaraig, M.D.
Pediatric Ophthalmology
University of Santo Tomas
Department of Ophthalmology
Leukocoria
 “white pupil”
 Any white tissue mass behind a clear lens obscuring the pupil and
occurring in infants or young children
 Always consider RETINOBLASTOMA

Differential Diagnosis of Leukocoria

 Retinoblastoma
 PHPV
 Retinopathy of Prematurity
 Toxocariasis
 Coloboma of the choroid
 Uveitis
 Coat’s disease

Differentials of leukocoria cont’d.

  Retinal Dysplasia
 Vitreous Hemorrhage
 Retinal Dysplasia
 Other tumors
 Retinal Detachment
 Myelinated nerve fiber

Retinoblastoma
 The most common ocular malignant tumor of childhood
 Currently with a 90% long-term survival rate
 Unilateral in 70%
Retinoblastoma: Incidence
 11 cases per million children < 5 years or
 1 case per 18,000 live births
 Most common age at diagnosis: 18months

Retinoblastoma: Inheritance
 Rb gene: for regulation of cell division
 An inactivating mutation of both maternal and paternal alleles of
chromosome 13q14
 Knudson Hypothesis: 2 hit theory
- 2 independent mutational events

Inheritance (cont’d.)
 60% unilateral nonhereditary-present later
 40% with a heterozygous germline mutation which is inherited from
an affected parent (10%) or occurs around time of conception
 Second tumorigenic mutation occurs by chance alone
Retinoblastoma: histopathology
 Flexner-wintersteiner rosettes
 fleurettes
Clinical Presentations of Retinoblastoma
 Leukocoria
 Strabismus
 + family history
 Inflammation with tumor hypopyon
 Glaucoma
 Hyphema
 Preseptal cellulitis
 Nystagmus

Retinoblastoma: Exophytic Growth

 Growth into the subretinal space
 Retina overlies mass
 Can cause partial or total serous retinal detachment
Retinoblastoma: Endophytic Growth
 Growth into the vitreous as a single mass then cause seeding into vitreous
cavity
 a serous retinal detachment forms at the base of the tumor
Diffuse Infiltrating Retinoblastoma
 A rare subtype
 Found in older children
 Presents as a pseudohypopyon
Retinoblastoma : Diagnosis
 Ophthalmoscopic appearance
 Other presentations or masquerade syndromes
 Imaging techniques

Retinoblastoma: Diagnosis
 Unilateral, with classic fundus picture
 Calcification by CT/US
Retinoblastoma on CT Scan
Intra-lesional calcifications
Retinoblastoma on B-scan Ultrasonography
 B-scan at low gain to show endophytic mushroom shaped lesion and foci of
calcification
Histology
 Less differentiated tumors do not have increased metastatic
potential
Important Reminders
 When a fundus can’t be seen in a young child, pre-operative
imaging (CT or b-scan) is essential
 Always suspect retinoblastoma

Retinoblastoma: Natural History

 Small mass that later fills the eye
 Spreads to brain by local extension
 Blood-borne metastases to bone, bone-marrow
Retinoblastoma: Treatment
 Enucleation
 Radiation
 Chemotherapy
 Cryotherapy
 Photocoagulation
 Radioactive plaques
Enucleation
 Procedure of choice for unilateral tumors
 Use blunt tipped scissors
 Do not pass needles through sclera
 Grasp muscle stump for fixation instead
 Obtain as long a stump of optic nerve as possible

External Beam Radiotherapy

 For larger posteriorly located tumors
  Can be used in combination with chemotherapy
 Side effects: deforming effect on bone growth; inc. risk for
secondary malignancies
 For significant vitreous seeding, multiple tumors, tumors >10mm,

Radioactive Plaque
 Minimizes radiation to normal tissue
 Disadvantages:
incomplete treatment
high dose to local sclera
significant lens irradiation for anterior ones
difficulty placing posterior ones

Chemotherapy
 VP-16, VM-26, carboplatin
 In combination with laser generated hyperthermia
Factors Affecting Visual Outcome
 Tumor size
 Tumor location-macula
 Treatment modality- chemo and focal tx
 Complications
 Amblyopia
Prognosis
SURVIVAL RATES:
 No violation of lamina cribrosa: >90%
 Beyond lamina cribrosa: 60%
 + transection site: <20%

 Most common site of recurrence is anterior inferior periphery

Post-Treatment Follow-Up Care
 Risk of developing rb in 2nd eye is small after 24 months
 Examine siblings and parents
 Patients at risk for hereditary rb:
 examine q 4 months until 3 or 4 yrs. (EUA)
examine q 6 months until 6 yrs (EUA)
office exam after 8 yrs.
Late secondary Tumor Formation
 For hereditary rb, incidence is >50% by 30 yrs of age
 Osteosarcoma, malignant melanoma, fibrosarcoma, tumors of the
pineal region, leiomyosarcoma, ca of tongue, medulloblastoma, Ewing
Persistent Hyperplastic Primary Vitreous
 Usually unilateral
 Persistence and secondary fibrosis of primitive hyaloid vascular system.

PHPV
 Fibrovascular stalk emanating from optic nerve to ciliary processes and
posterior lens capsule

PHPV
 Iridohyaloid vessels over superotemporal aspect of iris dip behind to
anastomose with vascularized mass behind behind the lens
Coloboma of the Choroid
 Absence of tissue that may occur through abnormal fusion of the optic fissure
 Often associated with microphthalmia

Coat’s Disease
 Idiopathic, unilateral (80%)
 Male predilection (3:1)
 Telangiectatic and aneurysmal dilatation of retinal vessels with massive
subretinal exudate

Ocular Toxocariasis
 Invasion of the eye by the dog roundworm Toxocara canis
 Most commonly confused with retinoblastoma
 Presents as a hazy yellow-white mass
Toxocara cont’d.
 Cataract is common
 Aqueous humor cytology can differentiate the two conditions
 Treatment is with antihelminthics like thiabendazole

RETINOPATHY OF PREMATURITY
(RETROLENTAL FIBROPLASIA)

Catherine Qui-Macaraig, MD
Instructor
DEPARTMENT OF OPHTHALMOLOGY
UNIVERSITY OF SANTO TOMAS

ROP STAGE 5
RETINOPATHY OF PREMATURITY
INCIDENCE & BIRTH WEIGHT*
1,000-1251 g(2lb 3-2lb 13oz) 47%
750-999g(1lb 10oz-2lb 2oz) 78%
<750g(1lb 10oz) 90%
85% of children who have some form of ROP develop, with no intervention,
fully vascularized retina without the severe sequelae of retinal
detachment.
*Palmer EA, Flynn JT et al. Incidence and early course of retinopathy of prematurity. Ophthalmology. 1991; 98:1628-40

INCIDENCE & GESTATIONAL AGE

<28 weeks’ gestational age 80%
28-31weeks’ gestational age 60%

Multiple births are associated with an increased risk of severe

disease
Risk & severity of ROP are inversely
 proportional to birth weight & gestational age
Development of retinal vessels
16 weeks vasculogenesis:
mesenchymal spindle cells transform into capillary networks. From these
networks, mature vessels differentiate.
36 weeks mesenchyme reaches nasal ora
40 weeks mesenchyme reaches temporal ora
Pathogenesis
Injury to the vascular endothelium

Other Factors Involved

 Birth weight
 Lack of anti-oxidants (Vit. E)
 Apnea with bag and mask ventilation
 Septicemia
 Blood transfusion
 Mechanical ventilation
 Premature exposure to light-refuted

The Role of Oxygen in ROP

 Variation in oxygenation affects immature vasculature
 Incidence and severity of ROP is associated with duration of
exposure to >80mmHg O2
 It is not possible to prevent ROP with current methods of
monitoring

STAGE 5 ROP(RETROLENTAL FIBROPLASIA

RETROLENTAL FIBROPLASIA(ROP stage 5)
PLUS DISEASE(ROP)
CICATRICIAL ROP: DRAGGING OF THE
DISC
 REGRESSED ROP
Dragged Disc with
Positive Angle Kappa
DRAGGING OF THE DISC

OCULAR COMPLICATIONS OF ROP

 myopia
 strabismus
 cataract
 exudative or rhegmatogenous retinal detachment
 neovascular glaucoma
 cystoid macular edema

DIAGNOSTIC PROCEDURE
INDIRECT OPHTHALMOSCOPY
best procedure to visualize the PERIPHERAL
RETINA
in ROP

INDIRECT OPHTHALMOSCOPY

Field of view with direct ophthalmoscopy

Screening Examination of Premature Infants

for ROP
THRESHOLD ROP FOR TREATMENT
 STAGE 3 RETINOPATHY
 5 CONTINUOUS OR 8 INTERRUPTED CLOCK HOURS
  “PLUS” DISEASE
 POSTERIOR TO ZONE 3

THRESHOLD ROP
TREATMENT
 CRYOTHERAPY
 LASER PHOTOCOAGULATION
 SURGERY- SCLERAL BUCKLING
VITRECTOMY, LENSECTOMY, MEMBRANE
PEELING
EARLY DETECTION & TREATMENT IS
MOST CRUCIAL IN ROP TO PREVENT
TREATABLE BLINDNESS.

THANK YOU!

Retina
Catherine Qui-Macaraig, md
UST, CSMC, American Eye Center

Anatomy: Retina
 Thin
 Transparent
LAYERS:
 Outer RPE and Bruch’s membrane
 Inner sensory retina
 In between is Subretinal space in retinal
detachments
 Special points of attachment: optic disc and ora
serrata
Retina Function
Layers of the Retina
 (1) retinal pigment epithelium;
 (2) rods and cones;
 (3) external limiting membrane;
 (4) outer nuclear layer;
 (5) outer plexiform layer;
 (6) inner nuclear layer;
 (7) inner plexiform layer;
 (8) ganglion cell layer;
 (9) nerve fiber layer;
 (10) inner limiting membrane
Layers of the Retina
 All vertebrate retinas are composed of three layers of nerve cell
bodies and two layers of synapses. The outer nuclear layer contains
cell bodies of the rods and cones, the inner nuclear layer contains
cell bodies of the bipolar, horizontal and amacrine cells and the
ganglion cell layer contains cell bodies of ganglion cells and
displaced amacrine cells. Dividing these nerve cell layers are two
neuropils where synaptic contacts occur.
photoreceptor density in the human
retina (x 1000 cells/mm2)

 Macula (posterior pole, macula lutea, central

retina, area centralis)
 ganglion cells are reduced to a single layer
 5.5 mm diameter centered 4.0 temporal and 0.8 mm
inferior to the center of the optic disc
 Fovea (fovea centralis)
  cones
 1.5 mm diameter

Vitreous
Choroid

 Choroid
 densely pigmented and richly vascularized layer that
nourishes the outer retina and RPE
 high blood flow --> heat dissipation
 orange color of posterior segment
Macula
 The whole foveal area including foveal pit,
foveal slope, parafovea and perifovea is considered
the macula
 the most essential part of the retina for human
vision
macula lutea
 yellow pigmentation
This pigmentation is the reflection from yellow
screening pigments, xanthophyll carotenoids
zeaxanthin and lutein present in the cone axons
of the Henle fibre layer
 thought to act as a short wavelength filter,
additional to that provided by the lens
 protective mechanisms for avoiding bright light
and especially ultraviolet irradiation damage
Berlin's edema or commotio retinae
 macular edema secondary to blunt trauma to the
globe.
CME
central retinal artery occlusion
 of “cherry-red spot”
 The perifoveal edema (white opacification) does not
extend beyond the macula because of the reduced
concentration of ganglion cells in the extramacular retina.
Marked pallor of the optic disc is present.
retinal tear
 The pigment epithelium and choroid visible through
the retinal break have an orange-red color.
 The retina surrounding the tear has lost its
transparency because of edema and underlying fluid.

 Reflection from the surface of the retina during

ophthalmoscopy is attributable to the presence of a
normal internal limiting membrane
 a preretinal membrane. B. Fundus photograph of
preretinal membrane (epimacular membrane, epimacular
fibrosis, macular pucker,
OPHTHALMOSCOPY
Optic atrophy
 The disc has lost its pink color because of loss of
vascularity and replacement of neural tissue by opaque
glial tissue
Myopic Crescent
lipemia retinalis
 Cream-colored artery and vein in a patient with an
extremely high blood lipid level
arteriolosclerosis
 opacified blood vessel in the region of a previous
branch retinal vein occlusion

 Section of retina demonstrating marked thickening of

the arteriolar (A) wall as seen in arteriolosclerosis. Notice
the compression of the adjacent venule (V).
EMBOLI
  White emboli are of composed of calcium, and
their source may be either a calcified cardiac valve
or calcified atheromatous plaque.
 Cholesterol and lipid emboli, most likely from
noncalcified atheromatous plaques in the carotids,
appear yellow (Hollenhorst plaque)
 The more evanescent platelet emboli are gray-
white.
Hollenhorst plaque
lodged at the bifurcation of a cilioretinal artery
Retinal hemorrhages
 flame-shaped hemorrhage with an indistinct border. The
detached posterior hyaloid is marked by four small arrows.
 Fundus photograph of nerve fiber layer hemorrhageare oriented
parallel to the plane of the internal limiting membrane. Because of
their dispersal within the ganglion cell layer, the borders are
“feathery” (flame shaped).
Cotton wool spoots
 Multiple cotton-wool spots along with retinal
hemorrhages in a superior temporal branch retinal vein
occlusion.
hard exudates
 macular edema in a diabetic. Yellow-white edema
residues and a few paramacular hemorrhages are present
 Multiple microaneurysms are present on fluorescein
angiography
OPTICAL COHERENCE
TOMOGRAPHY(OCT)
 Low coherence interferometry
 Echo time of light backscattered from different
structures
 Light source 810nm/ 200uW(optical media)
 Micron resolution cross-sectional or
tomographic imaging in biological tissues
 Complimentary to fundus photo & FA

Fluorescein Angiography
 Side Effects
 Nausea, vomiting
 anaphylactoid reaction 91%)
 anaphylactic reaction (less than 1%)

Indocyanine Green Angiography

 Water soluble dye
 high peak absorption and emission allowing
fluoresecnce through pigment and hemorrhage
 detection of choroidal neovascularization
beneath hemorrhage
Common uses of diagnostic imaging
technology
 Choroidal neovascularization
 Chorioretinal inflammatory conditions
 Subretinal fluid accumulation
 Retinal perfusion abnormalities
 Macula edema
 Vitreomacular interface changes
Electroretinogram

Electroretinogram
  Diffuse electrical response generated by retina
 a wave - negative waveform; photoreceptor
response
 b wave- positive waveform; Muller and bipolar
response
Electroretinogram
 Photopic ERG - patient ligt adapted state to
suppress rod response then elicit ERG response
with bright white flash *cones)
 Scotopic EG - patient is dark adapted for 45
minutes to evoke response from rods
ERG
Electro-oculogram
Electro-oculogram
Symptoms suggestive of retinal dse.
 Distortion- image magnification/minification-
wavy lines
-lesions of macula or ON
 Flashes of light- retinal traction/ migraine
 Floaters- syneresis/ blood, pigment, or
inflammatory cells
 Abrupt or progressive loss of peripheral visual
field
 Abrupt or progressive dimming of vision

Thank you!
Neuro-ophthalmology
catherine qui-macaraig, md
UST, CSMC, American Eye Center
Pupils: Direct Pupillary Light Reflex
- dim lights
- ask patient to fix in the distance
- shine light on test eye

- weak with impaired afferent conduction of light in the optic nerve

Consensual Pupillary Light Reflex
-observe constriction of the fellow eye after shining light for a direct response
-usually weaker than the direct response
-not elicited by a blind eye in its fellow eye

Swinging Penlight Test

 Compares the direct with the consensual response

 Shine on R (R direct & L consensual),

 Then swing to L (direct)- should constrict more

Swinging Penlight Test

 Tests for Relative Afferent Pupillary Defect or Marcus Gunn Pupil
 RAPD- pupil widens when light is swung because consensual is stronger than
direct response
 + in optic nerve or retinal dse. (afferent pathway)
Swinging Flashlight Test
Marcus Gunn Pupil or RAPD
Testing with only one pupil
Pupils
Horner’s Syndrome: a lesion
of the sympathetic pathway
 Heterochromia iridis –lighter right-melanocyte maturation in 1st 2 years of life
 miosis –no iris dilator innervation
 Ptosis- Muller’s muscle
 Anhidrosis- pre-ganglionic
Horner’s Syndrome
 Pre-ganglionic (sup. Cervical)- cervical rib, cervical vertebral fractures, apical
lung lesions (Pancoast tumor)
 Post-ganglionic- carotid a. dissection, skull base tumor, cluster headache
Pupillary Light-Near Dissociation
 More miosis with near than light
 Light reflex-dorsal midbrain
 Near reflex- ventral midbrain
 Caused by:
 +RAPD
 tonic pupil
  Midbrain tumors
 DM/ alcoholism / encephalitis

Horner's pupil
 as accentuated after administration of topical cocaine.
 A true Horner's pupil does not dilate with cocaine. Cocaine blocks the reuptake of
norepinephrine

Adie’s Tonic Pupil

 Delayed dilate after near
 Segmental iris constriction
 From damage to ciliary ganglion or short ciliary nerves: near recover before light
 Denervation hypersensitivity
 Adie’s – with loss of DTR’s
Tonic pupil
 "Bunching" of radial folds from 2 to 4 o'clock is noted, indicating functioning
segment of sphincter. Note non-radial appearance of folds from 4 to 6 o'clock, toward
working area of sphincter, indicating that 4 to 6 o'clock sphincter segment is paretic.

Pituitary Tumor causing Parinaud’s

Pituitary Tumor causing Parinaud’s

 light-near dissociation
loss of upgaze
convergence retraction nystagmus
eyelid retraction


Ocular Motility
 Cardinal Gazes
 Cover Testing
Cardinal Gazes
Cover Test
 Cover-uncover Test – shows a manifest deviation e.g. esotropia

 Alternate Cover Test – shows latent deviations too e.g. exophoria

 Orthophoria- no shift on cover testing

Myasthenia
Gravis
Cogan’s Lid Twitch-
abn fatigability of striated m after repeated contraction
 Antibodies to the neuromuscular junction
 Anti-Ach receptor antibodies
 Tx: cholinesterase inhibitors
Sturge-Weber
 congenital cutaneous angioma ( flame nevus) in the distribution of the trigeminal
nerve, usually unilateral, homolateral
 meningeal angioma with intracranial calcification and neurologic signs,
 angioma of choroid, often with secondary glaucoma
Sturge-Weber
Tuberous Sclerosis
 adenoma sebaceum
 seizures
 mental retardation
 Ungual fibromas
Marcus Gunn Jaw Winking
Multiple Sclerosis
 Multiple white matter lesions on MRI FLAIR images. Elongated peri-ventricular
white matter lesions are known as "Dawson's Fingers".
Tensilon Test