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OPHTHALMOLOGY©POKEMONMDTRANSES

UVEA
FEUNRMFBATCH2019-20233RDYR1ST SEM|ADASTRAPERASPERA

UVEA Middle, highly vascularized, pigmented 3. ATROPHY OF THE CHORIOCAPILLARIES

BENIGN TYPE DEGENERATIVE TYPE


• Depigmentation in situ • Choriocapillaries are absent
• No functional impairment • Severe functional impairment
• Seen in simple myopia & aging

2 TYPES OF DEGENERATIVE TYPE OF ATROPHY


Generalized Choroideremia
Gyrate atrophy
Toxic retinal pigment degeneration
Focal Central choroidal sclerosis

CILIARY BODY The structure that produces aqueous humor. Contraction


of the ciliary muscle changes tension on the zonular
fibers that suspend the lens and allows the eye to focus
from distant to near objects (accommodation)
IRIS The colored part of the eye that screens out light,
primarily via the pigment epithelium.
4. HETEROCHROMIA IRIDIS
CHOROID The vascular part, pigmented tissue layer between the
sclera and the retina. The choroid provides the blood • Presence of color differences in the iris
supply for the outer retinal layers. • It doesn’t mean that if you have iris that is different in color you will
have a vision impairment
I. CONGENITAL AND DEVELOPMENTAL ABNORMALITIES HYPOCHROMIC HYPERCHROMIC
1. COLOBOMA (iris is light-colored) (iris is dark-colored)
• Failure of the optic cup to close in the retinal fissure • Simple congenital • Ocular melanosis
• “Missing tissue” in certain parts of the eye hypochromia • Ocular nevus
• Involves the inferior nasal quadrant of uvea • Horner’s syndrome • Ocular hamartoma
• Fuchs’ Heterochromic • Iris ectropion
Choroidal visible as a white patch in a dilated fundus exam (white Iridocyclitis • Pigmented tumors
Coloboma patch is actually the sclera) • Waardenburg syndrome • Siderosis bulbi
Ciliary body notching defect of the lens (dilate the pupil to examine • Non-pigmented iris tumors • Rubeosis iridis
Coloboma properly) • Trauma • Long-standing hyphema
Iris Coloboma key-hole shaped pupil • Drug-induced (Xalatan)
IRIS CHOROIDAL CILIARY
5. IRIS ATROPHY

• Blood vessel and collagen fibers are atrophied resulting in a thinned-


2. ANIRIDIA out iris and replaced by a sclerosed network of collagen tissues
• Failure of anterior growth and • Network of abnormal collagen tissues causes pooling in the iris that
differentiation of the optic cup resulting causes tears and corectopia (pupil is displaced from center)
in the loss of the iris • If severe, may cause holes in the iris resulting to polycoria (more
• Presents as a “rudimentary iris” where than one pupillary opening in the iris)
only parts of the iris are present and/or • Seen in Iridocorneal Endothelial (ICE) Syndrome
visible
6. RUBEOSIS IRIDIS
Signs and Symptoms: • Fine, tortuous, tightly meshed network of blood vessels on the
❖ Photophobia (a lot of light enters the surface & w/in stroma of the iris
eye because of large pupil) • Eventually cause neovascular glaucoma
❖ Poor vision due to corneal opacification • Very angry and red-looking eye
or cataract Risk factors:
❖ Glaucomatous optic atrophy
❖ Retinal ischemia ❖ Ocular tumors

❖ Ocular inflammatory diseases ❖ Surgical complications

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II. UVEITIS Irregularly shaped pupils and non-
• A condition that involves inflammation of the uveal tract (iris, ciliary eccentric because of adhesions. They
body, choroid) and adjacent ocular structures (retina, optic nerve, have very poor dynamics and does not
vitreous, sclera dilate or constrict normally anymore

STEPS IN EXAMINATION OF PATIENT WITH UVEITIS


1. HISTORY TAKING
• Good patient history and thorough examination are indispensable 3. SLIT LAMP EXAMINATION
❖ Saves the patient from undergoing unnecessary investigations • A slit-lamp examination is performed to evaluate the different
❖ Provides the basis for the generation of differential diagnoses structures in the eye
❖ Allows determination of response to treatment
STRUCTURE FINDINGS
• Systemic association is common in Uveitis A. CORNEA KERATIC PRECIPITATES (KPS)
❖ Joint pains in arthritis-related uveitis • Shapes are difficult to differentiate
❖ Skin changes (psoriasis) • Round deposits of Inflammatory cells on corneal
❖ Auto immune diseases (RA and SLE) endothelium
❖ TB-related uveitis • They may be large (Mutton fat KPs), small, medium,
❖ Other systemic infections causing uveitis: pigmented or non-pigmented
o Toxoplasma (from cats) o Herpes virus infections • Usually located in lower 2/3 of the cornea in a
o Toxocara (from dogs) o HIV-AIDS triangle base down configuration called the Arlt’s
triangle
*Important to do a targeted review of systems* • Slit lamp examination is used to appreciate KPs

Common Signs and Symptoms of Uveitis DENDRITES (HERPES OR VIRAL INFECTION)


❖ Eye redness ❖ Tearing ❖ Pain or Discomfort • These are swollen corneal nerves (virus laden)
❖ Blurring of vision ❖ Photophobia ❖ Floaters
BAND KERATOPATHY
2. BASIC EYE EXAMINATION • Calcium deposits on corneal epithelium or Bowman’s
layer
EXAMINATION UVEITIS FINDINGS • Caused by chronic inflammation
Visual Acuity • Usually reduced from a combination of problems B. IRIS • “Moth eaten” iris or thinned out
involving various eye structures such as the cornea, • Absence of crypts (more smooth or velvety
lens, vitreous, optic nerve, etc. appearance)
• Determines the source of poor vision as this helps • Iris atrophy (depigmented areas)
in determining the mode of treatment. • Formation of membranes (over the iris)
• Adhesions (synechia) due to membrane (iris is stuck
*If the problem is in front of the eye, use topical to the lens capsule)
medications but if it is in the back of the eye, use other • Inflammatory deposits may be seen as iris nodule
mode of treatment like injection*
Extraocular • Generally, not affected Nodule is labeled depending on its location
Muscles (EOM) ❖ Koeppe’s Nodules located in the pupillary borders
*If it is a uveitis secondary to another cause like a mass ❖ Busacca Nodules located in the body of the iris
in the eye, you can have problems in EOM movement* ❖ Berlin’s Nodules located in the angles of the eye
*All nodules present with Heterochromia*
Intraocular • Can be normal (10-21 mmHg) or decrease/hypotonic
Pressure (IOP) or increased/hypertonic/Firm/Hard C. ANTERIOR CELLS and FLARE
CHAMBER ❖ Cells: Immune cells that are sign of active
inflammation
*If decreased, there is ciliary body involvement and IOP
❖ Flare: increased protein content in aqueous due to
pressure is usually < 10 mmHg *
damaged iris blood vessels
*If increased, there is open angle closure glaucoma
caused by steroids causing ocular hypertension and IOP *Lesions are graded from 1 to 4 with 4 is the most
pressure is usually > 21 mmHg but the severity depends severe*
on how high the pressure is* D. ANTERIOR • Increased cells and protein
VITREOUS • Arise from choroid, retina and ciliary body
Pupils • Irregular shaped, eccentric caused by adhesions of
• It is graded as well according to certain levels (same
the iris to lens capsule
with anterior chamber)
• Poor dynamics due to adherence to the lens and
E. LENS Presents with cataracts
membrane formation causing the eye hard to dilate
❖ Underlying inflammation
Conjunctiva • The conjunctiva may be injected, presenting in
❖ Cataract of the young
varying patterns (conjunctival, perilimbal, ciliary
❖ Steroid use
and mixed).
❖ Conjunctival redness due to conjunctivitis
❖ Ciliary redness due to inflammation of ANTERIOR CHAMBER MNEMONICS:
deeper structures (area of ciliary body) ❖ Koeppe’s Nodules- SPELLED WITH A P- pupillary borders
❖ Pericorneal redness due to keratitis ❖ Busacca Nodules- SPELLED WITH AN S -body of the iris
❖ Mixed redness mostly seen in ulcers ❖ Berlin’s Nodules- SPELLED WITH A L- angles of the eye

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*TRACTION RD and SEROUS RD are NONE RHEGMATOGENOUS RD*
4. FUNDUS EXAMINATION (EXAMINATION OF POSTERIOR EYE) H. SUBRETINAL • Appear as plaques and bands of yellow white
Indirect • Ideal for defining extent and height of retinal and FIBROSIS tissue
Ophthalmoscope choroidal lesions • Scars and fibrosis may be observed serving as
• Penetrates vitreous haze and media opacities signs of previous infection or inflammation
• It is easier to view the back of the eye using • Found deep in the retina
indirect ophthalmoscope I. CHOROIDAL • Appear as grayish yellow elevated masses
78/90D Lens • Hand-held lens with a slit lamp LESIONS • This is underneath the retina because the
• Can view same structures seen in indirect vessels still traverse over it
ophthalmoscope but this provides a larger view J. CYSTOID • Macula may be edematous
while the indirect ophthalmoscope will give a MACULAR • Caused by inflammation of the uvea
wider view EDEMA • Very important cause of blurring of vision in
• Provides inverted view intermediate uveitis
• Ideal for viewing: • Flower formation or petaloid appearance in
❖ Vascular abnormalities fluorescein angiography is pathognomonic seen
❖ Intraretinal lesions thru the OCT (Ocular Coherence Tomography)
❖ Vitroretinal tractions K. RETINAL • Pars Plana Snowbanking
Hruby Lens • Penetrates haze better than 78/90D PERIPHERY • Accumulation of white fibroglial mass over pars
• Better for assessing macular edema plana and adjacent retina
Mirrored • Detailed exam of peripheral chorioretinal lesions • Usually restricted to inferior pars plana
Contact Lens e.g. Gonioscopy lens (used in glaucoma) L. OPTIC NERVE • Neuritis • Neovascularization
• Disc edema • Atrophy
• Papilledema • Glaucomatous nerve
FINDINGS OF FUNDUSCOPIC EXAMINATION IN UVEITIS
A. VITREOUS • Snowballs • Membranes
CLASSIFICATION OF UVEITIS
• Haze • Vitreo-retinal
1. Anatomical 5. Age-group / Race
• Strands traction
2. Infectious vs Non-infectious 6. Unilateral vs Bilateral
B. VASCULAR Vascular Sheathing
CHANGES • inflammatory cells around blood vessels 3. Onset and Course of Inflammation
• this is like the vessels are being enveloped by 4. Granulomatous vs Non-
white membrane or sheathe granulomatous
• this is actually inflammatory cells around the
blood vessels 1. ANATOMICAL CLASSIFICATION OF UVEITIS

Hemorrhages, Exudates and Cotton Wool Spots TYPE PRESENTATION INFLAM. SITE
• related to retinal ischemia ANTERIOR • HLA-B27-related: • Anterior Chamber
• conditions that causes retinal ischemia include ❖ Ankylosing • Everything in front
hypertension and diabetes spondylitis of the lens
C. INFILTRATES • Similar to cotton wool spots ❖ Reiter’s and JIA
• Found deeper within the retina ❖ Viral
D. GRANULOMAS • Also found deeper within the retina but are • Iritis and Iridocyclitis
larger and more consolidated
• Organized collection of macrophages POSTERIOR • Posterior scleritis • Retina or Choroid
• Masses such as tubercles, granulomas, tumors • Retinitis
may also be seen affecting the retina and the • Choroiditis
choroid • Papillitis
• Condition that can cause granuloma is TB of the • TB
eye
E. DEPIGMEN • Seen in very specific condition which is called
-TATION Vogt-Koyanagi-Harada (VKH) disease and INTERMEDIATE • Pars planitis • Vitreous
Sympathetic ophthalmia – Dalen-Fuch’s nodules • Cyclitis • Pars Plana
• There is fading in the normal yellow-orange color • TB • Middle part of the
of the retina and choroid (like in disorders of eye (behind the
choriocapillaris) pupil and lens)
F. ATROPHIC • Scars surrounded by areas of hyperpigmentation
LESION • Sign of old inactive uveitis seen in Toxoplasma
Scar PANUVEITIS • Diffuse Uveitis • All parts of the eye
G. RETINAL • Varying degrees of retinal detachment but is • TB and VKH
DETACHMENT typically exudative or serous type in nature • Toxoplasma and
• Retina can be seen as shallow or bullous Toxocara
Other types of Retinal detachment: • Most severe type
❖ Traction RD – membranes are found in the
fundus and they pull on the retina.
Eventually, they produce Rhegmategenous
RD
❖ Serous/Exudative RD – most common form
❖ Rhegmatogenous RD – tear, rip or hole in
the retina

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2. INFECTIOUS VS NON-INFETIOUS D. SKIN TESTING • Allergy testing • Histoplasmin
• Anergy testing • Kveim - Sarcoidosis
INFECTIOUS NON-INFECTIOUS • Behcetin/Pathergy • PPD
• Toxocara • HIV-AIDS • JIA • Behcet’s E. BIOPSY SPECIMEN • Conjunctiva • Choroid and retina
• Toxoplasma • TB • Ankylosing • Vogt- • Lacrimal gland • Skin
• Herpetic spondylitis Koyanagi- • Aqueous humor • For malignancies
Harada • Vitreous

Malignancies: SUMMARY OF OTHER ANXILLARY TESTS


❖ Melanomas
❖ Lymphoma INVESTIGATION SUSPECTED ETIOLOGY
❖ Retinoblastoma (common 1. Sacroiliac Joint X-Ray • HLA-B27 related disease
in children) (AS)
2. Angiotensin Converting Enzyme • Sarcoidosis
COMPLICATIONS OF UVEITIS (ACE), Kveim Test& Chest CT
• Cystoid macular edema • Complicated cataracts 3. Toxoplasma Dye Test/ IgG • Toxoplasmosis
• Secondary glaucoma (due to • Retinal detachment Antibodies
neovascular growth causing • Band keratopathy (obscures the 4. ELISA • Toxocara
rubeosis) view of the posterior part of the eye)
5. HLA-B typing and Behcetin • Behcet’s Disease
• Neovascularization
6. ANA, RF and X-rays of joints • JRA
7. Mantoux test and Chest X-Ray • Tuberculosis
ANCILLARY EXAMINATIONS
8. CT Scan of Orbits/ B-Scan • Posterior Scleritis
Ultrasound
It is important that we request for additional tests and diagnostics
9. MRI Head Scan • Demyelination
judiciously because resources are finite, Will it identify any underlying
• Non-Hodgkins lymphoma
systemic disease process or association?
• Neurosarcoidosis
Will it provide a “definitive” etiology? Will it confirm or reject a diagnosis?
Will it help in the management of the patient? If yes, then the test is 10. CSF Studies • Demyelination
warranted. • Non-Hodgkins lymphoma
• VKH
11. Polymerase Chain Reaction (PCR) • Herpesviral DNA
of Intraocular Fluid • Propionibacter DNA
GENERAL INVESTIGATIONS
❖ CBC, ESR, CRP (underlying systemic ❖ Chest x-ray
disease) ❖ PPD Skin Test TREATMENT OF UVEITIS First line of management is medical and
❖ Syphilis serology Goal of Treatment: various medications may be given
❖ Urinalysis
• Control the inflammation through different routes
• Control the infection
Any other tests that need to be ordered would depend on the clinical findings
• Treat complications
and index of suspicion for a particular diagnosis.
MEDICAL MANAGEMENT
• Color vision testing • Visual evoked potential
1. Topical (drops, gels, ointments)
• Electroretinogram • Visual field testing
• Electrooculogram • OCT (Optical Coherence) ❖ Mydriatic-cycloplegics (dilating drops)
• Fluorescein angiography • ICG Angiography (Indocyanine ❖ Anti-inflammatory medications (NSAIDS and corticosteroids)
• Laser interferometry Green) ❖ IOP lowering medications (anti-glaucoma medications)
• Laser flare reading • Imaging – xray, CT ❖ Antibiotics (anti-bacterials, anti-virals, anti-fungals)
• Ultrasound *Used when the frontal part of the eye is affected *
ANCILLARY TEST UVEITIS FINDINGS and REMARKS
A. ULTRASOUND OF • Choroidal thickening 2. Regional/Periocular/Intravitreal Injections
THE EYE • Vitreous condensation/ bands ❖ Corticosteroids
(warranted if there is • Vitritis ▪ Triamcinolone
no view of the • Retinal detachment and masses ▪ Dexamethasone
posterior segment of ❖ Antibiotics (anti-bacterials, anti-virals)
the eye) ❖ Anti-Vascular Endothelial Growth Factor (for signs of ischemia and
B. FLUORESCEIN • Cystoid macular edema diabetic retinopathy)
ANGIOGRAPHY • Neovascularization ▪ Ranibizumab
(requested to further • Disc leakage ▪ Bevacizumab
characterize fundus • Vessel leakage *Used when the posterior part is affected*
pathologies) • Staining and pooling
• Retinal lesions 3. Uveitis Associated with Systemic Illness (Oral/Intravenous)
C. IMAGING TESTS • Cranial CT Scan ❖ Corticosteroids
• CT scan of sinuses ▪ Prednisone
• Gallium scan ▪ Methylprednisolone
• Hand x-ray ❖ Immunomodulatory agents (antimetabolites, biologics, etc.)
• Cranial MRI ❖ IOP lowering medications (anti-glaucoma medications)
• Chest x-ray ❖ Antibiotics (anti-bacterials, anti-virals, anti-fungals)
• Sacroiliac x-ray (for ankylosing spondylitis)

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SURGICAL MANAGEMENT
For patients who are unresponsive in medications due to severity of the
disease
1. Glaucoma Surgery for uncontrolled, elevated IOP
2. Cataract Surgery uveitic cataracts, steroid-induced cataracts
3. EDTA Chelation for band keratopathy applied in the cornea to
be able to scrape the calcium deposits
4. Corneal Transplant for corneal decompensation, ulcers, opacified
scars
5. Retinal Surgery for retinal complications such as retinal
detachment, traction membranes, intraocular
infections

EMERGING DISEASES
• New and emerging diseases have been reported in recent years
• Case reports have documented ocular inflammation in Dengue,
Chikungunya, Ebola and Zika infections but further studies are warranted to
fully understand the pathophysiology of ocular inflammation in these diseases
• COVID 19 can cause conjunctivitis and could be transferred through tears

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