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ANATOMY OF

THE UVEA
RUTH ANTOLIN, MD
DOH EYE CENTER

UVEAL TRACT
Main vascular compartment of the eye
Uvea was derived from the Latin word uva: grape
Located between the sclera and the retina
3 parts
Iris
Ciliary body
Choroid

UVEAL TRACT
Attachment
Scleral spur
Exit points of vortex veins
Optic nerve

EMBRYOLOGY OF THE
UVEAL TRACT
has two components.
anterior three fourths (i.e., the stroma) is from the neural
crest cells, mesoderm
posterior one fourth (i.e., the muscles and the pigmented
epithelium) is neuroectodermal.
The sphincter and the dilator muscles : from the outer lamina of the primitive
optic cup,
pigmented epithelium layer: from the inner layer of the optic cup.
Thus, the pupillary ruff (margin) in a fully developed eye represents the
anterior tip of the primitive optic cup.

IRIS
Most anterior portion of the uvea
Measurements:
12mm dm
37 to 38mm circumference
0.5mm thickness
iris is thickest near the collarette and
thinnest at the iris root

musculovascular diaphragm with a


central opening, the pupil ; blood
vessels, connective tissue and
melanocytes (pigmentation)
Divides the anterior segment of the
eye into ant and postchambers

IRIS: ANTERIOR
RELATIONS
Anteriorly: Aqueous humor
Posteriorly: lens
Peripherally: aqueous humor in the posterior chamber

IRIS
Macroscopically:
the anterior surface is
irregular, with furrows
and crypts.
The pupillary zone is
located between the
collarette and the
pupillary ruff.
the remainder of the iris
is called the ciliary zone.
The fringed iris collarette
is located approximately
2 mm from the pupillary
border; and is the
thickest portion of the iris

IRIS

IRIS
Posterior Iris Surface
smoother and more uniform
2 layered: anterior and posterior iris epithelia
relatively shallow furrows

Radial folds:
Contraction folds of Schwalbe- small sulci that extends
from the pupillary aperture to the anterior surface of the iris
Structural folds of Schwalbe- extends 1.5mm from the
pupillary border and is seen between the ciliary processes.

IRIS: Stroma
Contains pigmented and non pigmented cells, collagen
fibrils, blood vessels, nerves and a matrix of hyaluronic
acid
Difference in color is due to the amount of pigmentation
of the stroma
Blue:
lighter pigmentation

Brown:
heavy pigmentation

IRIS: ANTERIOR
PIGMENTED EPITHELIUM
2 parts:
Muscular basal portion:
makes up the pupillary dilator muscle
o 4microns in thickness
o arranged in an overlapping manner; joined by tight
junctions
Epithelial apical portion
o Almost similar to the posterior pigment epithelium
o Separated from the posterior pigment epithelium via intercellular
spaces.

IRIS: Posterior
Pigmented Layer
Continuous with the
nonpigmented epithelium
of the CB and with the
neurosensory retina
Curves around the pupillary
margin to form the pigment
ruff

IRIS: Posterior
Pigmented Layer

IRIS: Vessels and Nerves


Supplied by the major arterial circle
which is formed by the long posterior
ciliary arteries and anterior ciliary
arteries

IRIS: Vessels and Nerves


Minor arterial (vascular)
circle formed by
anastomoses between
arterial and venous
arcades at the region of
the iris collarette
Myelinated and non
myelinated nerve fibers
are present throughout
the stroma.

IRIS: Dilator Muscle


Derived from the ectoderm;
composed of smooth
muscle cells with
myofilaments and
melanosomes
Has both parasympathetic
(inhibitory role) and
sympathetic innervation

IRIS: Dilator Muscle


Sympathetic chain
1st ON:
Hypothalamus to T1
2nd ON:
T1 to sup cervical ganglion
3rd ON:
Sup cervical ganglion to Dilator
muscle

IRIS: Sphincter Muscle


Also derived from the
neuroectoderm
Composed of circular band
of smooth muscle fibers
located near the pupillary
margin
Primary innervation is via
parasympathetics from CN
III

IRIS: Sphincter Muscle


Parasympathetic Pathway
EW nucleus to ciliary
ganglion to iris sphincter

CILIARY BODY

CILIARY BODY
Connects the anterior and
posterior segments of the
uvea
Apex is directed towards the
ora serrata
Base gives rise to the iris;
also serves as attachment of
the CB to the sclera via the
scleral spur
Major functions:
aqueous humor
production
lens accomodation

CILIARY BODY
6-7 mm wide
2 parts:
pars plana 4mm wide;
extends from ora serrata to
ciliary processes
Safest posterior surgical approach
to the vitreous cavity
Located 3-4mm from the corneal
limbus

pars plicata- has ciliary


processes that serves as
attachment of the zonular
fibers of the lens

CILIARY BODY

CILIARY BODY

CILIARY BODY
Each ciliary process is supplied by arterioles from the
major arterial circle
Venous drainage of each process is via 1-2 venules
located at the crest
Aqueous humor production is dependent on the blood
flow within the capillary network of the ciliary processes
which is governed by sphincter tone, neuronal innervation
and humoral vasoactive substances
Functions:
1. for accommodation
2. formation of aqueous humor
3. keeps the lens in place

CILIARY BODY
Microscopic structures:
1. supraciliary lamina
2. Ciliary body stroma- surrounded by flattended fibrocytes
rather than collagen fibers
3. Pigmented Epithelium Layer
4. Non Pigmented Epithelium Layer
5. Internal Limiting Membrane

CILIARY BODY: Histology


Double layer of epithelial
cells
Non pigmented: inner
layer; situated
between the post
chamber and the
pigmented epithelium
* Zonula occludentes
(tight junctions), located
along the lateral intercellular
spaces near the apical border
of the NPE, maintain the
blood aqueous barrier.

CILIARY BODY: Histology

Pigmented:

outer layer; basal


layer is located on
the iris stroma

Organelles (ER and


Golgi complex) vital
in aqueous humor
production

CILIARY BODY: Histology


NPE

Location of
Basal
Lamina
Width

Nucleus
Melanosom
es
Cell type

Pigmente
d
Epitheliu
m

Inner
Outer
Borders the Faces the
post
iris stroma
chamber
Thick
Thin
More
homogenou
s
Large
Few

Large
plenty

Cuboidal
(pars
plana)
Columnar
(pars
plicata)

Cuboidal
(throughout
)

CILIARY BODY: Blood


Supply
Main arterial supply
from the anterior and
long posterior ciliary
arteries
Superficial episcleral
plexus
Intramuscular plexus
Major arterial circle

CILIARY BODY: Blood


Supply
Venous drainage via the vortex system, intrascleral
venous plexus and episcleral veins

CILIARY BODY: NERVE


SUPPLY
mainly derived from the parasympathetic fibers of CN III
via short ciliary nerves
97% to the ciliary muscle
3% to the iris sphincter

CILIARY BODY: Ciliary


Muscle
3 layers
Longitudinal: outer layer
attached to the scleral
spur
Radial / middle oblique
Circular: innermost
portion
Ciliary muscle has also been linked
to the development of presbyopia
due to increasing amounts of
connective tissue between muscle
bundles and loss of muscle
elasticity that is responsible in lens
accommodation.

CILIARY BODY: Ciliary


Muscle

CILIARY BODY: Ciliary


Muscle
Behave like smooth, non striated muscle fibers with
multiple myofibrils, prominent nucleus, electron dense
attachment bodies, mitochondria, glycogen particles
Rich in type VI collagen
Muscles inserts into the scleral spur and around tips of
oblique and circular muscle fibers
Innervation: parasympathetic via ciliary nerves

CHOROID

CHOROID
posterior portion of the uvea
Provides nourishment to the outer portion of
the retina
Regulates IOP
Heat diffuser

Measures 0.25mm in thickness


Located between the sclera and the retina;
extending from the ora serrata to the optic
nerve

CHOROID

3 layers of vessels
Choriocapillaris
Middle layer
(Sattler) of small
vessels
Outer layer
(Hallers) of large
vessels

CHOROID

CHOROID
perfusion comes from the
long and short ciliary
arteries, perforating
anterior ciliary arteries
Venour drainage via the
vortex veins
Blood flow compared to
other tissues is high;
oxygen content of venous
blood 2-3% less than that
of arterial blood

CHOROID: Bruchs
Membrane
Lamina situated between the RPE and the choriocapillaris
Extends from the optic disc margin to the ora serrata
5 elements:

Basal lamina of the RPE


Inner collagenous zone
Band of elastic fibers
Outer collagenous zone
Basal lamina of the choriocapillaris

CHOROID: Bruchs
Membrane

CHOROID: Bruchs
Membrane
Defects can develop spontaneously such as in myopia or
pseudoxanthoma elasticum or via trauma or inflammation
Subretinal neovascular membranes may develop from
these defects that can lead to disciform macular changes
and ocular histoplasmosis syndrome

CHOROID:
Choriocapillaris
Continuous layer of large
capillaries beneath the RPE.
Vessels facing the retinal
surface are extremely thin
and contain multiple
fenestrations with pericytes
line the outer wall.
Middle and outer choroidal
vessels are not fenestrated.

CHOROID:
Choriocapillaris
melanocytes, macrophages, lymphocytes, mast cells and
plasma cells
are abundant in the choroidal stroma.
Pigmentation depends on the number of pigmented
melanocytes in the choroid and RPE
Degree of pigmentation in the choroid must be
considered when one is performing photocoagulation
because it influences absorption of laser energy

CLASSIFICATION,
SIGNS AND
SYMPTOMS OF
UVEITIS

OVERVIEW:
uvea consists of the middle, pigmented, vascular of the
eye
Iris
Ciliary Body
Choroid

Uveitis- (-itis) inflammation (uva) uvea


study of uveitis can be categorized into infectious and
non infectious
uveitis is frequently associated with systemic diseases*

CLASSIFICATION
Uveitis classification is spearheaded by the
Standardization of Uveitis Nomenclature (SUN) Working
group in 2005
based on :

Anatomy: what portion of the uvea is involved?


Course: acute? Chronic? Recurrent?
Etiology: infectious or non infectious
Histology: granulomatous or non granulomatous

Anatomical Classification of
Uveitis (SUN Working Group)
Type

Primary Site of
Inflammation

Includes

Anterior Uveitis

Anterior Chamber

Iritis
Iridocyclitis
Anterior cyclitis

Intermediate
Uveitis

Vitreous

Pars Planitis
Posterior cyclitis
Hyalitis

Posterior
Uveitis

Retina or choroid

Focal, multifocal,
diffuse choroiditis
Chorioretinitis
Retinochoroiditis
Retinitis
Neuroretinitis

Panuveitis

Anterior chamber,
vitreous, retina or choroid

Anatomical Classification of
Uveitis: Anterior Uveitis
can range from a quiet looking eye with low grade
inflammatory reaction to a painful red eye with moderate
to severe inflammation
Iritis: anterior chamber only
Iridocyclitis: anterior chamber + retrolental space
keratouveitis: cornea
sclerouveitis: sclera and uveal tract

Anatomical Classification of
Uveitis: Intermediate Uveitis
inflammation of the middle portion (posterior ciliary
body, pars plana)
manifests primarily as floaters
frequently appears quiet externally
Vision loss can result from chronic cystoid macular edema
(cme), cataract formation

Anatomical Classification of Uveitis:


Posterior Uveitis and Panuveitis
involves the retina and/or choroid
ocular exam: focal, multifocal, diffuse areas of retinitis or
choroiditis with varying degrees of vitreous cellular activity and
involvement of the retinal vasculature
structural complications of uveitis such as macular edema,
retinal vasculitis, retinal/ choroidal neovascularization not
considered essential in classifying posterior uveitis
Panuveitis:
diffuse uveitis
Associated with systemic (non infectious/ infectious)diseases such as
VKH, syphilis and cysticercosis

ANATOMICAL CLASSIFICATION:
POSTERIOR UVEITIS with RETINITIS
Focal Retinitis

Multifocal Retinitis

Toxoplasmosis

Syphilis

Onchocerciasis

HSV

Cysticercosis

VZV

Masquerade syndromes

CMV
Diffuse Unilateral
Subacute Neuroretinitis
Candida infection
Sarcoidosis
Cat scratch disease
Masquerade syndromes

ANATOMICAL CLASSIFICATION:
POSTERIOR UVEITIS with
MULTIFOCAL RETINAL LESIONS
With Vitreal Cells

Without Vitreal Cells

Birdshot
retinochoroidopathy

Ocular Histoplasmosis
Synd

Multifocal choroiditis
and panuveitis

Punctate Inner
Choroiditis

Subretinal Fibrosis and


uveitis

Punctate Outer Retinal


Toxoplasmosis

Vogt- Koyanagi- Harada

Acute Retinal Pigment


Epithelitis

Sarcoidosis

Subacute Sclerosing
Panencephalitis

West Nile Virus

Serpiginous choroiditis

Cat Scratch disease


Malignant Masquerade
Syndromes

ANATOMICAL CLASSIFICATION:
POSTERIOR UVEITIS with RETINAL
VASCULITIS
Primarily Arteritis Primarily
Phlebitis

Arteritis Phlebitis

SLE

Sarcoidosis

Toxoplasmosis

Polyarteritis nodosa

Multiple Sclerosis

Relapsing
polychondritis

Syphilis

Behcet

Wegener
granulomatosis

HSV

Birdshot
Choroidopathy

Crohn disease

VZV

HIV paraviral
syndrome

Frosted branch
angiitis

Idiopathic Retinal
Vasculitis,
Aneurysms and
Neuroretinitis

Eales disease

Churg Strauss

ANATOMICAL CLASSIFICATION:
POSTERIOR UVEITIS with FOCAL
CHORIORETINAL LESION
With Vitreal Cells

Without Vitreal Cells

Toxocariasis

Tumor

Sarcoidosis

Serpiginous choroiditis

Tuberculosis
Nocardia
Cat Scratch

UVEITIS CLASSIFICATION BASED


ON THE CLINICAL COURSE
Acute:

sudden onset and limited duration that usually


resolves within weeks to months

Recurrent: repeated episodes separated by periods of


inactivity
without treatment for > 3 months
duration
Chronic:persistent; relapse occurs in less than 3
months after
discontinuing treatment

Further divided into


granulomatous has lymphocytic and plasma
infiltrate
non granulomatous- epitheloid and giant cells

SYMPTOMS

SYMPTOMS OF UVEITIS
depends on the part of the uveal tract affected, onset,
duration

Pain
Photophobia
Redness
Blurred vision
Pupillary miosis/ irregularity
Floaters- shadows caused by vitreous cells and snowballs on
the retina
Decreased visual acuity
Disturbances in vision: photopsia, metamorphopsia,
scotomata
nyctalopia

SYMPTOMS OF UVEITIS
Pain and Photophobia
primarily related to ciliary spasm.

Pain physiology can be explained by axon reflex


wherein there is a retrograde reflex from the inflamed
peripheral nerves (cornea and iris) to the blood vessels
causing vasodilation or to the muscles causing
contraction
Cycloplegia is therefore useful in cases of iritis and in
some cases of keratitis because paralysis of the ciliary
muscle alleviates the pain of ciliary spasm

SYMPTOMS OF UVEITIS
Blurred vision
may be caused by cloudy media, although vision is
often surprisingly good in the presence of dense
inflammatory reaction in the anterior chamber and
vitreous.
Floaters are a more frequent manifestation of cells
and debris in the vitreous cavity.
Macular edema, which frequently occurs with both
anterior and posterior uveitis, can produce blurred
vision, micropsia, and metamorphopsia.

SYMPTOMS OF UVEITIS
Ciliary Injection
Ciliary injection, or ciliary flush, is manifest by a
ring of dilated episcleral vessels radiating from the
limbus.
should be distinguished from scleritis and from
episcleritis. Overlying conjunctival injection may
mask ciliary flush but topically applied
neosynephrine blanches the overlying conjunctiva,
allowing visualization of deeper episcleral vessels.

BAND KERATOPATHY

SIGNS

SIGNS OF UVEITIS:
ANTERIOR CHAMBER
brought about by inflammatory response to infectious,
traumatic, neoplastic, autoimmune
chemical mediators and inflammatory cells play a role
reactions are usually appreciated in the anterior chamber
wherein they can be described as

serous
purulent (presence of hypopyon)
Fibrinous (plasmoid)
sanguinoid (hypopyon + Hyphema)

GRADING OF ANTERIOR
UVEITIS (SUN Working
Group)
According to the number of cells in field (1 x 1mm slit
beam)
Grade

Cells in Field *1 x
1mm slit beam

<1

0.5+

1- 5

1+

6- 15

2+

16- 25

3+

26- 50

4+

> 50

GRADING OF ANTERIOR
UVEITIS (SUN Working
Group)
According to the flare intensity
Grade

Description

None

1+

Faint

2+

Moderate (iris and lens


details clear)

3+

Marked (iris and lens


details hazy)

4+

Intense (fibrin or
plasmoid aqueous)

SIGNS OF UVEITIS:
ANTERIOR CHAMBER
if with uveitic involvement of CB
and TM, IOP is usually low due to
decreased aqueous production
or increased alternative outflow
If iris is involved, the following
may manifest
synechiae (anterior or posterior)
iris nodules (Koeppe, Busacca,
Berlin nodules)

Iris granulomas
heterochromia
stromal atrophy

SIGNS OF UVEITIS IN
THE ANTERIOR
SEGMENT
keratic precipitates
inflammatory cells
flare
fibrin
Hypopyon

pigment dispersion

pupillary miosis
iris nodules
synechiae both anterior and posterior
band keratopathy

SIGNS OF UVEITIS:
INTERMEDIATE SEGMENT
Grade

Number of Cells

Description

No cells

Clear

0.5+/ trace

1- 10

Trace

1+

11- 20

Few opacities, mild


blurring

2+

21- 30

Significant blurring
but still visible

3+

31- 100

Optic nerve visibile,


no vessels seen

4+

> 100

Dense opacity
obscures optic nerve
head
Consensus between National Institutes of Health (NIH) grading
system and SUN group

SIGNS OF UVEITIS:
INTERMEDIATE
SEGMENT
Additional uveitic changes
Snowball opacitiessarcoidosis or intermediate
uveitis
Snowbanks- pars planitis
Vitreal strands

SIGNS OF UVEITIS:
POSTERIOR SEGMENT
retinal or choroidal inflammatory infiltrates
inflammatory sheathing of the arteries and veins
retinal detachment (exudative, tractional,
rhegmatogenous)
retinal pigment hypertrophy or atrophy
atrophy or swelling of the retina, choroid, optic nerve
head
preretinal/ subretinal fibrosis
retinal/ choroidal neovascularization

SIGNS OF UVEITIS
Eyelid and skin: vitiligo and nodules
Conjunctiva: perilimbal diffuse injection; nodules
Corneal endothelium: KP, fibrin, pigment, band
keratopathy
Anterior/ posterior chamber: inflammatory cells; flare;
pigment
Iris: synechiae, atrophy, nodules, heterochromia,
*pupillary miosis
Angle: PAS, nodules, vascularization
IOP: hypotony or glaucoma

SIGNS OF UVEITIS
Vitreous: inflammatory cells, traction bands
Pars plana: snow banking
Retina: inflammatory cells, cuffing of blood vessels,
edema, CME, hypertrophy/ clumping/loss of RPE;
epiretinal vessels
choroid: inflammatory infiltrates; atrophy;
neovascularization
Optic Nerve: Edema; neovascularization

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