Chapter 1

Normal

Retinal histology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2
Fluorescein angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Indocyanine green angiography . . . . . . . . . . . . . . . . . . . . . . . . . . 5
Optical coherence tomography (OCT) imaging . . . . . . . . . . . . . . . . . . 6
1
Retinal Histology
The sensory retina extends to the ora serrata, where it is tissue of irregular density. It has a cuboidal and hexagonal
continuous with the non-pigmented ciliary epithelium of the shape with villous processes that envelop the photoreceptor
CHAPTER

pars plana. The ora serrata is 2.1 mm wide temporally and outer segments. It also contains melanin granules and is taller,
0.7–0.8 mm wide nasally. It is located more anteriorly on the more densely pigmented and columnar in shape in the central
nasal than on the temporal side. The nasal ora is about 6 mm macula.
posterior to the limbus, and the temporal ora is about 7 mm Bruch’s membrane refers to a sheet-like condensation of
posterior to the limbus. The average distance from the ora the innermost portion of the choroidal stroma that consists
serrata to the optic nerve is 32.5 mm temporally and 27 mm of two layers of collagen, one on either side of a layer of
NORMAL

nasally, and 31 mm superiorly and inferiorly. The retina itself elastic tissue. The basement membrane of the retinal pigment
is a thin transparent tissue, which is thickest near the optic epithelium (RPE) and the choriocapillaris endothelium are
nerve, where it measures 0.56 mm. It thins to 0.18 mm at the the boundaries of Bruch’s membrane, although this interpre-
equator and to 0.1 mm at the ora serrata. At the foveal area, tation is controversial. Some consider Bruch’s membrane as
it has thinned to about 0.2 mm. The nerve fiber layer increases a part of the choroidal stroma. The choroidal circulation is
at the edge of the disc and is the only retinal structure that supplied by the short ciliary or choroidal arteries that are
continues into the disc to become the optic nerve. The concentrated in the macula and peripapillary region. A luxuri-
sensory retina is composed of nine contiguous layers, linked ous anastomotic network of vessels form a sinusoidal
to each other by synaptic connections between axons and network, referred to as the choriocapillaris, enframed by the
dendrites in the inner and outer plexiform layers and to the outer part of Bruch’s membrane. In the macula, the chori-
ganglion cells. The neuronal cells are supported by fibers of ocapillaris is composed of a lobular pattern of highly concen-
Müller cells and the astrocytes from the inner portion of the trated and interconnecting capillaries supplied by a central
retina. The retinal pigment epithelial layer is a monocellular arteriole and drained by circumferential venules.

area centrallis

d c b a b c d fovea

ILM

foveola
1.5 0.5 0.75 .35 0.75 0.5 1.5 mm. terminal capillary
net
XLM
“fovea externa”
©1
Left: fundus photograph matched with a horizontal section of the macula, delineating the a) foveola, b) fovea, c) parafovea,
and d) perifovea. Right: schematic diagram showing the dimensions of the fovea, foveola, macula, and peripheral fundus.
ILM
GC NF

IPL
INL
MLM

OPL

ONL EXL
IS
IS/OS
OS
RPE
The histology of the fovea, macula, peripheral retina and optic nerve, which are
represented in these images. The retina begins with the internal limiting membrane (ILM).
Also shown are the nerve fiber (NF) layer and its ganglion cells (GC), the inner plexiform
layer (IPL), the inner nuclear layer (INL), middle limiting membrane (MLM), the outer These are histological specimens of the
plexiform layer (OPL), the outer nuclear layer (ONL), the external limiting membrane retina, the foveolar area, including the
(EXL), the internal segments of the photoreceptors (IS), the outer segments of the sclera, forming the outer segment of the
2
photoreceptors (OS), and the retinal pigment epithelium (RPE). globe (middle) and the optic nerve (top).
The Fundus 1

CHAPTER
This is a montage of a

RETINAL HISTOLOGY
relatively lightly pig-
mented fundus. The
choroidal circulation is
visible through a mildly
pigmented retinal
pigment epithelium.
This eye had four
vortex veins (arrows) in
the outer choroidal
circulation, accommo-
dating the very high
flow supplied posteri-
orly by 10–20 short
posterior ciliary
branches of the oph-
thalmic artery. A nasal
and temporal long
posterior ciliary artery
supplied the anterior
choroid and uvea.

Vitreous
The vitreous body extends from the posterior lens to the surface of the
retina. It is slightly less than 3.9 mm in volume, approximately 2/3 to
3/4 of the adult globe. It is spherical posteriorly and saucer-shaped
anteriorly due to a depression caused by the convexity of the posterior
lens surface. The vitreous cortex is made of three visible components:
(1) collagen-like fibers; (2) cells; and (3) mucopolysaccharides and other
proteins. The vitreous cortex is covered by the hyaloid membrane, a
thin enveloping structure. In the posterior pole, there is a precortical
vitreous pocket which may extend to the retinal vascular arcades.
The normal eye has a posterior precortical vitreous pocket (PPVP) that is
located immediately anterior to the posterior fundus and is surrounded by
the temporal vascular arcades (arrows). The posterior wall of the PPVP is
composed of a thin layer of vitreous cortex. The rest of its border is
contoured by formed vitreous. Occasionally, the PPVP expands to become
confluent with adjacent lacunae in the vitreous. This structure is inconsistently
detectable clinically when there is posterior vitreous detachment. Otherwise
it is consistently present in normal eyes.
Courtesy of Dr. Lennart Berglin, Dr. Louise Bergman and Dr. Henry F. Edelhauser

3
 Retina
1 The retina lines the inner surface of the eye with neuronal its outer aspect in the photoreceptor layer, containing rods
connections to the optic nerve and eventually to the central and cones. There are approximately 6 million cones, most
CHAPTER

nervous system. It is a layered structure with neurons and densely packed within the fovea and 125,000,000 rods
interconnected synapses with principal light-sensitive cells at spread predominantly throughout the peripheral retina.
NORMAL

This image illustrates the distribution of the

retinal vessels throughout the fundus in
a relatively normal fashion. The retinal
venules are darker than the reddish-orange
arterioles, forming in this case four vascular
arcades, two temporally and two nasally.

Macula
The macula refers to an area inclusive of the parafoveolar area (about
2.85 mm in diameter), but some retinal specialists equate the macula to
the foveolar area (about 1.8 mm in diameter). The fovea itself is a 1.5 mm
depression in the center of the macula. It is located about 4 mm temporal
and 0.8 mm inferior to the center of the horizontal plane of the optic disc.
The average thickness of the fovea is about 0.25 mm, roughly half that the
adjacent parafoveal area. The central 0.35 mm of the fovea is the foveola,
which is located in a retinal capillary-free zone which measures about
0.5 mm in diameter. A small protuberance in the center of the foveola is
called the umbo, where there is a great concentration of cell bodies of elon-
gated cones. A 0.5 mm wide annular zone surrounding the fovea is the area
where the ganglion cell, intranuclear layer, and outer plexiform layer of
Henle are the thickest. This is referred to as the parafoveal area. This area The morphological landmarks of the macula are
is surrounded by a 1.5 mm ring zone called the perifoveal area where the not very distinct clinically. However, a dark zone
ganglion cell layer is reduced from 5–7 layers to a single layer of nuclei, as surrounding the fovea is clearly evident due to the
intrinsic pigmentation of the retina (xanthophyll)
seen elsewhere in the peripheral retina.There are several modifications in
and, above all, the retinal pigment epithelium
the retinal architecture in the macular area, beginning with the absence of
(melanin).
retinal vessels in the perifoveal region. There are no rods in the foveola, and
the cones have become so modified that they resemble rods in form. The
external segments of the cones are long and approach the apical side of the
RPE cells. At the edge of the fovea, the ganglion cell layer and the inner
nuclear layer thicken, but both layers disappear within the fovea. In the
foveolar area, only photoreceptor cells and Müller cell processes are present.
Each cell is united with a single bipolar cell and possibly with a single ganglion
4 cell, plus yielding maximal transmission of the visual stimulus.
Fluorescein Angiography 1

CHAPTER
INDOCYANINE GREEN ANGIOGRAPHY
The best way to study the retinal circulation is with high-speed stereo fluorescein This image shows the fluorescein
angiography (FA) of high resolution. The perifoveal capillary-free zone and its marked angiographic filling of the choriocapillaris
variability are best seen with this form of imaging (left and middle). The upper right FA with high-speed angiography and serial
is the arteriole-venous face of the study with lamellar flow in the veins (arrows). subtraction technique. There is a lobular
Lower middle image courtesy of Ethan Friel filling pattern to the choriocapillaris which
is seldom appreciated, except in eyes that
have ischemic choroidopathies.

Indocyanine Green Angiography

The best way to image the choroidal circulation is with indo- of the choroidal circulation without high-speed serial
cyanine green (ICG) angiography. The longer wavelength subtraction techniques. The pigment epithelium–Bruch’s
penetrates the pigment epithelium to enhance the choroidal membrane–choriocapillaris have been collectively referred
circulation in the normal and abnormal eye. The capillary to as the tunica Ruyschiana, given commonalities in develop-
network in the choriocapillaris is immediately adjacent to ment, anatomy, and physiology.
Bruch’s membrane. It is not possible to image that portion

5
1
Optical Coherence
Tomography (OCT) Imaging
CHAPTER

For the past several years, optical coherence tomography (OCT) has become
ILM
the most important diagnostic adjunct in imaging the macula and paramacular
region. Histological-type imaging can now be used for high-resolution, three- NF
dimensional reconstruction in the fundus.
GC
ILM
LM OPL IPL
IPL I
INL O
ONL GCL
GC
CL NFL
NORMAL

IPL

Fov
ovvea
ea
Fovea INL

OPL MLM

RPE
PE
E PIL
PIL
L IS
S ELM
LM
M OS
OS IS/OS
S//O Choriocapillaris
Chorio
oca
capillaris
sCChoroid
Ch
Chor
Cho
h ro ONL

This is a high-resolution OCT showing the various levels of the retina, beginning from
the nerve fiber layer (NFL), ganglion cell layer (GCL), outer nuclear layer (ONL), IS ELM
inner nuclear layer (INL), inner plexiform layer (IPL), outer plexiform layer (OPL), IS/OS
internal limiting membrane (ILM), external limiting membrane (ELM), inner segments OS
(IS), junction between the inner segment and outer segment line (IS/OS), outer RPE
segments (OS) and retinal pigment epithelium (RPE). The choriocapillaris and choroid
can also be imaged.

These two OCT images show the optic nerve and thickness of the nerve fiber layer (arrow, left image) and a disturbance of the
vitreoretinal interface (arrow, right image).
Courtesy of Dr. Elias Reichel

The laminated appearance of the central macula and depression of the fovea, prominence of the neurofiber layer in the papillomacular
bundle and the integrity of the IS/OS photoreceptor junction (arrows) can be seen clearly in these images. Detachment of the
posterior hyaloid can also be documented in some eyes (right image).
Courtesy of Dr. Gabriel Coscas

Optic Nerve The optic nerve head is seen here with

retinal vessels emerging from physiological
cupping in each eye. The surface of the
optic nerve is perfused by branches from
the central retinal artery whereas the
posterior portion of the nerve receives
its circulation from the peripapillary ciliary
vessels and small pial vessels that are
derived from the ophthalmic artery. There
is a rich and axonally oriented anastomotic
bed within the nerve between these two
circulations. The autoregulation of the optic
nerve head capillary bed is comparable to
that of the retinal circulation. These eyes
have central physiologic cupping (arrows).
6
Courtesy of Ophthalmic Imaging Systems, Inc