Retinal Detachment: Pathogenesis

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Retinal Detachment

Detachment of the retina signifies an inward year age group. There is a smaller peak in the
separation of the sensory part of the retina from mid-20s to 30s owing to traumatic detachments
the retinal pigment epithelium (RPE). There is in young males.
an accumulation of fluid in the space between Certain groups of people are especially liable
the neural retina and the RPE known as subreti- to develop detachment of the retina: severely
nal fluid (Figure 13.1). The retina bulges inwards shortsighted patients have been shown to have
like the collapsed bladder of a football. Once an incidence as high as 3.5% and about
detached, the retina can no longer function and, 1% of aphakic patients (see Chapter 11) have
in humans, it tends to remain detached, unless detachments.
treatment is available. In just under one-quarter of cases,if there is no
Although the condition is relatively rare in intervention, the other eye becomes affected at
the general population, it is important for a later date. This means that the sound eye must
several reasons. First, it is a blinding condition be examined with great care in every instance.
that can be treated effectively and often dra-
matically by surgery. Second, retinal detach-
ment can on occasions be the first sign of
malignant disease in the eye. Finally, nowadays Pathogenesis
the condition can often be prevented by pro-
phylaxis in predisposed eyes. There is an embryological explanation for
retinal detachment in that the separating layers
open up a potential space that existed during the
Incidence early development of the eye, as described pre-
viously (Chapter 2). The inner lining of the eye
Retinal detachment is rare in the general pop- develops as two layers. In its earliest stages of
ulation but an eye unit serving a population of development, the eye is seen as an outgrowth
500,000 might expect to be looking after three of the forebrain, the optic vesicle, the cavity of
or four cases a week. It can be seen, therefore, which is continuous with that of the forebrain.
that a doctor in general practice might see a case The vesicle becomes invaginated to form the
once in every two or three years, especially if we optic cup, and the two-layered cup becomes the
consider that some retinal detachment patients two-layered lining of the adult eye. Anteriorly in
go directly to eye casualty departments without the eye, the two layers line the inner surface of
seeking nonspecialist advice. Although children the iris and ciliary body. Posterior to the ciliary
are sometimes affected, the incidence increases body, the outer of the two layers remains as a
with age and reaches a maximum in the 50–60- single layer of pigmented cells, known as the

103
104 Common Eye Diseases and their Management

tractional retinal detachment, and exudative


retinal detachment.

Rhegmatogenous
Retinal Detachment
This is the most common form of retinal
detachment, caused by the recruitment of fluid
from the vitreous cavity to the subretinal space
via a full-thickness discontinuity (a retinal
“break”) in the sensory retina.
Retinal “breaks” can be further subdivided
into “tears”, which are secondary to dynamic
Figure 13.1. Histology of retinal detachment showing the vitreoretinal traction, and “holes”, which are the
location of subretinal fluid.This eye has an underlying choroidal
result of focal retinal degeneration (see below).
melanoma.
Tractional Retinal Detachment
pigment epithelium. The inner of the two layers This form of retinal detachment develops as a
becomes many cells thick and develops into the result of tractional forces within the vitreous gel
sensory retina. In the adult, the sensory retina is pulling on the retina, causing the retina to
closely linked, both physically and metabol- be tented up from the RPE. The pure form of
ically, with the RPE and, in particular, the tractional retinal detachment is different from
production of visual pigment relies on this rhegmatogenous retinal detachment in that
juxtaposition. When the retina becomes there are no retinal breaks. Examples of trac-
detached and the sensory retina is separated tional retinal detachment include proliferative
from the RPE, the retina can no longer function diabetic retinopathy and vitreomacular traction
and the sight is lost in the detached area. Both syndrome.
RPE and sensory retina are included in the term
“retina” and in this sense “retinal detachment” Exudative Retinal Detachment
is a misnomer.
The retina receives its nourishment from two This group of retinal detachments also occurs in
sources: the inner half deriving its blood supply the absence of retinal breaks. The fluid gains
from the central retinal artery, and the outer half access to the subretinal space through an
from the choroid. The important foveal region abnormal choroidal circulation (e.g., from a
is supplied mainly by the choroid. When the choroidal malignant melanoma) or, rarely, sec-
retina is detached, the central retinal artery ondary to inflammation of the RPE or deeper
remains intact and continues to supply it layers of the eye (e.g., scleritis).
because it is also detached with it. The outer
half of the retina is deprived of nourishment,
being separated from the RPE and choroid.
Rhegmatogenous
Eventually degenerative changes appear, the Retinal Detachment
fovea being affected at an early stage. It is inter-
esting that after surgical replacement the retina The Presence of Breaks in
regains much of its function during the first few Retinal Detachment
days but further recovery can occur over as long
a period as one or even two years. It was noticed as long ago as 1853, only a short
time after the invention of the ophthalmoscope,
that many detached retinae have minute full-
Classification thickness discontinuities (breaks) in them, but
it was not until the 1920s that the full signific-
Detachment of the retina can be classified as ance of these breaks as the basic cause of the
follows: rhegmatogenous retinal detachment, detachment became realised. The breaks can
Retinal Detachment 105

be single or multiple and are more commonly original position again. The vitreous is usually
situated in the anterior or more peripheral part perfectly transparent but most people become
of the retina. In order to understand how these aware of small particles of cellular debris, which
breaks occur, it is necessary to understand can be observed against a clear background
something of retinal degeneration and vitreous such as a blue sky or an X-ray screen (vitreous
changes. floaters). These particles can be seen to move
slowly with eye movement and appear to have
Retinal Degeneration momentum, just as one would expect if one con-
siders the way the vitreous moves.
When examining the peripheral retina of other-
wise normal subjects, it is surprising to find that Posterior Vitreous Detachment
from time to time there are quite striking degen-
erative changes. Perhaps this is not so surprising Vitreous floaters are commonplace and tend to
when one considers that the retinal arteries are increase in number as the years pass. But the vit-
end arteries and these changes occur in the reous undergoes a more dramatic change with
peripheral parts of the retina supplied by the age. Often in the late 50s, it becomes more fluid
distal part of the circulation. Peripheral retinal and collapses from above, separating from its
degenerations are more commonly seen in normal position against the retina and event-
myopic eyes, especially in association with ually lying as a contracted mobile gel in the
Marfan’s and Ehlers–Danlos syndromes and inferior and anterior part of the cavity of the
Stickler’s disease (see reading list). globe. The rest of the globe is occupied by clear
Different types of degeneration have been fluid. This then is the process known as poste-
described and named and certain types are rior vitreous detachment (PVD).
recognised as being the precursors to formation When this happens, the patient might com-
of retinal breaks. The most important degener- plain of something floating in front of the vision
ations are lattice degeneration and retinal tufts. and also the appearance of flashing lights. This
Lattice degenerations consist of localised areas is because the mobile shrunken vitreous some-
of thinning in the peripheral retina. Progressive times causes slight traction on the retina. As a
thinning of the retina within areas of lattice rule, the same symptoms are then experienced
degeneration can eventually lead to formation subsequently in the other eye. On the other
of retinal “holes”. hand, it is also common to find a detached vit-
In addition, both lattice degenerations and reous in an elderly person’s eye in the absence
retinal tufts also represent areas with abnor- of any symptoms.
mally strong adhesions between the vitreous
and the retina. The presence of exaggerated Retinal Breaks Formation
vitreoretinal adhesions can result in the for-
mation of retinal “tears” within areas of lattice In the majority of eyes the vitreous separates
degeneration and retinal tufts during posterior “cleanly” from the retina during PVD. Such
vitreous detachment (see below). “uncomplicated” PVD is common and is usually
of no pathological significance. Unfortunately,
on rare occasions, the collapsing vitreous causes
The Vitreous a retinal “tear” to form at a point of abnormally
The normal vitreous is a clear gel, which occu- strong adhesion between vitreous and retina, for
pies most of the inside of the eye. Its consistency example within an area of lattice degeneration or
is similar to that of raw white of egg and, being retinal tufts. There might even be an associated
a gel, it takes up water and salts. It is made up of vitreous haemorrhage, when the PVD causes the
a meshwork of collagen fibres whose interspaces avulsion of a peripheral retinal blood vessel.
are filled with molecules of hyaluronic acid. The
vitreous is adherent to the retina at the ora Mechanism of Rhegmatogenous
serrata (junction of ciliary body and retina) and Retinal Detachment
around the optic disc and macula. If we move
our eyes, the vitreous moves, and, being Once a retinal tear forms as a result of abnor-
restrained by its attachment, swings back to its mal vitreous traction following PVD, the fluid
106 Common Eye Diseases and their Management

from within the vitreous cavity can gain access can save sight and they will, therefore, be con-
to the subretinal space through the retinal tear. sidered in more detail.
The progressive accumulation of fluid in the
subretinal space eventually causes the retina to Flashes (“Photopsiae”)
separate from the underlying RPE, similar to
wallpaper being stripped off a wall. This inward When questioned, the patient usually says that
separation of the retina from the RPE through these are probably present all the time but are
the recruitment of fluid via a retinal break is only noticeable in the dark. They seem to be
the basis for “rhegmatogenous” retinal detach- especially apparent before going to sleep at
ment, which is the most common form of night. The flashes are usually seen in the periph-
retinal detachment. eral part of the visual field. They must be dis-
tinguished from the flashes seen in migraine,
which are quite different and are usually fol-
Rhegmatogenous Retinal lowed by headache. The migrainous subject
Detachment Associated tends to see zig-zag lines, which spread out from
the centre of the field and last for about 10 min.
with Trauma Elderly patients with a defective vertebrobasilar
Most rhegmatogenous retinal detachments circulation may describe another type of pho-
occur as a result of spontaneous PVD-induced topsia in which the flashing lights tend to occur
retinal breaks. However, retinal tears can also only with neck movements or after bending.
occur as a result of trauma. A perforating injury
of the eye can produce a tear at any point in the Floaters
retina, but contusion injuries commonly
produce tears in the extreme retinal periphery It has already been explained that black spots
and in the lower temporal quadrant or the super- floating in front of the vision are commonplace
ior nasal quadrant. This is because the lower but often called to our attention by anxious
temporal quadrant of the globe is most exposed patients. When the spots are large and appear
to injury from a flying missile, such as a squash suddenly, they can be of pathological
ball. The threatened eye makes an upward significance. For some reason, patients often
movement as the lids attempt to close. Tears of refer to them as tadpoles or frogspawn, or even
this kind often take the form of a dialysis, the a spider’s web. It is the combination of these
retina being torn away in an arc from the ora symptoms with flashing lights that makes
serrata. Warning symptoms in these patients are it important.
usually masked by the symptoms of the original Flashes and floaters appear because the vit-
injury and they tend to present some months, or reous has tugged on the retina, producing the
occasionally years, after the original injury with sensation of light, and often when the tear
the symptoms of a retinal detachment. This is appears there is a slight bleeding into the vit-
unfortunate because the tear can be treated if it reous, causing the black spots. When clear-cut
is located before the detachment occurs. symptoms of this kind appear, they must not be
overlooked. The eyes must be examined fully
until the tear in the retina is found. Sometimes,
a small tear in the retina is accompanied by a
Signs and Symptoms large vitreous haemorrhage and thus sudden
of Retinal Tear and loss of vision.

Retinal Detachment Shadow


Let us now consider a typical patient, possibly a Once a retinal tear has appeared, the patient
myope in the mid-50s, either male or female, might seek medical attention, and effective
who suddenly experiences the symptoms of treatment of the tear can ensue. Unfortunately,
“flashes and floaters”, sometimes spontaneously some patients do not seek attention, or, if they
or sometimes after making a sudden head move- do, the symptoms might be disregarded. Indeed,
ment. Proper interpretation of such symptoms in time the symptoms might become less, but
Retinal Detachment 107

after a variable period between days and years,


a black shadow is seen encroaching from the
Exudative Retinal
peripheral field. This can appear to wobble. If Detachment
the detachment is above, the shadow encroaches
from below and it might seem to improve spon- In such detachments, there are no photopsiae
taneously with bedrest, being at first better in but floaters can occur from associated vitritis or
the morning. Loss of central vision or visual vitreous haemorrhage. A visual field defect is
blurring occurs when the fovea is involved by usual. Exudative detachments are usually convex
the detachment, or the visual axis is obstructed shaped and associated with shifting fluid.
by a bullous detachment. Inspection of the A malignant melanoma of the choroid might
fundus at this stage shows that fluid seeps present as a retinal detachment. Often the
through the retinal break, raising up the sur- melanoma is evident as a black lump with an
rounding retina like a blister in the paintwork adjacent area of detached retina. If the retina is
of a car. A shallow detachment of the retina can extensively detached over the tumour, the diag-
be difficult to detect but the affected area tends nosis can become difficult. It is important to
to look slightly grey and, most importantly, the avoid performing retinal surgery on such a case
choroidal pattern can no longer be seen. The because of the risk of disseminating the tumour.
analogy is with a piece of wet tissue stuck Suspicion should be raised by a balloon detach-
against grained wood. If the tissue paper is ment without any visible tears, and the diag-
raised slightly away from the wood, the grain is nosis can be confirmed by transilluminating the
no longer visible. As the detachment increases, eye to reveal the tumour.
the affected area looks dark grey and corrugated Retinal detachments secondary to inflam-
and the retinal vessels look darker than in flat matory exudates are not common. One example
retina. The tear in the retina shines out red as is Harada’s disease, which is the constellation of
one views the RPE and choroid through it. exudative uveitis with retinal detachment,
Once a black shadow of this kind appears in patchy depigmentation of the skin, meningitis
front of the vision, the patient usually becomes and deafness. Its cause is unknown. Exudative
alarmed and seeks immediate medical atten- detachments do not require surgery but treat-
tion. Urgent admission to hospital and retina ment of the underlying cause.
surgery are needed.

Management of
Tractional Retinal Rhegmatogenous Retinal
Detachment Detachment
Prophylaxis
In tractional retinal detachment, the retina can
be pulled away by the contraction of fibrous Retinal tears without significant subretinal fluid
bands in the vitreous. Photopsiae and floaters can be sealed by means of light coagulation. A
are usually absent but a slowly progressive powerful light beam from a laser is directed at
visual field defect is noticeable. The detached the surrounds of the tear (Figure 13.2). This pro-
retina is usually concave and immobile. duces blanching of the retina around the edges
Advanced proliferative diabetic retinopathy of the hole and, after some days, migration and
can be complicated by tractional retinal detach- proliferation of pigment cells occurs from the
ment of the retina when a contracting band tents RPE into the neuroretina and the blanched
up the retina by direct traction. Not infrequently area becomes pigmented. A bond is formed
such a diabetic patient experiences further across the potential space and a retinal detach-
sudden loss of vision in the eye, when the trac- ment is prevented. This procedure can be
tion exerted by the contracting vitreous pulls a carried out, with the aid of a contact lens, in a
hole in the area of tractional retinal detachment, few minutes.
resulting in a combined rhegmatogenous and A wider and more diffuse area of chorio-
tractional retinal detachment. retinal bonding can be achieved by cryopexy,
108 Common Eye Diseases and their Management

cautery to the site of the tear combined with the


release of subretinal fluid was effective, it also
became evident that not all cases responded
to this kind of treatment. It was almost as if
the retina was too small for the eye in some
cases, an idea that led to the design of volume-
reducing operations, which effectively made the
volume of the globe smaller. This, in turn, led to
the concept of mounting the tear on an inward
protrusion of the sclera to prevent subsequent
redetachment.
Modern retinal reattachment surgery is
carried out using either the cryobuckle or
Figure 13.2. Laser photocoagulation of retinal tear (with vitrectomy technique.
acknowledgement to Mr R. Gregson).

which entails freezing from the outside. Cry-


opexy is occasionally necessary if the retinal
hole is peripheral, or when there is limited
blanching of the retina from laser photocoagu-
lation because of the presence of vitreous haem-
orrhage. A cold probe is placed on the sclera
over the site of the tear and an ice ball is allowed
to form over the tear. A similar type of reaction
(as occurs after photocoagulation) develops
following this treatment, but it tends to be
uncomfortable for the patient and local or
general anaesthesia is required.

Retinal Surgery
In the early part of the twentieth century, it was a
generally accepted that there was no known
effective treatment for retinal detachment. It
was realised that a period of bedrest resulted in
flattening of the retina in many instances. This
entailed a prolonged period of complete
immobilisation, with the patient lying flat with
both eyes padded. This treatment can restore
the sight but only temporarily because the
retina redetaches when the patient is mobilised.
It was also dangerous for the patient in view of
the risk of venous thrombosis and pulmonary
embolism. In the 1920s, it began to be realised
that effective treatment of retinal detachment
depends on sealing the small holes in the retina
(Figure 13.3). It was already known by then that
the fluid under the retina could be drained off
externally simply by puncturing the globe, but b
up till then no serious attempt had been made
to associate this with some form of cautery to Figure 13.3. Retinal detachment a before and b after
the site of the tear. Once it became apparent that treatment. (After Gonin).
Retinal Detachment 109

Vitrectomy
The detached retina can also be reattached from
within the vitreous cavity. This involves the use
of fine-calibre instruments inserted through the
pars plana into the vitreous cavity. A light probe
is used to illuminate the operative field, while a
“vitrectomy cutter” is used to remove the vit-
reous, hence relieving the abnormal vitreous
adhesions that produced the retinal tear in the
first instance (Figure 13.5). The detached retina
is “pushed back” into place from within and
temporarily supported by an internal tamp-
onade agent (air, gas or silicone oil) while the
retina heals. The retinal breaks are identified
and treated by either laser photocoagulation or
a cryopexy at the same time. Vitrectomy can also
be combined with a silicone strap encirclement
if further support of the peripheral retina
is needed.
Historically, vitrectomy is reserved for the
more difficult and complex cases of rheg-
matogenous retinal detachment, where multiple
tears and posteriorly located tears are present,
or as a “salvage” operation following failed cryo-
buckle. With advances in instruments, vitrec-
tomy is increasingly being used as the primary
operation for the repair of most acute PVD-
related rhegmatogenous retinal detachments,
regardless of the complexity of the detachment.

Prognosis
b The retina can now be successfully reattached
by one operation in about 85% of cases. Of the
Figure 13.4. a Retinal detachment surgery: retinal tear sur- successful cases, those in which the macular
rounded by cryopexy and covered by indent. b Retinal detach- region was affected by the retinal detachment
ment surgery: indent and encirclement band (with
acknowledgement to Professor D. Archer).

Cryobuckle
This involves the sewing of small inert pieces of
material, usually silicone rubber, onto the
outside of the sclera in such a way as to make a
suitable indent at the site of the tear (Figure
13.4). This is combined with cryopexy to the
break. It is often necessary to drain off the sub-
retinal fluid and inject air or gas into the vit-
reous. In more difficult cases, the eye can be
encircled with a silicone strap to provide all-
round support to a retina with extensive
degenerative changes. Figure 13.5. Vitrectomy.
110 Common Eye Diseases and their Management

do not achieve a full restoration of their central “scarring” following initial retinal reattachment
vision, although usually the peripheral field surgery, with the formation of fibrous tractional
recovers. The degree of recovery of central membrane within the eye, resulting in recurrent
vision in such macula-detached cases depends detachment of the retina.
largely on the duration of the macula detach- When retinal surgery has failed, further
ment before surgery. Even when the retina has surgery might be required and for a few patients
been detached for two years, it is still possible to a series of operations is necessary. If it is
restore useful navigational vision. thought that more than one operation is going
The main cause of failure of modern retinal to be needed, it is helpful to the patient if he
reattachment surgery is proliferative vitreo- is warned about this before the treatment
retinopathy. This is characterised by excessive is started.

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