Part V Limbus

14 CHAPTER

Vascularized limbal keratitis

The limbus is prone to pathological changes for a variety Grade 1

of reasons. A rich capillary plexus is embedded in the
limbus and these vessels may become engorged (see The patient is asymptomatic. The epithelium adjacent to the
Chapter 13) or may constitute the platform from which limbus appears disrupted as evidenced by superficial punc-
vessels may penetrate into the cornea (see Chapter 23). The tate staining. An apparent ‘heaping’ or accumulation of
limbus is the site of the corneal stem cells, which are the hyperplastic corneal and/or limbal epithelial tissue can be
primary source for the differentiation and proliferation of observed. This appears as an elevated, opaque or whitish
the corneal epithelium. A deficiency in limbal stem cells elevated mass at the 3 and 9 o’clock corneal locations, with
may lead to a plethora of corneal complications.1 A sharp a diffuse, ill-defined border. The mass typically appears to
transition in the topography of the anterior eye occurs at bridge from the conjunctiva, over the limbus, on to the
the limbus, whereby there is a pronounced flattening from cornea, although it can form almost exclusively on the
the corneal surface to the conjunctival surface, forming a cornea or the conjunctiva, but always immediately adjacent
physical ‘ridge’.2 These three limbal features – the capillary to the limbus. The tear film meniscus at the edge of the rigid
plexus, stem cells and the limbal ridge – render the limbus lens is absent or disrupted (Figure 14.1).
to be susceptible to metabolic, immunological, toxic and
physical insult, and all of these challenges may be induced
or exacerbated by contact lenses.
It has long been recognized that the integrity of the
limbus is more likely to be compromised by soft lenses
because they rest in physical proximity to that tissue.3 The
limbus is less likely to be insulted by rigid lenses, which are
invariably smaller than the corneal diameter and only inter-
mittently impinge upon the limbus. Vascularized limbal
keratitis (VLK) is an unusual complication of rigid contact
lens wear that, as the name suggests, is characterized in its
most severe manifestation as an inflammation of the limbus
in association with a process of vascularization.4
The condition is usually associated with rigid lens
extended wear, and has been demonstrated to occur with
both low oxygen permeable lenses4 and super-permeable
rigid lenses5 – in the latter case with an incidence of only
0.8% of patients per year. VLK has also been observed in
patients wearing rigid lenses on a daily wear basis. It is
always observed at the 3 and 9 o’clock corneal locations.
Figure 14.1 Grade 1 VLK viewed in cobalt blue light with fluorescein
instilled. (Courtesy of Robert Grohe.)
Signs and symptoms
According to Grohe and Lebow,4 VLK can be characterized
as a condition that develops in four grades, which represent Grade 2
stages of increasing severity; these grades are outlined
below. This condition typically develops over a period of The patient will typically complain of mild ocular discom-
up to 6 months, but the time course can be more protracted, fort and increased lens awareness, and may observe that
taking up to 24 months for the condition to become severe. his/her eyes are red. Corneal infiltrates may be present as
© 2012 Elsevier Ltd

well as mild overlying staining (grade 2) and mild conjunc-
tival redness (grade 2) (Figure 14.2).
Figure 14.2 Grade 2 VLK. (Courtesy of Robert Grohe.)
Grade 3
The patient will complain of mild to moderate discomfort,
and may report reduced lens wearing time. Moderate con-
junctival redness (grade 3), more extensive corneal epithe-
lial staining and mild limbal and conjunctival staining are
observed. There may also be a greater infiltrative response.
The conjunctiva and limbus may appear to be slightly oede-
matous. A vascular leash, emanating from the conjunctiva
and across the limbus, encroaches upon the hyperplastic
epithelial mass (Figure 14.3).
Figure 14.3 Grade 3 VLK viewed in red-free (green) light. (Courtesy of
Robert Grohe.)
Vascularized limbal keratitis
Grade 4
Patients will report considerable discomfort and photopho-
bia. Pain is experienced when the lens edge impinges upon
the affected region, making lens wear almost intolerable.
Significant conjunctival redness and staining are apparent,
often associated with an erosion of the elevated hyperplas-
tic epithelial mass. Superficial and deep neovascularization
may be present. The patient will be aware of the hyperplas-
tic epithelial mass and may refer to this as a ‘lump’ on the
eye (Figure 14.4).
Figure 14.4 Grade 4 VLK viewed in cobalt blue light with fluorescein
instilled. (Courtesy of Robert Grohe.)
Pathology
The precise pathological changes that are occurring at a
cellular level are unknown because studies to this effect
have not been reported in the literature. However, it can
be deduced from the clinical observations outlined above
that there is a syndrome of concurrent tissue pathologies,
including epithelial cell hyperplasia (Figure 14.5), vessel
engorgement and progression, tissue erosion, tissue oedema
and corneal infiltrates.
Aetiology
The aetiology of VLK is unknown, but Grohe and Lebow,4
hypothesize that this condition is caused by an interruption
to the normal tear film dynamics at the limbus induced by
rigid lenses of inappropriate design. Specifically, they
propose that design faults such as a low lens edge lift create
abnormal fluid dynamics at the edge of the lens. This could
in turn compromise the normally full tear meniscus at the
lens edge, resulting in a desiccation effect and consequent
interruption to surface wetting in the region of the limbus.
Constant ongoing physical irritation of the poorly lubri-
cated ocular surface by a combination of the eyelids and
141
Chapter 14 Part V: Limbus
Figure 14.5 Vascularized epithelial mass in VLK. (Courtesy of Robert Grohe.)
lens induces a low-grade inflammatory reaction which, if
left unchecked, progresses through the various grades of
VLK as described above. In a case report, Miller6 found that
VLK was caused by surface crazing on a rigid lens.
The theory as to the cause of VLK as outlined above, and
the clinical description of this condition, strongly suggest
that VLK represents a severe clinical sequel of 3 and 9
o’clock staining, albeit uncommon. Indeed, Grohe and
Lebow4 admit that five of the eight VLK patients they
described had previously been fitted because of unaccept-
able peripheral 3 and 9 o’clock corneal staining with rigid
lenses, and the patient described by Miller6 also had a pre-
vious history of 3 and 9 o’clock corneal staining. If this
is the case, considerable clinical significance ought to be
attached to the early detection and management of 3 and 9
o’clock staining in rigid contact lens wearers.
Treatment
Treatment of VLK can be outlined with respect to the four
grades of severity of the condition.4
Grade 1
Instruct the patient to change from extended wear to daily
lens wear. If already in daily wear, wearing time should be
reduced from all day wear to 6–8 hours per day. The lens
should be redesigned such that the peripheral curve con-
figuration is flatted to create greater edge lift and improved
fluid dynamics at the lens edge. Lubricating drops used
throughout the day may provide symptomatic relief and
assist in improving lens dynamics.
Grade 2
Lens wear should be discontinued for 5 days, and ocular
decongestants may be prescribed to alleviate lens redness.
The lens should be redesigned as per grade 1, as well
142
as possibly reducing the lens diameter and/or flattening
the lens base curve. Efforts should be made to eliminate
mechanical bearing at the peripheral cornea. Daily lens
wear can be recommenced initially, and if the eye remains
quiet, extended wear may be resumed.
Grade 3
Lens wear should be discontinued for 5 days, and tissue
scrapings should be taken and sent for analysis to dif­
ferentiate from possible infectious causes (see Differential
diagnosis). Topical corticosteroids may be required if the
infiltrative response is severe. The lens should be rede-
signed utilizing the principles outlined as per grade 2
above. Daily lens wear can be recommenced initially, and
extended wear should only be resumed if there has been
full recovery and there is a pressing need.
Grade 4
Lens wear should be discontinued for 3 weeks. Tissue
scrapings should be taken and the eye should be treated
with an antibiotic steroid combination. Topical corticoste-
roids may be required if the infiltrative response is severe.
The lens should be redesigned utilizing the principles out-
lined as per grade 2 above. Daily lens wear can be recom-
menced initially, but future extended wear is generally
contraindicated.
Miller6 was able to manage a case of VLK by prescribing
a new set of lenses of a different material to replace lenses
that had suffered surface crazing. The parameters of the
replaced lenses were not altered. Thus, changing lens mate-
rial is a possible alternative strategy to altering lens param-
eters in the management of VLK.
Prognosis
If a patient recommences lens wear prematurely after lens
wear has been suspended due to VLK, a ‘rebound’ phe-
nomenon may occur whereby the condition flares up once
again and rapidly progresses to the equivalent level of
severity at the time of cessation. This situation typically
occurs in cases when the patient recommences wearing the
original offending lens (i.e. the lens design has remained
unaltered). This phenomenon strongly suggests a lens
design aetiology.
The prognosis for recovery from VLK is generally very
good; even severe cases can recover within days4 or a few
weeks.6 As an illustration of this rapid time course of reso-
lution of this condition, consider the following case. A
patient presented 3 months after being fitted with 9.2 mm
diameter fluorosilicone acrylate rigid lenses for extended
wear; these lenses were worn continuously for up to 7
days at a time. She was assessed as having grade 3 VLK.
Objective findings included a raised limbal epithelial mass
with fluorescein staining and a prominent vascular leash.
The lens edge can be seen impinging against the raised
mass in Figure 14.6. With the aid of fluorescein, the tear
film meniscus at the lens edge can be seen to thin out and
virtually disappear in the proximity of the raised mass
(Figure 14.7).
 Vascularized limbal keratitis

Figure 14.8 Same eye as in Figure 14.6 but the patient has been refitted
Figure 14.6 Grade 3 VLK in a patient wearing a 9.2 mm diameter rigid lens. with an 8.0 mm diameter rigid lens, which is now not impinging upon the
(Courtesy of Robert Grohe.) raised epithelial mass. (Courtesy of Robert Grohe.)

Figure 14.9 Same eye as in Figure 14.6 but the wearing schedule was
restricted to a maximum of 3 days of extended wear at a time, followed by a
caessation of lens wear for 5 days. The raised mass has now almost
completely resolved. (Courtesy of Robert Grohe.)
Figure 14.7 Same eye as in Figure 14.6 after instillation of fluorescein and
viewed with a cobalt blue light. The tear film meniscus at the lens edge is
virtually absent at the point of contact between the raised epithelial mass
and the lens. (Courtesy of Robert Grohe.)

The lens diameter was subsequently reduced to 8.0 mm;

as can be seen in Figure 14.8, the lens no longer impinges
on the epithelial mass. Comparing Figure 14.8 with Figure
14.6, it can be seen that this strategy has resulted in a slight
reduction in the size of the epithelial mass, and the vascu-
larization has subsided. In a further attempt to resolve
the condition, the wearing schedule was restricted to a
maximum of 3 days of extended wear at a time; this resulted
in a further resolution, but not elimination, of the raised
mass and vascular leash. Lens wear was then ceased for 5
days, resulting in almost complete resolution of the raised
mass (Figure 14.9) and vascular leash (Figure 14.10). The Figure 14.10 Same eye and lens as in Figure 14.6 viewed with a red-free
patient was subsequently able to resume extended wear for (green) light, confirming the absence of vascular leashing. (Courtesy of
2 days at a time, and the VLK did not recur. Robert Grohe.)
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Chapter 14 Part V: Limbus

Differential diagnosis
The key conditions that need to be differentially diagnosed
from VLK are phlyctenulosis, peripheral microbial kerati-
tis, pterygium, pseudopterygium and pinguecula.
Phlyctenulosis is an inflammatory disorder involving the
conjunctiva, limbus and/or cornea.7 It is usually bilateral
and primarily affects young children and young adults.
Phlyctenules are pinkish white nodules that vary in size
from pinpoint to several millimetres. They may be solitary
or multiple. The limbus is generally affected first, but there
may be isolated involvement of the bulbar conjunctiva
or cornea. The condition is generally self-limiting, whereby
the phlyctenule progressively becomes greyish, ulcerates,
and heals completely within 10 to 15 days to leave residual
scarring and vascularization. Symptoms include a foreign
body sensation and the reporting of eye redness. The
patient will report severe photophobia if the cornea is
Figure 14.11 Pterygium. (Courtesy of Brian Tompkins.)
affected.
The pathogenesis of this condition is believed to be a local
immunological reaction of the ocular surface to bacteria- likely to occur in the case of pseudopterygium because of
elaborated antigens; the most commonly associated micro- its lack of adherence to the limbus.7
bial agent is Staphylococcus aureus. It can also potentially Pinguecula is a horizontal, triangular or oval, elevated
occur secondary to antigens from a variety of different milky-yellow area of bulbar conjunctival thickening in the
organisms, such as mycobacteria and intestinal worms.8 palpebral fissure adjacent to the limbus.7 It may encroach
The condition may be associated with blepharitis. Differen- upon the limbus, but when the cornea is involved, it
tial diagnosis between VLK and phlyctenulosis is effected becomes a pterygium. The aetiology of pinguecula is uncer-
by culturing the eyelid margins and conjunctiva; a positive tain, but may be the same as pterygium. Mimura et al.10
culture for staphylococcus, and the presence of residual suggest that contact lens wear – and especially rigid lens
pathology after the active phase of the condition has wear – is a risk factor for the development of pinguecula.
resolved should heighten suspicion of phlyctenulosis. Also, A pinguecula that lies close to the limbus can give the
phlyctenules can present at any location around the limbus, appearance of VLK, but these conditions can be differenti-
whereas VLK only presents at the 3 and 9 o’clock ated because the raised tissue mass of the pinguecula, by
positions. definition, does not fully encroach onto the limbus. The
Microbial keratitis is considered in detail in Chapters 24 difficulty in differentially diagnosing pinguecula from VLK
and 25. A microbial keratitis may be only mildly uncom- can be illustrated by comparing the pinguecula in Figure
fortable in the early stages, resulting in symptoms similar 14.12 with the VLK in Figure 14.13. Certain features,
to those experienced in VLK. A small ulcer may be present. such as the yellow colour and extensive vascularity of the
The term ‘ulcer’ implies an erosion of tissue, which is the tissue mass are almost identical. However, differential
opposite of the raised tissue mass seen in VLK; however, diagnosis can be made by noting that the base of the pin-
in the early stages of a microbial keratitis, the edges of the guecula is separated by about 1 mm from the limbus,
ulcer may be uneven and slightly raised at certain points, whereas the raised mass in the VLK – although located
making differential diagnosis difficult. As is the case with
phlyctenulosis, microbial keratitis can present at any loca-
tion in the cornea or around the limbus, whereas VLK only
presents at the 3 and 9 o’clock positions. Vascularization
usually only occurs when the condition is very advanced.
A pterygium is a triangular growth of fibrovascular tissue
into the cornea (Figure 14.11). It can be differentiated from
VLK because of its chronic time course (thought to be
caused by chronic exposure to ultraviolet light), classic tri-
angular shape and extensive corneal encroachment in its
late stage. Also, pterygium is largely asymptomatic. Exci-
sion and adjunctive treatment with mitomycin C or con-
junctival autograft is the most acceptable and most popular
mode of treating both primary and recurrent pterygium.
Outcomes seem to have been further improved with adju-
vant combination therapy.9
A pseudopterygium is a conjunctival adherence to the
cornea caused by limbal or corneal inflammation or trauma.
It may have an atypical shape or position, giving a similar
appearance to VLK. Differential diagnosis between pseu-
dopterygium and VLK can be effected by determining Figure 14.12 Pinguecula, with the raised epithelial mass separated from
whether a probe can be passed behind the lesion; this is the limbus by about 1 mm. (Courtesy of Brian Tompkins.)
144

Figure 14.13 VLK, with the raised epithelial mass impinging upon the
limbus. (Courtesy of Debbie Jones.)
primarily on the conjunctiva – clearly impinges upon the
limbus.
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