What Is Mooren’s Ulcer?

Overview

Mooren’s ulcer (MU) is an eye condition that causes damage to and degeneration of the cornea. The
cornea is the outside layer of your eye that covers the front of your eye.

MU is a type of keratitis. Keratitis is inflammation of the edges of the cornea. MU is different from
other types of corneal ulcers because it happens along the edge of the cornea where it meets the
sclera. The sclera is the white of your eye. Because of this, it’s known as a type of peripheral
ulcerative keratitis (PUK).

MU is extremely rare. There’s not much information about how common it is in the United States.
Cases of MU are recorded more frequently in China, India, and Africa.

What are the types of Mooren’s ulcer?

There are various classifications of Mooren’s ulcer. One common classification divides the disease
into two types based on laterality (one or both eyes) and age of onset:

Limited (benign) type. This type of MU doesn’t cause much pain or discomfort. It usually only
happens in one eye (unilaterally). Only 25 percent of cases of the benign type happen in both eyes
(bilaterally). It’s more common if you’re older.

Atypical (malignant) type. This type is more painful and can quickly cause your cornea to break down
if it’s not treated. It usually happens in both eyes. About 75 percent of cases of the malignant type
happen in both eyes.

More recent classifications group Mooren’s ulcer into three types based upon their clinical
presentation:

Unilateral Mooren's ulceration (UM). This is a painful and progressive corneal ulcer typically seen in
elderly patients.

Bilateral aggressive Mooren's ulceration (BAM). This type occurs in young patients. The ulcer
progresses circumferentially then centrally in the cornea.

Bilateral indolent Mooren's ulceration (BIM). This type usually occurs in middle-aged patients. It
presents with progressive peripheral corneal ulceration in both eyes.

What are the symptoms of this ulcer?

When MU first appears, the ulcer usually starts around the circumference, or edge, of the cornea. As
it progresses, MU can spread to the rest of the cornea and surrounding eye tissues.

The symptoms of MU can include:

 intense pain in the affected eye(s)

 thinning or tearing of the corneal tissue
 redness in the affected eye(s)
 unusual sensitivity to light (photophobia)
 inflammation of the uvea, the eye’s middle layer (iritis or uveitis)
If it’s not treated, complications can include:

 sclera and eyelid Inflammation and irritation (conjunctivitis)

 inflammation and pus in the front of your eye (hypopyon)
 blurriness in the eye lens (cataracts)
 punctures in the cornea (perforation)
 optic nerve damage (glaucoma)
 loss of vision or blindness

What causes this ulcer?

The exact cause of MU is unclear. Many MU symptoms are similar to those that affect the eye due to
immune system disorders, such as rheumatoid arthritis. This may mean that MU is caused by an
exaggerated immune response due to an autoimmune disease response to eye injury or infection.

Some research suggests that MU may be linked to hepatitis C. In several cases, people with MU were
also diagnosed with long-term, or chronic, hepatitis C infections.

How is this ulcer diagnosed?

MU is only diagnosed when other underlying inflammatory conditions, such as rheumatoid arthritis,
can be ruled out as a cause of corneal damage.

Other, more common corneal conditions, such as Terrien’s degeneration, also need to be ruled out
before your doctor can give you a confident diagnosis. Unlike other corneal conditions, MU doesn’t
happen along with inflammation of the whites of your eyes (scleritis), so your doctor will check for
this symptom, too.

Your doctor may use several tests, such as a slit-lamp test or fluorescein staining, to diagnose MU.
Your doctor will look for specific signs of the ulcer using a symptom list known as Watson’s criteria.

Signs your doctor will look for include:

 ulcer in the shape of a crescent around the edge of the cornea

 inflammatory cells found around the edge of the ulcer (corneal infiltrates)
 tissue damage underneath the edge of the ulcer (undermining)
 non-inflamed whites of the eyes (no scleritus)
 no diagnosis of other autoimmune or systemic conditions

To rule out the similar corneal condition called Terrien’s degeneration, your doctor will check if the
ulcer has spread to the middle of your cornea. If it hasn’t, they can rule out Terrien’s degeneration.

How is this ulcer treated?

Benign MU often doesn't need to be treated if it doesn’t cause pain or doesn’t have any risk of
complications.

If treatment is needed, both benign and malignant MU may be treated using one or more of the
following:

 topical treatments to keep tissue from degenerating

 antibiotics, such as moxifloxacin (Vigamox), to prevent infections
  interferon a2b for hepatitis C infections, sometimes combined with the antiviral medication
ribavirin (Rebetron)
 resection, or surgical removal of tissues surrounding the ulcer
 cryotherapy, which involves freezing and surgically removing ulcer tissue
 tissue adhesion, which involves placing materials near the ulcer to stop it from spreading

Outlook

Benign MU can cause discomfort but is harmless and doesn't need to be treated right away. You can
often go for years without treating MU and not experience any complications.

Malignant MU can be painful and quickly cause irreversible damage to your cornea, sometimes
resulting in vision loss. In many cases, this type of MU can be treated and you won’t permanently
lose any vision. Prompt treatment is important to avoid long-term complications.

Mooren's Ulcer

Figure 1. Mooren’s ulcer. A crescent-shaped ulceration with an overhanging central edge and
a vascularized base is seen in in the temporal periphery of the cornea. Ⓒ 2014 American
Academy of Ophthalmology.

Disease Entity
Mooren’s ulceration (MU) is characterized by painful peripheral corneal ulceration of
unknown etiology. The disease generally begins with intense limbal inflammation and
swelling in the episclera and conjunctiva [1]. Corneal changes begin 2-3 mm from the limbus,
first appearing as grey swellings that rapidly furrow, affecting the superficial one-third of the
cornea and then proceeding circumfrentially and centrally over 4-12 months [1],[2]. The bed of
the furrow becomes vascularized, with vessels advancing into the base of the undermined
edges of the ulcers [1] (figure 1). These ulcers are often described as crescent-shaped and can
leave behind either an opaque, edematous central cornea. Alternatively, they can completely
consume the corneal stroma and replacing it with a thin fibrovascular membrane [3].
Inflammation is not seen in in the sclera adjacent to the peripheral ulcers, nor does it affect
the underlying Descemet’s membrane [4]. Destruction of the cornea generally affects stromal
tissue only, leaving behind an intact endothelium and epithelium [5]. The central edges of the
ulcer can develop an overhanging edge with or without opacification, and neovascularization
of the cornea can occur, extending from the limbus into the ulcer bed [3]. Neovascularization
can occur up to the advancing edge of the ulcer but not beyond it [5].

Etiology
Although the etiology for Mooren’s ulcer (MU) is unknown, there is evidence suggesting an
autoimmune basis. Gottsch and colleagues have suggested that this disorder can result from a
host response to calgranulin C, a normally hidden antigen expressed by keratinocytes in the
corneal stroma [6]. This molecule has also been found in circulating polymorphonuclear
leukocytes [5]. Receptors for this antigen have also been found on the surface of certain
helminths, which has led to speculation regarding an association with helminthic infections.
However, MU has not been proven to be more prevalent in endemic areas of ascariasis [4].
It has been found that certain HLA alleles may be associated with MU. One study suggested
that 83% of patients (n=12) with MU were HLA-DR17(3) positive, and 83% were HLA-DQ2
positive, as compared to control populations where frequencies varied between 5% and
40% [5]. The same study found that only one out of 10 patients with another form of
destructive sclerokeratitis expressed the HLA-DR17(3) allele and three expressed HLA-DQ2.
HLA-DQ5 has also been found in increased frequencies in patients with MU [7].

Risk Factors
Risk factors for MU include corneal surgery, previous trauma, and infection. Although
corneal surgery is a well-described risk factor, one study of 242 eyes affected with MU in
South India described an interesting caveat. In this study, Srinivasan and colleages described
36 eyes that developed ulceration following extracapsular cataract extraction through a
superior limbal incision; however, only 31% of these eyes developed ulceration at or
contiguous to the site of the surgical wound. Nevertheless, this ratio of superior corneal
involvement of MU is still higher than expected when compared with 10% superior
involvement found in eyes with no history of ocular surgery.
An association has also been demonstrated between Mooren’s ulceration and hookworm
infection [8]. Certain helminths express receptors for calgranulin C, suggesting that there may
be a helminthic antigen that cross-reacts with calgranulin C [5]. Previous infection has also
been found to be a risk factor [9].
An association with pyoderma gangrenosum has also been described, implying a potential
common pathogenesis to the two processes since both pyoderma gnagrenosum and MU show
aseptic neutrophilic inflammation and both respond well to immunosuppression [10].

Epidemiology
Mooren’s ulceration is a rare disease. One study approximated the incidence to be 0.03% in
China [2]. The disease is more common in the southern hemisphere including southern and
Central Africa and India, indicating a genetic and/or geographic predisposition [5]. Although it
is generally agreed upon that MU is more common in men, figures differ from one
geographic population to the next in terms of age distribution.
Studies from South India and China have suggested that patients tend to be affected between
their sixth and eighth decades of life, with men being affected more than women by a ratio
between 1.6:1 to 5:1 [3],[8],[9],[11][12]. However, this epidemiological picture may differ in
African populations, as suggested by a study in Nigeria where the disease mostly affected
men in their 20s-30s [13]. Another study from West Africa supported this epidemiologic
finding, causing the authors to postulate that there were two distinct populations of patients
with MU [14]. The first population is older, more often develops unilateral disease, and
responds to treatment whereas the second population is younger, tends to have bilateral
disease which perforates more often and does not respond well to treatment. Further studies
consisting of patients from the Republic of Zaire, Togo, and Sierra Leone further supported
this hypothesis [3]. However, the categorization described by Wood and Kaufman has been
refuted by others that found bilateral disease to be more common in older patients [15].

General Pathology

Figure 2     Lymphocytic infiltration and neovascularization of the cornea near an area of MU.  (Reproduced from Chen et al. [2] with permission from the
British Journal of Ophthalmology.)

On histological examination (figure 2), samples of conjunctival and corneal specimens show
a lymphocytic infiltration along with neutrophils, eosinophils, plasma cells, and mast cells [4],
[16]
 . High levels of proteolytic enzymes have been found in affected conjunctival tissue [16].
When the limbal cornea is examined, it can show vascularization and infiltration plasma cells
and lymphocytes into the superficial stroma in addition to destruction of the collagen
matrix [4]. The midstroma may show disorganized collagen lamellae and hyperactive
fibroblasts while the deep stroma contains a macrophage infiltrate. The leading edge of the
ulcer can show neutrophilic infiltration with evidence of degranulation. Adjacent conjunctiva
may show a hyperplastic epithelium and subconjunctival lymphocytic and plasma cell
infiltration [4].

Pathophysiology
It has been postulated that Mooren’s ulceration may result from an autoimmune etiology.
Sensitization to calgranulin C, an antigen expressed by corneal stromal keratinocytes, may
occur after trauma or infection of the cornea, causing unveiling of this hidden corneal
antigen [5]. Unveiling of the hidden antigen may also occur naturally in predisposed people
with age. It has been hypothesized that antigen-presenting cells at the limbus can present the
unveiled self-antigens via certain HLA class II molecules to T-cells, priming them.
Alternatively, antigen-presenting cells may present a helminthic antigen that cross-reacts with
calgranulin C (since certain helminths express receptors that bind calgranulin C) resulting in
a similar reaction [5]. Later, upon injury of the cornea, humoral and cellular responses result in
MU [4],[5].
Martin and colleagues have suggested that infection, trauma, or a systemic disease can
expose corneal antigens and stimulate an immune response in which complement is activated
and neutrophils degranulate and release collagenases. Collagenases then destroy the corneal
stroma, further perpetuating the process by exposing more altered corneal antigens.
Eventually, the entire cornea becomes consumed [17]. Evidence for a humoral immune
response comes from a study that found circulating IgG against corneal and conjunctival
epithelium in patients with MU [18]. Studies have also found antibodies and complement
bound to conjunctival epithelium in addition to elevated serum IgA levels [4].

Diagnosis

Figure 3     (A) Partial peripheral Mooren’s ulcer with a descemetocele within.  Conjunctival and episcleral injection is present along with deep vessels in the
base of the ulcer.  A characteristic overhanging central ulcer margin is also seen.  (B)  Total peripheral Mooren’s ulcer with an edematous, opacified central
cornea.  (C) Complete Mooren’s ulcer where a fibrovascular membrane has replaced the corneal stroma.  (Figure reproduced from Srinivasan, Zegans, Zelefsky,
Kundu, Lietman, Whitcher, and Cunningham [3] with permission from the British Journal of Ophthalmology.)   
The diagnosis of MU requires the absence of any ocular infection or systemic
rheumatological diseases known to cause peripheral corneal ulceration [3]. Srinivasan and
colleages described three patterns of ulceration: partial peripheral, complete peripheral, and
total corneal ulceration (Figure 3). In complete peripheral ulceration, the disease process has
completely encompassed the corneal periphery, leaving behind a “central island of cornea”
that is often opacified (Figure 3b). In total corneal ulceration, the corneal stroma has been
completely replaced with a fibrovascular membrane (Figure 3c). Partial peripheral ulceration
can be sub-divided into nasal, temporal, superior, and inferior ulceration, of which, temporal
and nasal (the so-called intrapalpebral cornea) involvement is more common [3].

Figure 4 Unilateral MU. Early venous phase of angiography shows intense vascular leakage from deep limbal vessels. Non-perfusion of superficial capillary and
venous vessels is also seen. Non-perfusion of the superficial limbal vasculature in addition to disruption of the limbal arcade in the area ahead of the advancing
ulcer is seen. (Reproduced from Watson [1] with permission from the British Journal of Ophthalmology.)

Watson and colleagues classified the disease into three types based on clinical features,
anterior segment fluorescein angiographic findings, and treatment response [1]: The first type,
unilateral Mooren’s ulceration (figure 4), is excessively painful and occurs in elderly (>60
years old) patients. Affected eyes are seen to be red and congested but inflammation does not
extend beyond 3 mm from the limbus. Vascularization of the ulcer bed is seen along with
leakage at the tips of these new vessels. Ulceration extends around the globe and often leaves
a thick, opaque central cornea. The central corneal stroma eventually is removed completely
and a thin layer of scar tissue remains overlying an intact endothelium and covered by an
epithelium derived from conjunctiva. When the stroma disappears, the pain subsides. If scar
tissue retracts to the point where Descemet’s membrane is exposed, it is likely that the same
process will recur if corneal transplant in performed [1]. Anterior segment fluorescein
angiography shows venular occlusion of local episcleral and conjunctival blood vessels along
with disruption of the limbal arcade and vascular leakage from deep vessels at the limbus and
base of the ulcer. In addition, vaso-obliteration of superficial vascular networks is
characteristic of unilateral Mooren’s ulceration.
The second type, bilateral aggressive Mooren’s ulceration, occurs more in younger patients
(between ages 14 and 40) and presents with pain that is less severe than in unilateral
Mooren’s ulceration [1]. These patients may present with an ulcer in one eye and conjunctival
congestion in the other, which eventually progresses to develop grey patches within the
corneal stroma about 2 mm from the edge of the limbus. These grey patches then aggregate
and lead to a typical MU that progresses first

Figure 5     Bilateral Aggressive MU.  On angiography (b), inflammation and leakage from deep limbal vessels in addition to extension of vessels into the base of
the ulcer is seen.  The superficial vascular plexus is dilated but perfused normally.  (Reproduced from Watson [1] with permission from the British Journal of
Ophthalmology.)

circumferentially and then centrally. Fluorescein angiography (figure 5) shows vascular

leakage and new vessel formation that reaches the base of the ulcer. Angiography can also
show changes in the architecture of episcleral vessels and blockage in addition to break-up of
the limbal arcade. The superficial vascular plexus remains perfused although it may be
dilated.
The third type, bilateral indolent Mooren’s ulceration (figure 6), occurs in middle-aged (mid-
50s) patients who exhibit corneal guttering in both eyes with little inflammation. Although
both eyes tend to be involved at presentation, the disease is often more severe in one eye and
patients complain of discomfort rather than pain [1]. Most cases are gradually progressive but
some heal spontaneously. Others may reactivate after a long period of quiescence. Vascular
architecture in this type is normal with the exception that new vessels may extend into the
base of the ulcer [19].
Figure 6     Bilateral indolent MU with perforation at 12 o’clock (a).  Angiography at the ulcer’s advancing edge shows no changes in superficial vessel
architecture.  Mild vasodilation is noted in the region of the ulcer.  (Reproduced from Watson [1] with permission from the British Journal of Ophthalmology.)

History and Physical examination

Patients often experience severe pain, photophobia, and tearing along with a red inflamed
eye. About one third of cases present bilaterally [19]. Slit-lamp examination may reveal a
crescent-shaped peripheral corneal ulcer with an undermined central edge. A linear epithelial
defect may develop at the central margin, followed by progressive stromal melting. The ulcer
progresses both circumfrentially and centrally, resulting in a re-eipithelialised,
conjunctivalised thinned cornea [19]. Consequently, severe irregular astigmatism may result.
Conjunctival and episcleral inflammation may occur; however, the sclera is spared.
Decreased visual acuity can occur secondary to iritis, central corneal involvement, or
irregular astigmatism [4].

Differential diagnosis
To rule out other causes of peripheral ulcerative keratitis
(http://eyewiki.org/Peripheral_Ulcerative_Keratitis), laboratory investigations include CBC
with differential, platelet count, ESR, rheumatoid factor, complement fixation, ANA, ANCA,
circulating immune complexes, LFT’s, VDRL/FTA-ABS, BUN and creatinine, serum protein
electrophoresis, and urinalysis [4]. In addition, cultures should be performed to rule out
microbial keratitis.
Terrien’s marginal degeneration differs from Mooren’s ulceration in that it is a painless,
noninflammatory disease causing peripheral corneal thinning without ulceration. Terrien’s
degeneration also usually begins in the superior cornea (rather than the interpalpebral region)
and proceeds circumfrentially but not centrally. A clear zone with superficial vascularization
remains between the limbus and the infiltrate [4].
Pellucid marginal degeneration causes inferior corneal thinning and causes irregular against-
the-rule astigmatism but lacks the inflammation and pain seen in MU [4]. Senile furrow
degeneration causes thinning between the limbus and an arcus in elderly patients; however,
no inflammation or vascularization occurs [4].
Rheumatoid arthritis (RA) can cause peripheral corneal ulcers that start as grey, swollen areas
within 2 mm of the limbus [1]. Corneal thickness diminishes rapidly and may leave behind a
fragile descemetocoele. Angiography can show venous non-perfusion and disrupted limbal
arcades with neovascularization reaching the base of the gutter. Scleritis may be associated,
whereas MU spares the sclera. Other findings in RA include keratoconjunctivitis sicca,
episcleritis, and sclerosing keratitis [4].
Keratolysis is a disease of central corneal stromal disintegration that is often seen in patients
with long-standing rheaumatoid arthritis. First, the central cornea swells and the epithelium
begins to shed [1]. The disease process spreads until the corneal stroma is fully resorbed and a
fragile Descemet’s membrane is left. This disease is generally painless.
Other collagen vascular diseases in which peripheral ulcerative keratitis can occur include
systemic lupus erythematosus, systemic sclerosis, and relapsing polychondritis [4]. Peripheral
ulcerative keratitis has also been reported to occur in association with inflammatory bowel
disease, giant cell arteritis, staphylococcal marginal keratitis, HSV, and acanthamoeba
keratitis.
Ocular involvement in granulomatosis with polyangiitis (GPA; formerly known as
Wegener's) can include proptosis, scleritis, and uveitis in addition to peripheral ulcerative
keratitis. Polyarteritis nodosa can also cause choroidal or retinal vasculitis, scleritis, optic
atrophy, papilledema, exudative retinal detachment, CRAO, and conjunctival lesions [4].
Granulomatosis with polyangiitis and polyarteritis nodosa can display severe leakage and
neovascularization is seen from the tips of the limbal arcades. In GPA, granulomas
destroying limbal tissue can be seen in both the corneal and scleral side of the vascular
changes [1].
Limbal involvement generally occurs in MU, whereas other forms of peripheral ulcerative
keratitis usually spare the limbus [4]. The distribution of vascular involvement can differ when
comparing MU to other causes of peripheral corneal ulceration. Mooren’s ulceration
generally causes leakage from deep vascular networks that come from the long posterior
ciliary circulation, with the unilateral form also blocking perfusion to the superficial network.
In connective tissue disorders such as RA, angiography shows vaso-occlusion of capillary
and venous circulation in the episcleral and conjunctival circulation [1]. In vascular disorders
such as GPA and polyarteritis nodosa, marked vascular leakage and granulomatous
inflammation may be seen involving the episcleral and venous vessels.

Management
Wood and Kaufman noted that the two types of MU they described—one type being
unilateral and developing in older patients, and the other type being bilateral in younger
patient—respond differently to treatment. The first type tends to heal well after a conjunctival
flap procedure or lamellar keratoplasty whereas the second type is not responsive to
treatment.
Watson described three types of MU, described above under “Diagnosis”. While the
unilateral from has been said to be readily treatable, other studies have suggested
otherwise [1]. The suggested treatment for this form involves a trial of aggressive systemic and
local immunotherapy (described below), followed by surgical removal of the corneal stroma
up to Descemet’s membrane. Corneal transplant often fails due to recurrence of the disease or
allograft rejection. Bilateral aggressive Mooren’s ulceration can also be treated with
aggressive local and systemic immunosuppression. Bilateral indolent Mooren’s ulceration
often resolves with dietary changes and treatment of any systemic infection that may be
present. It can also be treated with local steroids and cyclosporine A [1].
Initial treatment of Mooren’s ulceration consists of topical corticosteroids. If corticosteroids
do not control the inflammation, limbal conjunctival excision can be performed [19]. Excision
of a 3-4 mm ring of limbal conjunctiva at least 2 clock hours adjacent to a MU has been
shown to be an effective treatment. Studies have shown that when less than half of the limbus
is involved, the cure rate after conjunctival excision and corneal ulcer resection is performed
is 51.3% [2]. When more than half of the limbus is involved, the cure rate after the procedure
decreases to 36.8%. In a 1975 study where limbal conjunctival excision was performed, 8 out
of 10 eyes healed and one developed recurring ulcers which then healed upon re-
treatment [20]. It was hypothesized that the limbal conjunctiva may contain antibodies that
react with antigens in the corneal stroma in addition to enzymes that destroy the corneal
stroma—therefore, excision may interrupt the disease process.
Surgical interventions can also include lamellar keratoplasty, keratoepithelioplasty,
delimiting keratotomy, and conjunctival flap and patch grafts using periosteum or fascia
lata [19]. Resection of the corneal lesion and adjacent conjunctiva combined with lamellar
keratoplasty can achieve a final healing rate of 89.6%. If topical 1% cyclosporine A is added,
a 95% final healing rate can be achieved [2].
Cryotherapy to the conjunctiva surrounding ulcers may temporarily halt the progression of
the disease, but healing may not occur until the conjunctiva is excised [21]. Other potential
local therapies include bandage contact lenses, tissue adhesive, subconjunctival heparin, and
artificial tears and collagenase inhibitors [19]. Lecinthinated superoxide dismutase has also
been found to be decrease epithelial defect size in patients with sterile corneal ulcers that do
not respond to conventional therapy in some studies [22].
Systemic immunosuppression using cyclophosphamide, azathioprine, or methotrexate can be
attempted if conjunctival resection yields no improvement [4],[19]. Interferon alpha-2b has been
used in patients who had hepatitis C and concomitant Mooren’s ulceration. In more
aggressive cases, oral corticosteroids, cyclosporine A, and methotrexate have also been used
with some success [23].
A systematic review performed by Alhassan et al. found that there have not been any
randomized controlled trials to compare the various treatment modalities for MU. The
authors recommend that studies measuring outcomes such as percentage of area healed after
treatment and speed of healing are necessary in order to ensure high quality care for these
patients.

Complications
In addition to iritis being occasionally associated with Mooren’s ulceration, complications
can include glaucoma, cataracts, and perforation [4]. Anterior uveitis occurs 6.8% of the time
and can involve fine dusty keratic precipitates and posterior synechiae locally [2]. 2.3%
develop complicated cataracts [2]. The rate of perforation has varied in different studies in
various geographical locations. Perforation (figure 7) occurs most often in the limbal cornea,
followed by the peripheral and then central cornea [2].
Figure 7     Perforated MU with iris herniation.  (Reproduced from Chen et al. [2] with permission from the British Journal of Ophthalmology.)

A study in South India described a perforation rate of 11% at the time of presentation [3]. This
rate was higher for patients under 60 years old (27% vs 10%) and was more likely to occur in
patients who had complete peripheral involvement of the corneal ulceration (73% of eyes
with complete peripheral ulceration perforated, vs 20% of those with total peripheral
ulceration and 6% of those with partial peripheral ulceration). Other studies of MU in South
India described perforation rates varying from 0% [11] to 26% [9]. A study of 550 patients in
China reported a perforation rate of 13% [2].
A Nigerian study suggested that, in addition to Mooren’s ulceration tending to affect more
patients in their third and fourth decades of life, the perforation rate in these patients was
more than one third [13]. This study was further supported by another study consisting of 9
patients from West Africa [14]. The authors of the latter study postulated that there were two
distinct populations of patients with MU in Africa. The first population is older, more often
develops unilateral disease, and responds to treatment whereas the second population is
younger, tends to have bilateral disease which perforates more often and does not respond
well to treatment. Further studies consisting of patients from the Republic of Zaire, Togo, and
Sierra Leone further supported this hypothesis [3].
Another complication that often occurs is post-operative recurrence. Chen and colleagues
described a recurrence rate of 25.6% of the time, which can occur between 2 weeks and 15
years postoperatively with 70.2% of recurrences occurring within 2-6 months. In recurrent
cases, the first recurrence often occurs in the same location as the original ulcer and the
interface of the donor graft and lamellar bed. Multiple recurrences, on the other hand, can
occur in originally unaffected areas of the cornea suggesting a difference between the
immunological mechanisms of initial and multiple recurrences [2].