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systemically, there is a decrease in the number of suppressor T cells relative to the number of helper T cells in Mooren&apos;s ulcer patients. From this it was proposed that unregulated helper T cells could induce production of autoantibodies, resulting in the deposition of immune complexes, complement activation, inflammatory cell infiltration, and proteolytic enzyme release. Elevated serum IgA levels and circulating immune complexes in patients with Mooren&apos;s ulcer have also been reported. In the process, complement activation leads to neutrophil chemotaxis and degranulation with release of collagenases, causing corneal melting and further alteration and exposure of altered corneal antigens, thus perpetuating the process. This cycle continues until the entire cornea is consumed. circulating antibodies to the corneal stroma and specific cell mediated immune reaction toward a partially purified corneal antigen (Co-Ag). circulating IgG antibodies to human corneal and conjunctival epithelium in patients with Mooren&apos;s ulcer. In addition, antibodies and complement have been demonstrated bound to the conjunctival epithelium in the affected areas.
Young and Watson studied the corneas of three patients who underwent grafting. They observed that the involved limbal cornea consisted of three zones.
Slit lamp photograph of Mooren&apos;s ulcer shows advanced corneal ulceration involving corneal center with vascularized corneal base, corneal thinning and adjacent conjuctival inflammation.
Infectious aetiologies should be excluded by appropriate cultures, because microbial keratitis can rapidly progress and are usually amenable to antibiotic therapy Mooren&apos;s ulcer is easily distinguished from the noninflammatory corneal degenerations, such as Terrien&apos;s or Pellucid marginal degeneration, in which the epithelium remains intact and pain is absent.
when topical therapy is ineffective after 7 to 10 days or in cases where topical steroids may be dangerous of the eye may be beneficial at this stage. because of precariously deep ulcer or infiltrate. any concomitant eye disease, such as acne rosacea meibomeitis, blepharitis, dry eye, or eyelid abnormalities should be addressed.
In Mondino&apos;s series,conjunctival resection healed 3 of 4 unilateral ulcers and 3 of 3 bilateral nonsimultaneous ulcers, but only 2 of 15 bilateral simultaneous ulcers. On the other hand Stilma studied 38 Mooren&apos;s corneal ulcers and found that conjunctival excision with thermocoagulation gave some relief at the site of the ulcers, but recurrences at other places occurred in at least 52% of cases. Brown reported that excision of the limbal conjunctiva adjacent to the progressing ulcer, resulted in healing of the ulcers in 8 of 10 eyes treated; Kinoshita and colleagues reported success in their series of 20 Mooren&apos;s patients treated with keratoepithelioplasty. In this procedure, donor corneal lenticles are sutured onto the scleral bed after conjunctival excision. It should be emphasized, however, that whether or not the presence of this donor material added a therapeutic effect above and beyond the resection of tissue in preparation for the graft is unclear. , may be risk of epithelial rejection, glaucoma secondary to the chronic steroid use necessitated by keratoepithelioplasty and development of neurotrophic keratitis.
Foster reported arrest of the inflammatory process and preservation of ocular anatomy and function in 8 of 9 patients with bilateral involvement treated with immunosuppressives for 6 to 24 months.The ninth patient, who developed perforation, did not seem to receive an adequate level of immunosuppression as measured by blood parameters Mondino has reported that immunosuppressive drugs halted progression in 4 of 13 patients with Mooren&apos;s ulcer who did not respond to topical corticosteroids or conjunctival resection. In this series immunosuppressive drugs were reserved for only the most severe, resistant cases. We disagree with the statement by Schanzlin that &quot;since the value of immunosuppressive therapy is less clear than other treatments, it is recommended only in the severest and most resistant cases&quot;. On the contrary, we believe that the evidence for the efficacy of systemic immunosuppressive chemotherapy for progressive bilateral Mooren&apos;s ulcer is quite strong, and further believe that such treatment should be employed sooner rather than later in the care of such patients, before the corneal destruction has become too extensive to need for surgery.
In most instances, systemic immunosuppressive therapy is best handled by close collaboration between an ophthalmologist and an oncologis. The potentially serious side effects of systemic immunosuppressive agents have led several groups to investigate the efficacy of topical applied cyclosporin A in the treatment Mooren&apos;s ulcer. In Holland&apos;s series of a variety of anterior segment inflammatory diseases; two patients had Mooren&apos;s ulcer. The first, with bilateral disease, showed reduced inflammation with minimal progression over 3 years, whereas the second, with unilateral disease, showed resolution of the ulcer on this medication. In Zhao and Jin&apos;s series,15 of 18 eyes with Mooren&apos;s ulcer improved, and 11 of these were cured with topical cyclosporin A therapy (0.5% solution).
After refining the technique in previous series, Nian and colleagues found 34 of 40 eyes to be successfully healed after lamellar keratectomy. Four of 6 eyes with recurrent ulcer healed with retransplantation while the other patients refused further intervention Some cases may progress to perforation despite management as just detailed.
It is also believed that patients with treated Mooren&apos;s ulcer should be immunosuppressed prior to cataract surgery or corneal grafting procedure. In absence of donor corneas Stilma has suggested the usage of a free lamellar scleral autograft to restore the corneal defect, followed by penetrating keratoplasty later. In his series of 38 eyes with corneal ulcer six eyes with a progressive iris prolapse and a flat anterior chamber were reconstructed with the above technique.
Once the active ulceration has ceased and the remaining cornea has been completely opacified, it is possible to perform penetrating keratoplasty on these patients, even in the face of a thinned and vascularized cornea. Some authors believe that the risk of recurrence is so great that patients are best served not by any intervention but by maintaining the current status - i.e., the vision provided by their own thinned, scarred cornea. In these instances, a 13 mm tectonic corneal graft is first sutured in place with interrupted 10-0 nylon or prolene sutures with the recipient bite extending into the sclera so that the suture will not pull through the thin host cornea and then a 7.5 or 8.0 mm therapeutic graft is placed. Glaucoma and cystoid macular oedema have been a problem in these cases, and although the graft remains clear, the visual result may only be 6/60. Because of the immune system&apos;s remarkable memory, surgical attempts at rehabilitation in Mooren&apos;s ulceration should be done only with concurrent immunosuppression, even when the active disease has been arrested, or is &apos;burned out,&apos; because attempts at penetrating keratoplasty often are associated with recurrence and graft failure.
First described in detail as a clinical entity by
Mooren in 1867.
Mooren’s ulcer is a chronic, painful, peripheral
More common in males. (M:F = 1.6:1)
Wood and Kaufman classified the disease
into 2 groups according to the age of onset.
older patients (fourth decade
More responsive to local
surgical and medical therapy.
younger individuals (third
resistant to systemic
Precise pathophysiological mechanism
autoimmune process: with both cell-mediated
and humoral components.
A type III hypersensitivity mechanism has
been implicated in the etiopathogenesis
of Mooren’s ulcer.
partially purified corneal
Ab & complement
bound to conjunctival
Mooren’s ulcer is characterized by a progressive,
crescentic, peripheral corneal ulceration that is
slightly central to the corneoscleral limbus.
The histopathology of
Mooren's ulcer suggests an
Involved limbal cornea
consisted of three zones.
The superficial stroma
vascularized and infiltrated with
plasma cells and lymphocytes.
destruction of the collagen
Epithelium and Bowman's layer
hyperactivity of fibroblasts
disorganization of the collagen lamellae.
The deep stroma
heavy macrophage infiltrate
Descemet's membrane and the endothelium were
Leading edge of the ulcer
Heavy neutrophil infiltration
dissolution of the superficial stroma
The adjacent conjunctiva
epithelial hyperplasia and a
subconjunctival lymphocytic and plasma cell infiltration.
Patients with Mooren’s ulcer will
present with redness, increased
lacrimation and photophobia, but
pain is typically the outstanding
The pain is excruciating and may
seem well out of proportion to the
Decreased visual acuity may be
secondary to associated iritis,
irregular astigmatism due to the
peripheral corneal thinning.
The disease may begin as several
patchy peripheral stromal infiltrates
that then coalesce, commonly in the
region of the palpebral fissure.
Generally there is involvement upto
The ulcerative process spreads
circumferentially and then centrally
to involve the entire cornea
The anterior 1/3 to 1/2 of the stroma is involved
characteristically with a steep overlying central and leading
It is difficult to judge the depth of the involvement unless the
lesion is gently probed at the overlying edges.
Adjacent conjunctiva may be
inflamed and edematous
iritis is sometimes associated
with Mooren’s ulcer.
Hypopyon is rare unless
secondary infection is present.
Glaucoma and cataract may
complicate the process.
Perforation can occur,
especially following minor
Healing and vascularization
occurs slowly with the disease
running a chronic course over 4-
Portions of the ulcer may be
quiescent while the remaining
may be active.
severe irregular astigmatism
and peripheral steepening.
The end stage is a typical scarred, vascularised
thinned cornea with the patient experiencing sudden
relief from the excruciating pain
Chronic Mooren’s ulcer ultimately results in a central
island of hazy stromal tissue with severe peripheral
It is a diagnosis of exclusion.
The differential diagnosis is that of peripheral
ulcerative keratitis and is extensive.
Infectious aetiologies should be excluded.
Mooren's ulcer is easily distinguished from the non-inflammatory
corneal degenerations, in which the
epithelium remains intact and pain is absent.
The presence of Mooren's-like ulcer requires an
extensive search for occult and potentially lethal
Total blood count
erythrocyte sedimentation rate,
antinuclear antibodies (ANA),
antineutrophil cytoplasmic antibody (ANCA),
circulating immune complexes,
liver function tests, blood urea nitrogen and creatinine,
Serum protein electrophoresis, urinalysis, and a chest
step-wise approach to the management of
Mooren's ulcer, which is outlined as follows:
1. Topical steroids
2. Conjunctival resection
3. Systemic immunosuppressives
4. Additional surgical procedure
The overall goals of therapy are to arrest the
destructive process and to promote healing and
reepithelialization of the corneal surface
1. Topical steroids
Initial therapy should include intensive topical steroids
Prednisolone 1%, hourly in association with topical
cycloplegics and prophylactic antibiotics.
If epithelial healing does not occur within 2 to 3 days,
the frequency of topical steroid application can be
increased to every half hour.
Once healing occurs, the frequency can be reduced, and
tapered slowly over a period of several months.
Such management, especially in unilateral, benign form
gives good results.
Oral therapy (60 to 100 mg daily of oral
Topical tetracycline or medroxyprogesterone
may be used for anticollagenolytic properties
A therapeutic soft contact lens or patching
2. Conjunctival resection
Conjunctiva adjacent to the ulcer contains
inflammatory cells that produce antibodies
against the cornea and cytokines which amplify
the inflammation and recruit additional
conjunctival excision to bare sclera extending at
least 2 clock hours to either side of the peripheral
ulcer, and approximately 4 mm posterior to the
corneoscleral limbus and parallel to the ulcer.
The overhanging lip of ulcerating cornea may also be
Tissue adhesive and a therapeutic soft contact lens
may be beneficial.
Cryotherapy of limbal conjunctiva may have a
3. Systemic immunosuppressives
The most commonly used agents are
cyclophosphamide (2 mg/kg/day),
methotrexate (7.5 to 15 mg once weekly) and
azathioprine (2 mg/kg body weight/day).
The degree of fall in white blood cell count is
considered as the most reliable indicator of
immunosuppression produced by
Agents such as cyclophosphamide may be effective by
suppressing B lymphocytes, which produce
autoantibodies and promote immune complex
oral cyclosporin A (10 mg/kg/day) has been
successfully used to treat a case of unresponsive
bilateral Mooren's ulcer
It work by suppression of the helper T cell population
and stimulation of the depressed population of
suppressor and cytotoxic T cells present in patients
with Mooren's ulcer.
Adverse effects of these, such as anaemia,
alopecia, nausea, nephrotoxicity and
local or systemic side effects attributable to
topical cyclosporin A were not observed.
4. Additional Surgical
Superficial lamellar keratectomy, has been shown to
arrest the inflammatory process and allow healing.
Application of isobutyl cyanoacrylate, a tissue adhesive,
forms a biological barrier between host cornea and the
reepithelializing conjunctiva and the immune
components it may carry.
Small perforations may be treated with
application of tissue adhesive and
placement of a soft contact lens to provide
comfort and to prevent dislodging of the
When a perforation is too large for tissue
adhesive to seal the leak, some type of
patch graft will be necessary.
This may range from a small tapered plug
of corneal tissue to a penetrating
In case of larger peripheral perforations, a
partial penetrating keratoplasty may be
It should be emphasized that the
prognosis of corneal graft in the setting of
acute inflammation in patients with
Mooren's ulcer is very poor.
Rehabilitative surgical therapy in two stages, namely initial
lamellar tectonic grafting followed by central penetrating
keratoplasty may be required in advanced cases.
LKP is the most widely practiced surgery at present.
It removes antigenic targets of the cornea,
prevents immunological reactions,
reconstructs the anatomical structure,
prevents perforation and improves vision.
The principle of lamellar keratoplasty
surgery in Mooren’s ulcer is to remove
necrotic ulcerative cornea and to reconstruct
the anatomical structure of the cornea.
For an ulcer smaller than half
circle of the limbus and the
central 7-8 mm of the cornea
uninvolved crescent shaped
lamellar graft can be used.
For an ulcer larger than 2/3 of
a circle of the limbus where
the central 7-8 mm of cornea
is intact, a doughnut shaped
lamellar graft is
Double lamellar grafts (a fresh thin inner graft with
corneal endothelial cells is used to repair the
perforation, on which another lamellar graft shaped in
accordance with the shape of the ulcer is placed) can be
used for perforations of the peripheral cornea.
Postoperative use of topical steroids and 1%