Mooren’s ulcer

2.  First described in detail as a clinical entity by
Mooren in 1867.
 Mooren’s ulcer is a chronic, painful, peripheral
ulcerative keratitis.
 More common in males. (M:F = 1.6:1)

3.  Wood and Kaufman classified the disease
into 2 groups according to the age of onset.
Unilateral
older patients (fourth decade
and older)
 limited
More responsive to local
surgical and medical therapy.
Bilateral
younger individuals (third
decade)
Progressive
resistant to systemic
immunosuppression

4. Pathogenesis
 Precise pathophysiological mechanism
unknown
 autoimmune process: with both cell-mediated
and humoral components.
 A type III hypersensitivity mechanism has
been implicated in the etiopathogenesis
of Mooren’s ulcer.

5. partially purified corneal
antigen (Co-Ag).
Ab & complement
bound to conjunctival
epithelium
Neutrophils,
lymphocytes &
macropages

6. PATHOLOGY
 Mooren’s ulcer is characterized by a progressive,
crescentic, peripheral corneal ulceration that is
slightly central to the corneoscleral limbus.

7.  Characteristic extensive,
undermined, “overhanging”
edge
 The histopathology of
Mooren's ulcer suggests an
immune process.
 Involved limbal cornea
consisted of three zones.
 The superficial stroma
vascularized and infiltrated with
plasma cells and lymphocytes.
destruction of the collagen
matrix.
Epithelium and Bowman's layer
are absent.

8.  The midstroma
hyperactivity of fibroblasts
disorganization of the collagen lamellae.
 The deep stroma
heavy macrophage infiltrate
Descemet's membrane and the endothelium were
spared

9.  Leading edge of the ulcer
Heavy neutrophil infiltration
dissolution of the superficial stroma
degranulated neutrophils
 The adjacent conjunctiva
epithelial hyperplasia and a
subconjunctival lymphocytic and plasma cell infiltration.

10. Clinical features
 Patients with Mooren’s ulcer will
present with redness, increased
lacrimation and photophobia, but
pain is typically the outstanding
feature.
 The pain is excruciating and may
seem well out of proportion to the
corneal inflammation.
 Decreased visual acuity may be
secondary to associated iritis,
irregular astigmatism due to the
peripheral corneal thinning.

11.  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 limbus.
 The ulcerative process spreads
circumferentially and then centrally
to involve the entire cornea
eventually.

12.  The anterior 1/3 to 1/2 of the stroma is involved
characteristically with a steep overlying central and leading
edge.
 It is difficult to judge the depth of the involvement unless the
lesion is gently probed at the overlying edges.

13.  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
trauma.

14.  Healing and vascularization
occurs slowly with the disease
running a chronic course over 4-
18 months.
 Portions of the ulcer may be
quiescent while the remaining
may be active.
 Topography demonstrates
severe irregular astigmatism
and peripheral steepening.

15.  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
thinning.

16. DIFFERENTIAL DIAGNOSIS
 Collagen Vascular Diseases
Rheumatoid Arthritis
Wegener's Granulomatosis
Polyarteritis Nodosa
Systemic lupus erythematosus
 Corneal degenerations
Terrien's Degeneration
Pellucid Degeneration
 Other
Staphylococcal Marginal Keratitis
Ocular Rosacea

17. DIAGNOSIS
 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
systemic diseases.

18.  Investigation includes
Total blood count
differential count,
erythrocyte sedimentation rate,
rheumatoid factor,
complement fixation,
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
roentgenogram.

19. TREATMENT
 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
5. Rehabilitation
 The overall goals of therapy are to arrest the
destructive process and to promote healing and
reepithelialization of the corneal surface

20. 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.

21.  Oral therapy (60 to 100 mg daily of oral
prednisone)
 Topical tetracycline or medroxyprogesterone
may be used for anticollagenolytic properties
of each.
 A therapeutic soft contact lens or patching

22. 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
inflammatory cell.
 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.

23.  The overhanging lip of ulcerating cornea may also be
removed.
 Tissue adhesive and a therapeutic soft contact lens
may be beneficial.
 Cryotherapy of limbal conjunctiva may have a
similar effect.

24. 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
cyclophosphamide.

25.  Agents such as cyclophosphamide may be effective by
suppressing B lymphocytes, which produce
autoantibodies and promote immune complex
disease.
 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.

26.  Adverse effects of these, such as anaemia,
alopecia, nausea, nephrotoxicity and
hepatotoxicity,
 local or systemic side effects attributable to
topical cyclosporin A were not observed.

27. 4. Additional Surgical
Procedures
 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.

28.  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
glue.

29.  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
keratoplasty.

30.  In case of larger peripheral perforations, a
partial penetrating keratoplasty may be
performed.
 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.

31. 5. Rehabilitation
 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.

32.  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.

33.  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
recommended.

34.  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%
cyclosporine