A case of Mooren

1
A case of Moorens Ulcer

• Dr Johnson Tan
• Medical Officer
• Tan Tock Seng Hospital

2
Presenting history

• 50/Indian/Male h/o PTB and DM
• Left prosthetic eye (trauma 28 yrs ago)
• Aug 03 R eye redness x 1/52
• VA 6/6, peripheral infiltrate with central
  epithelial defect associated with localized
  injection and early pannus, CDR of 0.8
• Imp R marginal keratitis glaucoma suspect
• Treated with gutt Tobradex
• Epithelial defect healed with an area of
  localised pannus that appeared inflammed

3
June 2004

• R eye irritation, redness and blurring of vision
  x 4/7 (minimal pain)
• VA 6/18, area of pannus with 80 peripheral
  thinning and localised injection, no ED
• Imp MK vs Moorens ulcer.
• Treated with gutt FML/ciloxan ?responded

4
July 2004

• While steroids were being tailed down, pain and
  BOV worsened ? Steroids stepped up with minimal
  improvement
• Peripheral epithelial defect with 60-70 thinning
  a/w pseudopterygium 5-7 oclock
• Imp R Peripheral Ulcerative Keratitis ?Moorens

5
Management

• Gutt predforte hourly and gutt ciloxan qds
• Added oral steroids
• Right conjunctival resection 17/8/04
• Histology ulcer with mixed inflammatory base /no
  malignancy
• Oral steroids were tailed down with improved
  response by late Aug 04
• Subsequently defaulted FU

6
18/8/04
7
Progression and Management

• Feb 05 flare 5-8 oclock thinning
• conjunctival resection (2nd) 1/3/05
• Oral prednisolone restarted
• Defaulted FU
• Jun 05 recurrence 5-11 oclock
• Restarted on aggressive topical oral steroids

8
27/6/05
9
Progression and Management

• Aug 05 Minimal improvement. 3-11 oclock
  thinning
• Oral prednisolone stepped up to 60mg/day
• Oral cyclophosphamide started
• conjunctival resection (3rd) 26/8/05
• Pulsed IV cyclophosphamide commenced 2/52 after
  2/52 of oral cyclophosphamide

10
23/8/05
11
30/8/05
12

• 2/52 later, VA 6/24, progressive thinning gt95
  with descematocele high perforation risk
• Oct 05 lamellar keratoplasty complicated by
  wound leak ? resuturing of graft on POD4
• Recovering well VA 6/24, small persistent
  epithelial defect.
• On reducing dose of prednisolone and g tobramycin
  and pred forte.
• Currently completed 6 doses of IV
  cyclophosphamide (25 Jan 06)

13
3/10/05
14
Treatment summary

• Topical steroids with antibiotic cover
• Oral steroids
• Oral cyclophosphamide, pulse IV
• Conjunctival resection x3
• Lamellar keratoplasty

15
Peripheral ulcerative keratitis

• Definition crescent shaped destructive
  inflammation of the juxtalimbal corneal stroma
  associated with an epithelial defect, presence of
  stromal inflammatory cells, and stromal
  degradation
• Diagnosis
• Rule out CTD first

16
Causes of PUK

• Ocular
• Infective
• Non infective
• Moorens ulcer
• Pellucid Degeneration
• Terriens Degeneration
• Senile Furrow Degeneration
• Blepharitis (MK, rosacea)
• Keratoconjunctivitis sicca
• Neurotrophic keratopathy
• Exposure keratopathy
• Contact lens use
• chem injury/trauma

• Systemic
• Infective (TB, Syphillis, HZV, NG, HIV)
• Non infective
• CTD (RA, SLE, RP, WG, PAN)
• Sarcoidosis
• leukemia

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Moorens ulcer

• "chronic serpiginous ulcer of the cornea or ulcus
  roden "
• painful, relentless, chronic ulcerative
  keratitis, with no associated scleritis
• steep, overhanging
• central edge
• starts in periphery and
• may progress centrally or
• circumferentially to
• involve the entire cornea

18

• idiopathic, with no associated scleritis
• MF 1.61
• Complications irregular astigmatism, iritis,
  hypopyon, glaucoma, cataract, perforation
  (13-36), blindness

19
Classification
Type I Type II
Age Older (gt35) Young (lt35)
Symptoms Mild-moderate aggressive
Prognosis responds well to medical and surgical treatments Poor response to therapy
Bilaterality 25 75

• Wood T, Kaufman H. Mooren's ulcer. Am J
  Ophthalmol 197171417-422

20
Classification

1. Unilateral MU - painful progressive corneal
   ulceration in elderly patients, non-perfusion of
   the superficial vascular plexus of the anterior
   segment.
2. Bilateral aggressive MU - young, progresses
   circumferentially, then centrally in the cornea
   vascular leakage and new vessel formation,
   extending into the base of the ulcer.
3. Bilateral indolent MU - middle-aged patients,
   progressive peripheral corneal guttering in both
   eyes, with little inflammatory response no
   change from normal vascular architecture except
   an extension of new vessels into the ulcer

• Watson PG. Management of Moorens Ulceration.
  Eye, 11 349-356, 1997

21
Etiology

• Unknown etiology
• Pathophysiology
• Autoimmune (cell-mediated and humoral)
• still unknown if cell-mediated and/or humoral
  immune mechanisms are involved directly in the
  pathogenesis of MU or may be that they just
  accompany the corneal destruction that is caused
  by another mechanism

22
Diagnosis

• Typical ulcer morphology
• History and physical examination

23
Investigations

• FBC
• ESR
• RF
• complement fixation, circulating immune complexes
• ANA, ANCA,
• VDRL, TPHA
• CXR
• Scrapings for culture sensitivity

24
Treatment

• Stepwise approach
• Topical steroids
• Oral steroids and Immunosuppressive chemotherapy
• Conjunctival resection
• Lamellar keratoplasty/ penetrating keratoplasty

25
Steroids

• Intensive topical steroids
• Oral prednisolone 60-100mg od
• Indication - when topical therapy ineffective
  after 7-10days or topical steroids may be
  contraindicated because of precariously deep
  ulcer or infiltrate

26
Conjunctival resection

• Indication ulcer progresses despite the steroid
  regimen
• conjunctiva adjacent to the
• ulcer contains inflammatory
• cells that may produce
• antibodies against the cornea
• and cytokines, which amplify
• the inflammation and recruit
• additional inflammatory cells
• - Chow C, Foster CS. Moorens Ulcer. Int
  Ophthalmol Clin, 361-13, 1996
• - Brown SI. Mooren's ulcer. Treatment by
  conjunctival excision.
• Br J Ophthalmol. 1975 Nov59(11)675-82

27
Immunosuppressive chemotherapy

• bilateral or progressive MU that fails
  therapeutic steroids and conjunctival resection
• combination of oral prednisolone and an
  immunomodulatory agent is initiated at the same
  time.
• Oral prednisone controls the active inflammatory
  process until the immunomodulatory agent takes
  effect (abt 4-6/52).
• Prednisone is subsequently tapered and the
  patient maintained on the systemic
  immunomodulatory agent.

28
Immunosuppressants

• Cyclophosphamide
• Oral
• IV 1g monthly IV Cy
• Severe Moorens ulcer efficacy of monthly
  cyclophosphamide intravenous pulse treatment ev
  Med Interne. 2003 Feb24(2)118-22. French.
• Cyclosporin A
• 0.5 Topical CSA
• Zhao JC, Jin XY.Immunological analysis and
  treatment of Mooren's ulcer with cyclosporin A
  applied topically.cornea. 1993 Nov12(6)481-8
• 1 Topical CSA
• Mooren's ulcer in China a study of clinical
  characteristics and treatment Br J Ophthalmol
  2000841244-1249 ( November )
• Systemic CSA
• plasma trough levels of 150-200 ng/ml is
  recommended as initial treatment of choice
• Mooren ulcer. 4 severe bilateral disease courses
  with systemic cyclosporin A therapy Klin
  Monatsbl Augenheilkd. 1997 Nov211(5)306-11
• Hill J, Potter P. Treatment of Moorens Ulcer
  with cyclosporine A Report of three cases. Br J
  Ophthalmol, 7111-15, 1987.

29
Immunosuppressants

• NO comparative studies on type of
  immunosuppressants and route of administration

30
Additional surgical procedures

• Lamellar keratectomy - arrest the inflammatory
  process and allow healinG
• Mgmt of perforation tissue glue, BCL, patch
  graft
• Agrawal V, Kumar A, Sangwan V, Rao GN .
  Cyanoacrylate adhesive with conjunctival
  resection and superficial keratectomy in Mooren's
  ulcer. Indian J Ophthalmol. 1996 Mar44(1)23-7

31
Lamellar Keratoplasty

• Principle remove necrotic ulcerative cornea
  thoroughly and to reconstruct anatomical
  structure of the cornea
• removes antigenic targets of the cornea
• eliminates the risk of graft rejection
• prevents perforation
• improves vision
• Martin NF, Stark WJ, Maumenee AE. Treatment of
  Mooren's and Mooren's-like ulcer by lamellar
  keratectomy report of six eyes and literature
  review. Ophthalmic Surg. 1987 Aug18(8)564-9.
• Kinoshita S, Ohashi Y, Ohji M, Manabe R.
  Long-term results of keratoepithelioplasty in
  Mooren's ulcer.Ophthalmology. 1991
  Apr98(4)438-45.