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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 481-483

Successful visual rehabilitation with penetrating keratoplasty and doughnut amniotic membrane transplant for corneal scarring in advanced Mooren's ulcer

1 Cornea and Anterior Segment Service, L V Prasad Eye Institute, KVC Campus, Vijayawada, Andhra Pradesh, India
2 Cornea and Anterior Segment Service, L V Prasad Eye Institute, Bhubaneswar, Odisha, India
3 Cornea Service, The Cornea Institute, L V Prasad Eye Institute, Kallam Anji Reddy Campus, Hyderabad, Telangana, India

Date of Submission24-Jan-2021
Date of Acceptance21-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Somasheila I Murthy
Head of Service, Department of Cornea, The Cornea Institute, Kallam Anji Reddy Campus, L V Prasad Eye Institute, LV Prasad Marg, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_203_21

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A 37-year-old one-eyed woman presented with a 3-month history of active Mooren's ulcer in her right eye. Conjunctival resection with tissue adhesive and bandage contact lens was performed thrice, despite which the eye progressed to total vascularized corneal scarring. After control of inflammation with systemic corticosteroids and immunomodulators, 10 months later she underwent penetrating keratoplasty with amniotic membrane transplant and tarsorrhaphy. At 2-year follow-up, the graft remained clear with no disease recurrence. This case highlights the modified surgical technique and the role of systemic immunosuppression while planning keratoplasty to achieve a successful outcome in end-stage Mooren's ulcer.

Keywords: Amniotic membrane transplant, conjunctival resection, Mooren's ulcer management, penetrating keratoplasty, peripheral ulcerative keratitis immunosuppression

How to cite this article:
Roy A, Cruz RD, Vuyyuru S, Murthy SI. Successful visual rehabilitation with penetrating keratoplasty and doughnut amniotic membrane transplant for corneal scarring in advanced Mooren's ulcer. Indian J Ophthalmol Case Rep 2021;1:481-3

How to cite this URL:
Roy A, Cruz RD, Vuyyuru S, Murthy SI. Successful visual rehabilitation with penetrating keratoplasty and doughnut amniotic membrane transplant for corneal scarring in advanced Mooren's ulcer. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:481-3. Available from: https://www.ijoreports.in/text.asp?2021/1/3/481/319987

Mooren's ulcer is an idiopathic progressive peripheral ulcerative keratitis affecting both young and elderly.[1] Often bilateral, the management of the relentless inflammation in active disease and visual rehabilitation in burnt-out disease are challenging. Penetrating keratoplasty (PK) has been traditionally associated with poor outcomes. Only a few isolated cases with short follow-up have reported success.[2] We report a variation of a technique for visual rehabilitation in a case of advanced Mooren's ulcer in a one-eyed patient, where the inflammation was first controlled with systemic immunosuppression and vision could be restored by PK with doughnut amniotic membrane transplant.

  Case Report Top

A 37-year-old woman presented with 3-month history of redness and painful visual loss in her right eye (RE). She had undergone RE conjunctival resection (CR) and cyanoacrylate glue application elsewhere and had stopped all medications. She had loss of vision in her left eye (LE) since 2 years, due to similar clinical course. On examination, best-corrected visual acuity (BCVA) in RE was 20/100, N24 and in LE was accurate projection of rays. The RE cornea showed 360° peripheral corneal thinning, overhanging edges, and vascularization of the stromal bed [Figure 1]a. The anterior chamber was formed. LE showed diffusely thinned-out cornea with scarring, vascularization, disorganized anterior chamber, and adherent iris [Figure 1]b. She was diagnosed as bilateral Mooren's ulcer and LE anterior staphyloma. Systemic workup to rule out any connective tissue disease was either negative or within normal limits. Topical prednisolone acetate (1%) q2 hourly, topical ofloxacin 0.3% q4 hourly, and homatropine hydrobromide (2%) bid were started. She underwent RE CR, tissue adhesive (TA), and bandage conjunctival resection(CR) contact lens (BCL) application. Oral prednisolone 1 mg/kg body weight was advised with weekly taper. However, she stopped the oral medications and followed up after 2 months with disease progression [Figure 1]c. Oral prednisolone was restarted and oral azathioprine (AZA) 50 mg twice daily was added as a steroid-sparing agent. A repeat CR + TA + BCL was performed. The TA + BCL were removed after 3 months [Figure 1]d. The eye remained quiescent for 10 months on oral AZA. She underwent PK with cataract extraction and intraocular lens implantation along with amniotic membrane transplant (AMT) and lateral tarsorrhaphy under local anesthesia. An excellent grade donor cornea was used. The trephine sizes were 8.0 mm for the host and 8.5 mm for the donor. Large bites were taken to include the limbus and avoid cheese wiring of the thinned out peripheral cornea. The AMT was fashioned with a trephine and applied similar to a technique described earlier.[3] After securing with fibrin glue over the surface, a central window was cut out and peripheral membrane was tucked and firmly pressed over the bare sclera 2 mm beyond the limbus to ensure a firm adhesion [Figure 2]a, [Figure 2]b, [Figure 2]c. At 2-year follow-up, her BCVA improved to 20/50, N12, intraocular pressure was normal, graft was clear and tarsorrhaphy intact [Figure 3]a and [Figure 3]b. She was advised to continue topical steroids with oral methotrexate 10 mg once a week (which had been switched from AZA due to the expense) along with folvite 5 mg/week. Complete blood counts, liver and renal parameters were tested once in 3 months and continued to remain normal.
Figure 1: (a) Slit-lamp photograph of the right eye at presentation shows 360 degrees peripheral corneal thinning and overhanging edematous edges. (b) Left eye shows diffusely thinned out cornea with scarring and vascularisation and areas of descemetocele formation. (c) Two months later worsening of the disease with progressive corneal involvement centrally is noted in the right eye. (d) Five months after initial presentation (2 months after re-starting oral corticosteroids and adding Azathioprine) there is corneal scarring and vascularization and early disease quiescence in the right eye.

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Figure 2: Acknowledgement: Dr T Suchi Smita (a) Illustration showing the graft sutured in place with 16 interrupted sutures, with long bites to include the limbus. (b) The amniotic membrane (AM) was fashioned with a trephine and a central window was cut out and secured with fibrin glue over the surface. (c) The graft at the end of surgery covered by amniotic membrane which was firmly pressed over the bare sclera 2 mm beyond the limbus to ensure a firm adhesion.

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Figure 3: (a) Low magnification slit-lamp picture shows tarsorrhaphy in situ with a clear graft. (b) Slit-lamp photograph with diffuse illumination shows large residual sutures and compact graft, 2 years later

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  Discussion Top

A step ladder pattern is recommended in the management of Mooren's ulcer, based on the severity, age, and bilaterality.[4],[5] CR + TA + BCL is conventionally performed as initial therapy to stabilize the disease. TA forms a biological barrier between the host cornea and the re-epithelializing conjunctiva and the immune components it may carry;[4] however, this is inadequate as a stand-alone technique to prevent recurrences[6] and should be combined with systemic immunosuppression.

The first successful outcome of PK was reported by Brown and Mondino in 1980 for end-stage Mooren's.[2] They too emphasized the need for a disease-free period (minimum 4 months) before attempting keratoplasty.[2] Due to the immunological nature of the underlying etiology and the necessity of a large graft, PK in Mooren's ulcer has a high risk of rejection and failure.[2],[7] Therefore, we were hesitant initially to operate in our case. Cho et al. reported a case of advanced Mooren's ulcer successfully treated with deep anterior lamellar keratoplasty and emphasized the need for intensive immunosuppression.[8] Jerez-Pena et al. reported a case of Mooren's ulcer with history of repeated failed keratoplasties treated with bilateral Boston keratoprosthesis. Though initially the eyes responded well, the final outcome was not rewarding.[9] Guindolet et al. described rituximab infusions in five refractory cases with a successful outcome, however the cost and systemic screening before administration limit its use.[10]

We did consider an option of scleral contact lens in our case but based on the density of scarring it appeared unlikely that she would have visual improvement. Based on the limited options available to us, in our case we decided to perform PK with AMT after a quiescence of 10 months on immunosuppression. The use of doughnut AMT has been recently described as a technique for partial LSCD combined with PK.[3] In this technique, it is placed over the bare scleral region adjoining the limbus and may act as a barrier to block new cytotoxic stimuli to the limbus. In the current case, it also added substrate over the thinned out peripheral corneal rim and promoted ocular surface healing.

  Conclusion Top

In conclusion, modification of the technique under the cover of systemic immunosuppression appears to be a viable option for visual rehabilitation in Mooren's ulcer. The role of immunosuppressive therapy and frequent follow-ups cannot be over-emphasized.


Dr T Suchi Smitha, Consultant, Cornea and Refractive surgery, Deenanath Mangeshkar hospital, Pune for the illustrations in [Figure 2].

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This work was supported by Hyderabad Eye Research Foundation, Hyderabad, India. The funders had no role in the preparation, review, or approval of the manuscript.

Conflicts of interest

There are no conflicts of interest.

  References Top

Watson PG. Management of Mooren's ulceration. Eye 1997;11:349-56.  Back to cited text no. 1
Brown SI, Mondino BJ. Penetrating keratoplasty in Mooren's ulcer. Am J Ophthalmol 1980;89:255-8.  Back to cited text no. 2
Singh A, Murthy SI, Gandhi A, Sangwan VS. “Doughnut” amniotic membrane transplantation with penetrating keratoplasty for vernal keratoconjunctivitis with limbal stem cell disease. Cornea 2020. doi: 10.1097/ico. 0000000000002553.  Back to cited text no. 3
Sangwan VS, Zafirakis P, Foster CS. Mooren's ulcer: Current concepts in management. Indian J Ophthalmol 1997;45:7-17.  Back to cited text no. 4
[PUBMED]  [Full text]  
Ashar JN, Mathur A, Sangwan VS. Immunosuppression for Mooren's ulcer: Evaluation of the stepladder approach—topical, oral and intravenous immunosuppressive agents. Br J Ophthalmol 2013;97:1391-4.  Back to cited text no. 5
Lal I, Shivanagari SB, Ali MH, Vazirani J. Efficacy of conjunctival resection with cyanoacrylate glue application in preventing recurrences of Mooren's ulcer. Br J Ophthalmol 2016;100:971-5.  Back to cited text no. 6
Skeens HM, Holland EJ. Large-diameter penetrating keratoplasty: Indications and outcomes. Cornea 2010;29:296-301.  Back to cited text no. 7
Cho YK, Lee SY, Cho UJ, Ambati BK. Large-diameter Deep Anterior Lamellar Keratoplasty: An Alternative Treatment for Mooren Ulcer. Korean J Ophthalmol 2020:34:413-5.  Back to cited text no. 8
Jerez-Peña M, Salvador-Culla B, de la Paz MF, Barraquer RI. Bilateral Boston keratoprosthesis type 1 in a case of severe Mooren's ulcer. Eur J Ophthalmol 2020;6:1120672120909768.  Back to cited text no. 9
Guindolet D, Reynaud C, Clavel G, Belangé G, Benmahmed M, Doan S, et al. Management of severe and refractory Mooren's ulcers with rituximab. Br J Ophthalmol 2017;101:418-22.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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