|Year : 2012 | Volume
| Issue : 1 | Page : 50-53
Mooren's corneal ulceration in a pseudophakic eye: A case report and literature review
Emmanuel R Abah, Adeyemi O Akinwande, Victoria A Pam
Department of Ophthalmology, Ahmadu Bello University Teaching Hospital, Shika-Zaria, Nigeria
|Date of Web Publication||28-Aug-2012|
Emmanuel R Abah
Department of Ophthalmology, Ahmadu Bello University Teaching Hospital, Shika-Zaria
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Surgery (ECCE+ PCIOL) is an unusual possible aetiology of Mooren's corneal ulceration. A clinical and laboratory evaluation of a case of a 60-year-old male Nigerian who in the immediate postoperative period developed Mooren's corneal ulceration after an uneventful extracapsular cataract extraction and a posterior chamber intraocular lens (ECCE+PCIOL) implantation at an outreach center is presented. Healing occurred within 2 weeks of medical and surgical intervention, but no remarkable visual improvement. A review of literature from relevant sources with emphasis on management options is also incorporated. Many cases of Mooren's corneal ulceration remain refractory to treatment even in advanced countries. However, early case finding and prompt referral by trained primary eye care personnel may help to reduce severe visual morbidity in this environment.
Keywords: ECCE+PCIOL, Mooren′s corneal ulceration, Nigerian male
|How to cite this article:|
Abah ER, Akinwande AO, Pam VA. Mooren's corneal ulceration in a pseudophakic eye: A case report and literature review. Ann Nigerian Med 2012;6:50-3
|How to cite this URL:|
Abah ER, Akinwande AO, Pam VA. Mooren's corneal ulceration in a pseudophakic eye: A case report and literature review. Ann Nigerian Med [serial online] 2012 [cited 2020 Apr 5];6:50-3. Available from: http://www.anmjournal.com/text.asp?2012/6/1/50/100229
| Introduction|| |
Mooren's ulcer (MU) is a painful, chronic ulcerative keratitis that begins peripherally and progresses circumferentially and centrally. Diagnosis used to be predominantly by exclusion, but currently specific histological diagnosis can be made. 
MU is typically seen in adult males without evidence of systemic disease. However, it may occur at any age and in both sexes. 
The disorder has been classified by Watson  into three distinct varieties based on clinical presentation and anterior segment fluorescein angiographic findings. The varieties include the following:
1. Unilateral Mooren's Ulceration (UM): Painful, progressive, and seen in elderly patients. Tends to be benign and responds fairly well to medical and surgical treatment. It is associated with nonperfusion of the superficial vascular plexus of the anterior segment.
2. Bilateral Aggressive Mooren's Ulceration (BAM): This occurs in young patients and is more malignant. Vascular leakage and new vessel formation extending into the base of the ulcer is a characteristic of BAM.
3. Bilateral Indolent Mooren's Ulceration (BIM): Tends to occur in middle-aged patients and often presents as bilateral progressive peripheral corneal guttering with little inflammatory response. The vascular architecture remains unchanged; however, an extension of new vessels into the ulcer is seen.
| Case Report|| |
A 60-year-old man presented with a 2 months history of blurred vision, pain, photophobia, and purulent discharge in the OS(Left Eye). This occurred soon after an uneventful extracapsular cataract extraction and posterior chamber intraocular lens implantation (ECCE/PCIOL) at a cataract eye camp.
Seven months earlier HU had an uneventful ECCE/PCIOL implantation in the OD(Right Eye) at the same eye camp. Two weeks into the postoperative period, the patient developed severe purulent conjunctivitis in the OS which lasted 3 weeks and resolved on topical and systemic broad-spectrum antibiotics, topical nonsteroidal anti-inflammatory, and cycloplegic drugs.
Eye swab for culture and sensitivity from OU (Both Eyes) at various times of presentation yielded no growth. Systemic review was normal. The patient was neither a diabetic nor hypertensive.
General physical examination of the patient revealed a well-nourished elderly man who was neither pale nor febrile. He was anicteric and showed no sign of any skin lesions. His pulse rate was 82/minute, regular, and of moderate volume. Blood pressure was 130/80 mmHg. The respiratory and musculoskeletal systems were normal. He had no urethral discharge and no focal neurological deficits.
Ocular examination revealed a visual acuity (VA) of 6/9 and hand motion (HM) in the OD and OS, respectively. Further examination of the OD revealed a pseudophakia with healed corneal incision wound from 10 to 2 o'clock position. Anterior and posterior segments of OD were normal. However, OS revealed a pseudophakia, scanty purulent discharge, lid edema, and circumcorneal injection. A peripheral corneal ulcer with overhanging edge from 7 to 4 o'clock position was noted [Figure 1].
In addition, central corneal epithelial edema, few keratic precipitates, and moderate aqueous flare were present. Posterior segment examination of OS was precluded by the corneal edema.
A diagnosis of bilateral pseudophakia and postoperative MU in the OS was made.
The treatment of this condition (OS) was both medical and surgical. The medical treatment included oral acetazolamide and diclofenac potassium and topical tropicamide, and diclofenac sodium , while the surgical treatment was conjuctivectomy of 360 degrees and cryotherapy.
Healing with scaring of the ulcer crater occurred within 2 weeks. The left eye was quiet and pain free although vision remained unimproved
| Discussion|| |
The etiology of MU is unknown; however, it has been associated with entities such as infections, e.g., helminthiasis, hepatitis C, syphilis, tuberculosis, herpes simplex, and herpes zoster infections; physical trauma ; corneal foreign bodies ; chemical burns ; surgery, e.g., cataract extraction (as is the case with this patient) and genetic/racial factors.
Majekodunmi  in Lagos, Nigeria, observed that previous trauma, corneal foreign bodies, and helminthiasis precipitate MU via collagenolytic activity of the conjunctiva. This is supported by the fact that their cases healed after limbal conjunctivectomy. However, Acharya et al.,  in a retrospective study of patients who had extracapsular cataract extraction in Madurai, India, concluded that exposure of normally concealed antigens may contribute to the pathogenesis of MU in some patients. Zelefsky et al.  demonstrated an association between MU and human leucocyte antigen (HLA) type DR17(3) in patients from Tamil Nadu state of south India. Forty percent of the patients with MU tested positive for HLA DR17(3) compared with 16% of the control. Another 45% of the patients also tested positive for the closely linked HLA DQ2 compared with 11% of the control.
In general, the pathophysiological mechanism of MU remains unknown, but there is evidence to suggest that an autoimmune process, with both cell-mediated and humoral components exists. Plasma cells, neutrophils, mast cells, and eosinophils have been found in the involved areas. 
Martin and colleagues  have proposed a mechanism for the perpetration of the ulcerative process, suggesting that a systemic disease, infection, or trauma may alter corneal antigens, stimulating both humoral and cellular responses. In the process, complement activation leads to neutrophil chemotaxis and deregulation with release of collagenases, causing corneal melting and further alteration and exposure of altered corneal antigens, thus perpetrating the process. This cycle continues until the entire cornea is destroyed.
The conjunctiva adjacent to the lesion shows a hyperemic and edematous stroma infiltrated mainly by plasma cells and lymphocytes without signs of vasculitis. The peripheral portion of the ulcer is characterized by necrobiotic and hemorrhagic elements along with an absence of the overlying epithelium and Bowman's membrane. Inflammation of the corneal stroma is present in the peripheral but absent in the central edge of the ulcer, a characteristic sign of a rodent ulcer. Therefore, histopathological study is very useful for the final diagnosis and the differential diagnosis of Mooren's ulcer from other causes of peripheral ulcerative keratitis. 
The difficulty in establishing the exact etiology of MU has made provision of a single treatment option farfetched. Hence, the need for a stepwise approach in the management of MU.
The stepwise approach in the management of MU guides the clinician's choice of treatment. Progress is made from one step to the other depending on patient's response. The overall goals of therapy are to arrest the destructive process and to promote healing and reepithelization of the corneal surface. The treatment options include the following:-
1. Steroid Therapy: Initial therapy should include intensive topical regimen of prednisolone acetate or prednisolone phosphate 1% hourly in addition to cycloplegics and prophylactic antibiotics. Once healing occurs, topical steroids can be tapered slowly over several months. This management option has yielded result in the benign (unilateral) MU.
Oral pulse therapy (prednisolone 60-100 mg daily) can be considered where topical steroids may be contraindicated because of precariously deep ulcer or infiltrates. 
Topical steroids can be used along with topical tetracycline or medroxy progesterone for anticollagenolytic properties. This helps to prevent the breakdown of corneal matrix by countering the effects of collagenases released by the conjunctiva.
Therapeutic soft contact lenses or patching can be used along with these medications to encourage reepithilization of the cornea and prevent denuding of the cornea by avoiding direct pressure from the eyelids.
2. Conjunctival Resection: If the ulcer progresses despite the steroid regimen, conjunctival resection should be performed under topical and subconjuctival anesthesia. The conjunctiva is excised to bare sclera, extending at least 2 clock hours to either side of the peripheral ulcer and about 4 mm posterior to the corneoscleral limbus parallel to the ulcer. Postoperatively, a firm pressure dressing should be applied. Multiple resections may be needed. It is thought that the 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. 
Mpyet et al.  in Jos, Nigeria, found that patients who did not use traditional eye medicines or indulge in self medication did well on conjuctivectomy with systemic and topical steroids.
3. Immunosuppressive Chemotherapy: Is recommended for bilateral or progressive MU that fails to respond to therapeutic steroids and conjunctival resection. These may require systemic cytotoxic chemotherapy which brings to a halt the progressive corneal destruction. This action is predominantly via immunomodulation. Cyclosporine A acts by suppression of the helper T-cell population and stimulation of the depressed population of suppressor and cytotoxic T-cell present in patients with MU.
The most commonly used cytotoxic drugs include the following:
Either of these is used for 6-24 months.
- Oral cyclophosphamide (2 mg/kg/day for 4-5 days every 3-4 weeks).
- Oral methotrexate (7.5-15 mg once weekly).
- Oral azathioprine (2 mg/kg/day).
Oral cyclosporine A (3-4 mg/kg/day). This has also been successfully used to treat bilateral MU unresponsive to other forms of treatment. ,
4. Additional Surgical Procedures: May be necessary when topical steroids, conjunctival resection, and immunosuppressives have failed. These additional surgical procedures include:
Superficial lamellar keratectomy: This procedure involves excision of overhanging edge of the ulcer and scraping of the ulcer bed with size 15 Bard-parker blade. It reduces activated keratocyctes and polymorphs which are sources of collagenase, thereby limiting corneal destruction.
Surgical procedures for perforation: For small perforations, tissue adhesives or soft bandage contact lens may be used, while patch graft and partial penetrating keratoplasty are used for moderate perforations. 
5. Rehabilitation: This is necessary for patients with low residual vision. Low vision aids may be useful. Penetrating keratoplasty at this stage is associated with high failure rate because of the thinned and vascularized cornea.
| Conclusion|| |
In developed countries, advances have been made in the step-wise approach to the management of MU. However, a significant percentage of cases remain refractory to available therapies and eventually result in severe visual morbidity. Increased patient awareness through health education, early case finding, and prompt referral of cases by trained primary eye care personnel may help to reduce the severe visual morbidity in this environment.
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