Mooren's ulcer is a chronic, painful, progressive idiopathic ulceration of the peripheral cornea. The ulcer progresses circumferentially at first and then centrally until it involves the entire cornea. The peripheral ulcerative keratitis is not associated with scleritis. Although the cause of this rare disease is unknown, evidence gathered over the years favors an autoimmune etiology. In affected patients, autoantibodies are directed against a specific target molecule in the corneal stroma, probably triggered in genetically susceptible individuals. These patients also have a deficiency of suppressor T cells in the serum and a blastogenic response to the corneal stroma in the lymphocytes.1,2 Wilson et al.3 recently reported an association between Mooren's ulcer and hepatitis C infection.
Based on the clinical presentation and low-dose anterior segment fluorescein angiographic findings, 3 distinct types of Mooren's ulceration have been described.4 Unilateral Mooren's ulceration, which usually occurs in older persons, is characterized by excessively painful, progressive corneal ulceration in 1 eye. Bilateral aggressive Mooren's ulceration progresses circumferentially and may perforate before central involvement. Bilateral indolent Mooren's ulceration, which usually occurs in middle-aged persons, presents with progressive peripheral corneal guttering in both eyes and a minimal inflammatory response.4
Wood and Kaufman5 suggest 2 types of Mooren's ulcer: limited unilateral, which occurs in older patients and responds well to conservative therapy, and progressive bilateral, which occurs in younger patients and does not respond readily to conventional medical or surgical therapy.
Mooren's ulcer may be associated with posterior capsule opacification secondary to keratitis or chronic steroid use; this reduces the visual acuity even after successful resolution of the inflammation. Mooren's ulcer often occurs in patients with senile cataract who are visually impaired because of the cataract. Surgical trauma frequently induces the inflammation associated with the disease process. To our knowledge, there are few reports of cataract surgery in patients with advanced Mooren's ulcer.6,7
We describe 6 patients with advanced Mooren's ulcer who had successful cataract surgery and intraocular lens (IOL) implantation after complete control of the inflammation.
Patients and Methods
In this interventional case series, the medical records of all patients with Mooren's ulcer who had had cataract surgery between 1997 and 2003 at L.V. Prasad Eye Institute, Hyderabad, India, were retrospectively reviewed. The review revealed 6 patients with clinically diagnosed Mooren's ulcer who had cataract surgery. The diagnosis of Mooren's ulcer was made in the presence of a crescent-shaped peripheral corneal ulcer that began slightly central to the corneoscleral limbus, with extensive undermining of the central edge of the ulcer. In addition, there was no scleral involvement or detectable systemic disease. The indication for cataract surgery in these patients was subjective visual impairment and objective evaluation of the density of the lens opacification.
Table 1 shows the characteristics of Mooren's ulcer in each patient on presentation. Postoperative outcome measures were unilateral or bilateral involvement, visual acuity at presentation, presence of perforation, duration of symptoms before presentation, type of initial intervention, previous immunosuppression, and period of inactivity of the disease.
All 6 patients had conjunctival resection with tissue adhesive and bandage contact lens application to treat the ulcer. They were given prednisolone acetate 1% eyedrops every hour in the initial period. The medication was gradually tapered based on the clinical response. Three patients who presented with corneal perforation required anterior chamber reformation.
Patients 1 and 6 received intravenous methylprednisolone 500 mg/day stat for 3 days, intravenous cyclophosphamide 1 gm stat on the first day, and oral prednisolone 1 mg/kg tapered over 1 month. Patient 6 also had a lamellar patch graft and received 1 tablet of oral azathioprine (50 mg) twice daily for 2 years, after which he was maintained on oral methotrexate 7.5 mg/week until the last follow-up. The duration of immunosuppression ranged from 2 to 11 months.
After a period of inactivity of the disease process, ranging from 2 months to 11 months, cataract surgery was planned. No patient received immunosuppressive therapy preoperatively as the disease was quiescent.
Postoperative follow-up included at least 3 evaluations (1 week and 1 and 3 months). The best corrected visual acuity (BCVA) was noted. Slitlamp biomicroscopy was done to rule out evidence of a recurrence of Mooren's ulcer.
The age of the patients ranged from 60 to 76 years. The symptoms of Mooren's ulcer lasted between 10 and 365 days before presentation. Patient 4 was lost to follow-up after the first week. The mean follow-up was 11 months (range 3 months to 2 years).
Six patients had standard extracapsular cataract extraction (ECCE) with IOL implantation through a limbal incision. Patient 6 had phacoemulsification with IOL implantation through a scleral tunnel incision.
Four patients had bilateral Mooren's ulcer, and 2 had unilateral ulcer. Three patients presented with perforated Mooren's ulcer.
All patients received topical prednisolone acetate 1% eyedrops in tapering doses over 6 weeks. Three patients required systemic immunosuppressive therapy to control the disease. The duration of treatment ranged from 2 to 11 months.
The preoperative uncorrected visual acuity ranged from 20/60 to hand motions and the BCVA, from 20/30 to hand motions. The mean postoperative BCVA ranged from counting fingers at 1 m to 20/30 at 1 month and from counting fingers close to face to 20/20 at 3 months (Table 2).
Eight months after surgery, Mooren's ulcer recurred in patient 6 and was treated with intravenous methylprednisolone 500 mg and intravenous cyclophosphamide 1 gm stat on the first day. He continued to receive oral methotrexate 7.5 mg/week after having a conjunctival resection with tissue adhesive application.
Mooren's ulcer is an idiopathic, chronic, progressive, and painful disorder of the peripheral cornea. Steroids and immunosuppressives have been advocated for the management because of the possible autoimmune etiology of the disease.
Various treatments have been tried; these include topical corticosteroids, perilimbal conjunctival resection, lamellar keratectomy, oral corticosteroids, and immunosuppressive therapy in a stepladder approach.8–11 Penetrating keratoplasty and lamellar keratoplasty have also been performed. Recurrence of the disease is known to occur in patients who have surgery for corneal perforation during the active phase of the disease.12
There are few reports in the literature of the results of cataract surgery in patients with Mooren's ulcer. Such surgery can be complicated by extensive peripheral corneal thinning and reactivation of the inflammation associated with the disease process. Therefore, the approach to use for cataract surgery in these patients must be carefully considered and individualized. A tailored approach would avoid the corneal periphery because the corneal stroma is the target of destruction and inflammation can be precipitated by surgical trauma. With this in mind, we elected to perform ECCE with a limbal incision in 5 of the 6 patients. Phacoemulsification through a scleral tunnel incision, which we performed in 1 patient, is another safe approach. Akova and coauthors7 describe a patient with bilateral Mooren's ulcer who had successful phacoemulsification with IOL implantation after the inflammation was completely controlled with medical therapy. Watanabe and coauthors6 report similar results in 2 patients and emphasize the role of the scleral tunnel incision.7
In our patients, we achieved complete control of the inflammation before surgery. This is important as surgery in the inactive stage of the disease has a better prognosis. A noninflamed eye facilitates uneventful surgery and is less susceptible to recurrence of the inflammation.
Peripheral ulcerative keratitis, conjunctival ulcers, and scleritis in patients after uneventful clear corneal cataract extraction and standard ECCE have been reported.13,14 The peripheral cornea is closer to the conjunctiva, which has all the immunologic machinery necessary to generate an immune response. The peripheral cornea has more Langerhan's cells and immunoglobulin M than the central cornea.15 Infection, trauma, or surgery creates a risk for an immune flare-up. Hence, extra caution is advisable when considering surgical intervention in patients with Mooren's ulcer or diseases associated with peripheral ulcerative keratitis.
It is imperative that the eye is quiet for an adequate period before surgery. In our series, all patients had a quiescent period of at least 2 months before surgery was performed. One patient had a recurrence after surgery; however, it occurred 8 months postoperatively and was not attributed to surgical trauma.
A drawback of our study was the relatively short follow-up period. A longer follow-up is needed as Mooren's ulcer could reactivate during that time.
In conclusion, successful cataract surgery in patients with Mooren's ulcer requires careful control of inflammation and an adequate quiescent period preoperatively. Meticulous, individualized surgery results in satisfactory visual rehabilitation.
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