Amniotic Membrane Transplant with a Special Technique (Motowa's Sandwich Technique) in Mooren's Ulcer.

To illustrate amniotic membrane transplant (AMT) with a novel surgical technique ("sandwich technique") for treating patients with malignant Mooren's ulcer. A case report of a patient with bilateral, malignant Mooren's ulcer who had undergone systemic steroid therapy and topical immunosuppresive therapy to stabilize his condition. However, perforation of cornea occurred in one eye. AMT with a new surgical technique ("Motowa's sandwich technique") was performed to treat this case. On the 1(st) day postoperatively, there was no pain, no photophobia, and visual acuity was same as preoperatively. At 4 weeks postoperatively, visual acuity improved in the right eye to 20/160 with pinhole, and there was no fluorescein staining. The right eye was quiet. Amniotic membrane transplant with "Motowa's sandwich technique" resulted in the preservation of the anatomical integrity and progression of disease was halted along with an improved vision. This technique is a novel surgical modality in treating Mooren's ulcer. Further study on a large cohort of patients is required for evidence-based data to verify the outcome of this initial case report.

INTRODUCTION

Mooren's ulcer is a rare, chronic painful corneal disorder of unknown etiology that can lead to the loss of the eye if untreated. This ulcerative keratitis usually begins in the peripheral cornea and progresses circumferentially to the central cornea. It is an idiopathic condition with no known systemic association. Mooren's ulcer usually presents in healthy adult males. Significant morbidity is caused by the disease itself as well as by local or systemic immunosuppressive therapy. The incidence of corneal perforation and loss of vision can be as high as 13.5%.1 Malignant Mooren's ulcer is typically more recalcitrant to therapy. In majority of cases after surgery, continued adjunctive topical and or systemic immunosuppressive therapy is required.234 We describe a case of amniotic membrane transplant (AMT) with a novel sandwich technique to halt the melting process and progression in a patient with malignant Mooren's ulcer.

CASE REPORT

An 87-year-old male presented to the emergency department on March 2002 with a 10-day history of severe pain, redness and tearing in his right eye that was worse in the left eye. In 1986, the patient had undergone extracapsular cataract extraction with intraocular lens implantation in the left eye. There was no history of trauma including ocular trauma, no history of joint pain, skin rashes or drug allergies. The pupils were normal and equally reactive to light bilaterally. Ocular motility was normal. Slit lamp examination revealed acute conjunctival hyperemia and inferior thinning in both corneas. The thinning was 360o degree from the periphery extending to the center of the cornea. The ulcer was circumferentially at the periphery Best corrected visual acuity was 5/200 and 20/200 in his right and left eyes respectively. The dilated retinal examination was unremarkable [Figure 1].

Figure 1

360° peripheral corneal melting with area of severe thinning with positive sedaltits

Autoimmune diseases had been excluded by the general physician. Laboratory results for rheumatoid factor was negative, antinuclear antibody was negative by immunoflourescent assay, c-ANCA (titre of < 1:2), p-ANCA (titer of < 1:2), SSA antibodies (anti-Ro, 60 kD) were negative, SSB antibodies (anti-La) was also negative, IgA quantitative (Immunoglobulin A) titer of 2.32 g/L (normal adult range 0.7–4.0), IgG quantitative (Immunoglobulin G) titer of 12.3 g/L (normal adult range 7.0–16.0), IgM quantitative (Immunoglobulin M) titer of 1.07 g/L (normal adult range 0.4–2.3), thyroid stimulating hormone titer 1.27 (normal male adult range 0.32–5.00), chest X-ray was normal. The patient was prescribed prednisolone by the general physician.

After the ophthalmic examination, the patients was advised to use topical antibiotic, oral doxycycline and lubricants and observed. A month later, his condition continued to worsen, and he still complained of severe pain, photophobia, tearing and poor vision. Examination revealed hand motion vision in both eyes with severe conjunctival injection, inferior corneal thinning and descemetocele inferiorly in the right eye. The left eye had a greater conjunctival injection, with corneal thinning inferiorly, a small perforation, aqueous leak, shallow anterior chamber and pseudophakia [Figure 1]. We elected to performed surgery. The Patient underwent administration of general anesthesia, and a double AMT action using the Motowa's sandwich technique was performed in both eyes.

At day 1 postoperatively, there was no pain, no photophobia, and visual acuity was the same as preoperatively. At 4 weeks postoperatively, visual acuity improved in the right eye to 20/160 with pinhole. 9 weeks postoperatively, the patient was admitted to the hospital due to complaints of severe pain and an inability to open his right eye spontaneously. The visual acuity was hand motion in both eyes and perforation of the right cornea with iris prolapse, moderate anterior chamber depth, and cataractous lens. The left eye was unremarkable. The patient underwent a tectonic corneal graft, extra capsular cataract extraction and double AMT in the right eye. At 3 weeks postoperatively, there was no pain, no photophobia, and visual acuity was 20/160 and counting fingers at 3 feet with a pinhole for his right and left eye respectively. The intraocular pressure was 18 mm Hg in the right eye and 24 mm Hg in the left eye. The patient was prescribed topical betaxolol hydrochloride (Betoptic) 0.5% twice a day for the left eye.

At last visit after 6 years, the patient denied pain or photophobia. Visual acuity as 4/200 in the right eye and 20/125 improving with a pinhole to 20/60 in the left eye. Intraocular pressure was 11 mm Hg and 17 mm Hg in the right and left eyes respectively. Slit-lamp examination of the right eye showed a clear corneal tectonic graft and with no fluorescein staining and a quiet eye [Figure 2]. The left eye was quiet with a clear central cornea and no fluorescein staining, and the patient had no complaints.

Figure 2

6 years after surgery with peripheral corneal scarring and quite eye

CONCLUSION

The etiology of Mooren's ulcer is still not well understood. It is probably an autoimmune process as anti-corneal, and anti-conjunctival tissue antibody had been isolated from patients suffering from this disorder.5 Another confirmation of the autoimmune nature origin is the presence of abundant proteo-glycolytic enzymes secreted by the mononucleated cells and neutrophils from the conjunctival tissue surrounding the corneal lesion that progressively infiltrate the area surrounding the ulcer.6 Furthermore, the T-suppressor lymphocytes deficit in patient blood samples may also support that.7

Mooren's ulcer is classified in two main forms by Wood and Kaufman.8 Type 1 is the benign form, generally monolateral, which mainly affects the elderly. The symptoms are unclear, but this type responds well to medical treatment and surgery. Type 2 is the malignant form. It can occur bilaterally in 25% of cases in white subjects and 75% in black subjects. It mainly affects the young. Watson divided the disease into three types based on the clinical presentation: Unilateral, bilateral aggressive and bilateral indolent Mooren's ulcer.9

It is extremely difficult to treat Mooren's ulcer and in many cases, the results are poor. Treatment starts with steroids administered systemically and locally, but if this is unsuccessful, the complete excision of the perilimbal conjunctiva and episclera near the ulcer is made.9 The use of immunosuppressive drugs, in particular cyclosporin A, should be reserved for more severe forms.10 In the event of ulcer perforation, the area can be covered with amniotic membrane or a lamellar keratoplasty can be performed.11 The current case was a bilateral malignant Mooren's ulcer. Local and systemic treatment with cortisone failed to prevent the progression of the disorder to a corneal perforation and the significant comorbidity of increased intraocular pressure. Hence, a tectonic corneal graft was warranted, and we elected to perform AMT using the Motowa sandwich technique.

This new surgical technique allowed preservation of the cornea, obviating the administration of systemic and topical cyclosporine thereby, reducing the possibility of secondary glaucoma. Hence, we believe that this surgical technique can be used for early Mooren's ulcers where the amniotic membrane may halt disease progression by acting as a barrier to preserve corneal tissue.