Case Reports

Clinical challenges in the approach of aggressive bilateral Mooren's Ulcer

Case Report Details

First Author: J.Roque PORTUGAL

Co Author(s):    J. Almeida   R. Basto   S. Henriques   D. Silva   B. Grima   I. Prieto     

Abstract Details

Purpose:

To report a case of aggressive bilateral Mooren’s ulcer refractory to multiple treatment strategies.

Setting:

Prof. Fernando Fonseca Hospital, Amadora, Lisbon, Portugal

Report of Case:

We present the case of a 26 year-old patient from Sub-Saharan Africa who had been treated for recurrent bilateral corneal ulcers in his country of origin since the past year. The patient was maintained under topical and systemic cyclosporine because of suspicion an autoimmune disease. Nevertheless left eye had already total corneal opacification because of a perforated ulcer that was presumably treated with a conjunctival flap. In December 2019, he presented to our Emmergency Department with a perforated peripheral corneal ulcer in the right eye (OD) and subcapsular cataract. Immediate surgical repair of the perforation was performed, with release of incarcerated iris and application of bovine pericardium (Tutopatch). Early postoperative course was favorable and the patient maintained reasonable visual acuity OD (4/10). Extensive laboratory investigation for auto-immune and infectious diseases was positive only for active hepatitis B infection, which was subsequently treated. Nonetheless we observed subsequent clinical deterioration manifested by progressive peripheral corneal thinning with no scleral involvement. We excluded all predisposing factors and admitted the clinical diagnosis of bilateral Mooren’s ulcer. Immunosuppressive treatment included topical corticosteroids, topical cyclosporine, successive oral cyclosporine dose augmentations as well as intravenous (IV) pulses of methylprednisolone followed by oral prednisolone. Corneal ulceration did not respond to any of those therapies, so the patient was then submitted to 3 cycles of IV cyclophosphamide. Lastly, stabilization of thinned corneal areas was noted. The patient is currently without active ulceration but remains under tight monitoring as we manage an extremely slow tapering of oral corticosteroids according to the evolution of corneal thinning.

Conclusion/Take Home Message:

Mooren's ulcer is a rapidly progressive ulcerative keratitis that initially affects the peripheral cornea but may spread circumferentially and then centrally. Its exact pathogenesis as well as the optimal treatment for severe progressive forms remains unclear. We highlight the importance of a multidisciplinary team in excluding systemic disorders that could be responsible for corneal ulceration. This approach is also critical in the management of aggressive immunosuppressive treatments that need to be rapidly instituted.

Financial Disclosure:

None