Intraocular involvement in multifocal fibrosclerosis

Abstract

Objective To report the clinical and histopathologic findings of intraocular involvement in a patient with multifocal fibrosclerosis and the response of the patient’s choroidal masses to external beam radiotherapy. Design Case report with clinicopathologic correlation of enucleated eyes. Methods The patient was studied by clinical observation, contact B- and A-scan ultrasonography, orbital magnetic resonance imaging, fluorescein angiography, indocyanine green angiography, choroidal biopsy, and gross and histopathologic examination of the enucleated eyes. Tissue obtained at an earlier laparotomy was also reviewed. External beam radiotherapy was used when high-dose corticosteroid and low-dose methotrexate therapy failed to decrease the size of the choroidal masses or improve the patient’s vision. Main outcome measures Changes in the clinical and ultrasonographic size of the choroidal masses, the clinical appearance of these masses, and the patient’s visual acuity in response to external beam radiotherapy were monitored premortem. Histopathologic findings in the enucleated eyes were compared with the changes in previous abdominal and choroidal biopsy specimens and with tissue alterations reported in multifocal fibrosclerosis. Results Biopsy of the choroidal mass revealed a fibrosclerosing process similar to that found in the abdomen. The patient received external beam radiotherapy with disappearance of the masses. Fibrosclerosing changes similar to those seen in the abdomen were observed replacing the choroid in the enucleated eyes. Conclusions Multifocal fibrosclerosis may involve the choroid with histopathologic changes similar to those that have been described in other locations in the body. External beam radiotherapy may be an effective treatment for intraocular involvement by multifocal fibrosclerosis.