INTRAOCULAR RETINOBLASTOMA PRESENTING AS OCULAR AND ORBITAL INFLAMMATION

Retinoblastoma is the most common primary intraocular malignancy of ch ildhood, usually presenting by age 3 years and only rarely after age 5 [1]. Common presenting signs include leukocoria and strabismus [2] an d, with a complete, dilated, funduscopic examination, this malignancy often is accurately and expediently diagnosed. Less frequent presentin g signs include hyphema, glaucoma, and inflammation. Ocular and orbita l inflammation have been reported as initial signs of intraocular reti noblastoma [3-17], and both have been frequently associated with delay ed or incorrect diagnosis. A delay in diagnosis can adversely affect p rognosis in this potentially lethal disease. A rare subtype of retinob lastoma, diffuse infiltrating retinoblastoma, characterized by flat in filtration of the retina by tumor cells, is more likely to present wit h signs of intraocular inflammation and is discussed in detail in this chapter. Its demographics and diagnostic challenges, along with treat ment options and prognosis, are reviewed. In addition, intraocular ret inoblastoma sometimes can cause orbital inflammation. The characterist ics of such cases are highlighted. The need to include retinoblastoma in the differential diagnosis of childhood ocular and orbital inflamma tory conditions is stressed.