CLINICAL AND FLUORESCEIN ANGIOGRAPHIC CHARACTERISTICS OF LATE–ONSET RETINOBLASTOMA WITH COATS’ RESPONSE IN CHILDREN

Abstract

Abstract: : Purpose: To review clinical features and fluorescein angiographic characteristics of children presenting with late retinoblastoma. Methods: Retrospective review of clinical cases seen at a major tertiary referral center, including fundus photographs and fluorescein angiograms. Results: Three boys were identified with unilateral non–heritable retinoblastoma ranging from 3 to 6 years of age. All had evidence of chronic disease with inflamed eyes, and neovascular glaucoma. All had significant intra–retinal and subretinal exudates mimicking Coats’ disease. None had intra–ocular calcification by ultrasound or CT imaging. All had diffuse sub–retinal and pre–retinal infiltrates that were difficult to distinguish from gliosis or vaso–proliferative tumors. All had significant retinal vascular remodeling with engorged and dilated retinal and tumor vessels that demonstrated arterial–venous communications and capillary non–perfusion. These were best visualized with wide–field fluorescein angiography. Histopathology in all three cases demonstrated well–differentiated retinoblastoma with characteristic rosettes. None had features typical of diffuse infiltrating retinoblastoma. No case had definite extra–ocular disease, though one had extension beyond the lamina cribrosa. Conclusions: These three cases demonstrate the diagnostic dilemma usually associated with atypical retinoblastoma. The presentation of an older male infant or toddler with unilateral ocular inflammation and heavy retinal exudates may represent late–onset retinoblastoma. The inability to confirm this diagnosis in a timely manner may lead to delays in definitive management. The unusual angiographic pattern may at first be confused with Coats’ disease. This small series demonstrates retinal vascular remodeling with arterio–venous communication, peripheral non–perfusion, and the absence of aneurysms or telengiectasia in three patients with delayed diagnosis of retinoblastoma. The clinical presentations and angiographic fundus appearance described in this series should not be confused with that of Coats’ disease. Furthermore, the absence of calcification or an identifiable distinct mass should not rule out retinoblastoma.

Keywords: retinoblastoma • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)