Is ultra wide-field retinal imaging alone appropriate for retinal angioma screening in lower risk subjects attending Von Hippel-Lindau (VHL) clinics?

Purpose: To determine if non-mydriatic ultra wide-angle digital retinal imaging alone is effective for screening subjects considered to be at 50% or lower risk for developing retinal angiomas.Methods: Digital records and retinal images of subjects attending a regional Von-Hippel-Lindau disease multi-disciplinary clinic over a 12 month period were reviewed. Individuals were stratified for risk of developing retinal angiomas on the basis of age, clinical features and genetic risk. The image quality and necessity for subsequent mydriatic fundoscopy were assessed.Results: Eighty subjects from 55 pedigrees attended the VHL clinic over 12 months. Of these, 44 (55%) were considered to be at lower risk for retinal angiomatosis: 34 (77%) because they had reached at least 30 years of age without developing an angioma, 16 with a presenting solitary tumour of the type associated with VHL but no identifiable gene mutation, and 12 with a family history giving them a 50% risk of carrying a gene mutation. Eighteen patients fulfilled two low risk criteria. All were able to comply with imaging but poor image quality (limited view of the inferior retinal far-periphery) required subsequent dilated fundoscopy in five (6%) eyes of three patients.Conclusions: Non-mydriatic ultra wide-field retinal imaging enabled satisfactory assessment in over 95% of lower-risk VHL subjects. Virtual clinics or remote imaging of lower-risk subjects may improve both the efficiency and flexibility in the provision of multi-disciplinary VHL services and the patient experience.