Abstract
Purpose:
Fluorescein angiography (FA) is used for the identification of retinal capillary hemangiomas in patients with von Hippel Lindau disease (VHL). We show that new retinal hemangiomas can also be identified using wide-field autofluorescence imaging, which may obviate the need for fluorescein administration in some cases.
Methods:
A consecutive, retrospective case series of patients with von Hippel Lindau disease that were seen between 2011 and 2013.
Results:
Thirty-one patients with previously identified mutations of the VHL gene were seen between 2011 and 2013. During this time period, they underwent 102 wide-field retinal imaging studies using the Optos system, which included autofluorescence as well as FA. Seventeen patients (55%, median age 34 years) were found to have retinal capillary hemangiomas, of whom 11 developed a total of 17 new hemangiomas during the study period. Fourteen patients (45%, median age 14.5 years) remained hemangioma-free. Of the newly identified hemangiomas, 9 were located within the autofluorescence image field. Notably, all 9 of these lesions (100%) were detectable using autofluorescence. All new hemangiomas that were not visualized with autofluorescence were located outside of the autofluorescence image field. Hemangiomas that remained active despite treatment did not appear significantly different on autofluorescence from ones that had been treated successfully.
Conclusions:
Wide-field autofluorescence imaging is as effective as FA for detecting new retinal capillary hemangiomas associated with von Hippel Lindau disease. While FA is superior for surveillance of previously treated hemangiomas, obtaining autofluorescence images from retinal periphery may obviate the need for fluorescein administration in screening younger VHL patients for new hemangiomas.
Keywords: 688 retina •
552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) •
550 imaging/image analysis: clinical