EARLY DETECTION OF RETINAL HEMANGIOBLASTOMAS IN VON HIPPEL-LINDAU DISEASE USING ULTRA-WIDEFIELD FLUORESCEIN ANGIOGRAPHY

Retina. 2018 Apr;38(4):748-754. doi: 10.1097/IAE.0000000000001601.

Abstract

Purpose: To study the use of ultra-widefield fluorescein angiography (UWF FA) in the detection and management of retinal capillary hemangioblastomas in patients with von Hippel-Lindau disease.

Methods: This is a retrospective study of patients with von Hippel-Lindau disease who underwent UWF FA using the Optos camera at a single center from June 2009 to May 2015. The clinical use of UWF FA was reviewed, and the number of hemangioblastomas identified on UWF FA was compared with ophthalmoscopy and a simulated seven standard field (7SF) FA montage.

Results: Twenty eyes of 10 patients were identified. Only 33% of lesions seen on UWF FA were also found on ophthalmoscopy, and 88% of lesions visualized on UWF FA were located outside the 7SF overlay. In 5 eyes that had gaze steering, 18% of lesions could be visualized only on gaze-steered images. For the 14 eyes with data available, 6 had procedures recommended and 8 eyes observed based on data from UWF FA. One of 20 eyes had a lesion on ophthalmoscopy that was missed by imaging.

Conclusion: Ultra-widefield FA using the Optos camera is helpful for the evaluation and management of patients with von Hippel-Lindau disease. The UWF FA with gaze steering appears to detect more hemangioblastomas than ophthalmoscopy and conventional angiography.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Diagnostic Techniques, Ophthalmological*
  • Early Diagnosis
  • Female
  • Fluorescein Angiography / methods*
  • Hemangioblastoma / diagnostic imaging*
  • Hemangioblastoma / etiology
  • Humans
  • Male
  • Middle Aged
  • Retinal Neoplasms / diagnostic imaging*
  • Retinal Neoplasms / etiology
  • Retrospective Studies
  • Young Adult
  • von Hippel-Lindau Disease / complications*