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Ophthalmic Images
September 8, 2016

Spontaneous Devascularization and Detachment of Optic Nerve Hemangioblastoma in a Patient With Von Hippel-Lindau Disease

Author Affiliations
  • 1Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
JAMA Ophthalmol. 2016;134(9):e161119. doi:10.1001/jamaophthalmol.2016.1119

A woman in her 30s with known von Hippel-Lindau disease was evaluated. Visual acuity was 20/20 OU. The right eye was normal while the left eye showed optic nerve hemangioblastoma (Figure, A, blue arrowhead) with a fine feeding artery (white arrowhead) and draining vein (Figure, A, left). Optical coherence tomography documented an epipapillary location with thin attachment to the optic nerve. At 1-year follow-up without intervention, vision was stable and there was spontaneous devascularization and detachment of the optic nerve hemangioblastoma (blue arrowhead), leaving the sclerotic draining vein (black arrowhead) without a feeding artery (Figure, B). Optical coherence tomography showed a detached tumor. Optic nerve hemangioblastoma may be difficult to treat when balancing potentially visually damaging therapies with tumor-related morbidity.1 While 1-year follow-up is available in this case and incidence of spontaneous regression is unknown, the spontaneous devascularization and detachment of optic nerve hemangioblastoma in this case suggest that careful observation sometimes may be warranted in patients with good baseline vision.24

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