Helicoid peripapillary chorioretinal degeneration complicated by choroidal neovascularization

Eur J Ophthalmol. 2016 Feb 15;26(2):e30-1. doi: 10.5301/ejo.5000695.


Purpose: Helicoid peripapillary chorioretinal degeneration (HPCD) is a hereditary disease of the fundus that is characterized by atrophic chorioretinal areas that appear early in life and expand gradually from the optic disc towards the macula and the periphery.

Methods: We describe the case of an elderly man with a known diagnosis of HPCD who developed choroidal neovascular membrane (CNV) in both eyes during the course of the disease.

Results: The patient was treated with intravitreal injection of ranibizumab, to which he had excellent response. The CNV subsided with 2 injections in the right eye and 1 in the left. Two years after the initial diagnosis of CNV in the right eye, visual acuity was 5/10 OD and 9/10 OS.

Conclusions: Helicoid peripapillary chorioretinal degeneration is rarely complicated by CNV as the fundus lacks the trigger factors that would sustain this process. Although rare, HPCD complicated by CNV can be seen bilaterally, but responds well to few ranibizumab injections.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Angiogenesis Inhibitors / therapeutic use
  • Choroidal Neovascularization / diagnosis
  • Choroidal Neovascularization / drug therapy
  • Choroidal Neovascularization / etiology*
  • Corneal Dystrophies, Hereditary / complications*
  • Corneal Dystrophies, Hereditary / diagnosis
  • Corneal Dystrophies, Hereditary / drug therapy
  • Fluorescein Angiography
  • Humans
  • Intravitreal Injections
  • Male
  • Ranibizumab / therapeutic use
  • Retinal Degeneration / complications*
  • Retinal Degeneration / diagnosis
  • Retinal Degeneration / drug therapy
  • Tomography, Optical Coherence
  • Vascular Endothelial Growth Factor A / antagonists & inhibitors
  • Visual Acuity / physiology


  • Angiogenesis Inhibitors
  • VEGFA protein, human
  • Vascular Endothelial Growth Factor A
  • Ranibizumab

Supplementary concepts

  • Sveinsson Chorioretinal Atrophy