Helicoid peripapillary chorioretinal degeneration

What is Helicoid peripapillary chorioretinal degeneration?

Helicoid peripapillary chorioretinal degeneration is a rare autosomal dominantly inherited chorioretinal degeneration disease, presenting at birth or infancy, characterized by progressive bilateral retinal and choroidal atrophy, appearing as lesions on the optic nerve and peripheral ocular fundus and leading to central vision loss. Congenital anterior polar cataracts are sometimes associated with this disease.

Other condition names

  • Atrophia areata
  • SCRA
  • Sveinsson chorioretinal atrophy

Inheritance type

Autosomal dominant


  • Worldwide: <1 in 1,000 000
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.