A class of congenital retinal dystrophies where a baby is born without vision or develops severe loss of vision at an early age. It is marked by a sharp decrease in visual acuity and is associated with nystagmus, high hyperopia, keratoglobus, photophobia, and almost zero or reduced pupillary responses. The affected child pokes, presses, or rubs the eyes with a finger or knuckle (Franceschetti’s oculo-digital sign).

Also Known As

  • Aquula
  • Crystalline Lens