Retinal degeneration in children: Dark adapted visual threshold and arteriolar diameter

To assess the condition of the retina in children with retinal degeneration due to Bardet-Biedl syndrome (BBS, n = 41), Leber congenital amaurosis (LCA, n = 31), or Usher syndrome (USH, n = 13), the dark adapted visual threshold (DAT) and arteriolar diameters were measured. Compared to controls, the initial DATs of nearly all (83/85) were significantly elevated, and in 26/62 with serial DATs, significant progressive elevation occurred. Arteriolar diameters were significantly attenuated and narrowed with age in BBS and USH, but not LCA. Higher DATs were associated with narrower arterioles. Such non-invasive procedures can document the natural history of these retinal diseases and have the potential to assess response to future treatment.