Cerulean cataract, first described by Vogt (1922), is an autosomal dominant, early-onset, bilateral cataract with complete penetrance. Newborns appear asymptomatic until the age of 18 to 24 months, at which time they can be clinically diagnosed by slit-lamp ... Cerulean cataract, first described by Vogt (1922), is an autosomal dominant, early-onset, bilateral cataract with complete penetrance. Newborns appear asymptomatic until the age of 18 to 24 months, at which time they can be clinically diagnosed by slit-lamp examination through the appearance of tiny blue or white opacities that form first in the superficial layers of the fetal lens nucleus. The opacities progress throughout the adult lens nucleus and the cortex, forming concentric layers, with central lesions oriented radially. Histologically the lesions appear to be tapered cavities between lens fibers. Progression of the cataract is slow, such that patients may have lens extractions between the ages of 16 and 35 years (Armitage et al., 1995). The preferred title/symbol of this entry was formerly 'Cataract, Congenital, Cerulean Type, 1; CCA1.'
Kivlin et al. (1985) reported affected members of a large family segregating 'juvenile' cerulean cataract. In a follow-up of this family, Armitage et al. (1995) characterized the cataract as early in onset and progressive. Affected newborns appeared asymptomatic ... Kivlin et al. (1985) reported affected members of a large family segregating 'juvenile' cerulean cataract. In a follow-up of this family, Armitage et al. (1995) characterized the cataract as early in onset and progressive. Affected newborns appeared asymptomatic until the age of 18 to 24 months, at which time they could be clinically diagnosed by slit-lamp examination through the appearance of tiny blue or white opacities that formed first in the superficial layers of the fetal lens nucleus. Armitage et al. (1995) suggested that cerulean cataracts should be classified as a developmental cataract rather than a congenital cataract. The opacities progressed throughout the adult lens nucleus and the cortex, forming concentric layers, with central lesions oriented radially.