lamellar cataract; zonular cataract (includes Marner type cataract)

Epidemiology

• common type of infantile cataract

Pathology

• concentric opacities, broad or narrow, usually consisting of powdery white dots affecting one lamella or zonule of an otherwise clear lens
• the opacity may be so dense as to render the entire central region of the lens completely opaque, or so translucent that vision is hardly affected (type 2)
• generally do not involve the embryonic nucleus, though sometimes they involve the fetal nucleus (type 2)
• usually sharply separated from a clear cortex; however, they may have projections from their outer edges known as riders or spokes (type 2)

Genetics

• autosomal dominant
• associated with defects in HSF4
• associated with defects in CRYBA1
• associate with defects in CRYBA4 (type 2)

Clinical manifestations

• infantile cataract
• finger malformation is observed in some kindreds

More general terms

• autosomal dominant cataracts

Database

• OMIM: https://mirror.omim.org/entry/116800
• OMIM: https://mirror.omim.org/entry/600881
• OMIM: https://mirror.omim.org/entry/610425