This type of cataract is inherited in an autosomal dominant pattern. The causative mutation is unknown but a presumed locus has been identified at 1pter-p36.13. This type of cataract may be allelic to congenital posterior polar cataract (116600).
Other forms of autosomal dominantly inherited, congenital, progressive lens opacities include congenital cerulean (115660, 601547, 608983, 610202), Coppock-like (604307), and lamellar (116800), cataracts. Due to clinical heterogeneity, it is not always possible to classify specific families based on the appearance and natural history of the lens opacities alone.