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Congenital primary aphakia

ORPHA:83461· ICD-10 Q12.3

Definition

A rare developmental defect during embryogenesis characterised by an absence of the lens. CPAK can be associated with variable secondary ocular defects.

Prevalence
1-5 / 10 000
Inheritance
Autosomal recessive
Age of onset
Antenatal, Infancy, Neonatal