Congenital aphakia

This code describes a rare birth defect where the lens of the eye is completely absent from birth. This condition results in severe visual impairment due to the eye's inability to focus light onto the retina. It can affect one or both eyes.

Use this code when documentation explicitly states congenital absence of the crystalline lens. This diagnosis is typically made in infancy or early childhood following ophthalmic examination. Supporting documentation will include findings such as aphakia noted during slit lamp examination or ultrasound.

• Differentiate from acquired aphakia (H27.0-), which occurs after cataract extraction or trauma.
• Specify laterality (e.g., right, left, bilateral) if documented, using the appropriate subcategory.
• Code any associated congenital anomalies of the eye or other systems separately.