E70.311

HCC Category Mapping

What This Code Means

A rare inherited condition affecting only the eyes where melanin production is reduced, causing vision problems and light sensitivity, inherited when both parents carry the gene.

Coding Tips

• •This autosomal recessive form can affect both males and females equally
• •Document associated ocular manifestations such as reduced pigmentation of the iris or optic nerve hypoplasia

Clinical Significance

Autosomal recessive ocular albinism is a rare inherited condition where reduced melanin production is confined to the eyes, affecting both males and females equally when both parents carry the gene mutation. Patients experience decreased visual acuity, nystagmus, iris transillumination, and photophobia, though to a generally lesser degree than oculocutaneous albinism. Skin and hair pigmentation remain relatively normal.

Documentation Requirements

• ✓Documentation should include genetic testing results confirming autosomal recessive inheritance, detailed ophthalmologic examination (visual acuity, iris transillumination, fundoscopy, optical coherence tomography for foveal hypoplasia), and family history.
• ✓Normal or near-normal skin and hair pigmentation should be noted to distinguish from oculocutaneous forms.
• ✓Document all visual impairment management including corrective lenses and low-vision aids.

Commonly Confused Codes