๐ What is Aniridia?
Aniridia is a rare congenital eye disorder characterized by the partial or complete absence of the iris.
While the iris controls how much light enters the eye, aniridia affects not only the iris but also multiple ocular structures, making it a panocular disease.
๐ฌ Key Ocular Features
FeatureDescription
| Photophobia | Iris absence leads to light sensitivity |
| Visual delay | Due to corneal opacity, cataract, macular hypoplasia |
| Nystagmus | Pendular type eye movement common in early years |
| Strabismus | Eye misalignment due to visual impairment |
| Glaucoma | Caused by immature iris blocking the drainage angle |
โก๏ธ Glaucoma onset often begins before puberty, requiring regular IOP monitoring
โ Pathophysiology of Glaucoma in Aniridia
- Residual iris tissue causes angle closure
- Aqueous humor outflow blocked → IOP increases
- Leads to optic nerve damage → glaucoma
๐งฌ Associated Systemic Condition: WAGR Syndrome
LetterStands for
| W | Wilms tumor (kidney cancer) |
| A | Aniridia |
| G | Genitourinary abnormalities |
| R | mental Retardation |
โ Summary
- Aniridia is more than just “absence of iris” — it involves:
- Cornea, lens, retina, optic nerve, and potentially systemic abnormalities
- Primary symptoms:
- Light sensitivity, poor vision, nystagmus, and glaucoma
- May be part of WAGR syndrome
- Requires early diagnosis and life-long ophthalmic monitoring
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