Aniridia, WAGR syndrome, nystagmus, glaucoma, strabismus

👁 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.

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🔬 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

➡️ Glaucoma onset often begins before puberty, requiring regular IOP monitoring

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⚠ Pathophysiology of Glaucoma in Aniridia

• Residual iris tissue causes angle closure
• Aqueous humor outflow blocked → IOP increases
• Leads to optic nerve damage → glaucoma

 

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🧬 Associated Systemic Condition: WAGR Syndrome

LetterStands for

W	Wilms tumor (kidney cancer)
A	Aniridia
G	Genitourinary abnormalities
R	mental Retardation

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✅ 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