Essential iris atrophy

A rare eye disorder affecting the iris

Essential iris atrophy
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Progressive iris thinning, corectopia, polycoria
Complications	Glaucoma, corneal edema
Onset	Adulthood
Duration	Chronic
Types	N/A
Causes	Unknown
Risks
Diagnosis	Clinical examination, slit-lamp biomicroscopy
Differential diagnosis	N/A
Prevention	N/A
Treatment	Management of complications, surgical intervention
Medication	N/A
Prognosis	Variable
Frequency	N/A
Deaths	N/A

Essential iris atrophy is a rare ocular condition characterized by progressive thinning and degeneration of the iris, the colored part of the eye. It is considered a part of the iridocorneal endothelial syndrome (ICE syndrome), which also includes Chandler syndrome and Cogan-Reese syndrome.

Pathophysiology

The exact cause of essential iris atrophy is unknown, but it is believed to involve abnormal proliferation of the corneal endothelium, which leads to changes in the iris and anterior chamber angle. This abnormal endothelial layer can migrate across the trabecular meshwork, leading to glaucoma.

Clinical Features

Patients with essential iris atrophy typically present with:

• Iris thinning: The iris progressively thins, leading to a change in eye color and appearance.
• Corectopia: Displacement of the pupil from its normal central position.
• Polycoria: The presence of multiple holes or openings in the iris.
• Glaucoma: Increased intraocular pressure due to angle closure or trabecular meshwork dysfunction.
• Corneal edema: Swelling of the cornea due to endothelial dysfunction.

Diagnosis

Diagnosis of essential iris atrophy is primarily clinical, based on the characteristic features observed during a slit-lamp examination. Additional tests may include:

• Gonioscopy: To assess the angle of the anterior chamber.
• Specular microscopy: To evaluate the corneal endothelium.
• Optical coherence tomography (OCT): To assess the structure of the iris and anterior segment.

Management

There is no cure for essential iris atrophy, and treatment focuses on managing complications:

• Glaucoma management: Medications, laser therapy, or surgical procedures to lower intraocular pressure.
• Corneal edema treatment: Hypertonic saline drops or ointments, and in severe cases, corneal transplantation.
• Surgical intervention: In some cases, surgical procedures may be necessary to correct iris abnormalities or manage glaucoma.

Prognosis

The prognosis for essential iris atrophy varies. While some patients may maintain good vision with appropriate management, others may experience significant visual impairment due to complications such as glaucoma or corneal decompensation.

Also see

• Iridocorneal endothelial syndrome
• Chandler syndrome
• Cogan-Reese syndrome
• Glaucoma
• Corneal edema

External

• Age-Related Macular Degeneration

• Amblyopia (Lazy Eye)

• Anophthalmia and Microphthalmia

• Astigmatism

B

• Behçet's Disease* Bietti's Crystalline Dystrophy

• Blepharitis* Blepharospasm

C

• Cataracts

• Coloboma

• Color Blindness

• Corneal Conditions

D

• Diabetic Retinopathy

• Dry Eye

F

• Floaters

G

• Glaucoma

I

• Idiopathic Intracranial Hypertension

L

• Low Vision

M

• Macular Edema

• Macular Hole

• Macular Pucker

P

• Pink Eye

R

• Rare Diseases

• Refractive Errors

• Retinal Detachment

• Retinitis Pigmentosa

• Retinoblastoma

• Retinopathy of Prematurity

S

• Stargardt Disease

U

• Usher Syndrome

• Uveitis

V

• Vitreous Detachment