Iridocorneal Endothelial Syndrome (ICE) - essential iris atrophy:

63-year-old female with PAS, "iris mass", corectopia, and increased IOP OS

Andrew Doan, M.D., Ph.D., Wallace Alward, MD

February 21, 2005

Chief Complaint: 63-year-old female with PAS, "iris mass", corectopia, and increased IOP OS.

History of Present Illness: Previously healthy female with progressive corectopia and PAS formation OS over the last year. IOP in mid-20s OS. Patient consulted for evaluation of "iris mass".

PMH/FH/POH: non-contributory.

EXAM

• Best corrected visual acuities: 20/20 OD & OS.
• mRx: +1.75+2.00 x 179 OD, +0.75+1.50 x 174 OS
• Pupils: normal OD, see below for OS, no RAPD
• Humphrey's 24-2 VF: full OU
• EOM: normal
• IOP: 15 mmHg OD, 17 mmHg OS
• SLE: Normal OD, see below for OS.
• DFE: Normal OU.
• ON: 0.4 C/D ratio OU (normal).

Figure 1: SLE Photo and Gonioscopy

OS	Gonioscopy OS
Corectopic (i.e., oval) left pupil .	Broad area of peripheral anterior synechiae (PAS) at 6:00 o'clock position with area of iris atrophy and pigmented iris stroma.

Figure 2: SLE Photography of Cornea

Cornea OS	Cornea OS
Cornea with hammered silver appearance of endothelium.	Magnified view: cornea with hammered silver appearance of endothelium.

Figure 3: Specular Microscopy

Endothelium OD Specular Microscopy	Endothelium OS Specular Microscopy
Normal, hexagonal shaped cells. Normal cell count (2380)	Abnormal endothelium with large spaces between cells and low cell count (1353). The color of the cells are also reversed with black surrounded by a white border.

Discussion

Iridocorneal Endothelial Syndrome (ICE) - essential iris atrophy

This patient presented with unilateral corectopia, PAS, hammered silver appearance of the corneal endothelium OS, areas of iris atrophy OS, and history of intraocular pressure spikes OS. The unilaterality of the presentation must include a differential diagnosis of unilateral glaucomas, which can be divided into glaucomas with heterochromia and those without heterochromia.

Specular microscopy demonstrated an abnormal corneal endothelium with low cell counts OS. This case is an example of early essential iris atrophy. This patient has an increased risk of developing glaucoma OS and must be routinely followed with intraocular measurements, optic nerve exam, gonioscopy, and visual field testing.

ICE syndrome is a spectrum of diseases. There are three clinical categories: Chandler's syndrome, Cogan-Reese syndrome, and essential iris atrophy. The unifying abnormality in all three entities is the abnormal, unilateral, hammered silver appearance of the corneal endothelium. Chandler's syndrome presents with only the hammered silver appearance of the corneal endothelium. Cogan-Reese syndrome presents with the corneal findings and pigmented nodules on the iris. Essential iris atrophy presents with the corneal findings and corectopia with developments of stretch and melt holes with disease progression.

Diagnosis: Iridocorneal Endothelial Syndrome (ICE) - essential iris atrophy

EPIDEMIOLOGY incidence: unknown, rare. About 1-2 dozen patients at UIHC. women affected more than men. age: early to middle adulthood genetics: unknown

SIGNS Unilateral glaucoma. All types of ICE have "hammered silver" appearance of corneal endothelium. Chandler’s syndrome: abnormal corneal endothelium. Cogan-Reese syndrome: pigmented nodules on iris. Essential iris atrophy: corectopia, stretch & melt holes. Abnormal corneal endothelial cells act like epithelial cells and may grow into the angle to block trabecular meshwork outflow. PAS formation in the angle.

SYMPTOMS Asymptomatic early in disease. Severe visual loss if associated with untreated glaucoma.

TREATMENT Topical anti-glaucoma medications. Trabeculectomy filtering surgery. Seton or valve surgery. Cyclodestruction of the ciliary body in cases not responding to the above.

Differential Diagnoses

• ICE syndrome
• Axenfeld-Rieger
• Posterior polymorphous corneal dystrophy (PPMD)

Differential Diagnoses of lighter colored iris with glaucoma (heterochromia)

• Chronic iridocyclitis
• Fuchs heterochromic iridocyclitis (10% of cases in the darker colored eye)
• Glaucomatocyclitic crisis

Differential Diagnoses of darker colored iris with glaucoma (heterochromia)

• Cogan-Reese syndrome
• Diffuse iris nevus
• Latanoprost use
• Malignant melanoma of the iris
• Melanomalytic glaucoma
• Neovascular glaucoma
• Neurofibromatosis
• Pigmentary glaucoma
• Siderosis