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Diagnosis of Essential Iris Atrophy in a patient with uncontrolled unilateral
intraocular pressure
Shikha Yadav, O.D.
Ocular Disease Resident
Baltimore Veterans Affairs, MD
This case report describes the diagnosis of essential iris atrophy (ICE syndrome) in a
young patient with corneal edema, progressive corectopia and uncontrolled unilateral
intraocular pressure despite multiple topical medical therapy.
I. Case History
 A 41-year-old white female presents for a glaucoma evaluation with the chief
complaint of blurred vision in her left eye. The patient was previously managed
by her optometrist for increased intraocular pressure of her left eye, and was given
Lumigan and Brimonidine ophthalmic solutions.
 Ocular History: sudden onset of corectopia after child birth, 11 years ago.
 Medical History: seasonal allergies
 Medications: birth control pill, Lumigan and Brimonidine.
II. Pertinent findings
 Clinical findings
o VA: OD CC 20/15-, OS CC 20/20o EOM: normal
o CVF: normal
o Pupils: normal OD, corectopia OS, (-) RAPD OU
o SLE: Normal OU, unless noted otherwise below.
o Cornea: fine, beaten metal appearance of corneal endothelium and mild
corneal edema
o Iris: subtle iris transillumination defects with localized iris-corneal
adhesion OS
o IOP: 13 OD, 40 OS
o DFE: Normal OU
o Optic Nerve: 0.3 C/D OD; 0.6 C/D OS
o NFL grossly intact OU
 External photos
 Gonioscopy
 Specular Microscopy
o OD: Normal, hexagonal shaped cells. Normal cell count (2684)
o OS: Abormal endothelium with large spaces between cells and low cell
count (1899). The color of the cells are reversed with black surrounded by
a white border.
 Fundus photos
III. Differential diagnosis
 Essential Iris Atrophy
 Chandler’s syndrome
 Cogan-Reese syndrome
 Axenfeld-Reiger Syndrome
Posterior polymorphous corneal dystrophy (PPMD)
Fuchs endothelial dystrophy
IV. Diagnosis and discussion
 Essential iris atrophy
o Anterior segment presentation
o Ruling out differentials with specular microscopy and gonioscopy
o Medical and surgical techniques to control intraocular pressure.
 Proposed viral etiology for ICE syndromes
 Endothelial keratoplasty for corneal edema
 Prognosis of long term corneal and IOP management
V. Treatment
 Initial management
o Continue Lumigan qhs OS and Brimonidine BID OS. Add Combigan BID
OS. Target pressure set to <21.
o RTC 1 month for IOP check
 Follow-up examinations:
o IOP reduced to 22 in OS but diurnal fluctuation considered.
o Patient was followed every 6 weeks for IOP checks.
o 3 months after initial presentation, IOP spiked to 40mmHg with good
medication compliance. Pilocarpine 1% TID was added to current topical
o IOP remained below target pressure for another 4 months.
o 7 months after initial presentation, patient’s IOP spiked to 37 mmHg and
major change in optic nerve appearance was noted.
o Trabeculotomy was performed in OS.
 Final post-op: 5 months post Trabeculotomy
o Vision: 20/20 OS
o IOP: 12mmHg on Lumigan qpm OS
VI. Conclusion
 Clinical pearls of essential iris atrophy
o Always consider ICE syndrome on your list of differentials for
unilateral glaucoma
o Know when to consider surgical management for uncontrolled IOP
 Bibliography