iridocorneal endothelial (ice) syndrome

New England Eye Center Grand Rounds

Ihuoma U. Alozie-Uddoh, M.D.

May 24, 2001


Case Presentation:• 39 year old male presents with a three year history

of decreased and fluctuating vision in his left eye.• The patient states that his vision is very blurred in

the morning but gets progressively better during the day.

• The patient also reports increasing eye pain and photophobia in the left eye especially over the past six months.

New England Eye CenterGrand Rounds

• Case Presentation:

• Past Ocular History– Nine years ago patient experienced recurrent

foreign body sensation and light sensitivity OS.– The patient has also noted that his left pupil has

been changing shape over the past 3-4years.– There was no history of trauma associated with

his presenting symptoms.

New England Eye CenterGrand Rounds

• Case Presentation:• Past Medical History: Unremarkable • Family History: Unremarkable• Medications: None• Allergies: None


• Case Presentation:• On presentation, BCVA 20/20 OD and 20/60 OS. • Pupils were 4mm round OD, reactive to light and

accommodation with no relative afferent pupillary defect OU. OS pupil was eccentric and oval

• Intraocular pressures were 15mmHg OD and 24mmHg OS at 3:00pm.

• SLE – OD within normal limits– OS Pupil oval and eccentric. There was visible beaten metal

appearance to cornea OS with endothelial pigment.

Figure 1. Anterior segment photograph and Right and Left Eyes

OD OS


• Case Presentation:• Gonioscopy

– OD: angle open to ciliary body band – OS :There was broad based peripheral anterior

synechiae to schwalbes line at approximately the 10 o’clock to 2:30 o’clock hours causing angle closure in these areas.

• Dilated fundus examination– Healthy optic nerves OU. Normal macula vessels and

periphery OU.

Figure 2. Goniophotograph Left Eye


• The differential diagnosis of glaucoma associated with disorders of the cornea and iris is:

• Corneal endothelial disorders – ICE syndrome

• Essential iris atropthy• Chandlers syndrome• Iris nevus syndrome

– Fuchs endothelial dystrophy – Posterior polymorphous dystrophy

• Dissolution of the iris– Axenfeld-Reiger syndrome– Iridoschisis– aniridia

• Nodular lesions of the iris – Iris nevus syndrome– Iris melanomas– Nodular inflammatory disorders such as sarcoidosis

• Iridoschisis


• Case Presentation:• Based on the clinical presentation, the patient was

diagnosed with iridocorneal endothelial syndrome – essential iris atrophy.

• Because intraocular pressure was elevated in the left eye, optic nerve photographs and a baseline visual field were obtained. These were normal.

• Specular microscopy was also obtained which was normal in the right eye, of poor quality in the left eye with a few notable ICE cells.

Figure 3. Specular microscopy photographs of Right and Left Eyes.

OD OS

Figure 4. Optic nerve photographs of Right and Left Eyes.

OD OS

Figure 5. Visual field photographs of Right and Left Eyes.

OS OD


• Case Presentation• The patient was placed on muro128 ointment at

nightime and muro 128 drops during the day and asked to follow up in a week for IOP evaluation.

• On follow up, Va OD 20 /20 ; OS 20/30. IOP OD 13mm Hg; OS 12mmHg

• The fluctuating visual acuity and fluctuating IOP was attrributed to corneal edema and the patient was asked to continue muro and to follow up in 6-8 weeks.

New England Eye Center Grand Rounds.

• Discussion• Iridocorneal endothelial (ICE) syndrome is a spectrum of

disease that is characterized by a primary corneal endothelial abnormality.

• Principal features of this disease include– distortion and atrophy of the iris

– corneal edema caused by dystrophy of the corneal endothelium

– peripheral anterior synechiae of the iris to the cornea at or anterior to schwalbes line.

• Glaucoma may also be associated with this disease


• Discussion• ICE syndrome is typically unilateral, more

common in women and usually becomes clinically manifest in early to middle adulthood

• Presenting symptoms include – Visual disturbance- halos or photophobia occasionally

intermittent and more prominent in the morning– Change in the iris which may be described as a

distorted or second pupil– Pain usually occurring later in the disease after visual

change and iris change have occurred


• Discussion• Three clinical variations within the ICE syndrome

can be distinguished based on changes in the iris– Chandlers syndrome– Essential Iris atrophy– Iris nevus syndrome

• In all three forms of the disease, a hammered silver appearance of the posterior cornea may be observed when viewed by slit lamp biomicroscopy


• Discussion• The iris changes in essential iris atrophy usually occur late

in the disease.• Corectopia is almost always toward a prominent peripheral

anterior synechia associated with ectropion uvea in the quadrant corresponding to the direction of pupillary displacement

• The iris on the side opposite to the direction of pupillary distortion is stretched and stromal atrophy develops.

• In addition, there may be dissolution of pigment epithelium forming iris holes.


• Discussion• Histologically, a membrane which resembles

Descemet’s membrane may extend over the angle and onto portions of the iris.

• On specular microscopy, there is diffuse abnormality of the corneal endothelial cells with pleomorphism in size and shape, dark areas within the cells and loss of clear hexagonal margins (ICE cells).

• Decreased endothelial cell counts and cellular pleomorphism may also be observed in fellow asymptomatic eyes of patients with ICE syndrome


• Discussion• Glaucoma and corneal decompensation are serious

sequelae of ICE syndrome.• Glaucoma results from obstruction of the anterior

chamber angle with the abnormal membrane and angle closure by the PAS.

• The extent of PAS does not correlate with the degree of reduction in outflow facility elevation of IOP in ICE syndrome patients because additional angle obstruction results from the unseen ICE membrane covering the anterior chamber angle.


• Discussion• The prevalence of glaucoma in the ICE syndrome

varies from 46% to 82% with younger patients being more commonly affected.

• Patients with ICE syndrome may require treatment for corneal edema, the associated glaucoma or both

• Control of corneal edema can be achieved by– lowering of IOP. – Additional use of hypertonic saline solutions– soft contact lenses


• Discussion• In the early stages of the disease, glaucoma can be

controlled medically with drugs that reduce aqueous production.

• Long term medical treatment of glaucoma from ICE syndrome is generally ineffective .

• When IOP can no longer be controlled medically, surgical intervention is required.


• Discussion • Surgical procedures

– Trabeculectomy with antimetabolite– Aqueous tube shunts– Laser trabeculoplasty ineffective

• Usually performed after medical therapy has failed and also have variable success.– Laganowski et al total 22 pts at 1 and 5 yrs 60% and 20%– Wright et al with 5FU 80% (4/6) at 54 months follow up– Lanzl et al with MMC (8/10) at mean 14.9 months


• Discussion• The high failure rate is attributed partly to the young

age of the patients and also to the progressive nature of the disease with obstruction of the filtration site with synechiae and the abnormal membrane.

• Failure of filtering procedures may also be attributed to an aggressive inflammatory response in ICE patients. Adjunctive antimetobolite use is highly recommended.

• When there is failure due to obstruction of ostium at filtration sites, a yag membranectomy may be performed.


• Discussion• In order to minimize tube occlusion by iris or ICE

membrane AND/OR • Minimize membrane induced anterior migration of tube in

tube shunt surgery– Tube cut slightly longer to facilitate tube repositioning– Placement through pars plana if patient is pseudophakic

and has high PAS– Placing tube tip through surgical iridectomy into space

posterior to iris plane


• Summary

• 39 year old male with essential iris atrophy that presents with fluctuating intraocular pressure and visual acuity from corneal changes. The patient has been managed on hypertonic saline drops with no further IOP spikes but continued fluctuating vision.

New England Eye Center Grand Rounds.

• References:• Kidd M, Hetherington et al. Surgical Results in Iridocorneal Endothelial

Syndrome. Arch Ophthalmol. Feb 1988 106: 199-201.• Kim D, Ioanneis M et al. Long-term outcome of Aqueous Shunt Surgery in

ten Patients with Iridocorneal Endothelial syndrome. Ophthalmol. May 1999 106(5):1030-1034.

• Lanzel I, Wilson RP et al. Outcome of Trabeculectomy with Mitomycin -C in the Iridocorneal Endothelial Syndrome. Ophthalmol Feb 2000;107(2) 295-297.

• Laganowsky H, Kerr-Muir et al. Glaucoma and the Iridocorneal endothelial syndrome. March 1992. Arch Ophthalmol. 110 346-350.

• Shields MB. Progressive Essential Iris Atrophy, Chandler’s Syndrome, and the Iris Nevus (Cogan-Reese) Syndrome : a spectrum of Disease. Surv Ophthalmol. Jul-Aug 1979 ;24(1)3-20.

• Shields MB. Textbook of Glaucoma Fourth Edition . Williams ans Wilkins pp226-236.

iridocorneal endothelial (ice) syndrome

Health & Medicine

right eye

left pupil

increasing eye pain

cornea os

mmhg os

clinical presentation

mmhg od

iop od