Iridocorneal endothelial syndrome is a rare eye disease in which abnormal cells from the inner layer of the cornea grow into the iris and out to the eye’s drainage angle. That process changes the look of the pupil, causes the cornea to swell, and also interferes with the normal outflow of fluid from the eye. As pressure increases some patients may get secondary glaucoma.
Although not a common diagnosis, early recognition of iridocorneal endothelial syndrome is important as persistent corneal swelling and uncontrolled eye pressure may progressive[ly] affect vision. The disease usually presents in one eye and what is reported to be a higher incidence among women in early to middle adulthood.
People report to look up what is ICE syndrome eye which is indicated when an ophthalmologist notes an atypical pupil, unexplained corneal swelling or high pressure in one eye. ICE is an acronym for iridocorneal endothelial. Also unlike many hereditary corneal diseases this is an acquired as opposed to a congenital condition.
According to the National Eye Institute report which details the primary features of ICE syndrome they are iris and pupil shape changes, cornea swelling and glaucoma. Iridocorneal Endothelial Syndrome?
The corneal endothelium is a layer of adapted cells at the bottom of the cornea’s which is the inner surface of the cornea. Also this tissue’ role is to put in charge of the fluid levels within the corneal tissue. In order to have clarity which is needed for light to enter the eye and to focus properly, the cornea should be transparent.
In their iridocorneal endothelial syndrome, endothelial cells start to act abnormally. They begin to multiply or to migrate out of their normal position and may also spread to the drainage angle and the iris. The atypical cells may form a membrane which over time will shrink; this in turn will pull at the iris and affect the flow of fluid out of the eye.
A basic description of what is ICE syndrome eye is that it is a group of very related conditions which affect the corneal endothelium, iris and drainage angle. We see in Chandler syndrome, in essential iris atrophy and in Cogan-Reese syndrome.
Among rare eye conditions in humans,ICE syndrome stands out as one in which a corneal endothelial issue may in time cause changes in the iris, corneal clouding and a result of raised intraocular pressure which in turn damages the optic nerve. Does CE Syndrome Affect the Eye?
The front of the eye has a clear fluid which we call aqueous humour. This fluid usually drains through a structure named the trabecular meshwork which is at the angle where the cornea and iris meet.
In the case of iridocorneal endothelial syndrome, abnormally affected endothelial cells may grow over the trabecular meshwork. This may in turn block fluid outflow which in turn may raise intraocular pressure. Also the abnormal membrane may shrink which in turn pulls the iris into the drainage angle and may produce what is known as peripheral anterior synechiae.
At the same time the corneal endothelial cells may not be able to keep the proper fluid balance. That in turn causes buildup of extra fluid within the cornea which results in corneal oedema or swelling. The person may report foggy vision, starburst around lights or seeing colored circles around them.
At the same time the iridocorneal endothelial syndrome may not be able to keep the proper fluid balance. That in turn causes buildup of extra fluid within the cornea which results in corneal oedema or swelling. The person may report foggy vision, starburst around lights or seeing colored circles around them.
Types of Iridocorneal Endothelial Syndrome
The three types of ICE syndrome which are recognized may have the same basic cause but each type presents with corneal, iridial and trabecular meshwork involvement in a different way.
| Type of ICE Syndrome | Common Clinical Features | Possible Effect on Vision |
| Chandler syndrome | Corneal swelling is usually prominent, while iris changes may be comparatively mild | Hazy vision, glare, halos and discomfort caused by corneal oedema |
| Essential iris atrophy | Progressive thinning of the iris, pupil displacement and formation of iris holes | Secondary glaucoma, pupil distortion and reduced visual clarity |
| Cogan-Reese syndrome | Pigmented spots, nodules or a diffuse abnormal appearance on the iris | Raised eye pressure, iris changes and gradual visual disturbance |
Chandler Syndrome
Chandler syndrome presents with marked corneal swelling. Iris changes in this case are of a lesser degree as compared to other types. Patients may have blurred vision which in particular happens post awakening which is a result of fluid that accumulates in the cornea at night.
Essential Iris Atrophy
Essential and progressive iris atrophy mainly affects iris structure. Pupils may shift out of place and areas of thin out to full thickness holes. Also the abnormal tissue may close off parts of the drainage angle which in turn causes secondary glaucoma.
Cogan-Reese Syndrome
Cogan-Reese syndrome which may present as pigmented nodules or spots in the iris. Also there is a change which causes the iris to have a dark, non-uniform color like that of an iris nevus. Eye pressure may also be increased and cornea swelling may present also.
These differences, which present in iridocorneal endothelial syndrome,are what ophthalmologists use to put patients into different categories, though some patients may have similar features.what is ICE syndrome eye, as it turns out does not have a single picture, we see that point out. In one patient the primary issue may be corneal swelling and in another the issue may first present as glaucoma or an abnormal pupil.
What Causes Iridocorneal Endothelial Syndrome?
The cause of iridocorneal endothelial syndrome has not been determined. We know what the abnormal endothelial cells do, but we are still to identify what is the initial event which triggers the cell change.
Some research has looked at the question of a viral cause in which herpes simplex virus is a primary player in the behavior of corneal endothelial cells. This is a put forth theory which has not been borne out in all patients.
ICE syndrome does not present as a simple inherited disease. Most patients report no clear family history and the condition usually presents later in which birth. This is also a reason why iridocorneal endothelial syndrome is put forward with other rare eye conditions in humans. Diagnosis is mainly based on characteristic eye features instead of a cause specific blood or genetic test.
Symptoms of ICE Syndrome
Symptoms present in a great variation based on which area of the eye is most affected; the cornea, the iris or the drainage angle. In some patients only minor changes are noticed, in others there is progressive visual impairment
Corneal swelling may produce foggy or fluctuating vision. In the morning time vision may sometimes go to a bad level due to the cornea filling up with more fluid which is trapped under closed eyelids. Also there may be experience of glare and colored halos around lights.
Changes of the iris which may present as a stretched, displaced or irregular pupil. In essential iris atrophy small holes may appear. Cogan-Reese syndrome may present with pigmented spots or nodules on the iris.
When iridocorneal endothelial syndrome causes a large increase in intraocular pressure the patient may present with pain in the eye, redness, headaches, seeing halos or loss of vision. Also some cases of glaucoma related optic nerve damage may not present at first with any obvious symptoms.
For those of you that are researching what is ICE syndrome eye,you will note that not all symptoms present in a mirror. Some of the more important changes can only be identified through a detailed eye exam.
Persistent one sided blurred vision, a new note of abnormal pupil, or very high intraocular pressure should be referred to a specialist eye care facility instead of using over the counter eye drops.
How Is ICE Syndrome Diagnosed?
Diagnosis starts out with a very in depth review of the patient’s symptoms and medical history. The ophthalmologist then does an exam of the affected eye which includes the cornea, iris, pupil, drainage angle, intraocular pressure and optic nerve.
A slit lamp exam may present with corneal swelling, abnormal endothelial changes, iris thinning or pupil displacement. The endothelial surface at times has an irregular appearance which we describe as that of beaten bronze or hammered silver.
Tonometry is used to measure intraocular pressure. With gonioscopy ophthalmologists are able to see the drainage angle and also identify abnormal membrane growth or adhesions between the iris and adjacent structures.
Specular microscopy provides in depth images of the cornea’s endothelial cells. In ICE syndrome those cells may present larger than normal, irregular or different shapes. In vivo confocal microscopy reports also at the cellular level.
| Examination | What the Examination Helps Assess |
| Slit-lamp examination | Corneal swelling, endothelial changes, iris thinning and pupil distortion |
| Tonometry | Intraocular pressure and the risk of secondary glaucoma |
| Gonioscopy | Drainage-angle obstruction and peripheral anterior synechiae |
| Specular microscopy | Size, shape and arrangement of corneal endothelial cells |
| Confocal microscopy | Detailed cellular changes within the cornea |
| OCT and visual-field testing | Optic nerve damage and glaucoma-related loss of peripheral vision |
| Ultrasound biomicroscopy | Anterior chamber and drainage-angle structures when direct viewing is difficult |
A close evaluation is required in which we must note that iridocorneal endothelial syndrome may present similarly to other ocular issues. The ophthalmologist may have to tell which is which from Fuchs endothelial corneal dystrophy, posterior polymorphous corneal dystrophy, Axenfeld-Rieger syndrome, aniridia and certain iris tumors.
Some of them are also included as rare eye conditions in humans,we see however that their age of onset, inheritance patterns and which eye they affect may be different.
Treatment for Iridocorneal Endothelial Syndrome
At present there is no cure which fully reverses the abnormal behavior of the endothelial cells. In the case of iridocorneal endothelial syndrome we treat to reduce eye pressure, to decrease corneal swelling and to protect what vision the patient has.
The care plan is dependent upon if the issue is glaucoma, corneal decompensation or a mix of both.
Treatment for Secondary Glaucoma
Pressure reducing eye drops are a treatment option for ICE which causes secondary glaucoma. Which drugs are given is based on the patient’s eye pressure, the state of the optic nerve and the health of the eye as a whole.
When medicines don’t control the pressure enough, glaucoma surgery may be done. ICE related glaucoma at times may be a tough case to handle because the abnormal membrane still may play a role in affecting the drainage angle.
Patients may look up more info on glaucoma treatment options but the right medicine, laser procedure or surgery has to be chosen based on the results of an individual exam.
Treatment for Corneal Swelling
Mild corneal swelling at first may be treated with medications which reduce fluid build up and improve comfort. For an irritated eye surface we may use lubricating drops.
When endothelial health deteriorates greatly the cornea may stay cloudy or it may become painful. In such a case endothelial keratoplasty or some other type of corneal transplant may be performed.
The National Eye Institute reports that in severe cases of ICE syndrome which may include extensive corneal damage we may see corneal transplants. For iridocorneal endothelial syndrome which may affect the cornea and the eye’s internal pressure (glaucoma) we may see involvement of the cornea specialist and the glaucoma specialist. This team approach is good because the patient may need us to watch at the same time for changes in corneal clarity as well as in the health of the optic nerve.
Can ICE Syndrome Cause Permanent Vision Loss?
Yes, in some cases iridocorneal endothelial syndrome may cause permanent vision loss when corneal swelling is persistent or glaucoma damages the optic nerve. Also the rate of progression varies between patients.
Some patients do well for a long time with routine check ups and medication. Others may require surgery to reduce eye pressure or to restore corneal clarity.
Glaucoma related injury is also of great importance which is because once retinal nerve tissue is lost it usually does not regenerate. Also cornea clouding may play a role in blocking the entry of light into the eye.
Early assessment is a good tool but it doesn’t guarantee that the disease will stop from progressing. What it does do is allow the ophthalmologist to identify pressure changes, corneal degeneration and optic nerve damage before they progress to a more advanced stage.
In certain rare eye conditions in humans, ICE syndrome is a case in point which shows that regular follow up is critical even for patients that present with mild symptoms.
Is ICE Syndrome the Same as Aniridia?
No.Iridocorneal endothelial syndrome is also a separate condition from aniridia.
ICE syndrome is mostly an acquired condition which presents with affected one eye at a time and which may cause progressive thin or abnormal iris structure. Aniridia is a disorder which sees the iris to be only partially or fully absent. It usually affects both eyes and at the same time we see that the PAX6 gene is involved in many of these cases
People have some use of vision with aniridia but may have reduced visual acuity which in turn is a result of foveal underdevelopment, cataract, glaucoma, uncontrolled eye movements or corneal disease. ion also plays a role in the discussion of rare eye conditions in humans.
Development in adulthood of an irregular pupil or iris defect is a feature of ICE syndrome which is different from the at birth presentation of an absent or underdeveloped iris.
Living With ICE Syndrome
A diagnosis of iridocorneal endothelial syndrome will require at times long term follow up. Follow up tests may include pressure assessment, study of the cornea, optic nerve imaging and visual field testing.
Patients are to use prescribed eye drops regularly and report to follow up even when vision is stable. Eye pressure may change without notice and corneal swelling may progress gradually.
Anyone that is a researcher into what is ICE syndrome eye may find themselves concerned as the condition is rare. Ask the ophthalmologist which ICE variant is present, if glaucoma has developed and how the corneal endothelium is doing which will in turn make the diagnosis easier to understand.
It is also useful to have care at an eye specialist hospital which does corneal imaging, glaucoma evaluation and surgery as a coordinated service when required.
When Should You Consult an Ophthalmologist?
In the event of persistent morning blur in one eye, repeatable halos, unexplained pain, a changed pupil in the eye, or high intraocular pressure an ophthalmic assessment should be done.
These symptoms do not in fact point to iridocorneal endothelial syndrome, as they may also present in a number of other ophthalmic conditions. A full exam is needed to determine the cause.
Urgent medical care is required when a person has severe eye pain, sudden vision loss, a red eye that is painful to the touch, nausea or halos which accompany a very painful headache. These symptoms present when eye pressure has increased quickly and should not be treated at home.
An ophthalmologist will determine which of the following is true for the patient ICE related monitoring, glaucoma treatment, infection management or care for a corneal ulcer.
Conclusion
Iridocorneal endothelial syndrome is a rare yet important disease which affects the cornea, iris and drainage angle. It causes corneal swelling, pupil distortion and secondary glaucoma. Also it mostly presents in one eye and may progress slowly which may cause its early signs to go unnoticed.
Understanding of what is ICE syndrome eye should prompt for timely medical evaluation which in turn is better than creating unnecessary alarm. A detailed eye exam may report abnormal endothelial cells, drainage angle changes, corneal swelling and also may point out to possible optic nerve damage.
Regular evaluation, proper pressure management and treatment of corneal complications may help preserve useful vision. Any person which reports persistent one sided blur, unexplained eye pressure or a changing pupil should see an ophthalmologist.
Frequently Asked Questions
The cause is unknown. What we do see is that abnormal growth and migration of corneal endothelial cells is at the core of the issue. Also studied was a possible viral trigger which did not play out to be proven.
ICE is a term which stands for Iridocorneal Endothelial. This term describes a condition which affects the corneal endothelium, iris and eye’s drainage angle. This is the basic explanation of what is ICE syndrome eye.
There is no single rare eye condition which is recognized worldwide. Some genetic and developmental eye diseases present in only a few cases which report very variable rates of rarity in different populations.
Reference Links
- National Eye Institute — Other Types of Corneal Disease:
https://www.nei.nih.gov/eye-health-information/eye-conditions-and-diseases/corneal-conditions/other-types-corneal-disease - NCBI Bookshelf — Iridocorneal Endothelial Syndrome:
https://www.ncbi.nlm.nih.gov/books/NBK594227/ - NCBI Medical Subject Headings — Iridocorneal Endothelial Syndrome:
https://www.ncbi.nlm.nih.gov/mesh/68057129 - NIH Genetic and Rare Diseases Information Center — Isolated Aniridia:
https://rarediseases.info.nih.gov/diseases/5816/isolated-aniridia - MedlinePlus Genetics — Aniridia:
https://medlineplus.gov/genetics/condition/aniridia/