Iridocorneal endothelial (ICE)

syndromes A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek σύνδρομον, meaning "concurrence". When a syndrome is paire ...

are a spectrum of

diseases A disease is a particular abnormal condition that adversely affects the structure or function of all or part of an organism and is not immediately due to any external injury. Diseases are often known to be medical conditions that are asso ...

characterized by slowly progressive abnormalities of the

corneal endothelium The corneal endothelium is a single layer of endothelial cells on the inner surface of the cornea. It faces the chamber formed between the cornea and the iris. The corneal endothelium are specialized, flattened, mitochondria-rich cells that ...

and features including corneal

edema Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue (biology), tissue. Most commonly, the legs or arms are affected. S ...

, iris distortion, and secondary

angle-closure glaucoma Glaucoma is a group of eye diseases that can lead to damage of the optic nerve In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual ...

.Weisenthal RW. ''2012-2013 Basic and Clinical Science Course, Section 8, Chapter 12: External Disease and Cornea'' (pp 344–345). San Francisco CA: American Academy of Ophthalmology The Eye M.D. Association ICE syndromes are predominantly unilateral and nonhereditary. The condition occurs in predominantly middle-aged women.Iridocorneal Endothelial (ICE) syndrome presents a unique set of challenges for both patients and

ophthalmologists Ophthalmology (, ) is the branch of medicine that deals with the diagnosis, treatment, and surgery of eye diseases and disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Following a ...

, and effective treatment of this group of rare ocular diseases requires a combination of

diagnostic Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " ...

and

therapeutic A therapy or medical treatment is the attempted remediation of a health problem, usually following a medical diagnosis. Both words, ''treatment'' and ''therapy'', are often abbreviated tx, Tx, or Tx. As a rule, each therapy has indications an ...

complexity. It's important to understand

Signs and symptoms

Many cases are

asymptomatic Asymptomatic (or clinically silent) is an adjective categorising the medical conditions (i.e., injuries or diseases) that patients carry but without experiencing their symptoms, despite an explicit diagnosis (e.g., a positive medical test). P ...

, however patients many have decreased vision, glare, monocular

diplopia Diplopia is the simultaneous perception of two images of a single object that may be displaced in relation to each other. Also called double vision, it is a loss of visual focus under regular conditions, and is often voluntary. However, when occ ...

or polyopia, and noticeable iris changes. On exam patients have normal to decreased

visual acuity Visual acuity (VA) commonly refers to the clarity of visual perception, vision, but technically rates an animal's ability to recognize small details with precision. Visual acuity depends on optical and neural factors. Optical factors of the eye ...

, and a "beaten metal appearance" of the corneal endothelium, corneal edema, increased

intraocular pressure Intraocular pressure (IOP) is the fluid pressure inside the eye. Tonometry is the method eye care professionals use to determine this. IOP is an important aspect in the evaluation of patients at risk of glaucoma. Most tonometers are calibrated t ...

, peripheral anterior synechiae, and iris changes.

Mechanism

The exact mechanism is unknown, however there appears to be a component of abnormal corneal endothelium that proliferates onto the iris forming a

membrane A membrane is a selective barrier; it allows some things to pass through but stops others. Such things may be molecules, ions, or other small particles. Membranes can be generally classified into synthetic membranes and biological membranes. Bi ...

that then obstructs the

trabecular meshwork The trabecular meshwork is an area of tissue in the eye located around the base of the cornea, near the ciliary body, and is responsible for draining the aqueous humor from the eye via the anterior chamber (the chamber on the front of the ...

, leading to iris distortion.

Nodule Nodule may refer to: * Nodule (geology), a small rock or mineral cluster * Manganese nodule, a metallic concretion found on the seafloor *Nodule (medicine), a small aggregation of cells *Root nodule Root nodules are found on the roots of plants, ...

formation can also occur when the abnormal corneal endothelium causes contractions around the iris stroma.

Herpesvirus ''Orthoherpesviridae'', previously named and more widely known as ''Herpesviridae'', is a large family of DNA viruses that cause infections and certain diseases in animals, including humans. The members of this family are commonly known as herp ...

DNA Deoxyribonucleic acid (; DNA) is a polymer composed of two polynucleotide chains that coil around each other to form a double helix. The polymer carries genetic instructions for the development, functioning, growth and reproduction of al ...

has been identified in some patients following keratoplasty, suggesting the possibility that

herpes simplex virus Herpes simplex virus 1 and 2 (HSV-1 and HSV-2) are two members of the Herpesviridae#Human herpesvirus types, human ''Herpesviridae'' family, a set of viruses that produce Viral disease, viral infections in the majority of humans. Both HSV-1 a ...

may induce the abnormal endothelialization in the

anterior chamber angle The anterior chamber angle is a part of the eye located between the cornea and iris which contains the trabecular meshwork. The size of this angle is an important determinant of the rate aqueous humour flows out of the eye, and thus, the intraocu ...

and on the surface of the iris.

Variations

The Chandler variant of ICE is characterized by

pathology Pathology is the study of disease. The word ''pathology'' also refers to the study of disease in general, incorporating a wide range of biology research fields and medical practices. However, when used in the context of modern medical treatme ...

on the inner surface of the

cornea The cornea is the transparency (optics), transparent front part of the eyeball which covers the Iris (anatomy), iris, pupil, and Anterior chamber of eyeball, anterior chamber. Along with the anterior chamber and Lens (anatomy), lens, the cornea ...

leading to abnormal

endothelial The endothelium (: endothelia) is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the res ...

pump function. Other features include possible mild iris changes, corneal edema, and normal to slight elevations in

. Cogan-Reese variant is characterized by multiple pigmented iris

nodules Nodule may refer to: *Nodule (geology), a small rock or mineral cluster *Manganese nodule, a metallic concretion found on the seafloor *Nodule (medicine), a small aggregation of cells *Root nodule Root nodules are found on the roots of plants, p ...

. This variant is most commonly unilateral and seen in middle-aged females.

Diagnosis

Treatment

Penetrating keratoplasty and endothelial keratoplasty can be used as treatments for severe cases of ICE. Because

glaucoma Glaucoma is a group of eye diseases that can lead to damage of the optic nerve. The optic nerve transmits visual information from the eye to the brain. Glaucoma may cause vision loss if left untreated. It has been called the "silent thief of ...

and elevated intraocular pressure are often present in ICE patients, long term follow up may be needed to ensure adequate intraocular pressures are maintained.

Prognosis

The disease is chronic and often progresses slowly.

Prognosis Prognosis ( Greek: πρόγνωσις "fore-knowing, foreseeing"; : prognoses) is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) ...

is generally poor when associated with

References

External links

Facts About the Cornea and Corneal Disease
The National Eye Institute (NEI). {{Eye pathology Eye diseases Syndromes