laitimes

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

author:Dr. Lee popularized science

Introduction: Iridician corneal endothelial disease (ICE) is a clinical manifestation of abnormalities in the cornea, iris and iris cornea. There are three types of clinical variants: primary (progressive) iris atrophy, Chandler syndrome, Kogan-Reis syndrome. Direct cut-out lamps show that the cornea and anterior part of patients with ICE syndrome do not accurately and objectively evaluate the structure of the lesion.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

1. Corneal endothelial syndrome (ICE)

1. Primary corneal endothelial dysfunction

Corneal endothelial syndrome (ICE) is a primary corneal endothelial dysfunction discovered by Eagle and Yanoff. There are three types of clinical. Chandler syndrome, parenchymal (progressive) iris atrophy, Kurgen-Rise syndrome (Erisneffers syndrome) is a series of ICE syndromes. Cogan-Reese syndrome (Cogan-Reese syndrome) is an iris syndrome clinically characterized by multiple small cylindrical nodules on the anterior surface of the iris, including iris atrophy, heterochromia, uveal valgus, ectopic pupils, corneal edema, and iris corneal abnormalities. Anterior slit lamps show limited results as corneal stromal edema progresses. Imaging techniques such as microscopy (SM), front optical coherence tomography (SDOCT), and ultrasonic biological microscopy enable objective analysis of disease progression, grading, and staging.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

2. The real cause of ICE syndrome is not known

From early to moderate, female preference, vision loss, pain, unilateral presence of varying degrees of corneal edema, iris atrophy, corneal abnormalities, and secondary glaucoma. The true cause of ICE syndrome is unclear. Alvarado et al. proposed a viral etiology and performed histological analysis based on the presence of inflammatory cells. Heteromorphic endothelial cells can migrate backwards, forming a membrane that covers surrounding structures, including the iris and trabecular network. The contraction of such membranes causes characteristic changes in the iris, the iris trabecular adhesions, the pupil elongates toward the most common pupil, causing secondary angle-closure glaucoma.

3. It is characterized by abnormalities of corneal endothelial cells

Corneal endothelial syndrome (ICE) is a unique ophthalmic disorder characterized by corneal endothelial cell abnormalities that can cause corneal edema, progressive iris atrophy, secondary angle-closure glaucoma, and no pupillary block. Cogan Reese Syndrome syndrome (ICE) is one of three variants of the iris corneal endothelial syndrome (ICE), which manifests as nodular and pigment-like lesions of the iris, with corneal and other iris defects visible. Secondary glaucoma is the main complication. Treatment is determined based on the severity of ocular symptoms and glaucoma is controlled for medication and surgery. Cogan-Reese syndrome is one of the iris corneal endothelial (ICE) syndromes that usually affects a young and middle-aged woman. There are differences between ICE syndromes (primary iris atrophy, Chandler syndrome, and Kogan-Rees syndrome). However, because all diseases affect the eyes, and some of their symptoms overlap, it is difficult to distinguish them. These two variants can represent different stages of the disease.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

2. Kogan-Raes syndrome

1. The cause of Kogan-Raess syndrome is still unknown

The cause of Kogan-Raess syndrome remains unknown. Some researchers suspect that inflammation or chronic infection causes the disease. It has also been suggested that primary lesions are related to the arrangement of the cornea (corneal endothelial cells) and that the effect on the iris is secondary or related. Some scientists believe that these three types of iridoretinal disease (ICE) syndrome may represent different stages of disease development. Although reported in children, Corgenres syndrome is rare. Most of these victims were white. The ratio of men to women is 1:2 and 1:5.

2. Patients with ICE syndrome

In patients with ICE syndrome, metaplasian endothelial cells begin to move backwards to Schwalbe's line, blocking the iris, and then continue to move to the anterior chamber, covering the front of the iris, forming an abnormal basement membrane with several layers contracting. Valgus causes uveal contraction, atrophy, and adhesions around the iris. Under the microscope, ICE cells can be seen to be large, rounded and multi-shaped, and the clinical manifestation is a specular reflection, which is consistent with the appearance of the cornea of the hammered silver bronze. Sherrard et al. describe four forms of ICE: diffusion ICE, which is characterized by transmission through healthy endothelial cells; in ICE, abnormal cells completely replace healthy endothelial cells; in ICE, abnormal cells replace healthy epithelial cells and the rest are small cells; intercontinental cell subspecies, where abnormal cells replace variable parts of normal endothelial cells.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

Although these SM morphological changes contribute to the staging of ICE symptoms, endothelial cell density (ECD) is not a reliable way to measure disease progression because poor recognition of cell margins limits the calculation of average cell density. In addition, ECD counts contain both ICE cells and residual normal endothelial cells and therefore may not reflect the true function of the endothelium.

3, the iris changes of this syndrome are unique

The iris changes of this syndrome are unique and provide clear diagnostic clues. This change manifests as multiple footed nodules, which in the early stages of the disease may appear as fine yellow nodules, followed by more pigmentation (Reese nodules), accompanied by atrophy of the iris, valgus of the uveus, and irregular pupils. In this case, the patient presents with multiple highly colored iris nodules in the foot, peripheral interstitial atrophy, and the crypt disappears. For ICE syndrome, long-term follow-up is required due to the nature of the disease itself is progressive. Continuous intraocular pressure monitoring and HFA can be used to assess disease progression and severity.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

Worldwide, glaucoma is the main cause of blindness. Elevated intraocular pressure is characteristic of glaucoma. If left untreated, increased stress can affect the optic nerve and eventually lead to blindness. Its etiology is unclear and it is still an active area of research. In Cogan-Reese syndrome, glaucoma may be a disease that follows Cogan-Reese syndrome. It is thought that the pathogenesis of this syndrome is related to the cell membranes where endothelial cells secrete abnormally. The film is covered with a small girder net at the drainage angle, blocking the outlet facilities in the room and increasing intraocular pressure. Treatment of Kogan-Rees syndrome usually involves treatment of glaucoma and edema with eye drops. Mild conditions or corneal edema are usually treated with soft-touch lenses and hypertonic saline. Currently, penetrating corneal transplantation or endothelial transplantation is required, although the failure rate is high and repeated corneal transplantation is required.

Iris corneal endothelial disease (ICE) causes abnormalities in the cornea, with 3 types of clinical variants

Conclusion: In conclusion, although ICE syndrome is not clinically common, its diagnosis and treatment have attracted widespread attention. The focus of this case report is its controversy and complexity, as people with ICE syndrome are usually middle-aged women. Treating glaucoma is complicated. Medications were initially used to treat glaucoma, but drug therapy or angle surgery ultimately failed due to the fact that the full angle was gradually covered or enclosed by a film covering or adhesions.

ICE

Read on