Chandler's disease

Chandler's Syndrome is a rare eye disorder that is part of a group of diseases known as the IridoCorneal Endothelial Syndrome (ICE Syndrome). It primarily affects the cornea, the clear front surface of the eye, leading to progressive vision loss if not managed appropriately. This condition is characterized by the abnormal growth of endothelial cells, which line the back of the cornea. These cells migrate across the trabecular meshwork (an area of tissue in the eye that drains aqueous humor) and onto the surface of the iris, causing changes in eye pressure and the appearance of the iris.

Symptoms and Diagnosis

The symptoms of Chandler's Syndrome can vary but often include blurred vision, the sensation of seeing halos around lights, and sometimes pain or discomfort in the affected eye. As the disease progresses, more severe symptoms such as glaucoma and corneal swelling (corneal edema) may develop, leading to significant vision impairment.

Diagnosis of Chandler's Syndrome involves a comprehensive eye examination. Tools such as slit lamp examination, gonioscopy, and specular microscopy are essential in assessing the extent of endothelial cell abnormality and its impact on the eye's structures. Imaging tests like anterior segment optical coherence tomography (AS-OCT) can also provide detailed images of the cornea and help in the diagnosis.

Treatment

Treatment for Chandler's Syndrome focuses on managing symptoms and preventing further damage to the eye. In the early stages, topical eye drops that reduce eye pressure can help manage glaucoma and corneal swelling. In more advanced cases, surgical interventions such as trabeculectomy or the implantation of glaucoma drainage devices may be necessary to control intraocular pressure. For patients with significant corneal edema, corneal transplantation might be considered to restore vision.

Epidemiology

Chandler's Syndrome is the most common form of ICE Syndrome, yet it remains a rare condition. It predominantly affects middle-aged individuals and is more common in females than in males. The exact prevalence of Chandler's Syndrome is difficult to determine due to its rarity and the potential for misdiagnosis.

Pathophysiology

The pathophysiology of Chandler's Syndrome involves the abnormal proliferation and migration of corneal endothelial cells. These cells typically function to pump excess fluid out of the cornea, maintaining its clarity. In Chandler's Syndrome, the endothelial cells spread over the trabecular meshwork and the surface of the iris, leading to increased eye pressure and changes in the iris's appearance. The underlying cause of this endothelial dysfunction is not fully understood, but it is believed to be a combination of genetic and environmental factors.

Prognosis

The prognosis for individuals with Chandler's Syndrome varies depending on the severity of the condition at the time of diagnosis and the effectiveness of treatment. Early detection and management are crucial in preventing vision loss. With appropriate treatment, many patients can maintain good vision and control the progression of the disease.