What Is Chandler’s Syndrome?

Overview

Chandler's Syndrome (CS) is a rare condition that affects the eye. More specifically, CS is a special kind of glaucoma that falls under the larger category known as iridocorneal endothelial syndrome (ICE).¹ In this condition, there is unusual growth and movement of certain cells that form a fibrous layer over the angle structures and the front surface of the iris in the eye. These changed cells can cause problems with the cornea and result in iris atrophy, corectopia (iris displacement), and the sticking together of parts in the front of the eye. There are three types of ICE syndrome: progressive iris atrophy, Chandler's syndrome, and Cogan-Reese iris nevus syndrome.⁷ Approximately 57% of cases of ICE are associated with Chandler’s syndrome.²

Although we can tell these conditions apart based on specific features, all of them can lead to serious vision problems due to glaucomatous optic neuropathy (damage to the optic nerve) or cloudy corneas, which make treating them difficult.

Causes of chandler’s syndrome

A thin layer of cells inside the front part of the eye, the endothelium, pushes out a watery liquid (aqueous humour) from the front part of the eye (cornea). This liquid, the aqueous humour, is needed for the eye to work properly. When the endothelium does not work as it should, like in CS, the liquid cannot be pushed out and stays in the front part of the eye. This causes the eye to swell and look blurry (corneal edema). Also, these cells can move around and create a layer over parts like the colourful part of the eye (iris) and the drainage area of the eye (trabecular meshwork). This can lead to changes in the iris (corectopia) and blockages that cause another eye problem called glaucoma.

The exact cause of CS is not well understood.³ Some experts think it could be because of inflammation or a long-lasting viral infection. More specifically, tiny bits of herpes virus in the eye fluid of people with Chandler's syndrome using a special test called polymerase chain reaction (PCR). 

Clinical manifestation

Initial reports indicated that individuals with ICE syndrome displayed signs of inflammation, with some experiencing uveitis, an inflammation of the eye. Certain cases exhibited clusters of persistent inflammatory cells within the iris and the vitreous, a gel-like substance within the eye. In a small number of cases, inflammation affected the front chamber of the eye. Occasionally, a type of immune cell known as macrophages was identified on the inner layer of the cornea in a select few cases.

Many patients diagnosed with Cogan-Reese syndrome had a mild yet persistent form of eye inflammation called chronic iridocyclitis. Noteworthy changes within the inner eye layer were observed, resembling those occurring in viral infections.⁴ Usually, the condition begins in one eye, suggesting a potential viral infection during early life in one eye while the other remains protected. In some instances, both eyes are impacted, possibly due to simultaneous infections.

The presence of herpes simplex virus (HSV) DNA was detected in the clear fluid and eye tissue of those with ICE syndrome. A specialized test called polymerase chain reaction (PCR) identified HSV-DNA in over half of the samples tested. Other viruses like herpes zoster and Epstein-Barr virus were typically absent in most cases.⁵

Signs and symptoms

There are several symptoms of CS, including the following: 

• Cells lining the cornea grow too much
• Thinning of the cornea²
• Swelling of the cornea
•  Iris distortion
• The pressure inside the eye can harm the nerve (glaucoma)
• Unusual changes in the iris and cornea, such as the pupil being in an odd position and looking different (corectopia), milder iris changes compared to similar issues, and the back of the cornea having a hammered silver appearance
• Blurry vision and eye pain, leading to gradual vision loss over time, usually affecting one eye with rare involvement of both

Management of treatment

The cause of ICE syndrome remains uncertain. Some researchers think antiviral medicine might help, but this has yet to be proven. 

No definite cure exists for any type of ICE syndrome. Rather, the focus is on managing problems like cloudy corneas and glaucoma.

1. Treating corneal issues:
   • Special saline eye drops in the morning can reduce corneal swelling
   • Medicines that lower eye pressure are often used to improve corneal swelling. They reduce eye fluid production.
   • Surgery is an option if medicines are not effective. Filtration surgery makes a new path for eye fluid drainage.
   •  Glaucoma drainage implants can be placed to manage glaucoma in ICE syndrome.
2. Glaucoma management:
   • Chandler’s syndrome might respond better to glaucoma management and surgery than other ICE forms due to less aggressive cell growth.
   • Cyclophotocoagulation might be necessary to control eye pressure, given the difficulty of ICE syndrome.
3. Severe corneal edema treatment:
   • Corneal surgery could help when the cornea is cloudy but eye pressure is controlled.
   • Surgeries like penetrating keratoplasty (PK) or Descemet stripping endothelial keratoplasty (DSEK) replace damaged corneal parts.

These surgeries cannot stop certain issues like glaucoma progression.

• Iris restoration:

Fixing the iris with or without an artificial lens can address vision issues related to iris irregularities.⁵

Diagnosis of chandler’s syndrome

There are several techniques that can be used to diagnose CS:

• Specular microscopy: This tool helps doctors look at the inside of the cornea. Change in the eye can be seen under the microscope as dark areas with a bright centre or bright borders, a unique appearance known as "light-dark reversal," and these cells are named "ICE cells.”
• Pachymeter: is a tool used to measure how swollen the cornea is.
• Gonioscopy: is a tool used to check for glaucoma (high eye pressure), by measuring the pressure inside the eye and evaluating the eye's angle.
• Heidelberg retinal tomogram (HRT) or optical coherence tomography (OCT): are more detailed tests that examine the nerves of the eye, measure the field of vision, and track glaucoma and nerve damage over time. 

Special techniques like slit-lamp examination, gonioscopy, and ultrasound biomicroscopy⁶ have been used to examine eye structures, and in-vivo (living) confocal microscopy has been used to observe the inner parts of the cornea. The unique appearance of cells in the cornea can confirm the diagnosis of Chandler's syndrome.¹

FAQs

How can I prevent chandler’s syndrome?

There are no known ways to prevent Chandler's syndrome. 

Which population is affected by chandler's syndrome?

Chandler's syndrome is uncommon and more frequently affects people assigned to females at birth (AFAB) than people assigned to males at birth (AMAB). It often happens when people are in their young adult or middle-aged years. However, incidents have also been reported in children and teens. Although CS can affect different populations of people, the frequency with which the three distinct forms manifest varies across populations. 

When should I see a doctor?

An eye doctor should be consulted if you notice abnormal changes in your vision (e.g., blurry vision, eye pain, sensitivity to light). Also, if you experience signs of glaucoma, such as high pressure in your eye or changes to your field of vision, see your doctor right away. It is important to get your eyes checked and treated early so that you can avoid problems and maintain healthy vision.

Summary

Chandler’s syndrome is a rare eye condition that mainly affects people with AFAB, usually beginning when they are young adults or middle-aged. This condition leads to cornea swelling, resulting in cloudy vision, light sensitivity, and impaired vision. Sometimes, the iris might stick to the cornea, too. A big worry is angle-closure glaucoma, where eye fluid does not drain properly, which may damage the optic nerve and, ultimately, vision. We are not exactly sure why this happens, but it might be due to inflammation or a virus.  Medical progress aids diagnosis and treatment, involving methods like eye drops, medications, and surgery. Collaborative efforts are vital for understanding and managing this complex disorder.