Iridocorneal endothelial syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:64734H21.1
Who is this for?
Show terms as
17Specialists8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Iridocorneal endothelial syndrome, often called ICE syndrome, is a rare eye condition that affects the inner layer of the cornea (the clear front part of the eye). In ICE syndrome, the cells lining the back of the cornea behave abnormally — they grow where they should not, spreading across the drainage channels of the eye and onto the iris (the colored part of the eye). This causes a chain of problems including increased eye pressure, changes to the shape and appearance of the iris, and damage to the optic nerve over time. ICE syndrome actually includes three closely related conditions: Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome (also called iris nevus syndrome). Each has slightly different features, but they all share the same underlying problem with abnormal corneal cells. The condition almost always affects only one eye and is not passed down through families. The most common symptoms are blurred vision, seeing halos around lights, eye pain, and noticeable changes in how the iris looks. Over time, the rising eye pressure can lead to glaucoma, which can permanently damage vision if not treated. There is no cure for ICE syndrome, but treatments like eye drops, laser therapy, and surgery can help manage eye pressure and protect vision. Most people with ICE syndrome need long-term monitoring and care from an eye specialist.

Also known as:

Key symptoms:

Blurred or hazy visionSeeing halos or glare around lights, especially at nightEye pain or discomfortIncreased pressure inside the eye (glaucoma)Changes in the shape or appearance of the irisThe pupil looking distorted or off-centerSmall bumps or nodules on the surface of the irisSwelling or cloudiness of the corneaGradual loss of peripheral (side) visionSensitivity to light

Clinical phenotype terms (16)— hover any for plain English
Iris atrophyHP:0001089Ectopia pupillaeHP:0009918Anterior synechiae of the anterior chamberHP:0011483Abnormal migration of corneal endotheliumHP:0011489Corneal stromal edemaHP:0012040Central heterochromiaHP:0007818Hypoplastic iris stromaHP:0007990Posterior synechiae of the anterior chamberHP:0011484PolycoriaHP:0011500Iris nevusHP:0011525Abnormal pupil shapeHP:0025309Uveal ectropionHP:0025358
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

4 events
Apr 2026Methamphetamine, PrEP, and Intersectional Stigma Study

Arizona State University — NA

TrialNOT YET RECRUITING
Apr 2025Efficacy of Fuzheng Tongfu Jiedu Prescription for Treating Gastrointestinal Dysfunction Associated With Sepsis

Dongzhimen Hospital, Beijing — PHASE1, PHASE2

TrialNOT YET RECRUITING
Dec 2023Epidemiology of Injuries in the Professional Women's Swedish National IceHockey League Permitted to Body-Checking

Lund University

TrialRECRUITING
Apr 2021PrEP Intervention for People Who Inject Substances and Use Methamphetamine

San Francisco Department of Public Health — PHASE4

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Iridocorneal endothelial syndrome.

View clinical trials →

No actively recruiting trials found for Iridocorneal endothelial syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Iridocorneal endothelial syndrome community →

Specialists

17 foundView all specialists →
CO
Chen Ouyang
KENT, WA
Specialist
2 Iridocorneal endothelial syndrome publications
XD
Xiaojuan Dong
Specialist
2 Iridocorneal endothelial syndrome publications
HM
Hua Ma
EDINA, MN
Specialist
1 Iridocorneal endothelial syndrome publication
MX
Mingfang Xia
Specialist
1 Iridocorneal endothelial syndrome publication
QG
Qing Gu
NEW YORK, NY
Specialist
1 Iridocorneal endothelial syndrome publication
LZ
Lingling Zheng
Specialist
1 Iridocorneal endothelial syndrome publication
SH
Shaoping Ha
Specialist
1 Iridocorneal endothelial syndrome publication
YL
Yaoming Liu
Specialist
1 Iridocorneal endothelial syndrome publication
GL
Gen Li
Specialist
1 Iridocorneal endothelial syndrome publication
JJ
Jiaxuan Jiang
Specialist
1 Iridocorneal endothelial syndrome publication
SF
Sujie Fan
Specialist
1 Iridocorneal endothelial syndrome publication
LL
Lan Lu
FLOWERY BRANCH, GA
Specialist
1 Iridocorneal endothelial syndrome publication
TW
Ting Wang
Specialist
1 Iridocorneal endothelial syndrome publication
GL
Guigang Li
Specialist
1 Iridocorneal endothelial syndrome publication
WZ
Wenzong Zhou
Specialist
1 Iridocorneal endothelial syndrome publication
FM
Francis W Price, Jr., MD
Specialist
PI on 3 active trials
XM
Xiulan Zhang, MD,PhD
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Iridocorneal endothelial syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Iridocorneal endothelial syndromeForum →

No community posts yet. Be the first to share your experience with Iridocorneal endothelial syndrome.

Start the conversation →

Latest news about Iridocorneal endothelial syndrome

Disease timeline:

New recruiting trial: Epidemiology of Injuries in the Professional Women's Swedish National IceHockey League Permitted to Body-Checking

A new clinical trial is recruiting patients for Iridocorneal endothelial syndrome

New recruiting trial: PrEP Intervention for People Who Inject Substances and Use Methamphetamine

A new clinical trial is recruiting patients for Iridocorneal endothelial syndrome

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which type of ICE syndrome do I have — Chandler syndrome, progressive iris atrophy, or Cogan-Reese syndrome — and does it change my treatment?,How often do I need to come in for monitoring, and what tests will you do each visit?,What eye pressure level are we aiming for, and how will we know if my current treatment is working?,At what point would you recommend surgery, and what type would be best for me?,Is there any risk that my other eye will be affected?,Are there any activities or lifestyle changes I should make to protect my vision?,What signs should prompt me to call you or go to the emergency room right away?

Common questions about Iridocorneal endothelial syndrome

What is Iridocorneal endothelial syndrome?

Iridocorneal endothelial syndrome, often called ICE syndrome, is a rare eye condition that affects the inner layer of the cornea (the clear front part of the eye). In ICE syndrome, the cells lining the back of the cornea behave abnormally — they grow where they should not, spreading across the drainage channels of the eye and onto the iris (the colored part of the eye). This causes a chain of problems including increased eye pressure, changes to the shape and appearance of the iris, and damage to the optic nerve over time. ICE syndrome actually includes three closely related conditions: Chand

How is Iridocorneal endothelial syndrome inherited?

Iridocorneal endothelial syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Iridocorneal endothelial syndrome typically begin?

Typical onset of Iridocorneal endothelial syndrome is adult. Age of onset can vary across affected individuals.

Which specialists treat Iridocorneal endothelial syndrome?

17 specialists and care centers treating Iridocorneal endothelial syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.