Overview
Congenital cataract microcornea with corneal opacity is a rare eye condition that is present at birth. In this disease, babies are born with cataracts (clouding of the lens inside the eye), microcornea (an abnormally small cornea, which is the clear front window of the eye), and corneal opacity (a cloudy or hazy appearance of the cornea that blocks light from entering the eye properly). Together, these three problems significantly reduce vision from a very early age. The condition affects the structures at the front of the eye that are responsible for focusing light onto the retina. When the lens is cloudy (cataract) and the cornea is both small and opaque, very little clear light can reach the back of the eye, which can lead to severe visual impairment. If not treated early, the lack of clear visual input during critical periods of brain development can lead to amblyopia (lazy eye), where the brain never fully learns to process images from the affected eye. Treatment typically involves early surgical removal of the cataracts, often within the first few weeks or months of life, to allow light to reach the retina during the critical window of visual development. Corneal opacity may require additional procedures such as corneal transplantation in some cases. Corrective lenses, contact lenses, or intraocular lens implants are used after cataract surgery. Ongoing vision therapy and close monitoring by eye specialists are essential throughout childhood. Despite treatment, some degree of visual impairment may persist depending on the severity of the corneal changes.
Also known as:
Key symptoms:
Cloudy or white appearance in the pupil of the eye (cataract)Abnormally small cornea (front part of the eye)Hazy or opaque cornea that looks cloudyPoor vision or blindness from birthDifficulty tracking objects or making eye contactNystagmus (involuntary shaking or wobbling of the eyes)Sensitivity to lightCrossed eyes or misaligned eyes (strabismus)Lazy eye (amblyopia)Small eye size overall (microphthalmos) in some casesDifficulty with depth perception
Variable
Can be inherited in different ways depending on the underlying gene
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Congenital cataract microcornea with corneal opacity.
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Specialists
View all specialists →No specialists are currently listed for Congenital cataract microcornea with corneal opacity.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Congenital cataract microcornea with corneal opacity.
Community
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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.How severe are the cataracts and corneal opacity, and how much are they affecting my child's vision?,When should cataract surgery be performed, and what are the risks and expected outcomes?,Will my child need a corneal transplant, and if so, when is the best time?,What is the risk of developing glaucoma after surgery, and how will it be monitored?,Should we pursue genetic testing, and what would the results mean for our family?,What early intervention and vision rehabilitation services should we start now?,What is the long-term visual outlook for my child with this condition?
Common questions about Congenital cataract microcornea with corneal opacity
What is Congenital cataract microcornea with corneal opacity?
Congenital cataract microcornea with corneal opacity is a rare eye condition that is present at birth. In this disease, babies are born with cataracts (clouding of the lens inside the eye), microcornea (an abnormally small cornea, which is the clear front window of the eye), and corneal opacity (a cloudy or hazy appearance of the cornea that blocks light from entering the eye properly). Together, these three problems significantly reduce vision from a very early age. The condition affects the structures at the front of the eye that are responsible for focusing light onto the retina. When the
At what age does Congenital cataract microcornea with corneal opacity typically begin?
Typical onset of Congenital cataract microcornea with corneal opacity is neonatal. Age of onset can vary across affected individuals.