Overview
X-linked corneal dermoid is a very rare eye condition that is present from birth. In this condition, a dermoid — a benign (non-cancerous) growth made up of skin-like tissue — develops on the cornea, which is the clear front surface of the eye. These growths can contain tissue such as skin, hair follicles, and fat, which are not normally found on the eye. Because the condition follows an X-linked inheritance pattern, it primarily affects males, while females may be carriers or show milder symptoms. The main concern with corneal dermoids is their effect on vision. Depending on the size and location of the growth, it can block or distort vision in the affected eye. This can lead to amblyopia (lazy eye) in children if not addressed early. Some patients may also experience eye irritation or cosmetic concerns due to the visible growth on the eye surface. Treatment typically involves surgical removal of the dermoid. The specific surgical approach depends on how deep the growth extends into the cornea. Superficial dermoids may be removed with a procedure called lamellar keratectomy, while deeper ones may require more complex surgery, sometimes including corneal transplantation. Early evaluation and management are important to preserve vision and prevent complications like amblyopia during the critical years of visual development in childhood.
Also known as:
Key symptoms:
White or yellowish growth on the surface of the eyeReduced vision in the affected eyeLazy eye (amblyopia)Astigmatism or irregular shape of the corneaEye irritation or discomfortTearing or watery eyesSensitivity to lightCosmetic difference in the appearance of the eyePossible hair-like fibers growing from the eye surface
Clinical phenotype terms (4)— hover any for plain English
X-linked recessive
Carried on the X chromosome; typically affects males more than females
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for X-linked corneal dermoid.
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Specialists
View all specialists →No specialists are currently listed for X-linked corneal dermoid.
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 X-linked corneal dermoid.
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 deep is the dermoid, and what surgical approach do you recommend?,What are the risks and benefits of surgery at my child's current age?,Is there a risk of amblyopia, and how will we monitor and treat it?,Will my child need a corneal transplant, and what does that involve?,Should we pursue genetic testing, and what would it tell us?,Are other family members at risk of being carriers or affected?,What is the long-term vision outlook for my child after treatment?
Common questions about X-linked corneal dermoid
What is X-linked corneal dermoid?
X-linked corneal dermoid is a very rare eye condition that is present from birth. In this condition, a dermoid — a benign (non-cancerous) growth made up of skin-like tissue — develops on the cornea, which is the clear front surface of the eye. These growths can contain tissue such as skin, hair follicles, and fat, which are not normally found on the eye. Because the condition follows an X-linked inheritance pattern, it primarily affects males, while females may be carriers or show milder symptoms. The main concern with corneal dermoids is their effect on vision. Depending on the size and loca
How is X-linked corneal dermoid inherited?
X-linked corneal dermoid follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does X-linked corneal dermoid typically begin?
Typical onset of X-linked corneal dermoid is neonatal. Age of onset can vary across affected individuals.