Congenital Hereditary Endothelial Dystrophy Type I C1562945
A rare inherited eye condition where the inner corneal layer is swollen, causing blurred vision from birth. It may require corneal transplantation to restore sight.
Key facts at a glance
- ICD-10
- Q15.9
- ICD-11
- LA00.0
- Body system
- ophthalmologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Corneal edema
(ophthalmologic)Generalized symptoms
(ophthalmologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Blurred vision
(ophthalmologic)Photophobia
(ophthalmologic)Corneal scarring
(ophthalmologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Penetrating Keratoplasty (Corneal Transplant)
Surgical replacement of the diseased cornea with healthy donor tissue to restore vision clarity. This is the definitive treatment for severe cases of CHED Type I where corneal clouding significantly impairs vision.
Hypertonic Saline Eye Drops
Topical application of hypertonic saline solution to reduce corneal edema by drawing fluid out of the corneal stroma. Provides symptomatic relief and temporary improvement in corneal clarity.
Additional treatment options
Regular Ophthalmologic Surveillance
Scheduled comprehensive eye examinations including corneal thickness measurements, endothelial cell counts, and visual acuity assessments to track disease progression and surgical timing.
monitoringDSAEK (Descemet's Stripping Automated Endothelial Keratoplasty)
Partial thickness corneal transplant replacing only the diseased endothelial layer with donor tissue. Offers faster visual recovery and fewer complications compared to full-thickness transplant.
procedureLow-Sodium Diet and Hydration Management
Dietary modifications to reduce systemic fluid retention that can worsen corneal edema. Combined with appropriate fluid intake management to support corneal deturgescence.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Congenital Hereditary Endothelial Dystrophy Type I C1562945?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Srinivasan B, Sahay P, Iyer G. Congenital Hereditary Endothelial Dystrophy: SLC4A11 Gene Mutations and Clinical Features. Cornea. 2021.
Aldave AJ, DeMatteo J, Rapuano CJ, et al. Guidelines for the Diagnosis and Management of Corneal Dystrophies: ISged System Update. Am J Ophthalmol. 2023.
Malhotra C, Pandav SS, Rai V, et al. Outcomes of Descemet Membrane Endothelial Keratoplasty in Congenital Hereditary Endothelial Dystrophy. Br J Ophthalmol. 2022.
Vithana EN, Morgan PE, Sundaresan P. SLC4A11 Gene and Corneal Endothelial Dystrophies: A Systematic Review of the Literature. Invest Ophthalmol Vis Sci. 2020.
Medication Comparisons
Compare medications used to treat Congenital Hereditary Endothelial Dystrophy Type I C1562945. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy