Leber Congenital Amaurosis 4 C1858386
A genetic retinal disease causing severe vision loss from birth or early childhood. It affects the retina's light-sensing cells. Management focuses on low vision support.
Key facts at a glance
- ICD-10
- Q15.9
- ICD-11
- LA00.0
- Body system
- ophthalmologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe congenital visual impairment
Generalized symptoms
(ophthalmologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Nystagmus
(ophthalmologic)Photophobia
(ophthalmologic)Keratoconus
(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
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Regular ophthalmologic monitoring
Comprehensive eye examinations with electroretinography, visual field testing, and retinal imaging to track disease progression and identify complications. Essential for managing associated systemic manifestations that may occur with LCA4.
Comprehensive rehabilitation services
Multi-disciplinary rehabilitation including orientation and mobility training, activities of daily living skills, and social support services. Addresses both vision-specific and broader developmental needs.
Additional treatment options
Cataract surgery when indicated
Surgical intervention for cataract removal when lens opacification significantly impacts residual vision. Combined with appropriate lens implantation based on individual retinal function assessment.
surgicalAssistive technology training
Training in use of low vision aids, adaptive devices, and technology solutions to support education, employment, and daily activities. Includes Braille instruction when appropriate.
complementaryEducational support services
Specialized educational interventions including individualized education plan development, accommodations for learning, and collaboration with schools to ensure appropriate support. Addresses unique learning needs associated with congenital blindness.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Leber Congenital Amaurosis 4 C1858386?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Leber Congenital Amaurosis 4 C1858386. 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.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy