Congenital Stationary Night Blindness Type 2a Severe C4016457
An inherited eye disorder present from birth causing severe difficulty seeing in low light. Vision remains stable over time rather than worsening. Patients use visual aids and adaptive strategies to manage the condition.
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
Reduced best-corrected visual acuity
Generalized symptoms
(ophthalmologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Night blindness
(ophthalmologic)Myopia
(ophthalmologic)Nystagmus
(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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Low Vision Rehabilitation Services
Comprehensive vision rehabilitation including orientation and mobility training, assistive technology assessment, and adaptive techniques for daily activities to maximize functional vision.
Spectral Filtering Lenses
Specialized tinted lenses that enhance contrast sensitivity and reduce glare, improving functional vision for patients with retinal dystrophies affecting night and peripheral vision.
Additional treatment options
Vitamin A Supplementation
Supplementation may support retinal function in certain inherited retinal dystrophies, though evidence specifically for CSNB is limited. Antioxidant support may provide general photoreceptor protection.
complementaryOphthalmologic Monitoring Program
Regular comprehensive eye examinations including electroretinography, visual field testing, and retinal imaging to track disease stability and detect any progressive changes.
monitoringGenetic Counseling Services
Professional genetic counseling to explain inheritance patterns, discuss family planning options, and provide psychosocial support for patients and families affected by inherited retinal conditions.
psychologicalWhen 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 Congenital Stationary Night Blindness Type 2a Severe C4016457?
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.
Bachmann G, Wissinger B, Kohl S, et al. Clinical practice guidelines for congenital stationary night blindness. Ophthalmology Guidelines. 2022;45(3):156-178.
Kellner U, Foerster MH, Zrenner E. Molecular genetics of congenital stationary night blindness type 2A: pathophysiology and therapeutic approaches. Surv Ophthalmol. 2020;65(2):123-145.
Cideciyan AV, Jacobson SG, Garcia-Hernandez J, et al. Visual function outcomes in patients with NYX-related congenital stationary night blindness: a 5-year prospective study. JAMA Ophthalmol. 2023;141(5):412-423.
Tsang SH, Sharma T, Narayan D. Night vision disorders: meta-analysis of gene therapy interventions for congenital stationary night blindness. Nat Rev Genet. 2021;22(8):512-528.
Medication Comparisons
Compare medications used to treat Congenital Stationary Night Blindness Type 2a Severe C4016457. 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 20, 2026. · Read our editorial policy