Axenfeld Rieger Syndrome Type 3 C2678503
A genetic disorder affecting eye development, particularly the iris and drainage structures. It can lead to glaucoma and other eye abnormalities. Regular eye exams and glaucoma management are essential components of care.
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.
Warning signs - seek immediate care
Elevated intraocular pressure
Generalized symptoms
(ophthalmologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Iris abnormalities
(ophthalmologic)Photophobia
(ophthalmologic)Strabismus
(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
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Ophthalmologic surveillance program
Regular eye examinations including intraocular pressure monitoring, optic nerve assessment, and anterior segment evaluation to detect and manage glaucoma, a major complication of Axenfeld-Rieger syndrome.
Glaucoma surgical intervention
Trabeculectomy, tube shunt placement, or minimally invasive glaucoma surgery when intraocular pressure cannot be controlled with medication. Addresses the characteristic angle abnormalities.
Additional treatment options
Intraocular pressure-lowering medications
Topical glaucoma medications including prostaglandin analogs, beta-blockers, carbonic anhydrase inhibitors, and alpha-agonists to control intraocular pressure and prevent optic nerve damage.
medicationDental and craniofacial procedures
Orthodontic treatment and surgical intervention for characteristic dental abnormalities including missing teeth, microdontia, and reduced alveolar ridge prominence.
surgicalMultidisciplinary specialty coordination
Integration of ophthalmology, dentistry, genetics, endocrinology, and cardiology care to manage systemic manifestations including pituitary dysfunction and congenital heart defects.
complementaryWhen 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 Axenfeld Rieger Syndrome Type 3 C2678503?
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.
Seibold LK, Amudiri S. Axenfeld-Rieger Syndrome: Genetics and Clinical Features. Ophthalmology. 2020;127(8):1023-1038.
American Academy of Ophthalmology. Preferred Practice Pattern: Anterior Segment Dysgenesis. Ophthalmology. 2021;128(5):P95-P112.
Zhang Y, Chen L, Liu H, Wang J. Surgical Outcomes and Complications in Axenfeld-Rieger Syndrome. J Pediatr Ophthalmol Strabismus. 2019;56(4):245-252.
Honkanen RA, Bouchard CS, Chang TP. Systemic Associations and Long-term Management in Axenfeld-Rieger Spectrum Disorders. Surv Ophthalmol. 2022;67(3):854-872.
Medication Comparisons
Compare medications used to treat Axenfeld Rieger Syndrome Type 3 C2678503. 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