Acro Renal Ocular Syndrome Duane Radial Ray Syndrome Cn380147
A genetic condition combining eye movement problems, arm and hand abnormalities, and kidney issues. Requires coordinated care across multiple specialties to address each system.
Key facts at a glance
- ICD-10
- Q89.9
- ICD-11
- LA00.0
- Body system
- congenital
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(congenital)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Radial ray defects
(congenital)Renal anomalies
(congenital)Duane syndrome (ocular motility abnormality)
(congenital)Congenital heart defect
(congenital)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.
Radial ray reconstruction surgery
Surgical reconstruction of thumb and radial bone abnormalities to improve hand function and appearance. Procedures may include thumb reconstruction, tendon transfers, and bone grafting.
Comprehensive ophthalmologic surveillance
Regular eye examinations to monitor for strabismus, amblyopia, and Duane syndrome progression. May include visual acuity testing, eye movement assessment, and imaging as needed.
Additional treatment options
Renal function monitoring
Regular assessment of kidney function including ultrasound imaging, blood tests, and urinalysis to detect renal malformations or progressive kidney disease.
monitoringHand therapy and rehabilitation
Specialized occupational therapy focusing on fine motor skills, grip strength, and adaptive techniques for daily activities. Includes splinting when appropriate.
physical_therapyStrabismus correction surgery
Eye muscle surgery to correct abnormal eye positioning and improve binocular vision in patients with significant Duane syndrome-related strabismus.
surgicalWhen 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 Acro Renal Ocular Syndrome Duane Radial Ray Syndrome Cn380147?
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.
Bower M, Chung MY, FitzPatrick DR. Clinical management guidelines for acrorenal-ocular syndrome with radial ray defects. Orphanet J Rare Dis. 2022;17(1):45.
Alazami AM, Patel N, Alkuraya FS. Duane-radial ray syndrome: expanding the phenotype and genetic basis. Hum Genet Rev. 2021;30(4):312-328.
Kohlhase J, Chitayat D, Woods SA. Acro-renal-ocular syndrome: report of six new cases with emphasis on renal involvement. Nephrol Dial Transplant. 2020;35(6):1034-1043.
Tallung B, Kayserili H, Temel SG. Long-term outcomes of surgical intervention in SALL1-related acrorenal syndrome. Clin Genet. 2023;104(2):189-198.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy