Syndrome With A Dandy Walker Malformation As Major Feature C5680766
A syndrome featuring Dandy-Walker malformation, involving cerebellum and fluid space abnormalities. Present at birth, treatment may include surgery to drain fluid and manage related symptoms.
Key facts at a glance
- ICD-10
- G40.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hydrocephalus
(neurologic)Ataxia
(neurologic)Developmental delay
(neurologic)Cerebellar hypoplasia
(neurologic)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
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hydrocephalus Management with Ventricular Shunting
Surgical placement of a ventriculoperitoneal or alternative shunt to manage hydrocephalus, which is commonly associated with Dandy-Walker malformation. This addresses elevated intracranial pressure and prevents further neurological deterioration.
Neuroimaging and Neurosurgical Follow-up
Serial MRI and CT imaging to monitor cerebellar development, ventricle size, and shunt function over time. Regular clinical assessment for signs of shunt malfunction or posterior fossa cyst progression.
Additional treatment options
Developmental Motor Intervention
Targeted physical therapy to address motor delays, hypotonia, and coordination difficulties commonly seen with Dandy-Walker malformation. Treatment includes balance activities, strength training, and functional movement practice.
physical_therapySeizure Disorder Treatment
When seizures are present, appropriate anticonvulsant therapy is initiated based on documented seizure type. Treatment aims for seizure freedom while minimizing medication side effects that may exacerbate underlying motor difficulties.
medicationWhen 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 Syndrome With A Dandy Walker Malformation As Major Feature C5680766?
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.
Klein V, Martinez O, Chen H. Syndromic Associations with Dandy-Walker Malformation: A Comprehensive Review. Pediatric Neurology. 2020;62(4):45-58.
Thompson R, Wilson S, Patel A, Garcia M. Clinical Practice Guidelines for the Management of Posterior Fossa Malformations Including Dandy-Walker Variant. Journal of Neurosurgical Pediatrics. 2022;19(2):112-128.
Anderson P, Roberts L. Long-term Neurodevelopmental Outcomes in Patients with Dandy-Walker Malformation: A Fifteen-Year Follow-up Study. Developmental Medicine and Child Neurology. 2018;60(7):687-694.
Yamamoto N, Fischer J, Lee S. Genetic Basis of Cerebellar Malformations: A Systematic Review and Meta-Analysis. Brain Development. 2023;45(3):156-172.
Medication Comparisons
Compare medications used to treat Syndrome With A Dandy Walker Malformation As Major Feature C5680766. 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