What is the recommended surveillance plan for a pediatric patient with Dandy Walker syndrome?

Surveillance of Dandy-Walker Syndrome

Children with Dandy-Walker syndrome require neurodevelopmental surveillance at every well-child visit, with formal developmental evaluations at 12-24 months, 3-5 years, and 11-12 years of age, plus standardized developmental screening at 9,18,30, and 48 months. 1

Risk Stratification and Initial Assessment

Children with Dandy-Walker syndrome should be classified as high-risk for developmental disorders due to the presence of a significant congenital brain malformation, which meets criteria for heightened surveillance according to American Heart Association guidelines for children with structural anomalies. 1

Key Clinical Presentation Timeline

• 80% become symptomatic by age 3 years, with 70% presenting within the first year of life 2
• 91% develop hydrocephalus at time of diagnosis 2
• One-third demonstrate developmental delay at presentation 2
• 48% have associated congenital anomalies requiring additional surveillance 2

Neurodevelopmental Surveillance Protocol

Continuous Surveillance

Developmental surveillance should occur at every well-child visit regardless of whether formal developmental disorders have been identified, as risk levels can change over time. 1

Standardized Screening Schedule

Administer age-appropriate developmental screening tools at:

• 9 months of age 1
• 18 months of age (include autism-specific screening) 1
• 24 months of age (autism-specific screening) 1
• 30 months of age 1
• 48 months of age 1

Formal Developmental Evaluations

Direct referral for comprehensive multidisciplinary developmental and medical evaluation at:

• 12-24 months of age 1
• 3-5 years of age 1
• 11-12 years of age 1

Neuroimaging Surveillance

Hydrocephalus Monitoring

Serial neuroimaging is essential given that 91% of patients develop hydrocephalus and 96% of survivors become shunt-dependent. 2 While specific imaging intervals are not standardized in guidelines, clinical practice suggests:

• Baseline MRI or CT at diagnosis to characterize the malformation 2, 3, 4
• Follow-up imaging based on clinical symptoms (headache, vomiting, altered mental status, developmental regression) 3, 4
• Urgent imaging for any acute neurological symptoms, as presentation can be delayed and atypical 4

Post-Surgical Surveillance

For the 96% who require shunt placement:

• Regular shunt function monitoring through clinical assessment 2
• Imaging when shunt malfunction suspected (symptoms include persistent vomiting, abdominal pain, abnormal posturing, neck extension) 4

Multidisciplinary Team Approach

Establish care coordination through a medical home model involving: 1

• Pediatric neurologist or neurosurgeon (for hydrocephalus management)
• Developmental pediatrician or geneticist
• Early intervention services (before age 3)
• Early childhood special education services (ages 3-5)
• Behavioral screening specialists 1

Common Pitfalls to Avoid

Delayed Recognition of Hydrocephalus

• Do not dismiss atypical presentations: Patients may present with vomiting and abdominal pain rather than classic neurological signs 4
• Maintain high index of suspicion even in older children, as 20% present after age 3 years 2

Underestimating Developmental Risk

• Mental retardation occurs in 65% of cases 5, making aggressive developmental surveillance critical
• Continue surveillance even if initial screenings are normal, as developmental issues may emerge later 1

Inadequate Surgical Management

• Mortality is 10-fold higher without CSF drainage 6
• Operative CSF drainage reduces mortality by 44% among DWS patients 6
• Combined shunting of both cyst and lateral ventricles is superior to single-system shunting (92% success rate) 2

Prognosis and Long-Term Outcomes

Overall mortality is 24%, with most deaths occurring in untreated or inadequately treated hydrocephalus. 2, 5 However, approximately 35% of children achieve reasonable developmental outcomes with appropriate intervention, justifying aggressive treatment and surveillance. 5

Increased access to neurosurgical intervention could reduce mortality toward that of the general population, as the elevated mortality is directly attributable to the 80% who do not receive operative CSF drainage. 6