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The Dandy-Walker syndrome: diagnostic and surgical considerations
M Marinov1, S Gabrovsky, S Undjian
1Department of Neurosurgery, Medical Academy, Sofia, Bulgaria.
British Journal of Neurosurgery
|January 1, 1991
Summary
Dandy-Walker syndrome management requires careful diagnosis to differentiate from CSF cysts and assess aqueduct patency. Surgical outcomes show potential for normal IQs despite an 18% mortality rate.
Area of Science:
- Neurology
- Pediatric Neurosurgery
- Medical Imaging
Background:
- Dandy-Walker syndrome is a congenital brain malformation.
- Hydrocephalus is a common complication, occurring in 77% of cases.
- Associated central nervous system anomalies are present in 8 patients, necessitating thorough evaluation.
Purpose of the Study:
- To review preoperative evaluation, surgical treatment, and outcomes for Dandy-Walker syndrome.
- To emphasize the importance of differentiating Dandy-Walker syndrome from extra-axial CSF cysts.
- To highlight the necessity of establishing aqueduct patency for appropriate surgical management.
Main Methods:
- Review of 13 Dandy-Walker syndrome cases over 12 years.
- Discussion of diagnostic methods, emphasizing metrizamide computed tomographic ventriculography.
- Analysis of surgical treatment approaches and outcomes.
Main Results:
- Hydrocephalus incidence was 77%.
- Direct surgical approaches like membrane excision showed unsatisfactory results.
- The aqueduct was invariably patent, allowing for cyst or ventricular shunting.
- Mortality rate was 18%, with 54.5% of surviving children achieving normal IQs.
Conclusions:
- Accurate preoperative diagnosis is critical for effective surgical management of Dandy-Walker syndrome.
- Shunting procedures are preferred over direct surgical intervention.
- Early identification of associated anomalies and CSF dynamics is crucial for patient outcomes.