Dandy-Walker syndrome: an updated literature review
Maria Isabel Ocampo-Navia1,2,3, Wilfran Perez-Mendez4, Maria Paula Rodriguez-Alvarez4
1Department of Neurosurgery, Pontificia Universidad Javeriana, Bogotá, Colombia. maria_ocampo@javeriana.edu.co.
Summary
Dandy-Walker syndrome (DWS) is a midline cerebellar disorder affecting 1 in 25,000 births. Diagnosis uses neuroimaging, and management focuses on hydrocephalus and associated anomalies for better outcomes.
Area of Science:
- Neurology
- Developmental Biology
- Genetics
Background:
- Dandy-Walker syndrome (DWS) is a spectrum of midline cerebellar developmental disorders.
- It includes Dandy-Walker malformation, Blake's pouch cyst, and mega cisterna magna, potentially sharing embryological origins.
- Etiology involves genetic, chromosomal, and environmental factors.
Purpose of the Study:
- To provide an updated review on Dandy-Walker syndrome.
- To cover embryology, pathophysiology, diagnostic approaches, and management strategies.
- To consolidate current knowledge on this rare neurological condition.
Main Methods:
- This study presents an unsystematic updated review.
- Literature search on embryology, pathophysiology, diagnosis, and management of DWS.
- Synthesis of information from existing research and clinical data.
Main Results:
- DWS occurs in 1 in 25,000 to 35,000 live births.
- Commonly presents with hydrocephalus and other central nervous system anomalies.
- Clinical manifestations range from neonatal to adult stages.
Conclusions:
- Diagnosis relies on neuroimaging of the posterior fossa and associated anomalies.
- Management requires addressing hydrocephalus, associated anomalies, and multidisciplinary neurological follow-up.
- Prognosis is significantly influenced by the presence and severity of associated malformations.
Keywords:
Blake’s pouch cystDandy-Walker malformationDandy-Walker syndromeHydrocephalusMega cisterna magnaMore Related Videos
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