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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Classic Dandy-Walker (DW) malformation (DWM)
Synonyms
- Terms DW spectrum, DW variant, & DW complex have complicated clinical & imaging implications in literature
Definitions
- DW continuum represents clinically & radiologically heterogeneous group of posterior fossa (PF) malformations
- Referred to as continuum by some based on belief that DWM, vermian hypoplasia (VH), Blake pouch (BP) remnant/cyst, & mega cisterna magna (MCM) are spectrum of developmental anomalies of rhombencephalic vesicle roof
- Terminology controversial & not uniformly accepted
- Clinical severity ranges widely in this group
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Kau T et al: Blake's pouch cysts and differential diagnoses in prenatal and postnatal MRI : a pictorial review. Clin Neuroradiol. ePub, 2020
- Pertl B et al: The fetal posterior fossa on prenatal ultrasound imaging: normal longitudinal development and posterior fossa anomalies. Ultraschall Med. 40(6):692-721, 2019
- Spinelli M et al: The "vermian-crest angle": does it allow accurate categorisation of fetal upward rotation of cerebellar vermis on intrauterine MRI? A pilot study. Clin Radiol. 74(6):489.e1-7, 2019
- Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
- Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
- Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Lippincott Williams & Wilkins. 477-9, 2012
- Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
- Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
- Volpe P et al: Brainstem-vermis and brainstem-tentorium angles allow accurate categorization of fetal upward rotation of cerebellar vermis. Ultrasound Obstet Gynecol. 39(6):632-5, 2012
- Aldinger KA et al: FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 41(9):1037-42, 2009
- Barkovich AJ et al: A developmental and genetic classification for midbrain-hindbrain malformations. Brain. 132(12):3199-230, 2009
- Walbert T et al: Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. J Clin Oncol. 27(17):2886-7, 2009
- Elsen GE et al: Zic1 and Zic4 regulate zebrafish roof plate specification and hindbrain ventricle morphogenesis. Dev Biol. 314(2):376-92, 2008
- Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
- Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
- ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
- Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
- Tortori-Donati P et al: Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 12(6):303-8, 1996
- Kollias SS et al: Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics. 13(6):1211-31, 1993
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
- Classic Dandy-Walker (DW) malformation (DWM)
Synonyms
- Terms DW spectrum, DW variant, & DW complex have complicated clinical & imaging implications in literature
Definitions
- DW continuum represents clinically & radiologically heterogeneous group of posterior fossa (PF) malformations
- Referred to as continuum by some based on belief that DWM, vermian hypoplasia (VH), Blake pouch (BP) remnant/cyst, & mega cisterna magna (MCM) are spectrum of developmental anomalies of rhombencephalic vesicle roof
- Terminology controversial & not uniformly accepted
- Clinical severity ranges widely in this group
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Ultrasonographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Reporting Tips
Selected References
- Kau T et al: Blake's pouch cysts and differential diagnoses in prenatal and postnatal MRI : a pictorial review. Clin Neuroradiol. ePub, 2020
- Pertl B et al: The fetal posterior fossa on prenatal ultrasound imaging: normal longitudinal development and posterior fossa anomalies. Ultraschall Med. 40(6):692-721, 2019
- Spinelli M et al: The "vermian-crest angle": does it allow accurate categorisation of fetal upward rotation of cerebellar vermis on intrauterine MRI? A pilot study. Clin Radiol. 74(6):489.e1-7, 2019
- Bosemani T et al: Congenital abnormalities of the posterior fossa. Radiographics. 35(1):200-20, 2015
- Robinson AJ: Inferior vermian hypoplasia--preconception, misconception. Ultrasound Obstet Gynecol. 43(2):123-36, 2014
- Barkovich AJ et al: Pediatric Neuroimaging. 5th ed. Lippincott Williams & Wilkins. 477-9, 2012
- Guibaud L et al: Prenatal diagnosis of 'isolated' Dandy-Walker malformation: imaging findings and prenatal counselling. Prenat Diagn. 32(2):185-93, 2012
- Patek KJ et al: Posterior fossa anomalies diagnosed with fetal MRI: associated anomalies and neurodevelopmental outcomes. Prenat Diagn. 32(1):75-82, 2012
- Volpe P et al: Brainstem-vermis and brainstem-tentorium angles allow accurate categorization of fetal upward rotation of cerebellar vermis. Ultrasound Obstet Gynecol. 39(6):632-5, 2012
- Aldinger KA et al: FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nat Genet. 41(9):1037-42, 2009
- Barkovich AJ et al: A developmental and genetic classification for midbrain-hindbrain malformations. Brain. 132(12):3199-230, 2009
- Walbert T et al: Symptomatic neurocutaneous melanosis and Dandy-Walker malformation in an adult. J Clin Oncol. 27(17):2886-7, 2009
- Elsen GE et al: Zic1 and Zic4 regulate zebrafish roof plate specification and hindbrain ventricle morphogenesis. Dev Biol. 314(2):376-92, 2008
- Millen KJ et al: Cerebellar development and disease. Curr Opin Neurobiol. 18(1):12-9, 2008
- Boddaert N et al: Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology. 45(5):320-4, 2003
- ten Donkelaar HJ et al: Development and developmental disorders of the human cerebellum. J Neurol. 250(9):1025-36, 2003
- Patel S et al: Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol. 23(7):1074-87, 2002
- Tortori-Donati P et al: Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Mega cisterna magna and persisting Blake's pouch: two separate entities. Childs Nerv Syst. 12(6):303-8, 1996
- Kollias SS et al: Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis. Radiographics. 13(6):1211-31, 1993
