Congenital cystic malformations

of the posterior fossa: diagnostic

approach
CLAUDIO ALEXANDER RIVAS PALACIOS
Resident of Neurosurgery
2022
 Objetive 

To review the radiological differences in the postnatal diagnostic approach to

congenital cystic malformations of the posterior fossa.
 Historical background
Dandy – Blackfan 1914
4th V  +  vermis 1
Taggart - Walker 1942
Report three cases Benda 1954
2 Dandy-Walker syndrome

Gibson, D’Agostino,
Hart (1965-1972) 3 Harwood‑Nash
and Fitz 1976
Triad of Dandy-Walker “Dandy–Walker variant”
malformation
4 Barkovich 1989
“Dandy–Walker complex”
Arachnoid cysts
Kollias 1993 5
“vermian‑cerebellar hypoplasia”
Tortori‑Donati 1996 
6 Blake’s pouch cyst (1900 JB)
Mega cisterna magna

Azab WA, Shohoud SA, Elmansoury TM, Salaheddin W, Nasim K, Parwez A. Blake's pouch cyst. Surg Neurol Int 2014;5:112.
Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Embryological development of the posterior
fossa

Carlson Bruce M. Human Embryology and Developmental Biology. 6th Edición (2018). Chap 11, Nervous system, pag 217.
Embryological development of the posterior
fossa

Metencephalon

Carlson Bruce M. Human Embryology and Developmental Biology. 6th Edición (2018). Chap 11, Nervous system, pag 239
Embryological development of the posterior
fossa

Metencephalon
Myelencephalon

Azab WA, Shohoud SA, Elmansoury TM, Salaheddin W, Nasim K, Parwez A. Blake's pouch cyst. Surg Neurol Int 2014;5:112.
 Abstract pathogenesis
Fusion of the cerebellar hemispheres:
formation of the vermis

Cranio-caudal development of the

Arrested development
vermis

DWM
Reabsorbtion of the AMA Persistence of the AMA

Reabsorbtion of the PMA: formation
Bulging of the PMA
opening of the foramen of Magendie

MCM Late permeabilization No permeabilization BPC

Adapted of De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
Classification of congenital malformations
of the PF

Mahalingam HV et al. Imaging spectrum of posterior fossa anomalies on foetal magnetic resonance imaging with an algorithmic approach to diagnosis. Pol J Radiol 2021; 86: e183-e194.
 Posterior Fossa Anatomy
Overview

Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.

 Posterior Fossa Anatomy
Overview

Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.

 Dandy-Walker Malformation
Definition:  cystic dilation of the fourth
ventricle associated with agenesis/hypoplasia
of the cerebellar vermis and elevation of the
tentorium.
Radiologic-Pathologic Findings PF: 
“Dandy-Walker variant,” “Dandy- Walker 1. Complete or partial agenesis of the
complex,” or “Dandy-Walker spectrum”: not vermis
meet the criteria for DWM (Blake pouch 2. Cystic dilatation of the fourth ventricle
cyst, megacistern magna and vermian 3. Enlargement of the posterior fossa with
hypoplasia) upward displacement of the lateral
Associated: hydrocephalus, macrocephaly sinuses, tentorium, and torcular
(dolichocephalic), supratentorial alterations
(dysgenesis of the corpus callosum),
neurocutaneous melanocytosis, occipital
meningocele
Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.
Yildiz H et al. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology (2006) 48: 595–605.
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
Dandy-Walker Malformation

Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Dandy-Walker Malformation

De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
Dandy-Walker Malformation

De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
 Vermian hypoplasia
Definition:  pathologic condition that is
characterized by partial absence of the
inferior portion of the cerebellar vermis
“Dandy-Walker variant” Radiologic-Pathologic Findings PF: 
1. Varying degrees of vermian and
Associated: with supratentorial abnormalities, cerebellar hypoplasia
similar to those seen in Dandy-Walker 2. Prominent retrocerebellar space of
malformations. Joubert syndrome, Walker- cerebrospinal fluid that communicates
Warburg syndrome freely with the 4th ventricle through a
prominent vallecula
More than 75% of patients have a favorable
3. Normal-sized posterior fossa
outcome

Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.
Yildiz H et al. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology (2006) 48: 595–605.
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
 Vermian Hypoplasia

De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
 Blake’s pouch cyst
Definition:  persistence of Blake pouch, for
lack of fenestration this, resulting in absence
of communication between the fourth
ventricle and the subarachnoid space Radiologic-Pathologic Findings PF: 
1. The presence of a cyst in a
"Dandy- Walker complex” “Dandy-Walker retrocerebellar or infraretrocerebellar
spectrum” location + enlarged fourth ventricle
Associated: tetraventricular hydrocephalus; 2. Normal Fastigium, vermis and tentorium
not supratentorial alterations (except
ventriculomegaly)

Azab WA, Shohoud SA, Elmansoury TM, Salaheddin W, Nasim K, Parwez A. Blake's pouch cyst. Surg Neurol Int 2014;5:112.
Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.
Yildiz H et al. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology (2006) 48: 595–605.
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
 Blake’s pouch cyst

Kau T et al. Blake’s Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI. Clin Neuroradiol (2020) 30:435–445
De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
 Blake’s pouch cyst

Kau T et al. Blake’s Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI. Clin Neuroradiol (2020) 30:435–445
De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
 Blake’s pouch cyst

Azab WA, Shohoud SA, Elmansoury TM, Salaheddin W, Nasim K, Parwez A. Blake's pouch cyst. Surg Neurol Int 2014;5:112.
 Megacistern magna
Definition:  is a developmental variation of
the posterior fossa characterized by a
retrocerebellar CSF space greater than 10 mm
in anteroposterior diameter with 4th Radiologic-Pathologic Findings PF: 
ventricle, vermis and cerebellar hemispheres 1. Expansion of the cisterna magna 
2. Free communication with the
are normal.
perimedullary subarachnoid space
The vast majority (90%–95%) of children 3. Morphologically intact vermis and
develop normally cerebellar hemispheres
Although MCM tends to have a lesser mass
effect

Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.
Yildiz H et al. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology (2006) 48: 595–605.
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
 Megacistern magna

Tortori-Donati R et al. Cystic malformations of the posterior cranial fossa originating from a defect of the posterior membranous area. Child's Nerv Syst (1996) 12:303-308.
De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
 Megacistern magna

Reith W and Haussmann A. Dandy-Walker-Malformation. Radiologe 2018, 58:629–635.

 Arachnoid cyst
Definition:  is an encapsulated collection of
cerebrospinal fluid trapped between
duplicated layers of the arachnoid membrane. Radiologic-Pathologic Findings PF: 
It can be retrocerebellar, supravermian, 1. Cerebrospinal fluid-like collection
lateral to cerebellar hemispheres, or anterior developing within the layers of the
to brainstem. arachnoid membrane
Mass effect on adjacent structures in the 2. Lack of communication with the
ventricular and perimedullary
form of displacement and flattening; The
subarachnoid space. *Communicating AC*
4th ventricle can be flattened or effaced 3. Morphologically intact vermis and
Scalloping of the occipital bone can cerebellar hemispheres
occasionally be seen

Kollias S et al. Cystic Malformations ofthe Posterior Fossa: Differential Diagnosis Clarified through Embryologic. RadloGraphics 1993; 13:1211-1231.
Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.
Yildiz H et al. Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging. Neuroradiology (2006) 48: 595–605.
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
 Arachnoid cyst

De Nardi S et al. Cystic lesion of posterior cranial fossa: is it Dandy-Walker? Journal of Pediatric and Neonatal Individualized Medicine 2018;7(1):e070104
Araujo E Júnior et al. Fetal posterior fossa malformations: review of the current knowledge. Radiol Bras. (2019) 52(6):380–386.
 Diagnostic approach

Kau T et al. Blake’s Pouch Cysts and Differential Diagnoses in Prenatal and Postnatal MRI. Clin Neuroradiol (2020) 30:435–445
 Diagnostic approach

Mahalingam HV et al. Imaging spectrum of posterior fossa anomalies on foetal magnetic resonance imaging with an algorithmic approach to diagnosis. Pol J Radiol 2021; 86: e183-e194.
 ¿What you should check?

Posterior fossa Vermis

-Size  -Size
-Retrocerebellar -Morphology

Four ventricle Supratentorial

-Torcula
-Size
-Associated malformation
-Tegmento vermian angle
-Hydrocephalus
Cystic lesion of posterior fossa: is it
Dandy-Walker?

Bosemani T et al. Congenital Abnormalities of the Posterior Fossa. RadioGraphics 2015; 35:200–220.

 ¿What is the most difficult?
"What seems simplest...to see what
is in front of one's eyes"

Johann Wolfgang Von

Goethe