DANDY WALKER MALFORMATION: A RARE CASE REPORT

2021 ◽  
pp. 71-72
Author(s):  
Triza Kumar Lakshman ◽  
Yeshwanthini J ◽  
Ravindra S Pukale
Keyword(s):  
Case Report ◽  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Rare Case ◽  
Male Fetus ◽  
Dandy Walker Malformation ◽  
Rare Case Report ◽  
Upward Displacement ◽  
Walker Malformation ◽  
Posterior Fossa Malformation

Dandy-Walker malformation (DWM) or syndrome is a posterior fossa anomaly characterized by agenesis or hypoplasia of the vermis and cystic enlargement of the fourth ventricle causing upward displacement of tentorium and torcula. Most patients have hydrocephalus at the time of diagnosis. DMW is the most common posterior fossa malformation, and it typically occurs sporadically. This is a case report of male fetus that was diagnosed to have Dandy Walker Malformation during the antenatal obstetric scans.

Dandy-Walker malformation with meningioma and lissencephaly: A rare case report

2015 ◽  
Vol 3 (1) ◽  
pp. 180
Author(s):  
Saryu Sain ◽  
Shrish Patil ◽  
S Vijaykumar ◽  
M Gurushanthaiah ◽  
G Mahesh
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Dandy Walker Malformation ◽  
Rare Case Report ◽  
Walker Malformation

scholarly journals Neurocutaneous melanosis with Dandy-Walker malformation: A rare case report

2016 ◽  
Vol 5 (1) ◽  
pp. 63
Author(s):  
Sandeep Velicheti ◽  
SonylalErwin Palaparthy ◽  
Rabiya Nelofer ◽  
Ashirwad Pasumarthy
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Neurocutaneous Melanosis ◽  
Dandy Walker Malformation ◽  
Rare Case Report ◽  
Walker Malformation

scholarly journals Hydrocephalus and Dandy- Walker Malformation: a review

2020 ◽  
Vol 2 (3(September-December)) ◽  
pp. e442020
Author(s):  
Patricia Alessandra Dastoli Dastoli ◽  
Jardel Mendonça Nicácio ◽  
Marcos Devanir Silva Da Costa ◽  
Italo Capraro Suriano ◽  
Marcia Cristina Da Silva ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Correct Diagnosis ◽  
Lateral Sinus ◽  
Dandy Walker Malformation ◽  
Superior Dislocation ◽  
Posterior Fossa Malformations ◽  
Magnetic Resonance Imaging Mri ◽  
Hydrocephalus Treatment ◽  
Walker Malformation ◽  
Posterior Fossa Malformation

Introduction: Dandy Walker malformation (DWM) is characterized by agenesis or hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle leading to an increase of the posterior fossa and superior dislocation of the lateral sinus, tentorium and torcula. Although it is the commonest posterior fossa malformation, its pathogenesis is still not fully understood, making the differential diagnosis with other posterior fossa malformations difficult and as a result the choice of therapeutic strategy. Material and methods: An extensive review of the literature relating to Dandy Walker malformation was performed. Historical, genetic, embryologic, epidemiologic, clinical and radiological presentation, treatment and prognosis were revised. Conclusion: The correct diagnosis of Dandy Walker malformation can be made through careful interpretation of magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) flow studies.  The choice of hydrocephalus treatment depends on whether there is aqueduct stenosis.  And, although ventriculoperitoneal (VP) shunts have been the treatment of choice for many years, neuroendoscopic techniques such as endoscopic thirdventriculostomy and stent placement are being frequently performed lately.

Recurrent Pseudo-TORCH Appearances of the Brain Presenting as “Dandy-Walker” Malformation

2012 ◽  
Vol 15 (1) ◽  
pp. 45-49 ◽  
Author(s):  
Marta C. Cohen ◽  
Ilgin Karaman ◽  
Waney Squier ◽  
Tom Farrel ◽  
Elspeth H. Whitby
Keyword(s):  
Posterior Fossa ◽  
Autosomal Recessive ◽  
Congenital Infection ◽  
Giant Cells ◽  
Cerebellar Vermis ◽  
Inflammatory Infiltration ◽  
Dandy Walker Malformation ◽  
Upward Displacement ◽  
Magnetic Resonance Imaging Mri ◽  
Walker Malformation

Dandy-Walker malformation (DWM) is a developmental abnormality characterized by cystic dilatation of the fourth ventricle, complete or partial agenesis of the cerebellar vermis, and enlarged posterior fossa with upward displacement of the lateral sinuses, tentorium, and torcula. Calcification of brain tissue is a feature of congenital infection, particularly those involving the TORCH ( Toxoplasma gondii, rubella virus, Cytomegalovirus, and herpesvirus) group. An autosomal-recessive congenital infection-like syndrome with intracranial and extracranial calcifications has been categorized as pseudo-TORCH syndrome. We describe two male siblings diagnosed as DWM by ultrasound and by in utero and postmortem magnetic resonance imaging (MRI) but in whom the neuropathology revealed features of intracranial necrosis and calcification in the absence of extracranial calcific deposition. The fetal anomaly was identified by routine prenatal ultrasound scan at 16 weeks. In both cases the postmortem MRI showed bilateral ventriculomegaly with distortion of the overlying cortices, enlarged posterior fossa with a cyst related to small cerebellar hemispheres, and an incomplete cerebellar vermis. The diagnosis of DWM was offered. The histology revealed hypoplastic cerebral hemispheres with poorly cellular developing cortex. The white matter and periventricular matrix were disrupted by areas of necrosis and calcification not associated with any inflammatory infiltration, organisms, inclusions, or giant cells. To our knowledge, these two male siblings are the 1st cases that show pseudo-TORCH syndrome with distinctive intracranial calcification presenting as DWM. An autosomal-recessive or X-linked inheritance needs to be considered. Our study confirms the relevance of the multidisciplinary teamwork involved in the diagnosis of these complex cases.

scholarly journals Dandy-Walker variant with precocious puberty: a rare association

2018 ◽  
Vol 11 (1) ◽  
pp. e226281
Author(s):  
Rajesh Rajput ◽  
Sanat Mishra ◽  
Parul Ahlawat ◽  
Pawan Kumar Yadav
Keyword(s):  
Case Report ◽  
Posterior Fossa ◽  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Secondary Sexual Characteristics ◽  
Rare Association ◽  
Dandy Walker Malformation ◽  
Secondary Hypothyroidism ◽  
Sexual Characteristics ◽  
Walker Malformation

Precocious puberty is characterised by premature appearance of secondary sexual characteristics before the age of 7 years in girls and 9 years in boys. Dandy-Walker malformation comprises a spectrum of intracranial malformations of the posterior fossa. We present a case of a 7-year-old male child who has presented with features of central precocious puberty and on further evaluation has been found to have Dandy-Walker variant and secondary hypothyroidism. The following case report describes this association which is extremely rare and has never been described in literature.

Cerebellar Agenesis: A Case to Remember

2019 ◽  
Vol 18 (01) ◽  
pp. 045-048
Author(s):  
John V. Dennison ◽  
Dharmesh R. Tailor
Keyword(s):  
Posterior Fossa ◽  
Congenital Anomalies ◽  
Rare Case ◽  
Genetic Causes ◽  
Cerebellar Function ◽  
Dandy Walker Malformation ◽  
Wide Range ◽  
Walker Malformation

AbstractThere is a wide range of congenital anomalies of the posterior fossa. Of these, absence of cerebellar components seems to exist on a spectrum, much like anomalies seen with the more frequently encountered Dandy–Walker malformation. Here, we present a rare case of cerebellar agenesis, as seen at our institution, which falls on the extreme side of the spectrum. We also discuss normal and aberrant posterior fossa development, established or hypothesized genetic causes of such development, and implications on cerebellar function.

scholarly journals Foxc1 dependent mesenchymal signalling drives embryonic cerebellar growth

eLife ◽  
2014 ◽  
Vol 3 ◽  
Author(s):  
Parthiv Haldipur ◽  
Gwendolyn S Gillies ◽  
Olivia K Janson ◽  
Victor V Chizhikov ◽  
Divakar S Mithal ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Fourth Ventricle ◽  
Ventricular Zone ◽  
Considerable Influence ◽  
Radial Glial Cells ◽  
Dandy Walker Malformation ◽  
Radial Glial ◽  
Walker Malformation ◽  
Embryonic Brain Development

Loss of Foxc1 is associated with Dandy-Walker malformation, the most common human cerebellar malformation characterized by cerebellar hypoplasia and an enlarged posterior fossa and fourth ventricle. Although expressed in the mouse posterior fossa mesenchyme, loss of Foxc1 non-autonomously induces a rapid and devastating decrease in embryonic cerebellar ventricular zone radial glial proliferation and concurrent increase in cerebellar neuronal differentiation. Subsequent migration of cerebellar neurons is disrupted, associated with disordered radial glial morphology. In vitro, SDF1α, a direct Foxc1 target also expressed in the head mesenchyme, acts as a cerebellar radial glial mitogen and a chemoattractant for nascent Purkinje cells. Its receptor, Cxcr4, is expressed in cerebellar radial glial cells and conditional Cxcr4 ablation with Nes-Cre mimics the Foxc1−/− cerebellar phenotype. SDF1α also rescues the Foxc1−/− phenotype. Our data emphasizes that the head mesenchyme exerts a considerable influence on early embryonic brain development and its disruption contributes to neurodevelopmental disorders in humans.

scholarly journals Hemorrhagic Stroke in a Young Adult with Undiagnosed Asymptomatic Dandy–Walker Malformation

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Haleem Abdul ◽  
Joseph Burns ◽  
Andrea Estevez ◽  
Carlos Nasr El-Nimer ◽  
Brinsley Ekinde ◽  
...  
Keyword(s):  
Young Adult ◽  
Fourth Ventricle ◽  
Hemorrhagic Stroke ◽  
Brainstem Infarction ◽  
Rare Presentation ◽  
Ischemic Event ◽  
Dandy Walker Malformation ◽  
Upward Displacement ◽  
Walker Malformation ◽  
Rare Opportunity

The Dandy–Walker Malformation was first described in 1914 by Dandy and Blackfan and is characterized by hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses, and anteroposterior enlargement of the posterior fossa. This syndrome commonly manifests as hydrocephalus in children, though rare adult cases have been reported. The literature reveals adult symptomatology including brainstem infarction, psychosis, and neuromuscular disease. Stroke is an exceptionally rare presentation of this malformation, with only one ischemic event reported in the literature. This case offers a rare opportunity for diagnosis in an adult presenting with a hemorrhagic stroke of the basal ganglia in an otherwise asymptomatic young adult male. To the best of our knowledge, this is the first reported case of a hemorrhagic stroke in an adult patient with Dandy–Walker Malformation.

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