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Congress: ECR25
Poster Number: C-26580
Type: Poster: EPOS Radiologist (educational)
DOI: 10.26044/ecr2025/C-26580
Authorblock: L. Topuria; Tbilisi/GE
Disclosures:
Lika Topuria: Nothing to disclose
Keywords: CNS, Interventional vascular, Neuroradiology brain, MR, MR-Angiography, MR-Diffusion/Perfusion, Ablation procedures, Angioplasty, Biopsy, Arteriovenous malformations, Embolism / Thrombosis, Grafts
Learning objectives Dandy-Walker malformation (DWM) is a generalised disorder of mesenchymal development that affects both the cerebellum and the overlying meninges. It is characterised by a large posterior fossa (PF) with a high-inserting venous sinus confluence, a large pia-ependyma-lined cyst extending dorsally from the fourth ventricle, and varying degrees of vermian and cerebellar hemispheric hypoplasia. [fig 1] Due to its overlapping features with other neurological conditions—such as mega cisterna magna, epidermoid cyst, retrocerebellar arachnoid cyst, Blake’s pouch cyst, Joubert anomaly, and isolated fourth...
Read more Background According to the relevant literature, case reports, and incorporating research studies, DWM is likely due to multifactorial causes and can be isolated or associated with chromosomal abnormalities, Mendelian disorders, syndromic malformations, congenital infections, and various other comorbidities. Rare mutations have been described in some genes including FOXC1 , ZIC1, and ZIC4, FGF17, LAMC1, and NID1. [fig 5] After a detailed investigation of the diagnostic findings, MRI appears to be the modality of choice for assessing Dandy-Walker malformation, although both CT and ultrasound...
Read more Findings and procedure details After an examination of MRI diagnostic images, the characteristic features of the disease became evident. The results underline that DWM is frequently associated with other CNS anomalies. Almost two-thirds of patients have grey matter abnormalities such as pachygyria, polymicrogyria, or heterotopic GM. [fig 6] Callosal dysgenesis is common and often results in widely separated lateral ventricles with prominent occipital horns, known as colpocephaly. In 75-90% of patients, obstructive hydrocephalus is evident, primarily due to aqueduct stenosis. MRI findings: Classically, DWM consists of...
Read more Conclusion Upon thorough evaluation of the characteristic features of Dandy-Walker malformation, this study underscores the pivotal role of MRI in accurately diagnosing the condition. While CT and ultrasound can assist in identifying DWM, MRI remains the gold standard for early investigation. It offers a comprehensive assessment of key anatomical abnormalities, including posterior fossa enlargement, vermian and cerebellar hypoplasia, and associated supratentorial anomalies. Additionally, MRI effectively determines the extent of hydrocephalus, callosal dysgenesis, and other CNS anomalies, making it paramaunt for guiding...
Read more References   Anne G. Osborn, Osborn's brain imaging: Pathology and anatomy, 2nd ed., Elsevier, 2017. Hamid, H.A., 2007. Dandy-Walker malformation. Egyptian Journal of Human Genetics, 8(2), pp.123-130. Gaillard F, Walizai T, Obaid A, et al. Dandy-Walker malformation. Reference article, Radiopaedia.org https://doi.org/10.53347/rID-4590. Incesu, L., 2023. Dandy-Walker malformation imaging. Medscape. [online] Available at: https://emedicine.medscape.com/article/408059-overview?form=fpf. Millen, K., & Hevner, R. F. (2018). Dandy-Walker malformation. In H. Biassette, B. N. Harding, & J. A. Golden (Eds.), Developmental neuropathology (2nd ed., pp. 345-368). Wiley-Blackwell. Barkovich, A.J., Kuzniecky, R.I., Jackson, G.D., Guerrini, R. & Dobyns, W.B., 2001. Classification...
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