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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
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: Sagittal view of the posterior fossae on histological sections or brain MRI. Histological sections stained with hematoxylin–eosin from a control at 18 WG (A) compared with those of Patients 1 (B), 2 (C), and 3 (D). Macroscopic view of a brain inter-hemispheric section from Patients 1 (E) and 2 (F). T1-weighted brain MRI in Patient 5 (G). Note the wide spectrum of Dandy–Walker malformation from a mild form with enlargement of the fourth ventricle (B,G) to a more severe form with posteroinferior vermis agenesis and enlargement of the fourth ventricle (C,D). Note also the short corpus callosum (E–G).
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 can also reveal the pertinent features. Since Dandy-Walker is truly a spectrum, there are many intermediate cases. From a clinical perspective, it is indispensable for the radiologist to specifically describe vermis, cerebellar structures, and any coexisting supratentorial anomalies. With the help of the Barkovich classification, which uses the development of the cerebellar vermis as the standard, the differentiation becomes clearer. According to this framework, the Dandy-Walker spectrum can be divided into Dandy-Walker malformation, Dandy-Walker variant, and simple posterior fossa cistern widening.
Fig 2: a) Surgical view of Dandy-Walker malformation. The thick, translucid membrane is the Dandy-Walker cyst wall. Cerebrospinal fluid is visible in the lower half of the cystic cavity. (b) Surgical view after complete removal of the cyst wall. (c) Explanatory version. F: floor of the third ventricle, CH: cerebellar hemispheres, A: aqueduct, V: remnants of the vermis (hypoplastic and rotated upward), TH: tentorial hiatus, G: vein of Galen, D: dura. Contributed by Jean-François Hirsch, M.D.
GALLERY