Hydrocephalus and the Dandy-Walker Malformation

The Most Common Comorbidities in Dandy-Walker Syndrome Patients: A Systematic Review of Case Reports

“…Dandy-Walker Syndrome (DWS) is a rare congenital posterior fossa malformation, reported in only 1 in 25-30,000 live births, which occurs during the embryonic development of the cerebellum and 4 th ventricle 1,2 . There is an ongoing debate among researchers when it comes to categorizing the variant forms of DWS; the most acceptable classification has been Barkovich's which identifies two types to the DW complex , A and B, wherein A split into the classic DW malformation and the DW variant, and B comprises the DW "mega cisterna magna" 1,3 .…”

“…There is an ongoing debate among researchers when it comes to categorizing the variant forms of DWS; the most acceptable classification has been Barkovich's which identifies two types to the DW complex , A and B, wherein A split into the classic DW malformation and the DW variant, and B comprises the DW "mega cisterna magna" 1,3 . The aforementioned classification is now in dispute and some of these terms, like "variant" or "complex" , are largely avoided .Most cases of DWS are sporadic.…”

“…Hydrocephalus is a common complication of the disease (in almost 80% of the cases) 1,3 . Dandy-Walker malformation could be diagnosed before birth sonographically (by identifying certain sonographic features such as the size of ventricles or measurements of the brainstem-vermis angle) 6,7 but the majority of cases seem to be diagnosed postnatally and, in fact, before the age of 1-year-old.…”

“…Other less common signs include focal neurologic signs such as strabismus, nystagmus and palsies of cranial nerves , truncal ataxia and speech difficulties 1,9,10 . Some patients with DWS may also suffer from seizures which are usually associated with supratentorial malformations 11 .…”

“…Further, a seemingly high percentage of children with DWS have dysmorphic characteristics 15 , other malformations of the Central Nervous System (agenesis of the corpus callosum, rachischisis, ectopic brain or cerebellar tissue) 1,16 , as well as disorders of the cardiovascular 17 , urogenital 18 , and gastrointestinal systems 19 . Multiple types of neoplasms and neurocutaneous disorders have also been observed in variable frequencies 20,21 .…”