Clinical and imaging characteristics of children with Dandy-Walker syndrome before and after peritoneal shunt

Objective To summarize the clinical and imaging characteristics with Dandy-Walker syndrome (DWS) in children before and after peritoneal shunt. Methods and Results All 6 children with DWS admitted to Fujian Sanbo Funeng Brain Hospital from January 2016 to January 2023 were selected as the objects of observation, including one case of Dandy-Walker malformation (DWM) and 5 cases of Dandy- Walker variant (DWV). Before surgery, the main manifestations were cerebellar vermis defect, upward movement of the tentorial and sinusional area, severe dilatation of the fourth ventricle, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (one case); or cerebellar vermis dysplasia, cerebellar hemisphere compression, the fourth ventricle dilatation, posterior cranial fossa cystic lesions, supratentorial ventricle dilatation and hydrocephalus (5 cases). Right lateral ventriculoperitoneal shunt (2 cases) or posterior cranial fossa cyst-peritoneal shunt (4 cases) were performed, and the operations were successful without surgery - related complications. After 2.31-7.00 years of follow - up, 6 cases of hydrocephalus and posterior cranial fossa cystic malformation were gradually relieved, 3 cases of good prognosis, cerebral hemisphere and cerebellum development, speech, movement and intelligence normal; there were 3 children with general prognosis, including cerebellar disequilibrium symptoms (one case), autism, claustrophobia (one case), and speech, motor and intellectual disorders (one case) complicated with neurocutaneous melanosis. Conclusions The brain structure of the children with DWS presented with hydrocephalus can be well developed after the hydrocephalus is corrected by single shunt. Among them, the cerebellar vermis of the DWV is well developed, while the development of the DWM presented with cerebellar vermis defect needs to be observed in more cases.