Dandy-Walker syndrome

Dandy–Walker syndrome (also known as Dandy–Walker malformation, DWS) is a rare congenital neurological disorder of the brain. The cause of this condition is the so‑called Dandy–Walker malformation, which consists of aplasia (absence of development) of the vermis, i.e., a specific part of the cerebellum, and the formation of a large cyst in the posterior cranial fossa that widely communicates with the fourth ventricle.

As a result of this congenital alteration of cerebellar tissue, membranous atresia (obstruction) of the outlets of the fourth cerebral ventricle occurs, leading to pathological accumulation of cerebrospinal fluid within the brain ventricles.

Treatment

Treatment consists of surgical removal of the obstruction during a neurosurgical procedure; associated hydrocephalus is most often managed by insertion of a shunt.

• typicky aplázia vermis cerebelli (vrodené nevyvinutie strednej nepárovej červovitej časti mozočku)
• cysta v zadnej jame lebečnej, ktorá komunikuje so IV. mozgovou komorou
• veľmi priestranná zadná jama lebečná
• u 90% detí rozvoj hydrocefalu - patologicky zvýšené množstvo mozgovomiechového moku v centrálnom nervovom systéme
• rastúci obvod hlavy (makrocefália)
• napätá veľká fontanela ( "mäkký vrcholok hlavičky")
• rozostup lebečných švov
• tenšia koža na hlave
• vracanie, dávenie
• deviácie očnej gule smerom nadol (tzv. príznak zapadajúceho slnka - anglicky setting-sun sign)
• niekedy až porucha vedomia
• poruchy dýchania
• až polovica postihnutých má zníženú inteligenciu