Hydrocephalus in Dandy–Walker malformation

Abstract

The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

Congenital Brain Malformations in the Neonatal and Early Infancy Period

“…The degree of cognitive impairment depends on other associated brain anomalies as well as adequate and early management of hydrocephalus. (49,52,53) Walker variant unless caused by another concurrent malformation (Fig. 24).…”

“…The falx cerebelli is usually absent. (49,51,52) Vermian hypoplasia is more common in Dandy Walker malformation than agenesis. When the large fourth ventricular cyst superiorly displaces the cerebellar vermis, the vermis appears to have rotated in a counterclockwise position (Fig.…”

“…More recently, an alternative hypothesis has challenged the current concept of cerebellar development and proposed that the pathogenesis of Dandy Walker malformation may be due to secondary degeneration or regression of the cerebellum at midline. (18,(49)(50)(51)(52) Increased pressure from dilatation of the fourth ventricle and cerebrospinal fluid pulsation cause bony changes in Dandy Walker malformation. There is scalloping of the occipital bone and petrous ridges.…”

“…(49,51,52) MRI is the imaging of choice for Dandy Walker malformation, though prenatal ultrasound can make the diagnosis as early as 14 weeks of gestation. Dandy Walker malformation is thought to result from arrested regression of the anterior membranous area.…”

Anaesthetic management for ventriculoperitoneal shunt insertion in an infant with Dandy–Walker Syndrome

“…These abnormalities are typically associated with hydrocephalus. 2 Patients often present in infancy for cerebrospinal fluid shunt procedures. Anaesthetic management of patients with DWS may prove challenging due to the frequent association of abnormalities in other systems.…”