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Web End = Acta Neurochir (2016) 158:12851288 DOI 10.1007/s00701-016-2837-x
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Hydrocephalus in vein of Galen malformation. Another paradigm shift in neurosurgery
Domenico dAvella1 & Francesco Causin2
Received: 30 April 2016 /Accepted: 4 May 2016 /Published online: 23 May 2016 # Springer-Verlag Wien 2016
The timely and due article by Meila et al. [1] addresses a topic of definite neurosurgical interest that, surprisingly, has been somehow neglected in the current neurosurgical literature. The aim of the article was to assess the evolution of treatment options for hydrocephalus in vein of Galen malformation (VGM) over the last few decades, with a particular emphasis on the dangerous implications of the old paradigm Bubi hydrocephalus ibi shunt^.
The vein of Galen is a short midline venous structure resulting from the confluence of the two internal cerebral veins and the basal vein of Rosenthal. It represents the caudal relic of the median prosencephalic vein, a centrally located vessel draining the choroid plexus. Malformations of the Galenic vein are believed to result from a dysembryogenic event involving the cerebral vasculature at between 6 and 11 weeks of gestation [2]. Although rare, VGM accounts for an estimated 30 % of all paediatric vascular anomalies [3]. VGM can have different clinical manifestations, in relation to the entity of the hydrodynamic changes in the venous and cerebrospinal fluid (CSF) compartments that they determine or to their mass effect. The shunt from cerebral arteries into a dilated vein of Galen can lead to an increased cardiac output and finally to heart failure. This scenario is more frequent in newborns. In infants and toddlers, the chief symptom associated with the presence of a VGM is ventricular dilation,
leading to hydrocephalus and macrocrania. Actually, obstructive or communicating hydrocephalus is present in a significant number of patients.
Noncommunicating hydrocephalus results from obstruction of the aqueduct of Sylvius by the mass effect of the aneurysmatic dilation of the VGM. Communicating hydro-cephalus is thought to be...