Hydrocephalus in vein of Galen malformation: etiologies and therapeutic management implications

Abstract

HC in VGM is a common phenomenon with several etiologies requiring different treatments. In most cases, embolization of the VGM as sole treatment is completely sufficient in order to decrease high venous pressure. However, certain other causes of HC should be treated in an interdisciplinary setting by specialized neurosurgeons.

Presentation, course, and outcome of postneonatal presentations of vein of Galen malformation: a large, single‐institution case series

“…Hydrocephalus in this context is thought to relate both to impairment to cerebrospinal fluid resorption in the high pressure venous system, but also to direct compression of the cerebral aqueduct by the VGM sac. 15 Of note, parents tell us that the significance of presenting features (macrocrania, prominent facial veins) often goes unrecognized for a long time, even by medical professionals. We had a minority of cases presenting with developmental arrest; however, none of these were associated with the catastrophic so-called 'melting brain syndrome' that indicates critically decompensated acute venous hypertension.…”

Late Discovery of a Vein of Galen Aneurysmal Malformation Complicated with Hydrocephalus: A Case Report

“…The mechanism of the hydrocephalus related to the malformation was not clarified in our case. It was due either to direct compression of the Sylvius' aqueduct by the dilated vein, or to an increase of intracranial pressure leading to a decrease in cerebrospinal fluid resorption [7]. Hydrocephalus may appear shortly after birth or in early childhood, often representing the first telltale sign of the malformation.…”

Hydrocephalus in a patient with an unruptured pial arteriovenous fistula: hydrodynamic considerations, endovascular treatment, and clinical course

“…Ventriculoperitoneal shunting prior to this exclusion decreases the pressure in the ventricles that may result in hygromas, subdural hemorrhage, 3 or hemorrhage as shown in different clinical scenarios with venous overload and associated hydrocephalus. 26,30,34,41 This paradigm shift is clearer in vein of Galen malformation treatment in which endovascular obliteration is the first-line option rather than direct management of the hydrocephalus by CSF diversion. 10,11,27 Endovascular treatment has been reported in other articles to be effective in the obliteration of the fistula, with excellent outcomes in most patients.…”