Management und Outcome der Vena-Galeni-Malformation im Kindesalter

Zusammenfassung

Die Vena-Galeni-Malformation (VGM) ist eine seltene angeborene zerebrale Gefäßmalformation, welche heute überwiegend pränatal diagnostiziert wird. Die Prognose hängt maßgeblich davon ab, ob der arteriovenöse Shunt zu einer Herzinsuffizienz führt oder nicht. Dabei ist die Prognose der neonatal symptomatischen VGM mit frühzeitiger Herzinsuffizienz besonders schlecht. Da das Krankheitsbild selten ist und die Behandlung eine interdisziplinäre Zusammen-arbeit erfordert, ist es besonders wichtig, Patienten frühzeitig in einer Klinik zu behandeln, welche über ein Perinatalzentrum und eine interventionelle Neuroradiologie sowie über eine Neurochirurgie verfügt und in der Behandlung der VGM geübt ist. Dargestellt werden die Genese, die Pathophysiologie, die Diagnostik, das therapeutische Vorgehen und die Prognose der VGM.

Abstract

The vein of Galen malformation (VGM) is a rare congenital cerebral vascular malformation. In most cases it already has been diagnosed prenatally. The impact of heart failure due to the arteriovenous shunt is relevant for the prognosis.Prognosis of neonatal symptomatic VGM with early heart failure is severe. Therefore an interdisciplinary cooperation is necessary and it is important to treat patients in a clinic with a perinatal center and a neuroradiology and neurosurgery that is experienced in treating such patients. The genesis, the pathophysiology, the diagnostic agents, the therapeutic agents and the prognosis of VGM will be described.

Vena-Galeni-Malformation - kongestive Herzinsuffizienz - Therapieoptionen - Outcome

vein of Galen malformation - congestive heart failure - therapeutic options - outcome

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