Vena-Galeni-Malformation – What does the stethoscope has to look on the head?

Aims: The Vena-Galeni-Malformation arises with a frequency of about 1:25000 caused by a regression error of the primitive Vena Markowski which serves as a central vessel until the 11th week. It mainly drains the strong arterial supply of the primitive Plexus choroideus and flows into the later Vena Galeni. If its regression and the acquisition of the functions through the veins within the brain doesn't take place, an arteriovenous short circuit develops with an aneurysmal enlargement of the primitive structure. Depending on the size of the shunt volume it leads to further brain parenchym lesions.

Already prenatal the diagnosis can be made sonographically. The clinic is determined depending on the degree of the shuntvolume of the AV-Malformation. The majority of patients (>60%) is striking through a high output cardiac failure in the newborn-age. This is shown among other things in Tachydyspnoe, increased tendency to sweat, weakness of drinking. But ultimately the cardiac failure can lead to a mutiple organ failure (renal or liver failure, respiratory failure). Clinical the diagnosis can be established by the auscultation of the fontanelle. The treatment happens neuro radiologic- interventionell.

Methods: We report on a twin premature infant of 35+5 SSW, on which prenatal it is situated the suspicion of a Vena-Galeni-Malformation. During the skull sonography a roundish echo-free structure with a diameter of 2cm, arteriel and venous flow profile, is represented dorsal of the third ventricle. The MR Angiography, the MRT native and with KM confirmed the suspicion of a Vena-Galenie-Malformation in the media section of the right temporal lobe. Clinical and echo cardiographic there were no signs of a cardiac failure in the course.

Results: A neuro radiologic- interventionall operation is pending to the present time.

Conclusion: The Vena-Galeni-Malformation can vary significantly interindividuel in its size. The diagnosis of a Vena-Galeni-Malformation requires a strict interdisciplinary cooperation. Time and kind of the endovascular embolization depend on the clinical symptomatolgy of the patient.