G. Hahn (Bereich Kinderradiologie/Uniklinikum Dresden/Deutschland)  

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0 Months  

Female  

Head-Brain and brain nerves  

congenital  

Already intrauterine suspicion of intracranial dysplasia. Postnatal development of a severe heart insufficiency and multi organ failure, which was the reason why the child subsequently died.  

The genesis of this dysplasia is not clear. It is a connection between arteries (thalamostriatal,choroidal and anterior cerebral arteries) and the Vena Galeni or other primitive middle line veins. Large or multiple small fistulas or combinations of both may exist.  

<- view MRI 1

MRI 1: Signal void in the vessels, plane level under the Vena Galeni: In the transversal T2-wighted and proton dense weighted sequences are visible: A. carotis interna bilat., the A. vertebralis bilat. and the A. basilaris with relatively for the age of the child significant enlarged caliber and pronounced Flow void phenomenon.
Dilated Sinus rectus (ca. 1 cm transversal).
The Confluenc sinuum also shows a pronounced dilatation and drains in a widened Sinus transversus, sigmoideus, occipitalis bilat. and further in the great jugular veins.






<- view MRI 2

MRI 2: Signal void in the vessels, plane level under the Vena Galeni: In the transversal T2-wighted and proton dense weighted sequences are visible: A. carotis interna bilat., the A. vertebralis bilat. and the A. basilaris with relatively for the age of the child significant enlarged caliber and pronounced Flow void phenomenon.
Dilated Sinus rectus (ca. 1 cm transversal).
The Confluenc sinuum also shows a pronounced dilatation and drains in a widened Sinus transversus, sigmoideus, occipitalis bilat. and further in the large jugular veins bilateral.






<- view MRI 3

MRI 3: Pronounced dilatation and extensive Flow void phenomenon in the Vena galeni (ca. 2 x 1 ½ cm transversal), which drains in a dilated Sinus rectus (ca. 1 cm transversal).
The Confluence sinuum shows also a significant dilatation and drains in a widened Sinus transversus.






<- view MRI 4

MRI 4: Pronounced dilatation and extensive Flow void phenomenon in the Vena galeni (ca. 2 x 1 ½ cm transversal), which drains in a dilated Sinus rectus (ca. 1 cm transversal).
The Confluence sinuum shows also a significant dilatation. Multiple large caliber arteries from the supply area of the A. cerebri posterior bilateral flow into the dilated Vena galeni.






<- view MRI 5

MRI 5: T1 weighted coronal slice (GE).
Significant dilatation und pronounced Flow void phenomenon in the Vena galeni (ca. 2 x 1 ½ cm transversal), which drains into a dilated Sinus rectus (ca. 1 cm transversal).






<- view MRI 6

MRI 6: Arterial TOF-MR-Angiography: 3D-Reconstruction: Vena galeni (ca. 2 x 1 ½ cm transversal). Multiple large caliber arteries from the supply area of the A. cerebri posterior bilateral flow into the dilated Vena galeni.






<- view MRI 7

MRI 7: Arterial TOF-MR-Angiography: 3D-Reconstruction: Vena galeni (ca. 2 x 1 ½ cm transversal). Multiple large caliber arteries from the supply area of the A. cerebri posterior bilateral flow into the dilated Vena galeni.






<- view MRI 8

MRI 8: Venous TOF-MR-Angiography: 3D-Reconstruction: Vena galeni (ca. 2 x 1 ½ cm transversal), which drains into a dilated Sinus rectus (ca. 1 cm transversal).
The Confluence sinuum shows also a significant dilatation and drains in a widened Sinus transversus, sigmoideus, occipitalis bilat. and further into the large jugular veins bilateral.



 

Total constellation (Consens)  

Cavum veli interposition,Arachnoidalcyst  

Prognosis without therapy is 90% letal, with surgery almost 100% letal, with embolisation.
After consultation with Prof. Lasjaunias in Paris was - regarding the weight (2500g) of the child and the severe heart insufficiency - a surgical intervention no option.  

N/A  

N/A  

1. Medline:
"Pediatric Neuroimaging", A.J.Barkovich  

Vein of Galen, Vena Galeni, malformation, postnatal heart insufficiency, multi organ failure, Vein of Galen Malformation, child, childhood, pediatric radiology  

G Hahn. Vein of Galen Malformation. PedRad [serial online] vol 4, no. 10.
URL: www.PedRad.info/?search=20041021162722