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    Go to the top of the page   ID: 20041021162722 Original case in english  More links about this topic on Pubmed (PubMed Reader)
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    | The Authors | Discussion | Write a Comment to this Case |
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    Vein of Galen Malformation
    G Hahn. Vein of Galen Malformation. PedRad [serial online] vol 4, no. 10.
    URL: www.PedRad.info/?search=20041021162722


     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     Pediatric Radiology CasesAuthor/s:

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

     
     Pediatric Radiology CasesEmail Address:

    Viewable for logged on visitors (Log on)  

     
     Pediatric Radiology CasesAge:

    0 Months  

     
     Pediatric Radiology CasesGender:

    Female  

     
     Pediatric Radiology CasesRegion-Organ:

    Head-Brain and brain nerves  

     
     Pediatric Radiology CasesMost likely etiology:

    congenital  

     
     Pediatric Radiology CasesHistory:

    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.  

     
     Pediatric Radiology CasesPathomorphology or Pathophysiology of this disease :

    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.  

     
     Pediatric Radiology CasesRadiological findings:


    MRI 1 <- 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.






    MRI 2 <- 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.






    MRI 3 <- 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.






    MRI 4 <- 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.






    MRI 5 <- 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).






    MRI 6 <- 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.






    MRI 7 <- 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.






    MRI 8 <- 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.



     

     
     Pediatric Radiology CasesDiagnosis confirmation:

    Total constellation (Consens)  

     
     Pediatric Radiology CasesWhich DD would be also possible with the radiological findings:

    Cavum veli interposition,Arachnoidalcyst  

     
     Pediatric Radiology CasesCourse / Prognosis / Frequency / Other :

    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.  

     
     Pediatric Radiology CasesComments of the author about the case:

    N/A  

     
     Pediatric Radiology CasesFirst description / History:

    N/A  

     
     Pediatric Radiology CasesLiterature:

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

     
     Pediatric Radiology CasesKeywords:

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

     
     Pediatric Radiology Cases Cite this article:

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

     
     Pediatric Radiology Cases Read similar articles: with corresponding keywords
    in the same field: Head-Brain and brain nerves
    or in the region: Head
    or in the tissue/organ: Brain and brain nerves
    or with the etiology: congenital
     
     Pediatric Radiology CasesImages to this case: There are MRI-images available for this case. [ MRI ] View all modalities [ All ]   
     
    Vein of Galen Malformation
    G Hahn. Vein of Galen Malformation. PedRad [serial online] vol 4, no. 10.
    URL: www.PedRad.info/?search=20041021162722


     

    Search similar cases in:
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    Vein of Galen Malformation
    Other cases by these authors:

    Search G. Hahn in Medline G. Hahn (22)   

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


     

    Which diagnosis have other collegues guessed?


    • Cavum veli interposition
      Votes: 1 (8 %)


    • Cavum vergae
      Votes: 1 (8 %)


    • Cavum septi pellucidum
      Votes: 0 (0 %)


    • Aneurisma venae Galeni
      Votes: 9 (75 %)


    • Ectasia of the Vena cerebri interna
      Votes: 1 (8 %)


    • Arachnoidalcyst
      Votes: 0 (0 %)



        Total answers: 12

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


     

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


     




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