Vein of Galen malformation

Case contributed by Dr Mahmoud Yacout Alabd

Presentation

Motor weakness and delayed development.

Patient Data

Age: 2 years
Gender: Female
Modality: MRI

Large venous malformation of the vein of Gallen, showing early arterial filling on the MRA. It is fed by the right posterior circulation as we see the right PCA (posterior cerebral artery) and SCA (superior cerebellar artery) significantly prominent in caliber, the basillar artery and both vertebral arteries are also prominent. The direct feeding branches are mainly the posterior choroidal and thalamoperforating arteries, both branches of the right PCA. The draining venous sinuses namely the straight, right transverse and sigmoid sinuses are also prominent. The T2 and FLAIR scans show diffuse prominence of the cortical and parenchymal arterial circulation as well as associated cerebral atrophy and abnormal white matter signal and gliosis denoting chronic global cerebral ischemia. These changes are most likely due to hyperdynamic shunting with steel phenomenon. Interestingly the subcortical and periventricular patches of gliosis are showing mrginal enhancement on post contrast study likley due to sever gliosis and ongoing ischemia together with the abnormal cerebral hemodynamics.

Case Discussion

The vein of Gallen malformation, also know as median prosencephalic arteriovenous fistula,  is a congenital condition caused by abnormal development and failure of regression of the median prosencephalic vein, which is the precursor of the vein of Gallen, leading to AVF formation.

This case is showing early arterial filling on the MRA. The AVF is fed by the right posterior circulation as we see the right PCA (posterior cerebral artery) and SCA (superior cerebellar artery) significantly prominent in caliber, the basillar artery and both vertebral arteries are also prominent. The direct feeding branches are mainly the posterior choroidal and thalamoperforating arteries, both branches of the right PCA. The draining venous sinuses namely the straight, right transverse and sigmoid sinuses are also prominent.

The T2 and FLAIR scans show diffuse prominence of the cortical and parenchymal arterial circulation as well as associated cerebral atrophy and abnormal white matter signal and gliosis denoting chronic global cerebral ischemia. These changes are most likely due to hyperdynamic shunting with steel phenomenon. Interestingly the subcortical and periventricular patches of gliosis are showing mrginal enhancement on post contrast study likley due to sever gliosis and ongoing ischemia together with the abnormal cerebral hemodynamics.

PlayAdd to Share

Case Information

rID: 40600
Case created: 27th Oct 2015
Last edited: 2nd Nov 2015
System: Paediatrics
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.