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NEUROLOGY 2009;72:e111
© 2009 American Academy of Neurology
Resident and Fellow Section

Teaching NeuroImages: Macrocephaly with subcortical calcifications in vein of Galen aneurysmal malformation

Suvasini Sharma, MD, Naveen Sankhyan, MD and Atin Kumar, MD

From the Division of Pediatric Neurology, Department of Pediatrics (S.S., N.S.), and Department of Radiodiagnosis (A.K.), All India Institute of Medical Sciences, New Delhi, India.

Address correspondence and reprint requests to Dr. Naveen Sankhyan, Senior Resident, Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029 India drnsankhyan{at}yahoo.co.in

A previously asymptomatic 3-year-old boy presented with a large head, generalized seizures, and vision impairment for 7 months. Examination revealed macrocephaly (52 cm) and prominent scalp and facial veins. There was bilateral disc pallor and sixth nerve palsies. Developmental assessment and the remainder of the neurologic and systemic examinations were unremarkable. Neuroimaging led to a diagnosis of vein of Galen aneurysmal malformation (VGAM) (figures 1 and 2).


Figure 118
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  		Figure 1 CT brain in vein of Galen aneurysmal malformation

Noncontrast (A) CT axial image of brain showing bilaterally symmetric calcifications in basal ganglia and subcortical white matter (black arrow). The hyperdense (white arrow) dilated vein of Galen shows intense enhancement in the contrast-enhanced CT (B).

 


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  		Figure 2 Magnetic resonance venography shows the dilated vein of Galen

 
The clinical presentation in VGAM is either related to the high output cardiac failure or cerebral venous congestion and abnormal CSF flow. In untreated VGAMs, ischemia due to venous congestion in watershed region results in development of subcortical white matter calcifications and subependymal atrophy with ventricular dilation.1

Disclosure: The authors report no disclosures.


   REFERENCE
Top.
 REFERENCE
 

  1. Alvarez H, Monaco G, Rodesch G, Sachet M, Krings T, Lasjaunias P. Vein of Galen aneurysmal malformations. Neuroimaging Clin N Am 2007;17:189–206.[Medline]




This Article
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PubMed
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Right arrow Articles by Sharma, S.
Right arrow Articles by Kumar, A.
Related Collections
Right arrow CT
Right arrow MRI
Right arrow Pediatric stroke; see Cerebrovascular Disease/ Childhood stroke
Right arrow Arteriovenous malformation
Right arrow Infarction

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