Child's Nervous System

, Volume 35, Issue 3, pp 569–574 | Cite as

Congenital pial AVF along the falx cerebri with complete agenesis of the corpus callosum and bilateral parasagittal pachygyria-polymicrogyria secondary to chronic ischemia

  • Pei Ing Ngam
  • Syed Shahzad Hussain
  • Ai Peng TanEmail author
Case Report



Pial arteriovenous fistula (AVF) is an extremely rare entity due to direct arterial connection with the venous plexus without an intervening capillary network. The objective of this article is to describe a unique case of congenital pial AVF along the interhemispheric falx with complete callosal agenesis and malformation of cortical development within the bilateral anterior cerebral artery territories. We also demonstrated the distinctive feature of temporal stability of the extensive intracranial abnormalities without active intervention. Less than 100 cases have been reported thus far, most of which involve the adult rather than pediatric age group. A comprehensive literature review of congenital pial AVF will also be included.

Case description

A 5-year-old child presented with headache and complex partial seizures. Imaging of the brain revealed the presence of polymicrogyria-pachygyria in the parasagittal frontoparietal lobes with associated underlying white matter hypodensities. Complete agenesis of the corpus callosum was also seen. In addition, enlarged and tortuous vessels were noted along the interhemispheric falx with no appreciable nidus. Bilateral dilated and tortuous ACAs were seen supplying the network of abnormal vessels along the falx. The radiological findings were stable on a follow-up MRI 12 years later.


Our reported case adds to current limited knowledge of this rare entity in the pediatric age group, which is traditionally treated aggressively and urgently. Our case demonstrated temporal stability of this lesion with no detrimental complications observed. This suggests that the outcome of pial AVFs with conservative treatment may not be as grim as previously thought.


Pial arteriovenous fistula Vascular malformation Malformation of cortical development Agenesis of corpus callosum 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019
corrected publication 2019

Authors and Affiliations

  1. 1.Department of Diagnostic ImagingNational University Health SystemSingaporeSingapore
  2. 2.Yong Loo Lin School of MedicineNational University of Singapore (NUS)SingaporeSingapore

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