Skip to main content
Advertisement

Main menu

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home

User menu

  • Alerts
  • Log in

Search

  • Advanced search
American Journal of Neuroradiology
American Journal of Neuroradiology

American Journal of Neuroradiology

ASHNR American Society of Functional Neuroradiology ASHNR American Society of Pediatric Neuroradiology ASSR
  • Alerts
  • Log in

Advanced Search

  • Home
  • Content
    • Current Issue
    • Accepted Manuscripts
    • Article Preview
    • Past Issue Archive
    • Video Articles
    • AJNR Case Collection
    • Case of the Week Archive
    • Case of the Month Archive
    • Classic Case Archive
  • Special Collections
    • AJNR Awards
    • Low-Field MRI
    • Alzheimer Disease
    • ASNR Foundation Special Collection
    • Photon-Counting CT
    • View All
  • Multimedia
    • AJNR Podcasts
    • AJNR SCANtastic
    • Trainee Corner
    • MRI Safety Corner
    • Imaging Protocols
  • For Authors
    • Submit a Manuscript
    • Submit a Video Article
    • Submit an eLetter to the Editor/Response
    • Manuscript Submission Guidelines
    • Statistical Tips
    • Fast Publishing of Accepted Manuscripts
    • Graphical Abstract Preparation
    • Imaging Protocol Submission
    • Author Policies
  • About Us
    • About AJNR
    • Editorial Board
    • Editorial Board Alumni
  • More
    • Become a Reviewer/Academy of Reviewers
    • Subscribers
    • Permissions
    • Alerts
    • Feedback
    • Advertisers
    • ASNR Home
  • Follow AJNR on Twitter
  • Visit AJNR on Facebook
  • Follow AJNR on Instagram
  • Join AJNR on LinkedIn
  • RSS Feeds

AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

LetterLetter

MR Angiography of a 1-Year-Old Girl with Semilobar Holoprosencephaly

C.K. Byun, K.H. Chao, M.S. Tenner, J.Y. Hu and G.K. Paek
American Journal of Neuroradiology April 2009, 30 (4) e56; DOI: https://doi.org/10.3174/ajnr.A1391
C.K. Byun
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
K.H. Chao
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
M.S. Tenner
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
J.Y. Hu
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
G.K. Paek
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF
Loading

Holoprosencephaly encompasses a variety of brain malformations and is thought to be a result of failure of cleavage of the prosencephalon into the diencephalon and telencephalon and/or stemming from a defect in the normal induction and patterning of the rostral neural tube, which normally occurs in the first 4 weeks after conception.1 We present a case of semilobar holoprosencephaly in a 1-year-old girl, with emphasis on the arterial vasculature demonstrated by MR angiographic (MRA) imaging.

A 1-year-old girl with a history of developmental delay presented with failure to thrive and decreased oral intake. Physical examination showed a microcephalic infant with right microphthalmos, bilateral colobomas, and poor muscle strength and tone. MRA of the head demonstrated paired internal carotid arteries, each of which bifurcated in a roughly symmetric manner. The bilateral bifurcating vessels supplied the frontoparietal cortices and probably represented analogues of the anterior and middle cerebral arteries. The segmental arteries coursed anteriorly and laterally but did not cross the midline; there was no anterior communicating artery. The posterior circulation was unremarkable, and a left posterior communicating artery was present (Figs 1 and 2).

Holoprosencephaly has classically been categorized as lobar, semilobar, or alobar, in order of increasing severity of manifestation. Although the anatomic features of holoprosencephaly have been well described, the accompanying anomalous cerebral vasculature has only been sporadically demonstrated in the literature via angiographic case reports2 and schematics from an autopsy study.3 van Overbeeke et al3 described 3 different vascular patterns in the anterior part of the circle of Willis in the autopsy study. The first pattern (type A) is essentially normal, with a closed anterior part of the circle of Willis. The second pattern (type B) describes roughly symmetric paired internal carotid arteries supplying the cortical surface, giving rise to smaller vessels, but failing to cross the midline; no anterior communicating artery is present. The third pattern (type C) describes a dominant internal carotid artery and an azygous anterior cerebral artery, which supplies most of the cortical surface. The 3 patterns roughly correlate with the degree of holoprosencephalic severity, with type A occurring in arrhinencephalic brains and type C occurring in both semilobar and alobar holoprosencephalic brains. The vascular pattern shown in this case study corresponds to the type B vascular pattern.

The failure of prosencephalon cleavage leading to holoprosencephaly occurs at 3 to 4 weeks after conception, whereas the development of anterior and middle cerebral arteries occurs at approximately 4 to 7 weeks.4 Therefore, the anomalous vascular patterns found in holoprosencephalic brains likely reflect an adaptation of embryologic cerebrovascular growth to early anatomic malformations in the developing brain. Specifically, the anterior communicating artery forms at 7 weeks from plexiform anastomoses between the 2 anterior cerebral arteries. The failure of prosencephalon cleavage likely results in nonformation of the anterior communicating artery because of aberrant anatomy of the underlying brain parenchyma.

Fig 1.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 1.

Axial view of maximum intensity projection from 3D time-of-flight MR angiogram of the head demonstrates paired internal carotid arteries bifurcating into segmental arteries, representing analogues of the anterior and middle cerebral arteries. No anterior communicating artery is present. The posterior circulation is unremarkable.

Fig 2.
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig 2.

Coronal view of maximum intensity projection from gadolinium MR angiogram of the head and neck demonstrates paired internal carotid arteries bifurcating into segmental arteries, representing analogues of the anterior and middle cerebral arteries.

References

  1. ↵
    Golden JA. Towards a greater understanding of the pathogenesis of holoprosencephaly. Brain Develop 1999;21:513–21
    CrossRefPubMed
  2. ↵
    Maki Y, Kumagai K. Angiographic features of alobar holoprosencephaly. Neuroradiology 1974;6:270–76
    CrossRefPubMed
  3. ↵
    van Overbeeke JJ, Hillen M, Vermeij-Keers CH. The arterial pattern at the base of arhinencephalic and holoprosencephalic brains. J Anat 1994;185:51–63
    PubMed
  4. ↵
    Okahara M, Kiyosue H, Mori H, et al. Anatomic variations of the cerebral arteries and their embryology: a pictorial review. Eur Radiology 2002;12:2548–61
    PubMed
  • Copyright © American Society of Neuroradiology
PreviousNext
Back to top

In this issue

American Journal of Neuroradiology: 30 (4)
American Journal of Neuroradiology
Vol. 30, Issue 4
April 2009
  • Table of Contents
  • Index by author
Advertisement
Print
Download PDF
Email Article

Thank you for your interest in spreading the word on American Journal of Neuroradiology.

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
MR Angiography of a 1-Year-Old Girl with Semilobar Holoprosencephaly
(Your Name) has sent you a message from American Journal of Neuroradiology
(Your Name) thought you would like to see the American Journal of Neuroradiology web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Cite this article
C.K. Byun, K.H. Chao, M.S. Tenner, J.Y. Hu, G.K. Paek
MR Angiography of a 1-Year-Old Girl with Semilobar Holoprosencephaly
American Journal of Neuroradiology Apr 2009, 30 (4) e56; DOI: 10.3174/ajnr.A1391

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
0 Responses
Respond to this article
Share
Bookmark this article
MR Angiography of a 1-Year-Old Girl with Semilobar Holoprosencephaly
C.K. Byun, K.H. Chao, M.S. Tenner, J.Y. Hu, G.K. Paek
American Journal of Neuroradiology Apr 2009, 30 (4) e56; DOI: 10.3174/ajnr.A1391
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  • Tweet Widget
  • Facebook Like
  • Google Plus One
Purchase

Jump to section

  • Article
    • References
  • Figures & Data
  • Info & Metrics
  • Responses
  • References
  • PDF

Related Articles

  • No related articles found.
  • PubMed
  • Google Scholar

Cited By...

  • No citing articles found.
  • Crossref (5)
  • Google Scholar

This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking.

  • Head and Neck Imaging
    Thomas P. Naidich, Susan I. Blaser, Ruby J. Lien, David G. Mclone, Girish M. Fatterpekar, Bruce S. Bauer
    2011
  • “Twig-like” cerebral vessels are not pathognomonic for ACTA A2 mutations: A case report
    Krishnan Nagarajan, Elango Swamiappan, Sathiaprabhu Anbazhagan, Ashwin Dalal, Subathra Adithan, Timo Krings
    Interventional Neuroradiology 2018 24 4
  • Variant course of bilateral anterior cerebral artery in semilobar holoprosencephaly
    Hima Pendharkar, Bhaskar Madivala Venkateshappa, Chandrajit Prasad
    Surgical and Radiologic Anatomy 2015 37 10
  • Diagnostic Imaging: Pediatrics
    2017
  • Dual white matter pathology in fetal holoprosencephaly featuring concurrent malformative and destructive features: A case series
    Sumit Das, Lindsay Brown, Sarah M Nikkel, Jessica Saunders, Christopher Dunham
    Journal of Neuropathology & Experimental Neurology 2024 83 9

More in this TOC Section

  • Letter to the Editor regarding “Automated Volumetric Software in Dementia: Help or Hindrance to the Neuroradiologist?”
  • Reply:
  • Brain AVM’s Nidus: What if We Hadn’t Understood Anything?
Show more LETTERS

Similar Articles

Advertisement

Indexed Content

  • Current Issue
  • Accepted Manuscripts
  • Article Preview
  • Past Issues
  • Editorials
  • Editor's Choice
  • Fellows' Journal Club
  • Letters to the Editor
  • Video Articles

Cases

  • Case Collection
  • Archive - Case of the Week
  • Archive - Case of the Month
  • Archive - Classic Case

More from AJNR

  • Trainee Corner
  • Imaging Protocols
  • MRI Safety Corner
  • Book Reviews

Multimedia

  • AJNR Podcasts
  • AJNR Scantastics

Resources

  • Turnaround Time
  • Submit a Manuscript
  • Submit a Video Article
  • Submit an eLetter to the Editor/Response
  • Manuscript Submission Guidelines
  • Statistical Tips
  • Fast Publishing of Accepted Manuscripts
  • Graphical Abstract Preparation
  • Imaging Protocol Submission
  • Evidence-Based Medicine Level Guide
  • Publishing Checklists
  • Author Policies
  • Become a Reviewer/Academy of Reviewers
  • News and Updates

About Us

  • About AJNR
  • Editorial Board
  • Editorial Board Alumni
  • Alerts
  • Permissions
  • Not an AJNR Subscriber? Join Now
  • Advertise with Us
  • Librarian Resources
  • Feedback
  • Terms and Conditions
  • AJNR Editorial Board Alumni

American Society of Neuroradiology

  • Not an ASNR Member? Join Now

© 2025 by the American Society of Neuroradiology All rights, including for text and data mining, AI training, and similar technologies, are reserved.
Print ISSN: 0195-6108 Online ISSN: 1936-959X

Powered by HighWire