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Revised Classification of Posterior Fossa Cysts and Cystlike Malformations Based on the Results of Multiplanar MR Imaging

A. James Barkovich, Bent O. Kjos, David Norman and Michael S. Edwards
American Journal of Neuroradiology September 1989, 10 (5) 977-988;
A. James Barkovich
1 Department of Radiology, Letterman Army Medical Center, Presidio of San Francisco, CA 94129-6700. Address reprint requests in care of Medical Editing HSHH-CI-ME, Department of Clinical Investigation, Letterman Army Medical Center, Presidio of San Francisco, CA 94129-6700.
2 Department of Radiology, Neuroradiology Section, University of California School of Medicine, San Francisco, CA 94143.
3 Department of Neurosurgery, Division of Pediatric Neurosurgery, University of California School of Medicine, San Francisco, CA 94143.
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Bent O. Kjos
2 Department of Radiology, Neuroradiology Section, University of California School of Medicine, San Francisco, CA 94143.
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David Norman
2 Department of Radiology, Neuroradiology Section, University of California School of Medicine, San Francisco, CA 94143.
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Michael S. Edwards
3 Department of Neurosurgery, Division of Pediatric Neurosurgery, University of California School of Medicine, San Francisco, CA 94143.
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Abstract

MR and clinical data on 31 patients with posterior fossa CSF collections were analyzed. A clear separation of these patients into classical categories was not possible because of new information obtained from the MR images. We present a new classification of these disorders. The Dandy-Walker malformation, Dandy-Walker variant, and mega-cisterna magna seem to represent a continuum of developmental anomalies of the posterior fossa. A possible embryologic basis for this continuum is suggested. Discrete posterior fossa CSF collections that are clearly separate from the fourth ventricle and vallecula are classified as posterior fossa cysts. Posterior fossa CSF collections that communicate with the fourth ventricle and are associated with cerebellar atrophy are classified as prominent cisterna magna. Both the Dandy-Walker complex and posterior fossa cysts can cause enlargement of the posterior fossa and scalloping of the inner table of the occipital bone. The Dandy-Walker complex presents with seizures, developmental delay, and enlarging head size; it requires CSF diversion when associated with hydrocephalus. Posterior fossa cysts present with symptoms of a posterior fossa mass; they generally require surgical resection. Prominent cisterna magna is a result of degenerative disorders and requires no surgical therapy. This new classification facilitates both diagnosis and therapy of these disorders.

MR revealed that disorders previously referred to as the Dandy-Walker malformation, the Dandy-Walker variant, and the mega-cisterna magna actually are not separate entities, but appear to represent steps on a continuum of developmental anomalies of the posterior fossa. Because of this, we suggest a new term, the Dandy-Walker complex, be used to describe this continuum.

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American Journal of Neuroradiology
Vol. 10, Issue 5
1 Sep 1989
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A. James Barkovich, Bent O. Kjos, David Norman, Michael S. Edwards
Revised Classification of Posterior Fossa Cysts and Cystlike Malformations Based on the Results of Multiplanar MR Imaging
American Journal of Neuroradiology Sep 1989, 10 (5) 977-988;

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Revised Classification of Posterior Fossa Cysts and Cystlike Malformations Based on the Results of Multiplanar MR Imaging
A. James Barkovich, Bent O. Kjos, David Norman, Michael S. Edwards
American Journal of Neuroradiology Sep 1989, 10 (5) 977-988;
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