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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Tokumaru, A. M. Articles by Edwards, M. S. Search for Related Content PubMed PubMed Citation Articles by Tokumaru, A. M. Articles by Edwards, M. S. Hotlight (NEW!) What's Hotlight? American Journal of Neuroradiology, Vol 17, Issue 4 619-630, Copyright © 1996 by American Society of Neuroradiology ARTICLES Skull base and calvarial deformities: association with intracranial changes in craniofacial syndromes AM Tokumaru, AJ Barkovich, SF Ciricillo and MS Edwards Department of Radiology, University of California, San Francisco 94143, USA. PURPOSE: To analyze the skull and brain malformations in patients with craniofacial syndromes. METHODS: A retrospective analysis of imaging studies of 21 children with craniofacial anomalies (8 with Apert syndrome, 6 with Pfeiffer syndrome, 4 with Crouzon syndrome, 1 with Robert syndrome, 1 with Coffin-Lowry-syndrome, and 1 with Saethre- Chotzen syndrome) was carried out using CT (21 patients), MR imaging (9 patients), and MR venography (2 patients). A series of qualitative and quantitative assessments of the skull base and intracranial structures was performed. RESULTS: Skull base abnormalities were present in all patients. Intracranial abnormalities included ventriculomegaly, frank hydrocephalus, callosal anomalies, hypoplasia/absence of the septum pellucidum, hypoplasia/dysplasia of the hippocampus, dysplasias or distortions of the cerebral cortex, and parenchymal hemorrhage. The anomalies of the corpus callosum, septum pellucidum, and hippocampus appeared primary, whereas the others may have been the result of brain distortion by the calvarial anomaly. MR imaging was more useful than CT for evaluating brain abnormalities. In the two patients in whom it was performed, MR venography showed anomalies of the venous system, indicating that venous anomalies, possibly related to the skull base hypoplasia, may contribute to the intracranial abnormalities. CONCLUSION: A wide range of neuroimaging abnormalities are present in the craniofacial syndromes. Some of these are clearly primary, whereas others appear to be related to the small skull base and sutural synostoses. MR venography may prove useful in defining the cause of some of the associated anomalies. This article has been cited by other articles: P. M. Rich, T. C. S. Cox, and R. D. Hayward The Jugular Foramen in Complex and Syndromic Craniosynostosis and Its Relationship to Raised Intracranial Pressure AJNR Am. J. Neuroradiol., January 1, 2003; 24(1): 45 - 51. Abstract Full Text PDF C. D. Robson, J. B. Mulliken, R. L. Robertson, M. R. Proctor, D. Steinberger, P. D. Barnes, A. McFarren, U. Muller, and D. Zurakowski Prominent Basal Emissary Foramina in Syndromic Craniosynostosis: Correlation with Phenotypic and Molecular Diagnoses AJNR Am. J. Neuroradiol., October 1, 2000; 21(9): 1707 - 1717. Abstract Full Text L. H. Lowe, T. N. Booth, J. M. Joglar, and N. K. Rollins Midface Anomalies in Children RadioGraphics, July 1, 2000; 20(4): 907 - 922. Abstract Full Text PDF Home Subscribe Author Instructions Submit Online Search the AJNR Archives Feedback Help Copyright © 2010 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X
American Journal of Neuroradiology – American Journal of Neuroradiology
Published: Apr 1, 1996
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