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This Article Figures Only Full Text Full Text (PDF) CME: Take the course for this article: Volume 71, Number 5, July 29, 2008 All Versions of this Article: 01.wnl.0000304087.91204.95v1 71/5/351    most recent Correspondence: Submit a response Correspondence: View responses Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Services Email this article to a friend 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 Google Scholar Google Scholar Articles by Goh, S. Articles by Wu, Y. W. Search for Related Content PubMed PubMed Citation Articles by Goh, S. Articles by Wu, Y. W. Related Collections MRI All Pediatric All Spinal Cord Cerebrospinal Fluid

Presyrinx in children with Chiari malformations

From the School of Medicine (S.G., C.L.B., Y.W.W.) and Department of Radiology (A.H.A., W.P.D.), University of California San FranciscoB. is an MD candidate.

Address correspondence and reprint requests to Dr. Suzanne Goh, Resident in Child Neurology, University of California San Francisco, 350 Parnassus Ave., Suite 609, San Francisco, CA 94143 Suzanne.goh{at}ucsf.edu

Background: Presyrinx is a reversible state of spinal cord edema caused by alterations in CSF flow dynamics. Only three pediatric cases have been reported previously. We describe the clinical and radiologic features of presyrinx in six pediatric patients.

Methods: We electronically searched pediatric spine MRI reports generated at our institution from January 1995 to April 2007 for the keyword "presyrinx" and identified six patients with this radiologic diagnosis. We reviewed the neuroimaging studies and medical records for information regarding symptoms, treatment, and outcome.

Results: Of six patients identified with presyrinx, four had a Chiari I malformation and two had a Chiari II malformation. The MRI characteristics of the presyrinx included T2 prolongation, mild indistinct T1 prolongation, and cord enlargement without frank cavitation. Cine phase-contrast MRI studies were performed in three patients and showed severely diminished or absent CSF flow at the foramen magnum. Five patients underwent surgical decompression. All three patients with postoperative spine imaging showed restoration of CSF flow and resolution of the presyrinx. Symptoms of chronic or acute myelopathy attributable to the presyrinx were present in two patients. These symptoms resolved postoperatively.

Conclusions: Chiari I and II malformations obstructing CSF flow at the craniocervical junction may cause presyrinx in children. Presyrinx should be considered in the differential diagnosis of chronic or acute myelopathy in patients at risk for abnormal CSF flow dynamics.

e-Pub ahead of print on June 18, 2008, at www.neurology.org.

Disclosure: The authors report no disclosures.

Received August 30, 2007. Accepted in final form November 5, 2007.

This article has been cited by other articles:

				S. Ravaglia, A. Moglia, and E. I. Bogdanov Presyrinx in children with Chiari malformations Neurology, June 2, 2009; 72(22): 1966 - 1967. [Full Text] [PDF]

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