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Published online before print June 18, 2008, doi:10.1212/01.wnl.0000304087.91204.95)
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 Volume 71, Number 5, July 29, 2008
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NEUROLOGY 2008;71:351-356
© 2008 American Academy of Neurology

Presyrinx in children with Chiari malformations

S. Goh, MD, C. L. Bottrell, BA, A. H. Aiken, MD, W. P. Dillon, MD and Y. W. Wu, MD, MPH

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.




Correspondence:

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Presyrinx in children with Chiari malformations
Sabrina Ravaglia, et al.
Neurology Online, 29 Aug 2008 [Full text]

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