Tags

Type your tag names separated by a space and hit enter

Presyrinx in children with Chiari malformations.
Neurology. 2008 Jul 29; 71(5):351-6.Neur

Abstract

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.

Authors+Show Affiliations

School of Medicine, University of California San Francisco, San Francisco, CA 94143, USA. Suzanne.goh@ucsf.eduNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18565831

Citation

Goh, S, et al. "Presyrinx in Children With Chiari Malformations." Neurology, vol. 71, no. 5, 2008, pp. 351-6.
Goh S, Bottrell CL, Aiken AH, et al. Presyrinx in children with Chiari malformations. Neurology. 2008;71(5):351-6.
Goh, S., Bottrell, C. L., Aiken, A. H., Dillon, W. P., & Wu, Y. W. (2008). Presyrinx in children with Chiari malformations. Neurology, 71(5), 351-6. https://doi.org/10.1212/01.wnl.0000304087.91204.95
Goh S, et al. Presyrinx in Children With Chiari Malformations. Neurology. 2008 Jul 29;71(5):351-6. PubMed PMID: 18565831.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Presyrinx in children with Chiari malformations. AU - Goh,S, AU - Bottrell,C L, AU - Aiken,A H, AU - Dillon,W P, AU - Wu,Y W, Y1 - 2008/06/18/ PY - 2008/6/21/pubmed PY - 2008/8/30/medline PY - 2008/6/21/entrez SP - 351 EP - 6 JF - Neurology JO - Neurology VL - 71 IS - 5 N2 - 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. SN - 1526-632X UR - https://www.unboundmedicine.com/medline/citation/18565831/Presyrinx_in_children_with_Chiari_malformations_ L2 - http://www.neurology.org/cgi/pmidlookup?view=long&pmid=18565831 DB - PRIME DP - Unbound Medicine ER -