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Joseph Junewick, MD FACR
over 7 years ago
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Chiari I Malformation with Impending Syrinx

Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

4 year old aymptomatic patient; follow up Chiari I malformation.


Case Images


Diagnosis

Chiari I Malformation with Impending Syrinx

Findings

MR – Sagittal T1, T2, and IR and axial T2 images show downward displacement of the cerebellar tonsils which have a peg-like configuration. Also, note the subtle T2 and IR hyperintensity in the cord near the obex and the flagrant long segment central hyperintensity at the cervicothoracic junction.

Discussion

The pathogenesis of syringomyelia may be explained by 1) Communication of hydrocephalus betweeen the 4th ventricle and central canal as a result of outlet foramina obstruction, 2) Non-communicating related to reduced resorption of CSF at the level of the spinal subarachnoid space, or 3) Extracanalicular related to leukomalacia from ischemia, trauma or hemorrhage.

Noncommunicating syringomyelia is often seen with Chiari I malformation, cervical spinal stenosis, basilar invagination and arachnoiditis. Normal CSF flows from the spinal subarachnoid space into the perivascular spaces and interstitium of the spinal cord towards the central canal and is driven by pulsitile (arterial and/or transmitted CSF pulsations) and bulk flow. The location of the syrinx may be remote from the site of CSF obstruction secondary to normal variations in patency of the central canal.

The central cord edema seen in this case represents disturbed CSF flow related to obstruction at the foramen magnum, the so-call “pre-syrinx”. Relief of the CSF obstruction usually results in reversal of cord edema and prevention of progression to syrinx.

Reference

Fischbein NJ, Dillon WP, Cobbs C, Weinstein PR. The presyrinx state: A reversible myelopathic condition that my precede syringomyelia. AJNR (1999); 20:7-20.



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