Short case...Congenital syringomyelia

1. CONGENITAL SYRINGOMYELIA
Professor Yasser Metwally
www.yassermetwally.com
Short case
Edited by
Professor Yasser Metwally
Professor of neurology
Ain Shams university school of medicine
Cairo, Egypt
Visit my web site at:
http://yassermetwally.com
CONGENITAL SYRINGOMYELIA
Professor Yasser Metwally
www.yassermetwally.com

2. Figure 1. A case of congenital syringomyelia, MRI T1 images showing a longitudinal multisegmental
continuous syringomyelic cavity involving the whole of the cervical spinal cord and extending to the
upper dorsal segments with complete absence of transverse band and septations (Type I syringomyelic
cavity). Notice herniation of the cerebellar tonsils below the level of the foramen magnum. The
intracavitary MRI signal of the syringomyelic slit is identical with that of the CSF signal in the
subarachnoid spaces in MRI T1 and T2 images.
Figure 2. A case of congenital syringomyelia, MRI T1, T2 images showing a longitudinal multisegmental
continuous syringomyelic cavity involving the whole of the cervical spinal cord and extending to the upper
dorsal segments with complete absence of transverse band and septations.(Type I syringomyelic cavity).
Notice herniation of the cerebellar tonsils below the level of the foramen magnum. The intracavitary MRI
signal of the syringomyelic slit is identical with that of the CSF signal in the subarachnoid spaces in MRI
T1 and T2 images.

3. Figure 3. A case of congenital syringomyelia, MRI T1,T2 images showing a longitudinal multisegmental
continuous syringomyelic cavity involving the whole of the cervical spinal cord and extending to the upper
dorsal segments with complete absence of transverse band and septations (Type I syringomyelic cavity).
Notice herniation of the cerebellar tonsils below the level of the foramen magnum causing marked stenosis
at that level. The intracavitary MRI signal of the syringomyelic slit is identical with that of the CSF signal in
the subarachnoid spaces in MRI T1 and T2 images.
Figure 4. MRI T1,T2 images showing a central syringomyelic cavity representing dilatation of the central
canal of the spinal cord, notice the peripheral signal void area (A) that probably represent a CSF flow
void sign inside the syringomyelic cavity. The intracavitary MRI signal of the syringomyelic slit is
identical with that of the CSF signal in the subarachnoid spaces in MRI T1 and T2 images.

4. The case represents a congenital subtype syringomyelia because of the following
The presence of Arnold Chiari malformation which represents the aetiopathogenic factor of
congenital syringomyelia
The central location of the syringomyelic cavity which represents dilation of the central canal of the
spinal cord.
The syringomyelic cavity is a continuous slit without transverse bands or septations.
The involvement of the cervico-dorsal region of the spinal cord which is commonly the site of
involvement in congenital syringomyelia.
The intracavitary MRI signal of the syringomyelic slit is identical with that of the CSF signal in the
subarachnoid spaces in MRI T1 and T2 images.
Table 1. Differences between congenital and neoplastic syringomyelia
Congenital hydrosyringomyelia Neoplastic syringomyelia
The presence of Arnold Chiari malformation Absence of Arnold Chiari malformation
which represents the aetiopathogenic factor of
congenital syringomyelia
The syringomyelic cavity is centrally located Two types of cavities are noted.
and represents dilation of the central canal of
the spinal cord. (Hydromyelia) 1- A peripheral irregular cavities inside the
tumors which represents cystic breakdown of
tumor tissue (the cavitations are part of the
tumor).
2- Cavitations rostral and caudal to the spinal
tumors, these cavitations are not part of the
tumors and represents intramedullary
cavitations due to CSF flow obstruction.
It commonly involves the cervico-dorsal region Any part of the spinal cord can be involved.
The syringomyelic cavity is a continuous slit Transverse bands and septations are common.
without transverse bands or septations.
The intracavitary MRI signal of the The intracavitary MRI signal of the
syringomyelic slit is identical with that of the syringomyelic slit is different from that of the
CSF signal in the subarachnoid spaces in MRI CSF signal in the subarachnoid spaces in MRI
T1 and T2 images. T1 and T2 images.
References
Metwally, MYM: Textbook of neurimaging, A CD-ROM publication, (Metwally, MYM editor)
WEB-CD agency for electronic publishing, version 9.1a January 2008