Syringomyelia
Syringomyelia is a chronic disorder of the spinal cord which describes a slowly expanding intramedullary cyst. The syrinx contains clear fluid which appears to be indistinguishable from cerebrospinal fluid (CSF) or extracellular fluid (ECF).
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4.7 Syringomyelia Jörg Klekamp
4.7.1 Definition Syringomyelia is a chronic disorder of the spinal cord which describes a slowly expanding intramedullary cyst. The syrinx contains clear fluid which appears to be indistinguishable from cerebrospinal fluid (CSF) or extracellular fluid (ECF).
4.7.2 Pathophysiology and Aetiology Syringomyelia is not a disease in its own right. Syringomyelia is a feature or a late complication of an underlying, chronic disease process, which has caused either a disturbance of CSF flow in the spinal canal (Fig. 4.7.1a, b) or impairs spinal cord mobility, or may be associated with
Fig. 4.7.1 a Sagittal T2-weighted MRI scan of a 27-year-old woman with a Chiari type I malformation and a huge septated syrinx throughout the entire spinal cord. She presented without any neurological deficits but a slowly progressing scoliosis. b Sagittal T2-weighted MRI scan of a 51-year-old man with a complete spinal cord lesion at T7 and a posttraumatic syrinx ex-
a spinal cord tumour (Fig. 4.7.1c). Up to the present, no commonly accepted pathophysiological concept exists as to how or why a syrinx may develop. Whereas most authors in the past century assumed that the fluid inside the syrinx was CSF and produced theories how CSF may be forced into the spinal cord, recent papers explained the development of a syrinx on the basis of altered ECF dynamics: due to alterations of CSF flow and/or spinal cord mobility or due to intramedullary tumours, ECF may accumulate in the spinal cord [3, 6]. On the basis of this concept, almost any disease of the spinal canal may cause a syrinx [7]: CSF flow obstructions and impaired spinal cord mobility may be associated with malformations of the spinal canal, degenerative disorders of the spine, spinal leptomeningeal diseases, neoplastic lesions or may be a late complication of spinal trauma or spinal surgery. In a series of 1,115 patients with syringomyelia, 414 were associated with craniocervical
tending up to C6. The patient developed a progressive sensory loss in his arms more than 30 years after the accident. c Sagittal T1-weighted MRI scan with contrast of a 35-year-old man with an intramedullary ependymoma at C3/C4 and a syrinx above and below the tumour
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4.7 Syringomyelia
malformations (Fig. 4.7.1a), 331 with spinal arachnoid scarring (Fig. 4.7.1b), 114 with intramedullary tumours (Fig. 4.7.1c), 57 with degenerative diseases of the spine, 21 with spinal dysraphism and 178 presented as dilatations of the central canal (Fig. 4.7.2a) (see below). In cases of posttraumatic syringomyelia, a lesion of the spinal cord is not required to produce a syrinx! The posttraumatic syrinx is the consequence of CSF flow obstruction at the level of the trauma and may be related to posttraumatic arachnoid scarring and/or posttraumatic spinal stenosis (Fig. 4.7.1b) [7]. As a general rule, a syrinx requires a long time before symptoms appear. For instance, most of the patients with a Chiari I malformation – one of the commonest diseases associated with syringomyelia (Fig.
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