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Posttraumatic and Idiopathic Syringomyelia


Summary of Key Points

  • Posttraumatic and idiopathic syringomyelia represent a heterogenous group of neurological conditions characterized by fluid collections in the parenchyma of the spinal cord.

  • Obtaining a detailed history and examining the entire neuraxis is required for effective treatment to be directed at the underlying pathology when possible.

  • Magnetic resonance imaging evaluation with phase-contrast cine flow imaging is useful and may on occasion identify subarachnoid adhesion with obstruction of cerebrospinal fluid flow.

  • The natural history of the condition is unknown, but it often progresses through stages of edema, presyrinx, and finally fluid cavity formation.

  • Symptoms range from dysesthesia to progressive neurological decline and paralysis, and are not correlated with the size of the syringomyelia.

  • It is prudent to follow mildly symptomatic cases intermittently with expectant observation for delayed development or extension of the syrinx or associated worsening neurological function.

  • Surgical treatment with decompression of stenosis, correction of deformity, or lysis of subarachnoid adhesions can be considered when progression occurs.

  • Intradural procedures and shunting are reserved for severe debilitating cases, including idiopathic pathology.

  • A realistic goal of surgical treatment is mostly to stabilize clinical symptoms, although modest improvement may be expected.

Acknowledgment

I would like to thank my partner, Ajit Krishnaney, for his work on this chapter in the previous edition.

Syringomyelia denotes a collection of heterogeneous pathological conditions characterized by abnormal, longitudinally oriented, fluid-filled cavities within the spinal cord ( Fig. 95.1 ). Syringomyelia may be the result of congenital, traumatic, or neoplastic processes, and therapy is directed at correcting the underlying pathology. Therefore, obtaining an extensive history and performing imaging of the entire neural axis to elucidate the etiology are of paramount importance before developing a treatment plan. However, in many cases the etiology of the condition remains unknown, and treatment results may be unsatisfactory.

Fig. 95.1
Sagittal T2-weighted magnetic resonance imaging (MRI) ( A ) demonstrates an idiopathic syrinx. Axial T1-weighted MRI ( B ) of the idiopathic syrinx shows irregular cord T1-weighted hypointensity.

Although Charles Estienne first described the condition in 1546, the term syringomyelia, from the Greek syrinx , meaning “pipe,” “tube,” or “channel,” and myelos , meaning “marrow” was suggested by Olivier d’Angers in 1827. , The term is restricted to this condition and should not be used to describe similar entities such as proteinaceous cysts or a terminal ventricle. The term hydromyelia refers to cystic dilation of the ependyma-lined central canal by cerebrospinal fluid (CSF) ( Fig. 95.2 ). In some cases, this dilatation may dissect into the parenchyma of the spinal cord, and its original connection with the central canal may be lost. Therefore, the type of cellular lining is not a reliable criterion to distinguish between syringomyelia and hydromyelia. Because of these difficulties, the term syringohydromyelia has been used in the literature to refer to both entities. In this chapter, syringomyelia is used to denote all abnormal fluid-filled cavities in the spinal cord.

Fig. 95.2
Sagittal T2-weighted magnetic resonance imaging through the cervical spine shows an extensive epidural collection with resultant cord expansion, hyperintense cerebrospinal fluid signal within the cord, and central canal dilation (hydromyelia).

Epidemiology

Syringomyelia primarily affects children and young adults, usually presenting before the third decade of life. Before the widespread availability of magnetic resonance imaging (MRI), a prevalence of 9 per 100,000 and an incidence of approximately 0.44 cases per year were cited in the literature. Approximately 50% of patients presenting with syringomyelia have a Chiari malformation, whereas 25% present with a history of spinal cord trauma or arachnoiditis. The incidence of syringomyelia appears to be similar after quadriplegia or paraplegia. There is a statistically significant male preponderance in posttraumatic syringomyelia (80%), which follows the gender distribution of spinal cord injury. , , Of patients with a spinal cord injury investigated between 1 and 30 years after the initial insult, 21% to 28% have a syrinx, and 30% to 50% have some degree of spinal cystic change. However, symptomatic syringomyelia is reported in only 1% to 9% of the spinal cord injury population. ,

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