eMedicine Specialties > Physical Medicine and Rehabilitation > Spinal Cord Injury
Posttraumatic Syringomyelia
Updated: Oct 22, 2009
Introduction
Background
First described by Bastian in 1867, posttraumatic syringomyelia (PTS) refers to the development and progression of a cyst filled with cerebrospinal fluid (CSF) within the spinal cord. PTS is a relatively infrequent, but potentially devastating, complication following traumatic spinal cord injury (SCI). PTS is characterized clinically by the often insidious progression of pain and loss of sensorimotor function that may manifest many years after traumatic SCI. If left untreated, PTS can result in loss of function, chronic pain, respiratory failure, or death. (See images below and Images 1-2.)
This illustration shows a T1-weighted, cervical magnetic resonance imaging (MRI) scan of multiple syrinx cavities (arrows). Note the lowest thin cavity extending into the thoracic spinal cord.
This T2-weighted magnetic resonance imaging (MRI) scan (same patient as above) delineates the syrinx cavity. Note the spinal cord edema extending rostrally from the upper limit of the cavity.
Pathophysiology
The pathophysiology is not understood fully (see Causes). Formation of a cavity within the spinal cord is common after traumatic SCI. Factors related to initial cavity formation include liquefaction of intraparenchymal hematoma, ischemia due to tethering, arterial or venous obstruction, release of intracellular lysosomal enzymes and excitatory amino acids, and mechanical damage from cord compression. Cavity formation alone is not considered PTS.
In PTS, cavity formation is followed by enlargement and extension of the cystic cavity. Rostral or caudal cyst extension may occur due to turbulent CSF flow or a "one-way valve" phenomenon that allows CSF into, but not out of, the cyst cavity. Tethering of the spinal cord, which results in impaired CSF circulation around the traumatized segment of spinal cord, occurs as a sequela of bleeding-induced arachnoiditis, scarring, spinal canal stenosis, or kyphotic deformity.
The "slosh-and-suck" theory proposes that increased epidural venous flow occurring during activities (eg, coughing, sneezing) that produce effects like the Valsalva maneuver results in increased pressure around the spinal cord, which cannot be dissipated because of disruptions in CSF flow. This pressure may force CSF into the cyst, resulting in expansion and extension.
A model developed by Carpenter et al suggests that a cough or sneeze can produce a pressure wave that would, in turn, give rise to a shocklike elastic jump.1,2 According to the model, the elastic jump could create a transient high-pressure region in the spinal cord, resulting in fluid accumulation. However, in an analysis of the model, Elliott et al maintained that the effect of an elastic jump would probably be too weak for fluid accumulation to result and that "the polarity of the pressure differential set up by cough-type impulses opposes the tenets of the elastic-jump hypothesis."3 The authors conclude that, based on their analysis, cough-based pressure impulses cannot cause syringomyelia.
Frequency
United States
Approximately 3-4% of persons with traumatic SCI develop clinically symptomatic PTS. A larger percentage of persons have clinically silent syrinx cavities diagnosed by imaging techniques.
Mortality/Morbidity
Morbidity is associated with weakness, loss of function, and chronic pain. Mortality can occur from involvement of brainstem respiratory centers or surgical complications.
Race
No racial differences are known for development of PTS.
Sex
The incidence of PTS is higher in men due to the increased frequency of SCI in males; however, there is no association of manifestations of the condition with the patient's sex.
Age
Development of PTS can occur at any age, and may begin at any time after traumatic SCI. Cases are reported as early as 1 month or as late as 45 years following injury.
Clinical
History
- Pain is the most commonly reported symptom. Pain may be localized or diffuse and commonly is reported as a dull ache or a burning or stabbing sensation.
- Other symptoms include increased weakness, numbness, increased spasticity, and hyperhidrosis (increased sweating).
- Symptoms often are aggravated by postural change or the effects of the Valsalva maneuver.
- Decreased reflex micturition, progressive orthostasis, autonomic dysreflexia, and relatively painless joint deformity or swelling (Charcot joint) also may be reported.
Physical
- Spasticity often is increased compared to findings noted in prior examinations. Deep tendon reflex changes (either increased or decreased) may be noted compared with findings from prior examinations.
- Ascending sensory level and sensory dissociation (selective loss of pain and temperature sensation) are very sensitive indicators for detecting progressive PTS. Numbness may involve the face if the syrinx has ascended into the brainstem. (See image below and Image 4.)
T2-weighted sagittal image of large, multiloculated cervical syrinx extending into brainstem. Patient had preserved functional status.
- Progressive weakness and wasting can occur but may be a late finding.
- Other signs may include a complete or partial Horner syndrome or other evidence of dysautonomia (eg, labile blood pressure, hyperhidrosis).
- Signs may be unilateral because ascension of syrinxes often occurs unilaterally.
Causes
Traumatic SCI with tethering of the spinal cord to the dura results in impaired CSF circulation. Incomplete spinal canal decompression may predispose the person to tethering and CSF obstruction. These factors are thought to cause syrinx development.Research supports the concept that chronic mechanical stress to the spinal cord increases the risk for development of syringomyelia. Spinal instrumentation without decompression is also associated with earlier onset of syringomyelia.
More on Posttraumatic Syringomyelia |
 Overview: Posttraumatic Syringomyelia |
| Differential Diagnoses & Workup: Posttraumatic Syringomyelia |
| Treatment & Medication: Posttraumatic Syringomyelia |
| Follow-up: Posttraumatic Syringomyelia |
| Multimedia: Posttraumatic Syringomyelia |
| References |
| Further Reading |
| Next Page » |
References
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Further Reading
Clinical trials:
Establishing the Physiology of Syringomyelia
Study and Surgical Treatment of Syringomyelia
Keywords
posttraumatic syringomyelia, syringomyelia, SCI, spinal cord injury, syrinx, spinal cyst, spinal cysts, spinal cord cyst, syringomyelia symptoms, syringomyelia surgery, posttraumatic spinal cord injury
