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Physical Medicine and Rehabilitation > SPINAL CORD INJURY
Posttraumatic Syringomyelia
Article Last Updated: Jan 26, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11
Author: Lance Goetz, MD, Staff Physician, Assistant Professor of Physical Medicine and Rehabilitation, Spinal Cord Injury Center, Dallas VA Medical Center
Lance Goetz is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Medical Association, American Paraplegia Society, and American Spinal Injury Association
Coauthor(s):
Michael Priebe, MD, Associate Professor, Department of Physical Medicine and Rehabilitation, Mayo Clinic
Editors: Robert L Sheridan, MD, Assistant Chief of Staff, Chief of Burn Surgery, Shriners Burns Hospital; Associate Professor of Surgery, Department of Surgery, Division of Trauma and Burns, Massachusetts General Hospital and Harvard Medical School; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain (Tailbone Pain, Coccydynia) Service, UMDNJ-New Jersey Medical School; Kelly L Allen, MD, Consulting Staff, Department of Physical Medicine and Rehabilitation, Lourdes Regional Rehabilitation Center, Our Lady of Lourdes Medical Center; Denise I Campagnolo, MD, MS, Director of Multiple Sclerosis Clinical Research and Staff Physiatrist, Barrow Neurology Clinics, St. Joseph's Hospital and Medical Center; Investigator for Barrow Neurology Clinics; Director, NARCOMS Project for Consortium of MS Centers, Phoenix
Author and Editor Disclosure
Synonyms and related keywords:
syringomyelia, posttraumatic cystic myelopathy, progressive posttraumatic cystic myelopathy, progressive posttraumatic noncystic myelopathy, cystic myelopathy, syrinx, posttraumatic spinal cord injury, syringomyelia, late neurological deterioration following spinal cord injury
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.
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.
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.
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.
- 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.
Recent 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.
Autonomic Dysreflexia in Spinal Cord Injury
Carpal Tunnel Syndrome
Cervical Myofascial Pain
Cervical Spondylosis
Myofascial Pain
Rotator Cuff Disease
Spasticity
Spinal Cord Injury and Aging
Swallowing Disorders
Traumatic Brachial Plexopathy
Other Problems to be Considered
Cervical, thoracic, or lumbar radiculopathy
Spinal cord tumor
Spinal cord infarct
Epidural abscess or hematoma
Tethered cord syndrome
Progressive noncystic myelopathy
Spinal instability
Lab Studies
- Pulmonary function tests, especially vital capacity, should be ordered on any patient with symptoms or suggested respiratory impairment. Serial studies are useful to document and monitor for progression.
- No specific laboratory blood studies have proven useful in the diagnosis or monitoring of PTS.
Imaging Studies
- Magnetic resonance imaging (MRI), myelography-enhanced computed tomography (CT-myelogram), and plain radiographs of the spine are useful in the diagnosis and management of PTS.
- MRI is the preferred initial imaging study for the diagnosis of PTS. Most PTS develops around the site of the original spinal cord lesion. T1 and T2 sequences provide differentiation between CSF and normal spinal cord tissue and areas of spinal cord edema, myelomalacia, or gliosis. Serial examinations are necessary to evaluate for changes in cavity size over time. In addition, there is a marked lack of correlation between cavity size and severity of clinical symptoms.
- CT-myelography delineates the extent of the syrinx cavity, arachnoid scarring, and tethering of the spinal cord. This study demonstrates the extent of obstruction to CSF flow.
- Radiographs of the spine delineate spinal deformities such as fractures, dislocations, and abnormal spinal kyphotic or lordotic changes. Flexion/extension views assist in evaluation of spinal stability.
- Ultrasonography may be used intraoperatively after laminectomy to visualize syrinx cavities and septations.
Other Tests
- Serial quantitative strength measurements including pinch and grip tests or hand-held myometry are useful in confirming progression of weakness.
- Calculation of the central motor conduction time using motor evoked potentials is useful in monitoring PTS; however, this technique is not widely available.
- Standard electromyographic techniques, including nerve conduction studies, F-wave latencies, and needle electromyography (EMG), are less sensitive and specific in detecting PTS. Needle EMG may demonstrate a variety of abnormalities, including continuous motor unit activity, synchronous motor unit potentials, myokymic discharges, segmental and propriospinal myoclonus, and respiratory synkinesis. However, as these studies are best used to exclude other causes for the person's symptoms.
Histologic Findings
On pathologic section, cavitation of the gray matter is seen within the spinal cord. This phenomenon may involve the central canal or may be located eccentrically. An inner layer of gliotic tissue usually is present. The gray matter between the dorsal horns and posterior columns often is involved, possibly because of its relative avascularity and lack of connective tissue. Multiple cyst cavities, separated by complete or partial septae, are often present.
Rehabilitation Program
Physical Therapy
The focus of physical therapy in patients with syringomyelia should be preservation of range of motion and maintenance of function, including transfers, wheelchair mobility, and gait if applicable. Selection of appropriate assistive devices also is important. The physical therapist (PT) is helpful in monitoring manual muscle strength and joint function. Exercises and other mobilization activities that produce effects like the Valsalva maneuver should be avoided until normal CSF flow has been restored.
Occupational Therapy
The occupational therapist (OT) is helpful in assessing and treating the function of the person in performance of activities of daily living. The OT may perform splinting to maintain functional positions of the upper extremities and prevent contracture formation. Functional splints and other assistive devices can facilitate the performance of self-care tasks. The OT may perform detailed sensory testing of the upper extremities and quantitative pinch and grip strength testing. The PT and OT may work together in the selection of manual or power wheelchairs and seating systems.
Medical Issues/Complications
- Increased weakness can result in functional loss, including transfers, wheelchair propulsion, gait, or self-care abilities.
- Functional losses, as well as impairments in sensation, predispose the patient to burns or skin breakdown.
- Progressive impairments in respiratory function place patients at risk for atelectasis, pneumonia, or respiratory failure.
- Neuropathic arthropathy (Charcot joint) can occur as a result of lack of protective joint position sense.
Surgical Intervention
Surgery frequently is performed to prevent further expansion and collapse syrinx cavities. Neurologic deterioration, pain, or autonomic dysreflexia may be indications for surgery. No surgical procedure has been uniformly successful in relief of symptoms or resolution of radiographic abnormalities.
Surgical treatment has included simple drainage, a variety of shunting procedures, and decompressive laminectomy with expansion duraplasty. Cordectomy has also been performed. The question of which persons to treat surgically is controversial. Ideally, surgery should be performed on persons with syrinx cavities that are enlarging but are not yet symptomatic or that have become symptomatic only recently. All surgical procedures potentially can cause loss of motor, sensory, reflex, or autonomic function.
- Shunting of syrinx cavities, when performed alone, historically has been complicated by a high rate (up to 50%) of shunt failure or blockage and recurrent cyst expansion.
- Duraplasty/dural grafting and adhesiolysis may be performed with the goal of reestablishing unrestricted subarachnoid CSF flow. An expansile duraplasty is felt by some to be a more physiologic way of treating a tethered spinal cord with associated syringomyelia.
- Percutaneous CT-guided drainage has been performed but rarely is used.
- Recently, fetal neural tissue has been used to treat progressive PTS in cases where other treatments have failed. Successful obliteration of cyst cavities and survival of fetal tissue have been demonstrated in humans.
- Surgical approaches are evolving with the aim of improving long-term success.
Consultations
Consultation with a neurosurgeon generally is indicated.
Medical therapy is only for symptomatic control. Definitive treatment to date is surgical in nature.
Drug Category: Centrally acting antispasticity agents
Antispasticity agents are indicated when spasticity interferes with function, causes pain, or interferes with sleep.
| Drug Name | Baclofen (Lioresal) |
| Description | May induce the hyperpolarization of afferent terminals and inhibit both monosynaptic and polysynaptic reflexes at the spinal level. |
| Adult Dose | 5 mg PO tid; up to 30 mg 5 times/d |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Coadministration with antidepressants, specifically amitriptyline, imipramine, clomipramine, has induced short-term memory loss or additive muscle relaxant effect; opiate analgesics, benzodiazepines, alcohol, tricyclic antidepressants, guanabenz, MAO inhibitors, clindamycin, and hypertensive agents may increase effects |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Epilepsy or convulsive disorders, history of autonomic dysreflexia, children with insufficient body mass to accommodate the implantable pump, stroke patients, breastfeeding, impaired renal function, severe psychiatric disturbances or confusional states; caution in patients with history of autonomic dysreflexia and when spasticity is used to increase function; seizures or autonomic dysreflexia can result from abrupt withdrawal of this medication |
Drug Category: Tricyclic antidepressants
Although not an FDA-labeled use, TCAs are considered first-line treatment by specialists to treat many types of neuropathic pain. Doses used generally are less than those required to treat depression.
| Drug Name | Amitriptyline (Elavil) |
| Description | Analgesic for certain chronic and neuropathic pain. |
| Adult Dose | 30-100 mg/d PO hs |
| Pediatric Dose | Children: 0.1 mg/kg PO hs; increase, as tolerated, over 2-3 wk to 0.5-2 mg/d hs Adolescents: 25-50 mg/d PO initially; increase gradually to 100 mg/d in divided doses |
| Contraindications | Documented hypersensitivity; patient has taken MAO inhibitors in past 14 d; has history of seizures, cardiac arrhythmias, glaucoma, and urinary retention |
| Interactions | Phenobarbital may decrease effects; coadministration with CYP2D6 enzyme system inhibitors (eg, cimetidine, quinidine) may increase levels; inhibits hypotensive effects of guanethidine; may interact with thyroid medications, alcohol, CNS depressants, barbiturates, and disulfiram; additive effects with other anticholinergics increase risk of paralytic ileus; coadministration with anticholinergics or neuroleptics can cause hyperpyrexia |
| Pregnancy | D - Unsafe in pregnancy
|
| Precautions | Caution in cardiac conduction disturbances and history of hyperthyroidism, renal or hepatic impairment; avoid in elderly patients; patients with schizophrenia may develop increased symptoms of psychosis; patients with paranoid symptomatology may have an exaggeration of symptoms |
Drug Category: Anticonvulsants
Although not an FDA-labeled use, anticonvulsants are used commonly by specialists to treat neuropathic pain. Gabapentin has the advantage of reduced toxicity and side effects.
| Drug Name | Gabapentin (Neurontin) |
| Description | Has anticonvulsant properties and antineuralgic effects; however, the exact mechanism of action is unknown. Structurally related to GABA but does not interact with GABA receptors. Titration to effect can take place over several days (300 mg on day 1, 300 mg bid on day 2, and 300 mg tid on day 3). |
| Adult Dose | Day 1: 100 mg PO tid or 300 mg hs Day 2: 400 mg PO tid over 3 d and titrate prn; not to exceed 1200 mg PO qid |
| Pediatric Dose | <12 years: Not established >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | Antacids may reduce bioavailability of gabapentin significantly (administer at least 2 h following antacids); may increase norethindrone levels significantly; no appreciable interactions with other anticonvulsants |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Caution in severe renal disease |
Drug Category: Anticholinergics
Reduce gastrointestinal, salivary, and sudomotor (sweat gland) activity and can alleviate excess sweating in patients with PTS.
| Drug Name | Scopolamine (Transderm Scop) |
| Description | Blocks action of acetylcholine at parasympathetic sites in smooth muscle, secretory glands, and the CNS. Antagonizes histamine and serotonin action. Transdermal scopolamine may be the most effective agent for motion sickness. Its use in vestibular neuronitis is limited by its slow onset of action. Anticholinergic activity used in the treatment of nausea but also can inhibit the secretion of saliva and sweat. |
| Adult Dose | 1 mg (2.5 cm2) transdermal patch to hairless area behind ear q3d |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; primary glaucoma (including initial stages), pyloric obstruction, toxic megacolon, hepatic disease, paralytic ileus, severe ulcerative colitis, renal disease, obstructive uropathy, and myasthenia gravis |
| Interactions | Antipsychotic effectiveness of phenothiazines may be decreased by coadministration with scopolamine; anticholinergic side effects may be increased by concurrent therapy and phenothiazine dosages should be adjusted as necessary; coadministration with tricyclic antidepressants, may increase anticholinergic side effects (e.g., dry mouth, constipation, urinary retention) due to additive effect (tricyclic antidepressants with less anticholinergic activity may be beneficial) |
| Pregnancy | C - Safety for use during pregnancy has not been established.
|
| Precautions | Caution in elderly patients because of increased incidence of glaucoma; large doses may suppress intestinal motility and precipitate or aggravate toxic megacolon; anticholinergics may aggravate hiatal hernia associated with reflux esophagitis; patients with prostatism can have dysuria and may require catheterization; use cautiously in patients with asthma or allergies; a reduction in bronchial secretions can lead to inspissation and formation of bronchial plugs. |
Drug Category: Narcotic analgesics
May be necessary in patients whose pain is not controlled with other agents. Long-acting agents are preferred for chronic use.
| Drug Name | Oxycodone SA (OxyContin) |
| Description | Indicated for the relief of moderate to severe pain. |
| Adult Dose | 20 mg PO q12h; adjust dose upward to efficacy or downward for side effects; no theoretical maximal dose |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | Phenothiazines may antagonize analgesic effects; MAOIs, general anesthesia, CNS depressants, and tricyclic antidepressants may increase toxicity |
| Pregnancy | B - Usually safe but benefits must outweigh the risks.
|
| Precautions | Pregnancy category D if used for prolonged periods or in high doses; caution in COPD, emphysema, and renal insufficiency; phenothiazines may decrease analgesic effect of narcotic medications; toxicity increases with coadministration of either CNS depressants or tricyclic antidepressants |
Prognosis
- The natural history of untreated syringomyelia is variable, with stabilization of symptoms in some persons and slow but persistent progression in others. Some persons continue to demonstrate progression of the condition, despite surgical intervention. The exact percentage of persons with initially asymptomatic syrinx cavities who become symptomatic is not known.
Patient Education
- Medical professionals should educate the patient with PTS to avoid actions such as straining or Valsalva-type activities that may worsen symptoms. After successful treatment, this precaution may be lifted.
Medical/Legal Pitfalls
- Failure to recognize the symptoms of syringomyelia or attributing symptoms (pain, excess sweating, increased spasticity, numbness, or weakness) to other causes can result in morbidity, including neurologic deterioration. Clinicians should have a high index of suspicion for syringomyelia in any patient with SCI presenting with new onset or worsening of any of the aforementioned symptoms.
- The presence of a peripheral nerve disorder can alter the signs of myelopathy, masking both the sensory loss and distal hyperreflexia. Worsening syringomyelia could be missed in this setting.
| Media file 1:
This illustration shows a T1-weighted cervical MRI of multiple syrinx cavities (arrows). Note the lowest thin cavity extending into the thoracic spinal cord. |
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Media type: MRI
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| Media file 2:
This MRI shows the same patient as in Image 1 with a T2-weighted MRI image delineating syrinx cavity. Note the spinal cord edema extending rostrally from the upper limit of the cavity. |
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Media type: MRI
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| Media file 3:
T2 weighted MRI image of same patient as in Images 1 and 2, after patient underwent expansile duraplasty. Note dramatic reduction in size of the main syrinx cavity (white). |
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Media type: MRI
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| Media file 4:
This image demonstrates a T1-weighted MRI image of a slender syrinx (arrow) extending from the C5 vertebral level. This syrinx extends beyond the image to an area of spinal cord disruption at the T3 vertebral level. |
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Media type: MRI
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| Media file 5:
Same patient as in Image 4, with MRI image slightly farther down the cervicothoracic region of the spine |
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Media type: MRI
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| Media file 6:
T2 proton density MRI image demonstrating syrinx cavity (arrow) extending from approximately C6-C7 to T2. The syrinx cavity is 9 mm at its widest dimension. The spinal cord is reduced to a thin membrane at this level and is atrophic below. |
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Media type: MRI
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| Media file 7:
T1-weighted image demonstrating large, multiloculated cervical syrinx cavity. This image represents a recurrent syrinx despite prior attempt at drainage utilizing expansile duraplasty. |
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Media type: MRI
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Posttraumatic Syringomyelia excerpt Article Last Updated: Jan 26, 2007
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