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
See the list below:
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Increased weakness can result in functional loss, including transfers, wheelchair propulsion, gait, or self-care abilities.
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Functional losses, as well as impairments in sensation, predispose the patient to burns or skin breakdown.
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Progressive impairments in respiratory function place patients at risk for atelectasis, pneumonia, or respiratory failure.
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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 syringomyelia expansion and collapse syrinx cavities. Neurologic deterioration, pain, or autonomic dysreflexia may be indications for surgery. [1] 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, [2] and decompressive laminectomy with expansion duraplasty. [3, 4, 5, 6, 17] Cordectomy has also been performed. [7] 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. [18]
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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.
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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. A literature review by Ghobrial et al suggested that in adult patients with postinfectious or posttraumatic syringomyelia, arachnolysis, but not CSF diversion, extends the length of time to clinically symptomatic syringomyelia recurrence. [19]
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Percutaneous CT-guided drainage has been performed but rarely is used.
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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.
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Surgical approaches are evolving with the aim of improving long-term success.
A study by Bratelj et al of patients with posttraumatic spinal cord tethering and syringomyelia found that after untethering surgery, with expansion duraplasty and with or without syrinx shunting, the rate of neurologic improvement was 65.9%, while the rate of spasticity and/or neuropathic pain improvement was 50.0%. The investigators stated that “[i]n symptomatic spinal cord tethering and syringomyelia after trauma, surgical untethering with expansion duraplasty provides a promising treatment strategy to recover clinical deterioration in SCI patients.” [20]
See the images below.
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.
T2-weighted magnetic resonance imaging (MRI) scan (same patient as above) after patient underwent expansile duraplasty. Note dramatic reduction in size of the main syrinx cavity (white).
T1-weighted image demonstrating a large, multiloculated cervical syrinx cavity. This is a recurrent syrinx, having come back despite an attempt at drainage utilizing expansile duraplasty.
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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.
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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.
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T2-weighted magnetic resonance imaging (MRI) scan (same patient as above) after patient underwent expansile duraplasty. Note dramatic reduction in size of the main syrinx cavity (white).
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T2-weighted sagittal image of large, multiloculated cervical syrinx extending into brainstem. Patient had preserved functional status.
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T1-weighted magnetic resonance imaging (MRI) scan 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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Same patient as in image above, with the magnetic resonance imaging (MRI) scan slightly farther down the cervicothoracic region of the spine
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T2 proton density magnetic resonance imaging (MRI) scan 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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T1-weighted image demonstrating a large, multiloculated cervical syrinx cavity. This is a recurrent syrinx, having come back despite an attempt at drainage utilizing expansile duraplasty.
