Posttraumatic Syringomyelia
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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.)
Pulmonary function tests, especially vital capacity, should be ordered on any patient with symptoms or suggested respiratory impairment.
Magnetic resonance imaging (MRI), myelography-enhanced computed tomography (CT-myelography), and plain radiography of the spine are useful in the diagnosis and management of PTS. MRI is the preferred initial imaging study for the diagnosis of the condition. 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; there is a marked lack of correlation between cavity size and severity of clinical symptoms.
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 occupational therapist (OT) is helpful in assessing and treating the function of the person in performance of activities of daily living.
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] Cordectomy has also been performed. [7]
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.
Syringomyelia can be categorized as “communicating” (dilation of the central canal) and “noncommunicating” (eccentrically located within the substance of the spinal cord). Congenital conditions, such as Chiari malformations, are associated with either communicating or noncommunicating syrinxes. However, PTS is generally considered noncommunicating. [8]
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. [9, 10] 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.” [11] The authors conclude that, based on their analysis, cough-based pressure impulses cannot cause syringomyelia.
A study by Krebs et al of 138 patients indicated that complete SCI and patient age over 30 years are risk factors for developing syrinx early (within 5 years of injury). The study also found that in almost 60% of patients with PTS, the cervical spine was involved. [12]
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.
Morbidity is associated with weakness, loss of function, and chronic pain. Mortality can occur from involvement of brainstem respiratory centers or surgical complications.
No racial differences are known for development of PTS.
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.
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. The risk of syrinx development within 5 years following traumatic SCI is greater in persons over age 30 years. [12]
Sixt C, Riether F, Will BE, et al. Evaluation of quality of life parameters in patients who have syringomyelia. J Clin Neurosci. 2009 Oct 7. [Medline].
Hayashi T, Ueta T, Kubo M, Maeda T, Shiba K. Subarachnoid-subarachnoid bypass: a new surgical technique for posttraumatic syringomyelia. J Neurosurg Spine. 2013 Apr. 18(4):382-7. [Medline].
Lam S, Batzdorf U, Bergsneider M. Thecal shunt placement for treatment of obstructive primary syringomyelia. J Neurosurg Spine. 2008 Dec. 9(6):581-8. [Medline].
Cacciola F, Capozza M, Perrini P, et al. Syringopleural shunt as a rescue procedure in patients with syringomyelia refractory to restoration of cerebrospinal fluid flow. Neurosurgery. 2009 Sep. 65(3):471-6; discussion 476. [Medline].
Kunert P, Janowski M, Zakrzewska A, et al. Syringoperitoneal shunt in the treatment of syringomyelia. Neurol Neurochir Pol. 2009 May-Jun. 43(3):258-62. [Medline].
Byun MS, Shin JJ, Hwang YS, Park SK. Decompressive surgery in a patient with posttraumatic syringomyelia. J Korean Neurosurg Soc. 2010 Mar. 47(3):228-31. [Medline]. [Full Text].
Ewelt C, Stalder S, Steiger HJ, Hildebrandt G, Heilbronner R. Impact of cordectomy as a treatment option for posttraumatic and non-posttraumatic syringomyelia with tethered cord syndrome and myelopathy. J Neurosurg Spine. 2010 Aug. 13(2):193-9. [Medline].
Svircev JN, Little JW. Syringomyelia. Lin V, ed. Spinal Cord Medicine: Principles and Practice. 2nd ed. New York, NY: Demos; 2010. 569-575/42.
Berkouk K, Carpenter PW, Lucey AD. Pressure wave propagation in fluid-filled co-axial elastic tubes. Part 1: Basic theory. J Biomech Eng. 2003 Dec. 125(6):852-6. [Medline].
Carpenter PW, Berkouk K, Lucey AD. Pressure wave propagation in fluid-filled co-axial elastic tubes. Part 2: Mechanisms for the pathogenesis of syringomyelia. J Biomech Eng. 2003 Dec. 125(6):857-63. [Medline].
Elliott NS, Lockerby DA, Brodbelt AR. The pathogenesis of syringomyelia: a re-evaluation of the elastic-jump hypothesis. J Biomech Eng. 2009 Apr. 131(4):044503. [Medline].
Krebs J, Koch HG, Hartmann K, Frotzler A. The characteristics of posttraumatic syringomyelia. Spinal Cord. 2016 Jun. 54 (6):463-6. [Medline].
Jackson K, Ramadorai U, Abell B, Devine J. Charcot arthropathy of the wrist associated with cervical spondylotic myelopathy. Global Spine J. 2012 Dec. 2(4):227-30. [Medline].
Yeo J, Cheng S, Hemley S, Lee BB, Stoodley M, Bilston L. Characteristics of CSF Velocity-Time Profile in Posttraumatic Syringomyelia. AJNR Am J Neuroradiol. 2017 Sep. 38 (9):1839-44. [Medline].
Aghakhani N, Baussart B, David P, Lacroix C, Benoudiba F, Tadie M, et al. Surgical treatment of posttraumatic syringomyelia. Neurosurgery. 2010 Jun. 66(6):1120-7; discussion 1127. [Medline].
Ghobrial GM, Dalyai RT, Maltenfort MG, Prasad SK, Harrop JS, Sharan AD. Arachnolysis or cerebrospinal fluid diversion for adult-onset syringomyelia? A Systematic review of the literature. World Neurosurg. 2015 May. 83 (5):829-35. [Medline].
Lance L Goetz, MD Associate Professor, Director of Spinal Cord Injury Medicine Fellowships, Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University School of Medicine; Staff Physician, Spinal Cord Injury and Disorders, Hunter Holmes McGuire Veterans Affairs Medical Center
Lance L Goetz, MD is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Physical Medicine and Rehabilitation, American Spinal Injury Association, Association of Academic Physiatrists, International Spinal Cord Society
Disclosure: Nothing to disclose.
Revati Mummaneni, MD Chief Resident, Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University School of Medicine
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Patrick M Foye, MD Director of Coccyx Pain Center, Professor of Physical Medicine and Rehabilitation, Rutgers New Jersey Medical School; Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, University Hospital
Patrick M Foye, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation
Disclosure: Nothing to disclose.
Stephen Kishner, MD, MHA Professor of Clinical Medicine, Physical Medicine and Rehabilitation Residency Program Director, Louisiana State University School of Medicine in New Orleans
Stephen Kishner, MD, MHA is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.
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
Robert L Sheridan, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American College of Surgeons
Disclosure: Received research grant from: Shriners Hospitals for Children; Physical Sciences Inc, Mediwound.
Michael Priebe, MD Associate Professor, Department of Physical Medicine and Rehabilitation, Mayo Clinic of Rochester, Minnesota
Michael Priebe, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Congress of Rehabilitation Medicine, American Paraplegia Society, American Spinal Injury Association, International Society of Physical and Rehabilitation Medicine, and International Spinal Cord Society
Disclosure: Nothing to disclose.
Posttraumatic Syringomyelia
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