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Posttraumatic Syringomyelia Clinical Presentation

  • Author: Lance L Goetz, MD; Chief Editor: Stephen Kishner, MD, MHA  more...
 
Updated: Dec 21, 2015
 

History

See the list below:

  • 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. Syringomyelia is the most common cause of Charcot joint in the upper extremity.[6]
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Physical

See the list below:

  • 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.)
    T2-weighted sagittal image of large, multiloculateT2-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.
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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.

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Contributor Information and Disclosures
Author

Lance L Goetz, MD Staff Physician, Spinal Cord Injury and Disorders, Hunter Holmes McGuire Veterans Affairs Medical Center; Associate Professor, Director, Spinal Cord Injury Medicine Fellowships, Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University School of Medicine

Lance L Goetz, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, Academy of Spinal Cord Injury Professionals, American Spinal Injury Association, Association of Academic Physiatrists, International Spinal Cord Society

Disclosure: Nothing to disclose.

Coauthor(s)

Revati Mummaneni, MD Chief Resident, Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

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 and Interim Chair 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, International Spine Intervention Society, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists

Disclosure: Nothing to disclose.

Chief Editor

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.

Additional Contributors

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<br/>Received income in an amount equal to or greater than $250 from: SimQuest Inc -- consultant on burn mapping softwear ($1,500).

Acknowledgements

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.

References
  1. Svircev JN, Little JW. Syringomyelia. Lin V, ed. Spinal Cord Medicine: Principles and Practice. 2nd ed. New York, NY: Demos; 2010. 569-575/42.

  2. 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].

  3. 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].

  4. 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].

  5. Krebs J, Koch HG, Hartmann K, Frotzler A. The characteristics of posttraumatic syringomyelia. Spinal Cord. 2015 Dec 1. [Medline].

  6. 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].

  7. 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].

  8. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. Bonsanto MM, Metzner R, Aschoff A. 3D ultrasound navigation in syrinx surgery - a feasibility study. Acta Neurochirurgica. 2005. 147(5):533-41.

  17. Carroll AM, Brackenridge P. Post-traumatic syringomyelia: a review of the cases presenting in a regional spinal injuries unit in the north east of England over a 5-year period. Spine. 2005. 30(10):1206-10. [Medline].

  18. Laxton AW, Perrin RG. Cordectomy for the treatment of posttraumatic syringomyelia. Report of four cases and review of the literature. Journal of Neurosurgery Spine. 2006. 4(2):174-8.

  19. Lee TT, Alameda GJ, Camilo E. Surgical treatment of post-traumatic myelopathy associated with syringomyelia. Spine. 2001. 26(24 Suppl):S119-27.

  20. Nogues MA. Spontaneous electromyographic activity in spinal cord lesions. [Review]. Muscle & Nerve. 2002. Suppl. 11:s77-82.

  21. Rittenberg JD, Burns SP, Little JW. Worsening myelopathy masked by peripheral nerve disorders. J Spinal Cord Med. 2004. 27(1):72-7.

  22. Silber JS, Vaccaro AR, Green B. Summary statement: chronic long-term sequelae after spinal cord injury: post-traumatic spinal deformity and post-traumatic myelopathy associated with syringomyelia.[comment]. Spine. 2001. 26(24 Suppl):S128.

  23. Vannemreddy SS, Rowed DW, Bharatwal N. Posttraumatic syringomyelia: predisposing factors. Br J Neurosurgery. 2002. 16(3):276-83.

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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.
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.
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).
T2-weighted sagittal image of large, multiloculated cervical syrinx extending into brainstem. Patient had preserved functional status.
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.
Same patient as in image above, with the magnetic resonance imaging (MRI) scan slightly farther down the cervicothoracic region of the spine
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.
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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