Syringomyelia Workup

  • Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Apr 10, 2012
 

Laboratory Studies

  • Cerebrospinal fluid analysis (not performed because of the risk of herniation)
    • CSF pressure sometimes is elevated. A complete subarachnoid block may be noted.
    • Cell count is rarely more than 10/mm3.
    • Mild elevation of the CSF protein content occurs in half of these cases.
    • In cases of subarachnoid block, CSF protein may exceed 100 mg/dL.
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Imaging Studies

  • Plain radiograph
    • Plain films cannot detect the syrinx directly.
    • Cervical canal commonly is widened, and the pedicles may be eroded.
    • Flexion and extension films exclude bony instability.
    • Basilar impression or craniovertebral anomalies may be demonstrated.
  • Computerized tomography scan - Assists in detailed assessment and is especially useful in evaluation of bony spinal canal components
  • Myelography
    • Myelography is performed in special situations when MRI cannot be used.
    • Widening of the cord and complete subarachnoid block may be observed.
  • CT myelography
    • Myelogram combined with immediate and delayed high-resolution CT scan also can be performed.
    • Delayed CT scans are obtained 4-24 hours after the initial testing and can demonstrate cyst filling.
  • Magnetic resonance imaging
    • Imaging of the entire rostrocaudal extension of the cyst or cysts is important. Gadolinium-enhanced images are indicated if a tumor is suspected. Gadolinium-enhanced images are helpful in differentiating between scar or disk material associated with a syrinx, especially in postoperative or posttraumatic cases.
    • MRI examination should include sagittal and transverse views in T1 and T2 images (see image below). Proton density scans also can be helpful. Sagittal T1-weighted image showing a thoracic syriSagittal T1-weighted image showing a thoracic syrinx.
  • Magnetic resonance angiography - Can be especially helpful in cases of syringomyelia associated with vascular lesions
  • Cine phase-contrast MRI - Used to analyze CSF flow dynamics near the spinal cord cyst
  • Real-time ultrasonography - Rarely utilized for imaging syringomyelia since the development of MRI; ultrasonography for this purpose is technically more feasible in young children or in thin patients.
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Other Tests

  • In neurophysiological assessment by somatosensory evoked potentials (SSEPs), low-amplitude or delayed responses are present in myelopathy.
  • Neurophysiological assessment by motor evoked response may be more sensitive than SSEPs in the evaluation of spinal cord dysfunction.
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Procedures

  • The initial evaluation of patients suspected of having a spinal cord syrinx includes a comprehensive history and physical examination.
  • Information obtained from examinations guides the imaging studies. Essential tests include plain radiographic series with dynamic views and high-resolution CT scan to assess the bony spinal canal.
  • The most sensitive imaging test for soft tissue is an MRI scan. Gadolinium-enhanced images are also helpful in differentiating between tumor, scar, and disk material, especially in postoperative or posttraumatic cases.
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Histologic Findings

The syringomyelic cavity, or syrinx, forms most commonly in the lower cervical region, particularly at the base of the posterior horn and extending into the central gray matter and anterior commissure of the cord.

Histopathologic findings include (1) cavitation of spinal cord gray matter, (2) syrinx continuous with or adjacent to the central canal, and (3) an inner layer of gliotic tissue.

In association with the syrinx, other pathological conditions such as tumors, vascular anomalies, or infective processes also may be evident.

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Contributor Information and Disclosures
Author

Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE,  Associate Professor, Department of Neurosurgery, Suez Canal University; Co-Director, Center of Research and Development in Medical Education and Health Services Suez Canal University Hospital

Disclosure: Nothing to disclose.

Coauthor(s)

Ayman Ali Galhom, MD, PhD  Lecturer (Associated Professor), Department of Neurosurgery, Suez Canal University Faculty of Medicine, Egypt

Ayman Ali Galhom, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Franklin C Wagner, Jr, MD  Former Chief, Division of Spine and Spinal Cord Surgery, Former Professor, Department of Neurosurgery, University of Illinois at Chicago College of Medicine

Franklin C Wagner, Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Surgery of Trauma, American Association of Neurological Surgeons, American College of Surgeons, American Medical Association, Sigma Xi, Society for Neuroscience, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Christopher Luzzio, MD  Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

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: Medscape Salary Employment

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

References

  1. Nakanishi K, Uchiyama T, Nakano N, et al. Spinal syringomyelia following subarachnoid hemorrhage. J Clin Neurosci. Apr 2012;19(4):594-7. [Medline].

  2. Kim J, Kim CH, Jahng TA, Chung CK. Clinical course of incidental syringomyelia without predisposing pathologies. J Clin Neurosci. Feb 29 2012;[Medline].

  3. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. Jun 1965;28:247-59. [Medline].

  4. Williams B. Progress in syringomyelia. Neurol Res. Sep 1986;8(3):130-45. [Medline].

  5. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. Jan 1994;80(1):3-15. [Medline].

  6. Viswanatha B. Syringomyelia with syringobulbia presenting as vocal fold paralysis. Ear Nose Throat J. Jul 2009;88(7):E20. [Medline].

  7. Tubbs RS, Bailey M, Barrow WC, Loukas M, Shoja MM, Oakes WJ. Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia. Childs Nerv Syst. Jun 2009;25(6):689-92. [Medline].

  8. Nacir B, Arslan Cebeci S, Cetinkaya E, Karagoz A, Erdem HR. Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformation. Rheumatol Int. Jun 23 2009;[Medline].

  9. Ono A, Suetsuna F, Ueyama K, Yokoyama T, Aburakawa S, Numasawa T. Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation type I: the relationship between scoliosis and neurological findings. J Neurosurg Spine. Mar 2007;6(3):216-21. [Medline].

  10. Cardoso M, Keating RF. Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976). Aug 1 2009;34(17):1775-82. [Medline].

  11. Kiran NA, Kasliwal MK, Suri A, Mahapatra AK. Giant posterior fossa arachnoid cyst associated with syringomyelia. Clin Neurol Neurosurg. Feb 4 2010;[Medline].

  12. Lucchetta M, Cagnin A, Calderone M, Manara R, Rotilio A, Briani C. Syringomyelia associated with Chiari I malformation. Neurol Sci. Sep 19 2009;[Medline].

  13. Prat R, Galeano I. Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation. J Clin Neurosci. Apr 2009;16(4):531-4. [Medline].

  14. Falci SP, Indeck C, Lammertse DP. Posttraumatic spinal cord tethering and syringomyelia: surgical treatment and long-term outcome. J Neurosurg Spine. Oct 2009;11(4):445-60. [Medline].

  15. Sixt C, Riether F, Will BE, Tatagiba MS, Roser F. Evaluation of quality of life parameters in patients who have syringomyelia. J Clin Neurosci. Oct 7 2009;[Medline].

  16. Attal N, Parker F, Tadie M, et al. Effects of surgery on the sensory deficits of syringomyelia and predictors of outcome: a long term prospective study. J Neurol Neurosurg Psychiatry. Jul 2004;75(7):1025-30. [Medline].

  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. May 15 2005;30(10):1206-10. [Medline].

  18. Chang HS, Nakagawa H. Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. J Neurol Neurosurg Psychiatry. Mar 2003;74(3):344-7. [Medline].

  19. Colombo A, Cislaghi MG. Familial syringomyelia: case report and review of the literature. Ital J Neurol Sci. Dec 1993;14(9):637-9. [Medline].

  20. Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. Oct 2006;29(4):251-63; discussion 264. [Medline].

  21. Gruber DP, Crone KR. Neuroendoscopy. In: Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998:757-62.

  22. Huewel N, Perneczky A, Urban V, Fries G. Neuroendoscopic technique for the operative treatment of septated syringomyelia. Acta Neurochir Suppl (Wien). 1992;54:59-62. [Medline].

  23. Kaminsky SB, Clark CR, Traynelis VC. Operative treatment of cervical spondylotic myelopathy and radiculopathy. A comparison of laminectomy and laminoplasty at five year average follow-up. Iowa Orthop J. 2004;24:95-105. [Medline].

  24. Koyanagi I, Iwasaki Y, Hida K, Houkin K. Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. Surg Neurol. Apr 2005;63(4):350-5; discussion 355-6. [Medline].

  25. Lin JW, Lin MS, Lin CM, Tseng CH, Tsai SH, Kan IH. Idiopathic syringomyelia: case report and review of the literature. Acta Neurochir Suppl. 2006;99:117-20. [Medline].

  26. Madsen III PW, Green BA, Bowen BC. Syringomyelia. In: Herkowitz HN, Garfin SR, Balderston RA, et al, eds. The Spine. 2. 4th ed. Philadelphia: WB Saunders Company; 1999:1431-59.

  27. Mancall EL. Syringomyelia. In: Rowland LP, ed. Merritt's Textbook of Neurology. 1989. 8th ed. Philadelphia: Lea & Febiger; 687-91.

  28. Milhorat TH, Capocelli AL Jr, Kotzen RM. Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension. Neurosurgery. Nov 1997;41(5):1102-10. [Medline].

  29. Milhorat TH, Kotzen RM, Mu HT, et al. Dysesthetic pain in patients with syringomyelia. Neurosurgery. May 1996;38(5):940-6; discussion 946-7. [Medline].

  30. Mueller D, Oro' JJ. Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. Neurosurg Focus. Feb 15 2005;18(2):ECP2. [Medline].

  31. Oakes WJ. Chiari malformation and syringomyelia. In: Rengachary SS, Wilkins RA, eds. Principles of Neurosurgery. St. Louis, Mo: Wolfe; 1994:9.1-9.17.

  32. Penagaricano JA, Linskey ME, Ratanatharathorn V. Accelerated cerebral vasculopathy after radiation therapy to the brain. Neurol India. Dec 2004;52(4):482-6. [Medline].

  33. Rhoton AL, Hamilton AJ. Chiari malformation and syringomyelia. In: Benzel EC, ed. Spine Surgery: Techniques, Complication Avoidance, and Management. 2. Boston: Churchill-Livingstone; 1999:793-812.

  34. Rusbridge C, Greitz D, Iskandar BJ. Syringomyelia: current concepts in pathogenesis, diagnosis, and treatment. J Vet Intern Med. May-Jun 2006;20(3):469-79. [Medline].

  35. Simon RP, Aminoff MJ, Greenberg DA. Clinical Neurology. 4th ed. Norwalk, Conn: Appleton-Lange; 1999:220-1.

  36. Sudo K, Miyazaki Y, Tajima Y. Spontaneous resolution of idiopathic syringomyelia. Neurology. May 28 2002;58(10):1576-7; author reply 1577. [Medline].

  37. Wisoff JH. Chiari Malformations and Hydromyelia. In: Tindall GT, Cooper PR, Barrow D, eds. The Practice of Neurosurgery. 3. Baltimore: Williams & Wilkins; 1995:2743-53.

 
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Sagittal T1-weighted image showing a thoracic syrinx.
 
 
 
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