Syringomyelia 

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

Background

Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord. Hydromyelia is a dilatation of the central canal by cerebrospinal fluid (CSF) and may be included within the definition of syringomyelia. The following are types of syringomyelia.

Syringomyelia with fourth ventricle communication

About 10% of syringomyelia cases are of this type. This communication can be observed on MRI. In some cases, a blockage of CSF circulation occurs. A shunt operation may be the best therapeutic option for these patients.

Syringomyelia due to blockage of CSF circulation (without fourth ventricular communication)

Representing at least 50% of all cases, this is the most common type of syringomyelia. Obstruction of CSF circulation from the basal posterior fossa to the caudal space may cause syringomyelia of this type. The most common example is Arnold-Chiari malformation, which is also associated with communicating syringomyelia. Other causes include the following:

  • Basal arachnoiditis (postinfectious, inflammatory, postirradiation, blood in subarachnoid space)[1]
  • Basilar impression or invagination
  • Meningeal carcinomatosis
  • Pathological masses (arachnoid cysts, rheumatoid arthritis pannus, occipital encephalocele, tumors)

Syringomyelia due to spinal cord injury

Fewer than 10% of syringomyelia cases are of this type. Mechanisms of injury include (1) spinal trauma, (2) radiation necrosis, (3) hemorrhage from aneurysm rupture or arteriovenous malformation or in a tumor bed, (4) infection (spinal abscess, human immunodeficiency virus, transverse myelitis), and (5) cavitation following ischemic injury or degenerative disease.

Syringomyelia and spinal dysraphism

Spinal dysraphism may cause syringomyelia through a variety of mechanisms, including those mentioned under the previous 3 categories. Identification and treatment of associated dysraphism has the greatest impact on arresting progression of syringomyelia.

Syringomyelia due to intramedullary tumors

Fluid accumulation is usually caused by secretion from neoplastic cells or hemorrhage. The tumors most often associated with syringomyelia are ependymoma and hemangioblastoma. Extramedullary intradural and extradural tumors are considered separately under the second category because the mechanism of syrinx formation is blockage of the CSF pathway.

Idiopathic syringomyelia

Idiopathic syringomyelia has an unknown cause and cannot be classified under any of the previous categories.[2] Surgical decompression can help in some patients with remarkable neurologic deficit.

Next

Pathophysiology

Although many mechanisms for syrinx formation have been postulated, the exact pathogenesis is still unknown. Frequently cited theories are those of Gardner, William, and Oldfield.

Gardner's hydrodynamic theory[3]

This theory proposes that syringomyelia results from a "water hammer"-like transmission of pulsatile CSF pressure via a communication between the fourth ventricle and the central canal of the spinal cord through the obex. A blockage of the foramen of Magendie initiates this process.

William's theory[4]

This theory proposes that syrinx development, particularly in patients with Chiari malformation, follows a differential between intracranial pressure and spinal pressure caused by a valvelike action at the foramen magnum. The increase in subarachnoid fluid pressure from increased venous pressure during coughing or Valsalva maneuvers is localized to the intracranial compartment.

The hindbrain malformation prevents the increased CSF pressure from dissipating caudally. During Valsalva, a progressive increase in cisterna magna pressure occurs simultaneously with a decrease in spinal subarachnoid pressure. This craniospinal pressure gradient draws CSF caudally into the syrinx.

Oldfield's theory[5]

Downward movement of the cerebellar tonsils during systole can be visualized with dynamic MRI. This oscillation creates a piston effect in the spinal subarachnoid space that acts on the surface of the spinal cord and forces CSF through the perivascular and interstitial spaces into the syrinx raising intramedullary pressure. Signs and symptoms of neurological dysfunction that appear with distension of the syrinx are due to compression of long tracts, neurons, and microcirculation. Symptoms referable to raised intramedullary pressure are potentially reversible by syrinx decompression.

The intramedullary pulse pressure theory

The here-proposed intramedullary pulse pressure theory instead suggests that syringomyelia is caused by increased pulse pressure in the spinal cord and that the syrinx consists of extracellular fluid. A new principle is introduced implying that the distending force in the production of syringomyelia is a relative increase in pulse pressure in the spinal cord compared to that in the nearby subarachnoid space. The formation of a syrinx then occurs by the accumulation of extracellular fluid in the distended cord.

Previous
Next

Epidemiology

Frequency

United States

Estimated prevalence of the disease is about 8.4 cases per 100,000 people.

International

No geographic difference in the prevalence of syringomyelia is known.

Mortality/Morbidity

Assessing treatment results is difficult because of the rarity of syringomyelia, variability of presentation and natural history, and the relatively short follow-up in most studies.

  • In one study, half of all patients with syringomyelia were in clinically stable condition for several years.
  • Although an older study had suggested that 20% of patients died at an average of 47 years, mortality rates are likely lower in today's patients as a result of surgical interventions and better treatment of complications associated with significant paresis, such as pulmonary embolism.

Race

  • Occurrence of syringomyelia in different races is unknown.
  • Familial cases have been described.

Sex

  • Syringomyelia occurs more frequently in men than in women.

Age

  • The disease usually appears in the third or fourth decade of life, with a mean age of onset of 30 years.
  • Rarely, syringomyelia may develop in childhood or late adulthood.
Previous
Proceed to Clinical Presentation
 
 
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.

 
Previous
Next
 
Sagittal T1-weighted image showing a thoracic syrinx.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.