Syringomyelia Treatment & Management

  • Author: Hassan Ahmad Hassan Al-Shatoury, MD, PhD, MHPE; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Mar 17, 2010
 

Medical Care

  • No medical treatment is known for patients with syringomyelia. However, a chronic, stable clinical course is common. Identifying the underlying cause of syrinx formation is very important. Surgical treatment most likely will be necessary.
  • Neurorehabilitative care facilitates preservation of remaining neurological functions and prevents complications of quadriparesis such as infection and decubitus ulcers.
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Surgical Care

A variety of surgical treatments have been proposed for syringomyelia.

  • Suboccipital and cervical decompression
    • This operation includes suboccipital craniectomy; laminectomy of C1, C2, and sometimes C3; and duraplasty.[10, 11]
    • Some authors report microsurgical lysis of any adhesions, opening of the fourth ventricular outlet, and plugging of the obex (later steps are based on Gardner's hydrodynamic theory).
  • Laminectomy and syringotomy (dorsolateral myelotomy)
    • After decompression, the syrinx is drained into the subarachnoid space through a longitudinal incision in the dorsal root entry zone (between the lateral and posterior columns), usually at the level of C2-C3.
    • Incision in the dorsal root entry area has the minimum risk of increasing neurological deficit.
  • Shunts
    • Ventriculoperitoneal shunt - Indicated if ventriculomegaly and increased intracranial pressure are present
    • Lumboperitoneal shunt - Placed infrequently because of increased risk of herniation through the foramen magnum
    • Syringosubarachnoid dorsal root entry zone shunt
    • Syringoperitoneal shunt
  • Fourth ventriculostomy
  • Percutaneous needling: This technique is advocated as a possible mode of therapy; however, rapid refilling of the hydromyelic cavity from the ventricular system follows aspiration of fluid at the time of surgery. Moreover, a needle track seems unlikely to remain open.
  • Terminal ventriculostomy
    • The terminal ventricle is the dilated portion of the central canal that extends below the tip of the conus medullaris into the filum terminale. A laminectomy is performed over the caudal limit of the fluid sac, and the filum is opened.
    • This procedure is suitable only in patients with symptoms of syrinx without Chiari malformation. It is inappropriate in cases in which the hydromyelic cavity does not extend into the lumbar portion of the spinal cord or into the filum terminale.
  • Neuroendoscopic surgery
    • A fibroscope inserted through a small myelotomy allows inspection of the intramedullary cavity.
    • This technique is particularly useful in evaluating and treating multiple septate syrinxes.
    • Septa are fenestrated, either mechanically or by laser. Fluid from the cavity is then shunted into the subarachnoid space.
  • Surgical untethering in select cases with posttraumatic tethering associated with syringomyelia[12]
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Consultations

  • Neurosurgeon
  • Psychiatrist
  • Urologist
  • Physical therapist
  • Occupational therapist
  • Recreational therapist
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Diet

No specific diet is recommended for syringomyelia; however, normalizing weight is encouraged, especially in patients with neurological deficits.

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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

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

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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

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; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

Chief Editor

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

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; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

References

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

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

  3. 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].

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

  5. 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].

  6. 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].

  7. 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].

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

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

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

  11. 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].

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

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

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

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

  20. 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].

  21. 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].

  22. 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].

  23. 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].

  24. 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.

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

  26. 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].

  27. 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].

  28. 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].

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

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

  31. 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.

  32. 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].

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

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

  35. 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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