eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases
Syringomyelia: Treatment & Medication
Updated: Dec 7, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
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.
Surgical Care
A variety of surgical treatments have been proposed for syringomyelia.
- Suboccipital and cervical decompression
- 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.
Consultations
- Neurosurgeon
- Psychiatrist
- Urologist
- Physical therapist
- Occupational therapist
- Recreational therapist
Diet
No specific diet is recommended for syringomyelia; however, normalizing weight is encouraged, especially in patients with neurological deficits.
Medication
No specific medication is indicated for treatment of syringomyelia. However, analgesics and muscle relaxants may be given for symptomatic treatment.
Nonsteroidal anti-inflammatory drugs (NSAIDs)
NSAIDs commonly are used as analgesics in patients with syringomyelia. If one class seems to be ineffective after a 2-week trial, a formulation from another class may be tried. The most commonly used drugs are ibuprofen, acetylsalicylic acid, naproxen, indomethacin, mefenamic acid, and piroxicam.
Ibuprofen (Ibuprin, Advil, Motrin)
One of propionic acid derivatives group. Effective inhibitor of cyclooxygenase, which is responsible for biosynthesis of prostaglandins; rapidly absorbed after PO administration; half-life in plasma is about 2 h; passes slowly into synovial spaces and may remain there in higher concentration as concentrations in plasma decline; excretion is rapid and complete, mainly in urine as metabolites or their conjugates.
Adult
Maintenance dose: 1200-1800 mg PO q4-6h; not to exceed 3200 mg in divided doses
Pediatric
Not established
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely in patients taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; avoid in peptic ulcer disease, recent GI bleeding or perforation, renal insufficiency, and high risk of bleeding
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studies in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in congestive heart failure, hypertension, and decreased renal or hepatic function; caution in coagulation abnormalities or during anticoagulant therapy
Aspirin (Anacin, Ascriptin, Bayer Aspirin)
Treats mild to moderately severe pain and headache. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2; acts on heat-regulating center of hypothalamus and vasodilates peripheral vessels to reduce fever.
Adult
325-650 mg PO q4-6h; not to exceed 4 g/d
Pediatric
10-15 mg/kg/dose PO q4-6h; not to exceed 60-80 mg/kg/d
Antacids and urinary alkalinizers may decrease effects; corticosteroids decrease serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur in patients taking anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma
Because of association with Reye syndrome, do not use in children ( <16 y) with flu
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studies in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid using in patients with severe anemia, history of blood coagulation defects, or taking anticoagulants
Naproxen (Naprelan, Naprosyn, Aleve, Anaprox)
For relief of mild to moderately severe pain; inhibits inflammatory reactions and pain by decreasing activity of cyclooxygenase, which is responsible for prostaglandin synthesis.
Adult
500 mg PO initial dose, followed by 250 mg q6-8h; not to exceed 1.25 g/d
Pediatric
<2 years: Not established
>2 years: 2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely in patients taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug
Indomethacin (Indocin, Indochron E-R)
Rapidly absorbed. Metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation. Inhibits prostaglandin synthesis.
Adult
25-50 mg IR PO bid/tid
75 mg PO SR PO bid; not to exceed 200 mg/d
Pediatric
1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely in patients taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; GI bleeding; renal insufficiency
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if persistent leukopenia, granulocytopenia, or thrombocytopenia)
Piroxicam (Feldene)
Decreases activity of cyclooxygenase, which in turn inhibits prostaglandin synthesis. These effects decrease formation of inflammatory mediators.
Adult
10-20 mg/d PO qd
Pediatric
0.2-0.3 mg/kg/d PO qd; not to exceed 15 mg/d
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely in patients taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; active GI bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue if persistent leukopenia, granulocytopenia, or thrombocytopenia)
Mefenamic acid (Ponstel)
Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
Adult
500 mg PO initially followed by 250 mg q4h prn
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely in patients taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studies in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May have adverse effects in fetus; caution in congestive heart failure, hypertension, and decreased renal or hepatic function; caution in coagulation abnormalities or during anticoagulant therapy
Muscle relaxants
These agents treat muscle spasms to decrease the patient's level of discomfort.
Methocarbamol (Robaxin)
Skeletal muscle relaxant used in conjunction with other therapeutic efforts to treat pain and discomfort associated with musculoskeletal conditions. Acts on CNS to relax certain reflexes.
Adult
<60 years: 1.5 g PO qid for first 48-72 h; usual maintenance dose is 750 mg to 1 g PO qid or 1.5 g tid, not to exceed 6 g/d for first 2-3 d or 8 g/d in severe conditions
>60 years: 6 g/d PO initially (8 g in severe cases); reduce dose prn
Pediatric
<12 years: Not established
>12 years: 800 mg (2 tab) PO qid
Increases toxicity of CNS depressants
Documented hypersensitivity; renal impairment
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Observe extreme caution in patients with impaired liver or kidney function; caution in patients with history of seizures; prolonged use requires regular monitoring
Because of risk of potential harm to newborn, avoid using while breastfeeding
Adverse effects include light-headedness, blurred vision, dizziness, drowsiness, itching, conjunctivitis, fever, headache, hives, nasal congestion, nausea and vomiting, rash, urticaria (itching attack, may be due to drug sensitivity), anaphylaxis (severe allergic reaction), extreme weakness, temporary vision loss, transient paralysis
Overdosage symptoms include convulsions, vomiting, diarrhea, headache, nausea, difficult breathing, sensation of paralysis, coma, severe weakness
Drug may cause color interference in certain screening tests for 5-hydroxyindoleacetic acid (5-HIAA) and vanillylmandelic acid (VMA)
To prevent additive CNS depression (eg, excessive sleepiness, slurred speech, decreased awareness), avoid drinking alcoholic beverages or taking other CNS depressants
Patients >60 years are more likely to experience adverse reactions
More on Syringomyelia |
| Overview: Syringomyelia |
| Differential Diagnoses & Workup: Syringomyelia |
 Treatment & Medication: Syringomyelia |
| Follow-up: Syringomyelia |
| Multimedia: Syringomyelia |
| References |
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References
Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. Jun 1965;28:247-59. [Medline].
Williams B. Progress in syringomyelia. Neurol Res. Sep 1986;8(3):130-45. [Medline].
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].
Viswanatha B. Syringomyelia with syringobulbia presenting as vocal fold paralysis. Ear Nose Throat J. Jul 2009;88(7):E20. [Medline].
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].
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].
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].
Cardoso M, Keating RF. Neurosurgical management of spinal dysraphism and neurogenic scoliosis. Spine (Phila Pa 1976). Aug 1 2009;34(17):1775-82. [Medline].
Lucchetta M, Cagnin A, Calderone M, Manara R, Rotilio A, Briani C. Syringomyelia associated with Chiari I malformation. Neurol Sci. Sep 19 2009;[Medline].
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].
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].
Boman K, Iivanainen M. Prognosis of syringomyelia. Acta Neurol Scand. 1967;43(1):61-8. [Medline].
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].
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].
Colombo A, Cislaghi MG. Familial syringomyelia: case report and review of the literature. Ital J Neurol Sci. Dec 1993;14(9):637-9. [Medline].
Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. Oct 2006;29(4):251-63; discussion 264. [Medline].
Gruber DP, Crone KR. Neuroendoscopy. In: Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998:757-62.
Hopkins A. Clinical Neurology: A Modern Approach. 1993. New York: Oxford University Press; 342-4.
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].
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].
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].
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].
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.
Mancall EL. Syringomyelia. In: Rowland LP, ed. Merritt's Textbook of Neurology. 1989. 8th ed. Philadelphia: Lea & Febiger; 687-91.
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].
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].
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].
Oakes WJ. Chiari malformation and syringomyelia. In: Rengachary SS, Wilkins RA, eds. Principles of Neurosurgery. St. Louis, Mo: Wolfe; 1994:9.1-9.17.
Penagaricano JA, Linskey ME, Ratanatharathorn V. Accelerated cerebral vasculopathy after radiation therapy to the brain. Neurol India. Dec 2004;52(4):482-6. [Medline].
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.
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].
Simon RP, Aminoff MJ, Greenberg DA. Clinical Neurology. 4th ed. Norwalk, Conn: Appleton-Lange; 1999:220-1.
Sudo K, Miyazaki Y, Tajima Y. Spontaneous resolution of idiopathic syringomyelia. Neurology. May 28 2002;58(10):1576-7; author reply 1577. [Medline].
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.
Wisoff JH, Epstein F. Management of hydromyelia. Neurosurgery. Oct 1989;25(4):562-71. [Medline].
Further Reading
Keywords
syringomyelia, hydromyelia, syrinx, syringohydromyelia, syringocephalus, syringobulbia, spinal cord cavity, Arnold-Chiari malformation, Chiari malformation, Chiari, spinal cord tethering, dysraphism, laminectomy, duraplasty, suboccipital craniectomy, syringotomy
