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{{Infobox_Disease |
__NOTOC__
  Name          = Syringomyelia |
{{SI}}
  Image          = syringomyelia.jpg |
  Caption        = An idiopathic [[Syrinx (medicine)|syrinx]]. |
  DiseasesDB    = 12769 |
  ICD10          = {{ICD10|G|95|0|g|90}}, {{ICD10|Q|06|4|q|00}} |
  ICD9          = {{ICD9|336.0}} {{ICD9|742.53}} |
  ICDO          = |
  OMIM          = 272480 |
  OMIM_mult      = {{OMIM2|186700}} |
  MedlinePlus    = 001398 |
  eMedicineSubj  = |
  eMedicineTopic = |
  MeshID        = D013595 |
}}
{{Search infobox}}


'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


{{CMG}}
{{CMG}} {{AE}}{{TarekNafee}} {{AAA}} {{EAM}}


==Overview==
==Overview==
'''Syringomyelia''' '''is a [[spinal cord]] cavitation, which is a central dilation due to [[cystic]] degradation expands and destroyed the spinal cord. Caused by an injury, tumors or congenital malformation like [[hernia]]. The damage can Effect the [[brain]] and [[nerves]], that leade to Bilateral loss of [[pain]] and [[temperature]] sensation in upper [[extremities]]. [[weakness]], stiffness, hyperReflexives in lower extremities with [[hyposcoliosis]].''' Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, on the location of the [[Syrinx (medicine)|syrinx]] within the spinal cord.


'''Syringomyelia''' is a generic term referring to a disorder in which a [[cyst]] or tubular cavity forms within the [[spinal cord]]. This cyst, called a [[Syrinx (medicine)|syrinx]], can expand and elongate over time, destroying the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may result in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, to the location of the syrinx within the spinal cord.
==Historical Perspective==
*In the 16th century, Estienne, a French anatomist, observed [[spinal cord]] cavitation for the first time.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*In 1545, Stephanus reported a case of spinal cord dilatation in one of his patients.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*In 1688, Brunner reported a liquor filled cavity with hydrocephalus in a pediatric case.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*In 1824, Olivier d'Angers coined the term syringomyelia.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*Also in 1824, Schuppel defined the fluid in the [[Syrinx (medicine)|syrinx]] as a hydromyelia and proposed the [[pathogenesis]] as the persistence of the fetal central canal.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*In 1882, Otto von Kohler and Fredrich Schultz defined the clinical syndrome of syringomyelia.<ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*Between 1883 to 1891, Cleland and Hans Chiari associated syrinx formation with hyind brain herniation in what was subsequently named Chiari malformations types I to III. <ref name="pmid12181698">{{cite journal| author=Klekamp J| title=The pathophysiology of syringomyelia - historical overview and current concept. | journal=Acta Neurochir (Wien) | year= 2002 | volume= 144 | issue= 7 | pages= 649-64 | pmid=12181698 | doi=10.1007/s00701-002-0944-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12181698  }} </ref><ref name="pmid26591071">{{cite journal| author=Walusinski O| title=History of the Emergence and Recognition of Syringomyelia in the 19th Century. | journal=Vesalius | year= 2012 | volume= 18 | issue= 1 | pages= 18-29 | pmid=26591071 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26591071  }} </ref>
*In 1959, Gardner described the waterhammer theory for pathogenesis of syringomyelia.<ref name="pmid13500572">{{cite journal| author=GARDNER WJ, ANGEL J| title=The cause of syringomyelia and its surgical treatment. | journal=Cleve Clin Q | year= 1958 | volume= 25 | issue= 1 | pages= 4-8 | pmid=13500572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=13500572  }} </ref>
*In 1972, Ball and Dyan described the cavitation of the perivascular space as the source of syrinx formation.<ref name="pmid4116236">{{cite journal| author=Ball MJ, Dayan AD| title=Pathogenesis of syringomyelia. | journal=Lancet | year= 1972 | volume= 2 | issue= 7781 | pages= 799-801 | pmid=4116236 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4116236  }} </ref>
*In 1980, Williams described the "suck and slosh" theory of syrinx formation.<ref name="PMC1437943">{{cite journal | author=Williams B|title=On the pathogenesis of syringomyelia|journal=Journal of Royal Society of Medicine| volume=73 |issue=11 |pages=798-806 |year=1980 |pmcid="pmc1437943"}}</ref>
*In 1994, Oldfield describes the piston effect of Chiari I malformation resulting in syringomyelia.<ref name="pmid8271018">{{cite journal| author=Oldfield EH, Muraszko K, Shawker TH, Patronas NJ| title=Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. | journal=J Neurosurg | year= 1994 | volume= 80 | issue= 1 | pages= 3-15 | pmid=8271018 | doi=10.3171/jns.1994.80.1.0003 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8271018  }} </ref>
*In 2003, the Cisterna magna theory aimed to harmonize the Gardner, Williams, Oldfield and Ball&Dyan theories with neural imaging findings to propose an objective proposal of the pathogenesis of syringomyelia.<ref name="pmid12588922">{{cite journal| author=Chang HS, Nakagawa H| title=Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics. | journal=J Neurol Neurosurg Psychiatry | year= 2003 | volume= 74 | issue= 3 | pages= 344-7 | pmid=12588922 | doi= | pmc=1738338 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12588922  }} </ref>


Other, more common disorders share the early symptoms of syringomyelia. In the past, this has made diagnosis difficult. The advent of one diagnostic test, however, called magnetic resonance imaging or [[MRI]], has significantly increased the number of syringomyelia cases diagnosed in the beginning stages of the disorder.
==Classification==
Syringomyelia may be classified in various ways according to the anatomical features of the [[Syrinx (medicine)|syrinx]], or according to the underlying etiology of the disease.<ref name="pmid16676921">{{cite journal| author=Milhorat TH| title=Classification of syringomyelia. | journal=Neurosurg Focus | year= 2000 | volume= 8 | issue= 3 | pages= E1 | pmid=16676921 | doi=10.3171/foc.2000.8.3.1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16676921  }} </ref>


[[Syringomyelia]] has a prevalence estimated at 8.4 cases per 100,000 people,<ref>Brewis M, Poskanzer DC, Rolland C, et al, "Neurological disease in an English city". ''Acta Neurologica Scand Suppl'' 24:1--89, 1966.</ref> or about 21,000 Americans, with symptoms usually beginning in young adulthood. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing, straining, or [[myelopathy]]. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and [[chronic pain|chronic, severe pain]].
===By Anatomy===
Syringomyelia may be classified according to the anatomical description of the lesion ([[Syrinx (medicine)|syrinx]]) as follows:<ref name="pmid16676921">{{cite journal| author=Milhorat TH| title=Classification of syringomyelia. | journal=Neurosurg Focus | year= 2000 | volume= 8 | issue= 3 | pages= E1 | pmid=16676921 | doi=10.3171/foc.2000.8.3.1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16676921  }} </ref>
*'''Communicating''' vs. '''Non-communicating''':  
**A '''communicating syringomyelia''' is one where the [[Syrinx (medicine)|syrinx]] is continuous from the spinal cord, across the [[foramen magnum]] and the dilatation is continuous with the fourth [[ventricle]] 
**A '''non-communicating syringomyelia''' is characterized by absence of the continuity across the [[foramen magnum]]. It may present as isolated syringomyelia, or as isolated [[syringobulbia]] only affecting the [[brainstem]] with [[cranial nerve palsies]].
*'''Intracannalicular''' vs. '''Extracannalicular''':
**'''Intracannalicular synringomyelia''' originates within the [[central canal]] of the [[spinal cord]].
**'''Extracannalicular syringomyelia''' originates within the spinal cord parenchyma.


== The Cause ==
===By Etiology===
A watery, protective substance known as [[cerebrospinal fluid]] normally flows around the [[spinal cord]] and [[brain]], transporting nutrients and waste products. It also serves to cushion the brain.
Syringomyelia may be classified according to the underlying etiology or associated condition as follows:<ref name="pmid11807404">{{cite journal| author=Larner AJ, Muqit MM, Glickman S| title=Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging: an illustrative case and review of the literature. | journal=Medicine (Baltimore) | year= 2002 | volume= 81 | issue= 1 | pages= 41-50 | pmid=11807404 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11807404  }} </ref><ref name="pmid16506486">{{cite journal| author=Laxton AW, Perrin RG| title=Cordectomy for the treatment of posttraumatic syringomyelia. Report of four cases and review of the literature. | journal=J Neurosurg Spine | year= 2006 | volume= 4 | issue= 2 | pages= 174-8 | pmid=16506486 | doi=10.3171/spi.2006.4.2.174 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16506486  }} </ref><ref name="pmid16676930">{{cite journal| author=Batzdorf U| title=Primary spinal syringomyelia: a personal perspective. | journal=Neurosurg Focus | year= 2000 | volume= 8 | issue= 3 | pages= E7 | pmid=16676930 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16676930  }} </ref>
*Congenital Malformations
*Post-infectious
*Post-traumatic
*Post-inflammatory
*[[Neoplastic]]


A number of medical conditions can cause an obstruction in the normal flow of cerebrospinal fluid, redirecting it into the spinal cord itself. For reasons that are only now becoming clear, this results in syrinx formation. Cerebrospinal fluid fills the syrinx. Pressure differences along the spine cause the fluid to move within the cyst. Physicians believe that it is this continual movement of fluid that results in cyst growth and further damage to the spinal cord.
==Pathophysiology==
===Pathogenesis===
The exact pathogenesis of syringomyelia remains unknown. Several theories have been postulated about the development of a syrinx in the spinal cord. The underlying mechanisms of the disease involves disruption of CNS flow, subsequent formation of a syrinx, and enlargement of the lesion to impinge on the surrounding nerve fibers resulting in a symptomatic presentation.
:'''1. Disruption of CSF Flow'''
:Disruption of CSF flow may occur secondary to any of the following conditions:
:*Congenital craniocervical abnormalities obstructing [[CSF]] flow from the ventricles to the central canal (e.g., arnold chiari malformation, scoliosis)
:*Tumor or arachnoid cyst exhibiting a mass effect on the spinal cord
:*Sequelae of spinal cord [[trauma]] results in abnormal obstruction of the central canal
:*Sequelae of [[meningitis]] resulting in inflammation of the spinal cord meninges
:*Sequelae of inflammatory conditions such as [[sarcoidosis]], [[multiple sclerosis]], [[seronegative spondyloarthropathies]]
:'''2. Syrinx formation'''
:The pathogenesis of a syrinx is largely unknown. There are five primary theories of intra and extra cannilicular
:*'''Gardner's hydrodynamic theory:''' Blockage of the foramen of Magendie at the [[obex]] results in a pulsatile "water-hammer" effect of the mass lesion on the spinal cord CSF. This pressure differential results in extravasation of fluid into the perivascular and extracellular spaces.<ref name="pmid12588922">{{cite journal |vauthors=Chang HS, Nakagawa H |title=Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics |journal=J. Neurol. Neurosurg. Psychiatr. |volume=74 |issue=3 |pages=344–7 |year=2003 |pmid=12588922 |pmc=1738338 |doi= |url=}}</ref><ref name="pmid1525970">{{cite journal |vauthors=Pillay PK, Awad IA, Hahn JF |title=Gardner's hydrodynamic theory of syringomyelia revisited |journal=Cleve Clin J Med |volume=59 |issue=4 |pages=373–80 |year=1992 |pmid=1525970 |doi= |url=}}</ref><ref name="pmid13500572">{{cite journal |vauthors=GARDNER WJ, ANGEL J |title=The cause of syringomyelia and its surgical treatment |journal=Cleve Clin Q |volume=25 |issue=1 |pages=4–8 |year=1958 |pmid=13500572 |doi= |url=}}</ref>
:* '''William's theory:''' Increased intracranial pressure combined with decreased subarachnoid spinal pressure results in a "vaccuum-like" effect which further herniates the cerebellar tonsils through the foramen magnum. This results in sloshing of the subarachnoid CSF and causes extravasation of the CSF into the spinal cord parenchyma, thus creating a syrinx.<ref name="pmid12588922">{{cite journal |vauthors=Chang HS, Nakagawa H |title=Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics |journal=J. Neurol. Neurosurg. Psychiatr. |volume=74 |issue=3 |pages=344–7 |year=2003 |pmid=12588922 |pmc=1738338 |doi= |url=}}</ref><ref name="PMC1437943">{{cite journal | author=Williams B|title=On the pathogenesis of syringomyelia|journal=Journal of Royal Society of Medicine| volume=73 |issue=11 |pages=798-806 |year=1980 |pmcid="pmc1437943"}}</ref>
:* '''Ball & Dyan theory:''' With the CSF pressure difference created by the lesion, fluid moves, collects, and expands within [[Perivascular cell|Robin Virchow's perivascular space]].<ref name="pmid4116236">{{cite journal |vauthors=Ball MJ, Dayan AD |title=Pathogenesis of syringomyelia |journal=Lancet |volume=2 |issue=7781 |pages=799–801 |year=1972 |pmid=4116236 |doi= |url=}}</ref>
:* '''Oldfield's theory:''' The pulsatile waves of the CSF which occur during systole result in increased ICP. This increased pressure herniates the cerebellum to obstruct the subarachnoid space at the level of the foramen magnum. This pulsatile fluid wave against the surface of the spinal cord results in extravasation of fluid into the parenchyma and creates a syrinx.<ref name="pmid8271018">{{cite journal |vauthors=Oldfield EH, Muraszko K, Shawker TH, Patronas NJ |title=Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment |journal=J. Neurosurg. |volume=80 |issue=1 |pages=3–15 |year=1994 |pmid=8271018 |doi=10.3171/jns.1994.80.1.0003 |url=}}</ref>
:* '''Cisterna Magna theory:''' This theory claims that the fluid storage capacity of the cisterna magna is the primary shock absorber of the CNS that allows the spinal cord parenchyma to remain protected from increases in intracranial pressure. Mass effect of any lesion "stiffens" the cisterna magna and results in decreased compliance of the space and increased transferrance of pressure to the spinal cord. This results in extravasation of fluid to the spinal cord parenchyma.<ref name="pmid12588922">{{cite journal |vauthors=Chang HS, Nakagawa H |title=Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics |journal=J. Neurol. Neurosurg. Psychiatr. |volume=74 |issue=3 |pages=344–7 |year=2003 |pmid=12588922 |pmc=1738338 |doi= |url=}}</ref>
:'''3. Development of symptoms'''
:Once the syrinx enlarges enough to impinge on the surrounding spinal cord segments, symptoms related to the affected nerve fibers will develop due to the mass effect of the syrinx itself.


== Different Origins ==
===Genetics===
Generally, there are two forms of [[syringomyelia]].
There is no genetic predilection for the development of syringomyelia. However, Chiari malformations which are the primary cause of syringomyelia have demonstrated a strong familial and genomic association.<ref name="pmid17103432">{{cite journal| author=Boyles AL, Enterline DS, Hammock PH, Siegel DG, Slifer SH, Mehltretter L et al.| title=Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15. | journal=Am J Med Genet A | year= 2006 | volume= 140 | issue= 24 | pages= 2776-85 | pmid=17103432 | doi=10.1002/ajmg.a.31546 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17103432  }} </ref><ref name="pmid10232534">{{cite journal| author=Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C et al.| title=Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. | journal=Neurosurgery | year= 1999 | volume= 44 | issue= 5 | pages= 1005-17 | pmid=10232534 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10232534  }} </ref><ref name="pmid26141174">{{cite journal| author=Speer MC, Enterline DS, Mehltretter L, Hammock P, Joseph J, Dickerson M et al.| title=Review Article: Chiari Type I Malformation with or Without Syringomyelia: Prevalence and Genetics. | journal=J Genet Couns | year= 2003 | volume= 12 | issue= 4 | pages= 297-311 | pmid=26141174 | doi=10.1023/A:1023948921381 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26141174  }} </ref><ref name="pmid22462700">{{cite journal| author=Markunas CA, Tubbs RS, Moftakhar R, Ashley-Koch AE, Gregory SG, Oakes WJ et al.| title=Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations. | journal=J Neurosurg Pediatr | year= 2012 | volume= 9 | issue= 4 | pages= 372-8 | pmid=22462700 | doi=10.3171/2011.12.PEDS11113 | pmc=3678957 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22462700  }} </ref><ref name="pmid8433785">{{cite journal| author=Stovner LJ, Bergan U, Nilsen G, Sjaastad O| title=Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation. | journal=Neuroradiology | year= 1993 | volume= 35 | issue= 2 | pages= 113-8 | pmid=8433785 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8433785  }} </ref><ref name="pmid7861232">{{cite journal| author=Cavender RK, Schmidt JH| title=Tonsillar ectopia and Chiari malformations: monozygotic triplets. Case report. | journal=J Neurosurg | year= 1995 | volume= 82 | issue= 3 | pages= 497-500 | pmid=7861232 | doi=10.3171/jns.1995.82.3.0497 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7861232  }} </ref>


'''The first major form''' relates to an abnormality of the brain called an [[Arnold-Chiari malformation]], named after the physicians who first characterized it. This is the most common cause of syringomyelia, where the anatomic abnormality causes the lower part of the [[cerebellum]] to protrude from its normal location in the back of the head into the cervical or neck portion of the spinal canal. A syrinx may then develop in the cervical region of the spinal cord. Because of the relationship that was once thought to exist between the brain and spinal cord in this type of syringomyelia, physicians sometimes refer to it as communicating syringomyelia. Here, symptoms usually begin between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate suddenly. Some patients, however, may have long periods of stability. Some patients with this form of the disorder also have [[hydrocephalus]], in which cerebrospinal fluid accumulates in the skull, or a condition called [[arachnoiditis]], in which a covering of the spinal cord--the arachnoid membrane--is inflamed.
==Causes==
The most common cause of syringomyelia is [[Arnold chiari malformation|Chiari I malformation]]<ref name="pmid14580664">{{cite journal| author=Carlson MD, Muraszko KM| title=Chiari I malformation with syrinx. | journal=Pediatr Neurol | year= 2003 | volume= 29 | issue= 2 | pages= 167-9 | pmid=14580664 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14580664  }} </ref>. Other causes of syringomyelia may include:
===Common Causes===
*Other congenital abnormalities (e.g., scoliosis, [[Chiari malformation|Chiari I and II malformations]])<ref name="pmid2136163">{{cite journal| author=Williams B| title=Syringomyelia. | journal=Neurosurg Clin N Am | year= 1990 | volume= 1 | issue= 3 | pages= 653-85 | pmid=2136163 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2136163  }} </ref><ref name="pmid14580664">{{cite journal| author=Carlson MD, Muraszko KM| title=Chiari I malformation with syrinx. | journal=Pediatr Neurol | year= 2003 | volume= 29 | issue= 2 | pages= 167-9 | pmid=14580664 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14580664  }} </ref><ref name="pmid12838099">{{cite journal| author=Ozerdemoglu RA, Denis F, Transfeldt EE| title=Scoliosis associated with syringomyelia: clinical and radiologic correlation. | journal=Spine (Phila Pa 1976) | year= 2003 | volume= 28 | issue= 13 | pages= 1410-7 | pmid=12838099 | doi=10.1097/01.BRS.0000067117.07325.86 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12838099  }} </ref><ref name="pmid27430461">{{cite journal| author=Shankar P, Zamora C, Castillo M| title=Congenital malformations of the brain and spine. | journal=Handb Clin Neurol | year= 2016 | volume= 136 | issue=  | pages= 1121-37 | pmid=27430461 | doi=10.1016/B978-0-444-53486-6.00058-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27430461  }} </ref>
*Traumatic spinal cord injury<ref name="pmid21139799">{{cite journal| author=Agrawal A, Shetty MS, Pandit L, Shetty L, Srikrishna U| title=Post-traumatic syringomyelia. | journal=Indian J Orthop | year= 2007 | volume= 41 | issue= 4 | pages= 398-400 | pmid=21139799 | doi=10.4103/0019-5413.37006 | pmc=2989527 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21139799  }} </ref>


'''The second major form''' of syringomyelia occurs as a complication of [[Physical trauma|trauma]], [[meningitis]], [[hemorrhage]], a [[tumor]], or arachnoiditis. Here, the syrinx or cyst develops in a segment of the spinal cord damaged by one of these conditions. The syrinx then starts to expand. This is sometimes referred to as noncommunicating syringomyelia. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma.
===Less Common Causes===
*Infections (e.g., [[meningitis]]/[[Arachnoiditis|arachnoiditi]]<nowiki/>s)<ref name="pmid12754657">{{cite journal| author=Nardone R, Alessandrini F, Tezzon F| title=Syringomyelia following Listeria meningoencephalitis: report of a case. | journal=Neurol Sci | year= 2003 | volume= 24 | issue= 1 | pages= 40-3 | pmid=12754657 | doi=10.1007/s100720300021 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12754657  }} </ref><ref name="pmid2313296">{{cite journal| author=Caplan LR, Norohna AB, Amico LL| title=Syringomyelia and arachnoiditis. | journal=J Neurol Neurosurg Psychiatry | year= 1990 | volume= 53 | issue= 2 | pages= 106-13 | pmid=2313296 | doi= | pmc=487945 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2313296  }} </ref>
*Mass lesions or [[neoplasia]] (e.g., [[Ependymoma]], [[hemangioblastoma]], [[meningioma]], or [[arachnoid cyst]])<ref name="pmid24381458">{{cite journal| author=Joshi VP, Valsangkar A, Nivargi S, Vora N, Dekhne A, Agrawal A| title=Giant posterior fossa arachnoid cyst causing tonsillar herniation and cervical syringomyelia. | journal=J Craniovertebr Junction Spine | year= 2013 | volume= 4 | issue= 1 | pages= 43-5 | pmid=24381458 | doi=10.4103/0974-8237.121627 | pmc=3872663 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24381458  }} </ref><ref name="pmid19096585">{{cite journal| author=Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES| title=Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment. | journal=J Korean Neurosurg Soc | year= 2007 | volume= 42 | issue= 6 | pages= 436-40 | pmid=19096585 | doi=10.3340/jkns.2007.42.6.436 | pmc=2588179 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19096585  }} </ref><ref name="pmid25029412">{{cite journal| author=Gluf WM, Dailey AT| title=Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: case report and review of the literature. | journal=J Spinal Cord Med | year= 2014 | volume= 37 | issue= 6 | pages= 791-4 | pmid=25029412 | doi=10.1179/2045772314Y.0000000210 | pmc=4231969 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25029412  }} </ref><ref name="pmid2259411">{{cite journal| author=Mock A, Levi AD, Levi A, Drake JM| title=Spinal hemangioblastoma, syrinx, and hydrocephalus in a two-year-old child. | journal=Neurosurgery | year= 1990 | volume= 27 | issue= 5 | pages= 799-802 | pmid=2259411 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2259411  }} </ref>
*Inflammatory conditions (e.g. [[multiple sclerosis]], t[[Myelitis|ransverse myelitis]], [[amyotrophic lateral sclerosis]], [[sarcoidosis]], other myelitis)<ref name="pmid17467892">{{cite journal| author=Ravaglia S, Bogdanov EI, Pichiecchio A, Bergamaschi R, Moglia A, Mikhaylov IM| title=Pathogenetic role of myelitis for syringomyelia. | journal=Clin Neurol Neurosurg | year= 2007 | volume= 109 | issue= 6 | pages= 541-6 | pmid=17467892 | doi=10.1016/j.clineuro.2007.03.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17467892  }} </ref><ref name="pmid26495810">{{cite journal| author=Markh A, Wainapel S| title=Transverse Myelitis and Syrinx Formation Caused by Varicella-Zoster Infection. | journal=Am J Phys Med Rehabil | year= 2016 | volume= 95 | issue= 1 | pages= e12-3 | pmid=26495810 | doi=10.1097/PHM.0000000000000391 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26495810  }} </ref><ref name="pmid11603356">{{cite journal| author=Sotgiu S, Pugliatti M, Rosati G, Sechi GP| title=Which syringomyelia is truly associated with multiple sclerosis? | journal=J Neurol Sci | year= 2001 | volume= 190 | issue= 1-2 | pages= 99-100 | pmid=11603356 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11603356  }} </ref><ref name="pmid15249639">{{cite journal| author=Charles JA, Berger M, Cook SD| title=Thoracic syringomyelia and suspected multiple sclerosis: cause and effect or coincidence? | journal=Neurology | year= 2004 | volume= 63 | issue= 1 | pages= 185-6 | pmid=15249639 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15249639  }} </ref><ref name="pmid2206638">{{cite journal| author=Hamada K, Sudoh K, Fukaura H, Yanagihara T, Hamada T, Tashiro K et al.| title=[An autopsy case of amyotrophic lateral sclerosis associated with cervical syringomyelia]. | journal=No To Shinkei | year= 1990 | volume= 42 | issue= 6 | pages= 527-31 | pmid=2206638 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2206638  }} </ref>
*[[Idiopathic]] <ref name="pmid19525795">{{cite journal| author=Nakamura M, Ishii K, Watanabe K, Tsuji T, Matsumoto M, Toyama Y et al.| title=Clinical significance and prognosis of idiopathic syringomyelia. | journal=J Spinal Disord Tech | year= 2009 | volume= 22 | issue= 5 | pages= 372-5 | pmid=19525795 | doi=10.1097/BSD.0b013e3181761543 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19525795  }} </ref><ref name="pmid17684895">{{cite journal| author=Porensky P, Muro K, Ganju A| title=Nontraumatic cervicothoracic syrinx as a cause of progressive neurologic dysfunction. | journal=J Spinal Cord Med | year= 2007 | volume= 30 | issue= 3 | pages= 276-81 | pmid=17684895 | doi= | pmc=2031960 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17684895  }} </ref>


The primary symptom of post-traumatic [[syringomyelia]] (often referred to using the abbreviation of PTS) is pain, which may spread upward from the site of injury. Symptoms, such as pain, numbness, weakness, and disruption in temperature sensation, may be limited to one side of the body. Syringomyelia can also adversely affect sweating, sexual function, and, later, bladder and bowel control. A typical cause of PTS would be a car accident or similar trauma involving a whip-lash injury.
==Differentiating Syringomyelia from other diseases==
Syringomyelia must be differentiated from other diseases which cause gait abnormalities, sensory and motor deficit, chronic pain syndromes, urinary and fecal incontinence, and cranial nerve palsies such as:<ref name="pmid18848313">{{cite journal| author=van Doorn PA, Ruts L, Jacobs BC| title=Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. | journal=Lancet Neurol | year= 2008 | volume= 7 | issue= 10 | pages= 939-50 | pmid=18848313 | doi=10.1016/S1474-4422(08)70215-1 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18848313  }} </ref><ref name="pmid23564314">{{cite journal| author=Dimachkie MM, Barohn RJ| title=Chronic inflammatory demyelinating polyneuropathy. | journal=Curr Treat Options Neurol | year= 2013 | volume= 15 | issue= 3 | pages= 350-66 | pmid=23564314 | doi=10.1007/s11940-013-0229-6 | pmc=3987657 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23564314  }} </ref><ref name="pmid14977560">{{cite journal| author=Krishnan C, Kaplin AI, Deshpande DM, Pardo CA, Kerr DA| title=Transverse Myelitis: pathogenesis, diagnosis and treatment. | journal=Front Biosci | year= 2004 | volume= 9 | issue=  | pages= 1483-99 | pmid=14977560 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14977560  }} </ref><ref name="pmid22282714">{{cite journal| author=Kiefer M, Unterberg A| title=The differential diagnosis and treatment of normal-pressure hydrocephalus. | journal=Dtsch Arztebl Int | year= 2012 | volume= 109 | issue= 1-2 | pages= 15-25; quiz 26 | pmid=22282714 | doi=10.3238/arztebl.2012.0015 | pmc=3265984 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22282714  }} </ref><ref name="pmid22605909">{{cite journal| author=Goldenberg MM| title=Multiple sclerosis review. | journal=P T | year= 2012 | volume= 37 | issue= 3 | pages= 175-84 | pmid=22605909 | doi= | pmc=3351877 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22605909  }} </ref>
*[[Normal pressure hydrocephalus|Normopressure hydrocephalus]]
*[[ALS]]
*[[Meningitis]]
*Transverse [[Myelitis]]
*[[Stroke|Cerebrovascular accidents]]
*[[Brain tumor]]
*[[Peripheral neuropathy]]
*[[Fibromyalgia]]
*[[Parkinsonism]]
*[[Cerebellar ataxia]]
*[[Multiple sclerosis]]
*Neurogenic bladder
*Spinal cord space occupying lesion (e.g., [[tumors]], [[cysts]], [[Abscess|abscesses]])
*[[Guillain-Barré syndrome|Guillian Barre]]
*[[Polyneuropathy|Chronic demyelinating polyneuropathy]]


What can make PTS difficult to diagnose is the fact that symptoms can often first appear long after the actual cause of the syrinx occurred, e.g. a car accident occurring and then the patient first experiencing PTS symptoms such as pain, loss of sensation, reduced ability on the skin to feel varying degrees of hot and cold, a number of months after car accident.
==Risk Factors==
  '''The main risk factors associated with the development of syringomyelia are based on the underlying causes:'''


Some cases of [[syringomyelia]] are familial, although this is rare. In addition, one form of the disorder involves a part of the brain called the [[brainstem]]. The brainstem controls many of our vital functions, such as respiration and heartbeat. When syrinxes affect the [[brainstem]], the condition is called [[syringobulbia]].
*'''Congenital Malformations:''' [[Chiari malformation|Chiari malformations]], [[Klippel-Feil syndrome|Klippel Feil syndrome]], [[congenital scoliosis]]<ref name="pmid14580664">{{cite journal| author=Carlson MD, Muraszko KM| title=Chiari I malformation with syrinx. | journal=Pediatr Neurol | year= 2003 | volume= 29 | issue= 2 | pages= 167-9 | pmid=14580664 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14580664  }} </ref><ref name="pmid12838099">{{cite journal| author=Ozerdemoglu RA, Denis F, Transfeldt EE| title=Scoliosis associated with syringomyelia: clinical and radiologic correlation. | journal=Spine (Phila Pa 1976) | year= 2003 | volume= 28 | issue= 13 | pages= 1410-7 | pmid=12838099 | doi=10.1097/01.BRS.0000067117.07325.86 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12838099  }} </ref><ref name="pmid25092126">{{cite journal| author=Guo A, Chitayat D, Blaser S, Keating S, Shannon P| title=Fetal syringomyelia. | journal=Acta Neuropathol Commun | year= 2014 | volume= 2 | issue=  | pages= 91 | pmid=25092126 | doi=10.1186/s40478-014-0091-0 | pmc=4167126 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25092126  }} </ref>
*'''Post-infectious:''' Development of [[arachnoiditis]] and development of dural abscess.<ref name="pmid25896347">{{cite journal| author=Garg RK, Malhotra HS, Gupta R| title=Spinal cord involvement in tuberculous meningitis. | journal=Spinal Cord | year= 2015 | volume= 53 | issue= 9 | pages= 649-57 | pmid=25896347 | doi=10.1038/sc.2015.58 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25896347  }} </ref>
*'''Post-traumatic:''' Increasing age at time of trauma , cervical and thoracic injuries compared with lumbar, displaced fractures, and spinal instrumentation without decompression.<ref name="pmid12201398">{{cite journal| author=Vannemreddy SS, Rowed DW, Bharatwal N| title=Posttraumatic syringomyelia: predisposing factors. | journal=Br J Neurosurg | year= 2002 | volume= 16 | issue= 3 | pages= 276-83 | pmid=12201398 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12201398  }} </ref><ref name="pmid22487955">{{cite journal| author=Ko HY, Kim W, Kim SY, Shin MJ, Cha YS, Chang JH et al.| title=Factors associated with early onset post-traumatic syringomyelia. | journal=Spinal Cord | year= 2012 | volume= 50 | issue= 9 | pages= 695-8 | pmid=22487955 | doi=10.1038/sc.2012.35 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22487955  }} </ref>
*'''Post-inflammatory:''' [[Multiple sclerosis]], [[neurosarcoidosis]], [[Amyotrophic lateral sclerosis|Amyotrophic Lateral Sclerosis (ALS)]]<ref name="pmid17467892">{{cite journal| author=Ravaglia S, Bogdanov EI, Pichiecchio A, Bergamaschi R, Moglia A, Mikhaylov IM| title=Pathogenetic role of myelitis for syringomyelia. | journal=Clin Neurol Neurosurg | year= 2007 | volume= 109 | issue= 6 | pages= 541-6 | pmid=17467892 | doi=10.1016/j.clineuro.2007.03.007 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17467892  }} </ref>
*'''Neoplastic:''' [[Ependymoma]] and [[hemangioblastoma]] are most strongly associated with development of syringomyelia.<ref name="pmid7889696">{{cite journal| author=Lohle PN, Wurzer HA, Hoogland PH, Seelen PJ, Go KG| title=The pathogenesis of syringomyelia in spinal cord ependymoma. | journal=Clin Neurol Neurosurg | year= 1994 | volume= 96 | issue= 4 | pages= 323-6 | pmid=7889696 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7889696  }} </ref>


== Symptoms ==
==Screening==
There is no recommended screening for syringomyelia.


Syringomyelia causes a wide variety of neuropathic symptoms due to damage of the spinal cord. Patients may experience chronic pain, [[paresthesia|abnormal sensations]] and loss of sensation particularly in the hands. Some patients experience [[paralysis]] or [[paresis]] temporarily or permanently. A syrinx may also cause disruptions in the [[parasympathetic]] and [[sympathetic_nervous_system|sympathetic]] nervous systems, leading to abnormal body temperature or sweating, bowel control issues, or other problems. If the syrinx is higher up in the spinal cord or affecting the brainstem as in syringobulbia, [[Vocal_cord_paresis|vocal cord paralysis]], ipsilateral tongue wasting, [[trigeminal nerve]] sensory loss, and other signs may occur.<ref>Greenberg, David A, et al: ''Clinical Neurology''. 5th ed. Feb 9, 2002.</ref> Rarely, bladder stones can occur in the onset of weakness in the lower extremities.<ref>Nishida, Takayasu, et al. [http://sciencelinks.jp/j-east/article/200613/000020061306A0443986.php "A large bladder stone caused by syringomyelia"]. ''Japanese Journal of Clinical Urology'', Vol.60, No. 6, pp 413-415, 2006. ISSN:0385-2393.</ref>
==Epidemiology and Demographics==
===Incidence===
* More than 25% of patients with spinal cord injury progress to develop syringomyelia.<ref name="pmid12852875">{{cite journal| author=Brodbelt AR, Stoodley MA| title=Post-traumatic syringomyelia: a review. | journal=J Clin Neurosci | year= 2003 | volume= 10 | issue= 4 | pages= 401-8 | pmid=12852875 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12852875  }} </ref>


== Diagnosis ==
===Prevalence===
[[Image:Gray 111 - Vertebral column-coloured.png|120px|left|]]
[[Syringomyelia]] has a prevalence estimated at 8.4 cases per 100,000 people,<ref>Brewis M, Poskanzer DC, Rolland C, et al, "Neurological disease in an English city". ''Acta Neurologica Scand Suppl'' 24:1--89, 1966.</ref> or about 21,000 Americans, with symptoms usually beginning in young adulthood.
Physicians now use magnetic resonance imaging (MRI) to diagnose syringomyelia. The MR imager takes pictures of body structures, such as the brain and spinal cord, in vivid detail. This test will show the syrinx in the spine or any other conditions, such as the presence of a tumor. MRI is safe, painless, and informative and has greatly improved the diagnosis of syringomyelia.


The physician may order additional tests to help confirm the diagnosis. One of these is called [[electromyography]] (EMG), which measures muscle weakness. The doctor may also wish to test cerebrospinal fluid pressure levels and to analyze the cerebrospinal fluid by performing a [[lumbar puncture]]. In addition, [[computed axial tomography]] (CT) scans of a patient's head may reveal the presence of tumors and other abnormalities such as hydrocephalus.
==Natural History, Complications, and Prognosis==
===Natural History===
'''The essential Course of disease in Syringomyelia is temperamental'''. Without treatment, syringomyelia may progress to develop the worsening of neurological deficit in cases of obstructive lesions. However, the disease has a slower progression in cases of mild disease.<ref name="pmid22133172">{{cite journal| author=Singhal A, Bowen-Roberts T, Steinbok P, Cochrane D, Byrne AT, Kerr JM| title=Natural history of untreated syringomyelia in pediatric patients. | journal=Neurosurg Focus | year= 2011 | volume= 31 | issue= 6 | pages= E13 | pmid=22133172 | doi=10.3171/2011.9.FOCUS11208 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22133172  }} </ref><ref name="pmid1779249">{{cite journal| author=Mariani C, Cislaghi MG, Barbieri S, Filizzolo F, Di Palma F, Farina E et al.| title=The natural history and results of surgery in 50 cases of syringomyelia. | journal=J Neurol | year= 1991 | volume= 238 | issue= 8 | pages= 433-8 | pmid=1779249 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1779249  }} </ref><ref name="pmid4024858">{{cite journal| author=Anderson NE, Willoughby EW, Wrightson P| title=The natural history and the influence of surgical treatment in syringomyelia. | journal=Acta Neurol Scand | year= 1985 | volume= 71 | issue= 6 | pages= 472-9 | pmid=4024858 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4024858  }} </ref>


Like [[MRI]] and [[CT scan]]s, another test, called a [http://www.umm.edu/radiology/myelog.htm myelogram], takes x-ray-like pictures and requires a contrast medium or dye to do so. Since the introduction of [[MRI]] this test is rarely necessary to diagnose [[syringomyelia]].
===Complications===
Complications that can develop as a result of syringomyelia are:<ref name="pmid17684895">{{cite journal| author=Porensky P, Muro K, Ganju A| title=Nontraumatic cervicothoracic syrinx as a cause of progressive neurologic dysfunction. | journal=J Spinal Cord Med | year= 2007 | volume= 30 | issue= 3 | pages= 276-81 | pmid=17684895 | doi= | pmc=2031960 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17684895  }} </ref><ref name="pmid16676923">{{cite journal| author=Todor DR, Mu HT, Milhorat TH| title=Pain and syringomyelia: a review. | journal=Neurosurg Focus | year= 2000 | volume= 8 | issue= 3 | pages= E11 | pmid=16676923 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16676923  }} </ref><ref name="pmid23489298">{{cite journal| author=Campos WK, Almeida de Oliveira YS, Ciampi de Andrade D, Teixeira MJ, Fonoff ET| title=Spinal cord stimulation for the treatment of neuropathic pain related to syringomyelia. | journal=Pain Med | year= 2013 | volume= 14 | issue= 5 | pages= 767-8 | pmid=23489298 | doi=10.1111/pme.12064 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23489298  }} </ref><ref name="pmid8581529">{{cite journal| author=Samuelsson L, Lindell D| title=Scoliosis as the first sign of a cystic spinal cord lesion. | journal=Eur Spine J | year= 1995 | volume= 4 | issue= 5 | pages= 284-90 | pmid=8581529 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8581529  }} </ref><ref name="pmid12544968">{{cite journal| author=Whitaker C, Schoenecker PL, Lenke LG| title=Hyperkyphosis as an indicator of syringomyelia in idiopathic scoliosis: a case report. | journal=Spine (Phila Pa 1976) | year= 2003 | volume= 28 | issue= 1 | pages= E16-20 | pmid=12544968 | doi=10.1097/01.BRS.0000038240.88287.0B | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12544968  }} </ref><ref name="pmid3343678">{{cite journal| author=Nogués MA, Gené R| title=Diaphragm weakness and syringomyelia. | journal=J R Soc Med | year= 1988 | volume= 81 | issue= 1 | pages= 59 | pmid=3343678 | doi= | pmc=1291446 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3343678  }} </ref><ref name="pmid22397655">{{cite journal| author=McCool FD, Tzelepis GE| title=Dysfunction of the diaphragm. | journal=N Engl J Med | year= 2012 | volume= 366 | issue= 10 | pages= 932-42 | pmid=22397655 | doi=10.1056/NEJMra1007236 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22397655  }} </ref><ref name="pmid8321799">{{cite journal| author=Ellis C, Curzen N, Katifi H| title=Obstructive sleep apnoea associated with syringomyelia. | journal=Postgrad Med J | year= 1993 | volume= 69 | issue= 810 | pages= 308-11 | pmid=8321799 | doi= | pmc=2399647 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8321799  }} </ref><ref name="pmid21982271">{{cite journal| author=Benjamin MD, Santiago J, Hebert JC, Thirion S, Ranaivojaona S, Alvarez C et al.| title=[Hemihypertrophy and scoliosis revealing a Chiari 1 malformation with syringomyelia]. | journal=Arch Pediatr | year= 2011 | volume= 18 | issue= 11 | pages= 1210-5 | pmid=21982271 | doi=10.1016/j.arcped.2011.08.022 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21982271  }} </ref><ref name="pmid16369334">{{cite journal| author=Cullen AB, Ofluoglu O, Donthineni R| title=Neuropathic arthropathy of the shoulder (Charcot shoulder). | journal=MedGenMed | year= 2005 | volume= 7 | issue= 1 | pages= 29 | pmid=16369334 | doi= | pmc=1681408 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16369334  }} </ref><ref name="pmid3357616">{{cite journal| author=Brown LK, Stacy C, Schick A, Miller A| title=Obstructive sleep apnea in syringomyelia-syringobulbia. | journal=N Y State J Med | year= 1988 | volume= 88 | issue= 3 | pages= 152-4 | pmid=3357616 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3357616  }} </ref>
*[[Complex regional pain syndrome]]
*[[Scoliosis]]
*[[Diaphragmatic paralysis]]
*[[Spastic ataxia]]
*Obsturctive sleep apnea
*Central [[respiratory failure]]
*[[Horner's syndrome]]
*Skin ulceration
*[[Raynaud's syndrome]]
*[[Hemihypertrophy]]
*[[Upper motor neurone lesion]]
*[[Lower motor neurone lesion]]
*Charcot's joints
*[[Raynaud's phenomenon]]


The possible causes are [[trauma]], [[tumor]]s and congenital defects. It is most usually observed in the part of the spinal cord corresponding to the neck area. Symptoms are due to spinal cord damage and are: pain, decreased sensation of touch, weakness and loss of muscle tissue. The diagnosis is confirmed with a spinal CT, myelogram or MRI of the [[spinal cord]]. The cavity may be reduced by surgical decompression.
===Prognosis===
The prognosis of syringomyelia varies depending on factors such as cause, extent of neurological damage, presence of complications, and general health status of patient before undergoing surgical treatment.<ref name="pmid22794351">{{cite journal| author=Klekamp J| title=Treatment of posttraumatic syringomyelia. | journal=J Neurosurg Spine | year= 2012 | volume= 17 | issue= 3 | pages= 199-211 | pmid=22794351 | doi=10.3171/2012.5.SPINE11904 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22794351  }} </ref><ref name="pmid24633622">{{cite journal| author=Nagoshi N, Iwanami A, Toyama Y, Nakamura M| title=Factors contributing to improvement of syringomyelia after foramen magnum decompression for Chiari type I malformation. | journal=J Orthop Sci | year= 2014 | volume= 19 | issue= 3 | pages= 418-23 | pmid=24633622 | doi=10.1007/s00776-014-0555-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24633622  }} </ref><ref name="pmid7815110">{{cite journal| author=Sgouros S, Williams B| title=A critical appraisal of drainage in syringomyelia. | journal=J Neurosurg | year= 1995 | volume= 82 | issue= 1 | pages= 1-10 | pmid=7815110 | doi=10.3171/jns.1995.82.1.0001 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7815110  }} </ref>
*Patients with mild neurological deficit may respond better to surgical management.
*Early management of syringomyelia in cases of trauma is associated with decreased morbidity.
*'''The symptoms of 80% of patients usually end due to their response to surgical treatment, as well a mild cure.'''
*20% of patients gets worse despite treatment in cases of advanced disease.
*Recurrence is not uncommon in syringomyelia.


Furthermore, evidence also suggests that impact injuries to the thorax area highly correlate with the occurrence of a cervical-located syrinx.
==History and Symptoms==
Specific areas of focus when obtaining the history such as onset, duration and progression of symptoms; history of trauma; associated conditions like [[multiple sclerosis]], transverse myelitis, [[amyotrophic lateral sclerosis]], [[sarcoidosis]], congenital malformation, infection such as [[meningitis]] or [[neoplasia]] ([[ependymoma]], [[meningioma]], [[hemangioblastoma]]) may help diagnosing syringomyelia. Symptoms depends on the anatomical site of spinal cord involved.<ref name="pmid15808720">{{cite journal| author=Koyanagi I, Iwasaki Y, Hida K, Houkin K| title=Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. | journal=Surg Neurol | year= 2005 | volume= 63 | issue= 4 | pages= 350-5; discussion 355-6 | pmid=15808720 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15808720  |first=|date=|via=}} </ref><ref name="pmid7205304">{{cite journal| author=Shannon N, Symon L, Logue V, Cull D, Kang J, Kendall B| title=Clinical features, investigation and treatment of post-traumatic syringomyelia. | journal=J Neurol Neurosurg Psychiatry | year= 1981 | volume= 44 | issue= 1 | pages= 35-42 | pmid=7205304 | doi= | pmc=490816 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7205304  }} </ref><ref name="pmid19119392">{{cite journal| author=Rene Hudson B, Cook C, Goode A| title=Identifying myelopathy caused by thoracic syringomyelia: a case report. | journal=J Man Manip Ther | year= 2008 | volume= 16 | issue= 2 | pages= 82-8 | pmid=19119392 | doi=10.1179/106698108790818512 | pmc=2565119 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19119392  }} </ref><ref name="pmid27169036">{{cite journal| author=Kim HG, Oh HS, Kim TW, Park KH| title=Clinical Features of Post-Traumatic Syringomyelia. | journal=Korean J Neurotrauma | year= 2014 | volume= 10 | issue= 2 | pages= 66-9 | pmid=27169036 | doi=10.13004/kjnt.2014.10.2.66 | pmc=4852605 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27169036  }} </ref><ref name="pmid9521293">{{cite journal| author=Jones J, Wolf S| title=Neuropathic shoulder arthropathy (Charcot joint) associated with syringomyelia. | journal=Neurology | year= 1998 | volume= 50 | issue= 3 | pages= 825-7 | pmid=9521293 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9521293  }} </ref><ref name="pmid8558154">{{cite journal| author=Schurch B, Wichmann W, Rossier AB| title=Post-traumatic syringomyelia (cystic myelopathy): a prospective study of 449 patients with spinal cord injury. | journal=J Neurol Neurosurg Psychiatry | year= 1996 | volume= 60 | issue= 1 | pages= 61-7 | pmid=8558154 | doi= | pmc=486191 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8558154  }} </ref>
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
|+
! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Anatomical site}}
! style="background: #4479BA; width: 350px;" | {{fontcolor|#FFF|Early symptoms}}
! style="background: #4479BA; width: 350px;" | {{fontcolor|#FFF|Late symptoms}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Cervical'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Inability to feel hot or cold
*Decreased ability to feel pain sensation
*Increased or decreased sweating
*Muscle weakness ++
| style="padding: 5px 5px; background: #F5F5F5;" |
*Shoulder [[Arthralgia|joint pain]], swelling and loss of function
*Muscle paralysis
*Loss of all sensations in hands, forearm, arm and upper back
*Headaches with coughing or straining
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Thoracic'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Decreased to absent temperature sensation in trunk region
*Abnormal pain sensation
*Muscle weakness
*Abnormal sweating
| style="padding: 5px 5px; background: #F5F5F5;" |
*Muscle paralysis
*Loss of pain and temperature sensation
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Lumbar'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Lower limb muscle weakness
*Decreased to absent temperature sensation
*Abnormal pain sensation
*Joint sweling, pain and redness (charcot joints)
| style="padding: 5px 5px; background: #F5F5F5;" |
*Loss of vibration, position sense and two point discrimination(recurrent falls)
*Failure to maintain balance
*Absent pain and temperature sensation
*Muscle paralysis
*Impaired ambulation
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Sacral'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Bladder or bladder dysfunction 
*Sexual dysfunction (erectile dysfunction)
*Tingling and numbness in foot or anogenital region 
*Muscle weakness ++
| style="padding: 5px 5px; background: #F5F5F5;" |
*Loss of penile erection
*Loss of ankle plantar flexion(S1)
*Urinary or fecal incontinence
*Loss of temperature and pain sensation in foot and perineum
|-
|}


== Treatment ==
==Physical Examination==
The first step after diagnosis is finding a neurosurgeon who is experienced in the treatment of syringomyelia. [[Surgery]] is the only viable treatment for syringomyelia, and a [[Neurosurgery|neurosurgeon]] is the only specialist qualified to provide a fully informed recommendation. Not all patients will advance to the stage where surgery is needed. Evaluation of the condition is often difficult because syringomyelia can remain stationary for long periods of time, and in some cases progress rapidly.
'''The following includes how syringomyelia might be revealed''' :<ref name="pmid19030463">{{cite journal| author=Janicki JA, Alman B| title=Scoliosis: Review of diagnosis and treatment. | journal=Paediatr Child Health | year= 2007 | volume= 12 | issue= 9 | pages= 771-6 | pmid=19030463 | doi= | pmc=2532872 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19030463  }} </ref><ref name="pmid11075799">{{cite journal| author=Kuruvilla A| title=Syringomyelia and respiratory failure. | journal=Lancet | year= 2000 | volume= 356 | issue= 9241 | pages= 1609 | pmid=11075799 | doi=10.1016/S0140-6736(05)74457-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11075799  }} </ref><ref name="pmid3147011">{{cite journal| author=Bullock R, Todd NV, Easton J, Hadley D| title=Isolated central respiratory failure due to syringomyelia and Arnold-Chiari malformation. | journal=BMJ | year= 1988 | volume= 297 | issue= 6661 | pages= 1448-9 | pmid=3147011 | doi= | pmc=1835174 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3147011  }} </ref><ref name="pmid19623519">{{cite journal| author=Viswanatha B| title=Syringomyelia with syringobulbia presenting as vocal fold paralysis. | journal=Ear Nose Throat J | year= 2009 | volume= 88 | issue= 7 | pages= E20 | pmid=19623519 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19623519  }} </ref><ref name="pmid19547980">{{cite journal| author=Nacir B, Arslan Cebeci S, Cetinkaya E, Karagoz A, Erdem HR| title=Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformation. | journal=Rheumatol Int | year= 2010 | volume= 30 | issue= 7 | pages= 979-83 | pmid=19547980 | doi=10.1007/s00296-009-1013-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19547980  }} </ref><ref name="pmid19602995">{{cite journal| author=Cardoso M, Keating RF| title=Neurosurgical management of spinal dysraphism and neurogenic scoliosis. | journal=Spine (Phila Pa 1976) | year= 2009 | volume= 34 | issue= 17 | pages= 1775-82 | pmid=19602995 | doi=10.1097/BRS.0b013e3181b07914 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19602995  }} </ref>
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
|+
! style="background: #4479BA; width: 150px;" | {{fontcolor|#FFF|Anatomical site}}
! style="background: #4479BA; width: 350px;" | {{fontcolor|#FFF|Physical examination}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Syringobulbia'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Facial sensory loss (sensory nucleus of [[Trigeminal nerve|5th nerve]])
*Absent gag reflex ([[Glossopharyngeal nerve|9th]] and [[Vagus nerve|10th nerve]])
*[[Hoarseness]] of voice ([[Vocal cords|vocal cord]] paralysis)
*Decreased muscle strength of [[Sternocleidomastoid muscle|sternocleidomastoid]] and [[trapezius muscle]] ([[Accessory nerve|11th nerve)]]
*Atrophy and weakness of tongue muscle ([[Hypoglossal nerve|12th nerve]])
*[[Apnea]] (respiratory failure)
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Cervical'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Anhydrosis, [[miosis]] and [[ptosis]] ([[Horner's syndrome]])
*Loss of temperature and pain sensation in cervical dermatomes
*[[Claw hand]]
*Decreased muscle strength in hands, arms and shoulders
*Decreased to absent deep tendon reflexes in upper limb
*Muscle atrophy of hands, arms and shoulders
*Tenderness, [[erythema]], swelling and joint resorption (charcot joints)
|-
| style="padding: 5px 5px; background: #DCDCDC;" |'''Thoracic'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Scoliosis]]
*Decreased to absent pain and temperature sensation in thoracic dermatomes
*[[Muscle atrophy]] ++
*Absent superficial abdominal reflexes
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Lumbar'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Lower limb spasticity
*[[Hyperreflexia]] or [[Areflexia]]
*[[Babinski's sign]] ++
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''Sacral'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Decreased to absent pain and temperature sensation in sacral dermatomes
*Loss  of rectal tone
*Absent [[cremasteric reflex]]
*[[Scoliosis]]
|-
|}


Surgery is not always recommended for syringomyelia patients. For many patients, the main treatment is analgesia. A typical treatment of syringomyelia involving severe chronic pain would involve two or more medications. One medication for "classical" back pain such as a weak or strong [[opioid]] (e.g. [[tramadol]] and [[oxycodone|Oxycontin]] respectively) combined with a medication to combat any neuropathic pain symptoms such as shooting and stabbing pains (e.g. [[gabapentin|Neurontin]] or [[pregabalin|Lyrica]]). In addition, [[paracetamol]] can be used to combat headaches. Such long term treatment of chronic pain should be monitored with blood tests to assess any adverse effects of the medication on the liver, with the dosages being then changed accordingly, depending on the outcome of such blood tests.
==Imaging Findings==
Imaging studies that may help diagnose syringomyelia, its cause and complications include:<ref name="pmid2633734">{{cite journal| author=Dowling RJ, Tress BM| title=MRI--the investigation of choice in syringomyelia? | journal=Australas Radiol | year= 1989 | volume= 33 | issue= 4 | pages= 337-43 | pmid=2633734 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2633734  }} </ref><ref name="pmid11071442">{{cite journal| author=Bogdanov EI, Ibatullin MM, Mendelevich EG| title=Spontaneous drainage in syringomyelia: magnetic resonance imaging findings. | journal=Neuroradiology | year= 2000 | volume= 42 | issue= 9 | pages= 676-8 | pmid=11071442 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11071442  }} </ref><ref name="pmid21878699">{{cite journal| author=Terae S, Hida K, Sasaki H| title=[Diagnosis of syringomyelia and its classification on the basis of symptoms, radiological appearance, and causative disorders]. | journal=Brain Nerve | year= 2011 | volume= 63 | issue= 9 | pages= 969-77 | pmid=21878699 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21878699  }} </ref>
===MRI===
Spinal MRI may help to diagnose and follow up syringomyelia. It is characterized by the following findings:<ref name="pmid2633734">{{cite journal| author=Dowling RJ, Tress BM| title=MRI--the investigation of choice in syringomyelia? | journal=Australas Radiol | year= 1989 | volume= 33 | issue= 4 | pages= 337-43 | pmid=2633734 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2633734  }} </ref><ref name="pmid17890352">{{cite journal| author=Hofkes SK, Iskandar BJ, Turski PA, Gentry LR, McCue JB, Haughton VM| title=Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. | journal=Radiology | year= 2007 | volume= 245 | issue= 2 | pages= 532-40 | pmid=17890352 | doi=10.1148/radiol.2452061096 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17890352  }} </ref><ref name="pmid7668137">{{cite journal| author=Tanghe HL| title=Magnetic resonance imaging (MRI) in syringomyelia. | journal=Acta Neurochir (Wien) | year= 1995 | volume= 134 | issue= 1-2 | pages= 93-9 | pmid=7668137 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7668137  }} </ref><ref name="pmid3936900">{{cite journal| author=Aichner F, Poewe W, Rogalsky W, Wallnöfer K, Willeit J, Gerstenbrand F| title=Magnetic resonance imaging in the diagnosis of spinal cord diseases. | journal=J Neurol Neurosurg Psychiatry | year= 1985 | volume= 48 | issue= 12 | pages= 1220-9 | pmid=3936900 | doi= | pmc=1028605 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3936900  }} </ref><ref name="pmid25905945">{{cite journal| author=Timpone VM, Patel SH| title=MRI of a syrinx: is contrast material always necessary? | journal=AJR Am J Roentgenol | year= 2015 | volume= 204 | issue= 5 | pages= 1082-5 | pmid=25905945 | doi=10.2214/AJR.14.13310 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25905945  }} </ref>
*Presence of [[Syrinx (medicine)|syrinx]] in the [[spinal cord]], its extent, location and complications
*Enlargement of [[central canal]] of [[spinal cord]]
*Presence of [[Syrinx (medicine)|syrinx]] in the brain stem (syringobulbia)
*Presence of assocaited congenital anomalies such as [[chiari malformation]], [[Klippel-Feil syndrome|klippel feil syndrome]], [[spina bifida]], [[Tethered spinal cord syndrome|tethered cord syndrome]]
*Presence of [[tumors]] such as [[ependymoma]]
*May be used for follow up post surgery


Surgery of the spinal cord has certain, characteristic risks associated with it and the benefits of a surgical procedure on the spine have to be weighed up against the possible complications associated with any procedure. Surgical treatment is aimed at correcting the condition that allowed the syrinx to form. It is vital to bear in mind that the drainage of a syrinx does not necessarily mean the  elimination of the syrinx-related symptoms, but rather is aimed at stopping progression. In simple terms this means that a syrinx with the main symptom of pain, may not actually lead to any reduction (or only a part reduction) in the pain symptoms after the syrinx has been drained! In cases involving an [[Arnold-Chiari malformation]], the main goal of surgery is to provide more space for the [[cerebellum]] at the base of the skull and upper [[Neck#Anatomy of the human neck|cervical spine]] without entering the brain or spinal cord. This often results in flattening or disappearance of the primary syrinx or cavity, over time, as the normal flow of [[cerebrospinal fluid]] is restored. If a [[tumor]] is causing syringomyelia, removal of the tumor is the treatment of choice and almost always eliminates the syrinx.
===CT===
Syringomyelia may be diagnosed as an incidental finding on CT scan. However, delayed CT scan may have a diagnostic importance in early cases of syringomyelia without clinical manifestaions.<ref name="pmid2620591">{{cite journal| author=Wang GP| title=[Diagnostic value of delayed enhancement CT scan for syringomyelia]. | journal=Zhonghua Shen Jing Jing Shen Ke Za Zhi | year= 1989 | volume= 22 | issue= 4 | pages= 225-8, 253 | pmid=2620591 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2620591  }} </ref><ref name="pmid2023674">{{cite journal| author=Toriyama T, Kawauchi M, Koike J, Harada T, Murata A, Kyoshima K| title=[A case of disproportionately large communicating fourth ventricle (DLCFV) combined with syringomyelia and Chiari malformation]. | journal=No Shinkei Geka | year= 1991 | volume= 19 | issue= 2 | pages= 167-72 | pmid=2023674 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2023674  }} </ref> Metrizimed CT scan using non-ionic contrast may be used to identify a [[Syrinx (medicine)|syrinx]] in postinflammatory conditions (postinfectious and posthemorrhagic arachnoiditis).<ref name="pmid6410816">{{cite journal| author=Simmons JD, Norman D, Newton TH| title=Preoperative demonstration of postinflammatory syringomyelia. | journal=AJNR Am J Neuroradiol | year= 1983 | volume= 4 | issue= 3 | pages= 625-8 | pmid=6410816 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6410816  }} </ref><ref name="pmid3176472">{{cite journal| author=La Haye PA, Batzdorf U| title=Posttraumatic syringomyelia. | journal=West J Med | year= 1988 | volume= 148 | issue= 6 | pages= 657-63 | pmid=3176472 | doi= | pmc=1026203 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3176472  }} </ref> In addition, [[computed axial tomography]] (CT) scans of a patient's head may reveal the presence of [[tumors]] and other abnormalities such as hydrocephalus.


Surgery results in stabilization or modest improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury. Recurrence of syringomyelia after surgery may make additional operations necessary; these may not be completely successful over the long term.
===X ray===
There are no specific [[X ray]] findings associated with syringomyelia.


In some patients it may also be necessary to drain the syrinx, which can be accomplished using a [[catheter]], drainage tubes, and valves. This system is also known as a [[Shunt (medical)|shunt]]. Shunts are used in both the communicating and noncommunicating forms of the disorder. First, the surgeon must locate the syrinx. Then, the shunt is placed into it with the other end draining [[cerebrospinal fluid]] (CSF) into a cavity, usually the abdomen. This type of shunt is called a ventriculoperitoneal shunt and is particularly useful in cases involving [[hydrocephalus]]. By draining syrinx fluid, a shunt can arrest the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.
===Myelogram===
[[Myelogram]] is rarely used to diagnose syringomyelia. It uses a contrast material combined with x ray or CT to image spinal cord in case of syringomyelia. However, CT metrimised myelography is more sensitive to diagnose syringomyelia as compared to conventional myelography.<ref name="pmid3176472">{{cite journal| author=La Haye PA, Batzdorf U| title=Posttraumatic syringomyelia. | journal=West J Med | year= 1988 | volume= 148 | issue= 6 | pages= 657-63 | pmid=3176472 | doi= | pmc=1026203 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3176472  }} </ref><ref name="pmid3101451">{{cite journal| author=Li KC, Chui MC| title=Conventional and CT metrizamide myelography in Arnold-Chiari I malformation and syringomyelia. | journal=AJNR Am J Neuroradiol | year= 1987 | volume= 8 | issue= 1 | pages= 11-7 | pmid=3101451 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3101451  }} </ref>


The decision to use a shunt requires extensive discussion between doctor and patient, as this procedure carries with it greater risk of injury to the spinal cord, infection, blockage, or hemorrhage and may not necessarily work for all patients.  Draining the syrinx more quickly does not produce better outcomes, but a shunt may be required if the fluid in the syrinx is otherwise unable to drain.
==Other Diagnostic Studies==
===Electromyography===
[[EMG]] findings of syringomyelia include increased excitability of spinal [[motor neurons]]. However, the findings are non specific and does not hold diagnostic importance in case of syringomyelia.<ref name="pmid10567077">{{cite journal| author=Nogués MA, Stålberg E| title=Electrodiagnostic findings in syringomyelia. | journal=Muscle Nerve | year= 1999 | volume= 22 | issue= 12 | pages= 1653-9 | pmid=10567077 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10567077 }} </ref><ref name="pmid7359152">{{cite journal| author=Schwartz MS, Stålberg E, Swash M| title=Pattern of segmental motor involvement in syringomyelia: a single fibre EMG study. | journal=J Neurol Neurosurg Psychiatry | year= 1980 | volume= 43 | issue= 2 | pages= 150-5 | pmid=7359152 | doi= | pmc=490490 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7359152  }} </ref>
===Lumbar Puncture===
Role of [[lumbar puncture]] in syringomyelia is controversial since it may result in herniation in cases of [[Chiari malformation]]. However, it may be used by some clinicians as a diagnostic tool to look for [[CSF|CSF pressure]] and signs of [[inflammation]] based on individual patient assessment.<ref name="pmid19119392">{{cite journal| author=Rene Hudson B, Cook C, Goode A| title=Identifying myelopathy caused by thoracic syringomyelia: a case report. | journal=J Man Manip Ther | year= 2008 | volume= 16 | issue= 2 | pages= 82-8 | pmid=19119392 | doi=10.1179/106698108790818512 | pmc=2565119 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19119392  }} </ref>


In the case of trauma-related syringomyelia, the surgeon operates at the level of the initial injury. The syrinx collapses at surgery but a tube or shunt is usually necessary to prevent re-expansion.
==Medical Therapy==


Drugs have no curative value as a treatment for syringomyelia. [[Radiation]] is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.
Drugs have no curative value as a treatment for syringomyelia. The role of medical therapy in management of syringomyelia is for treatment of underlying conditions or management of associated symptoms.
===Treatment of Underlying Conditions===
Radiation and [[chemotherapy]] may be utilized for management of an underlying spinal cord tumor.  
[[Antibiotics]] may be used to treat an underlying infection ([[meningitis]] or [[encephalitis]]).
===Management of Symptoms===
*[[Analgesics]] may be used to control pain.
*[[GABA antagonist|GABA inhibitors]] [[neurontin]] [[Gabapentin|pregebalin]] may be used to control [[peripheral neuropathy]].
*Drugs may be used for urinary or fecal incontinence or [[neurogenic bladder]] (e.g., [[oxybutynin]], [[prazosin]], [[loperamide]], [[atropine]])<ref name="pmid8727819">{{cite journal| author=Milhorat TH, Kotzen RM, Mu HT, Capocelli AL, Milhorat RH| title=Dysesthetic pain in patients with syringomyelia. | journal=Neurosurgery | year= 1996 | volume= 38 | issue= 5 | pages= 940-6; discussion 946-7 | pmid=8727819 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8727819  }} </ref>


In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.
In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.


Since the natural history of syringomyelia is poorly understood, a conservative approach may be recommended. When surgery is not yet advised, patients should be carefully monitored by a neurologist or neurosurgeon. Periodic MRI's and physical evaluations should be scheduled at the recommendation of a qualified physician.
==Surgery==
Surgery is not always required for syringomyelia. Indications for surgical management of syringomyelia include the following:<ref name="pmid27029329">{{cite journal| author=Zuev AA, Pedyash NV, Epifanov DS, Kostenko GV| title=[Results of surgical treatment of syringomyelia associated with Chiari 1 malformation. An analysis of 125 cases]. | journal=Zh Vopr Neirokhir Im N N Burdenko | year= 2016 | volume= 80 | issue= 1 | pages= 27-34 | pmid=27029329 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27029329  }} </ref><ref name="pmid15715455">{{cite journal| author=Mueller D, Oro' JJ| title=Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. | journal=Neurosurg Focus | year= 2005 | volume= 18 | issue= 2 | pages= ECP2 | pmid=15715455 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15715455  }} </ref>
*Progressive neurological deterioration and worsening of symptoms
*Presence of [[tumor]] causing obstruction
*Congenital malformation([[Chiari malformation]])
*Traumatic injury to [[spinal cord]]
Surgical options used to treat syringomyelia include:<ref name="pmid1513429">{{cite journal| author=Milhorat TH, Johnson WD, Miller JI, Bergland RM, Hollenberg-Sher J| title=Surgical treatment of syringomyelia based on magnetic resonance imaging criteria. | journal=Neurosurgery | year= 1992 | volume= 31 | issue= 2 | pages= 231-44; discussion 244-5 | pmid=1513429 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1513429  }} </ref><ref name="pmid17856740">{{cite journal| author=Frazier CH, Rowe SN| title=THE SURGICAL TREATMENT OF SYRINGOMYELIA. | journal=Ann Surg | year= 1936 | volume= 103 | issue= 4 | pages= 481-97 | pmid=17856740 | doi= | pmc=1391054 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17856740  }} </ref>
===Posterior fossa decompression===
*Usually performed in [[Chiari malformation]] patients to expand the size of the posterior fossa and reverse tonsillar herniation resulting in relief of the obstructive lesion.<ref name="pmid15715455">{{cite journal| author=Mueller D, Oro' JJ| title=Prospective analysis of self-perceived quality of life before and after posterior fossa decompression in 112 patients with Chiari malformation with or without syringomyelia. | journal=Neurosurg Focus | year= 2005 | volume= 18 | issue= 2 | pages= ECP2 | pmid=15715455 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15715455  }} </ref><ref name="pmid23176335">{{cite journal| author=Batzdorf U, McArthur DL, Bentson JR| title=Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients. | journal=J Neurosurg | year= 2013 | volume= 118 | issue= 2 | pages= 232-42 | pmid=23176335 | doi=10.3171/2012.10.JNS12305 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23176335  }} </ref>
*This procedure may be combined with shunt, creating a large subarachanoid cistern to drain the [[Syrinx (medicine)|syrinx]] or in conjunction with duroplasty in which [[dura]] is opened after bony removal of [[Occiput|posterior occiput]] and posterior arch of C1 vertebra and a graft is sewed to [[dura]] effectively creating extra space in posterior fossa.<ref name="pmid23176335">{{cite journal| author=Batzdorf U, McArthur DL, Bentson JR| title=Surgical treatment of Chiari malformation with and without syringomyelia: experience with 177 adult patients. | journal=J Neurosurg | year= 2013 | volume= 118 | issue= 2 | pages= 232-42 | pmid=23176335 | doi=10.3171/2012.10.JNS12305 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23176335  }} </ref><ref name="pmid8752413">{{cite journal| author=Iwasaki Y, Hida K, Koyanagi I, Kuroda S, Abe H| title=[Surgical treatment for syringomyelia associated with Chiari malformation]. | journal=Rinsho Shinkeigaku | year= 1995 | volume= 35 | issue= 12 | pages= 1409-11 | pmid=8752413 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8752413  }} </ref>
*Another procedure used along with posterior fossa decompression and duraplasty to help relieve obstruction is tonsillar resection or shrinkage.<ref name="pmid23695377">{{cite journal| author=Galarza M, Gazzeri R, Alfieri A, Martínez-Lage JF| title="Triple R" tonsillar technique for the management of adult Chiari I malformation: surgical note. | journal=Acta Neurochir (Wien) | year= 2013 | volume= 155 | issue= 7 | pages= 1195-201 | pmid=23695377 | doi=10.1007/s00701-013-1749-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23695377  }} </ref><ref name="pmid23251294">{{cite journal| author=Bao C, Yang F, Liu L, Wang B, Li D, Gu Y et al.| title=Surgical treatment of Chiari I malformation complicated with syringomyelia. | journal=Exp Ther Med | year= 2013 | volume= 5 | issue= 1 | pages= 333-337 | pmid=23251294 | doi=10.3892/etm.2012.784 | pmc=3524293 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23251294  }} </ref>


== Research ==
===Shunt formation===
The precise causes of syringomyelia are still unknown. Scientists at the National Institute of Neurological Disorders and Stroke in Bethesda, Maryland, and at grantee institutions across the country continue to explore the mechanisms that lead to the formation of syrinxes in the spinal cord. For instance, Institute investigators have found that as the heart beats, the syrinx fluid is abruptly forced downward. They have also demonstrated the presence of a block to the free flow of cerebrospinal fluid that normally occurs in and out of the head during each heartbeat. Duke University is conducting research to see if syringomyelia might be genetic.<ref>"Information about a Genetic Research Study for Chiari Type I Malformation (CMI) with or without Syringomyelia."</ref>
Shunts are placed in order to drain the syrinx and alleviate symptoms such as headache and chronic pain syndromes. The choice of shunt is determined by the neurosurgeon; however, the following types of shunts may be performed:<ref name="pmid8752413">{{cite journal| author=Iwasaki Y, Hida K, Koyanagi I, Kuroda S, Abe H| title=[Surgical treatment for syringomyelia associated with Chiari malformation]. | journal=Rinsho Shinkeigaku | year= 1995 | volume= 35 | issue= 12 | pages= 1409-11 | pmid=8752413 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8752413  }} </ref><ref name="pmid6747690">{{cite journal| author=Barbaro NM, Wilson CB, Gutin PH, Edwards MS| title=Surgical treatment of syringomyelia. Favorable results with syringoperitoneal shunting. | journal=J Neurosurg | year= 1984 | volume= 61 | issue= 3 | pages= 531-8 | pmid=6747690 | doi=10.3171/jns.1984.61.3.0531 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6747690  }} </ref><ref name="pmid3267280">{{cite journal| author=Williams B, Page N| title=Surgical treatment of syringomyelia with syringopleural shunting. | journal=Br J Neurosurg | year= 1987 | volume= 1 | issue= 1 | pages= 63-80 | pmid=3267280 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3267280  }} </ref>


Surgical techniques are also being refined by the neurosurgical research community. In one treatment approach currently being evaluated, neurosurgeons perform a decompressive procedure where the dura mater, a tough membrane covering the cerebellum and spinal cord, is enlarged with a graft. Like altering a suit of clothing, this procedure expands the area around the cerebellum and spinal cord, thus improving the flow of cerebrospinal fluid and eliminating the syrinx.
'''Ventriculoperitoneal shunt'''
*Performed in patients with communicating syrinxes with hydrocephalus due to increased ventricular pressure.
*This surgery is more commonly associated with Chiari II malformations
'''Syringosubarachnoid shunt'''
*Performed more commonly in non-communicating syrinxes and is effective in rapidly reducing the size of the syrinx.
*This shunt drains the syrinx into the subarachnoid space, and the CSF is subsequently reabsorbed by the arachnoid villi.
'''Syringoperitoneal shunt'''
*Drainage of syrinx into the peritoneal cavity.


It is also important to understand the role of birth defects in the development of hindbrain malformations that can lead to syringomyelia. Learning when these defects occur during the development of the fetus can help us understand this and similar disorders, and may lead to preventive treatment that can stop the formation of many birth abnormalities. Dietary supplements of folic acid during pregnancy have already been found to reduce the number of cases of certain birth defects.
===Removal of obstruction===
*In cases of [[tumors]] or [[arachnoid cysts]] as the underlying cause of the obstructive precursor lesion to syrinx formation, removal of said mass lesion may reverse the symptoms and reduce syrinx size.<ref name="pmid16148987">{{cite journal| author=Fox B, Muzumdar D, DeMonte F| title=Resolution of tonsillar herniation and cervical syringomyelia following resection of a large petrous meningioma: case report and review of literature. | journal=Skull Base | year= 2005 | volume= 15 | issue= 1 | pages= 89-97; discussion 98 | pmid=16148987 | doi=10.1055/s-2005-868168 | pmc=1151707 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16148987  }} </ref>


Diagnostic technology is another area for continued research. Already, MRI has enabled scientists to see conditions in the spine, including syringomyelia, even before symptoms appear. A new technology, known as dynamic MRI, allows investigators to view spinal fluid pulsating within the syrinx. CT scans allow physicians to see abnormalities in the brain, and other diagnostic tests have also improved greatly with the availability of new, non-toxic, contrast dyes. Patients can expect even better techniques to become available in the future from the research efforts of scientists today.
===Surgical untethering and expansive duraplasty ===
*Performed in post-traumatic syringomyelia.<ref name="pmid19119392">{{cite journal| author=Rene Hudson B, Cook C, Goode A| title=Identifying myelopathy caused by thoracic syringomyelia: a case report. | journal=J Man Manip Ther | year= 2008 | volume= 16 | issue= 2 | pages= 82-8 | pmid=19119392 | doi=10.1179/106698108790818512 | pmc=2565119 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19119392  }} </ref>
*This procedure involves establishing access to the spinal cord at the level of the injury, [[laminectomy]], lysis of adhesions, entry into [[spinal cord]] at the site of [[Syrinx (medicine)|syrinx]] and drainage of the syrinx into said space.<ref name="pmid19119392">{{cite journal| author=Rene Hudson B, Cook C, Goode A| title=Identifying myelopathy caused by thoracic syringomyelia: a case report. | journal=J Man Manip Ther | year= 2008 | volume= 16 | issue= 2 | pages= 82-8 | pmid=19119392 | doi=10.1179/106698108790818512 | pmc=2565119 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19119392  }} </ref>
*Duroplasty is usually performed following this procedure, for regional structural support.<ref name="pmid19119392">{{cite journal| author=Rene Hudson B, Cook C, Goode A| title=Identifying myelopathy caused by thoracic syringomyelia: a case report. | journal=J Man Manip Ther | year= 2008 | volume= 16 | issue= 2 | pages= 82-8 | pmid=19119392 | doi=10.1179/106698108790818512 | pmc=2565119 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19119392  }} </ref>


== See also ==
===Neuroendoscopic surgery===
*Novel endoscopic procedure used for chiari malformation and syrinx drainage<ref name="pmid12494359">{{cite journal| author=Buxton N, Jaspan T, Punt J| title=Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy. | journal=Minim Invasive Neurosurg | year= 2002 | volume= 45 | issue= 4 | pages= 231-4 | pmid=12494359 | doi=10.1055/s-2002-36195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12494359  }} </ref><ref name="pmid20819571">{{cite journal| author=Deng K, Li YN, Li GL, Gao J, Yang Z, Di X et al.| title=Neural endoscopic assisted micro-invasive management of Chiari I malformation. | journal=Chin Med J (Engl) | year= 2010 | volume= 123 | issue= 14 | pages= 1878-83 | pmid=20819571 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20819571  }} </ref><ref name="pmid12182791">{{cite journal| author=Métellus P, Dufour H, Levrier O, Grisoli F| title=Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations. | journal=Neurosurgery | year= 2002 | volume= 51 | issue= 2 | pages= 500-3; discussion 503-4 | pmid=12182791 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12182791  }} </ref>
*Minimally invasive
*Fast recovery
*Fewer complications


* [[Syringobulbia]]
==Prevention==
* [[Dissociated sensory loss]]
* [[Ependymoma]] tumors
* [[Scoliosis]] is rarely caused by syringomyelia.
* [[Otto Kahler]] was a neurologist from the last 1800's who published the first complete description of syringomyelia.
* Bobby Jones, a famous golfer, was diagnosed with tumor-related syringomyelia in 1948.


== References ==
===Primary Prevention===
{{reflist}}
There are no primary preventive measures associated with syringomyelia.


== External links ==
===Secondary Prevention===
*[http://www.mayoclinic.com/invoke.cfm?id=AN00464 Mayo Clinic's page]
*[[Physiotherapy]]
*[http://www.syringo.org/ Syringomyelia Facts]
*Prompt Surgery
*[http://www.neuro.wustl.edu/neuromuscular/spinal/syrinx.htm Syringomyelia syndromes, effects and associated disorders]
*[http://sm-acm.com/public_html/index.php SM/ACM Community]


=== Organizations ===
== References ==
*'''American Syringomyelia Alliance Project (ASAP)''' http://www.asap.org
{{reflist|2}}
*'''Christopher S. Burton Syringomyelia Foundation''' http://www.thesmfoundation.org
*'''National Organization for Rare Disorders (NORD)''' http://www.rarediseases.org
*'''National Spinal Cord Injury Association (NSCIA)''' http://www.spinalcord.org
*'''Brain Resources and Information Network (BRAIN)''' http://www.ninds.nih.gov
*'''The Ann Conroy Trust (ACT)''' http://www.theannconroytrust.org.uk
*'''Irish ANTS (Syringomyelia Self Help Group)''' http://homepage.eircom.net/~syringoselfhelp
<br>
 
{{CNS diseases of the nervous system}}
{{Congenital malformations and deformations of nervous system}}


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[[ru:Сирингомиелия]]


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Tarek Nafee, M.D. [2] Aysha Aslam, M.B.B.S[3] Eman Alademi, M.D.[4]

Overview

Syringomyelia is a spinal cord cavitation, which is a central dilation due to cystic degradation expands and destroyed the spinal cord. Caused by an injury, tumors or congenital malformation like hernia. The damage can Effect the brain and nerves, that leade to Bilateral loss of pain and temperature sensation in upper extremities. weakness, stiffness, hyperReflexives in lower extremities with hyposcoliosis. Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, on the location of the syrinx within the spinal cord.

Historical Perspective

  • In the 16th century, Estienne, a French anatomist, observed spinal cord cavitation for the first time.[1][2]
  • In 1545, Stephanus reported a case of spinal cord dilatation in one of his patients.[1][2]
  • In 1688, Brunner reported a liquor filled cavity with hydrocephalus in a pediatric case.[1][2]
  • In 1824, Olivier d'Angers coined the term syringomyelia.[1][2]
  • Also in 1824, Schuppel defined the fluid in the syrinx as a hydromyelia and proposed the pathogenesis as the persistence of the fetal central canal.[1][2]
  • In 1882, Otto von Kohler and Fredrich Schultz defined the clinical syndrome of syringomyelia.[1][2]
  • Between 1883 to 1891, Cleland and Hans Chiari associated syrinx formation with hyind brain herniation in what was subsequently named Chiari malformations types I to III. [1][2]
  • In 1959, Gardner described the waterhammer theory for pathogenesis of syringomyelia.[3]
  • In 1972, Ball and Dyan described the cavitation of the perivascular space as the source of syrinx formation.[4]
  • In 1980, Williams described the "suck and slosh" theory of syrinx formation.[5]
  • In 1994, Oldfield describes the piston effect of Chiari I malformation resulting in syringomyelia.[6]
  • In 2003, the Cisterna magna theory aimed to harmonize the Gardner, Williams, Oldfield and Ball&Dyan theories with neural imaging findings to propose an objective proposal of the pathogenesis of syringomyelia.[7]

Classification

Syringomyelia may be classified in various ways according to the anatomical features of the syrinx, or according to the underlying etiology of the disease.[8]

By Anatomy

Syringomyelia may be classified according to the anatomical description of the lesion (syrinx) as follows:[8]

  • Communicating vs. Non-communicating:
  • Intracannalicular vs. Extracannalicular:
    • Intracannalicular synringomyelia originates within the central canal of the spinal cord.
    • Extracannalicular syringomyelia originates within the spinal cord parenchyma.

By Etiology

Syringomyelia may be classified according to the underlying etiology or associated condition as follows:[9][10][11]

  • Congenital Malformations
  • Post-infectious
  • Post-traumatic
  • Post-inflammatory
  • Neoplastic

Pathophysiology

Pathogenesis

The exact pathogenesis of syringomyelia remains unknown. Several theories have been postulated about the development of a syrinx in the spinal cord. The underlying mechanisms of the disease involves disruption of CNS flow, subsequent formation of a syrinx, and enlargement of the lesion to impinge on the surrounding nerve fibers resulting in a symptomatic presentation.

1. Disruption of CSF Flow
Disruption of CSF flow may occur secondary to any of the following conditions:
  • Congenital craniocervical abnormalities obstructing CSF flow from the ventricles to the central canal (e.g., arnold chiari malformation, scoliosis)
  • Tumor or arachnoid cyst exhibiting a mass effect on the spinal cord
  • Sequelae of spinal cord trauma results in abnormal obstruction of the central canal
  • Sequelae of meningitis resulting in inflammation of the spinal cord meninges
  • Sequelae of inflammatory conditions such as sarcoidosis, multiple sclerosis, seronegative spondyloarthropathies
2. Syrinx formation
The pathogenesis of a syrinx is largely unknown. There are five primary theories of intra and extra cannilicular
  • Gardner's hydrodynamic theory: Blockage of the foramen of Magendie at the obex results in a pulsatile "water-hammer" effect of the mass lesion on the spinal cord CSF. This pressure differential results in extravasation of fluid into the perivascular and extracellular spaces.[7][12][3]
  • William's theory: Increased intracranial pressure combined with decreased subarachnoid spinal pressure results in a "vaccuum-like" effect which further herniates the cerebellar tonsils through the foramen magnum. This results in sloshing of the subarachnoid CSF and causes extravasation of the CSF into the spinal cord parenchyma, thus creating a syrinx.[7][5]
  • Ball & Dyan theory: With the CSF pressure difference created by the lesion, fluid moves, collects, and expands within Robin Virchow's perivascular space.[4]
  • Oldfield's theory: The pulsatile waves of the CSF which occur during systole result in increased ICP. This increased pressure herniates the cerebellum to obstruct the subarachnoid space at the level of the foramen magnum. This pulsatile fluid wave against the surface of the spinal cord results in extravasation of fluid into the parenchyma and creates a syrinx.[6]
  • Cisterna Magna theory: This theory claims that the fluid storage capacity of the cisterna magna is the primary shock absorber of the CNS that allows the spinal cord parenchyma to remain protected from increases in intracranial pressure. Mass effect of any lesion "stiffens" the cisterna magna and results in decreased compliance of the space and increased transferrance of pressure to the spinal cord. This results in extravasation of fluid to the spinal cord parenchyma.[7]
3. Development of symptoms
Once the syrinx enlarges enough to impinge on the surrounding spinal cord segments, symptoms related to the affected nerve fibers will develop due to the mass effect of the syrinx itself.

Genetics

There is no genetic predilection for the development of syringomyelia. However, Chiari malformations which are the primary cause of syringomyelia have demonstrated a strong familial and genomic association.[13][14][15][16][17][18]

Causes

The most common cause of syringomyelia is Chiari I malformation[19]. Other causes of syringomyelia may include:

Common Causes

Less Common Causes

Differentiating Syringomyelia from other diseases

Syringomyelia must be differentiated from other diseases which cause gait abnormalities, sensory and motor deficit, chronic pain syndromes, urinary and fecal incontinence, and cranial nerve palsies such as:[37][38][39][40][41]

Risk Factors

  The main risk factors associated with the development of syringomyelia are based on the underlying causes:

Screening

There is no recommended screening for syringomyelia.

Epidemiology and Demographics

Incidence

  • More than 25% of patients with spinal cord injury progress to develop syringomyelia.[47]

Prevalence

Syringomyelia has a prevalence estimated at 8.4 cases per 100,000 people,[48] or about 21,000 Americans, with symptoms usually beginning in young adulthood.

Natural History, Complications, and Prognosis

Natural History

The essential Course of disease in Syringomyelia is temperamental. Without treatment, syringomyelia may progress to develop the worsening of neurological deficit in cases of obstructive lesions. However, the disease has a slower progression in cases of mild disease.[49][50][51]

Complications

Complications that can develop as a result of syringomyelia are:[36][52][53][54][55][56][57][58][59][60][61]

Prognosis

The prognosis of syringomyelia varies depending on factors such as cause, extent of neurological damage, presence of complications, and general health status of patient before undergoing surgical treatment.[62][63][64]

  • Patients with mild neurological deficit may respond better to surgical management.
  • Early management of syringomyelia in cases of trauma is associated with decreased morbidity.
  • The symptoms of 80% of patients usually end due to their response to surgical treatment, as well a mild cure.
  • 20% of patients gets worse despite treatment in cases of advanced disease.
  • Recurrence is not uncommon in syringomyelia.

History and Symptoms

Specific areas of focus when obtaining the history such as onset, duration and progression of symptoms; history of trauma; associated conditions like multiple sclerosis, transverse myelitis, amyotrophic lateral sclerosis, sarcoidosis, congenital malformation, infection such as meningitis or neoplasia (ependymoma, meningioma, hemangioblastoma) may help diagnosing syringomyelia. Symptoms depends on the anatomical site of spinal cord involved.[65][66][67][68][69][70]

Anatomical site Early symptoms Late symptoms
Cervical
  • Inability to feel hot or cold
  • Decreased ability to feel pain sensation
  • Increased or decreased sweating
  • Muscle weakness ++
  • Shoulder joint pain, swelling and loss of function
  • Muscle paralysis
  • Loss of all sensations in hands, forearm, arm and upper back
  • Headaches with coughing or straining
Thoracic
  • Decreased to absent temperature sensation in trunk region
  • Abnormal pain sensation
  • Muscle weakness
  • Abnormal sweating
  • Muscle paralysis
  • Loss of pain and temperature sensation
Lumbar
  • Lower limb muscle weakness
  • Decreased to absent temperature sensation
  • Abnormal pain sensation
  • Joint sweling, pain and redness (charcot joints)
  • Loss of vibration, position sense and two point discrimination(recurrent falls)
  • Failure to maintain balance
  • Absent pain and temperature sensation
  • Muscle paralysis
  • Impaired ambulation
Sacral
  • Bladder or bladder dysfunction
  • Sexual dysfunction (erectile dysfunction)
  • Tingling and numbness in foot or anogenital region
  • Muscle weakness ++
  • Loss of penile erection
  • Loss of ankle plantar flexion(S1)
  • Urinary or fecal incontinence
  • Loss of temperature and pain sensation in foot and perineum

Physical Examination

The following includes how syringomyelia might be revealed :[71][72][73][74][75][76]

Anatomical site Physical examination
Syringobulbia
Cervical
  • Anhydrosis, miosis and ptosis (Horner's syndrome)
  • Loss of temperature and pain sensation in cervical dermatomes
  • Claw hand
  • Decreased muscle strength in hands, arms and shoulders
  • Decreased to absent deep tendon reflexes in upper limb
  • Muscle atrophy of hands, arms and shoulders
  • Tenderness, erythema, swelling and joint resorption (charcot joints)
Thoracic
  • Scoliosis
  • Decreased to absent pain and temperature sensation in thoracic dermatomes
  • Muscle atrophy ++
  • Absent superficial abdominal reflexes
Lumbar
Sacral

Imaging Findings

Imaging studies that may help diagnose syringomyelia, its cause and complications include:[77][78][79]

MRI

Spinal MRI may help to diagnose and follow up syringomyelia. It is characterized by the following findings:[77][80][81][82][83]

CT

Syringomyelia may be diagnosed as an incidental finding on CT scan. However, delayed CT scan may have a diagnostic importance in early cases of syringomyelia without clinical manifestaions.[84][85] Metrizimed CT scan using non-ionic contrast may be used to identify a syrinx in postinflammatory conditions (postinfectious and posthemorrhagic arachnoiditis).[86][87] In addition, computed axial tomography (CT) scans of a patient's head may reveal the presence of tumors and other abnormalities such as hydrocephalus.

X ray

There are no specific X ray findings associated with syringomyelia.

Myelogram

Myelogram is rarely used to diagnose syringomyelia. It uses a contrast material combined with x ray or CT to image spinal cord in case of syringomyelia. However, CT metrimised myelography is more sensitive to diagnose syringomyelia as compared to conventional myelography.[87][88]

Other Diagnostic Studies

Electromyography

EMG findings of syringomyelia include increased excitability of spinal motor neurons. However, the findings are non specific and does not hold diagnostic importance in case of syringomyelia.[89][90]

Lumbar Puncture

Role of lumbar puncture in syringomyelia is controversial since it may result in herniation in cases of Chiari malformation. However, it may be used by some clinicians as a diagnostic tool to look for CSF pressure and signs of inflammation based on individual patient assessment.[67]

Medical Therapy

Drugs have no curative value as a treatment for syringomyelia. The role of medical therapy in management of syringomyelia is for treatment of underlying conditions or management of associated symptoms.

Treatment of Underlying Conditions

Radiation and chemotherapy may be utilized for management of an underlying spinal cord tumor. Antibiotics may be used to treat an underlying infection (meningitis or encephalitis).

Management of Symptoms

In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.

Surgery

Surgery is not always required for syringomyelia. Indications for surgical management of syringomyelia include the following:[92][93]

  • Progressive neurological deterioration and worsening of symptoms
  • Presence of tumor causing obstruction
  • Congenital malformation(Chiari malformation)
  • Traumatic injury to spinal cord

Surgical options used to treat syringomyelia include:[94][95]

Posterior fossa decompression

  • Usually performed in Chiari malformation patients to expand the size of the posterior fossa and reverse tonsillar herniation resulting in relief of the obstructive lesion.[93][96]
  • This procedure may be combined with shunt, creating a large subarachanoid cistern to drain the syrinx or in conjunction with duroplasty in which dura is opened after bony removal of posterior occiput and posterior arch of C1 vertebra and a graft is sewed to dura effectively creating extra space in posterior fossa.[96][97]
  • Another procedure used along with posterior fossa decompression and duraplasty to help relieve obstruction is tonsillar resection or shrinkage.[98][99]

Shunt formation

Shunts are placed in order to drain the syrinx and alleviate symptoms such as headache and chronic pain syndromes. The choice of shunt is determined by the neurosurgeon; however, the following types of shunts may be performed:[97][100][101]

Ventriculoperitoneal shunt

  • Performed in patients with communicating syrinxes with hydrocephalus due to increased ventricular pressure.
  • This surgery is more commonly associated with Chiari II malformations

Syringosubarachnoid shunt

  • Performed more commonly in non-communicating syrinxes and is effective in rapidly reducing the size of the syrinx.
  • This shunt drains the syrinx into the subarachnoid space, and the CSF is subsequently reabsorbed by the arachnoid villi.

Syringoperitoneal shunt

  • Drainage of syrinx into the peritoneal cavity.

Removal of obstruction

  • In cases of tumors or arachnoid cysts as the underlying cause of the obstructive precursor lesion to syrinx formation, removal of said mass lesion may reverse the symptoms and reduce syrinx size.[102]

Surgical untethering and expansive duraplasty

  • Performed in post-traumatic syringomyelia.[67]
  • This procedure involves establishing access to the spinal cord at the level of the injury, laminectomy, lysis of adhesions, entry into spinal cord at the site of syrinx and drainage of the syrinx into said space.[67]
  • Duroplasty is usually performed following this procedure, for regional structural support.[67]

Neuroendoscopic surgery

  • Novel endoscopic procedure used for chiari malformation and syrinx drainage[103][104][105]
  • Minimally invasive
  • Fast recovery
  • Fewer complications

Prevention

Primary Prevention

There are no primary preventive measures associated with syringomyelia.

Secondary Prevention

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Klekamp J (2002). "The pathophysiology of syringomyelia - historical overview and current concept". Acta Neurochir (Wien). 144 (7): 649–64. doi:10.1007/s00701-002-0944-3. PMID 12181698.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Walusinski O (2012). "History of the Emergence and Recognition of Syringomyelia in the 19th Century". Vesalius. 18 (1): 18–29. PMID 26591071.
  3. ↑ 3.0 3.1 GARDNER WJ, ANGEL J (1958). "The cause of syringomyelia and its surgical treatment". Cleve Clin Q. 25 (1): 4–8. PMID 13500572.
  4. ↑ 4.0 4.1 Ball MJ, Dayan AD (1972). "Pathogenesis of syringomyelia". Lancet. 2 (7781): 799–801. PMID 4116236.
  5. ↑ 5.0 5.1 Williams B (1980). "On the pathogenesis of syringomyelia". Journal of Royal Society of Medicine. 73 (11): 798–806. Unknown parameter |pmcid= ignored (|pmc= suggested) (help)
  6. ↑ 6.0 6.1 Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994). "Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment". J Neurosurg. 80 (1): 3–15. doi:10.3171/jns.1994.80.1.0003. PMID 8271018.
  7. ↑ 7.0 7.1 7.2 7.3 Chang HS, Nakagawa H (2003). "Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics". J Neurol Neurosurg Psychiatry. 74 (3): 344–7. PMC 1738338. PMID 12588922.
  8. ↑ 8.0 8.1 Milhorat TH (2000). "Classification of syringomyelia". Neurosurg Focus. 8 (3): E1. doi:10.3171/foc.2000.8.3.1. PMID 16676921.
  9. ↑ Larner AJ, Muqit MM, Glickman S (2002). "Concurrent syrinx and inflammatory central nervous system disease detected by magnetic resonance imaging: an illustrative case and review of the literature". Medicine (Baltimore). 81 (1): 41–50. PMID 11807404.
  10. ↑ Laxton AW, Perrin RG (2006). "Cordectomy for the treatment of posttraumatic syringomyelia. Report of four cases and review of the literature". J Neurosurg Spine. 4 (2): 174–8. doi:10.3171/spi.2006.4.2.174. PMID 16506486.
  11. ↑ Batzdorf U (2000). "Primary spinal syringomyelia: a personal perspective". Neurosurg Focus. 8 (3): E7. PMID 16676930.
  12. ↑ Pillay PK, Awad IA, Hahn JF (1992). "Gardner's hydrodynamic theory of syringomyelia revisited". Cleve Clin J Med. 59 (4): 373–80. PMID 1525970.
  13. ↑ Boyles AL, Enterline DS, Hammock PH, Siegel DG, Slifer SH, Mehltretter L; et al. (2006). "Phenotypic definition of Chiari type I malformation coupled with high-density SNP genome screen shows significant evidence for linkage to regions on chromosomes 9 and 15". Am J Med Genet A. 140 (24): 2776–85. doi:10.1002/ajmg.a.31546. PMID 17103432.
  14. ↑ Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C; et al. (1999). "Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients". Neurosurgery. 44 (5): 1005–17. PMID 10232534.
  15. ↑ Speer MC, Enterline DS, Mehltretter L, Hammock P, Joseph J, Dickerson M; et al. (2003). "Review Article: Chiari Type I Malformation with or Without Syringomyelia: Prevalence and Genetics". J Genet Couns. 12 (4): 297–311. doi:10.1023/A:1023948921381. PMID 26141174.
  16. ↑ Markunas CA, Tubbs RS, Moftakhar R, Ashley-Koch AE, Gregory SG, Oakes WJ; et al. (2012). "Clinical, radiological, and genetic similarities between patients with Chiari Type I and Type 0 malformations". J Neurosurg Pediatr. 9 (4): 372–8. doi:10.3171/2011.12.PEDS11113. PMC 3678957. PMID 22462700.
  17. ↑ Stovner LJ, Bergan U, Nilsen G, Sjaastad O (1993). "Posterior cranial fossa dimensions in the Chiari I malformation: relation to pathogenesis and clinical presentation". Neuroradiology. 35 (2): 113–8. PMID 8433785.
  18. ↑ Cavender RK, Schmidt JH (1995). "Tonsillar ectopia and Chiari malformations: monozygotic triplets. Case report". J Neurosurg. 82 (3): 497–500. doi:10.3171/jns.1995.82.3.0497. PMID 7861232.
  19. ↑ 19.0 19.1 19.2 Carlson MD, Muraszko KM (2003). "Chiari I malformation with syrinx". Pediatr Neurol. 29 (2): 167–9. PMID 14580664.
  20. ↑ Williams B (1990). "Syringomyelia". Neurosurg Clin N Am. 1 (3): 653–85. PMID 2136163.
  21. ↑ 21.0 21.1 Ozerdemoglu RA, Denis F, Transfeldt EE (2003). "Scoliosis associated with syringomyelia: clinical and radiologic correlation". Spine (Phila Pa 1976). 28 (13): 1410–7. doi:10.1097/01.BRS.0000067117.07325.86. PMID 12838099.
  22. ↑ Shankar P, Zamora C, Castillo M (2016). "Congenital malformations of the brain and spine". Handb Clin Neurol. 136: 1121–37. doi:10.1016/B978-0-444-53486-6.00058-2. PMID 27430461.
  23. ↑ Agrawal A, Shetty MS, Pandit L, Shetty L, Srikrishna U (2007). "Post-traumatic syringomyelia". Indian J Orthop. 41 (4): 398–400. doi:10.4103/0019-5413.37006. PMC 2989527. PMID 21139799.
  24. ↑ Nardone R, Alessandrini F, Tezzon F (2003). "Syringomyelia following Listeria meningoencephalitis: report of a case". Neurol Sci. 24 (1): 40–3. doi:10.1007/s100720300021. PMID 12754657.
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  88. ↑ Li KC, Chui MC (1987). "Conventional and CT metrizamide myelography in Arnold-Chiari I malformation and syringomyelia". AJNR Am J Neuroradiol. 8 (1): 11–7. PMID 3101451.
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  98. ↑ Galarza M, Gazzeri R, Alfieri A, Martínez-Lage JF (2013). ""Triple R" tonsillar technique for the management of adult Chiari I malformation: surgical note". Acta Neurochir (Wien). 155 (7): 1195–201. doi:10.1007/s00701-013-1749-2. PMID 23695377.
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  105. ↑ Métellus P, Dufour H, Levrier O, Grisoli F (2002). "Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations". Neurosurgery. 51 (2): 500–3, discussion 503-4. PMID 12182791.

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