Syringomyelia: Difference between revisions
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'''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | ||
{{CMG}} {{AE}}{{TarekNafee}} {{ | {{CMG}} {{AE}}{{TarekNafee}} {{AAA}} {{EAM}} | ||
==Overview== | ==Overview== | ||
'''Syringomyelia''' is a | '''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. | ||
==Historical Perspective== | ==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> | |||
==Classification== | ==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.<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 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> | ||
===By Anatomy=== | ===By Anatomy=== | ||
Syringomyelia may be classified according to the anatomical description of the lesion (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> | 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''': | *'''Communicating''' vs. '''Non-communicating''': | ||
**A '''communicating syringomyelia''' is one where the syrinx is continuous from the spinal cord, across the [[foramen magnum]] and the dilatation is continuous with the fourth [[ventricle]] | **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. | **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''' vs. '''Extracannalicular''': | ||
**'''Intracannalicular synringomyelia''' originates within the central canal of the spinal cord. | **'''Intracannalicular synringomyelia''' originates within the [[central canal]] of the [[spinal cord]]. | ||
**'''Extracannalicular syringomyelia''' originates within the spinal cord parenchyma. | **'''Extracannalicular syringomyelia''' originates within the spinal cord parenchyma. | ||
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*Post-traumatic | *Post-traumatic | ||
*Post-inflammatory | *Post-inflammatory | ||
*Neoplastic | *[[Neoplastic]] | ||
==Pathophysiology== | ==Pathophysiology== | ||
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:'''1. Disruption of CSF Flow''' | :'''1. Disruption of CSF Flow''' | ||
:Disruption of CSF flow may occur secondary to any of the following conditions: | :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) | :*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 | :*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 spinal cord [[trauma]] results in abnormal obstruction of the central canal | ||
:*Sequelae of meningitis resulting in inflammation of the spinal cord meninges | :*Sequelae of [[meningitis]] resulting in inflammation of the spinal cord meninges | ||
:*Sequelae of inflammatory conditions such as sarcoidosis, multiple sclerosis, seronegative spondyloarthropathies | :*Sequelae of inflammatory conditions such as [[sarcoidosis]], [[multiple sclerosis]], [[seronegative spondyloarthropathies]] | ||
:'''2. Syrinx formation''' | :'''2. Syrinx formation''' | ||
:The pathogenesis of a syrinx is largely unknown. There are five primary theories of intra and extra cannilicular | :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> | :*'''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> | :* '''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 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> | :* '''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> | :* '''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> | :* '''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> | ||
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===Genetics=== | ===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.<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> | |||
=== | |||
=== | |||
==Causes== | ==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: | 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=== | ===Common Causes=== | ||
*Other congenital abnormalities (e.g., scoliosis, 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> | *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> | *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> | ||
===Less Common Causes=== | ===Less Common Causes=== | ||
*Infections (e.g., [[meningitis]]/ | *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> | *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, | *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> | *[[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> | ||
==Differentiating Syringomyelia from other diseases== | ==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]] | |||
==Risk Factors== | ==Risk Factors== | ||
'''The main risk factors associated with the development of syringomyelia are based on the underlying causes:''' | |||
*Congenital Malformations | *'''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> | |||
* | |||
* | |||
==Screening== | ==Screening== | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
===Incidence=== | ===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> | |||
===Prevalence=== | |||
[[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. | [[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. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
The | '''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> | ||
===Complications=== | ===Complications=== | ||
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*Patients with mild neurological deficit may respond better to surgical management. | *Patients with mild neurological deficit may respond better to surgical management. | ||
*Early management of syringomyelia in cases of trauma is associated with decreased morbidity. | *Early management of syringomyelia in cases of trauma is associated with decreased morbidity. | ||
*80% of patients | *'''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. | *20% of patients gets worse despite treatment in cases of advanced disease. | ||
*Recurrence is not uncommon in syringomyelia. | *Recurrence is not uncommon in syringomyelia. | ||
==History and Symptoms== | ==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, | 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="border: 0px; font-size: 90%; margin: 3px;" align=center | ||
|+ | |+ | ||
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*Muscle weakness ++ | *Muscle weakness ++ | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Shoulder joint pain, swelling and loss of function | *Shoulder [[Arthralgia|joint pain]], swelling and loss of function | ||
*Muscle paralysis | *Muscle paralysis | ||
*Loss of all sensations in hands, forearm, arm and upper back | *Loss of all sensations in hands, forearm, arm and upper back | ||
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==Physical Examination== | ==Physical Examination== | ||
'''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="border: 0px; font-size: 90%; margin: 3px;" align=center | ||
|+ | |+ | ||
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| style="padding: 5px 5px; background: #DCDCDC;" | '''Syringobulbia''' | | style="padding: 5px 5px; background: #DCDCDC;" | '''Syringobulbia''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Facial sensory loss (sensory nucleus of 5th nerve) | *Facial sensory loss (sensory nucleus of [[Trigeminal nerve|5th nerve]]) | ||
*Absent gag reflex (9th and 10th nerve) | *Absent gag reflex ([[Glossopharyngeal nerve|9th]] and [[Vagus nerve|10th nerve]]) | ||
*Hoarseness of voice (vocal cord paralysis) | *[[Hoarseness]] of voice ([[Vocal cords|vocal cord]] paralysis) | ||
*Decreased muscle | *Decreased muscle strength of [[Sternocleidomastoid muscle|sternocleidomastoid]] and [[trapezius muscle]] ([[Accessory nerve|11th nerve)]] | ||
*Atrophy and weakness of tongue muscle (12th nerve) | *Atrophy and weakness of tongue muscle ([[Hypoglossal nerve|12th nerve]]) | ||
*Apnea (respiratory failure) | *[[Apnea]] (respiratory failure) | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | '''Cervical''' | | style="padding: 5px 5px; background: #DCDCDC;" | '''Cervical''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Anhydrosis, miosis and ptosis (Horner's syndrome) | *Anhydrosis, [[miosis]] and [[ptosis]] ([[Horner's syndrome]]) | ||
*Loss of temperature and pain sensation in cervical dermatomes | *Loss of temperature and pain sensation in cervical dermatomes | ||
*Claw hand | *[[Claw hand]] | ||
*Decreased muscle strength in hands, arms and shoulders | *Decreased muscle strength in hands, arms and shoulders | ||
*Decreased to absent deep tendon reflexes in upper limb | *Decreased to absent deep tendon reflexes in upper limb | ||
*Muscle atrophy of hands, arms and shoulders | *Muscle atrophy of hands, arms and shoulders | ||
*Tenderness, erythema, swelling and joint resorption (charcot joints) | *Tenderness, [[erythema]], swelling and joint resorption (charcot joints) | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" |'''Thoracic''' | | style="padding: 5px 5px; background: #DCDCDC;" |'''Thoracic''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Scoliosis | *[[Scoliosis]] | ||
*Decreased to absent pain and temperature sensation in thoracic dermatomes | *Decreased to absent pain and temperature sensation in thoracic dermatomes | ||
*Muscle atrophy ++ | *[[Muscle atrophy]] ++ | ||
*Absent | *Absent superficial abdominal reflexes | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | '''Lumbar''' | | style="padding: 5px 5px; background: #DCDCDC;" | '''Lumbar''' | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Lower limb spasticity | *Lower limb spasticity | ||
* | *[[Hyperreflexia]] or [[Areflexia]] | ||
*Babinski's sign ++ | *[[Babinski's sign]] ++ | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;" | '''Sacral''' | | style="padding: 5px 5px; background: #DCDCDC;" | '''Sacral''' | ||
Line 222: | Line 244: | ||
*Decreased to absent pain and temperature sensation in sacral dermatomes | *Decreased to absent pain and temperature sensation in sacral dermatomes | ||
*Loss of rectal tone | *Loss of rectal tone | ||
*Absent cremasteric reflex | *Absent [[cremasteric reflex]] | ||
*Scoliosis | *[[Scoliosis]] | ||
|- | |- | ||
|} | |} | ||
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===MRI=== | ===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> | 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 in the spinal cord, its extent, location and complications | *Presence of [[Syrinx (medicine)|syrinx]] in the [[spinal cord]], its extent, location and complications | ||
*Enlargement of central canal of spinal cord | *Enlargement of [[central canal]] of [[spinal cord]] | ||
*Presence of syrinx in the brain stem (syringobulbia) | *Presence of [[Syrinx (medicine)|syrinx]] in the brain stem (syringobulbia) | ||
*Presence of assocaited congenital anomalies such as [[chiari malformation]], | *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 | *Presence of [[tumors]] such as [[ependymoma]] | ||
*May be used for follow up post surgery | *May be used for follow up post surgery | ||
===CT=== | ===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 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. | 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. | ||
===X ray=== | ===X ray=== | ||
There are no specific X ray findings associated with syringomyelia. | There are no specific [[X ray]] findings associated with syringomyelia. | ||
===Myelogram=== | ===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> | ||
==Other Diagnostic Studies== | ==Other Diagnostic Studies== | ||
===Electromyography=== | ===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> | [[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=== | ===Lumbar Puncture=== | ||
Role of lumbar puncture in syringomyelia is controversial since it may result in herniation in cases of | 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> | ||
==Medical Therapy== | ==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. | 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 | ===Treatment of Underlying Conditions=== | ||
Radiation and chemotherapy may be utilized for management of an underlying spinal cord tumor. | 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) | [[Antibiotics]] may be used to treat an underlying infection ([[meningitis]] or [[encephalitis]]). | ||
===Management of Symptoms=== | ===Management of Symptoms=== | ||
Analgesics may be used to control pain. | *[[Analgesics]] may be used to control pain. | ||
Drugs may be used for urinary or fecal incontinence or neurogenic bladder. | *[[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. | ||
==Surgery== | ==Surgery== | ||
Surgery is not always required for syringomyelia. Indications for surgical management of syringomyelia include the following: | 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 | *Progressive neurological deterioration and worsening of symptoms | ||
*Presence of | *Presence of [[tumor]] causing obstruction | ||
*Congenital malformation( | *Congenital malformation([[Chiari malformation]]) | ||
*Traumatic injury to spinal cord | *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> | 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=== | ===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> | *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 | *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> | ||
===Shunt formation=== | ===Shunt formation=== | ||
Line 290: | Line 313: | ||
===Removal of obstruction=== | ===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. | *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> | ||
===Surgical untethering and expansive duraplasty === | ===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> | *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 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> | *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> | *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> | ||
===Neuroendoscopic surgery=== | ===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 | |||
==Prevention== | ==Prevention== | ||
Line 322: | Line 332: | ||
===Secondary Prevention=== | ===Secondary Prevention=== | ||
*Physiotherapy | *[[Physiotherapy]] | ||
*Prompt Surgery | *Prompt Surgery | ||
== References == | == References == | ||
{{reflist|2}} | {{reflist|2}} | ||
{{WH}}{{WS}} | {{WH}}{{WS}} |
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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:
- A communicating syringomyelia is one where the 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.
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
- Other congenital abnormalities (e.g., scoliosis, Chiari I and II malformations)[20][19][21][22]
- Traumatic spinal cord injury[23]
Less Common Causes
- Infections (e.g., meningitis/arachnoiditis)[24][25]
- Mass lesions or neoplasia (e.g., Ependymoma, hemangioblastoma, meningioma, or arachnoid cyst)[26][27][28][29]
- Inflammatory conditions (e.g. multiple sclerosis, transverse myelitis, amyotrophic lateral sclerosis, sarcoidosis, other myelitis)[30][31][32][33][34]
- Idiopathic [35][36]
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]
- Normopressure hydrocephalus
- ALS
- Meningitis
- Transverse Myelitis
- Cerebrovascular accidents
- Brain tumor
- Peripheral neuropathy
- Fibromyalgia
- Parkinsonism
- Cerebellar ataxia
- Multiple sclerosis
- Neurogenic bladder
- Spinal cord space occupying lesion (e.g., tumors, cysts, abscesses)
- Guillian Barre
- Chronic demyelinating polyneuropathy
Risk Factors
The main risk factors associated with the development of syringomyelia are based on the underlying causes:
- Congenital Malformations: Chiari malformations, Klippel Feil syndrome, congenital scoliosis[19][21][42]
- Post-infectious: Development of arachnoiditis and development of dural abscess.[43]
- Post-traumatic: Increasing age at time of trauma , cervical and thoracic injuries compared with lumbar, displaced fractures, and spinal instrumentation without decompression.[44][45]
- Post-inflammatory: Multiple sclerosis, neurosarcoidosis, Amyotrophic Lateral Sclerosis (ALS)[30]
- Neoplastic: Ependymoma and hemangioblastoma are most strongly associated with development of syringomyelia.[46]
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]
- 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
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 |
|
|
Thoracic |
|
|
Lumbar |
|
|
Sacral |
|
|
Physical Examination
The following includes how syringomyelia might be revealed :[71][72][73][74][75][76]
Anatomical site | Physical examination |
---|---|
Syringobulbia |
|
Cervical |
|
Thoracic |
|
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]
- Presence of syrinx in the spinal cord, its extent, location and complications
- Enlargement of central canal of spinal cord
- Presence of syrinx in the brain stem (syringobulbia)
- Presence of assocaited congenital anomalies such as chiari malformation, klippel feil syndrome, spina bifida, tethered cord syndrome
- Presence of tumors such as ependymoma
- May be used for follow up post surgery
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
- Analgesics may be used to control pain.
- GABA inhibitors neurontin 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)[91]
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
- Physiotherapy
- Prompt Surgery
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 3.0 3.1 GARDNER WJ, ANGEL J (1958). "The cause of syringomyelia and its surgical treatment". Cleve Clin Q. 25 (1): 4–8. PMID 13500572.
- ↑ 4.0 4.1 Ball MJ, Dayan AD (1972). "Pathogenesis of syringomyelia". Lancet. 2 (7781): 799–801. PMID 4116236.
- ↑ 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) - ↑ 6.0 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.
- ↑ 7.0 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.
- ↑ 8.0 8.1 Milhorat TH (2000). "Classification of syringomyelia". Neurosurg Focus. 8 (3): E1. doi:10.3171/foc.2000.8.3.1. PMID 16676921.
- ↑ 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.
- ↑ 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.
- ↑ Batzdorf U (2000). "Primary spinal syringomyelia: a personal perspective". Neurosurg Focus. 8 (3): E7. PMID 16676930.
- ↑ Pillay PK, Awad IA, Hahn JF (1992). "Gardner's hydrodynamic theory of syringomyelia revisited". Cleve Clin J Med. 59 (4): 373–80. PMID 1525970.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 19.0 19.1 19.2 Carlson MD, Muraszko KM (2003). "Chiari I malformation with syrinx". Pediatr Neurol. 29 (2): 167–9. PMID 14580664.
- ↑ Williams B (1990). "Syringomyelia". Neurosurg Clin N Am. 1 (3): 653–85. PMID 2136163.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Caplan LR, Norohna AB, Amico LL (1990). "Syringomyelia and arachnoiditis". J Neurol Neurosurg Psychiatry. 53 (2): 106–13. PMC 487945. PMID 2313296.
- ↑ Joshi VP, Valsangkar A, Nivargi S, Vora N, Dekhne A, Agrawal A (2013). "Giant posterior fossa arachnoid cyst causing tonsillar herniation and cervical syringomyelia". J Craniovertebr Junction Spine. 4 (1): 43–5. doi:10.4103/0974-8237.121627. PMC 3872663. PMID 24381458.
- ↑ Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES (2007). "Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment". J Korean Neurosurg Soc. 42 (6): 436–40. doi:10.3340/jkns.2007.42.6.436. PMC 2588179. PMID 19096585.
- ↑ Gluf WM, Dailey AT (2014). "Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: case report and review of the literature". J Spinal Cord Med. 37 (6): 791–4. doi:10.1179/2045772314Y.0000000210. PMC 4231969. PMID 25029412.
- ↑ Mock A, Levi AD, Levi A, Drake JM (1990). "Spinal hemangioblastoma, syrinx, and hydrocephalus in a two-year-old child". Neurosurgery. 27 (5): 799–802. PMID 2259411.
- ↑ 30.0 30.1 Ravaglia S, Bogdanov EI, Pichiecchio A, Bergamaschi R, Moglia A, Mikhaylov IM (2007). "Pathogenetic role of myelitis for syringomyelia". Clin Neurol Neurosurg. 109 (6): 541–6. doi:10.1016/j.clineuro.2007.03.007. PMID 17467892.
- ↑ Markh A, Wainapel S (2016). "Transverse Myelitis and Syrinx Formation Caused by Varicella-Zoster Infection". Am J Phys Med Rehabil. 95 (1): e12–3. doi:10.1097/PHM.0000000000000391. PMID 26495810.
- ↑ Sotgiu S, Pugliatti M, Rosati G, Sechi GP (2001). "Which syringomyelia is truly associated with multiple sclerosis?". J Neurol Sci. 190 (1–2): 99–100. PMID 11603356.
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