Original Article
How Should Syringomyelia be Defined and Diagnosed?
Jörg Klekamp
- OBJECTIVE: Syringomyelia is considered as a fluid-filled
cavitation inside the spinal cord. However, there is no
agreement whether a dilated central canal should be
included under this heading or how glioependymal cysts,
myelomalacias, or cystic tumors should be distinguished
from syringomyelia. This article provides a definition of
syringomyelia and guidelines for its diagnosis.
- METHODS: Between 1991 and 2015, of 3206 patients with
spinal cord pathologies 2276 demonstrated cystic features.
All patients underwent magnetic resonance imaging.
Syringomyelia was differentiated from cystic intra-
medullary tumors, glioependymal cysts, myelomalacias,
and dilatations of the central canal by clinical and radio-
logic criteria.
- RESULTS: A total of 1535 patients were diagnosed with
syringomyelia, 635 with dilatations of the central canal, 52
with glioependymal cysts, 52 with mylomalacias, and 2
with cystic intramedullary spinal cord tumors. Additional
neuroradiologic studies revealed the causes of syringo-
myelia. As a result 604 patients showed pathologies at the
craniocervical junction leading to disturbances of cere-
brospinal fluid (CSF) flow. The commonest was a Chiari I
malformation in 543 patients. Nine hundred thirty-one pa-
tients presented with pathologies in the spinal canal. The
commonest causes were spinal arachnopathies, leading to
CSF flow obstructions in 533 patients, intramedullary
tumors in 152 patients, and tethered cord syndromes in 69
patients.
- CONCLUSIONS: The diagnosis of syringomyelia should
be reserved for patients with a fluid-filled cavity in the
Key words
- Central canal
- Cerebrospinal fluid flow
- Glioependymal cyst
- Intramedullary spinal cord tumors
- Myelomalacia
- Syringomyelia
- Tethered cord syndrome
Abbreviations and Acronyms
CSF: Cerebrospinal fluid
MRI: Magnetic resonance imaging
WORLD NEUROSURGERY 111: e729-e745, MARCH 2018
spinal cord related to either a disturbance of CSF flow,
spinal cord tethering, or an intramedullary tumor. For
patients in whom such a relation cannot be established,
the diagnosis of syringomyelia should be withheld.
INTRODUCTION
O llivier D’Angers1 introduced the term syringomyelia at
the beginning of the 19th century for the central canal
of the spinal cord, which he considered an abnormal
finding. Later the central canal was recognized as a normal
anatomic structure and the term hydromyelia was introduced for
its dilatation.2 Magnetic resonance imaging (MRI) shows any
cystic change inside the spinal cord and may even visualize a
normal central canal. But, how should we deal with a central
canal visible on high resolution scans? Does it represent an early
stage of syringomyelia or a harmless variant without clinical
significance? Should any cystic change inside the spinal cord be
considered as a syrinx or which entities should be distinguished
from syringomyelia? At present, the International Statistical
Classification of Diseases and Related Health Problems (ICD ed
10) distinguishes syringomyelia (G95.0) and hydromyelia (Q06.4)
as separate entities for fluid-filled cavities in the spinal cord.
However, there appears to be no general agreement on how these
entities should be differentiated. Other investigators have sug-
gested to completely abandon the term hydromyelia.3
Criteria are required to distinguish entities, such as myeloma-
lacia, glioependymal cysts, cystic tumors, or a dilated central ca-
nal, from syringomyelia. Based on neuroradiologic and clinical
examinations, the present article provides a definition of syrin-
gomyelia and how it should be diagnosed.
Christliches Krankenhaus Quakenbrück, Department of Neurosurgery, Quakenbrück, Germany
To whom correspondence should be addressed: Jörg Klekamp, M.D.
[E-mail: j.klekamp@ckq-gmbh.de]
Citation: World Neurosurg. (2018) 111:e729-e745.
https://doi.org/10.1016/j.wneu.2017.12.156
Journal homepage: www.WORLDNEUROSURGERY.org
Available online: www.sciencedirect.com
1878-8750/$ - see front matter ª 2018 Elsevier Inc. All rights reserved.
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JÖRG KLEKAMP
METHODS
Between 1991 and 2015, 3206 patients presented with spinal cord
pathologies (i.e., intradural and extradural spinal tumors, spinal
cord malformations, or spinal arachnopathies). These patients
were consecutively entered into a spinal cord database. General
Figure 1. Neuroradiologic flow chart for patients with
cystic changes of the spinal cord. Contrast
enhancement, space-occupying effects, changes of
shape and volume over time, atrophy of the spinal cord,
and analyses of cerebrospinal fluid flow and fluid
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patient data, neurological examinations, and specific features of
each pathology were evaluated. A total of 2276 MRI scans showed
cystic features of the spinal cord, therefore syringomyelia was
considered by the referring physicians, and they formed the basis
of this study. All MRI underwent a stratification protocol, as
movements inside the cyst can be used to differentiate
syringomyelia from cystic intramedullary tumors,
glioependymal cysts, myelomalacia, or dilatations of
the central canal.

JÖRG KLEKAMP
outlined in Figure 1. Cystic changes were classified either as
intramedullary tumors, glioependymal cysts, myelomalacias,
dilatations of the central canal, or syringomyelia.
All patients agreed in writing upon admission that their medical
data may be used for scientific purposes in anonymous form. For
statistical tests of significance, Student’s t-tests and c2 tests were
used. Means are given with the standard deviation. Differences
were considered statistically significant, if a P value
0.05 was
reached. For all statistical analyses the software package
PC-Statistik version 5.0 was used (Hoffmann-Software, Gießen,
Germany).
RESULTS
Of 3206 patients with spinal cord pathologies, 2276 featured cystic
changes on MRI. A total of 1535 patients were diagnosed as having
syringomyelia, 635 with dilatations of the central canal, 52 with
glioependymal cysts, 52 with myelomalacias, and 2 with cystic
intramedullary spinal cord tumors.
Cystic Intramedullary Tumors and Solid Intramedullary Tumors
with Syringomyelia
Two cystic intramedullary tumors of the cervical cord were iden-
tified. Both patients had some contrast in their wall after appli-
cation of gadolinium (Figure 2). No other cystic spinal cord
pathology displayed such contrast enhancement.
Figure 2. (A) T2-weighted sagittal magnetic resonance imaging scan of a
cystic lesion at C2 to C3 associated with another cystic change at C4 to C6
and some cord edema at C1. (B) The T1-weighted image demonstrates
both cystic lesions, whereas the diagnosis of a cystic intramedullary tumor
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Among the 326 patients with intramedullary tumors, 152
(46.6%) showed an associated syrinx (Figure 3). In this series,
every tumor associated with syringomyelia showed some uptake
of contrast on MRI. Comparing intramedullary tumors with or
without syringomyelia (Table 1) disclosed no differences in
clinical presentation with an even sex distribution in both
groups and no age differences. Histologies, however, were
found to differ significantly (c2 test: P< 0.0001). Ependymomas,
angioblastomas, and astrocytomas were the commonest to show
an association with syringomyelia (Table 2). In 33.9% of patients
the syrinx expanded only in the cranial direction, with the
tumor located at the lower pole, whereas the tumor was located
in the upper pole of the syrinx in 5.5%. In 60.6% of patients,
the syrinx expanded in both directions in relation to the tumor
(Figure 3).
Gliopendymal Cysts
Glioependymal cysts were morphologically characterized by a
profound space-occupying effect, a symmetrical shape in the
sagittal plane with rounded cyst poles, no contrast uptake in their
walls, no septations, no evidence of fluid movements inside, and
no cerebrospinal fluid (CSF) flow abnormalities in the spinal
region involved (Figures 4 and 5). Almost half of them (n ¼ 25)
were encountered in the conus region extending over a
maximum of 3 spinal segments (Figures 4 and 5). The
remainder were found in the cervical region (n ¼ 24), with a
C2 to C3 with a small syrinx below becomes clear after contrast application
(C). The patient underwent surgery and the diagnosis of a pilocytic
astroctoma World Health Organization grade I was established.
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Figure 3. (A) T2-weighted sagittal magnetic resonance the T1-weighted image discloses an intramedullary
imaging scan of a large syrinx from C2 to Th2 with flow tumor at C4-5. The histologic diagnosis was a World
voids inside the syrinx. (B) After contrast application Health Organization grade II ependymoma.

minority in upper thoracic levels (n ¼ 3). The largest were years, with an average history of 34.5  50.3 months. Women
observed in the cervical area, with 3 cysts extending over 5e6 predominated men by 2:1.
segments. Only 5 cysts (9.6%) in this series demonstrated an
increase until presentation or during follow-up (Figure 4). Myelomalacia
Back pain affected about two thirds of these patients, with 65% Myelomalacia was the result of a spinal cord disease or trauma
of glioependymal cysts being asymptomatic, representing inci- leading to destruction of cord tissue and a tissue defect, which
dental findings. In 10 patients (19.2%) local pain involved the displayed cystic features without a space-occupying effect and
spinal region of the cyst. Apart from sphincter problems (11.3%), surrounded by cord atrophy (Figure 6). On MRI, no intracystic
additional neurological symptoms were reported by about a third fluid movements were detectable.
of the patients (Table 3) corresponding to the cyst’s spinal
representation. Such patients underwent surgery by cyst
fenestration. Patients presented at a mean age of 47.0  14.4
Table 2. Histologies of Patients with Intramedullary Tumors
Intramedullary Intramedullary
Histology Tumor with Syrinx Tumor without Syrinx
Table 1. Symptoms of Patients with Intramedullary Tumors
Astrocytomas 18.3% 39.7%
Intramedullary Intramedullary
Tumor with Tumor without Ependymomas 48.9% 28.7%
Symptom Syrinx (n [ 152) Syrinx (n [ 174)
Angioblastomas 24.4% 1.5%
Pain 54.4% 51.4% Cavernomas 2.3% 11.0%
Sensory disturbances 81.6% 83.1% Gangliogliomas — 5.2%
Dysesthesias 54.4% 55.4% Melanocytomas 1.5% 2.2%
Motor weakness 68.0% 71.5% Dermoid cysts 3.1% 7.4%
Gait ataxia 77.6% 74.6% Neurinomas — 1.5%
Sphincter disturbances 33.6% 36.2% Metastases 1.5% 2.9%

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JÖRG KLEKAMP
Figure 4. (A) T2-weighted sagittal magnetic resonance imaging (MRI) scan
of a glioependymal cyst at Th12 with a significant space-occupying effect.
(B) The sagittal T2-weighted image 6 years later shows an increase of the
cyst. (C) The axial scan demonstrates the spinal cord displaced anteriorly
Men predominated women by 2:1. Patients presented at a mean
age of 49.7  13.9 years with a history of 65.7  84.6 months. In
most patients (78.8%) a severe spinal cord injury preceded mye-
lomalacia, which extended
3 segments and without progression
over time (Figure 6). Neurological symptoms were the result of the
initial spinal cord injury (Table 3). If symptoms progressed, they
were related to post-traumatic arachnopathies, cord tethering, or
spinal instability (Figure 6).
In the remaining 11 patients, an unknown disease process had
resulted in profound atrophy of the entire spinal cord leading to
myelomalacia in the central area of the cord for 6 segments,
affecting the entire spinal cord in 5 patients (Figure 7; the
complete thoracic cord in 2 and the entire cervical cord in 4
patients). Pain was not a typical feature for this subgroup, with
only 4 patients reporting unspecific back pain. All but 2 of these
patients presented with progressive and severe motor weakness
and gait problems (Table 3).
Central Canal Dilatations
Central canal dilatations were asymptomatic at presentation
and remained so during follow-up. They typically displayed a
spindle shape, no space-occupying effect in an otherwise
normal-appearing spinal cord, no fluid movements inside, and
no CSF flow obstructions in the involved spinal segments
(Figures 8 and 9). If neurological symptoms were present, they
did not correspond to the spinal representation of the central
canal dilatation and were explained by other neurological or
spinal diseases, with degenerative spinal diseases as the
commonest.
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and to the left side. There was no uptake of gadolinium in the cyst wall on
corresponding T1-weighted images. The patient’s symptoms remained
restricted to slight dysesthesias in her right foot. She is followed with MRI
and clinical examinations.
Central canal dilatations were mainly detected in 2 areas: the
lower cervical and the mid-thoracic levels. In 23.1% of patients,
the cervical region (Figure 9) was affected and in 69.5%, the
thoracic levels (Figure 8) were affected exclusively, with the
remainder showing dilatations in both areas. Patients presented
at a mean age of 40.8  14.8 years, with an average history of
44.2  70.8 months. Women were more commonly affected
than men by a factor of 2:1. The predominating symptom
leading to MRI imaging was back pain in 79.5%. In 120 patients
(18.9%) chronic pain syndromes were the major clinical problem
and for 40.9% pain was related to degenerative diseases of the
cervical spine (Table 3).
Syringomyelia
Radiologic signs suggesting the diagnosis of syringomyelia were a
volume increase of a fluid-filled cavitation with time, expanding
from the region of its largest diameter in the upper and/or lower
direction with evidence of fluid movements inside the cavitation
(Figure 10). To confirm the diagnosis of syringomyelia, further
diagnostic efforts were undertaken to determine the underlying
pathology located either at the craniocervical junction or in the
spinal canal.
In this series, the commonest cause for syringomyelia was a
CSF flow obstruction due to a Chiari I malformation in 543 pa-
tients (35.4%), with 73.2% of Chiari I patients presenting with
syringomyelia (aged, 43.9  16.3 years; history, 68.6  103.7
months). Sex distribution favored women by 2:1. Comparing
patients with and without syringomyelia revealed significant dif-
ferences for sensory changes, dysesthesias, and motor weakness,
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Figure 5. (A) T2-weighted sagittal magnetic resonance
imaging (MRI) scan of a glioependymal cyst at
Th10-Th12. Its shape is symmetrical in contrast to a
syrinx as shown in Figure 3. There was no uptake of
gadolinium in the cyst wall on T1-weighted images. (B)
The cine-MRI in diastole demonstrates no
cerebrospinal fluid flow obstruction and no flow signals
inside the cyst. The patient had complained about back
pain for the past 18 months. No surgery was
recommended. The patient has remained
neurologically intact for the past 8 years.

which were more common in patients with syringomyelia arachnoiditis (32 patients), a Chiari II malformation (27 pa-
(c2 tests: P< 0.0001) (Table 4). tients), or a Chiari III malformation (2 patients). Whereas
Other pathologies at the craniocervical junction causing CSF foramen magnum arachnoiditis was always associated with
flow obstructions and syringomyelia were foramen magnum syringomyelia, only 60% of Chiari II malformations presented
with a syrinx.
In 931 patients without pathologies at the craniocervical
Table 3. Symptoms of Patients with Glioependymal Cysts, junction, MRI scans of the spine were analyzed first for evidence
Myelomalacias, and Central Canal Dilatations of an intramedullary tumor, which affected 152 patients (i.e.,
Central Canal about 1 of 6 patients with a cystic spinal cord lesion without a
Glioependymal Myelomalacias Dilatations pathology at the craniocervical junction). Much rarer, syringo-
Symptom Cysts (n [ 52) (n [ 52) (n [ 635) myelia was related to extramedullary tumors (92 patients: 14.2%
of 649 extramedullary tumors), extradural tumors (15 patients:
Back pain 64.2% 60.9% 79.5% 3.0% of 492 extradural tumors), or extradural compression
Sensory disturbances 35.9% 78.3% 25.6% related to degenerative disc diseases in 70 patients. In all of
Dysesthesias 32.1% 32.6% 28.4%
these, the syrinx was asymptomatic with symptoms related
exclusively to the tumor or the degenerative disease,
Motor weakness 30.2% 65.2% 10.0% respectively.
Gait ataxia 35.9% 87.0% 15.4% In 163 patients syringomyelia was related to spinal trauma
Sphincter disturbances 11.3% 69.6% 5.8% (Figure 11), with the syrinx starting at the trauma level and
expanding from there in cranial (55.1%), in caudal (12.6%), or

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JÖRG KLEKAMP
Figure 6. (A) T2-weighted sagittal magnetic resonance
imaging (MRI) scan of a myelomalacia at C5 years after
a spinal cord injury and fracture at C5, which was
managed by a ventral fusion C5-6. The patient
presented with aggravation of his post-traumatic
Figure 7. (A, B) T2-weighted sagittal magnetic resonance imaging scans
demonstrating atrophy of the entire spinal cord with a prominent central
canal from C1 down to the conus. (C) An axial scan in the thoracic area
shows the characteristic triangular shape of the spinal cord due to tension
on both dentate ligaments caused by the profound loss of cord tissue. The
71-year-old patient had suffered from a meningoencephalitis of unknown
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myelopathy due to osteochondrosis at C4-5 and
instability at C3-4. He underwent a ventral fusion
C3-C6. (B) As expected, the postoperative MRI scan
showed no change of the myelomalacia, whereas
motor power in his left hand and his gait improved.
etiology 45 years earlier and had developed a profound scoliosis since
childhood. She presented with a distal motor weakness of her right arm,
which had gradually progressed during the past 20 years followed by gait
ataxia predominantly affecting the right leg. Conservative management
was recommended.
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JÖRG KLEKAMP
Figure 8. (A) T2-weighted sagittal magnetic resonance
imaging (MRI) scan of central canal dilatations at
Th5-Th7 and Th11-Th12 without a space-occupying
effect and no evidence for a tethered cord in a
5-year-old boy with a gait disturbance related to an
orthopedic foot deformity on the left side. The
neurological examination was normal. (B) The axial
in both directions (32.3%). Men predominated women by 3:1 in
this subgroup. Mean age at presentation was 48.2  13.0 years,
with a history of 50.9  74.4 months.
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scan demonstrates the central position, whereas the
cine-MRI scan (C) discloses no flow signals inside both
dilatations and no obstructions of cerebrospinal fluid
flow. The T2-weighted MRI scans, 10 years later,
demonstrate a complete resolution of the dilatation at
Th11-Th12 (D) and a decrease at Th5-Th7 (E).
Neurological examinations remained normal.
The severity of spinal cord trauma was variable (complete cord
lesion, 40.0%; incomplete cord lesion, 40.5%; radiculopathy, 8%;
no neurological symptoms related to trauma, 12%). The free

JÖRG KLEKAMP
interval between accident and symptoms of syringomyelia aver-
aged 142.2  126.5 months with extensive variations. For 2.4% of
patients symptoms started within 3 months of the accident and for
another 11.2%, within the first year, whereas 63.3% reported an
interval of at least 5 years.
Among the remaining 439 patients, 69 patients presented a
tethered cord syndrome with the syrinx starting at the level of
tethering, expanding cranially (Figure 12). The tethering was
related to a low positioned conus in 58 patients (84.1%) or to
split cord malformations in 11 patients. A total of 146 patients
(67.9%) with tethered cord syndrome showed no associated
syrinx. Women predominated men by 2:1. Comparing
tethered cord patients with and without syringomyelia
revealed no significant differences for neurological symptoms
(Table 5).
In 370 patients with syringomyelia, CSF flow obstructions
related to spinal nontraumatic arachnopathies (i.e., arachnoid
webs, pouches, or cysts) were responsible (Figure 10). In 48.0% of
these patients the syrinx expanded in the cranial direction, in
22.3% the syrinx was found below that level, and in 29.7% the
syrinx had progressed in both directions.
For patients with a history of meningitis (n ¼ 65), subarachnoid
hemorrhage (n ¼ 43) (Figure 13), intrathecal injections of
irritating substances, such as myodil (n ¼ 6) or previous
intradural surgery (n ¼ 25), secondary arachnopathies had
developed leading to syrinx formation. In 115 of these patients
(82.7%) the arachnopathy exceeded 3 spinal segments, encasing
the spinal cord completely. The free interval between its
formation and new neurological symptoms averaged 114.3 
133.0 months. All patients presented with severe neurological
deficits at a mean age of 53.1  11.9 years, with an average
history of 54.9  85.9 months (Table 6).
Figure 9. (A) T2-weighted sagittal magnetic resonance imaging (MRI) scan
of a space-occupying, fluid-filled cavitation at C5-Th1. Its shape is
symmetrical, suggesting a low pressure compared with a glioependymal
cyst as shown in Figures 4 and 5. (B) The axial scan demonstrates its central
location. (C) The cine-MRI in systole discloses no fluid movements inside
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For the remaining 231 patients with no known cause for an
arachnopathy (i.e., primary arachnopathies), direct visualization
of these more subtle pathologies required thin-sliced sagittal
images with short acquisition times. Axial T2-weighted images
often demonstrated indirect signs such as displacement or
compression of the spinal cord. In every patient, the arachnoid
pathology was located in the posterior subarachnoid space
(Figure 10). Cine-MRI imaging was useed for inconclusive cases
to demonstrate disturbances of CSF flow. In 90.4% of patients
with primary arachnopathies, the upper to mid-thoracic spine
was affected. Patients presented at a younger age of 45.9  14.4
years and an average history of 52.5  71.6 months with an equal
sex distribution. Comparing patients with primary arach-
nopathies with and without syringomyelia (n ¼ 62) revealed
significant differences for sensory deficits only (c2 tests:
P ¼ 0.01) (Table 6).
DISCUSSION
Not every fluid-filled cavitation of the spinal cord warrants the
diagnosis of syringomyelia.4 According to neuroradiologic and
clinical criteria, cystic spinal cord tumors, glioependymal cysts,
and myelomalacias should and can be distinguished from
syringomyelia. This may not always be possible ad hoc and
require several MRI scans and clinical examinations over time
(Figures 8, 9, and 14). The distinction between syringomyelia
and central canal dilatation may be particularly difficult.
However, it is the author’s experience that all of these spinal
cord pathologies can be differentiated, once clinical signs of a
myelopathy have appeared. Whenever neurological symptoms
are missing or cannot be connected to the spinal cord segments
of an intramedullary fluid collection, these patients should be
the cyst and no obstruction of cerebrospinal fluid flow. The 29-year-old
patient presented with a 5-month history of back pain. The lesion was
considered asymptomatic and the provisional diagnosis of a central canal
dilatation was made. Follow-up examinations were recommended.
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Figure 10. (A) T2-weighted sagittal magnetic resonance imaging (MRI) scan
of a syrinx C2 to Th3. The syrinx displays its largest diameter in its lower
part. The arrow marks the level of Th3-4 corresponding to B. (B) The
cine-MRI in diastole shows flow signals inside the syrinx in its lower half
(arrowhead) and a cerebrospinal fluid (CSF) flow obstruction at Th3-4
(arrow). (C) The axial T2-weighted image at Th3-4 demonstrates an
arachnoid web in the posterior subarachnoid space slightly displacing the
cord anteriorly. The patient presented with a 5-year history of radicular
monitored with regular neurological and MRI examinations before
a definite diagnosis is made.
Cystic and Solid Intramedullary Tumors with Syringomyelia,
Glioependymal Cysts and Myelomalacias
Cystic intramedullary tumors, which could be misdiagnosed for
syringomyelia, are very rare entities.5 With the application of
gadolinium some uptake on MRI could be detected in both
patients with such tumors (Figure 2). Likewise, all solid
intramedullary tumors with an associated syringomyelia took up
gadolinium in this series (Figure 3).6-8
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dysesthesias in his trunk followed by pain and sensory changes in his right
arm. (D, E) After resection of the arachnopathy at Th3-4 and insertion of a
duraplasty the postoperative sagittal MRI scans demonstrate a free CSF
passage at the operated level and an almost complete collapse of the
syrinx. Although pain in his right arm improved, sensory changes and
dysesthesias in his trunk persisted unchanged for the past 4 years since
surgery.
Glioependymal cysts contain fluid similar to CSF and are lined
by cuboidal cells with no separate basement membrane. Their
pathogenesis is not established.9,10 Their wall will not take up
contrast on MRI.6,11 About half of these cysts were observed in
the conus region or lower thoracic cord (Figures 4 and 5), with
the other half in the cervical area.6,10,11 In the literature, other
names applied are ependymal cyst,9,12 terminal syringomyelia,13
or terminal ventricle.14-16 In the sagittal plane, they appear as
symmetrical, ovoid cysts without septations, with a considerable
space-occupying effect and round-shaped cyst poles, suggesting
an elevated pressure. A syrinx, on the other hand, displays no
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Table 4. Symptoms of Patients with Chiari I Malformations

Symptom Chiari I Malformation with Syrinx (n [ 543) Chiari I Malformation without Syrinx (n [ 199)

Pain 75.9% 79.8%

Sensory disturbances 66.8% 26.9%
Dysesthesias 44.7% 18.3%
Motor weakness 39.2% 13.5%
Gait ataxia 58.7% 51.4%
Sphincter disturbances 12.8% 8.7%
Caudal cranial nerve dysfunction 13.6% 18.3%

Figure 11. (A) T2-weighted sagittal magnetic resonance
imaging scan in a 46-year-old woman with
post-traumatic syringomyelia C4 to Th12 due to spinal
trauma 25 years ago, resulting in complete paralysis at
Th12. The syrinx size is largest close to the
post-traumatic arachnopathy and gradually decreases
in a cranial direction. (B) The postoperative scan after
establishment of a free cerebrospinal fluid pathway at
Th12 and a duraplasty demonstrates a complete
collapse of the syrinx. Sensory deficits and pain in her
arms improved, motor deficits remained unchanged.

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Figure 12. (A, B) T2-weighted sagittal magnetic resonance postmyelographic computed tomography disclosed a small cyst
imaging scans demonstrating a large syrinx from C3 to L1. The at L1, displacing the conus anteriorly and a thick filum terminale
syrinx is largest in the cervical and upper thoracic areas (i.e., a tethered cord). (D, E) After transsection of the filum and
suggesting an underlying pathology in this region. (C) A cyst removal, the syrinx decreased considerably.

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venous congestion,18,19 leading to breakdown of spinal cord

Table 5. Symptoms of Patients with Tethered Cord Syndromes tissue.20 Once the destroyed cord tissue has disappeared, a
Tethered Cord Tethered Cord defect results appearing like a cyst (Figure 6).4,21-25 These mye-
Syndrome with Syndrome without lomalacias will not cause progressive symptoms and remain
Symptom Syrinx (n [ 69) Syrinx (n [ 146) unchanged over time, requiring no specific treatment. If symp-
toms progressed, post-traumatic cord tethering, arachnoiditis,
Pain 76.4% 78.7% or degenerative diseases of the spine in adjacent levels were
Sensory disturbances 54.2% 62.0% responsible in this series (Figure 6).21,26 Other pathologies
Dysesthesias 31.9% 24.0%
leading to profound spinal cord atrophy may also lead to an MRI
appearance mimicking a syrinx or a central canal dilatation.27
Motor weakness 59.7% 59.3% Typically, the atrophied thoracic cord shows a triangular shape
Gait ataxia 69.4% 52.0% in axial scans with the dentate ligaments on either side and
Sphincter disturbances 61.1% 60.0% the posterior arachnoid septum in the midline, holding the
cord in its normal position (Figure 7). No signs of cord
compression or CSF flow obstruction could be detected in
these patients.
symmetrical shape in the sagittal plane but resembles the shape
of an elongated drop, with the largest diameter close to the Central Canal Dilatation
underlying pathology and a smaller one at the other end, Dilatations of the central canal have been named as idiopathic
marking the direction of further expansion (Figures 10, 11, and syringomyelia28 or hydromyelia3,29 under the assumption that they
13)17—with rare exceptions (Figure 12). In cine-MRI studies of are the result of CSF entering the central canal from the 4th
CSF flow, no flow signals could be detected in glioependymal ventricle through the obex. This theory is consistent with animal
cysts (Figure 5), whereas syrinx cavities displayed flow signals models producing hydromyelia after kaolin injection into the
particularly close to the area of the underlying pathology cisterna magna.30,31 However, different from some animal species
(Figure 10). Likewise, flow voids could never be detected in the central canal does not remain patent in humans as demon-
glioependymal cysts and were an exclusive feature of strated by autopsy findings.32,33 Therefore, terms, such as
syringomyelia (Figures 11 and 12). As most are incidental communicating syringomyelia, hydromyelia, or syringohy-
findings, surgery should be reserved for patients with dromyelia, are misleading and should be abandoned.3
corresponding neurological symptoms. Yearly MRI are Modern MRI may be able to demonstrate patent parts of the
advisable as some cysts may enlarge with time or may turn out central canal.34,35 Most of these are detected in the lower cervical
as an intramedullary tumor (Figure 14). and in the mid-thoracic cord. Characteristically, they show no
Myelomalacia is a tissue defect inside the spinal cord related space-occupying effect, with those in the cervical cord more
to trauma, ischemia, degenerative spine diseases, or chronic prominent. In MRI scans taken over several years, these

Figure 13. (A) T2-weighted sagittal magnetic resonance imaging scan of a the distorted shape of the cord due to the arachnoid scarring encasing the
syrinx C4 to Th8 related to a posthemorrhagic arachnopathy, Th3 to Th7 cord completely. (C) The scan in the cervical region demonstrates the
due to a subarachnoid hemorrhage from a cerebellar arteriovenous syrinx extension into the left dorsal root entry zone explaining the patient’s
malformation 5 years earlier. (B) The axial scan at a thoracic level discloses neuropathic pain syndrome.

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JÖRG KLEKAMP
Table 6. Symptoms of Patients with Spinal Arachnopathies
Primary Arachnopathy
Symptom with Syrinx (n [ 231)
Pain 75.1%
Sensory disturbances 65.2%
Dysesthesias 53.0%
Motor weakness 37.9%
Gait ataxia 60.1%
Sphincter disturbances 19.4%
dilatations either remain unchanged or regress (Figure 8).28,36
However, some dilatations may express a space-occupying effect
(Figure 9). The spindle shape of these dilatations suggests a lower
intracavitary pressure (Figure 9) compared to glioependymal cysts
with their round cyst poles (Figure 5). To distinguish central canal
dilatations from syringomyelia, cine-MRI will demonstrate no
flow signal inside the dilatation and no obstruction of CSF
flow adjacent to it (Figure 9), unlike in syringomyelia (Figure 10).
This distinction may require repeated scans and clinical
examinations.4,34,37
Finally, in patients with central canal dilatations, clinical
symptoms will not correspond to their spinal representation.28,36
A lesion in the center of the cord may cause a dissociated sen-
sory loss in corresponding dermatomes, but will certainly not
explain back pain, which was the commonest reason for MRI in
these patients.
It has been suggested that central canal dilatations can be
differentiated from syringomyelia by electrophysiologic measure-
ments of so-called silent periods related to spinothalamic tract
fibers.29,38 Very often the diagnosis of a central canal dilatation
remains based on exclusion. If features of cystic tumors, glioe-
pendymal cysts, myelomalacia, or pathologies known to lead to
syringomyelia are not detectable on MRI and the patient’s
symptoms cannot be anatomically related to the spinal cord
region in question, the diagnosis of a central canal dilatation is
the most likely diagnosis. With the diagnostic efforts made as
suggested in this article it is the author’s experience that these
patients face a very low risk of a revised diagnosis in the years to
come.
Diagnosing Syringomyelia
Syringomyelia does not represent a spinal cord disease in its
own right but is a secondary event of another disease that causes
it.3,4,39-41 This implies that idiopathic syringomyelia does not
exist: for each and every syrinx there is an underlying pathology.
In this series, the commonest cause of syringomyelia in a
patient referred with the provisional diagnosis of idiopathic
syringomyelia was a thoracic arachnopathy.4,40 Whenever
syringomyelia is suspected, the patient’s history, clinical exam-
ination, and MRI scans have to be analyzed for evidence of
pathologies known to be associated with it (i.e., disorders of
CSF flow [Figures 10 and 11], spinal cord tethering [Figure 12],
e742 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.12.156
ORIGINAL ARTICLE
SYRINGOMYELIA
Primary Arachnopathy Secondary Arachnopathy
without Syrinx (n [ 64) with Syrinx (n [ 139)
57.1% 61.2%
52.4% 94.8%
34.9% 37.9%
44.4% 80.2%
63.5% 96.6%
30.2% 68.1%
or an intramedullary tumor [Figure 3]).4,40,42-44 Whenever such
an association cannot be established, the diagnosis of syringo-
myelia should not be made.
It may be argued that intramedullary cysts associated with solid
intramedullary tumors should be considered as a separate entity
and not be included under the heading of syringomyelia. However,
all neuroradiologic features of tumor-associated cysts are identical
to syrinx cavities related to other causes, as outlined in this article.
Successful treatment of the cause of a particular syrinx leads to its
decrease regardless of etiology. A tumor-associated cavitation
does not require any surgical measures just like those related to
other entities. Therefore, intramedullary cavitations associated
with intramedullary tumors should be considered as syringomyelia
as well.4,40
How can the pathology responsible for a syrinx be identified?
Unless an intramedullary tumor cannot be ruled out otherwise,
contrast application is not mandatory for the neuroradiologic
workup,45 but each syrinx should be examined over its entire
length. As a general rule it will display its largest diameter close
to the area of the underlying pathology (Figures 10 and 13).
Whereas pathologies at the craniocervical junction,
intramedullary tumors, tethered cord syndromes, and secondary
arachnopathies are usually well demonstrated on standard MRI,
localizing a primary arachnopathy (i.e., arachnoid webs, cysts,
or pouches that may move with the pulsatile flow of CSF)
requires to look carefully for signs of subtle, circumscribed areas
of cord compression.17,46-48 These features, however, render
their detection difficult in standard images.17,49-51 It is in this
subgroup, that cine-MRI studies were found to be most helpful, as
these arachnopathies always alter CSF flow (Figure 10). In
comparison with cine-MRI, myelography will miss a large pro-
portion of nontraumatic arachnopathies.17,52
If in doubt about the diagnosis of syringomyelia, it is recom-
mended to follow the patient and to repeat imaging and neuro-
logical examinations. In the author’s experience no harm is done
if the diagnosis of a central canal dilatation has to be changed to
syringomyelia once new aspects appear during follow-up. On the
other hand, a wrong diagnosis of syringomyelia may have
unnecessarily placed a psychologic burden on the patient with
further side effects such as problems getting insurance policies or
a having a potentially harmful neurological diagnosis in their
medical record.

JÖRG KLEKAMP
Figure 14. (A) T2-weighted sagittal magnetic
resonance imaging (MRI) scan of a cyst at C5-C6.
At that time, the patient had complained about
sensory disturbances of varying intensity in her
face and upper extremities for the past 15 years.
(B) The cine-MRI in systole showed no flow
signals inside the cyst, yet cerebrospinal fluid flow
was compromised due to its space-occupying
effect. An examination with contrast in T1 (not
available) was reported to show no enhancement
in this region. A glioependymal cyst was
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ORIGINAL ARTICLE
SYRINGOMYELIA
suspected and the patient was followed with
regular MRI and clinical examinations. (C) The MRI
scan 7 years later showed a solid intramedullary
tumor inside the cyst, which enhanced after
contrast application in T1 (D). The patient still
complained about inconsistent sensory changes
but had developed a slight gait ataxia in this
period. She underwent surgery; the histologic
diagnosis was a World Health Organization grade
II ependymoma.
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 ORIGINAL ARTICLE
JÖRG KLEKAMP SYRINGOMYELIA

If we accept the concept of syringomyelia as a secondary phe-
nomenon, such a patient can be expected to present a mixture of
symptoms related to the underlying pathology and the syrinx
(Tables 1, 4e6). With a precise analysis of clinical symptoms in
relation to their spinal cord representation, it can be determined
which symptoms may be attributed to a particular syrinx and
which may be related to the underlying pathology. For this pur-
pose, it is important to note whether the syrinx is restricted to the
central region of the cord or involves the posterior horns.53 The
characteristic clinical symptom of a centrally located syrinx is
the dissociated sensory loss with disturbances of pain and
temperature sensation and preserved sensation for light touch
and joint position.54 With a syrinx affecting the posterior horns,
on the other hand, the typical symptom is neuropathic pain,
which is often described as a burning type of constant pain
aggravated at times of rest (Figure 13).55,56 Dysesthesias, sensory
disturbances, or pain provoked by maneuvers, such as Valsalva,
coughing, laughing, or sneezing in corresponding dermatomes,
are typical signs of syringomyelia and were more common in
Chiari I malformations and primary arachnopathies with syrin-
gomyelia compared with those without (Tables 4 and 6). Pain
related to exercise or local back pain, on the other hand,
cannot be explained by a syrinx, regardless of its cord
representation, but may be explained by the underlying
pathology or additional degenerative spinal disorders. Motor
deficits tend to be a late symptom of syringomyelia and are
more commonly related to the underlying pathology if they
appear early in the clinical course.
As a general rule, the underlying pathology becomes clinically
manifest before symptoms of syringomyelia appear.4,17 Syringo-
myelia is a secondary phenomenon. Analyzing how clinical
symptoms began and identification of those not attributable to a
particular syrinx should be used as a guide to find the underlying
pathology from a clinical viewpoint. This course of events may
explain why symptoms are so similar irrespective of presence or
absence of a syrinx (Tables 1, 4e6) and why the extent of a syrinx
has such a weak correlation to a patient’s neurological status at
presentation. In this series, neurological symptoms could often be
fully explained by the primary disorder. If patients with syringo-
myelia showed additional neurological symptoms, these tended to
involve mostly sensory changes, dysesthesias, and neuropathic
pain (Tables 1, 4e6).
CONCLUSIONS
Among fluid-filled cavitations in the spinal cord cystic tumors,
glioependymal cysts, myelomalacias, dilatations of the central
canal, and syringomyelia should be differentiated. The diagnosis
of syringomyelia should be reserved for patients with a fluid-filled
cavity in the spinal cord of any size related to either a disturbance
of CSF flow, spinal cord tethering, or an intramedullary tumor.
Once the cause of the syrinx has been determined, treatment can
be focused exclusively on this underlying pathology. For patients
in whom an underlying pathology cannot be established, the
diagnosis of syringomyelia should be withheld until it can be
proven.

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Conflict of interest statement: The author declares that the
article content was composed in the absence of any
commercial or financial relationships that could be construed
as a potential conflict of interest.
Received 4 October 2017; accepted 26 December 2017
Citation: World Neurosurg. (2018) 111:e729-e745.
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