J Neurosurg Spine 17:199211, 2012

Treatment of posttraumatic syringomyelia

Clinical article
Jrg Klekamp, M.D.
Department of Neurosurgery, Christliches Krankenhaus, Quakenbrck, Germany
Object. This paper presents results of a prospective study for patients undergoing surgery for posttraumatic
syringomyelia between 1991 and 2010.
Methods. A group of 137 patients with posttraumatic syringomyelia were evaluated (mean age 45 13 years,
mean follow-up 51 51 months) with pre- and postoperative MRI and clinical examinations presenting in this period
and followed prospectively by outpatient visits and questionnaires. Surgery was recommended for symptomatic patients with a progressive course. Short-term results were determined within 3 months of surgery, whereas long-term
outcomes in terms of clinical recurrences were studied with Kaplan-Meier statistics.
Results. Three groups were distinguished according to the type of trauma: Group A, patients with spinal trauma
but without cord injury (ASIA E, n = 37); Group B, patients with an incomplete cord injury (ASIA C or D, n = 55);
and Group C, patients with complete loss of motor function or a complete cord injury (ASIA A or B, n = 45). Overall,
61 patients with progressive symptoms underwent 71 operations. Of these operations, 61 consisted of arachnolysis,
untethering, and duraplasty at the trauma level (that is, decompression), while 4 ASIA A patients underwent a cordectomy. The remaining procedures consisted of placement of a thecoperitoneal shunt, 2 opiate pump placements, and
2 anterior and 1 posterior cervical decompression and fusion. Seventy-six patients were not treated surgically due
to lack of neurological progression or refusal of an operation. Neurological symptoms remained stable for 10 years
in 84% of the patients for whom surgery was not recommended due to lack of neurological progression. In contrast,
60% of those who declined recommended surgery had neurological progression within 5 years. For patients presenting with neurological progression, outcome was better with decompression. Postoperatively, 61% demonstrated a
reduction of syrinx size. Although neurological symptoms generally remained unchanged after surgery, 47% of affected patients reported a postoperative improvement of their pain syndrome. After 3 months, 51% considered their
postoperative status improved and 41% considered it unchanged. In the long-term, favorable results were obtained
for Groups A and C with rates for neurological deterioration of 6% and 14% after 5 years, respectively. In Group B,
this rate was considerably higher at 39%, because arachnolysis and untethering to preserve residual cord function
could not be fully achieved in all patients. Cordectomy led to neurological improvement and syrinx collapse in all 4
patients.
Conclusions. The technique of decompression with arachnolysis, untethering, and duraplasty at the level of the
underlying trauma provides good long-term results for patients with progressive neurological symptoms following
ASIA A, B and E injuries. Treatment of patients with posttraumatic syringomyelia after spinal cord injuries with
preserved motor functions (ASIA C and D) remains a major challenge. Future studies will have to establish whether
thecoperitoneal shunts would be a superior alternative for this subgroup.
(http://thejns.org/doi/abs/10.3171/2012.5.SPINE11904)

Key Words posttraumatic tethering spinal arachnoiditis syringomyelia

n the past it was believed that posttraumatic syringomyelia was the result of a severe spinal cord injury
and that it developed from an area of myelomalacia
after resorption of intramedullary blood and breakdown
products of destroyed cord tissue.7 Although treatment
was based on syrinx shunting to various compartments
for decades, modern treatment principles follow different concepts, which have mainly evolved since the introduction of MRI in the 1980s. Current concepts recognize
and treat the pathophysiology of syringomyelia, which
is related to obstruction of CSF flow.24,32 In patients with
posttraumatic syringomyelia, posttraumatic arachnopa-

J Neurosurg: Spine / Volume 17 / September 2012

thies at the injury level disturb CSF flow and require surgical management to reduce the syrinx and to stabilize
the neurological status. This relationship between spinal
arachnoid scarring and syringomyelia was recognized
as early as 1938 by Adelstein,2 but its importance was
not appreciated for a long time. The modern treatment
of posttraumatic syringomyelia started in the 1980s with
Bernard Williams,75 who still inserted syringoperitoneal
or syringopleural shunts but also attempted to create a
free CSF passage at the level of posttraumatic arachnoid
scarring.
Since 1991, all patients presenting with spinal cord
199

J. Klekamp
pathologies have been entered into a spinal cord database and followed prospectively. In 1997, a first study on
patients with syringomyelia related to spinal arachnoid
scarring using this database was published comparing
results for traditional syrinx shunting operations with a
modified Williams technique that established a free CSF
passage at the level of arachnoid scarring in combination
with an expansile duraplasty without additional shunting
of the syrinx.33 This technique had been developed independently at the University of California in Los Angeles by Ulrich Batzdorf in the late 1980s and at Nordstadt
Hospital Hannover, Germany, in the early 1990s based on
the original ideas of Bernard Williams. The 1997 study
came to the conclusion that improving CSF flow at the injury level and enlarging the subarachnoid space gave far
better long-term results than shunting operations.33 Since
then, a number of other groups have published their favorable results with this technique as well.3,15,29,43,50,53,67
The present paper describes the treatment results
for patients with posttraumatic syringomyelia who were
treated by improving CSF passage at the trauma level and
presented between 1991 and 2010.
Patient Population

Methods

A total of 1151 patients with syringomyelia presented

at the Nordstadt Hospital in Hannover, Germany, between
1991 and 2003 (chairman until 2002, Madjid Samii, M.D.;
chairman in 2003, Michael Gaab, M.D.) or the Christliche Krankenhaus in Quakenbrck, Germany, between
2004 and 2010 with spinal cord pathologies and had their
data entered into the spinal cord database. Apart from
general patient data and specific features of each spinal
cord pathology, the neurological examinations before surgery, before discharge from the hospital, 3 months postoperatively, and yearly thereafter were documented with
respect to individual symptoms using a scoring system
(Table 1).34 In 137 of the 1151 cases, the syringomyelia
was related to trauma, and these cases were included in
the present study. All patients presented with MR images.
Additional cardiac-gated cine MRI studies were performed in some patients if the CSF flow obstruction was
expected to extend beyond the injury levelas in patients
who had undergone intradural operationsor in those

without obvious spinal injuries. Depending on the associated posttraumatic pathology of the spine, CT scans and
conventional radiographs were obtained. Myelograms and
postmyelographic CT scans were obtained only in exceptional cases, such as in patients with severe metal artifacts
on MRI. In general, surgery was recommended as soon as
neurological symptoms started to progress. For patients
wishing to undergo surgery predominantly for pain relief,
it was emphasized before surgery that successful operations with resolution of the syrinx do not ensure improvement of dysesthetic or neuropathic pain syndromes even
if other neurological symptoms may improve. In patients
with profound degenerative changes of the cervical spine
the decision which pathology required treatment first
the arachnopathy or the degenerative processwas based
on clinical examinations.
Surgical Management

Decompression. All operations were performed with

the patient in the prone position. In patients who had undergone fusion and for those with spinal stenosis, laminectomies were performed to expose the dura mater. In
all other patients, the lamina were removed with a small
craniotome so that they could be reinserted with titanium
miniplates at the end of the procedure. After exposure of
the dura, the extent of the arachnoid pathology was visualized with ultrasound. Then the dura was opened under
the operating microscope in the midline without opening
the arachnoid mater. With the dura held open with sutures,
the arachnoid pathology could be studied and adequate
cranial and caudal exposure was ensured to gain access
to normal and unaffected subarachnoid space on either
side. Only sharp dissection of the arachnoid was used to
avoid tearing blood vessels which might be embedded in
the arachnoid, as illustrated elsewhere.35,36 The arachnoid
was grasped with microforceps and held under slight tension during dissection. The posterior medial septum was
identified first and incised with microscissors, leaving
the so-called intermediate layer of the arachnoid, which
covers spinal cord blood vessels,46 untouched on the cord
surface. Once the area of the arachnopathy was reached,
sharp dissection was continued in the same fashion. In
this way, a free CSF passage in the posterior subarachnoid space could be created in every patient across the

TABLE 1: Neurological scoring system

Pain

5
4

none
slight, no medication

normal
present, not significant

good control w/ medication

insufficient control
w/ medication
severe despite medication
incapacitating

significant, function not restricted

some restriction of function

2
1
0

200

Sensory Disturbance,
Dysesthesias

Score

Motor Weakness
full power
movement against
resistance
movement against
gravity
movement w/o gravity

severe restriction of function contraction w/o

movement
incapacitated function
plegia

Gait Ataxia

Sphincter
Function

normal
normal
unsteady, no aid slight disturbance, no
catheter
mobile w/ aid
residual, no catheter
few steps w/ aid rarely incontinent
standing w/ aid
plegia

frequent use of catheter

permanent catheter

J Neurosurg: Spine / Volume 17 / September 2012

Posttraumatic syringomyelia
region of trauma. Dissection was then continued laterally
on either side toward the dentate ligaments. This led to
complete untethering of the cord in the majority of cases.
No arachnoid dissection was performed anterior to the
dentate ligaments unless the patient had suffered a complete spinal cord injury. In those cases, a complete untetheringposteriorly, laterally, and anteriorlywas performed. In all other patients, areas of anterior arachnoid
scarring were left untouched. If syrinx shunts had been
implanted close to or at the level of the posttraumatic
arachnopathy they were removed, provided they were not
stuck inside the spinal cord. Syrinx shunts implanted in
areas unaffected by the posttraumatic arachnopathy were
left in place. At closing of the microsurgical part of the
operation, an expansile duraplasty was performed with
a tight running suture and finally lifted up with tenting
sutures on either side. To avoid scar formation and tethering between duraplasty and spinal cord, artificial material
was preferred for duraplasty (Gore-Tex, W. L. Gore & Associates GmbH) (Figs. 1 and 2). In patients with profound
soft tissue scarring after multiple surgeries or severe soft
tissue trauma, a lumbar drain was placed and removed
after 510 days to prevent the formation of a CSF fistula.
Cordectomy. For patients with a complete cord injury, a cordectomy was offered as an alternative whenever surgery aiming at improving the CSF pathway was
considered not likely to succeed, as in patients who had
undergone multiple shunting operations (Fig. 3) or those
with severe posttraumatic kyphosis. For that purpose,
the same surgical approach was used as described for
decompression. After the dura was opened at the injury
level, the spinal cord was detached from all fixations to
surrounding structures and then completely transected
immediately above the area of cord destruction. Another
transection was then performed below the level of cord

injury to remove the destroyed cord segment. With the

cranial and caudal part of the remaining cord untethered
in this fashion, a duraplasty was inserted and the wound
closed as described.
Outcome Analysis and Statistical Methods

After discharge from the hospital, all surgically treated patients were examined 3 months postoperatively, and
yearly follow-up was conducted by means of further outpatient visits or questionnaires.
For statistical tests of significance, Student t-tests
and Fisher tests were employed. Surgical morbidity was
defined as a new, permanent postoperative deficit or a
permanent aggravation of a preexisting deficit within 30
days after the operation. Long-term follow-up data were
analyzed with Kaplan-Meier statistics30 to determine the
percentages of patients with a stable neurological status (progression-free survival) or progressive symptoms
(clinical recurrence).
Mean values are presented with standard deviations.

Results
Preoperative Data

Between 1991 and 2010, 137 patients with posttraumatic syringomyelia (101 males, 36 females) were
encountered. The mean age at presentation was 47 14
years (range 374 years) (Table 2). The mean duration of
follow-up was 55 55 months (range 2 weeks19 years).
According to the degree of spinal cord involvement with
the underlying trauma, 3 groups could be distinguished.
Group A was made up of patients with spinal injury
without spinal cord trauma. Neurological symptoms had
either been entirely absent or had consisted of radicular

Fig. 1. A: Sagittal T2-weighted MR image obtained in a 49-year-old male patient 30 years after a conservatively managed
fracture at T-6 that was not associated with any neurological symptoms. It shows a syrinx from the T-1 to the T-10 level and a
profound kyphosis. Ten years after the accident the patient started to notice weakness in his left leg. A syrinx was found between
T-4 and T-10, which subsequently ascended to T-1. B: Axial T2-weighted image demonstrating an area of posterior tethering
on the right side. At presentation 20 years after the onset of new symptoms, the patient had to use a walker due to a spastic
paraparesis. A correction of the kyphosis was discussed but refused by the patient. He underwent T-6 and T-7 laminotomy with
arachnolysis and duraplasty. C: Postoperative MR image obtained 7 months after surgery showing a reduction of the syrinx.
As of this writing, the patients neurological situation has remained stable for 3 years.

J Neurosurg: Spine / Volume 17 / September 2012

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J. Klekamp

Fig. 2. A: Sagittal T2-weighted MR image obtained in a 46-year-old male patient 17 months after a motor vehicle accident that
caused a spinal cord concussion with a persistent sensory level at T78, showing a small syrinx from T-2 to T-4. B: Sagittal MR
image obtained 1 year later showing a slight expansion of the syrinx now crossing the level of the T12 disc space. C and D:
Axial T2-weighted images, adjacent slices. The first image (C) poorly demonstrates the surface contour of the cord at the lower
end of the syrinx due to pulsation artifact from the arachnoid pouch that compresses the cord posteriorly on the next axial image
below that level (D). E: Axial MR image showing that the demarcation and form of the spinal cord returned to normal below the
arachnopathy at T-5. The abrupt change of syrinx caliber at T-4, the cranial ascent of the syrinx, the slight posterior compression
at T-4, and the motion artifacts at that level are indirect signs of the posttraumatic arachnopathy causing the syrinx. A decompression at T-4 was undertaken. F: Postoperative MR image obtained 3 months after decompression showing complete resolution
of the syrinx. The patients clinical condition was unchanged.

symptoms that resolved completely (ASIA E4). There

were 37 patients in this group (Fig. 1).
Group B was made up of patients who had an incomplete spinal cord injury with preserved motor function with or without subsequent recovery (ASIA C or D).
There were 55 patients in this group (Fig. 2).
Group C was made up of patients who had a complete
spinal cord injury or incomplete cord injury with complete
loss of motor function without subsequent recovery (ASIA
A or B). There were 45 patients in this group (Fig. 3).
All syrinx cavities started at the level of trauma and
expanded from there (Fig. 2). In 46% of cases, they extended in the rostral direction, in 20% in the caudal direction, and in the remaining 36%, they extended in both
directions, with the rostral component predominating in
all cases. The lower the spinal level of trauma, the more
likely the syrinx was to expand only in rostral direction
and vice versa. There was no correlation between syrinx
orientation and severity of the original cord injury.
202

The interval between trauma and the onset of new neurological symptoms was extremely variable and extended
between 1 month and 46 years (mean 130 121 months).
From the time of symptom onset, an average of 56 71
months (range 1 week30 years) passed before the patients
presented for neurosurgical evaluation. Clinical histories
related to syringomyelia tended to be shorter for patients
with more severe cord injuries (p = 0.1005, Fisher test), but
there was no similar trend for a shorter interval between
trauma and the onset of syrinx-related symptoms for patients with more severe cord injuries (Table 2).
The most common initial symptoms of posttraumatic
syringomyelia were a new motor deficit (the initial symptom in 28% of patients) and a new type of pain (in 26%).
New sensory deficits, dysesthesias, or a worsening of gait
were less common initial signs (in 14% each). Two patients noticed sphincter disturbances and one had swallowing problems as the first new sign. Table 3 lists the
percentages for individual neurological symptoms in all
J Neurosurg: Spine / Volume 17 / September 2012

Posttraumatic syringomyelia
TABLE 3: Symptoms at presentation
Percentage of Pts w/ Symptom Described
Symptom

Group A

Group B

Group C

All Pts

hypesthesia
dysesthesia
pain
motor weakness
gait ataxia
sphincter disturbance

84
46
70
78
89
57

96
62
71
85
94
71

73
42
73
62
100
100

85
51
71
77
95
78

First Operations

groups, providing an overview of the clinical spectrum

at presentation.

Among the surgically treated patients, 5 of 17 patients in Group A, 9 of 28 patients in Group B, and 3 of 16
patients in Group C had already undergone placement of
syrinx shunts without success. The remainder had undergone no surgery other than interventions to deal with the
initial spine trauma.
Four patients with asymptomatic syringomyelia (1
from Group A, 1 from Group B, and 2 from Group C)
underwent cervical fusion procedures without decompression. The Group A patient and 1 Group C patient underwent posterior fusions for instability, and the Group B
patient and 1 Group C patient underwent ventral fusions
for cervical disc disease. One Group A patient refused decompression surgery and was treated with an opiate pump
for his pain syndrome. One patient in each of Groups B
and C was treated with a cordectomy; the Group B patient
had progressed from ASIA C to ASIA A, and the Group
C patient presented after multiple shunting attempts and
had posttraumatic kyphosis (Fig. 3).

Management

Subsequent Operations

Fig. 3. Left: Sagittal T2-weighted MR image obtained in a 38-yearold male patient 20 years after a burst fracture at T67 that resulted in
complete paraplegia. Within 3 years before presentation the patient underwent 7 attempts at syrinx shunting at other institutions (to control the
syrinx) without any success. Due to the postsurgical arachnoid scarring
and the kyphosis at the injury level, a decompression was considered
unlikely to stabilize the ascending neurological symptoms. Right: After cordectomy, the syrinx completely collapsed and the patient noticed
recovery of his hand function mainly due to improved sensory function.
As of this writing the improvement has lasted for 2 years.

Of 137 patients with posttraumatic syringomyelia, 76

patients (20 of 37 in Group A, 27 of 55 in Group B, and
29 of 45 in Group C) were not treated surgically, either
for lack of progression of neurological symptoms (50 patients) or because they refused surgery (26 patients). The
61 remaining patients underwent 71 surgical interventions (Table 4). Two of these made the decision to undergo surgery 6 and 11 years after the initial presentation.

One patient in Group C underwent subsequent insertion of an opiate pump for treatment of persistent pain
despite successful treatment of the syrinx with stabilization of his neurological status by decompression. Eleven
patients experienced a clinical recurrence after decompression. Four of these refused further surgical attempts.
The other 7 patients underwent 9 subsequent surgeries:
1 patient in Group C underwent a cordectomy for recur-

TABLE 2: Summary of clinical and demographic characteristics in 137 patients with posttraumatic syringomyelia*
Characteristic

Group A

Group B

Group C

All Pts

mean age at presentation (yrs)

mean clinical history (mos)
mean latency (mos)
trauma level
cervical
thoracic
conus

46 15
80 97
103 88

49 13
50 58
160 132

45 13
44 54
119 127

47 14
56 71
130 121

13
20
4

8
36
11

10
34
1

31
90
16

* Group A comprised patients with no spinal cord trauma (n = 37); Group B, patients with incomplete cord trauma (n = 55); Group
C, patients with complete cord trauma (n = 45). For details, see Methods. Abbreviation: Pts = patients.
Duration from onset of symptoms of syringomyelia to presentation.
Interval between the initial trauma and the onset of new neurological symptoms.

J Neurosurg: Spine / Volume 17 / September 2012

203

J. Klekamp
TABLE 4: Types of operations*

Type
decompression
decompression & fusion
cordectomy
posterior fusion
ventral fusion
thecoperitoneal shunt
opiate pump

Group A

Group B

Group C

Total

1st Op 2nd Op Total

1st Op 2nd Op Total

1st Op 2nd Op Total

1st Op 2nd Op Total

14
1
0
1
0
0
1

0
0
0
0
0
0
0

14
1
0
1
0
0
1

25
1
1
0
1
0
0

4
1
1
0
1
1
0

29
2
2
0
2
1
0

13
2
1
0
0
0
0

0
0
1
0
0
0
1

13
2
2
0
0
0
1

52
4
2
1
1
0
1

4
1
2
0
1
1
1

56
5
4
1
2
1
2

* Values represent numbers of operations.

rence of symptoms and syringomyelia. All other subsequent surgeries involved 6 patients in Group B: 4 patients
underwent revision at the site of decompression for recurrent tethering of the spinal cord. One patient in Group B
had progressed to ASIA A after multiple syrinx shunting operations at another institution before presentation.
A cordectomy was suggested but refused. After unsuccessful decompression he underwent placement of a thecoperitoneal shunt with short-term success (1 year), and
then, after it became blocked, finally accepted the cordectomy, which was followed by sustained neurological improvement. One patient with a cervical trauma underwent
a second posterior decompression plus fusion and an anterior revision with corpectomy, vertebral reconstruction,
and plating to deal with spinal stenosis and instability after the initial decompression.
Outcome for Surgically Treated Patients

Among 61 operations undertaken with the goal of

improving CSF flow at the site of the trauma, 10 complications were encountered, for a complication rate of 16%.
Three postoperative hematomas and 3 wound infections,
of which 2 were aseptic, were observed. One CSF leak
developed and 1 paraplegic patient experienced a cardiac
arrest 3 hours after surgery but recovered with no sequelae after resuscitation. Two patients suffered a urinary
tract infection. Permanent surgical morbidity (permanent
neurological worsening within 1 month after surgery) was
observed after 4 operations (6.5%). Transient postoperative deterioration occurred in 8% of patients and mainly
involved sensory function.
Table 5 gives an overview of postoperative changes
in syrinx size after decompression and shows that the best
results were obtained in Group C. The rate of postoperative syrinx decrease was not influenced by the presence
or absence of kyphotic angulation at the injury level (syrinx decrease in 71% of patients with kyphosis and 61%
of those without) (Fig. 1). After cordectomy, all syrinx
cavities collapsed completely (Fig. 3), while syrinx cavities remained unchanged after ventral or posterior cervical fusion.
Short-term clinical outcome data for patients who
underwent decompression surgery are provided in Table
6. Overall, significant improvements were observed for
dysesthesias and pain in this period, whereas other neu-

204

rological symptoms remained unchanged. These trends

were observed for each individual patient group. Overall,
51% considered their postoperative outcome as improved
and 41% as unchanged, whereas 8% reported further
worsening within 3 months after surgery. Overall, 47% of
affected patients reported an improvement of dysesthetic
or neuropathic pain syndromes with the remainder observing no change.
Seven surgically treated patients were lost to followup. Long-term outcomes in terms of neurological recurrence rates are presented in Table 7. The analysis revealed
profound differences between Groups A, B, and C (Fig.
4), whereas a posttraumatic kyphosis did not have a negative predictive effect. The best results were observed for
patients in Groups A and C, with the difference from the
results in Group B just failing to reach statistical significance (p = 0.07, log-rank test). Comparing first and revision surgeries demonstrated a trend for a lower recurrence rate in patients undergoing a first decompression.
In the long term, however, recurrence rates after 10 years
were almost identical, with no statistically significant differences. Likewise, recurrence rates tended to be lower
for patients without posttraumatic kyphosis, but this difference was not statistically significant (Table 7).
All patients treated with cordectomy demonstrated
sustained neurological improvements without a clinical
recurrence during follow-up.
Among 7 patients undergoing further operations after a first decompression, 4 were eventually stabilized.
The condition of 3 patients in Group B continued to deteriorate, and the patients declined further operations.
Outcome for Patients not Treated Surgically

Seventy-six patients (20 in Group A, 27 in Group B,

and 29 in Group C) were not treated surgically. Of these
TABLE 5: Postoperative changes in syrinx size after
decompression
Percentage of Cases
Postop Size

Group A

Group B

Group C

All Pts

decrease
no change
increase

50
36
14

55
24
21

88
6
6

61
24
15

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Posttraumatic syringomyelia
TABLE 6: Early postoperative clinical results after
decompression*
Group
hypesthesia
preop
postop
3 mos
dysesthesia
preop
postop
3 mos
pain
preop
postop
3 mos
motor weakness
preop
postop
3 mos
gait ataxia
preop
postop
3 mos
bladder dys
preop
postop
3 mos
overall result
improved
unchanged
worse

Group A

Group B

TABLE 7: Clinical recurrence rates after decompression or

conservative management

Group C

Recurrence Rate (%)

All Pts
Treatment & Group

3.1 0.5
3.1 0.8
3.2 0.7

2.7 0.8
3.1 0.8
3.1 0.7

3.1 1.0
3.2 1.1
3.3 1.2

2.9 0.8
3.2 0.8
3.3 0.8

3.4 1.3
3.9 0.9
3.9 0.9

3.9 1.0
4.1 0.9
4.1 0.8

3.7 1.2
4.0 1.1
4.1 1.1

3.8 1.1
4.1 0.9
4.2 0.9

3.8 1.2
4.0 1.0
4.2 0.7

3.9 1.0
4.2 0.7
4.1 0.9

3.3 1.0
3.8 1.0
3.9 1.0

3.7 1.1
4.0 0.9
4.1 0.9

3.5 0.8
3.3 0.8
3.4 0.9

3.1 1.0
3.2 1.1
3.2 1.0

3.7 1.2
3.8 1.2
3.9 1.3

3.4 1.0
3.4 1.0
3.5 1.0

2.7 1.2
2.5 1.3
2.6 1.3

2.1 1.4
2.1 1.4
2.1 1.4

2.4 1.4
2.4 1.4
2.4 1.4

3.4 1.5
3.2 1.6
3.2 1.6

2.6 1.8
2.6 1.9
2.7 1.9

2.9 1.8
2.9 1.9
2.9 1.9

38%
50%
13%

52%
39%
9%

66%
35%

51%
41%
8%

* Dys = dysfunction.
Significant difference between preoperative and 3-month postoperative scores (p < 0.01).

76 patients, 19 were lost to follow-up. Of the remaining 57 patients, 38 remained in stable condition for up
to 17 years after the initial presentation (mean 67 61
months). This figure corresponds to rates of progressionfree survival of 79% after 5 years and 65% after 10 years
in the Kaplan-Meier analysis. There were no differences
in outcome based on the type of cord trauma or whether
patients had previously undergone shunt placement. The
condition of 34 of the 38 patients in whom conservative
management was recommended remained stable, while
that of 4 patients progressed. Of 19 patients with a surgical recommendation who had refused the operation initially, 4 subsequently underwent decompression surgery
in 2011 and 2012; the condition of 11 patients remained
unchanged for up to 6 years while that of 4 patients deteriorated. Comparing progression-free survival rates for
patients with or without a recommendation for surgery
revealed a significantly worse outcome for patients with
a surgical recommendation after 5 years (40% vs 94%
J Neurosurg: Spine / Volume 17 / September 2012

decompression
Group A
Group B
Group C
all
1st surgery
revision surgery
conservative management
all
surgery not advised
surgery refused

5 Yrs

10 Yrs

6%
38%
14%
28%
22%
38%

6%
74%

55%
56%
53%

21%
6%
60%

35%
16%
100%

after 5 years and 0% vs 84% after 10 years, respectively;

p = 0.0004, log-rank test). In 6 of 8 patients with neurological deterioration, the progression was related to the
posttraumatic cord tethering, with worsening of gait and
sphincter functions, rather than the syrinx, with ascending neurological deficits.

Discussion
Incidence and Pathophysiology

The incidence of posttraumatic syringomyelia is difficult to determine. Estimates are based on trauma center surveys of patients who have had spinal cord injury
and were followed up regularly with MRI. These patients
carry a risk for neurological deterioration of 2% per year
from various causesspinal instability, cord tethering,
cord compression, and syringomyelia.55 However, areas
of cord destruction followed by cystic degeneration (myelomalacia) and formation of a new cyst expanding from
the level of cord injury (posttraumatic syringomyelia) are
not differentiated in most studies. Cystic changes at the
site of cord trauma are common and occur in at least 50%
of patients after severe spinal cord injuries.5 In a survey
of spinal cordinjured patients 28% were identified with
a posttraumatic syrinx.49 The spinal level of trauma appears to be irrelevant to the probability of developing a
posttraumatic syrinx.76 It has been proposed that the likelihood of developing posttraumatic syringomyelia correlates with the severity of cord injury.16,50,69 However,
posttraumatic syringomyelia may develop in patients after complete recovery from cord injury63,68 as well as in
patients without any spinal cord injury at all.13
The treatment of posttraumatic syringomyelia as described in this paper is based on the pathophysiological
link to posttraumatic arachnoid scarring and CSF flow
obstruction at the level of trauma.76 Spinal cord tethering may contribute further to its development.9 Syringomyelia does not require a spinal cord injury to develop.
Arachnoid scarring has been shown to raise the subarachnoid space pressure above the obstruction in a computer
205

J. Klekamp

Fig. 4. Kaplan-Meier analysis demonstrating progression-free survival (lack of clinical recurrence) after decompression in Groups A, B,
and C (p = 0.07, log-rank test).

model11 and in experimental studies.38 Obstruction of

CSF flow also causes changes in the distribution of extracellular fluid in the spinal cord,38 leading to syringomyelia.24,32,37 Increased flow in the perivascular spaces has
been implicated in this effect.10,14,32,38,60,61 There appears
to be an evolution from spinal cord edema (that is, the socalled presyrinx state) to syringomyelia.21 Once syringomyelia has developed, the increased intramedullary pressure45 and fluid movement inside the syrinx3,64 may lead
to progressive spinal cord damage23,52,59 and neurological
symptoms.
Apart from the syrinx, posttraumatic tethering and
compression of the cord by arachnoid scars, pouches, and
cysts at the level of injury may also cause progressive
neurological deficits1,16,1820,42 (Figs. 1 and 2). This aspect
is of particular significance for patients of Group B in
this study. A further aspect to consider is posttraumatic
spinal deformity. Restoration of spinal alignment in the
acute phase may decrease the risk of posttraumatic syringomyelia1,6,49,58 and should improve the chance of successful treatment for those patients who have developed
it. Patients with dislocated spine fractures and spinal stenosis tend to develop a posttraumatic syrinx earlier than
those without spinal canal compromise.49,69 A study by
Perrouin-Verbe et al.,49 published in 1998, determined a
cutoff point of 30% canal stenosis as a predictor for the
development of a posttraumatic syrinx.
Therefore, patients with a posttraumatic syrinx may
present new neurological symptoms from a variety of
causes: spinal cord tethering or compression and spinal
instability, as well as syringomyelia. Furthermore, degenerative processes of the spine have to be considered.
Every patient with a posttraumatic syrinx requires a thorough evaluation to address the right target for surgery.
Neuroradiological Diagnosis

Neuroradiological assessment in patients with posttraumatic syringomyelia has to account for the different
mechanisms that may be involved in neurological progression. It is not sufficient to just demonstrate the exact extent of the syrinx. To demonstrate a posttraumatic
arachnopathy, a technically adequate MRI examination

206

is required, with particular emphasis on thin-slice axial

T2-weighted images at the area of injury (Figs. 1 and 2).
As the arachnoid is not visible on conventional MR images, indirect signs of the arachnopathy have to be looked
for, such as an area of cord displacement, adhesion, or
compression18,28 (Figs. 1 and 2). As arachnoid webs move
with the pulsatile flow of CSF, they may cause motion
artifacts, distorting the surface contour of the spinal cord
right at the level of the arachnopathy in sagittal and axial
T2-weighted images (Fig. 2).28 Constructive interference
steady state sequences can be used not only for the demonstration of the syrinx26 but may sometimes be helpful to
detect arachnoid webs, scars, and cysts, because this technique is less susceptible to CSF flow artifacts.26 Cine MRI
should be applied to demonstrate the extent and precise
levels of CSF flow obstruction,3 particularly in patients
in whom previous operations may have caused additional
arachnoid scarring on top of the posttraumatic arachnopathy or in those without cord injury. In all patients, the
syrinx starts at the level of injury and expands mainly in
the rostral direction in the majority of patients12,25 (Fig. 2).
Myelography, on the other hand, is less sensitive than
cine MRI for demonstrating areas of CSF flow obstruction,44 because arachnopathies may involve the posterior
subarachnoid space only,16 and intrathecal contrast may
bypass such areas almost without any delay via unobstructed sections of the subarachnoid space.
For patients with spinal instrumentation, MRI may
no longer be able to show the intradural anatomy at the
injury level in sufficient detail. In such instances CT myelography is recommended and extremely helpful for intraoperative orientation.
Depending on the posttraumatic anatomy of the
spine, a CT in bone window technique and functional radiographs may be required to demonstrate the size and
form of the spinal canal in the involved area and to rule
out insufficient instrumentation or spinal instabilities.
Clinical Presentation

The clinical picture is characterized by an extremely

variable interval after trauma before symptoms of posttraumatic syringomyelia begin.3,11,15,17,24,28,39,42,50,53,54,57,66,67,76
When symptoms develop, they may involve pain, sensory
changes, motor weakness, deteriorations of gait, autonomic dysfunctions, or increased spasticity.3,12,16,18,39,42,54,76
Depending on the presence and severity of cord injury,
the clinical course and the major concerns of the patients
vary considerably. Patients in Group A became symptomatic although they had a functionally intact spinal cord
after the initial trauma. This explains the longer history
before the diagnosis is made and the less severe neurological symptoms before surgery compared with patients
in Groups B and C. For patients in Group B, the most
common major complaint was a deterioration of gait,
which could be attributed to cord tethering or syringomyelia. The clinical course should be considered in surgical
planning: A patient with progressive gait problems but
no signs of ascending neurological deficits will require
a successful untethering procedure in addition to restoration of CSF flow to benefit from surgery. On the other
hand, if a patient complains about ascending neurological
J Neurosurg: Spine / Volume 17 / September 2012

Posttraumatic syringomyelia
deficits only, he may benefit from restoration of CSF flow
and control of the syrinx even if untethering is not completely achievable.
For patients in Group C, pain and new motor deficits
predominated the clinical picture. Their new symptoms
were exclusively related to the syrinx, as the complete
cord injury rendered arachnoid scarring and tethering at
the injury level either clinically irrelevant or of minor significance.
Surgical Management

Considering the clinical courses for patients with and

without surgery as documented in this study, surgery for
posttraumatic syringomyelia should be reserved for patients with progressive neurological symptoms indicating
a need for early intervention.16,18,57 The first line of treatment should focus on restoration of normal CSF flow at
the trauma level combined with untethering of the cord
and duraplasty (Figs. 1 and 2). Spinal realignment or fusion may be added, depending on the biomechanical status at that level.27 This strategy leads to better long-term
results than syrinx shunting operations.3,29,33,57
The superior results for decompression can be attributed to 3 reasons. First of all, the cause of the syrinx
can be treated effectively with this technique. Second, the
posttraumatic tethering, which may lead to a progressive
myelopathy in its own right,16,18,20 can be released as well.
Third, syrinx shunting procedures are associated with a
high rate of long-term complications requiring frequent
revisions.8,25,56 By comparison, such shunts can only address symptoms related to the syrinx but will not influence the neurological problems related to posttraumatic
tethering or cord compression from an arachnopathy.
For the development of the surgical technique as described in this paper, the contributions of Bernard Williams have to be acknowledged. He emphasized that a
posttraumatic syrinx starts at the level of the injury and
is related to arachnoid scarring and CSF flow obstruction
right there. So he applied his strategy of decompressions
for Chiari malformation to patients with posttraumatic
syringomyelia. He restored CSF flow and created a pseudomeningocele by suturing the dura into the muscle at
the injury level. At first, he combined this with a syrinx
shunt.75 In part due to the first results in Los Angeles and
Hannover of using this technique without syrinx shunt
placement, he did not recommend additional shunts in
later publications.57,76 He was a strong advocate of large
pseudomeningoceles to counterbalance the pressure dissociations he found with his pressure recordings,73 arguing that any duraplasty would unnecessarily limit the
subarachnoid space at the level of the previous CSF flow
obstruction. Two problems, however, may arise with this
technique: 1) The subarachnoid space may be contaminated with blood products from the wound provoking an
arachnoiditis that can be severe enough to cause a myelopathy or lead to reobstruction of CSF flow. 2) The size
of the pseudomeningocele may decrease with time due to
bulging of muscles into this artificial CSF space.
In the 1990s several centers published reports of large
case series involving patients with spinal cord injuries
who had suffered late progressive neurological symptoms
J Neurosurg: Spine / Volume 17 / September 2012

attributed to posttraumatic cord tethering. Even though an

intraoperative collapse of an associated syrinx was commonly seen after untethering, these groups did not fully
appreciate the effects of cord untethering and improving
CSF flow for treatment of posttraumatic syringomyelia.
If they observed no syrinx collapse after untethering, a
syrinx shunt was placed.16,1820,42
To obtain a sufficient CSF passage at the injury level,
arachnolysis and duraplasty alone may not be sufficient.
For patients with a spinal stenosis or severe kyphotic
deformity it may also be required to restore the sagittal
alignment of the spine.15,27,50 In fact, this alone has been
shown to decrease the size of a posttraumatic syrinx in
some cases.27,54 Whereas establishing a sufficient size of
the spinal canal to deal with a stenosis usually provides
no problem, the correction of ventral compressions and
kyphotic angulations may have to involve major surgical
interventions depending on the involved spinal level. 58
Falci et al.18 were rather restrictive in recommending
kyphectomies for patients with posttraumatic cord tethering. Such surgery does carry considerable risks, particularly in the mid- and upper thoracic spine in patients with
residual cord function.
In this study, a negative effect of posttraumatic kyphosis on postoperative outcome after decompression could
not be demonstrated (Fig. 1). This may be explained by
the small number of patients with severe kyphosis but may
also represent the selection of patients for surgery. Surgery
to restore a free CSF pathway was not recommended for
patients in Group C if severe kyphotic angulation was present, and a cordectomy was suggested instead.
Alternatively, thecoperitoneal shunts have been introduced, which drain CSF from the subarachnoid space
above the trauma level to the peritoneal cavity.40,48,62,70,71
For cavities extending into the cervical cord, ventriculoperitoneal shunts have been used for the same purpose. 51,77
However, there exists little experience concerning the
optimal pressure setting for such shunts. Programmable
valves may be used, but they are not designed for this
purpose. Where should the valve be placed? No data exist
as to how much tissue coverage may be allowed over the
valve without losing the ability to change the pressure setting with the programming device. Low-pressure valves
have also been used. In one patient in this series even a
low-pressure valve provided too much pressure resistance,
so eventually a valveless drain was used. This worked for
a year, after which the catheter became blocked and the
paraplegic patient finally agreed to a cordectomy.
For patients with a complete cord lesion, cordectomy is a very effective form of treatment for syringomyelia17,22,31,41,57,74 (Fig. 3). However, the psychological burden
entailed by a patients decision to accept this operation
should not be underestimated. Most patients prefer to
undergo a decompression first, and this procedure does
provide good results in the great majority of patients.22
Patients will accept a cordectomy if the ascending neurological symptoms cannot be arrested by a decompression
or shunting procedures. On the other hand, in Group C
patients with severe kyphotic deformities, a cordectomy
is the reasonable first choice of treatment.
In patients with a posttraumatic syrinx, distinguish207

J. Klekamp
ing between symptoms related to syringomyelia and
those related to cervical degenerative disc disease may
be difficult. Such degenerative changes can be a profound
problem for patients confined to a wheelchair.57 In a patient without radicular symptoms or neck pain, the clinical picture of a cervical myelopathy can be indistinguishable from a posttraumatic syrinx or posttraumatic cord
tethering. In such instances functional radiographs of the
cervical spine should be evaluated carefully. If signs of
hypermobility or instability are detectable, a ventral or
posterior fusion should be considered. The spinal cord of
a patient with syringomyelia may react to hypermobility
in the cervical spine earlier and more profoundly than one
would generally expect. This should also be kept in mind
for patients presenting with new neurological problems
after successful treatment of a syrinx.
Complications and Surgical Morbidity

Postoperative complications were observed in 16%

of cases in this series, a rate similar to those reported in
the literature.3,19,57,67 Surgical treatment of arachnoid scarring, which may encase the spinal cord from all sides, is
not without risks. In patients without previous spinal cord
injury (Group A) severe tethering is very unlikely. In such
cases, the arachnoid pathology is almost always restricted
to the posterior subarachnoid space. If sharp arachnoid
dissection respects the intermediate layer of the arachnoid
on the spinal cord surface, no permanent surgical morbidity should occur. For patients with complete cord lesions
(Group C), surgery at the injury level poses no danger of
further loss of neurological function. Restoring CSF flow
and untethering the cord can be performed without any
restrictionseven to the extent of a cordectomywithout
risking additional deficits. However, surgical morbidity
may be a significant problem for patients after multiple
syrinx shunting attempts and those with incomplete cord
injuries (Group B). It is in these patients that arachnoid
dissection and untethering threaten residual spinal cord
function in an area of already existing structural damage. The low surgical morbidity in this study (6.1%) is a
consequence of the conservative approach in these cases.
A free CSF pathway can almost always be created posteriorly. However, restrictions apply for the extent of untethering anteriorly.
Most papers on posttraumatic syringomyelia do not
report data for surgical morbidity. Ushewokunze et al.67
reported a surgical morbidity rate of 10% in their group
of 40 patients treated by means of arachnolysis and duraplasty. Falci et al.18 presented a series of 362 patients with
posttraumatic cord tethering after spinal cord injury. The
authors described their surgical untethering in this and a
previous paper.19 Their untethering extended further anteriorly. The dentate ligaments were cut and the cord rotated
along its longitudinal axis to achieve a complete untethering if found necessary. They stated that 9% of patients experienced a loss of motor function, 7% a loss of light touch,
and 12% a loss of pinprick sensation due to these operations. According to their patients self assessment, 11% had
lost some sensory or motor function with this surgery. The
lower figure determined in this study may be attributable to
the more conservative strategy toward anterior untethering.
208

Postoperative Radiological Outcome

Analyzing postoperative MRI studies showed a reduction of the syringomyelia cavity in 61% of cases (Figs.
1 and 2), while 24% remained unchanged. Reexpansions
were seen in 15% (Table 5). The best results were obtained for patients in Group C, in whom reexpansions
were exceptional events. As a consequence of the surgical restrictions in patients in Group B, radiological results
were considerably worse in this group.
Other investigators reported similar figures for syrinx
reductions with the decompression technique as applied
in this study.3,57,67 However, they did not analyze these
results in relation to the severity of the original cord injury. Even though it is well established that a syrinx may
decrease very slowly after successful restoration of CSF
flow,72 several authors recommend a syringosubarachnoid
shunt if the syrinx does not collapse intraoperatively after
untethering the cord.1820,42 As insertion of a catheter may
also cause additional arachnoid scarring, this strategy is
not recommended.
Short-Term Outcome

The best effect of surgery was obtained for pain.19,39,47,66

However, not all types of pain respond equally to successful treatment of posttraumatic syringomyelia. Pain provoked by maneuvers such as coughing or sneezing usually
improves if the syrinx regresses postoperatively. On the
other hand, the response of neuropathic and dysesthetic
pain is unpredictable.65 A positive effect was reported by
9 (47%) of 19 affected patients after surgery in this study
irrespective of changes of the syrinx or other neurological
symptoms, which is similar to other reports in the literature.18,65
Even with successful reduction of syrinx size, neurological deficits usually remained unchanged in this study
(Table 6). Similar experiences are reported in the literature.3,18,29,76 Nevertheless, 51% of patients considered their
condition improved 3 months after surgery, while 41%
reported an unchanged status. Given the fact that surgery
was restricted to patients with progressive symptoms, this
can be considered a favorable short-term result.
Long-Term Outcome of Operated Patients

Analysis of long-term results was performed with

Kaplan-Meier statistics to account for the varying followup times and to determine the rate of patients experiencing a neurological relapse after surgery (Table 7). Sixtyone operations were performed to improve CSF flow with
arachnolysis and duraplasty. Overall, 14 clinical recurrences were encountered (representing a recurrence rate
of 23%). According to Kaplan-Meier statistics, this figure
indicates 5- and 10-year progression-free survival rates of
72% and 45%, respectively. Similar figures were reported
by Aghakhani et al.3 in their study involving 19 patients
treated in this manner. Their analysis also clearly showed
significantly better results with this technique than with
syrinx shunt procedures. Ushewokunze et al.67 presented
a series of 40 patients, continuing Williams work in Birmingham. They used his technique first but later changed
their policy regarding the handling of the dura and perJ Neurosurg: Spine / Volume 17 / September 2012

Posttraumatic syringomyelia
formed duraplasties. Their series included only patients
with complete or incomplete cord injuries. Twenty-one of
33 patients adequately studied with MRI showed a reduction of the syrinx postoperatively. After 6 months, 11 of
25 patients were neurologically improved and 6 considered
their status unchanged, whereas 8 demonstrated progression. In the long term, 23 of 40 patients required no further
operations, while 17 patients underwent subsequent procedures for neurological recurrences. In 9 of these 17 cases,
the patients condition could be stabilized with additional
operations. Postoperative dysesthetic pain was observed
in 5 patientspossibly related to leaving the dura open in
some of the authors earlier cases.
Other authors mentioned the number of subsequent
reoperations that were necessary after arachnolysis, untethering, and duraplasty to comment on long-term results.
Lee et al.42 observed that only 1 of 19 patients required a
revision after untethering the cord without syrinx shunting. In the group of 25 patients treated with shunting only,
3 patients condition worsened and 2 needed shunt revisions, while in the group treated with untethering plus
shunting 33% had a clinical recurrence.
Long-term results were considerably better for patients in Groups A and C than for those in Group B (Table
7; Fig. 4). This could lead one to consider syrinx shunts
for this subgroup instead. However, if the data on shunted
patients from the study in 199733 are compared with the
results for decompression in Group B of the present study,
the recurrence rates for decompressions are significantly
lower (38% at 5 years postoperatively, as compared with
88% in the 1997 study; p = 0.0004, log-rank test).
Long-Term Outcome Without Surgery

The analysis of cases in which surgery was not performed clearly indicates that patients with posttraumatic
syringomyelia who present in clinically stable condition
should not undergo surgery. Their course appears to be
rather benign for a considerable amount of time. The
rates for progression-free survival for up to 10 years for
this subgroup of patients who did not undergo surgery
are comparable to those for surgically treated patients in
Groups A and C. For patients presenting with a history
of progressive symptoms, however, results in surgically
treated patients in Groups A and C are better than the
results in patients who refused the operation.
For patients in Group B, on the other hand, the situation is more complex. The number of cases with sufficient
follow-up data involving patients who did not undergo
surgery despite its being recommended is too low to allow any conclusion to be drawn about whether decompression would have provided a better result.

Conclusions

This study indicates that long-term results of surgery

for posttraumatic syringomyelia have improved with the
technique of arachnolysis, restoration of CSF flow, untethering the cord, and duraplasty compared with times
when a syrinx shunt was considered the only option.
But the results are still far from perfect for a significant
number of patients (Fig. 4). Patients who have progresJ Neurosurg: Spine / Volume 17 / September 2012

sive neurological signs without initial cord injuries have

a very good prognosis with this type of surgery. Clinical recurrences were observed in only 6% in this group
within 10 years. Patients with complete cord injuries and
deteriorating neurological condition can always be stabilized. If a decompression fails, a cordectomy can arrest
further neurological progression once and for all. However, problems remain for patients with syringomyelia after
incomplete cord injuries (Group B in this study). Stabilizing their neurological status without risking their fragile
residual cord function is a real challenge. Looking at the
results of this study, it remains open whether decompression provided a benefit for this subgroup compared with
patients who were not treated surgically. Even if the syrinx has been controlled, residual tethering may still cause
a progressive myelopathy, threatening the patients walking abilities and sphincter function. Performing surgery
on an injured spinal cord does carry significant risks.
Looking at this case series, one can see that a learning
curve was clearly evident. It remains to be seen whether
results can be improved for this subgroup with growing
experience or whether thecoperitoneal shunts are a better
alternative, even though they do not address symptoms
related to posttraumatic tethering.
Disclosure
The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this
paper.
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Manuscript submitted October 17, 2011.
Accepted May 30, 2012.
Please include this information when citing this paper: published online July 13, 2012; DOI: 10.3171/2012.5.SPINE11904.
Address correspondence to: Jrg Klekamp, M.D., Department of
Neurosurgery, Christliches Krankenhaus, Danziger Strasse 2, 49610
Quakenbrck, Germany. email: j.klekamp@ckq-gmbh.de.

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