From the Annals of Weill Cornell Neurological Surgery
Persistent Syringomyelia After Posterior Fossa Decompression for Chiari Malformation
Umberto Tosi, Jacques Lara-Reyna, John Chae, Roshann Sepanj, Mark M. Souweidane, Jeffrey P. Greenfield
- BACKGROUND: Chiari malformation (CM) is often co-
morbid with syringomyelia. The treatment of CM via pos-
terior fossa decompression (PFD) may not improve
syringomyelia in up to 40% of patients, based on historical
cohorts. Management of these patients is problematic, as
both reoperation and syrinx shunting have high failure
rates in the long term.
- METHODS: We retrospectively reviewed our cases in
which patients with CM type 1 or 1.5 and syringomyelia
underwent PFD without postoperative improvement in sy-
ringomyelia. Symptomatology and radiographic measure-
ments were collected at presentation and on the first and
latest available postoperative scans and analyzed. We
present 2 cases to illustrate the challenges in the man-
agement of these patients.
- RESULTS: Our cohort consisted of 48 consecutive pa-
tients with CM and syringomyelia who underwent PFD. Of
these, 41 patients had postoperative improvement in or
resolution of syringomyelia. We subsequently studied the
cohort of 7 patients who underwent PFD with (n [ 5) or
without (n [ 2) durotomy and demonstrated worsening of
syringomyelia following surgery. This cohort had mean
(
SEM) preoperative syrinx area of 23.9
10.0 mm2.
Postoperatively, the mean syrinx area increased to 40.5

9.6 mm2 and 57.3
12.5 mm2 on the first and latest post-
operative scans available (P [ 0.02), for an increase of
106.9%
94.4% and 186.0%
107.4% (P [ 0.04).
Presenting symptoms included occipital headache, pares-
thesias, visual deterioration, and paraspinal pain. On last
follow-up (mean 13.9
4.9 months), the majority of
Key words
- Chiari
- Posterior fossa decompression
- Syringomyelia
Abbreviations and Acronyms
AP: Anteroposterior
CC: Craniocervical
CCI: Craniocervical instability
CM: Chiari malformation
CM-1: Chiari malformation type 1
CM-1.5: Chiari malformation type 1.5
CSF: Cerebrospinal fluid
454 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2020.01.148
symptoms were resolved in this cohort, despite persistence
of syringomyelia.
- CONCLUSIONS: In this small cohort of unique patients,
syrinx resolution was not achieved via decompression
surgery. Despite “radiographic failure,” good symptom
control was achieved, with most patients remaining or
becoming asymptomatic postoperatively, thus supporting
our rationale for what has largely been a conservative
approach in this population.
INTRODUCTION
C hiari malformation type 1 (CM-1), characterized by
descent of the cerebellar tonsils below the foramen
magnum, and type 1.5 (CM-1.5), characterized by
accompanying brainstem herniation, are often concomitant with
syringomyelia.1-6 The origins of both syringomyelia and its cere-
brospinal fluid (CSF) build-up remain unclear, with most studies
suggesting that the cause is altered CSF dynamics at the level of
the craniocervical junction as a consequence of tonsillar occlusion
of the foramen magnum.3 Others have shown CSF flow from the
subarachnoid space into the syrinx cavity.2 Patients presenting
with both syringomyelia and CM-1 or CM-1.5 have different
symptomatology from those with no syrinx, as CSF abnormalities
in the spine are associated with sensory anomalies (most
commonly, paresthesias), muscle weakness, and other spinal de-
formities, such as scoliosis.7-10 The classic Chiari symptomatology
(occipital headaches, visual disturbance, unsteady gait, etc.) may,
nonetheless, be present in these patients. The presence of syrin-
gomyelia is often considered sufficient for surgical intervention in
MRI: Magnetic resonance imaging
PFD: Posterior fossa decompression
Department of Neurosurgery, Weill Cornell Medicine, New York, New York, USA
To whom correspondence should be addressed: Jeffrey P. Greenfield, M.D., Ph.D.
[E-mail: jpgreenf@med.cornell.edu]
Supplementary digital content available online.
Citation: World Neurosurg. (2020) 136:454-461.
https://doi.org/10.1016/j.wneu.2020.01.148
Journal homepage: www.journals.elsevier.com/world-neurosurgery
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UMBERTO TOSI ET AL.
asymptomatic patients, although observation of asymptomatic
patients is absolutely acceptable with close monitoring.11
The objective of surgery in the treatment of syringomyelia
associated with CM-1 or CM-1.5 is 2-fold: (1) to reduce the over-
crowding of the posterior fossa and reestablish normal CSF flow at
the level of the foramen magnum (as in any patients with CM-1)
and (2) by doing so, to indirectly eliminate the pathophysiology
at the origin of syringomyelia, thus preventing progression of
myelopathy.12 Although posterior fossa decompression (PFD) has
high success rates in the reestablishment of CSF flow and
normalization of posterior fossa crowding, syrinxes may fail to
resolve; failure rates of 10% to 40% have been reported.13-15
Improvement usually occurs within 3 months of PFD and con-
tinues thereafter, with most patients experiencing syrinx reduction
in the first postoperative year.12 When such an approach fails, a
second PFD or syrinx shunting procedure is often performed;
however, long-term results suggest that direct shunting presents
challenges as a long-term solution, requiring reoperation in up to
50% of cases.16,17
Several factors might explain failure of a syrinx resolution after
PFD. A prospective study of patients with persistent syringomyelia
after PFD found impaired CSF flow at the level of the foramen
magnum in all patients observed. When this partial obstruction
was removed by enlargement of the previous craniotomy, syrinx
size decreased in 15 of 16 patients.18 Another study similarly found
that obstruction of the foramen of Magendie was associated with
persistent syringomyelia after PFD; removal of the obstruction
resulted in syrinx resolution in all but 1 of 8 patients.6 It is
unclear whether these patients would benefit from syrinx
shunting.
In this study, we present 7 cases, all from our Chiari/syrinx
database, in which PFD failed to reduce or stabilize a patient’s
syrinx. These patients demonstrated an increase in syrinx diam-
eter and area postoperatively. Although they constitute only a
small subset of our overall Chiari cohort, we believe they represent
a population that is important to study. Despite their radiographic
progression, all of the patients who had been symptomatic pre-
operatively reported excellent symptom improvement that war-
ranted postponing a second intervention. We present 2 cases in
detail in order to illustrate symptomatology at presentation and
radiographic findings in the preoperative and postoperative
periods. For such a cohort of patients we thus advocate for con-
servative management and repeated imaging rather than imme-
diate syrinx shunting or a second PFD.
METHODS
We performed a retrospective analysis of prospectively collected
data from cases of CM-1 and CM-1.5 with associated syringomyelia
in which patients underwent PFD between 2015 and 2019. Among
these patients, we identified those with worsening postoperative
syringomyelia for further analysis. For the purpose of this study,
we used the following inclusion criteria: patients with CM-1 or
CM-1.5 and syringomyelia (>3 mm in the largest diameter) who
underwent a first-time PFD surgery and for whom complete
medical records, including both preoperative and postoperative
magnetic resonance (MR) images, were available. We collected
general demographic data, presenting symptoms (headache, neck
WORLD NEUROSURGERY 136: 454-461, APRIL 2020
FROM THE ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION
pain, sensory symptoms, etc.), and clinical outcomes. We defined
and obtained the following measurements on T2-weighted MR
images: maximum extent of tonsillar descent; syrinx size, utilizing
the maximum size in anteroposterior (AP) orientation and cross-
sectional diameter; and number of spinal levels involved. Imag-
ing was performed preoperatively and postoperatively, generally—
but not consistently—at 6-month intervals. Syrinx area was
calculated by multiplying the 2 (lateral and AP) radii using the
following formula: (RAP  RLATERAL  p). These data points were
collected on the first postoperative scan available and on the latest
postoperative scan performed. Change in syrinx area was calcu-
lated using the following formula: (postopAREA e preopAREA)/
preopAREA  100.
Statistical Analysis
Mean values are presented with the standard error of the mean.
Analyses were performed using GraphPad Prism (version 7.0 or
later). Paired Student’s t test was used to compare 2 groups. One-
way analysis of variance with post-hoc Tukey’s multiple compar-
ison test was used to compare 3 groups.
RESULTS
Our cohort consisted of 7 patients (2 male and 5 female) between
4.1 and 54.5 years old (mean: 25.6 years); 3 met criteria for CM-1.5,
and 4 had CM-1. Preoperatively, the mean tonsillar descent was
14.0 mm (
2.5 mm); the mean syrinx area was 23.9 mm2 (
10.0
mm2), and the mean syrinx span was 7 spinal levels (
2 levels).
There was no difference in these metrics between CM-1 and CM-
1.5 patients. One patient (patient 6) did not have a syrinx preop-
eratively. Demographics and preoperative information are sum-
marized in Table 1 (syrinx lateral and AP dimensions are given in
Supplementary Table 1). Presenting symptoms (summarized in
Table 2 along with postoperative status) included occipital
headaches, paresthesias, balance problems, memory
deterioration, visual deterioration, back and neck pain, apnea,
and syncope (this last not directly related to CM). Patient 4 did
not have any Chiari-related presenting symptom, but imaging
performed because of febrile seizures revealed a substantial syrinx
and tonsillar descent that led to the intervention. Patients had, on
average 3.0 (
0.7) presenting symptoms, with an average post-
operative resolution rate of 84% of them (
7.6%). Four patients
had complete symptom resolution postoperatively. Neck pain,
imbalance, and paresthesias are the only symptoms that failed to
resolve in all patients. No patient had hydrocephalus preopera-
tively or postoperatively.
In all 7 cases, the surgical approach (summarized in Table 3 for
those patients in whom the dura was opened and in Table 4 for
those in whom the dura was spared) included suboccipital
craniectomy and resection of the posterior arch of C1. A
durotomy was performed in 5 cases; in all of these cases, the
dura was closed with autologous duraplasty. In one patient in
whom an extradural approach was performed, this decision was
made due to our primary goal of addressing craniocervical
instability. The goal of craniocervical instability management
with occipital-cervical fusion superseded addressing what we
believed was a small and asymptomatic syrinx. Although dura-
plasty can be performed at the same time as fusion we believed
www.journals.elsevier.com/world-neurosurgery 455
 FROM THE ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
UMBERTO TOSI ET AL. WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION

Table 1. Demographic Information and Preoperative Radiographic Measurements of the Patient Cohort
Preoperative

ID Age (years) Sex Chiari Subtype Syrinx Area (mm2) Syrinx Span Tonsillar Herniation (mm) Tonsillar Shape

1 17.2 M 1.5 18.4 C5-T3 13 Peg-shaped

2 44.9 F 1 4.6 C1-C3 8.5 Peg-shaped
3 25.5 F 1 10.8 C2-T3 6 Rounded
4 4.1 F 1 69.1 C3-T1 10 Peg-shaped
5 22.6 M 1 53.1 C2-T10 19.5 Peg-shaped
6 10.2 F 1.5 0 N/A 25 Peg-shaped
7 54.5 F 1.5 11.3 C6-T2 15.7 Peg-shaped

this was unnecessary and spared the dura. In the second patient, a month) after surgery. The latest scan available was performed,
44-year-old woman, magnetic resonance imaging (MRI) demon- on average, 15.4 months (
4.2 months) after surgery. Average
strated a borderline syrinx (2.3  3.0 mm) and the patient syrinx areas were 40.5 mm2 (
9.6 mm2) and 57.3 mm2 (
12.5
demonstrated predominantly cranial symptoms rather than syrinx- mm2) on the first and last postoperative scans, respectively; the
related symptoms (tinnitus, trouble with word finding, dizziness, latter measurement being significantly greater than the
occipital headaches that worsened with exertion, photophobia, preoperative area (P ¼ 0.02) (Figure 1A). Average changes in
vertigo, dysphagia, and balance instability). Intraoperatively, we syrinx area were 106.9% (
94.4%) and 186.0% (
107.4%) for
found a severely dysmorphic skull with inward compression at the the first and last postoperative scans, respectively, relative to the
foramen magnum, and based upon our preoperative discussion preoperative scan. The change in syrinx area on the latest
with the patient and the non-worrisome syrinx, we elected to defer postoperative scan was statistically significantly greater than that
a duraplasty. In both of these patients it is not clear that the syrinx on the first postoperative scan, indicating continuous growth
was clinically relevant preoperatively with respect to symptoms, (P ¼ 0.04, Figure 1B). All 7 patients had experienced an
but they met criteria for inclusion in our study. increase in syrinx size by the latest imaging study; 2 (patients 2
The arachnoid was opened in 2 cases; tonsillopexy was per- and 4), however, had interval reduction in syrinx area on the
formed in 1 case. C2 laminectomy, fusion, and dens resection first postoperative scan. Overall, the average duration of
were performed in 1 case. All patients tolerated the procedures postoperative follow-up was 13.9 months (
4.9 months); the
well, with no complications in the immediate postoperative average radiographic follow up (from the first available scan to the
period. latest available one) was 30.7 months (
8.7 months).
Postoperatively, patients were monitored via office visits and
imaging studies (as summarized in Table 5). The first
postoperative scan was done, on average, 3.2 months (
1.0 Case 1
A 10-year-old girl (patient 6) presented with a CM-1.5 diagnosis
after workup performed for frequent occipital headaches that
worsened with exertion. She also complained of daily episodes of
bilateral finger paresthesia and intermittent diplopia. A sleep
Table 2. List of Preoperative Symptoms with Relative
Postoperative Improvement
Symptom % Frequency (n) % Postoperative Improvement (n) Table 3. Operative Approach Summary for Patients Undergoing
Durotomy
Occipital headache 86 (6) 100 (6)
Intervention % Performed (n)
Paresthesia 57 (4) 75 (3)
Visual Deterioration 43 (30) 100 (3) Suboccipital craniectomy 100 (5)
Apnea 29 (2) 100 (2) C1 laminectomy 100 (5)
Balance 29 (2) 50 (1) Durotomy 100 (5)
Back/neck pain 29 (2) 0 Duraplasty 100 (5)
Memory 29 (2) 100 (2) Arachnoid opening 40 (2)
Asymptomatic 14 (1) Remains asymptomatic Tonsillopexy 20 (1)
Syncope 14 (1) 100 (1) Obex Exploration 20 (1)

456 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2020.01.148

 FROM THE ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
UMBERTO TOSI ET AL. WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION

develop symptoms. The patient was followed with imaging every

Table 4. Operative Approach Summary for Patients Who
Underwent a Bone-Only Decompression
Intervention
Suboccipital craniectomy
C1 laminectomy
C2 laminectomy, fusion, and dens resection
3 months. On the latest scan available (Figure 2E and F), the
syrinx was still present, extending from C3 to T11 and now
% Performed (n) measuring 11.9 mm x 8.9 mm (lateral  AP; area of 83.0 mm2).
Nevertheless, the patient remained entirely asymptomatic
100 (2) throughout the follow-up period, with complete resolution of
100 (2) preoperative sleep apnea as indicated on the most recent poly-
somnography (showing an obstructive apnea-hypoxia index of 2.7/
50 (1)
hour and a central apnea index of 8.6/hour). Further intervention
has not yet been offered.

study showed 651 episodes of central apnea, with an obstructive

apnea-hypoxia index of 12/hour and a central apnea index of 80/
hour with desaturation to 76% as determined by preoperative
polysomnography. Preoperative imaging (Figure 2A and B)
showed tonsillar descent to 25 mm below the foramen magnum.
Signal anomalies were observed in the cord, but no clear syrinx
could be identified. Given the severity of her apnea, a decision
was made to proceed with surgery.
Craniectomy and removal of the posterior arch of C1 were
performed, followed by a double-Y-shaped durotomy initiated at
the level of C1 and extending below the tonsils as determined by
ultrasound. Intra-arachnoid dissection demonstrated compressed
tonsils that were bruised with whitish tips. The brainstem was
clearly flattened and depressed where the tonsils were lying, and
thus a tonsillopexy was performed. This resulted in visualization
of improved CSF flow at the lateral aspects from the foramen of
Luschka around the brainstem and improved flow from the
midline. A pericranial graft was then obtained and sutured in
place in watertight fashion.
The postoperative course was uncomplicated, and the patient
was discharged on postoperative day 3. Three months after sur-
gery, MRI revealed the presence of an expansile cord syrinx
extending from C3 to T11 and measuring 10.7 mm  5.7 mm
(lateral  AP; area of 48.6 mm2) at its greatest width
(Figure 2C and D). The patient, however, reported complete
symptom resolution, and polysomnography also showed marked
improvement; thus, a decision was made to wait and observe
the syrinx radiographically, with a plan to intervene operatively
were the patient to experience deterioration of her condition or
Case 2
A 25-year-old woman (patient 3) presented after months of back
pain prompted a spinal MRI that revealed the presence of CM-1.5.
She reported daily occipital headaches that grew worse on exer-
tion, transient diplopia, and leg weakness that significantly
limited her activities of daily living. MRI revealed a 6-mm descent
of round-shaped cerebellar tonsils and a syrinx extending from C2
to T3, measuring at maximum 6.5 mm  2.1 mm (lateral  AP;
area of 10.8 mm2). Signal abnormalities consistent with myelo-
malacia were seen between T3 and T9. Given her significant
symptom burden and imaging findings, a decision was made to
operate.
Craniectomy and resection of the posterior arch of C1 and an
arachnoid-sparing autologous duraplasty were performed. The
postoperative course was uncomplicated. However, on the first
imaging study, performed 2.7 months following surgery, the syr-
inx was again appreciated and observed to have increased in
diameter, now measuring 7.0 mm  2.5 mm (lateral  AP; area of
13.7 mm2) at maximum. The syrinx was again noted to extend
from C2 to T3. The patient complained of persistent back pain but
noted that her intermittent diplopia and suboccipital headaches
had resolved since the surgery. It was decided to continue with
conservative management given the partial symptom resolution.
On MR images obtained 10.6 months after surgery, the syrinx was
again appreciated, now measuring 7.2 mm  3.0 mm (lateral 
AP; area of 16.4 mm2) at maximum width. On this occasion, she
still had resolution of headaches and visual disturbance but
continued to have mild back pain. It was unclear whether this was

Table 5. Summary of Postoperative Findings

First Postoperative Visit Latest Postoperative Visit
% Symptom
Distance from Syrinx Area (mm2) Syrinx Distance from Syrinx Area (mm2) Syrinx Postoperative Radiologic Resolution
ID Surgery (mo) (% change) Span Surgery (mo) (% change) Span Follow-up (mo) Follow-up (mo) (fraction)

1 7.2 45.4 (147) C5-T3 16.6 54.2 (195) C5-T3 24.7 26.7 100 (3/3)
2 0.03 3.4 (-25) C1-C3 3.6 10.9 (137) C1-C3 11.8 8.9 75 (3/4)
3 2.7 13.7 (26) C2-T1 10.6 16.4 (52) C2-T3 2.7 11.8 33 (1/3)
4 6.3 35.1 (-49) C4-T1 34.2 87.0 (26) C4-T1 34.4 38.2 N/A (0/0)
5 0.10 62.0 (17) C2-T10 21.9 59.0 (11) C2-T10 0.3 29.1 100 (1/1)
6 3.2 48.6 (n/a) C3-T11 18.4 83.0 (N/A) C3-T11 20.5 22.6 100 (4/4)
7 2.8 75.0 (565) C4-T4 2.8 90.8 (705) C4-T4 3.0 77.9 80 (4/5)

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UMBERTO TOSI ET AL.
A *
n.s.
100
n.s.
Syrinx Area (mm2)
80
60
40
20
0 0
p
O
t-o
-o
e-
st
os
Pr
po
tp
st
rs
te
Fi
La
Figure 1. Change in syrinx area. (A) Syrinx area as
calculated from the preoperative (pre-op) and
postoperative (post-op) magnetic resonance imaging
attributable to the syrinx, and she was first referred to rheuma-
tology, given a concomitant rise in inflammatory markers.
DISCUSSION
We have reviewed 7 cases involving CM patients in whom PFD
resulted in progressive postoperative syrinx enlargement, persis-
tent up to 34 months postoperatively. Various surgical approaches
were used: Some operations included a durotomy; in others, a
dural-sparing decompression was utilized. In 2 cases, the arach-
noid was explored, and in 1 case tonsillopexy was performed.
Irrespective of the variety of surgical nuances, all the patients
within this cohort demonstrated uncommon radiographic pro-
gression of syringomyelia following decompression. This is
consistent with the literature, where no single approach has been
demonstrated to result in syrinx reduction more than any other.19
All patients who had symptoms preoperatively, however, reported
significant symptom improvement and, in many cases, frank
resolution of most preoperative symptoms dictating our
conservative approach of continuous radiographic follow-up.
Surgical management of CM is often recommended within the
context of symptoms that can be directly attributed to posterior
fossa crowding or for those asymptomatic patients with large sy-
ringes.20,21 Surgical options are varied and include suboccipital
craniectomy potentially accompanied by resection of the
posterior arch of C1, often followed by durotomy. Some
advocate the use of duraplasty, although this remains
debated.22-24 A recent meta-analysis showed that patients with
syringomyelia are more likely to benefit from duraplasty, even
though it also found that this technique may be associated with
higher postoperative complication rates.25 Other studies have,
however, demonstrated the safety of duraplasty.26 Surgical
approaches may also include C2 laminectomy, intra-arachnoidal
exploration, and tonsillopexy.27-31
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WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION
B
800 *
% Area Change
600
400
200
p
t-o
t-o
os
os
tp
p
st
rs
te
Fi
La
scans. (B) Percent change in syrinx area from
preoperative scan to the first and latest postoperative
scan available. n.s., not significant.
With the exception of duraplasty, recently shown to improve
outcomes for patients with syringomyelia, it is a matter of debate
what approach is best at reducing syringomyelia.25 In this series, a
clear trend could not be identified because of variable surgical
nuances and limited cohort size. We did identify an unexpected
dissociation between radiologic and clinical outcomes, as most
patients in our series had complete symptom resolution, which
is consistent with what has been shown in other case series.27,32-35
Reflecting upon potential sources of the unresolved CSF flow
obstruction and residual or worsened syringomyelia, several pos-
sibilities exist. In a patient, despite surgical decompression,
physiologic reestablishment of a patent cisterna magna could not
be achieved—this could contribute, at least in part, to the
persistent syringomyelia. For those 2 patients in whom dura-
sparing procedures were performed, the decision to address
other pathology (as deemed a more immediate concern) rather
than the syrinx may have contributed to less optimal improve-
ments in CSF dynamics. In addition, even for those other 5 pa-
tients in whom duraplasty was performed, in only 1 was obex
exploration explicitly mentioned in the operative note. Whether to
explore the obex was decided intraoperatively based on the new
CSF flow dynamics observed after PFD, on a case-by-case basis. By
not exploring the obex, though, webs or adhesions may have been
missed. For those patients in whom the obex was not clearly
visualized and freed of possible arachnoid webbing, a reoperation
could involve such an inspection.36,37 Similarly, it was recently
shown that dural opening and duraplasty could achieve higher
syrinx resolution than a bone-only decompression.24,25 This
information suggests that surgeons may want to consider
expansile duraplasty and intra-arachnoidal exploration as part of
their routine surgical principles in patients with syringomyelia. It
is not possible to say that these steps are essential, as in fact the
majority of our patients had excellent outcomes with the same
variety of techniques, but increasing literature and data are
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UMBERTO TOSI ET AL.
Sagittal Axial
A B
Pre-op
C surgical intervention even in the absence of symptoms; there is
D evidence, however, that syrinx size can diminish without inter-
3 months
E F
18 months
Figure 2. Syrinx evolution over time (patient 6). (A and B) Preoperative
(pre-op) sagittal and axial cervical magnetic resonance (MR) images. No
syrinx is clearly visible. (C and D) Sagittal and axial MR images from first
postoperative scan (3 months post-op). (E and F) Sagittal and axial MR
images from latest post-operative scan. Arrows show the syrinx; red
dotted line shows level of axial image (18 months post-op).
supporting these as techniques that might maximally improve CSF
flow at the craniocervical junction and thus improve syrinx
reduction as close to 100% as possible.
Finally, it is also possible that even when maximal surgical
technique is applied, postoperative sequelae such as chemical
meningitis or subarachnoid hemorrhage from the dural edges could
result in enough absorptive compromise or focal blockage of the
obex that CSF dynamics are not improved, but in fact worsened.
This is difficult to assess without CSF profiles from these patients,
WORLD NEUROSURGERY 136: 454-461, APRIL 2020
FROM THE ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION
and none of our cohort had CSF sampling performed. Nevertheless,
a slow low-dose steroid taper may be considered as way to minimize
any inflammatory component to the postoperative course.
Surgical options are available for the 10%e40% of patients in
whom PFD fails to improve syringomyelia.16,18 Some authors have
advocated syringo-subarachnoid shunting. Such a procedure,
however, carries intrinsic risks and, despite short-term success at
reducing syrinx size, has a high long-term failure rate.17,38,39
Others have attributed syringomyelia persistence to impaired
CSF flow at the level of the foramen magnum or foramen of
Magendie and have suggested correcting this by performing a
second PFD to explore the subarachnoid space.6,18,37,40 It also
remains unclear whether it is necessary to reintervene in a
patient with demonstrable syrinx evolution without concomitant
symptom progression.
Syrinx presence has often been considered sufficient to warrant
vention.41 This evidence supports our current decision to observe
our symptomatic patients despite an absence of radiographic
improvement, even in cases where the syrinx occupied most of
the spinal cord space, as for some of our patients (e.g.,
Figure 2). Other studies have shown persistence of
symptomatology despite postoperative syrinx reduction,
prompting questioning of whether syrinx size should be
followed at all postoperatively in the absence of symptoms
clearly attributable to it.42,43 Given the significant improvement
in symptomatology observed in our cohort, we believe that a
conservative approach is warranted in patients in whom PFD
results in symptom improvement despite radiologic evidence of
failure to improve of syringomyelia, at least in the short term.
We suggest intraoperative nuances to minimize risk of failure
but recognize that the natural history of syringomyelia is still
not perfectly understood and symptoms must often dictate
decision making in these situations. Careful observation should
be considered, and reintervention may be warranted for patients
with symptom recurrence or progression in the setting of
persistent or worsening syringomyelia. If reoperation is
necessary, we recommend reexploration of the subarachnoid
space, followed by direct syrinx shunting only for recalcitrant
clinical or radiographic syrinxes.
CONCLUSIONS
We present the symptomatology, radiographic findings, and sur-
gical management of 7 patients in whom PFD with or without
durotomy or arachnoid exploration failed to reduce syringomyelia.
We observed a postoperative progressive increase in syrinx size
that did not correlate with symptoms, however—as most preop-
erative symptoms resolved postoperatively. We thus advocate for a
conservative approach with continuous radiologic follow-up, but
anticipate that longer-term follow-up will likely result in some
subset of these patients requiring further intervention.
ACKNOWLEDGMENTS
We acknowledge the contribution of Anne Stanford, ELS, who
provided professional editing services.
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UMBERTO TOSI ET AL.
REFERENCES
1. Tubbs RS, Iskandar BJ, Bartolucci AA, Oakes WJ.
A critical analysis of the Chiari 1.5 malformation.
J Neurosurg. 2004;101(2 Suppl):179-183.
2. Heiss JD, Jarvis K, Smith RK, et al. Origin of
syrinx fluid in syringomyelia: a physiological
study. Neurosurgery. 2019;84:457-468.
3. Heiss JD, Patronas N, DeVroom HL, et al. Eluci-
dating the pathophysiology of syringomyelia.
J Neurosurg. 1999;91:553-562.
4. Menezes AH. Chiari I malformations and hydro-
myelia—complications. Pediatr Neurosurg. 1991;17:
146-154.
5. Milhorat TH, Chou MW, Trinidad EM, et al.
Chiari I malformation redefined: clinical and
radiographic findings for 364 symptomatic pa-
tients. Neurosurgery. 1999;44:1005-1017.
6. Tubbs RS, Webb DB, Oakes WJ. Persistent sy-
ringomyelia following pediatric Chiari I decom-
pression: radiological and surgical findings.
J Neurosurg. 2004;100(5 Suppl Pediatrics):460-464.
7. Isu T, Iwasaki Y, Akino M, Abe H. Hydro-
syringomyelia associated with a Chiari I malfor-
mation in children and adolescents. Neurosurgery.
1990;26:591-596 [discussion 596-597].
8. Strahle J, Muraszko KM, Kapurch J, Bapuraj JR,
Garton HJ, Maher CO. Chiari malformation type I
and syrinx in children undergoing magnetic
resonance imaging. J Neurosurg Pediatr. 2011;8:
205-213.
9. Huebert HT, MacKinnon WB. Syringomyelia and
scoliosis. J Bone Joint Surg Br. 1969;51:338-343.
10. Krieger MD, Falkinstein Y, Bowen IE, Tolo VT,
McComb JG. Scoliosis and Chiari malformation
type I in children. J Neurosurg Pediatr. 2011;7:25-29.
11. Kahn EN, Muraszko KM, Maher CO. Prevalence of
Chiari I malformation and syringomyelia. Neuro-
surg Clin N Am. 2015;26:501-507.
12. Wetjen NM, Heiss JD, Oldfield EH. Time course of
syringomyelia resolution following decompression
of Chiari malformation Type I. J Neurosurg Pediatr.
2008;1:118-123.
13. Depreitere B, Van Calenbergh F, van Loon J,
Goffin J, Plets C. Posterior fossa decompression in
syringomyelia associated with a Chiari malforma-
tion: a retrospective analysis of 22 patients. Clin
Neurol Neurosurg. 2000;102:91-96.
14. Navarro R, Olavarria G, Seshadri R, Gonzales-
Portillo G, McLone DG, Tomita T. Surgical results
of posterior fossa decompression for patients with
Chiari I malformation. Childs Nerv Syst. 2004;20:
349-356.
15. Matsumoto T, Symon L. Surgical management of
syringomyelia—current results. Surg Neurol. 1989;
32:258-265.
460 www.SCIENCEDIRECT.com
FROM THE
WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION
16. Sgouros S, Williams B. A critical appraisal of
drainage in syringomyelia. J Neurosurg. 1995;82:
1-10.
17. Iwasaki Y, Hida K, Koyanagi I, Abe H. Reevalua-
tion of syringosubarachnoid shunt for syringo-
myelia with Chiari malformation. Neurosurgery.
2000;46:407-412 [discussion 412-403].
18. Heiss JD, Suffredini G, Smith R, et al. Patho-
physiology of persistent syringomyelia after
decompressive craniocervical surgery. Clinical
article. J Neurosurg Spine. 2010;13:729-742.
19. Park YS, Kim DS, Shim KW, Kim JH, Choi JU.
Factors contributing improvement of syringomy-
elia and surgical outcome in type I Chiari mal-
formation. Childs Nerv Syst. 2009;25:453-459.
20. Ito K, Yamada M, Horiuchi T, Hongo K. Appro-
priate surgical procedures for Chiari type 1 mal-
formation and associated syrinx based on
radiological characteristics of the craniovertebral
junction [e-pub ahead of print]. Neurosurg Rev.
2019. https://doi.org/10.1007/s10143-019-01079-3.
21. Fan T, Zhao H, Zhao X, Liang C, Wang Y, Gai Q.
Surgical management of Chiari I malformation
based on different cerebrospinal fluid flow pat-
terns at the cranial-vertebral junction. Neurosurg
Rev. 2017;40:663-670.
22. De Vlieger J, Dejaegher J, Van Calenbergh F.
Posterior fossa decompression for Chiari malfor-
mation type I: clinical and radiological presenta-
tion, outcome and complications in a
retrospective series of 105 procedures. Acta Neurol
Belg. 2019;119:245-252.
23. Gurbuz MS, Karaaslan N, Caliskan T, Unal E,
Berkman MZ. Comparison of the surgical results
for foramen magnum decompression with and
without duraplasty in Chiari malformation type 1.
Turkish Neurosurg. 2015;25:419-424.
24. Honeyman SI, Warr W. Posterior fossa decom-
pression with or without duraplasty in the treat-
ment of paediatric Chiari malformation type I: a
literature review and meta-analysis. Neurosurgery.
2019;84:E270.
25. Lin W, Duan G, Xie J, Shao J, Wang Z, Jiao B.
Comparison of results between posterior fossa
decompression with and without duraplasty for
the surgical treatment of Chiari malformation type
I: a systematic review and meta-analysis. World
Neurosurg. 2018;110:460-474.e465.
26. Hoffman CE, Souweidane MM. Cerebrospinal
fluid-related complications with autologous
duraplasty and arachnoid sparing in type I Chiari
malformation. Neurosurgery. 2008;62(3 Suppl 1):
156-160 [discussion 160-151].
27. Alfieri A, Pinna G. Long-term results after poste-
rior fossa decompression in syringomyelia with
adult Chiari Type I malformation. J Neurosurg Spine.
2012;17:381-387.
28. Kunert P, Janowski M, Zakrzewska A, Marchel A.
Comparision of results between two different
techniques of cranio-cervical decompression in
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2020.01.148
ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
patients with Chiari I malformation. Neurol Neu-
rochir Pol. 2009;43:337-345.
29. Pomeraniec IJ, Ksendzovsky A, Awad AJ, Fezeu F,
Jane JA Jr. Natural and surgical history of Chiari
malformation type I in the pediatric population.
J Neurosurg Pediatr. 2016;17:343-352.
30. Stanko KM, Lee YM, Rios J, et al. Improvement of
syrinx resolution after tonsillar cautery in pediatric
patients with Chiari Type I malformation.
J Neurosurg Pediatr. 2016;17:174-181.
31. Vidal CHF, Brainer-Lima AM, Valenca MM,
Farias RL. Chiari 1 malformation surgery:
comparing non-violation of the arachnoid versus
arachnoid opening and thermocoagulation of the
tonsils. World Neurosurg. 2019;121:e605-e613.
32. Galarza M, Martinez-Lage JF, Ham S, Sood S.
Cerebral anomalies and Chiari type 1 malforma-
tion. Pediatr Neurosurg. 2010;46:442-449.
33. Galarza M, Sood S, Ham S. Relevance of surgical
strategies for the management of pediatric Chiari
type I malformation. Childs Nerv Syst. 2007;23:
691-696.
34. Kumar A, Pruthi N, Devi BI, Gupta AK. Response
of syrinx associated with Chiari I malformation to
posterior fossa decompression with or without
duraplasty and correlation with functional
outcome: a prospective study of 22 patients.
J Neurosci Rural Pract. 2018;9:587-592.
35. Mottolese C, Szathmari A, Simon E, Rousselle C,
Ricci-Franchi AC, Hermier M. Treatment of Chiari
type I malformation in children: the experience of
Lyon. Neurol Sci. 2011;32(Suppl 3):S325-S330.
36. Di Lorenzo N, Palma L, Palatinsky E, Fortuna A.
"Conservative" cranio-cervical decompression in
the treatment of syringomyelia-Chiari I complex.
A prospective study of 20 adult cases. Spine (Phila
Pa 1976). 1995;20:2479-2483.
37. Naftel RP, Tubbs RS, Menendez JY,
Wellons JC 3rd, Pollack IF, Oakes WJ. Worsening
or development of syringomyelia following Chiari
I decompression: case report. J Neurosurg Pediatr.
2013;12:351-356.
38. El-Ghandour NM. Long-term outcome of surgical
management of adult Chiari I malformation.
Neurosurg Rev. 2012;35:537-546 [discussion 546-
537].
39. Raffa G, Priola SM, Abbritti RV, Scibilia A,
Merlo L, Germano A. Treatment of holocord
syringomyelia-Chiari complex by posterior fossa
decompression and a syringosubarachnoid shunt
in a single-stage single approach. Acta Neurochir
Suppl. 2019;125:133-138.
40. Sayal PP, Zafar A, Carroll TA. Syringomyelia sec-
ondary to "occult" dorsal arachnoid webs: report
of two cases with review of literature.
J Craniovertebr Junction Spine. 2016;7:101-104.
41. Ramnarayan R, Ganesh CVS, Kumar R. Sponta-
neous resolution of Chiari 1-associated
 FROM THE ANNALS OF WEILL CORNELL NEUROLOGICAL SURGERY
UMBERTO TOSI ET AL. WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION

syringomyelia: a report of two cases. Pediatr Neu-
rosurg. 2018;53:238-242.
42. Munshi I, Frim D, Stine-Reyes R, Weir BK,
Hekmatpanah J, Brown F. Effects of posterior
fossa decompression with and without duraplasty
on Chiari malformation-associated hydromyelia.
Neurosurgery. 2000;46:1384-1389 [discussion 1389-
1390].
43. Sakamoto H, Nishikawa M, Hakuba A, et al.
Expansive suboccipital cranioplasty for the
treatment of syringomyelia associated with Chiari
malformation. Acta Neurochir (Wien). 1999;141:
949-960 [discussion 960-961].
Conflict of interest statement: The authors declare 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 30 September 2019; accepted 15 January 2020
Citation: World Neurosurg. (2020) 136:454-461.
https://doi.org/10.1016/j.wneu.2020.01.148
Journal homepage: www.journals.elsevier.com/world-
neurosurgery
Available online: www.sciencedirect.com
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UMBERTO TOSI ET AL. WORSENING SYRINGOMYELIA IN CHIARI MALFORMATION

SUPPLEMENTARY DATA

Supplementary Table 1. Preoperative and Postoperative Syrinx

Lateral and Anteroposterior (AP) Dimensions
First Postoperative Latest
Preoperative Visit Postoperative Visit

Lateral AP Lateral AP Lateral AP

Patient (mm) (mm) (mm) (mm) (mm) (mm)

1 5.69 4.11 6.94 8.33 8.81 7.83

2 3.06 1.91 2.10 2 3.91 3.54
3 6.52 2.11 6.95 2.51 7.19 2.9
4 11 8 8.46 5.29 11.91 9.31
5 11.42 5.92 10.52 7.51 10.98 6.84
6 0 0 10.68 5.8 11.87 8.91
7 5.01 2.87 10.95 8.73 12.66 9.14

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