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Surgical results of arachnoid-preserving

posterior fossa decompression for Chiari I
malformation with associated syringomyelia
ARTICLE in JOURNAL OF CLINICAL NEUROSCIENCE APRIL 2012
Impact Factor: 1.38 DOI: 10.1016/j.jocn.2011.06.034 Source: PubMed

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Journal of Clinical Neuroscience 19 (2012) 557560

Contents lists available at SciVerse ScienceDirect

Journal of Clinical Neuroscience

journal homepage: www.elsevier.com/locate/jocn

Clinical Study

Surgical results of arachnoid-preserving posterior fossa decompression

for Chiari I malformation with associated syringomyelia
Hyun Seok Lee, Sun-Ho Lee , Eun Sang Kim, Jong-Soo Kim, Jung-Il Lee, Hyung Jin Shin, Whan Eoh
Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University, School of Medicine, 50 Ilwon-dong, Gangnam-gu, Seoul 135-710, South Korea

a r t i c l e

i n f o

Article history:
Received 2 May 2011
Accepted 26 June 2011
Keywords:
Arachnoid
Chiari I malformation
Posterior fossa decompression
Syringomyelia

a b s t r a c t
We analyzed the outcome of posterior fossa decompression accompanied by widening of the cisterna
magna, without disturbing the arachnoid, in patients with Chiari I malformation (CMI) associated with
syringomyelia. Twenty-ve adult patients with CMI and syringomyelia, who underwent surgery between
October 2000 and December 2008, were enrolled in this study. All patients underwent foramen magnum
decompression with C1 decompression, with or without C2 decompression. Three surgeons performed a
dura opening with duraplasty in 20 patients, and another surgeon excised the outer layer of the dura
without duraplasty in ve patients. Clinical and radiological assessments were performed preoperatively
and during the follow-up period. After surgery, 20 (80%) patients achieved a signicant improvement in
their clinical symptoms. However, four patients (16%) achieved only a stable state, and one patients
symptoms worsened. Radiological analysis showed that 17 patients (68%) had a favorable result; that
is, a total collapse, or a marked reduction, of the syrinx. Seven patients (28%) were stable in terms of syrinx size. However, the syrinx enlarged in one patient who had undergone excision of the outer dura.
Twenty-four patients achieved a widened cisterna magna with ascent of the cerebellar tonsils into the
posterior fossa and acquisition of a more rounded shape. Postoperative complications included a transient headache and vomiting in three patients and transient motor weakness in one patient. Two patients
developed a supercial wound infection. This study shows that arachnoid-preserving posterior fossa
decompression is a safe and effective treatment for patients with CMI with associated syringomyelia.
2011 Elsevier Ltd. All rights reserved.

1. Introduction
Chiari I malformation (CMI) is a cerebellar tonsillar herniation
through the foramen magnum associated with an abnormal posterior fossa constitution.14 This condition may result in syringomyelia due to an obstruction of the ow of cerebrospinal uid
(CSF) at the craniocervical junction.2,5,6 Although a number of surgical techniques have been described to restore the CSF circulation
and decompress the neuraxis, no consensus has been reached as to
the optimal technique.5,6 Usually, surgery consists of a posterior
decompressive craniectomy of the cervico-occipital junction associated with duraplasty, arachnoid opening and, sometimes, tonsillectomy. However, a number of surgical adjuvants to standard
bony decompression remain controversial. Furthermore, some disadvantages, such as a high rate of CSF stulas, aseptic meningitis,
and long hospitalization periods, have been reported in patients
who have undergone tonsillectomy and arachnoid dissection.79
To minimise the risks related to handling of the arachnoid
membrane and tonsillectomy, we performed decompressive

Corresponding author. Tel.: +82 2 3410 3491; fax: +82 2 3410 0048.
E-mail address: sobotta72@hotmail.com (S.-H. Lee).
0967-5868/$ - see front matter 2011 Elsevier Ltd. All rights reserved.
doi:10.1016/j.jocn.2011.06.034

craniectomy and arachnoid-preserving duraplasty consecutively

in adult patients with CMI and syringomyelia. This procedure resulted in decompression and shrinkage of the syringomyelia and
ascension of the cerebellar tonsils. We report the functional results
achieved by adult patients with symptomatic CMI and syringomyelia using arachnoid-preserving posterior fossa decompression and
show its efciency using radiography.

2. Materials and methods

2.1. Patient characteristics
Forty-three consecutive patients with CMI with associated
syringomyelia underwent arachnoid-preserving foramen magnum
decompression with excision of the outer dura or duraplasty at our
institution between October 2000 and December 2008. Medical
charts, radiographical features and outcomes determined by patient interviews were retrospectively assessed. Inclusion criteria
were: (i) a diagnosis of CMI with syringomyelia; (ii) an age
>18 years at the time of surgery; (iii) a minimum of two years of
postoperative follow-up; and (iv) at least one preoperative and
postoperative MRI. Patients with a tumor, a craniocervical bony

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H.S. Lee et al. / Journal of Clinical Neuroscience 19 (2012) 557560

anomaly, hydrocephalus, a history of meningitis, or history of previous posterior fossa decompression or shunt surgery were excluded. Twenty-ve patients who met the inclusion criteria were
enrolled in this study.
2.2. Surgical technique
Patients underwent surgery in the prone position with the head
xed in a Mayeld head holder. A midline linear skin incision was
made from the inion to C2 or C3, depending on the extent of cerebellar herniation. The suboccipital region below the inferior nuchal
line was carefully decompressed by removing the posterior lip of
the foramen magnum. After laminectomy of C1 and C2 (if necessary), the dural surface of the craniocervical junction was examined under an operative microscope and bands of thickened
tissue on the outer dura were carefully removed. A constricting
band of thickened tissue on the outer surface of the dura was removed at the craniospinal level. Three surgeons (ESK, JSK, HJS)
opened the dura and performed duraplasty (Supplementary video).
Duraplasty was performed using an autologous periosteum patch
or a Lyoplant (B. Braun, Melsungen AG, Melsungen, Germany). Care
was taken to ensure that the underlying arachnoid was not compromised. Osteodural decompression was considered appropriate
when we could observe, under a transparent arachnoid, good pulsation of the cerebellar tonsils and a free ow of CSF at the craniospinal level. Another surgeon (JIL) opened the outer layer of the
dura, without duraplasty, to widen the cisterna magna.
2.3. Clinical and radiological assessment
Clinical and radiological assessments were performed preoperatively and during follow-up by two neurosurgeons not involved
with the surgery. Preoperative assessments considered symptom
duration, neurological features, and syrinx levels and diameters.
Patients underwent a cerebrospinal MRI and a tonsilar herniation
evaluation. After surgery, neurological ndings and complications
were assessed. Direct examinations were performed one, three,
and six months after surgery, and subsequently at six-monthly
intervals. Patients underwent craniospinal MRI three months after
surgery, and this was repeated depending on the clinical evolution
and MRI ndings. MRI were used to determine the postoperative
size of the syrinx, widened cisterna magna volumes in the midsagittal plane, and the size of the CSF space surrounding the
medulla. Syrinx improvement was dened as a decrease in the
maximal syrinx diameter on MRI.

3. Results

3.2. Surgery and complications

Twenty patients (80%) underwent posterior cranial fossa
decompression, C1 laminectomy, and duraplasty. An additional
C2 laminectomy was required in ve patients (21%) who had
incomplete tonsillar decompression after C1 laminectomy. No severe adhesive arachnoiditis or subarachnoid scarring associated
with CMI was observed. Accidental pinholing of the arachnoid
membrane occurred during dural opening in three patients who
underwent duraplasty. In addition, a large accidental arachnoidal
rent occurred in one patient during a difcult opening of the dura
mater. Postoperative complications included a transient headache
and vomiting in three patients who underwent dural opening
and transient motor weakness occurred in one patient. Two patients experienced a supercial wound infection, but responded
to antibiotics. No complications occurred in the ve patients who
did not undergo dura opening.
3.3. Follow-up outcomes
After surgery, 20 of 25 patients (80%) showed signicant
improvement of their clinical symptoms. However, four patients
(16%) achieved only a stabilized state of clinical symptoms and
one patients symptoms worsened. Improvements were observed
in 18 (78.3%) of 23 patients with sensory disturbances, after surgery (Table 2). A suboccipital headache, when present, improved
in eight (88.9%) of nine patients, motor weakness improved in four
(44%) of nine patients and lower cranial nerve decits in two of
four patients (Fig. 1). Postoperative MRI showed that 17 of 25 patients (68%) achieved a favorable outcome; that is, syrinxes were
totally collapsed, or syrinxes or segments were remarkably reduced in diameter. Syrinx size stabilized in seven patients (28%).
Of the 20 patients who underwent duraplasty, 14 showed a decrease in syrinx volume and clinical improvement. Six patients
showed symptomatic improvement without syringomyelia
improvement. Of the ve patients who did not undergo duraplasty,
three had clinical improvement with a decrease in cavity size. One
patient had symptomatic improvement with no syringomyelia
improvement, and in one patient, who had no clinical improvement, the cavity size increased. A wider cisterna magna was detected in 24 patients and ascent of the cerebellar tonsils into the
posterior fossa with acquisition of a more rounded shape occurred
in 24 of 25 patients (Fig. 2).

Table 1
Demographic data of 25 patients who underwent extra-arachnoidal cranio-cervical
decompression for Chiari I malformation associated with syringomyelia
No. patients

3.1. Clinical and radiological ndings

The cohort was composed of 17 female and eight male patients
(mean age = 36.6 years, range = 1867 years). Follow-ups were
conducted over 24 to 76 months, and the duration of symptoms
ranged from two to 120 months. Sensory disturbances were the
presenting symptom in 23 patients (92%); eight patients (32%)
exhibited motor weakness, and eight patients (32%) had a suboccipital headache. Suboccipital headaches were exacerbated by valsalva maneuvers and sudden changes in posture. Lower cranial
nerve decits were observed in four patients (16%). One patient
had a basilar impression and KlippelFeil syndrome, which did
not interfere with the CMI assessment. The cerebellar tonsils were
positioned just below the foramen magnum in two patients, at the
C1 level in six, and at the C2 level in three. Preoperative clinical and
radiological features are summarized in Table 1.

Gender
Male
Female
Mean age (years) SD (range)
Clinical symptoms
Sensory disturbance
Motor weakness
Neck pain
Cranial nerve symptoms
Syrinx level
Cervical
Cervicothoracic
Entire cord
Type of operation
FMD + DO
FMD + DO + DP

8
17
36.6 14.2 (1867)
23
9
9
4
7
16
2
5
20

DO = opening of the outer layer of the dura, DP = duroplasty without arachnoid

opening, FMD = foramen magnum decompression, SD = standard deviation.

H.S. Lee et al. / Journal of Clinical Neuroscience 19 (2012) 557560

Table 2
Clinical and radiological outcomes of 25 patients who underwent extra-arachnoidal
craniocervical decompression for Chiari I malformation associated with
syringomyelia
Parameter
Clinical outcomes
Improved
Stable
Worse
Radiological outcomes
Improved
Stable
Worse
Complications
Transient headache
Transient weakness
Supercial wound infection

No. patients
20
4
1
17
7
1
3
1
2

Fig. 1. Graph showing the presenting signs and symptoms and follow-up outcomes
of 25 patients operated for Chiari I malformation with associated syringomyelia.

4. Discussion
The association between CMI and syringomyelia has been well
documented.2,7,1014 Recent studies have attempted to explain
CMI-associated syringomyelia on the basis of CSF ow obstruction
at the craniocervical junction.1,4,9,11,14,15 It is generally accepted
that the genesis of spinal cord cavitations can be attributed to a dynamic anomaly of CSF ow.2,4,15 The signs and symptoms observed
in patients with this condition are related to compression of neural

559

structures by the herniated tonsils and/or by the presence of an

associated syrinx. In patients with CMI associated with syringomyelia, surgical intervention is indicated in symptomatic patients
with neuroradiological abnormalities.12,15
Posterior fossa decompression directly relieves bony compression at the craniocervical junction. However, the usefulness and
safety of additional procedures such as duraplasty, tonsilar resection, syringosubarachnoid shunting, or obex plugging is debatable.5,6,16,17 Several previous studies have suggested that foramen
magnum decompression alone is not sufciently effective without
duraplasty.6,16 In the most recent survey conducted, the majority
of respondents favored a treatment combination of posterior fossa
craniectomy, C1 laminectomy and duraplasty, but no consensus
was reached regarding whether intradural exploration or tonsillar
manipulation was warranted. Some authors have reported that
opening the arachnoid does not guarantee improved effectiveness,
and that plugging the obex in addition to the arachnoid opening
does not have a positive effect.4,7,1820 It has also been reported
that complementary tonsilar resection does not offer substantial
benets compared to foramen magnum decompression with duraplasty; in addition, only complete opening of the dura achieved
slightly better results than a simple incision in the outer dura layer
alone.7,9,17,21 Some authors advocate that duraplasty is essential for
the prevention of scar formation and symptom recurrence.6,9,17
Debate exists regarding whether arachnoid dissection should be
performed once the dura is opened.5,17 Arachnoid dissection allows
direct observation of CSF pulsatile ow as it exits the fourth ventricle. Furthermore, the tonsils can be directly inspected and decisions
made regarding whether resection is required and if subarachnoid
adhesions need to be released. Conversely, opening the arachnoid
membrane probably incurs risks of CSF leakage and exposes the patient to the risk of symptomatic sterile meningitis, which can be
very debilitating and difcult to treat.8,9,14 Interestingly, Imae
et al., in a comparative study, described four patient groups who
underwent tonsillectomy with duraplasty, intra-arachnoidal lysis
with duraplasty, duraplasty alone, or delamination alone.8 No signicant differences were found between these groups with respect
to degree of syrinx reduction, but duraplasty was clearly less invasive than tonsillectomy or arachnoidal lysis.8,17,22
Thus, avoiding arachnoid opening achieves the same outcomes
with fewer complications. Furthermore, if duraplasty must be per-

Fig. 2. (a) Preoperative sagittal T1-weighted MRI of a 21-year-old women showing acquired tonsillar herniation and the syringomyelic cavity. (b) Sagittal T1-weighted MRI
one month after posterior fossa decompression and duraplasty showing marked shrinkage of the intramedullary cavity. (c) Sagittal T1-weighted MRI 12 months after surgery
showing a reduction in the size of the cavity and a normal intracranial cerebellar tonsil position.

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H.S. Lee et al. / Journal of Clinical Neuroscience 19 (2012) 557560

formed, it is strongly advisable to leave the arachnoid intact. In this

study, opening the CSF pathways did not require either resection or
coagulation of the cerebellar tonsils. Additionally, comparison of
pre- and postoperative T1-weighted MRI showed that expansion
of the CSF pathways and posterior fossa resulted in a normal cerebellar morphology with ascent of the cerebellar tonsils and acquisition of a rounded, rather than pointed, shape. The tendency of the
cerebellum to return to its normal morphology after surgical
enlargement of the posterior fossa supports the contention that
CMI is a secondary deformation that arises from a small posterior
fossa and not a primary abnormality of the cerebellum.
Our experience indicates that preservation of the arachnoidal
layer reduces the risk of postoperative complications, such as CSF
collection in the operative wound, hydrocephalus, aseptic meningitis, arachnoiditis, and pseudomeningocele. Recently, there have
been a small number of reports of infratentorial subdural extraarachnoid uid collection associated with attempted arachnoidpreserving duraplasty in patients with a pinhole arachnoidal injury.16,19 These studies suggest that arachnoidal injury may be
associated with non-communicating hydrocephalus, which is
potentially life threatening. Suggested management options include closing the pinhole in the arachnoid or opening the arachnoid widely and suturing it to the dura. The authors of these
reports also advocate routine thorough microscopic examination
of the arachnoid plane to identify any breaches or CSF leakage, in
patients for whom an arachnoid-sparing procedure is attempted.
The arachnoid-preserving procedure is challenging in patients
with CMI and extensive subarachnoid scarring at the craniospinal
level, although preservation of the arachnoid membrane, whenever
there is no evidence of obstruction of the foramen of Magendie
and/or arachnoiditis, decreases complications. In other words, for
patients with CMI with syringomyelia and/or hydrocephalus, the
arachnoid should be dissected whenever simple decompression
does not appear to be sufcient to re-establish CSF ow. It is difcult to draw conclusions based on a series of limited size, but this
study suggests that the use of duraplasty to treat CMI leads to a
greater decrease in concurrent syringomyelia. Further studies are
needed to better characterize patients with this condition and to
determine which patients with CMI are better treated with an
arachnoid-sparing procedure and those who require arachnoid dissection for syringomyelia resolution.
5. Conclusion
Foramen magnum decompression and arachnoid preservation
with duraplasty is a safe and effective treatment for patients with
CMI and associated syringomyelia. The procedure achieves a reduction in size, or collapse, of the syrinx in the majority of patients and
is associated with improvements in many neurological disturbances. Although our results are encouraging, a larger number of
patients and a longer follow-up are required to determine the efcacy of arachnoid-preserving foramen magnum decompression.

Appendix A. Supplementary data

Supplementary video data (surgical technique of posterior fossa
decompression using duraplasty for Chiari I malformation) associated with this article can be found, in the online version, at
doi:10.1016/j.jocn.2011.06.034.
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