Foramen Magnum Decompression for Syringomyelia

Associated with Basilar Impression

and Chiari I Malformation
— Report of Three Cases—

Kanehisa KOHNO, Saburo SAKAKI, Hisashi NAKAMURA, Masaharu SAKOH,

Sadanori TAKEDA* and Kazuhiko SADAMOTO*

Department of Neurosurgery, Ehime University School of Medicine, Ehime;

*Department of Neurosurgery
, Washokai Sadamoto Hospital, Ehime

Abstract

Anterior or posterior decompression of the foramen magnum was performed in three patients with
syringomyelia associated with basilar impression and Chiari I malformation. The operative results
were evaluated using the pre- and postoperative magnetic resonance (MR) images. Two patients with
combined anterior and posterior cervicomedullary compression due to basilar impression and tonsillar
descent received suboccipital craniectomy, upper cervical laminectomy, and dural plasty without any
intradural manipulations via the posterior approach. One patient with prominent anterior cer
vicomedullary compression due to basilar impression and a sharp clivoaxial angle was operated on by
the transoral anterior approach. Postoperatively, all patients showed a sustained shrinkage of the
syrinx and rounding of the flattened cerebellar tonsils. Two patients showed upward movement of the
herniated tonsils. All patients had improved symptoms during 2-4 years follow-up. Treatment of
syringomyelia associated with basilar impression and Chiari I malformation requires more efficient
decompressive procedures at the foramen magnum based on neurological and MR findings.

Key words: syringomyelia, basilar impression, Chiari malformation, foramen magnum,

decompression surgery, magnetic resonance imaging

Introduction pathophysiological features of this anomaly.

Syringomyelia associated with Chiari I malforma
tion can easily be diagnosed by magnetic resonance
(MR) imaging. However, the treatment is still con
troversial. Foramen magnum decompression with or
without plugging of the obex is the most common
surgical treatment for patients with syringomyelia
Chiari I malformation complex.'-','-' 1,13-20,22-24)
A sub
jective improvement or arrest of their signs and symp
toms is achieved in more than half of the patients
treated .4,1,9-11,14-16,19,20,23)
However, long-term follow
up studies show that progressive neurological
deterioration occurs in nearly one-fifth of such pa
tients postoperatively.9,'4,15,'9,20) The unsatisfactory
results may be due to the diversity in clinical and
Received December 21, 1990; Accepted
1991
However, the main cause is considered to be inade
quate relief of compression of the cervicomedullary
junction caused by the bony structures around the
foramen magnum in some patients. In fact, nearly
one-fourth of patients with Chiari I malformation
have associated basilar impression9,'4,'9,20) and over
one-half have syringomyelia.3,5,7,'5) Therefore, sy
ringomyelia-Chiari I malformation complex is cer
tainly sometimes associated with basilar impression.
However, surgical treatment of anterior encroach
ment due to basilar impression and a sharp clivo
axial angle has not been much discussed in the
literature.
Here, we evaluate our operative results on three pa
tients with syringomyelia associated with basilar im
pression and Chiari I malformation, that is so-called
April 30, adult Chiari malformation without hydrocephalus or
meningocele. We also describe our current concepts
about indications for anterior or posterior foramen level in the enlarged spinal canal. The cervico
magnum decompression in patients with syringo medullary junction was compressed by both slight
myelia, basilar impression, and Chiari I malforma anterior encroachment due to basilar impression
tion with special reference to the MR findings. and by the herniated tonsils. The cervicomedullary
junction was laterally kinked due to atlantoaxial
Case Reports rotatory fixation (Fig. IA, B). Case 3 presented in
vagination of the dens 7 mm above McGregor's
The clinical features of our three cases are summa line and a 143' clivoaxial angle in the neutral posi
rized in Table 1. The diagnoses of syringomyelia and tion. The cerebellar tonsils were herniated to the C1
Chiari I malformation were based on MR imaging level. There was an oval-shaped syrinx in the poste
(0.5 T; Hitachi, Tokyo). rior spinal cord at the C 1 level and another long wide
syrinx below in the enlarged spinal canal (Fig. 2A).
I. Radiological investigations MR imaging and delayed computed myelotomog
We classified cervicomedullary compression into raphy found no connection between the fourth ven
three types using the preoperative MR images: 1) the tricle and the syrinx in any patient.
anterior type, prominent anterior encroachment due
to basilar impression and a sharp clivoaxial angle; 2) II. Operative procedures
the posterior type, isolated posterior compression The anterior or posterior approach to decompress
due to tonsillar descent; and 3) the combined type, the foramen magnum was determined by the direc
with both anterior and posterior compression. tion of greatest compression on the cervicomedullary
Anterior compression: Case 1 had severe anterior junction, based on MR images and clinical signs and
encroachment caused by invagination of the dens 15 symptoms. When the cervicomedullary junction was
mm above McGregor's line, a sharp 130° clivoaxial compressed bilaterally, the direction of greatest com
angle in the neutral position, and slight subluxation pression was chosen.
of the atlantoaxial joint with the atlanto-odontoid Case 1 (anterior compression) received transoral
distance ranging from 2 mm in extension to 6 mm in anterior decompression with fusion by removing the
flexion. Occipitalization of the atlas was also pres bony structures from the inferior part of the clivus to
ent. The cerebellar tonsils were herniated to the C2 the upper half of the axis vertebral body and wedg
level, and syringomyelia was present below the C2 ing an iliac bone graft into the gap.
level. This case was previously reported. 12) Cases 2 and 3 (combined type) demonstrated no
Posterior compression: This type is the common prominent anterior encroachment on MR images or
presentation of syringomyelia-Chiari I malforma signs of anterior compression. Thickening of the
tion complex, but not found in the present series. dura mater was observed in the foramen magnum,
Combined type: Case 2 presented invagination of but no thickening or adhesion of the arachnoid to
the dens 10 mm above McGregor's line, a 141' the cerebellar tonsils was present. These cases re
clivoaxial angle in the neutral position, assimilation ceived posterior decompression using suboccipital
of the atlas, scoliosis, and atlantoaxial rotatory fixa craniectomy and wide C1-C2 laminectomy. Dural
tion. The cerebellar tonsils had descended to the C2 plasty with autologous fascia lata was used to treat
level. There was marked syringomyelia below the C2 the thickened dura mater.

Table I Clinical summary for three patients with syringomyelia-Chiari I malformation complex
 Fig. 1 T,-weighted MR images in Case 2. A: Preoperative sagittal image shows flattened cerebellar ton
sils herniating to the C2 level, a large multiloculus syrinx, and slight cervicomedullary compression
caused by anterior encroachment. B: Preoperative coronal image clearly shows lateral kinking of
the cervicomedullary junction due to atlantoaxial rotatory fixation. C: Sagittal image 3 weeks
after surgery shows syrinx shrinkage, but the cervicomedullary junction is still compressed. D:
Sagittal image 1.5 years after surgery shows significant reduction of the syrinx, but the subarach
noid space at the foramen magnum is insufficiently widened.

Fig. 2 T,-weighted MR images in Case 3. A: Preoperative sagittal image shows the cerebellar tonsils her
niating to the CI level, an oval-shaped syrinx in the posterior spinal cord at the C1 level, and a
large syrinx below the oval one. B: Sagittal image 2 weeks after surgery shows reduction of the
syrinxes. C: Sagittal image 1 year after surgery shows further reduction of the syrinxes. The cis
terna magna is fully widened, but anterior compression by the hypertrophied dens and basilar im
pression still remains.

III. Operative results patients 3 months postoperatively. Case 1 demon

MR images revealed shrinkage of the syrinx in all strated complete reduction of the syrinx after ade
quate widening of the subarachnoid space around Therefore, adequate decompression at the foramen
the cervicomedullary junction at the foramen mag magnum should be carried out as early as possible
num. However, Cases 2 and 3 only achieved incom after clinical signs or symptoms manifest to achieve a
plete reduction of the syrinx because the subarach better outcome.
noid space was insufficiently widened in the anterior We consider intradural manipulations during
or posterior part of the cervicomedullary junction. posterior foramen magnum decompression unneces
Sequential MR images demonstrated that syrinx sary. Plugging of the obex based on the Gardner's
reduction began 2-3 weeks postoperatively and hydrodynamic theory"' has no purpose in patients
became maximum at 3 months. Thereafter, the with syringomyelia-Chiari I malformation complex
syrinx size did not change significantly. Cases 1 and 3 without hydrocephalus or a connection between the
showed upward movement and rounding of the her fourth ventricle and the syrinx. Intradural manipu
niated, flattened cerebellar tonsils clearly. Case 2 had lation does not achieve an improved outcome, 14)
insufficient decompression at the foramen magnum and has a higher incidence of complications.",")
due to associated anomalies, and showed only Our results show that syringomyelia associated
rounding of the cerebellar tonsils without upward with Chiari I malformation cannot be caused by the
movement (Figs. 1C, D and 2B, C). syrinx filling mechanism from the fourth ventricle
All patients demonstrated improved signs and via the central canal because no communication be
symptoms postoperatively. The outcome was ex tween the syrinx and the fourth ventricle was
cellent in one and good in two patients. No tem demonstrated radiologically.3, 15,") Therefore, cranio
porary or late neurological deficit developed during spinal pressure dissociation or disturbed cerebro
follow-up ranging from 2 to 4 years. spinal fluid flow between the head and the spine at
the foramen magnum is the main cause of syrinx
Discussion formation. A syrinx forms by cerebrospinal fluid
filtering through the spinal parenchyma, as suggest
We classified cervicomedullary compressions at the ed by Aboulker'' and Ball and Dayan.2'
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