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The Neuroradiology Journal 25: 593-597, 2012 www.centauro.it

SUMMARY Syringomyelia is a disorder in which a cyst or cavity forms within the spinal cord.
This cyst, called syrinx, can expand and elongate over time, destroying the spinal cord. We de-
scribe the case of a young patient with partial spontaneous regression of syringomyelia in Chiari
I malformation, confirmed by magnetic resonance imaging three years after the diagnosis. During
this period the patient did not experience any clinical symptoms. Although described in literature,
spontaneous regression is an unusual event and very few cases have been reported. This case report
supports the belief that conservative management together with both clinical and imaging periodic
controls should be preferred in stable mild-symptomatic patients.
Spontaneous Regression of Syringomyelia
in a Young Patient with Chiari Type I
Malformation
F. TORTORA
1
, M. NAPOLI
2
, F. CARANCI
3
, M. CIRILLO
1
, D. PEPE
1
, S. CIRILLO
1
, F. BRIGANTI
2
1
Department of Neurological Sciences, Neuroradiology Division, Second University of Naples; Naples, Italy
2
Unit of Interventional Neuroradiology, Department of Neurological Sciences, Federico II University; Naples, Italy
3
Department of Diagnostic Radiology and Radiotherapy, Federico II University; Naples, Italy
Key words: syringomyelia, Chiari I malformation, basion-opisthion line, cerebellar tonsillar ectopia
Case Report
A 24-year-old male student visited our hos-
pital with complaints of slight headache and
cervical pain. He described the headache like
a diffuse head pressure lasting about ten min-
utes, usually beginning in the back of the head,
worsened by coughing or laughing. He had suf-
fered for occasional headache for at least three
years, but during the last six to nine months
he noticed a mild reduction of his symptoms.
Generally he did not use drugs because his
symptoms did not trouble his study. He had no
history of trauma and the neurologic examina-
tion was negative for relevant signs. Despite
that, he was in follow-up for a Chiari Malfor-
mation-1 (CM-1) already diagnosed three years
before. Magnetic resonance (MR) examinations
were performed with a 1.5 Tesla (T) scanner
(Symphony, Siemens, Erlangen, Germany), ac-
quiring T2-weighted (T2-w) and T1-weighted
(T2-w) Turbo Spin Echo (TSE) scans on sagit-
tal planes.
The first MR study, when the patient was
21 year old, showed 0.8 cm cerebellar tonsils
herniation through the foramen magnum and a
cervical syrinx extending from the bulbo-med-
ullary junction to C2 with a maximum length
of 3 cm and a transverse diameter of 0.63 cm
(Figure 1A,2A). At that time because the symp-
tomatology was not significant the patient
declined the surgical decompression therapy
suggested. The current examination instead,
performed three years later, shows a partial
regression of the malformation: sagittal T1-w
TSE images demonstrate a partial rise up of
the tips of cerebellar tonsils placing at only
0.5 cm below the basion-opisthion (B-O) line,
almost at the limits considered borderline (Fig-
ure 1B). The sagittal T2-w TSE images also
show a marked reduction both of the maximum
length (2.2 cm) and transverse diameter (0.25
cm) (Figure 2B).
Discussion
Syringomyelia was first recognized as a dis-
ease process approximately 400 years ago but
the term syringomyelia or cystic cavitation of
the spinal cord was coined by Ollivier dAngers
in 1827 in a monograph entitled La Moelle Epi-
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Spontaneous Regression of Syringomyelia in a Young Patient with Chiari Type I Malformation F. Tortora
misalignment, spinal tumors, and spina bifida

2
. In 50%-75% of patients, siringomyelia results
from CM-1, a disorder of the paraaxial meso-
derm that leads to a maldevelopment of the
posterior cranial fossa with subsequent crowd-
ing and consequent translocation of normally
developed hindbrain structures into the spinal
canal
3-5
.
MRI plays a major role in the diagnosis be-
cause of the possibility of drawing an ideal
B-O line joining the mid-points of both ante-
rior and posterior margins of the foramen mag-
num to define the lower limit of the posterior
cranial fossa and serve as the reference line

6-8
for evaluating tonsillar ectopia. The extent
of tonsillar herniation is measured as the per-
pendicular distance from the tip of the tonsils
to the B-O line, considering cerebellar tonsils
situated no more than 3 mm below the B-O line
neire et Ses Maladies
1
. Syringomyelia is a dis-
order in which a cyst or cavity forms within the
spinal cord. This cyst, called syrinx, can expand
and elongate over time, destroying the spinal
cord.
Syringomyelia has a prevalence estimated
at eight cases per 100,000 people, with an in-
cidence of 0.3/100,000/year, no gender predi-
lection, with symptoms usually beginning in
young adulthood
2
.
Patients may experience pain, paralysis,
weakness, and stiffness in the extremities
3
.
Syrinx formation can result from the ob-
struction of the cerebrospinal fluid pulsation
in the subarachnoid space. A number of path-
ological conditions can cause an obstruction
of the normal cerebrospinal fluid pulsation.
These include Chiari malformation (CM), spi-
nal arachnoiditis, scoliosis, spinal vertebrae
A B
Figure 1 Sagittal T1-w TSE image (A) and T2-w TSE image (B) show marked tonsillar descent (0.8 cm below the B-O line) and
syringomyelia involving the spinal cord from the bulbo-medullary junction to C3 with a maximum length of 3 cm and a maximum
transverse diameter of 0.63 cm.
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www.centauro.it The Neuroradiology Journal 25: 593-597, 2012
trans-parenchymal fluid migration along the
Virchow-Robin spaces of the spinal cord in the
face of an obstruction at the foramen magnum
and suggested that such fluid then ruptured
secondarily into the remnants of the central
canal
13-14
. Aboulker, in his theory, states that
the obstruction of foramen magnum leads to
increased in-spinal pressure, forcing fluid into
the spinal cord through the Virchow-Robin
spaces or nerve roots. This condition favors the
rapid enlargment of the syrinx
13
. The evidence
of CSF dynamics, pressure studies, morphology
of the hindbrain structures, effect of surgical
intervention and recent MR imaging finding
of pre-syrinx state promote new insight into
the pathogenesis of the syrinx in Chiari type
1 malformations
15,16
. The latest evidence pro-
motes a new hypothesis of syrinx formation:
reduced compliance of the posterior spinal cord
non pathological, those 5 mm (or more) below
the limit significantly pathological, and those
between 3 and 5 mm borderline
9
. The exact
pathogenesis of syringomyelia associated with
CM-1 is unknown, although a number of au-
thors have reported their theories on syrinx
formation. Gardner was the first to explain
this correlation with his hydrodynamic theory
according to which syringomyelia is a result
of direct transmission of cerebrospinal fluid
(CSF) pulse through the obex because of a per-
sistent opening of the central canal of the obex
during embryonal development in the setting
of closed fourth ventricular outlet foramina
10-
12
. Subsequently, Williams proposed his theory
of cranial-spinal pressure dissociation, sup-
posing that the Chiari malformation works
like a valve obstruction at the foramen mag-
num
12
. Ball and Dayan proposed the theory of
Figure 2 Sagittal T1-w TSE image (A) and T2-w TSE images (B) show a partial regression of tonsillar descent to the limits with
normal values (0.57 cm below the B-O line), and a marked reduction both of the maximum length (2.2 cm) and the maximum
transverse diameter (0.25 cm).
A B
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Spontaneous Regression of Syringomyelia in a Young Patient with Chiari Type I Malformation F. Tortora
Untreated, the disorder worsens very slowly,
but it eventually causes severe disability. To
our knowledge, very few cases of spontaneous
resolution or regression of syringomyelia as-
sociated with Chiari malformation have been
reported
23-31
. In our case, the mechanism of
the spontaneous reduction of syringomyelia
is unknown. Various theories have also been
proposed to explain this phenomenon. Some
suggest that a decrease in the extent of hind-
brain herniation improves the flow of CSF
across the foramen magnum, allowing the res-
toration of normal physiology, and resulting
in the resolution of the syringomyelia
28-31
. This
theory certainly has some evidence from grow-
ing children, in whom improvement of Chiari
malformation has been attributed to growth of
the cranium and osseous spine relative to the
central nervous system
30,31
. The partial regres-
sion of the tonsils intracranially due to a grow-
ing cranium in a child or due to an atrophy de-
velopment in an adult may cause an improve-
ment in CSF circulation and syringomyelia
regression. In our case, there is no evidence of
cerebellar atrophy but only of an improvement
in CM-I. The evidence that syringomyelia can
spontaneously regress makes the treatment
strategy controversial.
A surgical procedure known as posterior fossa
decompression allows the cerebellar tonsils to
move into a normal position, restoring normal
CSF flow. After this procedure the syrinx will
often reduce or resolve on its own. Since the
outcome of surgical treatment for patients with
syringomyelia is not always satisfactory and
also some patients remain neurologically sta-
ble without surgery, clinical and radiological
follow-up may be adequate for patients without
progressive symptoms. Our case contributes
further to the growing body of evidence that the
natural history of Chiari type I malformation
associated with syringomyelia is not necessar-
ily towards progression. Asymptomatic children
with Chiari type I malformation and syringo-
myelia may benefit from conservative man-
agement with neurological and MRI follow-up.
Spontaneous reduction or resolution of syrin-
gomyelia is possible so the size of the yrinx alone
should not be considered an indication of sur-
gery. A conservative approach can be adopted
to monitor the progression of neurological dis-
abilities with periodic neurological examina-
tion and MRI measurement of syrinx which
has signal behavior equivalent to that of CSF.
veins, associated with the decreased spinal
CSF compliance due to the foramen magnum
blockade, will produce disturbed absorption of
the extracellular fluid through the intramedul-
lary venous channel and results in syringomy-
elia in Chiari type 1 malformation
17
.
MRI is currently the best imaging modality
available for diagnosing syringomyelia. Both
sagittal and axial T1-w TSE images should be
utilized because small syringes can escape de-
tection on sagittal cuts. They appear as low sig-
nal intensity cavitations inside the spinal cord,
and isointense to CSF. On T2-w TSE images
the syrinx has CSF signal and at its rostral
and/or caudal end a high signal intensity can
be observed which is attributed to microcystic
or gliotic changes induced by CSF pulsation.
They are more frequently located at the cervi-
cal and dorsal levels; in about 20% of the cases
the syrinx extends to the whole spinal cord
(holocord).
The spinal cord can be markedly distended
whereas the cavity can be collapsed. The ca-
nal is not always uniform in its extension; it
can have a lobular appearance of varying di-
ameter. These lobules can also display septa-
tions, which prevent communication of the
syrinx fluid throughout the cavity. It should be
noted that the syrinx can expand discontinu-
ously so that an apparent end of the syrinx
may be identified although it is in fact further
caudal. In these cases complete examination of
the spinal cord from cervical to lumbar region
is therefore essential
4,18,19
.
MRI can also be used to study the volume
of the posterior fossa and CSF flow dynamics

20-22
. Cine-mode magnetic resonance imaging
enables analysis of CSF dynamics in a car-
diac cycle in patients with Chiari I malforma-
tion, utilizing phase-contrast techniques 2D or
4D imaging. Some studies demonstrated CSF
movement as the displacement of the bands or
stripes. According to these MR studies, there
was a significant variety in the degree of sub-
arachnoid blockade and physiological param-
eter of the CSF flow in Chiari type 1 malfoma-
tion
22
.
MRI findings allow the radiologist to make
a differential diagnosis between syringomyelia
associated with Chiari type 1 malformation
and other diseases such as syringomyelia, hy-
dromyelia, ventriculus terminalis, myelomala-
cia, ependymoma, astrocytoma, hemangioblas-
toma according to several features.
597
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22 Deniz FE, Oksuz E. Spontaneous syringomyelia reso-
lution at an adult Chiari Type 1 malformation. Turk
Neurosurg. 2009; 19: 96-98.
23 Vaquero JS, Ferreira E, Parajn A. Spontaneous reso-
lution of syrinx: report of two cases in adults with Chi-
ari malformation. Neurol Sci. 2012; 33: 339-341.
24 Guillen A, Costa JM. Spontaneous resolution of a Chi-
ari I malformation associated syringomyelia in a child.
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25 Caranci F, Brunese L, Reginelli A, et al. Neck neoplas-
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detector computed tomography. Semin Ultrasound CT
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26 Sung WS, Chen YY, Dubey A, et al. Spontaneous re-
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27 Perrini P. Spontaneous resolution of syringomyelia in
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28 Klekamp J, Iaconetta G, Samii M. Spontaneous reso-
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29 Kyoshima K, Bogdanov EI. Spontaneous resolution of
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31 Sun PP, Harrop J, Sutton LN, et al. Complete spon-
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Fabio Tortora, MD
Department of Neurological Sciences
Neuroradiology Division
Second University of Naples
Viale Colli Aminei, 21
80131, Naples, Italy.
Tel.: +39 3397365573
E-mail: fabio.tortora@unina2.it
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