You are on page 1of 6


The Neuroradiology Journal 25: 593-597, 2012
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
Department of Neurological Sciences, Neuroradiology Division, Second University of Naples; Naples, Italy
Unit of Interventional Neuroradiology, Department of Neurological Sciences, Federico II University; Naples, Italy
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).
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 d’Angers
in 1827 in a monograph entitled La Moelle Epi-
Spontaneous Regression of Syringomyelia in a Young Patient with Chiari Type I Malformation F. Tortora
misalignment, spinal tumors, and spina bifida

. 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
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

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
. 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
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
Patients may experience pain, paralysis,
weakness, and stiffness in the extremities
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
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.
595 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
. 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
. 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
. 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”
. 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
. 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-
. 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).
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
. 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
. 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
. 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
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
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
MRI can also be used to study the volume
of the posterior fossa and CSF flow dynamics

. 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-
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 The Neuroradiology Journal 25: 593-597, 2012
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, Parajón 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.
Acta Neurochir (Wien). 2004; 146: 187-191.
25 Caranci F, Brunese L, Reginelli A, et al. Neck neoplas-
tic conditions in the emergency setting: role of multi-
detector computed tomography. Semin Ultrasound CT
MRI. 2012; 33: 443-448.
26 Sung WS, Chen YY, Dubey A, et al. Spontaneous re-
gression of syringomyelia – review of the current ae-
tiological theories and implications for surgery. J Clin
Neurosci. 2008; 15: 1185-1188.
27 Perrini P. Spontaneous resolution of syringomyelia in
an adult patient with tight cisterna magna. Neurol Sci.
2012 Jan 19. [Epub ahead of print].
28 Klekamp J, Iaconetta G, Samii M. Spontaneous reso-
lution of Chiari I malformation and syringomyelia:
case report and review of the literature. Neurosurgery.
2001; 48: 664-667.
29 Kyoshima K, Bogdanov EI. Spontaneous resolution of
syringomyelia: report of two cases and review of the
literature. Neurosurgery. 2003; 53: 762-769.
30 Avellino AM, Britz GW, McDowell JR, et al. Sponta-
neous resolution of a cervicothoracic syrinx in a child:
case report and review of the literature. Pediatr Neu-
rosurg. 1999; 30: 43-46.
31 Sun PP, Harrop J, Sutton LN, et al. Complete spon-
taneous resolution of childhood Chiari I malformation
and associated syringomyelia. Pediatrics. 2001; 107:
1 Gowers WR, Horsley V. A case of tumour of the spi-
nal cord. Removal; recovery. Med Chir Trans. 1888; 71:
2 Bergamasco B, Mutani R. Sindromi siringomieliche.
In: Neurologia di Bergamini. 2
ed. Turin: Edizioni li-
breria Cortina; 2010. p.601.
3 Oakes WJ. Chiari malformations, hydromyelia, syringo-
myelia. In: Wilkins RH, Rengachary SS, eds. Neurosur-
gery, Vol. 3. New York: McGraw-Hill; 1996. p. 3593-3616.
4 de Divitiis O, Elefante A. Historic background of spinal
disorders. World Neurosurg. 2012 Apr 28. [Epub ahead
of print].
5 Milhorat TH, Nishikawa M, Kula RW, et al. Mecha-
nism of cerebellar tonsil herniation in patients with
chiari malformations as guide to clinical management.
Acta Neurochir. 2010; 152: 1117-1127.
6 Forbes WSC, Isherwood I. Computed tomography in
syringomyelia and the associated Arnold-Chiari type I
malformation. Neuroradiology, 1978; 15: 73-78.
7 Worth DR, Milanese S. Chiari malformation type
1, presenting as scapulothoracic pain: A case report.
Manual Ther. 2012; 17: 172-174.
8 Cheng JC, Chau WW, Guo X, et al. Redefining the
magnetic resonance imaging reference level for the cer-
ebellar tonsil: a study of 170 adolescents with normal
versus idiopathic scoliosis. Spine (Phila Pa 1976). 2003;
28: 815-818.
9 Aboulezz A, Sartor K, Geyer C, et al. Position of cer-
ebellar tonsils in the normal population and in patients
with Chiari malformation: a quantitative approach
with MR imaging. J Comput Assist Tomogr. 1985; 9:
10 Gardner WJ. Hydrodynamic mechanism of syringomy-
elia: its relationship to myelocele. J Neurol Neurosurg
Psychiatry. 1965; 28: 247-259.
11 Gardner WJ, Angel J. The mechanisms of syringomy-
elia and its surgical correction. Clin Neurosurg. 1959;
6: 131-140.
12 Williams B. On the pathogenesis of syringomyelia: a
review. J R Soc Med 1980; 73: 798-806.
13 Aboulker J. La syringomyelie et les liquides intra-ra-
chidiens. Neurochirurgie. 1979; 25 (Suppl. 1): 1-144.
14 Elefante A, Peca C, Del Basso De Caro ML, et al.
Symptomatic spinal cord metastasis from cerebral oli-
godendroglioma. Neurol Sci. 2012; 33: 609-613. Epub
2011 Sep 17.
15 Fischbein NJ, Dillon WP, Cobbs C, et al. The ‘‘presyrinx’’
state: a reversible myelopathic condition that may pre-
cede syringomyelia. Am J Neuroradiol. 1999; 20: 7-20.
16 Koyanagi I, Houkin K. Pathogenesis of syringomyelia
associated with Chiari type 1 malformation: review of
evidences and proposal of a new hypothesis. Neurosurg
Rev. 2010; 33: 271-285.
17 de Divitiis O, Elefante A. Cervical spinal brucellosis:
a diagnostic and surgical challenge. World Neurosurg.
2012 Jan 28. [Epub ahead of print].
18 Jinkins JR, Sener RN. Idiopathic localized hydromy-
elia: dilatation of the central canal of the spinal cord of
probable congenital origin. J. Comput Assist Tomogr.
1999; 23: 351-335.
19 Pinto A, Caranci F, Romano L, et al. Learning from
errors in radiology: a comprehensive review. Semin Ul-
trasound, CT MRI. 2012; 33: 379-382.
20 Caldarelli M, Di Rocco C. Diagnosis of Chiari I mal-
formation and related syringomyelia: radiological and
neurophysiological studies. Childs Nerv Syst. 2004; 20:
21 Sakas DE, Korfias SI, Wayte SC, et al. Chiari malfor-
mation: CSF flow dynamics in the craniocervical junc-
tion and syrinx. Acta Neurochir. 2005; 147: 1223-1233.
Fabio Tortora, MD
Department of Neurological Sciences
Neuroradiology Division
Second University of Naples
Viale Colli Aminei, 21
80131, Naples, Italy.
Tel.: +39 3397365573
Copyright of Neuroradiology Journal is the property of Centauro srl and its content may not be copied or
emailed to multiple sites or posted to a listserv without the copyright holder's express written permission.
However, users may print, download, or email articles for individual use.