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VOLUME 31 • NUMBER 20
October 15, 2009

A BIWEEKLY PUBLICATION FOR CLINICAL NEUROSURGICAL

CONTINUING MEDICAL EDUCATION

Different Types of Syringomyelia and Their Management:

Part I
Gerardo Guinto, MD, Miguel Abdo, MD, Norma Aréchiga, MD, and Erick Zepeda, MD
Learning Objectives: After participating in this activity, the neurosurgeon should be better able to:
1. Explain various types of syringomyelia and the possible pathophysiologic mechanism involved in their formation.
2. Describe radiologic findings and clinical manifestations of syringomyelia.
3. Describe when and how to treat a patient with syringomyelia.

This article is the first of two parts.
The term syringomyelia derives from the Greek “συριγξ”
(syrinx, meaning cavity) and “µυελοξ” (myelos, meaning
spinal cord); it refers to a chronic illness characterized by
the presence of a liquid-filled cavity within the spinal cord.
Under some circumstances, this cavity may reach the brain-
stem, in which case, it is called syringobulbia.
Syringomyelia is more a nosologic condition than a patho-
logic entity, as it has diverse causes and appears in varied forms.
Traditionally, two terms have been used to refer to a cavity
within the spinal cord: hydromyelia, when it is produced by a
widening or dilation of the ependymal canal within the spinal
cord, hence its walls are covered by ependymal cells; and
syringomyelia, when the cavity is formed independently from
the ependymal canal, so the cellular covering is absent and
may either communicate with the central canal or remain inde-
pendent. In clinical analyses as well as in imaging studies, it is
often very difficult to establish such a difference. Therefore,
syringomyelia generally is used to describe any cavity within
the spinal cord, with or without ependymal communication.

Classification and Etiology

Dr. Guinto is Professor and Chairman, Department of Neurosurgery,
Dr. Abdo is Associate Professor, Department of Neurosurgery, Dr.
Aréchiga is Associate Professor, Department of Neurology, and Dr.
Zepeda is Associate Professor, Department of Neurosurgery, Hos-
pital de Especialidades del Centro Médico Nacional Siglo XXI; mail
to: Hospital Angeles del Pedregal, Periferico Sur 3697-1025, Colo-
nia Héroes de Padierna, CP 10700, México City, México; E-mail:
gguinto@prodigy.net.mx.
The authors have disclosed that they have no significant rela-
tionships with or financial interests in any commercial organiza-
tions pertaining to this educational activity.
All staff in a position to control the content of this CME activity
have disclosed that they have no financial relationships with, or
financial interests in, any commercial companies pertaining to this
educational activity.
Lippincott CME Institute, Inc., has identified and resolved all faculty
and staff conflicts of interest regarding this educational activity.
On the basis of their pathologic features and their cor-
relation to imaging studies, cavities within spinal cord have
been classified into three types.
Communicating Syringomyelia
Communicating syringomyelia corresponds to approx-
imately 10% of cavities of the spinal cord and is character-
ized by a widening of the central canal, which anatomically
is communicated with the fourth ventricle. This variant is
caused by flow obstructions of cerebrospinal fluid (CSF) at
Category: Spine
Key Words: Chiari malformation, Hydromyelia, Pathophysiology, Syringob-
ulbia, Syringomyelia, Thermoalgesic dissociation, Tonsillar herniation

Lippincott Continuing Medical Education Institute, Inc., is accredited by the Accreditation Council for Continuing Medical Education
to provide continuing medical education for physicians.
Lippincott Continuing Medical Education Institute, Inc., designates this educational activity for a maximum of 1.5 AMA PRA Category 1 Credits™.
Physicians should only claim credit commensurate with the extent of their participation in the activity. The American Association of Neurological
Surgeons attests that this educational activity has been recognized for co-sponsored/endorsement for 1.5 Category 1 CME credits of the American
Association of Neurological Surgeon’s Continuing Education Award in Neurosurgery. This activity expires on October 14, 2010.
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 2

the level of the exiting foramina of the fourth ventricle by ependymal cells, which rarely burst or eccentrically dis-
(Luschka and Magendie). A typical finding is tetraventric- sect the spinal cord.
ular hydrocephalus, and in some cases, there is dilation of
the ependymal canal, which participates in this process as Noncommunicating Syringomyelia
the “fifth ventricle;” “pentaventricular” hydrocephalus then Noncommunicating syringomyelia is the most common
can be identified. There are several causes of this condition variant, representing 70% of cases. These cavities do not
such as meningitis, subarachnoid hemorrhage, and con- have any communication to the fourth ventricle and are sec-
genital malformations of the posterior fossa (e.g., type II ondary to obstruction of CSF flow at the level of or below
Chiari or Dandy-Walker malformations, encephaloceles). the foramen magnum. Most frequent causes of this kind of
As was mentioned previously, these cavities are covered cavitations are type I Chiari malformation, basilar invagi-
nation, spinal arachnoiditis,
extramedullary compres-
sions, tethered cord, acquired
tonsillar herniation, and
spinal or craniovertebral
junction tumors. On histo-
logic analysis, they appear
as isolated cavities, without
ependymal covering, and
tending to dissect the medu-
llar parenchyma, usually
with an eccentric pattern.
Primary Cavitation of
The Spinal Cord
Primary cavitation of the
spinal cord is dilation outside
the central canal, which orig-
inates in the parenchyma of
the spinal cord, with no com-
munication with the central
canal or the fourth ventricle. It
is associated with conditions
that damage the medullary tis-
sue such as trauma, infarct,
spontaneous hemorrhages,
A B and multiple sclerosis. On his-
tologic analysis, these lesions
Figure 1. Schematic representation showing normal anatomy of craniovertebral junction (A) and syringomyelia are surrounded by glial or
associated with type I Chiari malformation (B). In this malformation, there is a caudal herniation of cerebel- fibroglial tissue, and there are
lar tonsils through the foramen magnum, which blocks cerebrospinal fluid circulation to cause the syrinx. variable degrees of necrosis,

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2
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 3
A B
Figure 2. Sagittal MRI scans in a patient with type I Chiari malformation and a large cervical syringomyelia. The cavity appears hypointense
on a T1-weighted scan (A) and hyperintense on a T2-weighted scan (B).
neuronophagia, and wallerian degeneration. This variant must
be differentiated from two other forms of cavitation, which are
different from syringomyelia but must be included in the differ-
ential diagnosis. Atrophic cavities, or syringomyelia ex-vacuo, are
produced by loss of tissue due to a degeneration or primary atro-
phy of the spinal cord, which leads to development of micro cysts,
fissures, and dilations. In general, these cavities do not grow,
because there is no an established mechanism of progression.
Neoplastic cavities, which originate from cystic degeneration of
intramedullary tumors, mainly astrocytomas and ependymo-
mas, contain a proteinaceous fluid and may be surrounded by
the tumor itself or glial tissue. On rare occasions, intramedullary
tumors may present infarcts or internal hemorrhages (apoplexy)
that may degenerate into confluent cavitations.
Pathogenesis
Diverse pathologic entities participate in the genesis of
syringomyelic cavities. These have various mechanisms of
production and therefore must be considered individually.
Type I Chiari malformation
Type I Chiari malformation is the most common cause of
syringomyelia; spinal cord dilations have been reported in
60% to 70% of cases. The exact mechanism of formation is
controversial, and although there are many theories in this
regard, none explains the pathogenesis completely. The com-
mon factor is an alteration of CSF flow in the foramen mag-
num. CSF is produced in the choroid plexus of the cerebral
3
ventricles (predominantly lateral), from where it flows to the
subarachnoid space (SAS) through the orifices located on the
fourth ventricle. Within the SAS, CSF circulates from the basal
cisterns toward the convexity and the spinal region, and it is
finally absorbed on the arachnoid granulations of the supe-
rior sagittal sinus. The “forces” that maintain this circulation
are the pressure waves generated by systole on the vessels of
the central nervous system and the changes generated by res-
piratory movements. Pressures and flows on CSF are rapidly
and equally distributed to the entire SAS (spinal, basal, and
convexity); hence, any obstruction of this space may produce
gradients of pressure in specific zones. Three theories have
been proposed to explain pathogenesis of syringomyelia asso-
ciated with type I Chiari malformation.
Hydrodynamic Theory. Gardner suggested that delayed
and incomplete opening of the outlets foramina from the fourth
ventricle generate a communication between this cavity and
the central spinal cord through the obex. CSF pulsations act
like a “water-hammer” into the ependymal conduct, causing
development and progression of the cavitation.
Theory of Craniospinal Pressure Dissociation. Williams
developed the idea that partial obstruction caused by ton-
sillar herniation in the foramen magnum generates a block-
ade of flow of CSF from the cranial to the spinal SAS. During
routine daily activities, especially when Valsalva maneu-
vers are applied (coughing, sneezing, defecation, exercise,
vomiting, etc.), pressure is transmitted from the spinal
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 4
epidural veins to produce an ascending flow gradient of
CSF, which, because of the cuneiform shape of the cerebel-
lar tonsils, surpasses the obstruction at the foramen mag-
num to push the fluid in a cephalic direction (but not in a
caudal one). This effect generates a differential pressure in
favor of the cranial compartment, which pushes the CSF
from the fourth ventricle toward the medullary central canal,
thereby forming the cavity.
Piston Theory. Oldfield proposed that in patients with
type I Chiari malformation, cerebellar tonsils are pushed
down with each systolic beat, functioning as a “piston,”
which generates a wave of pressure in the SAS on the sur-
face of the spinal cord, forcing CSF to cross toward the cen-
ter of the medulla through the perivascular and interstitial
spaces (Virchow-Robin), forming the cavity.
Even with present day high-precision imaging studies, it is
difficult to determine the most important pathophysiologic fac-
tor in generation of these cavities. It is generally accepted that
there are many mechanisms involved, but common findings
in patients with type I Chiari malformations are cerebellar ton-
sillar herniation, arachnoid scars, and reactive gliosis that gen-
erates a chronic compression and consequent blockade of CSF
flow at the craniovertebral junction (Figure 1).
Syringomyelia Associated With Intramedullary Tumors
Syringomyelia is present in 25% to 58% of intra-
medullary tumors, mostly from the glial variety. The most
widely accepted mechanism in the formation of these cav-
ities is related to vascular alterations generated by tumor
growth that produces necrosis and/or hemorrhage, and
by the blockade that these tumors may cause on the per-
imedullary CSF.
Figure 3. Sagittal T1-weighted MRI scans showing a segmented syringomyelia. A narrow communi- perature of the upper extrem-
cation connects all cavities.
4
Syringomyelia Associated With Spinal Cord Injury
Syringomyelia associated with spinal cord injury is a
noncommunicating form that is present in 0.3% to 3% of
traumatic lesions of the spinal cord. It is mostly located in
the thoracic segment, progresses slowly, and may appear
months or years after injury.
There are two types of cavitations in the spinal cord
related to trauma.
Primary Cyst. As a result of an injury, spinal cord contu-
sion generates local blood effusion, medullar edema, and sub-
sequently liquefaction and myelomalacia, which, in some cases,
lead to formation of a cavity called a primary cyst. This usu-
ally is located at the same level of the initial impact, but it can
extend two or three levels upward or downward. This cyst is
not strictly considered syringomyelia, but rather a zone of irre-
versible medullary damage. Surgical management is useless.
Post-Traumatic Syringomyelia. There are two possible
theories trying to explain formation and growth of these cav-
ities. The “sucking” theory states that a severe direct impact
on the spinal cord causes inflammation and adherences on
the SAS, which generates a local obstruction of CSF flow. After
each Valsalva maneuver, intrathoracic and intra-abdominal
pressures are transmitted toward the spinal canal through
the venous plexus. In the presence of partial blockade of the
SAS, the CSF finds less resistance against passing through the
blockade in a cephalic direction, but not caudally, causing a
collapse in SAS below the blockade; this creates a suction—
or sucking—effect, favoring entry of CSF to the center of the
spinal cord through the perivascular spaces. However, this
theory explains only dilations distal to the injury, but not prox-
imally. In the second theory, syringomyelia is formed initially
by a coalescence of primary microcysts. CSF then flows toward
the syrinx more easily than in
the SAS due to the greater
resistance provided by the
arachnoid adherences and
blockages caused by the injury.
Clinical Course
Clinical manifestations of
syringomyelia depend essen-
tially on the specific localiza-
tion and dimensions of the
cavity. The syrinx is mostly
located at the inferior cervical
region, and initially it lies
inside the gray matter of the
posterior horn of the spinal
cord. Due to this location, in
most cases, thermoalgesic sen-
sitivity is selectively affected
from the beginning, as the first
synapse of the spinothalamic
pathway lies precisely at the
level of the head of the poste-
rior horn. Clinically, the
patient manifests an alteration
of sensitivity to pain and tem-
ities, whereas deep, vibratory,
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 5

In post-traumatic syr-
ingomyelia, clinical pre-
sentation is variable:
unexplainable appearance
of new symptoms, after a
period of stability in a
patient with spinal cord
injury, suggests formation
of a syringomyelic cavity.
The clinical course is gen-
erally characterized by
pain and sensitive motor
and autonomic symptoms,
which may be unilateral,
even when the syringo-
myelic cavity lies at the
center of the spinal cord.
Appearance of symptoms
below the paraplegic level
may be more difficult to
detect; in such cases, hyper-
hydrosis and spasms of the
legs could be signs of cau-
dal extension of the syrinx.
Manifestations from
the pathologic entities
A B related to syringomyelia
Figure 4. Sagittal T1-weighted (A) and T2-weighted (B) MRI scans in a patient with syringomyelia related
(e.g., cerebellar tonsillar
to two possible mechanisms: cerebellar tonsillar herniation and disk extrusion. herniation, alterations of
the craniovertebral junc-
tion, tumors) are usually
diverse, and sometimes
positional, and discriminative sensitivity remain relatively it is difficult to define which signs or symptoms are due to
preserved, which is denominated thermoalgesic dissocia- the medullary cavitation and which to the underlying con-
tion. The syrinx may extend upward to reach the medulla dition. Nevertheless, the clinician must establish the dif-
(syringobulbia), affecting the trigeminal nucleus and thus ference as precisely as possible, to inform the patient of
generating alterations in facial sensitivity. symptoms that could improve with management and those
In general, these are accompanied by other signs and that will remain unchanged.
symptoms of bulbar affection such as dysphagia, lingual
atrophy, vertigo, dizziness, nystagmus, and cerebellar signs. Diagnosis
The cavity in the spinal cord may also extend ventrally and MRI is the best study to confirm diagnosis of syringomyelia
affect the anterior horns, which manifests as atrophy, par- in any location. This method shows the cavity within the spinal
ticularly on the small muscles of the hand. When the cav- cord from its initial phases and allows evaluating its exten-
ity invades the lateral columns, a pyramidal syndrome sion in a longitudinal as well as in an axial direction. Due to
appears, characterized by debility and spasticity. Muscular the liquid content of the cavity, the image appears hypointense
reflexes on the upper extremities are characteristically dimin- on T1-weighted scans and hyperintense on T2-weighted scans
ished or abolished, yet they are exacerbated in the lower (Figure 2). Some cavitations may show segmentations, which
extremities. Clonus may be present and, frequently, the are incomplete septa, as they only rarely form truly indepen-
Babinski sign. Some patients have Horner syndrome due dent cavities (Figure 3). MRI is also useful to identify related
to a lesion of the sympathetic neurons of the intermediate- anomalies such as type I Chiari malformation, cysts of the pos-
lateral column on segments C8-T1. terior fossa, basilar impressions, tumors of the foramen mag-
Finally, in rare and advanced cases, there may be loss of num, extramedullary cysts, intramedullary cysts, arachnoiditis,
sphincter control. Pain is a frequent symptom and usually medullary contusion, transverse myelitis, cervical spondylo-
appears in initial phases, affecting the cervicolateral region, sis, degenerative disease of the intervertebral disk, and hydro-
the shoulder, and the upper extremity ipsilateral to the cav- cephalus, among others (Figure 4).
ity; it is often insidious, multisegmentary, and has a variable Dynamic MRI, or cine-MRI, allows observing the pattern of
evolution time. There may be accompanying anomalies of flow of CSF and may be useful in defining the possible mecha-
the musculoskeletal system such as scoliosis (frequently tho- nism related to the formation and growing of the syrinx. MRI is
racic) and neurogenic arthropathies (Charcot’s joint) of the useful in patients with intramedullary tumors, particularly in
shoulder, the elbow, and the fist. making the differential diagnosis between a cystic degeneration
5
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 6
A B
Figure 5. Sagittal MRI scans showing a cervical spinal cord tumor with cystic degen-
eration. The cystic content is located inside the tumor; it appears hypointense on a
T1-weighted scan (A) and becomes highly brilliant on a T2-weighted scan (B),
whereas adjacent spinal cord appears normal.
of the tumor itself and a real syringomyelic cavity. Cystic tumors
usually have high protein content, and so they appear more bril-
liant on T2-weighted scans (Figure 5), whereas syrinx appears
with a less-brilliant hyperintensity, located adjacent to the mass
(Figure 6).
Neurophysiologic studies, particularly somatosensory
evoked potentials, allow evaluation of the degree of affection
of neural conduction and aid in establishing prognosis. Other
imaging studies such as CT, myelography, and myelotomog-
raphy are requested less frequently and only in selected cases.
Treatment
Management of syringomyelia is controversial, mainly
due to variability of the clinical course, the association with
multiple entities, a confusing pathogenesis, and the diver-
sity of therapeutic options. The primary goal is stopping
progression of neurologic deficit, and whenever possible,
eliminating the cause of this entity. It is important to recall
that when signs or symptoms are clearly present, only a few
of them can be reversed completely. Several factors figure
in the outcome, including the patient’s condition and type
of syringomyelia.
Patients with a long-term neurologic deficit do not usu-
ally improve significantly with surgical treatment. The main
indications for operation in these patients are appearance
of new symptoms or presence of signs suggesting bulbar
6
involvement, due to the risk of affects on lower
cranial nerves, cardiac rate, or breathing. The
natural history of syringomyelia in asympto-
matic patients has not been well defined; there-
fore, treatment decisions in these patients are
difficult. Nevertheless, taking into account the
low morbidity of present-day surgical tech-
niques when applied by expert hands, as well
as the irreversible condition of the majority of
clinical manifestations once they are established,
most surgeons recommend early surgery.
The type of syringomyelia probably is the
most important factor to be considered, because
the main focus in management must be elimi-
nation of the precipitating cause, when this can
be determined. Selection of the procedure will
depend on the specific variant of syringomyelia
according to the following concepts.
Communicating Syringomyelia
This variant of syringomyelia is frequently
associated with hydrocephalus, thus the initial
procedure recommended is the placement of a
ventriculoperitoneal shunt. This is a simple
treatment, associated to a low morbidity, gen-
erally definitive and effective in almost all cases.
Recently, an endoscopic third ventriculostomy
has been proposed on selective cases, trying to
avoid the application of foreign materials.
Type I Chiari Malformation
Two surgical procedures are used most com-
monly to treat type I Chiari malformation: pos-
terior fossa decompression (PFD) and the
placement of a syringosubarachnoid shunt (SSS).
According to most authors, PFD is the best way to treat type I
Chiari malformation, with or without syringomyelia, because
this procedure solves the main pathophysiologic mechanism,
a small posterior fossa, by increasing its volume. SSS place-
ment is a relatively simple and effective procedure, but it is
focused on treating the effect rather than the cause of cavity
formation. It must be considered the first option in patients
with ample cavities and fast progression of medullary symp-
toms, as it achieves rapid reduction of the syrinx faster than
PFD (1.8 weeks vs. 6.3 weeks).
Spinal Cord-Compressing Lesions
If syringomyelia is related to extra-axial spinal cord com-
pression such as disc herniation, basilar impression, arach-
noid cyst, or tumor, simple elimination of this compression
improves symptoms and provides reduction of the syrinx. In
the few cases in which this not occur, SSS placement might be
indicated. In patients with intramedullary tumors, ideal treat-
ment consists of complete resection whenever is possible, but
if it is not, the surgeon must remove the tumor as completely
as possible, aspirate the cavity, and communicate it with SAS,
either directly or with the installation of a Silastic tube.
Spinal Cord Injury
The vast majority of post-traumatic syringomyelia is caused
by spinal arachnoiditis and the formation of arachnoid scars,
which cause blockade of CSF flow, or by mechanical obstruction
CNE20.qxd:Layout 1 9/15/09 9:41 AM Page 7
A B
Figure 6. Sagittal MRI scans showing a cystic spinal cord tumor associated
with syringomyelia. On a T1-weighted scan (A), tumor is heterogeneous, and Holly LT, Johnson JP, Masciopinto JE, et al. Treatment of posttraumatic
the adjacent spinal cord looks hypointense. On a T2-weighted scan (B), both
cystic component of the tumor and adjacent spinal cord appear hyperintense;
however, the tumor cyst is more brilliant due to its higher protein content. In Iskandar BJ, Hedlund GL, Grabb PA, et al. The resolution of syringo
this case, syrinx was located caudal to the tumor.
from bone fragments. The best response to surgical treatment is
among patients that present with localized blockade of CSF cir-
culation that can be clearly identified on imaging studies. In these
patients, surgery must be focused only on elimination of arach-
noid adherences and bone fragments. However, in many trau-
matic cases, it is not rare to find more severe and extensive forms
of arachnoid scars and spinal cord fixation; in that event, it becomes
preferable to make a CSF diversion procedure, which, although
it does not eliminate the cause, may improve signs and symp-
toms. In paraplegic patients with irreversible neurologic deficits
and severe arachnoid scars, it may be necessary to create a wide
communication between the syrinx and SAS by totally transect-
ing the spinal cord. Finally, in some patients, the SAS shows mul-
tiple blockages, and distant CSF shunts may be necessary:
syringocisternal, syringopleural, or syringoperitoneal. Some
authors suggest surgical creation of a pseudo-meningocele, which
helps to restitute the perimedullary CSF circulation, but the high
risk of CSF leakage and infection must be considered.
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1. Communication between the spinal cord cavity and the fourth 6. Pain is rarely a symptom of syringomyelia.
ventricle is found in the majority of patients with cervical
True or False?
syringomyelia.
7. The cystic portion of medullary tumors usually shows a more
True or False?
brilliant intensity than a syringomyelic cavity on T2-weighted
2. The most common cause of syringomyelia is spinal cord injury. MRI scans.
True or False? True or False?
3. Traumatic syringomyelia is most frequently found in the tho- 8. Surgical management of post-traumatic primary cyst is unnec-
racic region. essary and useless.
True or False? True or False?
4. Deep, vibratory, positional, and discriminative sensitivity remain 9. Posterior fossa decompression achieves faster reduction of
relatively preserved during the initial clinical phase of the syrinx diameter than does a syringosubarachnoid shunt.
syringomyelia.
True or False?
True or False?
10. Posterior fossa decompression is the most important surgi-
5. The presence of septations forming independent cavities is cal procedure directed to treat the main pathophysiologic fac-
a very common finding in patients with syringomyelia. tor of syringomyelia related to type I Chiari malformation.
True or False? True or False?