SYRINGOMYELIA

Introduction
 Syringomyelia is a rare disorder in which a cyst forms within your spinal
cord. As this fluid-filled cyst, or syrinx, expands and lengthens over
time, it compresses and damages part of your spinal cord from its
center outward.

 Damage to the spinal cord caused by a syrinx can cause symptoms like
progressive pain, stiffness, and weakness in the:
 Back
 Shoulders
 Arms
 Legs

http://www.healthline.com/health/syringomyelia
 Introduction
 People with the disorder might lose the ability to feel cold and pain
normally.

 Syringomyelia will cause symptoms and complications that worsen

as the syrinx expands.

 Treatment aims to relieve the pressure on your spinal cord. The

treatment your doctor suggests for you will depend on the cause of
your syringomyelia. Follow-up care after surgery is important
because syringomyelia can reoccur.

http://www.healthline.com/health/syringomyelia
Syringomyelia cyst (syrinx) in the spinal cord
 Pathways of CSF flow

N.S.J. Elliott , et al,. Syringomyelia: A review of the biomechanics.2013. Journal of Fluids and Structures 40 (2013) 1–24.
 Symptoms
 The pressure the syrinx puts on the spinal cord and the damage
that follows causes the symptoms of this disorder.

 The symptoms may include:

 A progressive weakness and pain in the back, shoulders, arms, or legs
 An inability to feel hot or cold
 A loss of pain sensation
 Difficulty walking
 Bowel and bladder function problems
 Facial pain and numbness
 Curvature of the spine, or scoliosis
 Symptoms
 Other signs and symptoms of syringomyelia may
include:
 Stiffness in your back, shoulders, arms and legs.

 Pain in your neck, arms and back.

 Bowel and bladder function problems.

 Muscle weakness and spasms in your legs.

 Spinal curvature (scoliosis).

http://www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/dxc-20267744
 Causes
 It's unclear exactly how and why syringomyelia happens.

 It develops, cerebrospinal fluid — the fluid that surrounds,

cushions and protects your brain and spinal cord — collects
within the spinal cord itself, forming a fluid-filled cyst (syrinx).

 Several conditions and diseases can lead to syringomyelia,

including:
 Chiari malformation, a condition in which brain tissue protrudes into your
spinal canal.

http://www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/dxc-20267744
 Causes
 Meningitis, an inflammation of the membranes surrounding
your brain and spinal cord.

 Spinal cord tumor, which may interfere with the normal

circulation of cerebrospinal fluid.

 Conditions present at birth, such as a tethered spinal cord, a

condition caused when tissue attached to your spinal cord
limits its movement.

 Spinal cord injury, which may cause symptoms months or

even years after the initial injury.
http://www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/dxc-20267744
 Types of syringomyelia
 The brain and spinal cord (central nervous system) are bathed in a
clear fluid known as cerebrospinal fluid.

 Some of the functions of cerebrospinal fluid include nourishing and

cushioning the central nervous system.

 The three broad categories of syringomyelia include:

 Congenital brain defects: Such as Arnold Chiari malformation. The

bottom part of the brain (cerebellum) lies in the upper part of the
neck instead of within the skull. This obstructs the flow of
cerebrospinal fluid.
http://www.aselme.com/aselme/aselme.nsf/63bc81c7570122b9c12570b60042c6da/57f408f5a083bcfcc12571020053e574/$FILE/Syringomyelia.pdf
 Types of syringomyelia
 Injury to the spinal cord:
 Including trauma, ruptured aneurysm, infection (such as
HIV), certain degenerative diseases or some brain tumours.
The cyst forms at the damage site.

 Idiopathic Syringomyelia: The cause is unknown.

http://www.aselme.com/aselme/aselme.nsf/63bc81c7570122b9c12570b60042c6da/57f408f5a083bcfcc12571020053e574/$FILE/Syringomyelia.pdf
 Formation of the cyst
 Cerebrospinal fluid normally circulates around the
brain and spinal cord.

 If the flow of cerebrospinal fluid is obstructed and

turned back on itself, a cyst (syrinx) may form.

 The cyst may be small at first, but the differences in

pressure between the skull and spinal cord gradually
force cerebrospinal fluid into the cyst.
http://www.aselme.com/aselme/aselme.nsf/63bc81c7570122b9c12570b60042c6da/57f408f5a083bcfcc12571020053e574/$FILE/Syringomyelia.pdf
 Formation of the cyst
 Over time, the cyst grows larger and presses on the spinal cord.
This can eventually damage the nerves.

 Syringomyelia usually progresses slowly over years, but

symptoms can sometimes advance rapidly after straining or
coughing.

 This is because the temporary increase in cerebrospinal

pressure forces fluid into the cyst.

http://www.aselme.com/aselme/aselme.nsf/63bc81c7570122b9c12570b60042c6da/57f408f5a083bcfcc12571020053e574/$FILE/Syringomyelia.pdf
 Etiology
 Etiology of syringomyelia often is associated with craniovertebral
junction abnormalities.

 Bony abnormalities include the following:

 Small posterior fossa.
 Platybasia and basilar invagination.
 Assimilation of the atlas.

 Soft-tissue masses of abnormal nature include the following:

 Tumors (eg, meningioma at foramen magnum).
 Inflammatory masses.
Etiology
 Neural tissue abnormalities include the following:
 Cerebellar tonsils and vermis herniation
 Chiari malformation

 Membranous abnormalities include the following:

 Arachnoid cysts, rhombic roof, or vascularized membranes
 Posthemorrhagic or postinflammatory membranes
 Etiology
 Other etiologies not associated with craniovertebral
abnormalities may include the following:

 Arachnoid scarring related to spinal trauma.

 Arachnoid scarring related to meningeal inflammation.
 Arachnoid scarring related to surgical trauma.
 Subarachnoid space stenosis due to spinal neoplasm or vascular
malformation.
 Subarachnoid space stenosis, with possible scarring, related to disk
and osteophytic disease.
 Idiopathic.
Diagrammatic representations of
pathophysiological theories in syringomyelia.

John L. S, et al,. The MR appearance of syringomyelia: new observations. AJR. 1987; 148: 381-391.
 Diagnosis:
 The symptoms of syringomyelia can mimic those of other
conditions, so diagnosis can be difficult.

 Some of the tests for syringomyelia include:

 Medical history.

 Physical examination.

 Magnetic resonance imaging (MRI).

Diagnosis:
 Computerised tomography (CT) scans.

 Electromyography (to test muscle strength).

 Tests of cerebrospinal fluid pressure.

 Lumbar puncture (to take a sample of cerebrospinal fluid).

 Myelogram (x-ray requiring injection of contrast dye into the

cerebrospinal fluid).
 Magnetic resonance imaging (MRI)
 MRI of spine and spinal cord is the most reliable tool for diagnosing
syringomyelia.

 MRI uses radio waves and a strong magnetic field to produce detailed images
of your spine and spinal cord.

 It can detect if a syrinx has developed within spinal cord.

 In some cases, dye is inject into a blood vessel in your groin, which travels
through blood vessels to your spine and reveals tumors or other
abnormalities.

 MRI may be repeated over time to monitor the progression of syringomyelia.

 MR images

T2-weighted saggital MR images showing syrinxes in association with (a) and (b) Chiari malformation, and (c) spinal injury.
N.S.J. Elliott , et al,. Syringomyelia: A review of the biomechanics.2013. Journal of Fluids and Structures 40 (2013) 1–24.
 46 years old women with
chiari I malformation and
syringomyelia.
 Note “beaded’ shape of syrinx
cavity. A, SE 500/40. Pointed’
tonsil below foramen magnum
(arrow).
 B, SE 3000/1 20. “Beaded”
shape is more noticeable on
this T2-weighted image.
 Note CFVS in cavity
(arrowhead). Increased
intensity present at rostral end
of syrinx may represent gliosis.
 42 year-old man.
Posttraumatic focal syrinx.
Fusion at C5-C6.
 A SE 600/30. Cyst enlarges
cord (arrows).
 B, SE 2500/60. Diffuse
increased intensity in syrinx
and cord.
Computerized tomography (CT) scan.

 CT scan uses a series of X-rays to create a detailed view of

your spine and spinal cord.

 CT scan may show if you have tumors or other spine

conditions.
 Prognosis
 Prognosis depends on the underlying cause, the magnitude of
neurological dysfunction, and the location and extension of the syrinx.

 Patients presenting with moderate or severe neurological deficits fare

much worse than those patients with mild deficits. Patients with central
cord syndrome have poor response to treatment.

 Natural history of syringomyelia still is not well understood. Although

older studies had suggested that mortality rates are likely lower in
today's patients as a result of surgical interventions and better
treatment of complications associated with significant paresis, such as
pulmonary embolism.

http://emedicine.medscape.com/article/1151685-overview#a7
Treatment
 Treatment for syringomyelia depends on the severity and
progression of your signs and symptoms.

 Monitoring:

 If syringomyelia is discovered on an MRI scan that's done for

an unrelated reason, and syringomyelia isn't causing signs or
symptoms, monitoring with periodic MRI and neurological
exams may be all that's needed.
Treatment
 Medications:
 Syringomyelia can’t be treated with drugs, but pain killers and muscle
relaxants may help to ease some of the symptoms

 Shunting:
 A thin tube is inserted into the cyst to drain the fluid into the abdominal
cavity.
 The shunt contains a one-way valve to prevent backflow. This type of
treatment is often used if hydrocephalus (build-up of cerebral fluid inside
the brain) is also present.
Surgery
 If syringomyelia is causing signs and symptoms that interfere with
your daily life, or if signs and symptoms rapidly worsen, surgery is
recommend.

 The goal of surgery is to remove the pressure the syrinx places on

spinal cord and to restore the normal flow of cerebrospinal fluid.

 This can help improve your symptoms and nervous system

(neurological) function. The type of surgery you'll need depends on
the underlying cause of syringomyelia.
Surgery
 Several types of surgery options are available to reduce pressure on
your brain and spinal cord.
 Surgery types include:

 Treating Chiari malformation:

 If syringomyelia is caused by Chiari malformation, then surgery that
involves enlarging the opening at the base of your skull (suboccipital
craniectomy) and expanding the covering of your brain (dura mater).
 This surgery can reduce pressure on your brain and spinal cord, restore
the normal flow of cerebrospinal fluid, and may improve or resolve
syringomyelia.
Surgery
 Draining the syrinx:

 Surgically insert a drainage system, called a shunt.

 It consists of a flexible tube that keeps fluid from the syrinx

flowing in the desired direction.

 One end of the tubing is placed in the syrinx, and the other is
placed in another area of your body such as your abdomen.
 Surgery
 Removing the obstruction.
 If something within your spinal cord, such as a tumor or a bony growth, is
hindering the normal flow of cerebrospinal fluid, surgically removing the
obstruction may restore the normal flow and allow fluid to drain from the syrinx.

 Correcting the abnormality.

 If a spinal abnormality is hindering the normal flow of cerebrospinal fluid,
surgery to correct it, such as releasing a tethered spinal cord, may restore
normal fluid flow and allow the syrinx to drain.

 Surgery doesn't always effectively restore the flow of cerebrospinal fluid,

and the syrinx may remain, despite efforts to drain the fluid from it.
Complications
 In some people, syringomyelia can become a progressive disorder
and lead to serious complications.

 Others may not have any symptoms, and no intervention is

necessary.

 Complications that may occur as a syrinx enlarges or if it damages

nerves within your spinal cord include:
 Scoliosis — an abnormal curve of your spine.
 Chronic pain — damage to the spinal cord can cause severe, chronic
pain.
 Motor difficulties — weakness and stiffness in your leg muscles can
eventually affect your gait.
 Follow-up care
 Follow-up care after surgery is critical because syringomyelia
may recur.

 Need regular examinations, including periodic MRIs, to assess

the outcome of surgery.

 The syrinx may grow over time, requiring additional treatment.

 Even after treatment, some signs and symptoms of

syringomyelia may remain, as a syrinx can cause permanent
spinal cord and nerve damage.
 Patient advice during follow up after surgery:
 Hospital Course: pain relief will be the most significant factor.
Therefore it is important the patient receives a lot of bed rest and
moves carefully.

 During the first month after surgery: when the operation is successful, the
pain and neurological symptoms should improve rapidly.

 The patient should try to maintain a modestly active lifestyle. It is important

that these patients try to avoid tasks involving heavy lifting! Walking and
performing daily activities at ease is highly recommended.

 On the long term: An adequate follow-up is very important for these

patients. If they do not deteriorate, they should try to maintain a relatively
active lifestyle.
http://www.physio-pedia.com/Syringomyelia
Patient advice during follow up after surgery:

 Avoid:

 Activities that cause excessive strain on the nervous system

and spinal cord.
 Any activity in which high impact can be expected (e.g.
football, rugby…).
 Avoid straining due to heavy bowel movement.
 Avoid extensive coughing.
 Most important: avoid heavy lifting and straining.

http://www.physio-pedia.com/Syringomyelia
Thank you