Syringomyelia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Tarek Nafee, M.D. [2] Aysha Anwar, M.B.B.S[3] Eman Alademi, M.D.[4]

Overview

Syringomyelia is a spinal cord cavitation, which is a central dilation due to cystic degradation expands and destroyed the spinal cord. Caused by an injury, tumors or congenital malformation like hernia. The damage can Effect the brain and nerves, that leade to Bilateral loss of pain and temperature sensation in upper extremities. weakness, stiffness, hyperReflexives in lower extremities with hyposcoliosis. Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, on the location of the syrinx within the spinal cord.

Historical Perspective

  • In the 16th century, Estienne, a French anatomist, observed spinal cord cavitation for the first time.[1][2]
  • In 1545, Stephanus reported a case of spinal cord dilatation in one of his patients.[1][2]
  • In 1688, Brunner reported a liquor filled cavity with hydrocephalus in a pediatric case.[1][2]
  • In 1824, Olivier d'Angers coined the term syringomyelia.[1][2]
  • Also in 1824, Schuppel defined the fluid in the syrinx as a hydromyelia and proposed the pathogenesis as the persistence of the fetal central canal.[1][2]
  • In 1882, Otto von Kohler and Fredrich Schultz defined the clinical syndrome of syringomyelia.[1][2]
  • Between 1883 to 1891, Cleland and Hans Chiari associated syrinx formation with hyind brain herniation in what was subsequently named Chiari malformations types I to III. [1][2]
  • In 1959, Gardner described the waterhammer theory for pathogenesis of syringomyelia.[3]
  • In 1972, Ball and Dyan described the cavitation of the perivascular space as the source of syrinx formation.[4]
  • In 1980, Williams described the "suck and slosh" theory of syrinx formation.[5]
  • In 1994, Oldfield describes the piston effect of Chiari I malformation resulting in syringomyelia.[6]
  • In 2003, the Cisterna magna theory aimed to harmonize the Gardner, Williams, Oldfield and Ball&Dyan theories with neural imaging findings to propose an objective proposal of the pathogenesis of syringomyelia.[7]

Classification

Syringomyelia may be classified in various ways according to the anatomical features of the syrinx, or according to the underlying etiology of the disease.[8]

By Anatomy

Syringomyelia may be classified according to the anatomical description of the lesion (syrinx) as follows:[8]

  • Communicating vs. Non-communicating:
  • Intracannalicular vs. Extracannalicular:
    • Intracannalicular synringomyelia originates within the central canal of the spinal cord.
    • Extracannalicular syringomyelia originates within the spinal cord parenchyma.

By Etiology

Syringomyelia may be classified according to the underlying etiology or associated condition as follows:[9][10][11]

  • Congenital Malformations
  • Post-infectious
  • Post-traumatic
  • Post-inflammatory
  • Neoplastic

Pathophysiology

Pathogenesis

The exact pathogenesis of syringomyelia remains unknown. Several theories have been postulated about the development of a syrinx in the spinal cord. The underlying mechanisms of the disease involves disruption of CNS flow, subsequent formation of a syrinx, and enlargement of the lesion to impinge on the surrounding nerve fibers resulting in a symptomatic presentation.

1. Disruption of CSF Flow
Disruption of CSF flow may occur secondary to any of the following conditions:
  • Congenital craniocervical abnormalities obstructing CSF flow from the ventricles to the central canal (e.g., arnold chiari malformation, scoliosis)
  • Tumor or arachnoid cyst exhibiting a mass effect on the spinal cord
  • Sequelae of spinal cord trauma results in abnormal obstruction of the central canal
  • Sequelae of meningitis resulting in inflammation of the spinal cord meninges
  • Sequelae of inflammatory conditions such as sarcoidosis, multiple sclerosis, seronegative spondyloarthropathies
2. Syrinx formation
The pathogenesis of a syrinx is largely unknown. There are five primary theories of intra and extra cannilicular
  • Gardner's hydrodynamic theory: Blockage of the foramen of Magendie at the obex results in a pulsatile "water-hammer" effect of the mass lesion on the spinal cord CSF. This pressure differential results in extravasation of fluid into the perivascular and extracellular spaces.[7][12][3]
  • William's theory: Increased intracranial pressure combined with decreased subarachnoid spinal pressure results in a "vaccuum-like" effect which further herniates the cerebellar tonsils through the foramen magnum. This results in sloshing of the subarachnoid CSF and causes extravasation of the CSF into the spinal cord parenchyma, thus creating a syrinx.[7][5]
  • Ball & Dyan theory: With the CSF pressure difference created by the lesion, fluid moves, collects, and expands within Robin Virchow's perivascular space.[4]
  • Oldfield's theory: The pulsatile waves of the CSF which occur during systole result in increased ICP. This increased pressure herniates the cerebellum to obstruct the subarachnoid space at the level of the foramen magnum. This pulsatile fluid wave against the surface of the spinal cord results in extravasation of fluid into the parenchyma and creates a syrinx.[6]
  • Cisterna Magna theory: This theory claims that the fluid storage capacity of the cisterna magna is the primary shock absorber of the CNS that allows the spinal cord parenchyma to remain protected from increases in intracranial pressure. Mass effect of any lesion "stiffens" the cisterna magna and results in decreased compliance of the space and increased transferrance of pressure to the spinal cord. This results in extravasation of fluid to the spinal cord parenchyma.[7]
3. Development of symptoms
Once the syrinx enlarges enough to impinge on the surrounding spinal cord segments, symptoms related to the affected nerve fibers will develop due to the mass effect of the syrinx itself.

Genetics

There is no genetic predilection for the development of syringomyelia. However, Chiari malformations which are the primary cause of syringomyelia have demonstrated a strong familial and genomic association.[13][14][15][16][17][18]

Causes

The most common cause of syringomyelia is Chiari I malformation[19]. Other causes of syringomyelia may include:

Common Causes

Less Common Causes

Differentiating Syringomyelia from other diseases

Syringomyelia must be differentiated from other diseases which cause gait abnormalities, sensory and motor deficit, chronic pain syndromes, urinary and fecal incontinence, and cranial nerve palsies such as:[37][38][39][40][41]

Risk Factors

  The main risk factors associated with the development of syringomyelia are based on the underlying causes:

Screening

There is no recommended screening for syringomyelia.

Epidemiology and Demographics

Incidence

  • More than 25% of patients with spinal cord injury progress to develop syringomyelia.[47]

Prevalence

Syringomyelia has a prevalence estimated at 8.4 cases per 100,000 people,[48] or about 21,000 Americans, with symptoms usually beginning in young adulthood.

Natural History, Complications, and Prognosis

Natural History

The essential Course of disease in Syringomyelia is temperamental. Without treatment, syringomyelia may progress to develop the worsening of neurological deficit in cases of obstructive lesions. However, the disease has a slower progression in cases of mild disease.[49][50][51]

Complications

Complications that can develop as a result of syringomyelia are:[36][52][53][54][55][56][57][58][59][60][61]

Prognosis

The prognosis of syringomyelia varies depending on factors such as cause, extent of neurological damage, presence of complications, and general health status of patient before undergoing surgical treatment.[62][63][64]

  • Patients with mild neurological deficit may respond better to surgical management.
  • Early management of syringomyelia in cases of trauma is associated with decreased morbidity.
  • The symptoms of 80% of patients usually end due to their response to surgical treatment, as well a mild cure.
  • 20% of patients gets worse despite treatment in cases of advanced disease.
  • Recurrence is not uncommon in syringomyelia.

History and Symptoms

Specific areas of focus when obtaining the history such as onset, duration and progression of symptoms; history of trauma; associated conditions like multiple sclerosis, transverse myelitis, amyotrophic lateral sclerosis, sarcoidosis, congenital malformation, infection such as meningitis or neoplasia (ependymoma, meningioma, hemangioblastoma) may help diagnosing syringomyelia. Symptoms depends on the anatomical site of spinal cord involved.[65][66][67][68][69][70]

Anatomical site Early symptoms Late symptoms
Cervical
  • Inability to feel hot or cold
  • Decreased ability to feel pain sensation
  • Increased or decreased sweating
  • Muscle weakness ++
  • Shoulder joint pain, swelling and loss of function
  • Muscle paralysis
  • Loss of all sensations in hands, forearm, arm and upper back
  • Headaches with coughing or straining
Thoracic
  • Decreased to absent temperature sensation in trunk region
  • Abnormal pain sensation
  • Muscle weakness
  • Abnormal sweating
  • Muscle paralysis
  • Loss of pain and temperature sensation
Lumbar
  • Lower limb muscle weakness
  • Decreased to absent temperature sensation
  • Abnormal pain sensation
  • Joint sweling, pain and redness (charcot joints)
  • Loss of vibration, position sense and two point discrimination(recurrent falls)
  • Failure to maintain balance
  • Absent pain and temperature sensation
  • Muscle paralysis
  • Impaired ambulation
Sacral
  • Bladder or bladder dysfunction
  • Sexual dysfunction (erectile dysfunction)
  • Tingling and numbness in foot or anogenital region
  • Muscle weakness ++
  • Loss of penile erection
  • Loss of ankle plantar flexion(S1)
  • Urinary or fecal incontinence
  • Loss of temperature and pain sensation in foot and perineum

Physical Examination

The following includes how syringomyelia might be revealed :[71][72][73][74][75][76]

Anatomical site Physical examination
Syringobulbia
Cervical
  • Anhydrosis, miosis and ptosis (Horner's syndrome)
  • Loss of temperature and pain sensation in cervical dermatomes
  • Claw hand
  • Decreased muscle strength in hands, arms and shoulders
  • Decreased to absent deep tendon reflexes in upper limb
  • Muscle atrophy of hands, arms and shoulders
  • Tenderness, erythema, swelling and joint resorption (charcot joints)
Thoracic
  • Scoliosis
  • Decreased to absent pain and temperature sensation in thoracic dermatomes
  • Muscle atrophy ++
  • Absent superficial abdominal reflexes
Lumbar
Sacral

Imaging Findings

Imaging studies that may help diagnose syringomyelia, its cause and complications include:[77][78][79]

MRI

Spinal MRI may help to diagnose and follow up syringomyelia. It is characterized by the following findings:[77][80][81][82][83]

CT

Syringomyelia may be diagnosed as an incidental finding on CT scan. However, delayed CT scan may have a diagnostic importance in early cases of syringomyelia without clinical manifestaions.[84][85] Metrizimed CT scan using non-ionic contrast may be used to identify a syrinx in postinflammatory conditions (postinfectious and posthemorrhagic arachnoiditis).[86][87] In addition, computed axial tomography (CT) scans of a patient's head may reveal the presence of tumors and other abnormalities such as hydrocephalus.

X ray

There are no specific X ray findings associated with syringomyelia.

Myelogram

Myelogram is rarely used to diagnose syringomyelia. It uses a contrast material combined with x ray or CT to image spinal cord in case of syringomyelia. However, CT metrimised myelography is more sensitive to diagnose syringomyelia as compared to conventional myelography.[87][88]

Other Diagnostic Studies

Electromyography

EMG findings of syringomyelia include increased excitability of spinal motor neurons. However, the findings are non specific and does not hold diagnostic importance in case of syringomyelia.[89][90]

Lumbar Puncture

Role of lumbar puncture in syringomyelia is controversial since it may result in herniation in cases of Chiari malformation. However, it may be used by some clinicians as a diagnostic tool to look for CSF pressure and signs of inflammation based on individual patient assessment.[67]

Medical Therapy

Drugs have no curative value as a treatment for syringomyelia. The role of medical therapy in management of syringomyelia is for treatment of underlying conditions or management of associated symptoms.

Treatment of Underlying Conditions

Radiation and chemotherapy may be utilized for management of an underlying spinal cord tumor. Antibiotics may be used to treat an underlying infection (meningitis or encephalitis).

Management of Symptoms

In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.

Surgery

Surgery is not always required for syringomyelia. Indications for surgical management of syringomyelia include the following:[92][93]

  • Progressive neurological deterioration and worsening of symptoms
  • Presence of tumor causing obstruction
  • Congenital malformation(Chiari malformation)
  • Traumatic injury to spinal cord

Surgical options used to treat syringomyelia include:[94][95]

Posterior fossa decompression

  • Usually performed in Chiari malformation patients to expand the size of the posterior fossa and reverse tonsillar herniation resulting in relief of the obstructive lesion.[93][96]
  • This procedure may be combined with shunt, creating a large subarachanoid cistern to drain the syrinx or in conjunction with duroplasty in which dura is opened after bony removal of posterior occiput and posterior arch of C1 vertebra and a graft is sewed to dura effectively creating extra space in posterior fossa.[96][97]
  • Another procedure used along with posterior fossa decompression and duraplasty to help relieve obstruction is tonsillar resection or shrinkage.[98][99]

Shunt formation

Shunts are placed in order to drain the syrinx and alleviate symptoms such as headache and chronic pain syndromes. The choice of shunt is determined by the neurosurgeon; however, the following types of shunts may be performed:[97][100][101]

Ventriculoperitoneal shunt

  • Performed in patients with communicating syrinxes with hydrocephalus due to increased ventricular pressure.
  • This surgery is more commonly associated with Chiari II malformations

Syringosubarachnoid shunt

  • Performed more commonly in non-communicating syrinxes and is effective in rapidly reducing the size of the syrinx.
  • This shunt drains the syrinx into the subarachnoid space, and the CSF is subsequently reabsorbed by the arachnoid villi.

Syringoperitoneal shunt

  • Drainage of syrinx into the peritoneal cavity.

Removal of obstruction

  • In cases of tumors or arachnoid cysts as the underlying cause of the obstructive precursor lesion to syrinx formation, removal of said mass lesion may reverse the symptoms and reduce syrinx size.[102]

Surgical untethering and expansive duraplasty

  • Performed in post-traumatic syringomyelia.[67]
  • This procedure involves establishing access to the spinal cord at the level of the injury, laminectomy, lysis of adhesions, entry into spinal cord at the site of syrinx and drainage of the syrinx into said space.[67]
  • Duroplasty is usually performed following this procedure, for regional structural support.[67]

Neuroendoscopic surgery

  • Novel endoscopic procedure used for chiari malformation and syrinx drainage[103][104][105]
  • Minimally invasive
  • Fast recovery
  • Fewer complications

Prevention

Primary Prevention

There are no primary preventive measures associated with syringomyelia.

Secondary Prevention

References

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