Syringomyelia pdf

Background and aims In syringomyelia there is a known Substance P SP overexpression, predominantly in the external laminae of the dorsal horn. Support Center Support Center. External link. Please review our privacy policy. Review the complications of syringomyelia. Summarize interprofessional team strategies for improving care coordination and communication to enhance outcomes for patients affected by syringomyelia.

Access free multiple choice questions on this topic. Syringomyelia, at its core, is a disorder of an abnormal cerebrospinal fluid CSF circulation. A syrinx is a fluid-filled cavity that anatomically lies within the spinal cord parenchyma or the central canal [1].

This entity is most frequently associated with a CM-I, [2] although other known causes include spinal cord tumor, trauma, and post-traumatic or infectious adhesive arachnoiditis. The discovery of syringomyelia is becoming more common because of the increased use of MRI in the routine evaluation of back and neck pain.

The natural history of patients with syringomyelia is variable and unpredictable punctuated with periods of stability and progression. Although professionals do not completely understand the natural history of syringomyelia, the clinical course progresses over months to years, with an early rapid deterioration that gradually slows down.

The etiology of syringomyelia includes conditions that alter the physiologic CSF circulation dynamics. In most cases, it is secondary to spinal subarachnoid space obstruction. Etiology includes. Epidemiological data on syringomyelia is limited. Some studies have found the prevalence of syringomyelia to be from 8. Familial aggregation, twin studies and known genetic syndromes associated with CM1 and syringomyelia together suggest a genetic component of transmission.

Authors have proposed various theories to explain the origin and progression of syringomyelia. Although the pathophysiology concerning the origin of syringomyelia remains an elusive Gordian knot, the pathophysiology underpinning progressive syrinx enlargement may have a common theme.

The basic pathology in syringomyelia is a progressively expanding cavity in the central spinal canal. This expanding CSF filled "syrinx" compresses the spinothalamic tract neurons decussating in the anterior white commissure. However, the posterior columns are spared as they are located distally. This results in loss of pain and temperature sensation with preserved touch and vibratory sense segmental dissociated sensory loss.

The upper limbs are preferentially involved in a "cape-like" distribution sensory loss predominantly in the shoulder area. Since syringomyelia is commonly associated with Chiari malformation type 1 CM1 , it is pertinent to look at clinical features directly related to Chiari Malformation 1. The presentation is highly variable. In most cases, patients complain of pain, muscle weakness and atrophy especially in hands and arms, temperature insensitivity in the upper limb, spasticity or stiffness in lower limbs, progressive scoliosis.

The clinical course progresses over months to years, with an early rapid deterioration that gradually slows. In patients with syringomyelia secondary to foramen magnum obstruction treated only a syringosubarachnoid or a thecoperitoneal shunt, it has been observed that the spasticity improves despite the worsening of other neurological functions.

This suggests the spasticity in the limbs is possibly due to the bulk of the syrinx rather than the compression of the brainstem at the level of the foramen magnum. MRI with and without contrast is the investigation of choice.

It delineates relevant anatomy and allows accurate visualization of the syrinx in both sagittal and axial planes. MRI easily reveals the location, size, and extent of the syrinx cavity, the degree of cerebellar tonsillar ectopia. MRI also helps to rule out cystic lesions or spinal tumors. Leptomeningeal enhancement indicates infection.

MRI can also reveal any arachnoid scarring. One can also study syrinx progression over months or years to document the natural history of syringomyelia.

These can analyze CSF hydrodynamics non-invasively. This scan is indicated where MRI cannot be used patients with metallic implants in the joints, cardiac pacemaker. Delayed CT scans can visualize dye leeched into the syrinx cavity. However, some authors have criticized CT myelography for having a low sensitivity in detecting CSF blockage.

Electromyography has no diagnostic value in syringomyelia, but it helps to rule out peripheral neuropathy causing paresthesias. The goal of treatment is to correct the underlying causative pathophysiology. All current treatment strategies are directed toward improving CSF flow dynamics. In patients with Chiari malformation 1, craniocervical decompression is the best way forward. Intraoperative ultrasonography can be employed to confirm the decompression of the tonsil and pulsatile flow of the cerebrospinal fluid around the craniovertebral junction.

The duration of sensory deficits best predicts the symptomatic improvement following surgery. Studies have shown that shorter duration of the preoperative symptoms has better outcomes. Early surgery minimizes deficits. In the patients with post-inflammatory arachnoid scarring and post-traumatic syringomyelia, the operative procedure is directed toward reconstituting spinal subarachnoid CSF flow by arachnoid scar membrane resection, microsurgical lysis of arachnoid adherences and dural reconstruction.

Shunts are indicated for idiopathic syringomyelia and patients that have not responded to other treatment. Most commonly used is syringosubarachnoid shunt SSAS. If this fails syringoperitoneal shunt SPS may be used. While there are studies that claim to have better outcomes with SSAS as compared to foramen magnum decompression, [32] this thought is not universally echoed.

Professionals still do not understand the natural history of syringomyelia; it is unpredictable and highly variable which makes its prognostication very difficult. Although prognosis depends on the etiology, the degree of neurological deficit and the site and size of the syrinx cavity, syrinx diameter of more than 5 mm and associated edema predict a rapid deterioration.

The rarity of the condition, variable natural history, and short follow-up make treatment results assessment difficult. Box Bethesda, MD Information also is available from the following organizations:. Bobby Jones Chiari and Syringomyelia Foundation Symptoms may include: pain progressive weakness in the arms and legs stiffness in the back, shoulders, neck, arms, or legs headaches loss of sensitivity to pain or hot and cold, especially in the hands numbness or tingling imbalance loss of bowel and bladder control problems with sexual function curvature of the spine scoliosis that may be the only symptom in children.

Symptoms typically begin between the ages of 25 and 40 years. People with congenital syringomyelia may also have hydrocephalus, a buildup of excess CSF in the brain with enlargement of connected cavities called ventricles. Straining or coughing can increase pressure within the head and brain, causing the person to develop headache or even lose consciousness. Some people may have a disorder called arachnoiditis—an inflammation of the arachnoid, one of the three membranes that surrounds the spinal cord.

Acquired syringomyelia also known as primary spinal syringomyelia or noncommunicating syringomyelia can be caused by spinal cord injury, meningitis an inflammation of brain and spinal cord membranes usually caused by an infection , arachnoiditis, tethered cord syndrome a condition that is present at birth that causes the spinal cord to abnormally attach to the tissues in the lower spine, limiting its movement , and a spinal cord tumor.

Treating the Chiari malformation. The main goal of Chiari surgery is to provide more space at the base of the skull and upper neck. This reduces pressure on the brain and spinal cord and restores the normal flow of CSF. Surgery can allow the syrinx to drain, sometimes becoming smaller or even disappearing entirely.

Symptoms may improve even if the syrinx remains the same size or is reduced only slightly. Individuals should get treated sooner rather than later since delaying treatment can cause irreversible spinal cord damage.

Syringomyelia can reoccur after surgery, making additional operations necessary. Preventing a syrinx from forming or expanding after an injury.

In the case of trauma-related syringomyelia, the primary strategy is to prevent a syrinx from developing or growing in the first place. By clearing and expanding the space around the spinal cord, the normal flow of CSF may be restored. Removing the obstruction. Note diffuse swelling of the spinal cord and thoracic cord c. Sagittal T1WI c. Axial T1WI at mid-C2 level shows syrinx dissection obtained at 8 years of age shows herniated cerebellar into the dorsal quadrant of the cord communicating with tonsils to the lower C2 vertebral level a.

Axial T1WI at the subarachnoid space d upper C2 level demonstrates displaced cervical cord by the Clinical Presentation Where the syrinx extends to the upper cervical spinal cord, the nucleus of the trigeminal nerve The clinical presentation of syringomyelia can be affected resulting in altered facial sensa- includes symptoms of spinal cord dysfunction tion. Weakness is typically asymmetrical and may such as motor weakness and sensory disturbance.

Axial T2WI at the lower cervi- Guillain Barre Syndrome in year-old girl, two months cal level demonstrates cystic cavity in the right anterior later after acute presentation. Sagittal T2WI shows ventral quadrant of the cord b. Axial T2WI at the upper thoracic T2 hyperintensity in the cervicothoracic cord associated level depicts bilateral anterior cavities in the cord c Fig.

Incidentally diagnosed CIM and downward by one vertebral level, presyrinx extends into syringomyelia in year-old boy following fall from the the thoracic cord to the lower T3 level b. One year later, bike. Sagittal T2WI shows large cervical syrinx and cord CSF spaces at the craniovertebral junction are restored, edema below the syrinx seen as T2 hyperintensity a.

Atrophy of the of light touch and proprioception. The classic cape-like distri- standing syringomyelia. Thompson example, Chiari-associated syringomyelia will cord. They combined the results of intraoperative often be accompanied by paroxysmal, Valsalva- ultrasonography with phase-contrast MRI to induced occipital headache or bulbar symptoms.

These pressure waves are generated in It is also important to recognize that syringo- the spinal subarachnoid spaces during such activ- myelia may be present in individuals who have ities as coughing, Valsalva, etc. These suggest Repetitive pressure waves through the sur- that the natural history of the incidental or asymp- rounding CSF subarachnoid space propagate tomatic syrinx may be more benign than hitherto the syrinx.

Foramen magnum decompression considered. Syringomyelia Pathophysiology Contrary to this mode is the intramedullary pressure theory for syringomyelia based on Prominence of the central canal hydromyelia is Bernoulli principle Greitz Normally, not uncommonly encountered in neonates and CSF pressure in the subarachnoid space and infants; indeed expansion of the central canal in intramedullary pressure are equal.

This the intravascular compartment into the spinal will commonly lead to neurosurgical referral; cord parenchyma thus initiating syrinx formation. According to this model, the syrinx ship. The pathophysiology of syringomyelia in Despite numerous publications and hypotheses, Chiari I malformation is likely different to that the pathogenesis of syringomyelia is not entirely associated with Chiari II malformation.

In CIM, clear. Presyrinx secondary to CIIM would require treatment of the state is explained by transient focal cord edema associated hydrocephalus by CSF diversion. Only due to distention of the extracellular space within when shunt surgery fails to control the syringo- the cord and impaired spinal cord microcirculation. However, ation or tethered spinal cord. Children with CIM older than 5 years of age more often have syringomyelia compared Pathological Features to younger.

The extent of tonsillar descent corre- lates poorly with development of a syrinx. Communicating type I and tonsils have more severe impairment on CSF noncommunicating type IIa syringes, like hydrodynamics at the foramen magnum and hydromyelia, should have an ependymal lining. The may show extensive ependymal disruption and most common location of syringomyelia in CIM paracentral dissections; the paracentral dissec- patients is the upper and middle cervical cord, tions are lined by glia.

Communicating with the below the lower tip of herniated tonsils. By contrast, the primary paren- of the spinal motor horns by the syrinx Figs. Syringomyelia should be consid- ered if patient with CIM lost pain and temperature The clinical symptoms are variable depending on sensation in the upper extremities, while light the location and extent of the syrinx, with contri- touch and proprioception remain preserved.

Patients may present with variable function are rare in syringomyelia patients with combination of the symptoms in the different CIM, only seen in the terminal stage of the spinal stages of syringomyelia.

Coronal T2WI with fat saturation obtained at 4. Syringomyelia may also develop in patients While surgery is generally indicated for the child with congenital scoliosis, e. Some cases simply represent a focal hydromyelia, In the rare cases of syringobulbia, when cervi- while others may be true syringes secondary to cal syrinx propagated into the medulla Figs.

Spinal X-ray CIM. Coronal T1WI shows cervical central canal hydromyelia b—e — CIM was diagnosed incidentally at 1 year of age, and left-sided syringomyelia a. Coronal T2 FLAIR b syringomyelia developed 5 years later, secondary scoliosis and axial T2WI c obtained at 9 years of age demonstrate developed by 10 years of age complex geometry of the cervical syrinx which is nerve and respiratory dysfunctions, bradycardia, secondary neurological deterioration including a dysphagia, hiccups, and drop attacks.

Syringomyelia associated with Chiari II loss in the hands when syrinx extends into the malformation should be suspected in cases of cervical cord. Syringomyelia in Spinal Cord Tethering Syringomyelia may occur in association with occult spinal dysraphism, for example, lumbosa- cral lipomas.

There is some evidence to suggest that this is a poor prognostic factor for the devel- opment of symptoms Wykes et al. While in infants less than 3 months spinal ultrasound can reliably diagnose occult spi- nal dysraphism Hughes et al. All forms of spinal dysraphism may be Fig. Sagittal T2WI shows myelia Fig. In summary, while MRI is an L1 vertebral body fracture with focal narrowing of the spinal canal and a syrinx at the T12 vertebral body level.

Courtesy Amit syndrome. Stenosis-related syringes most local obstruction of the spinal CSF circulation.