What Is Syringomyelia

• Syringomyelia is a rare neurological disorder characterised by the formation of fluid-filled cavities within the spinal cord. These cavities, known as syrinxes, can cause damage to the spinal cord tissues, leading to a variety of symptoms. Syringomyelia can be caused by various factors, including Chiari malformation, spinal cord injury, tumours, or other conditions that obstruct the normal flow of cerebrospinal fluid. The treatment for syringomyelia focuses on addressing the underlying cause and relieving symptoms to improve the patient's quality of life.
• Read on to explore a comprehensive overview of syringomyelia, including its causes, symptoms, diagnosis, management, and potential complications. Understanding this condition is crucial for both patients and healthcare professionals, as early recognition and appropriate treatment can significantly impact the prognosis and overall well-being of individuals affected by syringomyelia.

Overview

Syringomyelia is a neurological disorder characterised by the presence of fluid-filled cavities, known as syrinxes, within the spinal cord. These syrinxes can gradually expand and damage the spinal cord tissues, leading to a range of symptoms and complications. 

The cerebrospinal fluid (CSF), a fluid-like substance that surrounds and shields the brain and spinal cord, builds up within the spinal cord's tissue, expands the central canal, and eventually forms a syrinx in syringomyelia. A syrinx typically forms when the CSF flow normally surrounding the spinal cord or lower brain stem is obstructed. Syringobulbia is the medical term for when syrinxes impact the brain stem.^[1] Syringomyelia can occur as a primary condition or as a secondary condition associated with other underlying factors.

Causes of syringomyelia

There are a number of potential causes for syringomyelia, but Chiari malformation is the most common one. This aberrant disease causes brain tissue to protrude through the foramen magnum at the base of the skull and into the spinal canal, obstructing CSF flow. Syringomyelia may also result from spinal cord tumours, spinal cord traumas, or damage from inflammation in the area around the spinal cord. Idiopathic conditions occur when there is no identified reason.

Based on the causative factor, syringomyelia syringomyelia can be classified into: 

Congenital (Communicating) Syringomyelia: most frequently brought on by a Chiari malformation and the syrinx that results, typically in the cervical (neck) area of the spine. 

Acquired/ Primary/ Non communicating Syringomyelia: Causes include: 

• Spinal cord injury  
• Meningitis (inflammation of the surrounding membrane) 
• Spinal cord  tumours
• Arachnoiditis 
• Tethered cord syndrome  (fixation of the spinal cord at the  distal end) 
• Spinal dysraphism (spina bifida occulta, abnormalities of the spinal canal and its contents)^1,2

Signs and symptoms of syringomyelia

The signs and symptoms of syringomyelia can vary depending on the location and size of the syrinx within the spinal cord. Although abrupt onset is possible with coughing, stifling, or myelopathy, symptoms of the disorder typically appear gradually.

Common symptoms include:

• Paresthesia/hyperesthesia is the most frequent sensory sign, followed by non-radicular segmental pain. Segmental dissociated sensory loss which refers to the loss of pain and temperature sensation but the preservation of touch and vibratory sense. Due to their inability to feel pain, patients frequently suffer from hand injuries and chronic skin ulcerations.
• Progressive weakness and wasting of the muscles could develop into a "claw hand" malformation.⁵
• Headaches, which are typically felt at the back of the head, can be aggravated or brought on by sneezing, coughing, or bending over.  
• Stiffness
• Loss of balance
• Loss of bowel and bladder control
• Problems with sexual function
• Abnormal reflexes 
• Impaired Balance and coordination^,3

Management and treatment for syringomyelia

The management and treatment approach for syringomyelia depend on the underlying cause and the severity of symptoms. Even while some syringomyelia patients with no symptoms may not require treatment, it is still important to keep an eye on them to see whether their disease develops. Syringomyelia is treated according to the distinct symptoms that each patient presents. The coordinated efforts of a group of professionals may be necessary throughout treatment. Ophthalmologists, paediatricians, neurologists, neurosurgeons, surgeons, and other medical experts.¹ 

Painkillers (analgesics), physical therapy, and a reduction in activities, especially strenuous ones like heavy lifting, are all common therapeutic alternatives. Syringomyelia needs to be treated in order to restore the proper flow of cerebrospinal fluid and remove the pressure that the syrinx places on the spinal cord. The primary goal of treatment for syringomyelia is often to address its underlying aetiology.

In cases where syringomyelia is associated with Chiari malformation, surgical intervention may be necessary to decompress the spinal cord and restore the normal flow of cerebrospinal fluid. 

Surgery, such as the removal of a tumour, may be performed to treat other diseases that lead to syringomyelia. Surgery may be necessary to alleviate the tension on a tethered spinal cord. In some situations, a shunt or stent may be inserted into the syrinx. The shunt enables the syrinx's fluid to drain to a location outside the spinal column. 

Syringomyelia caused by trauma may be challenging to treat. For people who have neurological decline and/or unbearable pain, surgery is advised. Surgery aims to reduce fluid volume and to increase the region surrounding the spinal cord by the site of trauma or lesion.¹

Diagnosis of syringomyelia

Diagnosing syringomyelia involves a combination of clinical evaluation, imaging studies, and neurological assessments. Syringomyelia can occasionally be found by chance while a person is being examined for another cause. Magnetic resonance imaging (MRI) is the gold standard imaging modality used to visualise the spinal cord and identify the presence of syrinxes. Additional tests such as computed tomography (CT) scans and electromyography (EMG) may be performed to assess the extent of spinal cord damage and evaluate nerve function.

Another test, called a myelogram, which also uses contrast media or dye to obtain x-ray-like images, is similar to MRI and CT scans. This test is rarely required to detect syringomyelia since the invention of the MRI.⁴ 

Complications

If left untreated or inadequately managed, syringomyelia can lead to several complications. These include progressive neurological deterioration, permanent damage to the spinal cord, and the development of associated conditions such as hydrocephalus, infection, haemorrhage or scoliosis. The primary side effect of the disease process is myelopathy. Spasticity, which can develop into paraplegia or quadriplegia, decubitus ulcers, recurrent pneumonia, and bowel and bladder problems, can result from this. Regular monitoring and appropriate treatment can help prevent or minimize these complications.⁵ 

FAQs

Can syringomyelia be prevented?

Prevention of syringomyelia depends on addressing the underlying causes. Prompt diagnosis and treatment of conditions such as Chiari malformation or spinal cord injuries can help prevent the development of syringomyelia. 

How common is syringomyelia

According to some research, the prevalence of syringomyelia varies by ethnicity and location, ranging from 8.4/100,000 to 0.9/10,000.^10,11 Most of the patients are between the ages of 20 and 50 when they arrive.⁵

Who are at risk of syringomyelia

Syringomyelia can affect individuals of any age or gender. However, it is most commonly diagnosed in young adults aged 20 to 40 years. Patients with spinal surgery, spinal cord injury, and tumours are at risk of developing the condition. 

When should I see a doctor?

If you experience persistent or worsening symptoms such as unexplained pain, muscle weakness, sensory disturbances, or loss of bladder or bowel control, it is important to consult a healthcare professional for a thorough evaluation.

Summary

Syringomyelia is a complex neurological disorder characterised by the formation of fluid-filled cavities, known as syrinxes, within the spinal cord. It is a rare condition with an estimated prevalence of 8.4 cases per 100,000 people, and it can affect individuals of any age or gender, although it is most commonly diagnosed in young adults aged 20 to 40 years.

The condition can be caused by various factors, with Chiari malformation being the most common underlying cause. Other causes include spinal cord injuries, tumours, tethered cord syndrome, and congenital abnormalities. Syringomyelia can lead to a range of symptoms, such as progressive muscle weakness, sensory disturbances, pain, stiffness, and impaired coordination. In some cases, individuals may also experience headaches, loss of bladder or bowel control, and abnormal reflexes.

Managing syringomyelia involves addressing the underlying cause and relieving symptoms to improve the patient's quality of life. Treatment options may include surgical intervention to decompress the spinal cord, medications to manage pain and other symptoms, physical therapy to improve muscle strength and mobility, and the use of assistive devices to aid with daily activities.

Diagnosing syringomyelia typically involves a combination of clinical evaluation, imaging studies such as MRI, and neurological assessments. Timely diagnosis and appropriate treatment are crucial in preventing complications and minimising permanent damage to the spinal cord. Regular monitoring and follow-up care are necessary to ensure the best possible outcomes for individuals affected by syringomyelia.

If you suspect you may have syringomyelia or are experiencing symptoms associated with the condition; it is important to consult a healthcare professional for a thorough evaluation. Early recognition and intervention can significantly impact the prognosis and overall well-being of individuals affected by syringomyelia.