From the Guidelines
Syringomyelia diagnosis and treatment should prioritize MRI imaging of the spine and brain to identify the underlying cause and guide surgical intervention, as this approach has been shown to improve outcomes in terms of morbidity, mortality, and quality of life. Syringomyelia is a disorder characterized by the formation of fluid-filled cavities (syrinxes) within the spinal cord, which can cause damage to the surrounding neural tissue and lead to progressive muscle weakness, pain, and loss of sensation. The most common cause is Chiari malformation, but it can also result from trauma, tumors, or infections 1.
Key Considerations
- MRI imaging is essential for evaluating the spinal cord and surrounding structures, and it has superior soft-tissue resolution and multiplanar capability, making it ideal for diagnosis 1.
- Intramedullary cord signal changes on MRI can represent prognostic factors for neurosurgical outcome, and IV contrast may be useful in certain cases, such as suspected spinal cord ischemia or inflammatory/infectious etiologies 1.
- Diffusion-weighted imaging can show signal alteration in the spinal cord earlier after symptom onset, and it should be included when considering spinal cord ischemia 1.
Treatment Approach
- Surgical intervention is the primary approach for treating syringomyelia, and it may involve decompression of the Chiari malformation, removal of obstructions to CSF flow, or direct drainage of the syrinx.
- Treatment should focus on addressing the underlying cause, and patients require long-term follow-up with regular MRI scans to monitor for recurrence or progression of the condition.
- Early diagnosis and intervention are crucial to prevent permanent neurological damage and improve outcomes in terms of morbidity, mortality, and quality of life.
From the Research
Definition and Diagnosis of Syringomyelia
- Syringomyelia is a condition characterized by the presence of a fluid-filled cavity or syrinx within the spinal cord 2
- Diagnosis is often made using MRI of the craniospinal junction, which can help identify the syrinx and any associated abnormalities such as Chiari malformation 3
Treatment Options for Syringomyelia
- Posterior fossa decompression is a common surgical treatment for syringomyelia associated with Chiari malformation, involving suboccipital craniectomy, removal of the posterior arc of C1, and duraplasty 3, 4
- The goal of surgery is to relieve compression on the spinal cord and improve CSF flow, which can help reduce the size of the syrinx and alleviate symptoms 5, 6
- Other treatment options may include syrinx shunting, although this is less commonly used as a first-line treatment 4
Outcomes and Complications of Surgery
- Studies have shown that posterior fossa decompression can be an effective treatment for syringomyelia, with improvement in symptoms and reduction in syrinx size in many patients 3, 6
- However, some patients may experience persistent or worsening syringomyelia despite surgery, and may require further treatment or management 5
- Complications of surgery can include CSF leakage, pseudomeningocele, and chemical meningitis, although these are relatively rare 4, 6
Predictors of Outcome and Prognosis
- Factors such as age and duration of symptoms may influence the outcome of surgery, with older patients and those with longer symptom duration potentially having a poorer prognosis 6
- Radiological predictors of outcome, such as the size and location of the syrinx, may also be important in determining the likelihood of successful treatment 5