What are the treatment options for syringomyelia?

From the Guidelines

Surgical intervention, specifically posterior fossa decompression, is the primary treatment approach for syringomyelia, particularly in cases associated with Chiari type 1 malformation, as it addresses the underlying cause of the condition. The decision to treat syringomyelia depends on the severity of symptoms and the presence of underlying conditions such as Chiari type 1 malformation, which is detected in 25–50% of children with X-linked hypophosphataemia by use of cranial MRI or CT 1. Symptoms of syringomyelia can include occipital or neck pain exacerbated by Valsalva manoeuvres, peripheral motor and/or sensory defects, clumsiness, hyporeflexia or hyperreflexia, respiratory irregularities, and central apnoeas and lower cranial nerve dysfunction 1.

When considering treatment options, the following approaches may be taken:

• Watchful waiting with regular MRI monitoring for asymptomatic patients or those with mild symptoms
• Surgical intervention, such as posterior fossa decompression, for patients with symptomatic Chiari type 1 malformation-associated syringomyelia
• Syrinx shunting procedures in cases where decompression is not appropriate
• Pain management using medications such as gabapentin, pregabalin, or amitriptyline
• Physical therapy to maintain function and prevent complications

It is essential to note that the goal of treatment is to stop the progression of the syrinx, alleviate symptoms, and prevent further neurological damage by addressing the underlying CSF flow abnormalities. Regular follow-up with neurology or neurosurgery is crucial to monitor disease progression, with MRI imaging typically performed annually or more frequently if symptoms worsen. Patients should avoid activities that increase intracranial pressure, such as heavy lifting or straining.

From the Research

Treatment Options for Syringomyelia

• Surgical procedures have been used for the treatment of syringomyelia, including foramen magnum decompression (FMD) and syringosubarachnoid (SS) shunting 2
• The choice of surgical procedure depends on the underlying cause of syringomyelia, with FMD preferred for patients with symptoms of Chiari I malformation and/or a small syrinx, and SS shunting preferred for patients with large syringes 2
• Syringosubarachnoid shunting is also recommended for syringomyelia associated with Chiari malformation, as it can effectively decompress the syrinx cavity and improve neurological symptoms 3, 4

Surgical Procedures and Outcomes

• Foramen magnum decompression has been shown to be effective in improving neurological symptoms and reducing syrinx size in patients with syringomyelia associated with Chiari malformation 2, 4
• Syringosubarachnoid shunting has been shown to be effective in improving neurological symptoms and reducing syrinx size in patients with syringomyelia associated with Chiari malformation, as well as in patients with post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis 3, 5
• Decompressive laminectomy and adhesiolysis have been shown to be effective in improving neurological symptoms and reducing syrinx size in patients with syringomyelia not associated with Chiari malformation 6

Factors Influencing Treatment Outcome

• The size of the syrinx and the presence of Chiari malformation are important factors in determining the choice of surgical procedure and the likelihood of a successful outcome 2, 3
• The presence of hydrocephalus or other underlying conditions, such as spinal adhesive arachnoiditis, can also influence the choice of surgical procedure and the likelihood of a successful outcome 3, 5
• The restoration of cerebrospinal fluid (CSF) flow dynamics between the syrinx and the subarachnoid space is an important factor in determining the success of surgical treatment for syringomyelia 6