Syringomyelia

Syringomyelia is a fluid-filled cavity, called a syrinx, inside the spinal cord. As it enlarges over years, it stretches and damages nerve fibres from within. It usually develops because the normal flow of cerebrospinal fluid around the cord is obstructed.

The commonest cause is a Chiari I malformation, in which the lowest part of the cerebellum sits in the opening at the base of the skull. Treatment targets the cause of the blockage, not the cavity itself. Surgery generally halts progression and may improve pain and strength, but symptoms that have been present for a long time often remain.

Symptoms

• Loss of pain and temperature sensation in the hands, arms or shoulders in a cape-like pattern, with painless burns or cuts
• Weakness and wasting of the hand muscles
• Burning or aching pain in the neck, shoulders and arms
• Headache at the back of the head on coughing or straining, with Chiari malformation
• Stiffness and weakness in the legs
• Scoliosis, especially in children
• Problems with sweating, the bladder or the bowel
• Difficulty swallowing or hoarseness if the cavity extends into the brainstem

Causes and risk factors

A syrinx forms when pulsating spinal fluid is forced into the cord because its normal pathway is narrowed. Causes include Chiari malformation, scarring of the membranes around the cord after meningitis, bleeding or surgery, spinal cord injury, where a syrinx can appear months or years later, tumours of the cord, and a tethered cord. Sometimes no cause is found. Small, slit-like cavities found by chance are often harmless remnants of the central canal and do not progress.

How it is diagnosed

• MRI of the whole spine and the brain: Shows the syrinx, its length and the junction between skull and spine. Contrast is given to exclude a tumour.
• Cine MRI flow study: Shows the blockage of fluid movement at the foramen magnum or along the cord.
• Neurological examination: Documents the pattern of sensory loss and weakness for comparison over time.
• Serial MRI: Small cavities without symptoms are rescanned, typically after 6 to 12 months and then less often.
• Standing spine X-rays: For scoliosis in children.

Treatment options

• Observation: For small cavities without symptoms and with no clear cause.
• Posterior fossa decompression: For Chiari-related syrinx. A small piece of bone is removed at the base of the skull and usually the covering membrane is widened with a patch. In most patients the syrinx then shrinks over months.
• Release of scar tissue with expansion of the dura: For cavities caused by scarring or previous injury.
• Tumour removal or untethering: When these are the cause.
• Syrinx shunt: A tube that drains the cavity into the space around the cord, the chest lining or the abdomen. It is kept for cases where the cause cannot be corrected, because shunts often block.
• Pain management and rehabilitation: Medicines for nerve pain, physiotherapy and protection of numb hands from injury.

When it is urgent

Rapid worsening of weakness, new difficulty with breathing or swallowing, loss of bladder control, or sudden severe headache with vomiting need urgent neurosurgical assessment at the nearest hospital.

Travelling to Türkiye for treatment

As the condition is uncommon, consulting a neurosurgeon who regularly treats Chiari malformation and syringomyelia is a reasonable motive for travel, both for an opinion on whether surgery is needed at all and for the operation itself. Decompression requires about 2 weeks on site, and flying is usually allowed 10 to 14 days after surgery. Follow-up MRI at 3 to 6 months and later can be done at home and shared. Avoid long journeys if your symptoms are deteriorating quickly.

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Frequently Asked Questions