Syringomyelia: symptoms, causes and treatment

Symptoms depend on where in the spinal cord the cavity sits and how far it extends. Contact says the cavity can extend over a variable length and that symptoms reflect its level in the cord. Sometimes there are no symptoms at all, but often it affects sensation and eventually movement.

Leeds Teaching Hospitals says a growing syrinx can damage the centre of the spinal cord, causing pain, weakness and stiffness in the back, shoulders, arms or legs. Other symptoms can include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Some people also have severe arm and neck pain.

The NHS, writing about people who have a Chiari malformation, says that if you develop syringomyelia you may experience problems using your hands, difficulty walking, pain, and problems with bladder or bowel control.

Leeds Teaching Hospitals adds that curvature of the spine, such as scoliosis (a bending to the left or right) or kyphosis (a forward bending), is common among people with syringomyelia or a Chiari I malformation. It is seen most often in children, whose skeleton has not fully matured.

• Pain, weakness and stiffness in the back, shoulders, arms or legs.

• Reduced ability to feel hot or cold, especially in the hands.

• Problems using the hands and difficulty walking.

• Headaches, and sometimes severe arm and neck pain.

• Bladder or bowel control problems.

• Curvature of the spine.

Treatment depends on the cause and on whether the syrinx is causing problems. The Walton Centre says a syrinx is always caused by something else, and treatment is usually directed at the cause rather than the syrinx itself. Contact makes the same point: where syringomyelia is secondary to another condition, the treatment is not aimed at the syrinx but at the cause.

Where there is no identifiable cause, Contact says treatment can be controversial, because in many cases none is required and periodic follow-up with neurological examination and scanning may be all that is needed.

The Walton Centre describes the range of operations that may be used for Chiari malformation and syringomyelia: operating at the back of the head to enlarge the bony space, endoscopic third ventriculostomy (a fluid bypass procedure), placing drainage tubes (shunts) in the fluid spaces in the head or syrinx, exploring the spinal cord and enlarging the space available for fluid to flow, and untethering the spinal cord. It says treatment can be difficult.

For a Chiari I malformation, the NHS describes the main operation as decompression surgery. Under general anaesthetic a cut is made at the back of the head and a small piece of bone is removed from the base of the skull, and sometimes from the top of the spine, to reduce pressure and let fluid flow normally. The NHS says the aim is to stop existing symptoms getting worse, that some people also improve, and that surgery sometimes results in no improvement or symptoms getting worse. It also notes a small risk of serious complications such as paralysis or a stroke.

Painkillers can help with headaches and neck pain from a Chiari malformation, according to the NHS. Leeds Teaching Hospitals says medication may ease certain symptoms such as pain.

The spinal cord runs down the spine and carries messages between the brain and the body. In syringomyelia, a fluid-filled cavity (a syrinx) forms inside it. Leeds Teaching Hospitals describes it as a cerebrospinal fluid-filled tubular cyst within the spinal cord, in its central canal.

The Walton Centre says the underlying problem may relate to changes in the normal flow of fluid around the brain and spinal cord, leading to pressure, blockage of flow and, in syringomyelia, damage to the spinal cord tissue itself.

Leeds Teaching Hospitals also uses the older term hydromyelia for the same kind of cavity.

A syrinx is a sign of another problem rather than a condition that arises alone. The sources list these causes.

• Chiari malformation, where part of the cerebellum sits low and protrudes through the foramen magnum, the hole at the base of the skull. The Walton Centre says some patients with a Chiari I malformation will develop a syrinx.

• Spinal cord injury, meningitis or previous surgery, and a spinal cord tumour (Walton Centre).

• Spinal cord tethering, scoliosis and hydrocephalus (Contact lists tethering and scoliosis as possible causes, and hydrocephalus as an associated condition).

• No identifiable cause in some people (Contact).

Diagnosis starts with a history and examination. Leeds Teaching Hospitals says the doctor checks things such as balance, touch, reflexes, sensation and motor skills, which are functions controlled by the spinal cord and cerebellum.

MRI is the imaging test most often used to diagnose Chiari malformation, according to Leeds Teaching Hospitals, and it is painless. The Walton Centre says imaging of the complete neuroaxis (the whole of the brain and spinal cord), with flow studies where appropriate, is routinely used to assess patients, and that intracranial pressure monitoring can sometimes help to decide on the best treatment.

Many people with a Chiari malformation have no symptoms and it is found only when they are scanned for another reason, the NHS says. A syrinx can be found in the same way.

The NHS advises that if you have been diagnosed with a Chiari malformation, you should contact your doctor for advice if you develop any new symptoms or your symptoms worsen. The same principle applies to a known syrinx.

New weakness, numbness or burning sensations in the arms or hands, problems with walking, or changes in bladder or bowel control are the kind of symptoms the NHS and hospital leaflets associate with spinal cord involvement, and they should be assessed rather than waited out. If you are unsure how urgent your symptoms are, NHS 111 can advise.

Because syringomyelia is slow to change in many people, the thing worth tracking is change: write down what you notice and when, and report it to the team that looks after you.

The outlook depends on the cause, the size and position of the syrinx, and how early it is treated. The NHS says that, in Chiari malformation, syringomyelia can damage the spinal cord if not treated promptly. Surgery can usually stop symptoms getting worse and can sometimes improve them, although some problems may remain.

The Walton Centre says treatment can be difficult and that it examines its outcomes using a spine surgery database, which reflects how individual the course can be. Contact notes that periodic neurological examination and scanning may be needed in follow-up.

Support is available from patient charities. Leeds Teaching Hospitals lists the Brain and Spine Foundation helpline, run by neuroscience nurses, and the Ann Conroy Trust, a UK charity for people with Chiari malformation and syringomyelia.

On the NHS, syringomyelia is usually suspected after a GP assessment and confirmed with an MRI scan, and care is led by neurology or neurosurgery. Specialist neurosurgical centres treat Chiari malformation and syringomyelia, and the Walton Centre notes that treatment can involve several types of operation and long-term follow-up.

Some people choose to arrange MRI imaging or a neurosurgical opinion privately. The tests and operations are the same in either setting. If you do, make sure the scan images and reports are shared with the NHS team that follows you, so comparisons over time are possible.