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Syringomyelia

Background

Syringomyelia refers to a cystic-like change (syrinx) within the spinal cord. It can have many different causes such as spinal cord trauma, spinal cord tumours, scoliosis, spinal cord tethering and Chiari malformation, or it may have no identifiable cause. Hydrocephalus can be an associated condition and treatment of it and some of the other associated problems above may resolve the syringomyelia.

Credits

Medical text written December 2002 by Mr N Buxton. Last updated June 2007 by Mr N Buxton, Consultant Paediatric Neurosurgeon, Alder Hey Children's Hospital, Liverpool, UK.

What are the symptoms?

The cyst-like change within the spinal cord can extend over a variable length and the symptoms it can cause reflects the level of the change within the spinal cord. Sometimes they can actually be asymptomatic. However, often they can affect sensory perception and ultimately can cause deranged motor function. As the syrinx is usually secondary to another condition, the patient most commonly presents with the symptoms attributable to that condition. Therefore in cases of syringomyelia secondary to another condition, the treatment is not aimed at the syrinx but at the cause.

Chiari malformation
Originally referred to as Arnold Chiari malformation it is also known as hindbrain hernia. In essence it is herniation (protrusion) of the bottom of the cerebellum, an area at the back and base of the brain, through the foramen magnum (the hole in the bottom of the skull for the spinal cord to exit). There are two basic types that are most commonly seen. Type one has no other associated cause and type two is associated with spina bifida. The treatment of type two may involve treatment of the spina bifida. Both types can cause syringomyelia. The treatment can cause resolution of the problem.

Idiopathic, or type one Chiari malformation, can present with headaches particularly at the back of the head, made worse by coughing, sneezing, straining. There may also be dizziness, disturbance of vision and abnormal sensations in the arms and legs. Treatment is usually surgical, but not in every case. Specialists will consider various approaches. Hydrocephalus may be associated and its treatment may resolve the Chiari malformation.

How is it treated?

The treatment of syringomyelia with no identifiable cause can be controversial as in many cases no treatment may be required and periodical follow-up with neurological examination and scanning may be necessary.

Inheritance patterns and prenatal diagnosis

Inheritance patterns
In general, none. However, some conditions causing syringomyelia may be genetic.

Prenatal diagnosis 
Where another condition is the cause, ultrasound scanning may be used in diagnosis.

Is there support?

Ann Conroy Trust

Tel: 0300 111 0004
Email: info@annconroytrust.org
www.annconroytrust.org

The Trust is a Registered Charity in England and Wales No. 510582. It provides information and support to those living wih Chiari Malformation, Syringomyelia and associated conditions.

Group details last updated September 2014.