Chiari malformation and syringomyelia

A Chiari (or Arnold Chiari) Malformation is an abnormality of the posterior fossa (back of the skull) and top of the spine.  There are different types. 

A Chiari I involves the dropping of the lowest part of the back of the brain (the cerebellar tonsils) into the top of the spinal canal, and is probably caused by the brain in this area being too big for the skull.  This may cause no symptoms or may cause severe headaches,  that is often worse on coughing or sneezing. 

Chiari II and III malformations are associated with spina bifida (a congenital condition that may be associated with hydrocephalus and abnormalities at the base of the spine).  

Syringomyelia is a condition in which fluid filled cavities form within the spinal cord and can lead to pain, and loss of function (weakness or numbness).  Many patients with a Chiari I malformation will develop a syrinx and the two conditions are thought to be related.  Syrinxes can also occur after a spinal cord injury, meningitis, surgery, or if there is a tumour present. 

It is thought that both conditions cause symptoms because they change the normal fluid dynamics within the persons central nervous system.

The treatment of Chiari malformations and syringomyelia can be difficult and can involve 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.

The Walton Centre has a number of surgeons with an interest in the treatment of patients with these conditions. 

There is a monthly Syringomyelia clinic where new patients or patients with complex Chiari or syringomyelia issues can be seen by up to four of the surgeons. 

Imaging of the complete neuroaxis, with flow studies where appropriate is routinely used to assess patients. Intracranial pressure monitoring can also prove helpful in deciding on the best treatment. Outcomes are examined with the spine tango database.

The Surgeons who have a particular interest in this condition are Mr Pigott, Mr Brodbelt, Mr Buxton, and Mr Mallucci. Referrals can be made to any of these surgeons (except Mr Mallucci, who contributes to the clinic, but works full time at Alder Hey Childrens Hospital).

 

Relevant Publications