A membrane that completes the anatomy of protection around our central nervous system, the dura mater is one “hard mother”. Beautiful and glistening in appearance, it is normally tough and leathery in consistency and difficult to physically tear - all the better for shielding our brain and spinal cord. Yet it has its sensitive side, particularly in Marfan syndrome when it is much thinner and stretchier. This can translate into severe back pain, while the existence of cysts along the spinal nerve roots is a well-known feature.

By Dr Anne Child & Victoria Hilton

Q: My son had a cyst removed from the spinal cord; the flow of fluid was trickling slowly before the operation but when the cyst was removed the spinal fluid flowed like a torrent. It took three weeks before he could sit up he has been left with scoliosis and the pain in his legs and buttocks is unbearable.

A: Dural ectasia is an enlargement of the dura (envelope) surrounding the spinal cord, especially in the lower back. It is a major diagnostic feature of Marfan syndrome, and although it is found on MRI in 65% of adult patients, most patients have no symptoms. It can cause lower back pain, headache, weakness and pain. The headache is relieved by lying down.

Occasionally surgical removal of a cyst is necessary, and should be performed by an experienced neurosurgeon. Blood patching of the dura may be necessary to stop spinal fluid leak.

In your son’s case, pain is now his main problem, and he will need expert medication and possibly injections around the spinal nerves, to relieve the pain. Please ask for a referral to your regional pain clinic for consideration of medication such as Gabapentin, and steroid injections to the nerves in the affected area, followed by nerve ablation. This may provide relief.

Please see our pamphlet on dural ectasia.

Marfan Trust, a CIO registered as a charity in England in Wales with charity number 1198847 at: c/o 24 Oakfield Lane, Keston, Kent, BR2 6BY. Contact us at [email protected] or by phone on + 44 (0)333 011 5256
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