Surgical treatments in severe IIH

Surgical treatments in severe IIH

You will have been told by your Doctor that you need to have a surgical procedure for Idiopathic Intracranial Hypertension. This may be because all the other treatments have not been a success, and they feel that this is the best option for you in the long term to treat your IIH. This may have come as a shock, as surgery is quite daunting and frightening for some people. You may go through many emotions, anger, fear, depression and worry. For some people it is a relief, because they have been so ill with the symptoms of IIH. Everybody is different and so are their reactions to surgery. This booklet will help you to understand why you need surgery and what the different types of surgery are, to treat raised intracranial pressure in IIH. Why do I need surgery? Surgery becomes necessary when your visual fields are decreasing at a rapid rate, and medication has been unsuccessful to control your raised cerebrospinal fluid (CSF) pressure. Your Doctor will only refer you to a Neurosurgeon when all medications and less invasive treatments have been tried without success. This means that you have Fulminant or Severe IIH. Page2

What kind of surgery will I have? Your Neurosurgeon will recommend the surgery you have based on your personal case. What are the different types of surgery? There are different types of surgery to treat IIH and depending on your case history it may be one of the following: Optic Nerve Sheath Fenestration (ONSF) This is also known as optic nerve sheath decompression and it is performed in order to reduce the swelling of your optic nerve (Papilloedema). It can be performed unilaterally (one nerve) or bilaterally (both nerves). Under anaesthetic, small cuts are made in the optic nerve sheath. This allows the CSF to drain out and release pressure on the optic nerve. This procedure is used to help the sight symptoms associated with IIH, but because it has little effect on reducing the overall pressure within the skull, it typically does not have much effect on other symptoms including headache. Lumbo Peritoneal (LP) shunt A lumbo peritoneal shunt or LP shunt is a catheter (thin tube) that is inserted between two vertebrae in the spinal column where the subarachnoid cavity is, this is a spongy tissue filled cavity that surrounds the brain and spinal cord, which is where Cerebrospinal fluid (CSF) is contained. It then drains the excess cerebrospinal fluid from the brain via the subarachnoid cavity and transports it to the peritoneal cavity (a fluid-filled gap between the walls of the abdomen and the organs in the abdomen); the fluid is eventually absorbed by the organs and passed out of the body during urination. Page3

Ventriculo Peritoneal (VP) shunt A ventriculo peritoneal shunt or VP shunt, is also a thin tube (ventricular catheter), but unlike the LP shunt it is connected to a valve, this is then connected to another catheter which is then inserted into your peritoneal cavity. The settings of the valve regulate the CSF pressure. The valve can have a fixed setting or be a programmable valve. If the valve is programmable this means that your settings can be adjusted if necessary without the need for surgery. Your Neurosurgeon will discuss this when he/she goes through your surgical procedure with you. Dural Venous Sinus Stent The dural venous sinuses are veins that are found between layers in the thick membrane (dura mater) that covers the brain and spinal cord. This keeps the CSF contained safely. Sometimes the venous sinuses can decrease the blood flow out of the brain. If this is the case a stent is put into open the vein for the blood to flow more freely. Your Doctors will have conducted tests first to see if this procedure is necessary. Sub Temporal Decompression Sub Temporal Decompressions are rarely performed but can be done in the case of slit-ventricle syndrome. Slit-ventricle syndrome is when the ventricles are very small or slit like. A portion of temporal bone is removed to relieve pressure and create more space. It has been described as a window. This procedure can be done unilaterally or bilaterally. Page4 Discussing your surgery Whatever surgical procedure you are having, your Neurosurgeon will discuss all the reasons why they are recommending the surgery, as well as talk you through the procedure. If you have any questions they will only be too happy to answer them and put your concerns at rest. You will

then be placed on a waiting list, and the length of time you wait will be dependent on several factors: how urgent you need the surgery and when there is available theatre time for your surgery. It is possible that you could be waiting a while for your surgery, this is because a hospital has only so many theatres, and there will be other surgeons from other specialities, who have patients that require surgery. Your Neurosurgeon will book you in as soon as there is a slot available for you. If you are concerned about the length of time you are waiting, or your symptoms become worse, contact your Neurosurgeon. Unless you are already in hospital, you will be sent an appointment for a pre-operative assessment so that you can be examined and confirmed as healthy for surgery. At your assessment as well as having some routine tests, your surgery will be discussed so that you understand what will happen and what the risks are. Don t be too alarmed, there are risks with any surgery, and you have to be informed of them by your Neurosurgeon. They may then ask you to sign a consent form. Page5 Recovery after surgery Recovery after a surgical procedure is different for everyone and your Neurosurgeon will be able to give you an idea of how long you may be in hospital. Usually it can be for a few days to a week. You can also ask about things to be careful of doing once you have had your surgery. With all these procedures, there may be some activities you are restricted from doing, such as fun fair rides, but you should be able to have a normal life, and the purpose of the surgery is to relieve your IIH symptoms and prevent further deterioration. When your Neurosurgeon is happy with your progress, they will discharge you with some medication, wound dressings, a letter for the District Nurse, who will have been informed of your discharge so that she can attend to your wounds and remove any staples or stitches you have. You will also be given a discharge letter to give to your GP.

Your experience of surgery To understand more about what it is like to undergo some of the operations used to treat IIH, we recommend you visit our website, www.ihaveiih.com, where you will be able to read the personal stories of individuals preparing for surgery, the kinds of questions they have asked, and what they have experienced. No two stories are the same. Some people go through their surgery very smoothly. Others take a little more time to recover, or may need additional procedures to adjust how well a shunt is working, for example. Helen says: Although the thought of having surgery was very daunting and a bit on the scary side, once the Neurosurgeon explained everything to me, and the reason why surgery was necessary, I knew that for the benefit of my health and my vision I had to have the surgery. I asked my Neurosurgeon if it was necessary to shave my head and he said it wasn't, so my hair was left as it was. After the surgery I was in pain which was managed by the nurses, and I was sore for about a week. Once the pain was under control, and the Physiotherapist had me moving about, I was allowed home and told to be sensible about my activities. Within a month I was feeling back to normal and was able to resume my life as before, and I realised the surgery had sounded worse than it actually was. Katie says: My LP shunt was installed last year. The surgery was uneventful. Recovery was a breeze for me. I was in the hospital just overnight. My surgery was first thing in the morning. By evening I was up and walking around. Pain was not bad, the only restrictions I had were light lifting, showers only - no baths until the stitches came out and take it easy for a couple of weeks. Most importantly, work out what questions you want to ask, and make sure you ask them. Sarah says: The time before surgery is tough. It s the not knowing. I m sure that if you ask your surgeon he/she will explain everything. It s an easy surgery for them. Page6

The I Have IIH Foundation The I Have IIH Foundation is registered charity in England and Wales, no. 1155833. We provide online support, information and practical help and advice. We support patients with IIH and their families, we also work with many other charitable organisations that can be found on our website, who provide valuable resources and information. We are also members of the RARE Foundation Alliance. You can find us: www.ihaveiih.com I Have IIH Foundation @IHaveIIH Other booklets available in this series Just Diagnosed What is IIH Diagnostic Tests for IIH Living with IIH Carer s Booklet Medical Treatment and Monitoring in IIH We are grateful to the Big Lottery Fund for providing the support to enable us to produce these booklets. Page7