Treating Pseudotumor Cerebri

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Adult Hydrocephalus Program

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Because of the threat of blindness, it is very important for patients who have symptoms of pseudotumor cerebri to be evaluated by a specialist who has experience with this condition.

Usually, the first approach to treatment for pseudotumor cerebri is with:

  • Medications (acetazolamide or furosemide, or rarely corticosteroids)
  • Weight loss with a low-sodium diet
  • An aerobic exercise program

The patient’s treatment plan also may include periodic lumbar punctures to remove some cerebrovascular fluid and, thus, relieve pressure on the brain.

Eye Surgery

Vision loss in patients with pseudotumor cerebri can come on quickly. Therefore, if your primary symptom is worsening vision, rather than headache or other symptoms, a neuro-ophthalmologist may recommend surgery to reduce the swelling in the optic nerve. This surgery is called optic nerve sheath fenestration (perforation). Sometimes this surgery is done on an emergency basis to protect your eyesight.

What happens during optic nerve sheath fenestration surgery?

Both a neuro-ophtalmologist and an anesthesiologist will talk with you before the procedure to ensure you understand everything that will happen. This procedure reduces the risk of blindness, but it does not cure pseudotumor cerebri. Therefore, the surgeon also will talk with you about the need for continued follow-up treatment. If you are experiencing vision loss in both eyes, the surgeon will perform two procedures on different days.

The surgery takes place in an operating room. You will be lying on your back on the operating table. When you are completely asleep, the surgeon will prepare your eye so it stays open throughout the procedure. The surgeon then performs a series of procedures to expose the covering (sheath) of the optic nerve. Next, the surgeon will make a series of small incisions to release the pressure on the optic nerve.

What happens after the surgery?

After your surgery, the OR staff will move you to the recovery room. After you are awake, the transport team will move you to a room in the hospital. You will probably spend just one night in the hospital so we can monitor your progress and check your vision and the function of your pupil. Your surgeon will prescribe an antibiotic to help protect you from an eye infection, and also may prescribe antibiotic-steroid drops, which you will use for the first week. Usually recovery is pain-free and does not require pain medicine. We will schedule you for a follow-up visit one to two weeks after your surgery.

Surgery to implant a shunt

In some cases, the most appropriate treatment is placement of a shunt to help drain the extra fluid. This procedure consists of implanting a small tube (shunt or catheter) into chambers in your brain called ventricles. The shunt allows the CSF to drain into your abdomen, where it is absorbed. A valve is attached to the shunt to control how much CSF drains. The shunt:

  • Controls how much CSF is in your brain
  • Helps prevent CSF from building up
  • Reduces pressure on your brain
  • Improves your symptoms

What is a shunt?

The shunt is a system that moves fluid from one place to another. The system includes:

  • Two tiny tubes (catheters)
  • A one-way valve that connects the two tubes

The valve is about the size of a silver dollar from one end to the other, but it is very thin and narrow. The valve allows fluid to move only one way – away from your brain.

How does a shunt work?

Shunts can be placed in various locations. Your surgeon will talk with you about these options.

  1. Typically, one of the tubes runs from the ventricle in your brain to the valve, with the other tube running from the valve to your abdomen. This is called a ventriculoperitoneal shunt.
  2. Sometimes the first tube may be placed into the lower spine, where CSF also circulates, and the second tube placed in your abdomen. This is called a lumboperitoneal shunt.
  3. The second tube may also end at a blood vessel near the heart or in the space around the lungs, instead of the abdomen.

The shunt system allows extra spinal fluid to drain into an area where it can be safely absorbed by your body. The valve can be adjusted to control the flow of fluid. This keeps fluid from building up, but also ensures the right amount of fluid is still available to protect and cleanse your brain.

What happens during the surgery to implant the shunt?

Before surgery, your neurosurgeon will talk with you about the procedure to be certain you understand everything that will take place. Your anesthesiologist will also talk with you.

The shunt implant surgery takes place in an operating room at the hospital. The procedure takes about 30 minutes. The anesthesiologist will give you medicine so you are completely asleep during the procedure, and will monitor you throughout the procedure.

If you are undergoing a ventriculoperitoneal (VP) shunt placement, the nursing staff will shave a small section of hair on your head. They will also thoroughly wash your head and abdomen with a special soap. This ensures the area is sterile.

When you are asleep, the neurosurgeon will make a small incision in your scalp, and a small hole in your skull and one in the protective covering of the brain. The surgeon will use special imaging equipment, called intra-operative navigation, to see inside your brain and locate the correct place for the shunt. The neurosurgeon will make an additional incision (usually above or behind your ear) to assist with placement of the tubing.

One end of the shunt is placed in the ventricle. The other end is connected to the valve. The second tube is connected to the other end of the valve. This tube is then tunneled just beneath your skin down to the abdomen below your ribs. The tubing is inserted into the abdomen either through a small incision, or with the assistance of a general surgeon using a laparoscope and two tiny incisions. 

After placing the shunt and valve, the doctor will cover the incisions with sterile bandages.

After the procedure, the OR team will move you to the recovery room. Once you are awake, the transport team will move you to a room in the hospital. You will stay overnight in the hospital, so we can monitor you during your recovery. Most patients leave the hospital after one or two days.

Will the shunt be visible after surgery?

You will notice a small lump behind your hairline and above your ear where the valve is located. Your hair will grow back in that area, so it will not be noticeable to most people.

Recovering after surgery

Some of your activities will be limited when you first leave the hospital. For example, you should not lift anything heavier than 10 pounds for two to four weeks, and you should not resume driving if you are taking narcotic pain medication. Although the valve is beneath the skin on your head and protected, you should be careful not to bump it. You should have someone look at your incisions every day. You should call your surgeon’s office if they notice any signs of infection, such as:

  • Redness and hardness around the incision
  • Hot to the touch
  • Green or yellow discharge
  • Fever
  • Excessive bleeding
  • The incision is getting bigger
  • The incision has a bad smell 

During the first few months our clinical scheduler will make several follow-up appointments for you at the clinic. During those visits, we will check the valve and ensure you are recovering well. Your doctor will also check to make sure the spinal fluid is flowing correctly. We use special equipment that allows us to adjust the valve in the clinic – even though it is beneath your skin – to make the flow just right. After the first few months, you will have checkups once a year.

The neurosurgeon also may prescribe physical therapy or an appointment with a rehabilitation specialist if you have any problems with walking, standing or balancing.

Will all of my symptoms go away after surgery?

Inserting a shunt should help reduce headaches, dizziness, and nausea and vomiting. How much you improve will depend on how serious your symptoms were by the time you had surgery. The shunt may also improve symptoms related to your vision.

A reminder: A magnet, like the magnets in an MRI, can affect the valve and possible change the setting. If you need an MRI, please let the doctor know about your valve. After you have an MRI, you will need to schedule an appointment for us to check your valve setting in the clinic