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Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

Treating Idiopathic Intracranial Hypertension

Michael Wall, MD
Department of Ophthalmology and Visual Sciences
University of Iowa

Peer Review Status: Internally Peer Reviewed
Creation Date: 1991
Last Revision Date: 2001


Treatment for patients with IIH can be divided into medical treatment and surgical treatment. The cornerstone of medical treatment is weight loss. It does not appear to be the total number of pounds lost. Some patients are effectively treated by losing one pound every week or two for several months and then maintaining the weight loss. It may be the loss of fluid accompanying weight loss that is the significant factor but this has not been proven.

Loss of fluid can also be obtained using diuretics (fluid pills). Diamox (acetazolamide) is the most commonly used medication. It is relatively safe but nearly all patients have tingling of the fingers and toes. This tingling is a benign symptom. Patients also experience that carbonated soft drinks taste metallic. Less commonly, kidney stones can occur and rarely other blood disorders. Another diuretic commonly used that appears to be effective in some patients is Lasix (furosemide). More about Diamox.

The surgical treatments currently used are optic nerve sheath fenestration (making slits in the optic nerve sheath or covering) and lumbar shunting procedures (running a tube from the spinal fluid space in the lower spine into the abdominal cavity). These procedures are used when patients do not respond adequately to medical therapy. Optic nerve sheath fenestration is done first by an incision into the orbit. The eyeball is moved to the side and the optic nerve sheath is exposed. Slits or a large hole are then placed in the optic nerve sheath and fluid drains out, thereby taking pressure off the optic nerve.

The second surgical procedure, called lumbar-peritoneal shunting, is done as follows. Tubing is placed in the spinal fluid space, (the space entered during a lumbar puncture or spinal tap and tubing is then run to the abdomen. This lowers the pressure around the brain and optic nerve, thereby eliminating the symptoms of raised intracranial pressure. Unfortunately, these procedures are complicated by various problems, the most severe one being some patients have periodic occlusion of the tubing with recurrence of symptoms and sometimes vision loss. A repeat operation is then needed. An overview of treatment is summarized in figure 12.

Treatment strategies for IIH

Figure 12. Treatment strategies for IIH.

Management of the pregnant IIH patient

Pregnancy occurs in IIH as often as in the general population and in any trimester. Patients with IIH during pregnancy do not have an increased spontaneous abortion rate. Therapeutic abortion to limit progressive disease is not indicated. The pregnant patient with IIH should be treated as any other patient with IIH. Also, the pregnancy should be managed like any other. The major exception is caloric restriction because of its adverse effect of ketosis on the fetus. Weight gain can be limited to 20 pounds.

Use of corticosteroids has not been associated with birth defects in humans. Acetazolamide may be used after 20 weeks gestation; use before 20 weeks has been associated with one case of sacrococcygeal teratoma. Glycerol and thiazide diuretics probably should not be used in the second half of pregnancy because of the risk of decrease in placental blood flow. There is no obstetric contraindication to surgery for those that require it.

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Last modification date: Mon Aug 7 13:11:45 2006
URL: http://www.uihealthcare.com /topics/medicaldepartments/ophthalmology/iih/treatment.html