Idiopathic Intracranial Hypertension Treatment & Management

The treatment goal for patients is to preserve optic nerve function while managing their increased intracranial pressure.

• Optic nerve function should be carefully monitored with an assessment of visual acuity, color vision, optic nerve head appearance, and perimetry.
• The medical management is multifaceted and consists of the following:

  • Weight control for obese patients
    • Most patients with this disorder are females who are overweight. Weight loss is a cornerstone in the management of these patients. Unfortunately, weight reduction generally proves to be a difficult task for these patients.^[16]
    • As little as a 5-10% weight loss has been demonstrated to result in a reduction of the intracranial pressure with the accompanying resolution of papilledema.^[5]
    • To formalize the process of weight reduction, referral to a dietitian is appropriate.
  • Treatment of related underlying diseases (see Causes)
  • Cessation of exogenous agents related to increased intracranial pressure
  • Use of diuretics to control the intracranial pressure
    • To protect the optic nerve function, the intracranial pressure must be lowered.
    • Acetazolamide appears to be the most effective diuretic in lowering the intracranial pressure. The initial dose should be 0.5-1 g/d. Although for compliance purposes, the 500 mg sequel taken orally twice a day is preferred; some insurers only cover an oral dose of 250 mg taken 4 times per day. Most patients respond to a dose of 1-2 g/d. Although this dose can be increased up to 3-4 g/d, most patients do not tolerate the troubling adverse effects (eg, extremity paresthesias, fatigue, metallic taste when drinking carbonated beverages, decreased libido) of this medication at this high dose.^{[5, 1]}
    • In the event of intolerance to acetazolamide, furosemide may be used as a replacement diuretic in this group. Unfortunately, furosemide does not appear to be as effective as acetazolamide.
  • Corticosteroids
    • Corticosteroids are effective in lowering the intracranial pressure in those patients with an inflammatory etiology for their idiopathic intracranial hypertension.
    • In addition, steroids may be used as a supplement to acetazolamide to hasten recovery in patients who present with severe papilledema.
    • Because of the significant adverse effects, corticosteroids should not be considered as a long-term solution for these patients. In addition, a rebound in the intracranial pressure may occur during the taper of the corticosteroids.^[1]

Patients with idiopathic intracranial hypertension should be closely monitored while on medical treatment. The frequency of visits is determined by the initial state of the patient's visual function and the response to medical treatment. Despite close follow-up care and maximum medical treatment, some patients experience deterioration of their visual function. In this situation, surgical intervention should be considered. Two procedures that can be performed are optic nerve sheath fenestration or a cerebrospinal fluid diversion procedure (ie, lumboperitoneal shunt, ventriculoperitoneal shunt). Treatment of this disorder by repeated lumbar punctures is considered to be of historic interest.

• Optic nerve sheath fenestration

  • Optic nerve sheath fenestration has been demonstrated to result in the reversal of optic nerve edema with some recovery of optic nerve function. In addition, it may result in the decrease in the headache of many patients. The approach to the optic nerve may be from the medial or lateral aspect of the orbit; each technique has its benefits and drawbacks.^[17]
  • Occasionally, a bilateral curative effect of the papilledema occurs from unilateral surgery. However, if this is not the case, then the opposite nerve must undergo the same procedure.
  • Although the intracranial pressure remains elevated in these patients postoperatively, the local filtering effect of the fenestration acts as a safety valve and eliminates the pressure from being transmitted to the optic nerve.
  • Complications related to this procedure include diplopia, optic nerve injury, vascular occlusion, a tonic pupil, and the inherent risk of hemorrhage and infection with intraconal surgery.
  • Unfortunately, Spoor has demonstrated that the long-term success rate of this operation may be only 16%.^[18]
• Cerebrospinal fluid diversion procedures (ie, lumboperitoneal shunt, ventriculoperitoneal shunt)

  • These two neurosurgical interventions are highly effective in lowering the intracranial pressure. In some facilities, they remain the procedures of choice for treating patients with idiopathic intracranial hypertension who do not respond to maximum medical treatment.^{[1, 3, 17]}
  • Shunts are also indicated in the following: patients with intractable headaches, regions where no access is available to a surgeon who is comfortable with optic nerve sheath fenestration, and patients with a failed optic nerve sheath fenestration.
  • Sinclair et al found that although cerebrospinal fluid (CSF) diversion minimizes visual decline and improves visual acuity, 68% of patients continued to have headaches and 28% had low-pressure headaches, which complicated surgery. Shunt revision was required for 51% of patients, with most requiring multiple revisions. For those with otherwise untreatable, rapidly declining vision, CSF diversion shunting should be conducted as a last resort. Other treatments, such as weight reduction, may be more effective and may have less associated morbidity.^[19]

• Weight reduction has been clearly demonstrated to be an important factor in the long-term management of these patients.
• As little as a 5-10% decrease in the total body weight can result in the resolution of papilledema. Unfortunately, weight loss in patients who are obese is difficult.^[5]
• A referral to a dietitian is worthwhile in patients who are motivated to lose weight.