What is the initial treatment for idiopathic intracranial hypertension (IIH) in pregnant women?

Management of Idiopathic Intracranial Hypertension in Pregnant Women

The initial treatment for idiopathic intracranial hypertension (IIH) in pregnant women should focus on weight management and serial lumbar punctures if there is imminent risk of vision loss, while avoiding medications like acetazolamide and topiramate due to potential fetal risks. 1

First-Line Management Approach

Weight Management

• If not already under a weight management program, refer to a weight service to ensure weight gain is appropriate for gestational age of the fetus 1
• Aim for weight gain within recommended guidelines for pregnancy, as excessive weight gain may exacerbate IIH 2
• Regular monitoring of weight is essential as weight loss is the most effective non-pharmacological intervention for IIH

Visual Monitoring

• Regular ophthalmologic evaluations are necessary to monitor:
  • Visual acuity
  • Pupil examination
  • Formal visual field assessment
  • Dilated fundal examination to grade papilloedema
  • BMI calculation 1
• Frequency of monitoring should be based on severity of papilloedema and visual field status

Management of Acute Exacerbations with Imminent Risk to Vision

Serial Lumbar Punctures

• If IIH is active with imminent risk of vision loss, serial lumbar punctures should be considered as a temporizing measure 1
• This approach is preferred over medication during pregnancy when vision is threatened

Surgical Interventions for Vision-Threatening Cases

• For cases with imminent risk of vision loss that don't respond to lumbar punctures, consider:
  • CSF diversion procedures (such as ventriculoperitoneal shunt)
  • Optic nerve sheath fenestration (ONSF) 1
• Patients with imminent risk of vision loss at time of delivery should be managed in a specialist center 1

Medication Considerations

Acetazolamide

• Manufacturers do not recommend acetazolamide use during pregnancy due to teratogenic effects in animal studies 1
• A clear risk-benefit assessment must be discussed with the patient if considering acetazolamide 1
• Limited evidence suggests no convincing adverse effects in human pregnancy, even when prescribed prior to the 13th week of gestation 3, but caution is still warranted

Topiramate

• Topiramate should not be used in pregnancy 1
• Clear evidence shows a higher rate of fetal abnormalities following its use 1
• If a patient on topiramate becomes pregnant, they should reduce and discontinue it as soon as possible 1

Headache Management

• A clear risk-benefit assessment regarding headache treatment during pregnancy should be discussed, as many headache medications are not recommended in pregnancy 1
• Short courses of NSAIDs may be considered for headache management in early pregnancy with gastric protection 4
• Avoid opioids for headache management 4

Multidisciplinary Approach

• Collaboration is essential among:
  • Obstetrician experienced in IIH management
  • Neurologist
  • Ophthalmologist (preferably neuro-ophthalmologist)
  • Obstetric anesthetist 1, 5
• Multidisciplinary communication should occur throughout pregnancy, peridelivery, and in the postpartum period 1
• Increased outpatient observation may be helpful to reassure healthcare professionals and patients 1

Delivery Considerations

• No specific mode of delivery should be suggested based solely on IIH diagnosis 1
• The vast majority of women with IIH can have a normal vaginal delivery 2
• Spinal or epidural anesthesia can be used if needed, provided the papilloedema is stable or the IIH is in remission 2

Important Caveats

• Pregnancy can affect numerous preexisting neurologic conditions and may increase risk of intracranial hypertension 6
• Aim to achieve disease remission or control before pregnancy through optimizing weight 2
• Patients should be counseled about contraception before pregnancy, noting that hormonal contraceptives are not contraindicated in IIH 2
• Extended follow-up is essential as late recurrences can occur 4