Idiopathic Intracranial Hypertension in Pregnancy. A Systematic Review on Clinical Course, Treatments, Delivery and...

Idiopathic intracranial hypertension (IIH) during pregnancy presents significant challenges due to the physiological gestational changes, which can exacerbate its symptoms.

We conducted a systematic review on studies reporting maternal-fetal outcomes of IIH during pregnancy, selecting 49 papers reporting on clinical course, management strategies, and mode of delivery.

We retrieved 165 patients with 178 pregnancies affected by IIH. Obesity represented a common risk factor (69.1%), but the association with other cardiovascular and metabolic risk factors was poorly discussed. Overall, 62.9% presented worsening of the headache and 66.8% impairing visual disturbances, but these data were extrapolated from single cases or small series comporting a selection bias potentially overestimating the real risk. First-line treatment is currently represented by acetazolamide (52 cases) or other diuretics (4 cases) associated with weight control. Serial lumbar punctures (LP) were reported in 26.9% of cases of ineffective pharmacological treatment. Shunt (3.9%) and optic nerve sheath fenestration (1.1%) were overall performed in a minority of cases. Second-line management was characterized by serial LP in patients initially treated only with diuretics and shunt placement (4.5%) or optic nerve sheath fenestration (1.7%) for patients requiring continuous CSF subtractions.

Although pregnancy-related physiological changes may exacerbate the IIH and the actual risk remains difficult to quantify, this appears overall low in terms of re-exacerbation of the disease or de-novo onset. Diuretics, in particular acetazolamide, that did not show a causal relationship with congenital malformations, and serial lumbar punctures represent safe and effective first-line managements, whereas shunt procedures should be reserved for fulminant cases. A pre-gestational symptoms relief seems to reduce the probability of a severe worsening in pregnancy.