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

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