Palermo Matteo, Trevisi Gianluca, D'Arrigo Sonia, Sturiale Carmelo Lucio
Department of Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.
Department of Neurosciences, Imaging and Clinical Sciences, G. D'Annunzio University, Chieti-Pescara, Italy.
Eur J Neurol. 2025 May;32(5):e70186. doi: 10.1111/ene.70186.
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.
妊娠期间的特发性颅内高压（IIH）由于妊娠期生理变化而带来重大挑战，这些变化会加剧其症状。
我们对报告妊娠期间IIH母婴结局的研究进行了系统评价，选择了49篇报告临床病程、管理策略和分娩方式的论文。
我们检索到165例患者，178次妊娠受IIH影响。肥胖是常见的危险因素（69.1%），但与其他心血管和代谢危险因素的关联讨论较少。总体而言，62.9%的患者头痛加重，66.8%的患者出现视力障碍，但这些数据是从单个病例或小系列病例中推断出来的，存在选择偏倚，可能高估了实际风险。目前一线治疗以乙酰唑胺（52例）或其他利尿剂（4例）联合体重控制为主。26.9%的药物治疗无效病例报告进行了连续腰椎穿刺（LP）。分流术（3.9%）和视神经鞘开窗术（1.1%）总体上在少数病例中进行。二线管理的特点是，对于最初仅接受利尿剂治疗的患者进行连续LP，对于需要持续脑脊液引流的患者进行分流术（占4.5%）或视神经鞘开窗术（占1.7%）。
尽管与妊娠相关的生理变化可能会加重IIH，且实际风险仍难以量化，但就疾病复发或新发而言，总体风险似乎较低。利尿剂，尤其是乙酰唑胺，与先天性畸形无因果关系，连续腰椎穿刺是安全有效的一线治疗方法，而分流手术应仅用于暴发性病例。妊娠前症状缓解似乎可降低妊娠期间病情严重恶化的可能性。
