Defining the Phenotype and Prognosis of People With Idiopathic Intracranial Hypertension After Cerebrospinal Fluid Diversion Surgery

Purpose To characterize the phenotype of patients with idiopathic intracranial hypertension (IIH) who received cerebrospinal (CSF) diversion surgery and to detail the trajectory of recovery. Design Prospective cohort registry study. Methods Patients with IIH with sight-threatening papilledema presenting to a single United Kingdom neuroscience center between 2019 and 2021 were included. Outcomes consisted of perimetric mean deviation (PMD) and optical coherence tomography measures of papilledema (retinal nerve fiber layer [RNFL]) and macular ganglion cell layer (GCL) in both eyes. Headache outcomes included monthly headache days (MHD). Logistic regression methods were used to model long-term outcomes. Results Fifty-one patients without previous surgical interventions were included (92% female, mean age 28.1 years [SD 8.4], body mass index 37.4 kg/m2 [SD 9.7], mean days of follow-up 330 [SD 209]). Measurements before surgery showed mean PMD −11.4 dB (SD 9.7), RNFL 364 µm (SD 128), Frisén grade papilledema 4.3 (SD 0.9). and MHD 23 (SD 10.6). At 1 month postoperatively, RNFL and PMD had improved by 38% and 4%, respectively. At 4 months postoperatively, papilledema had resolved. GCL declined by 13% over 12 months. MHD reduced by 75% 3 months postoperatively before returning to baseline levels by 12 months. Five patients (9.8%) required revision surgeries. Conclusions Detailed characteristics of patients with sight-threatening IIH who received CSF diversion surgery and their typical postoperative recovery are presented. These parameters should guide physicians as to when patients with IIH may require surgery and enable the early identification of outliers who fail to respond. Papilledema and PMD recovered but GCL atrophy continued for 12 months. The implication of this delayed atrophy is unknown.