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Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP\u2009&gt;\u200920\u2009mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fundoscopic examinations were available for 35 children and isolated VEPs for 30 children, 22 of whom had at least three serial VEPs. Sensitivity was 32.1% for fundoscopy (95% confidence intervals [CI]: 15.9\u201352.4) and 58.3% for isolated VEPs (95% CI 36.6\u201377.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0\u2013100) and 83.3% for isolated VEPs (95% CI: 35.9\u201399.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0\u201389.7), while specificity decreased to 60% (95% CI: 14.7\u201394.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0\u201372.2) and specificity increased to 100% (47.8\u2013100). Median final BCVA was 0.24 logMAR (<jats:italic>n<\/jats:italic>\u2009=\u200936). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as \u22650.30 logMAR in the better eye.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Papilloedema present on fundoscopy reliably indicated IH, but its absence did not exclude IH. VEP testing boosted sensitivity at the expense of specificity, depending on method of analysis.<\/jats:p><\/jats:sec>","DOI":"10.1038\/s41433-021-01839-w","type":"journal-article","created":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T06:41:52Z","timestamp":1641019312000},"page":"139-145","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Electrophysiological and fundoscopic detection of intracranial hypertension in craniosynostosis"],"prefix":"10.1038","volume":"37","author":[{"given":"Sohaib R.","family":"Rufai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2022-3669","authenticated-orcid":false,"given":"Oliver R.","family":"Marmoy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5491-3911","authenticated-orcid":false,"given":"Dorothy A.","family":"Thompson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara S.","family":"van de Lande","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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