Sohaib R Rufai1,2,3,4, Oliver R Marmoy1,2, Dorothy A Thompson1,2, Lara S van de Lande2,4, R William Breakey2,4, Catey Bunce5, Vasiliki Panteli1, Kemmy Schwiebert1, Shafquet Mohamed1, Frank A Proudlock3, Irene Gottlob3, David J Dunaway2,4, Richard Hayward2,4, Richard Bowman1,2, Noor Ul Owase Jeelani6,7. 1. Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. 2. UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, UK. 3. The University of Leicester Ulverscroft Eye Unit, Leicester Royal Infirmary, Robert Kilpatrick Clinical Sciences Building, PO Box 65, Leicester, LE2 7LX, UK. 4. Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. 5. Clinical Trials Unit, The Royal Marsden NHS Foundation Trust, London, SW3 6JJ, UK. 6. UCL Great Ormond Street Institute of Child Health, London, WC1N 1EH, UK. Owase.Jeelani@gosh.nhs.uk. 7. Craniofacial Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. Owase.Jeelani@gosh.nhs.uk.
Abstract
AIMS: To assess the diagnostic accuracy of fundoscopy and visual evoked potentials (VEPs) in detecting intracranial hypertension (IH) in patients with craniosynostosis undergoing spring-assisted posterior vault expansion (sPVE). METHODS: Children with craniosynostosis undergoing sPVE and 48-hour intracranial pressure (ICP) monitoring were included in this single-centre, retrospective, diagnostic accuracy study. Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP > 20 mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes. RESULTS: 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]: 15.9-52.4) and 58.3% for isolated VEPs (95% CI 36.6-77.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0-100) and 83.3% for isolated VEPs (95% CI: 35.9-99.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0-89.7), while specificity decreased to 60% (95% CI: 14.7-94.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0-72.2) and specificity increased to 100% (47.8-100). Median final BCVA was 0.24 logMAR (n = 36). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as ≥0.30 logMAR in the better eye. CONCLUSION: 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.
AIMS: To assess the diagnostic accuracy of fundoscopy and visual evoked potentials (VEPs) in detecting intracranial hypertension (IH) in patients with craniosynostosis undergoing spring-assisted posterior vault expansion (sPVE). METHODS: Children with craniosynostosis undergoing sPVE and 48-hour intracranial pressure (ICP) monitoring were included in this single-centre, retrospective, diagnostic accuracy study. Data for ICP, fundoscopy and VEPs were analysed. Primary outcome measures were papilloedema on fundoscopy, VEP assessments and IH, defined as mean ICP > 20 mmHg. Diagnostic indices were calculated for fundoscopy and VEPs against IH. Secondary outcome measures included final visual outcomes. RESULTS: 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]: 15.9-52.4) and 58.3% for isolated VEPs (95% CI 36.6-77.9). Specificity for IH was 100% for fundoscopy (95% CI: 59.0-100) and 83.3% for isolated VEPs (95% CI: 35.9-99.6). Where longitudinal deterioration was suspected from some prVEPs but not corroborated by all, sensitivity increased to 70.6% (95% CI: 44.0-89.7), while specificity decreased to 60% (95% CI: 14.7-94.7). Where longitudinal deterioration was clinically significant, sensitivity decreased to 47.1% (23.0-72.2) and specificity increased to 100% (47.8-100). Median final BCVA was 0.24 logMAR (n = 36). UK driving standard BCVA was achieved by 26 patients (72.2%), defined as ≥0.30 logMAR in the better eye. CONCLUSION: 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.
Authors: Karan R R Ramdat Misier; Richard W F Breakey; Cornelia J J M Caron; Silvia Schievano; David J Dunaway; Maarten J Koudstaal; Owase N U Jeelani; Alessandro Borghi Journal: J Craniofac Surg Date: 2020 Jul-Aug Impact factor: 1.046