Quantitative detection and follow-up of intracranial hypertension in craniosynostosis: an optical coherence tomography study

BACKGROUND: In patients with craniosynostosis, the authors evaluated the diagnostic accuracy of fundoscopy and optical coherence tomography (OCT) to detect intracranial hypertension (ICH), the time course of retinal thickness after treatment of ICH, and the relationship between high hyperopia (HH) and fundoscopy/OCT scan findings. METHODS: Patients with syndromic, multisuture, unicoronal, unilambdoid, or sagittal synostosis visiting the authors' national center were included in this longitudinal cohort study and formed a consecutive series. Retinal layers on OCT, OCT fundus images, and fundoscopy results were evaluated. ICH was scored according to presence of abnormal intracranial pressures, hydrocephalus, progressive cerebellar tonsillar herniation or fingerprinting, and growth arrest. Diagnostic accuracy of OCT, fundoscopy, and fundus image; the time course of retinal thickness after ICH; and interference of HH were analyzed using linear mixed models. RESULTS: A total of 577 OCT scans in 307 patients were included. ICH was found in 7.2%. Combining total retinal thickness (TRT), OCT fundus imaging and fundoscopy resulted in a sensitivity of 76% and 81% specificity to detect signs of ICH. TRT was increased in patients who had had signs of ICH versus patients who had never had signs of ICH (β +44.9 µm in patients who had had ICH [95% CI, 9.0 to 80.8]; P = 0.01). TRT decreased to normal in the years after surgery (β -3.6 µm/yr [95% CI, -7.2 to -0.05]; P = 0.047). There were greater odds of having increased TRT in patients with HH (OR, 2.9 [95% CI, 1.1 to 7.6]; P = 0.03). CONCLUSIONS: The correlation among TRT, OCT fundus image, fundoscopy, and particularly the combination of these measures with intracranial pressure surrogate markers is fair. Increased TRT in the presence of a clinical suspicion of ICH warrants further screening. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, III.