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Background and purpose: Craniosynostoses are rare congenital craniofacial malformations, variably affected by hearing loss, often requiring repeated CT examinations to assess skull or temporal bone (TB) abnormalities. In order to avoid radiation exposure in these young patients, efforts are made to assess the skull abnormalities on MR bone imaging sequences, such as Black Bone (BB). Our aim is to compare BB, a radiation-free imaging technique, with CT for the assessment of the TB. Materials and methods: 48 patients who underwent both BB and CT (2016–2021) in Sophia Children's Hospital, Erasmus MC, Rotterdam, were retrospectively investigated. BB and CT (the diagnostic gold standard for imaging the temporal bone) were evaluated blindly and independently by 3 observers; visibility and abnormalities of TB structures and cranial nerves were scored; abnormal findings were confirmed by a senior pediatric neuroradiologist. The statistical analysis was performed using Gwet's AC1 agreement and modified versions of the Wilcoxon signed-rank test and sign test with a Bonferroni-Holm correction (p < 0.05). Results: CT was rated higher than BB in structure visibility (global p = 0.0002), but was rated similar to BB when assessing TB anatomy and pathology (global p = 0.58). The visibility ratings showed better interobserver agreement values on CT than BB. In the normal/abnormal ratings, both BB (0.75-1) and CT (0.88-1) showed high interobserver agreement values. Conclusion: Our preliminary results suggest that BB is a promising tool for screening TB pathology in patients with craniosynostosis who require MR imaging. [ABSTRACT FROM AUTHOR]

Purpose: Trigonocephaly is associated with suboptimal visual outcomes in young children. However, the presence of orthoptic abnormalities after completed visual development remains unknown. The purpose of this study is to assess the prevalence of orthoptic abnormalities in trigonocephaly patients, after completed visual development. Methods: A retrospective study among non-syndromic trigonocephaly patients aged 8 years and older was conducted at the Dutch Craniofacial center. Ophthalmologic records were reviewed for refractive errors and a history of strabismus and amblyopia. Refractive errors were classified as follows: myopia: spherical equivalent (SE) ≤ -0.50 diopters (D), emmetropia: SE -0.50 D to + 0.50 D, hyperopia: ≥ + 0.50 D, astigmatism: cylinder error ≥ 1.00 D. The history of strabismus and amblyopia was assessed retrospectively. Prevalence and cumulative incidence of these abnormalities in healthy age-matched populations were retrieved from literature. Results: We included 78 trigonocephaly patients with a median (interquartile range) age of 10.0 (9.0–12.0) years. Thirty-four (43.6%) of these patients presented with a refractive error after the age of 8 years. Specific findings in these 78 patients versus controls were hyperopia in 35.9% vs 8.4%; myopia in 6.4% vs 11.4%; astigmatism in 20.5% vs 11.9%; strabismus in 14.1% vs 2.65%; amblyopia in 6.4% vs 3.4%. Conclusions: Trigonocephaly patients have a two-to-threefold higher risk of hyperopia, astigmatism, amblyopia, and strabismus, as compared to healthy populations. We recommend screening and, in case of abnormalities, regular follow-up before completed visual development. In addition, we recommend long-term surveillance of visual functioning by a pediatric ophthalmologist and/or orthoptist. [ABSTRACT FROM AUTHOR]

Objective: Stagnation of skull growth is correlated with papilledema in craniosynostosis. In this retrospective cohort study, we describe the postoperative skull growth after surgical correction for sagittal synostosis and its relation to the development of papilledema. Methods : Patients with isolated sagittal synostosis at our center between 2005 and 2012 were included. Occipitofrontal circumference (OFC) was analyzed, at 3 time points (preoperative, 2 years postoperative, and last OFC measurement) and 3 phases (initial postoperative growth, long-term growth, and overall growth), and related to papilledema on fundoscopy. Results : In total, 163 patients were included. The first time interval showed a decline in skull growth, with subsequent stabilization at long term. Papilledema occurred postoperatively in 10 patients. In these patients, the OFC at 2 years and at last follow-up (T3) were significantly smaller than in patients without papilledema. A larger OFC resulted in a decreased odds of developing papilledema at both postoperative time points (at T2 (OR = 0.40, p = 0.01) and at T3 (OR 0.29, p < 0.001)). Sensitivity and specificity analysis indicated that an OFC below 0.25 SD at T2 (sensitivity 90%, specificity 65%) and below 0.49 at T3 (sensitivity 100%, specificity 60%) are related to the occurrence of papilledema. Conclusion: A small OFC is correlated with the occurrence of papilledema. A decline in OFC within 2 years postoperatively is common in sagittal synostosis and is acceptable up to a value of 0.25SD. Patients with an OFC at last follow-up of less than 0.5SD are at risk for developing papilledema. [ABSTRACT FROM AUTHOR]

Purpose: To obtain pediatric normative reference values and determine whether optical coherence tomography (OCT) corresponds better with clinical signs of intracranial hypertension (ICH) compared to the traditional screening method fundoscopy in a large cohort of one type of single suture craniosynostosis. Methods: Control subjects without optic nerve diseases and isolated sagittal synostosis patients aged 3–10 years who underwent fundoscopy and OCT were included in this prospective cohort study. Normative reference values were obtained through bootstrap analysis. Main outcome was the association between peripapillary total retinal thickness (TRT) and total retinal volume (TRV) and appearance on fundoscopy. Signs and symptoms suggestive of ICH, including skull growth arrest, fingerprinting, and headache, were scored. Results: Sixty-four healthy controls and 93 isolated sagittal synostosis patients were included. Normative cut-off values for mean TRT are < 256 μm and > 504 μm and for mean TRV < 0.21 mm3 and > 0.39 mm3. TRT was increased in 16 (17%) and TRV in 15 (16%) of 93 patients, compared to only 4 patients with papilledema on fundoscopy (4%). Both parameters were associated with papilledema on fundoscopy (OR = 16.7, p = 0.02, and OR = 18.2, p = 0.01). Skull growth arrest was significantly associated with abnormal OCT parameters (OR = 13.65, p < 0.01). Conclusions: The established cut-off points can be applied to screen for ICH in pediatrics. The present study detected abnormalities with OCT more frequent than with fundoscopy, which were associated with skull growth arrest. Therefore, a combination of OCT, fundoscopy, and skull growth arrest can improve clinical decision-making in craniosynostosis. [ABSTRACT FROM AUTHOR]

Isolated synostosis of the frontosphenoidal suture is very rare and difficult to diagnose. Little has been reported on the clinical presentation and fetal development of this suture. To understand the development of the frontosphenoidal suture and the outcome of its synostosis. We studied the normal fetal development of the frontosphenoidal suture in dry human skulls and the clinical features of four patients with isolated synostosis of the frontosphenoidal suture. The frontosphenoidal suture develops relatively late during the second trimester of pregnancy, which explains the mild phenotype when there is synostosis. This rare craniosynostosis results in a deformity that causes recession of the lateral part of the frontal bone and supraorbital rim, with minimal facial asymmetry. Three-dimensional CT is the best examination to confirm the diagnosis. Isolated frontosphenoidal synostosis should be considered in patients with unilateral flattening of the forehead at birth that does not improve within the first few months of life. [ABSTRACT FROM AUTHOR]