Background: Craniosynostosis may lead to elevated intracranial pressure, which may be implicated with impaired neurocognitive development. However, accurately measuring intracranial pressure is challenging, and patterns in craniosynostosis patients are poorly characterized. Spectral-domain optical coherence tomography may enable noninvasive assessment of intracranial pressure in pediatric patients with craniosynostosis. Methods: Pediatric patients with craniosynostosis undergoing surgical intervention between 2014 and 2019 prospectively underwent optical coherence tomographic evaluation. Intracranial pressure was directly measured intraoperatively in a subset of cases. Optical coherence tomographic parameters were compared to directly measured intracranial pressure and used for pattern assessment. Results: Optical coherence tomography was performed in 158 subjects, among which 42 underwent direct intracranial pressure measurement during an initial cranial procedure. Maximal retinal nerve fiber layer thickness, maximal retinal thickness, and maximal anterior projection optical coherence tomographic parameters were positively correlated with intracranial pressure (p ≤ 0.001), with all parameters showing significantly higher values in patients with intracranial pressure thresholds of 15 mmHg (p < 0.001) and 20 mmHg (p ≤ 0.007). Patients with maximal retinal nerve fiber layer thickness and maximal anterior projection exceeding set thresholds in optical coherence tomography of either eye demonstrated 77.3 percent sensitivity and 95.0 percent specificity for detecting intracranial pressure above 15 mmHg, and 90.0 percent sensitivity and 81.3 percent specificity for detecting intracranial pressure above 20 mmHg. Patients with associated syndromes or multiple suture involvement and patients aged 9 months or older were significantly more likely to have elevated intracranial pressure above 15 mmHg (p ≤ 0.030) and above 20 mmHg (p ≤ 0.035). Conclusions: Spectral-domain optical coherence tomography can noninvasively detect elevated intracranial pressure in patients with craniosynostosis with reliable sensitivity and specificity. This technology may help guide decisions about the appropriate type and timing of surgical treatment. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, I.

OBJECTIVE: Children with multiple prematurely fused cranial sutures and those undergoing surgical correction later in life appear to experience worse neurocognitive outcomes, but it is unclear whether higher intracranial pressure (ICP) is implicated in this process. The purpose of this study was to elucidate the effect of age at intervention and number of involved cranial sutures on ICP, as well as to assess which cranial suture closure may be more associated with elevated ICP. METHODS: The prospective craniofacial database at the authors’ institution was queried for patients undergoing initial corrective surgery for craniosynostosis in whom intraoperative measurement of ICP was obtained prior to craniectomy. Age, involved sutures, and syndromic status were analyzed in the context of measured ICP by using multiple linear regression. RESULTS: Fifty patients met the inclusion criteria. Age at procedure (p = 0.028, β = +0.060 mm Hg/month) and multiple-suture involvement (p = 0.010, β = +4.175 mm Hg if multisuture) were both significantly implicated in elevated ICP. The actual number of major sutures involved was significantly correlated to ICP (p = 0.001; β = +1.687 mm Hg/suture). Among patients with single-suture involvement, there was an overall significant difference of median ICP across the suture types (p = 0.008), with metopic having the lowest (12.5 mm Hg) and sagittal having the highest (16.0 mm Hg). Patients with multiple-suture involvement had significantly higher ICP (p = 0.003; 18.5 mm Hg). Patients with craniofacial syndromes were 79.3 times more likely to have multiple-suture involvement (p < 0.001). Corrective surgery for craniosynostosis demonstrated significant intraoperative reduction of elevated ICP (all p < 0.050). CONCLUSIONS: Syndromic status, older age at intervention for craniosynostosis, and multiple premature fusion of cranial sutures were associated with significantly higher ICP.

Background: Fusion of cranial-base sutures/synchondroses presents a clinical conundrum, given their often unclear “normal” timing of closure. This study investigates the physiologic fusion timelines of cranial-base sutures/synchondroses. Methods: Twenty-three age intervals were analyzed in subjects aged 0 to 18 years. For each age interval, 10 head computed tomographic scans of healthy subjects were assessed. Thirteen cranial-base sutures/synchondroses were evaluated for patency. Partial closure in greater than or equal to 50 percent of subjects and complete bilateral closure in less than 50 percent of subjects defined the fusion “midpoint.” Factor analysis identified clusters of related fusion patterns. Results: Two hundred thirty scans met inclusion criteria. The sutures’ fusion midpoints and completion ages, respectively, were as follows: frontoethmoidal, 0 to 2 months and 4 years; frontosphenoidal, 6 to 8 months and 12 years; and sphenoparietal, 6 to 8 months and 4 years. Sphenosquamosal, sphenopetrosal, parietosquamosal, and parietomastoid sutures reached the midpoint at 6 to 8 months, 8 years, 9 to 11 months, and 12 years, respectively, but rarely completed fusion. The occipitomastoid suture partially closed in less than or equal to 30 percent of subjects. The synchondroses’ fusion midpoints and completion ages, respectively, were as follows: sphenoethmoidal, 3 to 5 months and 5 years; spheno-occipital, 9 years and 17 years; anterior intraoccipital, 4 years and 10 years; and posterior intraoccipital, 18 to 23 months and 4 years. The petro-occipital synchondrosis reached the midpoint at 11 years and completely fused in less than 50 percent of subjects. Order of fusion of the sutures, but not the synchondroses, followed the anterior-to-posterior direction. Factor analysis suggested three separate fusion patterns. Conclusions: The fusion timelines of cranial-base sutures/synchondroses may help providers interpret computed tomographic data of patients with head-shape abnormalities. Future work should elucidate the mechanisms and sequelae of cranial-base suture fusion that deviates from normal timelines.

Background: There have been few longitudinal studies assessing the effect of preoperative phenotypic severity on long-term esthetic outcomes in metopic craniosynostosis. This study evaluates the relationship between metopic severity and long-term esthetic outcomes using interfrontal angle (IFA) and CranioRate, a novel metopic synostosis severity measure. Methods: Patients with metopic craniosynostosis who underwent bifrontal orbital advancement and remodeling between 2012 and 2017 were reviewed. Preoperative computed tomography head scans were analyzed for IFA and CranioRate, a machine learning algorithm which generates quantitative severity ratings including metopic severity score (MSS) and cranial morphology deviation (CMD). Long-term esthetic outcomes were assessed by craniofacial surgeons using blinded 3-rater esthetic grading of clinical photos. Raters assessed Whitaker score and the presence of temporal hollowing, lateral orbital retrusion, frontal bone irregularities and/or “any visible irregularities.” Results: Preoperative scans were performed at a mean age of 7.7±3.4 months, with average MSS of 6/10, CMD of 200/300, and IFA of 116.8±13.8 degrees. Patients underwent bifrontal orbital advancement and remodeling at mean 9.9±3.1 months. The average time from operation to esthetic assessment was 5.4±1.0 years. Pearson correlation revealed a significant negative correlation between MSS and age at computed tomography (r=−0.451, P=0.004) and IFA (r=−0.371, P=0.034) and between IFA and age at surgery (r=−0.383, P=0.018). In multinomial logistic regression, preoperative MSS was the only independent predictor of visible irregularities (odds ratio=2.18, B=0.780, P=0.024) and preoperative IFA alone significantly predicted Whitaker score, with more acute IFA predicting worse Whitaker score (odds ratio=0.928, B=−0.074, P=0.928). Conclusions: More severe preoperative phenotypes of metopic craniosynostosis were associated with worse esthetic dysmorphology. Objective measures of preoperative metopic severity predicted long-term esthetic outcomes.

Objective: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). Design: Cross-sectional retrospective chart review. Setting: Tertiary care pediatric hospital. Patients/Participants: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. Main Outcome Measures: CLEFT-Q. Results: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. Conclusions: Patient perception of cleft outcomes is linked to psychosocial factors.