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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 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.

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 504 μm and for mean TRV 3 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 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.

Background: Multiple pregnancy is a known predisposing factor for sagittal suture synostosis, and intrauterine constraint has been suggested as a potential cause. This study evaluates prenatal images of single and multiple pregnancies of children with sagittal suture synostosis to assess the possible pathogenesis. Materials and Methods: Prenatal ultrasound data of scaphocephaly twins treated at Erasmus MC was retrieved retrospectively. The head circumference, cephalic index (CI), and biparietal diameter (BPD) were analyzed. Data were compared with the nonaffected sibling, singletons with scaphocephaly, and to general fetal growth reference values. Results: Data of 8 twin pregnancies with 10 cases of scaphocephaly was gathered. For the head circumference no difference was found between affected fetuses and reference values throughout pregnancy. For the BPD a reduced growth was found, compared with control values. The growth curve for the CI of the affected twins is lower from the start and declines over time. When comparing affected fetuses to nonaffected fetuses, the same differences for the BPD and CI were found as compared with controls. No differences in growth parameters were found between the nonaffected siblings and reference values, nor between the affected scaphocephaly twins and singletons with scaphocephaly. Conclusions: Scaphocephaly in twin pregnancy develops early on in pregnancy, similar to singleton pregnancy, making the theory of constraint as a cause less likely. The development of scaphocephaly in twins is likely to be attributed to the same disturbed developmental processes in suture development as in singletons, and not to mechanical constraint.

Background: Craniofacial surgery is the standard treatment for children with moderate to severe trigonocephaly. The added value of surgery to release restriction of the frontal lobes is unproven, however. In this study, the authors aim to address the hypothesis that the frontal lobe perfusion is not restricted in trigonocephaly patients by investigating cerebral blood flow. Methods: Between 2018 and 2020, trigonocephaly patients for whom a surgical correction was considered underwent magnetic resonance imaging brain studies with arterial spin labeling to measure cerebral perfusion. The mean value of cerebral blood flow in the frontal lobe was calculated for each subject and compared to that of healthy controls. Results: Magnetic resonance imaging scans of 36 trigonocephaly patients (median age, 0.5 years; interquartile range, 0.3; 11 female patients) were included and compared to those of 16 controls (median age, 0.83 years; interquartile range, 0.56; 10 female patients). The mean cerebral blood flow values in the frontal lobe of the trigonocephaly patients (73.0 ml/100 g/min; SE, 2.97 ml/100 g/min) were not significantly different in comparison to control values (70.5 ml/100 g/min; SE, 4.45 ml/100 g/min; p = 0.65). The superior, middle, and inferior gyri of the frontal lobe showed no significant differences either. Conclusions: The authors' findings suggest that the frontal lobes of trigonocephaly patients aged less than 18 months have a normal cerebral blood flow before surgery. In addition to the very low prevalence of papilledema or impaired skull growth previously reported, this finding further supports the authors' hypothesis that craniofacial surgery for trigonocephaly is rarely indicated for signs of raised intracranial pressure or restricted perfusion for patients younger than 18 months. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Introduction: Robin Sequence (RS) is characterized by retrognathia, glossoptosis, and upper airway obstruction (UAO). Nonetheless, the exact role of retrognathia in RS remains unclear. The aim of this study was to evaluate if measuring retrognathia could be of use next to the polysomnography (PSG) in the diagnosis of RS by differentiating RS patients and controls. Furthermore, this study endeavored to evaluate mandibular growth over time. Methods: A retrospective study was performed on RS patients without life-threatening UAO and controls. Mandibular length was assessed by using the Nasion-Mandibula Ratio (NMRatio) at the following three time points: 0 to 3 months, 1 year, and 4 years of age. Results: A total of 107 patients were included of whom 38 were diagnosed with RS. Thirty-two patients presented with clinical retrognathia but without obstructive sleep apnea (OSA), whilst 37 patients had an isolated cleft palate (CP). At 0 to 3 months, 1 year, and 4 years of age, significant higher NMRatios were found in RS patients compared to isolated CP patients, whilst no significant differences were found compared to retrognathia-only patients. Within RS patients, no significant differences were found between isolated versus non-isolated and invasively versus non-invasively treated patients. In all three patient groups, a significant decrease of the NMRatio was seen at the age of 4 years compared to 0 to 3 months and 1 year of age. Conclusions: Although the NMRatio differs between RS and isolated CP patients, a seemingly small mandible (in a-p direction) does not reflect functional outcomes in RS patients and a PSG should always be performed to confirm the diagnosis. Despite that mandibular length significantly increased after the first year of life, mandibular size of RS patients does not seem to reach values of normal infants. Accordingly, mandibular catch up growth, that is suggested to be typically present in RS patients, was not found by our study.

Study Objectives: Obstructive sleep apnea (OSA) is seen in up to two-Third of the patients with syndromic craniosynostosis. Gold standard to diagnose OSA is the hospital-based polysomnography, although alternatively ambulatory home sleep apnea devices are available. Aim of this study was to assess (1) accuracy of ambulatory sleep studies, (2) clinical decision making following sleep studies, and (3) course of OSA during long-Term follow-up. Materials and Methods: A retrospective cohort study was performed in children with syndromic craniosynostosis, of whom polysomnographies and home sleep apnea device recordings were collected. Measurements of apnea-hypopnea index, respiratory event index, total sleep/recording time, heart rate, oxygen saturation, and oxygen desaturation index were derived from the sleep studies. Primary clinical care subsequent to the sleep studies was determined using electronic patient files. Results: In total, 123 patients were included, with 149 polysomnographies and 108 ambulatory studies. Performing an ambulatory study was associated with increased age at time of measurement (OR=1.1, 95% CI=1.02 to 1.17, P=0.01). No significant difference was found between the 2 types of sleep studies regarding sleep study parameters. Subsequent to sleep studies, patients with no-mild OSA had expectant care whereas patients with moderate-severe OSA underwent OSA-related treatment. OSA was most prevalent up to the age of 5 years, but also noticeable after the age of 10 years in patients with the Crouzon syndrome. Conclusions: Ambulatory sleep studies are reliable for diagnosing OSA in older children and can be used to determine clinical decision-making. Hence, we recommend implementing ambulatory sleep studies in a protocolized management.

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.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.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 mmThe 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.

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.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.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.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.

Spatial normalization is an important step for group image processing and evaluation of mean brain perfusion in anatomical regions using arterial spin labeling (ASL) MRI and is typically performed via high-resolution structural brain scans. However, structural segmentation and/or spatial normalization to standard space is complicated when gray-white matter contrast in structural images is low due to ongoing myelination in newborns and infants. This problem is of particularly clinical relevance for imaging infants with inborn or acquired disorders that impair normal brain development. We investigated whether the ASL MRI perfusion contrast is a viable alternative for spatial normalization, using a pseudo-continuous ASL acquired using a 1.5 T MRI unit (GE Healthcare). Four approaches have been compared: (1) using the structural image contrast, or perfusion contrast with (2) rigid, (3) affine, and (4) nonlinear transformations – in 16 healthy controls [median age 0.83 years, inter-quartile range (IQR) ± 0.56] and 36 trigonocephaly patients (median age 0.50 years, IQR ± 0.30) – a non-syndromic type of craniosynostosis. Performance was compared quantitatively using the real-valued Tanimoto coefficient (TC), visually by three blinded readers, and eventually by the impact on regional cerebral blood flow (CBF) values. For both patients and controls, nonlinear registration using perfusion contrast showed the highest TC, at 17.51 (CI 6.66–49.38) times more likely to have a higher rating and 17.45–18.88 ml/100 g/min higher CBF compared with the standard normalization. Using perfusion-based contrast improved spatial normalization compared with the use of structural images, significantly affected the regional CBF, and may open up new possibilities for future large pediatric ASL brain studies.

Background Surgery for sagittal synostosis aims at correction of skull shape and restoration of growth potential. Small cranial volume is associated with raised intracranial pressure (ICP). Although many techniques have been described, information on postoperative volume related to early and late remodeling is lacking. Methods Between 2004 and 2008, a total of 95 patients were collected who underwent either early extended strip craniectomy or late total cranial remodeling according to age of presentation. Volume was measured on three-dimensional (3D) photogrammetry. Volume measurements were related to cranial index (CI), head circumference (HCsd), and signs of raised ICP. In a small subset of patients, volume measurements on 3D photogrammetry were assessed for inter- and intrarater reliability and compared to 3D computed tomography (CT). Results Volume was increased in all patients before and after surgery compared to normative values. Postoperatively, late total cranial remodeling resulted in a slightly larger volume than early extended strip craniectomy. Volume measurements showed a good correlation with HCsd (0.67) and a poor relationship with CI (0.13). Headache occurred more frequently in patients with a lower cranial volume. Although papilledema and reoperation showed the same trend, the numbers were too small for statistical analysis. Reproducibility of volume measurements on 3D photogrammetry was high, as was the correlation with measurements on CT. Conclusion Late total cranial remodeling results in a larger postoperative volume, as measured on 3D photogrammetry, than extended strip craniectomy. Clinical signs of raised ICP occur more frequently in patients with a smaller volume. To measure volume, 3D photogrammetry is a good alternative to CT.

Craniosynostosis, the premature ossification of cranial sutures, is a developmental disorder of the skull vault, occurring in approximately 1 in 2250 births. The causes are heterogeneous, with a monogenic basis identified in ~25% of patients. Using whole-genome sequencing, we identified a novel, de novo variant in BCL11B, c.7C>A, encoding an R3S substitution (p.R3S), in a male patient with coronal suture synostosis. BCL11B is a transcription factor that interacts directly with the nucleosome remodelling and deacetylation complex (NuRD) and polycomb-related complex 2 (PRC2) through the invariant proteins RBBP4 and RBBP7. The p.R3S substitution occurs within a conserved amino-terminal motif (RRKQxxP) of BCL11B and reduces interaction with both transcriptional complexes. Equilibrium binding studies and molecular dynamics simulations show that the p.R3S substitution disrupts ionic coordination between BCL11B and the RBBP4–MTA1 complex, a subassembly of the NuRD complex, and increases the conformational flexibility of Arg-4, Lys-5 and Gln-6 of BCL11B. These alterations collectively reduce the affinity of BCL11B p.R3S for the RBBP4–MTA1 complex by nearly an order of magnitude. We generated a mouse model of the BCL11B p.R3S substitution using a CRISPR-Cas9-based approach, and we report herein that these mice exhibit craniosynostosis of the coronal suture, as well as other cranial sutures. This finding provides strong evidence that the BCL11B p.R3S substitution is causally associated with craniosynostosis and confirms an important role for BCL11B in the maintenance of cranial suture patency.

Background: This study investigates the relationship between headache and the occurrence of signs associated with intracranial hypertension such as ophthalmic signs, restricted skull growth, and a vertex bulge in children who were operated on for sagittal synostosis. Methods: A total of 94 patients (aged 6 to 18 years) with sagittal synostosis were asked to indicate their headache frequency. Based on their age at referral, the patients had undergone either frontobiparietal remodeling or an extended strip craniotomy. Data on funduscopy, optical coherence tomography, occipitofrontal head circumference, and presence of vertex bulge on radiography were collected retrospectively. Results: Univariate analysis showed that extended strip craniotomy, the occurrence of ophthalmic signs, and a smaller occipitofrontal head circumference at last follow-up were related to more frequent headaches (p = 0.01, p = 0.04, and p < 0.01, respectively). On multivariate analysis, only type of surgery and occipitofrontal head circumference at last follow-up remained significant predictors (p = 0.04 and p < 0.01, respectively). Conclusions: Although the reported rate of frequent headaches in this study is within the norm reported for the normal population, this study shows that after correction for sagittal craniosynostosis, frequent headaches are independently related to type of surgery and to occipitofrontal head circumference at last follow-up. Headaches in the sagittal craniosynostosis population may be related to papilledema and/or an increased total retinal thickness. Therefore, the authors recommend that occipitofrontal head circumference be routinely measured and that patients be asked about the occurrence and frequency of headaches during their checkup at the clinic. (Plast. Reconstr. Surg. 143: 798e, 2019.)

Objective: Patients with craniosynostosis syndromes are at risk of increased intracranial pressure (ICP) and Chiari I malformation (CMI), caused by a combination of restricted skull growth, venous hypertension, obstructive sleep apnea (OSA), and an overproduction or insufficient resorption of cerebrospinal fluid. This study evaluates whether craniosynostosis patients with CMI have an imbalance between cerebellar volume (CV) and posterior fossa volume (PFV), that is, an overcrowded posterior fossa. Methods: Volumes were measured in 3D-SPGR TI-weighted MR scans of 28 'not-operated' craniosynostosis patients (mean age: 4.0 years; range: 0-14), 85 'operated' craniosynostosis patients (mean age: 8.0 years; range: 1-18), and 34 control subjects (mean age: 5.4 years; range: 0-15). Volumes and CV/PFV ratios were compared between the operated and not-operated craniosynostosis patients, between the individual craniosynostosis syndromes and controls, and between craniosynostosis patients with and without CMI. Data were logarithmically transformed and studied with analysis of covariance (ANCOVA). Results: The CV, PFV, and CV/PFV ratios of not-operated craniosynostosis patients and operated craniosynostosis patients were similar to those of the control subjects. None of the individual syndromes was associated with a restricted PFV. However, craniosynostosis patients with CMI had a significantly higher CV/PFV ratio than the control group (0.77 vs. 0.75; p = 0.008). The range of CV/PFV ratios for craniosynostosis patients with CMI, however, did not exceed the normal range. Conclusion: Volumes and CV/PFV ratio cannot predict which craniosynostosis patients are more prone to developing CMI than others. Treatment should focus on the skull vault and other contributing factors to increased ICP, including OSA and venous hypertension. (C) 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Background: The application of a new approach is presented, percutaneous aponeurotomy and lipofilling, which is a minimally invasive, incisionless alter native to traditional flap reconstructions. Methods: The restrictive subdermal cicatrix and/or endogenous aponeurosis is punctured, producing staggered nicks. Expansion of the restriction reconstructs the defect and creates a vascularized scaffold with micro-openings that are seeded with lipografts. Wide subcutaneous cuts that lead to macrocavities and subsequent graft failure are avoided. Postoperatively, a splint to hold open the neomatrix/graft construct in its expansive state is applied until the grafts mature. Thirty-one patients underwent one to three operations (average, two) for defects that normally require flap tissue transfer: wounds where primary closure was not possible (n = 9), contour defects of the trunk and breast requiring large-volume fat grafts (n = 8), burn contractures (n = 5), radiation scars (n = 6), and congenital constriction bands (n = 3). Results: The regenerated tissue was similar in texture and consistency to the surrounding tissues. Wider meshed areas had greater tissue gain (range, 20 to 30 percent). There were no significant wound-healing issues, scars, or donorsite morbidities. Advancement tension was relieved without flap undermining or decreased perfusion. Conclusions: Realizing that, whether scar or endogenous fascia, the subdermal aponeurosis limits tissue stretch and/or its three-dimensional expansion, a minimally invasive procedure that expands this cicatrix into a matrix ideally suited for fat micrografts was developed. Grafting this scaffold applies tissueengineering principles to generate the needed tissue and represents a regenerative alternative to reconstructive flap surgery. (Plast. Reconstr. Surg. 132: 1280, 2013.) CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

The monobloc advancement with distraction is a treatment modality for syndromic craniosynostosis, to correct exorbitism, upper airway compromise and malocclusion. In this report orbital volume and movements of (peri-)orbital structures and globes of seven patients following monobloc distraction are evaluated. In preoperative and postoperative CT-scans orbital volume was assessed and a 3D coordinate system with eleven landmarks was used to measure the movements of orbital structures and globes and to measure the change of exorbitism. Correlation between orbital volume, movements of the orbital structures and change in exorbitism was studied. The orbital volume increased by 49.9% (left) and 50.4% (right). The average anterior movement of the bone was 13.6 mm (left) and 13.9 mm (right). The mean anterior movement of the left globe was 5.8 mm and of the right globe 5.3 mm. The ratio of globe/bone movement was 0.4. Exorbitism decreased with 7.8 mm (left) and 8.1 mm (right). Differences between left and right for orbital volume, for movements and for the decrease in exorbitism were not significant. Volume enlargement and decrease in exorbitism were correlated (p ≤ 0.05). Following monobloc advancement orbital volume increases, (peri-)orbital structures and the globe move forward and exorbitism diminishes.

Trigonocephaly is caused by metopic suture synostosis. It is treated by fronto-orbital remodeling, not only to correct the deformity but also to prevent intracranial hypertension, the reported prevalence in trigonocephaly of which ranges from 0 to 33 percent. To support treatment analysis and the design of a treatment protocol for intracranial hypertension in these patients, the authors wished to more accurately quantify the prevalence of preoperative and postoperative intracranial hypertension in a large patient cohort.The authors included all trigonocephaly patients born between 2001 and 2013 who had all been operated on at a single center. During follow-up, the presence of intracranial hypertension was evaluated by funduscopy, and occipitofrontal head circumference was measured. The occipitofrontal head circumference curve was analyzed and its relation to intracranial hypertension assessed.In total, 262 patients with trigonocephaly were included. Before surgery, 1.9 percent of them had intracranial hypertension; after surgery, 1.5 percent did (mean age at last follow-up, 4.9 years). Sixteen of 176 patients (9 percent) had occipitofrontal head circumference curve stagnation, which was significantly related to intracranial hypertension (p = 0.001, Fisher's exact test).Intracranial hypertension occurs only sporadically in patients with metopic suture synostosis. Occipitofrontal head circumference measurement should take a prominent place in the postoperative follow-up of metopic suture synostosis patients; stagnation of the occipitofrontal head circumference requires additional screening for intracranial hypertension.Therapeutic, IV.

Background: Children with Apert and Crouzon-Pfeiffer syndromes are at risk of intracranial hypertension. Until 2005, when the authors switched to occipital expansion, their institution's preferred treatment was fronto-orbital advancement. However, it was still unclear whether (1) occipitofrontal head circumference (i.e., intracranial volume) was greater after occipital expansion than after fronto-orbital advancement; (2) the incidences of tonsillar herniation and papilledema were lower; and (3) visual acuity was better during follow-up. In these patients, the authors therefore compared fronto-orbital advancement with occipital expansion as the first surgical procedure. Methods: Measurements included repeated occipitofrontal head circumference as a measure for intracranial volume; neuroimaging to evaluate tonsillar herniation; funduscopy to identify papilledema; and visual acuity testing. Results: The authors included 37 patients (Apert syndrome, n = 18; Crouzon-Pfeiffer syndrome, n = 19). Eighteen underwent fronto-orbital advancement and 19 underwent occipital expansion (age at surgery, 1.0 versus 1.5 years; p = 0.13). Follow-up time in both groups was 5.7 years. The increase in occipitofrontal head circumference (+1.09 SD) was greater after occipital expansion than after fronto-orbital advancement (+0.32 SD) (p = 0.03). After occipital expansion, fewer patients with Crouzon-Pfeiffer syndrome had tonsillar herniation (occipital, three of 11; fronto-orbital advancement, seven of eight; p = 0.02); for both syndromes together, fewer patients had papilledema (occipital, four of 19; fronto-orbital advancement, 11 of 18; p = 0.02). Visual acuity was similar after fronto-orbital advancement and occipital expansion (0.09 versus 0.13 logarithm of the minimum angle of resolution) (p = 0.28). Conclusions: The authors' preference for occipital expansion as the initial craniofacial procedure in Apert and Crouzon-Pfeiffer syndromes is supported by the greater increase it produces in intracranial volume (as evidenced by the occipitofrontal head circumference), which reduces the incidences of tonsillar herniation and papilledema.

OBJECT Various techniques to correct sagittal synostosis have been described. The authors of this study assess the results of 2 techniques for late complete cranial remodeling and test the hypothesis that adding a widening bridge would improve outcome. METHODS In this retrospective study, the authors evaluated patients with nonsyndromic sagittal synostosis—those who underwent frontobiparietal remodeling (FBR) and those who underwent modified FBR (MFBR) involving the introduction of a bony bridge to increase the width of the skull. Outcomes for both groups are described in terms of the aesthetic results assessed on photographs and any changes in the cranial index (CI) and head circumference over time, the presence of papilledema, and complaints of headache. The effect of the surgical technique on CI and head circumference over time was assessed using linear regression analysis, with adjustment for preoperative CI and head circumference. RESULTS Sixty-nine patients with isolated sagittal synostosis were included in this study: 35 underwent MFBR and 34 underwent the original technique of FBR. The mean follow-up period was 7 years. In the 1st year after surgery, mean CI improved by 9% in the FBR group and by 12% in the MFBR group. One year after surgery, CI in the MFBR group was on average 4.7% higher than that in the FBR group (p < 0.001). During follow-up, CI decreased in both groups; however, at all time points CI was significantly higher in the MFBR group than in the FBR group. The impact of surgical technique on CI was less important than the impact of preoperative CI (R2= 0.26 vs 0.54), and this applied at all time points during follow-up. Head circumference declined during follow-up in both groups. It was influenced by preoperative head circumference, but not by surgical technique. Aesthetic outcome, prevalence of headache (42%), and papilledema (7%) were comparable in both groups. CONCLUSIONS Adding a widening bridge to late complete remodeling significantly improved CI and helped to prevent CI from decreasing in the long term. This addition did not affect the head circumference growth curve. Despite a mean head circumference remaining at +1 SD, patients continued to develop papilledema postoperatively (7%).

OBJECT Patients with syndromic and complex craniosynostosis are characterized by the premature fusion of one or more cranial sutures. These patients are at risk for developing elevated intracranial pressure (ICP). There are several factors known to contribute to elevated ICP in these patients, including craniocerebral disproportion, hydrocephalus, venous hypertension, and obstructive sleep apnea. However, the causal mechanism is unknown, and patients develop elevated ICP even after skull surgery. In clinical practice, the occipitofrontal circumference (OFC) is used as an indirect measure for intracranial volume (ICV), to evaluate skull growth. However, it remains unknown whether OFC is a reliable predictor of ICV in patients with a severe skull deformity. Therefore, in this study the authors evaluated the relation between ICV and OFC. METHODS Eighty-four CT scans obtained in 69 patients with syndromic and complex craniosynostosis treated at the Erasmus University Medical Center-Sophia Children’s Hospital were included. The ICV was calculated based on CT scans by using autosegmentation with an HU threshold < 150. The OFC was collected from electronic patient files. The CT scans and OFC measurements were matched based on a maximum amount of the time that was allowed between these examinations, which was dependent on age. A Pearson correlation coefficient was calculated to evaluate the correlations between OFC and ICV. The predictive value of OFC, age, and sex on ICV was then further evaluated using a univariate linear mixed model. The significant factors in the univariate analysis were subsequently entered in a multivariate mixed model. RESULTS The correlations found between OFC and ICV were r = 0.908 for the total group (p < 0.001), r = 0.981 for Apert (p < 0.001), r = 0.867 for Crouzon-Pfeiffer (p < 0.001), r = 0.989 for Muenke (p < 0.001), r = 0.858 for Saethre- Chotzen syndrome (p = 0.001), and r = 0.917 for complex craniosynostosis (p < 0.001). Age and OFC were significant predictors of ICV in the univariate linear mixed model (p < 0.001 for both factors). The OFC was the only predictor that remained significant in the multivariate analysis (p < 0.001). CONCLUSIONS The OFC is a significant predictor of ICV in patients with syndromic and complex craniosynostosis. Therefore, measuring the OFC during clinical practice is very useful in determining which patients are at risk for impaired skull growth.

The chances of success are better for 'beautiful people': a youthful, pretty and symmetrical face with the ideal proportions offers better prospects of social success. However, success does not equal happiness. Television shows such as Make Me Beautiful suggest that aesthetic treatments guarantee increased happiness. Hardly any attention is being paid to the possibility that significant complications may be associated with these treatments, and the potentially unrealistic expectations of the person involved are often totally ignored. Satisfaction with appearance is not easy to achieve and requires more from the physician than just performing a procedure correctly. The patient needs to realise that an aesthetic treatment has its limits in relation to achieving happiness.

Children with syndromic or complex craniosynostosis are evaluated for increased intracranial pressure (ICP) using funduscopy to detect papilledema. However, papilledema is a late sign of increased ICP. Because papilledema might be preceded by an increase in optic nerve sheath (ONS) diameter, the authors conducted a prospective study to establish the validity and applicability of measuring the ONS using ultrasonography.From January 2007 to December 2009, 175 bilateral ultrasonography ONS measurements were performed in 128 patients with syndromic or complex craniosynostosis during the daytime. The measurements were correlated with ONS diameter assessed on CT and simultaneous funduscopy, when available. Furthermore, results were compared by using thresholds for ONS diameters on ultrasonography that are available in the literature.The mean ONS diameter on ultrasonography was 3.1 ± 0.5 mm. The CT measurement was significantly correlated with the ultrasonography measurement (r = 0.41, p0.001). The mean ONS diameter in 38 eyes with papilledema was 3.3 ± 0.5 mm, compared with 3.1 ± 0.5 mm in the eyes of patients without papilledema (p = 0.039). Relative to the age-related thresholds, the ONS diameter was too large in 11 eyes (3%), particularly in patients with Crouzon syndrome. Compared with funduscopy, ultrasonography sensitivity was 11%, specificity was 97%, and positive and negative predictive values were 40% and 86%, respectively.Ultrasonography is a valid and easy way of quantifying the ONS. Although the ONS diameter is larger in children with papilledema, it cannot be used as a daytime screening tool instead of funduscopy. The ONS diameter is possibly a more real-time indicator of ICP.