Search

Background: Craniofacial asymmetry associated with unicoronal synostosis (UCS) may persist into the teenage years despite surgery in infancy. This study evaluated outcomes following a nasal monobloc procedure by mobilizing a united nasomaxillary and bilateral medial orbital segment of bone (nasal monobloc) to perform corrective translational and rotational movement for secondary correction of residual nasal-orbital asymmetry associated with UCS., Methods: A retrospective review of all UCS patients treated with nasal monobloc at our institution was performed. Demographic information was recorded, and pre- and postoperative 2D imaging was used for morphometric outcome analysis. Outcomes and complications were tabulated., Results: The study included 14 patients (5 males, 9 females; mean age 14.6 years; range 9.6 to 22.5 years; mean follow-up 70.6 months range 12 to 132 months). Ancillary procedures (scar revision, forehead/orbital contouring, MEDPOR® augmentation) were performed in all patients at the time of the nasal monobloc. One patient underwent a repeat procedure 6 years later following technique modification. Additionally, another patient experienced late overgrowth of the frontal sinus with forehead asymmetry. The morphometric analysis demonstrated significant (p < 0.05) pre-op to post-op improvements in naso-orbital asymmetry, as demonstrated by horizontal orbital aperture ratio (0.88 vs 0.99), midline to exocanthion ratio (0.91 vs 0.98), orbital index ratio (1.15 vs 1.01), and midline discrepancy (7.1 degrees vs 2.7 degrees)., Conclusion: Nasal monobloc osteotomy provides a reasonable surgical treatment to improve both the nasal and orbital asymmetries associated with unicoronal synostosis, including frontal nasal deviation, basal nasal deviation, and orbital aperture asymmetry. It is important to note that confounding anatomic variables such as globe dystopia, strabismus, and scleral show may affect the perception of orbital symmetry., (Crown Copyright © 2024. Published by Elsevier Ltd. All rights reserved.)

Introduction: This cohort study aimed to elucidate the caregiver burden of helmet therapy (HT), following endoscopic strip craniectomy (ESC) to treat craniosynostosis, in an effort to inform clinicians and future caregivers navigating this therapeutic option., Methods: Fourteen caregivers of children with positional plagiocephaly (6) and craniosynostosis treated by ESC (8) undergoing HT at a single center were recruited via convenience sampling. Using a phenomenological qualitative approach, semi-structured interviews were conducted to understand the experience of HT for caregivers. Data collection and analysis were iterative and conducted until thematic saturation was reached., Results: Emerging themes revealed five domains of caregiver burden: emotional, cognitive, physical, psychosocial, and financial. No caregiver felt the therapy was too burdensome to complete. Caregivers of both groups also expressed positive aspects of HT related to support from the team, the noninvasive nature of treatment, and the outcomes of therapy. Furthermore, caregivers report overall satisfaction with the process, stating willingness to repeat the treatment with subsequent children if required., Conclusion: HT is associated with five major domains of caregiver burden; however, none of the caregivers regret choosing this treatment option, nor was the burden high enough to encourage treatment cessation. This study will inform future prospective analyses that will quantify real-time caregiver burden throughout HT., (© 2024 S. Karger AG, Basel.)

Background: Turribrachycephaly is a common feature in many syndromic and multisuture craniosynostoses and is traditionally treated with total cranial vault reshaping and fronto-orbital advancement. A staged approach with posterior cranial vault distraction as a primary procedure followed by anterior cranial vault reshaping has the advantage of reducing the vertical dimension of the skull in a controlled and gradual manner while expanding the cranial volume. The purpose of this study was to evaluate outcomes following posterior cranial vault expansion using distraction osteogenesis at a single tertiary pediatric center., Methods: This retrospective review included all cases of posterior cranial vault distraction at a single institution from 2008 to 2022 performed by one surgeon. Morphometric outcomes such as Turricephaly Index (TI) and posterior cranial volume (PCV) were assessed from pre- and postoperative computerized tomography scans for patients who underwent posterior cranial vault distraction as a primary first-stage operation. Clinical outcomes and complications were collated., Results: A total of 41 patients (25 females, 16 males; mean age 11 months) with syndromic craniosynostosis (n = 32) and nonsyndromic craniosynostosis (n = 9) were included. The main indication for posterior cranial vault distraction in this cohort was turricephaly (63%). The mean distraction distance was 25.9 mm and the mean decrease in TI was 18%. The estimated increase in PCV from distraction in this cohort was 19.7%. In this cohort of patients, 13 patients (32%) experienced complications but there were no mortalities., Conclusion: Posterior cranial vault distraction osteogenesis is an effective surgical procedure to increase intracranial volume and correct turricephaly in children with syndromic and nonsyndromic craniosynostosis. This procedure is considered a safe and effective first-stage cranial operation in children with syndromic craniosynostosis with increased intracranial pressure and or turricephaly., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2023 British Association of Plastic, Reconstructive and Aesthetic Surgeons. All rights reserved.)

Background: Spring-assisted surgery (SAS) and cranial vault remodeling (CVR) are widely used surgical techniques to correct sagittal craniosynostosis (SC). The authors evaluated changes in regional morphology of patients with SC who had undergone SAS or CVR, using the frontal bossing index (FBI), occipital bulleting index, vertex narrowing index (VNI), and scaphocephalic severity index (SCI) to capture differences in anterior protrusion, posterior protrusion, width restriction, and global dysmorphology, respectively., Methods: Indices were measured on computed tomography and 3-dimensional photographs (n = 788) of 257 patients with SC from 2001 through 2022 who underwent SAS (n = 177) or CVR (n = 80). Short-term and long-term outcomes were evaluated., Results: Mean age at time of surgery was older in the CVR cohort (CVR, 22.55 ± 16.00 years; SAS, 4.56 ± 2.24 years) (P < 0.05). Before surgery, the SAS cohort had more severe regional dysmorphology in FBI, VNI, and SCI (P < 0.05) relative to the CVR cohort. Frontal bossing consistently improved over time in both cohorts. Head width improved as soon as 0 to 6 months postoperatively (P < 0.05), but it consistently regressed after the initial improvement in both cohorts. Global head shape initially improved in the CVR cohort but regressed over time. In contrast, patients in the SAS group maintained improvements in SCI over time, with significantly better percentage change in every age interval (P < 0.05)., Conclusions: The FBI, occipital bulleting index, VNI, and SCI are useful tools for monitoring head shape and growth. SAS and CVR achieve similar morphologic outcomes, despite more severe preoperative morphology in the SAS group. Trending index changes with growth between the 2 cohorts suggest improved longevity of correction in the SAS group., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2024 by the American Society of Plastic Surgeons.)

Objective: Craniosynostosis leads to craniofacial deformity and may result in raised intracranial pressure, neurocognitive deficits, and psychosocial issues if left untreated. The global epidemiology of craniosynostosis is unknown. We conducted a meta-analysis to estimate global birth prevalence., Methods: PubMed, Embase, and Scopus were searched. Articles were screened by title and abstract and then full text. Meta-analysis of birth prevalence was conducted. Birth prevalence figures were combined with metrics detailing the number of births in 2019 to estimate the number of children worldwide born with craniosynostosis annually., Results: Of 1378 resultant articles, 24 studies were included, including 20 providing data for craniosynostosis overall and 9 for nonsyndromic craniosynostosis. World Health Organization regions of included studies were 9 (37.5%) European Region, 8 (33.3%) Region of the Americas, 4 (16.7%) Western Pacific region, 2 (8.3%) African Region, and 1 (4.2%) Eastern Mediterranean Region. Lower middle-income countries represented only 4% of study manuscripts. The overall birth prevalence of craniosynostosis was 5.9 per 10,000 live births (20 studies; 95% confidence interval [CI]: 3.9, 8.4; I 2  = 100%). The birth prevalence of nonsyndromic craniosynostosis was 5.2 per 10,000 live births (9 studies; 95% CI: 3.4, 7.3; I 2  = 98%). The number of children born globally with craniosynostosis in 2019 was estimated to be 84,665 (95% CI: 55,965, 120,540), including 72,857 (95% CI: 47,637, 120,280) with nonsyndromic craniosynostosis., Conclusions: Craniosynostosis is a common condition that affects the neurocognitive and craniofacial skeletal development of children worldwide. Initiatives to scale up capacity for craniosynostosis epidemiologic research and clinical care are warranted, particularly in low- and middle-income countries., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Open middle and posterior cranial vault expansion (OPVE) or endoscopic (ES) strip craniectomy are two surgical techniques for normalization of head shape in isolated sagittal synostosis. This study aims to compare 2-year cranial morphometrics after these two approaches., Methods: The authors performed morphometric analysis on preoperative [time (T) 0], immediately postoperative (T1), and 2-year (T2) postoperative computed tomographic scans of patients who underwent OPVE or ES before 4 months of age. Perioperative data and morphometrics were compared between the two groups and age-matched controls., Results: Nineteen patients were included in the ES cohort, 19 age-matched patients were included in the OPVE cohort, and 57 were included as controls. Median surgery time and blood transfusion volume were less for the ES approach (118 minutes and 0 cc, respectively) compared with OPVE (204 minutes and 250 cc, respectively). Anthropometric measurements after OPVE were closer in normal controls at T1 compared with ES, but the skull shapes were comparable at T2. In the midsagittal plane, anterior vault was higher after OPVE at T2 compared with both ES and controls, but the posterior length was shorter and closer to controls than in the ES cohort. Cranial volumes were like controls for both cohorts at T2. There was no difference in complication rate., Conclusions: Both OPVE and ES techniques result in normalization of cranial shape in patients with isolated sagittal synostosis after 2 years with minimal morphometric differences. Family decision-making between the two approaches should be based on age at presentation, avoidance of blood transfusion, scar pattern, and availability of helmet molding and not on expected outcome., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Sagittal craniosynostosis is the most common form of congenital cranial deformity. Surgical interventions are performed either open or endoscopic. Advancements in minimally invasive surgery have enabled the development of the endoscopic suturectomy technique. This is contrasted to the traditional open cranial vault reconstruction. There is a paucity of data comparing the head shape changes from both techniques. This study aims to compare the morphological outcome of endoscopic suturectomy versus total cranial vault reconstruction., Methods: This is a retrospective comparative study involving 55 cases of sagittal craniosynostosis, 37 of which has open total cranial vault reconstruction and 18 had endoscopic suturectomy procedure. Preoperative and postoperative 3D photographs of both groups were analyzed and compared. The change in correction between preoperative and postoperative state was measured against a crowd-driven standard for acceptable head shape., Results: Total cranial vault had higher percentage change between pre and postoperative cranial index than endoscopic suturectomy (14.7% versus 7.7%, P = 0.003). However, both techniques were able to achieve the minimum standard of 70% correction (TCV 107.5%, ES 100.4%, P = 0.02)., Conclusion: Total cranial vault and endoscopic suturectomy are effective in correcting scaphocephaly among children with sagittal craniosynostosis. Additionally, both techniques are able to achieve a percentage correction that exceeds the 70% benchmark established by the lay public., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Background: Sagittal craniosynostosis results in varying degrees of frontal bossing and bilateral temporal pinching. This study assessed the three-dimensional changes in these regions using curvature analysis and volumetric analysis before and 1 year after extended sagittal strip craniectomy (ESC) with postoperative helmet therapy., Methods: A retrospective review of three-dimensional photographs of 50 subjects treated with ESC with postoperative helmet therapy and 50 age-matched controls was performed. Images were collected preoperatively and 1 year postoperatively. Forehead convexity and temple concavity were quantified. Computed tomographic scans of subjects with and without sagittal synostosis were analyzed to assess the percentage of total intracranial volume (ICV) in the anterior cranial fossa before and after ESC with postoperative helmet therapy., Results: Forehead convexity in the ESC with postoperative helmet therapy group preoperatively (24.49 ± 3.16 m -1 ) was significantly greater than controls (22.48 ± 3.84 m -1 ; P = 0.005). Forehead convexity significantly decreased after ESC with postoperative helmet therapy (18.79 ± 2.43 m -1 ; P < 0.001) and did not differ from controls (19.67 ± 3.08 m -1 ; P = 0.115). The ESC group had more concave temples preoperatively (-10.27 ± 4.37 m -1 ) as compared with controls (-6.99 ± 3.55 m -1 ; P < 0.001). Temple concavity significantly decreased after ESC (-4.82 ± 3.17 m -1 ; P < 0.001) and did not differ from controls (-5.64 ± 3.27 m -1 ; P = 0.075). In the ESC group, the percentage ICV in the anterior cranial fossa decreased from 22.03% to 18.99% after surgery, whereas the anterior volume in controls was stable (17.74% to 16.81%)., Conclusions: The ESC group had significantly greater forehead convexity, temple concavity and anterior cranial fossa volume compared with controls. One year after ESC with postoperative helmet therapy, forehead convexity, temple concavity, and percentage ICV in the anterior fossa were comparable to controls., Clinical Question/level of Evidence: Therapeutic, IV., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Background: Minor cranial sutural synostosis is currently regarded as a rare diagnosis. As clinical awareness grows, a greater number of cases are being documented. This study aims to describe the variants of unicoronal synostosis with regard to major and minor sutural involvement and secondary effects on cranial and orbital morphology. The information is aimed to improve clinical diagnosis and management., Methods: A retrospective study was conducted collecting preoperative computed tomographic scans of patients diagnosed with unicoronal synostosis and listed for surgical interventions, identified from a craniofacial database. Within these patients, different synostotic variants were identified based on which suture was affected. Scans of normal pediatric skulls (trauma) were used for a control group. Computed tomographic scans were analyzed for sutural involvement, cranial base deflection, and ipsilateral and contralateral orbital height and width. One-way analysis of variance was used to detect differences between synostotic variants and controls., Results: A total of 57 preoperative computed tomographic scans of patients with unicoronal synostosis were reviewed, in addition to 18 computed tomographic scans of normal skulls (control group). Four variants of unicoronal synostosis were identified: frontoparietal, frontosphenoidal, frontoparietal and frontosphenoidal, and frontosphenoidal and frontoparietal. The last two variants differ in their temporal involvement in the direction of sutural synostosis and ultimately cranial and orbital morphology. Three variants have been previously identified, but the fourth is presented for the first time., Conclusions: An understanding of the variants of unicoronal synostosis and their temporal relationships is integral for accurate clinical diagnosis and surgical correction. Recommendations for treatment are based on discrete changes in orbital morphology.

Introduction: Assessment of cranial dysmorphism in sagittal synostosis is often subjective but objective measures can be applied. These include cephalic index (CI) and midsagittal vector analysis (MSVA)., Objective: To assess discriminant validity, construct validity, and responsiveness of CI and MSVA measured from computed tomography (CT) in patients with sagittal synostosis., Methods: Patients with nonsyndromic isolated sagittal synostosis with complete preoperative (n = 30) and postoperative (n = 13) CT data were included. Age-matched control group (n = 24) comprised of normocephalic patients who underwent CT for reasons related to trauma., Outcome Measures: Retrospective CT evaluation of CI and MSVA was conducted and correlated with a dysmorphism numeric rating scale (D-NRS) that measured surgeon-rated severity of sagittal synostosis. Responsiveness of CI and MSVA was evaluated using dysmorphism global rating of change (D-GRC)., Results: Thirty patients with sagittal synostosis were demographically similar to 24 normocephalic patients. The difference in CI and MSVA was statistically significant between normocephalic and scaphocephalic patients. Cephalic index had a good correlation with D-NRS ( r = -0.665, ρ = -0.667), but not with MSVA ( r = 0.250, ρ = 0.203). Change in CI ( r = 0.738, ρ = 0.657) was well correlated with D-GRC, but not with MSVA ( r = -0.409, ρ = -0.301)., Conclusion: Cephalic index appears to quantify the severity of sagittal synostosis better than MSVA. Cephalic index also has better responsiveness than MSVA to measure a reduction in severity of disease; however, MSVA is a better descriptive craniometric measurement. Midsagittal vector analysis was able to quantify the shift in morphology in sagittal synostosis following surgical treatment.

Background: Modifications of the pi craniectomy technique are meant to address the occipital bullet deformity of sagittal synostosis, but it is not clear whether they result in persistent improvement. The authors' purpose was to use morphometric analysis to determine whether a low occipital osteotomy with verticalization results in improved occipital shape after a modified pi procedure 2 years after surgery., Method: The authors performed a retrospective cohort study comparing modified pi technique with and without a low occipital osteotomy with verticalization immediately and 2 years after surgery relative to age-matched normal controls. The authors used anthropometric measures and population-level anatomical templates using the multivariate template construction script from advanced normalization tools for comparison between groups. A subgroup analysis was performed for severe occipital bullet deformity at presentation., Results: The authors observed stable improvement in the angle of the inferior occiput with the occipital remodeling modification that persisted 2 years after surgery. This improvement was seen in the entire cohort and was greater in the severe subgroup analysis. Complications and blood transfusion volumes were not different between the 2 techniques. The low occipital osteotomy group demonstrated improved posterior vertical height and cephalic index immediately after surgery, but these did not persist 2 years later., Conclusions: Occipital remodeling improves the bullet deformity but does not affect posterior vertical height 2 years after surgery. The authors recommend direct inferior occipital remodeling when using the pi technique for young patients with acute occipital incline angles and occipital constriction., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Background: This retrospective cohort study evaluated the longitudinal three-dimensional cranial shape developments and the secondary treatment aspects after endoscopically assisted craniosynostosis surgery (EACS) with helmet therapy and open cranial vault reconstruction (OCVR) for scaphocephaly., Methods: Longitudinally collected three-dimensional photographs from scaphocephaly patients and healthy infants were evaluated. Three-dimensional cranial shape measurements and growth maps were compared between the groups over time. Secondary treatment aspects were compared for the treatment groups., Results: Both surgical techniques showed their strongest changes directly after surgery, with mean parietal three-dimensional growths up to 10 mm. At age 24 months, comparison of head shapes showed mean three-dimensional differences less than ±2 mm, with OCVR resulting in a lower vertex and longer cranial length when compared with EACS. At 48 months of age, no measurements were significantly different between treatment groups. Only the total head volume was somewhat larger in the male EACS group at age 48 months ( P = 0.046). Blood loss in EACS (mean, 18 mL; range, 0 to 160 mL) was lower than in OCVR (mean, 100 mL; range, 15 to 300 mL; P < 0.001). Median length of stay after surgery was shorter for EACS (mean, 2 days; range, 1 to 5 days) compared with OCVR (mean, 5 days; range, 3 to 8 days; P < 0.001)., Conclusions: The authors conclude that EACS for scaphocephaly shows equal craniometric results at age 48 months and has a better surgery profile compared with OCVR. Early diagnostics and referral for suspected scaphocephaly to allow EACS is therefore recommended., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2023 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of the American Society of Plastic Surgeons.)

Objective: This study assesses the level of child-patient satisfaction with the surgical result after scaphocephaly correction and the difference between child-patient and parents' perspectives., Methods: A questionnaire was sent out to children between 6 and 18 years old with isolated sagittal synostosis, who had undergone either a frontobiparietal remodeling or extended strip craniotomy, and separately to their parents., Results: The questionnaire was completed by 96 patients, 81.2% of the patients considered their head to be similar or slightly different from others. Despite the majority being satisfied with the outcome, 33% would change the shape of their head if they could. Patients who underwent extended strip craniotomy wanted to change the back of their head more often ( P = 0.002), whereas patients who underwent frontobiparietal remodeling wanted to change their forehead ( P = 0.005). The patients' own perspective on head shape was independent of the cephalic index (CI). However, patients with a relatively narrow CI received more remarks from others about their heads ( P = 0.038). Parent and child agreement was 49.7% on average. Differences between child-patients and parents were found in reporting adaptive behavior., Conclusion: The majority of patients were satisfied with the outcome of their intervention. The child's perspective seems to be a valuable addition to evaluate sagittal synostosis surgery as it is independent of the CI and differentiates between different surgical techniques. In addition, the patient's perspective is comparable to the parent's perspective, but gives more details on adaptive behavior., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Mutaz B. Habal, MD.)

Background: This study aimed to quantify the change in three-dimensional skull morphometrics for patients with sagittal synostosis at presentation, after surgery, and at 2-year follow-up., Methods: Computed tomography scans from 91 patients with isolated SS were age-, sex-, and race-matched with those from 273 controls. The authors performed vector analysis with linear regressions to model the effect of open middle and posterior cranial vault remodeling on cranial shape and growth., Results: Anterior cranial volume, bossing angle, and frontal shape were not changed by surgery but normalized without surgical intervention by 2 years. Biparietal narrowing and middle cranial volume were corrected after surgery and maintained at 2 years. Occipital protuberance was improved after surgery and normalized at 2 years. Posterior cranial volume was decreased by occipital remodeling and remained slightly lower than control volumes at 2 years, whereas middle vault volume was larger than in controls. Residual deformities that persisted at 2 years were decreased superolateral width at the level of opisthion and increased anterosuperior height (vertex bulge). Linear models suggested older age at surgery resulted in more scaphocephaly and enlarged posterior cranial vault volumes at 2 years but did not affect other volume outcomes. Preoperative severity was the variable most predictive of 2-year morphometrics., Conclusions: Initial severity of sagittal synostosis deformity was the best predictor of 2-year morphometric outcomes. Upper posterior cranial width decreases with time after surgery and an anterior vertex bulge can persist after open surgery, but frontal dysmorphology self-corrects without surgical intervention., Clinical Question/level of Evidence: Therapeutic, IV., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Objective: In this systematic review and meta-analysis the authors aimed to directly compare open surgical and endoscope-assisted techniques for the treatment of sagittal craniosynostosis, focusing on the outcomes of blood loss, transfusion rate, length of stay, operating time, complication rate, cost, and cosmetic outcome., Methods: A literature search was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant articles were identified from 3 electronic databases (MEDLINE, EMBASE, and CENTRAL [Cochrane Central Register of Controlled Trials]) from their inception to August 2017. The quality of methodology and bias risk were assessed using the Effective Public Health Practice Project Quality Assessment Tool for Quantitative Studies. Effect estimates between groups were calculated as standardized mean differences with 95% CIs. Random and fixed effects models were used to estimate the overall effect., Results: Of 316 screened records, 10 met the inclusion criteria, of which 3 were included in the meta-analysis. These studies reported on 303 patients treated endoscopically and 385 patients treated with open surgery. Endoscopic surgery was associated with lower estimated blood loss (p < 0.001), shorter length of stay (p < 0.001), and shorter operating time (p < 0.001). From the literature review of the 10 studies, transfusion rates for endoscopic procedures were consistently lower, with significant differences in 4 of 6 studies; the cost was lower, with differences ranging from $11,603 to $31,744 in 3 of 3 studies; and the cosmetic outcomes were equivocal (p > 0.05) in 3 of 3 studies. Finally, endoscopic techniques demonstrated complication rates similar to or lower than those of open surgery in 8 of 8 studies., Conclusions: Endoscopic procedures are associated with lower estimated blood loss, operating time, and days in hospital. Future long-term prospective registries may establish advantages with respect to complications and cost, with equivalent cosmetic outcomes. Larger studies evaluating patient- or parent-reported satisfaction and optimal timing of intervention as well as heterogeneity in outcomes are indicated.

Objective: Despite increasing adoption of endoscopic techniques for repair of nonsagittal single-suture craniosynostosis, the efficacy and safety of the procedure relative to established open approaches are unknown. In this systematic review the authors aimed to directly compare open surgical and endoscope-assisted techniques for the treatment of metopic, unilateral coronal, and lambdoid craniosynostosis, with an emphasis on quantitative reported outcomes., Methods: A literature search was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant articles were identified from 3 electronic databases (MEDLINE, EMBASE, and CENTRAL [Cochrane Central Register of Controlled Trials]) from their inception to August 2017. The quality of methodology and bias risk were assessed using the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool for Quantitative Studies., Results: Of 316 screened records, 7 studies were included in a qualitative synthesis of the evidence, of which none were eligible for meta-analysis. These reported on 111 unique patients with metopic, 65 with unilateral coronal, and 12 with lambdoid craniosynostosis. For all suture types, 100 (53%) children underwent endoscope-assisted craniosynostosis surgery and 32 (47%) patients underwent open repair. These studies all suggest that blood loss, transfusion rate, operating time, and length of hospital stay were superior for endoscopically treated children. Although potentially comparable or better cosmetic outcomes are reported, the paucity of evidence and considerable variability in outcomes preclude meaningful conclusions., Conclusions: Limited data comparing open and endoscopic treatments for metopic, unilateral coronal, and lambdoid synostosis suggest a benefit for endoscopic techniques with respect to blood loss, transfusion, length of stay, and operating time. This report highlights shortcomings in evidence and gaps in knowledge regarding endoscopic repair of nonsagittal single-suture craniosynostosis, emphasizing the need for further matched-control studies.

Scaphocephaly is the most common type of craniosynostosis and various surgical techniques are used for treatment. Due to late postoperative changes of the head shape, long-term outcome data is important for evaluating any new surgical technique. At our institution, minimally invasive strip craniectomy without regular helmet therapy is the standard treatment in scaphocephalic patients. Between October 2021 and February 2023, we retrospectively examined the skull shape of patients who underwent minimally invasive strip craniectomy for scaphocephaly using a 3D surface scan technique. The cephalic index (CI), the need for helmet therapy and additional cosmetic outcome parameters were investigated. We included 70 patients (72.5% male). The mean follow-up time was 46 (10-125) months and the mean CI was 75.7 (66.7-85.2). In 58 patients, the final cosmetic result was rated as "excellent/good" (mean CI: 76.3; 70.4-85.0), in 11 as "intermediate" (mean CI: 73.3; 66.7-77.6), and in one case as "unsatisfactory" (CI 69.3). The presence of a suboccipital protrusion was associated with a "less than good" outcome. The CI correlated significantly with the overall outcome, the presence of frontal bossing, and the interval between scan and surgery (age at scan). Minimally invasive strip craniectomy is an elegant and safe method to correct scaphocephaly. Our data show good cosmetic results in the long term even without regular postoperative helmet therapy., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Distraction osteogenesis is utilized to increase intracranial volume in the treatment of restrictive pathologies, most commonly syndromic synostosis. Children too young for open calvarial vault expansion or other systemic or local contraindications to a direct reconstructive approach benefit greatly from distraction osteogenesis, typically addressing posterior vault expansion. Wound infection, cerebrospinal fluid (CSF) leak, device failure, need for a second surgery for removal, and cost, are issues that can limit the use of this approach. These challenges are more pronounced in low- and middle-income countries (LMICs) due to lack of access to the device, the financial burden of the need for a second surgery, and the severity of the implications of infection and CSF leak. Over the last five decades, there has been an increased acceptance of bioresorbable instrumentation in craniofacial surgery. Poly L-lactic acid, polyglycolic acid, and polydioxanone are the most commonly used polymers. New resorbable fixation tools such as ultrasound-activated pins and heat-activated pins are superior to conventional bioresorbable screws in allowing attachment to thinner bone plates. In this paper, we present a review of the literature on cranial vault distraction and the use of bioresorbable materials and propose a novel design of a fully absorbable cranial distractor system using external magnetic distraction control, eliminating the need for external activation ports and a second surgery to remove the hardware. The application of this technology in LMIC settings could advance access to care and treatment options for patients with syndromic synostosis., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

When diagnosed and treated after the first year of life, the surgical management of sagittal synostosis typically necessitates total cranial vault reconstruction. The safety of total cranial vault reshaping has improved greatly over the past 3 decades. We report on the first published case of an asymptomatic 5-year-old patient who was found to have a large left sided chronic subdural hematoma 4 months after total cranial vault reshaping requiring surgery, detected on routine imaging. We hypothesize that augmentation of venous blood egression through the emissary veins during surgery may have led to venous hypertension and subsequently the development of the chronic subdural hematoma., (© 2018 S. Karger AG, Basel.)

Background: It is important to determine whether sagittal synostosis-associated scaphocephaly is static in the presurgical period, or whether there are morphologic differences with time to include in surgical decision-making. The authors' purpose was to perform cross-sectional analysis of cranial morphology before any surgical intervention in children with sagittal synostosis younger than 9 months compared to matched controls., Methods: The authors performed morphometric analysis on computed tomographic scans from 111 untreated isolated sagittal synostosis patients younger than 9 months and 37 age-matched normal controls. The authors divided the patients into three age groups and performed statistical comparison between sagittal synostosis and controls for each group., Results: Sagittal synostosis cephalic indices were stable and lower in patients than in controls across groups. Total cranial volume was equivalent, but sagittal synostosis patients had a greater posterior volume than controls at all ages and a smaller middle fossa volume at older ages. Pterional width was greater in sagittal synostosis patients than in controls for each age group. Frontal bossing vectors were most severe in the youngest age groups and least in the older group. Occipital protuberance was consistent across the age groups., Conclusions: Upper parietal narrowing and occipital protuberance were the consistent deformities across age groups, with the most parietal constriction seen in older patients. Frontal bossing was not consistent and was more severe in the younger patients. The authors did not detect significant pterional constriction, and the appearance of constriction is relative to adjacent morphology and not absolute., Clinical Question/level of Evidence: Risk, II., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Background: The diagnosis and management of metopic craniosynostosis involve subjective decision-making at the point of care. The purpose of this work was to describe a quantitative severity metric and point-of-care user interface to aid clinicians in the management of metopic craniosynostosis and to provide a platform for future research through deep phenotyping., Methods: Two machine-learning algorithms were developed that quantify the severity of craniosynostosis-a supervised model specific to metopic craniosynostosis [Metopic Severity Score (MSS)] and an unsupervised model used for cranial morphology in general [Cranial Morphology Deviation (CMD)]. Computed tomographic (CT) images from multiple institutions were compiled to establish the spectrum of severity, and a point-of-care tool was developed and validated., Results: Over the study period (2019 to 2021), 254 patients with metopic craniosynostosis and 92 control patients who underwent CT scanning between the ages of 6 and 18 months were included. CT scans were processed using an unsupervised machine-learning based dysmorphology quantification tool, CranioRate. The average MSS was 0.0 ± 1.0 for normal controls and 4.9 ± 2.3 ( P < 0.001) for those with metopic synostosis. The average CMD was 85.2 ± 19.2 for normal controls and 189.9 ± 43.4 ( P < 0.001) for those with metopic synostosis. A point-of-care user interface (craniorate.org) has processed 46 CT images from 10 institutions., Conclusions: The resulting quantification of severity using MSS and CMD has shown an improved capacity, relative to conventional measures, to automatically classify normal controls versus patients with metopic synostosis. The authors have mathematically described, in an objective and quantifiable manner, the distribution of phenotypes in metopic craniosynostosis., (Copyright © 2023 by the American Society of Plastic Surgeons.)

The purpose of this study is to describe the treatment strategies and outcomes of nonsyndromic single-suture sagittal craniosynostosis based on the patient's age at intervention. Studies from MEDLINE, Scopus, and Cochrane Central Register of Controlled Trials were systematically searched for patients with nonsyndromic single-suture sagittal craniosynostosis. Inclusion criteria encompassed studies with follow-up of at least 12 months, minimum of 25 patients per cohort, and first-time surgical intervention. The risk of bias in nonrandomized studies of intervention tool [Risk Of Bias In Non-randomized Studies-of Interventions (ROBINS-I)] was applied. A total of 49 manuscripts with 3316 patients met criteria. Articles were categorized based on age at intervention; 0 to 6, older than 6 to 12, and older than 12 months. Fifteen of the manuscripts described interventions in more than 1 age group. From the 49 articles, 39 (n=2141) included patients 0 to 6 months old, 15 (n=669) discussed patients older than 6 to 12 months old, and 9 (n=506) evaluated patients older than 12 months old. Follow-up ranged from 12 to 144 months. Over 8 types of open surgical techniques were identified and 5 different minimally invasive procedures were described. Minimally invasive procedures were exclusively seen in the youngest patient cohort, while open cranial vault reconstructions were often seen in the 2 older cohorts. Endoscopic surgery and open conservative procedures are indicated for younger patients, while complex open cranial vault reconstructions are common in older patients. However, there is no consensus on one approach over the other. Even with the analysis of this review, we cannot factor a strong conclusion on a specific technique., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Craniosynostosis is a relatively common birth defect characterized by the premature fusion of one or more cranial sutures. Examples of craniosynostosis syndromes include Crouzon (CS), Pfeiffer (PS), and Apert (AS) syndrome, with clinical characteristics such as midface hypoplasia, hypertelorism, and in some cases, limb defects. Mutations in Fibroblast Growth Factor Receptor-2 comprise the majority of known mutations in syndromic forms of craniosynostosis. A number of clinical reports of FGFR-associated craniosynostosis patients and mouse mutants have been linked to gastrointestinal tract (GIT) disorders, leading to the hypothesis of a direct link between FGFR-associated craniosynostosis syndromes and GIT malformations. We conducted an investigation to determine GIT symptoms in a sample of FGFR-associated craniosynostosis syndrome patients and a mouse model of CS containing a mutation (W290R) in Fgfr2. We found that, compared to the general population, the incidence of intestinal/bowel malrotation (IM) was present at a higher level in our sample population of patients with FGFR-associated craniosynostosis syndromes. We also showed that the mouse model of CS had an increased incidence of cecal displacement, suggestive of IM. These findings suggest a direct relationship between FGFR-related craniosynostosis syndromes and GIT malformations. Our study may shed further light on the potential widespread impact FGFR mutations on different developmental systems. Based on reports of GIT malformations in children with craniosynostosis syndromes and substantiation with our animal model, GIT malformations should be considered in any child with an FGFR2-associated craniosynostosis syndrome. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Cranio-orbital remodeling aims to correct the dysmorphic skull associated with craniosynostosis. Traditionally, the skull is reconstructed into a shape that is subjectively normal according to the surgeon's perception. We present a novel technique using a mathematical algorithm to define the optimal location for bony osteotomies and to objectively reshape the fronto-orbital bar into an ideal normal skull contour. Using pre-operative computed tomography images, the abnormal skull contour at the frontal-orbital region was obtained for infants planned to undergo cranio-orbital remodeling. The ideal skull shape was derived from an age- and sex-matched normative skull library. For each patient, the mathematical technique of dynamic programming (DP) was applied to compare the abnormal and ideal skull shapes. The DP algorithm identifies the optimal location of osteotomy sites and calculates the objective difference in surface area remaining between the normative and dysmorphic skull shape for each solution applied. By selecting the optimal solution with minimal objective difference, the surgeon is guided to reproducibly recreate the normal skull contour with defined osteotomies. The DP algorithm was applied in 13 cases of cranio-orbital remodeling. Five female and 8 male infants with a mean age of 11 months were treated for craniosynostosis classified as metopic (n = 7), unicoronal (n = 4), or bicoronal (n = 2). The mean OR time was 190.2  min (SD 33.6), mean estimated blood loss 244  cc (SD 147.6), and 10 infants required blood transfusions. Compared with a historical crania-orbital remodeling group treated without application of the algorithm, there was no significant difference in OR time, estimated blood loss, or transfusion rate. This novel technique enables the craniofacial surgeon to objectively reshape the fronto-orbital bar and reproducibly reconstruct a skull shape resembling that of normal infants.

Background and Objectives: Premature fusion of the sagittal suture is the most common form of craniosynostosis and can be treated using a variety of open or endoscopic approaches. Existing approaches have varying degrees of effectiveness. Open approaches, whether performed early or late, can be associated with significant blood loss and the need for transfusion. Endoscope-assisted approaches are minimally invasive but require months of postoperative helmet therapy to help remodel the skull. Implantation of springs or distractors requires a second operation for removal of the devices. Here, we present an alternative technique for early correction of sagittal craniosynostosis combining sagittal synostectomy with tension band sutures to remodel the skull without need for transfusion or helmet therapy., Methods: We retrospectively reviewed the medical records of all patients treated for sagittal craniosynostosis using a synostectomy with tension band sutures at a single tertiary care institution. Data on patient demographics, operative factors, and postoperative course were collected., Results: Thirty-four patients underwent the novel procedure. The median preoperative cephalic index was 68 and improved to 76 immediately postoperatively. The median blood loss was 10 mL while the operative duration was 112 minutes. No blood transfusions were needed. One small dural laceration was encountered that was promptly repaired. There were no postoperative complications. Patients presenting for follow-up visits showed continued improvement in head shape and cephalic index., Conclusion: A modified sagittal craniectomy with tension band sutures to remodel the skull is effective in achieving immediate correction of sagittal craniosynostosis. The correction remains durable over long-term follow-up. Importantly, the technique can be performed with minimal blood loss and reduces transfusion risk, operative time, and overall morbidity compared with traditional open approaches while avoiding the need for helmet therapy necessitated by endoscopic approaches., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: The purpose of this study is 2-fold: (1) to identify computed tomography (CT)-based morphometric parameters that differentiate the normal skull from one with sagittal synostosis and (2) to evaluate correction of sagittal synostosis with extended strip craniectomy and postoperative helmeting based on morphometric parameters., Methods: An institutional review board-approved, retrospective review was carried out at the Hospital for Sick Children for all patients who underwent an extended strip craniectomy and postoperative helmeting for sagittal synostosis from 1999 to 2005. Inclusion criteria consisted of patients who underwent a routine craniofacial CT preoperatively and 12 months postoperatively. Craniofacial CT scans of age-matched control subjects were used for preoperative and postoperative comparison., Results: Thirty-nine patients with sagittal synostosis met inclusion criteria. Median age at preoperative CT was 3.0 months. Nine control subjects were identified, with a median age at CT scan of 5.0 months. Patients with sagittal synostosis preoperatively had a significantly longer maximum cranial length, smaller maximum cranial breadth, more acute frontal takeoff and occipital incline angles, lower cephalic index, and an anteriorly positioned vertex. Postoperative CT scans (median, 17.0 months) were compared with 10 control subjects (median, 19.0 months). Patients with sagittal synostosis postoperatively had equivalent maximum cranial breadth, frontal takeoff, and occipital incline angles as compared with controls. Sagittal synostosis patients remained with a significantly longer maximum cranial length, lower cephalic index, and anteriorly positioned vertex., Conclusions: Twelve months following extended strip craniectomy and helmeting for sagittal synostosis, CT-based morphometric analysis demonstrated correction of cranial breadth, frontal bossing, and occipital bulleting. Skull length and vertex position did not fully correct.

Cranio-orbital reshaping for anterior cranial-vault deformities associated with craniosynostosis traditionally relies on the surgeon's subjective estimate of the shape and appearance of a normal forehead. Computer-aided design/computer-aided manufacture (CAD/CAM) bandeau templates to guide reconstruction were introduced in our centre to eliminate this subjectivity and to effect more reproducible surgical results. The aim of this study was to compare two groups of patients (template, n = 14 vs. no template, n = 23) to measure surgical outcomes. The virtual, computational version of the template was used as an outcome assessment tool. It was used to calculate an intervening area under the curve (AUC) between the normative template and the patient's reconstructed supra-orbital bar on a representative computed tomography (CT) axial section. A comprehensive chart review was conducted of patients in both groups to examine the preoperative and postoperative variables. Based on the analysis performed on the immediate postoperative CT scans, in the template group - as compared to the control, no-template group - the use of the bandeau template led to a greater reduction in AUC (74% vs. 56%, p = 0.016), indicating a better conformity between the reconstructed supra-orbital bar and the ideal, normal bandeau shape. The duration of operation was significantly reduced with the use of the template (212 vs. 258 min, p < 0.001). The application of prefabricated templates in cranio-orbital reshaping is highly useful for accurate preoperative planning; reproducible and efficient intra-operative correction of dysmorphology; and objective surgical outcomes assessment., (Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Learning Objectives: After studying this article, the participant should be able to: (1) Understand craniofacial dysmorphology and identify basic pediatric craniofacial syndromes. (2) Understand the functional concerns associated with these syndromes. (3) Achieve familiarity with the management protocols for the treatment of pediatric craniofacial syndromes., Summary: This article provides an overview of the diagnosis and management of infants and children with craniofacial syndromes. Treatment protocols from The Hospital for Sick Children, Toronto, Ontario, Canada, and Seattle Children's Hospital, Seattle, Washington, are highlighted.

Background: Syndromic craniosynostosis patients are at risk for sleep-related disordered breathing (SRDB) but the role of polysomnography (PSG) in assessing these patients has not been fully explored. Our aim was to evaluate the prevalence or severity of SRDB in children with syndromic craniosynostosis or the impact of treatments on their SRDB., Methods: We conducted a retrospective review of all patients with syndromic craniosynostosis referred between 1996 or 2008 for an initial PSG to rule out SRDB. For those with SRDB, we reviewed the interventions post PSG., Results: 35 patients (18 females) were included. Specific diagnoses were Crouzon's (n=18), Apert's (n=14), Pfeiffer (n=2) or Saethre-Chotzen (n=1) syndromes. Their mean age was 4.5 years or their mean body mass index (BMI) was 16.9 kg/m(2). Of these patients, 26/35 (74%) had evidence of SRDB. The median obstructive apnoea index was 6.6/h (range 0.5-36.4/h) or median central apnoea index was 1.0/h (range 0.0-66.4/h). A total of 16 children had interventions to treat SRDB, of which 14/16 had a follow up PSG or only 10/14 (x%) had a significant improvement of their SRDB., Conclusion: This review confirms a high prevalence SRDB in this referred population. Despite various interventions, complete resolution of SRDB could not be achieved., (Copyright © 2010 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Level of Evidence: III., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Background: Endoscopic craniosynostosis repair has emerged as an effective alternative to open repair, but data are limited on treatment of the 15% to 24% of patients with syndromic diagnoses. In this study, the authors examine postoperative outcomes after endoscopic repair in syndromic craniosynostosis., Methods: Retrospective review was performed of all consecutive patients undergoing endoscopic repair and all syndromic patients undergoing open repair from 2006 to 2021. Demographics, complications, and reoperations were compared between groups. Patient-reported measures of stigma and cognitive function were recorded at age 5 years and older., Results: A total of 335 patients underwent endoscopic repair, of which 38 (11%) had syndromic craniosynostosis. Syndromic craniosynostosis was associated with bicoronal involvement ( P < 0.001) and female sex ( P = 0.003). Secondary procedures were significantly more common in the syndromic group (24% versus 2.4%; P < 0.001), as were transfusions (18% versus 6.4%; P = 0.018). Secondary procedures were performed at a mean 2.8 years of age (range, 10 months to 8 years), and most commonly consisted of fronto-orbital advancement (seven in the syndromic group, and three in the nonsyndromic group). The degree of patient-reported stigma was higher in patients with syndromes ( P = 0.002), but cognitive function did not differ significantly ( P = 0.065). The incidence of reoperations after open repair was 13%, but baseline differences precluded direct comparison with the endoscopic group., Conclusions: Minimally invasive approaches in early infancy can alleviate the need for additional cranial procedures in the growing child. Syndromic craniosynostoses are a complex and heterogeneous group, and in more severe cases, endoscopic repair can be considered an adjunct technique to reduce the number of major craniofacial procedures., Clinical Question/level of Evidence: Risk, II., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Background: Variables interacting to predict outcomes following spring-mediated cranioplasty (SMC) for non-syndromic craniosynostosis, including spring parameters and calvarial thickness, are poorly understood. This study assessed interactions between spring parameters and calvarial thickness to predict changes in cephalic index (CI) following SMC., Methods: Patients undergoing SMC for non-syndromic sagittal craniosynostosis at our institution between 2014 and 2021 were included. Calvarial thickness was determined from patient preoperative CTs using Materalise Mimics at 27 points in relation to the sagittal suture. Linear mixed effects models were used to determine interactions between anterior, middle, and posterior calvarial thickness with spring force and length., Results: Sixty-nine patients undergoing surgery at mean age 3.7 months were included in this study. Stronger posterior spring force interacted with thinner posterior calvarial thickness to predict greater changes in CI at 3 months postoperatively (p = 0.022). When evaluating spring force and calvarial thickness set distances from the sagittal suture, stronger posterior spring force interacted with thinner posterior calvarial thickness 5 mm (p = 0.043) and 10 mm (p = 0.036) from the sagittal suture to predict changes in CI. Interactions between spring parameters and calvarial thickness in the anterior and middle positions did not significantly predict changes in CI., Conclusions: Stronger posterior spring force interacted with thinner posterior calvaria to predict greater changes in CI 3 months following SMC for non-syndromic sagittal craniosynostosis. These results suggest dynamic interactions between several variables may impact outcomes following SMC., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: The timing of posterior cranial expansion for the management of intracranial pressure can be "staged" by age and dysmorphology or "expectant" by pressure monitoring. The authors report shared outcome measures from one center performing posterior vault remodeling (PCVR) or distraction (PVDO) following a staged approach and another performing spring-assisted expansion (SAPVE) following an expectant protocol., Methods: Apert or Crouzon syndrome patients who underwent posterior expansion younger than 2 years were included. Perioperative outcomes and subsequent cranial operations were recorded up to last follow-up and intracranial volume changes measured and adjusted using growth curves., Results: Thirty-eight patients were included. Following the expectant protocol, Apert patients underwent SAPVE at a younger age (8 months) than Crouzon patients (16 months). The initial surgery time was shorter but total operative time, including device removal, was longer for PVDO (3 hours 52 minutes) and SAPVE (4 hours 34 minutes) than for PCVR (3 hours 24 minutes). Growth-adjusted volume increase was significant and comparable. Fourteen percent of PCVR, 33% of PVDO, and 11% of SAPVE cases had complications, but without long-term deficits. Following the staged approach, 5% underwent only PVDO, 85% had a staged posterior followed by anterior surgery, and 10% required a third expansion. Following the expectant approach, 42% of patients had only posterior expansion at last follow-up, 32% had a secondary cranial surgery, and 26% had a third cranial expansion., Conclusion: Two approaches involving posterior vault expansion in young syndromic patients using three techniques resulted in comparable early volume expansion and complication profiles., Clinical Question/level of Evidence: Therapeutic, III., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Consensus remains lacking regarding the optimal surgical treatment modality for sagittal synostosis. There is, however, wide agreement that objective analytical methods are required to demonstrate the characteristic morphology of the condition and to substantiate the benefits of specified surgical techniques. Simple calculated anthropomorphic indices, such as the cranial index, are commonly used but fail to provide satisfactory representation of morphology, which is far more complex than can be represented by its simple length-width ratio. Techniques to provide more comprehensive, yet practical, assessment of morphology are needed for analytic purposes. Herein, we introduce vector analysis as an objective, computed tomography (CT)-based morphometric technique for assessment of cranial morphology; this work represents the first application of the technique mid-sagittal vector analysis (MSVA). MSVA is a single plane application that was devised to address dysmorphology in sagittal synostosis. It was our hypothesis that MSVA would quantitatively and qualitatively depict preoperative morphology and postoperative correction in specific regions. Sixteen patients undergoing cranial reshaping surgery for sagittal synostosis were included in the study. All patients underwent routine preoperative and 1 year postoperative CT scans, from which the MSVA was derived. MSVA is a radial vector analysis in which distances to the cranial surface are measured from a single reference point origin in the sagittal plane. Preoperative morphology, characterized by respective vectors, was analyzed in three regions: the frontal, vertex, and occipital regions. Comparison with postoperative paired data was conducted for each patient. The analysis of postoperative change demonstrated (1) decrease in prominence in the frontal and occipital regions, (2) increase in height and forward translation of the vertex, and (3) ability to distinguish and qualify frontal versus occipital bossing and correction thereof. We conclude that the longitudinal differences associated with scaphocephaly are well characterized and differentiated by MSVA. Quantitative and qualitative assessment identifies three relevant regions affected by the condition and its treatment: the frontal, vertex, and occipital regions. The transverse dimension is not addressed in this single plane analysis; a more comprehensive application will require additional planes of analysis and the development of a normative database.

Quantitative changes of the 5 paired (right and left) projective linear measurements of the craniofacial complex were analyzed by anthropometric methods in 26 patients (9 males and 17 females) with right coronal synostosis, 16 in infancy (0-5 and 6-12 months) and 10 in early childhood (1-10 years). Measurements were taken from 3 regions: the supraorbital rim length of the head (t-g), the length (en-ex) and height (ps-pi) of the eye fissures in orbits, and the width (pra-pa) and length (sa-sba) of the ears. None of the patients had undergone corrective surgery.Anthropometric analysis of patients in infancy showed a large percentage of subnormal supraorbital rim lengths (t-g) on the right side of the head, with a higher frequency in females (90.9%) than in males (80.0%). Eye fissure lengths (en-ex) were subnormal more often in males than in females on both sides, while heights were generally normal bilaterally in both sexes (72.7-100% of cases). Ears of normal width (pra-pa) and length (sa-sba) were more frequent in females. In males, the frequency of subnormal ear widths and lengths surpassed those in females. The most remarkable changes between infancy and early childhood were noted in the orbital region, in which subnormal eye fissure lengths became bilaterally normal in both sexes.

Learning Objectives: After studying this article, the participant should be able to: 1. Understand the craniofacial dysmorphology of craniosynostosis, and the variation of each type. 2. Identify the functional concerns and learn the rationale behind timing of operative intervention. 3. Approach each dysmorphology critically and identify the operative intervention needed to improve form and function 4. Understand and address the specific issues related to syndromic craniosynostosis and be able to delineate management plan., Summary: Craniosynostosis is a condition in which premature fusion of one or more cranial sutures lead to abnormal head shape and growth restriction of the brain. Nonsyndromic craniosynostosis occurs in isolation, and usually involves a single suture, whereas syndromic craniosynostosis may involve multiple sutures and is associated with extracraniofacial findings. Although surgical management can be similar, the treatment plan must take into consideration issues specific to the syndromes. This article aims to provide a concise overview of the authors' current understanding regarding the presentation, treatment principle, surgical option, and debates in craniosynostosis., (Copyright © 2022 by the American Society of Plastic Surgeons.)

Background: The purpose of this manuscript is to introduce a novel endoscopic-assisted approach for creation of osteotomies and distractor hardware placement for unicoronal craniosynostosis., Methods: Only three small incisions are performed, one at the anterior fontanelle, pterional region, and upper lateral blepharoplasty location. Unicoronal strip craniectomy and osteotomies in temporal bone, nasofrontal junction, lateral orbital rim, sphenoid wing, orbital roof, and contralateral frontal bone are performed through these incisions with the assistance of a 30° 5-mm endoscope. One linear cranial distractor is inserted through the pterional incision and fixated across the unicoronal suturectomy., Results: Endoscopic-assisted fronto-orbital distraction osteogenesis has been performed on 3 patients with unicoronal craniosynostosis. Median age at surgery was 5.4 months (95%CI 5.0-6.1). Median operative duration was 98 minutes (95%CI 91-112), and estimated blood loss was 25 mL (95%CI 15-150). Median length of distraction achieved was 25.7 mm (95%CI 21.9-28.0), based on lateral skull x-rays obtained on the last day of activation. Distractors were removed 2.3 months postoperatively (95%CI 2.1-3.1), and operative duration of distractor removal was 20 minutes (95%CI 19-29). There was 1 complication, a transient cerebrospinal fluid leak thought to have been caused by a pinpoint injury to the dura at the nasofrontal junction, which was managed expectantly and resolved spontaneously by the fourth postoperative day., Conclusions: Endoscopic-assisted fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis combining the benefits of distraction osteogenesis with a minimally invasive approach. Additional experience and follow-up are needed to determine its utility, safety, and longevity., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Introduction: Hydrocephalus is variously associated to syndromic craniosynostosis (CS), while it is randomly encountered in monosutural CS. Pathogenesis is still debated and reliable criteria for the diagnosis of overt hydrocephalus are lacking. Additionally, optimal treatment is controversial since it should balance the need to relieve intracranial hypertension and the risk of recurrence favored by lowering intracranial pressure., Methods: A thorough review of the literature has been performed. Accordingly, pathogenic theories, diagnostic issues, and treatment options on hydrocephalus presenting in the context of CS are discussed., Results: The association of hydrocephalus to simple CS is considered a fortuitous event. Its treatment is usually driven by the etiology and clinical relevance of hydrocephalus, favoring treatment before surgical correction to reduce CSF-related complications. On the other side, pathogenesis of hydrocephalus in the context of syndromic CS has been mainly related to factors that are secondary to the synostostic process, such as craniocerebral disproportion and venous hypertension. Hydrocephalus complicates 12-15% of syndromic CS, though its incidence is more relevant in FGFR2-related CS and raises up to 88% in Pfeiffer syndrome. Overt hydrocephalus should be properly differentiated by non-tense ventriculomegaly that is more frequent in Apert syndrome. Since intracranial hypertension is constant in syndromic CS even in the absence of active hydrocephalus, radiological monitoring of ventricular size along with intracranial pressure monitoring is essential. Active hydrocephalus occurs more frequently in infants, though stable ventriculomegaly may evolve into overt hydrocephalus after cranial expansion. If hydrocephalus is not clinically prominent, cranial expansion should be favored as first surgical step. Although posterior cranial expansion may address posterior cranial fossa constriction and stabilize ventricular dilation, effectiveness in long-term control of hydrocephalus is not clear. ETV is an effective treatment option, though success rate is affected by the presence of brain malformations and patient age. Extrathecal CSF shunting should be used as last resource due to the increased risk of complications in this context., Conclusions: The pathogenesis of hydrocephalus complicating syndromic CS should be further investigated. Concomitantly, the definition of reliable diagnostic criteria is advocated in order to promptly and properly identify active hydrocephalus. Finally, treatment algorithm should refine the best timing and treatment options aiming to relieve intracranial hypertension on one side and reduce the risk of restenosis on the other side., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Correction (and over-correction) of asymmetries of the orbital shape and brow position in unilateral coronal craniosynostosis (UCS) is critical to successful fronto-orbital advancement. Here we quantify and three-dimensionally assess fronto-orbital irregularities in UCS patients compared to controls.Twenty-three patients with UCS evaluated at the Children's Hospital of Pittsburgh between 2006 and 2016 were age and gender-matched to controls. Computed tomography scans were reconstructed and evaluated for orbital metrics. A three-dimensional heat map of orbital regions was generated and evaluated for shape differences.Brow protrusion of the orbit ipsilateral to the synostotic suture did not differ significantly from healthy controls. Orbital height was significantly increased while orbital width was decreased on the UCS ipsilateral side compared to the contralateral side and controls. The ipsilateral cornea was overprojected relative to the brow and the infraorbital rim, but similar to controls relative to the lateral rim. The contralateral orbit had increased brow protrusion with decreased orbital height. The cornea was underprojected relative to the brow, but overprojected relative to the lateral orbital rim and similar to controls at the infraorbital rim. Three-dimensional comparison demonstrated significant overprojection of the contralateral brow, with some more mild and inconsistent underprojection of the lateral aspect of the ipsilateral brow.Key orbital and brow differences exist between the affected and unaffected sides in UCS. This study provides quantitative data that further characterize the orbital dysmorphology observed in UCS and identifies unique aspects of the diagnosis that should be taken into consideration during surgical planning., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

Background: Cranial CT is routinely taught to be the gold standard for diagnosis of craniosynostosis and used by craniofacial teams for suspected nonsyndromic single suture craniosynostosis. Given the risks associated with infant CTs, do these scans provide significantly enhanced diagnostic accuracy compared to the physical exam when performed by an experienced clinical provider?, Method: A retrospective chart review was performed for children who underwent corrective surgery for nonsyndromic, single-suture craniosynostosis over an 11 year period by a single craniofacial team. Ages at presentation and surgery, preoperative clinical diagnosis and imaging, co-existing radiographic findings, and correlation with the intraoperative diagnosis were analyzed., Results: A total of 138 patients were included in this study. The mean age was 4.2 months at initial craniofacial evaluation, and 8.0 months at time of surgery. Twenty-seven patients received imaging prior to our clinic. Of those, 21 had plain radiography and 6 had CT scans. Of the remaining 111 patients referred without imaging, craniosynostosis was clinically diagnosed in 102 (92%), whereas 9 (8%) had an unclear clinical diagnosis. Of these 9, 1 (1%) was diagnosed clinically at follow-up exam, and the remaining 8 (7%) were diagnosed using radiography (3 CT scans, 5 plain radiographs). In all patients, the preoperative diagnosis was confirmed during intraoperative assessment., Conclusions: Cranial CT was not needed by experienced craniofacial providers in 93% of nonsyndromic, single-suture craniosynostosis. Imaging obtained before craniofacial clinic referral may have been unnecessary. These findings question the classic teaching that preoperative cranial CT is the gold standard for diagnosis in infants with nonsyndromic, single-suture craniosynostosis., Competing Interests: The authors report no conflicts of interest, (Copyright © 2021 by Mutaz B. Habal, MD.)

Background: Different techniques to reshape the posterior skull vault have been developed in the last decades, all sharing the same goals of increasing the skull volume, decreasing the intracranial pressure (ICP), correcting the cranial dysmorphy, and contributing to a better growth of the skull. Though over the last years most refinements in these techniques have focused on the use of hardware as distractors or springs, the fixed posterior vault expansion remains a valuable procedure for cranial remodeling., Methods: We describe in details the technique used for fixed posterior vault expansion in children that is applied at the French Referral center for Craniosynostosis of Lyon, France., Discussion: The fixed posterior vault expansion increases the risk of perioperative complications due to the elevation of the posterior bone flap from the dura but allows an immediate decompression and correction of the shape, simplifying the postoperative course., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Abstract: Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ± 3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ± 0.90 mm with a mean consolidation period of 3.98 ± 0.72 months. Mean corrected change in intra-cranial volume was 236.30 ± 3.71 mL, at an average rate of 7.81 ± 2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ± 2.51 mm), middle cranial height (8.43 ± 4.21 mm), posterior cranial height (13.15 ± 7.45 mm), and posterior cranial fossa height (21.99 ± 8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ± 0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex., Competing Interests: The authors report no conflicts of interests., (Copyright © 2021 by Mutaz B. Habal, MD.)

Purpose: Studies have examined the impact of fusion of the sagittal suture in the skull base while others have evaluated the growth of the skull base before and after surgery. This study aims to perform the anthropometric measures of the skull base in children with scaphocephaly to evaluate the influence of surgical repair in the remodeling of the skull base and anthropometric measures., Methods: Twenty-one children with diagnosis of scaphocephaly were operated between April 2007 and October 2008, and anthropometric measures at the skull base were performed before and after a year of surgery. The measures were the cranial index (CI), distance between the crista galli and tuberculum sellar (CG-TS), distance between the crista galli and the internal auditory meatus (CG-IAM), distance between the oval foramen (OF-OF), distance between the internal auditory meatus (IAM-IAM), the angle of the skull base (Â1), and the angle between the nasion, center of sella, and basion (Â2)., Results: There was a normalization of the CI in all children, confirming an appropriate cranial remodeling. The CG-TS measure evaluated the anterior skull base, with proportional growth of 12.5%. The mediolateral growth was observed by the increase of OF-OF measures by 8.5% and IAM-IAM by 9.5%. The CG-TS measure grew by 7.2%. There was no statistical difference in the angles analyzed., Conclusion: Surgical treatment of scaphocephaly led to remodeling of the skull base, confirmed by the changes of anthropometric measures taken before and after a year of surgery.

Purpose: Unilateral coronal synostosis (UCS) results in an asymmetrical skull, including shallow and asymmetrical orbits, associated with reduced orbital volume and high prevalences of ophthalmic sequelae. Aim is to link orbital volumes in patients with UCS to severity according to UCSQ (Utrecht Cranial Shape Quantifier) and presence of ophthalmic sequelae., Methods: We included preoperative patients with UCS (≤ 18 months). Orbital volume was measured on CT scans by manual segmentation (Mimics software (Materialise, Leuven, Belgium)), and severity of UCS was determined by UCSQ. Orbital volume of affected side was compared to unaffected side using Wilcoxon signed rank test. Orbital volume ratio was calculated (affected/unaffected volume) and compared to the category of UCSQ by Kruskal-Wallis test. Opthalmic sequelae were noted., Results: We included 19 patients (mean age 7 months). Orbital volume on affected side was significantly lower (p = 0.001), mean orbital volume ratio was 0.93 (SD 0.03). No significant differences in group means of orbital volume ratio between different levels of severity of UCSQ were found (Kruskal-Wallis H (2) = 0.873; p > 0.05). Ophthalmic sequelae were found in 3 patients; one had adduction impairment and strabismus (mild UCS), one had astigmatism (moderate UCS), and one had abduction impairment (on both ipsi- and contralateral side) and vertical strabismus (severe UCS)., Conclusion: No association between orbital volume ratio and severity of UCS was found. Side-to-side asymmetry in orbital volume was noted. No association between either preoperative orbital volume ratio or severity of UCS and the presence of preoperative ophthalmic sequelae was found.

Background: Timing of frontofacial surgery for the syndromic craniosynostosis as it relates to various surgical risks has not been adequately studied. The purpose of this study was to investigate posterior dental complications of midface advancement in patients with syndromic craniosynostosis undergoing surgery at different ages and the effects on subsequent orthognathic surgery., Methods: A retrospective chart review of patients with syndromic craniosynostosis treated with midface advancement (monobloc or Le Fort III) from 1999 to 2018 was carried out. Patient demographics, records, and imaging studies were reviewed. A subanalysis of those patients who were also treated with orthognathic surgery from 2014 to 2018 with imaging studies available for analysis was also performed., Results: Thirty-seven patients met the inclusion criteria. Sixty-four percent of the patients had radiographic evidence of maxillary molar dental abnormality. Older age at the time of surgery was significantly associated with a lower odds of sustaining dental injury (OR, 0.55; p = 0.034). The odds of damaging second or third maxillary molars was significantly higher with a younger age at the time of surgery (p = 0.021 and p = 0.034). The odds of sustaining dental injury increased moving posteriorly, showing the risk of abnormal pattern of M3 greater than M2 greater than M1. Advanced age at the time of surgery was significantly associated with decreased odds of dental injury (OR, 0.55; p = 0.034)., Conclusions: Damage to the developing permanent maxillary molars may affect orthodontic management, mastication, and potentially maxillary development. Delaying frontofacial surgery until development of the permanent maxillary dentition should be considered if other indications do not mandate earlier intervention., Competing Interests: Disclosure:The authors report no conflicts of interest., (Copyright © 2020 by the American Society of Plastic Surgeons.)

Introduction: Nonsyndromic, multi-suture craniosynostosis is not common, especially those involving unilateral coronal and lambdoid sutures. Based on the experience on 6 cases, we analyzed the skull morphology of combined unilateral coronal-lambdoid suture synostosis and evaluated the surgical outcome of suturectomy., Methods: Patients who underwent an operation for craniosynostosis in Seoul National University Children's Hospital from 2010 to 2018 were reviewed. For qualitative analysis of the surgical outcome, five typical morphologic characteristics (ipsilateral superior orbital rim deviation, deviation of the nasal ridge, ipsilateral frontal flattening, contralateral parietal bulging, ipsilateral occipitomastoid bulging) in unilateral coronal-lambdoid suture synostosis were evaluated based on medical photos, plain skull radiographs, and CT scans. For quantitative analysis, three parameters (nasal root deviation, orbital asymmetry, posterior skull base deviation) were measured., Results: Among 316 patients with craniosynostosis, 41 patients had nonsyndromic, multi-suture synostosis. There were 6 unilateral coronal-lambdoid suture synostosis patients who were all treated with suturectomy. Qualitative evaluation of the postoperative outcome revealed that the nasal root and orbital rim deviations and the contralateral occipitomastoid bulging showed satisfactory improvements. However, ipsilateral frontal flattening and contralateral parietal bulging were improved but still present in most cases. Quantitative, craniometric analysis supported the qualitative outcomes. The nasal ridge deviation angle was corrected from 7.04 to 1.79 degrees. The posterior skull base deviation angle improved from 6.29 to 3.55 degrees., Conclusion: Unilateral coronal-lambdoid suture synostosis suturectomy followed by helmet therapy resulted in favorable outcomes, although the correction of frontal flattening was less satisfactory than the other measures. Considering the minimal invasiveness of the treatment, suturectomy may be a viable option for these patients.

Background: Spring-assisted surgery is an accepted alternative to cranial vault remodeling for treatment of sagittal craniosynostosis. The long-term safety and efficacy profiles of spring-assisted surgery have not been established., Methods: This study is a retrospective examination of all patients treated with spring-assisted surgery (n = 175) or cranial vault remodeling (n = 50) for sagittal craniosynostosis at the authors' institution from 2003 to 2017. Data collected included demographic and operative parameters, preoperative and postoperative Cephalic Indices, and complications. Whitaker grades were assigned blindly by a craniofacial surgeon not involved in patients' care., Results: The mean age at surgery was significantly lower for the spring-assisted surgery group compared with the cranial vault remodeling group (4.6 months versus 22.2 months; p < 0.001). Even when combining spring placement with spring removal operations, total surgical time (71.1 minutes versus 173.5 minutes), blood loss (25.0 ml versus 111.2 ml), and hospital stays (41.5 hours versus 90.0 hours) were significantly lower for the spring-assisted surgery cohort versus the cranial vault remodeling group (p < 0.001 for all). There were no differences in infection, reoperation rate, or headaches between the groups. The percentage improvement in Cephalic Index was not significantly different at 1 (p = 0.13), 2 (p = 0.99), and 6 (p = 0.86) years postoperatively. At 12 years postoperatively, the spring-assisted surgery group had persistently improved Cephalic Index (75.7 preoperatively versus 70.7 preoperatively). Those undergoing spring-assisted surgery had significantly better Whitaker scores, indicating less need for revision surgery, compared with the cranial vault remodeling group (p = 0.006)., Conclusion: Compared with the authors' cranial vault remodeling technique, spring-assisted surgery requires less operating room time and is associated with less blood loss, but it has equivalent long-term Cephalic Indices and subjectively better shape outcomes., Clinical Question/level of Evidence: Therapeutic, III.

: A question that remains unanswered is at what level of surgical correction does the public perceive a head shape to be "normal" or "acceptable?" For most cases of non-syndromic asymptomatic craniosynostosis, the parents desire for surgical correction is to improve the cosmetic appearance of head shape. At the time of this writing, the intraoperative surgeons' perspective of what constitutes an acceptable head shape is the target for surgical correction. In introducing an improved objectively cosmetic goal, an appropriate outcome measure would be to assess what the general public considers a normal or acceptable head shape in children with craniosynostosis., Method: Twenty-two unique images were presented via an online crowdsourcing survey of a severe case of non-syndromic sagittal craniosynosis gradually corrected to an age and gender matched normalized head shape. Participants were recruited via the Sick Kids Twitter account. Participants were invited to rate the head shapes as "normal" or "abnormal.", Results: The 538 participants completed the online survey. Participants were able to reliably and consistently identify normal and abnormal head shapes with a Kappa Score >0.775. Furthermore, participants indicated that a correction of 70% is required in order for the cranial deformity to be regarded as "normal." This threshold closely reflects a normal Cranial Index, which is a widely used morphometric outcome in craniosynostosis., Conclusion: Crowdsourcing provides an ideal method for capturing the general population's perspective on what constitutes a normal and acceptable head shape in children with sagittal craniosynostosis. Laypersons are able to reliably and consistently distinguish cranial deformities from a "normal" head shape. The public indicates a threshold correction of 70% in sagittal craniosynosis to regard it as a "normal" head shape.