Eyeing Risks: A Critical Analysis of the Use of Periorbital Steroids in Fronto-orbital Advancement.

OBJECTIVE: To evaluate the outcomes associated with two techniques of periorbital steroid administration in bilateral fronto-orbital advancement (FOA). DESIGN: Multi-institutional retrospective chart review. SETTING: Two high volume, tertiary US craniofacial centers. PATIENTS, PARTICIPANTS: Patients who underwent FOA between 2012 and 2021. INTERVENTIONS: Patients were divided into three cohorts based on method of steroid administration. Groups GEL and INJ represent those who received steroids in the form of triamcinolone soaked gelfoam or direct injection of dilute triamcinolone to the frontal/periorbital region, respectively. Group NON did not receive any periorbital steroids. MAIN OUTCOME MEASURE(S): Peri-operative outcomes including hospital length of stay and complications were evaluated based on method of periorbital steroid administration. Variables predictive of infectious complications were assessed using stepwise logistic regression. RESULTS: Four hundred and twelve patients were included in our sample (INJ:249, GEL:87, NON:76). Patients in the INJ group had a higher ASA class (P < .001) while patients in the NON group were significantly more likely to be syndromic (P < .001) and have multisuture craniosynostosis (P < .001). Rate of infectious complications for each cohort were NON: 2.6%, INJ: 4.4%, and GEL: 10.3%. There was no significant difference between groups in hospital length of stay (P = .654) or rate of post-operative infectious complications (P = .061). Increased ASA class (P = .021), increased length of stay (P = .016), and increased intraoperative narcotics (P = .011) were independent predictors of infectious complications. CONCLUSIONS: We identified a dose-dependent relationship between periorbital steroids and rate of postoperative infections, with key contributions from ASA class, hospital length of stay, and dose of intraoperative narcotics.

Single suture craniosynostosis: Identification of rare variants in genes associated with syndromic forms.

We report RNA-Sequencing results on a cohort of patients with single suture craniosynostosis and demonstrate significant enrichment of heterozygous, rare, and damaging variants among key craniosynostosis-related genes. Genetic burden analysis identified a significant increase in damaging variants in ATR, EFNA4, ERF, MEGF8, SCARF2, and TGFBR2. Of 391 participants, 15% were found to have damaging and potentially causal variants in 29 genes. We observed transmission in 96% of the affected individuals, and thus penetrance, epigenetics, and oligogenic factors need to be considered when recommending genetic testing in patients with nonsyndromic craniosynostosis.

Facial Expressiveness in Infants With and Without Craniofacial Microsomia: Preliminary Findings.

OBJECTIVE: To compare facial expressiveness (FE) of infants with and without craniofacial macrosomia (cases and controls, respectively) and to compare phenotypic variation among cases in relation to FE. DESIGN: Positive and negative affect was elicited in response to standardized emotion inductions, video recorded, and manually coded from video using the Facial Action Coding System for Infants and Young Children. SETTING: Five craniofacial centers: Children's Hospital of Los Angeles, Children's Hospital of Philadelphia, Seattle Children's Hospital, University of Illinois-Chicago, and University of North Carolina-Chapel Hill. PARTICIPANTS: Eighty ethnically diverse 12- to 14-month-old infants. MAIN OUTCOME MEASURES: FE was measured on a frame-by-frame basis as the sum of 9 observed facial action units (AUs) representative of positive and negative affect. RESULTS: FE differed between conditions intended to elicit positive and negative affect (95% confidence interval = 0.09-0.66, P = .01). FE failed to differ between cases and controls (ES = -0.16 to -0.02, P = .47 to .92). Among cases, those with and without mandibular hypoplasia showed similar levels of FE (ES = -0.38 to 0.54, P = .10 to .66). CONCLUSIONS: FE varied between positive and negative affect, and cases and controls responded similarly. Null findings for case/control differences may be attributable to a lower than anticipated prevalence of nerve palsy among cases, the selection of AUs, or the use of manual coding. In future research, we will reexamine group differences using an automated, computer vision approach that can cover a broader range of facial movements and their dynamics.

Characterizing facial features in individuals with craniofacial microsomia: A systematic approach for clinical research.

BACKGROUND: Craniofacial microsomia (CFM) is a congenital condition with wide phenotypic variability, including hypoplasia of the mandible and external ear. We assembled a cohort of children with facial features within the CFM spectrum and children without known craniofacial anomalies. We sought to develop a standardized approach to assess and describe the facial characteristics of the study cohort, using multiple sources of information gathered over the course of this longitudinal study and to create case subgroups with shared phenotypic features. METHODS: Participants were enrolled between 1996 and 2002. We classified the facial phenotype from photographs, ratings using a modified version of the Orbital, Ear, Mandible, Nerve, Soft tissue (OMENS) pictorial system, data from medical record abstraction, and health history questionnaires. RESULTS: The participant sample included 142 cases and 290 controls. The average age was 13.5 years (standard deviation, 1.3 years; range, 11.1-17.1 years). Sixty-one percent of cases were male, 74% were white non-Hispanic. Among cases, the most common features were microtia (66%) and mandibular hypoplasia (50%). Case subgroups with meaningful group definitions included: (1) microtia without other CFM-related features (n = 24), (2) microtia with mandibular hypoplasia (n = 46), (3) other combinations of CFM- related facial features (n = 51), and (4) atypical features (n = 21). CONCLUSION: We developed a standardized approach for integrating multiple data sources to phenotype individuals with CFM, and created subgroups based on clinically-meaningful, shared characteristics. We hope that this system can be used to explore associations between phenotype and clinical outcomes of children with CFM and to identify the etiology of CFM. Birth Defects Research (Part A) 106:915-926, 2016.© 2016 Wiley Periodicals, Inc.

Phenotypic sub-grouping in microtia using a statistical and a clinical approach.

The clinical presentation of microtia varies widely from minimal morphological abnormalities to complete absence of the ear. In this study we sought to identify and characterize sub-groups of microtia using a statistical and a clinical approach. Photographs of 86 ears were classified in relation to all the external ear components. We used cluster analysis and rater's clinical opinion to identify groups with similar phenotypes in two separate analyses. We used Cramer's Phi coefficient of association to assess the similarity among the clinician's groupings as well as among the statistical sub-phenotypic groups and each of the clinician's groupings. The cluster analysis initially divided the 86 ears into a more and a less severe group. The less severe group included two sub-groups that included ears classified as normal and a group that had very few anomalous components. The group of 48 more affected ears all had abnormalities of the helix crus; antihelix-stem, -superior crus and -inferior crus; and antitragus. These were further divided into 4 sub-phenotypes. There was a moderate degree of association among the raters' groupings (Cramer's Phi: 0.64 to 0.73). The statistical and clinical groupings had a lower degree of association (Cramer's Phi: 0.49 to 0.58). Using standardized characterization of structural abnormalities of the ear we identified six distinct phenotypic groups; correlations with clinicians' groupings were moderate. These clusters may represent groups of ear malformations associated with the same etiology, similar time of insult or target cell population during embryonic development. The results will help inform investigations on etiology.

Safety of Open Cranial Vault Surgery for Single-Suture Craniosynostosis: A Case for the Multidisciplinary Team.

INTRODUCTION: Single Suture Craniosynostosis (SSC) occurs in 1 in 2,500 live births and is the most common type of craniosynostosis treated in most centers. Surgical treatment has evolved over the past century and open techniques are tailored to the specific suture type. Additionally, the concept of multi-disciplinary team care has proliferated and is becoming the standard of care for SSC. The combination of these evolutions, we believe, has improved the safety of cranial vault surgery for SSC. METHODS: A retrospective review of patients participating in the Infant Learning Project at Seattle Children's Hospital who underwent cranial vault surgery for treatment of SSC between 2002 and 2006 was performed. Pre-operative assessment, surgical techniques, anesthetic and intraoperative events and both intra-operative and post-operative adverse events were analyzed. RESULTS: Eighty eight patients fulfilled the inclusion criteria (42 sagittal, 23 metopic, 19 unicoronal, 4 lambdoid). Length of procedure varied (FOA 5.2 hrs, modified pi 2.5 hrs, total vault 4.9 hrs and switch cranioplasty 4.6 hrs), as did transfusion amount (FOA 385 mL, modified pi 216 mL, total vault 600 mL, switch cranioplasty 207 mL) although 99% of patients received a transfusion of some sort. There were no deaths and no major intraoperative complications. Minor events include; ET tube malposition (1), desaturation (1), acidosis (1), hypothermia (9), coagulopathy (2), Hct < 25 (55). Average hospital stay was 3.4 days with no major post-operative complications. One patient was readmitted to the ICU and 1 had a scalp hematoma, but no patients returned to the operating room within 6 months after surgery. DISCUSSION: The surgical treatment of SSC has evolved from lengthy, risky procedures to become almost routine at most craniofacial centers. Additionally, the care for patients with SSC has evolved from a single provider to a multidisciplinary team concept based around protocols for workup, delivery of anesthesia, streamlined surgical procedures and post-operative care and assessment. This evolution has given open cranial vault surgery for SSC an acceptable safety profile.

What to do with an incidental finding of a fused sagittal suture: a modified Delphi study.

OBJECTIVE: As many as 5% of normocephalic children may have a prematurely fused sagittal suture, yet the clinical significance and best course of management of this finding remain unclear. Providers in the Synostosis Research Group were surveyed to create a multicenter consensus on an optimal treatment and monitoring algorithm for this condition. METHODS: A four-round modified Delphi method was utilized. The first two rounds consisted of anonymous surveys distributed to 10 neurosurgeons and 9 plastic surgeons with expertise in craniosynostosis across 9 institutions, and presented 3 patients (aged 3 years, 2 years, and 2 months) with incidentally discovered fused sagittal sutures, normal cephalic indices, and no parietal dysmorphology. Surgeons were queried about their preferred term for this entity and how best to manage these patients. Results were synthesized to create a treatment algorithm. The third and fourth feedback rounds consisted of open discussion of the algorithm until no further concerns arose. RESULTS: Most surgeons preferred the term "premature fusion of the sagittal suture" (93%). At the conclusion of the final round, all surgeons agreed to not operate on the 3- and 2-year-old patients unless symptoms of intracranial hypertension or papilledema were present. In contrast, 50% preferred to operate on the 2-month-old. However, all agreed to utilize shared decision-making, taking into account any concerns about future head shape and neurodevelopment. Panelists agreed that patients over 18 months of age without signs or symptoms suggesting elevated intracranial pressure (ICP) should not undergo surgical treatment. CONCLUSIONS: Through the Delphi method, a consensus regarding management of premature fusion of the sagittal suture was obtained from a panel of North American craniofacial surgeons. Without signs or symptoms of ICP elevation, surgery is not recommended in patients over 18 months of age. However, for children younger than 18 months, surgery should be discussed with caregivers using a shared decision-making process.

Interrater reliability of a phenotypic assessment tool for the ear morphology in microtia.

The Elements of Morphology Standard Terminology working group published standardized definitions for external ear morphology. The primary objective of our study was to use these descriptions to evaluate the interrater reliability for specific features associated with microtia. We invited six raters from three different subspecialities to rate 100 ear photographs on 32 features. We calculated overall and within specialty and professional experience intraclass correlation coefficients (ICC) and 95% confidence intervals. A total of 600 possible observations were recorded for each feature. The overall interrater reliability ranged from 0.04 (95% CI: 0.00-0.14) for the width of the antihelix inferior crus to 0.93 (95% CI: 0.91-0.95) for the presence of the inferior crux of the antihelix. The reliability for quantitative characteristics such as length or width of an ear structure was generally lower than the reliability for qualitative characteristics (e.g., presence or absence of an ear structure). Categories with very poor interrater reliability included anti-helix inferior crux width (0.04, 95% CI: 0.00-0.14), crux helix extension (0.17, 95% CI 0.00-0.37), and shape of the incisura (0.14, 95% CI: 0.01-0.27). There were no significant differences in reliability estimates by specialty or professional experience for most variables. Our study showed that it is feasible to systematically characterize many of structures of the ear that are affected in microtia. We incorporated these descriptions into a standardized phenotypic assessment tool (PAT-Microtia) that might be used in multicenter research studies to identify sub-phenotypes for future studies of microtia.

Making the diagnosis: metopic ridge versus metopic craniosynostosis.

INTRODUCTION: The metopic suture is the only calvarial suture which normally closes during infancy. Upon closure, a palpable and visible ridge often forms which can be confused with metopic craniosynostosis. Metopic ridging (MR) is treated nonsurgically while metopic craniosynostosis (MCS) is treated surgically. Differentiating between the two is paramount; however, consensus is lacking about where a clear diagnostic threshold lies. The goal of this study is to describe the physical examination and CT scan characteristics which may help to differentiate between physiological closure of the metopic suture with ridging (MR) and MCS. METHODS: A retrospective chart review of all patients seen at Seattle Children's Hospital between 2004 and 2009 with the diagnosis of either MCS or MR (n = 282) was performed. Physical examination characteristics described by diagnosing practitioners were analyzed. Clinical photos were assessed by 3 expert raters to determine the importance of these characteristics. CT scan findings were abstracted and compared between the two diagnoses. RESULTS: The "classic" triad of narrow forehead, biparietal widening, and hypotelorism was present in only 14% of patients with MCS. Ninety-eight percent of patients in both groups had a palpable metopic ridge. The photographic finding of narrow forehead and pterional constriction was present in all patients with MCS, but only in 11.2% and 2.8% of patients with MR. On CT scan, the presence of 3 or more MCS findings was diagnostic of MCS in 96% of patients. Patients with MCS were more likely to present before 6 months of age (66% vs. 32%). CONCLUSIONS: Patients with MCS tend to present earlier than those with MR. Upon physical examination, the relationship between the lateral frontal bone and the lateral orbit is important in distinguishing between the two diagnoses. A CT scan can be helpful in making the diagnosis not to confirm a closed suture but to identify 3 or more MCS characteristics.

Comparison of two-dimensional and three-dimensional images for phenotypic assessment of craniofacial microsomia.

Background and Purpose : Three-dimensional surface imaging is used in many craniofacial centers. However, few data exist to indicate whether such systems justify their cost. Craniofacial microsomia is associated with wide phenotypic variability and can affect most facial features. The purpose of this study is to compare three-dimensional versus two-dimensional images for classification of facial features in individuals with craniofacial microsomia. Methods : We obtained a series of two-dimensional and three-dimensional images of 50 participants, aged 0-20 years, diagnosed with craniofacial microsomia, microtia, or Goldenhar syndrome. Three clinicians classified the craniofacial features on each image, and ratings were compared by calculating kappa statistics. We also evaluated image quality using a 5-point Likert scale. Results : Reliability estimates were high for most features using both two-dimensional and three-dimensional image data. Our three-dimensional protocol did not allow for scoring of facial animation, occlusal cant, or tongue anomalies. Image quality scores for the mandible and soft tissue assessment were higher for three-dimensional images. Raters preferred two-dimensional photographs for assessment of the ear, ear canal, and eyes. Conclusions : Both three-dimensional and two-dimensional images provide useful data for objective characterization of the craniofacial features affected in craniofacial microsomia. A series of two-dimensional images has relative advantages for assessment of some specific features, such as the ear, though three-dimensional images may have advantages for quantitative analysis and qualitative assessment of deformities of the jaw and soft tissue. These results should apply to any assessment of these features with or without a craniofacial microsomia diagnosis.

Pediatric Panfacial Fractures.

Pediatric panfacial trauma is a rare occurrence with poorly understood implications for the growing child. Treatment algorithms largely mirror adult panfacial protocols with notable exceptions including augmented healing and remodeling capacities that favor nonoperative management, limited exposure to avoid disruption of osseous suture and synchondroses growth centers, and creative fracture fixation techniques in the setting of an immature craniomaxillofacial skeleton. The following article provides a review of our institutional philosophy in the management of these challenges injuries with important anatomic, epidemiologic, examination, sequencing, and postoperative considerations.

Morphometric Outcomes of Nonsyndromic Sagittal Synostosis following Open Middle and Posterior Cranial Vault Expansion.

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.

Cranial Shape Changes in Nonsyndromic Unilateral Lambdoid Synostosis after Open Posterior Vault Remodeling.

BACKGROUND: The purpose of this study was to quantify change in cranial morphology in patients with nonsyndromic unilateral lambdoid craniosynostosis (ULC) from presentation (t0), after open posterior switch-cranioplasty (t1), and at 2-year follow-up (t2). METHODS: Volumetric, linear, and angular analysis were performed on computed tomographic scans at the three time points and against normal control subjects. Significance was set at P < 0.05. RESULTS: Twenty-two patients were included. ULC cranial vault asymmetry index was higher than in control subjects before surgery (6.22 ± 3.55) but decreased after surgery (3.00 ± 2.53) to become comparable with the normal asymmetry range present in the controls. After surgery, both diagonals increased, but more on the fused side. In the 2 years after surgery, both diagonals in patients with ULC grew proportionately, but the fused diagonal remained slightly shorter than the patent side. Total cranial volume was higher in patients with ULC than in control subjects after surgery but became comparable at t2. Cranial base angulation improved by t2 but did not approach normal, and ear position remained unchanged. The facial twist was higher than in controls at t0 and t1 but was comparable at t2. Coronal asymmetry improved with surgery but remained undercorrected at t2, with the greatest residual asymmetry at opisthion. CONCLUSIONS: Open-switch cranioplasty normalizes cranial vault asymmetry index by increasing the fused cranial diagonal more than the patent side and is stable at 2 years. Skull base twist does not normalize, but facial twist approaches normal. Technique improvement should focus on residual coronal asymmetry present at opisthion. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Two Year Comparison of Sagittal Synostosis Morphometric Outcomes Following Open Posterior Expansion Versus Endoscopic Strip Craniectomy with Helmet Molding Performed before Four Months of Age.

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 two-year cranial morphometrics after these two approaches. METHODS: We performed morphometric analysis on preoperative (t0), immediately post-operative (t1) and 2-year (t2) postoperative CT scans of patients who underwent OPVE or ES prior to 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 in the OPVE cohort, and 57 as controls. Median surgery time and blood transfusion volume were less for the ES approach (118 min; 0cc) compared to OPVE (204 min; 250cc). Anthropometric measurements after OPVE were closer normal controls at t1 compared to ES, but the skull shapes were comparable at t2. In the mid-sagittal plane, anterior vault was higher after OPVE at t2 compared to both ES and controls, but the posterior length was shorter and closer to controls than 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 two 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. LEVEL OF EVIDENCE: III.

Duration of exposure to cranial vault surgery: associations with neurodevelopment among children with single-suture craniosynostosis.

OBJECTIVE: To evaluate associations between neurodevelopment and exposure to surgery and anesthetic agents in children with single-suture craniosynostosis (SSC). BACKGROUND: Young children with SSC have unexplained neurodevelopmental delays. The possible contributions of factors related to cranial vault surgery - including anesthesia - have not been previously examined. METHODS/MATERIALS: Two anesthesiologists reviewed the surgical records of 89 infants (70 had complete data). Primary exposures were duration of surgery and anesthesia and total duration of inhaled anesthesia (at age 6 months on average). Outcomes were the cognitive and motor scores from the Bayley Scales of Infant Development-II and language scores from the Preschool Language Scale, 3rd edition, given at age 36 months. Linear regression using robust standard error estimates was performed, adjusting for age at surgery and suture site. RESULTS: Anesthesia duration ranged from 155 to 547 min. For every 30-min increase in anesthesia duration, the estimated average decrease in developmental test scores ranged from 1.1 to 2.9 (P ranged from <0.001 to 0.30). Similar, but weaker findings were observed with surgery duration and total duration of inhaled anesthesia. Inverse relations between exposure amounts and neurodevelopment were stronger in children with nonsagittal synostosis. CONCLUSIONS: Average neurodevelopmental scores were lower among children experiencing longer surgeries and higher exposures to inhaled anesthesia. These associations may be due to anesthesia exposure, nonspecific effects of surgery, or unmeasured variables that correlate with surgery duration. Further study of potential causal mechanisms is warranted.

Syndromic Craniosynostosis: Cranial Vault Expansion in Infancy.

Syndromic craniosynostosis (CS) represents a relatively uncommon disease process that poses significant reconstructive challenges for the craniofacial surgeon. Although there is considerable overlap in clinical features associated with various forms of syndromic CS, key extracranial features and close examination of the extremities help to distinguish the subtypes. While Virchow's law can easily guide the diagnosis of single suture, nonsyndromic CS, syndromic CS traditionally results in atypical presentations inherent to multiple suture fusion. Coronal ring involvement in isolation or associated with additional suture fusion is the most common pattern in syndromic CS often resulting in turribrachycephaly.

Management of Sagittal and Lambdoid Craniosynostosis: Minimally Invasive Approaches.

The resurgence of strip craniectomies began in the mid-1990s with advances in surgical technique and anesthesia coupled with the critical observation that earlier interventions benefitted from an easily molded skull. Jimenez and Barone's pioneering introduction of endoscopic approaches to strip craniectomies coupled with postoperative helmeting in newborns and young infants and Claes Lauritzen's introduction of spring-mediated cranioplasty began the era of minimally invasive approaches in the surgical correction of craniosynostosis. This article provides technical descriptions of these treatment modalities, a comparative literature review, and our institutional algorithms for the correction of sagittal craniosynostosis and unilambdoid craniosynostosis.

Multidisciplinary Care Considerations for Patients with Craniosynostosis.

Infants and children with craniosynostosis require multidisciplinary care, and this is best accomplished when care is provided on a craniofacial team. Most patients with craniosynostosis will have non-syndromic presentations; however, longitudinal care remains critical to ensure appropriate growth and development throughout childhood. In patients with syndromic craniosynostoses, coordinated longitudinal care becomes even more paramount because of the high level of complexity across many different specialties or disciplines. Care delivery that includes perspective and expertise from multiple disciplines is important to help patients reach their full potential and optimal outcomes.

Morphologic Differences in Sagittal Synostosis with Age before Surgery.

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.

Evolution of Cranioorbital Shape in Nonsyndromic, Muenke, and Saethre-Chotzen Bilateral Coronal Synostosis: A Case-Control Study of 2-Year Outcomes.

BACKGROUND: The purpose of this study was to quantify change in cranioorbital morphology from presentation, after fronto-orbital advancement, and at 2-year follow-up. METHODS: Volumetric, linear, and angular analyses were performed on computed tomographic scans of consecutive bilateral coronal synostosis patients. Comparisons were made across three time points, between syndromic and nonsyndromic cases, and against normal controls. Significance was set at p < 0.05. RESULTS: Twenty-five patients were included: 11 were nonsyndromic, eight had Saethre-Chotzen syndrome, and six had Muenke syndrome. Total cranial volume was comparable to normal, age-matched control subjects before and 2 years after surgery despite an expansion during surgery. Axial and sagittal vector analyses showed advancement and widening of the lower forehead beyond control values with surgery and comparable anterior position, but increased width compared to controls at 2 years. Frontal bossing decreased with a drop in anterior cranial height and advanced lower forehead position. Middle vault height was not normalized and turricephaly persisted at follow-up. Posterior fossa volume remained lower at all three time points compared to control subjects. Supraorbital retrusion relative to anterior corneal position was overcorrected by surgery, with values comparable to those of control subjects at 2 years because of differential growth. There was no difference at 2 years between syndromic and nonsyndromic groups. CONCLUSIONS: Open fronto-orbital advancement successfully remodels the anterior forehead but requires overcorrection to be comparable to normal at 2 years. Although there are differences in syndromic cases at presentation, they do not result in significant morphometric differences on follow-up. Posterior fossa volume remains lower at all time points. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.