Sagittal Craniosynostosis Research Articles

Evaluating Tranexamic Acid Administration in Cranial Vault Reconstruction: The Experience of an Academic Center in the Deep South

Background: In infants with craniosynostosis, cranial vault reconstruction is performed to prevent sequela of premature fusion of cranial sutures. Open correction puts patients at risk for complications of major blood loss. We evaluated the impact of tranexamic acid (TXA) in children undergoing open surgical repair of a variety of types of craniosynostosis. Methods: A retrospective review of 102 infants who underwent open cranial vault reconstruction between January 2015 and December 2020 at a tertiary referral hospital was performed. The patients were stratified into TXA or non-TXA based on usage. Outcome measures included volume of blood transfused, perioperative blood loss and any adverse effects were noted. Results: In this cohort 42% of patients were treated with TXA. There was no significant difference between the patient demographics of TXA and non-TXA cohorts with the majority classified as Sagittal Craniosynostosis ( P = .1062), an average weight of 8.89 ± 1.37 kg, and age of 9.02 ± 2.02 months at time of surgery. The non-TXA cohort had longer length of hospital stay ( P = .04). The TXA cohort had an average 100 ml decrease in surgical drain output over the course of their hospital stay ( P = .02). Overall surgical complication was 14% ( P = .18) and clinical outcomes were not significantly different between the cohorts. Conclusions: The receipt of TXA in the interoperative period in patients with craniosynostosis undergoing cranial vault remodeling was associated with a decreased surgical drain output and length of stay.

Comparison of Fluid Replacement with Sterofundin ISO® vs. Deltajonin® in Infants Undergoing Craniofacial Surgery-A Retrospective Study.

The purpose of this study was to compare the effects of Sterofundin ISO® (SF) therapy with the balanced isochloremic solution Deltajonin® (DJ) (106 mmol/L chloride) on the acid-base status in infants undergoing craniofacial surgery. This retrospective, non-blinded study included 100 infants undergoing craniectomy due to isolated nonsyndromic sagittal craniosynostosis. The first 50 infants received Sterofundin ISO®. Due to changes in national guidelines, the infusion was changed to the isoionic Deltajonin® in an additional 50 infants in 2017. Pre- and postoperative values of chloride, pH, base excess, bicarbonate, and albumin and phosphate were determined, and the strong-ion difference, strong-ion gap, anion gap, and weak acids were calculated. Both groups were comparable in terms of their age, sex, underlying disease, preoperative electrolytes (except K at 3.9 ± 0.3 mmol/L (SF) vs. 4.1 ± 0.3 mmol/L (DJ) and lactate 8.7 ± 2.1 (SF) vs. 9.6 ± 2.6 mmol/L (DJ)). In the Sterofundin ISO® group, hyperchloremic metabolic acidosis was observed in 19 patients, whereas only 2 infants in the Deltajonin® group had hyperchloremic metabolic acidosis. The postoperative chloride level was 111 ± 2.7 mmol/L (SF) vs. 108 ± 2.4 mmol/L (DJ). The difference in anion gap was 12.5 ± 3.0 mmol/L (SF) vs. 14.6 ± 2.8 mmol/L (DJ), and the difference in SIDa (apparent strong-ion difference) was 30.9 mmol/L (SF) vs. 33.8 mmol/L (DJ). Hyperchloremic acidosis can be induced by the volume replacement with high-chloride-concentration crystalloids such as Sterofundin ISO®. This can be detected using the Stewart model.

Preoperative Frontal and Parietal Bone Thickness Assessment to Predict Blood Loss and Transfusion During Extended Suturectomy for Isolated Sagittal Craniosynostosis.

To predict the morbidity of sagittal suturectomy using preoperative computer tomographic measurement of frontal and parietal bone thickness in osteotomy sites. Retrospective analysis. Tertiary children's hospital. Fifty infants with nonsyndromic, isolated sagittal craniosynostosis who underwent extended sagittal suturectomy from 2015-2022. Mean thickness of the frontal and parietal bone in regions of osteotomies were determined for each patient from preoperative CT images obtained within 30 days prior to suturectomy. The relationship between bone thickness (mm) and estimated blood loss (mL) was evaluated using Spearman's correlation and a multivariable model that adjusted for patient weight and surgery duration. The association between bone thickness and perioperative blood transfusion was evaluated using a multivariable logistic model controlling for patient weight and surgery duration. Estimated blood loss, perioperative blood transfusion. Frontal and parietal bone thickness in the region of osteotomies were positively correlated with estimated blood loss (p < 0.01). After adjusting for patient weight and duration of operation, both parietal and frontal bone thickness were associated with intraoperative blood loss (R2 = 0.292, p = 0.002 and R2 = 0.216, p = 0.026). Thicker frontal and parietal bone in the line of osteotomies resulted in significantly higher odds of blood transfusion. Bone thickness in the line of parietal osteotomies was 76% accurate at identifying patients who would require blood transfusion (p = 0.004). Frontal and parietal bone thickness in the line of osteotomies is associated with blood loss and perioperative blood transfusion for sagittal suturectomy operations.

A New Measure of Posterior Morphology in Sagittal Craniosynostosis: The Occipital Bullet Index

Introduction: Sagittal craniosynostosis (SC) is associated with scaphocephaly, an elongated narrow head shape. Assessment of regional severity in the scaphocephalic head is limited by the use of serial computed tomographic (CT) imaging or complex computer programing. Three-dimensional measurements of cranial surface morphology provide a radiation-free alternative for assessing cranial shape. This study describes the creation of an occipital bulleting index (OBI), a novel tool using surface morphology to assess the regional severity in patients with SC. Methods: Surface imaging from CT scans or 3D photographs of 360 individuals with SC and 221 normocephalic individuals were compared to identify differences in morphology. Cartesian grids were created on each individual’s surface mesh using equidistant axial and sagittal planes. Area under the curve (AUC) analyses were performed to identify trends in regional morphology and create measures capturing population differences. Results: The largest differences were located in the medial regions posteriorly. Using these population trends, a measure was created to maximize AUC. The OBI has an AUC of 0.72 with a sensitivity of 74% and a specificity of 61%. When the frontal bossing index is applied in tandem, the two have a sensitivity of 94.7% and a specificity of 93.1%. Correlation between the two scores in individuals with SC was found to be negligible with an intraclass correlation coefficient of 0.018. Severity was found to be independent of age under 24 months, sex, and imaging modality. Conclusions: This index creates a tool for differentiating control head shapes from those with SC and has the potential to allow for objective evaluation of the regional severity, outcomes of different surgical techniques, and tracking shape changes in individuals over time, without the need for radiation.

Atypical sagittal suture craniosynostosis: pathological considerations for early closure of the anterior part of the sagittal suture.

Sagittal suture synostosis is one of the most common craniosynostoses and is often diagnosed by characteristic narrow and long skull shape, scaphocephaly. However, some patients with sagittal suture synostosis do not present with typical scaphocephaly, making early diagnosis difficult. In this study, five cases of characteristic skull deformity showing a narrowing of the cranium posterior to the coronal suture on computed tomography (CT) are presented. The three older children presented with papilledema and intellectual disability and a closed sagittal suture on CT. The two infant cases were diagnosed with the characteristic cranial deformities with aggravation of the deformity over time, but sagittal suture closure was not evident on CT. All patients underwent cranial remodeling surgery. In the two infant cases, the histopathological findings showed that the anterior part of the sagittal suture was firmly fused with fibrous tissue without bony fusion. These findings suggested that narrowing of the cranium posterior to the coronal suture might be due to functional fusion of the anterior portion of the sagittal suture prior to bony fusion. In an infant presenting with such a deformity that shows aggravation of the deformity over time, surgical treatment should be considered.

The Accuracy of Classification Systems in Nonsyndromic Sagittal Craniosynostosis.

Numerous classification systems of nonsyndromic sagittal craniosynostosis (NSC) are applied but none has gained a wide acceptance, since each classification is focused on distinct aspects. The aim of the study was to assess the accuracy of 4 classifications of NSC discussed in the literature by defining the associations among the classifications, individual features (sex, age, cranial index), and objective morphologic criteria (frontal bossing, retrocoronal constriction, sagittal ridge, and occipital bulleting). The study was conducted on anonymized thin-cut CT scans of 133 children with NSC 1 to 12 months old (mean age 5.42mo). The type of cranial dysmorphology was assessed using 4 classification systems, focusing on skull shape, pattern of sagittal suture closure (Heuzé classification), deformation of skull vault (Sakamoto classification), and a single-dominant feature (David classification). Each patient was also independently investigated for the presence of morphologic criteria. A multivariate analysis was performed to explore the relations among the classifications and assess their accuracy. In the analyzed cohort sphenocephaly (38.3%), CFF type by Heuzé (30.8%), type I by Sakamoto (72.9%), and a central type by David (42.9%) were dominant findings. Regarding the morphologic criteria, frontal bossing was observed the most frequently (91.7%). The age of patients and cranial index differed significantly among the shapes of skull and David classifications ( P <0.001). The shape-based system showed the strongest correlation with other classifications and with measurable variables. Other classifications have much in common and some overlap, but none of them constitutes a standalone system to define all aspects of cranial dysmorphology in NSC.

Sagittal Synostectomy With Tension Band Sutures for Correction of Sagittal Craniosynostosis.

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. 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. 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. 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.

Head Circumference is Relatively Larger Than Size: A Novel Screening Tool for Infants With Sagittal Craniosynostosis

Background: The efficacy of minimally invasive techniques for addressing isolated sagittal craniosynostosis decreases with age; thus, timely referral is critical. We sought to identify a screening tool using data from well-child visits. We hypothesized that many infants with sagittal craniosynostosis, but no macrocephaly based on growth chart plotting, in fact have a relative macrocephaly. Methods: All infants with CT and surgically/pathologically confirmed isolated sagittal craniosynostosis were identified. Growth chart data was collected (head circumference (HC), weight, crown-to-toe-length, and World Health Organization (WHO) and Centers for Disease Control and Prevention (CDC) percentiles and deviation ( z-scores)). Relative macrocephaly was defined as head circumference &gt;2SD from size (weight and/or length). Results: Initial pilot screen indicated that WHO growth chart data has utility as a screening tool. Subsequent retrospective case-control study with area under the curve (AUC) analysis revealed that HC:length ratio ≥0.71 is indicative of isolated sagittal craniosynostosis as young as 1 week of age, while HC:length ratio ≥0.67 represents the maximization of sensitivity (0.89) and specificity (1.00) as early as 2 weeks old. Prospective internal validation with logistic regression demonstrated that at ages 1 to 2 months greater discriminatory value HC:length ratio was observed. Conclusions: When length is added to head circumference as a screening tool we may identify an abnormal growth pattern beginning as early as 1 week. This represents an opportunity to design an automated alert easily programed into the electronic medical records and may prompt pediatricians to refer patients to craniofacial surgeons early.

Comparative Analysis of 2D and 3D Metrics for Evaluation of Postoperative Outcomes Following Endoscopic Suturectomy for Sagittal Craniosynostosis.

To assess whether 3-dimensional (3D) volumetrics can be used to track and evaluate postoperative course of patients treated with endoscopic suturectomy for nonsyndromic sagittal synostosis, we compared changes in 2-dimensional (2D) measurements along with 3D volumetric correlates throughout the period of helmet therapy. Forty-six patients treated at our institution with endoscopic suturectomy for sagittal synostosis were retrospectively reviewed. Head circumference (HC), cephalic index (CI), and total cranial volumes (TCVs) were measured at 3 timepoints following surgery using optical surface scans obtained for helmet orthotics. All measurements showed significant differences between timepoints on the analysis of variance ( P <0.001). There was a significant correlation between CI and TCV (r=0.35, P =0.004) and between HC and TCV (r=0.81, P <0.001). The normalized rate of change over the course of treatment was significantly higher for TCV (36.7%) than for CI (8.8%) and HC (8.4%, P <0.001), with no difference between HC and CI. The authors conclude that 3D metrics were able to reliably follow the course of postoperative 2D metrics. There was a direct and linear correlation between HC and CI with TCV. Total cranial volumes showed the highest rate of sustained change at every timepoint. Although CI and HC plateau after the first measurement, TCV continues to adapt over the course of treatment. These results demonstrate the feasibility and value of volumetrics from 3D imaging to provide a more comprehensive evaluation of postoperative surgical outcomes than traditional 2D metrics without the ionizing radiation traditionally utilized for CT to obtain 3D metrics.

Head Shape After Sagittal Craniosynostosis Surgery: Open Versus Endoscopic Strip Craniectomy

Purpose: Nonsyndromic sagittal craniosynostosis is treated surgically to improve skull cosmesis and to decrease the risk of raised intracranial pressure. The purpose of this study is to compare the outcomes of two current surgical techniques for craniosynostosis treatment, open and endoscopic strip craniectomy. Methods: A single institution retrospective chart review was conducted of patients with nonsyndromic sagittal craniosynostosis treated surgically from 2011 to 2016. Patients were divided into two groups based on surgical technique: open or endoscopic strip craniectomy. The head shape was assessed using pre- and postoperative cephalic index (CI). Complications and operative details were compared. Mean absolute CI over time and 95% confidence intervals were graphed. Results: A total of 51 children (36 male, 15 female; 13 open, 38 endoscopic) were included with an average length of follow-up of 27.2 months (range 4-60). The median age at surgery was 4.0 months for open and 3.0 months for endoscopic. There was no significant difference in preoperative CI between endoscopic and open groups (0.67 vs 0.66). The largest improvements in CI were seen 3 to 6 months postoperatively. There was a significant improvement in postoperative CI for both groups (endoscopic 0.75, P = .02; open = 0.74, P &lt; .01). From maximal postoperative CI to &gt;2 year follow up there was no significant regression in CI for the endoscopic group ( P = .12) and a small regression for the open group (−0.02, P = .01). There were no transfusions, brain injuries, or deaths. Patients in the endoscopic group had significantly less blood loss intraoperatively ( P = .01) and a significantly shorter duration of hospital stay compared to the open group ( P &lt; .001). Conclusions: Endoscopic and open surgical techniques are both effective treatments for nonsyndromic sagittal synostosis, with no difference in initial postoperative CI. These findings support the use of either technique and corroborate previous literature.

School-age anthropometric and patient-reported outcomes after open or endoscopic repair of sagittal craniosynostosis.

Several studies have compared perioperative parameters and early postoperative morphology between endoscope-assisted strip craniectomy with orthotic therapy (endoscopic repair) and cranial vault remodeling (open repair). To extend these results, the authors evaluated school-age anthropometric outcomes after these techniques across three institutions. School-aged children (age range 4-18 years) with previously corrected isolated sagittal craniosynostosis were enrolled. Upon inclusion, 3D photographs and patient-reported outcomes were obtained, and the cephalic index and head circumference z-scores were calculated. Analyses of covariance models controlling for baseline differences and a priori covariates were performed. Eighty-one participants (median [range] age 7 [4-15] years) were included. The mean (95% CI) school-age cephalic index was significantly higher in the endoscopic cohort, though within the normal range for both groups (endoscopic 78% [77%-79%] vs open 76% [74%-77%], p = 0.027). The mean change in the cephalic index from preoperation to school age was significantly greater in the endoscopic group (9% [7%-11%] vs open 3% [1%-5%], p < 0.001). Compared to preoperative measurements, mean school-age head circumference z-scores decreased significantly more in the open cohort (-1.6 [-2.2 to -1.0] vs endoscopic -0.3 [-0.8 to -0.2], p = 0.002). Patient-reported levels of stigma were within the normal limits for both groups. Endoscopic and open repair techniques effectively normalize school-age anthropometric outcomes. However, endoscopic repair produces a clinically meaningful and significantly greater improvement in the school-age cephalic index, with maintenance of head growth. These findings demonstrate the importance of early referral by pediatricians and inform treatment decisions.

Racial differences in the care of pediatric sagittal craniosynostosis: a single-institution cohort study affecting state Medicaid policy.

Although research has shown the cost-effectiveness of endoscopic versus open repair of sagittal synostosis, few studies have shown how race, insurance status, and area deprivation impact care for these patients. The authors analyzed data from children evaluated for sagittal synostosis at a single institution to assess how socioeconomic factors, race, and insurance status affect the surgical treatment of this population. They hypothesized that race and indicators of disadvantage negatively impact workup and surgical timing for craniosynostosis surgery. Medical records of patients treated for sagittal synostosis between 2010 and 2019 were reviewed. Area deprivation index (ADI) and rural-urban commuting area codes, as well as median income by zip code, were used to measure neighborhood disadvantage. Black and White patients were compared as well as patients using Medicaid versus private insurance. Fifty patients were prospectively included in the study. Thirty-one underwent open repair; 19 had endoscopic repair. All 8 (100%) Black patients had open repair, compared to 54.8% of White patients (p = 0.018). Black patients were more likely to use Medicaid compared to White patients (75.0% vs 28.6%, p = 0.019). White patients were younger at surgery (5.5 vs 10.0 months, p = 0.001), and Black patients had longer surgeries (147.5 minutes vs 110.0 minutes, p = 0.021). The median household income by zip code was similar for the two groups. Black patients were generally from areas of greater disadvantage compared to White patients, based on both state and national ADI scores (state: 7.5 vs 4.0, p = 0.013; national: 83.5 vs 60.0, p = 0.013). All (94.7%) but 1 patient undergoing endoscopic repair used private insurance compared to 14 (45.2%) patients in the open repair group (p = 0.001). Patients using Medicaid were from areas of greater disadvantage compared to those using private insurance by both state and national ADI scores (state: 6.0 vs 3.0, p = 0.001; national: 75.0 vs 52.0, p = 0.001). Because Medicaid in the geographic region of this study did not cover helmeting after endoscopic repair of sagittal synostosis, these patients usually had open repair, resulting in significant racial and socioeconomic disparities in treatment of sagittal synostosis. This research has led to a change in Alabama Medicaid policy to now cover the cost of postoperative helmeting.

Creation of the Scaphocephalic Index: Measurement of Global and Regional Severity in Scaphocephaly.

The recently described frontal bossing index (FBI) and occipital bullet index (OBI) allow for quantification of scaphocephaly. A similar index examining biparietal narrowing has not been described. Addition of such an index measuring width would allow for direct evaluation of the primary growth restriction in sagittal craniosynostosis and the formation of an optimized global width/length measure. Computed tomography scans and three-dimensional photographs were used to recreate scalp surface anatomy. Equidistant axial, sagittal, and coronal planes were overlaid, creating a Cartesian grid. Points of intersection were analyzed for population trends in biparietal width. Using the most descriptive point coupled with the sellion protrusion to control for head size, the vertex narrowing index is formed. By combining this index with the FBI and OBI, the scaphocephalic index (SCI) is created as a tailored width/length measure. Using 221 controls and 360 individuals with sagittal craniosynostosis, the greatest difference occurred superiorly and posteriorly at a point 70% of the head's height and 60% of the head's length. This point had an area under the curve of 0.97 and sensitivity and specificity of 91.2% and 92.2%, respectively. The SCI has an area under the curve of 0.9997, sensitivity and specificity greater than 99%, and interrater reliability of 0.995. The correlation coefficient between computed tomography imaging and three-dimensional photography was 0.96. The vertex narrowing index, FBI, and OBI evaluate regional severity, while the SCI is able to describe global morphology in patients with sagittal craniosynostosis. These measures allow for superior diagnosis, surgical planning, and outcome assessment, independent of radiation.

Intracranial Pressure Patterns in Children with Sagittal Craniosynostosis.

Elevated intracranial pressure (ICP) in sagittal craniosynostosis has a wide spectrum of reported incidence, and patterns are not well understood across infancy and childhood. Characterizing the natural history of ICP in this population may clarify risks for neurocognitive delay and inform treatment decisions. Infants and children with sagittal craniosynostosis and unaffected control subjects were prospectively evaluated with spectral-domain optical coherence tomography from 2014 to 2021. Elevated ICP was determined based on previously validated algorithms using retinal optical coherence tomography parameters. Seventy-two patients with isolated sagittal craniosynostosis and 25 control subjects were evaluated. Overall, 31.9% ( n = 23) of patients with sagittal craniosynostosis had evidence of ICP greater than or equal to 15 mmHg, and 27.8% ( n = 20) of patients had ICP greater than or equal to 20 mmHg. Children with sagittal craniosynostosis younger than 6 months were more likely to have normal ICP (88.6% <15 mmHg; 91.4% <20 mmHg) than those aged between 6 and 12 months (54.5%, P = 0.013; 54.5%, P = 0.005) than those older than 12 months (46.2%, P < 0.001; 53.8%, P = 0.001). ICP was directly correlated with severity of scaphocephaly ( P = 0.009). No unaffected control subjects at any age exhibited retinal thickening suggestive of elevated ICP. Elevated ICP is rare in isolated sagittal craniosynostosis younger than 6 months, but it becomes significantly more common after 6 months of age, and may correlate with severity of scaphocephaly. Risk, II.

Developmental Outcomes for Children With Unoperated Metopic or Sagittal Craniosynostosis: Findings at 3 and 7 Years From the Craniofacial Collaboration UK.

The Craniofacial Collaboration UK (CC-UK) is a shared initiative with the aim of addressing key limitations in the existing literature and examining the development of homogenous samples of children with craniosynostosis. This article preliminarily describes developmental, behavioral, and cognitive outcomes for children with either metopic synostosis (MS) or sagittal synostosis (SS), who were unoperated and managed conservatively under the CC-UK protocol. A total of 112 patients were included, and assessments conducted at 3 and/or 7 years of age are presented. The majority of unoperated patients were assessed as having mild clinical severity. Findings are broadly consistent with previous work, indicating that the majority of unoperated patients perform within the average ranges across assessments. For unoperated MS patients, higher than expected rates of developmental concerns were seen at 3 years, particularly relating to gross and fine motor skills, and personal social skills. Slightly elevated rates of behavioral concerns relating to hyperactivity and prosocial behavior were also consistently shown. Few developmental issues were found for SS patients at 3 years. Some minor concerns with peer relationships and prosocial behavior at 3 years, and emotional problems at 7 years were shown, but these were inconsistent over time. Cognitive ability in both groups at 7 years seems to be close to average. Overall findings are positive, and future work should build on these findings by recruiting larger samples and examining longer-term outcomes in adolescence and adulthood, to better understand the developmental trajectory of patients with unoperated craniosynostosis.

Incorporation of a biparietal narrowing metric to improve the ability of machine learning models to detect sagittal craniosynostosis with 2D photographs.

Sagittal craniosynostosis is the most common form of craniosynostosis and typically results in scaphocephaly, which is characterized by biparietal narrowing, compensatory frontal bossing, and an occipital prominence. The cephalic index (CI) is a simple metric for quantifying the degree of cranial narrowing and is often used to diagnose sagittal craniosynostosis. However, patients with variant forms of sagittal craniosynostosis may present with a "normal" CI, depending on the part of the suture that is closed. As machine learning (ML) algorithms are developed to assist in the diagnosis of cranial deformities, metrics that reflect the other phenotypic features of sagittal craniosynostosis are needed. In this study the authors sought to describe the posterior arc angle (PAA), a measurement of biparietal narrowing that is obtained with 2D photographs, and elucidate the role of PAA as an adjuvant to the CI in characterizing scaphocephaly and the potential relevance of PAA in new ML model development. The authors retrospectively reviewed 1013 craniofacial patients treated during the period from 2006 to 2021. Orthogonal top-down photographs were used to calculate the CI and PAA. Distribution densities, receiver operating characteristic (ROC) curves, and chi-square analyses were used to describe the relative predictive utility of each method for sagittal craniosynostosis. In total, 1001 patients underwent paired CI and PAA measurements and a clinical head shape diagnosis (sagittal craniosynostosis, n = 122; other cranial deformity, n = 565; normocephalic, n = 314). The area under the ROC curve (AUC) for the CI was 98.5% (95% confidence interval 97.8%-99.2%, p < 0.001), with an optimum specificity of 92.6% and sensitivity of 93.4%. The PAA had an AUC of 97.4% (95% confidence interval 96.0%-98.8%, p < 0.001) with an optimum specificity of 94.9% and sensitivity of 90.2%. In 6 of 122 (4.9%) cases of sagittal craniosynostosis, the PAA was abnormal while the CI was normal. This means that adding a PAA cutoff branch to a partition model increases the detection of sagittal craniosynostosis. Both CI and PAA are excellent discriminators for sagittal craniosynostosis. Using an accuracy-optimized partition model, the addition of the PAA to the CI increased model sensitivity compared to using the CI alone. Using a model that incorporates both CI and PAA could assist in the early identification and treatment of sagittal craniosynostosis via automated and semiautomated algorithms that utilize tree-based ML models.

Multicenter comparison of head shape outcomes for three minimally invasive strip craniectomy techniques for sagittal craniosynostosis.

A literature gap exists comparing whole head shape outcome following correction of sagittal craniosynostosis. The objective of this multicenter study was to provide an analysis of long-term results following three different endoscopic strip craniectomy techniques for correction of sagittal craniosynostosis: 1) spring-assisted strip craniectomy, 2) wide-strip craniectomy with biparietal and bitemporal barrel-stave wedge osteotomies plus helmet orthosis, and 3) narrow-strip craniectomy plus orthosis without barrel staves. Pre- and postoperative 3D stereophotogrammetric images were collected from patients who underwent craniosynostosis surgery. Procedures were divided among institutions as follows: spring-assisted strip craniectomies were performed at Atrium Health Wake Forest Baptist Hospital; narrow-strip craniectomies were performed at St. Louis Children's Hospital by one craniofacial surgeon; and wide-vertex craniectomies were performed at St. Louis Children's Hospital prior to 2010, and then continued at Children's Medical Center Dallas. Pre- and postoperative 3D whole-head composite images were generated for each procedure to visually represent outcomes at final follow-up and compared with age-matched normal controls. Patients in the spring-assisted strip craniectomy group showed normalization of frontal bossing and skull height compared with age-matched controls, whereas patients undergoing wide-strip craniectomy showed greater correction of occipital protrusion. Patients in the narrow-strip craniectomy cohort had intermediate results between these outcomes. Nested aggregate head shapes showed good correction of head shapes from all techniques. This large, retrospective, multicenter study illustrated whole head shape outcomes from three different craniectomy procedures. Although each procedure showed some differences in loci of primary correction, all three surgical methods demonstrated good correction of primary scaphocephalic deformity.

Characterization of Regional Morphological Changes in Sagittal Craniosynostosis Following Endoscopic Strip Craniectomy With Post-operative Helmeting: Predictors for Success.

Sagittal craniosynostosis (SC) restricts craniofacial growth perpendicular to the sagittal plane resulting in scaphocephaly. The cranium grows in the anterior-posterior dimension causing disproportionate changes, which can be corrected with either cranial vault reconstruction (CVR) or endoscopic strip craniectomy (ESC) combined with post-operative helmet therapy. ESC is performed at an earlier age, and studies demonstrate benefits in risk profile and morbidity compared to CVR, with comparable results if the post-operative banding protocol is strictly upheld. We aim to identify predictors of successful outcome and, using three-dimensional (3D) imaging, assess cranial changes following ESC with post-banding therapy. A single institution retrospective review was performed from 2015-19 for patients with SC who underwent ESC. Patients received immediate post-operative 3D photogrammetry for helmet therapy planning and implementation as well as post-therapy 3D imaging. Using these 3D images, the cephalic index (CI) for study patients was calculated before and after helmet therapy. In addition, Deformetrica™ was used to measure volume and shape changes of pre-defined anatomic skull regions (frontal, parietal, temporal, & occipital) based on the pre- and post-therapy 3D imaging results. Fourteen institutional raters evaluated the pre- and post-therapy 3D imaging in order to determine the success of the helmeting therapy. Twenty-one SC patients met our inclusion criteria. Using 3D photogrammetry, 14 raters at our institution rated 16 of the 21 patients to have had successful helmet therapy. There was a significant difference in CI following helmet therapy with both groups, but there was no significant difference in CI between the "successful" and "unsuccessful" groups. Furthermore, the comparative analysis demonstrated that the parietal region had a significantly higher change in mean RMS distance when compared to the frontal or occipital regions. For patients with SC, 3D photogrammetry may be able to objectively recognize nuanced findings not readily detectable when using CI alone. The greatest changes in volume were observed in the parietal region, which falls in line with treatment goals for SC. Patients deemed to have unsuccessful outcomes were found to be older at time of surgery and initiation of helmet therapy. This suggests that early diagnosis and management for SC may increase the likelihood of success.

Metopic and Sagittal Craniosynostosis in Williams Syndrome.

Craniosynostosis has been previously reported in patients with Williams syndrome. Due to the associated significant cardiovascular anomalies, with an attendant increased risk of death under anaesthesia, most patients have been managed conservatively. Here we report the multidisciplinary approach in a 12-month-old female infant with Williams syndrome who has metopic and sagittal craniosynostosis. The child successfully underwent calvarial remodelling procedures, with the clinical outcome demonstrating dramatically improved global development after surgery.

Use of Subcutaneous Injection of Epinephrine and Triamcinolone with Tranexamic Acid Reduces Blood Loss, Transfusion Rates, and Length of Stay in Open Sagittal Craniosynostosis Repair.

In 2017, we adopted the use of triamcinolone/epinephrine (TAC/Epi) scalp injection and later added tranexamic acid (TXA) in open sagittal synostosis surgery. We believe that this reduced blood loss and transfusion rates. A total of 107 consecutive patients operated for sagittal synostosis aged <4 months from 2007 to 2019 were retrospectively reviewed. We collected demographics [age, sex, weight at surgery, and length of stay (LOS)], intraoperative information [estimated blood loss (EBL)], administration of packed red blood cell, transfusion of plasmalyte/albumen, operating time, baseline hemoglobin (Hb) and hematocrit (Hct), type of local anesthetic (1/4% bupivacaine vs. TAC/Epi), and use/volume of TXA. Hb, Hct, coagulation studies, and platelets at 2 hours postoperatively and postoperative day (POD) 1 were recorded. There were 3 groups: 1/4% bupivacaine/epinephrine (N=64), TAC/Epi (N=13), and TAC/Epi with TXA bolus/infusion intraoperatively (N=30). Groups receiving TAC/Epi or TAC/Epi with TXA had lower mean EBL ( P <0.0001), lower rate/amount of packed red blood cell transfusion ( P <0.0001), lower prothrombin time/international normalized ratio on POD 1 ( P <0.0001), higher platelets ( P <0.001), and shorter operative time ( P <0.0001). LOS was shortest for TAC/Epi with TXA ( P <0.0001). No significant differences between groups were noted on POD 1 Hb, Hct, or partial prothrombin time. Post hoc testing revealed an advantage of TAC/Epi with TXA over TAC/Epi alone for 2-hour postoperative international normalized ratio ( P =0.0249), Operating Room time ( P =0.0179), and LOS ( P =0.0049). Use of TAC/Epi alone reduced EBL, LOS, Operating Room time, and improved laboratory values postoperatively in open sagittal synostosis surgery. Addition of TXA further improved operative time and LOS. It is likely that lower rates of transfusion could be tolerated.