Using the SCAR-Q to Evaluate Morbidity of Scars in Craniosynostosis Repair.

OBJECTIVE: While previous literature has investigated the psychosocial impact and aesthetic satisfaction associated with post-operative scarring for certain pediatric craniofacial conditions, the impact of the scar burden resulting from craniosynostosis surgery has not been adequately studied. PARTICIPANTS: SCAR-Q was shared with patients ages 8 and older. Thirty-two complete patient responses were recorded. INTERVENTIONS: SCAR-Q is a PROM that consists of three independent scales - appearance, symptoms, and psychosocial impact - associated with a scar. MAIN OUTCOME MEASURES: Mann-Whitney U, linear regression, and Pearson correlation tests were used to evaluate associations between the scales, in addition to patient characteristics such as sex and suture involvement. RESULTS: Mean ages at time of surgery and survey completion were 9.65 ± 10.10 months and 12.10 ± 3.92 years, respectively. Mean scale scores were 81.5 ± 17.9 for appearance, 86.8 ± 12.4 for symptoms, and 79.3 ± 25.7 for psychosocial impact. Higher patient dissatisfaction with scar appearance correlated with more scar-related symptoms (r = 0.389; p = 0.028) and a greater psychosocial impact (r = 0.725; p < 0.001). SCAR-Q scales did not significantly correlate with age at surgery, age at survey completion, type of synostosis, or type of surgery; however, female patients reported lower mean appearance (65.4 vs. 86.0; p = 0.012) and psychosocial impact (57.3 vs. 85.5; p = 0.010) scores when compared to their male counterparts. CONCLUSIONS: It is vital that surgeons discuss patients' aesthetic satisfaction following craniosynostosis surgery in order to appropriately address and limit deleterious, long-term physical and psychosocial outcomes.

Evolution of Virtual Surgical Planning Use Among Craniofacial Surgeons.

Virtual surgical planning (VSP) has benefits in craniofacial surgery with growing popularity. However, while specific use cases are highlighted in the literature, no studies exist providing an overview of VSP use among craniofacial surgeons, and little is known about the extent of exposure to VSP during plastic surgery training. This study surveyed members of The American Society of Maxillofacial Surgeons (ASMS) to better characterize both the landscape of VSP use among practicing craniofacial surgeons and the extent of exposure to VSP throughout surgical training. An electronic survey was administered in the fall of 2023. Response data included surgeon demographics, VSP usage, including the use in residency/fellowship, procedures for which VSP is used, and assessment of VSP's impact on the surgeon's practice. Demographics and VSP use were analyzed using descriptive statistics, while categorical and continuous variables were analyzed using χ2 tests and t-tests, respectively. Of the 44 respondents, 40 (90.9%) completed a craniofacial surgery fellowship, and 18 (40.9%) utilized VSP in either residency or fellowship. In respondents' current practice, VSP is utilized most commonly for orthognathic surgery (n=32, 91.4%), postablative reconstruction (n=23, 82.1%), and facial feminization (n=11, 73.3%). Shorter operative time and improved esthetic outcomes were frequently reported as benefits derived from VSP use. Finally, surgeons in practice for less than 10 years were significantly more likely to have used VSP in both residency (OR=20.3, P<0.01) and in fellowship (OR=40.6, P<0.01) than those practicing for more than 10 years. These findings suggest that craniofacial surgeons apply VSP more commonly for certain procedure types. Our results additionally suggest that incorporation of VSP into residency and fellowship training has become significantly more common over time, with a pivot towards integration in the last decade.

The role of genetics on behavioral outcomes in nonsyndromic sagittal synostosis.

OBJECTIVE: Previous work identified an association between genetics and neurodevelopmental delays in patients with nonsyndromic craniosynostosis. The authors investigated the role of genetic mutations on behavioral outcomes of patients with treated sagittal synostosis. METHODS: Parents of children aged 6-18 years with surgically corrected sagittal synostosis were recruited to complete the Child Behavioral Checklist (overall behavioral problems), Conners 3rd Edition-Parent (attention-deficit/hyperactivity disorder), Social Responsiveness Scale 2nd Edition (autism spectrum disorder [ASD]), and Behavior Rating Inventory of Executive Function 2nd Edition (executive function). Genomic analysis was completed, and patients were identified if they had mutations in high probability of loss of function intolerant (pLI) genes (high pLI vs nonhigh pLI). Genetic burden was assessed relative to controls. Multivariate linear regression determined the association of mutations in high pLI genes with behavioral scores, while controlling for sociodemographic factors, age at surgery, surgery type, and IQ. RESULTS: Sixteen of 45 patients were in the high pLI group. There were no differences between the groups in terms of sociodemographic factors. A greater proportion of children in the high pLI group scored at or above borderline clinical levels for aggression (18.8% vs 0.0%, p = 0.05) and externalizing problems (31.3% vs 3.7%, p = 0.02). Among children in the nonhigh pLI group, older age at surgery was associated with worse scores on the rule-breaking, aggression, and externalizing problems domains and four out of five ASD domains. CONCLUSIONS: Children with treated nonsyndromic sagittal synostosis and mutations in high pLI genes had worse behavioral problems in externalizing behaviors and aggression, whereas older age at surgery was a significant predictor of worse behavioral outcomes in patients without mutations in high pLI genes.

AXIN1 mutations in nonsyndromic craniosynostosis.

OBJECTIVE: Occurring once in every 2000 live births, craniosynostosis (CS) is the most frequent cranial birth defect. Although the genetic etiologies of syndromic CS cases are well defined, the genetic cause of most nonsyndromic cases remains unknown. METHODS: The authors analyzed exome or RNA sequencing data from 876 children with nonsyndromic CS, including 291 case-parent trios and 585 additional probands. The authors also utilized the GeneMatcher platform and the Gabriella Miller Kids First genome sequencing project to identify additional CS patients with AXIN1 mutations. RESULTS: The authors describe 11 patients with nonsyndromic CS harboring rare, damaging mutations in AXIN1, an inhibitor of Wnt signaling. AXIN1 regulates signaling upstream of key mediators of osteoblast differentiation. Three of the 6 mutations identified in trios occurred de novo in the proband, while 3 were transmitted from unaffected parents. Patients with nonsyndromic CS were highly enriched for mutations in AXIN1 compared to both expectation (p = 0.0008) and exome sequencing data from > 76,000 healthy controls (p = 2.3 × 10-6), surpassing the thresholds for genome-wide significance. CONCLUSIONS: These findings describe the first phenotype associated with mutations in AXIN1, with mutations identified in approximately 1% of nonsyndromic CS cases. The results strengthen the existing link between Wnt signaling and maintenance of cranial suture patency and have implications for genetic testing in families with CS.

Comparison of emotional and behavioral regulation between metopic and sagittal synostosis.

PURPOSE: Children with surgically corrected nonsyndromic craniosynostosis have been previously found to have neurocognitive and behavioral difficulties. Children with metopic synostosis have been described to have more difficulties than children with sagittal synostosis. This study aims to characterize the behavioral differences between children with metopic and sagittal synostosis. METHODS: Children with metopic and sagittal synostosis were recruited at school age. Parents completed four separated behavioral assessments: Conners-3 (evaluation of ADHD), Social Responsiveness Scale-2 (SRS-2: evaluation of autism), Behavior Rating Inventory of Executive Function-2 (BRIEF-2: evaluation of executive function), and Child Behavior Checklist (CBCL: evaluation of overall behavioral problems). Children underwent intelligence quotient (IQ) testing using the Wechsler Abbreviated Scale of Intelligence (WASI-II). RESULTS: There were 91 children (45 with metopic and 46 with sagittal synostosis). More children with metopic synostosis reported requiring supportive services (57.7% vs 34.7%, p = 0.02) and more reached or exceeded borderline clinical levels of two executive function subscales of the BRIEF-2 (emotion regulation index: 33.3% vs 17.4%, p = 0.05; global executive composite: 33.3% vs 17.4%, p = 0.05). Children with sagittal synostosis had higher scores on the rule-breaking and externalizing problem subscales of the CBCL. Increasing age at surgery was associated with worse executive function scores. CONCLUSIONS: A relationship between suture subtype and behavioral outcomes exists at school age. More children with metopic synostosis required social services indicating more overall difficulties. Children with metopic synostosis have more specific problems with executive function, while children with sagittal synostosis had more difficulties with externalizing behaviors.

Sports participation after craniosynostosis repair: the critical role of post-operative guidance in parental decision-making.

PURPOSE: Despite previous research supporting patient safety in sports after craniosynostosis surgery, parental anxiety remains high. This study sought to evaluate the role of healthcare providers in guiding patients and families through the decision-making process. METHODS: Parents of children with repaired craniosynostosis were asked to assess sports involvement and parental decision-making in children ages 6 and older. Questions were framed primarily on 5-point Likert scales. Sport categorizations were made in accordance with the American Academy of Pediatrics. Chi-squared, linear regression, and Pearson correlation tests were used to analyze associations between the questions. RESULTS: Forty-three complete parental responses were recorded. Mean ages at surgery and time of sports entry were 7.93 ± 4.73 months and 4.76 ± 2.14 years, respectively. Eighty-two percent of patients participated in a contact sport. Discussions with the primary surgeon were more impactful on parental decisions about sports participation than those with other healthcare providers (4.04 ± 1.20 vs. 2.69 ± 1.32). Furthermore, children whose parents consulted with the primary surgeon began participating in sports at a younger age (4.0 ± 1.0 vs. 5.8 ± 2.7 years, p = 0.034). The mean comfort level with contact sports (2.8 ± 1.4) was lower than that with limited-contact (3.8 ± 1.1, p = 0.0001) or non-contact (4.4 ± 1.3, p < 0.0001) sports. CONCLUSION: This study underscores the critical role that healthcare professionals, primarily surgeons, have in guiding families through the decision-making process regarding their children's participation in contact sports.

A diffusion tensor imaging comparison of white matter development in nonsyndromic craniosynostosis to neurotypical infants.

PURPOSE: Nonsyndromic craniosynostosis (NSC) is associated with neurocognitive deficits, and intervention at infancy is standard of care to limit the negative effects of NSC on brain development. In this study, diffusion tensor imaging (DTI) was implemented to investigate white matter microstructure in infants with NSC undergoing cranial vault remodeling, and a comparison was made with white matter development in neurotypical controls. METHODS: Infants presenting with NSC (n = 12) underwent DTI scans before and after cranial vault remodeling. Neurotypical infants (n = 5), age matched to NSC patients at preoperative scans, were compared to preoperative DTI scans. Pre- and postoperative NSC scans were compared in aggregate, and the sagittal synostosis (n = 8) patients were evaluated separately. Finally, neurotypical infants from the University of North Carolina/University of New Mexico Baby Connectome Project (BCP), who underwent DTI scans at timepoints matching the NSC pre- and postoperative DTI scans, were analyzed (n = 9). Trends over the same time period were compared between NSC and BCP scans. RESULTS: No significant differences were found between preoperative NSC scans and controls. White matter development was more limited in NSC patients than in BCP patients, with microstructural parameters of the corpus body and genu and inferior and superior longitudinal fasciculi consistently lagging behind developmental changes observed in healthy patients. CONCLUSION: Infant white matter development appears more limited in NSC patients undergoing cranial vault remodeling relative to that in neurotypical controls. Further investigation is needed to explore these differences and the specific effects of early surgical intervention.

Quantifying Facial Feminization Surgery's Impact: Focus on Patient Facial Satisfaction.

Background: Facial feminization surgery (FFS) has been associated with improving gender dysphoria in transgender patients. This study aimed to quantify the impact of surgery on patient facial satisfaction, using the FACE-Q and a quality-of-life (QoL) survey. Methods: Transgender female patients were recruited to complete the FACE-Q and the World Health Organization's QoL Scale-Short Form (WHOQOL-BREF) if they were planning to or had undergone FFS at our institution. FACE-Q modules completed included "Satisfaction with Facial Appearance Overall," individual facial attributes (forehead/eyebrows, nose, cheeks, cheekbone, chin, jawline, and neck), and the WHOQOL-BREF, which assesses patient QoL through four domains (physical, psychological, social relations, and environment). Both matched and unmatched analyses of preoperative versus postoperative cohorts were performed. Results: Overall, 48 patients participated in our study and completed 31 FACE-Q surveys preoperatively and 37 postoperatively. On average, patients were 37.2 ± 12.5 years old. FACE-Q scores increased significantly for all facial attributes and for Satisfaction with Facial Appearance Overall between cohorts (P < 0.05). The facial attribute with the greatest increase in satisfaction was the jawline, followed by the nose. The WHOQOL-BREF's psychological and physical domains both improved significantly (P < 0.05). Wait time for surgery of less than 6 months (b = 22.42, P = 0.02) was associated with higher overall facial satisfaction, whereas age at surgery (b = -1.04, P < 0.01) was associated with lower overall facial satisfaction. Conclusions: Transgender female patients experienced significant improvements in facial satisfaction and QoL after FFS. Undergoing surgery at a younger age and shorter wait times for surgery were associated with increased overall facial satisfaction.

Technique for Cephalometric Analysis of the Craniofacial Skeleton Following Facial Feminization Surgery.

Facial feminization surgery (FFS) is characterized by a series of relatively diverse procedures aimed at aligning skeletal and soft tissue facial appearance with one's experienced feminine gender. Although there are several well-described outcomes from surgical techniques, there is no standardized methodology to provide reliable analyses of postoperative FFS outcomes. This paper describes the first reliable and reproducible technique to accurately and consistently measure post-FFS changes to guide surgical planning to optimize patient outcomes.

"Morphologic Severity and Age at Surgery Are Associated with School Age Neurocognitive Outcomes in Metopic Craniosynostosis".

BACKGROUND: Radiographic severity of metopic synostosis has been suggested as a predictor of long-term neurocognitive outcomes, and artificial intelligence (AI) has recently been used to quantify severity. Age at surgery is predictive of long-term neurocognition in sagittal synostosis but has not been adequately explored in metopic synostosis. METHODS: Children ages 6 to 18 years old with corrected metopic synostosis underwent testing of intelligence quotient (IQ), academic achievement, and visuomotor integration (VMI). Various manual measurements and AI-derived severity scores were determined. Scans were categorized as moderate or severe for head-to-head comparisons and multivariable linear regressions were used to assess the relationship of age at surgery and severity with neurocognitive outcomes. RESULTS: 41 patients with average age at testing of 10.8 ± 3.4 years were included. 18 patients were in the severe group while 23 patients were in the moderate group with average ages at surgery 6.6 ± 2.7 and 10.6 ± 8.4 months, respectively (p = 0.062). Greater AI-derived severity was significantly associated with lower reading comprehension (p = 0.040 and 0.018) and reading composite scores (p = 0.024 and p = 0.008). Older age at surgery was significantly associated with lower VMI scores (p-values ranging from 0.017 to 0.045) and reading composite scores (p = 0.047 and 0.019). CONCLUSIONS: This study suggests an association between greater AI-derived radiographic severity and lower reading ability in corrected metopic synostosis. Older age at surgery was independently associated with lower reading ability and visuomotor integration. Surgical correction may mitigate neurodevelopmental differences based on severity that have been observed pre-operatively.

Quality and Readability of Accessible Facial Feminization Literature: Where Can We Improve?

BACKGROUND: Facial feminization surgery (FFS) consists of multiple, complex procedures. Well-informed patients have been shown to have better outcomes and expectations. However, there is limited data evaluating FFS patient-oriented material online. This study aims to evaluate the quality and readability of FFS literature online. METHODS: Facial feminization surgery-related terms were queried in Google with location, cookies, and user account information disabled. Websites were analyzed for readability using Flesch Reading Ease and Flesch-Kincaid Reading Grade Level, suitability using the suitability assessment of materials (SAM), and quality using the DISCERN scale. Unpaired t tests and χ2 tests were used to compare the websites of community-based and academic or public institutions. RESULTS: One hundred twenty websites met inclusion criteria (71 community-based and 49 academic). The average reading grade level was 11.68 ± 1.71. The average SAM score was 53.11 ± 11.75, denoting adequate readability. Only 16.67% of websites contained visual aids to assist in explaining procedures and benefits. Overall, 68.33% of websites' DISCERN score was rated very poor or poor, whereas only 8.33% were rated as good. The following DISCERN elements had the lowest scores across all graded websites: "clear source and date of information," "details of additional source of support," "refers to areas of uncertainty," and "describes risks of each treatment." Websites published by community-based institutions were significantly better in describing how FFS works and the benefits of each procedure. Academic sites overall were less biased (4.84 vs 4.62, P = 0.03) and provided additional sources of support (2.35 vs 1.32, P = <0.001). CONCLUSIONS: Online FFS patient resources should be written at a more inclusive reading level and should increase the use of pictorial aids to improve patient comprehension. Facial feminization surgery patient resources were significantly above the recommended sixth grade reading level for health literature. Academic and community-based institutions should include more information about procedural risks and limitations in surgical outcomes to ensure a broader scope of understanding.

Tranexamic Acid in Facial Feminization Surgery: Quantifying a High-risk Setting With Exogenous Estrogen Supplementation.

Tranexamic acid (TXA) has been increasingly utilized in orthognathic surgery, aesthetic surgery, and craniofacial surgery. However, the risk of increasing venous thromboembolic events (VTE) must be carefully considered as TXA is a prothrombotic agent. Our study aimed to investigate the safety of TXA in the setting of facial feminization surgery. These patients are at an elevated risk for VTE at baseline given their uniform history of exogenous estrogen supplementation. A retrospective review of all patients that underwent facial feminization surgery at our medical center between December 2015 and September of 2022 was performed. Demographic information, procedure type, Caprini scores, hematoma rate, VTE rate, estimated blood loss, and operative time were all studied. Unpaired t tests were used to compare patients that received TXA and those who did not. In total, there were 79 surgeries performed during our study period. There were 33 surgeries (41.77%) that used TXA intraoperatively. Ten patients (12.65%) received anticoagulation postoperatively, 5 of whom received TXA intraoperatively. Of the 33 patients who received TXA, 30 patients remained on estrogen therapy. There was no statistically significant difference in VTE rates in patients who received TXA (n=33, 41.77%) and those who did not (n=46, 58.23%). Bleeding events, Caprini scores, estimated blood loss, and operative time were also not found to be significantly different between the 2 cohorts. The authors found no significant increase in VTE in facial feminization patients undergoing estrogen supplementation when intraoperative TXA was utilized. This is the first known report investigating the safety of TXA in this higher risk patient population.

De novo variants implicate chromatin modification, transcriptional regulation, and retinoic acid signaling in syndromic craniosynostosis.

Craniosynostosis (CS) is the most common congenital cranial anomaly. Several Mendelian forms of syndromic CS are well described, but a genetic etiology remains elusive in a substantial fraction of probands. Analysis of exome sequence data from 526 proband-parent trios with syndromic CS identified a marked excess (observed 98, expected 33, p = 4.83 × 10-20) of damaging de novo variants (DNVs) in genes highly intolerant to loss-of-function variation (probability of LoF intolerance > 0.9). 30 probands harbored damaging DNVs in 21 genes that were not previously implicated in CS but are involved in chromatin modification and remodeling (4.7-fold enrichment, p = 1.1 × 10-11). 17 genes had multiple damaging DNVs, and 13 genes (CDK13, NFIX, ADNP, KMT5B, SON, ARID1B, CASK, CHD7, MED13L, PSMD12, POLR2A, CHD3, and SETBP1) surpassed thresholds for genome-wide significance. A recurrent gain-of-function DNV in the retinoic acid receptor alpha (RARA; c.865G>A [p.Gly289Arg]) was identified in two probands with similar CS phenotypes. CS risk genes overlap with those identified for autism and other neurodevelopmental disorders, are highly expressed in cranial neural crest cells, and converge in networks that regulate chromatin modification, gene transcription, and osteoblast differentiation. Our results identify several CS loci and have major implications for genetic testing and counseling.

Quantifying the Impact of Genetics on Neurocognition in Nonsyndromic Sagittal Craniosynostosis.

BACKGROUND: Previous work has identified an association between de novo and transmitted loss-of-function mutations in genes under high evolutionary constraint with neurodevelopmental delays in nonsyndromic craniosynostosis (NSC). The authors sought to quantify the neurocognitive effect of these genetic lesions. METHODS: In a prospective, double-blinded cohort study, demographic surveys and neurocognitive tests were administered to patients recruited from a national sample of children with sagittal NSC. Scores for academic achievement, Full-Scale Intelligence Quotient (FSIQ), and visuomotor skills were directly compared between patients with and without damaging mutations in genes with a high probability of loss of function intolerance using two-tailed t tests. Analysis of covariance was also used to compare test scores while controlling for surgery type, age at surgery, and sociodemographic risk. RESULTS: Fifty-six patients completed neurocognitive testing, 18 of whom had a mutation in a highly constrained gene. There was no significant difference between groups in any sociodemographic factors. After controlling for patient factors, patients with high-risk mutations had poorer performance compared with patients without high-risk mutations in every testing category, with significant differences in FSIQ (102.9 ± 11.4 versus 110.1 ± 11.3; P = 0.033) and visuomotor integration (100.0 ± 11.9 versus 105.2 ± 9.5; P = 0.003). There were no significant differences in neurocognitive outcome when stratifying groups based on type of surgery or age at time of surgery. CONCLUSIONS: Even after controlling for exogenous factors, the presence of mutations in high-risk genes led to poorer neurocognitive outcomes. High-risk genotypes may predispose individuals with NSC to deficits, particularly in FSIQ and visuomotor integration. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Long-term neurocognitive outcomes in 204 single-suture craniosynostosis patients.

PURPOSE: Craniosynostosis, which describes premature fusion of one or more cranial sutures, has been associated with a variety of neurocognitive deficits. We sought to explore the cognitive profiles of the various types of single-suture, non-syndromic craniosynostosis (NSC). METHODS: A retrospective review of children 6-18 years old with surgically corrected NSC who underwent neurocognitive testing (Weschler Abbreviated Scale of Intelligence, Beery-Buktenica Developmental Test of Visuomotor Integration) from the years 2014-2022 was conducted. RESULTS: 204 patients completed neurocognitive testing (139 sagittal, 39 metopic, 22 unicoronal, 4 lambdoid suture). 110 (54%) of the cohort was male, and 150 (74%) were White. Mean IQ was 106.10±14.01 and mean age at surgery and testing were 9.0±12.2 months and 10.9±4.0 years, respectively. Sagittal synostosis was associated with higher scores than metopic synostosis, with significant differences in verbal IQ (109.42±15.76 vs 101.37±10.41), full-scale IQ (108.32±14.44 vs 100.05±11.76), visuomotor integration (101.62±13.64 vs 92.44±12.07), visual perception (103.81±12.42 vs 95.87±11.23), and motor coordination (90.45±15.60 vs 84.21±15.44). Sagittal synostosis was associated with significantly higher scores for visuomotor integration (101.62±13.64 vs 94.95±10.24) and visual perception (103.81±12.42 vs 94.82±12.75) than unicoronal synostosis. CONCLUSIONS: Compared to patients with sagittal synostosis, patients with metopic synostosis exhibited lower scores in verbal IQ, full-scale IQ, visuomotor integration, visual perception, and motor control after surgical correction. Despite surgical correction for premature metopic suture fusion, the effect on the adjacent frontal lobe and white matter connections to other regions of the brain may have a lasting functional impact. Patients with unicoronal synostosis exhibited lower visuomotor integration and visual perception scores.

Role of State Insurance Policy in Orthotic Helmet Access for Deformational Plagiocephaly.

BACKGROUND: Helmet therapy for deformational plagiocephaly has an ideal window for treatment, and timely access to care is vital to achieving optimal benefit. The authors evaluated the hypothesis that patients insured through Medicaid experience decreased access to helmet therapy. METHODS: This was a retrospective analysis of referrals for helmet therapy to Cranial Technologies, Inc. between 2014 and 2020 across 21 states. Outcome measures were likelihood of receiving a helmet, receiving a second helmet, receiving delayed treatment, and having delayed presentation. RESULTS: From 2014 to 2020, a total of 219,869 patients were referred and 141,513 of these received a helmet. Patients with Medicaid were less likely to receive treatment (OR, 0.63; P < 0.001) and more likely to present late (OR, 1.55; P < 0.001) or receive delayed treatment (OR, 3.24; P < 0.001) compared with the commercially insured. Patients with Medicaid were less likely to receive helmet therapy in nine states, with the strongest association in Texas (OR, 0.32; P < 0.001), and more likely to receive helmet therapy in five states, with the strongest association in Colorado (OR, 1.89; P < 0.001). Medicaid was associated with late presentation and delayed treatment in all states. CONCLUSIONS: Patients with Medicaid presented later and were less likely to receive helmet therapy. Findings reflected state-specific Medicaid policies, with patients in some states more likely to receive a helmet with Medicaid than with commercial insurance. However, late presentation and delays in treatment with Medicaid were observed across all states. State-specific Medicaid restrictions are likely a driving factor in determining access to helmet therapy.

Neurocognitive Outcomes in Deformational Plagiocephaly: Is There an Association between Morphologic Severity and Results?

BACKGROUND: The neurodevelopmental effects of skull asymmetry and orthotic helmet therapy for deformational plagiocephaly (DP) have had limited investigation. This study assessed the long-term neurocognitive outcomes in patients with DP and their association with orthotic helmet therapy and head shape abnormality. METHODS: A total of 138 school-age children with a history of DP, 108 of whom received helmet therapy, were tested with a neurocognitive battery assessing academic achievement, intelligence quotient, and visual-motor function. Severity of presenting plagiocephaly was calculated using anthropometric and photometric measurements. Analysis of covariance was used to compare outcomes between helmeted and nonhelmeted cohorts, unilateral plagiocephaly and concomitant brachycephaly, and left-sided and right-sided plagiocephaly. The association between severity of plagiocephaly and neurocognitive outcome was assessed through a residualized change approach. RESULTS: There were no significant differences in neurocognitive outcomes between the helmeted and nonhelmeted DP cohorts or the unilateral plagiocephaly and brachycephaly cohorts. Participants with left-sided DP had significantly lower motor coordination scores than participants with right-sided DP (84.8 versus 92.7; effect size = -0.50; P = 0.03). There was a significant laterality by cephalic index interaction, with a negative association between cephalic index and reading comprehension and spelling for participants with left-sided DP. No significant associations were found between severity of presenting or posttreatment deformity and neurocognitive outcome. CONCLUSIONS: Pretreatment and posttreatment severity of plagiocephaly were not correlated with neurocognitive function at school age. Helmet therapy was not associated with better or worse long-term neurocognitive function. However, participants with left-sided DP demonstrated worse neurocognitive outcomes than participants with right-sided DP in the domains of motor coordination and some types of academic achievement. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Gaps in Access to Reconstruction Compared with Audiologic Care among Children with Microtia.

Reconstruction for microtia decreased psychosocial morbidity; timely referral for surgery is crucial. We evaluated specialist referrals for microtia at a major academic medical center. Only one-half of patients were evaluated at any point by a reconstructive craniofacial surgeon. Patients followed early for audiologic concerns may not be receiving timely referred for reconstruction.

Utilizing Computer-Assisted Design and Virtual Surgical Planning for Correction of Unilateral Lambdoid Synostosis.

Unilateral lambdoid synostosis is the rarest form of single-suture craniosynostosis. Although various surgical approaches have been described, cranial vault remodeling remains the predominant approach. To aid in surgical planning, preoperative virtual surgical modeling using a patient's presenting computed tomography scan can be used to increase reconstructive precision and to reduce operative time. Presented is a 7-month-old male with unilateral lambdoid synostosis who underwent medically modeled cranial vault reconstruction.