Cognitive Outcomes of Children With Sagittal Craniosynostosis Treated With Either Endoscopic or Open Calvarial Vault Surgery.

Importance: Several studies have reported a higher incidence of neurodevelopmental delays and cognitive deficits in patients with single-suture craniosynostosis; however, there are few studies examining the associations of repair type with cognitive outcomes. Objective: To measure differences in neuropsychological outcomes between school-age children who were treated for sagittal craniosynostosis and unaffected controls and explore differences in cognitive function among children with sagittal craniosynostosis who were previously treated with either endoscopic strip craniectomy or open calvarial vault surgery. Design, Setting, and Participants: This cohort study was performed between 2018 and 2022. Eligible participants included patients aged 5 to 17 years who had previously been seen as infants or toddlers (<3 years) at 1 of 3 surgical centers for craniosynostosis repair with either endoscopic surgery or open calvarial vault surgery. A separate cohort of unaffected controls were included for comparison. Data analysis was conducted from November 2023 to February 2024. Exposures: Open calvarial vault surgery or endoscopic repair for single-suture craniosynostosis. Main Outcomes and Measures: The primary outcome was the Differential Ability Scales-II (DAS-II) General Conceptual Ability (GCA) score, an index for overall intellectual ability. Secondary outcomes included DAS-II subscale scores (Verbal Ability, Nonverbal Reasoning, Spatial Ability, Working Memory, and Processing Speed), and Patient-Reported Outcomes Measurement Information System (PROMIS) cognitive function scores. Results: A total of 81 patients with sagittal craniosynostosis (59 male [73%]; 22 female [27%]) and 141 controls (81 male [57%]; 60 female [43%]) were included. Of the 81 participants with sagittal craniosynostosis, 46 underwent endoscopic repair and 35 underwent open repair. Median (range) age at time of follow-up assessment was 7.7 (5.0-14.8) years for children with sagittal craniosynostosis and median age at assessment was 8.5 (7.7-10.5) years for controls. After controlling for age at assessment, sex, and socioeconomic status, there was no statistically significant or clinically meaningful difference in GCA scores between children who underwent endoscopic repair (adjusted mean score, 100; 95% CI, 96-104) and open repair (adjusted mean score, 103; 95% CI, 98-108) (P > .99). We found no significant difference in PROMIS scores between repair types (median [range] for endoscopic repair 54 [31-68] vs median [range] for open repair 50 [32-63]; P = .14). When comparing the treatment groups with the unaffected controls, differences in subscale scores for GCA and working memory were observed but were within normal range. Conclusions and Relevance: In this cohort study, there were no statistically or clinically significant differences in cognitive outcomes among school-age children by and type of surgical procedure used to repair nonsyndromic sagittal craniosynostosis. These findings suggest primary care clinicians should be educated about different options for craniosynostosis surgery to ensure early referral of these patients so that all treatment options remain viable.

Helmet Therapy for the Management of Deformational Plagiocephaly in Pediatric Patients with Shunted Hydrocephalus.

OBJECTIVE: To evaluate the safety and efficacy of helmet therapy for deformational plagiocephaly in patients with shunted hydrocephalus. DESIGN: Retrospective chart review. SETTING: Institutional, tertiary-care hospital. PATIENTS: All patients at St. Louis Children's Hospital between 2014 and 2021 with shunted hydrocephalus who underwent helmet therapy for deformational plagiocephaly. INTERVENTIONS: Helmet therapy. MAIN OUTCOME MEASURES: Cranial vault asymmetry (CVA), cranial vault asymmetry index (CVAI), and cephalic index (CI) were measured before and after completion of helmet therapy. RESULTS: There were 37 patients with shunted hydrocephalus and documented deformational plagiocephaly. Twelve were managed with helmet therapy. Average age at helmeting initiation and time between shunt placement and helmeting initiation was 5.8 and 4.6 months, respectively. Average CVA, CVAI, and CI at helmeting initiation and termination was 11.6, 7.98, and 85.2, and 6.95, 4.49, and 83.7, respectively. Average duration of helmeting was 3.7 months. CVA and CVAI were significantly lower after helmeting (P = .0028 and .0021) and 11/12 patients had overall improvement in plagiocephaly. CONCLUSIONS: Helmet therapy appears to be a safe and efficacious management strategy for deformational plagiocephaly in patients with shunted hydrocephalus. Despite the occasional need for additional fittings and surveillance beyond the normal schedule, in all cases appropriately fitting helmets were achieved and no major adverse events occurred. This cohort represents a proof of principle for the safety and efficacy of helmet therapy in patients with shunted hydrocephalus. Further work in larger prospective cohorts is needed to confirm these initial findings.

Impact of Neighborhood Deprivation and Social Vulnerability on Outcomes and Interventions in Patients with Cleft Palate.

OBJECTIVE: To examine whether neighborhood disadvantage impacts length of follow-up, interventions, and outcomes for patients with cleft palate. DESIGN: Retrospective cohort. SETTING: Cleft Palate Craniofacial Institute Database at St. Louis Children's Hospital. PATIENTS/PARTICIPANTS: Patients with cleft palate following in St. Louis Children's Hospital Cleft Palate Multidisciplinary Team Clinic. INTERVENTIONS: Primary palatoplasty between 2012-2017. Patients were divided into quartiles across area deprivation index (ADI) and social vulnerability index (SVI), two validated, composite metrics of neighborhood disadvantage, to examine whether living in neighborhoods from different deprivation quartiles impacts outcomes of interest. MAIN OUTCOME MEASURE: Follow-up through age 5, surgeries and surgical complications, speech, developmental, and behavioral outcomes. RESULTS: 205 patients were included. 39% of patients belonged to the most deprived ADI quartile, while 15% belonged to the most vulnerable SVI quartile. There were no differences between ADI or SVI quartiles in number of operations received (p ≥ 0.40). Patients in the most deprived ADI quartile were significantly more likely to have speech/language concerns (OR 2.32, 95% CI [1.20-4.89], p = 0.01). Being in a more vulnerable SVI quartile was associated with developmental delay (OR 2.29, 95% CI [1.04-5.15], p = 0.04). ADI and SVI quartile did not impact risk of loss to follow-up in the isolated and combined cleft lip and palate subgroups (p ≥ 0.21). CONCLUSIONS: Neighborhood disadvantage impacts speech and developmental outcomes in patients with cleft palate despite comparable length of follow-up in multidisciplinary team clinic.

Shared Decision-Making: Process for Design and Implementation of a Decision Aid for Patients With Craniosynostosis.

To describe the process of developing a craniosynostosis decision aid.We conducted a mixed-methods exploratory study between August 2019 and March 2020 to develop a decision aid about surgical treatment for single suture craniosynostosis.A single tertiary care academic children's hospital.The decision aid development team consisted of surgeons, research fellows, a clinical nurse practitioner, clinical researchers with expertise in decision science, and a university-affiliated design school. Qualitative interviews (N = 5) were performed with families, clinicians (N = 2), and a helmeting orthotist to provide feedback on decision aid content, format, and usability.After cycles of revisions and iterations, 3 related decision aids were designed and approved by the marketing arm of our institution. Distinct booklets were created to enable focused discussion of treatment options for the 3 major types of single suture craniosynostosis (sagittal, metopic, unicoronal).Three decision aids representing the 3 most common forms of single suture craniosynostosis were developed. Clinicians found the decision aids could help facilitate discussions about families' treatment preferences, goals, and concerns.We developed a customizable decision aid for single suture craniosynostosis treatment options. This tool lays the foundation for shared decision-making by assessing family preferences and providing clear, concise, and credible information regarding surgical treatment. Future research can evaluate this tool's impact on patient-clinician discussions about families' goals and preferences for treatment.

Impact of a Palatal Fistula After Cleft Palate Repair on Velopharyngeal Closure.

PURPOSE: A palatal fistula is an adverse outcome of cleft palate repair. It is unknown if a palatal fistula will influence velopharyngeal closure, even after repair of the fistula. This study determines the effect of a soft palate fistula on the risk of developing velopharyngeal insufficiency. METHODS: A retrospective chart review was conducted on patients who underwent primary cleft palate repair between 2000 and 2015, with complete records at 4 years of age. Fistulae involving the secondary palate following primary palatoplasty were classified as the soft or hard palate. A forced-entry multivariate logistic regression model was built to detect predictors of velopharyngeal dysfunction. RESULTS: Records of 329 patients were analyzed with a mean follow-up of 8.7 years. A palatal fistula was identified in 89/329 patients (27%) and 29/329 patients (9%) underwent an independent fistula repair. Of the patients with fistula, 44% were located in the hard palate only and 56% had soft palate involvement. Compared to patients without a fistula, rates of velopharyngeal dysfunction were significantly higher in patients with a fistula involving the soft palate (OR 3.875, CI: 1.964-7.648, P < .001) but not in patients with a hard palate fistula (OR 1.140, CI: 0.497-2.613, P = .757). Veau class, age at primary repair, and syndromic status were not significant predictors of VPI (0.128≤P ≤ .975). CONCLUSIONS: A palatal fistula involving the soft palate is a significant predictor for development of velopharyngeal dysfunction after primary palatoplasty. Surgical intervention, at the time of fistula repair, to add vascularized tissue may be indicated to prophylactically decrease the risk of velopharyngeal dysfunction.

Multidisciplinary Optimal Outcomes Reporting and Team Clinic Retention in Isolated Nonsyndromic Cleft Palate.

OBJECTIVE: Optimal Outcomes Reporting was recently introduced to categorize outcomes after cleft palate repair. We seek to propose an expanded version of Optimal Outcomes Reporting and to determine if correlation exists between the expanded outcomes and persistence with team care follow-up through age 9. DESIGN: Retrospective cohort study. SETTING: Cleft team at large pediatric hospital. PATIENTS: Patients with isolated nonsyndromic cleft palate (n = 83) born from 2001-2012. MAIN OUTCOME MEASURES: Patients who continued to present at age 5 or greater were assessed for optimal outcomes. Optimal outcomes were: surgery - no fistula or velopharyngeal insufficiency; otolaryngology - no obstructive sleep apnea or signs of chronic middle ear disease; audiology - no hearing loss; speech-language pathology - no assessed need for speech therapy. RESULTS: Of the 83 patients identified, 41 were assessed for optimal outcomes. Optimal outcome in any discipline was not associated with follow-up through age 9 (0.112 ≤ p ≤ 0.999). For all disciplines, the group with suboptimal outcomes had a higher proportion of patients from geographic areas in the most disadvantaged quartile of social vulnerability index, with the strongest association in the group with suboptimal speech outcome (OR 6.75, 95% CI 0.841-81.1). CONCLUSIONS: Optimal outcomes and retention in team clinic were not statistically significantly associated, but clinically relevant associations were found between patients in the most disadvantaged quartile of social vulnerability and their outcomes. A patient-centered approach, including caregiver education about long-term care for patients with cleft palate, would allow for enhanced resource utilization to improve retention for patients of concern.

Family Experiences with Diagnosis of Craniosynostosis: Thematic Analysis of Online Discussion Boards.

OBJECTIVE: Apply thematic analysis of online discussion boards to characterize families' experiences and concerns regarding craniosynostosis diagnoses to aid physicians in tailoring care to families. DESIGN: Grounded theory-based qualitative analysis. SETTING: Discussion boards related to craniosynostosis identified via Google and Yahoo. PATIENTS/PARTICIPANTS: Posts about craniosynostosis between 2017-2022. INTERVENTIONS: Thematic analysis was performed using three rounds of coding. Post features including author type and use of technical language were examined. MAIN OUTCOME MEASURE: Overarching themes emerging from analysis of posts, with forums analyzed until sufficient thematic repetition was observed. RESULTS: 366 posts from 4 websites by 290 unique users were included. Parents of patients with craniosynostosis wrote 59% of posts while patients wrote 4%. Five selective codes were identified: 1) Building Community, 2) Diagnosis/Evaluation, 3) Treatment, 4) Outcomes, and 5) Emotional Concerns. Building Community was the most assigned code (85% of posts). 71% of parents' posts expressing emotional concerns expressed negative emotions, commonly regarding anxiety about diagnosis (71%), frustration about doctors' responses (21%), or negative reactions to online search results (17%). 88% of patients' posts expressed positive emotions, discussing positive long-term outcomes. Concerns that may guide physicians included anxiety about delayed diagnosis, difficulty distinguishing postpartum head shape changes from craniosynostosis, and difficulty finding a care team. CONCLUSIONS: Online discussion boards allow families of patients with craniosynostosis to share experiences and find community. Improving communication between surgeons, pediatricians, and families about timing of evaluation and revising online information about this condition may ameliorate some anxiety associated with this diagnosis.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Impact of neighborhood deprivation and social vulnerability on long-term outcomes and desire for revision in patients with craniosynostosis.

OBJECTIVE: The authors utilized the area deprivation index (ADI), a validated composite measure of socioeconomic disadvantage, and the social vulnerability index (SVI) to examine whether differences in neighborhood deprivation impact interventions and outcomes among patients with craniosynostosis. METHODS: Patients who underwent craniosynostosis repair between 2012 and 2017 were included. The authors collected data about demographic characteristics, comorbidities, follow-up visits, interventions, complications, desire for revision, and speech, developmental, and behavioral outcomes. National percentiles for ADI and SVI were determined using zip and Federal Information Processing Standard (FIPS) codes. ADI and SVI were analyzed by tertile. Firth logistic regressions and Spearman correlations were used to assess associations between ADI/SVI tertile and outcomes/interventions that differed on univariate analysis. Subgroup analysis was performed to examine these associations in patients with nonsyndromic craniosynostosis. Differences in length of follow-up among the nonsyndromic patients in the different deprivation groups were assessed with multivariate Cox regressions. RESULTS: In total, 195 patients were included, with 37% of patients in the most disadvantaged ADI tertile and 20% of patients in the most vulnerable SVI tertile. Patients in more disadvantaged ADI tertiles were less likely to have physician-reported desire (OR 0.17, 95% CI 0.04-0.61, p < 0.01) or parent-reported desire (OR 0.16, 95% CI 0.04-0.52, p < 0.01) for revision, independent of sex and insurance status. In the nonsyndromic subgroup, inclusion in a more disadvantaged ADI tertile was associated with increased odds of speech/language concerns (OR 4.42, 95% CI 1.41-22.62, p < 0.01). There were no differences in interventions received or outcomes among SVI tertiles (p ≥ 0.24). Neither ADI nor SVI tertile was associated with risk of loss to follow-up among nonsyndromic patients (p ≥ 0.38). CONCLUSIONS: Patients from the most disadvantaged neighborhoods may be at risk for poor speech outcomes and different standards of assessment for revision. Neighborhood measures of disadvantage represent a valuable tool to improve patient-centered care by allowing for modification of treatment protocols to meet the unique needs of patients and their families.

"Calvarial Vault Remodeling Technique for Lambdoid Craniosynostosis."

SUMMARY: Premature fusion of the lambdoid suture is the most uncommon single suture synostosis. It presents with a classic "windswept" appearance, with a trapezoid-shaped head and significant skull asymmetry notable for an ipsilateral mastoid bulge and contralateral frontal bossing. Due to the rarity of lambdoid synostosis, little is known about optimal techniques for its treatment. In particular, the proximity of the lambdoid suture to critical intracranial structures such as the superior sagittal and transverse sinuses represents a potential for significant intraoperative bleeding. Prior work has shown that parietal asymmetry persists after repair in these cases. Here, we present a technique for the treatment of unilateral lambdoid craniosynostosis along with two representative cases.This calvarial vault remodeling technique requires the removal of both ipsilateral and contralateral parietal bones. These are moved across hemispheres and re-inset on opposite sides to help correct the parietal asymmetry. Obliquely orientated barrel stave osteotomies are performed to provide a safe mechanism for correction of occipital flattening. Our early results show improvement in correction of volume asymmetry one year post-operatively relative to patients treated with prior calvarial vault remodeling techniques. We believe the technique presented here corrects the windswept appearance in patients with lambdoid craniosynostosis while also reducing the potential for complications. Further work will be necessary to confirm this technique's long-term efficacy in a larger cohort.

Outcomes after Endoscope-Assisted Strip Craniectomy and Orthotic Therapy for Syndromic Craniosynostosis.

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

Neurodevelopment in unilateral coronal craniosynostosis: a systematic review and meta-analysis.

OBJECTIVE: The current literature on unilateral coronal craniosynostosis is replete with repair techniques and surgical outcomes; however, information regarding neurodevelopment remains unclear. Therefore, the aim of this systematic review and meta-analysis was to comprehensively assess the neurodevelopmental outcomes of patients with unicoronal craniosynostosis compared with their healthy peers or normative data. METHODS: A systematic review of the Ovid MEDLINE, Embase, Web of Science, Scopus, Cochrane Library, and ClinicalTrials.gov databases from database inception to January 19, 2022, was performed. Included studies assessed neurodevelopment of patients with unicoronal craniosynostosis. Two independent reviewers selected studies and extracted data based on a priori inclusion and exclusion criteria. Results of developmental tests were compared with normative data or controls to generate Hedges' g statistics for meta-analysis. The quality of included studies was evaluated using the National Institutes of Health Assessment Tool. RESULTS: A total of 19 studies were included and analyzed, with an overall fair reporting quality. A meta-analysis of 325 postoperative patients demonstrated that scores of general neurodevelopment were below average but within one standard deviation of the norm (Hedges' g = -0.68 [95% CI -0.90 to -0.45], p < 0.001). Similarly, postoperative patients exhibited lower scores in verbal, psychomotor, and mathematic outcome assessments. CONCLUSIONS: This systematic review and meta-analysis found that patients with unicoronal craniosynostosis had poorer neurodevelopment, although scores generally remained within the normal range. These data may guide implementation of regular neurocognitive assessments and early learning support of patients with unicoronal craniosynostosis.

Impact of age at endoscopic metopic synostosis repair on anthropometric outcomes.

OBJECTIVE: Endoscopic strip craniectomy for metopic craniosynostosis relies on rapid growth and postoperative helmeting for correction. Endoscopic repair is generally performed before patients reach 4 months of age, and outcomes in older patients have yet to be quantified. Here, the authors examined a cohort of patients treated with endoscopic repair before or after 4 months of age to determine aesthetic outcomes of delayed repairs. METHODS: Data from eligible patients were retrospectively assessed and aggregated in a dedicated metopic synostosis database. Inclusion criteria were radiographically confirmed metopic synostosis and endoscopic treatment. Patients were dichotomized into two groups: those younger than 4 months and those 4 months or older at the time of repair. The frontal width and interfrontal divergence angle (IFDA) were measured on reconstructed CT images. These measurements, alongside operative time, estimated blood loss, and transfusion rates, were compared between groups using the Student t-test or chi-square test. RESULTS: The study population comprised 28 patients treated before 4 months of age and 8 patients treated at 4-6 months of age. Patient sex and perioperative complications did not differ by age group. Older age at repair was not significantly associated with 1-year postoperative IFDA (140° ± 4.2° vs 142° ± 5.0°, p = 0.28) or frontal width (84 ± 5.2 vs 83 ± 4.4 mm, p = 0.47). CONCLUSIONS: One-year postoperative IFDA and frontal width do not differ significantly between patients treated before and after 4 months of age. Further study with longer follow-up is necessary to confirm the longevity of these results at skeletal maturity.

Cognitive Development in Lambdoid Craniosynostosis: A Systematic Review and Meta-Analysis.

OBJECTIVE: Lambdoid craniosynostosis affects approximately 1 in 33 000 live births per year, and surgical correction is often sought in order to achieve normocephaly and allow for adequate brain growth. However, the effects of lambdoid synostosis and its treatment on cognitive development are unknown. DESIGN: Systematic review and meta-analysis. PATIENTS, PARTICIPANTS: A systematic review of Ovid Medline, Embase, Web of Science, Scopus, Cochrane Central Register of Controlled Trials, and Clinicaltrials.gov was conducted in January 2022. Included studies assessed cognitive development of patients with nonsyndromic unilateral lambdoid craniosynostosis. MAIN OUTCOME MEASURES: Results of developmental tests were compared to normative data or controls to generate Hedges' g for meta-analysis. RESULTS: Nine studies met the inclusion criteria. Meta-analysis of 3 studies describing general cognition showed that cases scored significantly lower than their peers, but within 1 standard deviation (g = 0.37, 95% CI [-0.64, -0.10], P = .01). Meta-analysis of verbal and psychomotor development showed no significant differences in children with lambdoid synostosis. Studies were of fair quality and had moderate-to-high heterogeneity. CONCLUSIONS: Patients with lambdoid craniosynostosis may score slightly below average on tests of general cognition in comparison to normal controls, but results in other domains are variable. Analyses were limited by small sample sizes. Multidisciplinary care and involvement of a child psychologist may be helpful in identifying areas of concern and providing adequate scholastic support. Further research recruiting larger cohorts will be necessary to confirm these findings and extend them to other developmental domains such as attention and executive function.

A multi-institutional survey on calvarial vault remodeling techniques for sagittal synostosis and outcomes analysis for patients treated at 12 months and older.

OBJECTIVE: Surgical treatment of sagittal craniosynostosis is challenging in older patients. This study aimed to assess the effect of increasing age on open surgical technique selection and patient outcomes using the multi-institutional Synostosis Research Group (SynRG) collaboration. METHODS: Surgeons in SynRG were surveyed for key influences on their preferred open calvarial vault remodeling techniques at various patient ages: < 6, 6-12, and > 12 months. The SynRG database was then queried for open repairs of nonsyndromic sagittal craniosynostosis performed for patients older than 12 months of age. Perioperative measures, complications, and preoperative and postoperative cephalic indices were reviewed. RESULTS: All surgeons preferred to treat patients at an earlier age, and most (89%) believed that less-optimal outcomes were achieved at ages older than 12 months. The modified pi procedure was the dominant technique in those younger than 12 months, while more involved open surgical techniques were performed for older patients, with a wide variety of open calvarial vault remodeling techniques used. Forty-four patients met inclusion criteria, with a mean (± SD) age at surgery of 29 ± 16 months. Eleven patients underwent parietal reshaping, 10 parietal-occipital switch, 9 clamshell craniotomy, 7 geometric parietal expansion, 6 modified pi procedure, and 1 parietal distraction. There were no readmissions, complications, or mortality within 30 days postoperatively. Patients' cephalic indices improved a mean of 6.4% ± 4.0%, with a mean postoperative cephalic index of 74.2% ± 4.9%. Differences in postoperative cephalic index (p < 0.04) and hospital length of stay (p = 0.01) were significant between technique cohorts. Post hoc Tukey-Kramer analysis identified the parietal reshaping technique as being significantly associated with a reduced hospital length of stay. CONCLUSIONS: Patient age is an important driver in technique selection, with surgeons selecting a more involved calvarial vault remodeling technique in older children. A variety of surgical techniques were analyzed, with the parietal reshaping technique being significantly associated with reduced length of stay; however, multiple perioperative factors may be contributory and require further analysis. When performed at high-volume centers by experienced pediatric neurosurgeons and craniofacial surgeons, open calvarial vault techniques can be a safe method for treating sagittal craniosynostosis in older children.

School-age outcomes in patients with unilateral coronal synostosis: comparison of fronto-orbital advancement and endoscopic strip craniectomy.

OBJECTIVE: Outcomes research on unilateral coronal synostosis is mostly limited to the early postoperative period. This study examines facial asymmetry, desire for revision, and patient-reported outcomes at school age in children who received either endoscopic strip craniectomy with helmet therapy or fronto-orbital advancement (open repair). METHODS: Patients with repaired unilateral coronal synostosis born between 2000 and 2017, with 3D photographs taken when they were between 3.5 and 8 years of age, were eligible for study inclusion. Three pairs of bilateral linear measurements and two angular measurements were taken. Parent- and physician-reported desire for revision and patient-reported outcomes (Patient-Reported Outcomes Measurement Information System cognitive function and Quality of Life in Neurological Disorders stigma scores) were collected from patient charts. RESULTS: Thirty-five patients qualified, including 25 with open repair and 10 with endoscopic repair. The median patient ages at repair were 3 months and 8 months in the endoscopic and open groups, respectively. The average ages at final 3D photography were 5.8 years and 5.5 years in the endoscopic and open groups, respectively. Digital anthropometry revealed no significant differences in measures of facial asymmetry between the repair groups (p ≥ 0.211). Midface depth (tragion to subnasale) was significantly less symmetric at school age than other linear measures (F(2,102) = 9.14, p < 0.001). Forehead asymmetry was significantly associated with parent- and physician-reported desire for revision (p ≤ 0.006). No significant associations were found between physical asymmetry and patient-reported stigma or cognitive function (p > 0.046, Holm-Bonferroni correction). CONCLUSIONS: Children who underwent open or endoscopic repair for unilateral coronal synostosis have comparable facial symmetry at school age, but midface depth remains highly asymmetrical in both groups. Forehead asymmetry at school age correlates with parent- and physician-reported desire for revision.

Using Patient-Reported Outcome Measures to Screen for Cognitive Function Deficits and Stigma in Patients with Single-Suture Craniosynostosis.

SUMMARY: Children with single-suture craniosynostosis have small but significant deficits in appearance ratings and neurodevelopment. Traditionally, these parameters are studied using a full battery of examinations, which are very time consuming. This study evaluated a convenient method to measure psychosocial parameters in this population by utilizing patient-reported outcomes measures to evaluate cognitive function and stigma. Stigma and cognitive function were measured, using the Patient-Reported Outcomes Measurement Information System and Quality of Life in Neurological Disorders questionnaires, in 59 consecutive patients at least 5 years old presenting to clinic from July of 2018 to January of 2020 with repaired single-suture craniosynostosis. Parents completed parent proxy cognitive function surveys for patients under age 8. Questionnaires were administered electronically as part of clinical care. Scores were automatically transferred to the electronic medical record and correlated with previously acquired Child Behavior Checklist results. Median time to complete the questionnaires was 57 and 49 seconds, respectively. Stigma and cognitive function were significantly correlated with the associated Child Behavior Checklist subscores (Spearman's rho, -0.384, p = 0.023; and Spearman's rho, -0.683, p = 0.001, respectively). The Patient-Reported Outcomes Measurement Information System and Quality of Life in Neurological Disorders questionnaires offer a convenient method of screening psychosocial parameters in children with single-suture craniosynostosis that otherwise would be difficult to obtain during standard visits. Short completion times and electronic scoring increase clinical utility. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Factors contributing to delay or absence of alveolar bone grafting.

INTRODUCTION: Alveolar bone grafting (ABG) delay can lead to suboptimal outcomes. This study seeks to categorize reasons patients with cleft lip and palate have no record of ABG or who underwent later than typical ABG (≥13 years). METHODS: At a single tertiary care center, a retrospective review was performed of all patients with unilateral, complete cleft lip and palate, born 1998-2005. Database query identified which patients had timely, late, or no record of ABG. The retrospective cohort study was performed to categorize ABG delay or absence of recorded ABG. RESULTS: Of 135 participants, 82 (61%) had timely, 8 (6%) had late, and 45 (33%) had no record of ABG. The primary factor for late ABG was noncompliance or refusal (n = 5 of 8, 63%), comorbidity or medical complexity (n = 1 of 8, 13%), orthodontic unpreparedness (n = 1 of 8, 13%), or inaccurate prior assessment of alveolar sufficiency (n = 1 of 8, 13%). The primary factor for ABG record absence was loss to follow-up (n = 40 of 45, 89%), noncompliance or refusal (n = 3 of 45, 7%), comorbidity or medical complexity (n = 1 of 45, 2%), or orthodontic unpreparedness (n = 1 of 45, 2%). Racial majority (White, Asian) patients received preferred care (timely ABG or medically appropriate absence or delay) at a significantly higher rate (67%) than underrepresented minorities (African American, Hispanic, Native American, other) (35%, P = 0.016). Families with private insurance and those who were self-pay received preferred care at a significantly higher rate (77%) than families with Medicaid (42%) (P <0.001). CONCLUSIONS: The high number of patients lost to follow-up highlights the impact of poor retention on ABG completion. Possible health disparities based on race and insurance status warrant clinical focus.

Cost of Cleft Team Care at an Academic Children's Hospital.

BACKGROUND: The financial burden of cleft-craniofacial team care is substantial, and high costs can hinder successful completion of team care. SOLUTION: Collaboration with multiple stakeholders including providers, insurers, and patient guardians, as well as hospital administrators, is critical to increase patient retention and improve final clinical outcomes. WHAT WE DO THAT IS NEW: At our cleft and craniofacial center, charges for a team care visit fall into one of three categories-hospital fees, professional fees, or external fees. There are four types of hospital fees depending on (1) whether the patient is new or returning, and (2) whether the patient saw ≤4 or ≥5 providers. To further elucidate the financial burden (out-of-pocket costs) directly borne by families of children with cleft lip and/or palate, we conducted a retrospective review of billing records of team care visits made between September 2019 and March 2020. Out-of-pocket costs for a single team care visit (on a commercial insurance plan) ranged from $4 to approximately $1220 and had a median (IQR) of $445 ($118, $749).

Long-Term Effects of Nasoalveolar Molding in Patients With Unilateral Cleft Lip and Palate: A Systematic Review and Meta-Analysis.

OBJECTIVE: The aim of this systematic review and meta-analysis was to investigate long-term treatment effects of nasoalveolar molding (NAM) in patients with unilateral cleft lip and palate (UCLP). DESIGN: Included manuscripts met the following criteria: (1) involved patients with UCLP who received NAM; (2) included comparison group(s) who either received non-NAM passive presurgical infant orthopedic appliances (PSIO) or who did not receive any PSIO; (3) reported at least one objective or validated measure of nasolabial, craniofacial, or palatal form; and (4) had patient follow-up beyond 4 years of age. RESULTS: A total of 12 studies were included in this review. Meta-analyses were possible for Asher-McDade parameters and cephalometric measurements. Compared to patients who did not receive any PSIO, those who underwent NAM therapy were more likely to have good to excellent frontal nasal form (Risk ratio: 2.4, 95% CI: 1.24-3.68) and vermillion border (Risk ratio: 1.8, 95% CI: 1.19-2.71). However, there were no statistically significant differences in cephalometric measurements between these groups. Additionally, there were no statistically significant differences between patients receiving NAM versus non-NAM PSIO. There was insufficient evidence to determine the impact of NAM on dental arch development. CONCLUSIONS: The preponderance of evidence in this review suggests that NAM produces benefits in nasolabial aesthetic form when compared with no appliance-based presurgical treatment. However, there is insufficient evidence to conclude whether NAM produces such benefits when compared with other passive PSIOs.