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.

Buccal Myomucosal Flap Repair for Velopharyngeal Dysfunction.

BACKGROUND: Velopharyngeal dysfunction (VPD) is the incomplete separation of the nasal and oral cavities during speech sound production that can persist following primary palatoplasty. Surgical technique used in management of VPD (palatal re-repair versus pharyngeal flap or sphincter pharyngoplasty) is often dictated by the preoperative velar closing ratio and closure pattern. Recently, buccal flaps have increased in popularity in management of VPD. Here, the authors investigate the effectiveness of buccal myomucosal flaps in the treatment of VPD. METHODS: A retrospective review was performed of all patients undergoing secondary palatoplasty with buccal flaps at a single center between 2016 and 2021. Preoperative and postoperative speech outcomes were compared. Speech assessments included perceptual examinations, graded on a four-point scale of hypernasality, and speech videofluoroscopy, from which the velar closing ratio was obtained. RESULTS: A total of 25 patients underwent buccal myomucosal flap procedures for VPD at a median of 7.1 years after primary palatoplasty. Patients had significantly increased velar closing postoperatively (95% versus 50%; P < 0.001) and improved speech scores ( P < 0.001). Three patients (12%) had continued hypernasality postoperatively. There were no occurrences of obstructive sleep apnea. CONCLUSIONS: Treatment of VPD with buccal myomucosal flaps leads to improved speech outcomes without the risk of obstructive sleep apnea. Traditionally, palatal re-repair techniques have been used for smaller preoperative velopharyngeal gaps; however, the addition of buccal flaps allows for anatomical velar muscle correction for patients with a larger preoperative velopharyngeal gap. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

What do patients want to know about gender-affirming surgery? Analysis of common patient concerns and online health materials.

PURPOSE: Patients considering gender-affirming surgery often utilize online health materials to obtain information about procedures. However, the distribution of patient concerns and content of online resources for gender-affirming surgery have not been examined. We aimed to quantify and comprehensively analyze the most searched questions of patients seeking gender-affirming surgery and to examine the quality and readability of associated websites providing the answers. METHODS: Questions were extracted from Google using the search phrases "gender-affirming surgery," "transgender surgery," "top surgery," and "bottom surgery." Questions were categorized by topic and average search volume per month was determined. Websites linked to questions were categorized by type, and quality of the health information was evaluated utilizing the DISCERN instrument (16-80). Readability was assessed with the Flesch Reading Ease Score and Flesch-Kincaid Grade Level. RESULTS: Ninety questions and associated websites were analyzed. Common questions were most frequently answered by academic websites (30%). Topics included cost (27%), technical details of surgery (23%), and preoperative considerations (11%). Median (interquartile range) DISCERN score across all website categories was 42 (18). The mean readability was of a 12th-grade level, well above the grade six reading level recommended by the American Medical Association. CONCLUSIONS: Online gender-affirming surgery materials are difficult to comprehend and of poor quality. To enhance patient knowledge, informed consent, and shared decision-making, there is a substantial need to create understandable and high-quality online health information for those seeking gender-affirming surgery.

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.

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.

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.