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Long-Term Neurocognitive Outcomes in 204 Single-Suture Craniosynostosis Patients
Adam H Junn1, Aaron S Long1, Sacha C Hauc1, Alexandra Junn1, Jean Carlo Rivera1, Jacob Dinis1, Mariana N Almeida1, David P Alper1, Linda Mayes1, Michael DiLuna1, Derek Steinbacher1, John A Persing1, Michael Alperovich1
Division of Plastic Surgery, Yale School of Medicine, New Haven, CT

Importance: Craniosynostosis has been associated with a variety of neurocognitive deficits. Categorizing these neurocognitive profiles is important for nurturing cognitive development in children.Objective: To critically evaluate our experience with non-syndromic craniosynostosis and explore the cognitive profiles of the main types of NSC.Design: Retrospective data analysis.
Setting: Database of neurocognitive tests conducted over the past decadeParticipants: Patients with surgically corrected NSC that have undergone neurocognitive testing between 2014-2022Main Outcomes and Measures: Type of suture affected by NSC and IQ test scores, and cognitive development.
Results: 204 patients completed neurocognitive testing (139 sagittal, 39 metopic, 22 unilateral coronal and 4 lambdoid). 110 (54%) of the cohort was male, and 150 (74%) were White. The mean IQ was 106.10 14.01 and the mean age at surgery and testing were 9.0 12.2 months and 10.9 4.0 years respectively. Patients with sagittal synostosis had higher scores in every testing category than patients with 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). Significantly higher scores were recorded for sagittal synostosis relative to unilateral coronal synostosis for visuomotor integration (101.62 13.64 vs 94.95 10.24) and visual perception (103.81 12.42 vs 94.82 12.75). There were no significant differences between groups in terms of age at surgery, age at testing, parental education level, prematurity, and sex.
Conclusions and Relevance: Compared to patients with sagittal synostosis, patients with metopic synostosis exhibited lower testing 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 (visual information processing) when compared to patients with sagittal synostosis.


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