Cognitive and Behavioral Outcomes of Cranially-Mature Unilateral Coronal Craniosynostosis
Robin T. Wu, BS1, Kyle S. Gabrick, MD1, Anusha Singh, BS1, Jesse A. Taylor, MD2, Scott P. Bartlett, MD2, Derek M. Steinbacher, DMD, MD1, John A. Persing, MD1, Michael A. Alperovich, MD, MSc1.
1Yale University School of Medicine, New Haven, CT, USA, 2Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Purpose: Unilateral coronal (ULC) nonsyndromic craniosynostosis asymmetrically restricts brain growth with potential long-term neuropsychiatric deficits. Given the limited understanding of neurocognitive outcomes, this study sought to outline the neurodevelopmental profile of cranially-mature patients following surgical correction of nonsyndromic ULC.
Methods: Cranially-mature, post-operative ULC patients the Yale Craniofacial Clinic and the Children's Hospital of Philadelphia (CHOP) completed a double-blinded neurodevelopmental assessment, which included Wechsler Fundamentals: Academic Skills, Wechsler Abbreviated Scale of Intelligence, and Beery-Buktenica Visual-Motor Developmental Test. Parents/guardians were also surveyed with the Behavior Rating Inventory of Executive Function, Child Behavior Checklist, and for 22 socioeconomic/demographic factors.
Results: Twenty patients (12 Yale, 8 CHOP) participated with a mean age of 12.1 years. All patients underwent cranial vault remodeling at a mean age of 8.0 months and included 55% female and 50% right-sided ULC craniosynostosis. ULC patients on average had mean academic achievement performance percentiles above the national mean (word reading 76.3%, reading comprehension 60.8%, reading composite 68.1%, spelling 61.4%) with the notable exception of numerical operations (47.2%); patients scored significantly lower for numerical operations compared to word reading (p=0.022). Mean verbal IQ (VIQ) was highest at 117.3 while mean performance IQ (PIQ) was lower at 106.4. Patients performed below average on all Beery-Buktenica visual-motor tests (visual-motor integration 42.5%, visual perception 49.6%, motor coordination 26.0%); motor coordination was poorer than both visual-motor-integration and visual-perception (p=0.027, p=0.005). Regarding behavioral surveys, patients performed the poorest on behavioral regulation (38.7%) and emotional control (39.4%) in the Behavior Rating Inventory of Executive Function survey. Patients performed the worst on externalizing problems (45.1%) in the Child Behavior Checklist. Surgery before 7 months trended towards improved motor coordination (p=0.067), and female patients had higher VMI (p=0.024). Breast-fed patients had higher overall PIQ (p=0.034), VMI (p=0.014), and visual perception scores (p=0.031). Significant correlations existed between, paternal education/visual perception (r=0.450; p=0.046), household income/VIQ (r=0.628; p=0.004), and birth weight/numerical operations (r=-0.578; p=0.015). Right ULC patients had improved spelling compared to left ULC patients (p=0.033). While no significant differences between laterality were found for any other neurocognitive or behavioral score, subjects with right sided fusion scored higher on all language/verbal tests. Follow-up multiple regression between coronal sidedness and spelling scores was performed to control for all three IQ measures (VIQ, PIQ, FSIQ), all variables that significantly impacted performance (age at surgery, sex, breast-feeding status, paternal education, household income), age at testing, and race. Adjusted analysis revealed right-sided ULC still significantly predicted higher spelling scores (R2 0.650, p=0.033).
Conclusion: Our prospective multi-institutional evaluation of cranially-mature patients with ULC revealed improved language academic achievement in comparison to mathematics, higher VIQ than PIQ, and overall poor visuo-motor skills. Left-sided ULC may lead to deficit spelling performance possibly in response to left brain restriction. Breast feeding was associated with overall improved VMI, visual perception, and PIQ, and should be encouraged for patients diagnosed with ULC. Patients and families with ULC should be counseled on expected outcomes and early neurocognitive/motor exams should be included in patient care.
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