John Persing, MD

Title A Novel Approach to Measuring the Impact of Surgery in Craniosynostosis using Event-Related Potentials (ERPs) Background Much debate surrounds whether children with non-syndromic craniosynostosis (NSC) sustain neurodevelopmental deficits. This controversy surrounding surgery in patients with NSC has initiated many studies involving neuropsychological measurements attempting to characterize the impact of surgical intervention on mental function. However, early studies employing developmental quotients to test infants fail to adequately predict the impact of surgery on neurodevelopment. Unlike these primitive measures, studies utilizing event-related potentials (ERPs), based on cerebral electrical activity, in infants have been shown to be predictive of future outcomes. Objectives This study addresses previous shortcomings by using more sensitive neuropsychological measures in patients with NSC undergoing surgical correction. Furthermore, the study allows us to identify which infants with NSC are at risk of future abnormal language development and neurocognitive deficits. Moreover, ERPs can potentially serve as a standardized measure to assess surgical intervention. Methods Infants with NSC were identified at the Yale Craniofacial clinic prior to surgery. These patients underwent pre-operative testing using ERPs and will be tested post-operatively using the same technique. Their results will be compared to age and gender matched controls. Results Thus far, fifteen patients have subjected to the above protocol. Once their post-operative testing is complete, they will be compared to control groups. Conclusions If the results show that there is a significant difference between the way infants with craniosynostosis process speech sounds as compared to controls, this will provide evidence that surgical intervention is not merely a cosmetic procedure, but rather one necessary to prevent the impairment of neurodevelopment. Also, comparing outcomes between infants subjected to surgery at different ages will elucidate the best timing for surgical intervention. This approach is being supplemented by fMR analysis and long-term neurocognitive studies. References 1. Kapp-Simon, K. A. Mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction. Plast Reconstr Surg 94: 408-410, 1994. 2. Magge, S. N., Westerveld, M., Pruzinsky, T., et al. Long-term neuropsychological effects of sagittal craniosynostosis on child development. J Craniofac Surg 13: 99-104, 2002. 3. Arnaud, E., Renier, D., Marchac, D. Prognosis for mental function in scaphocephaly. J Neurosurg 83: 476-479, 1995. 4. Renier, D., Marchac, D. Longitudinal assessment of mental development in infants with nonsyndromic craniosynostosis with and without cranial release and reconstruction [discussion]. PlastReconstrSurg 92: 840-841, 1993.

Title: Assessing Neurodevelopment in Positional Plagiocephaly Background: Positional plagiocephaly has been reported to affect as many as 48% of typical healthy infants. Once a rare condition, it has become a prevalent diagnosis since the early 1990s, concurrent with the American Academy of Pediatrics’ recommendation for supine sleep to reduce Sudden Infant Death Syndrome (SIDS). Traditionally, physicians have regarded positional plagiocephaly as a benign condition with no neurodevelopmental sequelae, but recent research has suggested some evidence to the contrary. These studies have relied on the Bayley Scales of Infant Development (BSID) for their assessment. While BSID is the most commonly-employed developmental assessment tool in infancy, it is a poor predictor of long-term intellectual function, and may not be sensitive enough to detect very subtle developmental delays. Unlike BSID, event-related potentials (ERPs, cerebral electrical responses to stimuli) have been shown to be predictive of future cognitive outcomes by other research teams. However, ERPs have never been used to study neurodevelopment in plagiocephaly. Objectives: This study seeks to establish baseline cortical ERPs for infants with positional plagiocephaly and determine if a difference exists between infants with the diagnosis and non-afflicted controls. As a secondary aim, we plan to investigate the effects of skull-molding helmet therapy on auditory ERPs. Methods: Infants with positional plagiocephaly are being identified during consultation at the Yale Craniofacial clinic, and at the Cranial Technologies, Inc. office in Madison, CT. These infants will undergo two ERP sessions: one at baseline, and a second one after 3 months of skull-molding helmet therapy or physical therapy. Results: Pending. Conclusions: Neurodevelopment in positional plagiocephaly remains a debated topic. If the results of this study show a significant difference between infants with the diagnosis and non-affected controls in terms of auditory ERPs, they would provide evidence against the notion that plagiocephaly is an entirely benign condition. We will investigate whether the severity of deformation can be correlated with ERP differences. Additionally, if differences do exist between affected infants and controls, we will investigate whether helmet therapy and/or physical therapy can ameliorate them. References: 1. Molfese, D.L., Predicting dyslexia at 8 years of age using neonatal brain responses. Brain Lang 2000; 72: 238-45. 2. Panchal J, Amirsheybani H, Gurwitch R, et al. Neurodevelopment in children with single-suture craniosynostosis and plagiocephaly without synostosis. Plast Reconstr Surg 2001;108:1492-8; discussion 9-500. 3. Persing J, James H, Swanson J, Kattwinkel J. Prevention and management of positional skull deformities in infants. American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery. Pediatrics 2003;112:199-202. 4. Speltz ML, Collett BR, Stott-Miller M, et al. Case-control study of neurodevelopment in deformational plagiocephaly. Pediatrics 2010;125:e537-42. 5. Teichgraeber JF, Ault JK, Baumgartner J, et al. Deformational posterior plagiocephaly: diagnosis and treatment. Cleft Palate Craniofac J 2002;39:582-6.neurodevelopment in craniosynostosis, neurodevelopment in deformational plagiocephaly, cosmetic surgery, efficacy/safety