Grants Funded

Abstract

Impact Statement: Neurologic follow-up after craniosynostosis is currently assessed 6 or more years after the initial surgical correction. A contemporaneous assessment is necessary in order to be able to address the neurologic impact of clinical decisions such as timing and type of surgery. Functional MRI provides both functional data as well as structural localization of which areas of the brain are most affected. It has been applied successfully in patients of this age before in other disciplines. By defining the presenting fMRI patterns in sagittal synostosis in infancy, this study will help define the impact of craniosynostosis on brain function and the role of fMRI for future neurologic studies.

Project Summary: Craniosynostosis is characterized by the pathological fusion of cranial sutures leading to restricted skull growth at the fused suture with compensatory growth at the remaining patent sutures. Sagittal synostosis is the most common form of non-syndromic craniosynostosis and has been associated with both an abnormal head shape and long-term neurocognitive deficits even after surgical correction.

Functional MRI allows a non-invasive and contemporaneous method to assess the aberrant functional connectivity patterns in craniosynostosis. Using resting-state fMRI and diffusion tensor imaging in pre and post-operative sagittal synostosis patients, we aim to define the microstructural brain abnormalities associated with sagittal synostosis, evaluate how these changes are affected by surgical correction, and ultimately, correlate these structural differences with neurocognitive outcomes at school age. The gold standard for evaluating neurological function has been neurocognitive testing, and it has traditionally been used to evaluate long-term outcomes following craniosynostosis surgery. This testing is most accurately performed after the age of 6, and some have criticized that the results can be impacted by sociodemographic factors. Previous efforts have been made to study brain function at the time of diagnosis in infancy. One modality, EEG, has been used effectively to assess brain function at infancy. By measuring response to auditory stimuli, EEG allows assessment of abnormal neural response. EEG is effective at providing insight at the time of diagnosis. However, EEG cannot be localized to specific structures in the brain. The potential advantage of functional MRI is its ability to provide both structural and functional data to provide insight into the changes associated with sagittal synostosis. Small pilot studies at Yale have demonstrated the feasibility of performing fMRI safely and with high fidelity in infants. Moreover, a recently published infant brain atlas provides age and gender-matched controls. This study aims to perform a focused evaluation of pre- and post-operative sagittal synostosis infants to identify any resting-state fMRI and diffusion tensor imaging patterns in the most common type of craniosynostosis pathology.

Biography
I have been an Assistant Professor at Yale University since 2016 and Co-Director of Craniofacial Surgery since 2019. Prior to Yale, I trained in plastic surgery and craniofacial surgery at NYU and was educated at Harvard, Oxford, and the Johns Hopkins School of Medicine. I am committed to a career as an academic craniofacial surgeon and have appreciated the invaluable support of academic societies and my institution. In 2019, I received the ASMS CRANIO traveling fellowship for promising young faculty in craniofacial surgery. In 2020, I received the Yale Department of Surgery’s Academic Development Award. My research interests are centered on understanding the relationship between brain function and craniosynostosis. I have analyzed neurocognitive function in patients with craniosynostosis by morphologic severity, laterality, and varying surgical techniques. To date, the research projects have resulted in over 100 peer-reviewed publications with a primary focus in craniosynostosis. In 2018, I partnered with CHOP to study the morphologic and neurocognitive outcomes of adolescents with unilateral coronal synostosis. In 2019, we completed a study with Wake Forest comparing cognitive outcomes between cranial vault remodeling and spring-assisted surgery for sagittal synostosis. In 2020, I became site-director of a multi-institutional collaboration to study the functional MRI profiles of patients with craniosynostosis.