Penn State Spina Bifida Clinic Registry

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ABSTRACT The development and progression of scoliosis in children and adults with myelomeningocele is complex and dependent on multiple neurological and musculoskeletal factors. Root neurosurgical contributors to scoliosis in this population may include hydrocephalus/shunt malfunction, Chiari malformation and syringomyelia, and tethered cord. The PI and team propose to analyze the NSBPR to identify these neurosurgical contributions to the need for scoliosis correction in the myelomeningocele population. The investigators will study the frequency of scoliosis correction as well as the variability among clinics, controlling for age, lesion level, and ambulation status (factors that influence scoliosis frequency and/or progression); analyze correlations with other neurological, urological and orthopedic factors among those undergoing scoliosis correction; identify the frequency of scoliosis correction subsequent to TCR for those presenting with scoliosis as the sole, or as one of other clinical indications for TCR; and compare the frequency of neurological/urological deterioration following scoliosis repair with, and without prior TCR to study whether TCR is necessary prior to scoliosis correction. The Penn State Spina Bifida Clinic (PSSBC) is a multidisciplinary clinic of the Penn State Health Hershey Medical Center (PSHMC), a tertiary/quaternary care University based medical center, and the Penn State Children’s Hospital (PSCH), a free standing Children’s Hospital on the University campus that functions as a regional pediatric referral center for the children of central Pennsylvania. The PSSBC has been in continuous operation for 40 years and currently cares for 563 adult and pediatric patients. The PSSBC has been an active participant in the Spina Bifida Clinic Registry Demonstration Project since 2011, and has enrolled a total of 481 patients (including 188 children and 293 adults) representing 85% of the clinic population. The PSSBC brings a unique research perspective for the spina bifida clinic registry project because 1) the clinic is one of only a few in the United States with a large, active, and growing adult spina bifida clinic; 2) it is one of a few clinics in the United States to serve primarily a rural population and could serve as a model to study the role of a spina bifida clinic in a widespread, decentralized, and mostly rural environment; and 3) the clinic is unusual in caring for a sizable number of individuals from the Amish, Mennonite and Brethren in Christ communities.