PENN STATE SPINA BIFIDA CLINIC REGISTRY

ABSTRACTThe development and progression of scoliosis in children and adults with myelomeningocele is complex anddependent on multiple neurological and musculoskeletal factors. Root neurosurgical contributors to scoliosis inthis population may include hydrocephalus/shunt malfunction, Chiari malformation and syringomyelia, andtethered cord. The PI and team propose to analyze the NSBPR to identify these neurosurgical contributions tothe need for scoliosis correction in the myelomeningocele population. The investigators will study the frequencyof scoliosis correction as well as the variability among clinics, controlling for age, lesion level, and ambulationstatus (factors that influence scoliosis frequency and/or progression); analyze correlations with otherneurological, urological and orthopedic factors among those undergoing scoliosis correction; identify thefrequency of scoliosis correction subsequent to TCR for those presenting with scoliosis as the sole, or as one ofother clinical indications for TCR; and compare the frequency of neurological/urological deterioration followingscoliosis 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 MedicalCenter (PSHMC), a tertiary/quaternary care University based medical center, and the Penn State Children’sHospital (PSCH), a free standing Children’s Hospital on the University campus that functions as a regionalpediatric referral center for the children of central Pennsylvania. The PSSBC has been in continuous operationfor 40 years and currently cares for 563 adult and pediatric patients. The PSSBC has been an active participantin 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 uniqueresearch perspective for the spina bifida clinic registry project because 1) the clinic is one of only a few in theUnited States with a large, active, and growing adult spina bifida clinic; 2) it is one of a few clinics in the UnitedStates to serve primarily a rural population and could serve as a model to study the role of a spina bifida clinicin a widespread, decentralized, and mostly rural environment; and 3) the clinic is unusual in caring for a sizablenumber of individuals from the Amish, Mennonite and Brethren in Christ communities.