Penn State Spina Bifida Clinic Registry

摘要 脊髓脊膜膨出的儿童和成人脊柱侧凸的发展和进展是复杂的， 取决于多种神经和肌肉骨骼因素。脊柱侧凸的根神经外科因素 该人群可能包括脑积水/分流功能障碍、基亚里畸形和脊髓灰质炎， 脊髓栓系PI和团队建议分析NSBPR，以确定这些神经外科贡献， 脊髓脊膜膨出人群需要脊柱侧凸矫正。调查人员将研究 脊柱侧凸矫正率以及诊所间的差异，控制年龄、病变水平和截肢率 状态（影响脊柱侧凸频率和/或进展的因素）;分析与其他因素的相关性 脊柱侧凸矫正患者的神经、泌尿和矫形因素;确定 TCR后脊柱侧凸矫正的频率对于那些表现为脊柱侧凸的患者来说是唯一的，或者是 TCR的其他临床适应症;并比较以下神经/泌尿系统恶化的频率 脊柱侧凸修复术（有或无既往TCR），以研究脊柱侧凸矫正前是否需要TCR。 宾夕法尼亚州立大学脊柱裂诊所（PSSBC）是宾夕法尼亚州立大学健康好时医疗中心的一个多学科诊所。 中心（PSHMC），一个三级/四级护理大学的医疗中心，和宾夕法尼亚州立大学儿童医院。 医院（PSCH），一个独立的儿童医院在大学校园，作为一个区域性的功能 宾夕法尼亚州中部儿童的儿科转诊中心。PSSBC一直在持续运作， 40年来，目前为563名成人和儿童患者提供护理。PSSBC一直积极参与 自2011年以来，脊柱裂临床登记示范项目，共招募了481名患者 （包括188名儿童和293名成人），占诊所人口的85%。PSSBC带来了一个独特的 脊柱裂临床登记项目的研究前景，因为1）该诊所是世界上仅有的几个 美国有一个大型的，活跃的，不断发展的成人脊柱裂诊所; 2）它是美国少数几个诊所之一 国家主要为农村人口服务，可作为研究脊柱裂诊所作用的模式 在一个广泛的，分散的，主要是农村环境;和3）诊所是不寻常的照顾相当大的 来自阿米什人、门诺派和基督教会的一些人。

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.