Penn State Spina Bifida Clinic Registry

摘要 患有脊髓脊膜膨出的儿童和成人脊柱侧弯的发展和进展是复杂的和 依赖于多种神经和肌肉骨骼因素。根神经外科因素在脊柱侧弯中的作用 这一人群可能包括脑积水/分流功能障碍、Chiari畸形和脊髓空洞症，以及 系绳。PI和团队建议分析NSBPR以确定这些神经外科因素对 脊髓脊膜膨出人群中脊柱侧弯矫正的必要性。调查人员将研究频率 脊柱侧弯矫正率以及临床之间的变异性，控制年龄、病变水平和行走能力。 状态(影响脊柱侧弯频率和/或进展的因素)；分析与其他 接受脊柱侧弯矫正的患者的神经、泌尿和骨科因素；确定 以脊柱侧凸为唯一症状的患者或其中一例患者接受TCR治疗后脊柱侧弯矫正的频率 TCR的其他临床指征；并比较以下神经/泌尿系统恶化的频率 脊柱侧弯矫形前是否需要行TCR矫形？ 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好时医疗集团旗下的一家多学科诊所 中心(PSHMC)，一个以大学为基础的第三/第四级医疗中心，以及宾夕法尼亚州立儿童医院 医院(PSCH)，大学校园内的一家独立的儿童医院，作为地区性的 宾夕法尼亚州中部儿童儿科转诊中心。PSSBC一直在持续运行 已有40年的历史，目前为563名成人和儿童患者提供护理。PSSBC一直是积极的参与者 自2011年以来，参加了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)，占临床人口的85%。PSSBC带来了独特的 脊柱裂临床登记项目的研究前景，因为1)该诊所是为数不多的几个 美国拥有一家规模庞大、活跃且不断增长的成人脊柱裂诊所；2)它是美国为数不多的几家诊所之一 该州主要服务于农村人口，可以作为研究脊柱裂诊所作用的典范 在一个广泛的、分散的和主要是农村的环境中；3)诊所在照顾相当大的 基督社区中来自亚米希人、门诺派和弟兄会的人数。

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.