Spina bifida is the most common congenital anomaly of the central nervous system and the first non-fatal fetal lesions to be addressed by fetal intervention. While research in spina bifida has been performed in rodent, nonhuman primate, and canine models, sheep have been a model organism for the disease. This review summarizes the history of development of the ovine model of spina bifida, previous applications, and translation into clinical studies. Initially used by Meuli et al. [Nat Med. 1995;1(4):342-7], fetal myelomeningocele defect creation and in utero repair demonstrated motor function preservation. The addition of myelotomy in this model can reproduce hindbrain herniation malformations, which is the leading cause of mortality and morbidity in humans. Since inception, the ovine models have been validated numerous times as the ideal large animal model for fetal repair, with both locomotive scoring and spina bifida defect scoring adding to the rigor of this model. The ovine model has been used to study different methods of myelomeningocele defect repair, the application of various tissue engineering techniques for neuroprotection and bowel and bladder function. The results of these large animal studies have been translated into human clinical trials including Management of Meningocele Study (MOMS) trial that established current standard of care for prenatal repair of spina bifida defects, and the ongoing trials including the Cellular Therapy for In Utero Repair of Myelomeningocele (CuRe) trial using a stem cell patch for repair. The advancement of these life savings and life-altering therapies began in sheep models, and this notable model continues to be used to further the field including current work with stem cell therapy.
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http://dx.doi.org/10.1159/000531750 | DOI Listing |
Birth Defects Res
January 2025
School of Nursing, Ulster University Belfast, Belfast, Northern Ireland, UK.
Introduction: While improved medical and surgical care for children with pina bifida has improved their survival, some may have lower cognitive, behavioral and educational performance. The paper assesses the effect of spina bifida on cognitive, behavioral, and educational outcomes in 5-11 year olds.
Methods: A cross-sectional study design was used where data were collected from parents/guardians and teachers using Behavior Rating Inventory of Executive Function, second edition (BRIEF2), Strengths and Difficulties Questionnaire (SDQ), and Teacher Academic Attainment Scale (TAAS).
Prune belly syndrome (PBS), or Eagle-Barrett syndrome, is a rare congenital disorder marked by abdominal wall muscle deficiency, urinary tract anomalies, and cryptorchidism, causing significant abdominal wall laxity and functional impairment. This case report discusses an innovative approach to abdominal wall reconstruction in a 19-year-old male patient with PBS and associated conditions, including chronic renal failure and spina bifida. Previously, he underwent distal ureterectomy and vesicoureteral reimplantation at the age of two years to correct urinary tract dilation and bilateral orchiopexy.
View Article and Find Full Text PDFRehabil Psychol
January 2025
Department of Rehabilitation Medicine, Children's Hospital Colorado.
Purpose/objective: The transition from childhood to adulthood often involves emotional challenges. These problems may be especially prominent for transition-age adults (TAA) with pediatric-onset disabilities, although there are currently few studies that speak to this. The aim of this study is to characterize depressive symptoms and the association with family functioning in a sample of TAA with pediatric-onset disabilities.
View Article and Find Full Text PDFSpine J
January 2025
Department of Orthopaedic Surgery, Anshin Hospital, Kobe, Japan.
Background: Pediatric lumbar spondylolysis (LS) is common in junior and senior high school athletes. Lower LS (L4-L5 level) is more common in children, and upper LS (L1-L3 level) is relatively rare; therefore, the pathogenesis of upper LS remains unclear.
Purpose: To elucidate the mechanisms of upper LS by identifying and comparing characteristics between upper and lower LS cases.
Hum Brain Mapp
January 2025
Center for MR Research, University Children's Hospital Zurich, Zurich, Switzerland.
The human brain connectome is characterized by the duality of highly modular structure and efficient integration, supporting information processing. Newborns with congenital heart disease (CHD), prematurity, or spina bifida aperta (SBA) constitute a population at risk for altered brain development and developmental delay (DD). We hypothesize that, independent of etiology, alterations of connectomic organization reflect neural circuitry impairments in cognitive DD.
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