{"title":"Etiology and Pathophysiology of the Spina Bifida","authors":"R. Opšenák, R. Richterová, B. Kolarovszki","doi":"10.5772/INTECHOPEN.97467","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.97467","url":null,"abstract":"The spina bifida is a congenital anomaly that results in an abnormal formation of the spine and the spinal cord. The two dominant types of spinal dysraphism are based on appearance - open spina bifida if the lesion is visible and closed spina bifida if the lesion is not visible on the body surface. These conditions lead to a different spectrum of neurological effects according to the degree of neurulation disruption. The prevalence of neural tube defects has different rates among different ethnicity, geography, gender, and countries. Genetic, nutritional and environmental factors play a role in the etiology and pathogenesis of the spina bifida. Congenital anomalies in the vast majority concern children living in the early neonatal period who have important medical, social or educational needs. The lifetime cost of a child born with the spina bifida is estimated at over €500,000.","PeriodicalId":108199,"journal":{"name":"Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications","volume":"651 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"132703838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Surgical Treatment of Neural Tube Defects","authors":"J. Šutovský","doi":"10.5772/INTECHOPEN.95408","DOIUrl":"https://doi.org/10.5772/INTECHOPEN.95408","url":null,"abstract":"Neural tube defects (NTDs) are developmental pathologies associated with undesirable lifelong consequences. Incidence of these pathologies differs between countries and regions depending on socio-economic and healthcare quality. It is also influenced by folic acid and zinc supplementation. Genetic factors influence probability of NTD, increasing risk of defect in siblings up to 3–8%. Estimated incidence in United States is 3–4/10000 live births, and worldwide incidence increases on about 10/10000 live births. Despite various types and localizations of spina bifida, in all of them neural tissue is in danger. This can lead to various types of neurologic disorders. Not only due to direct damaging of spinal cord and nerve roots but also other parts of central nervous system are also endangered by disturbed prenatal development. Other consequences as orthopedic abnormalities, bladder, and bowel dysfunction influence quality of life. Surgical therapy is often the only possibility to preserve existing function of neural tissue, allows its further development and prevents complications. In this chapter surgical techniques with aim to restore spinal cord and nerve roots anatomy, preservation of its function and defect closures are presented. Also, treatment of possible comorbidities and complications is discussed. Spina bifida management requires multi-speciality cooperation and care to monitor, prevent and treat various potential complication that can negatively influence quality of life and even survival. Prenatal diagnosis is based on maternal screening of serum alpha fetoprotein (AFP) levels and prenatal ultrasonography examination. As the suspicion of neural tube defect arises, an amniocentesis is recommended to complete a genetic analysis and obtain amniotic fluid for more precise AFP and acetylcholinesterase examination. Some types of neural tube defects are diagnosed after delivery, some are symptomatic until adulthood and some are diagnosed incidentally. Each of them requires specific management, based on underlying pathology.","PeriodicalId":108199,"journal":{"name":"Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-03-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130634005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
K. Adam, F. Hariri, Wei Lee Chee, K. Purmal, Mohd Faizal Abdullah, N. Berahim
{"title":"Craniofacial Corrective Surgery in Syndromic Craniosynostosis","authors":"K. Adam, F. Hariri, Wei Lee Chee, K. Purmal, Mohd Faizal Abdullah, N. Berahim","doi":"10.5772/intechopen.94584","DOIUrl":"https://doi.org/10.5772/intechopen.94584","url":null,"abstract":"This surgical field has now progressed and becoming an established subspecialty involving various surgical disciplines worldwide. Various complex CMF syndromes reported in syndromic craniosynostosis include Crouzon, Apert and Pfeiffer syndromes. These syndromes carry specific functional discrepancies associated with the affected structural anomaly and may therefore have functional issues involving the brain, eye and airway among others. As corrective surgery is often indicated depending on the affected vital functions, other factors that need to be considered are patient’s age, comorbidities, urgency, available expertise and patient’s overall prognosis based on the degree of anomaly. As such, the corrective surgery can be categorized into; (1) intermediate which is performed at an early phase and aimed to improve or salvage important vital functions such as the brain, eye, airway or feeding which are important for the child’s development and, (2) definitive treatment aimed at permanently correct the functional discrepancies. Intermediate corrective surgery may include invasive procedures such as ventriculo-peritoneal (VP) shunts, tarsorrhaphy, adenotonsillectomy and tracheostomy whereas definitive corrective surgery may include surgical procedures such as monobloc, Le Fort III osteotomy, posterior cranial vault expansion and mandibular advancement. This chapter will elaborate on the indications, types, challenges in the management and the proposed prevention measures in corrective surgery for specifically for syndromic craniosynostosis patients.","PeriodicalId":108199,"journal":{"name":"Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133049932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Orthopedic Approach to Spina Bifida","authors":"Roselle C. Okubo, C. Silveri, A. C. Belzarena","doi":"10.5772/intechopen.94901","DOIUrl":"https://doi.org/10.5772/intechopen.94901","url":null,"abstract":"Spina bifida is a common nervous system malformation and it encompasses a wide array of presentations with diverse orthopedic challenges. Manifestations of this disease can include dislocates hips, joint contractures, spine deformity such as scoliosis or kyphosis, clubfeet and limb rotational deformities. Additionally, many of these patients are non-ambulatory and prone to osteoporosis induced pathological fractures. The care of spina bifida patients is a challenging one, requiring many health care professionals from different areas to be working in conjunction. Nowadays, spina bifida patients live longer due to advances in health care and improving the quality of life of these patients is paramount.","PeriodicalId":108199,"journal":{"name":"Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-11-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128350414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"New Technologies to Improve Surgical Outcome during Open-Cranial Vault Remodeling","authors":"D. García-Mato, J. Pascau, S. Ochandiano","doi":"10.5772/intechopen.94536","DOIUrl":"https://doi.org/10.5772/intechopen.94536","url":null,"abstract":"Current approaches for the surgical correction of craniosynostosis are highly dependent on surgeon experience. Therefore, outcomes are often inadequate, causing suboptimal esthetic results. Novel methods for cranial shape analysis based on statistical shape models enable accurate and objective diagnosis from preoperative 3D photographs or computed tomography scans. Moreover, advanced algorithms are now available to calculate a reference cranial shape for each patient from a multi-atlas of healthy cases, and to determine the most optimal approach to restore normal calvarial shape. During surgery, multiple technologies are available to ensure accurate translation of the preoperative virtual plan into the operating room. Patient-specific cutting guides and templates can be designed and manufactured to assist during osteotomy and remodeling. Then, intraoperative navigation and augmented reality visualization can provide real-time guidance during the placement and fixation of the remodeled bone. Finally, 3D photography enables intraoperative surgical outcome evaluation and postoperative patient follow-up. This chapter summarizes recent literature on all these technologies, showing how their integration into the surgical workflow could increase reproducibility and reduce inter-surgeon variability in open cranial vault remodeling procedures.","PeriodicalId":108199,"journal":{"name":"Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications","volume":"31 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131568548","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}