scholarly journals Management of Pediatric Patients with Spina Bifida

2021 ◽  
Author(s):  
Romana Richterová ◽  
Branislav Kolarovszki ◽  
René Opšenák
Keyword(s):  
Spina Bifida ◽  
Neural Tube ◽  
Neurodevelopmental Disorder ◽  
Community Support ◽  
Primary Care Doctor ◽  
Substantial Part ◽  
General Management ◽  
Vertebral Arch ◽  
Open Spina Bifida

Spina bifida is a neurodevelopmental disorder and belong to most common congenital malformations. It is a neural tube defect that originates within first 28 days after conception. Although survival rate of these patients had changed rapidly within last decades, neural tube defects are still cause of substantial part of children morbidity. Occult type of spina bifida is a simple nonunion of vertebral arch without causing any symptoms. Open defects according to degree of involvement of neural tissue cause symptoms such as paralysis of lower extremities, bladder and bowel morbidity, delay in development of cognitive functions of various severity and other possible complications affecting morbidity of these patients. Early diagnosis and treatment of open spina bifida and accompaying complications is crucial and largely affects the outome. Succesfull treatment requires lifelong cooperation of a whole range of specialists and guiding of treatment by primary care doctor. Survival and quality of life are associated with acces to proper medical and surgical treatment as well as community support systems. This chapter offers overview of this topic with emphasis on general management of patients suffering from this congenital malformation.

Myelomeningocele

2018 ◽  
Author(s):  
Joanne Spaliaras
Keyword(s):  
Spina Bifida ◽  
Neural Tube ◽  
Anesthetic Management ◽  
Spinal Column ◽  
Term Survival ◽  
Operative Repair ◽  
Associated Conditions ◽  
Vertebral Arch ◽  
Open Spina Bifida

Spina bifida is a defect in which the vertebral arch of the spinal column is either incompletely formed or absent. Failure of closure of the neural tube during the third week of gestation leads to the constellation of defects observed in patients with meningomyelocele or open spina bifida. Myelomeningocele is the most common neural tube defect and the most severe birth defect compatible with long-term survival. It is associated with several characteristic central nervous system anomalies. Leak of cerebrospinal fluid (CSF) is commonly observed. The major indication for early operative repair (within 48 hours of delivery) is prevention of infection. Protection of the exposed neural tissue from trauma and drying is essential. An understanding of the pathophysiology and associated conditions of myelomeningocele helps guide anesthetic management of these patients.

Concanavalin A binding of alpha-fetoprotein in amniotic fluid as an aid in the diagnosis of neural tube defects.

1981 ◽  
Vol 27 (10) ◽  
pp. 1658-1660 ◽  
Author(s):  
P K Buamah ◽  
P Taylor ◽  
A M Ward
Keyword(s):  
Spina Bifida ◽  
Amniotic Fluid ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Concanavalin A ◽  
Gestational Age ◽  
Alpha Fetoprotein ◽  
Normal Outcome ◽  
Fetal Malformations ◽  
Open Spina Bifida

Abstract Concanavalin A nonreactive alpha-fetoprotein was determined in samples of amniotic fluid from 16 abnormal pregnancies complicated by anencephaly (7), open spina bifida (6), intra-uterine death (1), anencephaly with exomphalos (1), or open spina bifida with exomphalos (1), and in amniotic fluid from 50 normal pregnancies with gestational age between 13 and 24 weeks. In all 16 cases with fetal malformations, the proportion of nonreactive alpha-fetoprotein was significantly decreased (median 5.3%) as compared with amniotic fluid from pregnancies with a normal outcome (median 39.7%). The results confirm that this measurement is useful in the diagnosis of neural tube defects, especially when the concentration of alpha-fetoprotein in amniotic fluid is normal or only slightly above normal and gestational age is uncertain.

scholarly journals Surgical Treatment of Neural Tube Defects

2021 ◽  
Author(s):  
Juraj Šutovský
Keyword(s):  
Quality Of Life ◽  
Spinal Cord ◽  
Spina Bifida ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Neural Tissue ◽  
Prenatal Development ◽  
Nerve Roots ◽  
Live Births

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.

Prenatal Diagnosis and Patient Preferences in Patients with Neural Tube Defects around the Advent of Fetal Surgery in Belgium and Holland

2014 ◽  
Vol 37 (3) ◽  
pp. 226-234 ◽  
Author(s):  
Céline Ovaere ◽  
Alex Eggink ◽  
Jute Richter ◽  
Titia E. Cohen-Overbeek ◽  
Frank Van Calenbergh ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Spina Bifida ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Fetal Surgery ◽  
Training Material ◽  
Spinal Lesions ◽  
Spina Bifida Aperta ◽  
Prenatal Management ◽  
Open Spina Bifida

Introduction: We review the characteristics and prenatal choices of patients recently evaluated for neural tube defects (NTD) at two tertiary units. The prenatal diagnosis of NTD allows parents to consider all prenatal options. In selected cases of spina bifida aperta this also includes fetal surgery, which we started offering after combined ‘in-house' and ‘exported' training. Material and Methods: This is a retrospective review of prospectively collected data on NTD diagnosed over the last 8 years and recent fetal surgery referrals. Results: A total of 167 patients were referred for assessment at a median of 19 weeks. Cranial lesions were diagnosed significantly earlier than spinal lesions. Of the open spinal lesions, 77% were isolated. Of these, 22% were managed expectantly and 1 (1%) had fetal surgery. There was no correlation between parental decisions on prenatal management with disease-specific severity markers. We had 14 fetal surgery referrals, all but 1 from beyond our typical referral area; 6 of the assessed patients were operated on, 4 were expectantly managed and 4 requested termination of pregnancy (TOP). These pregnancy outcomes were in the expected range. Discussion: Open spina bifida is mainly diagnosed in the second trimester and 76% of subjects request TOP, irrespective of the severity indicators. The number of local patients considering fetal surgery is low.

scholarly journals Neurone Specific Enolase in Amniotic Fluid: A Potential Marker of Anencephaly

1988 ◽  
Vol 25 (1) ◽  
pp. 85-88 ◽  
Author(s):  
K Spencer ◽  
P Carpenter
Keyword(s):  
Prenatal Diagnosis ◽  
Spina Bifida ◽  
Amniotic Fluid ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Normal Values ◽  
Potential Marker ◽  
Neuronal Proteins ◽  
Non Gaussian ◽  
Open Spina Bifida

Normal values for neurone specific enolase in amniotic fluid have been found to follow a non gaussian distribution with a 1–99 centile range of 1·10–4·32 μg/L. Neurone specific enolase levels have been shown to be raised in the amniotic fluid of pregnancies complicated by anencephaly, although not those complicated by open spina bifida. Neurone specific enolase measured by radioimmunoassay is capable of totally discriminating between normal pregnancies and those complicated by anencephaly. The study demonstrates the possible value of investigating other neuronal proteins which may find value as adjuncts to amniotic fluid Alpha fetoprotein levels in the prenatal diagnosis of Neural Tube Defects.

scholarly journals Novel mouse model of encephalocele: post-neurulation origin and relationship to open neural tube defects

2019 ◽  
Author(s):  
Ana Rolo ◽  
Gabriel L. Galea ◽  
Dawn Savery ◽  
Nicholas D. E. Greene ◽  
Andrew J. Copp
Keyword(s):  
Spina Bifida ◽  
Mouse Model ◽  
Neural Tube ◽  
Neural Tube Defects ◽  
Small Gtpase ◽  
Late Gestation ◽  
Neural Tube Closure ◽  
Surface Ectoderm ◽  
Open Spina Bifida ◽  
Tube Closure

ABSTRACTEncephalocele is a clinically important birth defect that can lead to severe disability in childhood and beyond. The embryonic pathogenesis of encephalocele is poorly understood and, while usually classified as a ‘neural tube defect’, there is conflicting evidence on whether encephalocele results from defective neural tube closure, or is a post-neurulation defect. It is also unclear whether encephalocele can result from the same causative factors as anencephaly and open spina bifida, or whether it is aetiologically distinct. This lack of information results largely from the scarce availability of animal models of encephalocele, particularly ones that resemble the commonest, non-syndromic human defects. Here we report a novel mouse model of occipito-parietal encephalocele, in which the small GTPase Rac1 is conditionally ablated in the (non-neural) surface ectoderm. Most mutant fetuses have open spina bifida, and some also exhibit exencephaly/anencephaly. However, a large proportion of mutant fetuses exhibit encephalocele affecting the occipito-parietal region. The encephalocele phenotype does not result from a defect in neural tube closure, but rather from a later disruption of the surface ectoderm covering the already closed neural tube, allowing the brain to herniate. The neuroepithelium itself shows no down-regulation of Rac1 and appears morphologically normal until late gestation. A large skull defect develops overlying the region of brain herniation. Our work provides a new genetic model of occipito-parietal encephalocele, particularly resembling non-syndromic human cases. While encephalocele has a different, later-arising pathogenesis than open neural tube defects, both can share the same genetic causation.SUMMARY STATEMENTEncephalocele - a severe brain defect - arises after neural tube closure, but can share a common genetic cause with anencephaly, a defect of neural tube closure.

Penerapan Proses Keperawatan di Rumah Sakit dalam Meningkatkan Mutu Keperawatan

2020 ◽  
Author(s):  
Kristin Natal Riang Gea
Keyword(s):  
Quality Of Service ◽  
Nursing Care ◽  
Appropriate Technology ◽  
Nursing Process ◽  
General Management ◽  
Management Concepts ◽  
Optimal Health ◽  
Service Standards ◽  
Management Of Care

AbstrakManajemen asuhan keperawatan merupakan suatu proses keperawatan yang menggunakan konsep manajemen secara umum didalamnya seperti perencanaan, pengorganisasian, pengarahan dan pengendalian atau evaluasi. Peningkatan mutu pelayanan adalah derajat memberikan pelayanan secara efisien dan efektif sesuai dengan standar profesi, standar pelayanan yang dilaksanakan secara menyeluruh sesuai dengan kebutuhan pasien, memanfaatkan teknologi tepat guna dan hasil penelitian dalam pengembangan pelayanan kesehatan/ keperawatan sehingga tercapai derajat kesehatan yang optimal. Kualitas pelayanan keperawatan di rumah sakit tidak akan berjalan dengan baik apabila proses keperawatan yang dilaksanakan tidak terstruktur dengan baikKata Kunci : Manajemen Keperawatan, Kualitas Pelayanan,.standar proses keperawatanAbstract Nursing care management is a nursing process that uses general management concepts in it such as planning, organizing, directing and controlling or evaluation. Improving the quality of service is the degree of providing services in an efficient and effective in accordance with professional standards, service standards are implemented thoroughly in accordance with the needs of patients, utilizing appropriate technology and research results in the development of health services / nursing to achieve optimal health. The quality of nursing care in the hospital will not run properly if the nursing process does not properly implemented.Keywords: Management of Care of Nursing, Quality of Service, standard nursing process,

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