The significance of hindbrain herniation reversal following prenatal surgery for fetal neural tube defects (original) (raw)
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Management of Neural Tube Defect: A Single-Center Experience
2021
Objective: Neural tube defects are a heterogeneous and complex group of congenital central nervous system anomalies involving that involve the failed closure of the vertebral column. The spinal cord is frequently affected, and neural tube defects substantially increase the risk of disability and death within the first year of life. Neural malformations are often associated with abnormalities in other organ systems. This study presents our clinical experiences regarding the development and comorbidities of infants with neural tube defect and compares them with the literature. Materials and Methods: This retrospective study included 47 patients (24 females, 23 males) who underwent surgical treatment for neural tube defect in the neurosurgery department of Sivas Cumhuriyet University Faculty of Medicine Training and Research Hospital between 2000 and 2020. Results: Of the 47 patients, 34 (72.34%) had myelomeningocele and 13 (27.65%) had meningocele. Lesion location was thoracolumbar in...
Ultrasound in Obstetrics & Gynecology, 2019
Objective To determine if brain imaging in fetuses that underwent prenatal repair of neural tube defect (NTD) can predict the need for postnatal hydrocephalus treatment (HT) in the first year postpartum. Methods This was a retrospective study of fetuses diagnosed with open NTD that had in-utero myelomeningocele repair between April 2014 and April 2016. Independent variables were collected from four chronological sets of fetal images: presurgery ultrasound, presurgery magnetic resonance imaging (MRI), 6-week postsurgery MRI and predelivery ultrasound. The following independent variables were collected from all image sets unless otherwise noted: gestational age, head circumference, mean ventricular width, ventricular volume (MRI only), hindbrain herniation (HBH) score (MRI only), and level of lesion (LOL), defined as the upper bony spinal defect (presurgery ultrasound only). Based on these measurements, additional variables were defined and calculated including change in degree of HBH, ventricular width growth (mm/week) and ventricular volume growth (mL/week). The need for HT (by either ventriculoperitoneal shunt or endoscopic third ventriculostomy with choroid plexus cauterization) was determined by a pediatric neurosurgeon using clinical and radiographic criteria; a secondary analysis was performed using the MOMS trial criteria for hydrocephalus. The predictive value of each parameter was assessed by receiver-operating characteristics curve and logistic regression analyses. Results Fifty affected fetuses were included in the study, of which 32 underwent open hysterotomy
Demography and management outcome of neural tube defects in a Nigerian tertiary health institution
International Journal of Research in Medical Sciences, 2021
Background: Neural tube defects (NTD) are a group of congenital anomalies of the central nervous system (CNS). Its management is very challenging. A recognised leading cause is a folic acid deficiency, therefore prevented by taking a preconception folic acid. This study looked at the demographic features and management outcome of neural tube defect, a fairly common preventable condition with the need to raise awareness on its preventive measures. Methods: The study was conducted on 82 patients with NTD that were managed in federal medical centre Yola, in NorthEastern Nigeria. It was a retrospective study over a 4-year period, from January 2016 to December 2019. Results: Ages at presentations ranged from 1 to 93 days with a mode of 3 days. There were 29 males and 53 females with a male to female ratio of 1:1.8. Spina bifida constituted 74.4%, encephalocele (20%), Anencephaly (5%). Types of spina bifida managed were myelomeningocele (63.5%), meningocele (33.3%), and lipo-myelomeningocele (3.2%). Two had a 2-level meningocele. Syndromic associations of myelomeningocele were with hydrocephalus (78.1%), foot deformity (22.2%), cardiac (2.5%), and anorectal malformation (2.5%). Ninety-five-point one had various surgeries with 30.8% developing postoperative complications. commonest was post excision of myelomeningocele and encephalocele hydrocephalus (29.1%) in 7 patients. Post-operative Mortality was reported in 2 patients (8.3%). Conclusions: Myelomeningocele was the commonest NTD, and its syndromic association with hydrocephalus was common. A large number of patients had surgical interventions with a good outcome. Post-op mortality was minimal.
Taiwan Journal of Obstetrics and Gynecology, 2021
Objective: To modify the current neural tube defect (NTD) classification for fetal medicine specialists, and to investigate the impact of prenatal ultrasound conus medullaris position screening on the detection rate of closed spinal dysraphism and pregnancy outcomes. Materials and methods: The clinical data of 112 patients prenatally diagnosed with neural tube defects in Taiji clinic from 2008 to 2018 were retrospectively analyzed. All cases were classified following the modified classification. We compared the detection rate before and after introducing the conus medullaris screening and pregnancy outcomes for NTD types. Results: Closed spinal dysraphism type prevailed in our sample (43.8%). The median gestational age at the time of detection for cranial dysraphism was 13.3 weeks, open spinal dysraphism was 22.0 weeks, and closed spinal dysraphism was 22.6 weeks. All cranial dysraphism (n ¼ 43) and open spinal dysra-phism cases (n ¼ 20) had pregnancies terminated. For closed spinal dysraphism Class 1, the live-birth rate was 100.0% in the cases without other anomalies and 33.3% in the cases with other anomalies, respectively (X 2 ¼ 17.25, p < 0.001). Similarly, for Class 2, pregnancy continuation rate was 50.0% in cases without other anomalies and 20.0% in cases with other anomalies, yet it failed to reach statistical significance (X 2 ¼ 0.9, p ¼ 0.524). Conclusion: Our case series may help to improve early screening and prenatal diagnosis of NTDs. Modified classification is adjusted for use in ultrasound fetal care facilities, which could be used for predicting pregnancy outcome. We suggest promoting first-trimester anatomical screening in order to make an earlier diagnosis and therefore provide better prenatal care for open spinal dysraphism cases in the era of intrauterine repair. Our findings imply that the use of fetal conus medullaris position as a marker for closed spinal dysraphism improves the detection rate and would unlikely lead to a higher termination rate.
Surgical Treatment of Neural Tube Defects
Spina Bifida and Craniosynostosis - New Perspectives and Clinical Applications, 2021
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 prese...
Diagnostics
Spina bifida aperta (SBA), with (myelomeningocele) or without (myeloschisis) a hernia sack, is the most common congenital defect of the central nervous system. Prenatal surgical closure of SBA lowers the risk for developing shunt-dependent hydrocephalus, which offers a chance at improved motor, urinary, and gastrointestinal function. A total of 96 patients who had undergone open surgery prenatal repair for SBA were analyzed. The patients were divided into two groups: Group I—12 patients (12.5%)—without a hernia sack (myeloschisis) and Group II—84 patients (87.5%)—with a hernia sack (myelomeningocele). In this study, we demonstrated that prenatal SBA repair was statistically significantly less often associated with the need for ventriculoperitoneal shunting (p > 0.00001). The shunting was statistically significantly more often required in patients from Group I (p > 0.004). The absence of a hernia sack increases the risk for developing shunt-dependent hydrocephalus in patients a...
Introduction: Several congenital malformations affect developing fetuses, among which Neural tube defect (NTD) is most common. Folic acid supplementation brought decline in the incidence of NTDs. The present study aims at finding the incidence of NTDs in a tertiary care hospital and compares the results with the similar Indian studies published earlier. Materials and Methods: The study was done at Chettinad Hospital & Research Institute (CHRI), Kelambakkam. The total number of deliveries was recorded for a period of five years from 2009 to 2013. Fetuses which were still born with neural defect were collected and observed in detail externally for the sex, type of NTD and other associated anomalies. Indian studies published between 1987 and 2014 reporting the incidence of NTDs among the births occurred were retrieved from the Internet and their various observations were used for comparison. R esults: The number of deliveries conducted between 2009 and 2013 at CHRI was 3220. Of these, babies born with NTDs were nine (5 males and 4 females). The incidence of fetuses with meroanencephaly, holoanencephaly, craniorachischisis, encephalocele and myelocele were 0.62, 0.62, 0.93, 0.31 and 0.31 per 1000 births respectively. Overall incidence of NTDs in the present study was 2.79/1000 births. Fetuses with NTDs also had the following anomalies – Club foot, cleft lip and palate and exomphalos. C onclusion: Comparing the results with the previous studies it is clearly evident that the incidence of NTDs have significantly reduced from 11.42/1000 births to 2.79/1000 births. In most of the previous studies NTDs had a female preponderance whereas present study has a male preponderance.In older studies, spina bifida was the most common NTDs followed by anencephaly. But in the present study anencephaly was the common NTD than spina bifida. Incidence of NTDs has reduced due to various reasons like prenatal screening for fetal anomalies and folic acid supplementation.
American Journal of Obstetrics and Gynecology, 2000
The purpose of our study was to determine whether prenatal repair of myelomeningocele prevents or reverses hindbrain herniation in the sheep model. STUDY DESIGN: A myelomeningocele was surgically created in fetal sheep. One group was repaired later in utero; the others were delivered without repair. After delivery, lambs were assessed for the presence of hindbrain herniation. RESULTS: In all lambs that had not undergone repair of the myelomeningocele, severe hindbrain herniation developed, whereas the brains of all lambs that had undergone fetal repair were normal. CONCLUSION: Prenatal repair of myelomeningocele prevents or reverses development of hindbrain herniation in the fetal lamb model.