Open Spina Bifida

开放性脊柱裂
  • 文章类型: Journal Article
    背景:脊髓膨出是一种罕见的开放性脊柱裂。建议在产前或前48小时进行手术修复。在某些情况下,修复可能会延迟,和特定的手术因素需要考虑。
    方法:我们简要概述了外科解剖学,随后描述了一名11个月大儿童的胸腰椎髓囊肿的手术修复。
    结论:脊髓膨出的手术修复可以稳定神经状态,预防局部和中枢神经系统感染。对脊髓膨出解剖结构的了解可以在保留尽可能多的健康组织并恢复正常解剖结构的同时将其去除。
    BACKGROUND: Myelocele is a rare form of open spina bifida. Surgical repair is recommended prenatally or in the first 48 h. In some cases, the repair may be delayed, and specific surgical factors need to be considered.
    METHODS: We give a brief overview of the surgical anatomy, followed by a description of the surgical repair of a thoracolumbar Myelocele in an 11-month-old child.
    CONCLUSIONS: Surgical repair of the Myelocele stabilizes the neurological status, prevents local and central nervous system infections. The understanding of Myelocele anatomy enables its removal while preserving as much healthy tissue as possible and restoring normal anatomy.
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  • 文章类型: Journal Article
    目的:手术机器人倾向于开发认知控制架构,以提供一定程度的自主性,以提高患者安全性和手术效果,同时减少所需的外科医生的认知负荷致力于低级决策。认知需要工作空间感知,这是实现自动决策和任务计划能力的重要一步。在微创手术中,强大而准确的检测和跟踪受到可见性有限的影响,闭塞,解剖变形和相机运动。
    方法:本文开发了一种鲁棒的方法来实时检测和跟踪解剖结构,以用于机器人系统的自动控制和增强现实。这项工作的重点是在极具挑战性的手术实验验证:开放脊柱裂的胎儿镜修复。所提出的方法基于两个顺序步骤:首先,使用卷积神经网络选择相关点(轮廓),第二,通过可变形的几何图元重建解剖形状。
    结果:用不同的方案验证了方法性能。综合场景测试,专为极端验证条件而设计,证明该方法在手术过程中相对于标称条件提供的安全裕度。真实场景实验证明了该方法在准确性方面的有效性,鲁棒性和计算效率。
    结论:本文提出了一种针对摄像机突然运动的强大解剖结构检测,严重闭塞和变形。尽管论文的重点是案例研究,打开脊柱裂,该方法适用于所有可以通过几何图元近似轮廓的解剖结构。该方法旨在为需要精确跟踪敏感解剖结构的认知机器人控制和增强现实系统提供有效的输入。
    OBJECTIVE: Surgical robotics tends to develop cognitive control architectures to provide certain degree of autonomy to improve patient safety and surgery outcomes, while decreasing the required surgeons\' cognitive load dedicated to low level decisions. Cognition needs workspace perception, which is an essential step towards automatic decision-making and task planning capabilities. Robust and accurate detection and tracking in minimally invasive surgery suffers from limited visibility, occlusions, anatomy deformations and camera movements.
    METHODS: This paper develops a robust methodology to detect and track anatomical structures in real time to be used in automatic control of robotic systems and augmented reality. The work focuses on the experimental validation in highly challenging surgery: fetoscopic repair of Open Spina Bifida. The proposed method is based on two sequential steps: first, selection of relevant points (contour) using a Convolutional Neural Network and, second, reconstruction of the anatomical shape by means of deformable geometric primitives.
    RESULTS: The methodology performance was validated with different scenarios. Synthetic scenario tests, designed for extreme validation conditions, demonstrate the safety margin offered by the methodology with respect to the nominal conditions during surgery. Real scenario experiments have demonstrated the validity of the method in terms of accuracy, robustness and computational efficiency.
    CONCLUSIONS: This paper presents a robust anatomical structure detection in present of abrupt camera movements, severe occlusions and deformations. Even though the paper focuses on a case study, Open Spina Bifida, the methodology is applicable in all anatomies which contours can be approximated by geometric primitives. The methodology is designed to provide effective inputs to cognitive robotic control and augmented reality systems that require accurate tracking of sensitive anatomies.
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  • 文章类型: Journal Article
    背景:胎儿手术治疗开放性脊柱裂(OSB)需要使用影像学进行全面的术前评估,以选择合适的患者,并评估术后疗效和并发症。我们探索了胎儿磁共振成像(MRI)的患者进入和进行,以对符合胎儿手术资格的OSB患者进行产前评估。我们将影像采集和报告与国际妇产科超声学会MRI性能指南进行了比较。
    方法:我们调查了英国和爱尔兰的OSB在转诊胎儿医学单位(FMU)时使用胎儿MRI的情况,和两个NHS英格兰专家在伦敦大学学院医院委托的胎儿手术中心(FSC),和比利时鲁汶大学医院。为了研究MRI采集协议,我们回顾性分析了胎儿OSB手术前后的胎儿MRI图像。
    结果:胎儿OSB的MRI可由适当的专家监督,执行,并报告扫描。从请求开始安排胎儿MRI预约的平均时间为4±3天(范围,0-10),可用的平均扫描时间为37±16分钟(范围,20-80分钟),15±11分钟(范围,0-30分钟)根据需要重复序列的额外时间。特定的MRI采集协议,MRI报告模板仅有32%和18%的单位可用,分别。所有中心术前均在三个正交平面中获得满意的T2加权(T2W)脑成像,术后6周,96%的FSC和78%的转诊FMU。然而,对于T2W脊柱图像采集,参考FMU不太能够提供三个正交平面的手术前手术(98%FSC与50%FMU,p<0.001),手术后6周(100%FSCvs.48%FMU,p<0.001)。其他标准成像建议,如T1加权(T1W),与手术前和手术后的FMU相比,FSCs中一个或两个正交平面的梯度回波(GE)或超声平面胎儿脑和脊柱成像更有可能(p<0.001).
    结论:可以及时获得有监督的MRI来评估OSB胎儿手术。然而,在足够的正交平面中提供胎儿大脑和脊柱的图像,这是确定资格和确定胎儿手术后脑疝的逆转所必需的,不太经常被收购。我们的证据表明,需要对OSB的胎儿MRI进行具体指导。我们提出了MRI采集和报告的示例指导。
    BACKGROUND: Fetal surgery for open spina bifida (OSB) requires comprehensive preoperative assessment using imaging for appropriate patient selection and to evaluate postoperative efficacy and complications. We explored patient access and conduct of fetal magnetic resonance imaging (MRI) for prenatal assessment of OSB patients eligible for fetal surgery. We compared imaging acquisition and reporting to the International Society of Ultrasound in Obstetrics and Gynecology MRI performance guidelines.
    METHODS: We surveyed access to fetal MRI for OSB in referring fetal medicine units (FMUs) in the UK and Ireland, and two NHS England specialist commissioned fetal surgery centers (FSCs) at University College London Hospital, and University Hospitals KU Leuven Belgium. To study MRI acquisition protocols, we retrospectively analyzed fetal MRI images before and after fetal surgery for OSB.
    RESULTS: MRI for fetal OSB was accessible with appropriate specialists available to supervise, perform, and report scans. The average time to arrange a fetal MRI appointment from request was 4 ± 3 days (range, 0-10), the average scan time available was 37 ± 16 min (range, 20-80 min), with 15 ± 11 min (range, 0-30 min) extra time to repeat sequences as required. Specific MRI acquisition protocols, and MRI reporting templates were available in only 32% and 18% of units, respectively. Satisfactory T2-weighted (T2W) brain imaging acquired in three orthogonal planes was achieved preoperatively in all centers, and 6 weeks postoperatively in 96% of FSCs and 78% of referring FMUs. However, for T2W spine image acquisition referring FMUs were less able to provide three orthogonal planes presurgery (98% FSC vs. 50% FMU, p < 0.001), and 6 weeks post-surgery (100% FSC vs. 48% FMU, p < 0.001). Other standard imaging recommendations such as T1-weighted (T1W), gradient echo (GE) or echoplanar fetal brain and spine imaging in one or two orthogonal planes were more likely available in FSCs compared to FMUs pre- and post-surgery (p < 0.001).
    CONCLUSIONS: There was timely access to supervised MRI for OSB fetal surgery assessment. However, the provision of images of the fetal brain and spine in sufficient orthogonal planes, which are required for determining eligibility and to determine the reversal of hindbrain herniation after fetal surgery, were less frequently acquired. Our evidence suggests the need for specific guidance in relation to fetal MRI for OSB. We propose an example guidance for MRI acquisition and reporting.
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  • 文章类型: Journal Article
    目的:为了确定开放性脊柱裂胎儿的下肢运动功能是否在妊娠22周时的首次产前运动评估和妊娠后期(妊娠26-27周)的第二次术前评估之间的四周内恶化,并将产前和产后运动功能与病变的解剖水平进行比较。
    方法:多中心队列研究,涉及两个中心进行开放性脊柱裂(SAFER技术)的经皮胎儿镜修复的病例。94例开放性脊柱裂的胎儿在胎儿手术前进行了两次产前超声检查,间隔大约四周。出生后两个月内进行一次产后评估。根据从L1到S1的关键肌肉功能进行运动功能分类。在第一次就诊时通过超声系统地进行产前评估,第二次评估在胎儿镜修复之前进行。在出生后的头两个月内通过体格检查进行产后评估。每条腿都进行了单独分析;如果两条腿之间存在差异,最差的水平被考虑进行分析。将所有运动水平评估与解剖水平进行比较。使用逻辑回归模型评估了产后运动功能降低的独立因素。
    结果:在妊娠22.5周(20.7-24.3)和26.9周(25.4-27.3)时评估了产前运动水平,中位间隔为4周(2.4-6.0周)。手术的中位胎龄为27周(25.6-27.6周)。两次产前评估之间的运动功能没有显着差异(p=0.861)。所有产前和产后运动功能评估均与解剖水平显着不同(第1术前评估,p=0.0023;第二次术前评估,p=0.0015;产后评估,p=0.0333)。比较产前和产后运动功能,我们发现,与手术前水平相比,近90%的婴儿维持或改善了运动功能.在逻辑回归中,发现较低的解剖水平和较高的解剖和运动水平差异是出生后运动功能的独立因素(OR分别为0.237,p=0.002;和OR3.44,p<0.001).
    结论:首次评估和“晚期”胎儿手术修复之间的4周间隔没有显着改变运动功能,这表明后期修复>26周不会对运动结果产生负面影响。在进行开放性脊柱裂的产前修复的病例中,与病变的解剖水平相比,通过超声进行的术前神经运动功能评估可以更好地预测出生后的运动功能,应包括在术前咨询中。本文受版权保护。保留所有权利。
    To determine if the lower-extremity neurological motor function level in fetuses with open spina bifida deteriorates within the 4-week interval between a first prenatal motor assessment at around 22 weeks of gestation and a second evaluation, prior to \'late\' prenatal surgery, defined as surgery at 26-28 weeks and, in certain situations, up to 30 weeks, and to assess the association between prenatal presurgical motor-function level, anatomical level of the lesion and postnatal motor-function level.
    This was a two-center cohort study of 94 singleton fetuses with open spina bifida which underwent percutaneous repair using the skin-over-biocellulose for antenatal fetoscopic repair (SAFER) technique between December 2016 and January 2022. All women underwent two prenatal systematic ultrasound evaluations, approximately 4 weeks apart, with the second one being performed less than 1 week before surgery, and one postnatal evaluation via physical examination within 2 months of birth. Motor-function classification was from spinal level T12 to S1, according to key muscle function. Each leg was analyzed separately; in case of discrepancy between the two legs, the worst motor-function level was considered for analysis. Motor-function-level evaluations were compared with each other and with the anatomical level as observed on ultrasound. Independent predictors of a postnatal reduction in motor-function level were assessed using a logistic regression model.
    Prenatal motor-function level was assessed at a median gestational age of 22.5 (interquartile range (IQR), 20.7-24.3) and 26.7 (IQR, 25.4-27.3) weeks, with a median interval of 4.0 (IQR, 2.4-6.0) weeks. The median gestational age at surgery was 27.0 (IQR, 25.9-27.6) weeks and the postnatal examination was at median age of 0.8 (IQR, 0.3-5.4) months. There was no significant difference in motor-function level between the two prenatal evaluations (P = 0.861). We therefore decided to use the second prenatal evaluation for comparison with postnatal motor function and anatomical level. Overall, prenatal and postnatal motor function evaluations were significantly different from the anatomical level (preoperative assessment, P = 0.0015; postnatal assessment, P = 0.0333). Comparing prenatal with postnatal motor-function level, we found that 87.2% of babies had similar or improved motor function compared with that prior to prenatal surgery. On logistic regression analysis, lower anatomical level of defect and greater difference between anatomical level and prenatal motor-function level were identified as independent predictors of postnatal motor function (odds ratio, 0.237 (95% CI, 0.095-0.588) (P = 0.002) and 3.44 (95% CI, 1.738-6.813) (P < 0.001), respectively).
    During a 4-week interval between first ultrasound evaluation and late fetal surgical repair of open spina bifida, motor function does not change significantly, suggesting that late repair, ≥ 26 weeks, does not impact negatively on motor-function outcome. Compared with the anatomical level of the lesion, preoperative neurological motor-function assessment via ultrasound is more predictive of postnatal motor function, and should be included in preoperative counseling. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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  • 文章类型: Case Reports
    背景:开放性脊柱裂在动物中是一种罕见的畸形,缺乏成像,临床,和狗这种情况的病理特征。
    目的:由于高水平的围产期死亡率和频繁的安乐死,在动物中很少观察到开放性脊柱裂。据我们所知,我们介绍了第一例脊柱裂的狗在子宫内被诊断,然后在产后随访。
    方法:一只3岁的贵宾犬双胎妊娠。X线和超声检查结果提示一个胎儿椎体畸形和开放性脊柱裂伴脊髓膜膨出。给予保守治疗,但小狗出生后3天死亡。此后,进行了几个器官的解剖和组织病理学分析,以描述疾病的特征。
    结果:双胞胎出生时,一只小狗具有从椎骨L2-L6水平延伸的线性背侧中线皮肤缺损。RX线检查显示几种涉及胸段的先天性椎骨畸形,腰段,骶骨和肩胛骨.组织病理学检查证实了开放性脊柱裂的存在,并在几个内脏器官中发现了其他异常。
    结论:此病例表现为开放性脊柱裂的完整特征,出生前和死亡后,使用成像和组织病理学技术。
    Open spina bifida is an uncommon malformation in animals, and there is a lack of imaging, clinical, and pathological characterisation of this condition in dogs.
    Open spina bifida is rarely observed in animals due to high levels of perinatal mortality and frequent euthanasia. To the best of our knowledge, we present the first case of spina bifida in a dog was diagnosed in-utero and then followed post-partum.
    A 3-year-old Poodle was presented with twin pregnancy. Radiographic and ultrasonographic findings were suggestive of vertebral malformation and open spina bifida with myelomeningocele in one foetus. Conservative treatment was given but the puppy died 3 days after birth. Thereafter, anatomical and histopathological analysis of several organs was performed to characterise the disease.
    When the twins were born, one puppy had a linear dorsal midline cutaneous defect extending from the level of vertebrae L2-L6. R Radiographic examination showed several congenital vertebral malformations involving the thoracic segment, lumbar segment, sacrum and scapula. Histopathological examinations confirmed the presence of open spina bifida and identified additional abnormalities in several internal organs.
    This case presents a complete characterisation of open spina bifida, before birth and after death, using imaging and histopathology techniques.
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  • 文章类型: Journal Article
    目的:为了测量在转诊时和术后6周时,在转诊到大型胎儿中心进行开放性神经管缺损(ONTD)修复的胎儿的脑成像中发现穿孔的透明隔(CSP)的相关性,最终需要在1岁时进行脑积水治疗。子宫内ONTD修复可降低1岁时需要治疗的严重脑积水的风险。其他研究表明,子宫内修复前严重的胎儿侧脑室肥大(>15mm)的存在显着增加了需要产后治疗脑积水的风险。在胎儿成像中遇到CSP(pCSP)的穿孔,并且可以反映胎儿大脑中脑室内压力的增加。我们试图评估在子宫内ONTD修复之前或之后在胎儿影像学上看到的pCSP与最终需要脑积水治疗的关联。
    方法:回顾性队列研究,在2014年至2021年期间在一个中心进行了110例剖腹手术辅助胎儿镜ONTD修复的患者。手术的资格标准基于MOMS试验标准,尽管允许母亲BMI高达40kg/m2。在初次转诊时和术后6周用超声和MRI评估胎儿脑成像。使用存储的US和MRI扫描进行回顾性审查以评估CSP完整性。通过病历审查获得了有关需要在12个月内进行脑积水治疗的信息。适当地使用参数测试和非参数测试来比较转诊时通过超声评估的pCSP与完整CSP病例之间的结果。进行Logistic回归分析以评估脑积水治疗需要的预测值。
    结果:通过US和MRI进行术前pCSP,分别确定了20.6%和21.8%的病例,分别,术后US和MRI分别为26.6%和24.2%,分别。手术后心室大小增加[术前:11(5.89-21.45)mmvs.术后:16(7-43.5)mm;p<0.01]以及严重脑室增宽的病例比例[12.7%vs57.8%;p<0.01]。纳入转诊时由US进行完整CSP评估的107例病例进行比较:pCSP22例,完整CSP85例。pCSP组有较大的心室(14.32+3.45mmvs.10.37+2.37毫米);p<0.01)和更严重的脑室增宽(40.9%vs.5.9%;p<0.01)。术后6周观察到相同的关联[心室大小:21毫米(13-43.5毫米)与14.25毫米(7-29毫米);p<0.01],和[严重的脑室肥大:95%vs.46.8%;p<0.01]。pCSP患者术后后脑疝(HBH)逆转率较低(65%vs.88.6%;p=0.01)。与没有该特征的患者相比,pCSP患者需要更频繁地治疗脑积水[17/19(89.5%)与17/75(22.7%);p<0.01]。该结果的最强预测指标是缺乏HBH逆转(OR36.2,95%CI5.96-219.12;p<0.01),然后是转诊时的pCSP(OR23.4,95%CI5.42-100.98,p<0.01)和6周后的pCSP(OR19.48,95%CI5.68-66.68,p<0.01)。
    结论:在患有ONTD的胎儿中检测到pCSP可以可靠地识别那些在1岁时需要脑积水治疗的风险最高的病例。对这种大脑结构的评估可以极大地改善我们对考虑进行ONTD胎儿手术的家庭的咨询,以设定对产后结局的期望。本文受版权保护。保留所有权利。
    In-utero repair of an open neural tube defect (ONTD) reduces the risk of developing severe hydrocephalus postnatally. Perforation of the cavum septi pellucidi (CSP) may reflect increased intraventricular pressure in the fetal brain. We sought to evaluate the association of perforated CSP visualized on fetal imaging before and/or after in-utero ONTD repair with the eventual need for hydrocephalus treatment by 1 year of age.
    This was a retrospective cohort study of consecutive patients who underwent laparotomy-assisted fetoscopic ONTD repair between 2014 and 2021 at a single center. Eligibility criteria for surgery were based on those of the Management of Myelomeningocele Study (MOMS), although a maternal prepregnancy body mass index of up to 40 kg/m2 was allowed. Fetal brain imaging was performed with ultrasound and magnetic resonance imaging (MRI) at referral and 6 weeks postoperatively. Stored ultrasound and MRI scans were reviewed retrospectively to assess CSP integrity. Medical records were reviewed to determine whether hydrocephalus treatment was needed within 1 year of age. Parametric and non-parametric tests were used as appropriate to compare outcomes between cases with perforated CSP and those with intact CSP as determined on ultrasound at referral. Logistic regression analysis was performed to assess the predictive performance of various imaging markers for the need for hydrocephalus treatment.
    A total of 110 patients were included. Perforated CSP was identified in 20.6% and 22.6% of cases on preoperative ultrasound and MRI, respectively, and in 26.6% and 24.2% on postoperative ultrasound and MRI, respectively. Ventricular size increased between referral and after surgery (median, 11.00 (range, 5.89-21.45) mm vs 16.00 (range, 7.00-43.5) mm; P < 0.01), as did the proportion of cases with severe ventriculomegaly (ventricular width ≥ 15 mm) (12.7% vs 57.8%; P < 0.01). Complete CSP evaluation was achieved on preoperative ultrasound in 107 cases, of which 22 had a perforated CSP and 85 had an intact CSP. The perforated-CSP group presented with larger ventricles (mean, 14.32 ± 3.45 mm vs 10.37 ± 2.37 mm; P < 0.01) and a higher rate of severe ventriculomegaly (40.9% vs 5.9%; P < 0.01) compared to those with an intact CSP. The same trends were observed at 6 weeks postoperatively for mean ventricular size (median, 21.0 (range, 13.0-43.5) mm vs 14.3 (range, 7.0-29.0) mm; P < 0.01) and severe ventriculomegaly (95.0% vs 46.8%; P < 0.01). Cases with a perforated CSP at referral had a lower rate of hindbrain herniation (HBH) reversal postoperatively (65.0% vs 88.6%; P = 0.01) and were more likely to require treatment for hydrocephalus (89.5% vs 22.7%; P < 0.01). The strongest predictor of the need for hydrocephalus treatment within 1 year of age was lack of HBH reversal on MRI (odds ratio (OR), 36.20 (95% CI, 5.96-219.12); P < 0.01) followed by perforated CSP on ultrasound at referral (OR, 23.40 (95% CI, 5.42-100.98); P < 0.01) and by perforated CSP at 6-week postoperative ultrasound (OR, 19.48 (95% CI, 5.68-66.68); P < 0.01).
    The detection of a perforated CSP in fetuses with ONTD can reliably identify those cases at highest risk for needing hydrocephalus treatment by 1 year of age. Evaluation of this brain structure can improve counseling of families considering fetal surgery for ONTD, in order to set appropriate expectations about postnatal outcome. © 2023 International Society of Ultrasound in Obstetrics and Gynecology.
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  • 文章类型: Journal Article
    开放性脊柱裂(OSB)是一种先天性,具有多因素病因的非致命性畸形。经过准确的产前诊断和跨学科咨询,可以在一定条件下为父母提供胎儿治疗。自从产前OSB手术出现以来,原始手术技术的各种修改已经发展起来,包括剖腹手术辅助胎儿镜修复。经过两年的准备,吉森大学和马尔堡大学(UKGM)的团队成为第一个提供三端口的中心,在德语区通过剖腹辅助方法对OSB进行三层胎儿镜修复。我们指出,在经验丰富的中心的指导下,通过密集的多学科准备和培训,可以将先前描述和应用的技术转移到不同的设置。
    Open spina bifida (OSB) is a congenital, non-lethal malformation with multifactorial etiology. Fetal therapy can be offered under certain conditions to parents after accurate prenatal diagnostic and interdisciplinary counseling. Since the advent of prenatal OSB surgery, various modifications of the original surgical techniques have evolved, including laparotomy-assisted fetoscopic repair. After a two-year preparation time, the team at the University of Giessen and Marburg (UKGM) became the first center to provide a three-port, three-layer fetoscopic repair of OSB via a laparotomy-assisted approach in the German-speaking area. We point out that under the guidance of experienced centers and by intensive multidisciplinary preparation and training, a previously described and applied technique could be transferred to a different setting.
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  • 文章类型: Journal Article
    目的:调查call体(CC)的高度,以描述患有脑膜脊髓膨出(MMC)的胎儿的call体异常,并将这些发现与健康胎儿的call体进行比较。
    方法:在本研究中,对44例胎儿MMC畸形进行了胎儿MRI检查。作为对照组,34例胎儿MRI检查,在解剖学上是正常的,进行回顾性评估。在研究小组中,侧脑室直径,MMC缺陷的水平和直径,测量CC高度。在对照组中,测量CC高度和侧脑室直径。
    结果:研究组的平均CC身高为1.36mm,对照组为2.48mm。与对照人群相比,研究人群的CC身体区域的高度倾向于更薄(p<0.001)。
    结论:与正常胎儿相比,在MMC的胎儿MRI中发现CC体区的高度较薄,这表明各种call骨异常是不确定的,对其他call骨异常的调查可能有助于决定继续怀孕,以及在患有MMC的情况下终止或宫内手术。需要进一步的大型病例组研究。
    OBJECTIVE: To investigate height of the corpus callosum (CC) in order to describe the corpus callosum anomalies in fetuses with meningomyelocele (MMC) and compare these findings with the corpus callosum of healthy fetuses.
    METHODS: In this study, fetal MRI examinations were performed on 44 fetal MMC malformation cases. As the control group, 34 fetal MRI examinations, which were anatomically normal, were evaluated retrospectively. In the study group, lateral ventricle diameter, the level and diameter of the MMC defect, and CC height were measured. In the control group, CC height and lateral ventricular diameter were measured.
    RESULTS: The mean CC body height was 1.36 mm in the study group, and 2.48 mm in the control group. The height of the CC body region of the study population was inclined to be thinner compared with the control population (p<0.001).
    CONCLUSIONS: The fact that the height of the CC body region was found to be thinner in fetal MRI in cases of MMC compared with normal fetuses suggests that various callosal anomalies are uncertain, investigation of additional callosal anomalies may be beneficial in the decision for the continuation of pregnancy, and termination or intrauterine surgery in cases with MMC. Further large case group studies are needed.
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  • 文章类型: Journal Article
    目的:选择开放性脊柱裂(OSB)的胎儿进行胎儿手术,以改善长期预后。使用先进的磁共振成像(MRI)定量的体积,表面积,大脑结构的形状,和使用对应于回转的参数的表面曲率分析,我们研究了胎儿手术对大脑发育的影响。
    方法:我们比较了29个OSB胎儿在胎儿手术前(<24周)的MRI图像,手术后一周和六周,至36个年龄匹配的正常对照(21+2-36+2周)。自动超分辨率重建提供了3D各向同性体积脑图像。无髓鞘白质,小脑和脑室被自动分割和手动细化;脑容量,表面积,和形状参数(体积/表面积)被定量。使用数学标记(形状指数(SI)和曲率)来测量回转性。根据病变类型评估参数(例如,骨髓分裂(MS)),术后持续性后脑疝(HH),以及存在幕上异常,即call体部分发育不全(pACC),和异位症(HT)。
    结果:手术后六周,OSB心室容积增长(mm3/周)和形状参数(mm/周)高于对照组;(2500.94,IQR:1665.51-3604.1mm3/周vs708.21,IQR:435.06-965.77mm3/周,p<0.001),和(0.075,IQR:0.047-0.112毫米/周vs0.022,IQR:0.009-0.042毫米/周,p=0.046)。在pACC(p<0.001)和持续性HH(p=0.002)的情况下,心室容积增长在手术后六周增加。在此期间,与对照组相比,OSB无髓鞘白质形状参数变化(毫米/周)降低(0.056,IQR:0.044-0.092毫米/周vs0.159,IQR:0.1-0.247毫米/周,p=0.002),特别是在持续性HH(p=0.011)的情况下,MS(p=0.015),HT(p=0.022),具有CC异常的HT(p=0.017),和持续性HH,CC异常(p=0.007)。尽管曲率/周(mm-1)显着降低,但术后6周(0.061,IQR:0.040-0.093mm-1/周vs0.094,IQR:0.072-0.145mm-1/周,p<0.001)与一周后的初始增加相比(0.144,IQR:0.101-0.233mm-1/周vs0.072,IQR:0.059-0.08mm-1/周,p<0.001),与对照组相比,使用SI的旋化总体上有所增加。这一观察结果在患有pACC的胎儿中更为突出,和严重的脑室增宽(p值范围=0.006至<0.001)。
    结论:胎儿OSB修复后,volume,心室和无髓鞘白质的形状和弯曲度与正常胎儿有显著差异.胎儿手术后的脑形态变化不仅限于对CSF循环的影响。这些观察结果可能对产后结局具有神经认知意义。本文受版权保护。保留所有权利。
    Prenatal surgery is offered for selected fetuses with open spina bifida (OSB) to improve long-term outcome. We studied the effect of fetal OSB surgery on brain development using advanced magnetic resonance imaging (MRI) techniques to quantify the volume, surface area and shape of cerebral structures and to analyze surface curvature by means of parameters that correspond to gyrification.
    We compared MRI data from 29 fetuses with OSB before fetal surgery (mean gestational age (GA), 23 + 3 weeks) and at 1 and 6 weeks after surgery, with that of 36 GA-matched control fetuses (GA range, 21 + 2 to 36 + 2 weeks). Automated super-resolution reconstruction provided three-dimensional isotropic volumetric brain images. Unmyelinated white matter, cerebellum and ventricles were segmented automatically and refined manually, after which volume, surface area and shape parameter (volume/surface area) were quantified. Mathematical markers (shape index (SI) and curvedness) were used to measure gyrification. Parameters were assessed according to lesion type (myelomeningocele vs myeloschisis (MS)), postoperative persistence of hindbrain herniation (HH) and the presence of supratentorial anomalies, namely partial agenesis of the corpus callosum (pACC) and heterotopia (HT).
    Growth in ventricular volume per week and change in shape parameter per week were higher at 6 weeks after surgery in fetuses with OSB compared with controls (median, 2500.94 (interquartile range (IQR), 1689.70-3580.80) mm3 /week vs 708.21 (IQR, 474.50-925.00) mm3 /week; P < 0.001 and 0.075 (IQR, 0.047-0.112) mm/week vs 0.022 (IQR, 0.009-0.042) mm/week; P = 0.046, respectively). Ventricular volume growth increased 6 weeks after surgery in cases with pACC (P < 0.001) and those with persistent HH (P = 0.002). During that time period, the change in unmyelinated white-matter shape parameter per week was decreased in OSB fetuses compared with controls (0.056 (IQR, 0.044-0.092) mm/week vs 0.159 (IQR, 0.100-0.247) mm/week; P = 0.002), particularly in cases with persistent HH (P = 0.011), MS (P = 0.015), HT (P = 0.022), HT with corpus callosum anomaly (P = 0.017) and persistent HH with corpus callosum anomaly (P = 0.007). At 6 weeks postoperatively, despite OSB fetuses having a lower rate of change in curvedness compared with controls (0.061 (IQR, 0.040-0.093) mm-1 /week vs 0.094 (IQR, 0.070-0.146) mm-1 /week; P < 0.001), reversing the trend seen at 1 week after surgery (0.144 (IQR, 0.099-0.236) mm-1 /week vs 0.072 (IQR, 0.059-0.081) mm-1 /week; P < 0.001), gyrification, as determined using SI, appeared to be increased in OSB fetuses overall compared with controls. This observation was more prominent in fetuses with pACC and those with severe ventriculomegaly (P-value range, < 0.001 to 0.006).
    Following fetal OSB repair, volume, shape and curvedness of ventricles and unmyelinated white matter differed significantly compared with those of normal fetuses. Morphological brain changes after fetal surgery were not limited to effects on the circulation of cerebrospinal fluid. These observations may have implications for postnatal neurocognitive outcome. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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  • 文章类型: Journal Article
    目的:开放性脊柱裂(OSB)是最常见的神经管缺损。产前修复将脑积水引起的脑室腹膜分流(VPS)的需求从80-90%降低到40-50%。我们的目的是确定在我们的人群中,哪些变量是12月龄时VPS的危险因素。
    方法:39例患者通过小范围子宫切开术进行了OSB的产前修复。主要结果是在生命的前12个月发生VPS。Logistic回归用于估计产前变量与分流需求之间的比值比(OR)。
    结果:12个月时的VPS发生率为34.2%。手术前较大的心室大小(62.5%≥15毫米;46.2%在12至15毫米之间;11.8%<12毫米;p=0.008),较高的病变水平(80%>L2,vs.17.9%≤L3;p=0.002;OR,18.4[2.96-114.30]),和手术时的胎龄(25.25±1.18vs.24.37±1.06周;p=0.036;OR,2.23[1.05-4.74])与调车需求增加有关。在多变量分析中,手术前较大的心室大小(≥15mmvs.<12mm;p=0.046;OR,1.35[1.01-1.82])和更高的病变水平(>L2vs.≤L3;p=0.004;OR,39.52[3.25-480.69])是分流的危险因素。
    结论:在研究人群中,在接受小范围子宫切开术产前OSB修复的胎儿中,手术前较大的心室大小(≥15mm)和较高的病变水平(>L2)是12月龄VPS的独立危险因素。
    OBJECTIVE: Open spina bifida (OSB) is the most common neural tube defect. Prenatal repair reduces the need for ventriculoperitoneal shunting (VPS) due to hydrocephalus from 80-90% to 40-50%. We aimed to determine which variables work as risk factors for VPS at 12 months of age in our population.
    METHODS: Thirty-nine patients underwent prenatal repair of OSB by mini-hysterotomy. The main outcome was occurrence of VPS in the first 12 months of life. Logistic regression was used to estimate the odds ratios (OR) between prenatal variables and the need for shunting.
    RESULTS: VPS at 12 months occurred in 34.2% of the children. Larger ventricle size before surgery (62.5% ≥15 mm; 46.2% between 12 and 15 mm; 11.8% <12 mm; p=0.008), higher lesion level (80% >L2, vs. 17.9% ≤L3; p=0.002; OR, 18.4 [2.96-114.30]), and later gestational age at surgery (25.25 ± 1.18 vs. 24.37 ± 1.06 weeks; p=0.036; OR, 2.23 [1.05-4.74]) were related to increased need for shunting. In the multivariate analysis, larger ventricle size before surgery (≥15 mm vs. <12 mm; p=0.046; OR, 1.35 [1.01-1.82]) and higher lesion level (>L2 vs. ≤L3; p=0.004; OR, 39.52 [3.25-480.69]) were risk factors for shunting.
    CONCLUSIONS: Larger ventricle size before surgery (≥15 mm) and higher lesion level (>L2) are independent risk factors for VPS at 12 months of age in fetuses undergoing prenatal repair of OSB by mini-hysterotomy in the studied population.
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