Spinal bifida

  • 文章类型: Journal Article
    这篇综述涵盖了胚胎学,定义,和开放性脊柱发育不良的诊断,重点是胎儿超声和MR影像学发现。还将讨论在胎儿影像学上区分开放性和闭合性脊柱畸形。还回顾了当前的胎儿手术实践和胎儿手术中的影像学发现。
    This review covers the embryology, definition, and diagnosis of open spinal dysraphism with a focus on fetal ultrasound and MR imaging findings. Differentiating open versus closed spinal dysraphic defects on fetal imaging will also be discussed. Current fetal surgery practices and imaging findings in the context of fetal surgery are also reviewed.
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  • 文章类型: Case Reports
    目的是描述一例罕见的表现为进行性尿失禁的腰椎膜膜膨出症。脂膜脊膜膨出是一种闭合性脊柱发育不良,通常表现为脂肪瘤状肿块,在臀裂上方有神经缺损。脊髓栓系综合征被定义为由异常低的脊髓圆锥引起的过度脊髓张力引起的症状和体征。末端有一个异常厚的丝附于下部骶骨区。一名19岁男性,无明显病史,过去一年出现腰背痛和尿失禁。在身体检查中,患者的运动强度正常,所有方式的感官测试都完好无损。直肠音正常,并且没有注意到鞍式麻醉。MRI显示腰椎脂肪膜脊膜膨出伴脊髓栓系综合征。患者接受了栓系脐带松解术和脂肪瘤切除术。软组织病理显示纤维血管组织和成熟脂肪组织符合脂肪瘤。大多数脊髓栓系综合征病例与脊髓发育不良有关,一种罕见的儿科综合症.如果及早发现,它可能是可以治疗的,MRI可以帮助准确诊断病情。老年人更容易出现泌尿系统和神经系统疾病。有进行性症状的患者需要进行手术解开。在我们的案例中,唯一的症状是尿失禁,除腰椎椎旁压痛外,神经系统检查正常。
    The objective is to describe a rare case of lumbar lipomyelomeningocele presenting as progressive urinary incontinence. Lipomyelomeningocele is a type of closed spinal dysraphism typically presenting as a lipomatous mass contiguous with a neural defect above the gluteal crease. Tethered cord syndrome is defined as symptoms and signs caused by excessive spinal cord tension from an abnormally low conus medullaris, with an abnormally thick filum terminale attached to the lower sacral region. A 19-year-old male with no remarkable medical history presented with low back pain and urinary incontinence for the past one year. On physical exam patient had normal motor strength, sensory testing to all modalities was intact. The rectal tone was normal, and no saddle anesthesia was noted. MRI lumbar spine revealed lumbar lipomyelomeningocele with associated tethered cord syndrome. The patient underwent tethered cord release surgery with lipoma excision. Pathology of the soft tissue showed fibrovascular tissue and mature adipose tissue consistent with lipoma. The majority of cases of tethered cord syndrome are related to spinal dysraphism, a rare pediatric syndrome. It is potentially treatable if caught early, and MRI can help with an accurate diagnosis of the condition. Older adults are more likely to present with urological and neurological complaints. Surgical un-tethering is indicated in patients with progressive symptoms. In our case, the only presenting symptom was urinary incontinence, and the neurological exam was normal other than lower lumbar paraspinal tenderness.
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  • 文章类型: Journal Article
    OBJECTIVE: To characterize common clinical indications for urodynamic, a bladder function test, in adults with spina bifida.
    METHODS: A retrospective chart review was performed for 215 patients seen in an adult multidisciplinary spina bifida clinic who were registered with the National Spina Bifida Patient Registry from October 2011 to October 2018. Descriptive statistics were used for statistical comparisons.
    RESULTS: A total of 52 of 215 patients developed a clinical indication for urodynamics. Of these, 71 (33%) patients (8 of whom underwent testing twice) had urodynamics performed, resulting in a total of 79 urodynamic study encounters that were analyzed. Thirty-four (43%) urodynamic testing cases were performed due to a symptomatic change in lower urinary tract function; 14 (18%) were due to declining renal function or concern for upper tract deterioration based on imaging. The data obtained from urodynamic investigation led to new recommendations for urinary tract management in 59 (75%) of the urodynamic studies performed. A total of 32 of the 90 (35%) recommendations made were surgical interventions and 30 (33%) were for a change in medical management. Interestingly, 8 of the 18 (44%) routine or baseline urodynamic tests performed led to new recommendations in urinary tract management.
    CONCLUSIONS: A total of 24%of patients in the multidisciplinary spina bifida clinic developed an indication for urodynamic testing over a 7-year period which resulted in new recommendations for urinary tract management in most. As more patients with spina bifida enter adulthood, the indications for urodynamic evaluation may become more defined, since the results often lead to alterations in bladder management.
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  • 文章类型: Journal Article
    Neural tube defects remain a major problem in developing countries, but there are limited comprehensive national reports to date in Ethiopia. Therefore, this study aimed to assess the prevalence of neural tube defects and associated factors in Ethiopia. Electronic databases and other sources were used to retrieve studies. Fifteen out of 862 studies were included in the final analysis. The estimated pooled prevalence of neural tube defects among children in Ethiopia was 63.3 cases per 10 000 children. The pooled prevalence of spinal bifida, anencephaly, and encephalocele was 41.09, 18.90, and 1.07 per 10 000 children, respectively. Previous family history and unplanned pregnancy were risk factors for neural tube defects. Folic acid supplementation during the first trimester of pregnancy was found to be protective. Neural tube defects are widespread in Ethiopia. Hence, fortification of food with folic acid or folic acid supplementation during childbearing age is recommended.
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  • 文章类型: Journal Article
    BACKGROUND: Tethered cord syndrome (TCS) is an increasingly recognized clinical condition, with untethering surgery considered the classically effective treatment. Yet, as evidence has aggregated, the surgical outcomes of untethering remain controversial. This study aimed to systematically evaluate the clinical outcomes in patients who underwent primary or revision untethering.
    METHODS: This retrospective study was conducted at the Department of Spine Surgery of Changzheng Hospital between January and December 2016. Patients with TCS who underwent untethering surgery were recruited for the study. Information collected included demographic data, main clinical manifestations, and outcomes after untethering surgery.
    RESULTS: A total of 112 patients (60 males and 52 females) were included in this study, with mean follow-up of 13.7 years. The surgical outcomes of the primary untethering varied among symptoms, with remission rates of 30.0% for pain, 24.4% for paresthesia, 18.6% for motor deficit, 12.6% for bladder dysfunction, and 21.2% for bowel dysfunction. Moreover, 23.3%-40.2% of patients suffered deterioration after surgery during the follow-up period. Twenty-eight patients underwent revision untethering surgery. In these patients, the remission rate decreased by 5.6%-16.7% compared with the primary operation. Moreover, most patients (58.8%-70.6%) experienced no benefits after revision surgery.
    CONCLUSIONS: With a possible high risk of recurrence, further exploration of the indications and timing of the untethering surgery is needed. Conservative treatment and omnidirectional care might be a better choice for patients with retethering with recurrent symptoms.
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  • 文章类型: Journal Article
    Spina bifida is a common congenital anomaly of the nervous system. It is frequently associated with significant morbidity and sometimes mortality in affected children. In this paper, we review the clinico-epidemiological pattern, peculiarities, and therapeutic challenges of this condition in our practice setting.
    This is a retrospective study of all cases of spina bifida managed from July 2000 to March 2016 at a tertiary health facility located in the southwest region of Nigeria. Relevant information was retrieved from the medical records. The data was collected using a pro forma and analyzed with SPSS version 22.
    Data from 148 patients was reviewed and analyzed. There were 78 males and 70 females. Only 5.8% of these children were delivered at the health institution. A fifth (20%) of the patients were first born of their mothers. The mean maternal age was 29 years. Few (10.1%) mothers use folate medication prior to conception and only 58% of the mothers use folate during antenatal care. Mean duration of pregnancy was 38 weeks. The most common anatomical site was lumbosacral region (74.3%) while the most common pathology was myelomeningocele 80.4%. Mean age at surgery was 88.68 h. Mean duration of surgery was 92.8 min. Mean follow-up duration was 46.8 weeks. As many as 59% of the patients had some neurologic improvement noticed during follow-up clinic visits.
    Spina bifida occurs frequently in our environment. Low socio-economic status and poor antenatal clinic visits contributes significantly to its occurrence.
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  • 文章类型: Journal Article
    OBJECTIVE: To evaluate the accuracy of oblique view extended imaging (OVEI) in locating the position of the fetal conus medullaris.
    METHODS: One hundred and twenty-two normal fetuses and five counterparts with spinal bifida received prenatal ultrasound examination. The vertebral body at the terminal of the conus medullaris and the coronal section of over five vertebral bodies were reconstructed using OVEI. Development of the nervous system of normal fetuses was assessed at postnatal day 28. For spinal bifida cases, pathological examination was performed.
    RESULTS: Among 127 fetuses, the conus medullaris was accurately positioned in 120 (94.0%) cases according to OVEI. OVEI failed to locate the conus medullaris in three healthy fetuses due to obesity of the mother and four cases with spinal bifida due to abnormal fetal position. The conus medullaris was located at L3 or above in 115 healthy fetuses. The conus medullaris was positioned below L4 in five fetuses with spinal bifida, including L5 in two, S1 in two, and S3 in one, which was consistent with the findings of pathological examination.
    CONCLUSIONS: OVEI can display the 12th rib, T12, and conus medullaris simultaneously. OVEI is applicable to precisely locate the position of the conus medullaris and useful for prenatal evaluation of spinal bifida.
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  • 文章类型: Journal Article
    We report a case of rare cervical isthmic spondylolisthesis of C6-7 combined occult spinal bifida at C6, and review the radiologic finding, different diagnosis and treatment. A 23-year old female presented nuchal, back pain after traffic accident. Radiologic finding showed the 6(th) cervical isthmic defect, spondylolisthesis and dysplasia. The patient was conservatively treated about 8 weeks, and 10 months after injury, she was symptom free with full range of motion of cervical spine and she was followed up. Cervical spondylolysis is a very rare condition. This clinical importance is vulnerable to trauma. For whatever reasons, symptomatic patients need to be treated by conservative or surgical option.
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