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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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STUDY DESIGN: Retrospective cohort study. OBJECTIVES: We aimed to evaluate the effectiveness of terminal ventriculostomy in treating tethered cord syndrome (TCS) combined with terminal syringomyelia (TS) and describe "V"-type ostomy as an effective surgical method to avoid relapsing syringomyelia based on terminal ventriculostomy. METHODS: We retrospectively analyzed the clinical and radiological data of 28 patients admitted to the Department of Neurosurgery, PLA General Hospital who had been diagnosed with TCS combined with TS and underwent terminal ventriculostomy-associated "V"-type ostomy between January 2011 and January 2016. We classified patients' clinical outcomes into 4 levels according to the Spinal Bifida Neurological Scale: markedly improved, improved, stable, and deteriorated. The size of the syrinx cavity was quantified using the syrinx index, and there was a difference in syrinx cavity size between pre-operation and post-operation. RESULTS: Twenty-eight patients were followed up for 36 months. We found that each syrinx cavity shrunk by at least 50%. More than 90% of patients had achieved "markedly improved" and "improved" outcomes during the follow-up visit. Moreover, no patient relapsed for up to 36 months post-surgery. CONCLUSION: Terminal ventriculostomy has a beneficial effect on TS, particularly on the syrinx cavity extending to the filum terminale. For this special cavity, we advocate the use of terminal ventriculostomy-associated "V"-type ostomy to avoid potential relapse. As a safe, convenient, and persistently effective approach, terminal ventriculostomy-associated "V"-type ostomy can be considered a promising alternative method for treating TCS combined with TS in clinical practice.

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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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Spinal bifida aperta (SBA) is a congenital malformation with a high incidence. Bone marrow mesenchymal stem cell (BMSC) transplantation has the potential to repair the structure of damaged tissues and restore their functions. This is an optional treatment that can be used as a supplement to surgery in the treatment of SBA. However, the application of BMSCs is limited, as the neuronal differentiation rate of BMSCs is not satisfactory when used in treating severe SBA. Thus, we aimed to assess the effect of neural stem cell (NSC)-derived exosomes on BMSC neuronal differentiation and observe the therapeutic effect in an ex vivo rat SBA embryo model. We found that NSC-derived exosomes increased the neuronal differentiation rate of BMSCs in vitro and in the SBA embryo model ex vivo. Proteomic analysis showed that NSC-derived exosomes were enriched in Netrin1, which positively regulated neuronal differentiation. Netrin1 increased the neuronal differentiation rate of BMSCs and NSCs and upregulated the expression of the neuronal markers, microtubule-associated protein (Map2), neurofilament, and ß3-tubulin. Bioinformatic analysis revealed that Netrin1 treatment increased the expression of the transcription factors Hand2 and Phox2b, related to neuronal differentiation. Furthermore, the Netrin1-induced NSC neuronal differentiation was significantly blocked by Phox2b knockdown. We suggest that NSC-derived exosomal Netrin1 induces neuronal differentiation via the Hand2/Phox2b axis by upregulating the expression of Hand2 and Phox2b. Therefore, NSC-derived exosomes are a critical inducer of BMSC neuronal differentiation and represent a potential treatment agent that can benefit BMSC treatment in SBA.

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PURPOSE: 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. CONCLUSION: 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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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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Spinal bifida is one of the phenotypes of neural tube defects,which are influenced by genetic and environmental factors.There are many genes involved in the occurrence of spinal bifida,and the main genes and animal models are mainly concentrated in the signaling pathway.This article reviews the research progresses on pathogenesis of spinal bifida and relationship between key genes,and further clarifies the pathogenesis of spinal bifida,and achieves more effective prevention and control of neural tube defects.

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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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PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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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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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PURPOSE: Individuals with spina bifida are typically followed closely as outpatients by multidisciplinary teams. However, emergent care of these patients is not well defined. We describe patterns of emergent care in patients with spina bifida and healthy controls. MATERIALS AND METHODS: We reviewed Nationwide Emergency Department Sample data from 2006 to 2010. Subjects without spina bifida (controls) were selected from the sample using stratified random sampling and matched to each case by age, gender and treatment year at a 1:4 ratio. Missing emergency department charges were estimated by multiple imputation. Statistical analyses were performed to compare patterns of care among emergency department visits and charges. RESULTS: A total of 226,709 patients with spina bifida and 888,774 controls were identified. Mean age was 28.2 years, with 34.6% of patients being younger than 21. Patients with spina bifida were more likely than controls to have public insurance (63.7% vs 35.4%, p <0.001) and to be admitted to the hospital from the emergency department (37.0% vs 9.2%, p <0.001). Urinary tract infections were the single most common acute diagnosis in patients with spina bifida seen emergently (OR 8.7, p <0.001), followed by neurological issues (OR 2.0, p <0.001). Urological issues were responsible for 34% of total emergency department charges. Mean charges per encounter were significantly higher in spina bifida cases vs controls ($2,102 vs $1,650, p <0.001), as were overall charges for patients subsequently admitted from emergent care ($36,356 vs $29,498, p <0.001). CONCLUSIONS: Compared to controls, patients with spina bifida presenting emergently are more likely to have urological or neurosurgical problems, to undergo urological or neurosurgical procedures, to be admitted from the emergency department and to incur higher associated charges.

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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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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 6th 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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@#ObjectiveTo explore the efficacy and safety of sacral neuromodulation(SNM) for the treatment to neurogenic bladder.MethodsOne patient with neurogenic bladder after spinal bifida underwent the therapy of SNM 42 months ago.The therapeutic efficacy was evaluated and followed up by means of the symptom improvement and voiding diaries.ResultsDuring the test stimulation period,there were significant improvements(>50%) in the objective findings and subjective symptoms.This patient received permanent electrode and neurostimulator implantation and lower urinary tract symptoms were improved continuously until 42 months.ConclusionSNM may be effective for some neurogenic dysfunctions of the bladder.