Abnormal SEP reflects dysfunction of the medial lemniscus and posterior cervical cord. These structures are likely to be affected in Chiari malformation. Therefore, SEP abnormalities may provide valuable information i...Abnormal SEP reflects dysfunction of the medial lemniscus and posterior cervical cord. These structures are likely to be affected in Chiari malformation. Therefore, SEP abnormalities may provide valuable information in patients with CM. However, the consistency of SEP abnormality or normality with the damage is a matter of research. Knowing whether median nerve somatosensory evoked potential (SEP) is useful in revealing subclinical damage in patients with Chiari malformation is important in the treatment and follow-up plan of the disease. The aim of this study was to investigate the relationship between median nerve SEP values and the severity of cerebellar ectopia in patients with Chiari type 1 malformation. Median nerve SEP values were obtained from 30 healthy individuals and 146 individuals with Chiari malformation. The cerebellar ectopia degree and McRae line length were measured. SEP values were not significantly different between groups. The McRae line was found to be significantly shorter in the control group than in the Chiari malformation group (p = 0.031). There was no correlation between the degree of cerebellar ectopia and the length of the McRae line (r = 0.002, p = 0.979). Neither cerebellar ectopy degree nor McRae line length had a relationship with SEP values (r = -0.153, p = 0.066;r = -0.056, p = 0.500, respectively). There was no difference in cerebellar ectopy degree or SEP values between the groups with cerebellar ectopy with and without a syrinx (p = 0.899;p = 0.080, respectively). Likewise, McRae line length was not found to be related to the presence of a syrinx (p = 0.139). Median nerve SEP examination was not beneficial for diagnosing asymptomatic-oligosymptomatic Chiari malformation as a subclinical injury, whether accompanied by syringomyelia or not.展开更多
The use of live animal models for testing new therapies for brain and spinal cord repair is a controversial area. Live animal models have associated ethical issues and scientific concerns regarding the predictability ...The use of live animal models for testing new therapies for brain and spinal cord repair is a controversial area. Live animal models have associated ethical issues and scientific concerns regarding the predictability of human responses. Alternative models that replicate the 3 D architecture of the central nervous system have prompted the development of organotypic neural injury models. However, the lack of reliable means to access normal human neural tissue has driven reliance on pathological or post-mortem tissue which limits their biological utility. We have established a protocol to use donor cerebellar tonsillar tissue surgically resected from patients with Chiari malformation(cerebellar herniation towards the foramen magnum, with ectopic rather than diseased tissue) to develop an in vitro organotypic model of traumatic brain injury. Viable tissue was maintained for approximately 2 weeks with all the major neural cell types detected. Traumatic injuries could be introduced into the slices with some cardinal features of post-injury pathology evident. Biomaterial placement was also feasible within the in vitro lesions. Accordingly, this ‘proof-of-concept’ study demonstrates that the model offers potential as an alternative to the use of animal tissue for preclinical testing in neural tissue engineering. To our knowledge, this is the first demonstration that donor tissue from patients with Chiari malformation can be used to develop a benchtop model of traumatic brain injury. However, significant challenges in relation to the clinical availability of tissue were encountered, and we discuss logistical issues that must be considered for model scale-up.展开更多
Objective:To identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.Method:Patients who were clinically suspected to have Chiari malformation...Objective:To identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.Method:Patients who were clinically suspected to have Chiari malformation type 1 and were referred to the Department of Radiology between 2007 and 2020,and whose diagnosis was radiologically confirmed through magnetic resonance imaging(MRI),were retrospectively evaluated.A total of 49 Chiari malformation type 1 patients with both cervical and cerebral examinations and 49 control subjects of the same age and gender without a diagnosis of Chiari malformation type 1 were included in the study.In Chiari malformation type 1 diagnosed patients,the presence of pseudotumor cerebri,the presence of syringomyelia in cervical spinal MRI images,and the distance of cerebellar tonsils and obex according to McRae line were evaluated in millimeters.Result:In Chiari malformation type 1 clinically and radiologically diagnosed cases,the cerebellar tonsils and obex were located lower in patients with a radiological diagnosis of pseudotumor cerebri compared to those without,and the rate of accompanying syringomyelia appeared to be higher.However,no statistically significant difference was observed between the two groups.Conclusion:The coexistence of pseudotumor cerebri and Chiari malformation type 1 is more common than previously estimated.Different treatment protocols in the coexistence of pseudotumor cerebri and Chiari malformation type 1 emphasize the importance of making this diagnosis.Further radiological imaging studies are needed to identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.展开更多
We report the first case of a neonate with concurrent Chiari II malformation and achondroplasia. Although rare, both these conditions contribute to several deleterious anatomical changes at the cervicomedullary juncti...We report the first case of a neonate with concurrent Chiari II malformation and achondroplasia. Although rare, both these conditions contribute to several deleterious anatomical changes at the cervicomedullary junction and thus predispose to acute hydrocephalus. Although our patient was initially asymptomatic, hydrocephalus ensued several weeks after birth and required cerebral spinal fluid diversion. We discuss the potential links between the two conditions, the pathophysiology, and the important clinical implications for the management of the increased risk of hydrocephalus.展开更多
A growing number of children and adolescents are being diagnosed as Chiari malformation type I （CM- I ） for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this stu...A growing number of children and adolescents are being diagnosed as Chiari malformation type I （CM- I ） for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM- I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM- I underwent surgical treatment in our department. We divided the patients into two groups： pediatric group （n=l 1, age 〈18 years） and adult group （n=73, age 〉18 years）. Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis （36.4%） and developmental delay （36.4%） were more common in pediatric patients, whereas, sensory disturbance （58.9%） and motor weakness （41. 1%） were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group （P〈0.05）. Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments （P〈0.05）. The total Chicago Chiari Outcome Scale （CCOS） score in pediatric patients at the last follow- up was significantly higher than that in adult patients （P=0.002）. In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM- I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.展开更多
Chiari malformations encompass various radiological and clinical entities,sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic.They can be symptomatic or asymp...Chiari malformations encompass various radiological and clinical entities,sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic.They can be symptomatic or asymptomatic.The therapeutic strategies for these malformations differ on the basis of the diverse pathophysiologic processes that cause them.As Chiari malformations are caused by various pathophysiologic processes,they must be recognized promptly to select the best treatment for each single case.展开更多
Chiari malformation is a congenital anomaly that primarily involves the downward displacement of the cerebellar tonsils through the foramen magnum and elongation of forth ventricle and lower brainstem. Patients with C...Chiari malformation is a congenital anomaly that primarily involves the downward displacement of the cerebellar tonsils through the foramen magnum and elongation of forth ventricle and lower brainstem. Patients with Chiari I (congenital or acquired) malformation are asymptomatic or may presents with neurologic signs and symptoms. It is always a question of safety of neuraxial anesthesia in these patients. There is potential risk of dural puncture that can initiate the neurologic symptoms or worsen the existing symptoms due to CSF leakage or tonsillar herniation. Other side, performance of neuraxial anesthesia can cause acquired Chiari I type malformation due to CSF leak and intracranial hypotension. We reviewed the case reports and articles regarding safety of neuraxial anesthesia in the setting of Congenital or Acquired Chiari malformation.展开更多
Introduction: Chiari malformation Type I (CM-I) is typically defined as descent of the caudal tip of cerebellar tonsils at least 5 mm below the foramen magnum. The incidence of the malformation is not exactly known. T...Introduction: Chiari malformation Type I (CM-I) is typically defined as descent of the caudal tip of cerebellar tonsils at least 5 mm below the foramen magnum. The incidence of the malformation is not exactly known. Treatment of Chiari I malformation is debatable. Some advocate posterior fossa decompression (PFD) with duroplasty and others advocate posterior fossa decompression only without duroplasty. Aim of the Study: To compare the outcomes of patients who undergoing PFD with duroplasty and PFD without duroplasty, in Chiari I malformation. Patients and Methods: In the period between “January 2015-June 2016”, a prospective study was conducted involving 20 patients complaining of headache, motor and/or sensory affection secondary to Chiari I malformation. These patients were randomly divided into 2 groups. The first one had PFD with duroplasty and the other will have PFD without duroplasty. All patients had chiari one malformation in form of tonsillar herniation and cervical or cervico-dorsal syrinx. The average follow-up period was 9 months. Results: Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up. There were no major complications. The mean length of hospital stay was 2.0 days. There was no perioperative death or neurological deterioration. The use of duroplasty was significantly associated with presence of complications and longer duration of hospital stay. Conclusion: PFD without duroplasty in cases of chiari I malformation carries a good results as well as PFD with duroplasty with lower risk of complications.展开更多
<strong>Aim of the work:</strong> This study aims to assess the value of the surgical management in the improvement of the symptoms & signs of patients with Chiari malformation type 1 and radiological ...<strong>Aim of the work:</strong> This study aims to assess the value of the surgical management in the improvement of the symptoms & signs of patients with Chiari malformation type 1 and radiological follow up in adults.<strong> Patients and methods: </strong>This study included 30 consecutive patients with Chiari malformation type I who were indicated for surgery at neurosurgery department. Data were collected prospectively from the involved patients who were evaluated preoperatively and underwent evaluation by CT scanning of the brain & skull and MRI imaging of the brain and spine. CT and MRI were done as the routine follow up investigations for all patients. We operated through midline suboccipital craniectomy, durotomy in y shaped manner, shrinkage of cerebellar tonsils by bipolar electrocautery, duroplasty by fascia lata graft, watertight closure. <strong>Results: </strong>The assessment from E. J. N. S. (Egyptian Journal of Neurosurgery) vol. 24 no. 2 June 2009 used for evaluating the patients clinically. 18 patients reported good outcome, 6 fair, 6 poor. We evaluated the size of the syrinx if present preoperative in follow up. 15 (50%) patients showed marked reduction (more than or equal to 60%) in size of syrinx, 3 (10%) mild reduction (less than or equal to 30%) in size and 12 (40%) with no change. <strong>Conclusion:</strong> The Chiari type 1 malformation constitutes a controllable malformation with good outcomes. With current microsurgical techniques, the results of the bony decompression and duroplasty became excellent. Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications.展开更多
Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history ...Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history of headache associated with blurred vision, tinnitus and sickness. Imaging recognised the need for surgical intervention, but whilst awaiting for surgery she fell pregnant. Considering the risks of neurological deterioration, the woman underwent surgical decompression of type I ACM at 15 weeks gestation. She subsequently presented with progressively worsening headaches during late pregnancy from 35 weeks. The obstetric plan was initially induction of labour at term but since the onset of worsening symptoms, this date was brought forward to 39 + 1 weeks gestation. She proceeded to have a normal delivery with no neonatal complications and an uneventful puerperium followed. Since the delivery, the patient reported fewer symptoms, showed no signs of neurological deficit and a repeat magnetic resonance imaging of the head showed good relief of neural compression. This case illustrates how judicious selection of the appropriate mode of delivery of women following surgically corrected ACM and a multidisciplinary approach is critical in the successful management of the antepartum period and labour.展开更多
Objective To investigate the methods and effectiveness to treat Chiari malformation with surgical decompression of foramen magnum under endoscope. Methods 23 cases with Chiari malformation. (withoutatlanto-axial dislo...Objective To investigate the methods and effectiveness to treat Chiari malformation with surgical decompression of foramen magnum under endoscope. Methods 23 cases with Chiari malformation. (withoutatlanto-axial dislocation and basilar invagination) diagnosed by magnetic resonance imaging(MRI) were operated with the surgical decompression for foramen magnum under the endoscope. Results 23展开更多
文摘Abnormal SEP reflects dysfunction of the medial lemniscus and posterior cervical cord. These structures are likely to be affected in Chiari malformation. Therefore, SEP abnormalities may provide valuable information in patients with CM. However, the consistency of SEP abnormality or normality with the damage is a matter of research. Knowing whether median nerve somatosensory evoked potential (SEP) is useful in revealing subclinical damage in patients with Chiari malformation is important in the treatment and follow-up plan of the disease. The aim of this study was to investigate the relationship between median nerve SEP values and the severity of cerebellar ectopia in patients with Chiari type 1 malformation. Median nerve SEP values were obtained from 30 healthy individuals and 146 individuals with Chiari malformation. The cerebellar ectopia degree and McRae line length were measured. SEP values were not significantly different between groups. The McRae line was found to be significantly shorter in the control group than in the Chiari malformation group (p = 0.031). There was no correlation between the degree of cerebellar ectopia and the length of the McRae line (r = 0.002, p = 0.979). Neither cerebellar ectopy degree nor McRae line length had a relationship with SEP values (r = -0.153, p = 0.066;r = -0.056, p = 0.500, respectively). There was no difference in cerebellar ectopy degree or SEP values between the groups with cerebellar ectopy with and without a syrinx (p = 0.899;p = 0.080, respectively). Likewise, McRae line length was not found to be related to the presence of a syrinx (p = 0.139). Median nerve SEP examination was not beneficial for diagnosing asymptomatic-oligosymptomatic Chiari malformation as a subclinical injury, whether accompanied by syringomyelia or not.
基金funded by a grant from the North Staffordshire Medical Institute,UK (to DMC and NT)。
文摘The use of live animal models for testing new therapies for brain and spinal cord repair is a controversial area. Live animal models have associated ethical issues and scientific concerns regarding the predictability of human responses. Alternative models that replicate the 3 D architecture of the central nervous system have prompted the development of organotypic neural injury models. However, the lack of reliable means to access normal human neural tissue has driven reliance on pathological or post-mortem tissue which limits their biological utility. We have established a protocol to use donor cerebellar tonsillar tissue surgically resected from patients with Chiari malformation(cerebellar herniation towards the foramen magnum, with ectopic rather than diseased tissue) to develop an in vitro organotypic model of traumatic brain injury. Viable tissue was maintained for approximately 2 weeks with all the major neural cell types detected. Traumatic injuries could be introduced into the slices with some cardinal features of post-injury pathology evident. Biomaterial placement was also feasible within the in vitro lesions. Accordingly, this ‘proof-of-concept’ study demonstrates that the model offers potential as an alternative to the use of animal tissue for preclinical testing in neural tissue engineering. To our knowledge, this is the first demonstration that donor tissue from patients with Chiari malformation can be used to develop a benchtop model of traumatic brain injury. However, significant challenges in relation to the clinical availability of tissue were encountered, and we discuss logistical issues that must be considered for model scale-up.
文摘Objective:To identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.Method:Patients who were clinically suspected to have Chiari malformation type 1 and were referred to the Department of Radiology between 2007 and 2020,and whose diagnosis was radiologically confirmed through magnetic resonance imaging(MRI),were retrospectively evaluated.A total of 49 Chiari malformation type 1 patients with both cervical and cerebral examinations and 49 control subjects of the same age and gender without a diagnosis of Chiari malformation type 1 were included in the study.In Chiari malformation type 1 diagnosed patients,the presence of pseudotumor cerebri,the presence of syringomyelia in cervical spinal MRI images,and the distance of cerebellar tonsils and obex according to McRae line were evaluated in millimeters.Result:In Chiari malformation type 1 clinically and radiologically diagnosed cases,the cerebellar tonsils and obex were located lower in patients with a radiological diagnosis of pseudotumor cerebri compared to those without,and the rate of accompanying syringomyelia appeared to be higher.However,no statistically significant difference was observed between the two groups.Conclusion:The coexistence of pseudotumor cerebri and Chiari malformation type 1 is more common than previously estimated.Different treatment protocols in the coexistence of pseudotumor cerebri and Chiari malformation type 1 emphasize the importance of making this diagnosis.Further radiological imaging studies are needed to identify different radiological markers for the diagnosis of the coexistence of pseudotumor cerebri and Chiari malformation type 1.
文摘We report the first case of a neonate with concurrent Chiari II malformation and achondroplasia. Although rare, both these conditions contribute to several deleterious anatomical changes at the cervicomedullary junction and thus predispose to acute hydrocephalus. Although our patient was initially asymptomatic, hydrocephalus ensued several weeks after birth and required cerebral spinal fluid diversion. We discuss the potential links between the two conditions, the pathophysiology, and the important clinical implications for the management of the increased risk of hydrocephalus.
基金This project was supported by grants from the National Natural Science Foundation of China （No. 81702478 and No. 81270865） and China Postdoctoral Science Foundation （No. 2016M600596）.
文摘A growing number of children and adolescents are being diagnosed as Chiari malformation type I （CM- I ） for behavioral disorders, developmental delay, seizures, or abnormal orpharyngeal function. The aim of this study was to compare the clinical characteristics, imaging findings and surgical outcomes of CM- I in pediatric and adult patients. Between January 2014 and June 2017, 84 patients with CM- I underwent surgical treatment in our department. We divided the patients into two groups： pediatric group （n=l 1, age 〈18 years） and adult group （n=73, age 〉18 years）. Data on clinical characteristics, imaging findings, surgical outcomes, and prognosis were retrospectively reviewed and compared between these two groups. For clinical presentation, scoliosis （36.4%） and developmental delay （36.4%） were more common in pediatric patients, whereas, sensory disturbance （58.9%） and motor weakness （41. 1%） were more common in adult patients. Imaging findings showed that the incidence of hydrocephalus and craniovertebral junctional abnormalities was significantly higher in pediatric group than in adult group （P〈0.05）. Compared to adult group, pediatric group showed a better improvement or resolution of syrinx and tonsillar herniation after surgical treatments （P〈0.05）. The total Chicago Chiari Outcome Scale （CCOS） score in pediatric patients at the last follow- up was significantly higher than that in adult patients （P=0.002）. In conclusion, the clinical characteristics and imaging findings appeared to be different in pediatric and adult patients with CM- I. The surgical outcomes of pediatric patients were shown to be significantly better than those of adult patients.
文摘Chiari malformations encompass various radiological and clinical entities,sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic.They can be symptomatic or asymptomatic.The therapeutic strategies for these malformations differ on the basis of the diverse pathophysiologic processes that cause them.As Chiari malformations are caused by various pathophysiologic processes,they must be recognized promptly to select the best treatment for each single case.
文摘Chiari malformation is a congenital anomaly that primarily involves the downward displacement of the cerebellar tonsils through the foramen magnum and elongation of forth ventricle and lower brainstem. Patients with Chiari I (congenital or acquired) malformation are asymptomatic or may presents with neurologic signs and symptoms. It is always a question of safety of neuraxial anesthesia in these patients. There is potential risk of dural puncture that can initiate the neurologic symptoms or worsen the existing symptoms due to CSF leakage or tonsillar herniation. Other side, performance of neuraxial anesthesia can cause acquired Chiari I type malformation due to CSF leak and intracranial hypotension. We reviewed the case reports and articles regarding safety of neuraxial anesthesia in the setting of Congenital or Acquired Chiari malformation.
文摘Introduction: Chiari malformation Type I (CM-I) is typically defined as descent of the caudal tip of cerebellar tonsils at least 5 mm below the foramen magnum. The incidence of the malformation is not exactly known. Treatment of Chiari I malformation is debatable. Some advocate posterior fossa decompression (PFD) with duroplasty and others advocate posterior fossa decompression only without duroplasty. Aim of the Study: To compare the outcomes of patients who undergoing PFD with duroplasty and PFD without duroplasty, in Chiari I malformation. Patients and Methods: In the period between “January 2015-June 2016”, a prospective study was conducted involving 20 patients complaining of headache, motor and/or sensory affection secondary to Chiari I malformation. These patients were randomly divided into 2 groups. The first one had PFD with duroplasty and the other will have PFD without duroplasty. All patients had chiari one malformation in form of tonsillar herniation and cervical or cervico-dorsal syrinx. The average follow-up period was 9 months. Results: Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up. There were no major complications. The mean length of hospital stay was 2.0 days. There was no perioperative death or neurological deterioration. The use of duroplasty was significantly associated with presence of complications and longer duration of hospital stay. Conclusion: PFD without duroplasty in cases of chiari I malformation carries a good results as well as PFD with duroplasty with lower risk of complications.
文摘<strong>Aim of the work:</strong> This study aims to assess the value of the surgical management in the improvement of the symptoms & signs of patients with Chiari malformation type 1 and radiological follow up in adults.<strong> Patients and methods: </strong>This study included 30 consecutive patients with Chiari malformation type I who were indicated for surgery at neurosurgery department. Data were collected prospectively from the involved patients who were evaluated preoperatively and underwent evaluation by CT scanning of the brain & skull and MRI imaging of the brain and spine. CT and MRI were done as the routine follow up investigations for all patients. We operated through midline suboccipital craniectomy, durotomy in y shaped manner, shrinkage of cerebellar tonsils by bipolar electrocautery, duroplasty by fascia lata graft, watertight closure. <strong>Results: </strong>The assessment from E. J. N. S. (Egyptian Journal of Neurosurgery) vol. 24 no. 2 June 2009 used for evaluating the patients clinically. 18 patients reported good outcome, 6 fair, 6 poor. We evaluated the size of the syrinx if present preoperative in follow up. 15 (50%) patients showed marked reduction (more than or equal to 60%) in size of syrinx, 3 (10%) mild reduction (less than or equal to 30%) in size and 12 (40%) with no change. <strong>Conclusion:</strong> The Chiari type 1 malformation constitutes a controllable malformation with good outcomes. With current microsurgical techniques, the results of the bony decompression and duroplasty became excellent. Before undergoing surgical treatment for CM-I, symptomatic patients and their families should be given clear information about the success of treatment and potential complications.
文摘Type I Arnold-Chiari malformation (ACM) usually presents in adulthood and consists of a downward displacement of the cerebellar tonsils through the foramen magnum. A 25-year-old woman presented with a 5-month history of headache associated with blurred vision, tinnitus and sickness. Imaging recognised the need for surgical intervention, but whilst awaiting for surgery she fell pregnant. Considering the risks of neurological deterioration, the woman underwent surgical decompression of type I ACM at 15 weeks gestation. She subsequently presented with progressively worsening headaches during late pregnancy from 35 weeks. The obstetric plan was initially induction of labour at term but since the onset of worsening symptoms, this date was brought forward to 39 + 1 weeks gestation. She proceeded to have a normal delivery with no neonatal complications and an uneventful puerperium followed. Since the delivery, the patient reported fewer symptoms, showed no signs of neurological deficit and a repeat magnetic resonance imaging of the head showed good relief of neural compression. This case illustrates how judicious selection of the appropriate mode of delivery of women following surgically corrected ACM and a multidisciplinary approach is critical in the successful management of the antepartum period and labour.
文摘Objective To investigate the methods and effectiveness to treat Chiari malformation with surgical decompression of foramen magnum under endoscope. Methods 23 cases with Chiari malformation. (withoutatlanto-axial dislocation and basilar invagination) diagnosed by magnetic resonance imaging(MRI) were operated with the surgical decompression for foramen magnum under the endoscope. Results 23
