Effect of Body Mass Index on Chiari Malformation 1 Tonsil Ectopia Length in Adults.

OBJECTIVE: Diagnosis of Chiari I malformation (CM-1) is based on measurements of the inferior extension of the cerebellar tonsils into the foramen magnum on cranial or cervical spine magnetic resonance imaging. Imaging may be obtained before the patient is referred to the neurosurgical specialist. The length of time raises questions about the possibility that body mass index (BMI) fluctuations could affect the measurement of ectopia length. However, previous literature on BMI and CM-1 has reported conflicting findings on BMI. METHODS: We conducted a retrospective chart review of 161 patients who were referred to a single neurosurgeon for CM-1 consultation. Patients with multiple recorded BMI values (n = 71) were compared to see if BMI changes correlated with changes in ectopia length. In addition, we compared and tested 154 recorded ectopia lengths from the patients (1 per patient) and patient BMI values with Pearson correlation and Welch t tests to determine if BMI changes either influenced or were associated with ectopia changes. RESULTS: For the 71 patients with multiple BMI values, change in ectopia length ranged from -4.6-9.8 mm but was not statistically significant (r = 0.019; P = 0.88). For the 154 measured ectopia lengths, changes in BMI did not correlate with ectopia length (P > 0.05). Likewise, differences in ectopia length between patients in normal, overweight, and obese categories were not statistically significant (|tstat| < |tcrit|, P > 0.05). CONCLUSIONS: In individual patients, we found that BMI and changes in BMI were not accompanied by changes in tonsil ectopia length.

Natural History of Chiari 1 Deformity.

PURPOSE: Chiari type 1 deformity (Ch1) is associated with bony deformity of the skull base and herniation of cerebellar tonsils more than 5 mm below the foramen magnum. Magnetic resonance imaging (MRI) is used for diagnosis and surgery is advised for symptomatic children. We present a case series using MRI including cerebrospinal fluid (CSF) flow, in children with Ch1 to demonstrate a variety of outcomes, both surgical and spontaneous: spontaneous resolution, spontaneous worsening, postsurgical improvement, and postsurgical deterioration. CASE: A 2-week-old female newborn underwent brain MRI demonstrating an ectopic neurohypophysis, under opercularization suggesting brain immaturity and a normal craniocervical junction (CCJ). Follow-up (F/U) MRI at 6 years of age showed interval spontaneous development of Ch1 with decreased CSF spaces at CCJ. CASE: A 6-year-old girl referred for imaging with short stature and growth hormone deficiency demonstrated incidental findings of Ch1 without syringomyelia. There was 15-mm protrusion of pointed cerebellar tonsils through the foramen magnum and a reduced CSF space at the craniocervical junction. No surgery was performed, and F/U MRI at the age of 7 years demonstrated spontaneous resolution of the tonsillar ectopia (cerebellar tonsils now 3 mm right and 6 mm left) and expansion of the CSF spaces at CCJ. CASE: A 7-year-old boy with headaches and staring spells underwent an MRI demonstrating 6-mm protrusion of pointed cerebellar tonsils and CSF space reduction at CCJ. No surgery was performed, and F/U imaging at the age of 9 years demonstrated spontaneous improvement in cerebellar tonsillar position and increased bidirectional CSF flow at CCJ. CASE: A 17-month-old boy underwent brain MRI for unsteady gait and poor vestibular response, which showed Ch1 and narrow CSF spaces at the foramen magnum and with reduced CSF flow. At the age of 3 years, after posterior fossa decompression, F/U MRI showed postsurgical improvement of the position of the cerebellar tonsils and increased CSF space at CCJ. CASE: A 4-month-old male infant with a history of 34-week prematurity, prior germinal matrix hemorrhage, and neonatal subdural hemorrhage was referred for MRI of the cervical and thoracic spine for evaluation of developmental delay and hypotonia with torticollis. Magnetic resonance imaging of the spine demonstrated mild protrusion of inferiorly pointed cerebellar tonsils up to the foramen magnum, with visible CSF and without retroflexion of the dens. Follow-up MRI scans demonstrated progressive worsening of the Chiari 1 deformity, even after multiple surgeries. CONCLUSIONS: It is important to be aware of a variety of different outcomes with Ch1, including spontaneous resolution, spontaneous worsening, improvement with surgery, and even deterioration after surgery. More research is required to determine objective criteria for predicting outcome, which include both anatomic measures and physiologic measures of CSF flow, so that better surgical decisions can be made and for evaluating patients who have undergone surgery.

Atlas invagination through the foramen magnum: expanding the spectrum of craniovertebral junction malformations.

PURPOSE: Malformations of the craniovertebral junction (CVJ) range from mild, asymptomatic conditions to severe forms of instability with basilar invagination. Rarely, there have been accounts of forms of so-called paramedian basilar invagination, with abnormal bone masses invading the lateral portion of the foramen magnum. All these entities have been comprehensively classified both from an anatomical and embryological standpoint. METHODS: Here, we report a case of a unique CVJ malformation which is not included in any existing classification framework and could represent a novel pathologic entity. We also provide an overview of the pertinent literature. RESULTS: The patient was a 14-year-old boy with a recent onset of spastic tetraparesis. Radiological studies documented a malformation of the atlas which invaginated through the foramen magnum, causing anterolateral medullary incarceration. Surgical treatment involved posterior decompression with resection of the abnormal bone and occipito-cervical fusion. CONCLUSION: Our report enriches the panorama of CVJ malformations, showing how anatomical knowledge and embryological insights constitute the basis for the correct assessment and treatment of these complex entities.

Basilar Invagination: A Tilt of the Foramen Magnum.

OBJECTIVE: Congenital basilar invagination (BI) is a craniocervical deformity marked by odontoid prolapse into the skull base. The foramen magnum angle (FMA), which is formed by the Chamberlain's line and McRae's line, has not been fully studied. The study aimed to investigate the FMA and its relationship with other craniocervical parameters. METHODS: Participants were divided into control, type A BI, and type B BI groups. Parameters included Chamberlain line violation, atlantodental interval, clivus height, clivus anteroposterior dimension, FMA, basal angle, clivo-axial angle, head and neck flexion angle, Boogard's angle, and subaxial cervical spine lordosis angle. A comparison of these parameters among the 3 groups and correlation analysis between FMA and other parameters were performed. The significance level was set at P < 0.05. RESULTS: A total of 111 controls, 111 type A BI patients, and 62 type B BI patients were enrolled. The FMAs in the control, type A BI, and type B BI groups were 6.21° (3.67°, 8.71°), 22.16° ± 6.61°, and 22.39° (17.27°, 31.08°), respectively. Correlation analysis revealed correlations between the FMA and other variables. In the 2 BI subgroups, FMA was significantly correlated with Chamberlain line violation, clivus height, clivus anteroposterior dimension, basal angle, clivo-axial angle, and Boogard's angle. CONCLUSIONS: The FMA in patients with BI was approximately 22° and approximately 6° in controls, indicating that the foramen magnum in BI had a greater tilt. As a pathological condition, FMA can reflect the degree of BI. Clivus hypogenesis is a reason for the excessive tilt of the FM.

Chiari Malformation (Update on Diagnosis and Treatment).

Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is frequently associated with syringomyelia. Herein, we discuss the history of CMI and syringomyelia, including early pathological and surgical studies. We also describe recent investigations into the pathogenesis and pathophysiology of CMI and their practical implications on management and surgical intervention. We also highlight the recent development of the Common Data Elements for CMI, providing a framework for ongoing investigations. Finally, we discuss current controversies of surgical management in CMI.

The (extended) achondroplasia foramen magnum score has good observer reliability.

BACKGROUND: Achondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed. OBJECTIVE: To assess the interobserver reliability of the achondroplasia foramen magnum score. MATERIALS AND METHODS: Base of skull imaging of children with achondroplasia under the care of Sheffield Children's Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability. RESULTS: Forty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62-0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing. CONCLUSION: The achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes.

Far Lateral Approach.

The far lateral approach is an inferolateral extension of the lateral suboccipital approach. Designed for clipping of the aneurysms of the vertebrobasilar junction and proximal segments of the posterior inferior cerebellar artery, it became over the years a workhorse approach for ventral foramen magnum meningiomas and other intradural lesions located anterior to the dentate ligament.  This article summarizes the technical key aspects of the far lateral approach and transcondylar, supracondylar, and paracondylar extension.

Relevance of C1/2 Facet Configurations and Clivus-Canal-Angles for Adult Patients with Chiari I Malformation with and without Basilar Invagination.

OBJECTIVE: C1/2 facet configurations and clivus-canal-angles (CXAs) have been proposed as criteria for posterior fusion in Chiari I malformation (CMI). METHODS: Three-hundred and forty adults with CMI without basilar invagination (BI), 111 with CMI with BI, and 100 age- and sex-matched controls were studied using sagittal T2-weighted magnetic resonance imaging scans analyzing preoperative and postoperative values with their impact on progression-free survival rates. RESULTS: For CMI without BI, C1/2 facet configurations and CXA were similar to controls (142 ± 11 degrees and 144 ± 10 degrees, respectively) with low rates for posterior C1 displacements (7.1% and 10%, respectively). In CMI with BI, C1 facet displacements were common (54.9%) with lower CXA (120 ± 15 degrees). After foramen magnum decompression (FMD) in CMI without BI (n = 169), 1.8% developed posterior C1 facet displacements without CXA changes and a 97% progression-free survival rate for 10 years. In CMI with BI, patients without ventral compression or instability underwent FMD without fusion (n = 19). Among them, 5.3% developed a posterior C1 facet displacement without CXA changes and a 94% progression-free survival rate for 10 years. The remainder of CMI with BI underwent FMD with C1/2 fusion (n = 48). Among these, CXA values increased with 10-year progression-free survival rates of 74% and 93% with and without ventral compression, respectively. CONCLUSIONS: For adult CMI without BI, C1/2 facet configurations and CXA are irrelevant. FMD alone provides excellent long-term outcomes. In CMI with BI, anterior C1 facet displacements indicate C1/2 instability. Posterior fusions can be reserved for patients with ventral compression or C1/2 instability.

Spinal Cord Atrophy Predicts Progressive Disease in Relapsing Multiple Sclerosis.

OBJECTIVE: A major challenge in multiple sclerosis (MS) research is the understanding of silent progression and Progressive MS. Using a novel method to accurately capture upper cervical cord area from legacy brain MRI scans we aimed to study the role of spinal cord and brain atrophy for silent progression and conversion to secondary progressive disease (SPMS). METHODS: From a single-center observational study, all RRMS (n = 360) and SPMS (n = 47) patients and 80 matched controls were evaluated. RRMS patient subsets who converted to SPMS (n = 54) or silently progressed (n = 159), respectively, during the 12-year observation period were compared to clinically matched RRMS patients remaining RRMS (n = 54) or stable (n = 147), respectively. From brain MRI, we assessed the value of brain and spinal cord measures to predict silent progression and SPMS conversion. RESULTS: Patients who developed SPMS showed faster cord atrophy rates (-2.19%/yr) at least 4 years before conversion compared to their RRMS matches (-0.88%/yr, p < 0.001). Spinal cord atrophy rates decelerated after conversion (-1.63%/yr, p = 0.010) towards those of SPMS patients from study entry (-1.04%). Each 1% faster spinal cord atrophy rate was associated with 69% (p < 0.0001) and 53% (p < 0.0001) shorter time to silent progression and SPMS conversion, respectively. INTERPRETATION: Silent progression and conversion to secondary progressive disease are predominantly related to cervical cord atrophy. This atrophy is often present from the earliest disease stages and predicts the speed of silent progression and conversion to Progressive MS. Diagnosis of SPMS is rather a late recognition of this neurodegenerative process than a distinct disease phase. ANN NEUROL 2022;91:268-281.

Adult Age Differences in Self-Reported Pain and Anterior CSF Space in Chiari Malformation.

Chiari malformation type I (CMI) is a neural disorder with sensory, cognitive, and motor defects, as well as headaches. Radiologically, the cerebellar tonsils extend below the foramen magnum. To date, the relationships among adult age, brain morphometry, surgical status, and symptom severity in CMI are unknown. The objective of this study was to better understand the relationships among these variables using causal modeling techniques. Adult CMI patients (80% female) who either had (n = 150) or had not (n = 151) undergone posterior fossa decompression surgery were assessed using morphometric measures derived from magnetic resonance images (MRI). MRI-based morphometry showed that the area of the CSF pocket anterior to the cervico-medullary junction (anterior CSF space) correlated with age at the time of MRI (r = - .21). Also, self-reported pain increased with age (r = .11) and decreased with anterior CSF space (r = - .18). Age differences in self-reported pain were mediated by anterior CSF space in the cervical spine area-and this effect was particularly salient for non-decompressed CMI patients. As CMI patients age, the anterior CSF space decreases, and this is associated with increased pain-especially for non-decompressed CMI patients. It is recommended that further consideration of age-related decreases in anterior CSF space in CMI patients be given in future research.

Delayed-Onset Neuropathological Complications From a Foramen Magnum and Occipital Crest-Focused Traumatic Brain Injury of the Vietnam War and Other Conflicts: Part II, Research Analysis.

INTRODUCTION: Much of the research impacting diagnosis, outcome, and treatment of traumatic brain injuries (TBIs) has favored time of consciousness criteria indicative of hemispheric blast focus alone. However, recent animal-based research has widely expanded the diagnostic knowledge base and potential treatment options. METHODS: Recent animal-based research findings of foramen magnum and occipital crest-focused blast injuries in laboratory rats were reviewed and compared to the Part I human case report. RESULTS: Comparing the human case report (Part I) to that of animal research studies found very similar neuropathological outcomes, many deep and delayed, and supports why non-cerebral-focused TBIs have gone unrecognized. The overpressure wave is funneled through skull openings of the foramen magnum, with the possibility of a rebound secondary contrecoup injury impacting the orbits, oral-nasal cavity, and ears resulting in additional occult axonal and white matter injury. CONCLUSIONS: Research analysis prompted by a human case report (Part I) has helped identify mechanisms that assist in recognizing and defining non-cerebral hemispheric-focused TBI injuries. Position of the head in relationship to the blast wave, the setting in which the blast occurs, and close diagnostic follow-up are critical to the recognition, diagnosis, and treatment of injuries that have otherwise gone unrecognized and unstudied in humans since the Vietnam War.

Clinical Presentation of Foramen Magnum Meningioma Masqueraded by Carpal Tunnel Syndrome: A Report of 2 Cases.

CASE: Meningioma is the second most common intracranial tumor. We present 2 cases of foramen magnum meningioma (FMM) that was first operated on with the diagnosis of carpal tunnel syndrome (CTS). CONCLUSION: During the diagnostic assessment of CTS and recalcitrant CTS, a more proximal etiology of nerve compression should be considered, including FMM. If a more proximal cause of nerve dysfunction is suspected, cervical spine magnetic resonance imaging may be beneficial to evaluate a patient for spinal etiology.

Foramen Magnum Meningioma-The Attainment of the Intra-Arachnoidal Dissection: 2-Dimensional Operative Video.

Ventral foramen magnum meningiomas are a forbidding lesion. The stake is so high with a risk of devastating paralysis and respiratory failure. Careful preoperative clinical and radiological evaluation is necessary to implement the best treatment plan. Successful surgical intervention depends on paying high attention to minute details throughout the case, from intratracheal intubation to extubation. The neural head-on-neck position is critical to avoid further medullary compression at intubation and positioning.1 Extensive neurophysiological monitoring, including somatosensory, motor, brainstem evoked potential, and cranial nerves, during the positioning and throughout the case, is extremely helpful to detect early signs of dysfunction.1 To expose and access ventral tumors, partial condyle resection and vertebral artery transposition are invaluable techniques.2,3 Preservation and minute manipulation of the vital neurovascular structures at this junction that includes the medullar, anterior spinal artery, posterior inferior cerebellar artery, vertebral junction perforators, and lower cranial nerves are essential for good outcomes. This is achieved by microsurgical intra-arachnoidal dissection under high magnification and after debulking the tumor to establish that plane.1,3,4 The demonstration of this technique is the purpose of this article. We demonstrate these surgical tenets applied to the resection of a large ventral foramen magnum meningioma extending from the midclivus to the C3 vertebral body level in a 54-yr-old female presenting with swallowing difficulties. The patient consented to the surgical intervention and the publication of her images. Image at 1:38 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, © LWW, 1998.

Foramen magnum meningioma with excessive calcification and no dura tail sign: A case report.

RATIONALE: Foramen magnum meningiomas are very rare lesions. They frequently originate from the arachnoid cells at the dura matter of the craniocervical junction. Foramen magnum meningiomas are challenging for neurosurgeons because of the complex anatomy of foramen magnum. We present a rare case of FMM with excessive calcification and without the dura tail sign which made the lesion mimic a teratoma. PATIENTS CONCERNS: A 63 years old woman presented with progressive numbness and hyperesthesia of the shoulders and upper limbs for 2 and half years. She also experienced occasional headaches and dizziness with no nausea, vomiting or fever. DIAGNOSES: Computed tomography scan, and magnetic resonance imaging revealed a calcified mass at occipital cistern. The lesion did not show the usual "dura tail sign" which made it mimic a teratoma on magnetic resonance imaging. Histopathology established meningioma. INTERVENTION: The tumor was completely resected via suboccipital approach. OUTCOMES: Two years follow-up revealed no recurrence of the lesion and no neurological deficits. LESSONS: We advocate the use of electromyographic and auditory brainstem responses to monitor the inferior cranial nerves because the tumor often adheres to these nerves.

Achondroplasia Foramen Magnum Score: screening infants for stenosis.

BACKGROUND: Achondroplasia is associated with foramen magnum stenosis (FMS) and significant risk of morbidity and sudden death in infants. A sensitive and reliable method of detecting infants who require decompressive surgery is required. This study aims to describe the incidence and severity of FMS in an unselected, sequential series of infants using a novel MRI score and retrospectively correlate severity with clinical examination and cardiorespiratory sleep (CRS) studies. METHODS: The Achondroplasia Foramen Magnum Score (AFMS) was developed and scores were retrospectively correlated with clinical and CRS data over a 3-year period. RESULTS: Of 36 infants (M:F, 18:18), 2 (5.6%) did not have FMS (AFMS0); 13 (36.1%) had FMS with preservation of the cerebrospinal fluid (CSF) spaces (AFMS1); 3 (8.3%) had FMS with loss of the CSF space but no spinal cord distortion (AFMS2); 13 (36.1%) had FMS with flattening of the cervical cord without signal change (AFMS3); and 5 (13.9%) had FMS resulting in cervical cord signal change (AFMS4). Mean Total Apnea and Hypopnea Index (TAHI) for AFMS0-4 was 3.4, 6.41, 2.97, 10.5 and 25.8, respectively. Severe TAHI had a specificity of 89% but only a 59% sensitivity for AFMS3-4. Neurological examination was normal in 34/36 (94%) patients. Overall, 9/36 (25%) infants required neurosurgery with minimal surgical complications. CONCLUSIONS: Clinical examination and CRS have a low sensitivity for predicting the effects of foramen stenosis on the spinal cord. Routine screening with MRI using AFMS can aid in detecting early spinal cord changes and has the potential to reduce infant morbidity and mortality.

Far lateral approach without occipital condylar resection for intradural ventral/ventrolateral foramen magnum tumors and aneurysms of V4 segment of vertebral artery: Review of surgical results.

BACKGROUND: Controversies exist regarding the need and extent of condylar resection for safe surgical management of intradural ventral/ventrolateral foramen magnum (VFM) tumors and aneurysms of V4 segment of vertebral artery (VA) by far lateral approach. This retrospective study was conducted to evaluate the results of basic far lateral approach(retrocondylar approach) without upfront occipital condylar resection. METHODS AND RESULTS: Twenty one patients underwent surgery via far lateral approach for intradural VFM tumors and aneurysms of V4 segment of VA at Sri Sathya Sai Institute of Higher Medical Sciences during 9 years(2008-2016) study period. Eight patients had VA aneurysms and 13 patients had intradural VFM tumors. After basic far lateral approach(retrocondylar approach), dura was opened and checked if the exposure was adequate for safe surgery. Retrocondylar approach provided adequate exposure for all these lesions and resection of occipital condyle/jugular tubercle was not required in any of these cases. Skeletonization or transposition of VA was not done in any of these cases. Gross total resection of the tumor could be done in 9 patients(9/13-69.2 %) and near total excision (>95 %) in 4 patients (4/13-30.8 %). Seven of the 8 VA aneurysms were successfully clipped. Outcome at a final follow up of 3 months or more was good(mRS<2) in 19 patients(19/21-90.5 %) and poor in 2 patients. Complications included lower cranial nerve deficits [transient-2/21(9.5 %), persisting-2/21(9.5 %)], motor deficits(2/21-9.5%), seventh nerve paresis(1/21-4.8%), sixth nerve paresis(2/21-9.5%) and pseudomeningocele(1/21-4.8%). CONCLUSION: Basic far lateral (retrocondylar) approach provides excellent exposure for majority of VFM tumors and aneurysms of V4 segment of VA. Condylar resection(transcondylar approach), drilling of jugular tubercle (transtubercular approach), skeletonization/transposition of VA might not be required for safe surgical management of majority of these lesions.

Metastatic large cell neuroendocrine lung cancer to the foramen magnum: A case report.

RATIONABLE: Large cell neuroendocrine carcinoma of the lung is rare, especially in the area of the foramen magnum. No previous studies have reported metastatic large cell neuroendocrine lung cancer to the foramen magnum. This paper will be the first time to report this special case. PATIENT CONCERNS: A case of a 37-year-old woman presented with headache that had developed 20 days previously. Imaging examination revealed a circular abnormal signal at the posterior margin of the foramen magnum. DIAGNOSES: The patient we report was diagnosed with a metastatic intracranial tumor. INTERVENTIONS: The patient underwent occipital craniotomy. Pathological results showed metastatic neuroendocrine carcinoma of the brain. Whole body PET-CT examination showed that fusiform soft tissue shadows could be seen near the hilum of the lower lobe of the left lung. OUTCOMES: The final bronchoscopy pathological results showed the large cell neuroendocrine carcinoma of the lung. The patient underwent further chemotherapy and radiotherapy in the oncology department. LESSONS: Diagnosis and treatment of large cell neuroendocrine carcinoma of the lung are difficult. The prognosis is poorer, and effective treatment is urgently needed.

Intradural Pathology Causing Cerebrospinal Fluid Obstruction in Syringomyelia and Effectiveness of Foramen Magnum and Foramen of Magendie Dredging Treatment.

OBJECTIVE: This article discusses the procedure of foramina magnum and Magendie dredging, summarizing the pathologic changes in the intradural region of the craniocervical junction in patients with syringomyelia and the pathophysiologic mechanism of cerebrospinal fluid (CSF) circulation obstruction. METHODS: Clinical data from 50 adult patients with syringomyelia treated at Xuanwu Hospital from July 2018 to January 2019 were collected and retrospectively analyzed. All operations were performed with foramina magnum and Magendie dredging, and all intradural factors that may have induced the obstruction of CSF circulation were recorded. RESULTS: Intradural pathology was found in all patients. The pathologic changes that may have caused obstruction of the CSF circulation include tonsil occupying the foramen magnum and overlying foramen of Magendie in 88% (44/50), intertonsillar arachnoid adhesions in 36% (18/50), tonsil to medulla arachnoid adhesions in 18% (9/50), medialized tonsils in 70% (35/50), vermian branch of posterior inferior cerebellar artery in 22% (11/50), arachnoid veil in 16% (8/50), cisterna magna cyst in 4% (2/50), and tonsil to dura mater arachnoid adhesions in 8% (4/50). Mean duration of follow-up was 13.3 months. The long-term effective rate was 96.0%. Postoperative magnetic resonance imaging revealed that the size of the syringomyelia was reduced or completely resolved in 88% of patients. The mean preoperative Japanese Orthopaedic Association score was 12.9 ± 3.1, which improved to 14.7 ± 3.2 (P < 0.05) at last clinical follow-up. CONCLUSIONS: Intradural pathology that causes CSF circulation obstruction exists in many forms. Relieving the obstruction of the foramen magnum and foramen of Magendie is key to surgical treatment.