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"Chiari malformation"

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Chiari Malformation and Hindbrain Descent: Characterization and New Classification Based on Mechanism and Pathogenesis, and Surgical Management
Neurospine. 2025;22(3):696-712.   Published online September 30, 2025
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Chiari Malformation and Hindbrain Descent: Characterization and New Classification Based on Mechanism and Pathogenesis, and Surgical Management
Neurospine. 2025;22(3):696-712.   Published online September 30, 2025
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H. Chiari described 4 types of abnormal development of the posterior fossa, which were subsequently classified as Chiari malformation types I, II, III, and IV. Many issues in neurosurgery concerning classification and surgical management are without evolving concepts. This review aims to clarify the mechanisms and pathogenesis underlying hindbrain (the brain stem and cerebellum) descent, classify them accordingly, and discuss appropriate surgical management. We propose a classification of 4 independent pathogenic mechanisms: (1) constriction in the posterior cranial fossa (PCF) due to underdevelopment of the occipital bone; (2) enlargement of hindbrain; and (3) traction caused by tethering lesions. We examine the pathogenesis of hindbrain descent from embryological perspectives and neuroradiological findings, with a particular focus on lesser-known mechanisms. Additionally, another fourth mechanism is proposed: (4) instability at the craniocervical junction. We suggest a novel classification for Chiari malformation type I based on the underlying pathogenesis, guided by morphometric (occipital bone size) and volumetric (PCF volume) analyses. Furthermore, it delves deeper into their pathogenesis by drawing on insights from developmental biology, genetic studies, and experimental research. Surgical management is tailored to the underlying mechanism, and we proposed the algorithm for decision of surgical intervention. For crowding of the PCF due to underdevelopment of the occipital bone, posterior fossa decompression is the appropriate surgical intervention. For craniocervical instability, occipitocervical fixation is recommended. We also review the recent literature on surgical outcomes associated with each treatment approach. Finally, we highlight current genetic research related to the pathogenesis of hindbrain descent.

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  • Pediatric Chiari Formation – Atlantoaxial Instability is the Cause
    Abhidha Shah, Apurva Prasad, Atul Goel
    Journal of Spinal Surgery.2026; 13(1): 27.     CrossRef
  • 3,256 View
  • 173 Download
  • 1 Web of Science
  • 1 Crossref

Original Articles

Cervical Spine

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Surgical Strategy Analysis of Chiari Malformation With or Without Type II Basilar Invagination According to the Morphological Types of the Atlanto-Occipital Joint: A Retrospective Study of 212 Patients
Neurospine. 2025;22(2):500-513.   Published online April 16, 2025
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Surgical Strategy Analysis of Chiari Malformation With or Without Type II Basilar Invagination According to the Morphological Types of the Atlanto-Occipital Joint: A Retrospective Study of 212 Patients
Neurospine. 2025;22(2):500-513.   Published online April 16, 2025
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Objective
Our previous study categorized atlanto-occipital joint (AOJ) morphology into 3 types, with types II and III-AOJ associated with Chiari malformation (CM) with and without type II basilar invagination (II-BI), respectively. This study aimed to assess the feasibility of tailoring surgical strategies for patients with CM based on AOJ morphological types.
Methods
We retrospectively studied 212 CM patients who underwent foramen magnum decompression (FMD) or combined occipitocervical fusion (OCF). Patients were divided into 4 groups: (1) pure CM with II-AOJ who underwent FMD (CM-II-FMD); (2) pure CM with III-AOJ who underwent FMD+OCF (CM-III-OCF); (3) CM-III-FMD; and (4) CM+ II-BI with III-AOJ who underwent FMD+OCF (BI-III-OCF). Clinical data, including manifestations, imaging findings, surgical details, and neurological assessments, were analyzed at the final follow-up to assess surgical efficacy.
Results
Patients in the BI-III-OCF, CM-III-OCF, and CM-II-FMD groups exhibited a significant improvement in clinical symptoms (pain, sensory disturbances, motor weakness, gait ataxia, and bladder and bowel dysfunction) compared to preoperative levels (p<0.05). Results from the Japanese Orthopaedic Association scale and Neck Disability Index indicated a significant reduction in the degree of neurological impairment within these groups (p<0.05). Furthermore, the Chicago Chiari Outcome Scale scores indicated superior surgical outcomes for patients in these groups. Imaging analyses demonstrated significant reductions in the syringomyelic segment, syringomyelia width, and tonsillar herniation distance among these patients (p<0.05). However, the CM-III-FMD group did not significantly improve in these areas (p>0.05). Postoperative complications occurred in 4.3% of FMD+OCF patients and 3.3% of FMD-only patients.
Conclusion
AOJ morphological types can guide surgical treatment strategies for CM with or without II-BI. FMD alone is suitable for II-AOJ cases, whereas III-AOJ cases should be treated with FMD combined with OCF.
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Magnetic Resonance Imaging-Related Anatomic and Functional Parameters for the Diagnosis and Prognosis of Chiari Malformation Type I: A Systematic Review and Meta-analysis
Neurospine. 2024;21(2):510-524.   Published online June 30, 2024
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Magnetic Resonance Imaging-Related Anatomic and Functional Parameters for the Diagnosis and Prognosis of Chiari Malformation Type I: A Systematic Review and Meta-analysis
Neurospine. 2024;21(2):510-524.   Published online June 30, 2024
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Objective
Imaging parameters of Chiari malformation type I (CMI) development are not well established. This study aimed to collect evidence of general or specific imaging measurements in patients with CMI, analyze indicators that may assist in determining the severity of CMI, and guide its diagnosis and treatment.
Methods
A comprehensive search was conducted across various databases including the Cochrane Library, PubMed, MEDLINE, Scopus, and Embase, covering the period from January 2002 to October 2023, following predefined inclusion criteria. Meta-analyses were performed using RevMan (ver. 5.4). We performed a quantitative summary and systematic analysis of the included studies. This study was registered in the PROSPERO (International Prospective Register of Systematic Reviews) prior to initiation (CRD42023415454).
Results
Thirty-three studies met our inclusion criteria. The findings indicated that out of the 14 parameters examined, 6 (clivus length, basal angle, Boogard’s angle, supraocciput lengths, posterior cranial fossa [PCF] height, and volume) exhibited significant differences between the CMI group and the control group. Furthermore, apart from certain anatomical parameters that hold prognostic value for CMI, functional parameters like tonsillar movement, obex displacement, and cerebrospinal fluid dynamics serve as valuable indicators for guiding the clinical management of the disease.
Conclusion
We collated and established a set of linear, angular, and area measurements deemed essential for diagnosing CMI. However, more indicators can only be analyzed descriptively for various reasons, particularly in prognostic prediction. We posit that the systematic assessment of patients’ PCF morphology, volume, and other parameters at a 3-dimensional level holds promising clinical application prospects.

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  • Idiopathic intracranial hypertension and Chiari I malformation: Overlapping clinical and imaging features
    Om H. Gandhi, Sami Almasri, Shirley Yuan, Osman Baig, Luis O. Tierradentro-Garcia, Jaeha Kim, Warda Ahmed, Linda J. Bagley, Omar Choudhri
    Journal of the Neurological Sciences.2026; 486: 125968.     CrossRef
  • CT- and MRI-based volumetry for Chiari-like malformation and syringomyelia in Pomeranians
    Koen M. Santifort, Ines Carrera, Paul J. J. Mandigers
    Frontiers in Veterinary Science.2025;[Epub]     CrossRef
  • 12,533 View
  • 117 Download
  • 2 Web of Science
  • 2 Crossref

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Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience
Neurospine. 2023;20(4):1501-1512.   Published online December 31, 2023
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Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience
Neurospine. 2023;20(4):1501-1512.   Published online December 31, 2023
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Objective
To compare outcomes of posterior fossa bony decompression alone (PFD) versus PFD with duraplasty (PFDD) versus PFDD with additional obex exploration (PFDDO) in patients with Chiari malformation type I (CMI) combining syringomyelia.
Methods
Clinical records of adult patients who underwent decompressions from 2014 to 2022 were retrospectively analyzed. The decompression procedure was individualized based on the cerebrospinal fluid pulse in the surgical field. The Chicago Chiari Outcome Scale (CCOS) was used to assess the prognosis of the patients and a novel syringomyelia resolution scale, based on 3-dimensional volume, was introduced. The percentage change in the cervical syrinx volume was classified as follows by resolution: ≥ 70%, 30%–70%, and < 30%.
Results
Seventy-eight individuals were enrolled, of which 22, 20, and 36 underwent PFD, PFDD, and PFDDO, respectively. The three decompression groups had no significant difference in the preoperative characteristics and postoperative prognosis. Multivariate analyses revealed that better CCOS was significantly correlated with younger age at surgery (p = 0.018), syrinx originated from lower cervical levels (p = 0.037), narrower preoperative cerebral aqueduct (p = 0.005), and better syrinx volume resolution (p = 0.004). Additionally, a better cervical syrinx volume resolution was significantly correlated with higher CCOS (p = 0.017), narrower cerebral aqueduct (p = 0.035), and better tonsillar descent resolution (p = 0.007).
Conclusion
Individualized functional decompression induced an equal effect on CCOS and syrinx volume resolution for all CMI patients with syringomyelia. Our syringomyelia resolution scale facilitates communication and prediction of CMI prognosis.

Citations

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  • Cerebellar tonsil manipulation in Chiari malformation type I surgery: A systematic review and meta-analysis
    Guilherme L.O. Lima, Rui M.M. Deus, João Victor Massud, Derick Pedrosa Pachá, José Vitor Mota da Silva, Daniel Felipe Fernandes Paiva, Roger Schmidt Brock, Eberval Gadelha Figueiredo
    Clinical Neurology and Neurosurgery.2026; 261: 109264.     CrossRef
  • Integrated neuroimaging approach to the Chiari Malformation Spectrum from type 0 to 1.5: correlations between CSF flow impedance, tonsillar blackout sign, Obex position, and Clinico-anatomical features
    Leyla Salimli Mirzayeva, Murat Uçar, Emetullah Cindil, Sümeyye Nur Budak, Pelin Kuzucu
    Journal of Neuroradiology.2026; 53(2): 101527.     CrossRef
  • Outcomes of posterior decompression and duroplasty using pericranial graft harvested from separate skin incision in chiari malformation type I: a retrospective case series
    Ahmed Samir ElshikhAli, Ebrahim Shamhoot, Yasser Elsawaf, Mohamed Farouk Elfaresy, Essam Abdelhameed
    Egyptian Journal of Neurosurgery.2026;[Epub]     CrossRef
  • Clinical outcome of different surgical approaches for symptomatic Chiari malformation without syringomyelia: a 13-year retrospective study
    Qi-Shuai Yu, Teng Li, Ming Wan, Liang Zhang, Guang-Yu Qiao, Xin-Guang Yu, Yi-Heng Yin
    Neurosurgical Review.2025;[Epub]     CrossRef
  • Efficacy of neurosurgical intervention in syrinx resolution in patients presenting with Chiari malformation type I and syringomyelia: a systematic review and radiological meta-analysis
    Adharsh Suraj Prasad, Aqif Farhan bin Azmil Farid, Isaac Tang Jing Wen, Thomas Zhang, Chandrasekaran Kaliaperumal
    Neurosurgical Review.2025;[Epub]     CrossRef
  • 12,538 View
  • 404 Download
  • 6 Web of Science
  • 5 Crossref

Regular Issue

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Relationship Between Syrinx Resolution and Cervical Sagittal Realignment Following Decompression Surgery for Chiari I Malformation Related Syringomyelia Based on Configuration Phenotypes
Neurospine. 2022;19(4):1057-1070.   Published online December 31, 2022
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Relationship Between Syrinx Resolution and Cervical Sagittal Realignment Following Decompression Surgery for Chiari I Malformation Related Syringomyelia Based on Configuration Phenotypes
Neurospine. 2022;19(4):1057-1070.   Published online December 31, 2022
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Objective
Combined with different configuration types of syringomyelia, to analyze the correlation between syrinx resolution and changes in cervical sagittal alignment following Foramen magnum and Magendie dredging (FMMD) for syringomyelia associated with Chiari I malformation (CM-I), and to further explore the respective relationship with clinical outcome.
Methods
A consecutive series of 127 patients with CM-I and syringomyelia who underwent FMMD in our center met the inclusion criteria of this study. Their clinical records and radiologic data were retrospectively reviewed. The Japanese Orthopedic Association (JOA) scoring system and the Chicago Chiari Outcome Scale (CCOS) were used to evaluate the surgical efficacy. The phenotypes of syringomyelia and the clinical characteristics of the patients were analyzed according to grouping by cervical curvature at baseline.
Results
The preoperative straight or kyphotic cervical alignment is more common in the moniliform syrinx. After surgery, the syrinx resolution and cervical sagittal realignment in the moniliform group are more obvious, and the corresponding prognosis is relatively better. Spearman correlation analysis showed that the ΔS/C ratio (the change ratio of syrinx/cord) was positively correlated with the CCOS (p = 0.001, r = 0.897) and ΔC2–7A (the change of lower cervical angle) (p = 0.002, r = 0.560). There was also a correlation between the ΔJOA score (the change rate of the JOA score) and ΔC2–7A (p = 0.012, r = 0.467).
Conclusion
After decompression surgery, syrinx resolution may coexist with the changes in the subaxial lordosis angle, especially for syrinx in moniliform type, and the relationship between syrinx resolution and cervical sagittal realignment might be valuable for evaluating the surgical outcome.

Citations

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  • Efficacy of neurosurgical intervention in syrinx resolution in patients presenting with Chiari malformation type I and syringomyelia: a systematic review and radiological meta-analysis
    Adharsh Suraj Prasad, Aqif Farhan bin Azmil Farid, Isaac Tang Jing Wen, Thomas Zhang, Chandrasekaran Kaliaperumal
    Neurosurgical Review.2025;[Epub]     CrossRef
  • Correlation Among Syrinx Resolution, Cervical Sagittal Realignment, and Surgical Outcome After Posterior Reduction for Basilar Invagination, Atlantoaxial Dislocation, and Syringomyelia
    Chunli Lu, Wanru Duan, Can Zhang, Yueqi Du, Xinyu Wang, Longbing Ma, Kai Wang, Hao Wu, Zan Chen, Fengzeng Jian
    Operative Neurosurgery.2023; 25(2): 125.     CrossRef
  • Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience
    Shiyuan Han, Bo Hou, Zhimin Li, Feng Feng, Yongning Li, Jun Gao
    Neurospine.2023; 20(4): 1501.     CrossRef
  • 10,449 View
  • 170 Download
  • 4 Web of Science
  • 3 Crossref

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Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
Neurospine. 2022;19(3):816-827.   Published online September 30, 2022
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Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration
Neurospine. 2022;19(3):816-827.   Published online September 30, 2022
Close
Objective
The specific association between morphometric characteristics of the syrinx and the prognosis of Chiari malformation type I (CM-I) with syringomyelia following surgical procedure seems to have not been fully elaborated. This study focused on the preoperative clinical and radiologic parameters in CM-I patients with syringomyelia to find out the relationship between the patients’ clinical status and the phenotypes of the syrinx with surgical outcome.
Methods
A continuous series of pediatric and adult patients with CM-I and syringomyelia from a prospectively maintained database in a single center were included, and we explored the related factors affecting the prognosis following decompression surgery through retrospective analysis of clinical presentations, imaging characteristics, and the morphological features of syringomyelia, to provide a clinical reference for the treatment of syringomyelia.
Results
There were 28 pediatric patients (13.8%), and 174 adults (86.2%) included in our study. The average Chicago Chiari Outcome Scale score was 14.56 ± 1.78. The overall prognosis after surgery was good in our series, among them 152 cases (75.25%) with a favorable prognosis, and syrinx was resolved effectively in 172 cases (85.15%). According to the univariate and multivariate analyses, the preoperative symptom duration, observation time, and with/without moniliform type were independent factors affecting the prognosis in adults. The most obvious difference between moniliform type and nonmoniliform type lies in the preoperative symptom duration, ventral subarachnoid space at the foramen magnum, and with/without straightened cervical physio-curve.
Conclusion
Timely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis.

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  • Chiari 1 Malformation, Factors That May Influence Decision Making, and Introducing the Chiari 1 Malformation Severity Classification System
    Stuart Holder, Muath Abdelkarim Abbakr, Amelia Haynes, Taofiq Desmond Sanusi
    Journal of Clinical Medicine.2025; 14(17): 6113.     CrossRef
  • Efficacy of neurosurgical intervention in syrinx resolution in patients presenting with Chiari malformation type I and syringomyelia: a systematic review and radiological meta-analysis
    Adharsh Suraj Prasad, Aqif Farhan bin Azmil Farid, Isaac Tang Jing Wen, Thomas Zhang, Chandrasekaran Kaliaperumal
    Neurosurgical Review.2025;[Epub]     CrossRef
  • ChatGPT as a Decision Support Tool in the Management of Chiari I Malformation: A Comparison to 2023 CNS Guidelines
    Ethan D.L. Brown, Apratim Maity, Max Ward, Daniel Toscano, Griffin R. Baum, Mark A. Mittler, Sheng-Fu Larry Lo, Randy S. D'Amico
    World Neurosurgery.2024; 191: e304.     CrossRef
  • Post-traumatic syringomyelia resolution following surgical treatment: the moniliform syrinx with a better prognosis
    Chunli Lu, Jian Guan, Chenyuan Ding, Xingwen Wang, Zuowei Wang, Zan Chen, Hao Wu, Fengzeng Jian
    Acta Neurologica Belgica.2023; 123(3): 1061.     CrossRef
  • Single-cell transcriptomics reveals ependymal subtypes related to cytoskeleton dynamics as the core driver of syringomyelia pathological development
    Chunli Lu, Xianming Wu, Xinyu Wang, Zhifeng Xiao, Longbing Ma, Jianwu Dai, Fengzeng Jian
    iScience.2023; 26(6): 106850.     CrossRef
  • Global bibliometric and visual analysis of Chiari malformation
    Longnian Zhou, Haoru Dong, Yiming Tao, Yuanqing Ding, Haiyue Lin, Rong Xie
    Journal of Neurorestoratology.2023; 11(4): 100079.     CrossRef
  • The Physiological Occlusion of the Central Canal May Be a Prerequisite for Syringomyelia Formation
    Chuan Jiang, Xinyu Wang, Chunli Lu, Qian Li, Longbing Ma, Wei Li, Shengyu Cui, Kang Li, Xiang Wang, Yuxin Feng, Fengzeng Jian
    Neurospine.2023; 20(4): 1346.     CrossRef
  • Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience
    Shiyuan Han, Bo Hou, Zhimin Li, Feng Feng, Yongning Li, Jun Gao
    Neurospine.2023; 20(4): 1501.     CrossRef
  • 13,618 View
  • 215 Download
  • 8 Web of Science
  • 8 Crossref

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Type I Chiari Malformation Without Concomitant Bony Instability: Assessment of Different Surgical Procedures and Outcomes in 73 Patients
Neurospine. 2021;18(1):126-138.   Published online March 31, 2021
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Type I Chiari Malformation Without Concomitant Bony Instability: Assessment of Different Surgical Procedures and Outcomes in 73 Patients
Neurospine. 2021;18(1):126-138.   Published online March 31, 2021
Close
Objective
Posterior fossa decompression is the treatment of choice in type 1 Chiari malformation (CM-1) without bony instability. Although surgical fixation has been recommended by a few authors recently, comparative studies to evaluate these treatment strategies using objective outcome tools are lacking.
Methods
Seventy-three patients with pure CM-1 (posterior fossa bony decompression [PFBD], n = 21; posterior fossa bony and dural decompression [PFBDD], n = 40; and posterior fixation [PF], n = 12) underwent a postoperative outcome assessment using Chicago Chiari Outcome Score (CCOS). Logistic regression analysis detected predictors of an unfavorable outcome.
Results
Minimally symptomatic patients generally underwent a PFBD while most of the clinically severe patients underwent a PFBDD (p = 0.049). The mean CCOS score at discharge was highest in the PF (12.0 ± 1.41) and lowest in PFBDD group (10.98 ± 1.73, p = 0.087). Patients with minimal preoperative clinical disease severity (adjusted odds ratio [AOR], 4.58; 95% confidence interval [CI], 1.29–16.31) and PFBDD (AOR, 7.56; 95% CI, 1.70–33.68) represented risks for an unfavorable short-term postoperative outcome. Though long-term outcomes (CCOS) did not differ among the 3 groups (p = 0.615), PFBD group showed the best long-term improvements (mean follow-up CCOS, 13.71 ± 0.95), PFBDD group improved to a comparable degree despite a poorer short-term outcome while PF had the lowest scores. Late deteriorations (n = 3, 4.1%) occurred in the PFBDD group.
Conclusion
Minimally symptomatic patients and PFBDD predict a poor short-term postoperative outcome. PFBD appears to be a durable procedure while PFBDD group is marred by complications and late deteriorations. PF does not provide any better results than posterior fossa decompression alone in the long run.

Citations

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  • C1-C2 Fixation for Chiari with Syringomyelia Associated with Atlantoaxial Instability, Atlantooccipital Assimilation and an Anomalous Vertebral Artery Course
    Chirag Jain, Nupur Pruthi
    World Neurosurgery.2024; 191: 37.     CrossRef
  • Low versus High Intracranial Compliance in Adult Patients with Chiari Malformation Type 1–Comparison of Long-Term Outcome After Tailored Treatment
    Radek Frič, Geir Ringstad, Per Kristian Eide
    World Neurosurgery.2023; 173: e699.     CrossRef
  • Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review
    Raquel Rodríguez-Blanque, Cristina Almazán-Soto, Beatriz Piqueras-Sola, Juan Carlos Sánchez-García, Andrés Reinoso-Cobo, María José Menor-Rodríguez, Jonathan Cortés-Martín
    Journal of Clinical Medicine.2023; 12(20): 6694.     CrossRef
  • Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience
    Shiyuan Han, Bo Hou, Zhimin Li, Feng Feng, Yongning Li, Jun Gao
    Neurospine.2023; 20(4): 1501.     CrossRef
  • Letter to the Editor. Outcome prediction and assessment after surgery in CM-I: there is more to it than meets the eye
    Kuntal Kanti Das, Jaskaran Singh Gosal, Deepak Khatri, Arun K. Srivastava
    Journal of Neurosurgery.2022; 136(3): 934.     CrossRef
  • Commentary on “Frailty Status Is a More Robust Predictor Than Age of Spinal Tumor Surgery Outcomes: A NSQIP Analysis of 4,662 Patients”
    Moon-Jun Sohn
    Neurospine.2022; 19(1): 63.     CrossRef
  • Relationship Between Syrinx Resolution and Cervical Sagittal Realignment Following Decompression Surgery for Chiari I Malformation Related Syringomyelia Based on Configuration Phenotypes
    Chunli Lu, Longbing Ma, Jian Guan, Zhenlei Liu, Kai Wang, Wanru Duan, Zan Chen, Hao Wu, Fengzeng Jian
    Neurospine.2022; 19(4): 1057.     CrossRef
  • Role of Atlas Assimilation in the Context of Craniocervical Junction Anomalies
    Andrei Fernandes Joaquim, Alécio Cristino Evangelista Santos Barcelos, Jefferson Walter Daniel
    World Neurosurgery.2021; 151: 201.     CrossRef
  • 9,781 View
  • 140 Download
  • 8 Web of Science
  • 8 Crossref

Case Reports

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Anterior Spinal Cord Fissuring: A Predictor of Spontaneous Resolution of Syrinx?
Neurospine. 2021;18(1):240-244.   Published online November 17, 2020
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Anterior Spinal Cord Fissuring: A Predictor of Spontaneous Resolution of Syrinx?
Neurospine. 2021;18(1):240-244.   Published online November 17, 2020
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Syringomyelia is a disorder of the spinal cord usually seen in association with a variety of craniovertebral junction anomalies (e.g. , Chiari malformations, basilar invagination/impression, atlantoaxial instability, etc.). Its natural history is not very clearly understood and a majority of patients present with a slowly progressive neurological deficit followed by sudden rapid deterioration. At present, there is a general consensus to offer surgical decompression in all patients diagnosed with Chiari I malformation with syrinx irrespective of their symptoms in order to prevent delayed neurological worsening. Few authors have reported spontaneous resolution of syrinx with persistent tonsillar herniation without operative treatment. We report one such patient and propose anterior spinal cord fissuring as a plausible cause of spontaneous syrinx drainage. We also propose conservative management for patients with an anterior spinal cord fissure seen in index scans instead of early decompression of Chiari malformation.

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  • Syrinx reduction due to spontaneous spinal cord tear: demonstration on 3 T MRI and review of the literature
    Serena Staglianó, Elena Prodi, Barbara Goeggel Simonetti, Alessandro Cianfoni
    BMJ Case Reports.2021; 14(12): e246235.     CrossRef
  • Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case
    Jorn Van Der Veken, Marguerite Harding, Saba Hatami, Marc Agzarian, Nick Vrodos
    Journal of Neurosurgery: Case Lessons.2021;[Epub]     CrossRef
  • 9,759 View
  • 290 Download
  • 2 Web of Science
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Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism
Korean J Spine. 2015;12(3):150-152.   Published online September 30, 2015
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Intracranial Hypertension in a Patient with a Chiari Malformation Accompanied by Hyperthyroidism
Korean J Spine. 2015;12(3):150-152.   Published online September 30, 2015
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The Chiari malformation is an infrequently detected congenital anomaly characterized by the downward displacement of the cerebellum with a tonsillar herniation below the foramen magnum that may be accompanied by either syringomyelia or hydrocephalus. Surgery, such as foramen magnum decompression, is indicated for a symptomatic Chiari malformation, although an incidental lesion may be followed-up without further treatment. Infrequently, increased intracranial pressure emerges due to hyperthyroidism. A nineteen-year-old girl visited our outpatient clinic presented with a headache, nausea and vomiting. A brain and spinal magnetic resonance image study (MRI) indicated that the patient had a Chiari I malformation without syringomyelia or hydrocephalus. An enlarged thyroid gland was detected on a physical examination, and serum markers indicated Graves' disease. The patient started anti-hyperthyroid medical treatment. Subsequently, the headache disappeared after the medical treatment of hyperthyroidism without surgical intervention for the Chiari malformation. A symptomatic Chiari malformation is indicated for surgery, but a surgeon should investigate other potential causes of the symptoms of the Chiari malformation to avoid unnecessary surgery.

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