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Symptomatic CM1, also called the Chiari syndrome, is characterized mainly by headache but also by numbness or weakness in the hands or feet and visual, hearing, balance, and cranial-nerve abnormalities. It is treated surgically.

Purpose In patient with Chiari type I malformations (CM1), indication for surgery can be difficult to establish. Headaches are a common complaint. Factors that predict headache relief have not been clearly identified. Several studies have aimed to examine cerebrospinal fluid (CSF) hydrodynamics in patients with CM1 by using phase-contrast MRI (pcMRI), which is currently the only non-invasive method for assessing craniospinal hydrodynamics and hemodynamics. People with CM1 present alterations in cerebrospinal fluid (CSF) and cerebral blood dynamics. The objective of the present study was to identify hydrodynamic criteria that are predictive of positive clinical outcome (headache relief) after posterior fossa decompression surgery in patient with CM1. Method 41 patients who underwent posterior fossa decompression surgery at Amiens-Picardie University Hospital (Amiens, France) between 2016 and 2021 were retrospectively included. We used preoperative pcMRI to analyze CSF dynamics. Stroke volumes of cerebrospinal fluid were quantified at the aqueduct of Sylvius (SV aqu ), subarachnoid spaces near to C2-C3 (SV C2C3 ) vertebral junction, prepontine cisterns, foramen magnum, and brainstem. CSF pulsatility was analyzed in relation to whether patients reported postoperative headache relief. Statistical analyses were based on Student's t-test. Results 12 patients reported headache relief. The mean SV aqu was significantly higher in patients with headache relief than in those without relief (65 and 32.13µL/CC, p ≤ 0.05). The mean SV C2-C3 was significantly lower in patients with headache relief than in patients without relief (484.58 and 612.94µL/CC, p ≤ 0.05). The two groups of patients did not differ significantly in terms of the area of the narrowest part of the aqueduct of Sylvius or the Evans index. Conclusion SVaqu may have prognostic value for headache relief following surgery for CM1. Further investigation is warranted. This association is likely related to the recruitment of intraventricular pulsatility, which may help regulate potential intracranial pressure changes. Notably, this pulsatility does not appear to be linked to morphological features.

Background: The treatment of type 1 Chiari malformation (CM-1) with posterior fossa decompression without (PFD) or with duraplasty (PFDD) is controversial. The authors analyze both options in a national sample of pediatric patients. Methods: Utilizing the Kids' Inpatient Database, CM-1 patients undergoing PFD or PFDD from 2000 through 2009 were analyzed. Results: 1,593 patients with PFD and 1,056 with PFDD were evaluated. The average age was 10.3 years, slightly younger in PFD (9.8 vs. 10.9 years, p = 0.001). PFDD patients were more likely White (81.2 vs 75.6%, p = 0.04) and less likely admitted emergently (8.4 vs. 13.8%, p = 0.007). They also underwent more reoperations (2.1 vs. 0.7%, p = 0.01), had more procedure-related complications (2.3 vs. 0.8%, p = 0.003), a longer length of stay (4.4 vs. 3.8 days, p = 0.001) and higher charges (USD 35,321 vs. 31,483, p = 0.01). Conclusions: This large national study indicates that PFDD is performed more often in Caucasians, less so emergently, and associated with significantly more complications and immediate reoperations, while PFD is more frequent in those with syringomyelia and more economical, requiring fewer hospital resources. Overall, PFD is more favorable for CM-1, though it would be prudent to conduct a prospective trial, as this analysis is limited by data on preoperative presentations and long-term outcomes.

Fibrous dysplasia is a benign bone disease characterized by the replacement of normal bone tissue with fibrous tissue, resulting in irregular bone structure. Cases of craniofacial fibrous dysplasia in children associated with Chiari type I malformation and syringomyelia are extremely rare. This case illustrates the complex clinical manifestations of craniofacial fibrous dysplasia along with Chiari type I malformation and syringomyelia, in which surgical intervention significantly improved the prognosis, and follow-up revealed near-complete resolution of the syringomyelia. It offers valuable insights for managing similar cases in the future.

ObjectiveAs the understanding of pathophysiology behind Chiari malformation still is limited, the treatment of Chiari malformation type 1 remains rather empirical. This may result in suboptimal treatment strategy and outcome in many cases. In this review, we critically address whether the condition known today as Chiari malformation type I should rather be denoted Chiari syndrome.MethodsThe current knowledge of Chiari malformation type 1 is summarized from the historical, etymological, genetic, clinical, and in particular pathophysiological perspectives.ResultsThere are several lines of evidence that Chiari malformation type 1 represents a condition significantly different from types 2 to 4. Unlike the other types, the type 1 should rather be considered a syndrome, thus supporting the reasons to reappraise the traditional classification of Chiari malformations.ConclusionWe propose that Chiari malformation type 1 should rather be denoted Chiari syndrome, while the notation malformation is maintained for types 2–4.

Background Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction. Methodology The study presents a case of a 2.5-year-old female diagnosed with CM III and KFS. Diagnostic tools such as ultrasound, CT scans, MRI, and physical examinations were used to confirm the patient’s condition. Surgical interventions, including decompression and encephalocele repair, were performed. Results Successful surgical interventions, including encephalocele repair and duraplasty, were carried out. Follow-up visits indicated a stable condition, marked improvement in lower limb strength, and the patient’s ability to walk with assistance. CT follow-up affirmed a satisfactory surgical outcome. Conclusion This case study illustrates the potential for an optimistic prognosis in CM III, even when accompanied by complex conditions such as KFS, through early diagnosis and intervention. It underscores the significance of antenatal screening for effective care planning and calls for further research and publications due to the rarity of this association. These findings contribute to our understanding of CM III and its related conditions, emphasizing the need for open-minded consideration of potential embryological associations.

Abstract BACKGROUND: It has been well documented that, along with tonsillar herniation, Chiari Malformation Type I (CMI) is associated with smaller posterior cranial fossa (PCF) and altered cerebrospinal fluid (CSF) flow and tissue motion in the craniocervical junction. OBJECTIVE: This study assesses the relationship between PCF volumetry and CSF and tissue dynamics toward a combined imaging-based morphological-physiological characterization of CMI. Multivariate analysis is used to identify the subset of parameters that best discriminates CMI from a healthy cohort. METHODS: Eleven length and volumetric measures of PCF, including crowdedness and 4th ventricle volume, 4 measures of CSF and cord motion in the craniocervical junction, and 5 global intracranial measures, including intracranial compliance and pressure, were measured by magnetic resonance imaging (MRI) in 36 symptomatic CMI subjects (28 female, 37 ± 11 years) and 37 control subjects (24 female, 36 ± 12 years). The CMI group was further divided based on symptomatology into “typical” and “atypical” subgroups. RESULTS: Ten of the 20 morphologic and physiologic measures were significantly different between the CMI and the control cohorts. These parameters also had less variability and stronger significance in the typical CMI compared with the atypical. The measures with the most significance were clival and supraocciput lengths, PCF crowdedness, normalized PCF volume, 4th ventricle volume, maximal cord displacement (P < .001), and MR measure of intracranial pressure (P = .007). Multivariate testing identified cord displacement, PCF crowdedness, and normalized PCF as the strongest discriminator subset between CMI and controls. MR measure of intracranial pressure was higher in the typical CMI cohort compared with the atypical. CONCLUSION: The identified 10 complementing morphological and physiological measures provide a more complete and symptomatology-relevant characterization of CMI than tonsillar herniation alone.

Chiari malformation (CM) is a condition in which cerebellar tonsillar ectopia may manifest with various clinical presentations. This study reports from the only national, online patient registry available, the symptoms, comorbid neurocognitive and psychological conditions, and diagnostic experiences of patients living with CM type I (CM I). The current research is one component of a large investigation designed to collect information from individuals with CM through the online Conquer Chiari Patient Registry questionnaire. Analyses included descriptive statistics to study body system impact and patient diagnostic experiences. Participants were 768 individuals with CM I and were predominantly female (86.8 %) and Caucasian (93.8 %) with an average age of 35 years. Pain was the most frequently reported symptom (76.69 %) experienced prior to diagnosis with headaches implicated most often (73.44 %). Neurocognitive comorbidities included memory difficulties (43.88 %) and aphasia (43.75 %) and psychological disorders such as depression (31.77 %) and anxiety disorders (19.92 %) were reported. Average time to diagnosis from first physician visit to diagnosis was 3.43 years, and only 8.46 % of patients had previous awareness of CM. CM I diagnosis was found incidentally for 24.87 % of participants. Common misdiagnoses were classified as psychological (19.26 %) and neurological (19.26 %). Fear was the most frequent emotion elicited at the time of correct diagnosis (42.19 %). CM I can be a challenging condition for patients and physicians, during both the search for diagnosis and management of symptoms. Patient and physician education about CM I may permit early intervention and the prevention of further deterioration and patient suffering.

BACKGROUND:To develop evidence-based treatment guidelines for Chiari malformation type 1 (CM-1), preoperative prognostic indices capable of stratifying patients for comparative trials are needed. OBJECTIVE:To develop a preoperative Chiari Severity Index (CSI) integrating the clinical and neuroimaging features most predictive of long-term patient-defined improvement in quality of life (QOL) after CM-1 surgery. METHODS:We recorded preoperative clinical (eg, headaches, myelopathic symptoms) and neuroimaging (eg, syrinx size, tonsillar descent) characteristics. Brief follow-up surveys were administered to assess overall patient-defined improvement in QOL. We used sequential sequestration to develop clinical and neuroimaging grading systems and conjunctive consolidation to integrate these indices to form the CSI. We evaluated statistical significance using the Cochran-Armitage test and discrimination using the C statistic. RESULTS:Our sample included 158 patients. Sequential sequestration identified headache characteristics and myelopathic symptoms as the most impactful clinical parameters, producing a clinical grading system with improvement rates ranging from 81% (grade 1) to 58% (grade 3) (P = .01). Based on sequential sequestration, the neuroimaging grading system included only the presence (55% improvement) or absence (74% improvement) of a syrinx ≥6 mm (P = .049). Integrating the clinical and neuroimaging indices, improvement rates for the CSI ranged from 83% (grade 1) to 45% (grade 3) (P = .002). The combined CSI had moderately better discrimination (c = 0.66) than the clinical (c = 0.62) or neuroimaging (c = 0.58) systems alone. CONCLUSION:Integrating clinical and neuroimaging characteristics, the CSI is a novel tool that predicts patient-defined improvement after CM-1 surgery. The CSI may aid preoperative counseling and stratify patients in comparative effectiveness trials. ABBREVIATIONS:CM-1, Chiari malformation type 1CSI, Chiari Severity IndexQOL, quality of life

BackgroundSyringomyelia and Chiari malformation are classified as rare diseases on Orphanet, but international guidelines on diagnostic criteria and case definition are missing. Aim of the study: to reach a consensus among international experts on controversial issues in diagnosis and treatment of Chiari 1 malformation and syringomyelia in adults.MethodsA multidisciplinary panel of the Chiari and Syringomyelia Consortium (4 neurosurgeons, 2 neurologists, 1 neuroradiologist, 1 pediatric neurologist) appointed an international Jury of experts to elaborate a consensus document. After an evidence-based review and further discussions, 63 draft statements grouped in 4 domains (definition and classification/planning/surgery/isolated syringomyelia) were formulated. A Jury of 32 experts in the field of diagnosis and treatment of Chiari and syringomyelia and patient representatives were invited to take part in a three-round Delphi process. The Jury received a structured questionnaire containing the 63 statements, each to be voted on a 4-point Likert-type scale and commented. Statements with agreement <75% were revised and entered round 2. Round 3 was face-to-face, during the Chiari Consensus Conference (Milan, November 2019).ResultsThirty-one out of 32 Jury members (6 neurologists, 4 neuroradiologists, 19 neurosurgeons, and 2 patient association representatives) participated in the consensus. After round 2, a consensus was reached on 57/63 statements (90.5%). The six difficult statements were revised and voted in round 3, and the whole set of statements was further discussed and approved.ConclusionsThe consensus document consists of 63 statements which benefited from expert discussion and fine-tuning, serving clinicians and researchers following adults with Chiari and syringomyelia.