Chiari Malformation Type I Patients Research Articles

A - 30 Chiari Malformation Type I: the Powerful Impact of Participation in Computerized Working Memory Training on Mood and Self-Perception

Abstract Objective Chiari Malformation type I (CM-I), a disorder characterized by displaced cerebellar tonsils through the foramen magnum, is neuropsychologically complex and underrecognized. While decompressive neurosurgical intervention relieves physiological symptoms, cognitive and neuropsychiatric challenges may persist. Given the importance of attentional abilities and emotional functioning for post-operative quality of life for CM-I patients, exploring the effects of CogMed Computerized Working Memory Training (CCWMT) on aforementioned elements, specifically self-perception, is essential to the neurological surgery survivorship mission. Method Three CM-I patients diagnosed in adulthood were enrolled in CCWMT approximately three years post-operatively; each with self-reported decrements in attentional functioning following neurosurgical intervention with neurocognitive measures within normal limits and moderate levels of self-reported mood dysfunction and decline in self-perceived pre-surgical functional status. Comprehensive neuropsychological testing was completed pre and post CCWMT treatment, consisting of 25 sessions of progressively challenging working memory exercises completed within 5–12 weeks. Results There were no significant decrements in objective attentional-based measures pre-CCWMT treatment relative to baseline estimates and no significant changes pre to post CCWMT treatment. However, there were clinically significant decreases in self-reported depression. Further, post-treatment improvement in self-perception of functioning aligned with degree of improvement index within the CCWMT program. Conclusions Given the positive prognosis of post-surgical CM-I patients, clinical neuropsychological investigation can maximize a patient’s quality of life by extrapolating cognitive and emotional elements that interact as barriers to optimal outcomes. This case series underscores that effortful participation in intensive cognitive remediation may enhance mood and self-perception beyond objective cognitive enhancement, thereby supporting holistic aspects of a patient’s recovery.

Impact of chronic pain and depressive symptoms on the quality of life of adults with Chiari Malformation type I: A comparative study.

Chiari Malformation type I (CM-I) is a neurological disorder characterized by cerebellar tonsillar herniation. Chronic pain, particularly headaches, is a prevalent symptom in CM-I patients, significantly impacting their quality of life. The objective of this study was to evaluate the perceived quality of life in adults with CM-I and examine the influence of chronic pain and comorbid symptoms on their well-being. 26 CM-I patients (8 with decompressive surgery) and 26 matched healthy controls were recruited. Participants completed the following questionnaires: WHOQOL-BREF, HDI, NDI, OLBPDQ and HADS. CM-I patients exhibited significantly lower scores across all domains of quality of life when compared to healthy controls. Chronic pain, including headache, neck pain, and low back pain, was more pronounced among CM-I patients and demonstrated a significant correlation with depressive symptoms. Notably, after controlling for chronic pain, the differences in quality of life between CM-I patients and controls diminished. The results suggest that chronic pain, especially headaches, and comorbid depressive symptoms exert a substantial impact on the quality of life of CM-I patients. Surgical intervention alone may not fully address these issues, highlighting the importance of considering psychological interventions as part of the comprehensive treatment. Further research with larger samples and pre-post-surgery assessments is needed to validate these findings and explore the potential benefits of psychological therapies in enhancing the quality of life for CM-I patients.

The Surgical Strategy Cerebrospinal Fluid Decompression Facilitates Outcomes of Adults with Chiari Malformation Type I: An Observational, Real-World, Single-Center Study of 528 Patients

ObjectivesWe designed this study to introduce the surgical strategy “CSF decompression” in treating Chiari malformation type I (CMI), and compared the “CSF decompression” strategy with other surgical strategies to provide a solid basis for patient counseling. MethodsA total of 528 consecutive CMI patients who underwent surgical interventions from 2012 to 2022 were enrolled. The surgical strategy for these patients was bony and dural decompression (BDD), anatomical reduction of herniated tonsils (AR) or CSF decompression (CSFD). Short-term results were determined after 3 months; long-term outcomes were evaluated at last follow-up and at least 18 months. ResultsThe CSFD strategy was independently associated with better long- or short-term primary outcomes than AR or BDD (P < 0.001). Compared with short-term, the long-term outcomes were better in CSFD patients (P = 0.035), but were worse in BDD patients (P = 0.03). Specific surgical techniques cannot affect the long- and short-term outcomes of CMI patients. CSFD provided better long-term syringomyelia improvement than short-term (181/218, 83% vs 169/218, 77.5%; P < 0.001). ConclusionThe “CSF decompression” surgical strategy, but not a specific surgical technique or operative method, was associated with favorable neurological outcomes in adult CMI patients. The surgical technique and operative method should be selected according to the characteristics of each patient and the intraoperative condition to normalized CSF circulation at CVJ. The intraoperative target maybe smoothly CSF flow, out from the fourth ventricle and in to the bilateral Luschka foramina, could be observed.

Successful treatment of unilateral facial nerve palsy in a pediatric patient with syringobulbia and Chiari malformation type I: illustrative case.

Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy. A 7-year-old male presented with right facial weakness in addition to headaches, ataxia, urinary incontinence, and falls. Magnetic resonance imaging revealed CMI with a syrinx of the cervicothoracic spine and syringobulbia. Posterior fossa decompression with duraplasty was performed without complications, and the patient was discharged home on postoperation day 5. At the 3-week follow-up, the patient's neurological deficits had largely subsided. At the 3-month follow-up, his CN VII palsy and syringobulbia had completely resolved. Pediatric CMI patients with syringomyelia are at risk for developing syringobulbia and brainstem deficits, including unilateral facial palsy. However, craniocervical decompression can prove successful in treating such deficits.

Individualized Functional Decompression Options for Adult Chiari Malformation With Syringomyelia and A Novel Scale for Syringomyelia Resolution: A Single-Center Experience.

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. 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%. 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). 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.

A long-term follow-up study of adults with Chiari malformation type I combined with syringomyelia.

There is a considerable amount of controversy regarding the treatment and prognosis of adult patients with Chiari malformation type I (CM-I) at home and abroad; furthermore, no large-sample, long-term, follow-up studies have examined CM-I patients with syringomyelia (SM) comparing posterior fossa decompression with resection of tonsils (PFDRT) vs. posterior fossa decompression with duraplasty (PFDD). This study retrospectively analyzed the factors affecting the treatment and long-term prognosis of adults with CM-I combined with SM. We retrospectively analyzed data from 158 adult CM-I patients combined with SM who underwent PFDRT or PFDD, including 68 patients in group PFDRT and 90 patients in group PFDD. We examined the clinical manifestations, imaging features, and follow-up data of patients. Clinical outcomes were assessed using the Chicago Chiari Outcomes Scale (CCOS), and radiographic outcomes were indicated by the syrinx remission rate. Multivariate logistic regression analysis and multiple linear regression analysis were used to explore the relevant factors affecting the long-term prognosis of patients. This study showed that compared with preoperative patients in the PFDRT group and PFDD group, the sensory impairment, cough-related headache, and movement disorder were significantly improved (p < 0.01); meanwhile, the diameter of the syrinx and the volume of the syrinx decreased significantly (p < 0.001). Additionally, the study found that there were significant differences in the syrinx remission rate (p = 0.032) and the clinical cure rates (p = 0.003) between the two groups. Multivariate logistic regression analysis showed that age (p = 0.021), cerebellar-related symptoms (p = 0.044), preoperative cisterna magna volume (p = 0.043), and peak systolic velocity (p = 0.036) were independent factors for clinical outcomes. Multiple linear regression analysis showed that different surgical procedures were positively correlated with the syrinx remission rate (p = 0.014), while preoperative syrinx diameter (p = 0.018) and age (p = 0.002) were negatively correlated with the syrinx remission rate. In conclusion, this study suggested that, in a long-term follow-up, although both surgical procedures are effective in treating patients with CM-I and SM, PFDRT is better than PFDD; age and cerebellar-related signs independently affect the patient's prognosis. Additionally, an effective prognosis evaluation index can be developed for patients, which is based on imaging characteristics, such as preoperative cisterna magna volume, preoperative syrinx diameter, and preoperative cerebrospinal fluid (CSF) hydrodynamic parameters to guide clinical work.

The role of traditional social determinants of health in referral patterns, timing of surgery, and Chiari Health Index for Pediatrics-determined quality of life in children with symptomatic Chiari type I malformation.

Understanding the impact of the social determinants of health on the utilization of healthcare resources is an important step in eliminating inequalities. The goal of this study was to determine the role of social determinants of health in referral patterns, timing of consultation/intervention, and quality of life in children with Chiari malformation type I (CM-I). A retrospective study was conducted of children aged 0 to 18 years who underwent surgical treatment for CM-I at a single pediatric facility from 2015 to 2019. The variables included demographic and socioeconomic characteristics, referral patterns, timing, and quality of life data based on the Chiari Health Index for Pediatrics (CHIP). The cohort consisted of 103 surgically treated CM-I patients. No differences were seen in race, sex, insurance, or household income when evaluating referral source (community, specialist, or emergency department) or when comparing patients with incidental versus symptomatic findings. In the evaluation of timing from initial evaluation to surgery, no statistical differences were seen between racial, sex, insurance status, or income groups. Children from households of lower median family income were significantly more likely to report pain at the time of consultation (pain group median [interquartile range] $46,660 [$41,004-$50,367] vs nonpain group $53,604 [$41,427-$59,828], p = 0.004). Those in the lower-income group also reported lower CHIP scores corresponding to increased symptomatology in the nonpain physical symptoms (p = 0.004) and psychosocial domains (p = 0.018). There was no evidence of a difference in referral patterns or a delay in time from clinic presentation to surgery based on the traditional social determinants of health categories. Children from households in the lower-income group were associated with increased severity of pain and nonpain symptoms.

Pain-depression relationship, quality of life and acceptance of illness among patients with Chiari malformation type I: A cross-sectional study.

Chiari malformation type I (CM-I) is a congenital anomaly of the hindbrain. The most common symptoms include suboccipital tussive headache, dizziness and neck pain. Recently there has been growing interest in the psychological and psychiatric aspects of functioning in patients with CM-I, which have a significant effect on treatment outcomes and quality of life (QoL). The aim of the study was to assess the severity of depressive symptoms and the QoL in patients with CM-I and to identify the main factors responsible for these phenomena. A total of 178 people participated in the study and were divided into 3 groups: patients with CM-I who had undergone surgery (n = 59); patients with CM-I who had not undergone surgery (n = 63); and healthy volunteers (n = 56). Psychological evaluation included a set of questionnaires: the Beck Depression Inventory II, the World Health Organization shortened version of the WHOQOL-100 quality of life questionnaire, the Acceptance of Illness Scale and the Beliefs about Pain Control Questionnaire. Results showed that the control group participants obtained significantly better results than both groups of CM-I patients in terms of all indicators of QoL, symptoms of depression, acceptance of illness, pain level (average and present) and perceived influence of doctors regarding coping with pain. Patients with CM-I (operated and non-operated) obtained similar results in most of the questionnaires and the QoL indices correlated significantly with the majority of the analyzed variables. Moreover, CM-I patients with higher depression scores described their pain as more severe and had a stronger belief that pain levels were not influenced by them but only by doctors, or that it could be controlled randomly; they were also less willing to accept their illness. CM-I symptoms affect the mood and QoL of patients. Psychological and psychiatric care should be the golden standard in managing this clinical group.

Analysis of the Prechiasmatic Sulcus in Chiari Malformation Type I

To assess morphologic features of the prechiasmatic sulcus (PS) in Chiari malformation type I (CM-I). Computed tomography images were obtained in patients with CM-1 (mean age: 21.26 ± 16.46 years; sex: 25 females and 17 males) and 58 healthy control subjects (mean age: 28.12 ± 22.60 years; sex: 29 females and 29 males). The following parameters were measured to determine the anatomy of the PS: planum length, sulcal length, sulcal angle (SA), and interoptic distance. Considering sulcal length and SA measurements, 4 types of PS were identified: narrow-steep-groove (NSG), narrow-flat-groove (NFG), wide-steep-groove, and wide-flat-groove. SA was greater in the CM-I group (32.80 ± 18.21°) compared with the control group (23.05 ± 14.53°) (P= 0.004), but the other parameters were similar in both groups. Distribution ranking of PS types was as follows: NFG (31%) > NSG (26.20%) > wide-steep-groove (23.80%) > wide-flat-groove (19%) in the CM-I group; wide-flat-groove (37.90%) > wide-steep-groove (20.70%)= NFG (20.70%)= NSG (20.70%) in the control group. Distribution rate of PS types relative to both groups showed that the classification system was not affected by CM-I (P= 0.226). SA value was approximately 25% greater in the CM-I group than in the control group; thus, CM-I patients had more vertical groove. The percentage of narrow-type (NSG+ NFG) PS in the CM-I group was higher than in the control group.

Assessment of the Anterior Clinoid Process and Optic Strut in Chiari Malformation Type I: A Computed Tomography Study.

Objective This study aimed to evaluate morphological features of the anterior clinoid process (ACP) and the optic strut (OS) in Chiari malformation Type I (CM-I). Methods The study universe consisted of computed tomography images of 41 CM-I patients and 45 normal subjects. Comparison of the parameters for CM-I and the control group was performed with the Student's t -test. A " p < 0.05" was accepted as the significance level. Results ACP length was smaller in CM-I than the control group ( p < 0.001). In contrast to ACP length, ACP angle ( p < 0.001), OS length ( p = 0.022), and the distance between ACP and OS ( p = 0.020) were found greater in CM-I in comparison to the control group ( p < 0.05). ACP width ( p = 0.233) and OS width ( p = 0.376) were similar in both groups. ACP pneumatization in CM-I group was found as 12.20%, whereas in the control group as 8.90%. Two different types about the pneumatization were identified in CM-I group (Type 1: 4.9% and Type 2: 7.3%), whereas three different types in the control group (Type 1: 3.3%, Type 2: 4.4%, and Type 3: 1.1%). Relative to ACP, three different types about OS position were identified in CM-I group (Type C: 31.70%, Type D: 64.60%, and Type E: 3.70%) and the control group (Type C: 7.80%, Type D: 64.40%, and Type E: 27.80%). Conclusion Shorter ACP, wide-angled ACP, longer OS, and more anteriorly located OS were found in CM-I group compared with the normal group. Our findings showed that the pneumatization of ACP was not affected by CM-I.

442 Reversible Cognitive Dysfunction and Neuropsychiatric Symptoms in Patients With Chiari Malformation Type I

INTRODUCTION: Chiari malformation type I (CMI) is a congenital/acquired structural condition where the cerebellar tonsils herniate through the foramen magnum, resulting in cerebellar and lower medulla/upper cervical spinal cord compression. Symptoms typically include occipital headaches, however, additional symptoms have been reported by symptomatic patients. We have observed a proportion reporting cognitive dysfunction and neuropsychiatric symptoms, which appear to improve following posterior fossa decompression (PFD). METHODS: Patients completed comprehensive neuropsychological evaluations, including a 90-minute standardized assessment of cognitive function across multiple domains and a self-reported assessment of neuropsychiatric symptoms, pre- and approximately 6-months post-surgery. RESULTS: This prospective study included patients with CMI who opted to undergo PFD surgery and reported cognitive or neuropsychiatric impairment between 2015 to 2021. All patients completed a comprehensive pre-operative neuropsychological assessment. A parallel assessment was completed at 6-month post-operative follow-up. 112 patients (Mage = 35.1 years; SD = 10.4) were included in pre-operative analyses, and of these, 31 patients (Mage = 32.5 years; SD = 10.5) were included in paired pre-post-operative analyses. Pre-operatively, CMI patients performed significantly worse than a standard norm on measures of executive function, attention, visuospatial memory, verbal memory, and neuropsychiatric symptoms. CONCLUSIONS: This is the first study to 1) demonstrate significant impairments in cognitive function and neuropsychiatric symptoms in CMI patients pre-operatively, and 2) show significant improvements in these domains at post-operative follow-up. This work calls attention to a significant proportion of CMI patients who suffer debilitating and life altering symptoms and provides compelling evidence for a role of the cerebellum in such conditions. In addition to CMI, cerebellar pathology should be considered as potential neuroanatomical site for patients with cognitive and neuropsychiatric impairment from other etiologies.

136 Morphological Clusters of Chiari I Patients With Variable Response After Suboccipital Decompression

INTRODUCTION: Chiari malformation type I (CM-I) can cause debilitating neurologic symptoms, most notably distinctive headaches, and the only existing definitive treatment is suboccipital decompression. While CM-I is typically defined as ≥5 mm downward displacement of cerebellar tonsils, overall CM-I cerebellar morphology is highly heterogeneous, and it is uncertain if this variability represents distinct patient populations or influences clinical response to suboccipital decompression. METHODS: 186 adult CM-I patients with high-resolution T1-MRIs were retrospectively identified. Each patient’s cerebellum was non-linearly and diffeomorphically transformed to template brain, with this transformation’s inverse representing cerebellar deformation. A similarity matrix was generated between patients’ deformations using the Pearson correlation coefficient of their inverse transformations’ log Jacobian determinants within each cerebellar lobule. Spectral clustering was performed on this similarity matrix to identify patient clusters. Headache freedom after suboccipital decompression was compared between clusters by Kaplan-Meier analysis. RESULTS: Three morphological clusters of CM-I patients were identified. Cluster A is characterized by posteroinferior cerebellar fullness and platybasia; B by midline anterior-posterior compression; C solely by tonsillar descent. After suboccipital decompression, headaches were found to recur more frequently and sooner in Cluster B (p &lt; 0.01). CONCLUSIONS: These analyses suggest that CM-I encapsulates morphologically distinct subtypes, here identified as Cluster A and Cluster B, with Cluster C representing an intermediate phenotype. These clusters vary significantly in clinical response to suboccipital decompression, which may have implications for surgical patient selection or operative technique. Future research should involve independent validation of this cluster scheme and optimization of treatment for patients in Cluster B, which is found here to have poorer surgical outcomes.

Reappraisal of intradural findings in Chiari malformation type I.

Management of Chiari malformation type I (CM-I) requires the functional restoration of an obstructed cisterna magna. In posterior fossa decompression with duraplasty (PFDD), various intradural pathologies are suggested to alter CSF flow at the craniocervical junction and require surgical correction. However, reports of the spectrum of intraoperative intradural findings and their nuances are scarce, especially those characterizing rarer findings pertaining to the vascular structures and vascular compression. The authors conducted a retrospective cohort analysis of adults and children who underwent first-time PFDD for CM-I (2011-2021), with and without syringomyelia. The surgical reports and intraoperative videos were reviewed, and the frequency and nature of the intradural observations in regard to the tonsils, arachnoid, and vasculature were analyzed along with the clinical findings and surgical outcomes. All 180 patients (age range 1-72 years; median [interquartile range] 24 (14-38) years; 37% of patients were children < 21 years of age) exhibited multiple intradural findings, with a median of 7 distinct concurrent observations in each patient. Novel findings not previously reported included posterior inferior communicating artery (PICA) branches compressing the neural elements at the cervicomedullary junction (26.7%). Other common findings included arachnoid adhesions (92.8%), thickening (90.6%), webs at the obex (52.2%), tonsillar gliosis (57.2%), tonsillar hypertrophy (18.3%), adhesions obstructing the foramen of Magendie (FoM) (62.2%), PICA obstruction of the FoM (17.2%), and dural scarring (87.8%). Tonsillar gliosis and intertonsillar adhesions obstructing the FoM were more common in children than adults. Tonsillar gliosis and arachnoid webs were more common among syringomyelia patients. After multivariable adjustment, none of the observed findings were independently associated with syringomyelia, preoperative symptoms, or postoperative improvement. The vast majority of patients improved postoperatively. The complication rate was low: 1.2% of patients required revision PFDD at > 3 years postoperatively, 3.6% experienced other operative complications, and 0% had CSF leaks. The diversity of intradural findings and observations revealed in this study suggests that obstructive and compressive structural anomalies may be more common than previously reported among CM-I patients, both those patients with and those without syringomyelia and especially those with obstructive and compressive PICA branches. Although the authors cannot conclude that all these findings are necessarily pathological, further study may determine how they contribute to CM-I pathology and symptomatology in the setting of a compromised cisterna magna.

Cerebellar tonsil reduction for surgical treatment of Chiari malformation type I in children.

The goal of this study was to review the efficacy and safety of different surgical techniques used for treatment of Chiari malformation type I (CM-I) in children. The authors retrospectively reviewed 437 consecutive children surgically treated for CM-I. Procedures were classified into four groups: bone decompression (posterior fossa decompression [PFD]) and duraplasty (PFD with duraplasty [PFDD]), PFDD with arachnoid dissection (PFDD+AD), PFDD with tonsil coagulation of at least one cerebellar tonsil (PFDD+TC), and PFDD with subpial tonsil resection of at least one tonsil (PFDD+TR). Efficacy was measured as a greater than 50% reduction in the syrinx by length or anteroposterior width, patient-reported improvement in symptoms, and rate of reoperation. Safety was measured as the rate of postoperative complications. The mean patient age was 8.4 years (range 3 months to 18 years). In total, 221 (50.6%) patients had syringomyelia. The mean follow-up was 31.1 months (range 3-199 months), and there was no statistically significant difference between groups (p = 0.474). Preoperatively, univariate analysis showed that non-Chiari headache, hydrocephalus, tonsil length, and distance from the opisthion to brainstem were associated with the surgical technique used. Multivariate analysis demonstrated that hydrocephalus was independently associated with PFD+AD (p = 0.028), tonsil length was independently associated with PFD+TC (p = 0.001) and PFD+TR (p = 0.044), and non-Chiari headache was inversely associated with PFD+TR (p = 0.001). In the treatment groups postoperatively, symptoms improved in 57/69 (82.6%) PFDD patients, 20/21 (95.2%) PFDD+AD patients, 79/90 (87.8%) PFDD+TC patients, and 231/257 (89.9%) PFDD+TR patients, and differences between groups were not statistically significant. Similarly, there was no statistically significant difference in postoperative Chicago Chiari Outcome Scale scores between groups (p = 0.174). Syringomyelia improved in 79.8% of PFDD+TC/TR patients versus only 58.7% of PFDD+AD patients (p = 0.003). PFDD+TC/TR remained independently associated with improved syrinx outcomes (p = 0.005) after controlling for which surgeon performed the operation. For those patients whose syrinx did not resolve, no statistically significant differences between surgery groups were observed in the length of follow-up or time to reoperation. Overall, there was no statistically significant difference between groups in postoperative complication rates, including aseptic meningitis and CSF- and wound-related issues, or reoperation rates. In this single-center retrospective series, cerebellar tonsil reduction, by either coagulation or subpial resection, resulted in superior reduction of syringomyelia in pediatric CM-I patients, without increased complications.

Exploring the prognostic differences in patients of Chiari malformation type I with syringomyelia undergoing different surgical methods.

The best surgical treatment of Chiari malformation patients with syringomyelia remains controversial, and whether cerebellar tonsillectomy should be performed has not been decided. To evaluate the efficacy of posterior fossa decompression with duraplasty (PFDD) and Posterior fossa decompression with resection of tonsils (PFDRT) in patients of Chiari malformation type I (CM-I) with syringomyelia and explore relevant factors affecting prognosis. We retrospectively analyzed 182 adult patients of CM-I with syringomyelia who underwent PFDD or PFDRT over a 6-year period, and analyzed their clinical manifestations, imaging features, and follow-up data. Clinical outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), and imaging outcomes were assessed using the syrinx remission rate. Difference comparisons were performed to compare the differences between different surgical groups. Influencing factors associated with outcome were investigated using bivariate analysis and multiple linear regression analysis. There were statistically significant differences in CCOS score (p = 0.034) and syrinx remission rates (p = 0.046) between the PFDRT group and the PFDD group after surgery. Regression analysis showed that preoperative motor dysfunction, cerebellar-related symptoms and different surgical methods may have influenced the CCOS score and that brainstem-related symptoms and age may have influenced the syrinx remission rates in the total patient group (p < 0.05). Regression analysis showed that the duration of symptoms, cerebellar-related symptoms and preoperative syrinx diameter may have influenced the CCOS score and that the preoperative cerebellar tonsillar hernia distance may have influenced the postoperative syrinx remission rate in the PFDRT group (p < 0.05). Age and length of hospital stay may have influenced the CCOS score, and brainstem-related symptoms and age may have influenced the syrinx remission rates in the PFDD group (p < 0.05). This study showed that the CCOS score in the PFDRT group was better than that in the PFDD group. Preoperative motor dysfunction, cerebellar-related symptoms, and different surgical methods in patients of CM-I with syringomyelia affected postoperative CCOS score. Both the duration of symptoms and the age of the patients should be actively considered as factors influencing prognosis. Symptomatic CM-I patients with syringomyelia should undergo surgical treatment as early as possible.

Posterior fossa decompression and duraplasty with and without tonsillar resection for the treatment of adult Chiari malformation type I and syringomyelia

The current surgical management of adult Chiari malformation type I (CM-I) with associated syringomyelia remains controversial. The objective of this study was to explore posterior fossa decompression and duraplasty (PFDD) with and without tonsillar resection in adult patients with CM-I and syringomyelia. A total of 116 adult patients suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression at our institution between 2012 and 2020 were randomly divided into 2 groups: the PFDD group (n = 64) underwent PFDD without tonsillar resection, while the PFDD-T group (n = 52) underwent PFDD with tonsillar resection. The primary outcome was improvement or resolution of the syrinx. The secondary outcome was an improvement in clinical outcome based on Chicago Chiari Outcome Scale (CCOS) scores. All participants were followed-up to 1-year postoperatively. The proportions of patients who had >20% improvement in syrinx size were 60.9% and 78.8% in the PFDD and PFDD-T groups, respectively (P = .038). The improvement in clinical outcome based on CCOS scores was significantly different in the 2 groups (P = .004). The functionality sub-score was significantly different between the 2 groups (P = .027), but there were no significant differences in the pain symptoms, non-pain symptoms, and complications sub-scores. The total CCOS scores were higher in the PFDD-T group than in the PFDD group (P = .037). This study determined the role of tonsillar resection in achieving obvious syrinx improvement following PFDD-T. PFDD with tonsillar resection seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia.

A Novel Craniocervical Junction Compression Severity Index-Based Grading System for Multidirectional Quantification of the Biomechanics at Foramen Magnum of Chiari Malformation Type I.

Objective This study aimed to establish a novel grading system, based on the craniovertebral junction compression severity index (CVJCSI) for multidirectional quantification at the foramen magnum plane for Chiari malformation type I (CMI). Methods The CVJCSI grading system was established to stratify patients based on the ventral (modified clivoaxial angle < 138°), dorsal (tonsil herniation), and central (brainstem herniation) CVJ (craniovertebral junction) compression, the CVJCSI grading system was established to stratify patients. The optimal surgical method for each grade was recommended by intragroup comparisons regarding the efficacy of the three operations. Finally, according to the CVJCSI grading system, a prospective validation trial was performed and surgically treated for internal validation. Results Based on the retrospective study ( n = 310), the CVJCSI included six grades: I: syrinx alone without compression; II: dorsal compression; III: dorsal and central compression; IV: ventral compression; V: dorsal and ventral compression; and VI: ventral, dorsal, and central compression. Among all available variables, only the CVJCSI and surgical methods significantly affected the CCOS. The CCOS scores, overall and for each CVJCSI grade, increased in the prospective cohort ( n = 42) compared with that in the retrospective analysis. Conclusions The CVJCSI can be used to stratify CMI patients. The higher the CVJCSI grade, the more severe the CVJ compression and the worse posterior fossa deformity. Meanwhile, the CVJCSI was negatively correlated with the CCOS. The lower the CVJCSI grade, the better the response to surgery, and the less-invasive surgical procedures were warranted. Finally, the prospective cohort study validated the proposed CVJCSI-based surgical protocols.

Association between synthetic sealants and increased complication rates in posterior fossa decompression with duraplasty for Chiari malformations regardless of graft type.

Dural sealants are commonly used in posterior fossa decompression with duraplasty (PFDD) for Chiari malformation type I (CMI). Prior evidence suggests that combining certain sealants with some graft material is associated with an increased rate of complications. In 2018, the authors noted an increased rate of symptomatic pseudomeningocele and aseptic meningitis after PFDD in CMI patients. The authors utilized retrospective and prospective analyses to test the hypothesis that complication rates increase with the use or combination of certain sealants and grafts. The analysis was split into 2 periods. The authors retrospectively reviewed patients who underwent PFDD for CMI at their center between August 12, 2011, and December 31, 2018. The authors then eliminated use of DuraSeal on the basis of the retrospective analysis and prospectively examined complication rates from January 1, 2019, to August 4, 2021. The authors defined a complication as symptomatic pseudomeningocele, bacterial or aseptic meningitis, cerebrospinal fluid leak, subdural hygroma, hydrocephalus, surgical site infection, or wound dehiscence. From 2011 to 2018, complications occurred in 24.5% of 110 patients. Sealant choice was correlated with complication rates: no sealant (0%), Tisseel (6%), and DuraSeal (15.3%) (p < 0.001). No difference in complication rate was noted on the basis of choice of graft material (p = 0.844). After eliminating DuraSeal, the authors followed 40 patients who underwent PFDD after 2018. The complication rate decreased to 12.5%. All complications after 2018 were associated with Tisseel. At the authors' single center, use of sealants in PFDD surgery for CMI, especially DuraSeal, was correlated with a higher complication rate. Eliminating DuraSeal led to a significant decrease in the rate of symptomatic pseudomeningocele and aseptic meningitis.

Phenotypes and Prognostic Factors of Syringomyelia in Single-Center Patients With Chiari I Malformation: Moniliform Type as a Special Configuration.

ObjectiveThe 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. MethodsA 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. ResultsThere 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. ConclusionTimely decompression surgery could achieve a better outcome in CM-I patients with syringomyelia. Moniliform syringomyelia may suggest a relatively better prognosis.

Impact of sleep-disordered breathing on the management of children with Chiari malformation type I.

Children with Chiari malformation type I (CM-I) have an increased risk of sleep apnea. The aim of the study was to describe the management of CM-I patients in relation to symptoms, magnetic resonance imaging (MRI) findings and sleep apnea syndrome (SAS). We performed a retrospective analysis of clinical charts of all 57 CM-I patients seen between September 2013 and April 2017. A total of 45 patients had isolated CM-I or associated co-morbidity (CM-Iia), 5 had craniosynostosis (CM-Ics), and 7 a polymalformative syndrome (CM-Ipm). The prevalence of SAS, defined as an apnea-hypopnea index >1 event/h, was high in CM-I ranging from 50% to 80% according to the CM-I group. The prevalence of central sleep apnea (CSA) was low, with 5 (9%) patients having CSA and only 3 patients with CM-Iia having isolated CSA. A total of 17 patients (30%) had foramen magnum decompression (FMD). Neither positive symptoms of CM-I nor MRI findings alone, nor both combined were good indicators for FMD. No correlation was observed between the cerebellar tonsil descent and SAS in CM-I. But all 5 patients with CSA had a FMD. The combination of MRI findings and/or symptoms of CM-I together with moderate-to-severe SAS best discriminated patients who needed a FMD. Our findings highlight the importance of a combined evaluation of symptoms, MRI and polygraphy results in the management of CM-I patients.