Chiari Malformation Research Articles

A Staged Approach for Surgical Management of Basilar Invagination

BACKGROUND AND OBJECTIVES: Patients with basilar invagination (BI) can be treated with several surgical options, ranging from simple posterior decompression to circumferential decompression and fusion. Here, we aimed at examining the indications and outcomes associated with these surgical strategies to devise a staged algorithm for managing BI. METHODS: We conducted a retrospective cohort study in 2 neurosurgical centers and included patients with a BI, as defined by a position of the dens tip at least 5 mm above the Chamberlain line. Other craniovertebral junction anomalies, such as atlas assimilation, platybasia, and Chiari malformations, were documented. C1-C2 stability was assessed with a dynamic computed tomography scan. RESULTS: We included 30 patients with BI with a mean follow-up of 56 months (min = 12, max = 166). Posterior decompression and fusion (n = 8) was only performed in cases of obvious atlanto-axial instability (eg, increased atlanto-dental interval or hypermobility on flexion/extension), while anterior decompression (transoral or transnasal) was reserved to patients with lower cranial nerves deficits (eg, swallowing dysfunction) and irreducible anterior compression (n = 9). Patients with posterior signs (eg, Valsalva headaches) or myelopathy but without C1-C2 instability nor anterior signs were managed with an isolated foramen magnum decompression, with or without duraplasty (n = 13). Complications were more frequent for combined procedures, including neurological deterioriation (n = 4) and tracheostomy (n = 2), but reinterventions were more likely in patients undergoing posterior decompression alone (n = 3). CONCLUSION: Patient selection is key to determine the appropriate surgical strategy for BI: In our experience, combined approaches are only needed for patients with irreducible and symptomatic anterior compression, while fusion should be restricted to patient with obvious signs of atlanto-axial instability. Other BI patients can be managed by foramen magnum decompression alone to minimize surgical morbidity.

Headache to a Patient with the Coexistence of Chiari I Malformations and Primary Empty Sella Syndrome: A Case Report la Syndrome: A Case Report

Background: Chiari I malformations (CM-I) and Primary Empty Sella syndrome (PESS) are rare disorders of structural defects within a skull. Headache is the most common presenting symptom in both conditions. However, CM-I and ESS comorbidity in patients with severe headaches is yet to be reported. Case description: The patient was a 38-year-old woman with a one-year history of headaches at various localisations (occipital and bilateral frontal) preceded by mood agitation. Physical examination was significant for obesity. Magnetic resonance imaging (MRI) revealed a 6.4 mm cerebellar tonsil descent through the foramen magnum and hypointense (CSF-filled) flattened sella. The lipid panel showed elevated cholesterols (total) and low-density lipoprotein (LDL) levels.  She was medically managed, with no report of symptoms relapses at one-month and two-month follow-ups. Conclusion: This is the first report on comorbid CM-I and PESS in adults presenting with cephalalgia. PESS and CM-I co-occurrence may present with mixed headache localisation; careful history-taking and imaging are mandatory for diagnosis confirmation. Future extensive studies are warranted to analyse the pathophysiological interplay between these two rare disorders.

Familial Chiari malformation: a systematic review and illustrative cases.

Chiari malformations (CMs) are a group of congenital or acquired disorders characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa. CM is considered largely sporadic-however, there exists growing evidence of transmissible genetic underpinnings. The purpose of this systematic review of all familial studies of CM was to investigate the existence of an inherited component and provide recommendations to manage and monitor at-risk family members. This paper includes the following: 1) a unique case report of dizygotic twins who presented at the Toronto Western Hospital Spinal Cord Clinic with symptomatic CM type 1 (CM-1) and syringomyelia; and 2) a systematic review of familial CM. The EMBASE and MEDLINE databases were searched on June 27, 2023, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Only articles in the English language concerning the diagnosis of CM in > 1 human family member presented as a case study, case series, or literature review were included. Among the 29 articles included in the final analysis, a total of 34 families with CM were analyzed. An average of 3 cases of CM were found per family among all generations. Eighty-one cases (88%) reported CM-1, whereas the other 11 (12%) cases reported either CM-0, CM-1.5, or tonsillar ectopia. A syrinx was present in 37 (54%) cases, with 14 (38%) of these patients also reporting a skeletal abnormality, the most common comorbidity. Most family members diagnosed with CM were siblings (18; 35%), followed by monozygotic twins/triplets (12; 23%). Patients most often presented with headaches, sensory disturbances, or generalized symptoms. Overall, there exists mounting evidence for a hereditary component of CM. It is unlikely to be explained by a classic mendelian inheritance pattern, but is rather a polygenic architecture influenced by variable penetrance, cosegregation, and entirely nongenetic factors. For first-degree relatives of those affected by CM, the authors' findings may influence clinicians to conduct closer clinical and radiographic monitoring, promote patient education, and consider earlier genetic testing.

872 Natural History of Conservatively Managed Patients With Chiari Malformation Type I and Syringomyelia

INTRODUCTION: Chiari malformation type 1 (CM1) describes caudal ectopia of the cerebellar tonsils. Though often asymptomatic earlier in life and found incidentally on imaging, CM1 can disturb cerebrospinal fluid (CSF) flow and generate an array of symptoms. Surgical treatment for CM1 centers around procedures to increase the posterior fossa volume and improve CSF circulation. Many surgeons elect to operate on CM1 patients in the setting of a syrinx, making it difficult to understand the natural history of these patients. METHODS: Retrospective study of 37 CM1 patients with syrinx who were managed conservatively at our institution. All patients had initial and follow-up clinic visits with a neurologist or neurosurgeon. Of the 37 patients, 27 had initial and follow-up MRI images at least six months apart. Student t-tests were used to compare radiological parameters. RESULTS: A total of three patients saw complete resolution of their syrinxes. Regarding clinical symptomatology, 19 out of 32 patients who were initially symptomatic saw clinical improvement by follow up, while 13 remained stable, and five declined. Of the five patients that declined, three had worsening neurological conditions not attributed to the syrinx or CM1 while the remaining two patients were the only patients that declined surgery despite recommendation. A total of 16 patients saw decreases in both syrinx size and tonsillar descent. There were no statistically significant differences between tonsillar descent, syrinx size, and number of vertebral bodies spanned by syrinx between the initial and latest follow up. The presence of tonsillar pegging was predictive of decrease in syrinx size. Tonsillar descent and syrinx size were not predictive of clinical outcomes. CONCLUSIONS: Although the surgical treatment of the CM1 patient successfully resolves the syrinx, the natural history of the syrinx can be benign.

Machine learning for enhanced prognostication: predicting 30-day outcomes following posterior fossa decompression surgery for Chiari malformation type I in a pediatric cohort.

Chiari malformation type I (CM-I) is a congenital disorder occurring in 0.1% of the population. In symptomatic cases, surgery with posterior fossa decompression (PFD) is the treatment of choice. Surgery is, however, associated with peri- and postoperative complications that may require readmission or renewed surgical intervention. Given the associated financial costs and the impact on patients' well-being, there is a need for predictive tools that can assess the likelihood of such adverse events. The aim of this study was therefore to leverage machine learning algorithms to develop a predictive model for 30-day readmissions and reoperations after PFD in pediatric patients with CM-I. This was a retrospective study based on data from the National Surgical Quality Improvement Program-Pediatric database. Eligible patients were those undergoing PFD (Current Procedural Terminology code 61343) for CM-I between 2012 and 2021. Patients undergoing surgery for tumors or vascular lesions were excluded. Unplanned 30-day readmission and unplanned 30-day reoperation were the main study outcomes. Additional outcome data considered included the length of hospital stay, 30-day complications, discharge disposition, and 30-day mortality. Training and testing samples were randomly generated (80:20) to study the 30-day readmission and reoperation using logistic regression, decision tree, random forest (RF), K-nearest neighbors, and Gaussian naive Bayes algorithms. A total of 7106 pediatric patients undergoing PFD were included. The median age was 9.2 years (IQR 4.7, 14.2 years). Most of the patients were female (56%). The 30-day readmission and reoperation rates were 7.5% and 3.4%, respectively. Headaches (32%) and wound-related complications (30%) were the most common reasons for 30-day readmission, while wound revisions and evacuation of fluid or blood (62%), followed by CSF diversion-related procedures (28%), were the most common reasons for 30-day reoperation. RF classifiers had the highest predictive accuracy for both 30-day readmissions (area under the curve [AUC] 0.960) and reoperations (AUC 0.990) compared with the other models. On feature importance analysis, sex, developmental delay, ethnicity, respiratory disease, premature birth, hydrocephalus, and congenital/genetic anomaly were some of the variables contributing the most to both RF models. Using a large-scale nationwide dataset, machine learning models for the prediction of both 30-day readmissions and reoperations were developed and achieved high accuracy. This highlights the utility of machine learning in risk stratification and surgical decision-making for pediatric CM-I.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

274 Predicting Reoperation in Surgically Managed Pediatric Chiari I Malformation

INTRODUCTION: Chiari I malformation (CMI) involves the herniation of cerebellar tonsils into the spinal canal, without brainstem involvement. When patients are symptomatic, surgical decompression is recommended, but reoperation rates can be as high as 30%. METHODS: Patients were queried from the 2007-2016 MarketScan Databases with the CPT code (61343) for CM decompression. To be included, a patient had to have been diagnosed with CMI, received decompressive surgery, and be eighteen or younger at the time of first operation. Patients were excluded if they had a diagnosis of any other class of Chiari malformation at any point. Next, the Elixhauser Comorbidity Index was constructed for each patient. These variables were then used as covariates in a multivariate regression model to predict reoperation following surgical decompression. Odds ratios, their corresponding confidence intervals, and metrics of significance were determined. RESULTS: 2,823 patients were included, with an average age of 10.4 (SD = 5.4). 169 (6.0%) patients underwent reoperations. As patient age at first operation increased, their reoperation rate decreased steadily (OR = 0.95, 95% CI = 0.93-0.98, p = 0.002). This association was primarily driven by a sharp decline in reoperation rate after the first three years of life. Patients in their first year had a mean reoperation rate of 17.6%; by age three, the reoperation rate declined to 5.2%, remaining fairly stable from age four (5.1%) to eighteen (4.9%). Use of duraplasty (OR = 0.75, 95% CI = 0.52-1.06, p = 0.112) did not alter the likelihood of reoperation. CONCLUSIONS: In a national pediatric cohort of patients with surgically managed Chiari I malformations, there was a significantly increased likelihood of reoperation in younger children. Our findings suggest that, when medically feasible, initial CMI surgery should be delayed until the age of three to minimize the chance of reoperation.

358 Quantifying Cognitive and Affective Dysfunction in Chiari Malformation Type I Patients With the Cerebellar Cognitive Affective Syndrome Scale and the Cerebellar Neuropsychiatric Rating Scale

INTRODUCTION: Cognitive and affective dysfunction in Chiari Malformation Type I (CM1) patients are well-documented, but often rely on extensive neuropsychological tests. METHODS: Symptomatic CM1 patients considering surgical decompression were assessed by a board-certified neuropsychologist using the 105-point CNRS (N = 87) and the 10-task CCAS (N = 23), as well as a full neuropsychological battery (N = 24: Wechsler Full Scale IQ, Digit Span, Coding; WRAML Finger Windows; DKEFS Trail Making; verbal list learning; Grooved Pegboard). Descriptive and correlative measures were computed. RESULTS: The study included 66 adults and 21 children. A neuropsychological battery revealed impairment in 29% of patients on 1 test, 12.8% on 2 tests, and 20.8% on 3 or more tests. The CCAS indicated 26% with "possible CCAS" (1 task), 27% with "probable CCAS" (2 tasks), and 18% with "definite CCAS" (3 or more tasks). The median CNRS composite score was 31.0 (IQR: 18.5, 49.0), with positive and negative symptom sub-scores of 18.0 (10.0, 26.5) and 14.0 (7.5, 23.5), respectively. Moderate correlation with the neuropsychological battery was found between impairment detected by CCAS (Spearman rank coefficient ρ = 0.67, p = 0.002) and CNRS (ρ = 0.56, p = 0.003). CONCLUSIONS: A high prevalence of cognitive and affective impairments in pre-operative CM1 patients was observed by all three testing batteries. In this first report of CNRS and CCAS usage in CM1 patients, increased CNRS and CCAS impairment correlated with impairment on standard neuropsychological testing. These tools show promise for future studies CM1, providing a more concise alternative to traditional testing for cognitive and affective dysfunction.

A CASE REPORT OF MYELOMENIGOCELE WITH ARNOLD CHIARI MALFORMATIN II-HIGHLIGHTING THE IMPORTANCE OF ANEMIA MUKTH BHARATH PROGRAMME

Introduction:Neural tube defects are common complex congenital malformations resulting from failure of neural tube closure during embryogenesis, which usually completes by 28 days post conception. Myelomeningocele is almost always associated with Arnold Chiari Malformation II (CM II)and vice versa. Aims &amp;Objectives : As most of the neural tube defects can be prevented by pre-conceptional folate supplements, we would like to high light the importance of proper implementation and utilization of Anemia Mukhth Bharath Programme. Background: Neural tube defects (NTDs) are caused by a combination of multiple genes and multiple environmental factors like folic acid deficiency, insulin dependent diabetes, obesity and certain medications which interfere with folate metabolism.One of the beneficiaries under the Anemia Mukth Bharath programme are women in the reproductive age group in the pre-conception period and up to the first trimester of the pregnancy. They are advised to have400 mcg of Folic Acid tablets, daily, to reduce the incidence of neural tube defects in the fetus. Case report :We present a case of Chiari malformation associated with megaloblastic anemia with an attempt to highlight the importance of proper utilization of national program for prevention of iron and folate deficiency in women of reproductive age group. Clinical relevance : Neural tube defects are largely preventable anomalies by simple and effective management of folate deficiency preconceptionally. Conclusion :Proper utilization of the Anemia Mukth Bharath program which is available to the beneficiaries at their doorstep can not only prevent maternal anemia but also prevent fetal loss due to NTD.

Safe Delivery of Electroconvulsive Therapy in Postpartum Depression Patient With Type 1 Chiari Malformation: A Case Study.

Safe Delivery of Electroconvulsive Therapy in Postpartum Depression Patient With Type 1 Chiari Malformation: A Case Study.

MRI Insights in Chiari Malformation Type 1 and Variations With Hydrosyringomyelia.

Chiari malformation (CM) type 1 is a complex neurological disorder characterized by the displacement of the cerebellar tonsils into the upper spinal canal. Hydrosyringomyelia (HSM), which frequently coexists with this condition, presents diagnostic and treatment problems due to its broad spectrum of symptoms. There are various forms of CMs, with CM type 1 (CM1) being the most common type. Magnetic resonance imaging (MRI) is the best imaging technique to properly identifyand diagnose CM1 and HSM. Important imaging findings include downward displacement of the cerebellar tonsils across the foramen magnum, the appearance of the syrinx in the spinal cord, and the alteration of the flow dynamics of the cerebrospinal fluid. This study was conducted at Datta Meghe Medical College, Nagpur, and Government Medical College & Super Speciality Hospital, Nagpur, India. It focuses on the diagnostic use of MRI in CM1 and its variations associated with HSM. Individuals who are asymptomatic may not need any treatment; however, those who are symptomatic or have HSM may require surgical decompression and restoration of the flow. We discuss the findings of MRI of six cases of CM1 and its variants with HSMand search for possible underlying causes. We conclude that magnetic resonance imaging is an imaging modality for the identification and evaluation of CM1 in cases of HSM.

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.

Topographic Anatomy and Pneumatization of the Posterior Clinoid Process in Chiari Type I Malformation

ObjectiveThis study aimed to examine pneumatization and topographic location of the posterior clinoid process (PCP) in Chiari type I malformation (CIM) for skull base approaches. MethodsComputed tomography images of 52 (23 males / 29 females) CIM subjects aged 23.87±16.09 years, and 71 (26 males / 45 females) healthy subjects aged 42.48±21.48 years constituted the study universe. ResultsThe distances of PCP to the foramen magnum (p=0.037), superior orbital fissure (p<0.001), foramen rotundum (p<0.001), and foramen ovale (p<0.001) were smaller, but the distance of PCP to the crista galli (p=0.038) was greater in CIM patients, compared to normal subjects. In CIM, the fusion between PCP and the anterior clinoid process was observed in nine sides (8.70%), while in controls, in 12 sides (8.50%). PCP pneumatization was observed in 40 sides (38.50%) in CIM patients, while in 28 sides (19.70%) in normal subjects. This data displayed that PCP pneumatization was affected by CIM (p<0.001). ConclusionThe distances of PCP to the crista galli and foramen magnum indicate the anterior fossa length and the posterior fossa depth, respectively; thus, CIM patients have longer anterior fossa and shallow posterior fossa. In addition, the distances of PCP to superior orbital fissure, foramen rotundum and foramen ovale indicate the middle fossa width; thence, CIM patients have less middle fossa width than normal individuals. CIM patients have approximately 50% higher PCP pneumatization rate, and this may increase the risk of complications such as cerebrospinal fluid fistula during the application of posterior clinoidectomy.

Association between structural rib autograft and the rate of arthrodesis in children undergoing occiput-C2 instrumentation and fusion.

The purpose of this study was to identify factors associated with fusion success among pediatric patients undergoing occiput-C2 rigid instrumentation and fusion. The Pediatric Spine Study Group registry was queried to identify patients ≤ 21 years of age who underwent occiput-C2 posterior spinal rigid instrumentation and fusion and had a 2-year minimum clinical and radiographic (postoperative lateral cervical radiograph or CT scan) follow-up. Fusion failure was defined clinically if a patient underwent hardware revision surgery > 30 days after the index procedure or radiographically by the presence of hardware failure or screw haloing on the most recent follow-up imaging study. Univariate comparisons and multivariable logistic regression analyses were subsequently performed. Seventy-six patients met inclusion criteria. The median age at surgery was 9 years (range 1.5-17.2 years), and 51% of the cohort was male. Overall, 75% of patients had syndromic (n = 41) or congenital (n = 15) etiologies, with the most frequent diagnoses of Down syndrome (28%), Chiari malformation (13%), and Klippel-Feil syndrome (12%). Data were available to determine if there was a fusion failure in 97% (74/76) of patients. Overall, 38% (28/74) of patients had fusion failure (95% CI 27%-50%). Univariate analysis demonstrated that use of a rigid cervical collar postoperatively (p = 0.04) and structural rib autograft (p = 0.02) were associated with successful fusion. Multivariable logistic regression analysis determined that patients who had rib autograft used in surgery had a 73% decrease in the odds of fusion failure (OR 0.27, 95% CI 0.09-0.82; p = 0.02). Age, etiology including Down syndrome, instrumentation type, unilateral instrumentation, use of recombinant human bone morphogenetic protein, and other variables did not influence the risk for fusion failure. In this multicenter, multidisciplinary, international registry of children undergoing occiput-C2 instrumentation and fusion, fusion failure was seen in 38% of patients, a higher rate than previously reported in the literature. The authors' data suggest that postoperative immobilization in a rigid cervical collar may be beneficial, and the use of structural rib autograft should be considered, as rib autograft was associated with a 75% higher chance of successful fusion.

Chiari Type I Revision Decompressive Surgery Indications and Operative Technique: Experience in a Large Adult Cohort

IntroductionChiari Malformation is characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, frequently resulting in strain related headaches, and motor/sensory dysfunction. Chiari decompression technique varies significantly, possibly contributing to frequent revisions. We reviewed revision Chiari decompressions at our institution to determine the primary indications for revision and outcomes after revision. MethodWe retrospectively reviewed patients who underwent revision of Chiari decompression at our institution from 2005-2020. Demographics, indications for revision surgery, operative techniques, imaging findings, and pre-/post-operative symptoms were collected. Chi-squared test was performed to determine statistical significance using a p<0.05. Independent predictors of operative outcomes were identified. ResultA total of 46 patients (91% female, mean age 38.8 years) were included for analysis. The median time to revision surgery was 69.1 months (range 0-364 months) with headache (n=37, 80%) being the most commonly recurring symptom. Large craniectomy (n=28, 61%) was the most frequent indication for revision surgery. Thirty-two (70%) patients underwent cranioplasty, 20 (43%) required duraplasty, 15 (33%) required arachnoid dissection, and 15 (33%) required tonsillar reduction during revision surgery. Post-revision follow-up (at 8.9 ± 5.2 months average, range 1-18 months), revealed an average reduction in all Chiari-related symptoms relative to symptoms before the revision. ConclusionThe most common indication for revision Chiari decompression was a large craniectomy resulting in cerebellar ptosis. We found that tonsillar reduction paired with modest craniectomy achieved near-complete resolution of symptoms with minimal complications. For patients with recurrent or persistent sequelae of Chiari malformation after decompression, revision may reduce symptom severity.

A Single-center Study Comparing Volumetric and Morphometric Measurements of Chiari Malformation in the Turkish Population

A Single-center Study Comparing Volumetric and Morphometric Measurements of Chiari Malformation in the Turkish Population

Long-term outcome of operated Chiari I patients between 2005 and 2020 in Eastern Finland

PurposeThe purpose of our study was to examine the long-term outcomes of operated Chiari malformation type 1 (CM1) patients and evaluate whether different duraplasty techniques affected outcome after surgery in Kuopio University Hospital catchment area.MethodsIn this retrospective study, a total of 93 patients were diagnosed with CM1 and underwent posterior fossa decompression surgery with or without duraplasty between 2005 and 2020. All patients’ medical records were examined for baseline characteristics, surgical details, and long-term follow-up data after operation.ResultsThe mean age of CM1 patients was 25.9 years (SD 19.2 years), with female preponderance 69/93 (73.4%). The mean clinical follow-up time was 26.5 months (SD 33.5 months). The most common presenting symptoms were headache, symptoms of extremities, and paresthesia. Posterior fossa decompression with duraplasty was performed in 87 (93.5%) patients and bony decompression in 6 (6.5%) patients. After surgery, preoperative symptoms alleviated in 84.9% (79/93) and the postoperative syringomyelia regression rate was 89.2% (33/37) of all patients. The postoperative complication rate was 34.4% (32/93), with aseptic meningitis being the most common, 25.8% (24/93). Revision surgery was required in 14% (13/93) of patients. No significant correlation between postoperative outcome and extent of dural decompression, or type of duraplasty performed was found.ConclusionThis is the largest reported series of surgically treated CM1 patients in Finland. Posterior fossa decompression is an effective procedure for CM1 symptomology. Duraplasty technique had no significant difference in complication rate or long-term outcomes.

Arnold Chiari Malformation

Chiari malformations (CM) refer to a series of anomalies characterized by the descent of cerebellar tonsils into the cervical spinal canal. These malformations can be associated with abnormalities such as syringomyelia, hydrocephalus, spina bifida, and scoliosis. Additionally, craniocervical junction anomalies, endocrinopathies, craniosynostosis, and syndromic disorders are also linked to CM. The treatment of CM is surgical, and there is no known medical therapy. Patients diagnosed with CM are typically advised to undergo surgical treatment or follow-up. Although surgical intervention is supported in the literature, debates exist regarding which procedure is most suitable and when surgery should be performed. In this article, we will examine the historical background of CM, its anatomical forms, pathophysiology, clinical presentation, relationship with other diseases, and diagnostic procedures in the light of the literature.

Volumetric assessment of Posterior cranial fossa in a West African population

Background and Objectives: Posterior fossa pathologies can have potentially devastating outcomes. The volumetric capacity of this fossa, known to have ethnoregional variations, can thus be critical in determining outcomes and intervention measures and approaches to pathologies involving this region. This study aimed to evaluate the normal posterior fossa volumes within the West-African subpopulation. Methods: This was a descriptive study of all patients presenting for a cranial imaging study at the study location within a two-year period using a 1.5T MRI of this cranial region. Obtained data included the transverse and anteroposterior diameters, and the height of this fossa and the obtained data was analyzed. P values < 0.05 was statistically significant. Results: A total of 315 patients were recruited (165 males and 150 females). The average posterior fossa transverse diameter, anteroposterior diameter and height were 108.19 mm, 71.58 mm and 35.53 mm respectively for males, and 105.7 mm, 66.48 mm, and 34.24 mm for females respectively. The average posterior fossa volume for males (292.36 cm3) was significantly higher than for females (252.90 cm3) (p= 0.0038). The highest average posterior fossa volume was between 16-30 years for males and above 75 years for females. Conclusion: Posterior cranial fossa volumes for the West African population is significantly higher than those obtained for other regions. In addition to being beneficial in some posterior fossa space occupying lesions, this larger volume can explain the relative rarity and sexual preferences of some posterior fossa congenital abnormalities like Chiari-1 malformation amongst the West African population

Multicenter comparison of Chiari malformation type I presentation in children versus adults.

Treatment for Chiari malformation type I (CM-I) often includes surgical intervention in both pediatric and adult patients. The authors sought to investigate fundamental differences between these populations by analyzing data from pediatric and adult patients who required CM-I decompression. To better understand the presentation and surgical outcomes of both groups of patients, retrospective data from 170 adults and 153 pediatric patients (2000-2019) at six institutions were analyzed. The adult CM-I patient population requiring surgical intervention had a greater proportion of female patients than the pediatric population (p < 0.0001). Radiographic findings at initial clinical presentation showed a significantly greater incidence of syringomyelia (p < 0.0001) and scoliosis (p < 0.0001) in pediatric patients compared with adult patients with CM-I. However, presenting signs and symptoms such as headaches (p < 0.0001), ocular findings (p = 0.0147), and bulbar symptoms (p = 0.0057) were more common in the adult group. After suboccipital decompression procedures, 94.4% of pediatric patients reported symptomatic relief compared with 75% of adults with CM-I (p < 0.0001). Here, the authors present the first retrospective evaluation comparing adult and pediatric patients who underwent CM-I decompression. Their analysis reveals that pediatric and adult patients significantly differ in terms of demographics, radiographic findings, presentation of symptoms, surgical indications, and outcomes. These findings may indicate different clinical conditions or a distinct progression of the natural history of this complex disease process within each population, which will require prospective studies to better elucidate.