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Psychiatria Danubina 2022
Topics: Adolescent; Arnold-Chiari Malformation; Conversion Disorder; Dissociative Disorders; Humans; Magnetic Resonance Imaging
PubMed: 35772149
DOI: 10.24869/psyd.2022.318 -
Journal of Clinical Sleep Medicine :... Sep 2022The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to... (Review)
Review
STUDY OBJECTIVES
The main aim was to evaluate the prevalence of sleep-disordered breathing (SDB) in patients with Chiari II malformation (CM-II). The secondary objectives were to evaluate the association between SDB, morphological abnormalities, and neurological symptoms and to review the literature on patients with SDB and CM-II.
METHODS
The study has a cross-sectional, case-control design. Patients with CM-II (patients) were compared to control patients referred for clinical polysomnography in the Sleep Medicine Unit, matched for age and sex. All patients underwent brain and spinal cord magnetic resonance imaging, and polysomnography was conducted for all participants. A review of the literature about SDB in patients with CM-II was performed.
RESULTS
Forty patients were included (20 patients vs 20 control patients). SDB was identified in 45% of patients, a significantly higher prevalence compared to control patients. Three patients presented with purely obstructive SDB, 3 patients with purely central SDB, and 3 patients with both obstructive and central SDB. Compared with control patients, patients with CM-II showed a higher oxygen desaturation index (median: CM-II, 3.7; interquartile range, 1.6-19.5; control patients: 1.1; interquartile range, 0.3-3.2) and obstructive apnea-hypopnea index (median: CM-II, 1.5; interquartile range, 0.5-5.1; control patients, 0.1; interquartile range, 0.0-0.7). A logistic regression showed that the risk of developing SDB in patients affected by CM-II was 14.7 times higher than in the control population.
CONCLUSIONS
Our study and literature review showed a high prevalence of SDB in patients with CM-II. These patients are often asymptomatic at diagnosis, suggesting that PSG should be routinely provided in this population.
CITATION
Lazzareschi I, Curatola A, Massimi L, et al. Sleep-disordered breathing in patients with Chiari malformation type II: a case-control study and review of the literature. 2022;18(9):2143-2154.
Topics: Arnold-Chiari Malformation; Case-Control Studies; Cross-Sectional Studies; Humans; Polysomnography; Sleep Apnea Syndromes
PubMed: 35645039
DOI: 10.5664/jcsm.10062 -
Paediatric Anaesthesia Aug 2022Posterior fossa decompression for Chiari I Malformation is a common pediatric neurosurgical procedure. We sought to identify the impact of anesthesia-related...
Patient and operative factors associated with unanticipated intensive care admission and outcomes following posterior fossa decompressions in children: A retrospective study.
INTRODUCTION
Posterior fossa decompression for Chiari I Malformation is a common pediatric neurosurgical procedure. We sought to identify the impact of anesthesia-related intraoperative complications on unanticipated admission to the intensive care unit and outcomes following posterior fossa decompression.
METHODS
Medical records of all patients <18 years who underwent surgery for Chiari I malformation between 1/1/09 and 1/31/21 at the Ann & Robert H. Lurie Children's Hospital of Chicago were included. Records were reviewed for patient characteristics, anesthesia-related intraoperative complications, postoperative complications, and surgical outcomes. The primary outcome was the incidence of unanticipated admission to the intensive care unit, and the primary variable of interest was an anesthesia-related intraoperative complication. Patient, surgical characteristics, and year of surgery were also compared between patients with and without an unanticipated admission to the intensive care unit, and a multi-variable adjusted estimate of odds of unanticipated admission to the intensive care unit admission following an anesthesia-related intraoperative complication was performed. Secondary outcomes included anesthesia factors associated with an anesthesia-related intraoperative event, and postoperative complications and surgical outcomes between patients admitted to the intensive care unit and those who were not.
RESULTS
Two hundred ninety-six patients with Chiari I Malformation were identified. Clinical characteristics associated with an unanticipated admission to the intensive care unit were younger age, American Society of Anesthesiologist (ASA) physical status >2 and an anesthesia-related intraoperative complication. 29 anesthesia-related intraoperative complications were observed in 25 patients (8.4%). Two of 25 patients (8%) with an anesthesia-related intraoperative complication compared with 3 of 271 (1%) patients without anesthesia-related intraoperative complication had an unanticipated admission to the intensive care unit, odds ratio 7.8 (95% CI 1.2-48.8, p = .010). When adjusted for age, sex, ASA physical status, presenting symptoms, concomitant syringomyelia, previous decompression surgery and year of surgery, the odds ratio for an unanticipated admission to the intensive care unit following an anesthesia-related intraoperative complication was 5.9 (95% CI 0.51-59.6, p = .149). There were no differences in surgical outcomes between patients with or without an unanticipated admission to the intensive care unit.
CONCLUSION
Our study demonstrates that although anesthesia-related intraoperative complications during posterior fossa decompression are infrequent, they are associated with an increased risk of an unanticipated admission to the intensive care unit.
Topics: Arnold-Chiari Malformation; Child; Critical Care; Decompression; Humans; Intraoperative Complications; Postoperative Complications; Retrospective Studies; Treatment Outcome
PubMed: 35604044
DOI: 10.1111/pan.14496 -
BMJ Open May 2022Chiari I malformation (CM1) is an anatomical abnormality characterised by the cerebellar tonsils descending at least 5 mm below the foramen magnum. CM1 causes...
INTRODUCTION
Chiari I malformation (CM1) is an anatomical abnormality characterised by the cerebellar tonsils descending at least 5 mm below the foramen magnum. CM1 causes obstruction of cerebrospinal fluid (CSF) circulation as well as direct compression on the brainstem, thus causing typical consequences (syringomyelia), and typical clinical features (characteristic headaches and neurological impairment). Surgery is the only available treatment, indicated when symptomatology is present. However, sometimes patients have atypical complaints, which are often suggestive of otolaryngological (ears, nose and throat, ENT) involvement. This may be difficult for a neurosurgeon to explain. Our study aims to investigate the relationship between one of these atypical symptoms, for example, postural instability, in a paediatric population using a Computerised Dynamic Posturography (Equitest, NeuroCom, Clackamas, OR). To our knowledge, there are no previously published studies carried out on children with CM1, using dynamic posturography.
METHODS AND ANALYSIS
Forty-five children aged 6-18 years old presenting with radiologically confirmed CM1 and presenting ENT clinical complaints will be included in the study for a duration of 3 years. As primary endpoint, posturographic results will be described in the population study. Second, posturographic results will be compared between patients with and without indication for surgery. Finally, preoperative and postoperative posturographic results, as well as CSF circulation quality at foramen magnum level, syringomyelia, sleep apnoea syndrome, scoliosis and behaviour will be compared in the operated patient group.
ETHICS AND DISSEMINATION
This protocol received ethical approval from the Clinical Research Delegation of Nancy University Hospital, in accordance with the National Commission on Informatics and Liberties (Commission Nationale de l'Informatique et des Libertés) (protocol number 2019PI256-107). Our data treatment was in accordance with the Methodology of reference Methodology Reference-004 specification for data policy. The study findings will be disseminated via peer-reviewed publications and conference presentations, especially to the Neurosphynx's rare disease healthcare network.
TRIAL REGISTRATION NUMBER
NCT04679792; Pre-results.
Topics: Adolescent; Arnold-Chiari Malformation; Child; Headache; Humans; Magnetic Resonance Imaging; Observational Studies as Topic; Postural Balance; Prospective Studies; Syringomyelia
PubMed: 35551083
DOI: 10.1136/bmjopen-2021-056647 -
Neurologic Clinics May 2022Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is... (Review)
Review
Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is frequently associated with syringomyelia. Herein, we discuss the history of CMI and syringomyelia, including early pathological and surgical studies. We also describe recent investigations into the pathogenesis and pathophysiology of CMI and their practical implications on management and surgical intervention. We also highlight the recent development of the Common Data Elements for CMI, providing a framework for ongoing investigations. Finally, we discuss current controversies of surgical management in CMI.
Topics: Arnold-Chiari Malformation; Foramen Magnum; Humans; Magnetic Resonance Imaging; Syringomyelia
PubMed: 35465876
DOI: 10.1016/j.ncl.2021.11.007 -
Folia Morphologica 2023Typically, patients with Chiari I malformations (CM I) do not have other intracranial anatomical variations, especially vascular derailments. Here, we report the...
Typically, patients with Chiari I malformations (CM I) do not have other intracranial anatomical variations, especially vascular derailments. Here, we report the findings of a cadaveric specimen found to have CM I and cerebellar tonsils supplied by a single posterior inferior cerebellar artery (PICA) i.e., a bihemispheric PICA. An adult male cadaver was found to have CM I. It was also noted that the left PICA descended inferiorly to the level of C1 and that there was absence of the right PICA. The territory of the right PICA was supplied by the left PICA. The tonsillar component of the left PICA gave rise to a branch that crossed to the right inferior cerebellum and herniated cerebellar tonsil. A bihemispheric PICA is very rare. To our knowledge, this is the first report of this vascular variation in combination with CM I. Such a variation should be kept in mind, especially during posterior fossa decompression for symptomatic CM I as unilateral PICA injury could have catastrophic results.
Topics: Adult; Humans; Male; Arnold-Chiari Malformation; Cerebellum; Vertebral Artery; Cadaver; Head
PubMed: 35411545
DOI: 10.5603/FM.a2022.0038 -
Journal of Integrative Neuroscience Mar 2022The frequent association of basilar invagination (BI) makes the understanding of the pathogenesis of Chiari malformation type I (CMI) difficult. The influence of group B...
BACKGROUND
The frequent association of basilar invagination (BI) makes the understanding of the pathogenesis of Chiari malformation type I (CMI) difficult. The influence of group B type of BI (the BI without obvious atlantoaxial instability) on the skeletal morphology has not been thoroughly studied. The objective of this study is to evaluate the skeletal alterations in the posterior cranial fossa (PCF) of adult CMI cases with and without group B BI.
METHODS
Fifty-four adult CMI without BI cases (CMI-only group) and 30 adult CMI with group B BI cases (CMI-BI group) were retrospectively studied. Fifty-six adult patients with unruptured intracranial aneurysms were included as the controls. Several linear and angular variables, and the bony volume of the PCF were analyzed based on thin-slice computed tomography data.
RESULTS
Morphological analysis revealed a significant difference in several variables from controls compared to CMI-only, and CMI-BI patients. The clivus and occipital bone, shortened and elevated in CMI-only patients, were further flattened in BI-associated CMI patients. Furthermore, although out of the scope for the diagnostic threshold of BI, the CMI-only cases also had a tendency to form BI. The association of BI modified several variables, without further reducing the bony PCF volume.
CONCLUSIONS
These findings indicate that the variables associated with group B BI tend to be a continuum of the same pathological abnormalities that originate from the same pathological alterations in CMI patients.
Topics: Adult; Arnold-Chiari Malformation; Cranial Fossa, Posterior; Humans; Magnetic Resonance Imaging; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 35364658
DOI: 10.31083/j.jin2102070 -
Annals of Neurosciences Jul 2021Syringomyelia (SM) with Chiari malformation is a rare disease with an unpredictable course. Surgery and other interventions help reduce the severity of symptoms, but...
Syringomyelia (SM) with Chiari malformation is a rare disease with an unpredictable course. Surgery and other interventions help reduce the severity of symptoms, but over 50% patients require re-operation. Auto-resolution is rare in this type of SM, and most cases progress to complications, which may amount to a great burden. The patient of SM with Arnold-Chiari malformation type 1 in a 54-year-old Russian woman who was treated with individualized classical homeopathy for over eight years with remarkable improvement in the clinical signs and symptoms of the condition and comorbidities. On MRI, the syrinx completely resolved, which further confirmed the benefit of this therapy. This case of SM with Arnold-Chiari malformation type 1 seemed to benefit from individualized classical homeopathy. Scientific investigation into an individualized classical homeopathic approach towards SM is necessary to establish its relevance in this condition.
PubMed: 35341222
DOI: 10.1177/09727531211046370 -
Revue Medicale de Liege Mar 2022Medulloblastoma is a cerebellar grade IV tumour according to the WHO classification, mainly seen in children under the age of 15. This cancer can nevertheless occur in...
Medulloblastoma is a cerebellar grade IV tumour according to the WHO classification, mainly seen in children under the age of 15. This cancer can nevertheless occur in adults. We report the case of a 22-year-old patient with a medulloblastoma disseminated in the spine. The patient had a type 1 Arnold-Chiari malformation causing hydrocephalus treated by ventriculoperitoneal shunt. The current condition began with perineal and lower limb hypoesthesia, ataxic gait, erectile dysfunction and urinary incontinence. Subsequently, a predominant paraparesis of the right lower limb appeared. The patient was treated according to the PNET HR+5 protocol combining two courses of conventional chemotherapy followed by two courses of high-dose chemotherapy with autograft recovery. Given the excellent response, a proton therapy was then delivered to the whole cerebrospinal axis with boosts to the primary tumour sites. The case of this young adult patient shows on the one hand an atypical presentation, and on the other hand underlines, in the absence of a specific therapeutic strategy established for adults, the importance of collaboration between the adult and pediatric oncology departments, with management integrating innovations such as proton therapy and molecular typing.
Topics: Adult; Cerebellar Neoplasms; Child; Humans; Male; Medulloblastoma; Young Adult
PubMed: 35258863
DOI: No ID Found -
Cephalalgia : An International Journal... Aug 2022Chiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on...
BACKGROUND
Chiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on Chiari I malformation-associated headache semiologies and the effect of foramen magnum decompression on different headache types.
METHODS
A retrospective analysis complemented by structured phone interviews was performed on 65 patients with Chiari I malformation, treated at our hospital between 2010 and 2021. Headache semiology (according to ICHD-3), frequency, intensity, and radiological characteristics were evaluated pre- and postoperatively.
RESULTS
We included 65 patients. 38 patients were female and 27 male. Mean age was 43.9 ± 15.7 years. Headache was predominant in 41 patients (63.0%). Twenty-one patients had cough headache and 20 had atypical headache (12 migrainous, eight tension-type headache-like). Thirty-five patients with headache underwent surgery. Frequency, intensity, and analgesic use was significantly reduced in cough headache ( < 0.001). Atypical headaches improved less ( = 0.004 to 0.176). Exploratory analysis suggested that larger preoperative tonsillar descent correlated with larger postoperative headache intensity relief ( = 0.025).
CONCLUSION
Decompression was effective in Chiari I malformation-related cough headache. Atypical headache responded less well, and the causal relation with Chiari I malformation remains uncertain. For atypical headache, decompression should only be considered after failed appropriate preventive therapy and within an interdisciplinary approach involving a neurologist.
Topics: Adult; Arnold-Chiari Malformation; Decompression, Surgical; Female; Headache; Headache Disorders, Primary; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Migraine Disorders; Retrospective Studies
PubMed: 35236163
DOI: 10.1177/03331024221079296