2,495 results match your criteria Imaging in Chiari I Malformation

MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation.

Neuroradiology 2022 Jun 14. Epub 2022 Jun 14.

Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, WCB90, 330 Brookline Ave, Boston, MB, 02215, USA.

Purpose: Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions.

Methods: Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed. Read More

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Case Report: Downbeat Nystagmus Due to Epidural Puncture during Labor with Undiagnosed Arnold Chiari Malformation.

Optom Vis Sci 2022 Jun 9. Epub 2022 Jun 9.

Illinois College of Optometry, Chicago, Illinois.

Significance: Epidural anesthesia is a safe procedure used in pregnant patients during labor. However, caution should be exercised in those patients that have concurrent Arnold Chiari malformation.

Purpose: To report a rare and atypical presentation of downbeat nystagmus, in a pregnant patient with Arnold Chiari malformation type 1 (ACM1), secondary to accidental dural puncture. Read More

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Low occurrence of long-term subsequent fusion in pediatric patients following decompressive surgery for Chiari malformation: an institutional review.

Childs Nerv Syst 2022 May 19. Epub 2022 May 19.

Department of Neurologic Surgery, Mayo Clinic, Rochester, MN, USA.

Objective: Chiari I malformation is treated with suboccipital craniectomy with cervical laminectomy, a procedure which has been associated with the possibility of pre-existing or iatrogenic occipitocervical instability. The long-term risk of subsequent spinal deformity and need for occipito cervical fusion after standard Chiari decompression in pediatric patients has not yet been characterized.

Methods: We queried our institutional electronic database for patients aged 18 and under, with at least 5 years of follow-up, that underwent surgical decompression for Chiari I malformation. Read More

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Postural control in Chiari I malformation: protocol for a paediatric prospective, observational cohort - potential role of posturography for surgical indication.

BMJ Open 2022 05 12;12(5):e056647. Epub 2022 May 12.

EA 3450 DevAH - Development, Adaptation and Handicap, Faculty of Medicine, University of Lorraine, Nancy, France.

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. Read More

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Evaluation of cervical sagittal parameters on supine magnetic resonance imaging in patients with Chiari I malformation without syringomyelia.

Neurochirurgie 2022 May 5. Epub 2022 May 5.

Department of Neurosurgery, BHT Clinic Istanbul TEMA Hospital, İstanbul, Turkey.

Objective: The current study aimed to assess the effects of tonsillar herniation on cervical alignment in Chiari I patients without syringomyelia using new cervical sagittal alignment parameters, such as C0-2 Cobb angle, C2-7 cobb angle, T1 slope, and C2-7 sagittal vertical axis (SVA).

Methods: Two spinal surgeons independently evaluated midline T2-weighted sagittal magnetic resonance imaging findings of 28 Chiari I patients without syringomyelia and 40 patients without tonsillar herniation but with similar complaints. Thereafter, the measured C0-2 Cobb angle, C2-7 Cobb angle T1 slope, and C2-7 SVA were compared using the t-test. Read More

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Hemifacial spasm secondary to Chiari malformation type I: Systematic review with case illustration.

J Clin Neurosci 2022 Jul 2;101:21-25. Epub 2022 May 2.

Department of Neurosurgery, Clinical Neuroscience Center, University of Utah, 175 N. Medical Drive East, Salt Lake City, UT 84132, USA; Department of Biomedical Engineering, University of Utah, 36 S. Wasatch Drive, Salt Lake City, UT 84112, USA. Electronic address:

Hemifacial spasm (HFS) can be associated with Chiari malformation type I (CM1), but the treatment paradigm for these concurrent conditions has not been well-defined. We sought demographical differences between patients with HFS with and without CM1 and explored optimal surgical treatments for these patients. A systematic review of peer-reviewed literature identified 8 studies with 51 patients with CM1 and HFS. Read More

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[Dislocations of the cerebellar tonsils in the large occipital foramen and the spectrum of Chiari malformations Type 1].

E I Bogdanov

Zh Nevrol Psikhiatr Im S S Korsakova 2022 ;122(4):7-15

Kazan State Medical University of the Ministry of Health of Russia, Kazan, Russia.

A review on the pathogenesis, clinical picture and diagnosis of dislocations of the cerebellar tonsils in the large occipital foramen and the spectrum of Chiari malformations Type 1 is provided. The issues of surgical treatment of such patients are discussed. Read More

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Chiari Malformation (Update on Diagnosis and Treatment).

Neurol Clin 2022 05 31;40(2):297-307. Epub 2022 Mar 31.

National Institutes of Health, National Institute of Neurological Disorders and Stroke, Surgical Neurology Branch, 10 Center Drive, Room 3D20, Bethesda, MD, 20892 USA. Electronic address:

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. Read More

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Statistical shape models of the posterior cranial fossa and hindbrain volumes may provide a more robust clinical metric for Chiari malformation.

J Biomech 2022 May 12;137:111093. Epub 2022 Apr 12.

Neuroscience Research Australia and Prince of Wales Clinical School, University of New South Wales, Australia. Electronic address:

Chiari malformation is characterised by the herniation of the cerebellar tonsils through the foramen magnum. However, tonsillar herniation and other 2D morphometric measurements of the posterior cranial fossa (PCF) have a weak association with patients' symptoms and clinical outcomes. This study aimed to contrast current 2D metrics with a novel 3D shape analysis of the PCF and the hindbrain, to determine if 3D measurements provides further insight into the pathophysiology of Chiari. Read More

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Diagnostic performance evaluation of adult Chiari malformation type I based on convolutional neural networks.

Eur J Radiol 2022 Jun 2;151:110287. Epub 2022 Apr 2.

Department of Neurosurgery, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China. Electronic address:

Purpose: This study aimed to evaluate the diagnostic performance of convolutional neural network (CNN) models in Chiari malformation type I (CMI) and to verify whether CNNs can identify the morphological features of the craniocervical junction region between patients with CMI and healthy controls (HCs). To date, numerous indicators based on manual measurements are used for the diagnosis of CMI. However, the corresponding postoperative efficacy and prognostic evaluations have remained inconsistent. Read More

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Resolution of Primary or Recalcitrant Chiari-Associated Syringomyelia Requires Adequate Cerebrospinal Fluid Egress from the Fourth Ventricle.

World Neurosurg 2022 Jul 6;163:24. Epub 2022 Apr 6.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston. Electronic address:

Syringomyelia is often resistant to various treatment modalities. Chiari I malformations are associated with syringomyelia in approximately 69% of operative cases. Failure to resolve syringomyelia after Chiari decompression is common. Read More

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Conservative Treatment of Chiari Malformation Type I Based on the Phase-Contrast Magnetic Resonance Imaging: A Retrospective Study.

World Neurosurg 2022 Jul 1;163:e323-e334. Epub 2022 Apr 1.

Department of Neurosurgery, Faculty of Medicine, Afyonkarahisar Health Sciences University, Afyonkarahisar-Afyon, Turkey.

Background: The use of phase-contrast magnetic resonance imaging is interestingly increased in the diagnosis and follow up of patients with Chiari type I malformation (CM1). The current study aimed to elaborate the benefits of conservative treatment by evaluating consecutively treated adult patients with CM1 who were selected on basis of phase-contrast magnetic resonance imaging.

Methods: Medical records of patients diagnosed with CM1 were retrospectively reviewed at 2 neurosurgical centers spanning 8 years (2010-2017). Read More

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Morphometric and volumetric analysis of the posterior cranial fossa in adult Chiari malformation type I with and without group B basilar invagination.

J Integr Neurosci 2022 Mar;21(2):70

Department of Neurosurgery, The First Affiliated Hospital of Chongqing Medical University, 400016 Yuzhong District, Chongqing, China.

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. Read More

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Headache and Other Symptoms in Chiari Malformation Type I Are Associated with Cerebrospinal Fluid Flow Improvement After Decompression: A Two-Institutional Study.

World Neurosurg 2022 Jul 30;163:e253-e262. Epub 2022 Mar 30.

Department of Neurosurgery, The Ohio State University Medical Center, Columbus, Ohio, USA. Electronic address:

Objective: Chiari malformation type I (CMI) is a common neurologic condition and surgery is the standard treatment. We aim to establish the cerebrospinal fluid (CSF) flow change as a potential objective indicator of surgical efficacy to improve symptoms.

Methods: We performed a retrospective 2-center analysis of surgically treated patients with CMI. Read More

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Relevance of C1/2 Facet Configurations and Clivus-Canal-Angles for Adult Patients with Chiari I Malformation with and without Basilar Invagination.

Jörg Klekamp

World Neurosurg 2022 Jun 2;162:e156-e167. Epub 2022 Mar 2.

Department of Neurosurgery, Quakenbrück Christian Hospital, Quakenbrück, Germany. Electronic address:

Objective: C1/2 facet configurations and clivus-canal-angles (CXAs) have been proposed as criteria for posterior fusion in Chiari I malformation (CMI).

Methods: Three-hundred and forty adults with CMI without basilar invagination (BI), 111 with CMI with BI, and 100 age- and sex-matched controls were studied using sagittal T2-weighted magnetic resonance imaging scans analyzing preoperative and postoperative values with their impact on progression-free survival rates.

Results: For CMI without BI, C1/2 facet configurations and CXA were similar to controls (142 ± 11 degrees and 144 ± 10 degrees, respectively) with low rates for posterior C1 displacements (7. Read More

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Magnetic Resonance-Guided Diagnosis of Spontaneous Intracranial Hypotension in a Middle-Aged Woman.

Case Rep Neurol Med 2022 21;2022:4438923. Epub 2022 Feb 21.

Burrell College of Osteopathic Medicine, Las Cruces, NM, USA.

Spontaneous intracranial hypotension (SIH) is a rare condition caused by a fluid (CSF) leak. It is diagnosed by clinical features that include an orthostatic headache combined with imaging findings demonstrating intracranial hypotension and a CSF leak. We present the case of a 45-year-old woman with an orthostatic headache who was found to have a sagging brain with a downward-displaced cerebellum and pachymeningeal enhancement with gadolinium contrast. Read More

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February 2022

Factors Associated With Patient-Reported Postsurgical Symptom Improvement in Adult Females with Chiari Malformation Type I: A Report from the Chiari1000 Dataset.

World Neurosurg 2022 May 25;161:e682-e687. Epub 2022 Feb 25.

Department of Mechanical & Industrial Engineering, Department of Bioengineering, Northeastern University, Boston, Massachusetts, USA.

Objective: The goal of this study was to assess if a broad array of factors is predictive of patient-reported surgical impact on symptoms.

Methods: Sixty-five presurgical factors were selected from the self-report history questionnaires (12 variables, N = 653), standardized scales (14 variables, N = 494-581), and morphometric measurements from magnetic resonance imaging (39 variables, N = 137) data of adult women in the Chiari1000 dataset. The factors were tested independently to identify those that were either significantly different in terms of, or significantly related to, patient-reported surgical impact on symptoms (P < 0. Read More

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Use of deep learning in the MRI diagnosis of Chiari malformation type I.

Neuroradiology 2022 Feb 24. Epub 2022 Feb 24.

Macquarie Medical School, Macquarie University, NSW, 2109, Sydney, Australia.

Purpose: To train deep learning convolutional neural network (CNN) models for classification of clinically significant Chiari malformation type I (CM1) on MRI to assist clinicians in diagnosis and decision making.

Methods: A retrospective MRI dataset of patients diagnosed with CM1 and healthy individuals with normal brain MRIs from the period January 2010 to May 2020 was used to train ResNet50 and VGG19 CNN models to automatically classify images as CM1 or normal. A total of 101 patients diagnosed with CM1 requiring surgery and 111 patients with normal brain MRIs were included (median age 30 with an interquartile range of 23-43; 81 women with CM1). Read More

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February 2022

Craniocervical Stabilization After Failed Chiari Decompression: A Case Series of a Population with High Prevalence of Ehlers-Danlos Syndrome.

World Neurosurg 2022 May 19;161:e546-e552. Epub 2022 Feb 19.

Department of Neurosurgery, MedStar Georgetown University Hospital, Washington, DC, USA.

Background: In the treatment of Chiari malformation type I (CM-I), posterior fossa decompression is achieved via suboccipital craniectomy (SOC); however, some patients continue to experience symptoms after treatment, which may be due to craniocervical instability (CCI). The purposes of this study were to analyze data from patients who required an occipitocervical fusion (OCF) for the management of CCI after having previously undergone SOC for CM-I to determine if OCF is a safe and effective option and to determine any identifiable risk factors for CCI in these patients.

Methods: A retrospective review was done on all patients who underwent an OCF performed by the senior author between November 2013 and June 2020 after having previously undergone SOC for CM-I. Read More

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Superficial abdominal reflex in syringomyelia: Associations with Chiari I malformation.

J Clin Neurosci 2022 Apr 31;98:1-5. Epub 2022 Jan 31.

Department of Neurosurgery, Washington University, Saint Louis, MO, USA. Electronic address:

An abnormal or absent superficial abdominal reflex (SAR) may be associated with an underlying spinal cord syrinx. The sensitivity of an abnormal or absent SAR and the relationship to Chiari malformation type I (CM-I) or syrinx morphology has not been studied. We aimed to describe the relationship between SAR abnormalities and syrinx size, location, and etiology. Read More

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The factors affecting the outcomes of conservative and surgical treatment of chiari i adult patients: a comparative retrospective study.

Neurol Res 2022 Feb 2;44(2):165-176. Epub 2022 Feb 2.

Department Of Neurosurgery, University Of Health Sciences, Bakırköy Research And Training Hospital For Neurology Neurosurgery, And Psychiatry, Istanbul, Bakırköy - Istanbul; Turkey.

Background: The prognosis of the Chiari malformation type 1 (CM1) demonstrates a variant spectrum that varies from full recovery to complicated worse neurological disability.

Objective: To investigate the factors affecting the outcomes of conservative and surgical treatment for CM1 by evaluating adult patients consecutively managed at our institutions.

Materials And Methods: We retrospectively reviewed the medical records of patients diagnosed with CM1 at two reference neurosurgical centers for eight years (2010-2017). Read More

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February 2022

Hydrocephalus in Spina Bifida.

Neurol India 2021 Nov-Dec;69(Supplement):S367-S371

Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, Al. USA 35233, USA.

Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have required treatment with a shunt but recent advances including intra-uterine myelomeningocele closure and ETV-CPC are reducing this burden. The expression of hydrocephalus differs between patients and across the life span. Read More

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February 2022

A new hypothesis for the pathophysiology of symptomatic adult Chiari malformation Type I.

Med Hypotheses 2022 Jan 14;158. Epub 2021 Dec 14.

Department of Mechanical & Industrial Engineering, Department of Bioengineering, Northeastern University, 334 Snell Engineering, 360 Huntington Ave, Boston, MA 02115, United States.

Chiari malformation Type I (CMI) is characterized by herniation of the cerebellar tonsils through the foramen magnum. The pathophysiology of CMI is not well elucidated; however, the prevailing theory focuses on the underdevelopment of the posterior cranial fossa which results in tonsillar herniation. Symptoms are believed to be due to the herniation causing resistance to the natural flow of cerebrospinal fluid (CSF) and exerting a mass effect on nearby neural tissue. Read More

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January 2022

Occipital cranioplasty using customized titanium prosthesis yields successful outcome in association with foramen magnum decompression in dogs suffering by Chiari-like malformation.

Am J Vet Res 2021 Dec 27;83(3):275-282. Epub 2021 Dec 27.

Objective: To describe the use of a customized 3-D-printed titanium prosthesis as adjunctive treatment for foramen magnum decompression (FMD) in dogs with Chiari-like malformation (CM) and syringomyelia (SM).

Animals: 8 dogs with clinical signs and MRI findings of CM-SM.

Procedures: 3-D reconstruction of CT images of the head was used to simulate an occipital craniectomy and design the prosthesis. Read More

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December 2021

Syrinx reduction due to spontaneous spinal cord tear: demonstration on 3 T MRI and review of the literature.

BMJ Case Rep 2021 Dec 21;14(12). Epub 2021 Dec 21.

Neuroradiology, Ente Ospedaliero Cantonale, Ospedale Regionale Lugano, Neurocentre of Southern Switzerland, Lugano, Switzerland

A holocord syringomyelia due to Chiari 1.5 malformation (CM) in a 12-year-old girl was serially imaged with 3 T MRI over 4 years. The serial MRI showed reduction in size of the syrinx, without any surgical intervention or CM improvement, but rather due to spontaneous spinal cord tear. Read More

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December 2021

An anatomical model for studying cerebellar tonsillar herniation related to raised intracranial pressure.

Clin Anat 2022 Mar 20;35(2):251-255. Epub 2021 Dec 20.

Department of Neurosurgery and Ochsner Neuroscience Institute, Ochsner Health System, New Orleans, Louisiana, USA.

Brain herniation is one of the most feared complications of many neurological pathologies. However, current understanding of the mechanisms behind brain herniation syndromes is poor. By investigating the correlations between raised intracranial pressure (ICP) and herniation of the cerebellar tonsils, we hope to develop a model that can be used to study intracranial fluid dynamics and its effects on brain tissue. Read More

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New Association between Idiopathic Scoliosis and Luckenschadel Skull (Lacunar Skull).

Aysel Vehapoglu

Med Princ Pract 2022 7;31(2):125-132. Epub 2021 Dec 7.

Department of Pediatrics, Faculty of Medicine, Bezmialem Vakıf University, Istanbul, Turkey.

Objective: Luckenschadel skull is a skull that is radiologically characterized by lacunae in the cranial vault. To date, although the association between neurological abnormalities and scoliosis is well recognized, no relationship between idiopathic scoliosis and a lacunar skull has been defined. We explored the incidence and time courses of lacunar skulls in patients with idiopathic scoliosis. Read More

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Chiari i malformation presenting as unilateral toe walking in a 17-month-old.

J Paediatr Child Health 2022 Jan 3;58(1):214-215. Epub 2021 Dec 3.

Department of Paediatrics, Bathurst Base Hospital, Bathurst, New South Wales, Australia.

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January 2022

Fourth Ventricle Dimensions Increase in Chiari Malformation Type 1.

Turk Neurosurg 2022 ;32(2):305-308

Sivas Cumhuriyet University, School of Medicine, Department of Neurosurgery, Sivas, Turkey.

Aim: To investigate whether fourth ventricle dimensions and tentorial angulation differ in a healthy control population in our evaluation of patients with CM-1 malformation using MRI.

Material And Methods: The radiological and demographic data from 251 patients with CM-1 followed in our clinic between 2014 and 2019 were compared with data from 273 persons in a healthy control group. Fourth ventricle dimensions, amount of cerebellar tonsillar herniation, and tentorium twinning angle were measured. Read More

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