2,345 results match your criteria Imaging in Chiari I Malformation

Development of Progressive Chiari I Malformation in a Child with Unilateral Sporadic Retinoblastoma.

J Curr Ophthalmol 2021 Jan-Mar;33(1):88-90. Epub 2021 Mar 26.

Ophthalmic Epidemiology Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Purpose: To report a case of progressive Chiari malformation type I (CIM) in a patient with unilateral sporadic retinoblastoma (RB) treated with intra-arterial chemotherapy (IAC) and enucleation.

Methods: A 5-year-old male patient with a history of RB in his left eye treated with IAC and enucleation presented to our clinic for routine RB surveillance. Radiotherapy had not been used for the treatment of his RB. Read More

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Acquired unilateral upper limb hypertrophy as a late complication of tuberculous meningitis complicated by Chiari 1 malformation and syringomyelia.

BMJ Case Rep 2021 May 25;14(5). Epub 2021 May 25.

Paediatrics and Child Health, Stellenbosch University Faculty of Medicine and Health Sciences, Cape Town, Western Cape, South Africa.

Syringomyelia associated with tuberculous meningitis (TBM) is an extremely rare condition. Only a few adult cases have been reported. A 12-year-old woman, who previously suffered TBM at the age of 6 months, presented with a long-standing history of right upper limb panhypertrophy, dissociate anaesthesia, frequent headaches, scoliosis and acquired macrocephaly. Read More

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Nonoperative management of enlarging syringomyelia in clinically stable patients after decompression of Chiari malformation type I.

J Neurosurg Pediatr 2021 May 21:1-6. Epub 2021 May 21.

2Division of Neurosurgery, Children's National Health System, Washington, DC.

Objective: The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I).

Methods: A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. Read More

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Embryological considerations and evaluation of congenital anomalies of craniovertebral junction: A single-center experience.

Tzu Chi Med J 2021 Apr-Jun;33(2):175-180. Epub 2020 Oct 15.

Department of Medicine, INHS Kalyani, Visakhapatnam, Andhra Pradesh, India.

Objectives: Craniovertebral junction (CVJ) abnormalities constitute a group of treatable neurological disorders, especially in the Indian subcontinent. Thus, it is essential that clinicians should be able to make a precise diagnosis of abnormalities and rule out important mimickers on multidetector computed tomography (MDCT) as this information ultimately helps determine the management, prognosis, and quality of life of patients. CVJ is the most complex part of the cervical region. Read More

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October 2020

Imaging characteristics associated with surgery in Chiari malformation type I.

J Neurosurg Pediatr 2021 Apr 23:1-9. Epub 2021 Apr 23.

1Division of Pediatric Neurosurgery, Department of Neurosurgery; and.

Objective: In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression.

Methods: Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. Read More

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Weighing In on the Controversy: Preoperative Imaging in Unicoronal Craniosynostosis Leads to Strategic Changes in Surgical Care.

Plast Reconstr Surg 2021 May;147(5):1133-1139

From the Division of Plastic Surgery, Department of Surgery, and the Department of Neurosurgery, University of Michigan.

Background: Clinical findings in children with unilateral coronal craniosynostosis are characteristic, and therefore clinicians have questioned the need for confirmatory imaging. Preoperative computed tomographic imaging is a powerful tool for diagnosing associated anomalies that can alter treatment management and surgical planning. The authors' aim was to determine whether and how routine preoperative imaging affected treatment management in unilateral coronal craniosynostosis patients within their institution. Read More

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A case report of Arnold Chiari type 1 malformation in acromesomelic dwarf infant.

Pan Afr Med J 2021 18;38:58. Epub 2021 Jan 18.

Siddhi Diagnostic and Research Centre, Nallasopara West, Maharashtra, India.

Arnold Chiari malformation is one of the commonest cause of congenital hydrocephalus. Cause of fetal development of cerebellar tonsils remains unknown and may be diagnosed at later in life. The association of Arnold Chiari malformation with acromesomelic dwarfism is not known. Read More

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Pulsatile cerebrospinal fluid dynamics in Chiari I malformation syringomyelia: Predictive value in posterior fossa decompression and insights into the syringogenesis.

J Craniovertebr Junction Spine 2021 Jan-Mar;12(1):15-25. Epub 2021 Mar 4.

Department of Neurological Surgery, University of Illinois at Chicago, Chicago, IL, USA.

Background: Pathophysiological mechanisms underlying the syringomyelia associated with Chiari I malformation (CM-1) are still not completely understood, and reliable predictors of the outcome of posterior fossa decompression (PFD) are lacking accordingly. The reported prospective case-series study aimed to prove the existence of a pulsatile, biphasic systolic-diastolic cerebrospinal fluid (CSF) dynamics inside the syrinx associated with CM-1 and to assess its predictive value of patients' outcome after PFD. Insights into the syringogenesis are also reported. Read More

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Transcranial Doppler Plateau Wave in a Patient with Pseudo-Chiari Malformation.

Acta Neurochir Suppl 2021 ;131:319-322

Intensive Care Unit, Clinicas Hospital School of Medicine, University of the Republic, Montevideo, Uruguay.

Case Report: A 26-year-old woman presented a superior sagittal and transverse sinus thrombosis with venous infarction. Anticoagulation was started. Six months later headache and visual impairment developed, and intracranial hypertension was diagnosed-secondary pseudotumor cerebri. Read More

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A Possible Association Between Zika Virus Infection and CDK5RAP2 Mutation.

Front Genet 2021 19;12:530028. Epub 2021 Mar 19.

Center for Research on Congenital Anomalies and Rare Diseases (CIACER), Department of Basic Medical Sciences, Universidad Icesi, Cali, Colombia.

Introduction: Flaviviridae family belongs to the Spondweni serocomplex, which is mainly transmitted by vectors from the genus. Zika virus (ZIKV) is part of this genus. It was initially reported in Brazil in December 2014 as an unknown acute generalized exanthematous disease and was subsequently identified as ZIKV infection. Read More

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Anesthetic management for cesarean delivery in a woman with congenital atlantoaxial dislocation and Chiari type I anomaly: a case report and literature review.

BMC Pregnancy Childbirth 2021 Apr 1;21(1):272. Epub 2021 Apr 1.

Department of Anesthesiology, The Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China.

Background: The preferable choice of anesthesia for the patients with congenital atlantoaxial dislocation (CAAD) and type I Arnold Chiari malformations (ACM-I) has been a very confusing issue in clinical practice. We describe the successful administration of combined spinal-epidural anesthesia for a woman with CAAD and ACM-1 accompanied by syringomyelia.

Case Presentation: Our case report presents the successful management of a challenging obstetric patient with CAAD and ACM-1 accompanied by syringomyelia. Read More

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Chiari 1: Is decompression always necessary previous to scoliosis surgery?

Spine Deform 2021 Apr 1. Epub 2021 Apr 1.

Servicio de Patología Espinal, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Combate de Los Pozos 1881CABA, C1245AAM, Buenos Aires, Argentina.

The association between Chiari 1 malformation and scoliosis is well known in the literature. Prevalence has increased after the advent of magnetic resonance imaging. In children with this association, prophylactic suboccipital decompression prior to scoliosis correction is a common surgical procedure although the rationale for this surgical management and whether not performing it may lead to spinal cord injury has not been clearly elucidated. Read More

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Endoscopic Transnasal Odontoidectomy.

J Neurol Surg B Skull Base 2021 Feb 17;82(Suppl 1):S8-S9. Epub 2020 Sep 17.

Department of Otolaryngology-Head and Neck Surgery, Augusta Universtiy Medical Center, Augusta Georgia, United States.

 The video demonstrates the steps for an endoscopic transnasal approach for resection of the odontoid causing platybasia with basilar invasion.  The video reviews the clinical presentation, preoperative workup and imaging, patient positioning, technical nuances of the procedure, reconstruction, and clinical outcomes including postoperative imaging.  The surgery was performed by a skull base team including otolaryngologists and neurological surgeons at a large regional tertiary care facility. Read More

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February 2021

Endoscopic Endonasal Odontoidectomy Preserving Atlantoaxial Stability: a Pediatric Case.

J Neurol Surg B Skull Base 2021 Feb 4;82(Suppl 1):S2-S3. Epub 2020 Mar 4.

Department of Neurosurgery, University of Insubria, Ospedale di Circolo Fondazione Macchi, Varese, Italy.

 We illustrate endoscopic endonasal odontoidectomy for the Chiari-I malformation respecting craniovertebral junction (CVJ) stability.  Case report of a 12-year-old girl affected by the Chiari-I malformation. Magnetic resonance imaging (MRI) showed tonsillar herniation, basilar invagination, and dental retroversion, causing angulation and compression of the bulbomedullary junction. Read More

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February 2021

Management of Pre-existing Ventriculoperitoneal Shunt in Posterior Vault Distraction for Lambdoid Craniosynostosis: A Case Report and Technical Note.

Cureus 2021 Jan 20;13(1):e12814. Epub 2021 Jan 20.

Neurosurgery, Norton Children's Hospital, Louisville, USA.

Posterior vault distraction osteogenesis (DO) is an emerging treatment option for craniosynostosis. Operative nuances detailing surgical management are being described with increasing use and experience. In this article, we discuss the surgical management of an 8-month-old male with a ventriculoperitoneal shunt (VPS) diagnosed with bilateral lambdoid craniosynostosis and Chiari I malformation. Read More

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January 2021

[Treatment of syringomyelia in patients with arachnopathy within craniovertebral junction].

Zh Vopr Neirokhir Im N N Burdenko 2021 ;85(1):10-18

Pirogov Russian National Research Medical University, Moscow, Russia.

One of the causes of syringomyelia is arachnopathy following an infectious or non-infectious inflammation of the arachnoid membrane. It is extremely rare that adhesive arachnoiditis develops within the occipital cistern and impairs cerebrospinal fluid flow from the foramen of Magendie, along posterior cerebellar surface and into spinal subarachnoid space. These process result syringomyelia. Read More

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February 2021

A novel classification and its clinical significance in Chiari I malformation with syringomyelia based on high-resolution MRI.

Eur Spine J 2021 Feb 5. Epub 2021 Feb 5.

Department of Neurosurgery, Division of Spine, China, International Neurological Institute, Xuanwu Hospital, Capital Medical University, 45 Changchun Street, Beijing, 100053, People's Republic of China.

Background: The existing classification in Chiari I malformation (CM-I) has limited significance for the selection of surgical methods.

Objective: The purpose of this study was to investigate the surgery of CM-I with syringomyelia based on the high-resolution MR imaging (HRMRI) findings.

Methods: Data from 115 patients were collected and retrospectively analyzed. Read More

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February 2021

Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature.

Childs Nerv Syst 2021 Jan 25. Epub 2021 Jan 25.

The New Jersey Pediatric Neuroscience Institute, Morristown, NJ, USA.

We present a rare case of an 8-year-old male with Klippel-Trenaunay syndrome (KTS) and a Chiari I malformation (CIM). Magnetic resonance imaging (MRI) to investigate facial asymmetry and speech delay at age two revealed CIM with cerebellar tonsils 1.3 cm below the foramen magnum without syringomyelia. Read More

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January 2021

Chiari I malformation in patients with RASopathies.

Childs Nerv Syst 2021 Jun 6;37(6):1831-1836. Epub 2021 Jan 6.

Department of Neurosurgery, Children's Hospital of Soochow University, 92 Zhongnan Street, Suzhou, 215006, Jiangsu, People's Republic of China.

Purpose: Chiari I malformation (CIM) is a common pediatric neurologic anomaly which could be associated with a variety of genetic disorders. However, it is not always clear whether the observed associations between CIM and RASopathies are real or random. The knowledge of the real association could provide useful guidance to clinicians. Read More

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Cough syncope and hyperventilation-induced convulsion in Chiari 1.5 malformation.

Neurol Sci 2021 May 3;42(5):2069-2073. Epub 2021 Jan 3.

Department of Neurosurgery, Nara Medical University, Kashihara, Nara, Japan.

Chiari malformation type I (CM1) is defined as cerebellar tonsillar herniation below the level of the foramen magnum. Syncope, especially cough syncope, is a rare but important symptom of CM1 patients. Here, we report a CM1 patient, in combination with brainstem herniation (CM1. Read More

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Rare and de novo coding variants in chromodomain genes in Chiari I malformation.

Am J Hum Genet 2021 01 21;108(1):100-114. Epub 2020 Dec 21.

Department of Pediatrics, Washington University, St. Louis, MO 63110, USA; Department of Orthopaedic Surgery, Washington University, St. Louis, MO 63110, USA; Department of Neurology, Washington University, St. Louis, MO 63110, USA.

Chiari I malformation (CM1), the displacement of the cerebellum through the foramen magnum into the spinal canal, is one of the most common pediatric neurological conditions. Individuals with CM1 can present with neurological symptoms, including severe headaches and sensory or motor deficits, often as a consequence of brainstem compression or syringomyelia (SM). We conducted whole-exome sequencing (WES) on 668 CM1 probands and 232 family members and performed gene-burden and de novo enrichment analyses. Read More

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January 2021

In Reply to the Letter to the Editor Regarding "'Two-Birds-One-Stone' Approach for Treating an Infant with Chiari I Malformation and Hydrocephalus: Is Cerebrospinal Fluid Diversion as Sole Treatment Enough?"

World Neurosurg 2021 01;145:528-529

Department of Neurosurgery, Tulane Medical Center, New Orleans, Louisiana, USA; Department of Neurosurgery, Ochsner Medical Center, New Orleans, Louisiana, USA.

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January 2021

Chiari 1 malformation and exome sequencing in 51 trios: the emerging role of rare missense variants in chromatin-remodeling genes.

Hum Genet 2021 Apr 18;140(4):625-647. Epub 2020 Dec 18.

Medical Genetics Unit, Department of Clinical and Experimental Biomedical Sciences "Mario Serio", University of Florence, Florence, Italy.

Type 1 Chiari malformation (C1M) is characterized by cerebellar tonsillar herniation of 3-5 mm or more, the frequency of which is presumably much higher than one in 1000 births, as previously believed. Its etiology remains undefined, although a genetic basis is strongly supported by C1M presence in numerous genetic syndromes associated with different genes. Whole-exome sequencing (WES) in 51 between isolated and syndromic pediatric cases and their relatives was performed after confirmation of the defect by brain magnetic resonance image (MRI). Read More

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Management of Chiari in pregnancy.

J Clin Neurosci 2021 01 13;83:153-154. Epub 2020 Dec 13.

Department of Clinical Medicine, Faculty of Medicine, Health and Human Sciences, Macquarie University, Suite 305, Level 3, 2 Technology Place, Macquarie Park, NSW 2109, Australia. Electronic address:

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January 2021

Outcomes of Dura Splitting Decompression Versus Posterior Fossa Decompression With Duraplasty in the Treatment of Chiari I Malformation: A Systematic Review and Meta-analysis.

World Neurosurg 2021 Mar 5;147:105-114. Epub 2020 Dec 5.

Department of Neurosurgery, People's Hospital of Xinjiang Uygur Autonomous Region, Xinjiang, People's Republic of China. Electronic address:

Background: Surgery is the definitive treatment option for symptomatic Chiari malformation I (CMI), but there is no clear consensus as to the preferred surgical method. This study aimed to quantitatively assess and compare the effect and safety of dura splitting decompression (DSD) and posterior fossa decompression with duraplasty (PFDD) in treating patients with CMI.

Methods: A literature search of EMBASE, MEDLINE, PubMed, Cochrane Library, and Web of Science databases was conducted. Read More

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Chiari I malformation with craniosynostosis and persistent falcine sinus draining into the straight sinus.

Morphologie 2020 Dec 4. Epub 2020 Dec 4.

Department of neurosurgery, Tulane center for clinical neurosciences, Tulane university School of Medicine, 131 S. Robertson St. Suite, 1300 New Orleans, LA 70112, USA; Department of neurology, Tulane center for clinical neurosciences, Tulane university School of Medicine New Orleans, USA; Department of structural & cellular biology, Tulane university school of Medicine, New Orleans, LA, USA; Department of neurosurgery and ochsner neuroscience Institute, Ochsner health System, New Orleans, LA, USA; Department of anatomical sciences, St. George's university, St. George's, Grenada, West Indies.

Owing to the interconnected nature of the brain, anatomical variations in one area often coincide with, or are caused by, abnormalities in another. During dissection of a specimen with both Chiari I malformation and craniosynostosis, a persistent falcine sinus was observed to drain into the straight sinus. Such a variant should be noted by physicians as it could alter treatment plans and require more detailed imaging procedures prior to surgical correction. Read More

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December 2020

A Case of Luscan-Lumish Syndrome: Possible Involvement of Enhanced GH Signaling.

J Clin Endocrinol Metab 2021 Mar;106(3):718-723

Division of Diabetes and Endocrinology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.

Context: Luscan-Lumish syndrome (LLS) is characterized by postnatal overgrowth, obesity, Chiari I malformation, seizures, and intellectual disability. SET domain-containing protein 2 (SETD2) is a histone methyltransferase, where mutations in the gene are associated with the development of LLS. However, mechanisms underlying LLS remain unclear. Read More

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Acute Visual Loss Secondary to Arnold Chiari Type I Malformation Completely Resolving After Decompressive Posterior Fossa Surgery.

Int Med Case Rep J 2020 18;13:657-661. Epub 2020 Nov 18.

Department of Ophthalmology, University of Athens, G Gennimatas General Hospital, Athens, Greece.

We describe the case of a 22-year-old woman of southeast-Asian origin, presenting with unilateral sudden visual loss after a self-healing hearing loss a week before. Ophthalmological examination showed visual acuity of light perception in the left eye, mild RAPD, normal ocular motility and an elevated optic disc with indistinct margins. Neurological examination showed no acute pathology and brain CT-MRI imaging revealed a small-almost subclinical-herniation of the cerebellar tonsils. Read More

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November 2020

In Reply to the Letter to the Editor Regarding "Chiari Formation and Syringomyelia Associated with Hirayama Disease".

Atul Goel

World Neurosurg 2020 12;144:340

Department of Neurosurgery, K.E.M. Hospital and Seth G.S. Medical College, Parel, Mumbai, India. Electronic address:

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December 2020