5,915 results match your criteria: "Chiari Malformation"

Background: Chiari I malformation (CMI) is characterized by inferior descent of the cerebellar tonsils through the foramen magnum and is associated with headache and neck pain. Many morphometric research efforts have aimed to describe CMI anatomy in the midsagittal plane using classical measurement techniques such as linear dimensions and angles. These methods are less frequently applied to parasagittal features and may fall short in quantifying more intricate anatomy with fewer distinct homologous landmarks.

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  • Rubinstein-Taybi syndrome (RTS) is a rare genetic disorder characterized by unique physical traits, and this case series focuses on the neurosurgical care of RTS patients at Children's Hospital of Pittsburgh.
  • A retrospective review of RTS cases from 2010 to 2023 found that 95% of patients had a genetic variant linked to RTS type 1, with many presenting issues such as tethered cord syndrome and Chiari malformations.
  • The study highlights a notably high occurrence of Chiari I malformation requiring surgery, illustrating the need for neurosurgical intervention in RTS management.
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Meningitis due to is extremely rare, with an annual incidence of 1-3%. In this report, we present a rare case involving meningitis, an infected graft, and an infected fluid collection with two forms of in a patient who received a bovine brain graft status post-decompression and suboccipital craniectomy with C1 laminectomy and duraplasty for Chiari malformation. The treatment approach included surgical debridement and graft retention, followed by an extended course of antibiotic treatment with oxacillin and rifampin.

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  • A 76-year-old woman with long-lasting pain from Chiari malformation type 1 and syringomyelia had surgery but her pain came back after 10 months.
  • Doctors tried spinal cord stimulation (SCS) to help her pain, but only a special technique called FAST™ worked well along with another method called Contour™.
  • After using this method for three years, her pain improved a lot, even with lower doses of pain medication, and she had no bad side effects.
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  • * Surgical goals include repairing the defect, preserving brain tissue, ensuring skin coverage, and managing hydrocephalus, but many infants still face lasting disabilities.
  • * A case study describes a newborn diagnosed with this condition, who underwent surgery and later developed hydrocephalus, eventually requiring palliative care after respiratory issues and dependency on a ventilator.
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The peripartum management of obstetric patients with Chiari type I malformation remains a challenge due to the degree of cerebellar tonsillar herniation and a paucity of published evidence. There is concern about neuraxial anaesthetic blocks and uncertainty regarding the optimum mode of delivery. We systematically searched the literature for the obstetric management of patients with Chiari type I malformation, independent of publication date and language.

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Chiari I Deformity: Beyond 5 mm below the Foramen Magnum.

Radiographics

September 2024

From the Department of Imaging Sciences, New York University Langone Health, 550 1st Ave, New York, NY 10016 (J.R.L.); and Departments of Neurosurgery (C.W.) and Imaging Sciences (S.E.), University of Rochester Medical Center, Rochester, NY.

Although originally described as a consecutive series of pathologic changes, Chiari syndrome represents a spectrum of disease divided into two subsets: development deformities of the paraxial mesoderm manifesting after birth (types 0-1.5) and true congenital malformations due to failure of neural tube closure present in utero (types 2-5). Heterogeneity among patients with a Chiari deformity and incomplete understanding of its pathophysiologic characteristics have led to inconsistency in radiologic reporting and difficulty in defining appropriate management strategies tailored to an individual patient's condition.

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Invited Commentary: Chiari 1 Deformity: Not a Malformation.

Radiographics

September 2024

From the Edward B. Singleton Department of Radiology, Texas Children's Hospital and Baylor College of Medicine, 6701 Fannin St, Ste 470, Houston, TX 77030.

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Meta-analysis and systematic review. To understand the role of endoscopic third ventriculostomy (ETV) for the treatment of concurrent Chiari Malformation Type I (CMI) and hydrocephalus in adults. A literature search on PubMed and Medline with MeSH terms relating to ETV and CMI identified 155 articles between 1988 and 2024.

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Atlanto-occipital assimilation is a congenital craniovertebral junction malformation where the partial or complete fusion of the atlas and occiput occurs. Atlanto-occipital assimilation can be associated with numerous complications, including basilar invagination, atlantoaxial subluxation, Chiari malformation, congenital vertebral bodies fusion, and cord compression. As a result, vigilant identification and accurate reporting of atlanto-occipital assimilation are essential.

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  • - The text discusses pseudopapilledema, specifically caused by optic disc drusen (ODD), which can look similar to papilledema, posing a diagnostic challenge, especially in a young boy with Chiari malformation type 1.
  • - In such cases, advanced imaging techniques like fundus autofluorescence, optical coherence tomography (OCT), and B-scan ultrasonography are essential to differentiate between ODD and true papilledema due to elevated intracranial pressure (ICP).
  • - The consensus among specialists is to monitor patients with annual eye exams and imaging to catch any changes in the optic nerve head, as the presence of ODD doesn't rule out the possibility of future issues related to increased ICP
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Introduction In recent years, more emphasis has been placed on improving the health-related quality of life (HRQOL) in children with spina bifida (SB). Chronic disability is understood to impact various aspects of the person's life, family, and social functioning, in addition to the specific needs of the disease. The HRQOL is done to assess the patient's quality of life (QOL) in various domains including physical and mental.

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Cervical syringomyelia associated with cervical disc disease.

Ideggyogy Sz

July 2024

Department of Neurosurgery, Faculty of Medicine, Harran University, Şanliurfa, Turkey.

Background And Purpose:

Syringomyelia is a neurological condition in which a longitudinal fluid-filled cavity is formed within the spinal cord. It usually occurs in the cervical region and is associated with Chiari malformation, infections, trauma, and tumors of the spinal cord. However, syringomyelia associated with cervical disc disease (SCD) is very rare and only a few cases have been reported so far.

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Chiari malformations (CM) are a spectrum of hindbrain abnormalities involving the cerebellum, brainstem, skull base, and cervical cord. The most common is Chiari I malformation, in which the cerebellar tonsils descend through the foramen magnum. As opposed to types II-IV, which are congenital, type I can manifest in late childhood or adulthood with headaches and focal neurological symptoms.

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Lhermitte-Duclos disease (LDD) is a rare entity, which may or may not be associated with Cowden syndrome (CS). The authors present a 26-year-old male with a history of emergency treatment due to acute obstructive hydrocephalus and apparent Chiari malformation. In posterior evaluation, mild cerebellar symptoms, mucocutaneous lesions, and a left hemispheric cerebellar lesion were evident.

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Basilar invagination in a Chiari malformation associated with osteogenesis imperfecta in the pediatric population is a rare entity. We report a case of a seven-year-old female who presented with sudden-onset bilateral spastic quadriplegia and evidence of a basilar invagination on MRI. She underwent emergency decompression of the impinging odontoid via transoral approach followed by posterior wiring and fusion of the C1 and C2 vertebrae.

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Objectives: The aim of the current study was to describe the magnitude and pattern of congenital anomalies on routine second-trimester ultrasound and its practical implication in the management of pregnant women with fetal anomalies at a novice center in East Africa.

Methods: This cross-sectional study was conducted from September 2021 to May 2022 among women who had second-trimester anatomic scanning. Data were collected using a structured questionnaire and analyzed using SPSS version 23.

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The management of Chiari malformations (CMs) remains a clinical challenge and a topic of great controversy. Results may vary between children and adults. The purpose of the current single-center study is to critically assess the one-year surgical outcomes of a cohort of 110 children with CM-1 or CM-1.

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Background: Currently, there are no diagnostic imaging-based studies that have focused specifically on the craniocervical morphology of Pomeranian dogs in relation to Chiari-like malformation and syringomyelia (CM/SM). The aims of Part I of this two-part study are to assess the intra-observer, interobserver, and intermodality reliability and agreement for various aspects of the craniocervical morphology of Pomeranians with and without CM/SM.

Methods: Prospectively, Pomeranians were included that underwent both CT and MRI studies of the head and cervicothoracic vertebral column.

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Article Synopsis
  • The study aimed to explore clinical and imaging factors linked to sleep-disordered breathing (SDB) in children with Chiari type I malformation and assess the effectiveness of foramen magnum decompression (FMD) in treating SDB.
  • A review of charts from children diagnosed with Chiari from 2002 to 2022 revealed a significant prevalence of SDB, with notable associations found for factors like lower cranial nerve dysfunction and tonsillar position.
  • Post-FMD, children with SDB exhibited a substantial decrease in apnea-hypopnea index scores, and 35% of those treated achieved complete resolution of their SDB.
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BACKGROUND Neurodevelopmental disorders (NDD) are umbrella disorders that encompass global developmental delay (GDD), intellectual disability, autism spectrum disorders, motor developmental disorders, and sleep disorders. Both GDD and autism spectrum disorder are common and yet clinically and genetically heterogeneous disorders. Despite their high prevalence and the advent of sequencing detection methods, the genomic etiology of GDD and autism spectrum disorder in most patients is largely unknown.

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