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REVIEW
Alaina Dhawan, Jillian Dhawan, Ajay N Sharma, Daniel B Azzam, Ahmed Cherry, Michael G Fehlings
OBJECTIVE: 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. METHODS: 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...
April 12, 2024: Journal of Neurosurgery. Spine
#2
JOURNAL ARTICLE
Enver I Bogdanov, John D Heiss
The diagnosis of Chiari I malformation is straightforward in patients with typical signs and symptoms of Chiari I malformation and magnetic resonance imaging (MRI) confirming ≥5 mm of cerebellar tonsillar ectopia, with or without a syrinx. However, in many cases, Chiari I malformation is discovered incidentally on MRI to evaluate global headache, cervical radiculopathy, or other conditions. In those cases, the clinician must consider if cerebellar tonsillar ectopia is related to the presenting symptoms...
2024: Advances and Technical Standards in Neurosurgery
#3
JOURNAL ARTICLE
Anand M Hatgaonkar, Sandeep M Mahajan, Kajal A Hatgoankar, Gulshan R Bandre
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 identify and 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...
March 2024: Curēus
#4
JOURNAL ARTICLE
Alexander T Yahanda, Joyce Koueik, Laurie L Ackerman, P David Adelson, Gregory W Albert, Philipp R Aldana, Tord D Alden, Richard C E Anderson, David F Bauer, Tammy Bethel-Anderson, Karin Bierbrauer, Douglas L Brockmeyer, Joshua J Chern, Daniel E Couture, David J Daniels, Brian J Dlouhy, Susan R Durham, Richard G Ellenbogen, Ramin Eskandari, Herbert E Fuchs, Gerald A Grant, Patrick C Graupman, Stephanie Greene, Jeffrey P Greenfield, Naina L Gross, Daniel J Guillaume, Todd C Hankinson, Gregory G Heuer, Mark Iantosca, Bermans J Iskandar, Eric M Jackson, George I Jallo, James M Johnston, Bruce A Kaufman, Robert F Keating, Nickalus R Khan, Mark D Krieger, Jeffrey R Leonard, Cormac O Maher, Francesco T Mangano, Jonathan Martin, J Gordon McComb, Sean D McEvoy, Thanda Meehan, Arnold H Menezes, Michael S Muhlbauer, Brent R O'Neill, Greg Olavarria, John Ragheb, Nathan R Selden, Manish N Shah, Chevis N Shannon, Joshua S Shimony, Matthew D Smyth, Scellig S D Stone, Jennifer M Strahle, Mandeep S Tamber, James C Torner, Gerald F Tuite, Elizabeth C Tyler-Kabara, Scott D Wait, John C Wellons, William E Whitehead, Tae Sung Park, David D Limbrick, Raheel Ahmed
OBJECTIVE: 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...
April 5, 2024: Journal of Neurosurgery. Pediatrics
#5
JOURNAL ARTICLE
Ignazio G Vetrano, Arianna Barbotti, Tommaso Francesco Galbiati, Sabrina Mariani, Alessandra Erbetta, Luisa Chiapparini, Veronica Saletti, Laura G Valentini
Background: In patients with Chiari 1.5 malformation (CM1.5), a more aggressive disease course and an increased association with craniovertebral junction (CVJ) anomalies has been suggested. The best management of this subgroup of patients is not clearly defined, also due to the lack of specific series elucidating this anomaly's peculiar characteristics. Methods: We evaluated a series of 33 patients (25 females, 8 males; mean age at surgery: 13 years) fulfilling the criteria for Chiari 1.5 diagnosis who underwent posterior fossa decompression and duraplasty (PFDD) between 2006 and 2021...
March 15, 2024: Journal of Clinical Medicine
#6
JOURNAL ARTICLE
Shanthi Sarma, Ellen Quinn, Grace Branjerdporn
No abstract text is available yet for this article.
March 26, 2024: Journal of ECT
#7
JOURNAL ARTICLE
Alexander Eremiev, David B Kurland, Alexander T M Cheung, Danielle Cook, Yosef Dastagirzada, David H Harter, Juan Rodriguez-Olaverri, Douglas Brockmeyer, Joshua M Pahys, Daniel Hedequist, Matthew Oetgen, Amer F Samdani, Richard C E Anderson
OBJECTIVE: The purpose of this study was to identify factors associated with fusion success among pediatric patients undergoing occiput-C2 rigid instrumentation and fusion. METHODS: 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...
March 22, 2024: Journal of Neurosurgery. Pediatrics
#8
JOURNAL ARTICLE
David Zarrin, Keshav Goel, Wi Jin Kim, Langston T Holly, Ulrich Batzdorf
INTRODUCTION: Chiari 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. METHOD: We retrospectively reviewed patients who underwent revision of Chiari decompression at our institution from 2005-2020...
March 13, 2024: World Neurosurgery
#9
JOURNAL ARTICLE
Göksal Günerhan, Emin Çağıl, Zeynep Dağlar, Uğur Kemal Gündüz, Ali Dalgıç, Ahmet Deniz Belen
Objective The aim this study is to present the results of the minimal invasive neuroendoscopic-assisted system application as an alternative to traditional surgery in patients with Chiari malformation type 1 (CM type 1) with/without syringomyelia. Design, Setting, and Participants In the study, data of 22 symptomatic patients were prospectively collected. Before and after the operation, patient characteristics, computed tomography, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) flow dynamics MRI, and outcome scales scores were recorded...
April 2024: Journal of Neurological Surgery. Part B, Skull Base
#10
JOURNAL ARTICLE
Momin M Mohis, Kevin Cordeiro, Sarah Larson, Catharine Garland, James A Stadler
BACKGROUND: Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors' knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair. OBSERVATIONS: A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4...
March 4, 2024: J Neurosurg Case Lessons
#11
JOURNAL ARTICLE
Turkka Kirjavainen, Päriä Miraftabi, Laura Martelius, Atte Karppinen
OBJECTIVES: Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. METHODS: This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008-2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging...
February 16, 2024: Sleep Medicine
#12
JOURNAL ARTICLE
Samir Moniruzzaman, Aku Kaipainen, Joona Tervonen, Jukka Huttunen, Henna-Kaisa Jyrkkänen, Terhi J Huuskonen, Susanna Rantala
PURPOSE: The 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. METHODS: In 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...
February 28, 2024: Acta Neurochirurgica
#13
JOURNAL ARTICLE
Silvia Boeri, Monica Bodria, Rosa Maria Ammendola, Thea Giacomini, Domenico Tortora, Lino Nobili, Michela Malacarne, Andrea Rossi, Enrico Verrina, Giorgio Piaggio, Maria Margherita Mancardi, Mariasavina Severino
BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) represent 20-30% of all birth defects and are often associated with extra-renal malformations. We investigated the frequency of brain/spine malformations and neurological features in children with CAKUT. METHODS: We reviewed the clinico-radiological and genetic data of 199 out of 1,165 children with CAKUT evaluated from 2006 to 2023 (99 males, mean age at MRI 6.4 years) who underwent brain and/or spine MRI...
February 20, 2024: Pediatric Nephrology
#14
JOURNAL ARTICLE
Eric Vigneul, Nicole Del Gaudio, Loïc de Nijs, Christian Raftopoulos
BACKGROUND: Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended...
February 19, 2024: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
#15
JOURNAL ARTICLE
Eriko Nishi, Kumiko Yanagi, Tadashi Kaname, Nobuhiko Okamoto
BACKGROUND: Rauch-Steindl syndrome (RAUST) is a very rare genetic syndrome caused by a pathogenic variant in NSD2 on chromosome 4p16.3. Although NSD2 was previously thought to be the major gene in Wolf-Hirschhorn syndrome (WHS), a contiguous gene syndrome of chromosome 4p16.3 deletion, RAUST has been found to present different facial and clinical features from WHS. In this study, we report the details of two newly diagnosed individuals with RAUST in order to better understand the molecular and clinical features of RAUST...
February 2024: Molecular Genetics & Genomic Medicine
#16
JOURNAL ARTICLE
Xuan-Hong Tomai, Huu-Trung Nguyen, Thanh-Truc Nguyen Thi, Tuan-Anh Nguyen, Thuy-Vy Nguyen
BACKGROUND: Chiari malformation is one of the most common Central nervous system (CNS) abnormalities that can be detected in routine fetal scanning. Chiari malformation type I (CMI) is a congenital defect characterized by a displacement of the cerebellar tonsils through the foramen magnum. The etiology of CMI has not been well established and suggested having multifactorial contributions, especially genetic deletion. Clinical characteristics of this anomaly may express in different symptoms from neurological dysfunction and/or skeletal abnormalities in the later age, but it is rarely reported in pregnancy...
February 13, 2024: Journal of Medical Case Reports
#17
JOURNAL ARTICLE
Junji Koyama, Nobuyuki Akutsu, Atsufumi Kawamura
PURPOSE: In posterior fossa decompression for pediatric Chiari malformation type 1 (CM-1), duraplasty methods using various dural substitutes have been reported to improve surgical outcomes and minimize postoperative complications. To obtain sufficient posterior fossa decompression without cerebrospinal fluid-related complications, we developed a novel duraplasty technique using a combination of a pedicled dural flap and collagen matrix. The objective of this study was to describe the operative nuances of duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric CM-1...
February 7, 2024: Acta Neurochirurgica
#18
REVIEW
Robert Henderson, Rahul Lakshmanan, Aden McLaughlin, Omar Bangash, Snigdha Saha, Richard Carey-Smith
BACKGROUND: Chiari malformations are a rare group of rhomboencephalic abnormalities involving the brain, craniocervical junction and spine. They may manifest in a variety of clinical presentations which relate to the variable involvement of the cerebellum, brainstem, lower cranial nerves, spinal cord and altered CSF flow dynamics. METHOD: We report an unusual case of incidental diagnosis of a type I Chiari malformation with secondary cystic cerebellar tonsillar encephalomalacia and holocord syrinx following investigation of a 5YO girl presenting with heel swelling related to progressive neuropathic osteoarthropathy of the posterior calcaneal body and apophysis...
February 1, 2024: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
#19
Taha F Hassan, Ryan D Morgan, Kirie M Psaromatis, Benedicto C Baronia
Under certain classifications, a Chiari type I (CMI) malformation can be characterized as a herniation of the cerebellar tonsils greater than 3 mm. Patients with CMI often have a smaller posterior fossa volume, which results in a smaller amount of space for the cerebellum, leading to the herniation of the cerebellar tonsils through the foramen magnum. Although inheritable factors such as posterior fossa volume can be traced to specific genes, there has not been a gene that can be attributed to directly causing CMI...
April 2024: Radiology Case Reports
#20
JOURNAL ARTICLE
Elie Côté, Y Arun Reginald, Michael J Wan
PURPOSE: Acute acquired comitant esotropia (AACE) is defined as a sudden-onset constant nonaccommodative esodeviation. The purpose of this study was to determine the risk of serious intracranial pathology in children presenting with AACE. DESIGN: Retrospective observational cohort study. SETTING: Tertiary care pediatric hospital. METHODS: The study included consecutive children who met the diagnostic criteria for AACE and had neuroimaging at a tertiary care pediatric hospital between 2000 and 2020...
January 25, 2024: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
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