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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
#2
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
#3
Alessandro Ciarloni, Gianmaria Salvio
We present a case of acromegaly associated with Arnold-Chiari 1 malformation and a literature review regarding this association, mainly focusing on the importance of a clear distinction between Chiari malformation and herniation of cerebellar tonsils (CTH). Indeed, in many clinical cases, this distinction has not been properly made and a better description of the radiological findings could be important for the clinical management of these patients. In fact, Arnold-Chiari 1 malformation, as a congenital disease, is not caused by acquired growth hormone (GH) excess, but the latter could worsen pre-existing CTH or even induce it ex novo ...
2024: Case Reports in Endocrinology
#4
Amel F H Alzain
Chiari malformations are structural defects in the cerebellum that are characterized by the downward displacement of one or both cerebellar tonsils through the foramen magnum. A case report of a female neonate with clinical features of Chiari II malformation is presented. The diagnosis of this condition was made through a combination of clinical and radiological features. In this case, the diagnosis was confirmed by a noncontrast computed tomography (CT) scan of the brain and multiplanar brain magnetic resonance imaging (MRI)...
May 2024: Radiology Case Reports
#5
Felipe Chavelas-Ochoa, Ma de la Luz Bermúdez-Rojas, Virginia Medina-Jiménez, Antonio Helue-Mena, Savino Gil-Pugliese, Ivan Gutiérrez-Gómez, Miguel Martínez-Rodríguez, Carmen Julia Gaona-Tapia, Rosa Villalobos-Gómez, Karla Aguilar-Vidales, Rogelio Cruz-Martínez
INTRODUCTION: Open spina bifida (OSB) is the most common congenital anomaly of the central nervous system. It is associated with severe neurodevelopmental delay, motor impairment, hydrocephalus, and bowel and bladder dysfunction. In selected cases, intrauterine spina bifida repair has been shown to improve neonatal outcomes. Rarely, the spine can have a double defect compromising two different segments and there is a lack of evidence on the feasibility and benefits of intrauterine repair in these cases...
February 9, 2024: Pediatric Neurosurgery
#6
JOURNAL ARTICLE
Norman Ramirez, David Deliz-Jimenez, Norberto Torres-Lugo, Gerardo Olivella, Patrick Cahill, Purnendu Gupta, Sumeet Garg, Joshua Pahys, Jean-Marc Mac-Thiong
BACKGROUND: Back pain, as a clinical marker in scoliosis, has been associated with underlying pathology for many years, warranting further magnetic resonance imaging (MRI). Failures of segmentation, mixed defects, female gender, rib anomalies, congenital thoracic anomalies, and neurocutaneous markers are known risk factors for abnormal MRI pathology findings in patients with congenital early-onset scoliosis (Congenital-EOS). Yet, back pain has not been evaluated as a risk factor for underlying MRI pathology in patients with Congenital-EOS...
January 25, 2024: Journal of Pediatric Orthopedics
#7
REVIEW
Hyojeong Lee, Fatima Janjua, Ahmed Ragab, Jay Moran, Andrew Haims, Daniel Rubio, Dominick Tuason, Jack Porrino
Adolescent idiopathic scoliosis is a commonly encountered condition often diagnosed on screening examination. Underlying, asymptomatic neural axis abnormalities may be present at the time of diagnosis. At certain institutions, total spine MRI is obtained preoperatively to identify these abnormalities. We provide a framework for the radiologist to follow while interpreting these studies. In part 1, we discuss Arnold Chiari malformations, syringomyelia, and the tethered cord. In part 2, we focus on spinal cord tumors, dysraphisms, to include diastematomyelia, and vertebral anomalies...
January 20, 2024: Current Problems in Diagnostic Radiology
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REVIEW
Hyojeong Lee, Fatima Janjua, Ahmed Ragab, Jay Moran, Andrew Haims, Daniel Rubio, Dominick Tuason, Jack Porrino
Adolescent idiopathic scoliosis is a commonly encountered condition often diagnosed on screening examination. Underlying, asymptomatic neural axis abnormalities may be present at the time of diagnosis. At certain institutions, total spine MRI is obtained preoperatively to identify these abnormalities. We provide a framework for the radiologist to follow while interpreting these studies. In part 1, we discuss Arnold Chiari malformations, syringomyelia, and the tethered cord. In part 2, we focus on spinal cord tumors, dysraphisms, to include diastematomyelia, and vertebral anomalies...
January 18, 2024: Current Problems in Diagnostic Radiology
#9
JOURNAL ARTICLE
Arnold H Menezes
INTRODUCTION: Since the first description of os odontoideum in 1886, its origin has been debated. Numerous case series and reports show both a possible congenital origin and origin from the secondary to craniovertebral junction (CVJ) trauma. We conducted a detailed analysis of 260 surgically treated cases to document the initial symptoms, age groups, radiographic findings, and associated abnormalities, aiming to enhance the confirmation of the etiology. A literature search (1970-2022) was performed to correlate our findings...
2023: Frontiers in Surgery
#10
JOURNAL ARTICLE
Ivonne Bedei, Eyal Krispin, Magdalena Sanz Cortes, Hennie Lombaard, Roni Zemet, William E Whitehead, Michael A Belfort, Thierry A G M Huisman
OBJECTIVE: To evaluate the prenatal diagnosis of closed dysraphism (CD) and its correlation with postnatal findings and neonatal adverse outcomes. METHODS: A retrospective cohort study including pregnancies diagsnosed with fetal CD by prenatal ultrasound (US) and magnetic resonance imaging (MRI) at a single tertiary center between September 2011 and July 2021. RESULTS: CD was diagnosed prenatally and confirmed postnatally in 12 fetuses. The mean gestational age of prenatal imaging was 24...
November 27, 2023: Prenatal Diagnosis
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JOURNAL ARTICLE
Aliyar Zahedi Vafa, Stéphane Hans, Mohamad Khalife, Jérôme R Lechien
Background: Type 1 Arnold Chiari is a neurological malformation that may be associated with vocal cord paralysis in Children. In most cases, the vocal cord paralysis is related to protrusion of medulla and cerebellum in the foramen magnum, which led to compression on the vagus nerve. Case report: A 12-year-old child underwent suboccipital decompression and C1 laminectomy for a symptomatic type 1 Arnold Chiari malformation. After the surgery, patient reported severe dyspnea, aphonia, and dysphagia. The videolaryngostroboscopy and neurological examinations reported a postoperative bilateral vocal cord abduction paralysis due to bilateral IX and X cranial nerve paralyzes and a bilateral paralysis of the tongue...
March 8, 2023: Ear, Nose, & Throat Journal
#12
JOURNAL ARTICLE
Luke Bauerle, Brandon Rogowski, Aakash Shingala, Habib Emil Rafka, Timothy Webb, Brian F Saway, Edward F Kilb, Julio A Chalela, Nathan C Rowland
BACKGROUND: Syringomyelia is defined as dilation of the spinal cord's central canal and is often precipitated by skull base herniation disorders. Although respiratory failure (RF) can be associated with skull base abnormalities due to brainstem compression, most cases occur in pediatric patients and quickly resolve. The authors report the case of an adult patient with global spinal syringomyelia and Chiari malformation who developed refractory RF after routine administration of diazepam...
November 20, 2023: J Neurosurg Case Lessons
#13
JOURNAL ARTICLE
Mshari H Althomali, Omar I Aljohani, Abdulrahman J Sabbagh
BACKGROUND: Chiari malformation type III (CM III), a rare hindbrain anomaly, often presents with various concurrent anomalies. This paper reports a unique case of CM III associated with Klippel-Feil syndrome (KFS), a condition previously unreported in Saudi Arabia and documented in only one other case globally in Turkey. This study aims to share insights into the unusual association between CM III and KFS, considering their close embryological development and involvement in the craniocervical junction...
October 28, 2023: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
#14
REVIEW
Raquel Rodríguez-Blanque, Cristina Almazán-Soto, Beatriz Piqueras-Sola, Juan Carlos Sánchez-García, Andrés Reinoso-Cobo, María José Menor-Rodríguez, Jonathan Cortés-Martín
Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high...
October 23, 2023: Journal of Clinical Medicine
#15
JOURNAL ARTICLE
Daniel Staribacher, Guenther C Feigl, Gavin Britz, Dzmitry Kuzmin
BACKGROUND: Surgery for symptomatic Arnold-Chiari malformation type I involves posterior fossa decompression. There are various approaches, including endoscope-assisted ones. New possibilities and fields of application of fully endoscopic techniques are currently being developed since new and advanced endoscopic equipment and instrumentation are available. OBSERVATIONS: The authors describe the case of a fully endoscopic microsurgical procedure in a 30-year-old female patient with progressive vertigo who was diagnosed with Chiari malformation type I...
October 23, 2023: J Neurosurg Case Lessons
#16
Pooja R Tiwari, Ruchika J Zade, Snehal S Samal
Syringomyelia is a center-medullary syndrome characterized by the presence of fluid-filled spaces known as syrinx within the spinal canal. Arnold Chiari Malformation (CM-I), a rhombencephalon anomaly formerly identified as hindbrain hernia, is usually associated with it. This disorder causes the brain (cerebellum) to bulge through the opening in the skull known as the foramen magnum. Some asymptomatic patients may develop symptoms quickly if they jolt their heads and cough for a lengthy period of time...
September 2023: Curēus
#17
REVIEW
Aditi Mishra, Shoyeb Hirani, Sajid Hirani, Mohammed Yusuf D Shaikh, Shubham Khanholkar, Roshan Prasad, Mayur Wanjari
Arnold-Chiari malformations (ACMs) present unique challenges in pregnancy and labor, requiring a comprehensive understanding and multidisciplinary approach to care. This review article provides an overview of ACMs, including their definition, classification, and prevalence. The challenges in diagnosing ACMs during pregnancy, the available imaging modalities, and screening recommendations are discussed. The impact of ACMs on maternal health, fetal development, and the management strategies employed during pregnancy and labor are explored...
August 2023: Curēus
#18
JOURNAL ARTICLE
T B Shashidhar, Dilpreet Bajwa, Shubhi Tyagi, Indresh Chandra
Navigating Surgical Complexities associated with a case of Adenotonsillectomy in Arnold Chiari Malformation type 2. Arnold-Chiari or Chiari malformations (ACM) describe a group of deformities of the posterior fossa and hindbrain, which includes the cerebellum, pons, and medulla oblongata. Sleep-disordered breathing is a known but poorly evaluated comorbidity in patients with ACM. Obstructive sleep apnoea (OSA) in children is mainly caused by tonsillar and adenoid hypertrophy, and surgical resection of the palatine tonsils and adenoids is indicated depending on OSA severity...
September 2023: Indian Journal of Otolaryngology and Head and Neck Surgery
#19
Maryam Shahab, Tamanna Nazir, Sumaria Nazir
Arnold-Chiari malformation (ACM) is a rare neurological developmental disorder that presents at birth. No such cases have been reported in support of microbial infections causing Chiari malformation, yet there is evidence of how microorganisms can lead to brain abscess, brain empyema, and meningoencephalitis. We present a 23-year-old young woman with progressive back and leg pain after a streptococcal throat infection, followed by a single episode of syncope. Radiographs of the spine revealed a mild reversal of cervical spine curvature and minimal levocurvature of the lumbar spine...
July 2023: Curēus
#20
JOURNAL ARTICLE
Alamelu Alagappan, Amit Kumar Satpathy, Biswajit Sahoo, Manoj Kumar Nayak
Osteopetrosis is a rare genetic disorder resulting in increased bone density and decreased bone remodelling. Bone expansion results in the crowding of neural foramina causing cranial nerve compression. Here, we describe a female infant in her mid infancy presented with no eye contact since birth, and abdominal distension for 2 months. On CT evaluation, sclerotic bones with bilateral optic canal narrowing were present. A crowded posterior fossa with Arnold Chiari type I malformation was seen on MRI evaluation, suggesting a rare association of osteopetrosis with Arnold Chiari's malformation...
August 16, 2023: BMJ Case Reports
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