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Chiari malformation type 1

Ibrahim Hussain, Theodore H Schwartz, Jeffrey P Greenfield
Basilar invagination is defined as abnormal upward and/or posterior displacement of the odontoid leading to ventral compression of the cervicomedullary junction. This condition leads to lower cranial neuropathies, sensorimotor deficits, and myelopathy. These symptoms can persist even after posterior decompression, which is an indication for ventral decompression. Transoral approaches to the upper cervical spine carry significant morbidity, limiting their utility. The endonasal approach to the upper cervical spine presents an alternative for patients with amenable anatomy...
March 13, 2018: Clinical Spine Surgery
Zhi Chai, Xiaoming Xue, Huijie Fan, Lin Sun, Hongyu Cai, Yanmiao Ma, Cungen Ma, Ran Zhou
This study aimed to quantitatively assess and compare the effect and safety of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression (PFD) in treating patients with Chiari malformation type I (CM1). PubMed, Embase, and Cochrane Library were searched through May 2017. Fourteen cohort studies, involving a total of 3666 patients with CM1, were included. Studies were pooled, and the relative risk (RR) and its corresponding 95% confidence interval (CI) were calculated. The decrease in syringomyelia was better for patients in the PFDD group than for patients in the PFD group (RR=1...
February 23, 2018: World Neurosurgery
Michael Faloon, Nikhil Sahai, Todd P Pierce, Conor J Dunn, Kumar Sinha, Ki Soo Hwang, Arash Emami
BACKGROUND: Several studies have sought to address the role of routine preoperative MRI in patients with adolescent idiopathic scoliosis (AIS) undergoing deformity correction. Despite similar results regarding the prevalence of neuraxial anomalies detected on MRI, published conclusions conflict and give opposing recommendations. Lack of consensus has led to important variations in use of MRI before spinal surgery for patients with AIS. QUESTIONS/PURPOSES: This systematic review and meta-analysis of studies about patients with AIS evaluated (1) the overall proportion of neuraxial abnormalities; (2) the patient factors and curve characteristics that may be associated with abnormalities; and (3) the proportion of patients who underwent neurosurgical intervention before scoliosis surgery and the kinds of neuraxial lesions that were identified...
February 21, 2018: Clinical Orthopaedics and related Research
Michael Garcia, Christopher Daugherty, Bertha Ben Khallouq, Todd Maugans
OBJECTIVE The Internet is used frequently by patients and family members to acquire information about pediatric neurosurgical conditions. The sources, nature, accuracy, and usefulness of this information have not been examined recently. The authors analyzed the results from searches of 10 common pediatric neurosurgical terms using a novel scoring test to assess the value of the educational information obtained. METHODS Google and Bing searches were performed for 10 common pediatric neurosurgical topics (concussion, craniosynostosis, hydrocephalus, pediatric brain tumor, pediatric Chiari malformation, pediatric epilepsy surgery, pediatric neurosurgery, plagiocephaly, spina bifida, and tethered spinal cord)...
February 16, 2018: Journal of Neurosurgery. Pediatrics
Y C Zhang, S F Sha, E Z Jiang, W B Li, Y Qiu, Z Z Zhu
Objective: To compare the pulmonary function between adolescent patients with Chiari malformation associated scoliosis (CMS) and adolescent idiopathic scoliosis (AIS). Methods: A retrospective analysis was performed on 52 patients with CMS, and 52 patients with AIS were selected as the control group to match the CMS patients by age, sex, and Cobb angle. Preoperative pulmonary function tests were completed by all the patients, including vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in one second (FEV(1)), maximal mid-expiratory flow (MMEF), and ratio of FEV(1) to FVC...
February 6, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Chamara Jayamanne, Lakkumar Fernando, Sachith Mettananda
BACKGROUND: Foot drop is a disabling clinical condition with multiplicity of causes, which requires detailed evaluation to identify the exact aetiology. Here, we report an extremely rare cause of foot drop in a child, which if not recognized early, could lead to multiple complications. CASE PRESENTATION: A 6-year-old girl presented with difficulty in walking and left sided foot droop for1-month duration. On examination she had reduced muscle power in dorsiflexors and plantar flexors and diminished knee and absent ankle jerk in the left side...
February 7, 2018: BMC Pediatrics
Maggie S Eppelheimer, James R Houston, Jayapalli R Bapuraj, Richard Labuda, Dorothy M Loth, Audrey M Braun, Natalie J Allen, Soroush Heidari Pahlavian, Dipankar Biswas, Aintzane Urbizu, Bryn A Martin, Cormac O Maher, Philip A Allen, Francis Loth
Purpose: Researchers have sought to better understand Chiari type I malformation (CMI) through morphometric measurements beyond tonsillar position (TP). Soft tissue and bone structures within the brain and craniocervical junction have been shown to be different for CMI patients compared to healthy controls. Yet, several morphological characteristics have not been consistently associated with CMI. CMI is also associated with different prevalent conditions (PCs) such as syringomyelia, pseudotumor, Ehlers-Danlos syndrome (EDS), scoliosis, and craniocervical instability...
2018: Frontiers in Neuroanatomy
Toshihiko Mori, Eri Nishino, Tomomi Jitsukawa, Emiko Hoshino, Satoshi Hirakawa, Yuki Kuroiwa, Shigeto Fuse, Yuko Yoto, Hiroyuki Tsutsumi
We describe the case of a short-statured 12-yr-old boy who developed a Chiari type 1 malformation associated with central sleep apnea after administration of high-dose GH therapy, which he had been receiving since the age of 10 yr and 4 mo. He responded well to GH therapy, and his height increased by 18.8 cm in 2 yr. At 12 yr and 4 mo of age, his mother reported that he had developed sleep apnea during the previous year and it had worsened over a month prior to presentation at our hospital. Otolaryngological examination did not reveal tonsillar or adenoidal hypertrophy...
2018: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
Gordan Grahovac, Tatiana Pundy, Tadanori Tomita
OBJECTIVES: Chiari I malformation has been a well-recognized clinical entity; however, its occurrence among infants and toddlers is unusual. Their clinical presentations may be different from other age groups due to their lack of effective verbal communication. The authors analyze their personal series of patients focusing on symptomatology and MRI characteristics. Treatment methods, results, and outcome are analyzed in order to identify appropriate surgical management among infants and toddlers with Chiari I malformation...
February 2, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Gavin J Andrews
Human geography's varied engagement with the brain has involved considerations of the way people know and respond to their environments, and their place-based experiences with emotions, mental illnesses and disorders, intellectual disabilities and particular neurological conditions. This paper argues however that this scholarship could be augmented by, and existing expertise be directed towards, considering physical brain abnormalities and injuries. As a case in point it considers the spatial experience of living with Type 1 Chiari Malformation...
January 27, 2018: Social Science & Medicine
Min Sun Kim, Pyoung Han Hwang, Dae-Yeol Lee
A small percentage of individuals have the neurological anomaly of central precocious puberty (CPP). Common neurologic causes of CPP include a tumor or congenital lesions. Although Arnold-Chiari malformation can be caused by congenital or acquired causes, it is unusual in patients with CPP. We present the case of a girl aged 4.5 years who complained of breast budding. Her neurological examination and growth pattern were normal. She had no endocrinological abnormality, except for true precocious puberty. We performed brain magnetic resonance imaging, which showed an Arnold-Chiari type 1 malformation...
January 2018: Korean Journal of Family Medicine
Masakazu Sano, Junichi Yoshimura, Yukihiko Fujii
Craniosynostosis associated with Chiari malformation (CM) is usually found in infants with an underdeveloped posterior fossa. We here present a case of adult craniosynostosis, CM, and symptomatic syringomyelia caused by the protrusion of the posterior rim of the foramen magnum without a tight posterior fossa. A 22-year-old woman with an abnormal head shape and forearm hypesthesia was given a diagnosis of sagittal suture synostosis with CM and syringomyelia caused by foramen magnum stenosis. She underwent foramen magnum decompression with a C1 laminectomy without cranial vault expansion or duraplasty...
January 2018: NMC Case Report Journal
Fatima Azahraa Haddad, Ibraheem Qaisi, Nagham Joudeh, Hamada Dajani, Fareed Jumah, Amjad Elmashala, Nimer Adeeb, Joshua J Chern, R Shane Tubbs
In 1891, Hans Chiari described a group of congenital hindbrain anomalies, which were eventually named after him. He classified these malformations into three types (Chiari malformations I, II, and III), and four years later added the Chiari IV malformation. However, numerous reports across the literature do not seem to fit Chiari's original descriptions of these malformations, so researchers have been encouraged to propose new classifications to encompass these variants (e.g., Chiari 0, Chiari1.5, and Chiari 3...
April 2018: Clinical Anatomy
Tatsuya Ohtonari, Nobuharu Nishihara, Shinzo Ota, Akio Tanaka
OBJECTIVE: We investigated CSF dynamics at CCJ using Time-SLIP MRI to demonstrate the significance of ventral and dorsal combined CSF dynamics in assessing CSF flow disturbance in patients with CM-I. METHODS: Fifteen examinations were performed in nine cases of CM-I (three females patients; mean age, 24.7 years; age range, 11-46 years) before or after craniocervical decompression. The longitudinal maximum movement of the caudal edge of tagged mid-sagittal CSF at CCJ was measured as a LOM, and total on the ventral and dorsal sides was defined as total LOM...
January 8, 2018: World Neurosurgery
Vijay M Ravindra, Kaine Onwuzulike, Robert S Heller, Robert Quigley, John Smith, Andrew T Dailey, Douglas L Brockmeyer
OBJECTIVE Previous reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction. METHODS The authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center...
February 2018: Journal of Neurosurgery. Pediatrics
Viralkumar M Vasani, Subhas Kanti Konar, S Satish
Type 2 respiratory failure is defined as hypercapnia associated with hypoxia. Chiari 1.5 is known as herniation of the cerebellar tonsils along with brain stem and fourth ventricle. We report a 35-year-old male who presented with acute hypercapnic respiratory failure (Type 2), without any preexisting neurological or respiratory abnormality. Analysis of blood gases in emergency revealed a pH of 7.12, pCO2 of 132 mmHg, and arterial oxygen tension of 118 mm Hg. He was intubated and ventilated. Magnetic resonance imaging brain revealed herniation of the cerebellar tonsils along with brain stem and fourth ventricle...
October 2017: Indian Journal of Critical Care Medicine
Weiwei Lin, Guman Duan, Jinjin Xie, Jiashen Shao, Zhaoqi Wang, Baohua Jiao
BACKGROUND: Posterior fossa decompression without (PFD) or with duraplasty (PFDD) for the treatment of type 1 Chiari malformation (CM-1) is controversial. We thus performed a systematic review and meta-analysis of studies to assess the effect on clinical and imaging improvement, operative time, complications, and recurrence rate between PFD and PFDD in patients with CM-1. METHODS: We systematically searched PubMed, Embase, Cochrane, Web of Knowledge, and ClinicalTrials...
February 2018: World Neurosurgery
Siri Sahib S Khalsa, Ndi Geh, Bryn A Martin, Philip A Allen, Jennifer Strahle, Francis Loth, Desale Habtzghi, Aintzane Urbizu Serrano, Daniel McQuaide, Hugh J L Garton, Karin M Muraszko, Cormac O Maher
OBJECTIVE Chiari malformation Type I (CM-I) is typically defined on imaging by a cerebellar tonsil position ≥ 5 mm below the foramen magnum. Low cerebellar tonsil position is a frequent incidental finding on brain or cervical spine imaging, even in asymptomatic individuals. Nonspecific symptoms (e.g., headache and neck pain) are common in those with low tonsil position as well as in those with normal tonsil position, leading to uncertainty regarding appropriate management for many patients with low tonsil position and nonspecific symptoms...
November 10, 2017: Journal of Neurosurgery. Pediatrics
Sumit Thakar, Laxminadh Sivaraju, Kuruthukulangara S Jacob, Aditya Atal Arun, Saritha Aryan, Dilip Mohan, Narayanam Anantha Sai Kiran, Alangar S Hegde
OBJECTIVE Although various predictors of postoperative outcome have been previously identified in patients with Chiari malformation Type I (CMI) with syringomyelia, there is no known algorithm for predicting a multifactorial outcome measure in this widely studied disorder. Using one of the largest preoperative variable arrays used so far in CMI research, the authors attempted to generate a formula for predicting postoperative outcome. METHODS Data from the clinical records of 82 symptomatic adult patients with CMI and altered hindbrain CSF flow who were managed with foramen magnum decompression, C-1 laminectomy, and duraplasty over an 8-year period were collected and analyzed...
January 2018: Journal of Neurosurgery. Spine
Ahmet Öğrenci, Orkun Koban, Murat Ekşi, Onur Yaman, Sedat Dalbayrak
Downward displacement of cerebellar tonsils more than 5 mm below the foramen magnum is named as Chiari type I malformation and named benign tonsillar ectopia if herniation is less than 3 mm. It does not just depend on congenital causes. There are also some reasons for acquired Chiari Type 1 and benign tonsillar ectopia/herniation. Trauma is one of them. Trauma may increase tonsillar ectopia or may be the cause of new-onset Chiari type 1. The relationship between the tonsil contusion and its position is unclear...
October 15, 2017: Open Access Macedonian Journal of Medical Sciences
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