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Chiari malformation type 1

Radek Frič, Erika Kristina Lindstrøm, Geir Andre Ringstad, Kent-André Mardal, Per Kristian Eide
BACKGROUND: In symptomatic Chiari malformation type 1 (CMI), impaired intracranial compliance (ICC) is associated with an increased cranio-spinal pulsatile pressure gradient. Phase-contrast magnetic resonance imaging (MRI) represents a non-invasive modality for the assessment of the pulse pressure gradient at the cranio-cervical junction (CCJ). We wished to explore how the MRI-derived pulse pressure gradient (MRI-dP) compares with invasively measured pulsatile intracranial pressure (ICP) in CMI, and with healthy controls...
October 14, 2016: Acta Neurochirurgica
Sandi K Lam, Rory R Mayer, Thomas G Luerssen, I Wen Pan
OBJECTIVES: To develop a cost model for hospitalization costs of surgery among children with Chiari malformation type 1 (CM-1) and to examine risk factors for increased costs. STUDY DESIGN: Data were extracted from the US National Healthcare Cost and Utilization Project 2009 Kids' Inpatient Database. The study cohort was comprised of patients aged 0-20 years who underwent CM-1 surgery. Patient charges were converted to costs by cost-to-charge ratios. Simple and multivariable generalized linear models were used to construct cost models and to determine factors associated with increased hospital costs of CM-1 surgery...
September 21, 2016: Journal of Pediatrics
Woon Tak Yuh, Chi Heon Kim, Chun Kee Chung, Hyun-Jib Kim, Tae-Ahn Jahng, Sung Bae Park
OBJECTIVE: The pathophysiology of idiopathic Chiari malformation (CM) type 1 is disturbance of free cerebrospinal fluid (CSF) flow and restoration of normal CSF flow is the mainstay of treatment. Additional migration of the medulla oblongata in pediatric patients is referred to as CM type 1.5, but its significance in adult patients is unknown. This study is to compare surgical outcomes of adult idiopathic CM type 1.5 with that of type 1. METHODS: Thirty-eight consecutive adult patients (M : F=11 : 27; median, 33...
September 2016: Journal of Korean Neurosurgical Society
Ori Barzilai, Jonathan Roth, Akiva Korn, Shlomi Constantini
No abstract text is available yet for this article.
September 9, 2016: Journal of Neurosurgery
Gustavo Giraldo, Ana M Gómez, Lina Mora, Fernando Suarez-Obando, Olga Moreno
INTRODUCTION: Mosaic trisomy 8 or "Warkany's Syndrome" is a chromosomopathy with an estimated prevalance of 1:25,000 to 1:50,000, whose clinical presentation has a wide phenotypic variability. CASE DESCRIPTION: Patient aged 14 years old with antecedents of global retardation of development, moderate cognitive deficit and hypothyroidism of possible congenital origin. CLINICAL FINDINGS: Physical examination revealed palpebral ptosis, small corneas and corectopia, hypoplasia of the upper maxilla and prognathism, dental crowding, high-arched palate, anomalies of the extremities such as digitalization of the thumbs, clinodactyly and bilateral shortening of the fifth finger, shortening of the right femur, columnar deviation and linear brown blotches that followed Blaschko's lines...
2016: Colombia Médica: CM
Jayesh Sardhara, Sindgikar Pavaman, Kuntal Das, Arun Srivastava, Anant Mehrotra, Sanjay Behari
BACKGROUND: Congenital spondylolytic spondylolisthesis of C2 vertebra resulting from deficient posterior element of the axis is very rarely described in the literature. CASE DESCRIPTION: We describe a unique case of agenesis of posterior elements of C2 with cranio-vertebral junction anomalies consist of osseous, vascular and soft tissue anomalies. A young 26 year old young man presented with symptoms of upper cervical myelopathy of 12 months duration. CT scan of the cervical spine including the craniovertebral junction revealed spondylolisthesis of C2 over C3, atlanto axial dislocation, occipitalisation of atlas, hypoplasia of the odontoid and cleft posterior C1 arch...
August 12, 2016: World Neurosurgery
Marianne Pozetti, Telmo Augusto Barba Belsuzarri, Natalia C B Belsuzarri, Naira B Seixas, João F M Araujo
BACKGROUND: The association between neurofibromatosis type 1 (NF-I) and Chiari I malformation (CMI) is rare, and not many studies are reported in the literature. Performing magnetic resonance imaging (MRI) in patients with NF-1 is essential because several cases of Chiari type I are completely asymptomatic. We emphasize the need for inclusion of Chiari I as diagnosis in association with NF-1. CASE DESCRIPTION: The patient was a 51-year-old black man who presented with complaints of pain and decreased motion and sensibility of his hands, wrists, and forearms, along with progressive dysarthria...
2016: Surgical Neurology International
Ning Li, Wei-Guo Zhao, Chun-Hua Pu, Wen-Lei Yang
Hemifacial spasm (HFS) associated with type 1 Chiari malformation is particularly uncommon and is limited to isolated case report. The aims of this study were to report the clinical correlates of patients who had simultaneously HFS and type 1 Chiari malformation and to present the outcome of these patients treated with microvascular decompression (MVD) surgery. We retrospectively evaluated 13 patients who had simultaneously HFS and type 1 Chiari malformation among 675 HFS patients. Clinical features and radiological findings were collected from each patient and analyzed...
July 15, 2016: Neurosurgical Review
Keely Smith, Ana M Gomez-Rubio, Tomika S Harris, Lauren E Brooks, Ricardo A Mosquera
We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship...
2016: Case Reports in Pediatrics
Aditya Vedantam, Rory R Mayer, Kristen A Staggers, Dominic A Harris, I-Wen Pan, Sandi K Lam
PURPOSE: The multicenter National Surgical Quality Improvement Program-Pediatric (NSQIP-P) database maintained by the American College of Surgeons was used to describe 30-day outcomes following Chiari type 1 decompression in children and to identify risk factors for readmission, reoperation, and perioperative complications. METHODS: We identified patients aged 0-18 years who underwent posterior cranial fossa decompression for Chiari type 1 malformation in 2012, 2013, and 2014 in the NSQIP-Pediatric database...
July 8, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Antonin Leroy, Reda Kabbaj, Arnaud Dubory, Manon Bachy, Anne-Isabelle Vermersch, Raphaël Vialle
INTRODUCTION: Pedicle screw fixation allows purchase of all three spinal columns without encroaching into the spinal canal improving fracture fixation, as well as deformity correction. Fortunately, neurologic injury associated with pedicle screw malposition is rare. CASE PRESENTATION: A 19-year-old boy was surgically treated for severe right thoracic scoliosis associated with a Chiari Type 1 malformation and a C6 to T7 syringomyelia. Six months after the initial surgery, the patient was referred to our institution after three weeks of gait disturbances and repeated falls...
2016: SpringerPlus
Rajiv J Iyengar, Petra M Klinge, Wendy S Chen, Jerrold L Boxerman, Stephen R Sullivan, Helena O Taylor
BACKGROUND: The natural history of unrepaired craniosynostosis is not well defined. Delayed surgical intervention carries greater risk of postoperative complications and its functional benefits for older patients are poorly characterized. The authors reviewed patients in whom children presented beyond 1 year of age to better understand the natural history of craniosynostosis, and the risk-benefit relationship for delayed reconstruction. METHODS: After institutional IRB approval the authors conducted a retrospective review of patients who presented after 1 year of age with craniosynostosis...
July 2016: Journal of Craniofacial Surgery
Eren Fatma Akçıl, Özlem Korkmaz Dilmen, Yusuf Tunalı
Costello syndrome is a rare genetic disorder characterised by growth and mental retardation, macrocephaly, short neck and macroglossia. Cardiac involvement can also occur in Costello syndrome and is presented in the form of hypertrophic cardiomyopathy, tachyarrythmias and valvular dysfunction. Nervous system involvement including ventriculomegaly, hydrocephaly and Chiari type 1 malformation are also common. Predisposition of papillomata and malignant tumours are high. General anaesthesia practice in patients with Costello syndrome may be complicated by difficult airway because of macrocephaly, short neck, macroglossia and oral or laryngeal papillomas...
December 2015: Turkish Journal of Anaesthesiology and Reanimation
Andrea Poretti, Ramy Ashmawy, Tomas Garzon-Muvdi, George I Jallo, Thierry A G M Huisman, Charles Raybaud
Our understanding of cerebellar tonsillar herniation evolved over time and nowadays various pathomechanisms have been proposed. Causes of tonsillar herniation share a discrepancy between content (fore- and hindbrain) and container (supratentorial cranial vault, posterior fossa), may be associated with abnormalities of the craniocervical junction, and may have a developmental or acquired nature. In tonsillar herniation, the hindbrain is not malformed but deformed. Accordingly, "Chiari type 1 deformity," not "Chiari type 1 malformation" is the correct term to characterize primary tonsillar herniation...
October 2016: Neuropediatrics
Maud Pm Tijssen, Andrea Poretti, Thierry Agm Huisman
We describe the neuroimaging findings of an 11-year-old boy who presented with mild occipital headache and precocious puberty. This child was found to have a combination of various midline anomalies including a Chiari type 1 malformation, corpus callosum agenesis and patent craniopharyngeal canal with adjacent intracranial dermoid cyst.
October 2016: Neuroradiology Journal
Gaston O Camino Willhuber, Santiago T Bosio, Miguel H Puigdevall, Carolina Halliburton, Carlos A Sola, Ruben A Maenza
To present and describe an unusual case of spinal instability after craniocervical spinal decompression for a type-1 Chiari malformation. Type-1 Chiari malformation is a craniocervical disorder characterized by tonsillar displacement greater than 5 mm into the vertebral canal; posterior fossa decompression is the most common surgical treatment for this condition. Postoperative complications have been described: cerebrospinal fluid leak, pseudomeningocele, aseptic meningitis, wound infection, and neurological deficit...
June 2, 2016: Journal of Pediatric Orthopedics. Part B
Hao Xu, LinYang Chu, Rui He, Chang Ge, Ting Lei
The treatment of Chiari malformation type 1 (CM-I) with posterior fossa decompression without (PFD) or with duraplasty (PFDD) is controversial. Our aim is to compare the clinical outcome between the two methods for the treatment of CM-I. In this paper, the authors report a systematic review and meta-analysis of operation time, clinical improvement, and complications of PFD compared with PFDD for the treatment of CM-I. Randomized or non-randomized controlled trials of PFD and PFDD were considered for inclusion...
June 1, 2016: Neurosurgical Review
Maureen Lacy, Sarah E Ellefson, Samantha DeDios-Stern, David M Frim
BACKGROUND: Children with Chiari malformation type 1 (CM1) have increasingly presented to neurosurgery clinics. Limited research relating to the cognitive dysfunction experienced by this population has been completed. In adults, inhibition problems and executive dysfunction have been documented. METHODS: Seventy-seven parental reports of children with CM1 were included in the study. Parents completed questions on a scale rating daily executive functioning as well as reporting on common neurological symptoms...
2016: Pediatric Neurosurgery
Ahmed-Ramadan Sadek, Anan Shtaya, Ardalan Zolnourian, Ali Nader-Sepahi
No abstract text is available yet for this article.
June 2016: British Journal of Neurosurgery
Kamran Urgun, Baran Yılmaz, Zafer Orkun Toktaş, Akın Akakın, Deniz Konya, Mustafa Kemal Demir, Türker Kılıç
Aneurysmal bone cysts (ABCs) are defined as benign cystic lesions of bone composed of blood-filled spaces. ABCs may be a secondary pathology superimposed on fibrous dysplasia (FD). Concomitant FD and ABC in relation with McCune-Albright syndrome is an extremely rare condition. Here, we report concomitant, double ABCs in bilateral occipital regions and FD from the skull base to the C2 vertebra with Chiari type 1 malformation. A 14-year-old female with a diagnosis of McCune-Albright syndrome presented with swellings at the back of her head...
2016: Pediatric Neurosurgery
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