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Anandini Suri, Varun K Sharma, Prajakta R Ranade, Shaji Marar, Aabha Nagral
BACKGROUND: Hepatocellular carcinoma is an uncommon complication described in patients with Budd-Chiari syndrome. CASE CHARACTERISTICS: A 12-year-old boy with Budd-Chiari syndrome, who was earlier treated with Transjugular intrahepatic porto-systemic shunt (TIPS), presented with acute onset hemoperitoneum and hypotension. OUTCOME: It was diagnosed to be a case of ruptured hepatocellular carcinoma. MESSAGE: Successful TIPS may not prevent the development of hepatocellular carcinoma, and children with Budd Chiari syndrome should be monitored for the same...
September 8, 2016: Indian Pediatrics
Francesco Giganti, Alessandro Ambrosi, Maria Chiara Petrone, Carla Canevari, Damiano Chiari, Annalaura Salerno, Paolo Giorgio Arcidiacono, Roberto Nicoletti, Luca Albarello, Elena Mazza, Francesca Gallivanone, Luigi Gianolli, Elena Orsenigo, Antonio Esposito, Carlo Staudacher, Alessandro Del Maschio, Francesco De Cobelli
OBJECTIVES: To compare the diagnostic performance of Magnetic Resonance (MR) and diffusion-weighted imaging (DWI), Multidetector Computed Tomography (MDCT), Endoscopic Ultrasonography (EUS) and (18)F-FDG (Fluorodeoxyglucose) Positron Emission Tomography - Computed Tomography (PET-CT) in the preoperative loco-regional staging of oesophageal cancer. METHODS: Eighteen patients with oesophageal or Siewert I tumour (9 directly treated with surgery and 9 addressed to chemo/radiotherapy before) underwent 1...
October 21, 2016: British Journal of Radiology
Annah Lane, Ling Lee, Donna Traves, Andreas Lee
INTRODUCTION: The antenatal diagnosis of open spina bifida (OSB), a neural tube defect, is predominantly made at the second trimester morphology scan by ultrasound detection of structural abnormalities resulting from the associated Chiari II malformation. Evidence has emerged suggesting that these structural abnormalities can be detected earlier, by examination of the posterior fossa as part of the first trimester nuchal translucency scan. In particular, absence of the intra-cranial translucency (IT) of the fourth ventricle has shown promise as a diagnostic marker of OSB, although the sensitivity and specificity of this finding varies widely in the literature...
October 21, 2016: Journal of Medical Imaging and Radiation Oncology
Thomas Beez, Jennifer Brown
BACKGROUND: Atlanto-occipital dislocation (AOD) is a rare but severe sequela of high energy trauma. Children are at increased risk, due to their immature spine and biomechanical characteristics. LITERATURE REVIEW: The prevailing mechanism of injury is motor vehicle collision as passenger. AOD commonly presents with cardiorespiratory arrest, spinal cord injury or cranial nerve deficits. Concomitant injuries, such as traumatic brain injury, are frequently encountered...
October 18, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Sivashanmugam Dhandapani, Madhivanan Karthigeyan, Sushanta K Sahoo, Vivek Gupta
BACKGROUND: Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure. CASE DESCRIPTION: We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma...
October 18, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Silvia De-la-Iglesia, Hugo Luzardo, Angelina Lemes, Melissa Torres, Maria Teresa Gómez-Casares, Naylen Cruz, Teresa Molero
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with portal cavernoma, portal hypertension and hypersplenism. In September 2010, the patient suffered gastrointestinal bleeding, hematuria, and elevated plasma lactate dehydrogenase; he started eculizumab therapy with a good response...
September 28, 2016: Hematology Reports
Omar N Pathmanaban, Kerry A Burke, Paul Leach, John Thorne, Ian D Kamaly-Asl
BACKGROUND: Positional plagiocephaly is the most common cause of cranial asymmetry. The underlying cause of Chiari-1 malformation has many possible theories, and anecdotally some pediatric neurosurgeons have had experience of severe cases of positional brachycephaly with Chiari-1. However, to date there have been no published cases linking non-synostotic plagiocephaly with Chiari-1 malformation. CASE DESCRIPTION: An 18-month-old boy presented with a head injury...
October 15, 2016: World Neurosurgery
Max O Krucoff, Steven Cook, Owoicho Adogwa, Jessica Moreno, Siyun Yang, Jichun Xie, Alexander O Firempong, Nandan Lad, Carlos A Bagley
OBJECT: To examine the role of race, gender, and socioeconomics on presentations and outcomes of adult Chiari 1 malformations. METHODS: The charts of 638 adult patients with Chiari 1 malformations were reviewed, and 287 patients were included. Race, gender, insurance status, symptoms, depth of cerebellar tonsillar herniation, and presence of syringomyelia were examined as covariates in multivariate logistic regression models to identify independent predictors of presentation and outcome...
October 14, 2016: World Neurosurgery
Tomomi Kogiso, Etsuko Hashimoto, Taito Ito, Toshifumi Hara, Yuichi Ikarashi, Kazuhisa Kodama, Makiko Taniai, Nobuyuki Torii, Kentaro Yoshinaga, Satoru Morita, Yutaka Takahashi, Junji Tanaka, Shuji Sakai, Masakazu Yamamoto, Katsutoshi Tokushige
A 56-year-old man was diagnosed with aplastic anemia and paroxysmal nocturnal hemoglobinuria at 43 years of age and treatment with cyclosporin A was started. Liver cirrhosis, ascites, and thrombus in the hepatic veins were found at 56 years of age and Budd-Chiari syndrome (BCS) was diagnosed according to angiography findings. He was treated with diuretics and paracentesis was performed several times, but with limited efficacy. A Denver(®) peritoneovenous shunt (PVS) was inserted into the right jugular vein; his ascites and renal function improved immediately and his general condition has remained good for 12 months since starting the above treatment regimen...
2016: Internal Medicine
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
Chun Yang, Ceng Li, Mengsu Zeng, Xin Lu, Jingjing Li, Jiali Wang, Muhammad Umair Sami, Kai Xu
Non-CE MRA techniques (true steady-state free-precession, SSFP) have been used effectively for the selective visualization of the portal venous system and inferior vena cava. Budd-Chiari Syndrome (BCS) encompasses a number of conditions that cause the obstruction of the hepatic outflow tract from the small hepatic veins to the junction of the inferior vena cava (IVC) and right atrium. The purpose of this study was to diagnose BCS with IVC obstruction using respiratory triggered three-dimensional (3D) true SSFP with T-SLIP and compare to digital subtraction angiography (DSA)...
October 11, 2016: Magnetic Resonance Imaging
T Struffert
Many important structures are located in the confined space within the posterior cranial fossa. This article describes the main aspects of the anatomy. As a uniform classification of malformations of the posterior cranial fossa does not exist the main syndromes, such as Chiari malformations, zerebellar hypoplasia and dysplasia are discussed separately.
October 13, 2016: Der Radiologe
X Qi, G Han, X Guo, V De Stefano, K Xu, Z Lu, H Xu, A Mancuso, W Zhang, X Han, D C Valla, D Fan
BACKGROUND: China may have the largest number of Budd-Chiari syndrome (BCS) cases in the world (at least 1914 original papers were published, and at least 20 191 BCS patients were reported). Considering the discrepancy in the clinical profiles and preferred treatment selection of primary BCS between the West and China, understanding its aetiology in these two different regions is very important. AIM: To review the data from large cohort studies and meta-analyses to illustrate the epidemiology of risk factors for BCS in the West and China...
October 13, 2016: Alimentary Pharmacology & Therapeutics
Alessandro Gori, Laura Sola, Paola Gagni, Giulia Bruni, Marta Liprino, Claudio Peri, Giorgio Colombo, Marina Cretich, Marcella Chiari
The generation of robust analytical data using microarray platforms strictly relies on optimal ligand-target interaction at the sensor surface which, in turn, is inherently bound to the correct immobilization scheme of the interrogated bioprobes. In the present work, we performed a rigorous comparative analysis of the impact of peptide ligands immobilization strategy in the screening of Burkholderia cepacia complex (BCC) infections in patients affected by cystic fibrosis (CF). We generated arrays of previously validated Burkholderia-derived peptide probes, that were selectively oriented on polymeric coatings by means of different click-type reactions including thiol-maleimide, copper-catalyzed-azide-alkyne-cycloaddition (CuAAC) and strain-promoted-azide-alkyne-cycloaddition (SPAAC)...
October 12, 2016: Bioconjugate Chemistry
Alexander Kolb, Madeleine Willegger, Reinhard Schuh, Alexandra Kaider, Catharina Chiari, Reinhard Windhager
PURPOSE: Deformation of the talus in idiopathic congenital clubfeet is a known problem after treatment. However evidence on types of talus deformation and clinical relevance is rare. The aims of this study were first to define different types of talus deformation, and second, to evaluate the impact of these types on long-term results. METHODS: At a minimum follow-up of ten years 40 idiopathic clubfeet treated by a modified dorsomedial release were analyzed. Based on morphological appearance and the widened range of radius to length ratios (R/L-ratio) in treated clubfeet deformed tali were divided into two groups: tali with decreased R/L-ratios were classified as small-dome talus deformation (SD), tali with increased R/L-ratios were classified as flat-top talus deformation (FT)...
October 11, 2016: International Orthopaedics
Sepideh Sefidbakht, Sakineh Dehghani, Maryam Safari, Homeira Vafaei, Maryam Kasraeian
BACKGROUND: Magnetic resonance imaging (MRI) is gradually becoming more common for thorough visualization of the fetus than ultrasound (US), especially for neurological anomalies, which are the most common indications for fetal MRI and are a matter of concern for both families and society. OBJECTIVES: We investigated fetal MRIs carried out in our center for frequency of central nervous system anomalies. This is the first such report in southern Iran. MATERIALS AND METHODS: One hundred and seven (107) pregnant women with suspicious fetal anomalies in prenatal ultrasound entered a cross-sectional retrospective study from 2011 to 2013...
August 2016: Iranian Journal of Pediatrics
Daniele Starnoni, Roy Thomas Daniel, Mercy George, Mahmoud Messerer
BACKGROUND: Spontaneous meningoencephaloceles of the lateral sphenoid sinus are rare entities and their peculiar location represent a surgical challenge due to the importance of a wide exposure and skull base reconstruction. They are thought to arise from congenital base defect of the lateral sphenoid or in some cases have been postulated to represent a rare manifestation of altered CSF dynamics. We report the first case in literature of a Chiari malformation type I and a lateral sphenoid encephaloceles revising the theoretical etiology and surgical technique of endoscopic repair...
October 8, 2016: World Neurosurgery
Javed Malik
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Karen-Helene Støverud, Hans Petter Langtangen, Geir Andre Ringstad, Per Kristian Eide, Kent-Andre Mardal
PURPOSE: Previous computational fluid dynamics (CFD) studies have demonstrated that the Chiari malformation is associated with abnormal cerebrospinal fluid (CSF) flow in the cervical part of the subarachnoid space (SAS), but the flow in the SAS of the posterior cranial fossa has received little attention. This study extends previous modelling efforts by including the cerebellomedullary cistern, pontine cistern, and 4th ventricle in addition to the cervical subarachnoid space. METHODS: The study included one healthy control, Con1, and two patients with Chiari I malformation, P1 and P2...
2016: PloS One
Edith V Sullivan, Barton Lane, Dongjin Kwon, M J Meloy, Susan F Tapert, Sandra A Brown, Ian M Colrain, Fiona C Baker, Michael D De Bellis, Duncan B Clark, Bonnie J Nagel, Kilian M Pohl, Adolf Pfefferbaum
Structural MRI of volunteers deemed "normal" following clinical interview provides a window into normal brain developmental morphology but also reveals unexpected dysmorphology, commonly known as "incidental findings." Although unanticipated, these anatomical findings raise questions regarding possible treatment that could even ultimately require neurosurgical intervention, which itself carries significant risk but may not be indicated if the anomaly is nonprogressive or of no functional consequence. Neuroradiological readings of 833 structural MRI from the National Consortium on Alcohol and NeuroDevelopment in Adolescence (NCANDA) cohort found an 11...
October 8, 2016: Brain Imaging and Behavior
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