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Posterior fossa syndrome

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https://www.readbyqxmd.com/read/29037935/the-pathogenesis-of-delayed-epidural-hematoma-after-posterior-fossa-surgery
#1
Seunghoon Lee, Sang-Ku Park, Byung-Euk Joo, Jeong-A Lee, Doo-Sik Kong, Kwan Park
The purpose of this study was to analyze the pathogenesis of delayed epidural hematoma (EDH) after posterior fossa surgery. Non-traumatic, non-arterial origin delayed EDH after posterior fossa surgery is extremely rare. Moreover, the pathogenesis of its supratentorial extension is obscure. Between April 1997 and June 2016, over 3300 patients underwent microvascular decompression (MVD) for neurovascular compression syndrome. The medical chart of four patients with delayed EDH were retrospectively reviewed. The median time from MVD to re-CT scan was 58 h (range, 33-100)...
October 13, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29037562/dandy-walker-syndrome-with-duplex-kidney-abnormalities-in-trisomy-18-a-rare-case-report
#2
Tun-Jun Wang, Yi-Ying Li, Wan-Ju Wu, Chi-Kang Lin, Chun-Kai Wang, Chen-Yu Wang, Kwei-Shuai Hwang, Her-Young Su
OBJECTIVE: Trisomy 18 is one of the major numerical chromosomal disorders. The incidence of trisomy 18 is approximately one in 6000 live births. Dandy-Walker malformation (DWM) is the most common congenital malformation of the cerebellum, with an incidence of about one in 5000 live births. The incidence of trisomy 18 associated with DWM is rare and long-term survival rate is very low. CASE REPORT: A case involving a 39-year-old pregnant female with a case of trisomy 18 associated with DWM...
October 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/29030926/sternal-malformations-and-anesthetic-management
#3
REVIEW
John H Nichols, Viviane G Nasr
Shamberger and Welch classify sternal malformations into four types: thoracic ectopia cordis, cervical ectopia cordis, thoraco-abdominal ectopia cordis, and cleft sternum. Cleft sternum is the most common subset, with a reported incidence of 1 in 50,000 to 100,000 live births, representing 0.15% of all anterior chest wall malformations. Acostello et al further classify cleft sternum into complete or partial (superior, medium, inferior) with a simple superior partial cleft sternum being by far the most common with an orthotopic heart, intact pericardium, and normal skin coverage...
November 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29027018/accelerated-growth-of-hemangioblastoma-in-pregnancy-the-role-of-proangiogenic-factors-and-upregulation-of-hypoxia-inducible-factor-hif-in-a-non-oxygen-dependent-pathway
#4
REVIEW
Yosef Laviv, Joshua L Wang, Matthew P Anderson, Ekkehard M Kasper
Hemangioblastomas (HBs) are benign, highly vascular tumors, often characterized by loss of function of the von Hippel-Lindau (vHL) gene. They are the most common central nervous system tumor observed in vHL syndrome. Loss of function of the vHL gene creates a "pseudo-hypoxic" state, causing overactivation of hypoxia-inducible factor (HIF) and vascular endothelial growth factor (VEGF)-related pathways. In some cases, HBs can rapidly increase in size during pregnancy to then present acutely, which most frequently occurs after the 20th gestational week...
October 13, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/29016818/posterior-fossa-syndrome-and-long-term-neuropsychological-outcomes-among-children-treated-for-medulloblastoma-on-a-multi-institutional-prospective-study
#5
Jane E Schreiber, Shawna L Palmer, Heather M Conklin, Donald J Mabbott, Michelle A Swain, Melanie J Bonner, Mary L Chapieski, Lu Huang, Hui Zhang, Amar Gajjar
Background: Patients treated for medulloblastoma who experience posterior fossa syndrome (PFS) demonstrate increased risk for neurocognitive impairment at one year post diagnosis. The aim of the study was to examine longitudinal trajectories of neuropsychological outcomes in patients who experienced PFS compared with patients who did not. Methods: Participants were 36 patients (22 males) who experienced PFS and 36 comparison patients (21 males) who were matched on age at diagnosis and treatment exposure but did not experience PFS...
July 19, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28984782/chiari-type-i-malformation-with-occult-tethered-cord-syndrome-in-a-child-a-case-report
#6
Yuan Zhou, Lin Zhu, Yixing Lin, Huilin Cheng
RATIONALE: Chiari type I malformation (CM1) and occult tethered cord syndrome (OTCS) are considered to be malformations associated with subtle structural abnormalities of the terminal filum. Few studies have reported patients with CM1 and OTCS. Treatment strategy for patients of CM1 associated with OTCS is controversial. PATIENT CONCERNS: A 14-year-old child was admitted with intermittent pain and numbness in the right upper limb. And he had urinary frequency, neck pain, back pain, and numbness simultaneously...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28960048/coexistence-of-radiation-induced-meningioma-and-moyamoya-syndrome-10-years-after-irradiation-against-medulloblastoma-a-case-report
#7
Ji Yeon Han, Jung Won Choi, Kyu Chang Wang, Ji Hoon Phi, Ji Yeoun Lee, Jong Hee Chae, Sung Hye Park, Jung Eun Cheon, Seung Ki Kim
Radiotherapy is one of the standard treatments for medulloblastoma. However, therapeutic central nervous system irradiation in children may carry delayed side effects, such as radiation-induced tumor and vasculopathy. Here, we report the first case of coexisting meningioma and moyamoya syndrome, presenting 10 years after radiotherapy for medulloblastoma. A 13-year-old boy presented with an enhancing mass at the cerebral falx on magnetic resonance imaging (MRI) after surgery, radiotherapy (30.6 Gy craniospinal axis, 19...
November 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28924528/near-fatal-gastrointestinal-hemorrhage-in-a-child-with-medulloblastoma-on-high-dose-dexamethasone
#8
Derek Yecies, Daniel Tawfik, Jennifer Damman, Chad Thorson, David S Hong, Gerald A Grant, Rachel Bensen, Mihaela Damian
A four-year-old female was admitted to a university-based children's hospital with a newly-diagnosed posterior fossa tumor. She was started on famotidine and high-dose dexamethasone and underwent gross total resection of a medulloblastoma. She was continued on dexamethasone and famotidine. She exhibited postoperative posterior fossa syndrome and was started on enteral feeds via the nasoduodenal tube. She had small gastrointestinal bleeds on postoperative days eight, 11, and 18, and was found to have a well-circumscribed posterior duodenal ulcer...
July 7, 2017: Curēus
https://www.readbyqxmd.com/read/28892993/foetal-magnetic-resonance-images-of-two-cases-of-aicardi-syndrome
#9
Sebastián Gacio, Sebastián Lescano
Cerebral malformations are of fundamental importance in the clinical diagnosis of Aicardi Syndrome (AS). Some of these anomalies like callosal agenesis, cysts formation, posterior fossa anomalies and gross interhemispheric asymmetry are easily observed in the prenatal period with the use of foetal Magnetic Resonance Images (MRI). We present two cases of female newborns with cerebral MRI performed in the prenatal period and further diagnosed with AS. With the increase use of foetal MRI, AS will be easier suspected in the prenatal period in a female fetus with typical brain anomalies...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28887276/moyamoya-vasculopathy-in-phace-syndrome-six-new-cases-and-review-of-the-literature
#10
REVIEW
Domenico Tortora, Mariasavina Severino, Andrea Accogli, Carola Martinetti, Nadia Vercellino, Valeria Capra, Andrea Rossi, Marco Pavanello
PHACE syndrome is a neurocutaneous disorder including posterior fossa malformations, hemangiomas, arterial lesions, cardiac defects, and eye abnormalities. PHACE arteriopathies may be progressive and have been recently categorized based on the risk of acute ischemic stroke, raising attention to the potentially devastating consequences of cerebrovascular complications in this syndrome. On the other hand, the natural history of arteriopathy in PHACE syndrome remains poorly understood. At the moment, there are no established surgical guidelines for high risk vasculopathies, including quasi-moyamoya, in this syndrome...
September 5, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28814594/concomitant-carotid-aplasia-and-basilar-artery-occlusion-in-a-child-with-phaces-syndrome
#11
Noel Delgado, Jason J Carroll, Philip M Meyers
We report a case of an 8-year-old girl with posterior fossa abnormalities, haemangioma, arterial lesions, cardiac abnormalities or coarctation of the aorta and eye abnormalities syndrome with right carotid aplasia and complete basilar occlusion. The patient initially presented at 2.5 weeks of age with a growing right facial haemangioma involving segments 1, 3 and 4. Initial MRI at 2.5 weeks of age revealed an extraconal right orbital haemangioma without posterior fossa abnormalities and MR angiography (MRA) 3 weeks later showed right internal carotid aplasia...
August 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28761539/extensive-intracranial-calcification-of-pseudo-torch-syndrome-with-features-of-dandy-walker-malformation
#12
Ashis Patnaik, Sudhansu Sekhar Mishra, Srikanta Das
Pseudo-TORCH syndrome or congenital infection-like syndrome is a group of conditions which resemble congenital infections such as those caused by toxoplasmosis, rubella, cytomegalovirus (CMV), herpes (TORCH) group of organisms, clinico-radiologically, but serological tests are negative for the organisms. One of the variety shows features such as microcephaly, extensive intracranial calcification showing gross resemblance to congenital CMV infection, making its other name as microcephaly intracranial calcification syndrome (MICS)...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28713626/recurrent-hemorrhage-in-hemangioblastoma-involving-the-posterior-fossa-case-report
#13
Eric Marvin, Asad S Akhter, Jeroen R Coppens
BACKGROUND: Hemangioblastomas (HGBs) are the most common primary intra-axial posterior fossa tumor in adults. Although spontaneous hemorrhage of these tumors is exceedingly rare, despite their vascular nature, we describe a case of recurrent hemorrhage with associated tonsillar herniation, and demonstrate that a surgical approach can provide a suitable outcome. CASE DESCRIPTION: A 54-year-old female with von Hippel-Lindau (VHL) syndrome presented with acute loss of consciousness and Glasgow Coma Scale (GCS) was 4...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28686325/rnf213-variants-in-a-child-with-phace-syndrome-and-moyamoya-vasculopathy
#14
Kala F Schilter, Jack E Steiner, Wendy Demos, Mohit Maheshwari, Jeremy W Prokop, Elizabeth Worthey, Beth A Drolet, Dawn H Siegel
Segmental infantile hemangiomas (IH) can be associated with congenital anomalies in a regional distribution. PHACE refers to large cervicofacial segmental IH in association with congenital anomalies of the aortic arch and medium-sized arteries of the head and neck, as well as structural anomalies of the posterior fossa and eye. A subset of PHACE patients have arterial anomalies that progress to moyamoya vasculopathy (MMV). MMV is defined as stenosis of the supraclinoid segment of the internal carotid arteries and/or their major branches, with subsequent development of a compensatory collateral vessel network...
July 7, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28685259/extremely-large-sinus-pericranii-with-involvement-of-the-torcular-and-associated-with-crouzon-s-syndrome
#15
Felipe H Sanders, Bryan A Edwards, Matthew Fusco, Rod J Oskouian, R Shane Tubbs, James M Johnston
INTRODUCTION: Sinus pericranii is a rare vascular malformation that connects the intracranial dural sinuses to the extracranial venous drainage system and is caused by either trauma or congenital defects. Although the majority of these vascular structures are due to trauma, some are congenital. CASE REPORT: Herein, we report a 5-month-old patient with a very large and fluctuating subcutaneous mass over the occiput and the diagnosis of Crouzon's syndrome. The child presented with a large midline mass that on imaging, connected to the underlying torcular and was diagnosed as a sinus pericranii...
July 6, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28665854/morphometric-analysis-of-the-posterior-cranial-fossa-in-syndromic-and-nonsyndromic-craniosynostosis
#16
Thomas Beez, David Koppel, Meharpal Sangra
Posterior cranial fossa (PCF) anatomy can be abnormal in craniosynostosis, and hindbrain herniation may occur. This study analyzed PCF anatomy in single suture and complex craniosynostosis.Children with craniosynostosis and age-matched controls were identified. Cephalic index (CI) for cranial vault and PCF as well as tentorial (TA) and occipital angles (OA) were measured on preoperative imaging.Children with syndromic (N = 6), bicoronal (N = 4), sagittal (N = 12), and metopic synostosis (N = 4) as well as controls (N = 10) were enrolled...
July 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28637445/cerebellar-mutism-syndrome-in-children-with-brain-tumours-of-the-posterior-fossa
#17
Morten Wibroe, Johan Cappelen, Charlotte Castor, Niels Clausen, Pernilla Grillner, Thora Gudrunardottir, Ramneek Gupta, Bengt Gustavsson, Mats Heyman, Stefan Holm, Atte Karppinen, Camilla Klausen, Tuula Lönnqvist, René Mathiasen, Pelle Nilsson, Karsten Nysom, Karin Persson, Olof Rask, Kjeld Schmiegelow, Astrid Sehested, Harald Thomassen, Ingrid Tonning-Olsson, Barbara Zetterqvist, Marianne Juhler
BACKGROUND: Central nervous system tumours constitute 25% of all childhood cancers; more than half are located in the posterior fossa and surgery is usually part of therapy. One of the most disabling late effects of posterior fossa tumour surgery is the cerebellar mutism syndrome (CMS) which has been reported in up to 39% of the patients but the exact incidence is uncertain since milder cases may be unrecognized. Recovery is usually incomplete. Reported risk factors are tumour type, midline location and brainstem involvement, but the exact aetiology, surgical and other risk factors, the clinical course and strategies for prevention and treatment are yet to be determined...
June 21, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28626825/phace-s-syndrome-report-of-a-case-with-new-ocular-and-systemic-manifestations
#18
Raheleh Assari, Vahid Ziaee, Sasan Moghimi, Mohammad Reza Akbari, Arash Mirmohammadsadeghi
PURPOSE: To describe an infant with PHACE(S) syndrome [posterior fossa anomalies (P), hemangiomas (H), arterial anomalies (A), cardiac abnormalities and coarctation of aorta (C), eye abnormalities (E), and the sternal defects (S)] with unusual strabismus, congenital glaucoma, and new systemic manifestations. METHODS: A 6-month-old girl was referred with large hemangiomas on the left side of the face. RESULTS: In the ocular examination, right esotropia and hypotropia, and limitation of elevation in adduction in the right eye were seen...
June 2017: Journal of Current Ophthalmology
https://www.readbyqxmd.com/read/28607814/chiari-i-malformation-and-syringomyelia-in-mucopolysaccharidosis-type-i-hurler-syndrome-treated-with-posterior-fossa-decompression-case-report-and-review-of-the-literature
#19
Vyacheslav Makler, Christina L Goldstein, Daniel Hoernschemeyer, Tomoko Tanaka
BACKGROUND: Hurler Syndrome is the most severe phenotype of mucopolysaccharidosis type I. With bone marrow transplant and enzyme replacement therapy, the life expectancy of a child with Hurler syndrome has been extended, predisposing them to multiple musculoskeletal issues most commonly involving the spine. CASE DESCRIPTION: This is the case report of a 6-year-old male with Hurler syndrome who was diagnosed with Chiari I malformation and cervicothoracic syringomyelia on a preoperative magnetic resonance imaging (MRI) for his thoracolumbar kyphosis...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28555453/-a-phenotypic-description-of-26-patients-with-ritscher-schinzel-syndrome-cranio-cerebello-cardiac-dysplasia-or-3c-syndrome
#20
S M Pira-Paredes, J H Montoya-Villada, J L Franco-Restrepo, M Moncada-Velez, J W Cornejo
INTRODUCTION: Ritscher-Schinzel syndrome (also known as cranio-cerebello-cardiac dysplasia or 3C syndrome) is a rare genetic syndrome that is mainly characterised by the association of cardiac and craniofacial anomalies together with others affecting the posterior fossa. PATIENTS AND METHODS: We report on 26 patients with Ritscher-Schinzel syndrome at a hospital in Medellin, in the Department of Antioquia, Colombia. RESULTS: Males account for 69% of this cohort...
June 1, 2017: Revista de Neurologia
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