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Sample records for cerebral cavernous malformations

  1. Vascular permeability in cerebral cavernous malformations

    DEFF Research Database (Denmark)

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao;

    2015-01-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observ......Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case......-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability...... correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive...

  2. Vascular permeability in cerebral cavernous malformations.

    Science.gov (United States)

    Mikati, Abdul G; Khanna, Omaditya; Zhang, Lingjiao; Girard, Romuald; Shenkar, Robert; Guo, Xiaodong; Shah, Akash; Larsson, Henrik B W; Tan, Huan; Li, Luying; Wishnoff, Matthew S; Shi, Changbin; Christoforidis, Gregory A; Awad, Issam A

    2015-10-01

    Patients with the familial form of cerebral cavernous malformations (CCMs) are haploinsufficient for the CCM1, CCM2, or CCM3 gene. Loss of corresponding CCM proteins increases RhoA kinase-mediated endothelial permeability in vitro, and in mouse brains in vivo. A prospective case-controlled observational study investigated whether the brains of human subjects with familial CCM show vascular hyperpermeability by dynamic contrast-enhanced quantitative perfusion magnetic resonance imaging, in comparison with CCM cases without familial disease, and whether lesional or brain vascular permeability correlates with CCM disease activity. Permeability in white matter far (WMF) from lesions was significantly greater in familial than in sporadic cases, but was similar in CCM lesions. Permeability in WMF increased with age in sporadic patients, but not in familial cases. Patients with more aggressive familial CCM disease had greater WMF permeability compared to those with milder disease phenotype, but similar lesion permeability. Subjects receiving statin medications for routine cardiovascular indications had a trend of lower WMF, but not lesion, permeability. This is the first demonstration of brain vascular hyperpermeability in humans with an autosomal dominant disease, as predicted mechanistically. Brain permeability, more than lesion permeability, may serve as a biomarker of CCM disease activity, and help calibrate potential drug therapy.

  3. Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment

    Directory of Open Access Journals (Sweden)

    Nikolaos Mouchtouris

    2015-01-01

    Full Text Available Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment—microsurgical resection, stereotactic radiosurgery, and conservative management—depending on the lesion characteristics.

  4. Vascular permeability and iron deposition biomarkers in longitudinal follow-up of cerebral cavernous malformations

    DEFF Research Database (Denmark)

    Girard, Romuald; Fam, Maged D; Zeineddine, Hussein A;

    2017-01-01

    OBJECTIVE Vascular permeability and iron leakage are central features of cerebral cavernous malformation (CCM) pathogenesis. The authors aimed to correlate prospective clinical behavior of CCM lesions with longitudinal changes in biomarkers of dynamic contrast-enhanced quantitative permeability (...

  5. FAM222B Is Not a Likely Novel Candidate Gene for Cerebral Cavernous Malformations

    NARCIS (Netherlands)

    Spiegler, Stefanie; Kirchmaier, Bettina; Rath, Matthias; Korenke, G. Christoph; Tetzlaff, Fabian; Van De Vorst, Maartje; Neveling, Kornelia; Acker-Palmer, Amparo; Kuss, Andreas W.; Gilissen, Christian; Fischer, Andreas; Schulte-Merker, Stefan; Felbor, Ute

    2016-01-01

    Cerebral cavernous malformations (CCMs) are prevalent slow-flow vascular lesions which harbour the risk to develop intracranial haemorrhages, focal neurological deficits, and epileptic seizures. Autosomal dominantly inherited CCMs were found to be associated with heterozygous inactivating mutations

  6. Cerebral cavernous malformations: clinical insights from genetic studies.

    Science.gov (United States)

    Mindea, Stefan A; Yang, Benson P; Shenkar, Robert; Bendok, Bernard; Batjer, H Hunt; Awad, Issam A

    2006-07-15

    Familial disease is responsible for one third to one half of cerebral cavernous malformation (CCM) cases presenting to clinical attention. Much has been learned in the past decade about the genetics of these cases, which are all inherited in an autosomal dominant pattern, at three known chromosome loci. Unique features of inherited CCMs in Hispanic-Americans of Mexican descent have been described. The respective genes for each locus have been identified and preliminary observations on disease pathways and mechanisms are coming to light, including possible explanations for selectivity of neural milieu and relationships to endothelial layer abnormalities. Mechanisms of lesion genesis in cases of genetic predisposition are being investigated, with evidence to support a two-hit model emerging from somatic mutation screening of the lesions themselves and from lesion formation in transgenic murine models of the disease. Other information on potential inflammatory factors has emerged from differential gene expression studies. Unique phenotypic features of solitary versus familial cases have emerged: different associations with venous developmental anomaly and the exceptionally high penetrance rates that are found in inherited cases when high-sensitivity screening is performed with gradient echo magnetic resonance imaging. This information has changed the landscape of screening and counseling for patients and their families, and promises to lead to the development of new tools for predicting, explaining, and modifying disease behavior.

  7. ADVANCED MAGNETIC RESONANCE IMAGING OF CEREBRAL CAVERNOUS MALFORMATIONS

    Science.gov (United States)

    Shenkar, Robert; Venkatasubramanian, Palamadai N.; Wyrwicz, Alice M.; Zhao, Jin-cheng; Shi, Changbin; Akers, Amy; Marchuk, Douglas A.; Awad, Issam A.

    2008-01-01

    Objective We sought to assess the appearance of cerebral cavernous malformations (CCMs) on magnetic resonance (MR) imaging in murine Ccm1 and Ccm2 gene knockout models, and to develop a technique of lesion localization for correlative pathobiologic studies Methods Brains from eighteen CCM mutant mice (Ccm1+/-Trp53-/- and Ccm2+/-Trp53-/-) and 28 controls were imaged by gradient recalled echo (T2*)-weighted MR at 4.7 T and 14.1 T in vivo and/or ex vivo. After MR imaging, the brains were removed and stained with hematoxylin and eosin and cells were laser microdissected for molecular biologic studies. Results T2*-weighted MR imaging of brains in vivo and ex vivo revealed lesions similar to human CCMs in mutant mice, but not in control animals. Stereotactic localization and hematoxylin and eosin-staining of correlative tissue sections confirmed lesion histology, and revealed other areas of dilated capillaries in the same brains. Some lesions were identified by MR imaging at 14.1 T, but not at 4.7 T. PCR amplification from Ccm1 and β-actin genes was demonstrated from nucleic acids extracted from laser microdissected lesional and perilesional cells. Conclusions The high field MR imaging techniques offer new opportunities for further investigation of disease pathogenesis in vivo, and the localization, staging and histobiologic dissection of lesions, including the presumed earliest stages of CCM lesion development. PMID:18981891

  8. Novel, mutable site in the cerebral cavernous malformation-1 gene in Chinese sporadic intracranial cavernous malformation patients

    Institute of Scientific and Technical Information of China (English)

    Rong Xie; Xiancheng Chen

    2009-01-01

    BACKGROUND: A cerebral cavernous malformation-1 (CCM1) gene mutation might result in functional loss of KREV interaction trapped-1 (KRIT1), which is related to onset of cavernous malformations (CM). However, data addressing sporadic CM in Chinese patients remains limited to date.OBJECTIVE: To analyze CCM1 mutation of Chinese patients with sporadic intracranial CM.DESIGN, TIME AND SETTING: Genetics experiment was performed in the Department of Neurosurgery, Huashan Hospital Affiliated to Fudan University between January 2004 and December 2005.PARTICIPANTS: Ninety patients with sporadic CM served as the CM group, and 30 healthy subjects were considered to be the control group.METHODS: Peripheral blood was collected from patients with CM and from control group subjects.Genomic DNA was extracted, and exons 8, 9, 11, 12, 13, 15, 16, 17, and 18, as well as the related introns, were amplified using polymerase chain reaction. DNA sequences were compared with GeneBank.MAIN OUTCOME MEASURES: Abnormal mutable site of CCM1 gene in the two groups.RESULTS: Four exclusive mutations of CCM1 were detected in the CM group, with a sporadic CM mutational rate of 32% (6/19). Of the four exclusive mutations, there was one missense mutation [exon 12, 1172C→T (S391F)], one insertion mutation [exon 8, 704insT (K246stop)], one intervening sequence mutation (IVS12-4C→T), and one synonymous mutation (exon 17,1875C→T). With the exception of 1875C→T, all mutations detected in the CM group led to functional changes of the KRIT1 protein, which was encoded by the CCM1 gene. Gene mutations were not detected in the control group.CONCLUSION: Four exclusive mutations of the CCM1 gene were determined in Chinese patients with sporadic CM, which led to functional changes or loss of the encoding KRIT1 protein. KRIT1 protein is considered to be the genetic basis of CM occurrence.

  9. Detection of Novel Mutation in Ccm3 Causes Familial Cerebral Cavernous Malformations.

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    Scimone, Concetta; Bramanti, Placido; Ruggeri, Alessia; Katsarou, Zoe; Donato, Luigi; Sidoti, Antonina; D'Angelo, Rosalia

    2015-11-01

    Cerebral cavernous malformations are vascular lesions that usually involve brain micro-vessels. They can occur both in a sporadic form and familial one. Causes of familial forms are mutations at three loci: CCM1/KRIT1, CCM2/MGC4607, and CCM3/PDCD10. Here, we describe a novel CCM3 missense mutation (c.422T>G) detected in two Greek brothers showing multiple lesions at magnetic resonance imaging; to date, only the youngest is symptomatic. Bioinformatics tools showed this novel variant causes a loss of function in Pdcd10 protein due to its localization in the eighth helix and, particularly, affects Leu141, a highly conserved amino acid. Roles of Pdcd10 in angiogenesis regulation and its association with early development of cerebral cavernous malformations were also considered.

  10. Cerebral cavernous malformations as a disease of vascular permeability: from bench to bedside with caution.

    Science.gov (United States)

    Yadla, Sanjay; Jabbour, Pascal M; Shenkar, Robert; Shi, Changbin; Campbell, Peter G; Awad, Issam A

    2010-09-01

    Tremendous insight into the molecular and genetic pathogenesis of cerebral cavernous malformations (CCMs) has been gained over the past 2 decades. This includes the identification of 3 distinct genes involved in familial CCMs. Still, a number of unanswered questions regarding the process from gene mutation to vascular malformation remain. It is becoming more evident that the disruption of interendothelial junctions and ensuing vascular hyperpermeability play a principal role. The purpose of this review is to summarize the current understanding of CCM genes, associated proteins, and functional pathways. Promising molecular and genetic therapies targeted at identified molecular aberrations are discussed as well.

  11. Image-guided resection of cerebral cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    毛颖; 周良辅; 杜固宏; 陈亮

    2003-01-01

    Objective To evaluate retrospectively the effectiveness of image-guided navigation techniques in the management of cerebral CMs.Methods Between July 1997 and January 2001, 44 patients underwent image-guided resection of cerebral CMs. To counteract brain shift, a small silicon catheter was implanted as a guide in the case of deep-seated lesions (except in the case of brain stem CMs) and before excision of multiple lesions. Results A total of 27 men and 17 women with a mean age of 35 years underwent surgical procedures (5 patients had multiple lesions). The lesions were located in the frontal (n=14), lobe temporal lobe (n=12), parietal lobe (n=6), cerebellum (n=6), thalamus (n=5), pons (n=5), and orbital region (n=1). Under the guidance of a StealthStation navigator, total removal of the lesions was achieved in all patients. Follow-up revealed marked improvement of preoperative symptoms in 26 patients and no additional deficits in 13 patients. Five patients suffered from additional neurological deficits, but two of them gradually improved during the follow-up period. Conclusions With the assistance of an image-guided surgical system, functional areas can be effectively avoided and surgical injury can be decreased. This system is well suited for accurate localization and safe resection of small, deep-seated CMs.

  12. Surveying genetic variants and molecular phylogeny of cerebral cavernous malformation gene, CCM3/PDCD10.

    Science.gov (United States)

    Kumar, Abhishek; Bhandari, Anita; Goswami, Chandan

    2014-12-01

    The three cerebral cavernous malformations (CCMs) genes namely CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10 have been identified for which mutations cause cerebral cavernous malformations. However, the protein products of these genes involved in forming CCM signaling, are still poorly understood imposing an urgent need to understand these genes and their signaling processes in details. So far involvement of CCM3/PDCD10 in the cavernous angioma has been characterized from biochemical and biophysical analyses. However, there is no comprehensive study illustrating the phylogenetic history and comprehensive genetic variants of CCM3/PDCD10. Herein, we explored the phylogenetic history and genetic variants of CCM3/PDCD10 gene. Synteny analyses revealed that CCM3/PDCD10 gene shared same genomic loci from Drosophila to human and the gene structure of CCM3/PDCD10 is conserved from human to Branchiostoma floridae for about 500 MYs with some changes in sea urchin and in insects. The conserved CCM3/PDCD10 is characterized by presence of indels in the N-terminal dimerization domain. We identified 951 CCM3/PDCD10 variants by analysis of 1092 human genomes with top three variation classes belongs to 84% SNPs, 6.9% insertions and 6.2% deletions. We identified 22 missense mutations in the human CCM3/PDCD10 protein and out of which three mutations are deleterious. We also identified four stop-codon gaining mutations at the positions E34*, E68*, E97* and E140*, respectively. This study is the first comprehensive analysis of the CCM3/PDCD10 gene based on phylogenetic origin and genetic variants. This study corroborates that the evolution of CCM proteins with tubular organization evolvements by endothelial cells.

  13. ADVANCED MAGNETIC RESONANCE IMAGING OF CEREBRAL CAVERNOUS MALFORMATIONS: I. HIGH FIELD IMAGING OF EXCISED HUMAN LESIONS

    Science.gov (United States)

    Shenkar, Robert; Venkatasubramanian, Palamadai N.; Zhao, Jin-cheng; Batjer, H. Hunt; Wyrwicz, Alice M.; Awad, Issam A.

    2008-01-01

    Objectives We hypothesized that structural details would be revealed in cerebral cavernous malformations (CCMs) through the use of high field magnetic resonance (MR) and confocal microscopy, which have not been described previously. The structural details of CCMs excised from human patients were sought by examination with high field MR imaging, and correlated with confocal microscopy of the same specimens. Novel features of CCM structure are outlined, including methodological limitations, venues for future research and possible clinical implications. Methods CCM lesions excised from four patients were fixed in 2% paraformaldehyde and subjected to high resolution MR imaging at 9.4 or 14.1 Tesla by spin-echo and gradient recalled echo methods. Histological validation of angioarchitecture was conducted on thick sections of CCM lesions using fluorescent probes to endothelium under confocal microscopy. Results Images of excised human CCM lesions were acquired with proton density-weighted, T1-weighted, T2-weighted spin echo and T2*-weighted gradient-recalled echo MR. These images revealed large “bland” regions with thin walled caverns, and “honeycombed” regions with notable capillary proliferation and smaller caverns surrounding larger caverns. Proliferating capillaries and caverns of various sizes were also associated with the wall of apparent larger blood vessels in the lesions. Similar features were confirmed within thick sections of CCMs by confocal microscopy. MR relaxation times in different regions of interest suggested the presence of different states of blood breakdown products in areas with apparent angiogenic proliferative activity. Conclusions The high field MR imaging techniques demonstrate novel features of CCM angioarchitecture, visible at near histological resolution, including regions with apparently different biologic activity. These preliminary observations will motivate future research, correlating lesion biologic and clinical activity with

  14. Immune complex formation and in situ B-cell clonal expansion in human cerebral cavernous malformations.

    Science.gov (United States)

    Shi, Changbin; Shenkar, Robert; Kinloch, Andrew; Henderson, Scott G; Shaaya, Mark; Chong, Anita S; Clark, Marcus R; Awad, Issam A

    2014-07-15

    Cerebral cavernous malformations (CCMs) represent clusters of dilated vascular channels, predisposing to hemorrhagic stroke and seizures. They are associated with defective blood brain barrier, hemorrhages of different ages and a robust inflammatory cell infiltrate. We report for the first time evidence of co-localized IgG and complement membrane attack complexes in CCM lesions. CD4(+) and CD8(+) T-cells are aggregated with CD20(+) B-cells. And IgG repertoire analyses demonstrate in situ B-cell clonal expansion and antigen-driven affinity maturation in CCMs. These results suggest an organ-intrinsic adaptive immune response in CCMs that should be further characterized as a potential therapeutic target.

  15. Unilateral facial palsy in an infant: an unusual presentation of familial multiple cerebral cavernous malformation.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Cerebral cavernous malformation (CCM) in infants tends to have genetic predisposition. These cavernomas have a progressive course of events and associated neurological symptoms with increase in age. They most commonly present with seizure and syndrome of increased intracranial pressure comprising of headache, vomiting and focal neurological signs. We describe a case of a 7-month-old infant who presented with an acute onset of right facial paralysis with a background of familial CCM. The CT and MRI scan revealed fresh haemorrhage in the right cerebellar and pontine cavernomas with surrounding oedema and no evidence of obstructive hydrocephalus. These two cavernomas re-bled in a week duration causing episodes of incessant crying and irritability. After discussing the pros and cons of treatment, owing to stable clinical status, the patient is currently been managed conservatively.

  16. Stabiliztin of VEGFR2 Signaling by Cerebral Cavernous Malformation 3 is Critical for Vascular Development

    Energy Technology Data Exchange (ETDEWEB)

    Y He; H Zhang; L Yu; M Gunel; T Boggon; H Chen; W Min

    2011-12-31

    Cerebral cavernous malformations (CCMs) are human vascular malformations caused by mutations in three genes of unknown function: CCM1, CCM2, and CCM3. CCM3, also known as PDCD10 (programmed cell death 10), was initially identified as a messenger RNA whose abundance was induced by apoptotic stimuli in vitro. However, the in vivo function of CCM3 has not been determined. Here, we describe mice with a deletion of the CCM3 gene either ubiquitously or specifically in the vascular endothelium, smooth muscle cells, or neurons. Mice with global or endothelial cell-specific deletion of CCM3 exhibited defects in embryonic angiogenesis and died at an early embryonic stage. CCM3 deletion reduced vascular endothelial growth factor receptor 2 (VEGFR2) signaling in embryos and endothelial cells. In response to VEGF stimulation, CCM3 was recruited to and stabilized VEGFR2, and the carboxyl-terminal domain of CCM3 was required for the stabilization of VEGFR2. Indeed, the CCM3 mutants found in human patients lacking the carboxyl-terminal domain were labile and were unable to stabilize and activate VEGFR2. These results demonstrate that CCM3 promotes VEGFR2 signaling during vascular development.

  17. A novel CCM2 gene mutation associated with family cerebral cavernous malformation

    Directory of Open Access Journals (Sweden)

    Wen-Qing Huang

    2016-09-01

    Full Text Available Background: Cerebral cavernous malformations (CCMs are common vascular malformations that predominantly arise in the central nervous system and are mainly characterized by enlarged vascular cavities without intervening brain parenchyma. Familial CCMs (FCCMs is inherited in an autosomal dominant pattern with incomplete penetrance and variable symptoms. Methods: Mutations of three pathogenic genes, CCM1, CCM2 and CCM3, were investigated by direct DNA sequencing in a Chinese family with multiple CCM lesions. Results: Four heterozygous variants in the CCM2 gene, including one deletion (c.95delC, a missense mutation (c.358G>A, p.V120I, one silent mutation (c.915G>A, p.T305T, and a substitution (c. *1452 T>C, were identified in the subjects with multiple CCM lesions, but not in a healthy sibling. Among these variants, the c.95delC deletion is a novel mutation which is expected to cause a premature termination codon. It is predicted to produce a truncated CCM2 protein lacking the PTB and C-terminal domains, thus disrupting the molecular functions of CCM2. Conclusions: The novel truncating mutation in the CCM2 gene, c.95delC, may be responsible for multiple CCM lesions in a part of FCCM. In addition, it may represent a potential genetic biomarker for early diagnosis of FCCM.

  18. Sporadic Cerebral Cavernous Malformations: Report of Further Mutations of CCM Genes in 40 Italian Patients

    Directory of Open Access Journals (Sweden)

    Rosalia D’Angelo

    2013-01-01

    Full Text Available Cerebral cavernous malformations (CCMs are vascular lesions characterized by abnormally enlarged capillary cavities, affecting the central nervous system. CCMs can occur sporadically or as a familial autosomal dominant condition with incomplete penetrance and variable clinical expression attributable to mutations in three different genes: CCM1 (K-Rev interaction trapped 1 (KRIT1, CCM2 (MGC4607, and CCM3 (PDCD10. CCMs occur as a single or multiple malformations that can lead to seizures, focal neurological deficits, hemorrhagic stroke, and headache. However, patients are frequently asymptomatic. In our previous mutation screening, performed in a cohort of 95 Italian patients, both sporadic and familial, we have identified several mutations in CCM genes, three of which in three distinct sporadic patients. In this study, representing further molecular screening of the three CCM genes, in a south Italian cohort of CCM patients enrolled by us in the last three years, we report the identification of other four new mutations in 40 sporadic patients with either single or multiple CCM.

  19. Sporadic Cerebral Cavernous Malformations: Report of Further Mutations of CCM Genes in 40 Italian Patients

    Science.gov (United States)

    D'Angelo, Rosalia; Alafaci, Concetta; Scimone, Concetta; Ruggeri, Alessia; Salpietro, Francesco Maria; Bramanti, Placido; Tomasello, Francesco; Sidoti, Antonina

    2013-01-01

    Cerebral cavernous malformations (CCMs) are vascular lesions characterized by abnormally enlarged capillary cavities, affecting the central nervous system. CCMs can occur sporadically or as a familial autosomal dominant condition with incomplete penetrance and variable clinical expression attributable to mutations in three different genes: CCM1 (K-Rev interaction trapped 1 (KRIT1)), CCM2 (MGC4607), and CCM3 (PDCD10). CCMs occur as a single or multiple malformations that can lead to seizures, focal neurological deficits, hemorrhagic stroke, and headache. However, patients are frequently asymptomatic. In our previous mutation screening, performed in a cohort of 95 Italian patients, both sporadic and familial, we have identified several mutations in CCM genes, three of which in three distinct sporadic patients. In this study, representing further molecular screening of the three CCM genes, in a south Italian cohort of CCM patients enrolled by us in the last three years, we report the identification of other four new mutations in 40 sporadic patients with either single or multiple CCM. PMID:24058906

  20. Structure and vascular function of MEKK3–cerebral cavernous malformations 2 complex

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    Fisher, Oriana S. [Yale Univ., New Haven, CT (United States); Deng, Hanqiang [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China); Liu, Dou [Yale Univ. School of Medicine, New Haven, CT (United States); Zhang, Ya [Yale Univ. School of Medicine, New Haven, CT (United States); Wei, Rong [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China); Deng, Yong [Yale Univ. School of Medicine, New Haven, CT (United States); Zhang, Fan [Yale Univ. School of Medicine, New Haven, CT (United States); Louvi, Angeliki [Yale Univ. School of Medicine, New Haven, CT (United States); Turk, Benjamin E. [Yale Univ., New Haven, CT (United States); Boggon, Titus J. [Yale Univ., New Haven, CT (United States); Su, Bing [Shanghai Jiao Tong Univ. School of Medicine (SJTU-SM), Shanghai (China)

    2015-08-03

    Cerebral cavernous malformations 2 (CCM2) loss is associated with the familial form of CCM disease. The protein kinase MEKK3 (MAP3K3) is essential for embryonic angiogenesis in mice and interacts physically with CCM2, but how this interaction is mediated and its relevance to cerebral vasculature are unknown. Here we report that Mekk3 plays an intrinsic role in embryonic vascular development. Inducible endothelial Mekk3 knockout in neonatal mice is lethal due to multiple intracranial haemorrhages and brain blood vessels leakage. We discover direct interaction between CCM2 harmonin homology domain (HHD) and the N terminus of MEKK3, and determine a 2.35 Å cocrystal structure. We find Mekk3 deficiency impairs neurovascular integrity, which is partially dependent on Rho–ROCK signalling, and that disruption of MEKK3:CCM2 interaction leads to similar neurovascular leakage. We conclude that CCM2:MEKK3-mediated regulation of Rho signalling is required for maintenance of neurovascular integrity, unravelling a mechanism by which CCM2 loss leads to disease.

  1. Genetically diagnosed Birt-Hogg-Dubé syndrome and familial cerebral cavernous malformations in the same individual: a case report.

    Science.gov (United States)

    Whitworth, James; Stausbøl-Grøn, Brian; Skytte, Anne-Bine

    2017-01-01

    When faced with an unusual clinical feature in a patient with a Mendelian disorder, the clinician may entertain the possibilities of either the feature representing a novel manifestation of that disorder or the co-existence of a different inherited condition. Here we describe an individual with a submandibular oncocytoma, pulmonary bullae and renal cysts as well as multiple cerebral cavernous malformations and haemangiomas. Genetic investigations revealed constitutional mutations in FLCN, associated with Birt-Hogg-Dubé syndrome (BHD) and CCM2, associated with familial cerebral cavernous malformation. Intracranial vascular pathologies (but not cerebral cavernous malformation) have recently been described in a number of individuals with BHD (Kapoor et al. in Fam Cancer 14:595-597, 10.1007/s10689-015-9807-y , 2015) but it is not yet clear whether they represent a genuine part of that conditions' phenotypic spectrum. We suggest that in such instances of potentially novel clinical features, more extensive genetic testing to consider co-existing conditions should be considered where available. The increased use of next generation sequencing applications in diagnostic settings is likely to lead more cases such as this being revealed.

  2. Cerebral cavernous malformations proteins inhibit Rho kinase to stabilize vascular integrity.

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    Stockton, Rebecca A; Shenkar, Robert; Awad, Issam A; Ginsberg, Mark H

    2010-04-12

    Endothelial cell-cell junctions regulate vascular permeability, vasculogenesis, and angiogenesis. Familial cerebral cavernous malformations (CCMs) in humans result from mutations of CCM2 (malcavernin, OSM, MGC4607), PDCD10 (CCM3), or KRIT1 (CCM1), a Rap1 effector which stabilizes endothelial cell-cell junctions. Homozygous loss of KRIT1 or CCM2 produces lethal vascular phenotypes in mice and zebrafish. We report that the physical interaction of KRIT1 and CCM2 proteins is required for endothelial cell-cell junctional localization, and lack of either protein destabilizes barrier function by sustaining activity of RhoA and its effector Rho kinase (ROCK). Protein haploinsufficient Krit1(+/-) or Ccm2(+/-) mouse endothelial cells manifested increased monolayer permeability in vitro, and both Krit1(+/-) and Ccm2(+/-) mice exhibited increased vascular leak in vivo, reversible by fasudil, a ROCK inhibitor. Furthermore, we show that ROCK hyperactivity occurs in sporadic and familial human CCM endothelium as judged by increased phosphorylation of myosin light chain. These data establish that KRIT1-CCM2 interaction regulates vascular barrier function by suppressing Rho/ROCK signaling and that this pathway is dysregulated in human CCM endothelium, and they suggest that fasudil could ameliorate both CCM disease and vascular leak.

  3. Update on Novel CCM Gene Mutations in Patients with Cerebral Cavernous Malformations.

    Science.gov (United States)

    Scimone, Concetta; Bramanti, Placido; Alafaci, Concetta; Granata, Francesca; Piva, Francesco; Rinaldi, Carmela; Donato, Luigi; Greco, Federica; Sidoti, Antonina; D'Angelo, Rosalia

    2017-02-01

    Cerebral cavernous malformations (CCMs) are lesions affecting brain microvessels. The pathogenesis is not clearly understood. Conventional classification criterion is based on genetics, and thus, familial and sporadic forms can be distinguished; however, classification of sporadic cases with multiple lesions still remains uncertain. To date, three CCM causative genes have been identified: CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10. In our previous mutation screening, performed in a cohort of 95 Italian patients, with both sporadic and familial cases, we identified several mutations in CCM genes. This study represents further molecular screening in a cohort of 19 Italian patients enrolled by us in the few last years and classified into familial, sporadic and sporadic with multiple lesions cases. Direct sequencing and multiplex ligation-dependent probe amplification (MLPA) analysis were performed to detect point mutations and large genomic rearrangements, respectively. Effects of detected mutations and single-nucleotide polymorphisms (SNPs) were evaluated by an in silico approach and by western blot analysis. A novel nonsense mutation in CCM1 and a novel missense mutation in CCM2 were detected; moreover, several CCM2 gene polymorphisms in sporadic CCM patients were reported. We believe that these data enrich the mutation spectrum of CCM genes, which is useful for genetic counselling to identify both familial and sporadic CCM cases, as early as possible.

  4. Cerebral Cavernous Malformation: A Portuguese Family with a Novel CCM1 Mutation

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    Marto, João Pedro; Gil, Inês; Calado, Sofia; Viana-Baptista, Miguel

    2016-01-01

    Introduction Cerebral cavernous malformation (CCM) is a vascular disorder characterized by the presence of central nervous system cavernomas. In familial forms, mutations in three genes (CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10) were identified. We describe a Portuguese family harboring a novel CCM1 mutation. Case Presentation The proband is a woman who at the age of 55 years started to have complex partial seizures and episodic headache. Although nothing was found during her neurological examination, brain MRI showed bilateral, supra- and infratentorial cavernomas. She had a sister who, at the age 61 years, suffered a tonic-clonic seizure. Neurological examination was normal and imaging investigation demonstrated a right frontal intracerebral hemorrhage and multiple cavernomas. In the following years, she suffered several complex partial seizures and had a new intracerebral hemorrhage located in the right temporal lobe. Genetic analysis was performed and a novel nucleotide substitution, i.e. c.1927C>T (p.Gln643*) within the exon 17 of the CCM1 gene, was detected in both sisters. The substitution encodes a stop codon, with a consequent truncated KRIT1 protein, therefore supporting its pathogenic role. Further affected family members were detected, suggesting an autosomal dominant pattern of inheritance. Conclusion We report a Portuguese family with a novel CCM1 (KRIT1) mutation – c.1927C>T (p.Gln643*). A better knowledge of the phenotype-genotype correlation is needed to improve the management of CCM patients.

  5. Crystal Structure of CCM3, a Cerebral Cavernous Malformation Protein Critical for Vascular Integrity

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    Li, X.; Zhang, R; Zhang, H; He, Y; Ji, W; Min, W; Boggon, T

    2010-01-01

    CCM3 mutations are associated with cerebral cavernous malformation (CCM), a disease affecting 0.1-0.5% of the human population. CCM3 (PDCD10, TFAR15) is thought to form a CCM complex with CCM1 and CCM2; however, the molecular basis for these interactions is not known. We have determined the 2.5 {angstrom} crystal structure of CCM3. This structure shows an all {alpha}-helical protein containing two domains, an N-terminal dimerization domain with a fold not previously observed, and a C-terminal focal adhesion targeting (FAT)-homology domain. We show that CCM3 binds CCM2 via this FAT-homology domain and that mutation of a highly conserved FAK-like hydrophobic pocket (HP1) abrogates CCM3-CCM2 interaction. This CCM3 FAT-homology domain also interacts with paxillin LD motifs using the same surface, and partial CCM3 co-localization with paxillin in cells is lost on HP1 mutation. Disease-related CCM3 truncations affect the FAT-homology domain suggesting a role for the FAT-homology domain in the etiology of CCM.

  6. Cerebral cavernous malformations arise from endothelial gain of MEKK3-KLF2/4 signalling.

    Science.gov (United States)

    Zhou, Zinan; Tang, Alan T; Wong, Weng-Yew; Bamezai, Sharika; Goddard, Lauren M; Shenkar, Robert; Zhou, Su; Yang, Jisheng; Wright, Alexander C; Foley, Matthew; Arthur, J Simon C; Whitehead, Kevin J; Awad, Issam A; Li, Dean Y; Zheng, Xiangjian; Kahn, Mark L

    2016-04-07

    Cerebral cavernous malformations (CCMs) are common inherited and sporadic vascular malformations that cause strokes and seizures in younger individuals. CCMs arise from endothelial cell loss of KRIT1, CCM2 or PDCD10, non-homologous proteins that form an adaptor complex. How disruption of the CCM complex results in disease remains controversial, with numerous signalling pathways (including Rho, SMAD and Wnt/β-catenin) and processes such as endothelial-mesenchymal transition (EndMT) proposed to have causal roles. CCM2 binds to MEKK3 (refs 7, 8, 9, 10, 11), and we have recently shown that CCM complex regulation of MEKK3 is essential during vertebrate heart development. Here we investigate this mechanism in CCM disease pathogenesis. Using a neonatal mouse model of CCM disease, we show that expression of the MEKK3 target genes Klf2 and Klf4, as well as Rho and ADAMTS protease activity, are increased in the endothelial cells of early CCM lesions. By contrast, we find no evidence of EndMT or increased SMAD or Wnt signalling during early CCM formation. Endothelial-specific loss of Map3k3 (also known as Mekk3), Klf2 or Klf4 markedly prevents lesion formation, reverses the increase in Rho activity, and rescues lethality. Consistent with these findings in mice, we show that endothelial expression of KLF2 and KLF4 is increased in human familial and sporadic CCM lesions, and that a disease-causing human CCM2 mutation abrogates the MEKK3 interaction without affecting CCM complex formation. These studies identify gain of MEKK3 signalling and KLF2/4 function as causal mechanisms for CCM pathogenesis that may be targeted to develop new CCM therapeutics.

  7. Cerebral cavernous malformations arise from endothelial gain of MEKK3-KLF2/4 signaling

    Science.gov (United States)

    Zhou, Zinan; Tang, Alan T.; Wong, Weng-Yew; Bamezai, Sharika; Goddard, Lauren M.; Shenkar, Robert; Zhou, Su; Yang, Jisheng; Wright, Alexander C.; Foley, Matthew; Arthur, J. Simon C.; Whitehead, Kevin J.; Awad, Issam A.; Li, Dean Y.; Zheng, Xiangjian; Kahn, Mark L.

    2016-01-01

    Cerebral cavernous malformations (CCMs) are common inherited and sporadic vascular malformations that cause stroke and seizures in younger individuals1. CCMs arise from endothelial cell loss of KRIT1, CCM2, or PDCD10, non-homologous proteins that form an adaptor complex2. How disruption of the CCM complex results in disease remains controversial, with numerous signaling pathways (including Rho3,4, SMAD5 and Wnt/β-catenin6) and processes such as endothelial-mesenchymal transition (EndMT)5 proposed to play causal roles. CCM2 binds MEKK37–11, and we have recently demonstrated that CCM complex regulation of MEKK3 is essential during vertebrate heart development12. Here, we investigate this mechanism in CCM disease pathogenesis. Using a neonatal mouse model of CCM disease, we find that expression of the MEKK3 target genes KLF2 and KLF4, as well as Rho and ADAMTS protease activity, are increased in the endothelial cells of early CCM lesions. In contrast, we find no evidence of EndMT or increased SMAD or Wnt signaling during early CCM formation. Endothelial-specific loss of Mekk3, Klf2, or Klf4 dramatically prevents lesion formation, reverses the increase in Rho activity, and rescues lethality. Consistent with these findings in mice, we demonstrate that endothelial expression of KLF2 and KLF4 is elevated in human familial and sporadic CCM lesions, and that a disease-causing human CCM2 mutation abrogates MEKK3 interaction without affecting CCM complex formation. These studies identify gain of MEKK3 signaling and KLF2/4 function as causal mechanisms for CCM pathogenesis that may be targeted to develop new CCM therapeutics. PMID:27027284

  8. B-Cell Depletion Reduces the Maturation of Cerebral Cavernous Malformations in Murine Models.

    Science.gov (United States)

    Shi, Changbin; Shenkar, Robert; Zeineddine, Hussein A; Girard, Romuald; Fam, Maged D; Austin, Cecilia; Moore, Thomas; Lightle, Rhonda; Zhang, Lingjiao; Wu, Meijing; Cao, Ying; Gunel, Murat; Louvi, Angeliki; Rorrer, Autumn; Gallione, Carol; Marchuk, Douglas A; Awad, Issam A

    2016-06-01

    Cerebral cavernous malformations (CCMs) are relatively common vascular malformations, characterized by increased Rho kinase (ROCK) activity, vascular hyper-permeability and the presence of blood degradation products including non-heme iron. Previous studies revealed robust inflammatory cell infiltration, selective synthesis of IgG, in situ antigen driven B-cell clonal expansion, and deposition of immune complexes and complement proteins within CCM lesions. We aimed to evaluate the impact of suppressing the immune response on the formation and maturation of CCM lesions, as well as lesional iron deposition and ROCK activity. Two murine models of heterozygous Ccm3 (Pdcd10), which spontaneously develop CCM lesions with severe and milder phenotypes, were either untreated or received anti-mouse BR3 to deplete B cells. Brains from anti-mouse BR3-treated mice exhibited significantly fewer mature CCM lesions and smaller lesions compared to untreated mice. B cell depletion halted the progression of lesions into mature stage 2 lesions but did not prevent their genesis. Non-heme iron deposition and ROCK activity was decreased in lesions of B cell depleted mice. This represents the first report of the therapeutic benefit of B-cell depletion in the development and progression of CCMs, and provides a proof of principle that B cells play a critical role in CCM lesion genesis and maturation. These findings add biologics to the list of potential therapeutic agents for CCM disease. Future studies would characterize the putative antigenic trigger and further define the mechanism of immune response in the lesions.

  9. Vascular permeability and iron deposition biomarkers in longitudinal follow-up of cerebral cavernous malformations.

    Science.gov (United States)

    Girard, Romuald; Fam, Maged D; Zeineddine, Hussein A; Tan, Huan; Mikati, Abdul Ghani; Shi, Changbin; Jesselson, Michael; Shenkar, Robert; Wu, Meijing; Cao, Ying; Hobson, Nicholas; Larsson, Henrik B W; Christoforidis, Gregory A; Awad, Issam A

    2016-08-05

    OBJECTIVE Vascular permeability and iron leakage are central features of cerebral cavernous malformation (CCM) pathogenesis. The authors aimed to correlate prospective clinical behavior of CCM lesions with longitudinal changes in biomarkers of dynamic contrast-enhanced quantitative permeability (DCEQP) and quantitative susceptibility mapping (QSM) assessed by MRI. METHODS Forty-six patients with CCMs underwent 2 or more permeability and/or susceptibility studies in conjunction with baseline and follow-up imaging and clinical surveillance during a mean 12.05 months of follow-up (range 2.4-31.27 months). Based on clinical and imaging features, cases/lesions were classified as stable, unstable, or recovering. Associated and predictive changes in quantitative permeability and susceptibility were investigated. RESULTS Lesional mean permeability and QSM values were not significantly different in stable versus unstable lesions at baseline. Mean lesional permeability in unstable CCMs with lesional bleeding or growth increased significantly (+85.9% change; p = 0.005), while mean permeability in stable and recovering lesions did not significantly change. Mean lesional QSM values significantly increased in unstable lesions (+44.1% change; p = 0.01), decreased slightly with statistical significance in stable lesions (-3.2% change; p = 0.003), and did not significantly change in recovering lesions. Familial cases developing new lesions during the follow-up period showed a higher background brain permeability at baseline (p = 0.001), as well as higher regional permeability (p = 0.003) in the area that would later develop a new lesion as compared with the homologous contralateral brain region. CONCLUSIONS In vivo assessment of vascular permeability and iron deposition on MRI can serve as objective and quantifiable biomarkers of disease activity in CCMs. This may be applied in natural history studies and may help calibrate clinical trials. The 2 techniques are likely applicable in

  10. A locus for cerebral cavernous malformations maps to chromosome 7q in two families

    Energy Technology Data Exchange (ETDEWEB)

    Marchuk, D.A.; Gallione, C.J. [Duke Univ. Medical Center, Durham, NC (United States); Morrison, L.A.; Davis, L.E.; Clericuzio, C.L. [Univ. of New Mexico School of Medicine, Albuquerque, NM (United States)] [and others

    1995-07-20

    Cavernous malformations (angiomas) affecting the central nervous system and retina can be inherited in autosomal dominant pattern (OMIM 116860). These vascular lesions may remain clinically silent or lead to a number of neurological symptoms including seizure, intracranial hemorrhage, focal neurological deficit, and migraine. We have mapped a gene for this disorder in two families, one of Italian-American origin and one of Mexican-American origin, to markers on proximal 7q, with a combined maximum lod score of 3.92 ({theta} of zero) with marker D7S479. Haplotype analysis of these families places the locus between markers D7S502 proximally and D7S515 distally, an interval of approximately 41 cM. The location distinguishes this disorder from an autosomal dominant vascular malformation syndrome where lesions are primarily cutaneous and that maps to 9p21. 16 refs., 3 figs., 1 tab.

  11. A novel mouse model of cerebral cavernous malformations based on the two-hit mutation hypothesis recapitulates the human disease.

    Science.gov (United States)

    McDonald, David A; Shenkar, Robert; Shi, Changbin; Stockton, Rebecca A; Akers, Amy L; Kucherlapati, Melanie H; Kucherlapati, Raju; Brainer, James; Ginsberg, Mark H; Awad, Issam A; Marchuk, Douglas A

    2011-01-15

    Cerebral cavernous malformations (CCMs) are vascular lesions of the central nervous system appearing as multicavernous, blood-filled capillaries, leading to headache, seizure and hemorrhagic stroke. CCM occurs either sporadically or as an autosomal dominant disorder caused by germline mutation of one of the three genes: CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10. Surgically resected human CCM lesions have provided molecular and immunohistochemical evidence for a two-hit (germline plus somatic) mutation mechanism. In contrast to the equivalent human genotype, mice heterozygous for a Ccm1- or Ccm2-null allele do not develop CCM lesions. Based on the two-hit hypothesis, we attempted to improve the penetrance of the model by crossing Ccm1 and Ccm2 heterozygotes into a mismatch repair-deficient Msh2(-/-) background. Ccm1(+/-)Msh2(-/-) mice exhibit CCM lesions with high penetrance as shown by magnetic resonance imaging and histology. Significantly, the CCM lesions range in size from early-stage, isolated caverns to large, multicavernous lesions. A subset of endothelial cells within the CCM lesions revealed somatic loss of CCM protein staining, supporting the two-hit mutation mechanism. The late-stage CCM lesions displayed many of the characteristics of human CCM lesions, including hemosiderin deposits, immune cell infiltration, increased endothelial cell proliferation and increased Rho-kinase activity. Some of these characteristics were also seen, but to a lesser extent, in early-stage lesions. Tight junctions were maintained between CCM lesion endothelial cells, but gaps were evident between endothelial cells and basement membrane was defective. In contrast, the Ccm2(+/-)Msh2(-/-) mice lacked cerebrovascular lesions. The CCM1 mouse model provides an in vivo tool to investigate CCM pathogenesis and new therapies.

  12. Features of a Chinese family with cerebral cavernous malformation induced by a novel CCM1 gene mutation

    Institute of Scientific and Technical Information of China (English)

    WANG Xue; LIU Xue-wu; Nora Lee; LIU Qi-ji; LI Wen-na; HAN Tao; WEI Kun-kun

    2013-01-01

    Background Familial cerebral cavernous malformations (CCMs),characterized by hemorrhagic stroke,recurrent headache and epilepsy,are congenital vascular anomalies of the central nervous system.Familial CCMs is an autosomal dominant inherited disorder and three CCM genes have been identified.We report a Chinese family with CCMs and intend to explore clinical,pathological,magnetic resonance imaging (MRI) features and pathogenic gene mutation of this family.Methods Totally 25 family members underwent brain MRI examination and clinical check.Two patients with surgical indications had surgical treatment and the specimens were subjected to histopathological and microstructural examination.In addition,polymerase chain reaction (PCR) and direct sequencing were performed with genomic DNA extracted from 25 family members' blood samples for mutation detection.Results Brain MRI identified abnormal results in seven family members.All of them had multiple intracranial lesions and four cases had skin cavernous hemangioma.T2-weighted sequence showed that the lesions were typically characterized by an area of mixed signal intensity.Gradient-echo (GRE) sequence was more sensitive to find microcavernous hemangiomas.There was a wide range in the clinical manifestations as well as the age of onset in the family.The youngest patient was an 8-year-old boy with least intracranial lesions.Histopathological and microstructural examination showed that CCMs were typically discrete multi-sublobes of berry-like lesions,with hemorrhage in various stages of illness evolution.They were formed by abnormally enlarged sinusoids and the thin basement membranes.A novel T deletion mutation in exon 14 of CCM1 gene was identified by mutation detection in the seven patients.But unaffected members and healthy controls did not carry this mutation.Conclusions The clinical manifestations were heterogenic within this family.We identified a novel mutation (c.1396delT) was the disease-causing mutation for this

  13. The Role of Hemosiderin Excision in Seizure Outcome in Cerebral Cavernous Malformation Surgery: A Systematic Review and Meta-Analysis.

    Directory of Open Access Journals (Sweden)

    Di Ruan

    Full Text Available Whether the excision of hemosiderin surrounding cerebral cavernous malformations (CCMs is necessary to achieve a seizure-free result has been the subject of debate. Here, we report a systematic review of related literature up to Jan 1, 2015 including 594 patients to assess the effect of hemosiderin excision on seizure outcome in patients with CCMs by meta-analysis.Ten studies comparing extended hemosiderin excision with only lesion resection were identified by searching the English-language literature. Meta-analyses, subgroup analyses and sensitivity analysis were conducted to determine the association between hemosiderin excision and seizure outcome after surgery.Seizure outcome was significantly improved in the patients who underwent an extended excision of the surrounding hemosiderin (OR, 0.62; 95% CI: 0.42-0.91; P = 0.01. In subgroup analysis, studies from Asia (OR, 0.42; 95% CI: 0.25-0.71; P = 0.001, male-majority (female ratio 1 year before surgery (OR, 0.43; 95% CI: 0.22-0.84; P = 0.01, lesion diameter > 2 cm (OR, 0.41; 95% CI: 0.19-0.87; P = 0.02 and short-term (< 3 years follow-up (OR, 0.48; 95% CI: 0.29-0.80; P = 0.005 tended to correlate with a significantly favorable outcome.Patients who underwent extended surrounding hemosiderin excision could exhibit significantly improved seizure outcomes compared to patients without hemosiderin excision. However, further well-designed prospective multiple-center RCT studies are still needed.

  14. Variable expression of cerebral cavernous malformations in carriers of a premature termination codon in exon 17 of the Krit1 gene

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    Gamero Miguel A

    2003-07-01

    Full Text Available Abstract Background Cerebral cavernous malformations (CCM present as either sporadic or autosomal dominant conditions with incomplete penetrance of symptoms. Differences in genetic and environmental factors might be minimized among first-degree relatives. We therefore studied clinical expression in a family with several affected members. Methods We studied a three-generation family with the onset of CCM as a cerebral haemorrhage in the younger (four-year-old sibling. Identification and enumeration of CCMs were performed in T2-weighted or gradient-echo MRIs of the whole brains. Genetic analysis comprised SCCP, sequencing and restriction polymorphism of the Krit1 gene in the proband and at risk relatives. Results The phenotypes of cerebral cavernous malformations (CCMs in carriers of Krit1 mutations were very variable. We identified a novel frameshift mutation caused by a 1902A insertion in exon 17 of the Krit1 gene, which leads to a premature TAA triplet and predicts the truncating phenotype Y634X. A very striking finding was the absence of both clinical symptoms and CCMs in the eldest sibling harbouring the 1902insA. Conclusions Patients in this family, harbouring the same mutation, illustrate the very variable clinical and radiological expression of a Krit1 mutation. The early and critical onset in the proband contrasts with minor clinical findings in affected relatives. This consideration is important in genetic counselling.

  15. Cerebral Cavernous Malformations (CCM)

    Science.gov (United States)

    ... all children with HHT should have the same screening for lung AVMs recommended for adults. Children can be safely treated with embolization. ? What are the symptoms or signs of HHT in the gastrointestinal tract (stomach and intestines)? Unexplained anemia or low blood count, ...

  16. Brainstem cavernous malformations: a review with two case reports Malformações cavernosas do tronco cerebral: uma revisão com relato de dois casos

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    Adolfo Ramírez-Zamora

    2009-09-01

    Full Text Available Central nervous system (CNS cavernous malformations (CMs are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.Malformações cavernosas (MFC do sistema nervoso central são malformações do desenvolvimento do leito vascular com múltiplas apresentações clínicas devido a sua natureza dinâmica. Apresentamos dois casos de malformações cavernosas do tronco cerebral: o primeiro após radioterapia e o segundo em paciente grávida com hemorragia intracraniana sintomática. MFC são responsáveis por cerca de 8-15% de todas as malformações vasculares. Embora tradicionalmente sejam genéticas, as MFC podem também ser adquiridas, particularmente após radioterapia. As manifestações clínicas são variáveis e o tratamento cirúrgico deve ser considerado para pacientes com quadros neurológicos progressivos.

  17. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge;

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  18. Lesions from patients with sporadic cerebral cavernous malformations harbor somatic mutations in the CCM genes: evidence for a common biochemical pathway for CCM pathogenesis.

    Science.gov (United States)

    McDonald, David A; Shi, Changbin; Shenkar, Robert; Gallione, Carol J; Akers, Amy L; Li, Stephanie; De Castro, Nicholas; Berg, Michel J; Corcoran, David L; Awad, Issam A; Marchuk, Douglas A

    2014-08-15

    Cerebral cavernous malformations (CCMs) are vascular lesions affecting the central nervous system. CCM occurs either sporadically or in an inherited, autosomal dominant manner. Constitutional (germline) mutations in any of three genes, KRIT1, CCM2 and PDCD10, can cause the inherited form. Analysis of CCM lesions from inherited cases revealed biallelic somatic mutations, indicating that CCM follows a Knudsonian two-hit mutation mechanism. It is still unknown, however, if the sporadic cases of CCM also follow this genetic mechanism. We extracted DNA from 11 surgically excised lesions from sporadic CCM patients, and sequenced the three CCM genes in each specimen using a next-generation sequencing approach. Four sporadic CCM lesion samples (36%) were found to contain novel somatic mutations. Three of the lesions contained a single somatic mutation, and one lesion contained two biallelic somatic mutations. Herein, we also describe evidence of somatic mosaicism in a patient presenting with over 130 CCM lesions localized to one hemisphere of the brain. Finally, in a lesion regrowth sample, we found that the regrown CCM lesion contained the same somatic mutation as the original lesion. Together, these data bolster the idea that all forms of CCM have a genetic underpinning of the two-hit mutation mechanism in the known CCM genes. Recent studies have found aberrant Rho kinase activation in inherited CCM pathogenesis, and we present evidence that this pathway is activated in sporadic CCM patients. These results suggest that all CCM patients, including those with the more common sporadic form, are potentially amenable to the same therapy.

  19. Radiological features of childhood giant cavernous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Ozgen, Burce; Senocak, Efsun; Oguz, Kader K. [Hacettepe University, Department of Radiology, Faculty of Medicine, Ankara (Turkey); Soylemezoglu, Figen [Hacettepe University, Department of Pathology, School of Medicine, Ankara (Turkey); Akalan, Nejat [Hacettepe University, Department of Neurosurgery, School of Medicine, Ankara (Turkey)

    2011-04-15

    Giant cavernous malformations (GCM) are very large, low-flow vascular malformations, which usually have atypical imaging features and are commonly misdiagnosed preoperatively as neoplasms or vascular malformations. These lesions have mostly been reported in children. As cavernomas show different features in children compared to adults, we evaluated the imaging features of pediatric GCMs in order to help in the preoperative diagnosis of these malformations. Brain MR studies of nine children (mean age of 4 years; 8 months-9 years) with biopsy-proven GCM were retrospectively evaluated. We defined GCMs as cavernomas of {>=}4 cm. Lesions were evaluated regarding their size, location, signal characteristics, general appearance (uni/multilocular) as well as regarding the presence of mass effect, edema, and fluid-fluid levels and were classified according to the Mottolese classification of pediatric cavernomas. Lesion locations were parietal (n = 5), frontal (n = 2), temporal, and intraventricular. Seven lesions were in the periventricular region (with five in the periatrial region). Six patients had T1 hyperintense multilobulated lesions with ''bubbles of blood'' appearance and three patients had heterogeneous lesions with reticular core. All lesions had mass effect, edema (marked in four cases), and peripheral hemosiderin rim. Fluid-fluid levels were also common (n = 7). Most of our lesions (six of nine) were classified as type IIIA, two as type IIIC, and one as type IA. In children, a GCM should be considered in case of very large hemorrhagic intra-axial mass with ''bubbles of blood'' multicystic appearance, surrounding hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect, especially in the periatrial location. (orig.)

  20. Cerebral malformations without antenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Girard, Nadine J. [Diagnostic Neuroradiology, Hopital Timone, Marseille (France)

    2010-06-15

    Cerebral malformations are usually described following the different steps in development. Disorders of neurulation (dysraphisms), or diverticulation (holoprosencephalies and posterior fossa cysts), and total commissural agenesis are usually diagnosed in utero. In contrast, disorders of histogenesis (proliferation-differentiation, migration, organization) are usually discovered in infants and children. The principal clinical symptoms that may be a clue to cerebral malformation include congenital hemiparesis, epilepsy and mental or psychomotor retardation. MRI is the imaging method of choice to assess cerebral malformations. (orig.)

  1. Arteriovenous Malformation in Temporal Lobe Presenting as Contralateral Ocular Symptoms Mimicking Carotid-Cavernous Fistula

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    Fadzillah Mohd-Tahir

    2013-01-01

    Full Text Available Aim. To report a rare case of arteriovenous malformation in temporal lobe presenting as contralateral orbital symptoms mimicking carotid-cavernous fistula. Method. Interventional case report. Results. A 31-year-old Malay gentleman presented with 2-month history of painful progressive exophthalmos of his left eye associated with recurrent headache, diplopia, and reduced vision. Ocular examination revealed congestive nonpulsating 7 mm exophthalmos of the left eye with no restriction of movements in all direction. There was diplopia in left lateral gaze. Left IOP was elevated at 29 mmHg. Left eye retinal vessels were slightly dilated and tortuous. CT scan was performed and showed right temporal arteriovenous malformation with a nidus of 3.8 cm × 2.5 cm with right middle cerebral artery as feeding artery. There was dilated left superior ophthalmic vein of 0.9 mm in diameter with enlarged left cavernous sinus. MRA and carotid angiogram confirmed right temporal arteriovenous malformation with no carotid-cavernous fistula. Most of the intracranial drainage was via left cavernous sinus. His signs and symptoms dramatically improved following successful embolisation, completely resolved after one year. Conclusion. Intracranial arteriovenous malformation is rarely presented with primary ocular presentation. Early intervention would salvage the eyes and prevent patients from more disaster morbidity or fatality commonly due to intracranial haemorrhage.

  2. Treatment and Outcome of Epileptogenic Temporal Cavernous Malformations

    Institute of Scientific and Technical Information of China (English)

    Yong-Zhi Shan; Xiao-Tong Fan; Liang Meng; Yang An; Jian-Kun Xu; Guo-Guang Zhao

    2015-01-01

    Background:The aim of this study is to explore the treatment and outcome ofepileptogenic temporal lobe cavernous malformations (CMs).Methods:We analyzed retrospectively the profiles of 52 patients diagnosed as temporal lobe CMs associated with epilepsy.Among the 52 cases,11 underwent a direct resection of CM along with the adjacent zone of hemosiderin rim without electrocorticogram (ECoG) monitoring while the other 41 cases had operations under the guidance of ECoG.Forty-six patients were treated by lesionectomy + hemosiderin rim while the other six were treated by lesionectomy + hemosiderin rim along with extended epileptogenic zone resection.The locations of lesions,the duration of illness,the manifestation,the excision ranges and the outcomes of postoperative follow-up were analyzed,respectively.Results:All of the 52 patients were treated by microsurgery.There was no neurological deficit through the long-term follow-up.Outcomes of seizure control are as follows:42 patients (80.8%) belong to Engel Class Ⅰ,5 patients (9.6%) belong to Engel Class Ⅱ,3 patients (5.8%) belong to Engel Class Ⅲ and 2 patients (3.8%) belong to Engel Class Ⅳ.Conclusion:Patients with epilepsy caused by temporal CMs should be treated as early as possible.Resection of the lesion and the surrounding hemosiderin zone is necessary.Moreover,an extended excision of epileptogenic cortex or cerebral lobes is needed to achieve a better prognosis if the ECoG indicates the existence of an extra epilepsy onset origin outside the lesion itself.

  3. KRIT1 mutations in three Japanese pedigrees with hereditary cavernous malformation

    Science.gov (United States)

    Hirota, Kengo; Akagawa, Hiroyuki; Kikuchi, Asami; Oka, Hideki; Hino, Akihiko; Mitsuyama, Tetsuryu; Sasaki, Toshiyuki; Onda, Hideaki; Kawamata, Takakazu; Kasuya, Hidetoshi

    2016-01-01

    Cerebral cavernous malformation is a neurovascular abnormality that can cause seizures, focal neurological deficits and intracerebral hemorrhage. Familial forms of this condition are characterized by de novo formation of multiple lesions and are autosomal-dominantly inherited via CCM1/KRIT1, CCM2/MGC4607 and CCM3/PDCD10 mutations. We identified three truncating mutations in KRIT1 from three Japanese families with CCMs: a novel frameshift mutation, a known frameshift mutation and a known splice-site mutation that had not been previously analyzed for aberrant splicing. PMID:27766163

  4. A giant frontal cavernous malformation with review of literature

    Science.gov (United States)

    Sharma, Arvind; Mittal, Radhey Shyam

    2016-01-01

    Cavernous malformations (CMs) are vascular anomalies with dilated spaces called caverns. These spaces are lined by endothelial cells and collage and devoid of smooth muscle or intervening neural tissue, and filled with blood at various stages of stasis, thrombosis, organization, and calcification. Most CMs are relatively small in size but when they are large enough they can produce sing of mass effect and may simulate neoplastic, vascular, inflammatory pathology. Giant CM (size >6 cm) are very rare lesions and very few cases are reported in world literature. We are reporting such a rare case of a 16 year male. Our case is also unique in the sense that it is the largest reported CM in Indian population. PMID:27114662

  5. Electrocorticography-Guided Surgical Treatment of Solitary Supratentorial Cavernous Malformations with Secondary Epilepsy

    Institute of Scientific and Technical Information of China (English)

    Chao Wang; Chao You; Guo-qiang Han; Jun Wang; Yun-biao Xiong; Chuang-xi Liu

    2014-01-01

    Objective To evaluate the efficacy of electrocorticographic (ECoG) monitoring and the application of different surgical approaches in the surgical treatment of solitary supretentorial cavernous malformations with secondary epilepsy. Methods This study enrolled a consecutive series of 36 patients with solitary supratentorial cavernous malformations and secondary epilepsy who underwent surgery with intraoperative ECoG monitoring in the Department of Neurosurgery between January 2004 and January 2008. The patients were composed of 15 males and 21 females, aged between 8 and 52 years (mean age 27.3±2.8 years) at the time of surgery. Epilepsy history, the type of epilepsy at the presentation, lesion location, the incidence of residual epileptiform discharges, and postoperative outcomes were evaluated. Results Histopathological examination indicated cavernous malformations and hippocampal sclerosis in 36 and 5 cases, respectively. Neuronal degeneration, glial cell proliferation, and neurofibrillary tangles were found in all the resected cerebral tissues of extended lesionectomy of residual epileptic foci. Lesionectomy, anterior temporal lobectomy, anterior temporal lobectomy plus cortical thermocoagulation, extended lesionectomy, extended lesionectomy plus cortical thermocoagulation were performed in 4, 4, 1, 14, and 13 cases, respectively. Residual epileptiform discharges were captured in 9 out of the 14 patients who had additional cortical thermocoagulation. According to Engle class for postoperative outcomes, 27 cases were class I (75.00%), 5 were class II (13.89%), 2 were class III (5.56%), and 2 were class IV (5.56%), thus the total effective rate (class I+class II) was 88.89%. Neither of epilepsy history, the type of epilepsy, and the location of cavernous malformation was significantly related to outcomes (P>0.05). A significant relationship was found between the incidence of residual epileptiform discharges and outcomes (P=0.041). Conclusions Intraoperative ECo

  6. Cerebral malformations in Carpenter syndrome.

    Science.gov (United States)

    Taravath, S; Tonsgard, J H

    1993-01-01

    The inherited forms of craniosynostosis can be divided into 4 groups: isolated craniosynostosis, craniosynostosis with syndactyly, craniosynostosis with polydactyly and syndactyly, and craniosynostosis with other somatic abnormalities. Acrocephalopolysyndactyly or Carpenter syndrome consists of craniosynostosis, short fingers, soft tissue syndactyly, preaxial polydactyly, congenital heart disease, hypogenitalism, obesity, and umbilical hernia. As many as three-fourths of the patients have some degree of intellectual impairment. The etiology of mental retardation in this syndrome has not been explored. A patient is reported with the features of Carpenter syndrome who has profound developmental delay and cerebral malformations demonstrated by magnetic resonance imaging and computed tomography. Because mental retardation is not an invariable feature of this syndrome or other craniosynostosis syndromes, neuroradiologic examination may help in predicting the intellectual outcome in these patients.

  7. Endoscopic endonasal transclival resection of a ventral pontine cavernous malformation: technical case report.

    Science.gov (United States)

    Gómez-Amador, Juan Luis; Ortega-Porcayo, Luis Alberto; Palacios-Ortíz, Isaac Jair; Perdomo-Pantoja, Alexander; Nares-López, Felipe Eduardo; Vega-Alarcón, Alfredo

    2016-10-21

    Brainstem cavernous malformations are challenging due to the critical anatomy and potential surgical risks. Anterolateral, lateral, and dorsal surgical approaches provide limited ventral exposure of the brainstem. The authors present a case of a midline ventral pontine cavernous malformation resected through an endoscopic endonasal transclival approach based on minimal brainstem transection, negligible cranial nerve manipulation, and a straightforward trajectory. Technical and reconstruction technique advances in endoscopic endonasal skull base surgery provide a direct, safe, and effective corridor to the brainstem.

  8. Genetics Home Reference: cerebral cavernous malformation

    Science.gov (United States)

    ... Ibrahim R, Irthum B, Jacquet G, Lonjon M, Moreau JJ, Neau JP, Parker F, Tremoulet M, Tournier- ... Lapierre F, Loiseau H, Menei P, Mercier P, Moreau JJ, Nivelon-Chevallier A, Parker F, Redondo AM, ...

  9. Gamma knife radiosurgery for cerebral arteriovenous malformations.

    Science.gov (United States)

    Kemeny, A A; Radatz, M W R; Rowe, J G; Walton, L; Hampshire, A

    2004-01-01

    Since its introduction, gamma knife radiosurgery has become an important treatment modality for cerebral arteriovenous malformations. This paper is a brief overview of the technique used, of the clinical results achieved and of the experience gained in Sheffield.

  10. Effects of vascular endothelial growth factor on angiogenesis of the endothelial cells isolated from cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    TAN YuZhen; ZHAO Yao; WANG HaiJie; ZHOU LiangFu; MAO Ying; LIU Rui; SHU Jia; WANG YongFei

    2008-01-01

    Human cerebral cavernous malformation (CM) is a common vascular malformation of the central nervous system. We have investigated the biological characteristics of CM endothelial cells and the cellular and molecular mechanisms of CM angiogenesis to offer new insights into exploring effective measures for treatment of this disease. The endothelial cells were isolated from CM tissue masses dissected during operation and expanded in vitro. Expression of VEGFR-1 and VEGFR-2 was examined with immunocytochemical staining. Proliferation, migration and tube formation of CM endothelial cells were determined using MTT, wounding and transmigration assays, and three-dimensional collagen type Ⅰ gel respectively. The endothelial cells were successfully isolated from the tissue specimens of 25 CMs dissected without dipolar electrocoagulation. The cells show the general characteristics of the vascular endothelial cells. Expression of VEGFR-1 and VEGFR-2 on the cells is higher than that on the normal cerebral microvascular endothelial cells. After treatment with VEGF, numbers of the proliferated and migrated cells, the maximal distance of cell migration and the length and area of capillary-like struc-tures formed in the three-dimensional collagen gel increase significantly. These results demonstrate that expression of VEGFR-1 and VEGFR-2 on CM endothelial cells is up-regulated. By binding to re-ceptors, VEGF may activate the downstream signaling pathways and promote proliferation, migration and tube formation of CM endothelial cells. VEGF/VEGFR signaling pathways play important regulating roles in CM angiogenesis.

  11. Selection of the methods used in MR angiography with arteriovenous malformation, carotid cavernous fistula, moyamoya disease, and cerebral aneurysms; A comparison of 3-D phase-contrast MRA with 3-D time-of-flight MRA

    Energy Technology Data Exchange (ETDEWEB)

    Ikawa, Fusao; Uozumi, Tohru; Kuwabara, Satoshi (Hiroshima Univ. (Japan). School of Medicine) (and others)

    1993-02-01

    The methods used in MR angiography (MRA) can be roughly divided into two groups: time-of-flight methods (TOF methods) which make use of the influx effect of the flow, and the phase-contrast method of Dumoulin, Souza, and their collaborators (PC method), which utilizes phase differences induced by the flow. In this study, the selection of the MRA method was determined by a comparison of the three-dimensional (3-D) PC and the 3-D TOF method in normal and clinical cases. The tool used was SIGNA Advantage (1.5T) made by GE. The 3-D PC method was applied under a pulse sequence generated by gradient-recalled acquisition in the steady state (GRASS); the pulse sequence used for the 3-D TOF method was spoiled GRASS (SPGR) in a total of 28 cases. The following conclusions were reached: the TOF method should be used when aneurysms, arteriovenous malformations (AVM), and moyamoya disease have been diagnosed, and the PC method should be used when AVM and carotid cavernous fistulae (CCF) are to be evaluated in the flow and when CCFs have been diagnosed. (author).

  12. Direct orbital puncture of the cavernous sinus for the treatment of a carotid-cavernous dural AV fistula with a concomitant venous/lymphatic malformation.

    Science.gov (United States)

    Coumou, Adriaan D; van den Berg, René; Bot, Joost C; Beetsma, Daan B; Saeed, Peerooz

    2014-02-01

    A 37- year old male with a long history of a left orbital venous/lympathic malformation presented with ocular injection, increased proptosis and reduced left vision. Angiography demonstrated a carotid cavernous dural AV fistula combined with a concomitant venous/lymphatic malformation. After attempts at transvenous embolization, a direct uncomplicated transorbital puncture of the cavernous sinus via a lateral orbitotomy was performed with complete resolution of ocular symptoms.

  13. Endoscopic approaches to brainstem cavernous malformations: Case series and review of the literature

    Directory of Open Access Journals (Sweden)

    Nikhil R Nayak

    2015-01-01

    Conclusion: The endoscope is a promising adjunct to the neurosurgeon′s ability to approach difficult to access brainstem cavernous malformations. It allows the surgeon to achieve well-illuminated, panoramic views, and by combining approaches, can provide minimally invasive access to most regions of the brainstem.

  14. Gamma Knife treatment for cerebral arteriovenous malformations.

    Science.gov (United States)

    Kemeny, Andras A; Radatz, Matthias W R; Rowe, Jeremy G; Walton, Lee; Vaughan, Paul

    2007-01-01

    One of the earliest indications for Gamma Knife treatment, radiosurgery for cerebral arteriovenous malformations, has stood the test of time. While initially only the ideal cases (small, compact nidus in a non-eloquent site) were chosen, increasingly larger, more complex AVMs were treated. Combination treatment with embolisation and surgery enables most lesions to be treated with success and remarkably low complication rate. This paper is a brief overview of the experience gained in Sheffield.

  15. 颅内发育性静脉异常伴海绵状血管畸形三例患者的临床及影像学特点%Clinical and imaging feature of 3 patients with combined developmental venous anomalies and cerebral cavernous malformations

    Institute of Scientific and Technical Information of China (English)

    刘建军; 熊葶; 李尊波; 李盛彧; 贾颐

    2012-01-01

    Objective To investigate the clinical,neuroradiologic characteristics and possible causes in 3 patients with combined developmental venous anomalies (DVAs) and cerebral cavernous malformations (CCM).Methods The clinical examination,magnetic resonance imaging (MRI) T1-weighted (T1 WI),T2-weighted (T2WI),susceptibility-weighted imaging (SWI) or T2 fast field echo (T2 FFE),contrast-enhanced MRI at 1.5 T field strength and digital substrate angiography were performed in 3 patients.Results Three patients presented with the seizure,vertigo,and dizziness respectively.MRI findings of reticulated “popcorn like” lesion with complete hemosiden rim showed typical sign of CCM.DSA,contrast-enhanced MRI and MRI-SWI revealed the caput medusae of the medullary veins and collected veins which was drained into subcortical and deep venous system,which indicated DVAs in 3 patients.The angulated medullary veins and collected veins in approaching distal zone of CCM were observed.Conclusion DVAs can be combined with CCM.The angulated medullary veins and collected veins combined with CCM in same territory reveals that the angioarchitectural factors is a key factor in pathogenesis of cavernous malformation.%目的 探索颅内发育性静脉异常(DVAs)并发海绵状血管畸形(CCM)的临床及影像学特点.方法 对分别以癫痫、头昏、头晕发病的3例患者行头颅MRI磁敏感成像(MRI-SWI)或T2快速梯度回波(T2FFE)、钆喷酸葡胺增强、MRA及全脑血管造影检查.结果 MRI-SWI或T2FFE可见边缘低信号、中心为不同强度混杂信号的爆米花样或网格状的病灶,分布于额叶皮质及皮质下,提示CCM;在MRI增强及全脑血管造影中可见水母头样表现,提示有DVAs.在分析DVAs和CCM的关系中发现CCM病灶均发生在DVAs区域,可见引流静脉与CCM病灶呈成角弯曲.结论 DVAs可并发CCM,影像学可提供有特征性的改变,DVAs血管因素可能是CCM形成的原因之一.

  16. Unique double recurrence of cerebral arteriovenous malformation.

    Science.gov (United States)

    Nagm, Alhusain; Horiuchi, Tetsuyoshi; Ichinose, Shunsuke; Hongo, Kazuhiro

    2015-09-01

    Surgically treated patients with arteriovenous malformations (AVMs) are considered cured when the postoperative angiogram proves complete resection. However, despite no residual nidus or early draining vein on postoperative angiogram, rare instances of AVM recurrence have been reported in adults. In this paper, the authors present a case of a 24-year-old woman with asymptomatic double recurrence of her cerebral AVM after angiographically proven complete resection. To the authors' knowledge, this patient represents the first case with double de novo asymptomatic recurrence of Spetzler-Martin grade I AVM. Also, she represents the first case with unique AVM criteria in each recurrence.

  17. Tailored keyhole surgery for basal ganglia cavernous malformation with preoperative three-dimensional pyramidal tracts assessment and intraoperative electrophysiological monitoring

    Institute of Scientific and Technical Information of China (English)

    Kai Quan; Geng Xu; Fan Zhao; Wei Zhu

    2016-01-01

    Background:Accurately mapping the pyramidal tracts preoperatively and intraoperatively is the primary concern when operating on cavernous malformations (CMS) in the basal ganglia.We have conducted new methods for preoperative planning and have tailored lesion resection to prevent the damage of pyramidal tracts.Patients and methods:Eleven patients harboring cavernous malformations in basal ganglia were treated surgically from April 2008 to January 2015.Surgical planning was based on three-dimensional diffusion tensor pyramidal tractography and Virtual Reality system.Intraoperative detecting of pyramidal tracts with subcortical stimulation mapping and motor evoked potential monitoring were performed.The extent of resection and postoperative neurological function were assessed in each case.Results:Total removal of the cavernous malformations were achieved in each case.Four of the total eleven cases presented temporary neurological deficits,including one occurrence of hemiparesis and three occurrences of hemianesthesia.No permanent neurological deficit was developed in this series of cases.Conclusion:Three-dimensional diffusion tensor pyramidal tractography is quite helpful for preoperative planning of basal ganglia cavernous malformations,especially in choosing a suitable surgical approach.Intraoperative detection of pyramidal tracts with subcortical stimulation mapping and motor evoked potential monitoring play important roles in preventing damage to pyramidal tracts during lesion resection.

  18. A retrospective review of cases preoperatively diagnosed by radiologic imaging as cavernous venous malformations.

    Science.gov (United States)

    Jayaram, Anupam; Cohen, Liza M; Lissner, Gary S; Karagianis, Achilles G

    2017-04-03

    The purpose of this study is to examine orbital lesions identified on preoperative radiologic imaging as cavernous venous malformations (CVMs), identify their imaging characteristics, and determine if these may help differentiate CVMs from other intraorbital masses. An IRB-approved retrospective chart review over 30 years was undertaken identifying lesions "consistent with cavernous hemangioma" on radiologic studies, which were subsequently surgically resected with a tissue diagnosis. All radiologic images (CT and MRI) obtained preoperatively were re-reviewed by a single masked neuroradiologist. The pattern of contrast enhancement on sequential MRI views was used to determine whether the enhancing characteristics helped identify CVMs compared to other intraorbital masses. Fifty-seven orbital lesions consistent with a CVM were identified on imaging. Fourteen (25%) of them were resected, of which nine (64%) were found to be CVMs on pathologic examination. Five (36%) were found to be a different lesion, most commonly schwannoma (21%). On imaging, CVMs tended to display heterogeneous progressive enhancement, whereas other tumors, in particular schwannomas, enhanced at their maximum level immediately. Based on these characteristics, on re-review, the masked neuroradiologist was able to differentiate a CVM versus other tumors for all 14 imaging cases. This study suggests that examining the pattern of contrast enhancement may help to correctly differentiate a CVM from other isolated, encapsulated orbital lesions on CT/MR imaging.

  19. Quantitative Susceptibility Mapping and Dynamic Contrast Enhanced Quantitative Perfusion in Cerebral Cavernous Angiomas

    Science.gov (United States)

    Mikati, Abdul Ghani; Tan, Huan; Shenkar, Robert; Li, Luying; Zhang, Lingjiao; Guo, Xiaodong; Shi, Changbin; Liu, Tian; Wang, Yi; Shah, Akash; Edelman, Robert; Christoforidis, Gregory; Awad, Issam

    2015-01-01

    Background Hyperpermeability and iron deposition are two central pathophysiological phenomena in human cerebral cavernous malformation (CCM) disease. Here we used two novel magnetic resonance imaging (MRI) techniques to establish a relationship between these phenomena. Methods Subjects with CCM disease (4 sporadic and 18 familial) underwent MRI imaging using the Dynamic Contrast Enhanced Quantitative Perfusion (DCEQP) and Quantitative Susceptibility Mapping (QSM) techniques that measure hemodynamic factors of vessel leak and iron deposition respectively, previously demonstrated in CCM disease. Regions of interest encompassing the CCM lesions were analyzed using these techniques Results Susceptibility measured by QSM was positively correlated with permeability of lesions measured using DCEQP (r=0.49, p=<0.0001). The correlation was not affected by factors including familial predisposition, lesion volume, the contrast agent and the use of statin medication. Susceptibility was correlated with lesional blood volume (r=0.4, p=0.0001), but not with lesional blood flow. Conclusion The correlation between QSM and DCEQP suggests that the phenomena of permeability and iron deposition are related in CCM; hence “more leaky lesions” also manifest a more cumulative iron burden. These techniques might be used as biomarkers to monitor the course of this disease and the effect of therapy. PMID:24302484

  20. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    NARCIS (Netherlands)

    de Wit, Olga A; den Dunnen, Wilfred Fa; Sollie, Krystyne M; Muñoz, Rosa Iris; Meiners, Linda C; Brouwer, Oebele F; Rodríguez, Esteban M; Sival, Deborah A

    2008-01-01

    BACKGROUND: Fetal spina bifida aperta (SBA) is characterized by a spinal meningomyelocele (MMC) and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denud

  1. Pathogenesis of cerebral malformations in human fetuses with meningomyelocele

    Directory of Open Access Journals (Sweden)

    Brouwer Oebele F

    2008-03-01

    Full Text Available Abstract Background Fetal spina bifida aperta (SBA is characterized by a spinal meningomyelocele (MMC and associated with cerebral pathology, such as hydrocephalus and Chiari II malformation. In various animal models, it has been suggested that a loss of ventricular lining (neuroepithelial/ependymal denudation may trigger cerebral pathology. In fetuses with MMC, little is known about neuroepithelial/ependymal denudation and the initiating pathological events. The objective of this study was to investigate whether neuroepithelial/ependymal denudation occurs in human fetuses and neonates with MMC, and if so, whether it is associated with the onset of hydrocephalus. Methods Seven fetuses and 1 neonate (16–40 week gestational age, GA with MMC and 6 fetuses with normal cerebral development (22–41 week GA were included in the study. Identification of fetal MMC and clinical surveillance of fetal head circumference and ventricular width was performed by ultrasound (US. After birth, MMC was confirmed by histology. We characterized hydrocephalus by increased head circumference in association with ventriculomegaly. The median time interval between fetal cerebral ultrasound and fixing tissue for histology was four days. Results At 16 weeks GA, we observed neuroepithelial/ependymal denudation in the aqueduct and telencephalon together with sub-cortical heterotopias in absence of hydrocephalus and/or Chiari II malformation. At 21–34 weeks GA, we observed concurrence of aqueductal neuroepithelial/ependymal denudation and progenitor cell loss with the Chiari II malformation, whereas hydrocephalus was absent. At 37–40 weeks GA, neuroepithelial/ependymal denudation coincided with Chiari II malformation and hydrocephalus. Sub-arachnoidal fibrosis at the convexity was absent in all fetuses but present in the neonate. Conclusion In fetal SBA, neuroepithelial/ependymal denudation in the telencephalon and the aqueduct can occur before Chiari II malformation

  2. Evaluation of cerebral oximetry during endovascular treatment of carotid-cavernous fistula

    Science.gov (United States)

    Dujovny, Manuel; Misra, Mukesh; Alp, M. Serdar; Debrun, Gerard; Charbel, F. T.; Aletich, Victor; Ausman, James I.

    1997-08-01

    Endovascular treatment of carotid cavernous fistula is done routinely in our institution. We have been monitoring these patients with transcranial cerebral oximetry. The transcranial cerebral oximeter is a reliable, low-cost, non-invasive device that provides real-time evaluation of regional brain oxygen saturation during and after endovascular treatment of cerebrovascular diseases. We used the INVOS 3100A (Somanetics, Troy, MI) in our study. We discuss seven patients with carotid-cavernous fistulas treated by endovascular balloon occlusion, each monitored continuously before, during, and after the procedure with transcranial cerebral oximetry. The cerebral oxygen saturation depicted was directly related to the side of the venous drainage of the fistula, with the brain oxygen saturation 15 - 20% higher on the side of the venous drainage. Following endovascular occlusion of the fistula, oxygen saturation gradually became equal on both sides. In our patients treated for carotid-cavernous fistula, we evaluated the sensitivity and usefulness of cerebral oximetry as an important non-invasive monitoring tool for the endovascular treatment of carotid-cavernous fistula.

  3. [Macro- and microscopic systematization of cerebral cortex malformations in children].

    Science.gov (United States)

    Milovanov, A P; Milovanova, O A

    2011-01-01

    For the first time in pediatric pathologicoanatomic practice the complete systematization of cerebral cortex malformations is represented. Organ, macroscopic forms: microencephaly, macroencephaly, micropolygyria, pachygyria, schizencephaly, porencephaly, lissencephaly. Histic microdysgenesis of cortex: type I includes isolated abnormalities such as radial (IA) and tangential (I B) subtypes of cortical dislamination; type II includes sublocal cortical dislamination with immature dysmorphic neurons (II A) and balloon cells (II B); type III are the combination focal cortical dysplasia with tuberous sclerosis of the hippocampus (III A), tumors (III B) and malformations of vessels, traumatic and hypoxic disorders (III C). Band heterotopias. Subependimal nodular heterotopias. Tuberous sclerosis. Cellular typification of cortical dysplasia: immature neurons and balloon cells.

  4. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    OpenAIRE

    Andrew Conger; Charles Kulwin; Lawton, Michael T; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Cerebral arteriovenous malformations (AVMs) can be a heterogeneous pathological entity whose management requires a complex decision-making process due to the risks associated with their treatment and natural history. Despite the recently published conclusions of the aborted Randomized Trial of Brain Unruptured AVMs (ARUBA) trial, the authors of this article believe multimodality intervention in general and microsurgical resection in particular continue to play a major role in the ...

  5. Pediatric Stroke: The Importance of Cerebral Arteriopathy and Vascular Malformations

    OpenAIRE

    Beslow, Lauren A.; Jordan, Lori C

    2010-01-01

    Stroke is an important cause of neurologic morbidity in childhood. Population-based estimates of the annual incidence of childhood stroke range from 2 to 13 per 100,000 children. This article will review recent literature on both hemorrhagic and ischemic stroke in children with a focus on cerebral arteriopathy and vascular malformations as stroke risk factors. Additional risk factors include congenital heart disease, sickle cell disease, and hematologic abnormalities among others. Outcomes ar...

  6. Pre- and postoperative changes of regional cortical cerebral blood flow in patients with cerebral arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    石广志; 赵继宗; 王硕; 王永刚; 陆铮

    2003-01-01

    Objective To investigate pre- and postoperative changes of regional cerebral cortical blood flow in patients with cerebral arteriovenous malformation. Method Twenty-two adult patients with arteriovenous malformation(AVM) were recruited into this study at Beijing Tiantan Hospital from September 2001 to May 2002. Eight patients had giant cerebral AVM and the other 14 had a small one. Cortical cerebral blood flow (CBF) was measured by laser Doppler flowmetry (LDF) before and after AVM resections. After surgery, the probe of LDF was implanted adjacent to the area of AVM and monitored for 24 hours.Results CBF increased significantly after the resection in all patients regardless of AVM size. In patients with small AVM, CBF returned to the baseline level within 4 hours, but in patients with giant AVM, CBF remained high even after 24 hours.Conclusions Monitoring CBF is helpful to understand pre- and postoperative changes of regional cortical CBF in patients with cerebral AVM.

  7. Coexistence of a single cerebral arteriovenous malformation and spinal arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    Wang Yabing

    2009-01-01

    Full Text Available The coexistence of a cerebral and a spinal arteriovenous malformation (AVM together is extremely rare. We present a 31-year-old woman, who suffered from severe root pains in the left upper extremity. Magnetic resonance imaging (MRI revealed the abnormal vessels in the left occipital lobe and upper cervical segment of spinal cord. Cerebral angiography and spinal angiogram revealed two AVMs: One was in the right occipital lobe and the other was located in the C1-C2 segments of cervical cord. She had no other vascular lesions, and nor did her other family members. As the primary problem in her was left upper extremity root pains, which we considered was related to the spinal AVM, the first therapeutic treatment was focused on spinal AVM. The cerebral AVM of the right occipital lobe was surgically resected after part embolization.

  8. Magnetic resonance imaging of a cerebral cavernous haemangioma in a dog : clinical communication

    Directory of Open Access Journals (Sweden)

    J.P. Schoeman

    2002-07-01

    Full Text Available A 13-month-old, neutered, male Golden retriever presented with seizures and progressive depression. Clinical and neurological assessment was consistent with severe intracranial disease. The neurological condition progressively deteriorated and magnetic resonance imaging (MRI revealed the presence of a large, contrast-enhancing, space-occupying mass in the right cerebral hemisphere. Therapy with corticosteroids, mannitol and furosemide ameliorated the signs of depression and ataxia, but the owner elected euthanasia after 1 week. Post mortem examination of the brain confirmed the presence of a large haemorrhagic lesion in the right olfactory lobe, the histopathological appearance of which was consistent with cerebral cavernous haemangioma. This is the 1st case describing the MRI appearance of a cavernous haemangioma of the cerebrum in the veterinary literature.

  9. Long-Term Outcome of Endonasal Transsphenoidal Approach for the Treatment of Pontine Cavernous Malformation: Case Report with 11 Years of Follow-Up.

    Science.gov (United States)

    Banczerowski, Péter; Czigléczki, Gábor; Gádor, Ildikó; Nyáry, István

    2016-05-01

    Ventromedial localized cavernous malformations in the pons pose a difficult problem because of their surgical access and the high risk of deleterious consequences due to damage of the surrounding tissues. We report an endonasal transsphenoidal approach for the treatment of ventromedial pontine cavernomas that also follows principles of optimal access known as the "two-point method" for the resection of cavernous malformations. A 31-year-old woman presented with sudden left hemiparesis, nausea, and headache. Radiologic findings demonstrated a ventral pontine cavernous malformation in the midline slightly extending to the right, together with signs of acute hemorrhage. Surgical intervention was performed, and the cavernoma was completely resected via a transnasal transsphenoidal approach. Cerebrospinal fluid (CSF) rhinorrhea that ensued postoperatively needed an additional reconstructive surgery using the same approach. No further CSF leakage was evident, and an 11-year follow-up examination revealed neither signs of neurologic deficit nor recurrence of the resected pontine cavernoma. The long-term outcome proves the effectiveness and safety of this novel surgical route in the treatment of ventromedial cavernous malformations in the pons.

  10. Cavernous sinus syndrome due to rhino-orbital-cerebral mucormycosis

    Directory of Open Access Journals (Sweden)

    Vagner Moyses Vilela

    2013-06-01

    Full Text Available The present report describes the case of a 43-year-old diabetic patient with facial pain, protruding eyes, nasal congestion and decreased right vision (involvement of the ipsilateral cranial nerves III, IV and V. Computed tomography showed diffuse sinusitis at right, cribriform plate erosion and presence of a hypodense collection in the frontal lobe with peripheral enhancement. Magnetic resonance imaging confirmed the presence of a cerebral abscess. Samples were collected from the lesion, confirming hyphae compatible with mucormycosis.

  11. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    Science.gov (United States)

    Conger, Andrew; Kulwin, Charles; Lawton, Michael T.; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Cerebral arteriovenous malformations (AVMs) can be a heterogeneous pathological entity whose management requires a complex decision-making process due to the risks associated with their treatment and natural history. Despite the recently published conclusions of the aborted Randomized Trial of Brain Unruptured AVMs (ARUBA) trial, the authors of this article believe multimodality intervention in general and microsurgical resection in particular continue to play a major role in the management of carefully selected ruptured or unruptured AVMs. Methods: The authors provide an overview of their methodology for endovascular intervention and microsurgical resection and share their technical nuances for successful embolization and microsurgical resection of AVMs with special emphasis on complication avoidance. Results: The authors have achieved successful outcomes in embolization and resection of cerebral AVMs when using their methodology. Conclusions: These lesions are among the most technically difficult pathological entities handled by the cerebrovascular specialist, and an overview of technical concepts to help systematize this challenging and variable endeavor can improve the safety of their treatment. PMID:25883831

  12. Surgical strategy for cerebral arteriovenous malformation with acute hemorrhage

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: Presently, there have been craniocerebral operation, interventional embolization,stereotactic radiotherapy and other methods in treating cerebral arteriovenous malformation (AVM).However, the standard of different therapeutic regimens of cerebral AVM at the acute stage of hemorrhage has not been completely identified.OBJECTIVE: To observe the clinical characteristics and therapeutic effects of AVM at the acute stage of hemorrhage in patients, and to analyze corresponding therapeutic strategies.DESIGN: Non-randomized clinical observation.SETTING: Department of Neurosurgery, Foshan First People's Hospital, Sun Yat-sen University.PARTICIPANTS: Forty-six patients with cerebral AVM complicated by hemorrhage admitted to Department of Neurosurgery, Foshan First People's Hospital between January 1999 and December 2006,were involved in this study. All the patients were confirmed as cerebral AVM complicated by hemorrhage by brain angiography or/and postoperational pathology. The involved patients, 32 males and 14 females,averaged 25 years old, ranging from 6 to 62 years. Informed consents of therapeutic items were obtained from the relatives of all the patients.METHODS: ①On admission, skull CT and brain angiography were conducted in the involved subjects. ②The therapeutic method was confirmed according to the consciousness, hematoma region, hematoma volume,imageological results following comprehensive analysis: DSA examination was permitted to identify the size and position of abnormal vessel mass, and the distribution of feeding artery and draining vein. Craniocerebral operation was carried out as early as possible in patients with severe or progressive conscious disturbance, in which most of hematoma with obvious occupied effect or cerebral hernia was located in lobe of brain. The primary thing was to clean intracerebral hematoma for in time decompression. According to different situations, corresponding therapeutic measures were used for resecting abnormal

  13. Giant cavernous malformation in the ventrolateral midbrain with extension into the thalamus: a case report of a paramedian supracerebellar transtentorial approach.

    Science.gov (United States)

    Duan, Hongzhou; Hara, Yosuke; Goto, Tetsuya; Chiba, Akihiro; Hongo, Kazuhiro

    2016-08-01

    Cavernous malformations (CMs) of the midbrain and thalamus are relatively rare and particularly difficult to be resected given their location in eloquent tissues. Here, we report a case of a 14-year-old boy who experienced repeated and progressive right hemiparesis. Image examinations showed a gradually enlarged CM originated in the left ventrolateral midbrain extending to the left thalamus with repeated hemorrhage. By performing a paramedian supracerebellar transtentorial approach, the CM was totally removed, and the patient recovered without any new neurological deficit. The authors' experience suggests that this approach is eminent in treating giant lesions involving the ventrolateral midbrain and thalamus.

  14. Brainstem Cavernous Angioma

    Science.gov (United States)

    ... Elmaci I, Lee RR, Breiter SN, Rigamonti D. Dynamic nature of cavernous malformations: a prospective magnetic resonance ... intended for medical diagnostic use of any kind. | Website by Teramark MENU

  15. Spontaneous occlusion of cerebral arteriovenous malformation following partial embolization with Onyx.

    Science.gov (United States)

    Nas, Omer Fatih; Ozturk, Kerem; Gokalp, Gokhan; Hakyemez, Bahattin

    2017-02-01

    Management options for brain arteriovenous malformations (AVMs) are surgery, radiosurgery, and endovascular embolization. The aim of partial embolization in endovascular treatment is to make total resection possible. However, increased risk of bleeding in partial embolization creates some controversies about treatment options. Spontaneous total occlusion of cerebral AVMs following partial obliteration with embolization agents is a rarely seen condition. We present a case with an AVM vanishing from right posterior cerebral artery which spontaneously occluded following partial embolization with Onyx liquid agent.

  16. The role of SDF-1/CXCR4 in the vasculogenesis and remodeling of cerebral arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    Wang L

    2015-09-01

    Full Text Available Lingyan Wang,1 Shaolei Guo,2 Nu Zhang,2 Yuqian Tao,3 Heng Zhang,1 Tiewei Qi,2 Feng Liang,2 Zhengsong Huang2 1Department of Neurosurgery ICU, 2Department of Neurosurgery, 3Department of Neurology, The First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, People’s Republic of China Background: Cerebral arteriovenous malformation (AVM involves the vasculogenesis of cerebral blood vessels and can cause severe intracranial hemorrhage. Stromal cell-derived factor-1 (SDF-1 and its receptor, CXCR4, are believed to exert multiple physiological functions including angiogenesis. Thus, we investigated the role of SDF-1/CXCR4 in the vasculogenesis of cerebral AVM.Methods: Brain AVM lesions from surgical resections were analyzed for the expression of SDF-1, CXCR4, VEGF-A, and HIF-1 by using immunohistochemical staining. Flow cytometry was used to quantify the level of circulating endothelial progenitor cells (EPCs. Further, in an animal study, chronic cerebral hypoperfusion model rats were analyzed for the expression of SDF-1 and HIF-1. CXCR4 antagonist, AMD3100, was also used to detect its effects on cerebral vasculogenesis and SDF-1 expression.Results: Large amounts of CXCR4-positive CD45+ cells were found in brain AVM lesion blood vessel walls, which also have higher SDF-1 expression. Cerebral AVM patients also had higher level of EPCs and SDF-1. In chronic cerebral hypoperfusion rats, SDF-1, HIF-1, and CD45 expressions were elevated. The application of AMD3100 effectively suppressed angiogenesis and infiltration of CXCR4-positive CD45+ cells in hypoperfusion rats compared to controls.Conclusion: The SDF-1/CXCR4 axis plays an important role in the vasculogenesis and migration of inflammatory cells in cerebral AVM lesions, possibly via the recruitment of bone marrow EPCs. Keywords: cerebral arteriovenous malformation, SDF-1/CXCR4, chronic cerebral hypoperfusion, endothelial progenitor cells

  17. Moyamoya syndrome associated with γ knife surgery for cerebral arteriovenous malformation: case report.

    Science.gov (United States)

    Uozumi, Yoichi; Sumitomo, Masaki; Maruwaka, Mikio; Araki, Yoshio; Izumi, Takashi; Miyachi, Shigeru; Kato, Takenori; Hasegawa, Toshinori; Kida, Yoshihisa; Okamoto, Sho; Wakabayashi, Toshihiko

    2012-01-01

    A 30-year-old female developed moyamoya syndrome after gamma knife surgery (GKS) for cerebral arteriovenous malformation (AVM), and was treated with bypass surgery. She suffered from flittering scotoma, right transient hemianopsia, and headache for 1 year. Cerebral angiography revealed a Spetzler-Martin grade III AVM located in the left occipital lobe. After staged embolization, GKS was performed with a minimum dose of 20 Gy to the periphery of the nidus at the 50% isodose level of the maximum target dose. Gradual nidus regression was achieved, and the clinical symptoms disappeared completely. However, at 30 months after GKS, the patient suffered transient ischemic attack. Cerebral angiography showed left middle cerebral artery occlusion with moyamoya vessels. The patient underwent direct and indirect bypass surgery. After surgery, the patient was free from ischemic symptoms. Chronic inflammation and long-term changes in expression of cytokines and growth factors after GKS may have triggered this case.

  18. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

    Science.gov (United States)

    Moraes, Paulo L.; Dias, Rodrigo S.; Weltman, Eduardo; Giordani, Adelmo J.; Benabou, Salomon; Segreto, Helena R. C.; Segreto, Roberto A.

    2015-01-01

    Background: The aim of this study was to evaluate the clinical outcome of patients undergoing single-dose reirradiation using the Linear Accelerator (LINAC) for brain arteriovenous malformations (AVM). Methods: A retrospective study of 37 patients with brain AVM undergoing LINAC reirradiation between April 2003 and November 2011 was carried out. Patient characteristics, for example, gender, age, use of medications, and comorbidities; disease characteristics, for example, Spetzler–Martin grading system, location, volume, modified Pollock–Flickinger score; and treatment characteristics, for example, embolization, prescription dose, radiation dose–volume curves, and conformity index were analyzed. During the follow-up period, imaging studies were performed to evaluate changes after treatment and AVM cure. Complications, such as edema, rupture of the blood–brain barrier, and radionecrosis were classified as symptomatic and asymptomatic. Results: Twenty-seven patients underwent angiogram after reirradiation and the percentage of angiographic occlusion was 55.5%. In three patients without obliteration, AVM shrinkage made it possible to perform surgical resection with a 2/3 cure rate. A reduction in AVM nidus volume greater than 50% after the first procedure was shown to be the most important predictor of obliteration. Another factor associated with AVM cure was a prescription dose higher than 15.5 Gy in the first radiosurgery. Two patients had permanent neurologic deficits. Factors correlated with complications were the prescription dose and maximum dose in the first procedure. Conclusion: This study suggests that single-dose reirradiation is safe and feasible in partially occluded AVM. Reirradiation may not benefit candidates whose prescribed dose was lower than 15.5 Gy in the first procedure and initial AVM nidus volume did not decrease by more than 50% before reirradiation. PMID:26110078

  19. Outcome of cerebral arteriovenous malformations after linear accelerator reirradiation

    Directory of Open Access Journals (Sweden)

    Paulo L Moraes

    2015-01-01

    Full Text Available Background: The aim of this study was to evaluate the clinical outcome of patients undergoing single-dose reirradiation using the Linear Accelerator (LINAC for brain arteriovenous malformations (AVM. Methods: A retrospective study of 37 patients with brain AVM undergoing LINAC reirradiation between April 2003 and November 2011 was carried out. Patient characteristics, for example, gender, age, use of medications, and comorbidities; disease characteristics, for example, Spetzler-Martin grading system, location, volume, modified Pollock-Flickinger score; and treatment characteristics, for example, embolization, prescription dose, radiation dose-volume curves, and conformity index were analyzed. During the follow-up period, imaging studies were performed to evaluate changes after treatment and AVM cure. Complications, such as edema, rupture of the blood-brain barrier, and radionecrosis were classified as symptomatic and asymptomatic. Results: Twenty-seven patients underwent angiogram after reirradiation and the percentage of angiographic occlusion was 55.5%. In three patients without obliteration, AVM shrinkage made it possible to perform surgical resection with a 2/3 cure rate. A reduction in AVM nidus volume greater than 50% after the first procedure was shown to be the most important predictor of obliteration. Another factor associated with AVM cure was a prescription dose higher than 15.5 Gy in the first radiosurgery. Two patients had permanent neurologic deficits. Factors correlated with complications were the prescription dose and maximum dose in the first procedure. Conclusion: This study suggests that single-dose reirradiation is safe and feasible in partially occluded AVM. Reirradiation may not benefit candidates whose prescribed dose was lower than 15.5 Gy in the first procedure and initial AVM nidus volume did not decrease by more than 50% before reirradiation.

  20. Cerebral malformation induced by prenatal X-irradiation: an autoradiographic and Golgi study

    Energy Technology Data Exchange (ETDEWEB)

    Ferrer, I.; Xumetra, A.; Santamaria, J. (Neuropatologia, Depto. Anatomia Patologica, C.S. ' Principes de Espana' , Hospitalet de Llobregat, Barcelona (Spain))

    1984-01-01

    Brain malformations are produced after X-irradiation at different post-conceptional ages in the rat. Malformed cortical patterns result from abnormal organisation and capricious orientation of the neurons, while a radical migratory pattern of neuroblasts outwards to the cerebral cortex is preserved in animals irradiated on the fourteenth, sixteenth or eighteenth days of gestation. Migratory disturbances are restricted to the large subcortical ectopic masses found in rats irradiated on the fourteenth gestational day and to pyramidal ectopic nodules in the hippocampus in rats irradiated on the sixteenth gestational day. Subcortical ectopic masses develop from ectopic germinal rosettes and are formed by several types of cortical neuron distributed in a stereotyped pattern. The presence of large numbers of intrinsic, afferent and efferent connections are indicative of integrative functions of the subcortical masses.

  1. The Rare Association of Moyamoya Disease and Cerebral Arteriovenous Malformations: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Wu, Te Chang [Chi-Mei Foundation Hospital, Tainan (China); Guo, Wan Yuo; Wu, Hsiu Mei; Chang, Feng Chi; Shiau, Cheng Ying; Chung, Wen Yuh [Taipei Veterans General Hospital, Taipei (China)

    2008-07-15

    A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow- up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.

  2. Direct carotid cavernous fistula after submucous resection of the nasal septum.

    Science.gov (United States)

    Bizri, A R; al-Ajam, M; Zaytoun, G; al-Kutoubi, A

    2000-01-01

    A carotid cavernous fistula (CCF) is an abnormal arteriovenous anastomosis between the carotid artery and the cavernous sinus. Etiologies of this condition reported in the literature so far include facial trauma, rupture of an intracavernous aneurysm of the carotid artery, Ehler-Danlos syndrome and fibromuscular dysplasia of the cerebral arteries. Such fistulae were reported as complications of rhinoplasty, transsphenoidal surgery, embolization of cavernous sinus meningioma, and rhinocerebral mucormycosis. CCF may also occur spontaneously in children or as a congenital malformation. However, to our knowledge, submucous resection of the nasal septum has not been reported before to cause direct carotid-cavernous fistula. CT and angiographic findings are presented and a review of the literature for reported causes of CCF is made as well as a brief discussion of the possible pathophysiology.

  3. Limestone Caverns

    Science.gov (United States)

    Powell, Richard L.

    1970-01-01

    Describes the origin of limestone caverns, using Mammoth Cave as an example, with particular reference to the importance of groundwater information of caverns, the present condition of groundwater, and how caverns develop within fluctuating groundwater zones. (BR)

  4. MRI evidence for preserved regulation of intracranial pressure in patients with cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Meinel, Felix G.; Fischer, Judith; Pomschar, Andreas; Wöhrle, Natalie; Koerte, Inga K.; Steffinger, Denise [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany); Laubender, Rüdiger P. [Institute of Medical Informatics, Biometry and Epidemiology, Ludwig-Maximilians-University, Marchioninistr. 15, 81377 Munich (Germany); Muacevic, Alexander [European Cyberknife Center Munich, 81377 Munich (Germany); Reiser, Maximilian F. [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany); Alperin, Noam [Department of Radiology, Miller School of Medicine, University of Miami, Miami, FL 33136 (United States); Ertl-Wagner, Birgit, E-mail: birgit.ertl-wagner@med.uni-muenchen.de [Institute for Clinical Radiology, Ludwig-Maximilians-University Hospital, Marchioninistr. 15, 81377 Munich (Germany)

    2014-08-15

    Purpose: The purpose of this study was to investigate intracranial pressure and associated hemo- and hydrodynamic parameters in patients with cerebral arteriovenous malformations AVMs. Methods: Thirty consecutive patients with arteriovenous malformations (median age 38.7 years, 27/30 previously treated with radiosurgery) and 30 age- and gender-matched healthy controls were investigated on a 3.0 T MR scanner. Nidus volume was quantified on dynamic MR angiography. Total arterial cerebral blood flow (tCBF), venous outflow as well as aqueductal and craniospinal stroke volumes were obtained using velocity-encoded cine-phase contrast MRI. Intracranial volume change during the cardiac cycle was calculated and intracranial pressure (ICP) was derived from systolic intracranial volume change (ICVC) and pulse pressure gradient. Results: TCBF was significantly higher in AVM patients as compared to healthy controls (median 799 vs. 692 mL/min, p = 0.007). There was a trend for venous flow to be increased in both the ipsilateral internal jugular vein (IJV, 282 vs. 225 mL/min, p = 0.16), and in the contralateral IJV (322 vs. 285 mL/min, p = 0.09), but not in secondary veins. There was no significant difference in median ICP between AVM patients and control subjects (6.9 vs. 8.6 mmHg, p = 0.30) and ICP did not correlate with nidus volume in AVM patients (ρ = −0.06, p = 0.74). There was a significant positive correlation between tCBF and craniospinal CSF stroke volume (ρ = 0.69, p = 0.02). Conclusions: The elevated cerebral blood flow in patients with AVMs is drained through an increased flow in IJVs but not secondary veins. ICP is maintained within ranges of normal and does not correlate with nidus volume.

  5. Clinical and pathological changes in cerebral arteriovenous malformations after stereotactic radiosurgery failure

    Institute of Scientific and Technical Information of China (English)

    LIU Wei-ming; YE Xun; ZHAO Yuan-li; WANG Shuo; ZHAO Ji-zong

    2008-01-01

    Background Stereotactic radiosurgery is an alternative to resection of intracraniaI cerebral arteriovenous malformations (AVMs),while it will failin some cases.This study aimed to evaluate the changes after stereotactic radiosurgery for AVMs.Methods Nineteen cases with cerebral AVMs had failure after stereotactic radiosurgery therapy.The symptoms and angiography were assessed.All patients underwent microsurgery.Pathologic examination was performed for all cases and electron microscopic examination was carried out in 6 patients.Reaults Seven cases had hemorrhage from 12 to 98 months after stereotactic radiosurgery,5 had headache.4 had refractory encephalon edema,2 had epilepsy as a new symptom and 1 had a pressure cyst 5 years after radiosurgery.Angiography in 18 cases,8-98 months after radiation therapy,demonstrated no significant changes in 5 cases.slight reduction in 9,near complete obliteration in 1 and complete obliteration in 3.An abnormal vessel was found on pathologic examination in 17 cases,even one case had obliterated in angiography.Electron microscopy examination showed vessel wall weakness,but the vessels remained open and blood circulated.One case died because of a moribund state before surgery.The other 18 cases had no new neurological deficiencies,seizure control and no hemorrhage occurred after microsurgery at an average follow-up of 3 years.Conclusion Stereotactic radiotherapy for AVMs should have a long period follow-up.If serious complications occur,microsurgery can be performed as salvage treatment.

  6. Severe pulmonary oedema following therapeutic embolization with Onyx for cerebral arteriovenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Murugesan, C.; Saravanan, Sundararaj; Rajkumar, John; Prasad, Jagadish; Banakal, Sanjay; Muralidhar, Kanchi [Narayana Hrudayalaya Institute of Medical Sciences, Bangalore (India)

    2008-05-15

    Acute respiratory distress syndrome (ARDS) is characterized by sudden onset of respiratory distress, infiltrates on radiographs consistent with pulmonary oedema, hypoxaemia and increased work in breathing. Infiltrates on radiographs are bilateral, but may be patchy or diffuse and fluffy or dense. It is associated with absence of left heart failure and a PaO{sub 2}/FiO{sub 2} ratio of {<=}200. Ethylene vinyl alcohol copolymer dissolved in dimethyl sulfoxide (DMSO), which was approved by the US FDA in July 2005, is used as an embolic agent for cerebral arteriovenous malformation (AVM). It is a biocompatible liquid polymer that precipitates and solidifies on contact with blood, thus forming a soft and spongy embolus. We report a case of ARDS following therapeutic embolization with ethylene vinyl alcohol copolymer for cerebral AVM under general anaesthesia. Experienced perioperative physicians adopted standard anaesthetic technique and monitoring for this procedure. Acute respiratory distress and hypoxaemia developed in the patient following extubation of the trachea. Infiltrates seen on postprocedural chest radiographs were consistent with pulmonary oedema. DMSO, the solvent for the ethylene vinyl alcohol copolymer, is excreted via the lungs after administration and we postulate that DMSO was the possible cause of ARDS in this patient. Monitoring of haemodynamic parameters (invasive blood pressure, electrocardiography) and ventilatory parameters (ETCO{sub 2}, SpO{sub 2}, airway pressure monitoring) are important in the recognition of this possible event. One should be vigilant and anticipate this complication following therapeutic embolization with ethylene vinyl alcohol polymer for the treatment of cerebral AVM. (orig.)

  7. Gene expression signatures in the peripheral blood after radiosurgery of human cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Zabel-du Bois, Angelika [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Dept. of RadioOncology, Univ. of Heidelberg (Germany); Wagner-Ecker, Mechthild; Schwager, Christian; Wirkner, Ute; Huber, Peter E. [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Milker-Zabel, Stefanie; Debus, Juergen [Dept. of RadioOncology, Univ. of Heidelberg (Germany); Abdollahi, Amir [Dept. of Radiation Oncology, German Cancer Research Center, Heidelberg (Germany); Dept. of RadioOncology, Univ. of Heidelberg (Germany); Center of Cancer Systems Biology, Tufts Univ. School of Medicine, Boston, MA (United States)

    2010-02-15

    Purpose: To unravel biological mechanisms potentially resulting in the obliteration process after radiosurgery (RS) of human cerebral arteriovenous malformations (AVMs) by investigating molecular signatures on the transcriptomic level in peripheral blood of patients. Patients and Methods: Venous blood samples were obtained at definite points of time before and after RS. The samples were tested for radiation-induced changes regarding biological markers (mRNA) using cDNA and oligo-microarray technology. The corresponding expression profiles were correlated with clinical data and obliteration signs in radiologic imaging. Results: The proof of principle that RS outcome can be successfully correlated with transcriptomics of cellular blood components as disease parameter was demonstrated. The authors identified 76 differentially regulated genes (p < 0.001) after RS. Interestingly, in particular genes with known roles in antiangiogenic and procoagulative pathways were identified as potentially relevant. In particularly, the authors found a significant downregulation of neuropilin-2, protein C inhibitor and cyclin-dependent kinase 6. They also found that low pretreatment blood mRNA levels of TLR4 (toll-like receptor 4) and STAT3 (signal transducer and activator of transcription 3) correlated with fast obliteration of AVMs. Conclusion: The authors report on a novel technique for molecular biological analysis of blood from patients with cerebral AVM treated with RS. Differential regulation of genes in peripheral blood was successfully correlated with RS and time to obliteration of AVMs. The identified genes indicate a potential new methodology to monitor RS, which may result in an individualized therapy and optimized follow-up. (orig.)

  8. Extraosseous, Epidural Cavernous Hemangioma with Back Pain

    Science.gov (United States)

    Ozkal, Birol; Yaldiz, Can; Yaman, Onur; Ozdemır, Nail; Dalbayrak, Sedat

    2015-01-01

    Summary Background Cavernous malformations are characterized by enlarged vascular structures located in benign neural tissues within the cerebellum and spinal cord of the central nervous system. Cavernous hemangiomas (CHs) account for 5% to 12% of all spinal vascular malformations. Case Report We removed a hemorrhagic thoracic mass in a 40-year-old male patient who presented with progressive neurological deficits. Conclusions We found it appropriate to present this case due to its rarity. PMID:25960818

  9. Using a Machine Learning Approach to Predict Outcomes after Radiosurgery for Cerebral Arteriovenous Malformations.

    Science.gov (United States)

    Oermann, Eric Karl; Rubinsteyn, Alex; Ding, Dale; Mascitelli, Justin; Starke, Robert M; Bederson, Joshua B; Kano, Hideyuki; Lunsford, L Dade; Sheehan, Jason P; Hammerbacher, Jeffrey; Kondziolka, Douglas

    2016-01-01

    Predictions of patient outcomes after a given therapy are fundamental to medical practice. We employ a machine learning approach towards predicting the outcomes after stereotactic radiosurgery for cerebral arteriovenous malformations (AVMs). Using three prospective databases, a machine learning approach of feature engineering and model optimization was implemented to create the most accurate predictor of AVM outcomes. Existing prognostic systems were scored for purposes of comparison. The final predictor was secondarily validated on an independent site's dataset not utilized for initial construction. Out of 1,810 patients, 1,674 to 1,291 patients depending upon time threshold, with 23 features were included for analysis and divided into training and validation sets. The best predictor had an average area under the curve (AUC) of 0.71 compared to existing clinical systems of 0.63 across all time points. On the heldout dataset, the predictor had an accuracy of around 0.74 at across all time thresholds with a specificity and sensitivity of 62% and 85% respectively. This machine learning approach was able to provide the best possible predictions of AVM radiosurgery outcomes of any method to date, identify a novel radiobiological feature (3D surface dose), and demonstrate a paradigm for further development of prognostic tools in medical care.

  10. Congenital cerebral malformations and dysfunction in fetuses and newborns following the 2013 to 2014 Zika virus epidemic in French Polynesia.

    Science.gov (United States)

    Besnard, Marianne; Eyrolle-Guignot, Dominique; Guillemette-Artur, Prisca; Lastère, Stéphane; Bost-Bezeaud, Frédérique; Marcelis, Ludivine; Abadie, Véronique; Garel, Catherine; Moutard, Marie-Laure; Jouannic, Jean-Marie; Rozenberg, Flore; Leparc-Goffart, Isabelle; Mallet, Henri-Pierre

    2016-01-01

    We detected an unusual increase in congenital cerebral malformations and dysfunction in fetuses and newborns in French Polynesia, following an epidemic of Zika virus (ZIKV), from October 2013 to March 2014. A retrospective review identified 19 cases, including eight with major brain lesions and severe microcephaly, six with severe cerebral lesions without microcephaly and five with brainstem dysfunction without visible malformations. Imaging revealed profound neurological lesions (septal and callosal disruption, ventriculomegaly, abnormal neuronal migration, cerebellar hypoplasia, occipital pseudocysts, brain calcifications). Amniotic fluid was drawn from seven cases at gestation weeks 20 to 29. ZIKV RNA was detected by RT-PCR and infectious ZIKV isolates were obtained in four of five microcephalic, but not in two non-microcephalic cases with severe brain lesions. Medical termination of pregnancy was performed in eleven cases; two cases with brainstem dysfunction died in the first months of life; six cases are alive, with severe neurological impairment. The results show that four of seven tested fetuses with major neurological injuries were infected with ZIKV in utero. For other non-microcephalic, congenital abnormalities we were not able to prove or exclude ZIKV infection retrospectively. The unusual occurrence of brain malformations or dysfunction without microcephaly following a ZIKV outbreak needs further studies.

  11. Complications following linear accelerator based stereotactic radiation for cerebral arteriovenous malformations

    DEFF Research Database (Denmark)

    Skjøth-Rasmussen, Jane; Roed, Henrik; Ohlhues, Lars;

    2010-01-01

    Primarily, gamma knife centers are predominant in publishing results on arteriovenous malformations (AVM) treatments including reports on risk profile. However, many patients are treated using a linear accelerator-most of these at smaller centers. Because this setting is different from a large...... gamma knife center, the risk profile at Linac departments could be different from the reported experience. Prescribed radiation doses are dependent on AVM volume. This study details results from a medium sized Linac department center focusing on risk profiles....

  12. Developmental malformations of the cerebral cortex; Heterotopie, Polymikrogyrie, Lissenzephalie und Co. Malformationen der kortikalen Hirnentwicklung

    Energy Technology Data Exchange (ETDEWEB)

    Reiss-Zimmermann, Martin [Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie, Universitaetsklinikum Leipzig AoeR (Germany); Weber, D.; Sorge, I.; Hirsch, W. [Abt. Paediatrische Radiologie, Universitaetsklinikum Leipzig AoeR (Germany); Merkenschlager, A. [Universitaetsklinik und Poliklinik fuer Kinder und Jugendliche, Universitaetsklinikum Leipzig AoeR (Germany)

    2010-06-15

    Migration disorders (MD) are increasingly recognized as an important cause of epilepsy and developmental delay. Up to 25% of children with refractory epilepsy have a cortical malformation. MD encompass a wide spectrum with underlying genetic etiologies and clinical manifestations. Research regarding the delineation of the genetic and molecular basis of these disorders has provided greater insight into the pathogenesis of not only the malformation but also the process involved in normal cortical development. Diagnosis of MD is important since patients who fail three antiepileptic medications are less likely to have their seizures controlled with additional trials of medications and therefore epilepsy surgery should be considered. Recent improvements in neuroimaging have resulted in a significant increase in the recognition of MD. Findings can be subdivided in disorders due to abnormal neurogenesis, neuronal migration, neuronal migration arrest and neuronal organization resulting in different malformations like microcephaly, lissencephaly, schizencephaly and heterotopia. The examination protocol should include T1-w and T2-w sequences in adequate slice orientation. T1-w turbo-inversion recovery sequences (TIR) can be helpful to diagnose heterotopia. Contrast agent is needed only to exclude other differential diagnoses. (orig.)

  13. Cerebral hemodynamics and the role of transcranial Doppler applications in the assessment and management of cerebral arteriovenous malformations.

    Science.gov (United States)

    Busch, Kathryn J; Kiat, Hosen; Stephen, Michael; Simons, Mary; Avolio, Alberto; Morgan, Michael Kerin

    2016-08-01

    Dramatic hemodynamic changes occur following resection of brain arteriovenous malformations (AVM). Transcranial Doppler (TCD) records non-invasive velocity and pulsatility parameters. We undertook a systematic review to assess AVM hemodynamics including the time course of changes in velocity and pulsatility in patients undergoing AVM resection. The review employed the Embase and Medline databases. A search strategy was designed. An initial title search for clinical series on AVM and TCD was performed followed by a search for reports on AVM and TCD. A total of 283 publications were selected. Full text analysis produced 54 studies with extractable data regarding AVM, velocity and pulsatility. Two TCD techniques were utilized: conventional "blind" TCD (blind TCD); and transcranial color duplex Doppler (TCCD). Of these, 23 publications reported on blind TCD and seven on TCCD. The presence of high velocity and low pulsatility within AVM feeding arteries preoperatively followed by a postoperative decrease in velocity and subsequent increase in pulsatility of feeding arteries is established. The time sequence of hemodynamic changes following AVM resection using TCD remains uncertain, confounded by variations in methodology and timing of perioperative measurements. Of the two techniques, TCCD reported qualitative aspects including improved differentiation of feeding arteries from draining veins. However, there are a limited number of studies supporting this conclusion. Furthermore, none report reproducible changes with time from treatment. TCCD appears to be a useful technique to analyze the hemodynamic changes occurring following treatment of AVM, however little data is available. This is a field of research that is appropriate to pursue.

  14. Pathobiology of human cerebrovascular malformations: basic mechanisms and clinical relevance.

    Science.gov (United States)

    Gault, Judith; Sarin, Hemant; Awadallah, Nabil A; Shenkar, Robert; Awad, Issam A

    2004-07-01

    Cerebrovascular malformations affect more than 3% of the population, exposing them to a lifetime risk of hemorrhagic stroke, seizures, and focal neurological deficits. Cerebral cavernous malformations (CCMs) exhibit an immature vessel wall, a brittle hemorrhagic tendency, and epileptogenesis, whereas arteriovenous malformations (AVMs) lack capillary beds and manifest apoplectic bleeding under high-flow conditions. There are also more benign venous anomalies, capillary malformations, and lesions with mixed and transitional features. Advances have been made toward understanding the natural history, radiological and pathological correlates, and clinical management. Yet, mechanisms of lesion genesis and clinical manifestations remain largely unknown, and the clinical behavior in individual patients is highly unpredictable. Lesion pathogenesis likely involves abnormal assembly or maintenance of blood vessels, resulting in dysmorphic vessel phenotypes. Familial CCM disease is in part caused by mutations in a cytoskeletal-related protein that is likely integral to interendothelial cell connectivity and maturation of the vascular wall. Rare familial forms of AVM disease have been correlated with two different transforming growth factor-beta receptor components, possibly causing disturbance in signaling during vascular assembly. Relevance of these mechanisms to the more common and otherwise identical sporadic CCM and AVM lesions is being explored. In this report, basic mechanisms of vasculogenesis and angiogenesis and how they possibly relate to the common cerebrovascular malformation lesions are reviewed. Novel concepts are discussed related to the cellular, molecular, and genetic substrates in CCM and AVM as well as to how this knowledge can be applied to predict, explain, and possibly modify clinical disease manifestations.

  15. Cavernous hemangioma of Meckel's cave. Case report.

    Science.gov (United States)

    Fehlings, M G; Tucker, W S

    1988-04-01

    A case of a cavernous hemangioma located within Meckel's cave and involving the gasserian ganglion is described in a patient presenting with facial pain and a trigeminal nerve deficit. Although these lesions have been reported to occur in the middle fossa, this is believed to be the first case of such a vascular malformation arising solely from within Meckel's cave.

  16. Severe pulmonary oedema following therapeutic embolization with Onyx for cerebral arteriovenous malformation.

    Science.gov (United States)

    Murugesan, C; Saravanan, Sundararaj; Rajkumar, John; Prasad, Jagadish; Banakal, Sanjay; Muralidhar, Kanchi

    2008-05-01

    Acute respiratory distress syndrome (ARDS) is characterized by sudden onset of respiratory distress, infiltrates on radiographs consistent with pulmonary oedema, hypoxaemia and increased work in breathing. Infiltrates on radiographs are bilateral, but may be patchy or diffuse and fluffy or dense. It is associated with absence of left heart failure and a PaO2/FiO2 ratio of SpO2, airway pressure monitoring) are important in the recognition of this possible event. One should be vigilant and anticipate this complication following therapeutic embolization with ethylene vinyl alcohol polymer for the treatment of cerebral AVM.

  17. Non-enhanced MR imaging of cerebral arteriovenous malformations at 7 Tesla

    Energy Technology Data Exchange (ETDEWEB)

    Wrede, Karsten H.; Dammann, Philipp [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Neurosurgery, Essen (Germany); Johst, Soeren; Maderwald, Stefan [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); Moenninghoff, Christoph; Forsting, Michael [University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); Schlamann, Marc [University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); University Hospital Giessen, Department of Neuroradiology, Giessen (Germany); Sandalcioglu, I.E. [University Hospital Essen, Department of Neurosurgery, Essen (Germany); Nordstadtkrankenhaus Hannover, Department of Neurosurgery, Hannover (Germany); Ladd, Mark E. [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany); German Cancer Research Center (DKFZ), Division of Medical Physics in Radiology (E020), Heidelberg (Germany); Sure, Ulrich [University Hospital Essen, Department of Neurosurgery, Essen (Germany); Umutlu, Lale [University Duisburg-Essen, Erwin L. Hahn Institute for Magnetic Resonance Imaging, Essen (Germany); University Hospital Essen, Department of Diagnostic and Interventional Radiology and Neuroradiology, Essen (Germany)

    2016-03-15

    To evaluate prospectively 7 Tesla time-of-flight (TOF) magnetic resonance angiography (MRA) and 7 Tesla non-contrast-enhanced magnetization-prepared rapid acquisition gradient-echo (MPRAGE) for delineation of intracerebral arteriovenous malformations (AVMs) in comparison to 1.5 Tesla TOF MRA and digital subtraction angiography (DSA). Twenty patients with single or multifocal AVMs were enrolled in this trial. The study protocol comprised 1.5 and 7 Tesla TOF MRA and 7 Tesla non-contrast-enhanced MPRAGE sequences. All patients underwent an additional four-vessel 3D DSA. Image analysis of the following five AVM features was performed individually by two radiologists on a five-point scale: nidus, feeder(s), draining vein(s), relationship to adjacent vessels, and overall image quality and presence of artefacts. A total of 21 intracerebral AVMs were detected. Both sequences at 7 Tesla were rated superior over 1.5 Tesla TOF MRA in the assessment of all considered AVM features. Image quality at 7 Tesla was comparable with DSA considering both sequences. Inter-observer accordance was good to excellent for the majority of ratings. This study demonstrates excellent image quality for depiction of intracerebral AVMs using non-contrast-enhanced 7 Tesla MRA, comparable with DSA. Assessment of untreated AVMs is a promising clinical application of ultra-high-field MRA. (orig.)

  18. Vascular tumors and malformations of the colon

    Institute of Scientific and Technical Information of China (English)

    Israel Fernandez-Pineda

    2009-01-01

    The term "hemangioma" refers to the common tumor of infancy that exhibits rapid postnatal growth and slow regression during childhood. It may cause confusion with venous malformations that are often incorrectly called "cavernous hemangioma". Venous malformations comprise abnormally formed channels that are lined by quiescent endothelium. Accurate diagnosis is required for selecting the appropriate treatment.

  19. Radiosurgery with a linear accelerator in cerebral arteriovenous malformations Radiocirurgia com acelerador linear em malformações arteriovenosas cerebrais

    Directory of Open Access Journals (Sweden)

    Sérgio Carlos Barros Esteves

    2008-04-01

    Full Text Available OBJECTIVE: To evaluate results achieved with radiosurgery and complications of the procedure when treating arteriovenous malformations with linear accelerator. METHODS: This retrospective study was conducted between October 1993 and December 1996. Sixty-one patients with arteriovenous malformations were treated with radiosurgery utilizing a 6MV energy linear accelerator. Ages of the 32 female and 29 male patients ranged from 6 to 54 years (mean: 28.3 years. The most frequent initial symptom was cephalea (45.9%, followed by neurological deficit (36.1%. Cerebral hemorrhage diagnosed by image was observed in 35 patients (57.3%. Most arteriovenous malformations (67.2% were graded Spetzler III and IV. Venous stenosis (21.3% and aneurysm (13.1% were the most frequent angioarchitecture changes. The dose administered varied from 12 to 27.5Gy in the periphery of the lesion. RESULTS: Out of twenty-eight patients that underwent conclusive angiography control, complete obliteration was achieved in 18 (72% and treatment failed in 7 (absence of occlusion with more than 3 years of follow-up. Four were submitted to a second radiosurgery, and one of these has shown obliteration after 18 months of follow-up. DISCUSSION: Several factors were analyzed regarding the occlusion rate (gender, age, volume, localization, Spetzler, flow, embolization, total of isocenters, prescribed dose and chosen isodose and complications (total of isocenters, localization, volume, maximum dose, prescribed dose and chosen isodose. Analyzed variables showed no statistical significance for obliteration of the vessel, as well as for treatment complications. The largest diameter of the arteriovenous malformation, its volume and the dose administered did not influence time of obliteration. CONCLUSION: Radiosurgery is effective in the treatment of arteriovenous malformations and can be an alternative for patients with clinical contraindication or with lesions in eloquent areas. In the studied

  20. Advances in diagnosis and treatment of cerebral venous system diseases

    Directory of Open Access Journals (Sweden)

    Xiao-yun LIU

    2016-11-01

    Full Text Available Cerebral venous system diseases include cerebral venous thrombosis (CVT, venous sinus stenosis, carotid cavernous fistula (CCF, intracranial arteriovenous malformation (AVM and so on. In recent years, due to the rapid development of neuroimaging and interventional technology, more and more cerebral venous system diseases have been timely diagnosed and treated, such as magnetic resonance black-blood thrombus imaging (MRBTI in the diagnosis of CVT, stenting in the treatment of venous sinus stenosis, micro coil plus Onyx glue or covered stents in the treatment of CCF, which allow us to make a deeper recognition of cerebral venous system diseases. Therefore, this paper will introduce the latest diagnosis and treatment of cerebral venous system diseases. DOI: 10.3969/j.issn.1672-6731.2016.11.006

  1. Color-coded digital subtraction angiography in the management of a rare case of middle cerebral artery pure arterial malformation. A technical and case report.

    Science.gov (United States)

    Feliciano, Caleb E; Pamias-Portalatin, Eva; Mendoza-Torres, Jorge; Effio, Euclides; Moran, Yadira; Rodriguez-Mercado, Rafael

    2014-12-01

    The advent of flow dynamics and the recent availability of perfusion analysis software have provided new diagnostic tools and management possibilities for cerebrovascular patients. To this end, we provide an example of the use of color-coded angiography and its application in a rare case of a patient with a pure middle cerebral artery (MCA) malformation. A 42-year-old male chronic smoker was evaluated in the emergency room due to sudden onset of severe headache, nausea, vomiting and left-sided weakness. Head computed tomography revealed a right basal ganglia hemorrhage. Cerebral digital subtraction angiography (DSA) showed a right middle cerebral artery malformation consisting of convoluted and ectatic collateral vessels supplying the distal middle cerebral artery territory-M1 proximally occluded. An associated medial lenticulostriate artery aneurysm was found. Brain single-photon emission computed tomography with and without acetazolamide failed to show problems in vascular reserve that would indicate the need for flow augmentation. Twelve months after discharge, the patient recovered from the left-sided weakness and did not present any similar events. A follow-up DSA and perfusion study using color-coded perfusion analysis showed perforator aneurysm resolution and adequate, albeit delayed perfusion in the involved vascular territory. We propose a combined congenital and acquired mechanism involving M1 occlusion with secondary dysplastic changes in collateral supply to the distal MCA territory. Angiographic and cerebral perfusion work-up was used to exclude the need for flow augmentation. Nevertheless, the natural course of this lesion remains unclear and long-term follow-up is warranted.

  2. MRI对脑静脉畸形的诊断价值%Diagnostic value of MRI in cerebral venous malformation

    Institute of Scientific and Technical Information of China (English)

    牛磊; 朱蒙蒙; 王明皓; 崔文; 刘圆圆; 朱斌

    2011-01-01

    目的:探讨MRI对脑静脉畸形(CVM)的诊断价值.方法:收集本院18例脑静脉畸形病例,均行磁共振T1WI、T2WI、FLAIR、SWI、DWI、MRA及T1 Wl增强扫描的资料.结果:CVM位于幕下5例,幕上13例,18例均呈典型的“海蛇头”征.MRI显示髓静脉呈长T1长T2信号,引流静脉呈短T1稍长T2信号或长T1长T2信号.FLAIR上髓静脉为稍高信号,引流静脉为低信号或稍高信号.SWI及DWI上髓静脉和引流静脉均为低信号.增强呈“海蛇头”样改变,即多条髓静脉呈辐射状汇入粗大的引流静脉.结论:MRI能明确诊断脑静脉畸形.T1WI增强扫描及SWI均较敏感.SWI对CVM的诊断价值明显优于常规MRI序列.%Objective:To investigate the diagnostic value of MRI in cerebral venous malformations (CVM). Methods: 18 patients of CVM were collected, and all of them underwent T1 WI, T2WI. FLAIR. SWI. DWI. MRA. And T,W1 enhancement scans. Results:CVMs were located in infra in five cases, and supra tentorium in thirteen eleven patients. Eighteen presented the typical " caput medusae" appearance. As for MRI plain scans.the medullary veins showed hypointensity in T1 WI and hyperintensity in T2WI, while, the drainage veins featured hyperintensity in T1 WI and hyperintensity in T2 WI. FLAIR sequences manifested less medullary veins with hyperintensity and drainage veins with hypointensity or hyperintensity. Both the medullary and the drainage veins appeared hypointensity in DWI and SWI. After enhancement, numerous radiating medullary veins gathered together into one or two larger drainage veins and flowed into superficial or deeper veins. Demonstrating the so-called "Caput Medusae" appearance. Conclusion: MRI is the first choice for suspected CVM patients thus providing more diagnostic information. T1 WI enhancement and SWI are sensitive to manifesting CVMs. SWI diagnosis of CVM is significantly superior to conventional MRI sequences.

  3. 儿童脑动静脉畸形出血危险因素分析%Rick Factors for Cerebral Arteriovenous Malformation Hemorrhage in Pediatric Patients

    Institute of Scientific and Technical Information of China (English)

    陈晓霖; 马力; 王书磊; 赵元立

    2015-01-01

    Objective To investigate the clinical and morphological features of cerebral arteriovenous malformation (CAVM) in children and to assess its risk factors of hemorrhage in pediatric patients. Methods The clinical data of pediatric patients admitted to Beijing Tiantan Hospital for CAVMs between 2012 and 2014 were retrospectively analyzed. All patients were diagnosed using digital subtraction angiography. The effects of demographic characteristics and CAVM morphological characteristics on hemorrhage presentation were studied using univariate and multivariate Logistic regression analysis. The characteristics including the gender, age, deep location, malformation diameter, AVM side, venous drainage, associated aneurysms. Results Seventy-three pediatric CAVM cases were identified, 49 (67.1%) cases presented with hemorrhage. The malformation diameter had signiifcant difference on the malformation, (odds ratio [OR] 0.96, 95% confidence interval [CI] 0.93~0.99,P<0.05). The gender, age, AVM side, deep location, deep venous drainage, associated aneurysms, venous drainage did not have significant difference on the malformation. Conclusion Hemorrhagic brain arteriovenous malformations relate with the malformation diameter, and the malformations with small AVM diameter should be treated early to reduce morbidity and mortality.%目的:研究儿童脑动静脉畸形(cerebral arteriovenous malformation,CAVM)临床特点及形态特点,评估其破裂出血的相关因素。方法回顾性分析2012年1月~2014年12月首都医科大学附属北京天坛医院收治年龄小于14岁CAVM患者73例,均经数字减影血管造影(digital subtraction angiography,DSA)检查明确诊断。采用单变量及多变量Logistic回归分析儿童入院时CAVM破裂出血与患者性别、年龄、CAVM侧别、DSA最大径、是否合并动脉瘤、病变是否位于深部、是否纯深静脉引流及静脉引流类型(浅静脉、深静脉及浅静脉合并深静脉)

  4. ATLAS Cavern baseplate

    CERN Multimedia

    2002-01-01

    This video shows the incredible amounth of iron used for ATLAS cavern. Please look at the related links and also videos that are concerning the civil engineering where you can see even more detailed cavern excavation work.

  5. 小脑脑桥角海绵状血管畸形三例并文献复习%Cystic cavernous malformations in the cerebellopontine angle: report of three cases and literature review

    Institute of Scientific and Technical Information of China (English)

    于金录; 曲丽梅; 李叶; 王柏; 王宏磊

    2011-01-01

    Objective To investigate the clinical presentation,imaging features and treatment experience of cystic cavernous malformations (CMs) in the cerebellopontine angle (CPA).Methods Three patients with the CMs of the CPA between 2005 and 2010 in our institute were summarised.Five patients from MEDLINE search of the English literature from 1985 to 2010 for CMs of the CPA were identified.We reviewed the characteristics of these 5 cases and our cases.Results Their presenting symptoms of 5 cases in the literature included headache,injury of cranial nerves in the CPA and cerebellar symptoms.But they did not show signs of brain stem compression.MRI and/or CT scans showed that the lesions in the eight cases of CMs all had solid and cystic components,and no adhesion with internal auditory canal and skull base dura mater was present in the CPA.Despite the fact that cystic CMs also involved cranial nerves,cerebellum,brainstem and arteries,they could be separated from these surrounding structures thanked to the presence of well - margined adhesion,which was contrary to solid CMs.We reported three cystic CMs of the CPA in our institute that was the same as the above.Conclusion Although cystic CMs of the CPA had no established imaging features,a diagnosis of CMs may be suspected when a cystic lesion is present in the CPA and does not involve internal acoustic meatus or dura matter of the skull base.Skillful microsurgical techniques will secure good outcomes for patients with cystic CMs in the CPA.%目的 总结小脑脑桥角(CPA)区海绵状血管畸形(CMs)的临床表现、影像学特征及治疗特点.方法 2005年6月至2010年6月我科共收治CPA区CMs病例3例,进行报告.搜索MEDLINE上自1985年至2010年文献查到此类病例5例,进行文献复习.结果 文献复习的5例CMs临床表现为脑神经及小脑受累症状,但无脑干受压症状;影像见CMs均呈囊实性,与内听道及颅底硬膜无粘连;术中见CMs与周边结构有粘连,但可进行分

  6. Severe progressive scoliosis due to huge subcutaneous cavernous hemangioma: A case report

    Directory of Open Access Journals (Sweden)

    Toyama Yoshiaki

    2011-03-01

    Full Text Available Abstract Cavernous hemangioma consists mainly of congenital vascular malformations present before birth and gradually increasing in size with skeletal growth. A small number of patients with cavernous hemangioma develop scoliosis, and surgical treatment for the scoliosis in such cases has not been reported to date. Here we report a 12-year-old male patient with severe progressive scoliosis due to a huge subcutaneous cavernous hemangioma, who underwent posterior correction and fusion surgery. Upon referral to our department, radiographs revealed a scoliosis of 85° at T6-L1 and a kyphosis of 58° at T4-T10. CT and MR images revealed a huge hemangioma extending from the subcutaneous region to the paraspinal muscles and the retroperitoneal space and invading the spinal canal. Posterior correction and fusion surgery using pedicle screws between T2 and L3 were performed. Massive hemorrhage from the hemangioma occurred during the surgery, with intraoperative blood loss reaching 2800 ml. The scoliosis was corrected to 59°, and the kyphosis to 45° after surgery. Seven hours after surgery, the patient suffered from hypovolemic shock and disseminated intravascular coagulation due to postoperative hemorrhage from the hemangioma. The patient developed sensory and conduction aphasia caused by cerebral hypoxia during the shock on the day of the surgery. At present, two years after the surgery, although the patient has completely recovered from the aphasia. This case illustrates that, in correction surgery for scoliosis due to huge subcutaneous cavernous hemangioma, intraoperative and postoperative intensive care for hemodynamics should be performed, since massive hemorrhage can occur during the postoperative period as well as the intraoperative period.

  7. Disease: H00534 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00534 Cerebral cavernous malformation Cerebral cavernous malformations are vascular malformations...gene) Brouillard P, Vikkula M Genetic causes of vascular malformations. Hum Mol Genet 16 Spec No. 2:R140-9 (

  8. Sulindac metabolites decrease cerebrovascular malformations in CCM3-knockout mice.

    Science.gov (United States)

    Bravi, Luca; Rudini, Noemi; Cuttano, Roberto; Giampietro, Costanza; Maddaluno, Luigi; Ferrarini, Luca; Adams, Ralf H; Corada, Monica; Boulday, Gwenola; Tournier-Lasserve, Elizabeth; Dejana, Elisabetta; Lampugnani, Maria Grazia

    2015-07-07

    Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe neurological consequences. At present, the only recommended treatment of CCM is surgical. Because surgery is often not applicable, pharmacological treatment would be highly desirable. We describe here a murine model of the disease that develops after endothelial-cell-selective ablation of the CCM3 gene. We report an early, cell-autonomous, Wnt-receptor-independent stimulation of β-catenin transcription activity in CCM3-deficient endothelial cells both in vitro and in vivo and a triggering of a β-catenin-driven transcription program that leads to endothelial-to-mesenchymal transition. TGF-β/BMP signaling is then required for the progression of the disease. We also found that the anti-inflammatory drugs sulindac sulfide and sulindac sulfone, which attenuate β-catenin transcription activity, reduce vascular malformations in endothelial CCM3-deficient mice. This study opens previously unidentified perspectives for an effective pharmacological therapy of intracranial vascular cavernomas.

  9. Endothelial Gene Expression and Molecular Changes in Response to Radiosurgery in In Vitro and In Vivo Models of Cerebral Arteriovenous Malformations

    Directory of Open Access Journals (Sweden)

    Jian Tu

    2013-01-01

    Full Text Available Radiosurgery for cerebral arteriovenous malformations (AVMs is limited to 2-year latency. There is no early marker to monitor whether the lesion is responsive to radiosurgery. In this study, we examined endothelial gene expression and molecular changes in response to radiosurgery. Gene expression of E- and P-selectin, ICAM-1, PECAM-1, VCAM-1, tissue factor, and vWF in human cerebral microvascular endothelial cells was quantified by RT-qPCR at different radiation doses and time points. Soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in an animal model of AVMs were quantified by ELISA at different time after radiosurgery. We found that gene expression of E- and P-selectin, ICAM-1, PECAM-1, and VCAM-1 was upregulated by radiation in a dose-dependent manner (P<.05. Gene expression of E- and P-selectin and ICAM-1 was more sensitive to irradiation than that of PECAM-1 and VCAM-1. Radiosurgery induced gene expression of P-selectin, ICAM-1, PECAM-1, and VCAM-1 was linearly correlated with time (P<.05. Radiosurgery induced elevation of soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in a rat model of AVMs (P<.05. Thus, a combination of these molecules measured at different time points may serve as an early predictor of responsiveness of AVMs to radiosurgery.

  10. Endothelial gene expression and molecular changes in response to radiosurgery in in vitro and in vivo models of cerebral arteriovenous malformations.

    Science.gov (United States)

    Tu, Jian; Hu, Zhiqiang; Chen, Zhongbin

    2013-01-01

    Radiosurgery for cerebral arteriovenous malformations (AVMs) is limited to 2-year latency. There is no early marker to monitor whether the lesion is responsive to radiosurgery. In this study, we examined endothelial gene expression and molecular changes in response to radiosurgery. Gene expression of E- and P-selectin, ICAM-1, PECAM-1, VCAM-1, tissue factor, and vWF in human cerebral microvascular endothelial cells was quantified by RT-qPCR at different radiation doses and time points. Soluble E- and P-selectin, ICAM-1, VCAM-1, and tissue factor in an animal model of AVMs were quantified by ELISA at different time after radiosurgery. We found that gene expression of E- and P-selectin, ICAM-1, PECAM-1, and VCAM-1 was upregulated by radiation in a dose-dependent manner (P rat model of AVMs (P < .05). Thus, a combination of these molecules measured at different time points may serve as an early predictor of responsiveness of AVMs to radiosurgery.

  11. Abducens Nerve Palsy and Ipsilateral Horner Syndrome in a Patient With Carotid-Cavernous Fistula.

    Science.gov (United States)

    Kal, Ali; Ercan, Zeynep E; Duman, Enes; Arpaci, Enver

    2015-10-01

    The combination of abducens nerve palsy and ipsilateral Horner syndrome was first described by Parkinson and considered as a localizing sign of posterior cavernous sinus lesions. The authors present a case with right abducens nerve palsy with ipsilateral Horner syndrome in a patient with carotid-cavernous fistula because of head trauma. The patient was referred to the ophthalmology clinic with diplopia complaint after suffering a head trauma during a motorcycle accident. Cerebral angiography showed low-flow carotid-cavernous fistula.

  12. Spontaneous obliteration highlights the dynamic nature of cerebral arteriovenous malformations: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Su Lone Lim

    2016-01-01

    Conclusion: In our center′s 20-year experience of treatment of cerebral AVMs (approximately 600 cases, this is the only case that has been aborted due to spontaneous obliteration leading us to infer that the incidence of spontaneous AVM obliteration is <1%. Spontaneous obliteration of AVM is a rare but well-established phenomenon that bears testimony to the dynamics of this vascular disorder.

  13. Postoperative visual loss following cerebral arteriovenous malformation surgery: a case report [v2; ref status: indexed, http://f1000r.es/308

    Directory of Open Access Journals (Sweden)

    Nicolai Goettel

    2014-03-01

    Full Text Available We report the case of a 46 year-old woman presenting with unilateral postoperative visual loss after right frontal craniotomy for resection of an arteriovenous malformation in the supine position. The intraoperative course was uneventful with maintenance of hemodynamic stability. Blood loss was 300 ml; postoperative hemoglobin was 12.4 g/dl. In the recovery room, the patient reported loss of vision in her right eye. Ophthalmologic examination revealed decreased visual acuity, color vision, and visual field. Assessment of the retina was normal, but the patient showed a relative afferent pupillary defect consistent with the clinical diagnosis of ischemic optic neuropathy. Postoperative computer tomogram showed normal perfusion of ophthalmic artery and vein, no hemorrhage or signs of cerebral ischemia or edema. The patient recovered most of her vision 3 months after surgery. Anesthesiologists should be aware that this condition may follow uncomplicated intracranial surgeries in the supine position, and should obtain prompt ophthalmologic consultation when a patient develops postoperative visual loss.

  14. Incidence of cerebral edema in patients with cerebral arteriovenous malformations treated by radiosurgery%脑动静脉畸形γ刀放射治疗后脑水肿发生情况分析

    Institute of Scientific and Technical Information of China (English)

    利晞; 梁舜尧; 谭理连; 胡滨; 侯仲军; 陈耀棠; 于晓君; 曹兵艺

    2013-01-01

    目的 探讨脑动静脉畸形γ刀放射治疗后不同随访阶段放射性脑水肿的发生率.方法 回顾性分析广州医学院第二附属医院放射科自1995年1月至2010年12月十余年间内经γ刀治疗的脑动静脉畸形患者的临床资料,共经磁共振检查随访351例次.统计γ刀术后不同随访阶段放射性脑水肿发生率并采用采用x2检验分析. 结果 γ刀放射治疗后本组患者放射性脑水肿阳性率为36.2%;不同随访阶段放射性脑水肿阳性率分别为:≤12月43.0%,13~36月45.3%,37~60月23.1%,61~120月13.6%,>120月5.9%;各随访阶段间放射性脑水肿的发生率比较差异有统计学意义(x2=26.514,P=0.000). 结论 放射性脑水肿是脑动静脉畸形γ刀治疗后最常见的并发症之一,部分患者可出现顽固性放射性脑水肿.γ刀治疗后第一阶段(≤12月)、第二阶段(13~36月)放射性脑水肿发生率较高,应临床密切关注.%Objective To study the incidence of cerebral edema in patients with cerebral arteriovenous malformations (AVMs) treated by Gamma knife.Methods The clinical data of 351 patients with AVMs,admitted to and treated with gamma knife in our hospital from January 1995 to December 2010,were retrospectively analyzed.MR imaging was performed on these patients to review the characteristics of cerebral edema and compare the different incidences of cerebral edema at different follow up periods; chi-square analysis was employed on these data.Results After being treated with gamma knife,the total positive rate of cerebral edema was 36.2%.At different follow-up periods,the positive rates of cerebral edema were different:43.0% within 12 months of treatment,45.3% at 13-36 months after treatment,23.1% at 37-60 months after treatment,13.6% at 61-120 months after treatment,and 5.9% more than 120 months after treatment; significant difference was noted between each two follow up periods (x2=26.514,P=0.000).Conclusion

  15. Bilateral orbital cavernous haemangiomas.

    OpenAIRE

    Fries, P D; Char, D. H.

    1988-01-01

    Simultaneous bilateral orbital lesions are rare. The differential diagnosis includes orbital pseudotumour, metastasis, leukaemia, lymphoma, Wegener's granulomatosis, and neurofibromatosis. We report what we believe to be the first case of bilateral orbital cavernous haemangiomas.

  16. Advantages of T2 reversed fast spin-echo image and enhanced three-dimensional surface MR angiography for the diagnosis of cerebral arteriovenous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Tanabe, Sumiyoshi; Honmou, Osamu; Minamida, Yoshihiro; Hashi, Kazuo [Sapporo Medical Univ. (Japan). School of Medicine

    2001-09-01

    Although the anatomical investigation of cerebral arteriovenous malformation (AVM) with conventional neuro-imagings considerably supports the preoperative evaluation, it is still hard to dissect the detailed anatomical conformations of AVMs such as location of nidus, identification of feeding arteries or draining veins, and the three-dimensional configuration of nidus in sulci or gyri. In this study, we investigated the efficacy of enhanced three-dimensional surface MR angiography (surface MRA) and T2 reversed image (T2R image) in the diagnosis and surgical planning for cerebral AVMs. The diagnostic accuracy was studied in twelve AVMs: four AVMs closed to motor area, one to Broca area, one to Wernicke area, four in temporal lobe, and two in occipital lobe. Images were obtained with a SIGNA HORIZON LX 1.5T VER 8.2. To construct T2R, the brain is scanned by fast SE method with long TR and was displayed with the reversed gray scale, which seemed similar to T1WI. Surface MRA is a fusion image of MRA and surface image in the workstation. The original data was obtained by enhanced 3D-SPGR method. MRA image was reconstructed with MIP method, and surface image was manipulated with a volume rendering method. T2R images demonstrated seven sulcal AVMs, three gyral AVMs, and two sulco-gyral AVMs; five AVMs located on cortex, four extended to subcortex, and three to paraventricular brain. The images clearly showed six AVMs had hypervascular network such as modja-modja vascular formation. Surface MRA represented nidus adjacent to eloquent area. They were present in central sulcus, precentral sulcus, intraparietal sulcus, inferior frontal sulcus, sylvian fissure, superior temporal sulcus, inferior temporal sulcus, superior temporal gyrus, inferior temporal gyrus, medial temporal gyrus, premotor area and superior frontal sulcus, precuneus and parieto-occipital sulcus. It was easy to identify the point of feeding arteries going down into the sulcus and the junction-point of nidus

  17. Plasticity of motor function and surgical outcomes in patients with cerebral arteriovenous malformation involving primary motor area:insight from fMRI and DTI

    Institute of Scientific and Technical Information of China (English)

    Lijun Wang; Fuxin Lin; Jun Wu; Yuming Jiao; Yong Cao; Yuanli Zhao; Shuo Wang

    2016-01-01

    Background:Patients who have a cerebral arteriovenous malformation (cAVMs) in the motor cortex can have displaced function. The finding and its relationship to recovery from surgery is not known. Methods:We present the five cases with cAVMs involving precentral knob and/or paracentral lobule and without preoperative motor deficits. We used motor activation areas derived from Functional functional MRI (fMRI) as a region of interesting (ROI) to launch the plasticity of cerebrospinal tracts (CST). All the results were incorporated into the neuronavigation platform for surgical treatment. Intraoperative electric cortical stimulation (ECS) was used to map motor areas. Modified Rankin Scale (mRS) of hands and feets were performed on postoperative day 2, 7 and at month 3, 6 during follow-up period. All the patients suffered from motor deficits regardless of cortical activation patterns. Results:Three patients showed functionally seeded CST in or around the AVM, and were validated by intraoperative electrical stimulation (ECS). Patient 4 had two aberrant functionally seeded fiber tracts away from the lesion, but were proved to be non-functional by postoperative motor deficits. Patient 3 with motor cortex and fiber tract within a diffuse AVMs nidus, complete paralysis of upper extremity after operation and has a persistent motor deficit during 6-month follow-up period. Conclusions:The plasticity of motor cortex on fMRI doesn’t prevent post-operative motor deficits. Functionally mapped fiber tract within or abutting AVM nidus predicts transient and persistent motor deficit.

  18. Surveying the ATLAS cavern

    CERN Multimedia

    Laurent Guiraud

    2000-01-01

    The cathedral-like cavern into which the ATLAS experiment will be lowered and installed forms a vital part of the engineering work at CERN in preparation for the new LHC accelerator. This cavern, being measured by surveyors in these images, will have one of the largest spans of any man-made underground structure. The massive 46X25X25 cubic metre detector will be the largest of its type in the world when it is completed for the LHC start-up in 2008.

  19. Clinical study on CyberKnife for treating giant cavernous hemangioma in cavernous sinus region

    Directory of Open Access Journals (Sweden)

    Lu SUN

    2015-10-01

    Full Text Available Objective To investigate the efficacy and safety of CyberKnife for giant cavernous hemangioma in cavernous sinus. Methods The data of 7 cases of giant cavernous hemangioma in cavernous sinus region confirmed by imaging examination and treated with CyberKnife were collected. The tumor volume was 11.86-70.12 cm3 (median 23.30 cm3. CT and MRI were acquired and fused by MIM 6.5.4 software. The acquired data sets were imported into a CyberKnife Robotic Radiosurgery System (Multiplan 4.0.2 and used to delineate the target organs at risk. The irradiation doses received by the lesions were 21 Gy/3 times in 5 cases, 25 Gy/5 times in one case and 30 Gy/3 times in one case. The exposure field was 109-155, and target volume reached over 95%. Patients maintained at supine positionduring treatment and utilized a 6D-skull trace mode specific to CyberKnife. Changes of clinical symptoms and imaging immediately after treatment and during follow-up period were observed to evaluate the efficacy and safety of this therapeutic method. Results Patients were followed-up for 6-18 months, and the meanfollow-up period was 9.37 months. Volumes of lesions were calculated after operation. All of these patients showed decrease in tumor volume (35.48% to 84.03% and improvement in symptoms (including visual impairment, visual field defect and headaches after therapy. Postoperative MRI revealed the tumor volume was 6.75-28.35 cm3 (median 10.50 cm3, which were significantly lower than that of before treatment [11.86-70.12 cm3 (median 23.30 cm3; Z = -2.366, P = 0.018]. Only one case presented radioactive cerebral edema, and the symptom was disappeared after 5-day treatment with mannitol and dexamethasone. Conclusions CyberKnife is an effective treatment method for giant cavernous hemangioma in cavernous sinus region. It is suggested to be the first selection for patients with giant cavernous hemangioma in cavernous sinus, who are not suitable for traditional surgeries and general

  20. Endovascular treatment of traumatic carotid cavernous fistula with trapping technique

    Directory of Open Access Journals (Sweden)

    Benny Young

    2013-08-01

    Full Text Available Conventional endovascular treatment for carotid cavernous fistula (CCF involves a direct delivery of either coils, detachable balloon or both to the fistula with end point of CCF resolution and carotid artery preservation. But in few cases with severe laceration of carotid artery, the feasible endovascular technique applicable is by blocking the filling of fistula from cerebral circulation. This method known as trapping technique which implicates carotid artery occlusion, was performed in our present case with good result. (Med J Indones. 2013;22:178-82. doi: 10.13181/mji.v22i3.588Keywords: Carotid cavernous fistula (CCF, carotid occlusion, trapping technique

  1. Brain Malformations

    Science.gov (United States)

    Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it ... medicines, infections, or radiation during pregnancy interferes with brain development. Parts of the brain may be missing, ...

  2. Nevus vascularis mixtus (cutaneous vascular twin nevi) associated with intracranial vascular malformation of the Dyke-Davidoff-Masson type in two patients.

    Science.gov (United States)

    Ruggieri, Martino; Milone, Pietro; Pavone, Piero; Falsaperla, Raffaele; Polizzi, Agata; Caltabiano, Rosario; Fichera, Marco; Gabriele, Anna Lia; Distefano, Angela; De Pasquale, Rocco; Salpietro, Vincenzo; Micali, Giuseppe; Pavone, Lorenzo

    2012-11-01

    The term twin spotting refers to phenotypes characterized by the spatial and temporal co-occurrence of two (or more) different nevi arranged in variable cutaneous patterns, and can be associated with extra-cutaneous anomalies. Several examples of twin spotting have been described in humans including nevus vascularis mixtus, cutis tricolor, lesions of overgrowth, and deficient growth in Proteus and Elattoproteus syndromes, epidermolytic hyperkeratosis of Brocq, and the so-called phacomatoses pigmentovascularis and pigmentokeratotica. We report on a 28-year-old man and a 15-year-old girl, who presented with a previously unrecognized association of paired cutaneous vascular nevi of the telangiectaticus and anemicus types (naevus vascularis mixtus) distributed in a mosaic pattern on the face (in both patients) and over the entire body (in the man) and a complex brain malformation (in both patients) consisting of cerebral hemiatrophy, hypoplasia of the cerebral vessels and homolateral hypertrophy of the skull and sinuses (known as Dyke-Davidoff-Masson malformation). Both patients had facial asymmetry and the young man had facial dysmorphism, seizures with EEG anomalies, hemiplegia, insulin-dependent diabetes mellitus (IDDM), autoimmune thyroiditis, a large hepatic cavernous vascular malformation, and left Legg-Calvé-Perthes disease (LCPD) [LCPD-like presentation]. Array-CGH analysis and mutation analysis of the RASA1 gene were normal in both patients.

  3. Penile cavernous hemangioma

    Directory of Open Access Journals (Sweden)

    Abdelmoughit ECHCHAOUI

    2015-06-01

    Full Text Available Hemangiomas are benign lesions that occur in any part of the body in newborns or in young patients, they are typically capillary or cavernous. Cavernous hemangioma of the penis is extremely rare; and its etiology is not completely understood. Treatment options (surgical excision, laser therapy or sclerotherapy… are controversial and are required if pain, cosmetic defect and/or bleeding during intercourse.                                                  We report a case of a 26 years old man presented with a five years history of a painless lesion on his penis. Physical examination showed an ovoid, solid, 1 x 2 mm in size on the dorsum of penis (Panel A and two elevated irregular lesions on the ventral side (Panel B. The lesions were compressive, nonpulsatile and bluish-red in color with strawberry like appearance. Biopsy and histopathological examination of the lesion revealed a gaping and communicating vessels proliferated in the superficial and medium dermis which is pathognomonic for cavernous hemangioma (Panel C and D. Given the small size, the large number of the lesions, the young age, and the ulceration risk during intercourse if skin graft after excision, Neodymium: YAG laser coagulation was recommended to get a good cosmetic results and sexual function satisfaction.

  4. Spontaneous occlusion of traumatic carotid-cavernous fistula - the effect of angiography

    Energy Technology Data Exchange (ETDEWEB)

    Stampfel, G.

    1984-08-01

    In two patients with a traumatic carotid-cavernous fistula, permanent occlusion of the lesion was observed following cerebral angiography and confirmed by further angiography. A delay is therefore recommended between performing angiography and carrying out further treatment, which may carry some risk. Possibly the use of ionic contrast media, which irritate the vessels, compression of the carotid artery, which reduces flow through the fistula, and general anaesthesia, which may drop the blood pressure, initiate thrombosis in the cavernous sinus. 3 figs.

  5. Cavernous hemangioma of the dura mater mimicking meningioma

    Science.gov (United States)

    Di Vitantonio, Hambra; De Paulis, Danilo; Ricci, Alessandro; Marzi, Sara; Dehcordi, Soheila Raysi; Galzio, Renato Juan

    2015-01-01

    Background: Cavernomas are benign lesions that most commonly occur intra-parenchymally, but occasionally they have been described as arising from the dura mater. Extra-axial cavernous angiomas (or hemangiomas) account for 0.4–2% of all intracranial vascular malformations, and they usually occur in the middle cranial fossa, associated with the cavernous sinus. Other possible localizations (e.g. tentorium, convexity, anterior cranial fossa, cerebellopontine angle, Meckel's cave, sella turcica and internal auditory meatus) are rare, and they account only for 0.2–0.5%. Case Description: We report a case of a 30-year-old female presenting with a 2 years history of headache unresponsive to drug therapy. The magnetic resonance imaging showed a dural-based lesion in the left frontal region; the lesion size was: 1.5 cm × 3.5 cm. The appearance suggested a convexity meningioma. A left frontal craniotomy was performed, and the histopathological diagnosis deposed for a cavernous hemangioma of the dura mater. The follow-up at 1-year was good without any neurologic deficit. Conclusions: Dural-based cavernous hemangiomas of the convexity are uncommon lesions. Up to now, only 13 cases have been described in the literature. The authors have discussed clinical aspects, radiological features, surgical treatment, and operative findings. PMID:26421218

  6. Application and value of MRI in the diagnosis of cerebral venous malformation.%脑静脉性血管畸形MRI应用及价值

    Institute of Scientific and Technical Information of China (English)

    王秀兰; 田为中; 李瑗; 陈锦华; 陈小云

    2011-01-01

    Objective To explore the application and evaluation of a variety of imaging sequences of magnetic resonance (MR) imaging in diagnosing cerebral venous malformation (CVM). Methods The distribution of 22 CVM cases and corresponding MR features were retrospectively analyzed. All the patients underwent conventional routine MRI sean and following examinations including 18 cases with extra enhanced TIWI, 8 cases with contrast enhanced magnetic resonance angiography (CE MRA), 3 eases with 3D time of flight magnetic resonance angiography (3D TOFMRA) and 6 cases with susceptibility-weighted imaging (SWI) were processed. Results he conventional routine MRI scans of draining veins were shown as long T1 and long T2 signal intensities except for few short T2 signal intensifies. Most oft. he conventional routine MRI scans of medullary veins were unclear and shown as long T1 and long T2 or iso T2 signal intensities, and after enhancement, several radial medullary veins were dearly shown afflux into bulky drain veins, demonstrating typical “jellyfish head” appearance or “centipede” and “roots shape” changes.The SWI was rather susceptible to CVM with low intensity on draining veins and medullary veins. Conclusion The characteristic representation of CVM can be achieved by combining a variety of imaging methods, which provided significant value in the diagnosis of CVM and assessment of corresponding eomplieatious.%目的 探讨MRI多种成像序列在脑静脉性畸形诊断中的应用及评价.方法 回顾性分析22例CVM分布及MR征象.常规平扫后,其中18例加做增强后TWI扫描,CE-MRA 8例,3D-TOFMRA 3例,SWI 6例.结果 MRI平扫显示引流静脉(或导静脉)通常为长T长T信号影,少数为短T信号;髓静脉为长T、长T或等T信号,大部分显示不佳,增强后可清晰显示多根髓静脉呈放射状汇入粗大的引流静脉,呈典型"水母头"状或"蜈蚣"、"树根状"改变.SWI显示CVM更为敏感,引流静脉和髓

  7. Ceremony for ATLAS cavern

    CERN Multimedia

    2003-01-01

    Wednesday 4 June will be a special day for CERN. The President of the Swiss Confederation, Pascal Couchepin, will officially inaugurate the huge ATLAS cavern now that the civil engineering works have ended. The inauguration ceremony will be held in the ATLAS surface building, with speeches by Pascal Couchepin and CERN, ATLAS and civil engineering personalities. This ceremony will be Webcast live. To access the Webcast on 4 June at 18h00 go to CERN Intranet home page or the following address : http://webcast.cern.ch/live.php

  8. Gastrointestinal malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Loane, Maria; Dolk, Helen;

    2007-01-01

    of mortality for the prenatally diagnosed infants. Clinicians need to balance the risk of early delivery against the benefits of clinical convenience when making case management decisions after prenatal diagnosis. Very few studies have been able to show benefits of prenatal diagnosis of congenital...... malformations for liveborn infants. This may be because the benefits of prenatal diagnosis are outweighed by the problems arising from a lower GA at birth....

  9. A dural arteriovenous fistula in cavernous sinus developed from viral meningitis.

    Science.gov (United States)

    Hai, Jian; Zhang, Lin; Wan, Jue-Feng; Su, Shao-Hua; Wang, Fei; Zhang, Gui-Yun

    2011-06-01

    Although hormonal influences, inflammation, trauma, sinus thrombosis, venous hypertension, and congenital origin have been proposed as sources of dural arteriovenous fistulas (DAVFs) in cavernous and sigmoid sinuses, the etiology of these lesions remains controversial. We present a case with a cavernous sinus DAVF developed from viral meningitis which has not been previously described. A 24-year-old male was admitted to our institute because of periorbital pain, decreased vision, pulsatile tinnitus, chemosis, and exophthalmos on the right side after he had suffered viral meningitis four months before. Cerebral angiography demonstrated a cavernous sinus DAVF, which was successfully obliterated with several platinum coils using a transvenous approach. The viral meningitis most likely caused the inflammation, that may be responsible for the occurrence of the cavernous sinus DAVF. Prompt treatment for inflammation may help to prevent the development of DAVFs.

  10. B超对眶部血管畸形的诊断%B-ultrasound diagnosis in ophthalmic orbital vascular malformation

    Institute of Scientific and Technical Information of China (English)

    穆秀云; 蔡晓松; 刘杰

    2008-01-01

    Objective To explore the B-ultrasonographic diagnostie value in orbital vascular malformations Methods Features of orbital vascular malformations verified with B-ultrasonography specially for ophthalmology and other assistant exarnination were analyzed retrospectively in 21 cases.Results In 21 cases with orbital vascular malformation,carotid-cavernous sinus fistuls were in 12 cases,11 in whom were examined with cerebral angiography,1 scanned with NMR because of too young,9 with orbital varix scanned with color Doppler and CT and all the 21 cases were verified.Conclusions Ultrasonography iconological features of orbital vascular malformation were typical.it is a valuable assistant examination method of other examination.%目的 探讨眼科专用B超对眶部血管畸形的诊断价值.方法 回顾分析了对眼科专用B超诊断并经过其他辅助检查证实的21例眶部血管畸形患者声像图特征.结果 21例眶部血管畸形,颈动脉海绵窦瘘12例,其中11例行脑血管造影检查,1例因患儿太小行核磁共振检查.眶静脉曲张9例,均行彩色多普勒超声、CT检查得到证实.结论 眼眶部血管畸形具有较典型的超声图像特征,B超是一种有价值的辅助检查

  11. Extradural spinal cavernous angiomas: report of seven cases.

    Science.gov (United States)

    Santoro, Antonio; Piccirilli, Manolo; Bristot, Roberto; di Norcia, Valerio; Salvati, Maurizio; Delfini, Roberto

    2005-10-01

    The authors describe seven cases of extradural spinal cavernous angioma. Although cavernoma itself is not rare, the extradural spinal localization is uncommon and makes preoperative differential diagnosis difficult. Routine MRI investigation has aided neurosurgeons in evaluating the true incidence of these vascular malformations, which was understimated in the past. The data published so far have not entirely clarified the treatment of choice for these lesions. Considering their rarity in this site, their presenting symptoms and the difficulties involved in neuroradiological diagnosis, the authors discuss the role of surgery as the principal form of treatment and review the relevant literature. Seven patients (4 male, 3 female) were admitted to our Institute of Neurosurgery between 1992 and 2004, with a 5-6 month history (range=2-365 days) of low back pain or radicular pain, sometimes associated with paresthesia. All patients had a CT scan, as well as MRI with gadolinium when possible, which detected an extradural roundish lesion: differential diagnosis was very difficult, especially between neurinoma and cavernoma. Treatment was always surgical and resection of the lesion radical. Postoperatively, all patients presented complete regression of clinical symptoms. In all cases histological diagnosis was cavernous angioma. Postoperative MRI with gadolinium or CT scan with IV contrast, performed before discharge, confirmed radical removal of the vascular malformation in all cases. Our experience confirms that surgery should be the treatment of choice for these lesions, in view of both their tendency to bleed and their straightforward surgical removal.

  12. The huge ATLAS cavern now fully excavated

    CERN Multimedia

    2002-01-01

    Excavation of the ATLAS cavern is now complete! At the end of two years' work involving a tremendous technical challenge, the civil engineering contractors have succeeded in digging out one of the biggest experimental caverns in the world. Bravo!

  13. ATLAS Cavern - Sainte-Barbe evening

    CERN Multimedia

    2002-01-01

    The December at cavern of ATLAS was full of surprises, while during the iron mounting and concrete work the cavern got its new purpose for being the restaurant under little while -Live music and happy people.

  14. Surgical Treatment of Cerebral Cavernous Malformation Presenting Epileptic Seizures%以癫痫发作为主的脑内海绵状血管畸形的手术治疗

    Institute of Scientific and Technical Information of China (English)

    周峰; 吴俊; 彭磊; 王硕; 桑林; 郑重; 解飞; 葛留锁; 马延山

    2014-01-01

      结论对于合并癫痫的海绵状血管畸形患者应早期进行手术。术中应完整切除海绵状血管畸形病灶(包括病灶周边的胶质瘢痕层和含铁血黄素层),同时行皮层热灼术。

  15. Carotid cavernous fistula: Ophthalmological implications

    Directory of Open Access Journals (Sweden)

    Chaudhry Imtiaz

    2009-01-01

    Full Text Available Carotid cavernous fistula (CCF is an abnormal communication between the cavernous sinus and the carotid arterial system. A CCF can be due to a direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus, or a communication between the cavernous sinus, and one or more meningeal branches of the internal carotid artery, external carotid artery or both. These fistulas may be divided into spontaneous or traumatic in relation to cause and direct or dural in relation to angiographic findings. The dural fistulas usually have low rates of arterial blood flow and may be difficult to diagnose without angiography. Patients with CCF may initially present to an ophthalmologist with decreased vision, conjunctival chemosis, external ophthalmoplegia and proptosis. Patients with CCF may have predisposing causes, which need to be elicited. Radiological features may be helpful in confirming the diagnosis and determining possible intervention. Patients with any associated visual impairment or ocular conditions, such as glaucoma, need to be identified and treated. Based on patient′s signs and symptoms, timely intervention is mandatory to prevent morbidity or mortality. The conventional treatments include carotid ligation and embolization, with minimal significant morbidity or mortality. Ophthalmologist may be the first physician to encounter a patient with clinical manifestations of CCF, and this review article should help in understanding the clinical features of CCF, current diagnostic approach, usefulness of the available imaging modalities, possible modes of treatment and expected outcome.

  16. Cavernous sinus cavernous hemangioma largely extending into the sella turcica and mimicking pituitary adenoma: case report.

    Science.gov (United States)

    Hori, Satoshi; Hayashi, Nakamasa; Nomoto, Kazuhiro; Sato, Hikari; Hayashi, Tomohide; Nagai, Shoichi; Nishikata, Manabu; Endo, Shunro

    2010-01-01

    A 77-year-old female presented with a rare cavernous sinus cavernous hemangioma with extension to the sella turcica, neuroradiologically mimicking nonfunctioning pituitary macroadenoma. The lesion was partially removed via transsphenoidal surgery, and the histological diagnosis was cavernous hemangioma. After stereotactic radiosurgery using a cyber knife, the lesion decreased in size. Stereotactic radiosurgery may be a good option for cavernous sinus cavernous hemangioma with high risk of surgical bleeding.

  17. Malformations of cortical development: genetic mechanisms and diagnostic approach

    Science.gov (United States)

    2017-01-01

    Malformations of cortical development are rare congenital anomalies of the cerebral cortex, wherein patients present with intractable epilepsy and various degrees of developmental delay. Cases show a spectrum of anomalous cortical formations with diverse anatomic and morphological abnormalities, a variety of genetic causes, and different clinical presentations. Brain magnetic resonance imaging has been of great help in determining the exact morphologies of cortical malformations. The hypothetical mechanisms of malformation include interruptions during the formation of cerebral cortex in the form of viral infection, genetic causes, and vascular events. Recent remarkable developments in genetic analysis methods have improved our understanding of these pathological mechanisms. The present review will discuss normal cortical development, the current proposed malformation classifications, and the diagnostic approach for malformations of cortical development. PMID:28203254

  18. Prospective comparison of late 3T MRI with conventional angiography in evaluating the patency of cerebral arteriovenous malformations treated with stereotactic radiosurgery

    Energy Technology Data Exchange (ETDEWEB)

    Khandanpour, Nader [National Hospital for Neurology and Neurosurgery, London (United Kingdom); Griffiths, Paul; Hoggard, Nigel [University of Sheffield, Academic Unit of Radiology, C Floor, Royal Hallamshire Hospital, Sheffield (United Kingdom); Warren, Daniel [University of Sheffield, C Floor, Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2013-06-15

    Risk of further haemorrhage in patients suffering from arteriovenous malformation (AVM) would be eliminated only if complete obliteration of the AVM is obtained. Therefore, these patients frequently need long-term follow-up. Conventional catheter angiography (CCA) with a risk of 0.5 %.to 1.6 % of significant neurological complications has traditionally been used for this purpose. However, magnetic resonance imaging (MRI) at 3T may be a safer alternative. The aim of this study was to evaluate if MRI at 3T can accurately evaluate closure of AVM in 2 years after stereotactic radiosurgery. Twenty-three patients with both MRI at 3T and a CCA study were examined. The residual AVMs were evaluated by MRI at 3T against CCA in a prospective study. The time interval between radiosurgery and neuroimaging was on average of 25 months (range, 15-30 months) for MRI study and 33 months (range, 25-46 months) for CCA study. Ten patients showed closure of the AVM on MRI, all of which were confirmed on CCA. There was a complete agreement between late MRI at 3T scan and CCA in evaluation of AVM patency. (orig.)

  19. Anorectal malformations

    Directory of Open Access Journals (Sweden)

    Peña Alberto

    2007-07-01

    Full Text Available Abstract Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life.

  20. Digging the CMS experimental cavern

    CERN Multimedia

    Laurent Guiraud

    2001-01-01

    The huge CMS experimental cavern is located 100 m underground and has two access shafts through which the experiment's components will be lowered. Initially assembled on the surface, each part of the 12 500 tonne machine must be lowered individually with very little clearance.

  1. Cavernous Sinus Thrombosis in Children

    Directory of Open Access Journals (Sweden)

    Rochelle Sweis

    2016-03-01

    Full Text Available Investigators from the Children’s Hospital of Philadelphia analyzed the clinical and radiographic findings in 12 cases of cavernous sinus thrombosis (CST seen between 2000 and 2013, and conducted a literature search and review of the pooled data.

  2. Analysis on Diagnosis Results of Cerebral Arteriovenous Malformation by dual Phase Cerebrovascular Imaging Interventional Treatment%16层螺旋CT双期脑血管成像诊断脑动静脉畸形介入治疗结果分析

    Institute of Scientific and Technical Information of China (English)

    郝绪滨

    2014-01-01

    Objective To study the application value of 16 layer spiral CT dual phase cerebrovascular imaging interventional treatment application value in diagnosis of cerebral arteriovenous malformations,analysis the cerebral imaging results of interventional therapy and scanning characteristics.Methods Selecting 29 cases diagno-sised as cerebral arteriovenous malformation by DSA in our hospital neurosurgery,they were given 16 layer spiral CT dual phase cerebrovascular intervention treatment, analysis the imaging results.Results 16 layer spiral CT scan results suggest that cerebral arteries venous malformations are 28 cases,1 case was missed diagnosis.Conclu-sion 16 layer spiral CT dual phase cerebrovascular imaging interventional treatment has the advantages of noninvasive ,convenient operation,short time consuming,low cost,high resolution,good 3-d imaging effect and so on,which has high diagnosis rate in patients with cerebral arteriovenous malformation ,so it is worthy of populariza-tion and application.%目的:探讨研究16层螺旋CT双期脑血管成像在诊断脑动静脉畸形中的应用价值,分析脑血管成像介入治疗的结果及扫描特征。方法选取我院神经外科经DSA明确诊断为脑动静脉畸形的患者29例,给予16层螺旋CT双期脑血管成像介入治疗,分析所得图像结果。结果16层螺旋CT扫描结果提示,脑动脉静脉畸形者28例,有1例漏诊。结论16层螺旋CT双期脑血管成像介入治疗具有无创、操作简便、耗时短、费用低、分辨率高、三维成像效果好等优点,对脑动静脉畸形患者确诊率高,值得推广应用。

  3. Transvenous embolization of a dural carotid-cavernous sinus fistula via the inferior ophthalmic vein.

    Science.gov (United States)

    Michels, Kevin S; Ng, John D; Falardeau, Julie; Roberts, Warren G; Petersen, Bryan; Nesbit, Gary M; Barnwell, Stanley L

    2007-01-01

    A 76-year-old woman presented with an acute onset of right periocular pain, diplopia, ocular injection, progressive proptosis, and periocular swelling. She had an unremarkable past medical history, and the erythrocyte sedimentation rate and complete blood count were normal. A carotid-cavernous sinus fistula was suspected, and an MRI demonstrated enlargement of the superior ophthalmic vein posterior to the globe and enlargement of the inferior ophthalmic vein throughout its entire course. Cerebral arteriography demonstrated a dural cavernous sinus fistula. The inferior ophthalmic vein was accessed via the inferonasal orbital space and was catheterized for delivery of multiple platinum coils to the cavernous sinus fistula. Follow-up venograms demonstrated occlusion of the fistula. At 2-month follow-up, there was a residual sixth nerve palsy and resolution of symptoms, including proptosis and periocular swelling.

  4. 3D磁敏感加权成像在脑静脉畸形诊断中的价值%The role of 3D susceptibilitv weighted imaging in the evaluation of cerebral venous malformation

    Institute of Scientific and Technical Information of China (English)

    祁鸣; 王德杭

    2011-01-01

    Objective To discuss the value of 3D susceptibility weighted imaging(SWI) in the diagnosis of cerebral venous malformation (CVM). Methods Thirty CVM patients were examined with a 3. OT MRI scanner. The sequences included the conventional MRI, 3D SWI and contrast enhanced T1WL Comparisons were made among SWI, contrast enhanced T1W1 and conventional sequences. The image quality of CVM was graded. Results SWI could demonstrate conspicuously both numerous deep medullary veins and collector veins as the contrast enhanced MRI did, which were hardly visible on Tl-weighted images or T2-weighted images. Conclusion SWI is a sensitive sequence for detecting CVMs. It allows better visualization of CVMs without contrast agents.%目的 探讨3D磁敏感加权成像(SWI)技术对脑静脉畸形(CVM)的诊断价值.方法 30例CVM形患者采用3.0T磁共振常规MRI序列(T1WI、T2WI)、3D SWI及T1WI增强扫描,根据CVM血管显影情况对常规MRI序列、3D SWI及T1WI增强扫描进行分级比较.结果 SWI能显著显示常规T1WI、T2WI很难显示的髓静脉及引流静脉,并与增强扫描图像有很好的对应.结论 SWI是检出CVM较为敏感的序列,在不用造影剂情况下使静脉畸形血管获得较好的显示.

  5. 辅助技术在颅内动静脉畸形手术中的应用%Strategy for assisted cerebral arteriovenons malformation surgery

    Institute of Scientific and Technical Information of China (English)

    王硕; 刘泠; 赵元立; 张东; 王嵘; 赵继宗

    2010-01-01

    目的 探讨B超、神经导航以及吲哚菁绿血管造影在颅内动静脉畸形手术中的应用价值.方法 回顾性分析2009年1至10月北京天坛医院神经外科脑血管病区收治的43例颅内动静脉畸形患者临床资料.其中男26例,女17例;年龄8~53岁,平均26.51岁.所有患者在手术中分别或联合采用B超、神经导航或吲哚菁绿血管造影辅助病灶切除,且术后全部病例行脑血管造影(DSA)或CTA检查,同时记录术后新发并发症及恢复情况.结果 所有患者均全切病灶,术中辅助技术检测结果均与术后DSA或CTA吻合.术后新发神经功能障碍3例(7.0%),其中2例出现双侧视野同向性偏盲,另1例为复视.43例患者KPS(Karnofsky Performance Scale)评分均高于80分.结论 采用B超、神经导航或吲哚菁绿血管造影技术辅助颅内动静脉畸形手术切除,均有助于鉴别血管性质和反映病灶边界.但其侧重各有不同.因此,该3种辅助技术有选择的联合应用可以使术者增加术中判断的准确性,从而收到最佳手术效果.%Objective To evaluate the surgical effects of such adjuvant techniques as type B ultrasound, neuronavigation and intraoperative indocyanine green vedioangiography (ICGA) on intracranial arteriovenous malformation ( AVM) surgery. Methods From January to October 2009, 43 AVM patients treated at our center were retrospectively studied, including 26 males and 17 females with an age range of 8 to S3 years old (mean; 26. 51). Type B ultrasound, neuronavigation or ICCA were employed during surgical procedures in all cases. Intraoperative findings were compared with those of postoperative digital subtraction angiography ( DSA) or computed tomography angiography (CTA) . And the clinical outcomes were evaluated. Results As confirmed by postoperative DSA or CTA, the lesions of all 43 patients were totally removed. Only 3 patients (7.0%) suffered from new neurological deficits, including homonymous hemianopia

  6. Reinforcement of the concrete base slab of the ATLAS cavern

    CERN Multimedia

    Maximilien Brice

    2002-01-01

    Photo 02: UX15 cavern, preparation for concreting of base slab first lift. Photo 05: UX15 cavern, placing of reinforcement for base slab first lift. Photo 07: UX15 cavern, preparation for concreting of base slab first lift. Photo 09: UX15 cavern, placing of reinforcement for base slab first lift. Photo 10: UX15 cavern, view into PX14 shaft above. Photo 12: UX15 cavern, temporary access platform of RB16 tunnel. Photo 15: UJ17 chamber, invert excavation.

  7. Civil engineering in the ATLAS cavern

    CERN Multimedia

    Laurent Guiraud

    2000-01-01

    Work continues in the cathedral-like cavern that will soon contain ATLAS, the largest particle detector of its type in the world. For such a huge detector, an equally giant cavern must be excavated 100 m underground. The roof must be held without any normal rests at the base; instead it will be supported by huge anchors embedded in concrete that will stop the roof from caving in, located in galleries above the cavern.

  8. Twenty-seven years follow-up of a patient with congenital retinocephalofacial vascular malformation syndrome and additional congenital malformations (Bonnet-dechaume-blanc syndrome or wyburn-mason syndrome

    Directory of Open Access Journals (Sweden)

    Schmidt D

    2010-02-01

    Full Text Available Abstract Purpose Follow-up of vascular changes in a patient with congenital retinocephalofacial vascular malformation syndrome. Methods MRI and cerebral angiography. Results In a 36-year-old man, magnetic resonance im aging of the skull and cerebral angiography revealed left intracranial arteriovenous malformations. Follow-up observation of 27 years revealed no essential change of retinal and cerebral arteriovenous malformations. Additional congenital deficits in this patient were described. Conclusion Patients with retinal arteriovenous malformations should be early examined with neuroradiological methods.

  9. What the Neurosurgeon needs to know about Cerebral Developmental Venous anomalies

    Directory of Open Access Journals (Sweden)

    Alvis-Miranda Hernando Raphael

    2014-06-01

    Full Text Available Venous Angiomas or Developmental venous anomalies (DVA are extreme variations of normal transmedullary veins that are necessary for the drainage of white and gray matter, also are one type of cerebrovascular malformation (CVM, sharing category with capillary telangiectesias, cavernous malformations (CM, and arteriovenous malformations (AVM, each of which may also be associated with a DVA. DVA are the most commonly encountered CVM, accounting for up to 60% of all CVM. We present a review of the literatura

  10. Spontaneous Resolution of Direct Carotid Cavernous Fistula

    NARCIS (Netherlands)

    Ishaq, Mazhar; Arain, Muhammad Aamir; Ahmed, Saadullah; Niazi, Muhammad Khizar; Khan, Muhammad Dawood; Iqbal, Zamir

    2010-01-01

    Proptosis due to carotid cavernous fistula is rare sequelae of head injury. We report a case of post-traumatic, direct high flow carotid cavernous fistula that resolved spontaneously 06 weeks after carotid angiography. It however, resulted in loss of vision due to delay in early treatment. In the ca

  11. A 12-year cavern abandonment test

    Directory of Open Access Journals (Sweden)

    Brouard B.

    2010-06-01

    Full Text Available In 1997-1998, an abandonment test was performed in a 950-m deep, 8000-m3 salt cavern operated by GDF SUEZ at Etrez, France. In this relatively small brine-filled cavern, which had been kept idle for 15 years before the test, thermal equilibrium was reached. A special system was designed to monitor leaks, which proved to be exceedingly small. In these conditions, brine permeation and cavern creep closure are the only factors to play significant roles in pressure evolution. This test strongly suggested that obtaining an equilibrium pressure such that the effects of these two factors were exactly equal would be reached in the long term. Four years later, pressure monitoring in the closed cavern resumed. Pressure evolution during the 2002-2009 period confirmed that cavern brine pressure will remain constant and significantly smaller than geostatic pressure in the long term, precluding any risk of fracturing and brine seepage to the overburden layers.

  12. Noncavernous arteriovenous shunts mimicking carotid cavernous fistulae

    Science.gov (United States)

    Kobkitsuksakul, Chai; Jiarakongmun, Pakorn; Chanthanaphak, Ekachat; Singhara Na Ayudya, Sirintara (Pongpech)

    2016-01-01

    PURPOSE The classic symptoms and signs of carotid cavernous sinus fistula or cavernous sinus dural arteriovenous fistula (AVF) consist of eye redness, exophthalmos, and gaze abnormality. The angiography findings typically consist of arteriovenous shunt at cavernous sinus with ophthalmic venous drainage with or without cortical venous reflux. In rare circumstances, the shunts are localized outside the cavernous sinus, but mimic symptoms and radiography of the cavernous shunt. We would like to present the other locations of the arteriovenous shunt, which mimic the clinical presentation of carotid cavernous fistulae, and analyze venous drainages. METHODS We retrospectively examined the records of 350 patients who were given provisional diagnoses of carotid cavernous sinus fistulae or cavernous sinus dural AVF in the division of Interventional Neuroradiology, Ramathibodi Hospital, Bangkok between 2008 and 2014. Any patient with cavernous arteriovenous shunt was excluded. RESULTS Of those 350 patients, 10 patients (2.85%) were identified as having noncavernous sinus AVF. The angiographic diagnoses consisted of three anterior condylar (hypoglossal) dural AVF, two traumatic middle meningeal AVF, one lesser sphenoid wing dural AVF, one vertebro-vertebral fistula (VVF), one intraorbital AVF, one direct dural artery to cortical vein dural AVF, and one transverse-sigmoid dural AVF. Six cases (60%) were found to have venous efferent obstruction. CONCLUSION Arteriovenous shunts mimicking the cavernous AVF are rare, with a prevalence of only 2.85% in this series. The clinical presentation mainly depends on venous outflow. The venous outlet of the arteriovenous shunts is influenced by venous afferent-efferent patterns according to the venous anatomy of the central nervous system and the skull base, as well as by architectural disturbance, specifically, obstruction of the venous outflow. PMID:27767958

  13. Cerebral abscesses among Danish patients with hereditary haemorrhagic telangiectasia

    DEFF Research Database (Denmark)

    Kjeldsen, A D; Tørring, P M; Nissen, H;

    2013-01-01

    Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs), which due to paradoxical embolization may cause cerebral abscess.......Hereditary haemorrhagic telangiectasia (HHT) is a dominantly inherited disease characterized by a wide variety of clinical manifestations, including pulmonary arteriovenous malformations (PAVMs), which due to paradoxical embolization may cause cerebral abscess....

  14. EVOKED CAVERNOUS ACTIVITY: NEUROANATOMIC IMPLICATIONS

    Science.gov (United States)

    Yilmaz, Ugur; Vicars, Brenda; Yang, Claire C.

    2013-01-01

    We investigated the autonomic innervation of the penis by using evoked cavernous activity (ECA). We recruited 7 males with thoracic spinal cord injury (SCI) and sexual dysfunction and 6 males who were scheduled to have pelvic surgery (PS), specifically non-nerve-sparing radical cystoprostatectomy. In the PS subjects, ECA was performed both pre- and postoperatively. The left median nerve was electrically stimulated and ECA was recorded with two concentric electromyography needles placed into the right and left cavernous bodies. We simultaneously recorded hand and foot sympathetic skin responses (SSRs) as controls. In the SCI group, all but one subject had reproducible hand SSRs. None of these subjects had ECA or foot SSRs. All the PS subjects had reproducible ECA and SSRs, both preoperatively and postoperatively. There was no difference in the latency and amplitude measurements of ECA and SSRs in the postoperative compared to the preoperative period (p>0.05). In conclusion, ECA is absent in men with SCI above the sympathetic outflow to the genitalia. In men following radical pelvic surgery, ECA is preserved, indicating the preservation of sympathetic fibers. PMID:19609298

  15. Operative management of tumors involving the cavernous sinus.

    Science.gov (United States)

    Sekhar, L N; Møller, A R

    1986-06-01

    In the past, neurosurgeons have been reluctant to operate on tumors involving the cavernous sinus because of the possibility of bleeding from the venous plexus or injury to the internal carotid artery (ICA) or the third, fourth, or sixth cranial nerves. The authors describe techniques for a more aggressive surgical approach to neoplasms in this area that are either benign or locally confined malignant lesions. During the last 2 years, seven tumors involving the cavernous sinus have been resected: six totally and one subtotally. The preoperative evaluation included axial and coronal computerized tomography, cerebral angiography, and a balloon-occlusion test of the ICA. Intraoperative monitoring of the third, fourth, sixth, and seventh cranial nerves was used to assist in locating the nerves and in avoiding injury to them. The first major step in the operative procedure was to obtain proximal control of the ICA at the petrous apex and distal control in the supraclinoid segment. The cavernous sinus was then opened by a lateral, superior, or inferior approach for tumor resection. Temporary clipping and suture of the ICA was necessary in one patient. None of the patients died or suffered a stroke postoperatively. Permanent trigeminal nerve injury occurred in three patients; in two, this was the result of tumor invasion. One patient suffered temporary paralysis of the third, fourth, and sixth cranial nerves, and in another the sixth cranial nerve was temporarily paralyzed. Preoperative cranial nerve deficits were improved postoperatively in three patients. Radiation therapy was administered postoperatively to four patients. These seven patients have been followed for 6 to 18 months to date and none has shown evidence of recurrence of the intracavernous tumor.

  16. Rock cavern storage of spent fuel

    Energy Technology Data Exchange (ETDEWEB)

    Cho, Won Jin; Kim, Kyung Soo [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of); Kwon, Sang Ki [Inha University, Incheon (Korea, Republic of)

    2015-12-15

    The rock cavern storage for spent fuel has been assessed to apply in Korea with reviewing the state of the art of the technologies for surface storage and rock cavern storage of spent fuel. The technical feasibility and economic aspects of the rock cavern storage of spent fuel were also analyzed. A considerable area of flat land isolated from the exterior are needed to meet the requirement for the site of the surface storage facilities. It may, however, not be easy to secure such areas in the mountainous region of Korea. Instead, the spent fuel storage facilities constructed in the rock cavern moderate their demands for the suitable site. As a result, the rock cavern storage is a promising alternative for the storage of spent fuel in the aspect of natural and social environments. The rock cavern storage of spent fuel has several advantages compared with the surface storage, and there is no significant difference on the viewpoint of economy between the two alternatives. In addition, no great technical difficulties are present to apply the rock cavern storage technologies to the storage of domestic spent fuel.

  17. Split Cord Malformations

    Directory of Open Access Journals (Sweden)

    Yurdal Gezercan

    2015-06-01

    Full Text Available Split cord malformations are rare form of occult spinal dysraphism in children. Split cord malformations are characterized by septum that cleaves the spinal canal in sagittal plane within the single or duplicated thecal sac. Although their precise incidence is unknown, split cord malformations are exceedingly rare and represent %3.8-5 of all congenital spinal anomalies. Characteristic neurological, urological, orthopedic clinical manifestations are variable and asymptomatic course is possible. Earlier diagnosis and surgical intervention for split cord malformations is associated with better long-term fuctional outcome. For this reason, diagnostic imaging is indicated for children with associated cutaneous and orthopedic signs. Additional congenital anomalies usually to accompany the split cord malformations. Earlier diagnosis, meticuolus surgical therapy and interdisciplinary careful evaluation and follow-up should be made for good prognosis. [Cukurova Med J 2015; 40(2.000: 199-207

  18. Occlusion of pulmonary arteriovenous malformations by use of vascular plug

    DEFF Research Database (Denmark)

    Andersen, P E; Kjeldsen, A D

    2007-01-01

    Pulmonary arteriovenous malformations are commonly treated by embolization with coils or balloons to prevent cerebral complications and to raise the oxygenation of the blood. The Amplatzer vascular plug is a new occlusive device made of a self-expanding cylindrical nitinol mesh. It is fast and safe...

  19. Civil Engineering in the ATLAS cavern

    CERN Multimedia

    Laurent Guiraud

    2000-01-01

    Ghostly figures can be seen wandering the cavern that will eventually house the ATLAS experiment, part of the LHC at CERN. Quite fitting since the detector will hunt the illusive 'ghostly' particles, such as the Higgs boson and dark matter. These engineers are excavating the huge cavern that has to be anchored from above as the detector will fill so much space that there is no room for support pillars.

  20. End of construction of the CMS cavern

    CERN Multimedia

    Maximilien Brice

    2005-01-01

    View of the CMS cavern with its impressive dimensions: 53 m long, 27 m wide and 24 m high. The construction of this underground complex has been a spectacular feat of engineering. This second of the new caverns for the LHC experiments is the result of six-and-a-half years of work, and its completion marks the end of the large-scale engineering work for the LHC.

  1. Digging a cavern for a titan

    CERN Multimedia

    Patrice Loiez

    1999-01-01

    Civil engineers work 100 m underground near the France-Swiss border on the cavern that will soon house ATLAS, one of the experiments on CERN's new LHC accelerator. All personnel and equipment must be lowered by crane down the access shaft. When completed this cavern will have one of the largest spans constructed at 35 m, which required the roof to be supported by large steel anchors buried in concrete.

  2. Pulmonary cavernous hemangioma:a case report

    Institute of Scientific and Technical Information of China (English)

    Chunyi Jia; Shaolin Sun; Xiaokai Zhang; Lixin Zhang

    2014-01-01

    We report a rare case of pulmonary cavernous hemangioma in a 51-year-old female. A computed tomographic scan of the chest showed an il-defined mass measuring 2.3 cm × 2.2 cm in the right lower lobe. Surgical resection was per-formed and postoperative histological examination revealed cavernous hemangioma. We reviewed the clinical features and therapeutic methods of hemangioma.

  3. Demonstration of cerebral vessels by multiplane computed cerebral angiotomography

    Energy Technology Data Exchange (ETDEWEB)

    Asari S.; Satch, T.; Sakurai, M.; Yamamoto, Y. (Matsuyama Shimin Hospital, Matsuyama (Japan)); Sadamoto, K.

    1981-06-01

    1. Cerebral arteries and veins were demonstrated by multiplane computed cerebral angiotomography (combination of axial, modified coronal, half axial (Towne), and semisagittal planes). The vessels which were demonstrated by various planes were as follows: Axial plane: Willis ring, middle cerebral arteries (horizontal and insular portions), anterior cerebral arteries (Horizontal and ascending portions), posterior cerebral arteries, basal vein of Rosenthal, internal cerebral veins (and the subependymal veins which join the ICV), and vein of Galen. Coronal plane: intermal carotid arteries (supraclinoid portion), anterior cerebral arteries (horizontal portion), middle cerebral arteries (horizontal and insular portions), lenticulostriate arteries, basal vein of Rosenthal (and the subependymal veins which join this vessel), internal cerebral veins, and vein of Galen. Half axial plane (Towne projection): basilar artery, vertebral arteries, posterior cerebral arteries, superior cerebellar arteries, middle cerebral arteries (horizontal portion), and anterior cerebral arteries (horizontal and ascending portions). Semisagittal plane: internal carotid artery (supraclinoid portion), posterior communicating artery, posterior carebral artery, superior cerebellar artery, internal cerebral vein, basal vein of Rosenthal, vein of Galen, and straight shinus. 2. A detailed knowledge of normal cerebrovascular structures acquired by computed tomography (CT) is essential in detecting and more precisely localizing lesions such as cerebrovascular disease, neoplasm or abscess, in differentiating these lesions from the normal contrast-enhanced structures, and in understanding the spatial relationship between the mass lesion and the neighboring vessels. In addition, it will be possible to discover such asymptomatic cerebrovascular diseases as non-ruptured aneurysms, arteriovenous malformations, and Moyamoya disease by means of computed cerebral angiotomography.

  4. Congenital Vascular Malformation

    Science.gov (United States)

    ... also be effective for small, localized birthmarks (port wine stains). Patients with a rare venous malformation (Kleppel– ... 3) non-profit organization focused on providing public education and improving awareness about vascular diseases. For more ...

  5. [Neuro-ophthalmology and interventional neuro-radiology--co-treatment for carotid cavernous sinus fistula].

    Science.gov (United States)

    Platner, Eva; Bakon, Mati; Huna-Baron, Ruth

    2013-02-01

    Carotid-cavernous fistulas (CCFs) are abnormal arteriovenous communications in the cavernous sinus. In many cases of CCF's the primary signs are ocular manifestations, which include: pulsatile proptosis, orbital bruit, chemosis and conjunctival injection, elevated intraocular pressure, venous stasis retinopathy, and cranial nerve pareses. Patients in whom the fistula causes arterial drainage into the cerebral veins and sinuses are at risk for intracranial hemorrhage. The most common treatment for CCF's is endovascular occlusion of the lesion. The goal of this procedure is to occlude the fistula but preserve the patency of the internal carotid artery. The CCF itself, as well as its treatment, can be sight- and even life-threatening. We describe 3 case reports of patients with CCF, in order to demonstrate the cooperation between the neuro-opthalmologist and the invasive neuro-radiologist, in the follow-up of the patient and in the treatment timing decision.

  6. 荧光造影和B超在脑浅表动静脉畸形手术中的联合应用%Intraoperative integration of B-ultrasound and indocyanine green angiography in the surgery of superficial cerebral arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    王硕; 赵洪洋; 刘泠; 赵元立

    2011-01-01

    Objective To evaluate the surgical effect of intraoperative combined use of B-ultrasound and indocyanine green angiography (ICGA) in the surgery of superficial cerebral arteriovenous malformations (AVMs). Methods Sixteen patients with superficial cerebral AVMs,admitted to our hospital from January 2009 to December 2009, were performed surgery with the help of intraoperative B-ultrasound and ICGA; the clinical data of these patients were retrospectively collected.The ability of intraoperative B-ultrasound and ICGA in distinguishing AVMs' vessels (draining veins,feeding and nidal arteries) from each other and from normal vessel was both evaluated.Results The combination of B-ultrasound and ICGA allowed accurate and real-time localization of the AVMs' nidus,helped to define the boundary and identify the draining veins and feeding arteries. Total lesion removal was achieved by 16 successful surgeries in 16 patients, which was confirmed by postoperative DSA.Conclusion The combination of B-ultrasound and ICGA in the surgery of superficial cerebral arteriovenous malformations allows accurate and real-time localization of the AVMs' indus and helps to assess the completeness of lesion removal, indicating its value in the surgery of superficial cerebral AVMs.%目的 探讨B超和吲哚菁绿血管造影(ICGA)在脑浅表动静脉畸形(AVMs)手术中的应用价值.方法 回顾性分析自2009年1月至12月北京天坛医院神经外科血管组联合应用B超和ICGA辅助切除的16例脑浅表AVMs患者临床资料,同时分析2种术中辅助技术对AVMs定位、边界确定及血管类型鉴别的作用.结果 术中联合应用ICGA和B超能有效帮助定位AVMs,确定其边界,帮助辨认供血动脉和引流静脉.16例脑浅表AVMs患者共行开颅手术16次,均全切病灶,手术后经DSA证实AVMs无残留.结论 脑浅表AVMs手术中联合应用ICGA和B超能有效帮助准确切除病灶,判断有无畸形残留,具有较高的临床应用价值.

  7. Analysis of cavern stability at the West Hackberry SPR site.

    Energy Technology Data Exchange (ETDEWEB)

    Ehgartner, Brian L.; Sobolik, Steven Ronald

    2009-05-01

    This report presents computational analyses that simulate the structural response of caverns at the Strategic Petroleum Reserve (SPR) West Hackberry site. The cavern field comprises 22 caverns. Five caverns (6, 7, 8, 9, 11) were acquired from industry and have unusual shapes and a history dating back to 1946. The other 17 caverns (101-117) were leached according to SPR standards in the mid-1980s and have tall cylindrical shapes. The history of the caverns and their shapes are simulated in a three-dimensional geomechanics model of the site that predicts deformations, strains, and stresses. Future leaching scenarios corresponding to oil drawdowns using fresh water are also simulated by increasing the volume of the caverns. Cavern pressures are varied in the model to capture operational practices in the field. The results of the finite element model are interpreted to provide information on the current and future status of subsidence, well integrity, and cavern stability. The most significant results in this report are relevant to Cavern 6. The cavern is shaped like a bowl with a large ceiling span and is in close proximity to Cavern 9. The analyses predict tensile stresses at the edge of the ceiling during repressuization of Cavern 6 following workover conditions. During a workover the cavern is at low pressure to service a well. The wellhead pressures are atmospheric. When the workover is complete, the cavern is repressurized. The resulting elastic stresses are sufficient to cause tension around the edge of the large ceiling span. With time, these stresses relax to a compressive state because of salt creep. However, the potential for salt fracture and propagation exists, particularly towards Cavern 9. With only 200 ft of salt between the caverns, the operational consequences must be examined if the two caverns become connected. A critical time may be during a workover of Cavern 9 in part because of the operational vulnerabilities, but also because dilatant damage is

  8. Analysis of cavern stability at the Bryan Mound SPR site.

    Energy Technology Data Exchange (ETDEWEB)

    Ehgartner, Brian L.; Sobolik, Steven Ronald

    2009-04-01

    This report presents computational analyses that simulate the structural response of caverns at the Strategic Petroleum Reserve Bryan Mound site. The cavern field comprises 20 caverns. Five caverns (1, 2, 4, and 5; 3 was later plugged and abandoned) were acquired from industry and have unusual shapes and a history dating back to 1946. The other 16 caverns (101-116) were leached according to SPR standards in the mid-1980s and have tall cylindrical shapes. The history of the caverns and their shapes are simulated in a 3-D geomechanics model of the site that predicts deformations, strains, and stresses. Future leaching scenarios due to oil drawdowns using fresh water are also simulated by increasing the volume of the caverns. Cavern pressures are varied in the model to capture operational practices in the field. The results of the finite element model are interpreted to provide information on the current and future status of subsidence, well integrity, and cavern stability. The most significant result in this report is relevant to caverns 1, 2, and 5. The caverns have non-cylindrical shapes and have potential regions where the surrounding salt may be damaged during workover procedures. During a workover the normal cavern operating pressure is lowered to service a well. At this point the wellhead pressures are atmospheric. When the workover is complete, the cavern is repressurized. The resulting elastic stresses are sufficient to cause tension and large deviatoric stresses at several locations. With time, these stresses relax to a compressive state due to salt creep. However, the potential for salt damage and fracturing exists. The analyses predict tensile stresses at locations with sharp-edges in the wall geometry, or in the case of cavern 5, in the neck region between the upper and lower lobes of the cavern. The effects do not appear to be large-scale, however, so the only major impact is the potential for stress-induced salt falls in cavern 5, potentially leading to

  9. Rare malformation of glans penis: arteriovenous malformation.

    Science.gov (United States)

    Akin, Y; Sarac, M; Yucel, S

    2013-01-01

    Pediatric glans penis malformations, especially arteriovenous malformations (AVM), are very rare. Herein, we report two rare cases. A 14-year-old boy attended our outpatient clinic with chief complaints of purple swelling and rapidly growing lesion on the glans penis. The lesion was excised surgically after physical and radiological evaluations. Pathology reported AVM and the patient is being followed up. The second case is a 2-year-old boy who was admitted with a big lesion involving glans penis and genital area that has been present since birth. In physical and radiological evaluations, lesion on the glans penis was pulsatile. Parents of the patient did not want any surgery and patient has been in follow-up. Diagnosis of the vascular lesions on glans penis is very easy by physical and radiological examinations today. Long-term follow-up is very important for AVM. Clinicians must make a careful effort to document new glans lesions in the pediatric population and decrease anxiety in the parents of affected children.

  10. Arteriovenous malformation within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma

    Directory of Open Access Journals (Sweden)

    Grace Lai

    2015-01-01

    Full Text Available Background: The co-occurrence of intracranial arteriovenous malformations (AVMs and cerebral neoplasms is exceedingly rare but may harbor implications pertaining to the molecular medicine of brain cancer pathogenesis. Case Description: Here, we present a case of de novo AVM within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma (WHO Grade III and review the potential contribution of this mutation to aberrant angiogenesis as an interesting case study in molecular medicine. Conclusion: The co-occurrence of an IDH1 mutated neoplasm and AVM supports the hypothesis that IDH1 mutations may contribute to aberrant angiogenesis and vascular malformation.

  11. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  12. Cavernous haemangioma mimicking as clitoral hypertrophy

    Directory of Open Access Journals (Sweden)

    Sajid Nayyar

    2014-01-01

    Full Text Available Haemangioma is the most common benign neoplasm of infantile age. It is most commonly located in head and neck region, trunk and extremities but very rarely it can be located at clitoris. However, it is very important to differentiate clitoral haemangioma from enlargement of the clitoris secondary to androgen excess. Only four cases of clitoromegaly caused by cavernous haemangioma have been reported in the literature so far. Herein, we report our experience with a 10-year-old girl who presented with clitoromegaly and normal hormonal assay that turned out to be clitoral cavernous haemangioma after histopathological examination of the clitoral mass.

  13. 多层螺旋CT血管成像在脑动静脉畸形诊断及治疗中的价值%Value of multi-slice spiral CT angiography in diagnosis and treatment of cerebral arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    余海浪; 庹秀均; 李战辉; 张应和

    2013-01-01

    目的 探讨脑动静脉畸形(CAVM)在多层螺旋CT血管造影(MSCTA)中的表现及MSCTA的临床价值.方法 收集36例行MSCTA检查的CAVM患者的资料,采用容积再现重组(VR)血管生长技术(AV)进行血管重建,分析供血动脉、引流静脉的表现.结果 36例CAVM患者中,病变累及枕叶10例,颞叶9例,额叶2例,顶叶3例,枕叶、小脑半球7例,颞、枕叶3例,额、顶叶2例,均清楚显示畸形血管团、供血动脉及引流静脉;4例合并血管畸形内动脉瘤,2例合并血管畸形外动脉瘤,3例蛛网膜下腔出血,6例脑出血.结论 MSCTA能清楚显示CAVM的畸形血管团、供血动脉、引流静脉及合并的动脉瘤.%Objective To evaluate the manifestation of cerebral arteriovenous malformations (CAVM) on multi-slice spiral CT (MSCT) angiography and the clinical value of this examinaion.Methods The data on 36 patients with CAVM undergoing MSCT angiography were collected.Vascular reconstruction were performed by volume rendering and AV.The manifestations of feeding arteries and draining veins were then analyzed.Results Among these 36 patients with cerebral arteriovenous malformations,ten patients had the lesion in occipital lobe,nine in temporal lobe,two in frontal lobe,three in parietal lobe,seven in occipital lobe and cerebellar hemisphere,three in occipital and temporal lobe,and two in frontal and parietal lobe,with a clear display of masses of deformed vessels,feeding arteries and draining veins; 4 patients were complicated by intra-aneurysm of CAVM,2 were complicated by extra-aneurysm of CAVM,3 concurred with subarachnoid hemorrhage,and 6 concurred with cerebral hemorrhage.Conclusions Multi-slice spiral angiography can clearly detect masses of deformed vessels,feeding arteries,draining veins,and coexisting aneurysms.

  14. Imaging diagnosis of dural and direct cavernous carotid fistulae

    Energy Technology Data Exchange (ETDEWEB)

    Santos, Daniela dos; Monsignore, Lucas Moretti; Nakiri, Guilherme Seizem; Cruz, Antonio Augusto Velasco e; Colli, Benedicto Oscar; Abud, Daniel Giansante, E-mail: danisantos2404@gmail.com [Universidade de Sao Paulo (HCFMRP/USP), Ribeirao Preto, SP (Brazil). Faculdade de Medicina. Hospital das Clinicas

    2014-07-15

    Arteriovenous fistulae of the cavernous sinus are rare and difficult to diagnose. They are classified into dural cavernous sinus fistulae or direct carotid-cavernous fistulae. Despite the similarity of symptoms between both types, a precise diagnosis is essential since the treatment is specific for each type of fistula. Imaging findings are remarkably similar in both dural cavernous sinus fistulae and carotid-cavernous fistulae, but it is possible to differentiate one type from the other. Amongst the available imaging methods (Doppler ultrasonography, computed tomography, magnetic resonance imaging and digital subtraction angiography), angiography is considered the gold standard for the diagnosis and classification of cavernous sinus arteriovenous fistulae. The present essay is aimed at didactically presenting the classification and imaging findings of cavernous sinus arteriovenous fistulae. (author)

  15. Numerical Simulation of Gas Storage Caverns in Qom Region

    CERN Document Server

    Sharifzadeh, Mostafa

    2009-01-01

    The rock mechanical design of gas storage cavern in salt requires the analysis of the stability and the usability of the cavern over the planned operating time period. The design includes the build up of a rock mass model and a numerical model taking into account the geological situation, load condition, geometrical condition, and material parameters. In this paper multiple caverns in salt formation with geological and geomechanical situation in Qom (central part of Iran) was investigated a using creep model. Minimum safe center to center distances (CTCD) of multiple horizontal caverns also were studied. CTCD of caverns interact at less than two times of cavern diameter. With increasing the CTCD to 2.5 times cavern diameters, diminish most interaction.

  16. Congenital pial arteriovenous fistula in the temporal region draining into cavernous sinus: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Ziyin; Wang, Chaohua; Zhang, Changwei; Xie, Xiaodong [Dept. of Neurosurgery, West China Hospital of Sichuan University, Chengdu (China); Wang, Kun; Tang, Jianjian [Dept. of Neurosurgery, Affiliated Hospital of Hainan Medical College, Haikou (China)

    2013-06-15

    This report concerns a 4-month-old infant with progressive prominent and redness of his left eye since birth. This report concerns a 4-month-old infant with progressive prominent redness of his left eye since birth. Angiography revealed a congenital pial arteriovenous fistula between the temporal branch of the left posterior cerebral artery and left cavernous sinus through the sphenoparietal sinus, a condition not reported in the literature. The fistula was successfully occluded with two micro-coils by vertebrobasilar approach.

  17. Cavernous Hemangioma of the Bony Orbit

    Institute of Scientific and Technical Information of China (English)

    Jianhua Yan; Yu Cai; Zhongyao Wu; Ji Han; Youjian Pang

    2005-01-01

    Purpose: To study the clinical features, diagnosis and management of intraosseous cavernous hemangioma of the orbit.Methods: Five cases of intraosseous cavernous hemangioma seen in our hospital from Jan 1, 1986 to Dec 31, 2000 were reviewed.Results: Among all five cases, two were male and three were female. The mean age was 47.6 years old, ranging from 39.0 to 55.0 years. The left orbit was affected in 4 cases and the right one in 1 case. The bony involvement occurred in frontal bone (two cases),zygomatic bone (two cases) and sphenoid bone (one case). A painless, slowly enlarging hard bony mass fixed to the bone with no pulsations was the main clinical sign. The x-ray and CT appearance of intraosseous cavernous hemangioma of the orbit were characteristic and usually diagnostic. The differential diagnosis of it included fibrous dysplasia,eosinophilic granuloma, multiple myeloma and metastatic carcinoma. Treatment is local removal of the bone containing the tumor.Conclusions: Intraosseous cavernous hemangioma is a rare tumor of the orbit and usually has good surgical result.

  18. Special people visit the ATLAS cavern

    CERN Multimedia

    Muriel

    ATLAS has been host to many important visitors lately. Here are a selected few: Professor Stephen Hawking visits the ATLAS cavern On Tuesday 26 September 2006 the ATLAS Collaboration was honoured by a very special visit to the detector in the underground cavern. We were pleased to guide Professor Stephen Hawking, the famous cosmologist holding the post of Lucasian Professor of Mathematics at Cambridge University (position held by Isaac Newton in the 17th century), on a tour of the ATLAS pit and the LHC tunnel. The visit was accompanied by a few colleagues from the CERN Theory group, and was only possible thanks to the professional assistance of Olga Beltramello and Bernard Lebegue, who had also taken care of all the necessary preparatory work in the cavern. Professor Hawking was very keen to check for himself the status of the detector installation, and he admired, in particular, the spectacular TGC big wheel on side C. (left) Stephen Hawking in the ATLAS cavern side-C (right) and in the LHC tunnel...

  19. The ATLAS cavern in the spotlight

    CERN Multimedia

    On Wednesday, 4th June, the President of the Swiss Confederation, Pascal Couchepin, inaugurated the world's largest experimental cavern, which is to house the ATLAS detector in 2007, and announced Switzerland's gift to CERN of the "Palais de l'Equilibre".

  20. Familial Chiari malformation: case series.

    Science.gov (United States)

    Schanker, Benjamin D; Walcott, Brian P; Nahed, Brian V; Kahle, Kristopher T; Li, Yan Michael; Coumans, Jean-Valery C E

    2011-09-01

    Chiari malformations (Types I-IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%-0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

  1. Cerebellar Hemorrhage due to a Direct Carotid–Cavernous Fistula after Surgery for Maxillary Cancer

    Science.gov (United States)

    Kamio, Yoshinobu; Hiramatsu, Hisaya; Kamiya, Mika; Yamashita, Shuhei; Namba, Hiroki

    2017-01-01

    Infratentorial cerebral hemorrhage due to a direct carotid–cavernous fistula (CCF) is very rare. To our knowledge, only four such cases have been reported. Cerebellar hemorrhage due to a direct CCF has not been reported. We describe a 63-year-old female who presented with reduced consciousness 3 days after undergoing a maxillectomy for maxillary cancer. Computed tomography showed a cerebellar hemorrhage. Magnetic resonance angiography showed a left-sided direct CCF draining into the left petrosal and cerebellar veins through the left superior petrosal sinus (SPS). Her previous surgery had sacrificed the pterygoid plexus and facial vein. Increased blood flow and reduced drainage could have led to increased venous pressure in infratentorial veins, including the petrosal and cerebellar veins. The cavernous sinus has several drainage routes, but the SPS is one of the most important routes for infratentorial venous drainage. Stenosis or absence of the posterior segment of the SPS can also result in increased pressure in the cerebellar and pontine veins. We emphasize that a direct CCF with cortical venous reflux should be precisely evaluated to determine the hemodynamic status and venous drainage from the cavernous sinus. PMID:28061497

  2. 流体切应力对脑动静脉畸形内皮细胞增殖与c-myc表达的影响%Effect of flow shear stress on endothelial cell proliferation and c-myc expression in cerebral arteriovenous malformation

    Institute of Scientific and Technical Information of China (English)

    赵明光; 李彦兵; 吕博川; 梁勇; 薛洪利; 赵丽萍; 王丹玲

    2007-01-01

    BACKGROUND:Shear stress can directly mediate the expression of endothelial cells, especially some cytokine genes whose codes are related to angiogenesis. Otherwise, flow shear stress of blood plays an importantly biological role in regulating vascular structure and function.OBJECTIVE: To observe the effects of laminar flow shear stress on the proliferation of vascular endothelial cells and the expression of protooncogene c-myc in human cerebral arteriovenous malformation.DESIGN: Randomized controlled study.SETTING: Department of Neurosurgery, Shenyang General Hospital of Military Area Command of Chinese PLA.MATERIALS: The experiment was carried out in the Neuromedical Institute, General Hospital of Shenyang Military Area Command of Chinese PLA from November 2006 to February 2007. Fresh samples of human cerebral arteriovenous malformation were derived from 20 patients who were of grade Spetzler Ⅱ -Ⅲ and received resection of human cerebral arteriovenous malformation in the Department of Neurosurgery, General Hospital of Shenyang Military Area Command of Chinese PLA in 2006. All cases were diagnosed with whole-brain angiography before operation. The main reagents and equipments were detailed as follows: M199 culture media (Gilbco BRL), quality fetal bovine serum (HyClone), endothelial cell growth supplement (ECGS; Sigma, USA), CO2 incubator (Forma Scientific, USA), flow cytometry analysis of cell cycle kit (BD Company), flow cytometer (FACS Calibur, BD Company), rat-anti-human c-myc monoclonal antibody (Santa Cruz Company, USA), and reverse transcription polymerase chain reaction (RT-PCR) kit (Promega).METHODS: Tissue explants adherent method was used to culture vascular endothelial cells of human cerebral arteriovenous malformation, and then the cells were classified into 4 groups based on degree of shear stress, including control group, low shear stress group, moderate shear stress group and high shear stress group. Cultured endothelial cells of human cerebral

  3. The association of carotid cavernous fistula with Graves′ ophthalmopathy

    Directory of Open Access Journals (Sweden)

    Ozlem Celik

    2013-01-01

    Full Text Available Graves′ ophthalmopathy (GO is one of the frequent manifestations of the disorder which is an inflammatory process due to fibroblast infiltration, fibroblast proliferation and accumulation of glycosaminoglycans. Eye irritation, dryness, excessive tearing, visual blurring, diplopia, pain, visual loss, retroorbital discomfort are the symptoms and they can mimic carotid cavernous fistulas. Carotid cavernous fistulas are abnormal communications between the carotid arterial system and the cavernous sinus. The clinical manifestations of GO can mimic the signs of carotid cavernous fistulas. Carotid cavernous fistulas should be considered in the differential diagnosis of the GO patients especially who are not responding to the standard treatment and when there is a unilateral or asymmetric eye involvement. Here we report the second case report with concurrent occurrence of GO and carotid cavernous fistula in the literature.

  4. CNS cavernous haemangioma: 'popcorn' in the brain and spinal cord

    Energy Technology Data Exchange (ETDEWEB)

    Hegde, A.N. [Department of Neuroradiology, National Neuroscience Institute (Singapore); Department of Diagnostic Imaging, National University Hospital (Singapore); Mohan, S. [Department of Neuroradiology, National Neuroscience Institute (Singapore); Department of Radiology, University of Pennsylvania School of Medicine, Philadelphia (United States); Lim, C.C.T., E-mail: Tchoyoson_lim@nni.com.sg [Department of Neuroradiology, National Neuroscience Institute (Singapore); Department of Neurology, Duke NUS Graduate Medical School (Singapore)

    2012-04-15

    Cavernous haemangiomas (CH) are relatively uncommon non-shunting vascular malformations of the central nervous system and can present with seizures or with neurological deficits due to haemorrhage. Radiologists can often suggest the diagnosis of CH based on characteristic magnetic resonance imaging (MRI) features, thus avoiding further invasive procedures such as digital subtraction angiography or surgical biopsy. Although typical MRI appearance combined with the presence of multiple focal low signal lesions on T2*-weighted images or the presence of one or more developmental venous anomaly within the brain can improve the diagnostic confidence, serial imaging studies are often required if a solitary CH presents at a time when the imaging appearances had not yet matured to the typical 'popcorn' appearance.

  5. Nasopharyngeal carcinoma presented as cavernous sinus tumour.

    Science.gov (United States)

    Moona, Mohammad Shafi; Mehdi, Itrat

    2011-12-01

    A 32 year Libyan male presented with the complaints of headache and diplopia. He was diagnosed with a cavernous sinus meningioma on the basis of MRI findings but no initial biopsy was taken. Depending on the radiologic diagnosis the patient was treated with gamma knife surgery twice, abroad. During follow up he developed left ear deafness and left cervical lymph adenopathy. An ENT evaluation with biopsy from the nasopharynx and cervical lymph node was taken. The histopathologic diagnosis of the resected tumour showed a nasopharyngeal carcinoma with cervical lymph node metastasis (poorly differentiated lympho-epithelial carcinoma). The cavernous sinus tumour which was initially treated as a meningioma was in fact metastasis from the nasopharyngeal carcinoma, making this an interesting and rare occurrence.

  6. Rare cavernous lymphangioma of the bladder in an adult female:treatment and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Feng Qu; Jinyu Zheng; Yajun Xiao; Yifei Xing; Chuanguo Xiao

    2006-01-01

    Objective: Lymphangioma is a benign tumor representing a congenital malformation of the lymphatic channels. The cavernous lymphangioma of the bladder did not report before. Here we report 1 case of cavernous lymphangioma of the bladder in an adult female and review the literature in order to accumulate the experience of diagnosis and treatment for these diseases. Methods: In our case a 50-year-old woman presented with irritative voiding symptoms and had painless macroscopic hematuria at times. Cystoscopy showed a non-papillary tumor at apex vesicae and mucous membrane hyperaemia between the two orifices within the trigone. The pathologic diagnosis was cystitis glandularis with hyperplasia of urothelium and cavernous lymphangioma in lamina propria. Transurethral resection was performed and then bleomycin A5 was injected into lesion multipoint applying ureteral catheter with puncture needle. Results: Two months after operation the irritative voiding symptoms improved and the urine analysis was normal. Cystoscopy showed no residual tumor. Bleomycin A5 was injected into the lesion area again just like the operation before. All symptoms disappeared completely when the patient was examined a month later. There was no side effect after injection and no recurrence during the follow-up of 1 year. Conclusion: Lymphangioma of the bladder can be diagnosed exactly by cystoscopy and pathological examination. Surgery may be the best treatment. The bleomycin A5 intralesional sclerosant is also an effective therapy for the disease after surgical removal.

  7. Thoracic Cavernous Lymphangioma Provoking Massive Chyloptysis

    Directory of Open Access Journals (Sweden)

    Robert Ferguson MD

    2013-09-01

    Full Text Available Chyloptysis is a relatively rare embodiment of disease that encompasses a lengthy differential and provides many diagnostic and therapeutic challenges. Presented here is the case of a young woman with massive chyloptysis due to a thoracic cavernous lymphangioma arising in the peripartum period. The severity of her condition mandated the use of cardiopulmonary bypass to resect her lymphangioma. We believe that the extent of her symptoms, etiology of disease, and surgical management represent a unique scenario in the literature.

  8. Inauguration of the ATLAS cavern, June 2003.

    CERN Multimedia

    Maximilien Brice

    2003-01-01

    Before the inauguration, Pascal Couchepin visited the ATLAS cavern. From left to right: Peter Jenni, ATLAS spokesman; Maurice Bourquin, President of the CERN Council; Carlo Lamprecht, State councillor, Canton of Geneva; Pascal Couchepin, President of the Swiss Confederation; Luciano Maiani, CERN Director-General; Marzio Nessi, ATLAS technical coordinator; Arturo Henniger, ZSCHOKKE-LOCHER AG Director; Benno Baettig, personal advisor of President Couchepin; Jean-Luc Baldy, head of CERN Civil Engineering Group.

  9. Necrotizing Fasciitis of the Nose Complicated with Cavernous Sinus Thrombosis

    Directory of Open Access Journals (Sweden)

    D. Swaminath

    2014-01-01

    Full Text Available Necrotizing fasciitis is a rapidly progressive life threatening bacterial infection of the skin, the subcutaneous tissue, and the fascia. We present a case of necrotizing fasciitis involving the nose complicated by cavernous sinus thrombosis. Few cases of septic cavernous sinus thrombosis have been reported to be caused by cellulitis of the face but necrotizing fasciitis of the nose is rare. It is very important to recognize the early signs of cavernous thrombosis. Treatment for septic cavernous sinus thrombosis is controversial but early use of empirical antibiotics is imperative.

  10. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  11. Transvenous injection of Onyx for casting of the cavernous sinus for the treatment of a carotid-cavernous fistula.

    Science.gov (United States)

    Arat, Anil; Cekirge, Saruhan; Saatci, Isil; Ozgen, Burce

    2004-12-01

    A complex case of carotid-cavernous fistula was treated transvenously by injection of ethyl vinyl alcohol co-polymer into the cavernous sinus after an unsuccessful embolization attempt with detachable coils and liquid adhesive agents. There were no complications. At 3 months the patient's symptoms had resolved completely, and a control angiogram revealed persistent occlusion. The physical properties of ethyl vinyl alcohol polymer justify further investigation of this agent for the treatment of carotid-cavernous fistula.

  12. Classification and diagnosis of ear malformations

    OpenAIRE

    Bartel-Friedrich, Sylva; Wulke, Cornelia

    2008-01-01

    In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90%) and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations) or acquired in nature. Malformations can affect the outer ear (pinna and externa...

  13. Congenital cardiovascular malformations and the fetal circulation.

    Science.gov (United States)

    Rudolph, A M

    2010-03-01

    After birth, gas exchange is achieved in the lung, whereas prenatally it occurs in the placenta. This is associated with differences in blood flow patterns in the fetus as compared with the postnatal circulation. Congenital cardiovascular malformations are associated with haemodynamic changes in the fetus, which differ from those occurring postnatally. Obstruction to cardiac outflow may alter myocardial development, resulting in progressive ventricular hypoplasia. Alteration of oxygen content may profoundly influence pulmonary vascular and ductus arteriosus responses. Interference in blood flow and oxygen content may affect cerebral development as a result of inadequate oxygen or energy substrate supply. The circulatory effects may be gestational dependent, related to maturation of vascular responses in different organs. These prenatal influences of congenital cardiac defects may severely affect immediate, as well as longterm, postnatal prognosis and survival. This has stimulated the development of techniques for palliation of disturbed circulation during fetal life.

  14. Anorectal malformations : A multidisciplinary approach

    NARCIS (Netherlands)

    D. van den Hondel (Desiree)

    2015-01-01

    markdownabstractAbstract The research described in this thesis was performed with the aim to evaluate and improve multidisciplinary treatment of anorectal malformation patients. An overview of current literature on treatment of anorectal malformations is given in the Preface section, which also inc

  15. Rhino-orbito-cerebral mucormycosis: Magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Parveen A Lone

    2015-01-01

    Full Text Available Rhino-orbito-cerebral mucormycosis (ROCM is a potentially fatal fungal infection that commonly affects diabetic and other immunocompromised patients. Necrotizing and angioinvasive features of this class of pathogenic fungi facilitate spread from sinuses into orbit and brain with devastating consequences like cavernous sinus thrombosis. We describe magnetic resonance, including diffusion weighted imaging findings in a case of micobiologically documented ROCM in a 50-year-old diabetic man. Restricted diffusion was demonstrated in the distribution of infiltrating lesion right from soft issue facial component through left orbit and in the cavernous sinus. Favorable outcome was achieved with surgical debridement and prolonged amphotericin therapy.

  16. [Supratentorial cavernous malformations in an Argentinian institution: experience with surgical treatment].

    Science.gov (United States)

    Vergara, G E; Cervio, A; Farez, M; Mormandi, R; Condomi-Alcorta, S; Salvat, J

    2017-04-01

    Introduccion. Las malformaciones cavernosas son lesiones vasculares del sistema nervioso central constituidas por endotelio sinusoidal que forma capilares agrupados o cavernas que carecen de los elementos tipicos de una pared arterial madura y ausencia de tejido neural interpuesto. El endotelio esta rodeado por una densa capa de fibras colagenas que dejan pequeñas hendiduras por las que se extravasa hemosiderina. Se comunican con el sistema vascular a muy baja presion y su tratamiento puede ser por microcirugia o radiocirugia. Objetivos. Analizar las malformaciones cavernosas supratentoriales tratadas quirurgicamente en nuestra institucion (FLENI), determinar la epidemiologia y las caracteristicas intrinsecas, estudiar la clinica de presentacion, determinar las indicaciones quirurgicas y complicaciones, y establecer el pronostico. Pacientes y metodos. Estudio retrospectivo analitico de historias clinicas e imagenes de pacientes operados de malformaciones cavernosas supratentoriales en la FLENI desde enero de 1996 hasta diciembre de 2013. Resultados. Evaluamos a 51 pacientes, de 34 años de media, seguidos durante una media de 30 meses. El 1,96% de los pacientes presento diagnostico incidental y el resto mostro sintomas. El 23,52% presento hemorragia en el momento del diagnostico. En todas las cirugias se logro una exeresis total de las malformaciones cavernosas supratentoriales. Se observo un caso de meningitis postoperatoria. Conclusiones. La tasa de sangrado de las malformaciones cavernosas supratentoriales en nuestro medio es del 1,38% por paciente por año. El tratamiento quirurgico es eficaz para erradicar o disminuir los sintomas y para evitar un posible resangrado. Presenta una tasa muy baja de complicaciones y un pronostico neurologico favorable.

  17. 36 CFR 7.47 - Carlsbad Caverns National Park.

    Science.gov (United States)

    2010-07-01

    ... 36 Parks, Forests, and Public Property 1 2010-07-01 2010-07-01 false Carlsbad Caverns National Park. 7.47 Section 7.47 Parks, Forests, and Public Property NATIONAL PARK SERVICE, DEPARTMENT OF THE INTERIOR SPECIAL REGULATIONS, AREAS OF THE NATIONAL PARK SYSTEM § 7.47 Carlsbad Caverns National Park....

  18. Recovery of opthalmoplegia associated with cavernous sinus dural arteriovenous fistulas after transvenous cavernous sinus packing

    Energy Technology Data Exchange (ETDEWEB)

    Lv Xianli; Jiang Chuhan; Li Youxiang; Yang Xinjian [Beijing Neurosurgical institute, Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan Xili, Chongwen, Beijing (China); Wu Zhongxue, E-mail: ttyyzjb@sina.co [Beijing Neurosurgical institute, Beijing Tiantan Hospital, Capital Medical University, 6, Tiantan Xili, Chongwen, Beijing (China)

    2010-08-15

    Background: We report the recovery of ophthalmoplegia in 11 patients with cavernous sinus dural arteriovenous fistula (CSDAVF) after sinus packing at follow-up. Methods: Of 18 patients with CSDAVF treated with transvenous cavernous sinus packing between August 2002 and December 2007 at Beijing Tiantan Hospital, there were 9 patients with initial CNIII or CNVI dysfunction and 2 patients with CNVI dysfunction immediately after cavernous sinus packing selected and reevaluated. Results: Of 11 patients with CNIII or CNVI palsy, recovery was complete in 10. In 1 patient, complete CNVI palsy was unchanged because the CSDAVF was not cured. There were 6 men and 5 women with a mean age of 52.9 years. In 5 patients, CNVI palsy was associated with chemosis, proptosis and pulsatile tinnitus. Timing of treatment after onset of symptoms was from 4 to 35 days in 9 patients. All CSDAVFs were Barrow type D. Mean follow-up after treatment was 17.7 months (range, 2-54 months). Conclusion: CSDAVF-induced CNIII or CNVI palsies can be cured after cavernous sinus packing transvenously in most patients.

  19. Abordagem anestésica de grávida com malformação arteriovenosa cerebral e hemorragia subaracnoidea durante a gravidez: relato de caso Abordaje anestésico de embarazada con malformación arteriovenosa cerebral y hemorragia subaracnoidea durante el embarazo: relato de caso Anesthetic approach of pregnant woman with cerebral arteriovenous malformation and subarachnoid hemorrhage during pregnancy: case report

    Directory of Open Access Journals (Sweden)

    Catarina Santos Carvalho

    2013-04-01

    de 39 semanas, sana antes del embarazo, con antecedentes de HSA a las 22 semanas de gestación que se manifestó por medio de cefaleas, vómitos y mareos, sin la pérdida de la consciencia u otros déficits a la hora de su entrada en el servicio de urgencia. La resonancia magnética (RM arrojó MAV frontal izquierda. Después de un breve período de ingreso para la estabilización y el diagnóstico, se decidió mantener el embarazo y el acompañamiento ambulatorio multidisciplinario por neurocirugía y obstetricia en consulta de alto riesgo. Se optó por realizar la cesárea electiva a las 39 semanas bajo anestesia epidural lumbar. En el intraoperatorio ocurrió un episodio de hipotensión que fue rápidamente revertido con fenilefrina. El Índice de Apgar del recién nacido fue de 10/10. El catéter epidural fue usado para la analgesia postoperatoria, que también cursó sin intercurrencias. CONCLUSIONES: Son muy raros los casos publicados de abordaje anestésico de embarazadas con MAV sintomáticas. Todas las decisiones tomadas por el equipo multidisciplinario, desde optar por continuar con el embarazo, hasta el momento ideal para intervenir la MAV, pasando por el tipo de anestesia y analgesia, fueron sopesadas en función del riesgo de daño cerebral. Desde el punto de vista anestésico, los autores enfatizan la necesidad de estabilidad hemodinámica.BACKGROUND AND OBJECTIVES: Subarachnoid hemorrhage (SAH during pregnancy is a rare event, and about half the cases are due to arteriovenous malformations (AVM. The authors describe the anesthetic approach of a 39 week pregnant patient scheduled for cesarean section, with a history of SAH due to AVM at 22 week gestation. CASE REPORT: 39 week pregnant patient, healthy prior to pregnancy, with a history of SAH at 22 week gestation, manifested by headache, vomiting, and dizziness without loss of consciousness or other deficits on admission to the emergency room. Magnetic resonance imaging (MRI revealed a left frontal AVM

  20. Novel cases of amyotrophic lateral sclerosis after treatment of cerebral arteriovenous malformationss.

    Science.gov (United States)

    Linnebank, Michael; McDougall, Cameron G; Krueger, Stefanie; Biskup, Saskia; Neumann, Manuela; Weller, Michael; Valavanis, Antonios; Prudlo, Johannes

    2016-01-01

    Previous case studies reported nine patients with cerebral arteriovenous malformations (AVM) who developed amyotrophic lateral sclerosis (ALS) after AVM embolisation. Here, we describe three novel cases of ALS which developed 13-34 years after treatment, including embolisation, of cerebral AVM. This study provides further arguments supporting the thesis that embolisation of cerebral AVM might influence the risk of later ALS development.

  1. Optical coherence tomography of the rat cavernous nerves

    Science.gov (United States)

    Fried, Nathaniel M.; Rais-Bahrami, Soroush; Lagoda, Gwen A.; Chuang, Ying; Burnett, Arthur L.; Su, Li-Ming

    2007-02-01

    Improvements in identification, imaging, and visualization of the cavernous nerves during radical prostatectomy, which are responsible for erectile function, may improve nerve preservation and postoperative potency. Optical coherence tomography (OCT) is capable of real-time, high-resolution, cross-sectional, in vivo tissue imaging. The rat prostate serves as an excellent model for studying the use of OCT for imaging the cavernous nerves, as the rat cavernous nerve is a large, visible, and distinct bundle allowing for easy identification with OCT in addition to histologic confirmation. Imaging was performed with the Niris OCT system and a handheld 8 Fr probe, capable of acquiring real-time images with 11-μm axial and 25-μm lateral resolution in tissue. Open surgical exposure of the prostate was performed on a total of 6 male rats, and OCT images of the prostate, cavernous nerve, pelvic plexus ganglion, seminal vesicle, blood vessels, and periprostatic fat were acquired. Cavernous nerve electrical stimulation with simultaneous intracorporeal pressure measurements was performed to confirm proper identification of the cavernous nerves. The prostate and cavernous nerves were also processed for histologic analysis and further confirmation. Cross-sectional and longitudinal OCT images of the cavernous nerves were acquired and compared with histologic sections. The cavernous nerve and ganglion could be differentiated from the surrounding prostate gland, seminal vesicle, blood vessels, bladder, and fatty tissue. We report preliminary results of OCT images of the rat cavernous nerves with histologic correlation and erectile stimulation measurements, thus providing interpretation of prostate structures as they appear in OCT images.

  2. Pericallosal lipoma and middle cerebral artery aneurysm: a coincidence?

    Energy Technology Data Exchange (ETDEWEB)

    Sommet, Julie; Schiff, Manuel; Evrard, Philippe [Hopital Robert Debre, APHP, Department of Paediatric Neurology and Metabolic Diseases, Paris Cedex 19 (France); Blanc, Raphael [Fondation Rothschild, Department of Interventional Radiology, Paris (France); Elmaleh-Berges, Monique [Hopital Robert Debre, Department of Paediatric Radiology, Paris (France)

    2010-08-15

    Intracranial lipomas are rare congenital malformations that can often be seen in association with other brain malformations; agenesis or dysgenesis of the corpus callosum is the most frequently associated brain anomaly. They are usually pericallosal asymptomatic midline lesions. Intracranial lipomas associated with a non-contiguous cerebral aneurysm are extremely rare. We report an infant with partial agenesis of the corpus callosum and pericallosal lipoma associated with cerebral haemorrhage due to a distal middle cerebral artery aneurysm. Such an association is probably not fortuitous and could suggest a pathogenic relationship. (orig.)

  3. CAVERNOUS HEMANGIOMA OF THE INTERNAL AUDITORY CANAL

    Directory of Open Access Journals (Sweden)

    Mohammad Hossein Hekmatara

    1993-06-01

    Full Text Available Cavernous hemangioma is a rare benign tumor of the internal auditory canal (IAC of which fourteen cases have been reported so far."nTinnitus and progressive sensorineural hearing loss (SNHL are the chief complaints of the patients. Audiological and radiological planes, CTScan, and magnetic resonance image (MRI studies are helpful in diagnosis. The only choice of treatment is surgery with elective transmastoid trans¬labyrinthine approach. And if tumor is very large, the method of choice will be retrosigmoid approach.

  4. A Developmental and Genetic Classification for Malformations of Cortical Development: Update 2012

    Science.gov (United States)

    Barkovich, A. James; Guerrini, Renzo; Kuzniecky, Ruben I.; Jackson, Graeme D.; Dobyns, William B.

    2012-01-01

    Malformations of cerebral cortical development include a wide range of developmental disorders that are common causes of neurodevelopmental delay and epilepsy. In addition, study of these disorders contributes greatly to the understanding of normal brain development and its perturbations. The rapid recent evolution of molecular biology, genetics…

  5. Spontaneous intracranial hemorrhage in children – ruptured lobar arteriovenous malformations: report of two cases

    Directory of Open Access Journals (Sweden)

    Tascu A.

    2015-03-01

    Full Text Available Brain arteriovenous malformations (AVMs are lesions thought to be primarily congenital in origin, consisting of fistulous connections of abnormal arteries and veins, without normal intervening capillary beds and no cerebral parenchyma between vessels. In the pediatric population, AVMs represent the most common cause of spontaneous intracranial hemorrhage (ICH, with a high recurrent bleeding risk. The aim of this paper is to report 2 cases of ruptured lobar AVMs in children, presenting with spontaneous ICH. Due to the patients’ neurological status, the only imaging examination performed preoperatively was a CT scan, showing intraparenchymal hemorrhage. Thus, there was no MRI/angiographic examination to prove the existence of a brain AVM prior to the surgical interventions. Also, the cerebral angiography performed after the surgery showed, in both patients, no signs of residual vascular malformations. Therefore, the diagnosis of AVM was certified by macroscopic and microscopic pathological findings, with no brain imaging suggestive of a vascular malformation.

  6. Beta adrenergic receptors in human cavernous tissue

    Energy Technology Data Exchange (ETDEWEB)

    Dhabuwala, C.B.; Ramakrishna, C.V.; Anderson, G.F.

    1985-04-01

    Beta adrenergic receptor binding was performed with /sup 125/I iodocyanopindolol on human cavernous tissue membrane fractions from normal tissue and transsexual procedures obtained postoperatively, as well as from postmortem sources. Isotherm binding studies on normal fresh tissues indicated that the receptor density was 9.1 fmoles/mg. with a KD of 23 pM. Tissue stored at room temperature for 4 to 6 hours, then at 4C in saline solution for 19 to 20 hours before freezing showed no significant changes in receptor density or affinity, and provided evidence for the stability of postmortem tissue obtained within the same time period. Beta receptor density of 2 cavernous preparations from transsexual procedures was not significantly different from normal control tissues, and showed that high concentrations of estrogen received by these patients had no effect on beta adrenergic receptor density. Displacement of /sup 125/iodocyanopindolol by 5 beta adrenergic agents demonstrated that 1-propranolol had the greatest affinity followed by ICI 118,551, zinterol, metoprolol and practolol. When the results of these displacement studies were subjected to Scatfit, non- linear regression line analysis, a single binding site was described. Based on the relative potency of the selective beta adrenergic agents it appears that these receptors were of the beta 2 subtype.

  7. Endovascular management of carotid-cavernous fistulas

    Institute of Scientific and Technical Information of China (English)

    GAO Bu-lang; LI Ming-hua; LI Yong-dong; FANG Chun; WANG Jue; DU Zhuo-ying

    2007-01-01

    Objective To investigate endovascular treatment of traumatic direct carotid-cavernous fistulas (CCF) and their complications such as pseudoaneurysms. Methods: Over a five-year period, 22 patients with traumatic direct CCFs were treated endovascularly in our institution. Thirteen patients were treated once with the result of CCF occluded, 8 twice and 1 three times. Treatment modalities included balloon occlusion of the CCF, sacrifice of the ipsilateral internal carotid artery with detachable balloon, coil embolization of the cavernous sinus and secondary pseudoaneurysms, and covered-stent management of the pseudoaneurysms. Results All the direct CCFs were successfully managed endovascularly. Four patients developed a pseudoaneurysm after the occlusion of the CCF with an incidence of pseudoaneurysm formation of 18.2% (4/22). A total number of 8 patients experienced permanent occlusion of the ICA with a rate of ICA occlusion reaching 36.4% (8/22). Followed up through telephone consultation from 6 months to 5 years, all did well with no recurrence of CCF symptoms and signs. Conclusion Traumatic direct CCFs can be successfully managed with endovascular means. The pseudoaneurysms secondary to the occlusion of the CCFs can be occluded with stent-assisted coiling and implantation of covered stents.

  8. Carotid and cranial nerve reconstruction after removal of cavernous sinus lesions.

    Science.gov (United States)

    Sekhar, L N; Sen, C N; Lanzino, G; Pomonis, S

    1991-12-01

    During the last 7 years, approximately 170 neoplasms, and 35 vascular lesions involving the cavernous sinus were treated by the first two authors. During the treatment of such lesions, the direct vein graft reconstruction of the internal carotid artery from the petrous to the supraclinoid or infraclinoid ICA was performed in 23 patients. Graft occlusion occurred in 3 patients and in one of these, it was successfully salvaged by placing a long venous graft from the extracranial ICA to the M3 segment of the middle cerebral artery. The latter 3 patients were neurologically normal. One patient with significant atherosclerotic disease suffered the dissection of the distal internal carotid artery with the graft being patent. The suturing technique. This patient eventually died. Two patients with severely compromised collateral circulation suffered minor strokes due to the temporary occlusion of the ICA. This has been avoided in the more recent patients by the adoption of brain protection techniques such as moderate hypothermia, induced hypertension, and barbiturate coma. Low dose heparin therapy during grafting and high dose intravenous steroids prior to the grafting also appear to be beneficial. Direct vein graft reconstruction of the intracavernous carotid artery is a valuable tool during the management of cavernous sinus lesions. The advantages and disadvantages of this technique as well as the pros and cons of other revascularization techniques will be discussed. During microsurgical removal of cavernous sinus lesions, the cranial nerves III-VI were reconstructed by direct resuture or by nerve grafting in 16 patients. In the majority of these patients, recovery of cranial nerve function was observed, which was very encouraging.(ABSTRACT TRUNCATED AT 250 WORDS)

  9. CaveMan Version 3.0: A Software System for SPR Cavern Pressure Analysis

    Energy Technology Data Exchange (ETDEWEB)

    BALLARD,SANFORD; EHGARTNER,BRIAN L.

    2000-07-01

    The U. S. Department of Energy Strategic Petroleum Reserve currently has approximately 500 million barrels of crude oil stored in 62 caverns solution-mined in salt domes along the Gulf Coast of Louisiana and Texas. One of the challenges of operating these caverns is ensuring that none of the fluids in the caverns are leaking into the environment. The current approach is to test the mechanical integrity of all the wells entering each cavern approximately once every five years. An alternative approach to detecting cavern leaks is to monitor the cavern pressure, since leaking fluid would act to reduce cavern pressure. Leak detection by pressure monitoring is complicated by other factors that influence cavern pressure, the most important of which are thermal expansion and contraction of the fluids in the cavern as they come into thermal equilibrium with the host salt, and cavern volume reduction due to salt creep. Cavern pressure is also influenced by cavern enlargement resulting from salt dissolution following introduction of raw water or unsaturated brine into the cavern. However, this effect only lasts for a month or two following a fluid injection. In order to implement a cavern pressure monitoring program, a software program called CaveMan has been developed. It includes thermal, creep and salt dissolution models and is able to predict the cavern pressurization rate based on the operational history of the cavern. Many of the numerous thermal and mechanical parameters in the model have been optimized to produce the best match between the historical data and the model predictions. Future measurements of cavern pressure are compared to the model predictions, and significant differences in cavern pressure set program flags that notify cavern operators of a potential problem. Measured cavern pressures that are significantly less than those predicted by the model may indicate the existence of a leak.

  10. Linear accelerator radiosurgery for arteriovenous malformations: Updated literature review.

    Science.gov (United States)

    Yahya, S; Heyes, G; Nightingale, P; Lamin, S; Chavda, S; Geh, I; Spooner, D; Cruickshank, G; Sanghera, P

    2017-04-01

    Arteriovenous malformations (AVMs) are the leading causing of intra-cerebral haemorrhage. Stereotactic radiosurgery (SRS) is an established treatment for arteriovenous malformations (AVM) and commonly delivered using Gamma Knife within dedicated radiosurgery units. Linear accelerator (LINAC) SRS is increasingly available however debate remains over whether it offers an equivalent outcome. The aim of this project is to evaluate the outcomes using LINAC SRS for AVMs used within a UK neurosciences unit and review the literature to aid decision making across various SRS platforms. Results have shown comparability across platforms and strongly supports that an adapted LINAC based SRS facility within a dynamic regional neuro-oncology department delivers similar outcomes (in terms of obliteration and toxicity) to any other dedicated radio-surgical platform. Locally available facilities can facilitate discussion between options however throughput will inevitably be lower than centrally based dedicated national radiosurgery units.

  11. Strategic petroleum reserve caverns casing damage update 1997

    Energy Technology Data Exchange (ETDEWEB)

    Munson, D.E.; Molecke, M.A.; Neal, J.T. [and others

    1998-01-01

    Hanging casing strings are used for oil and brine transfer in the domal salt storage caverns of the Strategic Petroleum Reserve (SPR). Damage to these casings is of concern because hanging string replacement is costly and because of implications on cavern stability. Although the causes of casing damage are not always well defined, many events leading to damage are assumed to be the result of salt falls impacting the hanging strings. However, in some cases, operational aspects may be suspected. The history of damage to hanging strings is updated in this study to include the most recent events. Potential general domal and local operational and material factors that could influence the tendency for caverns to have salt falls are examined in detail. As a result of this examination, general factors, such as salt dome anomalies and crude type, and most of the operational factors, such as geometry, location and depressurizations, are not believed to be primary causes of casing damage. Further analysis is presented of the accumulation of insolubles during cavern solutioning and accumulation of salt fall material on the cavern floor. Inaccuracies in sump geometry probably make relative cavern insolubles contents uncertain. However, determination of the salt fall accumulations, which are more accurate, suggest that the caverns with the largest salt fall accumulations show the greatest number of hanging string events. There is good correlation between the accumulation rate and the number of events when the event numbers are corrected to an equivalent number for a single hanging string in a quiescent, operating cavern. The principal factor that determines the propensity for a cavern to exhibit this behavior is thought to be the effect of impurity content on the fracture behavior of salt.

  12. Study of Congenital Malformations in Central Nervous System AND Gastro- Intestinal Tract

    Directory of Open Access Journals (Sweden)

    Saiyad SS

    2012-04-01

    Full Text Available Introduction: Congenital malformations comprise 8% of the perinatal mortality in India. They rank fifth as a cause of perinatal mortality, after asphyxia, respiratory problems, infections and cerebral trauma. However, the pattern is changing rapidly with improvement in health care and living standards. Material & Method: In the present study, authors have tried to study the cases of congenital malformations specially related to Central nervous system and Gastro-intestinal system. 5240 cases of newborn babies were studied and results were analyzed and classified in to various categories. Findings: The results show that malformations are more common in still birth, more in female babies and more in central nervous system In live born babies the percentage of malformation is0.63 % whereas in still born baby it is6.53 %. Conclusions: Chances of having malformations increases as the age advances. Parity of mother also influences the incidence. Exposure to radiation & drugs also influences malformations. Incidence of congenital malformation is highest in central nervous system. [National J of Med Res 2012; 2(2.000: 121-123

  13. Anorectal malformations in neonates

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2011-01-01

    Full Text Available Background : Anorectal malformations (ARM are associated with congenital anomalies and other risk factors, yielding a poor prognosis, especially in neonatal life. Objectives: This study was performed to identify the congenital anomalies as a factor of poor prognosis (mortality in such patients. Settings: Department of Pediatric surgery, The Children′s Hospital and The Institute of Child Health, Lahore. Design: Prospective observational study, with statistical support. Materials and Methods: The information on the demography, clinical features, investigations, management performed, and outcome was entered in the designed proforma and analysed with the help of statistical software EpiInfo version 3.5.1. Statistical test: Chi-square test was used to determine statistical significance of the results. Results : Of 100 neonates with ARM, 77 were male and 23, female (3.4:1. The mean age at presentation was 3.4 days (range, 12 hrs to 28 days. In 60 patients (60%, the presentation was imperforate anus without a clinically identified fistula. In 28 patients (28%, associated anomalies were present. The common associated anomalies were urogenital (10%, cardiovascular (8%, and gastrointestinal (6%. Down′s syndrome was present in 8 (8% patients. A total of 15 (15% deaths occurred in this study. In patients having associated congenital anomalies, 11 deaths occurred, whereas, 4 deaths were in patients without associated anomalies (P < 0.5. Conclusion : The mortality is higher in neonates with ARM having associated congenital anomalies.

  14. Shunt for bypass graft of the cavernous carotid artery: an anatomical and technical study.

    Science.gov (United States)

    al-Mefty, O; Khalil, N; Elwany, M N; Smith, R R

    1990-11-01

    During direct surgery of neoplastic and vascular lesions of the cavernous sinus, the intracavernous carotid artery may be injured beyond repair, or its total isolation may be necessary for surgical management of these lesions. The newly developed procedure of a saphenous vein graft bypass of the cavernous carotid artery allows re-establishment of carotid circulation. Patients with poor collateral circulation are at high risk for ischemic complications induced by the prolonged temporary occlusion required to perform the bypass graft. Optimal management of these patients is to perform the venous bypass graft for permanent vascularization while maintaining carotid cerebral circulation through an intraoperative shunt. We studied this procedure in cadavers, and three shunt types were evaluated: the external intrapetrous-supraclinoid shunt (Type A), the internal intrapetrous-supraclinoid shunt (Type B), and the neck internal carotid-supraclinoid shunt (Type C). Anatomical landmarks, techniques, distances, caliber, and materials used are presented. The rationale and candidates for such a procedure are discussed. The specifications of an optimal balloon shunt are presented, and the three procedures are compared.

  15. Spontaneous Carotid-Cavernous Fistula in the Type IV Ehlers-Danlos Syndrome.

    Science.gov (United States)

    Kim, Jeong Gyun; Cho, Won-Sang; Kang, Hyun-Seung; Kim, Jeong Eun

    2014-02-01

    Ehlers-Danlos syndrome (EDS) is a rare inherited connective disease. Among several subgroups, type IV EDS is frequently associated with spontaneous catastrophic bleeding from a vascular fragility. We report on a case of carotid-cavernous fistula (CCF) in a patient with type IV EDS. A 46-year-old female presented with an ophthalmoplegia and chemosis in the right eye. Subsequently, seizure and cerebral infarction with micro-bleeds occurred. CCF was completely occluded with transvenous coil embolization without complications. Thereafter, the patient was completely recovered. Transvenous coil embolization can be a good treatment of choice for spontaneous CCF with type IV EDS. However, every caution should be kept during invasive procedure.

  16. [Giant cavernous hemangioma of the orbit (case report)].

    Science.gov (United States)

    Grusha, Ia O; Ismailova, D S; Eksarenko, O V; Fedorov, A A; Kharlap, S I

    2014-01-01

    The following case demonstrates a successful en bloc removal of a massive cavernous hemangioma of the orbit via vertical transpalpebral approach with postoperative improvement of optic nerve condition and optimal cosmetic result.

  17. ATLAS - End-Cap calorimeter lowered in to the cavern

    CERN Multimedia

    2006-01-01

    The End-cap calorimeter was lowered into the ATLAS cavern at POINT1. This calorimeter will measure the energy of particles close to the beam axis when protons collide. Cooling is important for maximum detector efficiency.

  18. Cavernous hemangioma of the dura mater mimicking meningioma

    Directory of Open Access Journals (Sweden)

    Hambra Di Vitantonio

    2015-01-01

    Conclusions: Dural-based cavernous hemangiomas of the convexity are uncommon lesions. Up to now, only 13 cases have been described in the literature. The authors have discussed clinical aspects, radiological features, surgical treatment, and operative findings.

  19. An interesting case of cerebral abscess.

    Science.gov (United States)

    Rafiq, Muhammad Khizar

    2009-01-01

    An immigrant from Romania was referred to the neurosurgical unit with a cerebral abscess. On examination she was cyanosed and had clubbing of her fingers. A cardiovascular system examination revealed a systolic murmur heard all over the precordium. However, the diagnosis was not congenital cyanotic heart disease. The patient had a history of frequent nosebleeds and had multiple telangiectases on her body, leading to the diagnosis of hereditary haemorrhagic telangiectasia (HHT). A search was carried out for the presence of arteriovenous malformations in internal organs. Large arteriovenous malformations were found in the lungs, causing her cyanosis due to right-to-left shunting of blood and cerebral abscess due to paradoxical septic embolisation into cerebral circulation.

  20. Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis.

    Science.gov (United States)

    Ellis, Michael J; Armstrong, Derek; Dirks, Peter B

    2011-01-01

    The management of large and giant arteriovenous malformations (AVMs) in patients presenting with nonhemorrhagic neurological deficits secondary to vascular steal phenomenon is challenging and controversial. In many cases, large AVMs cannot be completely excised or cured, leaving patients with residual or partially treated AVMs, the natural history of which is unknown. Additionally, large, diffuse vascular malformations with multiple, small feeders, slow flow, or so-called cerebral proliferative angiopathy represent a related but distinct clinical and angiographic entity that may require a different therapeutic approach than traditional brain AVMs. The current management of children with other conditions of chronic cerebral hypoperfusion, such as moyamoya disease, involves consideration of surgical revascularization to enhance blood flow to the compromised hemisphere. Here, the authors present the case of a young child with a large thalamic vascular malformation who presented with clinical and radiological features of vascular steal and ischemia. In an effort to augment flow to the hypoperfused brain and protect against future ischemia, the authors treated the child with unilateral pial synangiosis. At 12 months, postoperative angiography demonstrated robust neovascularization, and the child has not sustained any further ischemic events. The authors discuss concept of vascular malformation-related hypoperfusion and the utility of indirect revascularization for inoperable vascular malformations presenting with ischemic symptoms.

  1. Oil field waste disposal in salt caverns: An information website

    Energy Technology Data Exchange (ETDEWEB)

    Tomasko, D.; Veil, J. A.

    1999-12-10

    Argonne National Laboratory has completed the construction of a Website for the US Department of Energy (DOE) that provides detailed information on salt caverns and their use for disposing of nonhazardous oil field wastes (NOW) and naturally occurring radioactive materials (NORM). Specific topics in the Website include the following: descriptions of salt deposits and salt caverns within the US, salt cavern construction methods, potential types of wastes, waste emplacement, regulatory issues, costs, carcinogenic and noncarcinogenic human health risks associated with postulated cavern release scenarios, new information on cavern disposal (e.g., upcoming meetings, regulatory issues, etc.), other studies supported by the National Petroleum Technology Office (NPTO) (e.g., considerations of site location, cavern stability, development issues, and bedded salt characterization in the Midland Basin), and links to other associated Web sites. In addition, the Website allows downloadable access to reports prepared on the topic that were funded by DOE. Because of the large quantities of NOW and NORM wastes generated annually by the oil industry, information presented on this Website is particularly interesting and valuable to project managers, regulators, and concerned citizens.

  2. Arteriovenous Malformation of the Pancreas

    Directory of Open Access Journals (Sweden)

    Alexandros Charalabopoulos

    2011-01-01

    Full Text Available Pancreatic arteriovenous malformation (PAVM is a very rare and mostly congenital lesion, with less than 80 cases described in the English-published literature. It is defined as a tumorous vascular abnormality that is constructed between an anomalous bypass anastomosis of the arterial and venous networks within the pancreas. It represents about 5% of all arteriovenous malformations found in the gastrointestinal tract. Herein, we present a 64-year-old patient with symptomatic PAVM involving the body and tail of the organ, which was successfully treated by transcatheter arterial embolization. The disease spectrum and review of the literature are also presented.

  3. Pediatric central nervous system vascular malformations

    Energy Technology Data Exchange (ETDEWEB)

    Burch, Ezra A. [Brigham and Women' s Hospital, Department of Radiology, Boston, MA (United States); Orbach, Darren B. [Boston Children' s Hospital, Neurointerventional Radiology, Boston, MA (United States)

    2015-09-15

    Pediatric central nervous system (CNS) vascular anomalies include lesions found only in the pediatric population and also the full gamut of vascular lesions found in adults. Pediatric-specific lesions discussed here include infantile hemangioma, vein of Galen malformation and dural sinus malformation. Some CNS vascular lesions that occur in adults, such as arteriovenous malformation, have somewhat distinct manifestations in children, and those are also discussed. Additionally, children with CNS vascular malformations often have associated broader vascular conditions, e.g., PHACES (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies and sternal anomalies), hereditary hemorrhagic telangiectasia, and capillary malformation-arteriovenous malformation syndrome (related to the RASA1 mutation). The treatment of pediatric CNS vascular malformations has greatly benefited from advances in endovascular therapy, including technical advances in adult interventional neuroradiology. Dramatic advances in therapy are expected to stem from increased understanding of the genetics and vascular biology that underlie pediatric CNS vascular malformations. (orig.)

  4. Investigation of Brain Arterial Circle Malformations Using Electrical Modelling and Simulation

    Directory of Open Access Journals (Sweden)

    Klara Capova

    2006-01-01

    Full Text Available The paper deals with the cerebral arterial system investigation by means of electrical modelling and simulations. The main attention is paid to the brain arterial circle malformations (stenoses and aneurysms and their determination and evaluation by computer-aided methods as tools of a non-invasive diagnostics. The compensation possibilities of brain arterial circle in case of presence of concrete arterial malformations are modelled and simulated. The simulation results of brain arteries blood pressures and volume flow velocities time dependences are presented and discussed under various health conditions.

  5. ADVANCED UNDERGROUND GAS STORAGE CONCEPTS REFRIGERATED-MINED CAVERN STORAGE

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1998-09-01

    Limited demand and high cost has prevented the construction of hard rock caverns in this country for a number of years. The storage of natural gas in mined caverns may prove technically feasible if the geology of the targeted market area is suitable; and economically feasible if the cost and convenience of service is competitive with alternative available storage methods for peak supply requirements. It is believed that mined cavern storage can provide the advantages of high delivery rates and multiple fill-withdrawal cycles in areas where salt cavern storage is not possible. In this research project, PB-KBB merged advanced mining technologies and gas refrigeration techniques to develop conceptual designs and cost estimates to demonstrate the commercialization potential of the storage of refrigerated natural gas in hard rock caverns. Five regions of the U.S.A. were studied for underground storage development and PB-KBB reviewed the literature to determine if the geology of these regions was suitable for siting hard rock storage caverns. Area gas market conditions in these regions were also studied to determine the need for such storage. Based on an analysis of many factors, a possible site was determined to be in Howard and Montgomery Counties, Maryland. The area has compatible geology and a gas industry infrastructure for the nearby market populous of Baltimore and Washington D.C.. As Gas temperature is lowered, the compressibility of the gas reaches an optimum value. The compressibility of the gas, and the resultant gas density, is a function of temperature and pressure. This relationship can be used to commercial advantage by reducing the size of a storage cavern for a given working volume of natural gas. This study looks at this relationship and and the potential for commercialization of the process in a storage application. A conceptual process design, and cavern design were developed for various operating conditions. Potential site locations were considered

  6. Ataque cerebral

    OpenAIRE

    Takeuchi Tan, Yuri; Fundación Valle de Lili

    1998-01-01

    ¿Qué es un ataque cerebral?/¿Qué tipos de ataque cerebral existen?/¿Cuáles son los síntomas de un ataque cerebral?/Factores de riesgo para un ataque cerebral/Tratamiento médico del ataque cerebral/¿por qué es importante acudir temprano cuando se presentan las señales de alarma?/ Manejo preventivo del ataque cerebral isquémico/Tratamiento quirúrgico del ataque cerebral/Enfermedad vascular cerebral hemorrágica/¿Cómo está constituido el grupo de ataque cerebral de la fundación Clínica Valle d...

  7. 两种磁共振血管成像序列对脑静脉畸形的比较影像学研究%Comparative Imaging Study of Two Sequences of MR Angiography in Dioagnosis of Cerebral Venous Malformation

    Institute of Scientific and Technical Information of China (English)

    王任水; 李晶; 曲林涛

    2012-01-01

    Objective To discuss the diagnostic value of Venous Blood Oxygenation Dependent MR1 (VEN_BOLD) and time of flight MR angiography(TOF-MRA) in cerebral venous malformation (CVM). Methods Using 1.5T superconducting MRI scanner, 13 cases of our hospital are diagnosed by the contrast imaging analysis of TOF-MRA and VENBOLD. Results Original axial images of TOF-MRA not only could display drainage vein of all cases, but also could observe 3D images of maximum intensity prolection(MIP). Among these original axial images, only 6 cases could show few pulp vein, however, VENBOLD could clear display drainage vein and more pulp veins of all CVM cases. Conclusion TOF-MRA could directly show 3D images of drainage vein of CVM, however, VEN_BOLD could clear display drainage vein and more pulp veins of CVM, The combining of TOF-MRA and VENBOLD could be the necessary supplement sequence for CVM diagnosis.%目的 探讨磁共振静脉血氧水平依赖成像(VEN-BOLD)和时间飞跃法血管成像(TOF-MRA)对静脉畸形(CVM)的诊断价值.方法 采用1.5T超导磁共振扫描仪对我院收治的13例CVM患者进行TOF-MRA和VEN-BOLD对比成像分析.结果 TOF-MRA原始轴位图像可显示全部13例CVM的引流静脉,并可进行最大密度投影重建(MIP) 3D图像观察,但仅6例原始图像可显示稀少的髓静脉,而VEN-BOLD可清晰显示全部13例CVM的引流静脉及更多的髓静脉.结论 TOF-MRA可对CVM的引流静脉进行3D直观显示;而VEN-BOLD可清晰准确地显示CVM的引流静脉及髓静脉,二者结合可作为CVM诊断必要的补充序列.

  8. Evidence for remotely triggered microearthquakes during salt cavern collapse

    Science.gov (United States)

    Jousset, Philippe; Rohmer, Jérémy

    2016-04-01

    Microseismicity is a good indicator of spatio-temporal evolution of physical properties of rocks prior to catastrophic events like volcanic eruptions or landslides and may be triggered by a number of causes including dynamic characteristics of processes in play or/and external forces. We show evidence of triggered microseismicity observed in the vicinity of an underground salt cavern prone to collapse by a remote M ˜ 7.2 earthquake, which occurred ˜12 000 km away. High-dynamic range broad-band records reveal the strong time-correlation between a dramatic change in the rate of local high-frequency microseismicity and the passage of low-frequency seismic waves, including body, Love and Rayleigh surface waves. Pressure was lowered in the cavern by pumping operations of brine out of the cavern. We demonstrate the near critical state of the cavern before the collapse by means of 2-D axisymmetric elastic finite-element simulations. On this basis, we show that the increment of stress necessary for the failure of the Dolomite layer, which ensures the stability of the whole system, is of the same order of magnitude as the maximum dynamic stress magnitude observed during the passage of the earthquakes waves. This suggests that the stress oscillations due to the seismic waves correlated with the recorded microearthquakes induced damage of the overburden, which eventually led to the collapse of the salt cavern. We show that the contribution of Rayleigh waves is the most efficient to trigger microseismicity at periods close to the natural fundamental frequency of the cavern system found at about 10-20 s by investigating the impulse response of the cavern + overburden + brine system.

  9. Hemiparesis in carotid cavernous fistulas (CCFs):a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    王慧晓; 白如林; 黄承光; 卢亦成; 张光霁

    2004-01-01

    @@ Carotid-cavernous fistulas (CCFs) are abnormal arteriovenous anastamoses between the carotid artery and the cavernous sinus. These fistulas may be classified by cause (spontaneous or traumatic), flow velocity (high or low ), or pathogenesis (direct or indirect).

  10. Lunar Holes and Their Associating Subsurface Caverns: From SELENE (Kaguya) to UZUME

    Science.gov (United States)

    Haruyama, J.; Kawano, I.; Nishibori, T.; Iwata, T.; Yamamoto, Y.; Shimada, K.; Yamamoto, K.; Hasenaka, T.; Morota, T.; Nishino, M. N.; Hashizume, K.; Shirao, M.; Komatsu, G.; Hasebe, N.; Shimizu, H.; Kobayashi, K.; Yokobori, S.; Miyake, Y.; Michikawa, Y.; Tsuji, T.; Shinoda, R.

    2016-05-01

    We present a summary of lunar holes and associated caverns. Furthermore, we also introduce the project Unprecedented Zipangu Underworld of the Moon/Mars Exploration (UZUME) to explore the holes and caverns.

  11. Contemporary diagnosis of venous malformation

    Directory of Open Access Journals (Sweden)

    Lee BB

    2013-11-01

    Full Text Available BB Lee,1 I Baumgartner21Department of Surgery, George Washington University, Washington, DC, USA; 2Swiss Cardiovascular Center, University Hospital Bern, Bern, SwitzerlandAbstract: Venous malformation is a congenital vascular malformation resulting from defective development during various stages of embryogenesis and selectively affecting the venous system. Depending on the embryologic stage when the developmental arrest occurred, the clinical presentation of venous malformation is extremely variable in location, extent, severity, natural progression, and hemodynamic impact. Extratruncular lesions occur in the earlier stages of embryonic life, and retain characteristics unique to mesenchymal cells (angioblasts, growing and proliferating when stimulated internally (eg, by menarche, pregnancy, and hormones or externally (eg, by trauma or surgery. These lesions also have a significant hemodynamic impact on the venous system involved, in addition to the risk of localized intravascular coagulopathy. However, truncal lesions, as defective developments along the late stage, no longer carry the risk of proliferation and recurrence due to lack of mesenchymal characteristics. Although, they often have serious hemodynamic consequences due to direct involvement of the main vein trunk. Therefore, a thorough clinical history and careful physical examination should be followed by an appropriate combination of noninvasive and less invasive tests (eg, Doppler ultrasonography, magnetic resonance imaging, computed tomography to confirm the clinical impression as well as to define the extent and severity of the venous malformation. Invasive tests, eg, phlebography or angiography, are seldom needed for the diagnosis per se. Additional evaluation for coagulation abnormalities, eg, D-dimer and fibrinogen levels, is generally recommended, especially for the treatment of surgery and endovascular candidates with extensive lesions to assess the localized intravascular

  12. Cerebral Palsy

    Science.gov (United States)

    Cerebral palsy is a group of disorders that affect a person's ability to move and to maintain balance ... do not get worse over time. People with cerebral palsy may have difficulty walking. They may also have ...

  13. Underground hydrogen storage. Final report. [Salt caverns, excavated caverns, aquifers and depleted fields

    Energy Technology Data Exchange (ETDEWEB)

    Foh, S.; Novil, M.; Rockar, E.; Randolph, P.

    1979-12-01

    The technical and economic feasibility of storing hydrogen in underground storage reservoirs is evaluated. The past and present technology of storing gases, primarily natural gas is reviewed. Four types of reservoirs are examined: salt caverns, excavated caverns, aquifers, and depleted fields. A technical investigation of hydrogen properties reveals that only hydrogen embrittlement places a limit on the underground storage by hydrogen. This constraint will limit reservoir pressures to 1200 psi or less. A model was developed to determine economic feasibility. After making reasonable assumptions that a utility might make in determining whether to proceed with a new storage operation, the model was tested and verified on natural gas storage. A parameteric analysis was made on some of the input parameters of the model to determine the sensitivity of the cost of service to them. Once the model was verified it was used to compute the cost of service of storing hydrogen in the four reservoir types. The costs of service for hydrogen storage ranged from 26 to 150% of the cost of the gas stored. The study concludes that it is now both safe and economic to store hydrogen in underground reservoirs.

  14. CAVERN ROOF STABILITY FOR NATURAL GAS STORAGE IN BEDDED SALT

    Energy Technology Data Exchange (ETDEWEB)

    DeVries, Kerry L; Mellegard, Kirby D; Callahan, Gary D; Goodman, William M

    2005-06-01

    This report documents research performed to develop a new stress-based criterion for predicting the onset of damage in salt formations surrounding natural gas storage caverns. Laboratory tests were conducted to investigate the effects of shear stress, mean stress, pore pressure, temperature, and Lode angle on the strength and creep characteristics of salt. The laboratory test data were used in the development of the new criterion. The laboratory results indicate that the strength of salt strongly depends on the mean stress and Lode angle. The strength of the salt does not appear to be sensitive to temperature. Pore pressure effects were not readily apparent until a significant level of damage was induced and the permeability was increased to allow penetration of the liquid permeant. Utilizing the new criterion, numerical simulations were used to estimate the minimum allowable gas pressure for hypothetical storage caverns located in a bedded salt formation. The simulations performed illustrate the influence that cavern roof span, depth, roof salt thickness, shale thickness, and shale stiffness have on the allowable operating pressure range. Interestingly, comparison of predictions using the new criterion with that of a commonly used criterion indicate that lower minimum gas pressures may be allowed for caverns at shallow depths. However, as cavern depth is increased, less conservative estimates for minimum gas pressure were determined by the new criterion.

  15. Gallium scintigraphy in a case of septic cavernous sinus thrombosis

    Energy Technology Data Exchange (ETDEWEB)

    Palestro, C.J.; Malat, J.; Gladstone, A.G.; Richman, A.H.

    1986-09-01

    Septic cavernous sinus thrombosis, a relatively uncommon disease entity, frequently can be fatal. Early diagnosis is imperative in order that appropriate treatment be instituted. A 59-year-old woman who was admitted to our institution with complaints of diplopia, blurred vision and fevers that developed following a tooth extraction is presented. Initial CT and lumbar puncture on the day of admission were totally normal. A repeat CT performed 48 hours after admission, on the same day as gallium imaging, demonstrated findings consistent with cavernous sinus thrombosis. Gallium imaging demonstrated intense uptake in the left cavernous sinus and left orbit as well as moderately increased activity in the right cavernous sinus and orbit, confirming infection. The patient was treated with antibiotics, and repeat CT and gallium imaging were performed ten days later, both of which demonstrated near total resolution of the disease process. Conceivably, if gallium imaging had been initiated on the day of admission it may have been the first study to demonstrate an infectious process in the cavernous sinus. Gallium imaging should be considered as a diagnostic tool in the noninvasive workup of this entity.

  16. Logging Geology Study of the Caverned Paleokarst Reservoir Distribution

    Institute of Scientific and Technical Information of China (English)

    WuXinsong; YangLei; PanWenqing; GuQiaoyuan

    2005-01-01

    In recent years, many carbonate rock oilfields such as Lunnan and Tahe have been found in the Tarim Basin, and the main reservoir space of the stable high yield wells in these oilfields consists mainly of the caverns formed by the paleo-karstification and the dissolution pores and fractures connected with them. Nevertheless, it is difficult to predict effectively the distribution of the cavern reservoir because of its extremely serious heterogeneity. In this paper, a case study of the Lunxi Oilfield in the north uplift of the Tarim Basin is conducted to introduce the logging geology method for predicting the distribution of the caverned paleokarst, reservoir. By means of building up the logging recognition style of different caverns and differentiating and correlating the karst zones with logging curves, and through an analysis of the paleokarst topography background, the favorable karst zones and distribution areas of the high quality cavern reservoir have been located, which lays a new basis for further exploration in this area.

  17. Endoscopic transsphenoidal approach to pituitary adenomas invading the cavernous sinus

    Institute of Scientific and Technical Information of China (English)

    TAO Yu-xin; QU Qiu-yi; WANG Zhen-lin; ZHANG Qiu-hang

    2010-01-01

    Background Surgery of pituitary adenomas invading cavernous sinus has always been thought as a challenge due to the complex anatomical structures and high risk of complications. The purpose of this study was to evaluate endoscopic trarssphenoidal approach to pituitary adenomas invading cavernous sinus.Methods The clinical data of 22 patients who admitted to Xuanwu Hospital with pituitary adenomas invading cavernous sinus were analyzed retrospectively. All patients underwent endoscopic transsphenoidal surgery. To expose the surgical field sufficiently, the opening of sellar floor was exceeded the bone overlying the invaded cavernous sinus, and synthetic dura was used to repair and strengthen the sella floor for preventing the leak of cerebrospinal fluid.Results Among 22 patients, total resection was achieved in 14, subtotal resection in 5, and partial resection in 3; no patient underwent insufficient resection. Visual symptoms improved in 7 of 9 patients. In one patient diplopia disappeared.Headache was relieved to various extents in all patients. No serious complications were found. Patient's hospital stay ranged from 7 to 20 days.Conclusion These data suggest that the endoscopic transsphenoid approach is a safe, minimally invasive, and efficient surgical technique, which might be an important therapeutic strategy for the pituitary adenoms invading cavernous sinus.

  18. Classification and diagnosis of ear malformations

    Directory of Open Access Journals (Sweden)

    Bartel-Friedrich, Sylva

    2007-01-01

    Full Text Available In the ENT region 50% of the malformations affect the ear. Malformations of the outer and middle ear are predominantly unilateral (ca. 70-90% and mostly involve the right ear. Inner ear malformations can be unilateral or bilateral. The incidence of ear malformations is approximately 1 in 3800 newborns. Ear malformations may be genetic (associated with syndromes or not, with family history, spontaneous mutations or acquired in nature. Malformations can affect the outer ear (pinna and external auditory canal, EAC, middle ear and inner ear, not infrequently in combination. Formal classification is advisable in order to be able to predict the prognosis and compare treatment schedules. Various classifications have been proposed: pinna and EAC malformations according to Weerda [1], middle ear malformations according to Kösling [2], and inner ear malformations according to Jackler [3], [4], to Marangos [5] and to Sennaroglu [6]. Additionally, we describe Altmann’s classification of atresia auris congenita [7] and the Siegert-Mayer-Weerda score [8] for EAC and middle ear malformations, systems of great practicability that are in widespread clinical use. The diagnostic steps include clinical examination, audiological testing, genetic analysis and, especially, CT and MRI. These imaging methods are most usefully employed in combination. Precise description of the malformations by means of CT and MRI is indispensable for the planning and successful outcome of operative ear reconstruction and rehabilitation procedures, including cochlear implantation.

  19. Congenital malformations and damage in early infancy of the central nervous system; Fehlbildungen und fruehkindliche Schaedigungen des ZNS

    Energy Technology Data Exchange (ETDEWEB)

    Jansen, O. [Universitaetsklinikum Schleswig-Holstein, Kiel (Germany). Sektion Neuroradiologi; Stephani, U. (eds.) [Universitaetsklinikum Schleswig-Holstein, Kiel (Germany). Universitaetskinderklinik - Klinik fuer Neuropaediatrie

    2007-07-01

    Congenital malformations and cerebral damage in early infancy cause complex morphological and clinical changes. Modern imaging techniques, and especially NMR, have provided deeper knowledge of these diseases in the past few years. Based on the neuroradiological findings, the book presents a complete picture of congenital malformations of the central nervous systems and cerebral damage in early infancy; it describes the underlying pathomechanisms, clinical symptoms and therapies. Neurologists and neuropaediatricians are enabled to diagnose malformations correctly and to develop optimal therapy strategies in cooperation with other medical disciplines. Neuroradiologists and radiologists, on the other hand, will find a manual for correct interpretation and differential diagnosis of their findings and a guide for interpreting the findings and deciding further therapeutic or diagnostic interventions. (orig.)

  20. Endovascular and microsurgical treatment of cerebral arteriovenous malformations: Current recommendations

    Directory of Open Access Journals (Sweden)

    Andrew Conger

    2015-01-01

    Conclusions: These lesions are among the most technically difficult pathological entities handled by the cerebrovascular specialist, and an overview of technical concepts to help systematize this challenging and variable endeavor can improve the safety of their treatment.

  1. Variants in CUL4B are Associated with Cerebral Malformations

    NARCIS (Netherlands)

    Vulto-van Silfhout, Anneke T.; Nakagawa, Tadashi; Bahi-Buisson, Nadia; Haas, Stefan A.; Hu, Hao; Bienek, Melanie; Vissers, Lisenka E. L. M.; Gilissen, Christian; Tzschach, Andreas; Busche, Andreas; Muesebeck, Joerg; Rump, Patrick; Mathijssen, Inge B.; Avela, Kristiina; Somer, Mirja; Doagu, Fatma; Philips, Anju K.; Rauch, Anita; Baumer, Alessandra; Voesenek, Krysta; Poirier, Karine; Vigneron, Jacqueline; Amram, Daniel; Odent, Sylvie; Nawara, Magdalena; Obersztyn, Ewa; Lenart, Jacek; Charzewska, Agnieszka; Lebrun, Nicolas; Fischer, Ute; Nillesen, Willy M.; Yntema, Helger G.; Jarvela, Irma; Ropers, Hans-Hilger; de Vries, Bert B. A.; Brunner, Han G.; van Bokhoven, Hans; Raymond, F. Lucy; Willemsen, Michel A. A. P.; Chelly, Jamel; Xiong, Yue; Barkovich, A. James; Kalscheuer, Vera M.; Kleefstra, Tjitske; de Brouwer, Arjan P. M.

    2015-01-01

    Variants in cullin 4B (CUL4B) are a known cause of syndromic X-linked intellectual disability. Here, we describe an additional 25 patients from 11 families with variants in CUL4B. We identified nine different novel variants in these families and confirmed the pathogenicity of all nontruncating varia

  2. Variants in CUL4B are associated with cerebral malformations

    NARCIS (Netherlands)

    Vulto-van Silfhout, A.T.; Nakagawa, T.; Bahi-Buisson, N.; Haas, S.A.; Hu, H; Bienek, M.; Vissers, L.E.L.M.; Gilissen, C.F.H.A.; Tzschach, A.; Busche, A.; Musebeck, J.; Rump, P.; Mathijssen, I.B.; Avela, K.; Somer, M.; Doagu, F.; Philips, A.K.; Rauch, A.; Baumer, A.; Voesenek, K.E.J.; Poirier, K.; Vigneron, J.; Amram, D.; Odent, S.; Nawara, M.; Obersztyn, E.; Lenart, J.; Charzewska, A.; Lebrun, N.; Fischer, U.; Nillesen, W.M.; Yntema, H.G.; Jarvela, I.; Ropers, H.H.; Vries, B. de; Brunner, H.G.; Bokhoven, H. van; Raymond, F.L.; Willemsen, M.A.A.P.; Chelly, J.; Xiong, Y.; Barkovich, A.J.; Kalscheuer, V.M.; Kleefstra, T.; Brouwer, A.P.M. de

    2015-01-01

    Variants in cullin 4B (CUL4B) are a known cause of syndromic X-linked intellectual disability. Here, we describe an additional 25 patients from 11 families with variants in CUL4B. We identified nine different novel variants in these families and confirmed the pathogenicity of all nontruncating varia

  3. Cerebral malaria Malaria cerebral

    Directory of Open Access Journals (Sweden)

    Silvia Blair Trujillo

    2003-03-01

    Full Text Available Is the most common complication of P. falciparum malaria; nearly 90% of people who have suffered CM can recover without neurological problems. Currently there are four hypotheses that explain pathogenesis of CM: cytoadherence and sequestering of parasitized red blood cells to cerebral capillaries; rosette formation and parasitized red blood cells agglutination; production of cytokines and activation of second messengers and opening of the blood-brain barrier. However the main question remains to be answered; how the host-parasite interaction in the vascular space interferes transiently with cerebral function? Recently, the beta amyloid precursor peptide has been employed as marker of neural injury in CM. It is expected that the beta amyloid precursor peptide will help to understand the pathogenesis of CM in complicated patients of endemic areas of Colombia. La malaria Cerebral (MC es la complicación más frecuente de la malaria por P. falciparum; aproximadamente el 90% de las personas que la han padecido se recuperan completamente sin secuelas neurológicas. Aún no se conoce con claridad su patogénesis pero se han postulado cuatro hipótesis o mecanismos posibles: 1 citoadherencia y secuestro de glóbulos rojos parasitados en la microvasculatura cerebral; 2 formación de rosetas y aglutinación de glóbulos rojos parasitados; 3 producción de citoquinas y activación de segundos mensajeros y, 4 apertura de la barrera hematoencefálica. Sin embargo, queda un interrogante sin resolver aún: ¿qué proceso se lleva a cabo para que el parásito, desde el espacio microvascular, pueda interferir transitoriamente con la función cerebral? Recientemente se ha utilizado el precursor de la proteína b-Amiloide como un marcador de daño neuronal en MC; este precursor será de gran ayuda en futuras investigaciones realizadas en nuestro medio que aporten información para comprender la patogénesis de la MC.

  4. Magnetic Resonance Perfusion Imaging in Malformations of Cortical Development

    Energy Technology Data Exchange (ETDEWEB)

    Widjaja, ED.; Wilkinson, I.D.; Griffiths, P.D. [Academic Section of Radiolog y, Univ. of Sheffield, Sheffield (United Kingdom)

    2007-10-15

    Background: Malformations of cortical development vary in neuronal maturity and level of functioning. Purpose: To characterize regional relative cerebral blood volume (rCBV) and difference in first moment transit time (TTfm) in polymicrogyria and cortical tubers using magnetic resonance (MR) perfusion imaging. Material and Methods: MR imaging and dynamic T2*-weighted MR perfusion imaging were performed in 13 patients with tuberous sclerosis complex, 10 with polymicrogyria, and 18 controls with developmental delay but no macroscopic brain abnormality. Regions of interest were placed in cortical tubers or polymicrogyric cortex and in the contralateral normal-appearing side in patients with malformations. In 'control' subjects, regions of interest were placed in the frontal and parietal lobes in both hemispheres. The rCBV and TTfm of the tuber/contralateral side (rCBVRTSC and TTFMTSC) as well as those of the polymicrogyria/contralateral side (rCBVRPMG and TTFMPMG) were assessed. The right-to-left asymmetry of rCBV and TTfm in the control group was also assessed (rCBVRControls and TTFMControls). Results: There was no significant asymmetry between right and left rCBV or TTfm (P>0.05) in controls. There was significant reduction in rCBVRTSC compared to rCBVRControls (P<0.05), but no significant difference in TTFMTSC compared to TTFMControls (P>0.05). There were no significant differences between rCBVRPMG and rCBVRControls (P>0.05) or TTFMPMG and TTFMControls (P>0.05). Conclusion: Our findings imply that cerebral blood volume of polymicrogyria is similar to normal cortex, but there is reduced cerebral blood volume in cortical tubers. The lower rCBV ratio of cortical tubers may be related to known differences in pathogenetic timing of the underlying abnormalities during brain development or the presence of gliosis.

  5. Supernova hemorrhage: obliterative hemorrhage of brain arteriovenous malformations following γ knife radiosurgery.

    Science.gov (United States)

    Alexander, Matthew D; Hetts, Steven W; Young, William L; Halbach, Van V; Dowd, Christopher F; Higashida, Randall T; English, Joey D

    2012-09-01

    Hemorrhage represents the most feared complication of cerebral arteriovenous malformations (AVMs) in both untreated patients and those treated with gamma knife radiosurgery. Radiosurgery does not immediately lead to obliteration of the malformation, which often does not occur until years following treatment. Post-obliteration hemorrhage is rare, occurring months to years after radiosurgery, and has been associated with residual or recurrent AVM despite prior apparent nidus elimination. Three cases are reported of delayed intracranial hemorrhage in patients with cerebral AVMs treated with radiosurgery in which no residual AVM was found on catheter angiography at the time of delayed post-treatment hemorrhage. That the pathophysiology of these hemorrhages involves progressive venous outflow occlusion is speculated and the possible mechanistic link to subsequent vascular rupture is discussed.

  6. Endovascular embolization of pulmonary arteriovenous malformations

    Institute of Scientific and Technical Information of China (English)

    LIU Feng-yong; WANG Mao-qiang; FAN Qing-sheng; DUAN Feng; WANG Zhi-jun; SONG Peng

    2010-01-01

    had persistent blood oxygen saturation between 85% and 90%. Symptoms of hypoxia never recurred in the other cases. Conclusions Endovascular embolization of pulmonary arteriovenous malformations can significantly improve blood oxygen saturation and reduce serious complications such as cerebral infaction. Therefore, this kind of interventional procedure is a safe and effective treatment method with a low complication rate.

  7. CLINICAL STUDY OF ANORECTAL MALFORMATIONS

    Directory of Open Access Journals (Sweden)

    Umesh

    2015-01-01

    Full Text Available BACKGROUND: A norectal malformations are relatively encountered anomalies. Presentations may vary from mild to severe and bowel control is t he main concern. AIM: To study the modes of presentation , types of anomalies , associated anomalies , reliability of clinical signs and radiological investigations in the diagnosis and the prognosis and continence in the post - operative in relation to type of anomaly and associated anomaly (s. MATERIAL AND METHODS: 50 cases of anorectal malformations admit ted to Department of Paediatric Surgery , in Medical Coll ege and Research Institute , were included in the study. Data related to the objectives of the study were collected. RESULTS: Commonest mode of presentation was failure to pass meconium 50%. 59% of mal es had high anomalies , while 53% females had intermediate anomalies. The diagnosis of low anomaly was made clinically , while high and intermediate anomalies needed further investigations. Associated anomalies were noted in 46.6% of the cases. 71.42% of the se patients had either a high or intermediate ARM. All patients with high anomalies underwent a 3 stage procedure , while low anomalies underwent a single stage procedure followed by anal dilatations. Rectal mucosal prolapse (2 cases , wound infection (4 ca ses , stenosis (3 cases , retraction of neo anus (1 case was seen. All the patients with low anomalies had a good functional result post operatively , while 57% and 28% of patients with intermediate and high anomalies had good results. CONCLUSION : Anorectal malformations are common congenital anomalies. Males are more commonly affected (1.3:1. Low anomalies are the commonest lesions noted in both the sexes (36.67%. High anomalies are more frequent in males. Invertogram offer an accurate diagnosis for planning management in patients with anorectal malformations. Low anomalies have a better outcome following surgery. For intermediate and high anomalies a staged repair offers better results

  8. CAVERNOUS HAEMANGIOMA WITH RETIFORM HAEMANGIOENDOTHELIOMA - A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Mamina

    2013-10-01

    Full Text Available ABSTRACT: INTRODUCTION : Retiform hemangioendothelioma is a rare variant of lo w - grade angiosarcoma with an indolent clinical behaviour, with predilection for young adults. Mostly is seen in the extremities, especially the distal lower limbs. CASE REPORT: An eighty year old lady, presented with complaints of a solitary, well defined swelling below the nape of the neck since two years. The swelling was clinically diagnosed as cavernous haemangioma. However the colour of the lesion s was looking like a melanocytic naevus. Excision was done under local anaesthesia and was sent for histopa tho lo gical examination. Histopathological diagnosis was confirmed as “Cavernous haemangioma with Retiform Haemangioendothelioma”

  9. Heuber Maneuver in Evaluation of Direct Carotid-Cavernous Fistula

    Science.gov (United States)

    Rajagopal, Rengarajan; Mehta, Neeraj; Saran, Sonal; Khera, Pushpinder S.

    2016-01-01

    Summary Carotid-cavernous fistulas are abnormal communications between the carotid system and the cavernous sinus. Elevated venous pressure produces congestion in the orbit with resultant transudation of fluid and increased intraocular pressure, thereby leading to secondary glaucoma which may result in visual loss. Immediate treatment is hence, warranted in these cases. The planning of endovascular management is dependent on many parameters, the most important of which are the size and location of the fistula. Since these are high-flow fistulas, assessment requires certain manoeuvers. Heuber manoeuver is one of the manoeuvers used to demonstrate the size of the fistula.

  10. Congenital Chiari malformations: A review

    Directory of Open Access Journals (Sweden)

    Vannemreddy Prasad

    2010-01-01

    Full Text Available Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. This entity has rapidly evolved over the past decade with newer visualization techniques, thus posing new challenges to diagnosis and management. This review includes the developmental theories, the latest nomenclature and existing treatment modalities of this interesting anomaly. Five theories tried to explain the malformation of the hindbrain and the neuraxis but no single theory completes the development of embryonic defects. Several atypical presentations have been reported with either incidental/asymptomatic features resulting in further classifications. The new magnetic resonance imaging flow techniques attempt to substantiate the clinical presentations and correlate with the abnormality which can be subtle in correlation. Surgical correction to improve the cranial volume, decrease the hydrocephalus and improve flow across the foramen magnum is the mainstay but needs to be tailored to a given type of malformation. Further clinical and imageological studies, especially longitudinal natural history, might improve our understanding of the atypical/asymptomatic presentations and the management that is currently available.

  11. A spindle cell hemangioendothelioma on the head resembling an arteriovenous malformation.

    Science.gov (United States)

    Higashino, Takuya; Hirai, Rintaro

    2014-07-01

    A spindle cell hemangioendothelioma is a relatively uncommon lesion, especially on the head and neck. Recurrence occurs after local excision of 50% to 60% of these lesions; therefore, it is important to recognize this unusual neoplasm and avoid misdiagnosis. Here, we report a rare case of a spindle cell hemangioendothelioma of the head. A 37-year-old woman presented with a soft subcutaneous mass, 2.5 cm in size, on her right occipital region. The mass pulsated strongly and a thrill was present. Magnetic resonance imaging showed that some dilated feeding arteries flowed into the mass and that a flow-void sign was present. The lesion looked like an arteriovenous malformation, and a marginal resection was performed. Histologically, there was a mix of cavernous vascular cavities and Kaposi sarcomalike spindle cell vascular zones, which is compatible with a spindle cell hemangioendothelioma.

  12. Cavernous sinus thrombosis and air embolism following surgery for acoustic neurinoma : a case report.

    Directory of Open Access Journals (Sweden)

    Nadkarni T

    2002-04-01

    Full Text Available A 55 year old male patient was operated on for a massive and vascular acoustic neurinoma in a sitting position. The tumor was completely excised. Post-operatively, the patient developed irritability and clinical features suggestive of contralateral cavernous sinus thrombosis. CT scan showed air within the dural walls of the cavernous sinus on the side of surgery. However, there was no radiological evidence of cavernous sinus thrombosis on the contralateral side. Cavernous sinus thrombosis as a post-surgery complication has not been reported. Air within the dural confines of the cavernous sinus has also not been observed or radiologically recorded in the literature.

  13. Pediatric neuroradiology: Cerebral and cranial diseases

    Energy Technology Data Exchange (ETDEWEB)

    Diebler, C.; Dulac, O.

    1987-01-01

    In this book, a neuroradiologist and a neuropediatrician have combined forces to provide the widest possible knowledge in investigating cranial and cerebral disorders in infancy and childhood. Based on more than 20,000 pediatric CT examinations, with a follow-up time often exceeding ten years, the book aims to bridge interdisciplinary gaps and help radiologists, pediatricians and neurosurgeons solve the various problems of pediatric neuroradiology that frequently confront them. For each disease, the etiology, clinical manifestation, pathological lesions and radiological presentations are discussed, supported by extensive illustrations. Malformative, vascular, traumatic, tumoral, infectious and metabolic diseases are reviewed. Miscellaneous conditions presenting particular symptoms or syndromes are also studied, such as hydrocephalus and neurological complications of leukemia. Contents: Cerebral and cranial malformations; neurocutaneous syndromes; inherited metabolic diseases; infectious diseases - vascular disorders; intracranial tumors; cranial trauma - miscellaneous and subject index.

  14. Transvenous treatment of a complex cavernous sinus dural arteriovenous fistula secondary to balloon embolization of a traumatic carotid-cavernous fistula

    Institute of Scientific and Technical Information of China (English)

    HAI Jian; CHEN Zuo-quan; DENG Dong-feng; PAN Qing-gang; LING Feng

    2006-01-01

    @@ AIthough recurrent traumatic carotid-cavernous fistula (CCF) and its treatment have been reported sporadically,1 a complex cavernous sinus dural arteriovenous fistula (DAVF) secondary to balloon embolization of a direct traumatic CCF is rare. In 2005, we treated such a case via transvenous approach using coils and N-buty-2-cyanoacrylate (NBCA). The causes of recurrent cavernous sinus DAVF and its endovascular approach are discussed.

  15. Dynamic permeability and quantitative susceptibility

    DEFF Research Database (Denmark)

    Mikati, Abdul Ghani; Tan, Huan; Shenkar, Robert;

    2014-01-01

    Hyperpermeability and iron deposition are 2 central pathophysiological phenomena in human cerebral cavernous malformation (CCM) disease. Here, we used 2 novel MRI techniques to establish a relationship between these phenomena....

  16. Congenital malformations of the skull and meninges.

    Science.gov (United States)

    Kanev, Paul M

    2007-02-01

    The surgery and management of children who have congenital malformations of the skull and meninges require multidisciplinary care and long-term follow-up by multiple specialists in birth defects. The high definition of three-dimensional CT and MRI allows precise surgery planning of reconstruction and management of associated malformations. The reconstruction of meningoencephaloceles and craniosynostosis are challenging procedures that transform the child's appearance. The embryology, clinical presentation, and surgical management of these malformations are reviewed.

  17. Spontaneous ileal perforation complicating low anorectal malformation

    Directory of Open Access Journals (Sweden)

    TiJesuni Olatunji

    2015-01-01

    Full Text Available Anorectal malformation is a common anomaly in neonates. Although colorectal perforations have been reported as a complication, ileal perforation is rarely encountered. This is a report of a 2-day-old boy presenting with a low anorectal malformation, complicated with ileal perforation, necessitating laparotomy and ileal repair. Anoplasty was done for the low anomaly. Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication.

  18. Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Hannah E. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Moore, Kevin R. [University of Utah School of Medicine, Department of Radiology, Salt Lake City, UT (United States); Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2014-11-15

    Complex Chiari malformation is a subgroup of Chiari 1 malformation with distinct imaging features. Children with complex Chiari malformation are reported to have a more severe clinical phenotype and sometimes require more extensive surgical treatment than those with uncomplicated Chiari 1 malformation. We describe reported MR imaging features of complex Chiari malformation and evaluate the utility of craniometric parameters and qualitative anatomical observations for distinguishing complex Chiari malformation from uncomplicated Chiari 1 malformation. We conducted a retrospective search of the institutional imaging database using the keywords ''Chiari'' and ''Chiari 1'' to identify children imaged during the 2006-2011 time period. Children with Chiari 2 malformation were excluded after imaging review. We used the first available diagnostic brain or cervical spine MR study for data measurement. Standard measurements and observations were made of obex level (mm), cerebellar tonsillar descent (mm), perpendicular distance to basion-C2 line (pB-C2, mm), craniocervical angle (degrees), clivus length, and presence or absence of syringohydromyelia, basilar invagination and congenital craniovertebral junction osseous anomalies. After imaging review, we accessed the institutional health care clinical database to determine whether each subject clinically met criteria for Chiari 1 malformation or complex Chiari malformation. Obex level and craniocervical angle measurements showed statistically significant differences between the populations with complex Chiari malformation and uncomplicated Chiari 1 malformation. Cerebellar tonsillar descent and perpendicular distance to basion-C2 line measurements trended toward but did not meet statistical significance. Odontoid retroflexion, craniovertebral junction osseous anomalies, and syringohydromyelia were all observed proportionally more often in children with complex Chiari malformation than in

  19. Skeletal malformations in fetuses with Meckel syndrome

    DEFF Research Database (Denmark)

    Kjaer, K W; Fischer Hansen, B; Keeling, J W;

    1999-01-01

    four types, based on the number and morphology of metacarpals and metatarsals. In the individual fetus there was more often similarity in the pattern of malformation in the two hands or in the two feet than there was between the pattern of malformation seen in the hands and that seen in the feet. Only...... one foot was normal. Malformations of the cranial base (the basilar part of the occipital bone or the postsphenoid bone) occurred in five cases, and the vertebral bodies in the lumbar region of the spine were malformed (cleft) in three cases. It is proposed that a skeletal analysis be included...

  20. Fat deposition in the cavernous sinus in Cushing disease

    Energy Technology Data Exchange (ETDEWEB)

    Bachow, T.B.; Hesselink, J.R.; Aaron, J.O.; Davis, K.R.; Taveras, J.M.

    1984-10-01

    Fat density in the cavernous sinus on computed tomography (CT) is described in 6 out of 16 (37.5%) patients with Cushing disease. This finding may aid in making a specific diagnosis in patients with a pituitary mass. It was not seen in 30 random CT studies of the sella; however, supra seller fat was incidentally noted in the patient with acromegaly.

  1. Carotid-cavernous fistula after functional endoscopic sinus surgery.

    Science.gov (United States)

    Karaman, Emin; Isildak, Huseyin; Haciyev, Yusuf; Kaytaz, Asim; Enver, Ozgun

    2009-03-01

    Carotid-cavernous fistulas (CCFs) are anomalous communications between the carotid arterial system and the venous cavernous sinus. They can arise because of spontaneous or trauma causes. Most caroticocavernous fistulas are of spontaneous origin and unknown etiology. Spontaneous CCF may also be associated with cavernous sinus pathology such as arteriosclerotic changes of the arterial wall, fibromuscular dysplasia, or Ehler-Danlos syndrome. Traumatic CCFs may occur after either blunt or penetrating head trauma. Their clinical presentation is related to their size and to the type of venous drainage, which can lead to a variety of symptoms, such as visual loss, proptosis, bruit, chemosis, cranial nerve impairment, intracranial hemorrhage (rare), and so on. Treatment by endovascular transarterial embolization with electrolytically detachable coils is a very effective method for CCF with good outcomes. Carotid-cavernous fistulas have been rarely reported after craniofacial surgery and are uncommon pathologies in otolaryngology practice. In this study, we report a 40-year-old woman with CCF secondary to blunt trauma of functional endoscopic sinus surgery.

  2. Sensitivity analysis of influencing parameters in cavern stability

    Institute of Scientific and Technical Information of China (English)

    Abolfazl Abdollahipour; Reza Rahmannejad

    2012-01-01

    In order to analyze the stability of the underground rock structures,knowing the sensitivity of geomechanical parameters is important.To investigate the priority of these geomechanical properties in the stability of cavern,a sensitivity analysis has been performed on a single cavern in various rock mass qualities according to RMR using Phase 2.The stability of cavern has been studied by investigating the side wall deformation.Results showed that most sensitive properties are coefficient of lateral stress and modulus of deformation.Also parameters of Hoek-Brown criterion and σc have no sensitivity when cavern is in a perfect elastic state.But in an elasto-plastic state,parameters of Hoek-Brown criterion and σc affect the deformability; such effect becomes more remarkable with increasing plastic area.Other parameters have different sensitivities concerning rock mass quality (RMR).Results have been used to propose the best set of parameters for study on prediction of sidewall displacement.

  3. A Case of Brainstem Cavernous Angioma Presenting with Persistent Hiccups

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Arami

    2010-07-01

    Full Text Available "nIntractable hiccup most be considered as a symptom of underlying serious pathologies. We report a case of medulla oblongata cavernous angima presented with persistant hiccup and without any improvement during routine nonpharmacologic and pharmacologic treatment regimns. The patient is under our follow up visits and surgery is very high risk for this young girl.

  4. Valuation of gas stored in salt cavern facilities

    Energy Technology Data Exchange (ETDEWEB)

    Bond, Michael A. [St. Mary' s University, TX (United States); Grant, Floyd H. [Purdue University, IN (United States)

    2008-07-01

    Since natural gas production is relatively inelastic towards demand in the short term, underground storage is used as a buffer against periods of high demand. Of the three most common storage facility types, depleted reservoirs, aquifers and manmade salt caverns, the latter is the most costly to develop. The challenge then is to maximize profits through efficient operation, well-timed injection and withdrawal of gas. The valuation of a commodity in storage is a challenging problem and has been the subject of study for decades. We investigate selected existing valuation approaches and look for ways to leverage salt-cavern-specific physical characteristics for financial advantage. The basis for our valuation is the Black-Scholes model for pricing options. Then, applying Monte-Carlo methods and simulation, we model combinations of characteristics in multi-cavern facilities and their impact on profitability. We describe the theory behind our work and our analytical framework and provide numerical results of our analysis. Our approach offers increased efficiency in salt-cavern gas storage facility operations. (author)

  5. Lowering End-cap YE-1 in the CMS cavern

    CERN Multimedia

    2008-01-01

    On Tuesday 22 January, the dance of the CMS end-caps came to an end with the lowering of YE-1, the heaviest of them all. After a spectacular lowering operation lsting ten hours, this mammoth component completed the 100-metre descent and was gently placed on the floor of the CMS cavern to the applause of the many onlookers.

  6. Orbital cavernous hemangiomas: ultrasound and magnetic resonance imaging evaluation.

    Science.gov (United States)

    Diamantopoulou, A; Damianidis, Ch; Kyriakou, V; Kotziamani, N; Emmanouilidou, M; Goutsaridou, F; Tsitouridis, I

    2010-03-01

    Cavernous hemangioma is the most common intraorbital lesion in adults. The aim of our study was to evaluate the magnetic resonance imaging (MRI) and ultrasound (US) characteristics of cavernous hemangioma and their role in the differential diagnosis of orbital tumors. Eight patients with orbital cavernous hemangiomas, five women and three men with a mean age of 48 years were examined in a period of six years. All patients underwent MRI examination and four patients were also evaluated by US. In all cases MRI depicted a well-defined intraconal tumor. The lesions were homogeneous, isointense to muscle on T1-weighted sequence and hyperintense to muscle on T2-weighted sequence in six patients. In one patient the mass was isointense on T1WI with heterogeneous signal intensity on T2WI and in one patient the lesion had heterogeneous signal intensity on both T1- and T2-weighted sequences. After intravenous contrast medium administration, the tumors showed initial inhomogeneous enhancement with progressive accumulation of contrast material on delayed images in seven patients and initial homogeneous enhancement in one patient. On ultrasonography, the orbital masses appeared slightly hyperechoic, heterogeneous with small areas of slow blood flow. The analysis of imaging characteristics of a well-defined intraconal lesion in an adult patient with painless progressive proptosis can be highly suggestive of the diagnosis of cavernous hemangioma.

  7. Traumatic basilar pseudoaneurysm with a basilar-cavernous arteriovenous fistula

    Energy Technology Data Exchange (ETDEWEB)

    Connor, S.E.J.; Deasy, N.P.; Jeffree, M.A. [Dept. of Neuroradiology, King' s Coll. Hospital, London (United Kingdom); Martin, A.J.; Strong, A.J. [Dept. of Neurosurgery, King' s Coll. Hospital, London (United Kingdom)

    2001-03-01

    A traumatic pseudoaneurysm of the basilar artery with a basilar-cavernous sinus arteriovenous fistula was diagnosed in a 12-year-old girl using CT, MRI and angiography. It was successfully treated by coil embolisation. We speculate on the mode of formation of this rare traumatic lesion. (orig.)

  8. Fracture and Healing of Rock Salt Related to Salt Caverns

    Energy Technology Data Exchange (ETDEWEB)

    Chan, K.S.; Fossum, A.F.; Munson, D.E.

    1999-03-01

    In recent years, serious investigations of potential extension of the useful life of older caverns or of the use of abandoned caverns for waste disposal have been of interest to the technical community. All of the potential applications depend upon understanding the reamer in which older caverns and sealing systems can fail. Such an understanding will require a more detailed knowledge of the fracture of salt than has been necessary to date. Fortunately, the knowledge of the fracture and healing of salt has made significant advances in the last decade, and is in a position to yield meaningful insights to older cavern behavior. In particular, micromechanical mechanisms of fracture and the concept of a fracture mechanism map have been essential guides, as has the utilization of continuum damage mechanics. The Multimechanism Deformation Coupled Fracture (MDCF) model, which is summarized extensively in this work was developed specifically to treat both the creep and fracture of salt, and was later extended to incorporate the fracture healing process known to occur in rock salt. Fracture in salt is based on the formation and evolution of microfractures, which may take the form of wing tip cracks, either in the body or the boundary of the grain. This type of crack deforms under shear to produce a strain, and furthermore, the opening of the wing cracks produce volume strain or dilatancy. In the presence of a confining pressure, microcrack formation may be suppressed, as is often the case for triaxial compression tests or natural underground stress situations. However, if the confining pressure is insufficient to suppress fracture, then the fractures will evolve with time to give the characteristic tertiary creep response. Two first order kinetics processes, closure of cracks and healing of cracks, control the healing process. Significantly, volume strain produced by microfractures may lead to changes in the permeability of the salt, which can become a major concern in

  9. Acquired Chiari malformation type I associated with a supratentorial fistulous arteriovenous malformation: a case report.

    Science.gov (United States)

    Chen, Kuo-Wei; Kuo, Meng-Fai; Lee, Chung-Wei; Tu, Yong-Kwang

    2015-03-01

    A case of acquired Chiari malformation type I with frontal fistulous arteriovenous malformation (AVM) is presented, and the pathophysiology is discussed. The tonsillar herniation and hydrocephalus both resolved after AVM was excised. This case provides some insight into the complex hemodynamic change exerted by the fistulous AVM and the mechanism of the development of acquired Chiari malformation type I.

  10. Chiari II malformation. Pt. 4

    Energy Technology Data Exchange (ETDEWEB)

    Naidich, T.P.; McLone, D.G.; Fulling, K.H.

    1983-08-01

    Computed tomography successfully delineates the multiple components of the Chiari II malformation at the craniocervical junction, the hindbrain, and the cervical spinal cord. These include wide foramen magnum and upper cervical spinal canal; incomplete fusions of the posterior arches of C1 and lower cervical vertebrae; cascading protrusions of vermis, fourth ventricle, medulla, and cervical cord into the spinal canal; cervicomedullary ''kinking''; anterior displacement and sequential sagittal compression of each protrusion by the protrusions posterior to it; compression of all protrusions by the posterior lip of foramen magnum and the posterior arch of C1; and associated cervical hydromyelia, cervical diastematomyelia, and cervical arachnoid cysts.

  11. Four cases of trisomy 18 syndrome with limb reduction malformations.

    OpenAIRE

    Christianson, A L; Nelson, M. M.

    1984-01-01

    Limb reduction malformations of the arms are well documented in the trisomy 18 syndrome. Four cases of trisomy 18 syndrome with limb reduction malformations of the legs are described and compared with the upper limb malformations.

  12. Kernohan's phenomenon associated with left ruptured occipital arteriovenous malformation.

    Science.gov (United States)

    Fujimoto, A; Sato, H; Katayama, W; Nakai, K; Tsunoda, T; Kobayashi, E; Nose, T

    2004-05-01

    A 23-year-old woman presented with ipsilateral hemiparesia due to rupture of a left occipital arteriovenous malformation (AVM). Emergency decompression (the onset-operation interval was 46 minutes,) was carried out and the patient could leave the hospital upon recovery without neurological deficits. In general, Kernohan's phenomenon is caused by the gradual displacement of the cerebral peduncle against the tentorial edge caused by compression by the contralateral mass. This phenomenon is very rare among the cases with spontaneous intracranial hemorrhage and only three cases including the present one have been reported in the literature. In all cases the onset-operation intervals of were very short. Kernohan's phenomenon associated with a ruptured AVM is a rare condition and emergency decompression is required.

  13. Subfascial involvement in glomuvenous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Shaikh, Raja; Alomari, Ahmad I.; Chaudry, Gulraiz [Boston Children' s Hospital, Division of Interventional Radiology, Boston, MA (United States); Mulliken, John B. [Boston Children' s Hospital, Division of Plastic Surgery, Boston, MA (United States); Fishman, Steven J. [Boston Children' s Hospital, Department of Surgery, Boston, MA (United States); Kozakewich, Harry P.W. [Boston Children' s Hospital, Department of Pathology, Boston, MA (United States)

    2014-07-15

    Glomuvenous malformation (GVM) is an inherited autosomal dominant trait. The lesions, which appear as bluish nodules or plaque-like cutaneous elevations, are usually tender and more firm than sporadic venous malformations. Conventionally, the lesions are thought to be limited to the cutaneous and subcutaneous tissue planes. The objective was to characterize the depth of involvement of GVM lesions. Magnetic resonance imaging (MRI) findings in GVM were retrospectively evaluated by two radiologists. The signal characteristics, tissue distribution, pattern of contrast enhancement of the lesions in GVM were documented. Thirty patients (19 female) aged 1-35 years (mean 18 years) were diagnosed with GVM based on clinical features (n = 20) and/or histopathological findings (n = 10). The lesions were present in the lower extremity (n = 15), upper extremity (n = 6), cervico-facial region (n = 6), pelvis (n = 2), and chest wall (n = 1). All patients had skin and subcutaneous lesions. Fifty percent of the patients (n = 15) demonstrated subfascial intramuscular (n = 15), intra-osseous (n = 1), and intra-articular involvement (n = 1). Contrary to the conventional belief that GVMs are generally limited to the skin and subcutaneous tissue, deep subfascial extension of the lesions is common. (orig.)

  14. Cerebellar arteriovenous malformations in children

    Energy Technology Data Exchange (ETDEWEB)

    Griffiths, P.D. [Sheffield Univ. (United Kingdom). Acad. Dept. of Radiol.; Blaser, S.; Armstrong, D.; Chuang, S.; Harwood-Nash, D. [Division of Neuroradiology, The Hospital for Sick Children and University of Toronto, Toronto (Canada); Humphreys, R.P. [Division of Neurosurgery, The Hospital for Sick Children and University of Toronto, Toronto (Canada)

    1998-05-01

    We review the presentation, imaging findings and outcome in 18 children with cerebellar arteriovenous malformations (AVM). This group is of particular interest because of the reported poor outcome despite modern imaging and neurosurgical techniques. All children had CT and 15 underwent catheter angiography at presentation. Several of the children in the latter part of the study had MRI. Of the 18 children, 17 presented with a ruptured AVM producing intracranial haemorrhage. The remaining child presented with temporal lobe epilepsy and was shown to have temporal, vermian and cerebellar hemisphere AVM. This child had other stigmata of Osler-Weber-Rendu syndrome. Three other children had pre-existing abnormalities of possible relevance. One had a vascular malformation of the cheek and mandible, one a documented chromosomal abnormality and another a midline cleft upper lip and palate. Six of the 17 children with a ruptured cerebellar AVM died within 7 days of the ictus. Vascular pathology other than an AVM was found in 10 of the 14 children with a ruptured cerebellar AVM who had angiography: 4 intranidal aneurysms, 5 venous aneurysms and 2 cases of venous outflow obstruction (one child having both an aneurysm and obstruction). The severity of clinical presentation was directly related to the size of the acute haematoma, which was a reasonable predictor of outcome. (orig.) With 4 figs., 4 tabs., 23 refs.

  15. A measurement of creep and permeability of a salt cavern; Une mesure de la permeabilite et du fluage d`une caverne dans le sel

    Energy Technology Data Exchange (ETDEWEB)

    Berest, P.; Bergues, J.; Brouard, B. [Ecole Polytechnique, (LMS), 91 - Palaiseau (France); Durup, G.; Guerber, B. [Gaz de France (GDF), 93 - La-Plaine-Saint-Denis (France)

    1999-07-01

    Rock mass properties differ significantly from those measured on samples in the laboratory. A test has been performed on a deep brine-filled cavern, with the objective of measuring the equilibrium pressure reached when the cavern was closed. Such an equilibrium is reached when salt mass creep, which leads to cavern shrinkage, balances brine permeation through the cavern wall. A K = 2.10{sup -19} m{sup 2} value of the average in situ intrinsic permeability has been deduced from the test; it is significantly higher than the intrinsic permeability measured in a well or in the laboratory. This result supports cavern abandonment scenarios in which the risk of natural fracturing due to high brine pressures is alleviated. (authors) 14 refs.

  16. Vascular malformations of central nervous system: A series from tertiary care hospital in South India

    Science.gov (United States)

    Karri, Sudhir Babu; Uppin, Megha S.; Rajesh, A.; Ashish, K.; Bhattacharjee, Suchanda; Rani, Y. Jyotsna; Sahu, B. P.; Saradhi, M Vijaya; Purohit, A. K.; Challa, Sundaram

    2016-01-01

    Aims and Objectives: To describe clinicopathological features of surgically resected vascular malformations (VMs) of central nervous system (CNS). Materials and Methods: Histologically diagnosed cases of VMs of CNS during April 2010–April 2014 were included. Demographic data, clinical and radiological features were obtained. Hematoxylin and eosin slides were reviewed along with Verhoeff-Van Gieson (VVG), Masson's trichrome, periodic acid-Schiff, and Perls' stains. Morphologically, cavernomas and arteriovenous malformations (AVMs) were distinguished on the basis of vessel wall features on VVG and intervening glial parenchyma. Results: Fifty cases were diagnosed as VMs of CNS with an age range of 14–62 years. These included 36 cavernomas, 12 AVMs, 2 mixed capillary-cavernous angiomas. Most of the cavernoma patients (15/36) presented with seizures, whereas AVM patients (8/12) had a headache as the dominant symptom. Twenty-nine patients were reliably diagnosed on radiological features. Microscopic evidence of hemorrhage was seen in 24/36 cavernomas and 6/12 AVMs, as opposed to radiologic evidence of 10 and 4, respectively. Reactive gliosis was seen in 16 cavernomas. Conclusions: Histological features are important for classifying the VMs of CNS as there are no specific clinical and radiological features. Type of VM has a bearing on management, prognosis, and risk of hemorrhage. PMID:27114659

  17. Vascular malformations of central nervous system: A series from tertiary care hospital in South India

    Directory of Open Access Journals (Sweden)

    Sudhir Babu Karri

    2016-01-01

    Full Text Available Aims and Objectives: To describe clinicopathological features of surgically resected vascular malformations (VMs of central nervous system (CNS. Materials and Methods: Histologically diagnosed cases of VMs of CNS during April 2010–April 2014 were included. Demographic data, clinical and radiological features were obtained. Hematoxylin and eosin slides were reviewed along with Verhoeff-Van Gieson (VVG, Masson's trichrome, periodic acid-Schiff, and Perls' stains. Morphologically, cavernomas and arteriovenous malformations (AVMs were distinguished on the basis of vessel wall features on VVG and intervening glial parenchyma. Results: Fifty cases were diagnosed as VMs of CNS with an age range of 14–62 years. These included 36 cavernomas, 12 AVMs, 2 mixed capillary-cavernous angiomas. Most of the cavernoma patients (15/36 presented with seizures, whereas AVM patients (8/12 had a headache as the dominant symptom. Twenty-nine patients were reliably diagnosed on radiological features. Microscopic evidence of hemorrhage was seen in 24/36 cavernomas and 6/12 AVMs, as opposed to radiologic evidence of 10 and 4, respectively. Reactive gliosis was seen in 16 cavernomas. Conclusions: Histological features are important for classifying the VMs of CNS as there are no specific clinical and radiological features. Type of VM has a bearing on management, prognosis, and risk of hemorrhage.

  18. Sensitivity of storage field performance to geologic and cavern design parameters in salt domes.

    Energy Technology Data Exchange (ETDEWEB)

    Ehgartner, Brian L. (Sandia National Laboratories, Albuquerque, NM); Park, Byoung Yoon

    2009-03-01

    A sensitivity study was performed utilizing a three dimensional finite element model to assess allowable cavern field sizes for strategic petroleum reserve salt domes. A potential exists for tensile fracturing and dilatancy damage to salt that can compromise the integrity of a cavern field in situations where high extraction ratios exist. The effects of salt creep rate, depth of salt dome top, dome size, caprock thickness, elastic moduli of caprock and surrounding rock, lateral stress ratio of surrounding rock, cavern size, depth of cavern, and number of caverns are examined numerically. As a result, a correlation table between the parameters and the impact on the performance of storage field was established. In general, slower salt creep rates, deeper depth of salt dome top, larger elastic moduli of caprock and surrounding rock, and a smaller radius of cavern are better for structural performance of the salt dome.

  19. Acute Hemiparesis in a Child as a Presenting Symptom of Hemispheric Cerebral Proliferative Angiopathy

    Directory of Open Access Journals (Sweden)

    J. J. Gold

    2013-01-01

    Full Text Available A 9-year-old girl with a several-month history of unilateral intermittent headaches presented to the hospital with worsening headaches and unsteadiness. Neurologic exam was positive for a mild right hemiparesis and right homonymous hemianopsia. Noncontrast computed tomography revealed an engorged sagittal and straight sinus with prominent cortical veins concerning an arteriovenous malformation and the patient was admitted to the pediatric intensive care unit. Computed tomography angiogram demonstrated a left hemispheric vascular malformation, without evidence of dural arteriovenous fistula on conventional angiogram consistent with a diagnosis of cerebral proliferative angiopathy. There was no evidence of infarct on magnetic resonance imaging, and the patient’s symptoms were completely resolved within 24 hours. Cerebral proliferative angiopathy is a rare but important vascular malformation distinct from classic arteriovenous malformations that may present with stroke-like symptoms in childhood.

  20. Cluster Headache Secondary to Macroprolactinoma with Ipsilateral Cavernous Sinus Invasion

    Directory of Open Access Journals (Sweden)

    M. J. Levy

    2012-01-01

    Full Text Available We present a 25 year-old man with episodic cluster headache that was refractory to all standard pharmacological prophylactic and abortive treatments. Because of the lack of response, an MRI brain was performed which showed a large pituitary tumour with ipsilateral cavernous sinus invasion. The serum prolactin was significantly elevated at 54,700 miU/L (50–400 confirming a macro-prolactinoma. Within a few days of cabergoline therapy the headache resolved. He continues to be headache free several years after starting the dopamine agonist. This case highlights the importance of imaging the pituitary fossa in patients with refractory cluster headache, It also raises the potential anatomical importance of the cavernous sinus in pituitary-associated headache.

  1. Cavernous Hemangioma of the Rib: A Rare Diagnosis

    Directory of Open Access Journals (Sweden)

    Stavros Gourgiotis

    2010-01-01

    Full Text Available Hemangioma of the rib is an uncommon benign vascular tumour. A case of rib hemangioma in a 29-year-old woman is presented. Chest roentgenogram and computed tomography revealed a mass along the inner surface of the 7th left rib with bone destruction. She underwent resection of the 7th rib. The pathologic diagnosis was cavernous hemangioma. Hemangiomas of the rib are rare tumours but should be kept in mind in the differential diagnosis of rib tumours.

  2. Characterization of Live Birth with Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Carlos Acosta Batista

    2015-06-01

    Full Text Available Background: the congenital malformations constitute the first cause of infantile death in developed countries, as well as the second cause of death in Cuba, in younger children of an elderly year. Objective: characterizing the live birth newborns with congenital malformations at Marianao municipality during the year 2011. Methods: descriptive, cross-section study of 30- live birth with congenital malformations at Marianao municipality in Havana, during the year 2011. Some of analyzed variables were: sex, affected system, congenital malformation, type of malformation, severity, birth weight, gestational age, prenatal diagnosis, family history of congenital malformation, maternal age, among others. Results: the masculine sex was the more affected, with 18 cases that represented the 60 %. The Polydactyl was the malformation further frequent, with 23.3 %, followed by the pre-aural appendix, with 10 %. The 20 % was born pre-term and only in the 10 % of the cases was obtained a positive result in the tests of prenatal diagnosis. Conclusions: the live birth with MC were characterized to be males with isolated MC and less severe, full term, normopeso, without family history of the aforementioned affection and with a negative prenatal diagnosis. The majority of mothers belonged to 20-35's age group years, they suffered from hypertension and during pregnancy, the principal diseases that they presented were the sepsis and anemia.

  3. [Condition of cavernous tissue of the penis after unilateral incomplete proximal intracavernous falloprosthesis].

    Science.gov (United States)

    Taurashvili, G I; Medvedev, V L; Chilov, S A; Kochov, V N; Taruashvili, I G

    2011-01-01

    Unilateral incomplete proximal intracavernous falloprosthesis (UIPIF) was followed by US-dopplerography of the intact penis and penis in medical erection induced by E1 prostoglandin introduction into the intact cavernous body. UIPIF preserves 89-90% of functionally active cavernous tissue with effective blood flow. Investigation 2 months and more after operation demonstrated that systolic blood flow rate in the cavernous artery of the cavernous tissue around the endoprosthersis can reach values registered in normal erection. The conclusion is made that after UIPIF quality of sexual life of the patient improves due to creation of additional density and axial stability of the trunk of the penis.

  4. Isolated oculomotor nerve palsy caused by cavernous sinus dural arteriovenous fistula: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Ihn, Yon Kwon; Jung, Won Sang [The Catholic Univ. of Korea, Suwon (Korea, Republic of); Kim, Bum Soo [The Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2012-10-15

    Cavernous dural arteriovenous fistula (DAVF), which usually presents with conjunctival injection, proptosis, loss of visual acuity, and ophthalmoplegia, is a rare cause of ophthalmoplegia. Thus, it may be overlooked when the typical symptoms are lacking. There have been some cavernous DAVF case reports presenting with isolated oculomotor, abducens and trochlear nerve palsy. We report a patient presenting with isolated oculomotor palsy, caused by cavernous DAVF, which was treated by transvenous coil embolization. This case suggests that cavernous DAVF should be considered in the differential diagnosis of isolated oculomotor nerve palsy and for which case - selective angiography and embolization may be helpful in reaching a diagnosis and providing a guide for optimal treatment.

  5. Geotechnical issues and guidelines for storage of compressed air in excavated hard rock caverns

    Energy Technology Data Exchange (ETDEWEB)

    Allen, R.D.; Doherty, T.J.; Fossum, A.F.

    1982-04-01

    The results of a literature survey on the stability of excavated hard rock caverns are presented. The objective of the study was to develop geotechnical criteria for the design of compressed air energy storage (CAES) caverns in hard rock formations. These criteria involve geologic, hydrological, geochemical, geothermal, and in situ stress state characteristics of generic rock masses. Their relevance to CAES caverns, and the identification of required research areas, are identified throughout the text. This literature survey and analysis strongly suggests that the chief geotechnical issues for the development and operation of CAES caverns in hard rock are impermeability for containment, stability for sound openings, and hydrostatic balance.

  6. Post-traumatic carotid cavernous fistula of late manifestation report

    Directory of Open Access Journals (Sweden)

    Joanna Roskal-Wałek

    2015-01-01

    Full Text Available Carotid cavernous fistula (CCF is an abnormal connection between the carotid artery and the cavernous sinus. Carotid cavernous fistula of traumatic aetiology occurs more commonly. The characteristic triad of symptoms covers the following: pulsating exophthalmos, a humming sound within the skull, and dilation and tortuosity of conjunctival and episcleral veins. The diagnosis of CCF may constitute a diagnostic problem in the situation when the symptoms occur several weeks after injury, it may overlap with other post-traumatic changes, and the dominant symptom may be cranial nerve palsy. The lack of a correct diagnosis and adequate causative therapy creates the risk of not only loss of vision, but also of life. We present a case of a patient who developed fistula symptoms a few weeks after an injury. The diagnosis of post-traumatic CCF was confirmed by imaging examinations. The application of transluminal embolisation led to the resolution of the majority of symptoms.

  7. Intraosseous Cavernous Hemangioma in the Mandible: A Case Report.

    Science.gov (United States)

    Elif, Bilgir; Derya, Yildirim; Gulperi, Kocer; Sevgi, Bozova

    2017-01-01

    Intraosseous vascular lesions are rare conditions. They are most commonly seen in the vertebral column and skull; nevertheless, the mandible is a quite rare location. In this report, we present a case of intraosseous cavernous hemangioma in the mandible and discuss the clinical and radiological features. A 28-year-old male patient attended to our clinic with a complaint of painless swelling of mandible. Clinical evaluation revealed a bone-hard, smooth-surfaced, immobile mass in the left mandibular lingual area. The patient was evaluated with panoramic and occlusal radiography and computed tomography. The lesion surgically excised and pathological examination revealed an intraosseous cavernous hemangioma. Follow-up imaging 1 year later with cone beam computed tomography revealed recurrence of the lesion. The conclusion of this paper; when a bone hard, well-shaped mass was seen in the mandible, the possibility of intraosseous hemangioma must be remembered and before surgical procedure detailed radiographic evaluation should be performed. Key words:Hemangioma, intraosseous, mandible, cavernous, cbct.

  8. Intraosseous Cavernous Hemangioma in the Mandible: A Case Report

    Science.gov (United States)

    Derya, Yildirim; Gulperi, Kocer; Sevgi, Bozova

    2017-01-01

    Intraosseous vascular lesions are rare conditions. They are most commonly seen in the vertebral column and skull; nevertheless, the mandible is a quite rare location. In this report, we present a case of intraosseous cavernous hemangioma in the mandible and discuss the clinical and radiological features. A 28-year-old male patient attended to our clinic with a complaint of painless swelling of mandible. Clinical evaluation revealed a bone-hard, smooth-surfaced, immobile mass in the left mandibular lingual area. The patient was evaluated with panoramic and occlusal radiography and computed tomography. The lesion surgically excised and pathological examination revealed an intraosseous cavernous hemangioma. Follow-up imaging 1 year later with cone beam computed tomography revealed recurrence of the lesion. The conclusion of this paper; when a bone hard, well-shaped mass was seen in the mandible, the possibility of intraosseous hemangioma must be remembered and before surgical procedure detailed radiographic evaluation should be performed. Key words:Hemangioma, intraosseous, mandible, cavernous, cbct. PMID:28149481

  9. Gas hydrates in gas storage caverns; Gashydrate bei der Gaskavernenspeicherung

    Energy Technology Data Exchange (ETDEWEB)

    Groenefeld, P. [Kavernen Bau- und Betriebs-GmbH, Hannover (Germany)

    1997-12-31

    Given appropriate pressure and temperature conditions the storage of natural gas in salt caverns can lead to the formation of gas hydrates in the producing well or aboveground operating facilities. This is attributable to the stored gas becoming more or less saturated with water vapour. The present contribution describes the humidity, pressure, and temperature conditions conducive to gas hydrate formation. It also deals with the reduction of the gas removal capacity resulting from gas hydrate formation, and possible measures for preventing hydrate formation such as injection of glycol, the reduction of water vapour absorption from the cavern sump, and dewatering of the cavern sump. (MSK) [Deutsch] Bei der Speicherung von Erdgas in Salzkavernen kann es unter entsprechenden Druck- und Temperaturverhaeltnissen zur Gashydratbildung in den Foerdersonden oder obertaegigen Betriebseinrichtungen kommen, weil sich das eingelagerte Gas mehr oder weniger mit Wasserdampf aufsaettigt. Im Folgenden werden die Feuchtigkeits-, Druck- und Temperaturbedingungen, die zur Hydratbildung fuehren erlaeutert. Ebenso werden die Verringerung der Auslagerungskapazitaet durch die Hydratbildung, Massnahmen zur Verhinderung der Hydratbildung wie die Injektion von Glykol, die Verringerung der Wasserdampfaufnahme aus dem Kavernensumpf und die Entwaesserung der Kavernensumpfs selbst beschrieben.

  10. Bryan Mound SPR cavern 113 remedial leach stage 1 analysis.

    Energy Technology Data Exchange (ETDEWEB)

    Rudeen, David Keith; Weber, Paula D.; Lord, David L.

    2013-08-01

    The U.S. Strategic Petroleum Reserve implemented the first stage of a leach plan in 2011-2012 to expand storage volume in the existing Bryan Mound 113 cavern from a starting volume of 7.4 million barrels (MMB) to its design volume of 11.2 MMB. The first stage was terminated several months earlier than expected in August, 2012, as the upper section of the leach zone expanded outward more quickly than design. The oil-brine interface was then re-positioned with the intent to resume leaching in the second stage configuration. This report evaluates the as-built configuration of the cavern at the end of the first stage, and recommends changes to the second stage plan in order to accommodate for the variance between the first stage plan and the as-built cavern. SANSMIC leach code simulations are presented and compared with sonar surveys in order to aid in the analysis and offer projections of likely outcomes from the revised plan for the second stage leach.

  11. Vasoespasmo cerebral

    Directory of Open Access Journals (Sweden)

    Antonio A. F. de Salles

    1987-09-01

    Full Text Available Vasoespasmo cerebral ocorre em patologias como enxaqueca, hemorragia subaracnóidea, trauma de crânio, após isquemia e/ou hipoxia. A fisiopatologia do vasoespasmo cerebral nestas patologias não está completamente desvendada. Neste artigo são analisados os fatores neuroquímicos e morfológicos responsáveis pelo controle circulatório cerebral. As alterações circulatórias que seguem a hemorragia subaracnóidea são utilizadas como exemplo. Conclui-se que fatores bioquímicos, fisiológicos e morfológicos são responsáveis pelas manifestações vasculares que ocorrem após a hemorragia subaracnóidea. Alternativas de tratamento do vasoespasmo cerebral são discutidas.

  12. Computed tomography of congenital brain malformations

    Energy Technology Data Exchange (ETDEWEB)

    Sarwar, M.

    1984-01-01

    This book is illustrated showing each condition. This book is designed to correlate the pathology of CNS malformations with their CT scan appearance, mainly on the axial images. The author has drawn upon his personal experience and the information gleaned from the literature dealing with the description of the CT scan findings of these malformations. The emphasis is on simplicity of description. Since a large degree of morphological variation exists in each entity, numerous illustrations (wherever applicable) are shown to depict those variations. When appropriate, deficiency of the CT scan in the evaluation of these CNS malformations also is indicated. A description of CNS embryology is included as well.

  13. Anterior commissure absence without callosal agenesis: a new brain malformation.

    Science.gov (United States)

    Mitchell, T N; Stevens, J M; Free, S L; Sander, J W; Shorvon, S D; Sisodiya, S M

    2002-04-23

    The authors report a novel human brain malformation characterized by the absence of the anterior commissure without callosal agenesis, but associated with gross unilateral panhemispheric malformation incorporating subependymal heterotopia, subcortical heterotopia, and gyral abnormalities including temporal malformation and polymicrogyria. In contrast, a normal anterior commissure was found in 125 control subjects and in 113 other subjects with a range of brain malformations.

  14. Cerebral Paragonimiasis.

    Science.gov (United States)

    Miyazaki, I

    1975-01-01

    The first case of cerebral paragonimiasis was reported by Otani in Japan in 1887. This was nine years after Kerbert's discovery of the fluke in the lungs of Bengal tigers and seven years after a human pulmonary infection by the fluke was demonstrated by Baelz and Manson. The first case was a 26-year-old man who had been suffering from cough and hemosputum for one year. The patient developed convulsive seizures with subsequent coma and died. The postmortem examination showed cystic lesions in the right frontal and occipital lobes. An adult fluke was found in the occipital lesion and another was seen in a gross specimen of normal brain tissue around the affected occipital lobe. Two years after Otani's discovery, at autopsy a 29-year-old man with a history of Jacksonian seizure was reported as having cerebral paragonimiasis. Some time later, however, it was confirmed that the case was actually cerebral schistosomiasis japonica. Subsequently, cases of cerebral paragonimiasis were reported. However, the majority of these cases were not confirmed histologically. It was pointed out that some of these early cases were probably not Paragonimus infection. After World War II, reviews as well as case reports were published. Recently, investigations have been reported from Korea, with a clinicla study on 62 cases of cerebral paragonimiasis seen at the Neurology Department of the National Medical Center, Seoul, between 1958 and 1964. In 1971 Higashi described a statistical study on 105 cases of cerebral paragonimiasis that had been treated surgically in Japan.

  15. Geomechanical Analysis and Design Considerations for Thin-Bedded Salt Caverns

    Energy Technology Data Exchange (ETDEWEB)

    Michael S. Bruno

    2005-06-15

    The bedded salt formations located throughout the United States are layered and interspersed with non-salt materials such as anhydrite, shale, dolomite and limestone. The salt layers often contain significant impurities. GRI and DOE have initialized this research proposal in order to increase the gas storage capabilities by providing operators with improved geotechnical design and operating guidelines for thin bedded salt caverns. Terralog has summarized the geologic conditions, pressure conditions, and critical design factors that may lead to: (1) Fracture in heterogeneous materials; (2) Differential deformation and bedding plane slip; (3) Propagation of damage around single and multiple cavern; and (4) Improved design recommendations for single and multiple cavern configurations in various bedded salt environments. The existing caverns within both the Permian Basin Complex and the Michigan and Appalachian Basins are normally found between 300 m to 1,000 m (1,000 ft to 3,300 ft) depth depending on local geology and salt dissolution depth. Currently, active cavern operations are found in the Midland and Anadarko Basins within the Permian Basin Complex and in the Appalachian and Michigan Basins. The Palo Duro and Delaware Basins within the Permian Basin Complex also offer salt cavern development potential. Terralog developed a number of numerical models for caverns located in thin bedded salt. A modified creep viscoplastic model has been developed and implemented in Flac3D to simulate the response of salt at the Permian, Michigan and Appalachian Basins. The formulation of the viscoplastic salt model, which is based on an empirical creep law developed for Waste Isolation Pilot Plant (WIPP) Program, is combined with the Drucker-Prager model to include the formation of damage and failure. The Permian salt lab test data provided by Pfeifle et al. 1983, are used to validate the assumptions made in the material model development. For the actual cavern simulations two

  16. [Central nervous system malformations: neurosurgery correlates].

    Science.gov (United States)

    Jiménez-León, Juan C; Betancourt-Fursow, Yaline M; Jiménez-Betancourt, Cristina S

    2013-09-06

    Congenital malformations of the central nervous system are related to alterations in neural tube formation, including most of the neurosurgical management entities, dysraphism and craniosynostosis; alterations of neuronal proliferation; megalencefaly and microcephaly; abnormal neuronal migration, lissencephaly, pachygyria, schizencephaly, agenesis of the corpus callosum, heterotopia and cortical dysplasia, spinal malformations and spinal dysraphism. We expose the classification of different central nervous system malformations that can be corrected by surgery in the shortest possible time and involving genesis mechanisms of these injuries getting better studied from neurogenic and neuroembryological fields, this involves connecting innovative knowledge areas where alteration mechanisms in dorsal induction (neural tube) and ventral induction (telencephalization) with the current way of correction, as well as the anomalies of cell proliferation and differentiation of neuronal migration and finally the complex malformations affecting the posterior fossa and current possibilities of correcting them.

  17. Vein of Galen Malformation: Outcome after Embolization

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2003-12-01

    Full Text Available The neurodevelopmental outcome after endovascular treatment of vein of Galen malformation (VOGM in 27 patients seen between 1983 and 2002 was assessed by chart review and parental questionnaires at the University of California, San Francisco.

  18. Human malformations induced by environmental noxae

    Energy Technology Data Exchange (ETDEWEB)

    Hecker, W.C.; Angerpointner, T.A.

    1980-01-01

    The paper reviews congenital malformations in humans and presents possible causes. 60% of all malformations are a result of environmental and other factors; i.e. not hereditary or caused by a disease of the mother. The teratogenic effects of ionizing radiation, drugs, alcohol, polyvinyl chloride and trichlorophenol are discussed as well as the effect of the mother's working in certain fields, e.g. clinical laboratories or printing offices; in the latter case the teratogenic noxae are still unknown. Efficient research requires centralized storage of all data on children born with malformations and on the mother's health situation during pregnancy, and the legislator is asked to do so while observing the law on data protection. Foundation of a German Institute of Teratology is recommended. In order to intensify research, it is suggested to set up groups or departments for research on malformations in some major paediatric hospitals.

  19. Inner ear malformations: a practical diagnostic approach.

    Science.gov (United States)

    Mazón, M; Pont, E; Montoya-Filardi, A; Carreres-Polo, J; Más-Estellés, F

    2016-12-29

    Pediatric sensorineural hearing loss is a major cause of disability; although inner ear malformations account for only 20-40% of all cases, recognition and characterization will be vital for the proper management of these patients. In this article relevant anatomy and development of inner ear are surveyed. The role of neuroimaging in pediatric sensorineural hearing loss and cochlear preimplantation study are assessed. The need for a universal system of classification of inner ear malformations with therapeutic and prognostic implications is highlighted. And finally, the radiological findings of each type of malformation are concisely described and depicted. Computed tomography and magnetic resonance imaging play a crucial role in the characterization of inner ear malformations and allow the assessment of the anatomical structures that enable the selection of appropriate treatment and surgical approach.

  20. What Is an Arteriovenous Malformation (AVM)?

    Science.gov (United States)

    ... T. Quiz 5 Things to Know About Stroke What Is an Arteriovenous Malformation (AVM)? Updated:Nov 21, ... About AVMs Symptoms and Bleeding Diagnosis and Treatment What is a brain AVM? Normally, arteries carry blood ...

  1. Presenting Symptoms of Chiari Type I Malformation

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-06-01

    Full Text Available Clinical and radiographic predictors of neurologic symptoms were investigated in a population-based retrospective study of 51 children identified with Chiari I malformation at the University of California, and Kaiser Department of Radiology, San Francisco.

  2. Glomuvenous malformation: magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Flors, Lucia; Norton, Patrick T.; Hagspiel, Klaus D. [University of Virginia Health System, Department of Radiology and Medical Imaging, Charlottesville, VA (United States)

    2014-07-05

    We report a case of a glomuvenous malformation involving the dorsal aspect of the right hand and distal forearm in an 11-year-old boy. He had a history of multiple vascular anomalies since birth and presented with increasing right hand pain. MRI played an important role in characterizing and determining the extent of the lesion. In particular, dynamic time-resolved contrast-enhanced MR angiography precisely defined its vascularity. The diagnosis was made histopathologically after partial resection of the lesion. Glomuvenous malformation is a rare developmental hamartoma that originates from the glomus body. Clinically they usually resemble a venous malformation but they are a different entity. In the appropriate clinical setting this rare condition must be included in the differential diagnosis of a vascular malformation, especially when subtle arterial enhancement, early venous shunting and progressive filling of dilated venous spaces are depicted on MRA. (orig.)

  3. Analysis of cavern and well stability at the West Hackberry SPR site using a full-dome model.

    Energy Technology Data Exchange (ETDEWEB)

    Sobolik, Steven R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2015-08-01

    This report presents computational analyses that simulate the structural response of caverns at the Strategic Petroleum Reserve (SPR) West Hackberry site. The cavern field comprises 22 caverns. Five caverns (6, 7, 8, 9, 11) were acquired from industry and have unusual shapes and a history dating back to 1946. The other 17 caverns (101-117) were leached according to SPR standards in the mid-1980s and have tall cylindrical shapes. The history of the caverns and their shapes are simulated in a three-dimensional geomechanics model of the site that predicts deformations, strains, and stresses. Future leaching scenarios corresponding to oil drawdowns using fresh water are also simulated by increasing the volume of the caverns. Cavern pressures are varied in the model to capture operational practices in the field. The results of the finite element model are interpreted to provide information on the current and future status of subsidence, well integrity, and cavern stability. The most significant results in this report are relevant to Cavern 6. The cavern is shaped like a bowl with a large ceiling span and is in close proximity to Cavern 9. The analyses predict tensile stresses at the edge of the ceiling during repressurization of Cavern 6 following workover conditions. During a workover the cavern is at low pressure to service a well. The wellhead pressures are atmospheric. When the workover is complete, the cavern is repressurized. The resulting elastic stresses are sufficient to cause tension around the edge of the large ceiling span. With time, these stresses relax to a compressive state because of salt creep. However, the potential for salt fracture and propagation exists, particularly towards Cavern 9. With only 200 feet of salt between the caverns, the operational consequences must be examined if the two caverns become connected. A critical time may be during a workover of Cavern 9 in part because of the operational vulnerabilities, but also because dilatant damage

  4. Cirurgia estereotáxica guiada para angiomas cavernosos Stereotactic-guided surgery for cavernous angioma

    Directory of Open Access Journals (Sweden)

    MURILO S. MENESES

    2000-03-01

    Full Text Available Os angiomas cavernosos intracerebrais são lesões que podem causar hemorragias, crises convulsivas e déficits neurológicos. Essa patologia passou a ter diagnóstico mais precoce com o advento da ressonância magnética. A remoção radical por cirurgia é o melhor método de tratamento. Devido a frequente localização subcortical ou profunda, a utilização de métodos de localização por imagem como a estereotaxia apresentam várias vantagens como: menor incisão de pele, craniotomia e manipulação do tecido cerebral, consequentemente com menor morbidade. Apresentamos uma série de nove angiomas cavernosos tratados por ressecção cirúrgica radical guiada por estereotaxia. Em todos os casos o diagnóstico foi realizado por ressonância magnética e houve confirmação anátomo-patológica. A média de idade dos pacientes foi 30 anos, variando entre 20 e 54 anos. Como morbidade transitória, um paciente teve uma crise convulsiva no 3° dia pós-operatório e uma paciente apresentou disfasia e hemiparesia no 2° dia pós-operatório, com recuperação total na evolução. No total, a remoção foi radical em todos os casos e os pacientes permanecem sem nenhum déficit neurológico relacionado à cirurgia.Intracerebral cavernous angiomas may cause hemorrhage, epileptic seizures and neurological deficits. The diagnosis of these lesions became easier with the advent of the magnetic resonance image (MRI. Radical resection is the treatment of choice. Due to frequent subcortical or deep location, image-guided techniques, such as stereotactic-guided surgery, offer many advantages as smaller skin incision and craniotomy, less brain manipulation with consequently lower morbidity. We present a series of nine cavernous angiomas treated by stereotactic-guided radical surgical resection. The diagnosis was done by MRI and confirmed by pathologic studies in all cases. Mean age of patients was 30 years old (range 20-54 years. Postoperative morbidity

  5. Animal models for human craniofacial malformations.

    Science.gov (United States)

    Johnston, M C; Bronsky, P T

    1991-01-01

    Holoprosencephaly malformations, of which the fetal alcohol syndrome appears to be a mild form, can result from medial anterior neural plate deficiencies as demonstrated in an ethanol treated animal model. These malformations are associated with more medial positioning of the nasal placodes and resulting underdevelopment or absence of the medial nasal prominences (MNPs) and their derivatives. Malformations seen in the human retinoic acid syndrome (RAS) can be produced by administration of the drug 13-cis-retinoic acid in animals. Primary effects on neural crest cells account for most of these RAS malformations. Many of the malformations seen in the RAS are similar to those of hemifacial microsomia, suggesting similar neural crest involvement. Excessive cell death, apparently limited to trigeminal ganglion neuroblasts of placodal origin, follows 13-cis retinoic acid administration at the time of ganglion formation and leads to malformations virtually identical to those of the Treacher Collins syndrome (TCS). Secondary effects on neural crest cells in the area of the ganglion appear to be responsible for the TCS malformations. Malformations of the DiGeorge Syndrome are similar to those of the RAS and can be produced in mice by ethanol administration or by "knocking out" a homeobox gene (box 1.5). Human and animal studies indicate that cleft lips of multifactorial etiology may be generically susceptible because of small MNP)s or other MNP developmental alterations, such as those found in A/J mice, that make prominence contact more difficult. Experimental maternal hypoxia in mice indicates that cigarette smoking may increase the incidence of cleft lip by interfering with morphogenetic movements. Other human cleft lips may result from the action of a single major gene coding for TGF-alpha variants. A study with mouse palatal shelves in culture and other information suggest that a fusion problem may be involved.

  6. Syringomyelia associated with Chiari malformation in children

    Energy Technology Data Exchange (ETDEWEB)

    Sakamoto, Hiroaki; Kitano, Shouhei; Nishikawa, Misao; Yasui, Toshihiro [Osaka City General Hospital (Japan); Fujitani, Ken; Hakuba, Akira; Nakanishi, Naruhiko

    1997-02-01

    Among 28 patients with myelomeningocele (MMC group), the myelomeningocele in all patients was repaired shortly after birth, and a shunt was implanted for the associated hydrocephalus in 18 patients. MRI of the group of 28 indicated 20 were afflicted with Chiari II malformation, and the remaining 8 by Chiari I malformation. Among 8 patients lacking myelomeningocele (non-MMC group), seven demonstrated a large syrinx at the cervical and cervico-thoracic level; only one had a syrinx extending from the cervical level down to the lumbar level. None of these patients had hydrocephalus. Surgical decompression to improve cerebrospinal fluid (CSF) flow at the major cistern improved neurological signs in 7 patients. MRI indicated 4 patients were afflicted with Chiari I malformation, and the remaining 4 with Chiari II malformation. In the MMC group, the initial development of the syrinx at the lumbar level may be the result of a combination of occlusion of the caudal end of the central canal brought about by repair of the myelomeningocele and CSF flow into the hydromyelic cavity via the patent proximal portion of the central canal. In the non-MMC group, the syringomyelia may be considered an early onset type of syringomyelia associated with adult type Chiari malformation because the location of the syrinx was quite similar to that found in adult type Chiari malformation, and decompressive surgery was quite effective. In the non-MMC group, turbulence of the CSF now at the major cistern caused by the herniated cerebellum plays an important role in the enlargement of the syringomyelia. To offer greater appropriate management of pediatric Chiari malformation accompanied by syringomyelia, the malformation should be classified not by degree of the herniated brain tissue but by its association with neural tube defect (myelomeningocele). (K.H.)

  7. Terminal hemimyelocystocele associated with Chiari II malformation

    Directory of Open Access Journals (Sweden)

    Umamaheswara Reddy V.

    2014-06-01

    Full Text Available Terminal myelocystocele (TMC results from failure of embryonic CSF to drain outside the neural tube creating CSF reservoir within a dorsal meningocele. Association of Chiari II malformation with diastematomyelia and myelocystocele is extremely rare. Myelocystoceles do not have neural tissue so they have good prognosis after treatment, however when associated with hydromelia and Chiari malformation they present with neurological deficits. We present details of a 2 year old female who presented to us with this rare anomaly.

  8. Congenital pseudoarthrosis associated with venous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

    2007-06-15

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  9. Capillary malformation--arteriovenous malformation syndrome: review of the literature, proposed diagnostic criteria, and recommendations for management.

    Science.gov (United States)

    Orme, Charisse M; Boyden, Lynn M; Choate, Keith A; Antaya, Richard J; King, Brett A

    2013-01-01

    Capillary malformation-arteriovenous malformation syndrome is an autosomal dominant disorder caused by mutations in the RASA1 gene and characterized by multiple small, round to oval capillary malformations with or without arteriovenous malformations. Ateriovenous malformations occur in up to one-third of patients and may involve the brain and spine. Although making the diagnosis is straightforward in some patients, there are other patients for whom diagnostic criteria may be helpful in their evaluation. Here we review the literature regarding capillary malformation-arteriovenous malformation syndrome, propose diagnostic criteria, and discuss the care of patients with this condition.

  10. Features of West Hackberry SPR Caverns and Internal Structure Of the Salt Dome

    Energy Technology Data Exchange (ETDEWEB)

    Munson, Darrell Eugene [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States). Underground Storage Technology Dept.

    2006-09-01

    The intent of this report is to examine the internal structure of the West Hackberry salt dome utilizing the information from the geometric configuration of the internal cavern surfaces obtained from graphical representations of sonar survey data. In a general sense, the caverns of West Hackberry are remarkable in the symmetry of their shapes. There are only rather moderate deviations from what would be considered an ideal cylindrical solution mining geometry in these caverns. This finding is in marked contrast to the directional solutioning found in the elliptical cross sectioned, sometimes winged, caverns of Big Hill. None of the persistent lineaments prevalent in Big Hill caverns are evident in West Hackberry caverns. Irregularities of the West Hackberry caverns are restricted to preferential solution formed pits and protuberances with moderate dimensions. In fact, the principal characteristic of West Hackberry caverns is the often large sections of smooth and cylindrical cavern wall. Differences in the cavern characteristics between West Hackberry and Big Hill suggest that the former dome is quite homogeneous, while the latter still retains strong remnants of the interbeds of the original bedded Louann salt. One possible explanation is that the source of the two domes, while both from the Louann mother salt, differs. While the source of the Big Hill dome is directly from the mother salt bed, it appears that the West Hackberry arises from a laterally extruded sill of the mother salt. Consequently, the amount of deformation, and hence, mixing of the salt and interbed material in the extruded sill is significantly greater than would be the case for the directly formed diapir. In West Hackberry, remnants of interbeds apparently no longer exist. An important aspect of the construction of the West Hackberry caverns is the evidence of an attempt to use a uniform solutioning construction practice. This uniformity involved the utilization of single well solutioning and

  11. Vascular plug for ICA occlusion in cavernous carotid aneurysms: technical note

    Energy Technology Data Exchange (ETDEWEB)

    Scott, David A.; Keston, Peter; White, Philip; Sellar, Robin [Western General Hospital, Division of Clinical Neurosciences, Edinburgh (United Kingdom)

    2008-09-15

    Large, symptomatic aneurysms of the cavernous internal carotid artery (ICA) can be successfully treated by a combination of aneurysm coiling and occlusion of the parent vessel. We describe the use of an Amplatzer (AGA medical corporation, Plymouth, MA, USA) detachable nitinol vascular plug to occlude the ICA in four patients with symptomatic cavernous ICA aneurysms. (orig.)

  12. Tuberculoma of the Cavernous Sinus and Meckel's Cave in a Child.

    Science.gov (United States)

    Kumar, V R Roopesh; Madhugiri, Venkatesh S; Verma, Surendra Kumar; Barathi, S Deepak; Yadav, Awdhesh Kumar; Bidkar, Prasanna

    2013-01-01

    Tuberculous infection of the cavernous sinus and Meckel's cave is extremely rare. In this report, we describe a patient with tuberculoma of the cavernous sinus and Meckel's cave, extending to the petrous apex. The patient underwent microsurgical excision of the lesion and antitubercular chemotherapy resulting in a good outcome. We describe the diagnostic difficulties and review the relevant literature.

  13. Endovascular treatment with transvenous approach for embolization of carotid cavernous fistula

    Directory of Open Access Journals (Sweden)

    Benny Young

    2014-10-01

    Full Text Available The aim of endovascular treatment in carotid cavernous fistula is to close the tear side at cavernous sinus part. It can be dealt either by transvenous or transarterial approach. The option is influenced by type of fistula and angioarchitectural findings. We described two different transvenous routes emphasizing on anatomical consideration and its technical aspect.

  14. Endovascular management of dural fistulas into the cavernous sinus. A systematic review

    Directory of Open Access Journals (Sweden)

    Moscote-Salazar Luis Rafael

    2014-06-01

    Full Text Available Background: Dural fistula to the cavernous sinus (DFCS is an infrequent pathology that consists in the anomalous communication between the meningeal branches of the internal carotid artery (ICA and/or the external carotid artery (ECA and the cavernous sinus. Aim: To perform a systematic review to evaluate clinical and imaging findings in DFCS, and current indications for treatment.

  15. Metachronous occurrence of nonradiation-induced brain cavernous hemangioma and medulloblastoma in a child with neurofibromatosis type I phenotype

    Directory of Open Access Journals (Sweden)

    Luciano L Furlanetti

    2012-01-01

    Full Text Available Cavernous hemangioma (CH is a sporadic vascular malformation occurring either as an autosomal dominant condition or as a well-known complication of radiation exposure. Medulloblastoma is a primitive neuroectodermal tumor common in children and currently treated with surgical resection, chemotherapy, and radiotherapy. Neurofibromatosis is the most common single-gene disorder of the central nervous system. Posterior fossa malignant tumors in the context of neurofibromatosis type I (NF1 are very infrequent. This is the first documented case of an unusual metachronous occurrence of non-radiation-induced CH and medulloblastoma in a child with NF1 phenotype. We report the case of a 13-month-old boy with cafι-au-lait skin lesions associated with NF1-like phenotype who underwent surgical resection of a single CH in the temporal lobe due to recurrent seizures. Four years later he presented with signs of raised intracranial pressure associated with a posterior fossa tumor and hydrocephalus, thus requiring gross total resection of the lesion. Histological analysis revealed a medulloblastoma. After being treated with radiotherapy and chemotherapy, he achieved total remission. Six years later a massive recurrence of the tumor was observed and the child eventually died. The interest in this case lies in the rarity of NF1-like phenotype associated with a non-radiation-induced brain CH and medulloblastoma in a child.

  16. Vasoespasmo cerebral

    OpenAIRE

    1987-01-01

    Vasoespasmo cerebral ocorre em patologias como enxaqueca, hemorragia subaracnóidea, trauma de crânio, após isquemia e/ou hipoxia. A fisiopatologia do vasoespasmo cerebral nestas patologias não está completamente desvendada. Neste artigo são analisados os fatores neuroquímicos e morfológicos responsáveis pelo controle circulatório cerebral. As alterações circulatórias que seguem a hemorragia subaracnóidea são utilizadas como exemplo. Conclui-se que fatores bioquímicos, fisiológicos e morfológi...

  17. Spontaneous subarachnoid hemorrhage due to ruptured cavernous internal carotid artery aneurysm after medical prolactinoma treatment.

    Science.gov (United States)

    Khalsa, Siri Sahib; Hollon, Todd C; Shastri, Ravi; Trobe, Jonathan D; Gemmete, Joseph J; Pandey, Aditya S

    2016-06-08

    Aneurysms of the cavernous segment of the internal carotid artery (ICA) are believed to have a low risk of subarachnoid haemorrhage (SAH), given the confines of the dural rings and the anterior clinoid process. The risk may be greater when the bony and dural protection has been eroded. We report a case of spontaneous SAH from rupture of a cavernous ICA aneurysm in a patient whose large prolactinoma had markedly decreased in size as the result of cabergoline treatment. After passing a balloon test occlusion, the patient underwent successful endovascular vessel deconstruction. This case suggests that an eroding skull base lesion may distort normal anterior cranial base anatomy and allow communication between the cavernous ICA and subarachnoid space. The potential for SAH due to cavernous ICA aneurysm rupture should be recognised in patients with previous pituitary or other skull base lesions adjacent to the cavernous sinus.

  18. Fibrosis of corpus cavernosum in animals following cavernous nerve ablation

    Institute of Scientific and Technical Information of China (English)

    Wan-LiHu; Li-QuanHu; jianSong; Shi-WenLi; Xin-MinZheng; BeiCheng; Bing-ChunTian

    2004-01-01

    t Aim: To investigate alterations of smooth muscle cells and collagen fibers in corpus cavernosum following cavernous neurectomy and its relation to the expression of transforming growth factor-β1 (TGF-β1). Methods: Ten adult male SD rats (neurectomy group) were subject to a bilateral cavernous nerve (CN) resection aseptically under an operating microscope, with 6 sham-operated rats as the control. Fifteen weeks after the operation, the penile specimens were collected and prepared for quantitative-analyzing of ratio of smooth muscle to collagen fibers in corpus cavernosum with confocal microscopy, and for detecting the expression of TGF-β1 by RT-PCR and westernblot.Results: Smooth muscle cells that show red color after fluorescent-labeling with tetramethylrhodamine isothiocyanatephalloidin and collagen fibers that produce green autofluorescence after paraformaldehyde fixation were clearly identified under the confocal microscope. Quantification of fluorescent intensity showed that the ratio of smooth muscle to collagen fibers in corpus cavernosum in neurectomy group was 0.265± 0.125, which was significantly lower than that in sham-operated group (0.760±0.196, P<0.01). RT-PCR and western-blot analyses revealed a significantlyhigher expression of TGF-β1 in the penile tissues of the neurectomy animals than that in sham-operated group.Conclusion: Bilateral ablation of CN can lead to fibrosis of corpus cavernosum, which may be related to an increased expression of TGF-β1 induced by hypoxia in cavernous tissue after denervation.

  19. [Successful treatment using detachable coils for traumatic carotid cavernous fistula as a complication of transsphenoidal surgery for a pituitary adenoma: a case report].

    Science.gov (United States)

    Kobayashi, N; Abe, T; Furuya, H; Dohi, K; Shimazu, M; Sasaki, K; Izumiyama, H; Matsumoto, K; Ohki, S; Nemoto, S

    2000-02-01

    We report a case of a patient with traumatic carotid cavernous fistula (CCF) caused by transnasal-transsphenoidal surgery, who was successfully treated using detachable coils. A 47-year-old man was admitted to our hospital because of severe headache. He was confirmed to have a nonfunctioning pituitary adenoma with presellar-type sphenoid sinus. Cerebral angiography initially disclosed no vascular lesions. A transnasal-transsphenoidal adenomectomy was performed. When the anterior wall of the sphenoid sinus was dissected with a chisel, the chisel deeply stuck into the posterolateral part of the sinus. Profuse arterial bleeding was observed through the sphenoid sinus. The bleeding was stopped easily by compression and packing with bone wax. The operation was continued, the sellar floor was opened widely and the tumor was removed subtotally. The medial wall of the cavernous sinus was intact. Histological examination revealed a pituitary adenoma. Immediately after surgery, the patient noticed a bruit. He developed chemosis and abducent palsy on the right side. Cerebral angiography displayed a high-flow CCF, which was attributed to the carotid artery injury caused by the transnasal-transsphenoidal surgery. The CCF disappeared after two-staged embolization using detachable coils, 1st transvenous and 2nd transarterial. Ten months later, cerebral angiography showed persistent occlusion of the fistula, and the patient experienced no tumor recurrence. It is suggested that drilling is a safer procedure than using a chisel for dissection of a sphenoid sinus with incomplete pneumatization. Endovascular treatment using detachable coils proved useful to manage the CCF, an unusual complication of transsphenoidal surgery.

  20. Site investigations on cavernous limestone for the Remouchamps Viaduct, Belgium

    OpenAIRE

    Waltham, Antony; Vandenven, Georges; Ek, Camille

    1986-01-01

    POOR GROUND CONDITIONS on cavernous limestone created severe difficulties at the sites of four piers of the Remouchamps Viaduct. The discovery, during excavations for foundations, of large open cavities prompted a major re-appraisal of site investigation procedure, and also some redesign of the viaduct structure. La situation défavorable des fondations de quatre des piliers du viaduc autoroutier de Remouchamps posa des problèmes de construction. La présence de cavités karstiques largement ...

  1. Studies on activation in the ATLAS cavern with MPX detectors

    Energy Technology Data Exchange (ETDEWEB)

    Bergmann, Benedikt; Sopczak, Andre; Biskup, Bartolomej; Jakubek, Jan; Pospisil, Stanislav; Solc, Jaroslav; Sopko, Vit; Suk, Michal; Turecek, Daniel; Vykydal, Zdenek; Benes, Petr [IEAP CTU Prague (Czech Republic); Asbah, Nedaa; Leroy, Claude; Soueid, Paul [Universite de Montreal (Canada)

    2013-07-01

    The ATLAS-MPX detectors are based on Medipix2 silicon devices designed by CERN for the detection of different types of radiation. These detectors are successfully operating in the ATLAS detector at 16 positions and collect data independent of the ATLAS data-recording chain. Their data was used to study the activation of the surrounding material and the ATLAS-MPX detectors itself during and after collisions. As the detectors also offer the possibility to distinguish between different types of radiation, an attempt was made to estimate the corresponding dose rates at different locations in the ATLAS detector and in the cavern. First results are presented.

  2. Cavernous Hemangioma of the Skull and Meningioma: Association or Coincidence?

    Directory of Open Access Journals (Sweden)

    M. Kilani

    2015-01-01

    Full Text Available Intraosseous cavernous hemangiomas of the skull are rare. Meningiomas are quite frequently encountered in a neurosurgical practice. The association between these two entities is nevertheless very uncommon. The authors present a case of a 72-year-old woman suffering from headache. The MRI showed a parietal meningioma with adjacent thick bone. The meningioma and the bone were removed. The histological examination confirmed the diagnosis of meningioma and revealed a cavernoma of the skull. The relationship between the lesions suggests more than a coincidental association. Several hypotheses are proposed to explain common causal connections.

  3. Acute fulminant invasive fungal sinusitis with cavernous sinus syndrome.

    Science.gov (United States)

    Chi, Tzu-Hang; Chen, Hsien-Shen; Yuan, Chien-Han; Su, Feng-Ming

    2014-11-01

    Acute fulminant invasive fungal sinusitis is most commonly found in immunocompromised patients with conditions such as diabetes mellitus, malignancies and acquired immune deficiency syndrome. The most common pathogens are Aspergillus and Mucoraceae and the sinus most frequently involved is the maxillary sinus. Fever, rhinorrhea, facial pain, headache, and diplopia are common presenting symptoms. Complications of this infection include intracranial and / or intraorbital spread of the infection; the prognosis is poor. Here, a rare case of acute fulminant invasive fungal sinusitis with cavernous sinus syndrome is reported.

  4. Cavernous Angioma of the Corpus Callosum Presenting with Acute Psychosis

    Directory of Open Access Journals (Sweden)

    Giacomo Pavesi

    2014-01-01

    Full Text Available Psychiatric symptoms may occasionally be related to anatomic alterations of brain structures. Particularly, corpus callosum lesions seem to play a role in the change of patients’ behavior. We present a case of a sudden psychotic attack presumably due to a hemorrhagic cavernous angioma of the corpus callosum, which was surgically removed with complete resolution of symptoms. Although a developmental defect like agenesis or lipoma is present in the majority of these cases, a growing lesion of the corpus callosum can rarely be the primary cause. Since it is potentially possible to cure these patients, clinicians should be aware of this association.

  5. [Pontine cavernous angioma (cavernoma) with initial ENT manifestations].

    Science.gov (United States)

    Pino Rivero, V; González Palomino, A; Pantoja Hernández, C G; Trinidad Ruíz, G; Marqués Rebollo, L; Blasco Huelva, A

    2006-01-01

    We report the case of a 22 years old female who consulted us for facial parestesias, hearing loss in right ear and sudden tinnitus. Her audiometry showed an unilateral discreet sensorineural hipoacusia and the cranial IRM, a mass of 20 mm diameter in right pontine region and bulbus informed as cavernous angioma with signs of recent bleeding. The patient was sent to Neurosurgery but she refused the intervention. The risk of hemorrhage in the cavernomas is estimated at 0.25% to 1.6% per year and represents the main reason to advise a surgical treatment.

  6. A case report of pulsatile tinnitus as a symptom of brain arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    M. Sayadnasiri

    2015-12-01

    Full Text Available Pulsatile tinnitus is the result of blood flow related sounds transmitted to the inner ear and coincides with heartbeat. Although pulsatile tinnitus is a rare entity, this symptom is most often indicative of a serious underlying disease in central nervous system. Unfortunately, this symptom is often not properly assessed by clinician that leads to delayed diagnosis of underlying brain pathology. In this report, a patient is presented with chief complaint of tinnitus that had many medical visits for 2 years. Finally, a cerebral vascular malformation was diagnosed with regards to physical examination and neuroimaging findings.

  7. Malformation of the fetal brain in thanatophoric dysplasia: US and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Fink, A.M. [The Royal Children' s Hospital, Department of Medical Imaging, Melbourne, Victoria (Australia); The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); The University of Melbourne, Department of Radiology, Melbourne, Victoria (Australia); Hingston, Tania; Sampson, Amanda [The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); Ng, Jessica [The Royal Children' s Hospital, Department of Anatomical Pathology, Melbourne, Victoria (Australia); The Royal Women' s Hospital, Department of Anatomical Pathology, Melbourne, Victoria (Australia); Palma-Dias, Ricardo [The Royal Women' s Hospital, Fetal Management Unit, Melbourne, Victoria (Australia); The University of Melbourne, Department of Obstetrics and Gynaecology, Melbourne (Australia)

    2010-12-15

    We present a case in which the unusual cerebral malformations of thanatophoric dysplasia (TD) were identified on a 21-week fetal US and confirmed by antenatal MRI, postmortem imaging and autopsy. TD is the most common lethal skeletal dysplasia and is characterized by short long bones, which are often bowed (type 1), a small thorax, and skull deformities. There is also a recognised constellation of abnormalities of the brain primarily affecting the temporal lobes that, although well described in the postmortem setting, are not widely recognized in fetal imaging. Familiarity with this appearance will facilitate accurate antenatal diagnosis. (orig.)

  8. Recurrence of a paediatric arteriovenous malformation 9 years postcomplete excision: case report and review of literature.

    LENUS (Irish Health Repository)

    McCarthy, Claire

    2012-01-01

    Cerebral arteriovenous malformations (AVMs) are a common congenital vascular anomaly, which often present in both children and adults. Surgery is considered curative once postoperative angiography confirms the absence of vessels. We describe a 6-year-old girl, who had a Spetzler-Martin Grade II AVM resected successfully, in which a recurrent AVM was detected on routine follow-up over 9 years post excision. The aetiopathogenesis of this rare occurrence with a review of literature is discussed. Long-term postoperative follow-up in the form of MRI\\/MR angiogram is recommended for all fully resected AVMs in the paediatric age group, anticipating the possibility of future recurrence.

  9. Ethanol sclerotherapy of peripheral venous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Rimon, U. E-mail: rimonu@sheba.health.gov.il; Garniek, A.; Galili, Y.; Golan, G.; Bensaid, P.; Morag, B

    2004-12-01

    Background: venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. Objectives: to report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. Patients and methods: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. Results: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. Conclusion: sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur.

  10. Microcephaly; Correlation between cerebral CT index and developmental quotient

    Energy Technology Data Exchange (ETDEWEB)

    Azuma, Ototaka; Onozaki, Michihiko; Hidano, Fumio; Mizuguchi, Susumu; Kodama, Kimio (Akita Medical Center for Disabled Children, Kawajiri (Japan)); Komatsu, Eiko; Sakemi, Kikuo; Yamashita, Jun; Sawaishi, Ukio

    1991-09-01

    Thirty one children with microcephaly were referred to Akita Medical Center for Disabled Chilren. Of these children, 28 underwent cerebral computed tomography (CT). Cerebral CT indices were examined in relation to developmental quotient and underlying diseases. Mental retardation, cerebral palsy, epilepsy, and congenital malformations were associated with microcephaly. The most common abnormal CT finding was the ventricular-brain ratio (92.9%, 26/28). CT indices, including Evans' index, the caudal nuclei ratio, transverse width of the third ventricle, the ventricular/intracranial area ratio, the brain/intracranial area ratio, the basal cistern ratio, width of the cerebral longitudinal fissure, and integrated brain CT index, were all significantly correlated with developmental quotient. (N.K.).

  11. CEREBRAL VENOUS THROMBOSIS AND TURNER SYNDROME: A RARE REPORTED ASSOCIATION.

    Science.gov (United States)

    Guler, A; Alpaydin, S; Bademkiran, F; Sirin, H; Celebisoy, N

    2015-01-01

    Turner Syndrome is the only known viable chromosomal monosomy, characterised by the complete or partial absence of an X chromosome. It's the most common chromosomal abnormality in females. Apart from the well known dysmorphic features of the syndrome, it has been associated with a number of vascular pathologies; mainly involving the cardiovascular, renovascular, peripheral vascular and cerebrovascular system. It seems striking that thromboembolism is not considered as a feature of the syndrome. Most of the thromboembolism cases are related to the arterial vascular system; except for some rare reported portal venous thrombosis cases, peripheral venous thrombosis cases and to the best of our knowledge a single case of cerebral venous thrombosis with Dandy Walker malformation and polymicrogyria. We herein report a cerebral venous thrombosis case with Turner Syndrome. With no other found underlying etiology, we want to highlight that Turner Syndrome, itself, may have a relationship not only with the cerebral arterial vascular system pathologies but also with the cerebral venous thrombosis.

  12. Nitrogen Monitoring of West Hackberry 117 Cavern Wells.

    Energy Technology Data Exchange (ETDEWEB)

    Bettin, Giorgia; Lord, David

    2015-02-01

    U.S. Strategic Petroleum Reserve (SPR) oil storage cavern West Hackberry 117 was tested under extended nitrogen monitoring following a successful mechanical integrity test in order to validate a newly developed hydrostatic column model to be used to differentiate between normal "tight" well behavior and small-leak behavior under nitrogen. High resolution wireline pressure and temperature data were collected during the test period and used in conjunction with the hydrostatic column model to predict the nitrogen/oil interface and the pressure along the entire fluid column from the bradenhead flange nominally at ground surface to bottom of brine pool. Results here and for other SPR caverns have shown that wells under long term nitrogen monitoring do not necessarily pressurize with a relative rate (P N2 /P brine) of 1. The theoretical relative pressure rate depends on the well configuration, pressure and the location of the nitrogen-oil interface and varies from well to well. For the case of WH117 the predicted rates were 0.73 for well A and 0.92 for well B. The measured relative pressurization rate for well B was consistent with the model prediction, while well A rate was found to be between 0.58-0.68. A number of possible reasons for the discrepancy between the model and measured rates of well A are possible. These include modeling inaccuracy, measurement inaccuracy or the possibility of the presence of a very small leak (below the latest calculated minimum detectable leak rate).

  13. Nitrogen Monitoring of West Hackberry 117 Cavern Wells

    Energy Technology Data Exchange (ETDEWEB)

    Bettin, Giorgia [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Lord, David L. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2015-02-01

    U.S. Strategic Petroleum Reserve (SPR) oil storage cavern West Hackberry 117 was tested under extended nitrogen monitoring following a successful mechanical integrity test in order to validate a newly developed hydrostatic column model to be used to differentiate between normal "tight" well behavior and small-leak behavior under nitrogen. High resolution wireline pressure and temperature data were collected during the test period and used in conjunction with the hydrostatic column model to predict the nitrogen/oil interface and the pressure along the entire fluid column from the bradenhead flange nominally at ground surface to bottom of brine pool. Results here and for other SPR caverns have shown that wells under long term nitrogen monitoring do not necessarily pressurize with a relative rate (P N2 /P brine) of 1. The theoretical relative pressure rate depends on the well configuration, pressure and the location of the nitrogen-oil interface and varies from well to well. For the case of WH117 the predicted rates were 0.73 for well A and 0.92 for well B. The measured relative pressurization rate for well B was consistent with the model prediction, while well A rate was found to be between 0.58-0.68. A number of possible reasons for the discrepancy between the model and measured rates of well A are possible. These include modeling inaccuracy, measurement inaccuracy or the possibility of the presence of a very small leak (below the latest calculated minimum detectable leak rate).

  14. Ultrasonography guided percutaneous radiofrequency ablation for hepatic cavernous hemangioma

    Institute of Scientific and Technical Information of China (English)

    Yan Cui; Hong-Wen Zhang; Li-Yan Zhou; Man-Ku Dong; Ping Wang; Min Ji; Xiao-Ou Li; Chang-Wei Chen; Zi-Pei Liu; Yong-Jie Xu

    2003-01-01

    AIM: Hepatic cavernous hemangioma (HCH) is the mostcommon benign tumor of the liver and its management isstill controversial. Recent successin situ radiofrequencyablation of hepatic malignancies has led us to consider usingthis technique in patients with HCH. This study was to assessthe efficacy, safety, and complications of percutaneousradiofrequency ablation (PRFA) under ultrasonographyguidance in patients with HCH.METHODS: Twelve patients (four men and eight women,age ranged 33-56 years, mean age was 41.7 years) with 15hepatic cavernous hemangiomas (2.5 cm to 9.5 cm) weretreated using the RF-2000 generator and 10-needle LeVeenelectrode percutaneously guided by B-ultrasound. Lesionslarger than 3 cm were treated by multiple overlappingablations that encompass the entire lesion as well as a rimof normal liver tissue (approximately 0.5 cm).RESULTS: All the patients who received PRFA therapy hadno severe pain, bleeding or bile leakage during and afterthe procedures. Nine to 34 months′ follow-up (mean, 21months) by ultrasound and/or spiral CT scan demonstratedthat the ablated lesions in this group were shrunk remarkably,and the shrunken range was 38-79 % (mean, 67 % per 21months). The contrast enhancement was disappeared withinthe tumor or at its periphery in all cases on spiral CT scansobtained 3 to 6 months after treatment.CONCLUSION: The results of this study suggest that PRFAtherapy is a mini-invasive, simple, safe, and effective methodfor the treatment of selected patients with HCH.

  15. Cavernous sinus hemangioma: a fourteen year single institution experience.

    Science.gov (United States)

    Bansal, Sumit; Suri, Ashish; Singh, Manmohan; Kale, Shashank Sharad; Agarwal, Deepak; Sharma, Manish Singh; Mahapatra, Ashok Kumar; Sharma, Bhawani Shankar

    2014-06-01

    Cavernous sinus hemangioma (CSH) is a rare extra-axial vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors. Their location, propensity for profuse bleeding during surgery, and relationship to complex neurovascular structures are factors which present difficulty in excising these lesions. The authors describe their experience of 22 patients with CSH over 14 years at a tertiary care center. Patients were managed with microsurgical resection using a purely extradural transcavernous approach (13 patients) and with Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) (nine patients). Retrospective data analysis found headache and visual impairment were the most common presenting complaints, followed by facial hypesthesia and diplopia. All but one patient had complete tumor excision in the surgical series. Transient ophthalmoparesis (complete resolution in 6-8 weeks) was the most common surgical complication. In the GKRS group, marked tumor shrinkage (>50% tumor volume reduction) was achieved in two patients, slight shrinkage in five and no change in two patients, with symptom improvement in the majority of patients. To our knowledge, we describe one of the largest series of CSH managed at a single center. Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality and long-term morbidity, GKRS is an additional tool for treating residual symptomatic lesions or in patients with associated comorbidities making surgical resection unsuitable.

  16. Chiari type I malformation in children.

    Science.gov (United States)

    Massimi, L; Novegno, F; di Rocco, C

    2011-01-01

    The diagnosis of Chiari type I malformation (CIM) is more and more frequent in clinical practice due to the wide diffusion of magnetic resonance imaging. In many cases, such a diagnosis is made incidentally in asymptomatic patients, as including children investigated for different reasons such as mental development delay or sequelae of brain injury. The large number of affected patients, the presence of asymptomatic subjects, the uncertainties surrounding the pathogenesis of the malformation, and the different options for its surgical treatment make the management of CIM particularly controversial.This paper reports on the state of the art and the recent achievements about CIM aiming at providing further information especially on the pathogenesis, the natural history, and the management of the malformation, which are the most controversial aspects. A historial review introduces and explains the current classification. Furthermore, the main clinical, radiological, and neurophysiological findings of CIM are described to complete the picture of this heterogeneous and complex disease.

  17. Imaging of head and neck venous malformations

    Energy Technology Data Exchange (ETDEWEB)

    Flis, Christine M.; Connor, Stephen E. [King' s College Hospital, Neuroradiology Department, London (United Kingdom)

    2005-10-01

    Venous malformations (VMs) are non proliferative lesions that consist of dysplastic venous channels. The aim of imaging is to characterise the lesion and define its anatomic extent. We will describe the plain film, ultrasound (US) (including colour and duplex Doppler), computed tomography (CT), magnetic resonance imaging (MRI), conventional angiographic and direct phlebographic appearances of venous malformations. They will be illustrated at a number of head and neck locations, including orbit, oral cavity, superficial and deep facial space, supraglottic and intramuscular. An understanding of the classification of such vascular anomalies is required to define the correct therapeutic procedure to employ. Image-guided sclerotherapy alone or in combination with surgery is now the first line treatment option in many cases of head and neck venous malformations, so the radiologist is now an integral part of the multidisciplinary management team. (orig.)

  18. A NOVEL PROCESS TO USE SALT CAVERNS TO RECEIVE SHIP BORNE LNG

    Energy Technology Data Exchange (ETDEWEB)

    Michael M. McCall; William M. Bishop; Marcus Krekel; James F. Davis; D. Braxton Scherz

    2005-05-31

    This cooperative research project validates use of man made salt caverns to receive and store the cargoes of LNG ships in lieu of large liquid LNG tanks. Salt caverns will not tolerate direct injection of LNG because it is a cryogenic liquid, too cold for contact with salt. This research confirmed the technical processes and the economic benefits of pressuring the LNG up to dense phase, warming it to salt compatible temperatures and then directly injecting the dense phase gas into salt caverns for storage. The use of salt caverns to store natural gas sourced from LNG imports, particularly when located offshore, provides a highly secure, large scale and lower cost import facility as an alternative to tank based LNG import terminals. This design can unload a ship in the same time as unloading at a tank based terminal. The Strategic Petroleum Reserve uses man made salt caverns to securely store large quantities of crude oil. Similarly, this project describes a novel application of salt cavern gas storage technologies used for the first time in conjunction with LNG receiving. The energy industry uses man made salt caverns to store an array of gases and liquids but has never used man made salt caverns directly in the importation of LNG. This project has adapted and expanded the field of salt cavern storage technology and combined it with novel equipment and processes to accommodate LNG importation. The salt cavern based LNG receiving terminal described in the project can be located onshore or offshore, but the focus of the design and cost estimates has been on an offshore location, away from congested channels and ports. The salt cavern based terminal can provide large volumes of gas storage, high deliverability from storage, and is simplified in operation compared to tank based LNG terminals. Phase I of this project included mathematical modeling that proved a salt cavern based receiving terminal could be built at lower capital cost, and would have significantly higher

  19. Literature Survey Concerning the Feasibility of Remedial Leach for Select Phase I Caverns

    Energy Technology Data Exchange (ETDEWEB)

    Weber, Paula D. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Flores, Karen A. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Lord, David L. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2015-09-01

    Bryan Mound 5 ( BM5 ) and West Hackberry 9 ( WH9 ) have the potential to create a significant amount of new storage space should the caverns be deemed "leach - ready". This study discusses the original drilling history of the caverns, surrounding geology, current stability, and, based on this culmination of data, makes a preliminary assessment of the leach potential for the cavern. The risks associated with leaching BM5 present substantial problems for the SPR . The odd shape and large amount of insoluble material make it difficult to de termine whether a targeted leach would have the desired effect and create useable ullage or further distort the shape with preferential leaching . T he likelihood of salt falls and damaged or severed casing string is significant . In addition, a targeted le ach would require the relocation of approximately 27 MMB of oil . Due to the abundance of unknown factors associated with this cavern, a targeted leach of BM5 is not recommended. A targeted leaching of the neck of WH 9 could potentially eliminate or diminis h the mid - cavern ledge result ing in a more stable cavern with a more favorable shape. A better understanding of the composition of the surrounding salt and a less complicated leaching history yields more confidence in the ability to successfully leach this region. A targeted leach of WH9 can be recommended upon the completion of a full leach plan with consideration of the impacts upon nearby caverns .

  20. Cochlear Implantation in Children with Cochlear Malformation.

    Science.gov (United States)

    Saikawa, Etsuko; Takano, Kenichi; Ogasawara, Noriko; Tsubomatsu, Chieko; Takahashi, Nozomi; Shirasaki, Hideaki; Himi, Tetsuo

    2016-01-01

    Cochlear implantation (CI) has proven to be an effective treatment for severe bilateral sensorineural hearing loss (SNHL). Inner ear malformation is a rare anomaly and occurs in approximately 20% of cases with congenital SNHL. In cases with cochlear malformation, CI can be successfully performed in nearly all patients, the exceptions being those with complete labyrinthine and cochlear aplasia. It is important to evaluate the severity of inner ear deformity and other associated anomalies during the preimplantation radiological assessment in order to identify any complication that may potentially occur during the surgery and subsequent patient management.

  1. Papilloedema due to Chiari I malformation.

    Science.gov (United States)

    Zhang, Jason Chao; Bakir, Belal; Lee, Andrew; Yalamanchili, Sushma S

    2011-10-16

    The Chiari I malformation is a congenital abnormality characterised by downward displacement of the cerebellar tonsils through the foramen magnum into the cervical spine. It presents clinically most often in young adult women. Known ocular manifestations linked to Chiari I consist primarily of oculomotor paresis with cranial nerve VI palsy and convergence/divergence abnormalities. Papilloedema is a rare manifestation of Chiari I with a clinical presentation often similar to that of idiopathic intracranial hypertension. To highlight this unusual complication, the authors report a 64-year-old female who developed papilloedema as the only presenting neurological symptom resulting from a Chiari I malformation.

  2. Percutaneous Cryotherapy of Vascular Malformation: Initial Experience

    Energy Technology Data Exchange (ETDEWEB)

    Cornelis, F., E-mail: francoiscornelis@hotmail.com [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France)

    2013-06-15

    The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations.

  3. Diagnosis, treatment, and prevention of cerebral palsy.

    Science.gov (United States)

    O'Shea, Thomas Michael

    2008-12-01

    Cerebral palsy is the most prevalent cause of persisting motor function impairment with a frequency of about 1/500 births. In developed countries, the prevalence rose after introduction of neonatal intensive care, but in the past decade, this trend has reversed. A recent international workshop defined cerebral palsy as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain." In a majority of cases, the predominant motor abnormality is spasticity; other forms of cerebral palsy include dyskinetic (dystonia or choreo-athetosis) and ataxic cerebral palsy. In preterm infants, about one-half of the cases have neuroimaging abnormalities, such as echolucency in the periventricular white matter or ventricular enlargement on cranial ultrasound. Among children born at or near term, about two-thirds have neuroimaging abnormalities, including focal infarction, brain malformations, and periventricular leukomalacia. In addition to the motor impairment, individuals with cerebral palsy may have sensory impairments, cognitive impairment, and epilepsy. Ambulation status, intelligence quotient, quality of speech, and hand function together are predictive of employment status. Mortality risk increases incrementally with increasing number of impairments, including intellectual, limb function, hearing, and vision. The care of individuals with cerebral palsy should include the provision of a primary care medical home for care coordination and support; diagnostic evaluations to identify brain abnormalities, severity of neurologic and functional abnormalities, and associated impairments; management of spasticity; and care for associated problems such as nutritional deficiencies, pain, dental care, bowel and bladder continence, and orthopedic complications. Current strategies to decrease the risk of cerebral palsy include interventions to

  4. Effect of Pingyangmycin on human venous malformation endothelial cells

    Institute of Scientific and Technical Information of China (English)

    Yi Fang Zhao; Zhi Jun Sun; Yu Lin Jia; Jun Jia; Ya Meng Si; Ji Hong Zhao; Wen Feng Zhang

    2008-01-01

    @@ Purpose: Venous malformations are common vascular anomalies with a propensity of the head and neck. Intralesional injection of Pingyangmycin (PYM, bleomycin A5 hydrochloride) is a widely used sclerotherapy method for the treatment of venous malformation.

  5. Microsurgical thromboendarterectomy of the cavernous carotid artery--case report and surgical technique.

    Science.gov (United States)

    Fujitsu, K; Fujii, S; Tanaka, N; Kuwabara, T

    1990-10-01

    A 53-year-old male suffered a transient right hemiparesis and left monocular blindness. Angiography revealed 80% stenosis of the cavernous carotid artery. Microsurgical thromboendarterectomy was performed by a direct approach through Parkinson's triangle. During surgery, the carotid circulation was transiently trapped between the cervical and the supraclinoid segment and the trapped arterial lumen was irrigated with heparinized saline. Soft elastic lesion was easily removed. Cavernous carotid thromboendarterectomy through a direct approach is considered as a suitable operation for the solitary and localized stenotic lesions of the cavernous carotid artery, although this operation has not yet been reported to date.

  6. A STUDY OF POSTERIOR FOSSA MALFORMATIONS: MR IMAGING

    Directory of Open Access Journals (Sweden)

    Ravi

    2015-02-01

    Full Text Available AIMS AND OBJECTIVES: The aim of our study is to describe the imaging findings of various posterior fossa malformations and to evaluate the supratentorial abnormalities associated with posterior fossa malformations. MATERIALS AND METHODS: MR images of 30 patients wi th posterior fossa malformations detected in the department of Radiodiagnosis, BMCRI over a period of two years, from December 2012 to December 2014 were evaluated retrospectively. The various posterior fossa malformations were evaluated. Associated suprat entorial abnormalities were noted. RESULTS: 30 patients with posterior fossa malformations were included in the study. The age group of patients ranged from 1year to 53years. There were 18 males and 12 females. The various posterior fossa malformations det ected were Dandy Walker malformation (1 case, Dandy Walker variant (2 cases, mega cisterna magna (8 cases, arachnoid cysts (5 cases, Chiari 1 malformation (5 cases, Chairi 2 malformation (2 cases, Joubert malformation (1 case, lipoma (2 cases, verm ian and/or cerebellar hypoplasia without posterior fossa CSF collection or cyst (4 cases. Associated supratentorial abnormalities were seen in 8 cases . CONCLUSION: MRI is the imaging modality of choice in the evaluation of posterior fossa malformations. I t is very important to know the imaging findings of these malformations and to have knowledge about the various supratentorial and spinal abnormalities associated with them so as to provide an accurate diagnosis which is very essential for predicting the p rognosis and planning further management.

  7. RTTN mutations link primary cilia function to organization of the human cerebral cortex

    NARCIS (Netherlands)

    S.K. Kia; E. Verbeek (Elly); M.P. Engelen (Erik); R. Schot (Rachel); R.A. Poot (Raymond); I.F.M. de Coo (René); M. Leguin (Maarten); C.J. Poulton (Cathryn); F. Pourfarzad, F. (Farzin); F.G. Grosveld (Frank); A. Brehm (António); M.C.Y. de Wit (Marie Claire); R. Oegema (Renske); W.B. Dobyns (William); F.W. Verheijen (Frans); G.M.S. Mancini (Grazia)

    2012-01-01

    textabstractPolymicrogyria is a malformation of the developing cerebral cortex caused by abnormal organization and characterized by many small gyri and fusion of the outer molecular layer. We have identified autosomal-recessive mutations in RTTN, encoding Rotatin, in individuals with bilateral diffu

  8. The role of preoperative angiography in the management of giant meningiomas associated to vascular malformation

    Directory of Open Access Journals (Sweden)

    Fabio Papacci

    2015-01-01

    Full Text Available Background: The role of preoperative digital subtraction angiography (DSA in meningiomas is currently under discussion because of the introduction of noninvasive magnetic resonance imaging (MRI angiography to study vascular anatomy associated to the tumor. Preoperative DSA is mainly performed to obtain embolization of the lesion, although a number of complications have been reported after this procedure. Nonetheless, the coexistence of meningiomas with vascular malformations has previously been reported and it has been evidenced that this event could be underestimated because of neglect of preoperative DSA. Here, we report on two challenging cases of giant meningiomas associated to vascular malformations and we discuss the pertinent literature. Case Descriptions: In the first case: A large right temporal meningioma with erosion of the sphenoid greater wing and extension toward infratemporal fossa and right orbit - a large pseudoaneurysm of right middle cerebral artery branch was found end embolized during DSA. In the second case: A giant parieto-temporal meningioma - DSA permitted the full visualization of an abnormal drainage of superior sagittal sinus like a "sinus pericranii" that was respected during the following surgery. Conclusion: We think that MRI angiography is the exam of choice to study vascular anatomy in meningiomas. Nonetheless, DSA remains a useful tool in giant meningiomas not only to embolizate the lesion but also to treat tumor associated vascular malformation and to achieve the full knowledge of vascular anatomy. We think that a wide communication between interventionalist and surgeon is essential for the optimal management of these patients.

  9. Lymphatic malformations: a proposed management algorithm.

    LENUS (Irish Health Repository)

    Oosthuizen, J C

    2012-02-01

    OBJECTIVE: The aim of this study was to develop a management algorithm for cervicofacial lymphatic malformations, based on the authors\\' experience in managing these lesions as well as current literature on the subject. STUDY DESIGN AND METHODS: A retrospective medical record review of all the patients treated for lymphatic malformations at our institution during a 10-year period (1998-2008) was performed. DATA COLLECTED: age at diagnosis, location and type of lesion, radiologic investigation performed, presenting symptoms, treatment modality used, complications and results achieved. RESULTS: 14 patients were identified. Eight (57%) male and six (43%) female. There was an equal distribution between the left and right sides. The majority (71%) of cases were diagnosed within the first year of life. The majority of lesions were located in the suprahyoid region. The predominant reason for referral was an asymptomatic mass in 7 cases (50%) followed by airway compromise (36%) and dysphagia (14%). Management options employed included: observation, OK-432 injection, surgical excision and laser therapy. In 5 cases (36%) a combination of these were used. CONCLUSION: Historically surgical excision has been the management option of choice for lymphatic malformations. However due to the morbidity and high complication rate associated this is increasingly being questioned. Recent advances in sclerotherapy e.g. OK-432 injection have also shown significant promise. Based on experience in managing these lesions as well as current literature the authors of this paper have developed an algorithm for the management of cervicofacial lymphatic malformations.

  10. Congenital Malformations in River Buffalo (Bubalus bubalis

    Directory of Open Access Journals (Sweden)

    Sara Albarella

    2017-02-01

    Full Text Available The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital malformations most frequently reported in literature or signaled by breeders to the Department of Veterinary Medicine and Animal Production of the University Federico II (Naples, Italy in river buffalo are: musculoskeletal defects (transverse hemimelia, arthrogryposis, umbilical hernia and disorders of sexual development. In conclusion this review put in evidence that river buffalo have a great variety of malformations due to genetic causes, and TH and omphalocele are the most frequent and that several cases are still not reported, leading to an underestimation of the real weight of genetic diseases in this species.

  11. Fetal MRI clues to diagnose cloacal malformations

    Energy Technology Data Exchange (ETDEWEB)

    Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Patel, Manish N.; Kraus, Steven [Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States); Levitt, Marc A.; Pena, Alberto [Cincinnati Children' s Hospital Medical Center, Colorectal Center for Children, Pediatric Surgery, Cincinnati, OH (United States); Lim, Foong-Yen; Crombleholme, Timothy M. [Cincinnati Children' s Hospital Medical Center, Fetal Care Center of Cincinnati, Pediatric Surgery, Cincinnati, OH (United States); Linam, Leann E. [Arkansas Children' s Hospital, Department of Radiology, Little Rock, AR (United States)

    2011-09-15

    Prenatal US detection of cloacal malformations is challenging and rarely confirms this diagnosis. To define the prenatal MRI findings in cloacal malformations. We performed a retrospective study of patients with cloacal malformations who had pre- and post-natal assessment at our institution. Fetal MRI was obtained in six singleton pregnancies between 26 and 32 weeks of gestation. Imaging analysis was focused on the distal bowel, the urinary system and the genital tract and compared with postnatal clinical, radiological and surgical diagnoses. The distal bowel was dilated and did not extend below the bladder in five fetuses. They had a long common cloacal channel (3.5-6 cm) and a rectum located over the bladder base. Only one fetus with a posterior cloacal variant had a normal rectum. Three fetuses had increased T2 signal in the bowel and two increased T1/decreased T2 signal bladder content. All had renal anomalies, four had abnormal bladders and two had hydrocolpos. Assessment of the anorectal signal and pelvic anatomy during the third trimester helps to detect cloacal malformations in the fetus. The specificity for this diagnosis was highly increased when bowel fluid or bladder meconium content was identified. (orig.)

  12. Idiopathic hepatic arterial malformation: a case report

    Institute of Scientific and Technical Information of China (English)

    郑蔚巍; 周康荣; 王佩芬; 陈祖望

    2003-01-01

    @@ Hepatic arterial malformation is a rare disorder which either origi nates idiopathically or may be associated with hereditary hemorrhagic telangiect asia (also known as Osler-Weber-Rendu disease). Although previous reports presented only descriptions of sonographic and angiographic findings,1-6 we present a case of splenic infarct caused by this disorder with CT and CTA findi ngs.

  13. New concepts on posterior fossa malformations

    Energy Technology Data Exchange (ETDEWEB)

    Jaspan, Tim [Imaging Centre, University Hospital, Nottingham (United Kingdom)

    2008-06-15

    A full description of the embryology of the posterior fossa (PF) is beyond the scope of this review; several recent publications are recommended. Specific aspects of the processes involved are, however, reviewed as a background to malformations that involve defects or errors occurring at critical stages during the embryogenesis of the PF structures. (orig.)

  14. Ketogenic Diet for Epilepsy and Focal Malformation

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2008-09-01

    Full Text Available The efficacy and long-term treatment outcome of a classic ketogenic diet (KD addon treatment (4:1 lipid/nonlipid ratio, without initial fasting and fluid restriction were evaluated retrospectively in 47 children with intractable epilepsy and focal malformation of cortical development, in a study at Severance Children’s and Sanggye Park Hospitals, Seoul, Korea.

  15. [A woman with a rare vascular malformation

    NARCIS (Netherlands)

    Koning, G.G.; Vries, M. de

    2015-01-01

    A 30-year-old woman with trisomy 8 syndrome and coagulopathy was diagnosed with a malformation of the vena cava superior. This is a rare anatomical variation, which originates from a non-development of Marshall's ligament during the 8th week of gestation (prevalence: 0.3%).

  16. Percutaneous Treatment of Peripheral Vascular Malformations

    NARCIS (Netherlands)

    E. van der Linden (Edwin)

    2011-01-01

    textabstractVascular malformations arise from errors in the morphological processes that shape the embryonic vascular system during fetal development. These developmental errors result in abnormal clusters of blood vessels. Although these lesions are present at birth, they might not become visible u

  17. Congenital spinal malformations; Kongenitale spinale Malformationen

    Energy Technology Data Exchange (ETDEWEB)

    Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

    2001-12-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

  18. Phosphorus magnetic resonance spectroscopy in malformations of cortical development

    Directory of Open Access Journals (Sweden)

    Celi Santos Andrade

    2013-07-01

    Full Text Available Introduction Malformations of cortical development (MCD result from disruptions in the dynamic process of cerebral corticogenesis and are important causes of epilepsy, motor deficits and cognitive impairment. Objectives The aim of this study was to evaluate phospholipids metabolism in vivo in a series of patients with epilepsy and MCD. Methods Thirty-seven patients with MCD and 31 control subjects were studied using three-dimensional phosphorus magnetic resonance spectroscopy (31P-MRS at a 3.0 T scanner. Quantification methods were applied to the following resonances: phosphoethanolamine (PE, phosphocholine (PC, glycerophosphoethanolamine (GPE, glycerophosphocholine (GPC, inorganic phosphate (Pi, phosphocreatine (PCr, and a-, b-, and g-adenosine triphosphate (ATP. The magnesium (Mg2+ levels and pH were calculated based on PCr, Pi and b-ATP chemical shifts. Results Compared to controls, the MCD lesions exhibited lower pH values and higher Mg2+ levels (p<0.05. The lesions also presented significant reduction of GPC and PDE, and an increased PME/PDE ratio. The otherwise normal appearing parenchyma also demonstrated lower pH values in the frontoparietal cortex and bilateral centrum semiovale. Conclusions Our data support the idea that metabolic impairments occur in the lesions of MCD, with propagation to remote normal appearing parenchyma. The results also suggest that there are membrane turnover disturbances in MCD lesions.

  19. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Hedlund, Gary [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States)

    2006-07-15

    The newborn, infant, or young child who presents with a midline frontonasal mass often poses a diagnostic challenge to the clinician. The most pressing issue is whether the mass extends intracranially. The development of the frontonasal region or anterior neuropore is complex. Aberrant embryogenesis leads to three main types of anomalies: nasal dermal sinus, anterior cephalocele, and nasal glioma. Understanding the developmental anatomy of the anterior neuropore and postnatal maturation will serve the radiologist well when it comes to imaging frontonasal masses. Pitfalls particularly common to CT imaging interpretation include the evolving ossification of the frontal, nasal and ethmoid bones in the first year of life, morphology and size of the foramen cecum, and the natural intumescence of the anterior nasal septum. Determination of the presence of a connection between the frontonasal mass and the anterior cranial fossae is crucial in the imaging assessment and clinical management. In the case of the nasal dermal sinus, failure to appreciate the intracranial components of the malformation can lead to fatal meningitis. MR imaging is the modality of choice for assessing the pediatric frontonasal region. Its advantages include multiplanar imaging, distinguishing the interface among cartilage, bone, brain and fluid, diffusion imaging to detect epidermoid tumors, and the capacity to evaluate the brain for associated cerebral anomalies. (orig.)

  20. Congenital Malformations in River Buffalo (Bubalus bubalis)

    Science.gov (United States)

    Albarella, Sara; Ciotola, Francesca; D’Anza, Emanuele; Coletta, Angelo; Zicarelli, Luigi; Peretti, Vincenzo

    2017-01-01

    Simple Summary Congenital malformations (due to genetic causes) represent a hidden danger for animal production, above all when genetic selection is undertaken for production improvements. These malformations are responsible for economic losses either because they reduce the productivity of the farm, or because their spread in the population would decrease the total productivity of that species/breed. River buffalo is a species of increasing interest all over the world for its production abilities, as proved by the buffalo genome project and the genetic selection plans that are currently performed in different countries. The aim of this review is to provide a general view of different models of congenital malformations in buffalo and their world distribution. This would be useful either for those who performed buffalo genetic selection or for researchers in genetic diseases, which would be an advantage to their studies with respect to the knowledge of gene mutations and interactions in this species. Abstract The world buffalo population is about 168 million, and it is still growing, in India, China, Brazil, and Italy. In these countries, buffalo genetic breeding programs have been performed for many decades. The occurrence of congenital malformations has caused a slowing of the genetic progress and economic loss for the breeders, due to the death of animals, or damage to their reproductive ability or failing of milk production. Moreover, they cause animal welfare reduction because they can imply foetal dystocia and because the affected animals have a reduced fitness with little chances of survival. This review depicts, in the river buffalo (Bubalus bubalis) world population, the present status of the congenital malformations, due to genetic causes, to identify their frequency and distribution in order to develop genetic breeding plans able to improve the productive and reproductive performance, and avoid the spreading of detrimental gene variants. Congenital

  1. Epileptic Seizures Induced by a Spontaneous Carotid Cavernous Fistula

    Science.gov (United States)

    Yildirim, Erkan

    2016-01-01

    A 79-year-old woman was admitted to our emergency department with complaints of fainting and loss of consciousness three times during the past month. She was diagnosed with epilepsy and started to be treated with antiepileptic drug. Physical examination showed, in the left eye, chemosis, limited eye movements in all directions, and minimal exophthalmos as unexisting symptoms on admission developed on the sixth day. Orbital magnetic resonance imaging (MRI) and digital subtraction angiography (DSA) imaging revealed a carotid cavernous fistula (CCF). Epileptic attacks and ophthalmic findings previously present but diagnosed during our examinations were determined to ameliorate completely after performing the coil embolization. Based on literature, we present the first case with nontraumatic CCF manifesting with epileptic seizures and intermittent eye symptoms in the present report. PMID:28077946

  2. [Anatomical study of the cavernous nerve in relation to nerve sparing operation].

    Science.gov (United States)

    Hanawa, K

    1994-08-01

    Recently, nerve sparing radical prostatectomy has became widely considered as the primary goal for maintaining a high standard of quality of life (QOL). However, anatomical localization of the cavernous nerve has not yet been precisely clarified in terms of the terminal end in the corpus cavernous penis distal to the urogenital membrane. Here in attempt to demonstrate the precise localization of the cavernous nerve, in six adult male cadaver. The cavernous nerves ran between the prostatic capsule and the prostatic fascia, through the capsule of the seminal vesicle. The nerves penetrated the membranous urethra at 8 mm from the margin of the urethra at the position of 5 and 7 o'clock. Therefore, the following procedures are critical to achieve successful nerve sparing: 1) meticulous division of the seminal-vesicle, 2) precise separation of the neurovascular bundle between the prostatic capsule and fascia, and 3) the careful transaction of the membranous urethra.

  3. Diagnosis and management of trigemino-cavernous fistulas: case reports and review of the literature.

    Science.gov (United States)

    Miller, Timothy R; Jindal, Gaurav; Mohan, Suyash; Fortes, Manuel; Hurst, Robert; Pukenas, Bryan; Gandhi, Dheeraj

    2015-01-01

    Although usually asymptomatic, a persistent trigeminal artery (PTA) can rarely be associated with a direct fistula to the cavernous sinus (ie, trigemino-cavernous fistula). We present three patients with trigemino-cavernous fistulas; two were subsequently treated using modern endovascular techniques while the third initially declined therapy. We then review the literature of reported cases of this unusual entity. The aberrant anatomy associated with a PTA presents unique challenges to the management of these lesions, and must be well delineated prior to treatment. Finally, conservative management of trigemino-cavernous fistulas, either de novo or recurrent, may be considered if they demonstrate no evidence of cortical venous reflux and patient symptoms are tolerable.

  4. Transient behaviour of deep underground salt caverns; Comportement transitoire des cavites salines profondes

    Energy Technology Data Exchange (ETDEWEB)

    Karimi-Jafari, M

    2007-11-15

    This work deals with the transient behaviour of deep underground salt caverns. It has been shown that a cavern is a complex system, in which there are mechanical, thermal, chemical and hydraulic evolutions. The importance of the transient evolutions, particularly the role of the 'reverse' creep in the interpretation of the tightness test in a salt cavern is revealed. Creep is characterized by a formulation of the behaviour law which presents the advantage, in a practical point of view, to only have a reduced number of parameters while accounting of the essential of what it is observed. The initiation of the rupture in the effective traction in a salt cavern rapidly pressurized is discussed. A model fitted to a very long term behaviour (after abandonment) is developed too. In this case too, a lot of phenomena, more or less coupled, occur, when the existing literature took only into account some phenomena. (O.M.)

  5. Transorbital superior ophthalmic vein sacrifice to preserve vision in ocular hypertension from aseptic cavernous sinus thrombosis.

    Science.gov (United States)

    Ladner, Travis R; Davis, Brandon J; He, Lucy; Mawn, Louise A; Mocco, J

    2015-12-01

    Aseptic cavernous sinus thrombosis (CST) is rare and may clinically masquerade as a carotid cavernous fistula. Conventional management includes oral anticoagulation, but cases of ocular hypertension affecting vision may require more aggressive intervention. We report a case of a woman with spontaneous bilaterally occluded cavernous sinuses with elevated intraocular pressure (IOP), which resolved immediately following unilateral superior ophthalmic vein (SOV) sacrifice. She was subsequently placed on oral anticoagulants. By 4 months postoperatively her IOP was normalized and her vision had improved. Repeat angiography demonstrated stable venous filling, with some mild improvement of flow through the cavernous sinus. Coil-mediated sacrifice of the SOV might be an effective means to relieve ocular hypertension and preserve vision in the setting of aseptic CST.

  6. Dural cavernous sinus fistulas. Diagnostic and endovascular therapy

    Energy Technology Data Exchange (ETDEWEB)

    Benndorf, Goetz [Baylor College of Medicine, Houston, TX (United States). Dept. of Radiology; Ben Taub General Hospital, Houston, TX (United States). Interventional Neuroradiology

    2010-07-01

    Dural cavernous sinus fistulas (DCSFs) represent a benign vascular disease, consisting in an arteriovenous shunt at the cavernous sinus. In the absence of spontaneous resolution, the fistula may lead to eye redness, swelling, proptosis, chemosis, ophthalmoplegia and visual loss. Although modern imaging techniques have improved the diagnostic, patients with low-flow DCSFs are still misdiagnosed. These patients can get erroneously treated for infections and inflammation for months or years and are at risk of visual loss. Early and proper diagnosis helps to avoid deleterious clinical course of the disease. This volume provides a complete guide to clinical and radiological diagnosis as well as to therapeutic management of DCSF with emphasis on modern minimal invasive treatment options. It commences with an informative description of relevant anatomy. After sections on the classification, etiology and pathogenesis of DCSF, the clinical symptomatology of the disease is described in detail. The role of modern non-invasive imaging tools is then addressed with the use of computed tomography, magnetic resonance imaging and ultrasound. Intra-arterial digital subtraction angiography (DSA), although invasive, remains the gold standard and is mandatory for clinical decision-making and strategy in endovascular treatment. Hence, a throughout consideration is given to both, 2D-DSA and 3D rotational angiography, including recent technological advancements such as Dual Volume (DV) imaging and angiographic computed tomography (ACT). After a short section on arteriovenous hemodynamics, the therapeutic management of DCSFs is described in detail. In particular, various transvenous techniques, required for successful endovascular occlusion of DCSF, are discussed in depth. This well-illustrated volume will be invaluable to all who may encounter DCSF in their clinical practice. (orig.)

  7. Sonographic Findings of Cavernous Hemangioma in Fatty Liver

    Energy Technology Data Exchange (ETDEWEB)

    Hahm, Jin Kyeung; Kim, Ki Whang; Yoon, Sang Wook; Kim, Tae Hoon; Lee, Jong Tae; Yoo, Hyung Sik; Kim, Myung Jin [Yonsei University College of Medicine, Seoul (Korea, Republic of); Ji, Hoon [Aju Univeristy, College of Medicine, Suwon (Korea, Republic of)

    1995-06-15

    Typical cavernous hemangioma presents no diagnostic difficulty at sonography. However, in cases of atypical hemangioma, further evaluation is needed to differentiate it from malignancy. On the other hand, thcechogenicity of the lesion may be iso echo or hypoecho when it occurs in association with fatty liver. We analyzed the sonographic features of hemangioma in fatty liver. We reviewed the sonograms of 22 lesions from 19 patients. We divided the lesions into two groups; the lesion measuring less than 3cm in diameter (group I) and the lesions measuring same or greater than 3cm (group II). The lesions of each group were analyzed in terms of location, shape, distinction of margin, internal echogenicity, posterior enhancement, lateral shadowing, and peritumoral hypoechoic halo. The lesions were located in subcapsular or perivascular areain 86%. They strowed round or lobulated shape with well defined margin in 82%. Internal echo of the lesions was hypoechoic in 82% and homogeneous in 64%. Posterior enhancement was seen in 77%. The posterior wall of the lesion was distinct in 68%. There was no statistical difference in incidence of each finding described above between the two groups except the internal echogenicity(p<0.05). All of the four hyperechoic lesions measured greater than 3cmin diameter, and three of them showed uneven thickness of echogenic rind. Definitive diagnosis of hemangioma could be obtained in 82%. In remaining 18% of hemangioma, the lesions showed peripheral hypoechoic halo and lateral shadowing that made the diagnosis of hemangioma difficult. However, the possibility of hemangioma could be suggested because they showed haemangiomas internal eye-catching and posterior enhancement. Hepatic cavernous hemangioma presents with variable eye-catching as compared to the surrounding tissue when it is associated with fatty liver disease, Thus, in differentiating hemangiomas from other localized hepatic mass, other characteristics such as homogeneity of the

  8. EXAMINE AND EVALUATE A PROCESS TO USE SALT CAVERNS TO RECEIVE SHIP BORNE LIQUEFIED NATURAL GAS

    Energy Technology Data Exchange (ETDEWEB)

    Michael M. McCall; William M. Bishop; D. Braxton Scherz

    2003-04-24

    The goal of the U.S. Department of Energy cooperative research project is to define, describe, and validate, a process to utilize salt caverns to receive and store the cargoes of LNG ships. The project defines the process as receiving LNG from a ship, pumping the LNG up to cavern injection pressures, warming it to cavern compatible temperatures, injecting the warmed vapor directly into salt caverns for storage, and distribution to the pipeline network. The performance of work under this agreement is based on U.S. Patent 5,511,905, and other U.S. and Foreign pending patent applications. The cost sharing participants in the research are The National Energy Technology Laboratory (U.S. Department of Energy), BP America Production Company, Bluewater Offshore Production Systems (U.S.A.), Inc., and HNG Storage, L.P. Initial results indicate that a salt cavern based receiving terminal could be built at about half the capital cost, less than half the operating costs and would have significantly higher delivery capacity, shorter construction time, and be much more secure than a conventional liquid tank based terminal. There is a significant body of knowledge and practice concerning natural gas storage in salt caverns, and there is a considerable body of knowledge and practice in handling LNG, but there has never been any attempt to develop a process whereby the two technologies can be combined. Salt cavern storage is infinitely more secure than surface storage tanks, far less susceptible to accidents or terrorist acts, and much more acceptable to the community. The project team developed conceptual designs of two salt cavern based LNG terminals, one with caverns located in Calcasieu Parish Louisiana, and the second in Vermilion block 179 about 50 miles offshore Louisiana. These conceptual designs were compared to conventional tank based LNG terminals and demonstrate superior security, economy and capacity. The potential for the development of LNG receiving terminals

  9. Image–guided resection of small lesions in the cavernous sinus and Meckel's cave

    OpenAIRE

    Nakamura, M.; Krauss, J K

    2010-01-01

    Abstract Objective The microsurgical resection of tumors or vascular lesions in the cavernous sinus and the neighbouring Meckel's cave has been considered as hazardous because of often associated cranial nerve morbidity. Despite increasing consent that many of such tumors should not undergo surgical therapy, the cavernous sinus and Meckel's cave may harbour small lesions of various origin, which are amenable for surgical resection. Surgery in this anatomical area needs a...

  10. Color Doppler Imaging in the Diagnosis and Follow-up of Carotid Cavernous Sinus Fistulas

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    This report describes color doppler imaging (CDI) in theevaluation and follow-up of nine patients with carotid cavernous sinusfistulas.The orbits and carotid arteries were examined with CDI.In allcases,the diagnosis was confirmed by angiography.CDI clearlydemonstrated the dilated superior ophthalmic veins (SOVs) with retrogradeflow and low resistance arterial doppler waveform in all nine patients (10eyes).After the study of quantitative hemodynamics,we found that directcarotid cavernous sinus fistulas s...

  11. Detecting cerebral vascular diseases by transcranial Doppler ultrasound and dolor Doppler flow imaging in comparison with digital subtracted angiography%经颅多普勒及彩色多普勒超声与数字减影脑血管造影对脑血管病检测的比较研究

    Institute of Scientific and Technical Information of China (English)

    张小征; 吕健; 李俊; 胡军民; 龚杰; 徐国政; 马廉亭; 陈欣林

    2000-01-01

    目的 评价经颅多普勒(TCD)在脑血管病诊断、疗效评估中的应用价值.方法 应用TCD检测脑血管疾病患者306例,其中脑动静脉畸形170例,颈动脉海绵窦瘘(CCF)69例,颅内动脉瘤67例,并与数字减影脑血管造影(DSA)、彩色多普勒血流显像(CDFI)进行对比研究.结果 以DSA脑血管造影为金指标,TCD检出脑动静脉畸形93%,检出CCF 100%,判断CCF静脉回流方式96%,检出颅内动脉瘤67%.对颅内较大动脉瘤(>1.5 cm)的检测有特征性表现,提出"动脉瘤样频谱"的新概念.结论 TCD能实时、准确反映颅内血流动力学改变及侧支循环代偿情况,对脑血管病诊断、疗效评估有重要价值.%Objective To evaluate the effect of transcranial Doppler(TCD)on detecting cerebral vascular diseases.Methods One hundred and seventy patients with cerebral arterovenous malformation(AVM),69 carotid cavernous fistula (CCF)and 67 intracranial aneurysms were examined early or late by TCD, digital subtracted angiography(DSA)and color Dopplor flow imaging.The results of their examination were compared and analyzed.Results If the results of DSA examination was regarded as a golden criterion of diagnosing the cerebral vascular dNease,93%of the 170 patients with AVMs,100% of the 69 patients with CCFS and 67%of the 67 patients with the aneurysms were detected by TCD respectively.It was found that the relative larger aneurysm(diameter>1.5 crn)had a special TCD manifestation, the SO-called ”aneurysm-like spectrum”. Conclusion TCD is necessary for detecting the cerebral vascular lesions because it can reveal accurately a change in the intracranial hemodynamics and a condition of the collateral circulation.

  12. [Brainstem auditory evoked potentials and somatosensory evoked potentials in Chiari malformation].

    Science.gov (United States)

    Moncho, Dulce; Poca, María A; Minoves, Teresa; Ferré, Alejandro; Rahnama, Kimia; Sahuquillo, Juan

    2013-06-16

    Introduccion. La malformacion de Chiari (MC) incluye una serie de anomalias congenitas que tienen como comun denominador la ectopia de las amigdalas del cerebelo por debajo del foramen magno, lo que puede condicionar fenomenos compresivos del troncoencefalo, la medula espinal alta y los nervios craneales, alterando las respuestas de los potenciales evocados auditivos del tronco cerebral (PEATC) y de los potenciales evocados somatosensoriales (PESS). Sin embargo, las indicaciones de ambas exploraciones en las MC han sido motivo de estudio en un numero limitado de publicaciones, centradas en series cortas y heterogeneas de pacientes. Objetivo. Revisar los hallazgos de los PEATC y los PESS en los estudios publicados en pacientes con MC tipo 1 (MC-1) o tipo 2 (MC-2), y su indicacion en el diagnostico, tratamiento y seguimiento, especialmente en la MC-1. Desarrollo. Es un estudio de revision realizado mediante analisis de los estudios publicados en Medline desde 1966, localizados mediante PubMed, utilizando combinaciones de las palabras clave 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' y 'somatosensory evoked potentials', asi como informacion de pacientes con MC-1 valorados en los servicios de neurocirugia y neurofisiologia clinica del Hospital Universitari Vall d'Hebron. Conclusiones. Los hallazgos mas comunes de los PESS son la reduccion en la amplitud cortical para el nervio tibial posterior, la reduccion o ausencia del potencial cervical del nervio mediano y el aumento del intervalo N13-N20. En el caso de los PEATC, los hallazgos mas frecuentes descritos son el aumento del intervalo I-V y la alteracion periferica o coclear.

  13. Hazard assessment of the stability of a cavern roof along the coastline

    Science.gov (United States)

    Reina, A.; Lollino, P.

    2009-04-01

    This work concerns the hazard assessment about the stability of a large shallow depth cavern, located along the coastline rocky sector of Polignano town (Apulia, Southern Italy) under an intensely urbanised area. This cavern, which lies at the sea level, has been created by a prolonged process of sea erosion within a rock mass formed of a lower stratified limestone mass and an upper Gravina Calcarenite mass. The thickness of the cavern roof, which has a dome shape, is less than 10 metres in the centre. Important buildings, as hotels and private houses, are located just above the top of the roof. Erosion processes have been observed to be still active along the whole cavern due to climate factors and, in particular, to sea salt weathering and sea spray effects. In 2007 a large calcarenite block, 3 m large, fell down from the cavern roof and consequently a field investigation campaign was carried out for a rational stabilization plan in order to understand the current stability conditions of the roof and the potential failure mechanism. Therefore, a thorough geo-structural survey has firstly been carried out, together with laboratory and in-situ testing for measuring the physical and mechanical properties of the calcarenite rock and of the corresponding joints. A monitoring system has also been planned and installed in order to measure the erosional rate and the block displacements in the cavern.

  14. Intelligent Stability Design of Large Underground Hydraulic Caverns: Chinese Method and Practice

    Directory of Open Access Journals (Sweden)

    Xiating Feng

    2011-10-01

    Full Text Available The global energy shortage has revived the interest in hydroelectric power, but extreme geological condition always pose challenges to the construction of hydroelectric power stations with large underground caverns. To solve the problem of safe design of large underground caverns, a Chinese-style intelligent stability design, representing recent developments in Chinese techniques for the construction of underground hydropower systems is presented. The basic aim of this method is to help designers improve the stability and design efficiency of large underground hydropower cavern groups. Its flowchart consists of two parts, one is initial design with an ordinal structure, and the other is dynamic design with a closed loop structure. In each part of the flowchart, analysis techniques, analysis content and design parameters for caverns’ stability are defined, respectively. Thus, the method provides designers with a bridge from the basic information of objective engineering to reasonable design parameters for managing the stability of hydraulic cavern groups. Application to two large underground caverns shows that it is a scientific and economical method for safely constructing underground hydraulic caverns.

  15. Deformation and Failure Characteristics of the Rock Masses around Deep Underground Caverns

    Directory of Open Access Journals (Sweden)

    Chong Zhang

    2015-01-01

    Full Text Available The deformation and failure characteristics of deep rock masses are the focus of this study on deep rock mass engineering. The study identifies the deformation and failure characteristics of a deep cavern under different ground stress conditions using model test and theoretical analysis methods. First, the similarity theory for model tests is introduced, and then the scale factors used in the present study are calculated according to the Froude criterion. Based on the study objectives, the details of the study methods (the similarity coefficient, the loading conditions, the test steps, etc. are introduced. Finally, the failure characteristics of the deep cavern and the strain distribution characteristics surrounding the caverns under different ground stress conditions are identified using the model test. It was found that compared with shallow rock masses the rock masses of the deep cavern have a much greater tensile range, which reaches 1.5 times the diameter of the cavern under the conditions established in the present study. Under different ground stress conditions, there are differences in failure characteristics and the reasons of the differences were analyzed. The implication of the test results on the design of support system for deep caverns was presented.

  16. Probabilistic Analysis of a Rock Salt Cavern with Application to Energy Storage Systems

    Science.gov (United States)

    Mahmoudi, Elham; Khaledi, Kavan; Miro, Shorash; König, Diethard; Schanz, Tom

    2017-01-01

    This study focuses on the failure probability of storing renewable energy in the form of hydrogen or compressed air in rock salt caverns. The validation of the short- and long-term integrity and stability of rock salt cavern is a prerequisite in their design process. The present paper provides a reliability-based analysis of a typical renewable energy storage cavern in rock salt. An elasto-viscoplastic creep constitutive model is implemented into a numerical model of rock salt cavern to assess its behavior under different operation conditions. Sensitivity measures of different variables involved in the mechanical response of cavern are computed by elementary effect global sensitivity method. Subset simulation methodology is conducted to measure the failure probability of the system with a low computational cost. This methodology is further validated by a comparison with a Monte Carlo-based probabilistic analysis. The propagation of parameter uncertainties and the failure probability against different failure criteria are evaluated by utilizing a Monte Carlo-based analysis. In this stage, the original finite element model is substituted by a surrogate model to further reduce the computational effort. Finally, a reliability analysis approach is employed to obtain the minimum admissible internal pressure in a cavern.

  17. A rare case of Chiari type-1 malformation accompanied by symptomatic cerebrospinal fluid hypovolemia: comparison of congenital Chiari type-1 malformation and acquired Chiari malformation secondary to cerebrospinal fluid hypovolemia: case report.

    Science.gov (United States)

    Hatae, Ryusuke; Kohri, Ryusuke; Maeda, Kazushi; Miyazono, Masayuki

    2014-01-01

    A 23-year-old woman was injured in a rear-end collision. She had general malaise and posterior neck pain, which were more severe when she was in an upright position. Magnetic resonance imaging (MRI) revealed the presence of cerebellar tonsil descensus and syringomyelia in the spinal cord. Radioisotope (RI) cisternography showed signs of an early accumulation of RI in the bladder, and a delayed accumulation of RI in the cerebral fornix. We considered the possibilities of cerebrospinal fluid (CSF) hypovolemia and congenital Chiari type-1 malformation as being responsible for her headache. To obtain a definitive diagnosis, we performed gadolinium (Gd)-enhanced MR cisternography and found evidence of CSF leakage. We performed an epidural blood patch (EBP), and her symptoms resolved. In 2 years since the episode, her symptoms have not recurred, and additional treatment has not been required. In addition, MRI performed 2 years after the EBP did not reveal any changes. There seems no previous report which described successful differentiation of pre-existing congenital Chiari type-1 malformation from the acquired one caused by symptomatic CSF hypovolemia. Because treatment protocols differ between these two conditions, the establishment of a correct diagnosis is important.

  18. Acute abducens nerve palsy as a presenting feature in carotid-cavernous fistula in a 6-year-old girl [

    Directory of Open Access Journals (Sweden)

    Pawar, Neelam

    2013-04-01

    Full Text Available [english] Carotid-cavernous fistulas (CCF are abnormal communications between the internal carotid artery and the cavernous sinus. Traumatic carotid-cavernous fistulae are rare potential complications of craniofacial trauma. Typical findings of CCF are proptosis, chemosis, headache, oculomotor or abducens nerve palsy, trigeminal pain and pulsating bruit over the temporal skull and the bulb.CCF are reported very rarely in childhood. This report describes the clinical and radiological findings of a pediatric patient presented with CCF.

  19. PET in malformations of cortical development; La tomographie d'emission de positons (TEP) dans les malformations corticales de developpement

    Energy Technology Data Exchange (ETDEWEB)

    Bouilleret, V.; O' Brien, T.J. [Department of medicine, the Royal Melbourne Hospital, Royal Parade, Parkville, 3005 Victoria (Australia); Bouilleret, V. [Unite de neurophysiologie clinique et d' epileptologie, AP-HP, CHU Bicetre, 94275 Paris (France); Bouilleret, V.; Chiron, C. [Service hospitalier Frederic-Joliot, DRM, CEA, 4, place du General-Leclerc, 91401 Orsay cedex (France); Chiron, C. [Inserm U663, AP-HP, hopital Necker, 75015 Paris (France); University Paris-Descartes, 11, rue Pierre-et-Marie-Curie, 75005 Paris (France)

    2009-01-15

    Within the group of malformations of cortical development, focal cortical dysplasia (FCD) are an increasingly recognized cause of intractable epilepsy that can be cured by surgery. The success of cortical resection for intractable epilepsy is highly dependent on the accurate pre-surgical delineation of the regions responsible for generating seizures. [{sup 18}F]-FDG PET, which images cerebral metabolism studying brain glucose uptake, is the most established functional imaging modality in the evaluation of patients with epilepsy. The aim of this article is to review [{sup 18}F]-FDG PET usefulness as a pre-surgical tool in the evaluation of medically refractory partial epilepsy. It has an established place in assisting in the localisation and definition of FCD in patients with no lesion, or only a subtle abnormality, on MRI. The role of FDG-PET in defining the extent of the surgical resection is still uncertain and needs to be the focus of future research. (authors)

  20. Arteriovenous Malformation Detected by Small Bowel Endoscopy

    Directory of Open Access Journals (Sweden)

    Takaaki Fujii

    2014-10-01

    Full Text Available Gastrointestinal bleeding that originates in the small intestine is often difficult to diagnose. When successful diagnosis reveals a lesion that can be localized preoperatively, the laparoscopic approach is an appropriate and beneficial treatment modality for small bowel resection. A 69-year-old man presented with a 6-month history of gastrointestinal bleeding and symptomatic transfusion-dependent anemia. Upper and lower endoscopy were normal. Double-balloon endoscopy established the source of the bleeding as a 0.5-cm polypoid mass appearing as a submucosal tumor with redness and pulsation in the lower ileum, suggesting a vascular lesion. Laparoscopic small bowel resection was successful in removing the mass in the ileum. Histological evaluation of the mass revealed an arteriovenous malformation. Preoperative small bowel endoscopy can be useful for diagnosing the cause and localization of arteriovenous malformation in the small intestine.

  1. Combined spinal intramedullary arteriovenous malformation and lipomyelomeningocele

    Energy Technology Data Exchange (ETDEWEB)

    Weon, Y.C.; Roh, H.G.; Byun, H.S. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology, Seoul (Korea); Chung, J.I. [Medimoa Hospital, Department of Radiology, Seoul (Korea); Eoh, W. [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea)

    2005-10-01

    Combined spinal arteriovenous malformation and lipomyelomeningocele are extremely rare. We present a rare combined case of a lipomyelomeningocele with an intramedullary arteriovenous malformation (AVM) occurred at the L3-L4 level in a 30-year-old man who suffered from low back pain radiating to the lower extremities, dysuria, and frequency for 5 years. The MR studies showed an intradural mass with high-signal intensity on both T1-weighted and T2-weighted images, intermingled with multiple signal-void structures. The mass extended extradurally toward a subcutaneously forming fatty mass on the patient's back. Spinal angiography showed an AVM supplied by the radiculopial branches of the lumbar arteries and drained by tortuous, dilated, perimedullary veins. Endovascular embolization and surgical resection were performed. (orig.)

  2. Advanced Underground Gas Storage Concepts: Refrigerated-Mined Cavern Storage, Final Report

    Energy Technology Data Exchange (ETDEWEB)

    none

    1998-09-30

    Over the past 40 years, cavern storage of LPG's, petrochemicals, such as ethylene and propylene, and other petroleum products has increased dramatically. In 1991, the Gas Processors Association (GPA) lists the total U.S. underground storage capacity for LPG's and related products of approximately 519 million barrels (82.5 million cubic meters) in 1,122 separate caverns. Of this total, 70 are hard rock caverns and the remaining 1,052 are caverns in salt deposits. However, along the eastern seaboard of the U.S. and the Pacific northwest, salt deposits are not available and therefore, storage in hard rocks is required. Limited demand and high cost has prevented the construction of hard rock caverns in this country for a number of years. The storage of natural gas in mined caverns may prove technically feasible if the geology of the targeted market area is suitable; and economically feasible if the cost and convenience of service is competitive with alternative available storage methods for peak supply requirements. Competing methods include LNG facilities and remote underground storage combined with pipeline transportation to the area. It is believed that mined cavern storage can provide the advantages of high delivery rates and multiple fill withdrawal cycles in areas where salt cavern storage is not possible. In this research project, PB-KBB merged advanced mining technologies and gas refrigeration techniques to develop conceptual designs and cost estimates to demonstrate the commercialization potential of the storage of refrigerated natural gas in hard rock caverns. DOE has identified five regions, that have not had favorable geological conditions for underground storage development: New England, Mid-Atlantic (NY/NJ), South Atlantic (DL/MD/VA), South Atlantic (NC/SC/GA), and the Pacific Northwest (WA/OR). PB-KBB reviewed published literature and in-house databases of the geology of these regions to determine suitability of hard rock formations for siting

  3. Vascular malformations of the mandible (intraosseous haemangiomas)

    Energy Technology Data Exchange (ETDEWEB)

    Guibert-Tranier, F.; Piton, J.; Caille, J.M.; Riche, M.C.; Merland, J.J.

    1982-11-01

    Haemangiomas of the mandible are relatively rare arteriovenous malformations: 85 cases have been described since 1849. The severe risk of bleeding during tooth extraction or biopsy led us to describe the radiological and angiographic features of this condition. The afferent vessles are the inferior dental, the lingual and the facial arteries. Preoperative embolization reduces the risk of bleeding at operation. Surgical treatment must assure the complete removal of the lesion. Radiotherapy and vascular ligatures are useless and dangerous.

  4. Diagnosis and evaluation of intracranial arteriovenous malformations

    OpenAIRE

    Andrew Conger; Charles Kulwin; Lawton, Michael T; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Ideal management of intracranial arteriovenous malformations (AVMs) remains poorly defined. Decisions regarding management of AVMs are based on the expected natural history of the lesion and risk prediction for peritreatment morbidity. Microsurgical resection, stereotactic radiosurgery, and endovascular embolization alone or in combination are all viable treatment options, each with different risks. The authors attempt to clarify the existing literature′s understanding of the natu...

  5. Clinical Outcome Measures in Chiari I Malformation.

    Science.gov (United States)

    Yarbrough, Chester K; Greenberg, Jacob K; Park, Tae Sung

    2015-10-01

    Chiari malformation type 1 (CM-I) is a common and often debilitating neurologic disease. Reliable evaluation of treatments has been hampered by inconsistent use of clinical outcome measures. A variety of outcome measurement tools are available, although few have been validated in CM-I. The recent development of the Chicago Chiari Outcome Scale and the Chiari Symptom Profile provides CM-I-specific instruments to measure outcomes in adults and children, although validation and refinement may be necessary.

  6. Magnetic Resonance Imaging of Malformations of Midbrain-Hindbrain.

    Science.gov (United States)

    Abdel Razek, Ahmed Abdel Khalek; Castillo, Mauricio

    2016-01-01

    We aim to review the magnetic resonance imaging appearance of malformations of midbrain and hindbrain. These can be classified as predominantly cerebellar malformations, combined cerebellar and brain stem malformations, and predominantly brain stem malformations. The diagnostic criteria for the majority of these morphological malformations are based on neuroimaging findings. The predominantly cerebellar malformations include predominantly vermian hypoplasia seen in Dandy-Walker malformation and rhombencephalosynapsis, global cerebellar hypoplasia reported in lissencephaly and microlissencephaly, and unilateral cerebellar hypoplasia seen in PHACES, vanishing cerebellum, and cerebellar cleft. Cerebellar dysplasias are seen in Chudley-McCullough syndrome, associated with LAMA1 mutations and GPR56 mutations; Lhermitte-Duclos disease; and focal cerebellar dysplasias. Cerebellar hyperplasias are seen in megalencephaly-related syndromes and hemimegalencephaly with ipsilateral cerebellomegaly. Cerebellar and brain stem malformations include tubulinopathies, Joubert syndrome, cobblestone malformations, pontocerebellar hypoplasias, and congenital disorders of glycosylation type Ia. Predominantly brain stem malformations include congenital innervation dysgenesis syndrome, pontine tegmental cap dysplasia, diencephalic-mesencephalic junction dysplasia, disconnection syndrome, and pontine clefts.

  7. Congenital cystic lung malformations; Konnatale zystische Lungenfehlbildungen

    Energy Technology Data Exchange (ETDEWEB)

    Stoever, B.; Scheer, I.; Bassir, C. [Klinik fuer Strahlenheilkunde, Berlin (Germany). Abt. Paediatrische Radiologie, Charite; Mau, H. [Campus Virchow-Klinikum, Klinik fuer Kinderchirurgie, Berlin (Germany); Chaoui, R. [Campus Mitte, Klinik fuer Geburtsmedizin, Berlin (Germany); Henrich, W. [Campus Virchow-Klinikum, Klinik fuer Geburtsmedizin, Berlin (Germany); Schwabe, M. [Campus Mitte, Inst. fuer Pathologie, Berlin (Germany); Wauer, R. [Campus Mitte, Klinik fuer Neonatologie, Berlin (Germany)

    2006-04-15

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  8. Giant Arteriovenous Malformation of the Neck

    Directory of Open Access Journals (Sweden)

    P. A. Dieng

    2015-01-01

    Full Text Available Arteriovenous malformations (AVM have a wide range of clinical presentations. Operative bleeding is one of the most hazardous complications in the surgical management of high-flow vascular malformations. In the cervical region, the presence of vital vascular structures, such as the carotid artery and jugular vein, may increase this risk. This is a case of massive arteriovenous malformation deforming the neck and the face aspect of this aged lady and growing for several years. A giant mass of the left neck occupied the carotid region and the subclavian region. The AVM was developed between the carotid arteries, jugular veins, and vertebral and subclavian vessels, with arterial and venous flux. The patient underwent surgery twice for the cure of that AVM. The first step was the ligation of the external carotid. Seven days later, the excision of the mass was done. In postoperative period the patient presented a peripheral facial paralysis which completely decreased within 10 days. The first ligation of the external carotid reduces significantly the blood flow into the AVM. It permitted secondarily the complete ablation of the AVM without major bleeding even though multiple ligations were done.

  9. Malformations of cortical development and neocortical focus.

    Science.gov (United States)

    Luhmann, Heiko J; Kilb, Werner; Clusmann, Hans

    2014-01-01

    Developmental neocortical malformations resulting from abnormal neurogenesis, disturbances in programmed cell death, or neuronal migration disorders may cause a long-term hyperexcitability. Early generated Cajal-Retzius and subplate neurons play important roles in transient cortical circuits, and structural/functional disorders in early cortical development may induce persistent network disturbances and epileptic disorders. In particular, depolarizing GABAergic responses are important for the regulation of neurodevelopmental events, like neurogenesis or migration, while pathophysiological alterations in chloride homeostasis may cause epileptic activity. Although modern imaging techniques may provide an estimate of the structural lesion, the site and extent of the cortical malformation may not correlate with the epileptogenic zone. The neocortical focus may be surrounded by widespread molecular, structural, and functional disturbances, which are difficult to recognize with imaging technologies. However, modern imaging and electrophysiological techniques enable focused hypotheses of the neocortical epileptogenic zone, thus allowing more specific epilepsy surgery. Focal cortical malformation can be successfully removed with minimal rim, close to or even within eloquent cortex with a promising risk-benefit ratio.

  10. Otosclerosis associated with type B-1 inner ear malformation

    OpenAIRE

    De Stefano, A.; DISPENZA, F.; Aggarwal, N.; Russo, A.

    2010-01-01

    Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossi...

  11. "COCHLEAR IMPLANTATION IN PATIENTS WITH INNER EAR MALFORMATIONS"

    OpenAIRE

    P. Borghei S. Abdi; M. Motesaddi Zari; Khalessi MH

    2004-01-01

    Performing cochlear implantation in patients with inner ear malformation has always been a matter of dispute. This study was designed to analyze the operative findings,complications, and postoperative performance of patients with inner ear anomalies who underwent cochlear implantation. Six patients with inner ear malformations underwent implantation in our academic tertiary referral center from 1997 to 2002. The average follow-up period was 27 months. Malformations included one incomplete par...

  12. [Diagnosis of fetal malformations with ultrasound--state of development].

    Science.gov (United States)

    Fendel, M; Fendel, H

    1983-01-01

    Ultrasonography is of great importance for the prenatal diagnosis of fetal malformations and abnormalities. An early diagnosis in the second trimester is of great interest for an intrauterine or an extrauterine therapy planning (the choice of the time and mode of delivery). Defects of the neural tube including hydrocephalus, malformations of the extremities, the gastrointestinal tract, omphaloceles, the urogenital and cardiac system are described. Four cases of fetal malformations are presented: fetal myelomeningocele, hydrocephalus, bilateral hydronephrosis and lymphangioma with fetal ascites.

  13. Diagnosis and treatment of traumatic carotid cavernous fistula

    Institute of Scientific and Technical Information of China (English)

    杨东虹; 何奇元; 邹咏文; 许民辉

    2002-01-01

    Objective: To discuss the diagnosis and managementof traumatic carotid cavernous fistula (TCCF).Methods: In all 15 patients with TCCF confirmed byangiography, 8 patients got early diagnosis and cure. WithSeldinger technique adpoted in the puncture of femoralartery, Magic 3 F-1.8 F BD catheters combining withballoon were used to embolize the fistula or the internalcarotid artery.Results: Early diagnosis and cure were achieved in 8patients within one week and no sequelae occurred. Sevenpatients with delayed diagnosis who were cured beyond oneweek had some sequelae such as hypopsia in 5 cases,incomplete oculomotor paralyses in 3 and incompleteabducent paralyses in 2. Among all the 15 cases, theinternal carotid artery was preserved in 12 cases acountingfor 80%. Occluding the fistula with sacrifice of the internalcarotid artery was performed in 3 cases and no repatency of the fistula occurred by following up beyond three months.Conclusions: The preferred therapy for TCCF is toocclude the fistula using detachable balloon. The diagnosisand treatment for TCCF can significantly reduce occurrencerate of the complications and sequelae.

  14. Chlorophyll f-driven photosynthesis in a cavernous cyanobacterium.

    Science.gov (United States)

    Behrendt, Lars; Brejnrod, Asker; Schliep, Martin; Sørensen, Søren J; Larkum, Anthony W D; Kühl, Michael

    2015-09-01

    Chlorophyll (Chl) f is the most recently discovered chlorophyll and has only been found in cyanobacteria from wet environments. Although its structure and biophysical properties are resolved, the importance of Chl f as an accessory pigment in photosynthesis remains unresolved. We found Chl f in a cyanobacterium enriched from a cavernous environment and report the first example of Chl f-supported oxygenic photosynthesis in cyanobacteria from such habitats. Pigment extraction, hyperspectral microscopy and transmission electron microscopy demonstrated the presence of Chl a and f in unicellular cyanobacteria found in enrichment cultures. Amplicon sequencing indicated that all oxygenic phototrophs were related to KC1, a Chl f-containing cyanobacterium previously isolated from an aquatic environment. Microsensor measurements on aggregates demonstrated oxygenic photosynthesis at 742 nm and less efficient photosynthesis under 768- and 777-nm light probably because of diminished overlap with the absorption spectrum of Chl f and other far-red absorbing pigments. Our findings suggest the importance of Chl f-containing cyanobacteria in terrestrial habitats.

  15. Treatment Outcome Of Seizures Associated With Intracranial Cavernous Angiomas

    Directory of Open Access Journals (Sweden)

    Nievera Conrad C

    1999-01-01

    Full Text Available Seizures are among the typical presentations of intracranial cavernous angiomas (ICA. Twenty-one patients (age range: 2 to 53 years treated for seizures associated with ICA between 1983 and 1997 were restrospectively studied to evaluate their outcome following medical or surgical intervention. The mean interval between seizure onset and initial presentation at our institution was 7.6 years. Seizures were simple partial in 3 patients, complex partial in 15 and secondarily generalized tonic-clonic in 13. The commonest site of the lesion was the temporal lobe (52%. Multiple angiomas were observed in 5 (24% patients. Seven (32% patients were medically-managed with antiepileptic therapy and 14 (68% underwent either lesionectomy with resection of the epileptogenic zone (9 patients or temporal lobectomy (5 patients. Mean follow-up time was 4 years (range: 3 months to 14 years. Of the medically-managed patients, 3 (43% remained seizure-free whereas 4 (57% continued to have seizures with an average frequency of one per day. Of the surgically-managed patients, 12 (86% became seizure-free and 2 (14% had no more than two seizures per year. Surgery appears to be extremely effective in the management of seizures associated with ICA and should receive a strong and early consideration in patients who fail medical therapy.

  16. Does the risk of cerebral palsy increase or decrease with increasing gestational age?

    Directory of Open Access Journals (Sweden)

    Murphy-Kaulbeck Lynn

    2003-12-01

    Full Text Available Abstract Background It is generally accepted that the risk of cerebral palsy decreases with increasing gestational age of live born infants. However, recent studies have shown that cerebral palsy often has prenatal antecedents including congenital malformations, vascular insults and maternal infection. Cerebral palsy is therefore better viewed as occurring among fetuses, rather than among infants. We explored the epidemiologic implications of this change in perspective. Methods We used recently published data from Shiga Prefecture, Japan and from North-East England to examine the pattern of gestational age-specific rates of cerebral palsy under these alternative perspectives. We first calculated gestational age-specific rates of cerebral palsy as per convention, by dividing the number of cases of cerebral palsy identified among live births within any gestational age category by the number of live births in that gestational age category. Under the alternative formulation, we calculated gestational age-specific rates of cerebral palsy by dividing the number of cases of cerebral palsy identified among live births within any gestational age category by the number of fetuses who were at risk of being born at that gestation and being afflicted with cerebral palsy. Results Under the conventional formulation, cerebral palsy rates decreased with increasing gestational age from 63.9 per 1,000 live births at Conclusions The fetuses-at-risk approach is the appropriate epidemiologic formulation for calculating the gestational age-specific rate of cerebral palsy from a causal perspective. It shows that the risk of cerebral palsy increases as gestational duration increases. This compelling view of cerebral palsy risk may help refocus research aimed at understanding and preventing cerebral palsy.

  17. Notch receptor expression in human brain arteriovenous malformations.

    Science.gov (United States)

    Hill-Felberg, Sandra; Wu, Hope Hueizhi; Toms, Steven A; Dehdashti, Amir R

    2015-08-01

    The roles of the Notch pathway proteins in normal adult vascular physiology and the pathogenesis of brain arteriovenous malformations are not well-understood. Notch 1 and 4 have been detected in human and mutant mice vascular malformations respectively. Although mutations in the human Notch 3 gene caused a genetic form of vascular stroke and dementia, its role in arteriovenous malformations development has been unknown. In this study, we performed immunohistochemistry screening on tissue microarrays containing eight surgically resected human brain arteriovenous malformations and 10 control surgical epilepsy samples. The tissue microarrays were evaluated for Notch 1-4 expression. We have found that compared to normal brain vascular tissue Notch-3 was dramatically increased in brain arteriovenous malformations. Similarly, Notch 4 labelling was also increased in vascular malformations and was confirmed by western blot analysis. Notch 2 was not detectable in any of the human vessels analysed. Using both immunohistochemistry on microarrays and western blot analysis, we have found that Notch-1 expression was detectable in control vessels, and discovered a significant decrease of Notch 1 expression in vascular malformations. We have demonstrated that Notch 3 and 4, and not Notch 1, were highly increased in human arteriovenous malformations. Our findings suggested that Notch 4, and more importantly, Notch 3, may play a role in the development and pathobiology of human arteriovenous malformations.

  18. Anaesthetic management of a child with massive extracranial arteriovenous malformation

    Directory of Open Access Journals (Sweden)

    Faisal Shamim

    2012-01-01

    Full Text Available Vascular tumors affect the head and neck commonly but arteriovenous malformations are rare. Vascular malformations are often present at birth and grow with the patient, usually only becoming significant later in childhood. Embolization has been the mainstay of treatment in massive and complex arteriovenous malformations. We present a case of massive extracranial arteriovenous malformation in a 7-year-old boy causing significant workload on right heart and respiratory distress. The management of angioembolization under general anaesthesia and anaesthetic concerns are presented.

  19. Dandy-Walker Malformation Presenting with Psychological Manifestations

    Directory of Open Access Journals (Sweden)

    Yasodha Maheshi Rohanachandra

    2016-01-01

    Full Text Available Dandy-Walker malformation, which is a congenital malformation of the cerebellum, is documented in literature to be associated with psychotic symptoms, obsessive compulsive symptoms, mood symptoms, hyperactivity, and impulsive behavior. The pathogenesis of psychiatric symptoms in Dandy-Walker malformation is thought to be due to disruption of the corticocerebellar tracts, resulting in what is known as cerebellar cognitive affective syndrome. We present a case of Dandy-Walker malformation presenting with psychiatric symptoms. This case highlights the necessity to be aware of psychiatric manifestations of cerebellar disease as it has an impact on the diagnosis and treatment.

  20. Malformations of the tooth root in humans

    Directory of Open Access Journals (Sweden)

    Hans Ulrich eLuder

    2015-10-01

    Full Text Available The most common root malformations in humans arise from either developmental disorders of the root alone or disorders of radicular development as part of a general tooth dysplasia. The aim of this review is to relate the characteristics of these root malformations to potentially disrupted processes involved in radicular morphogenesis. Radicular morphogenesis proceeds under the control of Hertwig's epithelial root sheath (HERS which determines the number, length, and shape of the root, induces the formation of radicular dentin, and participates in the development of root cementum. Formation of HERS at the transition from crown to root development appears to be very insensitive to adverse effects, with the result that rootless teeth are extremely rare. In contrast, shortened roots as a consequence of impaired or prematurely halted apical growth of HERS constitute the most prevalent radicular dysplasia which occurs due to trauma and unknown reasons as well as in association with dentin disorders. While odontoblast differentiation inevitably stops when growth of HERS is arrested, it seems to be unaffected even in cases of severe dentin dysplasias such as regional odontodysplasia and dentin dysplasia type I. As a result radicular dentin formation is at least initiated and progresses for a limited time. The only condition affecting cementogenesis is hypophosphatasia which disrupts the formation of acellular cementum through an inhibition of mineralization. A process particularly susceptible to adverse effects appears to be the formation of the furcation in multirooted teeth. Impairment or disruption of this process entails taurodontism, single-rooted posterior teeth, and misshapen furcations. Thus even though many characteristics of human root malformations can be related to disorders of specific processes involved in radicular morphogenesis, precise inferences as to the pathogenesis of these dysplasias are hampered by the still limited knowledge on

  1. [Recurrent meningitis in inner ear malformations].

    Science.gov (United States)

    Claros, Pedro; Matusialk, Monika

    2008-01-01

    Authors present two cases of children with reccurent meningitis and unilateral deafness. Implemented diagnostics (CT, NMR, ABR) revealed one side inner ear congenital malformation in one case and anterior fossa bony defect accompanied by labirynthine deformation in the other case. The presence of perilymphatic fistulae in oval and round windows and cerebrospinal fluid leakage has been confirmed in both cases during surgery. Carefull obliteration of the Eustachian tube and both windows has been performed. Non- complicated postoperative course (2 months and 6 years - respectively) has prooved the effectiveness of applied treatment.

  2. Congenital malformations of the temporal bone.

    Science.gov (United States)

    Mukerji, Shraddha S; Parmar, Hemant A; Ibrahim, Mohannad; Mukherji, Suresh K

    2011-08-01

    Congenital ear or temporal bone malformations are a diagnostic challenge to radiologists and surgeons alike. Newer imaging techniques can detect subtle changes in middle ear and cochlear anatomy. This information is invaluable with increasing use of hearing restoration surgeries and/or cochlear implants in such patients. This article discusses the embryogenesis, classification system, and salient imaging findings of congenital outer, middle ear, and inner ear anomalies in children. Both high-resolution computerized tomography and magnetic resonance imaging scans of the temporal bones are described.

  3. Oral vascular malformations: laser treatment and management

    Science.gov (United States)

    Romeo, U.; Rocchetti, F.; Gaimari, G.; Tenore, G.; Palaia, G.; Lo Giudice, G.

    2016-03-01

    Vascular malformations are a very heterogeneous group of circulatory system's diseases that can involve different kind of vessels: arterial, venous or lymphatic ones. Many treatments, such as conventional surgery, embolization, steroid therapy and laser therapy, are available for vascular lesions. The laser approach relies more therapeutic techniques: the transmucosal thermophotocoagulation, intralesional photocoagulation, the excisional biopsy. Today laser is demonstrated to be the gold standard technique to treat vascular lesions that allows a safe and efficient treatment and a lower post-operative healing time. The only disadvantage is the risk of carbonization that could be avoided by using the multiple-spot single pulsed wave technique.

  4. Prevalence of Chiari I Malformation and Syringomyelia.

    Science.gov (United States)

    Kahn, Elyne N; Muraszko, Karin M; Maher, Cormac O

    2015-10-01

    Chiari I malformation (CM) is a common neurosurgical diagnosis and spinal cord syrinx is frequently found in patients with CM. Asymptomatic CM is a common imaging finding. Symptomatic CM is less common. Variation in prevalence estimates may be attributed to differences in sensitivity of CM detection between studies as well as differences in the populations being analyzed. The prevalence of low tonsil position and CM on MRI is higher in children and young adults compared with older adults. Studies that include a large number of older adults find a lower prevalence compared with analyses of children.

  5. Spectacular test of the fire extinguishing system in the underground cavern of the CMS experiment

    CERN Multimedia

    Maximilien Brice

    2006-01-01

    The enormous rumbling heard 100 m under the earth on Friday, 12 May, was not the start of a foam party at CMS. The Safety Team looked on from the second tier of the CMS underground cavern as it reechoed to the sound of water rushing through the two huge pipes overhead and the air was filled with a mixture of water and foam. A minute later it was a winter wonderland, as fluffy puffs of foam came shooting out of the twelve foam blowers lining the upper cavern walls on both sides. In less than two minutes 7 m3 of water mixed with a small percentage of foaming liquid, was transformed into 5600 m3 of foam and discharged into the cavern.

  6. Employees with Cerebral Palsy

    Science.gov (United States)

    ... problems in the muscles or nerves. Instead, faulty development or damage to motor areas in the brain disrupt the brain's ability to adequately control movement and posture (United Cerebral Palsy, 2010). "Cerebral" refers to the ...

  7. United Cerebral Palsy

    Science.gov (United States)

    ... be sure to follow us on Twitter . United Cerebral Palsy UCP educates, advocates and provides support services to ... Partners Merz Logo Sprint Relay Copyright © 2015 United Cerebral Palsy 1825 K Street NW Suite 600 Washington, DC ...

  8. The cavernous sinus in cluster headache - a quantitative structural magnetic resonance imaging study.

    Science.gov (United States)

    Arkink, Enrico B; Schoonman, Guus G; van Vliet, Jorine A; Bakels, Hannah S; Sneeboer, Marjolein Am; Haan, Joost; van Buchem, Mark A; Ferrari, Michel D; Kruit, Mark C

    2017-03-01

    Background It has been hypothesized that a constitutionally narrow cavernous sinus might predispose individuals to cluster headache. Cavernous sinus dimensions, however, have never been assessed. Methods In this case-control study, we measured the dimensions of the cavernous sinus, skull base, internal carotid and pituitary gland with high-resolution T2-weighted magnetic resonance imaging in 25 episodic, 24 chronic and 13 probable cluster headache patients, 8 chronic paroxysmal hemicrania patients and 22 headache-free controls. Dimensions were compared between groups, correcting for age, sex and transcranial diameter. Results On qualitative inspection, no relevant pathology or anatomic variants that were previously associated with cluster headache or chronic paroxysmal hemicranias were observed in the cavernous sinus or paracavernous structures. The left-to-right transcranial diameter at the temporal fossa level (mean ± SD) was larger in the headache groups (episodic cluster headache: 147.5 ± 7.3 mm, p = 0.044; chronic cluster headache: 150.2 ± 7.3 mm, p cluster headache: 146.0 ± 5.3 mm, p = 0.012; and chronic paroxysmal hemicrania: 145.2 ± 9.4 mm, p = 0.044) compared with controls (140.2 ± 8.0 mm). After adjusting for transcranial diameter and correcting for multiple comparisons, there were no differences in the dimensions of the cavernous sinus and surrounding structures between headache patients and controls. Conclusion Patients with cluster headache or chronic paroxysmal hemicrania had wider skulls than headache-free controls, but the proportional dimensions of the cavernous sinus were similar.

  9. Cavernous hemangioma-like kaposi sarcoma: histomorphologic features and differential diagnosis.

    Science.gov (United States)

    Onak Kandemir, Nilüfer; Barut, Figen; Doğan Gün, Banu; Solak Tekin, Nilgün; Hallaç Keser, Sevinç; Oğuz Özdamar, Sükrü

    2013-01-01

    Aim. Cavernous hemangioma-like Kaposi sarcoma is a rare morphologic type of Kaposi sarcoma. So far there are no cases in the literature defining the histological features of this morphologic spectrum in detail. In this study we presented two classical-type cutaneous Kaposi sarcoma cases with histologic findings resembling cavernous hemangioma in company with clinical and histopathological data. Cases. One hundred and eighty-five classical-type cutaneous Kaposi sarcoma lesions in 79 patients were assessed retrospectively in terms of histopathological features. Findings of two cases showing features of cavernous hemangioma-like Kaposi sarcoma whose clinical data could be accessed were presented in accompany with the literature data. Both cases were detected to have bluish-purple, protruded, irregularly bordered cutaneous lesions. Histopathological examination revealed a lesion formed by cavernous hemangioma-like vascular structures organized in a lobular pattern that became dilated and filled with blood. Typical histological findings of early-stage KS, consisting of mononuclear inflammation, extravasated erythrocytes, and a few immature vascular structures in superficial dermis, were observed. All cases were serologically HIV-1 negative. A positive reaction with HHV-8, CD31, CD34, and D2-40 monoclonal antibodies was identified at both cavernous hemangioma-like areas and in immature vascular structures. Results. Cavernous hemangioma-like Kaposi sarcoma is a rare Kaposi sarcoma variant presenting with diagnostic challenges, that may be confused with hemangioma. As characteristic morphological features may not be observed in every case, it is important for diagnostic purposes to show immunohistochemical HHV-8 positivity in this variant.

  10. Manifestations of hepatic cavernous hemangioma in carbon dioxidedigital subtraction angiography

    Institute of Scientific and Technical Information of China (English)

    LU Wei; LI Yan-ha0; HE Xiao-feng; CHEN Yong; ZENG Qing-le

    2002-01-01

    Objective: To describe the characteristic appearance of cavernous hemangioma of the liver (CHL)presented in carbon dioxide digital subtraction angiography (CO2-DSA) and to evaluate the significance of CO2-DSA in the diagnosis of CHL. Methods: Both CO2-DSA and iodinated contrast DSA (IC-DSA) were performed in all 16 patients with CHL, and the angiographic manifestations in the same patients were compared. The image quality was rated by three experienced angiographers, and the complications were also assessed. Results; There was good correlation between angiographers on image quality (R=0. 73). Diagnostic images were obtained with both CO2-DSA and IC-DSA in all CHL patients. No difference was noted between IC-DSA and CO2-DSA in visualizing the proper hepatic arteries and its branches (P>0. 05). CO2-DSA produced better images that clearly described the tumor size, shape and margination than those by IC-DSA (P<0. 05), but both demonstrated characteristic appearances of early opacification and persistent contrast enhancement of the tumors. The portal vein branches near the tumors were constantly demonstrated by CO2-DSA in 15 cases (15/16) but only in 2 cases (2/16) by IC-DSA. Conclusion: CO2-DSA is sensitive in CHL diagnosis, and in patients with contraindications to IC or with unsatisfactory imaging results by IC-DSA,CO2-DSA is a good alternative. As show in most cases by CO2-DSA, the portal veins might act as the main drainage vein of CHLs.

  11. Twin pregnancy in the congenital malformed uterus.

    Science.gov (United States)

    Heinonen, Pentti K

    2016-07-01

    The frequency and outcome of twin pregnancies in women with uterine malformation were studied. The cohort comprised 13 (4.9%) women with twin pregnancy found among 263 women. They had 483 deliveries, 13 of them twins (2.7%; 95% CI 1.6-4.6%). Among 38 patients with unicornuate uterus 5 (6.8%) out of 74 deliveries were twins, 39 women with didelphic uterus 2 (3.2%) out of 62 deliveries and 147 women with septate or subseptate uterus 6 (2.3%) out of 264 deliveries were twins. The mean duration of gestation was 249 days (range 190-268 days), 5 (38%) out of 13 deliveries were premature, 25 out of 26 newborns were alive. Mean durations of gestation and mean weights of newborns did not differ when 7 cases with unicornuate or didelphic uterus were compared to 6 cases with complete or partial uterine septum. A congenital malformed uterus can bear twin pregnancy without severe complications apart from prematurity.

  12. Imaging in spine and spinal cord malformations.

    Science.gov (United States)

    Rossi, Andrea; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo

    2004-05-01

    Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

  13. Imaging in spine and spinal cord malformations

    Energy Technology Data Exchange (ETDEWEB)

    Rossi, Andrea E-mail: a.rossi@panet.itandrearossi@ospedale-gaslini.ge.it; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo

    2004-05-01

    Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

  14. Stenogyria - not only in Chiari II malformation.

    Science.gov (United States)

    Bekiesinska-Figatowska, Monika; Duczkowska, Agnieszka; Brągoszewska, Hanna; Duczkowski, Marek; Mierzewska, Hanna

    2014-12-15

    Stenogyria, meaning multiple small compacted gyri separated by shallow sulci, is reported in the literature in association with Chiari II malformation (CM II) which in turn is reported in association with myelomeningocele (MMC). The authors present five cases of stenogyria (and other abnormalities found in CM II, like callosal hypoplasia/dysplasia, agenesis of the anterior commissure, hypoplasia of the falx cerebri) in children without the history of MMC or any other form of open spinal dysraphism. In these cases stenogyria was associated with Chiari I malformation, rhombencephalosynapsis and spina bifida. Stenogyria, which is not a true neuronal migration disorder, should not be mistaken for polymicrogyria which is also present in CM II. It is histologically different from polymicrogyria because the cortex is normally organized. Also on MRI, the general sulcal pattern is preserved in stenogyria, while it is completely distorted in polymicrogyria. The authors conclude that features traditionally attributed to CM II, like stenogyria, occur not only in the population of patients with MMC as opposed to the widely accepted theory.

  15. A NOVEL PROCESS TO USE SALT CAVERNS TO RECEIVE SHIP BORNE LNG

    Energy Technology Data Exchange (ETDEWEB)

    Michael M. McCall; William M. Bishop; Marcus Krekel; James F. Davis; D. Braxton Scherz

    2005-05-31

    This cooperative research project validates use of man made salt caverns to receive and store the cargoes of LNG ships in lieu of large liquid LNG tanks. Salt caverns will not tolerate direct injection of LNG because it is a cryogenic liquid, too cold for contact with salt. This research confirmed the technical processes and the economic benefits of pressuring the LNG up to dense phase, warming it to salt compatible temperatures and then directly injecting the dense phase gas into salt caverns for storage. The use of salt caverns to store natural gas sourced from LNG imports, particularly when located offshore, provides a highly secure, large scale and lower cost import facility as an alternative to tank based LNG import terminals. This design can unload a ship in the same time as unloading at a tank based terminal. The Strategic Petroleum Reserve uses man made salt caverns to securely store large quantities of crude oil. Similarly, this project describes a novel application of salt cavern gas storage technologies used for the first time in conjunction with LNG receiving. The energy industry uses man made salt caverns to store an array of gases and liquids but has never used man made salt caverns directly in the importation of LNG. This project has adapted and expanded the field of salt cavern storage technology and combined it with novel equipment and processes to accommodate LNG importation. The salt cavern based LNG receiving terminal described in the project can be located onshore or offshore, but the focus of the design and cost estimates has been on an offshore location, away from congested channels and ports. The salt cavern based terminal can provide large volumes of gas storage, high deliverability from storage, and is simplified in operation compared to tank based LNG terminals. Phase I of this project included mathematical modeling that proved a salt cavern based receiving terminal could be built at lower capital cost, and would have significantly higher

  16. Ultimate storage in salt caverns / status report; Endverwahrung von Salzkavernen / Stand der Entwicklung

    Energy Technology Data Exchange (ETDEWEB)

    Crotogino, F.; Schmidt, U. [Kavernen Bau- und Betriebs-GmbH, Hannover (Germany)

    1998-12-31

    The contribution reviews the state of knowledge on final storage in salt caverns. The long-term effects of a hermetically sealed, brine-filled cavern are discussed. So far, there are no valid predictions. (orig.) [Deutsch] In dem Beitrag wird der derzeitige Kenntnis- und Diskussionsstand zur Endverwahrung von Salzkavernen zusammengefasst. Aufbauend auf den bisher vorliegenden Vorstellungen zur Soleimpraegnation bei einem Innendruck, der nahezu dem Ueberlagerungsdruck entspricht, werden die denkbaren langfristigen Auswirkungen einer vollstaendig abgeschlossenen solegefuellten Kaverne skizziert; belastbare Prognosen sind derzeit noch nicht moeglich. (orig.)

  17. Adult onset segmental cavernous hemangioma, varicose veins and limb atrophy (klippel-trenaunay-Weber syndrome variant

    Directory of Open Access Journals (Sweden)

    Sawhney MPS

    1990-01-01

    Full Text Available A 22 year-old woman presented with multiple soft, compressible, protuberant, bluish cutaneous lesions as well as firm, non-compressible, subcutaneous masses and varicose veins affecting the right upper limb of three years duration. There was atrophy of soft tissue of forearm by 2.5 cm. X-ray showed soft tissue densities, multiple phleboliths and hypoplastic forearm bones. Histopathological examination from cutaneous lesions revealed cavernous hemangioma. Adult onset cavernous hemangioma involving one upper limb and breast with multiple phleboliths and limb atrophy is a very unusual presentation of Klippel-Trenaunay-Weber syndrome.

  18. NPHP4 variants are associated with pleiotropic heart malformations.

    NARCIS (Netherlands)

    French, V.M.; Laar, I.M. van de; Wessels, M.W.; Rohe, C.; Roos-Hesselink, J.W.; Wang, G.; Frohn-Mulder, I.M.; Severijnen, L.A.; Graaf, B.M. de; Schot, R.; Breedveld, G.; Mientjes, E.; Tienhoven, M. van; Jadot, E.; Jiang, Z.; Verkerk, A.; Swagemakers, S.; Venselaar, H.; Rahimi, Z.; Najmabadi, H.; Meijers-Heijboer, H.; Graaff, E. de; Helbing, W.A.; Willemsen, R.; Devriendt, K.; Belmont, J.W.; Oostra, B.A.; Amack, J.D.; Bertoli-Avella, A.M.

    2012-01-01

    RATIONALE: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. OBJECTIVE: To identify genetic m

  19. NPHP4 variants are associated with pleiotropic heart malformations

    NARCIS (Netherlands)

    V.M. French (Vanessa); I.M.B.H. van de Laar (Ingrid); M.W. Wessels (Marja); C.F. Rohe; J.W. Roos-Hesselink (Jolien); G. Wang (Guangliang); I.M.E. Frohn-Mulder (Ingrid); E.A.W.F.M. Severijnen (Lies-Anne); B.M. de Graaf (Bianca); R. Schot (Rachel); G.J. Breedveld (Guido); E.J. Mientjes (Edwin); M. van Tienhoven (Marianne); E. Jadot (Elodie); Z. Jiang (Zhengxin); A. Verkerk; S.M.A. Swagemakers (Sigrid); H. Venselaar (Hanka); Z. Rahimi (Zohreh); H. Najmabadi (Hossein); E.J. Meijers-Heijboer (Hanne); E. de Graaff (Esther); W.A. Helbing (Willem); R. Willemsen (Rob); K. Devriendt (Koenraad); J.W. Belmont (John); B.A. Oostra (Ben); J.D. Amack (Jeffrey); A.M. Bertoli Avella (Aida)

    2012-01-01

    textabstractRationale: Congenital heart malformations are a major cause of morbidity and mortality, especially in young children. Failure to establish normal left-right (L-R) asymmetry often results in cardiovascular malformations and other laterality defects of visceral organs. Objective: To identi

  20. Epizootic of ovine congenital malformations associated with Schmallenberg virus infection

    NARCIS (Netherlands)

    Brom, van der R.; Luttikholt, S.J.; Lievaart-Peterson, K.; Peperkamp, N.H.M.T.; Mars, M.H.; Poel, van der W.H.M.; Vellema, P.

    2012-01-01

    Epizootic outbreaks of congenital malformations in sheep are rare and have, to the best of our knowledge, never been reported before in Europe. This paper describes relevant preliminary findings from the first epizootic outbreak of ovine congenital malformations in the Netherlands. Between 25 Novemb

  1. [The progress of inner ear malformation in radiological research].

    Science.gov (United States)

    Kong, Dehua; Fu, Kuang; Zhao, Hui

    2016-01-01

    Inner ear malformations are anomalies linking to development insults at different periods of embryogenesis,which are common causes of congenital sensorineural hearing loss. The evaluation of pediatric sensorineural hearing loss mostly depends on high-resolution computed tomography and magnetic resonance imaging, which can excellently depict the temporal bones and inner ear malformations.

  2. Abernethy malformation with portal vein aneurysm in a child

    OpenAIRE

    Sheragaru H Chandrashekhara; Ashu Seith Bhalla; Arun Kumar Gupta; Vikash, C. S.; Susheel Kumar Kabra

    2011-01-01

    Abernethy malformation is an extremely rare anomaly of the splanchnic venous system. We describe multidetector computed tomography findings of an incidentally detected Abernethy malformation with portal vein aneurysm in a two-and-half-year old child. The computed tomography scan was performed for the evaluation of respiratory distress, poor growth, and loss of appetite.

  3. Pathogenesis and Cerebrospinal Fluid Hydrodynamics of the Chiari I Malformation.

    Science.gov (United States)

    Buell, Thomas J; Heiss, John D; Oldfield, Edward H

    2015-10-01

    This article summarizes the current understanding of the pathophysiology of the Chiari I malformation that is based on observations of the anatomy visualized by modern imaging with MRI and prospective studies of the physiology of patients before and after surgery. The pathogenesis of a Chiari I malformation of the cerebellar tonsils is grouped into 4 general mechanisms.

  4. Craniovertebral Junction Instability in the Setting of Chiari I Malformation.

    Science.gov (United States)

    Goldstein, Hannah E; Anderson, Richard C E

    2015-10-01

    This article addresses the key features, clinical presentation, and radiographic findings associated with craniovertebral junction instability in the setting of Chiari I malformation. It further discusses surgical technique for treating patients with Chiari I malformation with concomitant craniovertebral junction instability, focusing on modern posterior rigid instrumentation and fusion techniques.

  5. Abernethy malformation with portal vein aneurysm in a child

    Directory of Open Access Journals (Sweden)

    Sheragaru H Chandrashekhara

    2011-01-01

    Full Text Available Abernethy malformation is an extremely rare anomaly of the splanchnic venous system. We describe multidetector computed tomography findings of an incidentally detected Abernethy malformation with portal vein aneurysm in a two-and-half-year old child. The computed tomography scan was performed for the evaluation of respiratory distress, poor growth, and loss of appetite.

  6. Valproic acid monotherapy in pregnancy and major congenital malformations

    DEFF Research Database (Denmark)

    Jentink, Janneke; Loane, Maria A; Dolk, Helen

    2010-01-01

    The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited.......The use of valproic acid in the first trimester of pregnancy is associated with an increased risk of spina bifida, but data on the risks of other congenital malformations are limited....

  7. Dural sinus malformation (DSM) in fetuses. Diagnostic value of prenatal MRI and follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Merzoug, Valerie; Drissi, Cyrine; Adamsbaum, Catherine [Hopital Saint Vincent de Paul, Service de Radiopediatrie, Paris (France); Flunker, Sabrina; Couture, Alain [Hopital Arnaud de Villeneuve, Service de Radiopediatrie, Montpellier cedex 5 (France); Eurin, Danielle [Hopital Charles Nicolle, Service de Radiopediatrie, Rouen (France); Grange, Gilles [Hopital Cochin, Service de Gyneco-Obstetrique, Maternite Port-Royal, Paris (France); Garel, Catherine [Hopital Armand Trousseau, Service de Radiopediatrie, Paris (France); Richter, Brigitte [Hopital Clemenceau, Service de Radiopediatrie, Caen (France); Geissler, Frederic [Centre Hospitalier Universitaire, Service de Radiopediatrie, Clermont Ferrand (France)

    2008-04-15

    Dural sinus malformations (DSM) are rare malformations mainly reported after birth. The objectives of this study are to describe their prenatal patterns and to focus on their possible favorable outcome. This multicenter retrospective study reported 13 cases of DSM prenatally diagnosed. The admission criterion was a dural mass posterior to the vermis. In 12 patients, MRI was performed after US. Follow-up in 10 born babies (mean: 8 months) and three neuropathological examinations were available. In all fetuses, DSM presented as a well-delimited round mass involving the torcular. The follow-up examinations (n = 10) revealed progressive thrombosis of the DSM marked by a heterogeneous pattern (US and MRI) with concentric rings. The volume of the mass decreased, with complete regression in seven patients (five before and two after birth). One child died at the age of 5 months in the context of major hydrocephalus and another developed atrophy of the frontal lobes. The eight other babies were doing well (5 days to 3 years) without any treatment (n = 6) or following treatment for hydrocephalus (n = 2). Prenatal DSM may have a typical MR pattern, and the prognosis might not be as bad as has previously been reported. In the absence of criterion to predict the hydrovenous cerebral imbalance, it is mandatory to check the parenchyma and the ventricles during the pregnancy. (orig.)

  8. Timing in neural maturation: arrest, delay, precociousness, and temporal determination of malformations.

    Science.gov (United States)

    Sarnat, Harvey B; Philippart, Michel; Flores-Sarnat, Laura; Wei, Xing-Chang

    2015-05-01

    Timing is primordial in initiating and synchronizing each developmental process in tissue morphogenesis. Maturational arrest, delay, and precociousness all are conducive to neurological dysfunction and may determine different malformations depending on when in development the faulty timing occurred, regardless of the identification of a specific genetic mutation or an epigenetic teratogenic event. Delay and arrest are distinguished by whether further progressive development over time can be expected or the condition is static. In general, retardation of early developmental processes, such as neurulation, cellular proliferation, and migration, leads to maturational arrest. Retardation of late processes, such as synaptogenesis and myelination, are more likely to result in maturational delay. Faulty timing of neuronal maturation in relation to other developmental processes causes neurological dysfunction and abnormal electroencephalograph maturation in preterm neonates. Precocious synaptogenesis, including pruning to provide plasticity, may facilitate prenatal formation of epileptic circuitry leading to severe postnatal infantile epilepsies. The anterior commissure forms 3 weeks earlier than the corpus callosum; its presence or absence in callosal agenesis is a marker for the onset of the initial insult. An excessively thick corpus callosum may be due to delayed retraction of transitory collateral axons. Malformations that arise at different times can share a common pathogenesis with variations on the extent: timing of mitotic cycles in mosaic somatic mutations may distinguish hemimegalencephaly from focal cortical dysplasia type 2. Timing should always be considered in interpreting cerebral dysgeneses in both imaging and neuropathological diagnoses.

  9. Gamma knife radiosurgery for arteriovenous malformations located in eloquent regions of the brain

    Directory of Open Access Journals (Sweden)

    Javalkar Vijayakumar

    2009-12-01

    Full Text Available Background : Stereotactic radiosurgery is an effective treatment strategy for selected group of patients with cerebral arteriovenous malformations (AVMs. Aim : The aim of this study was to evaluate the obliteration rates, complications, and patient outcomes after Gamma knife radiosurgery for cerebral arteriovenous malformations (AVMs located in eloquent regions of the brain with an emphasis on neurological morbidity. Materials and Methods : Between 2000 and December 2005, 37 patients with AVMs in eloquent locations (sensory, motor, speech, visual cortex, basal ganglia, and brain stem underwent stereotactic radiosurgery. We retrospectively reviewed the clinical data of these patients to asses the outcomes. Of the 37 patients, only two patients had prior embolization. Three underwent prospective staged volume radiosurgery. Two patients needed redo-radiosurgery for residual AVM. Mean target volume was 9.1 cc. Three lesions had nidus volume more than 20 cc. Average marginal dose was 18.75 Gy. The median duration of follow-up was 23 months (range, 6-60 months. 15 patients had follow-up of more than 36 months. Results : A total of 15 patients had follow-up of more than 36 months, thus available for evaluation of angiographic obliteration rates. Complete angiographic obliteration was documented in seven patients (46.7%. Four patients experienced hemorrhage during the latency period. One patient who had subsequent hemorrhage on follow-up developed worsening of neurological deficit. One patient developed significant sensory symptoms which resolved after steroids. No additional clinical deterioration related to treatment was noted in rest of the patients. Conclusions : AVMs located in eloquent and in deep locations can be treated safely with stereotactic radiosurgery with acceptable obliteration rates and minimal morbidity.

  10. Venous malformations: classification, development, diagnosis, and interventional radiologic management.

    Science.gov (United States)

    Legiehn, Gerald M; Heran, Manraj K S

    2008-05-01

    Venous malformations are categorized as low-flow vascular malformations within the domain of vascular anomalies and are the most common vascular malformation encountered clinically. Venous malformations are by definition present at birth, undergo pari passu growth, and present clinically because of symptoms related to mass effect or stasis. Although diagnosis can usually be made by clinical history and examination, differentiation from other vascular and nonvascular entities often requires an imaging work-up that includes ultrasound, CT, MR imaging, and diagnostic phlebography. All decisions regarding imaging work-up and decision to treat must be coordinated though referral and discussions with a multidisciplinary team and be based on clearly defined clinical indications. Percutaneous image-guided sclerotherapy has become the mainstay of treatment for venous malformations and involves the introduction of any one of a number of endothelial-cidal sclerosants into the vascular spaces of the lesion, with each sclerosant possessing its own unique spectrum of advantages and disadvantages.

  11. [Cochlear implant for malformations of the inner ear].

    Science.gov (United States)

    Aschendorff, A; Laszig, R; Maier, W; Beck, R; Schild, C; Birkenhäger, R; Wesarg, T; Kröger, S; Arndt, S

    2009-06-01

    The radiologic evaluation of the temporal bone in cochlear implant candidates can detect malformations of the inner ear in up to 20% of cases. The aim of our study was to analyze and classify malformations of the inner ear in patients with cochlear implants carried out from 2001 to 2009. Malformations of the inner ear, including malformations of the internal auditory canal were detected in 12.7% of children and 3.4% of adults. Mondini dysplasia was most common and occurred in 45% of cases. The surgical procedure had to be adapted according to the individual malformation. Modification of surgical access, management of intraoperative CSF gusher, choice of electrode array, intraoperative imaging and the use of navigation were the most important factors. Rehabilitation results were generally very positive and corresponded to the expectation depending on the duration of deafness, if no additional handicaps were present.

  12. Cerebral microangiopathies; Zerebrale Mikroangiopathien

    Energy Technology Data Exchange (ETDEWEB)

    Linn, Jennifer [Klinikum der Universitaet Muenchen (Germany). Abt. fuer Neuroradiologie

    2011-03-15

    Cerebral microangiopathies are a very heterogenous group of diseases characterized by pathological changes of the small cerebral vessels. They account for 20 - 30 % of all ischemic strokes. Degenerative microangiopathy and sporadic cerebral amyloid angiography represent the typical acquired cerebral microangiopathies, which are found in over 90 % of cases. Besides, a wide variety of rare, hereditary microangiopathy exists, as e.g. CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy), Fabrys disease and MELAS syndrome (Mitochondrial myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like episodes). (orig.)

  13. Vascular malformations in the maxillofacial region

    Directory of Open Access Journals (Sweden)

    M. Jafari

    1994-06-01

    Full Text Available Congenital vascular lesions occur most often in children. Parents of these children take them to maxillofacial surgeons directly or during the treatment of other complications such as infection or jaw bone disorders. Various terms now used are unable to describe the pathogenesis and mechanism of the effect of vascular lesions on growth and development of facial bone.  Term of hemangioma is used in almost all cases of congenital and acquired vascular lesions, while fibrosis or shrinkage occurs in some of these lesions over the time. There is also some confusion in describing the vascular lesions which primarily affected bones and soft tissue vascular malformations associated with changes in hard tissues.

  14. Pretreatment imaging of peripheral vascular malformations

    Directory of Open Access Journals (Sweden)

    Johnson JB

    2014-10-01

    Full Text Available Joshua B Johnson, Petrice M Cogswell, Michael A McKusick, Larry A Binkovitz, Stephen J Riederer, Phillip M Young Department of Radiology, Mayo Clinic, Rochester, MN, USA Abstract: Peripheral vascular malformations (VMs are complex and diverse vascular lesions which require individualized pretreatment planning. Pretreatment imaging using various modalities, especially magnetic resonance imaging and time-resolved magnetic resonance angiography, is a valuable tool for classifying peripheral VMs to allow proper diagnosis, demonstrate complete extent, identify the nidus, and distinguish between low-flow and high-flow dynamics that determines the treatment approach. We discuss pretreatment imaging findings in four patients with peripheral VMs and how diagnostic imaging helped guide management. Keywords: time-resolved MRA, cartesian acquisition with projection-like reconstruction, endovascular treatment, magnetic resonance angiography

  15. Tratamento cirúrgico das patologias vasculares cerebrais nos pacientes epilépticos Surgical treatment of cerebral vascular pathologies in epiletic patients

    Directory of Open Access Journals (Sweden)

    Aziz Rassi-Neto

    1997-09-01

    Full Text Available A cirurgia das malformações arteriovenosas (MAV e dos cavernomas (angioma cavernoso na maioria das vezes é indicada após episódios de sangramento. Com o desenvolvimento das técnicas de diagnóstico e cirúrgicas na epilepsia de difícil controle, passou a ser cada vez maior a indicação cirúrgica destas lesões vasculares. Apresentamos nove pacientes com lesões vasculares cerebrais e crises muito frequentes, apesar do tratamento clínico adequado. A faixa etária variou de 12 a 42 anos de idade, com média de 25 anos; houve prevalência no sexo masculino (2:1. A cirurgia consistiu na exérese da lesão em todos os casos. Em quatro casos houve também ressecção da área irritativa perilesional, que foi demostrada pela eletrocorticografia. O estudo anatomopatológico das lesões mostrou cinco casos de cavernoma, três de MAV e um de angioma venoso. Quanto à localização, observamos três lesões no lobo temporal, quatro no frontal e duas na região parietal. O acompanhamento ambulatorial mostrou redução das crises em todos os pacientes, sendo que sete evoluíram sem crises após a cirurgia.Surgery of arteriovenous malformations (AVM and of cavernous angiomas (cavernoma in the majority of cases is indicated subsequently to episodes of bleeding. With the development of techniques for diagnosis and surgery for epilepsy of difficult control, indication for surgery of these vascular lesions has become greater. We present nine patients with cerebral vascular lesions and very frequent crises in spite of adequate clinical treatment. Ages ranged from 12 to 42 years with an average of 25 years; there was a prevalence of the male sex (2:1. Surgery consisted of exeresis of the lesion in all cases and in four there was also resection of the perilesional irritative area shown by electrocorticography. The pathologic study of lesions showed five cases of cavernoma, three cases of AVM, and one case of venous angioma. As to localization, we observed

  16. Pontine glioma extending to the ipsilateral cavernous sinus and Meckel's cave: MR appearance.

    Science.gov (United States)

    Yuh, W T; Nguyen, H D; Mayr, N A; Follett, K A

    1992-01-01

    The authors describe an exophytic glioma of the pons that grew into the Meckel's cave and cavernous sinus in a 75-year-old man. Pontine gliomas should be included in the differential diagnosis of a hyperintense, complex cystic mass seen along the distribution of cranial nerve V.

  17. Spall formation in solution mined storage caverns based on a creep and fracture analysis

    Energy Technology Data Exchange (ETDEWEB)

    MUNSON,DARRELL E.

    2000-02-02

    Because of limited direct observation, understanding of the interior conditions of the massive storage caverns constructed in Gulf Coast salt domes is realizable only through predictions of salt response. Determination of the potential for formation of salt spans, leading to eventual salt falls, is based on salt creep and fracture using the Multimechanism-Deformation Coupled Fracture (MCDF) model. This is a continuum model for creep, coupled to continuum damage evolution. The model has been successfully tested against underground results of damage around several test rooms at the Waste Isolation Pilot Plant (WIPP). Model simulations, here, evaluate observations made in the Strategic Petroleum Reserve (SPR) storage caverns, namely, the accumulation of material on cavern floors and evidence of salt falls. A simulation of a smooth cavern wall indicates damage is maximum at the surface but diminishes monotonically into the salt, which suggests the source of salt accumulation is surface sluffing. If a protuberance occurs on the wall, fracture damage can form beneath the protuberance, which will eventually cause fracture, and lead to a salt fall.

  18. Developments in Researches on the Disasters during Cavern Construction in Salt Beds with Multi-Interlayer

    Directory of Open Access Journals (Sweden)

    Huafu Qiu

    2013-08-01

    Full Text Available This study analyses the developments in researches on the disasters during cavern construction in salt beds with multi-interlayer. Relative to the foreign large salt dome landscape, there are many layers of mudstone in salt beds which has a thin single layer. And such special geological conditions increase the difficulty of solution mining in the control of cavity shape and stability of the cavity, which easily leads to disasters. To statistical analysis the disasters in process of cavern building with multi-interlayer in china, it show that: taking Chinese geological conditions for example, the disasters during cavern construction in salt beds with multi-interlayer caused the failure of cavity can be divided into three categories: geological conditions in beds; the damage of cavity wall in the process of cavity building; the distortion of cavity shape expansion. To analyze predisposing factors lead to salt cavern storage failure in detail, the mechanism of the corresponding factors lead to disaster has been summarized. Finally , some suggestions has been proposed to avoid the disaster maybe happened.

  19. CT diagnosis of cavernous hemangioma of the orbit. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Takaki, Yoshikazu; Takahashi, Mutsumasa; Fukui, Kohtaroh; Bussaka, Hiromasa; Takahama, Yuriko; Yano, Tatsushi (Kumamoto Univ. (Japan). School of Medicine)

    1982-11-01

    A case of cavernous hemangioma studied by dynamic CT was reported. Dynamic CT revealed that contrast enhancement progressed from the periphery toward the center and started early to persist over a long period. These findings seemed characteristic of hemangioma of the orbit and other sties including the liver.

  20. Preliminary Technical and Legal Evaluation of Disposing of Nonhazardous Oil Field Waste into Salt Caverns

    Energy Technology Data Exchange (ETDEWEB)

    Ayers, Robert C.; Caudle, Dan; Elcock, Deborah; Raivel, Mary; Veil, John; and Grunewald, Ben

    1999-01-21

    This report presents an initial evaluation of the suitability, feasibility, and legality of using salt caverns for disposal of nonhazardous oil field wastes. Given the preliminary and general nature of this report, we recognize that some of our findings and conclusions maybe speculative and subject to change upon further research on this topic.

  1. Antioxidative mechanism of Lycium barbarum polysaccharides promotes repair and regeneration following cavernous nerve injury

    Institute of Scientific and Technical Information of China (English)

    Zhan-kui Zhao; Hong-lian Yu; Bo Liu; Hui Wang; Qiong Luo; Xie-gang Ding

    2016-01-01

    Polysaccharides extracted from Lycium barbarum exhibit antioxidant properties. We hypothesized that these polysaccharides resist oxida-tive stress-induced neuronal damage following cavernous nerve injury. In this study, rat models were intragastrically administered Lycium barbarum polysaccharides for 2 weeks at 1, 7, and 14 days after cavernous nerve injury. Serum superoxide dismutase and glutathione peroxidase activities signiifcantly increased at 1 and 2 weeks post-injury. Serum malondialdehyde levels decreased at 2 and 4 weeks. At 12 weeks, peak intracavernous pressure, the number of myelinated axons and nicotinamide adenine dinucleotide phosphate-diaphorase-pos-itive nerve ifbers, levels of phospho-endothelial nitric oxide synthase protein and 3-nitrotyrosine were higher in rats administered at 1 day post-injury compared with rats administered at 7 and 14 days post-injury. These ifndings suggest that application of Lycium barbarum polysaccharides following cavernous nerve crush injury effectively promotes nerve regeneration and erectile functional recovery. This neu-roregenerative effect was most effective in rats orally administered Lycium barbarum polysaccharides at 1 day after cavernous nerve crush injury.

  2. Progress on CMS detector lowering: the YE+2 section arriving in the cavern

    CERN Multimedia

    2006-01-01

    On 12 December, a further section of the detector (YE+2) containing the cathode strip chamber made the 10-hour journey underground. This piece is 16 m high and weighs 880 tonnes. There are now four sections of the detector in the experimental cavern, with a further 11 to follow.

  3. Wegener's granulomatosis with unusual cavernous sinus and sella turcica extension

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, M.; Bobek-Billewicz, B. [Dept. of Radiology, Medical University of Gdansk (Poland); Bullo, B.; Hermann, A.; Rutkowski, B. [Dept. of Nephrology, Medical University of Gdansk (Poland)

    1999-07-01

    Intracerebral extension of Wegener's granulomatosis (WG) is rare. We present a patient with oculomotor and trochlear nerve palsy with histologically proved WG. An MR examination revealed granulomatous tissue in nasal cavity, paranasal sinuses with meningeal infiltration, and uncommon penetration into cavernous sinus and sella turcica. The MR images before and during pharmacological therapy are presented. (orig.)

  4. Call-Fleming Syndrome (Reversible Cerebral Artery Vasoconstriction and Aneurysm Associated with Multiple Recreational Drug Use

    Directory of Open Access Journals (Sweden)

    Doniel Drazin

    2013-01-01

    Full Text Available Drug abuse represents a significant health issue. Evidence suggests that recreational drug use has a direct effect on the cerebral vasculature and is of greater concern in those with undiagnosed aneurysms or vascular malformations. The authors report a case of thunderclap headache with a negative head CT and equivocal lumbar puncture after a drug-fueled weekend. The patient underwent diagnostic cerebral angiogram which demonstrated multisegmental, distal areas of focal narrowing of the middle, anterior, posterior, and posterior inferior cerebral artery and an incidental aneurysm. It is often difficult to determine the exact origin of symptoms; thus we were left with a bit of a chicken or the egg debate, trying to decipher which part came first. Either the aneurysm ruptured with associated concomitant vasospasm or it is a case of Call-Fleming syndrome (reversible cerebral artery vasoconstriction with an incidental aneurysm. The authors proposed their management and rationale of this complex case.

  5. Call-fleming syndrome (reversible cerebral artery vasoconstriction) and aneurysm associated with multiple recreational drug use.

    Science.gov (United States)

    Drazin, Doniel; Alexander, Michael J

    2013-01-01

    Drug abuse represents a significant health issue. Evidence suggests that recreational drug use has a direct effect on the cerebral vasculature and is of greater concern in those with undiagnosed aneurysms or vascular malformations. The authors report a case of thunderclap headache with a negative head CT and equivocal lumbar puncture after a drug-fueled weekend. The patient underwent diagnostic cerebral angiogram which demonstrated multisegmental, distal areas of focal narrowing of the middle, anterior, posterior, and posterior inferior cerebral artery and an incidental aneurysm. It is often difficult to determine the exact origin of symptoms; thus we were left with a bit of a chicken or the egg debate, trying to decipher which part came first. Either the aneurysm ruptured with associated concomitant vasospasm or it is a case of Call-Fleming syndrome (reversible cerebral artery vasoconstriction) with an incidental aneurysm. The authors proposed their management and rationale of this complex case.

  6. Gastrointestinal malformations in Gorgan, North of Iran: epidemiology and associated malformations.

    Science.gov (United States)

    Golalipour, Mohammad Jafar; Mobasheri, Elham; Hoseinpour, Kaniz-Reza; Keshtkar, Abbas Ali

    2007-01-01

    The aim of this prospective study was to evaluate the prevalence and pattern of gastrointestinal malformations (GIM) among Iranian newborns in Gorgan, North of Iran. From 1998 through 2003, 37,951 live births in Dezyani hospital in Gorgan, North of Iran, were screened for gastrointestinal malformations. Clinical and demographic factors of diagnosed cases were recorded in a pre-designed questionnaire for analysis; sex, ethnicity, type of GIM and associated anomalies. The overall prevalence rate of gastrointestinal malformations was 10 per 10,000 births. The imperforate anus (5 per 10,000) was the commonest birth defect in gastrointestinal tract. The prevalence rate of GIM was 8.2 per 10,000 in males and 10.7 per 10,000 in females. According to the parental ethnicity, the prevalence rates of GIM were 6.7, 15.8 and 17.6 per 10,000 in Fars, Turkman, and Sistani, respectively. There were eight cases (21%) with associated anomalies. The prevalence rate of GIM in North of Iran is not similar to the previous studies in Iran and Middle East and ethnic background may be a causative factor in the rate of GIM in this area.

  7. Temporary umbilical loop colostomy for anorectal malformations.

    Science.gov (United States)

    Hamada, Yoshinori; Takada, Kohei; Nakamura, Yusuke; Sato, Masahito; Kwon, A-Hon

    2012-11-01

    Transumbilical surgical procedures have been reported to be a feasible, safe, and cosmetically excellent procedure for various pediatric surgical diseases. Umbilical loop colostomies have previously been created in patients with Hirschsprung's disease, but not in patients with anorectal malformations (ARMs). We assessed the feasibility and cosmetic results of temporal umbilical loop colostomy (TULC) in patients with ARMs. A circumferential skin incision was made at the base of the umbilical cord under general anesthesia. The skin, subcutaneous tissue, and fascia were cored out vertically, and the umbilical vessels and urachal remnant were individually ligated apart from the opening in the fascia. A loop colostomy was created in double-barreled fashion with a high chimney more than 2 cm above the level of the skin. The final size of the opening in the skin and fascia was modified according to the size of the bowel. The bowel wall was fixed separately to the peritoneum and fascia with interrupted 5-0 absorbable sutures. The bowel was opened longitudinally and everted without suturing to the skin. The loop was divided 7 days postoperatively, and diversion of the oral bowel was completed. The colostomy was closed 2-3 months after posterior saggital anorectoplasty through a peristomal skin incision followed by end-to-end anastomosis. Final wound closure was performed in a semi-opened fashion to create a deep umbilicus. TULCs were successfully created in seven infants with rectourethral bulbar fistula or rectovestibular fistula. Postoperative complications included mucosal prolapse in one case. No wound infection or spontaneous umbilical ring narrowing was observed. Skin problems were minimal, and stoma care could easily be performed by attaching stoma bag. Healing of umbilical wounds after TULC closure was excellent. The umbilicus may be an alternative stoma site for temporary loop colostomy in infants with intermediate-type anorectal malformations, who undergo radical

  8. Pulmonary arteriovenous malformations: overview and transcatheter embolotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Pugash, R.A. [Univ. of Toronto, St. Michael' s Hospital, Dept. of Medical Imaging, Toronto, Ontario (Canada)

    2001-04-01

    The majority of pulmonary arteriovenous malformations (pAVMs) are found in people with hereditary hemorrhagic telangiectasia (HHT), a condition also known as Osler-Weber-Rendu syndrome. HHT is a clinically heterogeneous autosomal dominant disorder in which abnormal blood vessels cause bleeding and arteriovenous shunting. The 2 basic lesions of HHT - telangiectasias and arteriovenous malformations (AVMs) - are closely related. Multisystem involvement leads to a staggering array of clinical manifestations, making HHT one of medicine's less familiar 'great pretenders'. Telangiectasias are dilated blood vessels, typically located in mucocutaneous surfaces (i.e., skin, conjunctiva, respiratory tract, gastrointestinal tract, urinary tract). Small telangiectasias are simply dilated post-capillary venules, whereas larger telangiectasias are made up of dilated arterioles and venules, often with no intervening capillary. They are, in essence, diminutive AVMs. These tiny lesions are visible as punctate bright red spots on skin and mucosal surfaces (Fig. 1). Their fragility and superficial location account for the disabling epistaxis and chronic gastrointestinal bleeding, which are so common with HHT. Hematuria (caused by urothelial telangiectasias) occurs occasionally but is not a prominent feature of the disease. Although tracheobronchial telangiectasias do occur and may cause hemoptysis, severe hemoptysis is typically related to pAVM rupture. AVMs are direct artery-to-vein connections. Though larger and far more impressive radiologically than telangiectasias, AVMs are more likely to be clinically silent until they either declare themselves in a catastrophic fashion or are detected by screening tests. In contrast to telangiectasias, which are generally found in epithelial surfaces, AVMs tend to develop within organs, most commonly the lung and brain. As screening methods evolve, liver involvement with both telangiectasias and complex AVMs is being recognized

  9. The return of an old worm: cerebral paragonimiasis presenting with intracerebral hemorrhage.

    Science.gov (United States)

    Koh, Eun Jung; Kim, Seung-Ki; Wang, Kyu-Chang; Chai, Jong-Yil; Chong, Sangjoon; Park, Sung-Hye; Cheon, Jung-Eun; Phi, Ji Hoon

    2012-11-01

    Paragonimiasis is caused by ingesting crustaceans, which are the intermediate hosts of Paragonimus. The involvement of the brain was a common presentation in Korea decades ago, but it becomes much less frequent in domestic medical practices. We observed a rare case of cerebral paragonimiasis manifesting with intracerebral hemorrhage. A 10-yr-old girl presented with sudden-onset dysarthria, right facial palsy and clumsiness of the right hand. Brain imaging showed acute intracerebral hemorrhage in the left frontal area. An occult vascular malformation or small arteriovenous malformation compressed by the hematoma was initially suspected. The lesion progressed for over 2 months until a delayed surgery was undertaken. Pathologic examination was consistent with cerebral paragonimiasis. After chemotherapy with praziquantel, the patient was monitored without neurological deficits or seizure attacks for 6 months. This case alerts practicing clinicians to the domestic transmission of a forgotten parasitic disease due to environmental changes.

  10. Neuroimaging in Cerebral Palsy – Report from North India

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    Anju AGGARWAL

    2013-11-01

    Full Text Available How to Cite This Article: Aggarwal A, Mittal H, Debnath SKR, Rai A. Neuroimaging in Cerebral Palsy–Report from North India. Iran J Child Neurol. 2013 Autumn; 7(3:41- 46. ObjectiveOnly few Indian reports exist on neuroimaging abnormalities in children with cerebral palsy (CP from India. Materials & MethodsWe studied the clinico-radiological profile of 98 children diagnosed as CP at a tertiary centre in North India. Relevant investigations were carried out to determine the etiology. ResultsAmong the 98 children studied, 80.5% were males and 22.2% were premature. History of birth asphyxia was present in 41.9%. Quadriplegic CP was seen in 77.5%, hemiplegic in 11.5%, and diplegic in 10.5%. Other abnormalities were microcephaly (60.5%, epilepsy (42%, visual abnormality (37%, and hearing abnormality (20%. Neuroimaging was abnormal in 94/98 (95.91%.Abnormalities were periventricular white matter abnormalities (34%, deep grey matter abnormalities (47.8%, malformations (11.7%, and miscellaneous lesions (6.4%. Neuroimaging findings did not relate to the presence of birth asphyxia, sex, epilepsy, gestation, type of CP, or microcephaly. ConclusionsNeuroimaging is helpful for etiological diagnosis, especially malformations.  ReferencesSinghi PD, Ray M, Suri G. Clinical spectrum of cerebral palsy in north India-an analysis of 1000 cases. J Trop Pediatr 2002 48(3; 162-6.Sharma P, Sharma U, Kabra A. Cerebral Palsy-Clinical Profile and Predisposing Factors. Indian Pediatr 1999;36(10:1038-42.Nelson KB, Ellenberg JH. Antecedents of cerebral palsy. Multivariate analysis of risk. N Engl J Med 1986 315(2:81-6.Krägeloh-Mann I, Horber V. The role of magnetic resonance imaging in elucidating the pathogenesis of cerebral palsy: a systematic review. Dev Med Child Neurol 2007; 49(2:144-51.Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl 2007

  11. Outcome of cochlear implantation in children with cochlear malformations.

    Science.gov (United States)

    Bille, Jesper; Fink-Jensen, Vibeke; Ovesen, Therese

    2015-03-01

    The objective of the study was the evaluation of outcomes of cochlear implantation (CI) in children with cochlear malformations. A retrospective case-control study was conducted in a tertiary referral centre. The patients were children with inner ear malformation judged by high-resolution computed tomography and magnetic resonance imaging treated with uni- or bilateral CI and a follow-up period of at least 3 years. They were matched with a control group of children operated for other reasons. The patients were operated by one of two surgeons using similar techniques including a standard perimodiolar electrode in all cases. The intervention was therapeutic and rehabilitative. The main outcome measures were category of auditory performance (CAP) and speech intelligibility rating (SIR). Eighteen children were diagnosed with cochlear malformations (12 % of children receiving CI). No statistical differences regarding CAP and SIR scores were found between the two groups. Only one child was diagnosed with a common cavity and performed below average. Children with auditory neuropathy performed beyond average. Children with cochlear malformations performed equally to children without malformation in the long term. Standard perimodiolar electrodes can be used despite cochlear malformations. The most important factors determining the outcome is the age of the child at the time of implantation and duration of hearing loss before CI. Awareness towards an increased risk of complications in case of inner ear malformations is recommended.

  12. HISTOLOGICAL STUDY OF NEONATAL BOWEL IN ANORECTAL MALFORMATIONS

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    Amrish Tiwari

    2014-06-01

    Full Text Available Anorectal malformations are the congenital condition, seen in approximately 1 in 5000 live births. It affects male and female in the ratio of 1.3:1. Anorectal malformations include a wide range of malformations, that not only involves the anus and rectum, but it also involves urinary and genital tract. Aims and objectives of the study, was to understand the structures involved in anorectal malformations by histological study of surgically excised segments of involved part of neonatal intestine and to understand the degree and cause of possible structural impairment in different segments of involved parts of neonatal bowel that may help in the surgical management of anorectal malformations. Present study was conducted on surgically excised segments of fifteen cases of anorectal malformations, that have been collected from Department of Paediatrics Surgery, IMS, BHU. After that processing of the samples have been done and blocks have been prepared. Then after sectioning and staining with Hematoxyline and Eosin, findings have been noted under the microscope. Histopathological examination revealed the abnormalities of varying degrees. To conclude this study supports that the malformed segments should be excised, regarding controversial issue of preserving or excising the distal segment of anorectum for better functional outcome.

  13. Eye malformations in Cameroonian children: a clinical survey

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    Eballé AO

    2012-10-01

    Full Text Available André Omgbwa Eballé,1,2 Augustin Ellong,3 Godefroy Koki,3 Ngoune Chantal Nanfack,3 Viola Andin Dohvoma,3 Côme Ebana Mvogo2,31Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital, Yaoundé, Cameroon; 2Faculty of Medicine and Pharmaceutical Sciences, University of Douala, Cameroon; 3Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, CameroonSummary: The aim of this work was to describe the clinical aspects of eye malformations observed at the ophthalmology unit of the Yaoundé Gynaecology, Obstetrics and Pediatrics Hospital.Patients and methods: We carried out a retrospective study of all malformations of the eye and its adnexae observed among children aged 0–5 years who were seen at the ophthalmology unit from January 2003 to December 2009.Results: Out of the 2254 children who were examined, 150 (6.65% presented eye malformations. The mean age was 14.40 ± 4 months. Eye malformations were diagnosed in 71.66% of cases during the first year of life. The most frequent malformations were congenital lacrimal duct obstruction (66.66%, congenital cataract (10.9%, congenital glaucoma (10.9%, microphthalmos (5.03%, and congenital ptosis (3.77%.Conclusion: Eye malformations among children can lead to visual impairment and are a cause for discomfort to children and parents. Therefore, systematic postnatal screening is recommended to enable early management.Keywords: malformations, ophthalmology, child, Cameroon

  14. Maternal Hypothyroidism in Early Pregnancy and Infant Structural Congenital Malformations

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    Bengt Källén

    2014-01-01

    Full Text Available Background. The question is debated on whether maternal hypothyroidism or use of thyroxin in early pregnancy affects the risk for infant congenital malformations. Objectives. To expand the previously published study on maternal thyroxin use in early pregnancy and the risk for congenital malformations. Methods. Data from the Swedish Medical Birth Register were used for the years 1996–2011 and infant malformations were identified from national health registers. Women with preexisting diabetes or reporting the use of thyreostatics, anticonvulsants, or antihypertensives were excluded from analysis. Risk estimates were made as odds ratios (ORs or risk ratios (RRs after adjustment for year of delivery, maternal age, parity, smoking, and body mass index. Results. Among 23 259 infants whose mothers in early pregnancy used thyroxin, 730 had a major malformation; among all 1 567 736 infants, 48012 had such malformations. The adjusted OR was 1.06 (95% CI 0.98–1.14. For anal atresia the RR was 1.85 (95% CI 1.00–1.85 and for choanal atresia 3.14 (95% CI 1.26–6.47. The risk of some other malformations was also increased but statistical significance was not reached. Conclusions. Treated maternal hypothyroidism may be a weak risk factor for infant congenital malformations but an association with a few rare conditions is possible.

  15. Covered Stent Implantation for the Treatment of Direct Carotid-Cavernous Fistula and Its Mid-Term Follow-up

    Science.gov (United States)

    Briganti, F.; Tortora, F.; Marseglia, M.; Napoli, M.; Cirillo, L.

    2009-01-01

    Summary Carotid-cavernous fistulas are abnormal arteriovenous communications either directly between the internal carotid artery and the cavernous sinus or between the dural branches of the internal and external carotid arteries. These fistulas predominantly present with ocular manifestations and they are treated mainly by endovascular techniques in most cases. A detailed review of the literature allowed us to make a complete analysis of the information available on the topic. We describe a case of a direct carotid-cavernous fistula occluded by endovascular implantation of a covered stent, showing the persistence of results after three years. PMID:20465897

  16. Are cavernous sinus hemangiomas and cavernous malformations different entities?%海绵窦血管瘤和海绵状血管畸形是不同的病变吗?

    Institute of Scientific and Technical Information of China (English)

    L. Fernando Gonzalez,Gregory; P.Lekovic,Jennifer Eschbacher; 刘暌; 只达石

    2007-01-01

    海绵状血管畸形是发生在中枢神经系统内具有显著特征的病变,尽管其与海绵窦血管瘤在组织学方面的特征相似,但是表现各异(表1)。由于两种病变的自然病程和治疗手段不同,所以命名应该适当规范。本文对以往文献进行简要回顾。

  17. Neurturin enhances the recovery of erectile function following bilateral cavernous nerve crush injury in the rat

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    Klein Robert D

    2007-03-01

    Full Text Available Abstract Background The molecular mechanisms responsible for the survival and preservation of function for adult parasympathetic ganglion neurons following injury remain incompletely understood. However, advances in the neurobiology of growth factors, neural development, and prevention of cell death have led to a surge of clinical interest for protective and regenerative neuromodulatory strategies, as surgical therapies for prostate, bladder, and colorectal cancers often result in neuronal axotomy and debilitating loss of sexual function or continence. In vitro studies have identified neurturin, a glial cell line-derived neurotrophic factor, as a neuromodulator for pelvic cholinergic neurons. We present the first in vivo report of the effects of neurturin upon the recovery of erectile function following bilateral cavernous nerve crush injury in the rat. Methods In these experiments, groups (n = 8 each consisted of uninjured controls and animals treated with injection of albumin (blinded crush control group, extended release neurotrophin-4 or neurturin to the site of cavernous nerve crush injury (100 μg per animal. After 5 weeks, recovery of erectile function (treatment effect was assessed by cavernous nerve electrostimulation and peak aortic pressures were measured. Investigators were unblinded to specific treatments after statistical analyses were completed. Results Erectile dysfunction was not observed in the sham group (mean maximal intracavernous pressure [ICP] increase of 117.5 ± 7.3 cmH2O, whereas nerve injury and albumin treatment (control produced a significant reduction in ICP elevation of 40.0 ± 6.3 cmH2O. Neurturin facilitated the preservation of erectile function, with an ICP increase of 55% at 62.0 ± 9.2 cmH2O (p Conclusion Treatment with neurturin at the site of cavernous nerve crush injury facilitates recovery of erectile function. Results support further investigation of neurturin as a neuroprotective and/or neuroregenerative

  18. Adrenal Cavernous Hemangioma: A Case Report with Review of the Literature

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    Joseph J. Noh

    2014-05-01

    Full Text Available Context Adrenal cavernous hemangioma is a rare type of tumor that is usually diagnosed post-operatively. There have only been approximately 63 cases reported in the literature to date. Case report We report a case of adrenal cavernous hemangioma in a 27-year-old pregnant woman. The mass was discovered on ultrasonography when she visited a gastroenterologist for vague epigastric discomfort and vomiting. The laboratory tests were within normal limits and did not show any features suggestive of adrenal endocrinologic dysfunction. Computed Tomography (CT revealed a well-defined 7.8 × 7.8 oval mass in the right adrenal gland with speckled calcifications. The mass was removed by transabdominal laparoscopic surgery. Strong positive immunostaining for CD31 and CD34 with weakly positive staining for podoplanin/D2-40 confirmed the diagnosis of cavernous hemangioma. Conclusions We reviewed 52 case reports of adrenal cavernous hemangioma in an attempt to identify tumor characteristics. More than half of the patients reviewed showed a heterogeneous internal structure of the mass with peripheral patchy enhancement on CT. They also showed focal or speckled calcifications either on X-ray or CT. Nevertheless, many of these characteristics overlap with the imaging phenotypes of other common diseases of the adrenal gland and therefore do not seem to provide definite evidence for differential diagnosis. Laparoscopic approach is a feasible and safe modality to remove adrenal cavernous hemangiomas because they seem to form a rigid fibrotic capsule; hence the risk of bleeding due to surgical manipulation is relatively low.

  19. Cerebral gigantism associated with jaw cyst basal cell naevoid syndrome in two families.

    Science.gov (United States)

    Cramer, H; Niederdellmann, H

    1983-01-01

    We report 9 subjects from 2 families with the syndrome of cerebral gigantism, seven of the patients also had jaw cyst basal cell naevoid syndrome. Neurological, radiological, somatic and biochemical features of this hitherto unreported association are described. Neurological symptoms included mild hydrocephalus, ventricular malformation, cerebellar syndrome, intracranial calcification, oculomotor disturbances, EEG abnormalities and rarely, mild peripheral nervous disorders. A disturbance of calcium metabolism appears to be a prominent feature of the genetically determined nonprogressive syndrome.

  20. Anthelmintic induced congenital malformations in sheep embryos using netobimin.

    Science.gov (United States)

    Navarro, M; Cristofol, C; Carretero, A; Arboix, M; Ruberte, J

    1998-01-24

    Benzimidazole compounds have teratogenic effects in domestic and experimental animals. In this study, 14 Manchega ewes were treated orally, under controlled conditions, with 20 mg netobimin (a prodrug of a benzimidazole compound) per/kg bodyweight on the 17th day of pregnancy. Congenital malformations and abortions affected 60 per cent of the lambs. The main malformations were skeletal and renal, but vascular malformations were observed for the first time. The abnormalities were investigated using radiological, dissection and vascular injection techniques, and associations among them were recorded. The anomalies are discussed in terms of embryological considerations.

  1. [Arteriovenous malformation-glioma association: study of four cases].

    Science.gov (United States)

    Borges, Lia Raquel R; Malheiros, Suzana M F; Pelaez, Maria Paula; Stávale, João Norberto; Santos, Adrialdo J; Carrete, Henrique; Nogueira, Roberto Gomes; Ferraz, Fernando A P; Gabbai, Alberto A

    2003-06-01

    We reviewed the clinical presentation, imaging and histopathologic findings in 4 patients with the diagnosis of arteriovenous malformation associated with glioma that were operated on from 1991 to 2000 in our institution. Four patients (2 males; age between 15 and 52 years) presented with progressive headache with clinical evidence of intracranial hypertension (in 3) and partial seizures (in 1). CT scan showed a brain tumor without any detectable pathologic vessels. Histologic examination revealed astrocytic tumors associated with arteriovenous malformation. No patient presented the vascular component intermixed with the tumor. The arteriovenous-glioma association is rare and must be identified by a clear demarcation between the malformation and the tumor.

  2. Otosclerosis associated with type B-1 inner ear malformation.

    Science.gov (United States)

    De Stefano, A; Dispenza, F; Aggarwal, N; Russo, A

    2010-06-01

    Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral otosclerosis.

  3. Congenital inner ear malformations without sensorineural hearing loss in children.

    Science.gov (United States)

    Ozeki, Michio; Kato, Zenichiro; Sasai, Hideo; Kubota, Kazuo; Funato, Michinori; Orii, Kenji; Kaneko, Hideo; Fukao, Toshiyuki; Kondo, Naomi

    2009-10-01

    Inner ear malformations are frequently found in patients with congenital hearing loss. It has been reported that normal hearing is rare in patients with severe inner ear vestibular malformations. A 9-year-old boy had had complained of recurrent dizziness and disequilibrium for 2 months. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was normal. High-resolution magnetic resonance imaging, with three-dimensional reconstruction, showed dysplasia of the bilateral lateral semicircular canals (LSCCs). Isolated vestibular malformation might not be as rare as previously thought, and should be examined by imaging of the temporal bone.

  4. Role of sodium tetradecyl sulfate in venous malformations

    Directory of Open Access Journals (Sweden)

    Saraf Sanjay

    2006-01-01

    Full Text Available Venous malformations are one of the commonest anomalies of the vascular tree and their management has always remained a major challenge. Surgery and other treatment modalities are not always satisfactory and have a higher morbidity, recurrence and complication rate. The author retrospectively analyzed 40 patients of venous malformations who underwent sclerotherapy with sodium tetradecyl sulfate solely or as an adjunct to surgery. The purpose of the study was to evaluate the efficacy and safety of sodium tetradecyl sulfate sclerotherapy in the treatment of venous malformations.

  5. Evaluation of magnetic resonance imaging of Arnold-Chiari malformation

    Energy Technology Data Exchange (ETDEWEB)

    Yoshino, Kimihiro; Suga, Masakazu; Takemoto, Motohisa

    1987-06-01

    We evaluated the usefulness of magnetic resonance imaging (MRI) in the diagnosis of the Arnold-Chiari malformation. The patient, a 52-year-old man, complained of dizziness on walking. He initially refused to undergone myelography, but 4 months later, underwent MRI test, which lead to the diagnosis of Arnold-Chiari malformation (I type). Not all patients with symptoms of Arnold-Chiari malformation, syringomyelia, syringobulbia and cervical spinal tumor, undergone myelography, which is an invasive technique, therefore MRI should be the first examination for the patients with disorders involving the craniocervical junction.

  6. FULL SCIENTIFIC REPORTS - Complex vertebral malformation in Holstein calves

    DEFF Research Database (Denmark)

    Agerholm, Jørgen S.; Bendixen, Christian; Andersen, Ole;

    2001-01-01

    was characterized by shortening of the cervical and thoracic parts of the vertebral column due to multiple hemivertebrae, fused and misshaped vertebrae, and scoliosis. Symmetrical flexures of the carpal joints and the metacarpophalangeal joint in combination with a slight lateral rotation of the phalanges also were...... present. Similar low-grade arthrogryposis was present in the posterior limbs. Fifty percent of the calves had heart malformation. Other malformations occurred in a few calves. Complex vertebral malformation (CVM) is proposed as the designation for this defect. A genetic etiology is indicated because cases...

  7. Congenital malformations of the spinal cord without early symptoms.

    Science.gov (United States)

    Moffie, D; Stefanko, S Z; Makkink, B

    1986-01-01

    Description of 11 patients with congenital malformations of the spinal cord. Six of them were males, five females and the age varied from 7 to 70 years. Most of these cases produced clinical neurological signs indicating spinal cord disease in later life during an intercurrent disease. It was thought that changes in the bloodvessels and/or perfusion of the area of the spinal cord malformation was the ultimate cause of the neurological symptoms. An exact explanation of the origin of these developmental disturbances of the spinal cord remains unknown. Different hypotheses proposed in the literature, concerning these malformations, are not satisfactory.

  8. Post procedure headache in patients treated for neurovascular arteriovenous malformations and aneurysms using endovascular therapy

    DEFF Research Database (Denmark)

    Khan, Sabrina; Amin, Faisal Mohammad; Hauerberg, John

    2016-01-01

    BACKGROUND: Though endovascular therapy (EVT) is increasingly applied in the treatment of intracranial vascular lesions, little is known about the effect of EVT on post-procedure headache. We aimed to investigate the prevalence of headache in patients who have undergone EVT for cerebral arteriove......BACKGROUND: Though endovascular therapy (EVT) is increasingly applied in the treatment of intracranial vascular lesions, little is known about the effect of EVT on post-procedure headache. We aimed to investigate the prevalence of headache in patients who have undergone EVT for cerebral...... arteriovenous malformations (AVMs) and aneurysms. METHODS: A total of 324 patients underwent EVT treatment for aneurysms and AVMs at the Danish National Hospital from January 2012 to December 2014. We applied strict exclusion criteria in order to minimize the effect of other factors on headache occurrence, e.......g., craniotomy. Eligible subjects were phone-interviewed using a purpose-developed semi-structured questionnaire. Headaches were classified according to ICHD-III beta criteria. RESULTS: The 59 patients underwent treatment of aneurysms (n = 43), cranial dural fistulas (n = 11), and AVMs (n = 5...

  9. Is Intracranial Atherosclerosis an Independent Risk Factor for Cerebral Atrophy? A Retrospective Evaluation

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    Zou Kelly H

    2008-12-01

    Full Text Available Abstract Background Our purpose was to study the association between the intracranial atherosclerosis as measured by cavernous carotid artery calcification (ICAC observed on head CT and atrophic changes of supra-tentorial brain demonstrated by MRI. Methods Institutional review board approval was obtained for this retrospective study incorporating 65 consecutive patients presenting acutely who had both head CT and MRI. Arterial calcifications of the intracranial cavernous carotids (ICAC were assigned a number (1 to 4 in the bone window images from CT scans. These 4 groups were then combined into high (grades 3 and 4 and low calcium (grades 1 and 2 subgroups. Brain MRI was independently evaluated to identify cortical and central atrophy. Demographics and cardiovascular risk factors were evaluated in subjects with high and low ICAC. Relationship between CT demonstrated ICAC and brain atrophy patterns were evaluated both without and with adjustment for cerebral ischemic scores and cardiovascular risk factors. Results Forty-six of the 65 (71% patients had high ICAC on head CT. Subjects with high ICAC were older, and had higher prevalence of hypertension, diabetes, coronary artery disease (CAD, atrial fibrillation and history of previous stroke (CVA compared to those with low ICAC. Age demonstrated strong correlation with both supratentorial atrophy patterns. There was no correlation between ICAC and cortical atrophy. There was correlation however between central atrophy and ICAC. This persisted even after adjustment for age. Conclusion Age is the most important determinant of atrophic cerebral changes. However, high ICAC demonstrated age independent association with central atrophy.

  10. Maternal smoking in pregnancy and risk for congenital malformations

    DEFF Research Database (Denmark)

    Leite, Mimmi; Albieri, Vanna; Kjaer, Susanne K.

    2014-01-01

    OBJECTIVE: To examine the association between maternal smoking during pregnancy and risk for congenital malformations. DESIGN: Population-based prospective cohort study. SETTING: Denmark. POPULATION: A total of 838 265 singleton liveborn babies delivered in Denmark between 1997 and 2010...

  11. [Cystic adenomatoid malformation of the lung. Importance of prenatal diagnosis].

    Science.gov (United States)

    Cabeza, Beatriz; Oñoro, Gonzalo; Cantarín Extremera, Verónica; Sanz Santiago, Verónica; Sequeiros, Adolfo

    2011-04-01

    Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.

  12. Amphibian malformation monitoring Modoc National Wildlife Refuge January 18 2005

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — This document reports the preliminary findings associated with routine sampling efforts to determine the prevalence of frog malformation at Modoc National Wildlife...

  13. Chiari-I malformation in two fighter pilots.

    Science.gov (United States)

    Akin, Ahmet; Canakci, Zafer; Sen, Ahmet; Tore, Hasan F

    2003-07-01

    This report describes two cases of Chiari Malformation Type I (Chiari-I) in fighter pilots of the Turkish Air Force. Chiari-I is a congenital malformation characterized by herniation of cerebellar tonsils through the foramen magnum. Patients have symptoms and signs related to dysfunction of the brainstem, spinal cord, and cerebellum. They generally are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years. Symptoms can be provoked by increasing intracranial pressure (Valsalva or straining). We report on two pilots with Chiari-I malformation who had no symptoms or signs in their daily activities. Furthermore, these pilots had successfully completed physiological training, including centrifuge training, without any symptoms. However, they suffered from headache, neck spasms, and/or disequilibrium under +Gz during flight training sorties. The clinical presentation, diagnosis, treatment, possibility of acquired cases, and aeromedical disposition of Chiari malformations are discussed.

  14. Confusion between vascular malformations and hemangiomas-practical issues

    Directory of Open Access Journals (Sweden)

    Anca Chiriac

    2014-04-01

    Full Text Available A lot of confusion exists in daily practice regarding the terminology of vascular anomaly diagnosed in infants! Hemangioma is a vascular tumor and it is NOT a vascular malformation!

  15. Posterior fossa malformations: main features and limits in prenatal diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Department of Radiology, Paris (France)

    2010-06-15

    Posterior fossa (PF) malformations are commonly observed during prenatal screening. Their understanding requires knowledge of the main steps of PF development and knowledge of normal patterns in US and MR imaging. The vast majority of PF malformations can be strongly suspected by acquiring a midline sagittal slice and a transverse slice and by systematically scrutinizing the elements of the PF: cerebellar vermis, hemispheres, brainstem, fourth ventricle, PF fluid spaces and tentorium. Analysis of cerebellar echogenicity and biometry is also useful. This review explains how to approach the diagnosis of the main PF malformations by performing these two slices and answering six key questions about the elements of the PF. The main imaging characteristics of PF malformations are also reviewed. (orig.)

  16. Rare anorectal malformation with a non-terminal colovesical fistula

    OpenAIRE

    2015-01-01

    We describe a unique case of anorectal malformation (ARM) with a non-terminal colovesical fistula. While some aspects are similar to the congenital pouch colon (CPC), the differences make it a distinct form.

  17. Rare anorectal malformation with a non-terminal colovesical fistula

    Directory of Open Access Journals (Sweden)

    Sabine Vasseur Maurer

    2015-06-01

    Full Text Available We describe a unique case of anorectal malformation (ARM with a non-terminal colovesical fistula. While some aspects are similar to the congenital pouch colon (CPC, the differences make it a distinct form.

  18. Epidermal Nevus Syndrome Associated with Brain Malformations and Medulloblastoma

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-01-01

    Full Text Available Researchers at Juntendo University and Tokyo Women’s Medical University, Japan; and University of California, San Francisco, Ca, report a male infant with epidermal nevus syndrome associated with brainstem and cerebellar malformations and neonatal medulloblastoma.

  19. Dahomey NWR Malformed Frog Survey Data 2003-2004

    Data.gov (United States)

    US Fish and Wildlife Service, Department of the Interior — Data set contains information concerning surveys for malformed frog collections on Dahomey NWR in MS from 2003-2004. Data were collected as part of the national...

  20. Type I Chiari malformation presenting central sleep apnea.

    Science.gov (United States)

    Kitamura, Takuro; Miyazaki, Soichiro; Kadotani, Hiroshi; Kanemura, Takashi; Okawa, Masako; Tanaka, Toshihiko; Komada, Ichiro; Hatano, Taketo; Suzuki, Hideaki

    2014-04-01

    Sleep apnea is a rare but a well-known clinical feature of type I Chiari malformation. It may be obstructive or central in nature. Sleep apnea in patients with type I Chiari malformation rarely presents without accompanying neurological signs or symptoms. We here report a case of a 10-year-old girl who presented with central sleep apnea without any other neurological signs but was ultimately diagnosed with type I Chiari malformation. The patient initially showed mild improvement in symptoms after administration of an acetazolamide. Finally, posterior fossa decompression dramatically improved her respiratory status during sleep, both clinically and on polysomnography. This case suggests that type I Chiari malformation should be considered in the differential diagnoses of central apneas in children, even if there are no other neurological signs and symptoms. Furthermore, sagittal craniocervical magnetic resonance imaging may be necessary for a definitive diagnosis.

  1. Carotid-cavernous fistula caused by laceration of persistent fetal trigeminal artery treated with single catheter coil embolization

    Directory of Open Access Journals (Sweden)

    Benjamin L Brown

    2012-01-01

    Full Text Available We present the endovascular treatment of traumatic carotid-cavernous fistula from persistent fetal trigeminal artery (PFTA laceration. To date, there are six such cases of traumatic PFTA-cavernous fistulas reported in the literature. These injuries can pose a unique challenge in that rupture of a PFTA in its course through the cavernous sinus may produce a fistula feeding from both anterior and posterior circulations. Previously, these have been treated with dual catheter coil embolization from the carotid and basilar systems. We utilize a single catheter technique accessing the cavernous sinus through the origin of the PFTA on the internal carotid. Both anterior and posterior fistula components may be embolized through this single access. This represents a simple yet safe treatment option.

  2. Small bowel autotransplantation combined with pancreato-duodenectomy for enormous cavernous hemangioma of the small intestine mesentery

    Institute of Scientific and Technical Information of China (English)

    ZENG Yong; WU Hong; YANG Jia-yin

    2008-01-01

    @@ Recent advances in transplantation techniques have allowed pancreatoduodenectomy, distal gastrectomy, hemicolectomy and small bowel autotransplantation to be the therapy of choice for enormous cavernous hemangioma of the small intestine mesentery. There have been a few case reports about small bowel autotransplantation combined with pancreatoduodenectomy for enormous mesenteric cavernous hemangioma of small intestine.1-4 The present surgical methods for enormous cavernous hemangioma of the small intestine mesentery mainly included tumor excision and/or small bowel resection. However, these therapies are not effective for those patients in whom the angiocavernoma has infiltrated the mesenteric artery or pancreas, and these patients often give up therapy. It is recognized that enormous cavernous hemangioma of the small intestine mesentery is a benign lesion, and patients may have an excellent prognosis after complete resection of the lesion.

  3. Western Portion IKONOS and Landsat ETM Merge Satellite Imagery for Carlsbad Caverns National Park Vegetation Mapping Project

    Data.gov (United States)

    National Park Service, Department of the Interior — IKONOS and Landsat ETM+ image merge for Carlsbad Caverns National Park, New Mexico. The image has a spatial resolution of 12 meters and is comprised of 11 layers....

  4. Dependence of malformation upon gestational age and exposed dose of gamma radiation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Sung-Ho; Lee, Jong-Hwan; Oh, Heon; Kim, Se-Ra [Chonnam National Univ., Kwangju (Korea, Republic of). Coll. of Veterinary Medicine; Lee, Cha-Soo; Jo, Sung-Kee; Kim, Tae-Hwan; Lee, Yun-Sil

    2001-09-01

    In order to evaluate the importance of gestational age and the dose-incidence relationship by gamma radiation, pregnant ICR mice at gestational days from 2.5 to 15.5 days post-coitus (p.c.) were exposed to a single dose of 2.0 Gy and also at day 11.5 after conception, which was the most sensitive stage for the induction of major congenital malformations. The animals were sacrificed on day 18 of gestation and the fetuses were examined for mortality, growth retardation, changes in head size and other morphological abnormalities. The only demonstrable effect of irradiation during the pre-implantation period was an increase in prenatal mortality. Resorptions were maximal on exposure at day 2.5 after conception. The pre-implantation irradiated embryos which survived did not show any major fetal abnormalities. A small head, growth retardation, a cleft palate, dilatation of the cerebral ventricle, a renal pelvis, and abnormalities of the extremities and tail after exposure were prominent during the organogenesis period, especially on day 11.5 of gestation. As for the dose-incidence relationship, the incidence of a small head, growth-retarded fetuses, a cleft palate, dilatation of cerebral ventricle and abnormalities of the extremities in live fetuses rose as the radiation dose increased. The result indicated that the late period of organogenesis in the development of the brain, skull and extremities of a mouse was a particularly sensitive phase. The threshold doses of radiation that induced a cleft palate and dilatation of the cerebral ventricle, and abnormal extremities were between 1.0 and 2.0 Gy, and between 0.5 and 1.0 Gy, respectively. (author)

  5. Revision Stapedectomy in a Female Patient with Inner Ear Malformation

    OpenAIRE

    Patel, Tirth R.; Moberly, Aaron C.

    2016-01-01

    Objectives. We describe an unusual case of surgical management of congenital mixed hearing loss in a female patient with inner ear malformation. This report outlines the role of temporal bone imaging and previous surgical history in evaluating a patient’s risk of perilymph gusher during stapes surgery. Methods. A 68-year-old female patient with a history of profound bilateral mixed hearing loss due to ossicular and cochlear malformation presented to our otology clinic. She had undergone multi...

  6. Computed tomography of the pathological temporal bone. Malformations. Otospongiosis

    Energy Technology Data Exchange (ETDEWEB)

    Azais, O.; Drouineau, J.; Vandermarcq, P.; Barret, D.; Gasquet, C.

    1988-01-01

    The authors evaluate the value of computed tomography in the investigation of malformations and otospongiosis of the ear. Although the CT scan appears to be essential in the preoperative of malformations of the ear, especially as otological examination frequently contributes little to the evaluation of the middle ear, otospongiosis seems to be a less formal indication, except in the predominantly cochlear forms of the disease.

  7. RECTAL DUPLICATION CYST IN PREVIOUS ANORECTAL MALFORMATION AND DOWN SYNDROME

    Directory of Open Access Journals (Sweden)

    A. Burgio

    2012-12-01

    Full Text Available Gastrointestinal (GI tract duplications are rare congenital malformations. Most of them occur in the ileum and only 1-5%, of all duplication, were in the rectum. Different clinical features including chronic constipation, rectal prolapsed or polips. We report on a 4-years-old girl with Down syndrome and anorectal malformation (ARM who was found to have a rectal duplication cyst.

  8. Complex split-cord malformation associated with situs inversus totalis

    Directory of Open Access Journals (Sweden)

    Deepak Agrawal

    2007-01-01

    Full Text Available Although meningoceles are known to be associated with split cord malformations, the association of dextrocardia is extremely rare. The authors report a case of a 15 day male child who had an atretic meningocele in the lumbosacral region along with dextrocardia and a split cord malformation with a posterior spur. This importance of preoperative MRI for proper management of such patients is highlighted in this report.

  9. Treatment of venous malformations (VM): The cutting edge knowledge

    Institute of Scientific and Technical Information of China (English)

    Laurence M. Boon

    2005-01-01

    Venous malformations (VMs) are problematic common vascular malformations that are challenging even for experienced physicians. Several treatments are available including sclerotherapy, surgery, laser or combinations of these procedures. As none of these is specific, we still need therapies that would allow to cure these patients without complications. Hopefully, the unraveling of the causative defects of VMs will give us new tools for the management of this difficult condition.

  10. Failure Analysis of Overhanging Blocks in the Walls of a Gas Storage Salt Cavern: A Case Study

    Science.gov (United States)

    Wang, Tongtao; Yang, Chunhe; Li, Jianjun; Li, Jinlong; Shi, Xilin; Ma, Hongling

    2017-01-01

    Most of the rock salt of China is bedded, in which non-salt layers and rock salt layers alternate. Due to the poor solubility of the non-salt layers, many blocks overhang on the walls of the caverns used for gas storage, constructed by water leaching. These overhanging blocks may collapse at any time, which may damage the tubing and casing string, and even cause instability of the cavern. They are one of the main factors threatening the safety of caverns excavated in bedded rock salt formations. In this paper, a geomechanical model of the JJKK-D salt cavern, located in Jintan salt district, Jintan city, Jiangsu province, China, is established to evaluate the stability of the overhanging blocks on its walls. The characters of the target formation, property parameters of the rock mass, and actual working conditions are considered in the geomechanical model. An index system composed of stress, displacement, plastic zone, safety factor, and equivalent strain is used to predict the collapse length of the overhanging blocks, the moment the collapse will take place, and the main factors causing the collapse. The sonar survey data of the JJKK-D salt cavern are used to verify the reliability and accuracy of the proposed geomechanical model. The results show that the proposed geomechanical model has a good reliability and accuracy, and can be used for the collapse prediction of the overhanging blocks on the wall of the JJKK-D salt cavern. The collapse length of the overhanging block is about 8 m. We conclude that the collapse takes place during the debrining. The reason behind the collapse is the sudden decrease of the fluid density, leading to the increase of the self-weight of the overhanging blocks. This study provides a basis for the collapse prediction method of the overhanging blocks of Jintan salt cavern gas storage, and can also serve as a reference for salt cavern gas storage with similar conditions to deal with overhanging blocks.

  11. Combined Spinal-Epidural Analgesia for Laboring Parturient with Arnold-Chiari Type I Malformation: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Clark K. Choi

    2013-01-01

    Full Text Available Anesthetic management of laboring parturients with Arnold-Chiari type I malformation poses a difficult challenge for the anesthesiologist. The increase in intracranial pressure during uterine contractions, coughing, valsalva maneuvers, and expulsion of the fetus can be detrimental to the mother during the process of labor and delivery. No concrete evidence has implicated high cerebral spinal fluid pressure on maternal and fetal complications. The literature on the use of neuraxial techniques for managing parturients with Arnold-Chiari is extremely scarce. While most anesthesiologists advocate epidural analgesia for management of labor pain and spinal anesthesia for cesarean section, we are the first to report the use of combined spinal-epidural analgesia for managing labor pain in a pregnant woman with Arnold-Chiari type I malformation. Also, we have reviewed the literature and presented information from case reports and case series to support the safe usage of neuraxial techniques in these patients.

  12. Research perspectives in the etiology of congenital anorectal malformations using data of the International Consortium on Anorectal Malformations: evidence for risk factors across different populations.

    NARCIS (Netherlands)

    Wijers, C.H.W.; Blaauw, I. de; Marcelis, C.L.M.; Wijnen, R.M.H.; Brunner, H.G.; Midrio, P.; Gamba, P.; Clementi, M.; Jenetzky, E.; Zwink, N.; Reutter, H.; Bartels, E.; Grasshoff-Derr, S.; Holland-Cunz, S.; Hosie, S.; Marzheuser, S.; Schmiedeke, E.; Cretolle, C.; Sarnacki, S.; Levitt, M.A.; Knoers, N.V.A.M.; Roeleveld, N.; Rooij, I.A.L.M. van

    2010-01-01

    PURPOSE: The recently established International Consortium on Anorectal Malformations aims to identify genetic and environmental risk factors in the etiology of syndromic and nonsyndromic anorectal malformations (ARM) by promoting collaboration through data sharing and combined research activities.

  13. Research perspectives in the etiology of congenital anorectal malformations using data of the International Consortium on Anorectal Malformations: evidence for risk factors across different populations

    NARCIS (Netherlands)

    C.H.W. Wijers (Charlotte); I. de Blaauw (Ivo); C.L.M. Marcellis; R.M.H. Wijnen (René); H. Brunner (Han); P. Midrio (Paola); P. Gamba (Piergiorgio); M. Clementi (Maurizio); E. Jenetzky (Ekkehart); N. Zwink (Nadine); H. Reutter (Heiko); E. Bartels (Enrika); S. Grasshoff-Derr (Sabine); S. Holland-Cunz (Stefan); S. Hosie (Stuart); S. Märzheuser (Stefanie); E. Schmiedeke (Eberhard); C. Crétolle (Célia); S. Sarnacki (Sabine); M.A. Levitt (Marc); N.V.A.M. Knoers (Nine); N. Roeleveld (Nel); I.A.L.M. Rooij (Iris)

    2010-01-01

    textabstractPurpose: The recently established International Consortium on Anorectal Malformations aims to identify genetic and environmental risk factors in the etiology of syndromic and nonsyndromic anorectal malformations (ARM) by promoting collaboration through data sharing and combined research

  14. Diffusion tensor imaging of midline posterior fossa malformations

    Energy Technology Data Exchange (ETDEWEB)

    Widjaja, Elysa; Blaser, Susan; Raybaud, Charles [Hospital for Sick Children, Diagnostic Imaging, Toronto, ON M5G 1X8 (Canada)

    2006-06-15

    Diffusion tensor imaging and tractography have been used to evaluate a variety of brain malformations. However, these studies have focused mainly on malformations involving the supratentorial compartments. There is a paucity of data on diffusion tensor imaging of posterior fossa malformations. To describe the color vector maps and modified or abnormal tracts of midline posterior fossa malformations. Diffusion tensor imaging was performed in one patient with rhombencephalosynapsis and two with Joubert syndrome. Color vector maps of fractional anisotropy were used to place a region of interest for seed point of fiber tracking. The vermis was severely hypoplastic or absent in rhombencephalosynapsis and Joubert syndrome. In rhombencephalosynapsis, vertically oriented fibers were visualized in the midportion of the cerebellum. The location of the deep cerebellar nuclei could be inferred from the amiculum and were medially located in rhombencephalosynapsis. In the two patients with Joubert syndrome, the horizontally arranged superior cerebellar peduncles were well demonstrated on the color vector maps. Failure of the superior cerebellar peduncles to decussate in the mesencephalon was also well demonstrated on both color vector maps and tractography. The deep cerebellar nuclei were more laterally located in Joubert syndrome. The use of tractography in midline posterior fossa malformations expands our understanding of these malformations. (orig.)

  15. Socio- Cultural Variables Of Congenital Malformation In Newborns

    Directory of Open Access Journals (Sweden)

    Khan Zulfia

    1997-01-01

    Full Text Available Research question: Is there an association between common socio â€" cultural variables and congenital malformation? Objectives: To determine the rate of congenital malformation at birth in hospital deliveries and its association with socio- cultural factors. Study design : Cross- sectional. Setting: J.N. Medical College Hospital and Mohanlal Gautam Rajkiya Hahila Chikitsalaya (District hospital, Aligarh. Participants: All newborn babies (including still births delivered in the two hospitals and their mothers. Study variables: Social class, religion, consanguinity of marriage, age of mother, parity, urban rural status, history of viral illness, drug intake and tobacco use during pregnancy. Outcome variables: Congenital malformations at birth. Statistical analysis: Chi- square test. Results: The overall prevalence of congenital malformations was 2.8%, being 1.6% in live births and 15.6% in stillbirths. Social class, consanguinity of marriage, parity, urban or rural status and history of viral illness during pregnancy were significantly associated with the date of congenital malformation. Conclusion: Certain socio- cultural factors are associated with congenital malformations and can be used in screening during ante- natal period.

  16. Update on neuroimaging phenotypes of mid-hindbrain malformations

    Energy Technology Data Exchange (ETDEWEB)

    Jissendi-Tchofo, Patrice [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); CHU Saint-Pierre, Radiology Department, Pediatric Neuroradiology Section, Brussels (Belgium); Severino, Mariasavina [Istituto Giannina Gaslini, Neuroradiology Unit, Genoa (Italy); Nguema-Edzang, Beatrice; Toure, Cisse; Soto Ares, Gustavo [University Hospital of Lille (CHRU), Department of Neuroradiology, MRI 3T Research, Plateforme Imagerie du vivant, IMPRT-IFR 114, Lille-Cedex (France); Barkovich, Anthony James [University of California, Neuroradiology Section, Department of Radiology and Biomedical Imaging, San Francisco, CA (United States)

    2014-10-23

    Neuroimaging techniques including structural magnetic resonance imaging (MRI) and functional positron emission tomography (PET) are useful in categorizing various midbrain-hindbrain (MHB) malformations, both in allowing diagnosis and in helping to understand the developmental processes that were disturbed. Brain imaging phenotypes of numerous malformations are characteristic features that help in guiding the genetic testing in case of direct neuroimaging-genotype correlation or, at least, to differentiate among MHB malformations entities. The present review aims to provide the reader with an update of the use of neuroimaging applications in the fine analysis of MHB malformations, using a comprehensive, recently proposed developmental and genetic classification. We have performed an extensive systematic review of the literature, from the embryology main steps of MHB development through the malformations entities, with regard to their molecular and genetic basis, conventional MRI features, and other neuroimaging characteristics. We discuss disorders in which imaging features are distinctive and how these features reflect the structural and functional impairment of the brain. Recognition of specific MRI phenotypes, including advanced imaging features, is useful to recognize the MHB malformation entities, to suggest genetic investigations, and, eventually, to monitor the disease outcome after supportive therapies. (orig.)

  17. Sincipital Encephaloceles: A Study of Associated Brain Malformations

    Directory of Open Access Journals (Sweden)

    Shashidhar Vedavyas Achar

    2016-01-01

    Full Text Available Objective: The aim of this study was to evaluate the associated intracranial malformations in patients with sincipital encephaloceles. Materials and Methods: A hospital-based cross-sectional study was conducted over 8 years from June 2007 to May 2015 on 28 patients. The patients were evaluated by either computed tomography or magnetic resonance imaging whichever was feasible. Encephaloceles were described with respect to their types, contents, and extensions. A note was made on the associated malformations with sincipital encephaloceles. Results: Fifty percent of the patients presented before the age of 3 years and both the sexes were affected equally. Nasofrontal encephalocele was the most common type seen in 13 patients (46.4%, and corpus callosal agenesis (12 patients was the most common associated malformation. Other malformations noted were arachnoid cyst (10 patients, hydrocephalus (7 patients, and agyria-pachygyria complex (2 patients. Conclusion: Capital Brain malformations are frequently encountered in children with sincipital encephaloceles. Detail radiological evaluation is necessary to plan treatment and also to prognosticate such rare malformations.

  18. Commercial potential of natural gas storage in lined rock caverns (LRC)

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1999-11-01

    The geologic conditions in many regions of the United States will not permit the development of economical high-deliverability gas storage in salt caverns. These regions include the entire Eastern Seaboard; several northern states, notably Minnesota and Wisconsin; many of the Rocky Mountain States; and most of the Pacific Northwest. In late 1997, the United States Department of Energy (USDOE) Federal Energy Technology Center engaged Sofregaz US to investigate the commercialization potential of natural gas storage in Lined Rock Caverns (LRC). Sofregaz US teamed with Gaz de France and Sydkraft, who had formed a consortium, called LRC, to perform the study for the USDOE. Underground storage of natural gas is generally achieved in depleted oil and gas fields, aquifers, and solution-mined salt caverns. These storage technologies require specific geologic conditions. Unlined rock caverns have been used for decades to store hydrocarbons - mostly liquids such as crude oil, butane, and propane. The maximum operating pressure in unlined rock caverns is limited, since the host rock is never entirely impervious. The LRC technology allows a significant increase in the maximum operating pressure over the unlined storage cavern concept, since the gas in storage is completely contained with an impervious liner. The LRC technology has been under development in Sweden by Sydkraft since 1987. The development process has included extensive technical studies, laboratory testing, field tests, and most recently includes a storage facility being constructed in southern Sweden (Skallen). The LRC development effort has shown that the concept is technically and economically viable. The Skallen storage facility will have a rock cover of 115 meters (375 feet), a storage volume of 40,000 cubic meters (250,000 petroleum barrels), and a maximum operating pressure of 20 MPa (2,900 psi). There is a potential for commercialization of the LRC technology in the United States. Two regions were studied

  19. Psychosocial adjustment and craniofacial malformations in childhood.

    Science.gov (United States)

    Pertschuk, M J; Whitaker, L A

    1985-02-01

    Forty-three children between the ages of 6 and 13 years with congenital facial anomalies underwent psychosocial evaluation prior to surgery. Also evaluated were healthy children matched to the craniofacial subjects by sex, age, intelligence, and economic background. Relative to this comparison group, the craniofacial children were found to have poorer self-concept, greater anxiety at the time of evaluation, and more introversion. Parents of the craniofacial children noted more frequent negative social encounters for their children and more hyperactive behavior at home. Teachers reported more problematic classroom behavior. Examination of these results revealed craniofacial malformations to be associated with psychosocial limitations rather than marked deficits. These children tended to function less well than the comparison children, but with few exceptions, they were not functioning in a psychosocially deviant range. Explanations for the observed circumscribed impact of facial deformity include the use of denial as a coping mechanism, possible diminished significance of appearance for younger children, and the restricted environment experienced by most of the subjects. It can be predicted that time would render these protective influences ineffective, so that adolescent and young adult patients could be at far greater psychosocial risk.

  20. Diagnosis and evaluation of intracranial arteriovenous malformations

    Science.gov (United States)

    Conger, Andrew; Kulwin, Charles; Lawton, Michael T.; Cohen-Gadol, Aaron A.

    2015-01-01

    Background: Ideal management of intracranial arteriovenous malformations (AVMs) remains poorly defined. Decisions regarding management of AVMs are based on the expected natural history of the lesion and risk prediction for peritreatment morbidity. Microsurgical resection, stereotactic radiosurgery, and endovascular embolization alone or in combination are all viable treatment options, each with different risks. The authors attempt to clarify the existing literature's understanding of the natural history of intracranial AVMs, and risk-assessment grading scales for each of the three treatment modalities. Methods: The authors conducted a literature review of the existing AVM natural history studies and studies that clarify the utility of existing grading scales available for the assessment of peritreatment risk for all three treatment modalities. Results: The authors systematically outline the diagnosis and evaluation of patients with intracranial AVMs and clarify estimation of the expected natural history and predicted risk of treatment for intracranial AVMs. Conclusion: AVMs are a heterogenous pathology with three different options for treatment. Accurate assessment of risk of observation and risk of treatment is essential for achieving the best outcome for each patient. PMID:25984390