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Arterial venous malformation

Rizwana Yasmin, Dorte R Stærk, Anna Kalhauge, Henrik J Hansen, Tina E Olsen, Lisa L Maroun
Bilateral pulmonary sequestration (PS) is a very rare congenital malformation. We describe a case of bilateral intralobar pulmonary sequestration (ILS) in a newborn. Both sequestrations received arterial supply from separate branches of the descending aorta and venous drainage was into ipsilateral inferior pulmonary veins. Prenatal ultrasonography showed cystic changes in the lungs. Computed tomography angiography (CTA) with supplemental two-dimensional (2D) and three-dimensional (3D) images was performed to clearly define the pathology and revealed bilateral intralobar pulmonary sequestration with aberrant blood supply...
March 2018: Acta Radiologica Open
Clara Elisa Frare de Avelar Teixeira, Angélica de Fátima de Assunção Braga, Franklin Sarmento da Silva Braga, Vanessa Henriques Carvalho, Rafael Miranda da Costa, Giselle Ioná Teixeira Brighenti
INTRODUCTION: Klippel-Trenaunay syndrome is a rare congenital vascular disease characterized by cutaneous hemangiomas, varicosities, and limb asymmetry, which may evolve with coagulation disorders and hemorrhage as more frequent complications in pregnant patients. Pregnancy is not advised in women with this syndrome due to increased obstetric risk. CASE REPORT: Female patient, 29 years old, 99kg, 167cm, BMI 35.4kg.m-2 , physical status ASA III, with 27 weeks of gestational age and diagnosis of Klippel-Trenaunay syndrome...
March 2, 2018: Revista Brasileira de Anestesiologia
Arkadiusz Krzyżanowski, Dariusz Swatowski, Tomasz Gęca, Maciej Kwiatek, Aleksandra Stupak, Sławomir Woźniak, Anna Kwaśniewska
BACKGROUND: Persistent right umbilical vein (PRUV) is usually an isolated finding but it may be accompanied by other fetal malformations. AIMS: We aimed to determine the incidence of prenatally diagnosed PRUV in a referral population, assess the neonatal outcome and discuss the findings together with those from previous publications. MATERIALS AND METHODS: A total of 2360 women with low-risk singleton pregnancies were examined in the second and third trimesters...
March 2, 2018: Australian & New Zealand Journal of Obstetrics & Gynaecology
Jennifer Susanne Esser, Rahel Elisabeth Steiner, Meike Deckler, Hannah Schmitt, Bianca Engert, Sandra Link, Anne Charlet, Cam Patterson, Christoph Bode, Qian Zhou, Martin Moser
The bone morphogenetic protein (BMP) signaling pathway plays a central role during vasculature development. Mutations or dysregulation of the BMP pathway members have been linked to arteriovenous malformations. In the present study, we investigated the effect of the BMP modulators BMPER and twisted gastrulation protein homolog 1 (TWSG1) on arteriovenous specification during zebrafish development and analyzed downstream Notch signaling pathway in human endothelial cells. Silencing of bmper and twsg1b in zebrafish embryos by morpholinos resulted in a pronounced enhancement of venous ephrinB4a marker expression and concomitant dysregulated arterial ephrinb2a marker expression detected by in situ hybridization...
February 23, 2018: FEBS Journal
Xiang Zou, Zehan Wu, Jun Huang, Peixi Liu, Xuanfeng Qin, Liang Chen, Wei Zhu, Yao Zhao, Peiliang Li, Jianping Song, Guo-Yuan Yang, Ying Mao
BACKGROUND: The molecular mechanism of brain arteriovenous malformation (BAVM) is largely unknown. Intracranial venous hypertension (VH) may enhance focal angiogenesis and promote BAVM development and progression. A rat VH model effectively simulates the hemodynamic microenvironment of this disease. OBJECTIVE: To explore the effect of doxycycline in VH-related angiogenesis, as well as the role of matrix metalloproteinase-3 (MMP-3) and other molecular factors. METHODS: A rat VH model was generated by common carotid artery and distal external jugular vein anastomosis...
February 16, 2018: Neurosurgery
Yutaka Tomizawa, Akiko Tanaka, Hiroto Kitahara, Atsushi Sakuraba, Nir Uriel, Valluvan Jeevanandam, Takeyoshi Ota
BACKGROUND: Large scale data on preoperative risk stratification for gastrointestinal bleeding (GIB) following continuous-flow left ventricular assist device (CF-LVAD) implantation are scarce. AIMS: To identify independent predictors for GIB following CF-LVAD implantation. METHODS: We conducted a retrospective cohort study of consecutive 243 patients who underwent CF-LVAD implantation (HeartMateII) from January 2009 to March 2015 at the University of Chicago Medical Center...
February 8, 2018: Digestive Diseases and Sciences
Zsolt Kulcsár, Paolo Machi, Karl Schaller, Karl Olof Lovblad, Philippe Bijlenga
Treatment of ruptured deep-seated arteriovenous malformations is challenging and associated with elevated risks. This is due to the proximity or involvement of critical brain structures and the specifically fine and delicate angioarchitecture of these lesions, making both endovascular and surgical access technically complicated. We present the advantages of a true combined, open surgical and endovascular transvenous approach in a hybrid operating room. The technique may overcome in part the difficulties and may improve safety and risk related concerns...
February 1, 2018: Journal of Neuroradiology. Journal de Neuroradiologie
Arthur Hosmann, Ahmed El-Garci, Brigitte Gatterbauer, Gerhard Bavinzski, Engelbert Knosp, Andreas Gruber
BACKGROUND: The Vein of Galen Aneurysmal Malformation (VGM) is a rare intracranial arterio-venous fistula with a dramatic manifestation during infancy and a 100% mortality without treatment. Therapeutic strategies for VGMs have changed over time due to advances in endovascular techniques. We present our experience and multimodality approach within the last 4 decades. METHODS: Retrospective analysis and angiographic review of VGM patients between 1975 and 2016 at our institution...
February 2, 2018: World Neurosurgery
Miriam Kuhn, Andreas Hornung, Heidi Ulmer, Christian Schlensak, Michael Hofbeck, Gesa Wiegand
There are many complex cardiac malformations that are characterized by a functionally univentricular physiology. Staged surgical repair according to the Fontan principle separates the systemic and pulmonary circulations by connecting the systemic venous return to the pulmonary arteries. However, long-term follow-up studies demonstrate a gradual deterioration of cardiac function, particularly from the second or third decade. Noninvasive evaluation of the cardiac function is, therefore, important in the follow-up of these patients...
February 3, 2018: Pediatric Cardiology
Rupinder Singh, Vivek Gupta, Chirag Ahuja, Ajay Kumar, Kanchan K Mukherjee, Niranjan Khandelwal
Introduction The present study aimed to evaluate the accuracy of time-resolved-computed tomographic angiography (TR-CTA) on a 128-slice CT scanner vis-à-vis cerebral digital subtraction angiography (DSA) in defining the morphological and haemodynamic characteristics of cerebral arteriovenous malformation (AVM). Methods Twenty-one patients (age range 10-46, mean 24.8 years) with clinical suspicion of AVM and three patients (age range 23-35, mean 24.3 years) with diagnosed AVM who were on follow-up underwent DSA and TR-CTA, on average 1...
January 1, 2018: Neuroradiology Journal
Kiyoshi Uike, Hazumu Nagata, Yuichiro Hirata, Kenichiro Yamamura, Eiko Terashi, Toshiharu Matsuura, Eiji Morihana, Kazuhiro Ohkubo, Kanako Ishii, Yasunari Sakai, Tomoaki Taguchi, Shouichi Ohga
OBJECTIVE: Congenital portosystemic venous shunt (CPSVS) is a rare vascular malformation with a high risk of mortality from pulmonary arterial hypertension (PAH), but the treatment outcome of CPSVS closure remains elusive. Our aim was to investigate the clinical features and establish the optimal management of CPSVS with or without PAH. METHODS: Twenty-four patients with CPSVS treated in Kyushu University Hospital between 1990 and 2015 were enrolled in this study...
January 23, 2018: Pediatric Pulmonology
Masashi Shimohira, Tatsuya Kawai, Takuya Hashizume, Masahiro Muto, Masanori Kitase, Yuta Shibamoto
PURPOSE: To evaluate the usefulness of hydrogel-coated coils for preventing recanalization after coil embolization of pulmonary arteriovenous malformations (PAVMs). MATERIALS AND METHODS: Thirty-seven consecutive patients with 57 untreated PAVMs underwent coil embolization with hydrogel-coated coils between January 2013 and Jun 2017. The mean age was 49 years (range 9-83 years), and there were seven male patients and 30 female patients. The median size of the feeding artery was 3...
January 17, 2018: Cardiovascular and Interventional Radiology
Jay F Yu, Andrew D Nicholson, Jeffrey Nelson, Matthew D Alexander, Stephanie H Tse, Steven W Hetts, J Claude Hemphill, Helen Kim, Daniel L Cooke
Background and purpose Despite evidence regarding risk factors for brain arteriovenous malformation (bAVM)-associated spontaneous intracranial hemorrhage (ICH), few data exist describing the spectrum of clinical outcomes that bAVM-associated ICH may manifest. This study aimed to identify the demographical, clinical, and bAVM anatomical variables associated with ICH volume and the presence of intraventricular hemorrhage (IVH) of ruptured bAVMs, two indicators of worse clinical outcome, to help better predict outcome for unruptured bAVMs...
January 1, 2018: Interventional Neuroradiology
Hiroko Konno, Takeshi Murakoshi, Akiko Yamashita, Mitsuru Matsushita
AIM: We evaluated risk factors for birthweight discordance in monochorionic diamniotic (MCDA) twin pregnancies without twin-twin transfusion syndrome (TTTS). METHODS: We investigated all MCDA twin placentas injected with colored dye at our institution between 2007 and 2015. We excluded pairs of twins with TTTS, fetal demise, or severe fetal malformation. All pairs of twins were assigned to the discordant group (birthweight discordance ≥ 25%) or the concordant group (birthweight discordance < 25%)...
January 5, 2018: Journal of Obstetrics and Gynaecology Research
Ethan A Winkler, Harjus Birk, Jan-Karl Burkhardt, Xiaolin Chen, John K Yue, Diana Guo, W Caleb Rutledge, George F Lasker, Carlene Partow, Tarik Tihan, Edward F Chang, Hua Su, Helen Kim, Brian P Walcott, Michael T Lawton
OBJECTIVE Brain arteriovenous malformations (bAVMs) are rupture-prone tangles of blood vessels with direct shunting of blood flow between arterial and venous circulations. The molecular and/or cellular mechanisms contributing to bAVM pathogenesis and/or destabilization in sporadic lesions have remained elusive. Initial insights into AVM formation have been gained through models of genetic AVM syndromes. And while many studies have focused on endothelial cells, the contributions of other vascular cell types have yet to be systematically studied...
January 5, 2018: Journal of Neurosurgery
Srinivasa R Chandra, Eleanor Chen, Timothee Cousin, Dolphine Oda
Background: Hemangiomas of the soft tissue are common in the head and neck area, especially in the tongue and in children under ten years of age. Intraosseous hemangiomas of the mandible and maxilla (IHM), on the other hand, are exceedingly rare and are not well characterized. This study presents six IHM cases focusing on the clinical, radiographic, and histologic characteristics. Material and Methods: Six cases of IHM were retrieved from the archives of the Biopsy Services at the University of Washington...
November 2017: Journal of Clinical and Experimental Dentistry
Ramesh Bettaiah, Shwetha Sudhakar Kamath
No abstract text is available yet for this article.
December 28, 2017: Journal of Minimally Invasive Gynecology
E Giordan, C Bortolotti, G Lanzino, W Brinjikji
BACKGROUND AND PURPOSE: Neural tube defects, such as tethered cord, intradural lipoma, or myelomeningocele may coexist with spinal vascular malformations. The coexistence of these 2 rare entities is suggestive of a causal relationship between them, which may lead to further understanding of their pathogenesis. We present a series of 6 patients with epidural spinal arteriovenous fistulas associated with neural tube defects. MATERIALS AND METHODS: We retrieved cases of spinal vascular malformations associated with neural tube defects seen at our institution...
December 28, 2017: AJNR. American Journal of Neuroradiology
Bethany L Wisotzkey, Dari L Magyar, Thomas K Jones, Robert J Boucek, Lester C Permut, Mariska S Kemna, Yuk M Law
BACKGROUND: In single ventricle patients, aortopulmonary collaterals (APCs) and pulmonary arteriovenous malformations (PAVMs) following superior cavopulmonary shunt (CPS) can complicate orthotopic heart transplant (OHT) by cyanosis and hemoptysis. Although PAVMs can regress with the restoration of hepatic venous flow to the pulmonary circulation, the effects of hypoxemia on the "unconditioned" allograft are not known. CASES: Two patients with significant PAVMs after CPS were cyanotic following OHT...
February 2018: Clinical Transplantation
Marco Fresa, Andreas Erdmann, Oumama El Ezzi, Anthony De Buys, Michele Depairon, Amin Dabiri, Barbara Ney, Lucia Mazzolai
Venous malformations are slow flow dysplastic lesions, constituted by a vascular nest without arterial or capillary connections, more or less independent of the normal venous anatomy and circulation. In certain cases a treatment is required for symptom relief or for natural complications management. The percutaneous sclerotherapy under ultrasound guidance is increasingly used as an effective and mini-invasive option, allowing obtaining very good results with minor side effects. Several substances have been used with different efficacy and side effects rate...
December 6, 2017: Revue Médicale Suisse
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