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Venous malformation

Juanjuan Zhao, Mathilda T M Mommersteeg
The Slit ligands and their Robo receptors are well-known for their roles during axon guidance in the central nervous system, but are still relatively unknown in the cardiac field. However, data from different animal models suggest a broad involvement of the pathway in many aspects of heart development, from cardiac cell migration and alignment, lumen formation, chamber formation, to the formation of the ventricular septum, semilunar and atrioventricular valves, caval veins and pericardium. Absence of one or more of the genes in the pathway results in defects ranging from bicuspid aortic valves to ventricular septal defects and abnormal venous connections to the heart...
March 10, 2018: Cardiovascular Research
Anna Cavigelli-Brunner, Maja I Hug, Hitendu Dave, Oskar Baenziger, Christoph Buerki, Dominique Bettex, Vincenzo Cannizzaro, Christian Balmer
OBJECTIVES: Dobutamine and milrinone are commonly used after open-heart surgery to prevent or treat low cardiac output syndrome. We sought to compare efficacy and safety of these drugs in pediatric patients. DESIGN: Prospective, single-center, double-blinded, randomized clinical pilot study. SETTING: Tertiary-care university children's hospital postoperative pediatric cardiac ICU. PATIENTS: After written consent, 50 consecutive patients (age, 0...
March 13, 2018: Pediatric Critical Care Medicine
F Wang, D N Feng, Y Liu, Z Liu, T Sun
Objective: To evaluate the efficacy and safety of microsurgical in the treatment of temporal lobe cerebral cavernous malformation (CCM) with epilepsy. Methods: Temporal lobe CCM patients with epilepsy admitted to our department were collected from January 2010 to September 2016. Locations of the CCM were divided into (1) lateral-lateral to the collateral sulcus; (2) mesial-mesial to the collateral sulcus. In the lateral group, patients were underwent intraoperative electrocorticography (ECoG)-guided resection of lesion and hemosiderin rim...
March 6, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Alexandra Yannoutsos, Julie Malloizel-Delaunay, Emilie Tournier, Abdelaziz Abid, Alessandra Bura-Rivière
In this observation, we report an unusual presentation of a pilomatricoma in an 8-year-old girl who was initially referred to the department of vascular medicine for diagnosis and care of a suspected mixed lymphatic venous malformation. The lesion on her left shoulder presented as a giant bluish-purple red solitary mass, painful and rapidly growing, measuring 7 cm in anteroposterior diameter. This mass did not present the typical characteristics of a lymphatic venous malformation but exhibited warning signs of malignancy on clinical examination and imaging...
February 27, 2018: American Journal of Dermatopathology
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
Reid A Maclellan, Jeremy A Goss, Arin K Greene
Vascular malformations enlarge overtime, particularly during adolescence when follicle-stimulating hormone (FSH) rises. Lesions contain the receptor for follicle-stimulating hormone. FSH also becomes elevated during menopause. We present a patient with a venous malformation of the lip that presented for the first time after she entered menopause which was temporally related to a significant increase in her serum FSH levels that were measured. This observation supports the hypothesis that FSH might influence the pathophysiology of vascular malformations...
March 8, 2018: Journal of Craniofacial Surgery
Marta Ivars, Ana Martin-Santiago, Eulalia Baselga, Laurent Guibaud, Juan Carlos López-Gutiérrez
Blue rubber bleb nevus syndrome (BRBNS) is a rare congenital vascular disease associated with important morbidity and non-insignificant risk of mortality in cases of severe gastrointestinal or neurological involvement. Early diagnosis in the neonatal period can be difficult as very often skin lesions appear progressively during childhood having no correlation with gastrointestinal lesion development. The appearance of one large venous malformation (VM) in the neonatal period has suggested a characteristic finding of this syndrome...
March 8, 2018: European Journal of Pediatrics
Federico Scorletti, Manish N Patel, Adrienne M Hammill, Kiersten W Ricci, Charles M Myer, Roshni Dasgupta
BACKGROUND: Vascular malformations isolated to skeletal muscles are rare and often debilitating due to pain and very challenging to treat. Multi-modal management options include compression garments, medical therapy, sclerotherapy, and surgical resection. METHODS: A retrospective review of patients who underwent sclerotherapy for intramuscular venous malformations (IVM) between 2008 and 2016 was performed. Demographics, indications, and clinical follow-up were analyzed...
February 9, 2018: Journal of Pediatric Surgery
Ali Mohtashami, Andrew Kiat, Jane Cross, Robert Simon, Austin Curtin
INTRODUCTION: Abernethy malformations are extremely rare congenital anomalous portosystemic shunts. We report the case of a patient with a rare variant Abernethy malformation between the superior mesenteric vein and left renal vein, associated with a massive jejunal diverticulum. PRESENTATION OF CASE: A 37-year-old Caucasian female presented to our emergency department with severe abdominal pain and proceeded to laparotomy for a presumed small bowel obstruction...
February 27, 2018: International Journal of Surgery Case Reports
Zhong Du, Hai-Long Ma, Zhi-Yuan Zhang, Jia-Wei Zheng, Yan-An Wang
A TIE2 mutation causing arginine-to-tryptophan substitution at residue 849 ( TIE2-R849W ) is commonly identified in heredofamilial venous malformation. However, there is no in vivo model to confirm the pathogenic role of TIE2-R849W . Humanized TIE2-R849W plasmid was constructed via PCR-mediated site-directed mutagenesis. After transcription and micro-injection, TIE2-R849W significantly induces multiple malformations in zebrafish: caudal vein plexus (CVP) defect, eye abnormalities, forebrain formation perturbations, and mandibular malformation...
2018: International Journal of Medical Sciences
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
Tarun Raina Ramman, Nilanjan Dutta, Kuntal Roy Chowdhuri, Sunny Agrawal, Sumir Girotra, Sushil Azad, Sitaraman Radhakrishnan, Parvathi Unninayar Iyer, Krishna Subramony Iyer
Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects...
January 1, 2018: World Journal for Pediatric & Congenital Heart Surgery
Xue Sun, Wenjia Lei, Yu Wang, Faiza Amber Siddiqui, Ying Zhang
Partial anomalous pulmonary venous connection (PAPVC) is a rare malformation. We describe a case of PAPVC, in which the left pulmonary veins coursed to the left innominate vein through a vertical vein and finally drained into the right superior vena cava; the right pulmonary veins were connected to the left atrium. Tracing the origin and destination of abnormal vessels presented at the three-vessel and trachea view is useful for the diagnosis. Four-dimensional echocardiography with high-definition flow imaging and spatiotemporal image correlation facilitates the identification of the drainage of fetal pulmonary veins, which should be considered as a complementary modality in obstetric ultrasonic examination when cardiac abnormalities are suspected...
March 5, 2018: Echocardiography
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
Sophia F Shakur, Denise Brunozzi, Rahim Ismail, Dilip Pandey, Fady T Charbel, Ali Alaraj
OBJECTIVE: The pathogenesis of venous intimal hyperplasia and venous outflow stenosis associated with cerebral arteriovenous malformation (AVM) draining veins is poorly understood. We sought to determine the relationship between venous stenosis and age. METHODS: All patients with an AVM seen at our institution between 1990-2016 who underwent a diagnostic cerebral angiogram were retrospectively reviewed. Draining vein stenosis was measured from angiograms prior to any treatment...
February 26, 2018: World Neurosurgery
Francesco Pichi, K Bailey Freund, Antonio Ciardella, Mariachiara Morara, Emad B Abboud, Nicola Ghazi, Christine Dackiw, Netan Choudhry, Eduardo Cunha Souza, Leonardo Provetti Cunha, J Fernando Arevalo, T Y Alvin Liu, Adam Wenick, Lingmin He, Guadalupe Villarreal, Piergiorgio Neri, David Sarraf
Importance: Congenital retinal macrovessel (CRM) is a rarely reported venous malformation of the retina that is associated with venous anomalies of the brain. Objective: To study the multimodal imaging findings of a series of eyes with congenital retinal macrovessel and describe the systemic associations. Design, Setting, and Participants: In this cross-sectional multicenter study, medical records were retrospectively reviewed from 7 different retina clinics worldwide over a 10-year period (2007-2017)...
March 1, 2018: JAMA Ophthalmology
Nicholas J Volpe, Lee M Jampol
No abstract text is available yet for this article.
March 1, 2018: JAMA Ophthalmology
Kristina R Asdahl, Lene Hedelund, Johnny Keller, Thomas Baad-Hansen, Tine Damsgaard
OBJECTIVE: Sclerotherapy has become an important treatment option in the management of vascular malformations. However, little is known about success rate for treatment of venous malformations. This systematic review assesses the available published literature on outcome measures of sclerotherapy for venous malformations. DATA SOURCE: PubMed and EMBASE. REVIEW METHOD: A systematic search was conducted, and studies from March 2008 to October 2016 were included...
February 28, 2018: Cardiovascular and Interventional Radiology
Sumanta Kumar Kolay, Rajkumar Parwani, Sangeeta Wanjari, Pallav Singhal
Lymphangiomas are benign malformations that represent hamartoma of malformed lymphatics and are composed of cystically dilated lymphatic channels which do not communicate or drain into other lymphatic channels or veins leading to accumulation of lymph. They are congenital malformations with rare occurrence in oral cavity within which tongue dorsum is the most affected site. Venous malformations are often difficult to differentiate from lymphatic malformations leading to the development of several lymphatic markers like D2-40 which seems to be useful for distinguishing vascular from lymphatic vessels...
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
Hirofumi Obinata, Shinichi Nishibe, Yoko Ishihara
Background: Heterotaxy syndrome (HS) is characterized by a wide variety of cardiac and extra-cardiac malformations, including pulmonary valve stenosis, interruption of the inferior vena cava, total anomalous pulmonary venous connection (TAPVC), asplenia, polysplenia, intestinal malrotation, and preduodenal portal vein (PDPV). We report the case of a heterotaxic infant with an infracardiac TAPVC and preduodenal portal vein who experienced repetitive hemodynamic instability during urgent laparotomy for duodenal obstruction...
2018: JA Clin Rep
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