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https://www.readbyqxmd.com/read/29352588/pulmonary-reinterventions-after-complete-unifocalization-and-repair-in-infants-and-young-children-with-tetralogy-of-fallot-with-major-aortopulmonary-collaterals
#1
Holly Bauser-Heaton, Alejandro Borquez, Ritu Asija, Lisa Wise-Faberowski, Yulin Zhang, Laura Downey, Stanton B Perry, Andrew Koth, Lynn F Peng, Claudia A Algaze, Frank L Hanley, Doff B McElhinney
BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit...
December 13, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29343922/periureteral-inferior-vena-caval-venous-ring-presenting-as-urinary-obstruction
#2
Suprava Naik, Arashdeep Singh, Sudipta Mohakud, Nerbadyswari Deep
The embryological development of the inferior vena cava (IVC) is complex, and thus the vena cava may undergo a large number of congenital anomalies. Periureteric venous ring is a rare developmental anomaly of IVC where the right ureter passes through a slit-like opening in a partially duplicated infrarenal IVC, resulting in dilatation of upper urinary tract. Split-bolus multidetector computed tomography technique is useful in detecting such vascular anomaly causing ureteric obstruction as it can clearly show the vascular and ureteric phase in a single acquisition...
January 2018: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/29343896/management-of-vascular-anomalies-review-of-institutional-management-algorithm
#3
Lalit K Makhija, Sameek Bhattacharya
Introduction: Vascular anomalies are congenital lesions broadly categorised into vascular tumour (haemangiomas) and vascular dysmorphogenesis (vascular malformation). The management of these difficult problems has lately been simplified by the biological classification and multidisciplinary approach. To standardise the treatment protocol, an algorithm has been devised. The study aims to validate the algorithm in terms of its utility and presents our experience in managing vascular anomalies...
May 2017: Indian Journal of Plastic Surgery: Official Publication of the Association of Plastic Surgeons of India
https://www.readbyqxmd.com/read/29336902/surgical-management-of-pulsatile-tinnitus-secondary-to-jugular-bulb-or-sigmoid-sinus-diverticulum-with-review-of-literature
#4
W X Yeo, S H Xu, T Y Tan, Y M Low, H W Yuen
INTRODUCTION: Jugular bulb and sigmoid sinus anomalies are well-known causes of vascular pulsatile tinnitus. Common anomalies reported in the literature include high-riding and/or dehiscent jugular bulb, and sigmoid sinus dehiscence. However, cases of pulsatile tinnitus due to diverticulosis of the jugular bulb or sigmoid sinus are less commonly encountered, with the best management option yet to be established. In particular, reports on surgical management of pulsatile tinnitus caused by jugular bulb diverticulum have been lacking in the literature...
December 29, 2017: American Journal of Otolaryngology
https://www.readbyqxmd.com/read/29335391/renal-vascularization-anomalies-in-the-polish-population
#5
Henryk Sośnik, Katarzyna Sośnik
The aim of the study was to determine the incidence of renal venous system congenital anomalies in the Polish population. MATERIAL AND METHOD: Vascular kidney samples were investigated by means of preparations and X-ray contrasting. The study the group comprised 281 male and 269 female specimens. RESULTS: Congenital anomalies were diagnosed in 186 patients (33 8% of all cases), and they were more frequent in men than in women, albeit that difference was non-significant...
December 30, 2017: Polski Przeglad Chirurgiczny
https://www.readbyqxmd.com/read/29334594/thoracic-aortic-aneurysm-in-patients-with-loss-of-function-filamin-a-mutations-clinical-characterization-genetics-and-recommendations
#6
Ming Hui Chen, Sangita Choudhury, Mami Hirata, Siri Khalsa, Bernard Chang, Christopher A Walsh
The frequency and gender distribution of thoracic aortic aneurysm as a cardiovascular manifestation of loss-of-function (LOF) X-linked FilaminA (FLNA) mutations are not known. Furthermore, there is very limited cardiovascular morbidity or mortality data in children and adults. We analyzed cardiac data on the largest series of 114 patients with LOF FLNA mutations, both children and adults, with periventricular nodular heterotopia (PVNH), including 48 study patients and 66 literature patients, median age of 22...
February 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29329829/effectiveness-and-safety-of-foam-sclerotherapy-with-5-ethanolamine-oleate-in-the-treatment-of-low-flow-venous-malformations-in-the-head-and-neck-region-a-case-series
#7
M C Ribeiro, S de Mattos Camargo Grossmann, M B F do Amaral, W H de Castro, T P Navarro, R J Procopio, T A da Silva, C de Nazaré Alves de Oliveira Kato, R A Mesquita
The aim of this study was to evaluate the effectiveness and safety of 5% ethanolamine oleate (EO) foam in the treatment of low-flow venous malformations in the head and neck region. Seventeen consecutive patients (six male, 11 female) and 34 low-flow venous malformations were enrolled. The vascular anomalies ranged between 20mm and 80mm in size. The typical clinical indication was a swelling (88.2%) with a purple colour (85.3%); the most frequent location was the tongue (23.5%). Ethanolamine oleate foam was produced via the Tessari method and applied at 10mg per 1cm to the vascular anomalies...
January 9, 2018: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29324624/intravitreal-aflibercept-in-the-treatment-of-polypoidal-choroidal-vasculopathy-associated-with-morning-glory-syndrome
#8
Claudio Iovino, Maurizio Fossarello, Enrico Peiretti
PURPOSE: To describe an unusual case of polypoidal choroidal vasculopathy secondary to morning glory syndrome successfully treated with three aflibercept intravitreal injections. METHODS: Case report. RESULTS: A 68-year-old white man presented with a 2-month history of diminished vision of his left eye. Fundus examination showed a morning glory syndrome disk anomaly with some perimacular subretinal hemorrhages and lipid depositions. Fundus autofluorescence, fluorescein and green indocyanine angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography were performed and confirmed the presence of a juxtapapillary polypoidal choroidal vasculopathy with intraretinal and subretinal fluid...
January 10, 2018: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/29318367/-vascular-anomalies-part%C3%A2-ii-vascular-malformations
#9
S Mylonas, S Brunkwall, J Brunkwall
Vascular malformations are congenital vascular anomalies with normal endothelial turnover, which can affect the whole vessel tree (arterial, capillary or lymphatic) both as isolated defects or as part of a syndrome. Developmental errors during embryogenesis, such as abnormal signaling processes that control the apoptosis, maturation and growth of vascular cells, lead to the persistence of vascular plexus cells with some degree of differentiation. Vascular malformations are usually present at birth, although symptoms or complications may be manifested later...
January 9, 2018: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/29318090/c1-2-fixation-approach-for-patients-with-vascular-irregularities-a-case-report
#10
Alireza K Nazemi, Stetson R Bickley, Caleb J Behrend, Jonathan J Carmouche
In posterior spinal fusion (PSF), the vertebral artery is most vulnerable to injury at C1-2. C2 pedicle screws are often placed into the dorsomedial isthmus of C2. Alternative techniques include C2 laminar screws and wiring techniques. A 67-year-old male underwent PSF for persistent severe intractable neck pain and degeneration at C1-2. The patient had an enlarged left vertebral artery with midline migration into the C2 body. This pattern was within one standard deviation of normal; however, it rendered typical placement of a C2 pedicle screw unsafe...
December 2017: Geriatric Orthopaedic Surgery & Rehabilitation
https://www.readbyqxmd.com/read/29317980/a-history-of-kawasaki-disease-from-childhood-and-coronary-artery-ectasia-with-recurrent-st-elevation-myocardial-infarction-a-therapeutic-challenge
#11
Ashraf Abugroun, Daniel Vilchez, Osama Hallak, Anahita Shahrrava
Coronary artery ectasia (CAE) is an uncommon anomaly, usually found in 1.2-2% of patients undergoing coronary angiography, defined as a segment of the coronary artery that has a diameter of more than 1.5 times the normal adjacent segments. Atherosclerosis is considered as the cause of half of the CAE cases. We herein present a 65-year-old Asian male with past medical history of Kawasaki disease (KD) who developed recurrent episodes of inferior wall ST-elevation myocardial infarction (STEMI) despite treatment with dual antiplatelet therapy (DAPT)...
December 2017: Cardiology Research
https://www.readbyqxmd.com/read/29310957/revascularization-for-aplastic-or-twiglike-middle-cerebral-artery-a-case-report
#12
Yuki Matsunaga, Tsuyoshi Izumo, Yoichi Morofuji, Nobutaka Horie, Kentaro Hayashi, Takayuki Matsuo
An aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a congenital anomaly related to interference of normal embryonic development of the middle cerebral artery (MCA). This condition may cause ischemic and hemorrhagic strokes as a result of hypoperfusion, vulnerable collateral circulation, and associated aneurysms. We reported a case of Ap/T-MCA with transient ischemic attack, which was successfully treated with revascularization. A 19-year-old woman with a history of transient left upper limb hemiparesis, dysesthesia, and headache visited our hospital...
January 5, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29310402/a-rare-variation-of-celiac-trunk-and-hepatic-artery-complicating-pancreaticoduodenectomy-a-case-report-and-literature-review
#13
Zhou Ye, Song Ye, Dongkai Zhou, Shusen Zheng, Weilin Wang
RATIONALE: Anatomical variations of the celiac trunk and the hepatic artery are of considerable importance in hepatopancreatobiliary surgery, liver transplants, and radiological abdominal interventions. PATIENT CONCERNS: Here, we report a 57-year-old man with 2 weeks of painless progressive jaundice. Preoperative imaging and cytology brush results suggested an ampullary tumor and common hepatic artery anomaly (CTA) was reported. The patient underwent pancreaticoduodenectomy (PD)...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29301791/deletion-of-the-von-hippel-lindau-gene-in-hemangioblasts-causes-hemangioblastoma-like-lesions-in-murine-retina
#14
Herui Wang, Matthew Shepard, Chao Zhang, Lijin Dong, Dyvon T Walker, Liliana Guedez, Stanley Park, Yujuan Wang, Shida Chen, Ying Pang, Qi Zhang, Chun Gao, Wai T Wong, Henry Wiley, Karel Pacak, Emily Y Chew, Zhengping Zhuang, Chi-Chao Chan
von Hippel-Lindau (VHL) disease is an autosomal dominant tumor predisposition syndrome characterized by the development of highly vascularized tumors and cysts. Loss of heterozygosity (LOH) of the VHL gene results in aberrant upregulation of hypoxia-inducible factors (HIF) and has been associated with tumor formation. Hemangioblastomas of the central nervous system and retina represent the most prevalent VHL-associated tumors, but no VHL animal model has reproduced retinal capillary hemangioblastomas (RCH), the hallmark lesion of ocular VHL...
January 4, 2018: Cancer Research
https://www.readbyqxmd.com/read/29300248/fluorescein-angiographic-evaluation-of-peripheral-retinal-vasculature-after-primary-intravitreal-ranibizumab-for-retinopathy-of-prematurity
#15
C Armitage Harper, Lauren M Wright, Ryan C Young, Sarah P Read, Emmanuel Y Chang
PURPOSE: To evaluate angiographic findings in neonates up to 150 weeks postmenstrual age who received intravitreal ranibizumab for primary treatment of Type 1 retinopathy of prematurity. METHODS: Retrospective evaluation of fluorescein angiogram findings was completed for 30 eyes of 16 neonates who received intravitreal ranibizumab as primary treatment for Type 1 retinopathy of prematurity between April 2013 and January 2015. Outcome measures included maturity to Zone III, vascular blunting, vascular loops, vascular dilatation, capillary dropout, and vascular fluorescein leakage...
January 3, 2018: Retina
https://www.readbyqxmd.com/read/29288330/evaluation-of-vertebral-artery-anomaly-in-basilar-invagination-and-prevention-of-vascular-injury-during-surgical-intervention-cta-features-and-analysis
#16
Shuaishuai Xu, Shidong Ruan, Xiaoyu Song, Jinyu Yu, Jianrong Xu, Ruozhen Gong
OBJECTIVE: To evaluate vertebral artery anomaly at the craniovertebral junction (CVJ) in patients with basilar invagination (BI) by computed tomographic angiography (CTA), and to discuss the prevention strategy of vascular injury. METHODS: The primary axial, multiple planar reconstruction and volume-rendering cervicocranial CTA images of 39 BI patients were analysed to evaluate vertebral artery anomaly at the CVJ: persistent first intersegmental artery (PFIA), fenestrated vertebral artery (FEN), and extracranial C1/2 origin of posterior inferior cerebellar artery (PICA), high-riding vertebral artery, side-to-side asymmetry and irregular midline carotid artery loop was determined by subjective vision...
December 29, 2017: European Spine Journal
https://www.readbyqxmd.com/read/29288088/telangiectasias-in-ataxia-telangiectasia-clinical-significance-role-of-atm-deficiency-and-potential-pathophysiological-mechanisms
#17
M H D Schoenaker, N J H Van Os, M Van der Flier, M Van Deuren, M M Seyger, A M R Taylor, C M R Weemaes, M A A P Willemsen
Ataxia Telangiectasia (AT) is named after the two key clinical features that characterize its classical phenotype, namely a progressive cerebellar gait disorder (ataxia) and vascular anomalies (telangiectasias) visible in the conjunctivae and skin. AT is an autosomal recessively inherited disorder, caused by mutations in the ATM gene that encodes the ATM protein. While the ataxia is subject of many publications, the telangiectasias are under emphasised. We here describe the observation that the absence or presence of ATM protein and the level of residual ATM kinase activity are related to the occurrence of telangiectasias and describe the clinical consequences of these vascular malformations...
December 26, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29273297/loss-of-vascular-smooth-muscle-cell-autophagy-exacerbates-angiotensin-ii-associated-aortic-remodeling
#18
Azza Ramadan, Krishna K Singh, Adrian Quan, Pamela J Plant, Mohammed Al-Omran, Hwee Teoh, Subodh Verma
OBJECTIVE: The pathophysiologic processes of abdominal aortic aneurysms (AAAs) and atherosclerosis often intersect. Given that anomalies in vascular smooth muscle cell (SMC) autophagy have been noted in models of atherosclerosis, we sought to evaluate the potential role that SMC autophagy may play in the initiation and progression of AAAs. METHODS: Studies were conducted in ATG7flx/flxSM22α-Cretg/+ (SMC ATG7 knockout [SMC-ATG7-KO]) and ATG7WT/WT; SM22α-Cretg/+ (SMC ATG7 wild-type [SMC-ATG7-WT]) littermates that were continuously infused with angiotensin II (Ang II; 1...
December 19, 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/29260069/atypical-intravitreal-growth-of-retinoblastoma-with-a-multi-branching-configuration
#19
Sachiko Nishina, Satoshi Katagiri, Atsuko Nakazawa, Chikako Kiyotani, Tadashi Yokoi, Noriyuki Azuma
Purpose: To report the clinical and histopathological findings of atypical intravitreal growth of a retinoblastoma with a multi-branching configuration. Observations: A 7-month-old boy was referred to our hospital due to leukocoria in the right eye. Ophthalmic examinations identified multi-branching vessels surrounded by diaphanous tissue behind the lens in the right eye. Imaging modalities showed microphthalmos, band-shaped calcification, and cystic lesions in that eye...
September 2017: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29258533/incidental-branch-retinal-artery-occlusion-on-optical-coherence-tomography-angiography-presenting-as-segmental-optic-atrophy-in-a-child-a-case-report
#20
Ji Hyung Choi, Hee Kyung Yang, Ji Eun Lee
BACKGROUND: Retinal artery occlusion is extremely rare in the pediatric population and most patients have risk factors. We report a case of a healthy child with segmental optic atrophy, complicated by incidental branch retinal artery occlusion (BRAO). CASE PRESENTATION: A 10-year-old boy who had a history of his mother's gestational diabetes presented with an inferonasal visual field defect in the left eye. His best-corrected visual acuities were 20/20 in both eyes (OU)...
December 19, 2017: BMC Ophthalmology
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