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Lymphatic malformation

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https://www.readbyqxmd.com/read/29138199/electrosclerotherapy-for-capillary-malformations-study-protocol-for-a-randomised-within-patient-controlled-pilot-trial
#1
Sophie E R Horbach, Albert Wolkerstorfer, Daniel Martijn de Bruin, Chantal M A M van der Horst
INTRODUCTION: The current state-of-the-art treatment modality for hypertrophic capillary malformations (CMs), laser therapy, has a considerable rate of non-responders and recurrence. Intralesional bleomycin injections (or 'sclerotherapy') are commonly used to treat venous and lymphatic malformations with an excellent effect, but these intravascular injections are not possible in CMs due to the small diameter of the vessels. Electroporation-an electric field applied to the tissue-could increase the permeability of endothelial cells, which could theoretically facilitate targeted localised bleomycin delivery...
November 14, 2017: BMJ Open
https://www.readbyqxmd.com/read/29078703/bipolar-radiofrequency-ablation-coblation-of-external-auditory-canal-lymphatic-malformation-and-other-soft-stenoses
#2
Alex Gu, Nancy M Bauman
INTRODUCTION: Soft tissue occlusion of the external auditory canal (EAC) can cause intense pruritis, recurrent foul smelling otorrhea, recurrent otitis externa, and conductive hearing loss. Occlusion of the EAC can be challenging to treat as the area is prone to circumferential scarring. METHODS: We describe the novel use of serial bipolar radiofrequency ablation (coblation) to treat 3 children with complete EAC occlusion from congenital and acquired conditions including lymphedema (1), microcystic lymphatic malformation (1), and venolymphatic malformation (1)...
December 2017: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29066632/lymphatic-malformation-with-acquired-horner-syndrome-in-an-infant
#3
Birgit Spors, Joerg Seemann, Natalie Homer, Aaron Fay
An infantpresented with right upper eyelid ptosis and was subsequently diagnosed with acquired Horner syndrome. Further evaluation revealed a right-sided cervicothoracic lymphatic malformation. At 13 weeks of age, the child underwent percutaneous intracystic sclerotherapy with a mixture of sodium tetradecyl sulphate and ethanol. Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis. Percutaneous sclerotherapy not only effectively treated the space-occupying lymphatic malformation but also reversed the Horner syndrome that was presumably induced by neural tension (more likely) or compression...
October 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29062665/spontaneous-regression-of-lymphangiomas-in-a-single-center-over-34-years
#4
Motoi Kato, Shoji Watanabe, Reiko Kato, Hiroshi Kawashima, Takuya Iida, Azusa Watanabe
BACKGROUND: A lymphangioma, also called a lymphatic malformation, is a congenital condition that frequently occurs in young children. It is classified into 3 groups depending on the size of the cysts (macrocystic, microcystic, and mixed). Spontaneous regression occurs in some cases; however, the characteristics of patients who show regression have not been studied previously. Furthermore, the types and the timing of the initial treatment are still controversial. Therefore, we statistically analyzed the occurrence of short-term spontaneous regression, patient age at original occurrence, cyst types, cyst sizes, and cyst locations in patients diagnosed with peripheral localized lymphangiomas in a single children center over 34 years...
September 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/29061390/integrated-geometric-and-mechanical-analysis-of-an-image-based-lymphatic-valve
#5
Daniel J Watson, Igor Sazonov, David C Zawieja, James E Moore, Raoul van Loon
Lymphatic valves facilitate the lymphatic system's role in maintaining fluid homeostasis. Malformed valves are found in several forms of primary lymphœdema, resulting in incurable swelling of the tissues and immune dysfunction. Their experimental study is complicated by their small size and operation in low pressure and low Reynolds number environments. Mathematical models of these structures can give insight and complement experimentation. In this work, we present the first valve geometry reconstructed from confocal imagery and used in the construction of a subject-specific model in a closing mode...
October 7, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/29055573/bleomycin-electrosclerotherapy-new-treatment-to-manage-vascular-malformations
#6
L McMorrow, M Shaikh, G Kessell, T Muir
Venous malformations are congenital anomalies of the vascular system. The injection of bleomycin (a cytotoxic, antitumour drug) into the lesion is a safe and effective treatment for low-flow (venous and lymphatic) malformations, but its use systemically has been associated with pulmonary fibrosis. Intralesional injection of bleomycin is considered to have a lower risk, but caution should be used when planning treatment, with particular regard to respiratory function. Electroporation is the temporary application of an electrical field across a tissue to increase (briefly) the permeability of the cell membrane in that tissue...
November 2017: British Journal of Oral & Maxillofacial Surgery
https://www.readbyqxmd.com/read/29047253/percutaneous-drainage-and-povidone-iodine-sclerotherapy-of-cervical-lymphatic-malformation
#7
Seung Hyoung Kim, Mu Sook Lee, Gil Chai Lim, Chan Il Song
Lymphatic malformations in cases with macrocystic lesions can be treated with surgical excision or sclerotherapy using alcohol, bleomycin, doxycycline, or OK-432. We report a case of a 24-year-old woman who underwent percutaneous drainage and povidone-iodine sclerotherapy as primary treatment for cervical lymphatic malformation. The patient underwent povidone-iodine sclerotherapy for 3 consecutive days. After 8 months, ultrasonography of the lesion in the neck revealed complete resolution of the cervical lymphatic malformation without any complication...
November 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29046873/embryonic-stem-cell-like-subpopulations-in-venous-malformation
#8
Elysia M S Tan, Sam Duro Siljee, Helen D Brasch, Susana Enriquez, Swee T Tan, Tinte Itinteang
BACKGROUND: Venous malformation (VM) consists of a network of ectatic anomalous thin-walled venous channels. A role for an activating TIE2 mutation in the development of the dilated luminal vessels in VM, and its proposed involvement of embryonic stem cells (ESCs), led us to investigate the expression of ESC markers in subcutaneous VM (SCVM) and intramuscular VM (IMVM). METHODS: Formalin-fixed paraffin-embedded sections of SCVM from seven patients and IMVM samples from seven patients were analyzed for the expression of Nanog, pSTAT3, OCT4, SOX2, SALL4, and CD44, using 3,3'-diaminobenzidine (DAB) immunohistochemical (IHC) staining...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/29043695/-vascular-anomalies-in-the-neonatal-period
#9
M Bejarano, F Vicario, A Soria, F J Parri, A Albert
OBJECTIVE: Vascular anomalies in the neonatal period are a diagnostic challenge for the lack of evident signs, symptoms and follow-up, and the convenience of restricting aggressive diagnostic tests. The aim of this work is to review the characteristics of neonatal cases presented to our Vascular Anomalies Unit in the last 5 years. MATERIALS AND METHODS: All cases of suspected vascular anomaly presented to our unit before 1 month of age between 2010 and 2015 were reviewed, diagnostic tests and treatments carried out with chronology were analyzed...
July 20, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/29038775/intralesional-endoscopy-and-septectomy-as-a-diagnostic-tool-and-treatment-method-for-lymphatic-malformations
#10
Anne-Sophie Holler, Jan Gödeke, Veronika Engel, Oliver J Muensterer
No abstract text is available yet for this article.
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/29036002/multimodal-imaging-in-klippel-tr%C3%A3-naunay-weber-syndrome-clinical-photography-computed-tomoangiography-infrared-thermography-and-99mtc-phytate-lymphoscintigraphy
#11
Su Wan Kim, Heesung Song
We report the case of a 19-year-old man who presented with a 12-year history of progressive fatigue, feeling hot, excessive sweating, and numbness in the left arm. He had undergone multimodal imaging and was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS). This is a rare congenital disease, defined by combinations of nevus flammeus, venous and lymphatic malformation, and hypertrophy of the affected limbs. Lower extremities are affected mostly. Conventional modalities for evaluating KTWS are ultrasonography, CT, MRI, lymphoscintigraphy, and angiography...
October 13, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29028550/paternal-exposure-to-environmental-17-alpha-ethinylestradiol-concentrations-modifies-testicular-transcription-affecting-the-sperm-transcript-content-and-the-offspring-performance-in-zebrafish
#12
David G Valcarce, Elena Vuelta, Vanesa Robles, Maria Paz Herráez
The synthetic estrogen 17-α-ethinylestradiol (EE2), a major constituent in contraceptive pills, is an endocrine disrupting chemical (EDC) present in the aquatic environment at concentrations of ng/L. Developmental exposure to these low concentrations in fish can induce several disorders. Zebrafish (Danio rerio) is a perfect organism for monitoring the effects of environmental contaminants. Our hypothesis is that changes promoted by EE2 in the germ line of male adults could be transmitted to the unexposed progeny...
September 28, 2017: Aquatic Toxicology
https://www.readbyqxmd.com/read/29026809/spontaneously-resolved-macrocystic-lymphatic-malformations-predictive-variables-and-outcomes
#13
Michael J Phang, Douglas J Courtemanche, Marija Bucevska, Claudia Malic, Jugpal S Arneja
INTRODUCTION: Lymphatic malformations are benign, low-flow vascular malformations that typically present at or near birth. Due to morbidity associated with operative treatment, nonoperative treatment with injection of sclerosant has become the mainstay of therapy. Over the past 15 years, several patients at our centre with macrocystic (>2 cm cyst size) lymphatic malformations have seen their lesions resolve spontaneously while awaiting treatment. In this study, we review features of these patients that may contribute to spontaneous resolution...
February 2017: Plastic Surgery
https://www.readbyqxmd.com/read/29024832/somatic-second-hit-mutation-of-rasa1-in-vascular-endothelial-cells-in-capillary-malformation-arteriovenous-malformation
#14
Philip E Lapinski, Abbas Doosti, Valerie Salato, Paula North, Patricia E Burrows, Philip D King
Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal dominant vascular disorder that is associated with inherited inactivating mutations of the RASA1 gene in the majority of cases. Characteristically, patients exhibit one or more focal cutaneous CM that may occur alone or together with AVM, arteriovenous fistulas or lymphatic vessel abnormalities. The focal nature and varying presentation of lesions has led to the hypothesis that somatic "second hit" inactivating mutations of RASA1 are necessary for disease development...
October 9, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29019760/mr-lymphangiography-in-children-technique-and-potential-applications
#15
Govind B Chavhan, Joao G Amaral, Michael Temple, Maxim Itkin
The lymphatic system, an important component of the circulatory system with essential physiologic functions, can be affected by various disease processes. There has been a delay in the development of effective imaging methods for the lymphatic system due to its small size, which limits visualization as well as introduction of contrast material. Traditionally, the lymphatic system has been imaged by injecting contrast material or radiotracers into the feet or hands. This is not sufficient for assessment of the central conducting lymphatics (CCLs) (such as the thoracic duct or the cisterna chyli)...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28986976/development-of-an-international-core-outcome-set-for-peripheral-vascular-malformations-ovama-project
#16
S E R Horbach, C M A M van der Horst, F Blei, C J M van der Vleuten, I J Frieden, G T Richter, S T Tan, T Muir, A Penington, L M Boon, P I Spuls
BACKGROUND: An important limitation in vascular malformation research is the heterogeneity in outcome measures used for the evaluation of treatment outcome. OBJECTIVE: The Outcome measures for VAscular MAlformations (OVAMA) project aimed to reach international consensus on a core outcome set (COS) for clinical research on peripheral vascular malformations: lymphatic (LM), venous (VM) and arteriovenous malformations (AVM). In this consensus study, we determined what domains should constitute the COS...
October 7, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28963775/clinical-and-hemodynamic-risk-factors-associated-with-discrepancies-in-lower-limb-length-with-capillary-malformations-data-from-the-national-paediatric-french-cohort-conape
#17
M Samimi, M Durieux-Verde, A Caille, J Mazereeuw-Hautier, O Boccara, L Martin, C Chiaverini, C Eschard, N Beneton, P Vabres, X Balguerie, P Plantin, D Bessis, S Barbarot, A Dadban, C Droitcourt, S Leducq, G Lorette, B Morel, A Maruani
BACKGROUND: Genetics discoveries have allowed for better understanding capillary malformations (CMs) with overgrowth syndrome. However, molecular analyses are still not easy to perform or interpret. Other analytical methods are needed. OBJECTIVE: We aimed to identify clinical and hemodynamic factors associated with leg length discrepancy (LLD) in children with CM of lower limbs. METHODS: Data were obtained from the multicentre French national cohort CONAPE (COhorte Nationale d'enfants atteints d'Angiome Plan de membrE inférieur), including children from 2 to 12 years old with CM of lower limbs...
September 30, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28955596/cavernous-lymphangioma-of-the-tongue-in-an-adult-a-case-report
#18
Selin Eren, Ahmet Taylan Cebi, Sabri Cemil Isler, Metin Berk Kasapoglu, Nihan Aksakalli, Cetin Kasapoglu
Lymphangioma is a benign hamartomatous lesion caused by congenital malformation of the lymphatic system. This benign tumor is detected most commonly at birth or in early childhood but rarely in adults. On clinical examination, most lymphangiomas contain clear lymph fluid, but some may present as transparent vesicles containing red blood cells due to hemorrhage. In addition, lymphangioma may occur in association with hemangioma. This tumor occurs most commonly in the head and neck area, but rarely in the oral cavity...
2017: J Istanb Univ Fac Dent
https://www.readbyqxmd.com/read/28955119/magnetic-resonance-lymphangiography-and-lymphatic-embolization-in-the-treatment-of-pulmonary-complication-of-lymphatic-malformation
#19
REVIEW
Maxim Itkin
Lymphatic malformations (LMs; especially those involving the central conducting lymphatic channels) are characterized by dysplastic and incompetent lymphatic channels in multiple tissues and organs. The major cause of mortality and morbidity in patients with thoracic LM is deterioration of pulmonary function due to chronic chylous effusions and progressive interstitial lung disease. The etiology of these pulmonary processes is unknown, although lymphatic involvement is certain. Understanding of the changes in the lymphatic anatomy in patients with LM has been hindered by difficulty of imaging of the lymphatic system...
September 2017: Seminars in Interventional Radiology
https://www.readbyqxmd.com/read/28928923/adult-neck-hemangiolymphangioma-a-case-and-review-of-its-etiology-diagnosis-and-management
#20
Tyler Murphy, Daryl Ramai, Jonathan Lai, Kiyoe Sullivan, Cecil Grimes
Vascular anomalies constitute some of the most difficult diagnostic and therapeutic enigmas in the practice of medicine, ranging from an asymptomatic birthmark to life-threatening congestive heart failure. Hemangiolymphangiomas (HLA) are extremely rare vascular malformations of both lymphatic and blood vessels in which 80-90% are diagnosed during the first 2 years of life. Though rare, these vascular malformations have promising clinical outcomes. We report a case of a 28-year-old male who presented with a neck mass of unknown etiology...
August 2017: Journal of Surgical Case Reports
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