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Lymphatic malformation

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https://www.readbyqxmd.com/read/28639460/role-of-ct-scan-in-diagnosis-of-renal-lymphangiectasia-our-single-center-experience
#1
Vaidehi K Pandya, Harsh C Sutariya, Shruti P Gandhi, Sajni I Khemchandani, Himanshu V Patel, Maulin K Shah
BACKGROUND: Renal lymphangiectasia is rarely reported benign renal disorder of lymphatic malformation. Though found incidentally; it presents with nonspecific symptoms and shows characteristic findings in radiological imaging studies. AIM: Here, we report eight patients with symptoms, laboratory and imaging findings compatible with renal lymphangiectasia. This report describes clinical and laboratory characteristics, treatment, Imaging findings and outcome of a series of patients with renal lymphangiectasia and reviews the literature...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28635993/head-and-neck-vascular-anomalies-a-multidisciplinary-approach-and-diagnostic-criteria
#2
L Moneghini, V Sangiorgio, D Tosi, G Colletti, F Melchiorre, V Baraldini, D Graziani, R M Alfano, G Vercellio, G Bulfamante
Vascular anomalies represent a heterogeneous group of pathologies of the circulatory system that can affect any type of hematic and /or lymphatic vessel of different diameter or anatomic site. The extreme variability of tissue types and districts involved by these lesions determines a wide heterogeneity of clinical manifestations, resulting in involvement of different medical expertise. In this context, a commonly agreed terminology is crucial for the appropriate evaluation and multidisciplinary management of patients...
March 2017: Pathologica
https://www.readbyqxmd.com/read/28630776/sudden-onset-rapidly-expansile-cervical-cystic-hygroma-in-an-adult-a-rare-case-with-unusual-presentation-and-extensive-review-of-the-literature
#3
Vivek Dokania, Anagha Rajguru, Harmanjot Kaur, Ketan Agarwal, Sujata Kanetkar, Prajakta Thakur, Femina Patel, Dhirajkumar Shukla
Cystic hygroma (CH) is a benign infiltrative malformation of the lymphatic channels. We report a case of a 28-year-old Indian female who presented with rapidly enlarging right sided neck swelling over the posterior triangle since 5 days. Complete resection of CH is sometimes not amenable because of its infiltrative nature and involvement of surrounding vital structures. However, in our patient successful complete surgical resection was undertaken. The MRI findings of our patient were consistent with brachial cleft cyst; this posed a challenge in the diagnosis of CH...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/28623992/near-infrared-fluorescence-lymphatic-imaging-of-klippel-tr%C3%A3-naunay-syndrome
#4
John C Rasmussen, Rodrick C Zvavanjanja, Melissa B Aldrich, Matthew R Greives, Eva M Sevick-Muraca
The relationship between lymphatic and venous malformations in Klippel-Trénaunay syndrome is difficult to assess. Herein the authors describe near-infrared fluorescence lymphatic imaging to assess the lymphatics of a subject with a large port-wine stain and right leg edema. Although lymphatic vessels in the medial, affected knee appeared dilated and perhaps tortuous, no definitive abnormal lymphatic pooling or propulsion was observed. The lymphatics in the affected limb were well defined but less numerous than in the contralateral limb, and active, contractile function was observed in all vessels...
July 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28617243/fingertip-capillary-malformation-and-associated-disorders-report-of-9-cases
#5
Juan Carlos López-Gutiérrez, Pedro Redondo, Marta Ivars
Although capillary malformations (CMs) are not usually serious health problems in themselves, they can occasionally be warning signs for syndromes with more serious or aggressive vascular malformations not readily apparent at birth or on initial examination. We describe a series of 9 patients with a common phenotype: (1) CM on the fingertips; (2) associated combined vascular (lymphatic-venous) malformations on the trunk and/or extremities; and (3) in some cases, partial overgrowth and asymmetry of the extremities...
June 15, 2017: Pediatrics
https://www.readbyqxmd.com/read/28606692/percutaneous-sclerotherapy-of-pediatric-lymphatic-malformations-experience-and-outcomes-according-to-the-agent-used
#6
C Gallego Herrero, V Navarro Cutillas
OBJECTIVE: Analyze statistically the success, number of sessions required and complete duration of treatment of agents used in pediatric percutaneous sclerotherapy of lymphatic malformations, to determine the most suitable. MATERIAL AND METHODS: Retrospective study based on outcomes from percutaneous sclerotherapy performed on lymphatic malformations of 56 patients conducted by pediatric interventional radiologist for 14 years. As first approach, the procedure consists of ultrasound-guided introduction of sclerosing agent...
June 9, 2017: Radiología
https://www.readbyqxmd.com/read/28599969/prolonged-antibiotic-treatment-for-infected-low-flow-vascular-malformations
#7
Katy M Wagner, Zerina Lokmic, Anthony J Penington
BACKGROUND: Infection in low flow malformations is difficult to diagnose and treat. Initial presentation can be followed by cycles of recurrent infection lasting several years. The optimal duration of antibiotic therapy to prevent recurrence of infection has not been established. METHODS: All cases of infection in low flow malformations at the Royal Children's Hospital over a ten-year period were reviewed. Clinical markers of infection and duration of initial antibiotic treatment were correlated with the development of recurrent episodes of infection...
May 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28587325/sildenafil-suppresses-the-proliferation-and-enhances-the-apoptosis-of-hemangioma-endothelial-cells
#8
Xiaopo He, Yiran Liu, Kai Li, Aijun Yang, Rui Wang, Shaohua Liu
Treatment of infantile hemangiomas (IH) with propranolol was first reported in 2008. Regressions of lymphatic malformations combined with pulmonary hypertension was first reported in 2012 after three children received treatment with oral sildenafil, which serves as an antagonist of phosphodiesterase isoform-5 (PDE-5). A marked expression of endothelial cells in the cytoplasm of IH tissues was obtained in our previous study. Therefore, the present study hypothesized that the antagonist of PDE-5, sildenafil, may lead to the regression of hemangiomas...
June 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28577738/-pik3ca-related-overgrowth-syndrome-pros
#9
Quitterie Venot, Guillaume Canaud
This review presents an overview of a recently characterized spectrum of overgrowth syndrome: phosphoinositide-3 kinase (PI3K)-related overgrowth spectrum (PROS). This spectrum encompasses overgrowth syndromes associated with somatic mosaic activating PIK3CA mutations such as megalencephaly-capillary malformation (MCAP) syndrome, dysplatic megalencephaly (DMEG), congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies (CLOVES) syndrome, hemihyperplasia-multiple lipomatosis (HHML), fibroadipose overgrowth and Klippel-Trenaunay syndrome...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28576544/cystic-cervical-teratoma-a-diagnostic-and-management-challenge
#10
Nikolaus E Wolter, Bradford Siegele, Michael J Cunningham
Cervical teratomas are relatively rare congenital tumors pathologically defined by their three germ cell layer composition. Classically they manifest as solid or mixed solid-cystic lesions. Purely cystic teratomas pose a diagnostic challenge due to their clinical and radiologic similarity to lymphatic vascular malformations. Sclerotherapy, rather than operative excision, may be offered as therapy. We present an unusual cystic neck mass to highlight this dilemma.
April 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28557723/microcystic-lymphatic-malformation-successfully-treated-with-topical-rapamycin
#11
Pablo García-Montero, Javier Del Boz, Miguel Sanchez-Martínez, Isabel María Escudero Santos, Eulalia Baselga
Microcystic lymphatic malformations (MLM) are low-flow vascular malformations composed of multiple small cysts. MLM usually affect deep-lying structures, which makes their treatment even more difficult and complex. A novel and interesting treatment is rapamycin, a mammalian target of rapamycin inhibitor that when orally administrated has offered favorable results. However, until recently, topical rapamycin had not been used in the treatment of MLM. Case 1 is a girl aged 13 years with extensive MLM affecting the muscles in the right buttock...
May 2017: Pediatrics
https://www.readbyqxmd.com/read/28552828/down-regulation-of-polycystin-in-lymphatic-malformations-possible-role-in-the-proliferation-of-lymphatic-endothelial-cells
#12
Jian-Gang Ren, Hou-Fu Xia, Jie-Gang Yang, Jun-Yi Zhu, Wei Zhang, Gang Chen, Ji-Hong Zhao, Yan-Fang Sun, Yi-Fang Zhao
Lymphatic malformations (LMs) are composed of aberrant lymphatic vessels and regarded as benign growths of the lymphatic system. Recent studies have demonstrated that the mutant embryos of PKD1 and PKD2, encoding polycystin-1 (PC-1) and polycystin-2 (PC-2), respectively, result in aberrant lymphatic vessels similar to those observed in LMs. In this study, for the first time, we investigated PC-1 and PC-2 expression and assessed their roles in the development of LMs. Our results demonstrated that PC-1 and PC-2 gene and protein expression were obviously decreased in LMs compared with normal skin tissues...
May 25, 2017: Human Pathology
https://www.readbyqxmd.com/read/28552538/the-utility-of-glut1-as-a-diagnostic-marker-in-cutaneous-vascular-anomalies-a-review-of-literature-and-recommendations-for-daily-practice
#13
REVIEW
Lieke J van Vugt, Carine J M van der Vleuten, Uta Flucke, Willeke A M Blokx
OBJECTIVE: To assess the utility of GLUT1 as an immunohistochemical marker in the diagnostics of cutaneous vascular anomalies. METHODS: A systematic literature search was conducted for studies on GLUT1 staining patterns in cutaneous vascular lesions. Data was grouped according to the latest ISSVA classification for vascular anomalies. RESULTS: Vascular tumors: GLUT1 staining was positive in 368/386 (95%) of infantile hemangiomas. Congenital hemangiomas (16 cases) and kaposiform hemangioendotheliomas (62 cases) were all negative for GLUT1...
June 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28540347/interdisciplinary-management-of-head-and-neck-vascular-anomalies-clinical-presentation-diagnostic-findings-and-minimalinvasive-therapies
#14
REVIEW
Maliha Sadick, Walter A Wohlgemuth, Roland Huelse, Bettina Lange, Thomas Henzler, Stefan O Schoenberg, Haneen Sadick
OBJECTIVES: Vascular anomalies are included in the 30 000 rare diseases worldwide affecting less than 5/10 000 people. Depending on their morphology and biological properties, they can cause varied disorders with organ involvement. Almost 60% of vascular anomalies have a predilection for the head and neck region in children. Clinical and scientific effort to establish interdisciplinary management concepts for vascular anomalies is increasing worldwide. METHODS: Especially in the head and neck region, clinical impairment and organ dysfunction is associated with cosmetic issues that may represent a physical and psychological issue for the patient...
2017: European Journal of Radiology Open
https://www.readbyqxmd.com/read/28536688/isolated-fetal-lymphatic-malformation-of-the-thigh-prenatal-diagnosis-and-follow-up
#15
Huseyin Durukan, İlay Gözükara, Murside Cevikoglu, Talat Umut Kutlu Dilek
Fetal lymphatic malformation can be found in different parts of the fetal body. It occurs most frequently in the nuchal and axillary region and less frequently in the abdomen or inguinal areas. Lymphatic malformation has been associated with fetal aneuploidy, hydrops fetalis, structural malformations, and intrauterine fetal death. A 31-year-old gravida 3, para 2 woman was admitted to our hospital at 22 weeks of gestation (confirmed by ultrasonographic examination). The fetus was alive, and had a mass derived from the left inguinal region extending to the anterior left leg with fluid-filled cavities about 3-5 cm in size...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28536213/non-surgical-treatment-of-a-relapsed-cystic-hygroma-in-an-adult
#16
Rafael García Carretero, Belen Rodriguez-Maya, Oscar Vazquez-Gomez
Lymphatic malformations, also known as lymphangiomas or cystic hygromas, are benign masses that typically affect newborns and infants and involve the head and neck regions. They are, however, rare in adults and even rarer in the axillary region. Although surgery is considered to be the treatment of choice, we present a rare case of a recurrent cystic hygroma 32 years after the first surgical operation. Due to the cosmetic concerns and the risks of a surgical approach, non-surgical therapy with percutaneous sclerosants was performed, with a good outcome after a 2-year follow-up period...
May 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28530642/rasa1-regulates-the-function-of-lymphatic-vessel-valves-in-mice
#17
Philip E Lapinski, Beth A Lubeck, Di Chen, Abbas Doosti, Scott D Zawieja, Michael J Davis, Philip D King
Capillary malformation-arteriovenous malformation (CM-AVM) is a blood and lymphatic vessel (LV) disorder that is caused by inherited inactivating mutations of the RASA1 gene, which encodes p120 RasGAP (RASA1), a negative regulator of the Ras small GTP-binding protein. How RASA1 mutations lead to the LV leakage defects that occur in CM-AVM is not understood. Here, we report that disruption of the Rasa1 gene in adult mice resulted in loss of LV endothelial cells (LECs) specifically from the leaflets of intraluminal valves in collecting LVs...
May 22, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28515992/dermoscopy-of-cutaneous-lymphangioma-circumscriptum
#18
Abhijeet K Jha, Aimilios Lallas, Sidharth Sonthalia
Lymphangiomas are congenital lymphatic malformations. They are clinically characterized by clusters of translucent vesicles, and on dermoscopy, yellow lacunae surrounded by pale septa as well as reddish to bluish lacunae have been described. A young male presented with a seven-year history of a vesicular lesion. Dermoscopy revealed multiple white-yellowish well-circumscribed roundish areas (lacunae) surrounded by pale septa. A few lacunae contained blood, which was characteristically accumulated in the lowest part of the lacuna, resulting in an appearance similar to the so-called "hypopyon" of the eye...
April 2017: Dermatology Practical & Conceptual
https://www.readbyqxmd.com/read/28500279/new-navigation-surgery-for-resection-of-lymphatic-malformations-using-indocyanine-green-fluorescence-imaging
#19
Chiyoe Shirota, Akinari Hinoki, Masataka Takahashi, Yujiro Tanaka, Takahisa Tainaka, Wataru Sumida, Naruhiko Murase, Kazuo Oshima, Ryo Shirotsuki, Kosuke Chiba, Yuji Morimoto, Hiroo Uchida
BACKGROUND We used indocyanine green (ICG) fluorescence imaging to completely resect lymphatic malformations (LMs). This is the first report of navigation surgery utilizing ICG fluorescence imaging for resection of LMs. CASE REPORT A 15-year-old boy was diagnosed with LMs in the abdominal wall. The extent of the tumor was determined by an ultrasound, and ICG (Diagnogreen®, Daiichi-Sankyo Pharma, Tokyo, Japan) was injected subcutaneously and intradermally into the core and 2 marginal regions of the tumor (3 injections in total), respectively...
May 13, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28491199/emergent-sclerotherapy-of-a-newborn-with-expanding-lymphatic-malformation-causing-respiratory-distress
#20
Cameron C Sheehan, Winslo Idicula, Andrew Raabe, Kris Jatana, Tendy Chiang, Charles A Elmaraghy
The present report describes a case of acute airway obstruction in a newborn caused by an expanding hemorrhagic macrocystic lymphatic malformation (LM), which was successfully treated with emergent decompression and interventional radiology-guided sclerotherapy. The use of sclerotherapy for macrocystic LMs has been well described for various indications. The urgent interventional treatment obviated the need for a tracheostomy. This case describes the rapid diagnosis and use of sclerotherapy in a large expanding macrocystic LM...
June 2017: Radiology case reports
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