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Lymphatic malformation

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https://www.readbyqxmd.com/read/28540347/interdisciplinary-management-of-head-and-neck-vascular-anomalies-clinical-presentation-diagnostic-findings-and-minimalinvasive-therapies
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28483255/anatomic-distribution-of-cervicofacial-lymphatic-malformations-based-on-lymph-node-groups
#8
Phayvanh P Sjogren, Ryan W Arnold, Jonathan R Skirko, Johannes F Grimmer
OBJECTIVES: To evaluate radiographic characteristics and to identify locations of cervicofacial lymphatic malformations in children based on known lymph node groupings. METHODS: Retrospective chart review of pediatric patients with cervicofacial lymphatic malformations who underwent imaging with magnetic resonance imaging (MRI), computed tomography (CT) or ultrasonography (US). Ninety charts were reviewed from November 2005 to June 2015. Demographic information and imaging characteristics were evaluated...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28461089/vascular-lesions-of-the-female-genital-tract-clinicopathologic-findings-and-application-of-the-issva-classification
#9
Georgia Karpathiou, Celine Chauleur, Vanessa Da Cruz, Fabien Forest, Michel Peoc'h
INTRODUCTION: Vascular lesions of the female genital tract are extremely rare and their nomenclature does not widely follow the International Society for the Study of Vascular Anomalies (ISSVA) classification. AIM OF THE STUDY: To describe the clinicopathologic characteristics of vascular lesions of the female genital tract and to apply the ISSVA classification. MATERIAL AND METHODS: 19 vascular lesions were diagnosed during a 20 year period...
April 23, 2017: Pathophysiology: the Official Journal of the International Society for Pathophysiology
https://www.readbyqxmd.com/read/28458832/a-rare-presentation-of-klippel-trenaunay-syndrome-with-bilateral-lower-limbs
#10
Akira Baba, Shinji Yamazoe, Yumi Okuyama, Kanichiro Shimizu, Yuko Kobashi, Yosuke Nozawa, Yohei Munetomo, Takuji Mogami
Klippel-Trenaunay syndrome (KTS) is a vascular lymphatic malformation underlying with bony and soft tissue hypertrophy. It is a rare condition presenting in 1 out of 10 000 people. The growth disturbance due to KTS is more commonly unilateral (85%) than bilateral (12.5%), and most rarely crossed-bilateral (2.5%). A man in his 40s presented to our hospital with a complaint of lower limb discomfort. Radiograph, ultrasonography, computed tomography venography, magnetic resonance (and venography) showed various radiological findings characteristic for KTS...
February 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28450987/-a-rare-cause-of-spontaneous-hemoperitoneum-in-adults-ruptured-cystic-hemolymphangioma
#11
Boubacar Zan Traoré, Kamal Serrar, Omar Saoud, Khedid Yahia Zain El Abidine, Rachid Mohammed Chkoff, Rachida M'bida
Hemolymphangioma is a benign tumor resulting from the abnormal development of the lymphatic vessels associated with vascular malformations. 50% to 60% of these tumours are present at birth. Cervical forms are most frequent. Abdominales locations are very rare. We report, through a literature review, two cases of patients with cystic hemolymphangioma of the spleen revealed by spontaneous haemoperitoneum admitted to the Emergency Department of Visceral Surgery. The first case is a 50-year old patient and the second is 20-year old man admitted to the emergency department with diffuse abdominal pain associated with dullness to percussion on the dependent side and abdominal distension associated with haemorrhagic shock, pallor, unobtainable pulse, stabilized after a short resuscitation time respectively...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28438388/kaposiform-haemangioendothelioma-a-review-with-emphasis-on-histological-differential-diagnosis
#12
REVIEW
Juan Putra, Anita Gupta
Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive/borderline vascular tumour primarily seen in neonates and children. KHE is occasionally associated with Kasabach-Merritt phenomenon and tends to have a poor clinical prognosis. While the histological features of tufted angiomas and KHE overlap, some believe tufted angiomas are a milder, benign, more localised counterpart of KHE. The other histological differential diagnoses of KHE include infantile haemangioma, congenital haemangioma, spindle cell haemangioma, verrucous malformation/haemangioma, and Kaposi sarcoma...
April 21, 2017: Pathology
https://www.readbyqxmd.com/read/28390757/multistage-sclerotherapy-for-extensive-lymphatic-malformations-with-airway-involvement-in-infant-a-protocol-to-prevent-tracheotomy
#13
An-Wei Chen, Tao Wang, Ying-Ying Huang, Shao-Hua Liu
PURPOSE: The management of extensive head and neck lymphatic malformations (LMs) in infants is challenging because of life-threatening upper airway compression. The aim of this study was to present a management protocol and evaluate the clinical outcomes for preventing tracheotomy in these patients. MATERIALS AND METHODS: Fifteen infants with extensive head and neck LMs and airway involvement were enrolled from August 2010 through September 2015 at the Qilu Hospital of Shandong University (Jinan, China)...
March 18, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28390608/using-sildenafil-viagra-to-treat-congenital-lymphatic-malformations-of-the-head-and-neck-our-experience
#14
John Rocke, Gavin Quail, Graeme Copley
No abstract text is available yet for this article.
May 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28356336/near-infrared-fluorescence-lymphatic-imaging-of-a-toddler-with-congenital-lymphedema
#15
Matthew R Greives, Melissa B Aldrich, Eva M Sevick-Muraca, John C Rasmussen
Primary lymphedema in the pediatric population remains poorly diagnosed and misunderstood due to a lack of information on the causation and underlying anatomy of the lymphatic system. Consequently, therapeutic protocols for pediatric patients remain sparse and with little evidence to support them. In an effort to better understand the causation of primary pediatric lymphedema and to better inform clinical care, we report the use of near-infrared fluorescence lymphatic imaging on the extremities of an alert, 21-month-old boy who presented with unilateral right arm and hand lymphedema at birth...
March 29, 2017: Pediatrics
https://www.readbyqxmd.com/read/28342818/intraneural-microcystic-lymphatic-malformation-of-the-ulnar-nerve-at-the-guyon-canal-unusual-cause-of-ulnar-pain-in-a-child
#16
I González Pérez, F Corella Montoya, I Casado Fariñas
We present a case of an unusual cause of ulnar pain on a 9 years old patient. The patient had pain on the ulnar side of the wrist after a fall. MRI showed a poorly defined lesion on the ulnar nerve at Guyońs canal. The initial diagnosis was traumatic neuropathy. Following conservative treatment of symptoms for one year, the pain started again. Therefore, a new MRI was performed were progression of the lesion was observed. Excision of the lesion was performed and the specimen sent for pathologic analysis. The diagnosis was of microcystic lymphatic malformation...
March 22, 2017: Orthopaedics & Traumatology, Surgery & Research: OTSR
https://www.readbyqxmd.com/read/28328134/somatic-pik3ca-mutations-in-seven-patients-with-pik3ca-related-overgrowth-spectrum
#17
Kit San Yeung, Janice Jing Kun Ip, Chin Pang Chow, Evelyn Yue Ling Kuong, Paul Kwong-Hang Tam, Godfrey Chi-Fung Chan, Brian Hon-Yin Chung
Somatic mutations in PIK3CA cause many overgrowth syndromes that have been recently coined the "PIK3CA-Related Overgrowth Spectrum." Here, we present seven molecularly confirmed patients with PIK3CA-Related Overgrowth Spectrum, including patients with Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Scoliosis/Skeletal and Spinal syndrome, Klippel-Trenaunay syndrome, lymphatic malformation and two with atypical phenotypes that cannot be classified into existing disease categories. The literature on PIK3CA-Related Overgrowth Spectrum, suggests that PIK3CA c...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28242095/-superficial-cystic-lymphatic-malformations
#18
R Gabeff, G Lorette, D Herbreteau, A le Touze, D Goga, A Maruani
No abstract text is available yet for this article.
May 2017: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/28230604/stereophotogrammetric-evaluation-of-labial-symmetry-after-surgical-treatment-of-a-lymphatic-malformation
#19
Valentina Pucciarelli, Filippo Tarabbia, Marina Codari, Giulia Andrea Guidugli, Giacomo Colletti, Giovanni Dell'Aversana Orabona, Bernardo Bianchi, Chiarella Sforza, Federico Biglioli
Lymphatic malformations (LMs) are rare, nonmalignant masses, frequently involving the head and neck, potentially causing impairment to the surrounding anatomical structures. Major LMs frequently cause facial disfigurement with obvious consequences on self-esteem and social functioning. The attempt to restore symmetry is thus one of the main goals of treatment. In this study, the authors present a not-invasive method to objectively quantify the symmetry of the labial area before and after surgical treatment of a LM, affecting a 16-year-old woman...
February 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28228882/renal-lymphangiomatosis-a-rare-differential-diagnosis-for-autosomal-recessive-polycystic-kidney-disease-in-pediatric-patients
#20
Amna A Kashgari, Nabil Ozair, Amin Al Zahrani, Mohammed O Al Otibi, Khalid Al Fakeeh
Renal lymphangiomatosis is a rare, benign malformation, characterized by developmental malformation of the perirenal, peripelvic, and intrarenal lymphatics. Radiologist knowledge of the unique radiological features of this entity helps patient's safety in terms of management. We study the case of a 27-month-old boy presented to the emergency department with upper respiratory tract infection. He had a high blood pressure and had been diagnosed earlier with autosomal recessive polycystic kidney disease based on renal ultrasound findings...
March 2017: Radiology case reports
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