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Lymphatic malformation

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https://www.readbyqxmd.com/read/28928923/adult-neck-hemangiolymphangioma-a-case-and-review-of-its-etiology-diagnosis-and-management
#1
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
https://www.readbyqxmd.com/read/28927352/an-acute-presentation-of-pediatric-mesenteric-lymphangioma-a-case-report-and-literature-overview
#2
Céline Clement, Rob Snoekx, Pieter Ceulemans, Inez Wyn, Jan Matheï
INTRODUCTION: Lymphangioma accounts for 5% of benign pediatric tumors and less than 1% is located in the abdomen. While mesenteric lymphatic malformation or mesenteric lymphangioma (ML), mostly appears asymptomatic in adults, an acute clinical presentation is seen more often in children. Symptoms can mimic obstruction or peritonitis and diagnosis can therefore be challenging. Imaging studies are performed to exclude other causes of obstruction or peritonitis. Diagnosis of ML is always made by histology...
September 19, 2017: Acta Chirurgica Belgica
https://www.readbyqxmd.com/read/28913305/treatment-of-tongue-lymphangioma-with-intralesional-combination-injection-of-steroid-bleomycin-and-bevacizumab
#3
Jungil Hwang, Yung Ki Lee, Jin Sik Burm
Lymphangioma is a congenital malformed lymphatic tumor that rarely involves the tongue. In our clinic, a 10-year-old female presented with lymphangioma circumscriptum involving the right two-thirds of the tongue. We administered an intralesional combination injection of triamcinolone, bleomycin, and bevacizumab as a treatment. Almost complete remission after combination therapy was achieved without complications such as edema, swallowing difficulties or recurrence. Bevacizumab, an inhibitor of vascular endothelial growth factor, was effective for the treatment of lymphangioma of the tongue in this case...
March 2017: Arch Craniofac Surg
https://www.readbyqxmd.com/read/28884903/long-term-follow-up-of-lymphatic-malformations-in-children-treated-with-sildenafil
#4
Joanna H Tu, Elidia Tafoya, Michael Jeng, Joyce M Teng
BACKGROUND/OBJECTIVES: Lymphatic malformations (LMs) are challenging to treat. Reports on the benefits of sildenafil for LM management have been mixed. This study evaluated long-term clinical outcomes of pediatric LM patients after sildenafil treatment. METHODS: A retrospective chart review was performed on pediatric LM patients treated with sildenafil in the past 5 years. Patients were also contacted to complete a survey of comprehensive questions about their LM after sildenafil and subsequent interventions...
September 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28869282/percutaneous-sclerotherapy-using-bleomycin-for-the-treatment-of-vascular-malformations
#5
Hyun-Joo Lee, Tae-Wook Kim, Jeong-Min Kim, Gun-Wook Kim, Hyun-Chang Ko, Byung-Soo Kim, Moon-Bum Kim, Hoon-Soo Kim
BACKGROUND: Some vascular malformations are difficult to manage with surgical treatment. Thus, percutaneous sclerotherapy has been utilized with several advantages over surgical treatment. Bleomycin is one of several sclerosing agents used for the treatment of vascular malformations. OBJECTIVES: To evaluate therapeutic efficacy and safety of percutaneous sclerotherapy using bleomycin for the treatment of vascular malformations. METHODS: Thirty patients diagnosed with vascular malformations, including four with capillary malformations with nodular change, nine with lymphatic malformations, and 17 with venous malformations, were involved in the study...
September 4, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28846945/colonic-mesenteric-lymphatic-malformation-presenting-as-an-intraabdominal-abscess-in-an-infant-a-case-report
#6
Gonakoti Sriram, Benjamin Zendejas, Sara O Vargas, Catherine Chen
INTRODUCTION: Lymphatic malformations are low-flow vascular malformations most commonly located in the head and neck; isolated intraabdominal involvement is rare. PRESENTATION OF CASE: An 8-month-old previously healthy male presented with a 9-day history of fevers. On examination, right-sided abdominal tenderness was noted. Ultrasound revealed a large heterogeneous mass, and CT scan revealed a rim-enhancing cystic mass adjacent to the right colon. Laboratory investigation including blood cultures was normal...
August 5, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28838528/cardiac-lymphangioma-encasing-right-coronary-artery-in-an-infant
#7
Neha Bansal, Sara Haidar-El-Atrache, Henry L Walters, Daisuke Kobayashi
Cardiac lymphangioma is a rare primary benign tumor of the heart. We report a 3-year-old with cystic lymphangioma encasing the right coronary artery. Cardiac magnetic resonance imaging confirmed a intra-pericardial heterogeneous mass measuring 2.6 × 2.4 × 3.9 cm and situated right anterolateral to the ascending aorta and extending into the right atrioventricular groove. Furthermore, the right coronary artery traversed through the center of the mass. Surgical resection, on cardiopulmonary bypass, consisted of excision by skeletonizing the right coronary artery along the length of the mass...
September 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28838522/resection-of-a-giant-cardiac-lymphovenous-malformation-involving-the-right-atrioventricular-groove
#8
Peter Chiu, Amanda Edmonson, Zachary E Brewer, Y Joseph Woo
Lymphovenous malformations of the heart are rare, and optimal management is uncertain. We present a case of a 39-year-old gentleman with a giant symptomatic lymphovenous malformation involving the right atrium, ventricle, and coronary artery. Radical resection was performed with replacement of the tricuspid valve and bovine pericardial reconstruction of the atrium and ventricle. Additional coronary artery bypass grafting was performed to the acute marginal and distal right coronary artery. Radical resection for this benign process is feasible and may be considered given the possibility of recurrence seen with lymphatic malformations of other parts of the body...
September 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28837798/lymphotoxins-promote-the-progression-of-human-lymphatic-malformation-by-enhancing-lymphatic-endothelial-cell-proliferation
#9
Jie-Gang Yang, Yan-Fang Sun, Ke-Fei He, Jian-Gang Ren, Zhuo-Jue Liu, Bing Liu, Wei Zhang, Yi-Fang Zhao
Formation of inflammation-related tertiary lymphoid organs promotes human lymphatic malformation (LM) development. However, the role of lymphotoxins (LTs) and LT-related inducible ligand, the crucial mediators for tertiary lymphoid organ formation, is undetermined in LMs. Herein, we show that LTs and LT-related inducible ligand promote LM development by enhancing lymphatic endothelial cell (LEC) proliferation via activating NF-κB pathways. The expression of LTs and their receptors was increased in LMs, especially the infected ones, when compared with normal skins...
August 21, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28834841/lymphatic-malformation-architecture-implications-for-treatment-with-ok-432
#10
Claudia C Malic, Regan Guilfoyle, Rebecca J M Courtemanche, Jugpal S Arneja, Manraj K S Heran, Douglas J Courtemanche
PURPOSE: Herein, the authors aim to describe their findings of novel architectural types of lymphatic malformations (LM) and explain the relationship between these architectures and OK-432 treatment outcomes. METHODS: A retrospective review was conducted of all patients diagnosed with a LM treated with OK-432 at the Vascular Anomalies Clinic at BC Children's Hospital from December 2002 to January 2012. RESULTS: Twenty-seven patients were included in the study...
August 22, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28818226/percutaneous-ultrasound-guided-sclerotherapy-with-polidocanol-microfoam-for-lymphatic-malformations
#11
Takashi Yamaki, Yumiko Sasaki, Yuki Hasegawa, Atsuyoshi Osada, Hisato Konoeda, Atsumori Hamahata, Motohiro Nozaki, Hiroyuki Sakurai
BACKGROUND: Lymphatic malformations (LMs) are low-flow congenital lesions that consist of cysts of varying size. Sclerotherapy with intralesional bleomycin and OK-432 has been reported to yield dramatically beneficial results for this disorder. However, inflammation-related symptoms are often seen after treatment with these sclerosing agents. On the other hand, polidocanol (POL) is reportedly associated with fewer allergic and inflammatory reactions. Up to now, however, very few reports have documented the use of POL microfoam for treatment of LMs...
September 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28814672/vipar-a-quantitative-approach-to-3d-histopathology-applied-to-lymphatic-malformations
#12
René Hägerling, Dominik Drees, Aaron Scherzinger, Cathrin Dierkes, Silvia Martin-Almedina, Stefan Butz, Kristiana Gordon, Michael Schäfers, Klaus Hinrichs, Pia Ostergaard, Dietmar Vestweber, Tobias Goerge, Sahar Mansour, Xiaoyi Jiang, Peter S Mortimer, Friedemann Kiefer
BACKGROUND: Lack of investigatory and diagnostic tools has been a major contributing factor to the failure to mechanistically understand lymphedema and other lymphatic disorders in order to develop effective drug and surgical therapies. One difficulty has been understanding the true changes in lymph vessel pathology from standard 2D tissue sections. METHODS: VIPAR (volume information-based histopathological analysis by 3D reconstruction and data extraction), a light-sheet microscopy-based approach for the analysis of tissue biopsies, is based on digital reconstruction and visualization of microscopic image stacks...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28814666/rapamycin-reversal-of-vegf-c-driven-lymphatic-anomalies-in-the-respiratory-tract
#13
Peter Baluk, Li-Chin Yao, Julio C Flores, Dongwon Choi, Young-Kwon Hong, Donald M McDonald
Lymphatic malformations are serious but poorly understood conditions that present therapeutic challenges. The goal of this study was to compare strategies for inducing regression of abnormal lymphatics and explore underlying mechanisms. CCSP-rtTA/tetO-VEGF-C mice, in which doxycycline regulates VEGF-C expression in the airway epithelium, were used as a model of pulmonary lymphangiectasia. After doxycycline was stopped, VEGF-C expression returned to normal, but lymphangiectasia persisted for at least 9 months...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28810590/diagnosis-and-surgical-treatment-of-cervical-macrocystic-lymphatic-malformations-in-infants
#14
Jing Ma, Ruan Biao, Fan Lou, Ken Lin, Ying-Qin Gao, Mei-Lan Wang, Yan-Li Yang, Tie-Song Zhang
The treatment of lymphatic malformations (LMs) represents a great clinical challenge. The present study reported on the treatment of 68 infants with cervical macrocystic LMs using surgical resection. The cases were retrospectively analyzed. All patients underwent pre-operative ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) examinations. The surgery was performed under general anesthesia with endotracheal intubation. Ultrasonograms showed that 24 cases were monolocular, 44 were multilocular, 16 had no echo, 20 had a uniform low-level echo and 32 had a non-uniform low-level echo...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28791278/venous-thromboembolism-in-pediatric-vascular-anomalies
#15
REVIEW
Taizo A Nakano, Chadi Zeinati
The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28782106/sirolimus-as-treatment-for-19-patients-with-refractory-cervicofacial-lymphatic-malformation
#16
Julie E Strychowsky, Reza Rahbar, Meghan J O'Hare, Alexandria L Irace, Horacio Padua, Cameron C Trenor
OBJECTIVE: Lymphatic malformations (LMs) are challenging to manage, particularly those involving the cervicofacial region and airway. Traditional therapy is sclerotherapy and/or resection. We aim to establish the emerging therapeutic role of sirolimus. STUDY DESIGN: Institutional review board-approved retrospective review. METHODS: All patients treated for cervicofacial LM with sirolimus at Boston Children's Hospital, Massachusetts, from November 2012 to October 2016 were included...
August 7, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28776595/role-of-intralesional-bleomycin-sclerotherapy-as-the-sole-or-adjunct-treatment-of-superficial-ocular-adnexal-lymphatic-malformations
#17
N Bothra, L Panda, J Sheth, D Tripathy
ObjectiveTo report the role of intralesional bleomycin as sole or adjunct therapy in the management of superficial ocular adnexal lymphatic malformations.DesignRetrospective study.ParticipantsThree patients/three eyes.MethodsRetrospective chart analysis of patients receiving intralesional bleomycin sclerotherapy as sole or adjunctive treatment for superficial ocular adnexal lymphatic malformations at a single tertiary level eye care institution.ResultsDegree of clinical regression of the lesions (graded by percentage) and resolution of symptoms and signs associated with the lesions...
August 4, 2017: Eye
https://www.readbyqxmd.com/read/28759318/the-public-health-burden-of-lymphatic-malformations-in-children-national-estimates-in-the-united-states-2000-2009
#18
Jeffrey Cheng, Beiyu Liu, Alfredo E Farjat, Jonathan Routh
OBJECTIVE: Describe inpatient resource utilization trends in children with lymphatic malformations using a national database. DATA SOURCE: Kids' inpatient database, years 2000-2009. METHODS: Subjects included children 18 years and under with International Classification of Diseases (ICD), 9th revision code: 228.1-lymphangioma, any site. Data elements were extracted and used to calculate related inpatient costs and trended over time. RESULTS: No significant increase in admission rates was noted over time, p = 0...
July 31, 2017: Lymphatic Research and Biology
https://www.readbyqxmd.com/read/28752523/dermoscopy-of-lymphangioma-circumscriptum-a-morphological-study-of-45-cases
#19
Pedro Zaballos, Luis Javier Del Pozo, Giuseppe Argenziano, Isil K Karaarslan, Christian Landi, Angel Vera, Alex Llambrich, Carolina Medina, Jose Bañuls
BACKGROUND/OBJECTIVES: The dermoscopy of lymphangioma circumscriptum, also known as superficial lymphatic malformation, remains to be clarified. METHODS: Digital dermoscopic images of 45 histopathologically confirmed cases of lymphangioma circumscriptum collected from nine hospitals in Spain, Italy and Turkey were evaluated for the presence of dermoscopic structures and patterns. RESULTS: Our study shows that the most common structure found in lymphangioma circumscriptum was the presence of lacunae (89% of cases)...
July 28, 2017: Australasian Journal of Dermatology
https://www.readbyqxmd.com/read/28746255/normalization-of-congenital-venous-stasis-retinopathy-following-sclerotherapy-of-a-macrocystic-lymphatic-malformation
#20
Kristin E Hirabayashi, Evan Kalin-Hajdu, Greg J Bever, M Reza Vagefi, Alejandra G de Alba Campomanes, Daniel L Cooke, Christopher F Dowd, Robert C Kersten
The authors describe, for the first time to their knowledge, a case of a congenital macrocystic lymphatic malformation of the orbit with associated venous stasis retinopathy that acutely normalized after drainage and sclerotherapy of the lesion. Prenatal ultrasound revealed prominence of the left orbital soft tissue, and at birth, the patient was noted to have unilateral proptosis, tortuous retinal vessels, and intraretinal hemorrhages in all 4 quadrants in the left eye. MRI demonstrated a primarily intraconal, multiloculated, T2-hyperintense mass consistent with a lymphatic malformation...
July 25, 2017: Ophthalmic Plastic and Reconstructive Surgery
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