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https://www.readbyqxmd.com/read/27898632/point-of-care-thoracic-ultrasonography-in-the-diagnosis-and-management-of-kaposiform-lymphangiomatosis
#1
Manu S Raam, Ara Festekjian, Marsha A Elkhunovich
Kaposiform lymphangiomatosis is a generalized lymphatic disorder complicated by consumptive coagulopathy and pericardial and pleural effusions. We present the case of a 13-year-old female adolescent given a diagnosis of a large pleural effusion by point-of-care thoracic ultrasonography, which led to further evaluation and diagnosis of this rare disorder. We review the use of point-of-care thoracic ultrasonography for the diagnosis of pleural effusion.
December 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27857472/young-patient-with-generalized-lymphangiomatosis-differentiating-the-differential
#2
Tharani Putta, Aparna Irodi, Balamugesh Thangakunam, Ashwin Oliver, Rajesh Gunasingam
We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices...
July 2016: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/27845836/diffuse-pulmonary-lymphangiomatosis-mdct-findings-after-direct-lymphangiography
#3
Xiaoli Sun, Wenbin Shen, Song Xia, Tingguo Wen, Rengui Wang
OBJECTIVE: The aim of this study was to analyze the findings of MDCT performed after direct lymphangiography in patients with diffuse pulmonary lymphangiomatosis. MATERIALS AND METHODS: Twenty-three patients (13 male and 10 female patients) diagnosed with diffuse pulmonary lymphangiomatosis on the basis of clinical features and findings from imaging, bronchoscopy, and pathologic analysis were retrospectively evaluated. All patients underwent pulmonary MDCT after direct lymphangiography, surgical operation or open lung biopsy, and histopathologic examination...
November 15, 2016: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/27829197/gorham-disease-lymphangiomatosis-with-massive-osteolysis
#4
Ryan M Renacci, Roger J Bartolotta
Gorham disease (also referred to as vanishing bone disease or idiopathic massive osteolysis) is a rare skeletal condition that results in the localized replacement of bone with angiomas and fibrosis. The etiology and most effective treatment for this nonhereditary condition remain controversial in the medical literature. To our knowledge, we present the first case of post-traumatic Gorham disease that includes MR imaging (before and after radiation therapy), post-radiation CT with evidence of treatment effect (sclerosis), as well as asymptomatic bilateral renal lymphangiomata...
October 18, 2016: Clinical Imaging
https://www.readbyqxmd.com/read/27787381/cystic-angiomatosis-a-heterogeneous-condition-four-new-cases-and-a-literature-review
#5
Aurélie Najm, Elise Soltner-Neel, Benoît Le Goff, Pascale Guillot, Yves Maugars, Jean-Marie Berthelot
BACKGROUND: Cystic angiomatosis (CA) is a rare disorder causing bony cysts. It displays some similarity to Gorham-Stout disease (GSD), but has a much better local prognosis, despite the larger number of cysts. These 2 conditions also differ in terms of their location, visceral involvement, and response to treatment. METHODS: We report 4 cases of CA, including 1 sclerosing form, which we compare with cases from a literature review performed with PRISMA methodology...
October 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27784485/-diffuse-pulmonary-lymphangiomatosis-two-cases-report
#6
Q Y Shen, G M Nong, Y Y Gu
No abstract text is available yet for this article.
October 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27767681/percutaneous-transoral-clivoplasty-and-upper-cervical-vertebroplasties-for-multifocal-skeletal-lymphangiomatosis-resulting-in-complete-resolution-of-pain-case-report
#7
Christina Huang Wright, Dorian Kusyk, William S Rosenberg, Jennifer A Sweet
Lymphangiomatosis is a rare congenital disorder that results in multiorgan system lymphatic invasion. Symptoms due to axial skeletal involvement can range from chronic bone pain to severe deformity resulting in radiculopathy, myelopathy, and even paralysis. The authors present a case of lymphangiomatosis of the clivus, C-1, and C-2, resulting in chronic pain. The patient was successfully treated with percutaneous transoral clivoplasty and vertebroplasty, without disease progression or return of symptoms at 2 years...
October 21, 2016: Journal of Neurosurgery. Spine
https://www.readbyqxmd.com/read/27723921/sirolimus-in-the-treatment-of-vascular-anomalies
#8
Paloma Triana, Mariela Dore, Vanesa Nuñez Cerezo, Manuel Cervantes, Alejandra Vilanova Sánchez, Miriam Miguel Ferrero, Mercedes Díaz González, Juan Carlos Lopez-Gutierrez
Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM])...
October 10, 2016: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27708981/pregnancy-complicated-by-gorham-stout-disease-and-refractory-chylothorax
#9
Jessica Hellyer, Hunter Oliver-Allen, Majid Shafiq, Alisha Tolani, Maurice Druzin, Michael Jeng, Stanley Rockson, Robert Lowsky
Introduction Gorham-Stout Disease (GSD) is a rare disorder of bony destruction due to lymphangiomatosis, and is often triggered by hormones. One complication of GSD is the development of chylothorax, which carries a high mortality rate. Very little experience has been published to guide management in GSD during pregnancy to optimize both fetal and maternal health. Case Study A 20-year-old woman with known GSD presented with shortness of breath at 18 weeks of pregnancy, due to bilateral chylothoraces which required daily drainage...
October 2016: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/27606034/disseminated-spinal-lymphangiomatosis
#10
Mahmoud Reza Khalatbari, Yashar Moharamzad
No abstract text is available yet for this article.
April 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/27534888/primary-intestinal-lymphangiomatosis-of-the-ileum-in-an-adult-the-role-of-surgical-approach
#11
Mehmet Ilhan, Gizem Oner, Alpay Alibeyoglu, Gülçin Yeğen, Ali Fuat Kaan Gök, Filiz Akyüz, Fuat Bicen
Lymphangioma is a rare benign tumor that occurs due to abnormalities occurring during lymphatic development. It is usually seen in children and young adults. The incidence of lymphangiomas in the gastrointestinal tract is very low. Here we describe the case of 43-year-old woman with lymphangioma of the ileum with infiltrative polyposis-like appearing lesions diagnosed by capsule endoscopy and treated with segmental resection of affected intestinal part with laparotomy. Lesions involving mesentery and ileum were confirmed by pathology...
2016: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/27450410/disorders-of-the-lymphatic-system-of-the-abdomen
#12
REVIEW
A R Patil, S Nandikoor, J De Marco, R Bhat, S Shivakumar, G Mallrajapatna
The lymphatic system of the abdomen comprises of the cisterna chyli, its major and minor lymphatic tributaries, and lymph nodes. Disorders of the lymphatic system of the abdomen are rarely encountered and consist of primary and secondary types. Abdominal lymphangiomas constitute the majority and have characteristic imaging features. Complicated lymphangiomas may pose a diagnostic dilemma. Generalised systemic lymphangiomatosis is a rare condition and affects major organs with a poor prognosis. Retroperitoneal lymphangiectasia in the appropriate setting might predict underlying infection, such as filariasis...
October 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27386481/disseminated-lymphangiomatosis-presenting-as-chylous-ascites-and-diagnosed-with-endoscopic-ultrasound
#13
Surinder Singh Rana, Puneet Chhabra, Vishal Sharma, Nadeem Pervez, Ravi Sharma, Radhika Srinivasan, Deepak Kumar Bhasin
No abstract text is available yet for this article.
May 2016: Endoscopic Ultrasound
https://www.readbyqxmd.com/read/27266826/emergency-presentation-of-cystic-lymphangioma-of-the-colon-a-case-report-and-literature-review
#14
Luca Lepre, Gianluca Costa, Daniela Baldini, Francesco Cortese, Alessandra Saputelli, Aldo Gioffre, Pietro Fransvea
INTRODUCTION: Colonic lymphangioma is an unusual benign malformation. The clinical presentation of lymphangiomas vary from incidental discovery on imaging to presenting with acute abdomen. PRESENTATION OF CASE: We present the case of a 73-year-old male, undergoing surgery due to acute abdomen associated with severe anemia, in whose case a lymphangioma of the cecum was recognised only in the postoperative histopathological examination. DISCUSSION: The management of colonic lymphangioma depends on the individual situation; close surveillance or endoscopic therapy may be appropriate for asymptomatic lesions smaller than 2...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27246594/thoracic-involvement-in-generalised-lymphatic-anomaly-or-lymphangiomatosis
#15
REVIEW
Francesca Luisi, Olga Torre, Sergio Harari
Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches...
June 2016: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27212994/case-report-of-solitary-giant-hepatic-lymphangioma
#16
Hwan Hyo Lee, Seon Youl Lee
A hepatic lymphangioma is a rare benign neoplasm that is usually associated with systemic lymphangiomatosis. A solitary hepatic lymphangioma is extremely rare. Therefore, we present a rare case of a female patient who underwent right hepatectomy for solitary giant hepatic lymphangioma. A 42-year-old female presented to the emergency department with complaint of severe abdominal pain of the right upper quadrant. Abdominal computed tomography showed an approximately 23×30-cm sized, giant, relatively well-defined, homogenous cystic mass with few septa in the right liver (segments VII and VIII)...
May 2016: Korean Journal of Hepato-biliary-pancreatic Surgery
https://www.readbyqxmd.com/read/27143078/-clinical-analysis-of-two-cases-with-diffuse-pulmonary-lymphatic-disease
#17
J R Liu, W B Shen, Z Wen, R An, C J Zhou, S Y Zhao
OBJECTIVE: To analyze the clinical characteristics and diagnosis of 2 cases with diffuse pulmonary lymphatic disease. METHOD: Clinical manifestations of the children were retrospectively analyzed. Two patients were both from Beijing Children Hospital in 2013 and 2014.Diffuse pulmonary lymphatic disease was diagnosed by pathology of pleura in case 1 and by lymphoscintigraphy in case 2. RESULT: The first patient was a male aged 10-year-5-month who presented with a history of pleural effusion for 2 years...
May 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27127767/pathology-analysis-of-a-rare-case-of-diffuse-pulmonary-lymphangiomatosis
#18
Jin Zhao, Ruibin Wu, Yingying Gu
Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by uncontrolled proliferation of lymphatic vessels. Histologically benign, however, it can lead to death because of its progression. In this paper, we would like to present an unusual case of diffuse pulmonary lymphangiomatosis involving a 28-year-old young female who was suffered chest pain and polypnea for one year, and also a lot of chylous effusion in left chest. Lymphoma and lymphangitic metastasis was the primary diagnosis in other hospitals...
March 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/27059187/imatinib-treatment-of-lymphangiomatosis-generalized-lymphatic-anomaly
#19
Laura J Libby, Navneet Narula, Helen Fernandes, James F Gruden, David J Wolf, Daniel M Libby
Lymphangiomatosis (eg, generalized lymphatic anomaly) is an abnormal proliferation of lymphatic endothelial cells. It is often a childhood disease, but it may present in adulthood by infiltrating organs and cause obstruction, bleeding, or disruption of lymphatic flow. Pulmonary involvement may be mild or cause diffuse interstitial lung disease, airway obstruction, hemoptysis, chylothorax, chylopericardium, and culminate in respiratory failure. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy...
April 2016: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/27053281/imaging-features-of-kaposiform-lymphangiomatosis
#20
Pradeep Goyal, Ahmad I Alomari, Harry P Kozakewich, Cameron C Trenor, Antonio R Perez-Atayde, Steven J Fishman, Arin K Greene, Raja Shaikh, Gulraiz Chaudry
BACKGROUND: Kaposiform lymphangiomatosis is a rare, aggressive lymphatic disorder. The imaging and presenting features of kaposiform lymphangiomatosis can overlap with those of central conducting lymphatic anomaly and generalized lymphatic anomaly. OBJECTIVE: To analyze the imaging findings of kaposiform lymphangiomatosis disorder and highlight features most suggestive of this diagnosis. MATERIALS AND METHODS: We retrospectively identified and characterized 20 children and young adults with histopathological diagnosis of kaposiform lymphangiomatosis and radiologic imaging referred to the vascular anomalies center between 1995 and 2015...
August 2016: Pediatric Radiology
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