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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
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
Mahmoud Reza Khalatbari, Yashar Moharamzad
No abstract text is available yet for this article.
April 2016: Journal of Pediatric Neurosciences
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
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
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
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
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
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
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
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
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
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
Dan Levy Faber, Ronen Galili, Orna Nitzan, Erez Sharoni
No abstract text is available yet for this article.
December 2015: Israel Medical Association Journal: IMAJ
Aisheng Dong, Ling Zhang, Yang Wang, Tianlin He, Changjing Zuo
Kaposiform hemangioendothelioma (KH) is a rare vascular tumor of intermediate malignancy that occurs mainly in the childhood. Adult patients with KH are rare. Imaging findings of KH have rarely been reported before. We present magnetic resonance imaging (MRI), computed tomography (CT), and fluorine-18-fluorodeoxyglucose (F-FDG) positron emission tomography (PET)/CT findings in an adult patient with KH associated with lymphangiomatosis involving mesentery and ileum.A 22-year-old female complained of a 9-month history of intermittent melena, weakness, and palpitation...
February 2016: Medicine (Baltimore)
Michio Ozeki, Akihiro Fujino, Kentaro Matsuoka, Shunsuke Nosaka, Tatsuo Kuroda, Toshiyuki Fukao
BACKGROUND: Complex lymphatic anomalies are intractable lymphatic disorders, including generalized lymphatic anomaly (GLA), Gorham-Stout disease (GSD), and kaposiform lymphangiomatosis (KLA). The etiology of these diseases remains unknown and diagnosis is confused by their similar clinical findings. This study aimed to clarify the differences in clinical features and prognosis among GLA, KLA, and GSD, in Japanese patients. PROCEDURE: Clinical features, radiological and pathological findings, treatment, and prognosis of patients were obtained from a questionnaire sent to 39 Japanese hospitals...
May 2016: Pediatric Blood & Cancer
Cécile Herruela-Suffee, Mathilde Warin, Mélody Castier-Amouyel, Florine Dallery, Bruno Bonnaire, Jean-Marc Constans
OBJECTIVE: Generalized cystic lymphangiomatosis is a particularly rare disease with variable involvement of skeletal and extraskeletal sites. The key role of imaging in the diagnosis of this disease is no longer in doubt. The aim of our study was to demonstrate the contribution of whole-body magnetic resonance imaging (WB-MRI) at the diagnostic stage and during the follow-up in the pediatric population. SUBJECTS AND METHODS: Three children were included from 2008...
February 2016: Skeletal Radiology
Michio Ozeki, Akihiro Fujino, Kentaro Matsuoka, Shunsuke Nosaka, Toshiyuki Fukao
Lymphangiomatosis (recently renamed "generalized lymphatic anomaly") is a rare disease of unknown etiology that features an increase in the number of lymphatic vessels in many different tissues. Gorham-Stout disease(GSD) is a related disease characterized by lymphatic vessels involving the bones and resulting in progressive bone destruction. Respective definitions remain unclear because these conditions largely overlap in the clinical setting and are both associated with pleural effusion and other visceral lesions...
October 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
Hamidreza Jamaati, Bahareh Saeedi, Roya Farzanegan, Mohammad Behgam Shadmehr
Mediastinal cavernous lymphangioma is a rare mediastinal lesion and its association with lupus erythematosus has not yet been reported in the literature. We present a 25 year-old female with lupus erythematosus who had bilateral massive refractory and recurrent pleural effusion as well as ascites for a long period of time. During surgery, a huge multicystic lesion with a thick wall, covering the entire parietal and visceral pleura was found, which was subsequently proven to be a cystic cavernous lymphangioma...
2015: Tanaffos
Jingxi Zhang, Hai Jin, Yang Wang, Chong Bai, Yiping Han
Diffuse pulmonary lymphangiomatosis (DPL) is a rare interstitial lung disease characterized by intrathoracic lymphatic system abnormalities often with involvement of both lungs. Here, we report a 24-year-old male patient with DPL initially located in one lung, presenting only with transient fever. Resection of the right middle and lower lobes was performed for diagnosis and complete removal of the lesions. The pathologic features shown by diffuse smooth thickening of the interlobular septa, bronchovascular bundles, infiltration of patchy ground glass opacities and specific immunohistologic D2-40 and CD34 positive staining confirmed the diagnosis of DPL...
October 2015: Oxford Medical Case Reports
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