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Megan K Mills, Bryn Putbrese, Hailey Allen, Sarah E Stilwill
Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported...
February 21, 2018: Skeletal Radiology
Sophie Turpin, Raymond Lambert
Objective: In the pediatric setting, lymphoscintigraphy is mostly used for the evaluation of lymphedema. Only a few cases of chylous anomalies and lymphatic malformations imaged with lymphoscintigraphy, have been reported in the literature. The aim of this study was to review the use of lymphoscintigraphy in those pathologies. Methods: All lymphoscintigraphies obtained between 2001 and 2017 in our hospital for chylous anomalies were retrospectively reviewed. Results were correlated to clinical and radiological findings...
February 2, 2018: Journal of Nuclear Medicine Technology
Eugenia Manevitz-Mendelson, Gil S Leichner, Ortal Barel, Inbal Davidi-Avrahami, Limor Ziv-Strasser, Eran Eyal, Itai Pessach, Uri Rimon, Aviv Barzilai, Abraham Hirshberg, Keren Chechekes, Ninette Amariglio, Gideon Rechavi, Karina Yaniv, Shoshana Greenberger
Generalized lymphatic anomaly (GLA or lymphangiomatosis) is a rare disease characterized by a diffuse proliferation of lymphatic vessels in skin and internal organs. It often leads to progressive respiratory failure and death, but its etiology is unknown. Here, we isolated lymphangiomatosis endothelial cells from GLA tissue. These cells were characterized by high proliferation and survival rates, but displayed impaired capacities for migration and tube formation. We employed whole exome sequencing to search for disease-causing genes and identified a somatic mutation in NRAS...
February 3, 2018: Angiogenesis
(no author information available yet)
No abstract text is available yet for this article.
January 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Ekim Gumeler, Mehmet Ruhi Onur, Ali Devrim Karaosmanoglu, Mustafa Ozmen, Deniz Akata, Musturay Karcaaltincaba
Peripelvic and periureteric areas are frequently overlooked in the imaging evaluations of the urinary system on computed tomography and magnetic resonance imaging. Several neoplastic and non-neoplastic disorders and diseases including lipomatosis, angiomyolipoma and angiolipomatous proliferation, vascular lesions, lymphangiomatosis, Rosai-Dorfman disease, Erdheim-Chester disease, extramedullary hematopoiesis, IgG4-related disease, lymphoma, mesenchymal tumors, trauma, and Antopol-Goldman lesion may involve these areas...
December 28, 2017: Abdominal Radiology
Xuhua Fang, Ziling Huang, Yu Zeng, Xuyou Zhu, Siqi Wang, Xiaoting Yu, Xian Li, Chunyan Wu, Xianghua Yi
BACKGROUND: Diffuse pulmonary lymphangiomatosis (DPL) mainly affects the lung and pleura. There are very few pathological reports of lung damage accompanied by diffuse involvement of the extrapulmonary lymph nodes and surrounding soft tissue. The clinicopathological significance of coexistence of pulmonary and extrapulmonary lesions is unknown. METHODS: Here, we report a 16-year-old male patient. The pathological specimens of the supraclavicular lymph node and soft tissue together with the lung biopsy were analyzed by pathological observation and immunohistochemical staining...
December 2017: Medicine (Baltimore)
Ana Rosa Tardáguila Calvo, José María Angulo Madero, Alberto Parente, Rosa María Romero, Susana Rivas
OBJECTIVE: Lymphangiomatosis is a rare disease affecting lymphatic vessels that causes a marked increase of them in the affected area. The final objective of treatment of the genital disease is to preserve sexual function and voiding with a satisfactory aesthetic result with the aim to minimize the emotional impact. METHODS: For the first time in children, we report a case of local reconstruction using artificial dermis after the excision of a genital lymphatic malformation in an eight year old patient...
December 2017: Archivos Españoles de Urología
Safa A Al-Shaikh, Aalaa M Mubarak, Zainab F Harb
Splenic lymphangiomas are exceedingly rare benign neoplasms that occur mainly in children. They are commonly seen in the neck and axillary region. Abdominal lymphangiomas accounts for less than 5% of cases. So far, fewer than 100 cases of spleen lymphangiomas have been reported in the literature. In this paper, we present one case of solitary splenic lymphangioma in a 34-year-old woman who presented with abdominal pain and weight loss for one month. The patient underwent splenectomy and diagnosis was confirmed by histopathology and immunohistochemistry studies...
November 2017: Saudi Medical Journal
Hemail M Alsubaie, Khaled M Alsubaie, Mohammed Eid Mahfouz
Teratomas are tumors composed of tissues derived from more than one germ cell line. They manifested with a great variety of clinical and radiological features. We report a case of a giant left hemithorax teratoma in a female with huge spleen tumor and review the relevant literature. A 38-year-old female with progressively aggravating dyspnea at rest from a mild trauma. Absent breath sounds on the left side. There was splenomegaly. Computed tomography scan revealed a huge mass (20 × 15 × 18 cm), containing elements of heterogeneous density in the left hemithorax...
September 2017: Journal of Surgical Case Reports
W He, Y C Hao, H Z Xia, R Z Ma, B Yang, J Lu
Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Hiroki Kato, Michio Ozeki, Toshiyuki Fukao, Masayuki Matsuo
PURPOSE: To assess the MR imaging findings of vertebral involvement in Gorham-Stout disease (GSD), generalized lymphatic anomaly (GLA), and kaposiform lymphangiomatosis (KLA). METHODS: Nine patients (three patients with GSD, three with GLA, and three with KLA) who underwent whole-spine MR examinations were included. MR findings of fatty marrow replacement and prolonged T1 and T2 lesions of the vertebrae were retrospectively assessed. Prolonged T1 and T2 lesions were defined as well-defined and round-, oval-, or geographic-shaped areas...
October 2017: Japanese Journal of Radiology
Jineesh Valakada, Kumble S Madhusudhan, Gyan Ranjan, Pramod Kumar Garg, Raju Sharma, Arun Kumar Gupta
No abstract text is available yet for this article.
April 12, 2017: Current Problems in Diagnostic Radiology
Chih-Ping Chen, Kuo-Gon Wang, Hsu-Kuang Huang, Cheng-Ran Peng, Schu-Rern Chern, Peih-Shan Wu, Yen-Ni Chen, Shin-Wen Chen, Chen-Chi Lee, Wayseen Wang
OBJECTIVE: We present cytogenetic and molecular cytogenetic diagnoses of mosaic deletion of chromosome 15q11.1-q11.2 in a fetus with diffuse lymphangiomatosis. CASE REPORT: A 33-year-old woman underwent amniocentesis at 22 weeks of gestation because of fetal diffuse lymphangiomatosis involving left-side chest, abdominal cavity, thigh and vulva, and intrauterine growth restriction. Amniocentesis revealed a karyotype of 46,XX,del(15) (q11.1q11.2)[9]/46,XX[26]. The mother had a karyotype of 46,XX...
April 2017: Taiwanese Journal of Obstetrics & Gynecology
Elżbieta Radzikowska, Katarzyna Błasińska-Przerwa, Małgorzata Szołkowska, Agnieszka Miąsko, Włodzimierz Kupis, Elżbieta Wiatr
No abstract text is available yet for this article.
June 15, 2017: American Journal of Respiratory and Critical Care Medicine
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
Guifang Lu, Hongxia Li, Yuanyuan Li
The present study reports the case of a 79-year-old Chinese man who presented to The First Affiliated Hospital of Xi'an Jiaotong University (Xi'an, China) for the treatment of lower gastrointestinal bleeding. Multiple cystic masses in the sigmoid colon were observed with colonoscopy, and through endoscopic ultrasound (EUS), these cystic masses were confirmed to be echo-free and to exhibit septal walls in the submucosal layer; in consequence, lymphangiomatosis of the sigmoid colon was diagnosed. Considering the repeated bleeding, laparoscopy-assisted partial sigmoid colon resection was performed...
January 2017: Oncology Letters
Maxim Itkin
Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions...
December 2016: Techniques in Vascular and Interventional Radiology
Timothy D Le Cras, Paula S Mobberley-Schuman, Mary Broering, Lin Fei, Cameron C Trenor, Denise M Adams
Vascular anomalies can cause significant morbidity and mortality. Advances in diagnosis will be improved if noninvasive biomarkers can be identified, as obtaining a tissue biopsy can worsen the disease and precipitate complications. The goal of this study was to identify biomarkers for vascular anomaly patients to aid diagnosis and potentially give insights into pathogenesis. Blood was collected at baseline and then 6 and 12 months after treatment with the mTOR inhibitor sirolimus. Patients groups included generalized lymphatic anomaly (GLA), kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) with or without the Kasabach-Merritt phenomenon (KMP) coagulopathy...
February 2017: Angiogenesis
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
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
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