Thoracic Lymphangiomas, Lymphangiectasis,  Lymphangiomatosis, and Lymphatic  Dysplasia Syndrome

Faul, John L.; Berry, Gerald J.; Colby, Thomas V.; Ruoss, Stephen J.; Walter, Matthew B.; Rosen, Glenn D.; Raffin, Thomas A.

doi:10.1164/ajrccm.161.3.9904056

Cited by 370 publications

(367 citation statements)

References 121 publications

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“…Rarely do they occur in internal organs. About 1% of all lymphatic tumors occur in the chest; the heart is a particularly rare site [28,29]. The first time a heart lymphangioma was described was in 1911, by Armstrong [11].…”

Section: Discussionmentioning

confidence: 99%

Cardiac lymphangioma

Biskupski¹,

Waligórski²,

Mokrzycki³

et al. 2013

kitp

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StreszczenieLymphangioma to bardzo rzadki, pierwotny nowotwór serca. Rozpoznawany jest zwykle przypadkowo w badaniach autopsyjnych. Do tej pory wydano zaledwie kilkanaście publikacji dotyczących tego nowotworu. W pięciu przypadkach rozpoznano go przypadkowo, w pozostałych dawał objawy kliniczne. Wynikały one z ucisku na struktury serca lub z zaburzonego przepływu krwi przez serce. Najczęściej były to objawy niewydolności krążenia i zaburzenia rytmu serca. W diagnostyce najbardziej przydatne jest badanie echokardiograficzne przezklatkowe i przezprzełykowe (TTE, TEE), które obrazuje guz wraz z efektem hemodynamicznym. Ostateczne rozpoznanie ustalane jest zwykle na podstawie badania histopatologicznego preparatu po jego operacyjnym usunięciu. Leczeniem z wyboru jest chirurgiczne wycięcie guza. W pracy przedstawiono przypadek lymphangioma, który był zlokalizowany w świetle prawego przedsionka i powodował znaczne utrudnienie napły-wu krwi do prawej komory, co było przyczyną niewydolności krążenia. Omówiono także wszystkie znane opisy przypadków tego nowotworu, które dotyczyły serca. Słowa kluczowe: guz serca, echokardiografia, operacja. Kardiochirurgia i Torakochirurgia Polska 2013; 10 (3) 283 CASE REPORT AbstractLymphangioma is a very rare primary tumor of the heart. It is usually incidentally found during autopsies and there have only been a handful of documented cases of this tumor in the heart. In 5 cases, it was discovered accidentally and the remaining cases gave clinical symptoms. Major symptoms of heart lymphangiomas include compression of surrounding mediastinal structures and/or inadequate blood flow through the heart. Specifically the cardiac disturbances lead to rhythm and circulatory failure. The most useful diagnostic tests for this heart tumor include echocardiography, which shows not only the tumor but also its hemodynamic effects. The following article presents the accidental finding of a lymphangioma, localized in the right atrium. It caused considerable difficulty for the blood flow into the right heart chamber, which then led to circulatory failure. In addition we will also discuss all known cases of cardiac lymphangiomas.

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Section: Discussionmentioning

confidence: 99%

Cardiac lymphangioma

Biskupski¹,

Waligórski²,

Mokrzycki³

et al. 2013

kitp

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“…13 Lung biopsy is often necessary for confirmation. 14,15 Lymphangiomas are treated with surgical excision or sclerotherapy. 14 There are reports of the successful use of radiotherapy for cases in which chylothorax was refractory to surgical management.…”

Section: Causes Of Chylothoraxmentioning

confidence: 99%

“…14,15 Lymphangiomas are treated with surgical excision or sclerotherapy. 14 There are reports of the successful use of radiotherapy for cases in which chylothorax was refractory to surgical management. 16,17 Chylothorax can be a manifestation of Down, [18][19][20][21] Turner, [18][19][20] and Noonan syndromes.…”

Section: Causes Of Chylothoraxmentioning

confidence: 99%

Chylothorax in Infants and Children

2014

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Chylothorax, the accumulation of chyle in the pleural space, is a relatively rare cause of pleural effusion in children. It can cause significant respiratory morbidity, as well as lead to malnutrition and immunodeficiency. Thus, a chylothorax requires timely diagnosis and treatment. This review will first discuss the anatomy and physiology of the lymphatic system and discuss various causes that can lead to development of a chylothorax in infants and children. Then, methods of diagnosis and treatment will be reviewed. Finally, complications of chylothorax will be reviewed. Pediatrics 2014;133:722-733

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“…Although currently unused, congenital lymphatic anomalies have historically been classified according to their anatomopathological characteristics (3)(4)(5); however, these classifications sometimes overlap and are generally quite confusing. To obtain a homogeneous classification and to promote its use, the international society for the study of vascular anomalies (ISSVA) published a classification scheme in 1996, which was expanded and updated in 2014 (6).…”

Section: Introductionmentioning

confidence: 99%

Primary Intestinal Lymphangiectasia and its Association With Generalized Lymphatic Anomaly

Marugán

López‐Gutiérrez

2016

J Pediatr Rev

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Background: Lymph is a fluid originating in the interstitial spaces of the body that contains cells, proteins, particles, chylomicrons, and sometimes bacteria. Objectives: The aim of the present study is to demonstrate that primary intestinal lymphangiectasia (PIL) results from a disruption of lymphatic circulation, thus corresponding to a secondary rather than a primary event in the context of generalized lymphatic anomaly. Materials and Methods:In this case series and record review, an analysis of intestinal lymphatic involvement was performed on patients diagnosed with PIL between 1965 and 2013. Of the 21 patients included in the study, 10 had been diagnosed before 5 years of age (1 prenatal), 8 between 5 and 18 years of age, and 3 while older than 18 years of age. The follow-up period varied between 1 and 34 years. Clinical data, blood and fecal parameters, imaging studies, endoscopy results, biopsy analyses, treatment details, and outcome information were collected from medical records. Endoscopy, histological studies, magnetic resonance imaging, and lymphoscintigraphy were performed on all patients. Dynamic intranodal lymphangiography was performed on 8 patients. Results: Central lymphatic channel obstruction was identified in 12 patients (57%). Associated lymphatic malformation (LM) was present in 16, diarrhea in 10, chylothorax in 11, chylous ascites in 10, pericardial effusion in 6, coagulopathy in 3, and osteolysis in 7. Conclusions: We consider intestinal lymphangiectasia not as an entity in itself, but as a consequence of lymphatic flow impairment in the thoracic duct, producing chylous reflux into the intestinal lymphatics.

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Thoracic Lymphangiomas, Lymphangiectasis, Lymphangiomatosis, and Lymphatic Dysplasia Syndrome

Cited by 370 publications

References 121 publications

Cardiac lymphangioma

Cardiac lymphangioma

Chylothorax in Infants and Children

Primary Intestinal Lymphangiectasia and its Association With Generalized Lymphatic Anomaly

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