Generalized lymphangiomatosis and chylothorax in the pediatric age group

Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Trea...

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Bibliographic Details
Published inPediatric pulmonology Vol. 14; no. 2; p. 126
Main Authors Shah, A R, Dinwiddie, R, Woolf, D, Ramani, R, Higgins, J N, Matthew, D J
Format Journal Article
LanguageEnglish
Published United States 01.10.1992
Subjects
Bone Diseases - diagnostic imaging
Bone Diseases - etiology
Child
Chylothorax - etiology
Humans
Infant
Lung - diagnostic imaging
Lung - pathology
Lymphangiectasis - complications
Lymphangiectasis - diagnosis
Lymphangiectasis - pathology
Male
Splenic Diseases - diagnostic imaging
Splenic Diseases - etiology
Tomography, X-Ray Computed
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Summary:Four patients with generalized lymphangiomatosis presenting with chylothoraces are described. All four had bone involvement, two had involvement of the spleen, and one of the pericardium. The diagnosis was confirmed by typical radiology, histology, and in three patients by immunohistochemistry. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
ISSN:8755-6863
DOI:10.1002/ppul.1950140211