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. Treatment was mainly palliative. Three patients died within 1/2 to three years of presentation.
Shah
A R
AR
Respiratory Unit, Hospitals for Sick Children, London, United Kingdom.
Dinwiddie
R
R
Woolf
D
D
Ramani
R
R
Higgins
J N
JN
Matthew
D J
DJ
D001847:Bone Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D002648:Child; D002916:Chylothorax / Q000209:etiology*; D006801:Humans; D007223:Infant; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008200:Lymphangiectasis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D008297:Male; D013158:Splenic Diseases / Q000000981:diagnostic imaging / Q000209:etiology; D014057:Tomography, X-Ray Computed