Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome.
Noonan's syndrome is a rare congenital disorder that may be associated with abnormalities in the lymphatic drainage. In this case of a 21-year-old man CT after bipedal lymphangiography confirmed the diagnosis of intestinal lymphangiectasy causing protein-losing enteropathy in Noonan's syndrome by showing contrast-enhanced abnormal lymphatic vessels in the mesentery and the intestinal wall. Because of the benefit of diet in case of intestinal involvement, we recommend a thorough documentation of the lymphatic drainage with lymphangiography followed by CT, if clinical signs of lymphatic dysplasia, such as pleural effusions, lymphedema, or hypoproteinemia are present.
Keberle
M
M
Department of Radiology, Universität Würzburg, Germany.
Mörk
H
H
Jenett
M
M
Hahn
D
D
Scheurlen
M
M
D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000150:complications / Q000000981:diagnostic imaging*; D008220:Lymphography; D008297:Male; D009634:Noonan Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D011504:Protein-Losing Enteropathies / Q000000981:diagnostic imaging* / Q000209:etiology; D014057:Tomography, X-Ray Computed