title: Computed tomography after lymphangiography in the diagnosis of intestinal lymphangiectasia with protein-losing enteropathy in Noonan's syndrome.
abstract: 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.
authors.affiliation: Department of Radiology, Universität Würzburg, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Keberle
authors.forename: H
authors.initials: H
authors.lastname: Mörk
authors.forename: M
authors.initials: M
authors.lastname: Jenett
authors.forename: D
authors.initials: D
authors.lastname: Hahn
authors.forename: M
authors.initials: M
authors.lastname: Scheurlen
mesh_terms: 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