E2448. The Spectrum of Imaging Manifestations of Gorham–Stout Disease
Authors
Seunghyun Lee;
Seoul National University Hospital
Yuna Lee;
Seoul National University Hospital
Young Hun Choi;
Seoul National University Hospital
Jung-Eun Cheon;
Seoul National University Hospital
Yeon Jin Cho;
Seoul National University Hospital
Seul Bi Lee;
Seoul National University Hospital
Objective:
To describe the radiological features of Gorham–Stout disease (GSD) as evaluated using plain radiography and dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) imaging techniques.
Materials and Methods:
Clinical and conventional imaging data were retrospectively reviewed for 15 patients with GSD between January 2001 and December 2020. After December 2018, DCMRL examinations were performed for lymphatic vessel evaluation in patients with GSD and reviewed in four patients.
Results:
The median age at diagnosis was 9 years (range: 2 months–53 years). The clinical manifestations were dyspnea in seven patients (46.7%), sepsis in 12 (80.0%), orthopedic problems in seven (46.7%), and bloody chylothorax in seven (46.7%). The common sites of osseous involvement were the spine (73.3%) and pelvic bone (60.0%). Among the nonosseous involvements, periosseous infiltrative soft-tissue abnormalities adjacent to the area of bone involvement were the most common (86.7%), followed by splenic cysts (26.7%) and interstitial thickening (26.7%). DCMRL demonstrated weak central conducting lymphatic flow in two patients with abnormal giant tortuous thoracic ducts and no flow in one patient. All patients who underwent DCMRL in this study presented with altered anatomical lymphatics and functional flow with collateralization.
Conclusion:
DCMRL imaging and plain radiography are very useful for determining the extent of GSD and are essential for diagnosis. DCMRL is an essential imaging tool for the visualization of abnormal lymphatics in patients with GSD, which helps in further treatment. Therefore, in patients with GSD, it is necessary to obtain not only plain radiographs but also MR and DCMRL images.
