Publications
Detailed Information
The spectrum of imaging manifestations of Gorham–Stout disease: a novel dynamic contrast-enhanced MR lymphangiography
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Lee, Yuna | - |
dc.contributor.author | Lee, Seunghyun | - |
dc.contributor.author | Hur, Saebeom | - |
dc.contributor.author | Jeong, Yun Soo | - |
dc.contributor.author | Suh, Dong In | - |
dc.contributor.author | Moon, Jangsup | - |
dc.contributor.author | Kim, Man Jin | - |
dc.contributor.author | Choi, Young Hun | - |
dc.contributor.author | Cheon, Jung-Eun | - |
dc.date.accessioned | 2023-05-16T02:27:07Z | - |
dc.date.available | 2023-05-16T11:28:21Z | - |
dc.date.issued | 2023-04-26 | - |
dc.identifier.citation | Orphanet Journal of Rare Diseases, 18(1):96 | ko_KR |
dc.identifier.issn | 1750-1172 | - |
dc.identifier.uri | https://hdl.handle.net/10371/192423 | - |
dc.description.abstract | Background
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. 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 non-osseous involvements, peri-osseous 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. DCMRL is a novel imaging tool for the visualization of abnormal lymphatics in patients with GSD, which helps in further treatment. Therefore, in patients with GSD, it might be necessary to obtain not only plain radiographs but also MR and DCMRL images. | ko_KR |
dc.description.sponsorship | This research was supported by the Basic Science Research Program of the National Research Foundation of Korea (Grant No. NRF-2018R1C1B6007875), funded by the Ministry of Science, ICT, and Future Planning. The funder had no involvement or influence whatsoever in the study design at any stage, collection of the data or its analysis and interpretation, writing and preparation of the manuscript, or its submission for publication. | ko_KR |
dc.language.iso | en | ko_KR |
dc.publisher | BMC | ko_KR |
dc.subject | Gorham–Stout disease | - |
dc.subject | Osteolysis | - |
dc.subject | Lymphatic malformation | - |
dc.subject | Angiomatous lesion | - |
dc.subject | Dynamic contrast-enhanced magnetic resonance lymphangiography | - |
dc.title | The spectrum of imaging manifestations of Gorham–Stout disease: a novel dynamic contrast-enhanced MR lymphangiography | ko_KR |
dc.type | Article | ko_KR |
dc.identifier.doi | 10.1186/s13023-023-02704-7 | ko_KR |
dc.citation.journaltitle | Orphanet Journal of Rare Diseases | ko_KR |
dc.language.rfc3066 | en | - |
dc.rights.holder | The Author(s) | - |
dc.date.updated | 2023-04-30T03:12:50Z | - |
dc.citation.number | 96 | ko_KR |
dc.citation.volume | 18 | ko_KR |
- Appears in Collections:
- Files in This Item:
Item View & Download Count
Items in S-Space are protected by copyright, with all rights reserved, unless otherwise indicated.