王仁贵：MDCT直接淋巴管造影在弥漫性肺淋巴管瘤病中的诊断价值ECR 2012 / C-0917Diagnostic value of multi-detector computed tomography direct-lymphangiography in diffuse pulmonary lymphangiomatosisCongress: ECR 2012Poster No.: C-0917Type: Scientific PaperKeywords: Thorax, Lymph nodes, Contrast agents, CT, Lymphography, PET-CT, Staging, Computer Applications-Detection,diagnosis, Contrast agent-other, Lymphoma, Pathology,EpidemiologyAuthors: R. WANG, W. Shen; Beijing/CNDOI: 10.1594/ecr2012/C-0917DOI-Link: /10.1594/ecr2012/C-0917PurposeThe aim of our study was to evaluate the usefulness of MDCT direct-lymphangiography in the diagnosis of diffuse pulmonary lymphangiomatosis.Methods and MaterialsBetween 1997 and 2011, we collected 18 patients with pathology-proven DPL examined by direct-lymphangiography MDCT. Two independent observers analyzed CT findings and recorded how frequently the imaging features were seen on direct-lymphangiography MDCT.▪ The intrathoracic abnormalities included :─ abnormal return and distribution of contrast medium─ the dilatation of lymphatic channels─ mediastinal soft tissue infiltration and enlargement─ peribronchovascular thickening─ interlobular septal thickening─ diffuse ground glass opacities─ pleural effusion and pleural thickening─ extra-pleural soft tissue thickening─ pericardial effusion and thickened pericardium─ multiple lymphadenopathy in mediastinum and axilla─ pneumo-thorax/mediastunum/pericardium▪ The associated extrathoracic abnormalities included :─ abnormal return and distribution of contrast medium─ the dilatation of lymphatic channels─ abdominal effusion─ retroperitoneal and splenic cystic lymphangioma─ cervical involvement or thoracic duct obstruction─ cervical lymphatic vessels dilation─ multiple small lymphadenopathy in axilla─ lymphedema of lower limb─ bone or vertebral lesionsResultsFig. 1: ▪ 7-year-old boy with DPL, showed chylothorax for 1 year. ▪ The CT of thorax with/without enhancement after direct- lymphangiophy showed diffuse mediastinal infiltrations with nomass effect on the vascular structures, abnormal distribution of contrast medium, smooth peribronchovascular thickening and right chylothorax. References: Radiology, Beijing Shijitan hospital , Capital Medical University - Beijing/CNFig. 2: The enhanced CT in mediastinal window showed diffuse mediastinal and peribronchovascular infiltrations and left chylothorax & pericardium. The CT in lung window showed smooth and diffuse thickening of peribronchovascular and interlobular septum with GGO-like background and no fibrosis. References: Radiology, Beijing Shijitan hospital , Capital Medical University - Beijing/CNFig. 3: ▪21-year-old woman with DPL. ▪The diffuse mediastinal and peribronchovascular infiltrations and abnormal distribution of contrast medium were showed on the MIP and MPR reconstructed imagings. References: Radiology, Beijing Shijitan hospital , Capital Medical University - Beijing/CNFig. 4: ▪ 16-year-old boy with chylothorax for 2 years. ▪ MDCT direct-lymphangiography showed abnormal distribution of contrast medium and thickening of bilateral pleura and extrapleural soft tissue, pleural effusion and GGO-like background of the lung. ▪ Multiple air focus were distributed in bilateral pleural space, extrapleural space, interlobar fissures and pericardium. References: Radiology, Beijing Shijitan hospital , Capital Medical University - Beijing/CNThe important CT patterns of intrathoracic abnormalities of DPL were abnormal distribution and accumulation of contrast medium (18/18 patients, 100%), the dilatation and proliferation of lymphatic channels (18/18, 100%), diffuse mediastinal soft tissue infiltration and enlargement without displacement of the vessels (18/18, 100%), bilateral peribroncovascular thickening (18/18, 100%), interlobular septal thickening (18/18, 100%),multifocal or diffuse ground glass opacities (17/18, 94.4%), pleural effusion (17/18, 94.4%), parietal and visceral pleural thickening and extra-pleural soft tissue thickening (11/18, 61.1%), pericardial effusion and thickened pericardium (16/18, 88.9%), multiple small lymphadenopathy in mediastinum and axilla (16/18, 88.9%), pneurmo-thorax(3/18, 16.7), pneumo-mediastunum(2/18, 11.1%), pneumo-pericardium (1/18, 5.6%). The other associated extrathoracic abnormalities included retroperitoneal lymphatic vessels dilation and return of lymphatic fluid(4/18, 22.2%), abdominal effusion (1/18, 5.6%), cervical involvement or thoracic duct obstruction(8/18, 44.4%), retroperitoneal solid cystic lesions(1/18, 5.6%), splenic lesions (1/18, 5.6%), bone or vertebral lesions(3/18, 16.7%).Table 1, CT findings of intrathoracic abnormalities of DPLCT findings(Intrathoracic abnormalities) Number(n=18)Percent(%)abnormal return and distribution of contrastmedium18 100%the dilatation of lymphatic channels 18 100% mediastinal soft tissue infiltration andenlargement18 100% peribronchovascular thickening 18 100% interlobular septal thickening 15 83.3% diffuse ground glass opacities 14 77.8% pleural effusion and pleural thickening 17 94.4% extra-pleural soft tissue thickening 11 61.1% pericardial effusion and thickened pericardium 16 88.9% multiple lymphadenopathy in mediastinum 8 44.4% pneumo-thorax/mediastunum/pericardium 4 22.2%Table 2, CT findings of extrathoracic abnormalities of DPLCT findings(Extrathoracic abnormalities) Number(n=18)Percent (%)retroperitoneal abnormal return of contrastmedium4 22.2% retroperitoneal and splenic cysticlymphangioma1 5.6% abdominal effusion 1 5.6%cervical lymphatic vessels dilation 8 44.4% cervical thoracic duct obstruction 8 44.4% multiple small lymphadenopathy in axilla 16 88.9% multiple small lymphadenopathy in neck 10 55.6% lymphedema of lower limb 1 5.6%bone or vertebral lesions 3 16.7% ConclusionDirect lymphangiography MDCT can observed clearly the lymphatic abnormal return and the dilatation and proliferation and infiltration of lymphatic channels showed by the abnormal distribution of contrast agent on MDCT direct-lymphangiography and the typical imaging characteristics are quite helpful in diagnosis of DPL.References1.Du minghua, Ye ruanjian, Sun kunkun, et al. Diffuse pulmonary lymphangiomatosis: a case report with literature review. Chinese Medcial Journal, 2011, 124(5):797-800.2.El Hajj L, Mazieres J, Rouquette I, et al. Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature. Clin Radiol, 2005, 60(3): 921-925.3.Ozeki M, Fukao T, Kondo N. Propranolol for intractable diffuse lymphangiomatosis.N Engl J Med. 2011, 364(4):1380-1382.4.Yekeler E, Dursun M, Yildirim A, et al. Diffuse pulmonary lymphangiomatosis: imaging fingdings. Diagn Interv Radiol, 2005, 11(3): 31-34.5.Tamay Z, Saribeyoglu E, Ones U, et al. Diffuse pulmonary lymphangiomatosis with disseminated intravascular coagulation in a child. J Pediatr Hematol Oncol, 2005, 27(12): 685-687.6.Wunderbaldinger P, Paya K, Partik B, et al. CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients. AJR Am J Roentgenol 2000, 174(3):827-832.7.Zisis C, Spiliotopoulos K, Patronis M, et al. Diffuse lymphangiomatosis: are there any clinical or therapeutic standards? J Thorac Cardiovasc Surg, 2007, 133(6):1664-1665.8.Alvarez OA, Kjellin I, Zuppan CW. Thoracic lymphangiomatosis in a child. J Pediatr Hematol Oncol. 2004, 26(2):136-141.9.Wiatr E, Langfort R, Orłowski T, et al. Chylothorax in patients with diffuse pulmonary lymphangiomatosis. Pneumonol Alergol Pol. 2006;74(2):209-215.Personal InformationRengui Wang, Professor and director of Radiologic center of Beijing Shijitan Hospital of Capital Medical University of China.My special interest is Diffuse lung diseases and tumors of Chest and pulmonary lymphtic diseases evaluated by X-ray, CT, MRI and PETCT.My email: ********************.cnFROM：/esr/viewing/index.php?module=viewing_poster&doi=10.1594/ecr2012/C-0917。
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