title: Computed tomography lymphangiography findings in 27 cases of lymphangioleiomyomatosis.
abstract: Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema. The CT lymphangiography showed that 27/27 (100%) patients presented iliac or retroperitoneal lymphatic vessel dilation; 20/27 (74%) presented thoracic duct outlet obstruction; 9/27 (33%) presented pleural lymph leakage; and 13/27 (48%) presented an abnormal distribution of contrast agent in the contralateral lumbar trunk, 3/27 (11%) in the pulmonary field, 1/27 (4%) in the abdominal cavity, and 4/27 (15%) in the pelvic cavity. Five of the 27 patients (19%) had retroperitoneal lymphangioleiomyomas, with contrast agent accumulation in three cases during CT lymphangiography. Two of the 27 (7%) patients had enlarged lymph nodes in the retroperitoneum. All 27 patients (100%) diagnosed with LAM displayed various lung cysts on thoracic high-resolution CT scans. Conclusion CT lymphangiography findings can be used to diagnose most LAM cases accurately. Moreover, these findings also display the various lymphatic vessel abnormalities associated with LAM.
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Chunyan
authors.initials: C
authors.lastname: Zhang
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Xiaobai
authors.initials: X
authors.lastname: Chen
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Tingguo
authors.initials: T
authors.lastname: Wen
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Qijin
authors.initials: Q
authors.lastname: Zhang
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Meng
authors.initials: M
authors.lastname: Huo
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Jian
authors.initials: J
authors.lastname: Dong
authors.affiliation: 2 Department of Lymph Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Wen-Bin
authors.initials: WB
authors.lastname: Shen
authors.affiliation: 1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.
authors.forename: Rengui
authors.initials: R
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging*; D042601:Lymphatic Vessels / Q000000981:diagnostic imaging; D008220:Lymphography / Q000379:methods; D008875:Middle Aged; D015203:Reproducibility of Results; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult
keywords: Computed tomography (CT); X-ray; chylothorax; lymphangiography; lymphangioleiomyomatosis (LAM); thoracic duct