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.

Rozpoznanie i leczenie limfangioleiomiomatozy (LAM) z grupy PEComa

“…Single cases have been described of the occurrence of the disease in children and men without predisposing genetic factors [14][15][16]. Extrapulmonary perturbations within the lymphatic system are found often in patients with the pulmonary LAM form -perturbations in the patency of the thoracic duct are found in over 70%, lymph exudate in the retroperitoneal space is observed in 30% of the cases [17]. Lymphangioleiomyoma is less common -in 10-21% of patients with pulmonary S-LAM [12,[17][18][19] and is often accompanied by a swelling of lymph nodes within the abdominal cavity, ascites and a broadening of the thoracic duct [18].…”

“…Extrapulmonary perturbations within the lymphatic system are found often in patients with the pulmonary LAM form -perturbations in the patency of the thoracic duct are found in over 70%, lymph exudate in the retroperitoneal space is observed in 30% of the cases [17]. Lymphangioleiomyoma is less common -in 10-21% of patients with pulmonary S-LAM [12,[17][18][19] and is often accompanied by a swelling of lymph nodes within the abdominal cavity, ascites and a broadening of the thoracic duct [18]. It seems that the frequency of occurrence of lymphangioleiomyoma in patients with the pulmonary form of LAM increases with the severity of its course [18], but they are not more common in patients with tuberous sclerosis -in a retrospective analysis from the Mayo Clinic, among 403 patients with TSC, E-LAM occurred in only 3 [20].…”

“…Solid tumors have a density similar to the liver (59-71 HU), cyst-like lesions a lower one , corresponding to lymph accumulation [18]. The cyst walls may become more pronounced after contrast [17]. Lymphangioleiomyoma under magnetic resonance shows differentiated values of signal both in T1 and in T2 exposure [38,44,49,50].…”

The roles of iodized oil-based lymphangiography and post-lymphangiographic computed tomography for specific lymphatic intervention planning in patients with postoperative lymphatic fistula: a literature review and case series

“…120 kV p with adapted tube current for thorax). The reconstruction of slice thickness and increment was mostly set up as 1 or 2 mm for better spatial resolution (Ginat et al 2009;Safar et al 2011;Yamada et al 2017;Zhang et al 2017). Except for the axial images, maximum intensity projection (MIP) reformatted imaging with three axes orientations and 3-dimensional volume rendering Fig.…”

Recent advances in the management of lymphangioleiomyomatosis

“…The clinical effects and prognosis associated with chylothorax vary considerably 43 , 45 . Several different types of lymphatic abnormalities associated with chylothorax have been identified by CT and lymphangiography 46 . Iliac or retroperitoneal lymphatic vessel dilation and obstruction of the thoracic duct are most commonly observed.…”