Diagnosis of Lymphangiomatosis: A Study Based on CT Lymphangiography

Lymphangiomatosis is a rare disease characterized by the widespread presence of lymphangiomas in any part of the body. In previous studies, lymphatic vessel abnormalities in lymphangiomatosis have only rarely been mentioned. The objective of this paper is to discuss the imaging features of lymphangiomatosis, including cystic lesions and lymphatic abnormalities, on computed tomography lymphangiography (CTL).

All 34 patients who were diagnosed with lymphangiomatosis underwent direct lymphangiography followed by CTL. The CTL images were independently analyzed by two experienced radiologists. The CTL image analyses included assessment of the features of cystic lesions and lymphatic vessel abnormalities.

(i) CTL revealed several cystic lesions ranging in size from 3 mm to 14 cm; the lesions were located in the neck and shoulders (61.8%), mediastinum (52.9%), retroperitoneum (70.6%), and pelvis and perineum (64.7%). (ii) Approximately 29.4% of patients showed abnormal contrast medium accumulation in cystic masses. (iii) Approximately 67.6% of patients showed lymphatic reflux. (iv) Dilated lymphatic vessels were distributed in the mediastinum (38.2%), retroperitoneum (50.0%), pelvis and lower limbs (47.1%). (v) Finally, 8.8% of patients had perineal lymphatic fistulae, and 2.9% of patients had chyluria.

Lymphangiomatosis is a type of systemic lymphatic abnormality that is accompanied by multiple cystic lesions. The therapeutic measures for lymphangiomatosis are determined by the size of the cystic lesions. Furthermore, the prognosis of lymphangiomatosis is affected by lymphatic abnormalities.