[Clinical analysis of two cases with diffuse pulmonary lymphatic disease]

To analyze the clinical characteristics and diagnosis of 2 cases with diffuse pulmonary lymphatic disease.

Clinical manifestations of the children were retrospectively analyzed. Two patients were both from Beijing Children Hospital in 2013 and 2014.Diffuse pulmonary lymphatic disease was diagnosed by pathology of pleura in case 1 and by lymphoscintigraphy in case 2.

The first patient was a male aged 10-year-5-month who presented with a history of pleural effusion for 2 years. Examination revealed chylothorax and pericardial effusion. After pleural decortications and ligation of thoracic duct was performed, he still had recurrent chylothorax. Pathology of pleura revealed lymphangioma. Physical examination showed diminished breath sound and heart sounds. A bronchoscope revealed too much white viscous secretions. Thorax ultrasound revealed lymphangioma in bilateral lower thoracic cavity and post inferior mediastinum. Chest computed tomography showed diffuse thickening of the interlobular septa and bronchovascular bundle in both lungs, many punctate calcification in the lower field. Abdomen ultrasound revealed a small quantity seroperitoneum. Lymphoscintigraphy revealed radioactivity enhancement in bilateral thoracic cavity. The second patient was a female aged 6 years and 9 months, who presented with a history of recurrent cough for 2 years and 9 months. Physical examination showed normal result. Pulmonary function showed mixed ventilation function disturbance. A bronchoscopy showed extensive viscous secretions. Mediastinum, heart and abdomen were normal on ultrasound. Chest computed tomography showed diffuse thickening of the interlobular septa and bronchovascular bundle in both lungs and bilateral pleural thickening. Lymphoscintigraphy revealed diffuse lymphangiectasis in both lungs. Both patients received a diagnosis of diffuse pulmonary lymphatic disease. Case 1 who had died was diagnosed with diffuse pulmonary lymphangiomatosis. Case 2 had no exacerbation after 9 months' treatment with prednisone.

Diffuse pulmonary lymphatic disease might present with cough and shortness of breath and result in diffuse interstitial disease with thickening of the interlobular septa, refractory chylothorax, multiple lymphangioma in mediastinum, pericardial effusion and seroperitoneum. It could be diagnosed by pleura or lung tissue pathology and lymphoscintigraphy.