Case report |
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Hussam K. Hamadah Section of Pediatric Cardiac ICU, King Abdulaziz Cardiac Center, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Mail Code: 1423, P.O Box 22490, Riyadh 11426 Kingdom of Saudi ArabiaE-mail: hamadahmo@ngha.med.sa |
Hussam K. Hamadah
Section of Pediatric Cardiac ICU, King Abdulaziz Cardiac Center, King Abdulaziz Medical City, Ministry of National Guard-Health Affairs, Mail Code: 1423, P.O Box 22490, Riyadh 11426 Kingdom of Saudi ArabiaGraph 1: A 20-month-old boy with diffuse lymphangiomatosis who presented with chylothorax and chylopericardium; effect of therapy with pegylated interferon alfa-2b and other medications on drainage volume with correlated images (chest X-ray [CXR] and echocardiography). (a) CXR on presentation revealed cardiomegaly with the right pleural effusion, and echocardiography apical four chambers view is consistent with early signs of tamponade. (b) CXR and subcostal echocardiography view revealed significant effusion in the pleural and pericardial space in spite of drains. The decline in drainage volume before pericardial window and pleuroperitoneal shunt was due to frequent drains obstructions and not due to clinical improvement. (c) CXR and subcostal echocardiography view after pericardial window and pleuroperitoneal shunt revealed no further effusion. (d) CXR and apical four chambers echocardiography on discharge revealed no effusion after removal of chest tubes in the presence only of pleuroperitoneal shunt.
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Figure 1: A 20-month-old boy with diffuse lymphangiomatosis who presented with respiratory failure. (a) Chest computed tomography (CT) with contrast demonstrates diffuse smooth interstitial thickening, multiple patchy air space consolidations mainly in the left; bony lytic lesions involving the ribs bilaterally (arrows); evidence of pericardial effusion and bilateral pleural effusion. (b) Abdomen CT with contrast revealed heterogeneous enhancement of the spleen(s) with multiple tiny cysts. |
Figure 1: A 20-month-old boy with diffuse lymphangiomatosis who presented with respiratory failure. (a) Chest computed tomography (CT) with contrast demonstrates diffuse smooth interstitial thickening, multiple patchy air space consolidations mainly in the left; bony lytic lesions involving the ribs bilaterally (arrows); evidence of pericardial effusion and bilateral pleural effusion. (b) Abdomen CT with contrast revealed heterogeneous enhancement of the spleen(s) with multiple tiny cysts.
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Figure 2: A 20-month-old boy with diffuse lymphangiomatosis diagnosed by pleural biopsy. (a) Pleura histological section revealed focal reactive hyperplastic lymphoid follicles (arrows) surrounded by lymphangiectatic vessels with the characteristic lymphangitic distribution of abnormally dilated lymphatic spaces. (b) The immunohistochemical staining revealed abnormal lymphatic channels (arrows) positive for the lymphatic endothelial antigen recognized by D2-40 (podoplanin). |
Figure 2: A 20-month-old boy with diffuse lymphangiomatosis diagnosed by pleural biopsy. (a) Pleura histological section revealed focal reactive hyperplastic lymphoid follicles (arrows) surrounded by lymphangiectatic vessels with the characteristic lymphangitic distribution of abnormally dilated lymphatic spaces. (b) The immunohistochemical staining revealed abnormal lymphatic channels (arrows) positive for the lymphatic endothelial antigen recognized by D2-40 (podoplanin).
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