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Diffuse Pulmonary Lymphangiomatosis in Middle-Aged Patient

Rare case report of diffuse pulmonary lymphangiomatosis in a 33-year-old female with symptoms, imaging findings, differential diagnosis, treatment options, and prognosis discussed at a monthly chest imaging conference.

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Diffuse Pulmonary Lymphangiomatosis in Middle-Aged Patient

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  1. 2014년 11월 월례 집담회 흉부영상의학회 3월 월례 집담회 서울아산병원 서울아산병원 최원진

  2. Case #1 F/33 흉부영상의학회 3월 월례 집담회 서울아산병원 C.C.: Dyspnea (onset: 4 months ago) Cough (onset: 3 months ago) Hx.: Tx. of tuberculosis (2004, 2008)

  3. 2014-09-04

  4. 2013-10-14

  5. 2014-09-04

  6. 흉부영상의학회 3월 월례 집담회 What is the diagnosis? 서울아산병원

  7. 2014-09-22 Wedge resection, VATS ; RUL and RML 흉부영상의학회 3월 월례 집담회 H&E x40 CD31 (+) 서울아산병원 H& E x200 D2-40 (+)

  8. Diffuse pulmonary lymphangiomatosis (DPL) • Introduction • - very rare lymphatic disorder characterized by lymphatic channel proliferation • - mostly in children and young adults, progressive disease course • Symptom • - dyspnea, nonproductive cough, bronchial casts, milky sputum, fever, and recurrent pneumonia • Imaging findings • - CT (suggestive but not pathognomic) • smooth interlobular septal and peribronchiovascular thickening • patchy bilateral ground glass opacities • may upper lobe predominance • pleural thickening, pleural effusion, and mediastinal infiltration 흉부영상의학회 3월 월례 집담회 서울아산병원

  9. Diffuse pulmonary lymphangiomatosis (DPL) • Differential diagnosis • - pulmonary edema, pulmonary veno-occlusive disease, Erdheim-Chester disease, lymphangiectasis, lymphangitic carcinomatosis, sarcoidosis, pulmonary lymphoma • Treatment • - no established treatment for DPL • - surgical resections: indicated for localized mediastinal or lung lesions • - others: low-fat medium chain triglyceride diets, interferon-alpha, radiation, corticosteroids, chemotherapy, somatostatin, and propranolol • Prognosis • - generally poor • - often have a progressive course leading to respiratory failure and death in pediatric populations 흉부영상의학회 3월 월례 집담회 서울아산병원

  10. A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman. (Korean J Radiol 2014;15(2):295-299) Diffuse pulmonary lymphangiomatosis. (Can Respir J 2013;20(1):52-54.)

  11. (Left) Hematoxylin and eosin-stained section of lesion showing proliferation of thin-walled, anastomosing lymphatic vessels lined by single layer of endothelial cells lacking cytological atypia (arrows, × 200). • (Right) Immunohistochemical staining with D2-40 revealing proliferative lymphatic channels (arrows, × 200). A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman. (Korean J Radiol 2014;15(2):295-299)

  12. Case #2 F/58 흉부영상의학회 3월 월례 집담회 서울아산병원 C.C.: arrest after severe abdominal pain  CPCR (POD 5) s/p septal myectomy, MVP, PM & RVOT relieve op. for HCMP, SAM, RVOT obs.

  13. 흉부영상의학회 3월 월례 집담회 What is the problem? 서울아산병원

  14. 2014-07-30 POD 5 2014-07-07 Preop.

  15. Peripheral VA ECMO Imaging adults on extracorporeal membrane oxygenation (ECMO). (Insights Imaging 2014; 1-12)

  16. Type : veno-arterial (VA) and veno-venous (VV) • VA ECMO: deoxygenated blood from a vein with return of oxygenated blood into an arterial vessel • - direction of the aortic bloodstream is reversed, flowing from the caudal to the cephalad region • VV ECMO: deoxygenated blood from a venous vessel and return of oxygenated blood to a systemic venous vessel or RA • - aortic bloodstream flows in the normal direction Imaging adults on extracorporeal membrane oxygenation (ECMO). (Insights Imaging 2014; 1-12)

  17. 54-year-old male underwent VA-ECMO due to cardiogenic shock. arterial phase venous phase Multislice CT Scans in Patients on Extracorporeal Membrane Oxygenation: Emphasis on Hemodynamic Changes and Imaging Pitfalls. (Korean J Radiol 2014;15(3):322-329)

  18. 35-year-old male had pneumonia complicated by acute respiratory distress syndrome and underwent VV-ECMO for oxygenating support. Multislice CT Scans in Patients on Extracorporeal Membrane Oxygenation: Emphasis on Hemodynamic Changes and Imaging Pitfalls. (Korean J Radiol 2014;15(3):322-329)

  19. Thank you for your attention !

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