Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Thoraco-abdominal lymphangiomatosis
- Radiologic Findings
- Fig 1-2. Lung window setting of the chest CT scan shows interlobular septal thickening of both lower lobes and right middle lobe.
Fig 3-5. Contrast-enhanced mediastinal window setting of the chest CT scan shows peribronchial hypodense soft tissue attenuation in both lower lobes and small-sized hypodense matted lymph nodes in both paratracheal regions.
Fig 6. 1 month later, the contrast-enhanced mediastinal window setting of the abdomen CT scan shows a small amount of right sided pleural effusion and ascites.
Fig 7-9. Unenhanced mediastinal window setting of chest CT scan obtained after a direct lymphangiography shows iodinated oil scattered in surrounding mediastinum as well as collapsed retroperitoneum, right middle lobe and lower lobes. A moderate to large amount of pleural effusion is also seen in right lung.
She underwent video-assisted thoracic surgery resection of right paracardiac lymph node which revealed mild vascular proliferation with nodular lymphoid aggregates. She also underwent diagnostic laparoscopy which yielded chyloperitoneum.
Fig. 7 After direct lymphangiography
Fig. 8 After direct lymphangiography
Fig. 9 After direct lymphangiography
- Brief Review
- Lymphangiomatosis is an uncommon disease characterized by the diffuse infiltration of lymphangiomas that can occur in any part of the body containing lymphatics. In particular, diffuse pulmonary lymphangiomatosis is caused by lymphangiomas limited to the thorax. The onset of the disease is insidious, and its clinical presentations are nonspecific (e.g., dyspnea, cough, chest pain, milky sputum, fever, shortness of breath, and wheezing); thus, diffuse pulmonary lymphangiomatosis is often misdiagnosed as other chronic respiratory disease. Though pathologic analysis is the main basis of diagnosis in diffuse pulmonary lymphangiomatosis, it is extremely difficult to obtain satisfactory pathologic results in a clinical setting, which can be attributed to the method and site of biopsy as well as the size of the biopsy specimens.
MDCT after direct lymphangiography is well suited to the diagnosis of diffuse pulmonary lymphangiomatosis because it is capable of clarifying the dilatation and proliferation of lymphatic channels to more clearly depict intrathoracic abnormalities. These abnormalities include abnormal distribution of contrast medium, dilatation of lymphatic channels, mediastinal soft-tissue infiltration, peribronchovascular thickening, interlobular septal thickening, diffuse ground-glass opacities, pleural effusion, extrapleural soft-tissue thickening, pericardial effusion and thickened pericardium, multiple lymphadenopathy in mediastinum, and mediastinal, pericardial, and thoracic aeroceles.
- Please refer to
Case 819, Case 833, Case 922, Case 1095, -
- References
- 1. Diffuse pulmonary lymphangiomatosis Can Respir J 2013;20(1):52-54
2. Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography AJR 2017;208:300–305
3. World J Gastroenterol 2014 July 7; 20(25): 8320-8324
- Keywords
- thorax, abdomen, pulmonary lymphangiomatosis, thoraco-abdominal lymphangiomatosis,