Disseminated lymphangiomatosis presenting with massive chylothorax

Abstract

We suggest that MRI may be helpful to assess the extent of this disease more accurately.

Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

“…In systemic disease, LAVERDIERE et al [45] described cystic lesions with linear opacities in both lungs. In another case of diffuse systemic lymphangiomatosis, massive chylothorax was reported without pulmonary parenchymal infiltrations [46].…”

High-Resolution CT of the Lung in Children: Clinical Applications

“…Chest involvement in Gorham's or ''vanishing bone'' disease, in which there is replacement of a single or several contiguous bones by lymphangiomatous tissue, may present with severe and progressive osteolysis associated with septal thickening (Fig. 20) and chylothorax (Konez et al 2000). The presence of chylous pleural effusion usually indicates a poor prognosis (Yang and Goo 2006).…”

Chylothorax in Infants and Children

“…2 Diagnosis of pulmonary lymphangiomatosis and lymphangiectasia can be made by lymphoscintigraphy, 11 computerized tomography, 12 or MRI. 13 Lung biopsy is often necessary for confirmation. 14,15 Lymphangiomas are treated with surgical excision or sclerotherapy.…”

“…100,101 MRI has been used in at least 1 case of disseminated lymphangiomatosis in a child to help assess the extent of the disease. 13 If direct visualization of a chyle leak point is needed, thoracoscopy can be useful. It also allows for biopsies of any suspect areas.…”