9 yo M with recurrent chylothorax, chylous effusion, and multiple lytic bone lesions.



Lymphangiomatosis

Lymph - of the lymphatic system
Angio - of vessels
Oma - a tumor, cyst, or neoplasm
Tosis - a condition

Is thought to be a result of congenital errors of lymphatic development occurring prior to the 20th week of gestation. It may involve a single organ or multiple organs and is most common in the bones and lungs. Chylous effusion can occur in the pleural, pericardial, and peritoneal cavities with danger of visceral compression.


May be asymptomatic at first. Bone involvement may be wrongly attributed to "growing pains" in children. Initial presentation may be a pathologic fracture.

Associated with lytic lesions of the bone, pulmonary interstitial infiltrates, chylous effusions.

Incidence: Can occur at any age. Highest incidence in children and teenagers.

Prognosis: Isolated presentation usually better than multi-organ involvement.

Micro: Increase in the size and number of thin walled lymphatic channels along with lymphatic spaces that are interconnecting and dilated, lined by a single attenuated layer of endothelial cells.