Generalised lymphangiomatosis in an 8-year-old girl who presented with cardiomegaly

Abstract

Generalised lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. The computed tomography, ultrasonography and magnetic resonance images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung, and lower neck. We report here a case that was referred to us for investigation of marked cardiomegaly.

Lymphangiomatosis with Extensive Skeletal Involvement

“…This progressive disorder, results from a proliferation of lymphatic channels and an increase number of complex anastomoses forming between them, during intrauterine life [1,2] . The end product is a continuous dilation of these interconnected thin-walled lymphatic vessels, containing benign endothelial cells [3] .…”

“…Radiological investigations of importance include high resolution computed tomography and more so, magnetic resonance imaging [2] . These are pivotal in determining the extent of disease and hence excluding critical organ involvement.…”