Generalized cystic lymphangiomatosis

Abstract

A 2-year-old girl presented with acute shortness of breath and multiple subcutaneous nodules. Chest radiograph showed widening of the mediastinum. Contrast-enhanced CT of the thorax revealed interstitial thickening, lung nodules, pleural effusions (Fig. 1a, black arrow) and cystic mediastinal collections (Fig. 1a, white arrow). Sections of the abdomen showed ascites, multiple lowdensity lesions in the spleen (Fig. 1b, black arrow) and retroperitoneum (Fig. 1b, white arrow). No appreciable enhancement was noted following administration of contrast medium. Multiple osteolytic lesions with sclerotic margins were also detected in the vertebral bodies (Fig. 1b, black arrowhead). Drainage of the pleural effusions revealed chylothorax. Generalized lymphangiomatosis is a rare disease, characterized by a diffuse proliferation of lymphatic vessels that can involve any organ except the central nervous system [1]. Prognosis depends on the site and extent of the lymphangioma [1]. Imaging features are characteristic and crucial in the evaluation of the complete extent of disease and, thus, may contribute to clinical management of the disease by preventing initial misdiagnosis [2].