Background: Generalized lymphatic anomaly (GLA) is a rare condition, mainly involving bones, soft tissue, and internal organs. The diagnosis of GLA is often difficult.
Case Presentation: We report a case of GLA in a boy who was initially treated for suspected Langerhans cell histiocytosis and discuss the potential imaging features of GLA. The clinical and imaging data of a case of GLA in an 8-year-old boy were analyzed retrospectively, and the literature was reviewed.
Results: The case shows the imaging features of GLA with multiskeletal diffuse expansile cystic osteolytic lesions penetrating the cortical surface and extending within the cortex and a pumice-like or rotten wood-like rough appearance on volume-rendered 3D CT images. Soft tissue multi-cystic masses increase diagnostic confidence. Fatty infiltration appears in multiple vertebral bodies and sternum, namely, abnormal T1 and T2 hyperintense and fat-suppressed T2 hypointense on MRI and the corresponding low density similar to that of fat on CT, suggesting that GLA involves the vertebrae and sternum, which may be accompanied by chylothorax.
Conclusion: GLA in bone has typical features on CT. MRI reveals its cystic nature, and typical soft tissue lesions and chylothorax increase confidence in the diagnosis.
Keywords: generalized lymphatic anomaly, lymphangiomatosis, bone, CT, MRI