Table 1 Thoracic lymphatic malformations
From: Lymphatic malformations involving the thorax in children: a retrospective cohort study
Classification | Number | Clinical features | Treatment | Outcomes |
|---|---|---|---|---|
Cystic lymphatic malformations | 1 | Isolated lesions | Sclerotherapy | Improvement |
Complex lymphatic anomalies | 34 | Symptoms overlap; chylothorax, plastic bronchitis; diffuse mediastinal lesions; interlobular septal thickening | Comprehensive treatment | Overall improvement (80% response rate) |
Generalized lymphatic anomaly (GLA) | 18 | Involvement of thorax, bone, spleen, and soft tissue; combined with CCLA | Sirolimus, bevacizumab, surgery | Improvement/Stabilization/Death |
Kaposiform lymphangiomatosis | 8 | Like GLA; hemorrhagic effusions; consumptive coagulopathy (thrombocytopeni, hypofibrinogenemia, elevated D-dimer); spindled endothelial cells | Sirolimus, trametinib, vincristine, surgery | Improvement/Death |
Gorham–Stout disease | 2 | Like GLA; osteolysis, cortical destruction; pathological fracture | Sirolimus, symptomatic treatment | Improvement in chest lesions, progression in bone lesions |
Central conducting lymphatic anomaly (CCLA) | 6 | Pleural/pericardial/peritoneal effusions; persistent ground-glass opacity on CT, lymphangiography | Mainly depended on surgery, response to sirolimus, diet intervention | Improvement/Stabilization |
Primary lymphoedema | 4 | Chest lesions appeared several years following limb edema; combined with CCLA | Thoracic duct surgery, limb compression decongestive management | Stabilization |
Infancy chylothorax | 3 | Infancy, right chylothorax | Diet intervention | Improvement |