RESUMO
Objective To study the clinical characteristics of lymphatic malformation(LM).Methods Clinical data of 14 cases with LM were analysed and literatures were reviewed.Results ①Among the 14 cases,6 were diagnosed as primary intestinal lymphangiectasia(PIL)and 8 as primary lymphedema(PL);②The major manifestations of PIL are symmetry edema,diarrhea,lymphocytopenia,hypoproteinemia and chylopericardium.The manifestations of PL included asymmetry edema of lower limbs and respiratory symptoms;③Directed oil-contrast lymphography and isotope techniques demonstrated absence of contrast in some lymphatic segments and blockage of chylous reflux;④White opaque spots were found on the duodenal and intestinal mucosa in the endoscopy of 6 cases of PIL indicating lacteal vessels dilation.Pathological examinations revealed intestinal lymphangiectasia;⑤Six cases of 14 patients were treated with surgical therapy and 8 cases were treated with conservative therapy including medium chain triglyceride(MCT).Conclusion LM should be diagnosed on the bases of typical clinical manifestations specific laboratory findings and by lymphography and pathology.Combination of surgical therapy and conservative therapy is recommended to treat the LM patients.