Imaging characteristics of primary lymphatic dysplasia combined with Gorham-Stout syndrome

Objective To explore the imaging characteristics of primary lymphatic dysplasia combined with Gorham-Stout syndrome(GSS).Methods A retrospective analysis of the clinic and radiological data of 15 patients with primary lymphatic dysplasia and GSS was performed.All of patients underwent direct lymphangiography,CT and lymphoscintigraphy,5 underwent MR examination.Results Direct lymphangiography showed thoracic duct outlet obstruction(n=8),lower extremities and/or scrotum,perineum,abdominal refluxing(n=7),subclavian dry,mediastinal bronchial dry refluxing(n=4),disorder,tortuous,expansion of the retroperitoneal and iliac lymphatic duct(n=7).CT showed multiple destruction of the bone,9 cases in vertebral and vertebral attachments,5 cases in iliac,6 cases in ribs,1 case in scapula,1 case in clavicle,1 case in humerus,4 cases in dual femur,1 case in calcaneus and talus.CT also showed 7 cases of splenomegaly and multiple cysts the spleen,9 cases of pleural effusion,6 cases of ascites,1 case of pericardial effusion.Lymphoscintigraphy showed that there were 8 cases of thoracic duct outlet obstruction,6 cases of lower extremities and/or scrotum,perineum,abdominal refluxing,4 cases of subclavian dry,mediastinal bronchial dry refluxing,6 cases of expansion of the retroperitoneal and iliac lymphatic duct,2 cases of multiple radiation defect of the spleen.MRI showed multiple long T1 and T2 signals of the bone in all 5 cases,and there were 3 cases in iliac,4 cases in lumbar,sacral,caudal and vertebral attachments,2 cases in dual femur,1 case in calcaneus and talus.Conclusion Primary lymphatic dysplasia combined with GSS is a complex syndrome of lymphatic dysplasia.Imaging findings,especially CT after direct lymphangiography are very important and valuable for the diagnosis.