原发性淋巴管发育异常合并Gorham-Stout综合征的影像学表现

目的探讨原发性淋巴管发育异常合并Gorham-Stout综合征(GSS)的影像学特点。方法回顾性分析15例原发性淋巴管发育异常合并GSS患者的临床及影像学资料。15例患者均接受直接淋巴管造影,造影后CT、核医学淋巴显像检查;其中5例接受MR检查。结果直接淋巴管造影发现胸导管出口梗阻8例,下肢、下肢及阴囊、会阴、腹壁乳糜反流7例,锁骨下干、纵隔支气管干反流4例,腹膜后、髂窝淋巴管结构紊乱、纡曲、扩张7例。15例CT均发现多发溶骨性骨质破坏,累及椎体及椎体附件9例,髂骨5例,肋骨6例,肩胛骨1例,锁骨1例,肱骨1例,双侧股骨4例,跟骨、距骨1例;7例脾脏增大,可见多发类圆形低密度影,9例胸腔积液,6例腹腔积液,1例心包积液。核医学淋巴显像发现胸导管出口梗阻8例,下肢、下肢及阴囊、会阴、腹壁乳糜反流6例,锁骨下干、纵隔支气管干反流4例,腹膜后、髂窝淋巴管扩张6例,脾脏多发放射缺损2例。5例MR均发现骨质内多发长T1长T2异常信号,双髂骨3例,腰骶尾椎及椎体附件4例,双股骨2例,跟、距骨1例。结论原发性淋巴管发育异常合并GSS是淋巴系统发育异常的复杂综合征,其影像学表现、尤其是直接淋巴管造影术后CT表现具有特异性,对早期诊断本病非常重要。

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.