Ⅰ型Madelung综合征的临床特征及CT、MRI影像特点

Clinical characteristics and CT,MRI imaging features of TypeⅠ Madelung syndrome

目的探讨Ⅰ型Madelung综合征的临床特征及CT、MRI影像特点,提高对本病的认识。方法收集经临床证实的Ⅰ型Madelung综合征15例,回顾性分析其临床资料及CT、MRI影像表现,CT平扫5例,MRI平扫10例。结果15例中女性6例,男性9例。男性均有酗酒史,伴酒精性肝硬化,女性有代谢障碍性疾病,其中糖尿病2例,高血压2例,甲状腺功能减退症1例,高尿酸血症1例。临床上均表现为颈部对称性增粗,女性主要位于双侧后项部,男性主要位于双侧颈部、项部、肩背部及上胸部。CT均显示皮下及肌间隙内均一脂肪密度,呈弥漫性、对称性分布,边界不清,无包膜,无肿块,无钙化,内可见索条状纤维分隔,邻近肌肉受压、移位、萎缩、变细。MRI显示病变区均表现为短T1长T2信号,压脂序列均呈低信号。结论Ⅰ型Madelung综合征为男性发病,女性发病罕见,脂肪组织的异常沉积存在性别差异,女性患者病变范围比较局限,主要累及后颈部,而男性患者范围广泛,主要累及颈部、项部、肩背部及上胸部。

Objective To investigate the clinical characteristics and CT, MRI features of Type I Madelung syndrome, and to increase awareness of the disease. Methods Fifteen cases of Type I Madelung syndrome proved by surgery and pa- thology were collected. The CT and MRI features of Type I Madelung syndrome in 15 patients were reviewed retrospec- tively, including 5 cases scaned by unenhanced CT and 10 cases scaned by unenhanced MRI. Results There existed 6 fe- male cases and 9 male cases among these 15 patients. All male cases had alcoholism history, along with alcoholic cirrhosis. Female cases had metabolic disorders, including 2 cases of diabetes and hypertension respectively, one case of hypothyroid- ism and hyperuricemia respectively. 15 patients of type I Madelung syndrome all manifested thickened neck symmetrically. The lesions of females were mainly located in the bilateral neck, while lesions of males mainly located in bilateral neck, napex, shoulder and back, and upper chest. CT scans all showed uniform fat density in subcutis and muscle compartment, located pervasively and symmetrically, without envelope, lumps or calcification. Strip cable fiber separations can be seen in the involvement. What＇s more, the muscles around were compressed, translocated, atrophic and thin. All lesions were displayed hypointensity on T1WI and hyperintensity on T2WI, hypointensity on fat-suppression sequence. Conclusion Type I Madelung syndrome is frequently seen in males, rarely seen in women. Abnormal accumulation of fat tissue dif- fers from gender. The lesion of female patients are limited, mainly involving the neck. The lesion of male patients are wide, mainly involving the neck, napex, shoulder and back, and upper chest.