Klippel-Trenaunay综合征合并下肢淋巴水肿MR影像表现

MR Features of Klippel-Trenaunay Syndrome with Lymphedema in Lower Limbs

目的探讨Klippel-Trenaunay综合征(KTS)合并下肢淋巴水肿MR影像表现。方法回顾性搜集34例确诊为KTS合并下肢淋巴水肿患者的临床和MR影像资料,扫描序列为短时间反转恢复(STIR)序列,观察患肢MR影像学表现,记录各征象出现频次,并采用分类变量资料中的构成比进行统计描述。结果皮下软组织水肿征象:“平行线征”11例(32.35%)、“网格征”11例(32.35%)、“蜂窝征”12例(35.29%)、“条带征”12例(35.29%)、“淋巴湖征”9例(26.47%)、“新月征”10例(29.41%)、“星云征”10例(29.41%)、皮下脂肪扩张管征30例(88.24%)、肌肉扩张管征24例(70.59%)。由皮下软组织水肿征象划分的影像分型中:肿胀型3例(8.82%),扩张型10例(29.41%),混合型21例(61.77%)。结论MR检查能够显示KTS合并下肢淋巴水肿的异常表现,为该病的影像诊断、影像分型提供重要依据。

Objective To investigate the MR features of Klippel-Trenaunay syndrome complicated with lower extremity lymphedema.Methods The clinical and MR imaging data of 34 patients with Klippel-Trenaunay syndrome complicated with lymphedema were collected retrospectively.MRI was performed with STIR sequence.The MR features of the affected limbs were observed,and the frequency of each sign was recorded.The constituent ratio in the categorical variable data was used for statistical description.Results The signs of subcutaneous soft tissue edema:parallel line sign in 11 cases(32.35%),grid sign 11 cases(32.35%),honeycomb sign 12 cases(35.29%),strip sign 12 cases(35.29%),lymph lake sign 9 cases(26.47%),crescent sign in 10 cases(29.41%),star cloud sign 10 cases(29.41%),subcutaneous fat dilation tube sign 30 cases(88.24%)and muscle dilation tube sign 24 cases(70.59%).Three types were divided according to the signs of subcutaneous soft tissue edema:3 cases of swelling type(8.82%),10 cases of dilation type(29.41%),and 21 cases of mixed type(61.77%).Conclusion MR examination can show the abnormal manifestations of Klippel-Trenaunay syndrome with lymphedema,which provides an important basis for the imaging classification and imaging diagnosis of the disease.