CT Lymphangiography (CTL) in Different Type of Primary Intestinal Lymphangiectasia (PIL): A Comparative Study
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摘要: 目的：探讨CT淋巴管成像（CTL）在不同分型的原发性小肠淋巴管扩张症（PIL）患者的临床与影像特征比较和应用价值。方法：收集2016年1月至2019年12月确诊为PIL的所有患者，回顾分析其CTL图像，由两名放射科医师盲法评价，评价指标包括：性别、首发年龄、临床症状和体征、血清白蛋白、肠壁是否增厚、肠系膜密度增高、浆膜腔是否积液、异常淋巴管分布的部位及范围、淋巴液是否返流、淋巴瘘的有无，腹腔内是否有淋巴结和颈部是否有异常扩张的淋巴管。基于对比剂是否分布于小肠壁和肠系膜这一特异影像学征象，首次提出将PIL分型为Ⅰ型（阳性组）与Ⅱ型（阴性组），比较两组患者的临床和影像学特征。结果：34例PIL患者中，Ⅰ型15例，Ⅱ型19例。Ⅰ型首次发病年龄较大（15.3±9.4 vs.8.3±3.8），腹泻发生率较高，而肢体和/或颜面部肿胀的比例低于Ⅱ型，两组性别比和白蛋白水平差异无统计学意义；影像征象方面，Ⅰ型腹膜后异常扩张淋巴管和淋巴瘘的比例高于Ⅱ型，肠系膜密度增高和腹腔淋巴结出现的比例低于Ⅱ型。两组间肠壁增厚、浆膜腔积液、返流样表现和颈部异常扩张淋巴管的差异无统计学意义。结论：CTL可以评价PIL患者异常淋巴管分布的部位、范围和程度，基于CTL提出了PIL的影像分型，不同类型的PIL具有不同的临床和影像特征。
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关键词:
- CT /
- 淋巴管成像 /
- 原发性小肠淋巴管扩张症 /
- 分型
Abstract: Objective: To explore the clinical and imaging characteristics and application value CT lymphangiography (CTL) in patients with different types of primary intestinal lymphangiectasia (PIL). Methods: Patients diagnosed as PIL in our center were recruited in this retrospective study from January 2016 to December 2019, All CTL data were blindly reviewed by two radiologists separately, and the evaluation indicators included: sex, onset age, symptoms and signs, serum albumin, wall thickening, serous cavity effusion, abnormal distribution of lymphatics, lymph reflux, lymph nodes, fistula and abnormal lymphatics around neck area. Based on the abnormal lymphatics in intestinal wall and/or mesentery, PIL was classified into type Ⅰ (positive type) and type Ⅱ (negative type). The clinical and imaging features were compared between the two groups. Results: 34 PIL patients were recruited in this study, including 15 cases of Ⅰ and 19 cases of Ⅱ. Type I showed older age of first onset (15.3±9.4 vs. 8.3±3.8), higher rate of diarrhea, and lower rate of limb and/or facial edema than type Ⅱ, with no statistical difference in sex and serum albumin. For imaging features comparisons, type Ⅰ demonstrated higher rate of abnormal dilated lymphatics and fistula, lower rate of increased attenuation of mesentery and lymph nodes, while no statistical difference was found between wall thickening, serous cavity effusion, lymph reflux and abnormal dilated lymphatics around neck area. Conclusion: CTL demonstrated capability of evaluation in detection of location, distribution and range of abnormal lymphatics in PIL. Based on CTL, the imaging classification of PIL was proposed. Different types of PIL showed different clinical and imaging features, which was useful for therapeutic adoptions.-
Keywords:
- CT /
- lymphangiography /
- primary intestinal lymphangiectasia /
- type
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图 1 Ⅰ型PIL，多发对比剂异常分布
Figure 1. Type Ⅰ PIL with diffusive abnormal contrast media in abdomen
表 1 不同类型PIL的临床特征比较分析
Table 1 Comparison of clinical characteristics in different types of PIL
临床特征 类型 统计检验 Ⅰ型（n=15） Ⅱ型（n=19） t/χ2 P 首次发病年龄 15.3±9.4 8.3±3.8 2.995 ＜0.010 性别比（女/男） 7/8 10/9 0.119 0.730 腹泻 11（73.3％） 6（31.6％） 5.846 0.016 颜面和/或肢体肿胀 8（53.3％） 19（100％） 11.165 ＜0.010 血清白蛋白/（g/L） 17.1±4.4 18.6±6.3 -0.935 0.357 
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表 2 不同类型PIL的影像特征比较分析
Table 2 Comparison of CTL imaging features in different types of PIL
影像特征 类型 统计检验 Ⅰ型（n=15） Ⅱ型（n=19） χ2 P 小肠改变 肠壁环形增厚 2（13.3％） 1（5.3％） 0.679 0.410 肠系膜密度增高 7（46.7％） 18（94.7％） 9.952 0.002 浆膜腔积液 仅腹腔积液 1（6.7％） 3（15.8％） 0.672 0.412 腹腔+胸腔积液 1（6.7％） 4（21.1％） 1.383 0.240 返流样表现 8（53.3％） 7（36.8％） 0.925 0.336 淋巴瘘 5（33.3％） 0（0.0％） 7.425 0.006 腹腔内淋巴结 2（13.3％） 12（63.2％） 8.591 0.003 腹膜后淋巴管扩张 13（86.7％） 0 26.660 0.000 颈部扩张淋巴管 5（33.3％） 12（63.2％） 2.982 0.084
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