婴幼儿朗格汉斯细胞组织细胞增生症侵犯肝脏的影像表现

Imaging Findings of Liver Infiltration in Infants with Langerhans Cell Histiocytosis

目的:分析婴幼儿朗格汉斯细胞组织细胞增生症(LCH)侵犯肝脏的影像学表现特点,提高诊断能力。方法:11例经临床、病理证实的LCH患者纳入回顾性分析,男9例,女2例,平均年龄17个月,全部完成腹部CT增强检查,3例完成MRI检查。结果:肝脏弥漫增大,CT上肝内病灶呈低密度,无明显强化,MRI上呈长T1长T2信号;病灶形态和分布可分为三种类型:①中央型,沿中央门脉两侧分布的双线条状病变,7例;②周围型,肝实质内多发结节,2例;③弥漫型,同时有中央和周围分布的病灶,2例。伴有胆管扩张3例、肠壁增厚5例、腹腔积液1例、脾肿大9例、淋巴结肿大3例。结论:婴幼儿LCH肝脏侵犯影像表现有特点,有助于诊断和临床处理。

Purpose： To evaluate the imaging findings of liver infiltration in infants with Langerhans cell histiocytosis （LCH）, so as to improve the ability of diagnosis. Methods： Eleven patients with LCH confirmed by the clinical presentations and pathologic results were enrolled in our study, including 9 males and 2 females whose mean age was 17 months. All patients were undergone plain and post-contrasted MSCT scans, and three patients were undergone MRI examinations. Results： All the patients were with hepatomegaly. The lesions were shown as multiple low-density unenhanced lesions in CT scans and long TI and long T2 signal intensity lesions in MRI. The lesion distribution could be classified into three types： seven patients were with central type, whose lesions were linear and distributed alongside the central portal veins; two patients were with peripheral type, whose lesions were multi-nodular and distributed randomly in liver parenchyma; and the other two patients were with diffuse type, whose lesions were meanwhile located in parenchyma and alongside the central portal veins. The accompanying findings included bile duct dilatation （3/11, 27.3%）, bowel wall thickening （5/11, 45.5%）, ascites （1 / 11, 9.1%）, splenomegaly（9/11, 81.8%） and lymph node enlargement （3/11, 27.3%）. Conclusion： The imaging findings of LCH liver infiltration in infants are with characteristic and are helpful for the diagnosis and treatment.