儿童朗格汉斯细胞组织细胞增生症累及中枢神经系统及颅面骨的影像学特征分析  

Imaging features of Langerhans cell histiocytosis involving central nervous system and craniofacial bone in children

作　　者：李春旺[1] 尹一伟 蔡齐芳 汤静[1] Li Chunwang;Yin Yiwei;Cai Qifang;Tang Jing(Department of Radiology,Hunan Children’s Hospital,Changsha 410007,China)

机构地区：[1]湖南省儿童医院放射科,长沙410007

出　　处：《临床小儿外科杂志》2023年第8期768-772,共5页Journal of Clinical Pediatric Surgery

基　　金：湖南省卫生健康委员会一般指导课题(202209012654)

摘　　要：目的探讨朗格汉斯细胞组织细胞增生症累及儿童中枢神经系统及颅面骨的特征性影像学表现。方法回顾性分析2017年1月至2022年12月湖南省儿童医院收治的44例经病理检查证实的朗格汉斯细胞组织细胞增生症(langerhans cell histiocytosis,LCH)累及中枢神经系统及颅面骨患儿的影像学表现。结果44例累及中枢神经系统及颅面骨的朗格汉斯细胞组织细胞增生症(central nervous system-langerhans cell histiocytosis,CNS-LCH)患儿中,25例颅面骨骨质破坏,典型征象为"双边征"、"纽扣样死骨";16例下丘脑-垂体病变,主要表现为神经垂体T1WI高信号消失;硬脑膜及松果体病变各2例,表现为局部肿块及松果体囊性病变;29例累及小脑,病灶表现为稍长T1稍长T2信号;2例脑白质病变,表现为对称性稍长T1稍长T2信号;2例血管周围腔隙扩张,表现类圆形长T1长T2信号。结论LCH累及颅面骨的主要影像学特征为无硬化环溶骨性骨质破坏,累及中枢神经系统以灰质核团多见,为对称性病变,最终诊断依靠病理学检查。Objective To explore the characteristic imaging manifestations of Langerhans cell histiocytosis(LCH)involving central nervous system(CNS)and craniofacial bone in children to boost its clinical diagnosis.Methods The imaging findings of 44 patients with LCH involving CNS and craniofacial bones confirmed by pathologic examination were reviewed retrospectively from January 2017 to December 2022.Results Among 44 children with central nervous system-Langerhans cell histiplasia(CNS-LCH),25 cases had craniofacial osteodegeneration characterized by"bilateral sign"and"button dead bone".Hypothalamic-pituitary lesions were detected with neuropituitary T1WI hypersignal loss(n=16).Dural and pineal lesions were cystic(n=2).Cerebellar lesions displayed slightly longer T1 and slightly longer T2 signals(n=29);Leukoencephalopathy showed slightly longer symmetrical T1 and slightly longer T2 signals(n=2);Perivascular lacunae became dilated with similar circular long T1 and long T2 signals(n=2).Conclusion The major imaging features of LCH involving craniofacial bone are non-sclerotic cystolytic bone destruction and CNS involves gray matter nucleus mass with frequent symmetrical lesions.A definite diagnosis depends upon pathology.

关 键 词：组织细胞增多症 郎格尔汉斯细胞 中枢神经系统 颅骨 磁共振成像 计算机断层扫描术 儿童 

分 类 号：R725.5[医药卫生—儿科]