儿童神经纤维瘤病1型伴血管病变的影像学随访并文献复习  

Neurofibromatosis type 1 associated with vascular abnormalities in children:imaging follow-up and literature review

作　　者：陈静[1] 闫喆[1] 王春祥[1] CHEN Jing;YAN Zhe;WANG Chunxiang(Department of Radiology,Tianjin Children’s Hospital(Children’s Hospital,Tianjin University),Tianjin 300134,China)

机构地区：[1]天津市儿童医院(天津大学儿童医院)影像科,天津300134

出　　处：《国际医学放射学杂志》2023年第6期736-739,共4页International Journal of Medical Radiology

摘　　要：目的探讨儿童神经纤维瘤病1型(NF1)伴血管病变的类型和影像学特点,明确影像学检查用于儿童NF1全面评估和随访的价值。方法回顾性分析1例以血管病变为首发表现的NF1儿童随访5年间的历次影像资料,并复习相关文献。结果病人5月龄时主因“血压升高9天”入院,首次腹部CT血管成像(CTA)发现右肾动脉瘤、右髂内动脉瘤,此后5年间进行3次CTA检查,显示右肾动脉及右髂内动脉瘤逐渐血栓化、机化、体积缩小。期间病人3次头颅MRI平扫示脑实质多发病灶且随年龄增加而增多、增大,头颅MR血管成像(MRA)示Willis环右侧部分血管管腔变细,右侧大脑中动脉分支明显减少。结论儿童NF1以血管病变为首发表现者少见,血管病变是导致NF1儿童高血压的最常见原因,影像学检查需重视多部位受累血管的全面评估及长程随访。Objective To analyze the types and imaging features of neurofibromatosis type 1(NF1)associated with vascular abnormalities in children,in order to identify the value of imaging examination in the evaluation and follow-up.Methods All previous imaging examinations during a 5-year follow-up period of NF1 in a child with vascular abnormalities as the initial manifestation were analyzed retrospectively,and relevant literature was reviewed.Result The patient was admitted to the hospital at the age of 5 months with the main complaint of“increased blood pressure for 9 days”.The initial abdominal CTA indicated aneurysms of the right renal artery and internal iliac artery.Subsequently,the child underwent three additional CTA,during which the aneurysms of the right renal artery and the right internal iliac artery were gradually thrombosed and institutionalized with the volume gradually reduced.Three head routine MRI scans revealed multiple abnormal signal intensity lesions of the brain,which increased in number and volume with the increase in age.Head MRA showed right side partial stenosis of the Willis,with significant reduction in the branches of the right middle cerebral artery.Conclusion Vascular abnormalities are rare initial manifestation of NF1 in children.Vascular lesions are the most common cause of hypertension in NF1 children,imaging examination should pay attention to comprehensive evaluation and long-term follow-up of multiple affected blood vessels.

关 键 词：儿童 神经纤维瘤病1型 动脉瘤 脑 CT血管成像 磁共振成像 

分 类 号：R814[医药卫生—影像医学与核医学] R445[医药卫生—放射医学]