儿童血友病性椎管内血肿的临床及影像学特征分析  

Analysis of clinical and imaging features of intraspinal hematoma in children with hemophilia

作　　者：罗晓梅 刘淑仪 尹训涛 徐文婵 张明杰 Luo Xiaomei;Liu Shuyi;Yin Xuntao;Xu Wenchan;Zhang Mingjie(Department of Radiology,Guangzhou Women and Children's Medical Center,Guangzhou Medical University,Guangzhou 510623,China)

机构地区：[1]广州医科大学附属妇女儿童医疗中心放射科,广州510623

出　　处：《中华解剖与临床杂志》2025年第1期23-28,共6页Chinese Journal of Anatomy and Clinics

摘　　要：目的探讨儿童血友病性椎管内血肿的临床特征及其CT和MRI影像学特征。方法横断面研究。回顾性分析广州医科大学附属妇女儿童医疗中心2017年1月—2023年11月经临床及实验室检查确诊的7例血友病性椎管内血肿患儿的临床及影像学资料。7例患儿均为男性, 年龄9个月~7岁, 中位年龄为13个月, 其中血友病A型6例、B型1例。总结该病的临床及CT和MRI影像学特征。结果本组7例患儿中, 6例临床表现为不同程度的身体感觉及运动障碍(颈部疼痛、颈部歪斜、四肢乏力, 以及无法行走、坐立、双手抓握), 1例表现为倦怠、纳差;其中5例伴有发热。患儿CT和MRI检查均检出椎管内背侧硬膜外血肿, 累及9~20个椎体节段, 以颈、胸段较著, 其中1例同时合并脊柱颈段硬膜下血肿、颈髓血肿及胸腰骶部皮下血肿。CT影像上血肿呈稍高及高密度影;MRI上血肿信号多变, 且均见"蜂房状"分隔, MR增强扫描显示4例出现边缘及分隔强化、2例未见明显强化。结论儿童血友病性椎管内血肿好发于颈、胸段椎管内硬膜外腔, 其临床表现不典型, 影像学表现具有一定的特征性;当患儿出现不明原因肢体感觉及运动障碍时, 应尽快完善影像学检查尤其是MRI检查, 以排除该病。Objective:This study aimed to analyze the clinical features,and CT and MRI features of intraspinal hematoma in children with hemophilia.Methods:A cross-sectional design was adopted.The clinical and imaging data of seven children with hemophiliac intraspinal hematoma confirmed by clinical and laboratory examination admitted to Guangzhou Women and Children's Medical Center,Guangzhou Medical University were retrospectively analyzed.All the 7 patients were male aged 9 months to 7 years,with a median age of 13 months.Among them,there were 6 patients of hemophilia A and 1 patient of hemophilia B.The clinical features,and CT and MRI imaging features of the disease were summarized.Results:Six of seven children showed varying degrees of physical sensory and motor disorders(neck pain,neck skew,limb weakness,and inability to walk,sit,and grip),and five patients had fever.The CT and MRI examination findings of all the children showed dorsal spinal epidural hematoma,involving 9-20 vertebral segments;of which,the cervical and thoracic segments were the most common.One of the patients had a cervical subdural hematoma,a cervical intramedullary hematoma,and a thoracolumbosacral subcutaneous hematoma.Based on the CT scan,the hematoma showed a slightly high density;on the MRI scan,the hematoma showed variable signals and beehive-like separation.The enhanced examination showed marginal and septal enhancement in four patients and no significant enhancement in two patients.Conclusion:Intraspinal hematoma in children with hemophilia usually occurs in the epidural space of the cervical and thoracic vertebral canals and has atypical clinical manifestations and certain characteristic imaging findings.For children with unknown limb sensory and motor disorders,imaging examination,especially MRI examination,should be improved as soon as possible to rule out the disease.

关 键 词：血友病 椎管内血肿 临床特征 影像学特征 磁共振成像 体层摄影术 螺旋计算机 儿童 

分 类 号：R726.5[医药卫生—儿科]