摘要
目的分析巨指并手血管、脑血管畸形的临床特征及鉴别诊断,以提高对该病的认识。方法收集PICU收治的1例左手巨指并手血管、脑血管畸形病例的临床资料并进行文献复习。结果患儿,男,8岁,因生后发现左手示中指巨指畸形,伴活动受限,6岁时行左手中指、示指巨指畸形矫形术,术后外形改善不理想,8岁拟再次手术治疗时发现左手掌掌心血管瘤,并突发脑血管畸形伴脑出血,予脑动静脉畸形栓塞术后好转。结论临床工作中对巨指患者要定期随访,注意随着时间的增长是否出现骨骼、皮肤、脂肪组织为主的非对称性、不规则的过渡生长,以便进一步明确诊断;对巨指合并血管瘤就诊患儿,需要仔细查体及进行影像学检查,以便早期诊断及治疗;巨指合并多发血管畸形,需注意与Proteus综合征、Klippel-Trenaunay综合征和CLOVES综合征等相鉴别。
Objective To summarize the clinical features and differential diagnosis of macrodactyly with vascular malformations of the hand and brain,and to improve the recognition of this disease.Methods Clinical data of a child admitted to PICU for macrodactyly with vascular malformations of the hand and brain was collected,and the literature review was conducted.ResultsThe 8-year-old boy had macrodactyly of the index and middle fingers of the left hand with activity limitation.He had received orthopedic operation when he was 6 years old,but the postoperative appearance was not good.When he was 8 years old and scheduled for a second operation,palmar hemangioma of the left hand and sudden cerebral vascular malformation associated with cerebral hemorrhage were found.The improvement was achieved after embolization of cerebral arteriovenous malformations.Conclusion Regular follow-up is needed in patients with macrodactyly.In order to further confirm the diagnosis,we should pay attention to the presence of asymmetric and irregular transitional growth of bone,skin and adipose tissue in the later stage.Moreover,physical and imaging examinations should be performed for early diagnosis and treatment in children with macrodactyly complicated by hemangioma.In addition,macrodactyly combined with multiple vascular malformations should be distinguished from Proteus syndrome,Klippel-Trenaunay syndrome and CLOVES syndrome.
作者
徐玲玲
邓建荣
唐雯
韩尔乔
XU Ling-ling;DENG Jian-rong;TANG Wen;HAN Er-qiao(Department of Pediatrics,the First Affiliated Hospital,Sun Yat-sen University, Guangzhou 510080,China;Department of Pediatrics,North Guangdong People’s Hospital of Shaoguan,Shaoguan 512025,China)
出处
《实用临床医学（江西）》
CAS
2019年第11期40-42,108,共3页
Practical Clinical Medicine
关键词
巨指
血管畸形
血管瘤
脑血管畸形
脑出血
macrodactyly
vascular malformation
hemangioma
cerebrovascular malformation
cerebral hemorrhage
