儿童四肢肌肉内脉管畸形的临床分析  

Clinical analysis of vascular malformations in extremity muscles

作　　者：岳晓洁[1] 舒艳[2] 赵雄[1] Yue Xiaojie;Shu Yan;Zhao Xiong(Department of Burn and Plastic Surgery,The Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310000,China;Department of Pathology,The Children's Hospital,Zhejiang University School of Medicine,National Clinical Research Center for Child Health,Hangzhou 310000,China)

机构地区：[1]浙江大学医学院附属儿童医院烧伤整形科,国家儿童健康与疾病临床医学研究中心,杭州310000 [2]浙江大学医学院附属儿童医院病理科,国家儿童健康与疾病临床医学研究中心,杭州310000

出　　处：《中华小儿外科杂志》2020年第7期657-661,共5页Chinese Journal of Pediatric Surgery

摘　　要：目的:探讨儿童四肢肌肉内常见脉管畸形的临床特征、影像学表现及病理学特征,以降低误诊率、指导治疗、改善预后。方法:回顾性分析2015年1月至2019年1月收治的32例病理诊断为肢体肌肉内脉管畸形患儿的临床资料。其中,男19例,女13例;年龄为(8.1±3.7)岁,范围为3~16岁;21例因触及局部肿块就诊,11例因患肢疼痛就诊(4例并发跛行及患肢失用性萎缩)。初次就诊时,诊断肌肉内脉管畸形26例,生长痛4例,肌肉拉伤1例,软组织挫伤1例;误诊率18.8%(6/32)。所有患儿术前均接受B型超声检查,14例接受磁共振成像(magnetic resonance imaging,MRI)检查。总结患儿的临床特点,影像学及病理学诊断要点。结果:病灶累及上肢5例,累及下肢27例;术后病理学检查提示静脉畸形19例,纤维脂肪脉管异常7例,软组织血管瘤病6例。术后平均随访时间为32个月,随访时间范围为3个月至4年。1例股四头肌内静脉畸形于术后8个月复发,硬化剂瘤体内注射治疗后好转;其余病例局部病灶及临床症状均未见复发,患肢功能正常,手术切除治愈率96.9%(31/32)。结论:不同类型儿童肌肉内脉管畸形具有不同的临床表现及影像学、病理学特征,对其正确认识有助于指导诊断和治疗,手术切除是一种有效的治疗手段。Objective To explore the clinicopathological features and imaging findings of common vascular abnormalities in extremity muscles so as to lower the rate of misdiagnosis and to improve its prognosis.Methods The clinical data of 32 children hospitalized with a pathological diagnosis of limb intramuscular vascular abnormalities from January 2015 to January 2019 were retrospectively analyzed.Examinations were made by ultrasound(n=32)and magnetic resonance imaging(n=14).Their clinical features,imaging and pathological findings were summarized.Results There were 19 boys and 13 girls with an age range of(8.1±3.7)(3-16)years.The lesions involved 5 upper limbs and 27 lower limbs.The average age of initial consultation was 8.2 years and the misdiagnostic rate was 18.8%.There were venous malformation(n=19),fibroadipose vascular anomaly(n=7)and"angiomatosis"of soft tissue(n=6).During a follow-up period of 6 to 48 months,the functions of affected limbs were normal.One case of venous malformation recurred 8 months postoperatively and the lesion significantly improved after sclerotherapy.The curative rate of surgical resection was up to 96.9%(31/32).Conclusions Different types of pediatric intramuscular vascular abnormalities have different clinical manifestations,imaging and pathological features.Differential diagnosis of subtypes is helpful for diagnosis and treatment.Surgical resection is an effective option.

关 键 词：儿童 肌肉内脉管畸形 静脉畸形 纤维脂肪脉管异常 软组织血管瘤病 

分 类 号：R726.5[医药卫生—儿科]