摘要
目的探讨纤维脂肪性血管性病变(FAVA)的诊断、分期与治疗方法。方法回顾性分析西安国际医学中心医院2019年10月至2023年2月收治的FAVA患者的临床资料。术前常规行B超及MRI检查,对双下肢不等长、病变累及关节、关节已发生明显畸形的患者,同时行X线、CT等检查。根据分期制定治疗方案:Ⅰ期(疼痛期),采取开放式或在腔镜辅助下行根治性手术切除病变;Ⅱ期(挛缩期),采取根治性手术切除病变,有时需联合跟腱延长术或肌腱松解,术后2周进行康复训练;Ⅲ期(畸形期),采用以手术切除为基础的综合性治疗措施,即在切除病变肌肉肌腱的基础上,行关节囊松解、跟腱延长或离断、肌腱转移术,并在术前及术后口服西罗莫司,每天2次,每次0.08 mg/m^(2)。对因病变累及多个解剖区域的患者行分期手术,原则上每次手术只针对1个解剖区域。术后对患者疼痛、关节活动及复发情况进行随访。结果共纳入42例FAVA患者,其中男18例,女24例;发病年龄为(7.3±5.0)岁,诊断年龄为(12.5±6.0)岁;上肢病变4例,下肢病变38例;Ⅰ期17例,Ⅱ期17例,Ⅲ期8例。38例有误诊经历,误诊比例达90.5%。患者主要临床表现为持续性疼痛、肌肉挛缩、关节畸形。MRI表现:T1加权像上为不均匀的高低信号影混杂,高信号表现与皮下脂肪组织相同;T2抑脂序列表现出更强的异质性高信号。术后随访时间为(14.6±10.8)个月,术前或术后口服西罗莫司的10例患者疼痛症状均可得到明显缓解;42例患者中31例术后症状完全缓解,得到根治,11例术后仍残留疼痛,或关节活动障碍,甚至畸形;17例Ⅰ期患者中16例治愈,1例术后病变范围进一步扩大,疼痛复发;17例Ⅱ期患者中15例治愈,2例术后有轻度的踝关节活动受限;8例Ⅲ期患者术后均有不同程度的疼痛或关节活动障碍,术后口服西罗莫司可明显缓解症状。Ⅰ、Ⅱ期患者中有10例在腔镜辅助下行手�
ObjectiveTo explore the diagnosis,staging,and treatment of fibro-adipose vascular anomaly(FAVA).MethodsThe data of the patients with FAVA admitted to Xi’an International Medical Center Hospital between October 2019 and February 2023 were retrospectively reviewed.Ultrasound and magnetic resonance imaging(MRI)were routinely performed.X-ray and CT were performed for patients with unequal length of lower limbs,lesions involving joints,and obvious joint deformities.The treatment plan was made according to the stage:stageⅠ(pain stage),open or laparoscopi radical resection of the lesion was performed;stageⅡ(contracture stage),radical surgery was performed to remove the lesion,sometimes combined with Achilles tendon lengthening or tenolysis,and rehabilitation training was performed 2 weeks after surgery;stageⅢ(deformity stage),comprehensive treatment based on surgical resection was adopted,combined with joint capsule release,Achilles tendon lengthening or dissection,tendon transfer and oral sirolimus(each time 0.08 mg/m^(2),twice daily)before and after the operation.For patients with lesions involving multiple anatomical regions,staged surgery was performed,and in principle,only one anatomical region was operated per time.Patients were followed up for pain,joint activity and recurrence.ResultsA total of 42 patients were admitted,including 18 males and 24 females.The onset age was(7.3±5.0)years,but the average age of diagnosis was(12.5±6.0)years.The lower limbs were involved in 38 cases,the upper limbs in 4 cases.There were 17 cases of stageⅠ,17 cases of stageⅡand 8 cases of stageⅢ.Only 4 cases had no misdiagnosis experience,and the misdiagnosis rate was 90.5%(38/42).Persistent pain,muscle contractures and joint deformities were the main clinical symptoms of the disease.MRI showed heterogeneous high and low signal intensity on T1-weighted images,and the high signal intensity was the same as that of subcutaneous adipose tissue.T2 fat-suppressed sequences showed stronger heterogeneous hyperintensity.The foll
作者
谢崇
王怀杰
郭正团
蔺卫龙
周金榜
杨伟家
Xie Chong;Wang Huaijie;Guo Zhengtuan;Lin Weilong;Zhou Jinbang;Yang Weijia(Department of Pediatric Surgery and Vascular Anomalies,Xi’an International Medical Center Hospital,Xi’an 710000,China)
出处
《中华整形外科杂志》
CSCD
2023年第11期1175-1182,共8页
Chinese Journal of Plastic Surgery
基金
西安国际医学中心医院青年课题项目(2021QN016)。
关键词
诊断
治疗
纤维脂肪性血管性病变
磁共振成像
外科手术
Diagnosis
Treatment
Fibro-adipose vascular anomaly
Magnetic resonance imaging
Surgical procedures,operative
