Differential Diagnosis Of FAVA And Intramuscular Venous Malformation Of Extremities

Background:Fibroadipose Vascular Anomaly(FAVA)is a vascular malformation disease first proposed by Alomari et al.,Boston Children’s Hospital in 2014.The histological manifestation is excessive proliferation of Fibroadipose tissue.At the same time,Venous malformation(VMs)is characteristic.Clinically,pain,mass and functional limitation are the main manifestations,which are relatively rare.Until February 2022,only two literatures were retrieved and transliterated as "Fava disease" in China.Intramuscular venous malformation(IMVM)is a kind of common venous malformation,which locates in muscle and has certain similarities with FAVA in clinical symptoms such as pain and mass.As FAVA is a newly proposed disease,it is not thoroughly studied at present and is easily diagnosed as a common intramuscular venous malformation.The key points of differential diagnosis between FAVA and IMVM need to be studied by incorporating more case data.Objective:Summarize the clinical,imaging,and pathological characteristics of FAVA(fibro-Adipose Vascular Anomaly)and IMVM of extremities,compare the intraoperative characteristics of FAVA and IMVM of extremities,and observe and analyze the effect of surgical resection.Raise awareness of FAVA(fibroAdipose Vascular Anomaly)and intramuscular venous malformations of the extremitiesMethods:Collect and arrange from June 2019 to December 2021 in henan province hemangioma and vascular malformation of diagnosis and treatment center for surgery and the postoperative pathological diagnosis of23 patients with FAVA and the clinical data of 46 patients with intramuscular venous malformations,including parts,gender,age,imaging examination,treatment of characteristics,pathology and clinical characteristics of data,The characteristics of the two diseases were summarized,and the imaging,pathological and clinical characteristics as well as the number of muscle involvement,intraoperative blood loss and operation time of the two diseases were compared and analyzed.Meanwhile,the effect of surgical treatment was observed and analyzed in combination with the pain score.Result:1.The mean age of FAVA patients was 13.09±5.55 years old,and the mean age of intramuscular venous malformation was 16.80±5.80 years old,showing statistical difference(P < 0.05).The peak age distribution of FAVA patients was concentrated in 9～12 years old.The peak age of intramuscular venous malformation was 17～20 years old.There was no statistical difference in gender between FAVA and iv M patients of extremities(P=0.075).In terms of the location of the disease,both of them were common in lower limbs,and there was no statistical difference(P=0.052).There were statistically significant differences between the two groups in terms of pain and dysfunction(P < 0.05).The FAVA group had more severe pain and a higher rate of dysfunction(95.7%,22/23),while intraminovenous malformations were more likely to reach masses(76.1%,35/46).2.On ultrasonography,FAVA showed clear boundary hyperechoic mass,while IMVM showed unclear boundary hypoechoic mass.The blood flow signal of FAVA patients under color Doppler ultrasonography was weaker than IMVM.Mri findings: In FAVA group,T1 showed high signal and T2 high signal,T1 low signal or equal signal of IMVM,T2 showed high signal,and T2 signal intensity of IMVM was higher than FAVA.Microscopic observation of HE staining showed some differences between the two diseases.3.There were statistical differences in the number of muscle involvement,intraoperative blood loss and operation time between the two groups(P < 0.05).The ratio of intramuscular venous malformation involving≥2 muscles was 52.2%(24/46).The mean intraoperative blood loss in the FAVA group was 54.78±26.61 ml and the mean intraoperative time was 60.04±18.02 min,while the mean intraoperative blood loss in the FAVA group was 126.74±79.57 m L and the longer operative time was 104.46±24.20 min.4.All the 23 FAVA patients received surgical treatment without short-term or long-term complications,and the surgical results were clear.All 46 patients with intramuscular venous malformation received surgical treatment,2 patients developed short-term complications of subcutaneous fat liquefaction after surgery,and1 patient developed scar contracture but had no effect on limb activities,showing clear surgical results.Conclusion:1.FAVA patients are younger than those with IMVM,the pain is more severe than IMVM,and they are more likely to show dysfunction,while IMVM is more likely to touch masses;2.All patients underwent surgical treatment.During surgery,FAVA was found to be significantly less than intramuscular venous malformation in terms of the number of muscle involvement,operation time and intraoperative blood loss.3.Postoperative follow-up,to achieve the curative effect,under the current treatment methods,surgery can be an effective treatment for FAVA and intramuscular venous malformation;4.Comparison of clinical,radiological and pathological differences is helpful for differential diagnosis of the two diseases,but more case data still need to be included in the study.