Fibroadipose Vascular Anomaly: Diagnosis and Treatment

IF 1.4 Q3 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING Techniques in Vascular and Interventional Radiology Pub Date : 2024-06-01 DOI:10.1016/j.tvir.2024.100961
Raja Shaikh MD
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Abstract

Fibro-Adipose Vascular Anomaly (FAVA) is a recently identified type of vascular malformation predominantly affecting adolescent females. Comprising abnormal adipose and vascular components, FAVA is frequently misdiagnosed as other vascular anomalies. It primarily manifests with pain, functional impairment, and musculoskeletal symptoms, particularly in the lower extremities. Accurate diagnosis requires a combination of clinical, radiologic, and histopathologic evaluation, with MRI and ultrasound being the primary imaging tools. Management of FAVA is multidisciplinary and tailored to individual patients. Interventional radiology procedures, such as percutaneous cryoablation, sclerotherapy, and embolization, are effective in long term control of symptoms. Cryoablation is particularly successful in alleviating pain and improving function. Surgical resection is reserved for specific cases with extensive lesions involving joints or when there is severe muscle or joint dysfunction. Additionally, sirolimus, an mTOR inhibitor, has shown promise in symptom relief, although further research is needed to confirm its long-term efficacy. Early diagnosis and treatment are essential for improving the quality of life in FAVA patients. Advances in imaging and treatment strategies have enhanced the ability to manage this complex and rare condition effectively.

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纤维脂肪血管异常:诊断与治疗
纤维脂肪血管畸形(Fibro-Adipose Vascular Anomaly,FVA)是最近发现的一种血管畸形,主要影响青少年女性。纤维脂肪血管畸形由异常的脂肪和血管组成,经常被误诊为其他血管畸形。它主要表现为疼痛、功能障碍和肌肉骨骼症状,尤其是下肢。准确诊断需要结合临床、放射学和组织病理学评估,其中核磁共振成像和超声波是主要的成像工具。FAVA 的治疗是多学科的,并根据患者的具体情况量身定制。经皮冷冻消融术、硬化剂注射和栓塞术等介入放射学手术对长期控制症状非常有效。冷冻消融术在减轻疼痛和改善功能方面尤为成功。手术切除只适用于病变广泛累及关节或出现严重肌肉或关节功能障碍的特殊病例。此外,mTOR 抑制剂西罗莫司(sirolimus)有望缓解症状,但其长期疗效尚需进一步研究证实。早期诊断和治疗对于提高 FAVA 患者的生活质量至关重要。成像和治疗策略的进步提高了有效控制这种复杂而罕见疾病的能力。
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来源期刊
Techniques in Vascular and Interventional Radiology
Techniques in Vascular and Interventional Radiology RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING-
CiteScore
2.70
自引率
0.00%
发文量
47
期刊介绍: Interventional radiology is an area of clinical diagnosis and management that is highly technique-oriented. Therefore, the format of this quarterly journal, which combines the visual impact of an atlas with the currency of a journal, lends itself perfectly to presenting the topics. Each issue is guest edited by a leader in the field and is focused on a single clinical technique or problem. The presentation is enhanced by superb illustrations and descriptive narrative outlining the steps of a particular procedure. Interventional radiologists, neuroradiologists, vascular surgeons and neurosurgeons will find this a useful addition to the clinical literature.
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