纤维脂肪血管异常的综合治疗：一例报告。
Hybrid treatment of fibroadipose vascular anomaly: A case report.
作者信息
Stillo Francesco, Ruggiero Federica, De Fiores Antonio, Compagna Rita, Amato Bruno
机构信息
Vascular Malformation Unit, Department of Surgery, Istituto, Clinica Guarnieri, Via Tor de' Schiavi, 139, 00172, Rome (RM), Italy.
Department of Clinical and Molecular Medicine, Faculty of Medicine and Psychology, Vascular Surgery Unit, University of Rome La Sapienza, Via di Grottarossa 1035/1039, 00189 Rome, Italy.
出版信息
Open Med (Wars). 2020 Sep 11;15(1):890-897. doi: 10.1515/med-2020-0228. eCollection 2020.
BACKGROUND
First identified in 2014, fibroadipose vascular anomaly (FAVA) is a very rare type of venous and lymphatic malformation. Marked by tough fibrofatty tissue in the extremities overtaking portions of the muscles, it is associated with constant pain and contracture of the affected extremity. There is a paucity of literature, and no guidelines on treatment procedure are available. This case highlights the role of hybrid treatment with primary ethanol percutaneous ethanol embolization and additional surgery for radicality in excision of FAVA lesions.
CASE SUMMARY
A 9-year-old girl with FAVA underwent the hybrid treatment. The achievements of complete excision, clinical response, and patient satisfaction in long-term follow-up were assessed. Following the hybrid treatment, the patient experienced significant improvement in pain. Concurrent symptoms of physical limitation, leg swelling, and skin hyperesthesia also improved. The clinical benefit, supported by postoperative physiotherapy, was well stabilized at 6-month follow-up, resulting in complete patient satisfaction at 12- and 36-month follow-ups. No major complications were encountered.
CONCLUSION
Ethanol embolization plus surgery is a safe, effective, and long-term hybrid treatment of symptomatic FAVA lesions.
背景
纤维脂肪性血管异常（FAVA）于2014年首次被发现，是一种非常罕见的静脉和淋巴管畸形。其特征为四肢坚韧的纤维脂肪组织取代部分肌肉，与患肢持续疼痛和挛缩有关。相关文献较少，且尚无治疗程序指南。本病例突出了原发性乙醇经皮乙醇栓塞联合额外手术的混合治疗在FAVA病变切除根治性方面的作用。
病例摘要
一名患有FAVA的9岁女孩接受了混合治疗。评估了完全切除的效果、临床反应以及长期随访中的患者满意度。混合治疗后，患者疼痛明显改善。身体活动受限、腿部肿胀和皮肤感觉过敏等并发症状也有所改善。术后物理治疗支持下的临床益处，在6个月随访时得到良好稳定，在12个月和36个月随访时患者完全满意。未出现重大并发症。
结论
乙醇栓塞加手术是有症状的FAVA病变安全、有效且长期的混合治疗方法。
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