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一例罕见的多发性纤维脂肪静脉畸形病例报告。

A Report of a Rare Case of Multiple Fibro adipose Venous Anomaly.

作者信息

Kumar L Dhruva, Gopurathingal Anto, Chinder Pramod, Hindiskere Suraj

机构信息

Department of Orthopaedic Oncology, Healthcare Global, Bengaluru, Karnataka, India.

出版信息

J Orthop Case Rep. 2022 Jun;12(6):19-22. doi: 10.13107/jocr.2022.v12.i06.2848. Epub 2022 Jun 10.

DOI:10.13107/jocr.2022.v12.i06.2848
PMID:37065525
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10092387/
Abstract

INTRODUCTION

Although not described more than a decade ago, fibro adipose vascular anomaly has grown of increasing importance as conventional management of arterio venous malformation with interventional radiology measures carry little success and cause significant morbidity in pediatric age groups like the case report we present here. Surgical resection, even though requiring significant loss of muscle bulk, is the mainstay of the treatment.

CASE REPORT

An 11-year-old patient presented with equinus deformity and intensely tender calf and foot swellings in the right leg. Magnetic resonance imaging showed presence of two distinct lesions, one involving the gastrocnemius and soleus, and the other in the Achilles tendon for which en bloc resection of the tumor was performed. Histopathology of the specimens confirmed the diagnosis of fibro adipose venous anomaly.

CONCLUSION

As per our knowledge, this is the first case of multiple fibro adipose venous anomaly confirmed by clinical features, radiology, and histopathology.

摘要

引言

尽管纤维脂肪血管异常在十多年前才被描述,但随着介入放射学方法对动静脉畸形的传统治疗收效甚微且在儿科年龄组中会导致严重的发病率,就像我们在此呈现的病例报告一样,其重要性日益增加。手术切除尽管需要大量肌肉组织缺失,但仍是主要的治疗方法。

病例报告

一名11岁患者出现马蹄内翻畸形,右小腿和足部肿胀且压痛剧烈。磁共振成像显示有两个不同的病变,一个累及腓肠肌和比目鱼肌,另一个位于跟腱,为此对肿瘤进行了整块切除。标本的组织病理学证实了纤维脂肪静脉异常的诊断。

结论

据我们所知,这是首例经临床特征、放射学和组织病理学证实的多发性纤维脂肪静脉异常病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c92/10092387/efaa879fce8d/JOCR-12-19-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c92/10092387/efaa879fce8d/JOCR-12-19-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7c92/10092387/efaa879fce8d/JOCR-12-19-g001.jpg

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Fibro Adipose Vascular Anomaly: A Rare and Often Misdiagnosed Entity.纤维脂肪血管异常:一种罕见且常被误诊的病症。
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Fibro-adipose vascular anomaly (FAVA): three case reports with an emphasis on the mammalian target of rapamycin (mTOR) pathway.纤维脂肪血管异常(FAVA):三例病例报告,重点介绍哺乳动物雷帕霉素靶蛋白(mTOR)通路。
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