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正中神经纤维脂肪瘤性错构瘤(FLH):1例罕见病例报告及文献复习

Fibrolipomatous Hamartoma (FLH) of Median Nerve: A Rare Case Report and Review.

作者信息

Ranjan Rajni, Kumar Rakesh, Jeyaraman Madhan, Kumar Sudhir

机构信息

Department of Orthopaedics, School of Medical Sciences and Research, Sharda University, Greater Noida, Uttar Pradesh 201 306 India.

出版信息

Indian J Orthop. 2020 Jun 1;55(Suppl 1):267-272. doi: 10.1007/s43465-020-00149-9. eCollection 2021 May.

Abstract

INTRODUCTION

Fibrolipomatous hamartoma (FLH) is a benign tumorous condition of adipose tissue. It is a slow growing, rare tumour involving peripheral nerves with uncertain aetiology. The clinical presentation varies as per presenting severity. The gold standard to diagnose this rare entity is MRI and its management is still controversial. In this report, we have described one such rare presentation of FLH involving Median Nerve in an adolescent male who successfully witnessed resolution of symptoms following our surgical intervention for the same.

CASE PRESENTATION

A 17-year-old male presented to our hospital with a history of swelling in his left hand since past 1 year. The detailed clinical evaluation revealed an irregular fusiform swelling extending from the ulnar aspect of wrist proximally to the thenar eminence along with involvement of the base of 2nd, 3rd and 4th fingers of left hand with consistency varying from soft to firm, tinel's sign positive and a terminal restriction of palmar and dorsiflexion movements. Notably, motor weakness and sensory loss were not elicited. The definitive diagnosis was established by MRI of the left hand. With due consent, the patient was undertaken for surgical intervention whereby his carpal tunnel was decompressed and biopsy of the lesion was sent for further histopathological evaluation as per the protocol. The absolute resolution in symptoms was reported by the patient following our intervention.

CONCLUSION

A meticulous clinical and radiological correlation is required to diagnose such rare clinical entity for improvising the functional quality of life. There is no definitive treatment for lipomatosis of nerve. However, a conservative approach is commonly advocated with successful results from decompression as in our patient.

摘要

引言

纤维脂肪瘤性错构瘤(FLH)是一种脂肪组织的良性肿瘤性疾病。它生长缓慢,是一种罕见的累及周围神经且病因不明的肿瘤。临床表现因病情严重程度而异。诊断这种罕见疾病的金标准是磁共振成像(MRI),其治疗方法仍存在争议。在本报告中,我们描述了一名青少年男性中FLH累及正中神经的罕见病例,在我们对其进行手术干预后,该患者症状成功缓解。

病例介绍

一名17岁男性因左手肿胀1年病史前来我院就诊。详细的临床评估显示,一个不规则的梭形肿胀从腕部尺侧近端延伸至鱼际隆起,同时累及左手第2、3和4指基部,质地从软到硬不等,Tinel征阳性,掌屈和背屈运动终末受限。值得注意的是,未引出运动无力和感觉丧失。通过左手MRI确诊。在获得适当同意后,对患者进行了手术干预,术中对其腕管进行减压,并按照方案将病变组织活检送去做进一步的组织病理学评估。患者在我们的干预后报告症状完全缓解。

结论

需要细致地进行临床和影像学关联以诊断这种罕见的临床疾病,从而改善生活功能质量。对于神经脂肪瘤病尚无明确的治疗方法。然而,通常提倡采取保守方法,如我们的患者那样,减压治疗取得了成功。

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