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脊柱硬膜外脂肪增多症的疑难诊断:一例报告并文献复习

A challenging diagnosis of spinal epidural lipomatosis: A case report and review of the literature.

作者信息

Salman Ayman A, Ahmed Amani B, Sharabati Haneen, Bakri Izzeddin A, Al-Darawish Asad M

机构信息

Department of Neurosurgery, Al-Makassed Islamic Charitable Hospital, Jerusalem, Palestine.

Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.

出版信息

Int J Surg Case Rep. 2024 Aug;121:109948. doi: 10.1016/j.ijscr.2024.109948. Epub 2024 Jun 27.

Abstract

INTRODUCTION

Spinal Epidural Lipomatosis (SEL) is a rare disorder of pathological overgrowth of the spinal epidural fat in the extradural space. The pathogenesis of SEL usually involves exogenous steroid use or endogenous steroids overproduction. However, idiopathic cases have been reported. Magnetic resonance imaging (MRI) is the gold standard for diagnosis. Both conservative and surgical approaches are employed in management of these patients.

CASE PRESENTATION

A 17-year-old male presented to our hospital complaining of progressive lower limb weakness, loss of sensation with urinary incontinence which ended up with paraplegia. He underwent extensive investigations and received multiple inaccurate diagnoses. MRI of the thoracic spine showed spinal epidural lipomatosis with dorsal kyphosis. Hemi-laminectomy for spinal cord decompression and trans-pedicular fixation for correction of kyphosis were performed showing excellent outcomes.

CLINICAL DISCUSSION

Diagnosing SEL can be challenging due to its symptom overlap with other neurological conditions. Thus, higher levels of clinical suspicions and utilization of numerous diagnostic modalities including MRI are required. Treatment is largely determined by the clinical presentation and the severity of symptoms. Given the severity of neurological symptoms in our case, surgical intervention was performed resulting in fully regained functionality of previously paralyzed muscles.

CONCLUSION

This case highlights the rare presentation and the diagnostic challenges of spinal epidural lipomatosis SEL in a young patient who was misdiagnosed for 9 consecutive months before receiving the correct diagnosis, emphasizing the importance of considering SEL in the differential diagnosis for progressive neurological deficits and the importance of MRI, especially in atypical cases.

摘要

引言

脊髓硬膜外脂肪增多症(SEL)是一种罕见的疾病,其特征为硬膜外间隙的脊髓硬膜外脂肪病理性过度生长。SEL的发病机制通常涉及外源性类固醇的使用或内源性类固醇的过度产生。然而,也有特发性病例的报道。磁共振成像(MRI)是诊断的金标准。对于这些患者的治疗,既采用保守方法,也采用手术方法。

病例介绍

一名17岁男性因进行性下肢无力、感觉丧失伴尿失禁前来我院就诊,最终发展为截瘫。他接受了广泛的检查,并得到了多个不准确的诊断。胸椎MRI显示脊髓硬膜外脂肪增多症伴后凸畸形。进行了半椎板切除术以减压脊髓,并进行了经椎弓根固定以矫正后凸畸形,结果良好。

临床讨论

由于SEL的症状与其他神经系统疾病重叠,诊断SEL可能具有挑战性。因此,需要更高的临床怀疑度,并利用包括MRI在内的多种诊断方法。治疗很大程度上取决于临床表现和症状的严重程度。鉴于我们病例中神经症状的严重性,进行了手术干预,使先前瘫痪的肌肉功能完全恢复。

结论

本病例突出了脊髓硬膜外脂肪增多症SEL在一名年轻患者中的罕见表现和诊断挑战,该患者在得到正确诊断之前连续9个月被误诊,强调了在进行性神经功能缺损的鉴别诊断中考虑SEL的重要性以及MRI的重要性,尤其是在非典型病例中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5378/11268336/b94a6160e70c/gr1.jpg

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