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伴有脊髓空洞症的症状性胸椎硬膜外脂肪增多症:病例说明

Symptomatic thoracic epidural lipomatosis with syringomyelia: illustrative case.

作者信息

Przepiorka Lukasz, Wójtowicz Katarzyna, Kunert Przemysław

出版信息

J Neurosurg Case Lessons. 2023 Jul 31;6(5). doi: 10.3171/CASE23249.

Abstract

BACKGROUND

Spinal epidural lipomatosis is a rare but well-described disease. Here the authors present an extraordinary co-occurrence of spinal epidural lipomatosis with syringomyelia that did not improve after laminoplasty but required duroplasty as a definitive treatment.

OBSERVATIONS

This is the seventh case in the literature describing a co-occurrence of spinal epidural lipomatosis and syringomyelia. The pragmatic argument for a causative relationship between 2 phenomena is syrinxes reduction after surgery for spinal epidural lipomatosis. Additionally, duroplasty for spinal epidural lipomatosis in the setting of syringomyelia has not previously been reported. Duroplasty offers much better reconstruction of the subarachnoid space as compared with extradural decompression surgeries. It is one of the treatment options for spinal epidural lipomatosis. Last, most of the patients described in the literature with secondary syringomyelia were women with diabetes mellitus.

LESSONS

The co-occurrence of spinal epidural lipomatosis and syringomyelia is extremely rare. Neurological deficits in such cases respond well to surgical treatment. Accordingly, each case described in the literature resulted in syringomyelia reduction or resolution after surgery. Duroplasty may be necessary in selected cases of spinal epidural lipomatosis. Close follow-up and ongoing management of patients with epidural lipomatosis is therefore important.

摘要

背景

脊髓硬膜外脂肪增多症是一种罕见但已被充分描述的疾病。在此,作者报告了一例脊髓硬膜外脂肪增多症与脊髓空洞症同时出现的特殊病例,该病例在椎板成形术后病情未改善,但需要进行硬脊膜成形术作为最终治疗手段。

观察结果

这是文献中描述的第七例脊髓硬膜外脂肪增多症与脊髓空洞症同时出现的病例。支持这两种现象之间存在因果关系的一个实际论据是,脊髓硬膜外脂肪增多症手术后脊髓空洞缩小。此外,此前尚未有在脊髓空洞症背景下对脊髓硬膜外脂肪增多症进行硬脊膜成形术的报道。与硬膜外减压手术相比,硬脊膜成形术能更好地重建蛛网膜下腔。它是脊髓硬膜外脂肪增多症的治疗选择之一。最后,文献中描述的大多数继发性脊髓空洞症患者为患有糖尿病的女性。

经验教训

脊髓硬膜外脂肪增多症与脊髓空洞症同时出现极为罕见。此类病例中的神经功能缺损对手术治疗反应良好。因此,文献中描述的每一例病例在手术后脊髓空洞均缩小或消失。在某些脊髓硬膜外脂肪增多症病例中可能需要进行硬脊膜成形术。因此,对硬膜外脂肪增多症患者进行密切随访和持续管理非常重要。

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