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散发性及与冯·希佩尔-林道病相关的脊髓血管母细胞瘤的显微外科治疗:单中心经验

Microsurgical Treatment of Sporadic and von Hippel-Lindau Disease Associated Spinal Hemangioblastomas: A Single-Institution Experience.

作者信息

Das Joe M, Kesavapisharady Krishnakumar, Sadasivam Saravanan, Nair Suresh Narayanan

机构信息

Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, India.

出版信息

Asian Spine J. 2017 Aug;11(4):548-555. doi: 10.4184/asj.2017.11.4.548. Epub 2017 Aug 7.

Abstract

STUDY DESIGN

Retrospective cohort study.

PURPOSE

To examine the clinical profile and surgical complications in patients with spinal hemangioblastomas and to evaluate the long-term outcome in them.

OVERVIEW OF LITERATURE

Although considered to be histologically benign, hemangioblastomas may cause significant neurological deficits. The proportion of spinal hemangioblastomas associated with von Hippel-Lindau (VHL) disease has been estimated be 13%-59%. Preoperative neurological function correlates with postoperative neurological status. Studies have shown no difference in outcomes between sporadic and VHL-associated spinal hemangioblastomas.

METHODS

This retrospective study included 14 consecutive patients treated for spinal hemangioblastomas at our institute between January 2000 and June 2013. The mean follow-up period was 5 years. Magnetic resonance imaging of the complete neuraxis was performed in all cases, and preoperative embolization was performed in two cases.

RESULTS

In total, 14 patients underwent 18 surgeries, of which 15 were for spinal hemangioblastomas. Of all the patients, 86% had motor weakness and 79% presented with sensory disturbances. Preoperative McCormick functional grades were grade I in 7 (50%), grade II in 3 (21%), and grade III in 4 (29%) patients; 50% patients were diagnosed with VHL disease. All patients underwent complete resection of the tumor. Eight patients experienced deterioration in their neurological status in the immediate postoperative period; among them, five had gradual improvement. At 5-year follow-up, 11 (78.57%) patients showed good functional outcomes.

CONCLUSIONS

Microsurgical excision of spinal hemangioblastomas can cause postoperative morbidity, mainly in the form of neurological deterioration. Almost half of our patients had deterioration in the McCormick grade in the immediate postoperative period. However, a complete microsurgical excision can result in good long-term functional outcomes, as most of the immediate postoperative neurological deterioration in our patients was reversible. There was no difference in the long-term functional outcomes between sporadic and VHL-associated spinal hemangioblastomas.

摘要

研究设计

回顾性队列研究。

目的

研究脊髓血管母细胞瘤患者的临床特征和手术并发症,并评估其长期预后。

文献综述

尽管血管母细胞瘤在组织学上被认为是良性的,但仍可能导致严重的神经功能缺损。据估计,与冯·希佩尔-林道(VHL)病相关的脊髓血管母细胞瘤比例为13%-59%。术前神经功能与术后神经状态相关。研究表明,散发性和VHL相关的脊髓血管母细胞瘤在预后方面没有差异。

方法

本回顾性研究纳入了2000年1月至2013年6月期间在我院接受脊髓血管母细胞瘤治疗的14例连续患者。平均随访期为5年。所有病例均进行了全神经轴磁共振成像,2例患者进行了术前栓塞。

结果

共有14例患者接受了18次手术,其中15次手术针对脊髓血管母细胞瘤。所有患者中,86%有运动无力,79%有感觉障碍。术前麦考密克功能分级为I级的有7例(50%),II级的有3例(21%),III级的有4例(29%);50%的患者被诊断为VHL病全部患者均接受了肿瘤全切术。8例患者在术后即刻出现神经功能状态恶化;其中5例逐渐改善。在5年随访时,11例(78.57%)患者显示出良好的功能预后。

结论

脊髓血管母细胞瘤的显微手术切除可导致术后并发症,主要表现为神经功能恶化。我们几乎一半的患者在术后即刻麦考密克分级恶化。然而,完整的显微手术切除可带来良好的长期功能预后,因为我们患者中大多数术后即刻的神经功能恶化是可逆的。散发性和VHL相关的脊髓血管母细胞瘤在长期功能预后方面没有差异。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4f78/5573848/d5eaa894150f/asj-11-548-g001.jpg

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