Long-term Postoperative Clinical Outcomes after Intramedullary Cavernoma Resection


Authors: N. Svoboda 1;  O. Bradáč 1;  V. Beneš 1
Authors‘ workplace: Neurochirurgická a neuroonkologická klinika 1. LF UK a ÚVN – VFN Praha 1
Published in: Cesk Slov Neurol N 2017; 80/113(5): 564-568
Category: Original Paper
doi: https://doi.org/10.14735/amcsnn2017564

Overview

Introduction:
Cavernomas are rare vascular malformations originating from any part of the central nervous system (CNS). They are associated with severe morbidity. Resection of such a lesion is the only curative approach. Aim: To evaluate outcomes of patients undergoing resection of intramedullary cavernoma (IMC).

Methods:
We analysed retrospectively records of patients who underwent resection of pathologically confirmed IMC between 1998 and 2016. Preoperative status and magnetic resonance imaging were evaluated as well as immediate and long-term postoperative outcomes.

Results:
We performed 20 surgeries (12%) in 17 patients. Male to female ratio was 13 : 4. The mean patient age was 43 years at the time of surgery. Spinal levels of cavernomas were cervical in seven patients (35%) and thoracic in 13 patients (65%). The mean volume was 1.3 ml (0.2–6 ml). In six patients (35%), multiple cavernomas of the CNS were discovered and in one patient (6%), a hereditary CCM1 mutation was confirmed. Symptoms were motoric in 14 patients (70%), sensory in 13 patients (65%) and bladder and/or bowel in three patients (15%). Nine patients (45%) presented with an acute, three patients (15%) with a stepwise and eight patients (40%) with a progressive neurological decline. The calculated annual risk of haemorrhage was 2.3%. Long-term improvement was observed in seven patients (35%), 12 patients (60%) remained stable and one patient deteriorated.

Conclusion:
Based on our results, we conclude that it is convenient to perform IMC resection when it starts to be symptomatic. We should avoid waiting until the patient deteriorates.

Key words:
cavernoma – cavernous hemangioma – central nervous system – spinal cord vascular diseases

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manu­script met the ICMJE “uniform requirements” for biomedical papers.


Chinese summary - 摘要

髓内海绵状血管瘤切除手术后长期术后临床效果观察

介绍:

海绵状血管瘤是一种起源于中枢神经系统(CNS)任何部分的罕见型血管畸形。该疾病伴随着严重的并发症。切除这种病变是唯一的有效治疗方法。

目的:评估进行过髓内海绵状血管瘤切除术(IMC)患者的预后情况。

方法:

我们分析了1998至2016年期间接受病理证实的IMC切除术患者的回顾性记录。评估了患者的术前状态和磁共振成像结果,以及术后近期和远期的疗效情况。

结果:

我们对17名患者进行了20次手术(12%)。男女比例为13:4。手术时平均患者年龄为43岁。7名患者(35%)的海绵状血管瘤脊柱水平为宫颈,13名患者(65%)为胸椎。平均体积为1.3ml(0.2-6ml)。其中6例患者(35%)发现了CNS多发性海绵状血管瘤,1例患者(6%)确诊为遗传性CCM1突变。14例患者(70%)有肌肉运动症状,13例(65%)有感觉障碍,3例(15%)有膀胱和/或肠道感染。9例患者(45%)出现急性神经衰退,3例(15%)出现逐步神经衰退,8例(40%)出现进行性神经衰退。计算出的年出血风险为2.3%。7例患者(35%)出现长期改善,12例(60%)保持稳定,1例恶化。

结论:

根据结果,我们得出结论:当IMC开始出现症状时,进行IMC切除是很方便的。 我们应该避免等到病人病情恶化。

关键词:

血管瘤 - 海绵状血管瘤 - 中枢神经系统 - 脊髓血管疾病


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Labels
Paediatric neurology Neurosurgery Neurology

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