Long-term Postoperative Clinical Outcomes after Intramedullary Cavernoma Resection

Česká verzia

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

Summary

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

Zdroje

1. Del Curling O Jr, Kelly DL Jr, Elster AD, et al. An analysis of the natural history of cavernous angiomas. J Neurosurg 1991;75(5):702– 8.

2. Robinson JR, Awad IA, Little JR. Natural history of the cavernous angioma. J Neurosurg 1991;75(5):709– 14.

3. Liang JT, Bao YH, Zhang HQ, et al. Management and prognosis of symptomatic patients with intramedullary spinal cord cavernoma: clinical article. J Neurosurg Spine 2011;15(4):447– 56.

4. Jellinger K. Pathology of Spinal Vascular Malformations and Vascular Tumors. In: Pia HW, Djindjian R, eds. Spinal Angiomas: Advances in Diagnosis and Therapy. Berlin: Springer Berlin Heidelberg 1978:18– 44.

5. Ogilvy CS, Louis DN, Ojemann RG. Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management. Neurosurgery 1992;31(2):219– 29; discussion 229– 30.

6. Bian LG, Bertalanffy H, Sun QF, Shen JK. Intramedullary cavernous malformations: clinical features and surgical technique via hemilaminectomy. Clin Neurol Neurosurg 2009;111(6):511– 7. doi: 10.1016/ j.clineuro.2009.02.003.

7. Badhiwala JH, Farrokhyar F, Alhazzani W, et al. Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data: Clinic article. J Neurosurg Spine 2014;21(4):662– 76. doi: 10.3171/ 2014.6.SPINE13949.

8. Májovský M, Netuka D, Bradáč O, et al. Chirurgická léčba supratentoriálních kortiko-subkortikálních kavernomů. Cesk Slov Neurol N 2014;110(5):631– 37.

9. Bradac O, Majovsky M, de Lacy P, et al. Surgery of brainstem cavernous malformations. Acta Neurochir (Wien) 2013;155(11): 2079– 83. doi: 10.1007/ s00701-013-1842-6.

10. Gross BA, Du R, Popp AJ, et al. Intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29(3):E14. doi: 10.3171/ 2010.6.FOCUS10144.

11. Mitha AP, Turner JD, Abla AA, et al. Outcomes following resection of intramedullary spinal cord cavernous malformations: a 25-year experience. J Neurosurg Spine 2011;14(5):605– 11.

12. Labauge P, Bouly S, Parker F, et al. Outcome in 53 patients with spinal cord cavernomas. Surg Neurol 2008;70(2):176– 81; discussion 181. doi: 10.1016/ j.surneu. 2007.06.039.

13. McCormick PC, Torres R, Post KD, et al. Intramedullary ependymoma of the spinal cord. J Neurosurg 1990;72(4):523– 32.

14. Canavero S, Pagni CA, Duca S, et al. Spinal intramedullary cavernous angiomas: a literature meta-analysis. Surg Neurol 1994;41(5):381– 8.

15. Zevgaridis D, Medele RJ, Hamburger C, et al. Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141(3):237– 45.

16. Aoyama T, Hida K, Houkin K. Intramedullary cavernous angiomas of the spinal cord: clinical characteristics of 13 lesions. Neurol Med Chir (Tokyo) 2011;51(8):561– 6.

17. Endo T, Aizawa-Kohama M, Nagamatsu K, et al. Use of microscope-integrated near-infrared indocyanine green videoangiography in the surgical treatment of intramedullary cavernous malformations: report of 8 cases. J Neurosurg Spine 2013;18(5):443– 9. doi: 10.3171/ 2013.1.SPINE12482.

18. Jallo GI, Freed D, Zareck M, et al. Clinical presentation and optimal management for intramedullary cavernous malformations. Neurosurg Focus 2006;21(1):e10.

19. Kharkar S, Shuck J, Conway J, et al. The natural history of conservatively managed symptomatic intramedullary spinal cord cavernomas. Neurosurgery 2007;60(5):865– 72; discussion 865– 72.

20. Lu DC, Lawton MT. Clinical presentation and surgical management of intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29(3):E12. doi: 10.3171/ 2010.6.FOCUS10139.

21. Tong X, Deng X, Li H, et al. Clinical presentation and surgical outcome of intramedullary spinal cord cavernous malformations. J Neurosurg Spine 2012;16(3):308– 14. doi: 10.3171/ 2011.11.SPINE11536.

22. Odom GL, Woodhall B, Margolis G. Spontaneous hematomyelia and angiomas of the spinal cord. J Neurosurg 1957;14(2):192– 202.

23. Marusič P. Diagnostika epileptických záchvatů. Cesk Slov Neurol N 2015;78(111):253–62.

24. Cantore G, Delfini R, Cervoni L, et al. Intramedullary cavernous angiomas of the spinal cord: report of six cases. Surg Neurol 1995;43(5):448– 51; discussion 451– 2.

25. Kim LJ, Klopfenstein JD, Zabramski JM, et al. Analysis of pain resolution after surgical resection of intramedullary spinal cord cavernous malformations. Neurosurgery 2006;58(1):106– 11; discussion 106– 11.

26. Deutsch H. Pain outcomes after surgery in patients with intramedullary spinal cord cavernous malformations. Neurosurg Focus 2010;29(3):E15. doi: 10.3171/2010.6.FOCUS10108.

27. Andrašinová T. Spinální arteriovenózní malformace – dvě kazuistiky. Cesk Slov Neurol N 2014;77(110):505– 509.

28. Smrčka M. Problematika indikace operační léčby u intramedulárních lézí. Cesk Slov Neurol N 2010;73(106):393– 397.

29. Brotchi J. Intrinsic spinal cord tumor resection. Neurosurgery 2002;50(5):1059–63.