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2型神经纤维瘤病患者因多发性神经鞘瘤行胸外科手术后发生脑疝。

Cerebral hernia caused by a thoracic surgery for multiple schwannomas in a patient with Neurofibromatosis type 2.

作者信息

Qin Sida, Yang Chengcheng, Ren Hong

机构信息

First Affiliated Hospital, Xi'an Jiaotong University, Faculty of Medicine, Departments of Thoracic Surgery, Xi'an, P.R. China.

出版信息

Turk Neurosurg. 2013;23(2):245-8. doi: 10.5137/1019-5149.JTN.4091-11.1.

Abstract

Schwannoma is a benign nerve sheath tumor composed of Schwann cells. A solitary schwannoma in the thorax is not rare, but a patient with Neurofibromatosis type 2 (NF2) and multiple thoracic schwannomas is extremely rare. We report the case of a 27-year-old woman with NF2 and two large schwannomas in her mediastinum and chest wall. We performed thoracic surgery on the patient, but she developed a cerebral hernia immediately after the operation. We report this case in order to analyze the relationship between the operation and cerebral hernia and find a way to prevent this consequence in the future. We conclude that a cranial MRI must be performed for patients with multiple thoracic schwannomas before the surgery. If there is a tumor inside the cranium, the disease in the brain must be treated first to prevent the occurrence of cerebral hernia or hemorrhage.

摘要

施万细胞瘤是一种由施万细胞组成的良性神经鞘瘤。孤立性胸段施万细胞瘤并不罕见,但患有2型神经纤维瘤病(NF2)且有多发性胸段施万细胞瘤的患者极为罕见。我们报告一例27岁患有NF2的女性病例,其纵隔和胸壁有两个大的施万细胞瘤。我们对该患者进行了胸外科手术,但她在术后立即出现了脑疝。我们报告此病例以分析手术与脑疝之间的关系,并寻找未来预防这种后果的方法。我们得出结论,对于有多发性胸段施万细胞瘤的患者,术前必须进行头颅磁共振成像(MRI)检查。如果颅骨内有肿瘤,必须先治疗脑部疾病以防止脑疝或出血的发生。

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